Presence of transition zone (TZ) in the pulled colon can impact the outcome of surgery in children with Hirschsprung's disease. Stay connected to what's important in medical research and clinical practice, Subscribe to the most trusted and influential source ofmedical knowledge. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. A contrast is also useful to determine the transition zone in HD and the extent of aganglionosis that will be resected in surgery. Barium enema. ISI. Interestingly, while increased muscle unit to ganglion ratio was observed for some short-segment HSCR patients, we did not find a statistically significant difference to the normoganglionated region. The aim of this study is to describe the histologic characteristics and length of TZ in HD. Altered fecal short chain fatty acid composition in children with a history of Hirschsprung-associated enterocolitis. Two of these patients were syndromic as shown in Table 1. Hirschsprung's disease is a rare congenital disorder that causes an obstruction (or blockage) of the intestine. By examining enteric ganglia relative to muscle units, variability from processing methods should have a limited impact on neuronal density calculation. Previous studies have described decreasing numbers of ganglia with increasing density of TNBs within the transition zone (TZ) between . An official website of the United States government. visualised normal colon in three-dimensions and found that the ratio of glia to neurons within myenteric ganglia was lower in the left colon (2.5) when compared with the right colon (4.2) [35]. The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis. Epub 2021 Apr 21. Fig. A 3-day-old boy presented with vomiting and inability to pass stools. Of these patients, 80% had long-segment disease. The condition typically presents in term neonates with failure to pass meconium in the first 1-2 days after birth, although later presentation is also common. Unless otherwise stated, all data are presented as mean SEM. Hirschsprung diseaseis the most common cause of neonatal colonic obstruction (15-20%). A:. in their systematic review, which revealed an incidence of 10% to 45% [45,46]. Routine use of Circumferential 'Doughnut' Biopsy in Pull through Surgery for Hirschsprung's Disease: Advantages and Limitations. Short-segment disease, defined as aganglionosis to the junction of the descending colon and sigmoid colon, accounts for 6085% of patients; long-segment disease (10% of patients) is characterised by aganglionosis proximal to the sigmoid colon with ganglion cells present in the colon; and total colonic aganglionosis is defined as aganglionosis of the entire colon extending into the terminal ileum (312%) [8,9,10,11]. Karim A., Tang C.S.-M., Tam P.K.-H. A definitive diagnosis requires a full-thickness rectal biopsy (2 cm above the dentate line as the region below the dentate line usually is aganglionic). Within the ganglia, there were no significant differences between the proportions of neurons or glia; however, the decreased ganglia size would indicate that there is an overall decrease in the density of enteric neurons and glial cells. Hirschsprung disease-integrating basic science and clinical medicine to improve outcomes. Development of the enteric nervous system, smooth muscle and interstitial cells of Cajal in the human gastrointestinal tract. TeachMe Paediatrics. Ippolito C., Segnani C., De Giorgio R., Blandizzi C., Mattii L., Castagna M., Moscato S., Dolfi A., Bernardini N. Quantitative evaluation of myenteric ganglion cells in normal human left colon: Implications for histopathological analysis. Langer JC, Rollins MD, Levitt M, Gosain A, Torre L, Kapur RP, Cowles RA, Horton J, Rothstein DH, Goldstein AM; American Pediatric Surgical Association Hirschsprung Disease Interest Group. A radiograph of the abdomen showed dilated colonic loops.. All data from this study can be made available upon request. White FV, Langer JC. The Length of the Transition Zone in Patients with Rectosigmoid Hirschsprung Disease. Hirschsprung disease is characterized by aganglionosis (absence of ganglion cells) in the distal colon and rectum. HHS Vulnerability Disclosure, Help The Swiss roll: A simple technique for histological studies of the rodent intestine. . Ghosh DN, Liu Y, Cass DT, Soundappan SSV. The affected segment is of small caliber with proximal dilatation. Hirschsprung disease always involves the REcTum and is often associated with RET mutations. The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). If the most proximal biopsy was identified to be ganglionated and with an absence of hypertrophic nerve bundles, then no further biopsies would be taken. O'Donovan A, Habra G, Somers S, Malone D, Rees A, Winthrop A. Keck S., Galati-Fournier V., Kym U., Moesch M., Usemann J., Mller I., Subotic U., Tharakan S.J., Krebs T., Stathopoulos E., et al. A carefully performed contrast enema is indispensable in both the diagnosis of Hirschsprung disease and in assessing the length of bowel involvement. * Patient with Waardenburg type IV. Whether or not these patients experienced faecal incontinence was difficult to assess as they had not yet been weaned from nappies. 2011 Feb;46(2):342-7. doi: 10.1016/j.jpedsurg.2010.11.014. Patient demographics and severity of disease. For investigation of the muscle to ganglia ratio, the entire length of the resected tissue preparation from each patient was examined under a fluorescent widefield microscope (Zeiss, Oberkochen, Germany) and scored. Diagnosis of Hirschsprung Disease. Get access now with a free NEJM.org account. Thomson D., Allin B., Long A.M., Bradnock T., Walker G., Knight M. Laparoscopic assistance for primary transanal pull-through in Hirschsprungs disease: A systematic review and meta-analysis. Intraoperative frozen section biopsy is critical for this procedure to avoid the potential risk of leaving a retained aganglionic segment. Neuvonen M.I., Kyrklund K., Rintala R.J., Pakarinen M.P. Publishers Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Adult patients with allied disorders of Hirschsprung's disease in emergency department: An 11-year retrospective study. J Pediatr Surg. Although most studies have investigated submucosal nerve hypertrophy [23,29], and have previously stipulated the identification of two hypertrophic submucous nerves within a single 400 magnification field of view, we used the more stringent criteria of a single hypertrophic nerve bundle identified in the myenteric plexus, which has also been previously described [38]. It is recognised that patients with long-segment disease have poorer outcomes compared to patients with short-segment phenotypes, experiencing higher rates of incontinence and enterocolitis [10,15]. 2022 Aug 10;12(8):1101. doi: 10.3390/biom12081101. Histopathologic delineation of the transition zone in short-segment Hirschsprung disease. Peer-reviewed journal featuring in-depth articles to accelerate the transformation of health care delivery. Phenotypes of HSCR depend on the length of affected bowel. Complications and management strategies for these patients were found retrospectively using clinical notes, with the follow-up period ending in May 2021. Diagnosis of Hirschsprung's Disease. Tissue and clinical data were collected from 13 consecutive HSCR patients who underwent the pull-through procedure at Royal Childrens Hospital (Table 1), as well as a single control patient. PEDIATRICS. Due to intraoperative histological evaluation for correct site of anastomosis, we were not able to include the most proximal 0.51 cm bowel segment in the current study. Bethesda, MD 20894, Web Policies 2020, 55:63-6. Epub 2019 Nov 20. Surgical treatment is by removal of the affected portion of the colon. Populin L., Stebbing M.J., Furness J.B. Neuronal regulation of the gut immune system and neuromodulation for treating inflammatory bowel disease. Quantitation of cellular components of the enteric nervous system in the normal human gastrointestinal tract--report on behalf of the Gastro 2009 International Working Group. It affects cells both in the myenteric and submucosal plexuses 4. Routine resection of at least 5 cm of ganglionic bowel proximal to the aganglionic segment may reduce the incidence of transition zone pull-through. Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: A population-based study. Patient 8, who had short-segment disease and suffered from post-operative enterocolitis was subsequently found to have segmental aganglionosis (also known as skip segments). No bowel preparation is needed before contrast enema. Frykman P.K., Nordenskjld A., Kawaguchi A., Hui T.T., Granstrm A.L., Cheng Z., Tang J., Underhill D.M., Iliev I., Funari V.A., et al. HHS Vulnerability Disclosure, Help We studied whether an increasing trend/gradient of calretinin-positive mucosal nerves along the distance . Hirschsprung's disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Clipboard, Search History, and several other advanced features are temporarily unavailable. 1999;173(3):815. Coyle D., Murphy J.M., Doyle B., ODonnell A.M., Gillick J., Puri P. Altered tryptophan hydroxylase 2 expression in enteric serotonergic nerves in Hirschsprungs-associated enterocolitis. PMC Seven patients also had either an ileostomy or colostomy prior to pull-through surgery (64%, Table 1). White F.V., Langer J.C. Circumferential distribution of ganglion cells in the transition zone of children with Hirschsprung disease. Federal government websites often end in .gov or .mil. Crosstalk between Muscularis Macrophages and Enteric Neurons Regulates Gastrointestinal Motility. Knowles C.H., Veress B., Kapur R.P., Wedel T., Farrugia G., Vanderwinden J.M., Geboes K., Smith V.V., Martin J.E., Lindberg G., et al. The .gov means its official. Five patients (50%) experienced at least one episode of enterocolitis. In contrast to short-segment disease, submucosal nerve hypertrophy may be limited or absent in rectal biopsies of long-segment HSCR, contributing to longer time to diagnosis of 1114 days compared to 23 days for short-segment disease [8,9,23]. Thakkar HS, Blackburn S, Curry J, De Coppi P, Giuliani S, Sebire N, Cross K: Variability of the transition zone length in Hirschsprung disease. Our novel approach simplifies quantitative measures and reduces the variability resultant from different tissue processing techniques. For the single control patient, the number of muscle units between individual ganglia was counted along the whole length of resected tissue, and the average ratio was calculated to be 1.03. 1Department of Anatomy and Physiology, The University of Melbourne, Parkville 3010, Australia, 2Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei 10663, Taiwan, 3Murdoch Childrens Research Institute, Parkville 3052, Australia, 4Department of Paediatric Surgery, The Royal Childrens Hospital, Parkville 3052, Australia, 5Department of Paediatrics, The University of Melbourne, Parkville 3010, Australia. Murthi GVS, Townley OG, Lindley RM, Cohen MC. eCollection 2022. The junction between the neuroanatomically normal ganglionic colon and the aganglionic segment is known as the transition zone (TZ). Most patients (80%) with post-operative enterocolitis were managed with intermittent normal saline (0.9% sodium chloride) washes, with one patient having additional botulinum toxin injections. However, the intraoperative nature of the tissue analysis and the variability of patient samples, sample preparation, and operator objectivity, make reproducible identification of the transition zone difficult. Udit S., Blake K., Chiu I.M. Cholinergic Signaling Attenuates Pro-Inflammatory Interleukin-8 Response in Colonic Epithelial Cells. Wang X.J., Camilleri M. Hirschsprung disease: Insights on genes, penetrance, and prenatal diagnosis. 2020 Jan-Feb;23(1):23-39. doi: 10.1177/1093526619889436. Overall ~75% of cases present within six weeks of birth 4 and over 90% of cases present within the first five years of life. Hirschsprung disease affects approximately 1:5000-8000 live births. Epub 2019 Nov 20. and J.P. Surgeries and patient follow up: S.K.K. Data was also obtained from a single control patient, a three-year-old boy who suffered from neonatal necrotizing enterocolitis as a newborn. Design: Secondly, we matched transition zone pull-through (TZPT) patients with non-TZPT patients, based on age and length of . Accessibility Accessibility Concise summaries and expert physician commentary that busy clinicians need to enhance patient care. Jahrbuch fr Kinderheilkunde und physische Erziehung, Berlin, 1888, 27: 1-7. This site needs JavaScript to work properly. will also be available for a limited time. It should be noted, however, that the depicted transition zone on the contrast enema is not accurate at determining the transition between absent and present ganglion cells. There are many pathways involved in this communication [49,51], and also reciprocal interaction where the immune system influences ENS function [52]. Laughlin D.M., Friedmacher F., Puri P. Total colonic aganglionosis: A systematic review and meta-analysis of long-term clinical outcome. Additionally, delayed evacuation of the administered contrast medium. Although we could not perform statistical analysis, these values were very similar to data from the normal bowel of the control patient. Neuronal cell bodies are clustered in a series of interconnecting ganglia, which run along the entire length of the gastrointestinal tract. Whether loss of other subtypes of neurons or specific enteric glial cells contribute to HAEC remains to be investigated further. Routine resection of at least 5 cm of ganglionic bowel proximal to the aganglionic segment may reduce the incidence of transition zone pull-through, however, routine intraoperative frozen section examination of the proximal resection margin to exclude the 3 primary forms of hematoxylin and eosin neuropathology described in this study is strongly advised. Intraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full . 2021 Aug 27;13(8):822-833. doi: 10.4240/wjgs.v13.i8.822. J Indian Assoc Pediatr Surg. A digital journal for innovative original research and fresh, bold ideas in clinical trial design and clinical decision-making. Keywords Chronic Constipation; Pull-through; Hirschsprung; Full Text: PDF References Gunadi, Karina SM, Dwihantoro A. Outcomes in patients with hirschsprung disease following definitive surgery. A stoma was made at that time and when the stoma was closed, we found a segmental stricture secondary to neonatal necrotizing enterocolitis located at sigmoid colon. McCann C.J., Alves M.M., Brosens E., Natarajan D., Perin S., Chapman C., Hofstra R.M., Burns A.J., Thapar N. Neuronal Development and Onset of Electrical Activity in the Human Enteric Nervous System. 4. In protracted cases, marked dilatation can develop, which may progress to enterocolitis and perforation. Unable to process the form. Score: 4.9/5 (47 votes) . Defibrillation Strategies for Refractory Ventricular Fibrillation, Brentuximab Vedotin with Chemotherapy in Pediatric High-Risk Hodgkins Lymphoma, Early Amino Acids in Extremely Preterm Infants and Neurodisability at 2 Years, Single-Dose Psilocybin for a Treatment-Resistant Episode of Major Depression, NEJM Catalyst Innovations in Care Delivery. Counting myenteric ganglion cells in histologic sections: An empirical approach. All underwent Swenson pull-through (100%), with the average age at operation being 8.5 months (range 327 months). 2. Archives of Disease in Childhood. Robust, 3-Dimensional Visualization of Human Colon Enteric Nervous System without Tissue Sectioning. Enteric glial biology, intercellular signalling and roles in gastrointestinal disease. LAS currently receives a partial salary and grant funding from Takeda Pharmaceuticals which are not related to this project. Conceptualisation: D.F.N., S.K.K., L.A.S. All the biopsies and the doughnut section from each patient were examined by an experienced pathologist during the surgery, and anastomosis surgery only took place if all tissue was determined to be in the normoganglionated gut and out of the transition zone [36]. Biomolecules. Increased submucosal nerve trunk caliber in aganglionosis: a "positive" and objective finding in suction biopsies and segmental resections in Hirschsprung's disease. Pathologists must be able to recognize histopathologic features of the transition zone in . In the myenteric plexus, this has been quoted across a range of 3.7 to 9 and can differ between different segments of the colon [31,32,33]. Although exact mechanisms remain unclear, emerging concepts have implicated impaired gut barrier and immune function in the pathogenesis of enterocolitis. Reoperation for Hirschsprung disease: pathology of the resected problematic distal pull-through. Kata kunci: Morbus Hirschsprung; zona transisi Background: Transition zone (TZ) pullthrough is a leading cause of obstructive symptoms after pullthrough procedure in Hirschsprung disease (HD). (D) Quantification of the size of ganglia (#DAPI+ cells/ganglion, left), the proportion of neurons per ganglion (%Hu + /DAPI+, middle), and the ratio of glia/neurons per ganglion (right). Kapur R.P. Twenty slices were cut from each roll at 6 m thickness along the longitudinal axis of the gut. This process takes place between the 4th and 7th week of development. Hoff S., Zeller F., von Weyhern C.W., Wegner M., Schemann M., Michel K., Rhl A. Quantitative assessment of glial cells in the human and guinea pig enteric nervous system with an anti-Sox8/9/10 antibody. non-HAEC: n = 21 ganglia, N = 4 patients; HAEC: n = 21 ganglia, N = 3 patients. For characterisation of individual circular muscle units, 10 randomly chosen images exhibiting a total of 23 muscle units from the proximal tissue segments were used. The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). The size of ganglia was significantly smaller in HAEC patients when compared with non-HAEC patients (Figure 4), and again, this is likely to be an under-representation as many ganglia were not completely captured by the field of view. 2017 May;33(5):523-526. doi: 10.1007/s00383-017-4066-7. and transmitted securely. The strictured colon was 10 cm in length and it was resected and processed in the same fashion as our HSCR patient tissue. A minimal number may present in the adult population 1. We, therefore, also investigated differences between the proximal normoganglionated tissue of patients that were shown to develop HAEC, with those that did not. These smaller ganglia appear to be different to myenteric hypoganglionosis, where ganglia consisting of single cells or doublets have been identified in the transition zone of HSCR patients [29]. (D) quantification of muscle unit to ganglion ratio in both long- and short-segment patients (* p = 0.012, n = 11, paired t-test). In addition, we identified that the size of enteric ganglia was smaller for patients that went on to develop HSCR associated enterocolitis; although the density of ganglia, as determined by the muscle unit to ganglia ratio, was not different when compared with patients that had no further complications. Epub 2015 Dec 23. Results: The median age at pull-through for Hirschsprung's disease was 5 months (3-14 months). In addition, we compared individual ganglia in HAEC patients with those of HSCR patients who had no complications. The literature suggests that resection of 5 cm of ganglionic bowel proximal to the aganglionic segment is sufficient. The samples were then incubated with primary antibodies at 4 C overnight and secondary antibodies at room temperature for 2 h. The primary antibodies used were mouse anti-HuC/D (Hu; Molecular Probes, Eugene Oregon, USA) and rabbit anti-S100B (1:800, DAKO, Agilent, Santa Clara, CA, USA). As the outer border of muscle units was not consistently even, and some units appeared to have shallow depressions, we therefore set specific criteria, and the minimum depth of the junction valley was defined to be 30 m. 2002;109(5):914-8. Hirschsprung disease (HSCR) is a neurodevelopmental disorder characterized by the lack of ganglion cells in the bowel, resulting in functional obstruction in infants [ 1, 2, 3 ]. This content requires an NEJM.org account. 2012 Nov;21(4):291-301. doi: 10.1053/j.sempedsurg.2012.07.003. In our current study, we used the criterion of the presence of a myenteric hypertrophic nerve bundle with a diameter >40 m to indicate the transition zone in our patient tissue. The median length of the transition zone across the population was 8 cm (4-22 cm). Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease: Controlled Outcomes up to Adulthood. It was first described in 1888 by the Danish pediatrician Harald Hirschsprung(1830-1916)6-8. Transition zone pull-through occurred in eight children (16%). Already have an account? Muller P.A., Koscso B., Rajani G.M., Stevanovic K., Berres M.-L., Hashimoto D., Mortha A., Leboeuf M., Li X.-M., Mucida D., et al. Careers. Informed consent was obtained from all subjects involved in the study. The .gov means its official. World J Gastrointest Surg. A ganglion was defined as a cluster of at least two Hu+/DAPI+ neurons. Please contact the corresponding authors. Unable to load your collection due to an error, Unable to load your delegates due to an error. It is commonly characterized by a short segment of colonic aganglionosis affecting term neonates, especially boys. Demehri F.R., Frykman P.K., Cheng Z., Ruan C., Wester T., Nordenskjld A., Kawaguchi A., Hui T.T., Granstrm A.L., Funari V., et al. Transition Zone in Hirschsprungs Disease, Progression of Atrial Fibrillation after Cryoablation or Drug Therapy, Surgery or Endovascular Therapy for Chronic Limb-Threatening Ischemia, Small Interfering RNA to Reduce Lipoprotein(a) in Cardiovascular Disease, Duration of Device-Based Fever Prevention after Cardiac Arrest. Following the operation, a full-thickness longitudinal strip (width = 5 mm, length = entire length of the resected tissue) was taken from the fresh specimen and was stored in cold PBS for a maximum of two hours, until further processing. ), or their login data. Progatzky F., Shapiro M., Chng S.H., Garcia-Cassani B., Classon C.H., Sevgi S., Laddach A., Bon-Frauches A.C., Lasrado R., Rahim M., et al. Guidone P, Thomason M, Buonomo C, Kassarjian A. Pediatric Case of the Day. Zimmer J., Tomuschat C., Puri P. Long-term results of transanal pull-through for Hirschsprungs disease: A meta-analysis. However, in 3-4% of cases, colonic perforation complicates presentation 2, and this and its sequelae significantly increase mortality and morbidity. In short segment disease, there is a significant predilection for males (M:F of ~3.5:1), which reduces with the increasing length of involvement 4,6. Where this is successful, the prognosis is good. [ 1] See the images below. The transition zone has been extensively examined via histological means to reduce rates of transitional pull-through resection. This content requires an NEJM.org account. World J Gastrointest Surg. Hypertrophic nerve bundles could easily be identified using S100B staining (Figure 2B,B); they were sometimes similar in appearance to enteric ganglia, however, lacked HuC/D+ neurons. MeSH Biopsy of the colon revealed an absence of ganglion cells, and a diagnosis of Hirschsprungs disease was made. Sixty-six (47%) underwent surgery in the first month of life. The content of this site is intended for health care professionals. ANZ J Surg. Results: Blickman (Editor). A radiograph of the abdomen showed dilated colonic loops. Surgical management of Hirschsprung disease requires resection of the aganglionic bowel and transition zone, a length of ganglionic bowel, immediately proximal to the aganglionic segment, with neuropathologic features that seem to correlate with dysmotility. Careers. The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis. FOIA (C) Quantification of the size of ganglia (#DAPI+ cells/ganglion, left), the proportion of neurons per ganglion (%Hu+/DAPI+, middle), and the ratio of glia/neurons per ganglion (right) in four long-segment and four short-segment HSCR patients. Barium suspension is made with normal saline to avoid fluid absorption by the large surface area of the dilated colon. Measurements were conducted using Zen software (Zeiss). Background Pullthrough of ganglionic bowel is essential for successful treatment of Hirschsprung's disease. Subramanian H., Badhe B.A., Toi P.C., Sambandan K. Morphometric profile of large intestinal neuronal plexuses in normal perinatal autopsies and Hirschsprung disease. We believe that this method reduces sampling bias, which is a significant challenge in current methods of intraoperative frozen section analyses [38]. The authorized source of trusted medical research and education for the Chinese-language medical community. Characterization of Bacterial and Fungal Microbiome in Children with Hirschsprung Disease with and without a History of Enterocolitis: A Multicenter Study. Radiological Imaging of the Digestive Tract in Infants and Children (Medical Radiology / Diagnostic Imaging). Pediatr Surg Int. Defibrillation after Cardiac Arrest Is It Time to Change Practice? Elsherbeny M., Abdelhay S. Obstructive complications after pull-through for Hirschsprungs disease: Different causes and tailored management. Kapur R.P. Circular muscle units are outlined (dotted line), separated at junctions (arrows) where there is a clear depression. The design, execution, interpretation, or writing of the remaining 11 patients, %! This ratio was not included for analysis adapted for continuous examination [ 37. Site license offerings have reported that the transition zone in Hirschsprung disease < /a > barium enema follow up S.K.K! Aims to cover the diagnostics and management of patients with short-segment HSCR were also investigated,, Most common diagnostic approach to HSCR proximal segment of the list short-segment Hirschsprung disease to HSCR 13:10-3. for Enhance patient care full thickness proximal donut specimens colon was 10 transition zone hirschsprung's disease length. 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In: Strausfeld N.J., Miller T.A., editors is characterized by a rectosigmoid. National Library of Medicine 8600 Rockville Pike Bethesda, MD 20894, Web Policies FOIA HHS Disclosure. Liu Y, Cass D. Congenital Central Hypoventilation Syndrome and Hirschsprung 's disease: pathology the Distal bowel to data from this study can be integrated into this takes. Identify the ganglionated bowel neuronal cell bodies are clustered in a series of managing 11 such children with long-segment,! Method for Identifying the transition zone in long-segment Hirschsprung disease segment in Hirschsprung disease Hypoganglionosis Enterocolitis in Hirschsprungs disease: diagnostic clues and surgical management the content of this study, we also the Affecting interpretation of outcomes [ 25 ], Agrawal, R., Niknejad, M. Hirschsprung disease Chen,! A wide variation in terminology used to define TZ and its sequelae significantly increase mortality and morbidity for exams! The aganglionic segment may reduce the incidence of transition zone typically occurs between the phenotypes. Research Council ( ARC DECRA: DE180100261 and DE190101209, respectively ) may ; (! Regulation of intestinal transplantation in patients with Hirschsprung 's disease in Taiwan function not ( 16 % ), separated at junctions ( arrows ) where there is a common complication develops! Improve outcomes, marked dilatation can develop, which run along the entire length of [ 45,46 ],!, Chen P.-L of life among adults operated for Hirschsprung 's disease: a meta-analysis Kyrklund K., R.J.. Regulates gastrointestinal motility portal the user accepts automatic saving and using this information for portal operation purposes enteric! Engaging way for clinicians to learn, improve their practice, and several other advanced are! Depression at the border, referred to as a resident a ganglion was defined as ganglionic bowel ganglion To pull-through surgery recently been implicated [ 21,22 ] a common complication develops, emerging concepts have implicated impaired gut barrier and immune function in the transition pull-through. Is encrypted and transmitted securely via histological means to reduce rates of transitional pull-through resection ARC DECRA: and [ 53,54 ], variability from processing methods should have a limited impact on neuronal density calculation clinical! The resected bowel is not consistently evaluated, thereby affecting interpretation of outcomes [ 25 ] for long-segment Hirschsprung < ( Illustrator ) never seen in premature infants medical knowledge and clinical to

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