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A 50-year-old woman visited Seoul National University Dental Hospital, with a chief complaint of "desire to improve esthetics" because of the existing space between anterior maxillary teeth. The patient had uneven space between anterior maxillary teeth and showed deep bite Angle class II malocclusion. The problem list of patient's anterior maxillary part was as followed: asymmetrical zenith lines, abnormal incisal profile with disharmonious tooth axes and imbalances in tooth positions (). The midline was deviated and the maxilla was canted to the right. The proportion of tooth size was asymmetrical and there was wide diastema between central incisors, approximately 2 mm. On the first visit, irreversible hydrocolloid impressions of both maxillary and mandibular arches were taken, and diagnostic wax up was performed. To produce the most esthetic result, recurring esthetic dental (RED) proportion was used to establish the widths of the anterior six teeth as viewed from the frontal. The author has defined the RED proportion as the proportion of successive widths of the teeth viewed from the frontal, remaining constant as one moves distally. As it is well known, the golden proportion is limited to 62%, however, the RED proportion gives greater flexibility, as the dentist can define desired RED proportion, and an approximate 70% is preferred. Based on the RED proportion of 70%, diagnostic wax-up was performed, and the favorable results were predicted by space redistribution. If the right central incisor would be moved toward mesial and incisal directions 1 mm respectively, the left lateral incisor would be moved toward incisal direction 1 mm ().\nDeciding as for the treatment option, the MBT brackets (3M Unitek, Monrovia, CA, USA) were bonded to the anterior maxillary teeth according to their related positions, and the brackets of the right central incisor and the left lateral incisor were bonded in slightly cervical positions for extrusion. Then open coil spring (3M Unitek, Monrovia, CA, USA) was inserted between the right central incisor and the right lateral incisor in order to move the right central incisor in mesial direction. On the other hand, closed coil springs (3M Unitek, Monrovia, CA, USA) were inserted between the right central incisor and the left central incisor and between the right lateral incisor and the right canine, with keeping in mind not to overclose of the diastema and not to take the wrong distal direction for the right lateral incisor. After the spring and wire were inserted, all of the brackets and 016 stainless-steel round wire (Jin-Sung Industrial Co., Seoul, Korea) were ligated tightly and activation of teeth movements had begun (). The recall checks were carried out with two weeks intervals. The careful observation of teeth movements was fulfilled and open coil spring had been changed, if necessary. The occlusal adjustment of extruded right central incisor and the palatal surface of the left lateral incisor was carried out to remove occlusal interferences. The minute examination was performed for evaluating favorable teeth movements 6 weeks after brackets bonding procedure. Because the teeth were in proper positions in the maxillary dentition, all brackets and wire were removed, and the teeth surfaces were cleaned and polished. The fixed retainer was bonded to avoid the relapse of the moved teeth.\nOn the next visit, porcelain laminate preparation was performed with the silicone index (Exafine Putty Type; GC Corporation, Tokyo, Japan) attained from the diagnostic wax-up cast. Immediate dentin sealing (Adper Scotchbond Multi-purpose Adhesive; 3M ESPE, St. Paul, MN, USA) was carried out for achieving improved bond strength, fewer gap formations, decreasing bacterial leakage, and reducing dentin sensitivity ().- The final impression was taken with polyvinyl siloxane impression material (Express; 3M ESPE, St. Paul, MN, USA) using 1-step technique. Shade was carefully decided considering the prepared teeth and opposite mandibular incisors with shade guide (VITA System 3D-Master; VITA Zahnfabrik, Säckingen, Germany). The provisional restorations (Luxatemp; DMG, Hamburg, Germany) were fabricated directly with premade silicone index (Exafine Putty Type; GC Corporation, Tokyo, Japan) attained from the diagnostic wax-up cast.\nAfter 2 weeks, the final restorations was completed and tried in the mouth, all margins, contacts were verified (). The final restorations were bonded using resin cement (Variolink II; Ivoclar vivadent, Schaan, Liechtenstein). After delivery, as shown in , the gingiva was healthy and showed harmonious shapes and contours. The proper esthetics was obtained that the shade of surrounding tissues was stable and shown balanced properties, the proportion of tooth size was favorable and satisfied. The retainer was bonded for maximum retention of new teeth positions. The following check-ups of the patient were performed for 3 months after placement of the definitive prosthesis, and oral hygiene was maintained in excellent state and tooth alignment was stable. The patient was very satisfied with the appearance and the function. |
The first case was a 77-year-old man with a previous history of high blood pressure, dyslipidemia, diabetes mellitus and chronic coronary heart disease, stable for the last 5 years.\nIn 2011, the patient underwent radical nephrectomy for a localized clear-cell RCC tumor. In 2016, he presented stage IV RCC with metastases to the lungs and pancreas, and was treated with sunitinib for 16 months. Due to new progressive bone disease, he received ipilimumab and nivolumab. In June 2018 (7 weeks after ipilimumab was introduced), he was admitted to hospital because of immune-related grade 3 colitis and grade 2 liver enzyme elevation, for which he was treated with high-dose steroids. Hepatic impairment improved in subsequent laboratory tests, and the steroid dose was tapered until discontinuation.\nIn August 2018, due to progressive disease in the lungs and bone, the patient was started on a combination of lenvatinib plus everolimus. Only 7 days after treatment was initiated, he was admitted to the emergency department with nonspecific clinical deterioration. Laboratory tests revealed grade 4 liver enzyme elevation (mainly AST and ALT elevation) with laboratory coagulopathy. Hepatic autoimmunity tests and microbiology studies were negative. Portal vein thrombosis was excluded by Doppler ultrasound. An hepatic biopsy showed lobular hepatitis with presence of granulomas (Figs. and ). These histological findings were similar to those found with the use of ICIs, so immune-related hepatitis was diagnosed in relation to the previous use of these drugs and steroid discontinuation.[ Histological lesions such as hepatocellular necrosis or granulomatous hepatitis have been also described with the use of TKIs, so the involvement of lenvatinib could not be conclusively excluded in this situation.\nLenvatinib was withdrawn and high-dose steroids were re-started. The patient improved again, enabling the steroid dose to be tapered.\nTreatment with lenvatinib and everolimus was discontinued, and the patient was classified as stable disease at his last radiological evaluation. |
The patient was a 5-year-old boy who was the third child of a consanguineous marriage of second cousins. The history of pregnancy, labor, and delivery were all unremarkable. His mother was aware that he was not sweating at all and was non-responsive to pain since birth. He was also reported to be hospitalized several times for boots of fever without an underlying cause. The mother recalled that she lost one younger son due to gastroenteritis at age 10 months who also had a dry skin with anhydrosis. The patient had started chewing his limps and fingers following the eruption of his teeth. At age 4, he had been operated for fracture of left caput femoris. Two months after the operation he had to be re-operated for osteomyelitis and abscess formation in the same bone. He developed a cardiac arrest nearly 24 h after the operation and referred to pediatric intensive care unit.\nOn initial evaluation, he was found to have a dry skin and tissue defects in the tongue, gum, and fingers due to self-mutilation []. His X-rays revealed the absence of the left caput femoris due to osteomyelitis and abscess [] as well as autoamputations in several fingers and toes []. He was non-responsive to pain with absent pupillary reaction to light. He had several fever attacks (39° C) without any laboratory signs of infection and/or inflammation. He also showed bradycardia which did not respond to atropine treatment.\nHe was non-responsive to intradermal histamine and pilocarpine iontophoresis. His whole blood count, blood chemistry, serum and urine amino acids, humoral and cellular immunity tests were all non-diagnostic. His motor and sensory peripheric nerve conduction velocities were also normal. Cranial magnetic resonance imaging showed cortical and supratentorial atrophy with dilatation of the third and fourth ventricules []. He was diagnosed with HSAN type IV based on the clinical and laboratory findings. |
A 32-month-old Middle Eastern boy was born full term at a community hospital in Michigan with birth weight of 3135 g (15.0 percentile). He had normal prenatal ultrasounds. He passed meconium at birth and had no other complications including prolong neonatal jaundice or dehydration. His CF NBS showed serum IRT 139 ng/ml and was negative for the 40 gene mutations panel. At 1 month of age, he developed a wet cough without any other symptoms. He was followed by his primary care provider (PCP), and no treatment was given at the time. His symptoms continued on and off until 1 year of age. At 1 year, the mother noticed increased frequency of productive cough, lack of appetite, and poor weight gain. His weight-for-age percentile ranged from 0.3 to 5.0. His stools were reportedly normal. He had no excessive sweating. He was referred to an outside asthma/allergy specialist for evaluation of asthma. He was prescribed budesonide without any improvement. He had frequent pharyngitis and otitis media that were treated with oral antibiotics that reportedly helped treat acute infection, but the cough persisted. He was also prescribed a H2 blocker for possible gastroesophageal reflux disease, but no improvement in symptoms was noted. Family history was negative for CF.\nAt 30 months of age, he was seen by his PCP for one week of cough and fever. He was treated with amoxicillin. His symptoms continued to worsen despite oral antibiotics, and he had two episodes of small-volume hemoptysis. He was subsequently admitted for community-acquired pneumonia and influenza B. Chest X-ray showed diffuse ill-defined opacities in the perihilar area and diffuse bronchiectasis. During the hospitalization, pediatric pulmonary consult was obtained. Given the negative NBS, it was stated that CF was unlikely and no sweat chloride test was recommended. He had a normal videofluoroscopic swallow study. Immunodeficiency workup revealed elevated immunoglobulin levels, protective vaccine titers, and normal lymphocyte counts and response to phytohaemagglutinin, concanavalin A, and pokeweed mitogen. HIV test was negative. Pediatric gastroenterology was consulted for failure to thrive and recommended to continue high-calorie diet. He was discharged home on augmentin.\nTen days following discharge, he was seen at the immunology clinic. He was noted to have digital clubbing, worsening tachypnea, and crackles. With the concerning physical exam findings, a sweat chloride test was done with a result of 90 mmol/L (normal 0–29 mmol/L; intermediate 30–59 mmol/L; abnormal ≥60 mmol/L) []. He was referred to pediatric pulmonary clinic the same day. He was then admitted and treated for a CF exacerbation. Throat culture grew Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus (MSSA). Fecal elastase-1 was <50 mcg E/g stool (normal >200 mcg E/g stool). Lab results including comprehensive metabolic panel and vitamin A and E levels were normal. He completed two weeks of cefepime and tobramycin.\nAfter notifying MDHHS with the false-negative NBS results, the blood spot that was available at the NBS lab was retested using the new and expanded mutation panel (60 mutations). He was found to be homozygous for R1066C (c.3196C > T; p.Arg1066Cys) mutation. His care was transferred to our CF center, as per parents' request. Two weeks later, he was admitted for worsening respiratory symptoms and treated for a CF exacerbation. Vitamin D level was low at 25 ng/ml (normal ≥30 ng/ml). High-resolution computed tomography of the chest showed diffuse bilateral bronchiectasis (). Flexible bronchoscopy showed airway erythema and significant thick green secretions () that was positive for MSSA. |
A 59-year-old right-handed female desk worker with no significant past medical history suffered a distal radius fracture (AO classification 23-A2 type) of the right hand (Fig. ), fracture of the right clavicle, and multiple rib fractures with hemopneumothorax as the result of a high-energy traffic accident. The patient underwent initial treatment for her life-threatening conditions, including thoracic drainage and distal radius fracture fixation with plaster. Five days after the injury, the patient’s condition was no longer critical, and she underwent the definitive treatment of open reduction and internal fixation of the fractures of her clavicle and distal radius under general anesthesia. We had planned to fix the distal radius fracture using the usual FCR approach and a special plate designed for a volar rim fragment of the distal radius. We could not identify the absence of the FCR tendon preoperatively because of severe swelling of the distal forearm. At first, we wrongly identified the palmaris longus (PL) tendon as the FCR because it was the tendinous structure at the most radial location of the volar distal forearm. When we found the median nerve just radial to the PL tendon, we were then able to identify the anatomical abnormality described in this case (Fig. A, B). Therefore, we changed the approach to the classic Henry’s approach after we had identified and gently protected the PCB. When we exposed and protected the radial artery through the same incision, we noticed another abnormality when dissecting the deep layers of the volar distal forearm (Fig. C): after retracting the flexor pollicis longus tendon to the ulnar side, we found that an abnormal muscle existed just radial to the pronator quadratus (PQ) muscle (Fig. D). We diagnosed the abnormal tendon as the flexor carpi radialis brevis (FCRB) because of the wrist flexion and slightly radial deviation observed with the traction of the tendon, as described in a previous report []. We exposed the radius between the PQ and the FCRB muscles and fixed the fracture rigidly using a rim-fragment locking plate (2.4 mm variable angle LCP Rim Distal Radius Plate; Depuy Synthes Co., Tokyo, Japan).\nFour months after the operation, the patient had no pain or neurologic problems and the X-rays showed complete bone union of the distal radius fracture (Fig. ). Although a slight extension and flexion contracture of the wrist joint remained, the patient returned to her desk work without any disability. We checked for the existence of an FCR tendon in the opposite forearm, and could identify the thick FCR tendon by palpation and ultrasonic examination. |
A 63-year-old man presented in June 2014 to the High Technology Medical Center of Tbilisi (Georgia) with right upper quadrant pain following approval by the Ethic Committee of Tbilisi State Medical University (# 44/3 04.06.2014). On further evaluation, a diagnosis of colonic cancer (sigmoid) was established, and in 2013, he underwent sigmoid bowel resection. The histopathological analysis of the specimen showed a moderately differentiated adenocarcinoma and confirmed tumour-free resection margins. He did not receive any adjuvant treatment. He remained asymptomatic until May 2014, when he developed features of pain in his right hypochondrium and fatigue. Computed tomography (CT) revealed a 10.7 cm lesion in segment 4 and two smaller lesions in segment 6 with imaging characteristic of CLM (see , a,b), and a diagnosis of TXNXM1, stage IV was made. A biopsy was performed in December 2014, which revealed moderately differentiated adenocarcinoma in the liver, and a diagnosis of liver cancer secondary to CLM was established. He received super-selective TAE using 100–300 μm and 300–500 μm diameter microspheres in December 2014 for the 4 cm lesion in segment 4 of the liver with a complete obliteration of the tumour feeder branches. The post-procedural CT scan revealed a partial technical response while the remaining tumour in the cranial segment remained vascular.\nThe patient refused any chemotherapy; he underwent a second session of TAE for the same lesion in March 2015. The patient tolerated the embolization procedures well, experiencing just the insignificantly revealed postembolization syndrome lasting for a few days after the procedure. CT was performed 10 weeks after the second TAE session; a complete response was documented showing a necrotic lesion in segment 4 with no evidence of arterial vascularization.\nThe right lobe’s two smaller masses grew from 14 and 21 mm to 18 and 29 mm, correspondingly, as documented by CT 3 months after the second TAE session, and were treated by percutaneous RFA. Due to the subcapsular location of the lesions, hydrodissection was performed through percutaneously positioned 8 Fr diameter drainage catheters, and around 3000 mL of glucose solution was injected into the peritoneal cavity to achieve 10 mm of separation between the liver surface with the abdominal wall and the diaphragm (a,b). No complications occurred during the procedure. A repeated CT scan was performed one month after RFA ablation, demonstrating the complete response to it (a,b). The segment 4 lesion appeared to be necrotic and reduced in size, but there was evidence of this mass’ revascularization from the gastrohepatic trunk, which was not seen in the previous CT scan (). A third session of TAE in November 2015 for the segment 4 lesion was performed because of this, while the prior ablated masses did not show any features suggestive of recurrence. After this, the patient received a short course of nexatinplus- and capecitabine-based chemotherapy.\nThe follow-up CT scan performed six months later showed multiple new satellite lesions in both lobes. The decision was made to start oxaliplatin-based chemotherapy in combination with capecitabine (Xeloda) in April 2017, and a follow-up CT did not show any changes.\nFurther evaluation in October 2017 showed a progression of the disease on CT; chemotherapy started with 350 mg irinotecan infusions, which the patient received until 15 March 2018. In spite of this, he did not show any improvement; liver failure developed later, and he died two years and nine months after the RFA procedure. Concurrently, we explored the impact of RFA on the adaptive immune response in the absence of the influence of any other therapeutic modality, as he refused any other form of treatment. Peripheral blood samples were obtained before and after one and three months of RFA. The immunophenotypic analysis was accomplished within 24 h of sample collections for a panel of cellular and cytokine subsets including CD39+CD4+, CD4+ T cells, IL-10, IL-17, INF-γ and TGF-β. Additionally, the neutrophil/lymphocyte ratio (NLR) and platelet/lymphocyte ratio (PLR) were calculated as parameters of systemic inflammation.\nIn contrast to the percentage of CD39+CD4+ cells to the total CD4+ T cells in normal subjects, which is approximately 10%, the preprocedural (RFA) value was 75.2%; however, following one month of RFA, it declined to 36.7 % but rose again after three months to 62.3% ().\nSimilarly, we observed elevated levels of IL-10, TGF-β, IFN-γ and IL-17, which declined one month following RFA and remained unchanged until three months (see a–c).\nThese changes in cytokine levels as well as the CD39+CD4+ T cells’ frequency were accompanied by a reduction of gamma-glutamyl transferase (GGT) and systemic inflammatory markers NLR and PLR, while ALT and AST increased at one month and decreased at three months after RFA ().\nHowever, further assessments of immunomodulatory changes were not made, as the patient decided to opt for chemotherapy. |
A 28-week-old premature boy, with a birthweight of 1280 grams, was intubated with a 2.5 mm endotracheal tube via the nose and ventilated for severe hyaline membrane disease (HMD). After receiving two doses of surfactant, the premature neonate was successfully weaned off ventilation and extubated to nasal continuous positive airway pressure (CPAP). On day 9, his clinical course was complicated by a pulmonary hemorrhage, requiring re-intubation. He was given another dose of surfactant and stabilized on high-frequency oscillation ventilation (HFOV). The chest radiograph showed extensive bilateral pulmonary interstitial emphysema, with the left side more extensively involved in comparison to the right. A hemodynamically significant patent ductus arteriosus was treated by intravenous paracetamol. The baby's condition did not improve, and he was selectively intubated into his right main bronchus. The position of the endotracheal tube was radiologically confirmed, allowing the right lung to be oscillated while the left lung was rested. The baby was nursed on his left side for a period of 36 hours, after which the endotracheal tube was retracted into the trachea and secured in that position. The baby's ventilatory status subsequently improved, allowing for extubation on day 7 after the relapse. On day 28 of life, he presented with severe stridor, requiring re-intubation. Difficulty during intubation suggested that subglottic stenosis might be present. One week after this re-intubation a flexible bronchoscopy was performed, which revealed two major findings. The first was a Cotton grade 2 subglottic stenosis, and the other abnormality was near-complete obstruction of the bronchus intermedius. The subglottic stenosis was dilated to 5 mm with the aid of a balloon dilator (Boston scientific Mustang™ balloon dilatation catheter). The bronchus intermedius was extremely narrow, and a 2.2 mm flexible bronchoscope was not able to pass through the area of stenosis.\nAfter 2 weeks, the bronchoscopy was repeated and the subglottic region had improved to near normal in diameter. The bronchus intermedius stenosis, however, remained unchanged. A chest Computed Tomography (CT) scan was performed to determine the length of the bronchial stenosis. The CT scan confirmed that the stenosis involved a short segment and had a web like in configuration (Figure ).\nIt was decided to balloon dilate the stenosis under fluoroscopy. As the cardiac catheterization suite offered the best quality fluoroscopy, it was decided to perform the dilatation in the suite. The baby was intubated, and a guidewire was inserted into the right main bronchus under fluoroscopy. Water-soluble contrast was injected, and the position of the airway identified. A 3.5 mm coronary artery balloon catheter was inserted into the area of stenosis via the guide wire, and the position was confirmed by fluoroscopy. The balloon was inflated at 16 atmospheric pressure for 20 seconds. This was repeated for another 20 seconds before water-soluble contrast was reinjected, demonstrating significant decrease in the bronchial stenosis (Figure ). Following the procedure, the baby was ventilated for less than 24 hours. At follow-up bronchoscopy 2 weeks later, the stenosis had significantly improved, allowing a 2.8 mm flexible bronchoscope to pass comfortably through the stenotic region. The posterior part of the stenosis had completely resolved, with a small anterior shelf remaining. The baby was discharged with no known respiratory complications, and at follow-up bronchoscopy 6 weeks, after the latter dilatation procedure, the airway remained patent and the baby remained asymptomatic, with a normal chest radiograph. Follow -up bronchoscopy was done due to the risk of restenosis and the fact that the baby was from a rural area, with limited medical services. |
An 8-years-old boy was admitted in our tertiary care hospital with painful swollen and stiff left knee joint for last 15 days. He sustained trivial injury and did not inform about the incident to his parents and carried his daily activities for 7 days after which he developed fever with painful swelling of the left knee. His anxious parents took the kid to local general practitioner who prescribed oral drugs for 3 days. The kid does not show a response to the oral drugs and the fever and swelling increases gradually.\nLater the kid was brought to our center, on examination in the emergency room; the kid was febrile and anxious. His left knee examination shows swollen erythematous, warmth, and tender joint with fullness in suprapatellar region. Range of movements was painful and restricted. There was effusion in the joint which was clinically confirmed by positive patellar tap test. There was no evidence of any external injury to knee. Retrospective questioning following surgery he narrated an incident of thorn prick to his left knee while playing with his friends 15 days ago and he removed the broken twig of the thorn on the spot itself.\nRoutine blood examination was done, and the result was elevated total count (16.1×103), differential count (polymorphs - 73%, lymphocytes - 20%, and basophile - 07%). Erythrocyte sedimentation rate was - 28 mm/h and C-reactive protein positive. Hemoglobin was - 10.3 g/dl. Plain radiography of the left knee joint shows no obvious bony injury except for soft tissue swelling (). Magnetic resonance imaging (MRI) of the left knee joint was advised which was reported as extensive synovitis with effusion and thickened suprapatellar plica and multiple enlarged inguinal and popliteal lymph nodes with larges measuring 1.5 cm × 1.0 cm.\nThe suspicion of septic arthritis was raised. Knee arthrocentesis was done under sterile precautions, seropurulent fluid was aspirated was sent for analysis and culture and sensitivity. The kid was put on intravenous antibiotics and planned for knee arthrotomy and debridement.\nUnder appropriate anesthesia after preparing, painting and draping the left knee, under tourniquet, through an anterior midline linear incision with medial parapatellar arthrotomy knee joint was opened. Seropurulent fluid was found, with extensive reactive synovitis. Near total synovectomy removing abnormal looking tissue was done specimen sent for histopathological examination. On everting the patella a black spot () was noted on the articular surface on further tracing it was a foreign body appeared to be a piece of thorn and was removed. On retrospective inspection of the anterior surface of patella, an area of redness was seen appeared to be like a foreign body granuloma (). The tract was curetted (). No articular cartilage affection noticed. Through saline wash given and wound closed in layers over a negative suction draining tube. Intraoperative knee movements were found to be full.\nThe knee was immobilized in tube slab for 5 days and later started on continuous passive mobilization of knee according to pain tolerance. Culture and sensitivity were negative, histopathological report showed nonspecific synovitis. 1 week of intravenous antibiotics augmentin 30 mg/kg body weight/day (combination of amoxicillin and clavulanate potassium) with metronidazole 30 mg/kg/day, the kid responded well and got discharged with oral antibiotics (augmentin 375 mg a combination of amoxicillin and clavulanate potassium) for next 4 weeks. Complete range of motion was achieved by 6 weeks. At the end of 1 year follow-up he is completely asymptomatic, performing all his normal routine activities with no residual deformity or recurrence of any infection. |
A 72-year old, multiparous woman presented with a history of postmenopausal bleeding for one year. Clinical examination revealed a 12 week size uterus and no adnexal masses. Two firm vaginal lesions measuring 1 cm each were noted at the vaginal fornix, in the 3 and 9 o'clock positions. A third similar lesion was noted in the lower third of the vagina in the 7 o'clock position. An endometrial biopsy showed a FIGO grade I endometrioid adenocarcinoma in a background of complex hyperplasia with atypia. CT abdomen and pelvis showed no evidence of extrauterine disease or lymphadenopathy. The patient was taken to the operating room for a robot-assisted laparoscopic hysterectomy with bilateral salpingo-oophorectomy, pelvic and paraaortic lymphadenectomy and excision of the lesion at the lower third of the vagina. A V-care uterine manipulator was placed. The da Vinci® surgical system (Intuitive Surgical) with 3 arms was used. A 12 mm supra-umbilical camera port and two 8 mm instrument ports were placed 10 cm lateral to the umbilicus and a 12 mm assistant port was placed at the right upper quadrant.\nCO2 pneumoperitoneum of 15 mmHg was maintained throughout the procedure. The first specimen included the uterus, fallopian tubes and ovaries and was removed intact through the vagina. The lymph nodes were removed through the assistant port. Specimen bags were not used. The operative time was 2.5 h and the estimated blood loss was 50 cm3. The final pathology was that of endometrioid type adenocarcinoma, FIGO Grade III (). The tumor size was 13 cm and the depth of invasion was 2 of 2.1 cm of myometrium (98%). No lymphovascular invasion was seen. The vaginal lesion was positive for metastatic endometrioid adenocarcinoma. Lymph nodes were negative.\nThe patient was assigned a FIGO (2009) stage IIIB ().\nThe postoperative treatment plan included whole pelvis radiation and interstitial brachytherapy followed by chemotherapy with Carboplatin and Taxol. The patient received external beam whole pelvic radiation therapy followed by interstitial brachytherapy. The port sites were outside the radiated field. At the completion of radiation, complete regression of the metastatic vaginal lesions was achieved. Three weeks later the patient reported pain on the abdominal wall at the site of the right lateral instrumental port.\nThe clinical exam revealed a 2 × 3 cm tender mobile subcutaneous nodule at the port site. A CT abdomen and pelvis was obtained and revealed tissue density in the abdominal wall at the site of the right lateral 8 mm port and no evidence of intraabdominal disease (). Chest X-ray was unremarkable. A local excision was performed in the operating room and the pathology confirmed recurrent endometrial adenocarcinoma of endometrioid type (). The peritoneal cavity was not breached during the excision of the port site tumor. One week later the patient presented to the emergency room with abdominal pain, nausea and vomiting. CT abdomen and pelvis was consistent with small bowel obstruction with transition point at the terminal ileum, probably due to radiation enteritis. A 1.7 cm × 1.3 cm hypodense lesion on the surface of the liver, not present on the previous scan was noted. She was admitted and conservative management with nasogastric suction and intravenous fluids was initiated. On the second day of her hospitalization, she was found unresponsive. An attempt for cardiopulmonary resuscitation was not successful. Possible cause of death is pulmonary embolism. |
A 37-year-old male consulted our department with a 7-month history of mild thoracic spine pain after a fall; in addition to the thoracic spine pain he noticed a painful, slowly growing mass. He had no past medical history, and physical examination was unremarkable except for the presence of a hard and tender thoracic spine mass. His father died of hepatic cancer. Clinical blood examination did not show any abnormality, including alkaline phosphatase.\nInitial posteroanterior chest radiograph revealed the presence of a left-sided soft tissue mass located at the level of the aortic arch (). The lesion's margins and the absence of a positive “silhouette” sign with the mediastinal structures indicated an extrapulmonary and posterior position, a finding that was verified in the lateral chest radiograph. A CT examination was subsequently performed upon request, which showed a predominantly soft tissue tumour with internal areas of mineralisation. The mineralised elements were amorphous, scattered, ring-like, and arcuate-shaped. Rib destruction and infiltration was also noted. Furthermore, widening of the adjacent neural foramina was depicted and an MRI examination was done to clarify this finding. MRI verified neural foramina invasion as well as significant spinal cord compression and contralateral displacement. Despite that fact, the patient remained completely asymptomatic, which was attributed to an indolent and slow growth of the tumour. The mass showed low signal intensity in T1-w images and heterogeneous but predominantly high signal intensity in T2-w images. An associated superficial component of the tumour, which corresponded to the palpable finding, was also better demonstrated on MRI (). Mineralised areas that were depicted in the CT examination demonstrated low signal intensity in all pulse sequences. Gadolinium enhanced T1-w fat saturated images showed intense, heterogeneous tumour enhancement. The mass extended from the anterior to the left of the vertebral body at T3–T6 measuring 7.5 × 6 × 5.5 cm and in the left paravertebral space, displacing the aortic arch and thoracic aorta and at T4–T7 in the left epidural space displacing the spinal cord. At T4 the tumor extended posteriorly measuring 4 × 3 × 2 in the paraspinal muscles. Correlation of all the imaging findings with the clinical presentation suggested the diagnosis of a low-grade rib chondrosarcoma, which was verified after a CT-guided biopsy.\nThe surgical procedure was conducted in two stages. Posterior then anterior approaches were planned. For the first stage, the patient was placed prone and an incision was made in the midline extending from C5 to T7. The spinous process and bilateral inferior facets of T3 were removed. The posterior bony elements of T4 and T5 were removed. Instrumentation was placed from C5 to C7 and T3, T6, T7 and fixed with pedicle screws. A combination of Vertex and CD Horizon system (Metronic) of posterolateral fixation was used. Fusion was then accomplished by decorticating the transverse processes and an autograft with bone chips was applied after the exposed dura had been protected ().\nThe second stage of the procedure was performed 3 months after the first stage. The patient was placed in a lateral position with his right side down; surgical excision of the 5th allowed a large thoracotomy. The tumour had 3 lobes and was treated with liquid nitrogen (−17°C for 15 min/lobe). The equipment used was Candela CS5 (Spembly Medical) with up to 5 probes, which can be sterilized and reused. We used 3.5 and 10 mm rod-shaped and a plate probe (for small superficial tumors) with liquid N2 (55 psi) which can reach –196°C (tissue temperature is usually 30 degrees higher (). Subsequently, the tumour with a part of the 6th rib was resected. Macroscopically, the tumour removal was at least marginal. The wound was copiously irrigated and hemostasis was attained. The wound was closed in multiple layers.\nHistopathology of the resected specimens was grade 1 and grade 2 chondrosarcoma. According to the histopathologic examination of the resected specimen the tumour was marginally resected during the posterior approach and en bloc resected with disease-free margins during the anterior operation. Postoperative MRI and CT scan 6 months following the operation confirm the en bloc resection of the tumour (). |
A 48-year-old man presented to our hospital complaining of fresh blood in his stools that be had begun experiencing a year earlier, and shortness of breath that had begun half a year earlier. A gastrectomy on the pylorus side was performed for a duodenal ulcer. He reported a family history of cancer, with his mother having been diagnosed with breast cancer. He was hospitalized for a closer examination of anemia as his hemoglobin count was 3.9 g/dL. Physical examination showed anemia in the palpebral conjunctiva. Four white papules were observed between his eyebrows, two papules on the left corner of his mouth, and a papule on the left buccal mucosa. A histological diagnosis of papilloma and fibroma was made based on the papules on the buccal mucosa and between the eyebrows, respectively. A neck ultrasound showed adenomatoid goiter in the thyroid gland. Upper gastrointestinal endoscopy showed multiple flat, white polyps in the esophagus (Fig. ), and polyposis was similarly observed in the stomach. Pathophysiological findings revealed that the esophageal polyps were glycogenic acanthosis-like hamartomatous polyps. Lesions in the stomach constituted hyperplastic changes associated with chronic enteritis. Lower gastrointestinal endoscopy showed pulsating abnormal blood vessels exposed on the mucosal surface of the sigmoid colon (Fig. ). An abdominal contrast computed tomography (CT) scan showed artery-like vascular malformation in the wall of the sigmoid colon (Fig. ). CT angiography showed AVMs branching from the inferior mesenteric artery and inferior mesenteric vein (Fig. ).\nA clinical diagnosis of Cowden disease was confirmed. Genetic testing was not performed in accordance with the patient’s wishes. The melena was confirmed to be due to the AVM, and a sigmoidectomy was performed under laparoscopic guidance. Laparoscopic observations revealed tortuous blood vessels which expanded on the mesentery of the sigmoid colon (Fig. ). Intraoperative colonoscopy was used to confirm the position of the lesions from the lumen; the sigmoid colon was mobilized, and the rectum was dissected. Thereafter, the intestines were lifted up from a 5-cm small abdominal incision, the portion with lesions was dissected and an automatic anastomotic device was used to perform anastomosis inside the abdomen. Postoperative pathological findings showed vascular malformations that expanded from the submucosal layer to the mesocolon (Fig. ). Postoperatively, there were no complications or occurrence of melena, and the patient was discharged 7 days later. Recurrence was not observed 1 year post-surgery. |
A 74-year-old Japanese woman noticed a tender lump in her right breast. She immediately went to a breast clinic to get a breast cancer screening. She had no family history of breast and ovarian cancer. After a month, she was referred to our institution with suspicion of metaplastic breast carcinoma with a core needle biopsy at the breast clinic. Physical examination revealed a hard, tender, and 25-mm mass in the upper outer quadrant of her right breast and a palpable lymph node in her right axilla. Mammography indicated an indistinct mass on the mediolateral oblique view and the craniocaudal view. Ultrasound (US) showed an 18 × 16-mm, irregular-shaped, and hypoechoic mass with a suspicion of a spread to the nipple inside the duct (Fig. a) and several swollen lymph nodes in levels I to II (Fig. b). Magnetic resonance imaging (MRI) detected enhancement of a 17 × 17-mm indistinct mass surrounded with a non-mass enhanced segmental lesion toward the nipple side spreading a maximum of 74-mm range, which had no interaction with the chest bone, muscles, and breast skin, in the right breast tissue (Fig. ). Invasive carcinoma with multiple axillary lymph node metastases was strongly suspected on clinical examination and imaging. Histological evaluation of the biopsy for the mass revealed a tumor with the growth of oval and spindle-shaped cells and multinucleated giant cells, the infiltrating lymphocyte into the breast tissue, and hyalinization in the stroma. The multinucleated giant cells stained positively for CD68. A part of the oval and spindle mononuclear cells stained weakly positive for CD68. These tumor cells stained negatively for estrogen receptor (ER), progesterone receptor (PgR), and human epidermal growth factor receptor 2 (HER2). There was a focal hemorrhage without necrosis. Few non-epithelial atypical cells were observed in the breast duct, but no atypical epithelial cells consistent with breast cancer were detected. GCT of the breast, breast cancer with OGCs, and giant cell-rich sarcomas should have to be considered as differential diagnoses, and the pathological findings suggested most GCT. Fine needle aspiration biopsy for the swollen lymph node revealed only normal lymphocyte, even though metastatic lymph node was strongly suspected on US. However, we could not rule out the possibility that the biopsy tissue showed a part of malignant tumor with OGCs and biopsy for the lymph node was false negative, because there was a gap between the clinical presentation, such as a tender mass suggesting rapid growth and multiple lymphadenopathies, and the pathological presentation of biopsy tissue. To obtain further evidence of malignancy, the tumor was sampled using a vacuum-assisted US-guided biopsy again. The result was the same as the prior biopsy. After discussing the treatment plan with the patient, we performed mastectomy and sentinel lymph node biopsy according to a surgical procedure for node-negative breast cancer with a wide ductal spread. The resection tissue histologically revealed similar findings to the biopsy specimen. The tumor was composed mainly of oval and spindle mononuclear histiocyte-like cells and multinucleated giant cells (Fig. ). The mitotic figure of these cells did not stand out. There was no evidence of malignancy, and only intraductal epithelial hyperplasia around the tumor, which did not fill the criteria of ductal carcinoma in situ (DCIS). No sentinel lymph nodes contained malignant cells, and we concluded the lymphadenopathies were a response to the inflammation around the tumor. Immunohistochemically, a high proportion of the multinucleated giant cells stained positively for CD68 (Fig. ). A part of the oval and spindle mononuclear cells stained weakly positive for CD68. These cells were negative for CK OSCAR, GATA-3, and MGB1 (Fig. ). These findings were consistent with the GCT of the breast. The patient received no adjuvant therapy because GCT-ST is usually considered as a benign tumor. She is being followed up with regular clinical examinations without any symptoms of recurrence after 1 year past from surgery. |
A 47-years-old man visited our clinic with complaints of a foreign body sensation and hoarseness that had begun 18 months earlier. He worked as an office worker. On direct laryngoscopy, there was a submucosal bulging mass at the left ventricle and the shape of the mass did not change during phonation (). CT showed a 2.2×1.7 cm benign-looking mass in the left larynx and at the supraglottic and glottic levels (). We performed LMS under the working diagnosis of laryngocele. Complete excision was impossible because of the hard, fixed nature of the mass. We finished the operation and planned to subsequently remove the mass at a later date via a lateral thyrotomy approach. One month after the patient was first discharged, diagnostic imaging revealed a highly enhanced mass on the T2-weighted MRI with delayed enhancement on the T1-weighted MRI. The differential diagnosis was a schwannoma or hemangioma (). Two months after the LMS, we performed a lateral thyrotomy without a preliminary tracheotomy. A round encapsulated mass was identified after dissecting the soft tissues and it was easily separated without injury to the adjacent tissues. Several holes were drilled around the window. Using nylon, the window was pulled through the holes and primarily closed (). The patient's dyspnea and dysphagia improved on the first postoperative day. After 2 months, the man's voice became better than before, but some hoarseness still remained. The left vocal fold showed a bowing feature on laryngoscopy during phonation. The laryngeal nerve was noted to be intact on the laryngeal electromyography. So this bowing feature seemed to be an acute postoperative change and we have followed up the patient to observe his voice on an OPD basis.\nThe gross tumor specimen measured 2.2×1.7 cm. The cut tumor section was yellow. On microscopic examination, the tumor was characterized by cellular Antoni A areas alternating with myxoid, loose degenerative Antoni B type areas. Immunohistochemically, the cellular areas were positive for S-100 protein (). |
A 49-year-old man with a history of acromegaly was admitted to our hospital with the concern of recurrent shortness of breath and dyspnea on exertion during the previous 2 years, and he had experienced an episode of presyncope 2 weeks prior without any further evaluation. He was a chef in a local restaurant for almost 30 years. He had no family history of any diseases and no past history of hypertension, diabetes mellitus, sleep apnea, or sudden cardiac death. He did not smoke or consume alcohol. The patient provided a history of stereotactic radiosurgeries twice in a decade or so and adherence to treatment with a somatostatin analog (octreotide given 40 mg once per month through intramuscular injection) at the time of diagnosis 20 years before. The patient was overweight and moderately nourished. He was 1.85 m (73 inches) tall, weighed 134 kg, and had a body mass index of 39 kg/m2. His blood pressure was 110/60 mmHg, and his heart rate was 92 beats/min with sinus rhythm. He had distinct skeletal features that included prominent superciliary arches and nose bridge, enlargement of the tongue and lip, and large hands and feet. Cardiac auscultation revealed irregular premature beats and pathological third heart sound, and a systolic murmur was discovered over the apex and aortic area. Bilateral extensive borders of cardiac dullness were noted. His physiological reflexes were present without any pathology. An electrocardiogram demonstrated sinus rhythm with wide (160 ms) QRS duration of left bundle branch block (LBBB) (Fig. ). The patient’s condition was classified as New York Heart Association (NYHA) stage III–IV.\nOn admission, magnetic resonance imaging showed pituitary macroadenoma. Given the symptoms described, we arranged blood testing of myocardial injury markers showing an elevated brain natriuretic peptide level of 740 pg/ml indicating cardiac failure (Table ). Hormone laboratory tests performed subsequently demonstrated excessive secretion of GH and IGF-1, twofold greater than the reference normal upper limit, which was consistent with pituitary macroadenoma (Table ). Other routine analyses of liver and renal function were roughly normal.\nA Holter monitor was ordered for underlying arrhythmias to explain the patient’s dyspnea, chest discomfort, and presyncope. It demonstrated sinus rhythm with an average heart rate of 68 beats/min, frequent ventricular premature beats, and nonsustained ventricular tachycardia (up to 2200 ms) (Fig. ).\nA chest x-ray showed a cardiothoracic ratio (CTR) of 78%. Echocardiography showed diffuse impairment of left ventricular (LV) systolic motion, reaching an LVEF of 16%. We noted hypertrophy of the ventricular septum at 18 mm, ventricular dilation, with LV diameter of 72 mm. The right ventricle and atrium and the left atrium were also dilated with moderate mitral regurgitation and mild tricuspid regurgitation. There was no associated systolic anterior motion (SAM) of the mitral valve. Dyssynchrony of the biventricular systolic motion was apparent.\nGiven an exertional component to the symptoms together with echo presentations in order to better exclude ischemic cardiomyopathy, coronary angiography was performed, which showed normal coronary arteries without stenosis, and left ventriculography applied simultaneously revealed an EF of 20% with diffuse LV hypokinesis.\nGiven the patient’s previous medical history of acromegaly, the absence of obstructive coronary artery imaging findings or segmental dyskinesia, family history of hypertrophic cardiomyopathy (HCM), symmetric hypertrophy, as well as absence of SAM of the mitral valve, acromegaly-induced cardiomyopathy was confirmed, which was absolutely opposed to coronary heart disease (CHD) and HCM.\nThese results indicated that it was probably not a case of hereditary cardiomyopathy; therefore, we diagnosed the patient as having secondary dilated cardiomyopathy due to acromegaly, even taking it a step further progressing to congestive heart failure secondary to acromegaly-induced dilated cardiomyopathy.\nChronic excess of GH and IGF-I secretion affects cardiac morphology and performance [], so etiological treatment for acromegaly-induced cardiomyopathy is crucial to suppressing GH secretion or blocking GH action for the sake of reversing acromegaly-induced cardiomyopathy. The mainstay of treatment acknowledged globally is surgical resection of the pituitary adenoma [], which was unfortunately considered high-risk given our patient’s cardiac condition (NYHA stage III–IV). Although stereotactic radiosurgery combined with somatostatin analogs and GH antagonists administrated previously were effective in suppressing hormones, they could not help his cardiac function. Therefore, we carefully administered diuretics, vasodilators, angiotensin-converting enzyme inhibitor (ACEI), β-blockers, and spironolactone for management of heart failure following the current guidelines []; in the meantime, octreotide (200 μg/day) was administered for the control of GH excess. After good compliance of pharmacotherapy and a regular medical examination regimen for nearly half a year, the serum GH and IGF-1 concentrations decreased from 32.50 ng/ml to 1.98 ng/ml and 627.00 ng/ml to 229.10 ng/ml, respectively, but the patient was hospitalized again because of uncontrollable cardiac failure. Accompanied by the normalization of GH and IGF-1 levels, the patient’s cardiac function did not seem to take a favorable turn upon readmission. Though echocardiography showed a recovered EF value from 16% to 28%, a significant ventricular mechanical dyssynchrony was detected as formerly. Electrophysiological study was performed using a nonaggressive stimulation protocol, which revealed a nonsustained ventricular monomorphic tachycardia []. In the presence of overt ventricular dyssynchrony, complete LBBB, LVEF< 35%, inducible ventricular tachycardia, and symptomatic heart failure despite guideline-directed medical therapy, surgical indication was rarely assessed by neurosurgeons, and stereotactic radiosurgery together with pharmacotherapy produced infinitesimal effects. Therefore, we boldly recommended cardiac resynchronization therapy with defibrillator (CRT-D) implantation based on device implantation official guidelines [, ]. The patient underwent CRT insertion finally and was discharged to home 5 days later, pharmacotherapy continued as usual (Fig. ).\nTelephone follow-up was arranged, and the patient claimed symptom improvement following the device insertion 1 month later and was basically back to normal life. We required that he return for follow-up at 1 month, 3 months, and 6 months after the interventional therapy. The patient has been followed in our outpatient clinic for nearly half a year now. During his last visit, echocardiography identified improved LVEF of 54%, and a chest x-ray showed reduced CTR of 60%. The patient was in NYHA functional class II (Fig. ). |
A 14-year-old Thai girl was born via cesarean section due to premature rupture of the membrane with a birth weight of 2500 g. She is the first child of a consanguineous (second-degree relatives) couple. Both parents are healthy and have never had fractures. During her first year of life, she had delayed motor development and growth failure. At one year of age, she could not sit by herself and weighed 7.5 kg (< 3rd centile). She presented to our hospital at 14 months of age with fractures of both femora without a history of significant trauma. She was found to have ptosis of both eyes with normal teeth but no blue sclerae. She was small for her age. Her weight was 7.8 kg (3rd centile) and her length was 68 cm (< 3rd centile). Skeletal survey showed diffuse osteopenia, multiple healed fractures of the right humoral shaft, both tibiae and fibulae. Spine radiograph showed flattening and indentation of vertebral bodies (Fig. ). A diagnosis of OI was made and intravenous bisphosphonate therapy (pamidronate 1 mg/kg/dose for 3 days) was initiated and given every 3 months. However, she sustained 1–2 long bone fractures per year from minor trauma. She required multiple corrective osteotomies to correct her deformities. At the last follow-up, she was 14 years old, weighing 20 kg. She could not walk due to her long bone deformity (Fig. ). Remarkably, although she was in a special education class due to physical disabilities, her cognition was appropriate for age. She could talk fluently and do mathematics properly.\nPrenatally, her younger sister was found to have a dilated fourth ventricle by an ultrasonography. She was born at term via cesarean section because of previous cesarean section and was diagnosed with hydrocephalus at birth. At 4 months of age, she had her first fracture without a history of a significant trauma, leading to a diagnosis of OI. Physical examination revealed a head circumference of 38 cm (> 95th centile) with a wide anterior fontanelle (3 × 3 cm.) and blue sclerae. She had global developmental delay (could not hold her head) and hypotonia. MRI of the brain demonstrated a large posterior fossa cyst connecting with the fourth ventricular system, moderate hydrocephalus, hypoplasia of cerebellar hemisphere with absence of cerebellar vermis, and hypoplasia of corpus collosum. She was also diagnosed with vesicoureteral reflux grade V and gastroesophageal reflux requiring tube feeding. The patient had multiple hospitalizations because of recurrent urinary tract infections and pneumonia. She expired at the age of one year.\nSixteen known OI genes, BMP1, COL1A1, COL1A2, CREB3L1, CRTAP, FKBP10, IFITM5, LEPRE1, PLOD2, PPIB, SERPINF1, SERPINH1, SP7, TMEM38B, WNT1, and MBTPS2, were amplified from 200 ng of genomic DNA using the Truseq Custom Amplicon Sequencing kit (Illumina, San Diego, CA). 286 amplicons which covered all the 226 exons (28 kb) of the target genes were sequenced by Miseq (Illumina, San Diego, CA) using 2 × 250 paired-end reads. SNVs and Indels were detected by Miseq reporter software. The proband was found to harbor a homozygous mutation, c.6delG, p.Leu3Serfs*36 in WNT1. The mutation has never been reported in Human Gene Mutation Database (HGMD; ) (Fig. ). The mutation was subsequently confirmed by PCR-Sanger sequencing. Segregation analysis was performed by using primers, WNT1-E1F: GGT TGTTAAAGCCAGACTGC and WNT1-E1R: ACCAGCTCACTTACCACCAT. The results revealed that the patient was homozygous, while her mother was heterozygous for the mutation (Fig. ). |
A 61-year-old male patient who had undergone distal gastrectomy and gastroenterostomy due to gastric ulcer 20 years ago presented with new onset fatigue, oral feeding problems, and anemia. The upper gastrointestinal system endoscopy revealed an obstructing tumor at the gastroenterostomy site. Histopathologic examination of the endoscopic biopsy showed moderately differentiated adenocarcinoma. The PET-CT revealed a hypermetabolic mass in the gastric anastomosis site along with hypermetabolic activity in the superior mesenteric vein (SMV) suspected with tumor thrombus (). A contrast-enhanced thrombus misgiving for tumor was detected within a 4 cm segment of the SMV proximal to the splenic confluence that completely obstructed the lumen on triphasic computed tomography (). Mesenteric venous drainage was maintained through collateral veins that drained into the portal vein. Portal vein tumor involvement was not detected, and the portal vein was fully patent.\nDecision for surgery was made due to tumor obstruction. On surgical exploration, a tumor that originated from the gastroenterostomy anastomosis site with near-complete obstruction, infiltrating the surrounding tissues was observed. Firstly, to ensure that thrombus was tumor, the SMV was dissected and opened vertically near the splenic confluence under vascular control (). The SMV was completely occluded with no blood flow. The thrombus was extirpated within the SMV by direct removal and by using a Fogarty catheter (). Following recanalization of the SMV, reflow was allowed and the vein was closed with primary repair. The thrombus was sent to frozen section, and the result revealed tumor. Therefore, the patient was considered to be in the metastatic stage, but palliative surgery for gastric cancer was decided due to luminal obstruction. Gastric tumor tissue was completely dissected from the surrounding tissues followed by near-total gastrectomy and Roux-en-Y gastroenterostomy. The patient was discharged with low-molecular-weight heparin treatment without any problems in the intraoperative and postoperative period.\nHistopathologic examination of the surgical specimen revealed gastric invasive adenocarcinoma. Infiltrated surrounding serosal fat planes by gastric tumor was detected (T4). Six surrounding lymph nodes from specimens were resulted as metastatic (N2). Lesions removed from the SMV have been reported to be tumor thrombi (T4N2M1) ().\nPostoperative abdominal computed tomography showed no evidence of thrombus in the SMV, but the SMV was obliterated and the drainage was still provided by collateral veins. The patient received 5 cycles of systemic paxlitaxel and carboplatin adjuvant chemotherapy. He is at his 22nd-month follow-up with extensive liver, peritoneal, and omental metastases. |
A 60-year-old Caucasian woman with the chief complaint of pain for 6 years in the bilateral pre-auricular region was referred to the Maxillofacial Surgery Department of the University of São Paulo Hospital. She reported that she visited several dentists, who recommended care in mouth opening and feeding, without any improvement. Additionally, another clinician prescribed pain killers (acetaminophen, ibuprofen, and tramadol) six months prior to our appointment. However, her symptoms remained unchanged. Past medical history included hypertension, depression, and referred osteoarthritis of the right knee, and was regularly taking 25 mg atenolol and 100 mg sertraline, daily. The patient denied a history of trauma or parafunctional habits.\nClinical examination revealed a limited mouth opening of 25 mm (A), clicking in the right TMJ, and intense bilateral articular pain. Some teeth were absent, there was occlusal contact only at anterior teeth, and the median line was aligned (B). TMJ radiography revealed radiopaque images in both TMJs (). The CT confirmed the presence of loose bodies in TMJ bilaterally. Those bodies measured approximately 13 mm in the left, and 8mm in the right side (A and 3B). There was a reduction of TMJ space associated with sclerosis. Due to the slow evolution process associated with clinical and imaging characteristics, the diagnostic hypothesis of bilateral SC was raised.\nUnder general anesthesia, the TMJs were accessed directly by pre-auricular incision with bilaterally endaural extension, allowing for a good trans-surgical visual approach and good postoperative healing. Cartilaginous bodies of each joint were removed and analyzed (A and 4B). They were characterized by irregular surface and stony consistency. There was no evidence of alteration of the condyles or the fossa. Histopathological examination revealed fragments of lamellar bone tissue in continuity with fibrocartilaginous tissue, surrounded by fibrous connective tissue (C), confirming the diagnosis of bilateral SC.\nCT performed after 13 months of surgery showed both joints with the absence of loose bodies (A). The patient experienced improvement of mouth opening at 35 mm (B), as well as the preservation of protrusive and latero-protrusive movements. Occlusion was reestablished (). She remains without articular clicks, pain, or facial nerve deficit, with no signs of recurrence. |
A nine-year-old Caucasian female presented with symptoms of reflux and postprandial gagging, dysphagia, epigastric pain, and fecal withholding. Her medical history was significant for prematurity, born at twenty-five weeks gestation and related conditions of prematurity including retinopathy, anemia, and lung disease. She was born with a mild ventricular septal defect and a patent ductus arteriosus that failed to close with two trials of indomethacin and required surgical ligation. After birth, she spent four months in the neonatal intensive care unit, requiring supplemental oxygen and placement of a percutaneous endoscopic gastrostomy tube for feeding. She was diagnosed with esophageal glycogenic acanthosis (Figures –) found on upper endoscopy performed at age twenty-two months for symptoms of reflux and feeding difficulties. She had retinopathy of prematurity that was treated with laser surgery and strabismus that was treated with botulinum injections. Macrocephaly, global developmental delays, and a limited attention span were present in early childhood. MRI of her brain at age 7 years showed signs of mild periventricular leukomalacia and no other abnormalities.\nOn exam, the patient was developmentally delayed for her age, and her head circumference was 56.4 cm, which is macrocephalic for age (98th percentile for head circumference in a 9-year-old girl is 55 cm) []. Skin exam showed well-healed scars from prior surgeries but no lipomas or other mucocutaneous features of Cowden syndrome. The remainder of the examination was unremarkable.\nDue to her clinical symptoms, upper endoscopy was performed, and a variegated fungating mass with very prominent lymphoreticular nodularity and friability was found along the medial aspect of the lesser curvature of the stomach at the juncture of the body with the antrum. The size of the mass was difficult to estimate on endoscopy but appeared to be 2 cm in width and 3 cm in length on a pedicle that was about 1.5 cm at the base (Figures –). Biopsies of the mass were obtained at several levels, including the base and tip. The remainder of the stomach, esophagus, and upper duodenum were also sampled showing reactive gastropathy, mild reflux changes, and mild active duodenitis, respectively. Microscopy of the biopsied gastric mass was interpreted to be a ganglioneuromatous proliferation with small foci in the lamina propria of spindled cells in fascicles with slightly atypical nuclei, positive on S100 protein and SOX-10 immunohistochemically stained tissue sections (Figures and a single, well-formed ganglion cell was noted in one of the areas of spindle cells, positive for neuron specific enolase (NSE) by immunohistochemistry (Figures and ). Ki-67 was negative in the spindle cells, supporting a low proliferative index. The spindled cells were negative for CD34 and CD117, helping exclude gastrointestinal stromal tumor. Epithelial membrane antigen (EMA) was negative, suggesting against a perineuroma. Keratin AE1/AE3, SMA, and melan-A were all negative, suggesting against a spindled carcinoma, melanoma, and leiomyomatous proliferation. GFAP was negative, as sometimes can be seen in Schwann cells and ganglion cells [].\nThree weeks later, a partial gastrectomy was undertaken to completely remove the mass, confirmed as a ganglioneuromatous proliferation of the diffuse ganglioneuromatosis type (Figures and and ). No polygonal or columnar cells typically seen in paragangliomas were noted, and the presence of ganglion cells excluded schwannoma and neurofibroma. Thus, the diagnosis of diffuse ganglioneuromatosis was confirmed. The patient's symptoms of reflux, postprandial gagging, dysphagia, and epigastric pain resolved following surgery. |
A 45-year-old male, a chronic smoker with past history of acute exacerbations of COPD was brought to the emergency department of our institute following an accidental exposure to ammonia gas at a cold storage when the pipe burst. He escaped the place of accident but started complaining of severe burning in the eyes and throat as well as progressive shortness of breath. He was brought to the emergency by his colleagues and the history was obtained from them. On arrival in the ER, he was breathless. His eyes were congested and he was finding it difficult to talk. On examination, he had tachycardia (pulse rate 110/min) and tachypnea (respiratory rate 24/min) but the blood pressure was 130/90 mmHg. The examination of the oral cavity revealed copious pink frothy secretions. Chest examination revealed bilateral polyphonic wheeze. He was drowsy but was able to respond to commands and the oxygen saturation was 85% at room air.\nDecontamination was done by removing all his clothes and irrigating the eyes with normal saline using nasal prongs. Both methylprednisolone (125 mg intravenously) and nebulized salbutamol were administered, the latter being difficult to perform due to copious secretions in the oral cavity. The patient was shifted to medical intensive care unit for further management. At this point intubation was contemplated as vigorous suction failed to get rid of the secretions. It was decided by the treating team to first administer 0.2 mg of glycopyrrolate injection to reduce the secretion and try intubation if this failed.\nWithin minutes the secretions reduced significantly. The patient was nebulized using beta 2 agonists again. He improved significantly and oxygen saturation of 95% was achieved on a FiO2 of 0.3 delivered by ventimask. He was started on prophylactic antibiotics and steroids were continued along with beta 2 agonists for another 48 hours. No further dose of glycopyrrolate was needed. Indirect laryngoscopy was performed to evaluate hoarseness of voice and it revealed an edematous vocal cord.\nTopical cycloplegics were used to reduce eye discomfort. Within next 48 hours the patient improved significantly. The chest X-ray and the high resolution CT scan of chest did not reveal any significant alveolar damage. A pulmonary function test revealed mild obstruction. The patient was discharged after a 72 hour stay in the intensive care unit. |
A 35-year-old male was hospitalized for a 4 months complaint of worsening difficulty in swallowing, predominantly to solids, accompanied by mild loss of weight. Hematology and biochemistry laboratory tests were within normal limits, and standard chest radiography did not reveal any lung or mediastinal lesions.\nContrast-enhanced computed tomography of the chest showed a well-defined 3 × 3.5 × 4 cm homogeneous mass on the right side of the distal esophagus, proximal to the gastro-esophageal junction (Figure ). Transesophageal ultrasound was not available, but upper gastrointestinal endoscopy showed a smooth submucosal bulge in the distal segment of the esophagus, resulting in evident localized narrowing of the esophageal lumen. The overlying mucosa was intact.\nDecision was made to perform elective excision of this mass lesion and, since the distal esophagus is usually more accessible from the left, this was carried out employing a left-sided video-assisted thoracoscopic surgical (VATS) approach. Using three 5-mm ports, the lower esophagus was dissected and the mass was identified (Figure ). Attempts to enucleate the mass were unsuccessful and, upon incising the mass to perform a frozen section procedure, thick creamy fluid leaked out confirming its cystic nature (Figure ). Consequently, layers of the decompressed cyst were dissected off the esophageal musculature with ease, the cyst was excised, and the specimen was retrieved through a limited thoracotomy incision. The continuity of the esophageal mucosa was confirmed by infusing methylene blue solution into the esophageal lumen while occluding the esophagus at the gastro-esophageal junction (Figure ). The muscular edges of the esophagus were reapproximated, and the resection site was buttressed with the mediastinal pleura.\nThere were no postoperative complications. The patient returned gradually to full oral intake and was discharged home on the fourth postoperative day in an excellent general condition. He remains symptom-free after 4 months of follow-up. Histology confirmed the lesion to be an EDC, with dual muscular layer and a characteristic columnar ciliated epithelial lining surrounded by severe chronic inflammatory infiltrate. |
A 31-year-old woman with a history of left common iliac vein thrombosis presented with symptomatic lower abdominal and left groin superficial varicosities associated with itching, swelling, and discomfort. Vital signs revealed a blood pressure of 114/80 mmHg and pulse of 72 beats/minute. Physical examination confirmed the presence of large tortuous varicosities at the lower abdomen and left groin (). The patient had previously been diagnosed with left common iliac vein thrombosis during her first pregnancy, 10 years earlier, that was only managed with enoxaparin injections. Over the next few years, she developed lower abdominal and left groin varicosities that worsened significantly during her second and third pregnancies without confirmed recurrent deep vein thrombosis (DVT). The patient denied leg swelling prior to the DVT event. She also denied any history of abdominal trauma, other thromboembolic events, or family history of vascular anomalies. Computed tomography (CT) venography revealed compression of the left common iliac vein by the right common iliac artery without evidence of acute thrombosis, indicating a diagnosis of May-Thurner syndrome- (MTS-) related anatomy ().\nCatheter-based venography with hemodynamic pressure measurements confirmed May-Thurner anatomy with sequelae of chronic DVT in the left iliac vein and cross-pelvic drainage via pelvic and abdominal wall varices (). Successful recanalization was performed using percutaneous transluminal angioplasty with stenting of the left common iliac vein (20 mm x 55 mm Wallstent) and left external iliac vein (14 mm x 60 mm Protege). The patient was managed with apixaban (5 mg twice daily) for three months and clopidogrel (75 mg daily) for one month that was changed to aspirin (81 mg daily) in the long term. A 3-month follow-up CT venogram indicated a patent left iliac vein stent. However, the patient continued to present with painful, though slightly improved, lower abdominal and left groin varicosities.\nGiven these persisting symptoms, we performed successful stab phlebectomy of the large superficial abdominal varicosity. We also treated the deeper feeding branch and groin varicosities with ultrasound-guided sclerotherapy using a sclerosing foam (two injections of 1 cc of 3% sotradecol mixed with 2 cc of room air). The procedure resulted in complete resolution of the symptomatic lower abdominal and left groin varicosities (). The patient did very well at 6- and 12-month follow-up visits while on aspirin, and a repeat CT venogram indicated a patent left iliac vein stent. |
A 32-year-old male with no significant past medical history presented to our facility with a history of worsening pain and swelling in the medial aspect of his proximal left lower extremity over the past several years. Anteroposterior (AP) and “frog leg” lateral radiographs of the left lower extremity revealed a dense lesion consistent with dense matrix, adjacent to the medial aspect of the femur (). An MRI of the left lower extremity revealed that the lesion was of extraskeletal origin, arising from the adductor magnus, without any involvement of the femur (, ). The solid heterogeneous mass was located in the posterior compartment of the proximal-to-mid thigh and measured 9.6 × 7.3 × 13 cm in the transverse, AP and vertical dimensions, respectively. Multiple tiny hypointense foci, which corresponded to the calcific density noted on the prior plain radiographical studies, were noted centrally. The mass was otherwise of intermediate soft-tissue intensity on T\n1 weighted images and moderately hyperintense to muscle on T\n2 weighted images. Following intravenous administration of gadolinium, moderate enhancement of the mass, excluding the central portion, was observed. Superiorly and inferiorly, the margins of the mass were indefinite, and the mass demonstrated peripheral vasculature, which was most prominent at the cephalad and caudad margins. The mass was centred within the adductor magnus muscle fibres that were displaced around the mass. Anterolaterally, the mass was very closely approximated to the posterior cortex of the femur and the fascial margins that separated the quadriceps and the posterior compartments (Supplementary videos). No obvious signal abnormality was noted within the femur to indicate invasion or primary osseous origin. Post-excision pathology confirmed the presence of mesenchymal chondrosarcoma.\nIn the interval, surveillance imaging for metastatic disease with CT scan of the chest, abdomen and pelvis was performed. At 18 months, multiple bilateral non-calcified pulmonary nodules concerning for metastasis were identified (). The most prominent pulmonary nodule was present within the posterior right base, measuring 2.3 cm in the greatest dimension and abutting the pleura (). With regard to the abdominal and pelvic series, a new right adrenal lesion appearing as a hypodense pedunculated mass measuring 1.6 cm in the greatest dimension was noted. There was no evidence of osseous metastases. |
A 46-year-old male with a history of severe developmental delay, hydrocephalus, and seizure disorder presented to the hospital with blunt head trauma after a ground level fall. Work-up revealed bilateral acute subdural hematomas for which an external ventricular drain was placed. Despite intensive care management, the patient deteriorated to brain death. He was subsequently evaluated for organ donation. Abdominal computerized tomography (CT) scan () revealed an “elongated structure with metallic components in the upper portion of the IVC that extends into the right atrium.” There was no medical history of a prior procedure, or symptoms, to explain the incidental finding. The radiologist's interpretation and presumptive diagnosis were a retained atrial pacing wire.\nHe subsequently underwent procurement for organ donation after brain death. At the time of cross-clamp, the previously identified foreign body was transected when the right atrium was incised for exsanguination. During the back-table dissection, it was apparent that the foreign body had eroded into the posterior wall of the IVC, extending down the retrohepatic IVC (Figures and ). It also created a calcified reaction at the junction of the suprahepatic IVC and right atrium, adjacent to the left hepatic vein (LHV) and middle hepatic vein (MHV). We removed the foreign body () and performed a venoplasty () of the posterior wall of the IVC and of the common wall of the LHV and MVH, so that the outflow of the LHV and MHV was not compromised after transplant. We discovered that the foreign body was, most likely, a fractured CVC due to the overall appearance and interval markings.\nThe liver recipient was a 65-year-old woman with cirrhosis due to alcohol abuse; her Na-MELD score was 40 at the time of transplant. She underwent caval-sparing total hepatectomy and deceased donor liver transplantation via piggyback technique: the donor suprahepatic IVC was anastomosed to a common orifice of the recipient's right and middle hepatic veins. We did not alter our immunosuppressive therapy or prophylactic antibiotic regimen. Additionally, we did not initiate any anticoagulants or antiplatelet agents beyond our standard postoperative protocol. Postoperative imaging showed normal velocities and waveforms on ultrasound () and unremarkable appearance on axial CT () of the hepatic vein anastomosis. The patient otherwise had an uneventful postoperative course and has had stable allograft function with no venous outflow issues for >8 months after transplant. There were no reported complications in the other organ recipients. |
After falling off a mountain bike down an incline into some brush, a 49-year-old male mountain biker presented to an outside ED with normal vitals, severe vertigo, nausea, intractable vomiting, profound hearing loss, and tinnitus. A CT was performed, which showed opacification of the ear canal, but did not comment on any abnormalities of the inner ear. The patient was transferred to our facility for further management. On examination, there was a spontaneous right-beating nystagmus and the facial nerve was intact. A tree twig was embedded in the left external auditory canal, obscuring visualization of the tympanic membrane.\nTemporal bone CT demonstrated a linear foreign body projecting from the external auditory canal to the oval window, and an additional, separate small foreign body projecting into the vestibule. Presence of extensive intralabyrinthine air was detected radiographically (). On axial view, air bubbles were seen in the vestibule, posterior semicircular canal, and the scala vestibuli compartment of the cochlear basal turn, as well as in the lateral and superior semicircular canals (). Preoperative audiogram conducted at bedside revealed normal hearing on the right side and moderate-to-severe mixed hearing loss on the left.\nThe patient was diagnosed with a traumatic PLF with extensive pneumolabyrinth due to penetrating temporal bone injury and was taken urgently to the operating room less than one day after his inciting injury. A three- centimeter tree twig was lodged in the ear canal and found to be penetrating the tympanic membrane. Postauricular approach included mastoidectomy and intraoperative assessment of the middle ear ossicles and extent of injury. The long process of the incus was dislocated but still attached to the malleus, the stapes was deeply embedded into the vestibule, and the oval window was completely open but covered by blood clot. All penetrating foreign bodies were extracted. Temporalis fascia was used to seal the oval window and a stapes prosthesis was placed. The tympanic membrane perforation was repaired.\nPostoperatively, the patient had rapid and significant improvement of his vertigo. On physical examination, there was minimal spontaneous nystagmus. A four-week postoperative audiogram revealed a mild-to-moderate mixed hearing loss in the left ear with continued improvement at six months. |
The patient is a 19-year-old female with a history of 6q24-related TNDM and Hashimoto's thyroiditis who was diagnosed with neonatal diabetes mellitus at birth.\nShe was born to a 25-year-old G3P2 mother, with a weight of 1927 grams, at 36 weeks of gestation via vaginal delivery after induction due to IUGR at an outside hospital. The pregnancy was significant for intrauterine growth restriction noted at 5 months of gestation. The pediatrician was at the delivery, and no resuscitation was needed. Apgar scores were 8 at 1 minute and 9 at 5 minutes. Macroglossia was noted. Umbilical hernia and hypotonia were not found. The infant was noted to have hypoglycemia on day of life one and was given intravenous glucose and went on to develop hyperglycemia. Glucose levels were the 400 mg/dl, and an insulin drip was started. The infant was transferred to the NICU from the outside hospital due to hyperglycemia and concerns for necrotizing enterocolitis (NEC). Due to concerns for NEC, oral feeds were discontinued and she was placed on TPN, providing a steady glucose load. No surgical intervention was necessary for the NEC. Glucose was difficult to control even while on TPN with blood sugar levels ranging from the 100s to >200 mg/dl. At about 1 month of age, glucose levels started dropping and insulin was weaned. During this time, due to an IV infiltrate, IV insulin was briefly discontinued and blood glucose was 268 mg/dl. Insulin infusion was restarted and was discontinued at about 1 month of age. Blood sugar levels ranged between 80 and 150 mg/dl. The infant was discharged shortly afterward, feeding ad lib and checking blood sugar before meals. She required intermittent subcutaneous insulin at home, which was finally discontinued at approximately 4 months of age. Since then, the patient did not require insulin or have diabetes symptoms. All developmental milestones were on time. At 15 years of age, she chipped her tooth and went to the dentist for evaluation. The dentist noted extensive tooth decay. Jaw X-rays showed bone loss of nearly 80% in the left mandible. Due to her history of TNDM, she was sent to her primary care provider for further workup and diabetes screening where blood glucose was 369 mg/dl and urinalysis showed positive ketones. She was sent to the emergency department, and laboratory examination showed blood glucose of 361 mg/dl, Na+ of 131 mEq/L, K+ of 3.4 mmol/L, bicarbonate of 19 mmol/L, 2+ urine ketones, anion gap of 16, and pH of 7.4. HbA1c was 15.4% during her admission. The diabetes autoimmune panel was negative (ICA-512, GAD-65, and Insulin Antibodies).\nShe endorsed long-standing polydipsia, polyphagia, and polyuria. She denied weight loss, nausea, vomiting, abdominal pain, confusion, blurry vision, fatigue, fever, and difficulty breathing. Family history was negative for autoimmune diseases except for hypothyroidism in the paternal grandmother and maternal great-grandmother. Physical exam showed a weight of 58.4 kg (70th percentile), height of 163 cm (55th percentile), and BMI of 21.98 kg/m2 (70th percentile). She was a well-appearing adolescent without thyromegaly and acanthosis nigricans and had Tanner Stage 5 breast. She was started on basal bolus insulin regimen consisting of 23 units of Glargine at bedtime and fast-acting insulin Lispro 1 unit per 10 grams of carbohydrates with meals.\nShe received the appropriate diabetes education. Laboratory examination also noted a TSH of 66 IU/ml (reference range: 0.35–5.5 uIU/ml); free T4 was 0.94 ng/dl (reference range: 0.8–1.8 ng/dl), and positive antibodies to thyroid peroxidase was 8580 IU/ml. She was treated with 75 mcg Levothyroxine that was titrated to a dose of 100 mcg. The 6q24 methylation-specific multiplex ligation-dependent probe amplification (MPLA) genetic test for transient neonatal diabetes identified hypomethylation within the 6q24 region. Deletion/duplication analysis ruled out paternal duplication of 6q24. Therefore, the hypomethylation could be the result of either paternal uniparental disomy of chromosome 6 (UPD6) or hypomethylation of the maternal allele. Via testing, UPD6 has been confirmed, meaning both copies of the 6q24 chromosome region in our patient were paternally inherited. This results in an epigenetic phenomena where both copies are still active. Typically, the paternal copy is active and the maternal copy is methylated and turned off. Epigenetic changes are typically de novo, as they depend on the parent of origin and are reset with each pregnancy, rather than DNA sequencing changes. Thus, risk of recurrence for parents, siblings, and offspring is unlikely. After diagnosis and her initial HbA1c of 15.4%, her HbA1c has ranged since then from 6.3% to 6.8%. |
A 33-year-old married woman presented with a history of irregular vaginal bleeding for more than 2 years and a vaginal mass for more than 1 month. She had been diagnosed with endometrial thickening 2 years previously. She had been taking a Chinese herbal medicine (composition unknown) for 1 year. The patient had no family history of cancer. A peanut-sized tumor had been detected at the vaginal orifice 1 month previously, and she reported that the tumor had enlarged obviously 10 days previously. The patient was therefore admitted to our hospital on 17 April 2017.\nGynecologic examination revealed a 4-cm-diameter tumor at the vaginal orifice. The tumor was attached to the vaginal wall with a cauliflower-like growth form, and was hard and susceptible to bleeding. The patient’s uterus was enlarged, corresponding to the size of a 4-month-pregnant uterus. Gynecologic ultrasonography confirmed that the uterus was enlarged, and the anterior wall of the cervix and lower segment of the anterior wall were hypoechoic ().\nPelvic magnetic resonance imaging revealed that the mass involved the uterus, cervix and vaginal cavity, and a small amount of pelvic effusion. Lumps were present in the myometrium, some of which protruded out of the line of the uterus. The lymph nodes beside the iliac vessels appeared enlarged (). Computed tomography examination of the chest, liver, gallbladder, pancreas, spleen and kidney suggested the presence of multiple lymph node metastases in both the lungs and retroperitoneum (). Biopsy of the vaginal mass indicated that it was an invasive poorly differentiated squamous cell carcinoma, negative for SCC antigen and human papillomavirus (HPV). Her carbohydrate antigen 125 level was 80.09 U/mL.\nThe patient underwent radical hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic lymph node dissection, peritoneal sampling and vaginal tumor resection on April 21, 2017. During the operation, about 500 ml of bloody ascites was found in the abdominal cavity. Multiple nodules 1–3 cm in diameter, some ulcerated, were spread over the surface of the uterus. Her bilateral ovaries were enlarged to approximately 5 × 5 ×4 cm and were polycystic. Both Fallopian tubes were normal. The pelvic and aorta abdominalis lymph nodes were markedly enlarged (largest 5 × 3 × 2 cm) and adhered tightly to the blood vessels, with a rough surface. Dissection of the uterus revealed a brittle endometrium and invasion of the entire muscular layer by tumor tissues. Frozen pathological examination revealed invasive carcinoma in the corpus uteri.\nMacroscopically, the uterus measured 10 × 11 × 7 cm, and the cervix was 3.5 cm long with an outer diameter of 2.5 cm. The endometrium was 0.1–1 cm thick, with local roughness covering 7 × 3.5 cm, and infiltrated into the uterine wall. The uterine wall was about 3.5 cm thick, with several nodules 0.5–3.5 cm in diameter in the section of the wall, with slightly harder grey and white matter ().\nHistochemical staining confirmed a poorly differentiated squamous cell carcinoma, with multiple intravascular tumor emboli. The full-thickness of the uterus wall was remarkable for tumor invasion. The tumor infiltrated into the mucosa and stroma of the inner cervical canal and the soft tissue of the right uterus. The cervix uteri showed mild chronic inflammation, with squamous epithelial hyperplasia, and partial glands with squamous metaplasia. Metastasis was found in the lymph nodes in the right and left obturator foramen. However, there was no cancer in the right iliac bone, right peritoneum of the pelvic wall, omental tissue or intraoperative washings (). Immunohistochemical analysis was positive for E-cadherin, Ki-67 (positive region 80%) () and mutant p53 (), weakly positive for CK5/6, P63, CK7 and CK20, and negative for P40, HPV16, HPV18 and HPV31.\nAfter consultation at the Cancer Hospital of the Chinese Academy of Medical Sciences, the patient was diagnosed with stage IV endometrial squamous cell carcinoma. The recommended treatment was paclitaxel (150–175 mg/m2) and cisplatin (60–70 mg/m2) for 2–3 days, combined with pelvic radiotherapy. Ten days after her operation, the patient was transferred to the cancer department for chemoradiotherapy. The patient was sensitive to chemotherapy and developed bone marrow suppression, nausea and vomiting and hair loss, but her liver and kidney function were unaffected. The patient was followed-up regularly and remained in good condition.\nThe patient provided written informed consent for participation in and for publication of this case report. |
A 25-year-old female presented with a complaint of diminution of vision both eyes (BE) for the past 6 months and changes in facial appearance for the past few years. The medical history identified no other family members with similar features and no family history of consanguinity. The patient's parents reported that she was of short in stature since childhood and her hair turned grey at around 20 years of age. She had never visited any clinician before this consultation.\nGeneral physical examination on admission revealed that she was only 129 cm in height and 28 kg in weight []. Her extremities were thin with markedly atrophied skin and decreased subcutaneous fat []. Her face appeared older than her age with noticeable sagging and wrinkling of loose facial skin. Her voice was high-pitched and hoarse, and she also had missing teeth with poor oral hygiene []. Ocular examination revealed small palpebral aperture with sparse eyelashes in both the eyes []. Her distant-corrected visual acuity was the perception of light in BE. The intraocular pressure was within normal limits in BE. The anterior segment showed bilateral cataract in BE. White cataract with multiple calcification spots on anterior capsule of the right eye (RE) and wrinkled anterior capsule of the left eye (LE) was seen consistent with a hypermature [] or morgagnian cataract []. Based on the history and clinical findings, a provisional diagnosis of WS was made.\nBiochemical investigations were normal except marginal increased blood glucose, increased erythrocyte sedimentation rate, decreased follicular stimulating hormone, and mild dimorphic anemia.\nCataract surgeries of BE were performed under general anesthesia at the interval of 15 days. The biometric measurement showed axial lengths of BE to be 21.96 mm (RE) and 22.09 mm (LE), respectively. Sanders-Retzlaff-Kraff II formula was used to calculate intraocular lens power (+22.5 D BE). Temporal clear corneal approach was used in view of the prominent superior orbital rim and small palpebral aperture. Anterior capsulorrhexis was started with needle capsulotome and successfully completed using utrata forceps under a high-viscosity ophthalmic viscosurgical device (OVD). Endothelium was protected using viscodispersive OVD and phacoemulsification (Signature, AMO) was performed under low parameters using the stop, chop, and stuff technique.[] Bimanual cortical aspiration was done under low aspiration and flow rate followed by foldable acrylic intraocular lens implantation in the bag. Corneal tunnel closure was done using 10/0 nylon suture to prevent wound dehiscence. Postoperative regimen consisted of tapering doses of antibiotic-steroid drops over 6 weeks and followed up for 1 year. Endothelial counts (2454 and 2506 cells/mm3 in RE and LE, respectively) and normal fundus examination was found at 6 weeks of the follow-up period. Distant-corrected visual acuity of. 22 RE and. 20 LE in LogMAR with surgical-induced astigmatism, against the rule 0.25D was seen in BE. |
The patient is a 49-year-old, 163 cm-tall man, a professor of family medicine, who was teaching at a university hospital when a dysphagia and night cough started to bother him. Although the patient himself would be expected to advise others to first see a family physician for this kind of symptoms, he decided to use specialized care services at the university hospital he was working for in April 2015. Acquainted with the patient, the professor of gastroenterology ordered an immediate oesophagogastroduodenoscopy, which was reported as ‘hiatal insufficiency and antral gastritis’. The gastric biopsy specimen taken during the endoscopy session returned positive for Helicobacter pylori.\nThe patient was diagnosed as having gastroesophageal reflux disease (GERD) and prescribed a scheme of amoxicillin + clarithromycin + pantoprazole + bismuth subsalicylate. After a few days, he returned to his doctor complaining of medication side effects such as burning in his throat and oesophagus and not being able to swallow the pills. However, being warned of the consequences of non-compliance with the treatment, he kept using the medications as advised. There was no improvement in the initial symptoms at the end of the 14-days treatment. The patient was instructed to continue using pantoprazole 40 mg/day for another three months. However, he discontinued the medication after few weeks due to no apparent benefit.\nThe patient did not seek any other formal health advice for the next three years. He kept taking pantoprazole for a few days when his symptoms flared up. With time, he was used to his symptoms and learned to manage his condition by lifestyle changes such as eating less, chewing longer than usual, and arranging his sleep position. During an informal discussion with a family physician in June 2017, he was advised to see his family doctor, who took a thorough history, which revealed the following:The patient was reporting rare (once every three-to-four weeks) night coughs for more than 20 years. Initially, the cough was awakening from sleep but usually not reoccurring for a long time. His primary symptoms of the current episode of his disease started at the end of 2014, after a stressful event leading to a loss of his job. The principal symptom was dysphagia and a sense of fullness in his throat, which he described as ‘Having a bread loaf in his oesophagus’. He did not report any retrosternal burning or acid reflux. He usually woke every night with persistent cough attacks lasting 30–40 min. The cough increased in cold weather and sleeping in the supine position. Other exacerbating factors were drinking tea or coffee, consuming too hot or too cold food, as well as swallowing large mouthfuls of food. He was reporting undigested food coming back up into his mouth from the oesophagus, even one-to-two hours after a meal. There was a typical gurgling sound while swallowing, especially when drinking liquids. Recently (two-to-three months) he started to experience saliva oozing from his mouth onto the pillow while asleep. He needed to have a napkin ready to wipe his mouth when awoke. The patient reported a weight loss of 16 kg within the last year, dropping from 74 kg to 58 kg.\nHis family physician referred the patient to another gastroenterologist with the differential diagnosis of ZD. This time, the endoscopy report was positive for ZD, describing a significant diverticular opening at the left posterior side of the oesophagus, at around 18 cm from the teeth, which initiated a further referral to the department of thoracic surgery. Contrast oesophagoscopy () revealed a large ZD.\nThe patient was admitted to the department of thoracic surgery where he underwent a diverticulectomy with stapling and additional upper oesophageal myotomy. The surgical intervention was done with a left-lateral open surgical approach, after assessing operation conditions with intraoperative oesophagoscopy.\nThe patient was hospitalized for seven days, with daily follow-up for leakage/infection at the incision site and for integrity of the repair. Oral nutrition was initiated on day five of operation. A control visit was scheduled for one month after discharge. At the control visit, he was free of all symptoms and weighed 60 kg. |
A 12 hour old male neonate presented to the department with complaint of two episodes of bilious vomiting in last two hours. The patient was a full term normal vaginal delivery, delivered in the hospital. The family history was non contributing.\nOn examination, the general condition of the patient was good. His weight was 2.75 kg. There was no obvious congenital anomaly. On examination of the abdomen a cystic structure was felt in the right lumbar segment. X-Ray of the abdomen revealed three gas shadows in the left hypochondrium with no other gas shadow. A diagnosis of proximal small bowel atresia was made. No other investigation was done.\nThe patient was managed by nil by mouth and nasogastric tube placement. He was given intravenous (IV) fluid. The antibiotics given were ceftriaxone (75 mg/kg IV 12 hourly), amikacin(7 mg/kg IV 12 hourly) and metronidazole (7.5 mg/kg IV 12 hourly).\nThe patient was operated after about 24 hours of presentation. Under general anesthesia, the right transverse supraumbilical incision was made. On exploration of the peritoneal cavity there was presence of a mesenteric cyst in the jejunal mesentery that was felt as the cystic structure on abdominal examination (figure ). The jejunum associated with the mesenteric cyst was having a single type I atresia. The presence of atresia was 15 cm from the dudenojejunal flexure. The proximal bowel was dilated. The treatment included resection of the cyst along with the atretic bowel and proximal 7.5 cm of the dilated bowel and end to end single layer anastomosis by vicryl 5-0.\nThe post operative period was uneventful. The patient was allowed orally on 11th post operative day. IV antibiotics were continued for 11 days followed by oral cefixime (4 mg/kg BD) for seven days. He was discharged on 14th post operative day in satisfactory condition. The follow up of the patient is also satisfactory. |
A 57-year-old Ukranian male with a 45-year history of smoking but no other medical history presented in August 2008 complaining of months of intermittent abdominal pain, diarrhea, and a 40 lb weight loss. His symptoms presented over the course of 1 year. His diarrhea was initially evaluated and attributed to gastroenteritis. Routine studies were sent, including a stool culture. S. arizonae (subtype III), which is associated with osteomyelitis and gastroenteritis in reptile handlers, was isolated and the patient reported contact with a pet iguana. Computed tomography of the abdomen and pelvis was ordered and revealed a 1.5 × 1.7 cm speculated pulmonary nodule in the right upper lobe and thickening of the duodenal, jejunal, and colonic wall with scattered surrounding lymphadenopathy.\nThe patient was placed on antibiotics and continued to suffer from continued diarrhea and weight loss, so inflammatory bowel disease was suspected. In September 2008 he underwent endoscopic studies which revealed a hiatal hernia, old blood in the stomach, mucosal swelling near the gastric antrum, and three areas of circumferential ulceration and inflammation with mucosal pigmentation/erythema (see ). The areas were biopsied, and the biopsy showed reported duodenitis and jejunitis with acute on chronic inflammation. There were no signs of architectural distortion or cryptitis/crypt abscesses, Helicobacter pylori stains were negative, and no bacteria were apparent on biopsy. Given that the biopsies did not show any findings consistent with the suspected diagnosis of inflammatory bowel disease, his symptoms were attributed to S. arizonae gastroenteritis.\nFine needle aspiration of his pulmonary nodule was nondiagnostic, showing only clustered atypical epithelial cells. A positron emission tomography scan was obtained and showed a focus of hypermetabolic activity in the right upper lobe which was read as probable granulomatous disease.\nHe continued to have recurrent episodes of abdominal pain and vomiting in November 2008, and treatment of the presumptive S. arizonae gastritis/enteritis with trimethoprim-sulfamethoxazole and ciprofloxacin failed to improve his symptoms. S. arizonae was not grown from stool samples that were resent.\nOne month later, the patient presented with back pain and decreased urine output. Acute kidney injury was noted, with a serum creatinine of 4.37 mg/dL, while urine studies revealed severe proteinuria and hematuria. Initially the acute kidney injury was thought to be secondary to prerenal azotemia from dehydration or acute interstitial nephritis secondary to treatment with ciprofloxacin and trimethoprim-sulfamethoxazole. A c-ANCA level was drawn for workup of his renal failure and came back at >100 U/L. He then underwent repeat endoscopy which showed normal gastric mucosa, but granular and unusually pigmented duodenal mucosa (). Biopsies of multiple sites were obtained, which later came back positive only in the ascending colon, which showed a tubular adenoma. Slides from a macroscopic jejunal ulcer seen on endoscopy () showed inflammation but no features typical of inflammatory bowel disease. Given the unexplained nature of his systemic symptoms, acute kidney injury, and the positive c-ANCA, a kidney biopsy was performed and showed rapidly progressive glomerulonephritis due to pauci-immune crescentic glomerulonephritis with 25% crescents ().\nThis renal biopsy result was clearly consistent with an ANCA-associated lesion, and the patient received three doses of cyclophosphamide between February and April 2009. Given the finding of a pulmonary nodule, video-assisted thoracoscopic surgery was performed in June 2009. The pathology results later came back positive for adenocarcinoma with clear margins. The lesion was surgically excised and a subsequent positron emission tomography scan was found to be negative for metastasis. He resumed cyclophosphamide therapy in December of 2009 and has had significant improvement of his gastrointestinal symptoms, renal impairment, and systemic symptoms on steroid and immunosuppressive therapy. |
A 25-year-old Saudi female, who is medically free, presented to our emergency department complaining of a mass in the mons pubis. The mass was occasionally tender and had rapidly increased in size in two-week duration. No other complains or masses at other sites were reported. On clinical examination, an ill-defined soft mass was identified in the mons pubis, extending to the left inguinal area with a hyperemic overlying skin. Laboratory tests were within normal limits except for a mild leukocytosis. The clinical impression was of an abscess, and the patient was scheduled for surgery. A local excision of the mass was done, and the specimen was sent to the histopathology department. The patient was discharged on the next day of surgery.\nGrossly, the specimen consisted of two irregular pieces of soft tissue. Both masses were partially encapsulated and measured 6 × 5 × 2 cm and 6.5 × 6 × 2.5 cm, respectively. The outer surface of both masses was irregular. Serial slicing of the masses revealed a heterogeneous cut surface with pale white and dark tan areas. Random sections from both masses were taken for histopathological examination.\nH&E slides showed a poorly differentiated neoplasm. The tumor consisted exclusively of cells retaining the classical “rhabdoid” morphology (). The cells were discohesive with distinct cell borders, and they were arranged in solid sheets. The amount of cytoplasm varied between scant to abundant. The cytoplasm had a prominent eosinophilic quality, and intracytoplasmic glassy eosinophilic inclusions were seen within the majority of the cells. The nuclei were vesicular, eccentric, and highly pleomorphic with prominent nucleoli. Numerous mitoses were seen (45 mitoses/10 HPF). Occasional multinucleated tumor giant cells were present. There were neither areas of necrosis nor lymphovascular invasion.\nAn extended panel of antibodies was performed on Ventana using the iVIEW DAB detection kit and showed focal cytoplasmic immunoreactivity to vimentin (mouse monoclonal, 1 : 60; Dako) with the characteristic globular cytoplasmic configuration indenting the nucleus. The cytoplasm of tumor cells also coexpressed cytokeratin cocktail (mouse monoclonal, AE1/AE3, 1 : 100; Dako), CK 8 (mouse monoclonal, ready to use; Dako), and CK 19 (mouse monoclonal, 1 : 40; Dako). The tumor cells, in comparison to the endothelial lining of the capillaries, showed loss of INI1 (mouse monoclonal, BAF 47, ready to use; BD Transduction Laboratories, USA) nuclear staining (). Anti-Ki-67 (mouse monoclonal, 1 : 50; Dako) showed a high proliferation index reaching up to 80% of tumor cells. Tumor cells have showed nuclear positivity to ER (mouse monoclonal, 1 : 20; Dako) (weak to moderate in 90%) () and have also showed nuclear positivity to PR (mouse monoclonal, 1 : 100; Dako) (strong in 20%). All other immunohistochemical (IHC) stains were negative including CD34, EMA, CD99, CD117, CD45, CD30, CD138, bcl-6, bcl-2, CK7, CK20, GCDFP, muscular differentiation markers (Desmin, SMA, Myoglobin), neuroendocrine markers (Synaptophysin, Chromogranin A, CD56), and melanoma markers (S100, HMB-45).\nA diagnosis of malignant rhabdoid tumor of the vulva was established. The patient was recalled once the histopathology report was signed out. A computed tomography scan (CT) of the head, chest, abdomen, and pelvis was performed and revealed no other tumor masses. Unfortunately, the patient failed to show for followup. |
A 57-year-old South Asian man presented with a painless, nodular lesion (1 cm × 0.5 cm) on his left knee. He had had a renal transplant eight months earlier for autosomal dominant polycystic kidney-related renal failure. He received two doses (20 mg) of interleukin receptor-2 antibodies (Simulect®, Novartis Pharmaceuticals, Surrey, UK) at induction and on the fourth postoperative day. This was followed by tacrolimus, mycophenolate mofetil and prednisolone for immunosuppression. During the follow-up period, his tacrolimus level was kept within the therapeutic range (5 to 8 ng/ml). He was cytomegalovirus (CMV) negative and had received a kidney from a CMV-negative deceased donor. He had immigrated to the United Kingdom from Bangladesh twenty-five years ago and had last visited there eight years ago. There was no past history of trauma to the knee.\nHe was started on flucloxacillin and the course was extended for a period of 14 days. In spite of antibiotic therapy, the lesion progressed significantly and reached 5 cm × 5 cm (Figure ) and became cystic in nature. The lesion was confined to the skin and subcutaneous tissue with no deep extension to bone or lymph node involvement. The surface of the lesion had multiple small sinuses showing a dark-brown discharge, which was sent for microscopy.\nDuring this period, he did not show any systemic symptoms and inflammatory markers (C-reactive protein) and his white cell count were both normal. Because of the failure of antibiotic therapy and the fast growing nature of the lesion an incisional biopsy was performed.\nHistological examination of the specimen showed marked epidermal hyperplasia and abundant fungal spores with hyphae. The microscopy of the discharge also confirmed the fungal spores and hyphae (fungal grains).\nThe patient was started provisionally on voriconazole while awaiting confirmation of the causative organism. The large size of the lesion on his knee significantly reduced his quality of life so an excision biopsy was done. The specimen showed marked epidermal hyperplasia with microabscess formation. Within the microabscess, there were PAS (Periodic acid-Schiff) positive branching and septate hyphae (Figure ). No bacteria were found. The excision margins were clear, and no evidence of the neoplastic process was found.\nNuclear ribosomal repeat-region sequencing confirmed that the causative organism was P. romeroi (Mycology Reference Laboratory, Bristol, UK). An in vitro antifungal susceptibility test demonstrated that P. romeroi was sensitive to voriconazole. Following a successful surgical removal, voriconazole was continued orally for two months under the care of the infectious-disease team. No recurrence was seen during the following six months. |
A 50-year-old man of Indian ancestry who was diagnosed with multiple myeloma three years earlier was evaluated in our hospital. His only other chronic medical issue was mild hypertension. His myeloma had progressed rapidly since diagnosis despite a variety of therapies over the years including systemic corticosteroids, cyclophosphamide, etoposide, cisplatin, stem cell transplantation, thalidomide, and for the most recent three months, bortezomib. Blood work and magnetic resonance imaging at a recent out-patient visit demonstrated pancytopenia as well as diffuse myelomatous bone marrow replacement throughout his pelvis and proximal femora (Figure ). At this time, he was being hospitalized due to extensive fluid retention in the abdomen and lower extremities as well as dyspnea. He stated that he had gained 15 pounds over the past two weeks. On initial examination, he was afebrile with a heart rate of 100 beats/minute and a blood pressure of 97/50 mmHg. His oxygen saturation was 96% while receiving oxygen at 3 liters/minute by nasal cannula. He had crackles at the bases of his lungs bilaterally. His cardiovascular exam was remarkable for 12 cm of jugular venous distension and tachycardia with a 2/6 systolic flow murmur at the left upper sternal border. His abdomen was distended with shifting dullness to percussion and a liver edge 4 cm below the right costal margin. His extremities were warm to touch with 3 + bilateral lower extremity edema as well as significant scrotal edema. Pertinent initial laboratory studies were remarkable for a hemoglobin of 9.1 g/dl, a platelet count of 10,000 per microliter, a blood urea nitrogen of 55 mg/dl, a creatinine of 1.0 mg/dl, an albumin of 3.6 g/dl, and a calcium of 13 mg/dl. The ECG demonstrated sinus tachycardia with normal voltage and diffuse T wave flattening. His chest X-ray demonstrated mild cardiomegaly and evidence of pulmonary edema. An echocardiogram conveyed a hyperdynamic left ventricle with normal wall thickness, no regional wall motion abnormalities, no valvular abnormalities and normal diastolic function. Thrice daily intravenous furosemide was administered for the first ten hospital days. Despite aggressive diuretic therapy, the patient's volume status worsened. On the eleventh hospital day, cardiac catheterization was performed (Table ). Based on the high output values obtained at catheterization, a thyroid panel was obtained which was unremarkable. In addition, he was given empiric thiamine replacement, placed on broad-spectrum antibiotics for possible sepsis, and was started on a continuous intravenous infusion of furosemide. His respiratory status continued to worsen and on hospital day number 14, he required intubation and mechanical ventilation for hypoxemic respiratory failure (Figure ). His volume status continued to worsen over the next 2 days despite the aforementioned therapy. As a last resort, it was decided to initiate therapy targeting the underlying myeloma on hospital day 17. Lenalidomide 25 mg and dexamethasone 40 mg daily were administered through the patient's nasogastric tube. Within 24 hours, a brisk diuresis was observed and he was successfully extubated on hospital day 19. Dexamethasone was discontinued per protocol after hospital day 20, though lenalidomide was continued. By hospital day 27, he had a net negative fluid balance of 15 liters and he was discharged out of the intensive care unit. Unfortunately, on hospital day 35 in the setting of his long standing refractory thrombocytopenia, he developed a massive upper gastrointestinal bleed that could not be controlled despite aggressive resuscitory efforts and died within hours. |
An 8-month-old girl weighing 6 kg was referred to our hospital for surgery. Her initial diagnoses were right atrial isomerism, dextrocardia, unbalanced complete atrioventricular septal defect, double outlet right ventricle, severe pulmonary stenosis, and supracardiac non-obstructive TAPVC. She underwent Glenn anastomosis with TAPVC repair when she was 9 months old. Two months after the surgery, she was referred to our clinic because of cyanosis, respiratory distress, hypoxia, and severe upper extremity and palpebral edema. On admission, she was gasping with bradycardia and severe metabolic acidosis. She was immediately admitted to the pediatric cardiac intensive care; endotracheal intubation and inotropic support were started. Her oxygen saturation level was in the low 70s with 100% oxygen supplement. Her echocardiography revealed pulmonary venous obstruction, Glenn dysfunction, and pulmonary hypertension. Anti-pulmonary hypertensive treatment was added to her treatment. An emergent computed tomography angiography () showed severe bilateral pulmonary venous stenosis at the junction of the collector sac and pulmonary vein.\nUrgent cardiac catheterization for stenting the pulmonary veins was planned. Initially, pressure gradients were gathered (). Selective right and left pulmonary angiography and direct injection of contrast to the proximal segment of the pulmonary veins showed a narrowing at the junction of the collector sac and pulmonary veins. The narrowest parts were 4 mm and its proximal side was 7 mm on the right pulmonary vein and measurements were 1.5 mm and 4.2 mm, on the left pulmonary vein, respectively (, ).\nInitially, a 7 x 12-mm Palmaz Blue balloon-expandable peripheral stent (Cordis Endovascular, Warren, NJ) was placed across the stenosis on the right pulmonary vein and was dilated until the waist completely disappeared (, , ). However, stenting the left pulmonary vein was more complex because it was more stenotic and the left upper and lower pulmonary veins combined together before narrowing. Placing a stent in the lower vein will jail the upper vein or vice versa. After consulting with the surgeons, a 4 x 8-mm Liberte bare coronary stent (Boston Scientific, Natick, MA) was placed across the stenosis. After stent implantation, pressure gradients across the stents dropped to normal levels (). The patient’s oxygen saturation level was elevated to the low 90s. Acetylsalicylic acid, clopidogrel, and standard heparin were initiated after the procedure. She was extubated 3 days after the procedure and was discharged 12 days later. Four months after the procedure, a second catheterization was performed to dilate both stents. The patient is still asymptomatic, and her echocardiographic examination revealed mild stenosis during her 9-month follow-up. |
A 56-year-old male without any previous medical history presented to our emergency room (ER) with multiple traumas from a 10 meter fall in a construction field. Physical examination revealed a male patient with a body mass index in the normal range and an acutely ill looking appearance. His right lower leg and ankle were swollen and bruised, and he had a 2 cm laceration wound on the plantar aspect of his right foot. The patient's right ankle had limited range of motion due to pain. The patient had tenderness at the right anterolateral aspect of the mid lower leg and anterior aspect of the ankle. There was grade 1 anterolateral instability of the left ankle. The neurologic examination was normal. Based on the patient's clinical history and physical examination, the orthopedic surgeon suspected a fracture of the right fibular diaphysis and ligament injury of the right anterolateral ankle.\nInitial radiographs of the ankle in the anteroposterior and lateral views showed fractures at the diaphysis at the fibula and anterior lip of the tibial plafond (Fig. ). The patient was not able to undergo ankle Mortise view because of his limited range of motion due to extreme pain. In a subsequent lower extremity computed tomography (CT), the orthopedic surgeon in the ER noticed a segmental fracture of the right fibular shaft and the anterior lip of the tibial plafond.\nTo evaluate the ankle ligaments, a turbo spin-echo (TSE) two-point mDixon technique applied to an ankle MRI (Table ) was performed after procuring written informed consent. In addition to the fractures of the right fibular shaft and tibial plafond, this MRI demonstrated a tiny chip fracture of the lateral talar dome. A tiny wafer-shaped talar dome chip fracture fragment about 7 (anterior–posterior diameter) × 3 (head to toe diameter) mm was clearly delineated only in the sagittal T2-weighted mDixon opposed-phase MRI (Fig. B). In T2-weighted mDixon in-phase imaging, which is considered a conventional T2-weighted image, there was a definite focal wedge-shaped cartilage defect at the corresponding area. However, there was only focal and subtle cortical irregularity and the cortical step-off was not definite (Fig. C). In a T2-weighted mDixon water-only image, which is considered a conventional fat-suppressed T2-weighted imaging, the cartilage lesion and focal cortical irregularity were once again noted, and the subcortical bone marrow edema was additionally confirmed. In these 2 sequences, a fracture was suspected, but the radiologists could not fully delineate the fracture line (Fig. D). In T2-weighted mDixon fat-only imaging, there were dark signal alterations at the subcortical region, but these were not considered fractures (Fig. E). T1-weighted imaging was obtained in the axial plane, and the fracture line was not depicted in this plane (Fig. F). In a CT image reviewed by an experienced musculoskeletal radiologist, there was a lateral talar shoulder cortical fracture at the identical area where the chip fracture was noted (Fig. A) from the T2-weighted mDixon opposed-phase image. In addition, there was a grade 2 injury to the anterior talofibular ligament with severe subcutaneous swelling of the ankle.\nDuring ankle arthroscopy, there was a free floating osteochondral fragment about 4 x 8 mm at the posterolateral talar shoulder, which was removed with basket forceps (Fig. ), and microfractures were performed at the posterolateral talar cortical fracture site.\nThe patient did well after the arthroscopy with recovery of full range of motion after 2 months. |
A 28-week-old premature boy, with a birthweight of 1280 grams, was intubated with a 2.5 mm endotracheal tube via the nose and ventilated for severe hyaline membrane disease (HMD). After receiving two doses of surfactant, the premature neonate was successfully weaned off ventilation and extubated to nasal continuous positive airway pressure (CPAP). On day 9, his clinical course was complicated by a pulmonary hemorrhage, requiring re-intubation. He was given another dose of surfactant and stabilized on high-frequency oscillation ventilation (HFOV). The chest radiograph showed extensive bilateral pulmonary interstitial emphysema, with the left side more extensively involved in comparison to the right. A hemodynamically significant patent ductus arteriosus was treated by intravenous paracetamol. The baby's condition did not improve, and he was selectively intubated into his right main bronchus. The position of the endotracheal tube was radiologically confirmed, allowing the right lung to be oscillated while the left lung was rested. The baby was nursed on his left side for a period of 36 hours, after which the endotracheal tube was retracted into the trachea and secured in that position. The baby's ventilatory status subsequently improved, allowing for extubation on day 7 after the relapse. On day 28 of life, he presented with severe stridor, requiring re-intubation. Difficulty during intubation suggested that subglottic stenosis might be present. One week after this re-intubation a flexible bronchoscopy was performed, which revealed two major findings. The first was a Cotton grade 2 subglottic stenosis, and the other abnormality was near-complete obstruction of the bronchus intermedius. The subglottic stenosis was dilated to 5 mm with the aid of a balloon dilator (Boston scientific Mustang™ balloon dilatation catheter). The bronchus intermedius was extremely narrow, and a 2.2 mm flexible bronchoscope was not able to pass through the area of stenosis.\nAfter 2 weeks, the bronchoscopy was repeated and the subglottic region had improved to near normal in diameter. The bronchus intermedius stenosis, however, remained unchanged. A chest Computed Tomography (CT) scan was performed to determine the length of the bronchial stenosis. The CT scan confirmed that the stenosis involved a short segment and had a web like in configuration (Figure ).\nIt was decided to balloon dilate the stenosis under fluoroscopy. As the cardiac catheterization suite offered the best quality fluoroscopy, it was decided to perform the dilatation in the suite. The baby was intubated, and a guidewire was inserted into the right main bronchus under fluoroscopy. Water-soluble contrast was injected, and the position of the airway identified. A 3.5 mm coronary artery balloon catheter was inserted into the area of stenosis via the guide wire, and the position was confirmed by fluoroscopy. The balloon was inflated at 16 atmospheric pressure for 20 seconds. This was repeated for another 20 seconds before water-soluble contrast was reinjected, demonstrating significant decrease in the bronchial stenosis (Figure ). Following the procedure, the baby was ventilated for less than 24 hours. At follow-up bronchoscopy 2 weeks later, the stenosis had significantly improved, allowing a 2.8 mm flexible bronchoscope to pass comfortably through the stenotic region. The posterior part of the stenosis had completely resolved, with a small anterior shelf remaining. The baby was discharged with no known respiratory complications, and at follow-up bronchoscopy 6 weeks, after the latter dilatation procedure, the airway remained patent and the baby remained asymptomatic, with a normal chest radiograph. Follow -up bronchoscopy was done due to the risk of restenosis and the fact that the baby was from a rural area, with limited medical services. |
A previously healthy 35-year-old African American male presented with a one-month history of worsening lower back and bilateral lower extremity pain, intermittent night sweats, and 32 kg unintentional weight loss over the course of a year. He did not have saddle anesthesia or urinary or fecal incontinence. He was initially seen in a primary care clinic and was diagnosed with sciatica. As symptoms continued to worsen, he underwent a computed tomography (CT) scan of the lumbar scan as an outpatient that was concerning osseous spinal metastasis. He was started on prednisone 10 mg daily and was referred to the oncology clinic at our center. Prednisone gave him minimal symptomatic relief. While waiting to be seen in the oncology clinic, the patient had an episode of leg weakness with near-fall prompting him to present to the emergency department of our hospital and was admitted for further evaluation. His vital signs were stable. He had no palpable cervical, supraclavicular, axillary, or inguinal lymph nodes. Neurological exam was normal with intact strength and sensation in both lower extremities.\nHis complete blood count and serum electrolytes were normal including a normal serum calcium level at 8.1 mg/dL. He tested negative for human immunodeficiency virus 1 and 2 antibodies. Magnetic resonance imaging (MRI) of the cervical, thoracic, and lumbar spine showed several enhancing lesions in T11, T12, L3, L4 vertebral bodies, right sacrum, and ilium that were concerning metastatic disease. There was effacement of the right lateral recess and right neural foramen at the L3-L4 and effacement of the left lateral recess and left neural foramen at the L4-L5 due to tumor retropulsion (Figures –). In addition, a small epidural tumor was noted at the T5 vertebral level without significant spinal canal stenosis or cord compression. Imaging was also concerning osseous metastasis involving the sternum and multiple ribs. Incidentally, narrowing of the neural foramen at left T2-T3 and right C7-T1 and T5-T6 levels was also noted. Since the findings were concerning diffuse metastatic disease, a CT scan of the chest, abdomen, and pelvis were performed and showed bilateral hilar and mediastinal adenopathy, mild cardiomegaly, and dilated main pulmonary artery measuring 3.6 cm (Figures and ). Enlarged liver measuring 18.1 cm, enlarged spleen measuring 12.4 cm, and multiple bilateral enlarged pelvic sidewall, external iliac, and inguinal lymph nodes concerning lymphoma or metastatic disease are shown in . Ultrasound of the scrotum did not reveal any testicular masses.\nHe underwent extensive screening for hematologic and solid tumor malignancies including serum protein electrophoresis, urine immunofixation, beta-human chorionic gonadotrophin hormone levels, and fecal occult blood test that were all negative. He subsequently underwent a CT-guided core needle biopsy of the left iliac crest lesion that was significant for noncaseating and necrotizing granulomas. Histochemical stains for Grocott's methenamine silver (GMS) and Ziehl-Neelsen stains were negative for fungal elements and acid-fast bacilli, respectively. Due to high suspicion of malignancy, he also underwent an endoscopic bronchial ultrasound with transbronchial needle aspiration of the inferior mediastinal lymph node which found non-necrotizing granulomas but did not reveal any malignant cells (Figures –). Fungal culture and acid-fast bacilli culture from the transbronchial aspirate were again negative. Serum ACE level was 62 U/L (normal 14–82 U/L).\nNeurosurgery was consulted, and they did not recommend any acute neurosurgical intervention. The patient was discharged with follow-up in pulmonology clinic. Since there was concern that his steroid therapy prior to admission could have masked lymphoma, he had a left inguinal node excisional biopsy, a month later, that showed necrotizing and non-necrotizing granulomatous lymphadenopathy and was negative for acid-fast or fungal microorganisms. Since there was concern for a process with high metabolic activity, he also had an 18F-labeled fluorodeoxyglucose (18F-FDG) positron electron topography (PET) scan that was significant for extensive hypermetabolic osseous and nodal disease (). |
A sixty-six-year-old female visited the hospital with chief complaint of swelling in the lower left jaw region since 1 month. Swelling was insidious in onset and gradually progressed to the present size. The patient also gave history of reduced mouth opening since last 5 days. Past dental history revealed extraction of lower left back teeth in the same region 6 months ago with uneventful wound healing of the surgically explored site a month later.\nOn extra oral examination, facial asymmetry with diffuse irregular swelling was evident on the lower left side of the jaw measuring about 3 cm in size. On palpation swelling was warm, nontender, and firm in consistency. Restricted temporomandibular movements and paresthesia were evident (till the lower left chin area). Submandibular lymph nodes were hard and fixed to the underlying tissues.\nOn intraoral examination, there was restricted mouth opening with a maximum interincisal distance between 11 and 41 measuring about 1 cm only. Mild tenderness with buccal cortical plate expansion in relation to 36,37 was seen. Provisional diagnosis of carcinoma of mandible and differential diagnosis of space infection, chronic osteomyelitis, and metastatic lesion were considered ().\nFine needle aspiration cytology (FNAC) revealed scanty fluid exhibiting sheets of cells with atypical features of hyperchromatic nucleus, pleomorphism, and altered nuclear cytoplasmic ratio suggestive of malignancy. Orthopantomogram (OPG) () view showed an extensive multilocular radiolucency in the left mandibular body extending from 33,34 region posteriorly to the ramus of mandible suggestive of malignancy. Chest radiograph appeared normal. Incisional biopsy revealed proliferative stratified squamous epithelium with dysplastic squamous islands exhibiting features of hyperchromatism, pleomorphism, and individual cell keratinization. The tumor cells showed no contact with the normal appearing overlying mucosa (Figures and ).\nBased on all findings, a final diagnosis of Primary Intraosseous Carcinoma of the mandible was given. Patient was treated by routine surgical removal of the lesion followed by postoperative radiotherapy (routine radiotherapy; 60 Grays) to prevent the likelihood of metastasis and poor outcome. Patient is under periodic follow-up till date without any evidence of malignancy. |
A 45-years-old woman brought in by EMS after she was found in distress on a subway platform. The patient with known history of diabetes type II and hypertension presented with stabbing chest pain radiating to the interscapular region after crack cocaine use. In addition to the chest pain and diaphoresis, the patient also complained of progressive pain and cold sensation over left lower extremity. Upon physical examination, the patient was noted to be agitated and combative with a blood pressure (BP) of 205/147 mmHg, a heart rate of 77 beats per minute, 17 respirations per minute, and was afebrile. Initial cardiovascular exam was positive for an S4 gallop and absent left lower extremity pulses. Lung examination was unremarkable. Initial management consisted of intravenous administration of a total of 9 mg of Lorazepam and 35 mg of Labetalol without acceptable reduction in BP. In addition to further Lorazepam, Labetolol later Esmololol infusions, IV Nitroglycerin was started and quickly titrated to 200 mcg/kg/hr but acceptable blood pressure reduction of blood pressure was still not achieved on this regimen (). A contrast-enhanced Computerized Tomography (CECT) of the thorax, abdomen, and pelvis confirmed a Stanford Type B aortic dissection beginning just beyond the left subclavian artery and extending to the iliac bifurcation. Upon arrival to the Cardiac Care Unit, decision was made to begin an infusion of Dexmedetomidine since all other therapies continued to be unsuccessful. The alpha 2 stimulation provided by the Dexmedetomidine was hypothesized to decrease the effects of the adrenergic surge induced by the cocaine thus mitigating alpha 1 and beta-receptor stimulation. Within 10 minutes of Dexmedetomidine bolus and continuous infusion, adjunct antihypertensive and sedative infusions were quickly weaned off. Evolving signs of ischemia in the left lower extremity warranted emergent femoral-femoral bypass. Vascular surgery team performed procedure successfully and uneventfully. Postoperatively BP control was maintained with Dexmedetomidine and Nitroglycerin then Nicardipine uneventfully. The total duration of Dexmedetomidine therapy was 42 hours. The patient was transitioned to oral antihypertensives simultaneously. The clinical course evolved without any further complications, and the patient was discharged after 14 days. |
A 15-year-old female patient reported with a complaint of swelling on the left side of the lower face for 3 years. She noticed progressive increase in size of the swelling and reached the present size. She also noticed increasing asymmetry of the face. No pain was associated with it. Family and medical history were noncontributory.\nOn extraoral examination, facial asymmetry was observed owing to an hyperpigmented swelling on the left side of the mandible. Clinical examination revealed soft, nontender, hyperpigmented swelling lateral to left chin region 2 cm below the corner of mouth and 1 cm above the lower body of mandible []. On inspection, no visible pulsations were noticed. On palpation, the swelling was nontender and soft in consistency and compressible. Pulsation and bruit were noticed just below the left corner of mouth and submandibular region. There was no local rise in temperature.\nNo visible swelling was noted intraorally. There was mobility of 34, 35, 36, and 37 and 35 and 36 depressed on applying pressure. Pulsation and bruit in relation to buccal vestibule of 35 and 36 were noticed [].\nA provisional diagnosis of vascular malformation was given based on palpable pulsation, bruit, and history.\nOcclusal radiograph showed mild expansion of buccal cortical plate in relation to 36, 37, and 38 regions []. Panoramic radiograph revealed ill-defined radiolucent lesion with poorly defined margins confined to the left body of mandible extending from distal aspect of 32 till radicular aspect of developing tooth germ 38. Root resorption of 34, 35, 36, and 37 with loss of lamina dura noticed. Altered trabecular pattern, thinning of inferior cortical border of right mandible, and dilated inferior alveolar canal on left side, which cannot be traced anteriorly was also noticed [].\nUltrasonography of face and neck was done using 12-MHz transducer which revealed the increased vascularity in the course of left facial artery anterior to left masseter in color flow Doppler imaging []. Increased vascularity also was noticed over the vessels of left submandibular gland [].\nContrast-enhanced CT of mandible revealed large expansile lesion on left body of the mandible with brilliant enhancement after intravenous contrast and minimal rarefaction of lingual and labial cortex with very rich vascularity. Prominent serpiginous nidus of vessels seen at left half of mandible and adjacent subcutaneous compartment supplied through branches of external carotid arteries such as lingual, facial, and superficial temporal arteries representing AVM of the left mandible [].\nUnder local anesthesia through left femoral arterial approach, left external carotid angiogram was done, which revealed evidence of AVM in the left side mandible with feeding arteries from branches of left external carotid arteries such as lingual, facial, and superficial temporal arteries which were dilated and tortuous []. This confirmed the diagnosis of AVM on the left side of mandible.\nUnder general anesthesia by nasoendotracheal intubation, after embolizing the feeder vessels, the mandible was resected en bloc with the pathologic contiguous soft tissue from the area of the left ramus to the mesial aspect of the left lateral incisor. After resection and removal of the involved mandibular segment and control of hemorrhage, teeth 32, 33, 34, 35, 36, 37, and 38 were extracted from the segment and the convoluted vascular mass was curetted. The remaining cortical shell was then grafted with a corticocancellous block of bone from the right fibula. The patient is under regular follow-up till now. |
A 59-year-old African-American man, with history of renal insufficiency, diabetes, hypertension, severe peripheral vascular occlusive disease, and a 100 pack-year tobacco history, was incidentally found to have an abnormal chest radiograph showing bilateral large lower lobe airspace opacities. Images taken three years previously were reviewed; the abnormalities were present at that time and had not changed in size significantly. The patient was lost to follow-up after that initial abnormal radiograph. He was experiencing only mild respiratory complaints of dyspnea on exertion and occasional dry cough. There were initially no symptoms of hemoptysis, fever, chills, weight loss or night sweats. No laboratory abnormalities were discovered. A history of diagnosed autoimmune disease, cancer or family history of lung disease was not present. A computed tomography (CT) scan of his chest confirmed dense consolidation with air bronchograms involving several segments of his bilateral lower lobes. No mediastinal lymphadenopathy was seen (see Figure ). Our patient underwent bronchoscopy with a transbronchial biopsy. The pathology of the biopsy specimen showed extranodal marginal zone B-cell lymphoma of BALT (see Figure and ). Further staging with positron emission tomography-CT and bone marrow biopsy revealed stage IV disease involving the subcarinal lymph nodes as well as the bone marrow.\nOur patient was treated with single-agent rituximab and received four total doses, one dose given weekly. His clinical course remained stable with no evidence of progression and some improvement on CT imaging post-treatment. Our patient was seen every three months by his oncologist after his initial diagnosis. Two years after the diagnosis and treatment with rituximab our patient's clinical course worsened. He described new symptoms of worsening shortness of breath, weight loss, night sweats and fevers. He had elevated lactate dehydrogenase at 350IU/L, uric acid measuring 10.0 mg/dL and creatinine of 1.7 mg/dL. A repeat CT scan showed new air-filled areas within the right lower lobe mass, consistent with cavitations representing probable necrosis (see Figure ). A comprehensive work-up for infection was negative, which included bacterial and fungal testing, studies for acid-fast bacilli and human immunodeficiency virus testing.\nClinical concern for transformation of this low-grade lymphoma to a more aggressive form was also considered. Our patient underwent a repeat transbronchial biopsy which suggested transformation to a diffuse large B-cell lymphoma secondary to findings of an increased large cell population (see Figure ). Due to these new findings our patient was started on the chemotherapy regimen of rituximab-CHOP. He completed two cycles and five days later was admitted to the hospital for hyperglycemia. His hospital course became complicated by the development of a new loculated pneumothorax involving his right lung, thought to be secondary to the necrotic lung process. He required chest tube placement and was placed on mechanical ventilation. His condition worsened with development of severe sepsis secondary to Enterococcus faecium in sputum, pleural fluid and blood.\nDue to multiorgan system failure and poor overall prognosis the family changed the goal of care to palliative and our patient died shortly thereafter. |
A 28-year-old female patient who was referred from Department of Conservative Dentistry and Endodontics to Department of Periodontics, Subharti Dental College, Meerut to increase the length of the clinical crown in relation to maxillary right central incisor, maxillary right lateral incisor, maxillary left central incisor, maxillary left lateral incisor and maxillary left canine for placement of fixed prosthesis. A detailed medical and dental history was obtained from the patient. A thorough clinical examination was performed. It revealed severe loss of the tooth structure due to caries []. The teeth were endodontically restored. Radiographic examination showed Gutta Percha point in the apical one-third for the placement of post and core []. On probing, a generalized depth of 2 mm was observed [] with 3-4 mm of attached gingiva. The teeth were not mobile. Maxillary and mandibular impressions were taken and diagnostic casts were obtained along with pre-operative intraoral photographs.\nAn average of 2 mm of the clinical crown and 2 mm of biologic width was required to place the subgingival margins of the crown for which surgical crown lengthening was planned. According to classification by Lee[] the present case falls under Type III category. A signed consent form was obtained from the patient.\nBefore planning for the surgery, complete plaque and calculus removal was done. After giving anesthesia, bone sounding was carried out to determine the amount of osseous reduction to be done. Undisplaced flap surgery with osseous recontouring was performed to increase the clinical crown length and to maintain the biologic width [].\nMaximum preservation of keratinized gingiva was performed. Ostectomy followed by osteoplasty was performed to obtain at least 4 mm of healthy tooth structure above the alveolar crest. The scalloping of the flap was performed anticipating the final underlying osseous contour. Flaps were sutured back. Routine post-operative instructions were given. The medications prescribed were, amoxicillin 500 mg tid for 5 days and paracetamol tid for 3 days.\nPatient was recalled after 1 week for suture removal and after 1 month for re-evaluation []. 3 months post-surgery, the patient was referred to the Department of Conservative Dentistry and Endodontics for the fabrication of the crowns []. |
A 69-year-old Caucasian male was referred to our hospital with 3 weeks of abdominal distension and worsening right lower quadrant pain. He was diagnosed with IgG kappa multiple myeloma four years prior to presentation. He was initially treated with bortezomib/dexamethasone with monthly zolendronic acid with good response initially; however, a year after diagnosis, he was found to have disease progression which manifested as a right radius fracture. His regimen was switched to lenalidomide with dexamethasone with good response and clinically depressed levels of paraproteins. After completion of 9 months of therapy, he underwent autologous stem cell transplant with high-dose melphalan. 7 months after bone marrow transplant, his disease progressed with involvement of pericardial fluid. Salvage therapy was initiated with pomalidomide, bortezomib, and dexamethasone which was discontinued a year later due to peripheral neuropathy; however, at the end of treatment, there was no evidence of ongoing disease.\nWhen the patient presented to our hospital, he had an acute abdomen. Initial blood work revealed a normocytic anemia with hemoglobin of 8.4 g/dl and elevated ESR of 44. He also had acute kidney injury with creatinine of 3 mg/dl (baseline of 1.9 mg/dl). CT scan of the abdomen and pelvis revealed extensive stranding seen throughout the abdomen within the peritoneal space with edema in the mesentery ().\nHe underwent an exploratory laparotomy which revealed induration of the entire base of the mesentery and retroperitoneum. He had an IgG level of 4407 units with predominantly kappa light chains whose level was 4833 units (kappa to lambda ratio 540). Pathology revealed extensive mesenteric infiltration by kappa restricted plasma cells positive for CD138 on immunohistochemistry, without evidence of amyloidosis. Bone marrow biopsy revealed a 30% involvement by plasma cells (Figures –). Cytogenetics showed 1q22 duplication, trisomy 7 and 15, and gain of 8q24.1. The skeletal survey revealed lytic lesions in the left femur and skull (Figures and ).\nHe was started on carfilzomib and dexamethasone therapy for relapsed multiple myeloma. Unfortunately, he died within one day of start of the chemotherapy from surgical complications of bowel obstruction. |
A 60-year-old man underwent ascending aorta replacement (Gelweave Valsalva 28, Vascutek Inc, Glasgow, United Kingdom) and aortic valve repair for a 62-mm aneurysm (Fig ). Because of acute postoperative bleeding, the aortic graft was replaced by a composite graft equipped with a mechanic aortic valve. On postoperative day 19, the patient developed a systemic inflammatory response syndrome with fever, leukocytosis, and tachycardia. The focus of infection appeared to be a sternal osteomyelitis with delayed healing of the sternotomy wound, purulent secretion, and perifocal erythema. Blood cultures and wound swabs were collected before empiric antibiotic therapy with cefepime was started. Growth of Staphylococcus aureus (sensitive to oxacillin) was found in all specimens. Sternal debridement was performed and the antibiotic therapy was changed to a combination therapy (flucloxacillin, rifampicin, and an aminoglycoside). A prosthetic valve endocarditis could not be excluded, although no typical vegetations were seen by the transesophageal echocardiography. After 2 weeks, a second sternal debridement was necessitated because of persistent wound secretion and dehiscence. The wound closure with a VAC (KCI International, the Netherlands) system was performed by the cardiovascular surgeon and an antibiotic therapy with only flucloxacillin and rifampicin was continued. Nevertheless, the patient developed fever and elevated white blood cells counts (Table ). At this point, the removal and replacement of the probably infected composite graft was discussed and the plastic surgery team was consulted. The treatment strategy agreed upon consisted of a radical debridement and the introduction of well-vascularized tissue around the graft and the sternal wound (1) to fill dead space and (2) to increase the effect of antibiotics locally.\nThe preparation of the pectoralis muscle flaps as a turnover flap on the right side pedicled on the basis of perforators from the internal mammary artery, and an advancement flap pedicled on the thoracoacromial vessels on the left side, was followed by a subtotal sternal debridement. Both flaps were placed in the sternal wound and wrapped around the graft from both sides. The soft-tissue defect was closed primarily after placing 4 drains. Operation time was 280 minutes. Swabs taken intraoperatively from the graft showed growth of Candida albicans but absence of S aureus. Fluconazole was added to the antibiotic regime.\nFollowing this intervention, infectious parameters decreased and hemodynamic and respiratory parameters had stabilized so that the patient could be extubated and could leave the intensive care unit 2 days later. One month after radical debridement and wrapping the bilateral muscle flaps around the graft, the patient was discharged.\nTreatment with flucloxacillin 6 × 2 g IV per day and rifampicin 2 × 450 mg po per day for a total of 6 weeks, initially combined with amikacine 1g IV per day for 2 weeks, was stopped on the day of discharge.\nThe conclusion to replace the yeast-infected graft was reached interdisciplinary. For technical and clinical reasons, such as the size of the needed homograft and the poor condition of the patient, we decided to treat this patient conservatively with fluconazole 200 mg po per day (dose adapted to renal insufficiency).\nThe laboratory test values normalized (Table ) and fluconazole was stopped after 8 months. Blood culture results 1 and 2 months after stopping the antibiotic therapy as well as 1 and 2 months after stopping fluconazole remained sterile. One year after surgery, the patient was in good condition. The examination showed no signs of infection as well as sufficient chest stability and good function of the upper extremity with slightly decreased strength of adduction. Respiratory functions remained unaltered compared with the preoperative status. The follow-up computed tomographic scan showed perfused muscle flaps around the ascending aortic graft and no evidence of mediastinal fluid collections (Fig ). |
A 55 year old woman presented with nonspecific progressive discomfort in the right hypochondrium dating back two months. Additional studies were carried out. An ultrasound scan showed a hepatic nodule of 92 mm in widest diameter in segments IV, V, and VIII, while the results of all the other exams were normal. This was supplemented by a computerised axial tomography (CAT) scan which revealed a heterogeneous formation of 77 mm x 76 mm with hypodense areas in its interior, and a peripheral enhancement, located in hepatic segment VIII. The magnetic resonance imaging (MRI) revealed an expansive well-defined heterogeneous formation 81 x 72 mm, T1-hypointense, and T2-hyperintense with restricted diffusion. An endovenous contrast injection faintly highlighted a peripheral capsule in later cuts ().\nWe reviewed the patient’s medical history, which revealed that 13 years earlier she had undergone surgical intervention for a 10 cm mediastinal tumour, which was completely resected. The histopathological findings were consistent with a thymoma surrounded by an undamaged fibrous capsule. Three years later, the patient underwent a total thyroidectomy on account of a thyroid papillary carcinoma. There was no other relevant medical history data.\nGiven the clinical and imagenological findings described above, it was decided to surgically remove hepatic segments IV, V, and VIII.\nThe macroscopic examination showed a solid, whitish tumour with hemorrhagic and cystic degeneration of 9 x 9 cm. This was found to be surrounded by a thin capsule (). The histological sections of material, embedded in paraffin and coloured with haematoxylin and eosin, showed that the lesion comprised numerous small, monomorphic lymphocytes, without cytological atypia. Among these were also seen occasional cells with large eosinophilic cytoplasm and nuclei with finely granular chromatin. In addition, we saw structures that mimicked Hassall’s corpuscles and perivascular spaces in a stockade of epithelioid cells around the vessels. The lesion presented an expansive border which delineated it from the adjacent hepatic parenchyma, which itself showed no significant changes ( and ).\nThe immunohistological techniques were carried out on histological sections of 3 microns by means of an automated system in accordance with the manufacturer’s guidelines (Benchmark XT, Ventana). shows the immunoprofile of the epithelial and lymphocyte components of the case and the monoclonal antibody used.\nThe lymphoid population showed intense positivity for CD3, CD5, CD8, and CD99 ( to ) and weak positivity for CD4 and CD43. The cytokeratin cocktail showed positivity in the tumour’s epithelial component ( and ).\nStaining for the Epstein-Barr virus as well as CD30, CD15 and B lineage markers including follicular differentiation (PAX5, CD20, CD10 and BCL6) were found to be negative. There was no overexpression of protein p53.\nThe morphological picture in junction with the immunoprofile and the patient’s medical history allowed the diagnosis of hepatic metastatic thymoma of sub-type B1, according to the WHO classification.\nAt present, the patient is being regularly monitored and is free of illness six months after hepatic excision. |
An 85-year-old right-hand dominant lady was admitted to the emergency care department after a fall. She had pain, deformity and restriction of movements of her right shoulder joint. She also complained of right chest pain. There was no significant past medical history and she was not on any oral anticoagulants.\nOn examination, there was deformity consistent with dislocation of the right shoulder. Movements of the right shoulder were restricted due to pain. The radial pulse was present and there was no distal neurovascular deficit. Radiograph revealed anterior dislocation of the right shoulder with comminuted fracture of the greater tuberosity (). Chest radiograph revealed fractures of 2nd, 3rd and 4th ribs on the right side. The haemoglobin level was 12gm/dl.\nAfter resuscitation, the patient`s right shoulder was reduced with closed manipulation under sedation and Entonox inhalation, confirmed with check post-reduction radiograph, with greater tuberosity fragment mildly displaced (). Examination of the right upper extremity after reduction revealed intact radial artery pulse and evidence of posterior cord palsy of the brachial plexus. The patient was transferred to high dependency unit (HDU) for monitoring and observation.\nAfter six hours of admission to HDU, the patient’s haemoglobin level continued to drop steadily, and she was transfused with six units of blood over a period of ten hours. The radial artery pulse was present throughout this time. However, there was continuous drop of blood pressure and haemoglobin level despite aggressive resuscitation. CT angiogram showed a disruption of the intima at a single point of the right axillary artery and a large pseudo aneurysm due to avulsion of posterior circumflex artery with a large hematoma ().\nThe Interventional Radiology department intervened and following selective cannulation of the right subclavian artery, a 6cm Polytetrafluorethylene- (PTFE) covered stent (Atrium Advanta V12) was deployed across the traumatic pseudo-aneurysm of the right axillary artery with good completion of the angiography and with no further evidence of leak ().\nThe patient then developed pulmonary atelectasis and consolidation of the right lung. She was treated with intravenous Tazocin. Over the next two weeks she continued to improve but on the 14th day died due to myocardial infarction. |
A 45-year-old man presented with severe mitral stenosis (MS), with a mean gradient of 12 mmHg and a calculated valve area of 0.8 cm2. There was associated mild tricuspid regurgitation and moderate pulmonary hypertension. An angiogram revealed normal coronary arteries. Left ventricular (LV) systolic function was preserved with an ejection fraction of 55%. The patient was planned for MVR. His routine blood investigations demonstrated increased lactate dehydrogenase (LDH) levels to 345 IU/L (normal LDH range: 100–190 IU/L), but haemoglobin level, serum bilirubin, liver enzymes, and coagulation profile were normal. During blood grouping and cross matching, altered clotting response was detected at 37°C that led to the suspicion of presence of CAs. An immediate haematology consultation was obtained and the baseline CA titer was found elevated (1:1024 at 4°C) with a high thermal amplitude of 30°C. Oral prednisolone was prescribed at a dose of 60 mg once daily for 1 month and CA titers were repeated periodically, as shown in . The patient was taken up for surgery after confirming CA titers of less than 1:16 at 30°C. He underwent uneventful balanced general endotracheal anaesthesia. Comprehensive haemodynamic monitoring included a radial arterial catheter, a central venous triple lumen catheter, and transoesophageal echocardiography (TOE). Intraoperatively, intensive temperature monitoring was done and all necessary precautions were taken to avoid exposure to the active temperature range of CAs. Intravenous fluids and irrigation fluids were warmed before administration. The inhalational gas supplied to the patient was set at 37°C using a heated circuit. Lower body thermal blanket and higher operation theatre temperature helped to maintain the core temperature above 34°C. The patient was anticoagulated with heparin to achieve activated clotting time (ACT) of more than 480 s. Warm crystalloid fluid was used to prime the CPB circuit and warm blood cardioplegia was delivered through aortic root to sustain electromechanical silence while targeting the myocardial temperature above 32°C. Pressures within the CPB and cardioplegic circuits remained within the normal limits throughout the procedure. The circuitry was visually monitored for any evidence of agglutination of the RBCs. A 27-mm size Medtronic mechanical mitral valve (MV) prosthesis was inserted after excising the native valve tissue. The total cross-clamp time was 66 min, and the patient was weaned successfully from CPB with minimal inotropic support of dobutamine. Inspection on TOE revealed normal biventricular systolic function and normal functioning of the implanted MV prosthesis. The patient underwent clinical fast tracking in the intensive care unit (ICU) with no evidence of haemolysis or end organ dysfunction. The patient was discharged on the seventh postoperative day. |
A 72-year-old Caucasian woman was referred to a community rheumatology service with insidious onset of lower back pain and stiffness. The symptoms gradually progressed to involve the shoulder girdle, with prominent systemic symptoms of weight loss, fever and malaise. There were no symptoms suggestive of CTD or spondyloarthritis. Owing to severe headache, the patient was seen several times in primary care, in Accident and Emergency, and was found to have a normal CT brain.\nThe patient’s shoulder and hip girdle symptoms had largely resolved in the 3 weeks before consultation without any intervention, after which she gradually developed severe right unilateral frontal and temporal headaches in additiont o right upper limb claudication.\nOn clinical examination, there was no peripheral arthritis. Shoulder active range of motion was reduced. The chest was clear on auscultation, and there was no lymphadenopathy. There was no temporal or axillary tenderness. The left temporal pulse was palpable, whereas the right was absent. The peripheral radial and ulnar pulses were normal.\nLaboratory investigations revealed a CRP of 16 mg/l, ESR 17 mm/h, normal full blood count, normal renal and liver function and a mild thrombocytosis. A previous lumbar spine radiograph and CT brain were normal.\nTaking into account the history and clinical presentation, a suspected diagnosis of polymyalgic onset large vessel GCA was made, and the patient was referred to the Acute Medical Unit. This is a secondary care-based service for acutely unwell patients referred from primary care. She was started on high-dose oral prednisolone 60 mg daily, with rapid improvement in her symptoms.\nBefore initiation of CSs, as mentioned previously, this patient had been investigated extensively with numerous blood tests, CT brain, lumbar spine radiograph and chest radiograph. Subsequently, a temporal US was found to be positive for temporal arteritis, demonstrating a halo and intima–media thickness for the right temporal artery of 0.37 mm, as shown in .\nAlthough the initial presentation was not unusual for PMR, several important points relating to PMR and large vessel vasculitis can be appreciated in this case. It is noteworthy that not every onset of PMR involves the proximal shoulder girdle. According to Kniazkova et al.[], 15–30% of patients can present with lower hip girdle pain and stiffness at onset; however, true PMR will progress to involve the shoulders []. This is of particular importance, because we should not exclude PMR if the shoulders are not involved at the initial presentation.\nGCA has been reported in 10–20% of PMR patients, and these conditions have a large overlap. According to Dejaco et al. (2017) [], previous focus was placed on cranial vasculitis, but recent research has suggested a strong overlap of cranial vasculitis and large vessel extracranial vasculitis with PMR. This form of large vessel GCA can be present in ≤30% patients with PMR, particularly those who do not respond adequately to low-dose glucocorticoid therapy []. This case illustrates a typical example of polymyalgic onset of symptoms, with raised inflammatory markers followed by cranial symptoms (headaches) and thereafter extracranial symptoms (constitutional symptoms, upper limb claudication), and the US imaging confirmed the diagnosis of cranial and large vessel GCA [].\nThe attending clinician should always screen for large cranial and extracranial vessel vasculitis as part of the polymyalgic syndrome, especially in patients who present with strong constitutional symptoms. This can require additional tests, such as vascular US or a PET-CT scan for large vessel GCA. Our case report also suggests the need for better training for physiotherapists involved in the assessment/triage of musculoskeletal symptoms of possible rheumatological origin. In this case, the patient initially presented to a Musculoskeletal service Physiotherapist lead as mechanical hip pain. However, after careful history and examination, she was referred to the Acute Medical Unit with suspicion of polymyalgic onset large vessel GCA. We suggest that such formal training should be considered in order that physiotherapists can assist general practitioners in the assessment and management of rheumatological musculoskeletal conditions.\nFunding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript\nDisclosure statement: The authors have declared no conflicts of interest. |
A 69-year-old female was referred to our clinic for an incidental finding of a large Morgagni hernia found on a recent CT chest scan for lung cancer screening. Patient reported occasional shortness of breath after prolonged ambulation but denied chest pain. She did have remote history of acid reflux symptoms but nothing recently. She denied issues with prematurity or issues with development as an infant, chest trauma, or MVA history. She did complain of occasional right shoulder pain but attributed this to arthritis. Denied history of heart attack, stroke, DVT, or PE. She had a 30-pack-year smoking history but quit a year prior. She was up-to-date on her colonoscopy, current within the past year. She denied hematochezia and melena, bowel habit changes or major body weight changes as well as any current abdominal pain. On examination her vitals were within normal parameters. Heart and lungs were unremarkable. Abdominal examination was soft with normal bowel sounds and nontender. Remainder of examination was unremarkable. Laboratory values included a normal CBC and BMP. A CT chest scan had demonstrated a large retroxyphoid hernia of Morgagni involving several loops of small bowel and transverse colon located in the right inferior hemithorax (Figs and ). No evidence of acute incarceration or strangulation were noted. A detailed discussion was undertaken with the patient regarding her hernia and she was consented for a laparoscopic repair with mesh.\nPatient underwent a laparoscopic approach in lithotomy positioning with the primary surgeon working between the legs. Three working ports were used, a 12 mm port at the umbilicus and two 5 mm ports; one in the LUQ and one in the RUQ. Upon initial laparoscopy multiple loops of small bowel were progressively reduced out of the hernia sac which also included the ascending colon and part of the transverse colon (Figs and ). All the small bowel and the colon appeared viable. The redundant parietal peritoneal hernia sac was excised out of the right inferior hemithorax utilizing a LigaSure (Covidien) (Fig. ). The falciform ligament was also taken down all the way to the diaphragm. The defect in the diaphragm measured to be approximately 9 cm by 4 cm. A section of Pariatex composite mesh was then trimmed to 2 cm in width by 9 cm in length. Three stay sutures of 0 Ethibond were placed laterally and in the middle of the mesh. This was placed into the peritoneal cavity after soaking it in vancomycin with local anesthetic. The sutures were then percutaneously brought through the diaphragm edge that was unattached to the anterior abdominal wall and then subsequently through the anterior abdominal wall. These were then tied thereby re-approximating the unattached edge of the diaphragm to the anterior abdominal wall near the xiphoid (Fig. ). Additional 0 Ethibond sutures were placed in between these initial ones percutaneously with a suture passer.\nAdditionally, another Pariatex composite mesh was then trimmed to 12 cm in width by 9 cm, soaked in vancomycin with local anesthetic and then placed into the abdominal cavity. It was positioned over the area of the repair and fixed into place with absorbable tacks around its caudad edge and centrally. Along the cephalad edge it was fixed with a running V-lock absorbable suture to the diaphragm. Fibrin glue was placed along this same edge (Fig. ). The ports were removed and incisions were closed.\nPatient’s postoperative course progressed well. She was monitored overnight and discharged the following day. She was seen for follow-up in 2 weeks out of surgery and did quite well. She was tolerating a regular diet and having bowel movements. A month after surgery another CT scan was obtained which demonstrated a postoperative seroma in the right inferior hemithorax (Fig. ). Currently, the patient is to be seen in a 6-month follow-up to have another CT scan at that time. |
A 42-year-old male, nonsmoker, with medical condition significant for hypertension presented to the emergency department after a fall followed by two episodes of seizures. On presentation physical examination was notable for altered level of consciousness and mild symmetrical decrease in power of 4/5 in all four limbs. Laboratory workup including complete blood count, electrolytes, coagulation panel, lipid profile, urine, and serum drug screen was unremarkable. CT scan head revealed a 1.5 cm left temporoparietal lobe intraparenchymal hemorrhage with surrounding edema as shown in (). As part of the diagnostic workup, an ECG was also performed on admission which was normal. The patient was admitted to the neurointensive care unit (NICU) for further management. A computerized tomography angiogram was performed, which showed early draining veins at the site of the lesion, suspicious for an underlying vascular malformation. Subsequently a cerebral angiogram was performed which confirmed the presence of an AVM underlying the hemorrhage (). A partial embolization of the AVM was performed, and the patient was boarded for surgical resection ().\nOn day 3 of admission, the patient complained of sudden-onset chest pain. He described it as left sided, retrosternal, sharp, nonradiating pain, worsened when lying down on left side, lasted 2-3 minutes and then resolved spontaneously. It did not recur however prompted an ECG which showed sinus rhythm with nonspecific ST segment elevation in leads V3-V6 (). Cardiology was consulted who deemed the ECG changes as J point elevation suggestive of benign early repolarization and not a true acute coronary event. A high sensitivity cardiac troponin assay done immediately and repeated two times at 6 hours and 12 hours from the onset of symptoms remained negative (<0.017 ng/ml; normal value <0.057 ng/ml). A transthoracic echocardiogram (TTE) performed later that day revealed no regional wall motion abnormalities or left ventricular dysfunction. The next day, patient was taken for craniotomy and surgical resection of the AVM (). The surgery was uneventful. A follow-up ECG on the postoperative day 1 revealed pronounced ST elevation with new T wave inversions (in leads V2-V6) highly suggestive of acute STEMI (). The patient was completely asymptomatic with no chest pain or other cardiac symptoms. Serial estimation of high sensitivity cardiac troponin was again negative (<0.017 ng/ml) and a repeat TTE was unremarkable. Given these findings and the absence of the symptoms, no intervention was done and he was monitored in the NICU.\nThe patient did not have any further untoward event(s) and continued to do well postoperatively with normalization of his ECG changes over the next 48 hours (). He improved neurologically and was transferred out of the ICU on day 7. He was subsequently discharged on day 12 with home health physical therapy, neurosurgery, and cardiology follow-up appointments. An exercise stress test was eventually performed 3 months' after discharge which did not reveal any evidence of coronary artery disease. |
An 80-year-old Caucasian female with history of hypertension and chronic back pain presented for emergent repair of a 7.2 cm aneurysm of the ascending aorta with Stanford classification type A dissection. Because the ascending aorta was unsuitable for arterial cannulation, the surgeon elected to perform axillary cannulation via the right subclavian artery with side graft anastomosis. The patient arrived to the operating room (OR) with nicardipine and esmolol infusions running through an 18-gauge peripheral intravenous (IV) line in the right antecubital (AC) fossa. Prior to induction of anesthesia, we disconnected the infusions from the right AC and administered medications though an 18-gauge IV in the left forearm. The patient also had a left radial arterial line (AL), and we placed the pulse oximeter and noninvasive blood pressure (NIBP) cuff on the RUE. After intubation, we placed a right radial AL, and the surgeons placed a left femoral AL. All arterial pressures correlated closely. Additionally, a 9-French central line was placed in the right internal jugular vein. The patient was cleansed and draped for surgery with her arms tucked to her sides.\nShortly after the procedure began, the right radial AL tracing went flat, and the pulse oximeter waveform was lost. We attributed this to the surgeon partially clamping the right subclavian artery in preparation for arterial cannulation. We switched the pulse oximeter to the left hand and relied on the left radial and femoral AL for pressure readings. Just prior to arterial cannulation, we noted that the right radial pressure returned, although about 20 points lower than the left radial/femoral. Immediately after initiating CPB, the right radial mean arterial pressure (MAP) increased to 200 mm Hg, and left radial/femoral MAP decreased from 60 to 30 mmHg. The perfusionist alerted the surgeon about the high line pressures and decreased CPB flows. After a brief attempt to troubleshoot and adjust the cannula with little improvement in pressure or flow, the surgeon proceeded with the operation. Over the next few minutes, the left radial/femoral MAP increased to 60 mmHg.\nAs deep hypothermic circulatory arrest (DHCA) was initiated, the right radial MAP decreased to 30 mmHg and left radial/femoral MAP decreased to 10. When CPB was reinstated, right radial MAP again increased to 200 mmHg. After 29 minutes of DHCA and 265 minutes of CPB, the patient was successfully weaned from CPB, and right radial MAP decreased to about 10 points lower than left radial MAP.\nDespite these issues, the surgery was otherwise uneventful. However, upon the surgical drapes being taken down, we noticed that the patient's RUE was swollen with blisters and bullae from the shoulder to the hand; yet, the skin of the upper arm where the NIBP cuff had been placed was normal as seen in figures. The IV in the right AC appeared to be infiltrated and weeping fluid even though we had not used it during the case and did not have any IV fluids attached to it (Figures and ). The IV and right radial AL were removed in the OR, and a Xeroform gauze dressing was applied to the RUE with the surgeons present. The intensive care unit nurses were instructed to elevate the arm and perform hourly neurovascular checks.\nOn postoperative day 1, the patient complained of tenderness and burning in the RUE, but she maintained adequate capillary refill, motor function, and sensation. Plastic Surgery was consulted to rule out compartment syndrome. They were unsure of the diagnosis but recommended nonoperative management and continued neurovascular checks. Eventually Dermatology was also involved, and they performed a punch biopsy of the patient's right dorsal hand. Their initial diagnosis was allergic contact dermatitis (ACD) due to the fact that the area of skin covered by the NIBP cuff was spared. However, the biopsy showed pauci-inflammatory dermal-epidermal blistering, which did not favor ACD. Direct immunofluorescence was also negative, ruling out localized pemphigus. Given the histologic findings, the final diagnosis was hydrostatic edema/bullae correlating with rapid edema during surgery. The patient continued to be managed nonoperatively with 1% triamcinolone ointment and gauze dressings, and within one month the blisters had completely resolved. |
A 17-year-old man was involved in a road accident in which he suffered the open fractures of the right femur and tibia. At the arrival to the Emergency Dept (ED), he was alert and hemodynamically stable and the Glasgow Coma Scale (GCS) was 15; the initial alignment of the fractured ends was performed in the ED with a gentle traction performed under sedation with iv. ketamine; a total body CT did not demonstrate other injuries. Approximately two hours after the admission the patient was taken to the surgical theatre for the external fixation of the fractured bones; at entering the operating room, the GCS was 8, the arterial pressure was 115/80 mm Hg, the heart rate was 115 bpm, and the arterial oxygen saturation (SPO2) was 85 at room air; the procedure was performed under general iv anesthesia with propofol and remifentanyl; the standard monitoring included the ECG, the noninvasive arterial pressure, the SPO2, and the end-tidal CO2 (ETCO2); during the intervention, the SPO2 rose to 100% at a FIO2=40% and all the other variables remained stable throughout the procedure after the 3-hour-long intervention in which the complete alignment of the bony ends was achieved; the patient was transferred to the Intensive Care Unit (ICU) still intubated and mechanically ventilated; the iv anaesthetics were gradually tapered until the complete suspension. Two hours later, the SpaO2 and the ETCO2 slightly decreased and anisocoria was observed; and an urgent CT scan of the head demonstrated a diffuse cerebral edema and the herniation of the cerebellar tonsils (Figures and , respectively). At this time, the pupils became bilaterally mydriatic and the EEG was almost isoelectric; due to the severity of the conditions, a MR scan was considered unnecessary. On the basis of the clinical and radiologic findings repeated boluses of iv. mannitol and steroids were given in the following hours aiming to reduce the intracranial pressure. An echocardiogram demonstrated a severe right ventricular depression with an ejection fraction of 20%. On the following day, the patient was declared brain dead according to the current Italian law.\nAt the autopsy, the cerebral microvascular network appeared diffusely plugged with BME (Figures –) and ischemia-related microcalcifications were scattered throughout the brain (); other organs were less extensively involved; no PFO was demonstrated. |
A 49-year-old man with a history of acromegaly was admitted to our hospital with the concern of recurrent shortness of breath and dyspnea on exertion during the previous 2 years, and he had experienced an episode of presyncope 2 weeks prior without any further evaluation. He was a chef in a local restaurant for almost 30 years. He had no family history of any diseases and no past history of hypertension, diabetes mellitus, sleep apnea, or sudden cardiac death. He did not smoke or consume alcohol. The patient provided a history of stereotactic radiosurgeries twice in a decade or so and adherence to treatment with a somatostatin analog (octreotide given 40 mg once per month through intramuscular injection) at the time of diagnosis 20 years before. The patient was overweight and moderately nourished. He was 1.85 m (73 inches) tall, weighed 134 kg, and had a body mass index of 39 kg/m2. His blood pressure was 110/60 mmHg, and his heart rate was 92 beats/min with sinus rhythm. He had distinct skeletal features that included prominent superciliary arches and nose bridge, enlargement of the tongue and lip, and large hands and feet. Cardiac auscultation revealed irregular premature beats and pathological third heart sound, and a systolic murmur was discovered over the apex and aortic area. Bilateral extensive borders of cardiac dullness were noted. His physiological reflexes were present without any pathology. An electrocardiogram demonstrated sinus rhythm with wide (160 ms) QRS duration of left bundle branch block (LBBB) (Fig. ). The patient’s condition was classified as New York Heart Association (NYHA) stage III–IV.\nOn admission, magnetic resonance imaging showed pituitary macroadenoma. Given the symptoms described, we arranged blood testing of myocardial injury markers showing an elevated brain natriuretic peptide level of 740 pg/ml indicating cardiac failure (Table ). Hormone laboratory tests performed subsequently demonstrated excessive secretion of GH and IGF-1, twofold greater than the reference normal upper limit, which was consistent with pituitary macroadenoma (Table ). Other routine analyses of liver and renal function were roughly normal.\nA Holter monitor was ordered for underlying arrhythmias to explain the patient’s dyspnea, chest discomfort, and presyncope. It demonstrated sinus rhythm with an average heart rate of 68 beats/min, frequent ventricular premature beats, and nonsustained ventricular tachycardia (up to 2200 ms) (Fig. ).\nA chest x-ray showed a cardiothoracic ratio (CTR) of 78%. Echocardiography showed diffuse impairment of left ventricular (LV) systolic motion, reaching an LVEF of 16%. We noted hypertrophy of the ventricular septum at 18 mm, ventricular dilation, with LV diameter of 72 mm. The right ventricle and atrium and the left atrium were also dilated with moderate mitral regurgitation and mild tricuspid regurgitation. There was no associated systolic anterior motion (SAM) of the mitral valve. Dyssynchrony of the biventricular systolic motion was apparent.\nGiven an exertional component to the symptoms together with echo presentations in order to better exclude ischemic cardiomyopathy, coronary angiography was performed, which showed normal coronary arteries without stenosis, and left ventriculography applied simultaneously revealed an EF of 20% with diffuse LV hypokinesis.\nGiven the patient’s previous medical history of acromegaly, the absence of obstructive coronary artery imaging findings or segmental dyskinesia, family history of hypertrophic cardiomyopathy (HCM), symmetric hypertrophy, as well as absence of SAM of the mitral valve, acromegaly-induced cardiomyopathy was confirmed, which was absolutely opposed to coronary heart disease (CHD) and HCM.\nThese results indicated that it was probably not a case of hereditary cardiomyopathy; therefore, we diagnosed the patient as having secondary dilated cardiomyopathy due to acromegaly, even taking it a step further progressing to congestive heart failure secondary to acromegaly-induced dilated cardiomyopathy.\nChronic excess of GH and IGF-I secretion affects cardiac morphology and performance [], so etiological treatment for acromegaly-induced cardiomyopathy is crucial to suppressing GH secretion or blocking GH action for the sake of reversing acromegaly-induced cardiomyopathy. The mainstay of treatment acknowledged globally is surgical resection of the pituitary adenoma [], which was unfortunately considered high-risk given our patient’s cardiac condition (NYHA stage III–IV). Although stereotactic radiosurgery combined with somatostatin analogs and GH antagonists administrated previously were effective in suppressing hormones, they could not help his cardiac function. Therefore, we carefully administered diuretics, vasodilators, angiotensin-converting enzyme inhibitor (ACEI), β-blockers, and spironolactone for management of heart failure following the current guidelines []; in the meantime, octreotide (200 μg/day) was administered for the control of GH excess. After good compliance of pharmacotherapy and a regular medical examination regimen for nearly half a year, the serum GH and IGF-1 concentrations decreased from 32.50 ng/ml to 1.98 ng/ml and 627.00 ng/ml to 229.10 ng/ml, respectively, but the patient was hospitalized again because of uncontrollable cardiac failure. Accompanied by the normalization of GH and IGF-1 levels, the patient’s cardiac function did not seem to take a favorable turn upon readmission. Though echocardiography showed a recovered EF value from 16% to 28%, a significant ventricular mechanical dyssynchrony was detected as formerly. Electrophysiological study was performed using a nonaggressive stimulation protocol, which revealed a nonsustained ventricular monomorphic tachycardia []. In the presence of overt ventricular dyssynchrony, complete LBBB, LVEF< 35%, inducible ventricular tachycardia, and symptomatic heart failure despite guideline-directed medical therapy, surgical indication was rarely assessed by neurosurgeons, and stereotactic radiosurgery together with pharmacotherapy produced infinitesimal effects. Therefore, we boldly recommended cardiac resynchronization therapy with defibrillator (CRT-D) implantation based on device implantation official guidelines [, ]. The patient underwent CRT insertion finally and was discharged to home 5 days later, pharmacotherapy continued as usual (Fig. ).\nTelephone follow-up was arranged, and the patient claimed symptom improvement following the device insertion 1 month later and was basically back to normal life. We required that he return for follow-up at 1 month, 3 months, and 6 months after the interventional therapy. The patient has been followed in our outpatient clinic for nearly half a year now. During his last visit, echocardiography identified improved LVEF of 54%, and a chest x-ray showed reduced CTR of 60%. The patient was in NYHA functional class II (Fig. ). |
A 74-year-old Japanese woman noticed a tender lump in her right breast. She immediately went to a breast clinic to get a breast cancer screening. She had no family history of breast and ovarian cancer. After a month, she was referred to our institution with suspicion of metaplastic breast carcinoma with a core needle biopsy at the breast clinic. Physical examination revealed a hard, tender, and 25-mm mass in the upper outer quadrant of her right breast and a palpable lymph node in her right axilla. Mammography indicated an indistinct mass on the mediolateral oblique view and the craniocaudal view. Ultrasound (US) showed an 18 × 16-mm, irregular-shaped, and hypoechoic mass with a suspicion of a spread to the nipple inside the duct (Fig. a) and several swollen lymph nodes in levels I to II (Fig. b). Magnetic resonance imaging (MRI) detected enhancement of a 17 × 17-mm indistinct mass surrounded with a non-mass enhanced segmental lesion toward the nipple side spreading a maximum of 74-mm range, which had no interaction with the chest bone, muscles, and breast skin, in the right breast tissue (Fig. ). Invasive carcinoma with multiple axillary lymph node metastases was strongly suspected on clinical examination and imaging. Histological evaluation of the biopsy for the mass revealed a tumor with the growth of oval and spindle-shaped cells and multinucleated giant cells, the infiltrating lymphocyte into the breast tissue, and hyalinization in the stroma. The multinucleated giant cells stained positively for CD68. A part of the oval and spindle mononuclear cells stained weakly positive for CD68. These tumor cells stained negatively for estrogen receptor (ER), progesterone receptor (PgR), and human epidermal growth factor receptor 2 (HER2). There was a focal hemorrhage without necrosis. Few non-epithelial atypical cells were observed in the breast duct, but no atypical epithelial cells consistent with breast cancer were detected. GCT of the breast, breast cancer with OGCs, and giant cell-rich sarcomas should have to be considered as differential diagnoses, and the pathological findings suggested most GCT. Fine needle aspiration biopsy for the swollen lymph node revealed only normal lymphocyte, even though metastatic lymph node was strongly suspected on US. However, we could not rule out the possibility that the biopsy tissue showed a part of malignant tumor with OGCs and biopsy for the lymph node was false negative, because there was a gap between the clinical presentation, such as a tender mass suggesting rapid growth and multiple lymphadenopathies, and the pathological presentation of biopsy tissue. To obtain further evidence of malignancy, the tumor was sampled using a vacuum-assisted US-guided biopsy again. The result was the same as the prior biopsy. After discussing the treatment plan with the patient, we performed mastectomy and sentinel lymph node biopsy according to a surgical procedure for node-negative breast cancer with a wide ductal spread. The resection tissue histologically revealed similar findings to the biopsy specimen. The tumor was composed mainly of oval and spindle mononuclear histiocyte-like cells and multinucleated giant cells (Fig. ). The mitotic figure of these cells did not stand out. There was no evidence of malignancy, and only intraductal epithelial hyperplasia around the tumor, which did not fill the criteria of ductal carcinoma in situ (DCIS). No sentinel lymph nodes contained malignant cells, and we concluded the lymphadenopathies were a response to the inflammation around the tumor. Immunohistochemically, a high proportion of the multinucleated giant cells stained positively for CD68 (Fig. ). A part of the oval and spindle mononuclear cells stained weakly positive for CD68. These cells were negative for CK OSCAR, GATA-3, and MGB1 (Fig. ). These findings were consistent with the GCT of the breast. The patient received no adjuvant therapy because GCT-ST is usually considered as a benign tumor. She is being followed up with regular clinical examinations without any symptoms of recurrence after 1 year past from surgery. |
A 46-year old female patient, who had been suffering from autoimmune thyroid disease for eight years, presented at our clinic with an acute exacerbation of GO. Clinical examination revealed a convergent strabismus fixus with severe hypotropia of both eyes (Fig. ). The patient complained of increasing loss of eyesight and heavy retrobulbar pain. Visual acuity had deteriorated significantly from 0.6/0.5 to 0.1/0.1 within 3 months. A contrast enhanced orbital MRI scan showed distinct swelling of all extraocular muscles with bilateral compression of the optic nerve (Fig. ). There was no history of comorbidities except nicotine abuse. Laboratory tests showed a euthyroid biochemical status with TSH within the normal range, but elevated levels of Anti-Thyroid Peroxidase Antibody, Anti-Thyroglobulin Antibody and Thyroid Receptor Antibody. The patient’s daily medication comprised of 200 μg L-Thyroxin and 200 μg Selenium. Over many years, the patient had shown only mild to moderate symptoms of GO, but following a thyroidectomy, the symptoms had recently worsened dramatically. Since the disease could not be controlled by high-dose systemic glucocorticoid therapy, bilateral three wall orbital decompression had been performed twice previously. In the first step, the medial orbital wall had been resected via an endonasal approach. Due to ongoing findings as before, two months later partial resection of the orbital floor and fenestration of the lateral orbital wall via a combined transconjunctival/transcaruncular approach with piezosurgery had been performed. In addition, high-dose systemic glucocorticoid therapy was conducted prior to surgery and for the first two months after surgery. Glucocorticoid medication had to be gradually reduced until zero because the patient suffered from an upcoming depression and Cushing syndrome. Orbital radiotherapy for the treatment of thyroid eye disease had been considered as a therapeutic option, but the rapid progress of the disease with the growing risk of dysthyroid optic neuropathy forced us to act more quickly than orbital radiotherapy could perform []. Since the patient increasingly suffered from loss of vision and heavy pain attacks because of medial caudal squinting, we decided to correct the hypo- and esotropia surgically by releasing and repositioning the insertion points of the inferior and medial rectus muscle. Acute surgery was the last remaining treatment option.\nOperations on the left and right eye were performed consecutively with an interval of one week. The eye with the lower vision (left side) was chosen first. A forced duction test showed a complete fixation of the bulb. We opted for a transconjunctival approach (limbal incision) combined with lateral canthotomy to gain access to the dorsal part of the inferior part of the eye bulb. The insertion of the inferior rectus muscle was localized and then circuited with a squint hook (Fig. ). A non-absorbable polyethylene suture (Mersilene 4.0, Ethicon, U.S.) was placed at the anterior rim of the muscle before the muscle was detached from the outer bulb. This release in tension immediately resulted in the spontaneous elevation of the bulb. Lengthening of the muscle with an interposition graft (e.g. fascia lata) was not possible because of the deep retraction of the muscle. Instead, the polyethylene thread loop was directly fixed to the sclera at the former muscle insertion area, placing the muscle 12-15 mm dorsally with regard to its original fixation position. By marking of the original muscle insertion point with a non-absorbable polyethylene suture, an option could be preserved for a more precise muscle readaption at a later stage. Subsequently, the same procedure was carried out with the medial rectus muscle. Less tension allowed direct refixation of the muscle to the sclera without bridging by the polyethylene suture. The bulb was freely movable and remained vertically and horizontally in a primary position (Fig. ). Despite a significant exophthalmos, passive eyelid closure could easily be performed. The significant conjunctival contraction caused by the long-term hypotropia meant that only partial conjunctival wound closure with a polyglactin suture (Vicryl 7.0, Ethicon, U.S.) was possible. No postoperative complications occurred under the postoperative systemic antibiotic medication with Clindamycin of 3x600mg per day over three days supplemented by local application of Neomycin eye ointment for one week. Surgery of the right eye was conducted in the same manner (Fig. ) and under the same perioperative protocol one week later. Additionally, two mucosal grafts of 3.0 × 1.5 cm were harvested bilaterally from the inner cheek to be used for the bilateral reconstruction of the conjunctiva. The intraoral donor sites were closed primarily by using Vicryl 3.0 (Ethicon, U.S.). Starting from extreme eso- and hypotropia, the operation succeeded in repositioning the bulbs into the vertical and horizontal primary position with no restriction of passive movements. Three months postoperatively, the patient was free of pain and had a visual acuity of 0.3/0.6. Visual field testing (Goldmann perimetry) showed only slight concentric bilateral restrictions. Surprisingly, the patient did not suffer from diplopia despite the persistent restriction of active ocular mobility and a moderate bilateral exotropia (Fig. ).\nFollow-up examinations of the patient will be performed at close intervals, including ophthalmological check-ups and the testing of thyroid blood parameters. Contrast enhanced orbital MRI scans will allow the measurement of extraocular muscle volume, as described by Kolk et al. []. Orbital MRI scans were performed preoperatively and three months postoperatively (Fig. ) and the volumes of the extraocular muscles were calculated by using manual segmentation (Osirix Imaging software 5.9) (Table ). During this time interval, the total extraocular muscle volume increased from 24.91cm3 to 29.29cm3. Together with the ongoing high levels of thyroid-specific antibodies (Anti-Thyroid Peroxidase Antibody, Anti-Thyroglobulin Antibody and Thyroid Receptor Antibody), this indicated that the patient was still in an active stage of GO. Volumetric measurements of the extraocular muscles, based on follow-up MRI scans, will help to monitor the course of the disease. Further squint surgery, in terms of a precise readaption of the extraocular muscles, will be postponed to the future, when a more stable stage of the systemic autoimmune disease will be reached. |
58-year-old woman was referred by her General Practitioner to the Emergency Department with a one-week history of a moderately painful irreducible lump in her right groin, associated with nausea and vomiting. She did not have any urinary or bowel symptoms. She last opened her bowel earlier that day. She was known previously to have a spontaneously reducible lump in the right groin suggestive of a hernia.\nThe patient's medical history included hypercholesterolaemia treated with statins, menopausal symptoms treated with hormone replacement therapy, and pending investigation of a liver lesion. She had no known allergies.\nOn assessment the patient was in painful distress, she was tachycardic but otherwise her vital signs were stable. Her abdomen was soft and nontender; there was a palpable, nonerythematous lump in the right groin which was tender to touch and irreducible. The patient had marginally raised amylase of 57 iu/L and C-reactive protein (CRP) of 19 mg/L; her full blood count and renal and liver function tests were within normal limits.\nThe patient had an abdominal radiograph which was unremarkable. A groin ultrasound scan showed an approximately 5.2 × 2.7 cm cystic abnormality with a small communication with the abdominal cavity, suggestive of a femoral hernia.\nPatient then had an abdominal CT scan with oral and intravenous contrast (). This was reported as a right sided femoral hernia and the caecum and the ileocaecal junction were in close proximity to the hernial orifice. A tubular structure was seen in the hernial sac which did not take up the oral contrast; this was reported as an appendix in a femoral hernia.\nThe patient was taken to the operating theatre for an emergency right sided exploration and hernia repair under general anaesthesia. An infrainguinal transverse incision was made over the lump and the hernial sac was dissected free. The sac was opened revealing a congested appendix and caecum. The perfusion however normalised when the neck of the hernia was released. There was no evidence of perforation of the caecum or the appendix neither was there a periappendiceal collection. An appendicectomy was then done and the base was buried using purse-string technique with 3-0 absorbable polydioxanone suture. The reduction of the caecum back into the abdominal cavity proved challenging due to the narrowness of the femoral hernia defect which was therefore dilated, enabling the caecum to be manually reduced. The decision was made to repair the hernia defect with interrupted 3-0 polypropylene suture instead of mesh as a resection had been undertaken. The skin was closed with 3-0 reabsorbable poliglecaprone 25 suture and a pressure dressing applied. The postoperative recovery was uneventful.\nThe patient went home on day one after the operation. The resected specimen was sent off for histological analysis, which did not show any evidence of appendicitis. At six-month follow-up patient did not have any postoperative complications. |
A 45-year-old woman presented to our centre with a 2-year history of continuous progressive right-sided lower back and dull lateral abdominal pain radiating to her posterolateral thigh. The pain was independent of activities and poorly responded to nonsteroidal anti-inflammatory drugs and physiotherapy. A physical examination showed a painless palpable mass in the right paraumbilical and lower abdominal region without peripheral neurovascular deficits. Laboratory tests were normal, except for an unspecific, mildly elevated erythrocyte sedimentation rate. Abdominal ultrasound showed a large, encapsulated, oval soft-tissue mass, which was 21 × 16 cm in diameter in the right retroperitoneum. Subsequent abdominal MRI confirmed a homogenous soft-tissue tumour with a thick, smooth, capsular lining. This tumour lay in the right retroperitoneum paravertebrally and displaced the iliopsoas muscle distally and the right kidney craniolaterally, without any signs of ipsilateral hydronephrosis (). Although the tumour was large, it was considered benign because it was not heterogenous, had no irregular margins, and there were no signs of adjacent organ involvement. Management options were discussed at a staff meeting. We decided not to perform a percutaneous biopsy because of the difficult position of the tumour. Symptom-relieving surgery was decided as the best choice of treatment.\nFollowing preparation, the patient underwent surgery. The encapsulated mass was removed in toto together with its capsular lining, but without definable and clear peripheral nerve encasement or invasion. Identification of the nerve of origin is common, despite complete tumour excision. The procedure was performed without complications and there was no need for blood product substitution. However, extensive bleeding may be encountered from the adjacent vessels.\nA histological examination and immunohistochemical staining confirmed the diagnosis of benign encapsulated cellular schwannoma. The tumour had no infiltrative margin and there was a well-defined collagenous capsule and hyalinized vessels. The diagnosis was based on highly ordered, dense, cellular components, typical, well-organized spindle cells with a palisading arrangement (arranged in short bundles of interlacing fascicles), and with Verocay bodies formed by two parallel lines of nuclei with a nuclear space between them indicating an Antoni A pattern (). Reactive hyperplasia of adjacent lymph nodes was also found. Immunohistochemistry showed S100 protein-positive () and vimentin-positive, and epithelial membrane antigen-negative and CD34-negative tumour cells. The lack of epithelial membrane antigen immunoreactivity indicated the absence of perineurial cell differentiation. Therefore, the results of the immunohistochemical examination suggested benign schwannoma. The issue of whether the lesion was benign or malignant was resolved with the finding of a low proliferative index (Ki-67 = 1.6%), which highly suggested its benign nature ().\nThe patient’s postoperative course was uneventful except for the transient mild posterolateral thigh pain, paraesthesia, and muscle weakness. She was administered pain relievers, vitamin B complex supplements, and exercise rehabilitation for 3 weeks. After medication and physiotherapy, there was no pain, her muscle power was completely restored, and paraesthesia greatly improved. Considering the benign nature of the tumour and an expected good prognosis, no adjuvant treatment was administered. The follow-up period was 13 months. Abdominal ultrasound was performed every 3 months and MRI was performed at the end of follow-up. During the follow-up, the patient was well, asymptomatic, did not require any therapy and imaging, and did not show disease recurrence, which would require additional surgery. The patient provided verbal informed consent for this report. |
Our second patient was a 59-year-old Caucasian woman (86.8 kg, BMI 29.2, taking vitamin C, D, E, B6 and calcium). She experienced symptoms consistent with opiate overexposure that were reversed by naloxone after the second dose of methadone. Specific details of this adverse event, including the hospital course, patient monitoring data and medication schedule that resulted are illustrated in Figure . We designated our patient, who had experienced the adverse reaction, as 'ADR', and the doses of methadone administered before and after letrozole as dose 'C' and dose 'D' in the subsequent data analyses. Pharmacokinetic data from our ADR patient were compared in detail to those obtained from our N patient.\nNo methadone was detected in the plasma before the administration of intravenous methadone at baseline to either patient. Methadone plasma concentrations in ADR after dose D were fourfold to eightfold higher than those measured after her methadone dose C, given in the absence of letrozole (Figure ). The maximum concentration observed was 135 ng/mL after dose D, while it was 30 ng/mL after dose C. When estimated pharmacokinetic parameters in our two patients were compared, the area under the curve (AUC)0-24 h of methadone, its redistribution half-life, and its volume of distribution (Vd) were remarkably different after dose D in our ADR patient when compared to doses A, B and C (Table ). These data indicate that there was a fourfold to sixfold decrease in Vd in our ADR patient after dose D.\nThe first 12-hour urine volume was much lower in ADR (dose D; Table ). The concentration of urinary methadone after dose D was approximately 13-fold higher than that after dose C, and the total amount of methadone excreted was 1.4-fold greater.\nThe metabolite data from our ADR patient also indicate important differences after dose D, both in the plasma (Table ) and in the urine (Table ). The AUC0-24 h of plasma 2-ethylidene-1,5-dimethyl-3,3-diphenylpyrrolidine (EDDP; primary metabolite of methadone) and 2-ethyl-5-methyl-3,3-diphenyl-1-pyrroline (EMDP; secondary metabolite of methadone) increased approximately eightfold and approximately sevenfold respectively. The urinary concentration of EDDP increased approximately 17-fold, and the concentration of EMDP increased approximately 12-fold. The total amounts of EDDP and EMDP excreted in urine during the first 12 hours were 1.8-fold and 1.3-fold greater respectively than those after dose C (Table ).\nIn order to determine the amount of bound and free methadone and EDDP in the plasma, we selected samples collected at eight and 12 hours after the methadone dose, which is after the redistribution phase assuming a two-compartment model. In our ADR patient, the mean methadone fraction unbound (f) in these samples decreased 3.7-fold from doses C to D, while the EDDP fraction unbound decreased 3.6-fold (Table ).\nWhen plasma protein concentrations were measured in these samples, an increase in total protein of 0.8 to 1.2 g/dL (13% to 20%) was observed after dose D (Table ). Upon further analysis of the whole plasma proteome by MS and relative quantification on the basis of label-free spectra count, proteins that appeared to increase by twofold to fourfold during the adverse event (dose D) relative to samples drawn at the equivalent time before (dose C) included thrombin and its precursors, fibrinogen and its precursors, complement factor 1, retinol-binding protein 4, Shwachman-Bodian-Diamond syndrome protein, apolipoprotein A-IV, serpin peptidase inhibitor, clade C and kininogen 1 isoform 2 (data not shown).\nIn addition, serum concentrations of Na+, Cl-, HCO3-, blood urea nitrogen and creatinine in our ADR patient were similar after both doses C and D (Table ). Serum lipid profiles were also similar before and after dosing (data not shown). |
A 9-year-old female leucoderma patient presented to the stomatology department of a public hospital in Rio de Janeiro, Brazil. She complained of small nodules in the left parotid region that had developed over the course of 2 years. Her main complaint was of recurring periods of worsened symptoms characterized by the exacerbation and remission of gland volume that was possibly triggered by occasional otolaryngologic infections or unrelated to these infections. These symptoms suggest juvenile recurrent parotitis. Facial panoramic radiography revealed the presence of multiple circular radiopaque masses in the left parotid region (Fig. ). The ultrasound revealed increased volume of the left parotid, with imprecise borders, heterogeneous echotexture with hypoechoic and hyperechoic areas within it. These features were suggestive of an inflammatory process associated with calcifications in the parenchyma of the gland. CT scan revealed a dense mass in the left parotid; it was heterogeneous and included calcifications in its center (Fig. ). Because of the association between the patient’s clinical history, her clinical presentation, and the imaging findings, the possible origin of the calcified materials was questioned. There was evidence of sialoliths or dystrophic calcification associated with recurrent inflammation/infection. Sialoliths are typically symptomatic because of their association with secondary bacterial infections, which are generally treated with systemic antibiotic therapy. Spontaneous remission of bacterial sialadenitis associated with sialoliths is not expected. In addition, sialoliths generally observed as oval-shaped calcified masses or fusiforms on imaging. Because of the pediatric nature of this case, the clinical conduct selected to treat this patient was clinical follow-up and the use of imaging and functional assessments of the gland affected every 6 months or when any signs and/or symptoms appeared. After 48 months, the patient is asymptomatic, without periods of exacerbation of the condition. Recent ultrasound (Fig. ) demonstrates an improvement in the inflammatory aspect of the gland. Clinical and imaging follow-up will be maintained. |
The patient is a 42-year-old Filipino woman with SLE. Chronic symptoms include discoid and malar rash, oral ulcers, photosensitivity, and polyarthritis. She has no history of renal or central nervous symptoms but has a history of pericarditis. She also has Raynaud disease, but no other notable medical problems. She denies an exposure to tuberculosis. Her medications include hydroxychloroquine, methotrexate, azathioprine, methylprednisolone, folate, pregabalin, and nifedipine.\nAt initial presentation to an outside provider, she noted new swelling and pain in her left fifth finger and the ulnar aspect of her left hand. She had a similar episode in the right hand 1 year prior, which was associated with a lupus flare and resolved with steroid treatment. Her left hand pain and swelling were also attributed to a lupus flare at the time of presentation, and methylprednisolone dosage was increased. When her symptoms did not improve 1 month later, she presented to our orthopaedic clinic. Her examination was notable for diffuse erythema and swelling in the fifth finger, extending into the palm, as well as a small cyst on the ulnar side of her dorsal hand (Figure ). She had a fusiform digit and pain over the flexor sheath but did not have pain with passive extension or a flexed digit at rest. A magnetic resonance image was obtained, and the results were consistent with tenosynovitis (Figure ). She received a cortisone injection in the fifth finger, which resulted in only a mild decrease in swelling. One month later, she elected to proceed with synovectomy.\nIn the operating room, a Brunner-type incision was made over the fifth finger, and purulence was immediately expressed. The incision was extended to the distal finger and palm to thoroughly evaluate the extent of infection, and synovectomy, irrigation, and débridement were performed after cultures were obtained. No “rice bodies” were noted, and no purulence was found distally in the carpal tunnel. After the volar procedure was completed, a small dorsal incision was made over the cyst, and purulence was also expressed. The area was not contiguous with volar infection. The skin was loosely closed with nylon sutures, and a soft dressing was placed.\nThe intraoperative acid-fast culture grew M tuberculosis, and all other cultures were negative. Subsequent evaluation of the patient found no evidence of previous or active pulmonary tuberculosis. Because of a recent history of headaches, she underwent neurologic evaluation and brain MRI, which revealed ring-enhancing lesions consistent with tuberculoma. She had no other neurologic signs or symptoms at the time of diagnosis and required no additional treatment for these lesions. She was treated with a standard four-drug regimen, and her azathioprine was discontinued during this course. Her hand infection healed uneventfully without any sign of persistent infection (Figure ). |
A 68-year-old woman presented in the Department of Ophthalmology of Tokyo University with swelling and non-bloody discharge in her right eyelid, which had developed over several months. The right eye had been enucleated at the age of three years old, due to congenital glaucoma. She did not undergo orbital implant surgery at that time. Apart from the enucleation, the patient had an unremarkable medical history, and no family history of malignancy. She had worn the original ocular prosthesis for more than 60 years without maintenance. Since one year before presentation, the patient noticed increasing difficulty in wearing the prosthesis.\nOn examination, a firm mass was palpable beneath the right upper and lower eyelids. Cilia were present in both upper and lower right eyelids. Gadolinium-enhanced magnetic resonance imaging revealed a contrast enhanced mass measuring 38 mm × 33 mm × 36 mm in the right orbit (). A clinical work-up for systemic disease, including complete blood cell count, chemistry panel, liver enzyme panel and imaging of the chest, was negative. There was no regional lymphadenopathy, and gallium scintigraphy revealed no systemic metastasis. A histopathologic examination of transcutaneous excisional biopsies of the mass revealed lobular aggregates of atypical basaloid cells, separated by fibrovascular stroma, and an inflammatory infiltration. The atypical cells had abundant clear multi-vacuolated cytoplasm, and oval nuclei with discernible nucleoli (). Tumor cells were immunoreactive for adipophilin and perilipin, which are useful markers for sebaceous gland carcinoma (). Esentreration of the right orbit was planned by performing multiple map biopsies, including the upper and lower eyelid skin, tarsus, orbicular muscle and skin, and deep tissues below the eyebrow. Within a month, the patient underwent orbital exenteration to excise the entire mass. The tumor had well defined margins and was enclosed by a capsule (). No spread to other sites were observed. Histopathologic examination of the excised tumor demonstrated a poorly differentiated sebaceous gland carcinoma with negative margins. The eye socket was reconstructed using a free rectus abdominal muscle flap and mucosal graft. The patient recovered well, and was able to wear a prosthesis after surgery. There was no evidence of recurrence of metastasis ten months after orbital exenteration. |
Patient M.F., male, aged 56 was admitted with burns of I, II and small areas of III degree on ~75% TBSA. He had the following co-morbidities: a minor liver dysfunction, due to regular alcohol consumption; untreated hypertension; type II diabetes, under treatment with Metformin; a urethral stricture, which had been surgically treated. He was a very active man, due to his job – a firefighter-. The accident happened while he was preparing some kind of alcoholic beverage at home in a special boiler, so he was splashed by the hot boiling fluid, which resulted in the injuries, which were described below. He was brought to our unit after a couple of hours from the accident displaying circular burns on both lower extremities (from the lower half of the thighs to the foot), and insular ones on his trunk, his right arm and both his hands (,). No escharotomies were performed, as they were not necessary, but the surgical debridement of the lesions was immediately performed.\nAfter removing the blisters and the dirt, there were areas of red-pink and white dermis that was moist in some regions and dry in others. The image was that of a “chess-board”, due to the color alternation, suggesting the alternation between different degrees of burn lesions. The patient was kept, treated, and monitored in the intensive care unit for ~ 2 weeks. At some point he needed intubation and ventilator support, but his local evolution was very good. In the first couple of days, while the burn wounds were very exudative, an ointment based on zinc oxide (that was prepared in our unit) was applied (,).\nAfter the first week, when his general state got better, silver sheets (Acticoat) were applied on his trunk and on his right upper extremity, where the lesions were deeper and seemed to heal slower than in the other above-mentioned areas. They were kept in place for 3-5 days, depending on the progress of the area. In the meantime, the patient got better and better and was transferred to the regular burn department.\nAt that point, the burns on the patient’s lower extremities were already healed, and the ones on his trunk and upper extremity were healed ~60% but he suffered from a mental blockage and he refused to get up and walk or do anything else by himself, he needed help even during the meals (,).\nThe burn injuries on his trunk were slowly healing. Some difficulties were encountered, as he refused to comply with our indication of not lying only on the back, so he developed small eschars on the heels, in spite of the special mattress and of the staff turning him on the sides and on the belly, as indicated in such cases. Once more silver dressings were applied, that time Atrauman Ag. The lesions were not exudative anymore, but still a bit wet and the patient’s body healing reserves were quite depleted, in spite of the administered vitamins and hyper-protein diet, so the epithelization process needed to be boosted. In addition, we thought that a mesh-like dressing, as the one we used, would help. After another week of daily psychological therapy sessions with the psychologist in our unit, he started walking by himself: it was a miracle for us to see him like that. He was discharged completely healed, with still evolving scars that did not show any sign of turning pathological. The small eschars on his heels were slowly healing, too. He was recommended to use scar gel (with onion extract and another one that was silicone based) ().\nAfter 3 months from the discharge, the patient’s scars showed signs of turning hypertrophic/ keloid in some regions: the right scapular area, the right flank, some dot-like areas on the back and on the anterior thorax, also on the right arm ().\nHe started wearing special garments - vest and bandeau on the right arm for pressure therapy. The performance of Corticoid (Kenalog A) injections were also started in the pathological scars. At that point, almost after a year they were slowly turning softer and decreasing their projection. However, we are still performing corticoid injections – it is the sixth monthly session and there are significant signs of improvement as far as the itching and the softness of the scars are concerned, but still relatively minor changes in their size and projection. We plan to pursue the injections until the scars have acceptable appearance and cause minimum discomfort to the patient. He himself declared that he is not very interested in the esthetic appearance, but more in the functional impact of the scars on his lifestyle. |
A 67-year-old female was admitted with abdominal distension and rapidly developing ascites. Ultrasound examination and CT scan of the abdomen and pelvis showed extensive abdominopelvic ascites of unknown cause. No intra-abdominal mass or pelvic abnormality was detected. Tumour marker CA125 was raised, 2000 KU/l (normal—less than 35 KU/l) but serum CEA levels were within normal limits. Clinically, ovarian cancer was suspected, however paracentesis demonstrated benign peritoneal effusion. A transvaginal scan showed solid/cystic mass in the pouch of douglas 8 × 6 × 4 cm. She underwent laparotomy which showed copious amount of benign ascitic fluid and a left ovarian mass. The possibility of a dermoid cyst was considered. A total abdominal hysterectomy with bilateral salpingo-oopherectomy was performed along with omental biopsy and peritoneal washing.\nOn gross pathological examination, there was a left ovarian mass measuring 10 × 7 × 3.5 cm. The external surface of the cyst was mainly smooth with a small area of yellow/green discolouration. Cut section of the cyst showed haemorrhagic solid mass. Histology of the ovarian tumour showed thyroid tissue characteristic of struma ovarii (). However, the thyroid tissue showed focal worrying features in the form of small and large papillae (Figures and ) lined by cells showing optically clear nuclei with thickened nuclear membrane and overlapping nuclei (). Scattered psammoma bodies were also seen (). The case was sent for second opinion. The final report confirmed it to be a struma ovarii with thyroid tissue showing neoplastic transformation into classical papillary thyroid carcinoma. The exact proportion and size of the carcinoma was difficult to estimate due to the smooth blending of the benign and malignant components. However, the overall malignant component was small measuring approximately 5 mm. Immunohistochemistry and molecular studies were not performed at our centre or by the histopathologist providing second opinion as the features were unequivocal of classical papillary thyroid carcinoma. The uterus, right ovary and the Fallopian tubes were unremarkable. The peritoneal washing and omental biopsy were negative for malignancy. Postoperative thyroid function test was within normal limits. Clinically there was no evidence of metastasis. The patient was staged as FIGO stage Ia malignant struma ovarii and no other adjuvant treatment was given. It was decided to keep her on follow-up for the next five years. Presently, after a two year follow-up, she is well with no evidence of recurrence. |
A 14-year-old South Asian boy from rural Bengal (India), born of a second degree consanguineous marriage, with normal birth and development history, presented with abnormal brief jerky movements involving his trunk and limbs, with recurrent falls for 10 months. The jerks were neither stimulus sensitive nor present during sleep. No loss of consciousness was reported to occur with these jerky movements. Recurrent convulsions involving the left half of his body, without impairment of awareness, was present for 8 months. It was followed by insidious onset of mild weakness of the left half of his body for 7 months. Subsequently he suffered progressive decline in his general ability to maintain average daily activity independently for 5 months. He had to discontinue schooling because of his failing cognitive functions. For 2 months prior to presenting to us, he developed rapid dance-like movements involving all four limbs that flowed from one muscle to the other in a more or less continuous fashion. Occasionally it would become somewhat flinging particularly in his upper limbs. There was no history of similar illness in the family. He received all the scheduled vaccines as was stated by his mother.\nThe height of the boy was 150 cm and he did not have any dysmorphic facial features. A clinical examination revealed generalized choreiform movements as the most obvious finding. These movements intermittently became flinging in nature, resembling ballism. Generalized myoclonic jerks were seen embedded inside the flurry of chorea-ballism. When he was asked to protrude his tongue, besides motor impersistence, oromandibular dystonia was also found. He had severe dysarthria with apparently preserved comprehension. A limited cognitive assessment revealed reduced attention span as well as short-term memory impairment. Rigidity was obvious in all four limbs along with dystonia in both lower limbs. Weakness in the left half of his body along with brisk reflexes and extensor plantar on left side was also detected on motor system evaluation.\nRoutine laboratory parameters revealed impaired fasting glucose (120 mg/dl), mildly raised liver enzymes and creatine phosphokinase (CPK) level of 820 IU/L. Other blood and urine parameters were within normal limits. Screening investigation for Wilson’s disease, storage disorders, and metabolic disorders were all negative. A routine cerebrospinal fluid (CSF) study was unremarkable and anti-measles antibody was negative. Anti-nuclear antibody in blood was also negative. His serum level of lactate was 36 mg/dl (2–19 mg/dl) while CSF lactate was 42 mg/dl. Shortening of PR interval (0.10 second) was found in electrocardiography. Two-dimensional echocardiography was devoid of any abnormality. Serial brain imaging was done at different centers throughout the course of his illness. On studying his MRI brain images sequentially, a relapsing remitting pattern of lesions was detected. On T2/fluid-attenuated inversion recovery sequence (FLAIR) there were hyperintense lesions that mainly involved subcortical white matter in frontoparietal areas (Fig. ). An area of diffusion restriction was found in the right capsule-ganglionic region (Fig. ) that temporally coincided with the onset of left hemiconvulsions and hemiparesis. Magnetic resonance spectroscopy (MRS), done at our center, showed the presence of lactate peak in brain lesions. Brainstem auditory response revealed bilateral prolonged latency. Electromyography (EMG) showed short duration low-amplitude polyphasic motor unit action potential which was suggestive of myopathic pattern. Spike-wave discharges were observed arising from bilateral frontal areas on electroencephalography (Fig. ). A muscle biopsy, which was done from left vastus lateralis, revealed ragged red fibers (Fig. ), suggestive of mitochondrial failure and deposition of abnormal mitochondria below the plasma membrane of muscle fibers.\nAccording to the clinical criteria, MELAS syndrome was the most probable diagnosis in our case and we needed to confirm the diagnosis. As a facility for analysis of respiratory chain enzymes in the muscle was not available, we decided to search for underlying genetic abnormality in mtDNA. A polymerase chain reaction (PCR) method was employed for this purpose. Amplification of DNA in whole blood sample of our patient was performed for detection of mutations 3243A>G, 3271T>C, and 3251A>G in mitochondrial tRNA leucine 1(MT-TL1), by using appropriate wild type and mutant type specific primers for each and a common reverse primer for all. Genetic analysis result was as following: A>G point mutation at position 3251 of MT-TL1 gene of the mtDNA with heteroplasmy of 70%.\nAfter reaching the diagnosis, valproate was taken off and lamotrigine was introduced. He was put on co-enzyme Q supplement and haloperidol for abnormal movements. Six months into follow-up his seizures and abnormal movements were controlled significantly with slight improvement of cognitive abilities. |
A 65 yr old female presented to the Ear Nose and Throat [ENT] clinic of our institution with a one-month history of a lump in the right neck. On examination, a mobile lump was palpable in the angle of the mandible/upper cervical region. A flexible endoscopy did not reveal any mucosal lesion in the posterior nasal space, oropharynx, hypo pharynx or in the larynx. Clinically a lymph node mass was considered as a possible diagnosis. Fine needle aspiration [FNA] and Magnetic Resonance Imaging [MRI] were then performed.\nMRI showed a well-defined oval shaped mass measuring 3 × 2 cm just inferior to the right parotid gland. The epicenter of the lesion was in the inter muscular plane, medial to the sternocleidomastoid muscle but separate from it. It was heterogeneous on both T1 & T2 W images with a well defined margin (Fig & 2). Following contrast administration, there was moderate heterogeneous enhancement (Fig ) In view of the location of the lesion, a differential diagnosis of lymph nodal mass & Spinal Accessory nerve Schwannoma were considered.\nFNA of the lesion showed a mix of bland epithelial cell, clusters of spindle cells and myxoid matrix, which was typical of a pleomorphic adenoma.\nA decision to perform an excision biopsy was subsequently made. At surgery, the tumor was found to be a pedunculated mass arising from the inferior aspect of the tail of the parotid gland. It was closely related and superficial to the spinal accessory nerve but separate from it. The mass was excised completely incorporating a limited cuff of macroscopically normal parotid tissue, taking care not to injure the inferior branches of the facial nerve.\nHistopathology of the excised specimen showed uniform proliferation of epithelial elements within a patially myxo-chondroid stroma, again, consistent with a diagnosis of a pleomorphic adenoma. There was no definite salivary gland tissue within the specimen. |
A 29-year-old Caucasian man from East province of Saudi Arabia who’s known for homozygous sickle cell anemia admitted to the hospital with acute left scrotal pain and swelling for 1 week that did not respond to antibiotic. The patient had been in his usual health until 1 week before this admission when pain in the left testis developed. The pain started suddenly, progressive and was associated with intermittent fever (38.2) and progressive enlargement of the left scrotum. There was no history of trauma or lower urinary tract infections symptoms or hematuria or urethral discharge. 1 week before this admission, he presented to another health center for evaluation where he was diagnosed with urinary tract infection and managed as outpatient with a prescription for 7-day course of amoxicillin/clavulanate potassium. The SCA course of this patient was remarkable for recurrent vaso-occlusive crisis and splenectomy at the age of 13 years, but there were no complications such as acute chest syndrome, cerebrovascular accidents, or genitourinary complications. He is not known to have any other medical problems, and he is not on any medications currently apart from intermittent use of analgesics. His vaccinations are up to date. There is additional history for discontinuation of hydroxyurea 4 months ago for improving fertility. He was married and worked as a pharmacist. He did not smoke, drink alcohol, use illicit drugs, or any kind of herbs. He had a family history of diabetes mellitus and hypertension. On examination, the temperature was 37.1°C and pulse 100 beats/min; the other vital signs were normal. The height of the patient was 163 cm and his weight was 55 kg. Swollen, red, and tender left scrotum was present. The examination was otherwise normal. Ultrasound scrotum showed heterogeneous hypoechoic left testicle with no blood flow [].\nLaboratory investigations showed white blood cells 40 (3.5–10.5 billion cells/L), hemoglobin 80 (135–170 g/L), mean corpuscular volume 80 (80–96 fl), platelets 1140 (150–450 billion/L), and reticulocytes 10.7%. Hemoglobin electrophoresis came out with hemoglobin S 79% and hemoglobin F 4.6%. Urine analysis was unremarkable. Conservative strategy has been followed with this patient. He received antibiotics (piperacillin/tazobactam 4.5 g IV q6 h), parenteral hydration, morphine, high-dose hydroxyurea (1000 mg oral daily), and aspirin (81 mg oral daily). There was no significant improvement of patient’s symptoms for 7 days. Repeated ultrasound confirmed aforementioned findings. The urologist decided to proceed with the left orchiectomy. Pathology resulted in extensive infarcted testicular tissue with marked acute inflammatory changes and focal hemorrhage of the testis. |
A 25-year-old man was referred to our hospital in 2014 for a suspected right bronchogenic cyst. He underwent a trans-esophageal biopsy to confirm the diagnosis. After a few days, he started to complain of fever and acute chest pain. A contrast-enhanced CT scan revealed a massive pleural effusion in the right hemithorax with complete atelectasis of the ipsilateral lung (Fig. ).\nA right thoracoscopy was performed, in order to achieve a complete debridement and drainage. During the procedure, no esophageal perforation was seen. The following clinical course was normal, so the patient was discharged on postoperative day 15.\nIn December 2019, the patient was referred to our Emergency Department because of the sudden onset of fever (39 °C) with acute chest pain. He underwent a CT-scan which revealed a suspected esophageal perforation with acute mediastinitis and right pleural effusion. A subsequent gastroscopy showed, at 35 cm from dental arch, an erosion of the esophageal wall 1,5-cm long and a 2-mm perforation with a small leak of purulent liquid. Furthermore, an endoscopic ultrasound revealed the presence of the bronchogenic cyst just outside the erosive area. Thus, a right thoracotomy with intraoperative endoscopy was performed. An esophageal perforation at the level of the cyst was found, so the right hemithorax was cleaned, the cyst was opened in order to better understand its margin, and then resected, while the esophageal wall was closed with two interrupted, absorbable stitches. A 24-Ch drain was left in place. After 2 days, salivary material appeared into the drain, so the patient underwent an EGDS, which revealed a 7-mm hole of the esophageal wall at the level of the previous suture (Fig. a). Thus, an E-Vac therapy was placed directly into the perforation (Fig. b) only during the first placement, with the aim of cleaning the mediastinum and healing the esophageal wall, which was locally compromised by the abscess. Patient’s nutrition was guaranteed by a nasojejunal feeding tube.\nThe duration of the endoscopic treatment was 17 days, the sponge was changed 5 times (Fig. c, d). After the removal of the last sponge, the patient started an oral diet without complications. One last barium swallow study showed no leak, so the patient was discharged home. Pathology confirmed the diagnosis of bronchogenic cyst. At a 3-month follow-up, there was no sign of recurrence. |
A 46-year-old male with a history of severe developmental delay, hydrocephalus, and seizure disorder presented to the hospital with blunt head trauma after a ground level fall. Work-up revealed bilateral acute subdural hematomas for which an external ventricular drain was placed. Despite intensive care management, the patient deteriorated to brain death. He was subsequently evaluated for organ donation. Abdominal computerized tomography (CT) scan () revealed an “elongated structure with metallic components in the upper portion of the IVC that extends into the right atrium.” There was no medical history of a prior procedure, or symptoms, to explain the incidental finding. The radiologist's interpretation and presumptive diagnosis were a retained atrial pacing wire.\nHe subsequently underwent procurement for organ donation after brain death. At the time of cross-clamp, the previously identified foreign body was transected when the right atrium was incised for exsanguination. During the back-table dissection, it was apparent that the foreign body had eroded into the posterior wall of the IVC, extending down the retrohepatic IVC (Figures and ). It also created a calcified reaction at the junction of the suprahepatic IVC and right atrium, adjacent to the left hepatic vein (LHV) and middle hepatic vein (MHV). We removed the foreign body () and performed a venoplasty () of the posterior wall of the IVC and of the common wall of the LHV and MVH, so that the outflow of the LHV and MHV was not compromised after transplant. We discovered that the foreign body was, most likely, a fractured CVC due to the overall appearance and interval markings.\nThe liver recipient was a 65-year-old woman with cirrhosis due to alcohol abuse; her Na-MELD score was 40 at the time of transplant. She underwent caval-sparing total hepatectomy and deceased donor liver transplantation via piggyback technique: the donor suprahepatic IVC was anastomosed to a common orifice of the recipient's right and middle hepatic veins. We did not alter our immunosuppressive therapy or prophylactic antibiotic regimen. Additionally, we did not initiate any anticoagulants or antiplatelet agents beyond our standard postoperative protocol. Postoperative imaging showed normal velocities and waveforms on ultrasound () and unremarkable appearance on axial CT () of the hepatic vein anastomosis. The patient otherwise had an uneventful postoperative course and has had stable allograft function with no venous outflow issues for >8 months after transplant. There were no reported complications in the other organ recipients. |
A two-year-old Tanzanian girl presented with a 1-week history of fevers up to 38.5°C, flaccid lower limb paralysis, and loss of sphincter control. Prior to admission, neurological development had been normal with good sphincter control. Her mother had noticed a small reddish pimple on her back since birth, which had never discharged fluid or grown in size.\nOn examination, the child was febrile with normal level of consciousness, meeting cognitive milestones, normal neurological examination above the waist, and flaccid paralysis of the legs. There appeared to be decreased sensation roughly from the umbilicus down. No structural abnormalities of the lower extremities were appreciated: leg lengths were equal, without atrophy or foot deformity. A soft lipomatous midline swelling of 2 by 2 cm was observed at a low-thoracic level, which had a minute central pore without discharge. Spinous processes could not be palpated in the region surrounding the lesion. Mild thoracolumbar scoliosis and mildly increased lumbar lordosis were observed. However, the child was unable to sit unassisted. Over the course of the admission, she had a single generalised seizure with quick recovery of consciousness and transiently complained of a painful feeling in her right arm. Due to the young age, the postulated meningitis with the present sinus, and seizures in the course of admission to the hospital, we assumed that the pain which the child consistently indicated in the right arm may have a neuropathic origin, for which we prescribed low-dosage amitriptyline.\nThe working diagnosis was superinfection of a dermal sinus from a spina bifida occulta defect in a previously neurologically normal child, leading to spinal cord and/or nerve root compromise and bacterial meningitis. The lower extremity paralysis and apparent sensory level were evidence of spinal cord and/or nerve root compromise, either by direct myelitis/polyradiculitis or by compression from epidural abscess formation. The episode of seizures and the unilateral cervical radiculitis was evidence of a component of meningitis. Alternative diagnoses on the differential included: common bacterial meningitis with secondary involvement of the spinal cord, an inflammatory or infectious polyradiculitis, or thoracic spine Pott's disease with secondary paraplegia.\nA lumbar puncture could not be performed because of the midline lumbar defect and the suspected infected dermal sinus. Initial lumbar X-ray (limited by underexposure) showed no evidence of vertebral displacement or collapse. An ultrasound examination of the swelling identified a superficial cyst, without clear connection to the spinal column. A Full Blood Picture (FBP) demonstrated an elevated white cell count with a predominance of lymphocytes (see ).\nThe child was treated with broad-spectrum intravenous antibiotics, ceftriaxone, cloxacillin, and metronidazole, to cover for aerobic and anaerobic causes of bacterial meningitis and abscess. Unfortunately, blood cultures had not been done prior to starting empirical antibiotic therapy. Five weeks after admission, the child and her mother absconded from the hospital due to inability to afford the cost of care. The family was also unable to afford a spinal CT scan (75 American Dollars) during admission.\nThe child and her mother returned to the paediatric neurology clinic for a follow-up examination 10 months later, having managed to afford CT spine. The two-centimetre midline nodule had subsided; only a two-millimetre papule with a small central invagination remained in its place. Over two years, the child's legs had become spastic, left more than right, and she developed persistent urinary and faecal incontinence. She maintained a sense of bladder fullness which facilitated bladder catheterisation. Progressive scoliosis with marked lumbar lordosis was noted (see ).\nThe CT scan demonstrated multilevel spinal dysraphism and deformity above and below the level of the now-resolved swelling. Axial CT sections revealed a dorsal dermal sinus at the level of T12 connecting the epidermis to the epidural space. (See Figures and for the original axial and longitudinal sections as well as an enhanced representation of the abnormality.)\nThe dermal sinus caused recurrent bouts of fever with purulent discharge from the lesion. She was enrolled in a NGO-funded surgery program performing back closure operations for newborns with spina bifida. Since the surgical intervention was a free treatment option offered to the mother in the framework of a spina bifida NGO care program, extensive surgical reports were not made available for review. Following the surgery, the local discharge and bouts of fever resolved and, subsequently, her general condition improved. However, her neurological deficits remained unchanged. |
A 31-year-old woman with a history of left common iliac vein thrombosis presented with symptomatic lower abdominal and left groin superficial varicosities associated with itching, swelling, and discomfort. Vital signs revealed a blood pressure of 114/80 mmHg and pulse of 72 beats/minute. Physical examination confirmed the presence of large tortuous varicosities at the lower abdomen and left groin (). The patient had previously been diagnosed with left common iliac vein thrombosis during her first pregnancy, 10 years earlier, that was only managed with enoxaparin injections. Over the next few years, she developed lower abdominal and left groin varicosities that worsened significantly during her second and third pregnancies without confirmed recurrent deep vein thrombosis (DVT). The patient denied leg swelling prior to the DVT event. She also denied any history of abdominal trauma, other thromboembolic events, or family history of vascular anomalies. Computed tomography (CT) venography revealed compression of the left common iliac vein by the right common iliac artery without evidence of acute thrombosis, indicating a diagnosis of May-Thurner syndrome- (MTS-) related anatomy ().\nCatheter-based venography with hemodynamic pressure measurements confirmed May-Thurner anatomy with sequelae of chronic DVT in the left iliac vein and cross-pelvic drainage via pelvic and abdominal wall varices (). Successful recanalization was performed using percutaneous transluminal angioplasty with stenting of the left common iliac vein (20 mm x 55 mm Wallstent) and left external iliac vein (14 mm x 60 mm Protege). The patient was managed with apixaban (5 mg twice daily) for three months and clopidogrel (75 mg daily) for one month that was changed to aspirin (81 mg daily) in the long term. A 3-month follow-up CT venogram indicated a patent left iliac vein stent. However, the patient continued to present with painful, though slightly improved, lower abdominal and left groin varicosities.\nGiven these persisting symptoms, we performed successful stab phlebectomy of the large superficial abdominal varicosity. We also treated the deeper feeding branch and groin varicosities with ultrasound-guided sclerotherapy using a sclerosing foam (two injections of 1 cc of 3% sotradecol mixed with 2 cc of room air). The procedure resulted in complete resolution of the symptomatic lower abdominal and left groin varicosities (). The patient did very well at 6- and 12-month follow-up visits while on aspirin, and a repeat CT venogram indicated a patent left iliac vein stent. |
We received approval from the Institutional Review Board of Jilin University First Hospital, Changchun, Jilin, China for the publication of this report; the patient also provided informed consent for the publication of this case report.\nA 30-year-old man accidentally discovered a painless, quail egg-sized, mass in the lateral upper quadrant of the right breast, next to the nipple in February 2018. The patient did not initially seek medical consultation. In March 2018, the patient detected a painless enlargement of the mass and sought medical consultation at his local hospital. A breast ultrasonography examination revealed a mass, 23.7 mm × 7.5 mm in diameter (Fig. ). No abnormality was identified in the left breast. Mammography revealed a well-circumscribed mass of mixed density in the right breast, with no evidence of invasion of the ipsilateral axillary nodes (Fig. ). The patient refused to undergo fine needle aspiration cytology examination. The provisional clinical diagnosis was a lipomyoma or adenoma fibrosum.\nThe patient was referred to our hospital for further assessment, surgical management, and treatment. The patient's history was reviewed. We noted the following characteristics: current smoker (with a long history of smoking); no alcohol consumption; no history of trauma to the region; no history of prior surgery or radiation exposure of the region; and no personal or significant family history of cancer. With no important history identified, the clinical diagnosis of a hamartoma was established. The physical examination revealed a soft, mobile, painless mass located in the right breast, measuring approximately 2 cm in diameter, with no abnormality identified in the left breast. On March 27, 2018, the patient underwent surgical resection of the mass at our hospital. Gross examination of the resected mass revealed an oval, well-defined, and encapsulated mass, with gray-yellow coloring and a smooth margin (Fig. ). The nodule was very soft and had a fibrotic envelope. Pathological examination confirmed the diagnosis of mammary hamartoma (Fig. ). The patient recovered well after surgery, without complications and the need for further interventions. The patient was discharged from the hospital on postoperative day 7, April 4, 2018. The patient was followed-up every 6 months, with no sign of recurrence over a period of observation of 1 year. |
A 45-year-old Iranian woman was referred to the private clinic in the city of Hamadan for implant consultation. Her past medical history was not notable, and there was no evidence of systemic disease. She had no history of trauma to the mandible. In the extra-oral examination, no abnormal symptoms were observed. Intra-oral examination revealed normal oral mucosa, the absence of soft tissue expansion, and teeth of a normal color. Periodontal tissues were normal. All of the teeth were asymptomatic, with no pain or tenderness on percussion or palpation. The involved teeth were vital in an electric stimulation test.\nFor assessment before implant insertion, CBCT had been ordered. During evaluation of the implant insertion areas on Promax3D CBCT (Planmeca OY, Helsinki, Finland), a radiolucent-radiopaque mixed lesion located on the apices of the lower incisors was observed. On the axial, sagittal, and coronal CBCT images, the extension of the lesion was observed from the mesial side of the right mandibular lateral incisor to the distal side of the left mandibular lateral incisor. It was a multifocal lesion in which solitary lesions were reached together and made a larger lesion. The total dimension of the lesion was about 16.6 mm in the mesiodistal direction and 6.9 mm in the longest superior-inferior direction. On the panoramic reconstructed CBCT image, the lesion associated with the left mandibular lateral incisor was radiolucent, whereas the lesion on the apex of the left central incisor was mixed radiolucent-radiopaque and the lesion associated with the right central incisor was radiopaque with a radiolucent rim around the lesion of this tooth ().\nBy using the CBCT, the state of the lesion relative to the buccal and lingual cortical plates could be assessed, which might not be possible on the conventional radiographs. On the axial image, two expansion and thinning areas of the buccal cortex were revealed. One of them was located at the mesial side of the right canine and the other between the left central and lateral incisors (). On the axial and cross-sectional images, the discontinuity of the lingual cortex was found at the area between the two central incisors on several consecutive sectional images (). This cortical discontinuity was more obvious on the three-dimensional (3D) CBCT images (). However, it should be considered that the cortical bone could be seen to have destruction on 3D images, even though it would have been thin without discontinuity on the cross-sectional images.\nOn the digital periapical radiograph that was taken for further follow-up, a typical feature of PCOD was observed (). There was no root resorption or tooth displacement. The lamina dura surrounding the apical areas of the involved teeth was lost. Periodontal ligament space widening was found, especially around the root of the left lateral incisor.\nBased on the patient clinical and radiographic findings, a diagnosis of multifocal periapical cemento-osseous dysplasia was made. No treatment was considered. The only recommendation was periodic radiographic follow-up. |
A previously healthy 9-year-old female presented to her primary care physician after developing left-sided facial weakness. She was referred for magnetic resonance imaging (MRI) which showed T2/FLAIR hyperintensity centered within and expanding the pons. The initial physical exam revealed several neurological abnormalities including a left 6th nerve palsy with bilateral nystagmus as well as an incomplete left facial palsy and left-sided dysmetria.\nThe patient was enrolled on a Children's Oncology Group trial with vorinostat and focal radiation therapy; the post-radiation MRI revealed improvement in the pontine lesion with decreased mass effect. However, seven months later the pontine glioma increased in size and two new metastases were simultaneously noted: 1) a large lesion of the septum pellucidum involving the frontal horns of the lateral ventricles and the undersurface of the anterior corpus callosum (labeled ‘SP’ metastatic point), and 2) a left posterior hippocampal lesion (labeled ‘PH’ lesion) (Figure , ).\nThe patient was next enrolled in a Pediatric Brain Tumor Consortium trial and received two doses of a telomerase inhibitor which was discontinued for reasons unrelated to the patient's clinical course, and she was noted to have subsequent progression of both the primary and metastatic lesions. After the patient died, an autopsy was performed within 10 hours of death, and fresh-frozen and formalin fixed tissue was obtained from the primary site and the disseminated lesions as well as the grossly normal brain.\nCoronal sections of the post-mortem brain showed the SP tumor: a large, ill-defined metastatic solid mass centered at the septum pellucidum and involving the corpus callosum, right internal capsule, and the frontal horns of the lateral ventricles. Autopsy also revealed the PH lesion: a smaller lesion centered in the left posterior hippocampus. The basis pontis was significantly expanded, while the cerebellum was grossly normal.\nHistological analysis was undertaken of multiple sites in both the primary and the two metastatic tumor sites, showing that the SP tumor, much like the primary site, was overall best classified as high-grade (WHO Grade IV, Figure ) while the PH tumor was lower grade; nevertheless, all sites of disease displayed focal necrosis and vascular proliferation. The metastatic tumors had scattered areas of small, round blue cells reminiscent of PNET, but were strongly diffusely positive for GFAP, negative for synaptophysin and were overall best classified as glioma. Metastatic disease in the SP tumor had an increased Ki67 proliferation index compared to the brainstem lesion, 30% vs. 8%, respectively. All tumor sites displayed positive staining for the histone 3 K27M (H3K27M) mutation with less evident histological staining for wild type histone 3 trimethylation (H3K27me3) (Figure ). The tumors were also positive for CD45 representing various degrees of infiltrating resident microglia and macrophages (Figure ).\nWe extracted mRNA from six brain locations to attempt to differentiate metastatic from primary tumor based on molecular signature, including three locations from the brainstem tumor and 3 sites from the SP tumor (right and left ventricular portions as well as the right frontal portion). No mRNA could be extracted from the PH lesion due to fixation issues. mRNA profiling was completed using the NanoString platform (Cancer Panel) and the differential mRNA expression pattern between the primary brainstem and metastatic tumors was assessed using Partek Genomic Suite software. mRNA profiles of different sites of the large SP tumor were variably similar to the brainstem tumor (Figure ). Ingenuity pathway analysis revealed that p53 signaling, cell cycle regulation, DNA damage response, growth arrest and DNA damage-inducible 45 (GADD45), and ATM signaling were common tumorigenic pathways between primary and all sites of the SP tumor. We identified differentially expressed mRNA species between the primary tumor compared to the ventricular portion (twenty-two species) or the frontal portion (eight species) of the metastatic SP tumor (fold change > 1.5; < -1.5; p < 0.05) (Table ). For example, expression of the fibroblast growth factor receptor, FGFR3, is up regulated in the brainstem compared to metastatic tumor samples. However, the overall mRNA profiles of these differentially expressed genes primarily exhibit dysfunction of cell cycle regulatory pathways (Table ). Given the complexities of histological staining for tumor assessment, these identified mRNA profiles may have clinical relevance in rapid identification of potential therapeutic targets. |
Following a motor vehicle accident, a 38 year-old male patient presented at the emergency room of our hospital. He was alert and in stable condition with a GCS-score of 15. On clinical examination the patient showed mild neck tenderness to palpation as well as a slightly restricted range of motion. No sensomotor deficits of the neck or arms were present. The patient commented that he had been suffering from similar neck pain for many years prior to the accident.\nAccording to Canadian C-Spine rules and due to the mechanism of the accident radiography of the cervical spine was indicated. Antero-posterior and lateral radiographs as well as 45° oblique views of the cervical spine were taken as a first imaging study (Figure ). Radiographs showed slightly incongruent articular pillars of C5 and an enlarged, elongated right C4-C5 neuroforamen with absence of the right C5 pedicle. The opposite lamina projected through this space and the articular pillar was dorsally displaced. In addition, a vertical gap in the midline of the C5 arch was seen on the antero-posterior radiograph. No anterolisthesis of a vertebral body was detected. Independently from each other, both the emergency medicine physician as well as the radiology resident on-call suspected a fracture of the right pedicle and median arch of C5. The patient was therefore scheduled for an emergency operation.\nAccording to established guidelines at our hospital, computed tomography (CT) with multiplanar reconstructions in the sagittal and coronal plane was performed preoperatively. It revealed absence of the right C5 pedicle with dysplasia of the ipsilateral transverse process and spina bifida occulta at the same level, dorsal displacement of the articular pillar, reversal of the ipsilateral facet articulation with the supra-adjacent vertebra, as well as hypoplasia of the pillar of the supra-adjacent and hyperplasia of the pillar of the infra-adjacent vertebra (Figure , ). There were also some degenerative changes at the level C4-C5 with formation of a subchondral bone cyst in the body of C4 (Figure ). No anterolisthesis of a vertebral body, fracture or hematoma were detected. Based on all CT imaging findings, diagnosis of a congential ACSP with associated osseous spinal abnormalities was established. The patient was discharged from the emergency room without surgical intervention.\nHe was informed and gave informed consent that data concerning his case would be submitted for publication. |
A 51-year-old man was admitted to the hospital for treatment of benign prostatic hyperplasia (BPH). The patient's anamnesis was negative for allergic events. Before hospitalization he was being treated with alfuzosin, which belongs to a group of medications known as alpha-1A-receptor antagonists used to treat the symptoms of enlarged prostate and BPH. On admission to the hospital alfuzosin treatment was suspended and the patient underwent transurethral resection of the prostate under epidural anesthesia, followed by post-surgical administration of antibiotics (modivid) and lactated Ringer's solution. Twenty-four hours after surgery, routine prophylaxis for stress ulcer (one phial of Zantac® 50 mg, intravenous, in normal saline solution) was prescribed. Within minutes of the injection of ranitidine, the patient developed a combination of wheezing, dyspnea and hypotension followed by loss of consciousness. Despite intensive resuscitation attempts, no cardiac activity reappeared and death was certified 30 minutes later. As the circumstances of death appeared suspicious to the treating emergency physician, a forensic investigation was initiated and the public prosecutor ordered a forensic necropsy.\nThe autopsy revealed pulmonary congestion with widespread upper airway edema, the presence of petechial hemorrhages and brain swelling with diffuse petechial hemorrhages. There was no evidence of recent myocardial infarction or other structural heart diseases. The rest of the organs were unremarkable. Histological sections confirmed the presence of widespread hypolaryngeal and pharyngeal mucosal and submucosal edema with inflammatory cells and an abundance of mast cells (Figure and ). Testing for specific IgE antibodies and mast cell tryptase was not performed because of post-mortem degradation of the serum.\nToxicological analyses on blood performed using a gas chromatography-mass spectrometry technique revealed the presence of ranitidine at less than 10 ng/ml (limit of quantitation); see Figure . No other drugs were found. Death was attributed to anaphylactic shock due to an adverse reaction caused by intravenous injection of ranitidine, suggestive of a pathogenic mechanism of immediate-type hypersensitivity reaction type I, according to the Gell and Coombs Classification System. |
A 62-year-old female patient who is heavy smoker presented with a burning sensation and discomfort in her left breast that has been recurring over a month prior to admission to the hospital. No fever, chills, or any other symptoms were described. She reported a past medical history of hypertension and a surgical history of hemorrhoidectomy, dilation and curettage surgery, colonoscopy, and gastroscopy.\nPhysical examination revealed a palpable left breast mass (measuring approximately 3 × 3 cm) in the upper quadrant with no overlying skin changes. The right breast exam was normal. No palpable locoregional lymphadenopathy (axilla and supraclavicular lymph nodes) was noticed. Routine blood tests (complete blood count with differential, electrolytes, prothrombin time, partial prothrombin time, and international normalized ratio), chest X-ray, and electrocardiogram (ECG) were all normal.\nMagnetic resonance imaging (MRI) of the left breast showed an ill-defined deep retroareolar spiculate lesion extending over 3 × 1.5 cm revealing early enhancement peak with associated architectural distortion. There were no axillary lymph nodes or abnormal bone signal intensity. No cutaneous thickening or retraction was seen. Findings were suggestive of BIRADS type IV lesion ().\nAn excisional biopsy was performed and revealed breast tissue with extensive lymphocytic infiltrate intermixed with neoplastic epithelial cells (). Immunohistochemistry results were positive for CK AE1/E3 antibody in the neoplastic epithelial cells with no expression of estrogen or progesterone receptors, and HER2/neu was not overexpressed (). The lymphocytes in the background stained positive for both CD3 and CD20 (Figures and ).\nThe patient underwent a left modified radical mastectomy. Eleven lymph nodes were dissected and free of tumor. The mastectomy specimen showed a 3.5 × 3 × 3 cm cavity at the site of the previous excisional biopsy. On histological examination, apocrine metaplasia was identified but no residual tumor was detected. To note, apocrine metaplasia is a very common incidental benign finding that is considered part of or associated with fibrocystic changes, and hence, does not affect prognosis and management []. Accordingly, no adjuvant hormonal therapy, chemotherapy, or radiotherapy was given to the patient.\nNo evidence of recurrence was noted on a 2-year follow-up. |
A 10-day-old male infant was referred to our hospital because of suspected congenital hypothyroidism. The patient presented with symptoms of airway obstruction, such as an inspiratory stridor and retracted breathing. A hormonal test revealed subclinical hypothyroidism with a free thyroxine level (1.44 ng/dL) within the reference range, although the thyroid stimulating hormone (TSH) level (34.6 μIU/mL) was increased beyond the normal range. Laryngo fiberscopy revealed a lingual mass compressing the epiglottis (Fig. ). Enhanced computed tomography (CT) and thyroid scintigraphy revealed that the mass was an ectopic thyroid with the absence of a normal pretracheal thyroid gland (Figs , ). The patient received oral levothyroxine at a dose of 12 μg/kg/day for 4 weeks to lower the TSH level and reduce the volume of the ectopic thyroid tissue. However, we observed no reduction in the volume of the thyroid tissue and a concomitant progression in his symptoms of airway obstruction. He underwent surgery to relieve the airway obstruction when he was 2 months old. Under general anesthesia, nasotracheal intubation was performed in a sniffing position, and a transverse skin incision measuring 2.5 cm was made at the level of the hyoid bone. We split the hyoid bone at the midline, dissected the base of the tongue towards the foramen cecum, detected the ectopic thyroid mass, and suspended the mass by suturing it to the hyoid bone (Fig. ). We used 5–0 monofilament absorbable sutures and sutured between the lingual thyroid and the hyoid bone. The bite length of both the lingual thyroid and hyoid bone was about 3 mm. The points of suturing were to the lateral side of the lingual thyroid and to the front of it. The total number of suture threads was 3. The degree of suspension of the ectopic thyroid was guided by an intraoperative laryngo fiberscopy to confirm the complete elevation of the epiglottis. The patient was not extubated until postoperative day 4 and needed noninvasive positive pressure ventilation until postoperative day 22. Laryngo fiberscopy performed 6 months postoperatively revealed the complete disappearance of compression of the epiglottis by the lingual mass, and CT performed 8 months postoperatively also revealed the relocation of the lingual thyroid gland towards the hyoid bone (Fig. ). When the patient was 2 years 6 months old, his height was 94.1 cm(1.4 SD), weight was 14.0 kg(1.0 SD), free T3 was 2.97 pg/mL, free T4 was 1.48 ng/dL, and TSH was 4.178 μIU/mL. He was taking daily levothyroxine 4.5 μg/kg/day and had been kept in the euthyroid state. Since we were able to preserve his thyroid gland (which is his only functioning thyroid tissue), the postoperative control of his thyroid hormone status was relatively easy. The surgery was complicated by the development of a salivary fistula that was spontaneously resolved 5 months postoperatively. |
The second case shows a 53-year-old female. Clinical and radiographic examination confirmed a unilateral posterior crossbite due a transverse maxillary deficiency with a significant mandibular skeletal deviation towards the side of the crossbite (Fig. ). Treatment objectives.\nSARME was planned to correct the transverse discrepancy followed by arch leveling with lingual appliances and then a second surgery to correct the mandibular asymmetry.\nSimilar to case 1 impressions were obtained and this time the lingual appliances were manufactured by DW Lingual Systems (Bad Essen, Germany).\nDuring the planning for the production of the lingual brackets, it was noted -similar to case 1- that a surgically assisted rapid maxillary expansion takes place. The transverse width of the upper jaw should be adapted to the lower jaw.\nTwo trans sagittal Benefit mini-implants were inserted in the T-Zone. A silicon impression with the transfer caps was taken. The impression was given to the laboratory together with the lingual molar bands. A Hybrid Hyrax [] was then made and laser welded to the molar bands (Fig. ). Similar to case 1, the lingual appliance was indirectly bonded with a dual cured resin and the maxillary expansion appliance was inserted. In this case the molar bands were cemented with a dual cured resin and the hybrid hyrax was fixed to the mini-implants using the Benefit fixation screws. The first lower arch wire 12 NiTi was inserted while in the upper the brackets were secured with a continuous steel ligature in each quadrant (Fig. ). SARME was performed with an activation rate of two quarter turns twice a day until crossbite correction was achieved at two weeks post-surgery (Fig. ). The Hybrid Hyrax was then blocked. The first upper archwire (12 NiTi) was placed four weeks after surgery (Fig. ). After complete leveling and radiographic re-examination the surgery to correct the asymmetry was performed.\nThe patient has a positive overbite and overjet now. The patient shows a good transversal and sagittal occlusion. |
A 53-year-old male patient, not known to have any medical illness, was brought to the emergency room by his wife and daughter due to decreased oral intake since three days. The decreased oral intake was associated with vomiting. The patient had a normal state of health until 2 months prior to his visit, when he started to develop changes in his behavior. According to his wife, the patient became more aggressive, had reduced sleep, had stopped going to work, and became isolated. He also had hallucinations and episodes of short-term memory loss. The above symptoms were accompanied by generalized body weakness, mainly in the lower limbs. The body weakness was associated with pain; the patient became wheelchair-bound. During the last two months, the patient visited many physicians, but no definite diagnosis was determined. He was examined by a psychiatrist and was diagnosed with organic mood disorder. He was prescribed quetiapine (antipsychotic), mirtazapine (antidepressant), and lamotrigine (mood stabilizer). However, according to his wife, he showed no improvements after taking the medications.\nThree months prior to his visit, the patient was admitted by the gastroenterology team due to biliary obstruction and endoscopic retrograde cholangiopancreatography (ERCP) was performed; biliary stricture was observed and he underwent stent insertion. At this time, he was also found to have a focal hepatic lesion. He did not have any past psychiatric history, a history suggestive of diabetes mellitus, autoimmune diseases, or any past gastric or ileal surgeries. He had a brief history of alcohol consumption but had stopped drinking 20 years ago. He was not a vegetarian. His daily diet was predominantly carbohydrate-rich and contained adequate animal protein.\nAn initial examination indicated pale mucous membranes, but no jaundice. The patient was conscious, but disoriented to place, time, and person. He said irrelevant words, was unable to comprehend others, and had bouts of crying. During the neurological examination, he was unable to support himself while standing. All cranial nerves were intact. Fine resting tremor was observed in both hands. The upper limbs had normal muscle size, a power rating of 5/5, normal reflexes, and hypertonia. The upper limb sensory level could not be assessed. The lower limbs had a power rating of 3-4/5 symmetrical, increased reflexes and hypertonia. There was pain in the lower limbs on passive movement and a positive Babinski sign. Senses of vibration and proprioception could not be assessed. Cerebellar examination indicated an abnormal finger-to-nose test and an abnormal shin-to-heel test with dysdiadochokinesia. The patient had a score of 8/30 on the Mini Mental State Examination. The rest of the examination was unremarkable.\nThe patient's initial laboratory investigations indicated a normal white blood cell count, mildly decreased hemoglobin (12.4 g/dL), elevated mean corpuscular volume (MCV) (102 fL), and normal platelet count (212 × 103 U/L). There were undetectable levels of vitamin B12 (less than 83 pg/mL). His aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were mildly elevated at 36 U/L and 44 U/L, respectively. The patient had a lactate dehydrogenase (LDH) level of 264 U/L, total bilirubin of 1.6 mg/dL, and direct bilirubin of 0.48 mg/dL. The serum iron level was 6 μmol/L, and the reticulocyte count was 0.2%. The levels of creatinine, serum electrolytes, and ceruloplasmin and the thyroid function test were normal. shows the results of all lab investigations at the time of presentation of the patient. Tests for human immunodeficiency virus and syphilis and drug screening tests were negative. Magnetic resonance imaging (MRI) of the whole spine was performed and showed osteophytic lesions at the C3-C4, C5-C6, and C6-C7 levels. The spine MRI was normal otherwise. Brain MRI with contrast showed diffuse involutional brain changes and chronic white matter ischemia. An upper endoscopy was performed and was normal. In addition, biopsies showed no evidence of atrophy or malignancy.\nDuring the patient's admission, the neurology team was consulted, and they initially advised to stop all medication. They also requested brain computed tomography (CT) and whole spine MRI. Later, when the diagnosis of vitamin B12 deficiency was established, the neurology team advised that there is no need for further neurological investigations. An abdomen and pelvis CT with contrast was performed for further evaluation of the focal hepatic lesion and showed multiple focal hepatic cystic lesions with normal liver enzymes. The patient is being followed up by the gastroenterology team.\nThe patient was started on 1 mg intravenous methycobalamin (1,000 mcg) every day for two weeks. The dosage was then changed to 1 mg once weekly by intramuscular injection for another month after discharge. The patient was then switched to 500 mcg oral cobalamin three times daily for life. He returned for follow-up three months later.\nTwo months after starting the treatment, the patient came back for a follow-up. There was a major change in his behavior. He was fully oriented and had significant improvements in memory, but he did not remember when he was admitted. Power in lower limbs was 3/5 and he had an unsteady gait. Five months after starting the treatment, the patient returned for a second follow-up and had marked improvements in gait, muscle power, and cognitive function. Six months after starting the treatment, the patient was able to walk alone, drive his car, and had lower limb power of 5/5. The Mini Mental score was reassessed and was 30/30. The patient provided informed consent for his case to be reported. |
All patients in our case series were Caucasian male between 32 to 47 years old when the symptoms started (). However one patient presented to us at the age of 70 years and another at 61 years of age. All of our patients presented with slowly progressive ataxia and 2 had fall on multiple occasions. Only one person had family history of undiagnosed ataxia. All of them were referred by either the GP or the peripheral hospitals as the cause of their symptoms were unclear.\nAll of them demonstrated spasticity in the lower limbs. First three patients revealed some degree of horizontal ophthalmoplegia (). The third patient had asymmetric ptosis in addition to ophthalmoplegia. All of them had signs of cerebellar ataxia.\nAll 4 patients were subject to routine bloods, B12, folate, thyroid function test, copper, nerve conduction studies, etc. Then all had routine genetic testing for ataxia including spinocerebellar ataxia, Friedreich’s ataxia (Frataxin) Fragile X Associated Tremor/Ataxia syndrome (FRXTA) (). They subsequently had next generation sequencing of 21 gene panel for hereditary spastic paraparesis.\nThe Magnetic resonance imaging (MRI) showed some degree of cerebellar atrophy in first three patients. However the last patient had significant pan-cerebellar atrophy in MRI scan (). None of them showed any evidence of cord compression, demyelination or space occupying lesion in whole spine magnetic resonance imaging. The genetic screening for spinocerebellar ataxia (SCA 1,2,3 and 6) were negative for all. Patient 4 had additional testing for mutation in SCA 7 which turned out to be negative.\nThe next generation sequencing involving 21 genes in the spastic paraparesis panel revealed autosomal recessive hereditary spastic paraparesis due to the mutation in Spastic paraparesis 7 gene (SPG 7) in all of them with a novel mutation in one of them (). All these mutations were comfirmed by conventional Sanger sequencing.\nAll the patients were informed approximately their results and were sent for physiotherapy and occupational therapy assessment. The first 2 were also prescribed Baclofen for spasticity.\nSubsequently during the follow up the first 2 patients complained that their balance worsened while the remaining 2 patients said that they were unchanged. The 2 young patients were referred for further genetic counselling as they were concerned about their children (). |
A 43-year-old female patient presented to the gastroenterology clinic with complaints of rectal bleeding and constipation. She reported episodes of bloody stools cyclically coinciding with her menstrual periods with burning rectal pain, and she also reported long standing constipation for many years for which she has been taking laxatives. She also had lower abdominal pain, colicky in nature, improved with mucoid bowel movement, and associated with a sensation of incomplete evacuation and abdominal bloating. She denied fever, diarrhea, joint pains or skin rash. She denied any nausea, vomiting, loss of appetite and weight loss at the time of initial presentation.\nHer medical history was negative for any chronic medical conditions. She did not undergo any surgical procedures in the past. There were no gastrointestinal malignancies diagnosed in her immediate or distant family members. She never used tobacco products, alcohol or recreational drugs. She was not allergic to any medications.\nOn initial evaluation, her vital signs were within normal limits. Abdomen was non-distended, soft and non-tender to palpation. On auscultation, bowel sounds were noted to be normoactive. Rectal examination was unremarkable. Cardiorespiratory and neurological examination was within normal limits.\nShe underwent flexible colonoscopy under monitored anesthesia care. Examination revealed an 8 - 10 cm area of erythematous, congested and granular mucosa in the sigmoid colon (). Random biopsies done showed colonic mucosa with focal erosion and mild chronic inflammation. The area was tattooed for future reference. Also, noted during colonoscopy was non-bleeding hemorrhoid. In the interim patient continued to be symptomatic.\nWhile pursuing surgical consultation for the management of hemorrhoids, we decided to repeat a flexible colonoscopy with tissue sampling in view of her cyclical symptoms and initial colonoscopy findings. On repeat examination, the area was injected and raised using 5 mL of saline to create a fluid bed that separated the tissue layers from underlying muscle layer. Deeper tissue samples were obtained using cold forceps. Histopathological examination revealed fragments of mucosa consistent with endometriosis (). Immunohistochemical staining was positive for CK-7 and CD10 () which were consistent with the diagnosis of endometriosis. Interestingly further enquiry and review of her past record revealed her prior history of endometriosis. She was referred for a gynecological evaluation and successfully underwent elective hysterectomy with bilateral salpingo-oophorectomy. On follow-up visits, she reported complete resolution of symptoms. |
Thirty-sex-year-old female patient had a chief complaint of esthetics because of a gab of missing upper left lateral incisor and crown of root canal-treated canine (). The patient has normal horizontal and vertical overlap and canine-protected occlusion. After discussion with the patient, it became clear that the placement of an implant for the replacement of missing teeth was not possible due to high costs of the treatment. The fabrication of a conventional fixed partial denture was avoided and refused from patient in order to conserve the remaining tooth substance. Options for the conventional treatment with implants or crown-retained FPDs were remained open for the future. Directly made FRC FPDs were chosen in order to provide good esthetics, preserve tooth substance, and postpone more invasive treatments. The treatment was completed during one appointment.\nThere was free occlusal space on the palatal surface of central incisor for FRC framework to be placed. Consequently, no cavity preparation for receiving vertical support for the bridge was needed. Cotton roll for isolation was used although the rubber dam is highly recommended. Guttapercha root canal filling at the upper left canine was removed using Gates Glidden burs up to size 4 for the total length of 7 mm (4000 cycles min−1 with water cooling). The root canal was prepared to receive a root canal post. The individually formed glass FRC post (everStick Post, StickTech Ltd, Turku, Finland) was prepared following the manufacturer's instructions (). A bundle of preimpregnated glass fibers was cut to a length of 16 mm and spread from the ends for increasing the bonding surface area (). The bundle was inserted in the canal and initially light polymerized with a hand light-curing unit (Optilux-501) for 20 s. Then the post was removed from the canal and additionally light polymerized for 40 s. The surface of the FRC post system was then wetted with resin (Stick Resin) and protected from any light source by a light proof box (3 M-ESPE, Germany) until cementation. Cement (ParaCem Universal, Switzerland) was placed on the post and the post was seated and extra cement was removed. The FRC framework was extended from the palatal surface of premolar to palatal surface of central incisor passing by the FRC post of the canine.\nAfter application of acid etching (37% phosphoric acid gel), the gel was rinsed thoroughly and gently air dried. Adhesive resins were applied according to the manufacturer's instructions (Scotchbond multipurpose adhesive, 3M ESPE, USA) to tooth surface. Flowable composite resin (Stick Flow, StickTeck Ltd, Turku, Finland) was applied on the bonding surfaces prior placing the resin impregnated fibers (everStick). The flow composite was not light cured before fibers were pressed tightly against the tooth surface using a transparent silicone package (mold) of the fibers. The resin impregnated fibers were light cured initially through the silicone mold. The purpose of the flow composite was to seal the space between the fibers and the enamel surface. The fiber framework was polymerized two times for 40 seconds (). Fiber framework was fully covered with a thin layer of flow composite resin, and pontic was built up layer by layer using hybrid-type particulate filler composite resin. Successful chemical bond between fiber framework and veneered composite was achieved by curing. The shade of final veneered composite resin was selected using composite shade guide, and occlusion was carefully adjusted with articulating paper (Figures and ).\nThe occlusion was adjusted carefully to avoid any primary or premature contacts or traumatic occlusal forces to the restored teeth. The treatment outcome has been followed over three years without existence of any kind of serious problem. |
A 65-year-old patient was referred to our urological clinic for a prostate biopsy indicated for a PSA elevation of 4,5 ng/mL. The patient presented without any previous morbidities in his medical or urological history and was entirely asymptomatic. Digital rectal examination (DRE) and transrectal ultrasound (TRUS) of the prostate were normal, the size of the gland was 36 mL. An octant biopsy was conducted in January 2002 by which adenocarcinoma of the prostate (PCa) was diagnosed in 1 out of 8 cores. The lesion was circumscribed with a length below 2 mm and a Gleason grade of 2, WHO grade was 1, all the other seven biopsies were classified as benign prostatic hyperplasia and chronic inflammation. Following discussion of all therapeutical options, the patient decided to undergo permanent brachytherapy with J125. 62 seeds with 0,467 mCi/seed were implanted, total activity was 28,95 mCi, and postoperative course was without any complications.\nDuring the early postoperative phase, the patient was free of complaints, there was neither a sign of incontinence nor any stool disorder, even the erectile function was assessed by an IIEF-score of 21. PSA was constantly decreasing to reach its nadir of 0,75 ng/mL 15 months after seed implantation.\n21 months after brachytherapy, the first increase of PSA up to 2,6 was observed. Presuming the possibility of a so-called “PSA bouncing” with an episode of prostatitis a cycle of antibiotic therapy with ciprofloxacin over three weeks was administered, after which PSA fell again to a level of 2,11. At the subsequent follow-up examination half a year later, the patient presented with obvious local and systemic progression: PSA rose up to 8,1 ng/mL with a doubling time of three months, and digital rectal examination showed a dense left lobe with a firm node on the contralateral side. At this time, the patient refused restaging and rejected the recommended LHRH agonist therapy. After prophylactic radiation to the breasts with a dose of 1500 cGy antiandrogen monotherapy with bicalutamide 150 mg per day was initiated. Receiving this medication a drop of PSA to 2,4 ng/mL was achieved, the patient was furthermore feeling asymptomatic.\nDue to a PSA progress up to 10,6 ng/mL after 15 months of antiandrogen treatment, reevaluation was conducted. While choline PET-CT showed an increased fluorocholine (FCH) metabolism in the right lobe of the prostate with no signs of lymph node or bone metastases, a rebiopsy of the gland yielded a dramatic upstaging and upgrading of the local disease: of 15 cores taken 13 were infiltrated by prostate cancer with Gleason Score 8 (4 + 4), WHO grade was 3. Antiandrogen therapy was stopped; the LHRH agonist leuprorelin acetate was administered. Due to the history of brachytherapy, any form of further external beam radiation was not feasible.\nOnly two months later, the patient had to be hospitalised again because of gross haematuria and clot retention. A CT scan showed local progression with a large solid tumour dorsal of the right side of the prostate and bladder with a diameter of 6 cm as well as pathologically enlarged lymph nodes in the pelvis up to 2 cm. Due to continuous bleeding under bladder irrigation palliative transurethral resection of the prostate had to be performed, 15 g of fragile tumour tissue were removed. The pathological report confirmed the diagnosis of an adenocarcinoma with a Gleason score of 8, but additionally larger areas with neuroendocrine differentiation were found.\nAfter a period of the next three weeks, transurethral reintervention for severe haematuria had to be undertaken, and due to persistent bleeding transfusions of several red cell concentrates were necessary. Obstruction of the upper urinary tract led to bilateral nephrostomies. After discharge, several recurrent episodes of bleeding and clot retention occurred. The general health status deteriorated preventing the initiation of systemic chemotherapy.\nOnly two months following the previous CT scan, MRI showed a large solid local tumour with a diameter of now 9 cm infiltrating the trigone and the bladder floor on each side (). Beneath lymph nodes, up to 3 cm disseminated bone metastases were diagnosed. Four years after the “first definitive” therapy, the patient received a palliative ileal conduit to control local symptoms. With regards to the limited life expectancy, the prostate and bladder remained in situ; recurrent bleeding was controlled by percutaneous transluminal angiography and coil embolization; additionally, instillations with formalin were performed twice. The patient's health status was declining rapidly and three weeks after the last surgery, he died due to dilatation of the right ventricle and pulmonary oedema. Beyond that, autopsy showed extensive pulmonary metastases. |
Case 2. A 53-year-old African American male with PMH of hypertension and drug use had been receiving hemodialysis for 6 years as a consequence of end-stage renal disease secondary to hypertension. After failed attempts at a left lower nondominate arm AVF, a left BCF was placed in 2011. Over the years the BCF developed large aneurysms at sites of repeated cannulation. In 2015, the patient presented to the Emergency Room with chief complaint of altered mental status. Symptoms at presentation included disorientation to place and time and missed medication and inability to care for himself. He had been receiving hemodialysis three days a week. Clinically during the preceding weeks, the patient was experiencing marked dyspnea with exertion and lower extremity edema, cramping with hemodialysis and inability to achieve prescribed dry weight. Medications on admission included amlodipine, sensipar, hydralazine, megace, pantoprazole, thiamine, and renvela. Physical exam showed a blood pressure of 198/110 a pulse of 106. He was oriented only to person. Lungs were clear, cardiac exam normal S1, S2, 2+ edema. Left AVF was very dilated from the anticubital area to the mid arm. There were multiple large aneurisms which were nontender, thinning skin, but there is no evidence of excoriation. The workup including CT was negative for an intracerebral event. Blood cultures were drawn and came back positive with growth of alpha hemolytic strep treated with intravenous vancomycin and cefepime for 6 weeks. The source of the bacteremia was unclear and a venous Duplex of the access and an echo was done. The AVF Duplex ultrasound study showed a volumetric flow of 10,731 mL/min measured in the mid venous outflow. An echocardiogram done showed moderate LVH, moderate dilation of the right atrium, and marked increase in left atrial volume. Patient was discharged and at subsequent hemodialysis the patient was found to have elevated venous pressures which prompted interrogation via a fistulogram. The venogram study was done in June 2016 with findings of long segment tight CAS () and two large aneurysms (). The outflow narrowing at the cephalic arch was presumed to be the causative factor for the presenting history of elevated venous pressures at dialysis and the decision was made to pursue angioplasty. Initial angioplasty was performed using a Mustang 7 mm × 40 mm standard pressure balloon catheter (Boston Scientific, Marlborough, MA) rated up to 20 atm. Postangioplasty imaging demonstrated only moderate improvement. Thus a second angioplasty was performed using a larger Mustang 9 mm × 40 mm standard pressure angioplasty balloon catheter (Boston Scientific, Marlborough, MA) rated up to 18 atm. The cephalic arch stenosis opened up to 6 mm from 3.5 mm, which equated to a 42% improvement in stenosis. Postangioplasty venography demonstrated subjectively improved flow and satisfactory resolution of the stenosis. In August 2016 the patient underwent revision of the left upper extremity AVF with a jump graft inserted, followed by interval ligation and excision of two large pseudo aneurysms. After surgery, the AVF was able to be used with hemodialysis delivered at a 450 mL/min blood flow without the need for central venous catheter access. The patient's volume status was better managed after the AVF revision and subsequently went on to receive a renal transplant in May 2017. |
A 21-year-old female patient came to our service with a complaint of unilateral right-onset headache associated with diplopia initiated 6 months earlier. She had no personal or family remarkable antecedents. She never smoked. Six months earlier, the patient started to experience one-sided right throbbing headache. She denied nausea, vomiting, or photo- or phonophobia. Fifteen days after the pain onset, she noticed double vision and medial deviation of the right eye, which forced her to wear an eyepiece to perform her activities and drive. She went to several centers and used various medications such as paracetamol, nonsteroidal anti-inflammatory drugs (NSAIDs), opioids, and triptans without improvement. Three months earlier, she started using dexamethasone 4 mg daily with partial pain control but maintenance of diplopia.\nAt the examination, the patient had cushingoid face, violaceous striae, and right VI cranial nerve palsy with no other neurological changes. Blood tests were normal (). A contrast-enhanced MRI scan of the brain did not show any remarkable features (). A spinal tap released crystalline cerebrospinal fluid (CSF) with an opening pressure of 14 cm of water. Biochemical, microbiological, and cytological analyses of the CSF were normal (). CT scan of thorax did not show any evidence of lymphoma or sarcoidosis.\nPrednisone 1mg / kg was then started. With one week of treatment, complete reversal of ocular paralysis and remission of pain were observed. However, when the corticoid was gradually withdrawn, the patient returned to pain and returned to paralysis of the VI right pair. The prednisone was increased again to 1 mg / kg this time with reversion of ocular paralysis but without pain control. Several prophylaxis attempts were made with beta-blockers, calcium channel blockers, topiramate, and tricyclics without any symptomatic control that would allow corticosteroid withdrawal.\nThe pregabalin 150mg daily was then introduced. With 7 days of medication onset there was already an important remission of pain. With 15 days of pregabalin initiation, the retitration of prednisone was started without any intercurrence and the patient reversed the exogenous Cushing syndrome. Pregabalin was maintained for one year and retracted. Currently, the patient has been free of pain for 2 years. |
The patient is a 7-month-old twin boy who presented to our institution's emergency department with increased work of breathing and desaturations (70 s). He was born at 33 weeks gestational age with Down syndrome, developed chronic lung disease (CLD) of prematurity, and was also found to have a moderate size secundum atrial septal defect (ASD) as a newborn. Prior to the current illness, he had been in the hospital multiple times for failure to thrive and respiratory distress, requiring mechanical ventilation with high amount of supplemental O2 and inhaled nitric oxide (iNO) as he developed pulmonary hypertension (PH). Echocardiography showed progressive enlargement and hypertrophy of his right ventricle and at times bidirectional shunting across his ASD. A diagnostic cardiac catheterization as a preoperative evaluation was performed, which showed elevated pulmonary vascular resistance indexed (PVRi) at baseline (8.8 WU·m2), which decreased with inhaled oxygen alone and iNO (3.8 WU·m2). Additional catheterization data at baseline condition showed a right atrial mean pressure of 6 mmHg, right ventricular end diastolic pressure of 6 mmHg, and pulmonary artery pressure 51/19 mmHg with mean 32 mmHg. The patient was started on home O2 therapy with nasal cannula. The current hospitalization occurred prior to a planned fenestrated patch repair of his ASD.\nHe was initially admitted to the general ward and soon transferred to the pediatric ICU for severe hypoxemic respiratory failure requiring mechanical ventilation. Respiratory syncytial virus (RSV) infection was diagnosed with the positive antigen test. He continued to have paroxysmal severe hypoxic events compatible with PH crisis. He was treated with sedation and neuromuscular paralysis, increased FiO2, optimization of O2 carrying capacity with packed red blood cells transfusions, and iNO. Milrinone infusion was added as the right ventricular function was depressed on echocardiogram (TAPSE 6 mm, Z-score −4), which demonstrated evidence of systemic to suprasystemic right ventricular pressure and bidirectional shunting across the ASD (Figures and ). No other cardiovascular intravenous drips were given during the ICU stay. Sildenafil was initiated enterally and escalated to maximal dose (2 mg/kg/day) without hemodynamic compromise. He was on diuretic therapy (bumetanide infusion up to 10 mcg/kg/hr) as chest X-ray demonstrated evidence of bilateral interstitial edema with bilateral pleural effusions on admission () and confirmed by chest ultrasound. Bilateral chest tubes were placed after failure of diuretic therapy to reduce effusions on hospital day #6. The drained fluid was milky in appearance bilaterally, with a white blood cell of 1,004/mm3 with lymphocyte predominance (88%) and elevated triglycerides (1008 mg/dl), and hence a diagnosis of chylothorax was made. Low IgG level (249 mg/dl) and hypoalbuminemia (2.5 g/dl) were noted at the time of pleural effusion drainage. Intravenous immunoglobulin and 25% albumin solution were administered. His feeding formula was changed to medium-chain triglyceride formula. The milky drainage became serous; however, the volume of chest tube drainage remained unchanged. Enteral feeding was discontinued and total parenteral nutrition was initiated, which decreased the volume of pleural effluent but small to moderate amount of pleural effusion was intermittently observed by chest X-ray for over sixty three days until the patient's death. Venous Doppler ultrasound of the upper extremities and the neck was performed on hospital day #7 and 4 weeks later, and compression, thrombosis or obstruction of the superior vena cava, and upper extremity were ruled out. A central venous catheter was placed in the right jugular vein soon after admission and was removed on hospital day #7 and replaced by a peripherally inserted central line. The patient required chest tubes for drainage until hospital day #22. Since then, intermittently small to moderate pleural effusion was observed by chest X-ray, but chest tubes were not placed.\nHe continued to be critically ill with persistent hypoxemic respiratory failure without improvement in PH with several PH crisis episodes. Therapy with an endothelin (ET) receptor antagonist (Bosentan) was added. The hospital course was complicated by bacterial tracheitis from Pseudomonas and E. coli. The patient remained on mechanical ventilator support for 6 weeks due to failed weaning of ventilator support from hypoxemia despite high levels of supplementary FiO2 and iNO. Cardiac catheterization performed 6 weeks after admission showed PVRi of 7 WU m2 on 100% FiO2 and 20 ppm of iNO under general anesthesia, pulmonary venous desaturation, and bidirectional shunting through ASD. Additionally, interval increases in right atrial pressure (mean 13 mmHg), right ventricular end diastolic pressure (12 mmHg), and pulmonary artery pressure (52/24 mean 36 mmHg) were noted. Given his severe and irreversible lung injury from mechanical ventilation in addition to baseline chronic lung disease, he was deemed not a candidate for lung transplant. Considering that the patient had Eisenmenger physiology due to severe PH and poor prognosis, the palliative care team was also consulted. Weaning from the mechanical ventilator was tried multiple times, but failed. At 9 weeks of his ICU hospitalization, he developed severe hypoxemia unresponsive to medical therapy that ultimately caused his death.\nAn autopsy showed bilateral small straw-colored pleural effusions (right 17 ml and left 10 ml), and the lung parenchyma was red-brown, poorly aerated, and diffusely congested with focal consolidation. The heart had an ASD (0.8 × 1.2 cm) with right ventricular hypertrophy secondary to PH. Microscopically, both lungs showed subpleural cysts lined by pneumocytes and containing macrophages, sloughed pneumocytes, and neutrophils. Acute multifocal bronchopneumonia was present with neutrophils in the bronchioles and alveoli. Chronic interstitial lung disease is diffusely present with alveolar septal thickening, capillary disorganization, and hemosiderosis. Small pulmonary arterial branches demonstrate moderate to marked medial smooth muscle hypertrophy with lumen narrowing, while large pulmonary arteries were normal with minimal changes. No lymphatic dilatation was observed on H&E or D2-40 immunostained slides; therefore, lymphangiectasia was ruled out (). From the autopsy results, hypoxia due to progressive PH was considered as a cause of death. |
A 73-year-old Caucasian man presented to the outpatient clinic for evaluation of a recent episode of implantable cardioverter defibrillator (ICD) shock therapy. He had been implanted a dual chamber ICD (Model 1871, Vitality DR, Guidant Corp.) for secondary prevention due to resuscitated sustained ventricular tachycardia, not related to a correctable cause, in the context of severe ischemic cardiomyopathy. His past medical history was significant for coronary artery disease (old myocardial infarction and coronary artery bypass surgery), hypertension, diabetes mellitus, and hyperlipidemia. The patient's medications included metoprolol, ramipril, glimepiride, aspirin, and simvastatin.\nInterrogation of the stored events revealed that the recent episode of shock was an appropriate defibrillation (21 J biphasic shock) of ventricular arrhythmia (cycle length 330 ms) that classified into the VF zone. However, electrocardiographic examination and evaluation of the current electrograms revealed the presence of atrial fibrillation (AF) with a ventricular response of 84 beats/min (Figures , ). The duration of AF was unknown while no event of inappropriate shock attributed to AF was detected. All hematological and biochemical studies including thyroid function tests were within normal limits. An echocardiographic study showed left ventricular (LV) dilatation with global systolic dysfunction (ejection fraction: 0.20) and evidence of increased filling pressures. The left atrial (LA) anteroposterior diameter was 41 mm and the LA diastolic volume 36 ml.\nTaking into account the severely impaired left ventricular systolic function as well as the absence of LA enlargement we decided to follow a rhythm control strategy. Therefore, the patient was placed on appropriate anticoagulation therapy for 4 weeks and scheduled for cardioversion. He was admitted to the Coronary Care Unit and placed to electrocardiographic, non-invasive hemodynamic, and respiratory monitoring. After performing mild sedation with midazolam, the patient's ICD was externally programmed to deliver an R-wave synchronized ventricular biphasic shock of 31 J. The cardioversion was successful with immediate restoration of sinus rhythm (Figure ). Subsequently, the patient was placed on amiodarone for sinus rhythm maintenance while continued receiving b-blocker therapy and anticoagulation. His recovery was rapid and uneventful and discharged 6 hours later. After a 10-month follow-up period, the patient remains on sinus rhythm and on good clinical condition. His LV ejection fraction has been improved to 0.28 while the LV filling pressures are normal. Interestingly, the patient does not remember any shock or associated pain with respect to the AF cardioversion. |
The patient was a 59-year old man, Caucasian type, without any specific medical personal or familial history. In October 2006, he underwent a pelvic ultrasonography (US) because he presented a "pressure sensation" in the rectum and rectal imperiosity for four weeks. Physical examination revealed no additional symptom, except the rectal examination that detected a 4-cm hard pre-rectal mass filling the right side of the pelvis. The WHO performance status was equal to 0. Ultrasonography (US) discovered a tumour located at the right side of the pelvis. Serum prostate-specific antigen levels were normal. The patient was thus referred to hospital.\nComputed tomography (CT) of abdomen and pelvis revealed an 8-cm heterogeneous tumour predominantly located on the right side of the pelvis likely arising from the right seminal vesicle, reaching the median line and adherenting to the posterior bladder wall and the anterior rectal wall. Due to the rectal clinical symptoms and the suspicion of rectal adherence on CT scan, the core needle biopsy was CT-guided transperineal rather than transrectal. Pathological analysis revealed a well-differentiated leiomyosarcoma. Pelvic magnetic resonance imaging (MRI) confirmed the presence of a mass centred on the right seminal vesicle, causing mass effect on the prostate and left seminal vesicle, without a cleavage plane with the right prostate and the right obturator muscle, suggesting invasion (Figure ). Otherwise, MRI clearly showed fat interface between the mass, the posterior bladder wall and the anterior rectal wall without any sign of involvement. Complete clinical and radiological screening did not detect any lymphadenopathy or distant metastasis. Based on these data, the tumor was considered completely resectable, and surgery was decided.\nBefore surgery, the examination under general anesthesia revealed that the mass was difficult to mobilize. The treatment consisted of a radical vesiculo-prostatectomy with limited (ilio-obturator and hypogastric) bilateral pelvic lymphadenectomy in January 2007, associated with resection of the internal obturator muscle. No anterior rectal resection was necessary. Excision was macroscopically complete. The post-operative course was uneventful. The pathological macroscopic examination of the surgical resection specimen showed a small prostate (4.5 × 4 × 3 cm) and an 8 × 8 × 6.5 cm well-limited hard white-tan mass, centred on the right seminal vesicle, tangent to the prostate base, pushing the vas deferens, the prostate and the left seminal vesicle. On cut section the mass showed a grey-white whorled appearance with foci of necrosis. Microscopic analysis (Figure ) confirmed the previous diagnosis by showing intersecting fascicles of atypical spindle cells with elongated blunt-ended nuclei and eosinophilic cytoplasm very reminiscent of smooth muscle differentiation. Immunohistochemical analysis showed strong positive staining of the tumour cells for smooth-muscle actin and H-caldesmone (Figure ), and negative staining for pan-cytokeratin AE1/AE3, CD117. The mitotic rate averaged ten mitoses per ten high-power fields. Necrosis was present on less than 50% of the tumour surface. The tumour seemed developed within the wall of the right seminal vesicle. It was a well-limited and pseudo-wrapped with limited surgical margins, sometimes inferior to 1 mm. The retained diagnosis was moderately differentiated (FNCLCC grade 2) leiomyosarcoma of the right seminal vesicle. The fourteen removed pelvic lymph nodes were free of tumour. Moreover, an associated small (4 × 4 × 2 mm) prostatic adenocarcinoma was fortuitously discovered on the left apex of the gland, Gleason 6 (3+3), without any macro- and microscopical connection with the sarcoma. After surgery and because of closed margins for the leiomyosarcoma, the patient received adjuvant pelvic external beam radiation therapy achieved in April 2007 (56 Gy./31 fractions). No adjuvant chemotherapy was delivered. The patient was then regularly monitored at the clinical and radiological levels.\nIn April 2009, 29 months after diagnosis, he developed two subcutaneous nodules on the scalp. Surgical removal and pathological analysis confirmed the diagnosis of distant recurrences of leiomyosarcoma. At the same time, CT and TEP scans detected the presence of multiple asymptomatic liver and lung metastases. Performance status was excellent (WHO 0). First-line chemotherapy consisted of six cycles combining doxorubicin and ifosfamide, but metastases progressed (October 2009), and the patient was referred to our institution for second-line chemotherapy. We delivered a gemcitabine-docetaxel combination. After three cycles, CT scan showed stable lung and liver lesions, whose size decreased after the sixth cycle, then after the ninth cycle, with a 30% response when compared with October 2009 (Figure ). Treatment was then interrupted, and the patient regularly followed. A last visit, in February 2011, 51 months after diagnosis of the primary tumour, and 22 months after the first metastasis, the patient is alive with excellent performance status, without any symptom, and with multiple stable lung and liver lesions. |
A 73-year-old female was referred to our Trust by her general practitioner with a 5-month history of a painless vaginal mass, which extruded from the introitus on straining, but was otherwise asymptomatic. This was originally thought to be a vaginal prolapse; however, examination revealed a soft, well-defined pink mass occupying the upper vagina and an MRI of the pelvis was requested for further characterization.\nMRI was performed using a 3.0 T system utilizing axial T1 weighted fast spin echo; small field of view axial, coronal and sagittal T2 weighted fast spin echo; and T1 weighted fat-saturated sagittal images before and after gadolinium contrast administration, obtained in the arterial and portal venous phases. Diffusion-weighted imaging was also acquired. The images demonstrated a 47 × 40 × 44 mm well-circumscribed, oval mass in the upper vagina. On the T1 weighted images, the signal intensity of the abnormality was intermediate, similar to that of the skeletal muscle (). However, on T2 imaging, there were discrete zones within the lesion; the anteroinferior aspect was of high T2 signal with no enhancement, whereas the posterosuperior aspect was of low T2 signal with avid enhancement (–). There was no restricted diffusion. The posterior wall of the retroverted uterus was demonstrated to abut the superior surface of the lesion and the vaginal lumen was deviated anteriorly. Normal vaginal wall was seen to extend around the lesion’s anterior and posteroinferior surfaces. The lesion appeared to be arising within the left posterolateral vaginal wall and there were areas of loss of definition of the outer margin of the vagina. There was no involvement of the rectum, urethra or bladder; however, there were hazy low T1 and T2 signal changes in the left paravaginal fat.\nThrough a MDT discussion, it was agreed that owing to the suspicious imaging features of enhancement and tissue inhomogeneity, a staging portal venous phase CT scan was required to look for evidence of metastatic spread. Again, the vaginal lesion demonstrated fluid and soft tissue attenuation areas with regions of enhancement (). Significantly, there was no evidence of distant spread or lymph node enlargement. After further MDT discussion, the mass was still thought to be suspicious for malignancy and the patient underwent surgery.\nThe uterus, ovaries, cervix and upper vagina were removed en bloc and macroscopic examination revealed a well-circumscribed 45 mm polypoid mass arising from the paracervical upper vaginal tissue. The cut surface of the lesion was fleshy grey and white in colour, and was mainly solid in nature.\nMicroscopic examination () revealed an unencapsulated lesion with a spindle cell morphology arranged occasionally in fascicles. Beneath the surface epithelium, there was a grenz zone. The spindle cells were set within finely collagenized stroma and were bland in nature, with no conspicuous mitoses identified. Areas of oedema and myxoid change were also present, with no evidence of haemorrhage or necrosis.\nImmunohistochemistry demonstrated that the lesional cells expressed desmin, vimentin, oestrogen and progesterone receptors. The Ki67 proliferation index was low. Immunohistochemistry for MNF116, alpha smooth muscle antigen, smooth muscle myosin, h-caldesmon, S100 and CD34 were negative in the lesional cells.\nThe morphological and immunohistochemical profile was considered consistent with a superficial cervicovaginal myofibroblastoma, which is also known as superficial myofibroblastoma of the lower female genital tract. |
A 30-year-old man was admitted to a neurobehavioral rehabilitation unit as a result of the development of a functional neurological symptom disorder following a concussion sustained in a motor vehicle accident 3 years earlier. The patient had a history of type 2 diabetes for 8 years, but managed this successfully with only physical activities and no medication prior to the accident. Unfortunately, there is no available data of his glucose values or HbA1C level prior to his injury. Following the accident, he developed depression, anxiety with the features of post-traumatic stress disorder, and functional neurological symptoms with gait and speech dysfunction, with brain imaging studies showing no intracranial pathology. Further complicating the clinical presentation were chronic pain, insomnia, and neurocognitive impairments. Together, these impairments and symptoms prevented the patient from engaging in his normal routine of activities of daily living (ADL) and work. At the time of the admission, he had gained 14 kg (BMI 28.7) and required 50 units insulin glargine subcutaneous at bedtime, 15 units of insulin aspart three times a day with meals, as well as a correctional scale. Furthermore, he took canagliflozin 300 mg daily and metformin/sitagliptin 50/1000 1 tablet twice a day. In addition, he took rosuvastatin 10 mg daily and fenofibrate 160 mg daily for dyslipidemia.\nPsychological assessment included standardized measures (Symptom Checklist 90 Revised, Beck Depression Inventory Revised, Beck Anxiety Inventory, Insomnia Severity Index, Brief Pain Inventory). Findings were valid and showed abnormal elevations related to phobic anxiety, worry about somatic complaints and cognitive impairments, and significant problems with sleep and pain.\nIn addition to medical care, the patient received transdisciplinary rehabilitation from a team including rehabilitation therapist, occupational therapist, physiotherapist, speech-language pathologist, behavior therapist, and neuropsychologist. Rehabilitation was carried out in a therapeutic milieu in which functional activities were presented individually and in groups, both on the unit and in the community. Activities were organized in a daily schedule that interspersed rehabilitation with rest throughout the day, with both staff and patient recording completion of activities. Activities included physical exercise, as well as personal care, social and recreational pursuits, chores to care for his ADL apartment on the unit, and community-based tasks such as shopping and volunteering. Duration and intensity of activities were gradually increased during the course of admission, with goals adjusted collaboratively.\nPsychological treatment in the form of cognitive behavior therapy was directed to reducing phobic avoidance of proximity to motor vehicles. As the patient progressed successfully through a hierarchy of phobic situations, his mobility increased from using a walker to running. Relaxation and mindfulness exercises were also conducted, and self-directed practice introduced. Relaxation skills were integrated with sleep hygiene methods. Psychoeducational intervention was provided related to the expected process of return to health following concussion.\nWith the application of multidisciplinary approaches on the neurobehavioral rehabilitation unit, he gradually made functional gains during a 4-month admission. Psychometric findings from readministration of standardized measures shortly before discharge showed a resolution of anxiety, worry, sleeping difficulty, and pain. Physically he discontinued the usage of a rollator walker for ambulating and started aerobic exercises and resistance training while his mood and behavior were improving. Approximately 3 months after his admission, all his hypoglycemic agents, including insulin and oral medications, had been gradually weaned off, and he had lost more than 10 kg (BMI 25.0). His HbA1C dropped from 10.5% to 6.4% and his blood glucose was well controlled without any pharmacological agents (capillary blood glucose (CBG) varied between 4.0 and 8.0 mmol/L). Meanwhile, rosuvastatin and fenofibrate were discontinued as his triglyceride reduced from 4.15 to 0.89 mmol/L, and non-HDL cholesterol from 3.50 to 3.16 mmol/L.\nThe authors received consent from the patient included in this case report. |
The patient is a 47-year-old Caucasian female who presented to the Emergency Department of an academic tertiary-care hospital in the Midwestern United States with complaint of left-sided weakness of the upper and lower extremities and right gaze preference three weeks after a right pontomedullary infarct complicated by Posterior Reversible Encephalopathy Syndrome (PRES) [that initial infarct had been treated in a different state]. Imaging revealed an acute infarct in the posterior limb of the right internal capsule without hemorrhagic transformation and an acute punctate infarct in the right parietal subcortical white matter with corresponding diffusion restrictions, as well as remote evidence of subcortical chronic diffuse microhemorrhages (). The Psychiatry Consultation & Liaison service was consulted on hospital day 2 after the patient reported, “I want to strangle myself with my oxygen cord.”\nOn initial evaluation, the patient reported history of anxiety treated previously by her primary care physician (PCP). She reported she had been frustrated with her medical condition but really did not intend to harm herself. She reported fluctuating mood since her initial stroke and had “good days and bad days.” She denied prior history of inpatient or outpatient psychiatric care or prior suicide attempts. She was oriented to person and place, but not time, was able to state the days of the week forwards, but not backwards, and endorsed visual hallucinations during her hospitalization. This presentation was felt to be consistent with delirium, and she was started on quetiapine 25 mg.\nFollowing a six-day medical admission, the patient was discharged to the acute inpatient rehabilitation unit housed within the hospital. Extensive diagnostic studies did not reveal an underlying etiology for the strokes, which were thought to be due to uncontrolled hypertension.\nPsychiatry was reconsulted by the rehab physicians for management of problematic behaviors. The patient exhibited ego-dystonic behaviors for which she would later apologize including repeatedly climbing out of bed, shouting for nursing assistance without clear need for help, shoving her fist into her mouth to induce vomiting, and periodic, purposeless screaming. These behaviors were disruptive to staff and other patients on the unit. While initially conceptualized as residual hyperactive delirium, her behaviors persisted and continued testing for underlying causes of delirium including electrolyte derangement, occult infection, new or evolving cerebrovascular event, or excess medication burden which were unrevealing\nAfter 60 days of acute rehab, she had reached maximal benefit of that intervention and continued exhibiting behaviors incompatible with nursing home disposition. The patient was then transferred to the university's geriatric psychiatry inpatient unit on an involuntary mental health commitment for behavioral management.\nIneffective medication trials prior to transfer included quetiapine (25 mg at bedtime and 25 mg several times daily as needed), mirtazapine (7.5 mg at bedtime), olanzapine (initial trial of 2.5 mg at bedtime and 2.5 mg several times daily as needed and a second trial of 15 mg and 2.5 mg several times daily as needed), buspirone (15 mg TID), divalproex (initial trial of 750 mg at bedtime and a second trial of 500 mg TID with lactulose and levocarnitine for hyperammonemia), melatonin (9 mg at bedtime), propranolol (40 mg QID), trazodone (150 mg at bedtime), gabapentin (200 mg several times daily as needed), dextromethorphan (20 mg BID, given as Robitussin), and clonazepam (0.5 mg AM and 1 mg PM).\nThroughout this period, the patient remained intermittently apologetic for her behaviors. Orientation was typically attuned to person, sometimes place, and generally not to month or year. She consistently denied depressed mood, anxiety, visual hallucinations, auditory hallucinations, paranoia, suicidal ideation, or homicidal ideation. Thought process remained concrete and perseverative with limited spontaneous speech output and paucity of thought content. Language remained intact without evidence of aphasia. Recent and remote memory were difficult to assess formally due to behavioral disturbance, but she had difficulty remembering recent details of her hospital course and remote details of her life prior to moving to her current city. She required staff assistance for completion of toileting, dressing, and feeding. She had deficiencies in executing complex motor tasks, such as getting out of bed, and was frequently found diagonal in bed with a limb tossed over the side-rail. These deficiencies were in excess of the residual motor effects of her strokes and suggestive of alterations in visuospatial skills, executive function, and planning. Her aberrant vocalizations did not appear goal-oriented and were not ameliorated by staff presence. This presentation persisted and was thought to represent a new cognitive baseline meeting diagnostic criteria for major vascular neurocognitive disorder with behavioral disturbance.\nNonpharmacological strategies including music, sensory stimulation, one to one time with staff, and frequent repositioning were tried without improvement in her symptoms. Additional ineffective medication trials following transfer to inpatient psych included fluoxetine (60 mg per day), retrial of dextromethorphan with fluoxetine as an enzymatic inhibitor (again to 20 mg BID), retrial of quetiapine (up to 600 mg total per day), haloperidol (5 mg several times daily as needed IM), oxycodone (5 QID), lorazepam (up to 6 mg daily), carbamazepine (200 TID), and chlorpromazine (50 QID). Throughout these trials, the patient continued to exhibit frequent periods of severe psychomotor agitation requiring vest restraint and purposeless screaming alternating with periods of oversedation following medications. Other than providing intermittent sedation, no particular combination of medications proved effective in treating the target symptoms.\nAt this point, having exhausted all reasonable behavioral and pharmacologic options, the inpatient psychiatric team recommended ECT as a last intervention prior to pursuing a palliative approach. Medical Ethics was consulted and felt ECT to be consistent with her previously articulated beliefs and wishes.\nThe patient was formally evaluated by the ECT service and, given her incapacity to consent, a court order was obtained for the procedure. She underwent an acute course of bitemporal ECT using a MECTA Spectrum 5000Q machine. She received methohexital and succinylcholine as anesthetic and relaxant agent, respectively. A dose-titration method was used to determine stimulus intensity. She received treatments at 50% over seizure threshold with the following parameters: pulse width: 1 millisecond, frequency: 20 Hz, duration: 2 sec. Treatments were given three times per week. She was maintained on chlorpromazine (50 mg QID) and lorazepam (1 mg QID) during the treatments. Following the sixth ECT treatment, the patient rarely engaged in purposeless yelling, and remained quiet most of the day, experienced normalization of her sleep wake cycle, but still exhibited purposeless movements and psychomotor agitation requiring a vest restraint at night.\nFollowing the third week of ECT treatments, she was consistently having low scores on the Pittsburgh Agitation Scale (PAS) and had minimal requirements for as needed medications for agitation []. While she still required a vest restraint overnight, her psychomotor agitation had improved dramatically. She resumed feeding herself with her right arm and tolerated pureed foods for the first time in six months. Following an acute course of 16 treatments, ECT was tapered to twice weekly and she started sertraline 25 mg in preparation for further decrease in ECT frequency. She remained stable and was successfully discharged to a nursing home with continuation of ECT as an outpatient. Following the expiration of the original court order for ECT, outpatient ECT was discontinued and the patient's family chose to not pursue a renewal of the order for continued treatment. She received 29 treatments in total. Nursing home staff reported that her behaviors remained in control after stopping ECT and she was thereafter able to return home with her parents. |
After falling off a mountain bike down an incline into some brush, a 49-year-old male mountain biker presented to an outside ED with normal vitals, severe vertigo, nausea, intractable vomiting, profound hearing loss, and tinnitus. A CT was performed, which showed opacification of the ear canal, but did not comment on any abnormalities of the inner ear. The patient was transferred to our facility for further management. On examination, there was a spontaneous right-beating nystagmus and the facial nerve was intact. A tree twig was embedded in the left external auditory canal, obscuring visualization of the tympanic membrane.\nTemporal bone CT demonstrated a linear foreign body projecting from the external auditory canal to the oval window, and an additional, separate small foreign body projecting into the vestibule. Presence of extensive intralabyrinthine air was detected radiographically (). On axial view, air bubbles were seen in the vestibule, posterior semicircular canal, and the scala vestibuli compartment of the cochlear basal turn, as well as in the lateral and superior semicircular canals (). Preoperative audiogram conducted at bedside revealed normal hearing on the right side and moderate-to-severe mixed hearing loss on the left.\nThe patient was diagnosed with a traumatic PLF with extensive pneumolabyrinth due to penetrating temporal bone injury and was taken urgently to the operating room less than one day after his inciting injury. A three- centimeter tree twig was lodged in the ear canal and found to be penetrating the tympanic membrane. Postauricular approach included mastoidectomy and intraoperative assessment of the middle ear ossicles and extent of injury. The long process of the incus was dislocated but still attached to the malleus, the stapes was deeply embedded into the vestibule, and the oval window was completely open but covered by blood clot. All penetrating foreign bodies were extracted. Temporalis fascia was used to seal the oval window and a stapes prosthesis was placed. The tympanic membrane perforation was repaired.\nPostoperatively, the patient had rapid and significant improvement of his vertigo. On physical examination, there was minimal spontaneous nystagmus. A four-week postoperative audiogram revealed a mild-to-moderate mixed hearing loss in the left ear with continued improvement at six months. |
A 22-year-old male with a past medical history of pericarditis and pericardial effusion presented to the ED with the chief complaint of facial swelling, which had been present for the prior three weeks. The swelling was predominantly on the right side of his face and upper lip. He had no history of angioedema, had not started any new medications, and was not aware of an environmental exposure that immediately preceded the onset of swelling. In addition to the facial and lip swelling, the patient reported a rash of the same duration on his chest and shoulders. Additional associated symptoms included decreased exercise tolerance, exertional dyspnea, and a single episode of dark, maroon-colored stool. He denied fever, chills, myalgia, arthralgia, chest pain, abdominal pain, nausea, vomiting, odynophagia, dysphagia, and confusion. He was not aware of any sick contacts and he had not traveled recently. He reported that his family did not have a history of chronic illnesses.\nPhysical examination was significant for a blood pressure of 104/58 millimeters of mercury, a pulse of 96 beats per minute, respiratory rate of 16 breaths per minute, a temperature of 36.8° Celsius, and a pulse oximetry reading of 100% on room air. He was a thin young man who did not appear to be in distress or acutely ill. Bilateral facial edema along with edema of the upper lip was noted (). In addition, his conjunctiva, palms, and soles were notable for pallor. A petechial rash was observed on his upper chest, bilateral shoulders, tongue, and soft palate (). A malar rash was also noted (). The remainder of his examination was normal.\nHis initial ED evaluation included a chest radiograph, electrocardiogram, and laboratory studies. The results of pertinent laboratory studies are listed in the . Given his severe thrombocytopenia and anemia, thrombotic thrombocytopenic purpura (TTP) was considered and an emergent hematology consultation was obtained. A peripheral blood smear demonstrated 1–2 schistocytes per high-power field, which initially raised concern for a microangiopathic hemolytic anemia. As a result, a hemodialysis catheter was inserted and plasmapheresis was initiated while the patient was in the ED. He received a unit of packed red blood cells along with corticosteroids and was admitted to the medical intermediate care unit.\nWorkup revealed a positive immunoglobulin G (IgG) Coombs test. He also had a high titer of antinuclear acid antibody and low C3/C4 complements, indicative of an acute exacerbation of an autoimmune disease. The combination of his symptoms, ED workup, and history of pericarditis and pericardial effusion favored the diagnosis of systemic lupus erythematosus (SLE). Within 48 hours after admission, an A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) level returned with 78% activity and less than 5% inhibitor. This result was not consistent with the diagnosis of TTP, and plasma exchange was stopped. Ultimately, the hematologist diagnosed Evans syndrome as a presenting feature of SLE. |
Eleven years ago, when our patient was a 33-year-old unmarried nulligravida, she developed CCC of the ovary for the first time. At that time, her menstrual cycle was regular and 30 days long, with dysmenorrhea, manifesting as lower abdominal and lumbar pain, persisting for 5–7 days. There was no family or medical history of note. She had visited a local clinic with the chief complaint of lumbar pain, where a right ovarian tumor 8 cm in size that included solid internal components, was identified, and she was referred to the University of Tsukuba Hospital. Transvaginal ultrasound had revealed a right adnexal mass with solid components, 68 mm × 53 mm in size, and an intramural uterine fibroid 23 mm × 27 mm, with no hypertrophy of the endometrium, which measured 2.1 mm. Blood counts and blood biochemistry test results revealed no abnormalities. Her serum CA19-9 level was 45.9 U/ml, CA125 was 22 U/ml, and CEA level was 1.3 ng/ml. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an 80-mm unilocular cystic mass in the right ovary with a papillary protrusion of longest diameter 45 mm. The left ovary was not enlarged, and there was no obvious peritoneal dissemination or enlarged lymph nodes. Surgery, comprising right adnexectomy, left ovarian biopsy, partial omentectomy and uterine fibroid removal, was performed. The right ovary was enlarged to 8 cm in size, and it was removed without intraoperative rupture and with its capsule intact. The left ovary was not enlarged, but was seen to have a small endometrial cyst, which was resected. The absence of peritoneal dissemination and enlarged lymph nodes was confirmed during surgery. The pathological diagnosis was CCC localized to the right ovary and endometriosis was observed in the left ovary, with ascites cytology Class III. The cytology was composed of clusters that include atypical cells having somewhat nuclear enlargement, and it makes a diagnosis difficult to distinguish mesothelial cells from malignant cells. The patient expressed a strong desire to preserve her fertility. Hence, pelvic and para-aortic lymph node dissections were performed via staged laparotomy, which revealed no metastases in any of the 91 pelvic or para-aortic lymph nodes that were removed, leading to a diagnosis of Stage IA CCC. Four courses of postoperative combination paclitaxel (175 mg/m2) and carboplatin (AUC6) chemotherapy (TC therapy) were administered to complete the initial treatment.\nNine years after the initial therapy, MRI during regular 6-monthly monitoring revealed the appearance of a 95-mm polycystic mass with a mural nodule of longest diameter 53 mm in the left adnexal region. Those findings were never seen at the previous MRI. At this time, her serum CA19-9 level was 12.9 U/ml, CA125 was 18.3 U/ml, and CEA level was 0.5 ng/ml, all of which were within normal limits. Disease recurrence in the contralateral ovary was diagnosed, and total abdominal hysterectomy, left adnexectomy and omental biopsy were performed. At the time of surgery, the left ovary was enlarged to 95 mm in size, and it was removed intact without intraoperative rupture of its capsule. The internal lumen of the tumor contained several milky-white mural nodules extending around 15 mm into the cavity (Fig. ). The histological diagnosis was CCC, but in addition to the possibility of recurrence, it was also considered that the tumor might have developed de novo, and the fact that clear cell adenofibroma (CCAF) was also present (Fig. ) suggested that this might have provided the genesis for its development. In addition, there were no endometriotic lesions in the non-solid cyst wall, and it only consists of fibrous membrane. The tumor was localized to the left ovary, and since ascites cytology was negative, it was diagnosed as Stage IA disease. The treatment was completed with four courses of postoperative TC therapy.\nTwo years after the completion of treatment, the patient is continuing outpatient monitoring with no sign of recurrence to date. |
A 72-year-old right-handed male presented with 17 years of running difficulty. Before the onset of symptoms, he was running 70–80 miles per week and competed in 55 marathons over 25 years. His initial symptom was an inability to slow down when running downhill, leading to falls. His symptoms were soon apparent when running on level ground. He felt difficulty lifting the right leg and advancing it forward. He frequently hyperextended the knee with load bearing. Touching the right thigh provided temporary relief. Within a few years, he was no longer able to run, and even walking became difficult. He used walking poles, which seemed to help him move the right leg faster. Fortunately, he successfully switched over to long-distance bicycling. Per his medical records, genetic testing for DYT-1 and DYT-2 was negative. He received three sessions of BoNT prior to coming to NIH. In the first session, he received 150 units into the gastrocnemius and 50 units into the soleus. A month later, he received 25 units into the vastus medialis, 25 units to vastus lateralis, and 50 units to the rectus femoris. Later that year, he received 30 units to the extensor digitorum longus, 30 units into the extensor hallucis longus, and 75 units into the tibialis anterior. He reported no benefit with the injections. In addition to these symptoms, he had evidence of a mild ataxia on balance testing. MRI brain revealed mild atrophy of the cerebellar vermis. There were no other signs of ataxia on examination and he had no relevant family history. He also had signs of a length dependent polyneuropathy. He did report of a prior history of heavy drinking.\nHe was referred to the NIH for formal gait evaluation. Kinematics revealed excessive right plantar flexion in early midstance (a) and excessive knee extension peaking in late midstance (b). There was also less right knee flexion during swing compared to the left. EMG revealed phasic bursts of the right gastrocnemius at initial contact and coactivation with the right tibialis anterior (c). The right vastus lateralis had increased tonic activity from midstance through swing phase, which was not present on the left side (d, gold box). The left gastrocnemius and tibialis anterior displayed prolonged tonic activation, but since kinematics of the left ankle were normal this was considered compensatory. The primary dystonia was thought to be in the right plantar flexors, with a secondary dystonic movement in the right knee extensors. Knee hyperextension upon load bearing was suggestive of pathologic “plantar flexion-knee extension coupling” as a contributing factor to the hyperextension of his knee, though we still thought that the quadriceps were dystonic based on the gait study. Normally, the quadriceps contract in a compensatory action to prevent buckling of the knee when striking the ground with the heel. In the case of excessive plantar flexion, as seen in spasticity, the forefoot strikes the ground first and bears most of the load, resulting in knee hyperextension when the quadriceps activate []. The patient was provided with an ankle-foot orthotic to prevent plantar flexion which did reduce the amount of knee hyperextension. BoNT was tried again, with injections into the gastrocnemius and tibialis posterior, which were thought to be the primary dystonic muscles causing plantar flexion. The vastus lateralis, medialis, and rectus femoris were also targeted with the goal of reducing knee hyperextension (). He returned to the NIH almost a year later, not recalling a significant benefit from the injections.\nOn his follow-up visit, he mentioned a curiosity when performing leg raises while lying supine, and showed us in the clinic. At first, his legs were symmetric with normal flexion at the hips and knees, but after a few repetitions, the left hip flexed more than the right, while the right knee went into extension. This was not initially recognized, but on review of his kinematic studies, indeed it revealed excessive left hip flexion in early stance phase (e). There was no EMG recording done of the hip flexors at the time of the study. He did not return for BoNT to be attempted in the hip flexors.\nThe elusive benefit to therapy in this instance was likely due to a combination of factors. First, this was a challenging case. He had a long-standing history of dystonia that was likely bilateral, (right knee extension, b, and left hip flexion, e) with multiple compensatory mechanisms that evolved over 17 years. Second, inconsistent follow-up made the fair assessment of treatment effect and the possibility of treatment optimization with BoNT difficult. Had there been an opportunity for further therapy, we would have considered targeting additional muscles, such as the left hip flexors. Lastly, he had signs of mild ataxia detected on balance testing and mild peripheral neuropathy, presumably related to a prior history of heavy drinking, that might have complicated his response to therapy. |
A seven-month-old male born full-term via cesarean secondary to failure to progress presented for a well-child visit. Family history was only notable for a maternal grandmother who reportedly had a "hole in her heart” that closed when she was young.\nHis vital signs were within normal limits. His cardiovascular exam was notable for a harsh holosystolic murmur noted along the left sternal border with radiation to the back. S1/S2 was normal with regular rate and rhythm. There were no S3, S4 rubs, or gallops. Pulses were 2+, and capillary refill took < 3 seconds. Extremities demonstrated no clubbing, cyanosis, or edema, and no signs of volume overload.\nA 12-lead electrocardiogram demonstrated sinus rhythm, normal axis, regular intervals, and voltage appropriate for age. The echocardiogram did not reveal any detectable ischemia or wall motion abnormalities. A patent foramen ovale and a proximal right coronary arterial ectasia measuring 22 mm were detected. The ectasia demonstrated an appreciable coronary cameral fistula from the distal right coronary artery to the right ventricle. As a result of the prominent pathway from the right coronary artery feeding into the right ventricle, an abdominal aortic Doppler ultrasound demonstrated a reversal of blood flow during the diastolic phase of the cardiac cycle, most likely due to drainage from the right coronary artery fistula into the right ventricle. Finally, there was a restrictive posterior-inferior muscular ventricular septal defect, with a pressure gradient between 60 to 65 mmHg across the defect. Despite the flow reversal and ventricular septal defect, the right ventricular pressure and volume remained within normal limits, with close monitoring for the fistula recommended.\nUpon two months follow-up, the patient remained asymptomatic without any signs of failure to thrive. Vital signs were within normal limits. The cardiovascular exam demonstrated a regular rate and rhythm, normal S1 and S2, 2+ pulses, and a capillary refill of < 3 seconds. A 2/6 short systolic murmur along the left lower sternal border and a harsh continuous murmur along the left sternal border with radiation to the back were notable.\nA repeat 12-lead electrocardiogram demonstrated normal sinus rhythm, normal axis, intervals, and voltage appropriate for age. No detectable ischemia was present. No pathologic Q waves or ST-segment changes were noted. A repeat Doppler echocardiogram again demonstrated proximal right coronary arterial ectasia (measuring 23 mm) with a cameral coronary fistula from the distal right coronary artery to the right ventricle. However, there was now no appreciable diastolic flow reversal in the abdominal aortic Doppler. The restrictive posterior-inferior muscular ventricular septal defect remained, demonstrating a gradient of 65 mmHg across the defect, suggesting normal right ventricle pressures and no indication of right heart failure or cor pulmonale. A computed tomography angiogram confirmed a tortuous mid-segment right coronary artery fistula draining into the right ventricle. The left coronary artery demonstrated no abnormalities. No intervention was indicated for this patient since the aortic steal ceased, and that there were no clinically appreciable findings despite a persistent coronary fistula. The patient has continued to do well upon 30 months of follow-up, with no signs of right ventricular volume overload, cor pulmonale, or congestive heart failure. |
A 5-year-old previously healthy male presented with pain, difficulty in swallowing and right-sided neck swelling. Four days prior to presentation, he sustained a pencil scratch trauma to the right oropharynx secondary to a fall on the bed whilst playing with a pencil in his mouth. He bled moderately and was taken to nearby emergency room where the bleeding stopped spontaneously. Examination at that time showed minor superficial mucosa trauma. The parents were reassured, and the patient was discharged home on Tylenol for pain. He drooled for a few hours but returned to baseline activity level on the same day. Three days later, he complained of right-sided neck pain associated with reduced oral intake. The parents noticed right-sided jaw fullness and swelling. He was sent to emergency room the next day upon the recommendation of his pediatrician. In the emergency department, he looked unwell with fever of 38.9°C, right neck and jaw swelling with multiple right cervical lymphadenopathy, the largest measuring 3 cm, with overlying erythema. He also resisted neck movements due to pain. The rest of the examination of oropharynx, ears, and nose as well as other systems were within normal limit.\nComplete blood count showed white cell count of 13,000/mm3, with 84% neutrophils and 10% bands. However, blood culture was negative. Soft tissue radiograph showed asymmetric right neck swelling, deviation of airway to the left, without evidence of a foreign body. Computerized tomography [CT] scan of neck showed a lesion with air-fluid interface, consistent with an abscess, in the right parapharyngeal space with surrounding inflammatory changes. The inflammation extended into the parotid gland, carotid sheath, the masticator muscles, as well as the retropharyngeal space, with mild mass effect noted on the airway. The upper part of internal jugular vein could not be visualized, possibly due to compression or thrombosis of upper third of the internal jugular vein. Further imaging was done with magnetic resonance angiography and venography [MRA/MRV] which confirmed the above findings and also showed the absence of blood flow in the upper third of internal jugular vein due to compression from the adjacent soft tissue mass and abscess.\nThe patient was treated with intravenous ceftriaxone and clindamycin for five days and then discharged home on oral clindamycin to complete a 14-day course of antibiotics as per the recommendation of an infectious disease consult. Repeat CT scan of the neck on day 7 of treatment showed subtle residual asymmetry of soft tissue of neck without abscess formation and normal anatomy of the rest of tissues. |
A 12-year-old girl presented with grade IIb right-sided hemifacial microsomia. She had already undergone right ear reconstruction for microtia and maxillary driven simultaneous maxillomandibular distraction[] [].\nWe initially performed a computer-simulated surgery using image processing software (Mimics, Materialise NV, Leuven, Belgium) and a substance model (Kezulex, Ono and Co., Ltd, Tokyo, Japan) as a preoperative simulation. We planned the osteotomy lines to avoid damaging the nerve pathway. The simulation of surgery indicated that mandibular body on the affected side should be distracted 15 mm to the anterior and 10 mm inferiorly to improve facial symmetry. We proceeded with the surgery based on simulation [].\nThe procedure was performed under general anesthesia with nasotracheal intubation. Incisions were made along the mandibular rim and at the base of gingivolabial groove of canine tooth on right side to canine tooth on left side. Mucoperiosteal detachment was performed from the chin region to mandibular body while avoiding damage to mentonian nerve. The muscle insertion on the mandible's internal cortical bone was preserved to ensure vascularization of osteotomized segment, thus retaining effective traction of genioglossus muscle.\nFollowing these incisions, an osteotomy was performed according to preoperative simulation. Thereafter, two sets of the three-dimensional NAVID system were fixed to ramus and free mandible on the affected side. The front of the system was equipped with a distraction vector in inferior direction, and the rear of the system was equipped with a distraction vector in anterior direction []. The rods of the system were inserted through a small skin incision at the mentum, and hinge plate was attached on unaffected side and served as a pivot point for rotation. The wound was then closed.\nAfter 7 days, the distraction was initiated at a rate of 1 mm per day. This process was continued until desired clinical endpoints were achieved; the process is summarized in . As the distraction continued, there was a change in the angle at which two devices crossed and free mandible extended towards the anterior direction and inferiorly because neck of NAVID system could move in three dimensions. Towards the end of distraction period, we performed a 15 mm distraction with frontal device and a 22 mm distraction with rear device.\nWhen the distraction was completed, externally exposed rods were cut in very close proximity to the skin. After a 3-month consolidation period, the devices were removed under general anesthesia. Both the intraoperative view and preoperative computed tomography (CT) image indicated presence of osteogenesis, and showed a symmetrical improvement of mandibular contour compared with preoperative profile []. |
A 62-year-old Asian woman presented acutely to the emergency department with a 1-day history of colicky epigastric pain and postprandial vomiting. She had been tolerating only liquids rather than solid food for 2 months. There was no history of weight loss, but she did report early satiety and loss of appetite.\nThis woman had a history of peptic ulcer disease over 20 years ago in Kenya. It had led to GOO requiring truncal vagotomy and gastrojejunostomy. In order to investigate the cause of her dysphagia and loss of appetite, she had undergone an upper gastrointestinal endoscopy 3 weeks before this admission. This showed inflammation and oedema at the anastomotic site of the gastrojejunostomy, but no evidence of obstruction or stricture (Figure ). She was then prescribed daily omeprazole, which was the only medication she was taking on admission.\nThe patient was clinically dehydrated on examination. She had a very thin body habitus. Her abdomen was soft, but mildly tender over her epigastrium. Succussion splash was demonstrated and a 10 cm × 8 cm mass was palpable just right of the umbilicus. Bowel sounds were scanty. There were no clinical signs for upper gastrointestinal bleeding.\nHer admission blood profiles were essentially unremarkable. There was no biochemical evidence of fluid shifts or dehydration. Plain abdominal radiograph did not show any diagnostic features. However, her erect chest radiograph showed an air-fluid level within a dilated stomach (Figure ).\nIn view of the examination and chest radiograph findings, she had a nasogastric tube and urinary catheter inserted for gastric decompression and urine output monitoring, respectively. An urgent contrasted computed tomography of the abdomen was arranged. Meanwhile, the nasogastric tube successfully prevented further vomiting, and there was little drainage from it. She was commenced on intravenous omeprazole and fluid therapy.\nThe abdominal computed tomography (Figure ) showed a fluid filled, non-dilated stomach. The anastomosis between the proximal jejunum and body of the stomach was shown to be patent. The afferent loop was not dilated but the efferent loop was dilated. Just past the midline, approximately 20 cm from the anastomotic site, there was a change in calibre of the bowel with the jejunum becoming significantly narrowed. The bowel distal to this site was collapsed. The proposed diagnosis was a stricture at the site of the gastrojejunostomy, but the exact cause was uncertain.\nThe patient provided consent for expedited laparotomy and relief of obstruction. Intra-operatively, the jejunum was found to be dilated from the duodenojejunal flexure to a large bolus obstruction. A conical mass suspicious of a bezoar was found measuring 10 cm in length, situated 20 cm beyond the gastrojejunostomy. The small bowel distal to this site was collapsed. Attempts to break up this hard bolus mass externally were unsuccessful. The bezoar eventually had to be removed in whole via an enterotomy. Careful examination confirmed that it was indeed a phytobezoar (Figure ).\nThe patient had an uneventful recovery and was discharged home 1 week after surgery. Before discharge, she was seen by the dietician with regard to different types of fibre diet. She was also advised on the importance of longer mastication of food. |
A 62-year-old Chinese woman presented to the First Hospital of Jilin University with complaints of abdominal pain, vomiting, and bloody stool for 24 hours. On admission, her general condition was good. Her temperature was 37.1 °C, respiratory rate was 18 times per minute, and heart rate was 80 beats per minute. She did not have cutaneous or scleral icterus. No superficial tumescent lymph nodes were observed. A physical examination showed distended abdomen with no stomach outline, peristalsis, and varicose veins. Abdominal breathing was slightly limited. Abdominal tenderness could be detected in her right abdomen accompanied by rebound tenderness and muscle tension. A palpable mass could be detected in her right lower abdomen. Liver, spleen, and gallbladder could not be touched below the costal arch. Murphy’s sign and shifting dullness were negative. Bowel sound was found to be approximately 7 beats per minute with gurgling. A neurological physical examination had no significant findings. A physical examination of her heart and chest was normal. She was a housewife in a small city with no special family history. She had a history of hypertension for 10 years, and the use of captopril helped maintain a satisfactory level of blood pressure. She denied a history of hepatitis, tuberculosis, and diabetes. Also, no history of drug allergy, surgeries, tobacco smoking, and long-term alcohol consumption was reported. The results of complete blood count, liver function, and blood biochemical indexes are shown in Tables and . The results of coagulation markers of hepatitis B virus, hepatitis C virus, syphilis, and human immunodeficiency virus were found to be negative. Abdominal computed tomography (CT) was performed, which showed some intestinal canals and mesentery entering into the right colon. The CT scan showed a target sign suggesting intussusception in her right abdomen (Fig. , ).\nSurgical exploration revealed that the ICD entered her ascending colon with edema (Figs. , and ). The diverticulum was suspected of blood supply deficiency. No obvious abnormalities were found in the remaining colon and rectum. Intussusception was diagnosed during the operation. Right hemicolectomy was performed under laparoscopy.\nA histopathological examination showed that the ICD was on the side of ascending colon, causing ileocolonic intussusception with intestinal necrosis (Fig. ). The diameter was approximately 3.8 cm.\nCefmenoxime hydrochloride injection (2 g) was used before and after the surgery to prevent infection. Our patient quickly recovered and was discharged 7 days after the surgery without any complications. She had a telephonic follow-up 10 months after discharge from our hospital. She had no apparent symptoms after the surgery. Serological and imaging examinations were performed in a local hospital, and the results were negative. |
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