Several recent cases demonstrate how advancements in planning, imaging and surgical approaches can enable positive outcomes in very young patients.
Reviewed by Dr Janet L. Alexander.
Several innovations are revolutionising surgical care in paediatric ophthalmology, according to Dr Janet L. Alexander, an assistant professor of ophthalmology and paediatrics at the University of Maryland School of Medicine in Baltimore, Maryland, United States.
Speaking at the Women in Ophthalmology 2021 Summer Symposium, Dr Alexander presented a series of rapid-fire cases to discuss four such paediatric innovations: antenatal surgical planning to address congenital hereditary disease, endothelial keratoplasty, endocyclophotocoagulation (ECP) to treat refractory glaucoma and ultrasound biomicroscopy (UBM) as a tool for guiding complex cataract surgery.
One case involving a child born with Norrie disease highlighted the value of antenatal surgical planning to enable intervention prior to complications. A 30-year-old woman in her second trimester of pregnancy presented with carrier status for NDP mutation. Amniocentesis confirmed foetal NDP mutation, and the ultrasound showed no foetal retinal detachment. Labour was induced at 34 weeks’ gestation for early retinal treatment.
As well as having Norrie disease, the baby was diagnosed with bilateral vitreous haemorrhages at birth, and underwent bilateral treatment with intravitreal anti-vascular endothelial growth factor therapy and peripheral retinal laser. Six months later, both eyes demonstrated robust light perception vision with an attached retina.
Dr Alexander noted that the case was a reminder that it is never too early to start antenatal surgical planning. “Prenatal surgical planning requires proactive and timely genetic testing to identify surgical candidates, counselling for female carriers of high-risk genetic traits, and involvement of geneticists and genetic counsellors,” she said.
Dr Alexander discussed the benefits and drawbacks of descemet stripping automated endothelial keratoplasty (DSAEK) versus penetrating keratoplasty (PK) with reference to a case involving a 4-year-old child who presented with a cloudy cornea 2 years after undergoing cataract surgery. The child received a diagnosis of bullous keratopathy and underwent DSAEK.
“Compared to PK, DSAEK is associated with stronger wound integrity, less astigmatism and faster visual recovery. It also avoids the need for stripping of the host endothelium in paediatric eyes,” Dr Alexander said. “However, DSAEK graft positioning can be very difficult in paediatric patients, and complications such as graft folds; detachment and dislocation; pupillary block; endothelial immune rejection; and steroid response can occur.”
There are advantages to choosing ECP over trans-scleral ciliary ablation to treat refractory glaucoma in paediatric patients, according to Dr Alexander. She presented a case involving a 7-year-old child who had sustained a traumatic cataract, then presented with 20/250 vision and elevated intraocular pressure (IOP) despite maximal medical treatment, selective laser trabeculoplasty and goniotomy.
The most remarkable feature of ECP is that it is minimally invasive and has a more favourable safety profile compared with the trans-scleral technique. “For example, phthisis in the absence of retinal detachment occurs in over 30% of eyes treated trans-sclerally but in less than 1% of eyes that undergo ECP,” Dr Alexander said. Other advantages of ECP include avoidance of late infectious complications, such as wound leak and endophthalmitis.
However, ECP has a modest success rate and its benefit is somewhat short-lived. Outcomes data show that it effectively reduces IOP in about 30% of eyes, and about half of those eyes maintain IOP control for 1–2 years. In addition, ECP can lead to the serious complications of hypotony, retinal detachment and vision loss.
According to Dr Alexander, preoperative evaluation with UBM enables cataract surgery planning and helps to prevent intraoperative surprises. Dr Alexander demonstrated these points with several case examples.
In one case, a 1-month-old child presented with bilateral cataract, but cataract surgery was postponed for 3 months whilst the child underwent treatment for comorbid heart failure. Repeat imaging prior to the scheduled cataract surgery showed that crystalline lens thickness had decreased dramatically over time.
Dr Alexander said that knowledge of the absorbed cataract had guided appropriate surgical techniques. “Cataract surgery planning with UBM allows surgeons to evaluate IOL position, the lens periphery and the ciliary body,” she said. “Importantly, it allows us to avoid surprises, including absorbed cataract, foreign bodies, masses, cysts or membranes.”
Other cases involving uveitic cataracts showed how imaging with UBM can document pathology that might complicate cataract surgery and can inform the surgical approach. Dr Alexander presented a series of eyes in which UBM revealed the presence of a cyclitic membrane, pupil synechiae and calcified plaque on the anterior capsule.
Dr Alexander concluded by observing that quantitative UBM is an innovation that further enhances the clinical utility of this procedure. Quantitative UBM uses algorithms to analyse the images and generates information that helps clinicians interpret the images. The algorithms are also capable of suggesting clinical diagnoses and prognostic features, such as the risk of complications.