Basant Kumar

Neonatal oral tumors: congenital epulis and epignathus.

Oroparyngeal tumors in neonates protruding from the mouth are extremely rare. They have the potential to cause respiratory distress, or even death of the child during the perinatal period owing to airway obstruction and may need a multidisciplinary team approach at the time of birth. Congenital epulis and epignathus are the 2 rare lesions usually presenting like this and clinical manifestations depend on their position and size. They have an excellent prognosis, if treated properly. This paper will discuss our experience with these 2 lesions and review the literature.

Single-stage repair of vestibular and perineal fistulae without colostomy

PURPOSE This retrospective study was undertaken to evaluate the feasibility of primary anorectoplasty without a covering colostomy using the anterior sagittal anorectoplasty (ASARP) or posterior sagittal anorectoplasty (PSARP) technique in patients having vestibular and perineal fistulae, its complications, results, and remote outcome in our institute. METHODS From January 2000 to June 2007, patients with vestibular and perineal fistulae subjected to single-stage surgical correction at our institute were reviewed retrospectively from the data available in hospital records and follow-up complaints of patients and their parents in the outpatient department. Patients who had undergone a staged repair were excluded from the study. All patients were assessed for immediate and delayed complications including continence of the neorectum. RESULTS From January 2000 to June 2007, 123 patients having vestibular (94) and perineal fistulae (29), age range from 28 days to 10 years, were subjected to primary repair either by the ASARP (34) or PSARP (89) technique. Follow-up period ranged from 3 months to 7 years. Mortality was nil. Constipation (25.68%) was the major long-term problem. Incontinence occurred in 1 patient (1.85%), who also had associated sacral agenesis. A total of 98.15% of patients were continent with stool frequency of 1 to 4 per day. Recurrence of fistula (0.81%), anal stenosis (6.76%), mucosal prolapse (2.70%), and anterior migration of the neoanus (1.35%) were the other major problems. Other minor problems like wound infection, superficial wound dehiscence, transient constipation, and diarrhea, etc, were successfully managed by local wound care, antibiotics, laxatives, enema, anal dilatation, and dietary changes. CONCLUSION Primary anorectoplasty either by PSARP or ASARP is feasible in vestibular and perineal fistulae without covering colostomy. Associated sacral agenesis/hypoplasia, redundant rectosigmoid or pouch colon, and wound infections with dehiscence are the major confounding factors affecting overall outcome. Better outcome in terms of continence can be achieved by careful surgical technique and follow-up along with proper toilet training. Complication rate was greater in cases of vestibular fistula than of perineal fistula, regardless of technique used. Some sort of laxatives and enema are often required. Dilatation of the neoanus for varying periods is also needed.

The human tail: rare lesion with occult spinal dysraphism—a case report

Human tail or tail-like caudal cutaneous appendage is a rare fingerlike, midline protrusion at the lumbosacrococcygeal region, often associated with occult spinal dysraphism. A 2-month-old male child presented here had a lumbosacral tail-like appendage with underlying spinal dysraphism without any appreciable neurological deficit. In contradiction to a previous report, true vestigial tails are not benign because they may be associated with underlying dysraphic state. About 50% of the cases were associated with either meningocele or spina bifida occulta. Management of such lesions must include complete neurological history and examination as well as magnetic resonance or computed tomographic imaging. After diagnosis, microsurgery should be performed if there is any intraspinal component to avoid any damage and neurological deficit.

Ectopia cordis associated with Cantrell's pentalogy.

Cantrells pentalogy with ectopia cordis is an extremely rare and lethal congenital anomaly, with a reported incidence of 1:100000 births in developed countries. We report a neonate who presented with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus, diaphragmatic defect, and interventricular septal defect. The neonate had respiratory distress with peripheral cyanosis and died because of acidosis and electrolyte imbalance before surgical intervention could be undertaken. We discuss the case and present a brief review of literature and of embryogenesis

Congenital bronchopulmonary malformations: A single-center experience and a review of literature

PURPOSE: To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature. MATERIALS AND METHODS: We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.8 months). RESULTS: Out of 25 patients, 18 (72%) were male and 7 (28%) were female. Age of patients ranged from 1 day to 11 years. The histopathological diagnosis was congenital cystic adenomatoid malformations [CCAM; n = 14 (56%)], congenital lobar emphysema [CLE; n = 5 (20%)], pulmonary sequestrations [PS; n = 3 (12%)], and bronchogenic cysts [BC; n = 3 (12%)]. Antenatal diagnosis was available in only 2 (8%) patients. The common presenting symptoms were respiratory distress and chest infections. Lobectomy was the procedure of choice . Mortality was 16% (n = 4; M: F = 3: 1). Two patients died because of overwhelming sepsis, one from compromised cardiac function, and one from aspiration which might possibly have been prevented. CONCLUSION: Patients with progressive respiratory distress due to these anomalies may require urgent surgical intervention regardless of age. The surgical outcome is favorable, with manageable complications. Plain x-ray chest and CT of thorax are usually sufficient for diagnosis and planning of treatment. Pathological diagnosis may differ from the imaging diagnosis. Mortality is found to be more in neonates. Apart from initial stabilization, resection of lesion and careful postoperative care is necessary to reduce mortality and morbidity.

Supernumerary testis: a case report and review of literature

Supernumerary testis or polyorchidism is a rare anatomical and congenital anomaly, and approximately 100 cases have been reported in the literature so far. It is often associated with processus vaginalis anomalies and with increased risk of malignancy and infertility. We report a case and its management, having left-sided duplex testis and found incidentally during inguinal hernia repair, and review the literature.

Primary duodenal tuberculosis presenting as gastric-outlet obstruction: Its diagnosis

Introduction: Gastrointestinal tuberculosis often involves the ileocecal region. Duodenal and gastric tuberculosis found in only 1% of patients suffering from pulmonary tuberculosis with associated HIV infection in non-endemic areas. Duodenal obstruction due to tuberculosis is very rare and needs high index of suspicions for diagnosis. Mostly this entity is suspected on intraoperative findings. In this manuscript we emphasized on ways and means for establishing histopathological diagnosis before starting anti-tubercular treatment in such cases. Method and Material: All patients of suspected gastroduodenal tuberculosis presented with feature of gastric-outlet obstruction managed during Jan 2009 to June 2011 were included in the study. After proper evaluation (routine hematological and biochemical examination, microbiological examination, serological and endoscopic evaluation) exploratory laparotomy was done and if there is no mesenteric lymphadenopathy or it is not safe to take biopsy form the diseased duodenum, multiple FNAC were taken from the diseased portion for histopathological and microbiological diagnosis. Result: A total of five patients were treated during this period. The most common presentation was vomiting followed by failure to thrive and weight loss; two patients had abdominal pain. Biopsy of mesenteric lymph node was possible in two cases. FNAC from diseases portion was taken in all cases. FNAC showed granulomas in four cases. Cases where even FNAC finding was non-conclusive on HPE/Microbiology was not subjected to antitubercular drug. Conclusion: Multiple intra-operative FNAC may be taken from the diseased portion of the duodenum to establish the histopathological diagnosis if diagnosis is not established by any other mean.

Spontaneous biliary peritonitis: Is bed side diagnosis possible?

Background: Spontaneous biliary peritonitis is a rare cause of acute abdomen. In spontaneous biliary peritonitis there is perforation in the wall of the extra-hepatic or intra-hepatic duct occurs without any traumatic or iatrogenic injury and have been described more often in neonates. The symptoms may be acute or insidious delaying the diagnosis. Present manuscript deals with diagnosis and management of these cases. Materials and Methods: This is a prospective study and all patients of suspected biliary peritonitis presented during Dec 2010 to Feb 2012 were included in the study. After preliminary investigations in all patients abdominal paracentesis was done and in cases where intra-abdominal fluid bilirubin level was several fold higher than serum bilirubin level were subjected to exploratory laparotomy. Further investigation like T-tube cholangiogram and magnetic resonance cholangiopancreatography (MRCP) was done to rule out choledochal cyst before leveling these cases as SPBD. Results: A total of 6 patients were included in present series commonest presenting symptom was progressive abdominal distension without signs of overt peritonitis followed by progressive jaundice, fever and abdominal pain. On exploration site of perforation was observed in 50% of cases and in 50% of cases bile duct was not dilated. Second surgery was not required in 34% of cases. There was no mortality or significant morbidity in our series. Conclusion: Spontaneous perforation of bile duct is rare disease and high index of suspicion is required for diagnosis. Simple bed side test can help in diagnosis but T tube cholangiogram or MRCP are must to rule out choledochal cyst.

Congenital urethrocutaneous fistula: Case report with review of literature.

We are presenting two cases of congenital urethrocutaneous fistula on ventral penile shaft. Congenital urethral fistula is an extremely rare, but easily manageable anomaly that may be confused with hypospadias. Awareness of the entity will avoid complications. This condition may be associated with other anomalies like congenital hernias and anorectal malformations. Treatment of this entity is individualized according to site of fistula, associated anomalies and condition of the distal urethra. All the principles of hypospadias surgery should be strictly followed.

Right lung agenesis.

Congenital pulmonary agenesis or aplasia is extremely rare. Although more than fifty percent of patients die before first five years of age, some individuals may remain asymptomatic throughout their life. A three-month-old female child with right pulmonary agenesis presented to us with severe respiratory distress. She was misdiagnosed as a case of foreign body bronchus at the peripheral health centre. Bronchoscopy confirmed the diagnosis and relieved the symptoms. It is recommended that invasive diagnostic procedures and prophylactic surgery should not be done in asymptomatic cases.