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العنوان
Clinical and Radiological Assessment of Transanal Endorectal Pull-through in children with Hirschsprung disease /
المؤلف
Selim, Ahmed Mohamed Nabil Mahmoud.
هيئة الاعداد
باحث / أحمد محمد نبيل محمود سليم
مشرف / مجدي أحمد لولح
مشرف / سمير محند حنفي كحلة
مشرف / أحمد مدحت زكي
الموضوع
surgery. Hirschsprung Disease.
تاريخ النشر
2014.
عدد الصفحات
168 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
جراحة
تاريخ الإجازة
1/10/2014
مكان الإجازة
جامعة المنوفية - كلية الطب - الجراحة العامة
الفهرس
Only 14 pages are availabe for public view

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from 168

Abstract

Harold Hirschsprung, a Danish pediatrician ,is credited with the first definitive description of the disease that bears his name. Many earlier scattered reports can be found in the literature that describe children who had symptoms and findings consistent with Hirschsprung‘s disease (HD), but these descriptions are not definitive enough to be certain of the diagnosis. Some historians give credit to Fredericus Ruysche, a Dutch anatomist, who, in 1691, described a 5-year-old girl with abdominal pain and constipation who eventually died and was noted at autopsy to have megacolon. An understanding of the cause of the constipation and megacolon in HD did not develop until the 1920s when Valla and his associates described the absence of ganglion cells in the myenteric plexus of the distal colon in two brothers with megacolon.Over the next 30 years, poorly understood procedures that were intended to reduce sympathetic hyperactivity of the colon were reported to be, at least temporarily, successful in treating HD. Drs. Swenson, Neuhauser, and Pickett recognized the spastic rectum and distal rectosigmoid colon that defined the site of obstruction in patients with congenital megacolon using fluoroscopy and a barium enema. They established the barium enema as a useful diagnostic tool in HD. Swenson performed a proximal colostomy in six patients with severe constipation and a barium enema demonstrating a classic transition zone. The colostomy was therapeutic in these patients. Later, closure of the colostomy in three of these patients resulted in recurrence of their obstructive symptoms. Based on the findings of the spastic distal colon and the careful clinical observation that the obstructive symptoms recurred with closure of a colostomy proximal to this spastic segment, Swenson conceptualized and per-formed the first pull-through procedure in a patient with HD.5 Duhamel, Soave, and others have described operations that differed from Swenson‘s but clearly integrated concepts that he defined for successful management of HD. Despite the relative success of all of these procedures, the precise etiology and many of the complexities of this disorder remain unknown. Complications can be classified as either early (weeks to months) or late (months to years). Early postoperative complications include anastomotic leak and cuff abscess, bowel obstruction, perineal excoriation, stoma complications, and wound infection. Late complications include bowel obstruction, constipation, enterocolitis, incontinence, and stricture. There is some overlap between the early and late complications.The transanal endorectal pull-through approach represents a major revolution in treating Hirschsprung disease. This procedure can be performed as a primary pull-through or staged with a prior colostomy to achieve optimal results. The anatomical domain of this approach is vast encompassing the complex pelvic area. Pathology of the disease is broad and includes aganglionosis extending from the rectum to total colonic aganglionosis . The diverse sets of pathology and anatomy become unified by a common set of complications resulting from faulty approach, resection, and reconstruction. This article focuses on the analysis of complications, avoidance and management developed from an evolving experience with 50 transanal pull-through procedures. The aim of this study was to evaluate the outcome of transanal endorectal pull-through operation clinically and radiologically in patient with Hirschsprung disease. This prospective quasi-experimental cohort study was conducted on 40 children with Hirschsprung disease operated beyond 2 years, in whom transanal endorectal pull-through was indicated. For each patient, the following was done prior to the surgical procedure: Full clinical history taking. Full physical examination.Laboratory investigations.Radiological examination. Rectal biopsy.Our results showed that;As regards abdominal distension, 14 cases showed no distension, 24 cases showed mild distension and 2 cases showed moderate to severe abdominal distension.
 History of previous attack of enterocolitis was positive in all cases. Fourteen patients (35%) had a normal defecation. Fourteen patients (35%) had a frequency of defecation of 3-5 days. Eight patients (20%) had a frequency of defecation of 6-7 days. Four patients (10%) had a frequency of defecation of 8 or more days. Thirty two patients had true fecal incontinence and had a non-dilated colon on contrast enema and a tendency to have diarrhea and did not respond to medical treatment. Four patients had partial fetal incontinence and npatients had incontinence. Postoperative soiling accidents were observed occasionally in 4 patients (1often in 18 patients (45%).< Perianal skin excoriation was absent in 36 patients (90%) and present in 4 patients only (10%). Rectal sensation was preserved in 30 patients (75%).