Dyke-Davidoff-Masson syndrome, a rare entity in pediatrics. About a case

Abstract

Dyke-Davidoff-Masson syndrome is a clinical-radiological entity characterized by loss of the volume of a cerebral hemisphere with ipsilateral hypertrophy of the skull, elevation of the sphenoid wing and the petrosal edge of the temporal bone, as well as hyperpneumatization of the frontal sinus and mastoid cells associated with contralateral hemiplegia, facial asymmetry, seizures and intellectual disability. Initially described in 1933 by Dyke, Davidoff, and Masson. It mainly affects the pediatric age, and is attributed to an intrauterine or perinatal insult that affects the perfusion of a cerebral hemisphere. It may be of congenital or acquired origin (infectious, ischemic, hemorrhagic or tumor); its frequency is unknown since the diagnosis is  usually established at ages outside the pediatric age range. The correlation between the clinical and neuroimaging characteristics are fundamental for its correct identification. Management is mainly symptomatic, including  anticonvulsant medication for epilepsy and infant rehabilitation. We describe an unusual case with clinical and imaging features consistent with an early diagnosis of Dyke-Davidoff-Masson syndrome with maternal history of
in utero damage risk.