An Algorithm for the Application of Furlow Palatoplasty to the Treatment of Velocardiofacial Syndrome Associated Velopharyngeal Insufficiency
S. Alex Rottgers, Darren Smith, M.D., Matthew Ford, MS CCC-SLP, Lorelei Grunwaldt, M.D., Alexander Lin, M.D., Christopher Kinsella, M.D., Joseph Losee, M.D..
Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
BACKGROUND: Outcomes when treating velopharyngeal insufficiency (VPI) associated with velocardiofacial syndrome (VCFS) are often disappointing. Controversy remains regarding the primary treatment of patients with VCFS and submucous clefting (SMCP). Treatment with palatoplasty has varied success, and therefore a posterior pharyngeal flap (PPF) is often suggested. This procedure carries a risk of obstructive sleep apnea (OSA) though. Our center utilizes an algorithm to treat clefting in VCFS with Furlow palatoplasty as the primary treatment for overt clefting and SMCP when evidence of velopharyngeal (VP) mobility is present. A primary PPF procedure is used to treat akinetic palates. This study investigates the success with this therapy.
METHODS: An algorithm for treatment of VCFS associated clefting was created.
1) Overt clefts were treated with primary Furlow palatoplasty
2) SMCP with documented VP mobility were treated with primary Furlow palatoplasty
3) SMCP with documented absence of VP mobility were treated with primary PPF
4) Cases of persistent or recurrent VPI following primary Furlow palatoplasty were treated with secondary PPF
A retrospective review of all patients from our cleft-craniofacial database with the diagnosis of VCFS who were treated according to the above algorithm was performed. Palatal motion was documented through physical exam, nasoendoscopy, or videofluoroscopy. Speech was evaluated by a certified speech pathologist utilizing the Pittsburgh Weighted Speech Score (PWSS).
RESULTS: 31 patients with VCFS were treated according to the algorithm. 6% (n=2) of patients had overt clefts. 77% (n=24) had documented VP mobility and 16% (n=5) had no evidence of VP motion. 84% (n=26) of patients had primary Furlow palatoplasties, and 16% (n=5) underwent primary PPF. Adequate VP function (PWSS <7) was achieved in 52% (n=16) of patients. 19% (n=6) of patients were undergoing recommended f/u and speech therapy to determine the need for subsequent secondary PPF, 10% (n=3) were awaiting a recommended secondary PPF, and 19% (n=6) had insufficient post-op speech testing to determine the success of treatment. None of the patients with overt clefting achieved adequate VP competence with Furlow palatoplasty alone. 42% (n=10) of SMCP patients treated with primary Furlow palatoplasty achieved adequate VP function with this single procedure. 17% (n=4) of the SMCP primary Furlow palatoplasty group underwent secondary PPF. 60% (n=3) of patients with primary PPF and 75% (n=3) with secondary PPF demonstrated adequate VP function.
CONCLUSIONS: When treating VCFS related VPI in appropriately selected patients, Furlow palatopasty represents an alternative to primary PPF. This avoids the risk of sleep apnea associated with PPF in a substantial portion of patients, but the need for secondary pharyngoplasty must be considered. We present a treatment algorithm for proper application of Furlow palatoplasty in VCFS associated VPI. More patients and longer follow-up will allow us to determine the ultimate success rate and likelihood secondary procedures will be required.
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