重症化した頭蓋顔面多形性線維性異形成症の外科的管理における段階的凍結手術と三次元CT一体型ナビゲーションシステムの役割

The Role of Staged Cryosurgery and Three-Dimensional Computed Tomography Integrated Navigation System in the Surgical Management of Severe Involvement of Craniofacial Polyostotic Fibrous Dysplasia.

• Kemalettin Yildiz
• Tolga Turan Dündar
• Anas Abdallah
• Turan Mehdizade
• Ethem Güneren
• Halil Ibrahim Canter

抄録

線維性異形成症（FD）は，骨形成細胞のハマルタマト性増殖による発達性骨疾患である．FDと診断された29歳男性患者は、昨年よりFDの重度の頭蓋顎顔面病変を呈して当院に入院した。神経学的検査では複視、水平眼振、導電性難聴、部分的な視力低下を認めた。病歴では、約8年前に当院の整形外科医院で病的骨折により左大腿骨の髄内釘打ち手術を受けたことが報告されていた。この患者は、当院の整形外科・脳神経外科チームによる3回連続手術を受けた。手術の間は神経集中治療室で経過観察を行い、最後の手術から1週間後には神経集中治療室に移された。その後、脳神経外科に転科した。術後合併症は認められなかった。術前の徴候は回復していた。6ヶ月目の経過観察では、術前の症状はすべて改善されていた。また、術後の美観の改善にも満足していた。結論として、バーチャル手術計画と術中手術ナビゲーションシステムは、困難な症例でも手術を可能にし、多胞体FDの手術の安全性を高めることができると考えられる。

Fibrous dysplasia (FD) is a developmental bone disorder caused by the hamartamatous proliferation of bone-forming cells. A 29-year-old male patient with diagnosis of FD was admitted to our clinic with the symptoms of severe craniomaxillofacial involvement of FD beginning from last year. Neurological examination revealed diplopia, horizontal nystagmus, conductive hearing loss, and partial vision loss. In his medical history, it was reported that he had undergone intramedullary nailing operation in his left femur due to a pathological fracture approximately 8 years ago in the orthopedics clinic of our institution. The patient underwent 3 consecutive surgeries by our plastic and neurosurgical team. The patient was followed-up in the neuro-intensive care unit between the surgical sessions and 1 week after the last operation. Afterwards, he was transferred to the neurosurgical department. No postoperative complication was detected. The preoperative signs were recovered. During his 6th month follow-up control-visit, all his preoperative symptoms were improved. In addition, the patient was satisfied with his postoperative cosmetic improvement. In conclusion, virtual surgical planning and intraoperative surgical navigation systems can make the challenging cases possible to operate with increasing the safety margin of the surgical procedures for polyostotic FD.