Özet:

Long segment Hirschsprung disease (LSHD) or total colonic agangliosis (TCA) is one of the rarest forms of Hirschsprung disease (HD). It was among high mortality diseases since near future. Better understanding of clinical symptoms, early diagnosis with the help of radiological imaging, development of proper surgical techniques and methods for dietary supplementation have decreased mortality and morbidity in time. Every we present a mature newborn male patient suffering from abdominal distention and vomiting. HH was suspected and agangliosis was determined in rectal biopsy specimens. Transanal endorectal pull through (TEPT) was performed and TCA was diagnosed from biopsy specimens taken during TEPT. Ileal pull through and ileostomy was performed and metabolic problems occurring due to loss from ileostomy after operation was managed with collaboration of department of metabolic diseases. Patients who are operated for definitive operations for TCA and who have ileostomy are prone to water-electrolyte imbalance and have high morbidity. In order to decrease mortality and morbidity, enteral nutrition and multidisciplinary approach are important.

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