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Hirschsprung disease is now diagnosed neonatally, requiring adjusted management for infants. Early diagnosis and surgical treatment before two months of age lead to excellent outcomes.
Area of Science:
- Pediatric Surgery
- Neonatology
- Gastroenterology
Background:
- Hirschsprung disease diagnosis has shifted to the neonatal period.
- This demographic shift necessitates updated management strategies for affected infants.
- Previously, diagnosis often occurred after age two, with higher mortality.
Abstract:
Hirschsprung disease has become a neonatal diagnosis. Most cases identified now are children who would previously have died before the diagnosis of their condition which was usually established only after the age of two. Management of the disease should be adjusted to suit this new population of patients. Clinical features consist mainly in evidence of intestinal obstruction and enterocolitis shortly after birth and require the use of several investigative procedures since therapy cannot be initiated before the diagnosis is firmly established. Non-aggressive investigations are now available and include rectomanometry, conventional radiology, and suction mucosal biopsy of the rectum with histologic and cytologic studies of specimens. No single test is diagnostic, but taken in concert results of the different investigations are conclusive. Advances in nursing have made colostomy unnecessary and even harmful since it delays defecation by the natural route. Regardless of the technique selected (Duhamel, Soave or above all, Swenson), surgical treatment can be carried out before two months of age. A review of 90 patients, prove treatment is easy to perform at this age and yields satisfactory immediate and long term results.