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Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 66-69

Multiple jejunoileal and colonic atresias: Case report and review of literature

Department of Pediatric Surgery, Maternity and Children's Hospital, Najran, Saudi Arabia

Date of Web Publication12-Sep-2014

Correspondence Address:
R Rajendran
Department of Pediatric Surgery, Maternity and Children's Hospital, P.O. Box 3600, Najran
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-3846.140697

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Multiple intestinal atresia (MIA) associated with colonic atresia is very rare, especially with almost normal bowel length. This report deals with a case of multiple jejunoileal atresia associated with descending colon atresia, which was managed by multiple resection and primary anastomoses without use of stents and with total parenteral nutrition. Literature on the genesis, management and survival of MIA is reviewed.

Keywords: Five intestinal atresias, jejunoileal and colonic atresias, multiple intestinal anastomoses, tapering duodenojejunoplasty

How to cite this article:
Rajendran R, Hegab SM, Elsalam HA, Mukttash G. Multiple jejunoileal and colonic atresias: Case report and review of literature . Saudi Surg J 2014;2:66-9

How to cite this URL:
Rajendran R, Hegab SM, Elsalam HA, Mukttash G. Multiple jejunoileal and colonic atresias: Case report and review of literature . Saudi Surg J [serial online] 2014 [cited 2019 Aug 25];2:66-9. Available from: http://www.saudisurgj.org/text.asp?2014/2/2/66/140697

  Introduction Top

Prevalence of jejunoileal atresias (JIA) ranges from 1.3-2.9/10,000 live births [1] while colonic atresias (CA) are rarer (1 in 20,000). [2] Intestinal atresias are generally single event (>90%); multiple atresias can occur (6-20%) and most often involve the proximal jejunum. [1] According to Martin classification, [1] multiple intestinal atresias (MIA) are Type IV. They are associated with foreshortened intestines, prematurity and high mortality. [1] Association of JIA with CA is rare. Only 15-20% of infants with CA have proximal small intestinal atresia. [1] Only 20% of CA are seen in descending and sigmoid colon. [1] This report deals with a case of multiple JIA associated with descending colon atresia.

  Case Report Top

A preterm baby girl (34 weeks), with low birth weight (LBW) (2040 g), was born of normal spontaneous vaginal delivery to a 21 year-old mother, who was G5P4. The mother was suffering from polyhyramnios.

The baby was admitted with mild respiratory distress, and unconjugated hyperbilirubinemia. She started bilious vomiting on 3 rd day of birth. She did not pass meconium. The child had upper abdominal fullness. Plain and upper gastrointestinal (GI) contrast X-ray examinations revealed dilated stomach, duodenum and upper jejunum with complete obstruction of upper jejunum [Figure 1]. Complete investigations were carried out. Cranial ultrasound scan (USS) revealed mildly dilated lateral ventricles.
Figure 1: Plain abdominal skiagram and upper gastrointestinal contrast X-ray pictures showing dilated stomach, duodenum and upper jejunum with complete obstruction of upper jejunum

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At laparotomy, duodenum and upper 10cm of jejunum were dilated and hypertrophied to 6 cm diameter due to type I jejunal atresia, 10 cm distal to the ligament of Trietz [Figure 2]. In addition, three ileal atresias and one CA also were found. These were type I atresia of proximal ileum (about 40cm away from the 1 st atresia), type III atresia of midileum (about 20 cm away from the 2 nd atresia), type I atresia of distal ileum (15 cm away from 3 rd atresia and 20 cm proximal to the ileocaecal junction), and type I atresia of lowermost part of descending colon at the beginning of sigmoid colon. Small and large intestinal dispositions were normal. Excision of jejunal atresia with 5cm of dilated jejunum, tapering duodenojejunoplasty (by excision) of 3 rd and 4 th parts of duodenum and upper jejunum and end-to-side jejunojejunal anastomosis were done. For all other 4 atresias, resection and end-to-oblique anastomoses were done. After operation, the small intestinal length was about 100cm which is normally acceptable for this preterm. All excised intestines were subjected to histopathologic examination (HPE). Postoperatively, Total Parenteral Nutrition (TPN) was given. Child passed stool on 9 th postoperative day. Feeding was started on 14 th postoperative day. Contrast G.I follow through x-rays demonstrated easy passage of contrast upto rectum [Figure 3]. On 20 th postoperative day, TPN was discontinued. Body weight increased steadily, reached 1850 g on 26 th post-operative day, and then the child was discharged from the hospital. The child spent 30 days in the hospital. On the follow-up at the age of 80 days, the weight was 3000 g, and had mild jaundice due to conjugated hyperbilirubinemia, with elevated liver enzyme levels and negative HBSAg and anti-hepatitis C virus levels (which excluded viral hepatitis). USS of abdomen showed normal sized liver with increased parenchymal echogenicity, contracted gall bladder and no intrahepatic biliary dilatation. At the age of 5months, the weight was 4800 g, and had no jaundice. Liver function test results showed near-normal values.
Figure 2: Operative picture showing enormously dilated proximal jejunum due to Type I jejuna atresia, proximal ileal atresia and distal ileal atresia (shown by tips of hemostats)

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Figure 3: Contrast gastrointestinal follow through X-ray pictures showing easy passage of contrast through jejunum, ileum and colon up to rectum in 2 h. Pictures also show presence of good length of intestines

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  Discussion Top

Jejunoileal atresias are the most common congenital anomalies of small intestine and are major causes of neonatal intestinal obstruction. CA is the rarest atresia of the gastrointestinal tract (GIT) and accounts for 1.8-15% of the bowel atresias [2] . Intestinal atresias are generally single event (>90%); MIA occur in ≤10% cases. According to Martin classification [1] , MIA are Type IV. ≥50% of those with multiple atresias were infants with LBW. [1]

The causes of MIA are many. [1] One of them is multiple ischemic infarctions, as suggested by Louw and Barnard (1955). Other causes include abnormal development of the notochord in the area of developing midgut, placental vascular anomalies such as marginal insertion of umbilical cord, congenital and acquired immuno-deficiency, heredity and maternal use of pseudoephedrine, ergotamine tartrate, caffeine (cafergot), cocaine, and nicotine. Based on pathologic findings, it is suggested that all cases of hereditary MIA and some cases of nonhereditary MIA are consequences of malformative process of GIT rather than ischaemic process. TTC7A is 34 aminoacid sequence containing-domain protein. Samuels et al. (2013) [3] concluded that deletion or mutation in TTC7A gene was causal to MIA. Absence of embryonic expression of fibroblast growth factor 10 or its receptor resulted in colonic atresia. [2]

Intraoperatively, meticulous inspection of whole small and large intestine, after injection of normal saline into proximal bowel, helps to detect atresias at different levels, as in the present case. In the treatment of MIA, the aim is to preserve as much bowel length as possible, by any means such as enteroplasty, multiple resection, and anastomoses. Up to 7 anastomoses have been advocated. [4],[5] The use of a silicon tube as stent in multiple anastomoses were reported by different authors. [6],[7],[8] In our patient, to preserve maximum bowel length, 5 resection and anastomoses were done - 4 in a small bowel and 1 in a large bowel. To bring back peristalsis early in the severely dilated and hypertrophied duodenum and jejunum, tapering duodenojejunoplasty and primary jejunojejunostomy were done. Since there was sufficient distance between anastomoses and we had no so long transanastomotic tube as to reach beyond the distal ileal anastomosis, no tube was put. There was no indication for enterostomy as compromised vascularity, peritonitis or poor general condition. Resection and primary end-to end anastomosis of descending colon atresia, which we did, also agrees with the current practice. [2] Hirschsprung's disease, associated with at least 2% of patients with CA should be excluded typically by rectal suction biopsy. [2] In this case, HPE of the resected colon distal to the atresia ruled out aganglionosis.

Only postoperative morbidity in this case was conjugated hyperbilirubinemia, a possible complication of TPN plus prematurity. In order to reduce Parenteral Nutrition Associated Liver Disease (PNALD) and to prevent sepsis, we started feeding at the earliest possible, and weaned from TPN after 16 days. Soybean-based intravenous fat emulsions are causative agents of PNALD. Reducing these fats or replacing them with a fish-oil-based intravenous fat has been proven successful in reversing PNALD. [9]

The survival of MIA patients in different centers varies. Chadha et al. [5] and Ekwunife et al. [10] reported 67% survival while Khalaf et al. [11] have reported no survival without giving TPN. Dalla Vecchia et al. [12] reported 31 (24%) cases of MIA out of 128 cases of JIA over 25-year period, with no mortality. Late deaths in 16 cases (51.6%) among them were due to sepsis and multisystem organ failure. Baglaj et al. [13] and Romao et al. [8] have reported 100% survival whereas Calisti et al. [14] have reported 92% survival with TPN. The latter authors observed that the dependency on TPN was significantly correlated to the length of residual bowel. It is clear from the above review that use of TPN also is essential for 100% survival in MIA.

  Conclusion Top

A very rare combination of jejunal atresia, three ileal atresias and descending colon atresia is presented. In the presence of an atresia, vigilant per-operative search of whole intestines for other atresias must be done. Maximum preservation of intestinal length by enteroplasty and multiple anastomoses was done. If the gaps between multiple atresias are not very short, intestinal stenting is not needed.

  Acknowledgments Top

The authors thank Dr. Amanulla Mugheri, consultant, and Dr. Rayan Baz, specialist, Department of Pediatric Surgery, MCH, Najran, for their help in editing the manuscript of this article. We also thank Mr. Shafeeq C.P., lecturer in English, Najran University, Kingdom of Saudi Arabia, for language-editing of this article.

  References Top

1.Frischer JS, Azizkhan RG. Jejunoileal atresia and stenosis. In: Coran AG, editor-in-chief, Adzick NS, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, associate editors. Pediatric Surgery. 7 th ed., Vol. II, Ch. 82. USA: Elsevier, Saunders; 2012. p. 1059-71.  Back to cited text no. 1
2.Arca MJ, Oldham KT. Atresia, stenosis and other obstructions of the colon. In: Coran AG, editor-in-chief, Adzick NS, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, associate editors. Pediatric Surgery. 7 th ed., Vol. II, Ch. 99. USA: Elsevier, Saunders; 2012. p. 1247-53.  Back to cited text no. 2
3.Samuels ME, Majewski J, Alirezaie N, Fernandez I, Casals F, Patey N, et al. Exome sequencing identifies mutations in the gene TTC7A in French-Canadian cases with hereditary multiple intestinal atresia. J Med Genet 2013;50:324-9.  Back to cited text no. 3
4.Sato S, Nishijima E, Muraji T, Tsugawa C, Kimura K. Jejunoileal atresia: A 27-year experience. J Pediatr Surg 1998;33:1633-5.  Back to cited text no. 4
5.Chadha R, Sharma A, Roychoudhury S, Bagga D. Treatment strategies in the management of jejunoileal and colonic atresia. J Indian Assoc Pediatr Surg 2006;11:79-84.  Back to cited text no. 5
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6.Federici S, Domenichelli V, Antonellini C, Dòmini R. Multiple intestinal atresia with apple peel syndrome: Successful treatment by five end-to-end anastomoses, jejunostomy, and transanastomotic silicone stent. J Pediatr Surg 2003;38:1250-2.  Back to cited text no. 6
7.Hyseni N, Statovci S, Llullaku S, Rashiti I, Berisha M, Qeku G, et al. Successful treatment of multiple jejunoileal atresia by four primary anastomosis and transanastomotic silastic stents. JK Sci 2009;11:136-8. Available from: http://www.jkscience.org.  Back to cited text no. 7
8.Romão RL, Ozgediz D, de Silva N, Chiu P, Langer J, Wales PW. Preserving bowel length with a transluminal stent in neonates with multiple intestinal anastomoses: A case series and review of the literature. J Pediatr Surg 2011;46:1368-72.  Back to cited text no. 8
9.Teitelbaum DH, Btaiche IF, Coran AG. Nutritional support in the pediatric surgical patient. In: Coran AG, editor-in-chief, Adzick NS, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, associate editors. Pediatric Surgery 7 th ed., Vol. II, Ch. 12. USA: Elsevier, Saunders; 2012. p. 179-99.  Back to cited text no. 9
10.Ekwunife OH, Oguejiofor IC, Modekwe VI, Osuigwe AN. Jejuno-ileal atresia: A 2-year preliminary study on presentation and outcome. Niger J Clin Pract 2012;15:354-7.  Back to cited text no. 10
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11.Khalaf AA, Al Obaidy MA, Khalaf KF. Jejunoileal Atresia: A study of 60 cases in children welfare teaching hospital. Fac Med Baghdad 2010;52.  Back to cited text no. 11
12.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. 12
13.Baglaj M, Carachi R, Lawther S. Multiple atresia of the small intestine: A 20-year review. Eur J Pediatr Surg 2008;18:13-8.  Back to cited text no. 13
14.Calisti A, Olivieri C, Coletta R, Briganti V, Oriolo L, Giannino G. Jejunoileal Atresia: Factors Affecting the Outcome and Long-term Sequelae. J Clin Neonatol 2012;1:38-41.  Back to cited text no. 14
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