Encephalocele in a patient with Hurler syndrome: description and therapeutic alternatives review

Article Sidebar

PDF (Español (España))
Published: Mar 31, 2021
DOI: https://doi.org/10.37076/acorl.v50i1.579
Keywords:
mucopolisacaridosis I, encefalocele, colgajos quirúrgicos Mucopolysaccharidosis I, encephalocele, surgical flaps

Main Article Content

Andrés González Fernández
a:1:{s:5:"es_ES";s:70:"Servicio de Otorrinolaringología del Complejo Hospitalario de Navarra";}
Koro Zubimendi E
Miren Ondiz Arechaga O
Ignacio Sanhueza T
Sheila Huerga M

Abstract

Introduction: Hurler syndrome is the most severe version of mucopolysaccharidosis
I. The storage of dermatan and heparin sulfate in meninges allows the development
of hydrocephalus and meningoencephaloceles. Case report: We report a 23-year-old
female with this syndrome and a sinonasal encephalocele operated by endoscopic sinonasal
surgery and subsequent pericranial flap as support. Discussion: Endoscopic
sinonasal surgery has grown in last years in relation with treatment of sinonasal meningoencephaloceles
due to a high rate of success removing the lesion and closing
the subsequent cerebrospinal fluid fistula. Conclusion: Currently, the endoscopic
sinonasal surgery has become a useful tool in the management of skull base pathologies,
and reduces the morbidity due to a transcranial approach.

Downloads

Download data is not yet available.

Article Details

How to Cite
1.
González Fernández A, Zubimendi E K, Arechaga O MO, Sanhueza T I, Huerga M S. Encephalocele in a patient with Hurler syndrome: description and therapeutic alternatives review. Acta otorrinolaringol cir cabeza cuello [Internet]. 2021Mar.31 [cited 2024May18];50(1):73 - 76. Available from: https://www.revista.acorl.org.co/index.php/acorl/article/view/579
Issue
Vol. 50 No. 1 (2022): January - March
Section
Reportes de Casos
Creative Commons License

This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.

Este artículo es publicado por la Revista Acta de Otorrinolaringología & Cirugía de Cabeza y Cuello.

Este es un artículo de acceso abierto, distribuido bajo los términos de la LicenciaCreativeCommons Atribución-CompartirIgual 4.0 Internacional.( http://creativecommons.org/licenses/by-sa/4.0/), que permite el uso no comercial, distribución y reproducción en cualquier medio, siempre que la obra original sea debidamente citada.

eISSN: 2539-0856

ISSN: 0120-8411

References

Suarez-Guerrero JL, Gómez Higuera PJ, Arias Flórez JS, Contreras-García GA. Mucopolisacaridosis: características

clínicas, diagnóstico y de manejo. Rev Chil Pediatr.2016;87(4):295-304. doi: 10.1016/j.rchipe.2015.10.004.

Sakuru R, Bollu PC. Hurler Syndrome. 2022. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022.

Wraith JE, Jones S. Mucopolysaccharidosis type I. Pediatr Endocrinol Rev. 2014;12 Suppl 1:102-6.

Stavrakas M, Karkos PD, Triaridis S, Constantinidis J.Endoscopic management of paediatric meningoencephaloceles:

a case series. Eur Arch Otorhinolaryngol. 2018;275(11):2727-2731. doi: 10.1007/s00405-018-5116-z.

Greenfield JP, Anand VK, Kacker A, Seibert MJ, Singh A, Brown SM, et al. Endoscopic endonasal transethmoidal

transcribriform transfovea ethmoidalis approach to the anterior cranial fossa and skull base. Neurosurgery. 2010;66(5):883-92.doi: 10.1227/01.neu.0000368395.82329.c4.

Nyquist GG, Anand VK, Mehra S, Kacker A, Schwartz TH. Endoscopic endonasal repair of anterior skull base

non-traumatic cerebrospinal fluid leaks, meningoceles,and encephaloceles. J Neurosurg. 2010;113(5):961-6. doi:

3171/2009.10.JNS08986.

Bedrosian JC, Anand VK, Schwartz TH. The endoscopic endonasal approach to repair of iatrogenic and noniatrogenic

cerebrospinal fluid leaks and encephaloceles of the anterior cranial fossa. World Neurosurg. 2014;82(6 Suppl):S86-94. doi:

1016/j.wneu.2014.07.018.

Soudry E, Turner JH, Nayak JV, Hwang PH. Endoscopic reconstruction of surgically created skull base defects:

a systematic review. Otolaryngol Head Neck Surg. 2014;150(5):730-8. doi: 10.1177/0194599814520685.

Harvey RJ, Nogueira JF, Schlosser RJ, Patel SJ, Vellutini E, Stamm AC. Closure of large skull base defects after

endoscopic transnasal craniotomy. Clinical article. J Neurosurg. 2009;111(2):371-9. doi: 10.3171/2008.8.JNS08236.

Chin D, Harvey RJ. Endoscopic reconstruction of frontal, cribiform and ethmoid skull base defects. Adv Otorhinolaryngol. 2013;74:104-18. doi: 10.1159/000342285.

Gabriel PJ, Kohli G, Hsueh WD, Eloy JA, Liu JK. Efficacy of simultaneous pericranial and nasoseptal “double flap”

reconstruction of anterior skull base defects after combined transbasal and endoscopic endonasal approaches. Acta

Neurochir (Wien). 2020;162(3):641-647. doi: 10.1007/s00701-019-04155-1.

Tomio R, Toda M, Tomita T, Yazawa M, Kono M, Ogawa K, et al. Primary dural closure and anterior cranial base

reconstruction using pericranial and nasoseptal multi-layered flaps in endoscopic-assisted skull base surgery. Acta Neurochir (Wien). 2014;156(10):1911-5. doi: 10.1007/s00701-014-2174-x.