Sphenoorbital meningioma: surgical series and design of an intraoperative management algorithm.

Current surgical removal of sphenoorbital meningiomas (SOM) mainly aims at reduction of proptosis and restoration of visual function; some stages of the surgical technique are controversial. In this study, we aim to present a surgical decision-making algorithm for SOM. A retrospective study of 27 patients who underwent resection of SOM in our center (2005-2014) was conducted. The primary outcomes evaluated were postoperative visual function and radiological exophthalmos. In our study, clinical proptosis was the most common presenting sign (92%), followed by visual loss (37%). Our surgical algorithm includes (1) extracranial stage; (2) extradural stage, including removal of the anterior clinoid process only in cases of tumor invasion (22% of our cases); (3) intradural stage; (4) intraorbital stage, including opening of the periorbita only in the presence of resectable intraorbital tumor; and (5) reconstruction, including rigid orbital reconstruction only if the periorbita was violated (22%) and placement of a fat graft in the epidural space in most cases (85%). Complete tumor resection was achieved in 51.8%. The extent of resection was limited mainly due to invasion to the cavernous sinus (61.5%) and the superior orbital fissure (84%). Surgery achieved significant visual improvement in 80% and exophthalmos reduction in 77% of the patients. Preoperative visual deficit (P = 0.0001) and optic canal involvement (P = 0.04) appeared to predict postoperative improvement of visual function. Surgical complications were mainly transient cranial nerve deficits. Based on our results, we concluded that the proposed surgical algorithm leads to successful visual, cosmetic, and oncologic outcomes.

Toxina botulínica A en el músculo recto superior para la corrección de la retracción palpebral / Botulinum toxin A in the upper rectus muscle for the correction of eyelid retraction

Objetivo: evaluar los resultados terapéuticos obtenidos con la inyección de toxina botulínica en el músculo recto superior en pacientes con retracción palpebral moderada y grave en el curso de la orbitopatía tiroidea; determinar la influencia de algunos factores relacionados con estos e identificar las complicaciones y las reacciones adversas asociadas a su uso terapéutico. Métodos: se realizó un estudio descriptivo prospectivo en el Servicio de Oftalmología del Hospital Hermanos Ameijeiras en una serie de 21 casos con retracción palpebral moderada y grave en el curso de la orbitopatía tiroidea. La inyección de toxina botulínica en el músculo recto superior para la corrección de la retracción de la orbitopatía tiroidea se realiza por primera vez en el país. Resultados: fueron satisfactorios en el 66,7 por ciento de los casos. La edad y la gravedad clínica de la retracción fueron los factores que influyeron sobre la respuesta terapéutica (p= 0,013 y p= 0,015 respectivamente). Conclusiones: la inyección de toxina botulínica en el músculo recto superior es efectiva en la mayoría de los casos tratados, sobre todo en adultos jóvenes y con menor gravedad clínica de la retracción. La hipercorrección es la complicación más temida de este procedimiento(AU)

Objective: to evaluate the therapeutic results of the botulinum toxin injection in the upper rectus muscle in patients with moderate and severe eyelid retraction in the course of thyroid orbitopathy, and to determine the influence of some factors related to these patients and to identify the complications and adverse reactions associated to its therapeutic use. Methods: prospective and descriptive study was carried out at the ophthalmological service of Hermanos Ameijeiras hospital in a 21 case series study with moderate and severe eyelid retraction in the course of thyroid orbitopathy. The injection of botulinum toxin into the upper rectus muscle for the correction of retraction in thyroid orbitopathy was performed for the first time in the country. Results: in this group, 66.7 percent of patients had satisfactory results. Age and clinical severity of retraction were the factors having influence on the therapeutic response (p= 0.013 and p= 0.015 respectively). Conclusions: the botulinum toxin injection into the upper rectus muscle is effective in most of treated cases, mainly in young adults with less clinical retraction severity. Hypercorrection is the most fearful complication in this procedure(AU)

The therapeutic outcome to intravenous steroid therapy for active Graves' orbitopathy is influenced by the time of response but not polymorphisms of the glucocorticoid receptor.

BACKGROUND: Glucocorticoids are the mainstay of immunosuppression for active moderate-severe Graves' orbitopathy (GO). AIM: To analyze the response to therapy and the contribution of glucocorticoid receptor (GR) gene polymorphisms to the therapeutic outcome of intravenous glucocorticoids (IVGC) in active moderate-severe GO. METHODS: we have studied 58 patients treated with 7.5 g i.v. methylprednisolone (cumulative dose). ophthalmological assessment was performed at baseline and at 6-8, 12-16, and 24-30 weeks after the first infusion. Three GR gene polymorphisms, ER22/23EK, N363S, and BCL1, which have been associated to variable sensitivity to steroids, were studied in 43/58 patients. The therapeutic outcomes defined as: i) reduction of the clinical activity score (CAS) ≥2 points or ii) reduction of proptosis ≥2 mm or iii) improvement of diplopia according to the Gorman score were also studied in relation to treatment schedule, age, gender, duration of thyroid or GO, smoking habits, and serum TSH-receptor autoantibodies levels. RESULTS: In total, 70% of patients responded and had GO inactivation (CAS <4) as early as 6-8 weeks. At 12-16 weeks, the proportion of patients who became inactive increased by another 10% up to a total of 80%. ER22/23EK and N363S polymorphisms were present only in about 7%, while the Bcl1 variant was present in 30% of patients; no significant association of any of the GR polymorphisms with either the therapeutic response or the occurrence of side effects was observed. CONCLUSIONS: Most patients with active GO respond to IVGC as early as 6-8 weeks of therapy and the analyzed GR polymorphisms do not influence the therapeutic effect of steroids. Questions arise about the need of continuing therapy up to 12 weeks in nonresponders. We suggest that these patients may be switched to other treatments alone or in combination with steroids.

[Update on endocrine orbitopathy]. / Update endokrine Orbitopathie.

Graves' orbitopathy is an autoimmune disease of the ocular adnex connective tissue and most commonly occurs together with Grave's hyperthyroidism. Anti-TSH receptor antibodies are specific for Graves' disease and are related to both the course of thyroid and orbital diseases. An active inflammatory disease stage is followed by an inactive stage of incomplete remission in most patients. Periorbital swelling, proptosis, diplopia and lid retraction severely impair the patients' quality of life. In the active state anti-inflammatory treatment consists of i.v. steroids, off-label use of immunomodulatory medication, selenium and in emergency cases orbital decompression. Fortunately, defects in inactive stable Graves' orbitopathy can be successfully treated by surgery and involve decompression for proptosis reduction, muscle recession to correct diplopia and (finally) lid surgery.

[Rapidly progressing unilateral proptosis in a 6-year-old girl]. / Rasch fortschreitende unilaterale Proptosis bei einem 6-jährigen Mädchen.

This report describes the case of a 6-year-old girl who presented with painless swelling of the right orbit since 4 weeks and moderate proptosis. Tests revealed visual acuity RE 6/6, LE 6/6, normal intraocular pressure (IOP), anterior and posterior segments normal. Ultrasound examination showed multiple lesions in the spleen, normal liver, no abdominal mass and enlarged abdominal lymph nodes. Fine-needle aspirate results were not available at the time of clinical decision-making. In Malawi the treatment for all stages of Burkitt's lymphoma is intravenous cyclophosphamide (40 mg/kg on day 1 and oral cyclophosphamide 60 mg/kg on days 8, 18 and 28). Intrathecal hydrocortisone (12.5 mg) and methotrexate (12.5 mg) are given with each treatment cycle.

Time course of Graves' ophthalmopathy after total thyroidectomy alone or followed by radioiodine therapy: a 2-year longitudinal study.

The findings in hyperthyroid patients with Graves' orbitopathy (GO) of antibodies against antigens shared between the thyroid and orbit, such as the TSH-receptor (TRAb) and a novel protein G2s (G2sAb), suggested a possible common therapeutic strategy. However, the gold therapeutic standard for hyperthyrodism in these patients remains still unsettled and is mainly based on personal experience. Studies on the effect of total thyroidectomy (TT) alone or followed by radioiodine ablation (RAI) of thyroid remnants showed often conflicting results. This longitudinal study was aimed at evaluating the influence of TT alone or followed by post-surgical RAI with respect to methimazole treatment on the activity and severity of GO in patients with hyperthyroidism and GO. Sixty consecutive patients with Graves' disease and mild/moderate GO were studied and grouped as follows: group 1, including 25 patients (16F, 9M) undergoing TT alone; group 2, including 10 patients (8F, 2M) undergoing TT followed by RAI for histological evidence of differentiated thyroid cancer; group 3, including 25 patients (18F, 7M) euthyroid under methimazole therapy, studied as controls. Clinical study of ophthalmopathy and measurements of TRAb and G2sAb were performed in all patients at start of the study (time of TT for group 1 and RAI after TT for group 2 and of the first finding of euthyroidism under methimazole treatment for group 3) and after 6, 12, 24 months. Patients of both groups 1 and 2 showed an early significant decrease and a further progressive reduction of the activity and severity of GO with a disappearance of TRAb and a decrease of G2sAb levels during the follow-up, without statistically significant differences between the two groups. Patients in group 3 showed a much later and less marked improvement of GO with persistence of TRAb and G2sAb positivity, even if with reduction of TRAb levels at 12 and 24 months. Our results suggest that in Graves' patients with large goiter or relapse of hyperthyroidism and mild/moderate GO, TT alone could be an advisable choice to treat hyperthyroidism also improving GO with reduction of cost/benefit ratio.

[Results of treatment of orbital fractures with polydioxanone sheet]. / Ocena wyników leczenia zlaman oczodolów z uzyciem folii polidioksanowej.

UNLABELLED: Orbital fractures are one of the most frequent consequences following midfacial trauma. If not treated they can lead to serious optical complications as: double vision (diplopia), restriction of ocular motility, eyeball sinking (enophthalmos). Autogenic bone grafts although still wide and effectively used for reconstruction of the fractured orbital walls present some disadvantages. This is morbidity of the donor site, unforeseen resorption, time-consuming procedure and longer postoperative care. Because of that introduction of new materials for orbital reconstruction seems to be desirable. The aim of the work was to evaluate usefulness of the polydioxanone sheets (PDS) as a reconstructive material for orbital floor fractures. MATERIAL AND METHODS: From 1. 09. 2004 to 1. 02. 2006 111 patients with orbital fractures were treated in the Department of Maxillofacial Surgery Klinikum Minden. Age of the patients was between 15- 89 years (mean 43 y.); m:w ratio 78:33. There were 54 isolated orbital floor fractures (in 3 cases with additional medial wall fracture) and 57 zygomatico-orbito-maxillary fractures. The diagnosis was based on the clinical picture and coronal CT scans. The patients were operated through a transconjunctival (72%) or an infraorbital-Mustarde (28%) access and the orbital floor (medial orbital wall) was reconstructed with PDS sheet. Control examination was performed immediately, 1 moth and 6 months after the operative treatment. RESULTS: There were 47% true "blow-out", 34% "trap-door" and 19% "en-clapet" fractures. The most important preoperative symptom was double vision (23%), restriction of ocular motility (18%), enophthalmos (3,6%), impairment of function of the infraorbital nerve (41%). Size of the defect was 3+/-1,13 cm(2) in "blow out" fracture, 1,8 +/-0,9 cm(2) in "trap-door" and 2+/-0,5 cm(2) in ,"en-clapet" ones. The primary reconstruction was successful in 97,3% of the cases. Any inflammation or reaction against implant was noted. Persistent double vision was present in 2,7%, restriction of ocular motility in 1,8% and enophthalmos in 0,9% after the primary procedure. These patients were submitted to reoperation. In 1 case badly positioned PDS sheet causing diplopia was removed. In another one, eyeball movement restriction was due to adhesions between the sheet and periorbital tissue. Visual status of this patient has improved after adhesiolysis. In 1 patient with a large bony destruction reconstruction only with PDS sheet was an inadequate treatment. In 7,2% of patients disturbances in the field of innervation of the infraorbital nerve were present 6 months after the surgery. CONCLUSIONS: Alloplastic, resorbable PDS sheets in most cases were a valuable material for the reconstruction of the orbital floor (medial orbital wall). Mechanical properties of PDS seem to be not sufficient for the reconstruction of extremely large bony defects. In these cases use of autogenic bone grafts or a titanium mesh should be rather considered.

Lateral tarsorrhaphy for prevention of postoperative complications resulting from globe luxation.

We present a case of spontaneous globe luxation and wound dehiscence that developed in a patient with proptosis 1 day after phacoemulsification with posterior chamber intraocular lens implantation. The wound was repaired and a lateral tarsorrhaphy was performed with successful prevention of further luxations. Upon cataract extraction in the patient's other eye, lateral tarsorrhaphy was performed concurrently to prevent operative complications. This report underscores the importance of lateral tarsorrhaphy performed at the time of cataract surgery to avoid postoperative globe luxation with possible wound dehiscence in patients with proptosis or shallow orbits and a history of luxation.

Orbital floor reconstruction with flexible Ethisorb patches: a retrospective long-term follow-up study.

OBJECTIVE: The purpose of the study was to investigate whether a flexible, biodegradable material (Ethisorb) shows better long-term results with regard to diplopia, bulbus motility, and exophthalmos/enophthalmos compared to the use of lyophilized dura-patches and polydioxanone (PDS) foils. METHODS: During a period of 6 years 435 patients with an orbital fracture were investigated retrospectively. Inclusion criteria were patients with fractures of the orbital floor with a maximum size of 2 x 2 cm. Bulbus motility, exophthalmos, enophthalmos, and diplopia were investigated during a period of 2 years. RESULTS: One hundred twenty orbital floors were reconstructed by lyophilized dura-patches, 81 by PDS, and 136 by Ethisorb. An exploration without an implantation was performed in 91 patients. The long-term investigation 12 to 15 months after surgery showed an exophthalmos and enophthalmos incidence of 1%, whereas a reduced bulbus motility and diplopia were found in 5% and 4%, respectively. Fifteen to 24 months after surgery 2% of the patients had an exophthalmos and 1% had an enophthalmos. A reduction of bulbus motility was found in 4% of the patients, and diplopia was found in 3%. The use of Ethisorb resulted in a significantly lower incidence of exophthalmos 3 months after surgery compared to PDS. CONCLUSION: The low rate of acquired bulbus motility demonstrates acceptable results in using Ethisorb in the floor of the orbit.

Relation between therapy for hyperthyroidism and the course of Graves' ophthalmopathy.

BACKGROUND: The chief clinical characteristics of Graves' disease are hyperthyroidism and ophthalmopathy. The relation between the two and the effect of treatment for hyperthyroidism on ophthalmopathy are unclear. METHODS: We studied 443 patients with Graves' hyperthyroidism and slight or no ophthalmopathy who were randomly assigned to receive radioiodine, radioiodine followed by a 3-month course of prednisone, or methimazole for 18 months. The patients were evaluated for changes in the function and appearance of the thyroid and progression of ophthalmopathy at intervals of 1 to 2 months for 12 months. Hypothyroidism and persistent nyperthyroiaism were promptly corrected. RESULTS: Among the 150 patients treated with radioiodine, ophthalmopathy developed or worsened in 23 (15 percent) two to six months after treatment. The change was transient in 15 patients, but it persisted in 8 (5 percent), who subsequently required treatment for their eye disease. None of the 55 other patients in this group who had ophthalmopathy at base line had improvement in their eye disease. Among the 145 patients treated with radioiodine and prednisone, 50 (67 percent) of the 75 with ophthalmopathy at base line had improvement, and no patient had progression. The effects of radioiodine on thyroid function were similar in these two groups. Among the 148 patients treated with methimazole, 3 (2 percent) who had ophthalmopathy at base line improved, 4 (3 percent) had worsening of eye disease, and the remaining 141 had no change. CONCLUSIONS: Radioiodine therapy for Graves' hyperthyroidism is followed by the appearance or worsening of ophthalmopathy more often than is therapy with methimazole. Worsening of ophthalmopathy after radioiodine therapy is often transient and can be prevented by the administration of prednisone.

Does early administration of thyroxine reduce the development of Graves' ophthalmopathy after radioiodine treatment?

The roles of thyroid hormones and thyrotropin (TSH) in the development of Graves' ophthalmopathy are not clear. Some studies suggest a protective effect of thyroid hormones on experimental exophthalmos and an adverse effect of increased TSH levels. In September 1988 we introduced early thyroxine (T4) administration after 131I therapy for hyperthyroidism caused by Graves' disease. We carried out a retrospective study of records from all patients with this disease treated with 131I for 4 years. During the first 2 years 248 patients were treated (group A). They received T4 when the serum concentration of TSH and/or T4 indicated hypothyroidism. During the next 2 years 244 patients were treated (group B). They were all given 0.05 mg of T4 daily, starting 2 weeks after therapy, and 0.1 mg after a further 2 weeks. With a follow-up of 18 months, 45 patients (18%) in group A and 27 patients (11%) in group B developed or deteriorated in an already present ophthalmopathy (p = 0.03, relative risk = 1.64, 95% confidence interval = 1.05-2.55). Twenty-six patients in group A required specific therapy for the ophthalmopathy (e.g. antithyroid drugs, steroids, etc.) compared to 11 patients in group B (p = 0.02, relative risk = 2.33; 95% confidence interval = 1.18-4.60). Our results suggest that early administration of T4 after 131I therapy reduces the occurrence of Graves' ophthalmopathy.