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ISSN 2539-0859 (Electronic)
ISSN 0120-8411 (Print)
Asociación Colombiana de Otorrinolaringología y Cirugía de Cabeza y cuello, Maxilofacial y Estética Facial (ACORL)

2022, Number 1

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Acta de Otorrinolaringología CCC 2022; 50 (1)

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

González FA, Zubimendi EK, Ondiz AOM, Sanhueza TI, Huerga MS

Language: Spanish
References: 12
Page: 73-76
PDF size: 204.73 Kb.


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.


REFERENCES

  1. Suarez-Guerrero JL, Gómez Higuera PJ, Arias Flórez JS,Contreras-García GA. Mucopolisacaridosis: característicasclínicas, diagnóstico y de manejo. Rev Chil Pediatr.

  2. 2016;87(4):295-304. doi: 10.1016/j.rchipe.2015.10.004.2. Sakuru R, Bollu PC. Hurler Syndrome. 2022. In: StatPearls[Internet]. Treasure Island (FL): StatPearls Publishing; 2022.

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

  4. 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.

  5. Greenfield JP, Anand VK, Kacker A, Seibert MJ, Singh A,Brown SM, et al. Endoscopic endonasal transethmoidaltranscribriform transfovea ethmoidalis approach to the anteriorcranial fossa and skull base. Neurosurgery. 2010;66(5):883-92.doi: 10.1227/01.neu.0000368395.82329.c4.

  6. Nyquist GG, Anand VK, Mehra S, Kacker A, SchwartzTH. Endoscopic endonasal repair of anterior skull basenon-traumatic cerebrospinal fluid leaks, meningoceles,and encephaloceles. J Neurosurg. 2010;113(5):961-6. doi:10.3171/2009.10.JNS08986.

  7. Bedrosian JC, Anand VK, Schwartz TH. The endoscopicendonasal approach to repair of iatrogenic and noniatrogeniccerebrospinal fluid leaks and encephaloceles of the anteriorcranial fossa. World Neurosurg. 2014;82(6 Suppl):S86-94. doi:10.1016/j.wneu.2014.07.018.

  8. Soudry E, Turner JH, Nayak JV, Hwang PH. Endoscopicreconstruction of surgically created skull base defects:a systematic review. Otolaryngol Head Neck Surg.2014;150(5):730-8. doi: 10.1177/0194599814520685.

  9. Harvey RJ, Nogueira JF, Schlosser RJ, Patel SJ, VellutiniE, Stamm AC. Closure of large skull base defects afterendoscopic transnasal craniotomy. Clinical article. J Neurosurg.2009;111(2):371-9. doi: 10.3171/2008.8.JNS08236.

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

  11. Gabriel PJ, Kohli G, Hsueh WD, Eloy JA, Liu JK. Efficacyof simultaneous pericranial and nasoseptal “double flap”reconstruction of anterior skull base defects after combinedtransbasal and endoscopic endonasal approaches. ActaNeurochir (Wien). 2020;162(3):641-647. doi: 10.1007/s00701-019-04155-1.

  12. Tomio R, Toda M, Tomita T, Yazawa M, Kono M, OgawaK, et al. Primary dural closure and anterior cranial basereconstruction using pericranial and nasoseptal multi-layeredflaps in endoscopic-assisted skull base surgery. Acta Neurochir(Wien). 2014;156(10):1911-5. doi: 10.1007/s00701-014-2174-x.




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