The split notochord syndrome with dorsal enteric fistula.

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.

[Congenital uro-intestinal fistulas]. / Les fistules uro-digestives congénitales.

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.

Completely separated scrotum and vesicointestinal fistula without exstrophy as a novel manifestation of aphallia: a case report.

A male infant born at 32 weeks of gestation and weighing 1.44 kg had penile agenesis, bifid scrotums, an anorectal anomaly, and suspected omphalocele. An operation revealed complete urethral agenesis, duplicated and blind bowels, a vesicointestinal fistula without exstrophy, and a urachal fistula; however, no omphaloceles were detected. To our knowledge, this is the first report on aphallia accompanied with completely separated scrotum and a vesicointestinal fistula without exstrophy; this condition is associated with urorectal septum malformation sequence (URSMS) and covered cloacal exstrophy. The possible embryogenesis is discussed in this study.

Three cases of spontaneous duodeno-colic fistula.

Three cases of duodeno-colic fistula are presented, where a communication was demonstrated between the anterior aspect of the third part of the duodenum and the transverse colon at the point of mesocolic attachment. At operation the fistulae were found to be some 6 to 9 cm in length, and histological examination showed a well marked muscular layer with an intact epithelium. In view of the absence of any explanatory pathology, an embryological basis is suggested as the likeliest explanation for their origin.

Umbilical polyps.

We report three patients, ages 5 years, 3 years, and 4 days, with umbilical polyps. In the third child the polyp was associated with an umbilical enteric fistula. An umbilical polyp is the result of incomplete closure of the omphalomesenteric duct and becomes apparent after the umbilical cord is detached. It is a reddish tumor of a few millimeters; it seldom bleeds or is exudative. We consider it important to study every case in detail in order to exclude possible underlying embryologic anomalies such as Meckel's diverticulum and umbilical enteric fistula.

Split notochord syndrome with dorsal enteric fistula and sacral agenesis.

Split notochord syndrome of the lumbosacral spine in association with dorsal enteric fistula is a rare phenomenon. To date, only nine human cases have been reported in the English literature. We present another case of this type, with sacral agenesis as an additional and unique finding. Several etiological theories are discussed including the persistence of the neurenteric canal, the occurrence of an ectopic or accessory neurenteric canal, a division or local redundancy of the notochord, an entodermal-ectodermal adhesion, neural tube rupture caused by oversecretion of fluid, and failure or aberrancy of dorsal aortic distribution to the region of the neural folds resulting in prevention of timely neural tube closure.