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   Table of Contents      
Year : 2004  |  Volume : 52  |  Issue : 4  |  Page : 319-20

Fulminate type of retinopathy of prematurity.

Department of Vitreous & Retina, Aravind Eye Hospital, Coimbatore, India

Date of Submission09-Apr-2003
Date of Acceptance05-Sep-2003

Correspondence Address:
Parag K Shah
Department of Vitreous & Retina, Aravind Eye Hospital, Coimbatore
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Source of Support: None, Conflict of Interest: None

PMID: 15693324

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Routine ophthalmoscopic screening for retinopathy of prematurity (ROP) from the age of 2 weeks is necessary to detect and treat fulminate ROP.

Keywords: Retinopathy of prematurity, fulminate, plus diseases, zone 1, Rush diseases

How to cite this article:
Shah PK, Narendran V, Saravanan V R, Raghuram A, Chattopadhyay A, Kashyap M, Devraj S. Fulminate type of retinopathy of prematurity. Indian J Ophthalmol 2004;52:319

How to cite this URL:
Shah PK, Narendran V, Saravanan V R, Raghuram A, Chattopadhyay A, Kashyap M, Devraj S. Fulminate type of retinopathy of prematurity. Indian J Ophthalmol [serial online] 2004 [cited 2020 Jun 3];52:319. Available from: http://www.ijo.in/text.asp?2004/52/4/319/14563

The American Academy of Pediatrics, the American Association for Pediatric Ophthalmology and Strabismus and the American Academy of Ophthalmology recommend that ophthalmic screening of premature infants be carried out between 4 and 6 weeks after birth or 33 weeks post-menstrual age,[1] whichever is earlier. The Japanese literature suggests that ROP may also take a more rapid course within the first month of life; they describe it as 'Type II ROP'. Some reports refer to this condition as 'Rush' disease. 'Rush' disease refers to eyes with any stage of ROP in zone 1 in addition to the presence of plus diseases. But this definition is often confused with more benign forms of ROP in zone 1 or 2. So it is more appropriate to use the term Fulminate ROP suggested by Shapiro.[2]

Here we describe an infant with fulminate ROP who responded well to timely photocoagulation.

  Case report Top

The patient was a male infant born by Caesarean section in the 32nd week of gestation with a birth weight of 1390 gms. The indication of Caesarean section was breech presentation with oligohydroamnios. Apgar score was 6 at one minutes and 8 at five minutes after birth. The baby was admitted in the neonatal intensive care unit with respiratory distress syndrome. Haemoglobin at birth was 16.4 gm% and the platelet count was 50,000 cells / cu mm. Blood examination showed normocytic normochromic cells with lymphocytic preponderance and a reduction in platelets. Assisted ventilation was started with 100% oxygen and was continued for 10 days. Platelet transfusion was given once. Dobutamine (5-10 microgram/kg/min) was given, as the peripheral circulation was inadequate.

The first ophthalmic examination was made 2 weeks after birth. Anterior segment examination of both eyes with 20 D lens showed residual tunica vasculosum lentis with no active iris vessels. Fundus of both eyes showed retinal vessels in zone 1 only with mild dilatation of the vessels at the posterior pole along with arterio-venous anastomoses and looping [Figure - 1]. There was neither a demarcation line nor a ridge as described by the International Classification of Retinopathy of Prematurity (ICROP).[3] A diagnosis of Fulminate ROP stage 2 was made.[2]

Examination after one week revealed active iris vessels in both eyes. Fundus revealed mild vitreous haze with severe dilatation of the posterior pole vessels with flat neovascularisation in both eyes and a pre-retinal haemorrhage over the infero-temporal arcade in the left eye [Figure - 2]. This stage was classified as fulminate ROP stage 3A.[2] Photocoagulation of both eyes was done with double frequency Yttrium Aluminum Gallium (YAG) laser using indirect ophthalmoscope delivery system in two sittings. A total of 3462 and 3677 spots were given in the right and left eye respectively.

Examination four days after laser showed persistent plus disease. Additional photocoagulation was given to skip areas of both eyes. Four days after the additional laser, the iris vessels disappeared with markedly decreased posterior pole retinal vessels dilatation [Figure - 3] and [Figure - 4].

  Discussion Top

Fulminate ROP appears to occur only in premature babies of extremely low birth weight. The entire circumference of the posterior retina is involved by the fibrovascular proliferative changes.[2] Severely dilated and tortuous vessels are seen over the posterior pole with abnormally proliferating vessels having vascular loops and haemorrhages. The above changes appear only in zone 1. The retinal vessels on the temporal side reach the lateral border of the macula and on the nasal side run tortuously only two to three disc diameters from the optic nerve head. In most cases retinal detachment develops within a very short period from the onset of retinopathy changes.

This case was of 32 weeks gestation and of 1390 gm birth weight, which does not fall under the category of either extremely low birth weight or low gestational age. If the screening had been done between 4 and 6 weeks after birth, initial retinal changes may very likely been missed, causing retinal detachment or severe cicatricial changes compromising the visual prognosis.

There is a need to reconsider the earlier teaching that early stages of ROP usually appear by 6-8 weeks of birth in view of our case and other reported cases in the literature. Individualised screening criteria have been suggested for developing countries like India[4] as a higher birth weight and gestation age are reported to cause threshold ROP in India; this is well illustrated in our case too.

The timing of evaluation is also being reconsidered and recent guidelines by the CRYO-ROP group[5] and several authors from India[4] suggest that "Initial eye exam is done by 31 weeks post-menstrual age or 4 weeks (Day 30 strategy) of chronological age and earlier in smaller babies".[4]-[5] Since this baby was 32 weeks post menstrual age, it was appropriate to screen him at 2 weeks post delivery with further close follow ups. This greatly helped to detect the earlier stage of worsening of ROP, and instituting prompt treatment.

Therefore, when fulminate ROP is diagnosed immediate treatment is indicated. Circumferential ablation of the avascular peripheral retina while getting as close as possible to the vascularisation has been shown to achieve retinal quiescence within hours.[6] An important point is the difficulty involved in treating such small and ill babies who have an unstable general condition. So treatment in the presence of a neonatologist is advisable.

In conclusion we would like to emphasise the importance of screening premature infants from the age of 2 weeks and to start treatment immediately once fulminate ROP is diagnosed.

  References Top

American Academy of Pediatrics. Screening examination of premature infants for retinopathy of prematurity. Pediatrics 2001;108:809.  Back to cited text no. 1
Shapiro MJ. Type 2 or Fulminate ROP. In: Kumar H, Shapiro MJ, Azad RV, editors. A Practical Approach to Retinopathy of Prematurity Screening and Management . New Delhi: Malhotra Enterprises, 2001.pp 23-33.  Back to cited text no. 2
The Committee for the Classification of Retinopathy of Prematurity. An International Classification of Retinopathy of Prematurity. Archives of Ophthalmol 1984;102:1130-34.  Back to cited text no. 3
Jalali S, Anand R, Kumar H, Dogra MR, Azad R, Gopal L. Programme planning and screening strategies in retinopathy of prematurity. Indian J Ophthalmol 2003;51:89-99.  Back to cited text no. 4
Reynolds JD, Dobson V, Quine GC, Fielder AR, Palmer EA, Saunders RA, et al, for CRYO-ROP and LIGHT-ROP Cooperative Groups. Evidence based screening criteria for ROP. Natural history data from the CRYO-ROP and LIGHT-ROP studies. Arch Ophthalmol 2002;120:1470-76.   Back to cited text no. 5
Ben-Sira I, Nissenkorn I, Grunwald E, Yassur Y. Treatment of acute retrolental fibroplasias by cryopexy. Br J Ophthalmol 1980;64:758-62.  Back to cited text no. 6


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

This article has been cited by
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Indian Journal of Ophthalmology. 2005; 53(4): 261-265


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