Background-Hirschsprung’s disease has several surgical treatments, including curative surgery which consists of total or subtotal resection of the aganglionic segment followed by a pull-through of the normally innervated intestine. This study intends to assess the aftermath of two-stage endorectal pull-through procedures. Methods-Medical records of 62 patients (46 males, 16 females), who were treated for Hirschsprung’s disease during a 10-year period, were reviewed. Age, sex, extent of aganglionosis, primitive surgical procedure, age at colostomy, age at definitive procedure, number of stages, immediate and late postoperative complications, postoperative causes of death and time of the first postoperative defecation were retrieved in all patients. Results- Results showed that 61 patients had colostomy or ileostomy as the primitive procedure. Two-stage Soave Boley procedure was done in 54 cases; in 32 (52.4%) patients the Soave-Boley procedure was followed by appendectomy and tube cecostomy and in 22 (36%) patients, Soave-Boley pull-through was done without appendectomy and tube cecostomy. The results of the definitive operations were promising in 90.1% of the patients who gained normal defecation. Early complications were met in 9 cases (14.7%) and late complications were present in 14 cases (22.9%). The mortality rate was 4.9% (3 patients); the first patient had sepsis following ileostomy, the second patient suffered from enterocolitis 6 months post-operatively and the third patient died four days after treatment. Conclusions- By eliminating one of the stages in the surgical procedure, superior results with less complications are achieved. This in turn precludes the use of costly staplers. Altogether, the Soave-Boley procedure has proved to be a cost-effective and desirable procedure in comparison to other techniques in the treatment of Hirschsprung’s disease.

Objective: Successful results after one-stage trans-anal pull-through (OSTAPT) operation for Hirschsprung's disease (HD) depend on accurate identification of the aganglionic segment in intraoperative frozen section (FS). Misinterpretation of the findings of the rectal biopsy is an anxietyevoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section (PS) rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation.Methods: Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children’s hospital for open rectal biopsy to diagnose HD were included in the study. All biopsies were taken 2-3 cm above the dentate line.210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients, examined by two well experienced pediatric pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5.Findings: Two-hundred one infants and children underwent FS rectal biopsy to exclude HD.Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples.93.9% of positive results in FS studies were confirmed by PS studies.6.1% of FS reports were false positive and 21.7% were false negative (P<0.001). The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value (PPV) was 93.9% and negative predictive value (NPV) was 78.3% in FS studies (P<0.001). The accuracy of FS was 80.4%.Conclusion: Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base for performing primary pullthrough operation before the results of the permanent section are on hand.

Background: Early diagnosis and repair make a better prognosis in Hirschsprung's disease (HD) patients. Two basic approaches, namely single staged and multiple staged pull through are commonly applied to treat such patients. In this study we tried to compare short-term and long-term complications of the two procedures to provide a guide for choosing the safer and more effective approach.Methods: The study involved all the HD cases treated via either of the two common approaches during a seven-year period from 1995 to 2002 in Isfahan, Iran. Fourteen patients underwent single-staged repair (SS) but the remaining 48 were treated via a multiple-staged approach (MS). Short-term and long-term complications together with patients' defecation patterns were compared between the two groups.Results: Early wound infection was significantly more prevalent among MS group (9 (19%) in MS groups vs. 1 (7%) in SS group; P < 0.05). Intestinal obstruction, severe enterocolitis, and anastigmatic stricture were considerably lower in SS repair than in MS procedure. Defecation patterns in both groups were comparable. Compared with the MS approach, the number of hospital admissions and total length of stay were significantly lower in the SS group.Conclusions: Although restoration of normal function is achieved with both procedures, early and late complications, number of admissions and the total length of hospital stay are considerably lower with the SS pull through. Therefore, with an earlier diagnosis, SS repair can be an improved strategy, which will bring a better prognosis for HD patients.

Deiverting ileostomy was initially introduced and used by Turnbull and Weakley to provide fecal diversion in patients with toxic megacolon. Traditionally, transverse colostomy has been the preferred method for temporary proximal diversion in patients with colonic obstruction. However, recently, diverting ileostomy has been popularized for proximal diversion, mainly for lack of an offensive odor of the ileostomy effluent compared with fecal material from a colostomy. We have used diverting ileostomy routinely for protection of ileorectal anastomoses, especially when tension exists at the site of anastomoses or for those patients who have been on steroids.

Background: Hirschsprung disease is a complex genetic disorder of the enteric nervous system (ENS), often called congenital aganglionic megacolon and characterized by the absence of enteric neurons along a variable length of the intestine. The definitive diagnosis of Hirschsprung disease relies on histologic and/or histochemical staining of sections from suction rectal biopsies. Calretinin immunohistochemistry (IHC) may be a useful in its diagnosis. This study aimed to proof the usefulness of immunohistochemical staining for calretinin in rule out of Hirschsprung disease. Methods: Paraffin blocks and slides were retrieved from the pathology archives of Ali Asghar Hospital, Tehran, Iran from 2007 to 2011 with pathology report based on the presence (14 patients) or absence (70 patients) of ganglion cells and transitional zone anatomical region (10 patients). Slides were stained with hematoxylin and eosin method to confirm the initial diagnosis was verification again. After preparing the slides, they were stained by IHC for calretinin. Then, the results were analyzed using SPSS software. Results: In most patients, IHC for calretinin provided highly compatible results with hematoxylin-eosin findings in diagnosis of Hirschsprung disease. The values of specificity and accuracy between calretinin and standard histology (H&E) compared by the Fisher exact test declared calretinin presented significantly higher specificity and accuracy values than H&E staining (P <0. 0001). Conclusion: Calretinin IHC overcomes most of the difficulties encountered using the histology hematoxylin-eosin. Then, IHC for calretinin is a good ancillary method used by pathologists in diagnosis of Hirschsprung disease.