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Biomedical subjects

Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 145 records · Page 8Linked to original sources
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Tumors of the conjunctiva and cornea.

Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management.

Conjunctival Neoplasms↗
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Ocular tuberculosis masquerading as ocular tumors.

Tuberculosis has re-emerged as a serious public health problem in recent years. The ocular manifestations of tuberculosis are uncommon and diverse. Occasionally, patients initially present with ocular symptoms that simulate intraocular malignancy or other inflammatory conditions. We present five patients with ocular tuberculosis who were referred with the suspicion of ocular malignancy. Four of the five patients had recently emigrated to the United States. The presenting features of these patients were panophthalmitis (one patient), endophthalmitis leading to scleral perforation (one patient), active choroidal granuloma associated with uveitis (two patients), and amelanotic choroidal lesion without inflammatory signs (one patient). Of these five cases, two had a known history of systemic tuberculosis, while the ocular findings in the other three cases were the presenting manifestation of systemic tuberculosis. Multi-drug antituberculous regimen were employed in all cases for a mean of 9 months (median 6 months, range 6-12 months). Three patients responded well to therapy with salvage of the globe and the two remaining patients underwent primary enucleation for blind painful eye or perforated eye. In conclusion, ocular tuberculosis can have variable clinical manifestations and occasionally appears as an intraocular or epibulbar tumor. A high degree of clinical suspicion is important, especially in immigrants from developing countries.

Adult↗
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Orbital cysts of childhood--classification, clinical features, and management.

There is little in the literature about the clinical spectrum of orbital cysts of childhood and no comprehensive classification has been proposed. The authors propose a classification of orbital cysts of childhood and review their clinical features, pathology, and management. The major categories in the classification include cysts of surface epithelium, teratomatous cysts, neural cysts, secondary cysts, inflammatory cysts, and noncystic lesions with cystic component. Cysts of the surface epithelium are further divided into simple epithelial cyst (epidermal, conjunctival, respiratory, and apocrine gland), and dermoid cyst (epidermal and conjunctival). Epidermal dermoid cyst (dermoid) is by far the most common orbital cystic lesion in children, accounting for over 40% of all orbital lesions of childhood and for 89% of all orbital cystic lesions of childhood that come to biopsy or surgical removal. Neural cysts include those associated with ocular maldevelopment (congenital cystic eye and colobomatous cyst) and those associated with brain and meningeal tissue (cephalocele and optic nerve meningocele). The most important secondary cyst is mucocele that can occur in children with cystic fibrosis. Inflammatory cysts are generally due to parasitic infestations and are more common in tropical areas of the world. Noncystic lesions that can have a cystic component include adenoid cystic carcinoma, rhabdomyosarcoma, lymphangioma, and others. Each type of cyst has rather characteristic, but not pathognomonic, clinical features. Computed tomography and magnetic resonance imaging can help differentiate a cystic lesion from a solid tumor, suggest the type of cyst, and help in planning management. The pathology varies with the cells that line the cyst and with the inflammatory agent. Management varies from local excision to observation, depending on the location and type of cyst. Orbital cysts of childhood can be classified into categories, based mainly on their histopathology. The clinical, radiologic, and histopathologic features can be correlated with the classification in order to better evaluate a child with a cystic lesion in the orbit.

Adolescent↗
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Sebaceous carcinoma of the ocular region: a review.

Sebaceous carcinoma of the ocular region is a malignant neoplasm that is being recognized more frequently and managed by innovative techniques of local resection, cryotherapy, topical chemotherapy, and radiotherapy, resulting in improved visual and systemic prognosis.

Adenocarcinoma, Sebaceous↗
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Melanocytoma of the optic disk: a review.

Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10-15% of cases and can cause minor visual loss by a variety of mechanisms. In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy. More importantly, it can exhibit malignant transformation into melanoma in 1-2% of cases. Ophthalmologists should be familiar with melanocytoma of the optic disk and affected patients should be followed periodically.

Diagnosis, Differential↗
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Conjunctival amyloidosis: report of six cases and review of the literature.

Conjunctival amyloidosis is an uncommon condition that occasionally is associated with systemic involvement. The clinical presentations of conjunctival amyloidosis are diverse. We present six patients with conjunctival amyloidosis who were referred to us with the suspicion of another conjunctival lesion. The conjunctival lesion was circumscribed in two patients and diffuse in four patients. The lesion color was yellow in one patient and yellow-pink in five patients. The associated features were intrinsic vascularization (six patients), recurrent subconjunctival hemorrhage (four patients) and blepharoptosis that involved the palpebral conjunctiva (two patients). Systemic evaluation revealed primary systemic amyloidosis in one patient and no related systemic abnormalities in five patients. Management consisted of complete excisional biopsy for the two circumscribed lesions and incisional biopsy for the four diffuse lesions. Two patients with diffuse involvement showed progressive involvement of the conjunctiva over 3 years following incisional biopsy and the other four patients remained stable. Additionally, there was no systemic involvement in five patients. In conclusion, conjunctival amyloidosis generally manifests as a yellowish-pink, hemorrhagic mass deep to the epithelium. Most patients show no evidence of systemic amyloidosis.

Aged↗
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Two discrete choroidal melanomas in an eye with ocular melanocytosis.

We report a case of multifocal choroidal melanoma arising in an eye with ocular melanocytosis and review the pertinent literature. A 63-year-old Caucasian male with ocular melanocytosis in the left eye was found to have two discrete choroidal melanomas in the same eye. Histopathology of the enucleated eye confirmed the diagnosis of two discrete choroidal melanomas of mixed cell type within a region of choroidal melanocytosis. It is estimated that 1 in 160,000 patients with unilateral ocular melanocytosis may develop two uveal melanomas, based on the reported data. On the basis of random chance, patients with two melanomas in the same eye would be expected to have approximately 1000-fold greater likelihood of underlying ocular melanocytosis than the general Caucasian population. In conclusion, multifocal choroidal melanoma is rare and may be related to underlying ocular melanocytosis.

Choroid Neoplasms↗
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Rhabdomyosarcoma: review for the ophthalmologist.

Rhabdomyosarcoma is a malignant neoplasm that is composed of cells with histopathologic features of striated muscle in various stages of embryogenesis. It can occur in several sites in the body, including the ocular region. Ocular rhabdomyosarcoma is defined as the occurrence of this tumor in the area of the eye. Most ocular rhabdomyosarcomas arise in the soft tissues of the orbit but they can rarely occur in the other ocular adnexal structures and even within the eye. The purpose of this review is to provide a brief overview of rhabdomyosarcoma and a more detailed review of orbital rhabdomyosarcoma, with emphasis on changing concepts in the diagnosis and management of this ophthalmic neoplasm.

Combined Modality Therapy↗
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Optical coherence tomography of choroidal nevus in 120 patients.

OBJECTIVE: To describe the optical coherence tomography (OCT) findings of choroidal nevi. METHODS: Retrospective, single-center case series of 120 eyes of 120 consecutive patients with choroidal nevi who were evaluated by OCT. Diagnostic imaging was performed with a Zeiss StratusOCT Model 3000 (Carl Zeiss Ophthalmic Systems, Dublin, CA) using scan acquisition protocols of 6 radial lines and retinal thickness analysis overlying the nevus. RESULTS: The mean patient age was 59 years (median, 60 years; range, 14-87 years). The choroidal nevus was a mean of 5.2 mm in basal dimension and 1.7 mm in thickness and was located a mean of 2.7 mm from the optic disk and 2.5 mm from the foveola. Related retinal findings by ophthalmoscopic evaluation included overlying retina edema (3%), subretinal fluid (16%), retinal thinning (0%), drusen (58%), and retinal pigment epithelium (RPE) detachment (2%). In comparison, related retinal findings at the site of the nevus by OCT included overlying retina edema (15%), subretinal fluid (26%), retinal thinning (22%), drusen (41%), and RPE detachment (12%). Furthermore, OCT permitted classification of the overlying retinal edema as focal cystoid (3%), diffuse cystoid (8%), coalescent cystoid (3%), and noncystoid edema (1%). By OCT, the overlying retina was normal thickness (32%), thinned (22%), or thickened (45%), and photoreceptor loss or attenuation was noted in 51% of cases. Specific OCT findings of the choroidal nevus were limited to its anterior surface with minimal penetration into the mass. These findings included increased thickness of the RPE/choriocapillaris layer (68%) and optical qualities within the anterior portion of the nevus of hyporeflectivity (62%), isoreflectivity (29%), and hyperreflectivity (9%). Hyporeflectivity was observed in 68% of pigmented nevi and 18% of nonpigmented nevi. When comparing OCT with clinical examination, OCT was more sensitive in the detection of related retinal edema, subretinal fluid, retinal thinning, photoreceptor attenuation, and RPE detachment. CONCLUSIONS: OCT is a useful diagnostic modality for imaging the retina overlying a choroidal nevus. Numerous overlying changes such as subretinal fluid, retinal edema, retinal thinning, and photoreceptor attenuation are visible by OCT.

Adolescent↗
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Intravitreal triamcinolone acetonide for radiation maculopathy after plaque radiotherapy for choroidal melanoma.

OBJECTIVE: To evaluate the effect of intravitreal triamcinolone acetonide on patients with visually symptomatic radiation-induced maculopathy after plaque radiotherapy for choroidal melanoma. DESIGN: In this prospective, nonrandomized, single-center case series of 31 patients with visually symptomatic radiation-induced maculopathy after plaque radiotherapy for choroidal melanoma at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, triamcinolone acetonide (4 mg/1 mL) was injected through the pars plana into the vitreous cavity using sterile technique. Status of radiation maculopathy and final visual acuity were the main outcome measures. RESULTS: At the time of diagnosis of choroidal melanoma, visual acuity was 20/20 to 20/50 in 90% (n = 28), 20/60 to 20/200 in 10% (n = 3), and 20/400 or worse in none of the patients. The mean radiation dose to the foveola was 5,122 cGy (median, 3,280 cGy; range, 1,000-16,100 cGy). Radiation maculopathy developed at a mean of 22 months (median, 16 months; range, 6-96 months) after plaque radiotherapy. In all cases, the choroidal melanoma was regressed, and there was no retinal detachment or neovascularization of the retina, optic disk, or iris. At the time of diagnosis of radiation maculopathy, visual acuity was 20/20 to 20/50 in 19% (6/31), 20/60 to 20/200 in 58% (18/31), and 20/400 or worse in 23% (7/31) of patients. After intravitreal injection of triamcinolone acetonide, visual acuity was stable or improved in 91% (20/22) of patients by 1 month and 45% (14/31) by 6 months. Mean foveal thickness by optical coherence tomography was 417 microm at injection and 207 microm at 1 month and 292 microm at 6 months after injection. CONCLUSIONS: Intravitreal triamcinolone acetonide can stabilize or improve visual acuity in some patients with radiation-induced maculopathy, but its effect might not be lasting.

Adult↗
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Intravitreal triamcinolone acetonide for acute radiation papillopathy.

OBJECTIVE: To evaluate intravitreal triamcinolone acetonide for patients with visually symptomatic acute radiation-induced papillopathy. METHODS: In a prospective, nonrandomized, single-center case series, intravitreal triamcinolone acetonide (4 mg/0.1 mL) was injected through the pars plana using sterile technique in 9 patients with radiation papillopathy after plaque radiotherapy for choroidal melanoma. Status of radiation papillopathy and final visual acuity were the main outcome measures. RESULTS: At the time of diagnosis of the choroidal melanoma, visual acuity was 20/20 to 20/40 (n = 6), 20/60 (n = 2), and 20/100 (n = 1). The mean radiation dose to the optic disk was 6,175 cGy (median, 5,994 cGy; range, 3,571-12,760 cGy). Radiation papillopathy developed a mean of 18 months (median, 17 months; range, 6-33 months) after plaque radiotherapy. In all cases, the choroidal melanoma was regressed, and there was no retinal detachment or neovascularization of the retina, optic disk, or iris. Concomitant radiation maculopathy was found in 8 eyes manifesting as macular edema (n = 8), intraretinal dot hemorrhages (n = 6), intraretinal exudation (n = 6), or nerve fiber layer infarction (n = 3). The radiation papillopathy findings included optic disk hyperemia (n = 9), edema (n = 9), and circumpapillary hemorrhage (n = 8). At the time of diagnosis of radiation papillopathy, visual acuity was 20/70 (n = 1), 20/100 (n = 4), 20/200 (n = 1), and counting fingers (n = 3). At 1 week after injection of triamcinolone acetonide, visual acuity improvement was found in seven patients, and resolution of optic disk hyperemia and edema was noted for four and three patients, respectively. At a mean follow-up of 11 months (median, 9 months; range, 6-19 months), visual acuity was stable or improved in 7 patients, and resolution of optic disk hyperemia and edema was found in all 9 patients. The mean time to improvement in visual acuity by > or =2 lines was 3 weeks (median, 1 week; range, 1-12 weeks). The mean time to complete resolution of radiation papillopathy was 4 months. The two patients with worse final visual acuity also had macular hole and central retinal vein obstruction. The only complication of this therapy was possibly related cataract in three patients. CONCLUSION: During short-term follow-up, acute radiation-induced papillopathy appears to respond rapidly to intravitreal triamcinolone acetonide injection with resolution of optic disk hyperemia and edema and modest return of visual acuity. The long-term effects remain unknown.

Acute Disease↗
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Two discrete uveal melanomas in a child with ocular melanocytosis.

OBJECTIVE: To describe a case of two uveal melanomas in a child with mild ocular melanocytosis. METHODS: A 6-year-old girl was followed for 5 years with an ill-defined, slowly enlarging presumed choroidal nevus in the postequatorial fundus. Ocular oncology evaluation revealed mild sectorial scleral and uveal melanocytosis and an episcleral sentinel vessel superotemporally. Two discrete uveal melanomas were present. In the circumpapillary and macular region, tumor 1 was diffuse at 9.0 mm in base and 4.1 mm in thickness and with overlying subretinal fluid. In the ciliary body, tumor 2 was discovered by transillumination and was 6.0 mm in base and 2.2 mm in thickness. Enucleation was performed. RESULTS: Histopathologic analysis disclosed two discrete uveal melanomas in a bed of diffuse mild uveal melanocytosis. Tumor 1 was a mixed, predominantly epithelioid cell melanoma with active mitotic figures, and tumor 2 was a mixed, predominantly spindle cell melanoma. The choroid between the melanomas showed only benign, dendritic melanocytes consistent with melanocytosis. There was no extrascleral extension. CONCLUSIONS: Ocular melanocytosis can predispose to one or multiple uveal melanomas. Lifetime ophthalmic monitoring of affected patients is warranted.

Child↗
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Orbital lymphoma simulating thyroid orbitopathy.

An 82-year-old man with hypothyroidism had vertical diplopia and swelling around his left eye. Visual acuity was 20/20 OD and 20/50 OS. There was moderate blepharoptosis and edema of the left eyelids and superior scleral show of the right eye. The left eye showed 4 mm of proptosis, motility restriction, afferent pupillary defect, and normal optic disc. Orbital MRI revealed enlargement of the left superior and medial rectus muscles without tendinous involvement. These findings were initially suggestive of thyroid orbitopathy. Thyroid function tests were normal. Coronal MRI showed additional superior oblique enlargement and involvement of the levator superioris palpebrae muscle, which are both suggestive of a non-thyroid pathology. Muscle biopsy revealed large B-cell lymphoma. The patient was treated with chemotherapy, immunotherapy, and radiotherapy, with complete tumor control. Orbital lymphoma can simulate thyroid orbitopathy, even in patients with classic "thyroid-like" symptoms and imaging.

Aged, 80 and over↗
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Solitary intraosseous orbital myofibroma in four cases.

PURPOSE: To report four pediatric cases of a bone-destructive orbital tumor that proved to be a benign solitary intraosseous myofibroma. METHODS: Four children, aged 1, 7, 11, and 72 months, developed eyelid fullness or slight proptosis. A solitary intraosseous mass was detected in the inferior (n = 2), superotemporal (n = 1), and supranasal (n = 1) orbital walls. On CT, the mass showed bone destruction. On MRI, the mass appeared circumscribed within a shell of normal surrounding bone and with features of isointensity on T1-weighted images, intense homogeneous gadolinium enhancement, and midrange signal on T2-weighted images. Complete surgical excision of the mass in each case was performed. RESULTS: On histopathologic examination, the tumors were characterized by whorls and bands of plump myoid cells with eosinophilic cytoplasm consistent with myofibroblasts, sometimes associated with a hemangiopericytoma-like vascular pattern. The diagnosis was also supported by immunohistochemical studies showing positive immunoreactivity for vimentin and smooth muscle actin. None of the patients have shown recurrence in follow-up ranging from 6 months to 3 years. CONCLUSIONS: Myofibroma is a benign tumor that can occur in the orbital bony wall of children. It can manifest as a bone destructive mass and can simulate a malignant process.

Child↗