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Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases
Meni Mundama1, Serge Ayong1, Renaud Rossillon1
1Department of Orthopaedic Surgery, Clinique Saint-Pierre Ottignies, Avenue Reine Fabiola 9, 1340 Ottignies, Belgium.
Abstract:
Say-Barber-Biesecker-Young-Simpson's syndrome is one of the Ohdo-like syndromes. It is a very rare congenital condition that is commonly defined by its main clinical features that are blepharophimosis, ptosis, mental retardation, and delayed motor development. They are often associated with skeletal manifestations that are joint laxity, long thumbs and toes, and hypoplastic and/or dislocated patellae. To our knowledge, the available literature does not report any case where attention is drawn to management of skeletal aspect of this specific syndrome, especially surgically. We report 2 cases of SBBYS syndrome with patellar dislocation that we followed for 11 years. One case (with bilateral dislocation) was managed conservatively, and the other (with unilateral dislocation) underwent conservative and surgical treatment. Both had good functional outcome at follow-up. This experience shows that patellar abnormality in this condition can be efficiently addressed conservatively and/or surgically with satisfying results.
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