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

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.