• Advances in pediatric surgery
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• v.8 no.1
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• pp.48-53
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• 2002

Diagnosing Hirschprungs disease (HD) is a clinical challenge to pediatric surgeons. The cardinal symptoms are failure of passage of meconium within first 24 hours of life, abdominal distension, and vomiting. The severity of these symptoms and the degree of consitpation vary considerably between patients. HD is suspected on the basis of history and clinical findings and the diagnosis is established by radiological examination, anorectal manometry, and histochemical analysis of biopsy specimens. In this review, the advantages and pitfalls of each diagnostic modality are discussed. And a diagnostic approach utilizing these diagnostic modalities in children with suspicious HD is presented.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.39-44
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• 1999

Diagnosing Hirschprung's disease is one of the clinical challenges of this disorder. In the stomach and the intestines, Cathepsin D was readily detected in cytoplasm of the rat gastric and in intestinal ganglion cells of the autonomic nervous system. The objectives of the present study were to examine cathepsin D expression in ganglion cells of the submucosal and myenteric plexuses of the intestine of children and to determine the utility of immunohistochemical staining of cathepsin D for detection of immature ganglion cells. Paraffin blocks of 35 intestinal segments were reviewed for immunohistochemical staining with polyclonal antibody to cathepsin D and hematoxylineosin stainings from the compatible specimens. There were 9 aganglionic segments and 9 ganglionic segments of neonates with Hirschsprung's disease, 8 intestinal segments with non-Hirschsprung's disease in neonates and 9 intestinal segments with non-Hirschsprung's disease infants over the age of 10 months. All ganglion cells showed intense granular cytoplasmic reactivity for cathepsin D regardless of maturity and all aganglionic segments had no expression for cathepsin D in the submucosal and myenteric plexuses of the intestine. However, histiocytes within the laminar propria and submucosa stained positively for cathepsin D. In conclusion, intestinal ganglion cells in children have reactivity for cathepsin D, threrfore immunohistochemical staining for cathepsin D can be used for identification of ganglion cells in neonates.

• Advances in pediatric surgery
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• v.2 no.1
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• pp.33-41
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• 1996

This report present the result of the national survey of pediatric surgeons' preferences on diagnosis and treatment of Hirschsprung's disease(HD) carried out in 1993. The questionnaires were sent to twenty-seven members of the Korean Association of Pediatric Surgeons (KAPS) working in twenty-four institutions. The questionnaires were designed to determine the individual surgeon's preference for the methods of diagnosis and treatment of the disease. Twenty-three pediatric surgeons from twenty institutions returned completed forms. The total number of patients diagnosed with HD in 1992 was 190 in this group. The estimated incidence of HD was 1/3,900. The most important symptom was delayed meconium passing and the most preferred diagnostic procedure was barium study. Anorectal manometric examination was carried out by 13 pediatric surgeons and 19 confirmed the diagnosis before operation by rectal biopsy, 12 with full-thickness biopsy and 7 with suction. Frozen section biopsy during operation was done by 22 surgeons. Eight surgeons did one stage operation if the age of the patient is suitable. Definitive operation was usually done at the age of 6 to 11 months. The most preferred operation was Duhamel procedure done by 19. Enterocolitis was the most serious complication of HD. Most of patients had normal continence within 6 to 12 months after operation. The follow-up period was less than 6 years in 16 surgeons. The results were presented at the 9th annual meeting of KAPS in June of 1993. This is the first national survey of HD and it can provide guidelines of diagnosis and treatment of Hirschprung's disease even though it is not a detailed study of patient data.