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CASE REPORT Table of Contents   
Year : 2014  |  Volume : 11  |  Issue : 1  |  Page : 77-78
Ileocecal valve atresia: Introduction of a new surgical approach

1 Department of Pediatric Surgery, Mazandaran University of Medical Sciences, Sari, Iran
2 Clinical Research Development Unit of Booali Sina Hospital, Mazandaran University of Medical Sciences, Sari, Iran

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Date of Web Publication20-Mar-2014


Ileocecal valve atresia is a very rare anomaly with only a few cases reported in the literature. The conventional therapy includes ileocecal resection and ileocolic anastomosis. Here, we present a patient with inborn ileocecal valve atresia in whom we successfully preserved the ileocecal valve and the terminal ileum through opening a valve and tapering the ileum in one operation.

Keywords: Atresia, ileocecal, valve

How to cite this article:
Mousavi SA, Sarparast L. Ileocecal valve atresia: Introduction of a new surgical approach. Afr J Paediatr Surg 2014;11:77-8

How to cite this URL:
Mousavi SA, Sarparast L. Ileocecal valve atresia: Introduction of a new surgical approach. Afr J Paediatr Surg [serial online] 2014 [cited 2023 Feb 9];11:77-8. Available from:

   Introduction Top

Atresia of the ileocecal region and valve is a very rare anomaly, for which we found only five cases in the English literature [1-3]. It is noteworthy that the terminal ileum constitutes a physiologically unique region, and preserving it is of utmost importance. The classical approach to patients with ileocecal valve atresia is to eliminate the entire region and construct an ileocolic anastomosis anew. In this paper, we attempt to introduce a new approach for correcting ileocecal valve atresia.

   Case Report Top

A 1-day old male neonate weighing 3 kg was referred with abdominal distention and biliary vomiting. The family history of the patient yielded no problem and the boy was born through normal vaginal delivery. Abdominal X-ray revealed severe distention of bowel loops, indicating bowel obstruction. Barium enema visualized microcolon. The patient underwent surgery with the diagnosis of terminal ileum obstruction. During laparatomy, we found an intensely distended terminal ileum. The ileocolic atresia consisted of a mucosal (membranous) atresia with intact bowel wall and mesentery, consistent with type 1 atresia [Figure 1]a. [1] We first performed enterotomy of the distended region, followed by opening the mucosal membrane between the ileum and cecum, and finally tapering enteroplasty of the distended ileal segment with appendectomy [Figure 1]b. Nutrition was resumed on the fifth day after surgery. Although the patient suffered from surgical site infection, he was discharged from the hospital in excellent condition on the 12 th day after surgery. Eighteen months following the operation, the patient is still in excellent condition and has no problems.
Figure 1: (a) Ileocecal atresia with microcolon and ileal dilatation, (b) Repair of atresia with tapering of ileum and mucosectomy plus appendectomy

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   Discussion Top

As demonstrated by Quigley et al., the ileocecal junction is a true sphincter with accumulation of annular muscles. In response to bowel distention, pharmacological agents and nervous stimulation, this region constricts, distinguishing it from other parts of the intestines. On the other hand, observations in animal and human models have revealed that this sphincter causes the chime to enter the colon in small amounts. Therefore, preserving this sphincter will reduce complications in cases where extensive resection of the small intestine in indicated. Another point worthy of attention is the fact that hindering the reflux of colonic content into the ileum will prevent microbial colonization in terminal ileum. [2],[3]

Folaranmi et al., studied 99 patients with Crohn's disease to conclude that removing the ileocecal valve will lead to chronic diarrhea in 26.5% of cases. [3] On the contrary, Iwanaka et al., followed up five neonates with terminal ileum resection and reported that removing this region is well tolerated in patients and entails few complications. [4]

Ein et al., encountered ileocecal atresia in two patients who had undergone surgery for meconium ileus, and adopted ileocolic resection as the appropriate therapy. [5] In addition, Cserni et al. and Srivastava et al., performed resection of the distended ileum and right colon and ileocolic anastomosis in two newborns with ileocecal valve atresia and claimed that no complications occurred. [6],[7]

Our patient had type 1 atresia, which is similar to cases studied by Cacciari et al., Ein et al., and one of the patients reported by Srivastava et al.[5],[6],[8] Ours, however, is the only study where preservation of the valve and the ileocecal region was attempted. Although Cacciari et al. used ileocecal valve reconstruction for the first time in a neonate with ileum perforation; this was performed 4 months after the initial surgery (ileostomy). [8]

In conclusion, considering the crucial nature of the ileocecal valve and especially the terminal ileum, we believe that all efforts must be made to preserve the region. For this purpose, enterotomy, opening the obstructing mucosa and ileoplasty may provide a treatment option.

   References Top

1.Holcomb III GW, Murphy JP, editors. Ashcraft's pediatric surgery. 5 th ed. Philadelphia: Saunders, 2010. p. 405-15.  Back to cited text no. 1
2.Quigley EM, Phillips SF. The ileocecal (ileocolonic) sphincter. Z Gastroenterol 1983;21:47-55.  Back to cited text no. 2
3.Folaranmi S, Rakoczy G, Bruce J, Humphrey G, Bowen J, Morabito A, et al. Ileocaecal valve: how important is it? Pediatr Surg Int 2011;27:613-5.   Back to cited text no. 3
4.Iwanaka T, Tsuchida Y, Honna T, Makino S, Saito S. Ileocecal resection in neonates--a follow-up study. Jpn J Surg 1984;14:311-4.   Back to cited text no. 4
5.Ein SH, Venugopal S, Mancer K. Ileocaecal atresia. J Pediatr Surg 1985;20:525-8.  Back to cited text no. 5
6.Srivastava P, Gangopadhyay AN, Gupta DK, Sharma SP, Upadhyaya VD, Kumar V, et al. Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia. J Indian Assoc Pediatr Surg 2009;14:117-8.   Back to cited text no. 6
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7.Cserni T, Magyar A, Németh T, Paran TS, Csízy I, Józsa T. Atresia of the ileocecal junction with agenesis of the ileocecal valve and vermiform appendix: Report of a case. Surg Today 2006;36:1126-8.  Back to cited text no. 7
8.Cacciari A, Mordenti M, Ceccarelli PL, Ruggeri G. Ileocaecal valve atresia: Our surgical approach. Eur J Pediatr Surg 2004;14:435-9.  Back to cited text no. 8

Correspondence Address:
Seyed Abdollah Mousavi
Department of Pediatric Surgery, Booali Sina Hospital, Pasdaran Boulevard, Sari 4817846859
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-6725.129242

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