Michael P. Schittkowski

Expansion of the socket and orbit for congenital clinical anophthalmia.

Background: Congenital clinical anophthalmia requires fitting an artificial eye, achieving a pleasing aspect of the palpebral apparatus, and stimulating growth of the orbit, as well as of the entire midface. Methods: Out of 25 patients with clinical anophthalmia, 22 were treated according to a new protocol (first described by Wiese et al. in 1999) and followed for up to 6 years. In 10 patients, anophthalmia was bilateral, in nine patients, it was on the right side only and in three it was on the left side only. Treatment consisted of implantation of an osmotic expander to first stimulate growth of the conjunctival sac and eyelids followed by serial implantation of osmotic expanders to stimulate growth of the orbit (and thus of the midface). The material used was a copolymer of methylmethacrylate and N-vinylpyrrolidone. Results: Fitting of an artificial eye was without any problem within the first year of life for the most part. Growth of the orbit was stimulated by more than 65 percent of a healthy one. Aesthetically, the results were very largely pleasing. Complications were encountered, especially when the wrong expander sizes had been implanted. Conclusion: Using osmotic expanders of this type, through a staged therapy of minor operations, promises to achieve all goals before the child enters school.

Therapie des kongenitalen klinischen Anophthalmus mit hoch hydrophilen Hydrogelexpandern

ZusammenfassungEinleitungBei Kindern mit angeborenem klinischen Anophthalmus (echter Anophthalmus oder funktionsloser extremer Mikrophthalmus) fehlt der Stimulus für die Entwicklung und das Wachstum von Lidapparat, Bindehautsack und Augenhöhle. Daraus folgend ergeben sich Schwierigkeiten der adäquaten prothetischen Versorgung und damit erhebliche kosmetische Beeinträchtigungen. Seit wenigen Jahren steht nach der Vorarbeit von Wiese mit dem Einsatz hoch hydrophiler Osmoseexpander eine neue Behandlungsmethode für diese Patientengruppe zur Verfügung.Patienten und MethodeEs wird über die Anwendung selbstquellender Osmoseexpander bei der Dehnung des Bindehautsackes anophthalmischer Kinder berichtet. Bisher befinden sich 13 Patienten (8 mit einseitigem, 5 mit beidseitigem Anophthalmus) in unserer Behandlung.ErgebnisseDurch die Expanderanwendung konnte in allen Fällen eine Aufdehnung des Bindehautsackes erreicht werden. Allein durch diese Maßnahme gelang es, eine prothesenfähige Höhle zu schaffen. Bei allen Kindern konnte bisher ein gutes bis zufriedenstellendes kosmetisches Resultat erzielt werden, ohne auf weitere lidchirurgische Maßnahmen zurückgreifen zu müssen.DiskussionDer Einsatz der selbstquellenden Hydrogelexpander hat sich an unserer Klinik in den letzten Jahren bewährt. Resultierend aus der bisherigen klinischen Erfahrung konnte ein neues therapeutisches Konzept zur Behandlung des klinischen Anophthalmus aufgestellt werden. Der Vorteil des Verfahrens besteht im Verzicht auf ausgedehnte lidchirurgische Operationen, welche im Kindesalter oft zu ausgedehnter narbiger Schrumpfung führen.AbstractIntroductionChildren presenting with congenital anophthalmos usually develop a smaller bony orbit, a constricted mucosal socket, and a shortened eyelid fissure. This causes problems when fitting these patients with a prosthesis. Clinical evaluation of the Wiese self-inflating hydrogel expanders has demonstrated their ability to expand the socket and eyelid fissure for inserting a more realistic prosthesis in shorter periods of time.Patients and methodThe study included 13 consecutive anophthalmic patients, eight unilateral and five bilateral. Each patient received a hemispherical osmotic tissue expander in the rudimentary mucosal socket and later a sphere implanted in the deeper soft orbital tissue.ResultsThe use of hydrogel expanders enlarged the lid and palpebral fissure in all children, with good cosmetic results. It allowed insertion of custom-made glass prostheses with good cosmetic appearance very early in life. Growth of the bony orbit may be stimulated successfully by these expanders in the soft orbital tissue.ConclusionsThe enlargement of constricted mucosal sockets and short palpebral fissures using self-inflating hydrogel expanders is a new and successful concept in treating congenital anophthalmos.

Alternating Current Stimulation for Vision Restoration after Optic Nerve Damage: A Randomized Clinical Trial

Background Vision loss after optic neuropathy is considered irreversible. Here, repetitive transorbital alternating current stimulation (rtACS) was applied in partially blind patients with the goal of activating their residual vision. Methods We conducted a multicenter, prospective, randomized, double-blind, sham-controlled trial in an ambulatory setting with daily application of rtACS (n = 45) or sham-stimulation (n = 37) for 50 min for a duration of 10 week days. A volunteer sample of patients with optic nerve damage (mean age 59.1 yrs) was recruited. The primary outcome measure for efficacy was super-threshold visual fields with 48 hrs after the last treatment day and at 2-months follow-up. Secondary outcome measures were near-threshold visual fields, reaction time, visual acuity, and resting-state EEGs to assess changes in brain physiology. Results The rtACS-treated group had a mean improvement in visual field of 24.0% which was significantly greater than after sham-stimulation (2.5%). This improvement persisted for at least 2 months in terms of both within- and between-group comparisons. Secondary analyses revealed improvements of near-threshold visual fields in the central 5° and increased thresholds in static perimetry after rtACS and improved reaction times, but visual acuity did not change compared to shams. Visual field improvement induced by rtACS was associated with EEG power-spectra and coherence alterations in visual cortical networks which are interpreted as signs of neuromodulation. Current flow simulation indicates current in the frontal cortex, eye, and optic nerve and in the subcortical but not in the cortical regions. Conclusion rtACS treatment is a safe and effective means to partially restore vision after optic nerve damage probably by modulating brain plasticity. This class 1 evidence suggests that visual fields can be improved in a clinically meaningful way. Trial Registration ClinicalTrials.gov NCT01280877

Results of lacrimal assessment in patients with congenital clinical anophthalmos or blind microphthalmos

Aim: To report clinical findings relating to the lacrimal system in congenital clinical anophthalmos and severe blind microphthalmos. Methods: A retrospective (up to 2003) and prospective (2004 onwards) study of the notes of 60 consecutive patients treated surgically with highly hydrophilic self-inflating expanders for congenital anophthalmos or severe blind microphthalmos between 1997 and 2006. The lacrimal drainage system was always probed and irrigated under general anaesthesia before any other procedure was started. Results: Nine patients were excluded due to possible misdiagnosis because of previous lid or orbit surgery elsewhere or due to missing data. The analysis therefore included 23 girls and 28 boys aged between 1 and 90 months (median age: 4 months). Twenty-three patients presented with unilateral and 18 with bilateral anophthalmos, and 10 had unilateral microphthalmos; consequently, 102 orbits (of which, 69 were with probable pathology) were available for assessment. In unilateral cases, the lacrimal system on the normal side was never affected. On the anophthalmic or microphthalmic side, the lacrimal system was normal in 17 orbits only (24.6%). The most frequent finding was canalicular stenosis (40 orbits; 58%). Common canaliculus stenosis was observed in 5 orbits (7.3%) and nasolacrimal duct obstruction in 7 orbits (10.1%). There were no cases of punctal anomaly. Conclusions: In congenital clinical anophthalmos the lacrimal system is affected in up to 78% of cases, mostly due to canalicular stenosis. Even if there is no clear evidence of an embryological connection, this association is certainly not a random finding.

Self-Inflating Hydrogel Expanders for the Treatment of Congenital Anophthalmos

Clinical congenital anophthalmos is the absence of any visible globe Tissue expansion is necessary to insert a regular prosthesis in order to achieve a more normal appearance Congenital anophthalmos is very rare; estimated prevalence is 1–4/100,000 births The relationship with a variety of syndromes and anomalies makes it important to routinely undergo genetic evaluation Due to the variety of symptoms an individual treatment plan is needed for each patient The current preferred option is the use of non-expanding conformers In cases of severe anophthalmos with extreme underdeveloped cul-de-sacs, success with conformers is often limited Silicone balloon expanders, mucous membrane grafts, dermis fat grafts or even craniofacial surgery are not methods of first choice. They are reserved for those patients who have failed to achieve adequate expansion or facial symmetry despite aggressive conformer or expander therapy Hydrogel expanders can be recommended for congenital anophthalmic patients as a method with good results and minimal surgical trauma Hydrogel expanders may permit an earlier ability to wear a prosthesis than standard serial conformer expansion

Diseases of the lacrimal gland

ZusammenfassungAufgrund des seltenen Vorkommens von Tränendrüsenerkrankungen im Praxisalltag ist der Erfahrungswert in der differenzialdiagnostischen Einordnung vergleichsweise gering. Es ist deshalb von besonderer Bedeutung, Richtlinien zu erarbeiten, anhand derer der Kliniker eine Entscheidung treffen kann, ob eine konservative oder operative Therapie erforderlich ist und ob zuvor eine Biopsie zur Diagnosesicherung durchgeführt werden muss. Dabei ist zu berücksichtigen, dass das benigne pleomorphe Adenom durch eine primär komplette Tumorentfernung kurativ behandelt werden kann, während eine Tumorteilresektion, Biopsie oder versehentliche intraoperative Kapseleröffnung ein hohes Rezidivrisiko sowie die Gefahr einer malignen Transformation in sich birgt und somit einen negativen Effekt auf die Langzeitprognose hat. Die präoperativen differenzialdiagnostischen Überlegungen müssen deshalb im Besonderen das mögliche Vorliegen eines pleomorphen Adenoms berücksichtigen. Im Folgenden soll deshalb vor allem auf die differenzialdiagnostischen Aspekte der Tränendrüsenerkrankungen hinsichtlich der klinischen Befunde und der bildgebenden Charakteristika eingegangen werden.AbstractExperience with the differential diagnosis of lacrimal gland illnesses is limited in daily practice due to their rarity. It is therefore of particular importance to have guidelines for the practitioner by which he can decide on the application of conservative or surgical therapy, and whether it is necessary to take a biopsy to clarify the diagnosis. There is a wide spectrum of possible illnesses which include systemic diseases, inflammatory disorders, neoplastic, benign and malignant tumors. When a pleomorphic adenoma cannot be excluded, biopsy is contraindicated and complete tumor excision with its capsule is necessary to prevent the possibility of malignant transformation and a negative effect on long-term prognosis. In this overview, special interest is placed on the typical clinical aspects and imaging features of lesions of the lacrimal gland fossa, their differential diagnosis and management.

3 Jahre Erfahrung mit der niedrig dosierten fraktionierten perkutanen Teletherapie bei subfoveolären Neovaskularisationen Klinische Ergebnisse

AIM The effect of low dose fractionated percutaneous teletherapy to visual acuity and the changes in subfoveolar neovascular membranes in age-related macular degeneration were investigated. PATIENTS AND METHOD 126 eyes of 118 patients (age 55-89 years; mean 74 ys.) were treated. Best distal and near visual acuity was assessed prior to (= initial visual acuity [IVA]) and 3, 6, 12, 18, 24, 30 and 36 months after teletherapy. Fluorescein angiography was performed prior to and 6, 12, 24 and 36 months after radiation therapy. For analysis patients were divided into different groups by IVA and membrane size. Maximal duration of observation was 36 months. Teletherapy was done by a 9-MeV photon linear accelerator through a lateral port in half-beam technique with a single dose of 2 Gy up to a total dose of 20 Gy within 12 days. RESULTS No severe negative side effects have been observed. Eight patients reported of epiphora and four patients complained of transient sicca syndrome. Visual acuity decreased more than one line in the group IVA 0.05-0.2. The group IVA 0.3-0.5 remained unchanged for 1 year. We found a tendency for increased visual acuity in group IVA > or = 0.6 for 18 months. After that time both groups showed decreased visual acuity, but all these patients reported of reduced metamorphopsia and increased color and contrast perception. CONCLUSIONS There is an influence of low dose fractionated percutaneous teletherapy on visual acuity, subfoveal neovascular membranes and metamorphopsia. IVA and duration of anamnesis play an important role. There seems to be no persistent effect; possibly increased dosage will bring a benefit.Ziel: Es wurde die Wirkung der Bestrahlung auf die Sehschärfe bei altersabhängiger Makuladegeneration mit chorioidalen Neovaskularisationen, die nach den MPS-Kriterien einer Laserkoagulation nicht zugänglich sind, untersucht. Patienten und Methode: 126 Augen von 118 Patienten im Alter von 55–89 Jahren (Mittel 74) wurden behandelt. Ein ophthalmologischer Status wurde bei der Voruntersuchung sowie bei den Kontrollen 3, 6, 12, 18, 24, 30, 36 Monate nach der Therapie erhoben. Zur Auswertung, in die wegen okulärer Nebenerkrankungen nur 78 Augen von 70 Patienten einbezogen werden konnten, wurden die Patientendaten nach dem Ausgangsvisus und der Art der chorioidalen Neovaskularisationen (klassischokkult) in Gruppen unterteilt. Die maximale Nachbeobachtungsdauer beträgt 36 Monate. – Die 9-MeV-Photonen-Teletherapie erfolgte über ein seitliches Stehfeld mit einer Einzeldosis von 2 Gy bis zu einer Gesamtdosis von 20 Gy (90%-Isodose). Die zehn Einzeldosen wurden an jeweils 5 aufeinander folgenden Wochentagen mit Wochenendpause appliziert. Ergebnisse: Schwerwiegende Nebenwirkungen konnten wir nicht beobachten. Die Therapie wurde bis auf subjektiv beobachtete Epiphora (acht Patienten) und passagere Siccasymptomatik (vier Patienten) gut vertragen und angenommen. Die Gruppe Ausgangsvisus 0,05–0,2 zeigte einen durchschnittlichen Visusabfall von mehr als einer Zelle. Bei einem Ausgangsvisus 0,3–0,5 fanden wir Visusstabilität bis zu 12 Monaten, danach langsame Visusreduktion. Die Gruppe Ausgangsvisus ≥ 0,6 wies einen tendenziellen Visusanstieg für ca. 12 Monate auf, beginnend nach 18 Monaten zeigte sich ein allmählicher Visusabfall. Die Patienten der beiden letztgenannten Gruppen zeigten eine Abnahme der Metamorphopsien sowie gesteigerte Farb- und Kontrastwahrnehmung. Schlussfolgerung: Die vorliegende Untersuchung zeigt, dass die niedrig dosierte fraktionierte perkutane Teletherapie die subfoveoläre Membran, die Sehschärfe und das Auftreten von Metamorphopsien beeinflusst. Dabei spielen Ausgangsvisus und Anamnesedauer eine entscheidende Rolle. Ein anhaltender Effekt scheint nicht zu bestehen; möglicherweise kann ein Applikationsmodus mit erhöhter Dosis hilfreich sein.Aim: The effect of low dose fractionated percutaneous teletherapy to visual acuity and the changes in subfoveolar neovascular membranes in age-related macular degeneration were investigated. Patients and Method: 126 eyes of 118 patients (age 55–89 years; mean 74 ys.) were treated. Best distal and near visual acuity was assessed prior to (= initial visual acuity [IVA]) and 3, 6, 12, 18, 24, 30 and 36 months after teletherapy. Fluorescein angiography was performed prior to and 6, 12, 24 and 36 months after radiation therapy. For analysis patients were divided into different groups by IVA and membrane size. Maximal duration of observation was 36 months. Teletherapy was done by a 9-MeV photon linear accelerator through a lateral port in half-beam technique with a single dose of 2 Gy up to a total dose of 20 Gy within 12 days. Results: No severe negative side effects have been observed. Eight patients reported of epiphora and four patients complained of transient sicca syndrome. Visual acuity decreased more than one line in the group IVA 0.05–0.2. The group IVA 0.3–0.5 remained unchanged for 1 year. We found a tendency for increased visual acuity in group IVA ≥ 0.6 for 18 months. After that time both groups showed decreased visual acuity, but all these patients reported of reduced metamorphopsia and increased color and contrast perception. Conclusions: There is an influence of low dose fractionated percutaneous teletherapy on visual acuity, subfoveal neovascular membranes and metamorphopsia. IVA and duration of anamnesis play an important role. There seems to be no persistent effect; possibly increased dosage will bring a benefit.

[Ocular manifestations of Churg-Strauss syndrome: review article and case report].

This paper summarizes the possible ocular manifestations of Churg-Straus syndrome (CCS) from the literature and presents an unusual case report from routine clinical practice with conjunctival granuloma, orbital pseudotumor and choroidal folds. The CSS is an ANCA-associated granulomatous vasculitis which can be manifested in various organs and represents a life-threatening situation for the patient. Ocular manifestations are rare and can spread to all segments of the eye and orbit. The most frequent forms of ocular involvement described in the literature are retinal occlusion and orbital pseudotumor with various degrees of expression.ZusammenfassungDiese Arbeit fasst mögliche okuläre Manifestationen bei Churg-Strauss-Syndrom (CCS) in der Literatur zusammen und präsentiert eine ungewöhnliche Fallvorstellung aus dem klinischen Alltag mit konjunktivalen Granulomen, Pseudotumor orbitae und Aderhautfalten. CSS ist eine ANCA-assoziierte granulomatöse Vaskulitis, die sich in verschiedenen Organen manifestieren kann und eine lebensbedrohliche Situation für die Patienten darstellt. Okuläre Manifestationen sind selten und können sich auf alle Segmente des Auges und der Orbita ausbreiten. Die häufigsten beschriebenen Augenbeteiligungen in der Literatur sind retinale Verschlüsse und der Pseudotumor orbitae verschiedener Ausprägung.AbstractThis paper summarizes the possible ocular manifestations of Churg-Straus syndrome (CCS) from the literature and presents an unusual case report from routine clinical practice with conjunctival granuloma, orbital pseudotumor and choroidal folds. The CSS is an ANCA-associated granulomatous vasculitis which can be manifested in various organs and represents a life-threatening situation for the patient. Ocular manifestations are rare and can spread to all segments of the eye and orbit. The most frequent forms of ocular involvement described in the literature are retinal occlusion and orbital pseudotumor with various degrees of expression.

Entwicklung der okuloplastischen Chirurgie in Kinshasa (Hauptstadt der Demokratischen Republik Kongo)

The partnership between Saint Joseph Hospital in Kinshasa (Capital city of DRC) and University Eye Clinic Rostock, Germany exists since 2000. The ophthalmologists from Rostock University performed 12 visits of St. Josef Hospital with the aim to perform oculoplastic surgery in complicated cases as well as to teach the local ophthalmologists. There they performed about 150 surgeries (entropion, ectropion, ptosis, repair of lid injuries, eye lid reconstruction and tumour management, lacrimal surgery) in joint teams. The programme of on-site training was established during this time. Meanwhile, the Kinshasa colleagues are able to perform the basic techniques by themselves. Two colleagues have obtained advanced training in oculoplastics in Rostock. The fruitful cooperation enabled the development of oculoplastic surgery in St. Josef Hospital in Kinshasa. The further education and training proccesses will support the continuous advancement in ophthalmological health care in DRC.

Erdheim-Chester-Erkrankung der Orbita mit Kompressionsneuropathie

ZusammenfassungEin 60-jähriger Mann stellte sich mit einem zunehmenden Exophthalmus und einer allmählichen Sehverschlechterung des linken Auges vor. Es wurde die Diagnose einer bilateralen Erdheim-Chester-Erkrankung (ECH) der Orbita gestellt. Die weitere Diagnostik ergab eine retroperitoneale Fibrose. Eine Therapie mit Interferon-α wurde durchgeführt, aber im Verlauf kam es zu einer bilateralen Kompressionsneuropathie mit Visusabfall und Gesichtsfeldausfällen. Eine bilaterale orbitale Dekompression wurde durchgeführt.AbstractA 60-year-old man presented with left exophthalmos and deterioration in visual acuity of slow evolution. Bilateral orbital Erdheim–Chester disease was diagnosed. Systemic evaluation revealed a retroperitoneal fibrosis. Treatment with interferon-α followed, but bilateral compressive optic neuropathy with visual acuity deterioration and visual field defects evolved. Bilateral orbital decompression was performed.A 60-year-old man presented with left exophthalmos and deterioration in visual acuity of slow evolution. Bilateral orbital Erdheim-Chester disease was diagnosed. Systemic evaluation revealed a retroperitoneal fibrosis. Treatment with interferon-alpha followed, but bilateral compressive optic neuropathy with visual acuity deterioration and visual field defects evolved. Bilateral orbital decompression was performed.