To evaluate the efficacy of combined PDT and 4 mg intravitreal triamcinolone acetonide injection, performed 48-72 hours after PDT, in patients with wet form of AMD.
Nonrandomised, interventional case ...series, 13 eyes of 13 patients with subfoveal CNV due to AMD that did not respond to PDT monotherapy - 7 females, 6 males - at the age of 65-85 (average age 76.6 +/- 6.7 years); standard PDT was performed in all patients followed by a 4 mg intravitreal injection of triamcinolone acetonide given 48-72 hours after PDT. Follow up visits were scheduled 1 and 7 days after the injection and then every 3 months afterwards and included: BVCA (Snellen chart), IOP measurements, FA, OCT, slit lamp and eye fundus examination. Lesions with active CNV leakage in FA were retreated every 3 months.
Average observation time was 10.8 +/- 3.5 months. Baseline visual acuity before PDT monotherapy was applied (Vo) was 0.17 +/- 0.12 (0.06-0.5), and after the therapy decreased to (V1) 0.14 +/- 0.13 (0.05-0.2). After combined PDT and Tc treatment BVCA increased to (V2) 0.21 +/- 0.13 (0.06-0.5), p<0,03. 76,9% of patients gained or maintained visual acuity after combined therapy in the observation time. In 70% of eyes no signs of active CNV was observed in AF and OCT after 1 session of combined PDT and Tc treatment. Only 4 patients required 1 repeated treatment session.
1. Combination of PDT and IVTA may be effective in patients with wet AMD with no response to PDT alone and significantly reduces the repeated treatment rate. 2. Intravitreal Tc injection performed 48-72 hours after PDT may improve the final functional effects in treated eyes as compared with PDT monotherapy. Our results need further investigation.
A 37-year-old patient was irradiated after a subtotal excision of the hemangioblastoma of the cervical spinal cord. On the basis of history, physical examination and performed additional tests, the ...diagnosis of VHL syndrome was established. The genetic examination revealed VHL gene mutation. Genetic tests were also performed in two children of the patient and did not reveal any mutations. The last one child was not examined. In 1998 the patient underwent coagulation because of hemangioblastoma recurrence in the right retina. In 2003 patient developed a progressive disease in the kidneys. The patient died in December 2003.
The aim of the investigation was the assessment of the role of routine cytology and clonality evaluation using PCR in differential diagnosis of lymphoproliferative diseases of the orbit and eye ...adnexa. The investigations were carried out in cellular material collected via fine needle aspiration biopsy (FNAB) from 29 patients aged 31-82 years, including 17 women and 12 men. Apart from routine cytology, molecular-genetic studies were performed employing the PCR technique. In 21 cases histopathology was performed. In 2 patients, despite several attempts, FNAB failed to provide any diagnostic material for routine cytology. Based on cytology, non-Hodgkin's lymphoma was diagnosed in 11 patients and suspected in three. In 13 patients no firm diagnosis was possible based on cytological smears. The employment of PCR allowed for rendering the diagnosis more precise in 13 cases, confirming it in 13 patients, while in 3 cases the results of the above tests did not affect the final diagnosis. Clonality studies by PCR may be performed in material obtained through FNAB. Clonality assessment by PCR technique is very useful in differential diagnosis of lymphoproliferative disordes.
The goal of the investigation was to evaluate the validity of routine cytology and flow cytometry in the differential diagnosis of lymphoproliferative disorders of the orbit and eye adnexa. The ...investigations were carried out on materials originating from fine needle aspiration biopsy performed in 14 patients, including 9 females and 5 males aged 31-81 years. Apart from routine cytology, cytometric studies were also performed. Based on cytology, non-Hodgkin's lymphomas were diagnosed in six patients, while one was suspected of NHL. In seven patients the diagnosis was ambivalent, since based on cytology it was impossible to conclusively determine the biological character of the lesion, i.e. state whether it was benign or malignant. Flow cytometry was performed in 14 patients, but ultimately the results were available in 12 individuals, since in two cases the material was scant enough to exclude any assessment. Thanks to using a panel of monoclonal antibodies against light chains kappa and lambda, as well as against CD surface antigens, the authors demonstrated clonality in 90% (9/10) of NHL cases; of this number, in 7 instances the test confirmed the preliminary diagnosis and in 2 cases rendered the diagnosis more precise. On the other hand, in 3 cases no clonality was noted; of this number, in two instances the diagnosis was specified as a benign lesion (BLPL) and in one case the assessment of clonality had no impact on the final diagnosis. Out of 12 investigated aspirates, in 11 cases the result concerning clonality affected the final diagnosis. The evaluation of cellular phenotype in flow cytometry in materials obtained in the course of FNAB is a fast and sensitive method and in many cases allows for avoiding a surgical biopsy.
To assess the retinal function in patients with von Hippel-Lindau disease (VHL).
Studies were undertaken in 12 patients (17 eyes) with detected VHL gene mutation and 12 normal healthy controls (17 ...eyes).
Pattern ERG (PERG), standard flash electroretinogram (ERG) recordings were performed in accordance with the International Society for Clinical Electrophysiology of Vision (ISCEV) standards.
In VHL patients, electrophysiological statistically significant changes were found. In PERG examination, increased latency of P50 was found in the total VHL group (p < 0.02) and in the VHL subgroup with retinal angiomas (p < 0.04). In ERG examination, photopic b-wave latency was increased in the total VHL group (p < 0.03) and also in the VHL subgroup without retinal angiomas (p < 0.05). In OPs, latency increase of OP2, OP3 waves and reduced amplitude of OP3 wave in the total VHL group (OP2 latency, p < 0.05; OP3 latency, p < 0.01; OP3 amplitude, p < 0.03) and in the VHL subgroup with retinal angiomas (OP2 latency, p < 0.02; OP3 latency, p < .008; OP3 amplitude, p < 0.02) were obtained.
It can be hypothesized that dysfunction of the inner retinal layer is present in individuals with VHL disease even in patients without retinal angiomas.
Endolymphatic sac tumours (ELST) are aggressive papillary tumours of the temporal bone. The name was finally determined after the endolymphatic sac was determined as the site of their origin. They ...should be considered in patients with tumours eroding the petrous part of the temporal bone, extending to the cerebellopontine angle or other adjacent structures. These very rare tumours in the general population have much higher prevalence in von Hippel-Lindau disease. Hence molecular analysis of the VHL gene should be performed in patients with ELST and their relatives. The purpose of this study is to present a case report, histopathological characterization of endolymphatic sac tumours, their association with von Hippel-Lindau disease and use of molecular analysis.