British Journal of Ophthalmology, 1987, 71, 559-562

'Rush' type retinopathy of prematurity: report of threecasesI NISSENKORN, I KREMER, E GILAD, S COHEN, AND I BEN-SIRA

From the Department of Ophthalmology, Beilinson Medical Center, Petach Tikva and the Sackler School ofMedicine, Tel Aviv University, Israel

SUMMARY Three premature infants observed to develop severe stage III retinopathy ofprematurity (ROP) at 3 to 5 weeks of age received immediate treatment by cryoablation andphotocoagulation, with good results. The critical importance of the ophthalmic examination ofpremature babies from the age of 2 weeks, so as not to overlook such cases of 'rush' type ROP isstressed and the difficulty involved in treating such small neonates is discussed.

In the great majority of premature babies withretinopathy of prematurity (ROP) the diseasedevelops gradually and relatively slowly. The earlystages of ROP usually appear six to eight weeks afterbirth. 1-3 Therefore the Committee on Fetus andNewborn of the American Academy of Pediatrics'and Palmer23 recommended that the eyes of pre-mature infants should be examined on dischargefrom the nursery or at the age of 7 to 9 weeks.Reports in the Japanese literature, however, indicatethat ROP may also take a more rapid course withinthe first month of life.4`6 These investigators foundthat at the age of 3 to 5 weeks the disease present inzone I or II rapidly progressed to severe ROP (stageIII according to the new international classification')and they termed this 'rush' type ROP, concludingthat the prognosis for vision was very poor.We describe here three infants with 'rush' type

ROP in whom treatment by cryoablation and photo-coagulation yielded good results.

Case reports

CASE 1A female infant was born in the 26th week ofgestation with a birth weight of 950 g. The apgar scorewas 6 at birth and 8 five minutes later. Assistedventilation was immediately started with 100%oxygen and continued for three weeks with FiO2(fraction inspiratory oxygen) 25%. The Po2 wasmaintained at 50-70 mmHg, but there were threeshort episodes of Po2 above 100 mmHg. The first eyeCorrespondence to Dr I Nissenkorn, Dcpartment ofOphthalmology,Bcilinson Medical Centcr, Petach Tikva, 49 100, Isracl.

examination made at the age of two weeks disclosedremnants of tunica vasculosa lentis and a hazyvitreous in both eyes. The fundus examinationrevealed-the presence of retinal vessels in zone 1. Theangle between the large vessels was 120°. At the ageof 3 weeks congestion of posterior pole vessels andthe beginning of fibrovascular proliferation (FVP)were found. At 4 weeks there was severe congestionof the iris vessels with rigidity of the pupils, and thefundus examination showed 360° of severe stage IIIROP. Both eyes showed similar retinal changes(Figs. 1,2) and the angle between the large vesselswas 1000.Under general anaesthesia a conjunctival incision

3-4 mm in length was made in each quadrant. Afterundermining with blunt-ended scissors, a cataractcryoprobe was inserted into each of thf incisions wellbehind the equator, and cryotherapy of the avascularretina was performed through 3600. Particular carewas taken to defrost the probe before moving it toanother location. Because the inferior obliquemuscles interfered with the cryopexy probe owing tothe posterior location of the disease, the avascularretina close to the macula was photocoagulated withxenon light. Twenty-four hours after this procedurethe iris vessels were less engorged and the pupils wereless rigid. The FVP started to disappear, and threeweeks later progression of the retinal blood vesselsbetween the cryopexy scars was observed (Figs, 3 and4). The angle between the large vessels was 700, butthe macula was not displaced.Three months later the eyes appeared to be

orthophoric with central fixation, and there was nonystagmus. The anterior segments were normal, and

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Fig. 1 Fundus photograph oftheright eye ofcase 1 (at 30 weeks ofgestation) showing the posteriorpole with congested vessels in zone Ionly and extensivefibrovascularproliferation through 3600 at theedge ofthe advancing vasculature(severe ROP, stage III).

fundus examination revealed cicatricial changesfollowing peripheral retinal ablation (stage II

according to the classification of Reese et al.' Onretinoscopy a + 1-0 hypermetropia was found in botheyes.

CASE 2

This infant was born at 26 weeks of gestation with a

birth weight of 800 g. The apgar score was 6 at birthand 7 after five minutes. Assisted ventilation was

started because of respiratory distress. Po2 range

Fig. 2 runaus pnotograpn oj mhe rignh eye oj case Iw-iJweeks gestational age) showing the progression ofthecongested vessels in association withfloridfibrovascularproliferation near the advancing vascular border (severeROP, stage III).

I Nissenkorn, I Kremer, E Gilad, S Cohen, and I Ben-Sira

was 50 to 70 mmHg. Oxygen treatment was given for10 days. At the age of 2 weeks examination revealednormal anterior segments in both eyes and remnantsof tunica vasculosa lentis. Fundus examinationshowed retinal vessels only in zone I in both eyes. Atthe age of 4 weeks there were pupillary rigidity andengorgement of the iris vessels, with active stage IIIof severe ROP in zone I-II, 3600. On the same daycryotherapy was carried out on the avascular retinaanterior to the fibrovascular proliferation. Theprocedure was performed under local anaesthesiaachieved with drops of a local anaesthetic and fourinjections of lignocaine 2% into the subtenon space.Four conjunctival incisions at the limbus enabled thecataract cryoprobe to reach the entire avascularretina, so that xenon photocoagulation was notnecessary.Two weeks later disappearance of the pupillary

rigidity as well as regression of the large retinalvessels congestion and the FVP were observed. Atthe age of 2 months fundus examination showednormal posterior poles and progression of the retinalvessels between the cryoscars in the equator, withcicatricial stage I. No nystagmus was observed, andthe child has had a good fixation in both eyes.

CASE 3This female infant was born at 26 weeks of gestationweighing 870 g. The apgar score was 2 at birth and 5after five minutes. Assisted ventilation with 100% 02was started because of left lung hypoventilation andatelectasis of the upper lobe of the right lung, withrecurrent episodes of apnoea. Po2 was in the range of70 mmHg. Oxygen was given for 12 days. Eye

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'Rush 'type retinopathy ofpremnaturity: report ofthree cases

Fig. 3 Fundus photograph oftheright eye ofsame case at the age of3months followingphotocoagulation combined withcryotherapy. Photocoagulation andcryotherapy scars are seen temporalto the macula (bottom).

examination performed at the age of 2 weeksrevealed remnants of tunica vasculosa lentis withnormal anterior segments. The fundus examinationshowed extension of the retinal vessels to zone I. Twoweeks later congestion of posterior retinal vesselswas observed, and one week later, at the age of 5weeks, severe active ROP (stage III) was found inzone I-II, 3600. On the same day cryotherapy wasperformed on the avascular retina, 3600, with localanaesthesia, achieved as described for case 2. Oneweek later the congestion of retinal vessels hadlessened and there was some regression of the FVP.Three weeks later the FVP had disappeared. At theage of 3 months the angle between the large vessels

i..X iiI 7le_

Fig. 4 Fundus photograph ofthe right eye ofsame case atthe age of3 months showing cryotherapy scars nasal to thedisc, and retinal vessels progressing between the scars.

was 1000 with some dragging on the macula. Pro-gression of the retinal vessels between the cryoscarsand towards the equator through 3600 was observed.The retinal changes were those of cicatricial stage II.

Discussion

The rush type ROP described in the Japaneseliterature4 appears to occur only in prematurebabies of extremely low birth weight. The fibro-vascular proliferative changes involve the entirecircumference of the posterior retina. On thetemporal side the retinal vessels reach the lateralborder of the macula, and on the nasal side they runtortuously only 2-3 disc diameters from the opticnerve head. These changes appear in zone I or II ofthe new international classification of ROP.7 In mostcases retinal detachment was found to develop withina very short period from the onset of the retinopathicchanges.The increased chance for survival of neonates of

extremely low birth weight in critical generalcondition creates a much larger population at risk todevelop the severe stages of ROP and also rush typeROP. In our hospital the three infants presentedabove constitute 5% of the 50 cases of ROP requiringcryoablation treatment during recent years.'There can thus be little doubt that it would be

advisable to institute routine examination of allpremature infants, weighing less than 1000 g andborn at the age of 28 weeks or less, as early as twoweeks after birth. We are of the opinion that thisprocedure is harmless, as it is performed in thenursery intensive care unit while the baby is in the

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I Nissenkorn, I Kremer, E /ilad, S Cohen, and I Ben-Sira

incubator, in which body temperature, respiration,and all the vital signs are well controlled. When thefirst examination is made only at the age of 7 to 9weeks, initial retinal changes are apt to be missed,and in these eyes there may already be retinaldetachment or severe cicatricial changes which willcompromise the infant's vision.

Therefore, when rush type ROP is diagnosedimmediate treatment is indicated. Althoughcontrolled clinical trials and long term results are stilllacking, there is increasing evidence to show thatcryotherapy or photocoagulation, or a combinationof the two, are beneficial in treating active stage IIIROP.4-6 "-13 Circumferential ablation of the avascularperipheral retina, while getting as close to theadvancing edge of the retinal vasculature as possible,has been shown to achieve retinal quiescence withinhours.' We believe that when the disease exists inzone I a combination of cryotherapy and photo-coagulation is necessary to arrest it. This can bedone only under general anaesthesia or with localsubtenon injection. When signs of the disease arefound in zone II, cryotherapy alone should suffice,and this is performed with local anaesthetic drops orfour-quadrant injection of lignocaine.An important point is the difficulty involved in the

treatment of such small babies having a weight of lessthan 1000 g who require oxygen and whose generalcondition is unstable. For this reason a neonatologistshould attend throughout the procedure. Further-more, to prevent occlusion of the central retinalartery'4 particular care must be taken not to press toovigorously on the eye during cryoapplication and alsoto observe continuously the central artery even whilethe cryoprobe is being defrosted.

In conclusion, we should like to stress again thecritical importance of ophthalmic examination inpremature infants from the age of 2 weeks, with

immediate initiation of treatment when stage IIIROP is diagnosed.

References

I Eisenbaum AM. Timing offundus examination for the diagnosisof retrolental fibroplasia. Retinopathy of Prematurity Confer-ence Syllabus. Washington, DC, 1981: 318-24.

2 Palmer EA. Optical timing of examination for acute retrolentalfibroplasia. Ophthalmology 1981; 88: 662-8.

3 Flynn JT. Discussion of paper by Palmer EA: Optimal timing ofexamination for acute retrolental fibroplasia. Ophthalmology1981; 88: 667-8.

4 Ucmura Y. Current status of retrolental fibroplasia: report of theJoint Committee for the Study of Retrolental Fibroplasia inJapan. Jpn J Ophthalmol 1977; 21: 366-78.

5 Yamagishin N, Nagata M. Survey of the cicatricial stagesof retinopathy of prematurity after treatment with photo-coagulation. Folia OphthalmolJpn 1979; 30: 101-6.

6 Majima A. Studies on retinopathy of prematurity. 1. Statisticalanalysis of factors related to occurrence and progression in activephase. Jpn J Ophthalmol 1977; 21: 404-20.

7 Committee for Classification of Retinopathy of Prematurity.International classification of retinopathy of prematurity. ArchOphthalmol 1984; 102: 1130-4.

8 Reese AB, King M, Owens WC. A classification of retrolentalfibroplasia. An J Ophthalmnol 1953; 36: 1333-63.

9 Ben-Sira 1, Nisscnkorn 1, Grunwald E, Yassur Y. Treatment ofacute retrolental fibroplasia by cryopexy. BrJ Ophthalmol 1980;64: 758-62.

10 Topilow HW, Ackerman AL, Wang MF. The treatment ofadvanced retinopathy of prematurity by cryotherapy and scleralbuckling surgery. Ophthalmology 1985; 92: 379-87.

11 Sasaki K, Ymashita Y, Mackawa T. Adachi T. Treatment ofretinopathy of prematurity in active stage by cryocautery. Jpn JOphthalmol 1976; 20: 384-95.

12 Hindle NW. Cryotherapy for retinopathy of prematurity toprevent rctrolental fibroplasia. Can J Ophthalmol 1982; 17:2)7- 12.

13 Nissenkorn I, Kremer 1, Ben-Sira I, Cohen S. Garner A. Aclinicopathological case of retinopathy of prematurity (ROP)treated by peripheral cryopexy. Br J Ophthalmnol 1984; 68:36-41.

14 Tasman W. Zone I retinopathy of prematurity. Arch Ophthalmnol1985; 103: 1093-4.

Acceptedfor publication 2 September 1986.

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