Wills Eye Hospital Research Articles

RARE ANGIOMAS

Assistant Director of Nursing Education Programs Wills Eye Hospital Clinical Instructor Thomas Jefferson University Philadelphia, Pa.

Lesions Simulating Retinoblastoma

Of 500 consecutive patients referred to the Ocular Oncology Service at Wills Eye Hospital with the diagnosis of possible retinoblastoma, 288 (58%) were found on clinical evaluation to have retinoblastoma and 212 (42%) had lesions that simulated retinoblastoma. A total of 23 different conditions accounted for the 212 pseudoretinoblastomas. Three most common pseudoretinoblastomas were: persistent hyperplastic primary vitreous (28%), Coats' disease (16%), and presumed ocular toxocariasis (16%). Congenital cataract and retinopathy of prematurity accounted for a much lower percent of pseudoretinoblastomas in this series as compared to a previously reported series. The pertinent clinical features that serve to differentiate these simulating lesions from retinoblastoma are reviewed.

Long-term Efficacy of Repeat Argon Laser Trabeculoplasty

The role of repeat argon laser trabeculoplasty (RALT) in the management of open-angle glaucoma is controversial. To determine the long-term efficacy of RALT, the authors reviewed case records of 44 patients (50 eyes) who received RALT for uncontrolled glaucoma at the Wills Eye Hospital before 1986. Success was defined as a 3 mmHg or greater decrease in intraocular pressure (IOP) to less than 22 mmHg, and no further surgical intervention. By life table analysis, RALT was successful in 35% of eyes at 6 months, in 21 at 12 months, in 11% at 24 months, and in 5% at 48 months. Thirty-three percent of eyes in which primary ALT was successful for 1 year were successful 12 months after RALT. Complications included IOP spikes in 12%. In conclusion, RALT was generally not effective for long-term control of open-angle glaucoma, but may be useful as a temporizing measure. However, this use of RALT must be weighed against the risks of complications and the additional progression of disease before more definitive surgical intervention.

Wills Eye Hospital Office and Emergency Room Diagnosis and Treatment of Eye Disease

In an era in which the ophthalmic literature, both textbook and periodical, has proliferated, there has been a relative dearth of newly written front line resource material. As a resident in the emergency department of a busy urban eye hospital that is much like that described by the authors, I have encountered a myriad of patients with multifarious complaints and manifestations of disease. It is of paramount importance that residents and clinicians at all levels of expertise have quick access to lists containing the differential diagnoses of common and uncommon ophthalmic problems, as well as appropriate plans for the workup and treatment of these conditions. Drs Friedberg and Rapuano, residents at the Wills Eye Hospital, Philadelphia, Pa, have written a unique text that is likely to become an important resource for clinicians and residents alike. ... a unique text that is likely to become an important resource... The text begins with

Retinoblastoma in older Children

A review of 400 consecutive patients with retinoblastoma who presented to the Ocular Oncology Service at Wills Eye Hospital showed that 34 (8.5%) patients were older than 5 years of age at the time of initial diagnosis. The tumor was active in 26 (76%) cases and inactive (retinoma) in 8 (24%). An evaluation of the 26 patients with active retinoblastoma showed several unique features. At the time of diagnosis, their median age was 6 years and the oldest was 18 years. In 20 (77%) cases, the patient volunteered symptoms that prompted the eye examination; the presenting symptoms included leukocoria (9 cases), decreased vision (9 cases), strabismus (4 cases), pain (1 case), floaters (1 case), and no symptoms (2 cases). All of the 26 patients (100%) had unilateral sporadic retinoblastoma. Misdiagnosis before referral was common in these older children with active retinoblastoma. Five patients (19%) had prior vitrectomy for presumed vitreous hemorrhage or endophthalmitis while the retinoblastoma was unsuspected clinically, one patient (4%) had cryotherapy for presumed Coats disease, and one (4%) was observed for 7 months for presumed vitreous hemorrhage. The clinician should seriously consider the possibility of retinoblastoma in children who present with signs of unexplained vitreous hemorrhage or endophthalmitis, even if they are older than 5 years of age.

Does Optic Nerve Sheath Decompression Help Progressive Ischemic Optic Neuropathy?-Reply

In Reply. —We appreciate Dr Wilson's comments regarding our article on optic nerve sheath decompression for progressive nonarteritic ischemic optic neuropathy (NAION). Our relatively large number of patients with progressive NAION reflects the clinical activity of the Wills Eye Hospital Neuro-Ophthalmology Service, Philadelphia, Pa, where we see over 4000 patients a year. The incidence of progressive visual loss in our population correlates well with the incidence reported by Boghen and Glaser. 1 We agree with Dr Wilson that conclusive evidence is lacking to support a completely ischemic pathogenesis for NAION and believe that the alternative hypothesis proposed by Borchert and Lessell 2 may have merit. However, we doubt that an inflammatory etiology for NAION has been overlooked, because the most complete histopathologic study of this entity did not demonstrate any vascular or neural inflammation. 3 We believe that the focal ischemic neuropathies of diabetes mellitus satisfy the criteria for another

Indications for and Outcomes of Repeat Penetrating Keratoplasty

We reviewed data on 150 repeat corneal transplants performed between 1983 and 1988 at Wills Eye Hospital. Minimal postoperative follow-up was six months. The most common diagnoses before regraft were pseudophakic bullous keratopathy (29 of 150), aphakic bullous keratopathy (28 of 150), Fuchs' dystrophy (21 of 150), failed graft (17 of 150), and keratoconus (14 of 150). Herpes simplex keratitis, interstitial keratitis, chemical burns, traumatic scars, and corneal dystrophies accounted for most of the remaining regrafts. Of the 150 regrafts, 39 (26%) failed during the study period. Almost half of the regraft failures occurred within six months, and over four fifths occurred within 18 months. Of the 111 eyes with clear grafts, final visual acuity data were available for 68 eyes (minimal follow-up, two years; mean, 3.3 years). A best-corrected visual acuity of 20/20 to 20/40 (22 of 68) or 20/50 to 20/100 (27 of 68) was achieved in most patients.

Photoreceptor Cell Differentiation in Intraocular Medulloepithelioma: An Immunohistopathologic Study

We report a case of a teratoid intraocular medulloepithelioma that expressed S-antigen and rhodopsin immunoreactivity. Both proteins are known to be photoreceptor-cell specific and participate in the phototransduction of vision. Our study demonstrates that some medulloepitheliomas may differentiate along a neuronallike cell lineage. Report of a Case. —A 6-year-old white boy was referred to the Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pa, for evaluation of possible medulloepithelioma. The family medical history was positive for glaucoma and diabetes mellitus in addition to thyroid, breast, lung, and colon cancers. On ocular examination, the patient's best corrected visual acuity was 6/15 OD and 6/7.5 OS. An amelanotic cystic mass of the ciliary body was found inferotemporally in the right eye. Growth of the lesion produced a subluxated lens and minimal early cataractous changes. Because of the progressive growth of the lesion, excision by a partial lamellariridocyclectomy was performed in the right eye.

Long-Term Follow Up of Clear Cornea Trabeculectomy

We evaluated the long-term results of Cairns' clear cornea "trabeculectomy" performed on the Glaucoma Service of the Wills Eye Hospital in six patients with serious glaucoma. Although postoperative pressures initially were lower in all six, these lower pressures were not maintained. Pressure in three of the patients finally was controlled only after instituting medical treatment, including carbonic anhydrase inhibitors in two. The operation failed in the other three, two of whom underwent further surgeries. We conclude that Cairns' clear cornea trabeculectomy, in itself, is not adequate to control intraocular pressure in cases of serious glaucoma.

Canalicular Laceration

From 1977 through 1987, a total of 222 patients (166 male and 56 female patients) underwent surgical repair of canalicular laceration at Wills Eye Hospital. Demographic and clinical information were collected from the medical records and by written questionnaire or telephone interview. Most injuries occurred in children or young adults (median age, 20 years). Overall, blows from fists was the most common cause of injury (52 patients, 23.4%). Dog bites or scratches were the most frequent causes among children. A total of 147 injuries (66.2%) involved the lower eyelid, 61 (27.5%) the upper eyelid, and 14 (6.3%) the upper and lower eyelids on the same side. Constant or stress epiphora occurred postoperatively significantly more often among patients with combined upper and lower canalicular injuries (61.5%) than among those with single canalicular laceration (19.7%) (p less than 0.01). Analysis with logistic regression showed epiphora to be more common among adults than children (p less than 0.05) when the pigtail probe had been used intraoperatively (p less than 0.05), or when no canalicular stent had been placed at the time of surgical repair (p less than 0.05). No statistically significant associations were found between sex, cause of injury, type of canalicular stent, or time interval from injury to surgical repair and presence of postoperative epiphora.

Arnold-Chiari Malformation

Intermittent symptoms of obstructive hydrocephalus in a young woman with Chiari-I malformation are reported from the Neuro-Ophthalmology Service, Wills Eye Hospital, Philadelphia, PA.

Diphtheroids as Ocular Pathogens

Unlike Corynebacterium diphtheriae and Propionibacterium acnes, the pleomorphic gram-positive rods known as diphtheroids are generally regarded as nonpathogenic contaminants of the human external eye. We reviewed five years of microbiology records at Wills Eye Hospital and studied a series of eight cases of apparently infectious keratitis associated with heavy growth of diphtheroids on cultures of ulcer scrapings. All of these cases included indolent ulcers that occurred almost exclusively in elderly patients (mean age, 72 years; range, 11 to 92 years). All patients had preexisting ocular conditions that compromised the corneal surface such as exposed corneal sutures, eyelid surgery, aphakic extended wear contact lenses, viral keratitis, and diabetes mellitus. No other pathogens were isolated. All infections responded well to antibiotic therapy with all organisms sensitive to cefazolin and all but one sensitive to gentamicin.

Eye Injuries in Childhood: Demography, Etiology, and Prevention

A 1-year survey was conducted of all children with eye injuries seen in the Wills Eye Hospital emergency room to determine demographic, etiologic, and prophylactic factors. There were 810 children with ocular trauma. Childhood ocular injuries are frequent, often resulting in serious visual impairment. Many of these injuries are preventable.

Functional Blinking in Childhood

At the Department of Pediatric Ophthalmology, Wills Eye Hospital, 17 children, 18 months to 10 years of age, were seen with a chief complaint of intermittent excessive blinking. Eight (47%) of the patients had been referred for ophthalmologic examination by their pediatrician for evaluation of this symptom. All were in excellent health with no associated symptoms or signs of systemic or ocular disease. None of the children were taking topical ocular or systemic medications. The parents of seven (41%) of these children were able to identify a temporally related stressful event that coincided with the onset of blinking. Durations of the symptom prior to examination ranged from 1 week to 4 months. Spontaneous resolution occurred from one day to 5 months after the first ophthalmologic examination. Recurrence occurred in only one child, but resolution was still completed within 5 months. Excessive eye blinking unassociated with other systemic or ocular findings appears to be a medically benign, self-limited functional disorder.

Other Cancers in Uveal Melanoma Patients and Their Families

To determine associations with other cancers, 400 consecutive uveal melanoma patients examined at Wills Eye Hospital between 1984 and 1985 were surveyed regarding personal and family history of cancer. Responses were received from 333 (83%). Sixty patients reported 43 nonbasal cell second primary cancers, which were confirmed pathologically or by physician records. The overall prevalence of nonbasal cell cancers diagnosed in uveal melanoma patients by December 1985 was over two times greater than the expected prevalence, based on the Connecticut Tumor Registry data for an age- and sex-matched population. Gynecologic cancers tended to be more common in uveal melanoma female patients than in the comparison population. Although the observed prevalence of cutaneous melanoma was not significantly greater than expected, three cases with both primary cutaneous and uveal melanoma were reported. Family histories of cutaneous melanoma were confirmed in 14 patients, and uveal melanoma in two patients. Data suggested that the overall cancer prevalence in uveal melanoma patients may be increased, that hormonal factors may play a role in the genesis of this malignancy, and that there may be a link between cutaneous and uveal melanoma.

Visual Field Change in Low-tension Glaucoma over a Five-year Follow-up

There is some evidence that the nature and progression of disease in low-tension glaucoma may be distinct from other open-angle glaucomas. The authors assessed visual field change by retrospective case review of all patients treated for low-tension glaucoma by the Glaucoma Service, Wills Eye Hospital, for at least 5 years. Sixty-two glaucomatous eyes of 36 patients were identified. All eyes were treated medically and 40 (65%) underwent at least one surgical procedure. Twenty-eight eyes (47%) had initial field loss confined to a single hemi-field and in the remainder both hemi·fields were involved. Thirty of 57 eyes (53%) showed progression at 3 years and 38 (62%) of 57 had progressed by 5 years. A dense scotoma extending from the nasal periphery toward fixation was the most common visual field defect. The rate of field change in this population is significantly greater than in a cohort of primary open-angle glaucoma patients also seen at Wills Eye Hospital, but who had elevated intraocular pressures. Patterns of field loss and rate of progression in this lowtension glaucoma population suggest that the natural history of low-tension glaucoma differs from high-tension open-angle glaucoma.

Risk Factors for the Development of Tenon's Capsule Cysts after Trabeculectomy

Tenon's capsule cysts (TCCs) are a complication of glaucoma filtering surgery. They are frequently associated with substantial elevations in intraocular pressure (IOP) beginning 2 to 8 weeks postoperatively. To determine the incidence and possible risk factors for the development of TCCs, case records of all patients who received trabeculectomy over a 4-year period at the Wills Eye Hospital were reviewed. The incidence of TCCs was 28% in those who underwent trabeculectomy. Characteristics of patients after trabeculectomy in whom TCCs developed were compared with patients after trabeculectomy in whom TCCs did not develop. Both univariate and multivariate techniques were used to assess the association of characteristics associated with the development of TCCs. Factors associated with increased risk (P < 0.05) were: history of prior TCCs, argon laser trabeculoplasty, male gender, and the use of preoperative sympathomimetics. The use of a compression shell was associated with decreased risk (P < 0.05).

Seven Cases of Trilateral Retinoblastoma

Of 245 consecutive children with retinoblastoma referred to the Oncology Service at the Wills Eye Hospital between January 1974 and August 1988 and followed up for three months to 15 years, seven (3%) developed midline intracranial malignancies consistent with the diagnosis of trilateral retinoblastoma. Six of the seven had bilateral retinoblastoma, and four had a family history of retinoblastoma. The time of diagnosis of retinoblastoma varied between five months after the intracranial tumor was diagnosed and four years before the intracranial tumor was diagnosed. The midline intracranial malignancies were pineal tumors (five cases), suprasellar neuroblastoma (one case), and a parasellar undifferentiated calcified mass (one case). Despite control of the intraocular tumors, five of the seven children have died of the intracranial tumors.

The treatment of primary intraocular malignancy

This paper will summarize much of the information derived in an association between The Department of Radiation Oncology of Hahnemann University Hospital and the Oncology Service of Wills Eye Hospital of Thomas Jefferson University, a collaborative effort for the treatment of primary intraocular malignancies that has spanned the last dozen years. In that time we have treated malignant intraocular melanoma by radioactive eyeplaque brachytherapy and have begun to develop a similar program for treatment of recurring retinoblastoma. These experiences will be described.

Ocular Streptococcal Infections

Streptococcal infections are an important cause of corneal ulcers, endophthalmitis, conjunctivitis, and dacryocystitis. We retrospectively reviewed 53 ocular streptococcal infections diagnosed and followed at Wills Eye Hospital between January 1, 1984, and July 1, 1986. Corneal ulcers (23 of 53; 43%) and endophthalmitis (18 of 53; 34%) constituted a majority of the streptococcal infections in this series. Streptococcal infection was also a common cause of conjunctivitis, but clinical follow-up data were sufficient for inclusion in this series in only a small fraction of conjunctivitis patients (9 of 70; 13%). Streptococcal corneal ulcers and endophthalmitis were frequently associated with a poor visual outcome. When antibiotic sensitivity testing was done, approximately one third of the streptococcal isolates (13 of 38; 34%) were not sensitive to gentamicin. As topical gentamicin is frequently used by ophthalmologists, the possibility of streptococcal infection should be taken into account in the selection of antibiotics for conjunctivitis, corneal ulcers, endophthalmitis, and prophylaxis.