HEREDIATARY MULTIPLE EXOSTOSES - CASE SERIES

Abstract

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