Objective To study the effectiveness and acetabular prosthesis selection of the total hip arthroplasty (THA) for Crowe type IV congenital dysplasia of the hip with dislocation in adults. Methods Between June 2008 and May 2012, 8 adult patients (8 hips) with Crowe type IV congenital dysplasia of the hip with dislocation underwent THA. They were all female, aged 20-35 years with a mean age of 25 years. The left hip was involved in 5 cases and the right hip in 3 cases. The Harris score of involved hip was 53.9 ± 6.6. The shortened length of involved extremity was 4-6 cm (mean, 4.8 cm). The X-ray films showed complete dislocation in all cases. The acetabular prosthesis with diameter of 42-44 mm and S-ROM femoral prosthesis were used in THA. Results The incisions healed by first intention. There was no hip dislocation events and sciatic nerve injury during the follow-up. Femoral nerve injury occurred in 1 case and asymptomatic venous thrombosis of the leg muscle occurred in 2 cases. All the patients were followed up 1-5 years (mean, 3 years). All cases showed obvious improvement of claudication and could restore to work. At 6 months after operation, the mean length difference between affected and contralateral extremities was 0.4 cm (range, — 1.0-0.6 cm); the Harris score was significantly increased to 87.6 ± 0.3 (t=1.77, P=0.00). The X-ray films showed that all cases got bony union at 3-6 months after operation and stable interface between acetabular prosthesis and bone. No revision was involved during the follow-up. Conclusion THA with small acetabular cup and subtrochanteric osteotomy is an effective method in the treatment of Crowe type IV congenital dysplasia of the hip with dislocation in adults. The early effectiveness is satisfactory. The long-term survival rate of prosthesis needs to be followed up.
ObjectiveTo summarize the research progress in clinic, development, and genetics of the Klippel-Feil syndrome and its primary ear deformity. MethodsThe related 1iterature at home and abroad concerning the Klippel-Feil syndrome with ear malformation was reviewed, analyzed, and summarized. ResultsThe clinical manifestation and classification of Klippel-Feil syndrome are complicated. As one of the most important accompany malformations, ear deformity mainly leads to hearing impairment and abnormal appearance. However, it is still unclear exactly how the ear deformity forms in Klippel-Feil syndrome, and there is little deep study on the internal connection between the ear deformity and other malformations. The premise for the treatment of Klippel-Feil syndrome includes accurate diagnoses and comprehensive disease assessment, and multidisciplinary collaboration will be the important direction of clinical practice in the future. ConclusionEar malformation is one of the most important congenital dysplasias in the Klippel-Feil syndrome. Its etiology should be based on research in the development and genetic mechanism. And its diagnosis and treatment should be followed by multidisciplinary collaboration. It is important to pay attention to identifying with ear malformation in other syndromes as well.