Histologic studies of rectocolic aganglionosis and allied diseases

doi:10.1016/S0022-3468(78)80215-2

Journal of Pediatric Surgery

Volume 13, Issue 1, February 1978, Pages 67-75

https://doi.org/10.1016/S0022-3468(78)80215-2 Get rights and content

It is well-known that some patients exhibiting the symptoms of Hirschsprung's disease are shown to have intramural ganglia in the distal myenteric plexus. Some of them have been described as “pseudo-Hirschsprung's disease.”^1–3 It is likely that a variety of conditions have been included in this group. Using a silver impregnation technique we have made a detailed histologic study of the rectum and large bowel in a number of cases with provisional clinical diagnosis of Hirschsprung's disease but in which rectal biopsy showed small ganglia in Meissner's plexus demonstrated by acetylcholinesterase (Ach-E) staining. Three histologic variations were shown that may be called hypoganglionosis, immaturity of ganglia, and hypogenesis. An attempt is made to relate their clinical picture to the histologic findings.

References (18)

EhrenpreisT
Some newer aspects on Hirschsprung's disease and allied disorders
J Pediatr Surg
(1966)
AldridgeRT et al.
Ganglion cell distribution in the normal rectum and anal canal. A basis for the diagnosis of Hirschsprung's disease by anorectal biopsy
J Pediatr Surg
(1968)
HofmannS et al.
Histology of the myenteric plexus in relation to rectal biopsy in congenital megacolon
J Pediatr Surg
(1967)
SmithB
Pre- and postnatal development of the ganglion cells of the rectum and its surgical implications
J Pediatr Surg
(1968)
Meier-RugeW
Das Megacolon, seine diagnose und Pathophysiologie
Virchows Arch
(1968)
NixonHH
What is pseudo-Hirschsprung's disease?
Arch Dis Child
(1966)
WakeK
Motor endplates in developing chick embryo skeletal muscle: Histological structure and histochemical localization of cholinesterase activity
Arch Histol Jpn
(1964)
GarrettJR et al.
Autonomic nerves in rectum and colon in Hirschsprung's disease
Arch Dis Child
(1969)
TriggPH et al.
Experience with a cholinesterase histochemical technique for rectal suction biopsies in the diagnosis of Hirschsprung's disease
J Clin Pathol
(1974)

There are more references available in the full text version of this article.

Cited by (60)

Gastrointestinal Pathology
2020, Pediatric Gastrointestinal and Liver Disease, Sixth Edition
Interpretation of endoscopic gastrointestinal biopsy specimens requires adequate clinical information including medication history and illnesses that may have associated gastrointestinal findings. The clinical history can guide additional studies in addition to the standard hematoxylin and eosin staining. Precise identification of the biopsy site enables the most accurate and definitive diagnosis. The endoscopist should attempt to obtain the largest possible piece of tissue. Multiple biopsies for each site often provide the best information. If special studies such as culture for microorganisms, electron microscopy, or flow cytometry are required, communication with the laboratory before biopsy is recommended. Standard histopathologic evaluation is best performed on tissue immediately placed in fixative. The resulting “final diagnosis” may require re-review if additional clinical findings are obtained.
Gastrointestinal Pathology
2011, Pediatric Gastrointestinal and Liver Disease
Gastrointestinal Pathology
2010, Pediatric Gastrointestinal and Liver Disease
Large intestine (colon)
2009, Modern Surgical Pathology
Large Intestine (Colon)
2009, Modern Surgical Pathology
New classification of hypoganglionosis: congenital and acquired hypoganglionosis
2006, Journal of Pediatric Surgery
Hypoganglionosis has been associated with fewer intestinal ganglion cells. However, current reports questioned the validity of this clinical entity. The aim of this study is to demonstrate the existence of hypoganglionosis.
We have experienced 24 cases of functional intestinal obstruction with abnormalities of the intestinal ganglia. A precise histological examination was performed using quantitative morphometric studies. These results were compared with age-matched controls (n = 13).
Based on histological examination, disorders in the ganglion cells could be classified into 3 categories: immaturity of ganglia (n = 13), congenital hypoganglionosis (n = 7), and acquired hypoganglionosis (n = 4). In congenital hypoganglionosis, the number as well as the size of ganglion cells are small at birth. The size of ganglion cells tends to increase over time, but their numbers do not increase; as a result, the symptoms of dysmotility do not improve. On the other hand, acquired hypoganglionosis is late onset and characterized as a degeneration of ganglion cells and gliosis histologically. After performing a resection of the affected bowel, the prognosis is usually good.
Congenital and acquired hypoganglionosis are 2 distinct entities. The histological findings as well as the clinical characteristics of these 2 types of hypoganglionosis are different.

View all citing articles on Scopus

^†: Presented before the XXIV Annual International Congress of the British Association of Paediatric Surgeons, Oslo, Norway, August 2–6, 1977.

¹: From the First Department of Surgery, Nihon University School of Medicine, Tokyo, Japan.

View full text

Histologic studies of rectocolic aganglionosis and allied diseases†

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Das Megacolon, seine diagnose und Pathophysiologie

Virchows Arch

What is pseudo-Hirschsprung's disease?

Arch Dis Child

Motor endplates in developing chick embryo skeletal muscle: Histological structure and histochemical localization of cholinesterase activity

Arch Histol Jpn

Autonomic nerves in rectum and colon in Hirschsprung's disease

Arch Dis Child

Experience with a cholinesterase histochemical technique for rectal suction biopsies in the diagnosis of Hirschsprung's disease

J Clin Pathol