[Boston keratoprosthesis: 73 eyes from Germany : An overview of experiences from two centers]. / Boston-Keratoprothese: 73 Augen aus Deutschland : Übersicht der Erfahrungen aus zwei Zentren.

BACKGROUND: The Boston Keratoprosthesis (B-KPro) is a therapeutic option for corneal replacement for visual rehabilitation in eyes with poor a prognosis for penetrating keratoplasty (high-risk eyes). Since 2009, this approach has been used in the MVZ Prof. Neuhann, Munich, and since 2013 also at the Department of Ophthalmology, University of Cologne. An overview of results from the first 73 eyes in these departments is presented. METHODS: All recipient eyes of both centers which received a B-KPro type I (BI-KPro) between November 2009 and March 2017 were included. Indications, preoperative findings, intraoperative procedures, and clinical results, as well as postoperative complication and retention rates were documented and analyzed. RESULTS: A total of 73 eyes from 68 patients (mean age 55 ± 21.1 years, range 7 months-93 years; 26 women, 42 men) were treated with a BI-KPro. In 53 eyes (72.6%) the BI-KPro was implanted due to corneal graft failure, whereas BI-KPro was used as the primary corneal replacement in 20 eyes (27.4%). In 46 eyes (63.0%) a long-term increase in visual acuity was achieved and in 21.9% visual acuity was stabilized, while a decrease in preoperative visual acuity occurred in 15.1% of cases. During an average follow-up of 24.7 ± 23.0 months (range 1-78 months), 1.7 ± 2.4 re-interventions (range 0-9) were required. Only 4 keratoprostheses (5.5%) could not be retained. In 3 eyes (4.2%), a change of the BI-KPro device was necessary. CONCLUSION: There is a wide range of indications for BI-KPro in eyes with a poor prognosis for conventional keratoplasty. The surgical technique is safe but with a notable postoperative complication rate, which does not seem to decrease in the medium or long term. The retention rate is excellent for eyes without stem cell deficiency. Nevertheless, the BI-KPro is the only therapeutic option for high-risk eyes and leads to a gain in visual acuity in most cases.

[Hereditary ectopia lentis]. / Hereditäre Linsenluxation.

If not due to trauma, ectopia lentis is usually caused genetically. It is a main symptom of several syndromal disorders such as Marfan syndrome or homocystinuria. Also other connective tissue disorders convey an elevated risk for ectopia lentis. Isolated ectopia lentis is frequently caused by genetic alterations as well, most commonly due to mutations in ADAMTSL4. Depending on the molecular basis, the consequences for the management of patients may differ significantly: On the one hand, possible accompanying symptoms may require a specific surveillance and treatment. Also, the risk for other family members to develop ectopia lentis or accompanying symptoms can only be determined if the genetic cause and thus inheritance pattern are known. This review describes the different types and genetic causes of syndromal and isolated ectopia lentis as well as possible consequences for the patients; also it presents a sensible algorithm for the molecular diagnostic approach.

[Case numbers and revenues of main and affiliated ophthalmological departments: Developments since 2005]. / Fallzahlen und Erlöse in ophthalmologischen Haupt- und Belegabteilungen : Entwicklungen seit 2005.

BACKGROUND: Currently, very little data exist on the development of healthcare-related and financial parameters of both types of inpatient treatment: clinical units run by affiliated physicians and those run by hospital physicians. AIM: This study used a methodology based on published secondary data to estimate the annual number of cases and revenues for in inpatient ophthalmological treatment differentiated into clinical units run by affiliated physicians and those run by hospital physicians. MATERIAL AND METHODS: The case-based flat-rate catalogs and accompanying research data published annually by the Institute for the Hospital Remuneration System (Institut für Entgeltsysteme im Krankenhaus, InEK) served as a data source. The numbers of annual cases according to major diagnostic categories (MDC) and diagnosis-related groups (DRG), stratified by the unit type are reported for the period 2005-2012. The cumulative total revenues were calculated based on the number of ophthalmological cases, the effective DRG cost weighting, the length of stay and the national basic case values. RESULTS: Between 2005 and 2012 the units run by affiliated physicians showed a contrasting trend to those run by hospital physicians: the number of cases in units run by hospital physicians increased by 14 %, while those in units run by affiliated physicians decreased by 6 %. Up to 2012 the effective cost weighting for cases in units run by hospital physicians decreased to 0.60 (- 3 %) and increased to 0.43 (+ 5 %) for units run by affiliated physicians. In 2012 the corresponding effective case revenue accounted for 1767 euros and 1271 euros, respectively. Total revenue estimates for all inpatient ophthalmological treatment increased from 549 million euros in 2005 to 630 million euros in 2012, while the share of units run by affiliated physicians amounted to 10.6 % and 9.7 %, respectively. CONCLUSION: According to the indicators "number of cases" and "total revenue", the affiliated ophthalmologists lost ground compared with inpatient units run by hospital physicians over the period from 2005-2012.

[The White man's burden - a case study caught between bipolar affective disorder and Huntington's disease]. / White Man's Burden - Im Grenzgebiet zwischen bipolarer affektiver Störung und Huntington-Erkrankung.

We report upon a case of a 55 year old patient with a bipolar affective disorder, presenting herself with a depressive symptomatology in addition to a severe motor perturbation. The main emphasis upon admittance was perfecting and improving her latest medication. Four weeks prior to her stay at our clinic a thorough neurological examination had taken place in terms of an invalidity pension trial which did not result in any diagnostic findings. Therefore a neurological disease seemed at first highly unlikely. Even though the prior testing was negative, the ensuing neurological examination at our clinic resulted in movement disorders very much indicative of Huntington's Disease. A detailed investigation in regards to the particular family history of the patient was positive for Huntington's Disease. However, whether the patient's mother had also been a genetic carrier of Huntington's Disease was still unknown at the time the patient was admitted to our clinic. It was nevertheless discovered that her mother had also suffered from a bipolar affective disorder. A genetic testing that followed the neurological examination of the patient proved positive for Huntington's Disease. Neuro-imaging resulted in a bicaudate-index of 2.4 (the critical value is 1.8). In a clinical psychological test battery the ensuing results were highly uncommon for patients with solely a bipolar affective disorder people. Under the medical regimen of Quetiapine, Citalopram and Tiaprid the patient's mood could be stabilized and there was some improvement of her motor pertubation.

[Potentials and limitations of the planned compulsory quality assurance program for cataract surgery (Qesü)]. / Potenziale und Grenzen der geplanten obligatorischen Qualitätssicherung für Kataraktoperation (Qesü).

Cataract surgery is scheduled for a federal program for quality improvement across the different sectors of care (outpatient care and hospitals). In case of implementation not only ophthalmic surgeons but all ophthalmologists would have to contribute to the documentation. Urgency, potential benefits and limitations of a compulsory compared to a voluntary quality assessment system are analyzed.

[Conspicuous crystalline lens foreign body after intravitreal injection]. / Auffälliger Linsenfremdkörper nach intravitrealer Injektion.

In January 2011 a 63-year-old woman with retinal branch vein occlusion and a macular edema first presented with a conspicuous foreign body of the crystalline lens. The rare event of an inadvertently into the lens placed Ozurdex® was diagnosed. Due to recurrent macular edema several reinjections of Ozurdex® were necessary. Phacoemulsification was performed 11 months later for advanced cataract formation. An uncomplicated fixation of the intraocular lens (IOL) into the capsular bag was enabled due to fibrosis of the capsular defect.

[Reasons for exchange and explantation of intraocular lenses]. / Ursache von Austausch und Explantation von Intraokularlinsen.

BACKGROUND: This study was performed to analyse the reasons for explantation/exchange of intraocular lenses (IOL), which had originally been implanted for the correction of aphakia during cataract extraction. MATERIAL AND METHODS: All cases with IOL explantation, which had been performed at one institution between 1/2008 and 12/2009 were analysed retrospectively. RESULTS: A total of 105 eyes of 100 patients were analysed. The median time interval between implantation and explantation of the IOL was 5.9 years (min. 0, max. 29.6). The most frequent cause for the intervention was subluxation/dislocation of the implant in 55.2% of cases. This group comprised 21% of cases with subluxation within the capsular bag in pseudoexfoliation syndrome. Other reasons were optical problems/incorrect IOL power (21%), calcification of hydrophilic acrylic IOL (7.6%), corneal decompensation associated with an anterior chamber lens (4.8%), and single cases with varying problems. CONCLUSION: The reasons for IOL exchange presented in this study are comparable to those of other series in the literature. Explantations due to optical problems may gain weight in the future due to a rise in refractive procedures and demands.

[Results with the Boston keratoprosthesis]. / Ergebnisse nach Boston-Keratoprothese.

BACKGROUND: The Boston keratoprosthesis (BKP) is a surgical therapeutic option in patients with corneal disease and poor prognosis for penetrating keratoplasty. The purpose of this study was to summarize our results with this surgical technique which we have employed at our institution since November 2009. METHODS: All patients who underwent the BKP procedure at our institution between November 2009 and August 2011 were identified retrospectively and the data were analyzed. The surgical procedure and postoperative treatment were performed following the recommendations of the developers of the BKP. RESULTS: A total of 14 patients were included in the study and the patient age ranged from 36 to 78 years. All patients had superficial and stromal corneal opacification with loss of the normal corneal surface (conjunctivalization). In 13 patients the BKP was implanted after at least 1 penetrating keratoplasty (including 3 matched grafts) and in 1 patient it was performed as a primary procedure. The underlying diseases were Stevens-Johnson syndrome, chemical injury, chronic atopic dermatitis in neurodermitis, keratoconus, granulomatous uveitis, congenital glaucoma and eyeball injury/burn. The follow-up ranged from 1 to 21 months. Postoperative complications consisted of prolonged inflammatory anterior chamber reaction with synechia, deposits on the intraocular lens, posterior capsule opacification, secondary glaucoma, hypotension, conjunctival growth over the keratoprosthesis and cystoid macular edema. All cases had overall improvement of visual acuity at the last follow-up visit. The maximum improvement was from counting fingers to 0.7. CONCLUSION: Until now all BKPs have been preserved. At our institution the BKP is becoming increasingly more important even in such cases with a (very) poor prognosis for matched limbal and/or corneal grafts. Reimbursement for the BKP must, however be organized on a case by case basis.

A further patient with van Maldergem syndrome.

We report on a male patient with the proposed diagnosis of the rare but very distinct entity of van Maldergem syndrome. His parents are first cousins. At the age of 4 years the boy presented with severe developmental delay, talipes equinovarus, finger camptodactyly with interphalangeal pterygium, joint laxity, bilateral microtia, and a dysmorphic facies. He showed bilateral epicanthus, telecanthus, short palpebral fissures, broad flat nasal bridge, and dental malocclusion. The combination of the specific facial features with camptodactyly, interphalangeal pterygium, joint laxity and developmental delay led to the diagnosis of van Maldergem syndrome. The medical history was further on significant for pharyngeal instability requiring the placement of a tracheostomy tube, an inguinal hernia, hip subluxation, small kidneys and genital abnormalities (micropenis, bifid scrotum, cryptorchidism). Due to severe feeding difficulties permanent tube feeding was required. Metabolic tests (newborn metabolic screening, 7-dehydrocholesterol, amino acids, organic acids in urine) and chromosomal analysis (450-500 bands; 46,XY) were normal. Molecular karyotyping revealed two parental CNVs (paternal deletion of 9q33.1; maternal duplication of 11p15.1), which are unlikely to contribute to the patient's phenotype. Taken together, the report on a further patient with van Maldergem syndrome expands the clinical spectrum of the condition by adding genital malformations, hernia, pharyngeal instability, and subluxation of the hip.

[Evaluation and quality assurance of refractive surgery procedures by the German Ophthalmological Society and the Professional Association of German Ophthalmologists. Status: May 2011]. / Bewertung und Qualitätssicherung refraktiv-chirurgischer Eingriffe durch die DOG und den BVA. Stand Mai 2011.

The Commission of Refractive Surgery (KRC), which is a combined commission of the German Ophthalmological Society (DOG) and the Professional Association of German Ophthalmologists (BVA), has made an up-to-date evaluation of the operative techniques for correction of refraction errors. In the revised guidelines drawn up by the KRC for evaluation and safeguarding the quality of refractive surgical procedures the description, area of application, limitations, side-effects and complications of each procedure are given according to the published scientific literature. By this means a comprehensive explanation for and investigation of patients should be safeguarded, optimal treatment results achieved and complications avoided. In this article all relevant forms of refractive surgery (photorefractive keratectomy, LASEK, EPI-LASIK, LASIK, Femto-LASIK, RELEX, conductive keratoplasty, astigmatic keratotomy, limbal relaxing incisions, intracorneal ring segments, corneal cross-linking, phakic intraocular lenses, refractive lens exchange, corneal presbyopia implants, INTRACOR) are described in detail and quality guidelines are established. Refractive surgical care and additional medical services possibly arising prior to or following this treatment are not basically covered by the statutory health insurance (GKV).

A new autosomal dominant syndrome of distinctive face showing ptosis and prominent eyes associated with cleft palate, ear anomalies, and learning disability.

We report on three patients from two families with apparently a novel clinical entity. The main features of which include unusual craniofacial dysmorphism with ptosis, prominent eyes, flat midface, Cupid's bow configuration of the upper lip, low-set, posteriorly rotated small ears, as well as conductive hearing loss, cleft palate, heart defect, and mild developmental delay. We suggest that this entity is an autosomal dominant disorder given the occurrence in a mother and daughter as well as in an unrelated boy.

Expanding the clinical and neuroradiological phenotype of 6q27 microdeletion: olfactory bulb aplasia and anosmia.

Subtelomeric deletions of chromosome 6q may result in a syndrome with brain malformations, comprising hydrocephalus and hypoplasia of the corpus callosum. Aplasia of the olfactory bulbs (OB) or anosmia has not been described in this syndrome. We describe a 3-year-old girl and a 25-year-old man with subtelomere 6q deletions. Both patients had aplastic OB and hydrocephalus. Subtelomeric 6q deletions might be underdiagnosed as anosmia can be the only symptom.

Pontine tegmental cap dysplasia: the severe end of the clinical spectrum.

Pontine tegmental cap dysplasia (PTCD) is a newly described hindbrain malformation with distinct neuroradiological findings. Only 12 cases of PTCD have been described so far, all sporadic. We report 2 further patients. Both children presented after birth with significant feeding problems due to impaired mouth opening (previously not reported) and sucking difficulties. Facial, cochlear, and glossopharyngeal nerves were involved resulting in bilateral sensory deafness and a significant swallowing disorder requiring a gastrostomy. In one patient the trigeminal sensory nerve was also involved causing severe bilateral corneal clouding with impaired vision. Both patients showed only minimal developmental progress since birth and had no speech production. Furthermore, they had vertebral and rib anomalies. The patients died at the age of 15 and 32 months, respectively, due to intercurrent infections. The majority of patients reported previously were affected less severely. The presented patients may represent the severe end of the spectrum.

[Evaluation and quality assurance of refractive surgery procedures by the German Ophthalmological Society and the Professional Association of German Ophthalmologists]. / Bewertung und Qualitätssicherung refraktiv-chirurgischer Eingriffe durch die DOG und den BVA.

The Commission of Refractive Surgery (KRC), which is a combined commission of the German Ophthalmological Society (DOG) and the Professional Association of German Ophthalmologists (BVA), has made an up-to-date evaluation of the operative techniques for the correction of refraction errors. Refractive surgery has been accepted into the catalogue of the National Association of Statutory Health Insurance Physicians for individual health services to be financed. In the guidelines drawn up by the KRC for evaluation and safeguarding the quality of refractive surgical procedures, the 1) description, 2) the area of application and limitations and 3) side-effects and complications of each procedure are given according to the published scientific literature. By this means a comprehensive explanation for and investigation of patients should be safeguarded, optimal treatment results achieved and complications avoided. Refractive surgery is an ophthalmological invasive surgical operation which necessitates specialist knowledge. When carrying out such procedures, the general guidelines of the Federal Medical Council on quality assurance of ambulant operations must be followed. Furthermore, the following prerequisites must be fulfilled by KRC physicians: Participation in a theoretical course (basic course and extended course) recognised by and carried out in cooperation with the KRC Participation in a Wet laboratory recognised by the KRC Practical experience with a trainer recognised by the KRC The first surgical operations to be carried out in the presence of a trainer recognised by the KRC Fulfil equipment and spatial requisites as well as procedural quality. All physicians who fulfil these prerequisites can apply to be named in the official list of suppliers and after fulfilling the prerequisites will be awarded a certificate on application.

[Resistance of limbo-keratoplasty to blunt trauma]. / Stabilität der Limbo-Keratoplastik bei stumpfem Bulbustrauma.

BACKGROUND: Traumatic wound dehiscence is a rare complication after penetrating keratoplasty. Limbo-keratoplasty seems to alter the pattern of rupture after blunt trauma. CASE REPORT: We report on one patient with dehiscence of a penetrating limbo-keratoplasty after blunt trauma. RESULTS: The dehiscence occurred over the entire extent of the clear corneal scar, whereas the sclero-corneal interface remained adapted. CONCLUSIONS: This case suggests a different tensile strength of these two scar types, possibly associated with their different grades of vascularization or epithelialization.