Clinical features and long-term outcomes of pediatric spinal cord cavernous malformation-a report of 18 cases and literature review.


Journal

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
ISSN: 1433-0350
Titre abrégé: Childs Nerv Syst
Pays: Germany
ID NLM: 8503227

Informations de publication

Date de publication:
01 2021
Historique:
received: 09 05 2020
accepted: 21 05 2020
pubmed: 28 6 2020
medline: 25 6 2021
entrez: 28 6 2020
Statut: ppublish

Résumé

Pediatric intramedullary spinal cord cavernous malformation (ISCM) is a rare vascular disease with unclear natural history and long-term outcomes. We aim to determine the demographics, hemorrhagic risk, and long-term outcomes of this rare entity. A retrospective review of clinical data and treatment outcomes of pediatric patients treated with ISCM in our institution from 3/2000 to 3/2017 was conducted. In addition, we performed a systematic review of the literature on pediatric ISCM. Eighteen consecutive pediatric patients were included, with an average age of 12.9 ± 4.7 years (range: 4-18 years) and 66.7% being male. Locations were equally distributed in cervical and thoracic segments, with mean extension of 1.3 ± 0.7 segments. Clinical manifestation included extremity weakness (n = 15, 83.3%), pain (n = 10, 55.6%), sensory disorders (n = 8, 44.4%), sphincter disturbance (n = 6, 33.3%), muscular atrophy (n = 3, 16.7%), and spinal deformity (n = 1, 5.6%). Most patients presented with acute symptoms (n = 11, 61.1%), and 7 (38.9%) of them had severe neurological deficits. The annual retrospective hemorrhagic risk was 7.7 per patient-year. Two patients received conservative management, with one improved neurologically and the other remained unchanged. Total resection was achieved in 12 (75%) of the 16 surgical cases, with 8 patients (50%) improved their clinical outcomes, 7 patients (43.8%) remained unchanged, and 1 (6.3%) worsened. During follow-up, one patient had relapse of ISCM. Pediatric ISCM appears to have higher hemorrhage risk than their adult counterparts, and they can benefit from surgery whether in the acute phase of neurological deterioration or after clinical recuperation.

Identifiants

pubmed: 32591875
doi: 10.1007/s00381-020-04700-9
pii: 10.1007/s00381-020-04700-9
doi:

Types de publication

Journal Article Systematic Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

235-242

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Auteurs

Liang Zhang (L)

Medical School of Nankai University, No. 94, Weijin Road, Naikai District, Tianjin, 300071, People's Republic of China.
Department of Neurosurgery, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.

Guangyu Qiao (G)

Department of Neurosurgery, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.

Wuyang Yang (W)

Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD, USA.

Aijia Shang (A)

Department of Neurosurgery, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.

Xinguang Yu (X)

Medical School of Nankai University, No. 94, Weijin Road, Naikai District, Tianjin, 300071, People's Republic of China. yuxinguang_301@163.com.
Department of Neurosurgery, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China. yuxinguang_301@163.com.

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