1. HEAPHY 1 & 2 CASE RACE 1 – DIAG Kristina GREEN Sat 31 st Aug 2013 Session 3 / CR1-2 13:06 – 13:10 WAIKATO / BAY OF PLENTY ABSTRACT Fibrous Dysplasia is an abnormal bone growth where normal bone is replaced with fibrous tissue. It can affect any bone in the body and has two types: monostotic (affecting one bone) or polystotic (involving many bones). The most severe form of polystotic fibrous dysplasia is known as McCune-Albright Syndrome. This condition has a broad spectrum of severity and includes associated abnormalities of bone disease, endocrine disease, and skin abnormalities. The case for discussion follows a 27 year old female that has McCune-Albright Syndrome with severe polystotic fibrous dysplasia.

2. Kristina Green NZIMRT Conference 2013

3. Genetic disorder of bones, skin pigmentation, and hormonal problems Severe form of polyostotic fibrous dysplasia Unilateral café-au-lait spots Endocrine hyperfunction which can lead to premature puberty

4. Fibrous Dysplasia Abnormal bone growth where normal bone is replaced with fibrous bone tissue This causes abnormal growth and swelling of bone It can occur in any part of the skeleton, but bones of the skull, femur, tibia, ribs, and pelvis are most commonly affected.

5. Case Study 27 year old female with severe polyostotic fibrous dysplasia Severe facial deformity Almost entire skeleton affected Limited imaging performed

9. Treatment and Future Prognosis Pain management Amnio-biphosphonates Extensive reconstruction surgery Follow-up imaging

10. References Bruce, H (1937). “Progress in Pediatrics: Osteodystrophia Fibrosa”. Archives of Pediatrics & Adolescent Medicine 54 (4): 806. Albright F, Butler AM, Hampton AO, Smith P (1937). “Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases”. New Eng. J. Med. 216 (17): 727-746.