S. N. Kureel

Congenital Short Colon

Abstract. Congenital short colon (CSC) is a condition in which the colon is replaced, wholly or partially, by a dilated pouch together with anorectal malformation and a colourinary fistula. Most of the reported series have been from northern India where this malformation is frequent. This paper details the management of 108 patients with CSC over a period of 23 years. The patients were classified into two types: (1) Partial short colon, where a segment of normal colon is present between the ileum and the sac. These patients could be treated by excision of the pouch and pull-through of the segment of normal colon during either single- or two-stage surgery. (2) Complete short colon, where the ileum opened directly into the sac, and formation of a tube from this sac (coloplasty) was required in one or more stages to provide a length of normally functioning colon. Review of the results showed staged management to be superior. The follow-up has ranged between 3 and 7 years, with satisfactory long-term results in both groups of patients. We have evolved a protocol for the management of CSC that has improved the prognosis and quality of life of these patients.

Anterior sagittal anorectoplasty for anorectal malformations and perineal trauma in the female child

Vestibular fistula and perineal ectopic anus are the most common anorectal malformations in female children. Anterior saggital anorectoplasty (ASARP) was used to treat 416 patients with these anomalies, as well as cases of perineal canal and third-degree perineal tear, during a 20 year period. Preliminary colostomy was performed in only four patients, who had a perineal tear. For ASARP, a midline incision was made and the rectum was separated from the vagina and placed in the center of the sphincteric muscle complex. The perineal body was reconstructed, and normal appearance of the perineum was achieved. Six patients had complications postoperatively (4 had secondary hemorrhage, 2 had wound infection). One patient with hemorrhage and one with infection required laying open of the wound and revision surgery after 12 weeks. Both recovered well. Of the 416 patients, 397 returned for follow-up 12 weeks after the surgery. Of these, 390 had a normal-looking perineum and normal defecation. Of the remaining seven patients, three had retraction of the rectum, one had recurrence of the fistula, and another had acquired perineal canal. These five children required revision ASARP and recovered successfully. The other two patients had mucosal prolapse, which reduced spontaneously. Three hundred twenty-six children of the 416 patients have had follow-up beyond the age of 3 years. Of these, 291 had normal bowel habits without the use of laxatives. Of the other 35, four had fecal impaction of unknown etiology, which was managed conservatively, 6 had anal stenosis (four underwent posterior Barrows flap interposition and two responded to dilatation), and 25 had a posterior ledge that caused constipation and required cutback. Overall, eight patients (1.9%) required revision ASARP and 40 others (9.8%) had minor complications. This operation provides consistently good results, and the authors recommend it for the management of anorectal malformations and perineal trauma in female children.

Delayed presentation of anorectal malformations

Aims and Objectives: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined “delayed presentation of ARM” and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. Materials and Methods: Patients satisfying the criteria of “delayed presentation of ARM” were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. Results: Between 2003 and 2006, 43 patients satisfied our criteria of “delayed presentation of ARM”. There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These “delayed presenters” had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. Conclusion: “Delayed presentation of ARM” is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases

BACKGROUND Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. Although they are referred to as valves, these obstructive structures often occur in the form of a diverticulum. The urethra in these cases shows saccular or bulbar dilatation known as anterior urethral diverticulum (AUD). They typically occur where there is a defect in the corpus spongiosum, leaving a thin-walled urethra. This segment of the urethra balloons out during voiding, simulating a mass that is sometimes visible along the ventral wall of the penis. The swelling is fluctuant and urine dribbles from the meatus on compression. The present study highlights the clinical approach in identifying the condition and its treatment options, especially for those, presenting with urethral diverticula. MATERIALS AND METHODS We have studied children with congenital anterior urethral valves and diverticula. Six patients of AUVs with diverticula were admitted during the period of 2000-2007 and were prospectively evaluated. The mean age of presentation was 16 months (15 days to 4 years). Straining at micturition and a palpable penile swelling were the most common presenting features. The diagnosis was established by voiding cystourethrogram (VCUG) and supported by ultrasonography (USG). All patients were treated with single-stage open surgical excision except one who died preoperatively due to urosepsis. Initial lay opening of the penoscrotal urethra and delayed repair were done in one patient. RESULTS The surgical outcome was successful in all but one patient, who died of delayed presentation with severe back pressure changes, urinary ascitis and urosepsis. On long-term follow-up, all patients demonstrated good stream of urine. The renal functions were normal and the patients had no evidence of urinary infections. CONCLUSION We propose that, the patients of AUVs, if not excessively delayed for treatment are otherwise well in terms of general condition as opposed to patients of posterior urethral valves. The diagnosis is easily established by VCUG and the severity is revealed by a sonogram. Open surgical excision is the method of choice for patients with a urethral diverticulum; however, cystoscopic fulguration is also feasible in selected patients. The outcome is excellent with minimal morbidity and mortality.

Unusual Ventriculoperitoneal Shunt Extrusion: Experience with 5 Cases and Review of the Literature

Distal migration of ventriculoperitoneal shunt is rare. We present this unusual complication in 5 patients. The lower end of the shunt was extruded from right lumbar region, cervical area, umbilicus and rectum. The cause of such extrusion is not known. The patients were managed by shunt removal followed by shunt replacement on the opposite side.

Delayed presentation of strangulated congenital diaphragmatic hernia: learning from our experience

BackgroundStrangulation is very rare in congenital diaphragmatic hernia (CDH) of the Bochdaleck variety. Here, we share our experience with six cases of delayed presentation of strangulated CDH. The aim of this article is to provide information on how to diagnose and manage this situation using a systematic approach.Materials and methodsA retrospective review identified six cases of strangulated/obstructed CDH from 1998 to 2011. Demographic data, clinico-radiological findings, management and complications, along with final outcome were recorded.ResultsSmall bowel gangrene was found in one patient, gastric perforation in three, transverse colon perforation in one and colonic obstruction in one patient. Video-assisted thoracoscopic surgery (VATS) was used in all but one patients for definitive diagnosis, diaphragmatic repair, pleural lavage and management of empyema. Laparotomy was needed for management of strangulated or perforated bowel. Three patients in this study survived.ConclusionClinicians should always consider a diagnosis of obstructed Bochdaleck hernia in children, because X-ray findings are not always typical or even normal in complicated CDH. VATS may be considered as both diagnostic and therapeutic. Preventive measures for empyema or early intervention in the evolving stage can significantly reduce morbidity.

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.

NON SURGICAL MANAGEMENT OF CYSTIC LYMPHANGIOMA

Aim/Purpuse: To evaluate our experience of 19 patients of lymphangioma who were treated by intralesional Bleomycin.Materials and Methods: Nineteen patients of lymphangioma aged between 16 days to 11 years were managed in the department. The male-female ratio was of 2:1. Commonest sites were in the neck (58%) followed by axilla (21%). The patients were treated by intralesional bleomycin injection. Bleomycin was given at a dose not exceeding 0.5 unit/kg/dose at interval of 2 weeks. Reduction in size of the mass was noted in between 2 weeks to 16 weeks and number of injections required for each patient varied from 1 to 6. Follow up ranged from 1– 7 Year.Result: In injection group, significant reduction of mass was noted in 84% (n=l6) and 57% (n=11) of them showed complete disappearance. No serious complications were noted in any patient.Conclusion: Our experience showed that Bleomycin in aqueous solution is a good sclcrosing agent in the management of lymphangioma.

Antireflux status post Roux-en-Y anastomosis: An experimental study for optimal antireflux technique

Background: Roux-en-Y hepaticojejunostomy has been a gold standard to establish biliary-enteric anastomosis for various surgical indications, but associated with variable incidences of cholangitis. This experimental study was conducted to report a modification in Roux-en-Y anastomosis for possible better alternative to provide antireflux procedure after Roux-en-Y biliary-enteric anastomosis with the aim to minimize the possibility of reflux and its consequences. Materials and Methods: For experimental study, the required fresh segment of Lambs small intestine was procured. Three sets of Roux-en-Y anastomosis were created for each experiment. In set 1, there was simple Roux-en-Y anastomosis. In set 2, Roux-en-Y anastomosis along with 4–5 cm long spur between the hepatic and duodenal limbs was created. In set 3, in addition to Roux-en-Y with creation of spur, additional antireflux mechanism was created at the junction of upper two-third and lower one-third of the hepatic limb. Saline mixed contrast was infused by infusion pump to raise the intraluminal pressure to more than 10 cm of H2O. X-ray was taken at that time. Results: In set 1, all preparations demonstrated reflux of contrast in the hepatic limb. The set 2 also demonstrated the same findings of 100% reflux in the hepatic limb. In set 3, No reflux was observed in 8 (80%) preparations while remaining 2 (20%) preparations reveal partial reflux. Conclusion: This experimental study suggests that the provision of spur and additional valve may be able to decrease the possibility of reflux in Roux-en-Y biliary-enteric anastomosis.

IschioPubic osteotomy, A simple and effective technique for pelvic ring closure in repair of classic exstrophy bladder

Aim: The aim of this study is to report the technique and outcome of ischiopubic (IP) osteotomy for pelvic ring closure in classic exstrophy bladder. Methods: A total of 85 male classic exstrophies were selected based on trapezoid-shaped space between IP ramus on three-dimensional computed tomography pelvis. Using midline scroto-perineal approach, after bladder plate mobilization and radical corporal detachment; the pelvic surface of superior pubic ramus (SPR) was exposed. Above the obturator canal, H-shaped incision was made on periosteum of the SPR. The horizontal line of H was placed above the obturator canal. Rectangular periosteal flaps above and below the horizontal line was raised. In the subperiosteal plane, curved hemostats encircled the SPR that were divided using a bone drill. On the medial aspect of ischial tuberosity, a notch was created as hinge using a bone drill. Forks of bone holding forceps hooked the pubic bone and tightened for its midline approximation, lengthening of the SPR and inward rotation of IP ramus. Linea alba and pubic bones were approximated with interrupted Polygalactin sutures. Results: Midline approximation of pubic bone and linea alba was possible in all. There was no injury to obturator nerve, vessels, or other structures . Conclusions: IP osteotomy is the safe and effective technique of pelvic ring closure in patients with specific pelvic configuration.