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Intestinal atresia and stenosis: a 25-year experience with 277 cases
L K Dalla Vecchia1, J L Grosfeld, K W West
1Department of Pediatric Surgery, James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis 46202, USA.
Insights
This study analyzed 277 neonates with intestinal atresia, finding low operative mortality but identifying cardiac anomalies and short-bowel syndrome as key factors for long-term morbidity and mortality in infants.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Gastroenterology
Background:
- Intestinal atresia is a congenital condition requiring surgical intervention in neonates.
- Understanding the causes, management, and outcomes is crucial for improving patient care.
Purpose of the Study:
- To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcomes in infants with intestinal atresia.
- To identify factors contributing to morbidity and mortality in this patient population.
Main Methods:
- A retrospective case series of 277 neonates with intestinal atresia and stenosis treated between 1972 and 1997.
- Data collected on level of obstruction, associated anomalies, surgical interventions, and outcomes.
- Analysis of operative mortality, long-term survival, and causes of morbidity.
Main Results:
- Operative mortality rates were 4% for duodenal, 0.8% for jejunoileal, and 0% for colonic atresia.
- Long-term survival rates were 86% (duodenal), 84% (jejunoileal), and 100% (colonic).
- Short-bowel syndrome was present in 32 neonates, impacting outcomes.
Conclusions:
- Cardiac anomalies and ultrashort-bowel syndrome are major causes of morbidity and mortality.
- Long-term total parenteral nutrition can lead to complications like liver disease.
- Growth factors and potential advances in small bowel transplantation may improve long-term outcomes.
Objective:
To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia.
Design:
Retrospective case series.
Setting:
Pediatric tertiary care teaching hospital.
Patients:
A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%).
Interventions:
Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates.
Main Outcome Measures:
Morbidity and early and late mortality.
Results:
Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence.
Conclusions:
Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.