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


METHODS
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.


CONCLUSIONS
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.
Eylon
Sharon
S
Pediatric Orthopaedic Unit, Orthopaedic Department, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. sharone@hadassah.org.il
Beeri
Maurit
M
Joseph
Keenan
K
Meyer
Shirley
S
D000015:Abnormalities, Multiple / Q000601:surgery*; D000844:Ankylosis / Q000601:surgery*; D002648:Child; D004480:Ectromelia / Q000601:surgery*; D005260:Female; D005269:Femur / Q000002:abnormalities* / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007719:Knee Joint / Q000601:surgery; D013977:Tibia / Q000002:abnormalities* / Q000601:surgery; D016896:Treatment Outcome