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Disturbed pathoanatomy of diastrophic hips, knees makes surgical correction a challenge
Weiner DS. J Ped Orthop. 2010;30(4):403-410. Doi:10.1097/BPO.0b013e3181e27f12.
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Recent research provides the most extensive 3-D pathoanatomical observations of diastrophic hip and knee dysplasia to date.
“Surgical correction of the hip and knee in patients with diastrophic dysplasia is extremely difficult secondary to the markedly distorted pathoanatomy of both the bone and soft tissues,” Dennis S. Weiner, MD, and colleagues at Akron Children’s Hospital, Ohio, wrote in their study. The goal of their research was twofold: produce a 3-D model and methodically describe the pathoanatomy of the diastrophic hip and knee.
They based the 3-D computer model reconstructions on the clinical, radiographic and surgical observations performed by a single surgeon on 110 hips in 55 patients. The researchers also reviewed 13 patients under the care of another surgeon.
To bolster the clinical and radiographic evaluations, the researchers performed 10 computed tomography scans of the pelvis and hip, and they took 53 arthrograms of 13 patients. Sixty-six patients had various hip and proximal femur surgeries.
Weiner and colleagues found that the typical pathoanatomy in diastrophic dysplasia cases led to severe and rampantly progressive hip and knee flexion deformity. These patients had anatomical aberrations in the proximal femur and knee joints, which are highlighted in the researchers’ computer model.
Total hip and knee arthroplasty may be the only treatment for adolescents and adults with advanced disease, the researchers wrote. The dearth of long-term data, however, prohibits a solid management recommendation.