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J Ped Surg Case Reports 1 (2013) 232e234

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Intestinal atresia due to intrauterine intussusception of a Meckel’sdiverticulum

Benjamin R. Huebner*, Kenneth S. Azarow, Robert A. Cusick

University of Nebraska College of Medicine, Children’s Hospital & Medical Center, 8200 Dodge Street, Omaha, NE 68114, USA

a r t i c l e i n f o

Article history:Received 23 May 2013Received in revised form6 July 2013Accepted 9 July 2013

Key words:Meckel’s diverticulumIleal atresiaIntrauterine intussusception

* Corresponding author. 4257 Paxton Ave., Cincinn720 252 4618; fax: þ1 402 955 7405.

E-mail addresses: bhuebner09@gmail.com,bhuebner@unmc.edu (B.R. Huebner).

2213-5766 � 2013 The Authors. Published by Elsevierhttp://dx.doi.org/10.1016/j.epsc.2013.07.005

a b s t r a c t

It is widely acknowledged that jejunoileal atresias result from late intrauterine mesenteric vascularaccidents as shown in Louw and Barnard’s classic study. These accidents include intestinal volvulus,malrotation, intussusception, internal hernia, and strangulation in association with gastroschisis andomphalocele. The incidence of intussusception as the cause of intestinal atresia is between 0.6 and 13.1%.Few case reports have demonstrated Meckel’s diverticulum as the lead point of the intussusceptionleading to a jejunoileal atresia. We present such a case, with an intestinal atresia resulting from intra-uterine intussusception of a Meckel’s diverticulum. The neonate was treated with resection of the atresiawith primary bowel anastomosis and a Meckel’s diverticulectomy with a full recovery.

� 2013 The Authors. Published by Elsevier Inc. Open access under CC BY license.

Meckel’s diverticulum is the most common congenital abnor-mality of the GI tract, occurring in 1e2% of the population. Thelifetime risk of becoming symptomatic is 4%, with over 90% ofsymptomatic patients presenting with painless rectal bleeding,obstruction, or inflammation in a child. Rarely, a symptomaticMeckel’s diverticulum may present in the neonatal age group, withcase reports in the literature of obstruction, inflammation, perfo-ration, and intestinal atresia [1]. We present a case of an intestinalatresia due to an intussuscepted Meckel’s diverticulum, the fifthdescribed case in the English literature and review of the literature[2e5].

1. Case report

A 3146-g white male was born after an uneventful pregnancy at39 weeks gestation by spontaneous vaginal delivery. At birth, theApgar scores were unremarkable. Initially, he tolerated feeds andpassed meconium. However, on day of life 2, he developedabdominal distention with bilious emesis. On exam, the abdomen

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was non-tender but distended with visible loops of dilated bowel(Fig. 1). Abdominal X-rays showed a large stomach bubble withdilated loops of small bowel suggestive of a high-grade obstruction(Fig. 2). Given the presentation, a jejunoileal atresia was suspected,and the patient was taken to the OR. Laparotomy revealed an atresiaof the terminal ileum with proximal and distal loops of bowelending blindly. The proximal bowel was markedly distended. Therewas a dilated proximal end of ileum and a decompressed distal endwith a small mesenteric defect (Type IIIa atresia) (Fig. 3). In openingthe distal segment to perform an anastomosis, a blind intussus-cepted segment of intestine was encountered within the lumen.The location, size, and appearance of the intussuscepted segmentwere consistent with a Meckel’s diverticulum (Fig. 4). About 15 cmof the dilated proximal bowel and 2 cm of the distal bowel wereresected and an end-to-end anastomosis was made. The child hadan uneventful postoperative period and was discharged on post-operative day 14.

2. Discussion

The theory of the origin of intestinal atresia has evolved fromTandler’s theory of non-recanalization of the gut to the currentmost accepted theory of vascular disruption of mesenteric bloodflow leading to bowel necrosis and resorption [6]. Louw and Bar-nard confirmed this hypothesis in 1955 after producing intestinalatresia in puppies following in utero ligation of mesenteric vessels.The intestinal atresia seen in these animals was a Type IIIa atresia

Fig. 1. Patient’s abdomen was quite distended on exam with visible dilated loops ofbowel consistent with an obstruction. (For interpretation of the references to color inthis figure legend, the reader is referred to the web version of this article.)

Fig. 3. Intraoperative laparotomy revealed an ileal atresia with a markedly distendedproximal bowel loop followed by a 5-cm V-shaped (gap) mesenteric defect and a distaldecompressed blind loop. (For interpretation of the references to color in this figure

B.R. Huebner et al. / J Ped Surg Case Reports 1 (2013) 232e234 233

(Fig. 5), with a grossly distended proximal bowel loop followed by ashort V-shaped atresia and small distal bowel.

Dalla Vecchia et al. study reported the characteristics of 128jejunoileal atresias from a single institution [7]. Disruption ofmesenteric blood flow in utero leading to the atresia was associated

Fig. 2. Abdominal X-ray demonstrated a large stomach bubble with dilated loops ofsmall bowel suggestive of a high-grade obstruction.

legend, the reader is referred to the web version of this article.)

with volvulus in 34 neonates (27%), malrotation in 24 (19%), gas-troschisis in 21 (16%), omphalocele in 2 (1.6%), Meckel’s divertic-ulum (without intussusception) in 3 (2.4%), and intussusception in2 neonates (1.6%) [7]. A comprehensive meta-analysis of intestinal

Fig. 4. Specimens resected during laparotomy (top to bottom): 15 cm of dilated ileumproximal to the atresia; intussuscepted segment found in the distal decompressedbowel loop consistent with a Meckel’s diverticulum; stool found in the proximal blindloop; distal decompressed segment of the ileal atresia. (For interpretation of the ref-erences to color in this figure legend, the reader is referred to the web version of thisarticle.)

Fig. 5. Classification of intestinal atresia: Type I, mucosal web atresia with intact bowelwall and mesentery. Type II, blind ends separated by a fibrous cord. Type IIIa, blindends separated by a V-shaped gap mesenteric defect. Type IIIb, apple-peel atresia. TypeIV, multiple atresias. (From Grosfeld JL, Ballantine TVN, Shoemaker R. Operativemanagement of intestinal atresia and stenosis based on pathologic findings. J PediatrSurg 1979;14:368, with permission from Elsevier).

B.R. Huebner et al. / J Ped Surg Case Reports 1 (2013) 232e234234

atresia studies by Todani et al. shows the incidence of intussus-ception as the cause of intestinal atresia is 0.6e13.1% [8]. To date,less than 40 cases of intrauterine intussusception leading to intes-tinal atresia have been adequately documented in the world’sliterature [9]. Within these case reports, a recognizable cause, suchas a Meckel’s diverticulum, of the intussusception is veryinfrequent.

Meckel’s diverticulum results from persistence of the ompha-lomesenteric duct. Normally obliterated in the 5the7th week ofgestation, failure to do so results in an omphalomesenteric ductremnant, which includes a Meckel’s diverticulum. A Meckel’sdiverticulum can act as a lead point for an intussusception, resultingin a vascular accident and necrotic bowel. Resorption of thisnecrotic bowel causes a type IIIa intestinal atresia [6].

Similarly to previous case reports of intussusception inducedintestinal atresia, meconium was passed shortly after birth in ourpatient. Since meconium does not reach the colon until the fourthmonth of gestation, it is thought that Meckel’s diverticulum causesa late intrauterine intussusception and bowel obstruction [10].

The neonate in this casewas treatedwith resection of the atresiawith primary bowel anastomosis and a Meckel’s diverticulectomy.Dalla Vecchia et al. report similar treatment in 45 of their patients(35%) [7]. Additional treatments included resection with taperingenteroplasty in 23 neonates (18%), temporary ostomy in 54 (42%),enterotomy with web excision in 5 (4%), and a Bianchi longitudinalintestinal lengthening procedure in 1 (0.8%). Postoperative coursewas uneventful and the neonate made a full recovery in our case.

This is the fifth case report in the English literature of intra-uterine intussuscepted Meckel’s diverticulum leading to an

intestinal atresia. The first three cases were described in Turkey,with the first two cases from the same institution [3e5]. The fourthcase was described in Taiwan [2]. This rare presentation of a com-mon congenital abnormality has an excellent prognosis with earlyidentification and surgical correction. While this is only the fifthcase of intestinal atresia due to a Meckel’s diverticulum described,ileal atresias resulting from idiopathic intrauterine intussusceptionor other vascular accident in the neonatal period aremore common.We suspect that some or many of these previously described ilealatresias may be the result of an intrauterine intussusception of aMeckel’s diverticulum rather than an isolated idiopathic intussus-ception or other vascular accident.

3. Conclusion

The theory of jejunoileal atresia as a result of disruption ofmesenteric blood flow is now widely accepted doctrine. Althoughthe vascular accident is rarely associated with either a Meckel’sdiverticulum or intussusception, a Meckel’s diverticulum acting asthe intussusceptum is even more rare. We reported such a case in a2-day old neonate.

Sources of fundingNone.

Conflict of interest statementNone.

Consent

Written informed consent was obtained from the patient forpublication of this case report and accompanying images. A copy ofthe written consent is available for review by the Editor-in-Chief ofthis journal on request.

Acknowledgment

None.

References

[1] Sinha CK, Fishman J, Clarke SA. Neonatal Meckel’s diverticulum: spectrum ofpresentation. Pediatr Emerg Care 2009;25:348e9.

[2] Lin CH, Wu SF, Lin WC, Chen AC. Meckel’s diverticulum induced intrauterineintussusception associated with ileal atresia complicated by meconium peri-tonitis. J Formos Med Assoc 2007;106:495e8.

[3] Gundogdu HZ, Senocak ME. Intrauterine intussusception due to Meckel’sdiverticulum as a cause of ileal atresia: analysis of 2 cases. Eur J Pediatr Surg1996;6:52e4.

[4] Kilic N, Kiristioglu I, Kirkpinar A, Dogruyol H. A very rare cause of intestinalatresia: intrauterine intussusception due to Meckel’s diverticulum. Acta Pae-diatr 2003;92:756e7.

[5] Senocak ME, Buyukpamukcu N, Hicsonmez A. Ileal atresia due to intrauterineintussusception caused by Meckel’s diverticulum. Pediatr Surg Int 1990;5:64e6.

[6] Louw JH, Barnard CN. Congenital intestinal atresia. Lancet 1955;269:1065e7.[7] Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA.

Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg1998;133:490e7.

[8] Todani T, Tabuchi K, Tanaka S. Intestinal atresia due to intrauterine intus-susception: analysis of 24 cases in Japan. J Pediatr Surg 1975;10:445e51.

[9] Nguyen DT, Lai E, Cunningham T, Moore TC. In utero intussusception pro-ducing ileal atresia and meconium peritonitis with and without free air: reportof two cases and review of literature. Pediatr Surg Int 1995;10:406e8.

[10] Rachelson MH, Jernigan JP, Jackson WF. Intussusception in the newborn infantwith spontaneous expulsion of the intussusceptum; a case report and reviewof the literature. J Pediatr 1955;47:87e94.