Multiple Atresia of the Small Intestine: A 20-Year Review

Abstract

Background: Multiple intestinal atresia (MIA) presents with a wide spectrum of bowel pathologies. Its treatment is a challenging task since restoration of anatomical continuity of the affected intestine must be balanced against preservation of the intestine's maximal length. Material and Methods: A retrospective analysis of the medical notes of 26 patients with MIA treated over a 20-year period between 1986 - 2006 was undertaken with a special emphasis on the clinical and surgical perspectives. Results: All 26 cases of MIA were sporadic with no familial history. The mean gestational age and birth weight were 36.1 weeks and 2781 g, respectively. Twenty-three of the infants underwent operative repair within the first days of life. Three patients with gastroschisis had a delayed diagnosis of bowel atresia. The number of atresias per patient ranged from 2 to 10. In 24 newborns atresias were confined to the small bowel, with 2 other patients having additional obstruction of the ascending colon. Various combinations of anatomical types of atresias were found, with type I and type III occurring in 19 patients each. Type II was diagnosed in 7 newborns. Surgical management of MIA consisted of one-stage restoration of bowel continuity with multiple anastomoses and/or enteroplasties in 22 patients. Four patients had an enterostomy performed at initial operation. Early and late postoperative complications requiring operative treatment occurred in 8 patients. The duration of parenteral nutrition ranged from 6 days to 20 months, exceeding 100 days in 6 children. The follow-up ranges from 3 months to 16 years. All the patients are alive and are on a full oral diet. Conclusion: Clinical observations of sporadic cases of MIA confined to the small bowel lend support to the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with preservation of maximal intestinal length should be the basic principle of any operative management of MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these two entities should be classified separately in a modified classification of intestinal atresia.

Key words

multiple intestinal atresia - classification - small intestine - newborn (neonates)

References

• 1 Bilodeau A, Prasil P, Clouties R. et al . Hereditary multiple intestinal atresia: thirty years later.  J Pediatr Surg. 2004;  39 726-730
• 2 Chaet M, Warner B, Sheldon C. Management of multiple intestinal atresias with an intraluminal silastic stent.  J Pediatr Surg. 1994;  29 1604-1606
• 3 Della Vecchia L, Grosfeld J, West K. et al . Intestinal atresia and stenosis: a 25-year experience with 277 cases.  Arch Surg. 1998;  133 490-496
• 4 DeLorimier A, Fonkalsrud A, Hays D. Congenital atresia and stenosis of the jejunum and ileum.  Surgery. 1969;  65 819-827
• 5 El Shafie M, Rickham P. Multiple intestinal atresia.  J Pediatr Surg. 1970;  5 655-659
• 6 Federici S, Domenichelli V, Antonellini C. et al . 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 1250-1252
• 7 Fourcade L, Shima H, Miyazaki E, Puri P. Multiple gastrointestinal atresias result from disturbed morphogenesis.  Pediatr Surg Int. 2001;  17 361-364
• 8 Grosfeld J, Ballantine T, Shoemaker R. Operative management of intestinal atresia and stenosis based on pathologic findings.  J Pediatr Surg. 1979;  14 368-375
• 9 Guala A, Licardi G, Maghnie M. et al . A case of multiple intestinal atresias, brain anomalies, mental retardation, growth hormone deficiency and clitoris hypertrophy.  Clin Dysmorph. 1998;  7 209-211
• 10 Guttmann F, Braun P, Garance P. et al . Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum.  J Pediatr Surg. 1973;  8 633-640
• 11 Hasegawa T, Sumimura J, Nowe K. et al . Congenital multiple intestinal atresia successfully treated with multiple anastomoses in a premature neonate; report of a case.  Jpn J Surg. 1996;  26 849-851
• 12 Hatch E, Schaller R. Surgical management of multiple intestinal atresia.  Am J Surg. 1986;  151 550-552
• 13 Kimura K, Tsugawa C, Ogawa K. et al . Multiple intestinal atresia: successful reconstruction by six end-to-end anastomoses.  J Pediatr Surg. 1981;  21 200-201
• 14 Komuro H, Amagai T, Hori T, Hirai T. et al . Placental vascular compromise in jejunoileal atresia.  J Pediatr Surg. 2004;  39 1701-1705
• 15 Lane G, Yamataka A, Kato Y. et al . Multiple bowel atresias after syngenic fetal small bowel transplantation in rats.  J Pediatr Surg. 1998;  33 896-898
• 16 Louw J, Barnard C. Congenital intestinal atresia. Observation on its origin.  Lancet. 1955;  2 1065-1067
• 17 Merei J. Notochord-gut failure of detachment and intestinal atresia.  Pediatr Surg Int. 2004;  20 439-443
• 18 Moreno L, Gottrand F, Turck D. et al . Severe combined immunodeficiency syndrome associated with autosomal recessive familial gastrointestinal atresia. Study of a family.  Am J Med Genet. 1990;  37 143-146
• 19 Prasad S, Mitra D, Bhatnagar V. et al . Multiple atresias of the bowel with reference to Tandler's theory of embryopathogenesis.  Ind Pediatr. 1993;  30 1036-1039
• 20 Puri P, Fujimoto T. New observations of pathogenesis of multiple intestinal atresias.  J Pediatr Surg. 1984;  23 221-225
• 21 Puri P, Guiney E, Carroll R. Multiple gastrointestinal atresias in three consecutive siblings: observations on pathogenesis.  J Pediatr Surg. 1985;  20 22-24
• 22 Sato S, Nijishima E, Muraji T. et al . Jejunoileal atresia: a 27-year experience.  J Pediatr Surg. 1998;  33 1633-1635
• 23 Teji P, Schnatterly P, Shaw A. Multiple intestinal atresias: pathology and pathogenesis.  J Pediatr Surg. 1981;  16 194-199
• 24 Touloukian R. Diagnosis and treatment of jejunoileal atresia.  World J Surg. 1993;  17 310-317
• 25 Veyrac C, Couture A, Saguintaah M. et al . MRI of fetal GI tract abnormalities.  Abdom Imaging. 2004;  29 411-420
• 26 Walker M, Lovell M, Kelly T. et al . Multiple areas of intestinal atresia associated with immunodeficiency and posttransfusion graft-versus-host disease.  J Pediatr. 1993;  123 93-95