Issue: May 25, 2020
Source/Disclosures
Disclosures: Devgan reports he owns CataractCoach.com, which is a free teaching website.
May 19, 2020
3 min read
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Leukocoria in newborn requires immediate attention

Diagnosis and treatment must occur in a timely manner in order to limit amblyopia and give the best chance for visual recovery.

Issue: May 25, 2020
Source/Disclosures
Disclosures: Devgan reports he owns CataractCoach.com, which is a free teaching website.
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Leukocoria simply means that the pupil (coria) is white (leuko), and we see this in our adult patients who present with opaque white cataracts. But when we see leukocoria in a newborn infant, it is an urgent situation that needs immediate attention from an ophthalmologist.

Uday Devgan
Uday Devgan

The cause of the leukocoria can be retinoblastoma, congenital cataract, Coats disease, Norrie disease, persistent hyperplastic primary vitreous or another issue. Retinoblastoma is a deadly ocular cancer that must be treated immediately. Even for non-life-threatening causes of leukocoria, the diagnosis must be made in a timely manner and treatment given early in life in order to limit amblyopia and give the best hopes for visual recovery.

Infants are screened by pediatricians shortly after birth, typically using a direct ophthalmoscope to ensure a good red reflex from each eye. In this case, the infant had a red reflex in each eye, but there was asymmetry with one eye having a much stronger and larger red reflex than the other. The infant was referred to our eye clinic and examined by both the pediatric ophthalmologist and the vitreoretinal specialist. They determined that there was a membrane blocking most of the pupil and precluding a view of the posterior segment (Figure 1). The patient was scheduled for an examination under anesthesia and treatment for the underlying condition.

leukocoria
Figure 1. This infant was diagnosed with leukocoria, which required surgical intervention. Close examination showed a membrane covering much of the pupil. Intraoperative OCT helped with determining the extent of the lesion.
Source: Uday Devgan, MD

OCT testing using the operating microscope showed that the leukocoria was due to a pupillary membrane that blocked the visual axis (Figure 2). Fortunately, the crystalline lens was clear and without cataract development. Two small incisions, less than 1 mm wide, were made at the limbus with care taken to nick the limbal vessels and provide an adequate tunnel length to ensure good long-term healing and stability. These incisions were made 90° apart, which also helps minimize any potential astigmatic effects.

fibrotic membrane covering most of the pupillary aperture
Figure 2. The OCT scan showed that the crystalline lens was clear and that there was a distinct fibrotic membrane covering most of the pupillary aperture.

Small-gauge scissors and forceps, originally designed for vitreoretinal surgery, were used to carefully cut and remove the fibrotic membrane (Figure 3). Small aliquots of viscoelastic were used to elevate the membrane above the anterior lens capsule. This provided a margin of safety so that the crystalline lens would not be touched. Additional viscoelastic was used to fill and maintain the anterior chamber. OCT confirmed removal of the membrane and showed no evidence of cataract formation (Figure 4).

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cut off the membrane
Figure 3. Small-gauge scissors were used to carefully cut off the membrane without touching the underlying crystalline lens.

Using 25-gauge vitrectomy instrumentation, the viscoelastic was aspirated from the eye. The phaco machine was used in the irrigation/aspiration mode because we did not need to use the vitrector cutter. The infusion inflow was kept sufficiently high to maintain a deep anterior chamber during aspiration. The limitation to inflow was the small lumen size of the instruments, so we lowered the aspiration outflow rate to stay in the safe zone.

pupil and visual axis were clear
Figure 4. After the surgery, the pupil and visual axis were clear, and there was no evidence of cataractous changes in the crystalline lens. The remainder of the eye, including the posterior segment, was normal.

Once the membrane was surgically excised, the pupil was sufficiently large to allow a detailed examination of the posterior segment, which was normal. Both eyes underwent full examination under anesthesia by our pediatric ophthalmologist. The patient has done well after the surgery and is developing normally, reaching the normal vision milestones with both eyes.

While this type of case is relatively rare, it is something that every ophthalmologist will encounter over the course of their career. We are fortunate to have a multispecialty team of ophthalmologists at our academic county hospital to handle tough cases such as this. For this one infant, we had a pediatric ophthalmologist, a vitreoretinal specialist and a cataract surgeon working together. These are our youngest and most vulnerable patients, and our surgical interventions will benefit them for many decades to come.

Surgical video of this case is available at CataractCoach.com.

Disclosure: Devgan reports he owns CataractCoach.com, which is a free teaching website.