Jarosław Kocięcki

Oral mucosal manifestations in primary and secondary Sjögren syndrome and dry mouth syndrome

Introduction One of the most important symptoms of Sjögren syndrome is xerostomia. The oral cavity deprived of saliva and its natural lubricative, protective and antibacterial properties is prone to a number of unfavourable consequences. Aim To present the most important lesions on the oral mucosa in primary and secondary Sjögren syndrome and in dry mouth syndrome. Material and methods The study group comprised 55 patients including 52 women and 3 men aged 20–72 years (average: 28.25 years). Results Basing on the accepted criteria, primary Sjögren syndrome was diagnosed in 22 (40%) patients, secondary Sjögren syndrome in 18 (32.7%) patients, and dry mouth syndrome in 15 (27.27%) patients. The physical examination and the examination of the mouth were performed and history was elicited from every patient. Conclusions The most common pathologies appearing on the oral mucosa in primary and secondary Sjögren syndrome are angular cheilitis, cheilitis, increased lip dryness as well as non-specific ulcerations, aphthae and aphthoid conditions.

The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group

PURPOSE To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI. DESIGN Retrospective, multicenter observational study. PARTICIPANTS Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults. METHODS Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression. MAIN OUTCOME MEASURES Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality. RESULTS Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival. CONCLUSIONS This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups.

An update on thyroid-associated ophthalmopathy in children and adolescents.

Abstract Background: Risk of developing thyroid-associated ophthalmopathy (TAO) in children and adolescents is similar or may be even slightly higher than in adults. The aim of this article is to review and summarize current knowledge regarding diagnostic and therapeutic measures in pediatric TAO. Content: MEDLINE and EMBASE papers were searched using the terms ‘pediatric Graves’ ophthalmopathy’ ‘pediatric Graves’ orbitopathy’, ‘thyroid-associated ophthalmopathy in childhood and adolescence’ from the year 1970 to December 2015. Summary: TAO usually accompanies hyperthyreosis in Graves’ disease, but may also occur in patients with hypothyreosis due to chronic lymphocytic thyroiditis (Hashimoto’s disease) or in euthyroid patients. Current information regarding epidemiology, pathogenesis, symptoms and treatment of TAO in children and adolescents is presented. The course of the disease is usually mild, eye disturbances often regress after restoring euthyroidism and a ‘wait and see’ policy is appropriate in the majority of patients. In rare cases, sight-threatening complications [dysthyroid optic neuropathy (DON) or corneal breakdown] may develop and immediate surgical intervention might become necessary. Outlook: Close cooperation between pediatric endocrinologists and opthalmologists is extremely important to ensure best care and quality of life in patients with thyroid gland dysfunction. Further investigations on pathogenesis and course of TAO in children and adolescents should be performed for better management of this disease in this group of patients.

Primary uveal lymphoma effectively managed with oral chlorambucil: a case report

IntroductionOcular lymphomas account for five to 10 percent of all extra-nodal lymphomas. Primary uveal lymphoma is quite a rare entity and usually unilateral. We present a case of a primary uveal lymphoma with conjunctival and orbital extension, successfully managed with oral chlorambucil.Case presentationA 71-year-old Caucasian man presented to our facility with visual loss in his only functioning eye (left). On clinical examination, we found a conjunctival lesion with a choroidal infiltration and a secondary retinal detachment. Ultrasound and magnetic resonance imaging studies revealed a choroidal tumour mass and two other lesions around the optic nerve. Results from an incisional biopsy revealed a low-grade B-cell lymphoma (CD20+, CD43+, bcl2+, CD3-). A diagnosis of primary uveal lymphoma was made. Our patient was started on a chemotherapy regime with no effect, and then oral chlorambucil was administered, with a relatively good result. At 10 months after the start of chlorambucil treatment, a best-corrected visual acuity of 0.4 was recorded, the choroidal mass had practically disappeared and the extra-ocular lesions had shrunk.ConclusionsIn all, 61 to 80 cases of primary uveal lymphoma have already been described in the literature. Generally, it is an indolent tumor with a good prognosis. However, there are some reports of aggressive tumor behavior a few years after initial diagnosis (about eight percent of cases). Other treatment options are orbital irradiation at low doses (20 to 40 Gy) or steroid administration. This is the first documented report of the efficacy of oral chlorambucil in the treatment of primary uveal lymphoma.

Anatomy and function of the macula in patients after retinectomy for retinal detachment complicated by proliferative vitreoretinopathy

Purpose TO investigate the anatomy and function of the macula in patients after retinectomy for retinal detachment complicated by proliferative vitreoretinopathy (PVR) grade C. Methods A prospective study involving 25 patients operated with vitrectomy and retinectomy due to retinal detachment complicated by PVR grade C. Outcome measures included preoperative and postoperative best-corrected visual acuity (BCVA) at 6 months and the difference between preoperative and postoperative BCVA. Furthermore, the macular status was analyzed on postoperative optical coherence tomography (OCT) scans. Results Reattachment was observed in 96% (24/25) of patients. Median BCVA improved from 2.30 logMAR (range 1.00 to 2.69 logMAR) to 1.00 logMAR (range 0.38 to 2.90 logMAR). The change from preoperative visual acuity was statistically significant (p=0.0003, Wilcoxon signed rank test). Macular status with pathologies was observed in 75% of eyes on OCT (retinal pigment epithelium irregularities 37.5%, cystoid macular edema 33%, epiretinal membrane 8.3%, subretinal fluid 4%, and subretinal perfluorocarbon liquid 4%). These eyes had significantly worse final visual acuity (p=0.01, Wilcoxon rank sum test) compared to those with a clinically normal macula. The retinectomy size influenced neither visual acuity (p=0.06, Wilcoxon rank sum test) nor incidence of normal macular status (p=0.14, Fisher exact test). Conclusions Even though retinectomy is an effective technique to reattach the retina in complicated cases of retinal detachment, the functional outcome was found to be influenced by abnormal macular status, observed in 75% of eyes. Moreover, 360-degree retinectomies did not show different anatomic or functional results vs subtotal ones.

New insight into the pathology of macular detachment associated with an optic disc pit

Letter: New insight into the pathology of macular detachment associated with an optic disc pit

Toxocarosis of the organ of sight – the complex pathological and diagnostic problem

Selected parasitological and epidemiological aspects of Toxocara spp. invasion were discussed. Pathomechanism of lesions and involvement of the organ of sight in toxocarosis were presented. It was stressed that pathological lesions may involve various structures of the eyeball. Moreover, the pattern of lesions may vary depending on the early or late stage of the invasion. Diagnostic techniques were presented, indispensable in confirmation of toxocarosis and establishing its duration. In diagnosis of the ocular form of toxocarosis the significance of differential diagnostic analysis was stressed, particularly essential in oligosymptomatic cases and upon coexistence of other diseases progressing with involvement of the organ of sight. This is important for further specialised management and in selection of an appropriate therapy.

Small-cell lung carcinoma metastasis to the iris – case presentation

Introduction Metastatic tumors are the most common intraocular malignancies in adults. Anterior segment metastases account for about 7.2–14.1% of all intraocular metastatic tumors with those to the iris representing 3.6–7.8% of the cases. Breast and lung are the most frequent primary sites of metastasizing cancer. The aim of this study is to present the case of a 43-year-old patient with a rare small-cell lung carcinoma metastasis to the iris. Case report A patient with small-cell lung carcinoma and metastases in the brain who had been treated with chemo- and radiotherapy attended an outpatient clinic because of redness of her left eye. Based on the ophthalmological examination, a diagnosis of numerous metastatic tumors to the iris was made and the patient was referred for re-chemotherapy. Complete regression of the iris lesions was observed after 5 months but unfortunately two months later the patient died, due to progression of her systemic disease. Discussion Among lung cancers, adenocarcinomas are much more likely to produce distant metastases to the iris than small-cell carcinomas [4]. More than 50% of patients with small-cell lung carcinoma have metastases in the brain. The survival rate in cases of lung cancer with eye involvement is poor and approximates to 7 months. However, since there is a strong probability of angle involvement and subsequent secondary glaucoma, the early onset of treatment can improve the patients quality of life during the terminal phase of the disease.

Poststreptococcal syndrome mimicking conjunctival lymphoma

BackgroundPoststreptococcal syndrome (PSS) can be a consequence of nonpurulent primary infection with group A streptococci (GAS). Postreptococcal uveitis is a well recognized entity with quite a few descriptions in the literature, but so far no conjunctival involvement has been reported.The aim of the study is to present a rare case of postreptococcal conjunctival lesions mimicking a lymphoma.Case presentation19-years-old Caucasian female presented with pink, nodular infiltrates in the right conjunctiva that occurred a few months after upper respiratory tract infection and tonsillectomy. Histopathological examination of collected lesion samples revealed inflammatory reaction with lymphocytes proliferation and failed to rule out a myeloma. Complementary flow-cytometry did not show monoclonal proliferation of lymphocytes B. During follow-up we observed the complete regression of conjunctival lesions after the benzyl penicillin treatment prescribed by ENT specialist due to elevated plasma ASO levels. Therefore, we suppose that those lesions must have represented a part of poststreptococcal syndrome.ConclusionsTo conclude, this is, to the best of our knowledge, the first report of conjunctival involvement in the course of PSS related to group A streptococci.

A pedicled autologous choroid RPE patch: a technique to preserve perfusion

The aim of the study is to report a technique of a pedicled autologous choroid retinal pigment epithelium (RPE) patch that aims to preserve perfusion of the transplanted tissue. A case report of a patient with sudden vision deterioration due to submacular hemorrhage in age-related macular degeneration. The surgery involved a 180-degree peripheral retinectomy and the creation of a pedicled graft instead of an isolated one. Outcome measures included preoperative and postoperative visual acuity and optical coherence tomography scans at 1, 3, 6, 12 months and patch vascularization on postoperative indocyanine green angiography. Postoperatively the patch was positioned under the fovea with an intact pedicle. Indocyanine green angiography showed perfusion through the pedicle and patch vasculature on the third postoperative day. Best corrected visual acuity improved from 0.5/50 to 5/50 at 1 month and remained stable over 1 year follow-up. No choroidal neovascularization recurrence was observed. This case report demonstrates the feasibility of a pedicled RPE-choroid graft that is an alternative to a free isolated graft. Our modification of patch surgery, by demonstrating early perfusion, offers an advantage, similar to macular translocation, when photoreceptors are embedded in RPE and choroid with blood circulation immediately after the surgery.