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Increased fistula risk following palatoplasty in Treacher Collins syndrome
Stephen Bresnick1, John Walker, Noreen Clarke-Sheehan
1Division of Plastic Surgery, Childrens Hospital Los Angeles Cleft and Craniofacial Center, University of Southern California Keck School of Medicine, Los Angeles, CA, USA. sdbresnick@aol.com
Insights
Children with Treacher Collins syndrome have a significantly higher risk of palatal fistula after cleft palate repair. This suggests potential vascular abnormalities affecting healing in these patients.
Area of Science:
- Craniofacial Surgery
- Pediatric Plastic Surgery
- Medical Genetics
Background:
- Patients with Treacher Collins syndrome often exhibit abnormal palatal vascularity.
- The impact of this vascular anomaly on palatoplasty outcomes, specifically fistula formation, remains unclear.
Purpose of the Study:
- To investigate the correlation between Treacher Collins syndrome and the incidence of postoperative palatal fistula following palatoplasty.
- To compare fistula rates among patients with Treacher Collins syndrome, other craniofacial syndromes, and non-syndromic cleft palate.
Main Methods:
- A retrospective chart review of children undergoing palatoplasty between 1987 and 2000.
- Comparison of palatal fistula rates using chi-square analysis across three groups: Treacher Collins syndrome, other syndromes, and non-syndromic cleft palate.
- Evaluation included intraoperative anatomy and postoperative follow-up.
Main Results:
- Children with Treacher Collins syndrome experienced significantly higher palatal fistula rates (50%) compared to those with other syndromes (8.7%) or no syndrome (4.1%).
- Observed large fistulas and poor flap vascularity in Treacher Collins patients.
- The double-reversing Z-plasty technique was used for all repairs.
Conclusions:
- Treacher Collins syndrome is associated with a substantially increased risk of palatal fistula after cleft palate repair.
- Poor vascularity of palatal flaps in Treacher Collins patients likely contributes to higher fistula rates.
- Recommendations include minimizing flap dissection and employing closure techniques that preserve vascularity during palatoplasty.
Objective:
Patients with Treacher Collins syndrome have abnormal vascular supply to the palate, yet it is unknown whether there are increased postoperative healing problems following palatoplasty. This study investigated the correlation between Treacher Collins syndrome and postoperative palatal fistula formation.
Design:
Retrospective chart review was performed.
Patients:
Children undergoing palatoplasty at Children's Hospital Los Angeles from 1987 to 2000 were evaluated. Ten children with Treacher Collins syndrome, 92 children with other syndromes and cleft palate, and 458 nonsyndromic patients with isolated cleft palate were studied.
Interventions:
All children were treated with a one-stage, double-reversing Z-plasty cleft palate repair.
Main Outcome Measures:
Outcome measures included intraoperative observations of surgical anatomy and postoperative clinic follow-up of fistula formation. Palatal fistula rates between patients with Treacher Collins syndrome, other syndromes, and no syndrome were compared with chi-square analysis.
Results:
Children with Treacher Collins syndrome had significantly greater palatal fistula rates (50%) than children with other syndromes (8.7%) or no syndrome (4.1%). Treacher Collins patients demonstrated large palatal fistulas and poor flap vascularity.
Conclusions:
Children with Treacher Collins syndrome and cleft palate have significantly higher palatal fistula risk than other children with cleft palate when double-reversing Z-plasty palate repair is performed. Our findings suggest that children with Treacher Collins syndrome and cleft palate may have poor vascularity to palatal flaps created during palatoplasty. Furthermore, we recommend that surgeons performing palatoplasty minimize the dissection of mucoperiosteal flaps around the greater palatine arterial pedicle and utilize closure techniques creating the least vascular disruption of palatal tissue.