Abstract
The megacystis-microcolon-intestinal hypoperistalsis syndrome is a congenital disorder characterized by urinary bladder distension and hypoperistalsis throughout the entire gastrointestinal tract. We present a new case with the typical clinical, radiological, and pathological findings of the syndrome. The diagnosis should be suspected in a patient who presents clinically with intestinal obstruction and urinary retention, and confirmed with imaging studies, including abdominal plain films, urinary tract ultrasonography, and contrast studies of the colon and the bladder. The prognosis is generally very poor. Our patient died secondary to sepsis on day 5 of life.
This is a preview of subscription content, log in via an institution to check access.
References
Berdon WE, Baker DH, Blanc WA, Gay B, Santulli V, Donovan C (1976) Megacystis microcolon intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. Am J Roentgenol 126: 957–964
Anneren G, Meurling S, Olsen L (1991) Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), an autosomal recessive disorder: clinical report and review of the literature. Am J Med Genet 41: 251–254
Jona JZ, Werlin SL (1981) The megacystis microcolon intestinal hypoperistalsis syndrome: report of a case. J Pediatr Surg 16: 749–751
Kubota M, Ikeda K, Ito Y (1989) Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal bypoperistalsis syndrome: electrophysiological study. J Pediatr Surg 24: 1267–1270
Gillis DA, Grantmyre EB (1985) Megacystis microcolon intestinal hypoperistalsis syndrome: survival of a male infant. J Pediatr Surg 20: 279–281
Al-Rayess M, Ambler MW (1992) Axonal dystrophy presenting as the megacystis microcolon intestinal hypoperistalsis syndrome. Pediatr Pathol 12: 740–750
Puri P, Lake BD, Gorman F, O'Donnell B, Nixon HH (1983) Megacystis microcolon intestinal hypoperistalsis syndrome: a visceral myopathy. J Pediatr Surg 18: 64–68
Taguchi T, Ikeda K, Shono T, Goto S, Kubota M, Kawana T, Hiroe R, Toyohara T (1989) Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome: immunohistochemical study. J Pediatr Surg 24: 1264–1266
Dogruyol H (1989) Do certain drugs cause the megacystis microcolon intestinal hypoperistalsis syndrome. Turk J Pediatr 31: 253–256
Kirtane J, Talwalker V, Dastur DK (1984) Megacystis microcolon intestinal hypoperistalsis syndrome: possible pathogenesis. J Pediatr Surg 19: 206–208
Srikant MS, Ford EG, Isaacs H Jr, Mahour GH (1993) Megacystis microcolon intestinal hypoperistalsis syndrome: late sequelae and possible pathogenesis. J Pediatr Surg 28: 957–959
Milla PJ, Lake BD, Spitz L, Nixon HH, Harries JT, Fenton TR (1983) Chronic idiopathic intestinal pseudo-obstruction in infancy: a smooth muscle disease. Gastrointest Mot: 125–131
Stamm E, King G, Thickman D (1991) Megacystis microcolon intestinal hypoperistalsis syndrome: prenatal identification in siblings and review of the literature. J Ultrasound Med 10: 599–602
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kupferman, J.C., Stewart, C.L., Schapfel, D.M. et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome. Pediatr Nephrol 9, 626–627 (1995). https://doi.org/10.1007/BF00860960
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00860960