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CASE REPORT
Year : 2012  |  Volume : 39  |  Issue : 1  |  Page : 29-31

Apple peel jejunal atresia: Successful management of a rare case


1 Department of Pediatric Surgery, J.N.M.C and KLES Dr. Prabhakar Kore Hospital and MRC, Belgaum, India
2 Department of Obstetrics and Gynecology, J.N.M.C and KLES Dr. Prabhakar Kore Hospital and MRC, Belgaum, India
3 Department of Pediatrics, J.N.M.C and KLES Dr. Prabhakar Kore Hospital and MRC, Belgaum, India
4 Department of Anesthesia, J.N.M.C and KLES Dr. Prabhakar Kore Hospital and MRC, Belgaum, India

Date of Web Publication21-May-2012

Correspondence Address:
Santosh B Kurbet
Associate Professor, J.N.M.C and KLES Dr. Prabhakar Kore Hospital and MRC, Belgaum
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-5009.96470

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  Abstract 

Apple peel jejunal atresia is a rare congenital anomaly. This condition presents at birth as an intestinal obstruction, is diagnosed by X-rays and confirmed by laparatomy. Resection of the dilated proximal intestine and primary anastomosis is the treatment, but has a poor prognosis. We report here a rare form of atresia with apple peel deformity and its successful management.

Keywords: Apple peel deformity, jejunoileal atresia, primary anastomosis


How to cite this article:
Kurbet SB, Patil H S, Dhaded S M, Bhandankar M R, Mane R S. Apple peel jejunal atresia: Successful management of a rare case. J Sci Soc 2012;39:29-31

How to cite this URL:
Kurbet SB, Patil H S, Dhaded S M, Bhandankar M R, Mane R S. Apple peel jejunal atresia: Successful management of a rare case. J Sci Soc [serial online] 2012 [cited 2017 Mar 25];39:29-31. Available from: http://www.jscisociety.com/text.asp?2012/39/1/29/96470


  Introduction Top


Apple peel jejunal atresia is a rare condition, with multiple associated anomalies. It is characterized by atresia and wrapping of the distal small intestine in a spiral pattern around a thin vascular pedicle. [1] These babies present with neonatal intestinal obstruction and are treated by resection and primary anastomosis. The prognosis is usually not good, except in a few cases. Newborns surviving surgery and complications do well, as in our case.


  Case Report Top


A 34-week preterm baby, weighing 2.1 kg, presented on its third day of life, with, failure to pass meconium, abdominal distention, and bilious vomiting. Antenatal ultrasonography had shown mild polyhydramnios and dilated bowel. The baby on examination had a poor general condition, poor cry and activity, had massive abdominal distention, [Figure 1] and sluggish bowel sounds. Investigations revealed hemoglobin of 20.9 gm%, total counts of 5,100 cells / cmm, normal differential count, C-reactive protein (CRP) was positive, creatinine of 0.4 mg%, sodium of 116 mEq/L, chloride of 98 mEq/L, and potassium of 7 mEq/L. A plain X-ray abdomen showed dilated stomach and proximal intestine. After correction of fluids and electrolytes, the neonate was taken for exploratory laparatomy under general anesthesia. The intraoperative findings were proximal jejunal atresia with a massively dilated proximal jejunum and a small caliber ileum along with a microcolon [Figure 2]. The proximal, massively dilated jejunum was partially resected and end-to-oblique anastomosis was done in a single extramucosal layer. [Figure 3]. The postoperative period was uneventful and the baby was started on partial parenteral nutrition, enteral feeds by the end of a week, and full feeds were established by two weeks. The baby was discharged a day later and was doing well at the follow-up of three months [Figure 4].
Figure 1: Abdominal distention

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Figure 2: Apple peel jejunal atresia

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Figure 3: Primary jejunoileal anastomosis

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Figure 4: At three-month follow-up

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  Discussion Top


Intestinal atresia is one of the major causes of neonatal intestinal obstruction. Jejunal atresia with an apple peel deformity is one of the rare and most severe forms of intestinal atresia. Apple peel atresia (APA) is a serious congenital anomaly that affects approximately 1: 5000 live births. [1],[2] The apple peel type constitutes 5 - 10% of all small bowel atresia and was first reported by Santulli and Blanc, in 1961. APA can be familial, and an autosomal recessive inheritance has been reported. Associated anomalies include malrotation, prematurity, and low birth weight. [1] A mesenteric ischemic insult to the developing midgut during fetal development is widely accepted to result in jejunoileal atresia, as described by Louw and Barnard from their experimental studies. [3] The accepted classification is the Grosfeld modified Louw classification, with type I having mucosal atresia, type II having two atretic ends connected by a fibrous band, type III having two atretic ends separated by mesenteric defect, with subtype (a) having only a defect and subtype (b) having apple peel atresia, and type IV having multiple atresias. [1] Antenatal ultrasonography and magnetic resonance imaging (MRI) can predict intestinal atresia by the presence of dilated bowel and polyhydramnios.

At birth these newborns present with features of intestinal obstruction. An X-ray of the abdomen usually shows dilated bowel with gas shadows up to the level of atresia. Many operative techniques have been described for the correction of intestinal atresia, such as, resection with tapering enterostomy and anastomosis, resection with enterostomy or only enterostomy, which can be done by Mikulicz double barrel, Santulli, or Bishop-Koop enterostomy, and commonly by primary resection, with end-to-oblique anastomosis. The current surgery performed is, excision of the dilated proximal intestine and primary anastomosis. [1],[4],[5] Morbidity and mortality is due to the associated anomalies, anastomotic leak, functional obstruction, sepsis, prematurity, respiratory complications, short bowel syndrome, and malnutrition. [1],[4] Until lately, neonatal mortality rates with apple peel atresia were very high. [4],[6] If these newborns survived the operative and postoperative periods and the morbidity associated with malnutrition and TPN, then they developed normal bowel function and normal growth and development. Now with antenatal prediction, early postnatal diagnosis, appropriate surgery, and parenteral nutrition have led to better results and long-term outcome in these patients. [5],[7]


  Conclusion Top


Apple peel deformity is a rare form of intestinal atresia with poor results, due to the presence of high jejunal atresia, prematurity, low birth weight, associated anomalies, and postoperative complications. This condition, if detected antenatally, shifted to a tertiary care center, postnatally investigated to confirm it, and operated upon, may yield excellent results, as in our case.

 
  References Top

1.Grosfeld JL. Jejunoileal Atresia and Stenosis. In: Grosfeld J, O'Neil J Jr, Coran AG, editors. Pediatric Surgery. 6 th ed., Vol 2. Philadelphia: Mosby Elsevier; 2006. p. 1269-87.  Back to cited text no. 1
    
2.Millar AJ, Rode H, Cywes S. Intestinal atresia and stenosis. In: Ashcraft KW, Murphy JP, Sharp RJ, editors. Pediatric Surgery. 3 rd ed. Philadelphia: Saunders; 2000. p. 406-24.  Back to cited text no. 2
    
3.Louw JH, Barnard CN. Congenital intestinal atresia: Observations on its origin. Lancet 1955;269:1065-7.  Back to cited text no. 3
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4.Nixon HH, Tawes R. Etiology and treatment of small intestinal atresia: Analysis of series of 127 Jejunoileal atresias and comparison with 62 duodenal atresia surgery. Surgery 1971;69:41-51.  Back to cited text no. 4
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5.DallaVecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: A 25 year experience with 277 cases. Arch Surg 1998;133:490-6.  Back to cited text no. 5
    
6.Grosfeld JL. Alimentary tract obstruction in the newborn. Curr Probl Pediatr 1975;5:3-47.  Back to cited text no. 6
    
7.Festen S, Brevoord JC, Goldhoorn GA, Festen C, Hazebroek FW, van Heurn LW, et al. Excellent long-term outcome for survivors of apple peel atresia. J Pediatr Surg 2002;37:61-5.  Back to cited text no. 7
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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