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| Case of the Month |
by Dr Noreen Norfaraheen Lee Abdullah MD (UKM), MMed Radiology (UKM), AM(Mal)Answer to Case Of the Month Discussion of Findings |
| Fibrous Dysplasia (FD) |
| This condition is also known as the Lichtenstein Jaffe disease. It is a benign mesodermal disorder and affects the fibroosseous development which manifest as a defect in osteoblastic differentiation and maturation. It affects male and female alike with 75% presenting before the age of 30 years old. The peak age of presentation is between 3 and 15 years. There are three types of FD - Monostotic, polyostotic and craniofacial forms. Monostotic form is also known as the localized type and is more common (70–80%). The patients are usually asymptomatic until the 2nd or 3rd decade. One bone is normally involved and this form usually affects the ribs and proximal femur. The polyostotic form presents earlier in life, usually in the first decade. The usual presentation will be leg pain, limp, pathological fracture or limb length discrepancy. The femur is usually affected (90%), tibia (81%), pelvis (78%) and foot (73%). The ribs, spine and clavicle are also affected but the incidence is less. Sometimes other disorders are present especially endocrinopathies such as hyperthyroidism, acromegaly, hyperparathyroidism, Cushing syndrome or rickets. McCune Albright syndrome is one such example whereby the patient has polyostotic fibrous dysplasia, skin pigmentation, accelerated skeletal maturation and precocious puberty. The craniofacial form is rare and is known as leontiasis ossea. The cranial bones usually involved are sphenoid, frontal, maxillary, ethmoid, occipital and temporal bones in decreasing order of frequency. Transformation into osteo/chondro/fibro sarcoma or malignant fibrous histiocytoma has been reported. The manifestation is increasing pain and the radiographic features are that of a pathological fracture with enlarging soft tissue mass and lytic lesions. A skeletal survey is required when first discovered. The patient should be on long term follow up and radiographs at yearly intervals would be appropriate for surveillance and early detection of complications. Reference: Wolfgang Dahnert – Radiology Review Manual |
| About the Author |
| Dr Noreen, a Consultant Radiologist is currently with the Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia. |
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