Síndrome de Berdon (megavejiga, microcolon, hipoperistalsis): Presentación de nuestros casos

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Abstract

El Síndrome de Berdon es una malformación congénita muy poco frecuente que asocia megavejiga y alteraciones intestinales severas, las cuales condicionan el pronóstico en la mayoría de los casos. Presentamos una revisión de tres casos diagnosticados en nuestro servicio en el periodo 1976- 2003. Se comentan los aspectos diagnósticos, terapéuticos y la evolución.

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Megacystis microcolon intestinal hypoperistalsis syndrome: late sequelae and possible pathogenesis.

G Mahour, H Isaacs, Tim E. Ford … (1993)

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by hypoperistalsis in the presence of ganglion cells, malrotation, microcolon, bladder distension, and female predilection. We draw attention to the long-term management of a patient with MMIHS and propose a pathogenetic mechanism to account for this syndrome. We propose that the initial event in the pathogenesis of MMIHS is an intramural inflammatory process that affects the gastrointestinal and urinary tracts. This leads to extensive fibrosis which destroys the intestinal neural network, producing hypoperistalsis. The same process causes neuromuscular incoordination in the bladder wall, resulting in irregular bladder contractions against a "closed sphincter" leading to bladder distension. The enlarged bladder then interferes with the rotation of the intestine causing malrotation.

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Microcolon-intestinal hypoperistalsis without megacystis: uncommon form of neonatal intestinal pseudoobstruction.

A Gökdemir, G Ozok, M Tunçyürek … (1995)

Two cases of microcolon-intestinal hypoperistalsis without megacystis are reported. They had dilated proximal small bowel and narrowed distal small bowel and malrotated microcolon. No organic obstructive intestinal lesion was found and double-barrel ileostomy was performed. The biopsy specimens showed ganglion cells to be normal in number and appearance in the entire intestinal wall. The ileostomy did not function postoperatively and drugs stimulating bowel movement failed to induce peristalsis. We have suggested that microcolon-intestinal hypoperistalsis without megacystis may be the cause of functional intestinal obstruction in neonates and it is a variant of megacystis-microcolon-hypoperistalsis syndrome.