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Case Reports
. 2021 Jan;9(1):e28-e32.
doi: 10.1055/s-0041-1726347. Epub 2021 Mar 25.

Skip Segment Hirschsprung Disease Managed by Pull-Through of the Right Colon

Hira Ahmad  1 , Alejandra Vilanova-Sánchez  2 , Isabel Amengual  3 , Laura Guerra-Pastrian  4 , Marta Garrido-Pontnou  5 , Cristina Montalvo  6 , Alba Bueno  7 , Jacob Langer  1   8 , Richard J Wood  1 , Marc A Levitt  9
Affiliations
Case Reports

Skip Segment Hirschsprung Disease Managed by Pull-Through of the Right Colon

Hira Ahmad et al. European J Pediatr Surg Rep. 2021 Jan.

Abstract

Hirschsprung disease is the most common neurocristopathy in children, resulting in the congenital loss of enteric ganglia. Rare reports of skip lesions have previously been reported in the literature. We present a case of skip lesions known prior to surgery and managed by pull-through of the right colon that allowed the preservation of the colon.

Keywords: aganglionosis; calretinin stain; continence; enterocolitis; reoperation.

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Conflict of interest statement

Conflict of Interest None declared.

Figures

Fig. 1
Fig. 1
Repeat contrast enema concerning for long-segment Hirschsprung disease: microcolon, the question mark-shaped colon, and the lack of features in an otherwise normal colon.
Fig. 2
Fig. 2
Results of colonic mapping at the time of ileostomy creation, showing Hirschsprung disease with skip lesions. (+) denotes presence of ganglion cells. (–) denotes absence of ganglion cells.
Fig. 3
Fig. 3
Colonic vasculature and our plan for the resection and pull-through. The blue lines mark the vasculature that will be taken down for the resection, derotation, and eventual pull-through of the right colon.
Fig. 4
Fig. 4
Cecal resection, extended left colectomy, ileocolic anastomosis, right colonic derotation pull-through of the right colon. The last figure shows the final postoperative anatomy.
Fig. 5
Fig. 5
( A ) Section of normal ileum (proximal margin) with ganglion cells in the myenteric plexus (hematoxylin and eosin [H&E] left, marked by arrows) and normal expression of calretinin in lamina propria of the mucosa (right, marked by arrows). ( B ) Section of aganglionic segment of the cecum demonstrating absence of ganglion cells in the submucosal and myenteric plexuses (H&E left) and loss of calretinin expression in lamina propria of the mucosa (right). ( C ) Descending colon with isolated ganglion cell in the myenteric plexus (H&E left marked by arrows) and few nerve fibers in lamina propria stained with calretinin (right, marked by arrows)
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