mangal parihar
2/Aug/2000
NOTES___________________________________________________
Essentials of caffeys pediatric x-ray diagnosis
silverman and kuhn, year book medical publishers 1996
pgs 836 to 899
Metaphyseal chondrodysplasia, type schmid
Mild manifestations, moderate, progressive shortening of stature, bowed legs.
Before epiphyseal union, radiographic examination discloses widening, cupping, and
defective irregular mineralisation of the metaphyses of the tubular bones that is much
less severe than in the jansen type.
Occasionally bony spicules passing into the epiphysis from the metaphysis, may be seen.
None of the disorganised metaphyseal clcification that occurs in the jansen type.
The round bones of the wrists and ankles and the epiphyseal ossification centers are not
affected. Confusion with the radiographic changes of vitamin d resistant rickets often
occurs.
Spondylo-metaphyseal dysplasias
involvement of the axial skeleton (platyspondyly, sail vertebra, ballooning of disc
spaces, pelvic changes)
Multiple epiphyseal dysplasia and other epiphyseal dysplasias
The most common form is the Fairbank type.
Patients come for poor growth, problems with walking or joint pains, but rarely before 2
years of age.
Diagnosis is radiographic. Ossification centers are late in appearance, small and
irregularly mineralised. Usually symmetrical. at maturity, affected bone ends may become
distorted as after osteochondroses.
Osteoarthritic changes are frequent in adults.
Verterbral bodies may show mild end plate deformities. Carpal and tarsal bones are also
affected.
Meyer dysplasia, isolated bilateral hip involvement
Ribbing type is milder than the Fairbank type and is often referred to as
the Flat epiphysis type to differentiate from the Small epiphysis
type of fairbank
Pseudoachondroplasia
A special form of spondyloepiphyseal dysplasia. Short limbed rhizomelic dwarfism of late
onset (2 - 4 yrs of age)
Mild to moderate spinal deformity, normal craniofacial structure slight irregularities of
vertebral end plates worsen with time epiphyseal ossification centers are small and
irregularly mineralised,
Spondyloepiphyseal dysplasia tarda
Short trunk dwarfism. Vertebral bodies flattened
Rickets
Normally sharply defined provisional zone of calcification fades out indistinctly into the
soft tissue density of the adjacent epiphyseal cartilage. In rickets the space between the
metaphysis and its poorly calcified epiphysis is deepened.
In the shaft a diffuse rarefaction develops, with coarsening of the trabecular
architecture. Greenstick fractures and radiolucent transverse bands resembling stress
fractures may occur.
Epiphyseal ossification centers in the carpal and tarsal bones are similar to those in the
shafts - margins disappear and the spongiosa becomes osteopenic and even invisible in
severe cases.
The first evidence of healing is a reappearance of the provisional zone of calcification.
As healing progresses, the metaphysis becomes mineralised from the shaft toward the
epiphysis, ultimately resulting in radiographic
continuity of the shoft with the provisonal zone of calcification.
Vitamin D resistant rickets
The several forms of Vitamin D resistant and Vitamin D dependant rickets often
dmonstrate marked lower-limb bowing in addition to metaphyseal changes resembling those in
metaphyseal chondrodysplasias.
Renal Rickets
is often associatied with a chalky appearance of the bones as well as auxiliary
signs of hyperparathyroidism theat are related to the retention of phosphate by the
damaged kidney
Hemant Sharma Aug/2/2000 csona@hotmail.com
I think it is very diff to have certain diagniosis, but
"Chondrometaphysealdysplasia" is the most likely one. The reason I feel that is
Normal Epiphysis
Patient is normal at birth
Spine is normal
Head and face normal
Widening of physis seen.
Though I am a bit worried about the distal radial growth plate's increased height (not the
widening) and fuzziness of distal Ulna. Nutritional rickets should also be considered.
I have not seen nutritional rickets but have done some work on Vitamin D resistant
rickets and this seems unlikely. We have also done deformity correction (what else,
Ilizarov frame) in few metaphyseal dysplasia's and I am attaching x ray of a patient for
you to compare. If you want I can send some more x rays of different patients as well.
I hope this helps.
best wishes
Hemant Sharma
MS(orth) M Ch(orth) FRCS(orth)
Musgrave Park Hospital
Belfast
Sanjeev Sabharwal sabharsa@UMDNJ.EDU July 30th
2000
The differential would include all types of rickets and metaphyseal chondrodysplasia.
Probably needs a metabolic workup to R/o rickets. Also get x rays of the remaining
joints of upper extremity.
Regards,
Sanjeev Sabharwal
Pediatric Orthopedics
This looks like multiple epiphyseal dysplasia
Dr.Idiculla
This is metaphyseal dysplasia. All epiphyses normal. Metaphyses of
femur are involved, so also of the radius and ulna.
G.S.KULKARNI
Please consider SPONDYLOEPIPHYSEAL DYSPLASIA.
This is multiple epiphyseal dysplasia. It is familial. Here the sibling has shown
invovement of many epipyses. The baby will result in Bil. Tibia vara, Coxavara. I have a
family where most of the siblings have shown Valgoid deformity of Ankles only. Amongst the
family, one has shown coxa vara but no valgoid deformity of ankles. One baby is born with
CTEV. Most of the members in the four generations have shown dwarfism to some extent. I
have presented this problem of valgoid ankles at Varanasi during IOAICL. One patient came
to me Valgoid ankles. The fibular epiphyses had not fused even at the age of 35 years. The
daughter has started developing the ankle problem at the age of 10 years. The males are
spared from the deformities but have general dwarfism. There are six individuals suffering
from this problem. It seems that the problem is transmitted by females.
Thanks.
Pravin Kanabar, Ahmedabad.
From: V.P. Bhavalkar bhavalka@barbourville.com
Sent: Monday, July 31, 2000 4:29 AM
Is this Blount's disease or Syndrome ?
Anil Bhavalkar. MD, FRCS
Barbourville, KY,USA
hip, wrist and ankle involved also. and knee valgus?
mangal
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