Surgical Treatment of Monostotic Craino-Facial Fibrous Dysplasia: Changing the Narratives

摘要

Background: Fibrous dysplasia mainly presents in its monostotic form in the cranio-facial region with serious cosmetic disfigurement and functional derangement of the affected and adjacent structures putting both patient and the attending surgeon in great dilemma. Surgical treatment is the only rewarding and generally accepted treatment option, however, controversy over the surgical technique to be adopted still exists. While in the past, surgeons generally adopted conservative shaving or contouring technique, over the recent years, advocates of radical surgery are winning more disciples. Objective: To highlight the locally destructive, functionally degrading nature of a neglected or poorly excised (shaved) lesion in patients and highlight the outcome of total excision and surgical technique adopted to obviate the need for autologous bone grafting and two-staged surgery. Subjects and Method: We present case series of three patients with giant monostotic fibrous dysplasia of the maxilla, surgically treated in our Centre, who were part of a total of eight cases managed over the past fifteen years in our department of Ear, Nose and Throat-Head and Neck Surgery. The pre-operative clinical assessment, relevant investigations and post-operative outcome are presented. Our surgical technique is highlighted. All the patients had unilateral lesion of the maxilla with gross cosmetic and functional defects. Two of the patients had ischaemic (pressure) atrophy of the cheek soft tissue and skin leading to skin metaplastic changes including leukoplakia, hyperpigmentation. Post-operative follow-up showed satisfactory cosmetic outcome and significant reversal of malocclusion and dental anarchy. There was no recorded recurrence throughout the follow-up period ranging from four to eleven years. Nasal airway was re-established bilaterally in all the cases. Conclusion: Total or near total excision surgical technique with periosteal preservation is our treatment of choice in the management of monostotic cranio-facial fibrous dysplasia. Given the fact that the growth of the tumours often does not cease after puberty against general belief, shaving or contouring technique should be relegated to the background. Our technique of no grafting which reduced cost and morbidity to the patient should be encouraged.