Megalencephaly-capillary malformation syndrome and associated hydrocephalus: treatment options and revision of the literature
Por:
Alamar, M, Candela, S, Flor-Goikoetxea, A, Salvador, H, Martinez-Monseny, A, Muchart, J and Hinojosa, J
Publicada:
1 ago 2021
Ahead of Print:
1 may 2021
Resumen:
Purpose We describe our series of 4 patients with megalencephaly-capillary malformation syndrome (MCAP) and review the literature in order to assess the optimal treatment for the associated hydrocephalus. Methods We review our institutional series of hydrocephalus associated with MCAP and review the literature, analyzing the causes that could originate the hydrocephalus and the different types of treatments proposed for them. Results Of our patients treated with ventriculoperitoneal (VP) shunt, one suffered a surgical revision of the shunt and died due to a cranial trauma unrelated to her syndrome or the previous shunt surgery, and the other did not undergo surgical revisions until the end of her follow-up. Our patients treated with endoscopic third ventriculostomy (ETV) have improved their symptomatology and have not suffered of any complications related to the hydrocephalus after the ETV surgery. Conclusions We update the treatment of MCAP-associated hydrocephalus and propose ETV as a valid treatment, as it seems a safe procedure with a low rate of complications.
Filiaciones:
Alamar, M:
Hosp St Joan Deu, Neurosurg Dept, Passeig St Joan Deu S-N, Barcelona 08950, Spain
Candela, S:
Hosp St Joan Deu, Neurosurg Dept, Passeig St Joan Deu S-N, Barcelona 08950, Spain
:
Hosp La Ribera, Neurosurg Dept, Alzira, Spain
Salvador, H:
Hosp St Joan Deu, Oncol Dept, Barcelona, Spain
Martinez-Monseny, A:
Hosp St Joan Deu, Clin Genet Dept, Barcelona, Spain
Hosp St Joan Deu, Pediat Inst Rare Dis IPER, Barcelona, Spain
Muchart, J:
Hosp St Joan Deu, Radiol Dept, Barcelona, Spain
Hinojosa, J:
Hosp St Joan Deu, Neurosurg Dept, Passeig St Joan Deu S-N, Barcelona 08950, Spain
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