Pitfalls in the management of Hirschsprung's disease

Abstract

Forty complicated cases of Hirschsprung's disease were reviewed for errors in management. We found that there were 9 common pitfalls in diagnosis, surgical technic, and postoperative care. Diagnosis 1.Abdominal exploration of a neonate with low small bowel obstruction or severe ileus, not obviously due to meconium ileus, without a prior barium enema. 2.Failure to recognize that diarrhea and abdominal distention during infancy may be due to enterocolitis associated with Hirschsprung's disease, particularly if there is a history of delayed passage of meconium. 3.Failure to recognize that severe prolonged ileus or partial low small bowel obstruction in infancy associated with a normal barium enema may represent total aganglionosis of the colon. Surgical Technic 4.Failure to place the colostomy where ganglion cells are present, preferably in the terminal portion of the normal colon. 5.Failure to resect all but 1 to 2 cm. of the aganglionic rectum. 6.Pulling through and anastomosing aganglionic colon to the rectal cuff. 7.Performing the anastomosis under tension. Postoperative Management 8.Failure to treat an anastomotic leak with an immediate colostomy. 9.Failure to recognize and treat with rectal irrigations enterocolitis developing in the early postoperative period. This review of complicated cases has reaffirmed our impression that the treatment of Hirschsprung's disease is an intricate and dangerous undertaking. The details of barium enema interpretation, preoperative preparation, rectal biopsy, colostomy and pull-through technics, and postoperative care are of equal importance. A mistake made in any one of these areas usually results in a less than satisfactory course, and occasionally a permanent colostomy or fatality. Forty complicated cases of Hirschsprung's disease were reviewed for errors in management. We found that there were 9 common pitfalls in diagnosis, surgical technic, and postoperative care. 1.Abdominal exploration of a neonate with low small bowel obstruction or severe ileus, not obviously due to meconium ileus, without a prior barium enema. 2.Failure to recognize that diarrhea and abdominal distention during infancy may be due to enterocolitis associated with Hirschsprung's disease, particularly if there is a history of delayed passage of meconium. 3.Failure to recognize that severe prolonged ileus or partial low small bowel obstruction in infancy associated with a normal barium enema may represent total aganglionosis of the colon. 4.Failure to place the colostomy where ganglion cells are present, preferably in the terminal portion of the normal colon. 5.Failure to resect all but 1 to 2 cm. of the aganglionic rectum. 6.Pulling through and anastomosing aganglionic colon to the rectal cuff. 7.Performing the anastomosis under tension. 8.Failure to treat an anastomotic leak with an immediate colostomy. 9.Failure to recognize and treat with rectal irrigations enterocolitis developing in the early postoperative period. This review of complicated cases has reaffirmed our impression that the treatment of Hirschsprung's disease is an intricate and dangerous undertaking. The details of barium enema interpretation, preoperative preparation, rectal biopsy, colostomy and pull-through technics, and postoperative care are of equal importance. A mistake made in any one of these areas usually results in a less than satisfactory course, and occasionally a permanent colostomy or fatality. Es revistate 40 complicate casos de morbo de Hirschsprung, resultante in un lista de 9 errores commun e recurrente. Tres esseva diagnostic: Celiotomia in un neonato con basse obstruction intestinal sin clyster a barium, maldiagnose de enterocolitis infantil con morbo de Hirschsprung como gastroenteritis, e maldiagnose de aganglionosis total. Quatro errores esseva technic: Placiamento del colostomia in un colon aganglionic, inadequate resection rectal, anastomose de un colon aganglionic al manchette rectal, e effectuation del anastomose sub tension. Duo errores esseva postoperatori: Retardo in le effectuation de colostomia a causa de un escappamento anastomotic, e maltractamento de enterocolitis postoperatori. Es revistate 40 complicate casos de morbo de Hirschsprung, resultante in un lista de 9 errores commun e recurrente. Tres esseva diagnostic: Celiotomia in un neonato con basse obstruction intestinal sin clyster a barium, maldiagnose de enterocolitis infantil con morbo de Hirschsprung como gastroenteritis, e maldiagnose de aganglionosis total. Quatro errores esseva technic: Placiamento del colostomia in un colon aganglionic, inadequate resection rectal, anastomose de un colon aganglionic al manchette rectal, e effectuation del anastomose sub tension. Duo errores esseva postoperatori: Retardo in le effectuation de colostomia a causa de un escappamento anastomotic, e maltractamento de enterocolitis postoperatori.