201. Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients
- Author
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Filippo Maria Fava, Andrea Pagano, Mario Francesco Fraioli, Giuseppe Emmanuele Umana, Pierpaolo Lunardi, and Federica Novegno
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Chiari malformation ,Settore MED/27 ,Asymptomatic ,03 medical and health sciences ,0302 clinical medicine ,Cerebrospinal fluid ,foramen magnum syndrome ,medicine ,hydrocephalus ,ventriculo-peritoneal shunt ,Foramen magnum ,business.industry ,Shunt malfunction ,General Medicine ,medicine.disease ,Shunt (medical) ,Hydrocephalus ,Surgery ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Neurology (clinical) ,medicine.symptom ,business ,Complication ,030217 neurology & neurosurgery - Abstract
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.
- Published
- 2019
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