Case Report

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Report of a Rare Case in Newborn

Abstract

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. The characteristic features of this congenital and fatal disease are abdominal distension, absent or decreased bowel peristalsis. Abdominal distension is a consequence of the distended, unobstructed urinary bladder with or without hydronephrosis. We present a case of female newborn with antenatal ultrasound revealing a large cystic mass in pelvic with urinary tract origin, abdominal distension, aperistalsis of the intestine and micro colon.

Berdon WE, Baker DH, Blanc WA, et al. Megacystismicrocolon- intestinal hypoperistalsis syndrome:a new cause of intestinal obstruction in the newborn: report of radiological findings in five newborn girls. AJR Am J Roentgenol 1976;126(5):957-64.

Jain VK, Garge S, Singh S, et al. Megacystis microcolon intestinal hypoperistalsis syndrome complicated by perforation. Afr J Paediatr Surg 2011;8(1):70-1.

Puri P, Shinkai M. Megacystis microcolon intestinal hypoperistalsis syndrome. Semin Pediatr Surg 2005;14(1):58-63.

Gosemann JH, Puri P. Megacystis microcolon intestinal hypoperistalsis syndrome: a systematic review of outcome. Pediatr Surg Int 2011;27(10):1041-6.

Chamyan G, Debich-Spicer D, Opitz JM, et al. Megacystis-microco- lon-intestinal hypoperistalsis syndrome and aganglionosis in trisomy 18. Am J Med Genet 2001;102(3):293-6.

Rolle U, O'Briain S, Pearl RH, et al. Megacystismicrocolon- intestinal hypoperistalsis syndrome: Evidence of intestinal myopathy. Pediatr Surg Int 2002;18(1):2-5.

Piotrowska AP, Rolle U, Chertin B, et al. Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Surg 2003;38(5):749-55.

Steiner SJ. Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). J Pediatr Gastroenterol Nutr 2004;39(3):301.

Hidaka N, Kawamata K, Chiba Y. Megacystis-microcolonintestinal hypoperistalsis syndrome: in utero sonographic appearance and the contribution of vesicocentesis in antenatal diagnosis. J Ultrasound Med 2006;25(6):765-9.

Oka Y, Asabe K, Shirakusa T, et al. An antenatal appearance of megacystis-microcolon-intestinal hypoperistalsis syndrome. Turk J Pediatr 2008;50(3):269-74.

Lorenzo AJ, Twickler DM, Baker LA. Megacystis microcolon intestinal hypoperistalsis syndrome with bilaterally duplicated systems. Urology 2003;62(1):144.

Mantan M, Singhal KK, Sethi GR, et al. Megacystis, microcolon, intestinal hypoperistalsis syndrome and bilateral streak gonads. Indian J Nephrol 2011;21(3):212-4.

Boman F, Sfeir R, Bonnevalle M, et al. Complexity of pathological interpretation in megacystis-microcolonintestinal hypoperistalsis syndrome. Ann Pathol 2006;26(2):115-21.

Zugor V, Schott GE, Labanaris AP. The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease. Pediatr Rep 2012;4(2):e20.

Tonni G, Ida V, Alessandro V, et al. Prune-belly syndrome: case series and review of the literature regarding early prenatal diagnosis, epidemiology, genetic factors, treatment, and prognosis. Fetal Pediatr Pathol 2013;31(1):13-24.

Raofi V, Beatty E, Testa G, et al. Combined living related liver and bowel transplantation for megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Surg 2008;43(2):e9-e11.

Manop J, Chamnanvanakij S, Wattanasarn C. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): a case report in Thailand. J Med Assoc Thai 2004;87(11):1385-8.

Köhler M, Pease PW, Upadhyay V. Megacystismicrocolon- intestinal hypoperistalsis syndrome (MMIHS) in siblings: case report and review of the literature. Eur J Pediatr Surg 2004;14(5):362-7.

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IssueVol 53, No 8 (2015) QRcode
SectionCase Report(s)
Keywords
Megacystis Microcolon Hypoperistalsis Newborn

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How to Cite
1.
Tarlan S, Mahyar A, Chegini V, Chegini V. Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Report of a Rare Case in Newborn. Acta Med Iran. 2015;53(8):518-522.