The patient was a 55-year-old African American female with systemic lupus erythematous, interstitial lung disease on chronic oxygen, history of pulmonary embolism, heart failure with preserved ejection fraction, and pulmonary arterial hypertension who presented with dyspnea and orthopnea. Her home medications included hydroxychloroquine, mycophenolate mofetil, prednisone, furosemide, and spironolactone. Family history included congestive heart failure due to hypertension suffered by her sister, stroke by her father, and ovarian cancer by her mother. There was no history of congenital heart disease in the family. Social history included no tobacco abuse and occasional alcohol use.
On physical examination, her temperature was 97°F, heart rate was 79 beats per minute, blood pressure was 130/93 mmHg, respiratory rate was 20 breaths per minute, and her oxygen saturation was 95% on 4 L oxygen. Heart sounds were audible with regular rhythm and a 2/6 systolic murmur heard best at the left lower sternal border. Lung sounds demonstrated inspiratory crackles from mid-to-lower bases bilaterally.
Pertinent lab values revealed NT-proBNP of 2,356 and negative troponins. Electrocardiogram (ECG) demonstrated normal sinus rhythm with RV hypertrophy and non-specific ST-T wave abnormalities. Computed tomography (CT) angiogram of the chest demonstrated peripheral cystic lung changes bilaterally, cardiomegaly with enlargement of RV and right atrium along with straightening of interventricular septum with no evidence of pulmonary embolism, and enlargement of the pulmonary artery. The patient was started on furosemide, spironolactone, budesonide/formoterol, and methylprednisolone. Pulmonology and cardiology were consulted. ECG demonstrated normal LV systolic function with an ejection fraction of 50-55% with grade I diastolic dysfunction, severely dilated RV with decreased function, and moderate-to-severe tricuspid regurgitation. Estimated peak RV systolic pressure was 67 mmHg, reflective of severe pulmonary hypertension. Three months prior, her estimated RV systolic pressure was 39 mmHg.
The patient’s worsening dyspnea was attributed to progression of her interstitial lung disease, and the lung transplant service was consulted for further workup. The patient gradually improved during the hospital course, and she was discharged home with outpatient cardiology follow-up to perform a right and left heart catheterization to confirm the diagnosis of pulmonary hypertension and evaluation for lung transplantation.
Outpatient right heart catheterization confirmed pulmonary artery pressures of 71 mmHg. Left heart catheterization demonstrated mid single-vessel atherosclerotic disease. However, there was a coronary fistula from the first obtuse marginal branch of the left circumflex artery to the LV (Figures , ). The LV end diastolic pressure was 12 mmHg with an ejection fraction of 55% with no wall motion abnormalities. Because the patient was asymptomatic, no further intervention was performed.
Patient presented after one week with acute hypoxic respiratory failure due to multi-focal pneumonia requiring intubation and subsequently developed pulseless electrical activity cardiac arrest with unsuccessful cardiopulmonary resuscitation.


A 22-year-old gravida 2, para 1 woman at 28 3/7 weeks' gestation presented to the emergency department at La Fe University and Polythecnic Hospital (Valencia, Spain) with a 3-day history of mucus and shortness of breath. Neither fever nor cough were shown. Her partner had been admitted into the hospital 24 hours before due to pneumonia and a positive RT-PCR test for SARS-CoV-2. The pregnancy was spontaneously conceived and uneventful, with a low risk first trimester screening for trisomies 21, 18, and 13, as well as a normal fetal anatomy visualized at the 20 weeks' morphological ultrasound. Physical examination revealed a body mass index, temperature, blood pressure, respiratory rate, and hemoglobin saturation by pulse oximetry within normal limits. Blood tests showed a D-dimer level of 1680 ng/ml, and the remainder parameters were within the normal range. The patient tested positive for SARS-CoV-2 infection on RT-PCR of nasopharyngeal swabs. A chest radiograph revealed the absence of abnormal images, and the fetal ultrasound was reassuring. Hence, she was discharged and her well-being was assessed every 48-72 hours by phone calls. The symptoms of infection lasted for a total of 10 days, and the RT-PCR for SARS-CoV-2 became negative at 32 6/7 weeks. IgG and IgM antibodies were negative in maternal serum at 32 6/7 weeks.
The fetal ultrasound at 35 0/7 weeks gestation revealed an estimated fetal weight in accordance with the gestational age (2645 g) and a maximum vertical pocket of amniotic fluid of 81 mm (). The subsequent follow-up with serial ultrasounds revealed a transitory fetal skin edema (11 mm in the axial plane of the head at 36 3/7 weeks, Figures and ; and 4 mm at 37 3/7 weeks gestation), as well as polyhydramnios (maximum vertical pocket 86 mm at 36 3/7 weeks, ; and 88 mm at 37 3/7 weeks gestation). Peak systolic velocity remained within normal limits. Maternal serology was negative, and the glucose level was normal. Both developed a spontaneous resolution. IgG and IgM antibodies remained negative in maternal serum at 38 3/7 weeks. In the ultrasound at 38 3/7 weeks gestation, no fetal skin edema of the head was seen, the estimated fetal weight was 3500 g, and the maximum vertical pocket of amniotic fluid was 76 mm. The patient underwent a spontaneous vaginal delivery at 39 4/7 weeks of pregnancy. The female newborn weighted 3700 g, associated an Apgar Score 10/10, an arterial pH 7.28, and a venous pH 7.33. Neither cord blood PCR nor antibodies were assessed. Both, the mother and the newborn, were healthily discharged 48 hours after birth.


Case 1 is a 24-year-old male with a virgin abdomen who presented with a 3-day history of crampy abdominal pain and vomiting. He was haemodynamically normal and afebrile. He had tenderness in the lower abdomen and no peritonism. There was a moderate leukocytosis with a WCC of 12.7 × 109/L. An abdominal CT demonstrated distal SBO and a transition point located in the right pelvis and a small amount of free fluid (). The appendix was identified as normal. On Day 0 of his admission, he underwent a diagnostic laparoscopy. Intra-operatively, an MD was found at the site of the transition point between small bowel dilated proximally and collapsed distally (). Enteric contents were thickened raising the possibility of a faecolith. Macroscopically, the MD appeared to be normal, with no features of intussusception, volvulus or inflammation at the site. The MD was exteriorized through a mini-laparotomy and small bowel resection with a side-to-side stapled anastomosis was performed. Histopathology revealed MD with no features of inflammation or ectopic mucosa.


Case 2 is a 56-year-old male, with a virgin abdomen, who had 2 days of crampy abdominal pain, vomiting and obstipation. He was haemodynamically normal and afebrile. He had a distended abdomen with generalized tenderness. Blood tests showed an elevated lactate of 3 and WCC of 11.7 × 109. CT demonstrated distal SBO, with distension of small bowel up to 6 cm (). He underwent a diagnostic laparoscopy with an identification of MD at the transition point, subsequently exteriorized through a mini-laparotomy (). The apex of the MD was tethered to the mesentery through a band containing the diverticular blood supply. Small bowel resection and anastomosis was performed. Histopathology showed MD with acute inflammation, haemorrhage and necrosis, and no ectopic tissue.


A 39-year-old man presented at our hospital with a thyroid nodule in the neck region, which the mass had been detected during a routine medical check-up. FNA were performed twice prior to the visit to our hospital, but the results of the cytological evaluations at both instances were atypia of undetermined significance (AUS). The patient had no clinical symptoms and the laboratory results were in normal range. Ultrasonography, performed at our hospital, revealed the presence of a relatively well circumscribed mass (2.2 cm in diameter) with microcalcifications on the right lobe of the thyroid gland (Fig. a). Additionally, another FNA conducted at our hospital. The cytologic evaluation showed some follicular cells with enlarged nuclei and pale chromatin pattern. However, the cytologic findings were not sufficient to diagnose a specific tumor, thereafter we diagnosed the cytologic specimen as AUS (Fig. b). Subsequently, the patient underwent right hemithyroidectomy for diagnostic and treatment purposes.
The results of the cytology could not confirm whether the mass was benign or malignant. Hence, histologic evaluation of a frozen section using hematoxylin-eosin (H&E) staining was performed. A relatively well-circumscribed mass comprising cells that were mostly arranged in a trabecular or solid sheet-like pattern and intersected by homogenous eosinophilic material was noted (Fig. ). The tumor cells consisted of amphophilic cytoplasm and round to oval-shaped nuclei with condensed chromatin and indistinct nucleoli (Fig. b). The eosinophilic material was suspected to be amyloid deposition and the mass was diagnosed as a medullary thyroid carcinoma. Neck lymph node dissection was additionally performed due to the results of the frozen biopsy. And the patient has been warned that if the results of permanent biopsy was confirmed to medullary thyroid carcinoma as of the frozen biopsy, total thyroidectomy is necessary.
Subsequently, a permanent section obtained from the patient was evaluated. The tumor showed well margination with thin fibrous capsule (Fig. a and b). The basic growth pattern and morphology of the tumor cells were similar to those seen in the frozen section (Fig. c). Additionally, follicular growth pattern was identified in the permanent section (Fig. e). Also, infarction-like necrotic area and hemorrhage were recognized (Fig. d). Congo Red staining of the homogenous eosinophilic material failed to show the characteristic apple-green birefringence under polarized light microscopy (Fig. a). And blue staining of the homogenous material using Masson’s trichrome staining method confirmed that the homogenous eosinophilic material was fibrosis (Fig. b). Immunohistochemical staining showed diffuse immunopositivity for thyroglobulin and thyroid transcription factor-1 (TTF-1) (Fig. c and d, respectively). Conversely, no staining for synaptophysin or calcitonin, known to be expressed in medullary thyroid carcinoma, was observed (Fig. e and f, respectively). Knowing the patient’s history of multiple FNA procedures during the diagnosis, we suspected that the fibrosis, hemorrhage and necrosis was due to the injury from the FNA. Additionally, there was a needle tract-like structure in low-power field (Fig. a, arrow). Therefore, based on above findings the mass was diagnosed as follicular adenoma with extensive fibrosis. The patient is alive without recurrence or metastasis, after 3 years of follow up.


A 69-year-old woman previously treated for hypertension, dyslipidemia, and gastric ulcer in another hospital presented with sudden left-sided chest pain radiating to the left arm. Electrocardiography showed ST-segment elevation in the precordial leads, and an echocardiogram confirmed reduced left ventricular motion in the broad anterior wall. Hence, ST-elevated AMI was suspected. She was hemodynamically stable (blood pressure was 135/89 mmHg, and pulse rate was 89-/min), and she underwent an urgent coronary angiogram via the femoral artery because the radial artery was difficult to puncture due to narrowing or spasm. Urgent coronary angiography (CAG) using a 5-Fr catheter confirmed occlusion of the ostial LAD (Figure ). Because our patient had a high bleeding risk, as assessed using the PRECISE-DAPT score: advanced age and the presence of anemia, hypertension, and a previous gastric ulcer, crossover stenting to the LMT was undesirable because of prolonged DAPT duration. Therefore, we thought that DCA could reduce the plaque volume, and stent-less PCI with DCB angioplasty would prevent LMT crossover stenting. Subsequently, emergent PCI was started using an 8-Fr Launcher JCL 4.0 (Medtronic). An initial thrombectomy aspirated a little red thrombus, and successful recanalization was achieved 20 minutes after starting angiography (door-to-balloon time: 49 minutes) (Figure ). Pre-dilatation with a 2.0-mm compliant balloon was performed for achieving coronary flow improvement (Figure ). Then, IVUS (OpticrossTM, Boston Scientific, MA, USA) revealed partially attenuated plaques extending from the LAD ostium to the LMT, as expected, which suggested the need for crossover stenting to the LMT rather than to the LAD; ostial stenting was also necessary for preventing coronary adverse events in the midterm postoperative period (Figure ). Therefore, we decided to perform DCA and exchanged the guidewire to a 300-cm ABYSS DCA support wire (NIPRO, Osaka, Japan). Subsequently, a DCA catheter (Atherocut™, L-size, NIPRO, Osaka, Japan) was delivered to the culprit site (Figure ). After 4 attempts and 13 atherectomy cuts, plaque reduction was confirmed without the occurrence of the coronary slow flow phenomenon and under stable hemodynamic conditions (Figure ); this was followed by additional ballooning with a 3.5-mm scoring balloon (Lacrosse NSE, NIPRO, Osaka, Japan) (Figure ). IVUS showed no deep dissection, hematoma, or residual thrombus postdilatation with the 3.5-mm scoring balloon (Figure ), and we performed drug delivery to the culprit lesion with a 3.5/20-mm DCB (SeQuent Please, paclitaxel-coated balloon, B. Braun, Melsungen, Germany) (Figure ). Both lumen area (LA) and external elastic membrane area (EEM) were obviously enlarged at both culprit site and LAD ostium [the culprit site: LA: 2.8 mm2, 8.3 mm2, and 9.3 mm2; EEM: 16.7 mm2, 17.1 mm2, and 18.3 mm2, at the timing of before DCA, after DCA alone, and after DCA followed by DCB, respectively], [the LAD ostium: LA: 5.8 mm2, 10.3 mm2, and 12.1 mm2; EEM: 20.2 mm2, 21.9 mm2, and 22.2 mm2, at the timing of before DCA, after DCA alone, and after DCA followed by DCB, respectively]. The percent plaque area (%PA) markedly decreased after DCA followed by DCB angioplasty, from 83.0% to 49.1% at the culprit site and from 71.4% to 45.7% at the LAD ostium (Figure H and A-D). Total procedure time, radiation time, radiation dose, and amount of contrast agent including CAG were 87 minutes, 58 minutes, 1.7 Gy, and 160 mL, respectively. Maximum creatine phosphokinase and creatine phosphokinase-myocardial band levels were elevated at 1253 IU/L and 116 IU/L, respectively. The patient recovered well and was discharged on postadmission day 11 without any cardiac complications. The patient remained asymptomatic during the 9-month follow-up under 6 months of DAPT (acetylsalicylic acid 100 mg/day, prasugrel 3.75 mg/day) and only aspirin after 6 months according to Japanese guidelines, with the ability to stop DAPT if major bleeding occurred. A follow-up angiogram at 9 months revealed no restenosis (Figure ), and IVUS revealed plaque regression over 9 months (Figure ) (%PA: 47.6% vs 49.1%, follow-up vs. index PCI) and enlargement of the LA and of the EEM of the culprit site and the LAD ostium (LA: 10.1 mm2 vs 9.3 mm2, EEM: 19.2 mm2 vs. 18.3 mm2, follow-up vs. index PCI at the culprit site, respectively) (Figure ), (LA: 12.9 mm2 vs 12.1 mm2, EEM: 22.8 mm2 vs 22.2 mm2, follow-up vs index PCI at LAD ostium, respectively) (Figure ).


A 41-year-old man presented with gait disorder, hoarseness, and dysphagia. He was diagnosed with left LMS due to infarction (Figure ), and oral antiplatelet therapy was initiated. His neurological deficit almost improved except for severe dysphagia. Eleven months after onset, he underwent percutaneous endoscopic gastrostomy. Fourteen months after onset, he was transferred to the rehabilitation department of our hospital for dysphagia treatment.
Videoendoscopic examination of swallowing revealed fixed left vocal cord in the midline position, insufficient left nasopharyngeal contraction, pooling of saliva in the left pyriform sinus, and weakened left pharyngeal contraction. Videofluoroscopic examination of swallowing revealed weak pharyngeal contractility and impaired UES function. The bolus remained in the pyriform sinus and did not pass through the pharynx (Figure ). The swallowing pressure along the pharynx and UES was measured using HRM (Unisensor AG). HRM revealed weak pharyngeal constriction and strong constriction of the UES muscle zone (Figure ). We used the pharyngeal contractile integral (CI; mmHg-cm-s) to evaluate pharyngeal swallowing pressure., Five dry swallows were examined during which the patient's velopharyngeal CI (VPCI) and mesohypopharyngeal CI (MHPCI) values were 4.1 ± 2.9 and 72.0 ± 16.2, respectively (as reference values for these data, the VPCI and MHPCI values for 3-mL thickened liquids in our previous report were 124.3 ± 50.3 and 193.2 ± 34.1, respectively). Regarding the patient's UES function, the UESCI value was 713.7 ± 72.7, indicating strong contraction during swallowing.
The patient received nutritional treatment, respiratory training, and swallowing rehabilitation (ie, balloon catheter dilatation, Shaker exercise, and vacuum swallowing therapies). He was discharged 16 months after the initial onset. He currently continues the swallowing rehabilitation at home and receives treatment on an outpatient basis. This study was approved by the hospital's ethics committee.


A 78-year-old male patient with TA was referred to our hospital for the treatment of a BCA aneurysm. He had been treated for TA with 10 mg/day oral administration of prednisolone for 24 years. He was also treated for hypertension at a local hospital and had no history of cardiovascular diseases. He had moderate pulmonary emphysema due to a long history of smoking. Furthermore, though there were no obvious sequelae, he had a history of brain infarction. Computed tomography showed a BCA aneurysm measuring 32 mm in diameter with a thickened aortic wall ranging from the orifice of the BCA to the bifurcation of the right common carotid and right subclavian artery (Fig. a). Computed tomography also showed severely atherosclerotic changes in the arch vessels (Fig. b). C-reactive protein level was 0.2 mg/dl, and erythrocyte sedimentation rate was 6.0 mm/hour. Taking into consideration of the patient’s comorbidities as mentioned above, partial arch replacement with ICP was scheduled. Following median sternotomy, cardiopulmonary bypass was established with femoral artery cannulation (Fig. a) and bicaval drainage with a low flow (0.5 L/min). When the cardiopulmonary bypass got stabilized, direct cannulation of bilateral carotid arteries was added with a proximal clamp not to flush debris to the distal site (Fig. a). Total cardiopulmonary bypass was gained by additional cannulation through the ascending aorta and an 8-mm graft anastomosed to the right subclavian artery (Fig. a). The chronic inflammation changes compatible with TA, such as wall thickening and increased hardness, were observed in the ascending aorta (Fig. b). When the body temperature reached to 25 °C, lower body circulatory arrest was introduced. The orifice of the right subclavian artery was closed with a continuous 4–0 polypropylene suture through inside of the aneurysm. The orifice of arch vessels presented with severe atherosclerotic changes. Partial arch replacement with 28 mm quadrifurcated graft (Triplex®, Terumo Corporation, Tokyo, Japan) was performed with an open distal anastomosis technique. After distal anastomosis, the neck vessels including right subclavian artery, right carotid artery, and left carotid artery, were reconstructed with each branch of the artificial graft individually (Fig. c). An artificial graft anastomosed to the right subclavian artery was used for branch reconstruction (Fig. c, d). Both distal and proximal anastomosis were reinforced with Teflon felt strips at inner and outer side of the aortic wall. Weaning from cardiopulmonary bypass was uneventful, and the patient recovered without any neurological deficits. The time of operation, cardiopulmonary bypass, aortic cross-clamp, cerebral perfusion, and circulatory arrest were 442, 229, 128, 133, and 60 min, respectively. There were no significant changes in the continuous bilateral cerebral regional oxygen saturation values between the right and left side during the surgery as monitored using INVOS™ Cerebral/Somatic Oximetry Adult Sensors. Postoperative brain imaging detected no significant cerebral infarctions. Postoperative computed tomography demonstrated no anastomotic aneurysms and no abnormal flow in the aneurysm (Fig. d). Pathological results reported that the intima of the aorta had focal and raised plaques. During the perioperative period, management with additional prednisolone infusion was done, and the patient has never experienced episodes of exacerbation of inflammation. The patient remained free from any major adverse aortic and cerebrovascular events 1 year after surgery.


A 32-year-old male with no significant family history noticed a plum-sized lump on the left side of his penis in April 2010. Otherwise completely asymptomatic, he underwent an examination at a regional university hospital. Five months later, a tumorous infiltration arising probably from the urinary tube was diagnosed on CT.
Magnetic resonance imaging showed infiltration of both cavernous bodies (Fig. A,B). There was no pathologic lymphadenopathy in the pelvis or any infiltration of the bladder. The histopathology from the first penile biopsy showed a small-cell neuroendocrine carcinoma; however, that result was based on a sample obtained at a different hospital than the Sarcoma Center and the investigating pathologist did not have adequate expertise. A second histology reading was not demanded at that time. Bone scintigraphy, chest and brain CT did not confirm any metastatic disease. A total penile amputation, left side orchiectomy and perineostomy were carried out. The right testicle was transposed in the abdominal cavity. The definitive histology with a second reading confirmed an extraskeletal ES (Fig. ) arising from the penis, with high proliferation and perineural propagation. The margins of resection were positive at the symphysis and the bulb of the penis. With these findings, the patient was referred for further treatment at the national Sarcoma Center. A quick restaging was conducted including trepanobiopsy. The assessment of the primary tumor showed a t11/22 translocation, the cytogenetic assessment of the bone marrow was negative. Using a multidisciplinary approach, treatment was started with curative intent, according to the EuroEwing 99 protocol and later Ewing 2008 protocol; however, with a nonstandard initiation due to the poor primary diagnosis. After the induction chemotherapy (6 cycles of vincristine, iphosphamide, doxorubicin, etoposide) had the patient no signs of disease. Consolidation chemotherapy was administered which contained 8 cycles of vincristine, dactinomycin, iphosphamide with concomitant radiotherapy of 45 Gray (Gy) in 25 fractions and 10.8 Gy in 6 fractions, respectively. The treatment tolerance was good, toxicity predictable, especially the grade 4 hematological toxicity in the induction phase. Maximal supportive care was provided. The treatment lasted for 10 months, ending one year after the diagnosis. The patient had been undergoing permanent antidepressant therapy, mainly due to the extent of initial surgical resection. The young man had been followed-up for 29 months with no signs of the disease. In October 2013, multiple lung metastases were diagnosed and induction chemotherapy combining irinotecan/temozolomide was started. In January 2014, a metastasectomy of all detectable pulmonary metastases was performed and the patient finished the chemotherapy treatment with an irinotecan/temozolomide combination in April 2014. In May 2014, the patient underwent high-volume lung irradiation with a dose of 12x1.5 Gy. After six months, a CT scan showed a relapse of the tumor in the dorsal mediastinum. In January 2015, the second thoracotomy was indicated. Marginal (R2) resection confirmed the recurrence of ES, with a 10% cell necrosis and high mitotic activity. Subsequently, metronomic chemotherapy modified with Combined Oral Metronomic Biodifferentiating Antiangiogenic Treatment including cyclophosphamide, celecoxib, vitamin D, and fenofibrate was started.[ In March 2015, the tumor in the mediastinum was irradiated with a dose of 40 Gy, showing a regression. The next chemotherapy with an etoposide/iphosphamide combination was started for the tumor progression in December 2015. After 6 cycles of chemotherapy, another progression of the tumor was determined and the patient was switched to fourth-line chemotherapy combining gemcitabine/docetaxel, which was terminated after 4 cycles due to further tumor progression. In still good overall condition, the patient started a new treatment with a topotecan/cyclophosphamide combination. The patient died due to rapid progression 3 months later, in February 2017.


A 29-year-old primiparous woman (body mass index 27.5 kg/m2) was referred to university hospital at 34 weeks and 2 days of gestation because of a positive throat swab for severe acute respiratory syndrome coronavirus 2 on quantitative real-time polymerase chain reaction. The patient had a 4-day history of fever and dyspnea. On admission, her body temperature was 39.0 °C and saturation O2 (SpO2) was 95% (room air). A chest computed tomography (CT) scan revealed bilateral multifocal ground-glass opacities with partial consolidation, corresponding to COVID-19 pneumonia (). Laboratory data showed a white blood cell (WBC) count of 8800/μL, lymphocyte 12.4%, C-reactive protein (CRP) 3.54 mg/L, D-dimer 2.3 μg/mL (<1.0 μg/mL), and IL-6487 pg/mL (<4.0). Maternal laboratory values during the course of treatment are presented in , and the timeline of her treatment is detailed in . Ciclesonide inhalation 400 μg/day, dexamethasone 6.6 g/day, heparin sodium 10,000 U/day, and sulbactam/ampicillin 6 g/day were initiated. On the second day after admission, the patient continued to have high fever with chill and shivers, and her dyspnea worsened. SpO2 was 95% with a 1 L/min oxygen flow through nasal cannula. Remdesivir 200 mg/day and tocilizumab 8 mg/kg were administered on the next day. On the fourth day after admission (at 34 weeks and 5 days of gestation), her respiratory condition rapidly worsened, and the SpO2 was 94%–95% with a 3–4 L/min oxygen mask. Laboratory data showed WBC count of 9000/μl, lymphocyte 11.5%, CRP 10.41 mg/L, and D-dimer 1.5 μg/mL, and chest CT findings showed rapid deterioration (). The patient's respiratory failure required ventilator or extracorporeal membrane oxygenation (ECMO) management.
It was believed that delivery might decrease maternal oxygen consumption and improve lung mechanics. In addition, it would be easier to manage the patient's breathing after delivery. An emergency cesarean section was performed in the negative-pressure operating room under spinal anesthesia, and a 2565-g male infant was delivered with an Apgar score of 8/8 and pH 7.295 of the umbilical artery. The newborn was admitted to the negative-pressure room of the neonatal ICU. The patient's fever subsided, but her SpO2 remained at 94%–95%, even with a 10 L/min oxygen mask on the day after the cesarean section. The patient's respiratory condition deteriorated, and pneumonia findings on chest CT had worsened, showing rapidly increased consolidation with air bronchogram in both lung fields. The patient was subsequently intubated and put on mechanical ventilation at the ICU. The initial conditions for the mechanical ventilation were as follows: tidal volume 400 mL, respiratory rate 16 breaths/min, positive end-expiratory pressure 15 cm H2O, and fraction of inspired oxygen (FiO2) 100%. The ventilator setting and arterial blood gas analysis are shown in . The patient's respiratory condition quickly improved; she was successfully taken off mechanical ventilation 4 days after her first day of intubation and was discharged 12 days after cesarean delivery. She did not develop sepsis, renal failure, stroke, thromboembolism or cardiomyopathy during the course of treatment. There was no evidence of maternal or fetal vascular malperfusion or acute or chronic inflammatory pathology in the placenta. The infant's breathing and circulation were stable, and chest X-ray showed no abnormal findings. Nasal swabs for SARS-CoV-2 were negative at 24 and 60 h postpartum.


A 42-year-old man, previously healthy apart from substantial alcohol use, presented with weight loss and fatigue, and no recent history of either nausea, vomiting, or diarrhea. He was seen at his local clinic with a profoundly decreased level of consciousness. Retrospectively, we were able to elicit that he had experienced loss of libido for 4 months prior to his presentation. His Glasgow Coma Score (GCS) was 3/15, laboratory glucose was 1.1 mmol/L (normal 4.0–5.4 mmol/L), and insulin level 1.3 mIU/L (normal 2.6–24.9 mIU/L), but no blood was taken to measure C-peptide, sulfonylurea, or IGF-I levels. He received two doses of 50 mL 50% dextrose intravenously, which restored his blood glucose to 12.8 mmol/L, with a marginal improvement in his level of consciousness.
A computed tomography (CT) scan of his brain revealed no intracranial pathology. His initial biochemistry revealed a serum sodium level of 131 mmol/L (normal 136–145 mmol/L), potassium 2.3 mmol/L (normal 3.5–5.1 mmol/L), creatinine 81 µmol/L (normal 64.0–104.0 µmol/L), and ionized calcium of 1.22 mmol/L (normal 1.05–1.30 mmol/L). At the time his glucose level was 12.8 mmol/L, a random cortisol level was 6 nmol/L (normal 68 nmol/L–327 nmol/L) and simultaneous plasma adrenocorticotropic hormone (ACTH) was 253 pmol/L (normal 1.6–13.9 pmol/L), suggestive of primary hypoadrenalism, and therefore an ACTH stimulation test was not deemed necessary. His dehydroepiandrosterone sulfate of 0.9 µmol/L (normal 2.4–11.6 µmol/L) was also corroborative of primary adrenal insufficiency. In addition, thyroid functions revealed a TSH of 16.61 mIU/L (normal 0.27–4.20 mIU/L), and free T4 7.6 pmol/L (normal 12.0–22.0 pmol/L) and antithyroid peroxidase antibodies were 10 U/mL (normal <34 U/mL). His arterial blood gas demonstrated a pH of 7.22 (acidotic), PO2 13.8 KPa, PCO2 4.82 KPa, standard HCO3 15.1 mmol/L, and base excess –12.3 mmol/L (normal –2 to +2 mmol/L), in keeping with metabolic acidosis. He was intubated for airway protection and referred to a tertiary hospital for intensive care unit management.
On examination, he was underweight but not emaciated, blood pressure was 103/62 mmHg, and he had a regular pulse rate of 98 beats per minute. He appeared clinically dehydrated and was noted to have some mild increased skin pigmentation of his palmar creases and extensor surfaces of his elbows. Cardiovascular examination was normal. Chest examination while intubated revealed no wheezes or crepitations. Examination of the abdomen was normal, and neurological review demonstrated no lateralizing signs.
In addition to mechanical ventilation, he received intravenous potassium chloride and stress doses of hydrocortisone 100 mg six hourly and thiamine. He was extubated after 24 hours of ventilation.
On normalization of his serum potassium to 3.9 mmol/L (normal 3.5–5.1 mmol/L), his serum aldosterone was less than 52.9 pmol/L (supine 49.0–643.0 pmol/L). While convalescing on a general medical ward, taking 20 mg of hydrocortisone per day and while normokalemic with potassium supplementation, investigation for his hypokalemia revealed a urinary potassium of 17.4 mmol/L (normal less than 20 mmol/L), urinary sodium of 113 mmol/L (normal less than 20 mmol/L), transtubular potassium gradient (TTKG) of 8, and urine potassium-to-creatinine [K/Cr] ratio of 3.9 mmol/mmol.
He was started on thyroxine replacement. As his blood pressure and serum potassium were normal, a decision was made to withhold fludrocortisone and stop oral potassium replacement. Investigation for renal tubular acidosis, after extubation, showed a normal arterial blood pH of 7.39 and urine pH of 6. Moreover, Fanconi syndrome was negative, including absent glycosuria, absent aminoaciduria, and urine phosphate-to-creatinine ratio of 1.22 (normal 0.20–3.14) mmol/mmol creatinine. Investigations for an underlying cause of primary adrenal insufficiency, including measurement of adrenocortical antibodies and very-long-chain fatty acids, and an intensive screen for tuberculosis, were negative. CT scan of the adrenal glands revealed a normal-size right adrenal gland, while the left adrenal gland revealed linear calcification of 827 Hounsfield units (HU), suggestive of previous tuberculosis infection. Two separate human immunodeficiency virus (HIV) tests were conducted using enzyme-linked immunosorbent assay (ELISA), both of which were negative. Considering his complaint of reduced libido, we investigated his early-morning testosterone concentrations, which were 7.7 nmol/L (normal 8.6–29.0 nmol/L), follicle-stimulating hormone (FSH) 1.8 IU/L (normal 1.5–12.4 IU/L), and luteinizing hormone (LH) 6.2 IU/L (normal 1.7–8.6 IU/L), indicating hypogonadotropic hypogonadism.
Ten days after admission, the patient was discharged from hospital well, on hydrocortisone 20 mg per day and thyroxine, as the abnormal thyroid functions persisted despite hydrocortisone. At follow-up, his oral potassium was tapered and stopped after 6 weeks, but he never required mineralocorticoid or testosterone replacement as the latter improved. On follow-up, 3 months after discharge, his urinary potassium level was 51.6 mmol/L (normal less than 20 mmol/L) and urinary Na 58 mmol/L (normal less than 20 mmol/L), suggesting that he had ongoing renal losses of potassium and sodium.


A 67-year-old male with history of lung adenocarcinoma and brain metastasis suffered from intermittent epigastric pain for several days before presentation. A sudden onset of sharp left chest pain was noted after several episodes of cough. He was brought to the Emergency Department of Changhua Christian Hospital, Taiwan. Initial physical examinations revealed sinus tachycardia without abnormal rhythm and abdominal tenderness with rebound pain. Acute myocardial infarction was initially excluded based on the electrocardiogram findings (absence of diagnostic wave change) and laboratory data (troponin I level, < 0.017 ng/mL; creatine kinase-MB mass, 0.87 ng/mL; both were within the normal range). Chest and abdominal computed tomography findings revealed left-sided hydeopneumothroax and mural wall swelling of the lower third of the esophagus with suspicion of perforation. Furthermore, distended stomach and pneumoperitoneum were also noted with a soft tissue around the pyloric region (Fig. a-b). A tentative diagnosis of an esophageal rupture with suspicion of peptic ulcer perforation was made. Emergent left side thoracoscopy was performed which showed turbid pleural fluid with food content. A 3-cm long rupture over the lower third of the esophagus was also observed. After evacuating the turbid pleural effusion and irrigating the pleural space, the muscle layer and mucosal layer of the esophagus were identified and repaired layer by layer thoracoscopically (Fig. c). No air leakage was found during the under-water test. There were two chest drains placed. Thereafter, exploratory laparotomy was performed, and a 2-cm perforated gastric ulcer over the antrum was discovered. Gastrorrhaphy and feeding jejunostomy were performed. The patient was admitted to the intensive care unit for further sepsis control and nutritional support. Enteral nutrition was resumed via jejunostomy on postoperative day 2. Esophagography was achieved on postoperative day 10 without evidence of leakage. Oral intake was then resumed, and the patient was uneventfully discharged on postoperative day 14.


A 62-year-old male with history of left middle cerebral artery infarction and right hemiparesis presented with hematemesis following dyspnea, fever, and chills. He was rushed to the emergency department, and his laboratory data revealed leukocytosis with left shift (white blood cell count, 10,690/uL; neutrophil count, 68.0%) and an elevated procalcitonin level of 14.13 ng/mL. Chest computed tomography revealed a right-sided hydropneumothorax with mediastinal fluid accumulation around the lower thoracic esophagus. Additionally, pneumoperitoneum was also noted (Fig. a-b). An upper gastrointestinal endoscopy performed in the operation theater showed a 2-cm long transmural rupture of the lower third esophagus. Right side thoracoscopy was then performed, and the pleural space was decontaminated. A layer-by-layer thoracoscopic primary repair of the esophageal rupture was done. After turning the patient to a supine position, laparoscopy was performed which showed a 2 × 1.5-cm perforation at the first portion of the duodenum (Fig. c). Laparoscopic duodenorrhaphy, decompressive gastrostomy, and feeding jejunostomy were then performed after the removal of contaminated ascites. After operation, nutrition support was kept via feeding jejunostomy, and infection was controlled with antibiotics. Esophagography performed on postoperative day 11 showed no evidence of leakage. After initiating oral intake without any specific discomfort, the patient was discharged on postoperative day 16.


A 32-year-old pregnant woman, G4 P2102, attended her first visit of antenatal care at 23+3 weeks of gestation. Ultrasound examination for anomaly screening demonstrated cardiac defects, including double-outlet right ventricle: DORV-TOF type, with severe pulmonary stenosis () and left superior vena cava. Detailed ultrasound showed no associated abnormalities. Fetal biometry was consistent with gestational age except abdominal circumference and estimated fetal weight, which were relatively low (at 10th percentile), reflecting some degree of growth restriction. However, detailed ultrasound on the follow-up scans at 28 weeks of gestation showed subtle abnormalities, including malrotation of both kidneys, the hilum or renal pelvis facing posteriorly to the abdominal wall (). Furthermore, 3D-ultrasound revealed abnormal external ear structure (markedly prominent crus of anti-helix) (). Non-stress tests (NST) showed spontaneous fetal heart rate (FHR) accelerations (normal reactive tests) (23+3 weeks). Interestingly, the fetus showed persistent non-response to acoustic stimulation tests at 26, 30, 32, 36 and 38 weeks (no FHR accelerations as well as no quickening perceived by ultrasound) (), probably reflective of auditory dysfunction. Based on the findings of heart defect, ear defect, renal defect and growth restriction, several differential diagnoses were listed, including CHARGE syndrome. Theoretically, fetal blood sampling for molecular genetic tests should be performed. Nevertheless, since no lethal condition was identified and the couple wanted to continue pregnancy regardless of investigation results, prenatal invasive diagnosis was avoided, and we waited for postnatal work-up instead. She had no significant underlying disease and no familial history of hereditary diseases. Her pregnancy was uneventful except that she developed gestational diabetes (GDM) at 28 weeks of gestation, which was well-controlled with diabetic diet.
At 38+4 weeks, she had vaginal delivery, giving birth to a female baby, with birthweight of 2580 g (9th percentile of WHO growth chart); APGAR score 8 and 9 at 1 and 5 min, respectively, with excessive secretion in both nostrils. The left ear showed a clipped off helix (). Otoscopic examination showed bilateral ear canal stenosis with cerumen. Funduscopic examination revealed optic disc coloboma on the right eye and choroidal coloboma on the left eye (). Abdominal ultrasound revealed mal-rotation of both kidneys but with normal size and echogenicity of the renal parenchyma; no dilatation of the pelvicalyceal system was observed. Neonatal echocardiography confirmed the prenatal findings. Because of inability to pass nasogastric tube, bilateral choanal atresia was suspected and confirmed by CT scans of the head ().
Prenatally, this case was suspected of CHARGE syndrome based on the findings of cardiac defects, ear abnormalities, growth restriction and renal abnormality. The diagnosis was postnatally confirmed by additional specific findings of coloboma, choanal atresia and ear canal stenosis. Finally, the diagnosis was confirmed by molecular genetic testing, as follows:
Molecular genetic study: Trio WES-analysis was performed for the neonate and her parents. Genomic DNA was isolated from peripheral blood leukocytes. The DNA samples were enriched by SureSelect Human All Exon V7 kit, Agilent: Santa Clara, CA, USA and sequenced onto Illumina HiSeq 4000 Sequencer Illumina: San Diego, CA, USA. A novel de novo heterozygous frameshift c.3506_3509dup variant (chr8:61741348 C > CTAAA, p.K1170Nfs*39) in the CHD7 gene was identified in the neonate (), but not her parents. This variant has not been identified in the ExAc, Gnomad, Clinvar and the in-house 3206 Thai Exome databases.
The baby underwent a left modified Blalock–Taussig shunt (MBTS) as palliative surgery at one month of age, with a successful outcome resulting in a well-oxygenated condition (oxygen saturation 98–100%). At the time of writing this report, the definitive corrective surgery has not been performed. The neonate was taken care of and followed-up by ophthalmologists and ENT specialists (for auditory brainstem response test).


A 54-year-old, 80-kg Asian man was scheduled for left thyroid lobectomy for a thyroid nodule. He was clinically euthyroid with normal thyroid function test results. The patient's medical history was insignificant except for BPH. His New York Heart Association classification was class I. For BPH, he was being treated with tamsulosin 0.4 mg at night for one year. He was a smoker and had been smoking approximately 20 cigarettes a day for the past 24 years. He had no known history of allergy to any drug. He consented to general anesthesia for thyroid lobectomy.
Before induction of anesthesia, the patient's recorded blood pressure (BP) was 137/88 mm Hg, heart rate was 72 bpm, and temperature was 36.5°C. Cefazolin 1 g was administered intravenously (IV) in the operating room without any adverse effects. After placement of routine monitors and preoxygenation, general anesthesia was induced with sodium thiopentone 5 mg/kg, pethidine 1 mg/kg and atracurium 0.5 mg/kg, and the patient's trachea was intubated with a reinforced endotracheal tube without any difficulty. The lungs were ventilated with a controlled mode of ventilation with O2/N2O and 1% isoflurane. The two BP readings taken after induction were 110/70 and 100/64 mm Hg. After laryngoscopy, the patient's maximum BP reading was 162/96 mm Hg, and his heart rate was 104 beats/min. Within 10 minutes, the patient's BP decreased to 75/45 mm Hg despite a rapid infusion of 1 L of lactated Ringer's solution. His heart rate remained at 70 bpm, and his oxygen saturation was between 98% and 99%. End-tidal carbon dioxide remained between 33 and 36 mm Hg, with a respiratory rate of 10 breaths/min. Ephedrine 30 mg in divided IV doses only transiently improved the hypotension (BP to 85/40 mm Hg), and frequent phenylephrine boluses (100 μg/bolus) were required to maintain systolic BP above 90 mm Hg. The patient's skin was warm but not diaphoretic. Skin erythema, urticaria, bronchospasm, facial edema and other features of a potential anaphylaxis or anaphylactoid reaction were absent. No ischemic changes were observed on a three-lead electrocardiogram (ECG). General anesthesia was maintained with O2/N2O and isoflurane minimum alveolar concentration (MAC) of 0.8%. At this point, the differential diagnosis of hypotension was primarily directed to deep anesthesia or hypovolemia. While these were being addressed, the surgeon was allowed to proceed. There was continuing hypotension requiring phenylephrine. At the time of surgical incision, the patient moved slightly in response to surgical stimulation. The total duration of surgery was 180 minutes (three hours), and the total estimated blood loss was 70 mL. A total of 1200 μg of phenylephrine was required to maintain the systolic a BP above 90 mm Hg throughout surgery.
At the end of surgery, isoflurane was turned off, and the patient's BP increased to 110/70 mm Hg with a heart rate of 75 bpm. The patient completely recovered from neuromuscular blockade after administration of 0.2 mg of glycopyrrolate and 2.5 mg of neostigmine. The patient's trachea was then extubated, and he was shifted to the postanesthesia care unit. He remained in the recovery room for two hours and remained hemodynamically stable with BP in the range of 120/75 to 140/80 mm Hg and heart rate between 70 and 76 bpm.


A 12-year-old boy was seen at our outpatient clinic because of a bulging mass with loss of overlying hair on the posterior aspect of his scalp. The mass was first observed at the age of 2 years, and it had slowly increased in size since then. Physical examination revealed a soft 4 × 4 cm skin colored mass with mild tenderness on the occipital scalp []. There was loss of hair on the skin overlying the mass. The mass had a ‘bag of worms’ feel on palpation. Neither were there any changes in the bone around the margins of the swollen mass [] nor was there any bruit over the mass. The rest of the cutaneous examination was unremarkable and failed to reveal any cafe-au-lait spots or axillary/inguinal freckling. An ophthalmologic opinion was also sought for which did not reveal any abnormality.
At the time, the tentative differential diagnoses included Alopecia Areata, hemangioma, lipoma, and eccrine spiradenoma.
Plain skiagram of the skull showed no bony erosion. An ultrasound was performed, which showed a hypoechoic mass suggestive of hemangioma. A computed tomography (CT) scan performed subsequently failed to reveal any intracranial extension. Rest of the laboratory data was normal.
The patient subsequently underwent surgery for wide excision of the mass. Histologic examination of the excised specimen [] showed a typical presentation of diffuse neurofibroma involving the entire dermis and infiltrating the subcutaneous fat. The lesion was characterized by a proliferation of spindle cells that contained elongated ovoid to curved nuclei, and these cells were surrounded by a matrix with wire like collagenous fibers. Few ecstatic blood vessels were also found [].
The tumor was completely excised. No recurrence was observed during the first year of follow-up. The patient’s physical and mental development showed no abnormalities, and a diagnosis of Von Recklinghausen’s neurofibromatosis could not be established.


A 46-year-old old lady presented with newly diagnosed hypercalcemia and elevated parathyroid hormone levels came to our department for a parathyroid scan.
Calcium-14 Phosphorus: 2.6 (2.4–4.5) Alkaline phosphatase level: 46 (35–104) PTH: 138 pg/ml (15–65) Vitamin D: 27.55 ng/ml (20–40) Renal function tests: Normal Ultrasonography (USG): MNG. ? Inferior lobulation of enlarged right lobe of thyroid. ??Parathyroid adenoma CT: Multinodular goiter
Parathyroid scintigraphy with SPECT-CT: Anterior planar images of the neck and chest were acquired for 30 min, at 10 min and 120 min after the intravenous injection of 740 MBq 99mTc-MIBI, using a large-field-of-view gamma camera equipped with a parallel-hole collimator. Immediately after the first planar image, a SPECT study was acquired using 60 projections of 30 s each over a 180° anterior arc from the right lateral to the left lateral position in a 128×128 matrix at 3° angular steps. Transaxial, coronal and sagittal slices 1-pixel-thick were reconstructed using a third-order Metz filter set to 8-mm full width at half maximum. shows an abnormal 99mTc-MIBI uptake in the region of the lower pole of the right lobe of the thyroid. illustrates the localization of the abnormal 99m-Tc MIBI uptake in the same patient to be intrathyroidal.
The patient elected to undergo surgery. The right side was investigated first, demonstrating no orthotopic normal or adenomatous inferior parathyroid gland and a normal orthotopic superior parathyroid gland. Palpation of the inferior pole of the thyroid confirmed a nodule, potentially representing intrathyroidal parathyroid adenoma, embedded in the thyroid tissue. The patient underwent right lobectomy
Postop biopsy: Parathyroid adenoma Postop calcium: Normal, 10.3 The postoperative course was uneventful.


An 11-year-old boy was admitted to our clinic with the complaint of a lesion of 15-month duration on the hairy scalp. Physical examination revealed an erythematous, slightly pigmented plaque lesion approximately 3 × 4 cm in diameter on the posterior scalp (). Excisional biopsy was done with a 10 mm surgical margin. On histopathologic examination, there were typical nodular basal cell carcinoma tumor islands with ulceration localized in the dermis. Asymmetrically growing solid nodules in the dermis with connections to epidermis at some zones were seen. Frequent mitoses were noted as well. Retraction artefact between tumor nodules and the stroma were visualized. Adenoid differentiation and pigmentation were observed, as well (). No recurrence was noted during the sixteen-month followup of the patient. No other signs or symptoms of syndromes have been encountered in the patient and his family. Any particular risk factors for BCC development have not been documented.


A 23-year-old female was referred from the Urology clinic as a case of solitary pelvic kidney and azotemia. She had history of primary amenorrhea, recurrent urinary tract infections and recent onset hypertension. Examination revealed her to be of average built (body weight of 62 kg and height of 168cms), euvolemic, with no pallor, BP- 110/70 mm of Hg (on medication), and no gross skeletal abnormality or hearing defects. Her cardiovascular, abdominal and respiratory examination was normal. She had feminine body features. Her gynecological examination showed a small vaginal pouch of about 2.5 cm ending blindly. There was no family history of any gynecological or renal abnormalities.
Investigations showed hemoglobin 117g/dL, WBC count- 7.5×103/dmm3, platelets - 3.55×109/mm3, urea-4.3 mmol/L, Creatinine 2mg/dl, albumin - 3.5g/dL, Calcium 9mg/dl, phosphorus- 6.5 mg/dL, sodium - 140 mmol/L, potassium- 4.8 mmol/L, PTH- 29 pmol/L. Urine examination showed 2+ proteins, leucocytes 4-6/HPF, no RBCs, 24-hour urinary protein of 1.2 gram and a creatinine clearance of 38ml/min (24-hour urinary creatinine estimation). Her hormonal profile was within normal limits. Detailed ultrasound revealed left kidney not localized, a 9.5 cm right pelvic kidney, with increased echotexture, loss of cortico-medullary differentiation, few cortical cysts and mild hydronephrosis Uterus was not seen but both ovaries were present []. A Magnetic Resonance Imaging (MRI) scan revealed solitary pelvic kidney in relation to right internal iliac vessels and gynecological abnormalities were confirmed [Figures –]. Tc 99 DMSA scan showed a poorly outlined right pelvic kidney showing nonhomogenous tracer uptake by functional parenchymal tissue. Left kidney was not visualized. A cystoscopy done by urologists prior to her referral had shown only right ureteric orifice and absent left ureteric orifice. Genetic study revealed 46 XX karyotype.
The features are consistent with type 2 MRKH syndrome with chronic kidney disease (CKD)-stage 3. Her CKD is likely because of chronic pyelonephritis secondary to anomalies of her urinary tract. She was counseled and is on regular follow-up in renal and gynecological clinics. Because of the social stigma she refused to be assessed by a plastic surgeon as was advised by her gynecologist. She is on conservative measures for her CKD but her follow-up in renal clinic is erratic.


A 64 year old female of Chinese origin underwent uncomplicated mechanical mitral valve replacement in 2002. She was reviewed six weeks following discharge. The sternal wound was healing slowly, but there were no signs of infection.
Past surgical history included sterilisation in 1962, haemorrhoidectomy in 1983 and hysterectomy for fibroids in 1985. None of these operations had resulted in scar hypertrophy. No other significant medical history was present.
She made an uncomplicated recovery from her mitral valve surgery and she was discharged back to her general practitioner (GP). In the next 9 months, she noticed increasing pain and thickness of the sternotomy and previous chest drain scars. She was initially treated by her GP with local anti-inflammatory agents with no noticeable improvement. She searched for other treatment options on the internet and chose to use Cica-Care™ (Smith & Nephew™), a silicone gel sheet for six months with little relief. At that stage her GP referred her to a plastic surgeon and a pain therapist who treated her with local steroid and lidocaine injections for approximately 4 years with no improvement. Eventually she was seen by her cardiologist who referred her back to our cardiothoracic unit.
Her clinical examination confirmed a 12 cm long keloid scar at the site of the sternotomy and two other keloid scars inferiorly at the site of previous chest drains and pacing wires. (Figure )
These were extremely tender on light palpation, particularly over the site of the sternal wires. None of the wires were palpable. Following discussion on treatment options she agreed to have surgical excision of the scar and primary closure.
In November 2008, the patient underwent excision of the keloid scar with removal of the sternal wires and reconstruction of the wound with three layers of suture (1 Dexon™ Covidien UK™) under general anaesthetic. Mobilisation of the subcutaneous tissues was required for approximation of the skin edges. Skin was closed with Prolene™ (Ethicon™). Sutures were removed after ten days and a pressure dressing was applied. We were not sure if this patient was allergic to the Monocryl™ and we therefore chose to use a removable material such as Prolene™. The histology of the keloid scar did not show evidence of malignancy.
The hypertrophied scar at the pacing wire site was deliberately left untouched in order to compare any future scar formation. (Figure ) She was discharged home three days later. (Figure )
The patient was reviewed in the outpatients clinic six weeks later. The wound was healing well with minimal tenderness over the scar sites (Figure ). Nine months after excision there was evidence of recurrence of her scar, however this was of a lesser degree (Figure ). The patient felt that pain related to her scar had markedly improved and was satisfied with the overall result.


A 57-year-old Taiwanese man presented to our facility with a one-month history of progressive difficulty in swallowing, particularly liquids. In addition, a body weight loss of 10 kg was noted. His medical history included chronic sinusitis and chronic serous otitis media. A chest X-ray revealed incidental right middle lobe collapse when he was admitted for surgical treatment for chronic paranasal sinusitis. Obstructive pneumonitis with a right lower lung field mass lesion was suspected. A physical examination revealed left ptosis, dysarthria, and mild bilateral shoulder girdle weakness without a definite diurnal change. He was subsequently diagnosed as having MG based upon the decremental response to repetitive stimulation on electrophysiological testing in association with a positive anti-acetylcholine receptor antibody test. The MG stage was assessed as grade IIA according to the Osserman classification at that time.
A clinical swallowing evaluation by a speech/language pathologist showed our patient had mild difficulty in oral preparation and transport during trial swallows of food and liquid. A delay in his swallow reflex trigger without fatigability was also noted. The strength of his oral muscles, head rotators, extensors and flexors were assessed as grade four by manual muscle testing. We accordingly conducted a videofluoroscopic study with three mL boluses of thin liquid, thick liquid, and pudding administration. In the oral phase of swallowing, it was noted our patient had poor oral holding and tongue movement. During the pharyngeal phase, we observed our patient had a delayed swallowing reflex (onset of swallow reflex trigger: 2.5 seconds) with incomplete laryngeal closure, and poor pharyngeal wall motility. Silent aspiration was observed on administration of three mL pudding. Residual food was pooled in the bilateral pyriform sinuses and valleculae after swallowing three consistencies of sample material. After repeated consecutive swallows, the residues in the bilateral pyriform sinuses increased in volume, particularly on the left side (Figure and ). However, the residues decreased in volume when his head was turned to the left side and there was no evidence of silent aspiration (Figure ). In contrast, chin tuck or head tilting to the right side were ineffective measures for reducing the pharyngeal stasis. Our patient was instructed on the correct postural technique to adopt (head turning to the left side) in order to improve his swallowing safety and efficiency. Due to MG crisis-related respiratory failure, our patient was admitted to an intensive care unit and underwent thymomectomy and tracheostomy. After seven plasma exchanges were performed, his condition improved and his tracheostomy tube was successfully removed. His swallowing ability was evaluated again after he was transferred to an ordinary ward. Mildly delayed swallow reflex (onset of swallow reflex trigger: 1.5 seconds) and occasional choking were noted only when he consumed about 10 mL of thin liquid from a cup. Following his discharge, he was able to eat food of ordinary consistency.


A 25-year-old paraplegic male patient was referred with grade IV sacral pressure sore []. The sacral defect was reconstructed using a superior gluteal artery perforator-based propeller flap []. There were no post-operative complications [].


A 28-year-old paraplegic female patient was referred with grade IV ischial presssure sore on the right side []. The patient was previously operated for contralateral ischial pressure sore with posterior thigh flap. Inferior gluteal artery perforator-based propeller flap was used for the reconstruction. Flap healed without any complication [].


A 30-year-old female patient with intergluteal pilonidal sinus [] was managed by wound debridement and the defect was reconstructed with superior gluteal artery perforator-based propeller flap. Flap recovery was uneventful [].


A 13-year-old female Thomson's gazelle (Eudorcas thomsoni) from a zoo was presented to Louisiana Animal Disease Diagnostic Laboratory, Louisiana State University (LSU) (Baton Rouge, LA, USA) for necropsy examination. The animal was found dead and had a history of severe parasitism. Postmortem examination revealed sparse body fat store in the gazelle. There was bilateral enlargement of thyroid glands (right: 6 × 3 × 2 cm; left: 2 × 2 × 1 cm), and the cut surfaces were pale tan with multifocal red areas. The liver was firm, rounded, and the cut surface was mottled tan and red (). There was marked serous atrophy of fat in the bone marrow and epicardial fat. Histologically, multifocal to coalescing areas of many hepatocytes contained large cytoplasmic vacuoles filled with pale eosinophilic homogeneous material and rare fine basophilic granules with hematoxylin and eosin stain (). Periportal areas had moderately increased fibrous connective tissue with marked biliary hyperplasia, mild oval cell hyperplasia, and moderate infiltrates of predominant lymphocytes and plasma cells with occasional neutrophils. Multifocal small aggregates of neutrophils occasionally admixed with plasma cells, lymphocytes, and rare degenerative hepatocytes were present in the hepatic parenchyma. There were multifocal areas of hepatocellular nodular regeneration. The histological findings in liver were diagnosed as chronic multifocal to coalescing severe hepatocellular vacuolation with unknown material and mineral, and multifocal moderate lymphoplasmacytic cholangiohepatitis, bridging portal fibrosis and biliary hyperplasia. There was no evidence of hepatocellular necrosis. The histopathological findings in liver were interpreted as severe chronic hepatic disease. The majority of intracytoplasmic inclusions failed to stain for fat with oil red O (), glycogen with periodic acid-Schiff stain (with and without diastase digestion) (), and acid mucopolysaccharides. The hepatocytes stained strongly with von Kossa staining consistent with calcium (mineral), mainly in the intracytoplasmic inclusions (). Additional stains including Hall's for bile pigment, fibrinogen, phosphotungstic acid hematoxylin for fibrin, Prussian blue for iron, auramine-rhodamine for acid fast organisms, Mayer's mucicarmine for polysaccharide, alcian blue (pH 2.5) for mucin, and congo red for amyloid failed to stain the pale eosinophilic cytoplasmic inclusions. Additionally, immunohistochemical staining for fibrinogen, kappa, and lambda light chains yielded negative results. An undetermined storage disease was considered as a differential in the tissue sections of liver. The enlarged thyroid glands were diagnosed as thyroid hyperplasia. No other significant microscopic findings were seen in this animal. Fecal floatation revealed moderate mixed parasitic burden including Trichostrongylus spp., Haemonchus spp., and Nematodirus spp. parasites eggs.
For transmission electron microscopic examination, 1-mm3 fragments of liver were fixed in 3% glutaraldehyde, postfixed in 1% osmium tetroxide, dehydrated in ethanol, and embedded in liquid epoxy resin. The ultrathin sections (70–90 nm) were stained with lead citrate and uranyl acetate and examined with an electron microscope (JEOL JEM-1011). Ultrastructurally, nonmembrane bound, irregular shaped crystalline inclusions were seen in the cytoplasmic vacuoles of hepatocytes (). Lipid with/without mineral deposits was noted within the vacuoles (). Scanning electron microscopy and energy dispersive X-rays analyses (SEM/EDXA) on the liver provided evidence of accumulations of calcium, oxygen, phosphorus, and smaller amounts of magnesium and sodium. Infrared spectroscopy showed the hepatocellular material consistent with protein and carbohydrate.


A healthy 27-year-old man with normal phenotype was referred for the evaluation of infertility. In his physical examination, right testis was 10 cc, left testis was 8 cc with grade 1 varicocele. Vasa deferentia were palpable on both sides. Consecutive two semen analyses of the patient revealed azoospermia with normal ejaculate volume. His serum FSH was 24.29 mIU/mL (1.6–12.4 mIU/mL) and total testosterone was 495 ng/dL (260–1600 ng/dL). The patient with the diagnosis of nonobstructive azoospermia (NOA) had a history of allogeneic bone marrow transplantation (BMT) from his sister due to chronic myeloid leukemia (CML). Karyotyping and Y chromosome microdeletion analysis, which are necessary for the evaluation of NOA, were done with peripheral blood leucocytes and buccal mucosa samples because of the probable change in peripheral blood leucocytes after allogeneic bone marrow transplantation. There was no family history of infertility.
The patient, who had the diagnosis of CML with positive Philadelphia (Ph) translocation in February 1999, was treated with Hydroxyurea 500 mg/day for two years. The patient was conditioned with cyclophosphamide 60 mg/kg for two days and TBI with a total dose of 12 Gy in 3 fractionated doses before BMT. In 2001, allogeneic BMT was performed. After allogeneic BMT, the patient was treated with 26 mg methotrexate and 30 gr IVIG. In cytogenetic analysis performed on cultured cells from bone marrow material before allogeneic BMT, no metaphases were examined. After BMT, cytogenetic analysis revealed 6 metaphases examined by trypsin GTG banding, and 4 of them had 46,XX chromosomal constitution.


AA is a 7-year-old boy delivered by spontaneous vaginal delivery in a primary health care center with uneventful pregnancy. While being investigated for jaundice in the 2nd day of life, he was discovered to have high cholesterol >500 mg/dL (>5.68 mmol/L, normal <4.40 mmol/L), low hemoglobin (HB) 6.7 g/dL (normal range 13.6–19.6 g/dL), and normal serum bilirubin and platelet count. The lipid profile was repeated. The laboratory work showed a very thick blood sample that was hyperlipidemic (). The repeated lipid profile showed (laboratory method used: AEROSET system and ARCHITECT c8000 system) serum cholesterol 7.4 mmol/L (normal <4.40 mmol/L), high density lipoprotein (HDL) 1.10 mmol/L (normal >1.55 mmol/L), and triglyceride (TG) 80 mmol/L (normal <1.70 mmol/L). Based on this very abnormal lipid profile compared to his age, the primary healthcare facility started him on lipid lowering agents: gemfibrozil (lipoid) 300 mg twice a day and pravastatin 10 mg once a day. AA was referred to a tertiary hospital at age 60 days.
Further history revealed that AA is the first child born to first-degree consanguineous parents with positive family history of hyperlipidemia, maternal side in old age (father and aunt). There is no history of sudden death, premature cardiovascular disease, or recurrent pancreatitis in the family. Both parents had no history of hyperlipidemia.
Examination revealed an active child with no dysmorphic features or skin lesions. Abdominal examination revealed hepatomegaly. Cardiovascular examination was normal, as well as blood pressure. He was referred to the ophthalmologist for retinal examination which showed lipemia retinalis.
Laboratory investigation showed normal low-density lipoprotein (LDL), high-density lipoprotein (HDL), liver enzymes, baseline echocardiogram (ECHO), electrocardiogram (ECG), and ultrasound spleen. Ultrasound of abdomen confirmed hepatomegaly. Blood investigations are summarized in ().
Based on this incidentally discovered hyperlipidemia with very high TG and low to normal VLDL at such a young age, laboratory investigations indicate that this child probably has FCS. He was continued on Gemifirazil (lipoid) 300 mg twice a day. Pravastatin was stopped as no evidence supports its use in treating hypertriglyceridemia. The parents were referred to a dietitian and a low-fat diet was recommended, including mixing food with olive oil and giving skimmed dairy products as he is growing. Followup is ongoing.
AA was admitted twice at the age of 6 months and again at the age of 2 years. Both times he had vomiting and loose stool. He was diagnosed with acute gastroenteritis and was managed accordingly. Acute pancreatitis was suspected but could not be proved. He had no further attacks of abdominal pain or admissions and was regularly followed up. Eruptive xanthomata developed on the elbows and ear lobes at the age of four years, which was transient and resolved spontaneously. Lipid profile, liver function, and complete blood count were closely monitored ().


SA is a 4-year-old boy. He was referred at age 16 days because of the positive family history of hypertriglyceridemia. He had full-term, spontaneous vaginal delivery with uneventful pregnancy. Examination revealed an active, but not dysmorphic baby. No skin manifestation. Abdomen showed no hepatosplenomegaly. Examination of the cardiovascular system was normal, including blood pressure. Eye examination was normal. Baseline ultrasound of abdomen, ECG, and Echo were normal. Laboratory investigations revealed TG 5.92 mmol/L, high (normal <1.70 mmol/L), cholesterol 2.32 mmol/L normal (normal <4.40 mmol/L), HDL 0.47 mmol/L (normal >1.55 mmol/L), LDL <1 mmol/L (normal <2.26 mmol/L), and HB 18.7 g/dL (13.6–19.6 g/dL). He was given dietary advice but no lipid-lowering drug was started. He was observed with close follow up.
Six months later, TG was significantly raised: TG 10.56 mmol/L (929 mg/dL), cholesterol 7.09 mmol/L, HDL 0.34 mmol/L, and LDL 1.03 mmol/L. Due to the positive family history and abnormal laboratory findings, he was started on Gemifirazil (lipoid) 300 mg twice a day. Liver enzymes were normal prior to commencing medications.
SA was also referred to a dietitian for low-fat milk and dietary advice. On follow up he was noticed to have hepatomegaly and eruptive xanthomas on the elbows, which was transient and resolved spontaneously. There was no history of abdominal pain or hospital admission for suspected pancreatitis. The eye examination showed no evidence of lipemia retinalis. Lipid profile, complete blood count, and liver enzymes were continuously monitored ().


A 40 year old man was admitted with history of left lower abdominal pain, fever and vomiting for the past two days. On examination, he was febrile with a temperature of 38.3°C and moderately dehydrated. There was tenderness with rebound tenderness in the left iliac fossa. Clinically he was diagnosed as acute diverticulitis. His subsequent blood investigations data were as follows: Urea 5.67 mmol/L (range 2.1-7.1), Creatinine 96.6 mmol/L (range 62-106), Glucose 22 mmol/L (range 3.6-5.5), Sodium 135, Potassium 5.1 and white cell count 16.1 10^3/µL (range 4.0-11.0). The blood gases analysis was suggestive of metabolic acidosis with a pH of 7.2 and decrease in PCO2. The urine examination showed three plus of ketone. A routine ultrasound examination of abdomen was requested, which revealed the liver on the left side, spleen on the right side and an inflammatory mass in the left iliac fossa. This changed the diagnosis to situs inversus and the inflammatory mass in the left iliac fossa was now thought to be appendicular in origin. The heart sounds were heard over the right chest and subsequent plain X-ray of chest showed dextrocardia. The ECG findings were suggestive of dextrocardia, sinus rhythm and intra-ventricular conduction defect. The CT with contrast confirmed the diagnosis of complete situs inversus with dextrocardia and the appendix was seen as a tubular structure in the left iliac fossa, in front of left psoas muscle with surrounding inflammation []. The patient was unaware of his diabetes mellitus and his situs inversus anomaly. He was managed with intravenous fluids, insulin infusion and parental antibiotics prior to his surgery.
At laparoscopy, the situs inversus findings were confirmed. The cecum and ascending colon was on the left side. An inflammatory mass was seen in left iliac fossa, covered with omentum. Another 10 mm port was inserted in the left iliac fossa and a 5 mm port in the suprapubic region. The omentum was separated from the mass; the appendix was laying partially retrocecal and acutely inflamed, but not perforated. The appendicular pedicle was ligated intracorporeally. The mesentery of the appendix was thick and bulky; the intracorporeal ligation of the appendicular pedicle helped in mobilizing the appendix and its delivery through the left iliac port. The appendectomy was performed extracorporeally [].


A 19-year-old man without significant past medical history was brought to the Emergency Department having been found unconscious at home, after an explosion of unclear origin was noticed in the basement of the family home. Upon admission, the patient was fully conscious and stated, that he had been handling a self-made pipe bomb that exploded unexpectedly, severely injuring his left hand and “somehow hitting his right side of the face.” At the scene, he complained about a headache and the inability to see with his right eye, which was blood crusted. The patient also reported that he could not feel his left hand, which was severely injured. On presentation to the ER (trauma level 1 institution), he was stable and conversant with a GCS of 15 with minimal residual bleeding from his face and a profusely bruised and swollen right orbit []. His left hand was partially amputated []. The patient subsequently underwent primary and secondary surveys according to ATLS protocol and was sent for imaging studies. An ophthalmology consult was obtained in the ER, which revealed gross destruction of the right eye with globe rupture and no residual vision. His contralateral left globe was fully intact the only neurological abnormality was progressive drowsiness, which led to expeditious imaging work-up.
Initially a noncontrast CT scan of the head was performed emergently and revealed an intracranial irregularly shaped metal fragment measuring approximately 5 × 1.5 × 0.2 cm as seen on the tomogram scout films []. The object was lodged in the back of the right orbit, reached through the superior orbital fissure into the middle cranial fossa and transversed the right temporal lobe. It was comparable to a near complete blow-out fracture of the right orbit with adjacent ethmoid- and sphenoid-fractures before the fragment penetrated posteriorly into the floor of the middle cranial fossa formed by the petrous portion of the right temporal bone and skull base []. For further analysis, a CT angiography was performed revealing the tip of the metal fragment had penetrated the petrous bone deeply reaching the right internal carotid canal [].
While examinations were ongoing, the patient became progressively restless and drowsy. He was emergently intubated in the ER and taken to the OR for neurosurgical exploration in conjunction with an orthopedic trauma team who simultaneously took care of the patient’s left hand. The patient received intravenous antibiotics (vancomycin 1 g, flagyl 500 mg, gentamycin 80 mg) and a tetanus vaccination. For brain relaxation 100 g I/V Mannitol and 20 mg I/V lasix with mild hyperventilation (pCO2 = 30) was administered along with 10 mg of I/V Decadron for a possible temporal lobectomy. Two units of fresh frozen plasma were infused to prevent DIC and other blood products were readily available.
Surgical exposure was accomplished via a right fronto-temporal craniotomy and simultaneous right neck exposure of the right common carotid artery, to allow for proximal control in case of significant intracranial hemorrhage. A right temporal lobectomy was carefully performed around the retained foreign body and the skull base was explored. The copper pipe fragment [] which was firmly lodged into the petrous bone and sphenoid wing was exposed. The part of the metal fragment in the petrous bone was safely loosened from the intracranial side. An occuloplastics consult was performed intraoperatively. As the eye was severely and irrepairably injured, a right-sided enucleation was performed. This procedure also facilitated further transorbital exploration and allowed to mobilize the metal fragment which was lodged in the sphenoid. After drilling down parts of the orbital apex and sphenoid wing around the metal fragment, which reached the floor of the middle cranial fossa, it was possible to mobilize and retrieve the entire foreign body in one single piece via the transorbital route[] []. Simultaneous packing of the petrous portion of the temporal bone was performed. As a precaution, we had exposed and slinged the ICA at the level of the CCA bifurcation to allow for possible ICA occlusion in the neck, should the packing be unsuccessful. During careful exploration, no laceration of the intracanalicular ICA was evident and the skull base defect was closed. The floor of the middle cranial fossa and the retro-orbital wall was closed with a titanium mesh (Synthes) and an onlay duroplasty allograft (Duragen; Integra). Due to the severity of the injury, we felt safer to leave the patient with a decompressive hemicraniectomy.
When the neurosurgery team had completed the critical portion of this procedure, the left hand was explored, surgically debrided and initially reconstructed as a claw with Steinman pins. Meanwhile, a spherical implant was placed by occuloplastics to reconstruct the orbital soft tissue following the enucleation. The postoperative CT scan of the head demonstrated normal post-surgical findings without complications []. The patient was then transferred to the ICU in stable condition. He was extubated on hospital day two, transferred to the floor the same day, and fully mobilized on hospital day three. The patient did not show any focal neurological deficit other than loss of vision on the right side, mild ipsilateral periorbital numbness, and left hand impairment. Due to extensive orthopedic wound care requirements, he was discharged from the neurosurgical service on postoperative day nine. The patient was scheduled to follow-up with outpatient care for planning of his further elective interventions: cranioplasty and staged repair for his upper left extremity and right eye. Of note, the 19-year-old patient was able to attend high-school graduation only 14 days after this incident [].
An uneventful cranioplasty was performed eight weeks after the initial bomb explosion. Outpatient follow up scans have been unremarkable for three years without signs of delayed complications. Clinically the patient remains unchanged. Due to the severe and destructive hand injury, the initially reconstructed claw-fingers had to be amputated at the proximal metacarpal line several weeks later to create a stump with palmar tissue only.
No additional neurological deficits occurred and the patient enrolled to college the year after his accident and successfully graduated in 2009 []. The patient also was able to master physical labor as field apprentice in a construction firm.


In March 2010, a 75-year-old male patient came to our department of Dermatology presenting on the trunk surface and proximal upper limbs, erythematosus, and oedematous patched lesions, sometimes vesicular, bright red, infiltrated, and round in shape ().
The patient, suffering from Alzheimer's dementia, was treated for about a month with rivastigmine 9.5 mg/24 h patch. The skin lesions appeared after about 15 days from the first application of the patch; then, he proceeding with topical corticosteroids, there was only partial skin lesions improvement. When the rivastigmine transdermal patch was applied, recurrent lesions in previous application skin sites are observed.
The rivastigmine transdermal application was stopped when the patient came to our evaluation, and one month after skin lesions full remission we started the allergological study with European standard series and we added supplementary series for rubbers and resins. All the tests were negative at 72 and 96 hours.
Therefore, rivastigmine patch was performed with positive result after 48 and 72 hours; in contrast, placebo patch test with support but without drug showed negative result. Three months after, we repeated the tests, confirming the previous results (control test).
Under anesthesiological surveillance, we administered orally rivastigmine at escalating doses from 1.5 mg daily, but after 72 hours from the first administration, the skin lesions reappeared, an event which could be supposed as an “oral trigger proof.”


A 68-year-old man who lived in Venezuela for the last four decades presented to our clinic with inexplicably incremental renal and hepatic failure with an obviously reduced general and nutritional condition for further diagnostic and therapy. Due to severely reduced creatinine-clearance (MDRD = 14 mL/min), he was scheduled for continuous ambulatory peritoneal dialysis (CAPD) both to drain his up to six litre nonmalignant, persistent hydroperitoneum as well as for dialysis. Patient's history was inconspicuous apart from a single vessel coronary heart disease that had lead to an acute myocardial infarction (AMI) interventionally treated by implantation of a bare metal stent into the LAD three years ago. One-month post-AMI, the patient complained of progressive abdominal heaviness and pressure as well as shortness of breath, which was nebulously explained by hepatic failure due to thienopyridine treatment. In contrast to the assumed hepatic cirrhosis, multiple abdominal ultrasound studies did not reveal cirrhotic hepatic tissue texture as well as excluded a veno-occlusive Budd Chiari disease. Shortly before the admission to our hospital echocardiography study was conducted externally with reduced acoustic windows to exclude an underlying cardiac disease and showed a moderately reduced left ventricular function, a mild aortic and mitral regurgitation as well as anterior hypo- to akinesia with potential apical adhesions. These findings were in far contrast to a holosystolic harsh loud murmur accompanied by a thrill being present in the third and fourth left intercostal space. For further clarification of morphology, function, the extent of myocardial scar, and exclusion of constrictive pericarditis, a gadolinium contrast enhanced and cine MRI was conducted. Cine short axis, two- and four-chamber MRI imaging showed a dilated right and left ventricle with an almost severely reduced left and right ventricular function (LV ejection fraction = 35%), a pronounced apical post-AMI aneurysm with transmurally infarcted myocardial tissue () as thin as 1.5 mm together with a moderate to severe mitral and tricuspid regurgitation. Short axis imaging () revealed a hemodynamically highly significant 12 mm large apical interventricular septal defect (IVSD) with an MRI estimated regurgitation fraction of 52%. Therefore, having come to the diagnosis of a post-AMI ventricular IVSD, we consequently conducted a left and right heart transseptal catheterisation. Heart X-ray confirmed MRI results and revealed a left to right ventricular shunt of almost 63%, a severe LAD in-stent restenosis, and a severe mitral regurgitation. Despite the administration of more than 180 mL of X-ray contrast agent, patient's renal function was preserved due to an intensified CAPD regime. Heart surgery was performed where the apical aneurysm was excised, the mitral valve was reconstructed, the IVSD was closed and the subtotally in-stent occluded LAD was bypassed with an internal mammaria coronary artery bypass graft. Post-surgery, the ascites were significantly reduced. CAPD therapy could be terminated since the renal function gradually improved (MDRD = 25 ml/min).
The overall incidence of post-AMI IVSD is hard to assess because clinical and autopsy series differ considerably []. A large autopsy study in 1989 suggests that the incidence of myocardial interventricular rupture has increased since the late 1960s, with a rate of 31 per cent among necropsied cases []. The prior use of corticosteroids or nonsteroidal anti-inflammatory agents has been implicated as predisposing to rupture as a result of impaired healing. Conversely, the early use of thrombolytic therapy appears to reduce the incidence of cardiac rupture [], an effect that is responsible in part for improved survival with effective thrombolysis. The size of the defect determines the magnitude of the left to right shunt and the extent of hemodynamic deterioration []. The likelihood of survival depends on the degree of impairment of ventricular function and the size of the defect []. Recently a non-ischemic surgical repair was described but was not suitable in this patient due to the complex situation this newer approach without aortic occlusion, systemic hypothermia and cardiolplegic arrest.
In our case, due to right-sided heart failure with increased right atrial pressure and tricuspid regurgitation, subsequent congestive hepatomegaly associated ascites was extensively treated with diuretics and consequently led to hepatorenal syndrome. Although echocardiography might not always suggest IVSD due to reduced acoustic windows, simple and inexpensive auscultation is almost pathognomonic. An IVSD is characterized by the appearance of a new harsh, loud holosystolic murmur at the lower left sternal border and is usually accompanied by a thrill. Although biventricular failure generally ensues within hours to days, in our case the patient astonishingly survived more than three years with accretive signs of hepatic and renal failure. Operative intervention is mostly successful and should not be delayed in patients with a correctable lesion [].
Our case report has two important issues. To our knowledge, for the first time we report successfully practiced CAPD catheter drainage of hydroperitoneum due to congestive liver and renal failure in combination with CAPD hemodialysis. Secondly, simple auscultation of the heart lead to the diagnosis of IVSD that, if correctly performed earlier, could have greatly relieved the patient's symptoms, his reduced physical state, and his commencing hepatic and renal failure. Furthermore cardiac TEE or MRI as not invasive and radiation-free methods should be considered in case of limited acoustic windows for echocardiography to exclude an IVSD.


A 28-year-old man presented with severe neck pain mainly on the left side with painful swallowing for 5 days. The patient initially had asked for medical advice at a primary health care facility and was treated with antibiotics and anti-inflammatory drugs, but there was no improvement and the pain was increasing in severity. On detailed history, the patient reported that he had an attack of choking and coughing, while he was eating chicken during dinner one day before the onset of symptoms.
On examination, there was a low-grade fever and tenderness in the left side of the neck, and the pain was exaggerated by swallowing attempts. A telescopic laryngeal examination was performed. Frothy saliva was detected accumulating in the left pyriform fossa. After giving the patient some few sips of water, the examination was repeated. A white linear chicken bone was seen impacted at the left pyriform fossa with a relatively free broad base pointing anteriorly, and the other tapering end was impacted in the posterior wall of the pyriform fossa (). To rule out development of a retropharyngeal abscess, anteroposterior and lateral X-ray views of the neck were performed for the patient. Apart from the chicken bone that was poorly visualized in the lateral X-ray view of the neck, there was no evidence of retropharyngeal abscess formation.
The bone was removed in an office-based setting under guidance of video laryngeal rigid telescopic examination. In this procedure, a video laryngeal examination was carried out using a 70-degree rigid telescope, 3CCD camera, LCD monitor, and a light source. The patient was seated in the upright position with his head supported from the back. Using a cotton swab soaked with Xylocaine 10% and held by a Magill's forceps, the patient's oro- and hypopharynx were adequately anesthetized. The patient was instructed to protrude his tongue and hold it by his hand with a piece of gauze. The telescope was held by the physician's left hand and introduced carefully into the oropharynx. When the foreign body was identified in the left pyriform fossa and clearly visualized on the monitor, it was carefully removed by a curved laryngeal forceps held by the right hand of the physician (). After that, the patient was prescribed oral antibiotics in the form of Amoxycillin-Clavulanic acid (1 gm b.i.d. for 10 days) to control possible infection and an analgesic and anti-inflammatory drug in the form of Diclofenac Sodium (50 mg b.i.d. for 10 days). Then, he was discharged with a scheduled followup appointment after 7 days. On the followup visit, the patient was completely improved with disappearance of the constitutional symptoms, no pain or tenderness on the neck and normal swallowing function.


A 77-year-old male with mediastinal lymphadenopathy was referred to our hospital. Chest computed tomography (CT) scanning demonstrated #4R and #7 lymphadenopathy without tumors in the lung field (Figure ). A gastrointestinal fiberscope examination found no malignancy in the upper gastrointestinal tract, while 18F-fluorodeoxy-glucose positron emission tomography showed a positive accumulation in the mediastinal lymph nodes without other suspicious lesions.
The patient underwent a video-assisted mediastinoscopy procedure in a supine position with a 5-cm cervical incision. The pretracheal lymph nodes adjoining the right pulmonary artery were found to be enlarged and carefully punch-biopsied several times. While a biopsy procedure was being performed, a massive amount of pulsate dark-red blood appeared and spilled out through the scope. Immediately, the mediastinoscope was removed from the body and bleeding from the incision was controlled with digital compression at the site of cervical incision adjacent to sternal notch closing the outlet of bleeding flow. Since the blood color was dark-red, we considered that the bleeding occurred from a low-pressure circulation system component, such as the pulmonary artery, and thought that it would cease in the mediastinum compartment after closure without making a large hematoma, even though the bleeding was massive in open air space. Digital compression was maintained for 10 minutes, and we confirmed that there were no changes of vital signs. Then we closed the incision in a three-layer manner without any gauze packing in the mediastinum.
A pathological examination of the biopsied specimens revealed a whole arterial wall with a smooth muscle layer and vascular endothelium indicating that pulmonary artery wall was punched out (Figure ). The bleeding came from the right main pulmonary artery, but not from the superior vena cava. A follow-up CT scan showed a small hematoma in the mediastinum (Figure ) that had not been observed before the mediastinoscopy procedure. Diagnostic specimens were not obtained during the mediastinoscopy, thus we performed a right thoracoscopic biopsy of a subcarinal lymph node two weeks later. There were no coagula seen in the subcarinal space. Squamous cell carcinoma metastasis in a subcarinal lymph node from an unknown origin was diagnosed and the patient underwent chemoradiotherapy.


A 36-year-old pregnant woman who had previously been diagnosed with APS had received anticoagulation treatment with 100 mg/day of aspirin and 12000 U/day of unfractionated heparin and had experienced abortion twice. She presented with gross hematuria at 20 weeks of pregnancy and discontinued aspirin administration. However, hematuria did not resolve, and lower left abdominal pain developed. Although hydronephrosis was not detected by ultrasound, left ureteral calculus was suspected from the patient's clinical course. Seven days later, hematuria and left abdominal pain developed again. The patient was treated conservatively with analgesics because ultrasound and plain abdominal X-ray results were unremarkable. Ten days later, the patient attended the hospital emergency department and was hospitalized urgently in urological ward for workup and treatment because of severe right back pain and severe gross hematuria developed. On admission, complete blood cell count (CBC) revealed elevated white blood cell counts (WBC 16.29 × 103/μL), decreased red blood cell and hemoglobin levels (RBC 2.76 × 106/μL and Hb 8.1 g/dL), and normal platelet counts (21.9 × 104/μL). The results of blood biochemical and clotting tests were unremarkable. There were no findings in physical examination except for a knocking pain of right costovertebral angle. Under an obstetrician's instruction, plain abdominal-pelvic computed tomography (CT) with reduced dose was performed although the patient was in second trimester. CT findings showed expanded right upper urinary tract and the hematoma in the right renal pelvis was suspected (Figures and ). There was no calculus and no mass lesion, and perinephric bleeding was not seen. The cause of bleeding in the right renal pelvis was unknown. Administration of unfractionated heparin was immediately discontinued. On day 2 after admission, severe back pain persisted and WBC was still elevated (26.64 × 103/μL). Therefore, antibiotic treatment was started for suspected urinary tract infection. On day 3, patient-controlled analgesia (PCA) with fentanyl was started for persistent pain by the anesthesiologist. Blood transfusion was performed on day 4 because CBC showed progression of anemia (Hb 6.7 g/dL). Ureteral stent placement under ultrasound guidance was performed because expansion of the right renal pelvis due to hematoma persisted. The pain and hematuria were subsequently relieved. On day 16, administration of unfractionated heparin was resumed based on consultation with an obstetrician and a hematologist. The patient was discharged on day 21. Although outpatient anticoagulation treatment was continued, gross hematuria and back pain did not recur. At 36 weeks of pregnancy, the patient was admitted to hospital and continuous instillation of heparin was started. One week later, she gave birth to a female infant with a birth weight of 2190 g by vaginal delivery after discontinuation of heparin infusion. Apgar scores at 1 and 5 min were 9 and 10, respectively. Continuous instillation of low-molecular-weight heparin was started postpartum. Abdominal-pelvic CT and urine cytology performed on day 4 postpartum revealed no abnormalities in the urinary tract. Heparin infusion was then switched to oral low-dose aspirin. The ureteral stent was withdrawn on day 8, and the patient has not developed any symptoms, such as pain or hematuria.


A 48-year-old female presented with low back pain radiating to both buttocks and the posterior thigh for 3 months. She also suffered from neurogenic intermittent claudication (NIC) within 10 minutes. The straight leg-raising test was positive at 60 degrees on both sides. However, sensory or motor of the lower extremity was normal.
Magnetic resonance imaging (MRI) demonstrated a cystic lesion measuring 6 × 16 × 16 mm with low-signal intensity on T1-weighted imaging and high-signal intensity on T2-weighted imaging at the level of the L3-L4 disc. The cystic mass, located in the ventral aspect of the extradural space, displaced the thecal sac dorsally [Figure and ]. Rim enhancement of the lesion was appreciated after administration of gadolinium []. The L3-L4 intervertebral disc appeared to have mild degeneration. After L3-L4 laminotomy, a tense dark blue-colored cystic lesion compressing the entire thecal sac was encountered. The cyst was found to be mildly adherent to both the thecal sac and the posterior longitudinal ligament (PLL). The bloody serous fluid was aspirated from the cyst. The cyst was traced back to its stalk, which communicated with the L3-L4 disc by means of a central annular tear. The cyst and stalk were excised completely at the base of the connection. Coagulation of the PLL surrounding a round defect was performed; however, the disc space was not entered. The patient achieved complete pain relief and was allowed to walk on the day after surgery. Histopathological examination of the cyst revealed thick fibrous connective tissue interspersed with areas of chronic inflammation. However, there was no evidence of specific lining cell layers or disc material []. MRI obtained 14 months after surgery showed no evidence of recurrence or progression of disc degeneration []. The patient has remained asymptomatic during a two-year follow-up period.


A 49-year-old female (gravida 1, para 1) presented to a gynecologist with complaints of right lower quadrant discomfort for 3–4 weeks, which was associated with nausea and poor appetite. There was no history of menstrual irregularity. Her last menstrual period was 4 weeks ago. An initial urine pregnancy test was negative. Physical examination revealed a normal uterus with a soft, mobile right adnexal mass.
A pelvic USG [] showed a complex 6.0 cm × 4.6 cm × 3.7 cm mass in the right adnexa, while the right ovary could not be separately identified, suggesting a possible ovarian origin. The mass had a cystic component with thick septae and also a solid component without definite evidence of posterior acoustic shadowing to suggest calcification. Minimal intralesional vascularity was identified. There was no free or loculated fluid in the pelvis. At this stage, our differential diagnosis included ovarian neoplasm, dermoid and tuboovarian abscess. Because of the absence of constitutional symptoms of infection, a tuboovarian abscess was considered relatively less likely. Further evaluation with MRI was recommended for characterization.
MRI of the pelvis was performed on a 1.5-Tesla scanner, which showed a lobulated, complex, multiloculated right adnexal cystic mass [] measuring 6.7 cm × 3.6 cm × 5.1 cm, with the right ovary not separately identified. There was a predominant cystic component demonstrating low-signal intensity on T1W and high-signal intensity on T2W images [Figure and ]. Solid components were also noted, which demonstrated intermediate signal intensity on T1W and low-signal intensity on T2W images – isointense to the skeletal muscle [Figure and ]; there was mild postgadolinium enhancement []. The features were suspicious for an ovarian cystic neoplasm with a fibrous component. There was no free fluid or adjacent pelvic lymphadenopathy.
The patient underwent total abdominal hysterectomy with bilateral salpingooophorectomy. The frozen section of the right ovarian mass demonstrated features of benign cystadenofibroma, a diagnosis that was confirmed on histopathology.


A 64 year old lady underwent electrophysiologic study for recurrent episodes of palpitation and documented narrow complex tachycardia. 12 lead ECG showed normal sinus rhythm and no evidence of preexcitation. At baseline, the cycle length, AH and HV intervals were 776, 52 and 46 ms respectively. Para-Hisian pacing was done at baseline and the response is shown in . The pacing output during the maneuver was 15 mA. There is evidence of combined His bundle and ventricular capture during the second and fourth complex (narrow complex) and ventricular capture (His bundle non-capture) during the first and third beats (wide complex). There is evidence of both nodal and extranodal response during the maneuver. The first two complexes are suggestive of nodal response and the last 2 complexes are suggestive of extranodal response. On incremental ventricular pacing, VA Wenckeback cycle length was at 340 ms. On programmed ventricular pacing from the right ventricular apex with a single extrastimulus, VA was concentric and decremental. On programmed atrial stimulus, there was AH jump and a narrow complex tachycardia was induced. The septal VA interval during the tachycardia was 30 ms. Entrainment of the tachycardia from the right ventricle resulted in V-A-V response and the PPI-TCL and SAVA intervals were suggestive of typical AVNRT (220 and 154 ms respectively). His refractory PVC failed to advance the atrial activation. Since there was no other evidence of pathway apart from the para-Hisian maneuver, we repeated it and the response is shown in . What is the mechanism of the mixed response during the para-Hisian pacing observed earlier?