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Child's Nervous System : ChNS :... Jul 2024Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm. (Review)
Review
INTRODUCTION
Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm.
CLINICAL PRESENTATION
Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy.
DIAGNOSIS
Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.
PubMed: 38953912
DOI: 10.1007/s00381-024-06510-9 -
CNS Neuroscience & Therapeutics Jul 2024To investigate the alterations of the optic nerve and visual cortex in dysthyroid optic neuropathy (DON), a subgroup of thyroid eye disease (TED).
AIMS
To investigate the alterations of the optic nerve and visual cortex in dysthyroid optic neuropathy (DON), a subgroup of thyroid eye disease (TED).
METHODS
Multiple orbital imaging biomarkers related to optic nerve compression and the amplitude of low-frequency fluctuations (ALFF) of the brain were obtained from 47 patients with DON, 56 TED patients without DON (nDON), and 37 healthy controls (HC). Correlation analyses and diagnostic tests were implemented.
RESULTS
Compared with HC, the nDON group showed alterations in orbital imaging biomarkers related to optic nerve compression in posterior segments, as well as ALFF of the right inferior temporal gyrus and left fusiform gyrus. DON differed from nDON group mainly in the modified muscle index of the posterior segment of optic nerve, and ALFF of orbital part of right superior frontal gyrus, right hippocampus, and right superior temporal gyrus. Orbital and brain imaging biomarkers were significantly correlated with each other. Diagnostic models attained an area under a curve of 0.80 for the detection of DON.
CONCLUSION
The combined orbital and brain imaging study revealed alterations of the visual pathway in patients with TED and DON as well as provided diagnostic value. The initiation of alterations in the visual cortex in TED may precede the onset of DON.
Topics: Humans; Male; Female; Middle Aged; Graves Ophthalmopathy; Visual Cortex; Adult; Magnetic Resonance Imaging; Optic Nerve Diseases; Orbit; Optic Nerve; Aged
PubMed: 38948947
DOI: 10.1111/cns.14820 -
NeoReviews Jul 2024
Topics: Humans; Graves Disease; Female; Pregnancy; Infant, Newborn; Pregnancy Complications; Thyroid Diseases
PubMed: 38945965
DOI: 10.1542/neo.25-7-e447 -
Journal of AAPOS : the Official... Jun 2024Thyroid eye disease (TED) can result in proptosis and ocular misalignment, leading to eye pain, diplopia, and vision loss. Teprotumumab, a humanized antibody against...
BACKGROUND
Thyroid eye disease (TED) can result in proptosis and ocular misalignment, leading to eye pain, diplopia, and vision loss. Teprotumumab, a humanized antibody against insulin-like growth factor 1 receptor, was approved in 2020 for the treatment of TED. The purpose of this study was to describe the effect of a full course of teprotumumab on ocular misalignment.
METHODS
The medical records of patients who underwent treatment with teprotumumab for active moderate-to-severe TED at a single institution from April 2020 to September 2023 were reviewed retroactively. Sensorimotor examination was performed at each visit using simultaneous prism-cover testing. Demographic information and previous history of radioactive iodine, steroids, strabismus surgery, and smoking were extracted from the record for analysis.
RESULTS
A total of 19 patients were treated during the study period, of whom 11 had strabismus and diplopia. The initial absolute horizontal misalignment in these 11 was 6.0 ± 1.5, vertical misalignment was 7.7 ± 2.4, and total misalignment was 11.5 ± 2.0. On completion of treatment, these measurements decreased by 2.0 ± 1.5, 2.2 ± 1.0, and 3.2 ± 1.6, respectively (P = 0.10, 0.02, and 0.04, resp.). Eight patients (73%) had a decrease in their strabismus, and 5 (46%) reported complete resolution of their diplopia at the final visit. No factors were predictive of which patients would have resolution of their misalignment. Of the remaining 3 patients who had no improvement in ocular alignment, 2 (66%) underwent strabismus surgery. Of the 8 patients with improvement of strabismus, only a single patient (13%) underwent strabismus surgery for persistent diplopia.
CONCLUSIONS
In our study cohort, a full course of teprotumumab coincided with complete resolution of diplopia in 46% of patients and a decrease in strabismus in 73% of patients.
PubMed: 38944235
DOI: 10.1016/j.jaapos.2024.103959 -
Scientific Reports Jun 2024This study aimed to investigate the effect of orbital wall decompression surgery and reduction of proptosis on the choroidal vascularity index (CVI) and subfoveal...
This study aimed to investigate the effect of orbital wall decompression surgery and reduction of proptosis on the choroidal vascularity index (CVI) and subfoveal choroidal thickness (SFCT) in patients with thyroid eye disease (TED). Fifty-one eyes from 38 patients with controlled TED and proptosis were enrolled in this study. The majority of the patients (50.9%) had a clinical activity score (CAS) of zero, and none had a CAS greater than 2. The patients underwent a complete baseline ophthalmologic examination, and their choroidal profile alterations were monitored using enhanced depth imaging optical coherence tomography (EDI-OCT) before and during the three months after surgery. Changes in SFCT, luminance area (LA), total choroidal area (TCA), and the choroidal vascularity index (CVI) were measured as the ratio of LA to TCA in EDI-OCT images. The participants had an average age of 46.47 years, and 22 were female (57.9%). The SFCT of the patients exhibited a significant reduction over the follow-up period, decreasing from 388 ± 103 to 355 ± 95 µm in the first month (p < 0.001) and further decreasing to 342 ± 109 µm by the third month compared to baseline (p < 0.001). The CVI exhibited a drop from 0.685 ± 0.037 at baseline to 0.682 ± 0.035 and 0.675 ± 0.030 at 1 and 3 months post-surgery, respectively. However, these changes were not statistically significant, indicating comparable decreases in both LA and TCA. There was a significant correlation between improved proptosis and reduction in SFCT (p < 0.001) but not with CVI (p = 0.171). In conclusion, during the three months of follow-up following orbital wall decompression, CVI did not change, while SFCT reduced significantly. Additionally, SFCT was significantly correlated with proptosis reduction, whereas CVI was not.
Topics: Humans; Female; Male; Middle Aged; Decompression, Surgical; Graves Ophthalmopathy; Choroid; Tomography, Optical Coherence; Adult; Orbit; Exophthalmos; Aged; Treatment Outcome
PubMed: 38942805
DOI: 10.1038/s41598-024-65884-7 -
Asian Journal of Surgery Jun 2024
PubMed: 38937230
DOI: 10.1016/j.asjsur.2024.06.040 -
Zhongguo Dang Dai Er Ke Za Zhi =... Jun 2024To investigate the expression of microRNA-142 (miR-142) in children with autoimmune thyroid disease (AITD) and its relationship with the imbalance of helper T cell 17...
OBJECTIVES
To investigate the expression of microRNA-142 (miR-142) in children with autoimmune thyroid disease (AITD) and its relationship with the imbalance of helper T cell 17 (Th17) and regulatory T cell (Treg).
METHODS
A total of 89 children hospitalized for AITD from January 2019 to December 2022 were prospectively selected as the study subjects, including 48 children with Graves' disease (GD group) and 41 children with Hashimoto's thyroiditis (HT group). Additionally, 55 healthy children undergoing physical examinations during the same period were selected as the control group. The differences in serum miR-142, antithyroglobulin antibody (TGAb), antithyroperoxidase antibody (TPOAb), Th17/Treg, and interleukin-17 (IL-17) expression were compared among the groups.
RESULTS
The expression of miR-142, TPOAb, TGAb, Th17, Th17/Treg, and IL-17 in the GD group and HT group was higher than that in the control group, while Treg was lower than that in the control group (<0.05). Pearson correlation analysis revealed that in the GD group, miR-142 was positively correlated with TPOAb, TGAb, Th17, Th17/Treg, and IL-17 (=0.711, 0.728, 0.785, 0.716, 0.709, respectively; <0.001) and negatively correlated with Treg (=-0.725, <0.001); in the HT group, miR-142 was positively correlated with TPOAb and TGAb (=0.752, 0.717, respectively; <0.001).
CONCLUSIONS
miR-142 is highly expressed in children with AITD, and its expression may be related to the Th17/Treg imbalance in children with GD.
Topics: Humans; MicroRNAs; Th17 Cells; Child; Male; Female; T-Lymphocytes, Regulatory; Interleukin-17; Hashimoto Disease; Child, Preschool; Graves Disease; Adolescent; Autoantibodies
PubMed: 38926377
DOI: 10.7499/j.issn.1008-8830.2312017 -
BMJ Case Reports Jun 2024A patient in his 20s presented with a change in the appearance of his left eye with evidence of relative afferent pupillary defect. Imaging revealed a giant...
A patient in his 20s presented with a change in the appearance of his left eye with evidence of relative afferent pupillary defect. Imaging revealed a giant frontoethmoidal osteoma, a benign sinonasal tumour, invading three-quarters of the orbit. Multidisciplinary discussion involving opthalmology, maxillofacial surgery, neurosurgery and otolaryngology resulted in the decision to attempt entirely endoscopic excision of this lesion, which was performed with successful outcomes. This case demonstrates how a sinonasal osteoma should be considered in the differential diagnosis for a patient presenting with proptosis or other eye signs suggestive of compression of the orbital compartment. This case report and literature review highlights the possibility of managing giant sinonasal osteomas with orbital extension through a completely endoscopic approach.
Topics: Humans; Osteoma; Male; Endoscopy; Paranasal Sinus Neoplasms; Ethmoid Bone; Orbital Neoplasms; Ethmoid Sinus; Orbit; Frontal Sinus; Tomography, X-Ray Computed; Young Adult; Exophthalmos; Diagnosis, Differential; Adult
PubMed: 38926120
DOI: 10.1136/bcr-2023-259236 -
Die Ophthalmologie Jun 2024Endocrine orbitopathy (EO) is an autoimmune disease mostly associated with a disease of the thyroid gland, which leads to inflammation, adipogenesis and fibrosis. The... (Review)
Review
BACKGROUND
Endocrine orbitopathy (EO) is an autoimmune disease mostly associated with a disease of the thyroid gland, which leads to inflammation, adipogenesis and fibrosis. The severity of EO can vary greatly between individuals, which makes it difficult to exactly predict the natural course of the disease; however, this is important to be able to individually adapt the treatment. The aim of this study was to compare the clinical features, course, treatment and prognosis for patients with EO under 50 years old with older patients. The results of the study with a focus on motility are presented in this special issue.
PATIENTS AND METHODS
The hospital records of a randomly selected sample of 1000 patients from the EO databank in Essen (GODE), which includes 4260 patients, were analyzed. The patients were divided into two groups: group 1 ≤50 years and group 2 >50 years. Only patients with complete data sets were included in the statistical analyses.
RESULTS
Younger patients (n = 484) presented significantly more frequently with milder EO (53% vs. 33%, p < 0.0001), whereas older patients (n = 448) more frequently suffered from moderate or severe forms (44% vs. 64%, p < 0.0001). Older patients showed more severe strabismus, motility and clinical activity scores (5.9 vs. 2.3 prism diopters, PD/310° vs. 330°, both p < 0.0001, CAS 2.1 vs. 1.7, p = 0.001). Proptosis and the occurrence of optic nerve compression showed no significant differences between the groups (3% each). Multiple logistic regression showed that the necessity for a second eye muscle surgery was most strongly associated with a previous decompression (OR = 0.12, 95 % CI 0.1-0.2, p < 0.0001), followed by orbital irradiation and age.
CONCLUSION
In summary, younger patients with EO presented with milder clinical features, such as a lower rate of restrictive motility disorders and weaker expression of signs of inflammation. Therefore, older patients needed steroids, irradiation, eyelid and eye muscle surgery more frequently; however, the risk of dysthyroid optic neuropathy and the necessity of a second eye surgery were not or only slightly associated with age.
PubMed: 38922402
DOI: 10.1007/s00347-024-02073-x -
Indian Journal of Thoracic and... Jul 2024Extracorporeal membrane oxygenation (ECMO) plays a crucial role in managing heart and respiratory failure. However, it is associated with complications such as bleeding,...
Extracorporeal membrane oxygenation (ECMO) plays a crucial role in managing heart and respiratory failure. However, it is associated with complications such as bleeding, acute renal failure, neurological issues, and thrombosis. Cerebral venous sinuses thrombosis (CVST) is a rare condition that primarily affects young females, especially those of childbearing age. CVST can result from multiple factors, both congenital and acquired, such as pregnancy, dehydration, oral contraceptives, and the postpartum period. The most common and nonspecific symptom is headache. Notably, the occurrence of CVST during ECMO has not been documented in adult patients. In this case report, we present the case of a 29-year-old lactating mother who was admitted to our hospital with complaints and clinical features indicative of community-acquired pneumonia. Despite conventional treatment, her oxygen requirement increased; therefore, she was placed on veno-venous ECMO (VV ECMO). Her condition gradually improved, and she was extubated while still on ECMO support (Awake ECMO). However, a day later of successful weaning from ECMO, she reported blurred vision and proptosis. Magnetic resonance imaging (MRI) and MR venography (MRV) of the brain showed features suggestive of CVST. She was promptly treated with anticoagulation and later discharged with minimal residual complications. This paper emphasizes the importance of early clinical recognition and treatment with anticoagulants in cases of CVST during ECMO for improved outcomes.
PubMed: 38919201
DOI: 10.1007/s12055-023-01672-8