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Orbital plasmacytoma is a rare plasma cell tumor that may arise as an aggressive form of extramedullary multiple myeloma. Treatment modalities include surgical excision, radiation, and chemotherapy. Chimeric antigen receptor T cell therapy is currently reserved for refractory disease. The authors present a case of a 69-year-old woman with an extensive orbital plasmacytoma refractory to multimodal therapy who was treated with idecabtagene vicleucel chimeric antigen receptor T cell therapy. Four days after infusion, the patient exhibited grade 1 cytokine release syndrome, which resolved with tocilizumab. The orbital plasmacytoma significantly decreased in size 1 month after treatment and demonstrated complete serological response and sustained tumor burden reduction at 10-month follow-up. This case highlights the efficacy of chimeric antigen receptor T cell therapy for refractory orbital plasmacytoma and calls attention to potential inflammatory toxicities.
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Neoplasias Orbitárias , Plasmocitoma , Receptores de Antígenos Quiméricos , Humanos , Idoso , Feminino , Neoplasias Orbitárias/terapia , Plasmocitoma/terapia , Plasmocitoma/diagnóstico , Receptores de Antígenos Quiméricos/uso terapêutico , Imunoterapia Adotiva/métodosRESUMO
PURPOSE: To describe a modified approach for full-thickness lower eyelid defect reconstruction. METHODS: This is a retrospective review of 5 patients with large full-thickness lower eyelid defects after tumor resection requiring reconstruction. For these lower eyelid defects, a lateral-based or superior-based tarsoconjunctival pedicle flap from the upper eyelid was used to rebuild the posterior lamella. In all cases, the anterior lamella was supplied from an adjacent myocutaneous flap in a bucket handle configuration. Data collection included tumor type and location, size of eyelid defect, presence of canalicular involvement, postoperative eyelid position, patient satisfaction, and complication rates. RESULTS: Five eyelids of 5 patients underwent Mohs micrographic excision of basal cell carcinoma followed by reconstruction with a bucket handle flap. Age range was 68 to 96 years old (mean of 81 y). Five patients presented with lower eyelid involvement. The defect size ranged from 80% to 100% of the eyelid. Three patients had canalicular involvement and required bicanalicular nasolacrimal stent placement. After reconstruction, all patients showed good eyelid apposition to the globe with excellent esthetic outcome. No revision procedures were done. Average follow-up time was 13 months (range of 8 to 21 mo). CONCLUSION: The bucket handle flap for full-thickness eyelid reconstruction is a simple technique that allows for preservation of all anatomical layers of the anterior lamella. This technique shows promising functional and esthetic outcomes.
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Carcinoma Basocelular , Neoplasias Palpebrais , Cirurgia de Mohs , Retalhos Cirúrgicos , Humanos , Idoso , Neoplasias Palpebrais/cirurgia , Idoso de 80 Anos ou mais , Estudos Retrospectivos , Masculino , Feminino , Carcinoma Basocelular/cirurgia , Resultado do Tratamento , Procedimentos de Cirurgia Plástica/métodos , Blefaroplastia/métodos , Pálpebras/cirurgia , Satisfação do PacienteRESUMO
PURPOSE: To assess whether transcutaneous retrobulbar amphotericin B injections (TRAMB) reduce exenteration rate without increasing mortality in rhino-orbital-cerebral mucormycosis (ROCM). METHODS: In this retrospective case-control study, 46 patients (51 eyes) with biopsy-proven ROCM were evaluated at 9 tertiary care institutions from 1998 to 2021. Patients were stratified by radiographic evidence of local orbital versus extensive involvement at presentation. Extensive involvement was defined by MRI or CT evidence of abnormal or loss of contrast enhancement of the orbital apex with or without cavernous sinus, bilateral orbital, or intracranial extension. Cases (+TRAMB) received TRAMB as adjunctive therapy while controls (-TRAMB) did not. Patient survival, globe survival, and vision/motility loss were compared between +TRAMB and -TRAMB groups. A generalized linear mixed effects model including demographic and clinical covariates was used to evaluate the impact of TRAMB on orbital exenteration and disease-specific mortality. RESULTS: Among eyes with local orbital involvement, exenteration was significantly lower in the +TRAMB group (1/8) versus -TRAMB (8/14) (p = 0.04). No significant difference in mortality was observed between the ±TRAMB groups. Among eyes with extensive involvement, there was no significant difference in exenteration or mortality rates between the ±TRAMB groups. Across all eyes, the number of TRAMB injections correlated with a statistically significant decreased rate of exenteration (p = 0.048); there was no correlation with mortality. CONCLUSIONS: Patients with ROCM with local orbital involvement treated with adjunctive TRAMB demonstrated a lower exenteration rate and no increased risk of mortality. For extensive involvement, adjunctive TRAMB does not improve or worsen these outcomes.
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Oftalmopatias , Mucormicose , Doenças Orbitárias , Humanos , Anfotericina B/uso terapêutico , Antifúngicos/uso terapêutico , Mucormicose/diagnóstico por imagem , Mucormicose/tratamento farmacológico , Estudos Retrospectivos , Estudos de Casos e Controles , Doenças Orbitárias/diagnóstico por imagem , Doenças Orbitárias/tratamento farmacológico , Oftalmopatias/tratamento farmacológicoRESUMO
PURPOSE: Teprotumumab, an insulin-like growth factor 1 receptor monoclonal antibody, is FDA-approved to treat thyroid eye disease (TED). The initial clinical trials excluded patients with previous orbital irradiation, surgery, glucocorticoid use (cumulative dose >1 gm), or prior biologic treatment. Information on the use of teprotumumab for patients who failed prior therapy is limited. Our purpose is to characterize the efficacy of teprotumumab for the treatment of recalcitrant TED. METHODS: This is a multicenter retrospective study of all patients treated with teprotumumab for moderate-to-severe TED after failing conventional therapy with corticosteroids, orbital radiation, surgical decompression, biologics, or other steroid-sparing medications. Treatment failure was defined as an incomplete response to or reactivation after previous treatment. Only patients who received at least 4 infusions of teprotumumab were included in the analysis. Primary outcome measures comprised proptosis response (≥2 mm reduction in the study eye without a similar increase in the other eye), clinical activity score (CAS) response (≥2-point reduction in CAS), and diplopia response (≥1 point improvement in Gorman diplopia score in patients with baseline diplopia) following treatment. Adverse events and risk factors for recalcitrant disease were also evaluated. RESULTS: Sixty-six patients were included in this study, 46 females and 20 males. Average age was 59.3 years (range 29-93). The mean duration of disease from TED diagnosis to first infusion was 57.8 months. The proptosis, CAS, and diplopia responses in this recalcitrant patient population were 85.9%, 93.8%, and 69.1%, respectively. Patients experienced a mean reduction in proptosis of 3.1 ± 2.4 mm and a mean improvement in CAS of 3.8 ± 1.6. Patients who underwent prior decompression surgery experienced a statistically significant decrease in diplopia response (46.7% vs. 77.5%, p = 0.014) and proptosis response (75.0% vs. 90.9%, p = 0.045) when compared with nondecompression patients. Additionally, there were no significant differences in proptosis, CAS, and diplopia responses between patients with acute (defined as disease duration <1 year) versus chronic (disease duration ≥1 year) TED. While most adverse events were mild to moderate, 4 patients reported serious adverse events related to persistent hearing loss. CONCLUSIONS: Patients with recalcitrant TED demonstrated a significant improvement after teprotumumab in each of the primary study outcomes. The degree of proptosis reduction, diplopia response, and CAS improvement in the recalcitrant group were similar to those of treatment-naïve patients from the pivotal clinical trials. Patients with a prior history of orbital decompression, however, demonstrated poor improvement in diplopia and less reduction in proptosis than surgery naïve patients. These results indicate that teprotumumab is a treatment option for the treatment of patients with TED recalcitrant to prior medical therapies.
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PURPOSE: To describe the use of a lateral wall implant as an adjunct in lateral orbital wall decompression in severe thyroid eye disease. METHODS: This study is a retrospective review of 6 patients who underwent prior orbital decompression but had persistent proptosis. These patients underwent lateral wall decompression with adjunct lateral wall implant placement with a manually vaulted 0.6-mm polyethylene-coated titanium mesh implant. Data collection included: visual acuity, intraocular pressure, exophthalmometry, ocular motility, eyelid position, and complication rates. RESULTS: Eight orbits in 6 patients underwent maximal lateral wall decompression and reconstruction using the polyethylene-coated titanium implant. Four males and 2 females were included with ages ranging from 25 to 73 years. Visual acuity improved an average of 2.4 lines (range 0-5 lines). Intraocular pressure improved an average of 7.5 mm Hg (2-13 mm Hg). There was reduction of proptosis by 3.4 mm on average (1-7 mm). Upper eyelid retraction improved on average by 1.8 mm (0-5 mm). Horizontal eye movements improved by 11% on average (-3.1% to +25%). Excellent cosmesis was achieved with no visible temple deformity, trismus, conjunctival scarring, orbital hemorrhage, or vision loss. CONCLUSIONS: The amount of volume created in lateral wall decompression is limited by the amount of native bone present and the temporalis muscle. In severe or recalcitrant cases, the authors propose the placement of a lateral wall implant as an adjunct to laterally displace the temporalis muscle and create additional volume. This technique accomplishes further reduction of proptosis in patients who have undergone prior orbital decompression.
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Exoftalmia , Oftalmopatia de Graves , Adulto , Idoso , Descompressão Cirúrgica/métodos , Exoftalmia/etiologia , Feminino , Oftalmopatia de Graves/complicações , Oftalmopatia de Graves/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Órbita/cirurgia , Polietileno , Estudos Retrospectivos , Titânio , Resultado do TratamentoRESUMO
Adenoid cystic carcinoma of the lacrimal gland is an aggressive, malignant epithelial neoplasm. We report the case of a 30-year-old male with lacrimal gland adenoid cystic carcinoma treated with neoadjuvant intra-arterial chemotherapy through the internal carotid artery, followed by orbital exenteration and chemoradiation. Treatment response was evaluated using a novel combination of pre- and posttreatment genome sequencing coupled with immunohistochemical evaluation, which showed diffuse tumor apoptosis. A posttreatment decrease in variant allele frequency of the NOTCH1 mutation, and robust tumor cytoreduction on imaging, supports exploration of NOTCH1 analysis as a potential marker of cisplatin sensitivity. The use of genome sequencing and immunohistochemical evaluation could provide a more targeted therapeutic assessment of neoadjuvant intra-arterial chemotherapy in the management of lacrimal gland adenoid cystic carcinoma.
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Carcinoma Adenoide Cístico , Neoplasias Oculares , Doenças do Aparelho Lacrimal , Aparelho Lacrimal , Adulto , Carcinoma Adenoide Cístico/diagnóstico , Carcinoma Adenoide Cístico/tratamento farmacológico , Carcinoma Adenoide Cístico/genética , Procedimentos Cirúrgicos de Citorredução , Neoplasias Oculares/diagnóstico , Neoplasias Oculares/tratamento farmacológico , Neoplasias Oculares/genética , Humanos , Aparelho Lacrimal/patologia , Doenças do Aparelho Lacrimal/diagnóstico , Doenças do Aparelho Lacrimal/tratamento farmacológico , Doenças do Aparelho Lacrimal/patologia , MasculinoRESUMO
PURPOSE: To identify risk factors for the development of new-onset, postoperative diplopia following orbital decompression surgery based on patient demographics, clinical exam characteristics, radiographic parameters, and surgical techniques. METHODS: We conducted a multi-center retrospective chart review of patients who underwent orbital decompression for thyroid eye disease (TED). Patient demographics, including age, gender, smoking history, preoperative exophthalmometry, clinical activity score (CAS), use of peribulbar and/or systemic steroids, and type of orbital decompression were reviewed. Postoperative diplopia was determined at a minimum of 3 months postoperatively and before any further surgeries. Cross-sectional area ratios of each extraocular muscle to orbit and total fat to orbit were calculated from coronal imaging in a standard fashion. All measurements were carried out using PACS imaging software. Multivariable logistic regression modeling was performed using Stata 14.2 (StataCorp, College Station, TX). RESULTS: A total of 331 patients without preoperative diplopia were identified. At 3 months postoperatively, 249 patients had no diplopia whereas 82 patients developed diplopia. The average postoperative follow-up was 22 months (range 3-156) months. Significant preoperative clinical risk factors for postoperative diplopia included older age at surgery, proptosis, use of peribulbar or systemic steroids, elevated clinical activity score, and presence of preoperative compressive optic neuropathy. Imaging findings of enlarged cross-sectional areas of each rectus muscle to the overall orbital area also conferred a significant risk of postoperative diplopia. Regarding surgical factors, postoperative diplopia was more common among those undergoing medial wall decompression, bilateral orbital surgery, and balanced decompression, whereas endoscopic medial wall decompression was found to be relatively protective. CONCLUSIONS: This study identifies risk factors associated with the development of diplopia following orbital decompression using multivariable data. This study demonstrates that several characteristics including age, clinical activity score, the cross-sectional muscle to orbit ratios, in addition to the type of orbital decompression surgery, are predictive factors for the development of new-onset postoperative diplopia.
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Oftalmopatia de Graves , Humanos , Oftalmopatia de Graves/diagnóstico , Oftalmopatia de Graves/cirurgia , Oftalmopatia de Graves/complicações , Estudos Retrospectivos , Descompressão Cirúrgica/efeitos adversos , Descompressão Cirúrgica/métodos , Diplopia/diagnóstico , Diplopia/etiologia , Diplopia/cirurgia , Órbita/diagnóstico por imagem , Órbita/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: Plastic surgeons are increasingly turning to social media to market their services. The newly released Twitter Academic Research Product Track (TARPT) database provides free, customizable analysis of keywords that are included in tweets on the Twitter platform. The TARPT tool may provide valuable insight into public interest in cosmetic surgery procedures. OBJECTIVES: The aim of this study was to determine TARPT's utility in tracking and predicting public interest in cosmetic surgery procedures and to examine temporal trends in tweets related to cosmetic facial and body procedures. METHODS: The TARPT tool was used to calculate the total number of tweets containing keywords related to 10 facial cosmetic procedures and 7 cosmetic body procedures from 2010 to 2020. Annual volumes for respective procedures were obtained from annual statistics reports of The Aesthetic Society from 2010 to 2020. Tweet volumes and procedure volumes were compared by univariate linear regression, taking P < 0.05 as the cutoff for significance. RESULTS: Variations in tweet volume were observed. Univariate linear regression analysis demonstrated statistically significant positive correlations between tweet volumes and procedure volumes for 7 search terms: "eyelid lift," "facelift," "lip injections," "mastopexy," "butt lift," "butt implants," and "liposuction." Many procedure-related keywords were not significant, demonstrating the importance of careful selection of Twitter search terms. CONCLUSIONS: The TARPT database represents a promising novel source of information for plastic surgeons, with the potential to inform marketing and advertising decisions for emerging trends in plastic surgery interest before these patterns become apparent in surgical or clinical volumes.
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Lipectomia , Mamoplastia , Mídias Sociais , Cirurgia Plástica , Estética , HumanosRESUMO
Purpose: To evaluate outcomes of primary eyelid and canalicular laceration repair using a self-retaining bicanalicular hydrophilic nasolacrimal stent.Methods: This study is a retrospective review of seven patients with canalicular laceration primarily repaired with a self-retaining bicanalicular nasolacrimal stent within 72 hours of initial injury. After lacrimal intubation, repair was performed by reapproximation of the lacerated tissues. Data collection included ophthalmic examination, mechanism of injury, involvement of upper/lower/common canaliculi, symptoms of epiphora, operative time, stent extrusion and canalicular irrigation.Results: Seven patients with monocanalicular laceration were repaired using the self-retaining bicanalicular nasolacrimal stent. The stent was left in place an average of 5 months. The average operative time was 29.3 minutes. None of the stents extruded prior to removal. After 5 months, the stent was removed successfully without difficulty. All patients reported subjective improvement in epiphora, with complete resolution of symptoms. Anatomic success was confirmed with irrigation in all cases. There were no complications associated with usage of the self-retaining bicanalicular nasolacrimal stent.Conclusions: Primary repair of canalicular lacerations can be successfully performed with a self-retaining bicanalicular nasolacrimal stent. This stent has several advantages, including potentially faster operative times, no need for intranasal fixation or retrieval, easier intubation in the lacrimal system, and improved anatomic reapproximation of lacerated tissues.
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Lacerações , Doenças do Aparelho Lacrimal , Aparelho Lacrimal , Ducto Nasolacrimal , Pálpebras/lesões , Pálpebras/cirurgia , Humanos , Intubação , Lacerações/cirurgia , Aparelho Lacrimal/lesões , Aparelho Lacrimal/cirurgia , Ducto Nasolacrimal/cirurgia , Estudos Retrospectivos , StentsRESUMO
PURPOSE: To examine the role of adjuvant surgical resection of infantile hemangiomas after systemic ß-blocker therapy. METHODS: This is a multicentered retrospective study. Standard protocol for oral propranolol was employed by the referring physicians. Ocular indications for surgery included ptosis obstructing the visual axis, high degrees of astigmatism causing amblyopia, or disfigurement from residual tumor. Patients underwent complete excision or debulking. RESULTS: Eleven girls and 4 boys were surgically treated with mean operative age of 34.4 months. Patients were followed for a mean of 19.6 months after surgery. Four patients required surgical treatment due to an inability to tolerate medical therapy secondary to drug-related side effects (including bradycardia). The other 11 patients proceeded to surgery due to residual eyelid and orbital lesions despite medical treatment. All 15 patients underwent orbitotomy for residual hemangioma excision. Four patients also underwent simultaneous levator advancement at the time of excision. In all cases, there was resolution of ptosis with clearing of the visual axis. No complications were incurred during the surgical treatment and there were no hemangioma recurrences. CONCLUSIONS: This is the first study to report surgical management of periocular infantile hemangiomas recalcitrant to standard therapy in the ß-blocker era. In patients with infantile hemangioma who have failed medical therapy, adjuvant surgical treatment still plays an important role. For patients with persistent tumor causing ocular sequelae, surgical intervention aimed at soft tissue debulking and ptosis repair can be successful in achieving excellent functional and aesthetic outcomes with minimal side effects.For patients with periocular infantile hemangiomas with residual soft tissue deformity following propranolol therapy, surgical treatment plays an important role in improving functional and cosmetic outcomes with minimal side effects.
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Hemangioma Capilar , Hemangioma , Antagonistas Adrenérgicos beta/uso terapêutico , Pré-Escolar , Feminino , Hemangioma/tratamento farmacológico , Hemangioma/cirurgia , Hemangioma Capilar/tratamento farmacológico , Hemangioma Capilar/cirurgia , Humanos , Lactente , Masculino , Recidiva Local de Neoplasia , Propranolol/uso terapêutico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
A 69-year-old woman presented to the oculofacial plastic service with a painless superotemporal subconjunctival mass in the OS. Over the past year, the lesion had been progressively enlarging, resulting in horizontal diplopia with lateral gaze. Visual acuity was within normal limits with no evidence of optic neuropathy. On examination, the lesion was tense, transilluminated, and was clinically consistent with a simple dacryops. Complete excision of the lesion was planned under local anesthesia with monitored care. To facilitate complete removal of the lesion, fibrinogen and a mixture of thrombin and trypan blue were injected to fill the cyst cavity. This blue-stained fibrin clot allowed for easy visualization of the border and ensured complete excision without collateral damage to surrounding normal tissue. Simple dacryops is often difficult to remove completely with its capsule intact and this technique allows for clear delineation of the cyst and preservation of epithelial integrity for complete and efficient removal.
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Cistos/cirurgia , Adesivo Tecidual de Fibrina/farmacologia , Doenças do Aparelho Lacrimal/cirurgia , Aparelho Lacrimal/cirurgia , Procedimentos Cirúrgicos Oftalmológicos/métodos , Azul Tripano/farmacologia , Idoso , Corantes/farmacologia , Cistos/diagnóstico , Feminino , Humanos , Doenças do Aparelho Lacrimal/diagnóstico , Microscopia com Lâmpada de Fenda , Adesivos Teciduais/farmacologiaRESUMO
A 5-year-old otherwise healthy girl presented to the oculoplastic service with a painless superotemporal subconjunctival mass in the left eye. Visual acuity was within normal limits, and there was no evidence of proptosis or orbital enlargement. Excision was performed to remove the anterior portion of the mass for alleviation of symptoms. On histopathological analysis, the mass was comprised of fibroadipose tissue consistent with dermolipoma and contained a hard nodule found to be a calcified tooth. In the periocular region, odontogenic choristoma (tooth) is a rare lesion, and has been reported to occur within teratomas, dermoid cysts, and displaced oral embryonic epithelium. We describe an unusual case of a tooth occurring within a sporadic dermolipoma. The clinical presentation, examination, management, and histopathology are reviewed.
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Coristoma/patologia , Doenças da Túnica Conjuntiva/patologia , Lipoma/patologia , Neoplasias Cutâneas/patologia , Dente , Pré-Escolar , Coristoma/cirurgia , Doenças da Túnica Conjuntiva/cirurgia , Feminino , Humanos , Lipoma/cirurgia , Procedimentos Cirúrgicos Oftalmológicos , Neoplasias Cutâneas/cirurgiaRESUMO
PURPOSE: To describe in detail cases with an initial diagnosis of Leber congenital amaurosis that were later found to have a hemizygous mutation in the CACNA1F gene. METHODS: The patients underwent a detailed ophthalmological evaluation and full-field electroretinography (ERG). Selective targeted capture and whole-exome next-generation sequencing (NGS) were used to find the disease-causing mutations. RESULTS: Patient 1 presented at age 3 months with nystagmus, normal visual attention, and a normal fundus exam. ERG responses were severely decreased. Patient 2 presented with nystagmus, severe hyperopia, esotropia, and visual acuity of 20/360 oculus dexter (OD) and 20/270 oculus sinister (OS) at age 5 months. His fundus exam showed slightly increased pigmentation around the foveae. The scotopic ERG responses were severely decreased and photopic responses mildly decreased. Based on the initial presentation, both patients received the clinical diagnosis of Leber congenital amaurosis (LCA). However, genetic testing showed no mutations in known LCA genes. Instead, broader genetic testing using NGS showed point mutations in the CACNA1F gene, which is reported to be associated with type 2 congenital stationary night blindness (CSNB2). CONCLUSIONS: These two cases demonstrate the clinical overlap between LCA and CSNB in infants and young children. Genetic testing is an essential tool in these cases and provides a more accurate diagnosis and prognosis for patients with inherited retinal degenerative disorders.
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Canais de Cálcio Tipo L/genética , Erros de Diagnóstico , Oftalmopatias Hereditárias/diagnóstico , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Amaurose Congênita de Leber/diagnóstico , Miopia/diagnóstico , Cegueira Noturna/diagnóstico , Mutação Puntual , Análise Mutacional de DNA , Eletrorretinografia , Exoma , Oftalmopatias Hereditárias/genética , Doenças Genéticas Ligadas ao Cromossomo X/genética , Testes Genéticos , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Amaurose Congênita de Leber/genética , Masculino , Miopia/genética , Cegueira Noturna/genética , Nistagmo Patológico/diagnóstico , Erros de Refração/diagnóstico , Testes de Campo Visual , Campos VisuaisRESUMO
PURPOSE: This study aimed to determine whether the presence of an uncleaved embryo on day 3 is predictive of cycle outcome after day 5 transfer (D5 ET). METHODS: In vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI) cycles from January 2013 to November 2014 with D5 ET were analyzed for the presence of at least one uncleaved embryo on day 3 (D3). Each index cycle (n = 70) was compared with two matched control cycles without uncleaved embryos. The main outcome measures included embryo quality, implantation rate, and clinical pregnancy rate. RESULTS: Fifty-nine of 3896 total embryos in this study were uncleaved on D3 (1.5 %). Cycles with uncleaved embryos had more oocytes retrieved (20.6 vs. 17.5), lower proportions of good quality embryos on D3 (52.4 vs. 66.1 %), and fewer usable embryos (transferred or frozen) on D5 (42.4 vs. 50.8 %). However, there were no significant differences in the incidence of cycles with a positive hCG, or in the rates of implantation, clinical pregnancy, or live birth. CONCLUSIONS: Although an uncleaved embryo on D3 is associated with reduced conversion of sibling embryos to the blastocyst stage on D5, overall quality of those embryos forming blastocysts is not markedly decreased and clinical outcomes are not compromised.
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Fase de Clivagem do Zigoto/citologia , Implantação do Embrião , Embrião de Mamíferos/citologia , Fertilização/fisiologia , Oócitos/citologia , Adulto , Fase de Clivagem do Zigoto/fisiologia , Transferência Embrionária , Embrião de Mamíferos/fisiologia , Feminino , Fertilização in vitro/métodos , Seguimentos , Humanos , Nascido Vivo , Oócitos/fisiologia , Gravidez , Taxa de Gravidez , PrognósticoRESUMO
OBJECTIVE: To determine the cost-effectiveness of endoscopic dacryocystorhinostomy (DCR). METHODS: We constructed a Markov model in which patients with nasolacrimal duct obstruction received endoscopic DCR or no surgery. Incremental cost-effectiveness ratios, 1-way sensitivity analyses, and probabilistic sensitivity analyses were used to evaluate for model sensitivity to multiple model inputs. RESULTS: Endoscopic DCR was found to be cost-effective with an incremental cost-effectiveness ratio of US$2162 per quality-adjusted life-year. The model was most sensitive to the health utility deduction from epiphora. Probabilistic sensitivity analysis found endoscopic DCR to be cost-effective over no surgery 93.7% of the time. CONCLUSIONS: Endoscopic DCR is a cost-effective treatment for patients with epiphora. The model is very sensitive to the negative effect epiphora has on quality of life. With the advancement of health care technology and surgical techniques, the success rates of endoscopic DCR continue to improve and to be an even more efficacious and economical treatment for nasolacrimal duct obstruction.
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PURPOSE: Impaired consciousness in epileptic seizures has a major negative impact on patient quality of life. Prior work on epileptic unconsciousness has mainly used retrospective and nonstandardized methods. Our goal was to validate and to obtain initial data using a standardized prospective testing battery. METHODS: The responsiveness in epilepsy scale (RES) was used on 52 patients during continuous video-electroencephalography (EEG) monitoring. RES begins with higher-level questions and commands, and switches adaptively to more basic sensorimotor responses depending on patient performance. RES continues after seizures and includes postictal memory testing. Scoring was conducted based on video review. KEY FINDINGS: Testing on standardized seizure simulations yielded good intrarater and interrater reliability. We captured 59 seizures from 18 patients (35% of participants) during 1,420 h of RES monitoring. RES impairment was greatest during and after tonic-clonic seizures, less in partial seizures, and minimal in auras and subclinical seizures. In partial seizures, ictal RES impairment was significantly greater if EEG changes were present. Maximum RES impairment (lowest ictal score) was also significantly correlated with long postictal recovery time, and poor postictal memory. SIGNIFICANCE: We found that prospective testing of responsiveness during seizures is feasible and reliable. RES impairment was related to EEG changes during seizures, as well as to postictal memory deficits and recovery time. With a larger patient sample it is hoped that this approach can identify brain networks underlying specific components of impaired consciousness in seizures. This may allow the development of improved treatments targeted at preventing dysfunction in these networks.
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Transtornos da Consciência/diagnóstico , Epilepsia/diagnóstico , Adolescente , Adulto , Idoso , Criança , Transtornos da Consciência/etiologia , Transtornos da Consciência/fisiopatologia , Eletrodiagnóstico/métodos , Eletrodiagnóstico/normas , Eletroencefalografia/métodos , Eletroencefalografia/normas , Epilepsia/complicações , Epilepsia/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Monitorização Fisiológica/métodos , Monitorização Fisiológica/normas , Estudos Prospectivos , Gravação em Vídeo/métodos , Gravação em Vídeo/normas , Adulto JovemRESUMO
PURPOSE: To characterize the frequency, severity, and resolution of hearing dysfunction in patients treated with teprotumumab for thyroid eye disease (TED). DESIGN: Prospective observational case series. METHODS: Ophthalmic examination and adverse event assessment, including otologic symptoms, were performed at baseline, after infusions 2, 4, and 8, and at 6-month follow-up in consecutive patients who received at least 4 teprotumumab infusions. Laboratory test results were collected at baseline and during treatment. Audiometry, patulous eustachian tube (PET) testing, and otolaryngology evaluation were obtained for patients with new or worsening otologic symptoms, with a subset obtaining baseline and posttreatment testing. RESULTS: Twenty-seven patients were analyzed (24 females, 3 males, average 56.3 years old). Twenty-two patients (81.5%) developed new subjective otologic symptoms, after a mean of 3.8 infusions (SD 1.8). At 39.2-week average follow-up after the last infusion, most patients with tinnitus (100%), ear plugging/fullness (90.9%), and autophony (83.3%) experienced symptom resolution, whereas only 45.5% (5 of 11) of patients with subjective hearing loss/decreased word comprehension experienced resolution. Six patients underwent baseline and posttreatment audiometry, 5 of whom developed teprotumumab-related sensorineural hearing loss (SNHL) and 1 patient also developed PET. Three of the 5 patients with teprotumumab-related SNHL had persistent subjective hearing loss at last follow-up. A prior history of hearing loss was discovered as a risk factor for teprotumumab-related SNHL (P = .008). CONCLUSIONS: Hearing loss is a concerning adverse event of teprotumumab, and its mechanism and reversibility should be further studied. Until risk factors for hearing loss are better understood, we recommend baseline audiometry with PET testing and repeat testing if new otologic symptoms develop. Screening, monitoring, and prevention guidelines are needed.
Assuntos
Oftalmopatia de Graves , Perda Auditiva Neurossensorial , Perda Auditiva , Anticorpos Monoclonais Humanizados , Audiometria/efeitos adversos , Feminino , Oftalmopatia de Graves/induzido quimicamente , Oftalmopatia de Graves/diagnóstico , Oftalmopatia de Graves/tratamento farmacológico , Audição , Perda Auditiva/complicações , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Purpose: To compare the reproducibility of two-dimensional (2D) peripapillary retinal nerve fiber layer (RNFL) thickness and three-dimensional (3D) neuroretinal rim measurements using spectral domain optical coherence tomography (SDOCT) in normal and glaucoma subjects. Methods: One eye per subject for 27 normal and 40 glaucoma subjects underwent repeat SDOCT RNFL thickness scans and optic nerve volume scans on the same day. From the volume scan, custom software calculated five neuroretinal rim parameters: 3D minimum distance band (MDB) thickness, 3D MDB area, 3D rim volume, 2D rim area, and 2D rim thickness. Within-subject variance (Sw), coefficient of variation (CV), and intraclass correlation coefficient (ICC) were analyzed. Results: MDB thickness and RNFL thickness have similar reproducibility among normal and glaucoma subjects (eg, global MDB thickness CVs of 2.4% and 3.6%, and global RNFL thickness CVs of 1.3% and 2.2%; P > 0.05 for both comparisons). Reproducibility of MDB thickness was lower in glaucoma patients for the superior and inferior quadrants compared to normal subjects (CVs of 9.6% versus 3.4% and 6.9% versus 2.7%; P < 0.05, respectively). There were no statistically significant differences between both groups for RNFL thickness in the four quadrants. For both patient groups and for all regions, MDB thickness had the lowest CVs among all five neuroretinal rim parameters (eg, global MDB thickness CVs of 2.4% and 3.6% versus 3.0% and 18.9% for the other four neuroretinal rim parameters). Conclusion: Global MDB and global RNFL thickness are similarly reproducible among normal and glaucoma subjects, though MDB thickness for the superior and inferior quadrants is less reproducible among glaucoma subjects.
RESUMO
Thyroid eye disease (TED) is a complex disease associated with myriad clinical presentations, including facial disfigurement, vision loss, and decreased quality of life. Traditionally, steroid therapy and/or radiation therapy were commonly used in the treatment of active TED. While these therapies can help reduce inflammation, they often do not have a sustainable, significant long-term effect on disease outcomes, including proptosis and diplopia. Recent advances in our understanding of the pathophysiology of TED have shifted the focus of treatment toward targeted biologic therapies. Biologics have the advantage of precise immune modulation, which can have better safety profiles and greater efficacy compared to traditional approaches. For instance, the insulin-like growth factor-1 receptor (IGF-1R) has been found to be upregulated in TED patients and to colocalize with the thyroid-stimulating hormone receptor (TSHR), forming a signaling complex. Teprotumumab is an antibody targeted against IGF-1R. By inhibiting the IGF-1R/TSHR signaling pathway, teprotumumab may reduce the production of proinflammatory cytokines, hyaluronan secretion, and orbital fibroblast activation in patients with TED. Due to promising phase II and III clinical trial results, teprotumumab has become the first biologic US Food and Drug Administration (FDA)-approved for the treatment of TED. In addition, there are currently ongoing studies looking at the use of antibodies targeting the neonatal Fc receptor (FcRn) in various autoimmune diseases, including TED. FcRn functions to transport immunoglobulin G (IgG) and prevent their lysosomal degradation. By blocking the recycling of IgG, this approach may dampen the body's immune response, in particular the pathogenic IgG implicated in some autoimmune diseases. Advances in our understanding of the pathophysiology of TED, therefore, are leading to more targeted therapeutic options, and we are entering an exciting new phase in the management of TED. This review will cover recent insights into the understanding of TED pathophysiology and novel treatment options as well as ongoing studies of new potential treatment options for TED.
RESUMO
Endocrine tumors such as aldosterone-producing adrenal adenomas (APAs), a cause of severe hypertension, feature constitutive hormone production and unrestrained cell proliferation; the mechanisms linking these events are unknown. We identify two recurrent somatic mutations in and near the selectivity filter of the potassium (K(+)) channel KCNJ5 that are present in 8 of 22 human APAs studied. Both produce increased sodium (Na(+)) conductance and cell depolarization, which in adrenal glomerulosa cells produces calcium (Ca(2+)) entry, the signal for aldosterone production and cell proliferation. Similarly, we identify an inherited KCNJ5 mutation that produces increased Na(+) conductance in a Mendelian form of severe aldosteronism and massive bilateral adrenal hyperplasia. These findings explain pathogenesis in a subset of patients with severe hypertension and implicate loss of K(+) channel selectivity in constitutive cell proliferation and hormone production.