Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome

Michele Masetti, Maria M. Rodriguez, John F. Thompson, Antonio D. Pinna, Tomoaki Kato, Rita L. Romaguera, Jose R. Nery, Werviston DeFaria, Mohammed F. Khan, Roberto Verzaro, Phillip Ruiz, Andreas G. Tzakis

Research output: Contribution to journalArticle

39 Citations (Scopus)

Abstract

Background. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare autosomal recessive disorder causing a functional neonatal bowel obstruction. Its etiopathogenesis is not fully understood. The prognosis is poor in the majority of cases; most patients die before the age of 6 months. In this report, we describe our experience with three patients with MMIHS in whom multivisceral transplantation was performed. Methods. Three patients with MMIHS underwent multivisceral transplantation. All patients were females with a history of long-term total parenteral nutrition (TPN) with TPN-related cholestatic liver disease. Results. Patient 1 died 17 months after transplantation because of aspiration after revision of her feeding gastrostomy. At the time of death, the graft was functioning and the patient was completely off TPN. Patient 2 is alive 17 months after transplant. She is a fully functional, active 2-year-old and has also recently begun oral feeding after intensive rehabilitation. Patient 3 died on day 44 of multisystem failure. Conclusions. This is the first report in the literature of multivisceral transplantation for MMIHS. Although one of the three patients died 44 days after surgery from multiorgan system failure, the other two patients had long-term survival after transplant and both grew well on enteral feeding alone. One patient died 17 months from a non-transplant- related complication, while the other is living at home off of TPN, with almost complete dietary rehabilitation 17 months after transplant. Our case reports suggest that multivisceral transplantation is a valuable therapeutic option for patients affected by MMIHS with TPN-induced liver failure.

Original languageEnglish (US)
Pages (from-to)228-232
Number of pages5
JournalTransplantation
Volume68
Issue number2
DOIs
StatePublished - Jul 27 1999
Externally publishedYes

Fingerprint

Transplantation
Total Parenteral Nutrition
Transplants
Megacystis microcolon intestinal hypoperistalsis syndrome
Home Total Parenteral Nutrition
Rehabilitation
Gastrostomy
Liver Failure
Enteral Nutrition
Ambulatory Surgical Procedures
Liver Diseases
Survival

ASJC Scopus subject areas

  • Transplantation
  • Immunology

Cite this

Masetti, M., Rodriguez, M. M., Thompson, J. F., Pinna, A. D., Kato, T., Romaguera, R. L., ... Tzakis, A. G. (1999). Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome. Transplantation, 68(2), 228-232. https://doi.org/10.1097/00007890-199907270-00011

Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome. / Masetti, Michele; Rodriguez, Maria M.; Thompson, John F.; Pinna, Antonio D.; Kato, Tomoaki; Romaguera, Rita L.; Nery, Jose R.; DeFaria, Werviston; Khan, Mohammed F.; Verzaro, Roberto; Ruiz, Phillip; Tzakis, Andreas G.

In: Transplantation, Vol. 68, No. 2, 27.07.1999, p. 228-232.

Research output: Contribution to journalArticle

Masetti, M, Rodriguez, MM, Thompson, JF, Pinna, AD, Kato, T, Romaguera, RL, Nery, JR, DeFaria, W, Khan, MF, Verzaro, R, Ruiz, P & Tzakis, AG 1999, 'Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome', Transplantation, vol. 68, no. 2, pp. 228-232. https://doi.org/10.1097/00007890-199907270-00011
Masetti, Michele ; Rodriguez, Maria M. ; Thompson, John F. ; Pinna, Antonio D. ; Kato, Tomoaki ; Romaguera, Rita L. ; Nery, Jose R. ; DeFaria, Werviston ; Khan, Mohammed F. ; Verzaro, Roberto ; Ruiz, Phillip ; Tzakis, Andreas G. / Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome. In: Transplantation. 1999 ; Vol. 68, No. 2. pp. 228-232.
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abstract = "Background. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare autosomal recessive disorder causing a functional neonatal bowel obstruction. Its etiopathogenesis is not fully understood. The prognosis is poor in the majority of cases; most patients die before the age of 6 months. In this report, we describe our experience with three patients with MMIHS in whom multivisceral transplantation was performed. Methods. Three patients with MMIHS underwent multivisceral transplantation. All patients were females with a history of long-term total parenteral nutrition (TPN) with TPN-related cholestatic liver disease. Results. Patient 1 died 17 months after transplantation because of aspiration after revision of her feeding gastrostomy. At the time of death, the graft was functioning and the patient was completely off TPN. Patient 2 is alive 17 months after transplant. She is a fully functional, active 2-year-old and has also recently begun oral feeding after intensive rehabilitation. Patient 3 died on day 44 of multisystem failure. Conclusions. This is the first report in the literature of multivisceral transplantation for MMIHS. Although one of the three patients died 44 days after surgery from multiorgan system failure, the other two patients had long-term survival after transplant and both grew well on enteral feeding alone. One patient died 17 months from a non-transplant- related complication, while the other is living at home off of TPN, with almost complete dietary rehabilitation 17 months after transplant. Our case reports suggest that multivisceral transplantation is a valuable therapeutic option for patients affected by MMIHS with TPN-induced liver failure.",
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AU - Rodriguez, Maria M.

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AU - Kato, Tomoaki

AU - Romaguera, Rita L.

AU - Nery, Jose R.

AU - DeFaria, Werviston

AU - Khan, Mohammed F.

AU - Verzaro, Roberto

AU - Ruiz, Phillip

AU - Tzakis, Andreas G.

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N2 - Background. Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare autosomal recessive disorder causing a functional neonatal bowel obstruction. Its etiopathogenesis is not fully understood. The prognosis is poor in the majority of cases; most patients die before the age of 6 months. In this report, we describe our experience with three patients with MMIHS in whom multivisceral transplantation was performed. Methods. Three patients with MMIHS underwent multivisceral transplantation. All patients were females with a history of long-term total parenteral nutrition (TPN) with TPN-related cholestatic liver disease. Results. Patient 1 died 17 months after transplantation because of aspiration after revision of her feeding gastrostomy. At the time of death, the graft was functioning and the patient was completely off TPN. Patient 2 is alive 17 months after transplant. She is a fully functional, active 2-year-old and has also recently begun oral feeding after intensive rehabilitation. Patient 3 died on day 44 of multisystem failure. Conclusions. This is the first report in the literature of multivisceral transplantation for MMIHS. Although one of the three patients died 44 days after surgery from multiorgan system failure, the other two patients had long-term survival after transplant and both grew well on enteral feeding alone. One patient died 17 months from a non-transplant- related complication, while the other is living at home off of TPN, with almost complete dietary rehabilitation 17 months after transplant. Our case reports suggest that multivisceral transplantation is a valuable therapeutic option for patients affected by MMIHS with TPN-induced liver failure.

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