Treatment

Treatment in the first instance is aimed at decompression of the distal GI tract by regular rectal washouts. Subsequently a number of surgical options exist, all of which aim to remove the agan-glionic segment and restore intestinal continuity by means of an anastomosis of ganglionated bowel to the rectal stump (so-called 'pull-through'). This may be performed either as a primary procedure or as a delayed procedure after initial colostomy formation

Hirschsprung Disease Abdominal Ray
Figure 2.6 Plain abdominal X-ray of a child with rectosigmoid Hirschsprung's disease showing extensively dilated loops of bowel.

to allow distal intestinal decompression.28 There are a number of surgical techniques, of which three are the most commonly used - the Swenson rectosig-moidectomy, the Duhammel retrorectal transanal pull-through and the endorectal pull-through of Soave. These techniques may also be carried out laparoscopically in suitable infants.

Of paramount importance in all surgical approaches is removal of the full length of aganglionic intestine. Failure to do so may result in recurrence of symptoms and complications of Hirschsprung's disease. To ensure normoganglionosis of the segment, pulled-through intraoperative biopsies are taken and rapidly examined histologically. Biopsies may be taken laparoscopically in infants undergoing a minimally invasive procedure. A number of authors have advocated laparoscopic colonic mapping prior to or during definitive surgery for exact characterization of the extent of the disease.29,30 Recently there has been interest in infants who have retained a portion of the transition zone and have recurrent symptoms.31,32 Attention has been drawn to the fact that the transition zone may not be linear around the circumference of the intestine, and it has been recommended that multiple circumferential biopsies be taken.31,33

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