Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari – I malformation: A case report

Syringohydromyelia, syringobulbia, and syringocephaly are rare manifestations of Chiari-I malformation (CM-I), each resulting from disrupted cerebrospinal fluid dynamics. While syringomyelia is commonly associated with CM-I, the rostral extension of syrinx cavities into the brainstem (syringobulbia) and cerebral parenchyma (syringocephaly) is very rare, with limited cases reported globally. This case report describes a 40-year-old woman presenting with progressive neurological deficits, including hemiparesis, sensory disturbances, and bulbar symptoms. Magnetic resonance imaging revealed CM-I with a syringohydromyelic cavity extending from the cervicomedullary junction to the thoracic spinal cord, syringobulbia involving the brainstem, and syringocephaly affecting the left cerebral hemisphere, internal capsule, and basal ganglia. Following posterior fossa decompression, the patient experienced significant neurological improvement, including restored motor function and resolution of intracranial edema, although the cervical syrinx cavity remained stable. This case demonstrates the importance of early diagnosis and surgical intervention in managing these rare yet debilitating complications of CM-I, highlighting the potential for improved outcomes despite the complex and extensive nature of these lesions.