Enterocolitis asociada a enfermedad de Hirschsprung / Enterocolitis associated with Hirschsprung's disease

La enterocolitis asociada a enfermedad de Hirschsprung (EAEH) se define como la presencia de diarrea, deposiciones explosivas, distensión abdominal y signos radiológicos de obstrucción intestinal o edema de la mucosa. Es la causa más común de mortalidad en pacientes con enfermedad de Hirschsprung (EH). Puede ocurrir en cualquier etapa de la enfermedad, con una incidencia media del 25 por ciento. La fisiopatología es poco conocida. Etiologías potenciales son la obstrucción mecánica, infecciones, barrera mucosa defectuosa, y un sistema inmunológico deficitario. Microscópicamente la EAEH se caracteriza por criptitis y abscesos en las criptas, seguida de ulceración de la mucosa progresiva conduciendo eventualmente a la necrosis transmural. Los pilares del tratamiento son la reanimación con fluidos, la descompresión del intestino y la terapia antibiótica. A veces se requiere de una estoma cuando estas medidas han fracasado. La mayoría de los pacientes con EAEH siguen teniendo trastornos de la función intestinal muchos años después de la cirugía para la enfermedad de Hirschsprung. Investigaciones en la fisiopatología de la EAEH pueden conducir a medidas preventivas y mejores modalidades de tratamiento.

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 percent. 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.

Hirschsprung's disease: revisited.

The diagnosis and therapy of Hirschsprung's disease has changed in recent times and a firm diagnosis of the entity can be made pre-operatively by immunohisto-chemistry. There has been a recent trend of switching over from the conventional staged surgical procedures to primary pull-through procedures. In this article the newer concepts referring to its aetiology, pathogenesis, and the current technical advancements like stapler anastomosis, laparoscopic assisted pull-through and single one stage operation without colostomy are discussed along with a brief mention of current concepts in intestinal neuronal dysplasia, enterocolitis and total colonic aganglionosis.

importance of pathological findings in identification of patients at risk of developing Hirschsprung's disease associated enterocolitis

Enterocolitis remains the most serious complication of Hirschsprung's disease, and has been reported in 15% to 50% of cases. Patients are prone to develop relapses despite a defunctioning colostomy and after definitive pull-through procedures. There are several risk factors as delayed diagnosis of Hirschsprung's disease, long spastic segment, trisomy 21 and .family history of Hirschsprung's disease. Currently no clinical factor or test is available to predict patients prone to develop postoperative enterocolitis. Thirty-two Hirschsprung's disease patients were studied in an attempt to correlate the histologic changes of the colonic mucosa with the clinical and post-operative course to outline a histological guide line of value in prediction of patients prone to develop clinical enterocolitis

Paracoccidioidomicose com enterocolite em criança com 12 anos / Paracoccidioidomycosis enterocolitis in a twelve-years-old girl

Menina de 12 anos com doença de evoluçäo subaguda caracterizada por notável comprometimento do estado geral, envolvimentos linfático, digestivo severo (enterocolite), pulmonar e cutâneo, além de sinais clínicos de hipertensäo portal, que regrediram com a instituiçäo de anfotericina B. O P. braziliensis foi encontrado em biópsias de gânglio cervical, de lesöes cutâneas, de sigmóide e de reto, tendo sido as manifestaçöes digestivas de enterocolite as iniciais, acompanhadas de dor e aumento do volume abdominal, aliados ainda a febre e notável perda de peso.