Biomedical subjects
C L Shields
Publications and source records attributed to C L Shields.
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.
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.
Leukemic infiltration of the optic nerve.
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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.
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.
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.
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.
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.
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.
Massive orbital extension of posterior uveal melanomas.
Among 2,135 consecutive patients with posterior uveal melanoma who were evaluated clinically in the Ocular Oncology Service of Wills Eye Hospital between February 1974 and December 1986, 123 (5.8%) were found to have some degree of extrascleral extension of the tumor. Ten of the 2,135 patients (0.5%) had massive orbital extension we defined as extrascleral tumor having an estimated volume exceeding 1,000 mm3. Three of these patients were characterized by orbital recurrence sometime after enucleation, whereas seven had massive orbital extension as the initial manifestation of the neoplasm with no prior history of enucleation. These cases of advanced orbital extension are presented in detail with emphasis on clinical features, diagnostic problems, pathologic findings, and follow-up observation. Based on our observations, suggestions are made regarding the diagnosis and management of advanced orbital extension of posterior uveal melanoma.
Magnetic resonance imaging in choroidal osteoma.
High resolution magnetic resonance imaging (MRI) with surface coil and contrast material was used to demonstrate the presence of bone in choroidal osteoma. Contrary to initial impressions, choroidal osteoma does not show typical negative image of the bone in MRI scans. The ossifying tumor of the choroid appears as a bright (hyperintense) signal on T1-weighted images and as an area of relative low intensity on T2-weighted images. On contrast T1-weighted scans, the tumor showed gadolinium-DPTA (Gd-DPTA) enhancement. These MRI findings are discussed in relation with histopathologic features of choroidal osteoma and the presence of fat marrow in the intertrabecular spaces of the bone at the level of the choroid.
Orbital exenteration with eyelid sparing: indications, technique, and results.
Of 22 orbital exenterations performed from January 1980 to December 1989, 16 were performed by an eyelid-sparing technique, and six were allowed to heal by spontaneous granulation. Reviewing these procedures, we conclude that the eyelid-sparing method is indicated mainly for advanced primary orbital malignancies and for certain secondary orbital malignancies such as orbital extension of uveal melanoma, conjunctival melanoma, and conjunctival squamous cell carcinoma. It is not usually applicable to orbital invasion of malignant eyelid tumors such as basal cell carcinoma and sebaceous gland carcinoma. Advantages of the eyelid-sparing method are more rapid healing and earlier fitting of a prosthesis.
Malignant tumors of the eye in geriatric patients.
The ocular tissues can be the site of a number of malignant tumors in adults and geriatric patients. In addition to posing a threat to the patient's life, these tumors can cause severe visual loss or blindness. Therefore, the primary care clinician should be capable of prompt diagnosis of the various malignant ocular tumors and be prepared to refer the patient for appropriate management. This article provides a photographic guide to the most common primary and secondary malignancies that can affect the eyelid, conjunctiva, intraocular structures, and orbit in the geriatric patient.
Epithelial downgrowth following the removal of iris inclusion cysts.
We present three patients in whom epithelial downgrowth occurred following the excision of iris inclusion cysts. The sheet-like downgrowth was aggressively treated and successfully eradicated in two of the three patients. Early identification and prompt therapy are essential.
Differential diagnosis of retinoblastoma.
Retinoblastoma is a malignant intraocular tumor of childhood that requires accurate diagnosis and prompt treatment. It is well known that several other ocular conditions of childhood can clinically simulate retinoblastoma. Knowledge of the clinical features that serve to differentiate retinoblastoma from simulating lesions may assist the clinician in arriving at the correct diagnosis and preventing misdirected therapy. This review provides a simple classification of those pseudoretinoblastomas and reviews the recent literature on this subject. It emphasizes the clinical features that help differentiate pseudoretinoblastomas from true retinoblastoma.
Band keratopathy secondary to an iris melanoma.
A 27-year-old white man presented with a sectoral band keratopathy overlying an iris melanoma. Clinically, it appeared as a 3 x 3-mm yellowish-white, granular subepithelial deposit in the inferonasal right cornea. Occasional small, round holes were scattered within the opacity. Clinical presentation of this lesion is described, and this unusual cause of band keratopathy is discussed.
Radiotherapy of retinoblastoma. A review of 63 children treated with different irradiation techniques.
This is a retrospective review of treatment results in 63 children with retinoblastoma (RB) who received therapeutic irradiation from January 1975 to June 1988. Patient ages ranged from birth to 5 years with a mean age of 12.5 months. Three treatment groups were identified. In Group A, episcleral plaque was the only irradiation modality. In this group, 24 patients and 25 eyes with tumors of Reese-Ellsworth Stages I to III, and selected Stage VB tumors were irradiated using eye plaques. Twenty-two of 25 (88%) eyes are without evidence of disease (NED), and all patients are alive. Two eyes were enucleated for recurrent RB. Two of 23 eyes developed minimal cataracts. In Group B, external-beam was the only irradiation modality. Twelve patients with 21 involved eyes comprise this group. Thirteen eyes received EBRT alone. Seventy-seven percent of 13 eyes have NED. Four eyes developed cataracts. Three of 13 eyes required enucleation for progression of disease; one of these was Stage IVA and two of them were Stage VA. For Group C, external-beam plus episcleral plaque was used. Twenty-seven patients and 51 eyes with advanced retinoblastoma were included in this group. Episcleral plaque was used in 29 eyes as a salvage procedure for recurrent RB, or in selected cases was used as a boost after initial external beam irradiation. Fifteen of 29 eyes (52%) remain with NED with useful vision, and nine developed cataracts. Enucleation was performed in 14 eyes for recurrent RB. The authors conclude that radiation therapy has a major role in the management of RB due to its ability to control even advanced disease, allowing preservation of vision in a significant number of patients.