David K Langford, Jonathan N Sorsok, Tung T Wynn, Marcio Guelmann, Sandra Natalia Gonzalez Rodriguez
A guideline-driven, stepwise protocol combining conservative caries arrest, glass ionomer sealants and restorations, and targeted general anesthesia care preserved oral function while minimizing mucosal trauma. Multidisciplinary coordination supported the care episode, with anesthesiology managing the difficult airway, dermatology advising on the treatment setting and mucosal protection, and hematology optimizing the patient's baseline iron-deficiency anemia.
AIMS: To describe the stepwise, atraumatic dental management of a pediatric patient with severe recessive dystrophic epidermolysis bullosa (RDEB), emphasizing conservative caries arrest, the challenges of restorative care within a severely microstomia-limited oral opening, and coordinated multidisciplinary care.
METHODS AND RESULTS: A male patient with severe RDEB was followed at a university pediatric dental clinic from Age 13 to 14 years within a dental home maintained since infancy. Serial silver diamine fluoride applications arrested multiple occlusal carious lesions. Glass ionomer sealants were placed on seven teeth without retraction or mirror use. At Age 14, comprehensive restorative care under general anesthesia included stainless-steel crown placement on a mandibular first molar and glass ionomer restorations on two additional teeth, all accomplished through a maximum interincisal opening of approximately 30 mm. Fiber-optic nasal intubation required multiple attempts. Six-month follow-up confirmed caries-free status and intact restorations.
CONCLUSION: A guideline-driven, stepwise protocol combining conservative caries arrest, glass ionomer sealants and restorations, and targeted general anesthesia care preserved oral function while minimizing mucosal trauma. Multidisciplinary coordination supported the care episode, with anesthesiology managing the difficult airway, dermatology advising on the treatment setting and mucosal protection, and hematology optimizing the patient's baseline iron-deficiency anemia.