A Rare Case of Nasal Polyposis in Hurler Syndrome

W. Lendoye *

ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Casablanca, Morocco.

S. Moujrid

ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Casablanca, Morocco.

Y. Oukessou

ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Casablanca, Morocco.

R. Abada

ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Casablanca, Morocco.

M. Mahtar

ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Casablanca, Morocco.

*Author to whom correspondence should be addressed.


Abstract

Mucopolysaccharidosis (MPS) type I 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 surgeries as adenotonsillectomy. Nasal polyposis is a rare occurrence.

The aim of this work is to present a very rare association observed in an 8-years-old patient with Hurler syndrome treated in the ENT Head and Neck Surgery department of Ibn Rochd University Hospital Casablanca, for nasal polyposis and adenoid hypertrophy.

Keywords: Mucopolysaccharidosis (MPS) I, glycosaminoglycan (GAG), nasal polyps


How to Cite

Lendoye , W., S. Moujrid, Y. Oukessou, R. Abada, and M. Mahtar. 2023. “A Rare Case of Nasal Polyposis in Hurler Syndrome”. Asian Journal of Case Reports in Surgery 6 (2):342-45. https://journalajcrs.com/index.php/AJCRS/article/view/430.


References

Arn P, Bruce IA, Wraith JE, Travers H, Fallet S. Airway-related symptoms and surgeries in patients with mucopolysaccharidosis I. Ann Otol Rhinol Laryngol. 2015;124(3):198-205. DOI:10.1177/0003489414550154

MacArthur CJ, Gliklich R, McGill TJI, Perez-Atayde A. Sinus complications in mucopolysaccharidosis I H/S (Hurler-Scheie syndrome). International Journal of Pediatric Otorhinolaryngology. 1993;26(1): 79-87. DOI:10.1016/0165-5876(93)90199-D

Martins AM, Dualibi AP, Norato D, et al. Guidelines for the management of mucopolysaccharidosis type I. J Pediatr. 2009;155(4 Suppl):S32-46. DOI:10.1016/j.jpeds.2009.07.005

Murgasova L, Jurovcik M, Jesina P, et al. Otorhinolaryngological manifestations in 61 patients with mucopolysaccharidosis. Int J Pediatr Otorhinolaryngol. 2020;135: 110137. DOI:10.1016/j.ijporl.2020.110137

Kiely BT, Kohler JL, Coletti HY, Poe MD, Escolar ML. Early disease progression of Hurler syndrome. Orphanet J Rare Dis. 2017;12:32. DOI:10.1186/s13023-017-0583-7

Hampe CS, Yund BD, Orchard PJ, Lund TC, Wesley J, McIvor RS. Differences in MPS I and MPS II Disease Manifestations. Int J Mol Sci. 2021; 22(15):7888.

DOI:10.3390/ijms22157888

Kabekkodu S, Sreedharan S, Gupta K, Murali N. Nasal Polyposis in mucopolysaccharidosis type II. BMJ Case Rep. 2021;14(3):e238778. DOI:10.1136/bcr-2020-238778

Keilmann A, Läßig AK, Pollak-Hainz A, Mann WJ, Beck M, Hainz M. Adenoids of patients with mucopolysaccharidoses demonstrate typical alterations. Int J Pediatr Otorhinolaryngol. 2015;79(2): 115-118. DOI:10.1016/j.ijporl.2014.11.014

Hampe CS, Wesley J, Lund TC, et al. Mucopolysaccharidosis Type I: Current Treatments, Limitations, and Prospects for Improvement. Biomolecules. 2021;11(2): 189.

DOI:10.3390/biom11020189

Lee CL, Lee KS, Chuang CK, et al. Otorhinolaryngological Management in Taiwanese Patients with Mucopolysaccharidoses. Int J Med Sci. 2021;18(15):3373-3379.

DOI:10.7150/ijms.61827