Symptomatic Multi-Nodular Fourth Ventricular Choroid Plexus Papillary Fibrosis in the Context of Chiari II Malformation: Case Report and Literature Review

Abstract

Introduction: Chiari II malformation (CM-II) is a congenital malformation characterized by the caudal displacement of the cerebellar tonsils, inferior cerebellar vermis, and fourth ventricle through the foramen magnum. Although rare, the association between CM-II and the development of a variety of mass lesions within the fourth ventricle and craniocervical junction has been described in the literature. Case Presentation: We present the case of a 9-year-old boy with a history of mid-lumbar myelomeningocele, CM-II, and ventriculoperitoneal shunt-dependent hydrocephalus who was discovered to have a syringomyelia and numerous spherical/ovoid masses within the caudal fourth ventricle and dorsal cervicomedullary subarachnoid space on a surveillance brain MRI. On questioning, the patient endorsed a longstanding history of poor bilateral hand dexterity and grip strength. After further imaging workup, the patient underwent suboccipital craniectomy and C1 laminectomy for resection of the mass lesions arising from the fourth ventricular choroid plexus (CP) and performance of an expansile pericranial duraplasty. The patient tolerated surgery well and had progressive improvement in hand dexterity/strength as well as radiographic improvement in the cervical cord syrinx after surgery. Pathologic analysis of the resected mass lesions demonstrated the lesions to be predominantly characterized by dense nodular fibrosis of the CP stromal cores which we are describing as choroid plexus papillary fibrosis. A minority of the lesional stromal cores were noted to be edematous, imparting a more “reticular” appearance. There were no features to suggest a neoplastic or infectious process. Conclusion: This combination of dysplastic/reactive CP histologic findings has not been previously reported in an extramedullary location. The unique pathology of this patient’s CP lesions will be discussed, and previously reported fourth ventricular mass lesions seen in association with CM-II will be reviewed.