Imaging characteristics of a rare case of monostotic fibrous dysplasia of the sacrum:A case report

BACKGROUND Fibrous dysplasia(FD)is a common benign intramedullary fibro-osseous lesion.Involvement of the spine is rare,with the literature including only case reports,and cases of monostotic FD(MFD)in the sacrum are extremely rare.A correct preoperative diagnosis of spinal MFD is important for clinicians to select proper treatment.CASE SUMMARY We retrospectively assessed a case report of MFD in the sacrum.This patient was examined by computed tomography(CT)and magnetic resonance imaging(MRI),and the diagnosis was confirmed by pathology.A review of the literature was performed to analyze the imaging characteristics and differential diagnoses of spinal MFD.For our patient,the CT scan showed the lesion to be expansile,with ground glass opacity and a sclerotic rim.On MRI,the lesion showed iso-low signal intensity on T1WI and iso-high signal intensity on T2WI.A low signal rim was found on T1WI and T2WI.Our patient was treated by posterior focal excision,decompression,bone grafting,fusion and pedicle screw fixation.A satisfactory result was achieved,with pain disappearance.No complications had occurred at the 1-year follow up.CONCLUSION MFD is an expansile osteolytic change.Ground glass opacity and a sclerotic margin are obvious characteristics.The lesion often involves the vertebral body and posterior element.Knowledge of these imaging characteristics of spinal FD could be helpful for diagnosis and prevent unnecessary procedures.

Giant monostotic osteofibrous dysplasia of the ilium: A case report and review of literature

BACKGROUNDOsteofbrous dysplasia （OFD） is a developmental ske-letal disorder, and cases with a giant affected area in the pelvis are rare.CASE SUMMARYIn this case report, a 48-year-old man presented with a large tumor in the right iliac region that turned out to be OFD. The patient had rebound tenderness in his right hip. After radiography examination, magnetic resonance imaging examinations and some physical examination, extensive bone destruction in the right ilium was confirmed. Moreover, changes in bone mi-neral density and peripheral cortical bone sclerosis with surrounding soft tissue swelling were observed. Thus, this patient was considered to have giant monostoticOFD of the ilium. The tumor-related area was removed completely by surgery, and the remaining cavity was filled by artificial bones from the opposite ilium. According to the results of follow-up, the patient had normal fexion and extension activities of the right hip joint, and there was no evidence of recurrence of the tumor.CONCLUSION Suture of iliopsoas and gluteus medius muscle following focus curettage and bone grafting is a promising and effective method to treat giant OFD of the ilium. It is a feasible way to fll a large cavity after removing a lesion like the one is this case.

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

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.

Fibrous dysplasia involving the calvarium in children

To gain a broader appreciation of the clinical presentation,operative treatment,and outcome offibrous dysplasia involving the calvarium in children,we retro-spectively reviewed a series of cases offibrous dysplasia involving the calvarium(4 males and 2 females)with patients’age ranging from 5 to 12 years old.The clinical manifestation,radiographicfindings,surgical treatment,outcome and follow-up were evaluated on the basis of medical records.Fibrous dysplasia in the series was monostotic,involving frontal bone(2 cases),temporal bone(1 case),parietal bone(2 cases)and occipital bone(1 case).The patients most commonly presented with enlarging mass and cosmetic complaints.The treatment given,depending on clinical presentation,was simple biopsy with conservative follow-up(2 cases)to cranial resection(4 cases).All the cases were histopathologically confirmed asfibrous dysplasia.It was demonstrated thatfibrous dysplasia involving the calvarium is a typically benign but slowly progressive disorder of bone.Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward.Surgery should be reserved for patients with functional impairment or cosmetic deformity.Because of the benign nature of the condition,the surgery itself should be contemplated with great caution in children.