CaseWise 3

Management of Stage 4b Retinopathy of Prematurity

Dr. Sheetal Bajoria, Dr. Sarang Lambat, Dr. Vinay Nangia
Suraj Eye Institute, Nagpur, India

Introduction:
Retinopathy of prematurity (ROP) is a retinal vasoproliferative disease which affects premature infants. Despite improvement in neonatal care and management guidelines ROP remains a major cause of childhood blindness worldwide.ROP screening is important as 15-30% babies develop blindness if untreated2. Premature infants with ROP need timely treatment due to critical period of visual development. Early diagnosis and prompt treatment of ROP can prevent blindness due to retinal detachment.

Case Report:
A baby girl, 3 months of age, with birth weight 1100 grams, gestational age 32 weeks was brought to us for ROP screening at post menstrual age  of 44 weeks. She was a born of a normal delivery. Baby was admitted to NICU for birth asphyxia for 2 months. She developed sepsis during NICU stay and received intravenous antibiotics and platelet infusion for the same. Her mother had a normal antenatal course and there was no history of prematurity in elder sibling. Anterior segment examination was normal. Dilated fundus examination showed both eyes 360 degrees tractional retinal detachment sparing the macula with fibrovascular proliferations suggestive of stage 4a ROP (Fig 1). She was advised both eyes pars-plana vitrectomy (PPV) under general anaesthesia. The baby was unfit for surgery as hemoglobin was 6.9 mg/dl. She was admitted and given packed cell transfusion. It took almost 2 weeks for the baby to get fitness for surgery and by that time the tractional retinal detachment in both eyes had progressed to macular involvement suggestive of stage 4b ROP (Fig.2). Patient underwent both eyes PPV under general anaesthesia. The surgery went well but postoperatively she developed vitreous haemorrhage in both eyes which resolved gradually over a period  of  3 months. 

Fig1a and 1b shows stage 4a ROP – 360 degree tractional Retinal detachment with fibrovascular proliferation not involving macula
Fig.2a and 2b shows stage 4b ROP – 360 degree tractional retinal detachment with fibrovascular proliferation  involving macula

An examination under anaesthesia done at 10 months of age showed a refractive error of OD -5.00 DS/-2.00 DC@90 OS -1.25DS/-2.00DC @ 90 and retina was well attached in both eyes (Fig. 3) She was advised glasses with full correction based on retinoscopy findings and was called every 6 months for regular follow up. She had an acceptable ocular and neurodevelopmental assessment at her age at further follow up.

Fig.3a and 3b  10 months postoperative fundus photograph showing attached retina in both eyes.

Discussion:
Capone and Trese reported a 90% retinal reattachment rate in a series of 40 eyes undergoing lens-sparing vitrectomy for stage 4A ROP.3 Recent reports of scleral buckling for stage 4 ROP have reported reattachment rates of 46% to 75%.4 Studies looking at lensectomy and vitrectomy for stage 4 ROP have reported reattachment rates of 64% to 84%.5 Thus while comparing the outcomes all the procedures, lens-sparing vitrectomy seems to be an effective way of achieving retinal reattachment in most eyes with stage 4 ROP.If adequate release of traction is achieved the retina will spontaneously reattach. Iatrogenic breaks and Internal drainage retinotomy should be avoided because of the difficulty in closing retinal holes5. Surgery in eyes with ROP is challenging as there are several difficulties including entry in the eye due to small size of palpebral fissure opening, difficulty in reaching the periphery, risk of inadvertent retinal breaks and risk of anaesthesia in these premature babies. In our case despite various intraoperative challenges we could not only save the eye from going blind but also we could have an acceptable anatomical and functional outcome .

References:
1.Kim SJ, Port AD, Swan R, Campbell JP, Chan RVP, Chiang MF. Retinopathy of prematurity: a review of risk factors and their clinical significance. Surv Ophthalmol. 2018 Sep – Oct;63(5):618-637. Epub 2018 Apr 19. Review. PubMed PMID: 29679617; PubMed Central PMCID: PMC6089661. DOI:10.1016/j.survophthal.2018.04.002

2.Gibson DL, Sheps SB, Uh SH, Schechter MT, McCormick AQ. Retinopathy of prematurity-induced blindness: birth weight-specific survival and the new epidemic. Pediatrics. 1990 Sep;86(3):405-12. PubMed PMID: 2388790. Increasing incidence of retinopathy of prematurity. [Pediatrics. 1990]

3. Hubbard GB 3rd, Cherwick DH, Burian G. Lens-sparing vitrectomy for stage 4 retinopathy of prematurity. Ophthalmology. 2004 Dec;111(12):2274-7. PubMed PMID: 15582086. doi:10.1097/ICU.0b013e328309f1a5.

4. Capone A Jr, Trese MT. Lens-sparing vitreous surgery for tractional stage 4A retinopathy of prematurity retinal detachments.Ophthalmology 2001. DOI:10.1016/s0161-6420(01)00809-0

5.Chong LP, Machemer R, de Juan E. Vitrectomy for advanced stages of retinopathy of prematurity. Am J Ophthalmol. 1986 Dec 15;102(6):710-6. PubMed PMID: 3789051. DOI:110.1016/0002-9394(86)90397-

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