Congenital Anomalies - Other Variants

Question 1

ARTHROGRYPOSIS MULTIPLEX CONGENITA

Answer 1

incomplete congenital fibrous ankylosis or many or all limbs

symmetric and trunk spared

worse - body has no joint

Question 2

clinical pic of ARTHROGRYPOSIS MULTIPLEX CONGENITA

Answer 2

Shoulders IR, wrist flexed

Elbow and knee flexed or extended

Hips Flexed and ER, may be dislocated

Patella absent or dislocated

Probable clubfeet

Congenital high scapula

sometimes biceps and brachioradialis are absent, no active flexion of the elbow

Contractures do not progress

Question 3

sprengels deformity

Answer 3

scapula elevated 1-4 in

(+) omovertebral bone - osseous bridge bet vertebra and scap

surgey bfore 5 yo and postural training

Question 4

clinical picture of sprengels deformity

Answer 4

Inferior angle is rotated medially, sometimes bilateral

Smaller scapula, decreased vertical length, increased width

Shortened cervical muscles on the affected side

sometimes bilateral, ABDUCTION is greatly restricted, ROTATION NOT AFFECTED

Question 5

congenital synotosis of cervical spine

Answer 5

klippel-feil - fusion of all or only lower cervical into one bone

surgery to improve appearance and mob

Question 6

clinical picture congenital synotosis of cervical spine

Answer 6

low post hairline

lateral motion more affected than flexion and ext

pterygium colli or web neck - traps stretch winglike from mastoid to shoulders

Question 7

cleidocranial dysostosis

Answer 7

partial or incomplete absence of both clavicles

delyaed ossification of fontalnels

little disability and not tx indicated