Hideki Soh1, Masahiro Fukuzawa2, Akio Kubota3, Hisayoshi Kawahara4, Takehisa Ueno5, Tomoaki Taguchi6. 1. Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Japan. Electronic address: soh@pedsurg.med.osaka-u.ac.jp. 2. Department of Pediatric Surgery, Osaka Medical Center and Research Institute for Maternal and Child Health, Japan. 3. Second Department of Surgery, Wakayama Medical University, Japan. 4. Department of Pediatric Surgery, Hamamatsu University School of Medicine, Japan. 5. Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Japan. 6. Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Japan.
Abstract
BACKGROUND: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. METHODS: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. RESULTS: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. CONCLUSIONS: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.
BACKGROUND:Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. METHODS: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. RESULTS: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. CONCLUSIONS:MMIHSpatients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHSpatients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.
Authors: Natalia Buinoiu; Anca Panaitescu; Mihaela Demetrian; Sebastian Ionescu; Gheorghe Peltecu; Alina Veduta Journal: Clin Case Rep Date: 2018-03-12