[Immunohistochemical and Molecular Genetic Profile of Mantle Cell Lymphoma of the Lacrimal Gland: A Case Series of an Uncommon Tumour of the Lacrimal Gland]. / Immunhistochemisches und molekulargenetisches Profil von Mantelzelllymphomen der Glandula lacrimalis: eine detaillierte Beschreibung einer seltenen Tumorentität der Tränendrüse.

BACKGROUND: Mantle cell lymphomas (MCL) represent a rare subclass of Non-Hodgkin Lymphoma affecting the lacrimal gland (GL). AIM: To extensively describe the immunohistochemical profile of GL-MCL. MATERIAL UND METHODS: Single center, retrospective electronic records review of 3 patients with biopsy proven LG-MCL. RESULTS: The herein presented case series of three patients comprises a focal case involving solely the lacrimal gland, a symptomatic LG-MCL manifesting as the first sign of a systemic disease as well as a case of LG-MCL presenting as a relapsed systemic lymphoma. The three patients presented positive CD19 and CD20, negative CD10 and CD23. One patient showed an uncommon negativity for CD5. The increased expression of cyclin D1 caused by the classical translocation t(11;14) (q13;q32) in the fluorescence-in-situ-hybridisation were observed in all cases. B-cell-lymphoma-2 protein (BCL-2) and transcription factor SOX-11 (SOX-11) were also overexpressed. DISCUSSION: LG-MCL show an immunohistochemical profile corresponding to the classical profile of MCL. Overexpression of molecules for target therapies was found in all cases (CD20 for rituximab, BCL2 for Bruton-kinase-inhibitors and CD19 for CAR-T cell therapy). The removal of the GL can potentially drive to severe complications, even if aimed to confirm diagnosis. Therefore, the choice between GL-biopsy and exstirpation should be carefully evaluated, especially in cases of suspected lymphoma.

Infranuclear Eye Movement Disorders. / Infranukleäre Augenbewegungsstörungen.

Infranuclear motility disorders are such of the cranial nerves, the extraocular muscles or changes in the orbit but definitely peripheral to the nuclei of the cranial nerves. Characteristic are movement deficits, a compensatory head posture and the pattern of incomitancy. The secondary angle of deviation is usually larger than the primary. Combined pareses suggest a lesion in the cavernous sinus, orbital apex or a multilocular event. It is essential to rule out supranuclear disorders, especially if the motility deficit is atypical. For clarification, an individual risk assessment is recommended, paying particular attention to risk factors.

[Congenital nasolacrimal duct obstruction : A real-life study from the first symptoms to the results of surgical treatment]. / Konnatale Tränenwegsstenose : Eine Real-Life-Datenstudie von den ersten Symptomen bis zum Ergebnis der operativen Sanierung.

OBJECTIVE: The optimal timing for surgery of congenital nasolacrimal duct obstruction (CNLDO) is controversially discussed . An aspect that has not yet been studied in this context is the path from first symptoms to the initial diagnosis and surgical treatment with respect to social factors and burden of suffering. The aim of this study was a real-life analysis of the entire course of the disease. METHODS: Monocentric, retrospective study evaluating children with CNLDO (n = 147) who underwent surgery. The minimum follow-up was 3 months (26 ± 14 months). The patient information was collected by telephone using a questionnaire especially created for this purpose. The famelial suffering was measured on a scale of 1-5. RESULTS: Data were collected from 118 parents with a mean follow-up of 26 months. First symptoms occurred at a median age of 1 month. The diagnosis was confirmed through a pediatrician/ophthalmologist after a median of 6 months. An assignment to a referral center for surgical treatment was performed a median of 18 months after the first presentation. The average age of the patients at the time of surgery was 23 months. Surgery before the age of 13 months showed a 100% success rate. The average age of those who needed revision surgery was 27 months (±12 months). The familial suffering was measured as 4.1 points before surgery and 1.3 after surgery. CONCLUSION: This study is the first real-life study, which illustrates the path of CNLDO from the first symptoms to rehabilitation, including influential social factors. Early surgery significantly shortens the suffering of both patients and parents. Considering the low operative risks and excellent success rate of operative treatment, an early consultation at a surgical department is recommended.

A comparison of the lateral tarsal strip with everting sutures and the Quickert procedure for involutional entropion.

BACKGROUND/AIMS: To provide evidence of statistically significant difference in the surgical outcome of the lateral tarsal strip with everting sutures (LTS + ES) versus the Quickert procedure (QP) in the treatment of involutional entropion. METHODS: In a prospective randomized comparative trial, 66 eyelids of 52 patients with primary involutional lower eyelid entropion were recruited. Thirty-six eyelids were randomized to QP, and 30 eyelids were randomized to LTS + ES. Surgery was performed by a single surgeon. Postoperative follow-up was scheduled after 2 weeks, 8 and 14 months. Successful surgery was defined as a normal eyelid position at rest and inability to induce entropion on forced eyelid closure at or before the 14-month follow-up visit. RESULTS: A total of 66 eyelids of 52 patients were enrolled in the study. Three patients did not complete follow-up (1 did not attend the 8 months follow-up visit; 2 did not attend 14 months follow-up visit). Of the 63 patients, a single eyelid [success probability 0.97; confidence interval (CI) 0.92-1] in the QP group and two treated eyelids [success probability of 0.93; CI: 0.85-1] in the LTS + ES group had a recurrence of a lower eyelid entropion after 14 months. There was no statistically significant difference in surgical failure between the LTS + ES versus QP (Log-rank test: p = 0.46). CONCLUSION: These data provide strong evidence that success rates at 14 months are similar in patients treated with either techniques (LTS + ES versus QP).

Manifestations and Treatment of Adult-onset Symptomatic Optic Pathway Glioma in Neurofibromatosis Type 1.

This report describes the diagnosis and treatment of a 27-year-old patient with neurofibromatosis 1 (NF1) and late progression of a pre-existing optic pathway glioma (OPG) that caused significant reduction in vision. OPG is one of the diagnostic criteria for establishing the diagnosis of NF1. Most common findings of NF1 are café-au-lait spots, axillary and inguinal freckling of the skin, iris hamartoma (Lisch nodules), and tumors of the central nervous system and peripheral nerves. We successfully applied a modified International Society of Paediatric Oncology chemotherapy regimen for low-grade glioma in children with carboplatin dose adjustment according to the area under the plasma drug concentration-time curve calculation. During and after the chemotherapy, a clear improvement of the visual capacity was achieved. Age-adapted chemotherapy for symptomatic adult-onset OPG in patients with NF1 should be considered in individual cases.

An experimental approach towards novel dyes for intraocular surgery.

Chromovitrectomy represents a recent development in the field of vitreoretinal surgery. Several dyes are available for intraocular application with different staining characteristics. Before the intraoperative use in humans, new dyes need to be evaluated thoroughly in experimental in vivo and ex vivo studies in order to detect potential adverse effects related to dye toxicity. This article describes a reasonable approach for the assessment of novel dyes prior to the use in humans.

Genetic animal models for retinal degeneration.

Inherited retinal degenerations are a common cause of blindness in Western countries. A mechanism for most retinal degenerations is still unknown; hence, a suitable treatment for most of these diseases has yet to be found. Before one can rationally design a treatment, it is necessary to understand the pathway from a gene mutation to the phenotype in patients. Animal models are crucial to understand this process and to develop a treatment. Some naturally occurring animal models are known. However, over the past few years, transgenic engineering has allowed the generation of a rapidly growing number of animal models. In this review, we give an overview of the broad variety of genetic animal models for retinal degeneration.