腦顱皮膚脂肪增多症患者之牙科處置－病例報告

Dental Management in Patient with Encephalocraniocutaneous Lipomatosis -A Case Report

唐培華(Pei-Hua Tang) ； 林怡君(Yi-Chun Lin) ； 許修銘(Hsiu-Ming Hsu)

《臺灣兒童牙醫學雜誌》 18卷3期 (2018/09) Pp. 118-125

https://doi.org/10.6319/TJPD.201809_18(3).0006

摘要

腦顱皮膚脂肪增多症（Encephalocraniocutaneous lipomatosis）是罕見的先天性神經皮膚症候群 （Neurocutaneous syndrome），臨床特徵為多種器官中發現脂肪瘤增生，合併皮膚、眼、骨及中樞神經系統侵犯等表現，亦可能有顎骨腫瘤的產生，包含齒瘤（Odontoma）、骨瘤（Osteoma）、骨化纖維瘤（ossifying fibroma）及多發性骨囊腫（Multiple bone cyst）等。本篇病例報告為四歲女童，於出生20天發現雙側上肢無力，經核磁共振檢查發現枕骨大孔到第九節胸椎的硬腦膜及脊髓內脂肪增多（C0-T9 intradural and intramedullary spinal lipomatosis），並於出生一個月接受減壓開顱及椎間板開孔手術。目前右上肢輕微麻痺，接受復健治療。右顳部、右眼可見脂肪瘤。初次牙科門診就診主訴為右臉紅腫尋求治療。經臨床及放射線檢查懷疑右上乳犬齒引起急性根尖膿瘍，安排牙髓治療及後續齲齒治療。腦顱皮膚脂肪增多症主要影響中樞神經系統，可能造成癲癇、智能、肢體等障礙。雖然在牙科臨床表現較不明顯，但有顎骨腫瘤產生的可能性，宜持續追蹤。

關鍵字

腦顱皮膚脂肪增多症 ； 神經皮膚症候群 ； 顎骨腫瘤

並列摘要

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome with unilateral scalp lesions, cranial lipomas, ocular lipodermoids and brain abnormalities. ECCL is also considered to be relative with jaw bone tumors. We presented a 4-year-old girl with ECCL. She came to our clinic for right facial swelling few days ago. Her past medical history was noted weakness of bilateral upper limbs when she was 20 days-old. MRI showed intradural and intramedullary spinal lipomatosis from C0 to T9. She underwent surgery at age one month. She presented xanthomatous lesions involve right temporal area, scalp, and right eyeball. In our clinic, clinical and radiographic examination showed acute periapical abscess from upper right primary canine. We arranged root canal treatment and further dental management. Although lipomas are non-progressive benign tumors, they may cause epilepsy, delayed development and motor impairment. They may also show jaw bone tumors. Long-term monitoring for clinical manifestations is advised.

並列關鍵字

encephalocraniocutaneous lipomatosis ； neurocutaneous syndrome ； jaw bone tumor