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Surgical Management of Chiari Malformation Type I in the Pediatric Patients with Syringomyelia and Related to Klippel-Feil Syndrome: A Case Series and Literature Review Cover

Surgical Management of Chiari Malformation Type I in the Pediatric Patients with Syringomyelia and Related to Klippel-Feil Syndrome: A Case Series and Literature Review

Open Access
|Mar 2026

Abstract

Background

Chiari type I malformation (CM-1) is a birth defect where the back part of the skull does not develop properly, leading to the formation of a small and shallow space at the back of the head. The aim of this case series is to determine the causes and pathologies associated with Chiari type 1 malformation and the possible management and treatment, as well as the best outcomes that aid in the postoperative evolution of CM-1 decompression.

Case Presentation

A 13-year-old patient presented to the emergency department with severe, frequent, and daily headaches of five years’ duration. An 8-year-old girl presented to our clinic with severe headaches of one year’s duration. A 9-year-old boy presented to the clinic with weeks of severe headaches and numbness in the fingers of his right hand. MRI was performed, which diagnosed Arnold-Chiari syndrome type 1, fusion of the C1-C2 vertebrae, and triphalangeal fingers of the hand, which are associated with the Klippel-Feil syndrome (KFS).

Conclusion

CM-1 is associated with syringomyelia, which is more prominent in Case 2, whereas Case 3 is associated with Klippel–Feil syndrome; therefore, karyotyping and Sanger sequencing of the GDF6 gene were recommended.

DOI: https://doi.org/10.2478/amb-2026-0046 | Journal eISSN: 2719-5384 | Journal ISSN: 0324-1750
Language: English
Page range: 63 - 68
Submitted on: Aug 19, 2025
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Accepted on: Jan 8, 2026
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Published on: Mar 17, 2026
In partnership with: Paradigm Publishing Services
Publication frequency: 4 issues per year

© 2026 D. A. Encarnacion-Santos, G. Chmutin, I. B. Anvardzhonovich, E. Chmutin, B. Arstanbekov, Sh. Kurbonov, S. Bodanova, B. Chaurasia, published by Medical University - Sofia
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.