Sella turcica measurements on lateral cephalograms of patients with neurofibromatosis type 1.

The aim of this study was to measure line segments and areas of sella turcica on lateral cephalograms with respect to the clinical diagnosis of facial phenotype of patients with neurofibromatosis type 1 (NF1). Special attention was given to correlate the measured values with certain tumour types that are typical for this disease. Lateral cephalograms of 194 individuals were investigated. Patients with NF1 were further divided according to the detection and topography of facial plexiform neurofibromas (PNF) taking into account the distribution pattern of the trigeminal nerve. All patients with PNF showed unilateral tumour localisation. Patients without any facial PNF constituted a separate group. Healthy volunteers with ideal occlusion and no history of any intervention in the maxillofacial region served as a control group. The following items were determined on the radiographs: sella entrance, sella width, sella depths, sella diagonal, and sella area. Patients with PNF of the first and second trigeminal nerve branch or affected in all branches showed highly statistically significant enlarged sella tucica measurement values. On the other hand, patients with PNF restricted to one branch only or simultaneously in the second and third branches showed measurement values that were not different to those obtained in NF1 patients devoid of facial PNF. The latter group also showed no difference of sella turcica parameters obtained in the control group. This study provides evidence for the association of a certain NF1 phenotype with distinct skeletal alterations of the skull base, shown here using the example of the representation of the sella turcica in the lateral radiograph. These findings are also relevant in the discussion of NF1 as a disease of bones and in the assessment of brain development in NF1. Both items are discussed in relationship to a facial plexiform neurofibroma. Furthermore, the knowledge of this association of findings provides the clinician with relevant information in the planning of skull base procedures in these patients.