Sunday, October 11, 2015

Young Adult with Multiple Hereditary Exostoses

Multiple Hereditary Exostosis is an uncommon condition with the growth of multiple benign tumors or bone/cartilage growths.  These can be called osteochondromas or exostoses.  It has been estimated to be present in 1 in 50,000 children.  Here is a great reference- NLM site. The growths can present in several different ways.
1. Bumps which are not painful
2. Bumps which are painful, often related to pressure on cutaneous nerves or other structures
3. Bumps which cause problems with bone growth.  These are usually in the forearm or lower leg- segments with 2 bones.  Altered growth of one of these bones affects the other bone.

There is a strong hereditary component to Multiple Hereditary Exostoses.  It can be inherited in an autosomal dominant fashion, related to the EXT1 and 2 genes.  Typically, we, in the United States, see these children at a relatively young age and certainly by the beginning of puberty.  The osteochondromas grow rapidly at the time of rapid bone growth.

We often have the chance to care for children from other countries, sometimes thanks to the generosity and coordination of the World Pediatric Project.  This amazing organization helps many children with orthopedic and non orthopedic conditions.  Check out there website HERE.  We recently took care of a patient from Central America with Multiple Hereditary Exostoses.  He had not undergone any previous treatment.  Here are some pictures:
Multiple Hereditary Exostoses.  Note the lesions on the right wrist and both legs.

Multiple Hereditary Exostoses

Here are some x-rays showing the bumps.
Multiple Hereditary Exostoses.  Note the bumps on the bone.

Multiple Hereditary Exostoses.  Note the big bump on the outside of the humerus.
This patient was taken to the operating room for removal of the painful bumps.
Multiple Hereditary Exostoses, the excision of 3 lesions.
Multiple Hereditary Exostoses.  One large osteochondroma prior to removal.

Charles A. Goldfarb, MD
My Bio at Washington University
congenitalhand@wudosis.wustl.edu

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