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Dr MADAN MOHAN, Consultant ,
Orthopaedics, KIMS TRIVANDRUM
Osteochondroma
Dr Madan's Slide
• Developmental malformations rather
than true neoplasms and are thought
to originate within the periosteum as
small cartilaginous nodules.
• Lesions consist of a bony mass, often
in the form of a stalk, produced by
progressive endochondral ossification
of a growing cartilaginous cap.
Dr Madan's Slide
• Their growth usually parallels that of
the patient and usually ceases when
skeletal maturity is reached
• Usually are found on the metaphysis
of a long bone near the physis
• They are seen most often on the distal
femur, the proximal tibia, and the
proximal humerus. They rarely
develop in a joint
Dr Madan's Slide
Trevor disease (dysplasia
epiphysealis hemimelica)
refers to an intraarticular
epiphyseal
osteochondroma.
Dr Madan's Slide
• Many of these lesions cause no symptoms and
are discovered incidentally.
• Some cause mechanical symptoms by irritating
the surrounding structures, and rarely one
becomes painful due to a fracture.
• False aneurysms of major lower extremity
vessels as the result of pressure from
osteochondromas have been reported.
• Also, neuropathies caused by pressure from a
contiguous osteochondroma have occurred; the
physical finding usually is a palpable mass.
Dr Madan's Slide
• Multiple hereditary exostoses is an
autosomal dominant condition
with variable penetrance.
• Most patients with this disorder
have a mutation in one of two
genes: EXT1, which is located on
chromosome 8q24.11-q24.13, or
EXT2, which is located on
chromosome 11p11-12.
Dr Madan's Slide
• The most striking feature is the presenceof
many exostoses, but disturbances in
growth also occur, such as abnormal
tubulation of bones, producing broad and
blunt metaphyses, and sometimes bowing
of the radius and shortening of the ulna,
producing ulnar deviation of the hand.
• The disease occurs only 5% to 10% as
often as solitary osteochondroma and is
more common in males.
Dr Madan's Slide
• Osteochondromas are of two types:
pedunculated and broad based or sessile
• Pedunculated tumors are more common,
and any definite stalk is directed away
from the physis adjacent to which it takes
its origin.
• The projecting part of the lesion has
cortical and cancellous components, both
of which are continuous with
corresponding components of the parent
bone.
Dr Madan's Slide
SOLITARY OSTEOCHONDROMA, LUSCHKA’S
TUBERCLE. Scapula
Dr Madan's Slide
OSTEOCHONDROM
A AND
NON-OSSIFYING
FIBROMA.
Distal Femur
Dr Madan's Slide
Sessile Osteochondromas
Dr Madan's Slide
Cauliflower Osteochondroma
Dr Madan's Slide
Coat-hanger
exostosis
Dr Madan's Slide
OSTEOCHONDROMA WITH A LARGE
OSTEOCARTILAGINOUS CAP
Dr Madan's Slide
• The lesion is covered by a cartilaginous
cap that often is irregular and usually
cannot be seen on radiographs;
occasionally, calcification within the cap
may be seen.
• Typically, the cap is only a few millimeters
thick in adults, although it may be 2 cm
thick in a child.
• A bursa frequently overlies the tumor and
may contain osteocartilaginous loose
bodies. Dr Madan's Slide
• Malignant degeneration is extremely
rare.
• Large series have estimated the
incidence of malignant degeneration
to be approximately 1% for patients
with a solitary osteochondroma and
5% for patients with multiple
hereditary exostoses
Dr Madan's Slide
• Malignant transformation should be
suspected when a previously
quiescent lesion in an adult grows
rapidly; it usually takes the form of a
low-grade chondrosarcoma.
• In these cases, the cartilage cap
usually is more than 2 cm thick.
• Malignant transformation is best
evaluated by CT or MRI.
Dr Madan's Slide
• Surgery (en bloc resection) is indicated
when the lesion is large enough to be
unsightly or produce symptoms from
pressure on surrounding structures or
when imaging features suggest
malignancy.
• Recurrence is rare and probably is caused
by failure to remove the entire
cartilaginous cap.
• Patients with multiple hereditary
exostoses may require osteotomies to
correct deformity.Dr Madan's Slide
Dr MADAN MOHAN, Consultant ,
Orthopaedics, KIMS TRIVANDRUM
Hereditary Multiple Exostosis
Dr Madan's Slide
• An inherited autosomal dominant
metaphyseal overgrowth that is
characterized by multiple
osteochondromas
• Originally described by Boyer in
1814
Dr Madan's Slide
Common sites of HME
Dr Madan's Slide
• A characteristic bayonet hand deformity occurs
about the wrist as a result of retardation of
bone growth; the deformity is characterized by
shortening of the ulna, outward bowing of the
radius, and a subluxation of the radioulnar
joint
Dr Madan's Slide
Bayonet
Hand
Deformity
Dr Madan's Slide
Malignant
Degeneration
Dr Madan's Slide
Dr MADAN MOHAN, Consultant ,
Orthopaedics, KIMS TRIVANDRUM
14 yr old boy
March 2012
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr MADAN MOHAN, Consultant ,
Orthopaedics, KIMS TRIVANDRUM
March 2013
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide
Dr MADAN MOHAN, Consultant ,
Orthopaedics, KIMS TRIVANDRUM
Multiple
Hereditary
Exostosis
Dr Madan's Slide
• Characterised abnormal
proliferation of epiphyseal
chondroblasts that cause a
subsequent defect in metaphyseal
remodelling
• AD with 96% penetrance
• Ch no 8,11 and 19
• Malignant transformation?
Dr Madan's Slide
Metaphyseal bony prominences
capped with cartilage and
retardation of longitudinal bone
growth
Dr Madan's Slide
• 30-60% patients with MHE present with
forearm lesions
• Most of the longitudinal growth of the ulna
occurs in the distal physis
• Ulnar tether
Dr Madan's Slide
Dr Madan's Slide
Dr Madan's Slide

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Dr Madan Mohan's Presentation on Osteochondroma and Hereditary Multiple Exostosis

  • 1. Dr MADAN MOHAN, Consultant , Orthopaedics, KIMS TRIVANDRUM Osteochondroma Dr Madan's Slide
  • 2. • Developmental malformations rather than true neoplasms and are thought to originate within the periosteum as small cartilaginous nodules. • Lesions consist of a bony mass, often in the form of a stalk, produced by progressive endochondral ossification of a growing cartilaginous cap. Dr Madan's Slide
  • 3. • Their growth usually parallels that of the patient and usually ceases when skeletal maturity is reached • Usually are found on the metaphysis of a long bone near the physis • They are seen most often on the distal femur, the proximal tibia, and the proximal humerus. They rarely develop in a joint Dr Madan's Slide
  • 4. Trevor disease (dysplasia epiphysealis hemimelica) refers to an intraarticular epiphyseal osteochondroma. Dr Madan's Slide
  • 5. • Many of these lesions cause no symptoms and are discovered incidentally. • Some cause mechanical symptoms by irritating the surrounding structures, and rarely one becomes painful due to a fracture. • False aneurysms of major lower extremity vessels as the result of pressure from osteochondromas have been reported. • Also, neuropathies caused by pressure from a contiguous osteochondroma have occurred; the physical finding usually is a palpable mass. Dr Madan's Slide
  • 6. • Multiple hereditary exostoses is an autosomal dominant condition with variable penetrance. • Most patients with this disorder have a mutation in one of two genes: EXT1, which is located on chromosome 8q24.11-q24.13, or EXT2, which is located on chromosome 11p11-12. Dr Madan's Slide
  • 7. • The most striking feature is the presenceof many exostoses, but disturbances in growth also occur, such as abnormal tubulation of bones, producing broad and blunt metaphyses, and sometimes bowing of the radius and shortening of the ulna, producing ulnar deviation of the hand. • The disease occurs only 5% to 10% as often as solitary osteochondroma and is more common in males. Dr Madan's Slide
  • 8. • Osteochondromas are of two types: pedunculated and broad based or sessile • Pedunculated tumors are more common, and any definite stalk is directed away from the physis adjacent to which it takes its origin. • The projecting part of the lesion has cortical and cancellous components, both of which are continuous with corresponding components of the parent bone. Dr Madan's Slide
  • 14. OSTEOCHONDROMA WITH A LARGE OSTEOCARTILAGINOUS CAP Dr Madan's Slide
  • 15. • The lesion is covered by a cartilaginous cap that often is irregular and usually cannot be seen on radiographs; occasionally, calcification within the cap may be seen. • Typically, the cap is only a few millimeters thick in adults, although it may be 2 cm thick in a child. • A bursa frequently overlies the tumor and may contain osteocartilaginous loose bodies. Dr Madan's Slide
  • 16. • Malignant degeneration is extremely rare. • Large series have estimated the incidence of malignant degeneration to be approximately 1% for patients with a solitary osteochondroma and 5% for patients with multiple hereditary exostoses Dr Madan's Slide
  • 17. • Malignant transformation should be suspected when a previously quiescent lesion in an adult grows rapidly; it usually takes the form of a low-grade chondrosarcoma. • In these cases, the cartilage cap usually is more than 2 cm thick. • Malignant transformation is best evaluated by CT or MRI. Dr Madan's Slide
  • 18. • Surgery (en bloc resection) is indicated when the lesion is large enough to be unsightly or produce symptoms from pressure on surrounding structures or when imaging features suggest malignancy. • Recurrence is rare and probably is caused by failure to remove the entire cartilaginous cap. • Patients with multiple hereditary exostoses may require osteotomies to correct deformity.Dr Madan's Slide
  • 19. Dr MADAN MOHAN, Consultant , Orthopaedics, KIMS TRIVANDRUM Hereditary Multiple Exostosis Dr Madan's Slide
  • 20. • An inherited autosomal dominant metaphyseal overgrowth that is characterized by multiple osteochondromas • Originally described by Boyer in 1814 Dr Madan's Slide
  • 21. Common sites of HME Dr Madan's Slide
  • 22. • A characteristic bayonet hand deformity occurs about the wrist as a result of retardation of bone growth; the deformity is characterized by shortening of the ulna, outward bowing of the radius, and a subluxation of the radioulnar joint Dr Madan's Slide
  • 25. Dr MADAN MOHAN, Consultant , Orthopaedics, KIMS TRIVANDRUM 14 yr old boy March 2012 Dr Madan's Slide
  • 32. Dr MADAN MOHAN, Consultant , Orthopaedics, KIMS TRIVANDRUM March 2013 Dr Madan's Slide
  • 38. Dr MADAN MOHAN, Consultant , Orthopaedics, KIMS TRIVANDRUM Multiple Hereditary Exostosis Dr Madan's Slide
  • 39. • Characterised abnormal proliferation of epiphyseal chondroblasts that cause a subsequent defect in metaphyseal remodelling • AD with 96% penetrance • Ch no 8,11 and 19 • Malignant transformation? Dr Madan's Slide
  • 40. Metaphyseal bony prominences capped with cartilage and retardation of longitudinal bone growth Dr Madan's Slide
  • 41. • 30-60% patients with MHE present with forearm lesions • Most of the longitudinal growth of the ulna occurs in the distal physis • Ulnar tether Dr Madan's Slide