<< Back Next >>
Rev Mex Cir Pediatr 2010; 17 (3)
Nieto-Zermeño J, Dávila-Pérez R, Tovilla-Mercado JM, Bracho-Blanchet E, Herappe-Mellado D
Language: Spanish
References: 42
Page: 127-143
PDF size: 589.12 Kb.
ABSTRACT
Introduction: Hirschsprung’s disease (HD) is characterized by absence of ganglion cells (GC) in a variable length of the colon. The gold standard diagnosis of EH is the full thickness rectal biopsy, although some authors consider the suction biopsy as the gold standard (consensus 2005). Anorectal manometry (MAN) is a noninvasive procedure that demonstrates the absence of rectoanal inhibitory reflex (IIRR) in the cases of HD. In recent publications are regarded MAN no diagnostic value in HD.
Material and Methods: A retrospective, observational, included all pediatric patients with suspected HD and counted with MAN and the diagnostic gold standard for our hospital (total thickness colorectal biopsies) from 1984-2008. 2x2 tables are made between the result of man vs. the result of the biopsy (gold standard) to determine the real value of the MAN as a diagnostic test (sensitivity, specificity, positive predictive value and negative, false positive and negative).
Results: Of a total of 455 MAN performed in our hospital from 1984 to 2008, 267 were in patients in our hospital and the rest were external consults. Of these 267 MAN, 79 counted gold standard diagnosis (intestinal biopsy), 55 and 24 did not show whether IIRR IIRR showed, the first in 52 found the absence of ganglion cells confirming the diagnosis of EH, was observed in 3 ruling out the presence of ganglion cells of HD. Of the second 24 (presence of IIRR), 100% showed presence of ganglion cells in the histological study discarding EH. In no patient of the latter group showed absence of ganglion cells in the histological study.
The MAN as a diagnostic test comprehensively demonstrates a sensitivity of 100%, specificity 88%, positive predictive value of 94% and negative predictive value 100%, and 5.4% false positive and false negative 0% compared with the standard gold. By grouping patients under and over two years are: children under 2 years showed that the MAN has a sensitivity of 100%, specificity 81%, 91% positive predictive value, negative predictive value 100%, 3 false positives and no false negatives, and over 2 years showed a sensitivity of 100%, specificity 100%, 100% positive predictive value, negative predictive value 100%, and no false positive nor negative.
Discussion: MAN in our experience is a key diagnostic pillar to help confirm EH with high sensitivity and specificity but also because of its ability to rule by EH negative predictive value 100%, the above is particularly noticeable in older patients 2 years.
Although the diagnostic gold standard in our hospital continues to be the full thickness intestinal biopsy because of the advantages offered by the MAN (less invasiveness, ease of implementation, and extensive experience in our department), the latter maintains a dominant role in the diagnosis EH, it is worth mentioning that the man in our hospital has been conducted in a standardized manner since 1984 and in 95% of the cases is done by the same staff, which gives a margin of safety to the test very high since technical standpoint.
In our study is very important to note that compared to the MAN in 100% of the cases against the diagnostic gold standard and our 2x2 table is perfectly reproducible, which is considered vital in the meta-analysis impartancia giving an important validity to our study
REFERENCES
McReady R, Beart R. Classic articles in colonic and rectal surgery. Disease Colon Rectum 1981, 24, 408-410.
Haricharan R, Georgeson K. Hirschsprung’s Disease. Seminars in Pediatric Surgery 2008, 17, 266-275.
Orr J, Scobie W. Presentation and incidence of Hirschsprung’s Disease. British Medical Journal 1983, 287, 1671-1673.
Martuciello G et al. Total colonic agangliosis associated with intersticial deletion of the long arm of chromosome 10. Journal of Pediatric Surgery 1991, 7, 308-310.
Puri P, Shinkai T. Pathogenesis of Hirschsprung’s disease and its variants: recent progress. Seminars in Pediatric Surgery 2004, 13, 18-24.
Ure B, Holschneider A, Meier W. neuronal intestinal malformations: A retro- and prospective study on 203 patients. European Journal Pediatric Surgery, 1994, 4, 279-280.
Caniano D, Qualman S. Management of Hirschsprung’s Disease in children with trisomy 21. American Journal Surgery, 1990, 159, 402-404.
Swenson O, Sherman J, Fisher J. Diagnosis of congenital megacolon: An analysis of 501 patients. Journal of Pediatric Surgery, 1973, 8, 587-594.
Clark D. Times of first void and first stool in 500 newborns. Pediatrics 1977, 60, 457-459.
Vietnem D, Spicer R. Enterocolitis complicating Hirschsprung’s disease. Seminars in Pediatric Surgery 2004, 13, 263-272.
Suita S, Taguchi T. Hirschsprung’s Disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. Journal of Pediatric Surgery 2005, 40, 197-201.
Swenson O. Hirschsprung’s disease: A review. Pediatrics 2002, 109, 914-918.
Lorijin F, Kremer L, Reitsma J. Diagnostic test in Hirschsprung’s Disease: A systematic Review. Journal of Pediatric Gastroenterology and Nutrition 2006, 42, 496-505.
Lorijin F, et al. Diagnosis of Hirschsprung’s Disease: a prospective, comparative accuracy study of common test. The Journal of Pediatrics 2005, 146, 787-792.
Klein M, Philippart A. Hirschsprung’s disease: Three decades experience at a single institution. Journal of Pediatric Surgery 1993, 28, 1291-1293.
Rosenfield N et al. Hirschsprung’s Disease: Accuracy of the barium enema examination. Radiology 1984, 150, 393-400.
Sun W, Rao S. Manometric assessment of the anorrectal function. Gastroenterology Clinics of North America 2001, 30, 15-32
Schnaufer L, et al. Differential sphincteric studies in the diagnosis of ano-rectal disorders of childhood. Journal of Pediatric Surgery, 1967, 2, 538-543.
Aaronson I, Nixon H. A clinical evaluation of anorectal pressure Studies in the diagnosis of Hirschsprung’s disease. Gut, 1972, 13, 138-146.
Shafie M, Suzuki H, Schnaufer L, Haller A, White J. A simplified method of anorectal manometry for wider clinical application. Journal of Pediatric Surgery 1972, 7, 230-235.
Mishalany H, Suzuki H, Yokoyama J. Report on the first international Symposium on anorrectal manometry. Journal of Pediatric Surgery 1989, 24, 356-359.
Kawahara H, et al. Anorrectal sleeve micromanometry for the diagnosis of Hirschsprung’s disease in newborns. 2007, 42, 2075-2079.
Swenson O, Fisher J, Gherardi G. rectal biopsy in the diagnosis of Hirschsprung’s Disease. Surgery, 1959, 45, 690-695.
Pini-Prato A et al. Rectal suction biopsy in the diagnosis of intestinal dysganglionoses: 5-year experience with Solo RBT in 389 patients. Journal of Pediatric Surgery, 2006, 41, 1043-1048.
Noviello C, Cobellis G, Papparella A, Amici G. Role of anorectal manometry in children with severe constipation. Colorectal Disease 2008, junio.
Meunier P, Mollard P, Jaubert M. Manometric studies of anorectal disorders in infancy and childhood: an investigation of the physiopathology of continence and defaecation. British Journal Surgery 1976, 63, 402-407.
Suzuki H, White J, El Shafle M, Shaker I, Haller A. Nonoperative diagnosis of Hirschsprung’s disease in neonates. Pediatrics 1973, 51, 188-191.
Tamate S, et al. Manometric diagnosis of Hirschsprung’s disease in the neonatal period. Journal of Pediatric Surgery 1984, 19, 285-288.
Frenckner B, Molander M. Activity of the internal anal sphincter during the first days of life. Acta Pediatric Scand. 1980, 69, 73-77.
Benninga M, et al. Characterizacion of anorectal pressure and the anorectal inhibitory reflex in healthy preterm and term infants. The Journal of Pediatrics 2001, 139, 233-237.
Lorijin F, et al. Diagnosis of Hirschsprung’s disease: a prospective, comparative accuracy study of common test. The Journal of Pediatrics 2005, 787- 792.
Keshtgar A, et al. Diagnosis and management of children with intractable constipation. Seminars in Pediatric Surgery, 2004, 13, 300-309.
Ryuichiro H, Yuzo H, Takaharu Y. The Simple technique of rectal mucosal biopsy for the diagnosis of Hirschsprung’s disease. Journal of Pediatric Surgery 1993; 28, 942-944.
Martuciello G, et al. Controversies concerning diagnosis guidelines for anomalies of the enteric nervous system: A report from the fourth International Symposium on Hirschsprung’s disease and related neurocristopathies. Journal of Pediatric Surgery 2005, 40, 1527-1531.
Madonna M et al. Swenson procedure for the treatment of Hirschsprung’s Disease. Seminars in Pediatric Surgery 1998, 7, 85-88.
Swenson O: My early experience with Hirschsprung’s Disease. Journal Pediatric Surgery 1989, 24, 839-844.
Mattioli G, et al. Results of a mechanical Duhamel pull-through for the treatment of Hirschsprung’s Disease and intestinal neuronal dysplasia. Journal of Pediatric Surgery, 2004, 39, 1349-1355.
Stockmann P, Philippart A. The Duhamel procedure for Hirschsprung’s Disease. Seminars in Pediatric Surgery, 1998, 7, 89-95.
Weinberg G, Boley S. Endorrectal Pull-through with primary anastomosis for Hirschsprung’s Disease. Seminars in Pediatric Surgery, 1998, 7, 96-102.
De la Torre L, Ortega A. Transanal versus open endorrectal pull-through for Hirschsprung’s Disease. Journal of Pediatric Surgery 2000, 35, 1630-1632.
De la Torre L, Ortega J. Transanal endorrectal pull-through for Hirschsprung’s disease. Journal of Pediatric Surgery, 1998, 33, 1283-1286.
Jona J, et al. Laparoscopic pull-through procedure for Hirschsprung’s Disease. Seminars in Pediatric Surgery, 1998, 7, 228-231.