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Histological characteristics of eosinophilic myenteric ganglionitis: an under-recognised cause of chronic intestinal pseudo-obstruction

  • Yoichi Akazawa
  • Takuo Hayashi
  • Tsuyoshi Saito
  • Koichiro Niwa
  • Hirohiko Kamiyama
  • Noriko Sasahara
  • Kazuhiro Sakamoto
  • Akihito Nagahara
  • Takashi Yao
Brief Reports
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Abstract

Eosinophilic myenteric ganglionitis (EMG) is characterised by eosinophilic infiltration of the myenteric plexus. EMG has been rarely reported as a cause of chronic intestinal pseudo-obstruction (CIPO), and its histopathological features are not fully elucidated. We analysed seven patients with CIPO. Three of them were diagnosed with EMG and four patients were categorised as non-EMG. Clinicopathological features were similar in both groups. These features included subtle to mild lymphocytic infiltration at the myenteric ganglia/muscularis propria, loss of myenteric ganglions and interstitial cells of Cajal (ICC), and no significant findings in the mucosa. The exceptions were moderate to severe degree of eosinophilic infiltration at the myenteric ganglia/muscularis propria in EMG. Functional gastrointestinal obstruction may be associated with inflammatory cell infiltration at the myenteric ganglia/muscularis propria, leading to subsequent hypoganglionosis and deficiency of ICC in EMG. Pathologists and clinicians should be aware of this distinction during differential diagnosis of patients with CIPO.

Keywords

Eosinophilic myenteric ganglionitis Chronic intestinal pseudo-obstruction Auerbach plexus Myenteric ganglia Interstitial cells of Cajal α-Synuclein 

Notes

Acknowledgements

The authors thank Katsuya Miyahara and Keiko Mitani for their technical assistance and advice.

Author’s contributions

Yoichi Akazawa, Takuo Hayashi, and Tsuyoshi Saito conceived and designed the study and wrote, edited, and reviewed the manuscript. Koichiro Niwa and Hirohiko Kamiyama provided and gave patient’s clinical information. Noriko Sasahara provided histological and immunohistochemical technical assistance. All authors gave final approval for publication. Takuo Hayashi, Kazuhiro Sakamoto, Akihito Nagahara, and Takashi Yao take full responsibility for the work as a whole, including the study design, access to data, and the decision to submit and publish the manuscript.

Funding

This study was supported by Grant-in-Aid for the Japan Society for the Promotion of Science (JSPS) KAKENHI (Grant Number 17K08704 to TY).

Compliance with ethical standards

The information contained in, and preparation of, this manuscript complies with the journal’s ethical standards.

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Faulk DL, Anuras S, Christensen J (1978) Chronic intestinal pseudoobstruction. Gastroenterology 74:922–931Google Scholar
  2. 2.
    Antonucci A, Fronzoni L, Cogliandro L, Cogliandro RF, Caputo C, De Giorgio R, Pallotti F, Barbara G, Corinaldesi R, Stanghellini V (2008) Chronic intestinal pseudo-obstruction. World J Gastroenterol 14:2953–2961CrossRefGoogle Scholar
  3. 3.
    De Giorgio R, Sarnelli G, Corinaldesi R, Stanghellini V (2004) Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction. Gut 53:1549–1552CrossRefGoogle Scholar
  4. 4.
    Di Nardo G, Blandizzi C, Volta U, Colucci R, Stanghellini V, Barbara G, Del Tacca M, Tonini M, Corinaldesi R, De Giorgio R (2008) Review article: molecular, pathological and therapeutic features of human enteric neuropathies. Aliment Pharmacol Ther 28:25–42CrossRefGoogle Scholar
  5. 5.
    Di Nardo G, Di Lorenzo C, Lauro A, Stanghellini V, Thapar N, Karunaratne TB, Volta U, De Giorgio R (2017) Chronic intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options. Neurogastroenterol Motil.  https://doi.org/10.1111/nmo.12945
  6. 6.
    Lee H, Kang D, Kim H, Cho B, Jang J (2017) Unusual histology of eosinophilic myenteric ganglionitis: a case report. J Pathol Transl Med 51:320–324CrossRefGoogle Scholar
  7. 7.
    Ooms AH, Verheij J, Hulst JM, Vlot J, van der Starre C, de Ridder L, de Krijger RR (2012) Eosinophilic myenteric ganglionitis as a cause of chronic intestinal pseudo-obstruction. Virchows Arch 460:123–127CrossRefGoogle Scholar
  8. 8.
    Chander B, Fiedler P, Jain D (2011) Eosinophilic myenteric ganglionitis: a case of intestinal pseudo-obstruction in a 93-year-old female. J Clin Gastroenterol 45:314–316CrossRefGoogle Scholar
  9. 9.
    Schappi MG, Smith VV, Milla PJ, Lindley KJ (2003) Eosinophilic myenteric ganglionitis is associated with functional intestinal obstruction. Gut 52:752–755CrossRefGoogle Scholar
  10. 10.
    Knowles CH, De Giorgio R, Kapur RP, Bruder E, Farrugia G, Geboes K, Lindberg G, Martin JE, Meier-Ruge WA, Milla PJ, Smith VV, Vandervinden JM, Veress B, Wedel T (2010) The London classification of gastrointestinal neuromuscular pathology: report on behalf of the Gastro 2009 International Working Group. Gut 59:882–887CrossRefGoogle Scholar
  11. 11.
    Knowles CH, De Giorgio R, Kapur RP, Bruder E, Farrugia G, Geboes K, Gershon MD, Hutson J, Lindberg G, Martin JE, Meier-Ruge WA, Milla PJ, Smith VV, Vandervinden JM, Veress B, Wedel T (2009) Gastrointestinal neuromuscular pathology: guidelines for histological techniques and reporting on behalf of the Gastro 2009 International Working Group. Acta Neuropathol 118:271–301CrossRefGoogle Scholar
  12. 12.
    Veress B, Nyberg B, Törnblom H, Lindberg G (2009) Intestinal lymphocytic epithelioganglionitis: a unique combination of inflammation in bowel dysmotility: a histopathological and immunohistochemical analysis of 28 cases. Histopathology 54:539–549CrossRefGoogle Scholar
  13. 13.
    De Giorgio R, Barbara G, Stanghellini V, De Ponti F, Salvioli B, Tonini M, Velio P, Bassotti G, Corinaldesi R (2002) Clinical and morphofunctional features of idiopathic myenteric ganglionitis underlying severe intestinal motor dysfunction: a study of three cases. Am J Gastroenterol 97:2454–2459CrossRefGoogle Scholar
  14. 14.
    Amiel J, Sproat-Emison E, Garcia-Barcelo M, Lantieri F, Burzynski G, Borrego S, Pelet A, Arnold S, Miao X, Griseri P, Brooks AS, Antinolo G, de Pontual L, Clement-Ziza M, Munnich A, Kashuk C, West K, Wong KK, Lyonnet S, Chakravarti A, Tam PK, Ceccherini I, Hofstra RM, Fernandez R (2008) Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet 45:1–14CrossRefGoogle Scholar
  15. 15.
    Heuckeroth RO (2018) Hirschsprung disease - integrating basic science and clinical medicine to improve outcomes. Nat Rev Gastroenterol Hepatol 15:152–167CrossRefGoogle Scholar
  16. 16.
    Annerino DM, Arshad S, Taylor GM, Adler CH, Beach TG, Greene JG (2012) Parkinson’s disease is not associated with gastrointestinal myenteric ganglion neuron loss. Acta Neuropathol 124:665–680CrossRefGoogle Scholar
  17. 17.
    Recasens A, Carballo-Carbajal I, Parent A, Bové J, Gelpi E, Tolosa E, Vila M (2018) Lack of pathogenic potential of peripheral α-synuclein aggregates from Parkinson’s disease patients. Acta Neuropathol Commun 6:8CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Yoichi Akazawa
    • 1
    • 2
  • Takuo Hayashi
    • 1
  • Tsuyoshi Saito
    • 1
  • Koichiro Niwa
    • 3
  • Hirohiko Kamiyama
    • 3
  • Noriko Sasahara
    • 1
  • Kazuhiro Sakamoto
    • 3
  • Akihito Nagahara
    • 2
  • Takashi Yao
    • 1
  1. 1.Department of Human Pathology, School of MedicineJuntendo UniversityTokyoJapan
  2. 2.Department of Gastroenterology, School of MedicineJuntendo UniversityTokyoJapan
  3. 3.Department of Coloproctological Surgery, School of MedicineJuntendo UniversityTokyoJapan

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