Skip to main content

Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome

  • Chapter

Abstract

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital and generally fatal cause of functional intestinal obstruction in the newborn. This syndrome is characterized by abdominal distension caused by a distended nonobstructed urinary bladder, microcolon and decreased or absent intestinal peristalsis [1]. Usually incomplete intestinal rotation and shortened small bowel are associated.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD   219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Berdon WE, Baker DH, Blanc WA, et al (1976) Megacystis-microcolon-intestinal hypoperistalsis syndrome. A new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls. AJR Am J Roentgenol 126:957–964

    PubMed  CAS  Google Scholar 

  2. Puri P, Lake BD, Gorman F, et al (1983) Megacystis-microcolon-intestinal hypoperistalsis syndrome: a visceral myopathy. J Pediatr Surg 18:64–69

    Article  PubMed  CAS  Google Scholar 

  3. Amoury RA, Fellows RA, Goodwin CD, et al (1977) Megacystis-microcolon-intestinal hypoperistalsis syndrome: a cause of intestinal obstruction in the newborn period. J Pediatr Surg 12:1063–1065

    Article  PubMed  CAS  Google Scholar 

  4. Wiswell TE, Rawlings JS, Wilson JL, et al (1979) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Pediatrics 63:805–808

    PubMed  CAS  Google Scholar 

  5. Vezina WC, Morin FR, Winsberg F (1979) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Antenatal ultrasound appearance. AJR Am J Roentgenol 133:749–750

    PubMed  CAS  Google Scholar 

  6. Patel R, Carty H (1980) Megacystis-microcolon-intestinal hypoperistalsis syndrome: a rare case of intestinal obstruction in the newborn. Br J Radiol 53:249–252

    PubMed  CAS  Google Scholar 

  7. Ando S, Makihara Y, Yamaguchi S, et al (1980) Megacystis microcolon-intestinal hypoperistalsis syndrome. J Jpn Pediatr Surg 16:1105–1110

    Google Scholar 

  8. Krook PM (1980) Megacystis-microcolon-intestinal hypoperistalsis syndrome in a male infant. Radiology 136:649–650

    PubMed  CAS  Google Scholar 

  9. Summer TE, Crowe JE, Klein A, et al (1981) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Am J Dis Child 135:67–68

    Google Scholar 

  10. Hoehn W, Thomas GG, Meradji M (1981) Urologic evaluation of Megacystis-microcolon-intestinal hypoperistalsis syndrome. Urology 17:465–466

    Article  PubMed  CAS  Google Scholar 

  11. Jona JZ, Werlin SL (1981) The Megacystis-microcolon-intestinal hypoperistalsis syndrome: report of a case. J Pediatr Surg 16:749–751

    Article  PubMed  CAS  Google Scholar 

  12. Young LW, Yunis EJ, Girdany BR, et al (1981) Megacystis-microcolon-intestinal hypoperistalsis syndrome. AJR Am J Roentgenol 137:749–755

    PubMed  CAS  Google Scholar 

  13. Osehc I, Jann X, Bettex M (1982) Ultrasonic antenatal detection of obstructed bladder. Z Kinderchir 35:109–111

    Google Scholar 

  14. Nelson LH, Reiff RH (1982) Megacystis-microcolon-intestinal hypoperistalsis syndrome and anechoic areas in the fetal abdomen. Am J Obstet Gynecol 144:464–467

    PubMed  CAS  Google Scholar 

  15. Shalev J, Itzchak Y, Avigad I, et al (1983) Antenatal ultrasound appearance of megacystis-microcolon-intestinal hypoperistalsis syndrome. Isr J Med Sci 19:76–78

    PubMed  CAS  Google Scholar 

  16. Oliveira G, Boechat MI, Ferreira MA (1983) Megacystis-microcolon-intestinal hypoperistalsis syndrome in a newborn girl whose brother had prune belly syndrome: common pathogenesis? Pediatr Radiol 13:294–296

    Article  PubMed  CAS  Google Scholar 

  17. Vinograd I, Mogle P, Lernau OZ, et al (1984) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Arch Dis Child 59:169–171

    PubMed  CAS  Google Scholar 

  18. Bagwell CE, Filler RM, Cutz E, et al (1984) Neonatal intestinal pseudo-obstruction. J Pediatr Surg 19:732–739

    Article  PubMed  CAS  Google Scholar 

  19. Redman JF, Jimenez JF, Golladay ES, et al (1984) Megacystis-microcolon-intestinal hypoperistalsis syndrome: case report and review of the literature. J Urol 131:981–983

    PubMed  CAS  Google Scholar 

  20. Kirtane J, Talwalker V, Dastur DK (1984) Megacystis-microcolon-intestinal hypoperistalsis syndrome: possible pathogenesis. J Pediatr Surg 19:206–208

    Article  PubMed  CAS  Google Scholar 

  21. Manco LG, Osterdahl P (1984) The antenatal sonographic features of megacystis-microcolon-intestinal hypoperistalsis syndrome. J Clin Ultrasound 12:595–598

    Article  PubMed  CAS  Google Scholar 

  22. Alexacos L, Skouteli H, Sofatzis J, et al (1985) Megacystis-microcolon-intestinal hypoperistalsis syndrome: a functional intestinal obstruction in the female newborn. Z Kinderchir 40:58–59

    PubMed  CAS  Google Scholar 

  23. Tomomasa T, Itoh Z, Koizumi T, et al (1985) Manometric study of the intestinal motility in a case of megacystis-microcolon-intestinal hypoperistalsis syndrome. J Pediatr Gastroenterol Nutr 4:307–310

    Article  PubMed  CAS  Google Scholar 

  24. Gillis DA, Grantmyre EB (1985) Megacystis-microcolon-intestinal hypoperistalsis syndrome: survival of a male infant. J Pediatr Surg 20:279–281

    Article  PubMed  CAS  Google Scholar 

  25. Dogruyol H, Gunay U, Esmer A, et al (1985) Megacystis-microcolon-intestinal hypoperistalsis syndrome in a newborn after clomiphene ingestion during pregnancy. Z Kinderchir 40:58–59

    Google Scholar 

  26. Bulut M, Kalayoglu M, Altin MA, et al (1985) The megacystis-microcolon-intestinal hypoperistalsis syndrome. A case report. Turk J Pediatr 27:169–176

    PubMed  CAS  Google Scholar 

  27. Vintzileos AM, Eisenfield LL, Herson VC, et al (1986) Megacystis-microcolon-intestinal hypoperistalsis syndrome: prenatal sonographic findings and review of literature. Am J Perinatol 3:297–302

    PubMed  CAS  Google Scholar 

  28. Winter RM, Knowles SAS (1986) Megacystis-microcolon-intestinal hypoperistalsis syndrome: confirmation of autosomal recessive inheritance. J Med Genet 23:360–362

    PubMed  CAS  Google Scholar 

  29. Willand DA, Gabriele OF (1986) Megacystis-microcolon-intestinal hypoperistalsis syndrome. A case report. Turk J Pediatr 14:481–485

    Google Scholar 

  30. Kovacs T, Toth Z, Szeifert G, et al (1987) Prenatal diagnosis of the megacystis-microcolon-hypoperistalsis syndrome. Orv Hetil 128:2257–2260

    PubMed  CAS  Google Scholar 

  31. Dogruyol H, Gunay U, Esmer A, et al (1987) Megacystis-microcolon-intestinal hypoperistalsis syndrome in a newborn after clomiphene ingestion during pregnancy. Z Kinderchir 42:321–323

    PubMed  CAS  Google Scholar 

  32. Aoki K, Ooba M (1987) A case of the megacystis-microcolon-intestinal hypoperistalsis syndrome. Rinsho Hoshasen 32:1135–1136

    PubMed  CAS  Google Scholar 

  33. Farrell SA (1988) Intrauterine death in megacystis-microcolon-intestinal hypoperistalsis syndrome. J Med Genet 25:350–351

    Article  PubMed  CAS  Google Scholar 

  34. Young ID, McKeever PA, Brown LA, et al (1989) Prenatal diagnosis of the megacystis-microcolon-intestinal hypoperistalsis syndrome. J Med Genet 26:403–406

    PubMed  CAS  Google Scholar 

  35. Bindl L, Emons D, Haverkamp F, et al (1989) Das megazystis-mikrokolon-intestinale hypoperistaltik-syndrom: Eine neuropatie? Z Kinderchir 44:249–252

    PubMed  CAS  Google Scholar 

  36. Penman DG, Lilford RJ (1989) The megacystis-microcolon-intestinal hypoperistalsis syndrome: a fatal autosomal recessive condition. J Med Genet 26:66–67

    PubMed  CAS  Google Scholar 

  37. Gakmak O, Pektas O, Maden HA, et al (1989) Megacystis-microcolon-intestinal hypoperistalsis syndrome in three siblings. Poster presentation at the Sixth International Congress of Paediatric Surgery, Istanbul, 29 August to 1 September

    Google Scholar 

  38. Yokoyama S, Fujimoto T, Tokuda J, et al (1989) Successful nutrition management of megacystis-microcolon-intestinal hypoperistalsis syndrome – a case report. Nutrition 5:423–426

    PubMed  CAS  Google Scholar 

  39. Taguchi T, Ikeda K, Shono T, et al (1989) Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome. I. Immunohistochemical study. J Pediatr Surg 24:1264–1266

    Article  PubMed  CAS  Google Scholar 

  40. Kubota M, Keiichi I, Yushi I (1989) Autonomic innervation of the intestine from a baby with megacystis microcolon intestinal hypoperistalsis syndrome. II. Electrophysiological study. J Pediatr Surg 24:1267–1270

    Article  PubMed  CAS  Google Scholar 

  41. Dogruyol HA (1989) Do certain drugs cause the megacystis-microcolon-intestinal hypoperistalsis syndrome? Turk J Pediatr 31:253–256

    PubMed  CAS  Google Scholar 

  42. Garber A, Shohat M, Sart D (1990) Megacystis-microcolon-intestinal hypoperistalsis syndrome in two male siblings. Prenat Diagn 10:377–387

    Article  PubMed  CAS  Google Scholar 

  43. De Vaux-Boitouzet V, Barau G, Blin G, et al (1990) Megavessie. Microcolon. Diagnostic echographique antenatal et revue de la literature. A propos d’un cas. J Gynecol Obstet Biol Reprod 19:327–332

    Google Scholar 

  44. Anneren G, Meurling S, Olsen L (1991) Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), an autosomal recessive disorder: clinical reports and review of the literature. Am J Med Genet 41:251–254

    Article  PubMed  CAS  Google Scholar 

  45. Couper RTL, Byard RW, Cutz E, et al (1991) Cardiac rhabdomyomata and megacystis-microcolon-intestinal hypoperistalsis syndrome. J Med Genet 28:274–276

    PubMed  CAS  Google Scholar 

  46. 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

    PubMed  CAS  Google Scholar 

  47. Carlsson SA, Hokegard KH, Mattsson LA (1992) Megacystis microcolon intestinal hypoperistalsis syndrome. Antenatal appearance in two cases. Acta Obstet Gynecol Scand 71:645–648

    Article  PubMed  CAS  Google Scholar 

  48. Al Rayess M, Ambler MW (1992) Axonal dystrophy presenting as the megacystis microcolon intestinal hypoperistalsis syndrome. Pediatr Pathol 12:743–750

    Article  PubMed  CAS  Google Scholar 

  49. Shono T, Suita S, Taguchi T, et al (1992) Manometric evaluation of gastrointestinal motility in a case of megacystis-microcolon-intestinal hypoperistalsis syndrome. Eur J Pediatr Surg 2:52–55

    PubMed  CAS  Google Scholar 

  50. Srikanth MS, Ford EG, Isaacs H, et al (1993) Megacystis microcolon intestinal hypoperistalsis syndrome: late sequelae and possible pathogenesis. J Pediatr Surg 28:957–959

    PubMed  CAS  Google Scholar 

  51. Gurgan T, Zeyneloglu HY, Develioglu O, et al (1993) Megacystis microcolon intestinal hypoperistalsis syndrome: antenatal ultrasound appearance. A case report. Asia Oceania J Obstet Gynaecol 19:383–386

    PubMed  CAS  Google Scholar 

  52. McNamara HM, Onwude JL, Thornton JG, et al (1994) Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case report supporting autosomal recessive inheritance. Prenat Diagn 14:153–154

    Article  PubMed  CAS  Google Scholar 

  53. Kobayashi H, O’Briain S, Puri P (1995) New observation on the pathogenesis of megacystis microcolon intestinal hypoperistalsis syndrome. Presentation at the Meeting of the American Pediatric Surgical Association, Boca Raton, FL

    Google Scholar 

  54. Dewan PA, Brown N, Murthy DP, et al (1995) Hydrometrocolpos and segmental colonic dilatation in a girl with megacystis-microcolon-intestinal hypoperistalsis syndrome. J Paediatr Child Health 31:479–482

    Article  PubMed  CAS  Google Scholar 

  55. Kupferman JC, Stewart CL, Schapfel DM, et al (1995) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Pediatr Nephrol 9:626–627

    Article  PubMed  CAS  Google Scholar 

  56. James C, Watson AR (1995) Megacystis-microcolon-intestinal-hypoperistalsis syndrome. Pediatr Nephrol 9:788–789

    Article  PubMed  CAS  Google Scholar 

  57. Ciftci AO, Cook RC, van Velzen D (1996) Megacystis microcolon intestinal hypoperistalsis syndrome: evidence of a primary myocellular defect of contractile fiber synthesis. J Pediatr Surg 31:1706–1711

    Article  PubMed  CAS  Google Scholar 

  58. Junior SR, Moreira MAF, Modelli MES, et al (1996) Megacystis-microcolon-intestinal hypoperistalsis syndrome. A case report. J Pediatria 72:109–112

    CAS  Google Scholar 

  59. Yigit S, Barlas C, Yurdakok M, et al (1996) The megacystis-microcolon-intestinal hypoperistalsis syndrome: report of a case and review of the literature. Turk J Pediatr 38:137–141

    PubMed  CAS  Google Scholar 

  60. Goldberg M, Pruchniewski D, Beale PG, et al (1996) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Pediatr Surg Int 11:246–247

    Article  Google Scholar 

  61. Smith VV, Milla PJ (1997) Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood. Histopathology 31:112–122

    Article  PubMed  CAS  Google Scholar 

  62. Ghavamian R, Wilcox DT, Duffy PG, et al (1997) The urological manifestations of hollow visceral myopathy in children. J Urol 158:1286–1290

    Article  PubMed  CAS  Google Scholar 

  63. Granata C, Puri P (1997) Megacystis-microcolon-intestinal hypoperistalsis syndrome. J Pediatr Gastroenterol Nutr 25:12–19

    Article  PubMed  CAS  Google Scholar 

  64. Chen CP, Wang TY, Chuang CY (1998) Sonographic findings in a fetus with megacystis-microcolon-intestinal hypoperistalsis syndrome. J Clin Ultrasound 26:217–220

    Article  PubMed  CAS  Google Scholar 

  65. Chung MY, Huang CB, Chuang JH, et al (1998) Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): a case report. Changgeng Yi Xue Za Zhi 21:92–96

    PubMed  CAS  Google Scholar 

  66. Colter KA (1998) Residents’ corner. Answer to case of the month #58. Megacystis-microcolon-intestinal hypoperistalsis syndrome. Can Assoc Radiol J 49:415–418

    PubMed  CAS  Google Scholar 

  67. Makhija PS, Magdalene KF, Babu MK (1999) Megacystis microcolon intestinal hypoperistalsis syndrome. Indian J Pediatr 66:945–949

    PubMed  CAS  Google Scholar 

  68. Al Harbi A, Tawil K, Crankson SJ (1999) Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with megaesophagus. Pediatr Surg Int 15:272–274

    Article  PubMed  CAS  Google Scholar 

  69. Faure C, Goulet O, Ategbo S, et al (1999) Chronic intestinal pseudoobstruction syndrome. Clinical analysis, outcome and prognosis in 105 children. Dig Dis Sci 44:953–959

    Article  PubMed  CAS  Google Scholar 

  70. Goulet O, Jobert-Giraud A, Michel JL, et al (1999) Chronic intestinal pseudo-obstruction syndrome in pediatric patients. Eur J Pediatr Surg 9:83–89

    PubMed  CAS  Google Scholar 

  71. Lashley DB, Masliah E, Kaplan GW, et al (2000) Megacystis microcolon intestinal hypoperistalsis syndrome: bladder distension and pyelectasis in the fetus without anatomic outflow obstruction. Urology 55:774

    Article  PubMed  CAS  Google Scholar 

  72. White SM, Chamberlain P, Hitchcock R, et al (2000) Megacystis-microcolon-intestinal hypoperistalsis syndrome: the difficulties with antenatal diagnosis. Case report and review of the literature. Prenat Diagn 20:697–700

    Article  PubMed  CAS  Google Scholar 

  73. Rite Gracia S, Fernandez Alvarez de Sotomayor B, Rebage Moises V, et al (2000) Megabladder-microcolon-intestinal hypoperistalsis syndrome. An Esp Pediatr 53:253–256

    PubMed  CAS  Google Scholar 

  74. Richardson CE, Morgan JM, Jasani B, et al (2001) Megacystis-microcolon-intestinal hypoperistalsis syndrome and the absence of the alpha3 nicotinic acetylcholine receptor subunit. Gastroenterology 121:350–357

    Article  PubMed  CAS  Google Scholar 

  75. Chamyan G, Debich-Spicer D, Opitz JM, et al (2001) Megacystis-microcolon-intestinal hypoperistalsis syndrome and aganglionosis in trisomy 18. Am J Med Genet 102:293–296

    Article  PubMed  CAS  Google Scholar 

  76. Kim KC (2001) Megacystis-microcolon-intestinal hypoperistalsis syndrome. Ryoikibetsu Shokogun Shirizu 34:159

    PubMed  Google Scholar 

  77. Witters I, Theyskens C, van Hoestenberghe R, et al (2001) Prenatal diagnosis of non-obstructive megacystis as part of the megacystis-microcolon-intestinal hypoperistalsis syndrome with favourable postnatal outcome. Prenat Diagn 21:704–706

    Article  PubMed  CAS  Google Scholar 

  78. Bloom TL, Kolon TF (2002) Severe megacystis and bilateral hydronephrosis in a female fetus. Urology 60:697

    Article  PubMed  Google Scholar 

  79. Chen LT, Yang W, Li CE, et al (2002) Megacystis microcolon intestinal hypoperistalsis syndrome with severe psychomotor retardation: report of one case. Acta Paediatr Taiwan 43:224–227

    PubMed  Google Scholar 

  80. Rolle U, O’Briain S, Pearl RH, et al (2002) Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. Pediatr Surg Int 18:2–5

    Article  PubMed  Google Scholar 

  81. Piotrowska AP, Role U, Chertin B, et al (2003) Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Surg 38:749–755

    Article  PubMed  Google Scholar 

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

    Article  Google Scholar 

  83. Hirato J, Nakazato Y, Koyama H, et al (2003) Encephalopathy in megacystis-microcolon-intestinal hypoperistalsis syndrome patients on long-term total parenteral nutrition possibly due to selenium deficiency. Acta Neuropathol 106:234–242

    Article  PubMed  Google Scholar 

  84. Hsu CD, Craig C, Pavlik J, et al (2003) Prenatal diagnosis of megacystis-microcolon-intestinal hypoperistalsis syndrome in one fetus of a twin pregnancy. Am J Perinatol 20:215–218

    Article  PubMed  Google Scholar 

  85. Lee NC, Tiu CM, Soong WJ, et al (2003) Megacystis-microcolon-intestinal hypoperistalsis syndrome: report of one case. Acta Paediatr Taiwan 44:238–241

    PubMed  CAS  Google Scholar 

  86. Jimenez Gil de Muro ST, Moros Pena M, Gimeno Pita P, et al (2004) Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case of prolonged survival. An Pediatr 60:369–372

    Article  CAS  Google Scholar 

  87. Piotrowska AP, Rolle U, Solari V, et al (2004) Interstitial cells of Cajal in the human normal urinary bladder and in the bladder of patients with megacystis-microcolon intestinal hypoperistalsis syndrome. BJU Int 94:143–146

    Article  Google Scholar 

  88. Xu W, Gelber S, Orr-Urtreger A, et al (1999) Megacystis, mydriasis and ion channel defect in mice lacking the alpha3 neuronal nicotinic acetylcholine receptor. Proc Natl Acad Sci U S A 96:5746–5751

    Article  PubMed  CAS  Google Scholar 

  89. Xu W, Orr-Urtreger A, Nigro F, et al (1999) Multiple autonomic dysfunction in mice lacking the β2 and β4 subunits of neuronal nicotinic acetylcholine receptors. J Neurosci 19:9298–9305

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Puri, P. (2008). Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome. In: Holschneider, A., Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-33935-9_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-33935-9_19

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-33934-2

  • Online ISBN: 978-3-540-33935-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics