Surgical management of a facial deformity secondary to craniofacial fibrous dysplasia

Sachs, Stephen A.; Kleiman, Michael; Pasternak, Richard C.

doi:10.1016/s0278-2391(84)80033-6

Cited by 16 publications

(6 citation statements)

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“…The surgical treatment of FD usually consists of conservative shaving/contouring. [33] Ideally, surgical intervention is recommended to be delayed until after skeletal maturity has been reached. [34] However, if the progression of the disease compromises neurological function or vision or threatens the airway,[35] a decompressive procedure should be considered early in childhood to preserve normal function.…”

Section: Discussionmentioning

confidence: 99%

Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex

Jeyaraj¹

2019

Ann Maxillofac Surg

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Background:Owing to the overlapping clinical, radiographic and histopathological features among the diverse group of Fibro-osseous lesions, a precise and definitive diagnosis of Fibrous Dysplasia (FD) can be quite challenging. Moreover, FD itself may manifest with widely varying clinical presentations, radiographic appearances and histological pictures, depending upon the maturity of the lesion, and the relative quantum of its ‘fibrous’ and ‘osseous components’. Prompt and accurate diagnosis of Fibrous Dysplasia (FD) of the Craniomaxillofacial region is particularly important, as the condition is capable of causing considerable facial asymmetry or deformity leading even to marked disfigurement, which can have a profound psychosocial impact on the patient. Involvement of Maxillofacial bones by aggressive forms of FD, can produce serious functional debility as well, by compromising airway, breathing, vision, hearing, occlusion, mastication and mouth opening. Calvarial bone involvement can produce cranial asymmetry, and cranial base involvement can lead to persistent headaches, facial pain, numbness, and other neurological deficits owing to compression of cranial nerves.Aims and Objectives:To evaluate the importance of early and precise diagnosis, with prompt surgical management of these lesions, for a successful overall esthetic and functional outcome.Materials and Methods:A Case series of 15 patients, showcasing the principal variants of FD affecting the Craniomaxillofacial complex, namely, the Monostotic and Craniofacial forms have been described. Diversity in their Clinical, Radiographic and Histopathological presentations; their management modalities elucidating the various surgical approaches employed to access and excise these bone pathologies, have been provided along with a review of existing literature.Results:Various surgical approaches may be employed to access the lesions, depending upon their location, extent and involvement. Treatment protocols range from complete surgical excision to surgical shaving and recontouring, and must be decided upon on a case to case basis, with the aim to achieve the best possible esthetic and functional outcome with the least postoperative morbidity.Conclusion:Correlation of HPE with history, clinical features, biological behaviour, radiographic and CT appearance, laboratory findings, and intra-operative findings is imperative, so that they can be distinguished from other bony lesions and an appropriate, ideal and effective treatment modality can be instituted in time, so as to achieve the most favourable esthetic and functional outcome.

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Section: Discussionmentioning

confidence: 99%

Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex

Jeyaraj¹

2019

Ann Maxillofac Surg

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“…Orthognathic surgery is suitable for reducing the asymmetry in these cases, 3 and several cases treated by orthognathic surgery have been reported. [3][4][5][6] However, Le Fort 1 osteotomy was performed in only four of these cases. 3,4,6 Yeow and Chan 3 reported that it was diffi cult to ensure the safety of a Le Fort 1 osteotomy in fi brous dysplasia because of the severe anatomical abnormality.…”

Section: Discussionmentioning

confidence: 99%

“…2 Although complete resection is diffi cult in polyostotic cases, severe facial asymmetry or malocclusion sometimes remains after conservative surgery only. 3 Several reports have mentioned performing orthognathic surgery for fi brous dysplasia [3][4][5][6] to correct these symptoms, but when Le Fort 1 osteotomy is performed to treat this disease, it is diffi cult to ensure safe surgery because of the severe anatomical abnormality. 3 Computer-assisted surgery using images acquired by computed tomography (CT) or magnetic resonance imaging has recently been developed for cerebral, skull base, and sinus surgery.…”

Section: Introductionmentioning

confidence: 99%

Navigation surgery for Le Fort 1 osteotomy in a fibrous dysplasia patient

et al. 2010

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Orthognathic surgery is sometimes performed for fibrous dysplasia to correct malocclusion or facial asymmetry. However, Le Fort 1 osteotomy for this disease is difficult because of severe anatomical abnormality. Computer-assisted surgery is a rapidly developing technique in oral and maxillofacial surgery that is helping to ensure the safety of the surgery. We report a case of polyostotic craniofacial fibrous dysplasia in which two-jaw orthognathic surgery was performed using a navigation system with the Le Fort 1 osteotomy procedure. A 29-year-old woman presented with swelling and asymmetry on the right side of her face. Craniofacial fibrous dysplasia on the right side had been previously diagnosed, and she had undergone conservative surgery several times before. The disease extended to the right mandible, maxilla, and zygomatic, temporal frontal, and orbital areas, including the skull base. We first performed conservative contouring around the frontal and orbital areas, and then Le Fort I osteotomy and sagittal split ramus osteotomy to correct the asymmetry and cant of the occlusal plane. A passive infrared navigation system (Vector Vision surgical navigation system) was used for the Le Fort I osteotomy. The postoperative course was stable, and the facial asymmetry and cant of the occlusal plane improved and remained suitable 2 years after surgery. Thus, Le Fort 1 osteotomy can be performed safely in fibrous dysplasia with the aid of a passive infrared navigation system.

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“…However, the FD lesion sometimes affects the alveolar bones of the maxilla or mandible, causing discrepancy in the occlusion secondary to the alveolar bone expansion. In such cases, complete osteotomy and repositioning of the maxilla or mandibular bones must be performed concomitantly with debulking of the lesion [5–10].…”

Section: Introductionmentioning

confidence: 99%

Orthognathic surgery for patients with fibrous dysplasia involved with dentition

Udayakumar

Paeng

Choi

et al. 2018

Maxillofac Plast Reconstr Surg

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BackgroundFibrous dysplasia (FD) is characterized by the replacement of normal bone by abnormal fibro-osseous connective tissue and typically treated with surgical contouring of the dysplastic bone. When dysplastic lesions involve occlusion, not only is surgical debulking needed, orthognathic surgery for correction of dentofacial deformity is mandatory. However, the long-term stability of osteotomized, dysplastic bone segments is a major concern because of insufficient screw-to-bone engagement during surgery and the risk of FD lesion re-growth.Case presentationThis case report reviewed two patients with non-syndromic FD that presented with maxillary occlusal canting and facial asymmetry. Le Fort I osteotomy with recontouring of the dysplastic zygomaticomaxillary region had been performed. The stability of osseous segments were favorable. However, dysplastic, newly formed bone covered the previous plate fixation site and mild bony expansion was observed, which did not influence the facial profile. Including the current cases, 15 cases of orthognathic surgery for FD with dentition have been reported in the literature.ConclusionThe results showed that osteotomy did not appear to significantly reduce the long-term stability of the initial fixation insufficiency of the screw to the dysplastic bone. However, based on our results and those of the others, long-term follow-up and monitoring are needed, even in cases where the osteotomized segment shows stable results.

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Surgical management of a facial deformity secondary to craniofacial fibrous dysplasia

Cited by 16 publications

References 1 publication

Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex

Histological diversity, diagnostic challenges, and surgical treatment strategies of fibrous dysplasia of upper and mid-thirds of the craniomaxillofacial complex

Navigation surgery for Le Fort 1 osteotomy in a fibrous dysplasia patient

Orthognathic surgery for patients with fibrous dysplasia involved with dentition

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