AJP Rep 2011; 01(02): 077-082
DOI: 10.1055/s-0031-1281510
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Concordant Intestinal Atresia in Two Pairs of Monozygotic Twins

Mario Giuffrè
1   Dipartimento Materno Infantile, Università degli Studi di Palermo, Palermo, Italy
,
Mariavalentina Catania
1   Dipartimento Materno Infantile, Università degli Studi di Palermo, Palermo, Italy
,
Giovanni Corsello
1   Dipartimento Materno Infantile, Università degli Studi di Palermo, Palermo, Italy
› Author Affiliations
Further Information

Publication History

Publication Date:
01 July 2011 (online)

Abstract

Intestinal atresia in both twins from the same pregnancy is very rare. Only seven pairs of twins have been described. The authors report on two cases of monozygotic twins with different types of intestinal atresia and clinical evolution. Both pairs of observed twins turned out to be concordant for the presence of intestinal malformations and for the absence of other linked malformations; nevertheless, the atresic lesions were anatomically discordant in each pair of monozygotic twins. Therefore, the diagnostic and therapeutic procedures have shown some differences in phenotypic expression between the twins of both pairs. Possible etiologic factors and pathogenetic pathways are discussed, and the importance of an accurate clinical and instrumental investigation and a long-term follow-up is underlined. Very rare models, such as pairs of monozygotic twins presenting intestinal atresia, represent an extraordinary resource to add new clinical and laboratory information likely to be useful in future advancements to understand the underlying etiology and pathogenesis.

 
  • References

  • 1 Chirdan LB, Uba AF, Pam SD. Intestinal atresia: management problems in a developing country. Pediatr Surg Int 2004; 20: 834-837
  • 2 Matsumoto Y, Komatsu K, Tabata T. Jejuno-ileal atresia in identical twins: report of a case. Surg Today 2000; 30: 438-440
  • 3 Shorter NA, Georges A, Perenyi A, Garrow E. A proposed classification system for familial intestinal atresia and its relevance to the understanding of the etiology of jejunoileal atresia. J Pediatr Surg 2006; 41: 1822-1825
  • 4 Barakat NA, Maati SH. Nutritional and surgical management of short bowel syndrome in neonates. Res J Medicine & Med Sci 2009; 4: 220-223
  • 5 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: 1250-1252
  • 6 Spencer AU, Neaga A, West B , et al. Pediatric short bowel syndrome: redefining predictors of success. Ann Surg 2005; 242: 403-409 ; discussion 409–412
  • 7 Waldhausen JHT, Sawin RS. Improved long-term outcome for patients with jejunoileal apple peel atresia. J Pediatr Surg 1997; 32: 1307-1309
  • 8 Tandler J. Entwicklungsgeschichte des Menschilichen Duodenum in Fruhen Embruonalstadien. Morphol Jahrb 1900; 29: 187-216
  • 9 Louw JH, Barnard CN. Congenital intestinal atresia; observations on its origin. Lancet 1955; 269: 1065-1067
  • 10 Yokoyama T, Ishizone S, Momose Y, Terada M, Kitahara S, Kawasaki S. Duodenal atresia in dizygotic twins. J Pediatr Surg 1997; 32: 1806-1808
  • 11 Bilodeau A, Prasil P, Cloutier R , et al. Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg 2004; 39: 726-730
  • 12 Lambrecht W, Kluth D. Hereditary multiple atresias of the gastrointestinal tract: report of a case and review of the literature. J Pediatr Surg 1998; 33: 794-797
  • 13 Fairbanks TJ, Kanard R, Del Moral PM , et al. Fibroblast growth factor receptor 2 IIIb invalidation—a potential cause of familial duodenal atresia. J Pediatr Surg 2004; 39: 872-874
  • 14 Fairbanks TJ, Sala FG, Kanard R , et al. The fibroblast growth factor pathway serves a regulatory role in proliferation and apoptosis in the pathogenesis of intestinal atresia. J Pediatr Surg 2006; 41: 132-136 ; discussion 132–136
  • 15 Kanard RC, Fairbanks TJ, De Langhe SP , et al. Fibroblast growth factor-10 serves a regulatory role in duodenal development. J Pediatr Surg 2005; 40: 313-316
  • 16 Tatekawa Y, Kanehiro H, Nakajima Y. Duodenal atresia associated with “apple peel” small bowel without deletion of fibroblast growth factor-10 or fibroblast growth factor receptor 2IIIb: report of a case. Surg Today 2007; 37: 430-433