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At least 1,405 records · Page 78Linked to original sources
PubMed2001

Nonfixation of an atretic colon predicts Hirschsprung's disease.

PURPOSE: After noting the colon to be nonfixed and coiled in the pelvis of a patient with colonic atresia and total colonic Hirschsprung's disease, the authors sought to determine whether this colonic position might be predictive of aganglionosis in the atretic colon. METHODS: The authors reviewed all cases of colonic atresia treated in their institution over the past 2 decades with regard to colonic orientation and the presence of aganglionosis. RESULTS: We identified 9 patients with colonic atresia. Two of these patients, as well as one patient with ileal atresia, also had long-segment Hirschsprung's disease. All 3 of these patients had foreshortened nonfixed colons located predominantly in the pelvis. None of the other 6 patients with colonic atresia had this configuration. Reviewing 12 reported cases from other institutions of total colonic aganglionosis associated with atresia, 7 appear to have had a pelvic nonfixed colon; colonic orientation could not be determined from the remaining reports. The diagnosis of Hirschsprung's disease was not established in any case before repair of the atresia, and each patient required a secondary enterostomy. CONCLUSIONS: An early gestational atresia, occurring before secondary retroperitoneal fixation of the colon at approximately 11 weeks may result in interruption of caudal migration of enteric nerves manifesting as Hirschsprung's disease distal to the atresia. Moreover, the aganglionosis may be predicted by a foreshortened, nonfixed colon coiled in the pelvis. Thus, biopsy of the colon should be performed at initial exploration in all cases of atresia in which the colon is not properly fixated to avoid immediate or delayed anastomosis to an aganglionic colon.

Colon↗
PubMed1976

Multiple gastrointestinal atresias, with intraluminal calcifications and cystic dilatation of bile ducts: a newly recognized entity resemblin "a string of pearls".

The siblings reported here represent a new and complex congential anomaly. A genetic basis is strongly suggested. The natural preparation of closed loop duodenal obstruction and cystic dilatation of the bile ducts is a unique finding. The hallmark of this new entity--intraluminal calcifications strung out through small and large intestine like pearls--is a striking radiographic feature and, in the proper clinical setting, pathognomonic. This entity has been uniformly lethal. Once the diagnosis is confirmed, no known therapy is useful.

Abnormalities, Multiple↗
PubMed1986

Preduodenal portal vein with situs inversus and duodenal atresia.

In a 7-day-old infant referred because of bile-stained vomiting, jaundice and lack of meconium, radiological examination revealed the 'double-bubble' sign of duodenal atresia as well as dextrocardia. This infant also had a strawberry haemangioma on the right shoulder. Operation disclosed situs inversus and a preduodenal portal vein as well as duodenal atresia. A side-to-side duodeno-jejunostomy was performed successfully without damage to the anomalous vein. The history of polyhydramnion during gestation, the presence of other anomalies, the rapid onset of bile-stained vomiting and the classic 'double-bubble' sign, together appeared to indicate that the duodenal atresia was intrinsic and not due to the external pressure of the anomalous vein on the duodenum.

Dextrocardia↗
PubMed2006

[Perioperative management for laparoscopic repair of congenital duodenal atresia].

BACKGROUND: Laparoscopic surgery has been applied even to neonates. To examine the safety and efficacy of laparoscopic repair of neonatal congenital duodenal atresia, we compared the 5 laparoscopic cases with the 5 conventional surgical cases. METHODS: The charts were retrospectively reviewed to investigate the anesthetic management, perioperative status and complications in the most recent 5 cases each of laparoscopic and conventional surgeries. RESULTS: There was a tendency to avoid laparoscopic repair in the patients with congenital heart disease. There were no intraoperative complications in both groups. Laparoscopic group exhibited less blood loss but longer operation time. In the two out of five laparoscopic cases re-operation was required due to technical issues, and the group needed a longer period before starting enteral feeding and longer hospitalization. CONCLUSIONS: Up to this time, laparoscopic repair of congenital duodenal atresia exhibited few advantages over conventional open repair.

Anesthesia↗
PubMed1994

[Duodenal atresia].

The article deals with 5-year (1986-1992) experience in the diagnosis and treatment of duodenal atresia in 26 infants. Timely diagnosis of this developmental anomaly, the variants of operative treatment, and the principles of postoperative management are discussed. The necessity of antenatal ultrasonic examination and the high diagnostic value of radiological and endoscopic methods of diagnosis are emphasized. Operations were conducted on 25 children for total high intestinal obstruction, on 23 infants in the neonatal period. The continuity of the intestinal tube was restored by duodenoduodenostomy (12), duodenojejunostomy (3), gastroduodenostomy (1), excision of the membrane (4), and dissection of the membrane (5). The latter is technically the simplest and is the operation of choice. Preliminary jejunostomy for adequate preparation for the radical operation is recommended in profoundly preterm infants with as serious premorbid background. Of high importance is postoperative enteral catheteral feeding which is begun from the first day after the operation. Seven out of the 26 patients died from severe combined developmental anomalies and infection. The mortality was 26.9%.

Duodenal Obstruction↗
PubMed1992

Hypoglossia-hypodactylia syndrome with jejunal atresia in an infant of a diabetic mother.

Despite advances in therapy for maternal diabetes, pregnancies of diabetic women remained at an increased risk of spontaneous abortion or delivery of an infant with major malformation. We report on an infant of a diabetic mother with hypoglossia-hypodactylia associated with complete jejunal atresia. A common pathogenesis for these 2 malformations could be a vascular disruptive mechanism with in utero arterial thrombosis.

Adult↗
PubMedDate not supplied

[Duodenal atresia and choledochal cyst: a rare combination].

Congenital duodenal atresia is usually associated with various congenital anomalies. The embryological events in the development of the biliary tract and duodenum are linked. Although an association between duodenal atresia and biliary anomalies is predictable, it is rarely observed. We describe the case of a 27 year-old woman, operated on for duodenal atresia in her childhood, who presented cholangiolitis. Morphologic investigation allowed the diagnosis of choledochal cyst with an intracystic stone developed in the pancreatic head. A pancreatico-duodenectomy was performed because of the importance and the location of dilatation and stone with biliary obstruction. To our knowledge, the association between a congenital duodenal atresia and a choledochal cyst revealed at adult age had never been described in the literature.

Adult↗
PubMed2006

Facial asymmetry with colon atresia.

This case report describes an unusual combination of birth defects in a black South African child presenting with colon atresia, facial symmetry due to facial microsomia and brain abnormalities. The suspected aetiology is thought to be vascular disruption in the late embryonic period.

Colon↗
PubMed1996

Rectal atresia as rare manifestation in EEC syndrome.

A newborn boy presented with bilateral split hand/foot malformation, sparse hair, dry and scaly skin, and nasolacrimal duct obstruction. Despite absence of cleft lip or palate, the findings fit the EEC syndrome. Additionally, the boy had rectal atresia. At least six further patients with EEC syndrome and anal atresia (two published, four unpublished) demonstrate, that anorectal malformation is a further, but rare anomaly in EEC syndrome.

Abnormalities, Multiple↗
PubMed1989

A case of VACTEL syndrome with antenatally diagnosed duodenal atresia.

A rare case of VACTEL syndrome with antenatally diagnosed duodenal atresia is reported. We performed one-staged radial surgery for the esophageal atresia with tracheoesophageal fistula and duodenal atresia. The prognosis of VACTEL syndrome with duodenal atresia is very poor. This is the first reported case cured operatively.

Abnormalities, Multiple↗
PubMed1987

Apple peel jejunal atresia.

Apple peel deformity of the small bowel is a variant of jejunal atresia with a high mortality. Forty five percent of these patients can be expected to die, most from anastomotic leaks with sepsis, or anastomotic failure with prolonged ileus and malnutrition. This report documents survival of three consecutive patients treated by an adaptation of the Santulli "chimney" anastomosis. This method accomplishes the goals of relief of intestinal obstruction, minimal resection, and preservation of tenuous intestinal blood vessels. All three patients had a benign postoperative course and made an uneventful recovery. Intestinal function is normal in each patient at 1 1/2 years. Based on this experience, it appears that a Santulli jejunojejunostomy combined with total parenteral nutrition offers the most promising operative strategy in the treatment of patients with apple peel bowel.

Female↗
PubMed1997

Small-bowel continuity: a crucial factor in determining survival in gastroschisis.

A retrospective analysis of a series of 63 cases of gastroschisis managed over an 11-year period distinguished a single statistically significant prognostic factor. There were 6 (9.5%) deaths, of which 4 occurred in the 8 infants with small-bowel atresia/stenosis (P < 0.005, Fisher's exact test). One died at 48 h and the remaining 3 of liver disease related to total parenteral nutrition. Of the 4 survivors, 1 developed a late biliary stricture necessitating hepaticoenterostomy but is alive and well aged 4 years. The remaining 3, following initially prolonged hospitalisations and multiple operations, are alive and well after 2, 4 and 7 years. In 3 patients the atresia was not detected at the primary operation. The small number of cases of gastroschisis-associated small-bowel atresia seen in any one unit may conceal the importance of the problem, and limits experience in the approach to management.

Abdominal Muscles↗
PubMed1987

A case of congenital colonic atresia associated with atresia ani.

The association of atresia ani with congenital colonic atresia is extremely rare, with only one such case having been reported up to date. We have recently treated a female infant with atresia ani (covered anus complete) accompanied by atresia of the sigmoid colon, who was managed by three stage surgery.

Anal Canal↗
PubMed1992

Simultaneous correction of biliary atresia and small bowel atresia in the neonate.

The coexistence of biliary atresia and small bowel atresia has been described. However, attempts to correct the biliary atresia at the same time as small bowel atresia repair have not been detailed. In 1986, a 2,170-g female was delivered at 36 weeks' gestation. Postpartum, duodenal obstruction was suspected after oral feedings were not tolerated. At 37 hours after birth, laparotomy showed unsuspected biliary atresia in addition to intestinal malrotation, duodenal web, and type IIIb jejunal atresia with an apple-peel configuration of the upper jejunum and mesentery supplied by the superior mesenteric artery with normal small bowel length and normal ileal mesentery. A modified Roux-en-Y hepatic portoenterostomy was performed using the blind end of the apple-peel configuration of jejunum, with a side-to-side duodenojejunostomy 25 cm from the portoenterostomy. Microscopic examination of the fibrous extrahepatic biliary tissue showed a disarray of small bile ductules. The maximal ductal size at the portoenterostomy site was 600 microns. A liver biopsy specimen showed normal architecture with eosinophils infiltrating the portal triads and periductal areas. Postoperatively, she has maintained normal growth and development with no episodes of cholangitis. Liver biopsies at 1 year and 3 years were normal. At 4 years, total and conjugated bilirubin, serum proteins, liver enzymes, coagulation factors, and trace elements were normal. We conclude that simultaneous correction of biliary atresia and small bowel atresia can be accomplished in the neonate with excellent early results.

Biliary Atresia↗