HEREDIATARY MULTIPLE EXOSTOSES - CASE SERIES

INTRODUCTION - Hereditary Multiple Exostoses has several synonyms like chondral osteoma, metapyseal achlasia, diaphyseal achlasia, etc. Being a rare condition the deformities disabilities need to be taken into account before planning management. Here we present a series of 4 cases with different age, sex, site, benign malignant distribution. METHODS AND MATERIAL - 2 male and 2 female patients. 2 patients presented before and 2 patients after skeletal maturity. All of them presented with multiple exostoses. First patient was operated on right elbow even though deformity was in left forearm. Second patient was operated on left proximal humerus. Third patient was advised conservative management. Fourth patient underwent wide excision in left femur for malignant transformation. Classification System Used - Masada et al. Karyotyping was done in all cases. Uncommon surgical approaches (Burger and Buckwalter for Prox. humerus, Antero Lateral Approach for Head of Radius, Medial approach for femur) were used for all the 3 cases which were operated.Even though all patients had multiple tumors only those causing symtoms,disability,malignant transformation were surgically managed. None of the patients had similar family history. DISCUSSION - Even though both our female patients had forearm deformities fit in Masada et al classification those forearms were left untouched because there was no disability there is no evidence that surgical intervention will improve forearm deformities. Biopsy prior to definitive management is absolutely essential in selected doubtful (malignant) cases. HPE was undifferentiable from solitary osteochondroma. Individualize the treatment plan or surgical approach for each patient. Masada et al guidelines may not be applicable to all cases. Karyotyping may point out only gross chromosomal aberrations. Only Gene sequencing may help to predict malignant transformation. CONCLUSION - Deformities cannot be prevented by early excision of osteochondromas. Only symptomatic lesions deserve surgical