Syringohydromyelia, syringobulbia and syringocephaly associated with Chiari - I malformation: A case report
Nebil A Shukralla1, Etsehiwot Demeke2, Fitsum A Gemechu1
1School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia.
Insights
Chiari-I malformation can cause rare syringohydromyelia, syringobulbia, and syringocephaly. Early surgical decompression significantly improved a patient
Area of Science:
- Neurology
- Neurosurgery
- Radiology
Background:
- Chiari-I malformation (CM-I) is often associated with syringomyelia.
- Syringobulbia and syringocephaly, rostral extensions of syrinx cavities into the brainstem and cerebral parenchyma, are exceptionally rare CM-I manifestations.
Observation:
- A 40-year-old female presented with progressive hemiparesis, sensory disturbances, and bulbar symptoms.
- Magnetic resonance imaging (MRI) confirmed CM-I with extensive syringohydromyelia, syringobulbia, and syringocephaly involving the brainstem and left cerebral hemisphere.
- The syrinx cavity extended from the cervicomedullary junction to the thoracic spinal cord.
Findings:
- Posterior fossa decompression surgery was performed.
- The patient showed significant neurological improvement post-surgery, including motor function recovery and reduced intracranial edema.
- The cervical syrinx cavity remained stable, while rostral extensions showed improvement.
Implications:
- This case underscores the importance of early diagnosis and surgical management for rare CM-I complications.
- Prompt intervention can lead to substantial neurological recovery, even in complex and extensive syringomyelia presentations.
- Highlights the potential for positive outcomes in managing severe syringobulbia and syringocephaly associated with CM-I.
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.
