Published in Retina
5 Pediatric Retina Surgery Pearls You Should Know
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Pediatric retinal disease encompasses a variety of diverse and rare diseases that non-pediatric ophthalmologists seldom encounter. Follow these five pearls for success in pediatric vitreoretinal surgery.
Many of the common retinal diseases we discuss affect adults, and most of us are well-versed in these adult conditions (e.g., age-related macular degeneration, diabetic retinopathy, rhegmatogenous retinal detachment). However, pediatric retinal disease encompasses a variety of diverse and rare diseases that non-pediatric ophthalmologists seldom encounter. Here we present five pearls for success in pediatric vitreoretinal surgery.
Children needing retina surgery almost always have either suffered ocular trauma or have an underlying ocular or systemic disease. In children—especially toddlers but even teenagers—it can be difficult to elicit a history of trauma. Kids often forget or deny being hit in the eye, but occult trauma is often the cause. Sometimes the ocular disease (e.g., retinal detachment) manifests years after the trauma.
If there is an underlying disease, it can often be genetic (e.g., Stickler’s syndrome). Sometimes the retinal disease is related to acquired factors such as prematurity (e.g., retinopathy of prematurity), uveitis (e.g., toxocariasis, pars planitis), or other developmental conditions (e.g., persistent fetal vasculature, Coats’ disease). Above all, there are two “never miss” pediatric diagnoses: retinoblastoma and non-accidental trauma (NAT). Both can have devastating ocular sequelae and systemic manifestations, including death; consequently, these cannot be missed!
It is easy as a pediatric retina surgeon to focus on treating retinal disease but difficult to manage refractive error and amblyopia. Having close colleagues who are experts in pediatric ophthalmology and optometry is essential. Many affected children need repeated cycloplegic refractions, intensive amblyopia therapy, and aphakic contact lens fittings. Co-managing these challenging patients leads to better outcomes.
Unfortunately, from personal observations and existing literature, scleral buckling is becoming a lost art, with many adult retina surgeons rarely or never using scleral buckles (SBs).1 Regarding pediatric retina, vitreoretinal surgeons must be facile in SB placement and management.
Most pediatric rhegmatogenous retinal detachments can and should be treated with SBs. Primary SBs are usually preferred over pars plana vitrectomy (PPV) because cataract in children has serious ramifications, the posterior hyaloid in children is usually tightly attached, children have trouble with post-operative positioning, and proliferative vitreoretinopathy (PVR) after PPV in children can be relentless.
SBs are also effective for many pediatric tractional RDs (TRDs), such as certain Stage 4 ROP TRDs, and almost all TRDs caused by familial exudative vitreoretinopathy (FEVR), pars planitis, and toxocariasis. SB surgery can be combined with laser photocoagulation or cryopexy, especially when there is prominent peripheral traction from neovascularization that could contract after laser retinopexy and worsen retinal detachments.
Sometimes vitrectomy is necessary for children. Examples include dense and non-clearing vitreous hemorrhage, certain ROP TRDs, giant retinal tears in Stickler’s syndrome, and traumatic full-thickness macular holes that do not close spontaneously.
Vitrectomy in children, especially babies and infants, differs from adult vitreoretinal surgery. General anesthesia is mandatory. Additionally, the very young pediatric eye is much smaller, and the lens comparatively larger. In young kids, trocar insertion is dangerous because the sclera is thin and rubbery, and the optimal position varies by age. Too posterior insertion can cause an iatrogenic retinal break, which in an infant usually dooms an eye to blindness. Too anterior insertion can compromise the lens, rendering a child aphakic.
When the posterior segment anatomy is complex or unknown, often the safest approach is limbal vitrectomy. The instruments are placed through the peripheral cornea, and vitrectomy proceeds anteroposteriorly. First, any anterior chamber opacities or membranes are addressed. The lens and capsule are removed. Vitrectomy and membrane peeling is then performed in the anterior vitreous cavity. It is sometimes safe to transition to a pars plicata (or pars plana) approach to complete the vitrectomy.
The vitreous in children is thick in its native state, making posterior vitreous detachment (PVD) induction difficult. Sometimes a PVD can be propagated to the arcades but no further without inducing retinal breaks. Special techniques for PVD induction are often necessary. In some situations, such as ROP surgery, there is no need to induce a PVD.
Certain techniques used sparingly in adults, such as triamcinolone staining of the vitreous, or external needle drainage of subretinal fluid (SRF) during vitrectomy, are often appropriate in children. Conversely, iatrogenic retinotomy formation for SRF drainage, commonly performed in adults, should be avoided in children because retinotomies can be a nidus for PVR. Draining from a peripheral break, with or without perfluorocarbon liquids, is preferred.
Children with complex retinal detachments treated with PPV often benefit from ancillary SBs, which support areas of peripheral traction where the hyaloid could not be removed or where PVR may develop. Relaxing retinectomies should be performed only as a last resort. Finally, because most children cannot position postoperatively, silicone oil tamponade is preferred as an endotamponade agent.
Most adult retina surgeries are “one and done,” but kids with retinal disease often require multiple surgeries and dozens of office visits over the years. Therefore, pediatric retina surgeons need to be optimistic and determined. In my experience, the surgeries are technically challenging and often have poor outcomes.
Amblyopia is the rule, and glaucoma and corneal complications are also common. However, children are amazingly adaptable, so maximizing even a small amount of vision can change a child’s life for the better. That is why we keep fighting.
This young child with Stickler’s syndrome had near-total rhegmatogenous retinal detachment from a posterior retinal tear associated with multifocal radial lattice degeneration, and dense, non-clearing vitreous hemorrhage.
This video illustrates principles of complex pediatric vitrectomy surgery, including the use of ancillary SB, vitreous staining with triamcinolone, inability to completely propagate PVD despite special techniques, use of perfluorocarbon for subretinal fluid drainage, and silicone oil tamponade because of inability to position. The oil was later removed, and the retina remained attached with excellent VA.
Krader CG, Berrocal M.”Survey: More retinal surgeons using primary vitrectomy, microincisional technology.” Ophthalmology Times. 2015, March. https://www.ophthalmologytimes.com/view/survey-more-retinal-surgeons-using-primary-vitrectomy-microincisional-technology