Idiopathic Megacolon—Short Review

Abstract

Introduction: Idiopathic megacolon (IM) is a rare condition with a more or less known etiology, which involves management challenges, especially therapeutic, and both gastroenterology and surgery services. With insufficiently drawn out protocols, but with occasionally formidable complications, the condition management can be difficult for any general surgery team, either as a failure of drug therapy (in the context of a known case, initially managed by a gastroenterologist) or as a surgical emergency (in which the diagnostic surprise leads additional difficulties to the tactical decision), when the speed imposed by the severity of the case can lead to inadequate strategies, with possibly critical consequences. Method: With such a motivation, and having available experience limited by the small number of cases (described by all medical teams concerned with this pathology), the revision of the literature with the update of management landmarks from the surgical perspective of the pathology appears as justified by this article. Results: If the diagnosis of megacolon is made relatively easily by imaging the colorectal dilation (which is associated with initial and/or consecutive clinical aspects), the establishing of the diagnosis of idiopathic megacolon is based in practice almost exclusively on a principle of exclusion, and after evaluating the absence of some known causes that can lead to the occurrence of these anatomic and clinical changes, mimetically, clinically, and paraclinically, with IM (intramural aganglionosis, distal obstructions, intoxications, etc.). If the etiopathogenic theories, based on an increase in the performance of the arsenal of investigations of the disease, have registered a continuous improvement and an increase of objectivity, unfortunately, the curative surgical treatment options still revolve around the same resection techniques. Moreover, the possibility of developing a form of etiopathogenic treatment seems as remote as ever.

The Classification and Surgical Treatments in Adult Hirschsprung’s Disease: A Retrospective Study

“…It should be noted that some patients with idiopathic megacolon may be associated with malnutrition due to longterm constipation and fecal obstruction (28). For these patients, an intestinal stoma is a compelling choice.…”

Idiopathic megabowel causing acute bowel obstruction: A case report

“…The different surgical options for idiopathic megabowel are essentially colon and/or rectal resection, pelvic floor procedures, or fecal diversion [ 7 ]. Bowel resection is the best option knowing that conservative treatments with oral laxatives and enemas are not efficient [ 11 ]. If only a part of the colon is affected, segmental colectomy is sufficient.…”

Jóvenes con megacolon crónico: características epidemiológicas, clínicas y manométricas

“…4 En el megacolon adquirido, la mayoría de los pacientes son adultos y se presentan con estreñimiento crónico, distensión abdominal y en ocasiones, con antecedentes de vólvulo o de fecalomas como complicaciones. 7 Una revisión sistemática reciente sobre megacolon adquirido ha intentado establecer criterios diagnósticos para el megacolon idiopático crónico que se presenta en la adolescencia o la adultez. Ellos son: exclusión de enfermedad orgánica, diámetro sigmoideo mayor a 10 cm en estudios radiológicos y la presencia de síntomas de estreñimiento, distensión abdominal, dolor abdominal o malestar por gases.…”

“…Particularmente, el megacolon idiopático se caracteriza por el diámetro aumentado del colon y la consecuente dismotilidad colónica en ausencia de una causa detectable aparente. 7 Existen series que describen familiares con megacolon idiopático, lo cual sugiere que ciertas alteraciones genéticas podrían estar vinculadas a su desarrollo. 11 En nuestra población, el 25% de los pacientes (6/23) presentó una probable EH por ausencia del RRAI en la manometría anorrectal.…”

“…Podría desarrollarse debido a anomalías en los plexos nerviosos entéricos, los nervios extrínsecos o el muscu-lo liso intestinal con la consecuente dilatación y trastornos de la motilidad intestinal. [6][7] El objetivo del tratamiento médico del megacolon es mejorar el estreñimiento. Cuando falla el tratamiento médico la cirugía es el tratamiento de elección y se indica en casos de fecalomas a repetición o vólvulos.…”