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Dwarfism, Achondroplasia and Hypochondroplasia

Dwarfism, Achondroplasia and Hypochondroplasia

Our aim fort he lenrthening program in dwarfism is the correction of the ankle, knee, hip and wrist deformities and treating lumbar hyperlordosis as well as increasing total height of the patient. Decreasing lumbar hyperlordosis lowers the risk of spinal stenosis in adult ages. There are two different treatmet strategies depending on the age at application.

Strategy for juvenile patients

First lengthening:

-between 6-8 years of age -a total of 10 cm lengthening at the femur and the tibia (6 cm femur and 4 cm tibia) -correction of O-bein deformity -mean external fixation time: 5 months Second, third and fourth lengthening: -the same as in adult strategy A total of 30-35 cm lengthening in the lower extremities 10-12 cm lengthening in the upper extremities

Strategy for adults

-between 14-16 years of age -a total of 10-12 cm lengthening at both femora -correction of flexion deformity at both hips (corrects lumbar hyperlordosis) -correction of varus deformity at both hips -mean external fixation time: 10-12 months

First lengthening:

-between 12-14 years of age -a total of 10-15 cm lenthening at both tibiae ( two staged lengthening) -correction of varus deformity of proximal tibia by proximal osteotomy -correction of varus deformity of distal tibia by distal osteotomy -stretching of lateral collateral ligament at the end of lengthening -mean external fixation time: 8-10 months

Second lengthening:

-between 13-15 years -a total of 10-12 cm lengthening at both humeri -correction of flexion deformity of the elbows -mean external fixation time: 6-8 months

Between two lengthening operations for lower limbs, upper limbs are lengthened so that lower limbs can rest.

After the removal of the device, second lengthening can start at least 6 months later. The patient cannot walk during bilateral femoral lengthening. Standing is allowed only for patient transfer. The patient can be mobilized only with wheelchair during lengthening phase. At the consolidation phase, gradually more weight bearing is allowed, and full weight bearing is allowed just before the device is removed.

The patient is allowed to walk with walker or crutches at first day after bilateral tibial lengthening. Wheelchair shall be used for long strolls. There is almost no activity limitation after bilateral humeral lengthening. The patients are controlled every two weeks during the lengthening phase; quantitative sensoryneural tests should be conducted by the doctor himself at each control visit. This approach allows for detection of advancing neural problems before they are clinically evident. Although encountered very rarely in femoral and humeral lengthening, neural problems can cause drop foot (paralysis of the nerve which innervates muscles that pull the foot upwards) if not treated early. If it is detected early and the rate of lengthening is decreased, the neural problem usually resolves by iself and lengthening is continued at a lower rate. If the neural problem continues even after slowing down of the lengthening process, the nerve shall be surgically released. This procedure can be performed through a 1 cm incision, and requires hospitalization for one night. It is similar to the neural release at the wrist performed for carpal tunnel syndrome.