Van Der Hoeve Research Articles

PRODUCTION OF CATARACT IN RATS BY BETATETRALOL AND OTHER DERIVATIVES OF NAPHTHALENE

SINCE Bouchard's¹discovery in 1886 that naphthalene produced cataract in rabbits, a large number of experimental papers have been published on the subject. Relatively few authors, however, have attempted to approach the mechanism of this production of cataract by studying the cataractogenic properties of chemically related substances. Kolinski²failed to produce cataract with nitronaphthalene, β-naphthol and anthracene. Salffner³had negative results with 1,5-dioxynaphthalene, 2,3-dioxynaphthalene and 2,7-dioxynaphthalene. He found the cataractogenic properties of naphthalene greatly enhanced by phthalic acid; however, control experiments with other agents which likewise produced renal damage led him to conclude that the effect was a result of damage to the kidney, producing a delayed excretion of naphthalene or a toxic derivative. Igersheimer and Ruben⁴reported negative results with both a- and β-naphthol, while van der Hoeve⁵produced cataracts in rabbits with β-naphthol. Jess⁶observed occasional fine reversible clefts and opacities in

SCLEROMALACIA PERFORANS

IN 1934 van der Hoeve 1 reported a new disease of the eye, which he called scleromalacia perforans. He concluded that the disease was a degenerative, rather than an infectious, process and that it usually accompanied rheumatoid He accurately noted the early yellowish excrescences and the holes in the sclera which succeeded them, producing exposure of the uvea and the characteristic picture of the disease. Occasional reports appeared after this, but they were so few that one must conclude that the disease is exceedingly rare. In 1938 Verhoeff and King 2 collected 14 cases from the literature; on the basis of an analysis of these cases, with 1 of their own, they gave the first pathologic report of the condition. In their summary, they stated, Histologically, in their initial state the scleral nodules are essentially similar to the subcutaneous nodules of rheumatoid arthritis. Since 1938, 1 case has been

BLUE SCLERAS, BRITTLE BONES AND DEAFNESS

For centuries physicians have witnessed the tragic instance of a child born with multiple fractures of the bones. Lobstein, 1 in 1833, designated this condition as Ostepsathyrosis, a descriptive term derived from Greek, meaning friable bones. In 1849, Vrolik reported a similar condition, which he named osteogenesis imperfecta, implying defective formation of bone as the cause. Spurway, 2 in 1896, reported the first instance in which the condition of fragile bones (fragilitas ossium) was associated with blue scleras. Bronson, 3 in 1917, and van der Hoeve and de Kleyn, 4 in 1918, added to the syndrome the third feature, deafness. Key, 5 in 1926, referred to the syndrome as hereditary hypoplasia of the mesenchyme and called attention to the hypotonicity of the ligaments with hypermobile joints. In 1928 Bell 6 collected 75 pedigrees including 489 cases of this abnormality. The condition is not so rare as is generally thought. Recent literature 7 has shown renewed

Osteogenesis Imperfecta

Armand, in 1716, reported a case of brittle bones of a newborn infant. This is said to be the first recorded observation of this condition. By the middle of the eighteenth century 30 such cases had appeared in the literature. In 1788 Eckman described a case of hereditary fragility of bones and traced the occurrence of this disease in three generations. Lobstein, in 1833, described the condition as idiopathic osteopsathyrosis or an idiopathic type of bone fragility. Vrolik described brittle bones in 1849 and renamed the condition “osteogenesis imperfecta.” Spurway in 1896 observed a peculiar bluish color of the sclera in association with brittle bones. A more detailed observation concerning the association of these two findings was made by Eddowes in 1900, when he suggested that “the transparency of the sclerotics indicates a want of the quality or quantity of the fibrous tissue forming the framework of the various organs of the body and probably explains the want of spring or toughness in the bones of these peculiar individuals.” Hereditary deafness was reported in 1912 by Adair-Dighton but van der Hoeve and de Kleyn did not observe its simultaneous occurrence with brittle bones and blue sclera until 1916. Since these cardinal symptoms were discovered and connected, numerous isolated cases and series of cases have been reported in the literature. The clinical picture has been studied and much laboratory investigation has been carried out in an effort to solve the riddle of this disease. A limited amount of pathologic material has been reviewed. Although most authors agree on the observed facts, conclusions concerning the etiology vary, and many physicians are still basing their treatment on unproved hypotheses concerning the true etiologic factor. Terminology and Classification used in Previous Reports As knowledge of this disease increased, many different names were applied to it. Lobstein's disease (maladie de Lobstein) or, as he called it, idiopathic osteopsathyrosis, was first renamed osteogenesis imperfecta by Vrolik. Since that time it has had many other names: fragilitas ossium (Klebs and Hochsinger), brittle bones, blue sclera and otosclerosis (Eddowes's syndrome), osteitis parenchymatosa chronica, dystrophia periostalis, periosteal aplasia, and periosteal dysplasia, have all been applied to one form or another of this disease. Classifications have been based on the time of appearance of the symptoms, as follows: (1) fetal cases; (2) infantile cases; (3) adolescent cases; (4) late cases. In the classification which probably is used most widely, the cases of brittle bones are divided into two groups: (1) the hereditary type, which is called an hereditary hypoplasia of the mesenchyme with brittle bones and blue sclera, and (2) the nonhereditary congenital type, which consists of two groups, osteogenesis imperfecta congenita and osteogenesis imperfecta tarda.

POSITION OF MUSCLES AFTER OPERATION FOR STRABISMUS

Among surgeons operating for strabismus no agreement has yet been reached concerning certain questions which are of importance in the choice of operation. Almost every operator has, for reasons which seem good to him, fixed his preference on two or at most three operations. He has obtained a fair average of results with these and, in considering the factors involved, tends to accept a theory which favors the operation of his choice. Reminiscent of the former battles between the advocates of tenotomy and those of advancement is the more recent argument between the late Dr. Jameson and Dr. O'Connor on the virtues of recession versus those of cinch shortening with central tenotomy. Worth, Landolt and their followers insist on the value of advancement with the idea that the insertion of the muscle is placed nearer the cornea, while van der Hoeve, Lancaster and others are firmly convinced that the muscle

Van Der Hoeve's Syndrome : Four occurrences in three generations of one family

Van Der Hoeve's Syndrome : Four occurrences in three generations of one family

Roentgenographic Findings in the Neurocutaneous Syndromes

The subject of neurocutaneous disease (congenital ectodermoses) has received but scant attention in the roentgen literature. This may be due, in part, to its relatively great rarity on the one hand, and, on the other, to the unfamiliarity of roentgenologists with the condition. In considering the subject of neurocutaneous disease, four outstanding entities attract attention. These are: 1. Bourneville's Tuberous Sclerosis (Epiloia) 2. von Recklinghausen's Neurofibromatosis 3. Angiomatosis Cerebri, associated with Trigeminal nevus (Sturge-Weber's Disease, Parkes-Weber-Dimitri's Disease) 4. von Hippel-Lindau's Disease. Tuberous Sclerosis Tuberous sclerosis is a rare condition, which, in its full-blown form (epiloia), frequently presents the following symptomatology: 1. Epileptic seizures (grand mal, petit mal, or Jacksonian type) 2. Mental deficiency 3. Acneform butterfly facial rash (adenoma sebaceum—morbus Pringle) 4. Periungual fibromas 5. Congenital tumors of the eye (pha-coma of van der Hoeve), and of many other organs, including rhab-domyomas of the heart and hyper-nephromas of the kidney. In addition, many other congenital defects may be apparent, such as harelip, cleft palate (5), hypoplasia of the kidney and ureter, pulmonary artery stenosis, simian hands, hypertrophy of the external ears (50), high-arched palate, spina bifida, microcephaly and accessory digits (111), syndactyly, hemihypertrophy, epicanthus (23), transposition of the great vessels, and intestinal polyposis. The entity was first described by Bourneville, in 1880 (6, 7, 8). His patient was a three-year-old child who had suffered from localized convulsive seizures for the first fourteen months following birth. There was present what we now know to be a typical adenoma sebaceum on the skin of the face, although at that time the importance of the skin manifestations was overlooked. The description, however, was accurate: “acné rosacée et pustuleuse de la face; de plus éruption vésiculo-papuleuse du nez, des joues, du front; nom-breux petits molluscums à la nuque et sur les parties du cou.” At necropsy, the reason for the right-sided convulsions was discovered. The ascending frontal and parietal convolutions on the left were obliterated by plaques of sclerotic tissue. Bilateral kidney tumors were found. The author came to the conclusion that he was probably dealing with a chronic inflammatory process, an hypothesis which is no longer tenable.

PHAKOMA RETINAE AND ADENOMA SEBACEUM

Since the first publication in 1921 by van der Hoeve¹describing the association of benign circumscribed retinal tumors with tuberous sclerosis of the central nervous system (Bourneville), I have been on the watch among clinical and private patients for this curious condition. The first case corresponding at least in part to this syndrome has recently been seen (1939). For some reason, the condition, a hereditary one, seems to occur or to be observed with especial frequency in Holland, so that van der Hoeve has collected records of a large number of affected families from his own experience. He coined the name "phakoma" to describe congenital tumors arising in various tissues which were distinct from nevi by the absence of nevus cells. It is from the Greek "phakos," the term for "mother spot," or birthmark, and has nothing to do with the root word for lens. He has shown that

BRITTLE BONES ASSOCIATED WITH DEAFNESS AND BLUE SCLERAS: REPORT OF THE SYNDROME IN TWO PERSONS OF ONE FAMILY

The syndrome of brittle bones, blue scleras, relaxed ligaments and deafness is an interesting hereditary condition which to the otologist is of particular interest because of the nature of the hearing impairment and its similarity to the deafness accompanying otosclerosis. Patients afflicted with this set of symptoms are usually first seen in the orthopedic service, because of frequent and multiple fractures, and the condition is promptly recognized by the blue scleras of the patients themselves and by the histories of frequent fractures both among the patients and among other members of the family. Reports in the literature regarding brittle bones and blue scleras have been numerous, but the associated deafness was reported first by van der Hoeve and de Kleyn¹and later by Bronson.²These writers brought forth the interesting observation that persons with this syndrome have a tendency to become deaf in the third or fourth decade of life.

NECROSCLERITIS NODOSA ASSOCIATED WITH CHRONIC RHEUMATOID POLYARTHRITIS

In 1934 van der Hoeve 1 reported 4 cases of a new disease which he had named scleromalacia perforans. Two of the cases were his own and the other 2 belonged to Rochat, who had independently named the condition scleritis necroticans. Scleromalacia perforans was described as a degenerative disease, accompanied by little or no inflammation and characterized by the appearance of multiple holes in the sclera. Some of the holes were covered by conjunctiva. Others exposed bare uvea to the outside. Adjacent holes often coalesced. The patients in all 4 cases were elderly. Three had advanced chronic rheumatic polyarthritis. One never had had any symptoms of arthritis. Van der Hoeve also mentioned that he had heard of the existence of 2 more cases of this disease, although he had not seen either of the patients. Both were reported as being elderly, and 1 suffered from chronic arthritis. Apparently all 4

The anniversary dinner

The 275th Anniversary Dinner of the Society was held at the May Fair Hotel on 30 November 1937. The health of the Society was to have been proposed by the Lord President of the Council, Viscount Halifax, but a bereavement in his family prevented him from attending, and his place was taken at short notice by the Chancellor of the Exchequer, Sir John Simon. The President of the Amsterdam Academy of Sciences, Professor J. Van der Hoeve, who had accepted an invitation to be the Society’s foreign guest of honour, was unfortunately prevented by illness from coming to England. Rising to propose the toast—'The Royal Society of London'— Sir John Simon said : ‘You will share with me the deep feeling of regret that Lord Halifax, who was to have come here to-night in order to propose the toast of the Royal Society, is kept away by a bereavement in his family. But uno avolso non deficit alter —which may be freely translated "As the Lord President of the Council cannot come, Simon must do his best." It is thus that the solidarity of the National Government is demonstrated.

RETINAL TUMORS IN TUBEROUS SCLEROSIS

Involvement of the retina in cases of tuberous sclerosis escaped notice until 1921, when van der Hoeve,¹of Amsterdam, Netherlands, published a clinical report describing six cases of tuberous sclerosis in which tumors of the retina were observed through the ophthalmoscope. Since then a number of clinical reports of this condition have appeared, indicating that such retinal tumors are not uncommon and that distrubance of vision and the ophthalmoscopic examination may give the first clue as to the disease. The case presented here is the sixth in which microscopic study of the retinal tumor is described and the first to be reported in this country in which such a study was made. REVIEW OF THE LITERATURE The term tuberous sclerosis, or tuberose sclerosis, was first used by Bourneville, who, in a series of articles published from 1880 to 1898, described a rare form of cerebral sclerosis observed at

SCLEROMALACIA PERFORANS

In the January 1934 issue of theArchivesDr. J. van der Hoeve. 1 of the university of Leyden, published a report of a series of four cases under the title of scleromalacia perforans. During the course of this report he stated that so far he had been unable to find any published account describing a similar condition and therefore concluded that his cases were the first recorded instances of a condition which he described elsewhere as follows: A small slightly elevated inflammatory nodule appeared under the conjunctiva at a distance of about 10 mm. from the limbus. This nodule underwent necrosis at the center, discharged a yellowish cheesy material and then disappeared, leaving the deep blue color of the uvea in evidence beneath. This process repeated itself and showed a tendency to travel around the circumference of the eye in ring fashion and exhibited no disposition to involve the

RELATION BETWEEN BLUE SCLERAS AND HYPERPARATHYROIDISM

The clinical entity consisting of blue scleras, osteoporosis with spontaneous fractures and deafness and the dominant hereditary transmission of the syndrome have long been known to ophthalmologists and pediatricians. The association of blue scleras with fragilitas ossium was noted by Ekman (1788), Lobstein (1833) and von Ammon. Van der Hoeve and de Kleyn 1 and Bronson 2 independently included hereditary deafness (which, according to some observers, may develop only after the age of 20) as a part of the entity. German authors commonly refer to this symptom complex as the van der Hoeve and de Kleyn syndrome, while in the English literature there is a tendency to link the entity with the name of Lobstein. It is not pertinent to the purpose of this article to delve into the early historical facts or to decide to which authorities credit should be given for advancement in the knowledge of

Fistula at Limbus (Scleromalacia Perforans): Report of a case

An annoying subconjunctival bleb, of more than one year's duration, near the limbus, came under observation of the author in March, 1932. Several diagnoses were suggested by colleagues and no pertinent etiologic factor was discovered. An operation, intended primarily for biopsy, disclosed a small opening near the limbus from which exuded aqueous humor. The patient had had rheumatic arthritis since childhood. No parallel cases were found in the literature except some of those reported by Van der Hoeve which he called scleromalacia perforans. Read before the St. Louis Ophthalmic Society, January, 1934. An annoying subconjunctival bleb, of more than one year's duration, near the limbus, came under observation of the author in March, 1932. Several diagnoses were suggested by colleagues and no pertinent etiologic factor was discovered. An operation, intended primarily for biopsy, disclosed a small opening near the limbus from which exuded aqueous humor. The patient had had rheumatic arthritis since childhood. No parallel cases were found in the literature except some of those reported by Van der Hoeve which he called scleromalacia perforans. Read before the St. Louis Ophthalmic Society, January, 1934.

O'CONNOR CINCH SHORTENING OPERATION FOR HETEROTROPIA AND HETEROPHORIA

It is well known that many procedures that are now standard practices in medicine and surgery were notoriously slow in being adopted and, conversely, that innovations are occasionally widely accepted, only to be quickly discarded by the medical profession. O'Connor operation in its original form was described in Journal of the American Medical Association in 1912. originator modified his technic and reported forty-two operations in 1916. Since that time several additional reports have been made by Dr. O'Connor. Several papers on the subject have appeared, but with the exception of a brief reference in Dr. Luther Peter's book on The Extra-Ocular Muscles and a brief description by van der Hoeve in the GraefeSaemish Handbuch der gesamten Augenheilkunde, they have all been written by oculists in San Francisco. There has been no widespread interest in the operation, nor has it been adopted by any great number

News and Notes

SOCIETY NEWS <b>International Congress of Ophthamology.</b>—The Fourteenth Meeting of the International Congress of Ophthalmology was held in Madrid, Spain, during the week of April 16, 1933. The session was opened on Sunday morning, April 16, by an address by the president of the congress, Professor van der Hoeve. This was followed by an address of welcome by Professor Marquez, at the conclusion of which he read a message of greeting and felicitation from Prof. S. Ramón y Cajal. The official delegates were then introduced as follows : France, Prof. Félix Terrien; Great Britain, Sir John H. Parsons; United States of America, Dr. Walter H. Parker ; Italy, Prof. G. Ovio ; Germany, Prof. Emil Krückmann ; Belgium, Prof. Marnix van Duyse; Spanish America, Professor Demaría; Scandinavia, Professor Nordenson; Japan, Prof. Chuta Oguchi ; Portugal, Professor Sousa; various other countries, Prof. Emil von Grosz, and Poland, Professor Szymanski. Following the reception of the official

THE AGE OF PATIENTS OPERATED ON FOR SENILE CATARACT

During a recent visit to some of the continental clinics, I was impressed by the disparity in age between the patients operated on for senile cataract there and at home. In order to determine whether that was merely an impression or whether such a disparity really existed, I sent a letter to a few of the leading ophthalmic surgeons in various parts of the world asking for the ages in their last 500 or 100 cases of senile cataract extraction, excluding juvenile, traumatic and complicated cataracts. The following surgeons were kind enough to reply, at the same time authorizing me to utilize their statistics: Benedict of Rochester, Derby of Boston, Ellett of Memphis, Elschnig of Prague, Kalt of Paris, Knapp of New York, Meller of Vienna. Sinclair of Edinburgh, Van der Hoeve of Leiden, Wessely of Munich, and Wright of Madras. A compilation of the statistics gleaned from these sources

Brittle Bones And Blue Sclerae. Report Of A Case With Glycosuria

Article1 January 1930Brittle Bones And Blue Sclerae. Report Of A Case With GlycosuriaHAROLD BOWCOCK, M.D., GEO. T. LEWIS, Ph.D.HAROLD BOWCOCK, M.D.Search for more papers by this author, GEO. T. LEWIS, Ph.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-3-7-700 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe recognition of the familial occurrence of brittle bones and blue sclerae is credited to Eddowes1, although this association of abnormalities was reported by Spurway2four years earlier. Later, Bronson3and Van der Hoeve and de Kleijn4called attention to the hereditary association of otosclerosis and labyrinthine disease with deafness in the affected families. The syndrome is usually transmitted by the female, though it may be transmitted by the male. A number of affected families has been reported; the articles by Bronson3and Key5are accompanied by extensive bibliographies. Since the latter publication, additional families have been studied by Moller6...Bibliography1 EDDOWES A: Dark sclerotics and fragilitas ossium. Brit. M. J., 2:222, July 28, 1900. Google Scholar2 SPURWAY J: Hereditary tendency to fracture. Brit. M. J., 2:844, Sept. 26, 1896. CrossrefMedlineGoogle Scholar3 BRONSON E: On fragilitas ossium and its association with blue sclerotics and otosclerosis. Edinburgh M. J., 18:240, April 1917. Google Scholar4 VAN DER HOEVEDE KLEIJN JA: Blauwe sclera. Nederl. Tijdschr. v. Geneesk. 1:1003, Mar. 31, 1917. Quoted by Key. Google Scholar5 KEY AJ: Brittle bones and blue sclera. Arch. Surg. 13, 523, Oct. 1926. CrossrefGoogle Scholar6 MOLLER E: The family tree of a patient with Eddowes' syndrome. Act. Med. Scandinav., 59, 223, Aug. 1928. Google Scholar7 PAAL H:Ueber blaue Scleren. Klin. Woch., 8,1304, July 9, 1929. CrossrefGoogle Scholar8 HERRMAN C: Blue sclerotics associated with brittle bones. Am. J. Dis. Child., 9,203, Mar. 1915. Google Scholar9 OSTHEIMER M: Fragilitas Ossium. J. A. M. A., 63, 1996. Dec. 5, 1914. CrossrefGoogle Scholar10 GUTZEIT R: Ueber blaue Sklera and Knochenbruchtigkeit. Klin. Monatsbl. f. Augenh., 68, 771, 1922. Google Scholar11 HEIN BJ: Osteogenesis imperfecta with multiple fractures at birth. An investigation with special reference to heredity and blue sclera. Jour. Bone and Joint Surg., 10, 243, April 1928. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: HAROLD BOWCOCK, M.D.; GEO. T. LEWIS, Ph.D.Affiliations: PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byPathogenetische Fragen bei der Osteogenesis imperfectaOsteogenesis imperfecta and odontogenesis imperfectaEinzelne Augenkrankheiten mit mono- und polyglandulären endokrinen ZügenDas Krankheitsbild der blauen Skleren, abnormen Knochenbrüchigkeit und Schwerhörigkeit (Adair-Dightons Syndrom)Osteogenesis imperfecta congenita et tarda 1 January 1930Volume 3, Issue 7Page: 700-706KeywordsBoneDeafnessGenetic diseases ePublished: 1 December 2008 Issue Published: 1 January 1930 PDF downloadLoading ...

THE INTERNATIONAL CONGRESS OF OPHTHALMOLOGY

Twenty years ago, the twelfth International Congress of Ophthalmology was held in Lucerne. The thirteenth, originally planned for St. Petersburg in the summer of 1914, was prevented by the World War. Ten years elapsed following the armistice before it was possible to gather the ophthalmologists of the world together. The stage was set in Amsterdam, the time September 5 to 13, and except for an occasional shower the sun shone brightly each day. A vast amount of preliminary spade work had been done by the Dutch colleagues Zeeman, Marx, van der Hoeve, Rochat, Weve, Mulock-Houwer, Roelofs and many others. There was a central registration hall at the Kolonial-Institut, in which building the exhibits were installed and many of the meetings held. Here each member had his own private box in which all notices and correspondence were placed each day, here was a banking office, the executive offices of