transition zone hirschsprung's disease

An official website of the United States government. * Patient with Waardenburg type IV. 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]. This process takes place between the 4th and 7th week of development. Semin Pediatr Surg. Bethesda, MD 20894, Web Policies 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. 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. 1999;173(3):815. Coyle D., ODonnell A.M., Tomuschat C., Gillick J., Puri P. The Extent of the Transition Zone in Hirschsprung Disease. Yang W., Chen S.-C., Lai J.-Y., Ming Y.-C., Chen J.-C., Chen P.-L. On average, tissue from each patient was divided into 3.8 rolls (range from 1 to 6 rolls, depending on the resected bowel length). Routine resection of at least 5 cm of ganglionic bowel proximal to the aganglionic segment may reduce the incidence of transition zone pull-through. 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. Part of the TeachMe Series. Our novel approach simplifies quantitative measures and reduces the variability resultant from different tissue processing techniques. Jarvi K., Laitakari E.M., Koivusalo A., Rintala R.J., Pakarinen M.P. All underwent Swenson pull-through (100%), with the average age at operation being 8.5 months (range 327 months). Nakamura H., Henderson D., Puri P. A meta-analysis of clinical outcome of intestinal transplantation in patients with total intestinal aganglionosis. Extended resection at a minimum 5 cm beyond the most distal ganglionic intra-operative biopsy and intra-operative histological examination of the proximal resection margin are required to minimize transition zone pull-through. Before Circumferential distribution of ganglion cells in the transition zone of children with Hirschsprung disease. It is possible that TZPT is responsible for some of these outcomes, however, the underlying causes of many of these complications remain unknown. Conclusion: The .gov means its official. BACKGROUND/PURPOSE It is generally accepted that if surgery for Hirschsprung's disease is to be successful . (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. Sixty-six (47%) underwent surgery in the first month of life. 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. MeSH Veras L.V., Arnold M., Avansino J.R., Bove K., Cowles R.A., Durham M.M., Goldstein A.M., Krishnan C., Langer J.C., Levitt M., et al. Pediatr Dev Pathol. Ganglia are more sparsely located in the transition zone, and no ganglia are visible in the aganglionic zone, although individual glial cells are present (some highlighted by arrowheads). However, our study is currently limited by sample size with only one control specimen, and further investigation is required before this can be integrated into intraoperative analysis for transition zone identification. Previous studies have described decreasing numbers of ganglia with increasing density of TNBs within the transition zone (TZ) between . Twelve patients underwent Swenson transanal pull-through procedure and one patient with total colonic aganglionosis had a Duhamel pull-through (Table 1). 8600 Rockville Pike Elsherbeny M., Abdelhay S. Obstructive complications after pull-through for Hirschsprungs disease: Different causes and tailored management. Transition zone pull-through occurred in eight children (16%). Keywords: Clipboard, Search History, and several other advanced features are temporarily unavailable. eCollection 2022. 2015 Nov-Dec;18(6):466-76. doi: 10.2350/15-07-1675-OA.1. Crossref. Learn more However, this facility is not available in most low-income countries. However, in addition, the distribution of ganglia in the transition zone is now understood to be uneven, resulting in a leading edge, which contributes to misidentification as the normoganglionated region and resulting TZPT [39]. Twenty slices were cut from each roll at 6 m thickness along the longitudinal axis of the gut. Complications and management strategies for these patients were found retrospectively using clinical notes, with the follow-up period ending in May 2021. Alnajar H., Murro D., Alsadi A., Jakate S. Spectrum of Clinicopathological Deviations in Long-Segment Hirschsprung Disease Compared with Short-Segment Hirschsprung Disease: A Single-Institution Study. This site needs JavaScript to work properly. Our data show that, although the ganglia are more sparsely located, the components of the ganglia are remarkably similar in the transition zone when compared with the normoganglionated region. Hypertrophic nerve bundles had a minimum width of 40 m. Hirschsprung Disease (HD) is caused by failure of neural crest cell migration, resulting in a distal aganglionic segment of the colon, preventing relaxation. Please enable it to take advantage of the complete set of features! Radiology. PMC legacy view Measurements were conducted using Zen software (Zeiss). The median length of the transition zone across the population was 8 cm (4-22 cm). In the current project, analysis of the muscle unit to ganglion ratio was performed following fixation and immunohistochemistry to provide further characterisation of the patient tissue. (B) shows a hypertrophic nerve bundle, which could be identified using S100B immunohistochemistry, but lacked neuronal cell bodies. Comparison of ganglia from the proximal normoganglionic tissue in non-HAEC and HAEC patients. The follow-up period of this study ended in May 2021, with a mean follow-up time of 10 months (range 217 months) from the date of their final surgery (stoma closure if an ostomy was performed). A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio. Background: The content of this site is intended for health care professionals. Representative H&E image of the tissue architecture in the transition zone of a long-segment HSCR patient. Zimmer J., Tomuschat C., Puri P. Long-term results of transanal pull-through for Hirschsprungs disease: A meta-analysis. Coupled with our institutes current protocols for identifying the transition zone, this suggests that TZPT may not be the cause of enterocolitis in our patients. Interestingly, further analysis of individual ganglia in our study revealed that while the density of ganglia in the transition zone was sparser, the composition of ganglia was remarkably similar to that of ganglia in the proximal, normal region. Shallow depressions (open arrows) were also present, but were <30 m in depth, and thus, were identified as being located within muscle units. A digital journal for innovative original research and fresh, bold ideas in clinical trial design and clinical decision-making. Swenson O. Hirschsprung's Disease: A Review. It was first described in 1888 by the Danish pediatrician Harald Hirschsprung(1830-1916)6-8. 2021 Mar-Apr;26(2):111-116. doi: 10.4103/jiaps.JIAPS_43_20. Neurostimulation-Guided Anal Intrasphincteric Botulinum Toxin Injection in Children with Hirschsprungs Disease Ambartsumyan L., Smith C., Kapur R.P. We present our series of managing 11 such children with considerations for conservative management. Images were taken with a 10 objective in order to capture the full radial thickness of the muscle (Figure 1). Seven patients also had either an ileostomy or colostomy prior to pull-through surgery (64%, Table 1 ). The newest Colorectal Quiz Episode # 34 "Newborn Perforation in ARM" is at the bottom of the list. A 3-day-old boy presented with vomiting and inability to pass stools. Chang X, Li S, Li K, Cao G, Zhang X, Li S, Yang D, Tang S. Pediatr Surg Int. 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. The proximal ganglionic portions of colonic resection specimens from 59 patients with distal aganglionosis were analyzed with closely spaced transverse sections to map the distribution of the 3 most commonly referenced . 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. Note: (C) Quantification of the muscle unit to ganglia ratio (ns: p > 0.05, Students t-test). {"url":"/signup-modal-props.json?lang=us\u0026email="}, Agrawal, R., Niknejad, M. Hirschsprung disease. Kawaguchi A.L., Guner Y.S., Smme S., Quesenberry A.C., Arthur L.G., Sola J.E., Downard C.D., Rentea R.M., Valusek P.A., Smith C.A., et al. McGraw-Hill Professional. Neuvonen M.I., Kyrklund K., Rintala R.J., Pakarinen M.P. Swaminathan M., Kapur R.P. 2011 Feb;46(2):342-7. doi: 10.1016/j.jpedsurg.2010.11.014. The strictured colon was 10 cm in length and it was resected and processed in the same fashion as our HSCR patient tissue. 2020, 55:63-6. Murthi GVS, Townley OG, Lindley RM, Cohen MC. Monforte-Muoz H, Gonzalez-Gomez I, Rowland JM, Landing BH. Between September 2019 and April 2021, 13 consecutive patients underwent the pull-through procedure for Hirschsprung disease at the Royal Childrens Hospital, Melbourne, Australia, and were included in this study. Due to the loss of structural integrity of tissue following processing, Patient 10 was not included for analysis. Keck S., Galati-Fournier V., Kym U., Moesch M., Usemann J., Mller I., Subotic U., Tharakan S.J., Krebs T., Stathopoulos E., et al. 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. Hirschsprung-associated enterocolitis is a common complication that develops in HSCR patients [17]. The affected segment is of small caliber with proximal dilatation. 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. The site is secure. Two of these patients were syndromic as shown in Table 1. [38]. Heuckeroth R.O. Surgical treatment is by removal of the affected portion of the colon. Somatosensory and autonomic neuronal regulation of the immune response. The median length of the transition zone across the population was 8 cm (4-22 cm). A number of key criteria have previously been described in the transition zone of HSCR patients [29]. Practical pathology and genetics of Hirschsprungs disease. Clear and reliable distinction of the normoganglionated bowel from the transition zone is key for successful resection of the entire defective bowel, and the avoidance of subsequent postoperative complications. However, there appears to be much variability in tissue handling and measurement of various parameters. Whether loss of other subtypes of neurons or specific enteric glial cells contribute to HAEC remains to be investigated further. 9. 2022 Aug 10;12(8):1101. doi: 10.3390/biom12081101. Although we could not perform statistical analysis, these values were very similar to data from the normal bowel of the control patient. Correctly and quickly identifying the transition zone during HSCR pull-through surgery is important for reducing post-operative complications. We studied whether an increasing trend/gradient of calretinin-positive mucosal nerves along the distance . ISI. Gunadi , Ryantono F., Sethi R., Marcellus , Kalim A.S., Imelda P., Melati D., Simanjaya S., Widitjiarso W., Pitaka R.T., et al. We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease. This ensured we did not underestimate the transition zone in our samples, however, comparisons of the equivalent regions to other studies could be more difficult. To prevent sampling error, all data were examined across three non-consecutive sections. 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. Neuroanatomical Techniques: Insect Nervous System. 2. 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. Abstract. The https:// ensures that you are connecting to the Federal government websites often end in .gov or .mil. It is now recognised that the ENS communicates with the gut immune system and has an important role in immune activation and suppression [48,49,50]. Of these patients, 80% had long-segment disease. 8. A transition point is seen at the junction between sigmoid and descending colon. As only four patients with short-segment disease were analysed in this study (due to exclusion of Patient 10), further investigation is warranted. Confocal imaging: W.Y., J.P. and H.M.Y. The .gov means its official. The Recent data shows that enteric glia also play important roles in this exchange [53,54]. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Friedmacher F., Puri P. Residual aganglionosis after pull-through operation for Hirschsprungs disease: A systematic review and meta-analysis. 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. The gut, its microbiome, and the brain: Connections and communications. Althoughmostpatientsmarkedlyimprovepostoperatively, some continue to experience obstruction and require reoperation. For patients with long-segment HSCR (n = 7), we identified a significant difference between the normoganglionated colon and transition zone; however, the two regions were not statistically different for short-segment patients (n = 4). A contrast is also useful to determine the transition zone in HD and the extent of aganglionosis that will be resected in surgery. Verkuijl SJ, Friedmacher F, Harter PN, Rolle U, Broens PM. The Length of the Transition Zone in Patients with Rectosigmoid Hirschsprung Disease. Enteric Glia Modulate Macrophage Phenotype and Visceral Sensitivity following Inflammation. TeachMe Paediatrics. Furness J.B. There is a wide variation in terminology used to define TZ and its management. FOIA Unable to load your collection due to an error, Unable to load your delegates due to an error. 2020 Jan-Feb;23(1):23-39. doi: 10.1177/1093526619889436. Copyright 2019 Elsevier Inc. All rights reserved. Ethics approval was obtained by The Royal Childrens Hospital Research Ethics Committee (HREC 38262). Barium. An official website of the United States government. All authors have read and agreed to the published version of the manuscript. Previous studies have shown that quantitative assessment, such as the measurement of submucosal nerve trunks or interganglion distance, has been proposed to prevent the morbidity associated with TZPT [38,40]. 1. Diagnosis of Hirschsprung's Disease. Three other neuroanatomic phenotypes (gangliosclerosis, ectopic myenteric ganglia, and eosinophilic ganglionitis) of uncertain clinical significance were distributed more irregularly and often over much longer distances. Hirschsprung disease is characterized by aganglionosis (absence of ganglion cells) in the distal colon and rectum. Score: 4.9/5 (47 votes) . A 3-day-old boy presented with vomiting and inability to pass stools. 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). Phenotypes of HSCR depend on the length of affected bowel. 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]. (A,B) Representative images of ganglia from the normal (A) and transition zone (B), with immunohistochemistry performed against DAPI, HuC/D (Hu) and S100B. To our knowledge, this study would be the first to investigate the changes in ganglion density in this manner. Regulation of intestinal immunity and tissue repair by enteric glia. Pathologists must be able to recognize histopathologic features of the transition zone in hematoxylin and eosin-stained sections in order to interpret intraoperative frozen sections and ensure adequate resection. Epub 2021 Mar 4. 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. The specimen was fixed in 10% formalin overnight and then embedded into paraffin. 3. 2022 Aug 10;12(8):1101. doi: 10.3390/biom12081101. Udit S., Blake K., Chiu I.M. Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease: Controlled Outcomes up to Adulthood. Reoperation for Hirschsprung disease: pathology of the resected problematic distal pull-through. 2022 Jul 27;14(7):656-669. doi: 10.4240/wjgs.v14.i7.656. The muscle unit to ganglia ratio was not significantly different between patients who experienced post-operative enterocolitis (1.31 0.1, n = 5) and patients who had no reported post-operative complications (1.29 0.1, n = 5; p = 0.90, Students t-test, Figure 4). an unprepared single-contrast barium enema may help establish the diagnosis by identifying a transition zone between a narrowed aganglionic segment and a dilated and normally innervated segment. Overview of the distribution of myenteric ganglia in relation to circular muscle units in the proximal normal zone (A, NZ), the transition zone (B, TZ), and the aganglionic zone (C) in longitudinal sections of HSCR patient tissue following immunohistochemistry against HuC/D (Hu), S100B and DAPI.

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