Radiological examination of cranial, ear-nose-throat, and maxillofacial system findings of Dyke-Davidoff-Masson syndrome in 28 patients

Abstract Objectives It was aimed to detail the intracranial and ear–nose–throat-maxillofacial (ENT-MF) system findings of Dyke–Davidoff–Masson Syndrome (DDMS), and to examine the association in a larger sample according to current literature. Patients and methods The study was designed retrospectively. Cranial magnetic resonance (MRI) and computed tomography (CT) Imaging records of patients were re-examined. Outcome parameters were identified as patients' demographics, the emergence of the central nervous system (CNS) pathologies, the asymmetric pneumatization of the skull bones, and ENT-MF system presentation. The correlation of the cranial and ENT-MF system findings was evaluated with Spearmen correlation analysis. Results Radiological images of 28 patients were examined. The number of men was 21 (75.0%) and the number of females was 7 (25.0%). The mean age of the patients was 23.6 ± 10.6 (min. 1, max. 44). DDMS involvement detected on the right side in 15 (53.6%) of the patients. Cerebral atrophy (n = 28, 100%), lateral ventricular dilatation (n = 26, 96.3%) and corpus callosum damage (n = 19, 67.9%) were most frequent findings respectively. Cerebellar atrophy (n = 4, 14.3) and venous sinus dominance (n = 4, 14.3) were the rarest pathologies. Facial asymmetry (n = 15, 53.6%) and frontal sinus hyperpneumatization (n = 15, 53.6%) were the most frequent findings in the ENT-MF system. Increased calvarial thickness showed a high positive correlation with facial asymmetry and frontal sinus hyperpneumatization (p = 0.002, r = 0.571 and 0.024, r = 0.424, respectively). Conclusion DDMS may occur with different radiological findings depending on the level of cerebral damage. Facial asymmetry often accompanies the disease and it should be considered in clinical practice of facial plastic and maxillofacial surgery.