Chronic subdural hematoma: A previously unreported life-threatening complication in adult with Sotos syndrome.
Sotos syndrome
adult
chronic subdural hematoma
macrocephaly
middle meningeal artery embolization
Journal
American journal of medical genetics. Part A
ISSN: 1552-4833
Titre abrégé: Am J Med Genet A
Pays: United States
ID NLM: 101235741
Informations de publication
Date de publication:
12 2020
12 2020
Historique:
received:
25
06
2020
revised:
28
08
2020
accepted:
02
09
2020
pubmed:
15
10
2020
medline:
25
6
2021
entrez:
14
10
2020
Statut:
ppublish
Résumé
Macrocephaly, defined as head circumference ≥ 2 SDs, is a cardinal feature of Sotos syndrome (SS) and generally persists in adulthood. Subdural fluid collection, typically associated with macrocephaly, is described in children due to anatomical conformation, and in adulthood due to brain atrophy and ex-vacuo hydrocephalus. On the other hand, a true, symptomatic, chronic subdural hematoma (CSH) is a previously unreported complication of SS in adulthood. Here we describe the first SS patient presenting symptomatic CSH, leading to frequent hospitalizations for surgical evacuations that consistently recurred. Middle meningeal artery (MMA) embolization and epidural blood patch (EBP) allowed to resolve the CSH with complete resolution of clinical signs and symptoms. We hypothesize that appearance and recurrences of CSH may be related to pathological biomechanics of brain, cerebro-spinal fluid and skull, secondary to anatomical features of SS. In this context, surgical evacuation may be less efficient than usual to cure CSH. Alternative treatment to avoid blood extravasation, as MMA embolization, or to cure concurrent causes of the pathology, as EBP, may be considered.
Identifiants
pubmed: 33052010
doi: 10.1002/ajmg.a.61900
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
3052-3055Informations de copyright
© 2020 Wiley Periodicals LLC.
Références
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