Abstract
Objective Justify multi-disciplinary team policy for Follow-up
PRS/PFA is a rare condition with variable presentation though progressive unilateral wasting of the face is the hallmark. It affects females more than males and the left side more than the right and may present to dermatologists, dentists, plastic surgeons, rheumatologists or neurologists. Cause is unclear and symptoms are treated on their merit. Mood is commonly affected and consensus is developing that it should be managed in a multi-disciplinary team setting, including psychologist.
An 18yr old female presented with mild left face wasting. Neurologic examination revealed a mild scoliosis and no morphea.
Childhood PRS/PFA is managed closely with whole-family involvement. Extra-cranial manifestations (ECM) occur in over 40%. Findings of: progressive intra-cranial atrophy, T2 hyperintensities, dementia, epilepsy, calcification, bleeding, trigeminal neuralgia, headaches, movement disorders; numerous eye complications; dental, Temporo-mandibular joint, joint and skin (morphea and sclerosis) – can arise at any time from diagnosis.
As a rare condition, with limited physician exposure, protocols for review differ for each specialty. With around a hundred ECM possible – spread over different fields of expertise, a unified approach would make for efficient use of time and resources over this long-term disease process. Progression of PRS can be expected mainly in the first 10 years. Recurrences and progression, after years of stability, have been reported.
Conclusion Recommendations have been made for: team review each 3–6 months for the first 10 years; MRI each 12–24 months; eye review each 6 months; 3D photography each 6 months
