A 51-year-old male presented with a two-year history of a growing painless mass under the right ear, initially with a size of a soybean. The mass grew noticeably in the last six months. There was a one-year history of night sweat and frequent stool (three to four times a day). There was no history of fever, weakness, dysphagia, dyspnea, cough, hoarseness, jaundice, vomiting, melena, hematochezia, abdominal pain, abdominal distension or significant weight loss. The patient had a 5-year medical history of hypertension and he was a hepatitis-B carrier of 30 years and a smoker of 40 pack-years. There was no family history of cancer.
On palpation, a 20 mm × 20 mm relatively well-defined and soft mass with no tenderness was observed along with multiple enlarged cervical nodules. Abdominal examination did not reveal any organomegaly or palpable lumps.
Ultrasonography of the neck two months ago revealed a relatively undefined hypoechoic mass measuring approximately 15 mm × 27 mm in its greatest dimension in the right parotid gland and submandibular gland (Figure ) along with multiple enlarged right supraclavicular and upper cervical lymph nodes. A needle biopsy of the mass was performed and the pathologic report found malignant tumor cells. The patient was recommended for surgery for the mass in the parotid gland. The preoperative blood routine examination showed that the HGB was 106 g/L. Therefore, the patient underwent positron emission tomography/computed tomography (PET/CT). A 36 mm × 33 mm intestinal mass with increased glucose uptake, and multiple peripheral lymph nodes in the right mid-abdomen were found (Figure ), and the maximum standard uptake value (SUV) was 6.6. An intestinal lesion with increased glucose uptake in the right hypogastrium was also seen and the SUV was 7.0. The mass in the right parotid and peripheral lymph nodes also showed increased glucose uptake, and the SUV was 10.3. Preoperative tumor makers, such as CA125, CA15-3, CA19-9,CA72-4, AFP, cyfra21-1, NSE,SCC, CEA, and ProGRP, did not show abnormal expression.
The patient underwent an exploratory laparotomy and the excision of multiple intestinal neoplasms. Operative exploration showed no ascites, pelvic, periaortic, peritoneal, omental deposits, or liver metastasis. No tumors were palpated in the cavity of the stomach, duodenum, colon, rectum, or the mesentery root. Three masses were found at the jejunum and ileum. Intra-operatively, the first tumor was present in the jejunum, located at 80 cm distal to the duodenojejunal junction. Intussusception was observed at the point, and the involved bowels were swollen and expanded (Figure ). The second tumor was at the end of the intussusception (approximately at the fourth loop of intestine). The third tumor was present in the ileum, located at 80 cm proximal to the ileocecal junction. These three tumors of varying sizes invaded the serosa, and the surface of the serosa had shrunk and was depressed. Multiple enlarged lymph nodes were observed in the intestinal mesentery. Following serial ligation of the mesenteric vessels, resection of the involved bowels, along with the masses and mesentery, was performed, with a proximal margin of 10 cm and a distal margin of 10 cm. The first and second tumors were removed together in one segment of the intestine (Figure ). Then, a primary anastomosis formed. The patient recovered gradually and then underwent right parotidectomy with retention of the facial nerve, followed by right cervical lymph node dissection 17 d after abdominal surgery because the pathology of the parotid gland neoplasms was undetermined.
Intestinal neoplasms: Upon gross examination, the specimen consisted of two segments of the small intestine: the longer one was approximately 26 cm with attached mesentery, and the other segment was 7.8 cm with attached mesentery. Two tumors were on the longer segment of intestine, one (2.5 cm × 2.2 cm × 1 cm) was at 11 cm from one margin and the other (6.5cm × 5.5cm × 4 cm) was at 19 cm from the same margin. A 2.5 cm × 1.9 cm × 1 cm tumor was on the other segment of the small intestine. The cut surface of the three tumors had hard, obscure borders that were white to tan in appearance.
Microscopically, the jejunum and ileum tissues were infiltrated with malignant cells, which was consistent with CCS-GI (a type of gastrointestinal neural ectoderm tumor, GNET) based on morphology and immunohistochemistry (Figure ). The tumors had invaded the mucosal and muscular layers. There was no focal necrosis, vessel invasion or nerve invasion. The mitotic index exceeded 20/10 HPFs, and the tumor was grade G3 according to the FNCLL (French Fédération Nationale des Centres de Lutte Contre le Cancer) system.
Lymph node metastases (1/29) without invasion of the outer lymph node capsule: (1) peripheral lymph nodes of the jejunum: 1/26; and (2) peripheral lymph nodes of the ileum, 0/3.
Immunohistochemistry: S100 (3+), Vim (3+), GFAP (-), HMB-45 (2+), Melan-A (2+), Melanomapan (1+), CD56 (2+), Syn (-), CgA (-), AE1/AE3 (-), CD138 (-), CD19 (-), CD20 (-), CD3 (-), CD38 (-), CD79a (-), Ki-67 (+40%), LCA (-), MUM1 (-), CD117 (lesion+), CD34 (-), DOG1 (-), CD10 (-), Calponin (-), P63 (-), EBER (-).
Gene detection: EWSR1 gene fusion transcripts were undetectable by fluorescence in situ hybridization (FISH).
Parotid gland neoplasms: Upon gross examination, a 1-cm diameter nodule was found in a 5.5 cm × 3 cm × 2 cm area of tissue; the cut surface of the nodule had a tough, grey-to-yellow appearance.
Microscopically, the parotid gland tissues were infiltrated with malignant cells, which was consistent with CCS morphology and immunohistochemistry and morphologically similar to the previously assessed intestinal tumor (Figure ). Lymph tissues were found in the tumor and at the tumor edge, which may be metastatic lesions.
No lymph node metastases (0/30): (1) right cervical lymph nodes, level II, 0/10; (2) right cervical lymph nodes, level III, 0/12; (3) right cervical lymph nodes, level V, 0/5; (4) peripheral lymph nodes of the superficial lobe of the right parotid gland, 0/2; and (5) peripheral lymph nodes of the caudate lobe of the right parotid gland and tumor, 0/1.
Immunohistochemistry: S100 (3+), Melan-A (3+), Melanomapan (3+), HMB-45 (3+), AE1/AE3 (-), CK18 (-), Calponin (-), P63 (-), SMA (-).
Twenty days after the surgery on the parotid gland, the patient underwent CT imaging of the neck, thorax and abdominopelvic area, and no recurrence or metastasis was observed. He then started with 6 cycles of chemotherapy using an EI regimen (epirubicin 100 mg + ifosfamide 2 g D1-4+mesna 0.4 g 0 h, 4 h, and 8 h after the ifosfamide D1-4). At the time that this article was written, the patient was on the first cycle of the chemotherapy.


A 22-year-old, right-handed housewife presented to the hospital with complaints of headache of 15 days duration. Headache was generalized, intermittent in duration, aching in nature, associated with vomiting and visual obscurations, especially while changing positions. These symptoms aggravated in morning hours. There was also history of single episode of generalized tonic–clonic seizure 10 days back, after which the patient was started on anticonvulsants and there were no further episodes. Patient also noticed weakness of the right upper limb since last 8 days, which was gradual in onset and progressive in nature. Weakness was more significant in the left hand as compared to the left shoulder. There was also history of drowsiness since last 2 days.
On neurological examination, the patient was conscious, cooperative, and well oriented in time, place, and person with normal higher mental functions. Visual acuity was 6/9 in both eyes. Fundus was showing grade 1 papilledema on both sides. The rest of the cranial nerves’ examination was normal. Nutrition and bulk was normal in all four limbs. Tone was increased on the right side. Power was 4+/5 in the right shoulder and 4/5 in the right elbow and right wrist. Grip was weaker on the right side as compared to the left side. Rest of the limbs had normal power. Sensory examination did not reveal any abnormality. Reflexes were brisk in the right upper limb, rest were normal. Gait was normal. Plain radiograph of the skull was normal.
Computed tomography (CT) scan [] showed a lesion of 3.4 cm × 2.4 cm in size with well-defined margins and multiple foci of calcification in the left fronto-parietal region. The lesion was strongly enhancing inhomogenously on contrast administration with surrounding perifocal edema and mass effect suggestive of glioma or tuberculoma.
Magnetic resonance imaging (MRI) [] scan was suggestive of a thick ring-enhancing lesion seen in the left high parietal region with perifocal edema with mass effect suggestive of granulomatous lesion, most likely tuberculosis.
CT angiography [] showed a small lesion in the left high parietal region with increased vascularity.
Patient was operated with left parietal craniotomy with total excision of lesion. The tumor was adhered to the undersurface of dura. The tumor was dark brown in color, necrotic, and solid with increased vascularity. There were feeders from the periphery. The brain was edematous and pulsating well. Lax duraplasty was done using pericranium. Tumor was excised in toto.
Postoperatively, the patient recovered well. She was relieved of headache completely and there were no further episodes of vomiting or seizures. The right upper limb weakness improved to 4+/5. The blurring of vision also improved gradually. There was no new neurological deficit postoperatively.
Postoperative CT scan [] showed complete excision of the lesion.
The patient was discharged on 10th postoperative day without any neurological deficit.
Neuropathological [] examination showed a tumor composed of peripherally arranged tumor cell in sheets and syncytium. These were large cells having moderate eosinophilic to clear cytoplasm with dense hyperchromatic nuclei and increased nucleocytoplasmic ratio and prominent macronucleoli. The tumor showed mitotic activity of about 1-2/high power field, and extensive areas of necrosis and hemorrhage suggestive of primary choriocarcinoma of the left parietal lobe.
In view of the diagnosis of choriocarcinoma, the patient was investigated extensively for the detection of the primary lesion. b-Human chorionic gonadotropin (b-HCG) was 20,000 IU. Gynecology opinion was taken Patient was pregnant once and had delivered full-term baby vaginally 2 years back without complications. Past and present menstrual history was normal. Transvaginal ultrasound was normal. X-ray chest was normal. CT scan abdomen and pelvis did not reveal any abnormality.
The patient has been referred to another specialized institute for adjunctive treatment. She has been following up regularly with evidence of widespread metastasis over a period of 6 months.


A 12-year-old girl presented to us with headache and ataxia for two months. Her headache was moderate to severe in intensity, more in the morning, and associated with nausea and vomiting. She had no other complaints then. On a general examination, she had no deformity or abnormal pigmentation. On neurological examination, she had normal higher psychic function with intact cranial nerves except bilateral papilloedema and balance problem. Her motor and sensory examination revealed no other abnormality. But her gait was ataxic and Romberg's test was positive. She had neither any cerebellar signs nor any spasticity.
Examination of other systems revealed no abnormality except a fixed mass in the right side of her abdomen. The mass was palpable, 5 cm away from the costal margin at the anterior axillary line, and it was about 10 cm long along its long axis. It was firm in consistency with an ill-defined margin.
MRI was performed and it revealed a bilateral VS []. Pure-tone audiometry (PTA) revealed right-sided mild hearing loss but left-sided normal hearing. Ultrasonography revealed a large retroperitoneal mass in the right side of her abdomen. CT scan of abdomen revealed a retroperitoneal mass just in front of the right kidney []. At first, we operated on the right cerebello-pontine angle through posterior fossa craniectomy in the sitting position. Near-total removal of tumour was accomplished. It was confirmed as schwannoma by histopathology [], where the sections show a capsulated spindle cell neoplasm composed of Antoni A and Antoni B areas.
Her recovery was normal except right-sided mild facial palsy and mild hearing loss confirmed by PTA. Two months later, we operated on the left-sided tumour []. It was also near totally removed and confirmed as before. Then she also had mild facial palsy and mild hearing loss in her left side.
She recovered well post-operatively, but after 2 weeks she became drowsy. One month after the operation, she became mute and developed urinary incontinence. She was again investigated by CT scan of the head, which revealed communicating hydrocephalus. She was treated with a ventriculoperitoneal shunt inserted into the right side. After 5 days, the shunt was found to be non-functional. The shunt was re-inserted into the left side of the abdomen, keeping the cranial end in situ. After this, she started to gain her consciousness gradually. She was then discharged.
After 2 months, she came for removal of the abdominal mass. We accessed her abdomen through a right transverse incision for the lump and debulked the tumour gradually and removed it completely. The patient eventually recovered well except bilateral mild seventh and eighth nerve palsy.


A 4-day-old preterm (36 weeks) male baby, weighing 2 kg, 1st in birth order, born by normal vaginal home delivery, presented with marked abdominal distension and nonpassage of meconium since birth. On physical examination, the baby was moderately dehydrated. The abdomen was distended with prominent veins all over the abdomen []. Perineum examination revealed meconuria, and normal urethral and anal openings, but infant feeding tube could not be inserted beyond 2 cm from the anal verge []. A preliminary diagnosis of RA was made. Abdominal radiograph suggested CPC Type 4 []. After preoperative optimization, laparotomy was performed with left hockey stick incision which confirmed the diagnosis of CPC Type 4 with colovesical fistula associated with RA. Excision of pouch colon with ligation of colovesical fistula and sigmoid end colostomy was performed at the distal end of the incision. Other associated gastrointestinal anomalies were ruled out and meticulous hemostasis was achieved before abdominal closure. Feeds were initiated on the 4th day and discharged in stable condition. The patient is awaiting evaluation of other associated genitourinary anomalies in follow-up.


A 2-day-old full-term male baby, weighing 2.25 kg, 2nd in birth order, born by normal delivery, presented with marked abdominal distension and nonpassage of meconium since birth. The abdomen was distended. Perineum examination revealed meconuria, and normal urethral and anal openings, but infant feeding tube could not be inserted beyond 2 cm through the anus. A preliminary diagnosis of RA was made. Babygram revealed the presence of CPC. On exploration, CPC Type 4 was found; pouch excision and sigmoid end colostomy were performed. The postoperative period was uneventful. On a follow-up visit, echocardiography revealed small PDA (patent ductus arteriosus) 2.5 mm with left to right shunting. Micturating cystourethrogram (MCU) suggested Grade II vesicoureteral reflux (VUR) on the right side. A combined abdomino-posterior sagittal anorectoplasty (A-PSARP) was performed at 5 months of age. Endoscopic treatment of VUR with injectable dextranomer/hyaluronic acid copolymer was performed for Grade II VUR in a further follow-up visit.


A 3-month-old female infant, who was born at 36 weeks of gestation with birth weight of 2300g, presented to the pediatric outpatient department (OPD) for increasing swelling at the left labial region since last 5 days, along with complaints of on and off swelling 3 times since birth; however, at presentation, the swelling was larger as compare to the previous state. During physical examination, two separate oval structures were palpated, which were nontender, along with normal appearing overlying skin. Provisional diagnosis was of indirect inguinal hernia containing either ovary or testis. The patient was send for ultrasonography for further confirmation. Ultrasound using high frequency probe showed two oval structures having multiple small anechoic cysts at left labial region with a hypoechoic structure having internal echogenic strip, which was continued in abdominal cavity through a defect in abdominal wall of 6.6 mm. Hence, USG findings were suggestive of indirect inguinal hernia containing uterus and bilateral ovary []. At colour Doppler ultrasound normal arterial vascularity was detected in bilateral ovary, suggesting viable ovarian tissue []. The baby was operated by the pediatric surgeon, and at the operation, both ovary with uterus were herniated into the left inguinal canal and left labia majora. A surgical reduction procedure of herniated structure and high inguinal ligation was performed.


A 1-month-old male infant present to pediatric emergency OPD with complaints of painful left inguinoscrotal swelling. On presentation, the infant was anxious and in a steady state of intense crying. On physical examination, a tender mass was palpated in the left inguinoscrotal region with inflamed reddish scrotal skin. Ultrasonography examination showed dilated fluid-filled bowel loop in inguinoscrotal region [] along with reduced peristaltic activity with mild amount of fluid in the herniated sac cavity around bowel and anterior laterally pushed left testis; the findings were suggestive of incarcerated hernia as peristalitic activity present in herniated loop was sufficient to rule out strangulation. Echogenic mesentery was seen attached to the dilated loop at superficial inguinal ring region, which showed vascularity on color Doppler, further confirming viability of loop. Emergency laprotomy was done, herniated loop was replaced in the abdomen along with high ligation of herniated sac.


A 5-day-old female newborn, who was born after 39 weeks of gestational with a birth weight of 2700 g, was brought to the pediatric OPD following the discovery of an asymptomatic lump in her right groin by her mother. On physical examination, a nontender mass was palpated in the right inguinal region extending up to the upper aspect of right valva just above the labium majora. She was advised ultrasonography for further evaluation. On B-mode ultrasound, an8 mm wide fascial defect and a herniation of well-defined oval structure contain internal small cysts, which was about 15 mm × 9 mm in diameter, with a attached pedicle. Thus, this was diagnosed as the right ovary herniated through the inguinal canal []. At Colour Doppler, vascular signals with arterial wave form were obtained at pedicle region, thus indicating viability. On the same evening, the swelling was readily reduced with gentle pressure.


A 78-year-old male with hemochromatosis complicated by locally advanced HCC was admitted to our hospital from a skilled nursing facility with new-onset bilateral lower extremity edema. His comorbidities included atrial fibrillation and hypertension, but he had never suffered from significant edema or symptoms of congestive heart failure previously. He was at the skilled nursing facility for rehabilitation after a prolonged hospitalization during which he suffered from postembolization syndrome after his fourth round of transarterial chemoembolization (TACE).
On questioning, he complained primarily of discomfort in his legs related to edema but also endorsed fatigue and progressive dyspnea on exertion. On presentation, his vital signs were within the normal limits. Physical exam was notable for moderate jugular venous distension and an expectedly irregularly irregular cardiac rhythm without audible murmurs. His lungs were clear to auscultation, and his extremities had 4+ pitting edema to the sacrum. Laboratory evaluation revealed a mildly elevated BNP of 236 pg/mL, a slightly low serum sodium of 133 mmol/L, and normal kidney function. Small bilateral effusions were seen on chest X-ray.
The patient underwent transthoracic echocardiography, which revealed a 6.4 × 4.3 cm mass occupying nearly the entire right atrium, and extending to the inferior vena cava (IVC) (Fig. ). Angiography was then pursued and showed subtotal occlusion of the IVC by a mass extending from the hepatic veins into the right atrium (Fig. ). Biopsy of the cardiac mass confirmed poorly differentiated HCC. Unfortunately, the patient was not a candidate for surgical resection or chemotherapy due to his comorbidities. Palliative radiation was considered, but the patient's symptoms progressed to the point of dyspnea at rest and anasarca before it could be initiated. Given his rapid decompensation, the patient elected to pursue hospice care. He was transitioned to a local hospice care facility and died 12 days later.


We report the case of a 14-year-old teen girl who presented with a skin lesion on the inner side of the left arm, approximately at the level of the elbow []. The lesion was initially observed 5 months before and was continuously increasing in size during that period. The history and the clinical examination of the patient did not reveal any other pathological background.
Upon examination, the lesion had a cystic, semi-transparent, fluid-filled, pedunculated appearance. At its inside, multiple, stony-hard, nodule-like structures were observed. The approximate dimensions of the lesion were 30 mm × 20 mm. During palpation, the patient did not refer any discomfort or pain.
The magnetic resonance imaging with and without the use of contrast media demonstrated a well-demarcated, broadly based mass with centrally solid and peripherally cystic structures []. That abnormal structure did not infiltrate the muscular or the bone tissues though. There was also an afferent vessel, originating from the basilar artery. The differential diagnosis based on the clinical finding of rapid growth included a subcutaneous sarcoma (fibroma-like or pseudogranulomatous), but the radiologic findings were more suggestive of a benign subcutaneous ring-like granuloma or a subcutaneous fibroma.
The lesion was surgically excised, and surgical exploration of the underlying tissue did not reveal any local infiltration or spread.
Histopathology showed a tumorous formation (diameter ~ 3 cm) of subcutaneous origin consistent with the morphology of calcified Malherbe's epithelioma. It was also consisted of dermoid cysts and dilated lymphatic vessels. No malignant cells were found [Figures and ].
Cutaneous hybrid tumor composed of epidermal cyst and cystic pilomatricoma as in our case is very rare.[] Pilomatricoma (pilomatrixoma), or calcifying epithelioma of Malherbe, is a tumor with differentiation toward hair cells, particularly hair cortex cells. Pilomatricoma occurs usually as a solitary lesion. The face and the upper extremities are the most common sites. In general, the tumor varies in diameter from 0.5 to 3.0 cm, but it may be as large as 5 cm. The tumors may arise in persons of any age, but about 40% of them arise in children younger than 10 years of age, and about 60% in persons in the first two decades of life.[]
Nil.
There are no conflicts of interest.


A 58-year-old male without any past history of comorbid illness or addictions presented to our emergency department with a history of left-sided constricting chest pain with excessive sweating for 1 hour duration. On examination, he was anxious with a pulse rate of 108/min, regular in rhythm blood pressure (BP) of 120/80 mmHg and respiratory rate of 16/min. Systemic examination was unremarkable. Electrocardiogram was suggestive of anterior wall myocardial infarction, with ST elevation in V1–V4 and ST depression in leads 1 and avL. Echocardiogram demonstrated regional wall motion abnormality in the anterior wall. He was initially loaded with aspirin and clopidogrel. He was thrombolysed with 1.5 million units of streptokinase. His chest pain subsided, and electrocardiogram taken 90 minutes after thrombolysis showed good ST resolution. After 6 hours, he developed massive hemoptysis and breathlessness. His oxygen saturation in room air fell to 86% from an initial value of 99%. Examination revealed bilateral fine-end inspiratory crepitations in all lung fields. His jugular venous pressure was normal, and there were no signs of heart failure. A chest X-ray () revealed bilateral alveolar infiltrates. High-resolution computed tomography (HRCT) of thorax () confirmed the presence of alveolar hemorrhage. Hemoglobin fell from 14 to 10 g/dL. He had no other bleeding manifestations. He was treated with oxygen and blood transfusion. Diffusion lung capacity for carbon monoxide was markedly elevated. His condition improved symptomatically with treatment. After 1 week, his oxygen saturation in room air was 98% and his lung fields were normal on auscultation. A chest X-ray taken after 10 days showed resolution of lesions (). The tests for anti-glomerular basement membrane antibodies, antinuclear antibodies, anti-double-stranded deoxyribonucleic acid antibodies, peripheral anti-neutrophil cytoplasmic antibodies (p-ANCAs) and cytoplasmic ANCAs showed negative results. Toxicology screening and retroviral screening were negative. On follow-up after 1 month, pulmonary function tests, including diffusion lung capacity for carbon monoxide, were done, which were reported as normal. He was restarted on clopidogrel after 3 months and is asymptomatic till date.


A 41 year old woman presented with painful proptosis and diplopia and was diagnosed with euthyroid, auto-antibody-negative thyroid eye disease at an outside institution. The patient was treated with oral corticosteroids, orbital radiation, bilateral orbital decompression, rituximab, and mycophenolate mofetil over the course of a year. Despite treatment, her symptoms remained steroid-dependent. She presented to our institution for a second opinion.
At presentation, she was taking 20 mg of oral prednisone daily and 3 g of oral mycophenolate mofetil daily. Her general appearance was cushingoid. Visual acuity was 20/25 OU with correction. Extraocular movements were painful with mild limitation in all positions of gaze. Exophthalmometer readings were 21 mm OD and 22 mm OS. Although periorbital edema was present with increased resistance to retropulsion, eyelid retraction and lag were notably absent. Slit lamp and funduscopic examinations were unremarkable. Computed tomography (CT) of the orbits showed enlargement of multiple extraocular muscles with tendon involvement (Fig. , ).
In preparation for orbital biopsy, oral prednisone was tapered over one week with rebound orbital inflammation. Biopsy of the left inferior oblique, left medial rectus, left inferior rectus, and orbital fat was performed. Oral corticosteroids were resumed post-operatively.
Histopathology showed extensive inflammation and noncaseating granulomas without evidence of microorganisms or foreign bodies (Fig. ). Rheumatology consultation was obtained. Systemic sarcoidosis screening, including chest radiograph, serum angiotensin-converting enzyme (ACE), and serum calcium, was negative. Serum levels of anti-neutrophil cytoplasmic antibodies (ANCAs), anti-nuclear antibodies, interferon-gamma release, and rapid plasma reagin (RPR) were within normal limits. A diagnosis of noncaseating granulomatous orbital myositis consistent with presumed isolated orbital sarcoidosis was made.
The patient was intolerant of methotrexate but was successfully switched to azathioprine as a steroid-sparing agent. Orbital inflammation remained controlled at an eleven month follow-up visit (Fig. , ).


A 71 year old woman with history of systemic hypertension, chronic obstructive pulmonary disease, and age-related macular degeneration presented with sudden periorbital edema and painful proptosis of both eyes. “Bulging” globes, headache, and decreased vision developed approximately two weeks after a prior fall. The patient was urgently referred to our institution by an outside ophthalmologist, who started the patient on brimonidine tartrate/timolol maleate ophthalmic solution for elevated intraocular pressure in both eyes.
On examination, visual acuity was 20/80 OD and 20/200 OS with correction. Color vision was reduced on the left, with two of eight color plates read correctly. Extraocular movements were largely intact, but eyelids were significantly swollen and erythematous. Eyelid retraction and lag were absent. Exophthalmometer readings were 27 mm OD and 26 mm OS with increased resistance to retropulsion but without palpable pulsations. Chemosis was also present in both eyes. Intraocular pressure was 16 mm Hg OD and 20 mm Hg OS on ocular hypertensive medication. Funduscopy revealed small macular drusen and hyperemic optic nerves with vascular congestion bilaterally.
Given clinical suspicion of a carotid-cavernous fistula in the setting of recent head trauma, cerebral angiography was performed. No evidence of a direct or indirect carotid-cavernous fistula was found on cerebral angiogram. Magnetic resonance imaging (MRI) of the orbits revealed multiple enlarged extraocular muscles with tendon involvement bilaterally (Fig. , ). Thyroid function was normal with normal serum levels of thyroid-stimulating immunoglobulin, anti-thyroglobulin antibodies, and anti-thyroid peroxidase antibodies. With a presumptive diagnosis of idiopathic orbital inflammation, the patient was started on 40 mg of oral prednisone. The patient’s pain, chemosis, and lid edema significantly improved with corticosteroids (Fig. , ), but symptoms reappeared with steroid tapering.
Orbital biopsy was performed for a definitive diagnosis. Biopsy of the left lateral rectus muscle showed multiple noncaseating granulomas without evidence of microorganisms or foreign bodies (Fig. ). Flow cytometric analysis was negative for lymphoma. Subsequent systemic sarcoidosis work-up, including chest radiograph, serum ACE, and serum calcium, was negative. Serum levels of ANCAs and RPR were within normal limits, and a tuberculin skin test was read as negative. The patient was diagnosed with noncaseating granulomatous myositis suggestive of orbital sarcoidosis in the absence of systemic features.
The patient was referred to a rheumatologist, who recommended long-term treatment with low-dose oral prednisone for persistent symptoms. Because she experienced recurrent inflammatory symptoms with steroid tapering, the patient received 20 Gy of external orbital radiation and was able to discontinue corticosteroids two months after completion of radiotherapy.


We present a case of a 43-year-old African-American male inmate who was referred to our hospital for worsening renal failure, proteinuria, and eosinophilia, with concern of acute interstitial nephritis. His medical history was remarkable for schizophrenia, hepatitis C genotype 3a, human immunodeficiency virus Centers for Disease Control Class A3, and Stage II chronic kidney disease. His medications were tenofovir, raltegravir, and trimethoprim-sulfamethoxazole for a history of Pneumocystis jiroveci pneumonia. The family history was positive for diabetes in a grandmother who was diagnosed at a young age. Three months prior to hospitalization, the patient was noted to have thrombocytopenia. Consequently, trimethoprim-sulfamethoxazole was switched to dapsone 100 mg daily. Two months later, the patient started to experience fevers, chills, fatigue, decreased appetite, nausea, vomiting, and diarrhea (4–6 episodes/day). He lost more than 30 pounds and developed a diffuse, dry pruritic rash.
Laboratory evaluation in the patient was remarkable for leukocytosis with 20% eosinophilia, transaminitis, and further worsening in thrombocytopenia and renal function []. Nonfasting serum glucose was normal at 78 ng/dL. Urine analysis revealed moderate blood, 2+ protein (100 mg/dL) with 14–20 granular casts. Renal ultrasound revealed changes consistent with chronic renal disease with increased cortical echogenicity without obstruction.
On presentation, the patient's temperature was 36.7°C, blood pressure was 104/57 mmHg, heart rate was 98 beats/min, respiratory rate was 17 with oxygenation 100% on room air, weight was 69 kg, and body mass index was 23.1. On physical examination, he appeared cachectic and fatigued with mild temporal wasting. He had dry mucous membranes and a nonpainful thyroid gland with no bruits or palpable nodules. Skin examination was notable for a diffuse, dry exfoliative dermatitis, mostly on the chest, abdomen, palms, and soles. The remainder of the physical examination was unremarkable.
Biochemical evaluation upon admission to the hospital was remarkable for thyrotoxicosis with thyroid-stimulating hormone of 0.01 uIU/ml (nl, 0.45–4.50), free thyroxine of 3.94 ng/dl (nl, 0.60–1.30), and thyroid-stimulating immunoglobulin (TSI) of 21% (nl, 0–139). A thyroid uptake and scan was not obtained because of a recent iodinated contrast administration during whole-body computed tomographic (CT) imaging. He was also noted to be hyperglycemic with serum glucose as high as 450 mg/dl without ketoacidosis []. His hemoglobin A1c level was 5.9% (nl, 4.1–5.6), fructosamine was 259 umol/L (nl, 0–285) with an albumin of 2.4 g/dl (nl, 3.5–5.2), glutamic acid decarboxylase (GAD) and islet cell autoantibodies were undetectable, and insulin antibodies were mildly positive []. Exocrine pancreatic enzymes were not checked, but the pancreas looked normal on CT scan imaging. The patient was tested for infectious etiologies including Cytomegalovirus, Cryptosporidium, Cyclospora, Parvovirus, histoplasmosis, Clostridium difficile, and Giardia infections, all of which were negative. A renal biopsy confirmed the diagnosis of acute interstitial nephritis, predominately with lymphocytes and eosinophils. He was diagnosed with DRESS, likely due to dapsone. Dapsone was stopped and the patient was started on a tapering dose of prednisone and given beta-blocker and multiple daily injections of insulin. The daily insulin requirement was 0.4 units/kg/day, which increased to 0.7 unit/kg/day on prednisone.
On follow-up, all of his symptoms including the rash had resolved within a few weeks. Leukocytosis, thrombocytopenia, eosinophilia, transaminitis, hyperbilirubinemia, and thyrotoxicosis had also resolved []. GFR returned to baseline, and hemoglobin A1c level a year after hospitalization was 6.1%. The daily insulin requirement decreased to 0.25u/kg/day (insulin NPH twice daily).


A 93-year-old woman presented to the emergency department with upper abdominal pain that began 5 days prior. She had been taking oral antihypertensive agents and had no other underlying diseases. Initial blood pressure, heart rate, respiratory rate, and body temperature were 140/90 mm Hg, 115 beats per minute, 20 cycles per minute, and 38.5°C, respectively. During physical examination, tenderness was noted in the right upper quadrant of the abdomen. Laboratory data were as follows: white blood cell count, 29,710 cells/μL; hemoglobin level, 9.9 g/dL; platelet count, 163,000/mL; aspartate aminotransferase, 341 IU/L; alanine aminotransferase, 186 IU/L; γ-glutamyltransferase, 586 IU/L; alkaline phosphatase, 212 IU/L; total bilirubin, 1.70 mg/dL; and high-sensitivity C-reactive protein, 5.67 mg/dL. Abdominal computed tomography scan showed common bile duct dilatation with multiple stones in the bile duct and gallbladder. Acute cholangitis and acute cholecystitis due to gallstones were diagnosed, and ERCP was performed. While inserting the lateral endoscope from the duodenal bulb to the second portion of the duodenum, the existence of a perforation at the descending limb of the duodenum was suspected. We immediately switched to a forward endoscope that had a transparent band apparatus and attempted to confirm the presence of the perforation at the suspected site. A 2.0-cm oval-shaped perforation at the lateral duodenal wall was found, and peritoneal structures such as the mesenteries were observed in some areas of the perforation. A diagnosis of ERCP-related perforation of the lateral duodenal wall was made, and repair of the perforation orifice with EBL was planned. However, single band ligation is considered to be ineffective for the repair of a 2.0-cm perforation; hence, double band ligation was attempted. The first EBL was performed at the proximal edge of the perforation orifice, and two-thirds of the perforated site were repaired along with the adjacent normal duodenal structures. A second EBL was performed that included the contents covered by the first ligation. Considering the possibility of the existence of a residual perforation, an endoclip was applied at the distal end of the perforation. The diagnosis and repair of the perforation were performed within 10 minutes ().
Although free air was not present, an air shadow was observed near the right kidney and retroperitoneal space on plain abdomen radiography performed after the procedure (). The patient complained of abdominal pain accompanied by persistent right upper quadrant tenderness, and treatment comprising the administration of analgesics, antibiotics, and intravenous fluids and bowel rest was initiated. Abdominal pain improved after 24 hours. During the 1-week hospital stay, abdominal pain and fever subsided, and other laboratory tests results were within the normal limits ().


A 35-year-old gentleman presented with clinical features suggestive of raised intracranial pressure lasting for 2 weeks. On clinical examination, he was conscious and oriented. On general physical examination, he had no palpable swelling anywhere else in the body. Neurological examination revealed left-sided homonymous hemianopia with bilateral papilledema. Rest of the examination was within normal limits. Hematological and biochemical parameters were within normal limits.
Cranial computer tomography (CT-plain and contrast) revealed a right parieto-occipital mass lesion. It was isodense on plain scan with an area of hyperdensity situated posteriorly, suggesting calcification. The lesion was enhancing heterogeneously on contrast with significant perilesional edema []. Magnetic resonance imaging of the brain (plain and contrast) revealed a lesion, which was iso- to hypo-intense on T1-weighted and iso- to hyper-intense on T2-weighted. Lesion was again enhancing heterogeneously on contrast and measured 5.2 cm × 4.4 cm × 4.2 cm [ and ]. He underwent right parieto-occipital craniotomy and gross total decompression of the lesion. Peroperatively, the lesion was found to be grayish white, firm to hard, and highly vascular. It had a clear plane of demarcation from the surrounding normal parenchyma.
Histopathological examination revealed a neoplasm which was sharply demarcated from surrounding normal parenchyma by reactive gliosis []. The tumor cells were arranged in tubular and cribriform pattern [Figure –], at places forming ductular structures [] lined by stratified cuboidal cells. Fascicular pattern was not seen. The tubules and acini contained hyaline [] or mucinous material []. The hyaline material was seen both surrounding the tubules as well as within the tubules. Tubules and acini were highlighted by periodic acid–Schiff stain [, inset]. The tumor was vascular with several hyalinized vessels. The tumor cells expressed cytokeratin (pan cytokeratin) but were negative for chromogranin, thus excluding neuroendocrine tumors. These histological features were characteristic of ACC. No perineural or perivascular spread was evident. Glial fibrillary acidic protein was negative.
A detailed evaluation including a chest X-ray, ultrasonography of abdomen, and whole-body single photon emission CT was performed to look for the primary site of the lesion. However, no primary site could be identified. Patient received postoperative radiotherapy. He showed good clinical improvement and remains recurrence free at 18 months postoperatively [].


A 27-year-old woman was involved in a frontal impact car collision with a pole; she was travelling in the front passenger seat and was not wearing a seat belt. Prolonged extrication was required. Intubation and initial fluid resuscitation were performed at the scene. On arrival at the Emergency Department at the Trauma Center in Sosnowiec, the patient was unconscious, her Glasgow Coma Scale score was 7, and bag ventilation was in progress. Chest examination revealed bilateral crepitus, muffled heart sounds, and massive subcutaneous emphysema of the chest, neck, and head; this precluded assessment of the trachea and neck veins. Other injuries included pelvic, femoral, and ulnar fractures. Due to hemodynamic instability, fluid resuscitation was continued and catecholamine infusion was started. Bilateral needle pleurocentesis was immediately performed without significant air outflow. Total body computed tomography (CT) revealed multiple rib fractures, a bilateral pneumothorax, tension pneumopericardium with a 35-mm air layer, and pneumomediastinum with extensive subcutaneous emphysema (). Additionally, rupture of the left main bronchus was suspected. Emergent pericardial needle aspiration (about 90 ml of air and bilateral pleural drainage) resulted in circulatory stabilization. Repeat chest CT revealed reduction of gas space in the pericardium to 12 mm without a tension effect and with gradual lung expansion. The patient was then transferred to the intensive care unit.
On the second post-trauma day, elective bronchoscopy revealed a complete transection of the left main bronchus (3–4 cm from the carina) with a slight constriction of the lumen caused by the emerging left pulmonary artery. This arterial vessel constituted part of the artificial canal (about 3 cm in length) created by other surrounding tissues (e.g., pleura, lung) and ended at the amputated distal parts of the bronchial tree (visible on deep exploration) (). On the next day, the patient’s condition worsened; hypoxemia occurred and 100% oxygen ventilation was required. Additionally, the chest expansion on the left side diminished along with a decrease in breath sounds. There was still no air leakage through the pleural drains. Emergent bronchoscopy conducted through the artificial canal into the amputated part enabled the aspiration of bloody mucus from the obstructed lobar bronchi. The intervention resulted in significant improvement in ventilation, a rise in the PaO2/FiO2 ratio, and improved chest expansion and breath sounds on the left side. A few hours later, the patient underwent a left posterolateral thoracotomy with successful reconstruction of the bronchus.
For several days after the surgical procedure, multiple bronchofiberoscopies were required to clear the bronchial tree and evacuate the discharge. At the bronchial repair site, mucous membrane edema and bronchial constriction gradually regressed (). The patient was extubated on the 19th day after the trauma; however, for a few days, proper oxygenation was maintained by noninvasive mechanical ventilation. The patient was discharged from the hospital 32 days after the trauma, and the 3-month follow-up yielded positive results.


An 18-year-old male presented to us with the complaint of pain and swelling around the right scapula since 20 days, which was gradually increasing in size and developed fever since 2 days. On history, patient was on AKT for pulmonary TB since 6 months which was diagnosed based on clinical history of a cough and chest X-ray finding and blood report showing raised erythrocyte sedimentation rate (ESR); sputum examination was normal. Considering it as possible tubercular swelling AKT was continued but after 3 days patient also developed left ankle swelling and pain difficulty in walking. Repeat blood investigation was done and magnetic resonance imaging (MRI) of both right shoulder and left ankle was done (, , ). Blood investigation showed raised ESR 73 mm, C-reactive protein (CRP) positive 96. MRI reported as osteomyelitis of scapula and lower end tibia. We aspirated swelling and sent for culture and sensitivity and histopathology considering it as possible multi drug resistant (MDR) TB. On microbiological examination, cryptococcal antigen latex agglutination test came positive. The patient was further investigated for immunology and serology. CD4 percentage 16% (normal = 27-51%) and absolute 345 (normal = 448-1611 cells/uL). Cerebrospinal fluid (CSF) culture/India ink was negative. Cryptococcus antigen latex test negative for CSF. HIV test was negative.
The patient was taken for surgery of debridement saucerization and bone grafting of dead space for tibia and debridement of scapular swelling and removal of loose fragments. Patient was started on amphotericin B injection and tab flucytosine for 14 days and on fluconazole for maintenance phase for 9 months.
Now 2-year follow-up patient is doing well. No complication or recurrence. CD4 count normal, also ESR and CRP.


A 30-year-old man presented to the emergency trauma room following a motor vehicle collision in which he was the front-seat unrestrained passenger. At the time of presentation, the patient was vitally stable, conscious, alert, oriented with a Glasgow Coma Scale 15/15 []. A cervical collar and spinal board were applied by the paramedics at the scene. The trauma surgery team assessed the patient initially and implemented the advanced trauma life support protocol []; he was deemed to be hemodynamically stable with no other systemic injuries. The patient was, however, complaining of moderate left hip and thigh sharp pain as well as left shoulder pain. On examination, he was noted to have abducted, shortened and internally rotated left lower limb. Active and passive movements of the left thigh were not possible due to pain with no neurovascular deficits detected distally. There were no significant examination findings of the left shoulder and upper limb. X-rays of the pelvis and left femur revealed a posterior fracture dislocation of left femoral head with an ipsilateral femoral mid-shaft fracture and a computed tomography (CT) scan of the pelvis showed large and small fracture fragments within the acetabulum originating from the medial aspect of the femoral head (). A shoulder X-ray also revealed a non-displaced left scapular body fracture. An attempt of left hip closed reduction was made while the patient was still in the emergency department and it was, however, unsuccessful.
After being cleared by the trauma team, the patient was admitted to the orthopedic service with a working diagnosis of left posterior hip dislocation with a Pipkin Type 1 ipsilateral femoral head fracture [] and an ipsilateral femoral shaft fracture. The patient was taken to the operating theater for closed versus open reduction and intramedullary nailing of the left femur. Within 5 h of his injury, the patient underwent closed reduction of the left hip with the aid of a temporary external fixator applied on the femoral shaft proximal to the fracture (). The external fixator rod was used as a handle, and the reduction was successful after the first attempt, as confirmed by the C-arm and CT reconstruction (). Subsequently, the external fixator was removed, and intramedullary nailing of the left femur was carried out. After completion of the procedure, the left hip was examined and found to be stable with the femoral head fragment not affecting the movement. The patient was discharged after 3 days with a clean, dry wound and full weight bearing ambulation as tolerated. Unfortunately, he was lost to follow-up and attempts to contact the patient were unsuccessful.


A 4-year-old boy presented with soft tissue painless swelling, insidious in onset, gradually progressive over the side of the left knee joint. There was no history of any associated significant trauma or inflammation in recent past.
The physical examination revealed a palpable, well-defined 3 cm × 1.5 cm, subcutaneous swelling over the posteromedial aspect of the left knee joint. The swelling was non-tender and not moveable. It was not adherent to the overlying skin. There was no functional or neurological deficit associated (, ).
X-ray of the knee joint was unremarkable (, ). Ultrasonography (USG) revealed a hypoechoic well-defined cystic lesion with clear contents is visualized on the antero-medial aspect of the left knee over the semitendinosus tendon. The lesion measures 25 mm × 10 mm. The lesion is causing splaying of the gracilis and sartorius muscles near knee; the lesion extends lower down and posteriorly and appears to fuse close to pes anserine insertion near the pulsation of the medial geniculate artery (, ). Doppler USG showed no evidence of vascular involvement.
At surgery, posteromedial longitudinal incision was taken centered over the swelling. Swelling was exposed by blunt dissection. There was no sign of any tendon inflammation or adhesions. Grossly, the mass appeared completely encapsulated and contained in the semitendinosus tendon. The tendon sheath was incised longitudinally, and the swelling was delivered intact after dissecting it from the tendon taking care not to damage the tendon. On incising, the mass a transparent gelatinous fluid spilled out indicating a ganglion cyst. The tendon sheath was repaired. The wound was closed in layers. The skin wound healed with the secondary intention (). Follow-up USG was done to confirm complete removal of the ganglion cyst (, ).


A 28-year-old married, uncircumcised male presented with 1 month history of noticing a reddish patch over inner preputial mucosa. It was increased in size over 1 month associated with mild pruritus. Patient had no history of multiple sexual partners, no co-morbid illness, and no previous similar complaint.
On clinical examination, there was 1 cm nontender well demarcated reddish nodular lesion involving inner preputial and adjacent part of glans. There was no active discharge and no bleeding on touch seen [].
Inguinal lymph nodes were not palpable. A punch biopsy was performed. It showed focal inflammation in surface epithelium [], subepidermal connective tissue shows dense lymphoplasmacytic infiltration predominantly plasma cells. Focal areas of thinned out epidermis with erosions and forming macrophages were seen confirming diagnosis of Zoon's balanitis [].
Patient opted for topical tacrolimus over circumcision. Tacrolimus 0.1% was advised for twice daily application for 6 weeks. Complete resolution of lesion was achieved [Figures and ]. We have followed the patient for 6 months. There was no recurrence of lesion by the time.


A 69-year-old male was admitted to our intensive care unit from the emergency department, where he had presented with septic shock and bloody diarrhea for the last three weeks. His medical history included hypertension, cardiomyopathy, chronic obstructive pulmonary disease and a recently diagnosed laryngeal carcinoma (T3N0) for which curative treatment was planned. Until admission, he had lived independently at home. Two months before admission, he had used amoxicillin/clavulanic acid as treatment for laryngitis. On admission, he had a confused mental state, hypotension of 77/28 mmHg, a temperature of 35.6°C and diffuse abdominal tenderness on physical examination. Laboratory tests showed increased inflammation parameters (leucocytes 38.9 × 109/l; C-reactive protein 318 mg/l), renal insufficiency (creatinine 534 umol/l), a low albumin (23 g/l) and a normal lactate (1.6 mmol/l). Upon admission, treatment was started with broad-spectrum antibiotics. An abdominal CT-scan showed diffuse colonic wall thickening with a mucosal enhancement pattern and infiltration, suspect for colitis. C. difficile was isolated from a stool sample (PCR-ribotype 001). With a diagnosis of severe CDI, antibiotic treatment was switched to oral vancomycin in combination with metronidazole intravenously. This did not result in clinical improvement and after nine days of therapy, the patient was still dependent of vasopressor support, and developed progressive abdominal distension, delirium and metabolic acidosis. A surgical consultation followed to explore the possibility of colectomy. At the same time, the curative potential of FMT was considered. After weighing the options, a decision was made to treat the patient with FMT, which was delivered through a nasoduodenal tube, 12 days after admission. One day before FMT, both vancomycin and metronidazole were stopped. However, because of his poor clinical condition and the ensuing risk of not treating his severe CDI with antibiotics on the day before FMT, the decision was made to continue antibiotic treatment with oral fidaxomicin, starting the day before FMT, and to continue this treatment during and after FMT. From the fourth day after FMT, the patient started to improve clinically, with a marked decrease in abdominal distension. Feces polymerase chain reactions were negative for C. difficile on days three and 12 after FMT. Fidaxomicin was continued until nine days after FMT. The patient could be discharged from the ICU 14 days after FMT. He did not develop recurrent CDI, despite several antibiotic treatments post FMT for various indications.


A 73-year-old Caucasian male smoker was admitted to the hospital with progressive dyspnoea due to multifocal pneumonia superimposed on underlying pulmonary fibrosis. He rapidly developed acute respiratory distress syndrome and was intubated and put on mechanical ventilation. Bronchoscopy revealed diffuse alveolar hemorrhage. He also developed non-oliguric acute kidney injury. Laboratory tests demonstrated urea nitrogen 97 mg/dL, creatinine 2.35 mg/dL (baseline 0.8 mg/dL) and potassium 4.4 meq/L. Urinalysis revealed 288 erythrocytes/high power field (hpf), dysmorphic erythrocytes, 2 white blood cells/hpf, dense, coarse granular casts and 1–2 erythrocyte cast/hpf. C-ANCA was positive but anti-PR-3 antibody was negative (optical density 0.032). Anti-MPO antibody was positive (optical density 111.0; cutoff 0.244). Renal biopsy revealed necrotising and crescentic glomerulonephritis with 50% glomerular involvement with necrosis and crescents (), arterial and arteriolar nephrosclerosis, intratubular casts with densely packed red blood cells ( and (c)). Immunofluorescence showed nonspecific staining of IgG, IgM, IgA, C3, C1Q, albumin, kappa and lambda. Treatment with steroids, cyclophosphamide and plasmapheresis resulted in improvement in renal function. Patient did not require renal replacement therapy. His total hospital length of stay was 30 days and time on mechanical ventilation was 12 days.


A 26-year-old female with Burkholderia cenocepacia presented with a two-month history of worsening fixed purpuric rash on the lower legs (). Skin biopsy confirmed leucocytoclastic vasculitis. She was treated with dapsone initially without improvement. On commencement of oral prednisolone, there was minimal improvement, which remained stable. Following this, she was managed with topical protopic 0.1%.


A 23-year-old female with B. cenocepacia developed an erythematous maculopapular rash on the lower legs with associated arthropathy of her finger joints. This was clinically felt to be reactive leucocytoclastic vaculitis and a biopsy was not conducted. The vasculitis did not respond initially to oral prednisolone, and she was then commenced on azathioprine and the rash responded to this. Two years later, she died following a severe infective exacerbation of her chest.


A 32-year-old male with a history of B. cenocepacia and Burkholderia multivorans developed an erythematous rash on the lower legs associated with an infective exacerbation of his chest. The rash resolved leaving grey discolouration but would occasionally flare up in the summer months. The biopsy showed features of capillaritis, and he was managed with topical steroids and compression hosiery.


A 26-year-old female with B. cenocepecia presented with a history of recurrent small joint pains in association with intermittent painful erythematous nodular rash on the lower legs. This came on at the onset of an infective exacerbation of her chest. Biopsy confirmed erythema nodosum. Other causes of erythema nodosum were excluded, and she did not respond to Non-steroidal anti-inflammatory medications. Her symptoms continue to relapse in association with infective exacerbations of her chest.
Of the remaining patients (n = 3), one presented with an erythematous rash on the lower legs and another had a purpuric rash on the lower legs and both were diagnosed clinically as leucocytoclastic vasculitis by a dermatologist. The third patient presented with purpuric areas and blistering of the lower legs which was confirmed as leucocytoclastic vasculitis on biopsy.
Overall, two patients had biopsy proven leucocytoclastic vasculitis, three were clinically diagnosed with vasculitis but did not have biopsy confirmation and two had biopsies, which revealed other pathologies (erythema nodosum and capillaritis), giving an overall prevalence of cutaneous vasculitis in patients with Burkholderia cepacia complex of 16.7%. Out of the five patients with vasculitis, three had a raised ESR/CRP ratio.
There was no evidence of renal involvement in any patient – all had a negative urine dipstick and normal serum creatinine. The antinuclear antibody and antineutrophil cytoplasmic antibody were negative in all patients apart from one with biopsy proven leucocytoclastic vasculitis who had a transiently positive antineutrophil cytoplasmic antibody. Antistreptolysin O titre was tested in only two out of the five patients and was negative.
Mean forced expiratory volume1 was 30% (range: 18–55) of the predicted value. In four patients (57%), the forced expiratory volume1 was lower at the time of presentation with rash compared to the baseline forced expiratory volume1 values for the previous two years.
All patients received antibiotics for a presumed pulmonary exacerbation at onset of the rash. Three patients received specific treatment for vasculitis (prednisolone ± additional immunosuppressive) after non-resolution of the rash.


An 11-year-old boy was admitted to our hospital with the complaint of shortness of breath. Two-dimensional echocardiography revealed a large malaligned perimembranous ventricular septal defect (VSD) with bidirectional shunt. A low infundibular and right ventricular outflow tract (RVOT) muscle bundle was detected causing severe RVOT obstruction with a maximum pressure gradient of 64 mmHg. Right coronary cusp prolapse was present causing mild aortic regurgitation. Trivial tricuspid regurgitation was present. The other important findings were: situs solitus, levocardia, normal systemic and pulmonary venous return, good sized and confluent branch pulmonary arteries, intact interatrial septum, atrioventricular-ventriculoarterial concordance, good biventricular function, left arch, absent major coronaries surrounding RVOT, absent coarctation of aorta and absent persistent left-sided superior vena cava (SVC).
The patient was diagnosed with “double chambered right ventricle (RV) with VSD.” An intracardiac repair comprising of RVOT bundle resection, pulmonary valvotomy (pulmonary valve was preserved), and “Sauvage Dacron” patch closure of VSD were done under standard general anesthesia technique. Intraoperative course was uneventful. Systemic cooling to 30°C along with del Nido cardioplegia (500 ml) at 3°C were used for myocardial protection. For an institution of CPB 16Fr DLP aortic, 16Fr DLP (SVC) and 18Fr DLP (inferior vena cava) cannulas, Minimax Plus® Oxygenator (Medtronic), PerfX® arterial filter with 40 μm Polyester screen filter efficiency (B. L. Lifesciences) were used. Mean perfusion pressure was maintained around 50-60 mm of Hg. Standard de-airing protocol was followed (de-airing through patent foramen ovale and aortic root suction). Total CPB and aortic cross-clamp time were 72 and 43 min. The patient came off CPB with moderate inotropic support (dobutamine at 5 μg/kg/min and adrenalin at 0.05 μg/kg/min). RV/left ventricle pressure ratio was found to be 0.6. Post-CPB heart showed normal sinus rhythm. The patient was shifted to Intensive Therapy Unit after on-table tracheal extubation. However, soon after regaining consciousness, he complained about the total loss of vision (without light perception) in both the eyes. A detailed ophthalmologic examination including direct and indirect ophthalmoscopy could not detect any ocular damage involving both the eyes. After consulting Neurologist and ophthalmologist, plain computed tomography (CT), and magnetic resonance imaging (MRI) of the brain were done on the same day. The CT [] did not find any obvious acute focal abnormality. However, MRI brain [] at different secretions revealed multiple regions of acute ischemic infarct in bilateral occipitoparietal and right frontal lobes. The T2-weighted image [] at the level of the orbits shows normal signal within the optic nerves bilaterally. A diagnosis of CB was made and conservative medical management was adopted. A gradual recovery of vision was noted as on a postoperative day (POD) 1 he was able to identify light in both the eyes and on POD 3 he was able to detect hand movement in both the eyes. On POD 9, he was able to identify faces of his parents and known people. He was discharged from the hospital on POD 10 and was explained about the slow progress of visual recovery.
On follow-up visit after 1 week following discharge from the hospital, he was found to have hazy vision with poor comprehension, and his vision acuity was found to be 6/12 (with ‘E’ chart) in both the eyes. One week later, on the second follow-up visit, his vision was re-examined for acuity and was found to be 6/9 in both the eyes. However, difficulty in visual comprehension persisted. One month later, on the third follow-up visit, his vision was re-examined which revealed improved vision with the restoration of visual comprehension (6/9 both eyes with English alphabets and N6 in both eyes at 25 cm distance with Hindi alphabets).


A 30-year-old male presented with a low-intensity, poorly localized headache for several months. The family members noticed disorganized behavior for a week prior to his hospitalization without any associated history of neurological deficit or seizures.
Non-contrast computed tomography (CT) scan showed a large fat density mass of size 54 × 47 mm, with a few eccentric calcific foci in the frontal region involving the anterior part of corpus callosum, causing effacement of frontal horns of lateral ventricles and suggesting a diagnosis of corpus callosum lipoma.
Magnetic resonance imaging (MRI) revealed a lesion which was hypointense on T1-weighted (T1W) images and hyperintense on T2-weighted (T2W) images without any contrast enhancement, suggesting the probable diagnosis of epidermoid cyst. Neurosurgeons performed endoscopy-guided procedure and the tissue was removed and sent for histological examination [].
Gross examination showed multiple gray-brown soft tissue pieces measuring 40 × 30 × 10 mm.
Microscopic examination revealed mature stratified squamous epithelium along with adnexal structures (hair shaft and sebaceous glands) and keratinous material, confirming the diagnosis of dermoid cyst []. Postoperative course of the patient was unremarkable and he was discharged without any neurological deficit.


A 62-year-old Japanese female with a 32 pack-year smoking history was diagnosed with primary lung adenocarcinoma lacking epidermal growth factor receptor-mutation in the right lower lobe, with a clinical stage of T2N2M0, stage IIIA. It was unknown whether her lung cancer harbored anaplastic lymphoma kinase-rearrangement. She received chemoradiotherapy as first line therapy. A year later, her lung cancer relapsed with a single brain metastasis and multiple lung metastases. She subsequently received gamma knife radiotherapy and six regimens of chemotherapy for six years and three months, followed by nivolumab treatment. After receiving seven cycles of nivolumab (12 weeks later), she presented with hemoptysis. Chest computed tomography (CT) revealed that the lung metastasis in the middle lobe had slightly enlarged within the range of stable disease according to Response Evaluation Criteria in Solid Tumors, version 1.1 (Fig a,b). Bronchofiberscopic examination revealed that her right basal bronchus was responsible for hemoptysis (Fig c). She received transcatheter arterial embolization therapy against the right lower lobe bronchial artery. In addition to the endoscopic gross view, positron emission tomography-CT revealed accumulation of 18F-fluorodeoxyglucose in the lung metastasis, and her carcinoembryonic antigen serum level was increased (Fig b,d). Based on these factors, we considered her lung cancer to be clinically progressive. The patient subsequently received chemotherapy with paclitaxel and S−1. Four weeks later, when 20 weeks had passed since the initiation of nivolumab, she visited our hospital with a fever and cough. CT examination revealed infiltrative shadows in the bilateral lung fields mainly in the right, in addition to enlargement of the lung metastasis (Fig c), which suggested ILD with a radiologic pattern of cryptogenic organizing pneumonia and progressive disease by RECIST v1.1. Chemotherapy was discontinued. She was admitted to our hospital, and bronchofiberscopic examination revealed infiltration of the lymphocytes without any cancer cells or bacteria in the right lung segment 1. We diagnosed grade III ILD, and 1000 mg of methylprednisolone was administered daily for three days. She then received 60 mg of prednisolone daily, which was altered to dexamethasone because of hypokalemia induced by prednisolone. The corticosteroid therapy improved the infiltrative shadow (Fig d). Surprisingly, during the tapering of dexamethasone, the lung metastasis also decreased in size, and reduced even further after the infiltrative shadows improved (Fig a–e). The anti-tumor effect was considered a partial response through pseudoprogression. Her carcinoembryonic antigen serum level also decreased (Fig e). Nineteen weeks after the initiation of paclitaxel and S−1, her lung cancer became progressive disease (Fig e).


A 25-year-old young female presented with ingestion of 5–6 pinches (around 2 g) rodenticide poison containing 3% phosphorous in the form of a paste. Multiple episodes of vomiting, abdominal pain, and hematemesis were the complaints on presentation. On evaluation, the patient had jaundice on the second day of admission. There was no evidence of hepatic encephalopathy. Serology for hepatitis B surface antigen, hepatitis C virus, dengue, Leptospira, and hepatitis was negative. Chest X-ray revealed mild right pleural effusion []. Abdominal sonography done on day 2 showed mild hepatomegaly and diffuse gallbladder (GB) wall edema []. Unenhanced computed tomography (CT) abdomen showed multiple patchy areas of homogeneous hyperattenuation (30–40 HU) in segment IVb, periportal region, and in the GB fossa [Figures and ]. GB wall appeared edematous []. Postcontrast, these regions showed enhancement (90–95 HU) suggesting patchy parenchymal hemorrhages of the liver. There was breach in the continuity of wall of second part of duodenum seen along the medial aspect with a contained pocket of air (pneumoperitoneum) suggesting contained duodenal perforation [], with adjacent duodenal wall thickening [] which could represent the site of viscus perforation and associated dirty fat density and local fluid between the duodenum and the pancreatic head []. Her total leukocyte count was 1.800 (109/L) on the day of admission, which improved to 4.400 (109/L) on the 7th day. She developed a spike of fever on day 5 of hospitalization. She had a temperature of 101.5°F, tachycardia (pulse of 150 counts/min), a respiratory rate of 30 counts/min, and a blood pressure of 100/70 mmHg. Her platelet counts were 1.53 (109/L) on the day of admission, which decreased daily from 1.28 (109/L) on the second day to 0.80 (109/L) on day 7 of admission. Platelet counts reached normal levels on day 11. Her renal function tests and electrolytes were normal (sodium 137 mmol/L, potassium 3.5 mmol/L, and chloride 100 mmol/L). Urine examination showed the presence of 48–50 red blood cells and 8–10 pus cells per high power field. Horseradish peroxidase 2 was negative. There was no hypoglycemia (blood glucose 108 mg/dl). Arterial blood gases revealed pH – 7.11, PCO2-20.6, PO2-75.4, and HCO3-6.3 suggesting metabolic acidosis. No cardiac dysrhythmias or electrocardiography changes were demonstrated. Intake and output were strictly monitored, and blood glucose was measured 6 hourly. Metabolic acidosis was corrected by sodium bicarbonate infusion. Central line insertion and endotracheal intubation were done, and the patient was treated with ventilatory support. Prothrombin time (PT) started deranging from the second day of admission with a crest of 38 s and international normalized ratio (INR) 2.98 noted on day 5. Total bilirubin levels mounted up to 10.5 mg/dl on day 2 of admission with liver enzymes serum glutamic pyruvic transaminase (SGPT) and serum glutamic oxaloacetic transaminase (SGOT) peaking to 1722 U/L and 2954 U/L, respectively. She was treated with intravenous (IV) Vitamin K, IV fluids, IV antibiotics, and fresh frozen plasma. In view of impending FHF, he was given N-acetyl cysteine, a loading dose of 150 mg/kg IV over 1 h, followed by 50 mg/kg IV over 4 h and 100 mg/kg IV over 16 h. Patient's PT/INR became stabilized. After bleeding parameters were stabilized, she was discharged from the hospital on day 15. On follow-up after 2 weeks, INR remained 1.11 with SGPT 29 (U/L), SGOT 27 (U/L), and total bilirubin 1.8 mg/dl. Laparoscopic surgical management of the perforated duodenal ulcer was done with Graham omental patch and a thorough abdominal lavage, and patient was started on proton-pump inhibitors.


This 15-year-old male had experienced symptoms for 3 weeks prior to admission, which were initially limited to persistent bilateral frontal headaches. Two weeks before presentation, he began to complain of peripheral vision loss in his left eye, which at one point became bilateral then returned again to just left-sided visual field deficits. His symptoms again worsened in the 4 days leading up to admission, when he began to complain of increased headache severity, along with new ataxia, left-sided hemiparesthesias, and worsening vision loss. His speech became rambling and incoherent, and he was generally sleepy. On the day he was admitted, all of his symptoms were at their peak severity.
Upon admission, the patient was sleepy but alert and oriented. His eye examination revealed decreased peripheral vision bilaterally and a lack of convergence. He reported left-sided facial numbness, and he demonstrated difficulty with finger-nose pointing and heel-to-shin on his left side. Strength was decreased in the left upper and lower extremities. Magnetic resonance imaging (MRI) revealed multiple areas of ischemic infarction throughout the posterior circulation of the brain. The lesions were seen in the left lobe of the cerebellum, the right and left occipital lobes, and thalamus bilaterally. They appeared to be of various ages, which suggested a stepwise and stuttering course of infarction. Selected MRI images are presented in . The MRI also revealed an abnormal bony process off the left occipital condyle that appeared to be in contact with a small left vertebral artery. It was felt that the bony spicule was likely traumatizing the left vertebral artery resulting in emboli showering to areas of the brain in the posterior circulation and leading to infarctions. The MRI findings prompted magnetic resonance vascular studies with 3-dimensional reconstruction. These images showing the left vertebral artery and the occipital bony spicule are presented as . Echocardiogram and coagulation studies were performed to rule out alternative causes of strokes, and these tests were all negative. The patient was immediately started on aspirin and placed in a cervical collar due to suspicion of vertebral artery dissection and distal embolization.
Once it was decided that the bony spicule was most likely causing vertebral artery dissection and distal embolization, the decision was made to schedule diagnostic transfemoral angiography under general anesthesia with potential endovascular treatment to confirm the adequacy of collateral circulation. Preoperative angiography showing a nondominant left vertebral artery and a dominant right vertebral artery is pictured in , along with postoperative angiography demonstrating sufficient collateral circulation. An alternative option could have been to attempt a conservative trial of anticoagulation and a cervical collar, but it was felt that the risk of further strokes outweighed the risk of the procedure. The patient underwent the endovascular occlusion of the left vertebral artery and tolerated the procedure without complication. The patient had no further strokes after his procedure. He returned to clinic 6 months after his procedure, and his only lingering deficit was homonymous hemianopia and mild blurring of his vision in his far right visual field.


A 3-month-old boy was referred to neurosurgery when a subcutaneous bulge in the lower lumbar region was incidentally noted. Initial workup included an ultrasound of the region that was concerning for spinal dysraphism, including sacral agenesis and an associated intraspinal mass. These findings prompted a lumbosacral magnetic resonance image (MRI), which confirmed the diagnosis of lipomyelomeningocele. The patient was clinically asymptomatic, with normal strength in his lower extremities, no evidence of hydrocephalus, and normal bowel and bladder function. Because he was meeting developmental milestones, he was managed observantly with annual clinical exams, which remained normal. At 3 years of age, he underwent urodynamic studies, which were unremarkable, and he was able to successfully toilet train. At around this time, his parents reported transient morning stiffness in the back and lower legs, which would resolve by the afternoon. This prompted a repeat MRI [], which demonstrated the development of a syrinx in the lumbar region of the spinal cord. Surgical debulking and untethering of the lipomyelomeningocele was discussed with the parents, along with the associated risks and potential benefits, and, ultimately, the decision was made to continue expectant management. At 5 years of age, he remains clinically asymptomatic and continues to meet developmental milestones.


An 11-day-old girl with an uncomplicated birth was noted by her parents to have a lump on her lower back, prompting further workup by her pediatrician. A physical exam revealed a 4–5 cm soft, nontender mass in the lumbar spine with hyperpigmented changes []. An ultrasound was performed, which showed the absence of sacral lamina and dorsal elements with an associated intraspinal mass and bilateral hydronephrosis. She subsequently underwent an MRI showing a spinal dysraphism of the sacrum associated with a large cystic intraspinal mass, concerning for a lipomyelomeningocele versus a terminal lipomyelocystocele. Her motor exam showed normal strength in both lower extremities but poor deep tendon reflexes and a slightly distended abdomen. Further urological workup revealed the presence of grade 4 vesicoureteral reflux for which she was started on a clean intermittent catheterization protocol. After a thorough discussion about the risks and benefits of operative intervention, she was taken to the operating room (OR) for debulking of the lipomatous mass and untethering. Electrical stimulation and neuromonitoring were used to monitor function. The dural tube was reconstructed using a synthetic patch duroplasty. No cyst was encountered during the debulking. There were no intraoperative complications, however, 1 week postoperatively she developed a cerebrospinal fluid (CSF) leak, which precipitated a Klebsiella wound infection. She returned to the OR for irrigation and debridement, placement of a lumbar drain, and revision of the dural closure. Postoperatively, she remained intubated and prone for 1 week, at which point the lumbar drain was removed and she was extubated. She had no further leakage from the wound and was discharged approximately 5 weeks after her first surgery. At 18-months’ postoperative follow-up, her wound was well healed, and she retained normal strength in her bilateral lower extremities. She progressed to grade 5 vesicoureteral reflux with moderate hydronephrosis without urinary tract infections, managed by clean intermittent catheterization every 3 hours.


A 43-year-old Japanese man presented with decreased vision in his right eye that had lasted for 7 days prior to the hospital visit. His ocular history consisted of the diagnosis of ODP at another hospital several years ago. The best-corrected visual acuity was 20/150 and intraocular pressure was 16 mm Hg in the right eye. Slit-lamp examination was unremarkable in both eyes, but fundoscopy disclosed a bullous retinal detachment involving the macula and superotemporal area in his right eye. Optical coherence tomography (Spectralis OCT-Heidelberg Engineering, Heidelberg, Germany) revealed retinal detachment without retinoschisis connected to the ODP (Fig. ).
After informed consent was received from the patient, he underwent combined phacoemulsification with in-the-bag implantation of an intraocular lens and a 25-gauge pars plana vitrectomy. After the core vitrectomy, posterior vitreous detachment was initiated by suction over the optic disc using a vitreous cutter. Triamcinolone acetonide was used during surgery to visualize the vitreous. Fluid-gas exchange was performed with aspiration of SRF through an intentional drainage hole at the superotemporal quadrant. Finally, intravitreal air was exchanged with 20% sulfur hexafluoride gas. Neither ILM peeling nor laser photocoagulation temporal to the disc was performed. The patient was instructed to remain in the prone position. Two days postoperatively, the migration of a small amount of gas in the subretinal space between the optic disc and the central macula was noted. The migrated gas resolved spontaneously in several days. OCT revealed an apparent connection between the ODP and the vitreous cavity (Fig. ).
Fifteen days after the initial surgery, macular detachment recurred and a second surgery was planned. The surgery consisted of removal of residual intraocular gas, SRF drainage with another intentional retinal hole near the first one, endophotocoagulation to the temporal peripapillary retina, and silicone oil tamponade. The patient was kept in the prone position. Five days after the second surgery, numerous silicon oil droplets were noted in the subretinal space associated with retinal re-detachment.
Nine days after the second surgery, an additional procedure was planned. Subretinal silicon oil was removed through the inferior peripheral retinotomy. The ILM was stained with ILM-Blue (DORC International), and a temporal optic disc edge-based 1.5-DD sized ILM flap excluding the foveal area was made to cover the optic disc including the ODP (Fig. ). Finally, the vitreous cavity was filled with 12% octafluoropropane gas, and the patient was instructed to remain in the prone position. SRF was rapidly absorbed, and the retina remained attached at 3 months, which was the patient's final postoperative visit. Postoperative OCT revealed the inverted ILM flap lying over the ODP (Fig. ). The best-corrected visual acuity was 20/200 in the right eye. It did not improve from the preoperative acuity because of preexisting extensive retinal pigmented epithelial atrophy involving the macula.


A 23-year-old female presented with diminution of vision, pain, and redness in the left eye for the past 2 months following a visit to a hill station when she wiped her face with a towel while sitting on a hill in the blowing wind.
On examination, the best-corrected visual acuity (BCVA) in the right eye was 20/20, N6 and in the left eye was 20/40, N6. Applanation tonometry recorded an intraocular pressure of 16 mmHg in the right eye, and it was deferred in the left eye. Slit-lamp examination of the right eye was within normal limits, and in the left, there was mild conjunctival congestion with multiple subepithelial corneal opacities and linear hair-like foreign bodies embedded within the corneal stroma [] Anterior chamber of the left eye showed anterior chamber cells 3+ and Flare +1 with multiple linear hair-like foreign bodies []. Fundus examination was within normal limits in both the eyes. She was treated with topical prednisolone acetate – 1% eye drops, atropine sulfate eye drops – 1%, and lubricating eye drops. She subsequently underwent anterior chamber wash during which the hair-like foreign bodies were removed and sent for microscopic examination.
Follow up on the next day showed no evidence of any foreign body in the anterior chamber of the left eye, and the topical medications were tapered and stopped. Microscopic examination confirmed the presence of caterpillar hair shafts.
She presented 2 months later with blurring of vision in the left eye. On examination, the BCVA in the left eye was 20/60, N12. Slit-lamp examination showed subepithelial corneal opacities in the left eye. Fundus examination showed vitreous haze with vitreous cells 3+ with exudates in the pars plana and traction over the peripheral retina with subretinal gliotic bands. She was started on oral corticosteroids 1 mg/kg weight, topical prednisolone acetate eye drops – 1%, and atropine sulfate – 1% eye drops. After 1 month, the BCVA in the left eye was 20/40, N6; however, there was diminution of vision with recurrence of vitritis whenever the oral corticosteroids were stopped. UBM was done of the left eye which showed three hyperreflective linear echoes suggestive of caterpillar hair in the pars plana and within the sclera along with the thickening of the pars plana in the 7 o'clock position [].
A 360°laser barrage to the peripheral retina in the left eye was done, and a pars plana lensectomy (PPL) and PPV were planned after the laser chorioretinal atrophy had developed. Anterior capsule sparing PPL was planned because it was not possible to remove embedded pars plana caterpillar hair without a lens touch. A (23-gauge) PPL and PPV surgery were done removing five caterpillar hair from the pars plana using fine end gripping forceps followed by laser barrage to the iatrogenic retinal breaks and silicone oil tamponade.
On 6-month follow-up, the left eye showed aphakia with no evidence of active inflammation. Fundus examination showed a well-attached retina. She underwent silicone oil removal with secondary IOL implantation in the left eye under local anesthesia and aseptic precautions. The last follow-up was at 2 years, and the left eye had the BCVA of 20/40, N6 with pseudophakia and a well-attached retina with no recurrence of inflammation.


A 69-year-old Caucasian woman presented with an asymptomatic hardened plaque on the
left axilla that had been slowly growing for 2 years. It consisted of a
well-demarked erythematous-violaceous plaque, measuring 26 x 11 mm, without an
underlying palpable mass (). Both
breasts were clinically normal.
An incisional skin biopsy was performed, and it showed dermal infiltration by small
nests and ductal structures lined by atypical cuboidal epithelial cells embedded in
a dense, desmoplastic stroma (). The
neoplastic cells were polygonal with centrally placed nuclei and abundant
eosinophilic cytoplasm. No mitoses were seen (). Immunohistochemistry was strongly positive for estrogen receptor and
cytokeratin 7 (). This morphology was
thought to be suggestive of metastatic invasive breast carcinoma of no special type.
However, further investigation with mammography and breast magnetic resonance
detected no alterations.
Referral was subsequently made to our institution, and the patient underwent wide
excision of the axillary lesion and sentinel lymph node biopsy. The histologic
findings of the surgical specimen were identical to the previous biopsy. The tumor
invaded the subcutis and excision was complete. No perineural or lymphovascular
invasions were observed. In light of the morphological and immunohistochemical
features of the tumor, and the absence of detectable breast carcinoma, final
diagnosis of DEC rather than invasive breast carcinoma was made.
Left axillary sentinel lymph node biopsy was positive, and subsequently left axillary
lymph node dissection was performed, revealing metastases in 3 of the 11 lymph nodes
resected (). Computed tomography scan
and positron emission tomography excluded the presence of further metastases or
other primary tumors. The patient received adjuvant radiotherapy at the left axilla
(total dose 60 Gy), and started oral letrozole 2.5 mg daily. After 14 months of
follow-up, she remains disease-free, under letrozole therapy.


We report a 68-year-old man presenting with a 6-year history of progressive
overgrowth of a bullous lesion of the lower lip with loss of its normal contour. The
lesion was asymptomatic and occasional itchy. His past medical history revealed
hypertension and type II diabetes, for which he was prescribed enalapril 10mg and
metformin 850mg/daily. He was a former moderate smoker (15 cigarettes/day) and had
worked as a farmer. The patient reported no other medical problem or family history
of melanoma or non-melanoma skin cancer. No alcohol consumption was reported.
Physical examination revealed a hyperkeratotic exophytic bullous mass involving more
than two-thirds of the lower lip with obliteration of its normal contour (). No locoregional lymph nodes were
observed.
Based on these features, we made an initial diagnosis of squamous cell carcinoma.
We discussed different surgical options. After considering the size of the lesion and
the risks and benefits of the different surgical techniques, we opted for lesion
excision followed by reconstruction with an Abbe-Estlander flap.
The procedure was performed under general anesthesia. We used a scalpel with a #15
blade to remove the lesion with a 5mm margin. The flap was designed with parallel
planes of skin and mucosa in a portion of the upper lip ( and ). The
flap was then advanced and twisted following the wedge resection of the tumor to
create a new oral commissure (). A
pressure dressing was applied to the entire lower lip for five days. Postoperative
period was satisfactory and the patient was followed up closely for dressing
changes. The wound healed well two weeks after the intervention. At a one-month
follow-up visit, a scar was barely present, with no local complications such as
venous return disturbance. The oral commissure was restored with good symmetry
(). Return of sensation occurred
after three months. The overall final outcome was good and the surgery was
functionally successful.


A 44-year-old male with a history of high blood pressure was admitted with the clinical symptoms of progressive dysphagia and hoarseness in the course of 2 days.
Esophago-gastro-duodenoscopy revealed irregular uplift lesions of 5 cm length, from 28 to 33 cm, and then histopathologic work-up of the specimens confirmed the diagnosis of squamous cell carcinoma of the esophagus ( and ). Computed tomography scan showed a circumferential middle-third esophageal growth, which was ~5 cm long, with significant regional lymphadenopathy but without extraesophageal spread. There were enlarged mediastinum and paracardiac lymph nodes in the computed tomography image, suggesting positivity for malignancy as well (). Unfortunately, the patient lost the opportunity to perform esophagectomy. Taking into account the poor prognostic factors such as transmural extension and lymph node positivity, the patient was given two cycles of Doxorubicin plus cisplatin. During the second course of chemotherapy, he discovered that his right ring finger had a protrusion for the first time, which was soft, cystic and 2.0×1.8 cm in size (). Initially, the patient thought that this protrusion was caused by swelling which occurred 1 day prior. Unfortunately, fine-needle aspiration biopsies from the site as well as the pathology revealed squamous cell carcinoma ( and ). Radiologic studies revealed no bony metastasis (). As a result, this lesion was considered metastatic, as it appeared simultaneously and was reported as squamous cell carcinoma on fine-needle aspiration biopsy. A subsequent metastatic work-up did not reveal any other site of metastases. Then, the patient underwent phalangectomy of his right ring finger. He was planned to undergo palliative chemotherapy and radiotherapy to the metastatic sites, but unfortunately his condition started deteriorating and he succumbed to the disease within 2 months of diagnosis of metastasis.


A 25-year-old female presented with complaints of increased bowel frequency, oliguria, fever and abdominal pain since one month. Detailed history revealed that the patient had difficulty in micturition for past two weeks. The routine laboratory investigations revealed a haemoglobin of 7.8 gm/dL, total leucocytes count 3200 cells/mm3, platelet count of 70000 cells/mm3. Renal function tests revealed normal sodium concentration (139 mEq/L), hyperkalemia (8.2 mEq/L), hyperuricemia (74 mg/dL) and elevated creatinine levels (7.5 mg/dL). Coagulation profile was normal. Ultrasonography (USG) revealed bilateral hydroureteronephrosis with normal renal parenchyma and features of vesicoureteric reflux. The midstream urine sample was subjected to microbiological testing. The wet mount microscopic examination showed 1-2 RBCs, numerous pus cells and bacteria per high-power field[]. The semi-quantitative culture done on the cysteine lysine electrolyte deficient agar showed significant bacterial growth (colony count > 105 CFU/mL). The colonies were non-lactose fermenting, translucent, greenish blue, smooth having entire edges and became mucoid on prolonged incubation. Subculture on MacConkey agar showed pale, translucent, glistening colonies with entire edges (Figure ). Gram staining showed 0.5 μm × 2 μm gram-negative bacilli, with no spores and no capsule. The isolate was also subjected to conventional identification using a battery of biochemical tests. The isolate was catalase positive, oxidase positive, produced indole, was non-nitrate reducing, mannitol fermenting, esculin and gelatinase hydrolysis positive. Urease was produced and this test helped to distinguish it from E. meningoseptica. The isolate was confirmed as Elizabethkingia miricola (E. miricola) (identification score of 2.29) by using MALDI-TOF-MS (BrukerDaltonics, Bremen, Germany). The antimicrobial susceptibility was carried out using Kirby-Bauer disc diffusion method and the antibiotics tested were chosen from the available literature as there are no CLSI guidelines available till now[,]. The isolate was sensitive to gentamicin, ceftriaxone, aztreonam, piperacillin-tazobactam and imipenem, and resistant to ampicillin, ciprofloxacin, levofloxacin, vancomycin and colistin. The patient was started on piperacillin-tazobactam and responded well to the treatment. The patient improved clinically and the follow-up urine culture after two weeks of therapy was sterile.


A 12-year-old girl was referred to the Endocrine Center at Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, for evaluation of polyuria and short stature. From the age of 4 years, her parents had noticed abnormalities of her teeth. She is the 5th of the six siblings born to native Kashmiri consanguineous (first cousins) parents. Her younger brother had died at the age of 4 years, and he had presented with hypokalemic quadriparesis. Examination revealed prepubertal girl with severe, proportionate short stature (height 117 cm, height standard deviation [SD] score − 5.32). Her weight was 27 kg (weight SD score − 1.76). There were no clinical features of rickets. Oral examination revealed generalized pitting, yellowish-brown pigmentation of all the surfaces of the teeth, delayed exfoliation of deciduous teeth, and delayed eruption of permanent teeth []. Detailed ophthalmic examination and pure-tone audiometry were normal. Radiographically, the radiodense enamel was absent in all the teeth with normal radiodensity of dentin, large, and open pulp canals. Few retained deciduous teeth and impacted canines and premolars were seen. The clinical and radiographic features led to the diagnosis of hypoplastic AI.
Investigations revealed a normal anion gap metabolic acidosis (serial serum pH 7.19, 7.20, and 7.22 and anion gap 11, 14, and 15), alkaline urine (serial urine pH 6.7, 6.5, and 7), and hypokalemia (serial serum K+ 1.85, 1.52, and 2.8 mEq/L). Urine anion gap was positive. Serum osmolality was 277 and urine osmolality was 128 mosm/kg. Routine lab investigations including blood glucose, serum calcium, serum phosphorus, alkaline phosphatase, thyroid function test, renal, and liver function tests were normal. The urinary calcium excretion was increased (236 mg/day). Urine analysis did not reveal glycosuria, proteinuria, or aminoaciduria indicating normal proximal tubular reabsorption. Serum 25-OH D levels were normal (37 ng/ml).
Bilateral nephrocalcinosis was identified by means of ultrasonography []. Radiograph of left wrist did not show any evidence of rickets, and bone age was normal (according to Greulich and Pyle Atlas).
On the basis of persistent normal anion gap, metabolic acidosis with alkaline urine, hypokalemia, hypercalciuria, and bilateral nephrocalcinosis, a diagnosis of dRTA was made. The diagnosis was confirmed by ammonium chloride test, and the serial urine pH after oral intake of ammonium chloride was 7.3, 7.5, and 6.9. We further investigated her for secondary causes of RTA, and autoimmune profile including antinuclear antibody was negative. She was started on oral potassium citrate supplementation. Treatment with potassium citrate resulted in correction of hypokalemia, systemic acidosis, and marked relief of polyuria.


A 65-year-old male patient was admitted due to a headache. On the neurological examination, right sixth nerve involvement was determined. At the clivus neighborhood, contrast diffuse holding 2 cm × 1.5 cm lesion on magnetic resonance imaging has been identified. To determine the unknown primary PET/CT scan was performed. In PET/CT imaging; middle lobe of the right lung was likely primary tumor with centrally located intense 18F FDG uptake foci; also, multiple bone and liver metastases were determined. In upper abdominal images of the PET/CT annular, 18F FDG uptake was seen in liver segment 4A (77 mm × 70 mm) and segment 2 (33 mm × 34 mm) []. In addition, conventional abdominal CT images showed lower density in the center of the lesions in the liver, indicating central necrosis. Patient's histopathology confirmed squamous-cell carcinoma of the lung. Separation of potential skeletal metastases with bone scintigraphy has been proposed. In bone scan addition to metastatic regions, the annular 99mTc MDP uptake in liver was observed the same as described in 18F FDG uptake [].


A 31-year-old woman, gravida 3, para 2, was first referred at 22+3 weeks for further evaluation of a cystic lesion in the fetal abdomen. Antenatal follow-up was uneventful until the fifth month of gestation, and the first trimester antenatal aneuploidy screening tests were normal. Her medical history was also normal. On referral, transabdominal sonography was performed by Medison SonoAce X8 system (Seoul, South Korea) and equipped with a 3DC2-5 volumetric probe. A singleton, live, female fetus with irregular subcutaneous cystic mass, measuring 55 × 38mm in size at the widest plane on the left inguinal region was revealed by ultrasound ().
Fetal biometry was appropriate for the gestational age. This cystic mass was extending to the left leg until the knee. Color Doppler flow mapping revealed no blood flow within the lesion. The possible diagnosis was fetal inguinal lymphatic malformation. The assessment of amniotic fluid and placenta were normal.
There was no evidence of any other fetal anomalies. The fetal thorax and intra-abdominal organs were normal in the magnetic resonance imaging (MRI) examination ().
Cytogenetic analysis revealed normal karyotype by genetic amniocentesis. Detailed information was given to the parents about the possible postnatal prognosis. They decided to continue with the pregnancy. Follow-up was uneventful until the 36th week of gestation. The dimensions of the cystic mass remained the same. At 36 weeks gestation, oligohydramnios was diagnosed, and the fetus was small for that gestational age. A 2600 g infant was delivered at 38 weeks’ gestation by cesarean section because of the mother’s previous history of cesarean delivery. In the postnatal examination, a multicystic and translucent mass was seen which had 5 cm maximum diameter without any skin lesion. The mass extended from the left inguinal region to the left thigh, and a hemangioma was detected on the left inguinal region of 25 mm width. No cyst damage occurred during the delivery. The Apgar scores were 7 and 9 after 1 and 5 min, respectively. MRI reported an inguinal lymphatic malformation, and an ultrasound revealed there was no fetal anomaly and no apparent invasion of the mass into the abdominal cavity and the intra-abdominal organs. The MRI detected lymphatic-venous vascular malformation, which was associated with the femoral vein (). It was not necessary to schedule any pediatric surgical intervention.
Sclerotherapy was recommended but not accepted by the family. Echocardiography revealed increased aortic gradient and patent foramen ovale. Additional structural anomaly was not detected. Propranolol and sildenafil were started. After 7 days, the patient was discharged and regular clinical examinations were recommended. Heart failure developed during the second year of follow-up.