Abstract
Full-thickness muscle biopsies have been taken from patients with severe disabling chronic constipation that has not responded to conservative measures. Assessment by neurohistochemical techniques has revealed that a range of neuronal dysplasias of the myenteric plexus are responsible in many cases; these include aganglionosis (Hirschsprung's disease), hypoganglionosis and hyperganglionosis. In cases considered unlikely to be Hirschsprung's disease on clinical grounds, the procedure used has often been anorectal myectomy; this has not only provided tissue for diagnosis but has also been of therapeutic value in most cases of hypoganglionosis and some cases reported as 'normal'.
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Selected References
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- Bennett A., Garrett J. R., Howard E. R. Adrenergic myenteric nerves in Hirschsprung's disease. Br Med J. 1968 Feb 24;1(5590):487–489. doi: 10.1136/bmj.1.5590.487. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Bishop A. E., Polak J. M., Lake B. D., Bryant M. G., Bloom S. R. Abnormalities of the colonic regulatory peptides in Hirschsprung's disease. Histopathology. 1981 Nov;5(6):679–688. doi: 10.1111/j.1365-2559.1981.tb01834.x. [DOI] [PubMed] [Google Scholar]
- Carney J. A., Hayles A. B. Alimentary tract manifestations of multiple endocrine neoplasia, type 2b. Mayo Clin Proc. 1977 Sep;52(9):543–548. [PubMed] [Google Scholar]
- Cohen I. T., Gadd M. A. Hirschsprung's disease in a kindred: a possible clue to the genetics of the disease. J Pediatr Surg. 1982 Oct;17(5):632–634. doi: 10.1016/s0022-3468(82)80124-3. [DOI] [PubMed] [Google Scholar]
- DAVIS B. J. Histochemical demonstration of erythrocyte esterases. Proc Soc Exp Biol Med. 1959 May;101(1):90–93. doi: 10.3181/00379727-101-24842. [DOI] [PubMed] [Google Scholar]
- Furness J. B., Costa M. Types of nerves in the enteric nervous system. Neuroscience. 1980;5(1):1–20. doi: 10.1016/0306-4522(80)90067-6. [DOI] [PubMed] [Google Scholar]
- Garrett J. R., Howard E. R., Nixon H. H. Autonomic nerves in rectum and colon in Hirschsprung's disease. A cholinesterase and catecholamine histochemical study. Arch Dis Child. 1969 Jun;44(235):406–417. doi: 10.1136/adc.44.235.406. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Howard E. R., Garrett J. R. Electron microscopy of myenteric nevrves in Hirschsprung's disease and in normal bowel. Gut. 1970 Dec;11(12):1007–1014. doi: 10.1136/gut.11.12.1007. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Howard E. R., Garrett J. R., Kidd A. Congenital neuronal disorders of the hind-gut: the value of ano-rectal biopsies. Scand J Gastroenterol Suppl. 1982;71:151–153. [PubMed] [Google Scholar]
- Jacobowitz D. Histochemical studies of the autonomic innervation of the gut. J Pharmacol Exp Ther. 1965 Sep;149(3):358–364. [PubMed] [Google Scholar]
- KAMIJO K., HIATT R. B., KOELLE G. B. Congenital megacolon; a comparison of the spastic and hypertrophied segments with respect to Cholinesterase activities and sensitivities to Acetylcholine, DFP and the barium ion. Gastroenterology. 1953 Jun;24(2):173–185. [PubMed] [Google Scholar]
- KOELLE G. B., KOELLE E. S., FRIEDENWALD J. S. The effect of inhibition of specific and non-specific cholinesterase on the motility of the isolated ileum. J Pharmacol Exp Ther. 1950 Oct;100(2):180–191. [PubMed] [Google Scholar]
- Kleinhaus S., Boley S. J., Sheran M., Sieber W. K. Hirschsprung's disease -- a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg. 1979 Oct;14(5):588–597. doi: 10.1016/s0022-3468(79)80145-1. [DOI] [PubMed] [Google Scholar]
- Le Marec B., Roussey M., Cornec A., Calmettes C., Kerisit J., Allanic H. Cancer de la thyroïde à stroma amyloïde, syndrome de Sipple, mégacolon congénital avec hyperplasie des plexus: une seule et même affection autosomique dominante à pénetrance complète. J Genet Hum. 1981 Feb;28(5):169–174. [PubMed] [Google Scholar]
- Lynn H. B., van Heerden J. A. Rectal myectomy in Hirschsprung disease: a decade of experience. Arch Surg. 1975 Aug;110(8):991–994. doi: 10.1001/archsurg.1975.01360140135026. [DOI] [PubMed] [Google Scholar]
- MacIver A. G., Whitehead R. Zonal colonic aganglionosis, a variant of Hirschsprung's disease. Arch Dis Child. 1972 Apr;47(252):233–237. doi: 10.1136/adc.47.252.233. [DOI] [PMC free article] [PubMed] [Google Scholar]
- MacMahon R. A., Moore C. C., Cussen L. J. Hirschsprung-like syndromes in patients with normal ganglion cells on suction rectal biopsy. J Pediatr Surg. 1981 Dec;16(6):835–839. doi: 10.1016/s0022-3468(81)80829-9. [DOI] [PubMed] [Google Scholar]
- Martin L. W., Buchino J. J., LeCoultre C., Ballard E. T., Neblett W. W. Hirschsprung's disease with skip area (segmental aganglionosis). J Pediatr Surg. 1979 Dec;14(6):686–687. doi: 10.1016/s0022-3468(79)80245-6. [DOI] [PubMed] [Google Scholar]
- Meier-Ruge W. Hirschsprung's disease: its aetiology, pathogenesis and differential diagnosis. Curr Top Pathol. 1974;59:131–179. doi: 10.1007/978-3-642-65857-0_4. [DOI] [PubMed] [Google Scholar]
- Munakata K., Okabe I., Morita K. Histologic studies of rectocolic aganglionosis and allied diseases. J Pediatr Surg. 1978 Feb;13(1):67–75. doi: 10.1016/s0022-3468(78)80215-2. [DOI] [PubMed] [Google Scholar]
- NORBERG K. A. ADRENERGIC INNERVATION OF THE INTESTINAL WALL STUDIED BY FLUORESCENCE MICROSCOPY. Int J Neuropharmacol. 1964 Sep;3:379–382. doi: 10.1016/0028-3908(64)90067-x. [DOI] [PubMed] [Google Scholar]
- Puri P., Lake B. D., Nixon H. H., Mishalany H., Claireaux A. E. Neuronal colonic dysplasia: an unusual association of Hirschsprung's disease. J Pediatr Surg. 1977 Oct;12(5):681–685. doi: 10.1016/0022-3468(77)90393-1. [DOI] [PubMed] [Google Scholar]
- Schärli A. F., Meier-Ruge W. Localized and disseminated forms of neuronal intestinal dysplasia mimicking Hirschsprung's disease. J Pediatr Surg. 1981 Apr;16(2):164–170. doi: 10.1016/s0022-3468(81)80344-2. [DOI] [PubMed] [Google Scholar]
- Stern M., Hellwege H. H., Grävinghoff L., Lambrecht W. Total aganglionosis of the colon (Hirschsprung's disease) and congenital failure of automatic control of ventilation (Ondine's curse). Acta Paediatr Scand. 1981 Jan;70(1):121–124. doi: 10.1111/j.1651-2227.1981.tb07184.x. [DOI] [PubMed] [Google Scholar]
- Torre J. C., Surgeon J. W. A methodological approach to rapid and sensitive monoamine histofluorescence using a modified glyoxylic acid technique: the SPG method. Histochemistry. 1976 Oct 22;49(2):81–93. doi: 10.1007/BF00495672. [DOI] [PubMed] [Google Scholar]
- Verdy M., Weber A. M., Roy C. C., Morin C. L., Cadotte M., Brochu P. Hirschsprung's disease in a family with multiple endocrine neoplasia type 2. J Pediatr Gastroenterol Nutr. 1982;1(4):603–607. doi: 10.1097/00005176-198212000-00027. [DOI] [PubMed] [Google Scholar]
- de Chadarévian J. P., Slim M., Akel S. Double zonal aganglionosis in long segment Hirschsprung's disease with a "skip area" in transverse colon. J Pediatr Surg. 1982 Apr;17(2):195–197. doi: 10.1016/s0022-3468(82)80213-3. [DOI] [PubMed] [Google Scholar]