Foreword by the President

Transactions 29th European Strabismological Association Meeting – de Faber (ed) © 2005 European Strabismological Association, ISBN 04 1537 211 9

Special lecture: History of Strabismology

G.K. von Noorden

Longboat Key, Florida (USA)

Johann Wolfgang von Goethe stated in the introduction to his History of Color Vision, that “History is a burden rather than a joy for the young for they want to create a history of their own. But those advanced in age and education recognize most gratefully how much valuable and useful information has been passed on to them by their predecessors.”1 During work on our book “History of Strabismology”3 on which most of this lecture is based, I learned to appreciate just how much we owe to our scientific forefathers.

Only certain highlights in the evolution of our specialty will be presented in this lecture and the reader is referred to our book and a previous publication2 for pertinent illustrations and much more detail.

The word strabismus probably originates from the noun strebloi (Greek for squinter) and the verb strebloun (to turn) and appears for the first time in the writings of Hippocrates (460–377 BC). Hippocrates was also first to point out the hereditary nature of strabismus.

Since strabismus causes a conspicuous alteration of facial configuration it is not surprising that this condition was noted even in the earliest depictions of the human face. For example, the statue of a Pharaoh of the 3rd Dynasty (2778–2723 BC) clearly shows esotropia. Other depictions of severe esotropia can be found on the painted lid of a sarcophagus from the 13th century BC or in a statue from the tomb of a Chinese emperor of the 3rd century BC.

Scientific strabismology did not begin until the 19th century and earlier mentioning of this condition and its treatment was often shrouded in superstition and quackery. For instance, the Papyrus Ebers (1553–1550 BC) records treatment of “distortion of the eyes” with equal parts of turtle brain and spices. Despite the unscientific nature of such treatment and the perceived causes of strabismus in earlier times there was an occasional flash of brilliance and extraordinary insight by some authors. For instance, Celsus (25 BC–50 AC) of Rome distinguished paralytic from non-paralytic strabismus and Galen (131–201 AC), also from Rome, described all 6 extraocular muscles, recognized the oblique muscles as the principal cyclorotators, and considered strabismus as a loss of equilibrium between agonistic and antagonistic muscles. Paulus of Aegina (625–690) designed masks “to guide the positions of the eye” and recognized an “obscuration of vision from invisible causes”, perhaps the first description of amblyopia? Throughout the history of ophthalmology and well into the 20th century we find the notion that strabismus should be treated by forcing the eyes into certain gaze positions by masks designed for esoand exotropia. The elaborate illustrations of such masks by Georg Bartisch of Dresden in his first textbook of ophthalmology in the German language (1583) are well known. Treating strabismus by blocking vision in certain gaze position has survived well into our time as “sector occlusion.”

Other methods to correct strabismus by active visual stimulation consisted of dyed pieces of wool attached to the temples of an esotropic child or attaching an object to the tip of the nose in exotropia. We should note in passing that esotropia was considered a sign of beauty in the Mayan culture. A ball of bees wax dangling before the eyes of an infant was thought to stimulate convergence and (hopefully) induce esotropia!

The Law of Reciprocal Innervation, a fundamental aspect of ocular motility was clearly and without the help of electromyography defined during the 17th century by the Frenchman René Descartes (1596–1650) and long before being refined and confirmed by Charles S. Sherrington (1859–1952) of England. Another surprisingly advanced concept concerns the treatment of strabismic amblyopia. During a visit to an eye hospital in Riyad (Saudi-Arabia) I became

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acquainted with an ophthalmologist (Dr. M.Z. Wafai, Damascus) who was in the process of translating into English an ancient book on “Vision and Perception” by Thabit Ibn Qurrah Al-Harani. Al-Harani had lived in Mesopotamia during the 9th century AC. Knowing of my interest in amblyopia Dr. Wahai directed my attention to a passage in this book, which stated that: “strabismus should be treated by patching the normal eye. Once you do that, the visual power will go in it’s entirety to the deviated eye whose vision will return to normal. You should not release the normal eye (from treatment) until the vision in the deviated eye has completely returned to normal.” Clearly, Al-Harani practiced occlusion treatment for amblyopia as we know it today and nearly 900 years prior to the French naturalist de Buffon (1707–1788) who is often credited for having originated occlusion therapy. However, we may find it difficult to agree with our Mesopotamian colleague from ancient times when he goes on to write “such patients must also be purged, should bathe every second day and be made to sneeze by putting the juice of olive leaves into their noses.”

A noteworthy event in the history of strabismus occurred during the early 18th century with the appearance of an itinerant English “oculist” known as the Chevalier John Taylor who lectured and performed eye surgery in several Western European countries. His self-glorification and shameless publicity stunts would make even the most aggressive advertising of some of our contemporary “laser surgeons” appear exercises in modesty. He introduced himself as “the inventor of ophthalmology, sent by God to cure blindness on earth.” Instead Taylor left a trail of deteriorating eyesight and blindness behind him wherever he performed. One of his unfortunate victims was Johann Sebastian Bach. Taylor claimed widely to have discovered how to cure strabismus by a “fast, nearly painless operation without risk.” This operation actually consisted of excising a piece of conjunctiva from the fornix after which the fixating eye was patched. The operated eye straightened and the spectators applauded spontaneously, thinking that a miracle had been performed when the operated eye straightened after applying a bandage to the fixating eye. The patient was instructed not to remove the bandage until several days later. Of course, by that time Taylor had been paid and he and his retinue had long left town.

Despite this obvious quackery Taylor’s writings contain several noteworthy truisms. He provided precise drawings of the decussation of the optic nerve fibers in the chiasm and wrote that by dividing a nerve or muscle the disturbance of equilibrium of agonistic and antagonistic muscles may be restored. However, nobody had ever witnessed him actually doing a neurotomy or myotomy.

Nearly 100 years passed since Taylor’s exploits on the European continent before an event occurred that marked the beginning of strabismology as we know it today. The orthopedic surgeon Georg Stromeyer (1804–1876) had published in 1836 that he performed a myotomy of the medial rectus muscle in a cadaver and suggested that this operation may be useful in strabismus. Johannes F. Dieffenbach (1792–1847), Professor of Surgery at the University of Berlin picked up on this idea and performed the first myotomy of the medial rectus muscle in a 7-year old esotropic boy on October 26, 1839. Only 17 days later (sic) this case was published in the Medicinische Zeitung. Just 3 days after Dieffenbach, Flaurent Cunier (1812–1853) of Brussels had also performed a myotomy, this one on the lateral rectus muscle in an exotropic patient and reported his case in the Annales d’Oculistique (which he had founded a year earlier) but not until 1840. The American surgeon William Gibson (1788–1868) of Baltimore wrote in 1841 that he had actually done myotomies of the extraocular muscles in several patients as early as 1818 but had given up on this procedure because of several unsatisfactory results. Since he had not published his results at the time they were obtained the priority of having performed the first myotomy for strabismus clearly belongs to Dieffenbach.

News of this procedure spread like a wildfire throughout the world with hundreds of cases being reported from London and Boston within months after Dieffenbach’s first case. Within 2 years he had operated on 1200 cases! But the results were often satisfactory and large overcorrections (the Berliners called them Dieffenbachers) were frequent. Re-operations became necessary and Dieffenbach introduced an advancement of the myotomized muscle followed by myotomy of the lateral rectus muscle. A suture (called Faden in German) was then passed through the tendon of the lateral rectus and taped to the bridge of the nose and to the opposite cheek to keep the eye in an esotropic position. Dieffenbach called this traction suture a Fadenoperation and it is as

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unfortunate as it is confounding that this term was re-introduced by Cüppers (1975) to describe what is better called retroequatorial myopexy or posterior fixation of an extraocular muscle.

By the middle of the 19th century the myotomy had become discredited and it was not until Albrecht von Graefe (1828–1870) of Berlin, one of the founders of modern ophthalmology, introduced the controlled tenotomy, which rekindled the interest in and improved the results of strabismus surgery. Von Graefe published extensively on trochlear paralysis, ocular torsion, eccentric fixation, anomalous retinal correspondence, paradoxical diplopia and introduced the cover test. His friend Frans Cornelius Donders (1818–1889) of Holland was first to recognize uncorrected hypermetropia as a frequent causes of esotropia and clearly defined the association of accommodation with convergence. Albrecht von Graefe’s cousin and student Alfred Graefe (1833–1899) pioneered the surgical treatment of paralytic strabismus and introduced surgery on the vertical rectus muscles. Strabismology had emerged as a scientific discipline.

At about the same time, physiological optics established itself as a new discipline. Hermann von Helmholtz (1821–1894) of Königsberg, the inventor of the ophthalmoscope, studied the physiology of normal binocular vision, of retinal rivalry, diplopia, normal and abnormal retinal correspondence. Ewald Hering (1834–1918) of Leipzig introduce the law of equal innervation and found that visual objects, separated in subjective visual space, are localized subjectively in a common visual direction. Charles Wheatstone (1802–1875) of England invented the mirror stereoscope and Peter L. Panum (1820–1885) of Kiel made a major contribution to spatial vision by explaining the basis of stereopsis.

Without a clear understanding of the physiology and pathophysiology of binocular vision discovered by these and other scientists during the 19th century, strabismology would be no more than a quasi orthopedic subspecialty. Indeed, these men and their students were the giants of the past on whose shoulders we stand today, trying to catch a glimpse of the future, as the saying goes.

The 19th century witnessed also the first attempts to treat strabismus by visual exercises. A pioneer in this area was Emile Javal (1839–1907) of Paris a former mining engineer whose father suffered from a consecutive exotropia after having undergone myotomy of the medial rectus muscle and whose daughter had esotropia. He studied medicine, became an ophthalmologist, modified the stereoscope of Wheatstone (1838) and invented numerous instruments for the training of binocular function. This was the beginning of orthoptics. Javal also introduced atropine to treat amblyopia by penalization of the sound eye, and miotics to treat esotropia. His Manuel du Strabisme (1836) became a big success in France. Ernest Maddox (1863–1933) of England invented numerous instruments for the training of binocular functions that bear his name and his daughter Mary became the first orthoptist. A countryman of his was Claud Worth (1896– 1936) whose book Squint, its Causes and Treatment (1903) appeared in 6 editions. Three additional editions appeared after his death and were written by Bernard Chavasse (1880–1941) and T. Keith Lyle (1904–1987) and Bridgeman. This book was based on clinical observation in 2,337 cases of strabismus and became the major text for many years in the Anglophonic parts of the world.

Strabismology in the United States was largely oriented towards Europe until the first part of the 20th century when American ophthalmologists began making important contributions to strabismology. Lucien Howe (1848–1928) of Buffalo, New York published 2 volumes in The Muscles of the Eye, measured the muscle force of extraocular muscles and saccadic velocity. Oscar Wilkinson (1870–1945) published an excellent book on Strabismus: Its Etiology and Treatment in 1927. Other major contributions were by Alexander Duane (1858–1926) provided a classification of exotropia that is still in use and described the alternate prism and cover test. In 1922 J. Chalmer Jameson introduced scleral suturing of a recessed muscle. Walter Fink (1895–1969) described new techniques for surgery of the oblique muscles and Richard Scobee (1914–1952) published his book on the Oculorotatory Muscles, which was the only American teaching text available when I began my training in ophthalmology in 1956. Frank Costenbader (1905–1978) was one of the most prominent leaders in strabismology at that time, the first to devote his practice exclusively to pediatric ophthalmology and a pioneer of early surgery.

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A major impetus for the development of strabismology in the United States was the arrival on the American scene of one of Europe’s greatest strabismologists, Alfred Bielschowsky (1871–1940). Bielschowsky had studied ophthalmology under Carl H. Sattler (1880–1958) in Leipzig. Sattler had a deep interest in strabismus and was the only strong proponent of energetic occlusion treatment for amblyopia of his time. In fact, during the first part of the 20th century many ophthalmologists still believed that amblyopia was the cause rather than the consequence of strabismus! The University Eye Clinic in Leipzig was adjacent to the Physiological Institute, which was under the direction of no other than Ewald Hering. Close contacts and a spirit of collaboration existed between the young doctors of both institutions and it was in this fertile medium that Bielschowsky’s first publications originated. The paper that launched Bielschowsky’s career of becoming the foremost strabismologist of his time was on “Monocular diplopia without physical cause with comments on how a stabismic person sees” (Arch. Ophthal. 46: 143–83, 1898). He reported in minute detail the case of a young man with longstanding amblyopia who had lost his good eye after a perforating injury and now complained of monocular diplopia. Both Hering and Sattler were baffled by this case. After months of studying the patient Bielschowsky came to the conclusion that the double vision was caused by a competition between normal and abnormal relative localization, producing simultaneous localization of one visual object in two different visual directions. With today’s emphasis on evidence based medicine and prospective, multi-center, double-blind studies it is often overlooked that meticulous observation of an individual patient, a precise analysis of subjective complaints and the application of simple psychophysical tests in the office have led to far-reaching discoveries in the field of strabismus. Bielschowsky’s description of monocular diplopia from a sensory cause and Hering’s discovery of the law of common visual directions by a simple but brilliant experiment, are cases in point.

Bielschowsky eventually became professor and chair at the University Eye Clinic, first in Marburg and later in Breslau (then in Germany but now in Poland) and rapidly rose to a position of pre-eminence in the field of strabismus and neuro-ophthalmology. Among his numerous contributions were the physiology of involuntary fusional movements, the head tilt test, internuclear ophthalmoplegia, the doll’s head phenomenon, the clinical features and mechanism of dissociated vertical deviations, divergence paralysis, and the after-image test (with Hering).

In 1934 the Nazis had come to power and anti-Semitism was on the rise in Germany. Bielschowsky, who was of Jewish background, was forced to resign his position in Breslau and immigrated to the USA where he joined a group of outstanding visual scientists and clinicians at the Dartmouth Eye Institute in New Hampshire. Among these were Adelbert Ames, Paul Boeder, Hermann Burian, David Cogan, Walter Lancaster, Arthur Linksz, and Werner Herzau. What was Germany’s loss of a great man became a huge gain for America. In he single-handedly put strabismus on the map in America, as Paul Boeder once told me.

After Bielschowsky’s death, his student Hermann Burian (1900–1972) continued to unravel the mysteries of sensorial adaptations in strabismus and followed in his foot steps in becoming one of America’s leading strabismologists.

Only a few years after Bielschowsky’s departure from Germany, World War II with all its devastation erupted. Millions of people were displaced from their homes during and after the war and in the course of this chaos the treatment of strabismus and amblyopia became a matter of small importance and was neglected. Thus, in the early fifties of the last century when things began to return to normal the eye clinics of Europe were flooded with older children with severe, untreated amblyopia and eccentric fixation. They were now at an age at which compliance with occlusion treatment could no longer be expected. A new era of strabismology began at this point in time with the introductions of pleoptics by Alfred Bangerter (1909–2002) of Switzerland and Curt Cüppers (1910–1995) of Germany. These authors developed several and separate methods, called pleoptics, to treat amblyopia in older children by active and passive stimulation of the fovea of the amblyopic eye. These clever and original approaches were aimed at normalizing the fixation behavior and creating awareness of the physiological visual direction of the fovea. So-called Sehschulen (schools for vision) sprouted in Switzerland to which amblyopic children were admitted and treated with daily training sessions as inpatients. Pleoptics rapidly spread to the rest of

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