Ashish Wakhlu

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.

Melanotic Neuroectodermal Tumor of Infancy: 2 Decades of Clinical Experience With 18 Patients

PURPOSE The purpose of this article is to report our experience in the management of 18 patients with melanotic neuroectodermal tumor of infancy involving the maxillary alveolus. PATIENTS AND METHODS All patients presented with hard nontender swelling involving the upper alveolus with facial deformity. Analysis included hematocrit, coagulation profile, serum creatinine, and screening for vanillyl mandelic acid and catecholamines. Imaging studies included x-ray of the maxilla and chest, ultrasound of the abdomen, computed tomography scan (1990 to 1999), and magnetic resonance imaging (after 1999). All surgeries were performed using endotracheal anesthesia, and complete gross excision of the tumor was achieved with coverage of the defect with mucoperiosteal flaps. All specimens were subjected to histopathology and immunohistochemistry. RESULTS The expansion of the alveolus produced by the tumor improved in 4 to 6 months. Subsequent dentition was affected by the removal of involved tooth buds during the operation. All the patients are in regular follow-up (maximum 206 months) and there has been no local recurrence or distant metastasis. Mean follow-up time was 130.8 months (95% confidence interval, 168.8-210.6). Overall survival at 17 years was 85.6%. Median survival could not be established due to statistically insignificant sample size, while mean survival time was 189.7 months (95% confidence interval, 103.7-157.8). CONCLUSIONS In the absence of metastatic disease, melanotic neuroectodermal tumors of infancy can be successfully managed by local excision.

Vaginal agenesis: Experience with sigmoid colon neovaginoplasty.

Aim: Objective of this study is to report our experience with sigmoid vaginoplasty in adolescents. Materials and Methods: A retrospective study of children with vaginal atresia and Mayer–Rokitansky–Kuster–Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the cases. Results: Eight children were studied over a period of 7 years. The postoperative complications were ileus in 2, mucosal prolapse of the neovagina in 1, and minor wound infection in 1 patient. Seven patients are on regular follow-up. All the neovaginas were patent and functional. One patient had unacceptable perineal appearance, that is, badly scarred perineum as a late complication. None of the patients had vaginal stenosis or excessive mucus discharge, during follow-up visits. Out of the 7 patients, 2 patients are sexually active and satisfied. Conclusions: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, and short recovery time.

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.

The role of endoscopic retrograde cholangiopancreatography in the management of choledochal cysts in children

Endoscopic retrograde cholangiopancreatography (ERCP) was performed in eight consecutively treated patients with choledochal cyst to assess its technical feasibility and complications. Satisfactory visualization of the complete anatomy of the biliary tract was possible in seven patients. Four patients had a type IV choledochal cyst and three had type I. In one patient, the procedure showed a congenital web in the lower end of the common bile duct; in another, there was complete membranous block of the lower end of the common bile duct. The findings of ERCP and intraoperative cholangiography were concordant for six patients. Only one patient had exacerbation of preexisting cholangitis. The authors conclude that ERCP is an essential investigative method in the management of choledochal cysts in children.

Long-term results of anterior sagittal anorectoplasty for the treatment of vestibular fistula

PURPOSE Vestibular fistula is the commonest anorectal malformation in the female child. This article reports the treatment and long-term follow-up of 1206 patients of vestibular fistula treated by anterior sagittal anorectoplasty (ASARP) in a single center for 38 years. MATERIAL AND METHODS All patients of vestibular fistula admitted and operated on at the Department of Pediatric Surgery, King George Medical University (Lucknow, UP India), from 1970 were included in the study; the age ranged from 2 days to 40 years. The diagnosis was made by clinical examination. We differentiated between anovestibular fistula (AVF) and rectovestibular fistula (RVF) in that the latter is a longer narrow fistula closely applied to the posterior wall of the vagina. Preoperative investigations included hemogram and blood glucose. Echocardiography was done in those patients showing a physical sign of cardiac anomaly. All patients were operated on in the lithotomy position by ASARP; this was done without colostomy in 1169 patients. In 6 patients, preliminary colostomy was done because of excessive perineal excoriation, and 31 others had colostomy done elsewhere. The striated muscle complex was delineated by electrostimulation, and anoplasty was performed after anchoring the rectum within the muscle complex. Washing of the perineum after passage of stools with application of povidone-iodine ointment constituted the local care. Intravenous antibiotics were administered for 48 hours and oral antibiotics (including metronidazole) for 5 days. The patient was discharged home by the fifth day. RESULTS Follow-up ranges from 3 months to 19 years; uneventful postoperative recovery was seen in 1147 patients. They had normal growth and development, normal appearance of the perineum, and a normal quality of life. Complications were seen in 60 patients (5%) of which 42 had AVF and 18 had RVF. Eight patients had postoperative wound disruption that was minor in 4 and required colostomy in the other 4. Four patients had recurrence of vestibular fistula thus creating an iatrogenic perineal canal; this could be repaired by a second ASARP in 3 patients and required colostomy and PSARP in one child. Anal stenosis was seen in 11 patients; this was treated by dilatation alone in 6 and required posterior Y-V plasty in 5. The rate of complications in RVF was lower than AVF probably because of lesser number of patients; there was no difference in stooling pattern or continence between uncomplicated patient of RVF and AVF; however, fecal staining was seen in all patients undergoing revision surgery for complications. CONCLUSIONS This experience with ASARP showed a good result in 95% patients in a single-stage procedure. The technical ease and minimal preoperative and postoperative measures make ASARP the procedure of choice for vestibular fistula in females at all ages. Until sufficient experience is gained, it may be safer to operate on patients with RVF under cover of a protective colostomy.

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.

Four-country surveillance of intestinal intussusception and diarrhoea in children.

Aim:  Establishment of baseline epidemiology of intussusception in developing countries has become a necessity with the possibility of reintroduction of rotavirus vaccine. The current study assessed the seasonal trend in cases admitted with intussusceptions and dehydrating acute watery diarrhoea in children aged 2 months to 10 years.

Congenital lumbar hernia: 20 years' single centre experience.

Aim:  Congenital lumbar hernia is an uncommon anomaly with only few cases reported in the English literature. This study was done to study the incidence, associated conditions, management and complications of congenital lumbar hernia repair in paediatric patients.