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A 38-year-old male presented to our institution for peri-implant fracture of left radius that occurred after two years of radius plating with LC DCP (Synthes, India). The X-ray showed a transverse fracture of the left radius at the proximal end of the plate []. After routine examination and work up, the patient was taken up for surgery.\nDuring the surgery, removal of the two screws from the plate on the opposing ends of the plate was difficult and resulted in stripping of the sockets of the screw heads [].\nThe tapering drill bit designed for removal of titanium screws by cutting the threads in the shaft of the screw in the reverse drilling direction was not available. We used a locally manufactured stainless steel hollow mill, actually designed to cut the bone around screw during jammed screw removal from the bone. Using it in a high-speed drill, we were able to grate out the titanium metal at the plate hole - screw head interface which facilitated the plate removal. The protruding screw heads from the bone were then easily held by a nose plier and removed by rotatory motion. To prevent contamination of local tissue by metal particles, wet gauge pieces were kept around each side of the plate. The fracture was reduced and fixed with a new 10-hole titanium locking plate, making new holes on each side of peri-implant fracture [].\nThe wound was washed using high-volume, low-pressure system to clear the wound of possible contamination from very small metal particles. It took us about 7 minutes each to grate out the metal around each screw head and total duration of surgery was 2 hours.\nNo complication was observed during postoperative period. There has been no systemic or local complication up till 2 years of follow-up.\nFigure – experimentally demonstrate the steps of removal of a screw from one of the undamaged holes of the extracted titanium plate by reproducing the steps used in the surgery. |
A 73-year-old man was admitted to our emergency department with chest pain. As he stated, he had been suffering from pain without radiation for the past 30 minutes, accompanied by nausea, vomiting, and diaphoresis. He claimed that he had had a previous similar episode the day before his admission, but the pain had resolved spontaneously after about one hour. He had a history of cigarette smoking, hypertension, and respiratory disease, for which he had received no appropriate and regular medical care. He had used Atenolol for the last three months in an irregular manner. No abnormal findings were detected on his physical examination (blood pressure = 140/80 mmHg, heart rate = 60 bpm, and normal breathing and heart sounds). His initial electrocardiogram (ECG) showed a left-axis deviation and left anterior hemiblock with ischemic changes (inverted T wave) on its pericardial leads. He had elevated cardiac troponin, and no other significant abnormalities were detected in his blood tests. He underwent medical management for acute coronary syndrome, and his pain was reduced within his first hour of admission. Echocardiography was performed for the patient and revealed a moderate left ventricular (LV) dysfunction with an LV ejection fraction of 40% and anterior and lateral wall hypokinesia. Mild LV hypertrophy was also detected. The patient was scheduled for coronary angiography on the following day. No chest pain or other symptoms were reported during the first night. The next morning, the ECG showed the evidence of a posterior (lateral) myocardial infarction (MI).\nThe patient underwent cardiac catheterization via the standard Judkins techniques. The selective angiography of the left coronary artery showed a critical and thrombotic stenosis on the midportion of the LAD as well as a moderate lesion on the midportion of the left circumflex (LCx) artery (). Mild stenoses were also detected at the proximal end of the LAD and high obtuse marginatus. The LAD ran through the interventricular groove but at the end of the midportion, it deviated from its usual course and transformed to a diagonal and supplied the anterolateral area of the LV. On the right side, the selective coronary artery injection revealed a dominant right coronary artery (RCA) and also an anomalous coronary artery arising just after the origin the RCA (). The accessory vessel ran into the left side and coursed into the mid and distal portions of the anterior interventricular groove and supplied the apex area. There were small septal arteries separating from it. On the left ventriculography, hypokinetic anterolateral and diaphragmatic walls were seen. The culprit lesions on the LAD were stented with a drug-eluting stent (DES), and the patient was discharged from the hospital with an acceptable condition on the fourth day of his hospitalization. |
A 45-year-old male presented to our hospital with diaphoresis, chills, and worsening right flank pain. He had a past medical history of cirrhosis due to alcoholism and portal hypertension with esophageal varices managed with prior transjugular intrahepatic portosystemic shunt (TIPS) procedure. He had a history of hepatic encephalopathy, hypertension, and type II diabetes. He had a recent admission to another institution for septic shock secondary to recurrent cholecystitis and had recently had an LC fifty-eight days prior. The patient was admitted to the outside hospital fifty-nine days prior to admission to our institution for subacute cholecystitis, which had initially required a cholecystostomy drain on prior admissions, and then ultimately an LC at that hospital. The gallbladder was not noted to be perforated, but the procedure was complicated by dropped gallstones, some of which were retrieved as stated in the operative report. On the current admission, the patient was hypotensive requiring vasopressors and was anemic and thrombocytopenic, requiring blood and platelet transfusions.\nA CT scan of the abdomen and pelvis with IV contrast was performed on admission demonstrating residual gallstones in the gallbladder fossa and/or cystic duct remnant and multiple small fluid collections and/or forming granulomatous masses surrounding additional retained/dropped gallstones in the surgical tracts and vicinity. A fistula with gallstones was also seen extending through the posterior and inferior wall of the second portion of the duodenum, to the anterior and superior aspect of a right renal cyst, which measured 3.4 cm x 3.0 cm and which contained gas in its superior aspect (Figures and ). A previous CT of the abdomen and pelvis performed with IV contrast sixty-five days prior to admission and seven days prior to the LC demonstrated a simple right renal cyst in the location of the now complex and infected cyst, measuring 2.8 cm x 2.5 cm ().\nDrainage of the infected renal cyst seen on the initial CT was considered; however, the cyst was relatively small and inaccessible. A repeat CT of the abdomen and pelvis with oral and IV contrast was performed four days after admission due to concern for abscess formation as the patient continued to have right flank pain. The CT demonstrated an unchanged superinfected cyst in the right kidney, with the fistula tract still visible, and heterogeneous retention of IV contrast in the right kidney, which was consistent with associated pyelonephritis (Figures and ).\nOn initial presentation, the patient was noted to have a history of Klebsiella pneumoniae and vancomycin-resistant enterococcus (VRE) in the cholecystectomy drain and was treated for the gallstone abscess and fistula accordingly with meropenem as there was no other source of infection. Blood cultures were later positive for K. pneumoniae and VRE. The patient was ultimately treated with linezolid and meropenem was deescalated to ceftriaxone.\nDiscussion between the patient's outside hepatologist, the abdominal radiologist, the interventional radiologist, and the gastroenterologists determined that the most likely etiology of the initial sepsis was an infected renal cyst secondary to an infected dropped gallstone. The patient was ultimately transferred to the outside hospital where he previously had his cholecystectomy for surgical follow-up. |
A female patient aged 51 years reported with severe, throbbing pain in left upper first premolar, that is, 24, since three days. The pain was aggravated on chewing and lingered on for few hours after removal of stimulus. There was history of sensitivity to cold beverages. The patient could recall episodes of similar pain with the same tooth for the past two years which was only temporarily relieved with desensitising tooth paste.\nOn clinical examination, no caries was detected nor restoration was present but a very faint craze line could be seen on the distal marginal ridge extending onto the distal proximal surface of 24 (). The extent of the craze line could not be determined. The tooth was tender on percussion, it did not exhibit mobility, and the fracture could not be separated using wedging forces. Periodontal probing depths were normal. Electric pulp test revealed immediate response. Radiograph did not confirm the fracture line or reveal any significant findings (). The patient was given a choice of using cone beam computed tomography (CBCT) to determine the extent of fracture and the prognosis of the tooth. She was explained the benefits and also assured that radiation exposure would be kept as minimal as possible. An informed consent was obtained from the patient. Cone beam computed tomography (Vatech, PaX-Reve 3D plus, pulse type generator, 5 × 5 cm field of view (FOV) and 0.08 mm voxel size) was used to determine the relative depth of fracture apically and the proximity of fracture to the pulp (Figures and ). The fracture line was found to extend mesiodistally involving lingual pulp horn and apically it did not extend below the level of alveolar bone. Prognosis was considered to be favourable. Root canal treatment followed by crown restoration was then decided as most appropriate treatment plan. The suspicion of fracture extending to deeper aspects of root, coupled with acute symptoms of pain in the patient prompted the usage of CBCT for a clearer diagnosis, prognosis, and treatment plan. |
A 53-year-old white man presented to our emergency department for blunt trauma following a high-speed motor vehicle collision as a restrained driver (he was wearing a three-point seatbelt). His history included nephrolithiasis 3 years ago that resolved with conservative medical therapy. He denied any surgical history. He was not on any anticoagulant or antiplatelet therapy prior to trauma. His social history included daily tobacco use and negative for any drug use. A family history did not reveal any coagulation disorders. On presentation, he complained of mild right upper quadrant and right flank pain. An examination revealed mild tenderness to deep palpation in his right upper quadrant and right flank, without evidence of ecchymosis, hematoma, or lacerations on his abdomen or his chest. There were no other abnormal findings on physical examination or laboratory values (Tables , , , , and ). He had normal vital signs without evidence of hypotension or tachycardia: blood pressure 158/110 mmHg, pulse 86 beats/minute, and temperature 37 °C (98.6 °F).\nA focused assessment with sonography for trauma (FAST) examination was performed in the emergency department and found to be negative, but owing to the high speeds involved in the crash, a computed tomography (CT) scan was subsequently ordered. The CT scan of his chest, abdomen, and pelvis revealed acute hemorrhage seen in the expected location of his right adrenal gland with an ovoid collection of increased density measuring 4.6 × 2.9 cm in size with periadrenal stranding and with blood tracking along the inferior margin of the right hepatic lobe (Fig. ). A normal right adrenal gland was not visualized due to the suspected hemorrhage in the area. The fluid seen on CT was consistent with blood product as opposed to adrenal mass. No other injuries were identified, specifically no injuries to his liver or kidneys. No rib or spine fractures were present.\nHe was admitted to our hospital for clinical observation and hemodynamic monitoring. His hemoglobin remained stable over 24 hours (Table ). He had normal coagulation studies, as well as normal liver functions (Tables and ). He had slight elevation in his renal function with admission blood urea nitrogen (BUN) of 19 and creatinine (Cr) at 1.24. Repeat laboratory tests at 24 hours were normalized with BUN 14 and Cr 0.89. His urine analysis (UA) was clinically insignificant with trace amount of blood with 3/high-power field (HPF), rare bacteria, and it was negative for bilirubin, leukocyte esterase, and nitrites. A prior UA done in 2013 showed similar trace blood in urine secondary to left ureterolithiasis. He had no electrolyte abnormalities and had a stable 24 hours of vital signs; therefore, there was no clinical indication to continue further workup for possible adrenal dysfunction.\nHis clinical condition was stable with improvement in right upper quadrant and flank pain and tenderness. He was discharged from our hospital with restrictions on physical activities and he was asked to avoid anticoagulants. A follow-up repeat CT scan was scheduled to assess resolution of adrenal hematoma. Furthermore, because adrenal gland injury is not usually present in the absence of other injuries and his right adrenal gland was not visualized, occult neoplasm must be included in the differential diagnosis and required a follow-up.\nAt the 1-month follow-up a CT scan with intravenously and orally administered contrast for better delineation of surrounding structures was completed and showed the adrenal gland hemorrhage had improved and reduced to approximately 3.0 × 2.4 cm in diameter, and the previously noted right periadrenal fatty stranding was mostly resolved (Fig. ). Again, there were no other abnormal findings noted. During the 1-month clinical examination, he reported intermittent right upper quadrant pain that occurred approximately one to two times per week but he was back to his regular activities. A physical examination showed no abdominal or flank tenderness.\nDue to persistent symptoms, he was instructed to follow-up in 3 months for another CT scan and examination. A 4-month CT scan was performed, which showed an improving appearance of right adrenal gland measuring maximally 1.2 cm transverse diameter and no other identifiable lesions (Fig. ). The time-dependent decrease in size of the right adrenal gland abnormality was consistent with hemorrhage, as opposed to adrenal neoplasm. He was asymptomatic at this time with resolution of abdominal pain and no symptoms of adrenal insufficiency. |
A 40-year-old White British female who mobilized in a wheelchair due to NMO presented to the emergency department in our center with a swollen, hot, painless right forearm. This patient had no current evidence of optic neuritis. She was treated with rituximab, a monoclonal antibody directed against the CD20 antigen. The arm had been in this condition for 8days, and the patient had taken antibiotics for what was presumed to be an infection. However, the forearm gradually worsened, leading to deformity and instability. After clinical evaluation, the forearm was placed in a cast, and a plain radiograph X-ray was taken to investigate bone fracture (). Comminuted fractures through the mid-radius and ulna were confirmed, and a significant soft-tissue swelling was also noted.\nDiagnoses considered for her symptoms were cellulitis, stress fractures of the radius and ulna, and pathological fractures of the radius and ulna due to malignancy or osteoporosis. Once instability was clinically evident, the presence of a fracture became obvious. Although this did not exclude the possibility of infection, the C reactive protein and white blood cell count were not suggestive of this differential. It was only after samples of bone were examined histologically that the presence of a malignancy was excluded. The presence of osteoporosis could not be completely excluded without a dual-energy X-ray absorptiometry bone density scan. However, since this patient was not on steroidal therapy for NMO but rather a biologic drug and given her young age, osteoporosis was deemed unlikely. This patient was pre-menopausal with a regular cycle and had no gynecological history of note. Stress fractures of the radius and ulna were the diagnosis most consistent with her symptoms, imaging, and histology.\nSince the patient was dependent on her upper limb to mobilize and transfer weight, we felt that it was important to stabilize the forearm contrary to usual practice. Therefore, the patient was listed for an open reduction and fixation of both the radius and ulna. Intraoperatively, the radial fracture was found to be simple with little communication. A ten-hole locking compression plate was selected with a reasonable working length and then contoured to respect the radial bow. The ulnar fracture was found to be comminuted with rounding of the fracture ends, suggesting that this had been partially fractured for some time, indicative of a fatigue fracture. Fixed-angle (locking) plates on both radius and ulna were used to minimize the risk of construct failure through implant cut-out, about which the patient would be unaware owing to her lack of sensibility in the arm. Bone samples from the fracture site were sent for histology to exclude malignancy, although this seemed unlikely from the macroscopic appearance of the fracture site. Further, the distal radioulnar joint was found to be stable, and the radiographs () were satisfactory.\nAs there was significant swelling at surgery, this patient was not suitable for a full plaster cast. In the short-term, a decision was taken to allow her to weight bear on the fixed forearm for transfers. She was encouraged to minimize the amount of load put through her forearm because her lack of sensation would have prevented her from noticing a construct failure. Despite fracture fixation, it was found in the initial follow-up clinic, 8days later, that the construct had failed. Radiographs showed that the radial plate had flexed and the deformity was approximately 30° (). Both wounds had also dehisced, due to the tension, they were under post-operatively. 5 weeks later, after satisfactory wound healing, a stronger bespoke radial plate was ordered, and the construct failure was successfully revised. The new construct was an eleven plus four hole volar rim plate pre-contoured into extension at the fracture site. It was protected in the immediate post-operative period with external casting in addition to the internal fixation (). 10 months after the revision, the patient has reported no further problems, the X-rays are progressing to union satisfactorily, and the patient has a good range of movement including forearm rotation despite heterotopic ossification in the interosseous membrane (). |
A 34-year-old Caucasian man with a 25-year history of CD was admitted to hospital for evaluation of a newly discovered liver mass. His past medical history was significant for an ileocolic resection when he was 14 years old and a proctocolectomy with end ileostomy when he was 22 years old for severe colonic disease resistant to medical therapy. At the age of 28, he developed peristomal pyoderma gangrenosum and seronegative polyarthritis for which therapy with azathioprine 1.5 mg/kg daily and infliximab 5 mg/kg every 6 weeks was initiated. For the previous 6 years, these doses remained the same and controlled his symptoms. He was otherwise well and took no additional medications. Born and raised in Canada, he worked as a structural engineer and was married without any children. He denied any smoking or alcohol or illicit drug use and had no family history of inflammatory bowel disease, liver disease, or malignancy. He had regular visits at his general practitioner and gastroenterologist. His last abdominal ultrasound was performed three years prior to presentation and was entirely normal. More recently, three months prior to presentation, he had routine blood work including liver enzymes as well as a gastroscopy and ileoscopy that were entirely normal. Unfortunately, over the ensuing months he developed progressive epigastric pain, nausea, fatigue, and 20 kg weight loss. Blood work revealed marked elevations in his transaminases and an abdominal ultrasound revealed a large liver mass. The patient was referred to our tertiary care academic institution to confirm the diagnosis and assist with management.\nOn presentation to hospital, the patient appeared well without any evidence of jaundice or stigmata of chronic liver disease. His liver enzymes and alpha-fetoprotein level were markedly elevated, while his liver function was normal ().\nAn abdominal CT scan revealed a 24-centimeter mass in his left hepatic lobe with tumor thrombosis involving the left portal vein and nodular masses in the right lobe (). A CT of the chest and pelvis did not reveal evidence of distant metastases. A complete liver disease workup was performed and included hepatitis B and C serology, alpha-1 antitrypsin, anti-nuclear antibody, smooth muscle antibody, anti-neutrophil cytoplasmic antibody, anti-myeloperoxidase antibody, proteinase 3 antibody, complement levels, immunoglobulin levels, iron studies, and ceruloplasmin. All laboratory results were entirely unremarkable. Multiple liver biopsies were performed which confirmed the diagnosis of HCC but were unable to identify any underlying liver tissue (Figures and ).\nThe case was presented at multidisciplinary rounds that included representatives from hepatology, medical oncology, radiation oncology, and surgical oncology. The diagnosis, prognosis, and treatment options were discussed with the patient and his family. Given the size of the tumor, neither surgery nor liver transplantation was a viable option, and the patient was commenced on Sorafenib therapy and was provided with transcatheter arterial chemoembolization (TACE). The decision was made to discontinue his azathioprine and infliximab since they may have been contributing to the rapid tumor growth. Immediately after TACE, the patient remained well without any evidence of liver dysfunction or distant metastases. He was discharged home with close follow-up from medical oncology and hepatology. Unfortunately, over the ensuing three months, he developed progressive liver dysfunction and sustained a massive oropharynx bleed that required multiple blood transfusions. It was determined that the severe bleeding episode was in the context of a tumor involving his right mandible that eroded into his oropharynx. Repeat imaging revealed widespread metastatic disease involving his lungs, adrenals, thoracic spine, maxilla, and mandible. Unfortunately, while in hospital the patient developed progressive liver and renal failure and was in considerable pain from his widespread metastatic disease. The patient was provided with palliative care and passed away approximately five months after his initial diagnosis with HCC. The family declined an autopsy. |
The case we present here is about a 32-year-old, female patient who reported to our hospital with the complaint of a painless swelling on the left side of the neck for past 8 months. The swelling was small initially and has progressed to the present size []. The swelling did not cause any functional restriction but was cosmetically evident, which was the patient's concern. On extraoral examination, there was a soft, fluctuant, compressible, nontender swelling of size 8-10 cm in the left submandibular region. The swelling extended anteriorly from the parasymphysis region of mandible, and extended posteriorly until the angle region of the mandible. Superiorly the swelling did not go beyond the lower border of mandible and inferiorly extended until the level of thyroid cartilage. On intraoral examination, there was no abnormal finding. The orifice of the left submandibular duct and the saliva flow was normal.\nOn intraoral examination, there were no abnormal finding and the patient did not complaint of any dryness of mouth or pain along the floor of the mouth or at the opening of the submandibular duct.\nAn aspiration was done from the swelling using a wide bore needle, which showed the presence of a Straw colored fluid. The aspirated fluid subjected for fine-needle aspiration cytology (FNAC), which revealed the presence of lymphocytes, few histiocytes, few plasma cells and squamous cells dispersed in a proteinaceous material. This almost proved that the lesion is a cyst from a lymphoepithelial origin.\nA computed tomography (CT) scan was done to assess the size and extent of the swelling which revealed the presence of a well circumscribed and encapsulated lesion measuring about 10 cm × 6 cm. The lesion did not show any invasion into the mandible or major blood vessels, except it was pushing the facial artery and vein medially into the neck. The capsule of the lesion was slightly adherent to the superficial part of the left side submandibular gland []. A routine blood investigation was performed. The patient was tested for human immunodeficiency virus (HIV) both with enzyme linked immunosorbent assay and Western blot test, both of which were negative.\nThe treatment was very definitive and the patient was planned for excision of the lesion under general anesthesia. The surgical approach was made with a horizontal neck incision along the neck crease. Facial artery and vein were seen adherent to the cystic wall and hence they were ligated. Marginal mandibular nerve was identified and preserved. The lesion was exposed and was completely enucleated along with the excision of the superficial part of the submandibular salivary gland. Hemostasis was achieved and closure was done. The specimen was sent for histopathological examination, which revealed the specimen consisting of cyst lined by glandular epithelium consisting of lymphoid follicles []. |
A 15-month-old girl presented with a right-side congenital craniofacial cleft. She also had syndactyly at the third webspace of the right hand and the second and fourth webspace of the left foot, but she was never diagnosed with syndromic disorder and never had a genetic consultation. A physical examination revealed that the cleft originated from the lateral to right Cupid’s bow and involved the lip, alveolus, palate, cheek, and lower eyelid (). The ipsilateral ala was displaced superiorly, but the circumferential shape of the nostril was intact. The exposed cornea had keratosis and she had severe dystopia and enophthalmos due to the absence of an inferior orbital wall. Preoperative computed tomography revealed that the cleft originated from the lateral to ipsilateral central incisor involving the medial and inferior wall of the maxillary sinus and inferior orbital wall (). This resulted in direct communication among the orbital, maxillary sinus, nasal, and oral cavity. Thus, we concluded that this craniofacial cleft corresponded to the number 3 cleft according to the Tessier classification.\nUnder general anesthesia, we performed soft tissue reconstruction using the straight-line advanced release technique, which was developed for unilateral cleft lip repair (). Design of the lateral mucosal (L) flap was extended to turn outward from the lower eyelid. Because it was difficult to cover all the inner lining of the cheek with the L flap alone, the medial mucosal (M) flap was widely designed to include the philtral tissue. The upper incision of the M flap was extended along the skin mucosal junction lateral to the nasofacial groove for the attachment of the L flap. As in the original technique, a triangular flap design and two slit incisions were also added in the design of the L and M flaps and the inner linings were marked through the gingivolabial sulcus. Finally, we designed a turn-over flap that turned outward from the lower eyelid along the subciliary incision line to cover the lateral lower eyelid defect.\nOn the cleft side, dissection was performed to separate the orbicularis oris muscle from the entire maxillary plane, and the extent of dissection was set as wide as possible to pull the cheek tissue maximally to the medial side. On the non-cleft side, the abnormally inserted orbicularis oris muscle was released from the anterior nasal spine and the base of pyriform aperture followed by subcutaneous dissection of the nasal dorsum and philtrum. After interchanging the L and M flap, the turn-over flap from the lower eyelid was added superiorly to complete the inner lining of the lower eyelid and cheek (). The freed orbicularis oris muscle was repaired with full width from the columellar base to the red vermilion. Finally, the oral lining of the lip was reconstructed by advancing the bilateral oral lining flap that was elevated through the gingivolabial incision, and skin closure was completed by advancing bilaterally undermined skin. Because of the widely performed subcutaneous dissection, there was no high tension on the skin suture.\nThe postoperative photographs showed the complete soft tissue reconstruction of the lip, cheek, and lower eyelid (). However, a defect remained in the medial lower eyelid forming the oroocular fistula, which needed to be repaired during a secondary surgery (). On the first postoperative day, there was no flap congestion, and the patient was discharged. The stitches were removed from the wound on the 7th postoperative day and there was no wound dehiscence 1 month after surgery (). |
A 44-year-old female with a <1 year history of locally advanced breast cancer presented for outpatient evaluation of new-onset neck pain. She noted the pain to be worse with rotation of the head and extreme flexion. She did not note any new weakness, numbness, or gait disturbance. On examination, she was guarded in her ability to move her head, as it elicited significant neck pain. She had normal reflexes and full strength on motor examination. Magnetic resonance imaging of the cervical spine revealed a new C2 vertebral metastasis centered about the base of the odontoid process without central canal stenosis []. There was evidence of partial posterior cortical surface violation and pathological fracture of the odontoid process without evidence of anterior cortex breakage on computed tomography imaging.\nAs this was the patient's first presentation of likely metastatic disease, biopsy and advancement of the patient's medical therapy was of high importance to her oncology treatment team. However, due to the location of the patient's lesion, her mechanical symptoms, and likely impending pathological complete odontoid fracture, a standard surgical treatment would involve C1–2 fusion with or without transoral resection. This would be fairly morbid in the short term and delay the onset of the needed oncological treatment. The alternative standard transoral needle biopsy and radiotherapy would not address the patient's bony pathology or mechanical symptoms, and could result in possible infectious complications. Thus, a hybrid approach was suggested, using an anterior cervical transodontoid biopsy and cement augmentation followed by SRS. Postoperative chemotherapy could be initiated early, and the patient could have a significantly shortened recovery time with some level of increased bony stability.\nA standard right-sided anterior cervical incision was made, and an approach similar to an odontoid screw was undertaken using a Jamshidi needle. After cannulating the C2 vertebral body, a biopsy was taken of the tumor in the odontoid process using a standard bone coring needle. Cement was then instilled within the C2 vertebra with continuous biplanar fluoroscopy until the lytic defects were adequately filled [].\nThe patient was discharged from the hospital on the first postoperative day without any new neurological deficits. Her preoperative neck pain had completely resolved with a full range of motion in her neck. The patient underwent SRS on the postoperative day 5 using the Cyberknife radiation delivery system at a dose of 18 Gy to the 87% isodose line conforming to the C2 vertebral body. Six-week and 6-month follow-ups were unremarkable, with no evidence of tumor recurrence or new evidence of atlantoaxial instability []. |
A 53-year-old female presented to our outpatient clinic with rather acute chronic severe left-sided knee pain. The pain was not trauma related and had lasted for a few days. There were symptoms of locking. The knee joint has not been operated on before. Clinical findings confirmed a stable left knee joint with limited motion for full flexion as well as extension (110-5-0). Lachman, anterior, and posterior drawer were negative. The collateral ligaments were stable in full extension as well as in 30° of flexion. The patellofemoral alignment was normal and there was no apprehension sign. Clinical test for the menisci was negative (partly false positive for the anterior part of the medial meniscus). There was mild effusion and clear sharp pain at the medial femoral condyle. Conventional X-rays confirmed no significant pathology, no signs of advanced arthritis, and a straight mechanical axis (not shown). Subsequent magnetic resonance imaging (MRI) confirmed a large area of freshly appearing bone marrow edema (BME) at the dorsomedial femoral condyle with overlying highly irregular cartilage (). The remaining joint appeared normal on MRI. After discussing the case with the patient we indicated to approach with knee joint arthroscopy first in order to inspect the medial condyle and debride the lesion plus potential antegrade drilling for relieve of the BME. During arthroscopy there appeared a large just recently separated pure chondral fragment at the dorsomedial femoral condyle with healthy appearing surrounding and opposing cartilage (). The medial meniscus appeared intact. The remaining joint structures appeared intact. With regard to a recent separation and healthy appearing surroundings we decided to proceed with arthrotomy in order to repair the cartilage defect by mincing the healthy appearing cartilage piece. Following arthrotomy the large fragment could be retrieved easily. It was purely chondral. A refixation was deemed not promising. Consequently the large fragment with healthy appearing cartilage was minced into multiple small cartilage chips (<1 × 1 × 1 mm) using a scalpel at the back table. In parallel the defect was debrided to create a stable and healthy cartilage rim. The subchondral bone was intact. Defect dimensions after debridement were 2.5 × 1.5 cm and ICRS grade 3b. Yet, with regard to the BME seen on MRI, we frequently drilled into the subchondral bone at different locations and in different angles using a constantly water cooled 1.4 K-wire in antegrade fashion. Hereafter, the autologous chips were placed into the debrided lesion and fixed using fibrin glue. The chips had more than enough quantity to cover the lesion. After dehydration the joint was put through multiple full range of motion procedures. The repair tissue remained in place. Subsequently, the joint was closed in layers. Rehabilitation was performed as previously reported []. Following an uneventful postoperative course the patient presented without pain or locking sensations at our outpatient department at 6 weeks, 12 weeks, and 6 months postoperatively. Albeit no full muscular function, swimming and biking were already possible at last follow-up. Six-month MRI was in display of almost full regression of the BME and satisfying novel cartilage surface with good integration into the surrounding cartilage and subchondral bone. The transplant signal appeared almost isointense to the neighbouring cartilage (). The calculated MOCART [] score was 85 points. Lysholm score was 80 points. The patient was subjectively very satisfied with the procedure and would undergo it every time again. |
A 35-year-old left-handed man who works as a mechanic and firefighter presented to our institution with hand pain and cold intolerance for 3 months. He was referred by a local vascular surgeon who had made the diagnosis of hypothenar hammer syndrome (HHS) following an angiogram of the right upper extremity showing complete occlusion of the ulnar artery (). On initial examination, Doppler signals of the ulnar artery, superficial palmar arch, and ring/small digital arteries were absent in the right hand. Subsequently, the patient developed an ulcer on his right long fingertip after minor trauma that had not healed over a 4-week period. He suffered ischemic hand pain and severe cold intolerance. After conservative management failed, the decision was made for operative intervention. We proposed using an arterial graft versus a venous graft in order to improve long-term patency. Our plan was to utilize the descending branch of the lateral circumflex femoral artery (DLCFA) as an arterial graft.\nIntraoperatively, Doppler ultrasound revealed a segmental thrombotic defect extending from the superficial palmar arch, across the Guyon canal, extending 10 cm proximally in the forearm. The ulnar artery was resected back to healthy bleeding pulsatile flow, resulting in a segmental defect of 12 cm. Final pathology of the submitted ulnar artery segment revealed organizing thromboembolus in the lumen, which at the time of resection was adherent to the lumen.\nThe DLCFA was harvested, with care taken to prevent injury to the lateral femoral cutaneous nerve. A 12-cm segment of the artery was circumferentially dissected, as well as approximately 2 cm of 2 branching vessels at the distal end of the segment that would be used to reconstruct the superficial palmar arch and common digital arteries with one graft. With the arterial graft completely dissected, it was ligated both distally and proximally.\nThe arterial graft and recipient vessels were examined under the operative microscope. The proximal anastomosis was preformed first. The two branches at the distal end of the arterial graft were anastomosed to the superficial palmar arch stump, and common digital artery to the ring and small finger, respectively. A bolus of 5000 units of heparin was given intravenously before release of the microvascular clamps. Adequate pulsatile flow was achieved though the graft and confirmed with Doppler ultrasound to each finger ( and , Video 1). The hand was then irrigated, and the ulnar nerve was explored to ensure that no injury had taken place. The skin was closed, and the patient's hand was placed into a dorsal splint.\nThe patient remained in the hospital overnight for monitoring and was discharged the following morning on oral aspirin as the only anticoagulant. On follow-up examination 2 days later, strong Doppler signals were elicited at the ulnar artery, palmar arch, and radial and ulnar aspect of each digit. At 1 month, Doppler signals remained strong and the ulcer previously present on the right long fingertip had completely resolved (, Video 2). During this visit he reported to be asymptomatic and has had complete resolution of the cold intolerance and burning pain of his right hand. |
A 15-year-old female patient with medically refractory epilepsy was treated surgically in N. N. Burdenko Neurosurgery Institute, Moscow, in October 2008. The patient had no family history of epilepsy. Pregnancy and delivery were reported as normal. First seizures occurred at the age of 9. The patient suffered from pharmacoresistant complex partial seizures without aura appearing with loss of consciousness, aversion to the right, falling, and sometimes with secondary generalization, with a frequency of 2–4 seizures per month, without any response to antiepileptic drug therapy. Therapy on admission was as follows: valproate — 2000 mg per day and carbamazepine — 1000 mg per day. She had no neurological deficit, and she was able to attend a regular school. Magnetic resonance imaging revealed bilateral malformation of cortical development and bilateral periventricular NT associated with CD in the right temporo-parietal region; the latter appeared in slight MRI abnormalities such as thickening of the cortex and dysgyria, which could be considered as CD. Mild atrophy of the right hemisphere was also noted (). The left hemisphere had no signs of CD. Surface EEG recording showed bilateral interictal epileptic discharges, without any focal seizure onset.\nAt first, the patient was considered as an unfavorable candidate for surgery because of bilateral lesions, but in view of the health hazard of unexpected falling during seizures, the decision was later made to continue presurgical evaluation and to make invasive neurophysiological investigation.\nInvasive monitoring was performed with the use of intra-cerebral electrodes placed according to stereotactic methods in the medial temporal and parietal regions and nodules of heterotopia on both sides. There was a marked disorganization of background activity with periods of rhythmic theta–delta activity in the medial temporal and parietal regions on the right side during the interictal period. Ictal onset was characterized by the appearance of fast activity in the right medial temporal regions followed by the rapid spreading of epileptic discharge to the left hemisphere ().\nBased on the neurophysiological data, the right temporal and parietal cortices were considered as part of the epileptogenic zone, but we supposed that the real dimension of the epileptogenic zone was more extensive than only the right temporal and parietal cortices. It was entirely possible that the left-side NH would be epileptogenic too. Nevertheless, we took the decision to perform a right-side partial disconnection procedure expecting a palliative result — the prevention of secondary generalization of seizures. The surgery was performed in December 2008.\nThe surgery procedure consisted of anterior temporal lobectomy and the opening of the posterior part of the temporal horn by dissection through the medial margin of the superior temporal gyrus to the trigonum, the removal of the right heterotopy nodus, the dissection of the parahippocampal gyrus and the medial part of the parietal lobe at the level of the posterior part of the ambiens cistern, the dissection of the posterior part of the corpus callosum, and the detachment of the temporo-parieto-occipital complex by dissection through the white matter of the parietal lobe just behind the sensorimotor cortex ().\nHistological examination of the basal temporal and parietal cortices and the hippocampus revealed CD type I according to the ILAE classification with immature neurons — type 1B according to the Palmini classification .\nThe patient was noted to have permanent hemianopia and transient hemiparesis for the first 2 months after surgery with complete recovery.\nStandardized follow-up evaluations were performed every 6 months after surgery. The patient has been seizure-free for more than 4 years after surgery and was treated with the same therapy as that used before surgery. She goes to college now. |
The patient was a 38-year-old nulliparous woman who suffered from infertility of unknown origin. She had been treated with assisted reproductive technologies including artificial insemination and in vitro fertilization for over four years. During her treatment for infertility, cytological review followed by colposcopic biopsy revealed an invasive nonkeratinizing squamous cell carcinoma (SCC). A 1 cm mass was identified in the uterine cervix, but a pelvic MRI did not describe the cervical mass or parametrial invasion. Additionally, a submucosal leiomyoma of 15 mm in diameter was found in the uterus (). CT scans showed no signs of lymph node swelling or distant metastases. Based on these findings, she was diagnosed with stage IB1 cervical squamous cell carcinoma. We offered radical hysterectomy and pelvic lymphadenectomy as standard treatment although she strongly desired fertility preservation. The submucosal leiomyoma may have been the cause of her infertility, and she was keen to resect the myoma during the same procedure. Submucosal leiomyomas can usually be resected with hysteroscopy but was not advised in this case from the oncological viewpoint. As such, we obtained informed consent and performed an abdominal radical trachelectomy followed by abdominal myomectomy.\nDuring the surgery, we first drained the ascites in the pelvic cavity, resected bilateral pelvic lymph nodes, and sent them for intraoperative pathology. They were reported to be negative. The paravesical and pararectal spaces were then developed. The ureters on either side were resected to their insertion into the bladder. The uterine arteries were ligated and cut at the origin where they branched from the internal iliac arteries. Next, the uterosacral ligaments were divided. A colpotomy was performed circumferentially, and the cervical specimen was excised together with the parametrium at least 2 cm below the internal os. During the surgery, a frozen section procedure was performed for histology. The patient was found to have a 5 mm free cervical margin. A permanent cerclage was placed at the level of the isthmus. The uterus was then reanastomosed to the vagina. We then performed resection of the submucosal myoma via a uterine vertical incision. An intrauterine device (FD-1; Fuji Latex Co., Tokyo, Japan) was placed in the uterine cavity. The operation duration was 339 min, and blood loss was 500 ml. The surgery was completed with no complications.\nThe final histological specimen confirmed the diagnosis of squamous cell carcinoma, keratinizing type of cervix uteri, pT1B1. Exocervical, endocervical, and deep margin regions were negative. There was no metastatic lesion in the lymph nodes or lymphovascular space invasion. Leiomyoma of the corpus uteri showed no malignancy. No adjuvant treatment was administered, and no recurrence has been reported for at least 18 months postoperatively.\nSix months after the surgery, she became pregnant following the postoperative first embryo transfer. The fetus was appropriate for gestational age. At 21 weeks of pregnancy, she claimed vaginal bleeding, and her lower uterine segment lengths were shortened from 23 mm to 13 mm. She was diagnosed with threatened abortion, and tocolysis was started. At 25 weeks, preterm premature rupture of membranes occurred. She received antibiotics, and intramuscular betamethasone was administered. At 26 weeks, a male baby weighing 980 g was delivered with an Apgar score 3/5/7 by caesarean section due to chorioamnionitis. The baby received general care in a neonatal intensive care unit for four months and weighed 4520 g when discharged. He is now 6 months old and is well. There has been no recurrent disease of her cervical cancer for 18 months from the trachelectomy and myomectomy. |
A 16-year-old boy visited an orthopedic hospital for pain in the right inguinal area that occurred after he fell down while standing on his hand during a physical exercise class, but as the simple X-ray did not show abnormal findings, the patient was observed with conservative management. Two days after the onset of symptoms, the patient had muscular weakness and hypesthesia on the right lower leg, so he underwent lumbar spine computed tomography (CT) at a general hospital, was hospitalized and treated conservatively under suspicion of spinal cord injury. Fourteen days after the onset of symptoms, since there was no improvement in his symptoms, the patient was transferred to the Department of Orthopedic Surgery in our hospital. He had no significant medical or trauma history before injury.\nThe simple X-ray of lumbar vertebrae and pelvic bone did not show abnormal findings. There was no abnormality on the thoracolumbar spine MRI except a mild herniated intervertebral disc between L5 and S1 and unilateral spondylolysis accompanied by spina bifida occulta.\nSeventeen days after the onset of symptoms, the patient was referred to our Department of Rehabilitation Medicine for the assessment of muscular weakness in the right lower extremity. On physical examination, muscular atrophy was observed as the circumference of the right thigh measured at 10 cm proximal to the patellar upper margin was 40 cm while that of the left thigh was 46 cm (), and severe tenderness along with ecchymosis was observed in the right inguinal area. According to the results of the manual muscle test (MRC, Medical Research Council), the left lower extremities were normal but the right lower extremities showed hip flexion 3/5, knee extension 2/5, ankle dorsiflexion 5/5, great toe extension 5/5 and ankle plantar flexion 5/5. On sensory tests, hypesthesia and paresthesia were observed in the right anteromedial and anterolateral thigh, and the medial side of the lower leg (). The deep tendon reflex of the right knee also decreased.\nOn motor nerve conduction study, the amplitude of compound muscle action potential on the right femoral nerve decreased to 0.8 mV compared to 12.0 mV on the left side (). Sensory nerve conduction study for lateral femoral cutaneous nerve stimulated 1 cm inferior part of the anterior superior iliac spine (ASIS), and active electrodes attached on the 17 cm distal to the ASIS along the virtual line connecting between the ASIS and the patellar lateral margin. According to the results, the amplitude of sensory nerve action potential decreased to 5 µV on the right side compared to 17 µV on the left side (). During somatosensory evoked potential study, we stimulated lateral femoral cutaneous nerves at 17 cm distal to the ASIS and used 1cm long cutaneous needle electrodes, inserting active electrodes at Cz' and reference electrodes at Fz in the international 10/20 system. While P37 latency was 30.5 msec and N45 latency 38.1 msec on the left side, no response was observed on the right side. On needle electromyography (EMG), abnormal spontaneous activities were observed at rest in right iliopsoas, rectus femoris, vastus medialis and vastus lateralis muscles, and reduced recruitment of motor unit action potentials was observed during muscle contraction. Other lower limb and paravertebral muscles were normal ().\nThrough electrodiagnostic study, we diagnosed the case as femoral neuropathy and accompanying meralgia paresthetica and performed pelvis MRI in order to identify the cause and locate the lesion. On pelvis MRI, a 9×5×4.5 cm large hematoma was found in the right iliacus muscle and was removed through suction drainage on day 23 after the onset of symptoms ().\nEighty-two days after the onset of symptoms, the results of manual muscle testing (MRC, Medical Research Council) were right hip flexion 4/5 and knee extension 3/5, showing gradual recovery, but while sensory test showed recovery by around 20%, paresthesia continued. On motor nerve conduction study, the amplitude of compound muscle action potentials for right femoral nerves increased to 2.0 mV, but on sensory nerve conduction study, the amplitude of sensory nerve action potentials for right lateral femoral cutaneous nerves did not noticeably change. On needle EMG, poly-phasic action potentials were observed in right iliopsoas, rectus femoris, vastus medialis and vastus lateralis muscles.\nThe results of manual muscle testing (MRC, Medical Research Council) performed 120 days after the onset of symptom at outpatient follow-up showed that muscle strength improved further to right hip flexion 4/5 and knee extension 4/5, and the results of sensory tests on that day were not different from those on day 82. Functionally, the patient was able to walk by himself both indoors and outdoors. |
In October 2012, a 46-year-old woman was referred to our center for the presence of a large mass (50 × 70 mm) in the superior lobe of the left lung with homolateral pleural effusion. The patient was never smoker, without family history of cancer and without comorbidity. The cytological diagnosis was made using a CT-guided fine needle aspiration of the primary tumor and revealed an adenocarcinoma of the lung (TTF1+, CK7+) with the EGFR ex19del mutation. A PET-CT demonstrated the presence of liver and bone metastases and a nodule in the right breast, confirmed as a metastasis by fine needle aspiration. The patient received zoledronic acid 4 mg every 28 days and gefitinib 250 mg daily since November 2012 obtaining a partial response (PR). In August 2013, a disease progression (PD) was documented, with an increase in size of the primary tumor and size and number of liver metastases. A brain MRI revealed the presence of two cortical nodules, which were treated with stereotactic radiotherapy. The patient was enrolled in the IMPRESS trial and received 6 cycles of cisplatin and pemetrexed plus gefitinib obtaining again a PR that lasted until June 2014. Thereafter, a new lung metastasis appeared in the superior lobe of the left lung and the mammary nodule increased in dimensions. From June 2014 to December 2014 the patient received erlotinib 150 mg daily obtaining an initial stabilization of the disease (SD); however, within 6 months, she experienced again a PD with the increase of the mammary nodule and the appearance of a new bone metastasis in the sacrum. In December 2014, EGFR ex19del and T790M mutations were detectable in a new needle biopsy of the primary tumor; only at this time a digital PCR-based method was available for the analysis of circulating tumor DNA (ctDNA). Briefly, the method was optimized in order to recover a suitable amount of ctDNA for molecular analysis from 3 ml of plasma using the QIAmp Circulating Nucleic Acid Kit (Qiagen®, Valencia, CA). ctDNA was examined using the Prime PCR Probe Assay on a QX100™ Droplet Digital™ PCR System (BioRad®, Hercules, CA) for EGFR mutations (ex19del, T790M, and C797S) []. The ctDNA sample was considered as EGFR mutant when at least one droplet was above the fluorescence intensity threshold of 3000 and results were reported as copies/ml. The first plasma specimen was obtained in December 2014 and confirmed the presence of ex19del and T790M mutations (480 and 260 copies/ml, respectively; Fig. ). The patient was treated with atezolizumab from March to May 2015 and received stereotactic radiotherapy on the lung primary tumor and on metastases of the left superior lobe, breast and bone (sacrum and D10). Due to PD, the patient was given afatinib 40 mg daily from June 2015 to September 2015, but she experienced a further PD with an increase in size of the primary tumor and lung nodules, the appearance of new multiple bilateral lung metastases, lymphangitic infiltration and liver metastases. At this time, the presence of ex19del and T790 M was again demonstrated in a biopsy of a liver metastasis. Since osimertinib was available, the patient was enrolled in the ASTRIS trial and given 80 mg daily starting from October 2015 with an immediate clinical benefit. At the first evaluation a PR was documented with disappearance of most of the lung nodules and lymphangitic infiltration, reduction of the primary tumor and of liver metastases as well. A disappearance of ex19del or T790M was demonstrated in ctDNA in a blood sample obtained in October 2016. However, in April 2017, ex19del appeared again (400 copies/ml) and in July 2017 it increased to 1000 copies/ml, while T790M was also detectable (330 copies/ml, Fig. ). Then, ex19del and T790 M continued to increase to, respectively, 1600 and 510 copies/ml in October 2017, 2100 and 550 copies/ml in November 2017, and 6900 and 1900 copies/ml in December 2017.\nA radiological progression was demonstrated with increase in size and number of liver metastases in December 2017. The patient underwent a new liver biopsy that confirmed the PD and the presence of ex19del and T790M, whereas the ctDNA showed also the appearance of C797S mutation (170 copies/ml), in addition to ex19del and T790M. The patient started chemotherapy with carboplatin and pemetrexed and in February 2018 she obtained an SD associated with a drop of ex19del (800 copies/ml), T790 M (380 copies/ml), and C797S (90 copies/ml) and then a PR in May 2018, with disappearance of C797S and reduction of ex19del (760 copies/ml) and T790M (90 copies/ml). In July 2018, however, ex19del strongly increased to 2200 copies/ml, even though T790M and C797S were undetectable. Finally, in October 2018, when a PD was documented, ex19del increased to 5100 copies/ml, while T790M and C797S appeared again in plasma with 600 and 180 copies/ml, respectively (Fig. ). At this time a NextSeq 550 NGS platform (Illumina®, San Diego, CA) was available to analyse ctDNA by the AVENIO ctDNA Expanded Kit (Roche®, Pleasanton, CA). A plasma sample collected in December 2018 and analysed by NGS confirmed the presence of the ex19del, T790M and C797S and found, in addition, EGFR and c-MET amplifications, which were not present in tissue in the last re-biopsy of December 2017. |
This case is of a 55-year-old female who initially presented with a palpable left neck mass. The mass had been noted by the patient 8 years ago and had progressively grown in size. A computed tomography (CT) of the neck was obtained, which demonstrated a 3.5 × 2.3 × 4.6 cm lesion deep to the left sternocleidomastoid (SCM) muscle in addition to a smaller 1 × 1 × 1.8 cm left posterior neck-enhancing mass. A fine needle aspiration (FNA) had been performed 2 years prior at an outside hospital with pathology indicating a low-grade spindle cell proliferation. She was thus referred to our institution for further evaluation. At the time of initial presentation to our group, the patient had noted dysphagia, left ear ache and tinnitus, and neck pain localized to the two masses. A positron emission tomography (PET) CT was performed, which demonstrated increased fluoro-2-deoxy-\nd\n-glucose uptake in the high cervical mass and, to a lesser degree, in the mass located within the posterior triangle of the neck (\n). The outside tissue blocks were reviewed at our institution and felt to be consistent with a peripheral nerve sheath tumor compatible with schwannoma from both lesions. Because of the patient's ongoing symptoms and because malignancy could not be completely excluded based on the results of the prior FNA, the decision was made to proceed with surgical excision of both lesions. Prior to proceeding, a magnetic resonance neurogram was obtained (\n). Tractography demonstrated that the two lesions appeared to originate from the spinal accessory nerve and that apparent diffusion coefficient values were elevated in both masses, supporting the diagnosis of a less aggressive tumor (\n).\nThe patient was taken to the operating room for surgical resection. The patient was positioned with her head turned slightly to the right with the neck extended (\n). The smaller lesion was approached first through the posterior triangle of the neck. Stimulation mapping of the tumor was conducted. The tumor was found to be located on the distal spinal accessory (cranial nerve XI) nerve, with evidence of trapezius activation with nerve stimulation (\n). After identifying no overlying nerve fibers, the tumor was removed en bloc. A separate incision was made in the upper cervical region to approach the larger second mass located lateral and deep to the SCM muscle, which was reflected medially (\n). During dissection of the tumor away from the nerve, motor evoked potentials (MEPs) to the trapezius were lost. As the tumor was of significant size, view of the proximal aspect of the afferent nerve was initially obstructed. Distally, the tumor was mapped, and the fascicle of origin was identified, which appeared to activate the SCM. After significant debulking of the mass, the proximal fascicle of origin was identified but did not provide any muscle activation after stimulation. The tumor was therefore removed in its entirety. All parameters for brachial plexus monitoring remained stable.\nPathology for both lesions was consistent with schwannoma without malignant features. Next-generation sequencing analyzing the coding regions of 479 cancer genes as well as select introns of 47 genes using the UCSF 500 Cancer Gene Test revealed a small in-frame insertion at codon p.R177 of the Sox 10 gene. There were no identifiable alterations in NF1, NF2, LZTR1, SMARCB1, and TRAF7 genes. Despite the change in MEPs, the patient was noted to be full strength in all muscle groups in the left upper extremity including shoulder shrug and head turning immediately postoperatively. At follow-up, her neck pain and prior dysphagia had improved significantly. |
The first case was a 59-year-old African American male with a past medical history notable for schizoaffective disorder, depression, and substance abuse who was brought in to the emergency room for disorganized behavior and agitation in the community. At the time of admission the patient demonstrated disorientation, repetitive motor behavior, and an alternation between agitation and psychomotor retardation. He had poor response to communication and tactile stimuli. A suspicion of altered mental status due to organic causes was suspected with the possibility of catatonic excitement and retardation. He was admitted to the medical floor, with a work-up revealing a positive toxicology screen for cocaine and opioids. The patients CBC and BMP were within normal limits except for his ammonia level which was 80 mg/dl. The patient was initially treated with Chlorpromazine Hcl 50 mg orally daily for his agitated behavior as well as Naltrexone 50 mg orally daily for his opiate intoxication.\nThe patient exhibited incoherent thought process in addition to mumbled speech that made a significant portion of his assessment evaluation difficult. During evaluation, he displayed abnormal movements of his arms and face, with tremors and restlessness. His affect was flat. He did not display any perceptual disturbances or delusions. An assessment for cognitive impairment was noncontributory during his most recent admission. The patient received Mirtazapine 45 mg orally at bedtime and Olanzapine 10 mg orally daily in his treatment and by day three of admission had shown improvement in his disorganized behavior with supportive care. The patient demonstrated more effort to directly communicate with house staff after treatment began.\nThe patient reported a past history of psychiatric illness that was late in onset. His first presentation at the age of 51 years was significant for depressed mood, paranoid delusions, and auditory hallucinations for which he was diagnosed with a major mood disorder. His symptoms responded poorly to medications including antidepressants. His disease course involved increasing periods of impulsive behavior and agitation. He became noncompliant with his prescribed medications. He was later admitted to the medical floors at the age of 54 years for “repetitive behavior” during which he was found moving from his bed to the bathroom repeatedly as if he wanted to use the bathroom all the time. He also showed some abnormal rocking movements during this time period. A medical work-up for seizure was negative. He was discharged with a presumptive diagnosis of a psychotic disorder. Thereafter, at the age of 56 years he had an episode of property destruction in the community and it was noted that he had “abnormal body movements” in addition to lability of mood. His diagnosis was revised to schizoaffective disorder and he was treated for mood lability at the time with risperidone.\nGiven the late onset of his neuropsychiatric symptoms, a computed tomography scan (CT) of his brain was done during his presentation, as seen in . Reviewing his chart, it was noted that the calcifications were apparent in his first head CT taken in January of 2012 with no changes to the current CT in January of 2019. |
A 25 years old female patient from semi-urban area reported with chief complaint of pain and swelling involving the right side of lower jaw for the last 1 year. The patient was aware of the lesion for the last 2 years. However, as the lesion was small and asymptomatic, she did not seek any treatment. The lesion gradually increased in size with localized intermittent pain for the last 1 year. Her past medical and family history was noncontributory. On extra oral examination, there was the presence of a diffuse swelling involving the lower right third of face extending from parasymphyseal to the angle of the mandible leading to mild facial asymmetry without any regional lymphadenopathy [].\nIntraoral examination revealed the presence of a diffuse swelling extending from 41 to 47 region covered by normal oral mucosa. On palpation, the lesion was bony hard, mildly tendered and associated with expansion of buccal and lingual cortical plates. 43 and 44 were absent along with over retained 84 []. Routine hematological and biochemical investigations were within normal limit.\nOrthopantomogram revealed the presence of multilocular, well-defined radiolucent lesion involving the right side of the whole of the body of the mandible extending from 34 to 48 region. Most striking feature was the presence of multiple radiopaque foci in the symphyseal and parasymphyseal region, radiodensity of which were similar to that of the normal bone. 43, 44 and 48 were impacted along with root resorption of 46 and 47. 84 was retained []. Computed tomography scan showed the presence of expansile bony lesion along with the presence of radiopaque foci involving the symphyseal and parasymphyseal region of the right side of the lower jaw []. Destruction of buccal cortical plate and thinning of lingual cortical plate were also noted.\nBased on the above clinical and radiological findings, the provisional diagnosis of fibro-osseous lesions and odontogenic neoplasms were made.\nEthical clearance of this study was received. Following this, incisional biopsy was performed from representative site of the lesion after obtaining informed consent from the patient, and the specimen was processed for histopathological examination.\nThe light microscopic features revealed the presence of scanty areas of polyhedral, neoplastic, odontogenic epithelial cells with prominent cellular outlines and intercellular bridges along with the presence of homogeneous, eosinophilic and amorphous materials at places [Figure and ]. The most characteristic feature is the presence of multiple rounded calcified areas, forming concentric “Liesegang ring-”like pattern in and around the epithelial cells and connective tissue []. Areas of osteoid tissue formation within the neoplasm were also noted []. The histological diagnosis was made as “CEOT.”\nTo confirm this diagnosis, immunohistochemical (IHC) evaluation was performed. IHC marker such as cytokeratin 19 confirmed the presence of odontogenic epithelium within the neoplasm []. Amyloid-like material after Congo red staining produces typical apple-green birefringence viewed under confocal microscope with polarized light [].\nAfter considering the clinical, radiological and histological features, confirmatory diagnosis of CEOT was made, and the patient was referred to the Department of Oral and Maxillofacial Surgery for further treatment and management. |
A 50-year-old female patient was referred to the Oral Medicine Clinic, Faculty of Dentistry, Universitas Indonesia, complaining of a painful lesion located on her upper right buccal mucosa, in addition to the palatal mucosa. She had experienced throbbing pain in the mucosal tissue around tooth 17 a few days earlier. She reported the self-application of a cotton roll that had been damped in propolis to relieve the pain in the mucosal area. The cotton roll was left in contact with the mucosa overnight. She noticed the eruption of a painful oral lesion the next morning, which caused difficulty in eating. The eruption of the lesion was not accompanied by any systemic symptoms, and no other body areas were involved. She also reported the daily consumption of propolis diluted in her drinking water. A review of her medical history revealed an allergic history to chloramphenicol and occasional gastric pain. Otherwise, the patient was healthy and was not under any medication. A clinical examination revealed multiple shallow and irregular ulcerations on the right buccal mucosa, the right hard palate mucosa, and the gingival area surrounding tooth 17. The size of the ulcers ranged from 0.3 to 1.5 cm in diameter (). Tooth 17 was in the middle of endodontic treatment for wide and deep caries lesion, and the radiograph showed no periapical lesion (). Palpation and percussion of tooth 17 were within normal limits. However, the patient considered having tooth 17 extracted. Furthermore, enlarged and painful submandibular lymph nodes were noted on palpation and were possibly the result of inflammation related to tooth 17. A working diagnosis of propolis-induced mucosal burns was made. The patient was advised to discontinue propolis use and was prescribed tetracycline mouthwash three times daily for 3 days and Doloneurobion twice daily for 7 days to manage the pain. She was advised to make a followup consultation after 5 days.\nOn the followup consultation, extra oral examination indicated a normal appearance of the affected area. Tooth 17 had been extracted by a different department, because the patient believed that it was the cause of the throbbing pain. Clinically, there was a healing extraction socket of tooth 17 and healing of the ulcerated area that was seen as the erythematous area. Pain related to the postulcerated area had mainly resolved; however, she reported persistent throbbing pain in the area where tooth 17 had been extracted. No submandibular lymphadenopathy was observed during this visit. Our department suspected trigeminal neuralgia as the cause of the throbbing pain. The patient was prescribed a gauze mucosal compress with 0.05% chlorhexidine gluconate three times daily for 3 days to heal the oral mucosa, in addition to 100 mg carbamazepine twice daily for 5 days. The patient was asked to return for a followup consultation in 5 days.\nOn the final consultation, the erythematous area was completely healed, and the rest of the mucosa appeared normal, with the socket of tooth 17 healing after extraction (). The patient reported no pain related in the postulcerated area. However, the throbbing pain in the area of tooth 17 was noted as a “funny feeling.” A 100 mg dose of carbamazepine was prescribed twice daily for 2 weeks. The ulcer was declared to be healed at this visit, and the patient was scheduled for a followup consultation 2 weeks later for the further evaluation of the suspected trigeminal neuralgia. |
A 55-year-old healthy woman was referred to our institution with a two-year history of progressive dysphagia to solids (). She reported a recent episode of solid food getting stuck in her throat, which prompted presentation to an outside endoscopist. The patient reported no alcohol use. She was a former smoker with a 15 pack-year history, but had quit over 20 years prior. The patient had a past medical history of gastroesophageal reflux disease, for which she was taking omeprazole, and hypothyroidism. She had no known history of any esophageal dysmotility disorder. There was a history of diabetes mellitus in her mother and son.\nPhysical exam and laboratory testing were unremarkable. Esophagography demonstrated a filling defect in the upper thoracic esophagus. Computed tomography (CT) demonstrated an 8 cm mass. Endoscopic ultrasound (EUS) demonstrated a pedunculated mass with a submucosal origin beginning at 20 cm from the incisors on the right side of the neck (). The lesion was felt to have the characteristic appearance of a FVP and the patient elected to proceed with resection.\nThe exploration began via a right cervical approach. The recurrent laryngeal nerve was identified and the cervical esophagus was mobilized. The mass was palpable on the posterior esophageal wall at the thoracic inlet. Upon a short myotomy, no stalk was identified and the mass could not be delivered to the neck. The cervical incision was closed and a right thoracotomy was performed. The mass was seen extending from the level of the azygos vein to the thoracic inlet. The esophageal muscular layer was intact. Following myotomy, the soft mass, which was densely adhered to the mucosa, was visualized and dissected from the underlying mucosa. It became evident that the mass maintained its attachment to a portion of the mucosa. Complete mobilization revealed the mass to be a lipoma at the tip of a large midesophageal diverticulum traveling in a submucosal plane. Repeat endoscopy demonstrated an ostium in the esophageal wall opening into a blind-ending pouch. The diverticulum was fully mobilized and resected using a stapler (). Mucosal closure was reinforced with overlying muscle and a pleural flap.\nThe patient was diagnosed with a large midesophageal diverticulum with a lead point lipoma. The patient’s postoperative course was uncomplicated. A postoperative esophagogram demonstrated no esophageal leak or obstruction. Pathology demonstrated a 7.5 cm diverticulum with a 4.5 cm lipoma without malignancy. At follow-up on the nineteenth postoperative day, the patient was tolerating a diet without dysphagia. |
A 52-year-old male patient referred to the Oral Medicine Clinic of Shiraz University of Medical Sciences complained of swelling and diffused pain in the right side mandibular teeth and recently paresthesia and dysesthesia of the right side of the lower lip. He reported the mass and expansion of his jaw occurred 1 year ago without any symptom, but diffused pain occurred 3 months ago. His medical history was significant for deafness and blood hypertension for which he was under treatment with a beta-blocker (atenolol).\nIn clinical examination, a mild diffused, painful and tender, bony hard swelling in the right side of the mandible that extended from the middle part of the mandible to the angle of mandible was observed. The surface color is normal and no sign of lymphadenopathy observed.\nIn intraoral evaluation, a tender swelling exhibited smooth surface and indistinct border soft mass extended at the right mandibular side from the first premolar to the second molar, caused displacement and mobility of first and second molars. Pus formation around the first molar occurred. Palpation of mass clarified expansion and thinning of buccal and lingual cortexes []. Multiple decayed teeth, calculus and stain were observed on this side. A small amount of blood was obtained in aspiration from the anterior border of the lesion, but in aspiration from the posterior part of the lesion, nothing was found.\nA plain panoramic radiography revealed an approximately 7 cm × 3.5 cm, ill-defined, multinodular radiolucent lesion extending from the apical and distal root of the third molar to the mesial side of the apex of the canine tooth in the right lingual side, with mild root resorption. The anterior aspect of the lesion showed soap bubble appearance. The inferior alveolar canal was eroded and discontinued []. Conventional occlusal view showed that buccal cortical perforation occurred []. Further investigation with a cone-beam computed tomography revealed radiolucency in the right mandibular area resorbing the buccal cortex, alveolar bone and the root of lateral root 6–8 representing an invasive process. A minimal and fine intralesional calcification was reported. A differential diagnosis consisted of uncommon odontogenic myxoma (OM), ameloblastoma and central giant cell granuloma was made by clinicopathologic correlation.\nAn incisional biopsy was performed under local anesthesia with a partial flap. The semi-hard gelatinous tissue was removed and excised. The histopathologic evaluation presented tissue consisted of a fragment of fibro collagenous connective tissue with many myxoid areas and infective area and pus accumulation observed. Residual bony trabeculae were seen and very small-sized trabeculae of mineralized tissue were occupied the mainly myxoid areas [].\nA surgical management involved a segmental resection under general anesthesia and a metal plate was inset to the defected area. The inferior alveolar nerve was preserved. The patient has been under regular follow-up and rehabilitation. A surgical defect has been healing gradually and no evidence of recurrence has been observed on the latest radiograph obtained 12 months after surgery. |
A 15-year-old male presented to our outpatient department with complaints of multiple bony deformities, short stature, and difficulty in walking since childhood. His parents told that at the time of birth, there was a shiny adherent membrane all around his body which shed off over 2 weeks revealing reddish pink skin following which a frequent cycle of scaling and shedding of the skin occurred. Parents also noticed the development of multiple deformities involving all extremities and spine as he began to walk which worsened as the child grew. He was unable to walk long distances, play with friends and had to drop out of school due to the nature of deformities and poor cosmesis. The child was using skin creams and medications intermittently for skin condition. He has one sibling who does not have such complaints. He had not received any orthopedic management for correction of his skeletal deformities.\nOn physical examination, the child had a height of hundred and 22 cm and weight of 24 kg, both these parameters were below the third percentile of the Indian Academy of Pediatrics chart for height and weight for boys. Cognitive and speech functions were intact.\nExamination revealed brown scales all over the body, including the scalp, flexural surfaces, and external auditory meatus. The scales were large in size and odorless, adherent to the skin. Thin hair was noted on the scalp with entangled fine scales. Abnormal dentition and missing teeth were also noted. There was fixed kyphoscoliosis with convexity to the right side. The right forearm showed bowing and the right upper limb appeared “S” shaped. Pronation and supination were severely restricted on the right side due to old malunited fracture in the forearm. There was anterior bowing of the right femur and lateral bowing of the left femur as well as anterolateral bowing of bilateral tibia and lateral dislocation of the right patella (). Plain radiographs showed multiple bony deformities in the lower limbs (). Skin biopsy confirmed the diagnosis of lamellar ichthyosis. Oral Vitamin D supplementation was started at 5000IU daily dose along with oral calcium supplementation [], oral tretinoin and topical emollients were given for skin disease.\nOnce biochemical parameters were normalized, he was planned for a staged correction of all lower limb deformities to prevent postoperative infection due to poor skin condition. We first performed a right-sided distal tibia corrective osteotomy. As there were no immediate signs of surgical site infection with good healing of surgical wound, after 1 week a right-sided supracondylar femoral osteotomy was done. Following this procedure, the patella spontaneously relocated. A third osteotomy procedure was performed after 3 weeks for correction of the left distal tibia. We followed standard pre and post-operative protocol for all procedures. All surgical sites healed without any complications and at 6 weeks follow-up assisted walking was started as the osteotomy sites were uniting on radiograph ().\nWe continued Vitamin D supplementation on a monthly basis. At 18 months follow-up, we observed improved gait mechanics, decreased pain and fatigue while mobilizing. His scaling and cosmetic appearance also improved and biochemical parameters were within normal limits (). |
A 38-year-old male with a history of an open right pyeloplasty in 1997 for a right UPJ obstruction presented 18 years later with intermittent right flank pain that he had been having since the initial operation as well as hematuria. His urine culture was positive for Klebsiella pneumonia and he was treated for pyelonephritis. A CT of the abdomen without contrast at that time showed moderate right intrarenal caliectasis with a dilated renal pelvis. Multiple surgical clips were seen at the right UPJ. There were no calculi seen (). A renal scan showed 50% function from both the right and left kidneys. The right kidney had both normal blood flow and cortical uptake with an adequate excretion. There was significant tracer retention on the right side that responded appropriately to Lasix administration.\nHe elected to undergo a cystoscopy and right ureteroscopy to evaluate a new or persistent UPJ obstruction. The flexible ureteroscope was able to be advanced into the dilated right renal pelvis. The ureteroscope was withdrawn and an encrusted metal surgical clip was seen at the UPJ that was embedded in the wall of the ureter. The encrustation was lasered with a holmium laser fiber and the stone fragments and clip were retrieved with a basket (). No other foreign bodies were found either in the kidney or in the ureter. A ureteral stent was placed and removed uneventfully several days later.\nHis symptoms completely resolved for 5 months after the procedure, until he had acute onset hematuria, dysuria, and right flank pain—which were the same symptoms he had before the clip removal. A repeat ureteroscopy was recommended to evaluate for additional eroded foreign bodies. Ureteroscopy revealed suture material eroding through the lumen of the ureter just distal to the UPJ. A basket was used to pull as much of the suture as possible into the lumen. Next, a 272 μm holmium laser fiber was used at 0.2 J and 10 Hz to sever the intraluminal suture. This fragment was then grasped and removed with a basket (). There was no exposed suture remaining. Gross inspection of the specimen revealed a 1 cm piece of suture material. He remains asymptomatic at follow-up at 6 months. |
A 63-year-old female with newly diagnosed atrial fibrillation with rapid ventricular response (RVR), history of type 2 diabetes, hypertension, and deep venous thrombosis six years ago presented to the emergency department with paresthesia of the left lower extremity. She had been feeling sick over the last few days with a sore throat, cough, and shortness of breath. She denied any chest pain.\nPhysical examination revealed absence of pulses in the left lower extremity and coolness to the touch. Initial laboratory workup in the emergency department was performed, and significant findings are illustrated in table . Initial differential diagnosis is broad and includes acute COVID-19 pneumonia, acute diabetic ketoacidosis, acute atrial fibrillation with RVR, and acute limb ischemia.\nA chest radiograph demonstrated asymmetric haziness in the right lower lateral lung field. A Computed Tomography (CT) scan of the thorax was obtained and showed bilateral pulmonary ground glass opacities and basilar atelectasis, but demonstrated no evidence of pulmonary embolism. Next, a CT of the abdomen was obtained and showed a 1.7 x 1.0 cm filling defect superior to the celiac artery in the abdominal aorta as well as a large occlusive filling thrombus defect just inferior to the renal artery to the level of the common femoral artery bilaterally (Figure ). The aortic thrombus was also appreciated under fluoroscopy as illustrated in (Figure ).\nIn this case, the patient was deemed a poor surgical candidate due to disease severity and comorbid conditions. Therefore, a treatment approach via thrombolysis was elected over traditional surgical bypass or thrombectomy.\nBilateral percutaneous radial artery access was obtained because the common femoral arteries were nearly completely occluded with thrombus. This approach was unique because access is traditionally gained via the femoral arteries. Angiography of the abdominal aorta demonstrated patent suprarenal segment with good perfusion to the patient's bilateral renal arteries. However, complete thrombotic occlusion below the level of the renal arteries was present. No flow was visualized into the pelvic arteries. Selective angiography of the bilateral external iliac arteries demonstrated stasis of flow. Bilateral transradial EKOS thrombolysis catheters were placed within the aorta, extending into the lower extremities (Figure ). The infusion side-holes were positioned to span the aortoiliac thrombus. Heparin and tissue plasminogen activator were initiated for thrombolysis.\nThe patient expired hours later from cardiopulmonary arrest, likely from severe COVID-19 infection and multiorgan dysfunction but also exacerbated by reperfusion injury. It is unknown whether or not the patient was vaccinated against COVID-19. |
A 35-year-old left-handed man who works as a mechanic and firefighter presented to our institution with hand pain and cold intolerance for 3 months. He was referred by a local vascular surgeon who had made the diagnosis of hypothenar hammer syndrome (HHS) following an angiogram of the right upper extremity showing complete occlusion of the ulnar artery (). On initial examination, Doppler signals of the ulnar artery, superficial palmar arch, and ring/small digital arteries were absent in the right hand. Subsequently, the patient developed an ulcer on his right long fingertip after minor trauma that had not healed over a 4-week period. He suffered ischemic hand pain and severe cold intolerance. After conservative management failed, the decision was made for operative intervention. We proposed using an arterial graft versus a venous graft in order to improve long-term patency. Our plan was to utilize the descending branch of the lateral circumflex femoral artery (DLCFA) as an arterial graft.\nIntraoperatively, Doppler ultrasound revealed a segmental thrombotic defect extending from the superficial palmar arch, across the Guyon canal, extending 10 cm proximally in the forearm. The ulnar artery was resected back to healthy bleeding pulsatile flow, resulting in a segmental defect of 12 cm. Final pathology of the submitted ulnar artery segment revealed organizing thromboembolus in the lumen, which at the time of resection was adherent to the lumen.\nThe DLCFA was harvested, with care taken to prevent injury to the lateral femoral cutaneous nerve. A 12-cm segment of the artery was circumferentially dissected, as well as approximately 2 cm of 2 branching vessels at the distal end of the segment that would be used to reconstruct the superficial palmar arch and common digital arteries with one graft. With the arterial graft completely dissected, it was ligated both distally and proximally.\nThe arterial graft and recipient vessels were examined under the operative microscope. The proximal anastomosis was preformed first. The two branches at the distal end of the arterial graft were anastomosed to the superficial palmar arch stump, and common digital artery to the ring and small finger, respectively. A bolus of 5000 units of heparin was given intravenously before release of the microvascular clamps. Adequate pulsatile flow was achieved though the graft and confirmed with Doppler ultrasound to each finger ( and , Video 1). The hand was then irrigated, and the ulnar nerve was explored to ensure that no injury had taken place. The skin was closed, and the patient's hand was placed into a dorsal splint.\nThe patient remained in the hospital overnight for monitoring and was discharged the following morning on oral aspirin as the only anticoagulant. On follow-up examination 2 days later, strong Doppler signals were elicited at the ulnar artery, palmar arch, and radial and ulnar aspect of each digit. At 1 month, Doppler signals remained strong and the ulcer previously present on the right long fingertip had completely resolved (, Video 2). During this visit he reported to be asymptomatic and has had complete resolution of the cold intolerance and burning pain of his right hand. |
The patient was a 41-year-old female with past medical history of appendectomy and dysmenorrhea. She presented to the emergency department with nausea, severe vomiting, and acute pain in the lower abdomen. She reported having experienced asthenia and weight loss for one month. On clinical examination, abdominal distension and tenderness were discovered. Blood tests revealed leukocytosis with neutrophilia, and a contrast-enhanced abdominal computed tomography (CT) showed a 7 × 7 × 4 cm hyperenhanced mass in the cecum that caused complete bowel obstruction (). Also, a 5 × 3 × 3 cm right adnexal mass that compromised the ovary with intimate contact with the uterus was found (). Furthermore, the CT showed dilated loops in the small bowel (>4 cm), some of which had an enlarged wall thickness and presence of intraluminal fluid stasis ().\nWith these findings, particularly the observation of a mass through the CT scan, and due to the evident weight loss that the patient had undergone, neoplasia could not be ruled out. Surgery was decided, and at laparotomy, a volume of 200 ml of inflammatory fluid was found in the cavity. Most of the loops of the distal ileum were dilated, and a 7 × 7 × 3 cm cecum mass was discovered, which compromised the ileocecal valve and caused complete bowel obstruction. Surgical decision was straightforward, the cecum mass was completely resected, and a right hemicolectomy was executed. An ileocolic anastomosis was also performed during the procedure. Furthermore, the right adnexal mass that was previously identified through the CT scan (measuring 4 × 3 × 2 cm) was observed to be firmly attached to the ovary and the fimbriae and displayed a pale external capsule surrounded by a cystic component. Gynecology consultation was required, and due to the size of the mass and its characteristics, surgical removal of the right adnexal mass was performed. After completion, closure of the abdominal wall was performed, and the remainder of the procedure continued without any complications.\nPathology revealed a 4 × 3 × 2.5 cm blueish heterogenic mass that occluded 90% of the lumen of the cecum and the ileocecal valve. Microscopy revealed that the colon wall was invaded by glands and endometrial stroma. The colonic epithelium showed inflammatory changes and was negative for malignancy (Figures and ). In the ovarian parenchyma, an endometrial cyst was discovered, covered with siderophages. Glands and endometrial stroma were observed in the fallopian tube as well ().\nThe postoperative course of the patient was uneventful. She initiated clear liquids a day after surgery and was discharged once full diet was resumed. On follow-up controls, the patient was completely asymptomatic, without any pain or complications. |
We present the medical history of a boy, currently 13 years old, who has been hospitalised multiple times in different medical centres due to repeated episodes of fever and respiratory infections since he was three years old. The first hospitalisation was associated with a severe bacterial infection: meningitis, pneumonia, and pericarditis. Several subsequent hospitalisations were due to fever of unknown origin. When the patient was seven years old, the symptoms exacerbated in the form of persistent fever and weakness, which resulted in another hospitalisation. The boy presented with anaemia that required transfusion of erythrocyte concentrate – physical examination revealed liver and spleen enlargement; laboratory tests revealed raised inflammatory markers, hypergammaglobulinaemia and elevated concentrations of IgG 2978 mg/dl; the IgG4 concentration was not assessed. The fact that the patient did not respond adequately to empirical wide-spectrum antibiotic therapy raised suspicion of an autoimmune disorder. He received glucocorticoid therapy ordered in another hospital, which resulted in the resolution of fever and normalisation of inflammatory markers. The glucocorticoid treatment was continued for six weeks. Four months after the treatment he started complaining about headaches, and left exophthalmos was observed on clinical examination. MRI revealed a mass around the posterior wall of the left orbit, involving the lateral rectus muscle, sphenoid wing, and masseters (). The image obtained from the test did not indicate a malignant tumour or demyelinating background. Available medical records do not confirm that other imaging examinations were performed. Unfortunately, histopathological examination of endoscopically collected biopsy specimens was non-diagnostic and suggests pseudotumour. The patient received glucocorticoid therapy again, which had been continued for four months. Control head MRI scan after the treatment showed progression of the tumour, which also involved the left pterygopalatine fossa. Earlier available medical documentation does not provide more detail about the diagnosis and treatment at that time.\nAt this point of time, nine years since initial manifestation of the disease, the patient was admitted to the Paediatric, Haematology, Oncology, and Rheumatology Unit of the District Paediatric Hospital in Bydgoszcz.\nThe boy underwent tumour surgery: first it included an open surgical biopsy of the tumour of pterygopalatine fossa, while histopathological examination of the specimens showed the characteristics of chronic inflammation. The result of histopathological examination did not allow determination of the aetiology of the inflammation. Five months later, enlarged lymph nodes were found on the neck and in the left supraclavicular fossa. The patient was qualified for another surgery, and some enlarged lymph nodes were collected for microscopic examination during the procedure. Histopathological analysis revealed reactive follicular hyperplasia; sinus histiocytosis of lymph nodes, and excess fibrous connective tissue, with signs of fibrosis and chronic diffuse infiltration of connective and adipose tissue; the pathologist suggested abnormal fibrosis IgG4 (+). Laboratory tests showed significantly elevated serum concentration of IgG 2528 mg/dl (N: 750–1650 mg/dl), with an increase in IgG4 fraction – 350 mg/dl (N: 4–230 mg/dl).\nBased on these findings and the results of the histopathological examination of lymph node biopsy specimens, the patient was eventually diagnosed with possible IgG4-RD.\nHe received prednisone 0.6 mg/kg, which has been continued for three months. Then prednisone dose was slowly tapered to 5 mg. The boy did not tolerate the treatment well; although the fever resolved, he complained about malaise and lower extremity pain.\nAfter one week of treatment with 5 mg prednisone, the patient again presented with fever, bone and joint pain, and elevated inflammatory markers. Hence, the prednisone dose has been maintained at 10 mg/day which resulted in adequate control of the symptoms and stabilisation of serum IgG4 within the normal range. Long-term course, dependence on corticosteroid therapy, lack of histopathological features of malignancy (pathologist suggested IgG4-RD), as well as complete regression of infiltrates in the pterygoid-palatal fossa and lymph nodes confirms our diagnosis of probable IgG4-RD according to Boston criteria and possible diagnosis based on criteria from 2012 [, ]. |
Our case was a 65-year-old right handed female who started having abnormal limb movements several years ago for which she was referred to our Movement Disorders Center. The patient had a history of generalized seizures with intermittent loss of consciousness since the age of 44 lasting several minutes.\nThe abnormal movements involved her left hand and left foot which would occur intermittently when she was awake and disappear when she was asleep. On examination the patient had irregular, involuntary, brief, fleeting, and unpredictable movements of her left upper and lower extremities (hand, fingers and foot) consistent with chorea (see supplementary video). Examination of her gait revealed that she would hold her left arm in dystonic posturing while walking. The patient would smile and laugh at times without any reason due to frontal disinhibition. The rest of her neurological examination including cognitive functioning was unremarkable. The patient's family history was negative for any neurological conditions including Huntington's disease, chorea and other movement disorders. The patient's physical examination showed that she had clinical features of two movement disorders, in particular chorea and dystonia.\nThe diagnosis of neurological diseases and movement disorders are based on good clinical history and examination. The patient in our case started having abnormal limb movements several years ago, though she was not able to recall the exact starting point. There were no other complains. In terms of the evolution of symptoms there was a very negligible progression of the symptoms of chorea and dystonia in her left hand over the years along with the size of the meningioma.\nAn MRI of the brain showed a hyperintense irregular lobulated mass of 3.7 × 3.5 × 3.2 cm in the right frontal lobe with minimal leftward midline shift involving the inferior aspect of the left frontal lobe with inhomogeneous enhancement [Figures and ]. This was consistent with a heavily calcified frontal lobe meningioma. There was no edema or mass effect in the surrounding brain. Her EEG was normal.\nExtensive serological investigations for causes of chorea and dystonia were normal. Furthermore, CBC electrolytes, ESR, ANA, rheumatoid factor, thyroid profile, and antiphospholipid antibodies were normal. |
The patient was a 38-year-old nulliparous woman who suffered from infertility of unknown origin. She had been treated with assisted reproductive technologies including artificial insemination and in vitro fertilization for over four years. During her treatment for infertility, cytological review followed by colposcopic biopsy revealed an invasive nonkeratinizing squamous cell carcinoma (SCC). A 1 cm mass was identified in the uterine cervix, but a pelvic MRI did not describe the cervical mass or parametrial invasion. Additionally, a submucosal leiomyoma of 15 mm in diameter was found in the uterus (). CT scans showed no signs of lymph node swelling or distant metastases. Based on these findings, she was diagnosed with stage IB1 cervical squamous cell carcinoma. We offered radical hysterectomy and pelvic lymphadenectomy as standard treatment although she strongly desired fertility preservation. The submucosal leiomyoma may have been the cause of her infertility, and she was keen to resect the myoma during the same procedure. Submucosal leiomyomas can usually be resected with hysteroscopy but was not advised in this case from the oncological viewpoint. As such, we obtained informed consent and performed an abdominal radical trachelectomy followed by abdominal myomectomy.\nDuring the surgery, we first drained the ascites in the pelvic cavity, resected bilateral pelvic lymph nodes, and sent them for intraoperative pathology. They were reported to be negative. The paravesical and pararectal spaces were then developed. The ureters on either side were resected to their insertion into the bladder. The uterine arteries were ligated and cut at the origin where they branched from the internal iliac arteries. Next, the uterosacral ligaments were divided. A colpotomy was performed circumferentially, and the cervical specimen was excised together with the parametrium at least 2 cm below the internal os. During the surgery, a frozen section procedure was performed for histology. The patient was found to have a 5 mm free cervical margin. A permanent cerclage was placed at the level of the isthmus. The uterus was then reanastomosed to the vagina. We then performed resection of the submucosal myoma via a uterine vertical incision. An intrauterine device (FD-1; Fuji Latex Co., Tokyo, Japan) was placed in the uterine cavity. The operation duration was 339 min, and blood loss was 500 ml. The surgery was completed with no complications.\nThe final histological specimen confirmed the diagnosis of squamous cell carcinoma, keratinizing type of cervix uteri, pT1B1. Exocervical, endocervical, and deep margin regions were negative. There was no metastatic lesion in the lymph nodes or lymphovascular space invasion. Leiomyoma of the corpus uteri showed no malignancy. No adjuvant treatment was administered, and no recurrence has been reported for at least 18 months postoperatively.\nSix months after the surgery, she became pregnant following the postoperative first embryo transfer. The fetus was appropriate for gestational age. At 21 weeks of pregnancy, she claimed vaginal bleeding, and her lower uterine segment lengths were shortened from 23 mm to 13 mm. She was diagnosed with threatened abortion, and tocolysis was started. At 25 weeks, preterm premature rupture of membranes occurred. She received antibiotics, and intramuscular betamethasone was administered. At 26 weeks, a male baby weighing 980 g was delivered with an Apgar score 3/5/7 by caesarean section due to chorioamnionitis. The baby received general care in a neonatal intensive care unit for four months and weighed 4520 g when discharged. He is now 6 months old and is well. There has been no recurrent disease of her cervical cancer for 18 months from the trachelectomy and myomectomy. |
Our patient was an obese 54-year-old female who presented to the emergency department with a chief complaint of shortness of breath. She presented with a blood pressure of 176/80 mmHg, a pulse of 115 bpm, and a respiratory rate of 40 bpm. Labs on initial presentation showed a blood glucose of 653 mg/dL, leukocytosis of 17.5 K/μL, and an elevated lactic acid of 2.5 mmol/L. She had no previous diagnosis of diabetes mellitus. She was transferred to the ICU for management of diabetic ketoacidosis. After the patient was stabilized in the ICU, she started complaining of left abdominal pain. Physical exam showed a poorly defined erythema with underlying crepitus and a central stellate-shaped violaceous patch on the left flank and abdomen that was concerning for impending necrosis as shown in Figure .\nAs shown in Figure , the CT of the abdomen and pelvis showed a large amount of subcutaneous emphysema within the left flank soft tissues that wrapped anteriorly along the left lower lateral abdominal wall into the anterior subcutaneous soft tissues of the left lower quadrant. There was also a small tract of air extending from the left retroperitoneum posterior to the left kidney and extending along the left iliopsoas muscle inferiorly. Mild soft tissue stranding and thickening adjacent to the proximal sigmoid colon in the region of the subcutaneous air was present as well.\nAs a result of these findings, the patient was immediately taken to the operating room for management of a necrotizing soft tissue infection of the left hemi abdomen and left flank. Debridement of skin, soft tissue, fascia, and muscle was performed as shown in Figure .\nAfter further review of the initial CT on the following day, there was suspicion that there was a perforation secondary to a colonic malignancy as evidenced by soft tissue thickening along the wall of the sigmoid colon. Thus, a CT of the abdomen and pelvis with rectal contrast was ordered which showed intraabdominal abscesses and concerns for a tumor within the sigmoid to the descending colon, as seen in Figure . Further debridement was continued with the drainage of the intraabdominal abscesses along with a takedown of the splenic flexure, and an open left hemicolectomy with the creation of an end colostomy. During the operation, a perforation of the colon was identified.\nTwo days later, a final debridement was completed with the placement of a wound VAC.\nBlood and specimen cultures grew Streptococcus anginosus (S. anginosus)and final pathology of the specimen demonstrated well-differentiated colonic adenocarcinoma measuring 6.5 cm in length and 1.1 cm in thickness, invading into the muscularis propria but not into the pericolonic fat or serosa. All fourteen lymph nodes were negative for malignancy, stage pT2N0M0 with all resection margins free of tumor. The surgical specimen is shown in Figure .\nPost-operatively, the patient was managed with broad-spectrum antibiotics and diabetes management with subcutaneous insulin. She was also followed up for surgical wound care and continuous antibiotics at a long-term acute care facility for two months. Follow-up wound healing at 2.5 months can be seen below in Figure . |
A 57 -year-old female presented with incidental ultrasonic evidence of left upper pole renal mass in Nov. 2001. Further evaluation with abdominal Magnetic Resonance Imaging revealed a mass in the upper pole of the left kidney with radiologic characteristics of renal cell carcinoma (). Left radical nephrectomy was performed sparing the left adrenal gland. The pathology specimen analysis showed a cystic mass 3 × 3 × 5 cm with yellowish friable tissue. Sections showed malignant epithelial cells, arranged in sheets. The picture was consistent with renal cell carcinoma, Grade II Fuhrman nuclear characteristics, confined to the capsule, neither pelvicalyceal nor vascular invasion was found (pT1bN0M0). Postoperatively she did not receive immunotherapy or chemotherapy. Apart from her hypertension which was well controlled with amlodipine and valsartan, subsequent clinical and radiological follow up showed no local or metastatic recurrence till 5 years after the operation then she stopped her visits. On October 2016 an incidental mass was found in the right adrenal gland during a checkup visit for the status of her right solitary kidney. Abdominal and pelvic computed tomography scan was done, revealing a well-defined mass with a smooth outline in the right adrenal gland measuring 54 × 48 × 39 mm with a central necrosis. The density of the solid component was 38 HU. In dynamic study the solid component showed significant enhancement after intravenous contrast administration (). Thorough hematological, biochemical and hormonal investigations were performed; all were within normal range. The results of laboratory examination showed the adrenal mass to be nonfunctional. The condition was well clarified for the patient and consent was taken to do right adrenalectomy. Under general anesthesia, in left lateral position through right transcostal incision, right adrenalectomy was done (). No any perioperative complications were recorded and she was discharged home on 4th post-operative day. Pathological examination revealed morphological and immunohistochemical findings in line with metastatic renal cell carcinoma, including positive staining for AE1/AE3, cytokeratin 7, vimentin, and CD10, and negative staining for CDX-2, inhibin, and synaptophysin (, ). During the last 2 years she has being on regular follow up. Whole body Positron Emission Tomography-Computed Tomography with fluorodeoxyglucose was performed, neither local nor metastatic recurrence was observed in any system. |
A 40-year-old woman (160 cm, 70 kg) at 30 weeks and 4 days of gestation was scheduled for cesarean section and pituitary tumor removal. Her past medical history revealed that she had undergone cesarean section under general anesthesia 10 years ago and her other past medical history was unremarkable. The patient was hospitalized for progressive bitemporal hemianopsia at 23 weeks of gestation. She was diagnosed with pituitary tumor extending into the suprasellar region and the tumor size was 26 × 21 × 21 mm. At that time, because of mild neurologic symptoms, low fetal weight, fetal lung immaturity and the possibility of premature birth, the initial decision was to delay the surgery of the pituitary tumor until after delivery at full term. At 30 weeks of gestation, however, she complained of rapidly deteriorating vision and bitemporal hemianopsia in both eyes and the ensuing radiological examination revealed increased tumor size (28 × 22 × 22 mm), displaced tumor location and compressed optic chiasm. Hence, she was scheduled for cesarean section and pituitary tumor resection due to the risk of irreversible blindness. Hormone tests revealed that prolactin, estrogen and progesterone levels were elevated, but these levels were within their normal ranges since she was pregnant. Other blood and biochemical tests were within their normal ranges. And the results of the chest radiography and electrocardiography were also within normal limits. Surgery was scheduled at 31 weeks and 3 days of gestation and neonatal intensive care unit was prepared to render specialized care to the newborn infant.\nThe patient was premedicated with ranitidine 50 mg and metoclopramide 10 mg intravenously. A left lateral tilt of about 15° was accomplished to prevent aortocaval compression when the patient arrived in the operating room. 100% oxygen was administered via a face mask and monitoring of noninvasive blood pressure, electrocardiogram, and pulse oxymetry was started. It showed blood pressure 123/72 mmHg, heart rate 85 beats/min and 100% oxygen saturation. Also, a 20-gauge catheter was inserted into her left radial artery for continuous blood pressure measurement and blood sampling. The initial arterial blood pressure was 132/60 mmHg. Anesthesia was induced by rapid sequence intubation using precurarization technique. First, pretreatment with vecuronium 0.7 mg was started intravenously. After 3 minutes, thiopental 300 mg and succinylcholine 100 mg were administered intravenously to facilitate endotracheal intubation. And then, vecuronium 8 mg was injected intravenously. Immediately after intubation, blood pressure and heart rate were 136/72 mmHg and 102 beats/min, respectively. Anesthesia was maintained with 0.8-1.2 vol% isoflurane, 2 L/min N2O and 2 L/min O2. Controlled ventilation was maintained at tidal volume of 8 ml/kg and respiratory rate of 10-13 breaths/min. The end tidal CO2 was measured. After 5 minutes from the time of induction, arterial blood gases revealed pH 7.35, PaCO2 33 mmHg, PaO2 110 mmHg, HCO3- 20.4 mEq/L, base excess -4.7 mEq/L, Hb 10.4 g/dl, hematocrit 31%. FiO2 was set at 0.6 to elevate PaO2. After 7 minutes from the time of induction, she delivered a male infant. The neonatal body weight was 2,740 g, one-minute Apgar score was four, and five-minute Apgar score was seven. The infant was then transferred to the nursery. Thereafter, midazolam 2 mg and fentanyl 100 µg was administered to maintain anesthetic depth. After placental extraction, 100 ml normal saline containing 20 units of oxytocin was infused and then, 1,000 ml normal saline containing 10 units of oxytocin was infused slowly to induce uterine contractions throughout the duration of both the obstetric surgery and neurosurgery. Intraoperative hemodynamic and respiratory variables were stable throughout the duration of cesarean section with systolic blood pressure between 100 to 110 mmHg, heart rate 70 to 80 beats/min. Cesarean section lasted for 50 minutes after induction. Arterial blood gases revealed PaCO2 32 mmHg, PaO2 152 mmHg, HCO3- 20.1 mEq/L, base excess -5.6 mEq/L, Hb 8.4g/dl, hematocrit 26%. Initially, fluid replacement was done against the intraoperative hemorrhage, but later 1 unit of packed red blood cells was transfused due to continuous bleeding. The estimated blood loss and urine output until the end of cesarean section were 800 ml and 60 ml respectively. 700 ml normal saline and 500 ml 6% hydroxyethyl starch (Voluven®, Fresenius Kabi, Germany) were administered during cesarean section.\nCentral venous catheter was inserted into the right subclavian vein for central venous pressure measurement, massive transfusion and fluid replacement before pituitary tumor resection. Otolaryngologist performed the transsphenoidal approach and then, a neurosurgeon resected the pituitary tumor. Anesthesia was maintained with 0.8-1.2 vol% isoflurane, 2 L/min N2O, 2 L/min O2. At the beginning of pituitary tumor resection, arterial blood gases revealed PaCO2 30 mmHg, PaO2 203 mmHg, HCO3- 18.0 mEq/L, base excess -7.4 mEq/L, Hb 9.6g/dl, hematocrit 29%. During the surgery, arterial blood gases revealed pH 7.35, PaCO2 36 mmHg, PaO2 242 mmHg, HCO3- 19.0 mEq/L, base excess -7.5 mEq/L, Hb 10.1 g/dl, hematocrit 30%. Hemodynamic variables were well maintained with systolic blood pressure between 90 to 110 mmHg, heart rate 70 to 90 beats/min. Cesarean section and brain surgery together lasted for 8 hours after induction. Pituitary tumor located in pituitary fossa was almost completely resected and was diagnosed as pituitary adenoma by histological study. Tumor extending into the suprasellar region was not resected via transsphenoidal surgery and was planned to be removed via craniotomy. The estimated blood loss and urine output during pituitary tumor resection were 200 ml and 990 ml respectively. 2,600 ml normal saline and 500 ml 6% hydroxyethyl starch (Voluven®, Fresenius Kabi, Germany) were administered. At the end of the surgery, cerebrospinal fluid drainage catheter was inserted at the L3-4 interspace and the neuromuscular blockade was reversed with 0.4 mg glycopyrrolate, and 15 mg pyridostigmine intravenously. The patient was extubated fully awake in the operating room and was transferred to the intensive care unit uneventfully. After surgery, the patient was given thyroid hormone and steroids for panhypopituitarism. The patient presented with transient diabetes insipidus, but recovered immediately. On the 4th day after surgery, she was transferred to the general ward. Bitemporal hemianopsia and visual loss mildly improved and she was discharged on the 14th day after surgery. The neonate was healthy and was discharged uneventfully on the 2nd day after delivery. |
The case concerns a 16-year-old female (referred to as Bella). On admission to the inpatient treatment service Bella was assessed by the consultant psychiatrist. She met DSM-IV diagnostic criteria for AN (restricting type). She exhibited no comorbid depressive or obsessive compulsive disorders and was not taking any prescribed medication. When she participated in the present study, her weight was 52.2 kg with a BMI of 17.0 kg/m2. Bella was undergoing a medium to long stay programme at the service, the duration of which is usually between four to nine months. It has been suggested that undertaking a saliva sampling protocol too close to admission may not be favourable because the participant may not have yet adjusted to their new environment, and the stress of the hospital environment may affect cortisol levels [], which is why Bella did not take part in the study immediately at admission. She was followed up for this research one year after being discharged from inpatient treatment.\nThe inpatient treatment service offers a “therapeutic milieu” which includes highly skilled nursing care and tailored therapeutic approaches for each young person. The centre's philosophy was one that understood AN to be the expression of major difficulties in self-regulation with consequent effects on emotional communication and on physical health.\nBella followed a comprehensive routine and treatment programme, which, in addition to milieu therapy, included weekly individual psychotherapy, family therapy, and group therapy. Problems identifying emotions in oneself and others and handling relationships were targeted within individual, group and family contexts. This supports research which suggests that exploring the function of emotions and practising emotion recognition might be useful treatment targets for AN []. Normalising eating behaviour and regaining a healthy weight was an integral part of Bella's treatment programme. Bella's plan set her rate of weight gain to roughly between 0.5 kg and 1 kg per week, and to steady her weight increase, her individual meal plans were adjusted according to her treatment progress.\nThe treatment centre participated in an established activeresearch programme, dedicated to investigating aspects of early onset eating disorders with a particular focus on neuroscience and affect regulation. The present study was part of that research programme, and Bella had volunteered to be a participant. Ethical approval for the study was obtained from both the University of Westminster and Barnet and Haringey Local Research Ethics Committees. The protocol involved the following assessments, described below. The questionnaire was chosen to assess Bella's eating pathology pre- and post-participation. After informed written consent was provided by both Bella and her parents, the following assessments were completed.\nThe EDI-3 contains 91 items which form various eating disorder risk scales and psychological scales that are rated using a six-point scoring format; the higher the scores for each scale, the more symptomatic the individual. The different scales regarding disordered behaviours and psychological traits can be collapsed to form two overall composite scores, named eating disorder risk and the general psychological maladjustment, the latter of which includes a measure of affective problems.\nBella was provided with a pack containing saliva sampling materials. She was instructed to collect saliva samples at awakening, 15, 30, and 45 minutes and 6 and 12 hours post-awakening on two consecutive weekdays. Saliva was collected by passive drool through a straw into the appropriately labelled small, plastic Eppendorf tube. For at least 30 minutes prior to the collection of each sample, she had to adhere by guidelines of nil by mouth other than water and the avoidance of vigorous exercise and brushing teeth. Other than these requests for compliance, Bella was free to follow her normal daily routine. After collection, Bella's samples were frozen (−20°C). On the last day of Bella's participation, the researcher used insulated packs to transfer samples to the laboratory where they were stored at −20°C until assay. On each study day, Bella recorded her awakening time, method of waking up (whether naturally or by alarm clock) and the exact times of collection of saliva samples. To maximise adherence to protocol, the timing of all Bella's samples was supervised by nursing staff.\nIn the laboratory, Bella's saliva samples were thawed and centrifuged at 1500 xg (3000 rpm) for 15 minutes. Her cortisol concentration were determined by the Expanded Range High Sensitivity Enzyme Linked Immuno-Sorbent Assay developed by Salimetrics LLC (USA). Similarly, the Salimetrics Salivary DHEA Enzyme. For full details of the assay procedure, see Oskis et al. [].\nResearchers now have the opportunity to utilise statistical techniques that allow single case studies to be amenable to the same statistical research questions as larger-N research studies. These have been especially useful within neuropsychology, where such research findings translate to the clinical setting and facilitate the development a case's profile of cognitive strengths and weakness []. However, these techniques are uncommon within neuroendocrinology where the use of single case studies is rare. Nevertheless, since our endeavour was the same, namely to see whether our case study was significantly different on assessments compared to a matched control sample, we chose to use the approach developed by Crawford and Howell []. This method is effectively a modified independent samples t-test in which the individual is treated as a sample of n = 1. The test is robust in that it controls the Type I error rate regardless of the size of the control sample. More recently, Crawford et al. [] have further developed this method so that as well as testing for a statistically significant difference, an effect size index for the difference between the case and controls can also be obtained. This index of effect size, termed z\nCC, is analogous to Cohen's d and is an estimate of the average difference, measured in standard deviation units, between a case's score and the score of a randomly chosen member of the control population. The computer programme Singlims-ES implements these methods and was used to analysis of Bella's assessments compared to a control group.\nBella's data were examined against a backdrop of comparator participant data derived from a parallel, larger study investigating HPA axis activity in healthy females []. This sample contained 15 post-menarche female adolescents who were age matched (mean (±SD) age 16.67 (±0.49) years), with normal BMI (21.39 kg/m2 ± 3.18).\nUsing Crawford and Howell's [] approach, Bella's mean cortisol and DHEA variables for the two samplings day were analysed against the corresponding values from the healthy control group to ascertain whether her values were significantly different. Composites were computed to represent the total concentrations and dynamic of each hormone, in line with conventions in neuroendocrine research see [].\nBoth and illustrate Bella's cortisol profile at baseline when she was ill with AN, and one year after being discharged from the inpatient service when she was recovered. At baseline, Bella's overall levels of cortisol after awakening were significantly higher than those of the control group. However, the dynamic of Bella's CAR mean increase did not differ significantly to that of the controls. For cortisol over the rest of the day, once again Bella's overall levels of cortisol from 6 to 12 hours post-awakening were significantly greater but there was no difference in the decline over this daytime period compared to the control group.\nUnlike cortisol, DHEA is not characterised by a marked awakening response [, ]. Given this, only the samples taken at awakening, 30 minutes, and 12 hours post-awakening were analysed for DHEA. Bella's concentrations in the 30 minute post-awakening period and in the evening were significantly higher than the healthy control group (see and ). As can also be seen in , Bella's eating pathology assessments at baseline confirmed her diagnosis and her EDI-3 scores for eating disorder risk, particularly the body dissatisfaction scale and general psychological maladjustment, were significantly higher than those of the control group. Bella's score for affective problems was also significantly higher than controls.\nBella was followed up one year after being discharged from her programme at the service. She was managing and maintaining her weight in the community and at follow-up Bella's weight was 58.5 kg with a BMI of 19 kg/m2. After leaving inpatient treatment, Bella was transferred to outpatient support in the form of a community practitioner nurse whom she saw roughly every six weeks. At the time of follow-up participation, Bella was at college getting ready to take her A-level exams in a few months' time and hoping to go to university in the coming year. She was happy socially, and had friends at college, including a boyfriend. She had just also started a part-time job in a local retail establishment.\nBella completed the same protocol as before. Crawford and Howell's [] approach was used once again to compare Bella's mean cortisol and DHEA composites for the two samplings day to the corresponding values from the healthy control group. One year after being discharged from the inpatient service when she was recovered, Bella's overall levels of cortisol after awakening were not significantly different to the control group and neither were her overall levels for the rest of the day. Furthermore, the dynamic of Bella's cortisol profile did not differ significantly to that of the controls for either the CAR or the rest of the daytime period. Bella's cortisol profiles at follow-up can be seen in and . Similarly for DHEA, Bella's concentrations were all comparable to the control group for both the 30 minute post-awakening period and also in the evening. Bella's DHEA profiles at follow-up can be seen in , and the accompanying statistics can be seen in .\nOne year on, all of Bella's EDI-3 scores had lowered and were not significantly different compared to the healthy control group (see ). Her general psychological maladjustment and her affective problems had improved to be comparable to the healthy group, and notably, the EDI-3 qualitative classification for these two indices had lowered from “typical clinical” during illness to “low clinical” at follow-up. |
A 43-year-old male visited the Department of Family Medicine with a history of mild headache, neck pain and nausea which had been ongoing for a day. He had a history of hypertension for 2 years and his blood pressure was 145/95 mmHg on his first visit. The patient had a chief complaint of a headache in both the occipital and temporal areas and tightness in the posterior neck which were aggravated by standing, coughing and shaking of the head. On the other hand the symptoms were relieved by lying down. Simple analgesics and muscle relaxants were prescribed, but they did not ease the headache. On the sixth day since the onset of headache, the patient experienced blurred and double vision and this led him to the hospital again. On neurologic examination, he had horizontal diplopia which became worse when gazing to the right side (). Ophthalmologic consultation confirmed right sixth cranial nerve palsy, and the consulting neurologist recommended performing brain and orbit MRI. The brain MRI showed diffuse pachymeningeal thickening and enhancement (). The patient was admitted on the supposition that intracranial hypotension might have caused the headache and sixth nerve palsy. Spinal tap was performed and revealed an opening pressure of 11 cmH2O in the right decubitus position. A CT myelogram was also taken, and contrast leakage was found in the posterior epidural space from C4 to C6 (). The patient's symptoms, MRI finding, and CT myelogram could all be explained by spontaneous intracranial hypotension in spite of the normal CSF pressure. He was referred to the pain clinic for EBP 10 days after the onset of the diplopia. We performed the EBP at the C4/5 level under fluoroscopic guidance by injecting 8 ml of autologous blood (). The headache was nearly relieved by the following day, but the diplopia was only partially improved. After discharge from the hospital, the patient was observed for 2 months and we confirmed his complete recovery from the diplopia (). |
A 25-year-old male student presented with persistent discomfort over the lateral malleolus following an uncomplicated twisting inversion injury of the ankle 3 months prior. The pain was responding to bandage compression and simple analgesia, however was limiting moderately strenuous exercise. On examination, there was full range of movement at the ankle in all directions, with discomfort on maximal dorsiflexion and eversion. A mild effusion of the ankle joint was appreciated compared to the contralateral side. No ankle deformities or overlying skin changes were appreciated. The patient was previously healthy, on no regular medication. He smokes and drinks only socially. Routine blood investigations taken recently had been unremarkable.\nAn x-ray was performed on first presentation which demonstrated multiple globular calcified loose bodies in the anterior aspect of the joint, in keeping with suspected synovial chondromatosis (). No fracture or joint deterioration was noted.\nMagnetic resonance imaging of the ankle joint was performed to better visualize the lesions ().\nThis confirmed the presence of multiple variably sized loose bodies within the ankle, primarily in the anterior and anterolateral recesses. The lesions were of indeterminate signal intensity on T1 and proton dense fat saturated images with a thin sclerotic rim. The largest loose body was 12 mm. The ankle and subtalar articular surfaces were normal with no evidence of osteochondral lesions or tarsal coalition. The visualized bones and ligaments were unremarkable.\nOpen removal of 27 loose bodies from the anterior gutter was performed, with the larger bodies sent for analysis (). An open approach, as opposed to arthroscopic, was opted for in view of the large number of bodies and to visually ensure complete removal. The procedure was successful having obtained complete clearance with no intraoperative complications. The patient was discharged home on the same day with simple analgesia. He was advised to keep the limb elevated, allowed full weight bearing with crutches, and referred for physiotherapy. A postoperative review at two weeks showed no interval complications, good functional outcome, and benign histology. No further imaging or arthroscopy was deemed necessary as the patient had improved back to his premorbid state and was completely pain free at a three-month follow up.\nThe patient represented 9 months later, as he was once again experiencing ankle discomfort for the past two weeks. A repeat x-ray of the affected joint showed the reaccumulation of loose bodies, however to a much lesser extent (). Once again, no fractures or joint abnormalities were detected. The management options were discussed, and the patient agreed to opt for a trial of conservative management, until the symptoms worsen. One year later, at the time of writing, his condition is clinically stable. |
A 56 year-old female was transported to the emergency department by EMS after physically threatening her husband with a knife. According to the patient, she experienced altered sensorium for one week prior to presentation, primarily resulting in suicidal and homicidal command hallucinations instructing her to overdose on NSAIDs and kill her husband. She denied any headaches, vertigo, fevers, head trauma, urinary symptoms, or use of illicit substances. There was no history of psychosis, schizophrenia, mania, or depression and a review of her medication list for any potential hallucinogenic agents did not indicate a pharmacologic etiology. Her past medical history was comprised of two prior strokes, the most recent being two years ago that involved the right frontal lobe. MRI of the brain at the time also showed evidence of an old right parietal lobe infarct. An echocardiogram during that hospitalization revealed a severely decreased left ventricular function and the patient was initiated on warfarin for anticoagulation. Since the two prior cerebral infarcts, the patient and her husband denied noting any behavioral changes, cognitive impairment, or any focal neurologic deficits. On presentation, she appeared disheveled and exhibited a flat affect with minimal verbalization. Physical examination revealed only a left hemianopia without hemiplegia. All cranial nerves were evaluated as well as gait, cerebellar function, and proprioception, which were all found to be normal. The patient was alert and oriented with intact mentation.\nShe was initially admitted to the psychiatric unit for further evaluation where the patient participated in daily activities and reported no symptoms. Given the acute onset of her symptoms, laboratory studies and brain images were obtained in order to rule out an organic etiology. A complete blood count was normal and a basic metabolic panel revealed normal electrolytes and renal function. Additional labs included liver function tests, lipid panel, cardiolipin antibody, and TSH, which were all normal and RPR was nonreactive. A urine sample was negative for urinary tract infection and 8-panel urine drug screen was negative. An MRI of the brain was obtained, which revealed old ischemic infarcts within the right parietal and frontal lobes along with a new acute right occipital lobe infarct with hemorrhagic transformation (). The patient was subsequently transferred to the medical unit for further workup and management.\nThe patient was placed on telemetry, which showed normal sinus rhythm. Given the involvement of multiple brain territories and circulations, a cardioembolic source of stroke was highly suspected. A cardiologist evaluated the patient and performed a transesophageal echocardiogram that revealed a low ejection fraction of less than 20%. In addition, a bubble study was performed that was positive for a patent foramen ovale. Given her significantly low ejection fraction, the patient underwent placement of an automated implantable cardioverter-defibrillator. The patient's presenting symptom of psychosis, primarily in the form of auditory hallucinations, was self-limited and resolved on day two of hospitalization without requiring the use of antipsychotic medications. |
Mr. M was a 27-year-old single male, who had been working as an engineer in Germany for 15 months. He attended my outpatients’ clinic in Chennai with his brother and mother. He had returned suddenly from Germany the previous day, as he was convinced that his life was under threat from his work colleagues. When asked why he was being targeted, he said that he had developed a software tool at work recently, which because of its effectiveness was able to do the job of several people; consequently, some of his colleagues were angry with him as they were at risk of losing their jobs. He believed that his drinks and food in Germany were being contaminated to harm him. He felt he was being used as a “human sample.” He was convinced that his mobile phone calls were hacked, his internet activity was being monitored, and that he was being followed. He had written to his company management about his perceived persecution, and he had even lodged a complaint with the local German police who visited him at home to reassure him that he was safe. Even though he felt safer in Chennai than in Germany, he was concerned about potential harm to himself and his family from his German colleagues. He was adamant that he would never return to Germany.\nHe denied any hallucinations. He reported disturbed sleep over the previous few nights. He denied any suicidal ideas or ideas of wanting to harm others. There were no incidents of aggression.\nHe reported that he was started on isotretinoin by a dermatologist in Germany for acne (which by his account and on appearance did not seem particularly severe) about 2 weeks previously. The psychiatric symptoms started about 5 days later, increasing in severity gradually.\nHe had no previous psychiatric history or family psychiatric history. He reported no history of any major medical illnesses and was not on any regular medication apart from the recently started isotretinoin. He had one older brother. He was born of a full-term normal delivery and his mother said that his developmental milestones were normal. Patient reported no neglect or abuse as a child. He held a Master's degree in engineering, and had work experience for 4 years in an Indian company before going to Germany. He was a nonsmoker and denied any abuse of illicit drugs. In Germany, he used to drink beer occasionally.\nDuring the assessment, he appeared tense but was cooperative and there was no formal thought disorder. He reported feeling “heavy” in his head. He had a slightly blunted affect, although he was able to smile at times. On testing his cognition, he was oriented in time and place, his immediate and delayed recall were normal, his concentration (serial 7s test) was preserved and he was able to interpret a proverb correctly. With respect to insight, he did not think he had a psychiatric problem and was more concerned about potential harm to his physical health due to deliberate “contamination” of food and drink in Germany. Nevertheless, he was willing to cooperate with the plan I suggested.\nA provisional diagnosis of an acute psychotic episode (International Classification of Diseases-10 category F23.3 – “other predominantly delusional psychotic disorders”) was made, on the basis of acute onset of delusions (mainly of a persecutory nature) within a period of <2 weeks. Isotretinoin was considered the likely precipitant as the onset of the symptoms followed its initiation. Although a functional origin of symptoms was possible (i.e. first episode of a functional mental illness like schizophrenia), absence of any prodromal symptoms, previous psychiatric history, or family psychiatric history made this unlikely.\nMr. M was advised to stop isotretinoin. He was started on a low dose of risperidone 1 mg at night. In addition he was prescribed clonazepam 0.25 mg twice a day and zolpidem 5 mg at night. Some routine investigations were ordered. He was reviewed 3 days later, accompanied by his mother. He showed remarkable improvement. His sleep had improved, the “heaviness” in the head was much less, he no longer believed he or his family were under threat, and he was even open to the possibility of returning to Germany (an option he was vehemently against just 3 days previously!). His investigations (complete blood count, thyroid function test, liver function test, glucose, cholesterol, creatinine, and electrocardiography) were within normal limits. He was advised to continue risperidone 1 mg at night and zolpidem 5 mg at night. The dosage of clonazepam was reduced to 0.25 mg once daily.\nMr. M was reviewed again 4 days later (7 days after his first presentation). He felt he was fully back to his normal self, and this was corroborated by his mother. There were no psychotic, affective, or anxiety symptoms. He was keen to return to Germany as soon as possible, and I provided him a certificate of fitness to return to work. Clonazepam and zolpidem were stopped. As he was going to be living on his own in Germany, I advised him to take risperidone 1 mg at night for a further 3 months, mainly as a precautionary measure. He returned to Germany about 10 days later to resume his previous employment. He continues to remain well 11 months after his original presentation (8 months without medication).\nThe Naranjo adverse drug reaction probability scale[] is sometimes helpful in determining whether a particular side-effect is due to a specific medication. The aggregate score from 10 items falls under four possible categories: “Doubtful” if the score is 0, “possible” if 1-4, “probable” if 5-8 and “definite” if the score is 9 or more. In this patient, the score was 7 (which falls in the “probable” category – i.e. that his psychosis was probably due to isotretinoin). |
We present a case of a 50-year-old African American male with a past psychiatric history of schizophrenia who was admitted to our ED after his neighbors complained of a foul smell coming from his apartment. The patient presented with mostly negative symptoms of schizophrenia. He initially stated that his neighbors called the police because “the weather is wet” and his allergies were out of control. He was found at home with his deceased mother who had shown signs of decomposing. When he was initially assessed, he only complained of feeling short of breath due to a foul smell of tuna fish coming from around his mother. When asked about his deceased mother he stated she was watching television and that she still had a pulse. According to the patient he had been giving his mother tea and medication for the past couple of days and had recently given her aspirin for her headaches before police arrival. It was determined that he needed psychiatric evaluation and he was brought into the ED by emergency medical services. He was determined to be acutely psychotic with poor reality testing and insight likely due to poor medication compliance. He continued to remain unaware of his mother's death during evaluation. He was admitted to inpatient psychiatry service for stabilization. On initial evaluation in inpatient psychiatry, the patient continued to maintain that he could communicate with his mother and spoke to her with a red phone next to his hospital bed. He was withdrawn, disheveled, malodorous, blunted in affect, internally preoccupied, and intermittently responding to internal stimuli (Table ). PANSS score was 32 on the positive scale and 39 on the negative scale.\nMontreal Cognitive Assessment (MOCA) test revealed a score of 26/30. The patient showed no significant cognitive impairment but had cognitive distortions in understanding the definitions and irreversibility of death. Collateral information provided by his stepbrother revealed that the patient was valedictorian in college before his first psychotic episode. Since then he has never returned to school; he has been cared for by his mother at home. The patient’s bereavement was best categorized to be in the denial stage which was complicated with delusions and auditory hallucinations of a living mother. His treatment plan included the resumption of home medications of clozapine starting at a dose of 50 mg and optimizing to 600 mg over a period of two weeks. In addition, to target his bereavement and distortions relating to his mother’s death, cognitive behavioral therapy (CBT) divided into 20 sessions was scheduled for the course of his hospitalization. CBT treatment usually involves efforts to change faulty thinking patterns, unhelpful behavioral patterns, and help individuals learn to develop coping skills. CBT was aimed at moving him through the stages of grief.\nIn the first week of hospital admission, the patient demonstrated denial which was modified by the presence of delusions. He was easily approachable and cooperative. However, he displayed poor insight into his reason for hospital admission, stating he had been in the hospital to get his allergies controlled. A gentle exploration of psychotic symptoms was performed given his poor insight and reality testing. He denied any auditory or visual hallucinations as well as suicidal or homicidal intentions at the time. The patient denied feeling sadness, anhedonia, excessive guilt, hopelessness, worthlessness, or a decrease in concentration. The patient did not display any signs of grief during the interview sessions, stating he lives at home with his mother and referring to her in the present tense. The patient’s denial of his mother's death increased as we explored his psychotic delusions. He stated he was expecting a visit from her to the hospital and requested we check the logbook for her signature under an alias name. He did not provide any further information but did provide a telephone number to contact her. The patient stated his mother did not visit as intended but he spoke with her over the phone and she said she would visit him within the next two to three days. He stated, “my mother needs to bring my medication for fatigue before I can go home.” When asked how he had contacted his mother, the patient looked at his palm and stated, “with a gloved telephone that my mother gave me when I was a baby. Can’t you see it? The red phone.” With an excited tone, the patient elaborated on the “gloved telephone” stating it was connected to the main desk and referred to it as a “cord wire.” The patient continued to refer to his mother in the present tense displaying poor insight into his illness and exhibited worsening hallucinations and delusions regarding the “red telephone glove.” The treatment team deferred further exploration of the patient's delusions or hallucinations at this time.\nAs we approached the middle of week one, the patient seemed to have regressed in certain aspects of his personality, exhibiting childish hallucinations. The patient elaborated on the “red telephone glove” stating it has “a radar on it, so my mother can track me at any time but I can’t do video chat on it because it would get too hot and burn my hand.” The patient continued to believe his mother was alive, stating she works as a social worker and that he was waiting to speak with her again with the hope that she will sign his medication list. The treatment team seized an opportunity to ask about his understanding of death. He displayed very poor insight into the topic stating he does not believe people die, rather they read books on how to build their muscles and watch television to relax. When asked if he has ever experienced the death of a family member or friend, the patient initially denied this and stated he has never been to a funeral. He later said, “Funerals are a place where people relax their muscles.” When asked about other patients on the unit, the patient stated they were all there for “allergies.” This further displayed his poor insight into his illness.\nThe patient had shown some improvement in his mood and affect as well as orientation to person, place, and time as compared to his initial day of hospital admission. The patient was sitting up in bed during his interviews covered in blankets and a towel around his head. This body demeanor remained constant up until the third week of his hospital admission. When asked about his time during college, the patient stated he went to Baruch College and studied “flowers” and later received his Masters in the subject over the span of five years. The patient denied any traumatic or significant event during these years. When asked about his experience as a Valedictorian, based on the collateral information provided by his step-brother, the patient's affect changed. He denied having a step-brother, stating the person who provided that information was a friend who is an alcoholic and should not be trusted. The patient, however, still seemed to be in denial regarding his mother’s death, constantly referring to her in the present tense and awaiting her calls. The patient denied any intimate relationships but enjoyed his time shopping, talking, and eating “eggs and toast” with his mother which they both enjoyed. The patient refused to allow anyone else to contact his mother other than himself to protect her “security.” The patient’s delusions continued to increase regarding his mother, stating he was waiting for his mother to come to be discharged, and that if she is unable to come, he will stay with a babysitter. His discussion on the babysitter was limited; he said it was a private topic. The patient was asked several questions on this day in order to assess fairness, judgment, punishment, reward, and experience. He displayed poor insight on each category, especially in relation to the severity of crimes, fair punishment, and reward. He stated, “The reward depends on the person. It’s private. I like ice cream as a reward”. His responses further proved a childish regression in his understanding of various topics. The patient was asked to assess his current situation of whether or not it was appropriate to treat someone with an allergy on the psychiatric ward versus the medical ward. The patient hesitated at first but eventually said the medical floor was better, however, “The medical floor has the medical tape that grows trees with soaked detergent for the nostrils” perhaps an elaboration of his delusions on the treatment of allergies.\nIn the second week of hospital admission, denial intensity decreased and no signs of anger or depression were shown. The patient had a PANSS score of 25 on the positive scale and 32 on the negative scale. CBT was geared towards establishing the moral stage and regression of the patient’s personality. Although the patient admitted to being 50 years old, he often displayed childish delusions. A series of questions were asked in accordance with Kohlberg’s scale to assess the patient’s moral reasoning. The series of questions demonstrated the patient’s poor insight into the idea of punishment and reward. When asked whether or not theft should be punished by law, the patient stated the person who commits the crime or to whom the crime affects, should decide the punishment, as opposed to law enforcement, such as the police or a judge. The patient also expressed his reluctance in helping strangers if they are in need but would help his mother if ever needed. Based on this interview, it was evident that the patient was in the preconventional stages of Kohlberg’s Moral Stage scale, particularly stage 1 (up to age 9). He expressed no insight on law and order and had no concept of punishment; as his responses were expressed in an egocentric viewpoint.\nThroughout week two, CBT was further geared towards assessing the patient’s defense mechanisms and observing his response to the news that his mother will not be coming to see him in the hospital. The patient’s mood during this week can be best described as depressed; the patient stated the reason for this was due to the weather. When confronted that his mother will not pick him up at discharge, nor will she be at home, the patient seemed to accept the idea that his mother is no longer alive. He asserted that he will take care of himself with the help of his mother’s trust fund and babysitters. When pressed to elaborate on this, the patient refused because he considered it a private matter. When asked how he felt not seeing his mother ever again he chuckled and replied, “it’s ok, we went different ways, we have to grow, she has her friends and I have mine.” Despite given unpleasant news, the patient’s reaction was inappropriate lacking proper emotion or reaction.\nFurther insight into the patient’s delusions was evident when the interview later shifted to a psychoanalytical point of view. When asked if the patient experienced dreams, he replied “I have a night lamp in my eyes. It helps me sleep. My mother put it there when I was younger. It keeps my mind occupied. It is like a flashlight sticker that was placed in my eye.” The dialogue further proved worsening delusions and insight. The patient seemed to regress back into the denial stage of his mother’s passing at this time. He displayed selective blocking when asked if he could recall the prior conversations, only recalling topics such as his time during college and friends. When confronted about his mother, he stated “yes, she’s divorced/separated now” but did not know from whom, and then eventually he stated he was “ok” with the idea of her passing.\nThe patient’s mood and affect seemed brighter as he was seen smiling throughout the interview at the end of week two of his hospital admission. He stated he was waiting for his brother to pick him up once discharged from the hospital. When asked if the patient could recall prior conversations regarding his mother, the patient stated, “Oh yeah, I remember. We talked about the divorce. She’s separated from us now; if she’s feeling better, she’ll be home. She has to have a pulse, she has a pulse now, her pulse is ok, it’s just at work where she has to rest.” In exploring his understanding of death, and after giving him a metaphor of flowers and death, the patient expressed the idea of death to be similar to that of a “wilting flower.” He was able to identify that the conversation mirrored his mother’s death, but he remained adamant on the idea of “divorce” as the reason his mother has “left and moved on.” When asked about his grandmother’s passing, the patient was able to identify her death as no longer having a “pulse” and later stated it was similar to the idea of his mother. This was the first time the patient acknowledged death which contradicted his initial response during week one when he was asked if he had experienced death in a family member. At this stage, we believe the patient began to approach the acceptance stage of bereavement. The patient’s elaboration of death seemed to circulate the idea of reincarnation, stating “people can come back, like reincarnation. Flowers can bring them back and then they will grow.” This demonstrated his understanding of his mother’s death, where he stated that if she wanted to come back in the form of reincarnation she can, however, she will not be present to pick him up from the hospital at the time of discharge.\nDuring the third week of hospital admission, the patient demonstrated resolution of denial. He elaborated on his understanding of death by exploring the idea of reincarnation and demonstrating his understanding that his mother is no longer present with him. By this time, his PANSS score was 10 on the positive scale and 23 on the negative scale. He remained focused on the hope that his brother will pick him up at discharge and he will go back to living his life with his brother and neighbors, gardening, and playing video games. Upon the conclusion of our CBT, the patient’s final statement when asked who was taking care of his garden, was “whoever my mother left in charge. She died so she can reincarnate herself. When people die, they just don’t come back, they go to dust, ashes to ashes.” This statement signified his acceptance stage in bereavement and his understanding of life after the death of his mother.\nThe goal of week four was to evaluate the permanence and effectiveness of the CBT techniques used over the past month. By this time, his PANSS score was 8 on the positive scale and 19 on the negative scale. This week's goal was to examine the overall effect that combined pharmacological and CBT therapy had on the patient’s understanding of his circumstances as well as his level of processing of grief within the accepted bereavement model. As with previous weeks where we were successful in modifying autonomic thought processes, maladaptive core beliefs, and schemas, week four focused on evaluating whether these modifications have aided the patient through the stages of bereavement and processing of the finality of his mother's passing. By establishing a timeline of events with the patient in weeks, we were able to guide him through the events leading up to his admission allowing him the opportunity to re-evaluate previous beliefs held about the weeks prior to admission and the day of admission. Assessment for possibilities of his mother still being alive and present demonstrated that the patient no longer held the same beliefs. He demonstrated an understanding that his mother was no longer alive and that her remains were in the form of ashes in a mantle. A family meeting was held with his stepbrother and arrangement for his mother’s funeral was discussed. He participated in the funeral arrangement discussions and made arrangements to attend the funeral. |
The first case was a 59-year-old African American male with a past medical history notable for schizoaffective disorder, depression, and substance abuse who was brought in to the emergency room for disorganized behavior and agitation in the community. At the time of admission the patient demonstrated disorientation, repetitive motor behavior, and an alternation between agitation and psychomotor retardation. He had poor response to communication and tactile stimuli. A suspicion of altered mental status due to organic causes was suspected with the possibility of catatonic excitement and retardation. He was admitted to the medical floor, with a work-up revealing a positive toxicology screen for cocaine and opioids. The patients CBC and BMP were within normal limits except for his ammonia level which was 80 mg/dl. The patient was initially treated with Chlorpromazine Hcl 50 mg orally daily for his agitated behavior as well as Naltrexone 50 mg orally daily for his opiate intoxication.\nThe patient exhibited incoherent thought process in addition to mumbled speech that made a significant portion of his assessment evaluation difficult. During evaluation, he displayed abnormal movements of his arms and face, with tremors and restlessness. His affect was flat. He did not display any perceptual disturbances or delusions. An assessment for cognitive impairment was noncontributory during his most recent admission. The patient received Mirtazapine 45 mg orally at bedtime and Olanzapine 10 mg orally daily in his treatment and by day three of admission had shown improvement in his disorganized behavior with supportive care. The patient demonstrated more effort to directly communicate with house staff after treatment began.\nThe patient reported a past history of psychiatric illness that was late in onset. His first presentation at the age of 51 years was significant for depressed mood, paranoid delusions, and auditory hallucinations for which he was diagnosed with a major mood disorder. His symptoms responded poorly to medications including antidepressants. His disease course involved increasing periods of impulsive behavior and agitation. He became noncompliant with his prescribed medications. He was later admitted to the medical floors at the age of 54 years for “repetitive behavior” during which he was found moving from his bed to the bathroom repeatedly as if he wanted to use the bathroom all the time. He also showed some abnormal rocking movements during this time period. A medical work-up for seizure was negative. He was discharged with a presumptive diagnosis of a psychotic disorder. Thereafter, at the age of 56 years he had an episode of property destruction in the community and it was noted that he had “abnormal body movements” in addition to lability of mood. His diagnosis was revised to schizoaffective disorder and he was treated for mood lability at the time with risperidone.\nGiven the late onset of his neuropsychiatric symptoms, a computed tomography scan (CT) of his brain was done during his presentation, as seen in . Reviewing his chart, it was noted that the calcifications were apparent in his first head CT taken in January of 2012 with no changes to the current CT in January of 2019. |
A 60-year-old man with the past medical history of AFib, pulmonary embolism (PE) and hypertension presented to the emergency room (ER) with the presenting complaint of weakness and numbness of left arm and left half of the face that started approximately 90 min prior to the presentation. The patient was taking apixaban (5 mg twice daily) for his AFib. However, mistakenly patient stopped taking apixaban after he was told by his primary medical doctor to stop losartan. On arrival to ER, stroke code was called given patient’s symptoms and his National Institutes of Health stroke scale (NIHSS) score in ER was 3. Being in the therapeutic window, the patient received intravenous tissue plasminogen activator (t-PA) bolus in ER which led to significant improvement in the symptoms. Later that day, the patient developed new onset numbness and tingling of his left hand with cold fingers as compared to the right hand. On assessment, patient was found to have a good brachial pulse, but the radial pulse was not palpable on the left side. An arterial duplex of the left arm was ordered, which revealed diminished blood flow in the brachial artery and occluded ulnar and radial arteries. After getting approval from neurology, vascular surgery performed brachial artery embolectomy, which was 24 h after he received t-PA. Patient was started on heparin infusion and blood flow to the distal extremity was adequately established. For the further workup of stroke, a transthoracic echocardiogram was done to rule out cardio-embolic source for the acute stroke and radial arterial occlusion. Surprisingly, the patient was found to have a 2.1 × 2.8 cm large mobile mass in the left atrium that was attached to the atrial septum (-).\nThis was a new finding as patient had transthoracic echocardiogram and computed tomography 2 months prior to this event and there was no evidence of left atrial mass at that time. Given the acute nature of development of this mass, it was presumed to be a thrombus. Repeat echocardiogram was performed on day 6 of hospital stay and showed the similar left atrial thrombus attached with the atrial septum, unchanged from the prior study. Patient was bridged to coumadin with heparin infusion during hospital stay and discharged home safely on coumadin after a total hospital stay of 1 week.\nAfter getting discharged from hospital, patient was followed up and workup for thrombophilia was done that came back negative. Patient had normal protein C and S activity, normal anticardiolipin IgG and IgM antibody level and negative beta-2 glycoprotein antibody assay. Patient was negative for factor V Leiden mutation and prothrombin gene mutation. Patient was advised to get a repeat echocardiogram. Unfortunately, patient lost follow-up because of pandemic crisis. An echocardiogram was repeated after 1 year that showed complete resolution of the thrombus in the left atrium (). |
A 37-year old homeless male, with a past medical history of peripheral vascular disease, type 1 diabetes mellitus, hypertension, and depression, presented to the emergency department with intermittent chest pain and progressive shortness of breath for a few weeks. The patient denied intravenous drug use, although admitted to the use of recreational marijuana. The patient’s vitals on admission were stable except for low saturation on a pulse oximetry of 86% on room air. On physical examination, the patient was tachypnic and had fine crackles in the bilateral lung fields on auscultation. Laboratory results on admission showed a normal complete blood count and basic metabolic panel, but a urine drug screen test was positive for opioids. Because of persistent hypoxia, a D-Dimer was checked and came back highly elevated. The patient underwent a computed tomography angiography (CTA) of the chest to rule out pulmonary embolism. The CTA was negative for pulmonary embolism, however, it displayed extensive miliary densities throughout the bilateral lung fields (). The patient was admitted to the floor with a differential diagnosis of military tuberculosis versus fungal infection. Human immunodeficiency virus (HIV), fungal, and Quantiferron testing were negative. The cardiac work up and autoimmune serology were also unremarkable. The patient was then started on intravenous steroids and inhaled albuterol, although no improvement was seen. The patient remained hypoxemic despite therapy, and, therefore, underwent a bronchoscopy with a lung biopsy to find out the etiology of the disease process. The lung biopsy showed alveolated lung tissue with a miliary pattern of perivascular foreign body histiocytes containing refractory material suggestive of microcrystalline cellulose material (). There was no evidence of malignancy and there were no fungal or acid fast bacilli organisms identified on special stains. The histological features suggested intravenous injection of foreign material and upon further questioning the patient admitted to injecting oral opiates. The patient was started on intravenous steroids, although his clinical condition continued to decline. The patient developed hypercapnic respiratory failure, which required intubation, and eventually suffered from a cardiopulmonary arrest and passed away. |
A 54-year-old man was referred to our infectious diseases outpatients clinic by his primary care physician for ongoing management of a possible disseminated fungal infection.\nThe patient's presenting complaint was a 7-year history of a pervasive “fungus” infection throughout his body. He states this infection moves from one part of his body to the next at random. He described the intermittent sensation of webbing over his eyes and further described formication that travels up from his feet to his scalp. He also stated that when he showered in the mornings and sunlight came into his bathroom, he could see the fungal spores and felt he was reinfecting his lungs and eyes. During the consultation he repeatedly attempted to rub the fungus off his skin to prove its existence. shows material the patient collected from his fingernails after vigorous scratching, which he incubated in water at room temperature for 6 weeks.\nInitially the patient was seen after a glancing injury to the eye for which he was diagnosed and treated for fungal keratitis to good effect by an ophthalmologist. Since this event he states the infection has spread and had been unsuccessfully treated. His primary care physician had reviewed him on numerous occasions for the same complaint and treated him with both topical and oral antifungal preparations including Clotrimazole and Griseofulvin. He had furthermore attempted oral antibiotic therapy with cephalexin and metronidazole, all with no effect. Following failed attempts at treatment the patient states he was reviewed by three dermatologists and two infectious disease physicians with no cause for his symptoms found. Unsatisfied with his consultations, he attended a naturopath whom he states performed confirmatory blood tests diagnosing a fungemia. Unfortunately he was unable to produce evidence of this visit or results of investigations performed. The patient's past medical history is notable for untreated Hepatitis C genotype 2b infection, a past Hepatitis B infection, and a distant history of intravenous drug use for which he did not require opioid therapy. He takes no oral medications and applies tea-tree oil to his skin when required, as he felt this assisted in controlling his fungal infection.\nOver the course of three reviews there were no abnormal examination findings of this patient. For completeness of consultation investigations including white cell count, C-reactive protein, human immunodeficiency virus, T-lymphocyte counts, cryptococcal antigen, galactomannan, and a skin scraping for microscopy and culture were performed, results of which were all within normal range or negative. On presentation of these results to the patient he was thankful the tests had been performed, but unfortunately he was incredulous as to the negative results. Once rapport had been developed we delved further into the patient's recent psychiatric history. During his second consultation he admitted to a depressed mood, decreased appetite, increased sleep, episodes of unprovoked teariness, hopelessness, and previous episodes of self-harm. He did however deny any active thoughts of self-harm or suicide. When the subject of depression was broached the patient wholeheartedly agreed but felt his depression stemmed from inadequate treatment of his fungal infection. At this point he agreed to a psychiatric review as well as a course of an antidepressant; however on review several months later he has failed to attend his appointments or commence his medication. |
A 5-month old female infant was brought by worried parents to our department with an accessory lower limb attached to the lower back. The infant was born with it and it was growing commensurate with the growth of the rest of the infant’s body. The infant was the fourth child of her parents, who had contracted a non-consanguineous marriage ten years ago. All the previous siblings were normal. The parents did not give any history of maternal illness, smoking, alcohol abuse, medication intake, radiation exposure or any other obvious gestational insult through the course of their pregnancy that yielded the anomalous baby.\nThe mother had not undergone any antenatal screening and hence, the condition was obvious only after natural delivery at home. Except for sluggish movement in the normal right lower limb, the infant had no other associated systemic abnormality. The parents were particularly concerned about the gruesome appearance and difficulty with care and posture of the infant. Clinical examination of the infant revealed a well-developed accessory lower limb arising from the back at the level of L2 through S1 vertebrae ().\nThe accessory lower limb was pointing cranially. Grossly, it resembled a normal lower limb with a partially developed foot containing only big toe and the adjacent next toe. On the accessory lower limb, there were four blind ending gut loops of red color (). These used to wet the infant’s clothing with their watery secretion. Additionally, there were areas with dimples mimicking the natal cleft and anal opening. There was no spontaneous movement, however withdrawal to pain was present in the accessory lower limb.\nThe normal left lower limb was neurologically intact. However, there was grade 3 weakness of the normal right lower limb. The infant had normal looking external genitalia, anus, abdominal and thoracic walls. Plain X-rays showed the osseous components of the accessory lower limb (). Lumbosacral computed tomography with three dimensional reconstruction revealed deficient posterior bony elements at the level of L2 through SI vertebrae along with evidence of lipomyelomeningocele.\nIt also confirmed an accessory lower limb attached to the lumbosacral spine and right gluteal muscles. The limb had a rudimentary iliac bone, femur, tibia, fibula, rudimentary foot bones, hip and knee joints (). Whole spine magnetic resonance imaging (MRI) confirmed the presence of spinal dysraphism involving lumbar spine with deficient posterior elements (). The spinal cord and thecal sac were protruding posteriorly and towards the left superiorly through the defect in the posterior elements at the level opposite L3 through S1 vertebrae, revealing a large lipomyelomeningocele which measured approximately 4.7×3×4.8 cm.\nThe CT scan findings of the accessory lower limb were also re-confirmed. The cranial magnetic resonance was normal with no associated cerebral anomalies found. Written informed consent was taken from the parents of the infant for undertaking surgery and taking serial photographs through the course of treatment. At the time of surgery, the patient was positioned prone on table (). An elliptical skin incision (transversely oriented on the lower back) was designed to ensure adequate closure of the resultant defect after limb extirpation ().\nThe limb was eschmarked to preserve blood and circulatory physiology of the infant during surgery. Following incision of the skin and subcutaneous tissues, deeper dissection was undertaken in a standard fashion and carried down to the fibro-osseous attachments of the rudimentary hemi-pelvis. The accessory lower limb was ablated into by disarticulation at the level of the accessory hemi-pelvis/ileum, which was carefully dissected from its attachments to the lumbosacral spine and gluteal muscles. Dissection and repair of the large lipomyelomeningocele was performed in a standard fashion. The neural placord was carefully de-tethered from attachments and returned.\nThe overlying dorsolumbar fascia was closed in double breasting manner. The skin flaps were approximated in a tension free manner (). The wound was closed with subcutaneous absorbable sutures and steristrips were employed (). The immediate postoperative period was uneventful and the infant was discharged home on 5th postoperative day (). shows the ablated accessory limb. She was clinically fine at her three months follow up visit with no additional neurologic deficits or any other complications. Her parents were instructed to ensure regular follow up visits at six months intervals. |
A term female infant born at 39 weeks by repeat caesarean section to a 31-year-old gravida 3, para 3 mother after uncomplicated pregnancy was transferred to our Neonatal Intensive Care Unit (NICU) at approximately forty-eight hours of life due to history of no urine output, ambiguous genitalia, and imperforate anus. Apgar scores were 7, 7, and 9 at 1, 5, and 10 minutes, respectively, with birth weight of 2.9 kg. Physical examination did not reveal any facial dysmorphism. Significant findings on clinical exam included an abnormal genitourinary exam with hypoplastic labia majora, small fused labia minora, and a single opening in the urethral/vaginal area which yielded turbid white fluid when a catheter was placed temporarily. Constellation of these findings suggested urorectal septum malformation. Lung exam was normal with good air entry and clear lung fields, and the infant remained in room air. A chest radiograph revealed normal lung volume and normal expansion, while an abdominal radiograph was remarkable for “double-bubble sign” concerning for duodenal atresia, which was later confirmed by an upper gastrointestinal contrast imaging study. A complete renal ultrasound () was concerning for bilateral renal agenesis which was confirmed by a MAG 3 scan (). Bilateral renal agenesis was also noted and confirmed on an MRI (). No gonads were identified on abdominal and pelvic ultrasounds. Normal female karyotype was confirmed by chromosome analysis. The infant had rapidly progressing anuric renal failure after birth. In view of complexity of the medical situation and the anticipated need for multiple surgeries on the background of perceived universal fatality of bilateral renal agenesis, a multidisciplinary care conference was held with the family to discuss further direction of care. The medical team supported the family's decision to proceed with full interventions including surgery for duodenal atresia and peritoneal dialysis with goal for renal transplantation in future. She underwent duodenal atresia repair with colostomy and mucous fistula formation. She has remained on room air since birth, and lung development and function were notably normal. She did well on peritoneal dialysis until 4 years of age, and her growth parameters and development remained within normal range. As she was awaiting a renal transplant and needed peritoneal dialysis, her pull through procedure for her colostomy was deferred and was planned to be done at a later time in combination with urogenital reconstruction surgery. She was scheduled for surgery for renal transplantation a few months after her fourth birthday, but she suffered an anaphylactic reaction while anesthesia was being induced, and her surgery was cancelled. She was taken off the transplant list while interdisciplinary teams collaborated to make a plan for safe anesthesia. Unfortunately, a few months later, she was found unresponsive in her bed and was pronounced dead after a full attempt at resuscitation. Notably she was clinically doing well until the time of her demise, and a full autopsy failed to reveal a definite cause of death. All cultures were negative, and it was determined that the most likely cause of death was an electrolyte imbalance that caused cardiac arrest. The autopsy also confirmed the diagnosis of bilateral renal agenesis. |
A 30 year old male patient having height of 180 cm and weight of 70 kg (Body Mass Index 21.6) presented to the emergency department of Civil Hospital Karachi with the complaint of a penetrating injury at the left side of upper torso. The patient was lying comfortably with no symptoms of respiratory distress or any obvious active bleeding. Vitally the patient was stable. There was slight blood staining of the upper garment with an obvious breach.\nOn examination a 1 cm × 1 cm entry wound at the left 3rd intercostal space in the mid clavicular line was identified. The chest and abdomen were otherwise unremarkable. Normal vesicular breathing was appreciated on chest auscultation. On palpation the abdomen was soft with no peritoneal signs.\nChest radiograph demonstrated clear lung fields with no indication of injury. The abdominal radiographs (Figures and ) displayed a bullet in the upper left quadrant of the abdomen lateral to spine. The radiographs were otherwise unremarkable for any other abnormality.\nWith this evidence of the bullets entry at 3rd intercostal space laterally to the mid clavicular line and its lodgment in the abdomen just lateral to the spine, the bullets trajectory was estimated to have encountered the left lung, left dome of the diaphragm, stomach, left lobe of the liver, splenic vessels, body of the pancreas and the left kidney. Despite this trajectory, the patient was clinically stable with no signs of either respiratory distress or any other visceral injury. An urgent CT scan was advised to identify any serious damage as was being predicted on the basis of the estimated trajectory.\nThe CT scan (Figure ) showed that the bullet was lying postero medially to the left kidney just lateral to the spine. All thoracic, intra peritoneal and retroperitoneal visceral structures were identified to be normal with no injury.\nAs the patient remained clinically and vitally stable with there being clear evidence of all visceral structures being unharmed from the bullet, the patient was admitted for nonoperative management and observation. Patients’ hemoglobin (Hb) concentration and other baseline investigations were performed on admission and subsequently after every 24 h. There was no drop in the Hb from the initial measurement of 12.5 mg/dl. Serial clinical examinations were performed that remained negative for any peritoneal signs, visceral damage or respiratory distress. The patient was therefore discharged after 48 h of observation. |
Our case is a previously healthy 42 year-old Caucasian female with biopsy proven idiopathic membranous nephropathy (MGN) who was being treated with FK506 and prednisone for nephrotic syndrome. She was a never smoker and did not have a prior history of hypertension or diabetes. She tested negative for ANA, ANCA vasculitis, HIV, hepatitis B and C. Her kidney biopsy showed typical features of membranous glomerulonephritis in addition to positive staining with the anti-phospholiapse A2 receptor antibody which identifies idiopathic MGN with 97 % specificity []. Serum Tacrolimus levels were maintained between 6 and 8 ng/mL. She responded favorably to treatment and her initial urine protein/creatinine ratio of 8 gm/gm declined to less than 1 gm/gm within the initial 2 weeks of therapy. Her serum albumin level of 2 gm/dL prior to initiation of therapy improved to 3.3 gm/dL after 3 weeks of initiation of treatment (Fig. ). The patient also developed hypertension as a side effect of calcineurin inhibitor therapy and was treated with Losartan 100 mg PO daily. Her blood pressure remained well controlled throughout the treatment period and hypertension resolved once therapy was discontinued. After 3 months of therapy she presented with diplopia. She was in complete remission at this time and her serum albumin had normalized to 4 gm/dL. Her clinical course is depicted in Fig. . The diplopia was gradual in onset, binocular and vertical and more prominent in the later part of the day. The patient was seen for an ophthalmologic evaluation. Her visual acuity was 20/20. Pupils were equal and reactive to light and accommodation. No nystagmus or ptosis was observed. Visual fields and color vision was also normal in both eyes. Assay for acetylcholine receptor antibody done to rule out myasthenia gravis was negative. A CT scan of the brain done to rule out an infarct was normal. The diplopia persisted for over 4 weeks while the patient remained compliant with her medications despite the side effects. The symptoms persisted even when her tacrolimus dose was reduced and repeat levels were between 4 and 5 ng/mL. 3 weeks after onset of diplopia she was switched to low dose CyA in anticipation that similar side effects may not be observed. Trough CyA levels were 44 ng/mL and 59 ng/mL on two occasions but her symptoms did not resolve. A consultation with neuro-ophthalmology was sought and the patient was instructed to discontinue CyA. The symptoms completely resolved 6 days after stopping CyA. Her nephrotic syndrome remains in remission till date. |
The patient was a 24-year-old male who came with symptoms of shortness of breath and cough. The patient underwent ECG, chest radiology, and ultrasound and was found to have right pleural effusion while his ECG was normal. Early vital signs of the patient at the time of visiting the hospital were BP: 125/80, PR: 105, T: 37.4, RR: 32, SpO2 = 96%. The history taken from the patient revealed that he had no underlying disease and no history of heart diseases in his family. Moreover, there was no history of inflammatory diseases in the patient or his family. The patient’s BMI was within the normal range, and he had a history of working in harsh conditions under high pressure. After admission, the patient underwent echocardiography ordered by a cardiologist, where according to the physician, evidence of aortic dissection was observed. For a better diagnosis, echocardiography through the esophagus was performed on the patient which confirmed the evidence of dissection. Since aortic dissection is a life-threatening emergency, an urgent aortic CT angiography was requested and performed as ordered by the patient’s physician, and aortic dissection of type A was confirmed in the patient. Immediately after the diagnosis, necessary arrangements were made for open-heart surgery and the patient was prepared for surgery. Blood samples were sent to a laboratory for the required tests (i.e., antibody, CBC, and similar tests), and to diagnose inflammatory processes and related syndromes tests such as RF, CRP, and IgG, among others, were used. The patient was immediately taken to the operating room for urgent surgery after obtaining informed consent from him and his family. It should be noted that blood samples were taken by professional nurses and sent to the laboratory under special conditions to reduce the error rate. To establish a cardiopulmonary pump for the patient, a number 19 canola was applied and the right femoral artery was used to cannulate the patient. After implanting a cannula in the femoral artery, the patient underwent sternotomy, his pericardium was opened, and his right atrium was cannulated. Then the patient went on the pump and his pump timing started. After performing sternotomy on the patient, we found that the dissection started from the aortic annulus and continued in the ascending aorta before the aortic arch. The size of the false lumen in the aneurysm was about 7–8 cm and led to severe aortic valve failure. After the start of the cardiopulmonary pump, the ascending aortic dissection was opened and imported into the right coronary artery and left main artery through a direct cannula and the patient received cardioplegia. The aortic annulus, ascending aorta, and aortic valve were completely removed. The composite graft with metal aortic valve was number 23 which was anastomosed to the aortic annulus, and the right coronary artery and left main artery were anastomosed to the composite graft. The distal composite was also anastomosed to the aortic arch. Three number 32 chest tubes were embedded in the mediastinum area and the right and left pleura. The patient had no arrhythmias during the surgery. The surgery lasted for about 6 h and the patient’s cardiopulmonary pumping took about 200 min. It should be noted that the patient received two units of blood during the surgery. After surgery, the patient was transferred to the cardiac surgery ICU with complete cardiac and respiratory monitoring and was placed under respiratory monitoring using a ventilator and a hemodynamic monitor upon arrival. The patient underwent intensive care in the cardiac surgery ICU and was constantly monitored for acute post-surgery side effects such as bleeding and cardiac tamponade. During the first 12 h of admission to the cardiac surgery ward, the patient’s mean arterial pressure was maintained between 65 and 70 mm Hg to prevent bleeding, and his systolic pressure was between 80 and 90 mm Hg. Moreover, due to the long duration of the surgery and the high possibility of bleeding, the patient was anesthetized for about 8 h using sedative drugs to ensure the post-surgery conditions. It is worth mentioning that during these 8 h, the patient’s pupils had normal reactions to light and were of normal size. The patient was constantly monitored. ABG was monitored and adjusted every hour. The patient’s electrolyte levels were also corrected. In the ward, the patient underwent chest radiography wherein no evidence of bleeding, tamponade, and pneumothorax was observed. The patient was hospitalized and remained under medical care in the cardiac surgery ICU for five days and his medication was carefully administered. After his conditions were stabilized, the patient was transferred to the post-cardiac surgery ICU ward. The patient was discharged from the hospital one week after the surgery and returned to the outpatient department (OPD) one week after his discharge. After visiting the OPD, the patient underwent CT angiography and echocardiography and his condition was stable. Moreover, his aortic valve, the ascending aorta, and right and left main coronaries worked well and the patient’s heart contraction was satisfactory with EF: 55% (Fig. ). |
A 47-year old female with no significant past medical history presented with a thyroid mass and a foreign body feeling when swallowing. The patient denied palpitation, dysphoria, fever, insomnia, cough, dyspnea, breathlessness, hoarseness, or other symptoms. The Doppler ultrasonography showed a hypoechoic solid mass about 67 × 40 mm with blood flow signals posterior to the lower border of the right lobe of the thyroid. Another hypoechoic solid mass about 70 × 43 mm with blood flow signals posterior to the lower part of the left lobe of the thyroid gland was also noted. Multiple enlarged lymph nodes with the largest ones measuring 18 × 6 mm in the right neck, and 15 × 5 mm in the left neck, were also detected. She then received a computed tomography (CT) scan of the neck and thorax that revealed a 85 × 54 mm hypodense-to-isodense space-occupying lesion in the posterior superior mediastinum, one solid mass in the right lobe, and another solid mass in the inferior posterior part of the left lobe of the thyroid. The lesions showed slight enhancement with a contrast-enhancement scan and caused compression of the trachea, esophagus and cervical vessels (, left panel). An esophagogastroscopy also revealed stenosis of the esophagus 15–20 cm distal to the upper incisors. Two months following the initial presentation, the patient underwent surgical treatment with right and left thyroidectomy, upper mediastinal tumor resection, left recurrent laryngeal nerve exploration, right recurrent laryngeal nerve anastomosis, and tracheotomy. During the surgery, the thyroid gland was found to be adherent to the surrounding tissue and a tumor measuring about 10 × 8 × 6 cm was present within the dorsal part of the right lobe of the thyroid gland was noted. Specifically, the tumor was between the trachea and esophagus, but the border of the tumor was indistinct. The tumor involved the tracheal membranous wall, the upper esophagus, and the right recurrent laryngeal nerve. The left lobe of the thyroid gland was not directly involved by the tumor. Pathological investigations demonstrated an ACC of the upper mediastinum involving the left lobe of the thyroid gland, the tracheal cartilage, and the adjacent muscles. The immunohistochemical staining profile was as follows: Alcian blue (AB) (+), Periodic Acid Schiff (PAS) (–), CD56 (–), Syn (–), Calponin (+), smooth muscle actin (SMA) (+), P63 (+), epithelial membrane antigen (EMA) (+), cytokeratin (CK)5/6 (+), CK8/18 (+), CD117 (+), thyroid transcription factor-1 (TTF-1) (–), thyroglobulin (Tg) (–), chromogranin A (CgA) (–) (). Perineuronal invasion was also noticed. The mass in the left lobe of the thyroid gland turned out to be a nodular goiter.\nPostoperative CT scan showed changes in the area of the thyroid, with thickening of the upper thoracic esophageal wall, but no obvious abnormalities elsewhere in chest (, middle panel). Other examinations including abdominal CT and bone scintigraphy were negative. The thyroid profile was as follows: free T3 3.27 pmol/L, free T4 10.76 pmol/L, TSH 48.400 μIU/mL, anti-Tg 12 IU/mL, anti-TPO 6 IU/mL. The patient was supplemented with oral thyroid hormone. The patient then received one cycle of chemotherapy in the form of 75 mg/m2 of Cisplatin and 75 mg/m2 of Docetaxel but were canceled because of intolerable gastrointestinal side effects. Since the tumor involved the tracheal cartilage and showed perineuronal invasion, radiation therapy was proposed. The patient underwent postoperative intensity-modulated radiation therapy (IMRT) with a prescribed dose of PTV-GTV of 70 Gy, PTV-CTV1 of 60 Gy, PTV-CTV2 of 54 Gy in 31 fractions (1 fraction per day, 5 fractions per week; ). She experienced grade II leucopenia, but the treatment was otherwise well tolerated.\nThe patient returned for post-treatment follow-up visit 3 months after the radiation therapy. CT scan showed no obvious changes compared with previous scans. The patient has undergone scheduled follow-up with CT scans as routine imaging since then.\nLocal control was quite satisfactory and the patient was free of local recurrence (, right panel). However, multiple tiny nodules appeared in both lungs 2-years later following the initial presentation showing very slow increase in size. However, she declined any further treatment. The patient is still under follow-up 3-years after initial treatment and is not reporting any discomfort. |
This report presents a 29-year-old G1P0 patient who presented to the obstetrics emergency room with severe abdominal pain of the left lower quadrant with signs of acute abdomen and knowledge of a 7 weeks’ intrauterine pregnancy. The patient was evaluated by the obstetric team upon arrival and her heart rate was 99 bpm, and blood pressure was 90/50 mm Hg. A transvaginal ultrasound was performed, which revealed a 7 weeks and 2 days estimated gestational age intrauterine pregnancy with fetal cardiac activity and was considered viable. Both of the patient’s fallopian tubes were dilated and showed evidence of a hydropic fallopian tube, which was evaluated as evidence of former pelvic inflammatory disease. Additionally, a 10.8-cm ectopic pregnancy was positioned on the left side with cardiac activity with an estimated gestational age of 7 weeks and 1 day. Blood products and abdominal free fluid were also detected. At the time, the patient’s hemoglobin and hematocrit levels were 12.2 g/dL and 35.4% respectively, and the other initial blood parameters checked were within the normal range (, ).\nThe patient’s condition was stabilized with a saline infusion and informed about her clinical condition. The patient expressed a desire to preserve the intrauterine pregnancy. Under close monitoring, the patient was admitted to the gynecology department and was given full information about her condition including the risk of miscarriage and more extensive surgery. After giving consent for the recommended surgery, the patient was taken to the operating room.\nThe patient was placed in the lithotomy position under general anesthesia. Following the sterile covering of the area, posterior colpotomy was used to enter the abdomen. Approximately 200 cc of blood containing clots was drained. The right and left ovary and right fallopian tube were observed in normal anatomic position and nature. The left tube was visualized, which had an approximately 5 cm ectopic mass with active bleeding. After visualizing using a camera, salpingectomy was performed on the left tube using a bipolar energy device. Afterwards, the pelvic area was examined and no bleeding was seen. The colpotomy was closed using sutures. There were no complications.\nFollow-up ultrasound was performed in the post-operative 9th hour. The intrauterine pregnancy was detected as viable. Two hundred milligrams of natural progesterone was ordered by vaginal route once the pregnancy was confirmed as viable. On postoperative day 1, the patient’s clinical condition and hemoglobin levels were stable and she was discharged. The patient was told to come for a follow-up examination.\nOne week after discharge, she presented for postoperative follow-ups and screening of the intrauterine pregnancy. Ultrasound showed a live fetus at 8-weeks 0-day gestation based on crown-rump length. |
A 59 year-old Caucasian female with past medical history of migraines, presented to our emergency department as a transfer from another facility for further management of a non-ST elevation myocardial infarction. In the emergency room she presented with notable anterolateral T wave inversions on ECG as well as elevated troponin I of 2.5 ng/mL (reference range: 0.000–0.039 ng/mL). vThe patient was having continued chest pain at time of arrival and stated that she experienced her first acute episode of chest pain 1 day earlier, occurring at her home, while at rest. She stated that earlier in the day she was experiencing a migraine and took her home medication of Treximet 85/500 mg with good relief of her symptoms. The patient stated that she occasionally gets migraines and on occasion uses Treximet to relieve her symptoms, however has not had to take the medication in quite some time. The patient claimed that a few hours after her migraine had resolved she began to experience non-radiating, left sided, substernal chest pressure with associated paresthesia in the lower extremities and some non-descriptive nausea. This prompted her to seek medical attention at a local urgent care facility. She was seen and assessed by a physician and had an ECG performed which demonstrated no significant issues so she was discharged home with an antacid and told to follow up with her primary physician as an outpatient. The patient stated that the chest pain never really dissipated however she went to sleep that night without issues. The next morning, she woke up with continued chest pressure, yet, decreased in quality from the previous day. She went to work that day and while talking to her coworkers she immediately felt worsening of her chest pressure and was taken to a local hospital, without catheterization capabilities, where she was noted to have new ECG changes and elevated troponins. Upon transfer to our facility she was given 325 mg of aspirin by mouth and started on IV heparin and nitroglycerine. ECGs at our hospital showed progression of her anterolateral T wave inversions (see ) therefore it was deemed necessary to take the patient for urgent coronary angiography with possible percutaneous intervention to evaluate for obstructive coronary artery disease. The patient was taken to the cardiac catheterization laboratory and successfully underwent a left heart catheterization with selective left and right coronary angiogram (see ) which did not reveal any significant obstructive atherosclerotic coronary artery stenosis; except a non-occlusive lesion in left circumflex artery which was not significant enough for any intervention and less likely to cause our patients acute symptoms and echocardiographic findings. Left ventriculography performed at the time demonstrated notable apical ballooning and hypokinesis of the apex with normal functioning basal, anterior, lateral, and inferior walls (see ) as well as an ejection fraction estimated at 30% consistent with TCM.\nThe patient had no post procedural complications and chest pain had since resolved. Her echocardiogram post-cardiac catheterization reported a left ventricular ejection fraction on 30% with hypercontractility of the basal wall and akinesis of the mid to distal anteroseptal wall, anterior wall, mid to distal inferior and inferolateral walls, and apex consistent with TCM (see ). Patient was placed on metoprolol succinate 25 mg once a day, advised to avoid Treximet or other migraine medications if possible, and was informed to follow up in the office as an outpatient for a follow up echocardiogram. Patient remained symptom free and had a follow up echocardiogram done 3 months post discharge, which demonstrated resolution of her wall motion abnormalities and improvement of her ejection fraction to 55%. |
A 15-year-old previously healthy boy was elbowed in the neck during a football game. He continued to play after the incident; however, later on that day, he started having headache which prompted his visit to the Emergency Room. He had no other symptoms, and his neurologic exam was normal.\nA CT scan of the head was done, and it was suspicious for a right internal carotid artery (ICA) dissection at the level of the skull base involving the extracranial portion of the artery. MRI and MRA of the brain and neck were subsequently performed and confirmed the diagnosis of a dissection resulting in minimal narrowing of the lumen of the ICA (Fig ). The patient was admitted to the hospital for observation, and was started on aspirin 81 mg daily. Work-up for collagen vascular disorders which may have predisposed him to carotid dissection was negative. He remained neurologically and was discharged home.\nAt 1 month of follow-up, he was asymptomatic with significant improvement of his headache. Follow-up MRI and MRA were performed and showed an unexpected progression of the dissection with high-grade stenosis of the true lumen of the right internal carotid artery (Fig ). The finding was confirmed with conventional angiography. Clopidogrel was added in addition to aspirin. After consulting the patient and his family, the patient underwent stenting of the right ICA at 2-month postinjury (Fig ).\nUsing a micropuncture set and under fluoroscopic guidance and strict sterile technique a 6 French femoral sheath was inserted into the right common femoral artery. ACT was kept between 250 and 300 with systemic anticoagulation.\nThe pretreatment internal carotid arteriogram demonstrated a persistent spiral dissection of the distal cervical segment of the right internal carotid artery with associated pseudoaneurysm. Following the diagnostic angiogram, and via a right internal carotid artery a small microcatheter was advanced over the wire through the spiral dissection. Over an exchange wire a self-expanding nitinol stent (Precise/Cordis), which measured 5 × 40 mm, was successfully deployed in the cervical segment of the left internal carotid artery across the spiral dissection.\nFollow-up arteriogram shows good apposition of the stent to the arterial wall with restoration of the luminal diameter with diminished filling of the aneurysm and stagnation of the contrast within the sac of the aneurysm. There is no evidence of platelet aggregation, or major branch occlusion.\nAt 6-month post-stenting, the patient remained asymptomatic. Angiogram showed complete resolution of the dissection without residual stenosis and with evidence of endothelialization of the stent (Fig ). Clopidogrel was stopped, and he was maintained on aspirin. The patient will be followed clinically and with annual CT angiogram. We would also stop aspirin if the result of CT angiogram is satisfactory without wall abnormalities at one year post-stenting. |
Our second patient was a 45-year-old right-handed Caucasian man with a history of traumatic brain injury in 2008 resulting in moderate cognitive impairment and left temporal partial epilepsy. He also has history of depression since he had traumatic brain injury. He was seen in our epilepsy clinic because of an increased frequency of seizures. The patient was on high doses of three anticonvulsants and continues to remain intractable. According to his mother, he has been having spells on a daily basis and they have to call the ambulance to take him to the hospital. The spells are described by the mother as putting his hands around his head and staring forward for several minutes. During that time, he also becomes unresponsive and this is followed by shaking of the upper extremity. The events may occur and last for about 3 to 5 hours or sometimes most of the day and they have to take him to the emergency room several times a week.\nHis magnetic resonance imaging demonstrated a significant decrease in signal throughout the left hemisphere, inferring compromised white matter integrity.\nThe patient was admitted to the epilepsy monitoring unit twice for localization and characterization of seizures secondary to epilepsy and spells of unclear etiology secondary to PNES. We were able to capture the patient's typical spells characterized by unresponsiveness and jerking of the upper extremities not associated with any EEG correlate. The diagnosis of PNES was discussed with the patient and his parents. The patient had an abnormal EEG with potential epileptogenicity in the left temporal region secondary to brain injury; however, the current spells were predominantly PNES. Since his EEG revealed no significant epileptiform abnormalities, the Keppra was discontinued during this admission, and he was sent home on low dose of gabapentin and Topamax for headaches. The study was reviewed with the patient and his parents. Follow-up visits were also arranged with psychiatry which significantly reduced the emergency room visits and inpatient hospital admissions. |
A 94-year-old female was admitted to our hospital after she fell and sustained an anterior pelvic ring fracture (Figure ). She was hospitalized for mobilization. Over the past 18 years she had been treated with anticoagulation therapy with marcumar due to atrial fibrillation, 13 years ago subsequently a cardiac pacemaker was implanted. In the first days in hospital mobilisation was painful but with a walker possible. In the early morning of the fourth day after admission she developed severe pain in the thigh. Clinically the thigh was swollen but the foot pulses were well palpable. Because of persisting severe pain and the suspicion of a deep vein thrombosis a CT-scan of the thigh and the pelvis was performed. In the further course the patient complained of a partial loss of sensation of her left leg and weakness of her shank muscles, following the innervation area of the ishiadic nerve. The CT-scan showed an extensive haematoma expanding abroad the adductor muscles in the medial compartment and in the posterior compartment (Figure &). The compartment of the M. rectus femoris was not involved. Immediate surgery was performed with a complete decompression of the compartments of the thigh as well as the draining of the haematoma and wound covering with epigard (Othomed, Vienna, Austria) (Figure &). On the second postoperative day a revision for draining of the refilled haematoma was performed. Finally five days after the initial treatment during the second revision complete skin-closure was performed strainless. In the postoperative course the patient recovered quickly and the sensomotor deficits resolved completely however she developed partial skin necrosis along the approach in particular in the popliteal fossa. Subsequently these necrosis were resected and the skin was closed again strainless. Only in the popliteal foassa a skin graft from the ipsilateral posterior thigh was needed. The skin graft healed. In the further course of the hospital stay the patient again recovered quickly and was mobilized under physiotherapy control. Fifty-seven days after the initial trauma the patient was dismissed and transferred to rehabilitation center for further mobilization. At the latest follow up one year post operatively the patient is mobile with full weight-bearing and regular soft tissue condition.\nA full-blown compartment syndrome in the thigh is a rare clinical occurrence. This is mainly due to the fact that the three muscle compartments (Figure ) in the thigh can compensate much higher volumes than the four compartments below the knee []. Not only the fascia seems to be more dilative also the thigh compartments are partly open to the pelvis explaining the higher compensation rate for increasing intracompartmental volume [,]. The small number of occurring cases makes scientific randomized prospective studies with bigger numbers difficult. However the spectrum of reported cases with different etiologies is wide: Deep venous thrombosis [], vein catheriztion [], vessel aneurysm [] the complete spectrum of femoral fractures [], total hip and knee replacement surgery, intramedullary nailing [,], gunshot and stab wounds [], sport trauma [,,-], traffic accidents [,,], external compression of the thigh [,], crush injuries [,], as well as severe blunt tissue trauma []. Mithoefer [] declares that in 46% of the cases a traffic accident is the cause for a thigh compartment syndrome. Holbein [] proclaims that regarding the literature the femur fracture is the most common cause for a thigh compartment syndrome. Kladny [] numbers this risk with 1-2%. An open fracture or wound will not safely secure the leg from a compartment syndrome [,]. Multiple trauma patients seem to have a higher risk to develope a compartment syndrome because of the concurrence of different clinical conditions. High-energy blunt trauma, external compression, systemic hypotension, vascular injury, and coagulopathy in those patients may assimilate and thus lead to a compartment syndrome []. According to Schwartz a patient with an isolated injury to the thigh without any of the above mentioned risk factors has therefore a lower risk for the development of a compartment syndrome [].\nIn cases were the patient is unconscious at the intensive care unit a compartment syndrome can stay unrecognized possibly causing devastating outcome [], as the patient can not express main symptoms like disproportional pain, paraesthesia or even paralysis. Only the palpatoric tension of the affected muscle is detectable. These cases must be taken into special account and the measurement of the compartment pressure has an important role in those cases for the diagnostic. Regarding the common literature addressing this topic the measurement of the intracompartmental pressure is the gold standard for the diagnosis []. However the reliability of these measurements is questionable, as especially in multiple traumatized patients the systolic blood pressure supported by catecholamines can pretend a stable hemodynamic situation neglecting a possibly severe disregulated microcirculation []. In addition there is no consensus in the literature about a specific intracompartmental point pressure or a rule regarding the diastolic blood pressure or the arterial middle pressure, clearly indicating the need of fasciotomy []. In our case we initially suspected a deep vein thrombosis and not a compartment syndrome. Therefore we decided for a CT scan. After the diagnosis surgery was performed immediatly and no further diagnostic was performed. Usually suspecting a compartment syndrome calculating the local perfusion pressure by measuring the the mean arterial pressure and the intramascular pressure is the gold standard.\nBecause of the wide variety of conditions in patients with huge differences in the physiological strength (young men in a car accident versus a 94-year-old women with a stable pelvic fracture) and the very limited numbers of cases with a compartment syndrome of the thigh explains why it is impossible to identify strict criteria for the need of a fascitomy. However the literature agrees that the damage caused by fasciotomy in a patient in which the tissue would not have become necrotic is far outweighed by the morbidity possibly associated by a full-blown compartment syndrome []. In a borderline compartment syndrome not only the intracompartmental pressure but close meshed checks of the sensormotor function of the affected leg as well as the typical clinical symptoms are essential for the indication of a fasciotomy.\nIn our patient the compartment syndrome developed with several days delay. This we hypothezised might be explained by the patient's clinical course. During the first days the patient was immobilized with bed rest. During remobilisation the vessels injured by the initial trauma started to bleed again, thereby creating a haematoma which followed gravity into the thigh. In our case the neurological symptoms resolved quickly after the decompression and no muscle necrosis were detected. However in this case the morbidity was caused by the high age of the patient resulting in multiple complications through a prolonged wound healing. Schwartz has already emphasized that infection is a further problem in the postoperative course in patients with a compartment syndrome. He reports local infection rate of 66% []. Although we were not able to detect any significant tissue necrosis in our patient we suppose that prolonged ischemia may have further compromised the microvascular perfusion in this old patient with already existing vascular sclerosis thus drastically increasing the risk of wound infection. |
A 2 year old male presented with chief complaint of a rash on his left thigh and left lower back for 7 days. The mother of the child initially noted the rash on the child’s left thigh after returning from a one week camping trip on a remote island in Canada. While the parents had gone hiking in the woods, the child was in day care during their trips and the parents said there was no point at which the child would have been exposed to any plants or other environmental exposures. After two days the mother took the child to an acute care clinic where the diagnosis of suspected contact dermatitis was made. The patient was treated with antihistamines and topical steroids. Over five days the rash progressed to involve the left lower back and appeared to spread outward from the initial area on the left thigh. It was at this time the patient presented to our emergency department for evaluation. The birth history was non-contributory. The patient was previously healthy, circumcised, and fully immunized through the age of two to include varicella. The patient was never exposed to chicken pox. The patient did not exhibit any signs of illness with the exception of a fever to 101.0°F orally the day prior to presentation. Upon further questioning, the father of the patient recovered from shingles the previous week but was currently asymptomatic. The child had normal vitals on physical examination. The rash was maculopapular with small vesicular lesions on the left anterolateral thigh () and left lower lumbar back () in the L3 dermatome that blanched to palpation. There were two areas of coalescing papules located at the right paraspinous region of the mid-lumbar spine. There were no oral or anogenital lesions. The palms and soles were not involved. The rest of the examination was unremarkable.\nPediatrics were consulted for evaluation of the rash which was suspected to be herpes zoster. After admission to the pediatrics ward, viral deoxyribonucleic acid (DNA) polymerase chain reaction (PCR) studies were obtained via blood samples of the child, which were positive for varicella zoster DNA. |
A 57-year-old right-handed woman presented with involuntary compulsive grasping with her right hand and intermanual conflict with her left hand. The typical symptom of involuntary compulsive grasping and grabbing with her right hand, which was associated with a frontal variant of AHS, manifested in this patient as an inability to release a comb or the hand of the examiner during the muscle strength examination (). In addition, the typical intermanual conflict associated with the callosal variant of AHS occurred while she combed her hair or ate food with her right hand; her left hand counteracted the purposeful movements of her right hand (). However, her hand movements were perfectly coordinated and smooth when she used her left hand for purposeful movement. She said that these symptoms, which were not under her control, affected her daily life. She presented to our hospital because she could not control her left hand. She had not received any medical treatment for this condition until she was admitted to our hospital. She had a history of hypertension. A history of epileptic seizures, cranial demyelinating pathologies, and other neurodegenerative diseases was not elicited. In addition to antihypertensive drugs (calcium channel blocker), there was no other medication history and no relevant family history. The general examination showed no obvious abnormalities, and her vital signs were normal. Neurologic examination revealed positive Babinski and Chaddock signs in her right lower extremity, and the muscle power of the right limbs was 4/5 grade. The grasp reflex was positive. The rest of the physical examination disclosed no abnormalities. Her National Institutes of Health Stroke Scale score was 3 points. Routine laboratory test results were normal.\nMagnetic resonance imaging (MRI) revealed that she had developed ischemic strokes in the corpus callosum and the left frontal lobe due to stenosis of the A4 segment of the anterior cerebral artery. MRI revealed cytotoxic edema in the left frontal lobe and the body of the corpus callosum. The infarct involved approximately two-thirds of the corpus callosum, as observed on the sagittal T2-weighted image (T2WI) (). Magnetic resonance angiography (MRA) revealed poor visualization of the A4 segment of the left anterior cerebral artery (pericallosal artery) and stenosis of the left middle cerebral artery ().\nTherefore, she was diagnosed with a mixed callosal–frontal variant of AHS. We considered the cause of her AHS to be ischemic strokes in the corpus callosum and left frontal lobe. The patient was administered routine aspirin antiplatelet therapy (100 mg QD) and verbal-cue rehabilitation to assist her in correcting the conflicting movements of her hands early for movement coordination. Whenever the patient performed an intentional movement with her right hand, such as combing her hair or eating with her right hand, her left hand interfered and prevented the movements of the right hand. This condition affected almost all movements that required coordination between her hands. Whenever she showed intermanual conflict in her left hand, her guardian verbally prompted her with instructions to complete the movement she wanted to perform correctly. Verbal cues were patiently used until the patient corrected her discordant movements herself. We found verbal cues a method to correct intermanual conflict in her left hand to be timely and effective, as can be observed in . With each repetition of verbal cues during rehabilitation training, the frequency of her intermanual conflict symptoms gradually decreased. The patient reported a confidence in herself and the training method and hoped for a speedy recovery. Her guardian said there was no rebound of the intermanual conflict symptoms as the frequency of verbal cues decreased. After 3 months, the intermanual conflict in her left hand disappeared as did the grasp reflex of her right hand. The movements between her hands became coordinated and orderly. Her guardian reported that her left hand no longer interferes with her right hand while she combs her hair, eats, or puts on clothes with her right hand. Her hand movements became as smooth and coordinated as those before symptom onset (). At the follow-up visit, after the strength test, she successfully released the hand of the examiner voluntarily instead of holding it tightly. Irrespective of whether she combs her hair with her left or right hand, the movements are coordinated and orderly, as can be seen in .\nShe also had a symptom of constructional apraxia of the dominant hand. She could feel shapes, distinguish objects, and locate them, but she could not identify the stereostructure, which is characteristic of the callosal variant of AHS. For example, she could not correctly complete drawing a clock and imitate a square schematic to draw a square (). On the return visit of the patient, she still could not correctly identify the stereostructure (). However, she has made some progress after a period of recovery.\nThe compliance of the patient to the intervention was good, and there was effective and timely correction of her motor coordination. The patient also showed apathy because of the discordant movements of the hands. Therefore, we assessed her compliance with this intervention based on her acceptance and the Modified Apathy Evaluation Scale (MAES) scoring scale. Her frustration lessened as the training progressed, and the MAES score gradually decreased from 20 to 8. This method of rehabilitation requires no special training, as long as the patient and guardians patiently cooperate with each other. There were no adverse or unexpected events during the intervention.\nAt the same time point, the sagittal T2WI and axial T1 fluid-attenuated inversion recovery (FLAIR) image showed obvious improvement in the cytotoxic edema of both the left frontal lobe and body of the corpus callosum (). Diffusion-weighted imaging and apparent diffusion coefficient scans showed no limitations of diffusion (). Despite improvement of the lesion in the left splenium of the corpus callosum, the lesions in the left frontal lobe and the body of the corpus callosum were persistent and more remarkable than before ().\nAll procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional research committee and with the Helsinki Declaration (as revised in 2013). Moreover, this study involving human participants was reviewed and approved by the ethics committee of the First Hospital of Jilin University. Written informed consent was obtained from the patient. |
We present the case of a 45-year-old woman who presented to the outpatient department with a history of right-sided abdominal pain and swelling. She first noticed the swelling eight months ago, and it had gradually progressed in size. The patient reported that the swelling increased in size during menses and was interfering with her daily activities. The associated pain was moderately improved after the use of non-steroidal anti-inflammatory drugs. However, there were no changes in the overlying skin and she reported no history of bowel or bladder symptoms.\nThe patient underwent a cesarean section two years before the presentation due to fetal distress. Otherwise, she did not have any remarkable past medical history. She was not taking any medications. Her menstrual periods were regular and were not associated with menorrhagia. She did not have any prior history of gynecological conditions.\nPhysical examination revealed a well-defined firm mass to the right of the midline. The mass had a smooth surface but limited mobility after abdominal wall muscle contraction, suggesting an infiltration of the underlying muscular structures. Basic hematological and biochemical laboratory investigations were within normal limits.\nIn light of the aforementioned clinical information, a contrast-enhanced abdominal computed tomography (CT) was planned for further characterization of the abdominal lesion. The CT scan demonstrated a large lobulated mass with heterogenous enhancement, measuring 17 × 15 × 10 cm and infiltrating the underlying muscular structure, confirming the diagnosis of abdominal wall endometrioma. The mass lesion was noted to displace adjacent structures.\nThe patient underwent surgical resection of the abdominal wall endometrioma along with a part of the underlying rectus abdominis muscle. It was noted that the endometrioma had risen at the site of the previous scar of the cesarean section. There was minimal blood loss. The postoperative course was uneventful. The patient was discharged seven days after the operation. The patient was followed up in the outpatient department for six months and reported major satisfaction and complete resolution of her symptoms. |
Ms N was a 36 year-old married lady who was diagnosed at birth on bloodspot screening. She adhered to a low Phe diet for the first 7 years of her life then ceased dietary control. She recommenced on diet at age 35 due to symptomatic PKU affecting her cognitive functioning and mental health. Ms N had a long history of mental health difficulties starting in childhood. She had difficulties regulating her mood and had episodes of depression. She had her first panic attack in her mid-teens, along with generalized anxiety symptoms and infrequent self-harming behaviors. She had been under psychiatric care since her teenage years and had several psychiatric admissions. She had worked in a variety of semi-skilled roles in a part-time capacity, but had found it difficult to maintain employment due to fluctuating mental health. She saw a psychologist on a regular basis and had been on pharmacological treatment with SSRIs since her early 30s, with some improvement. She reported significant cognitive limitations, including difficulties with attention and concentration, memory, planning, and organization and slowed mental processing. These difficulties had affected her ability to maintain productive employment, leading to feelings of inferiority and low self-esteem. Baseline neuropsychological assessment prior to resuming a low Phe diet revealed that Ms N was of average intelligence. However, she demonstrated moderate impairments on tasks of divided attention and psychomotor speed and severe impairments on task of planning, organization, and self-monitoring. After 12 months of good dietary control (reducing Phe from ∼700 to <300), Ms N had made statistically significant improvements on tasks of psychomotor speed (from 9 percentile at baseline to 63 percentile on diet), planning and organization (from <0.1 percentile at baseline to 77 percentile on diet), divided attention (from 9 percentile at baseline to 50 percentile on diet), and self-monitoring (from 1 percentile on diet to 37 percentile on diet). She also showed a significant regression in white matter lesions (). Her depression and anxiety symptoms also improved; however, they did not fully resolve and she benefitted from the introduction of escitalopram, initially at 20 mg but ultimately required a dose escalation to 40 mg. |
A 67-year-old male had presented to Dermatology OPD of our hospital 8 months back with a history of oral erosions for 2 months and cutaneous erosions for 1 month and was diagnosed as pemphigus vulgaris (). The patient was advised to take an oral steroid 60 mg daily along with other supportive treatments. In spite of proper counselling of the prognosis and long-term follow-up of pemphigus, he was taking prednisolone on and off and was unable to come for the follow-up because of the COVID-19 pandemic.\nThe patient presented again 5 weeks before the admission with multiple erosions that extended to the anterior chest and was advised to continue prednisolone 50 mg once daily, azathioprine (100 mg once daily), doxycycline (100 mg once daily), and nicotinamide (500 mg daily) for 5 weeks.\nHowever, he failed to show satisfactory improvement (), and his swab for bacterial culture and sensitivity revealed Pseudomonas aeruginosa sensitive to piperacillin; hence, he was admitted to the dermatology ward and started on the same antibiotic and intravenous, dexamethasone 8 mg once daily along with skin care. After receiving these medications for 2 weeks, once the pseudomonas infection resolved, dose of dexamethasone was increased to 16 mg over the next 2 weeks, and the patient got significant improvement. However, 1 week after initiating steroid dose escalation, the patient developed few monomorphic haemorrhagic crusts on the cheeks overlying the pre-existing lesions (), which progressed over the next week to involve bilateral cheeks, malar region, and bridge of the nose with few lesions in the forehead, bilateral ears, and chest. These lesions were associated with extensive cutaneous pain and myalgia ().\nAlthough he denied past history of herpes infection, his HSV-1 IgM and IgG also came out to be positive and hence was diagnosed as KVE. Then, he was immediately started on oral acyclovir 400 mg thrice daily, and dexamethasone was tapered rapidly to 9 mg from 16 mg once daily over a week (). Further ophthalmological consultation was done, and herpes keratitis was ruled out. Within 1 week of the treatment, the crusts decreased significantly, and treatment was continued. The erosions also healed rapidly thereafter.\nHis comorbidities were type 2 diabetes mellitus, grade 2 benign prostatic enlargement, right nephrolithiasis, and osteopenia.\nOnce there was 90% resolution in the haemorrhagic crust, the patient was discharged on 60 mg prednisolone and oral acyclovir 400 mg thrice daily (). Oral acyclovir was continued with the same dose for a total of 4 weeks until almost 100% improvement was achieved (). After that, monthly tapering of the dose of oral prednisolone was done, and the patient was instructed to follow up via teledermatology consultation during the COVID-19 pandemic as he came from remote hilly area. |
A 69-year-old male patient visited the hospital complaining of left sacral pain and radiating pain in the right lower extremities, which had occurred starting 5 years ago. There were no abnormalities in the past medical history of the patient, and the sacral pain had worsened during the last two years and it became more severe when standing or walking for about 10 minutes. The patient was treated in a private hospital but there was no effect so he visited the orthopedics department of our hospital for treatment. The results of the MRI-enhancement showed hypertrophy of the lumbar facet joints, spinal stenosis with thickened yellow ligament between L2-3, L3-4, and L4-5, and a perineural cyst with the dimensions of 1 × 1.1 cm2 in the left S2 area (). The orthopedics department diagnosed the pain as a spinal stenosis so they performed conservative treatment by administering NSAIDs, limaprost, and steroids; however, the pain did not improve so the patient was referred to our department.\nWhen visiting our department, the patient was continually complaining of numbness and stabbing pain in the sacral area of VAS (visual analog scale) 5-6, and complained that the symptoms worsened when standing for about 10 minutes. In addition, when walking for long periods, there was numbness and a pulling pain in the right leg. There were no abnormalities in the straight leg raising test, and there were no other symptoms other than pain in the physical and neurological examination. Since the patient complained of the pain continually and regularly occurring only in the left sacral area and radiating pain occurring in the right, the pain was believed to be caused by a perineural cyst in the S2 area together with the pain caused by the spinal stenosis. In addition, since the patient had received a facet joint block in a previous private hospital with no effect, the possibility of degenerative change such as degenerative arthritis was low. Especially since the chief complaint of the patient was continuous numbness and stabbing pain in the left sacral area, which was coincidental with the location of the perineural cyst according to the dermatome, it was decided for the differential diagnosis to first perform a caudal peridurography to confirm the cyst and perform a caudal epidural block. After having observed changes in the symptoms, treatment for spinal stenosis was supposed to perform.\nIn the operating room, the patient was put in a prone position while showing stable vital signs. Local infiltration was performed with 1% lidocaine on the needle insertion site. With the guidance of a C-arm, a Tuhoy needle was inserted, and contrast medium was injected with continually contrasting of the epidural space. After 3 ml of contrast medium were injected, a perineural cyst was observed so the contrast medium was discontinued (). The contrast results showed a filling defect of the epidural space caused by a perineural cyst in the left S2 area (), and 8 ml of 0.75% mepivacaine (Mepivacaine HCl Injection 2% Hana®, Hana Pharm., Korea), triamcinolone acetonide (TAMCETON INJ®, Han All Bio Pharma, Korea) 20 mg, and hyaluronidase (H-LASE®, Kuhnil Pharm., Korea) 1,500 IU were prepared and slowly injected. The C-arm was continually used to confirm the diffusion of the medication, and the medication was inserted while observing that the contrasted area around the cyst became weaker as the contrast medium was diluted by the medication. After injecting 6 ml of medication and the authors felt that the medication had sufficiently spread around the cyst and the injection of medication was discontinued. The medication was injected slowly; thus, it only spread around the perineural cyst and did not spread into the epidural space, and special caution was taken to insert only a small amount of medication so the pressure would not rupture the cyst.\nAfter the procedure, the patient showed improvement in his symptoms with the left sacral pain disappearing and only a slight numbness left. One month after the procedure, the patient again visited the hospital complaining of numbness and radiating pain in the right lower extremities of about VAS 5. According to the physical examination, there was pain in the L5 dermatome; therefore, the pain was suspected to be caused by a spinal stenosis; thus, a selective transforaminal block was performed on the right L5. Afterwards, the radiating pain in the right lower extremities had improved and the patient was able to perform daily activities without discomfort. |
A 23-year-old male patient was referred to the postgraduate clinic of the department of Endodontics, for opinion and management of left mandibular molars. The patient complained of recurrent pain over the left mandibular molars for a period of 2 months. His medical history was not contributory. At the time of intraoral examination both the left mandibular molars revealed caries and were extremely sensitive to percussion. Radiographic examination of the affected teeth revealed an abnormal tooth anatomy with the left mandibular second molar with unusually long pulp chamber which had no constriction at the cemento-enamel junction, two short roots were seen at the furcation area in the apical third, root resorption at the apical end. Radiographic examination of left mandibular second molar revealed deep dental caries involving pulp with periapical changes []. Based on the clinical and radiographic findings, a diagnosis of hypertaurodontism with chronic periapical periodontitis accompanied by root resorption was given to mandibular left 2nd molar. The left mandibular first molar was diagnosed with combined endo-perio lesion with chronic periapical periodontitis. Root canal treatment was advised for both the left mandibular molars accompanied by periodontal therapy.\nUpon approval of the patient the teeth were treated as in case 1. The pulp chamber of 2nd molar was huge and the floor could not be visualised, which on further exploration in the apical furcation area revealed two divisions one on the mesial side and one on the distal side with wide apical foramina. The apical divisions were almost near the apex, to about 3 mm and they were separately cleaned and shaped using hand K-files till ISO size 30. Working length as well as biomechanical preparation was performed as in case 1. [] [] The AH plus sealer was then applied using a # 20 reamer along the canal walls. Obturation was then done with lateral compaction technique in the mesial and distal canal. After that, the elongated pulp chamber was obturated by thermoplasticized gutta percha using OBTURA 2. The final radiograph confirmed a well condensed filling of the canals []. The patient was reviewed after three months and was found to be asymptomatic. They were referred to the Department of Periodontia and Prosthodontia, for further management. |
The patient was a 40-year-old woman with myoma of the uterus. She was 172 cm tall and weighed 60 kg. She was not taking medication and her family history was unremarkable. She did not smoke, but she had a habit of consuming a small amount of alcohol a day. Laparoscopic hysteromyomectomy was performed under general anesthesia for treatment of uterine myoma. In laparoscopic surgery, we took pneumoperitoneum at 15 to 20 mmHg for one hour. Around 2:00 am on post operative day (POD) 4, sudden chest and back pain and bilateral lower limb paralysis appeared. The discharge schedule was canceled because it became impossible for the patient to stand on both sides. The obstetrics and gynecology on-duty doctor then requested our department to perform a closer examination.\nAt the time of the examination, chest and back pain had alleviated, and motor paralysis was also improving, with the patient able to kneel on the right side to an extent. However, she was unable to kneel on the left side and her manual muscle test (MMT) score was 2/5. About 1 hour later, headache appeared and a head CT scan was performed. This showed no abnormal findings, and headache disappeared after administration of acetaminophen. There were no abnormal neurological findings in the upper limbs, but increased deep tendon reflexes, hypoesthesia and muscle weakness were observed in both legs. These findings led to suspicion of thoracic spine disease. Since it was nighttime and paralysis seemed to be improving, cervical and thoracic spine MRI was delayed until the morning. This showed an abnormal finding from the cervical vertebra to the upper thoracic vertebra, and the patient was then formally transferred to the orthopedic department.\nUpon transfer, her consciousness was clear and blood pressure was 139/93 mmHg. The electrocardiogram showed sinus rhythm with no arrhythmia, and there was no abnormality in respiratory spirometry. No abnormalities were found in deep tendon reflexes of both upper limbs, but bilateral patellar tendon and Achilles tendon reflexes were enhanced. Muscle weakness was found predominantly on the left side. In MMT, the quadriceps femoris was 5/5 on both sides, but the tibialis anterior, extensor pollicis longus and flexor pollicis longus were 5/5 only on the right, and 2/5 on the left side. Warm pain sensation was decreased below Th4, and the right side showed a greater decrease of deep sensation than the left. The temperature pain sensation was lost below the right knee and had almost disappeared below the left knee. Poor urination and dysuria were also apparent. She couldn't feel the urge to urinate and defecate and was incontinent. So a bladder catheter was inserted into her. There were no abnormalities in blood biochemical tests or in the blood coagulation system.\nA chest X-ray showed no clear enlargement of the upper mediastinum. On CT of the thoracic spine, a high-intensity region was found on the right dorsal side in the dura in the upper part of Th1 to Th6. In sagittal thoracic MRI, there was a zonal change with a septum on the right dorsal side of the spinal cord dura mater from Th1 to Th6. In this region, the signal intensity on a T1-weighted image was equal to that of the spinal cord and higher than that of the cerebrospinal fluid (CSF) (Figure ), and on a T2-weighted image the intensity was higher than that of the spinal cord and lower than that of the CSF (Figure ). A stenosis was located at an upper Th3 level, and the thoracic spinal cord was compressed to the left ventral side, with moderate to severe spinal cord compression (Figure ).\nAfter transfer to the orthopedic department, we decided to perform conservative treatment if symptoms improved, and surgery if progression was observed. Three hours after the first examination, there was no significant change in MMT right 5/5 and left 2/5 below the tibialis anterior muscle, and no change in paresthesia. Six hours later, there was no change in sensory deficits. However, lower limb MMT had decreased to 3/5 on the right side below the tibialis anterior muscle, and the left was still 2/5. This progression of bilateral lower limb paralysis led to the decision to perform emergency surgery 14 hours after the onset of symptoms.\nA posterior approach was used to open Th1-5, and the Th2, Th3, and Th4 laminae were excised. Bleeding occurred from the dilated epidural venous plexus, but there was no epidural hematoma. A dark hematoma was seen under the dura at the level of the Th3 pedicle, with a bulge of the dura observed at the same site (Figure ). Incision of the dura revealed a hematoma with a subdural coating (Figure ). The hematoma was located in the right upper part of Th3, especially around the Th3 nerve root, and was mainly in the subarachnoid space. Hematoma at the Th2 to Th4 levels was removed by suction to the extent possible, and the dura was displaced to the ventral side and flattened. No clear bleeding source was found in or around the Th3 nerve root, and there was no tumor or arteriovenous malformation.\nImmediately after the operation, sensation improved in the trunk and thighs. However, warm pain sensation was lost in the area below the right L5 region. MMT for below the tibialis anterior muscle was 3/5 on the right and 2/5 on the left. Moreover, excretion was difficult, although there was an urge to urinate and a bowel movement. Selective spinal angiography was performed one week after surgery (Figures , ), but there were no obvious abnormalities such as vascular malformations. Four weeks after the operation, MRI of the thoracic spine showed disappearance of the hematoma, but there was a high-intensity region on the left side of the spinal cord at the Th3 level, accompanied by atrophy. In addition, T2-weighted images of the lower thoracic cord to the conical spinal cord area showed a low signal, suggesting hemosiderin deposition.\nParalysis of both lower limbs recovered over time, and the patient was able to walk with a brace on her left ankle four weeks after surgery, and to walk independently five weeks after onset. Defecation improved to a controllable level, but urination was not possible, despite awareness of the need to urinate. For this reason, a urethral catheter was placed. The patient was discharged to a convalescent rehabilitation hospital six weeks after onset with independent walking. At 12 weeks postoperatively, the patient was able to walk independently and MMT below the quadriceps femoris was 5/5 on the right and left sides. Deep sensation was slightly reduced on the left, but improved overall. The patient was able to urinate, but with slight decrease in urgency due to bladder-rectal disorder. Thoracic spine MRI of the spinal cord showed a high-intensity region at the upper Th3 level on a T2-weighted image, but the hematoma had disappeared (Figures -). |
A 48-year-old man visiting the Korea University Ansan Hospital presented with the symptoms of exertional dyspnea and cough. He had no specific past history or family history. Physical examination showed no lymph node enlargement. Chest radiography showed pleural effusion, pulmonary congestion, and cardiomegaly. In a transthoracic two-dimensional echocardiography, a large amount of pericardial effusion and diffuse cardiac wall thickening were observed (). Cytologic analysis of the pleural fluid showed scattered lymphocytes only. We performed cardiac magnetic resonance imaging (MRI) for differential diagnosis of myocardial infiltrative diseases such as cardiac amyloidosis. MRI showed diffuse cardiac wall thickening and delayed enhancement in all chambers (). However, there was no lymphadenopathy or a mass lesion in the MRI images (). The patient underwent a myocardial and pericardial biopsy. However, the myocardial biopsy showed myocyte hypertrophy, and the pericardial biopsy showed chronic inflammation with fibrosis and the Congo red staining was negative. We could not identify a definite cause of the pericardial effusion; hence, the patient underwent conservative treatment and received outpatient follow-up.\nAfter 10 months, he was readmitted for recurrent massive pericardial effusion. Chest computed tomography (CT) revealed an infiltrative soft tissue mass in the anterior mediastinum and diffuse thickening of all ventricular and atrial walls (). We suspected the mediastinal mass to be a lymphoma and performed a biopsy. The biopsy results of the mediastinal mass showed chronic inflammation with fibrosis. Immunohistochemical staining was positive for CD45, but owing to the lack of specimen tissue, we could not perform more tests. We considered repeating the biopsy by performing a thoracostomy. However, the patient refused to undergo further evaluation and aggressive management.\nClinically, we suspected a diagnosis of fibrosing mediastinitis, and glucocorticoids were used empirically. We prescribed prednisolone 60 mg daily for 1 month, which was tapered to 15 mg per day within 5 months. After tapering of prednisolone, we maintained prednisolone 15 mg daily.\nTen months later, the patient complained of a nontender mass on the right side of the neck (). We performed an ultrasound-guided neck mass biopsy. Microscopic examination revealed a diffuse proliferation of large atypical lymphoid cells. Immunohistochemistry demonstrated a large lymphoid neoplasm, which was positive for CD45, CD79a, and CD20. Finally, the patient was diagnosed with primary cardiac diffuse large B cell lymphoma (DLBCL). He commenced chemotherapy with rituximab and CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone). After the second cycle of chemotherapy, we performed chest CT. In the chest CT, the infiltrative lesion, involving the myocardium and multiple lymph nodes, was decreased () compared with the chest CT performed before the R-CHOP therapy (). On the basis of this result, we are confident that the DLBCL originated in the heart and the final diagnosis was PCL.\nAfter 6 cycles of chemotherapy, chest CT and positron emission tomography scans were performed to assess the response to treatment. Imaging studies showed an improvement in the lesions, including the cardiac manifestations. However, the cardiophrenic angle lymph nodes were persistent (). He commenced radiation therapy (total 40 Gy) for the cardiophrenic angle lymph nodes. After radiation therapy, the lymph node size was slightly decreased. |
A 73-year-old Chinese man was found a cervical mass for 1 month. He had a history of coronary heart disease for 2 years, but no relevant personal or family history of malignancy. Physical examination showed bilateral neck asymmetry, the trachea was deflected to the left, the carotid pulse was normal, approximately 6 × 5 cm solid masses were felt in the right lobe of the thyroid gland. The tumor mass border was unclear with smooth surface, and the tumor moved up and down with swallowing. The left lobe of the thyroid gland had no palpable mass and the neck had no palpable swollen lymph nodes. Accessory examination of neck color Doppler ultrasound at our hospital showed increase in size of the right lobe of the thyroid gland, which had abnormal shape. The upper right pole of the thyroid gland had an approximately 5.6 × 6.0 × 4.2 cm sized mass, with unclear margin, and multiple cystic nodules in the thyroid. The CT of thyroid showed increase in size of the right lobe of the thyroid gland, and an approximately 5.6 × 6.0 × 4.2 cm sized mass, the trachea was compressed and deflected to the left, the thyroid cartilage was compressed, deformed and reached the right subglottic region. The right thyroid lump had multiple calcifications, indicative of a tumor (Fig. ). Electronic laryngoscope examination showed ventricular bands thickening, the right ventricular bands compartment showed a rice-like projection, arytenoid region movement was poor (Fig. ). Thyroid function test was 5.07 mIU/L. The patient underwent subtotal thyroidectomy and excisional biopsy of neck mass under general anesthesia. Intraoperative findings indicated a hard mass in the thyroid gland area, the lateral border and the lower bound were closely related to the surrounding soft tissues, but there was an extremely close relationship between the medial border, the thyroid cartilage, and cricoid cartilage, with no gap between them. An ENT doctor was invited for intraoperative consultation, who suggested that some of the tumors should be removed and frozen. The rapid pathology of the tumor (thyroid right lateral lobe) indicated chondroma. The ENT doctor suggested laryngeal chondroma resection and tracheotomy under general anesthesia. Intraoperative findings indicated that the right lower half of the thyroid cartilage and the right half of the cricoid cartilage were chondroma, and complete resection of the tumor was performed. Postoperative pathology examination showed (right lobe of thyroid gland and larynx) chondroma (Fig. ). Given the advanced age of the patient, long surgical duration and poor cardiorespiratory function, the patient suffered sudden cardiac death after the operation. |
A 55-year-old healthy woman was referred to our institution with a two-year history of progressive dysphagia to solids (). She reported a recent episode of solid food getting stuck in her throat, which prompted presentation to an outside endoscopist. The patient reported no alcohol use. She was a former smoker with a 15 pack-year history, but had quit over 20 years prior. The patient had a past medical history of gastroesophageal reflux disease, for which she was taking omeprazole, and hypothyroidism. She had no known history of any esophageal dysmotility disorder. There was a history of diabetes mellitus in her mother and son.\nPhysical exam and laboratory testing were unremarkable. Esophagography demonstrated a filling defect in the upper thoracic esophagus. Computed tomography (CT) demonstrated an 8 cm mass. Endoscopic ultrasound (EUS) demonstrated a pedunculated mass with a submucosal origin beginning at 20 cm from the incisors on the right side of the neck (). The lesion was felt to have the characteristic appearance of a FVP and the patient elected to proceed with resection.\nThe exploration began via a right cervical approach. The recurrent laryngeal nerve was identified and the cervical esophagus was mobilized. The mass was palpable on the posterior esophageal wall at the thoracic inlet. Upon a short myotomy, no stalk was identified and the mass could not be delivered to the neck. The cervical incision was closed and a right thoracotomy was performed. The mass was seen extending from the level of the azygos vein to the thoracic inlet. The esophageal muscular layer was intact. Following myotomy, the soft mass, which was densely adhered to the mucosa, was visualized and dissected from the underlying mucosa. It became evident that the mass maintained its attachment to a portion of the mucosa. Complete mobilization revealed the mass to be a lipoma at the tip of a large midesophageal diverticulum traveling in a submucosal plane. Repeat endoscopy demonstrated an ostium in the esophageal wall opening into a blind-ending pouch. The diverticulum was fully mobilized and resected using a stapler (). Mucosal closure was reinforced with overlying muscle and a pleural flap.\nThe patient was diagnosed with a large midesophageal diverticulum with a lead point lipoma. The patient’s postoperative course was uncomplicated. A postoperative esophagogram demonstrated no esophageal leak or obstruction. Pathology demonstrated a 7.5 cm diverticulum with a 4.5 cm lipoma without malignancy. At follow-up on the nineteenth postoperative day, the patient was tolerating a diet without dysphagia. |
In October 2012, a 46-year-old woman was referred to our center for the presence of a large mass (50 × 70 mm) in the superior lobe of the left lung with homolateral pleural effusion. The patient was never smoker, without family history of cancer and without comorbidity. The cytological diagnosis was made using a CT-guided fine needle aspiration of the primary tumor and revealed an adenocarcinoma of the lung (TTF1+, CK7+) with the EGFR ex19del mutation. A PET-CT demonstrated the presence of liver and bone metastases and a nodule in the right breast, confirmed as a metastasis by fine needle aspiration. The patient received zoledronic acid 4 mg every 28 days and gefitinib 250 mg daily since November 2012 obtaining a partial response (PR). In August 2013, a disease progression (PD) was documented, with an increase in size of the primary tumor and size and number of liver metastases. A brain MRI revealed the presence of two cortical nodules, which were treated with stereotactic radiotherapy. The patient was enrolled in the IMPRESS trial and received 6 cycles of cisplatin and pemetrexed plus gefitinib obtaining again a PR that lasted until June 2014. Thereafter, a new lung metastasis appeared in the superior lobe of the left lung and the mammary nodule increased in dimensions. From June 2014 to December 2014 the patient received erlotinib 150 mg daily obtaining an initial stabilization of the disease (SD); however, within 6 months, she experienced again a PD with the increase of the mammary nodule and the appearance of a new bone metastasis in the sacrum. In December 2014, EGFR ex19del and T790M mutations were detectable in a new needle biopsy of the primary tumor; only at this time a digital PCR-based method was available for the analysis of circulating tumor DNA (ctDNA). Briefly, the method was optimized in order to recover a suitable amount of ctDNA for molecular analysis from 3 ml of plasma using the QIAmp Circulating Nucleic Acid Kit (Qiagen®, Valencia, CA). ctDNA was examined using the Prime PCR Probe Assay on a QX100™ Droplet Digital™ PCR System (BioRad®, Hercules, CA) for EGFR mutations (ex19del, T790M, and C797S) []. The ctDNA sample was considered as EGFR mutant when at least one droplet was above the fluorescence intensity threshold of 3000 and results were reported as copies/ml. The first plasma specimen was obtained in December 2014 and confirmed the presence of ex19del and T790M mutations (480 and 260 copies/ml, respectively; Fig. ). The patient was treated with atezolizumab from March to May 2015 and received stereotactic radiotherapy on the lung primary tumor and on metastases of the left superior lobe, breast and bone (sacrum and D10). Due to PD, the patient was given afatinib 40 mg daily from June 2015 to September 2015, but she experienced a further PD with an increase in size of the primary tumor and lung nodules, the appearance of new multiple bilateral lung metastases, lymphangitic infiltration and liver metastases. At this time, the presence of ex19del and T790 M was again demonstrated in a biopsy of a liver metastasis. Since osimertinib was available, the patient was enrolled in the ASTRIS trial and given 80 mg daily starting from October 2015 with an immediate clinical benefit. At the first evaluation a PR was documented with disappearance of most of the lung nodules and lymphangitic infiltration, reduction of the primary tumor and of liver metastases as well. A disappearance of ex19del or T790M was demonstrated in ctDNA in a blood sample obtained in October 2016. However, in April 2017, ex19del appeared again (400 copies/ml) and in July 2017 it increased to 1000 copies/ml, while T790M was also detectable (330 copies/ml, Fig. ). Then, ex19del and T790 M continued to increase to, respectively, 1600 and 510 copies/ml in October 2017, 2100 and 550 copies/ml in November 2017, and 6900 and 1900 copies/ml in December 2017.\nA radiological progression was demonstrated with increase in size and number of liver metastases in December 2017. The patient underwent a new liver biopsy that confirmed the PD and the presence of ex19del and T790M, whereas the ctDNA showed also the appearance of C797S mutation (170 copies/ml), in addition to ex19del and T790M. The patient started chemotherapy with carboplatin and pemetrexed and in February 2018 she obtained an SD associated with a drop of ex19del (800 copies/ml), T790 M (380 copies/ml), and C797S (90 copies/ml) and then a PR in May 2018, with disappearance of C797S and reduction of ex19del (760 copies/ml) and T790M (90 copies/ml). In July 2018, however, ex19del strongly increased to 2200 copies/ml, even though T790M and C797S were undetectable. Finally, in October 2018, when a PD was documented, ex19del increased to 5100 copies/ml, while T790M and C797S appeared again in plasma with 600 and 180 copies/ml, respectively (Fig. ). At this time a NextSeq 550 NGS platform (Illumina®, San Diego, CA) was available to analyse ctDNA by the AVENIO ctDNA Expanded Kit (Roche®, Pleasanton, CA). A plasma sample collected in December 2018 and analysed by NGS confirmed the presence of the ex19del, T790M and C797S and found, in addition, EGFR and c-MET amplifications, which were not present in tissue in the last re-biopsy of December 2017. |
A 25-year-old man presented to our hospital which was involved in a high-energy traffic accident and suffered severe pain. Physical examination showed swelling and widespread tenderness over the left thigh. He had sustained a direct impact injury on his left thigh over the ground. No open wound and other evidence of associated injuries in the affected extremity were observed. There was no central or peripheral nerve injury. On the radiographs, a transverse fracture of the distal femoral shaft was found (Fig. ) (AO type: 32-A3). Subsequently, an immediate internal fixation was performed with a reamed retrograde locked intramedullary nail after complete pre-anesthetic checkup; the femoral nail was inserted through a split tendinous approach. The patient tolerated the procedure well.\nThe patient was progressively rehabilitated without complications in our hospital for 7 days postoperatively and only bedside activities were instituted in his daily life for 1 month. During the early postoperative follow-up, no associated problems were observed. He was prescribed physical therapy to increase his ROM. Unfortunately, the patient moved to another province and was then lost to follow-up.\nTwo years later, the patient returned to our department and reported persistent and progressive limited motion of the left knee. The patient did not experience other specified injuries of the affected knee after the surgery. He did not receive drug treatments or radiotherapy. Examination showed his previous wounds were healed. Two extensively hard ossification regions during palpation of the affected knee were observed. And one palpable tender mass was noticed within the area of the patellar tendon which was more evident in 50° knee flection. There was no evidence of erythema, swelling, and laxity. The ROM of the affected knee was restricted by pain at 0° to 50°. International Knee Documentation Committee (IKDC) score was 41.1. The radiological images revealed that the fracture ends were healed well with callus, which were accompanied with many ectopic ossifications including patellar ligament ossification, medial quadriceps tendon ossification, collateral ligament, and small ossification scattered in the medial of the knee (Fig. ). The computed tomography (CT) revealed an area of ossification (6.8 × 3.1 × 1.2 cm) within the patellar tendon, which was well presented in the 3D reconstructions (Fig. ). Blood tests including albumin, glucose, total proteins, lipids, creatinine, alanine amino transferase, blood urea nitrogen, aspartate amino transferase, sodium, alkaline phosphatase, potassium, bilirubin, calcium, and chloride were within normal ranges that exclude metabolic diseases. These findings revealed complete mature bone formation and then a diagnosis of “symptomatic heterotopic ossification within the patellar tendon” was rendered around knee joint. Therefore, open surgery was recommended, but the patient has refused further treatment. This study was approved by the institutional ethics board of the First Hospital of Jilin University and the written informed consent was obtained. |
We present the case of a 56-year-old female with history of syncope due to third degree atrioventricular heart block presenting initially with onset of stroke symptoms six days after pacemaker placement and two days after hospital discharge. At 5 PM she developed abrupt onset of left facial droop along with left upper and lower extremity weakness. The patient was initially treated at an outlying hospital and received alteplase at 6:35 PM for treatment of acute ischemic stroke.\nA chest radiograph performed at the outlying hospital prior to alteplase administration demonstrated an enlarged cardiac silhouette when compared to prior radiographs showing only borderline cardiomegaly. Upon administration, the patient reported mild chest pain and was given nitroglycerine and morphine. Her chest pain resolved and she was transferred to our comprehensive stroke center for admission. The patient presented to our emergency department at 10:10 PM with a heart rate of 122 beats per minute (bpm) and a blood pressure of 109/41 millimeters of mercury (mmHg).\nAt 11:20 PM the patient went for a computed tomography angiogram (CTA) after an initial assessment by the emergency physician in consultation with the stroke-team attending physician. After CTA at 10:28 PM, she was documented to have a blood pressure of 49/25 mmHg and heart rate of 109 bpm. She was returned to the resuscitation bay for re-evaluation. Cardiac tamponade was suspected due to the extreme hypotension in the setting of thrombolytic administration after recent pacemaker placement.\nOn reassessment, the patient had become confused with a Glasgow Coma Scale of 14. The emergency physician performed a POCUS, which demonstrated a pericardial effusion with features of cardiac tamponade including diastolic collapse of the right ventricle (). At that point the diagnosis of cardiac tamponade was made. The patient was alert and responsive, so an intravenous bolus of normal saline was given while a stat surgical consult was obtained. The surgical team evaluated the patient at the bedside within minutes and was able to review the POCUS findings. As the patient was conscious, they elected to take her immediately to the operating room rather than perform a bedside pericardiocentesis.\nWhile in the operating room, approximately 400 milliliters of coagulated blood were evacuated from the pericardial sac with 150 milliliters of surgical bleeding. The operative report notes resolution of tachycardia following this intervention with heart rate trending down to a range of 80–90 bpm with concomitant improvement in blood pressure. She was discharged two days post-operatively with a pericardial catheter in place. Echocardiogram performed on day of discharge noted a small, residual pericardial effusion. |
Male patient, 85 years old, with previous total left patellectomy due to patellofemoral arthritis at age 60 who subsequently underwent left TKA at age 79 ( surgeries performed in a different hospital ). He developed pain and progressive limitation of active and passive knee extension causing restriction of activities of daily living. He became accompanied by the Knee group from IOT-HC/FMUSP in December 2008, at that time four years and nine months after arthroplasty. On physical examination the patient had restriction on knee extension with lag of 40 degrees in active extension and 20 degrees in passive extension. The patient only walked with the aid of a walker, with no load on the compromised member. He did not show any ligament instability. Radiographs showed loosening of the tibial component with significant tear of the polyethylene insert. The inflammatory markers (ESR and CRP) were normal and bone scan was not suggestive of an infectious process. In April 2009 the patient underwent revision arthroplasty and allograft extensor mechanism transplant. The graft consisted of quadriceps tendon - patella (full) - patellar tendon - anterior tibial tuberosity (ATT). The graft was obtained from the IOT-HC/FMUSP Tissue Bank, having been previously prepared and frozen, without viable cartilage. The graft placement was based on the lower pole of the patella, which was kept 2.5 cm above the joint line. A pin in the tibia was inserted in the topography of the anterior tuberosity, where the previously molded plug bone allograft (ATT) was fitted under pressure and fixed with a large fragments screw. The articular surface of the patella graft was not replaced for being insensitive; replacement could be an unnecessary source of complications (such as fracture). The quadriceps tendon graft was then sutured to the patient's quadriceps, tensing toward the graft proximal and distal quadriceps, through Kracow technique with high strength nonabsorbable multifilament wire, keeping the knee in full extension. The capsule and what remained of the remnant extensor apparatus were sutured over the graft with simple absorbable wire, covering it in an attempt to increase its vascularization. ( and ) Postoperatively the patient was maintained immobilized in extension for six weeks performing only isometric strengthening exercises and assisted passive range of motion gain. After the sixth week active exercises with progressive weight were allowed until the third month, when immobilization was discontinued. |
A 53-year-old male presented chest discomfort and palpitations. Electrocardiography performed on admission revealed sinus tachycardia with no ischemic change. His blood pressure was 143/80 mmHg and pulse rate was 95 beats/min. Laboratory results including complete blood count and cardiac enzymes were within the normal range. There was no cardiomegaly or evidence of active lung lesion on chest radiography. Transthoracic echocardiography revealed normal ejection fraction, chamber size, and valve function, and regional wall motion abnormality was not present. However, there was abnormal blood flow into the pulmonary artery on the parasternal short axis view, suggesting a coronary artery fistula (CAF). The patient underwent coronary angiography, which revealed a coronary fistula between the proximal left anterior descending coronary artery (LAD) and the pulmonary artery (). The right coronary artery (RCA) also had a congenital anomaly. The right conal artery, originating from a separate orifice of the right coronary sinus and draining into the pulmonary artery, indicated the presence of a CAF between the right conal artery and the pulmonary artery (). Contrast-enhanced electrocardiogram-gated multidetector computed tomography (MDCT) was performed to evaluate the coronary fistulae and to reveal any other abnormalities. MDCT showed an abnormal connection between the right conal artery and the LAD draining to the pulmonary artery via a 6.2-mm aneurysm. In other words, the right conal artery originating from a separate orifice of the right coronary sinus had an abnormal connection with the RCA via a short vessel just distal to its origin. It passed up and over the right ventricular outflow tract to provide flow to the aneurysm, which was connected to the LAD via a highly tortuous artery. There was also a fistula between the aneurysm and the main pulmonary artery (, ). These features were compatible with the diagnosis of an aneurysm of VAR. The patient underwent an echocardiographic stress test and treadmill test to investigate cardiac ischemia; these tests showed no ischemic change. Surgical resection was offered as a treatment option; however, the patient refused treatment for personal reasons and is being followed closely. |
A 61-year-old female with past medical history significant for type 2 diabetes mellitus, hypertension, and dyslipidemia and family history of breast cancer in her brother and maternal aunt presented to the emergency room for recurrent chest pain. She also reported fatigue for the past few months and was being evaluated for worsening anemia by her primary care physician. Her hemoglobin was 6 gm/dl with iron panel consistent with anemia of chronic disease. Her electrocardiogram (ECG) at arrival showed an “RSR pattern” in V1 and V2 leads but was otherwise normal. She underwent a stress myocardial perfusion imaging (MPI) which was negative for cardiac ischemia. A transthoracic echocardiogram revealed a 1.6 cm × 1.5 cm atrial mass attached to the anterior wall of the left atrium, which appeared to cross the mitral valve in ventricular diastole. The left atrial mass was further characterized by a transesophageal echocardiogram (TEE) as a solid, irregularly shaped, partly mobile mass attached to the atrial septum and extending to the anterior mitral valve leaflet (). The segment attached to the septum measured 2.6 cm × 1.43 cm, and the segment attached to the valve measured 1.4 cm × 2.22 cm. In addition, MRI of the heart with gadolinium was done preoperatively which confirmed the circumscribed hypodense mass with speckled appearance which originated at the atrial septum and extended along the anterior mitral valve leaflet (). The mitral valve flow was normal with no evidence of obstruction, stenosis, or regurgitation. The patient underwent a minimal incision valve surgery for resection of the mass which was presumed to be myxoma due to its location. Intraoperatively, on open examination of the left atrium, it was noted that the mass originated from the fossa ovalis region of the interatrial septum and infiltrated the atrial wall down onto the entire anterior leaflet of the mitral valve. A fibrotic density that surrounded the tumor was also noted. The mass along with a portion of the interatrial septum and the mitral valve was resected. The mitral valve was replaced using a 27 mm Hancock II bioprosthetic valve. The atrial septal defect caused by the resection was repaired with a bovine pericardial patch. A postoperative TEE was performed which confirmed the successful placement of the bioprosthetic valve with no paravalvular leak. The patient had an otherwise unremarkable postoperative recovery and was discharged home after fourteen days of hospital stay.\nTwo weeks following discharge, the patient presented to the emergency with cough, diaphoresis, and palpitations. She was noted to have jugular venous distention and bibasilar crackles on auscultation of the lungs and was found to be in acute heart failure. An urgent transesophageal echocardiography demonstrated severe mitral regurgitation with paravalvular leak (Figures , , , and ). At the same time, the histopathologic examination of the atrial mass showed a high-grade sarcoma consistent with dedifferentiated liposarcoma. The tissue exhibited spindle cells with pleomorphism, multinucleated giant cells, and inflammatory cells. Immunohistochemical stains demonstrated that the neoplastic cells were positive for vimentin, focally positive for S-100, and weakly positive for CDK4 and negative for p53 (). FISH studies performed showed an MDM-2 gene amplification in 95–200 nuclei examined. The patient was aggressively treated with intravenous diuretics and afterload reduction using furosemide and nicardipine infusion. Blood cultures were obtained with suspicion of postsurgical infective endocarditis causing valvular dehiscence. However, cultures did not grow any bacteria. The patient clinically deteriorated due to new-onset atrial fibrillation and worsening heart failure despite medical treatment in CCU. CT chest obtained showed a 1.3 cm lytic iliac bone lesion and 3.1 cm × 2.5 cm right upper mediastinal soft tissue density. Due to high suspicion of metastatic disease, MVR and cardiac transplant were not offered until further evaluation for metastasis. Unfortunately, due to rapid clinical decline with a new diagnosis of high-grade cardiac tumor with possible metastases, the patient opted for hospice care. PET study was not obtained. |
A 56 year-old female was transported to the emergency department by EMS after physically threatening her husband with a knife. According to the patient, she experienced altered sensorium for one week prior to presentation, primarily resulting in suicidal and homicidal command hallucinations instructing her to overdose on NSAIDs and kill her husband. She denied any headaches, vertigo, fevers, head trauma, urinary symptoms, or use of illicit substances. There was no history of psychosis, schizophrenia, mania, or depression and a review of her medication list for any potential hallucinogenic agents did not indicate a pharmacologic etiology. Her past medical history was comprised of two prior strokes, the most recent being two years ago that involved the right frontal lobe. MRI of the brain at the time also showed evidence of an old right parietal lobe infarct. An echocardiogram during that hospitalization revealed a severely decreased left ventricular function and the patient was initiated on warfarin for anticoagulation. Since the two prior cerebral infarcts, the patient and her husband denied noting any behavioral changes, cognitive impairment, or any focal neurologic deficits. On presentation, she appeared disheveled and exhibited a flat affect with minimal verbalization. Physical examination revealed only a left hemianopia without hemiplegia. All cranial nerves were evaluated as well as gait, cerebellar function, and proprioception, which were all found to be normal. The patient was alert and oriented with intact mentation.\nShe was initially admitted to the psychiatric unit for further evaluation where the patient participated in daily activities and reported no symptoms. Given the acute onset of her symptoms, laboratory studies and brain images were obtained in order to rule out an organic etiology. A complete blood count was normal and a basic metabolic panel revealed normal electrolytes and renal function. Additional labs included liver function tests, lipid panel, cardiolipin antibody, and TSH, which were all normal and RPR was nonreactive. A urine sample was negative for urinary tract infection and 8-panel urine drug screen was negative. An MRI of the brain was obtained, which revealed old ischemic infarcts within the right parietal and frontal lobes along with a new acute right occipital lobe infarct with hemorrhagic transformation (). The patient was subsequently transferred to the medical unit for further workup and management.\nThe patient was placed on telemetry, which showed normal sinus rhythm. Given the involvement of multiple brain territories and circulations, a cardioembolic source of stroke was highly suspected. A cardiologist evaluated the patient and performed a transesophageal echocardiogram that revealed a low ejection fraction of less than 20%. In addition, a bubble study was performed that was positive for a patent foramen ovale. Given her significantly low ejection fraction, the patient underwent placement of an automated implantable cardioverter-defibrillator. The patient's presenting symptom of psychosis, primarily in the form of auditory hallucinations, was self-limited and resolved on day two of hospitalization without requiring the use of antipsychotic medications. |
A 54-year-old married mixed ancestry man and father of four children, working in the construction industry, was diagnosed simultaneously with type 2 diabetes mellitus and hypertension 8 years prior to admission. He also had a significant family history of type 2 diabetes mellitus, with his father and two of his brothers affected, all of whom are deceased, having suffered complications of ischemic cardiac failure and renal failure, respectively. However, none of the siblings had a history of admission for recurrent hypoglycemia. The father of the index subject died at 70 years of age, having had ischemic cardiomyopathy, which necessitated coronary artery bypass graft surgery prior to this event, whereas his two brothers died from diabetic nephropathy at age 48 and 50 years. In addition, there was no family history suggestive of pituitary disease or hyperparathyroidism. Between 2010 and 2017, our patient presented several times to his primary care physician with blood glucose measurements greater than 26 mmol/L. At each of these visits, he received short-acting human insulin and intravenous fluid. His final presentation to the primary health care facility with hyperglycemia occurred 12 months prior to admission.\nHis current presentation on June 27, 2018, involved a referral to his secondary care hospital, having been asymptomatic for a year, with no episodes of hyperglycemia except for weight gain of 14 kg in the preceding 3 months. According to his family, he suffered from confusion and sleepwalking, particularly in the early hours of the morning between 03:00 and 08:00. These symptoms occurred almost every day, associated with generalized body weakness and sweating, which were noticeable after each episode. His family reported physical and verbal aggression and confusion. During additional episodes in hospital, he was noted to be combative with the nursing staff and fellow patients and was found to be hypoglycemic on several occasions. Our patient appeared to develop symptoms indicative of hypoglycemic unawareness, as he manifested with no sympathetic symptoms, despite glucose concentrations below 1.8 mmol/L (3.8–5.5 mmol/L). During this admission, he exhibited recurrent episodes of fasting and post-prandial hypoglycemia, which measured between 1.2 and 3.0 mmol/L.\nThe possibility of nonconvulsive seizures was entertained; thus a computed tomography (CT) brain scan was ordered and found to be normal, whereas an electroencephalogram was not available at the secondary care facility. He was transferred to a tertiary hospital for evaluation by the endocrine service.\nAt admission, his chronic medications were metformin, enalapril, hydrochlorothiazide, and simvastatin. Aside from metformin, he was not taking any oral hypoglycemic agent or insulin and denied using any other agents. Pharmacy records failed to identify that he had received either a sulfonylurea or insulin. The two-hourly ward glucose measurements showed recurrent hypoglycemic episodes ranging from 1.2 to 3.4 mmol/L, occurring between 03:00 and 08:00.\nClinical examination failed to identify insulin injection sites or evidence of target organ damage relating to diabetes, hypertension, or any other chronic disease. In particular, he had no indication of melanoderma. |
A 68-year-old woman with a history of arterial hypertension well controlled on medical treatment, hypercholesterolemia treated with simvastatin, and paroxysmal atrial fibrillation treated with amiodarone was admitted with unstable angina, elevated troponin levels, and ischemic electrocardiographic changes on leads V4-V6. Moreover, she suffered from hepatopathy and subclinical hypothyroidism and had previously undergone mastectomy due to breast cancer.\nThe patient underwent diagnostic cardiac catheterization with normal coronary arteries. The left main coronary artery was selectively cannulated with a 6 F Judkins Left-4 guiding catheter and the right coronary artery was also selectively cannulated with a 6 F Judkins Right-4 guiding catheter (Medtronic, Inc., Minneapolis, Minnesota). The velocity of the contrast injection in both coronary arteries was 3 mL/s. In both arteries, the cannulation was uneventful without any complication. A second fluoroscopic injection into the right coronary artery demonstrated a 360° dissection of the right coronary artery extending to the coronary cusp with a subtotal occlusion of the artery ().\nThe patient developed severe bradycardia, which was treated with a temporary pacemaker. Emergency transthoracic echocardiography in the catheterization laboratory and later perioperative transesophageal echocardiography confirmed the dissection and demonstrated acute akinesis of the diaphragmatic myocardial wall and the interventricular septum with moderate left ventricular dysfunction ().\nThe patient underwent an emergency operation through a median sternotomy with ligation of the right coronary artery at its origin with a saphenous bypass vein graft. The aorta was opened, and no aortic wall dissection was seen. The operative finding showed a 360° dissection of the right coronary artery with the dissecting flap everting into the aortic lumen (). The dissection on the right coronary artery was extended distally to the bifurcation of the artery. The venous graft was placed distally before the bifurcation of the artery, and the lumen of the artery at the anastomotic site was dissected. The operative procedure is shown in .\nThe postoperative course was uneventful; and on postoperative day 6, she was discharged home in stable condition. |
A 65-year-old man with continuous irritable cough over 15 days was admitted to our department for a mass lesion in the right hilum. He presented with no face swelling and superficial varicose veins. He was a former smoker, and medical history included hypertension and diabetes mellitus. At bronchoscopy, the tumor was found occluding the right upper lobar bronchus and infiltrating the bronchus intermedius. It was diagnosed as squamous cell carcinoma by transbronchial tumor biopsy. Chest contrast computed tomography (CT) revealed that the mass of 80 mm in the maximum diameter located in the right upper lobe. The tumor directly invaded the arch of the right PA and a wide range of SVC. It was discovered that the left brachiocephalic vein (BCV) along the left margin of mediastinum walking down the line directly into the coronary sinus (). A venous flow was detected in the anterolateral descending aorta with dilatation of the coronary sinus (diameter of 21 mm) without other heart abnormalities by transthoracic echocardiography. Based on these findings, the diagnosis of PLSVC was made. There were no detectable metastases in other organs through brain magnetic resonance imaging (MRI), upper abdomen CT scan and bone nuclear scan.\nThe right posterolateral thoracotomy was performed on October 31, 2006. As expected, the tumor invaded the wall of SVC and no left brachial cephalic vein bifurcation was observed. The SVC was separated, clamped and resected following intravenous injection of 5, 000 IU of sodium heparin. The ringed PTFE graft of 12 mm in diameter was interpositioned between the right brachial cephalic vein and the origin of SVC by running sutures with 4-0 polypropylene. The tumor resection was completed by double sleeve lobectomy of right upper and middle lobes. The anastomosis between the right main bronchus and the cut end of the lower lobe bronchus was performed by interrupted suture using 3-0 Vicryl (Ethicon, USA). Right PA trunk and right lower PA reconstruction was completed by the running suture with 4-0 polypropylene (). And systematic mediastinal lymph node dissection was performed. All excised margins were tested microscopically negative for malignancy. As anticoagulation treatment, we began with low molecular Heparin on the operative day, then switched and continued to warfarin when thoracic drains removed. The postoperative course was uneventful, and the patient was discharged from the hospital on the 12th postoperative day. Final pathological diagnosis was poorly differentiated squamous cell carcinoma invaded the right SVC, right PA and mediastinal nodes were positive for malignancy.\nAfter 4 cycles of chemotherapy (Gemzar and cisplatin) and 1 cycle of radiotherapy, Chest MRI showed patency of the graft and PLSVC (). He died 21 months after operation because of remote metastasis. |
A 60-year-old man was brought to the emergency department with a chief complaint of periumbilical abdominal pain. He suffered from abdominal pain for two weeks but it was significantly increased in the three days prior to admission. The pain radiated to the back and no accompanying signs or symptoms were present. It was described as a constant pain without any alleviating or exacerbating factors. The patient was a heavy smoker without any history of previous surgery or drug use.\nThe patient showed stable vital signs and a nuisance general appearance. No objective signs of weight loss or cachexia were observed. The physical exam was normal in the head and neck, chest, and extremities. In the abdomen a pulsatile mass was palpated in the periumbilical region. Routine laboratory tests revealed mild normocytic normochromic anemia, normal ESR and CRP, and liver function and diagnostic tests within normal limits.\nDue to the pulsatile nature of the mass a CT angiography of the abdomen was ordered to look for vascular pathology. The tomography revealed a 5-by-4 cm pseudoaneurysm with hematoma formation in the infrarenal aorta alongside diffuse dilation of the infrarenal inferior vena cava with extension into both common iliac veins (), and the patient was prepped for surgical repair.\nDuring surgery a pulsatile mass 20 by 20 cm in diameter with surrounding hematoma was observed in left zones 1 and 2. Upon further investigation a huge retroperitoneal mass 11 by 8 by 6 cm in diameter with apparent local invasion to the aorta and the inferior vena cava was found. En bloc resection was attempted along with involved vascular segments. The abdominal aorta was reconstructed using 16 mm-by-8 mm Dacron bifurcated graft, while the IVC was ligated below the renal veins but perhaps because of chronic process of disease no lower extremity edema happened and the patient was discharged after a week.\nPathologic evaluation of the specimen showed an undifferentiated pleomorphic sarcoma (a.k.a. malignant fibrous histiocytoma) with a histologic grade according to French Federation of Cancer Centers Sarcoma Group III (tumor differentiation: III, mitotic rate >20/HPF: III, and tumor necrosis <50%: I) (). There was no evidence of lymph-vascular or perineural invasion. Aneurysmal wall resection of the abdominal aorta with blood clot and atherosclerosis was also reported. The specimen was also positive for vimentin (patchy areas), SMA (small portions), and CD68 (most parts). |
A 29-year-old female in 27th week of her gestation presented to obstetrical emergency ward with chief complaints of high-grade fever for the last 8 days. Initially, she was treated as an outpatient at the community hospital by a primary physician for fever and mild cough. She was prescribed tablet Amoxicillin 500 mg four times daily for 5 days on suspicion of upper respiratory tract infection on an empirical basis. In addition, her routine investigations done at a community hospital revealed a hemoglobin value of 10.8 g%, a random blood sugar value of 132 mg%, a blood count value of 10,600/cumm. No abnormality was detected in the routine urine examination. On the basis of investigation reports, she was discharged home on the same day by the primary care physician after the routine examination. In spite of taking the prescribed medication, no clinical improvement occurred and fever could be controlled only intermittently by administration of antipyretics. However, after completion of the prescribed duration of antibiotic therapy, fever remained persistently high in the range of 100–102 °F. The pregnant state, persistent fever refractory to treatment, discomfort, and resulting anxiety were the main decisive factors, which forced the primary physician to refer the patient to a higher center instead of going for the battery of tests for PUO during the follow-up visit. The entire treatment was carried out at home, and she was never admitted at the community hospital.\nOn admission at our tertiary care center, besides fever, she did not give any history of bowel symptoms but dysuria was one of the presenting features. After a thorough initial clinical evaluation, all the requisite investigations for possible causes of pyrexia were sent for. Her general physical examination revealed numerous maculopapular lesions over the upper abdomen including the inframammary region. [] On local examination of genitalia, a purulent and foul smelling discharge was present at the vagina along with extensive vulval ulceration as well as the presence of papular lesions on the perineum, groin, and inguinal region. The most striking feature during her physical examination were the presence of rashes which the patient did not convey to the primary care physician as these were thought to be of little significance and unrelated to pregnancy by the parturient. Moreover, in an Indian set-up, female pregnant patients are reluctant to allow for complete examination by the attending primary physician. Ultrasonography (USG) examination of the abdomen was not informative of any significant pathology and a live foetus of approximately 27 weeks gestation was confirmed by USG report. Awaiting the laboratory investigations, an urgent physician and dermatologist consultation was sought. She was provisionally diagnosed to be a case of PUO with possibility of systemic bacterial infection and local fungal infection (intertrigo). On the basis of provisional diagnosis, she was prescribed tablet cefoperazone in an empirical manner and local application of clotrimazole powder. The laboratory profile turned out to be absolutely normal including blood count, urine complete and culture sensitivity examination, blood culture and sensitivity, erythrocyte sedimentation rate, malaria parasite film, renal function panel, liver function tests, sputum culture and sensitivity, viral markers for HIV and hepatitis, VDRL, and other pathologies. Other routine investigations such as blood sugar were absolutely normal, but peripheral blood film revealed the presence of normochromic normocytic anemia. In spite of administration of antibiotic therapy, she had persistent fever in the range of 101–102°F with occasional remissions of few hours after administration of antipyretics.\nThe clinical scenario of refractory fever and the laboratory findings enforced us to review our diagnosis. After thorough discussion among the physician, dermatologist and the obstetrician, a possible diagnosis of intertrigo with severe invasive and systemic candidiasis infection was thought of. The clinical scenario was thought of as a flare-up of opportunistic fungal infection in the context of systemic administration of antibiotics. However, the contradictory fact to this clinical scenario was the presence of these rashes for the last one month. The diagnosis was further confirmed by the presence of candida growth on KOH preparation and she was treated with fluconazole tablet 150 mg once a week, washing with KMnO4 and local application of clotrimazole powder besides the administration of ongoing antibiotic regimen. She responded to this therapeutic regimen and fever subsided gradually over a period of 2 days. The entire later course of gestation was uneventful, and the patient delivered a normal healthy baby at term pregnancy. |
A 54-year-old man was presented to the outpatient clinic of the Department of Physical Medicine and Rehabilitation, Kyung Hee University Medical Center, with complaints of the right-side chest pain, which started in April, 2009. From the past medical history of the patient, he got peritonitis caused by perforated appendicitis and had surgery in 1976, and in 1984, he had pneumonia and received treatment. When he came to the clinic this time, for a basic inspection, he underwent an X-ray examination of the thorax and lumbar spine. Compared to the others in his age group, the patient had a relatively greater degenerative change in thoracic region and the lumbar vertebra. He also had slight spinal scoliosis, but any special findings were not revealed (). On that day, he received a trigger point injection, in accordance with myofascial pain syndrome, because no other neurological abnormal findings were perceived.\nOn his first visit, he had both lower extremity manual muscle test, and it showed a normal muscle power, including no symptom of dysaesthesia. Moreover, the result was fine and normal on both the lower extremity deep tendon reflexes, and there was no spasticity or ankle clonus. After giving the trigger point injection on the first visit, we prescribed NSAIDs and muscle relaxant. Additionally, the patient had physical therapy for a week. One week later, the pain itself showed a declining pattern, but the patient complained of numbness and weakness on both lower extremities. Physical examination was conducted again, but still, there was no special finding.\nFrom the first day of the treatment, two weeks had passed and the patient came to the clinic, again. He complained of persisting weakness, so we checked a thoracic MRI for further diagnosis. In an MRI scan, we found a lesion occupying the 5th vertebral body and part of the posterior vertebral structure. Because of this lesion, the spinal cord was thrust out on the left side. Moreover, from the T1 and T2 enhanced images, this lesion showed a low signal intensity of the tumor, similar to the bone (). Although the tumor invaded one vertebral body and one bone marrow, the right-side neural and spinal canal, posterior spinous process, it didn't greatly damage the cortex of the bone.\nIn the initial differential diagnosis, metastatic spinal tumor was suspected. As such, we checked the blood and urine of the patient, using a serum and urine free light chain (κ, λ), urine Bence-Jones protein, serum Beta-2-microglobulin, and tumor marker. From the laboratory findings, there was no significant incidence of tumor, like multiple myeloma or plasmacytoma (). Additionally, in the skull X-ray, there were no peculiar findings, either. Both somatosensory and motor evoked potential were examined; on the somatosensory evoked potential, on the right-side, the defect of sensory pathway were detected, and on the left-side, the test result was normal; the motor evoked potential showed non-significant findings.\nSubsequent bone scan examination showed only a slight reaction of the bone formation on the 5th thoracic spine, and there were no other unusual findings.\nAccording to the laboratory and radiologic findings, this case was suspected as a lymphoma, one kind of a metastatic spinal cord tumor, and the patient was transferred to the department of neurosurgery. He underwent an operation and a biopsy; during the operation, the biopsy result was differing from the initial diagnosis, which suspected plasmacytoma. Afterwards, for the clinical confirmation, a bone marrow biopsy was conducted additionally, and the lesion was diagnosed as a plasmacytoma on the vertebral body of the 5th thoracic spine, Monoclonal Gammopathy of Undetermined Significance (). After ten times of the local radiation therapy, the patient's weakness and numbness on both the lower extremities were gradually subsided. |
We present the case of a 52-year-old female with morbid obesity (body mass index of 40.8), noninsulin dependent diabetes mellitus and tobacco abuse who presented with Stage IIIA (T3, N2, Mo) infiltrating ductal carcinoma. The patient received neoadjuvant chemotherapy prior to bilateral skin-sparing total mastectomies with right axillary sentinel lymphadenectomy and staged reconstruction with temporary breast implants. Two months after chest wall and regional nodal radiation therapy, she developed a marked soft tissue reaction to radiation. She underwent a radical debridement of her right chest wall open wound resulting in a tissue defect of 25 cm in length, by 20 cm in width and by 10 cm in depth.\nDespite two rounds of surgical debridement, intravenous antibiotics, hyperbaric oxygen therapy, one year of wound care by a wound care specialists, the infection failed to resolve and wound failed to heal leaving her with a large, open defect. She required chronic medication for her pain management. The patient later was found to have developed a multidrug-resistant Pseudomonas wound infection (Figure ).\nLack of other treatment modalities led the surgeon to experimentally place a product composed of a porcine urinary bladder matrix. Three days after the application of the product, the patient reported that her pain was now manageable without opioid medication (Figure ). Her wound care regiment consistent of weekly painless applications of UBM powder matrix and sheet epithelial basement membrane on top of powder on the wound. The wound was then covered with lubricating jelly and vaseline gauze. On top of the vaseline gauze, plain gauze was placed and the wound was secured with tape. The patient then wore the same dressing without changing it for days. The patient was instructed to come back for dressing every week to the clinic.\nAfter two weekly applications, her infection had completely resolved and she was beginning to grow islands of new epidermis over her chronically open mastectomy wound (Figure ). By three months, her wound had begun contracting. Four months after placement of UBM, her wound has decreased in size to approximately 3 cm in length by 2 cm in depth and 2 cm in width (Figure ). Her pain has completely resolved, and the wound is growing epithelial islands which will eventually cover the entirety of the granulation tissue that is in her wound (Figure ). |
A five-year-old girl presented with urinary incontinence following a history of pelvic trauma. She had a single orifice at the introitus from which she was continuously leaking urine. On endoscopic examination the bladder neck was found opening into the vagina. Operative reconstruction proceeded []. Operative exposure was obtained with a midline vertical incision from the umbilicus to the vaginal orifice with division and partial excision of the symphysis pubis. The remnant of the proximal urethra and bladder neck were dissected off the anterior vaginal wall at the area of the bladder neck-vaginal fistula. The rupture in the posterior aspect of the bladder neck was then repaired anatomically resulting in a normal appearing bladder neck with a short proximal urethral stump which would not reach the perineum.\nA three cm segment of ileum was isolated on its pedicle to reach the perineum. The ileal segment was tubularized over an 8Fr feeding tube in the form of the Yang- Monti tube. It was then anastomosed proximally to the urethral stump and distally to the introitus at the site of expected urethral orifice. The lower half of the left rectus muscle was mobilized as a flap with its inferior attachment to the pubis left intact and carefully preserving the inferior epigastric vessels. This muscle flap was placed between the posterior bladder wall and the vagina to prevent refistulization.[] The anterior vaginal wall was left unrepaired as the posterior and lateral vaginal walls were intact and closure of the anterior vaginal wall may result in stenosis. The anterior vaginal wall was left to reepithelialize over the rectus muscle interposed between the posterior bladder wall and vagina. An appendicular Mitrofanoff was also created opening at the umbilicus. We contemplate closing the Mitrofanoff port when the child desires it.\nAt follow-up after 10 months, she is voiding normally with continent intervals of four to six hours. Residual urine measured after voiding was two ml. Ultrasound and serum creatinine remain normal and the micturating cystourethrogram was satisfactory []. She uses the Mitrofanoff channel once a day to maintain its patency. |
A 5-year-old female patient reported to the Department of Pediatric and Preventive Dentistry, KLE VK Institute of Dental Sciences, Belagavi, Karnataka, India with the chief complaint of intraoral swelling ( and ) and pain in the lower right back tooth region since 1 month. The patient was apparently alright 1 month back and noticed intraoral swelling of initial peanut size and gradually increased to the size of approximately 2 × 3 cm oval size, which was tender on palpation along with intermittent nature of pain in the lower right back tooth region.\nOn general examination, there was no history of any hospitalization, and a history of trauma to the jaws was noted. Extraoral examination revealed there was no evidence of swelling. Intraoral examination revealed the mixed dentition stage. 74 and 84 were carious, 75 was grossly decayed. The patient gives a past dental history of gross destruction of 85 and exfoliated eventually. The buccal vestibule on the right back region shows obliteration and swelling was extended from the distal aspect of the mandibular right first molar to the mesial aspect of the mandibular right first permanent molar. The swelling was hard in consistency and tender on palpation. For further investigation, OPG was advised and it revealed unilocular radiolucency measuring about 2 × 2 cm around the developing second premolar with a well-defined border, and radiolucency was seen which was extended up to the distal aspect of developing mesial root of permanent first molar ().\nThe provisional diagnosis was made based on clinical and radiographic examination as a dentigerous cyst.\nAll the surgical procedures were explained to the parents and written informed consent was taken for the same. Under aseptic conditions, surgical enucleation of the lesion along with the extraction of the 45, 84, 44, and 46 and grossly decayed 75 was done ( and ). A surgical socket was treated with Corney's solution for 9 minutes then it was packed with gauze piece followed by suture placement ( and ). The specimen was sent for histopathological examination.\nHistopathological examination revealed a cystic lesion lined by squamous epithelium which is ulcerated at places (). The subepithelial tissue shows dense chronic inflammatory infiltrate (). Based on histopathological findings, the final diagnosis was made as an infected radicular cyst of the right posterior mandibular region.\nSuture removal was done after 1 week, and the healing was uneventful. As the defect was larger in size, an obturator was planned to maintain the patency and for the eventful healing of the cavity, so upper and lower elastomeric impressions were made. Obturator was fabricated and delivered till further treatment was advocated (). The parents were instructed to irrigate the socket with betadine and saline solutions and to maintain proper oral hygiene. The patient was kept under follow up for every 15 days to check for healing and the adaptation of the obturator. After 3 months of follow-up, the surgical site shows proper closure of the wound healing and parents were satisfied with the treatment (). |
A 76-year old man presented with a four month history of penile pain and erythema to the urology department. Symptom progression over the preceding months included burning with urination and difficulty retracting foreskin. Initial treatment with a two-week course of nystatin-triamcinolone cream was unsuccessful. Physical exam demonstrated a two-centimeter large fungating mass on the ventral surface of the distal shaft, which appeared to originate from the glans. Digital rectal exam revealed an enlarged, firm, and nodular prostate. The remainder of the physical exam was within normal limits. There was no palpable lymphadenopathy, no focal spinal tenderness, and a normal neurological exam without focal or gross motor weakness. Given the suspicious features of the mass for SCC, particularly the fungating and superficial nature, a discussion with the patient was made regarding management options including biopsy for definitive diagnosis versus partial penectomy. The patient opted for surgical treatment and tolerated surgery well. Pathology following treatment demonstrated Gleason 9 prostate cancer. CT scan of the pelvis was performed following penectomy to assess treatment response, and demonstrated a stable sclerotic lesion on the iliac bone with potential sclerosis inferior to this known lesion and no lymphadenopathy. Bone scan at this time demonstrated no definitive evidence of metastatic disease.\nPast medical history is significant for Gleason 9 prostate adenocarcinoma diagnosed two years prior. Workup at the time included magnetic resonance imaging (MRI) of the prostate which demonstrated a Prostate Imaging Reporting & Data System (PI-RADS) 5 lesion, mildly enlarged pelvic lymph nodes, and a small sclerotic focus of the left iliac bone. Bone scan did not demonstrate evidence of osseous metastatic disease, and CT scan of the chest was normal. Given benign bone scan and following discussion with hematology oncology, it was believed the iliac lesion did not represent metastatic disease. An informed discussion was made with the patient involving treatment modalities, and the decision was ultimately made to pursue combination intensity-modulated radiation therapy and ADT with leuprolide acetate. The patient tolerated treatment well and demonstrated a decrease in PSA over the following two years. Following completion of ADT, PSA levels began increasing over the following year, prompting resumption of hormonal therapy. At this time the patient began experiencing his present penile pain and urinary symptoms. |
A 45-year-old male with a previous history of tympanoplasty and functional endoscopic sinus surgery with septoplasty 10 years earlier presented to the ear, nose, and throat (ENT) clinic with several months of left moderate-to-severe otalgia and a sensation of ear blockage in his left ear accompanied by ipsilateral hearing loss. He gave a history of multiple failed ear wax removal in his left ear that had been performed at several ENT clinics, despite the use of alkaline ear drops.\nOn examination, the patient was comfortable and afebrile, and his vital signs were stable. Otoscopic examination of the left ear revealed impacted left ear wax covering the tympanic membrane, which could not be assessed. Otoscopic examination of the right ear also demonstrated mild ear wax, and the tympanic membrane was unremarkable. Oropharynx examination was unremarkable, the lymph nodes of the neck were not palpable, and all cranial nerves were intact upon examination. Nasal endoscopy revealed no pathologies. Ear wax removal under suction was attempted and failed. Another trial of removal after using alkaline ear drops for several days was also attempted but was unsuccessful. However, the surgeon became suspicious that the patient had KO rather than impacted ear wax because the wax was thick, had the appearance of keratin plugs, and was hard to remove after several attempts, despite the use of ear alkaline drops.\nBlood test results of the patient were within normal limits. The patient was planned for microscope-guided examination of the ears under general anesthesia. The examination revealed that the left ear was full of wax that was accumulating in the skin and contained a thick keratinous plug that had dilated the external auditory canal (EAC) with pockets and bone remodeling. Furthermore, the patient ear canal was circumferentially distended with a normal annulus. The tympanic membrane became visible and was intact. The keratinous plug was removed, and a diagnosis of KO was established (). An ear pack was draped with antibiotics and placed in the left ear. The patient was extubated, shifted to the ward without any complications, and discharged the same evening with the ear pack, which was removed after 3 weeks in the outpatient clinic. The patient was started on ciprofloxacin ear drops and analgesia for 1 week.\nIn the follow-up, the ear pack was removed, his hearing returned to normal level, and the pain disappeared. Pathological analysis of the removed plug revealed acellular lamellated keratin flakes and keratinous material (Figures and ), which confirmed our diagnosis. |
This study presented a case of a 78-year-old female with an 18-month history of painless progressive swelling and erythema on the left forehead and right upper eyelid. The lesions were slightly developed and involved all of the scalp surface, eyelids, and face; however, there was no other abnormality. The chief complaint of the patient was the mass on the right eyelid. She had a proptosis of the right eye and an orbital mass; however, there were no signs of headache, nausea, vomiting, or double vision. The medical history of the patient demonstrated that she had hypertension and coronary artery disease, and had undergone angioplasty. However, she had no history of undergoing radiotherapy or chemotherapy. The result of the brain MRI in May 2015 demonstrated a mucosal thickening in the right maxillary sinus. A soft tissue with a slightly lobulated surface was observed using magnetic resonance imaging (MRI). The ventricles, subarachnoid space, and brain stem demonstrated normal appearance, and there was no evidence of mass lesion or midline shift. The MRI with and without contrast, performed 2 months later, revealed a tissue measuring 5 × 3 × 1 cm and weighing 12 g in the right orbital mass. The microscopic findings demonstrated a benign lesion composed of a mixture of angiomatous tissue and lobulated fatty tissue connected with skeletal muscle tissue, which was suggestive of hemangioma. The brain computed tomography (CT) findings showed that there were no abnormalities in the brain structure. There was diffused skin and subcutaneous soft tissue edema in the cranial area, which was more pronounced in the radiation therapy site. The ultrasound reviewed an inflammatory soft tissue in the right frontotemporal region progressing to the ear. The patient used atenolol 50 mg (twice daily), triamterene-h (half a pill every 12 h), acetylsalicylic acid (daily), and atorvastatin 20 mg (daily). In July 2016, she was hospitalized in the infectious diseases ward with a diagnosis of cellulitis and received oral antibiotic therapy (cephalexin) for 2 weeks. Owing to the lack of response to the treatment, she was then referred to the dermatology department. Swelling and slightly warm soft tissue along with erythema was observed throughout the facial surface and scalp. The swelling of the eyelids resulted in complete closure of the eyelids on both sides, which impaired the patient’s vision; however, the patient’s eye exam was normal (Fig. ). The complete blood count test was done for the patient (hemoglobin level: 14.7 g/dl, white blood cells: 5950 cells/mm3, neutrophils: 50%, lymphocytes: 35%, monocytes: 10%, eosinophils: 1%, basophils: 3%, sodium: 141 mEq/L, potassium: 3.8 mmol/L, blood urea nitrogen: 53 mg/dL, creatinine: 1.2 mg/dL, blood sugar: 163 mg/dL). Furthermore, the urinalysis and liver enzyme test demonstrated normal results. Based on the CT scan with and without contrast, no anomalies or bone lesions were reported. The brain MRI showed that there was an area of increased signal intensity on the axial T2-weighted images in the skin and subcutaneous tissue of the right cheek. Five months later, an elliptical biopsy at the margin of the lesion was done and the microscopic examination revealed a malignant vascular neoplasm composed of a poorly circumscribed dermal and subcutaneous tumor with an infiltrative growth pattern. In some areas, there were proliferations of solid epithelioid cells with vesicular nuclei, eosinophilic cytoplasm, and intracytoplasmic lumina, which intermixed through vascular channels with retiform architecture lined by plump hyperchromatic endothelial cells with a hobnail appearance. The lining of endothelial cells in some areas showed multi-layering plump and occasionally pleomorphic nuclei with scattered mitotic figures (Figs and ). The diagnosis was a low-grade malignant vascular neoplasm compatible with CHE. The patient was referred to undergo the immunohistochemistry (IHC) test. The IHC test demonstrated negative results regarding the tumor cell in MNF 116 and SMA; however, it showed positive results for CD31 and CD34 (Fig. ). Furthermore, Ki67 was observed to be positive in only 8% of the tumor cells. Based on the combination of histopathological findings and IHC results, the patient was diagnosed with CHE; therefore, she was recommended to undergo a complete lesion excision due to the involved cervical lymph node. The patient underwent partial excision of lesions and skin grafting. Post-surgery, she received chemotherapy by 100 mg thalidomide (daily). Following the thalidomide treatment, the erythema and swelling regressed, and she was able to open the eyelids. |
A 41-year-old G1P0 woman was referred to the emergency department of our hospital because of severe vaginal bleeding. As the patient was unconscious, her medical history was taken from her accompanying sister. The patient's history was significant for 18 weeks of amenorrhea and positive pregnancy tests, in addition to constipation and a misdiagnosis of incomplete abortion. As a result of the wrong diagnosis, an external obstetrician performed a dilatation and curettage (D&C) that ended up in perforating the uterus. Upon the clinical examination, the patient looked pale, her pulse was weak, her heart rate was 140 beats per minute, and her blood pressure was 80/40 mmHg. The transabdominal ultrasonography showed an empty heteromorphic uterus and excessive amount of free intra-abdominal fluid. Therefore the diagnosis of a perforated uterus was confirmed, and a ruptured ectopic pregnancy was suspected. The patient was moved immediately to the operating room to perform an emergent exploratory laparotomy and resuscitated with 6 full-blood units and 5 plasma units. A Pfannenstiel incision was made; the abdominal muscles and fascia were dissected. Upon reaching the peritoneal cavity, a big amount of blood clots was taken out and a fetus with his placenta inserted exclusively into the sigmoid colon were observed. The uterus was perforated in different locations on the contralateral side of the placental insertion. In addition, ileal and appendicular injuries were also observed. So the diagnosis of an abdominal pregnancy was achieved intraoperatively. The fetus was taken out, and the internal iliac arteries were ligated to reduce the hemorrhage. A hysterectomy was done due to the multiple large defects of the uterine wall. Regarding the wide placental insertion on the sigmoid colon and the potential risk of inducing additional hemorrhage by dissecting it, a sigmoidectomy was considered as the ultimate management. Therefore, the incision was dilated longitudinally superior to the umbilicus, and the sigmoid colon was resected (). The descending colon was isolated, and a skin colostomy was made.\nFinally, the intestinal injury was repaired before skin closure. The operation lasted for 7 hours. The patient's vital signs returned to their normal limits after the surgery and she was stable during the eight days of follow-up. The pathologic examination of the resected specimen showed the presence of normal chorionic villi invading the sigmoid wall (). However, due to the D&C, the endometrium was not fully evident when the uterus was examined microscopically. The endometrial remnants showed Arias-Stella reaction in the endometrial glands. |
A 30-year-old Caucasian man suffered from a distortion of his right ankle seven months prior to presentation. The incident happened when he was at work. Due to persisting pain in his joint he first saw a general practitioner. An X-ray image of the patient's right ankle showed no pathological findings. The joint was immobilized for six weeks in a plaster cast, which was then followed by physical therapy. Six months after the therapy, however, the patient still suffered pain in his ankle with no sign of any improvement. A magnetic resonance imaging (MRI) scan revealed an unknown but well-circumscribed localized tumor at the ventral part of the ankle, coupled with focal bulging and erosion of the tibia and talus (Figure ). The MRI detected no damage to the fibular collateral ligaments. On examination, about thirteen months after trauma of the ankle, tenderness to pressure was found at the ventral aspect of the right ankle next to the medial malleolus. A dorsal extension of the ankle was very painful. The collateral ligaments showed no insufficiency and a new X-ray still did not show any conspicuous findings. An ultrasound investigation showed a solid, homogeneous hypoechoic mass measuring 3.5 × 2.5 × 2 cm. It was not clear whether the tumor was directly connected to the joint. An impingement syndrome of the right ankle caused by a synovial hypertrophy was diagnosed preoperatively.\nThe tumor was resected through a ventral access. A brownish yellow tumor that was mainly solid was found during surgery. The tumor showed adhesions to the capsular of the patient's ankle and the complete tumor was treated with marginal resection (Figure ). A small hypertrophy of the cartilage below the tumor was also removed. However, the complete cartilage of the joint was not damaged.\nMicroscopically, the tumor was partially encapsulated and composed of round to polygonal cells. Some were spindle cells and some were multinucleated giant cells (Figure ). The diagnosis of localized tenosynovial giant cell tumor of the tendon sheath was confirmed on histopathology. Results of special stains indicated the presence of iron in both mononuclear and multinucleated giant cells in cytologic and histologic preparations.\nDuring follow-up the patient presented no complications. Investigations three, six, 12, and 24, as well as the MRI scan conducted 24 months after surgery, showed no recurrence of the TGCT (Figure ). To this day the patient is free of any symptoms. |
We present a case of a 65-year-old female patient admitted to the Cardiac Surgery Department in Wroclaw in January 2018 with severe mitral regurgitation (MR) and the history of ischemic heart disease, after elective percutaneous coronary intervention of the circumflex branch of left coronary artery with two drug-eluting stents (DES) implantation 4 years earlier. Furthermore, the patient diagnosed with many chronic conditions, such as metabolic syndrome, obesity with BMI 33 and gastroesophageal reflux disease. Currently, with an exercise dyspnoea for about 2 years, intensifying in recent weeks, she was hospitalized in the Cardiology Department for further diagnostics. The transthoracic echocardiography (TTE) revealed non dilated left ventricle with a normal systolic ejection fraction of 60%, and no evidence of segmental wall motion abnormalities, severe MR with the prolapse of the A2 segment and systolic restriction of the posterior leaflet. Colour Doppler showed a highly distinctive eccentric turbulent jet directed towards the lateral wall and the base of the left atrium with ERO 0.6cm2 and regurgitant volume of 60 ml. Additionally, in the performed coronary angiography, hemodynamically significant narrowing was found in the area of the previously implanted DES. The patient was then consulted by the cardiac surgeon and qualified for surgery. After admission to the Cardiac Surgery Department, as part of the pre-operative preparation, TTE was again performed, in which the severe MR was confirmed and no pathological structures in the right atrium were described. Due to the unclear image in the right pulmonary field, described by the radiologist in the chest X-ray (Fig. ), diagnostics was extended by performing a computed tomography of the chest, which excluded the presence of pathological shadow in the lung parenchyma. There was no referral to the atrial septum in the CT report. The patient was scheduled for mitral valve repair surgery and coronary artery bypass grafting (CABG) with the use of saphenous vein graft to the circumflex artery. During the standard procedure of commencing the cardiopulmonary bypass (CPB) and bicaval cannulation, it was found difficult to insert the cannulas from the atrium into both vena cavas. Therefore the cannulation was performed using the smaller cannula sizes, which eventually allowed to go on bypass. On the free wall of the atrial septum, there was a thickening and an excess of adipose tissue with a firm consistency and the size of a walnut, significantly impeding access to the operated mitral valve through the left atrium, and probably completely preventing surgery by the transseptal approach. In the transesophageal echocardiography (TEE), a characteristic image of LHAS was confirmed by the presence of hypertrophy of the septum, up to 2.7 cm, an hourglass shape with a characteristic indentation at the place of the fossa ovalis (Figs. and ). Based on the intra-operative TEE, as well as by analyzing the chest X-ray and CT scans, the diagnosis of LHAS was made. Due to the asymptomatic course of the LHAS and the complexity of the scheduled operation, the decision was made to leave the change intact. The mitral valve was replaced through the left atrial approach. The surgery was completed in a standard manner and the weaning from the CBP went uneventfully. The patient’s early postoperative period was a routine. |
A 59-year-old man reported with partially reducible swelling around ileal conduit for last 8 months, which was associated with intermittent dragging pain. He was diagnosed with adenocarcinoma of urinary bladder for which he underwent radical cystectomy with ileal conduit followed by adjuvant chemotherapy, completed 3 years back. He was on regular follow-up without any evidence of recurrent disease. He had no other comorbid conditions. On examination, he was found to have large parastomal hernia around ileal conduit which was partially reducible having small bowel and omentum as contents []. After thorough clinical and radiological evaluation, he underwent laparoscopic keyhole plus mesh repair (the surgical technique is described below) with an uneventful post-operative recovery.\nThe patient is kept in supine position. The entire team stands on left side of patient as most conduits are made on the right side. The camera surgeon stands near cranial end, while the scrub nurse stands near caudal end of the patient towards left side of surgeon. The monitor is placed on the right side of the patient, directly opposite to the surgeon. A Foley's catheter is placed in the stoma with balloon inflated as it helps to differentiate between the loop of small bowel forming the stoma and intestinal contents of the hernia.\nTotal four ports are used – one 10 mm camera port in the left anterior axillary line at the level of umbilicus, two 5 mm ports over the left midclavicular line in left hypochondrium and lumbar region 20 cm apart as right hand and left hand working ports, respectively. Another 5 mm port is placed for retraction in the right midclavicular line 2 cm beneath the costal margin.\nThe principles of surgery are, reduction of the contents, removal of sac, narrowing of the parastomal defect and mesh reinforcement. Pneumoperitoneum is established with Veress needle, ports made as described. Lysis of the extensive adhesions that are usually present has been done using both blunt and sharp dissection until the defect is delineated completely []. The contents are dissected and reduced and the defect is closed laterally with no. 1 prolene [] in continuous intracorporeal fashion, taking care not to constrict the stoma (usually checked by passing the tip of the instrument between the stoma and the defect). A composite mesh is placed in an intraperitoneal onlay manner [], laterally extending beyond the stoma site, with the absorbable collagen layer towards the intestines and the nonabsorbable polyester layer towards the parietal wall (Parietex, Covidien, Trevoux, France). Few seromuscular stitches are taken from the conduit to the parietal wall, to avoid a dragging effect. The mesh is then fixed using a combination of trans-fascial sutures and tackers (Strap 25R, J and J, Ethicon, NJ, USA), like in a conventional laparoscopic ventral hernia repair. Pneumoperitoneum was deflated, ports removed and skin closed with subcuticular stitches with absorbable suture material. The operative procedure took 108 min. In the post-operative period, oral liquids were started within 24 h and the patient was discharged on 3rd post-operative day. He was followed up for 2 years with no recurrence. |
A 27-year-old lady married one and a half years before and conceived 4 months before was having a normal growth of the fetus as per the ultrasound report. At 2 months of gestational age she had sudden onset of severe global headache associated with vomiting and one episode of generalized seizures for which she sought treatment in another neurosurgical center. CT scan of the brain revealed diffuse subarachnoid hemorrhage and foci of bleed in the right medial posterior temporal region (). It was suspected to be an aneurysmal bleed and CT angiogram was done which did not show any evidence of intracranial aneurysm. As there was early hydrocephalus she underwent right VP shunt. Gynaecological opinion was sought and in view of unprotected radiation and increased risk of congenital fetal malformations, she also underwent medical termination of pregnancy.\nSix weeks later she developed rapidly progressive weakness of the lower limbs and urinary retention for which she came to our institute. On examination she was conscious and oriented. Fundus was normal with no evidence of intraocular melanoma. Her motor power, sensations and reflexes were normal in the upper limbs. Motor power was 2/5 in right lower limb and 4/5 in left lower limb. There was no obvious sensory deficit. Her lower limb reflexes were brisk and plantars were extensor. She had multiple nevi over the body distributed over the face, back, thighs, legs, foot, sole or arms (). The nevi over the back and the sole had increased in the size and pigmentation in the recent past. Cardiovascular system, respiratory system and per abdominal examination were normal. No palpable lymph nodes felt. There was no spinal tenderness or deformity.\nMRI scan of the spine revealed multiple intradural extramedullary spinal lesions at D3-D4, D8 and D10-D11. All the lesions were isointense on T1W and hyperintense on T2W with no significant enhancement with the contrast ().\nShe underwent D4 and D8 laminectomies and total excision of the lesions at two levels. The lesions were intradural extramedullary dirty black in color, soft in consistency, easily separable from the spinal cord and were attached to the overlying dura. HPE and immunohistochemistry were consistent with metastatic deposits of malignant melanoma. Postoperatively she improved in her paresis by one MRC grade. On second postoperative day she started having frequent episodes of irrelevant speech and the next day she became drowsy with respiratory distress. She was intubated and ventilated. Repeat CT scan of the brain showed multiple small tumour emboli with evidence of right temporal bleed and diffuse cerebral edema (). The tumor emboli were clearly made out in the MRI of the brain (). She was given aggressive antiedema measures in spite of which she expired on the fourth postoperative day as a result of malignant cerebral edema. |
A 76-year-old African American lady presented to the hospital with new onset weakness and back pain. Her medical history consisted of essential hypertension, atrial fibrillation, abdominal aortic aneurysm, sick sinus syndrome, asthma, and gout. She reported that her back pain started after she came back from the bathroom at 4 : 00 a.m. while trying to sit down on the bed. The patient described the pain as shooting in nature, severe in intensity, and radiating down her left arm with no clear exacerbating or relieving factors. Over the following few minutes, she developed progressive weakness in her left lower and upper extremities and later on became unable to move her left leg which led to her being transferred to the Medical Intensive Care Unit. She also developed numbness in her left leg up to the level of the knee and in her left arm up to the level of the shoulder. There was no history of any urinary or stool incontinence or retention or any previous similar episodes.\nThe patient had been recently diagnosed with atrial fibrillation one month prior to presentation in an outside hospital and had since been maintained on apixaban 1 tablet 5 mg twice daily. Her other home medications included alprazolam, allopurinol, losartan, hydrochlorothiazide, and metoprolol. She reported full compliance with her medications prior to presentation. A dual chamber pacemaker was inserted 11 days before this visit in an outside hospital, and her other surgical history included a hysterectomy several years ago for uterine fibroids. She denied any history of smoking, alcohol abuse, or illicit substances usage. The patient did not have previous bleeding history.\nThe patient's vital signs on presentation were within normal limits, and her neurological exam showed a patient alert and oriented to time, place, and person with intact speech. Her motor power was 5/5 in her right upper extremity proximally and 4/5 distally and 3/5 in her left upper extremity proximally and 1/5 distally. In her lower extremities, the motor power was 5/5 on the right and 1/5 all over on the left. She had decreased sensation to light touch in her left upper and lower extremities. She had no clonus, and Hoffman and Babinski signs were negative bilaterally. Her reflexes were 2+ all over and symmetric.\nInitial lab tests were significant for hypokalemia (3.1 mmol/L), leukocytosis (12700 WBCs per microliter), and slight anemia with a hemoglobin level of 9.3 g/dl with unknown baseline. Her other lab tests including liver function and renal function were within normal limits.\nGiven her presentation of acute focal neurological deficit, a noncontrast head CT scan was ordered and showed signs of chronic vascular ischemic disease only. The patient continued to have severe back pain, and having a history of abdominal aortic aneurysm, an acute aortic dissection was suspected, and hence, a CT angiogram of the abdomen, pelvis, and thorax was ordered. The CT angiogram did not show any signs of aortic dissection. A CT scan of the neck was performed to investigate the cause of the left upper extremity weakness and numbness. It showed an epidural soft tissue density extending along the left aspect of the spinal canal causing compression and marked rightward deviation of the cord concerning for an epidural hematoma extending from C2-C3 level to C6-C7 ().\nAs the patient was on apixaban at the time, a decision was made to delay surgical treatment for 2 days to decrease the risk of intraoperative bleeding. A C2-C7 laminectomy, hematoma exclusion, and spinal fusion were then performed. On discharge, 9 days later, her physical exam was mostly unchanged from that on presentation except for the resolution of pain and improvement in motor power in her left lower extremity from 1/5 to 2/5 distally. |
An 88-year-old male patient presented to us with large fungating growth encircling the suprapubic cystostomy site of eight-month duration with gradual expansion in size. He underwent several surgical procedures for long urethral stricture, and as the measures finally failed, he has been on permanent suprapubic cystostomy for 25 years.\nPhysical examination revealed a fungating growth surrounding the suprapubic cystostomy site (). Biopsy of the lesion showed squamous cell carcinoma. Contrast enhanced computed tomography scan of the abdomen and pelvis revealed a tumor around the cystostomy tract without involving the bladder and lymph nodes were negative (). The upper tracts were normal and his renal function test was normal with serum creatinine of 90 μmol/L. Flexible cystoscopy through the cystostomy revealed no evidence of growth in the bladder. Cystogram was done to assess the possibility of perineal urethrostomy to place the urinary diversion after resection (). The cystogram showed the obstruction from the level of bladder neck and eliminated perineal urethrostomy as an option.\nThe surgery was performed as a combined procedure by both urology and plastic surgery team under general anesthesia. The growth was excised with a macroscopic margin of 1 cm from skin down to the rectus sheath and cuff of the bladder with the cystostomy tract also removed en bloc (). Intraoperatively, we found that the tumor did not invade into the bladder.\nFull-thickness reconstruction was done with inferior epigastric artery based vertical rectus abdominis myocutaneous pedicled flap from the left side. As shown in , skin incision was made vertically and the width of the skin paddle was decided by the defect in the excision site in a way to close the defect. The myocutaneous flap was harvested while preserving the posterior rectus sheath and the flap was delivered through the subcutaneous tunnel into the defect site without compromising the vasculature.\nA silicon catheter was passed through the center of the flap while taking care not to damage the vascular supply to the flap (). The catheter tip was placed into the bladder through the defect and the bladder defect was closed. The bladder wall was anchored to the under surface of the flap. The anterior rectus sheath of the flap was sutured to the muscular aponeurotic defect with size 2/0 polypropylene suture and then the deep dermal layers and skin were repaired accordingly. The laxity of the abdominal wall allowed the closure of the donor defect primarily with polyamide sutures.\nPostoperatively, the patient developed mild urine leak through the inferior border of the flap, but it settled with conservative measures by day 10. Otherwise, the patient had uncomplicated recovery after surgery. illustrates the healed surgical wound.\nHistopathology report revealed a well differentiated early invasive squamous cell cancer extending into the dermis (pT2), and the deep and radial resection margins were free of tumor ().\nThe patient did not undergo any other additional treatment modalities. At the follow-up of six months, the patient was without any symptoms. The abdominal wall strength was satisfactory and there was no incisional hernia. We plan to follow up this patient three-monthly. |
A previously healthy 17-year old boy was admitted to the pediatric ward due to a one-week history of fatigue, inspiratory left side chest pain with the intensity of 7/10, dry cough and nasal obstruction. He did not report any recent history of fever, dyspnea, night sweats, hemoptysis, ear or throat pain, abdominal pain, weight loss or recent alterations in his bladder and bowel habits. There were no known recent contacts with people presenting with infectious diseases, including tuberculosis. He recalled having had a fall and having landed on his left side while playing football 1 week prior to the onset of the symptoms. His family medical history and his past personal medical background was unremarkable except for an episode of acute bronchiolitis and an episode of community-acquired pneumonia that required hospitalization at the age of 2 years. He maintained a regular follow-up in the immunoallergology outpatient clinic due to a history of allergic rhinitis. His vaccine schedule was updated according to the national vaccination programme.\nThe adolescent denied alcohol or smoking habits and recreational drug use.\nOn admission at his first visit to the emergency department, he was afebrile, with no signs of respiratory distress, but with diminished breath sounds on the left side. He complained of pain on palpation of the left lower costal margin. A left costal grid and a chest x-ray was performed (Fig. ), which showed the same opacity on the posterior basal segment of the left lower lobe and no signs of rib fracture; thus, the patient was discharged with oral amoxicillin 1000 mg three times a day with the diagnosis of community-acquired pneumonia.\nDue to the progressive worsening of the productive cough with emission of purulent stinking thick green sputum, he returned to the emergency department 4 days after. On admission, he had a peripheral oxygen saturation of 98% (FiO2 21%), a heart rate of 95 bpm and an axillary temperature of 36.7 °C. His physical appearance was described as sickly, with no signs of respiratory distress, but with diminished breath sounds on the left lower lung, barely audible on the bases, and with crackles on the inferior two thirds of the left lung. The chest x-ray showed the same retrocardiac opacity on the left lower lobe. Laboratory studies including a complete blood count, glucose, ionogram, renal and hepatic function and c-reactive protein were normal, except for a leucocyte count of 15200/uL (neutrophil count of 12500/uL and lymphocyte count of 1700/uL) and a c-reactive protein of 172 mg/L. A chest computed tomography (CT) scan was ordered, which was suggestive of a consolidation with air bronchogram of the left lower lobe base associated with necrotizing characteristics and a central abscess measuring 5 cm in its greatest diameter (Figs. and ). Immunoglobulin, complement and alpha-1-antitrypsin levels were normal. Viral markers for hepatitis B and C and Human Immunodeficiency Virus (HIV) were negative. Serologic markers for rubeola, toxoplasmosis, CMV, EBV, herpes simplex virus, adenovirus, Chlamydia trachomatis and Chlamydia pneumoniae, Brucella and Mycoplasma pneumoniae showed no evidence of active infection. The virologic exam of nasopharyngeal aspirate and the bacteriologic and mycobacteriologic exam of the sputum were negative. Blood cultures for aerobic and anaerobic agents were negative.\nIt was decided to admit the patient to the pediatric ward in order to administer intravenous antibiotics and to maintain a closer surveillance.\nA course of intravenous ceftriaxone 2 g twice a day and clindamycin 30 mg/kg/day twice a day was initiated, with a favourable clinical outcome. He ceased complaining of chest pain on day three and reported a significant improvement of his cough. A single episode of fever was recorded on day seven without any further complications. After a consult with pneumology, it was decided to submit the patient to a bronchofibroscopy on day four after his admission. As his coagulation studies were abnormal with a prothrombin time (PT) of 16.7 s and a ratio of 1.5, 10 mg of intravenous vitamin K was administered twice with a decrease to a PT value of 15.9 s and a ratio of 1.4. A mixing test was ordered, with the result of a probable coagulation factor deficiency.\nThe bronchofibroscopy revealed the presence of a tracheal bronchus as well as numerous purulent secretions in the left posterior basal segmental bronchus, and excluded bronchial obstruction. Culture examination of bronchoalveolar lavage (BAL) fluid samples was positive (> 10^5) for Enterococcus faecalis (E. faecalis), which was sensitive to ampicillin, imipenem, nitrofurantoin, linezolid, teicoplanin, vancomycin and tigecycline, and resistant to levofloxacin, quinupristin/dalfopristin, and trimethoprim/sulfamethoxazole. BAL was negative for Mycobacterium tuberculosis DNA. Thus, it was decided to complete a 14-day course of intravenous antibiotics with ceftriaxone and clindamycin. An abdominal ultrasound was performed before he was discharged, which excluded the presence of a thoraco-abdominal fistula.\nNo complications were documented during his stay in the pediatric ward. A chest x-ray was repeated on day 12 showing an improvement of the opacity (Figs. and ).\nHe was discharged after 14 days with the recommendation to complete a four-week course of oral amoxicillin/clavulanic acid 875 mg/125 mg three times a day. He was referred to the pediatric and to the hematology outpatient clinic.\nOn his reevaluation 4 weeks after his discharge, he denied dyspnea, cough, chest pain, hemoptysis, respiratory distress or anorexia. His physical exam was unremarkable except for some crackles on the left lower lobe base and a slight decrease of breath sounds on the same side. The sweat test was negative. The patient completed the course of oral antibiotics without any complications; he is currently asymptomatic. |
A 22-year-old female patient complained about the amount of gingiva that was exposed when she smiled, which caused aesthetic discomfort for the patient. In the anamnesis, the patient reported not having any systemic problems, not continuously using any medication, and not being a smoker. A clinical examination confirmed the presence of an excessive exposure of the gingiva in the upper arch of teeth 14 to 24 (). The periodontal examination, which consisted of an analysis of gingival marginal bleeding, bleeding on probing, periodontal probing depth, and gingival margin position using a millimeter probe, confirmed that the periodontal tissues were in a healthy condition. In addition, conical beam tomography with an oral retractor was ordered to evaluate the bone and gingival tissue associated with the upper anterior teeth, and molding and photographs were also ordered.\nThe tomography showed that the bone tissue was at the level of the enamel-cementum junction and that the gingival tissue covered part of the anatomic crown (). Virtual planning was also performed using DSD, which showed the need to increase the size of the clinical crowns for better aesthetics (). With these data, it was shown that the patient suffered from altered passive eruption and, therefore, would need an osteotomy to enlarge the clinical crown. After this diagnosis, a flapless, minimally invasive surgical technique with piezoelectric ultrasound to perform the osteotomy and a mockup to guide the removal of soft tissue was proposed for the patient.\nSurgical planning was performed by DSD with the patient's smile photos and crown measurements using an 80% width to length ratio. After design of the optimal teeth, the distance from the cementum-enamel junction to the top of the bone crest and the position of the gingival margin in relation to the enamel-cementum junction was measured by tomographic analysis (). These measures were used as a reference for the wax model and mockup (). In addition, these measurements were used with tomography to plan the amount of osteotomy needed to maintain the biological space without recurrence of coronary covering by the gingival tissue.\nWith the mockup in position, the projection of what the gingival condition would look like after the surgical procedure was shown to the patient. After patient approval, the clinical crown-lengthening procedure was performed; this procedure consisted of marking the soft tissue height to be removed () and a sulcular incision to allow the removal of a gingival collar at the buccal face around all anterior and upper teeth without interfering with the interproximal region.\nAfter removing the gingival collar, the need for bone tissue removal due to the proximity of the new gingival margin was detected, since the bone tissue was practically at the level of the enamel-cementum junction (). A flapless osteotomy procedure was performed at the buccal bone with the aid of a piezoelectric ultrasound that induces bone tissue wear by ultrasonic vibrations (). According to the treatment plan, approximately 2.5 mm of bone was removed around all of the upper anterior teeth. Osteotomy was performed with minimal invasiveness, cutting only the bone tissue without causing damage to the root surface ().\nThen, the osteotomy height was confirmed by probing (), with the immediate results demonstrating an increase in the clinical crown length with minimal soft tissue trauma (). After 6 months, a good aesthetic result was observed with stability in the results obtained in the clinical crown-lengthening technique (). shows the initial condition () and final clinical condition 14 days after the surgical procedure (). |
A 52-year-old female patient reported with a chief complaint of nasal obstruction with facial swelling and facial pain on the right side of the face and also complained of a yellow-colored thick nasal discharge for the past 2 months. She was recently diagnosed with diabetes mellitus with blood sugar levels of 300 mg/dl and is being treated by oral hypoglycemic drugs. Extraoral clinical examination revealed diffuse swelling in the right maxillary region. On intraoral examination, prominent swelling was seen in the buccal vestibule extending from the infraorbital margin to the right angle of the mouth and extending posteriorly till second molar region. On palpation, the area was tender and the first and the second molars were mobile. To rule out the involvement of the maxillary sinus, computed tomography (CT) scan was advised. On CT scan of nose and paranasal sinus, she had the features of sinusitis with the destruction of walls of the right maxillary sinus as shown in . Ear, nose, and throat (ENT) consultation was taken. A strong suspicion of fungal sinusitis was made. They advised anterior rhinoscopy. Rhinoscopy revealed a thick yellow-colored mucopurulent discharge in the right middle meatus. A pinkish mass was seen in right middle meatus during fungal sinusitis of mucormycosis. Before endoscopic debridement, the patient's blood sugar level was controlled with human insulin 12 units twice a day. Endoscopic sinus surgery was performed, and debridement and clearance of right maxillary sinus was done. The patient was treated with an intravenous infusion of liposomal amphotericin B (5 mg/[kg body weight]) for 4 weeks. The dose was started with 1 mg/kg body weight and slowly increased to 5 mg/kg body weight depending on renal function which was continuously monitored by serum urea and creatinine levels. Serum potassium levels were also monitored and replenished accordingly. There was no adverse effect of antifungal treatment during the entire course. The patient responded well to surgical debridement and antifungal therapy. The patient was put on recall after every 1 month. The postoperative CT scan was taken at the recall as shown in . |
The patient was a 41-year-old female with past medical history of appendectomy and dysmenorrhea. She presented to the emergency department with nausea, severe vomiting, and acute pain in the lower abdomen. She reported having experienced asthenia and weight loss for one month. On clinical examination, abdominal distension and tenderness were discovered. Blood tests revealed leukocytosis with neutrophilia, and a contrast-enhanced abdominal computed tomography (CT) showed a 7 × 7 × 4 cm hyperenhanced mass in the cecum that caused complete bowel obstruction (). Also, a 5 × 3 × 3 cm right adnexal mass that compromised the ovary with intimate contact with the uterus was found (). Furthermore, the CT showed dilated loops in the small bowel (>4 cm), some of which had an enlarged wall thickness and presence of intraluminal fluid stasis ().\nWith these findings, particularly the observation of a mass through the CT scan, and due to the evident weight loss that the patient had undergone, neoplasia could not be ruled out. Surgery was decided, and at laparotomy, a volume of 200 ml of inflammatory fluid was found in the cavity. Most of the loops of the distal ileum were dilated, and a 7 × 7 × 3 cm cecum mass was discovered, which compromised the ileocecal valve and caused complete bowel obstruction. Surgical decision was straightforward, the cecum mass was completely resected, and a right hemicolectomy was executed. An ileocolic anastomosis was also performed during the procedure. Furthermore, the right adnexal mass that was previously identified through the CT scan (measuring 4 × 3 × 2 cm) was observed to be firmly attached to the ovary and the fimbriae and displayed a pale external capsule surrounded by a cystic component. Gynecology consultation was required, and due to the size of the mass and its characteristics, surgical removal of the right adnexal mass was performed. After completion, closure of the abdominal wall was performed, and the remainder of the procedure continued without any complications.\nPathology revealed a 4 × 3 × 2.5 cm blueish heterogenic mass that occluded 90% of the lumen of the cecum and the ileocecal valve. Microscopy revealed that the colon wall was invaded by glands and endometrial stroma. The colonic epithelium showed inflammatory changes and was negative for malignancy (Figures and ). In the ovarian parenchyma, an endometrial cyst was discovered, covered with siderophages. Glands and endometrial stroma were observed in the fallopian tube as well ().\nThe postoperative course of the patient was uneventful. She initiated clear liquids a day after surgery and was discharged once full diet was resumed. On follow-up controls, the patient was completely asymptomatic, without any pain or complications. |
A 38-year-old Japanese woman with a previous history of urticaria caused by an unknown allergen presented to a hospital in 2015 with nasal obstruction that had persisted for 1 month after symptoms of the common cold (e.g., low-grade fever, sore throat, and fatigue). An inspection of the nasal cavity with a soft fiberscope revealed a pedunculated polypoid tumor of ~20 mm in diameter on the posterior edge of nasal septum (). Magnetic resonance imaging (MRI) revealed a 20-mm tumor located in the epipharynx that originated from the posterior edge of the nasal septum, and T1- and T2-weighted images showed the same or slightly higher intensities compared to that of the nasal concha (). A chest X-ray examination showed no signs of a lung lesion. Enhanced computed tomography (CT) or MRI was not performed because of the patient's history of allergy due to an unknown allergen. A clinical examination revealed no signs of thyroid tumor, cervical lymphadenopathy, or other physical abnormalities. A biopsy of the pedunculated portion of the mass was performed, and it was diagnosed as a benign salivary gland-type tumor. The patient was referred to our hospital and presented for surgical treatment 2 months after first visiting the previous hospital in 2015. On the first inspection of the nasopharynx in our hospital, the main part of the tumor had disappeared and only the pedunculated portion of the tumor remained (). Plain CT revealed no invasive findings or metastatic lesions. The tumor was endoscopically resected 3 weeks after the patient's first visit to our department. In this operation, the tumor was completely excised with a surgical margin of ~5 mm using a needle electrode knife and was removed together with the periosteum from the vomer ().\nA histologic examination revealed a papillary structure with hyalinized fibrovascular cores lined by cuboidal to columnar stratified cells with round to oval vesicular nuclei and eosinophilic cytoplasm (). An increase in nuclear chromatin and mild nuclear atypia were found, but no nuclear polymorphism was detected. Some cells had clear chromatin; however, a nuclear groove and nuclear pseudoinclusion were absent. No mitotic figures were found and there was no necrosis (). A streaming pattern lining of the tumor cells with small round to oval nuclei (i.e., spindle cell component) was also found in some areas (). Psammoma bodies were not seen. The tumor showed invasive growth into the underlying fibrous connective tissue (). These morphological findings suggested polymorphous low-grade adenocarcinoma (PLGA) and low-grade nasopharyngeal papillary adenocarcinoma (LGNPPA) as differential diagnoses. Additional immunohistochemical examinations were needed to make a definitive diagnosis.\nImmunohistochemistry revealed that the tumor cells were positive for cytokeratin (CK) AE1/AE3 (), CK7, CK19, epithelial membrane antigen (EMA), vimentin (), and thyroid tissue factor-1 (TTF-1) () but negative for CK5/6, CK20, smooth muscle actin (SMA) (), calponin, p63, glial fibrillary acidic protein (GFAP), S100 (), CDX2, CEA, PAX8, CD10, DOG1, GATA3, SOX10, GCDFP-15, and thyroglobulin (). A pathological diagnosis of LGNPPA with TTF-1 (i.e., TL-LGNPPA) was finally made. The surgical margin was negative. Adjuvant therapy was not performed because of the free histopathological margin and information about the clinical characteristics of TL-LGNPPA reported in the relevant literature. There was no evidence of recurrence or distant metastasis at 5 years after surgery. The patient is currently being followed up and is satisfied with the good clinical course and lack of post-treatment symptoms. |
A 57 -year-old female presented with incidental ultrasonic evidence of left upper pole renal mass in Nov. 2001. Further evaluation with abdominal Magnetic Resonance Imaging revealed a mass in the upper pole of the left kidney with radiologic characteristics of renal cell carcinoma (). Left radical nephrectomy was performed sparing the left adrenal gland. The pathology specimen analysis showed a cystic mass 3 × 3 × 5 cm with yellowish friable tissue. Sections showed malignant epithelial cells, arranged in sheets. The picture was consistent with renal cell carcinoma, Grade II Fuhrman nuclear characteristics, confined to the capsule, neither pelvicalyceal nor vascular invasion was found (pT1bN0M0). Postoperatively she did not receive immunotherapy or chemotherapy. Apart from her hypertension which was well controlled with amlodipine and valsartan, subsequent clinical and radiological follow up showed no local or metastatic recurrence till 5 years after the operation then she stopped her visits. On October 2016 an incidental mass was found in the right adrenal gland during a checkup visit for the status of her right solitary kidney. Abdominal and pelvic computed tomography scan was done, revealing a well-defined mass with a smooth outline in the right adrenal gland measuring 54 × 48 × 39 mm with a central necrosis. The density of the solid component was 38 HU. In dynamic study the solid component showed significant enhancement after intravenous contrast administration (). Thorough hematological, biochemical and hormonal investigations were performed; all were within normal range. The results of laboratory examination showed the adrenal mass to be nonfunctional. The condition was well clarified for the patient and consent was taken to do right adrenalectomy. Under general anesthesia, in left lateral position through right transcostal incision, right adrenalectomy was done (). No any perioperative complications were recorded and she was discharged home on 4th post-operative day. Pathological examination revealed morphological and immunohistochemical findings in line with metastatic renal cell carcinoma, including positive staining for AE1/AE3, cytokeratin 7, vimentin, and CD10, and negative staining for CDX-2, inhibin, and synaptophysin (, ). During the last 2 years she has being on regular follow up. Whole body Positron Emission Tomography-Computed Tomography with fluorodeoxyglucose was performed, neither local nor metastatic recurrence was observed in any system. |
The patient is a 57 years old never smoker female who initially received a clinical diagnosis of pneumonia. As symptoms failed to resolve with antimicrobials, a subsequent CT scan of the chest revealed a partially cavitary mass in the right lower lung lobe. This imaging finding was followed with CT scans for two years at an outside facility showing slow growth. Eventually, a CT guided biopsy revealed mucinous adenocarcinoma of the lung with predominant lepidic pattern. A PET CT and MRI of the brain at the time did not show any other disease sites and she received a right lower lobectomy which confirmed the diagnosis and the stage as pT2bpN0M0 (IIA). Following surgery, the patient received adjuvant chemotherapy with carboplatin and paclitaxel for four cycles.\nShe carried a diagnosis of idiopathic autoimmune hearing loss, that had been successfully treated with mycophenolate mofetil. Her family history included lung cancer in both of her parents and her sister, all smoking related, as well as breast cancer in her maternal aunt.\nA year after her surgery, disease recurrence was documented on imaging in the right pleura. The same neoplasm was identified upon pathology review of a right pleural biopsy and she received local radiation therapy as salvage treatment. Follow up imaging in 3 months identified new lung nodules and the patient was referred to our institution. Figure shows the metabolically avid right pleural thickening that was radiated and one of the lung nodules at the time of disease recurrence following radiation. Molecular analysis of the original lobectomy material with next generation sequencing revealed a BRAF V600E mutation. Subsequently, she was initiated on combination of dabrafenib and trametinib treatment in the context of a clinical trial.\nWhile on the experimental drugs for two weeks, she experienced significant fatigue, persistent fevers up to 38 °C and generalized myalgias necessitating holding dabrafenib and trametinib. Nevertheless, symptoms persisted and infectious and rheumatology workups were initiated at the time. In addition, three weeks after stopping dabrafenib and trametinib, she was admitted for left eye vision loss and acute kidney injury. An ophthalmology exam with eye dilation indicated left central artery occlusion. Additional data from her history, exam and laboratory evaluation revealed the following: a history of recurrent sinusitis, acute onset visual loss and renal insufficiency during the current admission, and evidence of a saddle-nose deformity on exam which the patient believed was present for several years prior. She subsequently received a unifying diagnosis of granulomatosis with polyangiitis (GPA) on the basis of these findings as well as high-titer characteristic antibodies (p-ANCA titer 1:640, myeloperoxidase antibody > 30). Other lab results including rheumatology workup are shown in Table . Due to acute vision loss, giant cell arteritis was considered and a temporal artery biopsy was obtained and found negative. Her acute vision loss and creatinine elevation were thought secondary to retinal and renal vascular involvement by GPA. Nevertheless, review of the pleural biopsy and the resection specimen by pathology in retrospect, did not reveal any granulomatous change or vasculitis. She was initiated on rituximab, corticosteroids were successfully tapered, and further anti-neoplastic drugs were held. Lung cancer was followed clinically with scans.\nA year after diagnosis of GPA, a growing lung nodule was proven with biopsy to be malignant and was treated with SBRT. To-date, three years following GPA diagnosis and lung cancer recurrence, both conditions remain controlled without any further systemic therapy for lung cancer and while she continues on rituximab for GPA. |
In June 2002, a 36-year-old woman presented to her primary health care doctor with a history of flushing, diarrhoea, night sweats, and a clinically detectable mass in her left medial supraclavicular fossa. Her past medical history consisted only of essential hypertension for which she did not require prescribed therapy. Her family history included a brother with a diagnosis of sarcoma and two other non-first-degree relatives with primary brain malignancies.\nFine needle aspiration confirmed the diagnosis of medullary thyroid cancer, and in July 2002, she underwent total thyroidectomy with left-sided modified radical neck dissection and central compartment clearance. At this point, concerns were raised regarding optimal cytoreduction as the appearances of the central compartment, level 4 and level 5 nodes, were that of extensive disease. In order to maximise local disease control, she received adjuvant radical radiotherapy delivering 60 Gy to the thyroid bed.\nTwo years after completion of treatment, in February 2004, follow-up repeat imaging reported a recurrent nodule at level 4 of her neck. Subsequent resection confirmed this to be recurrent medullary thyroid cancer with no evidence of distant spread at the time. She continued to be monitored at the oncology clinic and remained disease-free until four years later, in May 2008, when computer tomography (CT) imaging revealed new pulmonary parenchymal metastases. These were closely monitored for the next 2 years with repeat imaging and measurement of calcitonin levels. In May 2010, it was decided that the patient should embark on systemic anticancer treatment.\nShe was offered participation in a phase 2 clinical trial with the agent lenvatinib (E7080) and she commenced treatment with 24 mg once daily in May 2010. One week into therapy, it was noted that she was marginally hypertensive with a blood pressure of 140/100 mmHg. No proteinuria was identified at this point, but she was commenced on 5 mg of amlodipine to manage hypertension. Monitoring of blood pressure and urinalysis continued as per study protocol.\nAfter two completed cycles of lenvatinib, CT imaging reported a reduction in size of all lesions. Further tumour assessment after 4 months confirmed a partial response to treatment with a 50% reduction of the sum of the long diameters of target lesions. She was experiencing various grade 1 toxicities throughout this time but was keen to maintain treatment given the good response. Due to the multiple low-grade toxicities, the dose of lenvatinib was initially reduced to 20 mg and thereafter to 14 mg.\nIn December 2011, 19 months after starting lenvatinib, she developed mild ankle oedema. Urinalysis carried out at the time identified proteinuria. A subsequent 24-hour urine collection identified 3.1 g/litre of proteinuria, equating to a urinary protein creatinine ratio (UPCR) of 625. The patient had not started any other medications and the incidence of proteinuria was felt to be lenvatinib related. Treatment with lenvatinib was ceased; however, due to concerns regarding possible intrinsic renal disease, she underwent screening for glomerulonephritis which was negative.\nA subsequent renal biopsy showed focal segmental glomerulosclerosis (FSGS) in two of twelve viable glomeruli, with tuft-capsule adhesion, hyalinosis, segmental intracapillary hypercellularity, and segmental splitting of capillary walls, predominantly in regions of segmental sclerosis. There was mild tubular atrophy, interstitial fibrosis, mild/moderate arterial intimal fibroelastic thickening, and mild arteriolosclerosis. Immunofluorescence showed no staining in glomeruli. Electron microscopy showed mild patchy reduplication of the basement membrane and effacement of only 20% of podocyte foot processes. There were no widespread electron dense deposits and no endothelial cell tubule-reticular inclusions. The endothelial cells showed no evidence of activation or damage. The appearances were consistent with a diagnosis of focal segmental glomerulosclerosis (FSGS). The lack of widespread podocyte foot process effacement suggests a secondary form of FSGS, which in the context of anti-VEGF treatment, could be mediated by microangiopathy. Whilst there was no histological evidence of acute thrombotic microangiopathy, it is possible that some of the pathological changes seen (splitting of glomerular capillary walls and mild arteriosclerosis) could be related to chronic low-grade endothelial cell damage. Based on histology, it is not possible to be certain whether the FSGS was caused by direct podocyte injury or whether it was related to endothelial cell injury. Histological slides are illustrated below in Figures , , and .\nThroughout this time, excretory function remained stable. Treatment with an ACE-inhibitor (ACEi) was introduced but due to poor tolerance and the quick improvement of the proteinuria after cessation of lenvatinib, the ACEi was stopped and patient's blood pressure was monitored closely.\nWithdrawal of lenvatinib had a marked effect on the levels of proteinuria, as illustrated in .\nThe patient continued follow-up at the renal clinic on a regular basis until July 2013, when she was discharged with no evidence of proteinuria, normotensive and with normal excretory renal function.\nAfter her discharge from the renal clinic and between 2013 and 2017, the patient was treated with vandetanib, nintedanib, and cabozantinib with no evidence of recurrent renal disease.\nE7080, also known as lenvatinib, is a potent inhibitor of the receptor protein kinases VEGFR-2 and VEGFR-3 but also displays inhibitory binding properties against VGFR-1, FGFR-1, and PDGFRα/β, albeit at significantly higher IC50 (half maximal inhibitory concentration, IC50). Its ability to restrain angiogenesis was shown on human umbilical vein endothelial cells (HUVEC) where E7080 inhibited VEGFR-2 phosphorylation and thereby capillary tube formation []. Apart from angiogenesis, E7080 decreased lymphangiogenesis in both the primary tumour of human breast adenocarcinoma cells in xenografts as well as in metastatic nodules in the lymph nodes of nude mice bearing these tumours []. Glen et al. showed in preclinical experiments that abrogation of FGFR and PDGFR signalling by E7080 inhibited invasion and migration of human melanoma cells lines (DX3) and human osteosarcoma epithelial cells (U2OS) []. Its potency against FGFR-1 differentiates E7080 from other currently approved tyrosine kinase inhibitors with antiangiogenesis properties [, ].\nThe preclinical data above were confirmed in several early phase human trials with E7080 in 2011 and 2012 in US, Europe, and Japan. Whilst establishing pharmacokinetic and pharmacodynamic properties of the drug, safety and preliminary efficacy was also well described. Lenvatinib was well tolerated at doses from 10 mg BID to 25 mg OD [–] and was associated with a reduction in disease activity biomarkers [], partial response, and stable disease according to response evaluation criteria in solid tumours []. These findings were further established in phase 2 trials and notably responses were demonstrated in thyroid cancer [–].\nApproval in thyroid cancer was granted in light of significant improvement in progression-free survival (PFS) compared with placebo in patients with radioiodine-refractory differentiated thyroid cancer in a phase 3 study (SELECT study) []. Lenvatinib improved median PFS over placebo by almost 15 months (HR 0.21; p < 0.01) and induced an objective response rate of 64.8%. The median survival results were diluted due to crossover of the patients from the placebo arm to the treatment arm; nevertheless, a subgroup analysis on patients stratified by age showed that older patients (>71 years old) had a survival advantage when treated with lenvatinib compared to placebo (HR, 0.53; p = 0.02), and the younger subgroup achieved a PFS of 20.2 months versus 3.7 m (p < 0.001) [].\nProteinuria and hypertension are the two most commonly reported side-effects of VEGF inhibitors and frequently the cause for therapy discontinuation. Proteinuria is used as a surrogate marker for glomerular damage and hypertension often accompanies and aggravates this.\nThe pathophysiology of proteinuria and glomerular damage in anti-VEGF therapy remains complex and far from well understood. Biopsy-proven cases of glomerular disease in anti-VEGF therapy are few; however, most have demonstrated changes in keeping with glomerular thrombotic microangiopathy (TMA) histology, with predominant endotheliosis and membranoproliferative changes [, ]. Other histological changes documented include cryoglobulinaemic glomerulonephritis, acute interstitial nephritis, collapsing and crescentic glomerulonephropathies, and FSGS plus TMA [–].\nIt has been theorized that hypertension is caused by decreased vascular production of nitrous oxide induced by inhibiting VEGF. This leads to renal haemodynamic compromise and subsequent proteinuria (much akin to exercise-related proteinuria) []. However, a mouse model study showed that glomerular injury preceded hypertension [] and many cases document glomerular injury in the absence of hypertension [], indicating that it cannot be the only trigger for proteinuria in anti-VEGF treated patients.\nInhibition of VEGF in podocytes (by injection of anti-VEGF antibodies or VEGF gene deletion) results in loss of endothelial fenestrations in glomerular capillaries, proliferation of glomerular endothelial cells, loss of podocytes, and proteinuria in mice [, ]. VEGF appears to be a crucial endothelial survival factor and its inhibition often manifests as TMA, a histology strikingly similar to that of severe preeclampsia—as placenta overproduces a soluble VEGF receptor (fms-like tyrosine kinase 1) that acts as a VEGF antagonist.\nIzzedine et al.'s 8-year follow-up study results from 2014 shed great light in anti-VEGF-related renal injury. It showed that in 100 patients who developed renal disease whilst on anti-VEGF treatment, the main histology associated with TKIs was minimal change disease and/or collapsing-like focal segmental glomerulosclerosis (MCN/cFSGS), a FSGC variant which is considered a separate entity to FSGS. In the same analysis, TMA histology was most frequently associated with VEGF-ligand targeted therapy (such as bevacizumab and aflibercept) suggesting two, possibly distinct pathophysiologies [, ] between renal damage caused by targeting the VEGF ligand as opposed to targeting the VEGFR tyrosine kinase domain. This could potentially be explained by considering the associations and signal transduction pathways between podocytes, endothelial cells, and VEGF. Podocytes produce vascular endothelial growth factor (VEGF), whereas VEGF receptor tyrosine kinases (RTKs) are expressed by both podocytes and glomerular endothelial cells.\nOur case demonstrates a secondary form of FSGS pathology which cannot confidently be attributed to TMA but could potentially represent the end result of chronic low-grade endothelial cell damage. The moderate histological findings were in keeping with a less-severe clinical course of the FSGS, with fast resolution of proteinuria and hypertension. More significantly, rechallenging the patient with additional three agents blocking the VEGF axis did not result in recurrence of the renal damage. |
A 64-year-old Japanese woman experienced pharyngeal discomfort late one night in July 2011, as well as pain in the right anterior region of her chest while coughing. She was brought to our hospital on the same night because the chest pain continued. A detailed examination showed right pleural effusion, based on which pleurisy was diagnosed. She was hospitalized on the day of admission and referred to our department the next day. Her past medical history revealed that, at the age of 26 years, she gave birth to her first child at 36 weeks of pregnancy, and underwent a total hysterectomy one month later because of an intractable uterine hemorrhage. At the age of 49, she underwent stripping of varicose veins of her lower extremities, and at the age of 62 years, she was diagnosed with arteriovenous fistulae of her lower extremities, for which she was being treated at a nearby dermatology clinic on an outpatient basis. She had a medical history of a skin ulcer, but had no history of easy and extensive bruising of the skin, enlargements of scars, or postoperative complications. There was no family history of vEDS, and our patient did not have a history of smoking or alcohol consumption.\nThe physical findings on admission were as follows: body temperature, 35.7°C; blood pressure, 94/60mmHg; pulse rate, regular at 67 beats/min; respiratory rate, 16 beats/min. Percutaneous monitoring of her arterial oxygen saturation in room air showed a blood oxygen saturation (SpO2) level of 95%. Our patient was conscious and lucid, and there were no abnormal findings on her neck. There was no abnormal heart sound, but her breathing sounds were diminished on the right side. The site of the right chest pain was around the second intercostal space at the right sternal border, and no tenderness was observed. There were no significant abdominal or neurological findings. Her face was normal. Thin subcutaneous veins were visible through the skin of the anterior chest region (Figure a), and pigmented scars due to cutaneous arteriovenous fistulae were found at the dorsum of her left foot (Figure b). Premature aging of her skin was found.\nOn admission, the collected blood samples showed an erythrocyte sedimentation rate of 56mm/h and immunoglobulin G levels that had elevated to 2025mg/dL. Her activated partial thromboplastin time, prothrombin time, fibrinogen level, D-dimer level and other laboratory findings were normal (Table ). A plain chest radiograph showed a right pleural effusion (Figure a); chest computed tomography (CT) showed pleural fluid with a CT value of 30 to 60 Hounsfield units in the right pleural cavity, which was accompanied by a region showing CT values comparable to those of pleural effusion, located dorsally from the area surrounding her right internal thoracic artery (Figure b,c). An abdominal CT showed a large number of saccular and fusiform aneurysms in her celiac artery, splenic artery, superior mesenteric artery, right and left renal arteries, and right and left common iliac arteries (Figure d).\nBlood samples collected eight hours after admission, at the time of referral to our department, showed hemoglobin levels of 9.5g/dL, and a plain chest radiograph showed an increase in the right pleural effusion compared with the image taken at the time of admission. Therefore, an exploratory pleural puncture was performed and a bloody pleural effusion was obtained. A drain was inserted into her thoracic cavity. Approximately 600mL of bloody pleural effusion was recovered during the two hours that followed the insertion of the drain. Her lung expansion was poor, so emergent irrigation and drainage and hemostatic surgery were performed using thoracoscopy. Surgical findings revealed the presence of an ecchymosis behind the mediastinal pleura that extended from the posterior surface of her sternum towards her right atrium. However, the site of bleeding, adhesions and cysts that caused the hemothorax were not found in her lung parenchyma or chest wall. Because the connective tissue was fragile, and the surgery could no longer be postponed, we affixed fibrin glue to the ecchymosis and completed the surgical procedure. Postoperative reaccumulation of the pleural effusion was not observed, and a blood transfusion was not required.\nThese skin findings, her past medical history and the presence of a large number of aneurysms were indicative of weak connective tissue disease. Our patient met two major diagnostic criteria for vEDS according to Beighton et al. [], thin translucent skin and arterial, intestinal or uterine fragility or rupture. For a definite diagnosis of vEDS, a biopsy examination of skin from her upper right arm was performed, along with analysis of the type III collagen production capacity of cultured dermal fibroblasts and an analysis of the COL3A1 gene. Histopathological examination of skin tissue sections stained with hematoxylin-eosin showed normal results. An electron microscope examination revealed that the collagen fibers were of an irregular size, which was most marked in regions surrounding the blood vessels. The amount of type III/I collagen had decreased by 14.7%, and a heterozygous missense mutation, c.2411 G>T p.Gly804Val (exon 36), was found in the COL3A1 gene. These findings confirmed the diagnosis of vEDS (Figure ).\nApproximately one month after surgery for the hemothorax, our patient was transported to our emergency department because of sudden abdominal pain. The clinical and surgical findings led to the diagnosis of acute peritonitis due to perforation of her digestive tract. Our patient was discharged from the hospital one month after laparotomy and drainage. In addition, our patient had a 38-year-old daughter, who was single. The daughter was asymptomatic, but we conducted the genetic tests because vEDS is an autosomal dominant inherited disease. The results showed that she carried the same genetic mutation as her mother. Currently, she and her mother are being treated on an outpatient basis. |
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