Module 52, General Orthopaedist Level
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A three year old girl is brought for evaluation because of possible
scoliosis. Her developmental history was of a normal vaginal delivery weighting
8 pound 6 ounce, full term female with no perinatal events and normal intrauterine
movement. She was developing normally until 6 months of age when parents
noticed she stopped moving her legs and lost the ability to roll over.
At that time a work up including muscle biopsy concluded that the diagnosis
was Spinal Muscular Atrophy (SMA). She was managed with outpatient and
inpatient rehabilitation consisting of intensive physical, occupational
and aquatic therapy and the use of long leg braces at age of 2. By age
three there was concern about her spine. Radiographic evaluation at age
of 3 (image 1) and age of 6 (image 2)were done.
Question 52A
The
correct treatment for this child is to have the child in a TLSO scoliosis
brace for 24 hours a day.
Question 52B
The
primary attention should be directed at the dislocated hips by doing an
open reduction and placing the child in cast.
Question 52C
A
careful genetic history should be obtained because this condition has a
sex linked recessive inheritance pattern.
Question 52D
The
anatomic location of the defect is in the cerebral motor cortex which cause
the spinal muscles to atrophy first.
Question 52E
The classic physical examination of SMA is a very weak child with hyper
reflexia but normal sensation and no cognitive limitation or eye findings.
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