Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients.

Cerebrospinal fluid overdrainage in long-lasting shunted hydrocephalus is a well recognized complication. It may lead to different clinical syndromes which may occur over time, namely cranioencephalic disproportion and hindbrain herniation. Among the latter, Chiari malformation type 1 classically refers to one of the mildest form. When symptomatic, the patients generally exhibit a chronic, slowly progressive disease course. Although well documented in Chiari type II malformation and hydrocephalus, ventriculo-peritoneal shunt malfunction causing acute neurological deterioration in acquired Chiari malformation type 1 has been described only in three cases. All these patients underwent urgent shunt revision and suboccipital decompression in two of them, achieving good clinical improvement. We report on a 20-year-old woman harboring a previously asymptomatic Chiari malformation type 1, who experienced an acute foramen magnum syndrome with ventriculo-peritoneal shunt malfunction. We performed an endoscopic third-ventriculo-cisternostomy and definite removal of the shunt, obtaining the complete resolution of symptoms and shunt independence at 2 years follow-up. The phisiopathogenetic mechanisms and surgical management are discussed.

Late Intramedullary Spinal Metastases from Esthesioneuroblastoma: Case Report and Prognostic Implications.

BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon neuroectodermal tumor that originates from the olfactory mucosa and often recurs locally. Distant metastases of ENB have been described, but there are few reports of intramedullary metastases to the spinal cord. CASE DESCRIPTION: Here we report a case of a patient presenting with a progressive paraparesis and magnetic resonance imaging findings of multiple drop metastases to thoracic and lumbar regions of the spinal cord, 17 years after diagnosis and treatment for an intracranial ENB with subsequent neck nodal metastases. The dorsal symptomatic lesion was treated with resection, radiotherapy, and adjuvant chemotherapy. The implications of spinal metastases for the clinical prognosis of this disease are discussed, with a review of the few reported cases of spinal ENB metastases in the literature. CONCLUSIONS: Through the presentation of this case we hope to further contribute to a better understanding of this rare disease's prognosis.