Jan-Hendrik Gosemann1, Prem Puri. 1. National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
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.
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.
Authors: Hau D Le; Vincent E de Meijer; David Zurakowski; Jonathan A Meisel; Kathleen M Gura; Mark Puder Journal: JPEN J Parenter Enteral Nutr Date: 2010 Sep-Oct Impact factor: 4.016
Authors: Eran Bornstein; Kristin Atkins; Shira Fishman; Ana Monteagudo; Ewa B Bajor-Dattilo; Farzana Arif; Susan Monda; Alba M Greco; Ilan E Timor-Tritsch Journal: J Ultrasound Med Date: 2008-07 Impact factor: 2.153
Authors: M Masetti; M M Rodriguez; J F Thompson; A D Pinna; T Kato; R L Romaguera; J R Nery; W DeFaria; M F Khan; R Verzaro; P Ruiz; A G Tzakis Journal: Transplantation Date: 1999-07-27 Impact factor: 4.939
Authors: C E Richardson; J M Morgan; B Jasani; J T Green; J Rhodes; G T Williams; J Lindstrom; S Wonnacott; G A Thomas; V Smith Journal: Gastroenterology Date: 2001-08 Impact factor: 22.682
Authors: Robert A Cowles; Kara A Ventura; Mercedes Martinez; Steven J Lobritto; Patricia A Harren; Susan Brodlie; Joanne Carroll; Dominique M Jan Journal: J Pediatr Surg Date: 2010-01 Impact factor: 2.545
Authors: Nurit Assia Batzir; Pranjali Kishor Bhagwat; Austin Larson; Zeynep Coban Akdemir; Maciej Bagłaj; Leon Bofferding; Katherine B Bosanko; Skander Bouassida; Bert Callewaert; Ashley Cannon; Yazmin Enchautegui Colon; Adolfo D Garnica; Margaret H Harr; Sandra Heck; Anna C E Hurst; Shalini N Jhangiani; Bertrand Isidor; Rebecca O Littlejohn; Pengfei Liu; Pilar Magoulas; Helen Mar Fan; Ronit Marom; Scott McLean; Marjan M Nezarati; Kimberly M Nugent; Michael B Petersen; Maria L Rocha; Elizabeth Roeder; Robert Smigiel; Ian Tully; James Weisfeld-Adams; Katerina O Wells; Jennifer E Posey; James R Lupski; Arthur L Beaudet; Michael F Wangler Journal: Hum Mutat Date: 2019-12-19 Impact factor: 4.878