1. Rebecca F. Brown1, MD; Kimberly Erickson1,2, MD
Large Gap Congenital Pyloric Atresia with Meconium Peritonitis: When Gastrojejunostomy is the Best Option
Rebecca F. Brown1, MD; Kimberly Erickson1,2, MD
1-Department of Surgery, 2-Division of Pediatric Surgery, University of North Carolina School of Medicine, Chapel Hill, NC
Case Presentation
A term (37 3/7) male, born to a 28 y/o G4P3 mother, was transferred to our facility for subspecialty care after developing abdominal distension at 7 hours of life. Pregnancy was complicated by polyhydramnios. Pre-operative work-up included an abdominal radiograph, upper GI, and barium enema (see images). The stomach was decompressed via an orogastric tube and IV resuscitation was initiated.
The patient was taken to the operating room on DOL 3 for an exploratory laparotomy. Loculated meconium stained fluid was encountered gastrotomy was performed to evaluate gastric outlet; complete obstruction of the distal stomach was found. This was consistent with a distal antral or pyloric atresia. The first portion of the duodenum was not well visualized due to significant meconium staining and associated inflammation of this area. The distal second and third portion of the duodenum were examined and noted to be normal. Given the lack of normal first portion of the duodenum, a Billroth I operation did not seem an appropriate choice. The decision was made to proceed with loop gastrojejunostomy, which was completed without difficulty.
Post-operatively, the stomach was decompressed with an orogastric tube and the patient was started on TPN and a proton pump inhibitor. The patient tolerated placement of orogastric tube to straight drain on POD 5 and passed a bowel movement on POD 8. Oral feeds were initiated and titrated to goal by POD 10. After tolerating goal oral feeds for several days, the patient was discharged home. Outpatient follow up initially revealed poor weight gain. After nutrition education and formula supplementation, the patient is thriving and gaining appropriate weight at 8 months after operation.
Image 1: Plain abdominal radiograph showing stomach bubble and calcifications in abdomen & scrotum
Image 2: Pre-operative upper GI showing no passage of contrast beyond the stomach
Image 3: Pre-operative barium enema showing patent colon and distal ileum
Background
Discussion
Congenital pyloric atresia
Rare – affects 1 in 100,000 live births, <1% of all intestinal atresias
Three types of CPA described
Type 1: pyloric membrane or web, spares muscular layer of stomach
Type 2: solid, fibrous cord connecting the antrum to the duodenum
Type 3: true gap between the stomach and duodenum
50% association with other anomalies
Epidermolysis bullosa
Other intestinal atresias
Few case reports describing neonatal gastric perforation or tearing of gastric serosa resulting from CPA, especially with delayed diagnosis
Traditional treatment options
Restore bowel continuity
Type 1: excision of diaphragm and pyloroplasty
Type 2 & 3: gastroduodenostomy
Congenital pyloric atresia (CPA) is a rate, but well described congenital defect of the intestinal tract
Gastroduodenostomy is the preferred procedure for Type 2 and 3 defects to decrease post operative morbidity
Our interesting patient presented with large duodenal gap and inflammation related to meconium peritonitis
 precluded gastroduodenostomy, loop gastrojejunostomy performed and well tolerated
 patient continues to meet nutritional and growth milestones 8 months post-operatively
Loop gastrojejunostomy is a viable alternate procedure in congenital pyloric atresia when inflammation or anatomy precludes formal Billroth reconstruction
References
1. Knouff S, Klein A, Kaminski M. Pyloric Atresia in the Neonate. Neonatal Network Nov-Dec;33(6):
2. Al-Salem AH, Abdulla MR, Kothari MR, Naga MI. Congenital pyloric atresia, presentation, management, and outcome: a report of 20 cases. J Pediatr Surg Jul;49(7):
4. Gunaydin M, Rizalar R, Bozkurter AT, Tander B, Ariturk E, Bernay F. Gastric serosal tear due to congenital pyloric atresia: a rare anomaly, a rare complication. Afr J Paediatr Surg May-Aug;8(2):232-4.
3. Joshi M, Krishnan L, Kuruvila. Large Gastric Perforation in Carmi Syndrome: A Morbid Complication in a Rare Association. J Neonatal Surg Oct 1;1(4):57
5. Tan KL, Murugasu JJ. Congenital Pyloric Atresia in Siblings. Arch Surg Jan;106(1):100-2