Deficient neurogenic innervation of the myenteric plexus with normal submucous plexus involving the entire small and large bowel

doi:10.1016/S0022-3468(89)80307-0

Journal of Pediatric Surgery

Volume 24, Issue 1, January 1989, Pages 83-87

https://doi.org/10.1016/S0022-3468(89)80307-0 Get rights and content

A newborn presented with a picture of intestinal obstruction. Multiple biopsies of the small and large bowel showed an unusual neurogenic innervation. The myenteric plexus of Auerbach was severely depeleted of ganglion cells and nerve fibers, while the submucous plexus of Meissner was normally innervated. An ileostomy failed to function and extended trial with experimental smooth muscle stimulant (Cisapride) was equally ineffective. The patient was finally treated by a myectomy from the duodenum to the descending colon with a sigmoid colostomy. This procedure, coupled with a Nissen fundoplication, stopped the vomiting and allowed normal defecation through the colostomy. The patient is presently taking increasing increments of oral fluids with a concomitant decrease in the volume of parenteral nutrition. The myectomy initiated marked hypertrophy of the muscularis mucosa. Could this muscular hypertrophy account for the improvement in bowel function? Possible etiology will be discussed. We caution that rectal submucosal suction biopsy alone may be misleading if normal ganglion cells and nerve fibers are found, yet the patient's clinical symptoms fail to improve. A full thickness bowel wall biopsy is then recommended.

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Cited by (9)

Counting myenteric ganglion cells in histologic sections: An empirical approach
2010, Human Pathology
Citation Excerpt :
Certainly, some severe forms of congenital or acquired (eg, Chagas disease, autoimmune enteric neuropathy) hypoganglionosis are well established and can be recognized without formal ganglion cell counts. However, milder forms of hypo- or hyperganglionosis have been postulated as the basis for other types of intestinal pseudo-obstruction or chronic constipation, often based on histologic impressions without either formal quantitative data or reliable controls [32-38]. Assessment of myenteric neuron density is particularly challenging for the general surgical pathologist, who has to rely on published criteria for neuronal identification and normative data, most of which are collected in a research context and have not been validated by other laboratories.
An abnormal density of myenteric neurons is a putative cause of intestinal pseudo-obstruction. Quantification of myenteric ganglion cells may be necessary to establish hyper- or hypoganglionosis, but published norms are very discordant. We investigated how observer bias and tissue sampling affect the accuracy and reproducibility of myenteric neuron counts obtained from histologic sections immunostained for HuC/D, a neuronal cell body-specific antigen. Despite a collective effort to standardize neuronal identification criteria, significant discrepancies were found between the counts obtained by different observers. In contrast, counts by a single observer, over a period of several months, revealed excellent reproducibility. To investigate effects of tissue sampling on the accuracy of ganglion cell density estimates, one observer counted immunoreactive neurons in 22 full-circumference rectal sections from the same paraffin block. The mean number of neurons per circumference from all 22 sections was considered a target, against which estimates from smaller samples were compared. To ensure an accurate estimate of the circumferential density (within 10% of the target value), counts had to be averaged from at least 5 sections of nearly the full circumference. Examinations of fewer sections or less than two thirds of the circumference were prone to errors. Application of these principles to sections from the transitional zone in Hirschsprung disease validated the approach and discriminated relatively subtle changes in neuronal density. We conclude that neuronal counts are best performed by individuals using their own normative data for reference and that biopsies of small portions of the circumference may not resolve potentially significant hypo- or hyperganglionosis.
Embryologic and anatomic basis of duodenal surgery
2000, Surgical Clinics of North America
Citation Excerpt :
Megaduodenum is most likely secondary to neuromotor function at the myenteric plexus of Auerbach, which produces a functional obstruction similar to that of patients with congenital megacolon. Mishalany et al40 reported on a case of a newborn infant with intestinal obstruction secondary to severe depletion of ganglion cells and nerve fibers at Auerbach's plexus; the submucous plexus of Meissner was normal. False diverticula of the duodenum (Fig. 5) are usually found on the concave wall of the second and third parts of the duodenum, often close to the duodenal papillae.
Increased expression of muscular neural cell adhesion molecule in congenital aganglionosis
1993, Gastroenterology
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2018, Pediatric Reports
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^*: Presented at the 19th Annual Meeting of the American Pediatric Surgical Association, Tucson, Arizona, May 11–14, 1988.

¹: From the Department of Surgery, Pathology, and Pediatrics, White Memorial Medical Center, Los Angeles; and the Division of Pediatric Surgery and Department of Pathology, Childrens Hospital of Los Angeles, University of Southern California, School of Medicine, Los Angeles.

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Deficient neurogenic innervation of the myenteric plexus with normal submucous plexus involving the entire small and large bowel*

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Lancet

J Pediatr Surg