Total hip arthroplasty in patients with skeletal dysplasia

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Skeletal dysplasia, also called osteochondrodysplasias, is different from developmental dysplasia of the hips (DDH), which has been introduced in Part 1 of this series. While DDH normally causes isolated hip joint deformity as a primary condition—with possible involvement of other joints as secondary problems—patients with skeletal dysplasia, a genetic disorder, are usually short in stature and have primary deformities in multiple joints, such as in hips, knees, and spine. It is not unusual for patients with severe forms of skeletal dysplasia to require total hip arthroplasty (THA) at a young age for pain relief and function improvement. Aside from whether good outcomes can follow a total hip arthroplasty in these severely deformed hips, maximizing the survivorship of such procedures is also a serious concern because these patients will likely have one or more revision surgeries in their lifetime. In this part, Seung-Jae Lim from the Department of Orthopedic Surgery, Samsung Medical Center, Seoul, South Korea, shares his knowledge with us on this topic.

Skeletal dysplasia: a term covering many hereditary conditions

Skeletal dysplasia (osteochondrodysplasia) is an umbrella term for more than 350 hereditary conditions of abnormal cartilage and bone development; patients suffering from skeletal dysplasia are afflicted with varying degrees of short stature [1, 2]. Skeletal dysplasia is genetically heterogenous and can be inherited as autosomal dominant, autosomal recessive, X-linked recessive, X-linked dominant disorders, or other rarer modes of transmission. Although each skeletal dysplasia is relatively rare, collectively, different forms of skeletal dysplasia have an incidence of about 1 in 5,000 births [1]. The classification of these skeletal disorders based on radiographic abnormality in the different parts of the long bones, ie, epiphysis, metaphysis, and diaphysis, are most widely used. Further, spondyloepiphyseal, spondylometaphyseal, and spondyloepimetaphyseal dysplasias describe disorders with spinal involvement.

Multiple epiphyseal dysplasia (MED) and spondyloepiphyseal dysplasia (SED) are two common forms of skeletal dysplasia [1, 3]. They are associated with different degrees of abnormalities of the skeleton, resulting in disproportionate short stature (ie, dwarfism) and orthopedic complications. A diagnosis of skeletal dysplasia is usually based on clinical, radiographic, and molecular findings [1].

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Radiographic features
Surgical challenges of total hip arthroplasty in skeletal dysplastic patients
Modular stems: why?
Controversies over modular stems: the neck-stem junction
Current clinical outcomes
Conclusion

Contributing experts

This series of articles was created with the support of the following specialists (in alphabetical order):

This issue was written by Maio Chen, AO Innovation Translation Center, Clinical Science, Switzerland.

References

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