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