OPHTHALMIC AND SYSTEMIC SIGNS OF CONGENITAL ANIRIDIA
Náray Annamária
Surgical Medicine Division
Dr. Szijártó Attila
SE Szemészeti Klinika, Tanterem
2026-10-21 14:00:00
Ophthalmology
Dr. Nagy Zoltán Zsolt
Dr. Szentmáry Nóra
Dr. Papp András
Dr. Kerényi Ágnes
Dr. Krenács Tibor
Dr. Pluzsik Milán
Dr. Szamosi Anna
Congenital aniridia is a complex, multisystemic ocular disorder characterized by a
constellation of ophthalmic manifestations, including AAK, secondary glaucoma, cataract,
and foveal and optic nerve hypoplasia. The prevention and management of these
complications require close collaboration among different ophthalmic subspecialties,
ensuring comprehensive and long- term patient care. Overall, individuals with congenital
aniridia often exhibit a range of systemic health issues, particularly hypertension, thyroid
disorder, metabolic disturbances, and increased body weight. The notable frequency of
previous surgical interventions and emotional instability reflects the broader systemic and
psychological burden associated with the condition. These observations highlight the
importance of a comprehensive, interdisciplinary care strategy, ensuring regular systemic
evaluation and mental health support in addition to ophthalmological follow-up.
For the grading of AAK, the Lagali classification system is recommended, as it allows
for straightforward clinical application and reliable documentation. The system reflects
disease progression accurately, thereby supporting clinicians in establishing prognosis and
selecting optimal therapeutic strategies. Patients with AAK should ideally be monitored and
treated in specialized referral centers with expertise in aniridia and ocular surface disease.
Our findings further suggest that previous cataract surgery in congenital aniridia may
contribute to the progression of AAK and an increased incidence of secondary glaucoma,
particularly in pseudophakic and aphakic eyes. Additionally, lens subluxation appears to
predispose patients to glaucoma development. These observations highlight the importance of
careful surgical planning and individualized risk assessment before undertaking intraocular
procedures in this vulnerable patient population.
Future research aimed at elucidating the molecular mechanisms underlying
postoperative exacerbation of keratopathy and glaucoma may uncover novel therapeutic
targets, ultimately improving long-term outcomes for individuals affected by congenital
aniridia.
