TENSION PNEUMOPERITONEUM: A RARE PRESENTATION OF MEGACYSTIS MICROCOLON INTESTINAL HYPOPERISTALSIS SYNDROME
AbstractMegacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) also known as Berdon syndrome is a rare autosomal recessive disorder characterized by enlarged urinary bladder, small colon and reduced or absent intestinal peristalsis. We report a case of a 4 days old female presenting with tension pneumoperitoneum. She underwent emergent decompression of the pneumoperitoneum followed by laparotomy which showed a sigmoid perforation. Her per-operative findings, antenatal and postanatal imaging were consistent with a diagnosis of MMIHS. She was treated with a palliative colostomy, vesicostomy and parenteral nutrition. To the best of our knowledge, this is the first reported case of Berdon syndrome presenting atypically with tension pneumoperitoneum. Megacystis microcolon intestinal hypoperistalsis syndrome also known as Berdon syndrome is characterized by enlarged urinary bladder, small colon and reduced or absent intestinal peristalsis. We report a case of 4 days old female suffering from MMIHS presenting with tension pneumoperitoneum. To the best of our knowledge, this is the first reported case of MMIHS, having this unusual presentation.Keywords: Tension pneumoperitoneum; megacystic microcolon; Intestinal hypoperistalsis; syndrome; perforation; Intestinal malrotation
Berdon WE, Baker DH, Blanc WA, Gay B, Santuli TV, Donovan C. Megacystis-microcolon-intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn - Report of radiological findings in five newborn girls. AM J Roentgenol 1976;126(5):957–64.
Anneren G, Meurling S, Olsen L. Megacystis-microcolon- intestinal hypoperistalsis syndrome (MMIHS): an autosomal recessive disorder - Clinical reports and review of the literature. Am J Med Genet 1991;41(2):251–4.
Verbruggen SC, Wijnen RM, van den Berg P. Megacystis microcolon-intestinal hypoperistalsis syndrome: a case report. J Matern Fetal Neonatal Med 2004;16(2):140–1.
Manop J, Chamnanvanakij S, Wattanasarn C. Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS): A Case Report in Thailand. J Med Assoc Thai 2004;87(11):1385–8.
Puri P, Lake BD, Gorman F, O'Donnel B, Nixon HH. Megacystis-microcolon-intestinal hypoperistalsis syndrome: a visceral myopathy. J Pediatr Surg 1983;18(1):64–9.
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.