Management of choanal atresia in CHARGE association patients: a retrospective review

RATIONALE: To examine the management and outcomes of choanal atresia in children with CHARGE association compared with non-CHARGE children. METHODS: This is a retrospective chart review at a tertiary care children's hospital examining the management and outcomes of choanal atresia repair in children with CHARGE from 1990-2005. At least two CHARGE features were used to define these children. Children with CHARGE association were compared with non-CHARGE children regarding type of atresia, method of repair, post-operative management, re-stenosis rate and revision surgery. RESULTS: Fifty-seven children (36 female, 21 male) with complete records were identified with 24% diagnosed with CHARGE association. The median age of primary repair was 0.75 months for CHARGE and 15 months for non-CHARGE children (p=0.047). Unilateral atresia was diagnosed in 36% of CHARGE children and 81% of non-CHARGE children. Bilateral atresia was diagnosed in 64% of CHARGE and 19% of non-CHARGE children (p=0.006). The median duration of stent placement was slightly longer in the CHARGE group with no difference in the median number of post-operative dilations. Of those with CHARGE, 36% required revision surgery compared to 44% of the non-CHARGE subjects (p=0.58). Of those undergoing primary transnasal repairs, 40% of the CHARGE and 37% of the non-CHARGE patients failed. 50% of CHARGE patients with primary transnasal repair for bilateral atresia failed. Only 25% of transpalatal CHARGE repairs failed, while 75% of transpalatal repairs failed in the non-CHARGE group. The median follow-up was 24.6 months for CHARGE and 24.9 months for non-CHARGE patients. CONCLUSIONS: Children with CHARGE and unilateral choanal atresia can be managed successfully with a transnasal approach. However, our data, as well as prior published reports, suggests that CHARGE patients with bilateral atresia should have primary transpalatal repairs due to the high failure rate with the transnasal approach in this population.