Cloverleaf Skull Deformity--A Case with Long-Term Treatment and Follow-Up

苜蓿草型態（Cloverleaf skull）之顱骨縫提早閉合長期追蹤及治療－個案報告

廖冠豪(Kuan-Hao Liao) ； 羅綸洲(Lun-Jou Lo)

《臺灣整形外科醫學會雜誌》 30卷4期 (2021/12) Pp. 257-265

摘要

Background: Cloverleaf skull deformity is a rare disease causing significant craniofacial disfigurement and functional problems. Only a few cases have been documented in the past decades. There are currently no standard treatment protocols, and treatment is based on the individual needs of the patient. Herein, we report a male patient with cloverleaf skull deformity who was managed with a stepwise approach and long-term follow-up. Patient: The boy received a ventriculoperitoneal shunt for hydrocephalus in November 2008. Combined surgery with cranial and supraorbital remodeling was then performed in June 2009. At 11 years of age, he presented with prominent midface retrusion and exophthalmos. A LeFort III osteotomy and gradual external distraction were performed to improve eye closure, malocclusion and obstructive sleep apnea. Results: The sequential treatments effectively corrected the problems of elevated intracranial pressure, exophthalmos, airway and midface retrusion with class III malocclusion. Conclusion: For the treatment of cloverleaf skull deformity, early intra-cranial decompression is critical for the prognosis. Stepwise surgical management can result in acceptable functional and cosmetic outcomes and also reduce the risk of post-operative complications.

關鍵字

cloverleaf skull deformity ； syndromic craniosynostosis ； cranioplasty ； hydrocephalus ； LeFort III distraction

並列摘要

背景：Cloverleaf skull deformity（苜蓿草型態之顱形異常）是一種非常罕見的顱縫早閉症，病人往往可以合併其他種症候群或先天性異常，有時因為嚴重地畸形，死胎或是早夭亦可能發生。診斷上除了頭部外形猶如三葉的苜蓿葉外（cloverleaf），比較常見的臨床表徵有中臉發育不全，低耳位，眼距過寬，凸眼症；又因為顱型結構的異常，水腦症或是腦壓過高也佔有一定比例。目的與目標：回顧過去文獻，此病例相當罕見；並沒有標準治療方針，長期追蹤案例也相當稀少，在此篇文章中，我們報告一例追蹤11年的Syndromic craniosynostosis合併cloverleaf skull deformity的病例。材料材料及方法：病患在7個月大的時候至本院求診，經由神經外科評估後進行V-P shunt置放手術以改善水腦症，於1歲3個月的時候，再由整形外科與神經外科同時進行顱骨整形手術，術後病人狀況與發育穩定，且在學齡到達時候進入學校就學。病童於11歲的時候，因為中臉的發育不全合併眼球無法閉合，於是我們為病童施行Lefort III osteotomy and HALO distraction術，在兩個月的時間內逐步地牽引，並再經過一個月的穩固期後，移除牽引器。結論：Cloverleaf skull deformity是一種罕見的顱縫早閉症，如果病人有機會接受手術，因及早照會神經外科進行顱內減壓，待病患年紀較長與情況較為穩定的時候，再逐步地安排各個階段的顱骨重建手術。