Grecchi Francesco, Mancini Giovanni, Parafioriti Antonina, Mineo Giuseppe, Zollino Ilaria, Pricolo Alfonso, Carinci Francesco
Maxillofacial Surgery, Orthopedic Institute Galeazzi, Milan, Italy.
Singapore Dent J. 2010 Jun;31(1):9-14. doi: 10.1016/S0377-5291(12)70003-2. Epub 2012 Jan 25.
Ectodermal dysplasia (ED) comprises a large heterogeneous group of inherited disorders that are characterized by primary defects in the skin, hair, nails, eccrine glands and teeth. The most characteristic findings are the reduced number of teeth. All rehabilitative programmes involve proper evaluation of skeletal relationships. Prosthetic-implantological treatment at the end of bony growth can be used. In this article a case of ED treated with Le Fort I for maxillary advancement, femur homografts, implants' insertion and immediate loading is described. In December 2007, a 38-year-old female was referred to the Maxillofacial Department of Galeazzi Hospital (Milan, Italy) who had a diagnosis of ED. Twelve implants were inserted in one-step surgical procedure. No implant was lost and all are stable. The occlusion is stable after 15 months of follow-up. The results indicate that the one-step oral rehabilitation can be performed in adults who are affected by ED. Also, this significantly reduces the time of oral and facial rehabilitation.
外胚层发育不全（ED）包括一大类遗传性疾病，其特征为皮肤、毛发、指甲、外分泌腺和牙齿存在原发性缺陷。最典型的表现是牙齿数量减少。所有康复方案都涉及对骨骼关系进行恰当评估。在骨骼生长结束时可采用修复 - 种植治疗。本文描述了一例采用勒福Ⅰ型截骨术进行上颌前徙、股骨同种异体移植、植入种植体并即刻加载治疗的外胚层发育不全病例。2007年12月，一名38岁女性被转诊至加莱亚齐医院（意大利米兰）颌面科，诊断为外胚层发育不全。在一次外科手术中植入了12颗种植体。没有种植体丢失，所有种植体都很稳定。随访15个月后咬合稳定。结果表明，受外胚层发育不全影响的成年人可进行一次性口腔修复。此外，这显著缩短了口腔颌面康复时间。
