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A 48-year-old female presented with swelling in the frontal region of the skull since 2 years and another swelling in parieto-occipital region since 4 months. Both the swellings over skull have been painless and gradually progressive in size, thus the delay in presentation. No significant past medical history. On examination, swelling in frontal region measured 18 cm in sagital and 13 cm in coronal directions and 7 cm in vertical dimension. The veins over swelling were distended and it was pulsatile in nature, firm in consistency and fixed to skull. The skin over the swelling was stretched but otherwise normal. Left orbit appeared distorted by the swelling but vision was normal []. The second swelling was located in parieto-occipital region right side and measured 3×4 cm pulsatile in nature and firm in consistency []. Incidentally a thyroid swelling was noted involving mainly right lobe around 3×2 cm, firm and not associated with any pressure symptoms []. Patient did not have any neurological deficit or psychological illness. The patient was clinically euthyroid with normal general physical, systemic, and neurological examination. Routine blood tests and thyroid function tests were normal. Ultrasound study of neck revealed 2 × 1.5 cm neoplastic solid nodule in right lobe of thyroid. Rest of the thyroid gland and neck was normal. X-ray of skull showed lytic lesion in frontal and occipital region of skull. Contrast enhanced computed tomography (CT) demonstrated a 13 × 18 cm size expansile osteolytic lesion in the fronto-parietal region on both right and left side of anterior half of skull. The lesion was extending intracranialy and causing compression of the underlying fronto-parietal lobes on both sides. Similar expansile osteolytic lesion of smaller size are seen in the occipital region on both sides largest measuring 4×3 cm on right side. On postcontrast study, these lesions showed significant enhancement suggesting increased vascularity and also areas of necrosis were present at centre []. Fine needle aspiration cytology (FNAC) from thyroid nodule was reported as follicular neoplasm. FNAC from skull swelling obtained was stained with Leishman stain. Smears showed many syncytial sheets of thyroid follicular cells having monotonous enlarged, hyper chromatic nuclei, and microfollicles containing colloid []. Patient was advised total thyroidectomy and radioiodine therapy but was not willing, hence was administered supraphysiological dose of thyroxine. Till date the patient is on follow-up and able to carry out her routine activities. |
A 59-year-old man with hypertension and a family history that included two brothers who died of an aortic aneurysm was brought to the Emergency Department after falling from his bicycle. He reported abdominal pain since one day. On examination, he was pale and had a pulse rate of 64/min, a blood pressure of 70/50 mmHg, a body temperature of 36°C, and a respiratory rate of 16 breaths/min. He had abdominal tenderness with muscular rigidity, especially in the upper abdominal region. A full blood count showed a haemoglobin level of 6,4 mmol/L, while all other laboratory tests were within the normal range. A chest X-ray showed normal lung fields and no pneumoperitoneum. As a vascular incident was feared, an abdominal ultrasound revealed free abdominal fluid around the liver and spleen without an aortic aneurysm. An abdominal computed tomographic (CT) demonstrated fluids in the omental bursa with a normal aspect of the pancreas but was unable to point to the origin of the free fluid. After resuscitation using standard techniques, the patient was transferred to the operating room under the diagnosis of intra-abdominal haemorrhage of unknown origin. During laparotomy, three litres of blood were obtained from the abdominal cavity but no active bleeding was found. Inspection of the omental bursa revealed a hematoma surrounding the pancreas. The patient was packed using several large volume gauzes and admitted to the intensive care unit. The next day, these gauzes were removed and again no active bleeding was seen. The next four days were uneventful. However, on the fifth day, the patient developed acute severe abdominal pain. An abdominal computed tomographic angiography (CTA) revealed a 14 mm splenic artery aneurysm () as well as a 12 mm aneurysm of the coeliac trunk. In retrospect, on the first CT, a 9 mm focal contrast-filled dilatation of the splenic artery could already have been identified in a large hematoma (). This was initially missed.\nA ruptured splenic aneurysm was now considered the source of the abdominal bleeding and the patient underwent a coil embolisation of the proximal splenic artery using a femoral approach (). The splenic artery appeared very delicate and reacted with intense vasospasm following minimal movement of the endovascular catheter tip. Fear of arterial dissection together with the tortuous aspect of the distal splenic artery urged the interventionalist to refrain from coil embolisation at the distal end of the aneurysm. Flow in the distal part of the splenic artery was absent after placement of 8 coils proximal to the aneurysm. During the endovascular procedure, it was noted that the aneurysm had grown further to 18 mm. Although initially stable, some 24 hours after the embolisation, the patient again experienced severe abdominal pain and became hemodynamically instable. After cardiopulmonary resuscitation, a CTA was repeated and revealed recanalization of the splenic artery without migration of the coils. An urgent laparotomy was performed with removal of the spleen and the tail of the pancreas, encountering the ruptured aneurysm. The patient recovered uneventfully and was discharged from the hospital 7 days later. He will undergo vaccination and follow-up for his coeliac trunk aneurysm. |
A 62-year-old woman (Figure , II-3) was admitted to the hospital for recurrent partial headache with weakness of one side and aphasia for about 45 years. In her first attack, the patient suddenly experienced an aura with visual disturbances which she described as increasing scotomata in the bilateral visual field. After a few seconds, the patient developed a serious headache, mainly located on the left side. After a few minutes, she presented a paralysis of the right side and speech difficulties accompanied by dizziness and vomiting. These symptoms resolved after about 2 h. After this initial onset, she had an attack nearly every 4–5 years, and the clinical presentations of her attacks were always similar to the first one. The duration of the aura symptoms and the migraine was typically 1–2 h but sometimes the migraine could last up to 4 days. Sometimes headaches occurred before the hemiplegia and aphasia. Each headache was accompanied by dizziness and vomiting but without loss of consciousness. In most attacks, this patient experienced additionally a flushing of the neck and face and felt that the skin temperature of this affected area was increased, but the temperature was never measured. These symptoms may be related to an extracranial vasodilation when a migraine attack occurred. She did not undergo regular treatment except for simple analgesics as a symptomatic therapy. Recently, her condition aggravated as the frequency of attacks increased from once every 4–5 years to once every 1–2 weeks which had a serious impact on her everyday life. Therefore, during a severe migraine attack, she visited our hospital. We reviewed her family history, and we found that three other subjects, her mother, brother, and nephew, had similar clinical symptoms (Figure ). Their presentations are as follows:\nThe proband's mother (Figure , I-2) died of uremia at the age of 72. According to her husband and children, she reported typical hemiplegic migraines since an age of 14 years with five attacks per year on average. The aura symptoms were similar to those in the proband, including bilateral visual symptoms (scotomata), speech difficulties, and hemiparesis.\nThe proband's 55-year-old brother (Figure , II-1) had first at the age of 15 headache attacks with nausea, vomiting, visual field defects, and one-sided motor weakness. Usually, these attacks last 5 h. The disease presentation was progressive with age.\nThe proband's nephew (Figure , III-1), a 25-year-old fitness coach, had first headache attacks with visual symptoms (scotomata) and lateralized motor weakness at the age of 13. Each attack lasts about 20 min.\nAfter admission, her neurological examinations were unremarkable and brain magnetic resonance imaging (MRI) and Magnetic Resonance Angiography (MRA) showed no meaningful abnormalities (Figure ). Thus, the suspected diagnosis was transient ischemic attack (TIA). During her hospitalization, the patient had several migraine attacks that were characterized first by visual symptoms, then aphasia and right limb paralysis 10 min later, and finally severe headaches after 20 min. At that time, the neurological examination revealed: no loss of consciousness, motor aphasia, muscle strength 2 in the right limb, and normal findings in the examination of the residual nervous system. After about 1 h, the symptoms of the aura were relieved, while the headache lasted for about 1 day. However, the symptoms were not relieved after dual antiplatelet aggregation treatment, and transthoracic echocardiography and carotid ultrasound failed to identify any underlying cerebrovascular etiology. After careful consideration of all aspects, she was diagnosed with hemiplegic migraine. So, we conducted a genetic test on the patient and found a heterozygous point mutation (c.4495T>C) in exon 26 of the SCN1A gene. This mutation caused amino acid 1499 to change from phenylalanine to leucine (p. Phe1499Leu), which may cause the disease by affecting the SCN1A protein function.\nTo establish the diagnosis, we performed a genetic test on those family members to analyze for the presence of mutations in genes including CACNA1A, ATP1A2, and SCN1A related to FHM. We only found a gene mutation in SCN1A, but this mutation was detected in all affected subjects in this family (Figure ). Therefore, this patient was diagnosed with FHM3. She was discharged after receiving a health education on migraine attacks, which suggested her staying away from stress, bright lights, sleep disturbances, physical exertion, and alcohol consumption because these have all been reported as trigger factors in FHM (). Upon being discharged from the hospital, she had intermittently taken flunarizine capsules and rizatriptan benzoate tablets to prevent and control migraine attacks. After 6 months of follow-up, the efficacy of the drug was uncertain, because the frequency of headache attacks was not adequately reduced. After the low efficacy of her medication became clear, we consulted again the literature and consider now a trial with lamotrigine or acetazolamide (). |
A 64-year-old woman underwent total knee arthroplasty for degenerative joint disease of the right knee in 2009. We used a posterior stabilizing type TKA (Balanced Knee System, Japan MDM Inc., Tokyo, Japan) with patellar resurfacing. We performed normal parapatellar approach and fixed the implants with bone cement. The standard rehabilitation included range of motion, muscle strengthening, and walking exercise under full weight bearing. Rehabilitation started from the first postoperative day.\nHer condition was well through the first six months after the surgery. She had no pain, and the range of motion of the knee was full extension to 120-degree flexion. She was feeling slight and occasional pain in her knee while walking from 2010, approximately six months after the surgery. She had no inflammatory symptoms, and the radiographs of her right knee were normal without loosening or osteolysis at that time. Since the knee pain was tolerable and was not getting worse, we continued the outpatient follow-up once a year.\nShe had severe knee pain and came to our hospital again on March 2017, 8 years after the surgery. She was limping due to severe lateral knee pain localized at the iliotibial ligament. The pain increased when she walked and also increased when she extended her knee, as well as flexed her knee from full extension. The range of motion was from full extension to 130-degree flexion without any catching or clicking. No redness or swelling was observed. She did not have any numbness on her leg, but she felt pain on the lateral side of her shank when we hit an iliotibial ligament at the point around about 3 cm proximal from a fibula head. The strength of the tibialis anterior and the extensor hallucis longus was weak. The MRI of her lumbar was normal (). Relatively, a large fabella with the size of 2 cm was found in the radiograph of her right knee (). The radiographs were normal, and no loosening of the implants was observed. Finally, from these clinical and image features, we diagnosed her with a fabella syndrome and determined excision of a fabella (fabellectomy). The operation was performed using a posterolateral approach between the iliotibial tract and the biceps femoris. Macroscopically, the peroneal nerve was pushed by the fabella located just near the nerve (). A posterior portion of the femoral implant was located under the fabella. The size of the fabella was 20 mm along the major axis, with osteophyte and deformation of the cartilage confirmed. Subjective symptoms resolved immediately after the surgery. At 1 month after the surgery, the range of motion was 0-130 degrees and the VAS score improved to 10 mm. She has no recurrence of snapping or pain on the posterolateral aspect of the knee. |
A 55-year-old Caucasian woman with a 5-month history of abdominal pain and vomiting was diagnosed as having a probable high-grade ovarian malignancy with a large volume of peritoneal disease. She had a prior ultrasound of her pelvis which demonstrated a 17 cm large irregular solid vascularized mass in her right ovary. Her comorbidities included obesity (body mass index of 33), asthma, and she had previously undergone a laparoscopic cholecystectomy. There was no significant family history. She did not smoke tobacco and she drank alcohol occasionally. She was scheduled for debulking surgery; however, she presented to our emergency department with worsening abdominal pain, vomiting, and diarrhea. A physical examination showed dry mucous membranes, capillary refill < 3 seconds, and jugular venous pressure of 4 cm. Her chest was clear on auscultation with dual heart sounds. Her abdomen was distended with generalized tenderness but no guarding or signs of peritonism. Bowel sounds were present. A computed tomography (CT) scan of her abdomen and pelvis was performed which demonstrated the large right ovarian tumor, peritoneal tumor deposits, and ascites. There was extrinsic compression of her sigmoid colon due to the tumor without radiological signs of large bowel obstruction. There were no other abnormalities of her bowel. She received intravenously administered fluids for rehydration, anti-emetics for nausea, and intravenously administered morphine for abdominal pain. She remained overnight in our emergency department for treatment. She was reassessed the following morning after resolution of her symptoms and was found to be hemodynamically stable and subsequently discharged home.\nShe re-presented 1 week later with similar symptoms and was admitted to hospital for further management. Following multidisciplinary discussion, she was recommended for neoadjuvant chemotherapy prior to surgical debulking. An urgent core biopsy of the mass confirmed the likely diagnosis of ovarian malignancy. This biopsy indicated a high-grade serous adenocarcinoma. The histology and CT findings were consistent with an International Federation of Gynaecology and Obstetrics (FIGO) stage III ovarian cancer. She was appropriately counselled as to the benefits and risks of chemotherapy prior to commencing treatment.\nShe was commenced on a first cycle of the commonly used platinum-based two-drug chemotherapy regime of paclitaxel and carboplatin []. The dose prescribed was a three weekly cycle of paclitaxel 100 mg/m2 and carboplatin 385 mg/body to achieve area under the curve (AUC) of 5 using the Calvert formula. The results of her pre-chemotherapy blood tests were within acceptable ranges. Specifically, her white cell count (WCC) was 10.1 × 109/L (normal 4–11 × 109/L) and neutrophils were 8.5 × 109/L (normal 1.5–8 × 109/L). She reported feeling better 1 day after chemotherapy. However, 3 days following the commencement of chemotherapy, persistent diarrhea developed. Stool cultures were negative for stool pathogens including Clostridium difficile. On the sixth day post-chemotherapy, she became febrile and acutely unwell with severe abdominal pain. Blood tests at the time revealed a WCC of 0.6 × 109/L and neutropenia of 0.1 × 109/L. A repeat CT of her abdomen and pelvis showed a thickened descending colon and rectal pneumatosis with perforation into the mesorectum (Figs. and ). Blood cultures were positive for Escherichia coli and meropenem was commenced. She was transferred to our intensive care unit for hemodynamic support due to septic shock. A surgical assessment was conducted but immediate surgical intervention was deemed inappropriate due to high predicted mortality. She was administered granulocyte colony-stimulating factor but her white blood cell counts did not improve and her respiratory function deteriorated. She died 9 days after the administration of the first dose of chemotherapy. An autopsy was not performed due to the wishes of our patient’s family. |
A 6-hour-old female neonate born from a 22-year-old Para 1 mother after term pregnancy. The newborn had protrusion of mass per vagina since birth; she also has a defect at the lower back with no discharge since the time of birth. Additionally the mother has noticed deformity on both legs and feet which barely move. The baby was active since the time of delivery and is sucking well and she passed meconium. The mother had two ANC visits and was vaccinated according to the national schedule and she reported the whole course of the pregnancy as uneventful. She did not have any known medical illness and has never taken any medication during the entire course of the index pregnancy except iron folate which was prescribed during the ANC visit.\nShe had spontaneous onset of labor and the membrane was ruptured spontaneously intrapartum. She gave birth to a 2330-gram female neonate after 5 hours of labor at a local health center. Baby had good APGAR score. Health care providers at the health center noticed defect at the back of the baby and referred her to Hawassa University Comprehensive Specialized Hospital (HUCSH) with the diagnosis of spinal bifida.\nDuring the initial evaluation at HUCSH the baby was active, vital signs were in the normal limits, and all neonatal reflexes were intact. There was 4x4 cm pink mass protruding through the introitus, cervical os is noted at the tip of the mass, the external genitalia appears normal, no discharge or bleeding from the mass, and the mass was reducible digitally and increases in size when the baby cries (see ). There was also 4x4 cm defect at the lumbosacral region the major portion of which is covered with skin while the lower edge is open, no discharge from the mass (see ). Additionally the newborn had bilateral club foot deformity (see ).\nOn investigation, the complete blood count was normal, creatinine level was 0.4mg/dl, and transfontanelle ultrasound scan shows mild dilatation of the lateral and third ventricles with an index of “mild hydrocephalus” and abdominal ultrasound was normal.\nAfter obtaining consent from the parents, under aseptic technique, the baby was catheterized, the vaginal mass was reduced digitally, and bandage was applied from the lower abdomen, both buttocks and legs were strapped in the bandage to the level of the mid-thigh leaving an opening at the anal orifice for passage of stool (see ). The bandage was removed after 72 hours at which time the mass was completely reduced and there was no recurrence of the prolapse afterwards (see ). Unfortunately one day after the removal of the bandage the baby started to shoot fever and had difficulty of sucking, on examination she had tachycardia and tachypnea and was febrile, she had depressed reflexes, the lower border of the meningocele got ruptured, her fontanels were bulged, and she was diagnosed with ruptured myelomeningocele and meningitis. The plan was to start her on antimeningitis drugs and repair the spinal cord defect after improvement but the parents insisted and went home against medical advice frustrated by her multiple anomalies. We communicated with the parents after her discharge and were informed that the baby died 7 days after she went home, but there was no recurrence of the prolapse throughout her stay. |
A 44-year-old female was transferred to our emergency department from an outside institution for the management of sudden onset, profuse vaginal bleeding and hematuria. Her history was significant for cervical cancer diagnosed 3 years prior to presentation, and she was post total abdominal hysterectomy and pelvic lymphadenectomy, as well as multiple chemotherapy and radiation therapy regimens. Her history was further complicated by persistent bilateral hydroureter and hydronephrosis for 6 months prior to presentation, likely secondary to radiation ureteritis.\nOn arrival, the patient was in profound hemorrhagic shock and diaphoretic with active vaginal bleeding. Her blood pressure was 74/49 mmHg, pulse rate was 117 beats/min, and respirations were 20/min. A Foley catheter was placed in the bladder, which yielded fresh blood and clots. She was taken emergently to the operating room by the gynecological surgery team for exploration and vaginal packing. Her bleeding seemed to respond initially after vaginal packing; however, profuse bleeding was subsequently noted in the operating room. At this time, the decision was made to take the patient from the operating room to the interventional radiology suite for an angiogram.\nA pelvic aortogram showed active extravasation of contrast from the left external iliac artery into the pelvis, in proximity to the course of the left ureter (). Contrast material appeared to pool centrally in the pelvis toward the vagina (). Given the finding of extensive vaginal bleeding along with hematuria, the diagnosis of arterio-ureteral-vaginal fistula was considered. A 7 mm × 30 mm covered stent (Wallgraft Endoprosthesis; Boston Scientific, Natick, MA, USA) was then placed across the area of extravasation from the left external iliac artery (). A repeat angiogram demonstrated no further extravasation of contrast from the previous site of hemorrhage (). The patient gradually became hemodynamically stable and was observed in the intensive care unit. Hematuria and vaginal bleeding resolved over the next 4 days. She was discharged 16 days later in stable condition without further episodes of hematuria or vaginal bleeding. The patient did not have any recurrence of her fistula for 12 months, after which she was lost to follow-up. |
A 61-year-old female presented to the emergency room with cough and chest pain. A CTA of the chest was performed to exclude pulmonary embolism as part of chest pain workup. The result of CTA was negative for pulmonary embolism. The patient was diagnosed with acute bronchitis and the patient's symptoms of cough and chest pain were resolved after antibiotic treatment of acute bronchitis. On the images of CTA, a 2.0 × 1.7 cm right paratracheal mediastinal mass was noted which appeared slightly hyperdense or showed mild contrast enhancement ().\nDifferential diagnosis for this upper mediastinal mass included an enlarged lymph node reactive to infection or a chronic inflammatory process, sarcoidosis, or nodal metastasis from occult malignancy. The patient underwent an endobronchial ultrasound-guided biopsy of the right paratracheal mass, which was found to be benign ectopic thyroid tissues by histological analysis of the biopsied tissue samples ().\nA 24 hours I-123 uptake and scintigraphic scan were performed for further functional characterization of this ectopic thyroid tissue mass within the mediastinum. The 24-hour radioiodine uptake by residual thyroid tissue in the thyroidectomy bed was measured at 1.5% and no thyroid tissue with I-123 uptake was visualized on the surgical bed, compatible with the patient's history of prior total thyroidectomy 10 years ago. One focus of increased I-123 radioiodine accumulation was identified in the region of upper mediastinum on the planar images of I-123 scan. For further anatomic localization of the focal uptake in the upper mediastinum visualized on planar scintigraphic images, a SPECT/CT was performed using a dual headed Siemens Symbia T2 SPECT/CT camera in a method as previously described []. On SPECT/CT images, the focus of increased radioiodine uptake in the mediastinum seen on planar imaging was localized to the 2.0 × 1.7 cm right paratracheal mediastinal mass visualized on CTA ().\nThe patient had a history of total thyroidectomy for thyroiditis and nodules 10 years ago. Post-total thyroidectomy hypothyroidism was treated with oral administration of 125 to 137 mg Levoxyl daily for one year. Subsequently, the dose of Levoxyl was reduced to 100 mg/daily and the results of thyroid functional tests were normal with a TSH level of 2.63 mIU/L (normal reference range 0.40–4.50 mIU/L) and a free T4 of 1.6 ng/dL (normal reference range of 0.8–1.8 ng/dL) at 4 years after the patient was maintained on 100 mg of Levoxyl daily for treatment of post-total thyroidectomy hypothyroidism. However, TSH level was low at 0.09 mIU/L and a free T4 level was high at 2.58 ng/dL when a thyroid functional test was performed at 6 days after mediastinal ectopic thyroid tissue was diagnosed with histological analysis of the biopsied tissue samples. In preparation for I-123 SPECT/CT, Levoxyl was stopped for 4 weeks and the patient developed symptoms of hypothyroidism (fatigue, constipation, and hair loss). In view of possible functional activity of benign mediastinal ectopic thyroid tissue confirmed by I-123 SPECT/CT, a reduced dose of 75 mg of Levoxyl daily was prescribed for this patient upon completion of I-123 SPECT/CT. One year later, the results of thyroid functional test were normal with a free T4 level 1.4 ng/dL and a TSH level 2.11 mIU/L. Follow-up CT of chest one year after I-123 SPECT/CT revealed no significant interval changes of the size and morphology of the ectopic thyroid tissue, supporting a conservative management without rebiopsy or surgical resection of the ectopic thyroid tissue within the upper mediastinum. |
We present the case of a 45-year-old Caucasian man suffering from psoriasis for more than 20 years at the time of the first visit. No relevant comorbidities were found at history collection, apart from benign prostatic hyperplasia, for which he is treated with tamsulosine. He was initially diagnosed and followed-up by dermatologists, who treated him with topical corticosteroids and salicylic acid for skin involvement and systemic corticosteroids for resistant lesions as needed ().\nThe skin component had always been <30% of the total body surface area (BSA). The patient reported distress in personal and social relationships with consequently reduced HRQoL. After ten years, he started experiencing dactylitis of feet and recurrent arthritis of the small joints of hands, shoulders and knees, together with inflammatory morning stiffness lasting around one hour, with negative rheumatoid factor (RF); thus fulfilling ClASsification criteria for Psoriatic ARthritis CASPAR criteria for PsA []. He was referred to the rheumatology outpatient clinic of another hospital and treated with indomethacine and systemic corticosteroids as needed for about five years, and then achieved complete remission of joint symptoms. Three years later, because of a flare of joint involvement (episodes of peripheral arthritis and dactylitis of the small joints of hands and feet once per week) and skin involvement, he was started on methotrexate 10 mg weekly with folate supplementation, in combination with cyclosporine 200 mg daily, with reduced extension and severity of skin lesions and decreased frequency of arthritis flares to once per month. However, he did not reach complete remission. Shortly after initiation, due to a 2-fold increase of liver enzymes (hepatitis excluded based on abdomen ultrasound and hepatitis B and C viral profile), methotrexate was reduced to 7.5 mg weekly []. The reduced methotrexate regimen led to normalization of the liver enzymes but was ineffective in controlling both skin and joint involvement. Two years later, due to elevated arterial pressure values (up to 150/90 mmHg) and serum creatinine increase >30% of baseline value (up to 1.4 g/L), cyclosporine was stopped.\nThe following year, he referred to our outpatient clinic for skin and joint flare. At referral, physical examination demonstrated mild psoriasis of trunk, arms and legs, with a psoriasis area severity index (PASI) score of 5.1 (), dactylitis of the third digit of the left foot and arthritis of the third interphalangeal joint of the left hand, which, together with a normal C reactive protein (CRP), activity visual analogue scale (VAS) and pain VAS of 4 and 4.5 respectively, accounted for a disease activity in psoriatic arthritis (DAPSA) score of 12 (low disease activity) [].\nLaboratory tests showed no relevant alterations. Radiographic assessment of hands and feet showed no erosions and no signs of axial involvement were found at magnetic resonance imaging (MRI). Ultrasound imaging of the third metacarpophalangeal (MCP) joint showed inflammation of the joint and of the peritendon of the extensor tendon (a).\nConsidering that the patient failed to respond to two DMARDs (cyclosporine and methotrexate), that he refused a parenteral drug, that he presented mild skin and joint involvement, no bone erosions, dactylitis and no axial involvement, apremilast was chosen [,,]. At baseline, the EQ-5D questionnaire reported altered HRQoL, with some difficulties in motility, no difficulties in self-care and some difficulties in usual activities, moderate pain/discomfort and moderately anxious/depressed mood. Patient’s global health (GH) VAS was 60 and pain VAS was 45 (). Furthermore, the patient was screened at baseline for CV risk; carotid arterial doppler ultrasound demonstrated only intimal thickening of the left bulb and 24 h blood pressure monitoring demonstrated mild systolic and diastolic daily hypertension for which he started lercanidipine 10 mg/daily. At baseline, total cholesterol (TC) and triglyceride (TG) serum levels were 173 mg/dL and 113 mg/dL respectively, and TC/high density lipoprotein (HDL) ratio was 4.94 (). After 4 weeks, the extension and severity of erythema and infiltration of psoriatic plaques were substantially reduced (PASI 2.5, ). Furthermore, the patient reported a marked improvement of joint involvement with no further episodes of arthritis or dactylitis, although inflammatory morning stiffness persisted (DAPSA 6.0). At the 2-month follow-up, the patient reported several side effects such as mild headache, dizziness and hypotension and, due to further amelioration of the skin and joint involvement he stopped apremilast, which was restarted shortly after, due to worsening of psoriasis (PASI 3.0) and occurrence of a new joint flare (DAPSA 10). At the time he was still taking methotrexate 7.5 mg weekly. At the 6-month follow-up he reported no arthritis flares and no inflammatory morning stiffness. At physical examination, the third MCP joint was swollen but not tender (DAPSA 3), and ultrasound imaging showed reduction of joint and soft tissue inflammation (b). A further improvement of skin involvement was observed and reported by the patient (PASI 1.2, ). During the follow-up, we observed an amelioration of the serum lipid profile already after 4 weeks, with a 5.8% reduction of TC and a reduction of TC/HDL ratio, and after 12 months, a further reduction of TC, LDL and TG values of 15.6%, 25.7% and 17.7% respectively, and a 20% increase of HDL levels compared to baseline values. TC/HDL ratio reached the favorable value of 3.48, as shown in . We even observed a 5% weight loss—although our patient already had a normal baseline weight and body mass index (BMI)—reaching a final normal weight with a normal body mass index (BMI 21.5). After 12 months, the clinical manifestations were stable. We therefore decided to stop methotrexate, but the patient experienced a slight relapse of both skin and arthritis (PASI 2.5, DAPSA 6), shortly after. Therefore, although dosage was low (7.5 mg), we reintroduced methotrexate, leading at the 18-month follow-up to stable minimal disease activity with PASI 75 and joint remission (PASI 1.2, DAPSA 2) [,] and a dramatic improvement in HRQoL (). |
Patient A was a 50-year-old construction worker. His medical history showed occasional migraines, for which he took sumatriptan (25 mg), but he was otherwise in good health. He had smoked 10 cigarettes a day for many years. He had a history of two small, plantar corns on his left foot which had been present for years (Fig. ). He was receiving monthly podiatry appointments for scalpel reduction of the corns to relieve the pain. Prior to the current treatment regime, he had tried prescription insoles to redistribute pressure away from the painful areas of the foot and the application of salicylic acid corn plasters. The latter had led to irritation of the skin, so consequently it was discontinued by the patient.\nFollowing a discussion with the patient and consent, it was decided to embark on a course of microwave treatment using the SWIFT® microwave device (Emblation, UK). A course of treatment was proposed using microwave treatment given at monthly intervals for up to 4 months. At each appointment visit, prior to debridement, both plantar corns were cleaned using chlorohexidine gluconate in 70% alcohol and enucleated with a scalpel. The microwave device was then placed onto each of the two corns, and 10 W of energy was delivered for 2 s − a total of 20 J of energy was delivered into each lesion at each visit. At each visit, the patient was also asked to rate the pain in the week prior to treatment on a scale of 0–10 (with 0 = no pain, 10 = the worst pain imaginable).\nPrior to the first application of treatment, the patient had rated the pain as 6 out of 10. On subsequent visits the pain score reduced to a score of 1. A month after the fourth application of microwaves, the patient reported no pain (score of 0) from the corn in the previous week. After completion of treatment, he extended his appointment intervals to 12 weeks with no active further treatment. At his review 6 months after the last treatment, he reported that although they were still present, the corns were no longer painful (Fig. ). He was advised to return should they become painful again, but did not return to the clinic after this appointment. |
A 17-year-old boy sustained a crush injury, in a contaminated environment, with extensive loss of dorsal and volar soft tissue involving 3/4 of his right forearm, complex displaced fractures of the radius and ulna, injury of the flexor tendons, median nerve, radial artery and laceration of the extensor tendons ().\nA combined procedure with the orthopaedic surgeon was therefore carried out as a matter of urgency. The orthopaedic timing consisted of a debridement of the wound and reduction of the fractures with external and intramedullary fixation (), followed by a further debridement and washout of the wound with a pulsed lavage system, performed by the authors. The flexor and residual extensor tendons were repaired using, respectively, 3/0 and 4/0 nylon and a neurorraphy of the median nerve was carried out with 8/0 nylon under microscope magnification.\nThe radial artery was injured at 2/3 of the forearm and its stump was tied at A&E.\nFollowing a removal of 1 cm thrombus, the proximal stump was trimmed for 4 cm, in order to be away from the zone of injury, but the radial artery had initially a low flow, due to the spasm of the vessel. One mL of papaverine was injected into the vessel and a few warm dumped swabs were wrapped around the forearm.\nFifteen minutes later the situation improved and a good pulsation and outflow allowed us to consider this recipient vessel reliable for a free flap. The cephalic vein was then dissected and prepared.\nThe choice of the flaps was restricted by the necessity to replace a large amount of skin and an LD flap was selected for its well known features and advantages to comply with an extensive soft tissue loss and infection.\nAn unilateral LD flap was harvested as the patient was left dominant hand.\nThe anastomosis was carried out between the radial artery and the toracodorsal artery using 8/0 nylon while the cephalic vein was anastomosed with the toracodorsal vein using the same suture both in end-to-end fashion under microscope magnification ().\nA meshed split thickness skin graft was applied on the flap and a penrose drain and dressing provided. The patient had a prophylaxis antigangrene (metronidazole 400–500 mg every 48 hours) since his admission at A&E, followed by an antibiotic therapy (cephalosporin of third generation) changed according to the microbiological findings (staphylococcus aureus and enterococcus foecalis).\nFurthermore a subcutaneous injection of 40 mg of enoxaparin sodium was administrated every day for 2 weeks.\nThree days later the patient developed a recurrent infection on the dorsal distal aspect of the forearm.\nThe flap was still viable and both anastomoses were patent at the hand Doppler.\nThe wound was therefore explored and a further debridement was carried out to remove the residual extensor tendons and 5 cm of necrotic distal ulna, followed by a washout with the pulsed lavage system.\nAfter surgery the patient underwent hyperbaric oxygen therapy and an aimed antibiotic therapy. The additional use of hyperbaric oxygen therapy allowed a successful healing of this complex severely contaminated wound in two weeks time (Figures and ).\nThe physiotherapy treatment was immediately begun initially with static and then dynamic splints.\nThe patient sustained another accident shortly after the first trauma with a complicated fracture of the radius and ulnar deviation of the wrist ().\nThe ortopedic surgeon removed the external fixation and reduced the fracture with plates and screws. The wrist was fixed in arthrodesis ().\nThe patient underwent a further physiotherapy treatment and the functional outcome was measured in degrees of range of motion (ROM) at 1-year followup as follows: 72° of flexion at MCPJ; 70° of flexion at PIPJ; 39° of flexion at DIPJ; 1 cm away from 13° of extension at PIPJ; minus 8° of extension at DIPJ. The range of extension motion was probably due to the vicarious action of the inteosseous muscles (Figures , , , and ).\nA potential delayed reconstruction of the extensor tendons with a tendinous graft or transfer to increase the range of motion was discussed with the patient.\nHowever the patient was satisfied with the outcome of the operation and was able to use his hand with a reasonable strength for the daily activities, as shown in the pictures (). |
An 81-year-old Caucasian man presented with a large painless swelling of the cranial vault, covering almost the whole left side and also major parts of the right side of the skull.\nHe had first noticed a small swelling over the left frontal region about 40 years ago. During the following four decades the swelling slowly expanded. For more than 15 years his family physician had recommended an MRI for further examination. The patient refused it over a long time period because he felt no pain or other discomfort. He experienced no significant problems during his customary life activities.\nHe finally agreed to further diagnostic procedures after a short period of dizziness following an ambulatory treatment of a focal retinal detachment. After this episode no further clinical symptoms appeared. Neurological examination was entirely normal. No cognitive deficits were found.\nExamination of the lesion revealed a subcutaneous mass covering most of the left cranial vault and major parts of the right side. Palpation revealed a soft elastic mass, adherent to the surface, without fluctuation.\nIn contrast to the giant size of the cyst, on first sight no severe deformation of the head had developed. The overlying skin did not show pathologic changes, such as defects or inflammatory signs.\nA spiral computed tomography (CT) (Philips Brilliance 64 CT-scanner) with surface volume rendering technique revealed a large swelling of the scalp, especially over the left hemisphere (Figure ). Computed tomography (CT) with surface reconstructing and insets of axial, coronal, and sagittal scans demonstrated a giant cystoid lesion of the skull, with large calvarial defects on both sides, left more than right. The outer and the inner table of the skull bone were widely destroyed; in some areas the inner table was thinned out (Figure ). A distinct compression of both brain hemispheres was caused by the tumor, with a slight midline shift to the right side (Figure ).\nThe MRI scan T2 weighted images (Philips Achieva 1.5 T) revealed an inhomogeneous, mainly hyperintense mass without penetration of the dura. Significant compression of brain hemispheres and ventricles without cerebral edema suggested a slow growing tumor. In diffusion weighted images there was a restriction of diffusion with low signal in the ADC maps (Figure ). The contrast enhanced T1 weighted images (0.1 mmol/kg Gadovist (gadobutrolum), Bayer Health Care) showed a mild thickening of the intact dura. No enhancement in the epidermoid tumor was found (Figure ).\nIn summary, radiological features were suggestive of the presence of an extradural intradiploic epidermoid cyst.\nAfter thorough discussion of the diagnostic findings with the patient, he favored a biopsy to assure the histological classification of the tumor but refused the option of a complete removal of the tumor and subsequent cranioplasty. A biopsy was taken in the left frontal region. Histological examination revealed laminated keratin material, cholesterol crystals, and cellular debris. Pathological findings were consistent with the diagnosis of an epidermoid cyst. Due to a superficial wound infection surgical revision was necessary. His postoperative course was uneventful. |
A 34-year-old female presented to the department of general surgery with complaints of unpainful mass, localized on the dorsal surface of the right foot. In out-patient clinic, she was examined by a general practitioner who established the diagnosis “foot hygroma” and referred her to the hospital for surgical excision. The patient noted for the first time a mass over her right foot during the second trimester of pregnancy with slow progressive enlargement of the tumor over the course of a year. Her recent medical history was unremarkable with no events of blunt trauma, puncture or any infectious process in the affected region of the right foot.\nAt admission to the department, inspection revealed the nontender, round, elastic, slightly mobile mass with a size 30 × 20 mm located subcutaneously on the dorsal surface of the right foot ().\nPalpation of the mass has not demonstrated any pulsation or thrill and there was no bruit on auscultation. Attempts to compress the mass against bone with patient upright resulted in only minimal decrease of tumor size whereas in recumbent position of the patient with elevated leg the fast spontaneous collapse of the tumor was observed. Based on clinical findings, the aneurysm of the superficial vein of the foot was suspected and Color Doppler and duplex ultrasound were performed to confirm the diagnosis. Duplex scanning showed the ovoid anechoic dilatation of the medial marginal vein of the foot measuring 2.5 cm in diameter and extending over a length of 3.5 cm with no thrombus inside. There was no detectable spontaneous flow in the lumen of the aneurysm with appearance of a turbulent to and fro flow (the so-called “yin-yang” sign) during the compression of the venous foot pump ().\nThe terminal and subterminal valves of the saphenofemoral junction and all valves along the course of great saphenous vein were competent. The scanning of the arterial and venous systems of both lower limbs did not show any other abnormalities.\nThe patient was operated under local tumescent anesthesia with 60 mL of 0.1% lidocaine solution. Via 5.0 cm longitudinal skin incision, the sac of aneurysm was visualized and has been dissected from the surrounding tissues ().\nExcision of the aneurysm was performed after bipolar ligation of marginal vein (diameter of vein at both sides of the aneurysm: approximately 2.0 mm) and wound was closed with absorbable running intracuticular suture. The foot and leg were wrapped with elastic bandage and after 6 hours the patient was discharged. The postoperative evolution was uneventful with moderate edema of the foot which resolved spontaneously to the second postoperative day.\nHistopathological examination of the sac of aneurysm revealed normal but thinned vein wall with reduced number of smooth muscle cells. At one-month follow-up, there were no signs of recurrence and the patient has declared complete satisfaction by outcome of intervention. |
A 48-year-old man underwent percutaneous cathether ablation for atrial fibrillation. Four years previously he had been diagnosed with a dilated cardiomyopathy after presenting with an episode of atrial fibrillation with an ejection fraction of 17%. He had no history of hypertension, diabetes or previous strokes. He was commenced on perindopril, metoprolol and warfarin. Initially, his atrial fibrillation was paroxysmal, however, over the last nine months he had noticed an increase in frequency of palpitations. Particularly in the last two months, he felt the atrial fibrillation was continuous and he was much less energetic and easily fatigued.\nThe patient had been anticoagulated with warfarin until 4 days prior to the procedure when the warfarin was withheld and anti-coagulation continued with subcutaneous low molecular weight heparin. A transoesophageal echocardiogram had been performed one week prior to the ablation procedure and showed no pre-formed atrial thrombus. The patient underwent segmental pulmonary vein isolation and linear ablation within the left atrium for substrate modification, as well as linear ablation of the cavo-tricuspid isthmus for ablation of the classic right atrial flutter circuit. An irrigated catheter tip was used. Intravenous heparin was commenced the morning of and during the procedure with a target activated clotting time of between 300 and 350 seconds.\nOn waking from the anaesthetic, the patient immediately complained of visual disturbance. On examination the patient had a visual acuity of 2/200 in both eyes and computerised static perimetry performed day 1 post ablation showed bilateral superior altitudinal field defects (figure ). Full neurological examination was undertaken and in addition to the visual deficit the patient also had subtle expressive dysphasia with word finding difficulty. Magnetic resonance imaging (MRI) of the brain on day 2 post ablation showed increased T2 FLAIR signal in both occipital lobes, extending into the inferomedial aspect of the left temporal lobe (figure ), no evidence of older infarcts were seen. A diagnosis of multifocal cerebral infarction post catheter ablation was made.\nDay 3 post ablation, he developed an episode of atrial arrhythmia and was commenced on oral amiodarone. Warfarin was withheld until day 5 post ablation and then recommenced and maintained at therapeutic levels. Blood tests for biochemical abnormalities, coagulation studies and a clotting screen were within normal limits. A review one week later, showed an improvement in the visual acuity to 20/60 in the right eye and 20/80 in the left eye with a persistent bilateral superior altitudinal field defect.\nOne year later, visual acuity and visual field remained unchanged and the patient continued to have ongoing difficulties with word finding and reading. His atrial fibrillation, however, was now well controlled on medical therapy (warfarin, amidarone, perindopril and metoprolol). |
A 44-year-old Japanese male, an office worker, was transported to our emergency department with a complaint of temporary loss of consciousness. He had experienced fatigue for 3 years and had fallen easily in his daily life starting 2 months before admission. He was feverish and had a cough and phlegm for several days before admission. On the way to work, he felt drowsy and fell down the stairs of the train station, necessitating transport to our hospital by ambulance. The peripheral blood analysis noted mild elevation of white blood cells. The blood chemistry test disclosed mild elevation of C-reactive protein and mild liver dysfunction (). He was thin, i.e., his height was 174 cm and he weighed 52 kg (). Chest computed tomography (CT) showed severe infiltration in the upper posterior fields of both lungs as well as food debris in the esophagus. He was diagnosed as having aspiration pneumonia. His past medical history included diabetes mellitus that had been treated by a local doctor; glycated hemoglobin was approximately 7%. He had experienced ileus six times since age 30 years. He was not married. His father had died of dilated cardiomyopathy at the age of 70 and had also been thin. On the other hand, his mother was healthy but his maternal uncle was diabetic. His younger sister (Case 2) also had mild muscle weakness of the four extremities. Aspiration pneumonia was treated by intravenous administration of antibiotics under conditions of food-take restriction combined with temporary insulin infusion, and the respiratory symptoms subsided. On the neurological examinations after improvement of pneumonia, he presented with typical clinical manifestations of DM1 (), such as forehead balding, hatchet face with bilateral ptosis, nasal speech, mild muscle weakness of the four extremities, handgrip myotonia, and diffuse muscle atrophy. Electromyography performed to test the biceps brachii muscle and femoral quadriceps muscle on the left side revealed frequent myotonic discharges. Because DM1 was strongly suspected, we recommended that, after providing informed consent during genetic counseling, he undergo genetic testing for DM1 together with his younger sister. The number of CTG repeats in the DMPK gene was abnormally expanded to about 600 repeats in the elder brother and about 900 repeats in the younger sister, in contrast to those of healthy individuals who have 5 to 37 repeats (), thereby confirming the diagnosis of DM1 in these siblings (). |
An 18-year-old female with a history of right pulmonary vein atresia who underwent a right pneumonectomy 2 years prior presented with a 6-month history of worsening dyspnea. Pulmonary function tests were notable for a 43% decline in her forced expiratory volume in 1 second from 1.34 L to 0.77 L. A CT scan of her chest revealed complete shift of the mediastinum to the right, severe narrowing of the left main bronchus between the descending thoracic aorta, thoracic spine, and the left pulmonary artery to 0.3 cm, hyperinflation of the left lung, and a dilated and right-shifted trachea ( and ). She was scheduled for a right thoracotomy with chest implantation of tissue expanders for medialization of her mediastinum.\nPrior to induction of anesthesia, a radial arterial line was placed with local anesthesia. An inhalational induction technique was performed with sevoflurane, with maintenance of spontaneous ventilation. Direct laryngoscopy revealed a grade 1 view and a 6.0 microlaryngeal ETT was passed through the vocal cords and into the trachea without issue. There were signs of obstruction on capnography; however, the patient was maintaining adequate oxygenation and ventilation despite her known left mainstem bronchial obstruction. Following intubation, fiber-optic bronchoscopy was used to confirm ETT placement and evaluate the lower airway. The right main bronchial stump was intact; the left main bronchus was significantly narrowed (). Prior to the start of surgery, additional vascular access was obtained with a central venous catheter placed in the right internal jugular vein. A transesophageal echocardiography (TEE) probe was placed to aid intraoperative monitoring.\nThe patient was ventilated with tidal volumes of 6 mL/kg of ideal body weight and a positive end-expiratory pressure of 4 cm H2O. Peak airway pressures were kept below 20 cm H2O. The surgery proceeded without issue. Careful attention was paid to hemodynamics and ventilator parameters during the filling of the tissue expanders to ensure that there was no cardiac or pulmonary compression from the medialization of her mediastinum. The patient tolerated this well.\nAt the conclusion of the surgery, repeat fiber-optic bronchoscopy was performed and confirmed complete resolution of the left main bronchus obstruction (). The patient was extubated in the operating room and taken to the postanesthesia recovery unit. Her postoperative pain control was managed with a thoracic epidural that was placed preoperatively. She had significant improvement in her dyspnea and she was discharged from the hospital on postoperative day 4. Her postoperative forced expiratory volume in one second was 1.35 L, which is her baseline. Repeat CT scan was performed 6 months after the surgery showed evidence of mild mediastinal rightward shift but no left main bronchus obstruction ( and ). |
This is a 50-year-old right-handed male, with 33-year history of T6 AIS A SCI from a gunshot wound complicated by chronic pain, left hip and knee heterotophic ossification, and a chronic dislocation of his right hip, who initially presented to the emergency room with a right shoulder mass in September 2014. While he initially noticed the mass about 2 months earlier, he presented for evaluation now because of acute onset of pain, weakness and paresthesias in the right arm. He was admitted to the general medicine service for pain management and underwent an initial work up for his right shoulder mass, including advanced imaging and a core biopsy. Physiatry was consulted due to his functional deterioration that precluded him from returning to his previous independent living arrangement. He demonstrated diffuse, mild weakness throughout the right arm that was variable and seemed to be correlated with his reported pain level, but his most consistent and weakest movement patterns were his grade 4/5 weakness in finger abduction and distal interphalangeal joint flexion. He had reduced pin prick sensation over the volar surface of digits 3–5, palm and forearm of the right arm and hand. He was not able to perform transfers to or from his manual wheelchair due to his level of pain. The magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass with a maximum diameter of 6.9 cm (Fig. ) that involved the right deltoid and pectoralis major muscles. His core biopsy demonstrated a STS that was classified as a high grade (III) spindle cell sarcoma.\nOncology recommended treatment for his STS with a course of outpatient neo-adjuvant radiation therapy followed by gross total resection with wide margins. Physiatry pre-operative consult focused heavily on functional prognostication. The patient expressed multiple times that he placed the highest priority on return to his previous modified independent living arrangement and not only survival after his STS treatment course. The patient’s personal values combined with the physiatric assessment informed the pre-surgical planning. In particular a decision was made to take a narrower surgical margin around key muscle group (pectoralis major and deltoids) in order to help maintain the man’s manual wheelchair mobility and his ability to independently transfer himself.\nHe completed neo-adjuvant radiation therapy in November of 2014 and underwent radical excision of his right shoulder mass with flap closure that December. He began intensive inpatient rehabilitation after he was given clearance to weight bear through his arm about 8 weeks later. Initially he required total assistance for most ADL’s, including transfers, manual wheelchair propulsion, dressing and toileting. Despite the extensive surgery and radiation treatments, the gentleman was able to return to a functional level, approaching his pre-morbid status (modified independence). He was successfully discharged home to live alone in his accessible apartment complex. |
A 9 years old, previously healthy boy presented with sudden onset of colicky abdominal pain of 4 h duration. The pain was mainly infra-umbilical. Associated with this he had gross abdominal distension and 2 episodes of non-bilious vomiting. Parents reported that the child had bowel movements daily before he joined school since when bowel movement became every 2–3 days. The parents do not remember when the child passed the first meconium. The child had had similar attack a month before presentation to our hospital and rectal tube deflation was done at a nearby hospital. Otherwise there was no other complaints. His growth and development is similar compared to his peers. He was not on any medications. There was no family history of similar illnesses.\nOn physical examination except for tachycardia other vital signs were all in normal range. All anthropometric measurements were in normal range. He was in pain while holding his abdomen. He had gross abdominal distension. There was no direct or rebound tenderness. Abdomen was hyper tympanic to percussion. Bowel sounds were increased. There was no organomegally or palpable masses on deep palpation. Per-rectum examination showed empty rectum. No stool or blood on examining finger. A Plain abdominal radiographs showed single hugely dilated bowel loops in the left lower quadrant with single air fluid level (Fig. ). Abdominal ultrasound was unremarkable except for gas filled abdomen. Complete blood count and organ function test was done and all were in normal range.\nWe put the patient in knee-chest position and gently introduced fully lubricated rectal tube into the rectum and slowed advanced upward. Gush of air and scanty stool came out. The abdomen became soft and the cramp subsided. We left the tube in the rectum for 24 h. We took full thickness rectal biopsy the next day. It showed aganglionated rectum with absent ganglion cell in both submucosal and muscles layers. We also obtained diagnostic barium enema and it showed redundant sigmoid with no radiologic evidence of Hirschsprung’s disease (Fig. ). After Hirschsprung’s disease confirmed by rectal biopsy, he underwent primary trans-anal Soave’s endorectal pull through (Fig. ). We resected the rectum and dilated part of sigmoid colon until normal appearing bowel caliber is reached. We did colo-rectal end-to-end anastomosis 2 cm above dentate line. He was kept nothing per-os for the 48 h post-op and was put on prophylactic antibiotics.\nHe was then started on feeding and tolerated it and discharged on 6th post-op day. He experienced passage of loose frequent stool for the first 10 days post-operatively which improved later by itself.\nCurrently, on his third post-operative months, he is having regular bowel movement. Result of the excision biopsy showed aganglionated distal colon and ganglionated proximal bowel. |
Our patient is a 63-year-old female with osteoarthritis in the lumbar spine and bilateral knees. Knee X-rays showed bilateral mild osteoarthritic changes. MRI of left knee showed grade II-III patellar chondromalacia and mild osteoarthritic changes. Her left knee pain was uncontrolled with conservative measures including NSAIDs and narcotics. Steroid injections were provided once with no pain relief. Subsequently, over a period of five years, she received multiple courses of sodium hyaluronate injections to the left knee without complications, waiting at least six months between each series of five injections. The skin was thoroughly examined prior to each injection and was noted to be in a good condition, with no breaks, rashes, or bruises evident. She had great pain relief from the Hyalgan injections and repeatedly requested them. Clean technique was employed prior to each of these injections, including use of betadine, alcohol, and gloves.\nPertinent past medical history included treatment for invasive ductal left breast cancer with lumpectomy and radiation five years prior to this hospital presentation. She had been in remission until four months prior to hospitalization, when she was diagnosed with recurrent left breast ductal carcinoma. She then underwent mastectomy with axillary lymph node dissection and received one round of chemotherapy treatment, with Taxotere, Carboplatin, and Herceptin, 11 days prior to hospital admission. The surgery and chemotherapy proceeded without complications.\nFive days after the last sodium hyaluronate injection to the left knee, she presented to the hospital with complaint of left leg pain that woke her from sleep. She initially noticed a small area of erythema in the left popliteal fossa. Over the next few hours, she developed rapidly progressive swelling, expansion of the erythema, and formation of multiple large bullae extending from the thigh to proximal leg. By the time she arrived at the hospital, the bullae and erythema had expanded to the gluteal fold and inguinal region. CT of left lower extremity showed free gas through the adductor musculature and posterior and superior to ischial tuberosity. Given the aggressive symptoms, the patient was emergently taken to operating room for extensive debridement and amputation at the lesser trochanter. She was then admitted to intensive care unit on broad spectrum antibiotics for septic shock secondary to necrotizing fasciitis of the left lower extremity, involving both posterior and anterior compartments. She deteriorated and required vasopressors, intubation and multiple debridements. Over the next 3 days, she suffered multiorgan failure and required hemodialysis. Given her wishes and poor prognosis, life support was withdrawn. She died 20 days after last chemo, 13 days after the most recent Hyalgan injection, and 7 days after the onset of necrotizing fasciitis. |
A 50-year-old woman had undergone craniofacial augmentation involving bilateral injection of a large amount of PAAG into the temporal epicranial aponeurosis in 1997. During the 6 months after the operation, the patient complained repeatedly of recurrent swelling of the affected regions. To alleviate the symptoms, the plastic surgery clinic where she underwent the augmentation attempted to squeeze out the filler material through a 5 cm coronal incision in the scalp. The volumes of PAAG originally injected and then later removed are both unknown. After the operation to remove the PAAG, the patient's symptoms did not improve.\nWhen the patient came to our department 15 years after the first operation, she complained of recurrent swelling of the temporal region, forehead, and eyelids as well as pain at the temporal regions that became even more intense with cold ambient temperature or with large facial expressions. Firm palpation of the temporal region of the scalp over the injected PAAG revealed persistent indention resembling pitting edema, which disappeared after about a minute. Magnetic resonance imaging (MRI) T2 images showed extensive migration of the PAAG as a hyperintense mass. The PAAG had spread from the temple regions to the cheek and neck regions () and from the forehead to both the upper and lower eyelids (). The filler had infiltrated the left temporalis more than the right temporalis, which may explain the more intense pain experienced by the patient in the left temporal region compared to the right temporal region. We performed an operation to remove the PAAG under general anesthesia with bilateral temporal incisions in the scalp. When we reached the epicranial aponeurosis, we found it was impossible to separate the PAAG from the patient's tissues because the PAAG had permeated the tissues (). Thus, we removed most of the PAAG in the epicranial aponeurosis at the temporal region and forehead together with the affected tissues.\nPathological analysis revealed the presence of an amorphous foreign material deposited in the tissue without any evidence of a foreign body reaction or inflammatory cells. In addition, bacterial cultures were negative. In the routine preoperative examination, the patient's white blood cell count (WBC) was 2.9 × 109/L. However, the patient denied taking any antibiotics or immunosuppression drugs within the previous month. After consultation from the hematologic department, we injected granulocyte colony-stimulating factor subcutaneously the night before the operation, and, in the morning of the operation, the patient's WBC had increased to 14.2 × 109/L. Three months after the operation, the patient returned for follow-up. She reported that the temporal pain and swelling of her forehead and temple were reduced and her WBC was 4.9 × 109/L. However, she refused additional MRI examinations. In the following year, we called the patient to learn whether the leukocytopenia had continued. The patient reported that two additional blood tests carried out since her last follow-up were both normal. |
A 46-year-old female patient reported to the Department of Oral and Maxillofacial Surgery, Postgraduate Institute of Dental Sciences, Rohtak, Haryana with the complaints of pain, slight swelling on the left side of mouth floor and discomfort during swallowing and limitation in mouth opening. Patient history revealed that a month earlier she had undergone an unsuccessful surgical procedure under local anesthesia performed by a general practitioner for removal of an impacted third molar on the left side of the mandible. The tooth fractured during extraction. The procedure described by the patient as being difficult and complicated. On clinical examination, the root was not palpated on the posterior region of the mouth floor and submandibular region extraorally. After the history and careful assessment of the socket, the patient was advised panoramic radiography. Panoramic radiograph showed the presence of a radiopaque mass that is similar to the appearance of the third molar tooth root. Two-dimensional radiographs were inadequate in this case. For detailed radiographic examination, computed tomography (CT) scans were taken by spiral technique and axial sections were obtained . Images were reconstructed to form sagittal and coronal sections and examined. CT examination demonstrated the presence of a high-density area located in the left submandibular region demonstrating the position of root fragment. Careful evaluation of the CT and orthopantomography (OPG) revealed the position of displaced root in submandibular space . After routine blood investigations and preanesthetic check-up, it was planned to retrieve the tooth from submandibular space via extraoral submandibular approach under local anesthesia with conscious sedation.\nThe surgical site was exposed carefully with layer-wise dissection. The dislodged root was started locating using blunt dissection with small artery forceps with extreme care because there are increased chances of root displacement into pharyngeal spaces further. The dislodged root was not directly visible. As 1 month had been already passed, we were suspecting some fibrosed or infected tissue mass must be enclosing the root segment. Considering this fibrosed tissue the displaced root segment, it was grasped with Ellis forcep and there was instant ooze of pus in that area, which further confirmed the presence of root fragment there only. Care must have to be taken because this pus could have been progressed into submandibular space infection and further progressing to pharyngeal spaces or may be Ludwig's Angina if not managed.[] The fibrosed tissue was also dissected carefully. Dislodged root was grasped with artery forcep and retrieved from the fibrosed tissue and pus was wiped with gauze piece and the area cleaned with normal saline and metronidazole solution.\nThe surgical site was sutured layer wise. The patient was prescribed oral antibiotics for 1 week. The postoperative OPG confirmed the retrieval of the displaced root from submandibular space. Postoperative course was uneventful and the patient was asymptomatic at the follow-up visit 3 months later. Written consent was obtained from the patient for case presentation. |
We report the case of a 31-year-old Caucasian woman, gravida 3, para 1, who was referred after a second trimester fetal anatomy screening at 20 weeks gestational for a suspicion of a complex fetal cardiac malformation, for which several specialized opinions tried to reach consensus.\nThe obstetrical history of the patient includes a previous Caesarian section with a normal course of parturition and a spontaneous miscarriage. The current pregnancy presented a low risk for aneuploidy according to the performed cell-free fetal DNA test. The classical karyotype performed after the abortion did not reveal any chromosomal abnormalities.\nPrevious ultrasound evaluations were incongruent and reported the following findings:an isolated aortic arch anomaly (supposedly aneurysmal dilation from which the left common carotid artery emerges) and coarctation of the aorta with the anterograde flow; ventricular septal defect, coarctation of the aorta, and a vascular formation located superior from the aortic arch with the appearance of an arteriovenous fistula; aneurysmal dilation located above the pulmonary trunk bifurcation and a dilated left common carotid artery with a retrograde flow; minor ventricular septal defect with a normal ductus venosus triphasic flow.\nWe performed fetal echocardiography, which demonstrated a mild cardiomegaly with a left deviated 72-degree heart axis, normal aspect of the four-chamber view, a small membranous ventricular septal defect, and ductal aortic coarctation; the ductus venosus flow was normal (, and ). In addition, we identified an aneurysmal structure measuring 1.63/1.25/1.16 cm with turbulent Doppler flow, situated above the emergence of the pulmonary trunk and continued by a dilated vascular structure that bifurcates in the cervical region; the aneurysm seemed connected to the left pulmonary artery as well. A dilated left subclavian artery was also suspected (, and ).\nIn the context of complex cardio-vascular malformations, the patient requested the termination of the pregnancy by drug-induced abortion.\nThe hands-on dissection of the fetus revealed a set of abnormalities that could stand as an anatomical basis for what has been found during the ultrasound examination.\nThe first and the most pronounced aspect was the distention of the whole venous system of the neck and mediastinum. The specimen presented a linguo-facial vein that described a rather sinuous pathway alongside the inferior margin of the mandible (). Both the linguo-facial vein and the external jugular vein appeared with a markedly increased caliber, around 4–5 times larger than expected for this gestational age. Both left and right jugular veins and the right subclavian vein were assessed as three times larger than usual, respecting the normal relations to the neighboring structures ( and ).\nThe confluence between the left jugular and subclavian vein into the left brachiocephalic vein was observed to be very dilated to superior and inferior, extending above the superior margin of the omohyoid muscle as well as below the inferior concavity of the aortic arch. Moreover, on the inferior side of the enlarged brachiocephalic vein, a vessel could be observed descending lateral to the left vagus nerve and communicating with the left pulmonary artery. The left pulmonary artery was observed to be dilated as well, around twice as normal ().\nRegarding the great vessels of the heart, there are some anomalies to be discussed. A narrowing of the aortic arch was identified distally to the emergence of the left subclavian artery ( and ). A large, patent ductus arteriosus was found, ending right at the narrowing level observed in the aortic arch (ending right at the coarctation level) (). The left subclavian artery was dilated as well, sizing as much as the ascending aorta and the aortic arch, creating the illusion of a terminal branch rather than a lateral one ( and ).\nHeart analysis concluded no distinct changes in heart architectural formation for this gestational age. Atrioventricular and ventriculoarterial concordance was noted. Atria and ventricles were increased in relation to the mediastinum. Surprisingly for an aortic coarctation, the right atrium was not found to be enlarged. |
A 62-year-old woman (Figure , II-3) was admitted to the hospital for recurrent partial headache with weakness of one side and aphasia for about 45 years. In her first attack, the patient suddenly experienced an aura with visual disturbances which she described as increasing scotomata in the bilateral visual field. After a few seconds, the patient developed a serious headache, mainly located on the left side. After a few minutes, she presented a paralysis of the right side and speech difficulties accompanied by dizziness and vomiting. These symptoms resolved after about 2 h. After this initial onset, she had an attack nearly every 4–5 years, and the clinical presentations of her attacks were always similar to the first one. The duration of the aura symptoms and the migraine was typically 1–2 h but sometimes the migraine could last up to 4 days. Sometimes headaches occurred before the hemiplegia and aphasia. Each headache was accompanied by dizziness and vomiting but without loss of consciousness. In most attacks, this patient experienced additionally a flushing of the neck and face and felt that the skin temperature of this affected area was increased, but the temperature was never measured. These symptoms may be related to an extracranial vasodilation when a migraine attack occurred. She did not undergo regular treatment except for simple analgesics as a symptomatic therapy. Recently, her condition aggravated as the frequency of attacks increased from once every 4–5 years to once every 1–2 weeks which had a serious impact on her everyday life. Therefore, during a severe migraine attack, she visited our hospital. We reviewed her family history, and we found that three other subjects, her mother, brother, and nephew, had similar clinical symptoms (Figure ). Their presentations are as follows:\nThe proband's mother (Figure , I-2) died of uremia at the age of 72. According to her husband and children, she reported typical hemiplegic migraines since an age of 14 years with five attacks per year on average. The aura symptoms were similar to those in the proband, including bilateral visual symptoms (scotomata), speech difficulties, and hemiparesis.\nThe proband's 55-year-old brother (Figure , II-1) had first at the age of 15 headache attacks with nausea, vomiting, visual field defects, and one-sided motor weakness. Usually, these attacks last 5 h. The disease presentation was progressive with age.\nThe proband's nephew (Figure , III-1), a 25-year-old fitness coach, had first headache attacks with visual symptoms (scotomata) and lateralized motor weakness at the age of 13. Each attack lasts about 20 min.\nAfter admission, her neurological examinations were unremarkable and brain magnetic resonance imaging (MRI) and Magnetic Resonance Angiography (MRA) showed no meaningful abnormalities (Figure ). Thus, the suspected diagnosis was transient ischemic attack (TIA). During her hospitalization, the patient had several migraine attacks that were characterized first by visual symptoms, then aphasia and right limb paralysis 10 min later, and finally severe headaches after 20 min. At that time, the neurological examination revealed: no loss of consciousness, motor aphasia, muscle strength 2 in the right limb, and normal findings in the examination of the residual nervous system. After about 1 h, the symptoms of the aura were relieved, while the headache lasted for about 1 day. However, the symptoms were not relieved after dual antiplatelet aggregation treatment, and transthoracic echocardiography and carotid ultrasound failed to identify any underlying cerebrovascular etiology. After careful consideration of all aspects, she was diagnosed with hemiplegic migraine. So, we conducted a genetic test on the patient and found a heterozygous point mutation (c.4495T>C) in exon 26 of the SCN1A gene. This mutation caused amino acid 1499 to change from phenylalanine to leucine (p. Phe1499Leu), which may cause the disease by affecting the SCN1A protein function.\nTo establish the diagnosis, we performed a genetic test on those family members to analyze for the presence of mutations in genes including CACNA1A, ATP1A2, and SCN1A related to FHM. We only found a gene mutation in SCN1A, but this mutation was detected in all affected subjects in this family (Figure ). Therefore, this patient was diagnosed with FHM3. She was discharged after receiving a health education on migraine attacks, which suggested her staying away from stress, bright lights, sleep disturbances, physical exertion, and alcohol consumption because these have all been reported as trigger factors in FHM (). Upon being discharged from the hospital, she had intermittently taken flunarizine capsules and rizatriptan benzoate tablets to prevent and control migraine attacks. After 6 months of follow-up, the efficacy of the drug was uncertain, because the frequency of headache attacks was not adequately reduced. After the low efficacy of her medication became clear, we consulted again the literature and consider now a trial with lamotrigine or acetazolamide (). |
A 60-year-old previously healthy female presented in June 2009 with an edematous left leg, with pain and swelling in the left groin due to palpable lymphadenopathy. She was referred to gynecologic oncology for evaluation of possible gynecologic malignancy. Dilatation and curettage of endometrium did not reveal malignancy. Left inguinal lymph node biopsy revealed FL with kappa-restricted B-cells, CD10 and CD19 positive, comprising 50% to 65% of total cellularity. She developed fever 4 days after biopsy, and CT scan of abdomen and pelvis revealed bulky masses extending from the obturator area into the retroperitoneum, as well as large masses within the pelvis. There were also areas of fluid and gas in the inguinal area and in subcutaneous fat in the left thigh. She was designated stage IIA. As a result of the infection following the nodal biopsy and the continued lymphatic obstruction, the wound required placement of a continuous drain and she received prolonged antibiotics. It was apparent that the wound could not heal until there was significant reduction in lymphadenopathy. She was, therefore, referred for treatment of her newly diagnosed lymphoma.\nBecause of the ongoing wound drainage and recent infection, she was initially started on therapy with R alone, 375 mg/m2 weekly × 4 weeks, with the plan to add cytotoxic chemotherapy after significant wound healing. However, although she demonstrated response, the wound remained open, so R was continued weekly with dose escalation. She continued this with excellent response and eventual wound healing. Chemotherapy was never administered due to resolution of lymphadenopathy with R alone. From January through June 2010, she received monthly maintenance R × 6 doses, escalating to 1500 mg/m2. She tolerated this well, with only a first dose infusion reaction. In September 2010, a CT scan revealed no evidence of disease. She continues to have annual scans and physical examinations and laboratory evaluation every 6 months. She remains in complete remission, now 6.5+ years from the end of R therapy. |
A 56-year-old male presented to the wound clinic with a large odorous wound covering the anterior and lateral part of the right ankle. The cause of the wound was unclear; however, the patient underwent an ankle surgery about thirty years ago and again last year for treating a fracture of the same ankle. It was not sure if the wound was related to this surgery, but it was at the same location as the old scar. The wound became bigger over several months although the patient was treated by multiple state hospitals with various topical ointments, including Bactroban (mupirocin) and Fucidin, and he received various oral antibiotics on numerous occasions. The patient is a smoker (10 cigarettes per day, and occasionally marihuana).\nThe ulcer was heavily infected with Pseudomonas aeruginosa, had the characteristic odor, and produced thick, green, copious amounts of exudate (a). The wound was very painful, and the patient used crutches to be able to walk. The wound was suspected to be cancerous due to the tissue structure. This needed to be confirmed with a biopsy; however, the patient refused this. L-Mesitran Soft was applied and covered with an absorbent secondary dressing. The patient was scheduled to come back the following day for a dressing change but did not show up because of low income and he did not want to spend any money on wound care. On Day 3, the wound dressing was changed pro bono. The wound was macerated, bled, and the bandages were very dirty due to the excessive amount of exudate and delayed dressing change. On Day 6, the patient came for the second dressing change, and after cleansing the area with saline, the wound odor was decreased and the wound remarkably improved. The same protocol was used for another two weeks without antibiotics. On Day 20, the wound size reduced with the presence of granulation and epithelial tissue, and there was a strong reduction in odor, necrotic tissue, and slough (b). Moreover, the pain was reduced, and the patient was able to walk with only one crutch. Against our advice, the patient decided not to come to the clinic anymore and take care of the wound himself. |
A 63-year-old man reported a rapidly growing soft mass on his glabellar region for 4 months (). The painless soft mass was approximately 3×2 cm with elevated skin. The patient was on medication for hypertension. On contrast computed tomography scan, we observed a 3.1×2.6×2.0 cm soft tissue mass at the right fronto-ethmoidal sinus destructing the outer table of the frontal sinus wall (). Our impression was a sinus-origin tumor. Thus, ultrasonography-guided fine needle aspiration was performed and a small, round cell malignancy was found on liquid-based cytology. For further evaluation, magnetic resonance imaging (MRI) () and positron emission tomography scans were performed and no evidence of distant metastasis or metastatic regional lymph nodes were found. For histologic confirmation, we attempted a transcutaneous open biopsy through the elevated skin and the final pathology report was sinonasal intestinal-type adenocarcinoma (, ). As this histologic finding could be related to a primary GI tract malignancy, we additionally screened for GI malignancies using endoscopy; no evidence of GI malignancy was found. Residual cancer and neck lymph node evaluation was verified by MRI, and there was also no evidence of neck lymph node metastasis. For surgical treatment, we widely excised the previous wound from the open biopsy and ablated the frontal sinus mucosa (). To cover the skin defect of the obliterated frontal sinus, immediate reconstruction with free anterolateral thigh flap was performed. The right superficial temporal artery and vein were used as the recipient vessels for microscopic anastomosis. There was no grossly residual tumor and the intraoperative surgical biopsy reported clear resection margins with no tumor involvement on the posterior mucosal wall of the frontal sinus. Immunohistochemical staining was performed to reveal sinonasal intestinal-type adenocarcinoma, favoring low-grade ( and ). After consultation with radiation oncologists, we elected to skip postoperative adjuvant radiation therapy and instead closely observe the patient at the outpatient clinic. The wound healed completely (). The patient is currently at 6 months after surgery without signs of recurrence at the radiologic follow-up. |
A 68-year-old male consulted for generalized abdominal pain and constipation. Past medical history included a RYGB for morbid obesity 7 years before presentation, and ischemic heart disease. Conservative therapy did not alleviate his symptoms. Computed tomography (CT) scan showed marked ascending colon dilatation () without an identifiable lesion indicative of CPO. The differential diagnosis for CPO included functional causes such as cardiac disease, metabolic or electrolyte imbalances, renal insufficiency, medications, and anatomic causes like inflammatory processes and malignancy. Over the course of 2 months several diagnostic procedures were done. Three colonoscopies with multiple biopsies of the right colon showed submucosal mucin extravasation without mucosal abnormalities. On workup for elevated liver enzymes, a magnetic resonance cholangiopancreatography demonstrated intrahepatic biliary dilation with a dilated common hepatic duct and could not rule out a lesion in the bile duct as the cause. A laparoscopically assisted endoscopic ultrasound (EUS) through the remnant stomach showed dilated intrahepatic ducts; a fine needle aspiration of the liver, performed to assess for a possible hilar lesion observed during the EUS, did not show abnormalities. Endoscopic retrograde cholangiopancreatography (ERCP) was unsuccessful due to distorted anatomy. After the ERCP attempt the patient developed shock and was found to have a perforation of the duodenum during emergent exploratory laparotomy. The perforation was closed and the dilated right colon excised. Shortly thereafter, additional procedures were necessary for intestinal infarction secondary to the perforation induced shock, emergent surgery, and poor cardiac function. He continued deteriorating, opted for comfort measures only and expired a week later. Pathologic examination of the colon showed prominent segmental dilatation but no discrete lesions (). Microscopically pyloric-type glands with minimal cytologic atypia were present in the muscularis propria and subserosa (). The tumor did not elicit a desmoplastic response. Architecturally, the tumor glands were well formed with no or minimal branching; the longest axis of the glands always followed the orientation of the collagen they were invading (). The mucosa was free of tumor. By immunohistochemistry the tumor was positive for cytokeratin (CK) 7, SMAD4, S100P and negative for CK20 and CDX2, consistent with foregut origin. Ki-67 proliferative activity was 25%. At autopsy the primary tumor was located in the antrum of the RYGB, where it caused diffuse thickening of the wall (). |
The patient is an 11-year-old boy with attention deficit/hyperactivity disorder and Tourette syndrome. Two years prior to the current incident he had been seen by a pediatric psychiatrist over a course of time and was finally treated with methylphenidate. He was slowly uptitrated to the maximum appropriate dose of 54 mg per day (36 + 18 mg per day/body weight 50 kg), which had been ongoing ever since.\nApparently, the child had been feeling well, but a week before his admission he felt dizzy and light headed after vigorous trampoline jumping. His mother noticed he had tachycardia but thought nothing of it. The incident only lasted a few minutes and afterwards he was well.\nOn the day of admission, the patient had been in conflict with one of his peers. To calm down, the patient was sent to the gym court, to exercise and play ball. After a few minutes he left the court and fell down with cardiac arrest due to ventricular fibrillation. He was successfully resuscitated by school staff and the prehospital service.\nUpon hospital arrival the child was sedated and intubated. He was circulatory stable and he was treated with hypothermia for 24 hours. A urine toxicology screening only showed traces of the anesthetics used during the resuscitation and methylphenidate according to the prescribed dose.\nBoth the initial ECG and the ECGs after hypothermia () showed Q-waves in leads I, AVL, and V4–6 and ventricular ectopic beats. The QT intervals were normal. The echocardiogram did not show any congenital defects but impaired left ventricular function due to regional wall motion abnormalities and thinning of the myocardial wall, consistent with a previous myocardial infarction in the circumflex artery area. There was only a minor increase in the troponin levels, which could be explained by the cardiac arrest, and the initial infection parameters were normal.\nA coronary computed tomography angiography scan showed normal coronary artery anatomy with a dominant left coronary artery and no signs of myocardial bridging. However, a subsequent cardiac magnetic resonance scan showed clear signs of an old myocardial infarction with delayed hyperenhancement and thinning of the lateral wall of the left ventricle, corresponding to the circumflex artery supply ().\nTo exclude paradox emboli, a transesophageal echocardiogram with bubble contrast was done. No atrial septal defects or mural thrombi were found. A full panel of blood coagulation tests did not reveal any coagulation disorder or hypercholesterolemia.\nBefore discharge, a coronary angiogram was done, which confirmed the computed tomography angiography result but also excluded abnormal coronary spasm tendency by a methylergometrine test. The patient also had an invasive electrophysiology study, which showed normal atrioventricular conduction without accessory pathways and an easily inducible sustained ventricular tachycardia, deriving from the scar tissue in the left ventricle. Subsequently, an implantable cardioverter defibrillator was implanted, and the patient was put on a low dose of metoprolol succiante (25 mg), with a treatment plan to increase the dose at a later stage. The methylphenidate treatment was discontinued. A wide panel of genetic disorders related to tachyarrhythmia was examined, which all came out negative.\nThree weeks after discharge the child was on his own in a bouncy castle on a Friday evening. After 15 minutes of exercise, he shortly felt ill and sat down but recovered quickly without any recollection of chest pain or any other discomfort. The following Monday, the defibrillator telemetry showed that the pacemaker had defibrillated an episode of ventricular fibrillation (). Following this episode, the metoprolol succiante dosage was increased to 100 mg o.d. over two weeks. |
A 9 years-old girl in her fourth grade at a public school was noted to have “inadequate response to individualized educational interventions” and so was referred for evaluation by an educational diagnostician. She was assessed to have a specific learning disability related to reading and was placed in a Special Education Program. Her mother was advised that she should expect for her daughter to remain in this program for the duration of her formal schooling (through high school). The following summer she experienced a traumatic emotional loss when her maternal aunt died, at which time according to her mother she began experiencing emotional difficulties, including tendencies for anger and being “closed off.” Her medical and behavioral health history included enuresis (bed-wetting) and seasonal allergies. She used no medications. In the spring of her fifth grade, the child’s mother arranged for a series of 14 sessions using the same allostatic neurotechnology described in Case 1 (), in the hope that doing so would support improvement in her reading comprehension, and also for possible emotional benefits. Initial effects noted by the mother included a greater degree of calm, being “not so wound up,” and more openness. Within about 9 months after the initial sessions, the mother felt that her learning capacity had increased markedly, with improvements demonstrable as faster speed for processing information. Behaviorally, the mother noted that the child had no episodes of enuresis for 9–10 months after starting sessions, followed by a recurrence of episodes that appeared to be related to anticipation of the end of school. The child underwent five more sessions in the winter of sixth grade. By the end of seventh grade, the mother’s impression was that the child was demonstrating significant improvements in reading comprehension. She was composing reports easily and in ways that included advanced word uses, and could communicate orally without stuttering. In the beginning of her eighth grade, the child’s skills were evaluated to be in a range that did not require the Special Education Program, and she began the year in a regular eighth grade classroom. Her initial marks (August 28, 2014) were English B+, Mathematics B, Physical Education A+, Science B+, and Bilingualism A+.\nThe child in Case 2 appeared to demonstrate a combination of behavioral and learning difficulties that may have been exacerbated by an emotional trauma, highlighting the influence of life events across brain domains, and possibly indicating that she was stalled at a developmental stage. Adults for example with complicated grief have been found to have deficits in cognitive function and structural brain changes (). Nocturnal enuresis is reported to be associated with autonomic dysregulation (). In this case study, use of allostatic neurotechnology was associated with improvement in emotional well-being as well as relief from enuresis, and these shifts are conceivably related to recovery from effects of emotional stress () that may have had implications for learning ability. |
A 23 year old young Fulani man presented to the Accident & Emergency room in the early hours of the morning with history of stab in the abdomen with a hand-held cow horn. An hour earlier he was at a local party with his peers when a scuffle ensued and he was stabbed with a cow horn on the left side of the abdomen. He had severe abdominal pain and noticed that his intestine was protruding through the wound. He was helped to the hospital by his friends. There was no loss of consciousness. He bled from the site of injury. No vomiting or abdominal distension. There was no hematuria. Examination revealed an anxious young male who was not pale. He did not have any alcohol smell or evidence of any parenteral drug abuse, but no objective alcohol test was done. There was tachycardia of 92/min and a blood pressure of 100/60mmHg. Chest examination was normal. There was an obvious eviscerated hyperemic loop of bowel in the left paraumbilical region. The abdomen was moderately tender but there was no evidence of significant free peritoneal fluid collection. Rectal exam was normal.\nHe was resuscitated with intravenous fluids and antibiotics, and tetanus prophylaxis was given. Eviscerated bowel was covered with sterile warm moist gauze soaked in saline. Complete blood counts, electrolytes and urea laboratory test results were within normal limits. Abdominal ultrasonography suggested minimal fluid collection in the peritoneal cavity. At surgery, the eviscerated bowel was found to be viable. It was thoroughly lavaged with saline. Via a midline incision, the abdomen was then opened. The peritoneal cavity was found to be relatively clean with just about 30 mls of hemoperitoneum in the pelvis. There was a 7 cm transverse rent involving all layers of the anterior abdominal wall to the left of the umbilicus, through which about 35 cm loop of jejunum eviscerated. There was no mesenteric injury. All other abdominal organs were normal. The eviscerated bowel was reduced with ease. The laceration was debrided and repaired in layers. The abdomen was lavaged with normal saline and the wound closed in layers. Post operative recovery was uneventful and he was discharged home on the 8th post operative day. The patient has remained well and stable at 12-month follow-up. |
A 44-year-old previously healthy Asian female reported to the neurology clinic with complaints of episodic, persistent, uncomfortable needle-like sensations in her genitalia extending to the anal area and the tip of the coccyx. She had been suffering from these symptoms for approximately 11 years. She had difficulty describing the actual nature of this phenomenon, which, according to her, was more of an irritation and discomfort than actual pain. The discomfort was aggravated during rest and periods of inactivity, particularly at night. It responded briefly to mefenamic acid tablets for four to five hours. She also reported the pain was worse before her monthly menstrual cycle but that sexual activity did not affect her discomfort. Sometimes, the discomfort was intense and would wake her from sleep causing severe discomfort and resulting in difficulty sitting; walking would relieve her symptoms partially. She was better in the morning, but the symptoms appeared again at end of the day. She had multiple gynecology and dermatology consults with no relief or clear diagnosis. She was prescribed antifungal creams, including fluconazole and ketoconazole, topical steroids (betamethasone and hydrocortisone), and mefenamic acid for pain relief. All failed to bring relief. She also consulted a homeopathy practitioner, who labeled her as suffering from a chronic skin disease and prescribed oral and topical medication. She took it for a few months but stopped it due to lack of relief. Ultimately, due to the distress and anguish associated with the condition, lack of response to various treatments, and social embarrassment, she stopped visiting doctors for some time. However, during a recent visit, the consulting gynecologist suspected it was not a dermatological or gynecological problem and referred her for a neurology consult.\nThere was no history of back pain, sensory symptoms in the legs, urinary incontinence, or neurological disorder, such as Parkinsonism or restless leg disorder. She had a stable and happy marital life of 22 years and had three children. She has used no substance of abuse or recreational drugs. Her neurological and gynecological examinations, as well as magnetic resonance imaging (MRI) of the spine, were normal (Figure ). Similarly, her biochemical profile (including complete blood count, urine analysis, serum urea, and creatinine) and an ultrasound examination of abdomen and pelvis were normal.\nConsidering her history of circadian rhythm variations in symptoms and aggravation on rest, RGS was considered the most likely cause of her symptoms. After a detailed discussion with the patient, she was prescribed 0.5 mg of ropinirole once at night. It was increased to twice daily after seven days, to which she responded well and reported a dramatic relief of symptoms after 14 days. The dose was increased to 1 mg in the morning and 0.5 mg at night, which led to further improvement. On her third follow-up after two months, she reported taking her medication regularly, which led to almost no episodes of discomfort, with exception of one or two times. The dose of ropinirole was maintained for one month. At the three-month follow-up, she reported only one episode of pain and itching, when she missed one dose of her medicine for a few days; otherwise, she had no complaints and reported no side effects of the medication. |
A 14-year-old girl presented to her primary care center with a severe unilateral headache that had progressed over a 4 h period from typical aura to moderate pain with photophobia and phonophobia to vomiting and to high-intensity headache. Her relevant past medical history included Migraine with Typical Aura (<1 per month) diagnosed at 12 years old after excluding major organic disease. The physical examination was notable for the normal blood pressure and temperature, absence of neurologic deficits and meningeal signs, as well as the absence of any other sign or symptom suggestive of a secondary headache. At this time, the whole clinical picture was compatible with an increased intensity flare of the previously diagnosed migraine with typical aura due to her lack of opportunity to administer herself the abortive therapy at the appropriate moment in time (earlier that morning, during a school class, she had felt the beginning of the aura; unfortunately, her teacher did not allow her to leave the room in order to have access to the abortive treatment).\nAt the primary care center, she received intravenous acetaminophen (paracetamol) and metoclopramide. No more than half an hour had passed when she started developing dystonia of the upper limbs. No improvement of her presenting headache was noted. Decision was made of transferring her to the Pediatrics emergency unit for a diagnostic and therapeutic procedure. After admission to the hospital, these extrapyramidal symptoms were managed with biperiden (a centrally acting antiparkinsonian anticholinergic drug). A few minutes later the teenager became confused, spatially disoriented and with short-term memory disturbance. This acute confusional state lead to a diagnostic procedure which included a lumbar puncture and the consideration of an electroencephalogram, although not immediately available. After disclosure of the laboratory results, which were entirely unremarkable, the patient remained under medical supervision for several hours and the delirium gradually faded away. This evolution helped reinforcing the etiological role of biperiden. During this time, a spontaneous improvement from the migraine was noted as well as total recovery of orientation and confusion. Later on, after admission to the Pediatrics ward, the patient complained of another headache, but this time with a different presentation (frontal pain, associated with the upright position, relieved by lying down). A post-lumbar puncture headache was diagnosed, which prolonged her hospital stay for over 48 h and her home rest and school absence for the following 5 days. At the time of hospital discharge a neurology consultation was scheduled in order to decide for the need of further diagnostic study for the migraine. At that neurology appointment, a brain magnetic resonance imaging (MRI) scan was requested (recall that 2 years before, at her 12-years-old, a diagnostic procedure for a recurrent headache, including a brain computerized tomography (CT) scan, had been done and supported the migraine diagnosis). Indeed, the brain MRI turned out to be, not surprisingly, unremarkable. |
A 76-year-old Caucasian female was referred to our department due to a 6-month history of constant aching pain over her left hip region. Imaging features were very typical of a primary bone tumor and thus, as tentative diagnosis chondrosarcoma was made by the reporting radiologist (). Subsequently performed tumor staging revealed the tumor as being a primary with an otherwise clear bone scan, CT chest, and abdomen. There was no evidence of metastatic disease. However, imaging of the kidneys suggested possible renal cell carcinoma involving the right kidney.\nBiopsy specimen of the proximal left femur revealed a biphasic tumor composed of a cartilaginous component with abrupt transition into a noncartilaginous malignant mesenchymal component (). The diagnosis of a dedifferentiated chondrosarcoma was made. The dedifferentiated part was consistent with an osteosarcoma.\nThe patient underwent limb salvage surgery, performed according to standardized local protocol by surgeons specialized in oncologic surgery, followed by endoprosthetic replacement. Wide surgical margins were achieved. Although the patient was reasonably fit, adjuvant chemotherapy was not given due to the patient's age.\nDuring the further course of treatment, the suspicious condition in the right kidney, that had already been present during initial tumor staging prior to limb salvage surgery, was diagnosed as clear-cell renal cell carcinoma () and immediately subjected to nephrectomy. There was no evidence of metastatic disease. After more than 5 years of followup the patient is still free of disease.\nBesides the current complaints described above, the patient underwent hysterectomy and cholecystectomy years ago. She could not remember the reason and there is no documentation discussing why those operations were performed. However, her medical family history () revealed that her youngest brother started suffering from invasive medium- to-low differentiated urothelial carcinoma () of the bladder when he was 67-year-old. He died at age 69 of a fibroblastic osteosarcoma () located in his left femur. Their mother presented with renal cell carcinoma as well when she was 65-year-old (specimen not available).\nTo assess the hypothesized diagnosis of LFS, DNA was isolated from blood samples of the female index patient, and we analyzed the tumor suppressor gene p53 using semiquantitative, multiplex ligation-dependent probe amplification (MLPA, MRC-Holland) to rule out alterations of the TP53 gene. Moreover, the entire coding (exons 2–11) region and flanking intron regions of TP53 were sequenced. Since both MLPA and sequenciation did not reveal pathologic changes of the TP53 gene, the GAG-banding pattern derived from cell division was analyzed as well. However, chromosomal aberrations were not detected in the cytogenetic analysis neither for structural or numerical reasons. Taking into account all the results obtained, the diagnosis of LFS could not be confirmed, but there is still uncertainty regarding a definitive diagnosis in terms of a possible new genetic syndrome. |
49-yr-old male visited our hospital because of dysphasia, hoa-rseness and a 10 kg weight loss experienced over the previous three months. Approximately five months prior to this visit, the patient was diagnosed with a central lung mass in the left hilar area based on a chest radiograph obtained during a health check. However, the patient refused further evaluation at the time. We performed computed tomography (CT) imaging, which revealed a huge central lung mass obstructing the left main bronchus and the proximal portion of the left upper and lower lobar bronchi. This mass invaded the carina, adjacent esophagus and left main pulmonary artery surrounding the descending thoracic aorta. A bronchoscopic biopsy was performed and the mass was finally confirmed as non-small cell lung cancer, specifically a squamous cell carcinoma. After a 12 cm covered esophageal stent was inserted to relieve dysphagia, chemotherapy was started. About one month later, the patient revisited the emergency department with sudden aggravation of dyspnea and coughing. Respiration rates were initially 36/min and increased to 44/min for one hour after hospitalization. The patient was intubated for mechanical ventilator support and was managed in the intensive care unit. Blood-tinged sputum developed during the first day of admission. Three days after admission, a chest radiography showed the left lung had totally collapsed and bloody secretions were aggravated with no evidence of coagulopathy. The hemoglobin level had dropped from 10.7 g/dL to 8.8 g/dL during the course of one day. At this time, a bronchoscopic examination demonstrated the presence of an endoluminal protruding mass with extrinsic compression from the distal trachea to the left main bronchus. The orifice of the left main bronchus was completely obstructed by the mass and was coated by bloody and fibrinous clots. There was diffuse bleeding from the mucosal surface of the mass without a localized focus. Epinephrine was sprayed on the area to control the bleeding. However, this was only temporarily effective and bleeding continued intermittently. From the eighth day after admission, blood was aspirated through endotracheal tube continuously. Bronchial artery embolization could not be considered as an option to control the bleeding, because it was not active arterial bleeding and there was no prominent contrast enhancement of the mass as seen on a CT scan. Surgical resection also could not be considered because of the advanced stage of the cancer. On the tenth day after admission, a bronchial stent was inserted to achieve a tamponade effect to stop the bleeding and to maintain luminal patency of the left main bronchus. A hydrophilic guidewire (Radiofocus: Terumo, Tokyo, Japan) was inserted under bronchoscopic guidance and an angiographic catheter (Angled Taper; Terumo, Tokyo, Japan) was then advanced into the lower lobar bronchus. A small amount of non-ionic contrast material was injected into the airway to identify the bronchial tree, as we were unable to discriminate the airway on a fluoroscopic image due to the total collapse of the left lung. A stiff guidewire (Amplatz Super Stiff; Boston Scientific, Watertown, MA, USA) was inserted into the left lower lobar bronchus and a 12 mm-6 cm self-expanding polytetrafluoroethylene (PTFE)-covered nitinol stent (Taewoong Medical, Seoul, Korea) was deployed in the left main bronchus under fluoroscopic guidance (). Blood in the endotracheal tube decreased markedly and dyspnea was relieved soon after bronchial stent placement. Two days after the stent placement, the patient was extubated and blood-tinged sputum had disappeared completely four days later (). Radiation therapy was started eleven days after stent placement. During radiation therapy, a total collapse of the left lung again developed. A mass in the proximal left main bronchus that was not covered by the stent was removed by bronchoscopy and left lung aeration improved. The patient expired three months after stent placement due to disease progression. During this period, there was no frank hemoptysis, except for several episodes of blood-tinged sputum. |
A 49-year-old African American woman presented with shortness of breath, who was discharged from the other hospital one week earlier with the diagnosis of acute exacerbation of CHF. Patient was given oral furosemide and carvedilol at discharge, however, she admitted to losing her prescription for carvedilol resulting in her not taking it since discharge. She exhibited bilateral leg edema on examination without crackles or jugular venous distention. Brain natriuretic peptide was elevated to 1330 pg/mL. Chest radiograph demonstrated cardiomegaly with minimal interstitial edema. Echocardiography from the other hospital showed an ejection fraction (EF) of 50%, pulmonary arterial pressure (PAP) of 56 mmHg, and normal left ventricular diastolic filling. Recurrent exacerbation of CHF secondary to idiopathic pulmonary arterial hypertension (PAH) triggered by medication noncompliance was initially diagnosed, and the patient was started on intravenous furosemide. However, no clinical improvement was noted with no reasonable urine output. The medical team doubted the diagnosis of CHF exacerbation and started to do more thorough history taking and a comprehensive physical examination.\nThe patient had a history of breast cancer and underwent left lumpectomy followed by 4 cycles of chemotherapy using docetaxel/cyclophosphamide and radiation therapy about a year ago. She was given 134 mg of docetaxel and 1,074 mg of cyclophosphamide on a monthly basis. On repeat examination, she demonstrated diffuse thickening/sclerosis of bilateral hands that crossed metacarpophalangeal joints extending up to bilateral proximal upper extremities (). Extensive sclerosis of skin and subcutaneous tissue of anterior chest wall and the left breast were also noted. Patient endorsed that she started to have pain and stiffness of both hands right after she finished the last cycle of chemotherapy. Echocardiography before the chemotherapy showed EF of 55%, PAP of 26 mmHg, and normal left ventricular (LV) diastolic filling. LV size has been normal without change before and after the chemotherapy. Therefore, her estimated systolic PAP has increased by more than 2-fold after the chemotherapy with newly developing skin sclerosis. Docetaxel-related systemic sclerosis was suspected. Serologic test showed positive antinuclear antibody, but negative anticentromere and anti-Scl-70 antibodies. Pulmonary function test was normal with slightly decreased diffusing capacity. High-resolution computed tomography of the chest did not show interstitial lung disease. Finally, it was noted that she met the 2013 American College of Rheumatology Classification Criteria of Systemic Sclerosis as she demonstrated skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (score 9) and PAH (score 2). Per the criteria, patients with a total score of ≥9 are classified as having definite systemic sclerosis [].\nWe discontinued diuretic treatment and continued on beta-blocker with calcium-channel blocker. Patient's clinical condition improved and was discharged with follow-up appointment with Rheumatology and Pulmonary Hypertension (PH) clinic.\nUpon follow-up chart review, the patient is currently following Rheumatology, Pulmonology, and Dermatology but is also on hemodialysis given worsening renal function with following Nephrology. Renal biopsy was performed and it showed thrombotic microangiopathy pattern (classic “onion-peeling” appearance) which is suggestive of scleroderma per pathologic documentation. She was diagnosed with scleroderma renal crisis by Nephrology and was started on captopril. Patient is currently stable with normal blood pressure and stable serum creatinine levels. |
A 45-year-old woman presented to medical attention for abdominal pain, episodic diarrhea, and a mild weight loss (<10% body weight). Informed consent was obtained from patients included in the study. All procedures performed were in accordance with the ethical standards of our institutional research committee. She underwent a colonoscopy that showed a 4 cm stenosis of the sigmoid colon, covered with pale mucosa that could not be passed by a conventional colonscope (13.2 mm) but could be passed with an enteroscope (9.2 mm) with which the cecum could be reached; the mucosa of the other colonic tract, as well as the mucosa of the terminal ileum, were unremarkable. Random ileal and colonic biopsies were obtained, which were unremarkable as well. Histology of the stenosis showed mild inflammation, mild stromal fibrosis, and architectural disruption. Subsequently, she underwent a computed tomography scan, which showed a mild thickening of the terminal ileum and sigmoid colon. The finding of thickened terminal ileum was further confirmed by a subsequent magnetic resonance imaging enterography, suggestive for Crohn disease (CD) localized at the terminal ileum. Due to the discrepancy of the radiological findings with the initial endoscopic examination, a further colonscopy with retrograde ileoscopy was performed, showing no visible endoscopic signs of inflammation and confirming the sigmoid stenosis. The repeated ileal and colonic biopsy sampling was again not diagnostic, showing a nonspecific pattern of inflammation affecting the sigmoid colonic specimens.\nThe physician in charge of the patient, in view of her clinical history and the radiologic finding of intestinal thickening, reckoned that the patient was affected by CD with terminal ileum and sigmoid colon localization and proposed a therapy immunomodulators: corticosteroids 1 mg/kg for 12 weeks together with antitumor necrosis factor (TNF) infliximab 5 mg at weeks 0, 2, and 6, followed by infliximab alone. After 6 months of treatment with infliximab (5 mg/kg every 8 weeks as maintenance), an endoscopic reevaluation was scheduled, showing a resolution of the stenosis in the sigmoid colon and a normal-appearing terminal ileum. The marked improvement of the sigmoid inflammation together with the finding of fibrosis on the corresponding histology further convinced the physician in charge of the patient in favor of a diagnosis of CD, and the anti-TNF treatment with infliximab 5 mg/kg was continued. After 3 months (9 months after the beginning of anti-TNF), the patient was admitted for obstructive symptoms. A computed tomography suspected neoplasia of ileocecal region. The patient underwent an uneventful ileocecal surgical resection. The surgical specimen consisted of a portion of 8 cm of terminal ileum with the cecum and the vermiform appendix. The ileal wall showed an increased consistency and a diffuse thickening, with cystic-hemorrhagic foci in the perivisceral fat. The ileal mucosa was corrugated and showed a polypoid structure with a wide base (1.5 cm maximum diameter) originating from the submucosa and determining ulceration of the mucosal layer. The ileocecal valve, the cecum, and the appendix were normal, except for a mild lipomatous filling of the submucosa. |
This patient is a 29-year-old white woman from the USA with a medical history significant for severe IBS-D (diagnosed at age 12) and anxiety disorder. In July 2015, she presented with severe bleeding hemorrhoids secondary to IBS, which required hemorrhoidectomy and anal sphincterotomy in August 2015. The week before the surgery she developed pharyngitis and was treated with azithromycin, which resulted in mucousy diarrhea and abdominal discomfort. She tested negative for C. difficile antigen and toxins at that time.\nA week after surgery, she developed a perirectal abscess that had formed at the site of the sphincterotomy and was prescribed orally administered ciprofloxacin. Despite moderate symptom improvement, in September 2015 she required an abscess incision and drainage procedure and Penrose drain insertion. Prior to the surgery she was given a single dose of clindamycin. An additional 2-week course of ciprofloxacin and metronidazole was then prescribed. In late September 2015 she was admitted to the hospital for two nights due to further complications related to the abscess and was then diagnosed as having a perianal fistula.\nIn November 2015, she was prescribed clindamycin for an episode of group C streptococcal-positive pharyngitis. In late November 2015, she was also diagnosed as having Ehlers–Danlos syndrome, which according to her medical record may partially explain the poor wound healing from the perirectal abscess. In December 2015, her fistula required an anus seton placement. She was treated with multiple courses of ciprofloxacin and metronidazole off and on from December 2015 to January 2016.\nIn January 2016, following up on her recurrent pharyngitis, she was diagnosed as having chronic tonsillitis which led to tonsillectomy. In February 2016, 2 weeks after the surgery she was prescribed clindamycin. At the beginning of March 2016, she was diagnosed as having bacterial vaginosis and was prescribed orally administered metronidazole. A week later she was diagnosed as having vaginal candidiasis and was prescribed orally administered fluconazole. In April 2016, she complained of dysuria and was prescribed ciprofloxacin. After 2 days, when urine analysis results came back negative, she was asked by her physician to stop the treatment.\nIn June 2016, she presented for follow-up with ongoing diarrhea and abdominal pain. She was diagnosed as having C. difficile diarrhea, her antigen and toxins laboratory results were indeterminate, and a toxigenic strain was confirmed by polymerase chain reaction (PCR). She was prescribed a 6-week course of orally administered vancomycin. After a week of treatment her symptoms worsened, and following discussion with her gastroenterologist her treatment was switched to a 2-week course of metronidazole. Hours later, she was admitted to the hospital for a 4-day period for colitis. Her C. difficile antigen and toxin test returned negative during her admission. She received intravenously administered metronidazole treatment during her hospitalization. Her symptoms improved during her hospital stay, with 1–2 soft bowel movements a day. At discharge her metronidazole course was stopped and she was again prescribed vancomycin, which she took for over a month. She continued to experience GI irregularity (3–5 bowel movements a day) beyond what she had experienced secondary to her IBS prior to her surgeries. In March 2017, she was prescribed rifaximin for 2 weeks to treat chronic diarrhea.\nIn November 2017, she was prescribed a series of clinical intestinal tests (SmartGut™, uBiome Inc., San Francisco, USA) with the instructions to administer the test at home whenever she was experiencing a noticeable change of GI symptoms, then follow-up with her health care provider to discuss the results. This sequencing-based test requires that patients use a sterile swab to transfer a small amount of fecal material from toilet paper into a vial containing a lysis and stabilization buffer that preserves the microbial DNA for transport by mail back to the laboratory for processing, which involves DNA extraction, 16S ribosomal RNA (rRNA) gene amplification, and sequencing []. She first used this test in November 2017, about a month after completing a 2-week course of rifaximin. The results revealed a number of microbial organisms that were outside the healthy reference ranges, but she was negative for all pathogenic organisms included in the test, including C. difficile (Fig. ).\nBetween November and December 2017, her GI symptoms worsened considerably; her daily bowel movements increased from 3–4 to 6–10, stool consistency became more mucous-like and gelatinous, and she was experiencing more pain with defecation. She re-tested with SmartGut™ test again in January 2018. Her results continued to reveal a number of microbial organisms outside the healthy range and, this time, her sample also indicated the presence of C. difficile (Fig. ). She immediately contacted her primary care provider, who re-tested her for C. difficile and confirmed indeterminate CDI by antigen and toxins A and B. Additional PCR testing at a regional laboratory confirmed the sample was positive for a toxigenic C. difficile strain. As a result of testing, her clinician started her on fidaxomicin; her symptoms improved rapidly. By April 2018, she had returned to her baseline in regard to her IBS-related GI symptoms with no blood in her stools.\nIn addition, the second SmartGut™ sample was tested for toxins A and B by sequencing at uBiome Inc. laboratory in San Francisco, USA, which resulted positive for both and confirmed the toxigenic nature of the C. difficile strain. |
A Caucasian 92-year-old lady attended the emergency department with acute onset epigastric/right hypochondrial pain, waking the patient from sleep. The pain was described as being constant since onset and radiated through to her back. The pain was immediately followed by profuse and intractable vomiting. There had been no previous episodes of similar pain and she had been well in the preceding days. Her past medical history included an open appendicectomy, hypertension, GORD, previous TIA, left carotid endarterectomy, peripheral vascular disease and a L2 crush fracture. The patient denied fevers/rigors and had no recent history of lower urinary tract symptoms or altered bowel habit. The vital signs recorded for the patient were within normal limits and she was afebrile. On examination of the patient’s abdomen it was noted that the abdomen was soft and not distended, with diffuse tenderness down the right side. The gallbladder was also palpable to the right iliac fossa. The white cell count was mildly elevated at 13.2, while there were no electrolyte abnormalities and no renal or liver function derangement evident on blood tests. A CT scan of the abdomen and pelvis (Fig. ) was arranged with contrast and was reported as showing a ‘distended gallbladder with prominent wall thickening and mild adjacent inflammatory change. The appearances are most consistent with cholecystitis’. An ultrasound was then performed that demonstrated a calculus together with minor gallbladder wall thickening but no evidence of biliary ductal dilatation. Thus, the patient was started on intravenous antibiotic therapy as per local guidelines for a presumed diagnosis of acute cholecystitis and admitted under the care of the general surgeons. The patient was reviewed on rounds the following morning and was noted to have increasing pain despite analgesia associated with tachycardia, tachypnoea and diaphoresis with a peritonitic abdomen. The white cell count was now elevated at 17.6 with a CRP of 198 and the liver function tests remained within normal limits. The patient was reimaged with a further CT abdomen/pelvis with contrast which was reported as showing ‘cholelithiasis and choledocholithiasis with intra and extra bile duct dilatation as well as marked gallbladder distension and pericholecystic fluid’. An emergency laparotomy was performed with findings on entry of a non-viable and gangrenous gallbladder secondary to gallbladder volvulus. The images below show the volvulus as well as the cystic duct and cystic artery after being detorted (Fig. ). The patient was managed in ICU immediately postoperatively but medically fit for discharge after five nights in hospital. |
A 17-year-old female presented to the ED with one day of dull, intermittent, 6/10 abdominal pain originating in the periumbilical region and migrating to the right lower quadrant. She complained of nausea and decrease in appetite but no vomiting or diarrhea. She reported she had not had a bowel movement in three days, a normal pattern for her. She denied fever, chills or dysuria. Her medical history was positive for depression and shellfish allergy. Family history and review of systems were negative. Vital signs were normal, and the only pertinent physical exam finding was moderate tenderness in the right lower quadrant with voluntary guarding without rebound. Laboratory data revealed a normal white blood count (WBC), urinalysis, electrolytes, blood urea nitrogen and creatinine and a negative urine pregnancy test. Computed tomography (CT) of the abdomen and pelvis showed no evidence of appendicitis nor renal, hepatic, adrenal, pancreatic or bladder abnormalities. The radiologist noted a low-lying cecum dilated to 8cm but no evidence of obstruction (). A pelvic ultrasound revealed two 1.5cm right ovarian cysts. With time the patient reported pain relief, and re-examination of her abdomen was unremarkable. She was discharged with instructions to follow up with her pediatrician and gynecologist.\nThe patient returned to the ED 11 hours later reporting recurring abdominal pain. The pain had increased in intensity from the previous day and was now associated with vomiting. Physical exam was significant for tenderness in the right lower quadrant with voluntary guarding. The patient refused a pelvic examination during her first visit, and the exam now failed to reveal any cervical motion tenderness, adnexal masses or tenderness. Complete blood count and chemistry profile were again normal and the patient was discharged once her pain improved with a diagnosis of abdominal pain secondary to ovarian cysts.\nThe patient returned to the ED 27 hours after the second visit reporting severe abdominal pain in the right lower quadrant, nausea, vomiting, and no bowel movement in four days. Her abdominal exam revealed decreased bowel sounds, distention and tenderness in the right lower quadrant with voluntary guarding. WBC was elevated (13.6) and a repeat CT showed marked dilatation of the cecum up to 10.5 cm with a transition point in the right lower quadrant suspicious for obstruction and cecal volvulus ().\nThe patient was taken to the operating room for laparatomy and reduction of cecal volvulus. Surgical findings revealed a mobile cecum with serosal tears and marked distention, and the patient underwent a right hemicolectomy. Pathologic findings of the surgical specimen revealed markedly dilated ileum and cecum with evidence of ischemic necrosis. The remainder of the patient’s hospital course was unremarkable, and she was discharged on post-operative day five. |
A 69-year-old female with history of type 2 diabetes, hypertension and dyslipidemia complained of intermittent left hip pain for 3 years with constitutional symptoms. She denied any headache, hearing loss, tinnitus, back pain, joint pain or failure symptoms. There was no history of fracture, trauma or heart failure. On examination, she was unable to ambulate due to her left hip pain. There was no bowing of tibia or femur. Apart from her left hip, her musculoskeletal examinations of other joints and neurological examination were unremarkable. Biochemical tests revealed normal serum calcium and phosphorus with markedly raised alkaline phosphatase (). The initial differential diagnoses were osteoarthritis, osteoporosis, secondary metastasis or metabolic bone diseases. Her Xray of the hip revealed diffuse sclerotic left pelvic bone and Magnetic Resonance Imaging (MRI) of the hip revealed extensive marrow signal abnormalities at the left pelvic bone (). The radiological images above were reported as primary, metastatic bone disease or other metabolic bone disorders like PDB. As part of workup of bony lesion in adult to rule out possibility of secondary malignancy, contrast enhanced computed tomography of thorax, abdomen and pelvis (CECT-TAP) were performed which revealed a spiculated lung nodule at the left lower lung lobe, suggestive of lung malignancy ().\nIn order to ascertain the lesion being a primary, metastatic bone tumour or PDB, an open biopsy of the left iliac bone was done. The histopathology result of bone biopsy of the left iliac bone was later reported as Paget’s disease of the bone (). A CT guided biopsy of the lung mass was done later which revealed adenocarcinoma of the lung (). She had F-FDG PET-CECT Scan for staging evaluation and showed intense tracer uptake in the left lower lobe of the lung with multiple mediastinal lymph nodes as well as right sacral bone, right ilium and bodies of L3-L5 suggestive of new bony metastases. There was also diffuse sclerosis of the left hemi pelvis (ilium, ischium and pubic bone) with minimal metabolic activity in keeping with PDB ( and ). The final diagnosis was metastatic bone disease secondary to adenocarcinoma of the lung with underlying PDB. Dual energy X-ray absorptiometry (DEXA) scan was unable to be interpreted due to osteoblastic changes at the lumbar spine and hip. Her echocardiogram did not show any systolic or diastolic dysfunction.\nPatient was initiated on high dose Vitamin D and subsequently IV Zoledronic acid for the treatment of the PDB. In view of the stage 4 lung adenocarcinoma with bony metastases, patient was scheduled for palliative systemic chemotherapy. |
A 67-year-old man with a history of coronary artery bypass grafting underwent a mitral and tricuspid valve repair through a redo sternotomy performed at an outside hospital. He was readmitted one month following surgery with serous drainage from his wound. Imaging demonstrated inferior sternal widening with fractured wires and a substernal fluid collection. Operative exploration revealed no purulent fluid, cultures were obtained, and the fluid collection was felt to be a result of the dehiscence. The sternum was closed with wires, reinforced with lower sternal plates under myocutaneous flaps, and the skin was left open with a wound vac. While undergoing vac changes, his sternal and soft tissue cultures grew Mycobacterium abscessus that was macrolide sensitive. Infectious disease was consulted, and he was placed on an appropriate triple antibiotic regimen. The patient returned to the operating room with the assistance of plastic surgery for removal of the lower sternal hardware, sternal debridement, bilateral pectoralis advancement flaps, and skin closure. Cultures taken during this final operation eventually grew Mycobacterium abscessus. After extensive multidisciplinary discussions, further chest wall resection was felt to be high-risk. A referral was placed to a center with expertise and he was discharged on oral azithromycin, intravenous amikacin, and intravenous imipenem.\nDuring the ensuing months, he was monitored closely as an outpatient. Drainage from the inferior wound recurred four months following discharge, prompting referral to our institution. Imaging revealed a substernal fluid collection with a draining sinus tract, three upper sternal wires, and sternal sclerosis (Figure postoperative). Transthoracic echocardiogram demonstrated competent valve repairs and no vegetations. Coronary angiography demonstrated a patent left internal mammary artery (LIMA) graft crossing directly beneath the left upper sternum. The case was reviewed with colleagues in infectious disease and plastic surgery. The decision was made to pursue radical surgical resection, removal of all hardware, serial debridements, and delayed sternal reconstruction after operative cultures cleared. Preoperative nutritional assessment demonstrated adequate nourishment. His antibiotic regimen was expanded to four agents including oral azithromycin, intravenous imipenem, intravenous tigecycline, and intravenous clofazimine.\nHe was taken to the operating room seven months following his initial valve repair. The sternal scar was excised. An abscess was encountered in the soft tissue at the lower third of the sternal incision that tracked through the pectoralis muscle flaps, inferior sternum, and into the mediastinum (Figure ). The inferior sternum was sclerotic and grossly diseased. Tissue cultures were obtained, the remaining sternal wires were removed, and a redo sternotomy with nearly complete sternectomy was performed. The left manubrium and upper sternum were not removed as they were remote from the nidus of infection, appeared normal, and we did not want to risk injury to the patent LIMA graft adherent to its underside (Figure ). The abscess cavity was debrided to bleeding tissue except for that directly over the heart. A pulse lavage system (Stryker Inc, Mahwah, NJ) was used to irrigate all exposed mediastinal surfaces with bacitracin (50,000 U/3 L 0.9% normal saline). Absorbable synthetic calcium sulfate beads (Stimulan, Biocomposites, UK) infused with amikacin (5 g in 50 cc of beads) were placed in the wound bed (Figure ). A temporary wound vac was placed, and the patient was admitted to the cardiac surgery ward.\nTissue staining revealed granulomas with acid-fast bacilli in the connective tissue surrounding the lower sternum, but no active infection of the bone (Figure ). Weekly operative debridements were performed while obtaining new cultures and replacing the amikacin beads. At five weeks following initial sternectomy, cultures showed no growth. Wound closure required an omental flap to fill the spatial defect. A pedicled omental flap supplied by the right gastroepiploic artery was tunneled through the diaphragm into the mediastinum (Figure ). Plastic surgery elevated bilateral full thickness skin flaps to the level of the remaining pectoralis major fascia. Indocyanine green fluorescence angiography confirmed vascularization of both omental and skin flaps. The skin flaps were advanced over the omentum and closed primarily over drains (Figure ).\nThe remainder of the patient's hospital course was uncomplicated, and he was discharged home after removal of both drains. He was monitored closely as an outpatient on the aforementioned guideline-directed antibiotic regimen. This was continued for four months following sternectomy, the recommended course for serious complex soft tissue infections. At one year following surgery, he has no evidence of recurrent infection. |
A 61-year-old man with no premorbid illness presented to the emergency department with concerns of chest pain and shortness of breath. Upon review of an electrocardiogram (ECG) showing an S1Q3T3 pattern and elevated d-dimer levels, a computerized tomography (CT) angiogram of the chest was performed that showed a moderately sized pulmonary embolism in the pulmonary artery with the right greater than left (Figure ). Anticoagulation therapy was initiated for the patient, but due to the high Pulmonary Embolism Severity Index score and evidence of right ventricular dilatation with a pulmonary artery systolic pressure (PASP) of 50 to 55 mmHg, the patient was taken to the catheterization laboratory []. A catheter-directed infusion of alteplase was performed []. After completion of the infusion, anticoagulation therapy with enoxaparin was continued, and there were no complications. Four days later, a repeat ECG was performed, and a persistent right ventricular strain with right ventricular dilation and an increase in PASP to 70 to 75 mmHg were shown. Based on this, a percutaneous thrombectomy of the right pulmonary artery was performed. This has led to successful recanalization of the pulmonary artery and resulted in a significant reduction in pulmonary arterial pressure. The patient was discharged and given DOACs.\nHe returned after five weeks with concerns of severe right-sided pleuritic chest pain. On further questioning, he revealed that he had stopped taking the DOAC for one week as his insurance did not cover the medication. A chest x-ray was performed and revealed evidence of an apparent cavity in the posterior right base of the lung measuring 3 cm x 4 cm x 3.6 cm with a thick irregular wall (Figure ). This finding was not present in the chest x-ray done a few days earlier. A CT angiogram of the chest was repeated that showed resolving bilateral pulmonary emboli and an interval development of a 4.2 cm x 6 cm x 5 cm cavitary mass in the right lower lobe (Figure ). The patient had no history of fever, recent travel, or loss of weight. To rule out tuberculosis, gamma interferon testing was done; the results were negative, and blood cultures were repeatedly negative for Mycobacterium tuberculosis. He was treated with ceftriaxone and metronidazole as a lung abscess could not be ruled out. A subsequent chest x-ray showed an air-fluid level in the cavity, but as the patient was clinically improved, he was discharged with DOAC and educated regarding compliance.\nOne month after discharge from the hospital, he presented to the emergency room with pain in the right lower chest but said the pain had significantly reduced in intensity. He claimed to be compliant with the DOAC, and a repeat CT angiogram was performed. The CT showed possible persistent stenosis or partial thrombosis of the right pulmonary artery branch vessel within the right mid-lung; a persistent 2-cm air space opacity within the posterior right lower lobe with a small cavitary component was significantly reduced from what had been seen in the previous CT (Figure ). |
A 21-year-old non-smoking young man presented with complaints of general fatigue and rapid weight loss in the past 3 months. During this period, he was referred to a local hospital for radiological examination. Computed tomographic (CT) scans revealed a huge mass in the left lung. A fibreoptic bronchoscopy was performed in the local hospital, but biopsy examination was negative because only inflammatory mucosa was observed under the microscopy. As a result, the patient was referred to our hospital for examination and treatment. Physical examination results were normal. The laboratory results, including blood count, serum tumor markers and liver and renal function, were within the normal range. The CT images acquired at the local hospital showed a 10.0 × 8.0 cm well-circumscribed mass in the left lower lobe of lung without signs of neighboring pleura invasion (Fig. and ). There was no enlarged lymph node of the pleural cavity found. Whole body F18-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT study was not preformed at that time because there was no sign of tumor distant metastasis. Since the patient was a young adult, the preoperative impression of the lesion was a mesenchymal sarcoma of lung. The patient underwent a left lobe-ectomy of lung, and the mass was gross totally resected. Postoperative recovery was uneventful without surgical complications. After diagnosis, the patient received Crizotinib, an oral anaplastic lymphoma kinase inhibitor, at a dose of 250 mg twice daily. However, the patient rapidly presented back pain and left lower limb dysfunction at the third month after pulmonary surgery. The patient was then referred to a whole body PET/CT study to search for the potentially secondary tumor. PET/CT revealed that multiple abnormally increased FDG uptake was found in pelvic bone and vertebra. An intraspinal mass at T10 was also observed (Fig. ). A second laminectomy was performed under the impression of tumor metastasis, and the majority of the intraspinal mass was resected. However, the patient developed persistent fever and cachexia. Finally, he died 4 months after the initial pulmonary surgery.\nThe surgical specimens was received and routinely fixed in 10 % neutral buffered formalin after tumor resection. Four micrometer thick sections were cut and stained with H&E. On microscopic examination, the pulmonary and intraspinal masses exhibited similar histological appearance. Both tumors were non-encapsulated and had an infiltrative margin. The pulmonary tumor was composed of two areas with distinct histological appearance. In one area, the interlacing bundles of spindle cells were found to intermingle with plasma cells and lymphocytes. The plump polygonal-like myofibroblasts with prominent nucleoli were also observed in myxoid background. The microscopical appearance of this area was consistent with the histological features of conventional IMT (Fig. -). However, in other area, the tumor was composed of diffused well-circumscribed polygonal and epithelioid tumor cells with distinct nucleoli and eosinophilic or pale cytoplasm. Nuclear atypia, mitotic figures (3/10 high power field) and focal necrosis were observed. Background of tumor in this area showed myxoid change with inflammatory cells infiltration, mainly neutrophils, eosinophils and lymphocytes. The intraspinal tumor was composed of only polygonal and epithelioid tumor cells. The spindle cell component was not present in intraspinal tumor (Fig. -).\nImmunohistochemically, the tumor cells in both messes were positive for vimentin and desmin diffusely. They were negative for Pan-CK (AE1/AE3), smooth muscle actin (SMA), HHF35, myogenin, Myo D1, S100, HMB45, Melan A, synaptophysin, CD34, CD68 and CD30. In addition, the tumor cells were positive for ALK, exhibiting a cytoplasmic pattern (Fig. -). The rearrangement of the ALK gene at chromosome band 2p23 was detected by fluorescence in situ hybridization (FISH) utilizing Vysis ALK break apart probe (Vysis, Abbott Laboratories Inc., Maidenhead, UK). FISH analysis showed rearrangement of ALK present in both pulmonary and intraspinal masses (Fig. ).\nOn the basis of gross, histopathological features and immunohistochemical phenotypes, its epithelioid morphology and myxoid background, the presence of inflammatory infiltrating and ALK cytoplasmic positive signal pattern, a diagnosis of epithelioid inflammatory myofibroblastic sarcoma (EIMS) with multiple bone metastases was made. |
A 54-year-old gentleman with cervical compressive spondylotic myelopathy and multiple congenital anomalies of the cervical spine such as cranio-vertebral junction anomaly with occipit-C1 synostosis and C4-C5 fused vertebrae [] underwent C3 median corpectomy, gardening, iliac crest graft placement, and stabilization using titanium plate and screws from C2 to C4 vertebral bodies []. Postoperatively, he had improvement in his symptomatology. He reviewed after 8 years with complaint that a screw had expelled through his mouth while having food. There was history of progressive difficulty in swallowing with occasional regurgitation of feeds of 2 months duration. He had no history of recent trauma or worsening of power in his limbs. He was conscious and his neurological examination was normal except for exaggerated deep tendon reflexes. On oral examination, there was a defect in the posterior pharyngeal wall, through which cervical plate along with a missed fixation screw and intact self-locking screw was seen []. X-ray cervical spine lateral view showed anterior dislodgement and migration of the plate with a missing screw socket []. Oral culture was taken, and he was started on intravenous antibiotics, betadine oral gargles, and was fed through a nasogastric tube. At resurgery, no abnormal movement was seen, but the plate and screw system was loose and had migrated, with no evidence of fracture of the plate system. The plate was removed in toto after removing the screws []. As the patient had no new neurological deterioration and no abnormal movement was seen intraoperatively, no further instrumentation was done. He was planned for posterior fusion in case he developed new neurological deficits or signs of instability. Postoperatively there was no injury to the peripheral nerves or cranial nerves. Since the posterior pharyngeal wall defect was small, it was expected to close over a period of time; hence, no attempt was made to close it. Postoperatively he was continued on nasogastric tube feeding and intravenous antibiotics for 2 weeks. Regular inspections were made at the posterior pharyngeal wall to rule out fistula formation. The posterior pharyngeal wall defect had closed by 3 weeks without any gaping/fistula formation. At 11 months of follow-up, the patient is asymptomatic with normal lower cranial nerves’ function and with no signs of instability or new neurological deficits. |
A 71-year-old overweight man with no known comorbidities presented to an orthopedic outpatient unit of our institution with complaints of trivial left hip pain and difficulty in walking for 1 month. He used to walk regularly and was able to perform his routine activities to begin with. However, the pain progressively increased in intensity with slight worsening for 5 days relieved to an extent after consumption of oral analgesics. There was no history of trauma or any precipitating event. Laboratory investigations showed low serum Vitamin D3 level at 20 ng/ml (normal >30 ng/ml). Serum calcium and phosphate levels were within normal limits. On clinical examination, there were no local signs of inflammation except for slight tenderness over Scarpa's triangle. Femoral nerve stretch test was negative ruling out possibility of lumbar radiculopathy. In view of his complaints and clinical suspicion of a possible fracture, the patient underwent anteroposterior radiograph of pelvis [], which showed no obvious fracture or displacement with maintained contours of visualized bones. Shenton's line was preserved with no clear disruption. The patient was then referred to Nuclear Medicine Department for 99mTc-MDP bone scintigraphy. Triphasic bone scan was performed. 20 mCi of 99mTc-MDP was injected intravenously with pelvis positioned in the field of view of gamma camera. No abnormal perfusion was noticed to the symptomatic region on arterial phase. Mildly increased pooling of radiotracer in the left hip region on 2 min initial static image was observed. Delayed skeletal phase image acquired at 3 h showed crescent shaped area of intense tracer uptake in femoral neck region []. Rest of the skeletal system was unremarkable. On subsequent fused SPECT/CT performed for further characterization, abnormal tracer uptake localized to subcapital left femoral neck with diffuse sclerosis on corresponding CT image []. No obvious fracture or displacement was noted. Considering patient's age, timing of injury, unexplained left hip pain, and factors such as obesity, osteoporosis, and radiologically equivocal findings, the imaging characteristics on bone scan pointed toward stress fracture. The patient underwent open reduction and internal fixation with dynamic hip screw plate and cancellous screws. Postsurgery, the patient, was referred for a rehabilitation program. The patient was clinically better at the time of the last follow-up. |
The patient, a 65-year-old woman, was admitted to the geriatric psychiatry inpatient unit due to depression, severe anxiety, paranoia, delirium, and extreme mood swings. She reported poor sleep and poor appetite and had expressed problems with concentration. She also reported painful migraines and had been admitted to a community hospital for a migraine episode prior to admission. When asked about headache triggers, the patient noticed that caffeine, particularly coffee, and loud voices were possible triggers but otherwise had not noticed any other trigger patterns for her chronic migraines. At least 2 reports of migraine headaches coinciding with overmedication were found within the 2 weeks prior to admission in external records (). Her relevant past medical history included depression, frequent migraines, arthritis, and chronic neck and spine pain from her degenerative spine condition. Her family history included migraines in her maternal grandmother, and her social history included substance abuse with opioids.\nThe patient reported being compliant with medications, though she stated that she had decreased her topiramate prior to admission due to her concern about weight loss. The patient had been seeing a psychotherapist and psychiatrist but failed to follow up after relocating and facing financial burden. The patient last saw her psychiatrist about a year prior to admission, and all her psychiatric medications were being prescribed by her neurologist. She also had a pain specialist who at first prescribed extended-release morphine sulfate 30 mg twice daily for her chronic spine pain but then switched her to oxycodone 5 mg 5 times daily prior to admission. All medications dispensed 3 months prior to admission came from 2 pharmacies.\nAccording to the patient, her current life stressors included finances, adjusting to her new home, and lack of sleep. During the first interview, the patient reported that she was addicted to morphine and was withdrawing from it. She also reported not abusing her medications and only following the instructions on the medication labels. Her husband and primary care physician did not believe she was abusing her medications.\nFor migraine treatment, the patient reported that she had tried beta blockers in the past and those did not work for her. The patient also reported that her divalproex delayed release was discontinued and that acetaminophen “spiked” her liver enzymes. Additionally, the patient mentioned that her neurologist said she was a candidate for botulinum toxin injection and before admission had scheduled her to have a treatment within the next couple of months. The patient admitted to consuming caffeinated sodas around the clock prior to admission.\nDuring admission, the patient's problem list included bipolar II disorder, anxiety, insomnia, migraines, chronic spinal pain, neuropathic pain, arthritis, hypertension, hypothyroidism, and gastroesophageal reflux disease. Bipolar II disorder was a new diagnosis during this admission, and relevant medications were added or increased in dose to minimize side effects and optimize overall therapy. Reports were unclear as to whether the patient's frequent migraines existed prior to medication overuse or if medication overuse caused the frequent migraines. Regardless, the patient was converted to oxycodone, topiramate was titrated to 100 mg daily for migraine prophylaxis, and the patient was counseled to reduce caffeine consumption. Also, methylphenidate was originally not included in overall therapy due to risk of worsening migraines but was eventually added at a low dose to improve patient affect and energy. Neuropathic pain was another new diagnosis during admission, and thus gabapentin was added into overall therapy. Toward the time of discharge, the patient reported worsening arthritis that affected her sleep, and naproxen was prescribed as needed to address the pain and improve sleep. The patient's overall pain ranged between 3 and 8 out of 10; however, most scores hovered around 5 of 10 with 10 being the worst pain imaginable.\nHer vital signs were all within normal limits and renal function remained normal. The medications prescribed upon discharge after 3 weeks of admission are shown in .\nThe patient was referred for follow-up with her neurologist and a new pain specialist upon discharge. |
The patient is an 85-year old man, who was referred to our center for BCC recurrence after previous resection in almost 30 years ago () . He underwent an incisional biopsy of lesion, with the result of BCC. In the mentioned surgery, median flap was used to cover the defected area. After a careful clinical and radiological evaluation, a surgical treatment with the demolition of the lesion and a wide removal of the region was planned in order to attain a safe margin. In the latter resection, at first, mucosal and osteocartilaginous lining were made and then paramedian flap in a size of 9×8 cm, raised from the left side of forehead, was used for coverage. Each of the supratrochlear arteries was absent because they have been sacrificed in the previous median flap surgery.\nAs it is well known, vascular pedicles in both sides of nasofrontal zone, containing supratrochlear arteries as axial, is the basis of median flap. If the stalk of flap is dissected for any reasons, other vessels and their multiple anastomoses supply forehead and supraorbital zones. In this case, we used paramedian flap in base of previous median flap pedicle. Since all of the cardinal arteries were dissected in the previous surgery, as the narrowed remained pedicle, the blood supply of the used paramedian forehead flap was only based on blood supply from a rich plexus of the anastomosing vessels from the terminal branches of angular artery in the nasal bridge.\nUnlike the absence of a certain arterial supply in the flap pedicle there was not any ischemic event after the flap raised and inset. Then the patient candidate for three stage flap operation. The long term follow-up did not show evidence of any abnormalities of the wound or recurrence of the tumor (). Median and paramedian forehead flaps have been recommended for insetting of defects greater than 2.5-3 cm in diameter, especially when the cartilage framework has been ravaged; for their success, it is important to preserve the vascular pedicle of these flaps (supratrochlear artery) and obtain sufficient thinning of the subcutaneous tissue from the distal flap. The paramedian forehead flap is a useful flap with a resilient vascular supply that is largely used for reconstruction of complex or large nasal defects.-\nIn conclusion, for special occasions, to inset in defected area, it is acceptable to raise paramedian flap in base of previously used flap pedicles, without the presence of any axial arteries and only in base of rich anastomotic arterial plexus in the nasofrontal angle of each side. |
A 70-year old woman was admitted from a dermatologist to the Department of Plastic and Breast Surgery, Roskilde, Denmark. A biopsy verified well-differentiated full-thickness squamous cell cancer was found at the right side of the lower lip. The tumour had been present for three years. The patient had a history of ischemic heart disease with a percutanous coronary intervention and implantation of a stent in 2002. She was a heavy smoker. No alcohol overconsumption was reported. By clinical examination, a 15 × 15 mm sore, firm, central ulcerating tumour was seen located at the vermillion border (). At the mucosal side of the lip, leukoplakia was observed.\nNo enlarged lymph nodes in the cervical region was found at the clinical examination.\nSurgical intervention would remove approximately 50% of the lower lip. The patient was offered radiotherapy as an alternative to surgery and chose the former. She underwent a series of radiotherapy for a period of two months.\nFive weeks after the initial clinical examination at our clinic, the patient was readmitted to our department from the Department of Oncology at Copenhagen University Hospital due to recurrence of the squamous cell cancer located to the lower lip. At this point, the patient presented a monstrous, ulcerating tumour, involving most of the lower lip (). No clinical signs of systemic spread to lymph nodes in area was found by thorough clinical examination and palpation of the head and neck. Furthermore, a 18FDG-PET-CT was performed and ruled out further local or systemic spread.\nFirst, a total excision of the lower lip including the commisures during which free resection borders of 1 cm were confirmed by intraoperative frozen section diagnosis. 8 days after primary excision, histopathology confirmed free resection borders, and reconstruction of the lower lip by bilateral Fujimori technique was performed (, , ). Due to paucity of mucosal tissue between the orifice of the parotid duct and commisures of the mouth besides a relatively narrow labial sulcus, the remaining lack of mucosal lining was reconstructed by use of split skin harvested from the right thigh.\nAt a clinical follow-up three months after surgery, palpable enlarged, firm lymph node was found located to the lower jaw. Biopsy confirmed the histological diagnosis of metastasis from squamous cell carcinoma. Position Emission Tomography Scan (PET) confirmed the localisation of metastasis from a squamous cell carcinoma on the left side of the jaw, but as well on the right side. No distant metastases were found. Clinical presentation 5 months after surgery is seen in .\n5 ½ months after the initial admission to our department, bilateral neck dissection was performed. Several metastases with extensive perinodular growth was found on the right side, and infiltrating underlying musculature on the left side. The patient was referred to the Department of Oncology, Herlev Hospital, Denmark, where radiotherapy was given.\nOne months after completed radiotherapy, the patient presented in our clinic a very sore, ulcerated tumour at the jaw line on the left side. The tumour was adherent and dripping with what appeared to be saliva. Punch biopsies were not representative. A computer tomography (CT) scan confirmed local recurrence of aggressive squamous cell cancer. The patient was referred for more extensive surgery and further treatment, which is why eventual defatting of the lip was postponed. |
A 62-year-old right-handed male with a longstanding history of diabetes mellitus and hypertension presented with sudden-onset left-sided body weakness as well as a loss of feeling over the left side of his body. These symptoms started approximately 3 days before the presentation to the hospital. He gave a history of difficulty looking to the left side as well. There was no history of loss of consciousness, speech difficulties, seizures, headaches, or vomiting. His neurologic examination showed an intact level of consciousness, normal higher mental functions as well as normal speech. The examination of vision showed conjugate horizontal left gaze palsy. He could maintain gaze near mid-position but could not cross the midline to the left side. Doll's head maneuver brought the eyes past the midline to the left. Visual acuity was normal at the bedside near-fixation test. Visual fields were assessed by bedside confrontation methods and did not show clear defects. Examination of facial nerves showed weakness of the upper motor neuron type on the left side, and the remainder of cranial nerves was intact.\nMotor system examination showed hypotonia, hemiplegia, hyporeflexia as well as extensor plantar response on the left side of the body. Sensory system examination showed left hemisensory loss of pain and temperature sensations.\nComputed tomography scan of the brain showed an area of infarction over the right subcortical corona radiata region (Fig. ). He received inpatient stroke care and then was discharged home.\nHe was re-assessed in the neurology outpatient clinic (OPD) at 1, 3, and 6-month intervals after discharge. Neurologic examination on the first visit to the neurology clinic showed some improvement in the range of eye movements; however, he had residual partial defects of both saccadic and pursuit functions on horizontal gaze. His motor power over the left side of his body was better as compared to his baseline. Tone and deep tendon reflexes were increased over the left side as well, and the other neurologic findings were the same as his baseline.\nHis neurologic assessment on the second visit to the neurology OPD did not show significant changes from the first visit. However, re-assessment on the third visit (i.e., 6 months after discharge) showed complete recovery of gaze palsy, better motor power on the left side of his body with otherwise similar findings as before. |
A 51-year-old Chinese male presented with a 6-month history of right ptosis and progressive muscle weakness. The patient had been diagnosed as myasthenia gravis at local hospital 6 months before, but for unknown reasons, he failed to receive workup and management at that time. Before one day the patient was admitted to our hospital, he suffered with a sudden onset of dysarthria. Therefore, the patient referred to our hospital for further examination and treatment. Physical examination showed right-sided partial ptosis. Diplopia was noticed on right lateral gaze due to right lateral rectus weakness. The patient did not have dysphagia or dyspnoea. But he was having generalized weakness in all his extremities, both proximal and distal, with marked diurnal variation in the form of more weakness during the evening. A confirmatory electrophysiological study with repetitive nerve stimulation showed decrement in amplitude of action potentials with further reduction post exercise and recovery after 15 minutes. Acetylcholine receptor (AChR) binding antibodies were markedly elevated. However, other routine laboratory test, including blood count, differential, liver and renal function, were within the normal range. A chest computed tomography (CT) scan demonstrated a solid mass, measuring 5.4 × 3.7 × 2.6 cm, with focal heterogeneously enhancement, in the right anterior mediastinum. The tumor was observed to adhere to the wall of aorta but did not infiltrate it. Minimal amount of right pleural effusion and pericardial effusion were associated, but neither enlarged lymph node nor remarkable image finding was noted in the lung parenchyma (Figure ). A CT guided fine needle biopsy of the anterior mediastinal mass was performed and pathological examination showed predominantly epithelial neoplastic cells with pan-cytokeratin (AE1/AE3) positive in a lymphoid component background suggesting a thymoma. Therefore, a preoperative diagnosis was thymoma and the tumor was totally resected. At surgery, the main tumor located in the right lobe of the thymus was found to have adhered to the periaortic tissues, but no invasion of the wall of aorta was observed. Thymectomy and resection of the adherent tissues were performed.\nMacroscopical examination revealed a gray-red solitary nodular mass, and measured 5.0 × 3.5 × 2.0 cm. The mass was well-circumscribed, but there was no fibrous capsule around the mass and no cystic cavity observed in the mass (Figure ). Under the microscopical examination, part of nodular mass was composed of solid and invasive nests of well-differentiated epidermoid cells with desmoplastic stroma. The mucin-producing and intermediate cells were also observed in the tumor. These tumor cells were intermingled or intimately mixed with epidermoid cells. Mucin-producing cells were cuboidal, columnar, or goblet-like with bland nuclear morphology. Bands of fibrous connective tissue were observed among the neoplastic elements. There was no extensive necrosis and neural invasion. Mitotic figures were infrequent and there was no remarkable cellular pleomorphism. Immunohistochemically, the epidermoid cells of the tumor were positive to pan-CK (AE1/AE3), CK5/6, CK7 and p63, but negative to CD5. Mucin-producing cells were negative to CK5/6 and p63. Alcian blue staining revealed mucin in the cytoplasm of the mucin-producing cells (Figure ). This part of tumor was diagnosed as low-grade MEC according to the histopathological criteria of WHO classification []. Interestingly, however, type B2 thymoma could be observed in other area of the same nodular mass. In this area, neoplastic cells were large and polygonal with large nuclei and prominent central nucleoli. The neoplastic cells formed delicate lose network, and small epidermoid foci resembling abortive Hassall’s corpuscles could be observed among the neoplastic epithelial cells, but no mucin-producing cells were found in these epidermoid foci. In these areas, the neoplastic cells were outnumbered by non-neoplastic lymphocytes. By immunohistochemical staining, the neoplastic cells revealed the positivity for pan-CK (AE1/AE3), CK5/6, CK19 and p63, as well as negativity for CD5 and CD117 (Figure ). The field of MEC and type B2 thymoma was distinct in the mass, there was no gradual transition of these two parts observed in mass although they were close to each other in some areas. Based on above findings, a final histological diagnosis of primary combined type B2 thymoma/MEC of thymus was made, and the final staging of this tumor was stage II (Masaoka staging system).\nThe postoperative phase was uneventful and the dysarthria resolved. After diagnosis, the patient was started on pyridostigmine with a remarkable improvement in weakness, diplopia and ptosis. Chemotherapy with regiments of cisplatin and mitomycin, and radiotherapy of the main tumor bed were performed on the patient. Since there was a possibility of tumor metastasis to another anatomical location, the patient was referred to a whole body positron emission tomography (PET)/CT study to search for the potentially secondary tumor, but no abnormality was found. The patient was on regular follow-up for 12 months after discharging from hospital. He had remained asymptomatic, and there was no evidence of tumor recurrence during the period of postoperative follow-up. |
Our patient was a two-month-old male referred for evaluation of a left postauricular mass, present since birth. Workup by the patient's pediatrician including an ultrasound suggested a cystic mass prompting referral for surgical excision. The parents endorsed noticing the lesion at birth and that it had been painless and slowly progressive. Physical exam demonstrated a firm 2 × 2 cm subcutaneous lesion of the postauricular region. An MRI was obtained demonstrating a 2.3 × 1.4 × 2.2 cm well-defined solid mass involving the outer table of the right temporal bone and temporoparietal suture with intense peripheral enhancement and without restricted diffusion (). Initial resection in the operating room was undertaken, and a deep plane between the mass and skull was identified and followed reflecting the lesion off of the skull. Unfortunately, pathology demonstrated focal presence of tumor cells at the peripheral margin. The patient underwent a repeat resection, with a canal wall up mastoidectomy. The lesion was again resected en bloc, and the underlying cortical bone was drilled down to the inner table of the temporal bone with healthy appearing bone stock. Despite clinically normal-appearing bone, the pathology again demonstrated presence of tumor cells at the soft tissue margins, and clinically the patient demonstrated significant regrowth of the lesion. The patient returned to the operating room once more, with a fairly impressive progression of gross tumor, nearly 2.5 × 2.0 cm (). A revision mastoidectomy was performed, and neurosurgical consultation was obtained. The mass was excised en bloc resulting in a full-thickness craniectomy. The dura appeared healthy and unaffected by the tumor (). The wound was closed primarily, and the patient was observed overnight in the PICU before being discharged home postoperative day one in stable condition. The patient developed purulence at his incision site one month postoperatively requiring intra-washout with neurosurgery. The infection resolved without further complication or treatment requirement. He was seen at six months postoperatively with no evidence of disease in good condition. |
A 20 year old Sri Lankan male who was employed as a helper in a grocery, admitted to our unit with weakness of both hands of 1 month’s duration. He was treated for serologically confirmed (Dengue NS1 antigen positive) dengue fever approximately 5 weeks ago at the local hospital and had made an uneventful recovery. He has been given 5 days of inward treatment and the records from the local hospital revealed that he had simple dengue fever with no evidence of fluid leakage.\nFive days after discharge from the hospital he has first noticed the weakness of his right hand when he dropped a glass of water due to poor grip. Weakness was more in the right hand which was his dominant hand and it was slowly progressive over 1 month. At the time of presentation to us he could not write or button on his shirt due the weakness of the hands. Weakness of the left hand was milder than that of the right. The weakness was confined to hands and did not involve forearms or arms. He denied any accompanying numbness, parasthesia or pain.\nOn inquiry he admitted that there was slight weakness of both feet which did not significantly interfere with walking. There was no associated neck/back pain or bladder/bowel incontinence. He did not complain of difficulty in breathing, diplopia, dysphagia, nasal regurgitation, dysarthria or fatigability. He did not give a recent history of trauma to the spine/neck or any preceding diarrheal illness or skin rash.\nHe had no previously diagnosed long term medical ailments and has not undergone any surgical procedures in the past. He was not on any long term medications and he denied smoking, use of alcohol or illicit drugs. He did not give a family history of any progressive neurological conditions.\nOn general examination he had an average built with no pallor, lymphadenopathy or any signs of malnutrition. No skin rashes or hypopigmented patches were noted. There was minimal small muscle wasting of bilateral hands and feet. No muscle fasciculations were noted. Distal upper limb (hand) power was diminished asymmetrically, right hand demonstrating a power of 3 out of 5 and left hand demonstrating a power of 4 out of 5. All fine finger movements including flexion, extension, abduction and adduction were affected with some degree of weakness in wrist extension as well. Bilateral supinator and biceps reflexes were diminished.\nDistal lower limb (feet) power was also diminished but was less pronounced (power grade 4) when compared to the degree of hand weakness. Bilateral foot dorsiflexion was weak. Ankle jerks were elicited with reinforcement whereas the knee jerks were elicited without reinforcement. There was no objective sensory impairment of touch, pain, temperature, vibration and joint position sensations in both upper and lower limbs. Bilateral plantar responses were down going. No palpable nerve thickening identified. No cerebellar signs were demonstrated and his gait showed a minor degree of high stepping due to weak dorsiflexion. Examination of higher functions and cranial nerves including the fundal examination revealed no abnormality.\nExamination of the cardiovascular, respiratory systems and the abdomen was essentially normal.\nFull blood count revealed white blood cell count: 8.5 × 109/L, platelet count: 274 × 109/L, hemoglobin 12 g/dl with normal red cell indices. Blood picture showed normochromic normocytic cells with some reactive lymphocytes suggestive of a recent viral infection. Serum creatinine 80 μmol/l (60 - 110 μmol/l), serum sodium 138 mmol/l (135 - 145 mmol/l), serum potassium 3.8 mmol/l (3.5 - 5 mmol/l), serum magnesium 0.9 mmol/l (0.8–1.1 mmol/l), serum ionized calcium 1.2 mmol/l (1.05–1.30 mmol/l). Liver profile: AST 21u/l (10 - 40u/l), ALT 13u/l (7–56 u/l), ALP 67u/l (100–360 u/l), serum total bilirubin 0.7 mg/dl (0.1–1.2 mg/dl), serum albumin 36 g/l (35 - 50 g/l), serum globulin 32 g/l (20 - 35 g/l). CPK levels were normal. Inflammatory markers: ESR 25 mm/hour and CRP < 6 mg/dl.\nNerve conduction study revealed findings in keeping with multifocal motor neuropathy with conduction blocks involving the distal upper and lower limb peripheral nerves without any conduction abnormalities in the sensory nerves (Fig. ).\nCSF analysis did not show any increase in proteins or cells and the values were within the normal limits. Anti-GM1 IgM antibody test was not carried out due to the high cost of the test and the patient’s unstable financial background. A sural nerve biopsy (a sensory nerve) was carried out and revealed histologically unremarkable nerve fibres and blood vessels with no evidence of inflammation, atrophy or granulomata formation. Recent dengue infection was confirmed with positive dengue IgM and IgG antibodies with enzyme-linked immunosorbent assay (ELISA).\nAs the patient fulfilled criteria, the diagnosis of multifocal motor neuropathy with conduction blocks was confirmed. He was then referred to the neurologist and was started on intravenous immunoglobulin (IVIg) therapy (2 g/kg/day) which was given for 5 days. He showed a mild improvement of his neurological weakness with the treatment and outpatient physiotherapy was arranged. The next immunoglobulin dose was planned to be given after 2 weeks. |
A 30-year old male patient weighing 60 kg, a victim of road traffic accident who sustained a head injury (depressed skull fracture) and loss of consciousness, had associated hoarseness of voice and difficulty in breathing. He was posted for emergent tracheostomy under local anaesthesia with monitored anaesthesia care. On examination, the patient was conscious (GCS of 15), in respiratory distress and there was a bruise extending from the left shoulder to right across the neck in the front. On examination of the neck, crepitus and tenderness were present on the anterior part of the neck. Computed tomography (CT) scan of neck revealed a tear on the anterior tracheal wall extending from C6 to T1 vertebrae level with loss of anterior tracheal wall and surgical emphysema into the surroundings []. Routine investigations and surveys of other body parts were grossly normal.\nIn the operating room, baseline values in the form of a heart rate of 112 bpm, blood pressure of 160/90 mmHg and a respiratory rate of 36–38/min with use of accessory muscles. Significantly the oxygen saturation was only 86–88% on oxygen supplementation by polymask. After administration of the local anaesthetic agent (lignocaine 2%), surgical tracheostomy was commenced with oxygen supplementation (100% oxygen by Bain's circuit). Dissection revealed a tracheal rent on the anterior tracheal wall, measuring 2 × 1 cm was observed at the site where the stoma should have been made []. Initially attempts were made to pass the tracheostomy tube through this tracheal rent. Further, failure of such attempts and an impending cardiorespiratory arrest situation prompted the passage of an endotracheal tube after direct laryngoscopy. Post intubation, the oxygenation of the patient improved, ventilation was partially effective due to leak of air from the tracheal rent. Subsequently, another attempt to negotiate tracheostomy tube through the rent also resulted in the failure in passing the tracheostomy tube into the tracheal lumen. Repeated failures and concomitant brainstorming between the anaesthesia and surgical team led to the exploring of the possibility of introducing the stylet of MINITRAC set (PORTEX) into the tracheal opening carefully []. The tracheostomy tube was then successfully negotiated over the stylet and subsequent intubation was confirmed by capnography tracing and bilateral air entry on auscultation following which the tracheostomy tube was secured. The subsequent stay in hospital was uneventful, and the patient was later discharged and referred to a higher institute for surgical repair of the laryngotracheal injury. |
An 11-year-old, male, neutered pug, weighing 8.7 kg, was presented for profound pain when opening the mouth. The dog has had, over a year, history of chronic bilateral middle ear disease treated with two ventral bulla osteotomy surgeries. Despite surgery and medical management, the dog continued to experience profound pain. The dog also had concurrent atypical Addison's disease, moderate to severe periodontal disease, and brachycephalic airway syndrome. The dog was receiving a daily dose of prednisone for the management of Addison's disease.\nOral and maxillofacial examination revealed that the dog experienced substantial pain on its attempt to open the mouth which had developed and worsened over the last year and a half. The dog had previously been seen for periodontitis and had had multiple extractions. In the interim between the previous extractions and the current presentation, the dog had had several ear infections that had been difficult to control and had been treated by a specialty group combining veterinary dermatology, surgery, and neurology, given the myriad needs of the dog. After a first bulla osteotomy, the dog developed further ear infection on the right side, and a second bulla osteotomy found a cholesteatoma on the right side. After the second surgery, the dog developed worsening signs of pain, difficulty in opening the mouth, and eating. On oral examination, apart from the previously extracted teeth and moderate dental calculus, no obvious oral abnormalities were detected. The dog was reluctant to allow an open-mouth examination or palpation of the ears and TMJ. Complete blood count and urinalysis were within the normal limits and the serum biochemistry analysis revealed very mild hyperproteinemia and hyperglobulinemia.\nCone-beam CT images of the skull were obtained. Severe erosive arthritis of the right TMJ was observed, which was characterized by a pronounced periosteal reaction of the condylar process, osteophytes present on the medial aspect of the joint, and subchondral bone cyst at the lateral aspect of the condylar process. There was also increased density of the bone of the condylar process as compared to the left side, suggesting a septic process. Importantly, the bulla on the right side was deformed, with bony expansion, and it engulfed the medial aspect of the condylar process (). A surgical approach to the TMJ area was performed as described earlier. Fluid and samples were obtained from the joint capsule and the surrounding tissues and were submitted for culture and sensitivity testing. An enlarged Lymph node in the proximity of the joint was removed and submitted for histopathological analysis.\nThe dog was discharged with the following medications: amoxicillin/clavulanic acid (20 mg/kg, q 12 h for 14 days), enrofloxacin (10 mg/kg, q 24 h for 12 days), tramadol (3 mg/kg q 8–12 h for 8 days), and carprofen (2.2 mg/kg q 12 h for 14 days). The owner was informed that medical therapy may need to be modified based on the culture and sensitivity results.\nHistopathological evaluation of the lymph node revealed a reactive process with no evidence of neoplasia. Culture and sensitivity analysis yielded a low number of non-enteric gram-negative rods, Pseudomonas putida, and Chryseobacterium indologenes. The organisms isolated were uniformly resistant to common antibiotics, such as cephalosporins, amoxicillin/clavulanic acid, doxycycline, chloramphenicol, and trimethoprim/sulfa. Based on the culture and sensitivity results, marbofloxacin (50 mg q 24 h) was prescribed for 2 months. The day following the procedure, the client updated that the dog exhibited an immediate remarkable improvement in his level of comfort. At the 2-week recheck, the dog had more energy, allowed a full oral examination, and had a good range of motion of the TMJ. Unfortunately, about 2 weeks after this recheck appointment, the dog exhibited a sudden severe decrease in the energy level and appetite that did not respond to hospitalized supportive care for 24 h and was humanely euthanized. |
A 46-year-old woman with morbid obesity, hypertension, hyperlipidemia, active smoking, and bipolar disorder was evaluated for paroxysmal atrial fibrillation as an outpatient. A transthoracic echocardiogram showed a right atrial mass close to the RA-IVC junction. A transesophageal echocardiogram confirmed the presence of a pedunculated right atrial mobile bilobular mass measuring 2.1 × 1.8 cm, not arising from the interatrial septum with extension into the inferior vena cava (Figures –). A secundum atrial septal defect was also identified. The patient was referred to cardiac surgery for resection of a presumed right atrial myxoma given the possibility of embolization. The intraoperative TEE suggested the presence of one bilobulated mass arising low in the right atrium.\nUsing aortic and bicaval cannulation, a right atriotomy was performed and a discrete 2 × 3 cm mass was removed from the IVC-RA junction near the Eustachian valve. The right atrium was closed and the patient weaned from bypass. The specimen did not appear to be a myxoma, so it was sent for frozen section and interpreted as being ectopic liver tissue. In the interim, a repeat TEE showed an additional discrete 2 × 2 cm mass in the IVC near the hepatic veins. Femoral venous cannulation was then performed to allow for visualization of the IVC below the pericardial reflection. Through the same right atriotomy and using additional suction directly in the IVC, the IVC mass was visualized and resected. Both specimens were interpreted as showing partially encapsulated liver parenchyma with mild steatosis, fibrosis, ductal proliferation, and periductal chronic inflammation, consistent with ectopic hepatic tissue ().\nDespite a brief period of cardiopulmonary bypass, the patient had persistent hypoxemic respiratory failure ascribed to her morbid obesity and underlying lung disease. She also developed extensive left lower extremity deep venous thrombosis from a presumed heparin-induced thrombocytopenia for which she was treated with argatroban and placement of an IVC filter. Because of chronic respiratory failure, she underwent tracheostomy and feeding tube placement, and she was placed on warfarin for intermittent atrial fibrillation due to her CHA2DS2-VASc score of 4. She gradually improved and was transferred to rehab where her tracheostomy tube and PEG tube were removed. A few months later, she looked quite well during an office visit. |
A 72-year-old male presented to emergency department with tender mass on lower back and posterior aspect of neck. Apparently, he had noticed it on his lower back 6 months ago and had grown in size with another similar swelling appearing on his neck, also they were increasingly painful and sore to touch. He had a history of ongoing sore throat for 3 years for which he was extensively investigated by ENT services. His CT neck and mediastinum did not show any abnormalities at that time. This was followed by laryngoscopy which showed a white patch on the right vocal cord, biopsy of which showed non-specific chronic inflammatory changes and cultures grew candida. Background history included COPD; he was heavy current smoker and smoked about 60 cigarettes per day. His medications included salmeterol/fluticasone propionate and ipratropium bromide inhalers. Vitals were within normal range. On examination, 4 cm nodular, firm, tender, immobile mass on lower back and 3 cm mass on posterior aspect of lower cervical spine were noted.\nHis laboratory investigation including renal and liver function, full blood count and coagulation profile were normal. A CT of thorax, abdomen and pelvis was arranged which showed 2 × 2 cm mass in right lung lower lobe likely malignant with no hilar or mediastinal lymphadenopathy, also 3 × 3 cm soft tissue mass at C6-C7 level posteriorly and 4 × 2 cm mass was noted on lumbar region with multiple prominent inguinal lymph nodes ().\nA referral for bronchoscopy was sent and biopsy of the nodule was planned. While awaiting bronchoscopy appointment, biopsy was taken from mass in lower back and send for histopathology. Histology showed poorly differentiated tumour cells in cohesive groups and sheets with moderate pleomorphism and eosinophilic cytoplasm; immunohistochemistry was positive for AE1/3, cytokeratin 7 and carcinoembryonic antigen antibody stain and periodic-acid schiff. Weak nuclear positivity for TTF-1 was also seen. Overall features were consistent with poorly differentiated adenocarcinoma likely of lung origin.\nHe was then referred to oncology services and unfortunately passed away within 5 months of diagnosis. |
We present the case of a 45-year-old Caucasian man suffering from psoriasis for more than 20 years at the time of the first visit. No relevant comorbidities were found at history collection, apart from benign prostatic hyperplasia, for which he is treated with tamsulosine. He was initially diagnosed and followed-up by dermatologists, who treated him with topical corticosteroids and salicylic acid for skin involvement and systemic corticosteroids for resistant lesions as needed ().\nThe skin component had always been <30% of the total body surface area (BSA). The patient reported distress in personal and social relationships with consequently reduced HRQoL. After ten years, he started experiencing dactylitis of feet and recurrent arthritis of the small joints of hands, shoulders and knees, together with inflammatory morning stiffness lasting around one hour, with negative rheumatoid factor (RF); thus fulfilling ClASsification criteria for Psoriatic ARthritis CASPAR criteria for PsA []. He was referred to the rheumatology outpatient clinic of another hospital and treated with indomethacine and systemic corticosteroids as needed for about five years, and then achieved complete remission of joint symptoms. Three years later, because of a flare of joint involvement (episodes of peripheral arthritis and dactylitis of the small joints of hands and feet once per week) and skin involvement, he was started on methotrexate 10 mg weekly with folate supplementation, in combination with cyclosporine 200 mg daily, with reduced extension and severity of skin lesions and decreased frequency of arthritis flares to once per month. However, he did not reach complete remission. Shortly after initiation, due to a 2-fold increase of liver enzymes (hepatitis excluded based on abdomen ultrasound and hepatitis B and C viral profile), methotrexate was reduced to 7.5 mg weekly []. The reduced methotrexate regimen led to normalization of the liver enzymes but was ineffective in controlling both skin and joint involvement. Two years later, due to elevated arterial pressure values (up to 150/90 mmHg) and serum creatinine increase >30% of baseline value (up to 1.4 g/L), cyclosporine was stopped.\nThe following year, he referred to our outpatient clinic for skin and joint flare. At referral, physical examination demonstrated mild psoriasis of trunk, arms and legs, with a psoriasis area severity index (PASI) score of 5.1 (), dactylitis of the third digit of the left foot and arthritis of the third interphalangeal joint of the left hand, which, together with a normal C reactive protein (CRP), activity visual analogue scale (VAS) and pain VAS of 4 and 4.5 respectively, accounted for a disease activity in psoriatic arthritis (DAPSA) score of 12 (low disease activity) [].\nLaboratory tests showed no relevant alterations. Radiographic assessment of hands and feet showed no erosions and no signs of axial involvement were found at magnetic resonance imaging (MRI). Ultrasound imaging of the third metacarpophalangeal (MCP) joint showed inflammation of the joint and of the peritendon of the extensor tendon (a).\nConsidering that the patient failed to respond to two DMARDs (cyclosporine and methotrexate), that he refused a parenteral drug, that he presented mild skin and joint involvement, no bone erosions, dactylitis and no axial involvement, apremilast was chosen [,,]. At baseline, the EQ-5D questionnaire reported altered HRQoL, with some difficulties in motility, no difficulties in self-care and some difficulties in usual activities, moderate pain/discomfort and moderately anxious/depressed mood. Patient’s global health (GH) VAS was 60 and pain VAS was 45 (). Furthermore, the patient was screened at baseline for CV risk; carotid arterial doppler ultrasound demonstrated only intimal thickening of the left bulb and 24 h blood pressure monitoring demonstrated mild systolic and diastolic daily hypertension for which he started lercanidipine 10 mg/daily. At baseline, total cholesterol (TC) and triglyceride (TG) serum levels were 173 mg/dL and 113 mg/dL respectively, and TC/high density lipoprotein (HDL) ratio was 4.94 (). After 4 weeks, the extension and severity of erythema and infiltration of psoriatic plaques were substantially reduced (PASI 2.5, ). Furthermore, the patient reported a marked improvement of joint involvement with no further episodes of arthritis or dactylitis, although inflammatory morning stiffness persisted (DAPSA 6.0). At the 2-month follow-up, the patient reported several side effects such as mild headache, dizziness and hypotension and, due to further amelioration of the skin and joint involvement he stopped apremilast, which was restarted shortly after, due to worsening of psoriasis (PASI 3.0) and occurrence of a new joint flare (DAPSA 10). At the time he was still taking methotrexate 7.5 mg weekly. At the 6-month follow-up he reported no arthritis flares and no inflammatory morning stiffness. At physical examination, the third MCP joint was swollen but not tender (DAPSA 3), and ultrasound imaging showed reduction of joint and soft tissue inflammation (b). A further improvement of skin involvement was observed and reported by the patient (PASI 1.2, ). During the follow-up, we observed an amelioration of the serum lipid profile already after 4 weeks, with a 5.8% reduction of TC and a reduction of TC/HDL ratio, and after 12 months, a further reduction of TC, LDL and TG values of 15.6%, 25.7% and 17.7% respectively, and a 20% increase of HDL levels compared to baseline values. TC/HDL ratio reached the favorable value of 3.48, as shown in . We even observed a 5% weight loss—although our patient already had a normal baseline weight and body mass index (BMI)—reaching a final normal weight with a normal body mass index (BMI 21.5). After 12 months, the clinical manifestations were stable. We therefore decided to stop methotrexate, but the patient experienced a slight relapse of both skin and arthritis (PASI 2.5, DAPSA 6), shortly after. Therefore, although dosage was low (7.5 mg), we reintroduced methotrexate, leading at the 18-month follow-up to stable minimal disease activity with PASI 75 and joint remission (PASI 1.2, DAPSA 2) [,] and a dramatic improvement in HRQoL (). |
A healthy 48 year-old Caucasian American man with no significant past medical history presented to the emergency department with severe left anterior thigh pain. Initially, he noticed a small tender mass in the left buttocks approximately 4 weeks ago. He also described that during his exercise in the gym 4 days prior to arrival he felt like mass was ruptured and reduced in size giving him slight relief in pain. Yet over the period of 4 days, he has worsening of pain and gradually he has decreased in the range of motion at the left hip joint. The patient’s home medication includes self-administered intramuscular testosterone injection usually to his either of buttocks for at least the past 3 years. Complete review of the system was negative for pain at another site, loss of sensation in left extremity, knee pain or ankle pain, skin rash or color change, fever, swelling of left hip or knee joint. Vitals and labs were within normal limits. On physical examination, he was in moderate distress, left thigh was tender to touch, and the patient experienced pain out of proportion on passive extension of the left thigh, with tense anterior thigh muscles, raising the suspicion of compartment syndrome. On admission, a Computed Tomography scan of the left hip showed a left gluteal fluid collection, and a 2 × 2.7 cm cystic mass (). Left knee synovial fluid was collected and results ruled out infectious processes.\nOrthopedic surgery had high suspicion for ACS; therefore, the patient underwent urgent fasciotomy of the left anterior thigh compartment with excision debridement of the left lower extremity including skin, subcutaneous tissues, fascia, and muscle with the placement of wound vacuum-assisted therapy. After 6 weeks, when the patient was seen in the clinic, he had no symptoms and a full range of motion was achieved. Interim he also went through rehabilitation and physical therapy. The patient was educated to refrain from further self-intramuscular testosterone injection. |
A 56-year-old female with a history of uncontrolled biopsy-proven bullous pemphigoid began treatment with cyclosporine 100 mg three times a day for a total daily dose of 300 mg. After five weeks of therapy, the patient complained of nervousness and shakiness, and the cyclosporine dose was decreased to 100 mg twice daily for a total daily dose of 200 mg. After an additional five weeks of cyclosporine therapy at 200 mg daily, the patient returned with complaints of the gradual onset of redness, swelling, and pain of her left hand over the past six weeks (Figure ). On examination, erythema and violaceous swelling were present on the patient’s left hand, most predominant over the dorsal and palmar aspects of the left second and third metacarpals, and left proximal and mid phalanges. There was tenderness to palpation and pain with movement of the left hand and phalanges. The patient described the associated pain as a burning sensation. Although erythromelalgia was considered, this was believed to be a local reaction, and the cyclosporine was continued as treatment for the patient’s bullous pemphigoid.\nSeven weeks later, the patient returned complaining of significantly worsened redness, swelling, and pain of the left hand with progression of involvement of the right hand. On examination, the erythema and edema involved the dorsal and palmar aspects of the left hand and phalanges, most severely affecting the left proximal phalanges. The right hand was also visibly erythematous with edema most prominently involving the right second proximal phalanx. The patient complained of worsened pain, described as “burning similar to a sunburn” that significantly worsened upon exposure to the sun or heat. The pain also worsened with passive and active movement of the hand or digits, as well as palpation of the affected areas. The patient endorsed improvement with exposure of the hands to cold temperatures, particularly describing relief of the pain while running the hands under cold water. At this time, the cyclosporine dose was decreased to 100 mg once daily with a diagnosis of erythromelalgia secondary to cyclosporine. It was hoped that a lower dose of cyclosporine could be used to treat the bullous pemphigoid, with resolution of the symptoms of erythromelalgia.\nSix weeks later, the patient returned for a follow-up to evaluate the erythromelalgia. The erythema and edema had significantly improved, with only minor swelling of the second and third phalanges of the left hand and the second phalanx of the right hand. The patient's symptoms improved following the transition to low-dose cyclosporine therapy (100 mg once daily). At an additional follow-up eight weeks later, the patient’s symptoms of erythromelalgia had completely resolved. |
A 24-year-old male with HA was admitted to our department with pain in multiple joints on May 23, 2011. The patient had a medical history of hemophilia A since the age of 3 and was intermittently treated with factor VIII. During these years, he sequentially developed left knee, left elbow, left hip, and right knee joint pain and swelling with limited activity and was soon diagnosed as HA. Initially, the joint manifestations could be largely relieved by factor VIII replacement therapy. Factor VIII inhibitor screening remained negative. Later, factor replacement therapy failed to achieve satisfactory effects, so in 2002 and 2006, he received left elbow synovectomy and left total hip arthroplasty, separately. In the subsequent years, the patient still suffered from the recurrent episodes of left elbow and bilateral knee joints hemorrhage, pain, and swelling. In recent 2 years, the frequency of joint hemorrhage had increased to approximately 2 times a week and only slightly relieved after factor VIII replacement therapy. Currently, the activity of those joints was limited to various degrees. Other medical history involved 2 cerebral hemorrhages 18 and 15 years ago, separately.\nOn physical examination, significant tenderness was noted in the left elbow joint with limited pronation and decreased grip strength. The preoperative Mayo elbow performance score (MEPS)[ was 55 for the left elbow. Moreover, knee valgus (left 20° and right 15°) was noted, and hyperextension, hyperflexion, and positive grinding test results were noted in both knee joints with a swollen and warm right knee. The preoperative Hospital for Special Surgery (HSS) knee scores[ were 58 for the left knee and 65 for the right knee.\nBilateral knee joints and left elbow joint exhibit advanced arthropathy on radiographs (Figs. A and 2A). These joints present narrowing of joint space, erosions of the articular facets, and bone deformation to various degrees.\nOur diagnosis was hemophilia A and HA of the left elbow joint, both knee joints, and left hip joint. The patient received left elbow synovectomy and left total hip arthroplasty, but the condition continued to deteriorate over time with worsening of the left elbow and both knee joints. Taking all of these factors into account, surgical methods were our top priority, and simultaneous total multi-joint replacement was indicated. Due to the complicated joint lesions and medical conditions, our preparations for this arthroplasty were far more sufficient than usual. Given that arthroplasty for patients with hemophilia A, particularly the simultaneous replacement of multiple joints, is challenging, the patient and his family were informed in detail of the possible benefits and risks of the surgery. We performed a full musculoskeletal assessment and thorough medical evaluation beforehand. Blood products were prepared for possible bleeding events. Then, our team performed bilateral total knee arthroplasty (Zimmer NexGen) and left total elbow arthroplasty (Zimmer) under tightly regulated factor VIII replacement therapy. Antibiotic prophylaxis was administered 30 minutes prior to surgery, and an additional dose was administered once during the operation. Local hemorrhage was carefully controlled to prevent secondary joint damage. Approximately 1800 mL blood was lost during the entire surgery. The patient received 900 mL blood by autotransfusion and 4 units of red blood cells plus 800 mL fresh frozen plasma by intraoperative infusion. During surgery, we observed hemarthrosis and villous synovial hypertrophy at the joints, and severe erosion of the articular surface and various degrees of bone deformation were noted. These findings confirmed the preoperative diagnosis and preoperative assessments. After surgery, hemostasis management, such as compressive bandage, factor VIII infusion, and rigorous monitoring of coagulation indicators, was performed. An early rehabilitation program was applied to achieve improved regain of function.\nWe managed factor VIII replacement therapy during perioperative period under the guidance of hematologists. On the day of surgery, 3000 U/12 h (the body weight of this patient is 63 kg) factor VIII (ADVATE) was administered intravenously followed by 2000 U/12 h on postoperative days 1 to 3 (POD 1–3). Then, on POD 4 to 6, a dose of 1500 U/12 h was administered followed by 1000 U/12 h over the following 6 weeks. Factor VIII inhibitor remained negative in perioperative tests.\nAt the follow-up, the patient's joints functioned well. The MEPS of the left elbow was 85, and the HSS score of knee joints were 71 (left) and 81 (right). On radiographs (3 months and 5 years after operation), the arthropathy of bilateral knee joints and left elbow joint was significantly relieved (Figs. B,C and 2B,C). |
A 65-year-old woman presented with worsening left lower extremity pain and swelling. She had a long history of superficial varicosities and painful venous hypertension, previously treated with sclerotherapy and bilateral great saphenous vein ablations. These therapies provided her with partial relief, but she was still experiencing daily symptoms. Six years prior, she also had L2–S1 spinal fusion for degenerative joint disease. In the emergency department, she reported that she had woken up with severe pain and heaviness in her left leg from the knee down with her right leg being at baseline. Initial workup was negative for deep venous thrombosis, and after obtaining a magnetic resonance imaging (MRI) of the lumbar spine, her neurosurgeon did not think that the pain was related to her back. She then was referred to our venous clinic for further evaluation.\nVascular ultrasound of her left leg showed venous insufficiency in the small saphenous and Giacomini veins. Those findings were not severe enough to cause the level of discomfort that she was experiencing, so magnetic resonance angiography (MRA) of the pelvis was ordered. This study showed narrowing of the left common iliac vein to 0.2 cm (Fig. ), with a tortuous right common iliac artery crossing over the constriction. The findings were consistent with non-thrombotic May–Thurner syndrome, which was thought to be the cause of her new symptoms.\nThe patient was scheduled for left iliac vein stent placement. During the procedure, impingement was noted upon the posterior aspect of the vein. At this location, there was perforation of a pedicle screw through the anterior cortex of her L5 vertebral body, from prior spinal fusion. This interaction was well visualized during intraluminal inflation of a 12 mm × 40 mm high-pressure balloon (Fig. ) and was also seen after final stent placement (Fig. ). Because the screw tip was completely unyielding, a 14 mm × 60 mm Wallstent was used instead of the 16 mm stent we typically use, so as to avoid undue compression of her overlapping right common iliac artery. After the procedure, the patient's symptoms were greatly improved. Follow-up venography performed 4 months postoperatively showed a patent stent with slight eccentric compression at the protruding screw and no evidence of in-stent stenosis. |
Our patient was a 68 year old African American female who developed diffuse abdominal pain, associated with nausea and bilious vomiting, 24 hours prior to her emergency room visit. The pain was localized to the epigastric region and had a progressive course. Her last bowel movement was reported as four days prior and she denied any recent weight loss, wheezing, flushing, palpitation or change in bowel habits.\nThe patient's past medical and surgical history included hypertension, hyperlipidemia, total abdominal hysterectomy and bilateral salpingoophorectomy secondary to fibroids.\nThe patient also reported a history of progressive loss of vision ten years earlier that was investigated by brain MRI after an extensive ophthalmological evaluation. The MRI showed a pituitary tumor and the patient benefited from a transphenoidal pituitary tumor resection. The pathological examination revealed a chromophobic pituitary adenoma.\nThe patient had a strong family history of cancer. Her father died from colon cancer, a brother died from esophageal cancer, an uncle died from brain tumor, one aunt had been diagnosed with breast cancer and one aunt with gastric cancer.\nUpon physical exam, the patient had a tense, distended abdomen, with a well healed paramedian incision and no bowel sounds. There was non localized diffuse tenderness with positive rebound and voluntary guarding. Rectal examination revealed no masses and an empty vault. Laboratory values revealed no leukocytosis, however the lactic acid level was elevated. A computed tomography scan of the abdomen revealed a small bowel obstruction. Subsequently, the patient was decompressed with a nasogastric tube and fluid resuscitation, and brought to the operating room for an exploratory laparotomy. Intraoperatively, there was significant small bowel congestion with no necrosis. One adhesive band was found and lysed at the mid jejunum where it was fixed to the pelvic wall. The bowel was thoroughly inspected to look for any other points of obstruction or abnormalities. A serosal lesion was found on the surface of the jejunum ten centimeters from the adhesion (Figure ). Also a suspicious hard draining mesenteric lymph node was seen. The serosal lesion, and the suspicious lymph node were both resected (Figure ), and sent for pathologic determination.\nPostoperatively, the patient did well, however, the pathological evaluation of both the serosal lesion and the mesenteric lymph node revealed carcinoid tumor.\nThree days later, the patient was brought back to the operating room for exploratory laparotomy and small bowel resection. During the surgery, there was no intestinal lesion noted, and about 15 cm of small bowel on each side from the previous serosal lesion was resected with its corresponded mesentery (Figure ). One enlarged and firm mesenteric lymph node, included in the specimen was marked with a stitch.\nThe pathological examination of the specimen revealed a carcinoid tumor approximately 0.4 cm in greatest dimension, penetrating subserosa five centimeters from the previously resected serosal lesion (Figure ). A metastatic carcinoid tumor was seen in three out of 17 lymph nodes (Figure ) including the one marked with the stitch. The surgical resection margins were negative.\nDuring the postoperative inpatient period, a 24 hour urine 5-Hydroxyindole Acetic Acid (5-HIAA) was within normal value and no focal area of increase uptake was noted on an octreotide scan.\nThere was no postoperative morbidity, and the patient was followed as an outpatient at two and six month interval. During these follow up visits, the patient reported feeling better and denied any weight loss, wheezing, flushing, palpitations or diarrhea.\nA postoperative CT scan of the chest abdomen and pelvis six months after the surgery, revealed no evidence of recurrent disease, and no intra abdominal masses. A repeat octreotide scan at six months after the surgery did not show any area of increase uptake. Chromogranin A level was followed, and was decreasing from 142 ng/ml at two months post resection, to 64 ng/ml at six months post resection. |
A 27-year-old woman was found to have a kidney tumor when she underwent a routine physical examination. The patient exhibited no history of hematuria, back pain, or abdominal mass. She also had no paraneoplastic syndromes, such as fever, hypertension, or a higher erythrocyte sedimentation rate. She had no family history of renal carcinoma and was a nonsmoker.\nA high echolocation, high resolution, color Doppler ultrasound revealed a 5.3×6.0 cm tumor in the upper pole of the left kidney. The tumor was well demarcated from the adjacent renal parenchyma and received blood supply.\nOn computed tomography (CT), the attenuation value of the tumor was approximately +38 HU and +56 HU on routine and enhanced CT, respectively. The contrast-enhanced CT exhibited heterogeneous density and showed that the renal capsule was invaded by the tumor (). Calcification was also observed in the tumor. There was no evidence of a tumor in the renal vein, the inferior vena cava, or in the respiratory or digestive systems. The patient was treated with laparoscopic left radical nephrectomy under general anesthesia in our surgical center.\nMacroscopically, a solid tumor of 5 cm in diameter was found to originate from the renal parenchyma in the upper pole of the left kidney. The cross surface of the tumor was light yellow and firm, and the central part was soft with hemorrhage and necrosis. The tumor had invaded the capsule of the kidney, but the renal vein and left adrenal gland were found to be free of tumor invasion.\nMicroscopically, the tumor was composed mainly of trabeculae and anastomosing cords of tumor cells within a highly vascularized stroma. The tumor cells possessed eosinophilic cytoplasm with inconspicuous nucleoli, and mitosis was rare (). Immunohistochemical staining showed strong and diffuse staining for synaptophysin, chromogranin A, and neuron-specific enolase ().\nThe institutional review board approval was not sought as per institutional guidelines around a clinical case report. The patient did provide written informed consent about this case report. |
A 67-year-old male patient was admitted to another surgery institution with a ruptured infrarenal abdominal aortic aneurysm (6.2 cm) 7 years ago. Left iliac artery occlusion and femorofemoral bypass were performed with right aortoiliac graft insertion. Afterwards, a type II endoleak was detected due to the inferior mesenteric artery and was treated with coil embolization at the first follow-up year, after which the patient was lost to follow-up.\nThe patient was admitted to our emergency department due to abdominal pain. Computed tomography angiography demonstrated a type Ia endoleak from the posterior side of the graft with a huge aneurysm sac (22.9 cm) without rupture (, ). His right kidney was atrophic, and the hemoglobin level was detected to be 8.1 g/dL. Endovascular repair techniques were considered first, but the proximal neck length was <6 mm, which was very near to the left renal artery; the vascular team lacked the experience of endovascular repair techniques for type Ia endoleaks, so the conventional open surgery was immediately planned. The patient was hemodynamically unstable, and inotropic drug infusion was initiated before the surgery. The proximal side of the endovascular graft was at the juxtarenal part of the aorta, and clamping by abdominal incision could be complicated; thus, left anterolateral thoracotomy was performed for safe clamping. The endovascular graft and the surrounding aortic tissue were resected with a wide laparotomy (, ). An aortobifemoral bypass was performed using a Dacron graft. The proximal anastomosis was done with end-to-end configuration just below the superior mesenteric artery, and left renal artery bypass was established with the saphenous vein. The right renal artery was ligated as the right kidney was known to be atrophic. The patient was transferred to the intensive care unit after the surgery; he died postoperative 6th hour due to persistent deep asidosis. Visceral or renal protection could not be provided during the surgery; total operation time was nearly 6 h, and four erythrocyte suspensions were replaced during the surgery and follow up. This study was approved ethically by Institutional Board. |
A 62-year-old man underwent a regular health check-up examination, which detected thoracic aortic aneurysm (TAA). So, he was admitted in the Department of Cardiothoracic Surgery. According to the patient's medical history, he had undergone a car accident 10 years ago and a motor cycle accident 18 years ago. The patient was asymptomatic initially, and a physical examination revealed no significant findings. The patient was on medications for hypertension, which was well under control. The American Society of Anesthesiologists physical status classification system denoted score of one for this patient. The Glasgow Aneurysm Score of this patient was 62 (62 [age in years] + 0 [7 for shock] + 0 [7 for myocardial disease] + 0 [10 for cerebrovascular disease] + 0 [14 for renal disease] = 62). The patient had not undergone any prior vascular surgery. He was hemodynamically stable with a blood pressure of 120/80 mm Hg.\nComputed tomography angiography revealed a saccular TAA having a maximum diameter of 63 mm at the greater curvature of the proximal descending thoracic aorta, which was located near the orifice of the left subclavian artery. Therefore, cardiac surgeons decided to perform hybrid TEVAR in zone 2 of this patient. The diameter and length of the proximal neck were 30 mm and 18 mm, respectively. The angulation between the top of the aortic arch and proximal descending thoracic aorta was 81 degrees. The distal neck was 26 mm in diameter without angulation. The radius of the aortic arch was 12 mm. Moreover, the neck of the saccular TAA was wide, having a width of about 5 cm (). In the CT, we could not detect any development of atheroma, plaque, or thrombus in this patient. So, there were no chances of developing embolism within the aneurysmal sac.\nAfter administering general anesthesia, we first performed bypass surgery on the left common carotid artery and left subclavian artery using an 8-mm vascular prosthesis. This surgery was conducted to maintain arterial flow to the left vertebral and subclavian artery. The proximal left subclavian artery was ligated to prevent type II endoleak. After conducting a successful bypass surgery on the patient, the right common femoral artery was accessed with two sets of Proglide (Proglide; Abbott, Redwood City, CA, USA) using the Preclose-technique. This procedure was performed to enable the entry of the stent-graft's introducer system. To perform control aortography, the left common femoral artery, which was located in the ascending thoracic aorta, was punctured with a 5-Fr marker catheter (Super Torgue; Cordis, Miami Lakes, FL, USA). An extra stiff Lunderquist Guidewire (Cook Medical, Bjaeverskov, Denmark) was inserted through the right common femoral artery sheath into the ascending aorta, over which a 22-Fr introducer system of Zenith Thoracic Pro-Form Endograft (36-157-32 mm; Cook Medical, Bjaeverskov, Denmark) was to be advanced at the target site. However, despite the use of an extra-stiff guidewire, the advancement of the introducer system failed repeatedly, because of a 5-cm wide-necked aneurysm at the greater curvature. A prolapse of the introducer system occurred repeatedly into the wide-necked aneurysm, because the guidewire was not supported by the aortic wall ().\nFirst, we gently tried transbrachial access through the right subclavian artery. The right subclavian artery was punctured and the snare was inserted; however, this method failed because it was not able to overcome the curvature describing the right subclavian artery, right brachiocephalic artery, and aortic arch.\nTo facilitate the passage of this introducer system to the target site, a compliant molding balloon (Reliant; Medtronic, Galway, Ireland), having a diameter of, was introduced through a 12-Fr sheath from the contralateral femoral artery. Thus, this balloon was placed within the aneurysm sac. When this compliant balloon was inflated, it created an artificial wall at the greater curvature that was strong enough to support the advancement of the introducer system into the target site. When the operator advanced the introducer system, an assistant pulled the occlusion balloon in inferior direction by carefully providing support to the introducer system. Thus, the assistant prevented an upward prolapse (). Finally, we were able to successfully advance the introducer system into the target zone () and deploy the stent-graft (). The patient was stable in the peri-operative period, so he was discharged on the sixth day after the operation. After the procedure, the patient did not exhibit any signs of neurological deficits or renal dysfunction. In the 2-year follow-up period, we periodically conducted CT-angiography of the patient. The CT-angiography revealed complete exclusion and shrinkage of the saccular aneurysm along with a patent left subclavian artery bypass graft (). |
A 54-year-old Japanese woman visited our ophthalmology department after experiencing proptosis, lid swelling, diplopia, and retro-orbital pain in her left eye lasting for 1 day. She had a medical history of poorly differentiated adenocarcinoma of the stomach, which had metastasized to her ovary and mesentery, diagnosed 2 years earlier. She had undergone four regimen courses of chemotherapy, yet these had failed and she thus received palliative treatment. There were metastases to subcutaneous tissue of her neck and thoracic bone marrow 3 months before her initial visit to our ophthalmic department. She had been admitted to our hospital 5 days previously without symptoms in either eye. She had undergone stenting in her esophagus against eating difficulties but she lived a self-reliant life at home.\nAt her first visit, an external examination showed lid swelling, red coloration, and proptosis of her left eye. A motility examination revealed an adduction deficit of − 4.0 and an abduction deficit of − 1.0. Ophthalmological examinations revealed a best-corrected visual acuity of 20/20 and an intraocular pressure of 15 mmHg in both eyes. No abnormal findings were found in the anterior segment. Her pupils were equally reactive without any relative afferent pupillary defect. A funduscopic examination showed partial optic disc edema in her left eye (Fig. a). No choroidal masses or striae were noted.\nA CT scan performed 10 days before her initial visit to our ophthalmology department revealed enlargement of the left medial rectus muscle. Retrospectively, similar findings were seen on a CT scan performed 3 months previously, and had worsened in the interim. Yet, a CT scan that had been performed 6 months previously showed no remarkable findings (Fig. ). There was no enlargement of other extraocular muscles and no swelling or mass lesion in other orbital tissues during the 6 months. So, gastric cancer metastasis to the medial rectus muscle of her left eye was suspected. Radiation therapy for metastasis to the subcutaneous tissue of her neck and thoracic bone marrow was effective; she received a total of 20 Gy/5 courses of radiation therapy to the orbit. A few days after completion of radiation therapy, lid swelling, red coloring, and pain disappeared. Two weeks post-radiation therapy, a motility examination revealed an adduction deficit of − 4.0 and Hertel’s exophthalmometry measurements with a 108-mm base were 14 mm (right eye) and 19 mm (left eye). At 1.5 months post-radiation therapy, a motility examination revealed an adduction deficit of − 2.0 and Hertel’s exophthalmometry measurements (108-mm base) were 14 mm (right eye) and 13 mm (left eye). A posterior ocular segment examination showed a normal left optic disc (Fig. b). She died 3 months after her initial presentation to our ophthalmology department. |
A 28-year-old female was referred to the Department of Restorative Dentistry and Endodontics of Gazi University Dental Faculty with a complaint of spontaneous pain. Clinical examination showed a fully dentate patient with deficient oral hygiene. Tooth 15 had a carious lesion which was verified by the radiographic examination. The clinical crown of 15 was tipped in the palatine direction and positioned 2 mm below the occlusal plane. Radiographic examination also revealed a sharp curvature located in the middle third of the root canal (). The apical third of the root 15 seems to have direct contact with the mesial root of 16 which was shortened severely (). The remaining teeth showed no unusual tooth morphology. The mobility of all teeth was within the physiological range. A periapical radiograph taken from the symmetrical region in the maxilla exhibited no abnormality in the root shape and morphology of the teeth 25 and 26 (). The medical history of the patient revealed no systemic disorder, an endocrine or a metabolic disease. She had no history of previous orthodontic treatment.\nOn the basis of the clinical and radiographical evaluation, the definitive diagnose of tooth 16 was external apical root resorption related to excessive pressure formed by unusual root morphology of the adjacent tooth. As this phenomenon did not mediate within the pulp chamber and the tooth-responded electric pulp testing, endodontic treatment of 16 was not indicated to arrest this type of external apical resorption. The tooth 15 was diagnosed as irreversible pulpitis, and endodontic treatment was initiated. The tooth was anesthetized with 2% articaine with epinephrine 1 : 200000 (Ultracaine DS, Hoechst Marion Roussel, Germany), and endodontic access cavity was prepared under rubber-dam isolation.\nIntraoral examination confirmed one oval shaped root canal. The working length was determined with an electronic apex locator (Root ZX, J. Morita Corp., Tokyo, Japan) and controlled with a periapical radiograph. The root canal was biomechanically prepared with H and K type files using an anticurvature step-back technique to a master apical file of 35. During instrumentation, the root canals were irrigated with 2 mL of 2.5% NaOCl (Wizard, Rehber Chemistry, Istanbul, Turkey). Final irrigation was performed with 2 mL of 15% EDTA (Wizard, Rehber Chemistry, Istanbul, Turkey), followed by a wash of 2.5% NaOCl.\nCalcium hydroxide-based intracanal dressing was applied in the instrumented root canal with a lentulo spiral. Access cavity was sealed with a temporary filling material (Cavit, ESPE, Seefeld, Germany). One week later, the root canal was obturated by the cold lateral condensation technique with standardized gutta-percha points and AH plus sealer (Dentsply de Trey, Konstanz, Germany). Another periapical radiograph was exposed to check the quality of obturation (). The cavity was restored with amalgam.\nThe patient was instructed to return for preservation radiographic exams, first, within 3 months; and finally 1 year after the completion of the endodontic treatment. After 1 year, clinical examination detected a small caries lesion in the occlusal surface of the tooth 16, which was restored with amalgam restoration. At this time, a cone-beam computerized tomography (ILUMA Cone Beam CT, IMTEC Imaging, Ardmore, Okla) of the maxilla was performed with a tube voltage of 120 kVp and tube current of 3.8 mA, for better definition and visualization of the root lengths and morphology (Figures and ). It was confirmed that endodontic treatment of the premolar had been successful, and there was no progression of the external root resorption in the neighbouring molar tooth. |
A 70-year-old Greek-Caucasian man was admitted to our hospital with diffuse abdominal pain of sudden onset three hours prior to his admission. The patient did not complain of nausea, vomiting or diarrhea and his temperature and arterial pressure were normal despite an elevated pulse rate (90 ppm.). His latest stool passage was blood-free and a digital rectal examination revealed nothing pathological.\nDuring physical examination, the patient's abdomen was mildly distended with diffuse guarding and marked rebound tenderness. Abdominal sounds were diminished during auscultation.\nLaboratory investigations revealed normal values for his hematocrit, hemoglobin, white blood cell count, and platelets. Renal and hepatic function tests were also normal and his blood glucose was a little elevated at 167.8 mg/dl (normal 70 to 110 mg/dl).\nThe patient's medical history included colonic diverticular disease, an endoscopic excision of benign rectal polyps four years prior to his presentation, and ongoing arterial hypertension and osteoporosis treatment. Ten years prior to presentation in a random ultrasound examination, the patient was found to have several simple liver cysts including two large hepatic cysts and other smaller ones. The largest cyst had a size of 13 cm. At the time, his pancreas, spleen and kidneys were normal (Figures and ). A second ultrasound examination was performed nine years after the first one (and just 13 months prior to his present admission) due to the patient being admitted after an accidental fall. A reduction in the size of the largest cyst form 13 cm to 4.6 cm and a small amount of free liquid in the patient's right abdominal fossa were identified as the only difference from the previous ultrasound report.\nDuring the patient's present admission, there was no free air in his abdomen. An abdominal X-ray examination did not show bowel air-fluid levels. Abdominal ultrasound examination showed a significant quantity of free liquid in his abdominal cavity, around the spleen and liver, as well as in the Douglas pouch. Since the patient's general condition was deteriorating and he was already showing symptoms of paleness, sweating, increased abdominal guarding and marked rebound tenderness in the whole abdominal area, we decided to perform an exploratory laparoscopy.\nThe laparoscopy revealed a vast amount of opaque-yellowish peritoneal fluid occupying majority of his abdominal cavity without any obvious origin, so the operation was converted to laparotomy.\nThe exploration of the patient's abdominal cavity revealed a ruptured liver cyst that originated from the lower surface of his right liver lobe (Figure ). Unroofing of the cyst using LigaSure to the liver parenchyma margin, plus omentoplasty and cholecystectomy, were performed as the gallbladder was part of the anterior cystic wall. Intraoperative frozen sections of multiple specimens from the cystic wall showed no evidence of malignancy, while cytology and cultures of the cystic fluid were negative. Serological tests for Echinococcus and tumor markers, CEA and CA 19-9, all showed negative results.\nTwo drains were positioned, one at the patient's cystic cavity area and the other at his Douglas pouch. The postoperative course of the patient was uneventful and three days later the drainages were removed. The patient was discharged in excellent general condition eight days after his admission. |
A 58-year-old man was admitted to the hospital, with a 3-day history of acute swelling of the right lower limb. He had undergone aortic valve replacement surgery due to severe aortic regurgitation at an outside facility 7 years before and was being treated with warfarin (5 mg daily, adjusted to maintain a prothrombin time of 2.0 INR). He denied any history of previous trauma, fractures, intravenous drug abuse, or recent infections. Physical examination revealed an 8 by 10 cm, tender, pulsatile mass in the right femoral area and swelling in the right lower limb. Both popliteal and pedal pulses were palpable. Apart from a prolonged prothrombin time (2.11 INR), blood chemistry parameters were unremarkable.\nA color Doppler ultrasound showed a huge deep femoral artery aneurysm posterior to the superficial femoral vein, measuring approximately 8.5 × 9.0 × 7.5 cm (). The deep femoral artery aneurysm compressed the superficial femoral vein resulting in venous stasis with suspicious soft thrombus formation in the right lower extremity veins (). A multi-detector CT scan (GE CT LightSpeed Ultra 16, GE, Milwaukee, Wisconsin, USA) revealed a large pseudoaneurysm arising from the deep femoral artery without evidence of aneurysms or occlusive lesions in the other arteries ().\nAn emergent operation was performed, during which an 8-cm diameter aneurysm, originating from the deep femoral artery, was identified posterior to the superficial femoral artery and vein. The superficial femoral vein was compressed by the aneurysm but there was no thrombus inside the vein. After clamping of the deep femoral artery proximal and distal to the aneurysm, the aneurysm was resected with ligation of the deep femoral artery. After resection of the aneurysm, venous flow of the superficial femoral vein was restored.\nAfter surgery, the patient experienced rapid resolution of symptoms. A follow-up color Doppler ultrasound and multi-detector CT scan showed normal flow in the superficial femoral and popliteal arteries and veins without evidence of an aneurysm (). The postoperative course was uneventful and the patient was discharged without complication. |
A 5-year-old girl was admitted to our Emergency Department after an episode of acute interscapular back pain occurring without trauma and followed by bilateral upper and lower limb weakness.\nHer family history included 8 Italian members harboring the same homoplasmic m.3460G > A mtDNA (Table , Fig. ). All the family members presented headache poorly responsive to NSAIDs and, except for the girl and her mother, visual loss due to optic nerve pathology. The patient’s medical history was unremarkable.\nThe patient’s vital signs were normal and stable. At the neurological examination, her cognitive functions were normal. There were no cranial nerve lesions. She was unable to stand and walk and had more severe weakness in the right lower limb. She also presented weakness in her upper extremities and difficulties with fine hand movements, particularly in the right hand. The right patellar and Achilles tendon reflexes were brisk. The plantar reflex was positive in the right foot. She presented impaired bladder control. No alterations in touch or pain sensitivity were present in the trunk and limbs. No concomitant signs of infection or inflammation were present, and no such signs had been reported in the previous weeks. Analyses of CSF pressure, glucose, protein, cell count, viral PCR, and culture were normal, and oligoclonal bands were absent (see timeline of events and treatment in Fig. ). The patient underwent a spinal cord MRI; the results showed hyperintense signal alterations in T2-weighted sequences and restricted diffusion in diffusion weighted imaging (DWI) sequences in the anterior portion of the cervical and dorsal spinal cord, suggesting anterior spinal artery territory involvement (Fig. ). Computed tomography angiography (CT) imaging showed no arterial dissection or other vessel abnormalities. Visual evoked potentials were normal. Somatosensory evoked potentials in both legs showed decreased conduction velocities. Motor evoked potentials showed lower amplitude for cortical derivation, prolonged latency in the upper limbs and normal in the lower limbs. Central conduction time was increased in the upper limbs and normal at the lower limbs. Complete autoimmunity and thrombophilia screening were unremarkable. Testing for anti-AQP4 antibodies was negative, and anti-MOG antibodies were not significantly elevated. A cardiologic consultation and echocardiography identified normal heart and aorta features. At the ophthalmological assessment, pupillary reactions were normal, the fundus oculi did not reveal pathological signs, and color vision was not affected. The visual acuity was 9/10 bilaterally. The digital visual field test displayed a mild defect in the peripheral portion of the visual field that was more evident in the left eye. Optical coherence tomography, visual evoked potential and electroretinograms were normal. As the child grows, her visual function will require careful monitoring, particularly when she reaches the adolescence.\nAfter obtaining written informed consent consistent with the principles set forth in the Declaration of Helsinki, total DNA was isolated from the patient’s peripheral blood according to standard protocols. Full-length mitochondrial DNA sequencing was conducted according to a previously described protocol [], and detected the haplogroup H27 was detected in the proband. The following variants were detected in the homoplasmic state: m.41C > T, m.73A > G, m.263A > G, m.1438A > G, m.3460G > A, m.4769A > G, m.8860A > G, m.11719G > A, m.15326A > G, m.16129G > A, m.16316A > G, m.16519 T > C. Based on the presence of the m.11719G > A and m.16316A > G nucleotide variants, the patient is predicted to belong to the H27 haplogroup. The m.3460G > A transition, a major mutation associated with LHON, was verified in blood-derived DNA of other family members by PCR-RFLP analysis.\nAcetylsalicylic acid was administered at low doses (2.5 mg/kg daily), and high doses of methylprednisolone (20 mg/kg daily) were empirically administered for three days. Although no proven treatments for LHON are available, early treatment with idebenone is thought to limit the progression of the disease; the patient was administered 45 mg of idebenone three times daily. Within two days following this therapy, clinical manifestations improved, and the patient regained bladder control and the ability to ambulate; within one week, the girl also recovered nearly normal strength in both arms. A control spinal cord MRI together with 1H-MRS to study the lactate peak was performed 10 days after hospital admission; the results showed complete regression of the alterations and no abnormal metabolites (Fig. ). The patient continued outpatient rehabilitation, and her motor functions improved, resulting in an almost completely normalized neurological examination after 2 months and preserving these achievements at follow-up one year later. |
A 65-year-old man with recurrent upper gastrointestinal bleeding was admitted to the Department of Internal Medicine in our institution to identify the source of the varices and therapy. The patient had two episodes of acute upper gastrointestinal bleeding within 1 year, which was treated by sclerosing of gastric varices in a primary hospital. An important note in his medical history was a splenectomy 6 decades before after splenic rupture by blunt abdominal trauma. Endoscopy of the upper gastrointestinal tract showed isolated gastric fundus varices with the absence of oesophageal varices (). To identify the source of the gastric varices, an enhanced computed tomography (CT) of the abdomen was performed and surprisingly demonstrated an 8 cm in diameter mass, mimicking an accessory spleen which\nwas located in the left upper quadrant of the abdomen, nearly adherent to the stomach and a moderate variceal conglomeration in the fundus of the stomach (). Liver cirrhosis or portal vein occlusion and other inflammatory or malignant diseases could be excluded from CT imaging. In addition, a CT scan was able to show clearly that the arterial blood supply of the accessory spleen is derived from the short gastric arteries. Splenic artery and vein could not be delineated. The patient was subsequently presented to our Department of Visceral Surgery. We decided to perform laparoscopic accessory splenectomy to relief the regional hypertension of the short gastric veins. In the operation room, the patient was positioned in right semi-decubitus position. One 12 mm Trocar, two 11 mm Trocars and on 5 mm Trocar were inserted in the left upper quadrant. The accessory spleen was extremely adherent to the diaphragm and retroperitoneal tissue (). After transection of the short gastric vessels with the vessel sealer (Ligasure™, Medtronic, Germany), the spleen was mobilized and removed (). Patient’s postoperative course was completely uneventful, and he was discharged in good health condition on the seventh postoperative day. The diagnosis was confirmed by immunohistologic examination (). |
A 79-year-old male with longstanding history of advanced prostate cancer on androgen deprivation therapy presented to his urologist after noticing a mass on the tip of his urethral meatus. A subsequent biopsy of the mass was positive for SCC, and the patient underwent partial penectomy and lymph node dissection that revealed positive right inguinal lymph nodes (three out of seven) revealing pathologic T2N2M0 disease. He received adjuvant chemotherapy by extrapolating data of its benefit when given in the neoadjuvant setting. The standard TIP regimen was not pursued given patient's concern for side effects. The patient proceeded with alternative plan of chemoradiation with 5 weeks of weekly low dose carboplatin and paclitaxel. In addition, he received radiation with a total dose of 5000 cGy over 25 fractions to the right inguinal region. However, the patient developed disease recurrence with nodal involvement nine months later. On restaging CT imaging, the patient was found to have new involvement of the left pelvis. A nodal conglomerate measuring 31×58 mm with central necrotic change was identified in the left inguinal region.\nGiven the patient's age, performance status, and local recurrence of disease, he was started on therapy with chemoradiation with curative intent one month later. Treatment with an additional round of chemoradiation with low dose carboplatin and paclitaxel was given for 5 weeks. He had radiation with a total dose of 5000 cGy over 25 fractions to the left pelvic region. He had stable disease with chemoradiation, but he eventually developed disease progression within a year from the end of chemotherapy. At that point, he was considered for second-line therapy with the PD-L1 inhibitor atezolizumab. After being on atezolizumab for approximately 2 years, he developed biopsy-proven bullous pemphigoid, an immune-mediated toxicity of the skin that has been described with those agents. A restaging scan at approximately 2 years showed near complete response, so patient has been placed on treatment holiday at the time of this report. He was started on prednisone 1 mg/kg per immune-mediated management guidelines and had quick resolution of his blistering symptoms []. |
A 59 year old female who is nonsmoker, non-hypertensive, non-diabetic with a known history of Giant Cell Arteritis (GCA), temporal arteritis on prednisone for 6 years and systemic lupus erythematous, presented with sudden onset shortness of breath, left sided chest pain and back pain. Initial chest x ray revealed widened mediastinum, opacification of left hemithorax & rightward mediastinal shift (Fig. ). CT chest revealed crescentic aortic IMH and large left sided hemothorax (Fig. ). Subsequent aortic dissection protocol CTA confirmed a focal dilation of distal transverse aortic arch (maximum diameter of 3.7 cm), a focal intimal irregularity in the distal transverse aortic arch and leaking IMH extending from the origin of the left subclavian artery to the origin of celiac artery without any evidence of active extravasation (Fig. ). There was no CT evidence of active aortitis or atherosclerosis. The patient was hemodynamically stable. Patient was urgently taken to an interventional radiology suite with an intention to perform an endovascular repair of this contained aortic rupture. Through a right groin access, an aortogram was performed and it showed a patent three vessel aortic arch, a distal transverse aortic arch dilatation near the origin of left subclavian artery and absence of active extravasation. It was suspected that this focally dilated segment of distal transverse aortic arch near the origin of left subclavian artery was responsible for large IMH and left sided hemothorax. Hence prior to aortic stent graft insertion, cerebral angiography was performed. It confirmed that the right and left vertebral arteries were communicating at the basilar confluence. The right groin access was then serially dilated to accommodate a 24 French sheath. Then two overlapping Gore thoracic stent grafts 37–37 mm × 15 cm and 31–26 mm × 10 cm were deployed within aortic arch intentionally covering the left subclavian artery and descending thoracic aorta respectively. We intentionally did not oversize the aortic stent grafts to reduce the risk of aortic intimal injury and possible dissection. Post stenting aortogram showed complete exclusion of dilated aortic arch segment. Follow up aortogram, initially showed intermittent active extravasation which ceased once the aortic stent graft was balloon molded. Then left hemothorax was decompressed by placement of a large bore chest tube. On postoperative day 1, repeat CTA showed widely patent aortic graft, significant decrease in the left hemothorax and resolution of mediastinal shift (Fig. ). Follow up CTA after one month showed widely patent aortic stent graft and complete resolution of aortic intramural hematoma (Fig. ). There was no evidence of active extravasation or endoleak. One year after the TEVAR, patient remained asymptomatic. Patient never reported any arm claudication. |
A 79-year-old male with longstanding history of advanced prostate cancer on androgen deprivation therapy presented to his urologist after noticing a mass on the tip of his urethral meatus. A subsequent biopsy of the mass was positive for SCC, and the patient underwent partial penectomy and lymph node dissection that revealed positive right inguinal lymph nodes (three out of seven) revealing pathologic T2N2M0 disease. He received adjuvant chemotherapy by extrapolating data of its benefit when given in the neoadjuvant setting. The standard TIP regimen was not pursued given patient's concern for side effects. The patient proceeded with alternative plan of chemoradiation with 5 weeks of weekly low dose carboplatin and paclitaxel. In addition, he received radiation with a total dose of 5000 cGy over 25 fractions to the right inguinal region. However, the patient developed disease recurrence with nodal involvement nine months later. On restaging CT imaging, the patient was found to have new involvement of the left pelvis. A nodal conglomerate measuring 31×58 mm with central necrotic change was identified in the left inguinal region.\nGiven the patient's age, performance status, and local recurrence of disease, he was started on therapy with chemoradiation with curative intent one month later. Treatment with an additional round of chemoradiation with low dose carboplatin and paclitaxel was given for 5 weeks. He had radiation with a total dose of 5000 cGy over 25 fractions to the left pelvic region. He had stable disease with chemoradiation, but he eventually developed disease progression within a year from the end of chemotherapy. At that point, he was considered for second-line therapy with the PD-L1 inhibitor atezolizumab. After being on atezolizumab for approximately 2 years, he developed biopsy-proven bullous pemphigoid, an immune-mediated toxicity of the skin that has been described with those agents. A restaging scan at approximately 2 years showed near complete response, so patient has been placed on treatment holiday at the time of this report. He was started on prednisone 1 mg/kg per immune-mediated management guidelines and had quick resolution of his blistering symptoms []. |
A 75-year-old Chinese man was admitted to our hospital with increasing chest pain and dyspnea upon exertion. The chest pain developed 4 weeks previously when he was climbing stairs, and this was relieved by several minutes rest. He also reported that 2 day preceding hospitalization, the pain was triggered by minimum exertion, appearing even at rest. His chest pain was characterized by a squeezing pattern and this was located in the substernal area and it radiated to his left arm. He had no history of hypertension, hypercholesterolemia, diabetes mellitus, smoking and drinking, and there was no history of ischemic heart disease in his family.\nEight years ago he was diagnosed of chronic ITP, but didn’t take any treatment. His platelet counts through the years had always been above 60 × 109/L. This earlier diagnosis of ITP was based on the observation of moderate thrombocytopenia and antiplatelet antibodies, with bone marrow findings consistent with the diagnosis. Five years ago primary PCI was performed in the proximal left anterior descending coronary artery using a drug-eluting stent (DES) because of acute anterior ST-segment elevation myocardial infarction at other hospital. At the time of the first PCI, his platelet count had been around 90 × 109/L. He received 300 mg of clopidogrel and 300 mg of aspirin along with 5000 units of heparin bolus before PCI. He was discharged on aspirin 100 mg and clopidogrel 75 mg orally daily for 1 year, and he never had bleeding before.\nOn physical examination, his blood pressure was 135/70 mmHg. His heart rate was regular at 76 beats/min. The chest was clear to auscultation and percussion bilaterally. There was no heart murmur. Findings of abdominal and neurological examinations were unremarkable, and lower extremities were without edema. A 12-lead ECG recorded on admission in the patient showed normal sinus rhythm and biphasic or inverted T waves in leads V1–3 (Fig. ). Blood examination revealed a platelet count of 18 × 109/L, mean platelet volume 13.6 fl (normal range: 6.5–11.0), troponin I level of 0.55 ng/mL (reference, <0.1), creatine kinase of 105 IU/L (normal range: 38─171), total cholesterol of 4.47 mmol/l, low density lipoprotein-cholesterol of 1.88 mmol/l, high density lipoprotein-cholesterol of 1.40 mmol/l, triglyceride of 1.29 mmol/l and fasting blood-glucose of 5.2 mmol/l. Blood coagulation tests showed a prothrombin time of 12.4 s (normal range: 12–15), an international normalized ratio of 0.97 (normal range: 0.85–1.15) and an activated partial thromboplastin time of 35.0 s (normal: 30–45 s). Brain natriuretic peptide and chest X-ray were normal. A transthoracic echocardiography demonstrated a normal left ventricular end-diastolic internal diameter of 50 mm with a slightly decreased left ventricular ejection fraction of 0.50. Diagnosis of ACS (acute non-ST-segment elevation myocardial infarction) was made. The GRACE (Global Registry of Acute Coronary Events) risk score of the patient was 143. The patient was administered high doses of intravenous nitroglycerin and oral metrolol and atorvastatin. Ten units of platelets were transfused after the patient was assessed by a hematologist, and the platelet count elevated to 87 × 109/L in the second day, and the patient was given 300 mg of aspirin and 300 mg of clopidogrel, prepared for a coronary artery angiography. Bolus injection of 3000 units unfractionated heparin was done at the beginning of the coronary angiography, and the activated clotting time (ACT) was monitored for the dose of heparin required, and then additional 3000 units were added during the PCI procedure, but GP IIb/IIIa inhibitors were not used. Coronary angiography was carried out via the radial artery, and revealed a 95 % in-stent restenosis in the middle part of left anterior descending artery (LAD; Fig. ). No other lesions were detected. The lesion in the left anterior descending artery was treated using a cutting balloon (Boston Scientific) with no complications (Fig. ). Heparin was not administered any more after the PCI, but combined anti-platelet therapy (aspirin 100 mg and clopidogrel 75 mg daily) was performed as usual. The ECG showed the T wave abnormalities in V1-3 were resolved after PCI (Fig. ). The patient was discharged asymptomatically at 3 days after PCI. Neither bleeding nor ischemic events were noted during hospitalization.\nA week after discharge the platelet count was declined to 27 × 109/L, and aspirin and clopidogrel were stopped in outpatient clinic. Oral methylprednisolone was begun at 1 mg/kg per day and tapered over the subsequent 6 weeks. The platelet count gradually recovered to 200 × 109/L after receiving 1 week of methylprednisolone. He continued the treatment with aspirin 100 mg/day, and clopidogrel 75 mg/day for one month without any further problems.\nFour months later, the patient was readmitted due to exertional chest pain he had felt for 1 month, even though he took all the medications (aspirin, clopidogrel, metrolol, atorvastatin and oral methylprednisolone) prescribed every day. On admission, the platelet count was 124 × 109/L, ECG showed normal sinus rhythm and ST segment depression in leads V2–5, and troponin I was mildly elevated (0.26 ng/ml; reference, <0.1). Diagnosis of unstable angina was made. Coronary angiography showed a 99 % restenosis at the site of the previous lesion and a 40 % stenosis in the proximal left circumflex artery (LCX; Fig. ). We used unfractionated heparin, a dose of 100 U/kg, aspirin 300mg and clopidogrel 600 mg were administrated. An activated clotting time of 358 s was achieved. A 3.5∗28 mm sirolimus eluted stent was deployed in the LAD with optimal angiographic result and Thrombolysis in Myocardial Infarction (TIMI) flow 3 in the LAD (Fig. ). There were no bleedings during or after the procedure, and at discharge the platelet count was 203 × 109/L.\nHe was then discharged on aspirin, clopidogrel, atorvastatin and oral methylprednisolone. Clopidogrel was discontinued 1 year after the procedure. During 18-month follow-up, the patient remained clinically free of symptoms without any ischemia events or bleeding complications. Coronary angiography showed stent patency at 11 months’ follow-up (Fig. ). The platelet count remained stable. |
A 17-year-old boy presented to a local hospital due to lower abdominal pain in September 2014. Abdominal computed tomography (CT) revealed a solid mass measuring approximately 74 mm × 45 mm that was attached to the greater curvature of the stomach (Fig. ). Esophagogastroduodenoscopy showed no remarkable findings. The tumor was diagnosed as a gastrointestinal stromal tumor (GIST) growing outward from the gastric wall.\nHe was referred to our hospital for management of his GIST in December 2014. He experienced no symptoms following medical examinations conducted at the local hospital. CT performed at our hospital revealed a solid mass with slightly inhomogeneous enhancement and axis lengths of 57 mm × 44 mm, which reflected a small reduction in size compared with prior CT findings (Fig. ). Therefore, we suspected that the mass might be a hematoma and suggested follow-up observation for the abdominal tumor.\nIn March 2015, 3 months after the previous examination, a third CT examination revealed that the mass had further shrunk to axis lengths of 56 mm × 37 mm and exhibited the same enhancement pattern observed previously (Fig. ). Because this slight shrinkage was consistent with the possibility of a hematoma, follow-up observation was continued.\nMagnetic resonance imaging (MRI) performed in April 2015 demonstrated a tumor with isointensity to the spleen on T1-weighted images and slightly inhomogeneous hypointensity on T2-weighted images (Fig. ).\nA fourth CT examination performed in June 2015 revealed that the mass had further reduced to axis lengths of 49 mm × 34 mm (Fig. ). Although the tumor had gradually shrunk, we could not definitively establish a diagnosis of a hematoma, as opposed to a GIST. During observation, he had no any symptoms including lower abdominal pain. Accordingly, a surgical procedure was chosen for treatment and diagnosis.\nLaparoscopic partial gastrectomy with partial splenectomy was performed by an automatic suture. Operative findings revealed that the tumor was a hard mass and was firmly attached to the greater curvature of the stomach and the inferior pole of the spleen (Fig. ). It was unclear whether this firm attachment was attributable to adhesion or direct invasion. The branches of the right and left gastroepiploic arteries fed the tumor. The feeding artery was clipped, and an automatic suture device was used to detach the tumor from the stomach and spleen (Fig. ).\nMacroscopically, the tumor measured 60 mm × 50 mm × 25 mm, and the cut surface of the resected specimen was pink and uniform (Fig. ). Microscopically, the tumor exhibited the proliferation of spindle-shaped cells and dense collagen bundles, mainly at the muscularis propria of the stomach (Fig. ), and was diagnosed as a stomach-derived mass. Immunohistological examination showed that the tumor was negative for CD34, CD117 (C-kit), desmin, S-100, and β-catenin (Fig. ). Therefore, this tumor was eventually diagnosed as a desmoid tumor derived from the stomach.\nThe patient’s postoperative course was uneventful and without complications. He continues to undergo surveillance for recurrence, and no signs of recurrence have been observed for 16 months after the operation.\nDesmoid tumors can be categorized based on three different localizations, the abdominal wall, intra-abdominal, and extra-abdominal, and the reported incidences of each type are 49, 8, and 43%, respectively. Intra-abdominal desmoid tumors are further classified into mesenteric and intrapelvic tumors. Desmoid tumors are also divided into FAP-associated and sporadic tumors. Although desmoid tumors can occur anywhere in the body, FAP-associated desmoid tumors are typically intra-abdominal. Moreover, almost all intra-abdominal desmoid tumors are associated with FAP and previous surgery. The incidence of desmoid tumors is approximately 10–15% among patients with FAP, and 12.3% of patients with desmoid tumors have been diagnosed with FAP [].\nGiven the aforementioned data, the present case was extremely rare because he had an intra-abdominal, sporadic desmoid tumor without a history of FAP, trauma, or surgery. In addition, it was particularly unusual that the patient’s tumor was derived from the stomach and gradually decreased in size. To the best of our knowledge, reports of desmoid tumors derived from the stomach and articles regarding spontaneous shrinkage of an intra-abdominal desmoid tumor are rather scant [–]. Thus, we initially suspected that the tumor was either a GIST or a hematoma. As far as I heard the patient, there were no any abdominal trauma that caused a desmoid tumor or hematoma. However, the patient was an active high school boy, and we also hypothesized that the tumor was a hematoma that resulted from unnoticed abdominal trauma sustained when the patient was playing with his friends. However, the shrinkage of this tumor during observation puzzled us. The gradual reduction of the tumor was not consistent with a GIST. However, the size reduction would have been unexpectedly small if the tumor had been a hematoma. Therefore, the patient underwent complete resection.\nIn fact, despite its observed reduction in size over time, the tumor was neither a GIST nor a hematoma but rather a desmoid tumor. Diagnosis was difficult given the spontaneous decrease in tumor size. Few literature reports have described spontaneous shrinkage of desmoid tumors without treatment. A retrospective review has reported the disappearance or diminishing of five of eight tumors []. The reasons underlying tumor shrinkage remain unclear.\nIntra-abdominal desmoid tumors have a tendency to recur locally after surgical resection, but they are not associated with the ability to metastasize []. The recurrence rate for desmoid tumors is high (30 to 40%) []. Although the recurrence rate given associated FAP that can reach 90%, the corresponding rate for sporadic desmoid tumors may only reach 10%. The optimal therapy for desmoid tumors remains controversial because large randomized studies are not abundant due to the rarity of such tumors. However, certain studies have suggested that surgical resection with negative margins is one of the most effective therapies [–]. In contrast, other authors have reported no relationship between surgical margins and local recurrence []. In any event, careful follow-up after surgery is required. |
A 34-year-old man sustained multiple injuries including a total left brachial plexus injury (BPI) in a motorcycle accident. He was referred to us about five months after the injury for the treatment of a total palsy of his left brachial plexus and left accessory nerve. Six and a half months after the injury, his left brachial plexus was explored and its somatosensory evoked action potentials were studied. No response to stimulation of the cervical fifth (C5), C6, and C7 nerve roots was found, but there was a response to medial cord stimulation. Although Tinel’s sign was noted in the front axillary area at the time of exploration, functional recovery of the ulnar nerve was unlikely. We thus used the ulnar nerve vascularized by the superior collateral vessels as an interposition graft to connect the affected median nerve to the contralateral C7 nerve root (CC7 transfer\n[]). For the restoration of elbow extension, the proximal ulnar nerve stump was connected to a branch of the radial nerve innervating the long head of the triceps brachii muscle. For the restoration of shoulder abduction, the median nerve with the medial cord contribution was connected to the axillary nerve.\nThe patient’s left elbow joint developed severe contracture because of heterotopic ossification, so three months after the nerve transfers, the patient received surgical resection of the ectopic bone and mobilization of his left elbow. The passive range of elbow motion was 120° in flexion and –20° in extension after a one-year rehabilitation exercise program.\nTwo years after the nerve transfers, X-ray examination showed that his left shoulder was not subluxated. He had obtained left shoulder abduction and elbow extension to the M2 level of the Medical Research Council scale. At this point, we planned to restore his elbow flexion and prehension using a double free muscle transfer (Doi’s procedure\n[]). For the first step of Doi’s technique, we identified a muscle with a very large muscle belly in the medial thigh, where the gracilis and adductor longus muscles should be located. The width of this large muscle was 12 cm, whereas that of the normal gracilis muscle is about 3.5 cm\n[,]. The deep femoral artery was exposed by retracting the large muscle medially (Figure \n). Based on this anatomical situation, we considered it probable that the very large muscle represented a fusion of the gracilis and adductor longus muscles (Figure \n).\nThere were two significant arteries nourishing the muscle. The proximal artery originating from the medial circumflex femoral artery entered the muscle at the center of the muscle belly, 6 cm caudal to the pubic tubercle. The distal artery, which was considered to arise from the superficial femoral artery, joined the muscle, 18 cm caudal to the tubercle. The diameters of both arteries were almost the same (about 2 mm). A branch of the obturator nerve, which looked anatomically normal, innervated the muscle. We wondered if the proximal vascular pedicle alone would be able to nourish the entire volume of this large muscle, but we were also concerned that anastomoses of both vascular pedicles at the recipient site would affect excursion of the muscle. Because several authors\n[,] have described the successful simultaneous transfer of the gracilis and adductor longus muscles with a common vascular pedicle, the distal vascular pedicle was ligated and the proximal pedicle was sutured to the left thoracoacromial vessels (one artery and one vein). Because of the persistent total paralysis of the patient’s left spinal accessory nerve, the muscle was innervated by a part of the vascularized ulnar nerve that had been transplanted in the previous CC7 transfer\n[]. A monitor flap could not be attached to the muscle because using Doppler sonography we could not identify septocutaneous or intramuscular perforators from the nutrient vessels to the skin overlaying the muscle. The vascularity of the transplanted muscle was monitored by Doppler sonography and a visual check of the color of the muscle belly through a small skin window created over the muscle at the recipient site.\nTwo weeks after the surgery, a significant exudate was found in the wound. The wound was opened extensively in the operating theatre. Although the muscle color seen through the skin window was good and the sound of arterial blood flow was clearly audible with Doppler sonography, the lateral one-third of the muscle exhibited ischemic changes (Figure \n). The wound was infected by Staphylococcus epidermidis, which was treated with a course of antibiotics. We debrided the ischemic part of the transplanted muscle, from which no bleeding was observed. The skin defect in front of the shoulder region was covered by a pedicled muscle flap from the pectoralis major muscle (clavicular portion) and a lateral thoracic flap.\nEight months after the FFMT, because the sternal portion of the pectoralis major muscle had contracted significantly, the muscle was attached to the side of the surviving portion of the previously transplanted muscle to augment the strength of the muscle. Ten months after the first FFMT, a second FFMT\n[] was performed for the restoration of finger flexion, using the left gracilis muscle, which was anatomically normal. The recipient vessels were the thoracodorsal artery and its corresponding vein. The recipient motor nerves were the fifth and sixth intercostal nerves. At the latest follow up, two years after the second FFMT, the patient has obtained M4 level motor function in his left elbow flexion and can use his hand with a hook grip. |
The patient is a 62-year-old Caucasian female who underwent a diagnostic workup for shortness of breath and persistent cough including CXR and CT. CT reported an enhancing mass in the upper abdomen, which was believed to be an exophytic mass from the stomach (Fig. ). At this time, a GIST was considered as a preoperative diagnosis. She then underwent endoscopic ultrasound (EUS) with simultaneous fine needle aspiration (FNA), which revealed a 4.6 cm × 2.7 cm mass adjacent to the angularis versus originating from the outside wall layers of the stomach pressing on the angularis. The mass did not appear to be vascular, but rather solid. Additionally, a conglomerate of lymph nodes was noted in the mid mediastinal area with some lymph nodes measuring over 3.0 cm. FNA of the mass and lymph nodes showed no evidence of metastasis, but proved non-diagnostic. It was decided to proceed to the operating room for resection of the lesion as findings raised concern for cancer.\nSurgical findings revealed a hemorrhagic appearing mass in the epigastrium that was not part of the liver and appeared to arise from the stomach near the lesser curvature. Grossly, the mass was soft and pliable without any typical features of malignancy. Examination of the mucosa via gastrostomy revealed no abnormalities thus local excision of the mass through gastrotomy with 0.5 cm margins was performed.\nMicroscopically, the mass appeared to be arising in the muscular wall or submucosa of the stomach and was composed of sheets and islands of small, uniform cells with round central nuclei, small amounts of clear cytoplasm and relatively distinct cell borders. The neoplasm contained many blood vessels, some with a staghorn pattern. The neoplasm had areas of cystic degeneration and calcification but no definite tumor necrosis. No mitoses were identified. The neoplasm had invaded through the muscularis propria into the subserosa. Areas were identified where serosa appeared to have been stripped away from the tumor; however, there did not appear to be neoplasm on the actual serosal surface. The maximum size of the tumor was 3.0 cm. Margins of excision appeared negative for involvement.\nMultiple immunohistochemical stains were performed with the following results: Actin and calponin positive; pancytokeratin (Lu5), chromogranin, synaptophysin, S100 protein, C117/c-kit and CD34 negative.\nIn this case, the patient was followed up appropriately post-operatively and recovered uneventfully. She was advised to follow up with an oncologist for preventative testing and monitoring of any future malignancies. |
A 67-year-old female underwent a radical laparoscopic hysterectomy, with adnexectomy and bilateral pelvic lymphadenectomy for a FIGO stage IB1 squamous cell cervical cancer in January 2010. Final pathology report showed a 9-mm squamous cell carcinoma with deep stromal invasion, positive resection margins, and one metastatic lymph node, without vascular embolism or parametrium invasion. Based on pathologic risk factors, adjuvant radiation therapy with radiosensitizing cisplatin was suggested. Unfortunately, the patient was not compliant, she was lost to follow-up and the adjuvant treatment was never delivered.\nIn September 2014, the patient presented to our department with abnormal vaginal bleeding and pelvic pain. On pelvic examination, a tumor of an approximately 4 cm gross circumferential at the vaginal cuff was palpated with left parametrial involvement. A pelvis magnetic resonance imaging (MRI) showed a central pelvic recurrence of the vaginal cuff, as a left hemi circumferential tumor, measuring 44 mm with intimate sigmoid contact and without obstructive uropathy or pelvic wall involvement (Fig. ). A biopsy of the lesion demonstrated infiltrate and moderately differentiated squamous cell carcinoma of the vaginal cuff. Additional work up including CT scan of the chest and abdomen does not found distant metastasis.\nThe patient was treated with dual arc RapidArc radiotherapy. The treatment plan was designed to deliver in one process with simultaneous integrated boost (SIB) a dose of 70 Gy to the planning target volume (PTV) based on the gross disease in a 2-Gy daily fraction, 5 days a week. At the same time, the subclinical disease was planned to receive 54 Gy in a 1.5-Gy daily fraction (Fig. ). Seven cycles of concurrent radiosensitizing cisplatin was also delivered without toxicity.\nA subsequent clinical evolution was marked by the disappearance of bleeding and the tumor mass. MRI scan realized 6 months after completing treatment showed a complete response (Fig. ).\nAfter primary surgery for cervical cancer, subsequent pelvic recurrence is effectively managed with external beam radiotherapy and concurrent cisplatin-based chemotherapy due to the presentation of a centrally recurrent cervical cancer of the vaginal cuff, as seen in 35–40 % of cases []. Surgical management is an option therapy but it imposes radical pelvic surgery in order to achieve surgical resection with curative intent. However, in locally recurrent disease, radiation offers long-term pelvic control and prolonged survival [].\nOne recent retrospective study examining outcomes of patients undergoing salvage radiotherapy alone for recurrent cervical cancer following radical hysterectomy found that is an effective treatment for who recur at the vaginal cuff with relatively little morbidity but it is less effective in patients where recurrence lies outside the central pelvis []. Generally, it is accepted that the increasing dose to the target volume in external beam radiotherapy will lead to increased local control. Intensity-modulated radiotherapy (IMRT) is seen as the way to achieve this. It facilitates the delivery of differential doses of radiation to a specified target volume and the escalating of dose prescription. Early experience found that IMRT were able to achieve excellent coverage of target volumes, and there was very little acute gastrointestinal toxicity as compared with conventional external beam radiotherapy techniques [].\nRapidArc radiotherapy is an excellent option used to salvage our patient with a central pelvic vaginal cuff recurrent lesion which seems to obtain better dosimetric results compared to IMRT, with fewer monitor units, and a significant decrease in treatment time []. Pelvic radiation can lead to urinary symptoms and bowel changes. However, RapidArc might reduce acute rectal and bladder toxicity compared with conventional techniques []. Concurrent cisplatin-based chemoradiotherapy has also been used in the management of recurrent cervical cancer. This approach has provided both better local control and survival with acceptable toxicities in women with locally recurrent cervical cancer in several studies [, ].\nSurveillance after primary curative therapy for cervical cancer is uniformly recommended, although its effectiveness is not well studied []. The concept of long-term surveillance for patients treated with curative intent is based on the premise that early detection of recurrence may lead to treatments that have lower morbidity and increase survival. Early detection of recurrence is aimed at treating patients with potentially curative salvage therapy. This is most likely in patients who have an isolated central pelvic recurrence. |
A 54-year-old man with right aortic arch and Kommerell's diverticulum presented with refractory hemoptysis due to a rapidly growing mycobacterium avium complex (MAC) infection, which could not be controlled by bronchial artery embolization. Left lower lobectomy and lingular segmentectomy were performed. Although antimycobacterial agents were continued, MAC infection relapsed in the left upper segment. Eight months after the initial surgery, complete pneumonectomy was performed. The lung was strongly adherent to pericardium and diaphragm. The adherent lung was slightly injured after the adhesiolysis, and the chest cavity was minimally contaminated. The wound was closed after thorough irrigation of the chest cavity. The postoperative course was uneventful and the patient was discharged on postoperative day 14. Three months after the pneumonectomy, he developed shortness of breath. He had neither a history of asthma nor congenital heart disease. The symptom worsened over the course of several days, and he was transferred to the emergency department with acute respiratory distress. He was intubated with a single-lumen orotracheal tube, and positive pressure ventilation was initiated. CT scan showed a narrowed bronchus intermedius and the right lower bronchus compressed between the right pulmonary artery and the right descending aorta, although the airway was barely patent (). Bronchoscopy revealed an almost completely obstructed bronchus intermedius. The patient became hypoxic soon after bronchoscopy, perhaps due to mucosal edema. Ventilation was maintained by systemic steroid and transbronchial administration of diluted adrenaline, but the hypoxic attacks frequently relapsed. Before the airway was completely obstructed, percutaneous cardiopulmonary support was initiated via a femoral artery and vein. The endotracheal tube was then replaced by a double-lumen endobronchial tube under bronchoscopy. The tip of the tube was placed into the bronchus intermedius, and the second lumen orifice was open to ventilate the right upper lobe. One of the treatment options was temporary placement of a bronchial stent, although the feasibility and efficacy were uncertain. Permanent placement of a bronchial stent was also not indicated due to the risk of aortobronchial fistula. Thus surgical repositioning of the mediastinum was indicated. The left thoracic cavity was reopened, and complete adhesiolysis was performed. To maintain the proper mediastinal position, considering the emergent setting, an open-window thoracostomy was created with resection of the second to ninth ribs, and piles of gauze were inserted, mildly compressing the heart and the mediastinum to the right side. The inserted gauze and dressings were exchanged every day. The patient was extubated on postoperative day 5. CT scan showed a repositioned mediastinum and sufficiently patent airways (). Three months after repositioning of the mediastinum, the thoracostoma was closed with a latissimus dorsi muscle flap covering the heart. Eventually, the patient was discharged without any dressings needed. Two months after thoracoplasty, no relapse of PPS has been seen so far (). |
A 29-year-old military male basic trainee (71 inches, 200 pounds, BMI 27.89) presented with a one-month history of atraumatic bilateral leg pain. He reported pain within the first week of initiating running at basic training. He was diagnosed with bilateral proximal tibia stress fractures at three weeks with instructions to stop impact activities. After failure of his symptoms to improve, he was referred to our facility. On presentation, he had tenderness about the medial aspect of each proximal tibia. Radiographs obtained at that time demonstrated bilateral proximal tibia stress fractures with varus alignment about each knee and articular collapse of the left tibial plateau. Metabolic labs were obtained and significant for a low vitamin D (15), but the remainders of labs including calcium, thyroid-stimulating hormone (TSH), and testosterone were unremarkable. MRI of the left knee demonstrated a medial tibial plateau fracture with two millimeters of articular depression and extension of the fracture to the tibial spine (). MRI of the right knee demonstrated a hypointense linear T2 signal surrounded by diffuse hyperintense signal suggesting a medial tibial plateau stress fracture with surrounding bone edema, without articular collapse or extension (). These fractures were at the level of the physis, and there was surrounding sclerosis on plain radiographs. Given the collapse of the articular surface in his left knee, the patient was indicated for open reduction internal fixation with allograft bone. The patient underwent the aforementioned procedure without complication, and the patient remained nonweight bearing to his bilateral lower extremities (). At the time of surgery, the bone at the fracture distal to the articular surface was softer and more friable in composition than expected for the stress fracture. It was easily debrided back to stable, healthy bone edges, and we were interested in analyzing its composition, so a biopsy was obtained and sent to the lab for analysis. Histology demonstrated nonossifying hyaline cartilage with admixed fibroconnective tissue consistent with a persistent physis ().\nAt the six-week follow-up visit after the procedure on his left knee, the patient was still having significant pain in his right lower extremity and, after extensive counseling, elected to undergo the same procedure on the contralateral limb in an effort to return to running and continue his military career (). We suspected that a similar pathology of a persistent physis was present in the right proximal tibia and that this was contributing to his slow healing progression. Similar intraoperative pathology specimens were again obtained at the fracture site distal to the articular surface (), and these demonstrated fragments of purple to purple-gray staining matrix with chondroid metaplasia. This presence of cartilage distal to the articular surface was consistent with a persistent physis.\nAt four-and-a-half months from the index procedure (three months from the staged procedure), the patient was ambulating without assisted devices and had painless range of motion from 0 to 120° in each knee with well-healed incisions, maintained hardware and alignment, but had not returned to impact activities. Due to his status as a basic trainee, he was released from active duty at six months' time postoperatively and was lost to follow-up. |
Early on October 2014, a 45-year-old woman was presented at our outpatient gynecology clinic with sudden lower pelvic discomfort and vaginal bleeding symptoms. The patient had a history of hysterectomy 13 months ago due to symptomatic multiple leiomyomas and adenomyosis.\nThe previous surgery was conducted as a single-port approach laparoscopic-assisted vaginal hysterectomy in July 2013. Surgical findings showed an enlarged uterus of about a 14 weeks gestational age size. Both adnexa were grossly normal in appearance and the peritoneum was clear with no signs of endometriosis. The vaginal vault was sutured vaginally using a 1-0 vicryl. No complications were observed during the postoperative period and the patient was discharged as scheduled. The histology of the uterus was confirmed as adenomyosis with leiomyoma with a secretory phase endometrium. Follow up examinations at 3 and 6 months showed no complications and thus no additional follow-ups were required afterwards.\nHowever on October 2014, after more than a year from her last check up, the patient appeared at our clinic complaining of sudden pelvic discomfort and vaginal bleeding symptoms. Pelvic examinations showed no signs of active bleeding. Yet, a dark wine colored papule suggestive of a small hematoma or ulcerative lesion was observed upon the previous operative vaginal vault site ().\nHer vitals were stable and she showed no signs of fever. The pain was tolerable during manipulation of the vault site. The ulcerative lesion was suspected as an old hematoma or granulation formation of the previous hysterectomy vault or possibly due to an unknown malignancy or cancerous change.\nFor further investigation, a quick excision biopsy using sharp scissors was performed under local anesthesia at the outpatient operation room. Bleeding control was done with sutures and tampon gauzes were inserted intravaginally. Nevertheless, the patient returned to the clinic within a few hours with excessive bleeding. Upon re-examination, active bleeding was visible at the vault site, and additional maneuvers including sutures were performed for hemostasis.\nThe pathology reports showed normal vaginal tissue with non-specific loss of surface epithelium and subepithelial hemorrhage. The diagnosis was uncertain but any kind of malignancy could be ruled out.\nTen days later, the patient revisited our clinic with another incidence of massive vaginal bleeding with large clots. She looked pale and anemic in general with vital signs showing an increased pulse with a decreased blood pressure at an initial 130/70 to 100/60. The patient also complained of symptoms of dizziness. Her lab results showed a hemoglobin level of 6.9 g/dL and hematocrit of 20.2%. Further attempts to achieve hemostasis with ball electrode cauterization were insufficient. The lesion healed only for a short while and the fragile tissue continued to bleed. Because of the massive bleeding, a clinical diagnosis of a possible vascular malformation or uterine artery pseudoaneurysm was considered.\nAn emergent exploratory laparoscopic operation was performed to control vault bleeding and to evaluate any possible vascular complication or collateral arterial bleeding. Due to anemic conditions, transfusion with 2 pints of packed red blood cells were necessary prior to the operation.\nUpon surgery, other peritoneal structures including both ovaries were grossly normal and showed no signs of endometriosis. The pelvic side vault site was securely sealed with normal peritoneum covering the vault with no dehiscence or any other complication. Under conventional laparoscopy, after dissecting the anterior rectal wall and bladder peritoneum, a full thickness excision of approximately 2 cm in diameter was performed at the vault site and resealed with sutures.\nPathology results of the excised lesion was confirmed to be consistent with endometriosis (). Hence, our difficult diagnosis of delayed vault site bleeding turned out to be a rare case of vault site iatrogenic endometriosis. |
A 78-year-old male presented to the authors' institution after noting a painful soft tissue mass located in his medial right upper thigh 4 months previously. Eight years priorly, he was hospitalized for lower GI bleeding. A colonoscopy performed at that time revealed the presence of a soft and friable rectal mass 2 cm proximal to the anal sphincter. He subsequently underwent transanal endoscopic sphincter-sparing surgery. Histology revealed the presence of a GIST. The patient remained free of disease for 3 years, when routine surveillance revealed local recurrence. The patient underwent interval transanal surgical resection and was started on systemic imatinib mesylate, which was discontinued 2 years later after the development of considerable side effects of nausea and vomiting. The patient was disease-free with no evidence of recurrence or metastatic disease until his current visit at the authors' institution due to the aforementioned painful soft tissue mass. An MRI with contrast enhancement of the right thigh was obtained, including T1- and T2-weighted as well as fat suppression images, which revealed the presence of a 9.0 × 6.0 × 5.0 cm lesion in the right adductor magnus muscle, with sparse areas of necrosis and irregular contrast enhancement (). Bone scanning Tc99m showed an area of increased metabolic activity in the right thigh with no other abnormalities indicative of metastases.\nA percutaneous CT-guided biopsy was performed which was consistent with a tumor of mesenchymal origin. The patient subsequently underwent wide excision of the lesion under general anesthesia. Grossly, the tumor presented as an intramuscular mass of 9 cm in largest diameter. Upon sectioning, it showed central necrosis and macrocystic change (). Histopathological examination revealed a hypercellular neoplasm. The neoplastic cells were arranged in either a fascicular or a whirling pattern and exhibited spindle and epithelioid morphology (). The mitotic index was high, counting over 10 mitoses per 20 high power fields. Areas of hemorrhage, myxoid change, and necrosis were also identified. At the periphery, the tumor was focally infiltrating the surrounding muscle fibers. Based on these findings, the tumor was classified in the prognostic group 6a (high grade) based on the Miettinen and Lasota/AFIP criteria for metastatic risk [], which is in concordance with the malignant behavior of the tumor. Immunohistochemically, the tumor cells were strongly positive for c-KIT and CD34, focally positive for SMA, and negative for desmin and S100 (). Interestingly, both the metastatic and primary tumors were negative for DOG-1. These findings were consistent with metastatic infiltration of the adductor magnus by a GIST. Surgical margins were free of disease for at least 5 mm. Instructions were given for close oncologic surveillance thereafter. At the last follow-up, 18 months postoperatively, the patient demonstrated no signs of local or systemic disease. |
A 27-year-old male patient with an unremarkable medical history was referred to the Department of Oral and Maxillofacial Radiology with a complaint of pain in the region of the lower right molar teeth.\nAn intraoral examination revealed irregular morphology of the mandibular permanent third molar. This morphology suggested the presence of the union of an extra cusp on the lingual aspect of a wisdom tooth. This union led to the development of a groove formation that caused periodontal problems. The gingiva around the right mandibular third molar appeared to be reddish in color, with loss of stippling and an inflamed appearance ().\nA panoramic radiograph indicated the fusion of a supernumerary tooth with the permanent third molar and the presence of a dilated follicular space associated with this abnormal formation. This irregular enlargement exhibited a sharply delineated area of radiolucency around the fused supernumerary tooth (). However, the panoramic radiograph was not sufficient to visualize this dental formation and its surrounding tissues because it only provided information in the mesiodistal plane. With the consent of the patient, a CBCT image was taken to observe the abovementioned teeth, the periradicular lesion, and the relationship thereof with the peripheral structures.\nThe CBCT scans of the right side of the mandible in all orthogonal planes showed a fused supernumerary molar on the lingual aspect of the third molar (). Around the periradicular region of these malformed teeth, a cystic lesion was identified in the axial, coronal, and sagittal planes (). Additionally, an axial section revealed perforations of this cystic lesion on the buccal and lingual borders of the compact bone (). Furthermore, the presence of a retromolar canal was incidentally identified on a sagittal CBCT image ().\nA treatment plan was developed, including the extraction of the fused teeth and the cystic lesion. The fused teeth were extracted with an inferior alveolar nerve block (); subsequently, the cystic lesion was curetted and sent to the Department of Pathology for a definitive diagnosis. Based on pathological and histological examinations, it was identified as a paradental cyst (). |
A 23-year-old, Caucasian, right hand dominant male, otherwise healthy, was seen in the emergency department after a skateboard accident. He reported falling onto his dorsal left proximal forearm and experienced immediate pain in the area without other injuries. Physical examination was notable for tenderness over the proximal radius and ulna and limited pronosupination secondary to pain. Plain radiographs demonstrated non-displaced but slightly comminuted, short oblique fractures of the proximal third of the radius and ulna (, ). After discussion with the patient and his family, the decision was made to place the patient in a long arm cast (, ) and follow-up in the clinic in 1 week.\nOn follow-up, the patient had satisfactory pain control, and the cast was appropriately in position. A hole was cut into the cast so that an ultrasound bone stimulator could be used to promote healing. The stimulator was positioned equidistant between the two fractures. The patient reported some mild increase in fracture site pain in the 1st week with the bone stimulator, but this abated after a few days. The next follow-up visit was 3 weeks later (4 weeks post-injury), and the patient reported significantly improved pain. The cast was removed, and his examination was significant for restoration of pronation and supination to 70 degrees each. Imaging demonstrated appropriate healing of the fracture with callus without displacement or malalignment (, ). Another long arm cast was placed for an additional month, at which time the fractures were completely clinically healed, so a removable splint was placed. With the cast off, the ultrasound stimulator could be placed directly over the fracture sites, one at a time.\nOn his final follow-up visit, the patient was 3 months out from his injury. He had been continuing to use the bone stimulator and reported no pain. On examination, he had 90 degrees of pronation and supination; wrist extension was 60 degrees and wrist flexion was 80 degrees, all of which were similar to the opposite side. Imaging demonstrated healed radius and ulna fractures with no malalignment (, ). Given these findings, the patient was told to return to baseline activity with the exception of a custom forearm shell splint for skiing and to return to the clinic as needed. |
A 37-year-old female visited with a complaint of gradually decreased visual acuity. At the first ophthalmological examination, the corrected best visual acuities in the right eye and the left eye were 1.0 and 0.4 respectively. Slit lamp examination revealed that there was no specific finding in the anterior segment, and the funduscopic finding of both eyes showed that cotton wool spots, macular edema and vascular tortuosity were present around the optic disc in the left eye (), but the right eyes was unremarkable except the suspected cotton wool spots near superotemporal arcade (). Fluorescein angiography revealed microaneurysms and macular edema in the left eye (), and right eye showed minimal microaneurysms without macular edema (). We found that the thickness of macula was increased when measured by optical coherent tomography (). Past medical history included modified radical mastectomy for right breast cancer 5 years ago. She had no medical history of diabetes, hypertension and systemic vascular disease and no evidence of abnormalities in laboratory finding.\nOne year before this visit, she was confirmed to have metastatic tumor on her right frontal lobe shown on MRI () which was taken after a sudden convulsion. And she had received radiation therapy of fractionated external irradiation to the whole brain (3,000 cGy/10fraction) for 2 weeks and local boost irradiation (1,200 cGy) After that, she was transferred to another hospital for gamma knife surgery on that lesion. Total dose of gamma knife surgery on the tumor bed was 40 Gy and the amount of irradiation for each eye was 0.2 Gy.\nImmediate intravitreal TA injection was performed for macular edema and two weeks later, the visual acuity was improved to 0.6. But the visual acuity was decreased to 0.3 again 3 months later, so we performed intravitreal TA injection again. Three months after the second injection, the fundus examination and optical coherent tomography revealed that retinal thickness was improved and the corrected best visual acuity was 0.5. |
A 60-year-old female patient reported with the complaint of a growth on the palate and inability to wear the denture since 3 months. On clinical examination, the lesional mass was seen involving the entire hard palate as a pedunculated mass, measuring approximately 3 cm × 3 cm, attached to the palatal mucosa with a narrow stalk. The base of the pedunculated mass, the palatal mucosa and the surrounding alveolar mucosa showed areas of brownish black pigmentation. The mucosa on the oral side of the lesion showed a pale whitish color []. On palpation the lesion was smooth, non-tender, well defined, firm to hard in consistency with induration of the posterior margins.\nOn computed tomography an enhancing, infiltrative soft tissue mass arising from the hard palate with subtle bony erosion noted over the mid portion of the hard palate [] and bilateral submandibular lymphadenopathy were evident.\nA provisional diagnosis of malignant melanoma was considered and an incisional biopsy was performed which confirmed the diagnosis. The patient underwent total maxillectomy with neck dissection.\nThe histopathological examination of the excised tissue revealed cells with a clear cytoplasm and hyper chromatic nuclei resembling atypical melanocytes seen proliferating from dermal-epidermal junction into the connective tissue. The atypical melanocytes were epitheloid in the superficial region [], and spindle shaped in the deeper regions of the connective tissue. The spindle cell type predominated showing an irregular branching pattern with intervening fibrous septae [], these cells appeared fusiform to round in different orientations []. Atypical features like mitosis, multi-nucleation, pleomorphism, hyper chromatism, increased nuclear cytoplasmic ratio and prominent nucleoli were seen []. Pigmented nests of atypical melanocytes along with melanophages were seen in the superficial region. The tumor-stroma interface showed a marked inflammatory response and dense fibrosis. The evaluation the harvested submandibular lymph node showed metastatic involvement. The histopathology confirmed the diagnosis of malignant melanoma with metastatic level I nodes. The spindle cell predominance with collagenous stroma was suggestive of desmoplastic malignant melanoma.\nThe patient underwent reconstruction, prosthetic rehabilitation, and has been on regular follow with no recurrence till date. |
A 28-day-old infant boy was referred from the district to the University Children’s Hospital to diagnose and treat severe spontaneous epidural bleeding, which was the original diagnosis. He was the fourth child of young parents, born from the fourth pregnancy, which ended at week 42. During the first 4 weeks of life, he did not show any symptoms of a congenital defect or any significant comorbidity.\nOn admission to the emergency department of a regional hospital, he was anxious and crying which was followed by a deep coma with a Glasgow Coma Scale score decreasing from 7 to 3 points. Anisocoria, bradycardia of 80–100 beats per minute, and bradypnea of 15 breaths per minute were also observed. Laboratory investigations revealed severe anemia and significant coagulopathy. No traumatic event or external action involving another person was reported in the anamnesis. Computerized tomography of the head revealed an intracranial hemorrhage and mass effect due to severe epidural hematoma of 0.11 cm with disseminated ischemic foci. The patient underwent urgent extensive frontoparietal craniotomy performed as a life-saving procedure by surgeons in the regional hospital. Broad-spectrum antibiotic prophylaxis was considered and ceftriaxone seemed to be the best choice. Ceftriaxone was administered intravenously at a dose of 100 mg/kg once a day. When the patient’s clinical condition was deemed to be compatible with stabilization of the respiratory and circulatory systems, he was transferred to the University Hospital for Children to continue advanced intensive neurosurgical treatment.\nOn admission to the pediatric intensive care unit, the severity of the neonate’s illness was assessed at 20 points according to PRISM (Pediatric Risk of Mortality), with a predicted risk of death of 34.4%. He was put into a barbiturate-induced coma as a neuroprotective strategy with supplementary sedation and analgesia due to the need for mechanical ventilation as a result of postoperative respiratory insufficiency. The patient was ventilated to normocapnia with pressure-controlled ventilation and parameters corresponding to his age and respiratory condition (Maquet ventilator, DrägerWerke AG, Lübeck, Germany). Empirical antibiotic therapy with ceftriaxone was continued, used previously as prophylaxis (100 mg/kg every 24 hours), because of its excellent penetration into the cerebrospinal fluid (CSF). The choice of ceftriaxone from the third-generation cephalosporins was consistent with the recommendations at our institution.\nLaboratory investigations revealed deep, normocytic (mean corpuscular volume 82.9–84.6 fl) and normochromic (mean corpuscular hemoglobin concentration 34.4–36.9 g/dL) anemia (hemoglobin 7.8 g/dL, hematocrit 23%) as an effect of severe brain hemorrhage and blood loss. Additional results revealed a slightly elevated level of total bilirubin (3.7 mg/dL), a lower total protein (4.4 g/dL), and normal aspartate transaminase (36 IU/L) and alanine transaminase (11 IU/L) (). The coagulation screen showed normal antithrombin activity (70%) with an increased international normalized ratio (3.57) and a decreased prothrombin time (60%). Protein C and protein S levels were 44.7 U/dL and 98.8 U/dL, respectively. The patient required transfusions of red blood cells, fresh frozen plasma, and vitamin K supplementation. At the same time, a number of metabolic investigations were carried out in search of the primary cause of the unexplained intracranial bleeding. However, these excluded any congenital metabolic disorder.\nOn the second day of hospital stay, the patient underwent computerized tomography of the head and ultrasound imaging of his abdomen as a routine pediatric intensive care procedure. Post-craniotomy axial computerized tomography scans revealed extensive hypodensity involving the gray as well as the white matter of the left hemisphere with bleeding foci. Midline shift and uncal herniation were also seen (). Also the following sonographic findings were observed in the abdomen: dilation of the biliary tree, thickening of the dilated cystic duct wall, common hepatic duct, and common bile duct () and mass-like sludge in the gallbladder ().\nIn view of the ceftriaxone therapy, the picture of biliary sludge suggested ceftriaxone-associated pseudolithiasis. Ceftriaxone was discontinued on the following day, after a total therapy that lasted 3 days. At this time, the ursodeoxycholic acid was administered to the child at a dose of 15 mg/kg twice a day and fat-soluble vitamins as supplementary therapy. After 2 weeks of treatment, ultrasonography revealed partial and gradual improvement and no surgical intervention was required (). Following another week, ultrasound examination showed complete normalization of image (). There was a gradual improvement of the radiological image over the course of 3 weeks and the bilirubin remained elevated for the first 14 days, reaching a maximum level of 8.38 mg/dL. Nevertheless, due to the prolonged hyperbilirubinemia, phototherapy was also introduced for the next 3 days, but no spectacular improvement was achieved. Despite this drug-related complication, the infant’s neurological status gradually improved, and he was able to be weaned and disconnected from the ventilator. He was successfully extubated on day 12 of his stay in pediatric intensive care. The first neurological examination was made when the child was conscious (Glasgow Coma Scale score of 12 points) and revealed signs of right-handed paresis. The patient was transferred to the surgical department to continue treatment and rehabilitation. |
A 64-year-old female patient presented to the emergency room with pain in her right distal thigh. She had a history of a minor fall. Physical examination revealed a swollen, painful right thigh. Except for a BMI of 31, the patient had no other known comorbidities and was under no medications. She lived in a rural area far from the capital.\nShe was eventually diagnosed with a distal femoral fracture (Figure ). Preoperative blood tests were normal with no elevation of C-reactive protein (CRP) or white blood cells detected. The fracture was reduced and internally fixed with a minimally invasive technique. The patient was discharged two days later. The surgery was performed by a junior surgeon who failed to recognize the initial nature of the fracture despite the CT scan (Figure ).\nInitial follow-ups were carried out by her GP. The patient described persistent pain at the operation site. She was able to be mobile indoors only. Her radiographs in the second month postoperatively (Figure ) showed no signs of healing and revealed lysis and resorption around the fracture site.\nA biopsy was scheduled to be performed for a differential diagnosis including osteomyelitis, benign cystic lesion, giant cell tumor, fibrous dysplasia, and neoplastic lesions/metastases. However, the patient did not consent to a biopsy and only a CT scan of the bone was performed, which revealed thinning of the cortexes, no callus on the fracture site, and lysis. The patient was informed of the possible outcomes. At six months postoperatively, she showed up at the outpatient clinic with the same swollen thigh, pain on palpation, and inability to bear weight. Her radiograph at that time showed extensive lysis and displacement of the fracture site with multiple failed distal screws (Figure ).\nMultiple cystic lesions were present at and around the fracture site. The patient was taken to the operating room; a thorough debridement, sequestrectomy, and saucerization were performed. We also observed and removed multiple pearly-white cystic entities throughout the affected bone and surrounding soft tissue (Figure ). The metaphyseal-diaphyseal part of the femur was the most affected part, but the articular surfaces were found to be intact. Samples were taken for a histopathological examination. We removed all implants and irrigated the site with saline and iodine solutions. A block of custom-shaped polymethyl methacrylate (PMMA) was placed on the remaining osseous structures and the wound was closed.\nThe histopathological exam revealed cuticular membrane fragments-containing cyst wall with inflammatory reaction in the presence of giant cells (Figure ). Scolices of E. granulosus with a chain of hooklets in the germinal layer were also present. The patient was started on albendazole 15 mg/kg/day, which was to be continued for six months. The patient was treated in a multidisciplinary manner, and we consulted with the departments of Infectious Diseases and General Surgery. She underwent abdominal ultrasonography and cranial, vertebral, thoracic, and abdominal CT scans, as well as multiple radiographs to rule out possible liver or lung cysts. A consensus was reached on the fact that this was an isolated lesion of the distal femur and no cysts were detected on other sites.\nFour months later, the patient presented with discharge from the wound site. She underwent two debridement sessions, and the wound was thoroughly debrided. Suspecting a superinfection, ciprofloxacin and rifampin were added to the initial albendazole. Despite the cultures coming back negative, the triple therapy was continued for a period of three months.\nSix months after the last operation, the patient presented again with serous discharge. Her general and mental status had suffered greatly due to the continuous interventions. She was anemic and in a state of depression. This time, we opted for a more aggressive debridement. All of the remaining distal femur together with a portion of the diaphysis and the indurated surrounding soft tissue was removed. The extensor mechanism had suffered from the repeated surgeries but was intact and was thus preserved. Both the femoral and the tibial intramedullary canals were reamed open, and an arthrodesis was performed using a long knee-spanning femoral nail centrally covered with a PMMA block (Figure ). Hypertonic saline and iodine solutions were used for wound washing. The wound was closed uneventfully, and the patient was started again on albendazole. She was allowed to be mobile with a cane and weight-bearing was restricted for the first two months. She was advised at this point that because of the extensive debridement procedures and infected bone removal, an amputation might be needed as a salvage procedure in the event of a lack of healing. A definitive arthrodesis or, less likely, a condylar prosthetic was also discussed in case of healing.\nOne year after the last operation, the patient finally had a clean wound. She was able to be mobile indoors with the help of a cane and was determined to undergo a final decisive procedure. Her physical examination showed a loss of strength in her right lower extremity and an atrophic quadriceps muscle. CRP and erythrocyte sedimentation rate were normal. There was no discharge sinus. Her case was discussed with other senior surgeons and a definitive arthrodesis procedure was planned. Long radiographs showed a bone defect of 22 centimeters compared to the healthy left side, and a segmental defect-bridging intramedullary knee arthrodesis system was chosen for the procedure (Figure ). A frozen section came back clean and the arthrodesis was performed without complications. She is now in her 16th postoperative month and can be mobile with full weight-bearing. Yearly checkup visits were recommended. |
A 27-year-old male patient was referred to our pain clinic after having had orchidectomy for a left sided testicular cancer, 2 years earlier. He continued to have a persistent, severe pain in his right groin and scrotal area. The pain was continuous and dull with a heavy feeling. He reported the severity to be 8/10, on average. He described this to be a severe, burning, sharp pain, which could make him nauseous and fainting with any physical activity such as running, jumping, sexual intercourse, and physical examination. He also reported significant sensitivity and allodynia. Prior to our consultation, he was investigated with an ultrasound and CT scan on the right side. Since they showed some signs of edema and possible epididymitis, he was treated with antibiotics, without much improvement. He was also tried on nortriptyline 10 mg and (lyrica) pregabalin 150 mg BID, without much improvement. There were no other comorbidities or allergies. He was referred to us for the possibility of inguinal nerve blocks. On examination, he was anxious and quiet worried. His gait and posture were normal. His scrotal examination showed an empty scrotal sac on the left side and a highly sensitive inguinal region and scrotal sac on the right side. There were no signs of infection, swelling, or redness. There were no signs of inguinal or femoral hernia. The maximum tenderness was found to be just at the pubic tubercle and below, extending up to the whole of the right side of scrotum and also slightly over the medial side of thigh. Since the area of the lower abdomen and groin can be supplied by IL, IH, or GF nerve, we decided to perform separate diagnostic blocks to confirm the diagnosis and for a possible treatment. Initially, he underwent an ultrasound guided IL and IH nerve block, by the corresponding author, using 2 mL of 2% lidocaine and 2 mL of 0.25% bupivacaine mixed with 40 mg of depomedrol. The sensory block achieved did not cover the area of his pain. Approximately a month after that we performed an ultrasound guided GF nerve block using 2 mL of 2% lidocaine and 2 mL of 0.25% bupivacaine mixed with 40 mg of depomedrol.\nWith patient in supine position, the inguinal area and the area above the femoral vessels were uncovered and wiped with chlorohexidine solution. A high frequency, linear, high resolution probe (GE Ultrasound, LOGIQ e machine) was initially kept perpendicular to the inguinal ligament just above the femoral vessels ().\nA cephalad movement of the probe identified the iliac artery splitting into femoral and external iliac arteries. This corresponds to the level of the internal inguinal ring []. An oval structure lying medial and superficial to the femoral artery is the inguinal canal with its contents. A longitudinal view of the femoral artery is also identified at the same site (). The contents of the inguinal canal were identified clearly, with testicular vessels shown laterally and spermatic cord shown medially. We used an in-plane approach to direct the needle towards the spermatic cord to block the genital branch of the GF nerve, using a 50 mm echostim needle (Benlan, Ontario, Canada) (). Soon after the block, the patient noticed considerable improvement and tested it by jumping and running, to see if it hurts. The intensity of pain came down to 4/10, and the attacks of sharp pain became infrequent. The initial relief lasted 3 months, and he had a similar effect for the 2nd injection which lasted for 6 months. With an aim to prevent recurrence, he was tried on long acting tramadol 100 mg taken one a day. He underwent a 3rd injection after which his pain relief has continued beyond 12 months. He continues to be fully functional and is able to take part in normal physical activities. |
A 65-year-old man was referred to our hospital due to abdominal pain and vomiting. Two years ago, he had been diagnosed with gallstone ileus and underwent enterotomy of the jejunum with surgical extraction of the gallstone. The stone measured 42 × 28 mm in the computer tomography (CT) image. However, another stone remained in the gallbladder, and the patient did not undergo cholecystectomy.\nOn admission, his physical examination showed whole abdominal tenderness but no muscular defense. Laboratory data showed slightly elevated CRP level (0.83 mg/dL) and WBC count (9500/μL), but other results showed no remarkable deviations. In addition, arterial blood gas did not show acidosis. CT showed that a gallstone, which was previously confirmed, was no longer in the gallbladder but had lodged in the jejunum. The gallstone measured 32 × 28 mm in the CT image. In addition, consecutive expansion of the oral side of the intestinal tract was confirmed (Figure ). However, areas of the intestinal wall with poor contrast or ascites were not observed. The patient had moderate to severe aortic regurgitation and a 55-mm-diameter ascending aortic aneurysm. The risk of surgery was high, and we decided to perform conservative treatment first. Therefore, a nasal ileus tube was immediately inserted.\nAfter insertion of the tube, the patient's symptoms were improved. Two days after admission, he had diarrhea several times. Four days after admission, CT showed that the gallstone had passed into the rectum, which was subsequently confirmed using colonoscopy. On the same day, the gallstone was spontaneously evacuated. Analysis of the calculus showed that the component of the stone was bilirubin 52%, cholesterol 43%, and calcium fatty acid 5%, respectively, which was consistent with gallstone.\nThe presence of cholecystoduodenal fistula was suspected in the CT image at the time of admission. On the following day of stone evacuation, a fistula in the posterior wall of the duodenal bulb measuring approximately 8 mm in diameter was confirmed using gastrointestinal endoscopy. A catheter was inserted into the fistula, and a contrast medium was injected. Through the fistula, the gallbladder and bile duct were imaged, and a cholecystoduodenal fistula was diagnosed. There were no residual gallstones in the gallbladder or bile duct, and no tumor was admitted into the gallbladder (Figure ). The clinical course remained uneventful, and the patient was discharged 9 days after admission. Because there were no stones left in the gallbladder, the cholecystoduodenal fistula was not treated. |
This is a 50-year-old right-handed male, with 33-year history of T6 AIS A SCI from a gunshot wound complicated by chronic pain, left hip and knee heterotophic ossification, and a chronic dislocation of his right hip, who initially presented to the emergency room with a right shoulder mass in September 2014. While he initially noticed the mass about 2 months earlier, he presented for evaluation now because of acute onset of pain, weakness and paresthesias in the right arm. He was admitted to the general medicine service for pain management and underwent an initial work up for his right shoulder mass, including advanced imaging and a core biopsy. Physiatry was consulted due to his functional deterioration that precluded him from returning to his previous independent living arrangement. He demonstrated diffuse, mild weakness throughout the right arm that was variable and seemed to be correlated with his reported pain level, but his most consistent and weakest movement patterns were his grade 4/5 weakness in finger abduction and distal interphalangeal joint flexion. He had reduced pin prick sensation over the volar surface of digits 3–5, palm and forearm of the right arm and hand. He was not able to perform transfers to or from his manual wheelchair due to his level of pain. The magnetic resonance imaging (MRI) revealed a heterogeneously enhancing mass with a maximum diameter of 6.9 cm (Fig. ) that involved the right deltoid and pectoralis major muscles. His core biopsy demonstrated a STS that was classified as a high grade (III) spindle cell sarcoma.\nOncology recommended treatment for his STS with a course of outpatient neo-adjuvant radiation therapy followed by gross total resection with wide margins. Physiatry pre-operative consult focused heavily on functional prognostication. The patient expressed multiple times that he placed the highest priority on return to his previous modified independent living arrangement and not only survival after his STS treatment course. The patient’s personal values combined with the physiatric assessment informed the pre-surgical planning. In particular a decision was made to take a narrower surgical margin around key muscle group (pectoralis major and deltoids) in order to help maintain the man’s manual wheelchair mobility and his ability to independently transfer himself.\nHe completed neo-adjuvant radiation therapy in November of 2014 and underwent radical excision of his right shoulder mass with flap closure that December. He began intensive inpatient rehabilitation after he was given clearance to weight bear through his arm about 8 weeks later. Initially he required total assistance for most ADL’s, including transfers, manual wheelchair propulsion, dressing and toileting. Despite the extensive surgery and radiation treatments, the gentleman was able to return to a functional level, approaching his pre-morbid status (modified independence). He was successfully discharged home to live alone in his accessible apartment complex. |
A 37-year-old Caucasian male presented to an emergency department with worsening epigastric pain of 4-day duration. He stated that his pain initially began as indigestion while he was leaning forward getting out of bed and progressed to a constant aching pain with associated nausea. He stated that bending forward exacerbated the pain. He denied prandial pain, melena, hematochezia, or recent change in bowel habits. History was notable for hypertension which was being managed without need for antihypertensive medications. Surgical history was not significant for any prior abdominal surgeries. His social history was significant for tobacco abuse which consisted of a half pack of cigarettes a day for 25 years. Initial vital signs were significant for hypertension. Physical exam on presentation was significant for minimal epigastric tenderness with normoactive bowel sounds without rebound or guarding. Lab analysis was not significant for elevated lipase, liver function abnormalities, anemia, or electrolyte abnormalities. Computed tomography (CT) of the abdomen and pelvis with IV contrast was performed which showed a filling defect in the celiac artery. Due to concern for dissection, a CT angiogram of the abdomen and pelvis was performed. The CT angiogram showed a 40.1 mm dissection from the celiac origin into the hepatic artery as well as a 37.9 mm dissection from the celiac origin into the splenic artery (Figures and ). He was also found to have a beaded-appearing left main renal artery, raising concerns for fibromuscular dysplasia.\nThe patient was admitted for observation, given 1000 mL unfractionated heparin bolus, and started on an unfractionated heparin infusion. The patient was evaluated by vascular surgeons and gastroenterologists. When given the choice for stenting the lesion for symptomatic control, he elected not to have the lesion stented. Prior to discharge he was started on 81 mg aspirin daily and a follow-up appointment with repeat CT angiography was scheduled in 2 months. Follow-up CT angiogram two months after his initial diagnosis showed minimal enlargement of the dissection. At that time during his vascular surgery follow-up, the patient stated improvement in his abdominal pain. At that time he was scheduled for a 6-month appointment and follow-up CTA. |
A 32-yr-old woman was referred to our center with the inability of weight bearing, five days after primary cesarean section for her twins. She had recently given birth to a pair of healthy twins at 33 wk of pregnancy and had two other pregnancies leading to another healthy child and a miscarriage. Her body mass index was 25 kg/m2. Physical examination showed the severe limitation of motion in the right hip and moderately painful left hip. Right femoral neck fracture was clearly identified in the X-rays. In pelvic magnetic resonance imaging, fracture lines at both femoral necks along with peripheral bone edema were visualized, confirming the presence of a simultaneous fracture in the left femoral neck of the patient too. The fracture of the right femoral neck was managed by close reduction and internal fixation (CRIF) along with dynamic hip screw, while CRIF and screw were used for the fixation of left femoral neck fracture ().\nSince no history of trauma was reported by the patient, she was referred to the rheumatologist, where further investigations of the underlying cause of the fractures were performed. The positive past medical history points were repeated weekly courses of antenatal Betamethasone injections for fetal lung maturity, started from 26 wk gestation, plus daily oral 5 mg prednisolone due to the history of a previous miscarriage and complete bed rest during the whole third trimester of pregnancy.\nIn the laboratory evaluation, patient serum calcium, phosphorus, creatinine, alkaline phosphatase, parathyroid hormone and 24 hr urine calcium were normal; Serum vitamin D was 15 ng/ml, which was compatible by vitamin D deficiency. The results of bone mineral density showed a Z-score of -2.2 in L1-L4, suggesting low bone mineral density (Z score <-2). The woman was advised to discontinue breastfeeding, while her medical treatment was initiated with a daily subcutaneous injection of teriparatide (CinnoPar® 20 µg per day) along with calcium and vitamin D supplementation.\nIn the short-term follow-up, no complication was observed. Five weeks later, signs of the union were observed in the follow-up radiographs and the patient was finally able to bear weight for short periods of time on a daily bases. The patient allowed us to report her case and informed consent has been obtained in this regard. |
A 27-year-old female with the past medical history of autism presented to our facility from another facility for a higher level of care. Patient had an episode of biliary pancreatitis approximately 4 months prior to being transferred to our facility and underwent a cholecystectomy 1 month prior to presentation to our facility. Patient continued to have abdominal pain, nausea and vomiting after cholecystectomy, as such a CT of the abdomen was done and two pseudocysts were noted. One was drained by IR at the transferring facility. Patient presented with sepsis and acute kidney injury at the time of transfer to our facility with the WBC of 15,000 and 100.5 °F fever. CT of the abdomen was significant for a 5.9 cm pseudocyst vs. abscess at the tail of the pancreas. Broad spectrum antibiotics were initiated and surgery consultation was obtained. Patient was transferred to ICU for closer monitoring, and she also became hypotensive, but responded appropriately to aggressive fluid recitation. GI consultation was obtained regarding the pseudocyst.\nPatient was transported to the endoscopy suit. We performed a EUS and a pseudocyst was visualized at the tail of the pancreas measuring 69 × 53 mm. The cyst was punctured under endosonographic guidance using a 19-gauge needle. Next a 0.035 jag wire was passed under direct vision into the cavity. We then used thermal therapy to the channel using the Cooks cystotome. Next a 12 mm CRE balloon was used to dilate the tract. Finally a fully covered 10 × 40 mm was deployed. A large amount of pus was drained into the stomach. The patient was then transferred back to the ICU. Broad spectrum antibiotics and anti-fungal therapy were resumed. Within 48 h after the procedure the abdominal pain had resolved completely, WBC was trending down and patient was discharged from the ICU. Patient was discharged home 1 week after the procedure on antibiotic therapy. Two-week follow-up reveled that patient was doing well, tolerating her diet with minimal abdominal pain ().\nWe performed a EUS on patient 7 weeks after the initial procedure. The entire stomach was carefully examined; there was no evidence of a fistula or scar in the stomach. The patient had spontaneously passed the stent. The EUS revealed a 3 cm pseudocyst in the tail of the pancreas, the pseudocyst was aspirated using a 22-gauge needle and fluid was sent for culture and sensitivity. Patient remained asymptomatic. |
This study presents a 55-year-old male admitted in our center with a 1 × 1 centimeter laceration on the sacral area skin, 12 cm above the anus. He had fallen from a mulberry tree and had removed the sunken tree branch. He presented no neurological deficit or fever and had a normal sphincter function. The laceration was dirty with slow CSF leakage. The pelvic X-ray showed an area of defect in the sacral region (). The computed tomography (CT) of the spine revealed a hypodense penetrating object that was introduced from the posterior aspect of the sacrum into the dural space and then stopped in the S1 vertebral body, and the object did not penetrate the anterior cortex of the vertebral body (Figures and ).Because of the possibility of pelvic organ injury in cases of penetrating sacral injury, we consulted with an expert general surgeon. Based on normal findings on physical exam and pelvic CT scanning, our consultant surgeon assured us. Thereafter, the patient was transferred to the operating room. Under general anesthesia, the patient was operated in prone position. Muscle dissection revealed a wooden foreign body on the sacral lamina that passed into the S1 body (). First, a laminectomy of an intact superior portion of the S1 lamina was performed and the wood was released from the surrounding bone, and finally, it was pulled out. Before the wood was removed, the dural sac was mildly compressed above the level continuously, to prevent CSF from filling our surgical field. Also, a portion of the patient's clothes was removed from the vertebral body (). The bone defect was massively irrigated, and the defect of the dura was repaired with Prolene suture separately in a watertight manner. Thereafter, the Valsalva maneuver was performed and no CSF leakage was observed. Finally, the surgical wound was closed in a layer-by-layer manner. The patient was under close observation for 3 days and then discharged without any deficit or complication. The patient was visited 2 weeks later and it was observed that his wound had healed. Unfortunately, as a result of the different nationality of the patient (Afghanistan), we did not see him again, but we were informed by phone that he was still doing fine, 8 months after the procedure. |
A 42-year-old female patient with a six-year history of SLE, who had been taking hydroxychloroquine 200 mg and prednisolone 5 mg once daily, presented to the cardiology clinic with complaints of persistent palpitations and shortness of breath for the past six months. She did not experience syncope and denied chest pain. She did not have any family history of coronary artery disease, sudden cardiac death, and cardiomyopathy. On examination, she was found to be tachycardiac with a regularly irregular pulse and had no signs of heart failure. A 12-lead ECG showed tachycardia with an upright P wave in leads I and II, P wave morphology mimicking sinus P wave with a unique pattern of grouped P waves followed by a pause, which was again followed by a run of tachycardia (Figure ).\nHer echocardiogram showed normal-sized left and right ventricles with normal left ventricular ejection fraction (LVEF), no valvular abnormalities, and no pericardial effusion.\nInitially, she had been treated at her community hospital as a case of sinus tachycardia, which is commonly seen in patients with SLE. She had been started on ivabradine and bisoprolol but her condition had not improved and she had continued to have symptoms despite being administered the maximum tolerable dose of the aforementioned drugs. She had then been referred to the local electrophysiology team who diagnosed the focal atrial tachycardia and she had been started on amiodarone and later on, she had undergone direct current cardioversion. However, despite all the above treatments, she had continued to have symptoms. She eventually presented to our hospital where a 12-lead ECG was consistent with focal atrial tachycardia, likely originating from the superior region of crista terminalis or right-sided pulmonary veins (Figure ).\nShe was advised to undergo an electrophysiological (EP) study and ablation procedure because of her incessant tachycardia that did not respond to medications. She initially declined and decided to continue medical treatment. She returned after six months and reported worsening of symptoms. This time, she also reported New York Heart Association (NYHA) class II-III heart failure symptoms. Her repeat echocardiogram revealed a drop in LVEF from 60% to 25%. After extensive counseling, she finally agreed to undergo an EP study and ablation procedure.\nThe EP study was performed with the EnSite Precision™ cardiac mapping system (St. Jude Medical/Abbott Inc, St. Paul, MN). A three-dimensional local activation map was created with a high-density catheter (Livewire™ Duo-Decapolar, 2-2-2, St. Jude Medical). The mean heart rate was 132 beats/min. The tachycardia had an extremely variable cycle length (406-616 msec). Both right- and left-sided atria were mapped, with activation mapping the location of tachycardia confirmed at the superior crista terminalis region in the right atrium (Figure ).\nRadiofrequency catheter ablation at this point resulted in the termination of atrial tachycardia (Figure ).\nFollowing an ablation procedure, the patient remained well and she was discharged the next day. Her repeat echocardiogram after three months of ablation showed a remarkable improvement in the LVEF, from 30% to 60%. She remained in normal sinus rhythm and asymptomatic at the one-year follow-up (Figure ). |
A 28-year-old man was admitted to the emergency after being referred to our hospital following a motor vehicle accident 22 h earlier. He was pedestrian when a four-wheeler hit him from the back. At admission, the vitals were stable, and the right leg was shortened and cooler than the left limb. There was no external wound except for bruises over his right knee. The hip was in abduction and external rotation while the knee was sagging posteriorly. His distal pulses, including popliteal, dorsalis pedis, and posterior tibial pulses were not felt though femoral was palpable. Plain radiography showed an anterior dislocation of the right hip without any acetabular fracture but a posterior dislocation of the knee []. He also had a closed segmental fracture of radius in the opposite upper limb. The other trauma series X-ray was normal. We did an urgent color Doppler that showed complete occlusion of the knee’s popliteal artery level, and we called our vascular surgeon for help. A computed tomography angiography confirmed the Doppler findings []. His blood parameters showed an increased total leukocyte count, and the urea, creatinine, or myoglobin levels were normal. The patient was informed about the counseled about prognosis as a trial of limb salvage was attempted. Under general anesthesia, two operating teams simultaneously operated. One team lead by the vascular surgeon exposed the femoral artery and removed the Fogarty catheter’s 2 cm of the clot while another group had fixed the upper limb. Next knee spanning fixator in 30° flexion was applied with knee reduced, and finally, the hip was pulled in using the upper femoral schanz pins a for traction while the assistant gave a lateral and another counter traction. The reduction was confirmed under the image intensifier []. The patient was shifted to the intensive care unit with heparin and other anti-coagulants as we waited for progress. Unfortunately, by the next 2 days, there was gradual blackening and an increase in limb swelling extending up to the knee. The patient relatives were informed, and color doppler confirmed no revascularization. Hence, an above-knee amputation was done. |
A 69-year-old Caucasian male was admitted to the hospital due to abdominal pain of ∼20 h duration. He described the abdominal pain as ‘achy’, constant, diffuse and with no radiation. Associated nausea and similar episodes of pain over the past 2 years were noted. Patient denied vomiting, fever, weight loss or change in bowel habits or urination. Only significant past medical and surgical history being prostate cancer treated with radiation. On examination, the patient appeared in mild distress and vital signs within normal limits. Physical exam revealed a moderately distended abdomen with an initially incarcerated umbilical hernia. The hernia was able to be manually reduced, which resulted in slight decrease of pain. Hematological and biochemical studies were within normal limits, and a computed tomography (CT) scan demonstrated borderline dilated loops of proximal small bowel; however, no transition point was identified. After discussion with a radiologist, a partial small bowel obstruction secondary to possible LPDH was confirmed (Fig. ).\nThe patient was taken to the operating room within 12 h of hospital admission where a diagnostic laparoscopy was performed. While running the bowel, a hernia sac was discovered arising from a defect to the left of the fourth part of the duodenum, consistent with a LPDH (Fig. ). An aggregation of intestinal loops was herniated through the defect into the fossa of Landzert. The bowel was then easily reduced using bowel graspers, and a scar was noted to be tacking the orifice to the mesentery, trapping the first part of the jejunum. The bowel was intermittently dilated and compressed. Due to a section of reduced bowel remaining visibly dusky, a small midline laparotomy was performed to allow tactile examination of the bowel. After the mild ischemia was observed to have reverted and no further obstruction was ensured, the bowel was returned to the abdominal cavity and the LPDH orifice was closed primarily by fixing the root of the mesentery to the posterior parietal peritoneum.\nThe postoperative course was uneventful with complete resolution of abdominal pain. The patient was discharged on the seventh postoperative day, with instructions for resumption of normal daily activities. Follow-up at 1 month revealed no recurrence of clinical symptoms. |
The patient, a 4-year-old boy, was referred to the rehabilitation medicine department for evaluation of facial deformities and posture abnormalities. He was born prematurely at 35 weeks via cesarean section due to the premature rupture of fetal membranes. The mother was healthy and did not report the use of alcohol, tobacco, or drugs during pregnancy. The family history was non-contributory. At birth, it was noticed that he had a congenital cardiac defect; ventral septal defect, atrial septal defect, and patent ductus arteriosus, for which he underwent surgical treatment 6 months after birth. According to his mother's description, he was otherwise healthy with normal development. After performing a careful physical examination, several dysmorphic features were noted including asymmetrical facial skeleton, left facial weakness, and right microtia (). Additionally, uvular bifida was noticed when the inside of his mouth was examined (). His neck was relatively short, the right shoulder blade was located relatively more superior than the left, and the range of motion of the shoulder was limited during abduction (). Moreover, the right pectoralis major appeared shrunken, and thoracic asymmetry was confirmed, where the right nipple was relatively lower than the left (). In addition to these malformations, the mirror movements of the opposite hand during voluntary movements of each hand were observed. Additional neurological examination of the patient showed left facial palsy, but no ocular movement disorder was observed. Furthermore, muscular strength of the limbs was normal, and pathological reflexes and upper motor neuron signs were not observed.\nAccording to the Korean-Wechsler Intelligence Scale administered to evaluate the cognitive function of the patient, verbal intelligence quotient (IQ) index was 71, performance IQ index was 86, and the overall IQ index was 76. Cervical spine and brain magnetic resonance imaging (MRI), and cervical and thoracic computerized tomography (CT) were performed to rule out the additional CNS malformations and musculoskeletal anomalies. Cervical spine and brain MRI showed medullary neuroschisis without pontine or cerebellar hypoplasia, and there was no evidence of abnormalities of descending tracts and transpontine fibers in the basis pontis in the diffusion tensor MRI (). In the cervical spine, vertebral body fusion at the level of C3 and C4 was observed (), and cervical and thoracic CT images showed dysplasia at the posterior cervical spine (). In addition, fusion deformities of the right 2nd and 3rd rib, incomplete development of the right 1st rib were observed (), and the right scapula was elevated compared with the left (). To reveal the motor organization pattern of both hands associated with mirror movements, motor evoked potentials (MEP) study was performed with transcranial magnetic stimulation. The optimal stimulation coordinate was selected if a MEP of 50 µV or more was evoked at least 5 times from 10 stimulations of transcranial magnetic stimulation with the lowest excitation threshold. The optimal stimulation position for the left cortex was (–2,1) according to the 10–20 system, MEPs were simultaneously evoked at the right and left first dorsal interossei muscles; the threshold was 89% of the maximum stimulus intensity. Similarly, the optimal stimulation position for the right cortex was (2,1) MEPs were simultaneously evoked at bilateral first dorsal interossei muscles (). Facial motor nerve conduction studies showed a delayed latency and low amplitude of the left facial nerve compared with the right, where facial skeletal deformities were observed. |
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