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Intussusception is one of the common causes of acute abdomen in early childhood, particularly in children younger than two years of age. The majority of cases in children are idiopathic. Pathologic lead points can be identified in only 25 percent of cases. Here. we present a case of 15 months female child with Ileoileal postoperative intussusception with an anastomotic margin as a lead point, following resection anastomosis done for patent vitello intestinal duct. Role of high clinical suspicion, investigations and judgement are highlighted in managing the case. Keywords: Anastomosis; ileoileal; postoperative intussusception; patent vitello-intestinal duct.

Contrast fistulogram (FG) and distal pressure colostogram (DPCG) are standard diagnostic methods for the assessment of anorectal malformations. Pelvic magnetic resonance imaging (MRI) earned a place among essential diagnostic methods in preoperative investigations after the Currarino syndrome and a high incidence of associated spinal dysraphism were described. The aim of our study was to evaluate the possibility of substituting FG and DPCG by a modified pelvic MRI, e.g. MRI fistulogram (MRI-FG) and MRI colostogram (MRI-DPCG). The prospective study involved 29 patients with anorectal malformations who underwent a modified pelvic MRI. The length and course of fistulas and rectum, and the presence of sacral anomalies were studied on MRI images and compared with images obtained by radiologic examinations. Modified MRI brought identical results as contrast studies in 25 patients when related to the fistula and rectum length and course. MRI was more accurate for the detection of sacral anomalies. MRI-FG was the only imaging method used in the four most recent patients. The results support the assumption that conventional contrast examinations for the assessment of anorectal malformations can be replaced by MRI, thus reducing the radiation dose., (© 2013.)

A 3-day-old male baby presented with clinico-radiological features of rectal atresia with colo-urinary fistula. There was no radiological evidence of congenital pouch colon (CPC), but per-operatively CPC of unusual anatomy was found. The CPC was intermediate between type I and type II of Narsimha's classification. The pouch had rudimentary appendix and proximal colovesical fistula. The distal end of CPC was free. In the first stage, diverting ileostomy was done. In follow-up, after 1 month because of rectourinary fistula baby developed hyperchloremic metabolic acidosis and uremic sepsis. First metabolic correction and control of sepsis were done. Thereafter, abdomino-posterior saggital approach was used for excision of colovesical fistula, appendectomy, coloplasty and colorectal anastmosis. The ileostomy was left undisturbed. The infant at present is waiting for the third stage ileostomy closure.

Patent Vitello-intestinal Duct (VID) results as of failed obliteration of the fetal omphalocele coelom (herniated loops of intestine in the umbilical cord) during the development of the midgut. We report a case of an infant who presented at 11 months of the age with history of persistent umbilical discharge since birth. The VID was confirmed with a fistulogram using gastrograffin contrast studies and a wedge resection with primary anastomosis. The infant was discharged 5 days post-op without any post-operative complications. This case report highlight a rare cause of umbilical discharge and the surgical intervention required.

Background: Management guidelines with regard to congenital pouch colon (CPC) are not clearly defined with regard to the type of pouch present. The aim of this study was to outline the management strategy and surgical approach to CPC using the Saxena-Mathur classification based on anatomical morphology of the pouch., Methods: During a 12-year period (1995-2007), 426 patients were surgically managed for anorectal malformations at the RNT Medical College, Udaipur. Congenital pouch colon was documented in 80 patients and categorized into 5 types according the anatomical morphology. The management strategy depended upon the location of the pouch and its condition at the time of the surgery., Results: In type 1 and type 2 CPC, a 1-stage (pouch excision and pull-through) or 3-stage procedure (ileostomy, pouch-coloplasty with pull-through, and ileostomy closure) was performed depending on the condition of the pouch (ischemic or healthy). In type 3 and type 4 CPC, a 3-stage procedure (pouch excision with colostomy, pull-through, and colostomy closure) was performed in all patients. In type 5 CPC, a 3-stage procedure (distal pouch excision with proximal pouch-coloplasty with ileostomy, pull-through, and colostomy closure) was successful., Conclusion: Management of CPC patients according the Saxena-Mathur classification provides a well-defined algorithm in the surgical approach according to the anatomical morphology of the pouch.

Anorectal malformations are common worldwide. Several classifications were used to describe the different types, but in spite of this, there are rare variants. This report describes 2 unusual cases of congenital pouch colon and anorectal malformations.

Purpose: The aim of this study was to perform a detailed histopathologic examination of the terminal colonic pouch and the colovesical fistula (CVF) excised during surgical management of male patients with the more severe forms (types I/II) of congenital pouch colon (CPC) associated with anorectal agenesis., Methods: From January 2005 to December 2006, 25 male patients with types I/II CPC underwent abdominal exploration with dissection of the terminal portion of the colonic pouch and associated CVF till the bladder, division-ligation of the fistula, and excision of the colonic pouch. In 6 of the 25 patients, a complete dissection of the fistula to the bladder was possible, and in them, the terminal portion of the colonic pouch and the CVF were subjected to detailed histopathologic examination. The 6 patients included 3 newborns in whom this surgery was performed as a primary procedure, and 3 patients aged 3 months, 15 months, and 2 years, respectively, in whom a window colostomy of the pouch had earlier been performed. After due processing, multiple sections from the specimens were stained using the routine H&E method and examined under the microscope under different magnifications., Results: In 4 specimens, the epithelial lining of the CVF consisted of transitional stratified epithelium with underlying anal/urethral glands. In 2 specimens, obtained from patients 15 months and 2 years old, respectively, the lining was of nonkeratinizing, stratified squamous epithelium. Other findings included aganglionosis in the muscle layers (n = 2), submucosal and subserosal fibrosis (n = 1), and thickening of muscle layers in the fistulous portion, suggestive of the presence of an internal sphincter (n = 2)., Conclusions: The CVF in patients with types I/II CPC shows histologic features of the normal anorectal canal.

The presence of Meckel's diverticulum in the exomphalos sac is a well known entity but the intrauterine perforation of the diverticulum is very rare. We report a case of intrauterine fistulation of Meckel's diverticulum to the surface of the intact sac of an exomphalos minor.

A male neonate had fecal loss from a patent omphalomesenteric duct.

Minor degrees of exomphalos have been known to be associated with vitellointestinal duct anomalies. The most frequent association is Meckel's diverticulum. It is unusual for a fistula to be communicating directly with the exomphalos sac. We report five neonates that presented with a fistula on the exomphalos sac over a 10-year period. The different variants of patent vitellointestinal duct in these cases have been discussed with a review of the literature.

Spilt notochord syndrome is an extremely rare form of spinal dysraphism characterized by a complete cleft of the spine and a persistent communication between endoderm and ectoderm. A variant of split notochord syndrome was diagnosed in a 25-week-old fetus showing a prolapsed congenital colostomy and a spinal cystic lesion. The final diagnosis included protruding colon segment, imperforate anus with a rectourethral fistula and lipomyelomeningocele. The ultrasound features of the condition and the post-natal management are discussed. The neonate was successfully treated with a posterior sagittal anorectoplasty, while the lipomyelomeningocele was resected at a later stage., (Copyright (c) 2005 John Wiley & Sons, Ltd.)

A patch graft of the pouch colon over the pulled-through ileum in a patient with long-length pouch colon (in whom the entire colon was replaced by a pouch that communicated with the urinary bladder) was tried with good results for the first time. At follow-up the patient was found to have normal bowel patterns and weight gain. Though colon patch grafts have been widely used in patients with total colonic aganglionosis, they have not been tried for the short colon. The authors discuss the clinical setting in which the procedure may prove useful in patients with pouch colon and why these patch grafts may yield comparable or even better results in patients with pouch colon compared with those with total colonic aganglionosis.

Introduction: Posterior sagittal anorectoplasty (PSARP) was introduced in 1982, by Pena and de Vries, as a new surgical procedure for patients with anorectal malformations. It was supposed to provide better chance for normal fecal continence., Material and Methods: Between 1991 and 2000, 50 patients with anorectal abnormalities underwent PSARP. In 43 patients PSARP was primary operation and in 7 it was a secondary procedure. At the time of study patients were not younger than 3, and not older than 13 years. Patients and their parents were interviewed, and fecal continence was graded as follows: voluntary bowel contractions, soiling less than once a week, soiling more than once a week, daily soiling and constipation., Results: Amongst patients who underwent primary surgical correction, 74% had voluntary bowel contractions. Babies with perineal fistula, rectal atresia and stenosis presented with best results in term of voluntary bowel contractions (100%), as with vestibular fistula, ten of eleven patients. Only 18% of patients (atresia without fistula, vestibular fistula and bulbourethral fistula) had soiling. Patients with perineal fistula and rectal atresia and stenosis 40% were totally continent (voluntary bowel movements without soiling). The most frequent sequel was constipation, which appeared in 48%, without coincidence with frequency of soiling. The problem of constipation was surprisingly more frequent in patients with expected better prognosis in fecal continence., Conclusion: Although PSARP offers a good esthetic result, only two third of patients have voluntary bowel movements and in about half there exists a problem with constipation, with necessary further treatment.

The authors describe a new case of the split notochord syndrome (SNS) with dorsal enteric fistula in an eight-month-old-boy. The child had also central nervous system anomalies including dysgenetic corpus callosum, absence of septum pellucidum, triventricular hydrocephalus, small posterior fossa, and lipomyelomeningocele, gastrointestinal system anomalies such as malrotation, wandering spleen, and right inguinal hernia. The clinical features, embryogenesis, and literature were reviewed briefly.

Materials and Methods: An infant was born with a congenital colocutaneous fistula to the right of the base of the umbilicus, along with distal small bowel atresia., Results: These findings produced a unique presentation of a prenatally closed gastroschisis with absorption of the extruded intestine., Conclusion: This child, like all five previously reported infants with prenatally closed gastroschisis, died from complications of short-gut syndrome.

A male infant was referred to our department because of lumbosacral meningomyelocele, dorsal enteric fistula and imperforate anus. The mother had received a parenteral drug containing estradiol benzoate and progesterone for inducing abortion in the first trimester. She also used an anal pomade containing triamcinolone and lidocaine-HCl during the pregnancy for hemorrhoids. Sigmoid end colostomy was performed after meningomyelocele repair. On abdominal exploration a wandering spleen was detected but no other anomalies. Two months later, an abdominoperineal pullthrough was performed, and the patient was discharged well after three weeks. Our case is the sixth that had split notochord syndrome associated with dorsal enteric fistula and imperforate anus. Additionally, penoscrotal transposition and wandering spleen were present in this case. To our knowledge, these associated anomalies have been extremely rare.

Umbilical anomalies arise from fetal structures such as the omphalomesenteric duct (OMD) or urachus or from failure of closure of the umbilical fascial ring. Persistence of the OMD may lead to several anomalies including umbilical sinus, umbilical cyst, Meckel's diverticulum, or patent OMD (POMD). A POMD is usually associated with the ileum, but rarely may be with the caecum or appendix. We describe a POMD of the vermiform appendix and discuss the possible pathogenesis and management.

Seventy-two patients with complete short colon (CSC) were treated at the authors' institution between 1971 and 1994. Initially, one-stage coloplasty with abdominoperineal pull-through was performed in 10 neonates, with a 50% survival rate. The subsequent 62 patients had staged management in which a window colostomy was performed at the time of presentation. Of the 50 survivors in this group, 20 had coloplasty with a abdominoperineal or sacroabdominoperineal pull-through by June 1994. A protective ileostomy was done at the time of coloplasty in 10 patients, and was closed 6 to 12 weeks later. Two of the 20 patients who had staged reconstruction died because of complications; the others recovered well. The overall survival rate after coloplasty was 76.7%. Eighteen of the 23 survivors have had follow-up for up to 8 years. They have shown normal growth and good continence and have been free of significant long-term complications. The other 30 patients are awaiting definitive surgery. A staged management plan is advocated for complete short colon because it provides consistent results and good quality of life.

The umbilicus is the site of a number of well-recognized and unusual congenital anomalies. The authors report two rare anomalies of the umbilicus, one involving the appendiceal artery and the other the appendix vermiformis.

The congenital uro-enteral fistula are observed essentially in boy and quite exclusively with anorectal malformation. Cloacal anomaly in girl and cloacal exstrophy in both sexes are so exceptional than complex malformations.

The case history of a child passing a roundworm through the umbilicus is presented. The diagnosis and management of a patent vitello-intestinal duct is discussed.

Exomphalos and vitellointestinal duct remnants are well-known entities. This report details the presentation of congenital fistulation of a Meckel's diverticulum to the surface of an exomphalos minor.

Split notochord syndrome with dorsal enteric fistula is an extremely rare congenital anomaly that may be associated with meningomyelocele or meningocele, and genitourinary anomalies. This case presented with an additional finding of bladder exstrophy, raising the possibility of a relationship between this syndrome and the OEIS complex.

Extrophic lesions of the ventral abdominal wall are represented by a range of anomalies of which the most severe is cloacal extrophy. By 1986, only 190 cases were reported in the world literature and it is estimated that only 15 cases are seen in the United States of America per year. In Nigeria today, with improvement in antenatal and perinatal services, children who one or two decades ago would have been stillborns or products of miscarriages are now born alive. This paper reports a case of cloacal extrophy in a Nigerian neonate and reviews the current management of the condition.

The analysis of 21 cases of congenital intestinal umbilical fistulas in children was carried out. It was established that in half of the patients the intestinal umbilical fistulas were associated with other developmental defects. The clinical picture, diagnosis, complications of the persistent umbilical duct and curative measures in this developmental defect are described. The continuous persistence of the congenital umbilical intestinal fistulas can result in disturbed physical development of children and the appearance of adhesive processes in the abdominal cavity. The treatment of patients with congenital intestinal fistulas should be started on diagnosing the disease, the operation of choice being the intra-abdominal closure of the fistula.