Acquired aganglionosis following surgery for Hirschsprung's disease: a report of five cases during a 33‐year experience with pull‐through procedures

Cohen, Marta C.; Moore, Samuel W.; Neveling, Ulane; Kaschula, R. O. C.

doi:10.1111/j.1365-2559.1993.tb00096.x

Cited by 33 publications

(10 citation statements)

References 26 publications

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“…In these studies, it has been reported that biopsies of the neorectum were devoid of ganglion cells, whereas sections obtained from the proximal margin of the previous pull-through segment contained ganglion cells. Interestingly, in one of the cases reported by Cohen and associates [6], ganglion cells were present only focally in a transverse section of proximal margin, suggesting resec- Closely spaced myenteric plexus ganglia, present at the tip of the leading edge in the transition zone.…”

Section: Discussionmentioning

confidence: 82%

Circumferential Distribution of Ganglion Cells in the Transition Zone of Children with Hirschsprung Disease

White

Langer

2000

Pediatr Dev Pathol

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We prospectively studied the circumferential distribution of ganglion cells in the transition zone from a study population of 21 patients with Hirschsprung disease (HD) undergoing a pull-through procedure over a 26-month period. Twelve cases were satisfactory for examination, in that the transition zone was contained within a single surgical specimen and specimen distortion was minimal. Ganglion cells in the submucosa were counted in all 12 cases. In seven of the cases, the transition zone was proximal to the rectal sleeve and myenteric plexus ganglion cells were also counted. We found an uneven circumferential distribution of ganglion cells in both myenteric plexus and submucosa of the transition zone, resulting in a "leading edge" of ganglion cells extending into aganglionic distal bowel. The maximum length of this leading edge was 2.4 cm and 2.1 cm in the myenteric plexus and submucosa, respectively. Ganglion cells at the tip of the leading edge were present in clusters of up to six ganglion cells, in marked contrast to an absence of ganglion cells for most of the remainder of the circumference. Closely spaced myenteric plexus ganglia were seen at the tip of the leading edge in some cases. The leading edge was more frequently observed along the antimesenteric side, but this was not statistically significant. Our findings have relevance in the interpretation of intraoperative biopsies at the time of pull-through surgery and subsequent biopsies of neorectum in patients with surgically corrected HD.

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Section: Discussionmentioning

confidence: 82%

Circumferential Distribution of Ganglion Cells in the Transition Zone of Children with Hirschsprung Disease

White

Langer

2000

Pediatr Dev Pathol

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“…Occasional reports supported these concepts as being uncommon variants sometimes seen in association with HSCR. [35][36][37] These are of considerable interest to surgeons, both from an etiological as well as a management point of view.…”

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confidence: 99%

Hirschsprung disease: current perspectives

Moore¹

2016

OAS

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Hirschsprung disease is a complex congenital condition of the intestine, which is recognized as being of genetic origin and results from a disturbance of the normal development of the enteric nervous system. As a result, aganglionosis of the distal bowel occurs. It is the most common cause of a low intestinal obstruction in the neonate as well as older children. Occurring as an isolated condition in 70% of cases, it may be associated with other associated congenital abnormalities as well as a number of syndromic phenotypes. A number of distinct genetic sites have been identified in these syndromic phenotypes, which identify potential underlying genetic associations of the disease and indicate the probable gene-gene interaction in its pathogenesis. This review looks at the prevalence, congenital associations, and possible genetic factors influencing the development of Hirschsprung disease. Diagnostic dilemmas, surgical management, potential postsurgical complications, and outcomes are also explored.

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“…Familiarization with the mesentery vascular arcades and adequate preservation are important to avoid the poor blood supply of the PT bowel. 2,13,14 This study showed that ganglion cell loss accounted for 29.8% (n ¼ 17) of redo cases. Therefore, when the obstructive symptoms persist after PT despite conservative management, it is important to perform a preoperative workup in consideration of the possibility of ganglion cell loss and consider a redo PT.…”

Section: Discussionmentioning

confidence: 76%