Module 5, Pediatric Orthopaedist Level
This girl was the product of 42 week gestation without complications.
Her birth weight was 7 lbs (3.2 kg), length 18-1/2" (47 cm). At birth
she was found to have bilateral clubfeet and, bilaterally dislocated hips.
Casts were applied to the feet and hips for the first 13 months of age.
The developmental milestones were delayed. She pulled up and walked without
help at 20 months. By age 7 years this girl had 8 mm of C1 occiput mobility
on flexion extension x-rays and 80 degree thoracolumbar scoliosis. She
was able to walk, although slowly. Neurological examination showed normal
reflexes and no asymmetric weakness. At 18 years and 6 months of age she
was 111.6 cm in height and 34.6 kg in weight. Her intelligence was mildly
retarded. Her eyes, and teeth were normal. But the palate was high. She
was a limited community ambulator with the following knee x-rays with definite
knee valgus and recurvatum in stance phase.
Question 5A
A child with this combination of symptoms should have a good genetic evaluation
with a recent chromosome study.
Question 5B
The
most definitive way to evaluate this girl for connective tissue disorders
is to do a skin biopsy.
Question 5C
This
girl may have a form of spondyloepiphyseal dysplasia based on the scoliosis
and knee deformities.
Question 5D
The
girl should probably have tibial osteotomies at this time to help her continue
walking.
Question 5E
This
girls symptoms are most consistent with Larsen's Syndrome, especially with
the dislocated hips, club feet, cervical instability and knee laxity.
Go to Next Question Module, Go
to General Orthopaedist Level Modules, Go
to Question Module Home Page, Go
to Case Presentation Home Page, Orthopaedic
Department Home Page.
