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Journal of the College of Physicians... May 2021Orbital teratoma is a rare tumour that is composed of tissues derived from all three germ cell layers. It presents, in otherwise, healthy neonates with unilateral...
Orbital teratoma is a rare tumour that is composed of tissues derived from all three germ cell layers. It presents, in otherwise, healthy neonates with unilateral proptosis that progresses rapidly, threatening vision through optic nerve compromise or exposure keratopathy. Globe conservation may be difficult too in advanced cases. Mature teratomas are benign, but the immature ones can sometimes become malignant. We report a case of a six-month female infant who presented with mature orbital teratoma since birth. Vision could not be salvaged; but following complete surgical excision, good cosmesis was achieved. Key Words: Mature teratoma, Orbit, Congenital tumour, Surger.
Topics: Dermoid Cyst; Exophthalmos; Female; Humans; Infant; Infant, Newborn; Orbit; Orbital Neoplasms; Teratoma
PubMed: 34027878
DOI: 10.29271/jcpsp.2021.05.596 -
Hormones (Athens, Greece) Jun 2021The successful treatment of Graves' ophthalmopathy (GO) remains a challenge, while the efficacy of rituximab (RTX) is at present controversial. The aim of this... (Meta-Analysis)
Meta-Analysis Review
AIM
The successful treatment of Graves' ophthalmopathy (GO) remains a challenge, while the efficacy of rituximab (RTX) is at present controversial. The aim of this meta-analysis was to investigate the potential impact of intravenous RTX therapy in patients with GO.
METHODS
We performed a search in the PubMed, Embase, and Web of Science databases for relevant studies published before July 2020. The primary outcome was the change of clinical activity score (CAS), and secondary outcomes were the change of proptosis and TSH receptor antibodies (TRAb). A meta-analysis was conducted to calculate the standard mean difference (SMD) for these outcomes by using fixed- or random-effect models.
RESULTS
Analysis of outcomes in 152 patients collected from 12 published articles was conducted. Compared to baseline value, CAS was significantly decreased at 1, 6, 12, and >12 months after RTX treatment. For proptosis, the results revealed no significant decrease at 1-3, 6, and ≥12 months. Moreover, the pooled analysis employed in this meta-analysis showed no significant difference of TRAb at 1 month, but significant declines were observed at 6 and ≥12 months.
CONCLUSION
Our results strongly suggest that intravenous RTX treatment has an acute and long-lasting beneficial effect on decreasing both CAS and TRAb. The study also indicates that the effect of RTX on proptosis is limited. There is evidently a need to investigate the mechanism behind RTX ineffectiveness on proptosis and explore other therapeutic regimens for the reduction of proptosis.
Topics: Exophthalmos; Graves Ophthalmopathy; Humans; Immunologic Factors; Rituximab
PubMed: 33783712
DOI: 10.1007/s42000-021-00282-6 -
Ophthalmic Plastic and Reconstructive...A 34-year-old primigravida woman at 26-week gestation presented with gradually progressive right eye proptosis since the early stage of pregnancy. On the first...
A 34-year-old primigravida woman at 26-week gestation presented with gradually progressive right eye proptosis since the early stage of pregnancy. On the first examination, the right eye had 7 mm proptosis, compared with the left eye. Magnetic resonance images demonstrated enlarged inferior and medial recti muscles with tendon involvement. The results of pathological examination of a specimen harvested from the medial rectus muscle lesion 1 month after the delivery corresponded to an intramuscular cavernous hemangioma. Three months after biopsy, the right eye had a 3-mm reduction in proptosis, and magnetic resonance images showed slight reduction in size of the medial rectus muscle lesion. The maximum diameter of the right medial rectus muscle measured on axial images decreased from 13.13 to 9.38 mm. The patient underwent balanced orbital decompression 8 months after the biopsy. At 9-month post-decompression period, the right eye had 3.5-mm proptosis, and the vision was stable.
Topics: Pregnancy; Female; Humans; Adult; Oculomotor Muscles; Exophthalmos; Hemangioma; Magnetic Resonance Imaging; Biopsy
PubMed: 35792735
DOI: 10.1097/IOP.0000000000002237 -
Archives of Endocrinology and Metabolism Apr 2022Graves' ophthalmopathy (GO) is a vision-threatening finding observed in approximately half of Graves' disease patients. The pathophysiology of GO is unclear, and one of...
OBJECTIVE
Graves' ophthalmopathy (GO) is a vision-threatening finding observed in approximately half of Graves' disease patients. The pathophysiology of GO is unclear, and one of the suspected factors is oxidative stress. In our study, we compared the relationship between proptosis and SH-SS in patients diagnosed with GO.
METHODS
In this prospective study, 40 recently diagnosed Graves' disease patients with proptosis, 40 recently diagnosed Graves' disease patients without GO and 30 healthy individuals with similar demographic characteristics were included. Serum thiol-disulfide (SH-SS) measurements were performed. Eye examinations were performed by a single ophthalmologist to check for the presence of GO, and proptosis values were recorded with a Hertel exophthalmometer.
RESULTS
Total SH values were lower in the group with proptosis than in the other groups (p < 0.05). Total and native SH values were lower in patients without proptosis than in the control group (p < 0.05). Total SH, native SH and SS levels were independently associated with proptosis (p < 0.05). According to this analysis, it was found that increasing SS and decreasing total and native SH levels increased the probability of proptosis by 24.4%, 32.7% and 32.4%, respectively.
CONCLUSION
A decrease in SH, which is a natural antioxidant that protects the body against oxidative stress, and an increase in SS are important signs of oxidative damage. Proptosis and SH-SS are closely related in GO. This may help us detect GO and proptosis in Graves' patients. It can also assist in developing new options for preventing and treating GO.
Topics: Disulfides; Exophthalmos; Graves Disease; Graves Ophthalmopathy; Humans; Prospective Studies; Sulfhydryl Compounds
PubMed: 35315985
DOI: 10.20945/2359-3997000000448 -
Ophthalmic Plastic and Reconstructive... Dec 2023Thyroid eye disease (TED) is the most common extrathyroidal manifestation of Graves disease. Patients may be severely affected with eyelid retraction, exophthalmos,... (Review)
Review
PURPOSE
Thyroid eye disease (TED) is the most common extrathyroidal manifestation of Graves disease. Patients may be severely affected with eyelid retraction, exophthalmos, diplopia, pain, and threatened vision. Autoantibodies against thyroid-stimulating hormone receptor and insulin-like growth factor 1 receptor have shown associations with pathophysiological and clinical traits. Autoantibodies against thyroid-stimulating hormone receptor is in current clinical use as biomarker, but not with unambiguous diagnostic performance. A biomarker with high diagnostic accuracy and/or prognostic capability would be of immense value in diagnosing TED, especially in subclinical cases or when TED precedes the thyroid dysfunction. This article is a literature review on molecular biomarkers of TED.
METHODS
A literature search was performed using PubMed and Embase. Studies on molecular biomarkers in blood, tear fluid, and urine were included in the review.
RESULTS
Forty-six papers were included, of which 30, 14, and 2 studies on biomarkers in blood, tears, and urine, respectively. Fourteen of the papers evaluated the diagnostic performance of various biomarkers, 12 in blood and 2 in tears. Most studies evaluated single biomarkers, but 3 tested a panel of several markers. Except for autoantibodies against thyroid-stimulating hormone receptor, the reported diagnostic performances for the biomarkers were not confirmed in independent cohorts. In 32 studies, no or insufficient performance data were given, but the findings indicated involvement of various biologic mechanisms in TED including inflammation, oxidative stress, fibrosis, lipid metabolism, and ocular surface microflora.
CONCLUSIONS
Currently, serum autoantibodies against thyroid-stimulating hormone receptor is the only molecular biomarker with clinical utility in patients with TED. Several potential biomarkers have been investigated, and particularly panels of multiple biomarkers in tears are promising. To improve patient care, biomarkers in TED should be studied further.
Topics: Humans; Graves Ophthalmopathy; Biomarkers; Graves Disease; Autoantibodies; Thyrotropin
PubMed: 38054982
DOI: 10.1097/IOP.0000000000002466 -
The Lancet. Oncology Dec 2021
Topics: Adenocarcinoma; Aged; Diplopia; Exophthalmos; Female; Humans; Pancreatic Neoplasms; Prognosis
PubMed: 34856154
DOI: 10.1016/S1470-2045(21)00592-1 -
The New England Journal of Medicine Apr 2020
Topics: Adult; Animals; Echinococcosis; Echinococcus granulosus; Exophthalmos; Female; Humans; Orbit; Orbital Diseases
PubMed: 32242360
DOI: 10.1056/NEJMicm1911903 -
Zhurnal Voprosy Neirokhirurgii Imeni N.... 2022Optic nerve glioma is a rather rare tumor. It predominantly arises in pediatric patients, including those with type I neurofibromatosis. This neoplasm is accompanied by...
UNLABELLED
Optic nerve glioma is a rather rare tumor. It predominantly arises in pediatric patients, including those with type I neurofibromatosis. This neoplasm is accompanied by decreased visual function and exophthalmos. Treatment strategy is individualized depending on age, volume and spread of tumor, as well as severity of clinical manifestations. Possible treatment options are surgical resection, chemotherapy, radiotherapy and their combination. Radiotherapy can be recommended for patients with intact visual functions, no severe proptosis and trophic lesions. Classic fractionation mode is used as a standard. Currently, the possibility of hypofractionated irradiation is being considered.
OBJECTIVE
To evaluate safety and efficacy of hypofractionated radiotherapy in patients with optic nerve glioma.
MATERIAL AND METHODS
Sixteen patients with optic nerve gliomas underwent hypofractionated stereotactic irradiation (CyberKnife) between May 2014 and October 2019. Single focal dose was 5.5 Gy. There were 5 fractions up to total focal dose of 27.5 Gy. The sample enrolled 14 children with a median age of 4 years (range 23 months - 13 years) and 2 adults aged 47 and 66 years, respectively. Median of tumor volume was 2.77 cm (range 1.69-10.01 cm).
RESULTS
Tumor growth control was achieved in all patients, partial remission was observed in 5 (32%) patients. None patient had deterioration of visual function. Improvement of visual acuity was noted in 3 (19%) cases. Visual field enlargement occurred in 4 (67%) out of 6 patients who were preoperatively examined. After irradiation, proptosis decreased by ≥ 1 mm in 9 (60%) out of 15 patients.
Topics: Adult; Child; Child, Preschool; Exophthalmos; Humans; Infant; Neoplasms; Optic Nerve Glioma; Radiation Dose Hypofractionation; Radiosurgery; Treatment Outcome
PubMed: 36252196
DOI: 10.17116/neiro20228605174 -
International Journal of Clinical... 2023In this study, we conducted a meta-analysis to assess the efficacy and safety of teprotumumab in treating thyroid eye disease. We searched the Cochrane Library, PubMed,... (Meta-Analysis)
Meta-Analysis Review
In this study, we conducted a meta-analysis to assess the efficacy and safety of teprotumumab in treating thyroid eye disease. We searched the Cochrane Library, PubMed, and Embase databases from inception to May 25, 2022, and included all randomized controlled trials. Odds ratios (ORs) were calculated using fixed- or random-effect models. A total of three studies involving 341 patients were identified. Overall, the analysis revealed that teprotumumab demonstrated superior integrated proptosis response compared to placebo in both the intention-to-treat (ITT) population (OR = 17.81, 95% CI = [10.32, 30.76], = 50%) and per-protocol population (OR = 24.53, 95% CI = [12.96, 46.45], = 14%). Furthermore, patients receiving teprotumumab showed significant improvement in overall response (OR = 8.35, 95% CI = [4.74, 14.71], = 79%), diplopia response (OR = 5.53, 95% CI = [3.24, 9.44], = 0%), and achieving a clinical activity score (CAS) of 0 or 1 (OR = 6.26, 95% CI = [3.87, 10.12], = 0%). Moreover, patients treated with teprotumumab experienced greater improvements in proptosis (MD = -2.49, 95% CI = [-2.54, -2.45], = 98%) and Graves' ophthalmopathy-specific quality of life (GO-QOL, MD = 11.48, 95% CI = [11.03, 11.93], = 95%). However, it is important to note that patients receiving teprotumumab had a higher risk of adverse events, including serious adverse events, gastrointestinal adverse reactions, and muscle spasms. In summary, teprotumumab demonstrated greater improvement in proptosis response, proptosis, diplopia response, overall response, GO-QOL, and CAS. Nonetheless, it should be considered that its use is associated with a higher risk of adverse events.
Topics: Humans; Graves Ophthalmopathy; Quality of Life; Diplopia; Randomized Controlled Trials as Topic; Exophthalmos
PubMed: 37588100
DOI: 10.1155/2023/6638089 -
Neuroimaging Clinics of North America Aug 2021Graves disease is an autoimmune disorder caused by the breakdown of immune tolerance to thyroid antigens against the TSH receptor. In approximately 25% of patients, an... (Review)
Review
Graves disease is an autoimmune disorder caused by the breakdown of immune tolerance to thyroid antigens against the TSH receptor. In approximately 25% of patients, an inflammatory condition, Graves eye disease (GED), affects the orbital soft tissues. About 60% of patients develop mild symptoms including fat expansion and inflammation of the levator muscle complex with resultant proptosis, eyelid retraction, and exposure of the globe. The remaining patients experience enlargement of one or more of the extraocular muscles, leading to conjunctival and eyelid edema and congestion, restricted ocular movement with resultant diplopia, and optic nerve compression leading to compressive optic neuropathy.
Topics: Exophthalmos; Graves Ophthalmopathy; Humans; Oculomotor Muscles; Optic Nerve; Optic Nerve Diseases
PubMed: 34243871
DOI: 10.1016/j.nic.2021.04.006