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PubMed1984

Neonatal colonic atresia: ultrasound findings.

High resolution real-time ultrasound examination of the abdomen was performed in a neonate with abdominal distention. Dilated distal small bowel and a complex mass in the course of the ascending colon were noted. Ultrasound correctly suggested the diagnosis of mid-colonic atresia with a patent ascending colon twelve hours prior to the radiographic demonstration of gas in the obstructed right colon. The sonogram was also able to accurately exclude the presence of ascites or a renal or hepatic mass as the cause for abdominal distention.

Colon↗
PubMed1980

Severe congenital skin defects in a newborn. Case report and relevance of several obstetrical parameters.

The clinical, laboratory, histologic and autopsy findings are reported from a live-born male infant with severe congenital skin defects (CSD) who survived for 2 days. The family history revealed consanguinity of the (Turkish) parents. The patient was compared with 10 cases from the literature with the most severe form of CSD. The combination of severe CSD, parental consanguinity and gastrointestinal atresia was found in 3 of these 11 cases, including our own patient. Differentiation from an atypical form of epidermolysis bullosa, complicated by pyloric atresia, is difficult. The mechanism of the (prenatally detected) elevated amniotic fluid alpha 1-fetoprotein (AFP) level is discussed. The finding of a balanced 13q14q chromosome translocation in the infant and his mother is considered a coincidence.

Abnormalities, Multiple↗
PubMed1994

[Characteristics of the surgical treatment of total duodenal atresia].

The authors analysed the results of surgical management of total duodenal atresia in 12 newborns. In 10 of them the operation was successful. Duodenojejunoanastomosis on a short jejunal loop was formed through a window in the mesocolon in all patients. The success of the treatment is determined by careful performance of all the details of the surgical intervention. Of particular importance are such elements of the operation as the formation of the spur from the afferent genu of the jejunum and fixation of the anterior duodenal wall in the window in the mesocolon. It is preferable to create the anastomosis with a single row of interrupted sutures of synthetic atraumatic material 5/0-6/0 with the knots in the intestinal lumen.

Anastomosis, Surgical↗
PubMed1998

Anomalies in Down syndrome individuals in a large population-based registry.

In a population of close to 2.5 million infants born from 1983 to 1993 registered in the California Birth Defects Monitoring Program, we compared the prevalence of structural birth defects among 2,894 infants with Down syndrome (DS) with that of infants without DS. Among 61 defects uniformly ascertained in affected and unaffected infants, 45 were significantly more common in DS, with atrioventricular canal (risk ratio = 1,009), duodenal atresia (risk ratio = 265), and annular pancreas (risk ratio = 430) being the most common. Most defects of blastogenesis and most midline defects were either nonsignificantly associated or not observed in infants with DS. Theories on the pathogenesis of defects in trisomies must account for the lack of and for the presence of specific defects.

Congenital Abnormalities↗
PubMed2002

Prenatally closed gastroschisis with midgut atresia.

Spontaneous prenatal closure of gastroschisis (GS) is rare and usually associated with atresia of the midgut. We describe a case of GS diagnosed at 20 weeks' gestation that resolved spontaneously in utero. At delivery the infant had an ileus. A laparotomy with a jejunocolostomy was performed, but she died at 2 months of age due to complications of total parenteral nutrition.

Adult↗
PubMed1992

Agenesis of the gallbladder in duodenal atresia: two case reports.

Agenesis of the gallbladder not associated with biliary atresia is quite rare. We wish to report two patients with gallbladder agenesis in association with duodenal atresia without biliary atresia. Liver biopsy in one patient was notable for an absence of both inflammation and ductal pathology. The unremarkable postoperative course included normal direct bilirubins and a HIDA scan that demonstrated very good bile excretion into the duodenum. Both children are doing quite well in follow-up.

Adult↗
PubMed1999

Ulceration in an ileocolic anastomosis treated with ranitidin.

The authors report the case of a child born with a gastroschisis and an ileal atresia. After surgery, only 100 cm small bowel and the distal one third of his colon remained. Perianastomotic ulcers developed 6 years later. These were treated successfully with ranitidin, a treatment not previously reported in the literature. The authors conclude that treatment with ranitidin was successful in a patient with an ulcer in an ileocolic anastomosis.

Anastomosis, Surgical↗
PubMed2000

Chromosome 22q11 deletion complicated by dissecting pulmonary arterial aneurysm and jejunal atresia in an infant.

We present an autopsy case of a 46-day-old male infant with chromosome 22q11 deletion, which is considered the primary cause of several diseases, including DiGeorge syndrome and velocardiofacial syndrome. The patient had 2 notable congenital abnormalities: multiple dissecting pulmonary arterial aneurysms distributed in both lungs and multiple jejunal atresia with apple-peel deformity. The former, a very rare pathologic condition especially in infancy, was found incidentally at autopsy and was the primary cause of death. To our knowledge, neither of these lesions has been reported previously in a patient with chromosome 22q11 deletion.

Aortic Dissection↗
PubMed2001

Colonic atresia: surgical management and outcome.

Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprung's disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome.

Anastomosis, Surgical↗
PubMed2002

Trends in the management and outcome of jejuno-ileal atresia.

The aim of this study was to evaluate contemporary patterns of presentation and trends in the management and outcome of newborn infants with jejuno-ileal atresia at a regional paediatric surgical centre in the United Kingdom. The hospital neonatal surgical registry was used to identify patients with jejuno-ileal atresia (n = 83) admitted between 1976 - 1998, excluding those associated with gastroschisis. The clinical records were reviewed and antenatal information, patient demographics, associated anomalies, operative treatment, post-operative management and outcomes were analysed in three time periods to identify trends in management and survival: Group 1 1976 - 1982 (n = 32), Group 2 1983 - 1990 (n = 21), and Group 3 1991 - 1998 (n = 30). Overall survival was 90 %. The number of patients with associated anomalies were Group 1, 10 (31 %); Group 2, 7 (33 %); and Group 3, 11 (37 %). Cystic fibrosis was encountered in 4 (13 %), 1 (5 %) and 4 (13 %) patients, respectively. Resection with primary anastomosis was the definitive management in most of patients: Group 1, 25 (78 %); Group 2, 17 (81 %); and Group 3, 27 (90 %). Initial stoma followed by delayed primary anastomosis was performed in 14 infants; eight patients had divided stomas while 6 had Bishop-Koop stoma. Tapering was used in 10 patients (12 %) with proximal jejuno-ileal atresia. Parenteral nutrition was increasingly utilised over the three time periods studied. There were no deaths in Group 3 compared to 6 deaths in Group 1 and 2 in Group 2 (P = 0.02). Most of the deaths were due to overwhelming sepsis. Mortality did not correlate significantly with the TYPE of atresia, presence of associated anomalies or the need for long-term total parenteral nutrition. The overall complication rate in survivors was 18 %. In the infants undergoing Bishop-Koop operation the complication rate was 50 %. This study has shown a significant reduction in mortality from jejuno-ileal atresia, which may be attributed primarily to advances in perioperative management, including parenteral nutrition. Generous resection of the atretic segment with primary anastomosis is more frequently employed in preference to initial stoma formation. Cystic fibrosis remains an important co-morbid condition that must be excluded promptly in all newborns.

Case-Control Studies↗
PubMed2005

Analysis of postoperative reoperation for congenital duodenal obstruction.

OBJECTIVE: To analyse the risk factors for reoperation after initial surgical repair of congenital duodenal obstruction and demonstrate that they can be decreased with more careful attention and more advanced techniques during surgery. METHODS: The records of newborns and infants (aged 0-2 months) who had surgical therapy for congenital duodenal obstruction in the past 30 years were reviewed and analysed. Of the 298 patients, 132 (44%) were boys and 166 (56%) were girls. All patients who underwent repeat surgery postoperatively were evaluated by the reasons for surgery. The number of patients with various combination lesions of congenital duodenal obstruction was also calculated and the relationship to postoperative reoperation was analysed. RESULTS: Twenty patients (6.7%) had congenital duodenal obstruction with combination lesions including duodenal web, malrotation, annular pancreas and multiple duodenal web. Twelve patients required further operation 5 days to 2 years postoperatively for complications (n = 5) and other duodenal atresias that were not discovered initially (n = 7). CONCLUSION: More than half of reoperated patients (7/12) had multiple lesions of duodenal obstruction that were missed during the primary operation. The postoperative reoperation rate for congenital duodenal obstruction could be decreased with more careful attention to operative details and more preoperative and intraoperative evaluation of the gastrointestinal tract.

Case-Control Studies↗
PubMed1981

[Gastrostomy in the newborn - indication, technique, complications (author's transl)].

Performing a gastrostomy is an easy operative procedure. Indications for this procedure include neonates and children with congenital deformities of the oesophagus, dysphagia for different reasons, small bowel atresias, exomphalos and diaphragmatic hernias. The gastrostomy is performed in the left upper abdomen through a small vertical midline incision. Between 1970 and 1979, gastrostomy has been performed in 143 newborns and children. In none of these cases did a serious complication occur. The main advantages are: decompression of the abdomen, improvement of the ventilation and early enteral quantitative feeding.

Child↗
PubMed2005

Rectal atresia.

Rectal atresia is a rare condition, with a reported incidence of 1-2% of all anorectal anomalies. The extensive list of ingenious operative procedures used for the correction of this malformation is testimony to the great difficulty faced in treating this anomaly. Posterior sagittal anorectoplasty using end-to-end anastomosis is a safe, technically well known that achieves this goal.

Anastomosis, Surgical↗
PubMed1999

Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia.

The embryogenesis of pancreaticobiliary maljunction is inferred from the embryogenesis of duodenal atresia. The epithelial cells of the duodenum begin to proliferate and completely plug the lumen, but a process of vacuolation recanalizes the duodenum. Recanalization of the common duct frequently appears with two lumina and openings into the duodenum with two orifices. These two major canals create a narrow segment of the duodenum and this narrow zone is the area in the duodenum that is most prone to faulty recanalization and atresia formation. A bifid biliary system inserts at blind upper and lower pouches of the duodenum, and the common bile duct inserts in a Y fashion. The common bile duct inserts at the stenotic segment, and the end result is a T-shaped formation in patients with duodenal stenosis. During the development of the bile duct, abnormal fusion may occur between the bile duct and branches of the right ventral pancreatic duct. The site in the bile duct where a branch of the pancreatic duct joins is likely to develop atresia due to disturbance of the recanalization process, as seen in duodenal atresia. Severe impairment of vacuolation causes divided atretic bile duct at the site where the pancreatic duct inserts in a Y-fashion into the upper and lower bile duct. Moderate impairment of vacuolation causes a stenosis at the site where the pancreatic duct inserts in a T-shape, with a moderate dilatation of the bile duct.

Choledochal Cyst↗
PubMed1989

Congenital intrinsic duodenal obstruction: problems in the diagnosis and management.

Nineteen infants with intrinsic duodenal obstruction are analyzed. Atresia was the most common lesion. An exceptionally high rate of associated anomalies (73.7%) were present; Down's syndrome, the single most common anomaly, was seen in 47% of the infants. In seven infants, the diagnosis was delayed and in another three it was made intraoperatively while establishing a gastrostomy for esophageal atresia. Three infants died without operation because of gross prematurity and multiple anomalies. Of the 16 operated on, three died, one due to peritonitis and the other two because of metabolic derangements. Of the various operative procedures used, no significant difference was found in the final outcome of treatment. A schematic approach to the diagnosis and management is proposed.

Anastomosis, Surgical↗
PubMedDate not supplied

[Oral cholangiography and duodenal atresia].

A newborn male patient with trisomy-21 presented with bilious hemesis. The patient was icteric with slight hepatomegaly. Simple abdominal X-ray and upper gastrointestinal series with barium showed a dilated duodenal loop and inflammatory changes involving the duodenal mucosa. This image known as "double bubble" is characteristic of congenital duodenal obstruction. Simultaneously the gallbladder and choledochus were visualized. The former X-ray finding is very unusual. An uneventful Kimura procedure was performed.

Cholangiography↗
PubMed1984

Early postoperative enteral feeding through a needle catheter jejunostomy.

The advantages of early postoperative enteral nutrition through a needle catheter jejunostomy in 13 operated children (1 day to 14 years) are analysed. They presented various surgical or medical diseases. 8 of them had a duodenal atresia or stenosis. Their postoperative follow-up was compared with other children suffering from the same pathology without early postoperative enteral feeding. The simplicity of the realimentation, the absence of septic complications and the metabolic advantages of this method prove to be extremely efficient when a protein and calorie malnutrition must be avoided and fractionated oral feeding is impossible.

Adolescent↗
PubMed1980

[Surgical management of sequelae from duodenal stenosis and atresia in the neonate (author's transl)].

Surgical sequelae from duodenal atresia treated by gastrojejunostomy or duodenojejunostomy are rarely encountered. They usually appear in the late course of gastrojejunostomies. They are related at the duodenal distension above the stenoses which constitute a poorly drained "blind-loop" usually developed from a side to side anastomosis as observed in the small bowel. The following complications, related to the duodenal pouch have been reported in the literature: peptic ulcer, duodenitis or gastritis, abdominal pain, dumping syndrom and malnutrition. One patient developed a gangrenous calculous cholecystitis at 10 years of age. 3 patients under went revision of their previous surgery because of sequelae. One had duodenal atresia above the ampulla, one had an anular pancreas: in one the duodenal atresia was at the ligament of Treitz. All patients had a revision according to our technic described in Annales de Chirurgie Infantile, consisting in: --calibration of the duodenal pouch --end to end or end to side duodeno-duodenal anastomonsis --suppression of previous anastomosis. A good result was obtained in all 3 cases.

Blind Loop Syndrome↗