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Hirschsprung’s-associated enterocolitis (HAEC) is defined as the presence of diarrhea, explosive stools, abdominal distension and radiologic evidence of bowel obstruction or mucosal edema.  HAEC is the most common cause of mortality in patients with Hirschsprung’s disease.  This condition may occur in any stage of the Hirschsprung’s disease, with a mean incidence of 25%.  The pathophysiology is poorly understood. Potential etiologies are mechanical obstruction, infections, defective mucosal barrier, and impaired immunological system.  Microscopically HAEC is characterized by cryptitis and crypt abscesses followed by progressive mucosal ulceration eventually leading to transmural necrosis.  The mainstays of treatment are fluid resuscitation, decompression of the bowel and antibiotic therapy.  Sometimes a stoma is required when such measures have failed.  The majority of patients with HAEC continue to have disturbances of bowel function many years after surgery for Hirschsprung’s disease.  Researchs into the pathophysiology of HAEC can lead to better preventative measures and treatment modalities.

Palabras Clave: Enfermedad de Hirschsprung; Enterocolitis; Síndrome de Down; Aganglionosis congénita.