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Year : 2016  |  Volume : 4  |  Issue : 2  |  Page : 100-102

Coexistent retinal vasculitis and aggressive posterior retinopathy of prematurity in a preterm infant

1 Department of Retina and ROP Services, H. V. Desai Eye Hospital, Pune, India
2 Byramjee Jeejeebhoy Medical College and Sassoon General Hospital, Pune, Maharashtra, India

Date of Submission04-Oct-2014
Date of Acceptance08-Jun-2015
Date of Web Publication9-Jun-2016

Correspondence Address:
Sucheta R Kulkarni
The Poona Blind Men's Association's H. V. Desai Eye Hospital, 93 Tarawade Vasti Mohammadwadi, Hadapsar, Pune-411 060, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-3897.183723

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We report a rare case with simultaneous occurrence of retinal vasculitis and aggressive posterior retinopathy of prematurity (APROP) in a preterm infant. During ROP screening at 5 weeks of age, RetCam imaging revealed early vascular shunts in zone I and plus disease diagnostic of APROP along with bilateral retinal vasculitis. APROP regressed with laser followed by intravitreal bevacizumab (IVB) in right eye and with laser only in left eye. Antifungal treatment was started (Fluconazole 6 mg/kg/day) on clinical suspicion of fungal sepsis with eventual resolution of retinal vasculitis. Retinal vasculitis can be an ocular sign of systemic fungal infection and can predispose an infant for development of retinopathy of prematurity (ROP). Simultaneous occurrence of both entities might pose a challenge in the management.

Keywords: Aggressive posterior retinopathy of prematurity, fungal sepsis, retinal vasculitis

How to cite this article:
Kulkarni SR, Labshetwar NA, Kakade NA, Chhaya VT. Coexistent retinal vasculitis and aggressive posterior retinopathy of prematurity in a preterm infant. J Clin Ophthalmol Res 2016;4:100-2

How to cite this URL:
Kulkarni SR, Labshetwar NA, Kakade NA, Chhaya VT. Coexistent retinal vasculitis and aggressive posterior retinopathy of prematurity in a preterm infant. J Clin Ophthalmol Res [serial online] 2016 [cited 2022 Dec 5];4:100-2. Available from: https://www.jcor.in/text.asp?2016/4/2/100/183723

Retinal vasculitis is a known complication of systemic infection inadults. This entity has been documented in a neonate [1] but natural course of the disease is unknown. Aggressive posterior retinopathy of prematurity (APROP) is characterized by vascular shunting in zone I or posterior zone II associated with plus disease. [2] Severe retinal ischemia with occluded vascular beds has been reported in APROP. [3] APROP needs an urgent, aggressive laser. intravitreal bevacizumab (IVB) is usually reserved for difficult cases (lasered nonregressing ROP or difficult laser due to small pupil, hazy media etc). [4] Both APROP and retinal vasculitis due to presumed fungal sepsis occurring together in a neonate have never been reported before.

  Case Report Top

A preterm infant with gestational age 32 weeks and birth weight 1448 grams was referred for retinopathy of prematurity (ROP) screening at 5 weeks of age in a tertiary care facility, earlier referral being deferred due to critical systemic status. The wide field digital imaging (Retcam shuttle, Clarity medical systems, USA) revealed plus disease with early shunting of retinal blood vessels in Zone I which was diagnostic of APROP. The periphery of the blood vessels showed white thread like appearance, no visible blood column and early perivascular exudates suggestive of retinal vasculitis [Figure 1]. However, systemic condition of the baby being very critical, laser treatment had to be deferred. The infant had respiratory distress syndrome requiring prolonged ventilatory support, hyperbilirubinemia, hypoglycemia, congenital heart disease, and anemia requiring blood transfusion.
Figure 1: Visit 1: Retinal vasculitis seen in temporal quadrants (white arrow), increased dilatation, tortuosity of blood vessels (black arrow), capillary looping in 1-2 quadrants. a: RE; b: LE

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Second screening examination within 2 days revealed nondilating pupils, neovascularization of iris and worsening of plus disease. Retinal vessels showed advanced perivascular exudation leading to semi opaque appearance of the posterior pole retina underneath inferotemporal arcade in right eye and peripapillary region in left eye [Figure 2]. Immediate laser treatment was instituted for both eyes.
Figure 2: Visit 2: Retinal vasculitis with perivascular exudation (blue arrow), semi-opaque retina in posterior pole, severe plus disease (black arrow) with capillary looping and retinal bleed. a: RE; b: LE

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Third examination after 3 days revealed more extensive perivascular exudates with opaque appearance of the retina suggestive of the worsening of the signs [Figure 3]. At this point of time (6 th week of life) the baby developed intolerance to feeds with abdominal distension suggestive of necrotizing enterocolitis (NEC) and was suspected to have late onset sepsis. Prolonged neonatal intensive care unit (NICU) stay of the already sick infant made us suspect fungal sepsis.
Figure 3: Visit 3: Plus increased (black arrow), bleed increased, laser marks seen (red arrow), posterior pole retina semi - opaque (white arrow). a: RE; b: LE

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Patient was investigated for TORCH titres, inborn errors of metabolism, galactosemia, disseminated intravascular coagulation, thrombocytopenia and blood culture for bacterial/fungal infection. All reports were negative. Fungal culture was negative even after 2 weeks of incubation.

With clinical suspicion of fungal sepsis, systemic antifungal (fluconazole 6 mg/kg/day) was started immediately within 24 hours (6 weeks of chronological age).

The infant subsequently needed IVB in right eye due to inability to add laser because of small pupils, whereas left eye was treated with two additional laser sessions. The infant was prescribed nonsteroidal antiinflammatory (Flurbiprofen 0.03%) and antibiotic (Tobramycin 0.3%) eye drops after treatment. Topical steroids were avoided due to suspected infective origin of the vasculitis.

At the longest follow-up of 11 weeks after completion of laser treatment and after 12 weeks of IVB (after which the infant was lost to follow-up), APROP had completely regressed and signs of vasculitis had resolved with blood column visible through retinal vessels, retina assuming its normal transparent appearance. Retina was flat with evidence of growing retinal blood vessels. Only sequel present was a subtle subretinal change along superotemporal first order vessel in right eye and subretinal fibrosis along superotemporal vascular arcade in left eye [Figure 4].
Figure 4: Visit 4: Resolved retinalvasculitis and ROP after treatment. Note complete resolution of plus disease (black arrow) and perivascular exudates. Fibrosis alongsuperotemporal arcade (blue arrow) and subtle subretinal change in right eye (white arrow) seen. a: RE; b: LE

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  Discussion Top

Systemic fungal infection in preterm infants after prolonged NICU stay has been reported, the ocular manifestations being fungal endophthalmitis, [5],[6] intralenticular abscess [7] and retinal vasculitis. [1] The factors responsible for fungal sepsis are treatment with higher order antibiotics and central venous line. Most common fungus seen is Candida. [8],[9]

In our case the systemic risk factors presumably predisposed the infant for development of APROP as well as retinal vasculitis secondary to presumed fungal sepsis. Acute onset of abdominal distension with intolerance to feeds suggestive of NEC and a prolonged NICU stay with ventilatory support and central venous line led us to suspect fungal sepsis in this particular infant as these factors are known to predispose this condition.

Vinekar et al., in their report described retinal vasculitis as a premonitory sign of systemic fungal infection in a preterm infant. [1] The infant did not develop ROP, however subsequently the infant was diagnosed to have perinephric fungal abscesses. In the report by Shah et al., [7] the infant developed stage 5 ROP with bilateral intralenticular abscesses after systemic candidiasis.

In our case, on clinical suspicion of fungal sepsis, systemic antifungal treatment (Fluconazole 6mg/kg/day × 2 weeks) was started though blood culture was negative. It led to resolution of systemic as well as ocular signs of fungal infection.

Retinal vasculitis may have added to the retinal ischemia leading to rapid worsening of ROP. Hseih CJ et al., have reported aggravation of severe ROP due to prolonged candidemia in a preterm infant. [10]

We had to resort to IVB in right eye due to inability to proceed with additional laser. This was done after detail discussion and informed consent from parents.

At the longest follow up of 11 weeks after completion of laser treatment in left eye and 12 weeks of IVB treatment in right eye (post menstrual age 50 weeks), ROP had completely regressed and signs of vasculitis had resolved [Figure 4].

Retina was flat with evidence of growing retinal blood vessels. Only sequel present was subretinal fibrosis in left eye (which was managed only with laser treatment) and was probably secondary to severe vasculitis with extensive perivascular exudation rather than ROP. Use of IVB in right eye despite presence of active retinal vasculitis did not affect the structural outcome adversely.

Retinal vasculitis and APROP can occur simultaneously in presumed fungal sepsis in a preterm infant. IVB can be used as rescue therapy in such cases if laser treatment is impossible.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Vinekar A, Avdhani K, Maralusiddappa P, Prabhu VM, Mahendradas P, Indumathi VA. Retinal vasculitis as an early indicator of systemic candida abscesses in a preterm infant. J AAPOS 2011;15:96-7.  Back to cited text no. 1
International Committee for the Classification of Retinopathy of Prematurity. The International Classification of Retinopathy of Prematurity revisited. Arch Ophthalmol 2005;123:991-9.  Back to cited text no. 2
Shah PK, Narendran V, Kalpana N. Aggressive posterior retinopathy of prematurity in large preterm babies in South India. Arch Dis Child Fetal Neonatal Ed 2012;97:F371-5.  Back to cited text no. 3
Darlow BA, Ells AL, Gilbert CE, Gole GA, Quinn GE. Are we there yet? Bevacizumab therapy for retinopathy of prematurity. Arch Dis Child Fetal Neonatal Ed 2013;98:F170-4.  Back to cited text no. 4
Baley JE, Anable WL, Kliegman RM. Candida endophthalmitis in the premature infant. J Pediatr 1981;98:458-61.  Back to cited text no. 5
Gago LC, Capone A Jr, Trese MT. Bilateral presumed endogenous candida endophthalmitis and stage 3 retinopathy of prematurity. Am J Ophthalmol 2002;134:611-3.  Back to cited text no. 6
Shah GK, Vander J, Eagle RC. Intralenticular Candida species abscess in a premature infant. Am J Ophthalmol 2000;129:390-1.  Back to cited text no. 7
Manzoni P, Stolfi I, Pugni L, Decembrino L, Magnani C, Vetrano G, et al., Italian Task Force for the Study and Prevention of Neonatal Fungal Infections; Italian Society of Neonatology. A multicenter, randomized trial of prophylactic fluconazole in preterm neonates. N Engl J Med 2007;356:2483-95.  Back to cited text no. 8
Ariff S, Saleem AF, Soofi SB, Sajjad R. Clinical spectrum and outcomes of neonatal candidiasis in a tertiary care hospital in Karachi, Pakistan. J Infect Dev Ctries 2011;5:216-23.  Back to cited text no. 9
Hseih CJ, Lin KC, Liu JS. Neonatal candidemia exacerbated the progression of retinopathy of Prematurity in an extremely-low-birth-weight infant with persistent tunica vasculosa lentis. Ophthalmic Surg Lasers Imaging 2010;9:1-4.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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