Congenital microphthalmia and anophthalmia Biometrics and treatment using three-dimensional techniques

Congenital microphthalmia and anophthalmia (MICA) are rare developmental anomalies characterized by the presence of a small or absent eye, respectively. In the Netherlands, approximately 4 to 17 children are born with this condition annually. In many cases, the visual prognosis is poor. Treatment then focuses on minimizing facial asymmetry as much as possible and ensuring the ability to wear a well-fitting and aesthetically pleasing ocular prosthesis.This dissertation focuses on practical questions regarding the management of MICA: when is this treatment indicated? How urgently should it be initiated? What does the treatment process entail? What constitutes the most appropriate outcome measure for evaluating success? Additionally, the potential applications of 3D-printed ocular prostheses are explored.
To address these questions, the MICA cohort at Amsterdam UMC was studied from 2013 to 2022. This research proposes a classification system based on axial length measurements obtained via ultrasonography. Axial length is expressed as a percentage relative to the unaffected (or least affected) eye: 0–45% indicates severe microphthalmia, 45–75% moderate microphthalmia, and 75–100% mild microphthalmia. Children with eyes smaller than 10.5 mm ideally require treatment within the first few months of life, as such cases are more frequently associated with orbital growth retardation and an increased risk of facial asymmetry.
Our findings, combining objective measurements with subjective assessments, suggest that the primary treatment goal should focus on achieving horizontal palpebral fissure (HPF) symmetry exceeding 80% relative to the unaffected (or least affected) side. Personalized, 3D-printed conformer therapy often achieves orbital dimensions exceeding 80% of the unaffected side, promotes the formation of adequate conjunctival fornices, and facilitates the use of aesthetically pleasing ocular prostheses.