Hirschsprung disease (HSCR) is a genetic disorder with an incidence of 1:5000, seen in the pediatric age group. The association between HSCR and neuroblastoma (NBL), ends of the neurocristopathy spectrum is rare. Less than 10 cases of this association are reported in the literature and the association between the Phox gene and Sox10 gene in the pathophysiology of these is being studied. We report a one-year-old baby, who presented to us, with chronic constipation on regular enemas and laxative usage. There was a history of delayed passage of meconium. At the time of Duhamel’s pull through a well-defined, bilobed hard presacral mass, was encountered. Excision and coccygectomy were done and the pull was completed. The histopathology showed a well-differentiated NBL. Fludeoxyglucose positron emission tomography scan and the N-Myc amplification were negative and the patient was managed with expectant treatment. She is doing well over a 3-year follow-up with no recurrence and good resolution of bowel functions.

Alimentary tract duplication cysts are rare congenital anomalies, most commonly located in the ileum, but may present anywhere from mouth to anus.Clinically, they may be asymptomatic, incidentally diagnosed or may present with obstruction, volvulus, intussusception or gastrointestinal bleed. Here we report a case of a one year old male child presenting in gasping state and shock. Despite the initial strong suspicion of Meckel's diverticulum and tubercular abdomen, the final diagnosis remained elusive till exploratory laparotomy was performed which revealed a duplication cyst of ileum with perforation into the umbilicus. Duplication cyst should always be kept as a differential diagnosis so that early intervention can help in better management.
Abdomen ; Ascites ; Child ; Diagnosis ; Diagnosis, Differential ; Early Intervention (Education) ; Humans ; Ileum ; Intestinal Volvulus ; Intussusception ; Laparotomy ; Male ; Meckel Diverticulum ; Mouth ; Shock ; Umbilicus*

Hernia of the umbilical cord associated with segmental dilatation of the ileum (SDI) is a rare entity with only 17 cases reported in literature. We hypothesize that the prominent mesenteric vessels or folds that splay out towards the ends of the SDI signify the presence of an antecedent duplication cyst which eventually got resorbed to form the dilated segment.
Dilatation* ; Hernia* ; Ileum* ; Umbilical Cord*

Although Meckel's diverticulum is the most common vitellointestinal duct (VID) anomaly, patent vitellointestinal duct (PVID) is the most common symptomatic embryological defect. Patient may present with the anomaly itself or due to complications like intestinal obstruction secondary to volvulus, intussusception or adhesions. Prolapse occurs if the diverticulum is wide-mouthed enough to allow bowel to come out or due to increased intra-abdominal pressure like cry or cough. Bowel prolapse through PVID is rare and double prolapse of proximal as well as distal loop in a newborn is extremely rare. Omphalocele with prolapsing bowel through PVID as found in our index case is even rarer in literature. The pediatric surgeon should be familiar with these varied manifestations in the newborn because the prolapsed bowel can progress to gangrene and complications if not identified and operated upon early.
Cough ; Diverticulum ; Gangrene ; Hernia, Umbilical* ; Humans ; Ileum* ; Infant, Newborn ; Intestinal Obstruction ; Intestinal Volvulus ; Intussusception ; Meckel Diverticulum ; Prolapse* ; Vitelline Duct

A case of congenital ectopic scrotum in neonatal period is described. The ectopic scrotum was located in the right inguinal area and the left hemiscrotum was found in normal location and each hemi-scrotum contained their testis. The neonate also had imperforate anus as low anorectal malformation with spinal abnormalities (hemi-sacrum and hemi-pelvis), right knee flexion contracture and right club foot. The embryological explanation in the literature of ectopic scrotum and its associated anomalies is discussed.
Anus, Imperforate ; Contracture ; Extremities* ; Foot ; Humans ; Infant, Newborn ; Knee ; Scrotum* ; Testis