A Case of Colonic Hypoganglionosis Complicated with Colonic Ulcers

Cho, Kyuman; Lim, Sung-Uk; Park, Seon-Young; Lee, Kyung‐Hwa; Ju, Jae Kyun; Rew, Jong Sun

doi:10.15746/sms.15.009

Cited by 4 publications

(3 citation statements)

References 7 publications

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“…In both forms there will be a transition zone where the diseased segment merges with normal colon. Numerous prior reports of HG have also identified a decrease in the density of ICCs which mirrors that of the ganglion cells and can be identified immunohistochemically by decreased staining for c-kit [2,6,11]. ICCs are regarded as pacemakers of the gastrointestinal tract due to their ability to generate slow wave activity and act as intermediaries in neural control of the gut by co-ordinating inhibitory and excitatory signals[12].…”

Section: Discussionmentioning

confidence: 99%

“…ICCs are regarded as pacemakers of the gastrointestinal tract due to their ability to generate slow wave activity and act as intermediaries in neural control of the gut by co-ordinating inhibitory and excitatory signals[12]. Other typical histological changes of HG include the presence of immature ganglion cells, mature ganglion cells of reduced size and number, gliosis within Auerbach’s plexus and hypertrophy of nerve fibres, muscularis mucosae and the inner circular layer of muscularis propria[2,5,11,13]. Lymphocytic or eosinophilic ganglionitis may also be seen[11].…”

Section: Discussionmentioning

confidence: 99%

“…Other typical histological changes of HG include the presence of immature ganglion cells, mature ganglion cells of reduced size and number, gliosis within Auerbach’s plexus and hypertrophy of nerve fibres, muscularis mucosae and the inner circular layer of muscularis propria[2,5,11,13]. Lymphocytic or eosinophilic ganglionitis may also be seen[11]. Specific immuno-histochemical stains for S-100, succinic dehydrogenase, acetylcholinesterase, NADPH-diaphorase, neuronal enolase, neuronal PGP and anti-neuronal IgG have all been described as having diagnostic utility[8,14,15].…”

Section: Discussionmentioning

confidence: 99%

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Acquired segmental colonic hypoganglionosis in an adult Caucasian male: A case report

Kwok

Still

Hart

2019

WJGS

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BACKGROUND Hypoganglionosis is a rare condition that most often presents with abnormal gastrointestinal transit and usually arises in early childhood or adolescence. Two types have been described (Type I and Type II). The adult-onset form (acquired hypoganglionosis) is extremely uncommon and is thought to arise due to cellular remodelling as a result of chronic inflammation. It differs from Hirschprung’s disease in that there is a reduction in ganglion cells in the colonic neural plexuses as opposed to being completely absent. CASE SUMMARY A 31 year-old male presented to hospital with recurrent abdominal pain and vomiting over thirteen months. Abdominal computed tomography scans demonstrated thickening and stranding affecting the transverse, descending and sigmoid colon. Endoscopic appearances were non-specific but confirmed a mixed picture of mucosal inflammation and necrosis in various stages of healing. Numerous investigations were performed to elucidate an underlying aetiology but neither an infective nor ischaemic cause could be proven. Biopsy features were not typical of inflammatory bowel disease. Due to persistence of his symptoms and failure of medical management, a segmental colectomy was performed. Histological examination of the specimen revealed an unexpected finding of segmental hypoganglionosis. Complete surgical excision of the diseased segment of colon was curative and since his operation the patient has had no recurrence of symptoms requiring hospitalisation. CONCLUSION Our case serves to raise awareness of acquired hypoganglionosis as a rare condition that can result from chronic colitis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Acquired segmental colonic hypoganglionosis in an adult Caucasian male: A case report

Kwok

Still

Hart

2019

WJGS

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Immunostaining for Hu C/D and CD56 is useful for a definitive histopathological diagnosis of congenital and acquired isolated hypoganglionosis

et al. 2017

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Isolated hypoganglionosis (IHG) has been proposed as a distinct entity with two subtypes: congenital IHG (CIHG) and acquired IHG (AIHG). However, due to the rarity of the disease and the lack of defining histological criteria, the concept of IHG is not widely accepted. We studied paraffin-embedded intestinal specimens from 79 patients diagnosed with Hirschsprung's disease (HD) (n = 49), CIHG (n = 25), and AIHG (n = 5) collected between January 1996 and December 2015. Histopathological diagnosis of HD, CIHG, and AIHG was confirmed by hematoxylin and eosin staining and immunohistochemical staining using Hu C/D and CD56. We evaluated (immuno)histopathological findings, counted the number of ganglion cells, and measured the size of Auerbach's plexus. Hu C/D labeled neuronal cell bodies, whereas CD56 was detected in all neuronal components. In HD, all ganglion cells in Auerbach's plexus in the normoganglionic segment (NGS) were immunoreactive for Hu C/D, whereas in the aganglionic segment (AGS), these were replaced by CD56-positive extrinsic nerve fibers and bundles. The number of ganglion cells in AIHG and CIHG was significantly lower than in the NGS of HD (p < 0.05). Auerbach's plexus was significantly smaller in CIHG (p < 0.05) but in AIHG equivalent to the NGS in HD. In summary, immunostaining for Hu C/D and CD56 is useful for definitive histopathological diagnosis of IHG.

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