Retrospective study of photodynamic therapy for the GNA-Related Capillary Malformation spectrum of pediatric patients.
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BACKGROUND: Vascular malformations caused by GNAQ/GNA11 somatic mutations are related to capillary malformations (CM), which include port-wine stains (PWS), and also include capillary malformation-related syndromes, such as Sturge-Weber syndrome (SWS), capillary malformation with overgrowth (CMO), diffuse capillary malformation with overgrowth (DCMO), and phakomatosis pigmentovascularis (PPV). These disorders are known as the GNA-Related Capillary malformation spectrum (GNARCMs). This spectrum presents a therapeutic challenge. OBJECTION: This study aimed to evaluate the efficacy and safety of photodynamic therapy (PDT) for treating the GNARCMs of pediatric patients. METHODS: The clinical manifestations of the pediatric GNARCMs patients were retrospectively analyzed, and the treatment data were assessed after undergoing PDT mediated with a combination of Hemoporfin and 532-nm light. For evaluation of improvement, photographs taken before and after photodynamic therapy were evaluated by three independent assessors blindly. Patient satisfaction was also used as a factor in the efficacy evaluation. Adverse events were recorded after treatment. RESULTS: We identified 25 pediatric patients in the study, including 11 patients of SWS, 8 patients of PPV, 5 patients of CMO, and 1 patient of DCMO. After an average of 1.64 sessions of PDT, based on the overall visual assessment, 72% of patients responded to treatment (with >25% color blanching), 48% of patients showed excellent or good levels of improvement (with >50% color blanching). Most adverse events were transient and self-limiting, except one patient of SWS, on the third day after PDT, the visual acuity of the eye on the non-treated side suddenly decreased, and the patient was diagnosed with retinal detachment, which was considered to be related to primary glaucoma rather than PDT. CONCLUSIONS: Focusing on the various clinical manifestations of the GNARCMs, except for patients with contraindications, PDT can be considered for treatment of pediatric patients, and its effectiveness and safety are worthy of affirmation.