Altamirano E, Drut R

Language: Spanish
References: 4
Page: 351-353
PDF size: 271.31 Kb.

ABSTRACT

In this short report we are presenting the peculiar finding in a 2 year-old girl of total aganglionosis (absence of intramural plexi in rectum, colon and a segment of the small bowel) combined with segmental Classic aganglionosis (absence of ganglion cells and presence of nerve fibers). The association is extremely unusual and the usefulness of its appropriate recognition stems in the difficulties to interpret it using the standard criteria when evaluating a patient suspected of having Hirschsprung’s disease.

REFERENCES

1. Qualman SJ, Pysher T, Schauer G. Hirschsprung’s disease: diferential diagnosis and sequelae. Perspect Pediatric Pathol 1997;20:111-26.

2. Negreen D, Young HM. Enteric nervous system: developmental disturbances-Part 1. Pediatr Dev Pathol 2002;5:224- 47.

3. Yunis E, Sieber WK. Does zonal aganglionosis really exist?Report of a rare variety of Hirschsprung’s disease and review of the literature. Pediatric Pathol 1983;1:33-49.

4. Kapur RP, deSa DJ, Luquette M, Jaffe R. Hypothesis: pathogenesis of skip areas in long-segment Hirschsprung’s disease. Pediatr Pathol Lab Med 1995;15:23-37.