Chiari Malformation: 15 Cases and Review of the Literature

Abstract

Chiari malformations represent the main anomalies of nerve structures and result in a low position of the cerebellar tonsils within the upper part of the cervical spinal canal with or without other associated intracranial or extracranial defects such as hydrocephalus, syrinx, encephalocele or spinal dysraphism. Our work is a retrospective study spread over 5 years; from January 2015 to December 2019, involving 15 patients with Chiari I or II malformation, hospitalized within the two neurosurgery departments of the Ibn Rochd University Hospital of Casablanca. A male predominance was observed, with 9 males and 6 females. 12 patients had Chiari I malformation, and 3 patients had Chiari II (age range: 14–48; mean age: 33). Time to diagnosis ranged from 2 to 25 years, with an average of 10 years. Revealing signs were dominated by motor disorders (73%), sensory disorders (73%) and painful symptoms (53%). Clinical examination of our patients revealed a pyramidal syndrome in 20% of cases, a motor deficit and syringomyelia dissociation in 60% of patients, damage to the cranial pairs and scoliosis were noted in 13% of cases, and 6% of patients had distal amyotrophy. MRI was the examination of choice due to its specificity and sensitivity. It confirmed the diagnosis of Chiari malformation, and syringomyelia was noted in 60% of cases. CT remains advantageous for exploring bone anomalies, which were noted in 26% of cases. All patients underwent craniocervical decompression with dural reconstruction. Shortterm outcomes were generally favorable. Improvement was observed in 73% of cases and stabilization in 13%. No deaths were reported. Surgical treatment is currently the only option, and surgical techniques are constantly evolving to guarantee stable and prolonged improvement and to reduce the risk of morbidity and surgical complications.