McCune-Albright syndrome progressing with severe fibrous dysplasia.
We present the case of an 11-year-old girl with McCune-Albright syndrome associated with severe fibrous dysplasia. In addition to bone lesions, she has apparent manifestations of precocious puberty. In examination, a mass at the mentum spreading to mandibular corpus bilaterally was seen. This mass has affected the mandibular teeth. It was nearly 20 x 20 x 15 cm in size and had local necrotic regions on it. The lower lip was expanded too much by the mass. Another mass filled the left maxillary sinus, expanding the left zygomatic region outwardly and closing the left nasal fossa completely. A rectangular skull shape was related to the involvement of cranial bones. After stainless steel, custom-made mandibular prosthesis was prepared, the patient underwent surgery. A partial mandibulectomy was performed, and resulting mandibular bone defect was reconstructed by steel prosthesis. Craniofacial involvement occurs in 100% of disseminated cases. In the dental literature, mandibular involvement was found in 20% of cases. However, in studied literature, we did not find a dramatic mandibular lesion as severe as that presented here.
Gürler
T
T
Department of Plastic and Reconstructive Surgery, Ege University School of Medicine, Izmir, Turkey.
Alper
M
M
Gençosmanoğlu
R
R
Totan
S
S
Güner
U
U
Akin
Y
Y
D000208:Acute Disease; D002648:Child; D018450:Disease Progression; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008334:Mandible / Q000601:surgery; D019930:Mandibular Prosthesis Implantation; D011629:Puberty, Precocious / Q000473:pathology; D012886:Skull / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed