PURPOSE: To report a case of Gorham disease, also known as vanishing bone disease, of the orbit. DESIGN: Interventional case report. METHODS: A 43-year-old Caucasian woman developed a depression in her left temple, pulsation in her left upper eyelid and temple, and bulging of the left upper eyelid and headache. Computerized tomography revealed a lytic lesion in the roof of the left orbit. RESULTS: Intraoperative examination of the lesion revealed a bony defect in the left orbital roof. Surgical intervention resulted in improvement of her symptoms, and pathology failed to reveal evidence of malignancy or other benign neoplastic process. CONCLUSION: Gorham disease should be considered in the differential diagnosis of lytic bony lesions of the orbit.

We report on a patient suffering a branch retinal artery occlusion immediately following percutaneous transluminal coronary angioplasty. Balloon angioplasty may produce ocular embolic events consistent with previously reported neurological complaints.

Three patients aged 15 to 40 years with fibromatosis of the periorbital region were studied. A history of acute onset, preceding trauma, and pain associated with a palpable mass were typical features. Rapid recurrence followed surgical excision. Misdiagnosis is common in this benign disease and may lead to unnecessary examinations for malignancy. Surgical excision or debulking of these masses is indicated for diagnosis and pain relief, but long-term follow-up indicates that these lesions will often spontaneously resolve with time.

High degrees of astigmatism are common in infants with hemangiomas but have not been well documented with other adnexal masses. We reviewed records of 65 patients (69 eyes) with chalazions, epibulbar or orbital dermoids, hemangiomas, and dacryoceles. Astigmatism greater than +1.25 diopters (as high as +5.50 diopters) was most commonly associated with dacryoceles (eight of 12 eyes) and with hemangiomas (14 of 17 eyes). Plus cylinder axes were consistently oriented toward the lesion, and astigmatism tended to resolve with resolution of the lesions. Only one of the 17 eyes with dermoids and two of the 23 eyes with chalazions had astigmatism. Anisometropic amblyopia has been a prominent concern in the treatment of infants with hemangiomas. Patients with dacryoceles may be at similar risk. Repeated cycloplegic refractions are important in determining appropriate treatment of adnexal masses.

Twenty-three children (16 girls, 7 boys, aged 6–17 years) who presented with the specific complaint of blurred vision were diagnosed as having functional visual loss. Symptoms were intermittent in seven children. Associated signs and symptoms were common and included headaches, visual field loss, diplopia, micropsia, voluntary nystagmus, and spasm of the near reflex. Our treatment consisted of reassurance and follow-up. Resolution of symptoms occurred within 24 hours in one third and within two months in three-quarters of our patients. Parental support and encouragement were associated with more rapid resolution. Recurrence of symptoms and late onset of somatic complaints were rare. Conflicts related to family or school environment were common. Four children had been sexually or physically abused. Our experience suggests that, regardless of the duration or severity of symptoms, functional visual loss in children can usually be treated with reassurance. We believe that psychiatric referral is not necessary for most patients. Sexual or physical abuse should be considered as a possible predisposing factor.

Convergence insufficiency was encountered following closed head trauma in 23 patients. The most common complaints included difficulty reading and diplopia at near. The severity of the head trauma varied. There was no correlation between the severity of the head trauma and the severity of the convergence insufficiency. A normal near point of convergence was encountered in six of the 23 patients; all six patients had abnormal convergence reserves when measured with prisms. Treatment consisted primarily of convergence exercises and prisms. Bilateral medial rectus resections were required in two patients. Response to therapy was variable and often incomplete. The anatomic localization of convergence insufficiency secondary to head trauma remains unknown, although lesions in the occipital lobe and upper midbrain both seem capable of producing this syndrome.

Patients with contrived histories and/or self-induced physical abnormalities (Munchausen's syndrome) are often successful in deceiving physicians. We recently cared for four patients with ocular Munchausen's syndrome. Self-induced ocular manifestations included voluntary nystagmus, subconjunctival hemorrhages, chronic orbital emphysema requiring exenteration, corneal alkali burns, erosions and ulcerations, and abscesses of the periorbital area. Correct diagnoses of ocular Munchausen's syndrome were made only after extensive medical and surgical investigations. Suggestions for evaluation and treatment will also be discussed.

Of nine patients (five men and four women, 25 to 55 years old) with localized orbital neurofibromas, only one had other systemic findings consistent with neurofibromatosis. The neurofibromas originated from sensory nerves of the orbit, producing gradual proptosis with expansion of the orbital walls in most cases. Mild orbital discomfort occurred in some patients. Preoperative and postoperative anesthesia in the distribution of the involved sensory nerves was also encountered. Five patients had multiple tumors within the same orbit. Four patients had tumors or a pedicle of the tumor extending into the superior orbital fissure. Two patients were initially thought to have fibrotic pseudotumors. Surgical excision is the treatment of choice for these tumors.

To the Editor. —We would like to emphasize a point made by Dr Kushner1in the May 1984 issue concerning functional amblyopia associated with optic nerve glioma. We recently encountered a patient with decreased visual acuity and a presumed optic nerve glioma. Occlusion therapy resulted in a marked improvement of visual acuity. Report of a Case. —A 20-month-old infant with neurofibromatosis was seen for an intermittent right exotropia that was first noted several months prior to our examination. He had been otherwise healthy, except for mild developmental delay. The examination revealed a strong visual preference for the left eye. A variable right intermittent exotropia of 25 to 30 prism diopters was present. Cycloplegic refraction was +1.00+0.25×80 OD and +2.50+0.75×180 OS. Several small neurofibromas were present on the skin of the left medial canthus and left upper lid. The remainder of the examination results were normal. The patient was given

Whereas most ocular complications following cerebral angiography are benign and transient, central retinal artery occlusion following cerebral angiography produces severe permanent visual deficits in nearly 40% of patients. Physicians ordering and performing cerebral angiography should be aware of this complication, since immediate attention to patients with visual disturbance from central retinal artery occlusion may save useful vision. All previously reported cases of central retinal artery occlusion following cerebral angiography have occurred after direct percutaneous carotid angiography. We report a case of central retinal artery occlusion following transfemoral cerebral angiography using a #5 French catheter and meglumine iothalamate (Conray-60) contrast.

It is frequently reported that calcification in the fossa of the lacrimal gland is a reliable sign of malignancy. We present a small, previously unreported series of lacrimal gland fossa calcifications, in which in three out of five cases the lesion was benign. Calcification can be a misleading radiographic sign and should not, in and of itself, be considered diagnostic of malignancy. This is important because proper preoperative diagnosis of lacrimal fossa tumors is essential as management differs.

Anterior segment ischemia (ASI) is a potentially blinding complication of extensive eye muscle surgery. Eyes have been described with this complication following surgery on as few as two rectus muscles, especially in patients with thyroid dysfunction or other medical illnesses. In an attempt to assess the risk of ASI, we have reviewed the records of 34 eyes in 26 patients who underwent surgery on three or four rectus muscles. Seven eyes of six patients with thyroid ophthalmopathy which underwent two-or three-muscle surgery were also studied. Evidence of clinically significant ASI was apparent in only one patient during a follow-up period of seven months to eleven years. This case was mild and no visual loss resulted. Our findings suggest that surgery on three or four rectus muscles in healthy patients is probably safe when performed in a staged fashion.

We studied 26 white patients (19 males and seven females), ranging in age from 1 to 57 years, with congenital nystagmus for the characteristics of albinism. None of the patients was known to be an albino and none had readily apparent signs of albinism. The study also included 26 controls matched by age, sex, race, and complexion. Twelve of the 26 patients with congenital nystagmus had one or more of the following characteristics: detectable iris transillumination (six of 25 patients), choroidal depigmentation (four of 22 patients), and abnormal tanning history (eight of 26 patients). Iris transillumination and abnormal tanning were not found in any of the controls but two controls showed choroidal depigmentation and one showed blunting of the macular reflex (this was determined to be age-related). These differences were significant for iris transillumination (P = .01), blunting of the macular reflex (P = .01), and abnormal tanning (P = .002) but not significant for choroidal depigmentation (P = .38). Within the group of patients with congenital nystagmus, iris transillumination was significantly associated with blunting of the macular reflex (P = .03), choroidal depigmentation (P = .03), and abnormal tanning (P = .004) but not with eye color (P = .16) or visual acuity (P = .33). These findings suggested that many patients with apparently isolated congenital nystagmus may have subclinical albinism or a form of albinoidism.

Of 22 patients (18 men and four women ranging in age from 50 to 89 years) with radiographic and clinical evidence of Paget's disease, nine had visual field defects. All nine had arcuate scotomas and five of the nine had generalized constriction. The visual field changes were asymptomatic in six of nine patients and progressive in two patients. Only two patients had radiographic evidence of optic canal constriction by bony impingement. There was no objective improvement in the optic neuropathy in the three patients treated with synthetic salmon calcitonin. Our data suggested that the optic neuropathy of Paget's disease cannot be explained solely on the basis of bony compression and the cause of optic neuropathy in patients with normal optic canals remains unknown.