Oral Rehabilitation as Part of a Multidisciplinary Treatment in a Case Study of Pigmentary Incontinence

We present the clinical course of a 9-year-old female patient with Bloch-Sulzberger syndrome and severe neurological deficit that met the major (classic cutaneous signs) and minor (dental anomalies and retinal pathology) diagnostic criteria of Landy and Donnai. Longitudinal multidisciplinary follow-up was carried out from birth to adulthood. Neurological involvement was assessed with electroencephalographic (EEG) and neuroimaging tests at different times during the patient's life. Cranio-maxillofacial involvement was evaluated using lateral skeletal facial and cephalometric analyses. The right and left facial widths were measured through frontal face analysis and using the vertical zygomatic-midline distance. Oral rehabilitation was performed through orthodontic treatment and major dental reconstruction using composite resins. This treatment aimed to improve the occlusion and masticatory function, relieve the transversal compression of the maxilla, and reconstruct the fractured teeth. We believe that, due to significant neurological and cognitive impairment, orthognathic surgery was not the best option for restoring function and improving oral health-related quality of life.

Automated summary

This article presents the clinical course of a 9-year-old girl with Bloch-Sulzberger syndrome and severe neurological deficit. Longitudinal multidisciplinary follow-up was conducted from birth to adulthood. Neurological involvement was assessed using EEG and neuroimaging tests, while cranio-maxillofacial involvement was evaluated using facial and cephalometric analyses. Oral rehabilitation included orthodontic treatment and dental reconstruction to improve occlusion and masticatory function.