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: 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.

Introduction: Constipation is one of the most common causes of referral to pediatric gastroenterology clinics. The histopathology and Immunohistochemistry (IHC) studies indicated that several neurons are currently detectable in motility of the gut. We aimed to evaluate calretinin in the children with chronic constipation. Materials and Methods In this cohort study, 40 children with chronic constipation, 28 boys (70%) and 12 girls (30%) referring to pediatric surgery ward in Shahid Mohammadi Hospital, Bandar Abbas-Iran, between January 2010 to February 2012, were recruited. Then, full rectal biopsy 1.5 cm above dentate line was performed for all children and calretinin immunoreactivity and pattern of staining for ganglion cells (nuclear and cytoplasmic) and also nerve fibers in different layers of the bowel (lamina propria, muscularis mucosa, submucosa, and muscularis propria) were measured. According to their pathologic results, the children were assigned in two groups for treatment: 1- Those with aganglionosis underwent pull- through operation, and 2- Patients with ganglion but abnormality in their immunohistochemistry received botulinum toxin. Results In this study, 9 (23.9%) AGANGLIONIC patients with the mean age of 3.6±1.7 years compared with 31 (76.1%) hypoganglionosis patients with the mean age of 3.2±1.2 years were examined. Pull- through operations were carried out for all patients in the AGANGLIONIC group, and for 6 (19.4%) patients in the hypoganglionosis group. Postoperative manometry was significantly better in both groups, but monomeric change was not significant between the two groups. Conclusion Our results revealed that calretinin is absent in AGANGLIONIC children, moreover we indicated, calretinin IHC is a very useful and valuable technique for detecting aganglionosis in patients with chronic constipation.

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.

To access the surgical progress of patients with long segment aganglionosis and long-term evaluation. During the past 15 years, we reviewed the therapy and outcome of 15 patients with Long-Segment Agangelionosis (LSA) in Mofid children's hospital. 2 with Agangelionosis to the midtransvers colon (MTA), 8 to the ascendant and cecum (A/CA), 4 with total colonic and distal ileum involvement (TCA), and one child with total intestinal Agangelionosis (TIA). The patient with TIA had only laparatomy and staged biopsies. 12were treated by Swenson technique and 2 patients by other procedures. All patients received total parenteral nutrition up to 10 days postoperative to ensure appropriate fluid and electrolyte status. The patients with TCA needed supplementary therapies (Diet, Drugs) up to 4 months to reduce the stool frequency. 2 patients died. The patient with TIA died 6 months after Biopsies. Another one died 5 months after pull-through: small bowel ileus with total intestinal necrosis. 9 Of the 13 alive patients have normal stool pattern: 1-3 times per day. 2 patients have 3-6 stools per day with 1-2 soiling per week. An adolescent patient has occasionall  nightly soiling (1- 2 per month). No actual data about one patient (A/CA). One TCA patient doing well immediately postoperative becomes obstipated 3 months later (after having Entrocolitis). He had washout enemas for 9 months. Now stools regularly. Awareness of long-segment Agangelionosis should lead to earlier diagnosis. Appropriate surgery and improvements in supportive care led to a significantly increased rate of survival. Patients with LSA even involving ileum (but no more than 50 cm of ileum) should attain a normal stooling behavior. Long-term outcome is quite favorable.

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.