Hirschsprung hastalığı. 18 yıllık deneyimimiz

Amaç: Çalışmadaki amacımız kliniğimizde Hirschsprung hastalığı (HH) tanısı koyularak çoğunluğu Duhamel ameliyatının Martin modifikasyonu ile tedavi edilen hastalarımızın tam ve tedavi özelliklerini belirlemek, morbidite, mortalite ve uzun dönem fonksiyonel sonuçlar açısından değerlendirmektir. Yöntem: Haziran 1983 ile Şubat 2001 tarihleri arasında HH tanısı alan 64 hasta yaş, cinsiyet, başvuru zamanı, semptom ve bulgular, konjenital anomaliler, aganglionik barsak uzunluğu, tedavi metodu, fonksiyonel sonuçlar, morbidite ve mortalite açısından geriye dönük değerlendirildi. Bulgular: Hastaların 52'si erkek, 12'si kız idi. Olguların % 37'si yaşamın ilk bir ay'ı içerisinde tanı aldı. Başvuru şikayeti en sık karında şişlik (% 34) idi. Olguların 13'ünde (% 20) ek konjenital anomali saptandı. Aganglionik barsak en fazla rektosigmoid (% 82) bölgede görüldü. Enterostomi 56 hastaya uygulandı. Enterostomi sonrası 7 hasta Soave-Boley, 41 hasta Duhamel-Martin yöntemi ile tedavi edildi. Dört hastaya enterostomisiz tek seans Duhamel-Martin uygulandı. Ameliyat sonrası 16 (% 25) hastada komplikasyon gelişti. En sık gelişen komplikasyon intraabdominal yapışıklığa bağlı intestinal obstrüksiyon idi (% 33). Definitif ameliyat öncesi 3 (% 2) ve sonrası 5 (% 9) hastada enterokolit gözlendi. Hastalarımızın 8'i (% 12) öldü bu hastaların birinde ek konjenital anomali ve 6'sında sepsis vardı. Definitif operasyonu yapılan bir hastamız enterokolit atağı sonrası kaybedildi. Fonksiyonel skor 10-15 ve 15 yaş üzeri grup da 5-10 yaş grubu hastalara göre anlamlı derecede daha iyi idi (p

Hirschsprung's disease: An 18-years experience

Aim: In this study, we aimed to analyse the diagnostic and therapeutic features, morbidity, mortality and long-term functional results of patients with Hirschsprung's disease, whom the majority were treated by Duhamel-Martin procedure. Method: A total of 64 Hirschsprung's disease cases were diagnosed and treated in the department between June 1983 and February 2001. We reviewed the charts of these retrospectively and evaluated the age, sex, symptoms and signs, associated anomalies, the length of bowel involved, type of pull-through performed, functional results of treatment, morbidity and mortality of these patients. Results: There were 52 boys and 12 girls. Thirty seven percent, of the cases were diagnosed below the age of one month. The most frequent presenting symptom was abdominal distention (34 %). Thirteen of the case's had associated anomalies (20 %). The most frequent site of aganglionosis was at the rectosigmoid (82 %). A decompressing enterestomy was performed in 56 patients. Soave-Boley's and Duhamel-Martin's technique with protective colostomy were, performed in 7 and 41 patients respectively. Postoperative complications were seen in 25 % of the patients. In 4 cases a Duhamed-Martin's procedure was performed without a colostomy. In the postoperative period 16 cases (25 %) developed a complication. The most frequent complication was intestinal obstruction due to adhesions (33 %). Preoperative and postoperative enterocolotis was found in three (2 %) and 5 (9 %) patients respectively. Eight children (12 %) died, one related to associated anomalies and six due to sepsis one patient died from enterocolitis after the definitive procedure. In the 10 to 15 year age and the greater than 15 year age groups, functional scores were significantly increased when compared to the 5 to 10 year age group (p<0.05). A significant correlation was not found between the functional score and age of the patients. Conclusion: As a conclusion, we have used Duhamel-Martin procedure in majority of patients with Hirschsprung's disease. Long-term functional evaluations have shown an improvement in functional score in children older than 10 year in comparison with younger ones.

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