Introduction Hirschsprung’s disease (HD) is a congenital disorder of the colon. Multi-staged surgeries result in complications in 20% patients and unsatisfactory results in 4%, while the irrational approach to decreasing the number of stages is fatal in 4–6% of cases.
Methods The study involved 228 children treated for different forms of HD: under 1, 29 (9.2%); 1–3, 72 (31.6%); 3–7, 66 (28.9%); and above 7, 69 (30.3%). Rectal HD was diagnosed in 63 (27.6%) patients, rectal-sigmoid, 138 (60.6%); near-total agangliosis, 21 (9.2%); and total, 6 (2.6%).
Results Radical surgery without colostomy was performed in 165 (72.4%) cases: Soave, 14; Duhamel, 5; Soave-Duhamel, 16; Lynn, 6; Swenson, 5; Rebein, 3; Boely hand-sutured primary perineal colorectal anastomosis (HSA), 56; Boley stapled primary perineal colorectal anastomosis (SA), 60.
Protective ostomy (one of the stages of treatment) was performed in 52 (22.8%) children with advanced HD. After colostomy, radical surgeries were performed upon stabilization of the patients’ health (disappearance of anemia and hypotrophy, subsidence of intoxication symptoms, and correction of the concomitant defects) within 6 months to 2 years: Swenson, 4; Rebein, 1; Duhamel, 4; Soave, 3; Soave-Duhamel, 1; Boley HSA, 6; Boley SA, 4; reconstructive plastic surgeries in severe agangliosis, 29.
Differentiated approach to treatment of HD patients resulted in a marked decrease of post-surgical mortality (0.4%) and good and (21.5–94.7%) and satisfactory (12–5.3%) outcomes.
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