Congenital Tibial Dysplasia with Intact Fibula

PERRY SCHOENECKER, MD*, A. M. CAPELLI, RN,EDWARD A. MILLAR, MD, M. R. SHEEN, MD, THOMAS HAHER, MD,MICHAEL D. MONA, MD, AND LESLIE C. MEYER, MDSaint Louis, Missouri


Sixty-six cases of tibial dysplasia (Group II), absent or deficient tibia with intact fibula, presented to the Saint Louis, Chicago, and Greenville Units of Shriners Hospitals for Crippled Children from 1961 to 1983. Fifty-seven patients (71 extremities) had sufficient follow-up for review. Medical records and radiographs documented the degree of dysplasia and the treatment.

Extremities were categorized into Jones' radiographic subtypes. Most had the greatest degree of tibial deficiency; 54 extremities were types I and II and 17 were types III and IV. Surgical ablation was performed on 61 extremities. Of the types I and II, 22 extremities had knee disarticulations, 25 had Syme's amputations and one had Chopart disarticulation. The Brown procedure generally yielded less than satisfactory results; 9 of 14 extremities required additional surgery after the fibular realignment and Syme's amputation. Of the types III and IV, nine had Syme's and four had Chopart's amputations. In spite of satisfactory ankle reconstruction in eight of ten type IV extremities, Syme's amputations were eventually performed in six because of anticipated severe leg length discrepancy. One patient had a successful tibial lengthening. Two type IV extremities retained the foot because the leg length could be accommodated by a contralateral prosthesis. At an average follow-up of nine years, all patients are independent ambulators, with the exception of one who has neuromuscular disease.

*Shriners Hospitals for Crippled Children, 2001 South. Lindbergh Boulevard, Saint Louis, MO 63131