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.

Novegno, F., Pagano, A., Fava, F., Umana, G., Lunardi, P., Fraioli, M.f. (2019). Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients. BRITISH JOURNAL OF NEUROSURGERY, 1-5 [10.1080/02688697.2019.1691977].

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

Pagano A.;Fava F.;Umana G.;Lunardi P.;
2019-01-01

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.
2019
Pubblicato
Rilevanza internazionale
Articolo
Comitato scientifico
Settore MED/27 - NEUROCHIRURGIA
English
Chiari malformation; foramen magnum syndrome; hydrocephalus; ventriculo-peritoneal shunt
Novegno, F., Pagano, A., Fava, F., Umana, G., Lunardi, P., Fraioli, M.f. (2019). Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients. BRITISH JOURNAL OF NEUROSURGERY, 1-5 [10.1080/02688697.2019.1691977].
Novegno, F; Pagano, A; Fava, F; Umana, G; Lunardi, P; Fraioli, Mf
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/233280
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