Assessment of Patients with Hirschsprung Disease and the Use of Laparoscopy

Objectives: This study aims to evaluate patients who were diagnosed and treated due to Hirschsprung disease (HD) in our clinic.
Methods: We retrospectively evaluated the demographic and clinical findings of the patients with HD, who were operated in our clinic between January 2010 and December 2015.
Results: During study period, 28 patients (19 male 9 female) were found to be operated due to HD in our clinic. Mean age was 16.8 months (1-168). “Transanal Endorectal Pull–through (TERPT)” was performed to 20 of them, Duhamell procedure to five and Soave procedure to three of them. TERPT was applied as laparoscopy assisted in four of them and biopsies were taken laparoscopically preoperatively from one of the patients from each group. Soave procedure was performed in three patients; one had anal stenosis and history of recurrent enterocolitis after TERPT procedure and pathologic analysis revealed neuronal intestinal dysplasia and the other one had total colonic HD and performed Soave procedure with colonic patch. Seven (25%) patients had enterocolitis. Frequencies of enterocolitis were three in two patients, two in two patients and one in three patients. Broad spectrum anibiotics and rectal washouts were supplied to these patients. Five of the seven patients with enterocolitis were operated with TERPT; two patients were operated with Duhamell procedure. Only one of them had total colonic HD. Three patients had total colonic HD diagnosis. Two of them were operated with Duhamell-Martin procedure and one was with Soave procedure with colonic patch according to Kimura technique. Anal stenosis developed in two patients after TERPT and treated with dilatations. Soiling rate was 3% (1/28) and this single patient was treated with laxatives and toilet training. Mean duration of hospitalization was 8.75 (2-14) days. Mean length of the removed intestinal segment was 23.6 (5-38) cm. Mean follow-up was for 35.5 (2-56) months. Neither of the patients was followed in the intensive care unit postoperatively nor died.
Conclusion: TERPT procedure win priority in HD, but other procedures keep importance. Recently, laparoscopy-assisted TERPT is preferred in our clinic in HD therapy due to easy biopsy, full exposure to the transitional zone, the advantage of meso preparation of colon and prevention of strained anastomosis.

Hirschsprung Hastalarýmýzýn Genel Deðerlendirilmesi ve Laparoskopinin Yeri

Amaç: Çalýþmamýzýn amacý kliniðimizde Hirschsprung’s Hastalýðý ( HH) nedeni ile taný konulup, tedavi edilen hastalarýmýzýn sonuçlarýnýn deðerlendirilmesi.
Gereç ve Yöntem: Ocak 2010-Aralýk 2015 tarihleri arasýnda HH nedeniyle operatif tedavisi yapýlan hastalarýmýzýn demografik ve klinik bulgularý dosyasý üzerinden geriye dönük olarak deðerlendirildi.
Bulgular: 28 (19 E, 9K) hastamýz kliniðimizde HH tanýsý ile operasyonlarý yapýldý. Ortalama yaþ 16.8 (1-168) aydý. 20’sine “Transanal Endorektal Pull –through (TERPT)”, 5’ine Duhamell operasyonu, 3’üne Soave prosedürü uygulandý. 20 TERPT hastasýný 4’ü laparoskopi yardýmlý yapýldý, diðer 16’sýnýn birine ve 5 Duhamel yapýlan hastanýnda 1’ine perop laparoskopi yapýldý ve biyopsiler alýndý. Üç hastaya Soave prosedürü uygulandý. Bu hastalardan birine de laparoskopi yapýlýp preop biyopsiler alýndý, birisi TERPT sonrasý enterokolit ve darlýk geliþen patolojik analizinde nöronal intestinal displazi olan hastaydý, diðeride total kolonik HH olan kolonik patch yapýlan hastaydý. Hastalarýmýzýn hiçbirinde postop yoðun bakým ihityacý ve eksitus olmadý. Hastanede kalýþ süresi ortalama 8.75 (2-14) gündü. Patoloji raporlarýndan alýnan sonuçlara göre çýkarýlan barsak segmenti ortalama uzunluðu 23.6 (5-38 ) cmdi. Enterokolit sýklýðý %25 di. Enterokolit geliþen 7 hastamýzýn 5 (% 25)’ine TERPT, 2 (%40)’sine Duhamel yapýlmýþtý. Bu hastalarda sadece biri total kolonik HH’dý. Total kolonik hastalarýnýn 2’sine Duhamell-Martin, 1’ine Kimura yöntemi ile hazýrlanan kolonik yama ile Soave prosedürü uygulandý. Enterokolit sýklýðý iki hastada 3 kere, iki hastada 2 kere, üç hastada 1 kere geliþti. Bu hastalarýn tedavileri rektal yýkama ve geniþ spektrumlu antibiyotik ile yapýldý. Anal darlýk TERPT yapýlan 2 hastamýzda geliþti dilatasyonlara cevap verdi. Gayta inkontinans oraný %3 (1/28)’dü, laksatifler ve tuvalet eðitimi ile tedavi edildi. Hastalarýn ortalama takip süresi 35.5 (2-56) aydý.
Sonuç: Hirschsprung Hastalýðýnýn tedavisinde TERPT yöntemi öncelik kazanmýþ olup diðer prosedürler de önemini korumaktadýr. Laporoskopi yardýmlý TERPT’de biyopsi almadaki kolaylýðý, geçiþ zonunun görülmesindeki faydasý, mezo hazýrlamadaki avantajý ve gergin olmayan anastomoz yapmayý saðladýðý için kliniðimizde tedavide yerini almýþtýr. (SETB-2018-07-096)