Retrospective study of results of Kawashima procedure.

BACKGROUND: Patients with single ventricle physiology and an interrupted inferior caval vein undergo the Kawashima procedure. With increasing follow-up, pulmonary arteriovenous malformations may develop requiring hepatocardiac venous redirection. METHODS: Between January 2005 and December 2012, 15 patients underwent the Kawashima procedure. Preoperative and postoperative characteristics along with functional class, intra-operative details, post-operative course, saturations and haematocrit were recorded. RESULTS: Median age at operation was five years (range 1- 20 years). Five patients had antegrade pulmonary blood flow interrupted and 10 patients had open antegrade flow. There were no deaths. Mean saturation in postoperative period was 90.8 ±1.36%. Median duration of mechanical ventilation and inotropic support was six and 16hours respectively. Median duration of pleural drainage was six days. Median duration of pleural drainage in antegrade flow open and interrupted group was six days and four days respectively. The mean hospital stay was 10.5 days ±7.1 days. Mean follow up was 40.2 ±31 months (median-30 months). Nine patients (60%) were in New York Heart Association (NYHA) functional class 1 and five patients (33.3%) were in NYHA functional class 2 at last follow-up. Only one patient was in class 3 and needs completion Fontan. CONCLUSIONS: The Kawashima procedure can be safely performed with acceptable early results. Although studies have shown the risk of pulmonary arteriovenous malformations after Kawashima procedure in the mid and long-term, our findings do not support this. Kawashima procedure with open antegrade pulmonary blood flow as a definite intervention in such patients is debatable.

Foreign body aspiration with iatrogenic right bronchus perforation - A case report and literature review.

INTRODUCTION AND IMPORTANCE: Evacuation of obstructed trachea foreign body (FB) can be challenging and may have several life-threatening complications. Iatrogenic tracheobronchial injury (TBI) is a rare and devastating complication which need prompt diagnosis and management. CASE PRESENTATION: An 11-year-old child was brought to the emergency with a history of cough and chest discomfort for the last 15 days. Chest x-ray and computed tomography (CT) chest were done which showed the presence of a FB in the right main bronchus. After all pre-operative investigations, rigid bronchoscopy and removal of the foreign body under general anesthesia was performed. After several attempts, the foreign body couldn't be removed and there was massive surgical emphysema of the chest. Immediately bilateral chest tube was inserted. A repeat CT chest was done which revealed a ruptured of the right main bronchus with migration of the foreign body to the right pleural cavity. Right thoracotomy was performed under general anesthesia. The foreign body was removed and the bronchus ruptured was repaired. The child's vital signs were normal throughout the procedure and she was discharged on the seventh post-operative day. CLINICAL DISCUSSION: TBI can have devastating scenarios in the absence of quick diagnosis and treatment. Around 80 % of patients with TBI due to accidents have been found to have fatal outcomes before reaching the hospital, probably due to tension pneumothorax, hypoxia, or respiratory failure. CONCLUSION: Prompt diagnosis and management of complications with definitive repair of the injury were key elements in saving lives after TBI.

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.

Penetrating Chest trauma with right atrium rupture - A case report.

Background: Penetrating chest trauma can have lethal consequences. Anterior chest wall penetrating injury can cause fatal lacerations over the chest wall, lungs, pericardium, and the heart. There are few reports on these kinds of injuries and operative management. Purpose: To describe a case of penetrating chest trauma with right atrium ruptured, after a fall from height. The patient was successfully managed with prompt resuscitation and definitive surgical intervention. Case presentation: A 48-years-old gentleman presented with history of fall from height and right sided penetrating chest injury. The patient had a penetrating injury to the right-fourth intercostal space in parasternal region. Chest X-ray showed massive right-sided hemothorax and chest tube drained more than 1700 ml upon insertion. Emergency right thoracotomy was performed, after initial resuscitation with fluids and blood transfusion in the emergency department. He had injury to the intercostal arteries, lacerations of the pericardium and the right atrium ruptured, which was managed successfully with definitive repair. Conclusion: Survival is rare after penetrating chest trauma with right atrium ruptured if not intervened on time. Prompt diagnosis and resuscitation, along with the definitive surgical repair were the key elements for successful management of the patients.

Pin-tract myiasis after external bone fixation: A case report and review of literature.

INTRODUCTION AND IMPORTANCE: Myiasis has been reported as a complication of fracture treatment with external fixation. Therefore, physicians should be aware of the possible re-emergence of myiasis as a complication of surgery and the use of metal fixators. CLINICAL PRESENTATION: A 45-year-old male, non-diabetic, chronic alcoholic treated with external bone fixation and flap coverage for Gustilo-Anderson type IIIB comminuted fracture of shaft of left tibia and fibula with intact distal neuro-vascular system presented with multiple maggots with foul-smelling discharge from the pin tract. A diagnosis of abscess with pin-tract myiasis was made and managed with wound debridement and complete removal of maggots. CONCLUSION: We report this rare pin-tract complication to acknowledge how simple precautions, wound care, and avoidance of risk factors play a vital role in preventing such infestations. Therefore, physicians should be aware of the possible re-emergence of myiasis as a complication of surgery and the use of metal fixators.

Hyoscine butylbromide induced psychosis: A case report.

Hyoscine butylbromide-induced pyschosis, though rare, should be considered in a child presenting with deteriorating cognitive functions and psychotic features acutely as evident in our case of a 9-year-old child taking hyoscine for her non-specific abdominal pain.

Sebaceous carcinoma of trunk with bilateral axillary lymph node metastasis: a rare presentation of malignant adnexal tumor in young adult.

Malignant adnexal tumors of skin are rare. Sebaceous carcinoma-a rare and aggressive cutaneous tumor-is frequently located in the periorbital region. Malignant adnexal tumors of sebaceous carcinoma type on trunk with axillary lymph node and distant metastasis in young adult is rare. The incidence of extraocular sebaceous carcinoma has been expected to be 0.06 per 100 000 person-years with an increased incidence in elderly patients and men. With a note of mimicking benign dermatologic conditions, definitive diagnosis of sebaceous carcinomas is often hindered, increasing morbidity and mortality for patients. Herein, we present a case of a 35-year-old man with a large ulcero-proliferating lesion of trunk region previously resected for a small swelling and eventually presented with the recurrent lesion and diagnosed as sebaceous carcinoma of trunk with bilateral axillary lymphnode and distant bone metastasis demonstrating several diagnostic and management challenges.

Ileosigmoid knotting: A case report of unique cause of acute abdomen in a Nepalese patient.

INTRODUCTION AND IMPORTANCE: Ileosigmoid knotting is a surgical emergency. Common in Asian males, it is presented with signs and symptoms of diffuse peritonitis. Aggressive resuscitation with intravenous fluids and antibiotics followed by workup enable the patient hemodynamically stable. Explorative laparotomy followed by colo-colic anastomosis and double barrel ileostomy is curative. CASE PRESENTATION: A 32-year-old-male from Nepal with a chief complain of diffuse abdominal pain for a day and signs of peritonitis presented to Emergency room. After assessing and stabilizing the patient, explorative laparotomy was performed. Ileal and sigmoid resection followed by colo-colic anastomosis and double barrel ileostomy was done. On regular follow-up, he is in his usual state of health. DISCUSSION: The incidence of ileosigmoid knotting is reported to be about 1.6 cases per year. It is often misdiagnosed as non-specific intestinal obstruction which confers poor prognostic value to the patient. It should be identified promptly and intervened timely. CONCLUSION: Ileosigmoid knotting is a rare but dangerous differential of acute abdomen not to be missed. Early assessment and quick decision making is the key to provide excellent prognosis to the patient in need, provided adequate surgical skills are demonstrated.

Intermediate syndrome following organophosphate poisoning: A case report in a low resource and poor socio-economic setting.

Intermediate syndrome with respiratory failure is a serious complication that can be fatal as in our case of a 24-year-old-man who developed intermediate syndrome requiring intubation and respiratory support. Furthermore, the patient's socio-economic situation significantly impacts the illness's progress and prognosis.

Von Hippel-Lindau disease: A case report.

INTRODUCTION AND IMPORTANCE: Von Hippel-Lindau (VHL) disease is a rare autosomal dominantly inherited genetic condition. Von Hippel characterized the illness independently in 1911, and Lindau in 1926. Its prevalence is estimated to be about 1 in every 36,000 live births. VHL is characterized by the production of several benign and malignant tumors, as well as cysts in other organs. For proper prognosis, good clinical judgement and timely diagnosis is warranted. CASE PRESENTATION: Herein, we report a case of a 50-year-old man with several central nervous system (CNS) lesions, retinal lesions, and renal cortical cysts with a diagnosis of VHL disease who was surgically treated. At a 3-month follow-up, he improved drastically with a marked alleviation of his signs and symptoms. DISCUSSION: VHL is characterized by the creation of various benign and malignant tumors, as well as cysts in multiple organs, and is passed down through generations in an autosomal dominant pattern with near-complete penetrance. CNS lesions are surgically treated. Regular follow-up should be ensured. CONCLUSIONS: VHL disease is an extremely complicated disease with the need for diagnosis and genetic tests in the patient and family members, as well as intensive supervision of carriers of the mutated gene, thereby improving early diagnosis and successful treatment of the malignancies. The high cost of diagnostics and surgical therapies is a severe issue. Government care and financial assistance are critical considerations.

Giant occipital encephalocele complicated with obstructive hydrocephalus: A case report.

Introduction: and importance: An encephalocele is a type of congenital neural tube defect defined by herniation of intracranial contents via a cranial defect. When an encephalocele is greater than the size of the head, it is referred to as a "giant encephalocele." The occurrence of encephalocele has been documented to be 1-4 instances per 10,000 live births. Surgery is challenging. Case presentation: A 1-month-old baby boy sustained a huge swelling on the back of his head since birth, and it increased gradually over time. On examination, he had a huge occipital swelling measuring about 20 × 15 × 17 cm in size. A diagnosis of giant occipital encephalocele was established. Surgical excision and repair was done. After 1 month, he developed obstructive hydrocephalus and a ventriculo-periotoneal shunting was performed. On regular follow-up, he is in a good state of health. Discussion: Surgery imposes challenges for the anaesthesiologists and neurosurgeons due to its complex site, enormous size, intraoperative blood loss, and prolonged anaesthesia. A team approach is necessary for its successful treatment. Conclusions: Based on our experience, we would like to deliver following recommendations in the surgical management of giant encephalocele. Surgery should be done quickly to ensure good prognosis. Proper positioning, efficient intubation, infections and sepsis control should be emphasized. Also, fortification of food with folic acid, as well as increased education and awareness of women on the need for antenatal care may also decrease the risk of this disease.

Invasive cutaneous squamous cell carcinoma of scalp: A case report.

Introduction and importance: Cutaneous Squamous cell carcinoma(cSCC) represents approximately 16% of scalp tumors. Overall, cSCC is significantly more common in the oropharyngeal mucosa than the skin. Smoking is a risk factor for mucosal lesions, risk factors for developing cutaneous SCC include age, ultraviolet light exposure, chronic scarring, history of ionizing radiation, androgenetic alopecia (in men), and immunosuppression. The rates of bone invasion in cutaneous SCC have not been well noted in the literature. Case report: We report a case of 57-year-old man with invasive squamous cell carcinoma of scalp with extension into bone, cortex and dura mater. Gross total removal of the tumor with extension of bony defect followed by repair of dura and repair of skin defect by VY advancement flap was done under general anaesthesia. At a 3-month follow-up, his wound is well healed. No evidence of metastasis is noted. Discussion: Cutaneous squamous cell carcinoma (cSCC) is the second most common non-melanoma skin cancer, and its incidence is steadily increasing. Although the majority of cSCCs are successfully eradicated by surgical excision, advanced cSCC poses a significant risk in terms of morbidity, impact on quality of life, and risk of death. Conclusions: Invasive cutaneous squamous cell carcinoma of scalp is a rare entity. Invasion to bone, cortex, and dura mater is furthermore rare. Therefore, proper management of advanced cSCC is of the utmost importance since local invasion, delayed diagnosis, and metastasis contribute to increased costs and morbidity. A multi-disciplinary team approach is recommended.

Large posterior mediastinal ganglioneuroma with intradural cervical spine extension: A rare case report and review of literature.

Introduction: Ganglioneuroma (GN) is a rare benign tumor of ganglion cell origin and can occur anywhere along the sympathetic chain. These tumors can grow to a significant size without any symptoms unless they exert a mass effect on the region they grow and start showing symptoms. Spinal extensions are rare and they may produce neurological symptoms warranting further investigation. Case presentation: We described a case of posterior mediastinal ganglioneuroma in a 4-year-old boy with cervical extension who presented with quadriparesis. The radiological scan revealed large ganglioneuroma having an intradural extramedullary extension with a large posterior mediastinal component compressing and displacing the surrounding structures. He underwent consecutive surgeries for complete excision of the tumor following which he regained his power in his upper and lower limbs over the period of 2 months. Conclusion: Para spinal mass with consistent radiological features suggests ganglioneuroma but confirmation should be done with biopsy. Complete excision of the tumor is the treatment of choice with close follow-up for clinical improvement and recurrence.

Benign rib tumors: a case series from tertiary care Centre of Nepal and review of literature.

Rib tumors are rare. They can present with wide varieties of symptoms and differentiating benign from malignant lesions is challenging. A careful history and supporting investigations can help to reach the definite diagnosis. Surgical resection is the treatment of choice with excellent prognosis. A total of four patients were operated for the management of rib tumors over a period of 5 years at our institution. All four were benign lesions and included simple bone cyst, fibrous dysplasia with aneurysmal bone cyst, aneurysmal bone cyst and enchondroma. There were three male and one female. None of them is having recurrence till now. Surgical resection is the treatment of choice for benign rib tumors as it provides diagnostic and curative treatment.

Pulmonary sequestration presenting as a massive haemoptysis in adult: A case report.

INTRODUCTION: Pulmonary sequestration is a rare congenital lung malformation characterized by non-functioning mass of pulmonary tissue that lacks normal communication with bronchial tree and receives one or more aberrant systemic arterial supply. It usually presents in children with recurrent chest infections. It is uncommon in adult and remains asymptomatic or present with recurrent chest infections, rarely with more severe symptoms like a massive haemoptysis as in seen in our case. CASE PRESENTATION: A 22-year-old male presented with a chief complain of multiple episodes of massive haemoptysis. After evaluation with chest x-ray, CT chest and angiogram, he was diagnosed to have intralobar pulmonary sequestration. He underwent successful thoracotomy and left lower lobectomy. He is asymptomatic after one year of follow up. DISCUSSION: Pulmonary sequestration is rare in adult and can present with various symptoms like chest pain, cough, sputum production, recurrent infection and rarely haemoptysis. Intralobar sequestration of left lower lobe is the most common as seen in our case. The diagnosis can be made by Computed Tomogram chest with angiogram. Surgical intervention is the definitive curative treatment. Post-operative outcome is excellent with early patient satisfaction and promising outcome in long term follow-up. CONCLUSION: Bronchopulmonary sequestration can rarely present in adults presenting with massive haemoptysis. CT chest is the best modality for diagnosis. Early surgical intervention is definitive treatment with good long term outcome.

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.

Single-stage hepaticojejunostomy for symptomatic portal biliopathy in a splenectomized patient: A report of two cases.

Single-stage biliary decompression without prior shunt surgery, although risks for catastrophic bleeding, it is feasible to perform upfront hepaticojejunostomy in a splenectomized patient where shuntable vein is not available in patient with portal biliopathy.

Outcome of Ligation without Revascularization in Pseudoaneurysms of Peripheral Arteries in Intravenous Drug Users.

INTRODUCTION: Management of pseudoaneurysms in intravenous drug users is complex and challenging due to an associated infection and unavailability of autologous vein grafts. Here we observe the outcomes of ligation and local debridement as a primary modality of treatment in this subset of patients with pseudoaneurysms. METHODS: This is a descriptive cross sectional study of 15 patients over a period of 4 years who presented with pseudoaneurysm of peripheral artery from intravenous drug use. In this study, we describe the presentations and management outcomes in 15 patients with peripheral arterial pseudoaneurysmfrom IV drug use. RESULTS: The most common site involved was common femoral artery among 12 (80%) patients followed by superficial femoral artery among 8 (13.3%) patients and external iliac artery in 1 (6.7%) patient. Twelve (80%) patients were having signs of infection. All patients underwent surgical intervention which comprised of excision of pseudoaneurysm and ligation of artery without revascularization among 12 (80%) patients and with revascularization with autologous venous graft among 3 (20%) patients. There was no mortality or a major bleeding requiring re-exploration. None of the patients developed limb ischemia necessitating amputation.One patient with femoral artery ligation without revascularization at one year of follow up is having claudication on brisk walking.There was one saphenous vein graft thrombosis in immediate postoperative period. CONCLUSIONS: With the use of ligation without revascularization technique, there was no mortality or major bleeding requiring re-exploration. None of the patients developed limb ischemia necessitating amputation so this treatment modality seems promising in treatment of pseudoaneurysms in intravenous drug users.

Simple Bone Cyst of Rib.

Simple bone cyst is a benign lytic lesion usually encountered in children and adolescents. It is a cystic, fluid-filled lesion that usually involves the metaphysis of the long bones. Simple bone cyst of the rib is very rare, with few cases reported in the literature. Here we describe an interesting case of a simple bone cyst of the sixth rib in a 15-year-old girl that was managed successfully with surgical excision.

Giant Calcified Retroperitoneal Teratoma.

Retroperitoneal teratoma involving adrenal gland is a rare entity in adults comprising only 4% of all primary teratomas. These tumors are mainly seen in infants and children. Diagnosis is made by X-ray and CT abdomen showing the presence of large tumor with cystic and calcified walls. Here, we present an interesting image of a 16-year-old girl who presented with large (12 × 10 cm) calcified cyst occupying left side of abdomen which was treated successfully by surgical excision.