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Congenital colonic atresia: should primary anastomosis always be the goal?

We report 4 cases of the rare condition, congenital colonic atresia, presenting over 9 years. Two patients had dilated loops of bowel noted on routine ante-natal ultrasound. Three had primary anastomosis for lesions in transverse or distal descending colon and one had a staged procedure with colostomy formation. All had type III atresia. One patient had an early leak following primary colo-colic anastomosis for atresia extending from the hepatic flexure to the sigmoid colon. If it is desired to preserve the proximal colon in a right sided lesion with significant loss of colonic length then primary anastomosis may not be safe and we would advocate a staged procedure. Otherwise we would support current moves towards primary anastomosis in this condition. We also urge early investigation in all patients who exhibit intestinal dilatation on antenatal ultrasound.

Anastomosis, Surgical↗

Preampullary congenital duodenal obstruction in a father and son.

This is the report of an infant seen on June 25, 1960 with preampullary total duodenal obstruction caused by a combined annular pancreas and duodenal stenosis above the ampulla. A gastroduodenostomy was performed. In December 1989, he fathered a 1,700-g boy with preampullary duodenal atresia. A duodeno-duodenostomy was performed successfully by the same pediatric surgeon. Second-generation duodenal obstruction is rare; to our knowledge, there are no other cases.

Adult↗

Anomalous biliary ducts associated with duodenal atresia.

Duodenal atresia is rarely associated with anomalous biliary ducts that permit communication between the proximal and distal duodenal segments. Two such cases are presented herein and the literature is reviewed. Although the typical radiographic pattern of duodenal atresia is the "double bubble" sign with absence of distal bowel gas, air may be present in the distal bowel when anomalous bile ducts provide a conduit around the atretic segment. Contrast studies are generally not performed in the typical clinical and radiographic evaluation of duodenal atresia; however, an upper gastrointestinal series may be useful in defining the more complex anomaly. Clinical presentation may occur relatively late if the biliary communication is large enough to permit passage of some milk or formula. Care should be taken at surgery to avoid obstruction or injury to the anomalous bile ducts, and operative cholangiography may be useful to document continued bile duct patency following repair of the atresia. Theories of the etiology of this anomaly relate to interference with recanalization of the duodenum by the process of hepaticopancreatic duct formation and persistence of the primitive "dual duct" stage.

Bile Ducts↗

Immune deficiency in familial duodenal atresia.

The familial occurrence of duodenal atresia is uncommon. This study evaluated the inheritance patterns, the nature and associations, and the presence of immunologic deficits in duodenal atresia recurring in at least three siblings each in two nonrelated families. In the first family, an association with Fanconi's anemia was observed in three of seven pregnancies (2 boys, 1 girl) suggesting an autosomal recessive mode of transmission. Patients died as a result of overwhelming (fungal) septicemia in association with pancytopenia. In a second family, identical multiple atresias occurred in two female siblings born 18 months apart and a third child with a duodenal stenosis. Overwhelming sepsis and a T-cell dysfunction was seen in the postoperative period, which had partially corrected by follow-up at 5 months. A history of family occurrence of duodenal atresia should alert the physician to the possibility of associated pathology including immune deficiency states.

Abnormalities, Multiple↗

Ring chromosome 4 in a child with duodenal atresia.

We report on a 19-month-old girl with ring chromosome 4 and a multiple congenital anomaly syndrome. The clinical and cytogenetic findings are compared with those of previous cases in whom the breakpoints in ring chromosome 4 are known.

Abnormalities, Multiple↗

Familial duodenal atresia: a report of two families and review.

We report on 2 families with recurrence of uncomplicated duodenal atresia. The presence of consanguinity in one family, the frequent occurrence of consanguinity in parents of affected children previously reported, and the equal sex ratio suggest autosomal recessive inheritance.

Consanguinity↗

Jejunoileal atresia: a proposed classification.

An experience with 59 infants with jejunal and ileal atresia is presented along with a proposed classification based on a combination of morphology and clinical characteristics. It would appear that the characteristics of each type are sufficiently distinct as to render this worthwhile classification.

Age Factors↗

Multiple gastrointestinal atresias in two consecutive siblings.

Two consecutive female siblings with multiple gastrointestinal atresias are described. The history of consanguinity in the parents and the presence of extensive typical pathological lesions suggest a genetically-induced developmental fault in the alimentary tract during the early embryonic period.

Consanguinity↗

Minimally invasive surgery in the neonate: review of current evidence.

Minimally invasive surgery has been one of the most important surgical advances in the last 15 years. The development of smaller instruments has allowed pediatric surgeons to apply this rapidly evolving technology to neonates. Congenital neonatal deformities including tracheoesophageal fistula, patent ductus arteriosus, duodenal atresia and anorectal malformations are now being managed with minimally invasive surgery. This article summarizes the status of these techniques in neonates.

Bronchopulmonary Sequestration↗

Microgastria--case report and a review of the literature.

The case of a female baby with microgastria, duodenal atresia, asplenia, hydrocephalus and many other anomalies is reported. She underwent repair of the duodenal atresia at the age of 3 days and died of sepsis at the age of 5 months. Twenty-nine cases of microgastria found reported in the literature are reviewed.

Abnormalities, Multiple↗

Situs inversus abdominus in association with congenital duodenal obstruction: a report of two cases and review of the literature.

This report describes two newborns with persistent bile-stained vomiting. Their radiological investigations revealed the existence of situs inversus and duodenal obstruction. In one, the duodenal obstruction was partial, secondary to a duodenal diaphragm with a central aperture, whereas the other child had complete duodenal atresia as well as Fallot's tetralogy. Such an association is extremely rare, with only 18 cases reported so far in the literature. Embryological aspects, investigations, and treatment are also discussed.

Duodenal Obstruction↗

[Aglossia-adactylia with jejunal atresia].

Case report of a newborn with the aglossy-adactyly syndrome associated with complete jejunal atresia. Review of the literature does not allow any conclusion concerning the etiology of the observed malformations. The jejunal atresia, present in this case is probably coincidental. Attention is drawn on the possibility of prenatal diagnosis of obstruction of the digestive tract by ultrasound whenever pregnancy is complicated by hydramnios.

Abnormalities, Multiple↗

Duodenal web with preduodenal portal vein.

This article described an unusual case of an infant with duodenal atresia and preduodenal portal vein without Down's syndrome or other anatomical anomalies associated with this condition. Duodenoduodenostomy was effective. Enteral feeding was re-established 72 hours post-operatively and the patient was discharged home one day later.

Appendectomy↗