Surgery, Surgical Pathology, and Postoperative Management of Patients With Hirschsprung Disease

Abstract

Endorectal pullthrough surgery is integral in the treatment of patients with Hirschsprung disease. Several different surgical procedures exist, which share as common goals to excise the aganglionic segment and upstream transition zone and attach ganglionic bowel just proximal to the anal canal. The operation requires collaboration between surgeon and pathologist to localize ganglionic bowel and prevent retention of transition zone. Intraoperative frozen sections are extremely important, first to establish that ganglion cells are present and subsequently to exclude features of transition zone (partial circumferential aganglionosis, myenteric hypoganglionosis, and submucosal nerve hypertrophy) at the proximal surgical (anastomotic) margin. Postoperative histopathological analysis of resection specimens should be tailored to document distal aganglionosis, document the length of the aganglionic segment and its proximity to the anastomotic margin, and confirm that transition zone has been resected completely. Adherence to the recommendations described in this review will reduce the likelihood of transition zone pullthrough and should decrease the incidence of persistent postoperative obstructive symptoms.

Neurons populating the rectal extrinsic nerves in humans express neuronal and Schwann cell markers

“…In normal bowel, extrinsic nerves penetrate the muscularis externa, fuse with intrinsic ganglionic interconnectives, and appear to merge imperceptibly with the myenteric plexus 23 . Distal to the points of intersection, adjacent ganglia and interganglionic connectives lose GLUT1‐immunoreactive perineurium, intrinsic collagen, and other features of extrinsic nerves.…”

“…In normal bowel, extrinsic nerves penetrate the muscularis externa, fuse with intrinsic ganglionic interconnectives, and appear to merge imperceptibly with the myenteric plexus. 23 Distal to the points of intersection, adjacent ganglia and interganglionic connectives lose GLUT1-immunoreactive perineurium, intrinsic collagen, and other features of extrinsic nerves. Only rare extrinsic nerves are thought to ascend or descend in the plane of the myenteric plexus before fusion with myenteric plexus or to penetrate the muscularis propria and merge with submucosal plexus, before the GLUT1-positive perineurium is lost.…”

Persistent bowel dysfunction after surgery for Hirschsprung’s disease: A neuropathological perspective

“…Surgical resection of the aganglionic segment remains the gold-standard treatment of HD[ 2 , 8 ]. The aim of surgery is to resect the aganglionic bowel and pull-through the bowel segment that contains a normal enteric nervous system.…”

“…Therefore, it is important to check intraoperatively in which part ganglion cells are present. This is usually performed by neuropathological review of a frozen biopsy of the whole bowel circumference during open surgery or multiple biopsies during laparoscopic surgery[ 8 ]. The most widely applied techniques to identify ganglionic and aganglionic bowel segments are hematoxylin and eosin (HE) and acetylcholinesterase (AChE) histochemistry[ 4 , 5 ].…”

“…There is growing evidence that immunohistochemistry against calretinin may also be supportive in detecting aganglionic segments[ 12 , 13 ]. In general, it is argued that the dissection level should be at least five centimeters proximal to the biopsy location were ganglion cells were confirmed to be sure that ganglionic bowel is pulled through[ 4 , 8 ]. However, the transition zone may extend even longer in patients with a longer aganglionic segment, which requires a longer resection margin[ 14 ].…”