Keywords
Arnold Chiari
Hydrocephalus
Pediatrics
Imaging Diagnosis
How to Cite
Abstract
Introduction
Arnold-Chiari Syndrome (AC) is a group of brain malformations characterized by
cerebellar herniation, with types I and II being most common in young patients.
Hydrocephalus is a frequent complication, leading to increased intracranial pressure
and clinical manifestations such as headache, neurodevelopmental delay, and
behavioral changes. Early recognition and management are critical to improve
outcomes.
Objective
This systematic review aimed to analyze the most effective diagnostic and therapeutic
approaches for hydrocephalus in pediatric patients with AC types I and II.
Methodology
Six articles published between 2020 and 2025 were selected from the PubMed
database, following PRISMA guidelines and the PICO strategy. Included studies were
original longitudinal, cohort, or case-control studies focusing on imaging diagnosis and
surgical treatment of hydrocephalus associated with AC.
Results
Chiari type I malformation associated with syringomyelia impairs thermal and pain
sensitivity, muscle strength, and upper limb coordination. Multiloculated
hydrocephalus presents a neurosurgical challenge but is not directly dependent on AC.
Imaging, especially MRI, identifies anatomical alterations such as basioccipital
convexity ≥ 4 mm and posterior atlanto-occipital membrane convexity, guiding surgical
intervention. Endoscopic fenestration is effective in connecting compartments and
reducing hydrocephalus, while posterior fossa decompression is successful in type I AC.
Prenatal repair of myelomeningocele in type II AC reduces hydrocephalus and prevents
cerebellar herniation progression. Prognosis depends on timing and type of
intervention, presence of ventriculomegaly, and continuous postoperative monitoring.
Persistent postoperative hydrocephalus correlates with adverse outcomes.
Conclusion
Management of hydrocephalus in young AC patients requires early diagnosis, detailed
imaging, and personalized therapeutic strategies, prioritizing endoscopic and
decompressive techniques. Prenatal interventions should be considered when
indicated. Further studies are needed to standardize protocols and improve survival
and quality of life.
Keywords: Hydrocephalus, Arnold-Chiari Syndrome, Pediatrics, Imaging Diagnosis,
Neurosurgery.
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Copyright (c) 2025 Mariana Stefani, Maria Cristina Regina Canali, Thais Kreps Schönhofen , Ana Laura Macedo Sokoloski , Edylaine Marquetti camara, Paula Iaizzo Magalhães
