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CASE REPORT Table of Contents   
Year : 2011  |  Volume : 8  |  Issue : 2  |  Page : 235-236
Apple-peel atresia presenting as foetal intestinal obstruction


1 Department of Surgery, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India
2 Department of Pathology, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India

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Date of Web Publication14-Oct-2011
 

   Abstract 

Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed.

Keywords: Apple-peel atresia, prenatal ultrasonography

How to cite this article:
Kshirsagar AY, Desai SR, Nagvekar MA, Vasisth GO. Apple-peel atresia presenting as foetal intestinal obstruction. Afr J Paediatr Surg 2011;8:235-6

How to cite this URL:
Kshirsagar AY, Desai SR, Nagvekar MA, Vasisth GO. Apple-peel atresia presenting as foetal intestinal obstruction. Afr J Paediatr Surg [serial online] 2011 [cited 2019 Sep 20];8:235-6. Available from: http://www.afrjpaedsurg.org/text.asp?2011/8/2/235/86072

   Introduction Top


Type 3 jejuno-ileal atresia (JIA), also known as apple peel atresia (APA), is a very rare entity with an incidence of 1 in 1000 live births. In this, blind ends of bowel are completely separated by a V-shaped mesenteric defect with loss of normal blood supply to distal bowel. Diagnosis of atresia as intestinal obstruction on prenatal ultrasonography is also difficult. We are discussing a case with Type 3 JIA, presenting as foetal intestinal obstruction on prenatal ultrasonography and surgically corrected on the first day of birth with interesting findings.


   Case Report Top


A 20-year-old primigravida was on routine antenatal check up. Prenatal ultrasonography at 30 th week of gestation showed single live foetus in vertex position with evidence of dilated lower gastrointestinal tract with grade 1 placenta previa without any other anomaly [Figure 1]. This was suggestive of distal small bowel obstruction.
Figure 1: Dilated bowel loops seen in prenatal ultrasonography

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A preterm vaginal delivery was conducted and male child was born with normal placenta except excessive torsion of umbilical cord. Child cried immediately after birth. It was weighing 2.2 kg with APGAR score of 7 at 1 minute. Though the infant passed meconium right after birth, he suffered from bilious vomiting and abdominal distension after 3 hours. As it was progressively aggravating, emergency laparotomy was performed under impression of bowel obstruction on the day of birth [Figure 2].
Figure 2: 1, Normal ileal loops; 2, V-shaped mesenteric defect; 3, gangrenous ileal loops

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Surgical exploration revealed multiple, gangrenous, atretic, completely separated segments of distal jejunum and ileum with distended proximal jejunum and collapsed colon. Mesenteric defects were observed as separated segments with blackening. Surgical resection of gangrenous and atretic segments was performed with jejuno-ascending colon end-to-end anastomosis. Histopathology revealed gangrene of the part of jejunum and ileum with atretic loops.

During postoperative period, the child was maintained on parenteral nutrition .On the fifth postoperative day, the child became breathless and X-ray showed severe bronchopneumonia. In spite of intravenous fluids and antibiotics, the condition worsened and the child expired on the eighth postoperative day.


   Discussion Top


Small bowel atresia is one of the most common surgical causes of neonatal intestinal obstruction, with an incidence of 1 in 1000 live births. [1] Antenatal diagnosis is suggested by visualisation of fluid-filled loops of bowel on ultrasound, and it is possible in 50% of cases. [2]

JIA is generally considered to result from intrauterine vascular disruptions to segment of developed intestine. [3],[4],[5] Disruptive events such as volvulus and intussusception have been observed at surgery in patients with JIA. [4],[5] However, APA is unlikely to be caused by such local mechanical obstruction. APA is considered to result from superior mesenteric artery (SMA) occlusion in earlier stage of gestation for unknown reason. [6],[7],[8]

In APA, it is speculated that vascular occlusions occur in the SMA distal to the origin of the right colic or the ileocolic artery, in the earlier stage of gestation. [6],[7],[8] Ischaemic necrosis of the distal SMA and mesentery follows. The intestine elongates more readily than its artery, resulting in the helical configuration of ileum.

SMA occlusion takes place after the 10 th embryonic week when all of the bowel returns from yolk sac and completes its rotation because APA is not associated with malrotation, omphalocoele. [8] But the exact mechanism of occlusion remains unknown. [8]

The potential benefits of antenatal diagnosis include earlier recognition and parental counselling, which helps in prompt intervention and reducing metabolic complications. [2],[7],[9]

The reduction in mortality in infants with isolated JIA is most likely due to concurrent improvements in various aspects of neonatal care, improved nutrition and improved surgical techniques to preserve bowel length in cases of foreshortened gut.

 
   References Top

1.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. 1
    
2.Stoll C, Alembik Y, Dott B, Roth MP. Evaluation of prenatal diagnosis of congenital gastrointestinal atresia. Eur J Epidemiol 1996;12:611-6.  Back to cited text no. 2
[PUBMED]    
3.Louw JH, Barnard CN. Congenital intestinal atresia: Observations on its origin. Lancet 1955;269:1065-7.  Back to cited text no. 3
[PUBMED]    
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
[PUBMED]    
5.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 Surg 1998;133:490-6.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Zerella JT, Martin LW. Jejunal atresia with absent mesentery and a helical ileum. Surgery 1976;80:550-3.  Back to cited text no. 6
[PUBMED]    
7.Ros Mar Z, Diez Pardo JA, Ros Miquel M, Benavent I. Apple peel small bowel. A review of twelve cases. Z Kinderchir Grenzgeb 1980;29:313-7.  Back to cited text no. 7
[PUBMED]    
8.Manning C, Strauss A, Gyepes MT. Jejunal atresia with apple peel deformity. A report of 8 survivors. J Perinatol 1989;9:281-6.  Back to cited text no. 8
[PUBMED]    
9.Beath SV, Booth IW, Murphy MS, Buckels JA, Mayer AD, McKiernan PJ, et al. Nutritional care and candidates for small bowel transplantation. Arch Dis Child 1995;73:348-50.  Back to cited text no. 9
[PUBMED]  [FULLTEXT]  

Top
Correspondence Address:
Ashok Yadavrao Kshirsagar
Krishna Institute of Medical Sciences University, Malkapur, Karad - 415 110, Dist. Satara, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.86072

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    Figures

  [Figure 1], [Figure 2]

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