Management of Severe Riga-Fede Disease in a Child With MIRAGE Syndrome

Riga-Fede disease (RFD) is a rare condition in infants and young children caused by repetitive trauma to the oral mucosa, often from newly erupted teeth. We present the case of a 13-month-old male child with a complex genetic disorder who developed persistent ulceration on the ventral tongue due to contact with mandibular incisors. Initial management included conservative smoothing of the teeth, which led to only partial improvement. Over time, the mucosal ulceration worsened, resulting in mucosal separation and soft tissue loss. A multidisciplinary surgical approach was pursued. Pediatric otolaryngology performed excision of the compromised mucosa, re-approximation of the muscular defect with absorbable sutures, and a lingual frenuloplasty to address ankyloglossia. Pediatric dentistry completed the extraction of the four mandibular incisors to eliminate the source of trauma. This case illustrates the importance of early recognition and definitive treatment of RFD to prevent progression to more complex soft tissue defects. In medically complex patients, delays in intervention may lead to increased morbidity and impaired healing. Successful management requires close collaboration between specialties and careful surgical planning to preserve function and reduce recurrence risk.