Our patient was a male, weighing 2400 g, born at 37 weeks of gestation to a 20-year-old G1P1 African-American mother by vaginal delivery. A third-trimester prenatal ultrasound revealed intra-abdominal calcifications and dilated loops of the bowel. The patient was delivered without complications, and his Apgar scores were 8 and 9 at 1 and 5 min, respectively. By day 3 of life, the newborn had still not passed meconium. He was transferred from the delivery hospital to our tertiary-care institution for further evaluation and management.
Physical examination revealed a soft, but distended abdomen. The patient was initially managed by nil per os, nasogastric tube decompression, hyperalimentation, and broad-spectrum antibiotics. Plain abdominal radiography showed multiple dilated bowel loops with air fluid levels, but no evidence of free air (Fig. 1). A water-soluble enema was administered the following day (Fig. 2), which revealed no contrast passage beyond 5 cm proximal to the terminal ileum, indicative of bowel obstructive process.
With failure of progression, the patient was taken to the operating room for an exploratory laparotomy. Upon entering the abdomen, a small amount of meconium-tinged ascitic fluid was encountered. The small bowel was eviscerated and examined from the ligament of Treitz to the terminal ileum. Near the terminal ileum, there was a transition zone with a dense collection of small bowel and a small rim of proximal necrosis. This segment of the bowel was resected back to viable margins, and an end-to-end primary ileo-ileal hand-sewn anastamosis was performed. Further exploration of the abdominal cavity revealed no additional abnormalities, and the abdomen was closed.
Examination of the specimen (Fig. 3) was performed on the back table. A longitudinal incision showed ileo-ileal small bowel intussusception with a rim of proximal necrosis. Meckel’s diverticulum was discovered to be intraluminal, consistent in location with the lead point of the intussusception.
The patient was transferred to our neonatal ICU postoperatively and had an uneventful postoperative course. His bowel function gradually returned, and feeding was initiated on postoperative day 6. His diet was advanced and well tolerated. The patient was discharged home on postoperative day 12. Pathology examination revealed no evidence of heterotopic tissue.
First described in 1508, and later characterized by the German anatomist Johann Friedrich Meckel, Meckel’s diverticulum is a remnant of the omphalomesenteric duct that fails to obliterate during the 50th week of fetal development. Occurring in 1–3% of the population, it is the most common congenital anomaly of the gastrointestinal tract 1. It is a true diverticulum, possessing all three layers of the intestinal wall. During development, the Meckel’s diverticulum contains pluripotent cells that are capable of differentiation to various tissue types, predominantly gastric or pancreatic.
Often found incidentally during radiological studies or at the time of surgery, the majority of Meckel’s diverticula remain asymptomatic 2. The largest case series to date, by following-up 202 patients over the course of 15 years, has revealed a 4–6% lifetime risk of developing a complication, of which gastrointestinal bleeding, obstruction, intussusception, diverticulitis, and perforation are the most common 3. In children, gastrointestinal bleeding due to the presence of acid-secreting gastric mucosa is the most common complication, with a peak incidence between 3 months and 3 years of age 4.
Intrauterine intussusception is a pathological event scarcely described in the literature. In 1960, Talwalker 5 archived 26 known cases of intussuception in newborns that were reported in the literature. At the time, the author noted a ‘cause and effect’ relationship between prenatal intussusception and intestinal atresia. In 1975, Todani et al. 6 reported 24 cases from Japan of intestinal atresia due to intrauterine intussusception. Since then, additional isolated cases of intrauterine intussusceptions in relation to intestinal atresia have been reported 7–11. Surgical intervention remains the definitive for childhood intussusception in the presence of complete small bowel obstruction, failure of hydrostatic reduction, the third recurrence following radiographic reduction, or peritonitis on examination.
Intussusception is a known complication of Meckel’s diverticulum. However, to date, intrauterine intussusception secondary to Meckel’s diverticulum has unknown incidence. In fact, only four reported cases are known to exist 12–15. The exact contribution of the Meckel’s diverticulum to the intrauterine intussusception remains unclear – whether it is by inversion or en-bloc invagination. It is presumed, however, that the Meckel’s diverticulum serves as a pathological lead point to which the bowel intussuscepts with failure for spontaneous reduction. It is likely that ischemia of the intussusceptions led to perforation and circumferential intestinal wall loss, which healed with an interposing segment having necrosed, leading to atresia being discovered at birth. Ileal atresia and/or perforation had occurred in all four reported cases. Furthermore, all cases required exploratory laparotomy, bowel resection, and intestinal primary anastomosis. To our knowledge, ours is the fifth case report of this phenomenon.
Conflicts of interest
There are no conflicts of interest.
1. Banli O, Karakoyun R, Altun H. Ileo-ileal intussusception due to inverted Meckel’s diverticulum. Acta Chir Belg 2009; 109:516–518.
2. Soltero MJ, Bill AH. The natural history of Meckel’s diverticulum and its relation to incidental removal. A study of 202 cases of diseased Meckel’s diverticulum found in King County, Washington, over a fifteen year period. Am J Surg 1976; 132:168–173.
3. Cullen JJ, Kelly KA, Moir CR, Hodge DO, Zinsmeister AR, Melton LJ. Surgical management of Meckel’s diverticulum. An epidemiologic, population-based study. Ann Surg 1994; 220:564–568.
4. Tseng YY, Yang YJ. Clinical and diagnostic relevance of Meckel’s diverticulum in children. Eur J Pediatr 2009; 168:1519–1523.
5. Talwalker VC. Intussusception in the newborn. Arch Dis Child 1962; 37:203–208.
6. Todani T, Tabuchi K, Tanaka S. Intestinal atresia dur to intrauterine intussusception: analysis of 24 cases in Japan. J Pediatr Surg 1975; 10:445–451.
7. Adejuyigbe O, Odesanmi WO. Intrauterine intussusception causing intestinal atresia. J Pediatr Surg 1990; 25:562–563.
8. Rattan KN, Singh Y, Sharma A, Pandit SK, Malik V. Intrauterine intussusception – a cause for ileal atresia. Indian J Pediatr 2000; 67:851–852.
9. Huang WC, Wang CH, Yuh YS, Chen YH, Chu CC. A rare type of ileal atresia due to intrauterine intussusception. Eur J Pediatr 2007; 166:1177–1178.
10. Pueyo C, Maldonado J, Royo Y, Skrabski R, Di Crosta I, Raventós A. Intrauterine intussusception: a rare cause of intestinal atresia. J Pediatr Surg 2009; 44:2028–2030–
11. Sarin YK. Intrauterine intussusception causing ileal atresia. APSP J Case Rep 2010; 1:18.
12. Senocak ME, Buyukpamukcu N, Hicsonmez A. Ileal atresia due to intrauterine intussusceptions caused by Meckel’s diverticulum. Pediatr Surg Int 1990; 5:65–66.
13. Gündoğdu HZ, Senocak ME. Intrauterine intussusception due to Meckel’s diverticulum as a cause of ileal atresia: analysis of 2 cases. Eur J Pediatr Surg 1996; 6:52–54.
14. Kilic N, Kiristioglu I, Kirkpinar A, Dogruyol H. A very rare cause of intestinal atresia: intrauterine intussusception due to Meckel’s diverticulum. Acta Paediatr 2003; 92:756–757.
15. Lin CH, Wu SF, Lin WC, Chen AC. Meckel’s diverticulum induced intrauterine intussusception associated with ileal atresia complicated by meconium peritonitis. J Formos Med Assoc 2007; 106:495–498.