Hirschsprung's disease, associated rare congenital anomalies.
Indian J Pediatr
;
2001 Sep; 68(9): 835-7
Artículo
en Inglés
| IMSEAR
| ID: sea-79407
ABSTRACT
OBJECTIVE:
Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease.METHODS:
A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed.RESULTS:
Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed.CONCLUSION:
Awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.
Texto completo:
Disponible
Índice:
IMSEAR (Asia Sudoriental)
Asunto principal:
Anomalías Múltiples
/
Femenino
/
Humanos
/
Masculino
/
Recién Nacido
/
Preescolar
/
Estudios Retrospectivos
/
Enfermedad de Hirschsprung
/
Lactante
Tipo de estudio:
Guía de Práctica Clínica
/
Estudio observacional
/
Factores de riesgo
Idioma:
Inglés
Revista:
Indian J Pediatr
Año:
2001
Tipo del documento:
Artículo
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