Femorotibial ankylosis in a child with Roberts syndrome: an "aggressive" approach to habilitation

Journal of Pediatric Orthopedics
Sharon EylonShirley Meyer

Abstract

Roberts syndrome is a rare autosomal recessive disorder affecting many organs. The marked disabilities are mainly due to extreme short stature and severe limb deformities. Treatment in children who survive focuses on surgical correction of facial and limb defects. Z.B., a 9.5-year-old girl, was diagnosed at birth with Roberts syndrome. She gradually achieved gross and fine motor developmental milestones and spontaneously ambulated by "walking" on her knees. Her primary obstacle to further improvement was the severe fixed flexion deformities of the knees. At age 5 years and 9 months, she underwent surgery which involved quadriceps splitting, exposure, and then excision of the bony ankylosis. Stable fixation was achieved with crossed Kirschner wires. Quadriceps reefing was then performed, and long-leg casts applied. Currently, she walks independently without orthoses and functions as well as her height permits. Knee ankylosis is a very rare entity, and the affected patients are usually treated nonoperatively. Careful evaluation and appropriate surgery afforded our patient a better quality of life and should be considered in other patients.

References

Jan 1, 1979·American Journal of Medical Genetics·Q H QaziS J Choi
Jun 1, 1977·European Journal of Pediatrics·J Herrmann, J M Opitz
Nov 15, 1993·American Journal of Medical Genetics·D J Van Den Berg, U Francke
Oct 23, 1997·American Journal of Medical Genetics·M UrbanP Krietsch
Nov 1, 1919·Annals of Surgery·J B Roberts

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