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Megacystis microcolon intestinal hypoperistalsis syndrome: systematic review of outcome

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Abstract

Purpose

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. This congenital condition is associated with non-obstructed urinary bladder, microcolon and decreased or absent intestinal peristalsis. This study was designed to determine the incidence and outcome of MMIHS.

Methods

A systematic review of the literature (1976–2011) was performed for key words “megacystis microcolon intestinal hypoperistalsis”. Resulting publications were reviewed for epidemiology and outcome. Reference lists were screened for additional cases.

Results

A total number of 227 MMIHS cases were reported from 1976 to 2011. A clear preponderance for female infants was found (female 70.6 vs. male 29.4%). One or more surgical interventions were reported in 115 patients (including gastrostomy, ileostomy, jejunostomy, segmental resections of small bowel, adhesiolysis and internal sphincter myectomy). For decompression of the megacystis, vesicostomy was performed in 41 patients. Outcome was reported in a total of 218 patients. Survival rate was 19.7% (survivors: n = 43, non-survivors: n = 175), the oldest survivor being 24 years old. The vast majority of the surviving patients had to be maintained by total or partial parenteral nutrition (TPN). Main causes of death were sepsis, malnutrition and multiple organ failure. Twelve multivisceral transplantations have been reported to date in patients with MMIHS. The majority of the survivors are reported to be free of parenteral nutrition and show improving gastric emptying. However, intermittent catheterisation remains obligatory due to persistent bladder dysfunction.

Conclusions

The survival in MMIHS in recent years has improved. The majority of survivors are either maintained by TPN or have undergone multiorgan transplantations.

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Authors and Affiliations

  1. National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Ireland

    Jan-Hendrik Gosemann & Prem Puri

  2. School of Medicine and Medical Science and Conway Institute of Biomedical Research, University College Dublin, Dublin 12, Ireland

    Prem Puri

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  1. Jan-Hendrik Gosemann
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  2. Prem Puri
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Correspondence to Prem Puri.

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Gosemann, JH., Puri, P. Megacystis microcolon intestinal hypoperistalsis syndrome: systematic review of outcome. Pediatr Surg Int 27, 1041–1046 (2011). https://doi.org/10.1007/s00383-011-2954-9

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