MYELODYSPLASIA
The number of infants with myelodysplasia who survive infancy has increased dramatically in the past 30 years, and as clinical experience with these patients has increased, new principles and techniques of treatment have emerged.
Management of the patient with myelodysplasia requires a team approach.
The team coordinator should visit the patient and family in the first days
after birth and discuss the long-term implications of the patient’s condition.
The urologist, orthopedist, and neurosurgeon should conduct the initial
evaluation, and physical therapy to improve and maintain joint motion should be
started as soon as possible.
The clinical findings dictate the specific orthopedic procedures
employed. The neurologic level of both motor and sensory function should be
estimated. However, in newborns, it is often impossible to determine the
exact level and the findings vary depending on the examiner and the child’s
age. Because there is considerable variation from one side of the body to the
other, each limb should be evaluated separately. The effects of muscle
imbalance and presence of soft tissue contractures must be considered in the
neurologic examination. Evaluation of the newborn should focus on determining
which joints the child can control, and muscle strength can be assessed more
accurately later. The lesion generally follows anatomic lines; thus, even in
children with only mild involvement of the foot there may be significant
weakness of the hip and the abductor muscles and increased tendency to late hip dislocation.
DEFORMITIES OF HIP
In an otherwise normal child, if the hips are dislocated but can be
easily reduced, treatment is the same as that for congenital dislocation of the
hip. If the hip has been dislocated early in fetal life (teratologic
dislocation) and there are advanced adaptive changes of bone and soft tissue,
reduction is not indicated. The primary goal is to produce a freely movable hip
joint.
Contracted and/or spastic adductor muscles in conjunction with weak
power of abductor muscles frequently lead to hip dislocation. If this imbalance
is discovered early, intervention with physical therapy, bracing, or splinting
can help prevent dislocation.
Many patients have contracture of the iliotibial band, which maintains
the hip flexed, abducted, and externally rotated. Early and intensive physical
therapy should be instituted to avoid a fixed deformity.
DEFORMITIES OF KNEE
The newborn with extension or flexion contractures should be treated
with stretching exercises. A major goal of treatment is the ability to extend
the knee and take advantage of the normal locking mechanism.
DEFORMITIES OF FOOT AND ANKLE
Both conservative and surgical management may be necessary to correct
the position of deformed feet, increase suppleness, and allow proper shoe fit.
Cavovarus and equinovarus (clubfoot) should be corrected before the
child learns to stand—before extensive adaptive and remodeling changes in both
bone and soft tissue occur, making surgical intervention necessary. In the
newborn period, calcaneovalgus with overpull of the anterior and paralysis of
the posterior muscles (S1 distribution) often responds well to
exercises, braces, or splints. Deformities such as vertical talus and stiff,
rigid feet are less common.
DEFORMITIES OF SPINE
Although common in patients with myelodysplasia, spinal deformities are
not usually a problem in the newborn period. However, congenital anomalies of
the vertebrae occur in about 30% of affected children. In addition, 50% of
patients eventually develop a curve in the
otherwise normal-appearing vertebral bodies (developmental scoliosis).
Congenital kyphosis is unique to myelodysplasia and is of such magnitude
that it can be recognized at birth. The kyphosis usually involves the entire
lumbar spine from the thoracolumbar junction down, including the sacrum, with
its apex at L2 or L3; there is usually complete paralysis below the level of
the lesion. In the newborn, the size of the cutaneous defect and the rigidity
and magnitude of the curve may make skin closure extremely
difficult.
