RESUMEN
BACKGROUND: Van der Woude syndrome (VWS) is a rare congenital malformation characterized by lower lip pits among patients with a lip and/or palate cleft. It is transmitted by an autosomal dominant inheritance with variable expressivity. METHODS: The study group consisted of 24 consecutive patients (13 males and 11 females) with VWS operated on at a single center between 2009 and 2022. They suffered from: bilateral cleft lip and palate - 6 patients; unilateral cleft lip and palate - 9 patients; cleft lip - 1 patient; and isolated cleft palate - 8 patients. RESULTS: In 16 (66%) cases pits of lower lip occurred on both side of midline, while in 8 (34%) the pits were detected unilaterally. The primary cleft repairs were performed according to one-stage principle at the mean age of 8.6 months (SD 1.4, range 6-12). In all patients lower lip pits repairs were performed after the primary cleft repairs as a separate procedure at the mean age of 37 months (SD 11.3 range 14-85). The mean number of all primary repairs of the syndrome-both cleft defect and lower lip pits repairs-was 2.46. Nine patients (37.5%) required additional secondary corrections of the lower lip due to the poor aesthetic post-operative outcome. CONCLUSIONS: The frequent need for secondary corrections of residual lower lip deformities indicates the considerable difficulties in obtaining a satisfactory outcome of the repairs to lip pits caused by VWS. The average number of the primary surgical interventions in evaluated material remained low.
Asunto(s)
Anomalías Múltiples , Labio Leporino , Fisura del Paladar , Labio , Humanos , Labio Leporino/cirugía , Femenino , Fisura del Paladar/cirugía , Masculino , Estudios Retrospectivos , Labio/anomalías , Labio/cirugía , Anomalías Múltiples/cirugía , Preescolar , Lactante , Niño , Resultado del Tratamiento , Procedimientos de Cirugía Plástica/métodos , Quistes/cirugíaRESUMEN
BACKGROUND: Presurgical plate therapy has been widely accepted as a treatment prior to palatal cleft closure. The effects of passive presurgical plate therapy on cleft morphology prior to single-stage unilateral cleft lip and palate (UCLP) repair were quantified. PATIENTS AND METHODS: We compared the dimensions of cleft width and cleft area (true cleft and palatal cleft) measured preoperatively at 2 European cleft centers. Center A performed single-stage UCLP repair in 8-month-old infants without any presurgical orthopedic treatment. Center B initiated passive presurgical plate therapy immediately after the birth of the neonates, followed by single-stage UCLP repair at 8 months of age. RESULTS: We included 28 patients with complete UCLP from Center A and 12 patients from Center B. The average anterior width of the true cleft before surgery was significantly smaller in infants at Center B than that in Center A (p = 0.001) with 95% confidence interval of (1.8, 5.7) mm, but the average posterior width was similar in the 2 groups. The mean presurgical true cleft area amounted to 106.8 mm2 (SD = 42.4 mm2) at Center A and 71.9 mm2 (SD = 32.2 mm2) at Center B, with a confidence interval for the difference being (9.8, 60.1) mm2. This corresponded to a 32.7% reduction of the true cleft area when passive presurgical plate therapy was used for the first 8 months of the infants' life. CONCLUSION: Passive presurgical plate therapy in UCLP significantly reduced the cleft area. Implications for the subsequent surgical outcome might depend on the surgical technique used.