(48) Ectodermal Dysplasia – Diverse Treatment Alternatives for Reconstruction

Abstract: Maxillofacial reconstruction and subsequent prosthodontic rehabilitation of the maxilla and mandible in patients with ectodermal dysplasia (ED) can present a physiologic challenge that stems from a loss of function, lack of alveolar ridge development, arch length, tooth size discrepancies, and hyposalivation. Although efforts have been made to create a consensus of timing to dental therapy and oral rehabilitation of this demographic, there are still gaps in the literature regarding maxillofacial reconstruction and subsequent prosthetic dental rehabilitation. We will elucidate treatment alternatives for reconstruction of ectodermal dysplasia patients following a treatment algorithm developed at our institution.

A retrospective chart review was completed to identify patients that have undergone treatment performed by a single surgeon and prosthodontist from 2011- 2023. Variables identified include the type of treatment, immediate vs delayed implant load, implant and prosthesis success rate, and use of bone graft.

Our analysis was limited to descriptive statistics. Mean age was 24 years old (range, 19 to 37). We reviewed 10 cases of patients with diagnosis of ED who underwent combination of either maxillomandibular reconstruction with microvascular free fibular flap, Lefort 1 osteotomy with bone graft, anterior iliac crest bone graft (AICBG) with zygomatic implants and/or traditional dental implants, or alloplastic bone graft with traditional dental implants. Following our institutional treatment algorithm, we developed reconstructive and oral rehabilitation alternatives that permitted 9 patients to be transitioned to a servicing prosthesis for at least one year. Longest supported prosthesis to date is 9 years. One patient remains within the first year of service of their prosthesis.

Maxillae were stratified as severe, moderate, and minimally hypoplastic to guide reconstructive and rehabilitative alternatives. One patient with severely hypoplastic maxilla received a microvascular free-fibula-flap with simultaneous jaw-in-a-day endosteal implants. One patient with moderate maxillary hypoplasia and concomitant mandibular prognathism underwent Lefort-1 osteotomy with subsequent mandibular alloplastic bone graft. Two patients with moderate hypoplasia of maxillae received a total of 4 zygomatic implants with simultaneous AICBG to the anterior maxilla. Six patients with minimal maxillary hypoplasia received a combination of standard dental implants with traditional alloplastic bone graft and membrane. One of these patients had a sinus lift. Eight patients with mandibular oligodontia or anodontia were rehabilitated with a combination of traditional alloplastic bone graft & membrane or AICBG to anterior mandible with eventual endosteal implant placement. One patient had gross micrognathia with co-morbid craniofacial dysostosis and non-erupted ankylosed mandibular teeth, underwent marginal mandibulectomy with simultaneous placement of all-on-4 endosteal implants.

All patients received bone grafting of at least one site. 2 patients received AICBG to anterior maxilla and 1 patient received AICBG to anterior mandible. Remaining patients received allograft to indicated sites. A total of 77 dental implants were placed with 96% success rate. A total of 4 zygomatic implants were placed with 100 % success rate. Success rate was defined as osseointegration of implant with servicing prosthesis for at least one year. 85% of implants were loaded in delayed fashion. 15 % were loaded immediately. All had successful functioning prosthesis. Full arch screw-retained prosthesis, partial arch screw-retained prosthesis, or overdenture prothesis replaced on average 10 teeth in the maxilla (range, 8-12) and 11 teeth in the mandible (range, 8-12) with no prosthetic fractures.

Our retrospective study further characterizes the use of several treatment alternatives to reconstruct patients with history of ectodermal dysplasia. We propose a treatment algorithm dictated by anodontia or oligodontia with comorbid degree of hypoplasia of maxilla and/or mandible that guides decision-making when providing reconstruction and dental prosthetic rehabilitation of patients with ED diagnosis.

WangY et al.Clinical-outcomes-of-implant-therapy in ectodermal dysplasia patients: a systematic review.IntJOralMaxillofacSurg.2016-Aug;45(8):1035-43.

Klineberg I, etal.Rehabilitation-of-children-with-ectodermal-dysplasia.Part 1:an international Delphi study. IntJOralMaxillofacImplants.2013-Jul-Aug;28(4):1090-100.