Ventral foramen magnum neurenteric cyst presenting as acute rapidly progressive quadriparesis and respiratory compromise: A case report and review of literature

摘要

Sir,Neurenteric cysts of the central nervous system are rare and result from failure of separation of the neuroectodermal and neuroendodermal elements during the third week of embryogenesis.[1/2] These cysts are extremely rare at the craniovertebral junction.11'31 An unexcised craniovertebral junction cyst maybe fatal.'11 We report a case of a ventral foramen magnum neurenteric cyst presenting with rapidly progressive neurologic" deficits.An 18-year-old girl noticed left hemiparesis after getting up from sleep. Over the next 12 hours, she developed similar weakness in her rightsided limbs. On admission, vital parameters were normal and single breath count was 26. Neurologic examination revealed quadriparesis with motor power of 3/5 in all four limbs; all deep tendon reflexes were brisk, jaw jerk was absent, and bilateral Babinski sign was present. There was no cranial nerve or sensory deficits. There were no bony anomalies in the radiograph of the cervical spine. Magnetic resonance imaging (MRI) of the cervical spine [Figure 1] showed a ventral foramen magnum cystic lesion measuring 1.2 x 1.3 x 1.2 cm compressing the cervicomedullary junction ventrally and displacing it posteriorly. Next morning, she developed breathing difficulty and further neurological deterioration. Respiratory rate was 36 breaths per minute with the activation of accessory muscles during inspiration and oxygen saturation was 99%. Motor power deteriorated to 2/5 in all four limbs. The patient was intubated ariditlked'on mechanical ventilation. She was taken up for emergency surgery and underwent a right far lateral approach, cyst decompression, and near-total excision of cyst wall as we failed to dissect the cyst wall free from the anterior surface of the cervicomedullary junction (due to dense adhesions). The cyst contained thick milky fluid. Triers was no evidence of hemorrhage or rupture of the cyst, Postoperatively, her respiration was normal and limbs power improved to 4/5. Microscopic examination [Figure 2]-showed that the cyst was lined with pseudostfatified efuboidal and columnar epithelium, thus confirming an enterogenous cyst.