Bruno Alonso, MD
Resident Doctor PGY-2
New York Medical College
NEW YORK, New York, United States
Caroline Bredthauer, BA
Medical Student
New York Medical College
New York, New York, United States
Mohammad S. Islam, MD
Attending Doctor
New York Medical College/ Metropolitan Hospital
New York, New York, United States
Antley-Bixler Syndrome
Case Description:
A 12-year-old boy with PMHx of Antley-Bixler syndrome, craniosynostosis and developmental delay presented with painless contractures of the bilateral 2nd through 5th digits in flexion at the PIP and DIP joints. He was following up after 6 sessions of outpatient occupational therapy (OT) which minimally increased the range of motion of the affected joints. The bilateral 2nd through 5th digits had varying degrees of limited AROM in the PIP and DIP joints. Grip strength was intact bilaterally. After assessment by OT, patient was initially fit for left 4th finger oval-8 finger splint to prevent swan neck deformity for wear during ADLs. Splinting of multiple digits was deferred so as not to interfere with the patient’s ability to use his hands. He was scheduled for further OT sessions and continued evaluation of splinting efficacy.
Discussions:
Antley-Bixler syndrome is a rare congenital disorder with varied manifestations, including craniosynostoses, multiple joint contractures, midface hypoplasia, choanal atresia, and visceral malformations. The use of PT/OT and spinal orthotics in improving musculoskeletal manifestations of Antley-Bixler syndrome has been described in a previous case study; however, more research is required to ascertain the efficacy of hand splinting towards improving the digital contractures seen in Antley-Bixler patients.
Conclusions:
Splinting of contracted DIP joints in Antley-Bixler syndrome patients may improve ROM and passive/active extension of these joints in combination with the current standard of care OT. Future larger studies may be warranted for further exploration of this treatment modality.
