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The use of Furlow Palatoplasty in Velocardiofacial Syndrome Related Submucous Clefting
S. Alex Rottgers MD
Matthew Ford MS-CCC-SLP
Alexander Lin MD
Lorelei Grunwaldt MD
Darren Smith MD
Christopher Kinsella MD
Joseph Losee MD
University of Pittsburgh, Children's Hospital of Pittsburgh, Division of Plastic and Reconstructive
2010-04-05
Presenter: S. Alex Rottgers MD
Affidavit:
Director Name:
Author Category: Resident/Fellow
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
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 primary pharyngeal flap (PPF) is often suggested. Our center utilizes the Furlow palatoplasty to treat SMCP in VCFS only when evidence of velopharyngeal (VP) mobility is present. This study investigates the success with this therapy.
Methods: A retrospective review of our cleft-craniofacial database with the diagnosis of VCFS was performed. Patients with VCFS and SMCP with preoperative evidence of VP mobility who underwent Furlow palatoplasty were reviewed. Speech was evaluated by a certified speech pathologist utilizing the Pittsburgh Weighted Speech Score (PWSS).
Results: 26 patients were identified as having VCFS, SMCP, and VP mobility. Of these, 25 (96%) were treated with a Furlow palatoplasy. 23 had a documented post-op PWSS. Post-operatively, 3 (13%) developed VP competence (PWSS=0). 9 (39%) obtained borderline VP incompetence and required no further surgery (Average PWSS=5). 11 (48%) had persistent VPI (Average PWSS=18). 15 patients had preoperative videofluoroscopy. Those with and without adequate speech outcomes exhibited a similar degree of lateral wall motion (35 vs 45%).
Conclusion: When treating VCFS related SMCP in appropriately selected patients, Furlow palatopasty is successful in 52% of cases, negating the need for pharyngoplasty with its life-long morbidity. At present, videofluoroscopy cannot preoperatively identify those patients who will be adequately treated with palatoplasty alone. Patients should be counseled about the potential need to reoperation.
