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Case report
Nasal Polyposis in mucopolysaccharidosis type II
  1. Sushmitha Kabekkodu1,
  2. Suja Sreedharan2,
  3. Kirti Gupta3 and
  4. Nirupama Murali4
  1. 1Otorhinolaryngology, Kasturba Medical College Mangalore, Manipal Academy of Higher Eduction, Mangalore, Karnataka, India
  2. 2Otorhinolaryngology, Kasturba Medical College Mangalore, Manipal Academy of Highe Education, Mangalore, Karnataka, India
  3. 3Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  4. 4Pathology, Kasturba Medical College Mangalore, Manipal Academy of Higher Education, Mangalore, Karnataka, India
  1. Correspondence to Dr Suja Sreedharan; suja.sreedharan{at}manipal.edu

Abstract

Mucopolysaccharidosis (MPS) type II is a rare multisystem disorder resulting from the accumulation of breakdown products of glycosaminoglycans in the body tissues. Many patients with this disease undergo ENT (ear, nose and throat) surgeries such as adenotonsillectomy and tympanocentesis at a very early age, much before the diagnosis of MPS. Nasal polyposis is a rare occurrence, with only one case of MPS II with polyposis reported in the literature. We present a patient who presented with recurrent nasal polyposis from the age of 2 years. Hale’s colloidal iron was used to stain these ‘nasal polyps’, which revealed that they are, in fact, mucopolysaccharide-laden sinonasal mucosa prolapsing into the nasal cavities. We believe this is the first time that this stain has been used to stain nasal polyps in MPS. In addition to the histopathological peculiarities of these nasal masses, we also discuss the natural history of nasal polyposis in MPS II.

  • ear
  • nose and throat
  • nasal polyps
  • pathology
  • otolaryngology / ENT
  • genetics

https://doi.org/10.1136/bcr-2020-238778

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    Footnotes

    • Contributors SK and SS contributed to the paper work-up, including discussion. KG provided valuable opinion on histopathology along with materials. NM provided materials for histopathology.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.