Stage 5 ROP Case 2

A boy was born prematurely at 25 weeks of gestation with a body weight of 1 lb 8 oz. (680 grams). Laser treatment for stage 3 ROP was unsuccessful. The patient developed a total retinal detachment (stage 5) in both eyes. Both eyes showed a dense white membrane behind the lens (Fig. 27). The retina was not visible. The ultrasound study demonstrated a total retinal detachment  (Fig. 28). The detachment formed a closed channel all along its length (closed-closed channel) in both eyes. This shape of retinal detachment has the poorest prognosis for retinal reattachment. Open-sky vitrectomy was performed in each eye. The operation successfully reattached the retina (Figs. 29,30,31). At two years of age, his vision was 20/800. Although low, his vision was good enough for him to see large objects, and entering into the "seeing world". A graph of his developing vision is shown in Fig. 32.

Fig. 29 Photograph of the left eye after open-sky vitrectomy. The white pupil disappeared.

Fig. 32 Graph showing the gradual development of vision in a normal infant (red). The development of the postoperative vision in case 2 is shown in yellow.

Conclusion

Cases of Stage 5 ROP should never be abandoned without a careful examination of the possibility offered by open-sky surgery.  The reason is that 40% of the cases so operated recover some degree of sight.  To refuse a premature baby a chance to enter ever so little into the seeing world, with all its marvels, really is a sign of incredible hardness of heart.  Would this be malpractice?  Prior to deciding whether or not to perform an open-sky procedure on a premature baby the following preoperative information must be obtained:

History of the pregnancy; history of the baby after birth; existence of other malformations in the baby’s organs, particularly brain, lungs and heart; status of the eye; existence of intraocular hemorrhage and status of the retina.

How to Determine the Status of the Retina

Preoperatively it is essential to perform an ultrasound examination of the affected eye.  This would reveal the presence of a substantial intraocular hemorrhage which is a contraindication to open-sky surgery.  The position of the retina is also determined by ultrasound.  The retina is detached by a double mechanism: 1) exudation of fluid from the tissue located under the retina and 2) traction on the retina by scar tissue and new vessels.  Retinal breaks are rare in retinopathy of prematurity in babies.  The result is that the retina loses its balloon shape (it normally lines the inside of the eye, which is roughly spherical).  The detached retina becomes funnel shaped, remaining attached to the optic nerve.  The funnel has four possible shapes: open-open (Fig.33A), open-narrow (Fig.33B), narrow-open (Fig.33C), and narrow-narrow (Fig.33D).  The chances of reattaching the retina with a narrow-narrow funnel are only 20%, whereas they are 40% with the other types of funnel. 

Since the reattached retina of a premature infant may improve postoperatively, visual training of the child postoperatively is very important.  The child should not be sent to a school with blind children, but with seeing children.  The parents and teachers should constantly stimulate the child visually.  Under those circumstances, the child will get the maximum visual recovery.