The prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. Chemotherapy has been ineffective in the few patients in whom it has been tried.
The posterior sagittal approach is an easy and safe surgical technique for the treatment of ASM, as it allows a complete isolation of the lesion and the removal of associated tumors without significant morbidity.
A 2-year-10-month-old girl presented with high fever and vomiting. One year ago, she had a period of muscular weakness in both legs that recovered completely. MRI of the brain revealed an axial enhancing lesion with ventricular spreading mainly to the left occipital horn and bilateral frontal periventricular infiltration. After steroid therapy, all the symptoms recovered. Partial removal of the occipital intraventricular lesion was performed and the diagnosis of Rosai-Dorfman disease was established and confirmed by the reference center. At the latest follow-up (16 months), the girl is without any neurological symptoms and without any treatment.
The operation was carried out by a multidisciplinary team in a single stage. The operating team included two distinct teams of neurosurgeons and anesthesiologists as well as a plastic surgeons' team. Both babies survived the operation. The postoperative course was uneventful. Both twins were discharged in good clinical and neurological condition after a 3-week period of hospitalization.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.