Rainer Rochels

Current concepts in the classification, diagnosis and treatment of hemangiomas and vascular malformations of the head and neck

Abstract There are many different classifications of vascular anomalies. As the correct classification of the vascular lesion has a direct influence on therapy it is difficult to decide which treatment should be considered as the treatment of choice. Based on an extensive review of the literature and personal experience of the treatment of more than 200 patients with hemangiomas or vascular malformations of the head and neck, a clinical classification is described that allows vascular lesions to be categorized in order to plan purposeful treatment. In general, hemangiomas represent the main group of vascular lesions in infancy and childhood. They are usually apparent a few weeks after birth and are characterized by an initially rapid growth of epithelial cells, followed by spontaneous involution. Hemangiomas should be differentiated from vascular malformations that are present at birth but may not be evident clinically. Spontaneous involution of vascular malformations has never been reported, whereas laser therapy can induce involution of hemangiomas at an early stage in a majority of cases. In certain situations steroids or surgical removal may seem to be the appropriate therapy of choice. In contrast, vascular malformations have to be treated according to their histopathology and location, as well as their hemodynamic features as shown radiographically with angiography. The accurate diagnosis of vascular anomalies is essential for further treatment, as shown by clinical experience at the University of Marburg.

Functional anatomy of human lacrimal duct epithelium.

Abstract Resorption of tear fluid in the lacrimal ducts has hitherto been controversial; one reason for this has been insufficient knowledge of the anatomical structure and function of the lacrimal duct epithelium. The present study analyzes the structure of lacrimal duct epithelium by means of histological, histochemical, immunohistochemical and electronmicroscopical methods and draws a conclusion about its physiological function regarding its role in immunodeficiency. Investigations were performed on 31 lacrimal systems of 17 male and 14 female individuals (aged 54–88 years). Lacrimal ducts are surrounded by a wide-ranging cavernous system, which is embedded in an osseous canal between the maxilla and the lacrimal bone. The internal wall of the lacrimal canaliculi is lined by a stratified epithelium. The lacrimal sac and nasolacrimal duct contain a double-layered epithelium, which rests on a broad basement membrane. In their apical part epithelial cells contain large lipid droplets and secretory vacuoles. Epithelial cells are faced by microvilli and some tufts of kinociliae are also visible. Goblet cells are integrated in the epithelium as solitary cells or in a characteristical arrangement of several cells. The secretory product of these cells contains carbohydrates including fucose and sialic acid. Inside the surrounding cavernous system serous glands are found that open their excretory ducts into the lacrimal sac and nasolacrimal duct. Some T- and B-lymphocytes and macrophages may be demonstrated immunohistochemically in the submucosa partly penetrating the epithelium. Synthesized mucins of goblet cells form a specialized protective layer on the epithelium of the lacrimal ducts, which functionally serves for a simplified drainage of tear fluid into the inferior meatus of the nose. Together with immunocompetent cells, the protective layer plays a role in antigen defense and prevents invasion of pathogenic agents. The facing of epithelial cells by microvilli gives hints of reabsorption of lacrimal fluid inside the lacrimal ducts.

Functional anatomy of the human efferent tear ducts: a new theory of tear outflow mechanism

Abstract · Background: The mechanism of lacrimal drainage under physiological conditions is controversial. The aim of this study was to analyze the three-dimensional architecture of human efferent tear ducts from functional and clinical points of view. A new theory of tear outflow is discussed. · Methods: Thirty-two prepared lacrimal systems of adults were examined by histological, immunohistochemical and scanning electron microscopic techniques. · Results: The wall of the lacrimal sac is made up of collagen bundles, elastic and reticular fibers arranged in a helical pattern. Wide luminal vascular plexus are embedded in this helical system and connected to the cavernous tissue of the inferior turbinate in the region of Hasner’s valve. Immunohistochemical analysis showed evidence of type I and type III collagen as well as chondroitin 4- and 6-sulfate. · Conclusion: With blinking, the lacrimal part of the orbicularis muscle contracts. The fornix of the sac moves in a cranial-lateral direction. Thus the lacrimal sac distends and may be ’’wrung out’’ due to its medial attachment and helically arranged fibrillar structures. The vascular plexus may play an important role in the absorption and drainage of lacrimal fluid.

Primary antiphospholipid antibody syndrome and retinal occlusive vasculopathy.

PURPOSE To report a 31-year-old healthy patient with retinal venous occlusion in his left eye attributable to primary antiphospholipid antibody syndrome. METHODS The patient was examined clinically. Multiple serologic and clinical investigations were performed to determine the causative disease. He was closely followed up for more than 3 years. RESULTS The presence of lupus anticoagulant in our patient was indicated by a kaolin clotting time index of 27 (normal, <17) and confirmed by the demonstration of IgG antibodies against phospholipids. After long-term oral anticoagulant treatment for 2 years, lupus anticoagulant levels returned to normal, and therapy was stopped. No further thrombotic event occurred during follow-up. CONCLUSIONS In retinal vascular occlusions of unexplained origin, antiphospholipid antibodies may play an important role in the pathogenesis. Detecting these antibodies in the serum of patients with retinal vascular occlusion helps determine the appropriate treatment with long-term oral anticoagulants.

Bilateral Cataract after Phakic Posterior Chamber Top Hat-style Silicone Intraocular Lens

BACKGROUND New strategies have been developed for surgical treatment of high myopia. Recently, implantation of minus power posterior chamber intraocular lenses (IOL) into phakic eyes has been introduced. METHODS We report a 37 year-old female patient who developed bilateral anterior subcapsular cataract 7 years after minus-power, top hat-style silicone posterior chamber IOL implantation in Russia. The corrected visual acuity was right eye 20/200 and left eye 20/63. Because of the high myopia and the posterior chamber IOL in her phakic eyes, biometry for IOL calculation gave contradictory results. Both posterior chamber IOLs were found to be adherent to the crystalline lenses. After posterior chamber IOL explantation and phacoemulsification, intraoperative retinoscopy was performed. With this aphakic refraction, the IOL power was calculated and implanted. The explanted posterior chamber IOLs were examined by scanning electron microscopy. RESULTS After bilateral operation the corrected visual acuity increased to right eye 20/32 and left eye 20/40, respectively. On scanning electron microscopy, a membranous structure of unknown origin was noted on the entire surface of the explanted posterior chamber IOLS. CONCLUSION There is a potential risk of cataract formation after implantation of this top hat-style silicone posterior chamber IOL. If cataract extraction in this specific situation is needed, a different approach for calculating the aphakic IOL power is necessary, such as intraoperative retinoscopy.

Scanning Electron-Microscopic Studies of the Collagen Architecture of the Human Sclera – Normal and Pathological Findings

The arrangement of the collagen fibrils of the human sclera was analyzed in the region of the limbus cornea, the corneoscleral trabeculum, at the zone of muscle insertion and at defined areas of the internal and external surface of the sclera. Adult eyes with no apparent pathological alterations and the sclera of a patient with staphyloma were examined by scanning electron microscopy. The investigations were performed to describe regularities in the collagen architecture in normal and pathologically altered eyes to understand pathomorphologic and pathophysiologic changes in scleral diseases.

Primary Orbital Leiomyoma and Leiomyosarcoma

A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.

SEM studies of the collagen architecture of the human lamina cribrosa: normal and pathological findings.

The arrangement of the collagen fibrils of the lamina cribrosa was analyzed by using scanning electron microscopy with clinical regard to morphological alterations in cases of glaucoma at different stages. Adult eyes with no apparent pathological changes and specimens from patients with absolute glaucoma were studied. In the lamina cribrosa of nonpathological eyes the collagen fibrils are arranged circularly around the points of passage of axons and vessels. In specimens from glaucoma patients the characteristic circular alignment of the collagen fibrils around the penetrating axons is generally absent. The collagen fibrils are arranged in coarse bundles, showing no preferred alignment. The present study supports a new hypothesis for glaucoma pathogenesis.

Delayed suprachoroidal hermorrhage after penetrating keratoplasty

We report on an aphakic 30 year-old patient who underwent penetrating keratoplasty on his only functioning eye. On the first day after the operation he developed a total choroidal detachment due to suprachoroidal bleeding. The patient had no sense of orientation. Vision decreased to faulty light perception. One week postoperatively a posterior sclerotomy was performed in order to drain the suprachoroidal blood. Via a paracentesis the globe was pressurized with hyaluronic acid. The following day the suprachoroidal blood had almost disappeared. The retina had become reattached. During a follow-up period of 2 years the visual acuity remained 20/30.

Die ableitenden Tränenwege des Menschen Morphologische und biochemische Untersuchungen

ZusammenfassungHintergrund. Entzündungen im Bereich der ableitenden Tränenwege sind häufig. Um ihre Pathomechanismen zu verstehen, ist eine genaue Kenntnis ihrer anatomischen Struktur und ihrer zellulären Abwehrmechanismen nötig. Ziel der vorliegenden Arbeit ist die Untersuchung der ableitenden Tränenwege hinsichtlich ihrer physiologischen Funktion und ihrer möglichen Abwehrmechanismen gegen Infektionen. Material und Methoden. Es wurden 31 Tränenwegssysteme von Organspendern im Alter von 54–88 Jahren histologisch, immunhistochemisch und elektronenmikroskopisch untersucht. Ergebnisse. Die ableitenden Tränenwege werden von einem zweireihigen Epithel ausgekleidet, das einer breiten Basalmembran aufliegt. Im apikalen Bereich der Zellen kommen zahlreiche Lipidtröpfchen und Sekretvakuolen vor. Intraepithelial sind Zellen teilweise zu mukösen Drüsen zusammengelagert, die morphologisch den Becherzellen des Tarsus palpebrae gleichen. Das Sekretionsprodukt dieser Zellen enthält unterschiedliche Kohlenhydrate. Die Submukosa ist reich an Lymphozyten und Makrophagen, die z. T. bis in das Epithel vordringen. Schlussfolgerungen. Die von den Tränenwegsepithelien und Becherzellen synthetisierten Lipide und Muzine bilden auf der Oberfläche der ableitenden Tränenwegsepithelien eine Schutzschicht, die dem verbesserten Abfluss der Tränenflüssigkeit in den unteren Nasengang dient und gemeinsam mit den spezifischen Abwehrzellen das Eindringen von Krankheitserregern verhindert.AbstractPurpose. Infections occur frequently in the region of the efferent tear ducts. Exact knowledge of the anatomical structure and of cellular defense mechanisms is necessary to understand the pathological processes. This sudy analyzed the efferent tear ducts with regard to physiological function and possible defense mechanism against infections. Materials and methods. We used histological, immunohistochemical and electron-microscopic techniques to investigate the lacrimal systems from 31 body doners aged 54–88 years. Results. The efferent tear ducts are lined by a double-layered epithelium resting on a broad basement membrane. These cells contain many lipid droplets and secretory vacuoles in their apical part. Inside the epithelium cells are arranged partly in cell groups forming mucous glands, which morphologically resemble goblet cells of the tarsus palpebrae. The secretory products of these cells contain various carbohydrates including sialic acid. Lymphocytes and macrophages are found in the submucosa partly penetrating the epithelium. Conclusions. Lipids and mucins of epithelial and goblet cells form a specialized protective layer on the epithelium of the tear ducts which enables easy drainage of tear fluid into the inferior meatus of the nose. Together with immunocompetent cells the protective layer prevents invasion of pathogenetic agents.