Division of Oral and Maxillofacial Surgery, Department of Surgery, Mayo Clinic and Mayo College of Medicine, Rochester, MN, USA.
Division of Oral and Maxillofacial Surgery, Section of Head & Neck Oncologic Surgery and Reconstruction, Department of Surgery, Mayo Clinic and Mayo College of Medicine, Rochester, MN, USA.
Oral Maxillofac Surg. 2023 Dec;27(4):711-719. doi: 10.1007/s10006-022-01107-5. Epub 2022 Jul 30.
Witkop tooth and nail syndrome is a rare, autosomal dominant type of ectodermal dysplasia that can have significant effects on dentition, including hypoplastic and malformed dentition and significantly atrophic maxillas. Endosseous implants have become one possible solution to replace missing teeth, although their use in areas where bone is sparse becomes challenging. Due to the severe atrophy of the maxillary alveolus, extensive preprosthetic surgeries including orthognathic surgery, extensive bone grafting, and sinus floor augmentations have been recommended prior to placement of endosseous dental implants. Although this treatment has shown favorable outcomes, it requires multiple surgical procedures, contributing to a prolonged treatment course and increased morbidity. An alternative treatment of atrophic maxillas in patients with ectodermal dysplasia includes the use of zygomatic implants. This familial case series discusses 3 siblings, all previously diagnosed with Witkop Syndrome, who underwent comprehensive preprosthetic surgery and prosthetic rehabilitation using zygomatic implants with a follow-up period up to 15 years.
威托克牙-爪综合征是一种罕见的常染色体显性遗传的外胚层发育不良，可对牙齿产生显著影响，包括牙发育不全和畸形以及上颌骨明显萎缩。骨内种植体已成为替代缺失牙齿的一种可能方法，尽管在骨量稀疏的区域使用种植体具有挑战性。由于上颌牙槽骨严重萎缩，在放置骨内牙科种植体之前，推荐进行广泛的术前手术，包括正颌手术、广泛的骨移植和鼻窦底增高术。尽管这种治疗方法显示出良好的效果，但需要进行多次手术，导致治疗过程延长和发病率增加。在患有外胚层发育不良的患者中，治疗上颌骨萎缩的另一种方法是使用颧骨种植体。本家族病例系列讨论了 3 名兄弟姐妹，他们均被诊断为威托克综合征，接受了全面的术前手术和使用颧骨种植体的修复治疗，随访时间长达 15 年。
