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Operative Entlastung bei endokriner Orbitopathie
Abstract
Background:
Endocrine orbitopathy is a common feature of autoimmune thyroid disease. The increase of pressure within the eye socket leads to proptosis and compression of the optic nerve. This results in cosmetic impairment as well as loss of vision. Apart from the medical treatment surgical decompression of the crowded orbit offers a valid therapeutic option.
Patients and Methods:
142 patients (264 orbits) were treated at the interdisciplinary orbital center, Johannes Gutenberg University Hospital, Mainz, Germany. The medial orbital wall was removed, followed by lipectomy. Indications for decompression included cosmetic reasons (196 orbits, 74.2%), dysthyroid optic neuropathy (67 orbits, 25.4%), and in one case corneal ulceration.
Results:
A median reduction of the severity score according to the NOSPECS classification from 7 points preoperatively to 4 points 3 months after surgery was achieved (p < 0.001). Median proptosis decreased from 23 to 20 mm (p < 0.001). Intraocular pressure in upgaze dropped from 23 mmHg by 3 mmHg during the first postoperative 3 months (p < 0.001). Additionally, the influence of age, sex and smoking behavior on the operation outcome was examined. Diplopia was present in 100 patients before surgery. Of the remaining 42 patients, two patients (4.8%) complained about new-onset constant diplopia 3 months after decompression, while 22 patients (52.2%) reported inconstant/intermittent motility disorders. Squint surgery corrected diplopia.
Conclusion:
The combined transnasal-endoscopic/transpalpebral approach represents a safe and efficient method for severe endocrine orbitopathy.
... Many of them are based on minimally invasive techniques and include a removal of the medial, lateral and inferior wall or a combination of these [5][6][7][8]. While lateral decompression alone has the lowest rate of new onset diplopia (NOD) (3-8%), it only results in a relatively small decrease in the Hertel Index of about 3-4 mm [9][10][11][12]. Medial decompression has the highest rates of NOD (35-40%), but with greater exophthalmos reduction than lateral decompression [13,14]. ...
... Different authors described a relatively high rate of NOD after resection of the medial orbital wall (35-40%) [13,14], that is up to 50% after resection of the medial and inferior wall [31]. If the lateral wall alone was resected, NOD occurred in only 3-8% [9][10][11][12]. Using techniques that combined the resection of the medial and the lateral wall the reported rate of NOD is about 7-35% [16][17][18][19][20]. Accordingly, the rate of NOD in our collective was lowest in patients with low requirement of exophthalmos reduction after resection of the lateral wall and the posterior part of the medial wall. ...
Purpose
To determine the outcome after orbital decompression using a graduated technique, adapting the surgical technique according to individual patients’ disease characteristics.
Methods
We retrospectively examined the postoperative outcome in patients treated with a graduated balanced orbital decompression regarding reduction of proptosis, new onset diplopia and improvement in visual function. 542 patients (1018 orbits) were treated between 2012 and 2020 and included in the study. Clinical examinations including visual acuity, exophthalmometry (Hertel) and orthoptic evaluation were performed preoperatively and at minimum 6 weeks postoperatively. Mean follow-up was 22.9 weeks.
Results
Mean proptosis values have significantly decreased after surgery (p < 0.01). In 83.3% of the patients Hertel measurement normalized (≤ 18 mm) after surgery, New onset diplopia within 20° of primary position occurred in 33.0% of patients, of whom 16.0% had preoperative double vision in secondary gaze. Patients suffering from dysthyroid optic neuropathy (DON) had a significant increase in visual acuity (p < 0.01).
Conclusion
We demonstrated that individually adapted graduated orbital decompression successfully improves key disease parameters of Graves’ orbitopathy with low morbidity.
Die Orbitadekompression ist ein wirksamer Eingriff zur Senkung des intraorbitalen Drucks. Ursächlich können Erkrankungen mit schneller Druckerhöhung sein, z. B. Einblutungen, und solche mit langsamer Drucksteigerung, z. B. Tumoren oder endokrine Orbitopathie. Bei der reinen Fettgewebsdekompression wird peri- und retrobulbäres Fettgewebe aus der Orbita entnommen, bei der knöchernen Dekompression werden knöcherne Wandungen entfernt (1-Wand, 2‑Wand oder 3‑Wand-Dekompression). Häufig werden beide Verfahren miteinander kombiniert. Neuere Entwicklungen sind die transkonjunktivalen Zugangswege, über die ebenfalls Teile der knöchernen Wände reseziert werden können. Komplikationen sind Doppelbilder, die je nach Op.-Methode in bis zu 30 % der Fälle auftreten können, sowie Blutungen, Infektionen, Entwicklung einer chronischen Sinusitis und Läsionen der Dura mit konsekutiver Meningitis. In der Hand des erfahrenen Nasennebenhöhlen- und Kopf-Hals-Chirurgen ist der Eingriff komplikationsarm.
The purpose of this study was to assess a piezosurgical device as a novel tool for bony orbital decompression surgery. At a multidisciplinary orbital center, 62 surgeries were performed in 40 patients with thyroid associated orbitopathy (TAO). Within this retrospective case-series, we analyzed the medical records of these consecutive unselected patients. The reduction of proptosis was the main outcome measure. Indications for a two (n = 27, 44%) or three wall (35, 56%) decompression surgery were proptosis (n = 50 orbits, 81%) and optic neuropathy (n = 12, 19%). Piezosurgery enabled precise bone cuts without intraoperative complications. Proptosis decreased from 23.6 ± 2.8 mm (SD) by 3 mm (95% CI: -3.6 to -2.5 mm) after surgery and stayed stable at 3 months (-3 mm, 95% CI: -3.61 to -2.5 mm, p < 0.001, respectively). The effect was higher in those with preoperatively higher values (>24 mm versus ≤24 mm: -3.4 mm versus -2.81 mm before discharge from hospital and -4.1 mm versus -2.1 mm at 3 months: p < 0.001, respectively). After a mean long-term follow-up period of 14.6 ± 10.4 months proptosis decreased by further -0.7 ± 2.0 mm (p < 0.001). Signs of optic nerve compression improved after surgery. Infraorbital hypesthesia was present in 11 of 21 (52%) orbits 3 months after surgery. The piezosurgical device is a useful tool for orbital decompression surgery in TAO. By cutting bone selectively, it is precise and reduces the invasiveness of surgery. Nevertheless, no improvement in outcome or reduction in morbidity over conventional techniques has been shown so far.
To present the results of endonasal endoscopic orbital decompression in patients with Graves' ophthalmopathy.
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.
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.
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.
Purpose
Graves' ophthalmopathy (GO) is an organ-specific autoimmune disease. Hydrophily of accumulated acidic mucopolysaccharides into bulbar adipose tissue leads to swelling of the eye muscles. Orbital surgical decompression is performed in severe cases of compressive optic neuropathy and severe corneal exposure or failure of steroid therapy. The study was designed to evaluate decompression surgery with respect to the clinical benefit and the patient's satisfaction by means of a disease-specific questionnaire.
METHODS
The 90-item study questionnaire was distributed to 105 patients with GO who underwent orbital decompression surgery at the authors' institution.
Results
A total of 88% of patients stated that decompression had helped them, 80% of the interviewees would undergo decompression again, 78% were content with their eye symptoms, and 71% were satisfied with the cosmetic result of decompression. Furthermore, analysis showed a clinically relevant increase in quality of life after surgery. The correlation between the clinical endpoint proptosis at last examination and the quality of life score proved to be significant (p=0.05).
CONCLUSIONS
The large majority of interviewees were satisfied with the result of the orbital decompression. These results confirmed that disfiguring proptosis is an important indication for decompression surgery.
From 1985 to 1998 at the Plastic Surgery Unit in Wesseling we carried out 1362 decompressions of the orbit on 697 patients with Graves' disease. Intraorbital fat was removed trough transpalpebral incision to achieve decompression. From this patients we have exact follow-up of 511 patients and 1000 orbits. (Follow-up from 6 months to 12 years, average 27 months.) Average history of disease was 3.8 years. Female 3.7 (0.5-30), male 4.0 (1-17). Mean age was 45 (22-75) years. 92% of the patients were female and only 8% male. When we compared the results to a traditional decompression (with resection of one, two or more orbital walls), we found that the postoperative complication rate was significantly lower and the success rate much higher. The exact causes of thyroid-associated orbitopathy (TAO) are still unknown; for this reason there is as yet no causal therapy. Despite considerable progress in understanding the pathophysiology of TAO, therapy has remained virtually unaltered over the last decades, viz. the following.
A surgical endonasal procedure is described to perform orbital decompression in patients suffering from ophthalmopathy in Grave's disease. The decompression technique employs removal of the lamina papyracea as an exclusive modality or in conjunction with a transmaxillary or lateral decompression approach. This present series contain 23 patients who underwent decompression over a 3-year period and were followed for at least 6 months postoperative. The results of decompression were assessed by measuring reduction in proptosis and visual acuity. All patients demonstrated improved visual acuity. Measuring retroplacement of the globe, the endonasal approach proved to equal the results obtained after a combined transantral-endonasal approach. Orbital fat was removed depending on the degree of proptosis. The complication rate was low, and establishment of an ocular muscle training program proved helpful.
Orbital decompression has been used to describe surgical procedures that remove some portion of the orbital walls to reduce pressure on the orbital contents. Substantial morbidity associated with these procedures includes infraorbital anesthesia, worsened extraocular motility, globe displacement, and blindness. The authors believe that orbital contents also may be decompressed by removing orbital fat.
Eighty-one patients with nonactive Graves orbitopathy were selected for orbital fat decompression based on the presence of proptosis and associated signs and symptoms to avoid bone removal. Soft-tissue analysis by computed tomography (CT) scan showed distended pockets of fat extending into the intraconal space, which were removed through medial-upper and lateral-lower anterior orbitotomies. Decompression with bone removal was reserved for those few patients with compressive optic neuropathy unresponsive to medical treatment and those patients with residual deforming exophthalmos after fat removal.
One hundred fifty-eight fat decompressions were performed on 81 patients over 9 years. The authors measured an average reduction in proptosis of 1.8 mm (range, 0-6.0 mm). The greatest average reduction in proptosis (3.3 mm) was produced in patients with preoperative Hertel measurements of greater than 25.0 mm. Morbidity was limited to temporary motility impairment of the inferior oblique in two patients.
The concept of orbital decompression can include removal of orbital fat to reduce proptosis, eliminate symptoms, and improve appearance with far less morbidity than when bone decompression is used as the primary decompressive procedure.
Thyroid-associated ophthalmopathy (TAO) has a major effect on the two compartments of the retro-orbital (RO) space, leading to enlargement of the extraocular muscles and other RO tissues. T lymphocyte infiltration of RO tissue is a characteristic feature of TAO and there is current interest in whether these T cells are specifically and selectively reactive to RO tissue itself. We recently established 18 T cell lines (TCL) from RO adipose/connective tissue of six patients with severe TAO by using IL-2, anti-CD3 antibodies and irradiated autologous peripheral blood mononuclear cells (PBMC) to maintain the growth of T cells reactive to autologous RO tissue protein fractions. Here we report on the phenotype characteristics and cytokine gene expression profiles of these orbital TCL and on their immunoreactivity to the organ-specific thyroid antigens thyrotropin receptor (TSH-R), thyroidal peroxidase (TPO) and thyroglobulin (TG). Flow cytometry revealed that 10 TCL were predominantly of CD4+ phenotype, three being mostly CD8+ and five neither CD4+ nor CD8+. Analysis with reverse transcriptase-polymerase chain reaction (RT-PCR) of cytokine gene expression revealed both Th1- and Th2-like products in all TCL: IL-2 product (in 17 TCL), interferon-gamma (IFN-gamma) (n = 10), tumour necrosis factor-beta (TNF-beta) (n = 15), IL-4 (n = 12), IL-5 (n = 17), IL-6 (n = 13), TNF-alpha (n = 12) and IL-10 (n = 4). Reactivity to thyroid antigens was observed only in two TCL, the other 16 being uniformly unreactive. Although 10 out of 18 RO tissue-reactive TCL were predominantly CD4+ there were no significant relationships between TCL phenotype, cytokine gene profile, magnitude of reactivity to RO tissue protein or the (rare) occurrence of thyroid reactivity. The findings of both Th1- and Th2-like cytokine gene expression in all RO tissue-reactive TCL support the concept that TAO is a tissue-specific autoimmune disease, distinct immunologically from the thyroid, and involving both T cell and B cell autoimmune mechanisms in disease pathogenesis.
In 35 patients with thyroid-associated orbitopathy, nonresponsive to conservative treatment, an endonasal microscopic approach with bimural osteotomy was performed for decompression in two ways. While in group A the periorbita was resected, and fat septa were cut, in group B periorbital strips were left, and fat septa were respected. In accordance with other authors, resection of the periorbital and cutting fat septa will improve vision and proptosis, but in up to 30% of the patients de novo diplopia occurs. Our data favor the assumption that a more conservative endonasal microscopic decompression of the orbita leaving periorbital strips and fat septa achieves similar good results for reduction of proptosis and visual gain but creates less often de novo diplopia in primary gaze. Based on our experience, rehabilitation for thyroid-associated arbitopathy comprises as a first step orbital endonasal decompression with cooperation of the ophthalmologist, then if necessary as a second step strabismus surgery, and thirdly eyelid repair for scleral show.
Orbital ultrasound, computed tomography, and magnetic resonance are commonly used as imaging techniques to demonstrate pathological changes in ocular adnexa of patients with Graves' ophthalmopathy. Low cost, short time of investigation, and lack of radiation characterize ultrasound. Nevertheless, a clear differentiation regarding disease activity is not possible, nor is the evaluation of orbital tissue precise enough. Short investigation time, precise imaging of the orbital apex and moderate costs are advantages of tomography. This method delivers a significant radiation dose to the lens, which if repeated constitutes a risk for cataract development. For this reason, magnetic resonance imaging is preferable, particularly if repeated scans are required to assess response to treatment. Precise tissue differentiation and lack of ionizing radiation uniquely suit magnetic resonance for eye studies. Although sensitive in demonstrating interstitial edema within the rectos muscles in active disease, as well as providing a good predictive value with respect to immunosuppressive therapy, quantitative magnetic resonance imaging is an expensive method and is non-specific for the orbital changes in ophthalmopathy. Because of a favorable target to background ratio, octreoscan carries a high sensitivity and may be regarded as a semi-objective tool in the evaluation of patients with Graves' ophthalmopathy, both at initial stages as well as during treatment. A positive orbital octreoscan indicates a clinically active disease in which immunosuppressive treatment might be of therapeutic benefit. However, it is an expensive method with a non-negligible radiation burden. Also, it is neither specific nor does it offer detailed orbital imaging. In summary, in unclear cases of proptosis or recently developed diplopia, prior to orbital decompression surgery, or if imaging is needed in subjects with ophthalmopathy, magnetic resonance actually is the imaging method of choice.
To review the literature related to thyroid-associated orbitopathy and to emphasize recent developments in its pathophysiology, diagnosis, and therapy. Current therapeutic trends and controversies are discussed.
Expression of thyroid stimulating hormone receptor is highest in the fat and connective tissue of patients with thyroid-associated orbitopathy, where fibroblasts have the potential for adipogenesis. Electrophysiology can now detect subclinical optic neuropathy, and somatostatin-receptor scintigraphy can help justify immunomodulation. Other than steroids, radiotherapy can control inflammation, but its use is controversial. Current trends in orbital decompression are to camouflage incisions and to limit strabismus with balanced decompression, deep lateral wall techniques, fat removal, and onlay implants. Proptosis reductions of 0.9 to 12.5mm are possible by the use of various algorithms. Before or after decompression, botulinum toxin can correct strabismus, intraocular pressure elevation, and retraction. The latter is now also treated with full-thickness blepharotomy.
As knowledge of the pathophysiology of thyroid-associated orbitopathy grows, there is a slow movement from nonspecific and invasive measures to more directed treatments causing less morbidity.
Alpha-fodrin, an intracellular organ-specific cytoskeleton protein is a recently identified autoantigen associated with Sicca- and Sjogren's syndrome (SS). SS frequently affects patients with Graves' ophthalmopathy (GO). We have therefore cloned and expressed the human recombinant 120-kDa fodrin-fragment. A sequential purification procedure was applied to isolate the recombinant protein. Using sera from patients with SS, the antigenicity of the purified fodrin fragment was demonstrated by immunoblotting. Sera from 144 patients with GO and 1200 blood donors were screened for the presence of anti-alpha-fodrin IgA and IgG antibodies by a newly developed ELISA using the human alpha-fodrin fragment as an autoantigen. In contrast to controls (<1% IgA only, P < 0.001) and to subjects with various autoimmune diseases (P < 0.001), alpha-fodrin antibodies were detected in 22% of patients with GO (n = 32). IgA and IgG antibodies were present in 21 (15%) and 14 (10%) GO subjects, respectively. A total of 45 patients with GO (31%) had at least one fodrin- or SS-antibody. GO patients with SS showed SS- and high titres of alpha-fodrin-antibodies. In GO patients, fodrin antibodies correlated with TPO- (P < 0.05) and SS-A (P = 0.002) antibodies. Thus, for the first time, antibodies reactive with fodrin are reported in patients with GO.
Glucocorticoids are effective for severe Graves' orbitopathy (GO), which causes substantial morbidity. The question at issue is how best to use them.
The objective of this study was to optimize glucocorticoid application in GO.
The study design was a randomized trial over 12 wk with 6-month follow-up.
The study was performed at university joint thyroid and ophthalmic clinics.
Seventy euthyroid out-patients with untreated, active, and severe GO were studied. Intervention: Patients received either once weekly iv methylprednisolone (0.5 g, then 0.25 g, 6 wk each) or oral prednisolone starting with 0.1 g/d, then tapering the dose by 0.01 g/wk.
At 3 months, the primary end point was a composite of improvements in proptosis, lid fissure width, and rate of diplopia in primary gaze, visual acuity, eye muscle thickness, and patient's quality of life.
Intravenous glucocorticoid therapy resulted in rapid, significant, and sustained improvement. At 3 months, 27 of 35 patients (77%) in the iv group had a treatment response compared with 18 of 35 (51%) in the oral group (P < 0.01). Improvements over baseline values for disease severity (e.g. visual acuity; P = 0.01) and activity (e.g. chemosis; P < 0.01) and for quality of life (P < 0.001) were greater in the iv group. TSH receptor antibody titers decreased during iv steroid administration (P < 0.001), and smoking had a strong impact on the therapy response (P < 0.001). Additional treatment was required less frequently in the iv group. Intravenous steroids were safe, with different rates of adverse events between the two groups (P < 0.001).
In patients with active and severe GO, iv glucocorticoids were more effective and better tolerated than oral steroids.
Thyroid-associated eye disease is an orbital manifestation of autoimmune thyroid disease. Its treatment, despite recent developments, is still challenging, and complete improvement of functional and/or cosmetic impairement is not always possible. This update tries to give an overview of actual concepts for conservative and surgical management.
Transpalpebrale Dekompressionsopera-tion bei endokriner Orbitopathie
- N Olivari
Olivari N. Transpalpebrale Dekompressionsopera-tion bei endokriner Orbitopathie. Wien Med Wo-chenschr 1988;138:452–5.