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Retrolental Fibroplasia: A Modern Parable
by William A. Silverman, M.D.

Chapter 1

A New Affliction in Premature Infants

On February 14, 1941, Doctor Stewart H. Clifford, a Boston pediatrician, made a routine home visit to examine an infant girl born prematurely the previous November. Doctor Clifford remembers this house call as follows:

The father, I recall, was a young rabbi and the family lived ... [at 2 Elm Hill Park, Roxbury]. Although the baby's general development was excellent, I was shocked to note roving nystagmus, and opacities in the eyes, and I had to tell the family I was afraid the baby could not see. I immediately referred the baby to one of our leading ophthalmologists, Doctor Paul [A.] Chandler.

Doctor Chandler examined the infant and made this note on his office record:

... shows a searching nystagmus of both eyes. Anterior chambers almost flat. Pupils react to light. Faint red reflex above, but below there is a grayish reflex.

He told Doctor Clifford he had not seen anything like it before, and arranged an admission to the Massachusetts Eye and Ear Infirmary for examination under ether and for consultation with Doctor Frederick Verhoeff, one of the period's leading authorities in eye pathology. The examination, carried out on February 19, 1941, revealed a grayish membrane covered with blood vessels which appeared to be on the back surface of the lens of both eyes. A descriptive label, "fibro-vascular sheath of the lens," was applied to the unusual condition. An operation was considered but dismissed because Doctor Verhoeff thought the outlook was so poor.

In the meantime, on a Sunday afternoon in the same month, Doctor Clifford received an urgent call from a prominent Boston family asking him to examine a twin infant who had been born on July 13, 1940, weighing 1.02 kg (2 lb 4 oz) at delivery; the co-twin had not survived. Doctor Clifford recalls:

I went to the family apartment on Beacon Street to find there had been a family gathering with the Philadelphia members of the family, and one of the relatives noted the eyes of the infant boy [now 7 months old] were behaving strangely. When I arrived I was shocked to find my second case within a week of my first. I told them [the family] of my suspicions and of my previous case. They wanted immediate action, so I called Doctor Chandler -- he was out of town. I called Trigve Gunderson-he was away. I finally got hold of Doctor [Theodore] Terry.

Terry soon arrived with all of his equipment and declared that the condition was congenital cataracts. He arranged for admission to Children's Hospital the next day for operation. I insisted that both Doctors Chandler and Verhoeff be called in consultation prior to any operation. This was done and we all gathered in the operating room amphitheatre at the Children's. They were able to convince Doctor Terry that the condition was not operable.

Doctor Chandler's memory of that interesting morning is as follows:

The day after I saw the first case, I happened to walk by the operating room door and Doctor Terry asked me to come in and look at a baby on whom he was about to operate for what he thought was a congenital cataract. Fresh from the experience of the day before, I recognized that this was the same thing, and of course, no operation was done.

Boston Lying-In Hospital has preserved the nursery records of these two children who were the first to be diagnosed as afflicted by what came to be called "retrolental fibroplasia" (literally, scar tissue behind the lens of the eye). The neologism was coined in 1944 by Doctor Harry Messenger of Boston, an ophthalmologist who was also a Latin and Greek scholar. The scion had been delivered by Doctor Frederick Irving on July 13, 1940, the first born of twins and, as noted, he weighed 1.02 kg (2 lb 4 oz). The fetal membranes had ruptured 4 days earlier, and the placenta and membranes showed signs of inflammation.

The co-twin was female and weighed 1.01 kg (21b 31/z oz). The fetal membranes were intact and there was no inflammation, but she had breathing difficulty and died at 6 hours of age. A diagnosis of "respiratory failure" was recorded (an autopsy was not performed).

The initial care prescribed for the surviving boy was typical for the time (Fig. 1-1). In describing the infant the nurses noted: "cries well" and "color good" (Fig. 1-2). The findings of physical examination were not recorded on the chart. .

The rabbi's daughter was born on November 23, 1940; premature labor was brought on by an abnormally low position of the placenta in the uterus. Her birthweight was 1.81 kg (4 lb). After a physical examination the baby was described as "O. K.," and the nurses' notes indicated that the infant's condition was good. The initial orders were "routine premi regime", which included the same measures used for the first infant (Fig. 1-1). On the second and third days of life there were several spells of cyanosis, but no other difficulties were recorded.

The two Boston infants were the forerunners of an epidemic of blindness which rose to unsurpassed heights throughout the world in the next twelve years. In 1942, Terry published a brief note describing the unique eye condition of the Beacon Hill twin and noted the following:

One of the consultants, Doctor Paul A. Chandler, has a case almost identical in appearance, in an infant also born prematurely ... Recently three other cases have been seen in the clinic of the Massachusetts Eye and Ear Infirmary ...

Terry went on to speculate about mechanisms to explain this odd affliction. Finally, he wrote two prophetic sentences in the first announcement of the disorder:

In view of these findings [all 5 instances occurred in infants born prematurely] perhaps this complication should be expected in a certain percentage of premature infants. If so, some new factor has arisen in extreme prematurity to produce such a condition.

Between 1942 and 1945 Terry collected 117 examples of the new type of blindness. He became convinced the condition developed following birth because in three prematurely born infants whose eyes were normal after delivery, he subsequently found well-established retrolental fibroplasia (RLF). Before his death in 1946, Terry made extensive studies on the development and regression of the fetal blood vessels of the eye and attempted, without success, to reproduce the disease via experiments in young opossums and rats.

Despite Terry's observation that the eyes of some RLF victims were normal at birth, the notion persisted among physicians until 1948 that there was either an inherent or acquired abnormality of the eye caused by factors which operated before birth or, at the latest, immediately thereafter.

In 1948, William and Ella Owens, husband-and-wife ophthalmologists at the Wilmer Ophthalmological Institute of Johns Hopkins Hospital and University, examined more than 200 premature infants at birth by direct inspection of the interior of the eyes with an opthalmoscope; none had RLF. Half of these babies were patiently examined at monthly intervals until the age of 6 months; 4 percent developed RLF. They described the progressive development of changes in the ophthalmoscopic appearance of the blood vessels of the retina, beginning at 2 1/2 to 3 1/2 months after birth.

As depicted in Figure 1-3, the first detectable abnormality was slight enlargement of the caliber of arteries and veins in the retina. The size of the vessels increased, the orderly course of the arteries and veins became convoluted, and soon the retina itself became elevated in the far peripheral regions of the eye. Grayish masses of detaching retinal tissue increased in size and extent and, after a short time, a gray membrane (the detached retina covered with many enlarged blood vessels) billowed forward in the chamber of the eye and formed an opaque cast behind the lens. At this stage of the abnormal process, the pupils of the eye appeared white even to an untrained observer (Fig. 1-4). Both eyes were usually involved and, not infrequently, the severity of the changes was unequal.

These observations, which made early diagnosis possible, were confirmed rapidly. It soon became routine in many large research hospitals throughout the world to examine the interior of the eyes of the premature infants at weekly intervals. By the end of the 1940s, the dimensions of the RLF epidemic were becoming evident. The startling new complication alarmed all who were making efforts to preserve the lives of premature infants and many now paused to review the development of special procedures in newborn care, in the hope of detecting a clue to this mystery.

 

Fig. 1-1. Doctor's orders prescribing the initial care for the surviving twin infant boy born in Boston Lying-In Hospital on July 13, 1940. The orders read as follows:

"1. Hess Bed [see Fig. 7-1]-Temp of Bed 85 to 88[°F]. Unless baby's temp drops below 96. If it drops below 96 raise [temperature of] Hess Bed gradually but not over 92. If baby's temp goes above 98 -- remove cotton wrapping.
2. Constant oxygen [piped into bed from an oxygen tank beside the bed -- see Fig. 7-1].
3. Baby's temp every 1 h until stabilized between 96-98, then every 4 hrs.
4. Nothing by mouth until ordered. Temp q 2° [hours] for 2 times-then q 4° V.O. [vocal order] Dr. Brines. D.W.F. [nurse's initials]"

Fig. 1-2. Hospital chart recording the events of the first 4 days of life in the surviving twin infant boy (see Fig. 1-1). The nurse's summarize the doctor's orders (lower left panel). The initial "Remarks" on admission to the nursery read:

"12 n [noon] cried well - Suctioned - Meconium
1. Cried well
2. q [every] i h [hour]
3. Color good. "

Fig. 1-3. The development of RLF described by Owens and Owens in 1948 (appearance of the retina through a direct ophthalmoscope). A. An early stage showing dilated and tortuous blood vessels of the retina. B. Later stage, showing an elevation of the retina developing in the periphery of the eye. C. Further extension of the elevated retina, appearing as a membrane at the edge of the retinal field. D. Complete retrolental membrane, with blood vessels coursing over the completely separated retina. At this stage the pupil appears white. (For a description of the early changes seen with modern equipment see Fig. 9-1.)

Fig. 1-4. End stage of RLF with completely detached retinas presenting as white membranes filling the pupil of the eye. The child is totally blind.


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