Sat 31stAug 2013 Session 3 / CR1-2 13:06 – 13:10 HEAPHY 1 & 2 CASE RACE 1 – DIAG Kristina GREEN 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.
McCune-Albright Syndrome Kristina Green NZIMRT Conference 2013
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
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.
Case Study • 27 year old female with severe polyostotic fibrous dysplasia • Severe facial deformity • Almost entire skeleton affected • Limited imaging performed
Treatment and Future Prognosis • Pain management • Amnio-biphosphonates • Extensive reconstruction surgery • Follow-up imaging
References Bruce, H (1937). “Progress in Pediatrics: OsteodystrophiaFibrosa”. Archives of Pediatrics & Adolescent Medicine54 (4): 806. Albright F, Butler AM, Hampton AO, Smith P (1937). “Syndrome characterized by osteitisfibrosadisseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases”. New Eng. J. Med. 216 (17): 727-746.