Buphthalmos: early glaucoma history
2010; Wiley; Volume: 89; Issue: 6 Linguagem: Inglês
10.1111/j.1755-3768.2009.01783.x
ISSN1755-3768
Autores Tópico(s)Cerebral Venous Sinus Thrombosis
ResumoGlaucoma as understood today could not have been detected before the means to see the optic nerve and reliably measure the intraocular pressure were available after the mid-1850s. One form, however, manifested itself as a visible enlargement of the globe and was thus recognized since antiquity. This article traces the nomenclature, signs and symptoms, and treatment of buphthalmos from the ancient Greeks to the second half of the 19th century, when the present methods began. As understood today, glaucoma is diagnosed and often defined as progressive optic nerve atrophy mostly attributed to elevated intraocular pressure (IOP); it could therefore not have been detected prior to the introduction in the 1850s of means to see the optic nerve, test visual fields, and reliably measure the IOP. Historians were thus limited, first, to tracing the name itself, understood to mean green/blue, which directed them to search for blinding diseases manifested by this eye discoloration; secondly, to discovering who first wrote about loss of vision in hard eyes. Actually, the history of glaucoma, its diagnosis, pathogenesis, and treatment, goes farther back than usually told. The type of glaucoma termed buphthalmos was easily diagnosed since early times, for it manifests itself by a visible enlargement of the globe, mostly, but not exclusively (Alme et al. 2008; Al-Shahwan & Khan 2006) at birth or soon thereafter. Two circumstances are prerequisite to enlargement of the globe. First, its coats, the cornea and sclera, must be distensible. This is natural in embryonic and early life, and pathological by chronic inflammation or necrotizing infection. The latter are seldom seen today, but were not unusual in centuries past. Secondly, the globe's contents must increase by augmentation of one or more of either: (1) aqueous, (2) vitreous, (3) blood, (4) pus, (5) new grows (tumour). The name buphthalmos means in Greek 'ox-eyed', but who first formally coined it is hard to ascertain, and could have simply been a common descriptive expression similar to 'cross-eyed'. Clinical diagnoses were early made by simple inspections, and in buphthalmos, the striking view was of a prominent eye, protruding forward and separating the lids. The distinction between a large globe and a normally sized globe protruding from the orbit was not recorded till the 18th century. The cause of the phenomenon, the aetiology, remained obscure, and hence the names given to this appearance varied and changed over time. Aristotle already used the term exophthalmos, probably meaning prominent eyes, without any allusion to abnormality. He also used the term 'hygrophthalmos' to vaguely denote moistness. Galenic literature used the terms 'proptosis', 'prolapsus', and later authors used 'procidentia',' ecpiesmos', or 'exophthalmos'. Ambroise Paré (1517–1590) called the enlarged globe 'exophthalmia' (Hirschberg 1887) but is also quoted as having written: 'Eye of ox (βoύςόϕταλμός) is a disease of the eye where it is big and eminent'. Later in the 18th century, the terms 'hydrophthalmia' and 'hydrophthalmus' were common. With the invention of the ophthalmoscope and the tonometer in mid-19th century, the term buphthalmos was generally reserved for a large globe with glaucoma, distinct from a large eye where the IOP and the optic nerve were normal, such as megalophthalmos, megalocornea, ocular hyperplasia, or progressive myopia. Because the vast majority of cases nowadays are diagnosed before the first year of life, the temporal terms 'congenital' and 'infantile' glaucoma are also applied to cases where the globe is not enlarged (Biglan 2006). Celsus (53 BC–7 AD) (Celsus 1840) mentioned proptosis when dealing with inflammation: 'But sometimes high inflammation breaks out so violently that it propels the eyes from their sockets; this the Greek call proptosis, because the eyes fall forward'. Magnus (1901) interpreted this to imply buphthalmos, but cautioned that the word proptosis should not be equated with modern panophthalmitis, but rather that the term proptosis in Celsus' time encompassed all the different forms of exophthalmia. It seems that in antiquity, two forms of prominent and enlarged eyes were noted: one with inflammation and pus, and the other without. Celsus recommended first the usual medical treatment: 'If by these means, the eye be not restored to its natural position, but remains prolapsed as before, we may rest assured the sight is lost; and that the necessary consequence will be, that the eye will grow hard, or be converted into pus.' The word 'hard' [scleros] as a state of the globe appears in Celsus again when talking about its treatment. It is, however, unlikely that the eye in those days was actually touched to determine its elastic state, a procedure not used till the 19th century. Nevertheless, here we have a description of a prominent hard eye to be much later diagnosed as buphthalmos. In the cases of prominent eyes, Celsus recommended the following: 'If the suppuration shew itself in the temporal angle, the eye ought to be incised, that by letting out the pus, the inflammation and pain may cease, and the internal tunics retreat; so that the face may afterwards be less disfigured… But if it has grown hard, and has become mortified without turning to pus, so much of it as constitutes an unseemly prominence must be cut away; this may be done by taking hold of the external tunic with a hook, and by making an incision below this by means of a knife: then the same medicines are to be injected till the pain be entirely gone.' This method of excising the anterior portion of the globe was understood by Magnus to have been an antecedent to modern exenteration. Some believed that Celsus applied the procedure only to anterior staphylomata, but actually he dealt with staphyloma and its treatment separately. Not much had been added to this understanding in the Arabic literature of the Middle Ages. Ali-Ibn-Isa in the 10th century (Wood 1936), prior to the chapter 'On Atrophy of the Eyeball', wrote 'On Protrusion of the whole Eye: This is a kind of "goggle-eye" outwards, the eyeball remaining in that abnormal position'. George Bartisch (1535–1607) in his historically famous book (Bartisch 1583) said: 'It happens that a person's eye is excessively and forcefully distended forward, called ekpiesmos, egresio, exitus oculi, or prolapsus (of which I have seen many, and also taken out), and it becomes very big, hideous, and repugnant to look at, and cannot be covered or hidden, as seen in the following figure (Fig. 1).' A protruding enlarged eye according to Bartisch. Bartisch lists several causes, but his sole innovation relating to our subject was his treatment by enucleation. Two years after Bartisch published his book, Jacques Guillemeau (1560–1613) published his 'Traite de maladies de l'oil' (Guillemeau 1585) The book immediately became popular, first, because Guillemeau was a world renown surgeon who studied with the famous Paré and Riolan, whereas Bartisch was an itinerary barber-surgeon who may have been illiterate. Secondly, it was well written in simple instructive prose augmented by pictures of the needed surgical instruments. It was already translated into English by Anthony Hunton 2 years after its appearance in Paris (Guillemeau 1587) including a small treatise by William Bailey. The first chapter in the second section was titled: 'Of the eie that falleth out of the place, which disease is called in Greek ecpiesmos or proptosis, and in Latin prolapsus, exitus, expression, exertio. Also of the Oxe eie, or great eie, which is in Greeke named exopthalmia, in Latine oculi prominentie, of the common sort goggle eye.' [sic] Exactly, the same words, with altered spelling, appeared again in 1622 in a book authored by Richard Banister (Banister 1622) and another by Sir William Read (Read 1708). An important original contribution by Guillemeau was the distinction between some loss of aqueous, which was harmless, and the loss of vitreous, which led to blindness. He also appears to have revived, after Celsus, therapeutic paracentesis of the globe. Guillemeau distinguished external from internal causes, the former were mostly traumatic or straining, the latter excess of fluids: 'For if the eye falleth out through the abundance of humours, it appears greater and more swell'd than if it fall out by strangling and choaking, by straining or blowing (if there be no other repletion of humours) albeit there is in both great protubernacy.' A clear case of buphthalmos was published by Dawbeney Tuberville of Salisbury (1612–1696) who wrote (Tuberville 1685): 'Not long after this, a young man (my patient) had an eye as large as a hen's egg, very fair, without blemish, rheum, or redness, and his sight was pretty tolerable. I judged these symptoms to proceed from thin humours fallen on the eye and extending its coats. I cured this distemper by applying drying medicines to the head and eyes, and making an issue in nucha, appello morbum oculorum Bovinum, sive oculi Hydropem.' No substantial insight into the pathogenesis of buphthalmos came until the beginning of the 18th century, when improved knowledge of anatomy and more careful diagnostic examinations lead to differentiation between a globe that protruded because it was large from one which was simply expressed from the orbit. Saint-Yves (1748) already expressed a modern understanding of glaucoma mechanism: 'As to the first cause, which augments the size of the eye, it is manifest, if the channels destined to carry back the aqueous humor, or the pores through which it escapes, become obstructed, whilst the vessels which supply it are in their natural state; it is manifest, I say, that the augmentation of this humor will necessarily occasion that [augmentation] of the globe'. In his book (Saint-Yves 1722) Charles Saint-Yves (1667–1733) definitely, and probably for the first time, separated the chapter on 'The excessive size of the eye's globe' (Saint-Yves 1748) from the chapter on diseases that expel the globe from the orbit, as well as from eyes that are naturally large. He distinguished two forms of abnormally large globes, one caused by excessive aqueous humour, which was not dangerous, and the other by fleshy carcinomatous growth, which was often mortal. The first kind was commonly bilateral, but in the second form, the eye may grow to 3–4 times its natural size, and this was mostly unilateral. The first separate monograph devoted to hydrophthalmos (buphthalmos) seems to having been written by Johann Ernst Schaper (1668–1721) whilst he was a professor of medicine in Rostock (Schaper 1713). He refers to the work by Anthon Nuck of 1695 who described afferent and efferent aqueous vessels and paracentesis by corneal incision. Most authors, however, refer to the dissertations by the famous professor in Tübingen, Burchard David Mauchart (1696–1751). He listed the signs and symptoms of 'hydrophthalmia' as tension, deepening of the anterior chamber, cloudy cornea, visual disturbances, and headaches (Mauchart 1744a). In 1781, Edward Ford reported the following case of hydrophthalmia treated by paracentesis (Ford 1781): 'Mary Bethell, aged twelve years…lost the sight of her left eye by the small pox; and in consequence of a blow received some time after, had a complete opacity of the cornea, attended with a dropsy of the aqueous humour.' In the second volume of his textbook, Georg Joseph Beer (1763–1821) detailed the signs and symptoms of excess aqueous (Beer 1817), which today would be diagnosed as glaucoma. First, enlargement of the corneal circumference, up to four times its normal size, and with it widening of the anterior chamber. The pupil is immobile in mid position. The eye becomes presbyopic, with impaired but not total loss of vision. 'The movements of the globe become more difficult the larger the eye, which always feels harder [emphasis added]'. The patient does not feel actual pain in the eye, but rather a sensitive pressure, a bothersome tension and heaviness of the whole globe. In buphthalmos because of excess vitreous, he described signs and symptoms of acute glaucoma: the globe is elongated, anterior chamber is shallow, vision is soon completely lost, 'and the globe feels like an egg-shaped flint stone'. The pain is from the start intolerable, and affects the whole half of the head and neck. It drives the patient almost mad, and he demands paracentesis from the surgeon, 'or, as I have seen, sticks a knife into his eye in his rage'. Most later authors, including William Mackenzie in his classical book and its later editions (Mackenzie 1830), repeated almost verbatim Beer's classification, signs and symptoms, and modes of treatment. Beer himself emphasized the distinct forms of 'what some have also called Elephantiasis oculi' (Beer 1817, p 222). 'Exophthalmia' is dealt with in a chapter on the consequences of ocular inflammations where the globe was already mostly disorganized. On the other hand, in his section 'On Hydrops in general, termed Hydrophthalmia, Hydrophthalmus, Hydrophthalmos; also Ox-eyed, Buphthalmus, Buphthalmos', he classified three forms according to their aetiology: (1) Excessive aqueous humour. (2) Excessive vitreous. (3) Superabundance of both. Hydrops in the eye arises in the same manner as elsewhere in the human body; based on disproportion between secretion and absorption of some fluid matter, which may occur either in the afferent or efferent vessels… The final cause of this disproportionate balance, as is the case in other ocular diseases, remains in complete darkness. The hereditary nature of buphthalmos and its bilaterality were already seriously discussed (Bell 1932) early in the 19th century, and the aetiology as a form of glaucoma was finally established with the aid of the ophthalmoscope and the tonometer. It was further refined with the introduction of gonioscopy, which placed the site of the causative pathology mostly in the anterior chamber angle. Midway between the palliative medical treatments and radical enucleation stood drainage of intraocular fluids by incision. However, drainage of aqueous was feared ever since Galen warned that its loss led to blindness, until the end of the 16th century when this had been proven false (Mark 2010). In cases of blind 'dry and withered' eyes Guillemeau (1587) recommended incision and drainage: 'And it is expedient to open the part towards the temples of the head where suppuration appeareth, to the end that the filthy flegmatick matter being voided the inflammation and pain may cease: Notwithstanding this, let the coats thereof remain safe and put them again into the place, to avoid thereby the deformity which might appear in the face.' A century later, Anton Nuck seems to have been the first to record (1690) paracentesis of the cornea specifically as a therapeutic measure for the relief of hydrophthalmos (Nuck 1695). He is cited by Mauchart (1744a) in his paper and by Hirschberg (1911). After mid-18th century, when opening of the anterior chamber for extraction of cataracts was introduced by Daviel, and with it harmless loss of aqueous, paracentesis also became an acceptable operation. Tuberville of Salisbury was reported to have performed paracentesis for hydrophthalmos (Hirschberg 1984), whilst some surgeons preferred scleral incision into the vitreous with a knife or an especially invented trocar. Edward Ford (Ford 1784) made a large incision through the cornea with a cataract knife to drain some aqueous, and then reintroduced the knife to drain some vitreous. 'I shall only observe that in three weeks the disease returned'. Three years later, he reported on an improved operation in cases were vision was already lost, 'the operation being merely to remedy the inconveniences occasioned by the increased bulk of the eye'. He used 'The seton needle armed with six threads of white sewing silk' and passed it behind the limbus across the posterior chamber from one side to the other. The threads were left in the eye for several weeks (Fig. 2). He reported on two patients cured in this manner. The operation had previously been described by Mauchart (1742), who used cotton thread instead of silk. Hydrophthalmos and the seton for its treatment by Ford. In cases of buphthalmos caused by excess aqueous, Beer cut into the inferior cornea and drained the anterior chamber, often repeatedly for weeks. One hundred years later, this procedure was still in vogue (Török & Grout 1913): 'It is also advised in juvenile glaucoma (buphthalmos), where it is said that by repeated paracentesis, the eye becomes accustomed to the decrease of tension, and the iridectomy may be performed later with less danger.' Although not curative, it was the proper palliative to reduce the signs and symptoms. In cases caused by excess of vitreous, Beer warned against its drainage through a pipe inserted in the sclera, which inevitably led to loss of the eye and often of life. Instead, he performed a corneal incision, as for cataract extraction, and through it, he removed the lens and some vitreous. 'However, to prevent the eye from refilling with water, one must finally excise a small part of the corneal flap, whereby the eye is kept continually empty'. An early treatment of glaucoma by fistula. After the introduction of gonioscopy in the 1930s, goniotomy became a favourite operation for buphthalmos, in addition to the other glaucoma surgeries, such as cyclodiathermy and sclerotomy. The history of buphthalmos provides insight into glaucoma prior to the current understanding of the disease that began with the introduction of ophthalmoscopy and tonometry. The first step towards progress was departure from Galen's and Celsus' anatomy and physiology, particularly the notion that the aqueous was essential to vision, and its loss led to blindness. A gradual conceptual shift from inflammatory causes of an enlarged globe to imbalance between secretion and outflow of aqueous emerged, and with it treatment by paracentesis and fistulizing procedures. This paper was presented in part at the Cogan Ophthalmic History Society, March 2009.
Referência(s)