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Neuropediatrics 2021; 52(06): 423-430
DOI: 10.1055/s-0041-1731801
Review Article

Subdural Hygroma in an Infant with Marfan's Syndrome

Lisa Ballmann
1   Department for Pediatrics III (Pediatric Cardiology), Medical University of Innsbruck, Innsbruck, Austria
,
Sabine Scholl-Bürgi
2   Department for Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria
,
Thomas Karall
1   Department for Pediatrics III (Pediatric Cardiology), Medical University of Innsbruck, Innsbruck, Austria
,
Irena Odri Komazec
1   Department for Pediatrics III (Pediatric Cardiology), Medical University of Innsbruck, Innsbruck, Austria
,
Daniela Karall
2   Department for Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria
,
Miriam Michel
1   Department for Pediatrics III (Pediatric Cardiology), Medical University of Innsbruck, Innsbruck, Austria
› Author Affiliations Funding None.
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Abstract

Based on a patient encounter in which genetically confirmed Marfan's syndrome (MFS) underlay a spontaneously resolving subdural hygroma (SDHy) diagnosed in infancy, we review the literature of MFS clinically manifest in early life (early-onset MFS [EOMFS]) and of differential diagnoses of SDHy and subdural hemorrhage (SDHe) at this age. We found that rare instances of SDHy in the infant are associated with EOMFS. The most likely triggers are minimal trauma in daily life or spontaneous intracranial hypotension. The differential diagnosis of etiologies of SDHy include abusive and nonabusive head trauma, followed by perinatal events and infections. Incidental SDHy and benign enlargement of the subarachnoid spaces must further be kept in mind. SDHy exceptionally also may accompany orphan diseases. Thus, in the infant, EOMFS should be considered as a cause of SDHe and/or SDHy. Even in the absence of congestive heart failure, the combination of respiratory distress syndrome, muscular hypotonia, and joint hyperflexibility signals EOMFS. If EOMFS is suspected, monitoring is indicated for development of SDHe and SDHy with or without macrocephaly. Close follow-up is mandatory.

Keywords

child abuse - infant - Marfan's syndrome - neonate - subdural hemorrhage - subdural hygroma

Article Summary

Through case presentation and literature review, this article provides information on subdural hemorrhage and hygroma formation in pediatric patients with early-onset Marfan's syndrome.


Authors' Contributions

M.M. and L.B. conceptualized and designed the work, collected and interpreted data, and drafted the manuscript. D.K. and S.S.-B. conceptualized and supervised the work, performed metabolic analyses and interpretation, and revised the article critically for important intellectual content. I.O.K. and T.K. conceptualized the work, performed cardiologic examinations and interpretation, and revised the article critically. All authors read and approved the final version of the manuscript as submitted and agree to be accountable for all aspects of the work.


Supplementary Material



Publication History

Received: 19 January 2021

Accepted: 05 April 2021

Article published online:
07 July 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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