[Papillary edema in Muckle-Wells syndrome]. / Papillenschwellung bei Muckle-Wells-Syndrom.

Papillary edema may occur isolated without functional impairment or secondary related to various syndromes, increased intracerebral pressure or associated with medicinal treatment. The Muckle-Wells syndrome is a rare disease, which among many other symptoms can lead to optic disc swelling and recurrent increase in intracerebral pressure. Besides familial cold-induced autoinflammatory syndrome (FCAS) and neonatal onset multisystem inflammatory disease (NOMID), the Muckle-Wells syndrome also belongs to the cryopyrin-associated periodic syndromes (CAPS). In most cases of CAP syndromes there is an underlying genetic disorder that leads to overproduction of interleukin-1ß (IL-1ß); therefore, typical symptoms include inflammation reactions, such as repeated skin rash, fatigue, fever, joint pain and conjunctivitis.

[Successful treatment of Fusarium-associated keratitis with multiresistant pathogen and multimorbid patient]. / Erfolgreiche Therapie einer Fusarium-assoziierten Keratitis bei multiresistentem Erreger und multimorbidem Patienten.

A 75-year-old man (not a contact lens wearer) presented with Fusarium-associated hypopyon keratitis. After several weeks of empirical and subsequently targeted antimycotic treatment, no considerable improvement was observed. However, after sclerokeratoplasty (11.2 × 11.2 mm) combined with prolonged antimycotic therapy a good local result with relapse-free long-term follow-up was achieved.

[Causes, diagnostics and therapy for paediatric ptosis]. / Ursache, Untersuchungsgang und Behandlung der kindlichen Ptosis.

BACKGROUND: The diagnosis of and therapy for paediatric ptosis present challenges because of difficulties in performing preoperative examinations and the inability of the patient to provide intraoperative cooperation for proper lid placement. The authors provide an overview of the different forms and findings in congenital ptosis patients and point out the difficulties of the surgical procedures. DIAGNOSTICS AND THERAPY: The majority of paediatric ptosis cases is simple unilateral congenital ptosis with dysgenesis of the levator palpebrae superioris muscle. Other different forms exist due to neurological, neuro-myogenic, aponeurotic, sympathic, and mechanical reasons or syndromes. The relevant history is obtained, including birth history and family history, careful observation and full ophthalmological examination are necessary. Amblyopia because of ptosis, strabismus or anisometropia with corneal astigmatism should be recognised and treated early. The preoperative examination is vital for determining the appropriate diagnosis and is useful for selecting the appropriate procedure. Ptosis correction is based on ptosis severity, Bell phenomenon and levator function. The primary goal is symmetry of the upper lids. Most frequently a levator resection is performed between the 3rd and 5th year with a levator function of more than 3 mm. The most common complication is undercorrection, poor lid contour or amblyopia. Overcorrection may be associated with dry eye syndrome and keratopathy. CONCLUSION: Levator resection is a useful procedure for the correction of mild to moderate ptosis. Frontalis suspension surgery is effective for congenital ptosis with poor levator function.

[Examination of preschool children for refractive errors. First experience using a handheld autorefractor]. / Untersuchung von Kindergartenkindern auf Refraktionsanomalien. Erste Erfahrungen mit einem handgehaltenen Autorefraktometer.

BACKGROUND: Since ametropia is a leading cause of amblyopia refractive screening of preschool children is recommended. Measurement of refraction using tabletop autorefractors can be difficult or impossible due to lack of cooperation in this age group. The intention of the present study is to evaluate the use of a new handheld autorefractor for screening in kindergartens. METHODS: A total of 216 children aged 3.5-4.5 years were examined without cycloplegia using the handheld autorefractor (SureSight, Welch Allyn) in their kindergartens. RESULTS: Using the test device the majority of the examined eyes showed mild hyperopia (on average +1.18+/-0.87 dpt spherical equivalent) and mild astigmatism (on average 0.60+/-0.46 dpt cylinder power). The reproducibility of repeated autorefractor readings was higher for cylinder power and axis than for the spherical equivalent. Autorefractor readings of 55 eyes could be compared with the results of cycloplegic retinoscopy. The results were similar between the two measurements with differences of no more than 0.5 dpt in 18.2% for the spherical equivalent, in 82.1% for cylinder power, and in 66.6% for axis (weighted axis difference). There was adequate cooperation in 99.5% of the children. CONCLUSION: The handheld autorefractor proved an accuracy of measurement comparable to that of conventional tabletop autorefractors for cylinder power and axis with limitations in accuracy for the spherical equivalent. Validity and threshold values for relevant abnormalities are to be evaluated in further studies. By the high cooperation rate the instrument proved useful for screening examinations in preschool children.

[Extended indications for decompression of the optic nerve: a differentiating analysis of restriction of the visual function, also in comatose patients]. / Erweiterte Indikationen zur Optikusdekompression: Eine differenzierte Analyse visueller Funktionseinschränkungen--auch bei bewusstlosen Patienten.

BACKGROUND: The care of indirect traumatic optic neuropathy is still treated differently. Special diagnostic and therapeutic difficulties exist in comatose patients without definable visual acuity and in patients with complex failure of the visual field with central visual acuity still receiving sufficiently. METHOD: The total collective of optic nerve decompressions within the period between January 1992 and August 2000 was comprised of 66 patients. 18 of these 66 patients (average age: 40.5 years) were comatose and required critical care. 3 of the 48 consciousness-clear patients showed post-traumatically different defects of the visual field with a visual acuity of 0.3, 0.4 and 1.0. The indication for surgical decompression in both groups was based on the ophthalmological findings and the CT-finding of traumatization of the optic nerve, or the orbit apex respectively. RESULTS: During the subsequent postoperative examinations (on average 12.3 months postoperatively) five patients within the group of the unconscious patients showed a normal visual acuity (0.9-1.0), 3 patients a visual acuity of 0.7, 0.4 and 0.3 and one a visual acuity of 0.1. Six patients remained amaurotic. Three patients died from the general consequences of injury. Improvement of visual field and correction of stereoscopic function occurred in all patients, operated on primarily because of the described visual defects. CONCLUSIONS: Due to these results the indication for the decompression of the optic nerve should find special consideration in comatose patients and in cases of severe restriction of the visual field.

Optic nerve decompression in the comatose and conscious patients after trauma.

OBJECTIVE: To investigate the efficacy of early optic nerve decompression in comatose and conscious patients with indirect traumatic optic neuropathy. STUDY DESIGN: Retrospective analysis of 65 optic nerve decompressions. METHODS: The total collective of optic nerve decompressions comprised 65 patients treated within the period between February 1987 and December 1998. Thirteen of these 65 patients (average age, 32 y) were comatose and required critical care treatment, so visual acuity could not be measured. The indication for surgical decompression in all patients was based on the ophthalmological examination and the finding on computed tomography (CT) scan of a lesion inside the optic nerve canal or the orbit apex, respectively. RESULTS: In the comatose patients the time interval between trauma and surgery was 16.1+/-12.1 hours (mean +/- standard deviation). During the subsequent postoperative examinations (on average, 12.3 mo postoperatively) five patients showed a normal visual acuity (20/20), two patients a visual acuity of 20/30 and 20/50, and one a visual acuity of 20/200. Three patients (three eyes) remained amaurotic. Two patients died of the general and severe consequences of injury. The beneficial visual acuity results achieved within the group of comatose patients were equivalent to those achieved within the group of patients who were conscious during the preoperative examination. The success rate, defined as an improvement of three lines with a final visual acuity of at least 20/1000, was 57.7% versus 61.5%. CONCLUSION: The results confirm our concept of early decompression of the optic nerve, based on close interdisciplinary cooperation and the ophthalmological findings.

[Unilateral eyelid swelling as an initial manifestation of angiosarcoma]. / Einseitige Lidschwellung als Erstmanifestation eines Angiosarkoms.

Cutaneous angiosarcomas mainly arise on the scalp and face of older patients. They present with reddish nodules or plaques and tend to bleed and ulcerate. Angiosarcomas show an aggressive and recurrent course and have a high potential for metastasis. We report on a 48-year-old patient with unilateral eyelid edema as the first manifestation of an angiosarcoma. The clinical presentation, the history of contact allergy, the presence of antinuclear antibodies, and abnormal thyroid function tests favoured a non-neoplastic disease. However, multiple biopsies confirmed the diagnosis of a highly differentiated angiosarcoma. Staging of the patient revealed no metastasis. After radical surgery including orbital exenteration and postoperative radiation therapy, no recurrence was observed with a follow up of 26 months. This case underlines the importance of including angiosarcoma in the differential diagnosis of unilateral eyelid edema.

Endophthalmitis induced by atypical mycobacterial infection.

Granulomatous uveitis induced by Mycobacterium tuberculosis infection has been less frequently encountered over the last 20 years. Differential diagnosis of M. tuberculosis and other atypical mycobacteria is nevertheless of particular interest in patients with acquired immune deficiency syndromes. We present the case of a 60-year-old man who was referred to our clinic because of therapy-refractive endophthalmitis. Diagnostic vitrectomy including microbiological examination as well as further extensive serological examinations did not reveal any further information on the etiology. Despite intensive anti-inflammatory treatment, the eye became blind and developed secondary glaucoma. Evisceration of the globe had to be performed. Histopathological examination showed a granulomatous inflammation without the tuberculosis-typical development of central caseous necrosis. Further molecular pathological investigations had to be performed with regard to differential diagnostic aspects. By means of the polymerase chain reaction we could detect mycobacterial gene sequences that corresponded to atypical mycobacteria. The diagnosis of atypical mycobacterial infection was confirmed Molecular-genetics examination techniques, especially those involving the polymerase chain reaction, will become more important in routine histopathological examination. These techniques can be performed on paraffin-fixed or formalin-embedded material for further differential diagnostic considerations.

[The MRT of the orbit: the value of T1-weighted frequency-selective fat saturation at 1.0 and 1.5 tesla]. / MRT der Orbita: Wertigkeit der T1-gewichteten frequenzselektiven Fettsättigung bei 1,0 und 1,5 Tesla.

PURPOSE: To evaluate the diagnostic performance of fat suppression T1-weighted compared to conventional T1-weighted gadolinium-DTPA-(Gd-)enhanced spin echo sequences in orbital imaging. MATERIAL AND METHODS: 31 patients with pathologic conditions or clinically suspected pathology of the orbita were examined using conventional T1-weighted (TR 400-500 ms/TE 15 ms/AC 3) and fat suppression T1-weighted (TR 370-500 ms/TE 15 ms/AC 3) spin echo-sequences before and after i.v. injection of 0.1 mmol/kg Gd-DTPA. All images were evaluated by three radiologists experienced in MR imaging, concerning anatomical and pathological structures as well as artifacts. The images were graded on a scale of 1-5 (5 = excellent to 1 = poor). RESULTS: The evaluation of anatomic structures showed that the fat-suppression technique was superior to the conventional technique in respect of visualising the extraocular muscles (4.4 to 3.4, p < 0.05) and also with regard to the optic nerve (3.68 to 3.78). Pathological conditions of the orbit (optic glioma and neuritis, spread of local tumours to the orbit), however, were better visualised using the fat suppression technique (4.3 to 2.95, p < 0.05). CONCLUSION: Contrast-enhanced T1-weighted fat suppressed spin echo-sequences are useful in pathological conditions of the orbita. In optic neuritis they may be the only sequences to show the lesion.

[Detection of atypical mycobacterial endophthalmitis by PCR]. / Nachweis einer atypischen mykobakteriellen Endophthalmitis durch PCR.

We report a case of a 60 year old male who presented with a therapy resistant endophthalmitis which led to an evisceratio bulbi. Histologically, a granulomatous inflammation was present, in which acid-fast bacilli could not be detected. Molecular genetic examination by means of polymerase-chain-reaction technique (PCR) of the routinely processed tissue material revealed the diagnosis of an atypical mycobacterial infection, because DNA specific for atypical mycobacteria was detected. This example shows that PCR technique provides a useful tool in the diagnosis of mycobacterial infections as an adjunct to surgical pathology.

[Early vitrectomy in endogenous juvenile uveitis intermedia--a long-term study]. / Frühvitrektomie bei endogener juveniler Uveitis intermedia--Eine Langzeitstudie.

BACKGROUND: The intermediate uveitis is one of the most common intraocular manifestations of inflammation during infancy and adolescence. The success of intensive pharmacological treatment is rather limited and often associated with heavy side effects. PATIENTS AND METHODS: In 25 eyes with juvenile uveitis intermedia (age of the patients between 5 and 18 years, mean 13.5 years) and visual acuities between 0.02 and 0.4 (mean 0.19) an early vitrectomy was performed after failure of conservative treatment and peripheral cryocoagulation. RESULTS: Almost all patients (22 out of 25) showed an improvement of visual acuity within the first 6 months of the surgery. Among the patients with a remarkable improvement of visual acuity 7 eyes had a chronic cystoid macular oedema which showed a regression postoperatively. Additionally, the number and the course of inflammatory periods could be reduced postoperatively. An increase of preexisting lens opacities, however, has not been noticed even after a follow-up period of 10 years. CONCLUSIONS: The results show that an early vitrectomy in cases of juvenile uveitis intermedia often leads to a stabilization or an improvement of visual acuity and a regression of the inflammatory attacks (episodes). Therefore, in children and adolescents with endogenous uveitis intermedia a pars plana vitrectomy instead of a long-term systemic immunosuppression associated with heavy side-effects should be considered.