Customize your JAMA Network experience by selecting one or more topics from the list below.
Endogenous intraocular Candida infection typicallypresents as chorioretinitis with varying degrees of vitreous infiltrationand inflammation. In patients with concurrent or recent candidemia, intralenticularfungal abscess is rare but has been reported previously in premature infants.1-3 We report afourth and fifth case of this unusual syndrome, emphasizing its clinical signs,peculiar clinical course, and the possibility of a good visual outcome withappropriate therapy.
Report of Cases
Case 1. An infant girl was born at 24 weekspostconceptual age and weighed 750 g. The postnatal course was complicatedby bronchopulmonary dysplasia, apnea, patent ductus arteriosis, anemia, hypertension,gastrointestinal reflux, and subclinical necrotizing enterocolitis. The patientalso developed candidemia with blood and urine cultures positive for organismsat 3 weeks of age. She was found to have a large right atrial mass compatiblewith fungus that showed evidence of inferior vena cava obstruction. She wastreated with amphotericin B and fluconazole for a total of 3 months. All cultureswere negative for organisms for the final 2 weeks of her treatment prior todischarge from the neonatal intensive care unit. Serial ocular examinationsrevealed intralenticular opacities and a persistent tunica vasculosa lentisin the right eye by 3 weeks of age, which resolved itself by age 5 weeks.The left eye was not involved. At 3 months of age she was noted to have achorioretinal scar in the right eye. At age 4 months, her mother observeda "white spot" at the edge of her pupil.
One month later, she was examined at our institution with a 3-day historyof a red eye. It was noted that there was a mass in the pupil and beneaththe iris inferotemporally, the cornea was cloudy, and there were dilated irisvessels as well as iris bombe. B-scan ultrasonography revealed no increasein vitreous opacities compared with the fellow eye. Examination results ofthe left eye were unremarkable except for 3 clock hours of stage 2, zone 3retinopathy of prematurity. A fungal etiology was suspected and blood andurine cultures were obtained. The right eye was treated with prednisoloneacetate 1% and atropine. After 1 week, the anterior segment inflammation wasclearing with contraction of the anterior chamber mass. There was iris vesseldilation at the 9-o'clock position with a focus of white material at the pupillaryborder and underneath the iris. There were mildly increased vitreous opacitiesdetected by ultrasonography.
An examination under anesthesia was performed (Figure 1). Intraoperatively, after performing an anterior chamberwashout and cultures, the white mass was more clearly visualized. It was locatedat the temporal pupillary border, extending underneath the pupil and pushingthe iris forward. The lens was opacified beneath this mass. An extracapsularcataract extraction was performed with aspiration of creamy-white materialfrom beneath the iris and generous capsulectomy, anterior vitrectomy, andintravitreal injection of amphotericin B. Intraocular cultures grew Candida albicans (Figure2). She received a 6-week course of amphotericin B because of previouscardiac problems. Her postoperative ocular course was uneventful. She wassuccessfully treated with a Silsoft pediatric aphakic contact lens (Bausch& Lomb, Rochester, NY) and bifocal lenses, as well as part-time occlusion.The 2 small perimacular chorioretinal scars remained stable. Esotropia andright hypertropia were treated with eye muscle surgery. On the most recentfollow-up at 31/2 years old, the patient's best-correctedvision in the right eye measured 5/200; her eyes were straight (Figure 3) and fundus unchanged.
Case 1 had inflamed eye with awhite mass beneath the pupil.
The intraocular cultures in case1 grew Candida albicans.
After aggressive treatment, case1 developed good visual function and cosmesis.
Case 2. A boy, born at 25 weeks postconceptionalage and weighing 855 g, had a postnatal course complicated by respiratorydistress syndrome, pneumonia, anemia, patent ductus arteriosus, and hypotension.He developed candidemia and coagulase-negative staphylococcal sepsis thatwere treated with amphotericin and vancomycin, respectively, at an outsidehospital.
All cultures were negative for organisms after transfer to our facilityat 38 days of age. Serial dilated fundus examinations were performed from7 weeks to 3 months of age, when threshold retinopathy of prematurity wasdiagnosed. Indirect laser photocoagulation of avascular peripheral retinausing approximately 1300 green diode laser spots was performed bilaterally.Regression of the retinopathy was noted at the first postoperative examination1 week later. During this examination a 1-mm, discrete, round, anterior lensopacity with an associated iridolenticular adhesion was noted superiorly inthe left eye and felt to be consistent with a laser burn. Follow-up examinationsthrough 6 months of age confirmed resolution of the retinopathy with no changein the lenticular opacity.
At 7 months of age the patient developed conjunctival injection in hisleft eye. Examination showed moderate corneal haze, iris bombe, miosis, andthe sectoral cataract superiorly. The intraocular pressure was 46 mm Hg andwas unresponsive to medical management. A trabeculotomy with iridectomy andlysis of posterior synechiae with removal of an anterior chamber membranewas performed. A red reflex was noted inferiorly through the miotic pupil.During the procedure, a thin, milky-white fluid flowed from the iridectomyand out through the limbal incision. A Candida abscesswas suspected and the white material was cultured.
The intraocular pressure normalized postoperatively but the culturesgrew C albicans on the second postoperative day.The patient was taken back to the operating room and examination after placementof iris hooks revealed a creamy-white lenticular opacity underlying the previousfocal cataract. A cataract extraction with anterior vitrectomy and intravitrealinjection of amphotericin B was performed. An infectious workup revealed anidus in the patient's right kidney, consistent with a Candida abscess, that resolved itself during a 28-day course of systemicfluconazole. The postoperative course was uneventful with treatment consistingof a Silsoft aphakic contact lens and part-time occlusion. He had a strongright fixation preference but was able to fix and follow with his left eyeon his most recent follow-up examination at 13 months of age.
To our knowledge these are the fourth and fifth reported cases of a Candida intralenticular abscess with a white plaque inthe pupil after treated candidemia in a premature, low-birth-weight infant.Similar to a previous case,2 our patientsresponded well to aggressive treatment that included cataract extraction,anterior vitrectomy, and intravitreal injection of amphotericin B. Case 1is unique in that intralenticular opacities were noted in the affected eyeat the time of initial candidemia. The opacities resolved with antifungaltreatment. Of interest is the fact that all reported cases of endogenous intralenticularfungal abscess have occurred in premature infants. We believe that in sucheyes, the persistent tunica vasculosa lentis serves as the route for lenticularseeding by fungal organisms at the time of Candida septicemia.As the tunica regresses, Candida organisms becomesequestered adjacent to and within the lens, resulting in inadequate treatmentby systemic antifungal therapy. The lenticular abscess then slowly activates,appearing months later as a focal lens opacity accompanied at first by relativelylittle intraocular inflammation. Case 1 represents the first infant treatedaggressively who developed good visual function and cosmesis. Case 2 is thefirst case to present as glaucoma.
Our cases and review of the literature suggest that premature neonateswho have been successfully treated for systemic candidiasis remain at riskfor developing sequestration of fungal organisms within the lens, which laterappears as a focal lens opacity that evolves into a white papillary plaqueup to 6 months following treatment. Close ophthalmologic follow-up and parentaleducation regarding this presentation is recommended for this group of patients.As demonstrated by these cases, a high index of suspicion and aggressive therapycan result in an excellent clinical outcome.
The authors have no relevant financial interest in this article.
Correspondence: Dr Singh-Parikshak, Simpson Eye Associates, 650 SpringhillRing Rd, West Dundee, IL 60118 (firstname.lastname@example.org).
Singh-Parikshak R, Bothun ED, Superstein R, Del Monte M, Archer S, Johnson MW. Sequestration and Late Activation of Lenticular Candida Abscess in Premature Infants. Arch Ophthalmol. 2004;122(9):1393–1395. doi:10.1001/archopht.122.9.1393
Artificial Intelligence Resource Center