Pull-through Procedure Research Articles

Index of Suspicion

* ALT: : alanine aminotransferase AST: : aspartate aminotransferase BUN: : blood urea nitrogen CBC: : complete blood count CNS: : central nervous system CSF: : cerebrospinal fluid CT: : computed tomography ECG: : electrocardiography ED: : emergency department EEG: : electroencephalography ESR: : erythrocyte sedimentation rate GI: : gastrointestinal GU: : genitourinary Hct: : hematocrit Hgb: : hemoglobin MRI: : magnetic resonance imaging WBC: : white blood cell An 11-year-old boy presents to the emergency department with a 1-week history of frequent episodes of nonbloody, nonbilious emesis associated with diarrhea and abdominal cramping. He has urgency and fecal incontinence. He has been afebrile and denies any rashes, joint pain, sick contacts, headaches, or other symptoms. On further questioning, his mother reveals that he has had 2 similar episodes of abdominal distension and diarrhea during the past 6 to 7 years. Between these episodes, she states that he normally has nonbloody, hard stools accompanied by straining and chronic abdominal pain and pain with defecation. When asked specifically, his mother does not remember when he passed his first stool after he was born. On physical examination, his temperature is 37.6°C, pulse is 100 beats per minute, respiratory rate is 16 breaths per minute, blood pressure is 113/62 mm Hg, oxygen saturation is 100% on room air, height is 63 inches (95%), and weight is 36.7 kg (25%). He has abdominal distention and diffuse abdominal tenderness with voluntary guarding. There is palpable stool in the left quadrant. There is no perianal erythema or skin tag, and rectal examination reveals a low anal tone. A CT scan of the abdomen (Figure 1) reveals a markedly distended rectosigmoid colon with circumferential, confluent, and contiguous mural thickening. Basic metabolic profile, CBC, and urinalysis results are unremarkable, but the C-reactive protein level is elevated at 131 mg/L (1248 nmol/L). Because of the concern for enterocolitis, he is empirically given broad-spectrum intravenous antibiotics and transferred to a children’s hospital for further evaluation. Figure 1. A CT scan of the abdomen shows a markedly distended rectosigmoid colon with circumferential mural thickening. A 4-year-old boy presents for evaluation of intermittent fevers of 4 weeks’ duration after 2 separate antibiotic courses prescribed for acute otitis media. He has experienced clear rhinorrhea and …

Spectrum of Esophageal Tumors

HomeRadioGraphicsVol. 33, No. 4 PreviousNext DepartmentsFree AccessIlluminationsSpectrum of Esophageal TumorsAletta Ann FrazierAletta Ann FrazierAuthor AffiliationsAmerican Institute for Radiologic Pathology, Silver Spring, Maryland, and University of Maryland School of Medicine, Baltimore, Maryland ([email protected]).Aletta Ann FrazierPublished Online:Jun 29 2013https://doi.org/10.1148/rg.332135910MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked InEmail Typical growth patterns and locations of esophageal tumors include an ulcerative squamous cell carcinoma involving the midesophagus (far left); an infiltrative adenocarcinoma associated with a hiatal hernia and involvement of the gastroesophageal junction (center left); an intraluminal, polypoid spindle cell carcinoma arising in the middle to lower esophagus (center); an intramural leiomyoma centered within the smooth muscle portion of the esophagus (center right); and an elongated, pedunculated fibrovascular polyp arising from the cervical esophagus (far right).Download as PowerPointOpen in Image Viewer The artist thanks Angela D. Levy, MD, for providing reference material for the illustration.Article HistoryPublished online: June 29 2013Published in print: July 2013 FiguresReferencesRelatedDetailsRecommended Articles Role of Multimodality Imaging in Gastroesophageal Reflux Disease and Its Complications, with Clinical and Pathologic CorrelationRadioGraphics2020Volume: 40Issue: 1pp. 44-71Clinical, Imaging, and Pathologic Features of Conditions with Combined Esophageal and Cutaneous ManifestationsRadioGraphics2019Volume: 39Issue: 5pp. 1411-1434Uncommon Intraluminal Tumors of the Gallbladder and Biliary Tract: Spectrum of Imaging AppearancesRadioGraphics2019Volume: 39Issue: 2pp. 388-412Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and ManagementRadioGraphics2019Volume: 39Issue: 5pp. 1280-1301Esophagectomy and Gastric Pull-through Procedures: Surgical Techniques, Imaging Features, and Potential ComplicationsRadioGraphics2016Volume: 36Issue: 1pp. 107-121See More RSNA Education Exhibits Upper Gastrointestinal Series (UGI): From the Normal Anatomy to the Main Pathologies Imaging FeaturesDigital Posters2018Hanging with Polyps from Head to Toe: Radiologic Imaging and Follow-upDigital Posters2020Are Gastrointestinal (GI) Series a Thing From the Past? The Importance of Fluoroscopy in the Diagnosis of Gastric Pathology: A Pictorial Review of GI Series with a 2020 VisionDigital Posters2020 RSNA Case Collection Esophageal Granular Cell Tumor RSNA Case Collection2022Esophageal Pseudodiverticulosis RSNA Case Collection2021Hiatus hernia with chronic organoaxial volvulusRSNA Case Collection2020 Vol. 33, No. 4 Metrics Downloaded 3,373 times Altmetric Score

Congenital anorectal atresia: MR imaging of late post-operative appearances in adult patients with anal incontinence

To describe the MR imaging findings in adults presenting with anal incontinence following pull-through perineoplasty for anorectal atresia. 15 adults (12 male, 3 female; age 22-52 years) with anal incontinence following a prior perineal pull-through procedure as an infant for anorectal atresia were identified retrospectively. MR imaging was performed using either an endoanal coil or body coil. MR images were reviewed by three observers who noted whether pelvic floor and sphincter muscles were present and, if so, whether they were thinned or not. Data were tabulated and raw frequencies determined. Images were unavailable for one patient, leaving 14 for analysis. Anal stenosis prevented endoanal coil placement in 5. The pull-through was anatomically correct in 12 (86 %) patients but was misdirected in 2. Thinned muscle was seen in 11 (79 %) patients. External sphincter thinning was commonest (present in 10 patients), with levator plate thinning least common (present in 4 patients). Only one patient had thinning of all muscle groups. MR imaging may be used to determine the extent and quality of residual pelvic floor and anal sphincter muscle in adults who have functional disability following pull-through perineoplasty for anorectal agenesis.

Surgical management of the base of tongue

Lesions of the base of tongue (BOT) present a considerable challenge to the head and neck surgeon. This area represents a 3-dimensional anatomic region that is integral to both speech and swallow functions in addition to forming an important part of the upper respiratory tract. Surgical access to this region is difficult, and is primarily dictated by the size and type of lesion involving the BOT. The head and neck surgeon must be adept in the full spectrum of surgical techniques available to access the BOT, while keeping in mind the associated impact of each procedure on both form and function. Such techniques include transoral procedures, suprahyoid pharyngotomy, lip-split mandibulotomy or mandibular swing, and pull-through procedures. This article describes these techniques and considers the advantages and disadvantages of each.

Totally Transanal LESS Pull-Through Colectomy: A Novel Approach for Avoiding Abdominal Wall Incision in Children with Long-Segment Intestinal Aganglionosis

Abstract Introduction: Minimally invasive surgery in children with long-segment intestinal aganglionosis aims to reduce the number of abdominal wall incisions. Conventional laparoscopic and laparoendoscopic single-site (LESS) surgeries fulfill this goal. In children, natural orifice translumenal endoscopic surgery (NOTES™; American Society for Gastrointestinal Endoscopy [Oak Brook, IL] and Society for American Gastrointestinal and Endoscopic Surgeons [Los Angeles, CA]) has been limited because of fear of access site complications. We present a novel technique of totally transanal LESS pull-through colectomy (TLPC), avoiding abdominal wall incision, which combines LESS technology and the NOTES approach. Two boys and one girl (2.5 months, 6 months, and 5 years of age, respectively) with sigmoid and transverse colon aganglionosis underwent surgery. The TLPC procedure consisted of an endorectal technique with submucosal dissection starting 1 cm orally from the dentate line to above the peritoneal reflection, where the rectal muscle was divided circumferentially. After ligation of the rectal mucosa, the proximal bowel was replaced into the abdominal cavity, and a TriPort(®) (Olympus Surgical Technologies Europe, Hamburg, Germany) was introduced transanally. Mesenterial resection of the aganglionic bowel was accomplished via transanal LESS until the normoganglionic colon segment was reached and pulled down to the site of anastomosis. After removal of the port, a conventional pull-through procedure was performed. All children displayed normal bowel movements and were complication-free during the follow-up period of up to 7 months. TLPC combines the minimally invasive LESS surgery with the scarless concept of NOTES and allows resection of long-segment aganglionosis without abdominal incision. TLPC is a safe, effective, and feasible surgical procedure in children with long-segment intestinal aganglionosis.

Changes in Hospital Utilization and Management of Hirschsprung Disease

This study investigates how the epidemiology, hospital utilization, and surgical management of Hirschsprung disease (HD) have changed over the last decade in the United States, using a statistically valid national sample. HD is a congenital gastrointestinal disorder that requires surgical resection for correction. Some patients experience long-term bowel dysfunction requiring hospital care. Historically, patients had multiple staged operations, whereas more recently, single-stage laparoscopic resection and pull-through operations are more common. Assessment of possible changes over time in HD-associated complications requiring hospitalization and length of hospital stay has not been quantified epidemiologically. The Kids' Inpatient Database was queried for all discharges with an International Classification of Disease, Ninth Revision, Clinical Modification code for HD in the years 1997, 2000, 2003, and 2006. The HD cohorts from these 4 time points were compared, specifically analyzing differences in demographic data, associated diagnoses, in-hospital mortality and length of stay, procedures performed during hospitalization, and frequency of hospitalizations for HD-associated complications. Results reported included estimated frequencies and means with 95% confidence intervals. The estimated numbers of HD discharges, associated demographic data, and numbers of pull-through procedures have remained stable over the decade. The mean age (years) at the time of pull-through has decreased from 1.45 to 1.16 to 1.18 to 0.97 (P = 0.01). The mean length of stay (days) for these procedures has increased from 8.40 to 8.46 to 9.25 to 10.55 (P = 0.002). The estimated numbers of hospital admissions for HD-related constipation increased in recent years from 395 to 340 to 536 to 566 (P = 0.001). The estimated numbers of admissions for enterocolitis suggest an increasing trend from 466 to 402 to 584 to 556 (P = 0.11). HD pull-through procedures are being performed at younger ages over time, and post-pull-through lengths of stay have increased. Admissions for some HD-related complications have increased over the decade. Prospective cohort studies are needed to determine whether causal relationships exist among these trends.

Probiotic prophylaxis after pullthrough for Hirschsprung disease to reduce incidence of enterocolitis: A prospective, randomized, double-blind, placebo-controlled, multicenter trial

ObjectiveHirschsprung-associated enterocolitis (HAEC) is one of the most troublesome problems encountered after a pullthrough. We hypothesized that prophylactic administration of probiotics after a pullthrough procedure would decrease the incidence of HAEC. Study DesignA prospective, double-blind, placebo-controlled, randomized trial was conducted at 2 children's hospitals. Infants undergoing pullthrough were randomized to probiotic or placebo for a period of 3months post-pullthrough. Primary outcome was incidence of post-operative HAEC. Other outcomes included severity of HAEC by clinical grade, number of HAEC episodes and extent of aganglionosis. Pearson Chi Square analysis, as well as logistic regression, was used for statistical analysis. ResultsSixty-two patients were recruited (Sites: A=40; B=22). One was lost to follow up and one immediate post-op death was not included in final analysis. Probiotics were administered to 32 patients. Distribution of placebo/probiotics was equal between sites (P=0.858). Mean age at pullthrough was 6.5±8.1(±SD) months. The incidence of HAEC was 28.3%. The incidence of HAEC was not statistically different between probiotic and placebo study groups. ConclusionsIncidence of HAEC was not reduced with prophylactic probiotics. Future studies are needed to better determine the etiology and possible ways of preventing this complex condition.

Primary Swenson′s pull-through for adult Hirschsprung′s disease: A report of two cases from Southwest Nigeria

Hirschsprung's disease is commonly diagnosed (up to 90% in developed countries) in the neonatal period and infancy although late presentation into adulthood still occurs albeit uncommon. Adult Hirschsprung's disease (AHD) is defined as the presentation with features of Hirschsprung's disease to the hospital for the 1 st time or being diagnosed for the 1 st time after the age of 10 years. The standard treatment for AHD is staged: An initial colostomy, a pull-through procedure and a closure of the colostomy. The colostomy is performed to decompress the bowel of the huge fecal load, to allow the proximal megacolon to shrink and thus reduce the coloanal disproportion that will result during anastomosis and also to allow the nutritional rehabilitation of the patient resulting from the chronic constipation. Recent reports suggest that primary pull-through is possible in adults either as an open procedure or laparoscopically assisted. This is a report two cases of AHD managed in our center and who had primary Swenson's pull-through without a stoma. The need for patient selection is emphasized for a good outcome.

Literature Review of the Outcome after One-Stage Transanal Endorectal Pull-Through Procedure for Hirschsprung’s Disease in Children

Background: Transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung’s disease (HD) is frequently used worldwide. In order to give the families realistic expectations and to plan the medical care for the years after TERPT, the long term outcome is of great importance. Aim: To collect information on the long term outcome reported after one stage TERPT procedure for HD in children 0 - 15 years. Method: A literature review on the outcome of planned TERPT from 2005 through 2012 was carried out. Information was collected on the number of daily stools a few months postoperatively, incontinence and constipation and the measures taken to deal with these. Results: The reports are few and prospective studies were missing. The results show an initial high frequency of daily stools, 12% had later abnormal stool patterns, 21% had fecal incontinence and 10% had problems with constipation. Conclusion: In order to compare the long term outcome, it would be desirable to have uniform regular reports on the daily frequency of passed stools, incontinence and constipation during the first years after TERPT. Such knowledge would be of importance for the information given to the guardians of children with HD preoperatively to TERPT and in the planning of the future care. The findings can, furthermore, provide a benchmark for the outcome from a single centre.

Soave transanal one-stage endorectal pull-through in the treatment of Hirschsprung′s disease of the child above two-year-old: A report of 20 cases

The definitive treatment of Hirschsprung's disease is the removal of the aganglionic bowel by a pull-through surgery. In most cases, this surgery is performed in infancy or in the neonatal period as presentation in older children and adulthood is uncommon. It is a retrospective study of 20 patients above two-year-old who underwent a transanal Soave one-stage endorectal pull-through procedure for Hirschsprung's disease between January 2002 and December 2010. Twenty patients were recruited in this study. Fourteen were males and six were females. Patient ages ranged from 2 to 14 years (median age: five years and three months). All patients presented with persistent constipation and abdominal distension. Two of them had an intestinal obstruction that required colostomy. Ten patients (50%) had a recto-sigmoid Hirschsprung's disease. All patients were operated on using a Soave one-stage endorectal pull-through procedure. The laparoscopy was necessary during the pull-through in three cases. The average duration of the intervention was 240 minutes. That represents almost the double of the duration of the same procedure in newborns and infants in our department (130 minutes). Early postoperative complications included one case of anastomosis leakage and one case of intussusception. Late postoperative complications were perineum irritation in five cases (25%), anal stenosis in four cases (20%) and enterocolitis in one case (5%). None of our patients developed fecal incontinence. Soiling was reported in four cases (20%). There was no death. Soave transanal one-stage endorectal pull-through is safely feasible in children of more than two years of age. Laparoscopy may be necessary whenever there are difficulties in the pull-through.

Subtotal colectomy with a single-incision laparoscopic surgery technique in children with long-segment Hirschsprung disease and allied disorders

Over the last 15years, the laparoscopic-assisted endorectal pull-through procedure has become the standard treatment for Hirschsprung disease in many centers around the world. Recently, single-incision laparoscopic techniques have drawn more attention. We describe a single-incision laparoscopic surgery (SILS) subtotal colectomy to treat long-segment Hirschsprung disease (LSHD) and Hirschsprung disease allied disorder (HAD) in children. A total of 22 patients who underwent SILS subtotal colectomy, including three patients with a failed first surgery, were included in this retrospective study. For SILS, a 1-cm skin incision was first made below the umbilical margin and a 5-mm trocar was placed into the abdomen after incising the peritoneum. Two 5-mm trocars were then placed on both sides of the umbilicus. Subsequently, based upon preoperative examination and biopsy results, we performed subtotal colectomy. The affected colon was mobilized successively beyond the peritoneum using high-frequency cutting and sealing devices, followed by a pull-through procedure and colon-anal anastomosis. The average operative time was 206.39min. No case needed conversion from SILS to either conventional laparoscopy or open surgery. Of the 22 patients, 15 were diagnosed as LSHD, while 6 cases were diagnosed with intestinal neuronal dysplasia and one was diagnosed with hypoganglionosis. There were no intra-operative complications. One child had incision dehiscence on postoperative day three. During the follow-up over 12months, all patients were noted to have excellent cosmetic outcomes, and enterocolitis was observed in four children. Subtotal colectomy with the SILS technique can be safely performed in LSHD or HAD patients in the pediatric population without major complications.

Sacral nerve stimulation for faecal incontinence secondary to congenital imperforate anus

After treatment in infancy, patients with imperforate anus can develop bowel dysfunction in adult life. Low anorectal malformations are often associated with congenital deformity of the sacrum and coccyx (sacrococcygeal agenesis). Sacral nerve stimulation (SNS) is an effective treatment for incontinent patients with an accompanying acquired sphincter defect. We report two patients treated by SNS for faecal incontinence secondary to congenital imperforate anus. Two adults presented with faecal incontinence. Both had a congenital imperforate anus. The first, a female 36 years old, suffered from lifelong incontinence. She had daily passive soiling, a sensation of incomplete emptying and urgency (less than 5min). The second, a male 35 years old, had had incontinence since adulthood. He had urgency (2min) and daily soiling. Both had undergone coloanal pull-through procedures in infancy. Anal manometry showed a low resting pressure in both patients (30 and 33cm H2O) and a reduced peak squeeze pressure increment (15 and 40cm H2O). Endoanal ultrasonography demonstrated a complete ring of muscle around the neo-anus in both patients. The patients underwent peripheral nerve evaluation (PNE). Both had partial sacral agenesis, which made this technically difficult. After 3weeks of PNE, the female patient showed no improvement, but the male patient had no further episodes of incontinence and urgency improved from 2 to 15min. Accordingly, he underwent implantation of a permanent pulse generator. SNS may be effective for some incontinent patients with imperforate anus even in the presence of partial sacral agenesis. The clinician should be aware of any associated sacral agenesis and the technical difficulty this may cause. Alternative methods of neuromodulation, such as pudendal nerve stimulation, could be considered in such cases.

Late diagnosis of Hirschsprung disease—patient characteristics and results

PurposeThe aim of the present study was to describe the characteristics and the postoperative results of children diagnosed as having Hirschsprung disease (HD) after the age of 3 years. MethodsAll patients with HD diagnosed after the age of 3 years in our hospital from 1998 to 2011 were included. Patient characteristics and postoperative results were prospectively registered. ResultsEleven children were included. Age at diagnosis was 3.0 to 9.6 years. Ten patients had rectosigmoid disease, whereas 1 had total colonic aganglionosis. Three children were given a diverting ileostomy before the pull-through procedure, and all 3 had ileostomy-related complications. Early postoperative complications were seen in 5 children, of whom 2 had anastomotic leakage. At final follow-up, with a median of 3 years postoperatively, 7 had normal bowel function, 1 had frequent loose stools, and 3 were soiling. ConclusionsEarly postoperative complications, especially anastomotic leakage, occurred frequently in children with late-diagnosed HD. Therefore, a diverting stoma should be considered in these patients. The long-term functional results were comparable with those seen in children operated on as neonates.

The Hirschsprungs patient who is soiling after what was considered a “successful” pull-through

After surgery for Hirschsprungs disease, most children thrive, have few if any episodes of abdominal distention and enterocolitis, and are fecally continent. However, there exists a small group of patients who do not do well. Either they suffer from persistent distension and enterocolitis or they experience soiling after their pull-through procedure. These patients can be systematically evaluated and successfully treated with a combination of bowel management, dietary changes, and laxatives, and, in certain circumstances, a reoperation.

Reoperative surgery for Hirschsprung disease

Despite most children undergoing a successful pull through for Hirschsprung disease, a small portion of children are left with persistent stooling issues. Most of these stooling issues can be addressed by nonoperative approaches. However, in a small group of remaining children, a reoperation may be necessary. Most children who may need a redo pull-through procedure may have a persistent area of aganglionosis, unremitting enterocolitis, or a torsion or stricture of the pull-through segment. Each of these influences the approach the surgeon must take to correct the presenting problem. The chapter details the diagnostic approach as well as the operative techniques, which best deal with each of these complications.

Primary laparoscopic pull-through procedure for Hirschprung disease at a low resource setting: Experience and early results

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.

Management of Recurrent Post Redo Pull-Through Stricture - What is the Option?

Redo pull-through procedures are not without their complications and have results that are satisfactory only in 70% to 80% of cases. Persistent obstructive symptoms due to anastomotic stricture are a common indication for redo operation in 30% to 50% of cases. Management of stricture following a redo operation can still be a complicated issue with limited available options. One such case is presented and management discussed.

10 Years of Experience with Laparoscopic-Assisted Endorectal Soave Pull-Through Procedure for Hirschsprung's Disease in China

To report early and late results of laparoscopic-assisted endorectal Soave pull-through (LAEPT) with a short and V-shaped partial resection muscular cuff for Hirschsprung's disease (HD) over a 10-year period. The clinical courses of 218 patients who underwent modified LAEPT for HD were reviewed. LAEPT was described by the Georgeson technique. The main modifications included less dissection of the bottom of the pelvis, rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff, and a V-shaped partial resection in the posterior wall of the muscular cuff. From September 1999 to July 2009, 218 patients were operated on by the same surgeon. Ages ranged from 15 days to 12 years old. The aganglionic segment was located in the rectum or sigmoid colon in 176 patients, in the left colon in 38 children, and in the transverse colon in 4 patients. The median operating time was 176 minutes. Conversion to open surgery was required in 2 patients. Bleeding of the left iliac vein occurred in 1 patient, and a 180° twist of the neorectum occurred in 2 cases. Median first bowel movement time was 23 hours, and median postoperative hospital stay was 10.4 days. Median daily defecation frequency was 4.6 within 2 weeks and 2.3 at 3 months postoperatively. The immediate postoperative complications included intestine herniation from the trocar site in 2 patients, perianal excoriation in 32 patients, and anastomotic leakage in 3 patients. Follow-up ranging from 6 to 120 months was obtained for 182 patients. Late postoperative complications included postoperative adhesive bowel obstruction (1.1%), enterocolitis (7.7%), anasomostic stenosis (2.2%), constipation (1.6%), and soiling (3.6%). Eighty-seven percent had excellent and good bowel function. LAEPT with a short muscular cuff with a V-shaped partial resection in the posterior wall is a safe and effective procedure for HD.

Transanal endorectal pull-through for Hirschsprung’s disease using long cuff dissection and short V-shaped partially resected cuff anastomosis: early and late outcomes

Transanal endorectal pull-through was described by De la Torre-Mondragon's technique. In the original transanal pull-through procedure, a long rectal muscular cuff was dissected and left for anocolic anastomosis, which would sometimes lead to postoperative obstructive symptoms and enterocolitis. While a modified short mucosal dissection may increase the risk of injury to pelvic and perirectal nerves and other structures when dissected on the outside of the rectum deep in the pelvis. We report early and late results of the modified transanal procedure for Hirschsprung's disease (HD) over 8 years. The clinical course of all children with aganglionic rectum or sigmoid colon receiving the modified transanal pull-through between May 2003 and April 2011 were reviewed. The main modifications were rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff and a V-shaped partial resection in the posterior wall of the muscular cuff. Children with preliminary stoma or total colonic disease were excluded. Short- and long-term follow-up was obtained in 137 patients with HD operated upon by the same surgeon. The aganglionic segment was located in the rectum or sigmoid colon in all patients. The mean age at surgery was 165 ± 74 days. The mean operating time was 108 ± 38 min. Mean intra-operative blood loss was estimated to be 15 ± 10 ml. No patient required a blood transfusion. Mean postoperative hospital stay was 7 ± 2 days. Early postoperative complications included perianal excoriation in 38 patients (27.7 %), enterocolitis in two patients (1.4 %), and anastomotic leak in two patients (1.4 %). Late postoperative complications included perianal excoriation in 16 patients (11.7 %), anal stricture in two patients (1.4 %), constipation in four patients (2.8 %), enterocolitis in 10 patients (7.3 %), and soiling problems in six patients (4.4 %). Mean follow-up was 56 months (6 months-9 years). In patients older than 4 years, 85.4 % of them had excellent/good bowel function, 9.4 % had fair bowel function, and 5.2 % of patients had bad bowel function. Transanal endorectal pull-through with a long cuff dissection and a short V-shaped resected cuff anastomosis is a safe and effective procedure for HD. It reduced incidence of anastomotic stricture and constipation without an increased soiling incidence.

Is there a link between the calcium sensing receptor and Hirschsprung's disease? A mutational analysis

IntroductionHirschsprung's disease (HD) is a congenital malformation of the hindgut characterized by the absence of enteric ganglion cells in the submucosal and myenteric plexuses. Hirschsprung's disease is routinely treated by resection of the aganglionic bowel and pull-through procedure of the proximal ganglionated bowel to the anorectal region. Occasionally, after resection of the aganglionic bowel, HD patients still experience persistent disturbances in gut motility. The etiology of HD as well as the underlying pathomechanism for postoperative disturbances in gut motility is unclear. Molecular analysis of putative candidate genes may help to clarify the pathophysiology of these conditions. In the present study, we investigated the correlation between mutations and polymorphisms in the calcium sensing receptor (CaSR) and HD patients. MethodsMutational screening of the CaSR coding sequence was performed via polymerase chain reaction and direct sequencing of the amplified samples from 63 HD patients and 100 controls. ResultsWe identified 3 common polymorphisms (p.A986S, p.G990R, and p.Q1011E) residing in exon 7 and 1 polymorphism in intron 5 (IVS 5-88 T>C) of the CaSR gene. Overall, 16 patients (25%) and 25 controls (25%) were carriers of the p.A986S polymorphism (P = 1). The incidence of p.R990G polymorphism of patients was twice as high as in the control group (P = .17). Furthermore, we verified a 4 times higher incidence of p.Q1011E polymorphism carriers in patients compared to the control group (P = .1). ConclusionWe found a higher incidence of R990G and Q1011E polymorphisms in HD patients compared to controls. However, there was no statistically significant association between HD and the 3 polymorphic variants in the intracellular signaling region of CaSR. It will be important to further investigate genetic variations in CaSR in more patient cohorts to better characterize the function of this gene and to establish a correlation between CaSR variants and HD.