Morphometric profile of large intestinal neuronal plexuses in normal perinatal autopsies and Hirschsprung disease

Subramanian, Hema; Badhe, Bhawana Ashok; Toi, Pampa Ch; Sambandan, K.

doi:10.1111/nmo.12939

Cited by 8 publications

(13 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A number of key criteria have previously been described in the transition zone of HSCR patients [ 29 ]. In our longitudinal colon sections, the presence of a myenteric hypertrophic nerve bundle (>40 µm) was used to identify the transition zone, as described by Subramanian et al [ 38 ]. This criterion was employed as the authors used similar patient samples as our current study (HSCR patient tissue resections from primary surgery sectioned along the longitudinal axis).…”

Section: Resultsmentioning

confidence: 99%

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

et al. 2022

View full text Add to dashboard Cite

Hirschsprung disease (HSCR) is characterised by the absence of enteric ganglia along variable lengths of the distal bowel. Current gold standard treatment involves the surgical resection of the defective, aganglionic bowel. 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, 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. Here, we have described a novel method for using muscle units as a distinctive landmark for quantifying the density of enteric ganglia in resection specimens from HSCR patients. We show that the muscle unit to ganglion ratio is greater in the transition zone when compared with the proximal, normoganglionated region for long-segment HSCR patients. Patients with short-segment HSCR were also investigated, however, the muscle unit to ganglion ratio was not significantly different in these patients. Immunohistochemical examination of individual ganglia showed that there were no differences in the proportions of either enteric neurons or glial cells through the different regions of the resected colon. 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. This suggests that subtle changes in the enteric nervous system, even in the “normoganglionated” colon, could be involved in changes in immune function and subsequent bacterial dysbiosis.

show abstract

Section: Resultsmentioning

confidence: 99%

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

et al. 2022

View full text Add to dashboard Cite

show abstract

“…2) [10,12]. The presence of ganglia in proximal biopsies, transition-zone hypoganglionosis, and absent ganglia distally are classical signs of HSCR [13][14][15][16]. The aganglionic segment was thus measured and optimum myotomy length was tailored accordingly.…”

Section: Methodsmentioning

confidence: 99%

“…[10,12] Presence of ganglia in proximal biopsies, transition zone hypoganglionosis and absent ganglia distally is classical of HSCR. [13][14][15][16] Aganglionic segment was thus measured and optimum myotomy length was tailored [9,10] High-definition gastroscope (GIF-HQ190, Olympus) with a distal transparent attachment (Olympus) was used. Submucosal (SM) elevation was achieved using normal…”

Section: Accepted Manuscriptmentioning

confidence: 99%

Initial experience with per-rectal endoscopic myotomy for Hirschsprung’s disease: medium and long term outcomes of the first case series of a novel third-space endoscopy procedure

et al. 2020

View full text Add to dashboard Cite

Introduction Hirschsprung’s disease (HSCR) is congenital aganglionosis affecting hindgut and presents with constipation. Surgical pull-through is current standard but has morbidity. Per-rectal endoscopic myotomy (PREM) is a novel third space endoscopy technique for treating SS-HSCR. Methods Retrospective series of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, anorectal manometry and treated by PREM. Aganglionic segment mapped pre-PREM. PREM performed using third space endoscopy principles. Objective – to compare stool frequency and unit laxative (UL) usage pre and post-PREM. Results N = 9; duration 4-years. Mean age – 7.5 (± 5.2) years; 7 males. Mean aganglionic segment length – 6.3 (± 4.4) cm. Mean procedure time – 96.1 (±37.9) minutes. Mean LOS – 2.5 (±0.7) days. Median follow-up –17 months (9 – 58). Stool frequency – pre – 1/4.4 (±1.5) vs. post – 1/1.2 (±0.4) days (p = 0.0004). Mean UL usage – pre – 5.4 (±4.9) vs. post – 0.4 (±0.7) units (p = 0.0002). No laxatives in 6 (66%). Single AE (anal stenosis) – dilatation. Conclusions PREM is a safe and effective minimally invasive procedure for SS-HSCR and provides long-term response.

show abstract

“…UHF probes deliver frequencies between 30 and 80 MHz, the highest frequency facilitating a resolution down to 30 μm, however at the cost of restricting imaging depth to only 5-10 mm [19] . In the bowel wall, nodules of ganglion cells measure about 30 μm and the organization of the microscopic tissue structure is reported to differ between ganglionic and aganglionic tissue [20] . Even though single cells may not be able to be distinguished, the tissue structure gives rise to different appearances of the image speckle pattern, suggesting that it would be possible to distinguish between aganglionic and ganglionic intestine using UHF ultrasound, especially since the tissue layers themselves are only 1-4 mm thick, making it difficult to employ ultrasound frequencies normally encountered in the clinic, but suitable for UHF ultrasonography.…”

Section: Theory/calculationmentioning

confidence: 99%

Ultra high frequency ultrasonography to distinguish ganglionic from aganglionic bowel wall in Hirschsprung disease: A first report

Granéli

Erlöv

Mitev

et al. 2021

Journal of Pediatric Surgery

View full text Add to dashboard Cite

Background/Purpose: In Hirschsprung disease (HD) surgery, confirming ganglionic bowel is essential. A faster diagnostic method than the current frozen biopsy is desirable. This study investigated whether aganglionic and ganglionic intestinal wall can be distinguished from each other by ultra high frequency ultrasound (UHF ultrasound). Methods: In an HD center during 2019, intestinal walls of recto-sigmoid specimens from HD patients were examined ex vivo with a 70 MHz UHF ultrasound transducer. Data from four sites were described. Histopathologic analysis was compared to the ultrasonography outcome at each site. Each patient's specimen served as its own control. Results: 11 resected recto-sigmoid specimens (median 20 cm long [range 6.5-33]) with transition zones of 5 cm (2-11 cm) were taken from children aged 22 days (13-48) weighing 3668 g (3500-5508); 44 key sites were analyzed. There was full concordance for 42/44 (95%) key sites and 10 of 11 (91%) specimens. The specimen with discordance of two key sites contained a segment of aganglionosis (3 cm) and a transition zone (1 cm): the site discordance was limited to the transition zone ends. Conclusions: This first report on UHF ultrasound in recto-sigmoid HD shows promising results in identifying aganglionosis, transition zones and ganglionic bowel. Further in vivo studies are required.

show abstract

Morphometric profile of large intestinal neuronal plexuses in normal perinatal autopsies and Hirschsprung disease

Abstract: The determined objective values, after testing on diagnostic rectal biopsies, may serve to formulate a diagnostic algorithm along with immunostaining for diagnosis of HSCR in colorectal specimens.

Cited by 8 publications

References 23 publications

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

A Novel Method for Identifying the Transition Zone in Long-Segment Hirschsprung Disease: Investigating the Muscle Unit to Ganglion Ratio

Initial experience with per-rectal endoscopic myotomy for Hirschsprung’s disease: medium and long term outcomes of the first case series of a novel third-space endoscopy procedure

Ultra high frequency ultrasonography to distinguish ganglionic from aganglionic bowel wall in Hirschsprung disease: A first report

Contact Info

Product

Resources

About