Kumar Abdul Rashid

Diagnosis and management of congenital bladder diverticulum in infancy and childhood: experience with nine cases at a tertiary health center in a developing country

ObjectivesThe purpose of the study is to present the authors’ clinical and surgical experience with congenital bladder diverticula in nine pediatric patients at a developing world tertiary care center.Patients and methodsRecords of nine patients diagnosed and treated as congenital bladder diverticula from 2000 to 2007 were retrospectively reviewed for age, sex, chief complaints, associated anomalies, investigative work-up, operative notes, and postoperative follow-up.ResultsAll were males. Age at presentation ranged from six months to eight years (mean three years). All were diagnosed postnatally by ultrasound and/or voiding cystourethrography (VCUG) and confirmed on urethrocystoscopy. Open surgical excision of diverticulum was done in all the patients. Ureteral reimplantation was simultaneously done only in three patients with VCUG-documented high-grade vesicoureteral reflux (VUR). With an average follow-up of four years, gradual resolution of symptoms was seen in seven of nine patients whose postoperative follow up records were available. There was no diverticulum recurrence at the defined mean follow-up.ConclusionPediatric patients with recurrent urinary tract infections and voiding dysfunction should always be evaluated for congenital bladder diverticulum. Although investigations such as intravenous urography (IVU), urodynamic studies, nuclear renal scanning, and, sometimes, CT scan and MRI, form an important part of preoperative diagnostic work-up and postoperative follow up, USG and VCUG may be enough when availability and cost are the constraints. Diverticulectomy, with ureteral reimplantation for high-grade reflux, provides good results without recurrence.

Congenital anterior penile isolated urethrocutaneous fistula: a case report.

Urethrocutaneous fistula is a common complication after hypospadias repair. If congenital, it is usually associated with other genitourinary and gastrointestinal anomalies. Isolated congenital urethral fistula is a very rare anomaly. We present a 4-year old circumcised boy with this unusual anomaly. Etiology, embryology, and management are discussed. We emphasize meticulous clinical examination for the diagnosis and to rule out other associated anomalies.

Congenital paraesophageal hiatal hernia with intrathoracic gastric volvulus in an infant: a case report with radiographic sequence.

Congenital paraesophageal hiatus hernia in children is a very rare condition, which may or may not be symptomatic [1–4]. The symptomatology of these patients is usually non-specific, in the form of repeated attacks of chest infection and/or recurrent vomiting, but can be associated with serious complications such as intra-thoracic gastric volvulus with incarceration and strangulation [1, 2, 5]. Plain chest radiographs, AP and lateral, may raise a suspicion of the condition, while upper GI contrast series is diagnostic [2, 6, 7]. The treatment is surgical consisting of excision of the hernial sac after reducing the stomach and repair of the diaphragmatic defect by tightening the crura of the esophageal hiatus [1, 2]. If the defect is large and associated with displacement of GEJ into the thorax, adding an antireflux procedure to the repair is appropriate [7]. This can be achieved transabdominally either by laparotomy or laparoscopically [2, 3, 7]. Congenital paraesophageal hiatal hernia being a very rare condition in infants, and more so when associated with volvulus of the intrathoracic stomach, a case with classical radiographic presentation is reported. The classical clinical and radiographic features are discussed with an anatomical interpretation. Case report

Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre.

Objective: To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome. Materials and Methods: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. Results: EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C. Conclusion Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

Tubularized Trapezoid Flap Neoumbilicoplasty: Simple Technique for Umbilical Reconstruction in Bladder Exstrophy

OBJECTIVES Umbilical preservation or reconstruction of a neoumbilicus has now become a part of exstrophy repair. We describe an easy method of umbilicoplasty concurrent with the initial bladder closure or at subsequent stages and present our experience with this technique in 36 patients during the past 5 years. METHODS Umbilical reconstruction using this technique was done in 36 patients (23 boys and 13 girls), with mean age of 1.5 years (range 2 days to 16 years). A superiorly based trapezoid skin flap was raised at the beginning of the procedure, with its base along a line joining the highest points on the iliac crests and leaving the umbilical remnant at the apex of the flap. At the end of the repair, the flap was vertically oriented and tubularized around the suprapubic and ureteral catheters. After removal of the catheters, the tubularized flap was allowed to follow the natural course of healing. RESULTS With a mean follow-up of 3 years (range 3 months to 5 years), all but 2 patients have developed a satisfactory dimpled umbilical scar. The sequence of events in the formation of an umbilical scar simulated that of the umbilical remnant in a newborn (ie, a cord to a proboscoid to a dimpled navel with a hidden central stalk). Even in the patients who developed wound infection, a cosmetic umbilicus was formed. CONCLUSIONS The technique is easy to perform, free of complications, and gives reproducible cosmetic results comparable to various other methods described in published reports.

Congenital duodenal obstruction with situs inversus totalis: Report of a rare association and discussion.

This report is to present and discuss an extremely rare association of situs inversus with duodenal atresia in an 11-day-old male neonate born full term and weighing 1.9 kg. The baby presented with recurrent bilious vomiting. Babygram revealed situs inversus and duodenal obstruction. Echocardiography showed dextrocardia with a small ASD. Exploration confirmed a duodenal diaphragm with a central perforation between the third and fourth part of the duodenum and situs inversus. The literature search revealed 20 cases reported so far.