Antimitochondrial antibody-negative primary biliary cirrhosis with secondary Sjogren syndrome: a case report.

Introduction and importance: Primary biliary cholangitis (PBC) is a rare immune-mediated liver disease characterized by the destruction of intrahepatic bile ducts and a positive antimitochondrial antibody (AMA), which is considered a serological hallmark for the diagnosis. Rarely, AMA can be absent/nondetectable in a few cases and is referred to as 'AMA-negative'. Case presentation: The authors present such an uncommon case of AMA-negative PBC in a 39-year-female with Sjogren's syndrome who presented with fatigue, pruritus, and dry eyes. Clinical discussion: Previously published studies state that approximately only about 5% of patients with PBC are 'AMA-negative'. For patients negative for AMA, the diagnosis has to be based on typical pathological features of this disease. Once a diagnosis of PBC is established, regardless of whether it is positive or negative for AMAs, ursodeoxycholic acid is a widely accepted treatment. Conclusion: The presence/absence of AMAs is associated with similar clinical, biochemical, and histopathological characteristics in PBC. The identification of AMAs alone should not impact the diagnosis or treatment of PBC.

Induction of Brugada electrocardiogram pattern with aluminum phosphide poisoning: a case report.

Introduction and importance: Aluminum phosphide (ALP) is a commonly used suicidal agent in an agrarian country like Nepal. The unmasking of the Brugada pattern in the electrocardiogram (ECG) associated with ALP poisoning is a rare phenomenon, and studies pertaining to it are scarce in the medical literature. Case presentation: An 18-year-old female presented to the emergency department with multiple episodes of vomiting, headache, blurring of vision, and abdominal pain after 4 h of consumption of ALP with suicidal intent. A 12-lead ECG revealed a coved ST-segment elevation and T-wave inversion in leads V1-V3 with right bundle branch block suggestive of a type 1 Brugada pattern. Her past medical and family history was not significant. The patient made an uneventful recovery with the required supportive treatments. Clinical discussion: Cardiac arrhythmias are the major cause of death in ALP poisoning. Unmasking of the Brugada ECG pattern is a rare but potentially fatal complication implicated in various pharmacological toxicities, including tricyclic antidepressants, cocaine, procainamide, disopyramide, flecainide, and rarely with ALP. Conclusions: ALP poisoning can unmask the Brugada ECG pattern, which can lead to ventricular fibrillation and/or sudden cardiac death.

Parasitic mature cystic ovarian teratoma: A rare case of autoimplantation of a twisted dermoid cyst.

Extragonadal parasitic dermoid cysts are rare. Diagnosis of such extragonadal parasitic teratoma is often done intraoperatively during surgical exploration of abdominal mass.

Chest wall mass as a sign of ignored hepatocellular carcinoma in an alcoholic cirrhotic patient: a case report.

Bone metastasis of hepatocellular carcinoma (HCC) is usually seen in the course of advanced intrahepatic HCC. Isolated bone metastasis as an initial manifestation of HCC is uncommon. Case presentation: The authors report a case of a 68-year-old male with a history of chronic alcohol consumption who presented with epigastric pain, abdominal distension, and a hard, tender lump on the right posterolateral aspect of his back. Investigation revealed HCC with isolated metastasis to the posterior thoracic wall. Clinical discussion: HCC usually develops secondary to chronic hepatitis B and C infection in the background of chronic liver disease. Common presenting symptoms of bone metastasis include local pain, neurological manifestations, palpable subcutaneous masses, and pathological fractures. An immunohistochemistry analysis is important to differentiate HCC from non-HCC metastasis in patients without known underlying HCC. Treatment is often directed towards palliative care as the prognosis is poor. Conclusion: An isolated rib mass can be an initial presentation of metastatic HCC. Thus, HCC with bone metastasis should be considered in the differential diagnosis in patients presenting with painful swelling in the thoracic region.

Breakage of an orogastric tube in a critically Ill patient: a case report.

Orogastric and nasogastric tubes are common methods of enteral feeding. Although the methods of tube feeding are simple, these methods are not free of complications. Case presentation: This case report describes a 58-year-old patient with the diagnosis of stroke, in whom there was breakage of an orogastric tube during a prolonged ICU stay. Clinical discussion: Early enteral feeding in patients without any contraindications is associated with improved organ survival and recovery, and decreased incidence of infections, which decreases the ICU stay and improves the overall outcome. Nasogastric and orogastric tubes are the most commonly inserted feeding tubes. Breakage of an orogastric tube is a rare complication that can occur due to manufacturing defects, exposure to a harsh acidic environment, and forceful flushing of an obstructed tube. Conclusions: Timely identification of the broken feeding tube can help the treating clinicians retrieve it easily even with the help of a laryngoscope in selected patients.

Diffuse large B-cell lymphoma in a patient treated with azathioprine for ulcerative colitis: a case report.

Azathioprine (AZA) used as an immunomodulator agent in the management of inflammatory bowel disease (IBD) increases the risk of the development of lymphoma. Case presentation: We present a case of a 45-year-old female receiving AZA for severe ulcerative colitis for 4 years. She presented with the chief complaints of bloody stool and abdominal pain for 1 month. Through a series of investigations including colonoscopy, contrast-enhanced computed tomography scan of the abdomen and pelvis, and biopsy with immunohistochemistry; she was diagnosed to have diffuse large B-cell lymphoma of the rectum. She is currently on a chemotherapeutic regimen and is planned for surgical resection after the completion of neoadjuvant therapy. Clinical discussion: AZA is classified as a carcinogen by the International Agency for Research on Cancer. Prolonged exposure to higher doses of AZA increases the risk of developing lymphoma in IBD. Previous meta-analysis and research indicate that the risk of development of lymphoma after the use of AZA in IBD increases by about four- to six-fold, especially in older age groups. Conclusions: AZA may increase the susceptibility to developing lymphoma in IBD, but the benefit far outweighs the risk. Precautions must be taken in prescribing AZA in older individuals which mandates periodic screening.

Pontine and bilateral cerebellar lesion in osmotic demyelination syndrome associated with uncontrolled type II diabetes mellitus: a case report.

Osmotic demyelination syndrome (ODS) as a result of the hyperosmolar hyperglycemic state is rare and can present with variable neurological manifestation due to lysis of myelin sheath. Case presentation: A 44-year diabetic male presented with complaints of sudden onset, progressive bilateral weakness in lower limbs, and slurring of speech for the past 1.5 months. Cerebellar examination showed a bilaterally impaired finger nose test, dysdiadochokinesia, impaired heel shin test, and an impaired tandem gait. MRI brain (T2 and fluid-attenuated inversion recovery sequences) showed high signal intensity in the central pons and bilateral cerebellum. With a diagnosis of ODS with poorly controlled diabetes, he was treated with insulin, metformin, and supportive measures following which his symptoms subsided gradually. Clinical discussion: A rapid correction of hyponatremia is considered the most common cause of ODS. Variations in plasma glucose levels, a rare cause of ODS, can cause an abrupt osmolality change causing pontine and extrapontine myelinolysis. Prevention of rapid correction of hyponatremia and rapid changes in plasma osmolality in vulnerable patients is the mainstay of treatment. Conclusions: Clinical features, imaging studies, and monitoring of serum osmolality, serum glucose, and electrolytes aid in diagnosis and favorable outcomes for the patient.

Bilateral Page kidney in a postpartum female: a case report.

Page kidney results from external compression of the kidney by a subcapsular hematoma and is one of the rare but treatable causes of secondary hypertension. The majority are traumatic or iatrogenic and often unilateral. Spontaneous bilateral Page kidney is rare. Case Presentation: A 35-year P1 with gestational hypertension presented with a persistent postpartum elevation of blood pressure (BP). Imaging studies revealed bilateral renal subcapsular hematoma (left>right). She was managed with an angiotensin receptor blocker initially and ultrasound-guided percutaneous drainage of the collection for the optimal control of elevated BP. Clinical Discussion: Ultrasonography and computed tomography of the kidneys are the most frequently used for diagnosing a Page kidney. Medical management with antihypertensive and regular follow-ups form the first line of treatment in Page kidneys. Percutaneous drainage, surgical decortication, laparoscopic intervention, and nephrectomy are necessary in cases of organized late hematomas. Conclusion: Spontaneous bilateral Page kidney is a rare but potentially treatable and curable form of hypertension. Percutaneous drainage is an effective method to drain the hematoma and control elevated BP.

Xanthogranulomatous Cholecystitis Mimicking Carcinoma Gallbladder.

Xanthogranulomatous cholecystitis (XGC) is a rare benign chronic inflammatory disease of the gallbladder that often presents as cholecystitis and can mimic gallbladder carcinoma. Distinguishing XGC from gallbladder cancer preoperatively is challenging. We present a case of a 62-year-old male who presented with features of carcinoma gallbladder in the CECT abdomen and MRCP. Intraoperatively, there was a mass in the gallbladder and extension into the adjacent structures with involvement of the hepatic artery, 1st part of the duodenum, portal vein, and hepatic flexure of the colon, and thus a palliative cholecystectomy was done. The histopathological report came out as XCG. The case aims to outline the clinical presentation of XGC and differentiate it from carcinoma gallbladder.

Bilateral central retinal vein occlusion as an initial presentation of Waldenström macroglobulinemia: a case report.

BACKGROUND: Waldenström macroglobulinemia is a rare hematological malignancy and is the most common diagnosis in patients with hyperviscosity syndrome. Bilateral central retinal vein occlusion as an initial presentation of hyperviscosity syndrome in Waldenström macroglobulinemia is rare. CASE PRESENTATION: A 42-year-old Nepalese male presented with sudden-onset bilateral painless blurring of vision. Fundus examination revealed bilateral, diffusely dilated, tortuous retinal veins and intraretinal deep blot hemorrhages in all four quadrants of the retina in both eyes; features of bilateral central retinal vein occlusion. Serum electrophoresis showed hypoalbuminemia with an immunoglobulin M kappa monoclonal spike. Bone marrow picture and immunohistochemistry analysis were suggestive of lymphoplasmacytic lymphoma. The patient received systemic therapy for Waldenström macroglobulinemia, along with intravitreal bevacizumab. CONCLUSION: Adequate hydration, plasmapheresis, and a combination of bortezomib, dexamethasone, and rituximab regimen as a systemic therapy may represent an ideal choice for patients with hyperviscosity in Waldenström macroglobulinemia.

Splenic artery aneurysm with extrahepatic portal venous obstruction: A case report.

INTRODUCTION AND IMPORTANCE: Splenic artery aneurysm is one of the most common visceral aneurysms. Patients are usually asymptomatic. Splenic artery aneurysm if untreated has the potential for rupture and is therefore life-threatening. Its association with extrahepatic portal vein obstruction is rare. CASE PRESENTATION: A 25-year female was incidentally diagnosed with a splenic artery aneurysm with extrahepatic portal vein obstruction with splenomegaly 8 years back during the 5th month of her second pregnancy. No intervention was done back then. Recently, she presented to the surgical gastroenterology outpatient department with an increasing abdominal mass. On examination, the patient was pale and splenomegaly was present. Hematological reports were suggestive of hypersplenism. The patient underwent splenectomy and aneurysmal resection with a proximal splenorenal shunt as the best course of treatment. DISCUSSION: Due to the rarity of the disease, the management is still challenging and needs further study. Diagnosis can be made clinically with support from imaging modalities. Surgical treatment has a good outcome in such patients. Even with the availability of less invasive procedures such as endovascular treatment, open surgery is preferred. CONCLUSION: Proximal splenorenal shunt is a well-accepted surgical procedure for extrahepatic portal vein obstruction. Splenectomy and aneurysmal resection can relieve hypersplenism and treat splenic artery aneurysm in patients with isolated splenic artery aneurysm at the splenic hilum.

Sleeve gastrectomy for multiple gastric perforations in a preterm: A case report.

Neonatal gastric perforation (NGP) is a rare life-threatening condition among preterm infants. NGP can occur secondary to necrotizing enterocolitis, distal obstruction, or as a result of gastric tube insertion. Sleeve gastrectomy can be a possible therapeutic option for multiple neonatal gastric perforations.

Delayed identification of massive pituitary apoplexy in pregnancy: A case report.

INTRODUCTION AND IMPORTANCE: Pituitary apoplexy is a very rare cause of sudden and severe headache with a neuro-ophthalmic deficit during pregnancy due to hemorrhage or infarction in the pituitary gland. Delayed identification can be life-threatening to both mother and baby. CASE PRESENTATION: A 40-year-old nulliparous female without any prior comorbidities in her 21 weeks of gestation presented with complaints of severe headache, vomiting, decreased vision, and altered sensorium for five days. On a low index of suspicion of pituitary apoplexy, she was managed in the line of impending eclampsia at a local center. On presentation to our center; the neurological deficit had progressed. CT head showed massive pituitary apoplexy with sellar and suprasellar extension. She underwent emergency right pterional craniotomy and resection of the tumor with hematoma evacuation but lost her life on her 2nd postoperative day. DISCUSSION: In the context of pregnancy, the diagnosis of pituitary apoplexy can get tricky and overlap with other common conditions such as preeclampsia or eclampsia. Magnetic resonance imaging (MRI) is the most sensitive investigation to confirm the diagnosis. Corticotropic deficiency with adrenal insufficiency is a potentially life-threatening disorder for both mother and the fetus if left untreated. The choice between conservative management and surgical approach depends on the neuro-ophthalmic signs, MRI findings, and gestational week. CONCLUSION: Pituitary apoplexy should be a differential diagnosis of acute severe headache in pregnancy which when suspected should be investigated promptly. Early identification and multi-disciplinary team management are imperative for better outcomes.

Delayed presentation of isolated ductal rupture of pancreatic head from blunt abdominal trauma managed conservatively: A case report.

Introduction: and Importance: Blunt abdominal injury causing significant and isolated major pancreatic injury is rare in adolescents and young adults, with a controversial approach to its management. Case presentation: We present our experience of diagnosis and management of the ductal injury of the pancreatic head (Grade III) in the setting of blunt abdominal trauma in a 20-year-old male diagnosed by a series of various tests including magnetic resonance cholangiopancreatography (MRCP) and managed by pigtail drainage and octreotide alone; contrary to the previous recommendations of management of high-grade pancreatic trauma through surgical approach or endoscopic retrograde cholangiopancreatography (ERCP) and stenting. Clinical discussion: Isolated ductal rupture of the pancreatic head can have delayed presentation within a window of time and can be diagnosed by a series of tests including hematological, biochemical, and radiological investigations. Conservative treatment is generally recommended for Grade I and II whereas a surgical approach is preferred for higher grade pancreatic injury. Conclusions: Pancreatic ductal injury must be kept in mind when present with vague symptoms in the setting of blunt abdominal trauma. Magnetic resonance cholangiopancreatography (MRCP) is the investigation of choice for the diagnosis of pancreatic ductal injury. Even higher-grade pancreatic injury (grade III) can be managed with a conservative approach with pigtail drainage and an appropriate dosage of octreotide.

Diagnosis and management of nitrobenzene poisoning in a low-resource setting: A case report.

Background: and Importance: Nitrobenzenes are aromatic oxidizing nitrate compounds, acute ingestion of which can cause methemoglobinemia (metHb) that impairs oxygen transport. Clinical presentation ranges from cyanosis to asphyxia depending on the level of methemoglobin in the blood. Lack of improvement of hypoxia despite administration of oxygen can also be a clue to the diagnosis. Case presentation: A 23-year-old male with accidental ingestion of nitrobenzene presented with multiple episodes of vomiting, headache, and dyspnea. His venous blood was dark brown in color and oxygen saturation was not improving despite high-flow oxygen. He was managed with methylene blue and Vitamin C on this background with an appropriate history of nitrobenzene ingestion. Discussion: Acute nitrobenzene poisoning results in methemoglobinemia which is diagnosed by Co-oximeter but in resource-limited settings, clinical findings such as no improvement in SpO2 despite supplemental oxygen and chocolate brown appearance of blood aid in the diagnosis. Prompt treatment of methemoglobinemia with methylene blue along with Vitamin C has shown successful and effective outcomes. Conclusion: Diagnosis of acute nitrobenzene poisoning can be made with proper history, physical and bedside examinations in resource-limited settings even without the aid of a co-oximeter.

Retrorectal schwannoma in a middle-aged female: A case report.

INTRODUCTION AND IMPORTANCE: Primary sacral and presacral lesions are uncommon and pose a significant diagnostic dilemma. Retrorectal schwannoma is a benign primary presacral lesion that is extremely rare and presents with non-specific symptoms. CASE PRESENTATION: A 42-years-old, female patient was admitted for lower-back pain for seven days. The retrorectal mass was detected on imaging, and she underwent laparoscopic excision. Pathological examination revealed a rare retrorectal schwannoma. CLINICAL DISCUSSION: Retrorectal location of schwannomas is rare and the preoperative diagnosis is very difficult as the clinical features and the radiological features are non-specific. In addition, these tumors can be found incidentally on routine rectal or pelvic examinations. As these tumors are benign, encapsulated, and non-invasive, this entity has an excellent prognosis. Despite the tumors' rarity and variety, we concur with the literature that surgical management is required to obtain a clear diagnosis and avoid consequences such as malignant transformation. CONCLUSION: Retrorectal Schwannoma presents a diagnostic and therapeutic challenge. Identification is important because surgical excision, either open or laparoscopic, is curative.