CC BY-NC-ND 4.0 · Asian J Neurosurg 2024; 19(01): 094-096
DOI: 10.1055/s-0043-1772757
Case Report

A Rare Neurological Presentation of Noonan Syndrome and Its Management—A Case Report

1   Department of Anaesthesia and Intensive Care, PGIMER, Chandigarh, India
,
2   Department of Anaesthesia, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India
,
1   Department of Anaesthesia and Intensive Care, PGIMER, Chandigarh, India
,
1   Department of Anaesthesia and Intensive Care, PGIMER, Chandigarh, India
› Author Affiliations
Funding None.

Abstract

Although Noonan syndrome is a relatively common congenital disorder with autosomal dominant inheritance, its association with cerebrovascular anomalies is rare. We report a case of a 20-year-old with Noonan syndrome with cerebrovascular aneurysm, who underwent successful endovascular coiling. Only four cases of cerebrovascular aneurysms in Noonan syndrome have been reported in the literature so far. To the best of our knowledge, this is only the fifth reported case and the first one that has been treated successfully with endovascular coiling. We hereby discuss the management of this case, which had several comorbidities like congenital heart disease and craniovertebral junction anomaly.

Previous Presentation

Case report has been presented in Indian Society of Neuroanesthesiology and Critical care Conference on 22 January 2021.


Authors' Contributions

SM was involved in writing up of the first draft of the paper and final editing. VN was involved in editing of the draft, data collection, and initial draft preparation. AD was involved in data collection.




Publication History

Article published online:
26 March 2024

© 2024. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 
  • References

  • 1 Carcavilla A, Suárez-Ortega L, Rodríguez Sánchez A. et al. [Noonan syndrome: genetic and clinical update and treatment options]. An Pediatr (Engl Ed) 2020; 93 (01) 61.e1-61.e14 (English Edition)
  • 2 Zarate YA, Lichty AW, Champion KJ, Clarkson LK, Holden KR, Matheus MG. Unique cerebrovascular anomalies in Noonan syndrome with RAF1 mutation. J Child Neurol 2014; 29 (08) NP13-NP17
  • 3 Dineen RA, Lenthall RK. Aneurysmal sub-arachnoid haemorrhage in patients with Noonan syndrome: a report of two cases and review of neurovascular presentations in this syndrome. Neuroradiology 2004; 46 (04) 301-305
  • 4 Still M, Miller P, Dodd W. et al. Chiari I malformation and Noonan's syndrome: shared manifestations of RASopathy. J Clin Images Med Case Rep 2021; 2 (05) 1334
  • 5 Agrawal S, Salunke P, Gupta S. et al. Fiberoptic bronchoscopy versus video laryngoscopy guided intubation in patients with craniovertebral junction instability: a cinefluroscopic comparison. Surg Neurol Int 2021; 12: 92