Syndromic and Nonsyndromic Systemic Associations of Congenital Lacrimal Drainage Anomalies: A Major Review

Purpose: To review and summarize the syndromic, nonsyndromic, and systemic associations of congenital lacrimal drainage anomalies. Methods: The authors performed a PubMed search of all articles published in English on congenital lacrimal anomalies (1933–2016). Patients of these articles were reviewed along with the literature of direct references to syndromes and other systemic associations. Data reviewed included syndromic descriptions, systemic details, demographics, lacrimal presentations, management, and outcomes. Results: Syndromic and systemic associations with congenital lacrimal drainage disorders are not known to be common. Although familial presentations have been reported, the inheritance patterns are unclear for most anomalies. There is an increasingly growing evidence of a genetic basis to many lacrimal anomalies. However, few syndromes have either widespread or are frequently associated with lacrimal anomalies. Few sequences of distinct lacrimal presentations and intraoperative findings are seen. Surgical challenges in these patients are distinct and a thorough pre and intraoperative anatomical assessment, detailed imaging when indicated, and assessment and correction of associated periocular and facial abnormalities may facilitate good outcomes. Conclusions: Lacrimal drainage anomalies associated with syndromic and nonsyndromic systemic conditions have certain unique features of their own and their surgical and anesthesia challenges are distinct. Diagnosis of congenital lacrimal drainage anomalies should prompt consideration of the possible presence of associated systemic abnormalities.