William F. Hoyt

Blindness caused by photoreceptor degeneration as a remote effect of cancer.

Three postmenopausal women developed photoreceptor degeneration one to four months preceding or following discovery of an anaplastic tumor. Two patients had transitory visual obscurations and bizarre visual sensations. Ring scotomas progressed to severe visual field loss. Retinal arteries were markedly narrowed. Electroretinograms revealed almost total absence of response in one patient, and another complained of the recent onset of night blindness. In all three patients severe degeneration of the photoreceptor cells associated with melanophagic activity was shown histologically. In two patients neuropathologic examination from the retinal bipolar cells to the occipital cortex revealed no significant alterations.

Optic glioma of childhood. Natural history and rationale for conservative management.

Controversy surrounds the management of childhood optic gliomata in the anterior visual pathways. This stems from insufficient knowledge of their natural history, varying definitions of their cytology, preoccupation with surgical indications for their excision, and lack of agreement regarding the role of radiation therapy. Although long survivals have frequently been reported, optic gliomata continue to be treated by surgical measures that often increase their morbidity. The first requisite for rational management of any slow-growing tumour is knowledge of its natural history. This report documents the long-term clinical behaviour of optic gliomata seen in 36 patients at the University of California Medical Center in San Francisco, and formulates a rational policy for their treatment. 23 of these patients were seen within the last io years and thirteen within the preceding 30 years. The follow-up periods range from 3 to 41 years.

Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases.

Transsphenoidal and transethmoidal encephaloceles are rare but important lesions that cause various signs and symptoms, some characteristic and some misleading. Their diagnosis, both clinical and radiologic, may be difficult. Failure to recognize these lesions has in the past led to surgical misadventure, often resulting in meningitis and death. We report here clinical and neuro-radiologic observations in 3 cases of transethmoidal encephalocele and in 5 of transsphenoidal encephalocele. Six of the 8 patients were studied at the University of California Hospital. Incidence of Basal Encephaloceles In their series of 546 cases of spina bifida and cranium bifidum, Ingraham and Swan (16) noted 84 encephaloceles. This figure represented an incidence of 15 per cent of all herniations involving the brain, spinal cord, or meninges. About 75 per cent of these encephaloceles were in the occipital area and 10 per cent in the parietal area. Anterior encephaloceles involving the nasofrontal or frontal area of the skull...

Current management of amaurosis fugax

We present a consensus on the pathophysiology, etiology, diagnosis, and treatment of amaurosis fugax. The phenomenon is defined and described, and the roles that extracranial and ocular vascular diseases play are discussed. Nonvascular ophthalmic and neurologic disorders that can be confused with amaurosis fugax are listed, and an algorithm for evaluation (which includes ophthalmic examination, laboratory studies, and noninvasive carotid artery studies) is given. Treatment of atherosclerosis, carotid artery disease, and other causes of amaurosis fugax are also discussed.

Homonymous hemioptic hypoplasia. Fundoscopic features in standard and red-free illumination in three patients with congenital hemiplegia.

Congenital lesions of a cerebral hemisphere may involve its optic tract primarily or transsynaptically. Such lesions produce a distinctive retrograde hypoplasia in each optic nerve and retina (Fig. I, overleaf) which we have identified ophthalmoscopically and have named homonymous hemioptic hypoplasia. This report describes and illustrates fundoscopic features of this neuro-ophthalmological anomaly viewed in standard and red-free illumination in three Caucasian patients with cerebral hemiatrophy.

Unilateral Retinocephalic Vascular Malformations

SummaryUnilateral vascular malformations in the retina, brain, and parts of the face, described clinically by Bonnet, Dechaume, and Blanc (1937) and by Wyburn-Mason (1943), comprise a rare but clinically distinct disorder. A total of 22 cases of retinocephalic vascular malformations have been documented angiographically or at necropsy. This study records clinical and roentgenographic features in three additional patients and reviews the findings in all 25 cases.RésuméDes malformations vasculaires unilatérales de la rétine, du cerveau et en partie de la face ont été décrites cliniquements par Bonnet, Dechaune et Blanc (1937) et par Wyburn-Mason (1943). Les auteurs font la revue de la littérature de 22 cas et apportent leurs constatations dans 3 cas.ZusammenfassungEinseitige Gefäßbildung im Bereich der Retina, des Gehirns und im Gesicht wurde klinisch von Bonnet, Dechaume und Blanc (1937) und von Wyburn-Mason (1943) beschrieben. Es handelt sich um eine seltene Erkrankung. 22 Fälle dieses Leidens sind bis jetzt angiographisch oder bei der Autopsie nachgewiesen worden. In der vorliegenden Arbeit werden die klinischen und röntgenologischen Zeichen von 3 zusätzlichen Patienten wiedergegeben. Gleichzeitig werden die Beobachtungen von allen 25 Patienten zusammengestellt.

Intrapapillary and Peripapillary Hemorrhage in Young Patients with Incomplete Posterior Vitreous Detachment: Signs of Vitreopapillary Traction

PURPOSE The authors describe a benign condition characterized by intrapapillary and subretinal peripapillary hemorrhage, incomplete posterior vitreous detachment with persisting attachments to the disc, and preservation of optic nerve function in young patients. METHODS Eight patients 11 to 42 years of age with no or mild symptoms (blur, spot, or smudge) were referred for disc hemorrhage; seven of these patients were Asian. All underwent complete ophthalmologic examination, including detailed slit-lamp microscopy; particular attention was paid to vitreous attachments. RESULTS Superficial hemorrhage occurred predominantly in the superior hemidisc and was often striking in appearance. Subretinal hemorrhage occurred at the superonasal disc margin in six patients and was centered inferonasally in two. Discs were generally small, mildly dysplastic, and tilted; all were mildly elevated. The posterior vitreous body was separated from the retina but remained attached to the disc. Six patients had subtle visual field abnormalities in the involved eye. The hemorrhages resolved without sequelae or impairment of vision. During a 6-month follow-up, no patient progressed to complete vitreous detachment, retinal tear, or retinal detachment or required surgery to release traction. CONCLUSION The authors postulate that vitreopapillary traction traumatized disc vessels, causing hemorrhage in and around the disc. The superior hemidisc received the shearing force of detachment, which tore superficial vessels; transmission of the force through the retina caused subretinal bleeding. Posterior vitreous detachment remained incomplete because of tenacious vitreopapillary attachments. Mildly dysplastic discs, as in the young patients with myopia reported here, may have unusual vitreous attachments, predisposing them to the occurrence of and trauma from premature vitreous separation. The condition described is benign and requires no further evaluation or intervention.

Paralytic pontine exotropia A sign of acute unilateral pontine gaze palsy and internuclear ophthalmoplegia

Paralytic external deviation of one eye with horizontal immobility of the fellow eye specifies an acute brainstem lesion involving the paramedian pontine reticular formation and the medial longitudinal fasciculus on the side opposite the deviated eye. This paralytic pontine exotropia is distinguished from other exotropias. The deviated (exotropic) eye shows abduction nystagmus during attempts to move it further laterally, and extreme slowness of adduction saccades. Normal midbrain ocular motor functions are evidenced by intact vertical gaze, convergence, and pupillary constrictor reflex activity.

OPTOCILIARY VEINS, DISC PALLOR AND VISUAL LOSS: A triad of signs indicating spheno-orbital meningioma

Four middle‐aged women presented with unilateral long‐standing blindness. All four had a poorly demarcated and atrophic optic disc, and prominent optociliary veins. All had meningioma of the cranio‐orbital junction. It is postulated that the optociliary veins, in this setting, indicate indolent tumor growing in the distal perioptic meninges.

Magnetic Resonance Imaging Signs of Optic Nerve Gliomas in Neurofibromatosis 1

We reviewed the magnetic resonance images of four children with neurofibromatosis 1 and orbital optic gliomas. The images showed double-intensity tubular thickening characteristic of perineural arachnoidal gliomatosis, elongation of the nerves, and downward kinking of the nerves in the midorbit. We believe these findings typify the orbital gliomas in patients with this disease.