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

Abstract

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.

Keywords: Chiari-I malformation; Magnetic resonance imaging; Syringobulbia; Syringocephaly; Syringohydromyelia.

Fig. 1

: Initial brain MRI Axial T2W (A), coronal T2W(B) and sagittal T2W(C&D), axial FLAIR (E&F), axial contrast enhanced T1W(G) images of the brain and spine shows a well-defined, lobulated, longitudinally oriented cystic cavity involving left centrum semi ovale (white arrow), basal ganglia, posterior limb of internal capsule (black arrowheads), brainstem (black arrow) and cervical cord (white arrowhead), with surrounding vasogenic edema.

Fig. 2

Post-op follow up brain MRI, axial T2W (A) and sagittal T2W(B) images shows suboccipital craniectomy defect with overlying pseudomeningocele (arrow heads), marked interval radiologic reduction in the intracranial cystic cavity with remaining slit like cavity in the posterior limb of internal capsule and brain stem (white arrow) and relatively stable cervical syringomyelic cavity(black arrow).