The answer is : Fibrous dysplasia, polyostotic type

Fig1: Radiograph right arm with elbow joint showing deformed humerus with ground glass appearance of the bone. There is well-defined lytic lesion in medulla of metaphyseal region of right humerus with no periosteal reaction. Similar lesion is noted in upper part of right radius.

Fig2: Radiograph pelvis with both hips shows ground glass appearance in right proximal femur with reduction in neck shaft angle and thickened short femoral neck. There is evidence of Shepherd crook deformity seen in right proximal femur.

Discussion:

• Fibrous dysplasia is abnormality in ostoblast differentiation and maturation. It presents in two forms: 1. Mono ostotic. 2. Poly ostotic
• Mono ostotic form is more common (70-80%)
• Poly ostotic form is less common. It is more common in young females. Usually the involvement is unilateral. Commonest sites involved are femur, tibia, ribs, hip, skull, humerus and less commonly vertebrae.
• At the involved site bone shows typical ground glass appearance of bone, well-defined lytic lesions in diaphyseal region and deformities. Also medullary expansion with cortical thinning is seen. Deformed bones are more susceptible to fractures.
• Poly ostotic form is associated with precocious puberty and skin pigmentation (café au lait) spots. This triad is known as McCune Albright syndrome.
• Also sometimes it is associated with benign myxomas and termed as Mazaburd syndrome.
• Conversion from mono to poly form is rare and also malignant transformation is rare.
• The only significant laboratory abnormality is an elevated alkaline phosphatase level.

Treatment Options:

• Non-surgical:
1.Braces may occasionally be used to prevent fracture
2.Bisphosphonates are medications that decrease the activity of cells that dissolve bone

• Surgical
1. Stabilization of the bone with metal implants and screws can be useful to fix a fracture or deformity, or prevent bone breakage.
2. Scooping out (curettage) of the fibrous dysplasia is generally performed along with bone grafting.

References :

• Daffner RH, Kirks DR, Gehweiler JA Jr, Heaston DK: Computed topography of fibrous dysplasia. AJR Am J Roentgen 1982 Nov; 139(5): 943-8.

• De Smet A, Travers H, Neff JR: Chondrosarcoma occurring in a patient with polyostotic fibrous dysplasia. Skeletal Radiol 1981; 7: 197Harris WH, Dudley HR, Barry RJ: The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study. J Bone Joint Surg Am 1962 Mar; 44-A: 207-33

• King RM, Payne WS, Olafsson S, Unni KK: Surgical palliation of respiratory insufficiency secondary to massive exuberant polyostotic fibrous dysplasia of the ribs. Ann Thorac Surg 1985 Feb; 39(2): 185-7Leet AI, Magur E, Lee JS, et al: Fibrous dysplasia in the spine: prevalence of lesions and association with scoliosis. J Bone Joint Surg Am 2004 Mar; 86-A(3): 531-7Lichenstein L, Jaffe HL: Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Pathol 1942; 33: 777.

• National Institutes of Health: Osteoporosis and Related Bone Disorders-National Resource Center Web site. Fast Facts on Fibrous Dysplasia page. Available at: http://www.osteo.org/default.asp. Washington, DC: National Institutes of Health; 2001

• Resnick D, Niwayama G: Diagnosis of Bone and Joint Disorders. 2nd ed. Philadelphia, Pa: WB Saunders; 1988: 4057-70.

• Schwartz DT, Alpert M: The malignant transformation of fibrous dysplasia. Am J Med Sci 1964 Jan; 247: 1-20

    News and Events
    Stay updated with campus
    news and events.