Congenital Infiltrating Lipomatosis of the Face: Case Report with Presentation of A Multistep Surgical Approach and 20 Years Follow Up

顏面先天性浸潤型脂肪瘤病人之二十年長期追蹤及治療方針－個案報告

莊凱棣(Kai-Ti Chuang) ； Srinisha P Murali ； 莊垂慶(David Chwei-Chin Chuang) ； 羅綸洲(Lun-Jou Lo)

《臺灣整形外科醫學會雜誌》 30卷2期 (2021/06) Pp. 79-87

摘要

Background: Congenital infiltrating lipomatosis of the face (CIL-F) is a developmental facial malformation characterized by infiltration of nonencapsulated, mature adipose tissue, resulting in facial soft and hard tissue hypertrophy and dental malformations. Due to the lacunae regarding its treatment protocol in literature, the management of this deformity can be challenging. This report describes the management of a patient with CILF, which to our knowledge is the first case to be recorded in Taiwan that not only addresses the soft tissue anomalies, but also provides information about the results obtained with facial reanimation surgery and orthognathic surgery. Aim and Objectives: The aim of this report is to present a case of CILF treated with multiple staged surgeries and the long-term results. Materials and Methods: Our multistage surgical approach comprised of three components: (1) tumor debulking at infancy, (2) facial reanimation, and (3) osseous surgery. The patient underwent a series of surgeries from infancy until skeletal maturity and was followed up for 20 years. Results: The results of this study indicate that satisfactory results for the management of CIL-F can be achieved by staged surgical procedures addressing both the soft tissue and bony discrepancies. Conclusion: Our multistage procedure has been shown to restore good functional and esthetic status, apart from achieving patient satisfaction. Our 20 years follow up has helped to evaluate our long-term result.

關鍵字

Congenital Infiltrating Lipomatosis of the Face ； facial reanimation surgery ； orthognathic surgery ； long-term follow up

並列摘要

背景：顏面先天型浸潤型脂肪瘤屬於脂肪腫瘤中罕見但獨具特色的一種，真正引起的原因目前仍然不明，此種瘤常於嬰幼兒或孩童時期被發現。臨床上顏面先天型浸潤性脂肪瘤具有下列特性：缺乏良好的包膜覆蓋，由成熟的脂肪組織廣泛浸潤正常的肌肉組織，生長快速，經常伴隨有異常骨質增生，和高手術復發率。由於腫瘤的廣泛浸潤與影響重要的顏面組織，往往無法以手術完全切除腫瘤。目的及目標：回顧過去文獻僅發現稀少案例，並無明確治療方針以及長期追蹤，在此我們報告單一個案，包含一系列手術治療方式及經二十年長期追蹤。材料及方法：治療原則分為三階段，第一階段：腫瘤切除及減積手術，第二階段：顏面表情肌重建手術及第三階段：顏面骨減積及齒顎重建手術。結果：各階段性手術均順利完成並無併發症，正顎手術術後放射性影像顯示顏面骨對稱性提升，此外臉部軟組織分佈及臉型也明顯改善，病人會繼續接受一系列齒顎矯正以達到進一步咬合及外觀改善。結論：顏面部先天型浸潤性脂肪瘤為罕見且困難治療疾病，需要長期之階段性多次手術。