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Diagnosis and outcome of Hirschsprung's disease: does age really matter?
D J Hackam1, K K Reblock, R E Redlinger
1Division of Pediatric Surgery, Children's Hospital of Pittsburgh, Department of Surgery, University of Pittsburgh School of Medicine, PA, USA.
Insights
Older children diagnosed with Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease (HD) is typically diagnosed in newborns.
- Presentation and management in older children are not well-defined.
- Older patients may represent a milder disease variant.
Purpose of the Study:
- To compare outcomes in early-diagnosed versus late-diagnosed Hirschsprung's disease.
- To investigate if delayed diagnosis impacts prognosis in pediatric HD patients.
Main Methods:
- Retrospective study of 66 pediatric Hirschsprung's disease patients (1995-2001).
- Patients divided into Group I (diagnosed <30 days) and Group II (diagnosed >30 days).
- Total colonic disease cases were excluded.
Main Results:
- Group II patients (older diagnosis) presented with different symptoms and shorter aganglionic segments.
- Surgical strategies were similar across groups.
- Complication rates were equal, but Group II had shorter hospital stays and lower costs.
Conclusions:
- Delayed diagnosis of Hirschsprung's disease does not worsen outcomes in older children.
- Older children with HD may have a milder form of the disease.
- Adaptation to the aganglionic state might explain better outcomes in late-diagnosed cases.
Abstract:
Although Hirschsprung's disease (HD) typically presents in the newborn period, it is often diagnosed in older children, in whom the presentation and management remain poorly defined. We hypothesized that older patients with HD have a milder variant of the disease with an improved prognosis compared with those diagnosed earlier. Children with HD (1995-2001) were divided into Group I (diagnosis before 30 days) and Group II (after 30 days). Patients with total colonic disease were excluded. There were 66 patients; 47 in Group I and 19 in group II. Mean age at diagnosis was 7.1+/-1.3 days (range 1-30 days) versus 27+/-10 months (1.3 months-19 years). Older children differed mainly in the symptoms at presentation and the length of the involved segment of aganglionosis. Surgical strategies were applied equally in both groups. Complications, including postoperative enterocolitis, occurred equally, but the length of stay and costs were lower in Group II. The delayed diagnosis of HD does not worsen outcomes of older children with HD. This finding implies that these children have a milder form of the disease, perhaps because of adaptation to the aganglionic state.