Peutz-Jeghers syndrome: A case series.

INTRODUCTION: Peutz-Jeghers syndrome (PJS) is a rare hereditary disorder characterized by gastrointestinal hamartomatous polyps, due to mutation of the STK11/LKB1 gene located on chromosome 19p. The polyps are most commonly found in the small bowel followed by colon. CASE PRESENTATION: Our case series includes 4 patients, three being male and one female. Each of them either presented with abdominal pain and other associated symptoms. Oral cavity and lip melanin pigmentation were common. CT abdomen revealed multiple large jejunal, ileal, gastric and colon polyps. Cancer was found in one patient. Different surgical approaches were adopted. All recovered well. DISCUSSION: PJS is an autosomal dominant disorder with an estimated incidence of 1:50,000 to 1:200,000 cases with a significant family history. Mostly found in small bowel followed by colon, it can also occur in a rare organ like gall bladder as evident in our case. PJS carries a substantial risk for gastrointestinal cancer. The treatment modality depends on the site of polyp, mode of presentation and availability of the expertise. CONCLUSION: PJS is a common disease in our part which is usually observed in teen age groups male. They have a varied presentation, from intestinal obstruction (due to intussusception) to GI bleeding. Colonic malignancy at young age may be the first presentation of the disease. Observation of melanin pigmentations on lips helps diagnose the disease; and one should always look at this findings in a young patient with pain abdomen or in intestinal obstruction to confirm/exclude the disease.

Tophaceous gout.

Hyperuricemic patients (≥7.8 mg/dL) can develop polyarticular tophaceous gout from intermittent arthritis if untreated. Acute flares and tophi development can be avoided by lowering blood urate levels with xanthine oxidase inhibitors.

Continuous versus interrupted anastomotic technique for the hepaticojejunostomy: a prospective cohort study.

Background: Interrupted sutures is the gold standard technique of hepaticojejunostomy (HJ) for bilioenteric anastomosis. This study compares the safety and early complications of continuous and interrupted suture HJ. Methods: A prospective study involving all elective HJ between September 2019 and June 2021 was conducted. Patients with type IV or V biliary strictures, duct diameter less than 8 mm and/or associated vascular injury, and bilateral HJ were excluded. The study patients were divided into two random groups; interrupted and continuous anastomotic technique. Patient demographics, preoperative parameters including pathology (benign vs. malignant), HJ leak, suture time, and postoperative morbidity were recorded. Results: Total 34 patients were enroled. Eighteen (52.9%) were into interrupted and 16 (47.1%) patients into the continuous group. Both the groups were comparable with regards to demographics, haemoglobin, serum albumin, preoperative cholangitis and biliary stenting. Total three (8.8%) patients in the entire study developed bile leak; interrupted-2 and continuous-1, which was not significant statistically (P=1.0). Similarly, total number of sutures used and the mean operating time to complete anastomosis in the continuous group was significantly lesser than the interrupted group (2.3±0.5 versus 9.6±1.6, P<0.001) and (16.2±3.1 versus 38.6±9.2 min, P<0.001), respectively. There were three (18.8%) re-exploration in the continuous anastomotic technique. Among them, only one re-operation was due to HJ anastomosis failure without mortality, remaining had re-exploration for bleeding (non-HJ). Conclusions: Both the techniques is safe with comparable morbidity. Further, continuous has an added advantage of decreased anastomotic time and cost.

Laparoscopic transhiatal en-mass thoracic duct ligation for persistent bilateral spontaneous Chylothorax: A case report.

Chylothorax is a rare entity associated with morbidity. Surgical thoracic duct ligation (TDL) by thoracoscopic approach is the recommended choice for persistent chylothorax. However, thoracoscopy is not feasible in case of previous pleurodesis. We describe a successful laparoscopic transhiatal en-mass TDL in a 61-year-old lady for persistent spontaneous chylothorax after failed optimal conservative management and three sessions of pleurodesis. The study shows that laparoscopic transhiatal thoracic duct ligation is an effective alternative in a case where thoracoscopy is not feasible due to various reasons.

Hookworm infestation as a rare cause of melaena in tropics: A case report.

Melaena due to hookworm infestation is a rare clinical presentation. It usually presents with symptoms of iron-deficiency anaemia owing to slow blood loss. Here we present a case of 45-year male who presented with a one-year history of intermittent melena requiring multiple blood transfusions. Preliminary endoscopies at different centres were normal. The presence of hookworm in the duodenum was only detected on repeat upper endoscopy as the cause of blood loss, and treatment with albendazole was successful in curing the patient.

Median nerve schwannoma with a diagnostic delay of 8 years: a case report.

Introduction: Schwannomas are benign tumors of the peripheral nerve sheath, and the median nerve is the most commonly involved nerve. These benign tumors of the peripheral nerve sheath are very rare; they are clinically and radiologically similar to most other benign swellings of the hand; thus, they are often misdiagnosed. Case Presentation: A 41-year-old lady presented with an 8-year-long history of swelling over the distal forearm. The tumor measured 3.5×3.5×3.5 mm and was located over the flexor aspect of the distal part of her right forearm. Schwannoma was suspected from the clinical presentation and imaging, but the final diagnosis was established only after the surgery and histopathological analysis. At the follow-up after 1 year, the patient is doing well and her symptoms have not recurred. Conclusion: Imaging characteristics of schwannoma can be misinterpreted as some other condition, making the preoperative diagnosis very difficult and important. Thus, clinicians should be aware of such swellings, especially those that have been unnoticed or misdiagnosed, and provide optimal diagnoses to confer good outcomes.

Left segmental hypoplasia of liver with concomitant gallbladder agenesis.

Key Clinical Message Congenital liver anomalies are rarely reported. To the best of our knowledge and literature review, associated gallbladder agenesis with left hepatic lobe agenesis is extremely rare. The rarity of this condition and its uncertain radiological features often leads to misdiagnosis and unwarranted surgical treatments.

Spontaneous idiopathic omental bleeding: a case report of rare cause of acute abdomen.

Idiopathic omental hemorrhage is a rare cause of an acute abdomen, which is potentially life threatening. Here, we report a case of a 34-year-old male who presented to the emergency department with sudden, severe pain abdomen and abdominal distension for 1 day. There was no history of trauma, abdominal surgeries or any significant past medical history. The diagnosis was suspected on contrast computed tomography, which revealed hyperdense areas of blood in the peritoneal cavity with contrast extravasation from the omentum. The patient underwent successful emergency laparotomy, peritoneal lavage and greater omentectomy to achieve hemostasis.

Recurrent pyogenic cholangitis.

The incidence of hepatolithiasis is variable. Globalization has additionally altered disease dynamics globally. A multi-disciplinary team approach is deemed necessary for the timely diagnosis, safe, affordable treatment, and good prognosis.

Resectability and prognosis of gallbladder cancer: a cross-sectional study of 100 cases from a tertiary care centre of Eastern Nepal.

Gallbladder cancer (GBC) is the fifth most common neoplasm of the digestive tract and has an overall incidence of 3 per 100 000 people. Only 15-47% of the preoperatively known GBC are suitable for resection. The objective of the study was to investigate the resectability and prognosis of GBC patients. Materials and methods: It is a prospective observational study including all cases of primary cancers of the gallbladder in the Department of Surgical Gastroenterology at a tertiary care center over the period from January 2014 to December 2019. The primary endpoint was resectability and overall survival. Results: During the study period, 100 patients with GBC were reported. The mean age at the time of diagnosis was 52.5 years, with a female predominance (67%). The curative intent resection (radical cholecystectomy) was possible in 30 (30%) patients; while 18 (18%) required palliative surgical treatment. The overall survival of the entire group was 9 months; while those patients who underwent surgery with curative intent had a median overall survival of 28 months after a median follow-up of 42 months. Conclusion: This study found that only one-third of patients achieve radical surgery with curative intent. Overall, the prognosis of patients is poor with a median survival of less than a year due to the advanced stage disease. Multimodality treatment, screening ultrasound, and neo-/adjuvant therapy may improve survival.

Extended Total Mesorectal Excision (e-TME) for Locally Advanced Rectal Cancer.

BACKGROUND: Locally advanced rectal cancer (LARC) can involve surrounding pelvic organs requiring multivisceral resection. Extended total mesorectal excision (e-TME) or multivisceral resection is a complex procedure associated with high morbidity, mortality, and R1 resection rates. However, e-TME in LARC with surrounding organ involvement is the only potential option for cure. The study aims to assess the clinical outcome of patients requiring e-TME for LARC. METHODS: The study is a retrospective review of all patients with LARC requiring multivisceral resection (2013 to 2019). The database includes clinic-demographic profile, pelvic organ involved, operative details, resection margin status, morbidity, mortality, and survival. RESULTS: Seven consecutive patients (9.2%) out of 76 LARC (median age 46 years; 5 females) required multivisceral resection. The organs involved were bladder (4); posterior wall of vagina (2); and uterus (1). The en bloc resection included total cystoprostatectomy - 1; partial cystectomy - 3; posterior vaginectomy - 2; and hysterectomy - 1. Additionally, four required abdominoperineal resection. All were adenocarcinoma: stage III, with R0 resection - 76%. The overall complications were seen in 60% of patients, majority being wound related. There was no operative mortality. The median survival was 32.2 months in the entire series, while one died with the disease at a 28-month follow-up. CONCLUSION: e-TME with curative intent, though a complex procedure, is associated with high wound-related morbidity, R1 resection, but improved median survival benefit.

Non-malignant gall bladder perforation: Our experience from an institution-based retrospective analysis of 25 cases.

Gallbladder perforation still continues to perplex surgeons; 25 such patients diagnosed either pre- or intra-operatively and managed at our institute over the last 10 years period were analysed. Only eight were diagnosed pre-operatively, while a large majority (17) had a wrong initial working diagnosis. Symptoms and signs were variable. No blood investigation was specific. A computed tomography scan was generally better than ultrasound in detecting the perforation. All our cases were managed operatively with no mortality and a mean duration of hospital stay of 6.8 days. Most perforations were extra-hepatic (84%) and those of Niemeier's type I (52.2%). Because of its varied clinical presentation, gallbladder perforation is often an intra-operative diagnosis, but early intervention carries a good outcome.

Post-pancreatic necrosectomy biliary fistula: thinking beyond the box.

Walled-off pancreatic necrosis is a challenging problem and pancreatic necrosectomy is associated with significant morbidity and mortality. Following necrosectomy, postoperative bile leak is a rare complication. We present such a case of delayed bile leak from the distal common bile duct in an 81-year-old lady following pancreatic necrosectomy, which was successfully managed by endoscopic stenting.

Inflammatory cap polyp of the sigmoid colon: a case report.

BACKGROUND: Inflammatory cap polyp is a very rare benign entity of the distal left colon, characterized by inflammatory polyp with a "cap" of fibrinopurulent exudates. They are usually multiple and commonly present with bleeding per rectum or mucoid discharge. Solitary polyp presenting with intermittent intussusceptions is rare. CASE PRESENTATION: We report the case of a 45-year-old Nepalese male with a solitary inflammatory sigmoid colon polyp. The patient presented with a 1-month history of rectal bleeding, mucoid discharge, and severe colicky abdominal pain due to intussusceptions. On colonoscopy, there was an exophytic mass with surface exudates. Colonic resection and anastomosis were performed, due to recurring partial intestinal obstruction. At a 6-month follow-up, the patient was asymptomatic. CONCLUSION: Inflammatory cap polyp is a benign entity, and it should be kept in mind as an important differential diagnosis of exophytic colonic mass with surface exudates.

Surgical treatment of tuberculous chronic constrictive pericarditis: A retrospective observational study from tertiary hospital of eastern Nepal.

BACKGROUND: Tuberculosis remains an important cause of chronic constrictive pericarditis (CCP) in developing countries. It is a surgically treatable cause of diastolic heart failure. Without surgery, it is associated with high morbidity and mortality. METHODS: We conducted a retrospective observational study of clinical presentations and perioperative outcomes of pericardiectomy in all patients operated from July 2015 to December 2018 for tuberculous CCP. RESULTS: A total 14 patients (mean age - 38 ± 13.3 years, 10 male), underwent pericardiectomy via median sternotomy without cardiopulmonary bypass. Eleven patients (79%) had completed treatment for pulmonary tuberculosis, and three (21%) were on anti-tubercular treatment at the time of referral for surgery. Ten patients (71%) had prior hospitalisation for cardiac failure. At the time of surgery, eight patients (57%) were in New York Heart Association (NYHA) class III-IV. The median duration of symptoms before surgical intervention was 15 months (range 11-24 months). Three patients (21%) had associated cardiac cirrhosis. Twelve patients (86%) underwent total pericardiectomy. Two patients (14%) underwent partial pericardiectomy. The mean operative time was 160 ± 33.8 minutes. The mean central venous press pressure before and after surgery were 28 ± 3.9 and 10 ± 2 mmHg respectively. The mean intensive care unit (ICU) and hospital stays were 4 ± 1.5 and 10 ± 2 days respectively. There was one (7%) 30-day mortality. There were two deaths (14%) due to non-cardiac causes at 10 and 16 months respectively. The remaining 11 patients (79%) are doing well (mean follow-up- 23 months), and are in NYHA class I. CONCLUSIONS: Tuberculosis is the most common cause of CCP in our region. Pericardiectomy provides definitive treatment to alleviate symptoms resolution and improve survival.