半侧颜面短小畸形(Hemifacial Microsomia),系胚胎期第一、第二腮弓和位于期间的咽囊及第一腮裂、颞骨原基发育不全所致,既往曾有第一、第二腮弓综合征(first and second brachial arch syndrome),颅面短小症(craniofacial microsomia)...
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半侧颜面短小畸形(Hemifacial Microsomia),系胚胎期第一、第二腮弓和位于期间的咽囊及第一腮裂、颞骨原基发育不全所致,既往曾有第一、第二腮弓综合征(first and second brachial arch syndrome),颅面短小症(craniofacial microsomia),耳下颌发育不全(otomandibulardysostosis),半侧下颌小耳综合征(hemignathia andmicrotia syndrome),耳颌面畸形(otomandibular-facialdysmorphogenesis)等命名。其临床表现为:1.单侧下颌骨发育不良——在半侧颜面短小畸形中最常见也最重要,下颌骨发育不良是颅面其他骨发育不良的基石。2.其他颅骨发育不良:颧骨发育不良,颧弓狭窄,乳突发育不良,咬合关系异常。3.咀嚼肌发育异常:咬肌,颞肌,翼内肌,翼外肌功能受损,下颌前伸时向患侧偏。4.耳畸形:轻者仅有副耳,耳前瘘管,重者耳廓大部分缺失,外耳道闭锁,中耳畸形。5.软组织受累:颊部皮肤、皮下组织发育不良,腮腺发育不良,单侧面横裂及大口畸形。6.面神经发育不良。半侧颜面短小畸形的序列治疗包括:1.轻度耳畸形,口角裂:出生后3个月后可进行整形手术,早期手术使面颊畸形在生长发育期得到矫正,而且使流涎改善,吸允、言语等功能得到恢复,预防牙颌畸形。2.耳廓缺损、外耳道及中耳畸形:学龄前后手术,包括皮肤软组织扩张器法耳廓再造:分三期手术,一期耳后乳突区扩张器植入术,二期手术组织扩张器取出,扩张皮瓣和自体肋软骨支架外耳再造,三期手术耳屏再造,耳甲腔加深和再造耳廓局部修整;外耳道重建术;骨嵌式助听器(Bone-anchored hearing aid,BAHA)改善听力等。3.面部骨骼重建:包括自体骨组织(如颅骨外板、下颌骨外板、肋骨等游离骨组织移植,吻合血管的骨、筋膜、脂肪复合组织移植等)充填植骨;骨组织代用品(如天然珊瑚,羟基磷灰石(hydroxyapatite,HA),珊瑚羟基磷灰石(Interpore),高密度多孔聚乙烯(Medpor),彭体聚四氟乙烯(ePTFE)等充填和生物人工材料贴附;上颌骨Le FortⅠ型或Ⅲ型截骨,下颌骨矢状劈开截骨旋转,颏部截骨移位等正颌手术;健侧下颌角弧形截骨术,颧骨"L"型截骨降低术,患侧颧骨"L"型截骨扩展术等;下颌骨牵张成骨技术(Distraction Osteogenesis)等。4.面部软组织的修复:自体脂肪颗粒注射移植,吻合血管的游离组织移植(如股前外侧筋膜脂肪瓣移植)等。
Background:Giant congenital melanocytic nevi (CMN) are rare,congenital,disfiguring lesions with a risk of degeneration to malignant melanoma.A variety of treatment options exists for their management,however,the surgi...
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Background:Giant congenital melanocytic nevi (CMN) are rare,congenital,disfiguring lesions with a risk of degeneration to malignant melanoma.A variety of treatment options exists for their management,however,the surgical excision is the only technique in which complete removal of nevus cells can be *** course,the major concern with giant CMN is its potential for malignant transformation,the cosmetic impairment can be also *** reviewed our surgical management of this disease and patient outcomes to assess the effectiveness of our management protocol.
Object:Alveolar and mandibular bone defects associated with resection of oral tumors and traumas impair occlusion,destroy the upper and lower dentition,and affect the aesthetics of the lower third of the facial contou...
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Object:Alveolar and mandibular bone defects associated with resection of oral tumors and traumas impair occlusion,destroy the upper and lower dentition,and affect the aesthetics of the lower third of the facial contouring, making it difficult to obtain a satisfactory therapeutic *** with distraction osteogenesis(DO) is particularly difficult in cases of extended partial mandibular defects in which the defective region extends to close to the inferior margin of the *** overcome the limits of current DO for more than 15 mm in vertical height we developed a method for bone -charged DO by combining distraction osteogenesis and iliac corticocancellous bone grafting.
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