Duodenal motility after tapering duodenoplasty for high jejunal and multiple intestinal atresia

Duodenal motility after tapering duodenoplasty was investogated by radiography and video-fluoroscopy. Two patients underwent tapering duodenoplasty, one at the time of duodenojejunostomy for double high jejunal atresias and another 6.5 months after the restoration of intestinal continuity for multiple intestinal atresias. Both patients showed a satisfactory postoperative course and were doing well. Radiographic and video-fluoroscopic studies demonstrated that the tapered portion of the duodenum had restored active peristalsis and excellent passage of contrast material, while the proximal, non-tapered portion remained dilated and dismotile soon after the operation. Tapering duodenoplasty proved to be a valuable procedure for restoring duodenal motility. Correspondence to: M. Honzumi     

Operative management of duodenal atresia

The management of duodenal atresia (DA) in two Scandinavian pediatric centers is reported. A total of 67 infants with DA were retrospectively analyzed. A high incidence of associated anomalies was present, including Down's syndrome in 40% and cardiac anomalies in 20% of the infants. No immediate postoperative mortality was noted. The operative procedures were duodenoduodenostomy and duodenojejunostomy. Follow-up did not disclose any frequent gastrointestinal disturbances or differences in postoperative complications when the two procedures were compared. The prognosis for these patients is thus determined exclusively by the presence of associated anomalies, especially Down's syndrome and cardiac malformations.     

The management of combined oesophageal and duodenal atresia

Duodenal atresia (DA) in babies with oesophageal atresia (OA) is associated with significant morbidity and mortality. The management protocol for this combination of anomalies is not well defined and evolving. The aim of this study was to review our experience with combined OA and DA and to note the evolving trend in management at Sydney Childrens Hospital. Over the last 30 years, 225 babies with OA have been treated at our institution. Ten babies had associated DA. A total of 19 anomalies were noted; one child had multiple lethal anomalies and received no treatment. In three babies the diagnosis of associated DA was missed initially. For the nine babies who were treated, four had a primary OA repair followed by a delayed DA repair; one had cervical oesophagostomy, gastrostomy and DA repair; and two had a simultaneous repair of OA and DA with a gastrostomy. The last two patients in this series have had a primary simultaneous repair of both anomalies without a gastrostomy. The complications noted in the nine patients treated included anastomotic strictures in five, recurrent tracheo-oesophageal fistula in one, food bolus obstruction in one, and a megaduodenum requiring tapering in one. We believe that adopting a management protocol of primary simultaneous repair of both anomalies without a gastrostomy is justified. There was no apparent increase in morbidity and mortality with such an approach.     

Is malrotation significant in the pathogenesis of duodenal atresia?

The association between duodenal atresia and intestinal malrotation has been recognised for many years. We discuss the hypothesis that embryonic bands crossing the developing duodenum can cause enough compression to create an atresia or stenosis. We support this hypothesis with the operative findings of all the duodenal atresias treated at our institution over a 15-year-period.     

Duodenal duplication cyst associated with duodenal atresia

A 2-day-old premature infant with a duodenal duplication cyst situated between the two blind ends of a duodenal atresia is presented. This entity has not been reported previously in the literature. This rare association of a duplication cyst and atresia may occur when recanalization is interrupted, with failure to form a single duodenal lumen.     

A new variant of esophageal atresia with tracheoesophageal fistula and duodenal atresia: a diagnostic dilemma

A new variant of esophageal atresia (EA) with tracheoesophageal fistula (TEF) associated with duodenal atresia is reported. The TEF was between the lower pouch and the trachea, with a cystic dilatation in the midportion. The tracheal end of the fistula was obstructed by a membranous septum at both ends of a cystic dilatation, leading to a diagnosis of pure EA (gasless abdomen). After the lower pouch was opened beyond the cystic dilatation, 100 ml nonbilious fluid was obtained. A laparotomy revealed a type III atresia of the first part of the duodenum.     

Multiple organ malrotation syndrome with duodenal atresia: a case report

Duodenal atresia (DA) can be associated with multiple anomalies. Multiple organ malrotation syndrome (MOMS) involves laevoversion of the liver and gall bladder and dextroversion of the stomach and spleen. We report a case of MOMS with DA. Embryologic aspects, investigation, and treatment are discussed. Accepted: 30 October 1997     

Duodenal atresia

The aim of this study was the assessment of mortality, morbidity, and associated anomalies in a series of 44 patients with congenital intrinsic duodenal obstruction admitted to the Amsterdam Pediatric Surgical Center between 1975 and 1989. Twenty-two patients were girls and 22 were boys. Severe associated anomalies were considered a contraindication for surgery in 8 children. Of the 36 who were operated upon, 8 died within the first year and a half of life. Associated anomalies consisted of trisomy 21 in 11 patients, cardiac defects in 14 (8 with trisomy 21), skeletal defects in 4, esophageal atresia in 3 (in 2 combined with imperforate anus), and various other anomalies in 5. Among the 6 patients with trisomy 21 who were operated upon, 3 died within 18 months after surgery. The surviving 3 in this group are all below the normal average weight for age. All patients without associated anomalies did well postoperatively. One patient developed a bowel obstruction at age 2 years. It appears from this study that the eventual outcome in children with duodenal atresia is determined almost exclusively by the presence or absence of associated anomalies. Correspondence to: A. Vos     

Late presentation of a duodenal web in a patient with situs inversus and apple peel jejunal atresia

A 16-year-old girl presented with signs of proximal intestinal obstruction. In the neonatal period, surgical correction of an apple peel atresia had been performed, and she also had a situs inversus abdominalis. Revision of the anastomosis had been done when she was 3 years old. Contrast studies apparently again showed a stricture of the anastomosis, which was treated by stricturoplasty. Because of persistent obstruction, reexploration was done and revealed a duodenal membrane. Anastomotic strictures are very rare several years after the primary operation, so other causes of obstruction should be sought.     

Choledochal cyst and duodenal atresia: a rare combination of malformations

A rare case of congenital duodenal atresia (DA) associated with a choledochal cyst (CC) is reported. At 38 weeks of gestation, a 1,610-g girl was born by cesarean section with a prenatal diagnosis of congenital DA. After the disease was confirmed by radiographs, she underwent a duodenoduodenostomy for complete separation of the duodenum with an annular pancreas. Thirty-two months after the initial operation, she developed upper abdominal pain and acholic stools. Abdominal ultrasonography demonstrated a CC and dilated intrahepatic bile ducts. Magnetic resonane cholangiopancreatography showed an anomalous arrangement of the choledochus and the main pancreatic duct. A diffusely dilated extrahepatic bile duct was resected, and a hepaticoduodenostomy was performed after a cholecystectomy. The patient was discharged without complications. We could not find a similar case report in the English literature. Although it is not reported that there is a close relation of DA and CC in embryologic development, the presence of this combination should be considered.     

The influence of trisomy 21 on the incidence and severity of congenital heart defects in patients with duodenal atresia

Duodenal atresia is associated with a wide variety of congenital malformations. Trisomy 21 occurs in approximately one-thirds of infants with duodenal atresia. Congenital heart disease in patients with trisomy 21 and duodenal atresia is well known. However, the frequency and spectrum of congenital heart defects in infants with duodenal atresia and a normal karyotype has not been outlined in the literature. Therefore, we conducted a retrospective chart review to clarify our knowledge about this population. Retrospective review of the medical record was performed on patients with duodenal atresia/stenosis from January 1995 to September 2007. Demographic data included birth weight and gestational age. Variables of interest included cardiac defects and karyotype. Surgical repair for duodenal and cardiac malformations were reviewed. Ninety-four patients with duodenal atresia/stenosis were identified. Average gestational age was 36 weeks and birth weight was 2,536 g. Trisomy 21 was identified in 39 (41%) patients. Overall, 37 patients (39.3%) had a congenital heart defect. Defects were identified in 24 (61.5%) patients with trisomy 21, when compared to 13 (23.6%) patients with a normal karyotype. Of the patients with congenital heart defects and trisomy 21, 11 (28.2%) required operative repair compared to the 6 (10.9%) patients with a defect and normal karyotype. Therefore, in patients with duodenal atresia, the presence of trisomy 21 carries a relative risk of 2.61 for congenital heart defects, and relative risk of 2.59 for open heart surgery. In patients with duodenal atresia, the presence of trisomy 21 carries a 2.5-fold increased risk of cardiac defect and the same increased risk for repairing a cardiac defect.     

Operative and postoperative management of congenital duodenal obstruction: a 10-year experience

Forty-five patients with congenital duodenal obstruction aged from 1 day to 11 months were operated upon during the last decade. Group A included 25 neonates with duodenal obstruction due to atresia type I or a complete diaphragm in 10 cases, atresia type II in 3, atresia type III in 1, stenosis or incomplete diaphragm in 4, annular pancreas in 6, and aberrant vessels in 1. Seven duodenoduodenostomies (D-D) were performed, 14 duodenoplasties (D-P) with occasional excision of the diaphragm, and 4 duodenojejunostomies (D-J). Tapering, plication of the proximal duodenum, or gastrostomy was not performed in any child and no transanastomotic tube was placed. Group B included 20 infants with duodenal obstruction due to peritoneal bands and associated malrotation. They all underwent Ladd's procedure. All children in group B and 15 in group A had an uneventful recovery and tolerated oral feedings within the first 10 days postoperatively without any complication. Six children in group A had prolonged postoperative ileus and were treated with total parenteral nutrition (TPN), gastric decompression, and radiologic evaluation of anastomotic patency. These 6 children eventually tolerated oral feeding between the 18th and 45th postoperative days. In 1 child a technical error was found that caused a prolonged ileus. Three children died within the 1st postoperative week. It is concluded that simple establishment of continuity of the gastrointestinal tract by performing the appropriate surgical procedure in combination with TPN and gastric decompression gives satisfactory results in the management of duodenal obstruction.Presented at the 15th Annual International Meeting arranged by the Greek Association of Pediatric Surgeons.     

Choledochal cyst and duodenal atresia: a rare combination

 A rare case of congenital duodenal atresia (DA) associated with a choledochal cyst (CC) is reported.At 38 weeks of gestation, a 1,610-g girl was born by cesarean section with a prenatal diagnosis of DA. After the disorder was confirmed by X-ray, she underwent a duodenoduodenostomy for a complete separation of the duodenum with an annular pancreas. Thirty-two months after the initial operation, she developed upper abdominal pain and acholic stools. Abdominal ultrasonography demonstrated a CC and dilatated intrahepatic bile ducts. Magnetic resonance cholangiopancreatography showed an anomalous arrangement of the choledochus and main pancreatic duct. A diffusely dilatated extrahepatic bile duct was resected, and a hepaticoduodenostomy was performed after cholecystectomy. The patient was discharged without complications. We could not find a similar case report in the English literature. Although it is not reported that there is a close relationship in embryologic development of DA and CC, one should be aware of the possibility of this combination. Accepted: 21 December 2000     

Primary anastomosis in esophageal atresia type I without a gap

This paper reports the case of an infant born with type I esophageal atresia (EA) associated with duodenal atresia (DA). The critical condition of the patient necessitated an exploratory laparotomy, which revealed severe dilatation of the stomach and duodenum. The routine procedure for repairing type I EA is a delayed primary anastomosis after 10 weeks of age because of the long gap between the two esophageal segments. In our case, due to the concomitant DA, the lower pouch was long enough to allow primary neonatal anastomosis. A radiograph taken with a Hegar dilator in the lower segment via the gastrostomy confirmed this suspicion, and the baby underwent a thoracotomy and primary anastomosis between the esophageal pouches. The authors propose the possibility of primary esophageal anastomosis in similar cases. Accepted: 6 August 1998     

Does gut atresia cause polyhydramnios?

Fetal gut atresia is variably associated with polyhydramnios. In order to determine which pregnancies will develop polyhydramnios, the case notes of 80 babies with gut atresia and stenosis were reviewed. Maternal polyhydramnios developed in all cases of pure oesophageal atresia (n=8), all cases of Type III duodenal atresia (DA) with a non-bifid bile duct (n=8), 80% of cases with type I DA (n=10), and 24% of atresias of the small intestine (n=34). Polyhydramnios did not develop in any case where there was not total obstruction except in 1 baby with DA and a bifid bile duct (BBD). These included stenosis of the oesophagus and duodenum (n=17) and DA type III with a BBD (n=3). These results support the role of fetal swallowing and fluid absorption by the fetal gastro-intestinal tract in the regulation of amniotic fluid volume. Accepted: 24 February 1997     

Pyloric atresia associated with duodenal and jejunal atresia and duplication

A case of congenital pyloric atresia associated with duodenal atresia, jejunal atresia, and a duplication is described. A review of the literature revealed nine previously reported cases of congenital pyloric atresia and multiple intestinal atresias, but this is the first report of such an association and a duplication. Accepted: 13 July 1998     

Transluminal endoscopic electrosurgical incision of fenestrated duodenal membranes

Duodenal fenestrated membranes are traditionally treated by side-to-side diamond-shaped duodenoduodenostomy, or duodenotomy and resection. We describe an alternative endoscopic approach for its resolution. A flexible panendoscopy reaching the duodenal membrane was performed. A balloon was inserted to dilate its orifice. Traction was applied to the balloon to differentiate the border of the membrane forming the duodenal wall. After visualizing the ampulla, the membrane was incised using a sphincterotome or needle knife on two sites opposite to the bile duct. From May 2001 to August 2007, ten patients with a fenestrated duodenal membrane underwent transluminal endoscopic electrosurgical incision (TEEI). Mean patient age was 3.4 years (range 1 month to 15 years). The endoscopic procedure lasted from 30 to 60 min. Oral intake began 24 h postsurgery in eight patients and at 48 h postsurgery in two patients. Hospital stay lasted for 2-5 days. After 1 year of follow-up, eight patients were asymptomatic and thriving at present, and one had a double membrane, required a second endoscopy with TEEI, and has experienced occasional vomiting. An additional asymptomatic patient was lost after 3 months of follow-up. TEEI of fenestrated duodenal membranes is a feasible and effective procedure in children.     

Additional congenital anomalies in babies with gut atresia or stenosis: when to investigate, and which investigation

A wide variety of additional congenital anomalies occur in babies born with a gut atresia or stenosis. The specific pattern of anomalies depends on the location of the atresia. The serious nature of many of them makes perioperative diagnosis imperative. Eighty-six babies born with pure oesophageal atresia (OA), duodenal atresia (DA) or stenosis, or jejuno-ileal atresia (JIA) have been studied. These, combined with over 2,000 cases in the literature, have been used to develop a protocol to optimally investigate babies with gut atresia for associated anomalies. The authors recommend routinely obtaining anterio-posterior and lateral chest and abdominal radiographs for babies with pure OA, DA and intestinal atresia, making sure the entire spine can be visualised. Cardiac and renal ultrasonography (US) should be routine in all babies with pure OA or DA. A micturating cystourethrogram should be done in those babies with abnormal urinary tract US or an associated anorectal anomaly. A sweat test should be obtained in babies with JIA, and a rectal biopsy should be taken in babies with the combination of Down's syndrome and DA to exclude Hirschsprung's disease.