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Biomedical subjects

J A Shields

Publications and source records attributed to J A Shields.

At least 325 records · Page 18Linked to original sources
PubMed1992

Misconceptions and techniques in the management of retinoblastoma. The 1992 Paul Henkind Memorial Lecture.

For many years, enucleation has been the most common treatment of retinoblastoma. It generally has been done on the affected eye of children with unilateral disease and on the more severely affected eye in children with bilateral disease. In recent years, there has been a trend toward earlier diagnosis of retinoblastoma, and there have been refinements in conservative therapeutic methods, such as radiotherapy, photocoagulation, and cryotherapy. Consequently, it often is possible to salvage the affected eye and retain useful vision in many patients who would have undergone enucleation in the past. This article provides an update on the diagnosis and treatment of retinoblastoma, with emphasis on certain misconceptions related to the management of this tumor. New genetic aspects and their relationship to counseling are discussed briefly.

Arizona↗
PubMed1992

Unusual retinal and renal vascular lesions in the Klippel-Trenaunay-Weber syndrome.

Ocular fundus abnormalities associated with the Klippel-Trenaunay-Weber syndrome are uncommon and include retinal vascular tortuosity and diffuse choroidal hemangioma. A case involving a young girl with Klippel-Trenaunay-Weber syndrome who had unusual bilateral, exudative, outer retinal vascular masses involving the peripheral fundus in one eye and the foveal area in the other eye is reported. Kidney biopsy for renal insufficiency disclosed abnormal excess mesangial tissue. The fundus lesions appear to represent vascular tumors of the retina that differ clinically from previously reported retinal vascular tumors and Coats disease. The simultaneous retinal and renal involvement suggest that Klippel-Trenaunay-Weber syndrome may be associated with more widespread vascular malformations than previously realized.

Biopsy↗
PubMed1992

Nocardia choroidal abscess.

Nocardia is a Gram positive, aerobic, filamentous branching micro-organism that rarely causes human infection. When infection does occur it usually takes the form of a subcutaneous abscess or a pneumonia-like illness. We describe a case of a patient with chronic lymphocytic leukaemia who developed painless loss of vision in the right eye secondary to a choroidal abscess after a prolonged course of treatment on several immunosuppressive agents. The patient also complained of right shoulder pain that was unresponsive to conventional therapy, and had been admitted and treated for several episodes of 'pneumonia'. A diagnostic transvitreal fine-needle aspiration biopsy of the ocular lesion was performed which demonstrated Nocardia asteroides. This allowed for appropriate antibiotic therapy to be instituted early in the course of the infection and prompted the systemic work-up which also demonstrated central nervous system and arthropic nocardial infection.

Abscess↗
PubMed1992

The CT and MRI features of an unusual case of isolated orbital neurofibroma.

A 35-year-old woman developed painful proptosis of the left eye over a period of 3 weeks. Orbital computed tomography and magnetic resonance imaging revealed a well-circumscribed superior orbital tumor with variable density. The mass was removed entirely by way of a superior orbitotomy. Histopathologically, it was found to be a neurofibroma. The patient had no clinical findings of neurofibromatosis. The presence of extensive myxomatous degeneration and the dense collection of collagen bundles in the tumor are correlated with the unusual computed tomography and magnetic resonance imaging features.

Adult↗
PubMed1991

Retinoblastoma manifesting as orbital cellulitis.

Five children with previously undiagnosed retinoblastoma had signs of unilateral orbital cellulitis and were treated with systemic antibiotics, corticosteroids, or both. When retinoblastoma was finally diagnosed clinically, radiologists believed in every case that, on the basis of the soft-tissue swelling seen with computed tomography, there was orbital extension of the tumor. However, intraoperative and histopathologic observations disclosed no demonstrable orbital tumor involvement. The affected eyes all had neovascular glaucoma, and all harbored highly necrotic tumors that were contained within the eye. We concluded that orbital inflammation can occur with retinoblastoma confined to the eye and that signs of orbital cellulitis do not necessarily imply orbital extension of the tumor.

Cellulitis↗
PubMed1991

Late consolidative radiation therapy in the treatment of limited-stage small cell lung cancer.

Two hundred twenty-three patients were enrolled on this randomized Phase III trial testing the value of late consolidative involved-field radiation therapy in the treatment of limited-stage small cell lung cancer (SCLC). Patients were treated with induction chemotherapy consisting of alternating cycles of procarbazine, vincristine, lomustine, and cyclophosphamide (POCC) and etoposide, doxorubicin, and methotrexate (VAM) for 6 to 9 months. Responding patients were then randomized at 6 or 9 months to chemotherapy alone or to involved-field radiation therapy. All partial and complete responders received prophylactic cranial irradiation. Of the 180 eligible and evaluable patients, 80 (44%) achieved a complete response and 39 (22%) achieved a partial response (overall rate of response, 66%). Actuarial median survival time was 11.6 months, with 16% of patients surviving 2 years and 11% surviving 5 years. Forty-eight patients were randomized to chemotherapy alone (24 patients) versus chemotherapy plus involved-field radiation therapy (24 patients). There were no significant differences in time to progression or survival between those patients receiving or not receiving involved-field radiation therapy. The thorax was the site of first relapse in 58% of patients randomized to chemotherapy alone versus 29% in patients randomized to chemotherapy plus involved-field radiation therapy (P equals 0.042). The major acute toxicity was reversible myelosuppression, and the major late toxicity was chronic central nervous system dysfunction. The authors conclude that the addition of late consolidative radiation therapy to induction chemotherapy in the treatment of limited-stage SCLC is well tolerated and improves local control, but does not improve time to progression or rates of survival.

Adult↗
PubMed1991

Histopathologic evidence of fibrovascular ingrowth four weeks after placement of the hydroxyapatite orbital implant.

We studied the histopathologic findings after placement of an orbital hydroxyapatite implant in a patient. The implant was placed after enucleation for a choroidal melanoma and was removed four weeks later because histopathologic examination of the enucleated globe disclosed tumor invasion of the optic nerve that necessitated further orbital tissue removal. Our histopathologic findings confirm that there is significant ingrowth of fibrovascular tissue in these implants, even within weeks of implantation.

Aged↗
PubMed1991

Color Doppler imaging of the eye and orbit. Technique and normal vascular anatomy.

Color Doppler imaging is a recent advance in ultrasonography that allows simultaneous two-dimensional imaging of structure and blood flow to be performed. Doppler information is superimposed in color over a conventional gray-scale ultrasound image. Using this technique, we examined 40 normal eyes. The central retinal artery, posterior ciliary arteries, ophthalmic artery, and central retinal vein could be located in all patients. Using the color image as a guide, Doppler spectral analysis allows quantitative assessment of blood flow velocity in these vessels. Color Doppler imaging is a new modality for the study of ocular and orbital hemodynamics.

Adult↗
PubMed1991

Orbital and eyelid involvement with Erdheim-Chester disease. A report of two cases.

Erdheim-Chester disease is an idiopathic condition characterized by infiltration of the heart, lungs, retroperitoneum, bones, and other tissues by a fibrosing xanthogranulomatous process composed of xanthomatous histiocytes and Touton giant cells. This condition is often fatal, with death due to cardiomyopathy, severe lung disease, or chronic renal failure. Ocular findings with this potentially fatal disease are rare. We report the clinical and histopathologic findings in two cases of bilateral xanthelasmas and bilateral orbital infiltrates in association with Erdheim-Chester disease. The first patient was a 38-year-old man with cardiovascular and renal disease and severe retroperitoneal fibrosis. The massive orbital infiltration produced bilateral blindness. The second patient was a 77-year-old man with severe cardiovascular disease and retroperitoneal fibrosis. The diagnosis was confirmed in both patients with retroperitoneal and orbital biopsies. Both patients had the unusual occurrence of bilateral xanthelasmas with bilateral, diffuse orbital masses, eye findings that should alert the clinician to the possibility of this serious systemic disease.

Adult↗
PubMed1991

Uveal melanoma and pregnancy. A report of 16 cases.

A review of 3706 consecutive patients with uveal melanoma over a 17-year period revealed that 16 patients (0.4%) were pregnant women at the time of diagnosis. The mean age at presentation in this group was 30 years and the mean months of gestation at the time of diagnosis of the posterior uveal melanoma was 6 months. Seven of the sixteen tumors were active uveal melanomas at the initial examination and were treated immediately, while the remaining nine tumors were initially diagnosed as suspicious choroidal nevi or dormant choroidal melanomas, seven of which grew into active melanomas during the course of the pregnancy, necessitating therapy. The tumors were managed by enucleation in 10 cases, plaque radiotherapy either during or after pregnancy in 4 cases, and observation in 2 cases. Histopathologically, the melanomas did not differ appreciably in cell type, mitotic activity, and other features when compared with a matched group of tumors in nonpregnant women. All of the patients who elected to carry the pregnancy to term (14 cases) delivered healthy babies with no placental or infant metastases. The 5-year survival rate using the life table method in these pregnant women with posterior uveal melanoma is 71% and is similar to the survival of nonpregnant women with posterior uveal melanoma reported in other series.

Adolescent↗
PubMed1991

Uveal melanoma in teenagers and children. A report of 40 cases.

A review of 3706 consecutive patients with uveal melanoma revealed that 40 patients (1.1%) were age 20 years or younger at the time of diagnosis. The youngest patient was age 6 years but the majority of patients (78%) were between 15 and 20 years old. The tumor occurred in the iris in 5 cases (12%) and in the posterior uvea in 35 cases (88%). The mean largest tumor dimension and thickness was 10 mm and 5 mm, respectively. In all cases, the diagnosis of uveal melanoma was suspected before referral, and misdirected treatment was avoided. The tumor was initially treated by enucleation in 24 cases (60%), local resection in 7 (18%), plaque radiotherapy in 3 (8%), and observation in 6 (15%). Secondary treatment was required in 7 cases in the form of enucleation (4 cases), ablative laser (1 case), plaque radiotherapy (1 case), and exenteration (1 case). The mean follow-up period was 68 months (median, 48 months) from the time of treatment, and only one patient died of metastases (from a massive ciliochoroidal melanoma 33 months after treatment). The remainder of the group of young patients are alive and healthy. Cumulative survival rates show that 96% of young patients with uveal melanoma survive at the 5-year period.

Adolescent↗
PubMed1991

Partial lamellar sclerouvectomy for ciliary body and choroidal tumors.

The authors reviewed the postoperative course, visual results, histopathologic findings, and mortality data on 95 consecutive patients with tumors of the ciliary body and/or choroid who were managed by a partial lamellar sclerouvectomy. This surgical procedure is designed to remove the uveal tumor and leave intact the outer sclera and sensory retina. In this group of 95 patients, vitreous hemorrhage occurred in 79 (83%) cases, intraretinal or subretinal hemorrhage in 33 (35%), retinal detachment in 26 (28%), and cataract in 32 (34%). Most of the vitreous and retinal hemorrhages resolved spontaneously, sometimes leaving subretinal or preretinal fibrosis. A number of the postoperative retinal detachments resolved spontaneously, but retinal detachment surgery was necessary in 16 patients (17%), mostly in the earlier years of the survey. Most of the cataracts were mild and did not require surgery. The eventual postoperative visual acuity was equal to or better than the preoperative visual acuity in 23 cases (24%) over a mean follow-up period of 5 years. Enucleation was eventually necessary in 15 cases (16%), usually because of residual or recurrent tumor. Histopathologic diagnosis was uveal melanoma in 81 cases (85%) and other tumors in 14 cases (15%). There were no orbital recurrences, but distant metastases developed in five patients (5%), all of whom had undergone enucleation for recurrence of the intraocular melanoma. Although the surgical technique is difficult, partial lamellar sclerouvectomy appears to be a reasonable therapeutic option in selected cases of posterior uveal tumors.

Adolescent↗
PubMed1991

Idiopathic sclerochoroidal calcification.

Idiopathic sclerochoroidal calcification is a disorder that is often overlooked, occurring at the level of the posterior sclera and choroid in older patients. Clinically, it may be confused with choroidal osteoma, choroidal metastasis, amelanotic choroidal nevus, or choroiditis. The authors present seven cases of this unusual entity. The patients ranged in age from 58 to 80 years. In all cases, the yellow lesions were clinically estimated to be at the level of the choroid and sclera, and they were usually located superior to the superotemporal retinal vascular arcade. Systemic investigation of calcium and phosphate metabolism was normal in all patients. The classic clinical manifestations and differentiation of this unusual entity from other similar fundus lesions is discussed.

Aged↗
PubMed1991

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.

Adolescent↗
PubMed1991

Transscleral leiomyoma.

A bluish-pink epibulbar lesion, which slowly enlarged over a period of 5 years, developed in a 31-year-old woman. The lesion initially resembled a staphyloma, based on the very thinned overlying sclera, but subsequent evaluation suggested that it was a solid mass. At the time of surgical removal, the mass appeared to have originated in the supraciliary region and to have eroded outward through the sclera. Histopathologically, the mass proved to be a leiomyoma. The authors propose theoretic possibilities to explain the location of this rare type of ocular smooth muscle tumor.

Actins↗