Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome

Raofi, Vandad; Beatty, Elizabeth; Testa, G.; Abcarian, Herand; Oberholzer, José; Sankary, Howard; Grevious, Mark A.; Benedetti, Enrico

doi:10.1016/j.jpedsurg.2007.09.073

Cited by 29 publications

(17 citation statements)

References 19 publications

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“…Gosemann et al reported that 12 of 227 patients with a diagnosis of MMIHS received multivisceral transplantations [5]. The indications of small bowel or multivisceral transplantation need to be discussed based on the systematic analyses of many cases of this condition [15][16][17][18].…”

Section: Discussionmentioning

confidence: 99%

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Soh

Fukuzawa

Kubota

et al. 2015

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 99%

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Soh

Fukuzawa

Kubota

et al. 2015

Journal of Pediatric Surgery

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“…Most of the cases required multivisceral transplantation as multiorgan failure was often involved. In other cases that required intestinal transplantation for reasons other than MMIHS, isolated intestinal transplantation before liver failure has developed has shown better outcomes such as earlier enteral feeding, when compared to multivisceral transplantation [23,24]. A series has reported 50% survival 3 years after intestinal and multivisceral transplantation, all survivors showed satisfactory gastric emptying and tolerated enteral feeds [21].…”

Section: Discussionmentioning

confidence: 99%

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Case Reports and Discussion of the Literature

Sousa¹,

Upadhyay

Stone

2015

Fetal Diagn Ther

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Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare intestinal dysmotility condition that also involves a dilated urinary bladder. It was believed to be an autosomal recessive condition, but genetic studies have suggested possibly an autosomal dominant inheritance pattern. Prenatal diagnosis can be challenging, but MRI and amniotic fluid/digestive fluid studies may be complementary investigations to improve diagnostic accuracy. Prognosis of MMIHS is generally poor and treatment is mostly supportive. To date, bowel transplantation remains the only viable treatment to restore bowel motility. Here we present two additional cases to contribute towards the scant literature on this condition.

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“…Two patients had prolonged survival after transplantation, and one died from multiple organ failure. Multivisceral transplantation is a valuable alternative therapy for children with severe gastrointestinal dysmotility that cannot be adequately managed with TPN, or who present with associated complications [29].…”

Section: Treatment Optionsmentioning

confidence: 99%

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): Report of a case with prolonged survival and literature review

López-Muñoz

Hernández-Zarco

Polanco-Ortiz

et al. 2013

Journal of Pediatric Urology

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Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) was first described in 1976. A rare congenital autosomal recessive alteration that predominantly affects females (4:1 ratio), it is characterized by the presence of distended bladder (without distal urinary tract obstruction), microcolon, and decreased or absent intestinal peristalsis. Inconsistent and non-specific histological changes affecting the bladder and intestinal smooth muscle, and intrinsic innervations, have been reported most frequently. MMIHS usually has a fatal prognosis in the first year of life; nevertheless there are some case reports of longer survival. Here is presented the case report of a boy with a diagnosis of MMIHS who has achieved prolonged survival, followed by a review of the literature.

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Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome

Cited by 29 publications

References 19 publications

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Case Reports and Discussion of the Literature

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): Report of a case with prolonged survival and literature review

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