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A portoenterostomy (PE) procedure for extrahepatic biliary atresia (EHBA) is sometimes performed with a stoma in an attempt to reduce the incidence of acute cholangitis. The purpose of this study was to determine if the presence of a stoma increased the complication rate of patients undergoing orthotopic liver transplantation (OLT) for EHBA. The medical records of 42 consecutive patients with EHBA who underwent primary OLT between October 1988 and October 1991 were retrospectively reviewed. Three patients were excluded, since their grafts were lost within 3 days of OLT. The remaining 39 patients were divided into three groups: no PE (n = 7), PE without stoma (n = 23), and PE with stoma (n = 9). The mean age of the whole group was 19.62 +/- 24.37 months, with a range of 5 to 132 months. Mean weight was 9.62 kg, with a range of 4.2 to 41 kg. Survival at 3 and 12 months as well as number of retransplantations were similar among the three groups. However, at the time of OLT increased morbidity was observed, consisting of increased operative time and number of reoperations, whether or not the stoma had been closed prior to OLT.

This article describes the MR appearances of the six most common congenital anomalies of the inferior vena cava. As a basis for understanding those anomalies, it describes the embryology of the inferior vena cava, based on an actual study of embryos and fetuses. The article takes a fresh look at the original research in this area, discusses the possible embryogenesis of the relevant anomalies, and describes different opinions on that subject, where different opinions exist.

Omphalocele and gastroschisis appear, by virtue of their differences in postnatal findings and embryological development, to be different entities. An hypothesis concerning the genesis of gastroschisis is presented which is consistent with observed embryologic sequential change and known teratogenic possibilities. The apparent increase in number of cases of gastroschisis and the frequency of omphalocele warrant further experimental study of their pathogenesis, preferably in primates.

From October 1980 to November 1981, 34 patients with anorectal anomalies have been operated upon by a sagittal midline approach. The skin incision extends from the sacrum to the perineum (ventral aspect of the anal dimple). The superficial and deep layers of the external sphincter are identified by electrostimulation and split, with the coccyx, in the midline. Ileo- and pubococcygeal portions of the levator dorsally and then the striated muscle complex of the external sphinctor, pubococcygeus and the presumed puborectalis are split ventrally to the urethra. In no cases has the ventral portion of the levators been separated from the thick ventral portion of the external sphincter, hence the term "striated muscle complex." When the terminal bowel is dilated (congenitally ectatic), the bowel is tailored prior to reconstruction of the sphinctors. The posterior sagittal approach provides an excellent exposure for evaluation and mobilization of the terminal bowel. It enables one to construct an anal canal, suture the bowel wall to the striated musculature and the mucosa to the skin, thereby reducing or avoiding the complications of prolapse and stenosis. In males with ectasia and a rectourethral fistula, transrectal closure of the mucosa at the fistula site, leaving the rectal longitudinal smooth muscle insertions on the prostatic capsule, avoids damage to the nerves and genital structures.

Posterior sagittal anorectoplasty (PSARP) is a new technique for the repair of high anorectal malformations. It is based upon complete exposure of the anorectal region by means of a median sagittal incision that runs from the sacrum to the anal dimple, cutting through all muscle structures behind the rectum. It was learned through this procedure that the external sphincter is a functionally useful prominent structure. No puborectalis sling, as such, could be identified. It was possible, however, to recognize a muscle continuity from the skin to the sacral insertion of the levator ani. Since it is impossible to pull the generally ectatic rectum through without destroying the muscle structures present, the rectum must be tapered to allow suturing the muscle behind it. In all these anomalies, the rectum and urethra (or vagina) are very closely joined, sharing a common wall, and their separation calls for extensive exposure. A number of technical details clarified in the course of applying the procedure in 54 patients are fully discussed. This approach also proved to be very successful in the management of two patients with rectocloacal fistula, two with rectal atresia and two with stenosis. Colostomy has been closed in 27 patients and fecal continence may be described as excellent, except in those patients with severe sacral anomalies, and unquestionably superior to that obtained by us with other techniques.

This article discusses the disparate views regarding sphincteric anatomy, both normal and abnormal, and describes preoperative procedures for and operative approaches to resolving anorectal anomalies.

PRIMARY OR SPONTANEOUS PERITONITIS is a we/lknown complication of the nephrotic syndrome? Peritonitis secondary to rupture of the small intestine in a child with nephrotic syndrome has not been reported; our experience with such a patient prompted this report.

In over 90 per cent of cases, hepatitis can be differentiated from extrahepatic obstruction by needle biopsy with experienced histologic examination. Extrahepatic obstruction should be corrected as soon as diagnosed to prevent progressive cirrhosis and liver failure. Hepatic porto-enterostomy appears to offer reasonable life, in terms of both “well being” and duration, to only about 30 per cent of infants with neonatal biliary obstruction, but it is the best approach currently available for selected cases.

An anomaly analogous to the congenital “H-type” tracheo-esophageal fistula may occur at the distal end of the gastrointestinal tract. This consists of a congenital ano-urethral fistula without anal atresia, here designated “H-type” ano-urethral fistula. Three boys with this condition are reported. All three also had anterior urethral strictures and other congenital anomalies. The authors present their views of the embryologic development of this anomaly.

Previously, patients commonly had fecal incontinence following repair of intermediate & high imperforate anus. Using a posterior sagittal approach, deVries & Pena have modified previous techniques by pulling the bowel within the puborectalis & external sphincter under direct vision so as to preserve these structures.