Cervicomedullary arachnoid cyst causing bulbar dysfunction and quadriparesis in an infant: A case report and review of literature
Rios-Vicil, C.I.; Fernandez-Abinader, J.A.; Vigo-Prieto, J.A.
Boletin de la Asociacion Medica de Puerto Rico 110(1): 17-19
2018
ISSN/ISBN: 0004-4849 Document Number: 703076
Purpose: To highlight a rare cause of rapidly developing quadriparesis and bulbar palsy secondary to a cervicomedullary arachnoid cyst in an infant with suspected ventriculoperitoneal shunt malfunction. Case description: We report the case of a spinal arachnoid cyst causing bulbar dysfunction and quadriparesis in a 5 month old female. After initial evaluation and ruling out ventriculoperitoneal shunt malfunction, a Brain/Cervical MRI revealed a cervicomedullary ventral arachnoid cyst causing severe spinal cord compression resulting in rapidly progressive bulbar dysfunction and quadriparesis. C1 and partial C2 laminectomies were performed. Intraoperatively, a 1.3 × 1.2 × 2.6 cm cervicomedullary arachnoid cyst was seen severely compressing and displacing the spinal cord with associated stretching of spinal roots in a left to right direction. Partial cyst microdissection with wide fenestration and spinal cord decompression was achieved as evidenced by post-operative MRI. The patient had a stable post-operative course and return of baseline strength in upper and lower extremities as well as resolution bulbar symptoms. Clinical relevance: Anterior cervicomedullary arachnoid cysts are rare entities seen in the pediatric population with 7 cases reported in the scientific literature. A misdiagnosis or delay in treatment could result in further neurologic injury associated with increased morbidity and loss of function, and in rare cases, death. We aim to highlight this pathologic process in order to contribute to standardization of treatment of this disease which has a lack of controlled clinical studies and management guidelines. Further studies should provide which timing, treatment modality and follow up is optimal in this patient population.
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