Gregory B. Krohel

Clinical presentation and management of lacrimal gland tumours.

This paper presents the clinical and pathological findings in 40 consecutive patients seen with primary tumours arising from the lacrimal gland. Twenty patients had a benign mixed-cell tumour. They presented in a recognisable clinical manner with a painless mass in the region of the lacrimal gland which slowly enlarged over a period of at least 1 year before consultation. Twenty patients had a carcinoma of the lacrimal gland. They had a short history and experienced pain. On clinical grounds they could not be distinguished from inflammatory lesions in the region of the lacrimal gland. The method of treating these 2 groups of patients is described and methods of dealing logically with their problems are suggested.

Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: Nine new cases and a review of the literature

OBJECTIVE The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN Retrospective, noncomparative case series. PARTICIPANTS Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Sinus Tumors Invading The Orbit

Forty -seven of 79 patients with sinus and paranasal tumors had clinical, radiographic or operative evidence of orbital involvement. Seventy percent of those patients with orbital extension had clinical or radiographic involvement of the orbit at the time of initial presentation. Common presenting signs and symptoms included proptosis, nasal obstruction or discharge, nasal mass, facial and/or eye pain, visual loss, facial and/or lid edema and diplopia. The most common tumor seen was squamous cell carcinoma. The maxillary sinus was the most frequent site of origin. Three patients were misdiagnosed as having sinusitis on initial evaluation. A diagnosis of sinusitis is tentative and should be reevaluated early with repeat roentgenographic studies and biopsy, especially in the presence of protracted facial and eye pain. When ordering CT scans, one must specifically request cuts of the base of the sinuses and skull as routine brain CT scans do not evaluate those regions. Two of four patients with intractable pain unrelieved by narcotics obtained pain relief with cisplatinum.

Levodopa may improve vision loss in recent-onset, nonarteritic anterior ischemic optic neuropathy.

OBJECTIVE To study the effect of levodopa in improving visual function in patients treated within 45 days of onset of nonarteritic anterior ischemic optic neuropathy (NAION). DESIGN Nonrandomized, retrospective, comparative trial. PARTICIPANTS The study involved 37 patients with NAION of less than 45 days duration. METHODS Eighteen patients who had been treated with levodopa were assigned to the case group, and 19 untreated patients were assigned to the control group. Snellen visual acuity converted to logMAR and mean deviation on Humphrey automated perimetry (Program 24-2, Humphrey Instruments, San Leardro, CA) were evaluated at the initial and 6-month visits. INTERVENTION The 18 patients in the case group were administered a capsule of 100 mg levodopa/25 mg carbidopa (Sinemet 25-100) three times daily for 3 weeks. MAIN OUTCOME MEASURES The primary outcome measures were changes in visual acuity and visual field at 6 months from baseline. Improvement in visual acuity was defined as a difference of -0.3 logMAR or less between the 6-month and initial visual acuities, whereas worsened visual acuity was a difference of +0.3 logMAR or more. Each 0.3 LogMar represented a doubling of the visual angle, i.e., a change by three lines on the eye chart. Improvement in visual field was defined as a difference in mean deviation of +3.0 dB or more between the 6-month and initial visual field tests, whereas worsened visual field was a difference in mean deviation of -3.0 dB or less. RESULTS The proportions of patients with worsened, unchanged, and improved visual acuity at 6 months were compared for the levodopa and control groups. There was a significant difference (P = 0.012) between the groups. Examination of the proportions showed that a higher proportion of patients who received levodopa had improved visual acuity with a corresponding lower proportion having worsened visual acuity as compared with the control patients. Ten of 13 patients (76.9%) in the levodopa group with 20/40 visual acuity or worse at baseline had improved visual acuity at 6 months, and none of the 18 patients had worsened visual acuity. In contrast, 3 of 10 control patients (30%) with 20/40 visual acuity or worse at baseline had improved visual acuity at 6 months, and 3 of 19 control patients (16.3%) had worsened visual acuity. The proportions of patients with worsened, unchanged, and improved visual fields at 6 months were compared for the levodopa and control groups. There was no significant difference between the groups (P = 0.25). CONCLUSIONS Patients treated with levodopa within 45 days of onset of NAION were more likely to experience improvement and less likely to have worsened visual acuity than untreated patients. Levodopa appears to be beneficial in the treatment of recent-onset NAION.

Lacrimal gland and fossa lesions: an approach to diagnosis and management.

Lesions of the fossa of the lacrimal gland present special problems in diagnosis and management. Clinical recognition and differentiation of benign mixed cell lacrimal gland tumors from other inflammatory and neoplastic lesions of the lacrimal gland fossa are feasible and should provide the basis for the approach to proper management. Lesions conforming to the clinical picture of benign mixed cell lacrimal gland tumors need not undergo incisional biopsy, but require en bloc excision with adjacent tissues through a lateral orbitotomy to ensure complete excision and to prevent late recurrence. Incisional biopsy is indicated and should not be delayed in suspected infectious and noninfectious inflammatory lesions that have not responded rapidly to medical therapy, and in other lesions suspected of being neoplastic and not amenable to total local excision. An expedient approach to these lesions is essential if the prognosis of epithelial lacrimal gland neoplasms is to be improved.

Functional Visual Loss in Children

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.

Orbital Abscess: Presentation, Diagnosis, Therapy, and Sequelae

Fifteen cases of orbital abscess were reviewed. Significant morbidity occurred despite treatment with intravenous antibiotics and surgical drainage. The complications included visual loss to less than 20/200 (four patients), residual proptosis (two patients), residual diplopia (two patients), osteomyelitis (one patient), and death (one patient). Seven patients reported no pain. Fever was absent in eight patients. Four patients had normal white blood cell counts. Many of these patients had received inappropriate or inadequate doses of oral antibiotics before referral. Partially treated cases of orbital abscess may not manifest the expected clinical findings of orbital infection. Four patients presented in an insidious fashion with symptoms evolving over weeks to months. Their subacute presentations in the absence of fever, pain, or elevated white blood cell count mimicked the onset of orbital tumors. CT scan failed to detect an abscess in two cases. Conjunctival and nasal cultures were not helpful in determining the bacteriologic etiology of these infections. Surgical drainage and appropriate antibiotic therapy is the definitive treatment of orbital abscess.

Localized Orbital Neurofibromas

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.

Prevention of Ocular Gunshot Injuries Using Polycarbonate Lenses

Two patients lost three eyes, and one his life, secondary to ocular gunshot wounds. In both patients, the pellets gained entry into the central nervous system through the eye and orbital bones despite only superficial wounds to other areas of the body. These tragic injuries prompted a study of polycarbonate lenses to determine their effectiveness in preventing small caliber ocular gunshot wounds. This investigation determined that 3.0 mm and the 2.2 mm polycarbonate lenses could prevent such injuries at distances greater than 15 yards. In comparing the two lenses, it was found that the 3.0 mm lens sustained less damage at 70 degrees F, but the 2.2 mm lens was more resistant to penetration at 32 degrees F. Since most hunting is done in the fall and winter months, the brittle nature of the thicker lens is significant. Polyamide sports frames with a posterior rim were also tested and found to prevent posterior lenticular expulsion at 15 yards. Standard acetate frames were shown to be ineffective in this regard at 30 yards.

Ocular Munchausen's Syndrome

Patients with contrived histories and/or self-induced physical abnormalities (Munchausens syndrome) are often successful in deceiving physicians. We recently cared for four patients with ocular Munchausens 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 Munchausens syndrome were made only after extensive medical and surgical investigations. Suggestions for evaluation and treatment will also be discussed.