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Biomedical subjects

Santosh G Honavar

Publications and source records attributed to Santosh G Honavar.

At least 19 recordsLinked to original sources

Pseudoepitheliomatous hyperplasia mimicking ocular surface squamous neoplasia following cultivated limbal epithelium transplantation.

A 12-year-old girl with total limbal stem cell deficiency in the right eye following chemical burns underwent autologous cultivated limbal epithelium transplantation from the healthy left eye. Postoperatively at 6 weeks a mass at the limbus was noted, which increased in size and involved infero-nasal limbus extending over 5 mm on bulbar conjunctiva. It was a gelatinous, placoid freely movable mass with irregular surface, multiple intralesional cysts without feeder vessels or intrinsic vascularization and stained brilliantly with rose bengal. Histopathology following excision biopsy showed hyperplastic epithelium with stratified columnar cells and goblet cells. At the last follow-up, 6 months following cultivated limbal epithelium transplantation the ocular surface was stable without any recurrence of the lesion. We herein report a rare complication of epithelial hyperplasia presenting as leukoplakia following cultivated limbal epithelium transplantation mimicking ocular surface squamous neoplasia.

Carcinoma, Squamous Cell↗

Ocular surface squamous neoplasia in Papillon-Lefevre syndrome.

PURPOSE: To report the occurrence of ocular surface squamous neoplasia (OSSN) in a patient with Papillon-Lefevre syndrome. DESIGN: Interventional case report. METHODS: A 14-year-old boy presented with a progressively growing limbal lesion in the right eye for 2 months. He had associated erythematous scaly lesions on the elbows, knees, and feet with periodontosis suggestive of Papillon-Lefevre syndrome. An excision biopsy of the limbal tumor and a skin biopsy was performed. RESULTS: The limbal tumor showed features of carcinoma in situ with clear margins. The skin biopsy showed epidermal hyperplasia and hyperkeratosis with perivascular infiltrates, consistent with Papillon-Lefevre syndrome. CONCLUSIONS: Ocular surface squamous neoplasia may occur in patients with Papillon-Lefevre syndrome, an association that to the best of our knowledge has not been previously reported.

Adolescent↗

Ethanolamine oleate sclerotherapy in the management of orbito-palpebral cyst associated with congenital microphthalmos.

PURPOSE: To report a novel, nonsurgical method of management of orbito-palpebral cyst associated with congenital microphthalmos. DESIGN: Interventional case series. METHODS: Two patients with orbito-palpebral cyst were included. Case 1 had bilateral orbito-palpebral cyst, whereas case 2 was unilateral. Transcutaneous cyst aspiration in both cases revealed a severely microphthalmic eye with no visual potential. Ethanolamine oleate sclerotherapy was performed immediately after cyst aspiration in both patients. RESULTS: Complete resolution of the orbito-palpebral cysts was noted within 6 weeks, and the custom prosthesis was well retained. CONCLUSIONS: Ethanolamine oleate sclerotherapy may be an effective minimal intervention treatment option for cosmetic rehabilitation of patients with orbito-palpebral cyst associated with congenital microphthalmos with no visual potential.

Cysts↗

Polycystic echinococcosis of the orbit.

PURPOSE: To report a case of polycystic echinococcosis of the orbit caused by Echinococcus oligarthrus. DESIGN: Interventional case report. METHODS: A 14-year-old girl presented with a massive proptosis of the right eye of 9 years duration and loss of vision. The eye was phthisic, and a cystic mass was palpable. Computed tomography scan showed closely packed multiple orbital cysts with destruction of the medial orbital wall. Surgical exploration was performed. RESULTS: The phthisic eye was enucleated. Dissection of the outer fibrous capsule yielded approximately 30 intact translucent white cysts of various sizes that were suggestive of polycystic echinococcosis. The diagnosis of echinococcosis was confirmed on histopathologic evaluation. The specific diagnosis of E oligarthrus was made on the basis of the presence of straight-backed hooks and central guards. CONCLUSION: Polycystic echinococcosis of the orbit caused by E oligarthrus is rare. It may be an entirely new entity or hitherto unrecognized form of orbital echinococcosis.

Adolescent↗

Neoadjuvant chemotherapy in the management of sebaceous gland carcinoma of the eyelid with regional lymph node metastasis.

A 55-year-old Asian Indian woman who had recurrent sebaceous gland carcinoma of the left lower eyelid with orbital extension and regional lymph node metastasis was treated with neoadjuvant chemotherapy, using a combination of carboplatin and 5-fluorouracil. Eyelid-sparing orbital exenteration was performed after 3 cycles of chemotherapy, followed by radiotherapy to the regional lymph nodes. Subsequently, 3 cycles of adjuvant chemotherapy were administered. Significant eyelid and orbital tumor volume reduction was achieved with neoadjuvant chemotherapy, making eyelid-sparing orbital exenteration possible. Chemotherapy also spared the patient from radical neck dissection. The patient had limited morbidity and was free of local, regional, and systemic disease at 26 months of follow-up.

Adenocarcinoma, Sebaceous↗

Rapid intraoperative diagnosis of tumors of the eye and orbit by squash and imprint cytology.

PURPOSE: To evaluate the diagnostic accuracy of intraoperative squash and imprint cytologic techniques in ocular and orbital lesions. DESIGN: Observational case series. METHODS: Forty-five consecutive ocular and orbital biopsy specimens subjected to squash and imprint cytologic techniques for rapid intraoperative diagnosis were included in this study. The indications for which the surgeon requested intraoperative diagnosis included: (1) infiltrative lesions where a preoperative tissue diagnosis was not available, (2) discrepancy between preoperative and intraoperative clinical diagnosis, and (3) unusual clinical presentations with an uncertain diagnosis. Fresh unfixed tissue was used for making squash preparations and imprint smears. Alcohol-fixed smears were stained with hematoxylin and eosin, whereas air-dried smears were stained with the Giemsa stain. The residual tissue was subjected to routine processing for histopathologic examination. MAIN OUTCOME MEASURE: Correlation of intraoperative cytologic diagnosis with the final histologic diagnosis was the primary outcome measure. The correlation was considered to have complete concordance if the diagnosis matched with respect to nature of the lesion (benign vs. malignant) as well as with the further classification of the benign or the malignant lesion. Partial concordance was considered when there was agreement with respect to the nature of the lesion, but there was a mismatch in the further classification of the benign or the malignant lesion. RESULTS: Biopsies were obtained from 45 patients whose ages ranged from 1 to 79 years (median, 21 years). The sites of biopsy included eyelids (n = 4), conjunctiva (n = 6), intraocular tissue (n = 3), and orbit (n = 32). Adequate cellularity was obtained in all cases by squash and imprint techniques. A cytologic diagnosis could be made within 5 to 8 minutes after receiving the sample. Of 44 cases in which a cytologic diagnosis could be made, 21 (48%) were malignant and 23 (52%) were benign. Cytologic analysis was inconclusive in 1 case. The cytologic-histologic correlation showed complete concordance in 41 of 45 cases (91%) and partial concordance in 3 cases. Sensitivity and specificity of the squash and imprint technique in detecting malignant lesions was 100%. CONCLUSIONS: Assuming the availability of a skilled cytologist, ophthalmic pathologist, or both, squash and imprint preparations are useful for rapid and reliable intraoperative diagnosis of ocular and orbital lesions.

Adolescent↗

Mutational screening of the RB1 gene in Indian patients with retinoblastoma reveals eight novel and several recurrent mutations.

Retinoblastoma is the most common primary intraocular malignancy in children, caused by inactivation of the RB1 gene on chromosome 13. We carried out a mutational screen of the exons and promoter of the RB1 gene in Indian patients with retinoblastoma in order to determine the range of mutations giving rise to disease. Forty-seven patients were screened for mutations in all exons and promoter of the RB1 gene by single strand conformation polymorphism followed by sequencing. Tumors were available from 27 patients (12 bilateral and 15 unilateral retinoblastoma) while only peripheral blood was available from 20 patients, all with bilateral disease. Mutations were found in 22 patients, 9 from the analysis of tumors and 13 from peripheral blood. Eight novel mutations were identified, including 4 single base changes, 2 small deletions and 1 duplication. These are g.64365T>G (Tyr325Ter), g.78131G>A (Trp515Ter), g.150061G>T (Glu587Ter), g.170383C>G (S834X), g.41924A>C (IVS3-2A>C), g.150064ins4, g.160792del22, and g.76940del14 (IVS15 del +20-33). Almost all mutations produced nonsense codons or frameshifts. Recurrent mutations, especially at CpG sites were seen predominantly. Detectable mutations in exons were found in 46% of patients tested. Large deletions, epigenetic changes as well as mutations in non-coding regions may be the cause of disease in the remainder of patients. Knowledge of the full range of mutations can aid in the design of screening tests for individuals at risk.

Child↗

Chemoreduction for unilateral retinoblastoma.

OBJECTIVE: To evaluate conservative management of unilateral retinoblastoma using chemoreduction and focal treatment. DESIGN: Prospective nonrandomized single-center clinical trial. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, Philadelphia, Pa, in conjunction with the Division of Oncology at The Children's Hospital of Philadelphia. PARTICIPANTS: Thirty eyes of 30 patients with unilateral retinoblastoma treated with chemoreduction between June 1, 1994, and August 31, 1999, that would otherwise have been managed with enucleation or external beam radiotherapy. INTERVENTION: All patients received treatment for retinoblastoma with a planned 6 cycles of chemoreduction using vincristine sulfate, etoposide, and carboplatin, combined with focal treatment (cryotherapy or thermotherapy) to each retinal tumor. MAIN OUTCOME MEASURES: The main outcome measure was the postchemoreduction need for external beam radiotherapy or enucleation. The cumulative probability of each outcome was estimated using Kaplan-Meier survival analysis. A secondary outcome measure was final visual acuity in the affected eye. The clinical features at the time of patient presentation were analyzed for their impact on the main outcomes using a series of Fisher exact tests and Cox proportional hazards regressions. RESULTS: Eighteen eyes (60%) were classified as having Reese-Ellsworth (RE) groups I through IV retinoblastoma and 12 eyes (40%), group V retinoblastoma. By using Kaplan-Meier estimates, we found a need for either external beam radiotherapy or enucleation in 68% of eyes by 5 years. In fact, 38% of those in groups I through IV required either treatment, whereas all of those in group V required the additional use of either treatment. Specifically, the need for external beam radiotherapy occurred in 27% of eyes by 5 years. Eleven percent of those in groups I through IV and 50% of group V required external beam radiotherapy by 5 years. The factors predictive of the need for external beam radiotherapy included RE group V disease, tumor thickness greater than 5 mm, and presence of vitreous seeds. The need for enucleation occurred in 47% of eyes by 5 years using Kaplan-Meier analysis. Specifically, 29% of those in groups I through IV and 67% of group V required enucleation by 5 years. The factors predictive of the need for enucleation included age at diagnosis older than 12 months, RE group V disease, tumor base diameter greater than 15 mm, and tumor thickness greater than 5 mm. At a mean follow-up of 29 months, the final visual acuity was 20/200 or better in 6 eyes (20%) and worse than 20/200 in 14 (47%); enucleation was needed in 10 (33%). Of the 26 eyes with initial macular involvement of retinoblastoma, final visual acuity was 20/200 or better in 6 (23%). No patient developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms. CONCLUSIONS: Chemoreduction is an option for selected eyes with unilateral retinoblastoma. Those with advanced RE group V retinoblastoma showed poorest results, while those with less advanced groups I through IV disease showed best results, maintaining the globe in 71% of eyes, sometimes with satisfactory functional visual acuity.

Antineoplastic Combined Chemotherapy Protocols↗

Enzymatic sclerostomy: pilot human study.

OBJECTIVE: To evaluate the feasibility and safety of enzymatic sclerostomy as a new modality to lower intraocular pressure in patients with open-angle glaucoma. METHODS: This single-center, prospective, noncomparative, interventional case series included 15 blind symptomatic eyes of 15 patients with primary open-angle glaucoma. Enzymatic sclerostomy was performed with the patient under topical or peribulbar anesthesia. A specially designed polymethylmethacrylate enzyme applicator filled with a mean +/- SD of 123 +/- 13 microg of collagenase was introduced through a 5-mm peritomy, and affixed to the limbus by means of cyanoacrylate tissue glue. After 22 to 24 hours, the applicators were removed and the patients were followed up for 1 year. Intraocular pressure changes from baseline and complications related to the procedure were the main outcome measures. RESULTS: Controlled thinning of the treated sclera associated with aqueous percolation and shallow filtration bleb was seen in all eyes in the immediate postoperative period. The mean +/- SD intraocular pressure decreased from 43.5 +/- 9.8 mm Hg (while the patients were receiving a mean +/- SD of 1.75 +/- 0.75 antiglaucoma medications) preoperatively to 24.8 +/- 10.6 mm Hg (a 43.0% decrease from baseline with no antiglaucoma medication) on the first postoperative day and to 34.8 +/- 10.5 mm Hg (a 20.0% decrease from baseline with no antiglaucoma medication) at the end of 1 year. Ophthalmic adverse effects were limited to the treated area and included immediate postoperative transient conjunctival reaction ranging from mild chemosis to conjunctival maceration. Immediate full-thickness perforation developed in 1 eye; the patient was treated and excluded from data analysis. Two eyes developed symptoms related to increase in intraocular pressure after 9 months; the patients were treated and excluded from further data analysis. No systemic complications were noted. CONCLUSIONS: Enzymatic sclerostomy demonstrated immediate and sustained intraocular pressure reduction and provided symptomatic relief in blind eyes with primary open-angle glaucoma. The procedure, however, needs further technical refinement.

Aged↗

Postenucleation adjuvant therapy in high-risk retinoblastoma.

PURPOSE: The main purpose of this study was to determine the efficacy of postenucleation adjuvant therapy in preventing metastasis in cases of high-risk retinoblastoma. METHODS: This was a retrospective, nonrandomized comparative study. Of 1020 consecutive patients with retinoblastoma had were managed at a referral center between January 1974 and December 1999, 80 (8%) of those analyzed had unilateral sporadic cases that were treated by primary enucleation and that had high-risk characteristics for metastasis on histopathology reports (anterior chamber seeding, iris infiltration, ciliary body infiltration, massive choroidal infiltration, invasion of optic nerve lamina cribrosa, retrolaminar optic nerve invasion, invasion of optic nerve transection, scleral infiltration, and extrascleral extension). The main outcome measure was the development of metastasis at a minimum follow-up period of 12 months. RESULTS: There were 44 male and 36 female patients, with age ranging from 1 day to 16 years (median, 33 months). A single histopathologic high-risk characteristic was present in 50 patients (62.5%). Thirty patients (37.5%) manifested 2 or more high-risk characteristics. Forty-six patients (58%) had received postenucleation adjuvant therapy (chemotherapy with or without orbital external beam radiotherapy). Adjuvant therapy was not administered in 34 patients (42%). Metastasis occurred in 10 patients (13%) at a median of 9 months (range, 6-57 months) following enucleation. Eight (80%) of those who developed metastasis had not received adjuvant therapy. A significant difference (P =.02) was found in the incidence of metastasis between the group that had received adjuvant therapy (4%; 2/46) and the group that had not (24%; 8/34). The beneficial effect of adjuvant therapy was statistically significant in subgroups of patients with massive choroidal infiltration (P =.04) or retrolaminar optic nerve invasion (P =.04). There were no adjuvant therapy-related serious systemic complications. CONCLUSION: Postenucleation adjuvant therapy is safe and effective in significantly reducing the occurrence of metastasis in patients with retinoblastoma manifesting histopathologic high-risk characteristics.

Adolescent↗

Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation.

PURPOSE: To report the results of chemoreduction and focal therapy for retinoblastoma with determination of factors predictive of the need for treatment with external beam radiotherapy or enucleation. DESIGN: Interventional case series. METHODS: One-hundred three patients with retinoblastoma (158 eyes with 364 tumors) at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at Children's Hospital of Philadelphia from June 1994 to August 1999 were enrolled for this prospective clinical trial. The patients received treatment for retinoblastoma with six planned cycles (one cycle per month) of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatments (cryotherapy, thermotherapy, or plaque radiotherapy). The two main outcome measures after chemoreduction and focal therapy were the need for external beam radiotherapy and the need for enucleation. The clinical features at the time of patient presentation were analyzed for impact on the main outcome measures using a series of Cox proportional hazards regressions. RESULTS: Using Reese-Ellsworth (RE) staging for retinoblastoma, there were nine (6%) eyes with group I disease, 26 (16%) eyes with group II disease, 16 (10%) eyes with group III disease, 32 (20%) eyes with group IV disease, and 75 (48%) eyes with group V retinoblastoma. All eyes showed initial favorable response with tumor regression. The median follow-up was 28 months (range, 2-63 months). Failure of chemoreduction and need for treatment with external beam radiotherapy occurred in 25% of eyes at 1 year, 27% at 3 years, and no further increase at 5 years. More specifically, external beam radiotherapy was necessary at 5 years in 10% of RE groups I-IV eyes and 47% of RE group V eyes. Multivariate factors predictive of treatment with external beam radiotherapy included non-Caucasian race, male sex, and RE group V disease. Failure of chemoreduction and the need for treatment with enucleation occurred in 13% eyes at 1 year, 29% at 3 years, and 34% at 5 years. More specifically, enucleation was necessary in 15% of RE groups I-IV eyes at 5 years and in 53% of RE group V at 5 years. Multivariate factors predictive of treatment with enucleation included patient age older than 12 months, single tumor in eye, and tumor proximity to foveola within 2 mm. Overall, of the 158 eyes, 50% required external beam radiotherapy or enucleation and 50% were successfully managed without these treatments. No patient developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms over the 5-year follow up. CONCLUSIONS: Chemoreduction offers satisfactory retinoblastoma control for RE groups I-IV eyes, with treatment failure necessitating additional external beam radiotherapy in only 10% of eyes and enucleation in 15% of eyes at 5-year follow-up. Patients with RE group V eyes require external beam radiotherapy in 47% and enucleation in 53% at 5 years.

Antineoplastic Combined Chemotherapy Protocols↗

Orbital tumors in the older adult population.

PURPOSE: To determine the types and frequency of orbital tumors in the older adult population. DESIGN: Retrospective consecutive case series. PARTICIPANTS: Two hundred consecutive patients aged 60 years or older with an orbital tumor managed at a tertiary referral center. MAIN OUTCOME MEASURES: The relative incidence and management of orbital tumors in patients aged 60 years or older. RESULTS: Of approximately 950 consecutive patients with orbital space-occupying lesions managed over a 25-year period on the Oncology Service, 200 patients (21%) were 60 years or older at the time of diagnosis. The orbital tumor was unilateral in 183 patients (92%) and bilateral in 17 patients (8%). The most common clinical features at presentation included mass in 51 patients (26%), proptosis in 36 (18%), and pain in 30 (15%). The mean duration of symptoms was 11 months before referral. The orbital tumor was malignant in 125 patients (63%) and benign in 75 (27%). The most common diagnoses were malignant lymphoma in 47 patients (24%), idiopathic orbital inflammation in 19 (10%), and cavernous hemangioma in 15 (8%). The diagnosis was made with clinical findings and imaging studies in 58 patients (29%) and additional histopathologic examination in 142 patients (71%). Of 217 tumors, the main tumor location was in the superior half of the orbit in 106 lesions (49%), in the anterior orbit in 100 (46%), and in the extraconal space in 135 (62%). Imaging studies showed a mass with well-defined margins in 90 tumors (41%) and ill-defined margins in 127 (59%). The tumors were solid in 203 cases (94%), cystic in 6 (3%), and mixed in 8 (3%). Over a mean of 31 months follow-up, 50 (25%) patients had orbital tumor-related systemic involvement (metastasis or lymphoma), and 150 (75%) remained free of systemic involvement. CONCLUSIONS: Orbital tumors in the senior adult population are malignant in 63% of cases. Malignant lymphoma is the most common tumor in this age group, accounting for 24% of cases. Overall, 25% of patients have systemic problems related to the orbital process develop, so systemic evaluation is warranted.

Aged↗

Diffuse iris melanoma: a report of 25 cases.

BACKGROUND: Diffuse iris melanoma is a rare variant of iris melanoma that has a flat growth pattern and often presents as unilateral hyperchromic heterochromia and glaucoma. There have been no large clinical reports of diffuse iris melanoma. DESIGN: Single-center retrospective case series. PARTICIPANTS: Twenty-five consecutive patients with diffuse iris melanoma. METHODS: A review of the clinical features, management, histopathologic analysis, and prognosis was performed. MAIN OUTCOME MEASURES: Clinical features, histopathologic features, management, and prognosis. RESULTS: At the time of diagnosis, the mean patient age was 49 years. The initial complaint was unilateral darker eye color in 13 cases (52%) and blurred vision in 6 (24%). Six patients (24%) were symptom free. On presentation elsewhere, the initial diagnosis was iris melanoma or nevus in 11 cases (44%) and glaucoma in 14 (56%), 8 (32%) of whom had prior laser or surgical treatment for glaucoma. The 25 patients were observed for a mean of 30 months before the diagnosis of melanoma was suspected and referral to our center for evaluation. The mean intraocular pressure at referral was 36 mmHg, and the mean number of clock hours involved by tumor was 11. Heterochromia iridis was present in all 25 cases (100%), corectopia in 23 (92%), and ectropion iridis in 21 (84%). Associated findings included unilateral cataract in 8 (32%), a prominent episcleral (sentinel) vessel in 7 (28%), and anterior chamber inflammation in 5 (20%), and synechiae (anterior or posterior) in none. The tumor was managed by enucleation in 22 cases (88%) and by plaque brachytherapy in 3 (12%). Five cases (20%) were classified as spindle melanoma, 17 (68%) as mixed cell type, and 3 (12%) as epithelioid cell type. Therefore, histopathologic examination showed that 80% of cases contained epithelioid cells. All 22 enucleated cases were found to have tumor invasion in the trabecular meshwork. Tumor invasion of Schlemm's canal was found in 18 (82%), minor pars plicata in 12 (55%), and episclera in 7 cases (28%). Of seven cases with episcleral invasion, four underwent surgical treatment for glaucoma. Excluding one recent case, the patients were observed for a mean of 78 months. Liver metastasis developed in 3 cases (13%). CONCLUSIONS: Diffuse iris melanoma is a serious ocular condition that causes unilateral hyperchromic heterochromia and secondary glaucoma, often leading to a delay in diagnosis. Local invasion of adjacent ocular structures is common, and distant metastasis occurs in 13% of cases at mean follow-up of 78 months.

Adolescent↗