Outcomes of primary powered endoscopic dacryocystorhinostomy in syndromic congenital nasolacrimal duct obstruction

Purpose: To evaluate the outcomes of powered endoscopic dacryocystorhinostomy (DCR) in syndromic individuals with congenital nasolacrimal duct obstruction (CNLDO). Methods: A retrospective multicenter case series of patients who required a primary powered endoscopic DCR for refractory CNLDO associated with syndromes or congenital craniofacial abnormalities was performed. A minimum follow-up of more than 6 months was considered for final analysis. Main outcome measures were anatomical and functional success at the last follow up. Results: Twenty primary powered endoscopic DCR's were performed in 12 patients (7 males, 5 females) with syndromic anomalies. The mean age at the time of surgery was 7.6 years (range: 2-22 years). The associated systemic anomalies were Down syndrome (42%, 5/12), ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome (17%, 2/12), gross craniofacial dysgenesis (25%, 3/12) Tessier cleft type 3 (8%, 1/12) and velocardiacfacial syndrome (8%, 1/12). Proximal system agenesis of the upper or lower canaliculus was present in 42% of patients (5 patients, 9 eyes). Adjunctive treatment in the form of Mitomycin C and/or intubation was performed in 55%(11/20) eyes. At a mean postoperative follow-up of 20 months, successful outcomes were noted in 95% of the eyes (19/20). The one eye with an anatomical failure was secondary to a complete cicatricial closure of the ostium and was successfully treated subsequently with a revision endoscopic DCR. Conclusion: Primary powered endoscopic DCR has good outcomes in patients with a CNLDO associated with craniofacial syndromes.