J Ped Surg Case Reports 1 (2013) 157e159

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Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Multiple intestinal atresia with apple peel syndrome successfully

treated with primary repair
Barrie S. Rich, Matthew Bott, Nitsana Spigland*
Department of Surgery, Weill Cornell Medical College, 525 East 68th Street, New York, NY 10065, USA

a r t i c l e i n f o a b s t r a c t

Article history: We report a case of mixed type IIIb and type IV intestinal atresia. The baby underwent exploratory
Received 3 April 2013 laparotomy where he was found to have the entire distal bowel supplied by one blood vessel that
Received in revised form appeared to have undergone an antenatal volvulus. The volvulus was untwisted and upon further
20 May 2013
exploration the patient was noted to have twelve distal atresias. We performed a total of six primary
Accepted 24 May 2013
small bowel anastomoses. The baby did not require any further procedures and did well post-operatively.
We conclude that patients with this congenital abnormality can be treated with primary anastomoses,
and when appropriate, without the need for enterostomy or stent.
Key words:
Apple peel atresia
Ó 2013 Elsevier Inc. All rights reserved.
Multiple intestinal atresia
Jejunoileal atresia

Intestinal atresia is a frequently encountered congenital mal- 1. Case report

formation seen in neonates, with jejunoileal atresias being the most
common anomalies of the small bowel. In the United States it is We present a 2.605-kg boy, born by spontaneous vaginal de-
seen in approximately 2.9 cases per 10,000 live births. The most livery at 35 2/7 weeks gestation with a known diagnosis of possible
common types of intestinal atresias are those that are categorized bowel atresia/obstruction. The mother was a 37-year-old, G2P2,
as type I, II, or IIIa, based on the Martin and Zerella classification [1]. whose pregnancy was complicated by colonization with group B
The complex jejunointestinal atresias, or type IIIb (apple peel streptococcus, which was adequately treated prior to delivery.
atresia) and type IV (multiple intestinal atresias) are less common Prenatal ultrasonography reports revealed echogenic bowel,
and are associated with higher morbidity and mortality rates, without any other abnormality. Repeat ultrasonography at a later
although there have been recent improvements in outcome [2]. Lee date showed multiple loops of dilated bowel up to 26 mm with
et al. recently reported a 12.1% rate of complex jejunoileal atresia active peristalsis. Upon birth, the baby was found to be distended
among all patients with small intestinal atresias, and these patients and a nasogastric tube was placed with 150cc of bilious drainage
suffered increased morbidity and mortality rates. None of these suctioned. There was no meconium at birth. The baby was admitted
patients had a combination of type IIIb and type IV [2]. This com- to the neonatal intensive care unit. An abdominal X-ray was
bination is rare, and recently was described by Federici et al. These obtained which showed mildly dilated small bowel present, likely
authors describe a 2.2 g premature newborn with 11 small bowel proximal jejunum, with paucity of distal air in the small intestine
atresias combined with an apple peel deformity. He was treated (Fig. 1).
with 5 end-to-end anastomoses, two ostomies, and a trans- The baby was resuscitated with intravenous fluid and then taken
anastomotic silicone stent [3]. We report another case of this rare to the operating room for urgent exploratory laparotomy. Upon
combination of congenital anomalies. However, we describe the entry into the abdomen, the baby was noted to have markedly
successful use of six intestinal anastomoses; no enterostomy or dilated proximal jejuna extending approximately 10 cm from the
stent placement was performed. ligament of Treitz, with a complete atresia. The entire distal bowel
was supplied by one blood vessel, which was a branch of the ileo-
colic artery. This appeared to have undergone an antenatal volvulus.
The volvulus was untwisted and a large mesenteric defect was
* Corresponding author. Department of Surgery, NewYork-Presbyterian Hospital/
Weill Cornell Medical Center, 525 East 68th Street, New York, NY 10065, USA.
noted. Mobilization of the proximal jejunal limb was completed in
Tel.: þ1 212 746 5648; fax: þ1 212 746 3884. addition to a Kocher maneuver of the duodenum. The entire small
E-mail address: naspigla@med.cornell.edu (N. Spigland). bowel was subsequently run and the patient was noted to have

2213-5766/$ e see front matter Ó 2013 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.epsc.2013.05.015
158 B.S. Rich et al. / J Ped Surg Case Reports 1 (2013) 157e159

Fig. 1. Abdominal X-ray. Abdominal X-ray showing mildly dilated small bowel, likely
proximal jejunum, with paucity of distal air in the small intestine.

twelve distal atresias. The initial pathology is depicted in Fig. 2a.

This required five small bowel resections and reanastomoses.
Attention was then directed at the proximal bowel. A tapering
enteroplasty was performed of the proximal dilated jejunum, and
then a hand-sewn end-to-oblique anastomosis was performed,
making a total of six small bowel anastomoses. The mesenteric
defect was closed. In total, approximately 15 cm of small bowel
segments was resected. The repair is shown in Fig. 2b.
The baby did well post-operatively. He was initially started on
total parenteral nutrition. On post-operative day (POD) 13, a small
bowel series was performed which showed contrast passing Fig. 2. Image of pathology and repair. Panel a illustrates the anatomy upon entrance
throughout the entire small bowel and continued into the rectum. into the abdomen, showing the dilated proximal jejunum, in addition to the multiple
intestinal atresias within the type IIIb atresia. Panel b demonstrates the anatomy
Oral feeds were therefore started on POD #15 and slowly increased
after repair, including the tapering enteroplasty of the proximal dilated jejunum and
to goal on POD #25. The baby was discharged home on POD #28. He multiple primary anastomoses.
was seen as an outpatient on POD #39, 75, and 244 eating well and
gaining weight, without any complication.
overall survival, apple peel atresia continues to have a grave prog-
2. Discussion nosis with significant morbidity and mortality [5,6]. Festen et al.
recently described 15 patients with apple peel atresia and reported
Intestinal atresia is a common anomaly seen in the neonatal a 20% mortality rate. Eight of these patients had multiple atresias,
population. Surgery remains the mainstay of therapy for these ranging from 2 to 4. Eleven patients received primary anastomosis,
children, with the goal of bowel preservation guiding treatment. while four patients received temporary enterostomies. Post-oper-
Patients with simple cases of intestinal atresia (types I, II, and IIIa) ative complications occurred in ten patients, and a re-operation was
have low mortality rates and do well after definitive operative performed in nine patients. Two patients had resultant short bowel
repair [4]. However, Lee et al. describe eleven patients with syndrome. Eleven patients were followed long-term (median 24
complex intestinal atresia. They report a higher morbidity rate months), and all but 3 children were doing well (2 had short bowel
(45.5% vs. 7.5%, p ¼ 0.0032) in this group of patients, compared syndrome and 1 had lactose intolerance, falling below the 5th
to those with simple intestinal atresias. There was one mortality percentile for height and weight). These authors conclude that
in this group as well. They conclude that patients with this complex patients with apple peel atresia that survive the initial operative
anomaly require a multidisciplinary team with long-term term and post-operative period, are likely to experience normal bowel
management of chronic sequelae and additional operative function with adequate growth and development [6].
procedures [2]. We report a case of mixed type IIIB and type IV intestinal atresia
Of the intestinal atresias, apple peel atresia (type IIIb) is the least (with 12 atresias) successfully treated with multiple primary
frequently encountered, comprising approximately 10% of all atre- anastomoses. Federici et al. describes a patient similar to ours with
sias [4]. Although there has been noteworthy improvement in 11 separate atresias in addition to an apple peel tract connected to
 B.S. Rich et al. / J Ped Surg Case Reports 1 (2013) 157e159 159

the cecum. They performed five end-to-end anastomoses. Because 3. Conclusion

of the size discrepancy between the proximal and distal bowel a
double jejunostomy was performed. Furthermore, a silicone stent Our case report documents a complicated case of a patient with
was placed into the distal stoma, threaded through the anastomo- the rare anomaly of a mixed type IIIB and type IV intestinal atresia
ses, ending in the proximal end of the apple peel. The patient was who received six primary anastomoses with a tapering enteroplasty.
seen at 3-year follow-up, and noted to be doing well [3]. The use of Our patient did not receive any further procedures, and was dis-
silicone stenting in the neonatal population has been described in charged home tolerating a diet. We conclude that patients with this
patients with both multiple intestinal atresias and necrotizing congenital abnormality can be treated with primary anastomoses,
enterocolitis, as a method to preserve bowel length. Romao et al. when appropriate, without the need for enterostomy and stent.
recently reported a review of nine patients who received intestinal
stents [7]. Five of these nine patients received proximal jejunos- Conflict of interest statement
tomies with mucous fistula, and the stent entry point was the No authors have any disclosures.
mucous fistula. The others had the stent placed through a stab
wound to the abdominal wall and enterostomy in the proximal References
bowel. Four complications developed, including two anastomotic
leaks and two strictures, all of which required surgical intervention. [1] Martin LW, Zerella JT. Jejunoileal atresia: a proposed classification. J Pediatr Surg
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with apple peel syndrome: successful treatment by five end-to-end anasto-
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[6] Festen S, Brevoord JC, Goldhoorn GA, Festen C, Hazebroek FW, van Heurn LW,
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