Dr. Kozin serves on the Scientific and Medical Advisory Board of the MHE Research Foundation

Publications authored by Dr. Kozin
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List of Publications via PubMed
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Dr. Scott Kozin
Dr. Kozin treats many case of MHE / MO / HME every week, he is one of the best in the world dealing with the arm deformities
that can occur in children with Multiple Hereditary Exostoses. Dr. Kozin cheif of staff at Shriners Hospital for
Children-Philadelphia. Dr. Kozin served as President of the
American Association for Hand Surgery (2008) and is a member
of the Scientific and Medical Advisory Board of our Foundation and served on the Conference Advisory Committee
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Wings of HOPE as we REACH
for the CURE to
Multiple Hereditary Exostoses Syndrome
Multiple Osteochondroma Syndrome

MHE of the Upper Extremity

Kozin, SH
Shriners Hospital for Children, Philadelphia, 3551 N. Broad Street, Philadelphia,PA 19140-4131.

Hereditary multiple exostoses (HME) is an inheritable disorder of enchondral bone growth. HME is inherited in an autosomal
dominant pattern with high penetrance and variable expressivity. Cartilaginous exostoses, grow from the physes of long bones
and from the pelvis, ribs, scapula, and vertebrae. The most common upper extremity sites of involvement are the, humerus,
scapula, distal radius and ulna, elbow, and hands. Approximately 1/2 of all patients have forearm involvement.

A clinical "bump search" of all long bones is indicated if HME is suspected. Shoulder, elbow, forearm, wrist, and digit range of
motion should be measured. Radiographs should be obtained of any part of the extremity where osteochondromas are
suspected or that have decreased range of motion. Serial measurements of forearm rotation are used to document progressive
forearm deformity.

Most exostoses are asymptomatic and do not require removal. Exostoses may cause local discomfort, nerve or tendon
impingement, decreased range of motion, and longitudinal and angulatory growth abnormalities. Several reports of adults with
untreated forearm deformities due to HME indicated that they maintained function and were comfortable with their appearance.
These reports question the role of aggressive surgical treatment to maintain or improve function.  

Upper Extremity Surgery: Osteochondromas may cause visible local pain, deformity, and growth disturbance. Malignant
transformation is uncommon. Local pain, caused by impingement of the osteochondroma on surrounding tissue, is a frequent
indication for exostosis removal, and is effectively relieved by excision.  If forearm rotation is blocked by an exostosis, removal
will often improve motion.

Forearm osteochondromas frequently cause a length discrepancy between the radius and ulna. The radius becomes longer than
the ulna and accepts the entire forearm load, resulting in radial bowing, radial tilting, and possible radial head dislocation. The
increased radial inclination and lack of ulnar support positions the wrist into ulnar deviation and causes the carpus to "slip"
toward the ulna.

Early osteochondroma removal to retard or prevent progressive growth disturbances is controversial. Hemiepiphyseal stapling
of the radial side of the distal radius retards radial growth and allows correction of the radial articular angle and ulnar length
discrepancy with growth. Ulnar lengthening may be performed in a single stage or using gradual distraction osteogenesis.
Differential lengthening combined with angular correction can be used to reduce the radial head, because restoration of normal
forearm anatomy may result in spontaneous radial head reduction.  In patients with an isolated distal ulnar lesion, freeing the
soft tissue tether between the ulna and ulnar sided carpus and distal radius may prevent radial head dislocation. The dislocated
radial head may become painful in the adolescent; resection relieves pain and removes the associated prominence. Radial head
resection is delayed until skeletal maturity, because removal of the radial head in the growing child may cause cubitus valgus or
proximal radial overgrowth.  Creation of a one-bone forearm may be used to salvage a severely disorganized forearm.

In adults, an indication for HME excision is suspected malignant transformation. Symptoms and signs of malignant
transformation include local pain and growth of an osteochondroma after skeletal maturity. Radiographic changes include
internal lytic areas, erosion or destruction of the adjacent bone, and/ or presence of a soft tissue mass containing irregular
calcifications.  Malignant transformation, however, is quite rare in the upper extremity.












































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