[Orbital-bony decompression in patients with dysthyroid orbitopathy--first Croatian experiences]. / Kostana orbitalna dekompresija u pacijenata s distireoidnom orbitopatijom--prva hrvatska iskustva.

INTRODUCTION: Dysthyroid orbitopathy or Graves's orbitopathy is an entity made of symptoms and signs found within ocular apparatus, orbit and adnexal tissue in patients with thyroid dysfunction. Orbital pathogenesis is based on organ-specific autoimmune reaction, resulting in extra ocular muscle dysfunction and retro bulbar tissue changes. These changes are characterized by different stages of orbital inflammation, swelling and passive congestion. No infiltrative or inactive stage of the disease is most commonly seen in younger population as mild, orbital inflammation, lid retraction, variable proptosis and retro bulbar discomfort. Infiltrative or active stage of the disease usually affects older people and is characterized by a broad spectrum of clinical symptoms such as moderate or severe orbital inflammation, retro bulbar pain, lid retraction, periocular edema, proptosis with corneal exposure, extra ocular muscle dysfunction, conjunctival and episcleral congestion and optic nerve compression with loss of visual acuity. The current treatment concept for dysthyroid orbitopathy is to achieve euthyroidism with antithyroid drugs, radioiodine and thyroidectomy. Subsequently, for the treatment of dysthyroid orbitopathy immunosuppressive drugs such as corticosteroids, cyclophosphamide, methotrexate, cyclosporine, octreotides, orbital radiotherapy and surgical decompression can be used. Indications for orbital decompression are differentiated as urgent and elective. Current opinion is that only severe proptosis with corneal exposure and damage and compressive optic neuropathy urge orbital bony decompression combined with aggressive medical treatment. Persistent, active orbitopathy, retro bulbar discomfort or pain and disfiguring proptosis are indications for elective orbital bony decompression. Orbital bony decompression is a surgical method based on principle of opening the confined orbital bony space and allowing herniation of orbital fat into paranasal sinuses, thus decreasing orbital pressure. PATIENTS, METHODS AND RESULTS: A series of patients surgically treated for dysthyroid orbitopathy from July 2004 till November 2005, were followed-up. The group included 8 patients previously treated for thyroid dysfunction. Five patients (10 eyes) were operated on by the "swinging eyelid" approach (group 1) and three patients (6 eyes) by "inferomedial approach" (group 2). In group 1 proptosis reduction occurred in all patients, varying from 4 to 6 mm within 6-10 months postoperatively; retrobulbar pain or discomfort decreased in all patients few days after the surgery; and 2 patients still felt numbness in the n. infraorbitalis innervation area at 10 months of follow up. In group 2, all patients had proptosis reduction by 3-4 mm within 6-10 months postoperatively; retro bulbar discomfort disappeared in all patients a few days after the surgery. In this group none of the patients complained of infraorbital numbness 6 months of the surgery. All patients filled out a brief questionnaire before and 4 months after the surgery, ranking their symptoms and complaints. CONCLUSION: Our patient series was rather small and selected, the data obtained are not representative of larger series. Based on these preliminary results and current literature data, we believe that orbital bony decompression is a highly effective, safe and quick procedure, easily adjustable to the surgeon as well as to the patient in achieving optimal results. We also believe that there is a need of better education of our patients considering the disease itself and treatment modalities through patient support groups or better physician-patient-nurse communication.

[Ocular findings in Fabry's disease]. / Fabryjeva bolest i promjene na ocima.

INTRODUCTION: Fabry's disease is a recessive X-linked disorder that results from a deficiency of the lysosomal hydrolase a-galactosidase A (alpha-Gal A). The absence of alpha-Gal A enzyme activity leads to accumulation of glycosphingolipid globotryaosyl ceramide (GL-3) in the lysosomes of a variety of cell types. Subsequently, angiokeratoma and ocular signs develop until, in most cases after the third decade of life, severe renal dysfunction or cardiomyopathy becomes obvious. Corneal opacities (cornea verticillata) occur in 90% conjunctival vascular changes in 60%, retinal vessel tortuosity in 55%, and cataracts in 50% of cases. Recently, enzyme replacement therapy has been shown to be an effective treatment modality that can eliminate glycolipid stores and reverse the disease pathology. CASE REPORT: A male patient born in 1971 was admitted for clinical examination due to proteinuria and erythrocyturia. During further evaluation nephrologist suspected Fabry's disease, because patient had skin changes early referred as petechiae, and acroparesthesias. He had also low heat tolerance and virtually no sweat. Physical status: angiokeratoma on gluteat regions and upper arms. Urine analysis in several occasions 10-15 E in sediment, alb. positive. On ECG, 2-mm depression of ST in precordial region. Heart ultrasound: low mitral regurgitation angio stage 1, left ventricle hypertrophy. Abdomen ultrasound: both kidneys around 12 cm large, parapyelic cysts in both kidneys of 2.5 cm in diameter. Biomicroscopy of both eyes: cornea verticillata. Fundus of both eyes: papillae n. optici with poorly defined edges, but without prominence, very tortuotic retinal blood vessels. In March 2002, very low alpha GAL enzyme activity in blood (alpha-Gal A = 0.5 +/- 0.2 nmol of substrate hydrolyzed hourly per serum mL--normal serum enzyme level 8.5-18.9 nmol/mL/h). In May 2002, enzyme replacement therapy was started with recombinant alpha-Gal A enzyme (Fabrazyme) 1 mg/kg every 14 days. Control evaluation and examination showed good cardiac and renal function. The patient felt better and stronger with improved heat tolerance. CONCLUSION: Fabry's disease occurs in all ethnic groups. It is estimated that one in 200 people is a carrier, and one in 40,000-100,000 has the disease. Today in Croatia, Fabry's disease has been diagnosed in only one patient, and according to the usual prevalence there are still 45-100 unrecognized patients. The ophthalmologists are in excellent position to diagnose Fabry's disease in early stages. Therefore it is very important that the ophthalmologists in Croatia become aware of the importance of ocular findings in Fabry's disease, so they can participate in the identification of unrecognized patients.

Review and new insights on Wegener granulomatosis.

The purpose of this paper is to present Wegener granulomatosis manifesting in its periocular form. Review with new developments in understanding of the etiopathogenesis, clinical and laboratory findings as well as therapy modalities are shown through a series of patients gathered in collaboration with colleagues from UMC Utrecht, Netherlands. In the period from 1992 until 2004 the group of 54 patients with established diagnosis of Mb. Wegener were observed. 13 patients developed periocular form but only 2 presented it as initial symptoms. Lacrimal stenosis and orbital infiltration were predominant periocular symptoms while nasal manifestations were predominant systemic symptoms of the disease. Different treatment modalities were employed showing that orbital disease is difficult to treat in spite of satisfying systemic answer to immunosuppressives which calls for alternative solutions.

Treatment of ocular motility disturbances in Graves' disease with botulinum toxin A.

The aim of this study was to evaluate the efficacy of botulinum toxin-A injections on motility disturbance in patients with dysthyroid eye disease. In 36 patients (52 orbits) with active phase of Grave's ophthalmopathy with motility disturbance, botulinum toxin-A injections were applied. Ocular motility was measured before and after treatment in four main directions (elevation, depression, abduction and adduction) with Förster perimeter. In all patients 20 units of botulinum toxin-A in one single injection was applied in the projection of the inferior rectus muscle. Statistically, the degree of upgaze increased notably in all three groups, but mostly in the first group where it amounted up to 5.8-6.0 (z = 10.0;p = 0.68). We could not prove notable increase of the motility grade in the adduction in none of the groups of the tested patients. Due to the fact that eyeball motility can be objectively measured, effect of the applied therapy can be clearly evaluated.

Long-term results of frontal lobe suspension in children with congenital dystrophic ptosis.

Long-term results achieved by our own operative technique in children with congenital dystrophic ptosis, with frontal muscle lobe shaping with or without shaping of corrugator muscle lobe attached to the tarsal plate, are presented. Data on 146 patients with congenital dystrophic ptosis operated on during the 1984-1998 period at Zagreb University Hospital Center were retrospectively analyzed. Postoperative success was defined as a situation with eyes open in which 1) upper eyelid covers the cornea at 12 o'clock position by 1-2 mm; 2) there is a good contour of the eyelid margin; 3) there is no lagophthalmos; and 4) there is symmetry with the other eye. Immediate re-operation due to undercorrection was required in 26 of 146 (18%) patients. Upon re-operation, 133 (91%) patients met the criteria for successful outcome at 6 months, 124 (85%) at one year, and 121 (83%) at 5 years. Correction of congenital dystrophic ptosis using a shaped frontal/corrugator lobe is an efficient and safe procedure ensuring long-lasting success.

Lens wearers non-compliance - is there an association with lens case contamination?

PURPOSE: This study aims to investigate the association of compliance amongst lens wearers and lens case contamination as assessed by the microbial profile of lens cases. METHODS: Fifty-two asymptomatic lens wearers filled out questionnaires seeking demographic data and several aspects of compliance to lens wear. Subsequently, contamination profiles of the inside bottom and rim of their lens cases was obtained for bacteria, fungi and Acanthamoeba. The association of the self-reported responses in the questionnaire with contamination profile of the lens case was then analyzed. RESULTS: Based on compliance criteria, 21% of the participants were described as fully compliant lens wearers. Contamination of lens case was prevalent in 42% of the cases. Frequent non-compliant behaviours reported by study participants included, showering and sleeping with contact lenses, and irregular lens case replacement. In comparison to soft contact lens wearers prevalence of non-compliance and contamination amongst rigid gas permeable (RGP) lens wearers was significantly greater. There was a statistically significant correlation between bacterial contamination and current case age. CONCLUSION: These results suggest that majority of lens wearers do not fully comply with recommendations for lens wear and case care. Crucially, none of the fully compliant patients had contaminated lens cases. However it would appear that several non-compliant behaviours significantly increase the risks of case contamination. These results suggest that greater efforts should be invested with lens wearers to ensure enhanced compliance as this is likely to reduce the possibility of case contamination.

A case of lacrimal gland dirofilariasis.

A patient with a diagnosis of chronic and idiopathic unilateral dacryoadenitis was submitted to our clinic for diagnostic reasons from a regional hospital. Within a period of 1 year, this was the second onset of a swelling in the region of the lacrimal gland which - unlike the first time - showed resistance to antibiotic treatment. The patient's general and ophthalmic condition was good, and his only complaint was of cosmetic nature because of the disfiguring swelling in the lateral part of the left upper eyelid. Basic laboratory findings showed no abnormalities and radiographic imaging showed enlargement of the left lacrimal gland without bony destruction. Guided by epidemiological data for noninfectious dacryoadenitis we decided to perform excisional biopsy of the left lacrimal gland. To our surprise, the pathologist verified the presence of a worm of the genus Dirofilaria, which is, to the best of our knowledge, the second reported case of lacrimal gland dirofilariasis in the literature and the second reported case of such a disease in Croatia.

Endonasal endoscopic orbital decompression in patients with Graves' ophthalmopathy.

AIM: To present the results of endonasal endoscopic orbital decompression in patients with Graves' ophthalmopathy. METHODS: Endonasal endoscopic orbital decompression was performed in 32 orbits of 21 patients with Graves' ophthalmopathy. In 17 patients the surgery was performed because of active ophthalmopathy non-responsive to conservative treatment, and in 4 patients for esthetic reasons. Preoperative and postoperative examination included visual acuity, examination of the eyelids and cornea, ocular motility, cover testing, Hertel exophthalmometry, and applanation tonometry. RESULTS: Visual acuity improved from preoperative 0.81+/-0.28 (mean +/- standard deviation) to postoperative 0.92+/-0.21 (p=0.0032, Student t-test). Retraction of upper and lower eyelids, as well as exposure keratitis, was reduced after operation (p<0.001). Mean proptosis reduction in all orbits was 4.6+/-1.7 mm (p<0.001). An average reduction of intraocular pressure was 3.4+/-3.0 mmHg (p<0.001). New-onset diplopia developed in 8 patients. Diplopia persisted in 9 out of 11 patients who had preoperative diplopia. Two patients experienced postoperative relief of diplopia. Ocular motility was subsequently corrected by eye muscle surgery in 13 eyes, whereas prisms were used in other 5 manifestly strabic eyes. CONCLUSIONS: Endonasal endoscopic orbital decompression procedure improved visual acuity, decreased proptosis and intraocular pressure, and also had favorable cosmetic results in most patients. Post decompression diplopia and strabismus were successfully managed by either eye muscle surgery or application of prisms.