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Year : 2020  |  Volume : 8  |  Issue : 1  |  Page : 30-31

Type 1 early treatment of retinopathy of prematurity with corneal haze? Cause

Department of Vitreo-Retina, Sarakshi Netralaya, Nagpur, Maharashtra, India

Date of Submission27-Dec-2018
Date of Acceptance29-Mar-2019
Date of Web Publication6-Mar-2020

Correspondence Address:
Shilpi Harshal Narnaware
Sarakshi Netralaya, Plot No. 19, Rajiv Nagar, Wardha Road, Nagpur - 440 025, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcor.jcor_92_18

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This is a case report which highlights the challenges faced in treating Type 1 early treatment of retinopathy of prematurity (ETROP) with corneal haze. We report a case of a preterm boy born at 28 weeks with birth weight of 1000 g who presented with corneal haze along with Type 1 ETROP. Because of inability to complete LASER and increase in posterior plus disease , intravitreal ranibizumab was given as a rescue therapy to buy time. Finally, we could succeed in treating the disease with complete regression and clear cornea on the last follow-up.

Keywords: Corneal haze, early treatment of retinopathy of prematurity, plus disease

How to cite this article:
Narnaware SH, Bawankule PK. Type 1 early treatment of retinopathy of prematurity with corneal haze? Cause. J Clin Ophthalmol Res 2020;8:30-1

How to cite this URL:
Narnaware SH, Bawankule PK. Type 1 early treatment of retinopathy of prematurity with corneal haze? Cause. J Clin Ophthalmol Res [serial online] 2020 [cited 2022 Aug 13];8:30-1. Available from: https://www.jcor.in/text.asp?2020/8/1/30/280218

Retinopathy of prematurity (ROP) is a disease that affects immature vasculature in the eyes of premature babies and is inversely related to gestation age and birth weight. Because of favorable outcomes only in one-third of cases with cryotherapy[1] in and with the results of early treatment of ROP (ETROP) study,[2] there has been a paradigm shift in the treatment to LASER therapy from cryotherapy. Type 1 ETROP includes (a) Zone I, any stage ROP with plus disease, (b) Zone I, Stage 3 ROP without plus disease, and (c) Zone II, Stage 2 or 3 ROP with plus disease. Type II ROP includes (a) Zone I, Stage 1 or 2 ROP without plus disease and (b) Zone II, Stage 3 ROP without plus disease. Plus disease is a sign of on-going activity. Anterior plus disease includes (a) pupillary rigidity and (b) iris vessels engorgement while posterior plus disease signs are (a) vitreous haze and (b) tortuosity and engorgement of postpole and peripheral vessels.

Postlaser transient corneal haze is a known entity, but there is no description of ROP with concomitant corneal haze with normal corneal diameters for age,[3] which cleared completely over a period of 4 months even in literature. Even in severe cases of plus disease or aggressive posterior ROP, corneal haze is not described.

This case report describes a case of Type 1 ETROP with corneal haze, difficulties in managing ROP in the presence of corneal haze, how case was managed, and probable causes of corneal haze.

  Case Report Top

Twin male infant (other twin expired) born by normal vaginal delivery at 28 weeks of gestation with a birth weight of 1000 g with risk factors: respiratory distress syndrome, hyaline membrane disease, anemia, seizures, hyperbilirubinemia, hypoglycemia, necrotizing enterocolitis, apnea, and surfactant needed and on ventilation. The first ophthalmic examination was performed at a corrected gestational age of 32 weeks which revealed vascularization in Zone 1 in both the eyes. On follow-up at postmenstrual age of 32 weeks 5 days, examination revealed Zone 2 posterior Stage 3 ROP with posterior plus disease, i.e., ETROP Type 1 [Figure 1] with corneal haze [Figure 2] in both eyes. The baby was advised LASERS in both eyes and written informed consent for the same was obtained from parents. Partial laser was possible in the right eye, but no laser was possible in the left eye due to increased corneal haze. The baby was started on topical antibiotic-steroid eye ointment twice a day for 2 weeks (Ocupol Dx – Centaur Pharmaceuticals: polymyxin B sulfate BP 10,000 units combination + chloramphenicol IP 10 mg + dexamethasone sodium phosphate IP 1 mg) and mydriatic eye drop twice a day for 2 weeks (Homide – Warren Excel product: homatropine hydrobromide IP 2% w/v + benzalkonium chloride solution IP 0.01% w/v) and was reviewed after 2 days. Hazy view of the fundus revealed increase in plus disease. Cornea and glaucoma specialist opinion was sought. Corneal diameters under topical anesthesia were 9.5 mm in the right eye and 10 mm in the left eye. Intraocular pressures (IOPs) (under topical anesthesia and with wire speculum) were around 16 mm of Hg in both eyes (using Perkins tonometer). Examination under anesthesia was done and corneal diameters were consistent with previous findings while the IOP measured was 14 mmHg (with Perkins) in both the eyes. Along with previous topicals, topical carbonic anhydrase inhibitor twice a day (Azopt – Alcon: brinzolamide ophthalmic suspension 1%), lubricants four times a day (Refresh tears – Allergan: carboxy methyl cellulose sodium eye drop IP 0.5% w/v), and osmotic agents four times a day (Hypersol 5 – Jawa Pharmaceuticals: sodium chloride ophthalmic solution USP) were started and continued for 2 weeks. Meanwhile, both eyes were injected with injection ranibizumab 0.25 mg (accentrix 10 mg/ml– Alcon) intravitreally. As the corneal haze started clearing peripherally, the laser was attempted in multiple sittings, and subsequently, both eyes laser was completed through peripheral clear cornea. On follow-ups, gradual regression of retinopathy and corneal haze was noted. It took almost 4 months for complete regression of corneal haze.
Figure 1: Fundus photograph of the baby showing Zone 2 Stage 3 threshold retinopathy of prematurity

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Figure 2: Anterior-segment photograph showing corneal haze

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  Discussion Top

ROP is a blood vessel disease characterized by increased fibrovascular proliferation, which primarily develops in children with a birth weight <2000 g. LASER is the gold standard treatment in cases of ROP warranting treatment, although anti-vascular endothelial growth factor has an adjunctive role where laser is not possible. Probable causes for corneal haze, in this case, can be fluid overload/hyperglycemia, preservative toxicity, trabeculitis, endothelitis, congenital corneal pathologies. Fluid overload or sudden hyperglycemia is known to cause sudden development of corneal haze. However, the corneal haze due to hyperglycemia or fluid overload gradually gets clear over a period of 3–4 weeks, while in our case, it took almost 4 months for complete clearance of cornea haze. Preservatives used in topical medications (chlorobutanol/benzalkonium chloride) can cause significant keratitis and damage to corneal epithelium, but corneal haze because of preservative toxicity also gets cleared within 2 weeks. Transient rise in IOP due to trabeculitis because of inflammation secondary to ROP can be a cause, but the points against transient glaucoma are (1) Haze was more centrally while haze due to raised IOP is seen limbus to limbus; (2) IOP was normal; (3) corneal diameters were in normal range; and (4) even in severe cases of plus disease, corneal haze is not noted. Endotheliitis due to herpes simplex virus is usually not seen before the age of 6 months of age as the baby is protected with maternal antibodies. Congenital corneal pathologies such as congenital hereditary endothelial dystrophy, Peters anomaly, and storage disorders have corneal haze which persists throughout life.

  Conclusion Top

ROP can present with corneal haze which can challenge the treatment of underlying pathology. To the best of our knowledge, this is the first case reported of Type 1 ETROP with concomitant corneal haze with normal corneal diameters for age, which cleared over a period of 4 months. This case highlights the difficulties encountered while treating such case and teamwork of various specialists in managing such case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the child's parents have given his consent for images and other clinical information to be reported in the journal. The child's parents understand that his name and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Cryotherapy for Retinopathy of Prematurity Cooperative Group. Multicentric trial or retinopathy of prematurity: 3 Months outcome. Arch Ophthalmol 1990;108:195-204.  Back to cited text no. 1
Early Treatment for Retinopathy of Prematurity Cooperative Group. Revised indications for the treatment of retinopathy of prematurity: Results of the early treatment for retinopathy of prematurity randomized trial. Arch Ophthalmol 2003;121:1684-94.  Back to cited text no. 2
Nanda L, Adarsh E, Nithisha, Srivastava VK, Varda N. Comparison of horizontal corneal diameter in premature infants and mature infants. IOSR J Dent Med Sci 2015;14:41-4.  Back to cited text no. 3


  [Figure 1], [Figure 2]


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