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.