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1.
Eplasty ; 24: e27, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38846502

RESUMEN

Background: Intraosseous hemangiomas are rare benign tumors comprising fewer than 1% of all osseous tumors; even more uncommon are intraosseous hemangiomas of the zygomatic bone. This case reports a multidisciplinary approach for excision and reconstruction of an intraosseous hemangioma of the zygomatic bone in a 54-year-old female. Methods: Multidisciplinary approach with both otolaryngology head and neck surgery and oculofacial plastics and reconstructive surgery included right lateral canthotomy and right transconjunctival orbitotomy with en-bloc excision of the zygomatic arch, followed by reconstruction of the orbital rim, orbital floor, and eyelid with Medpor implant. Results: Final surgical pathology was consistent with intraosseous hemangioma of the zygomatic bone. At 4-month follow-up, the patient was healing well with good midface projection and without any visual deficits. Conclusions: A multidisciplinary coordinated case allowed us to meet the standard of maintaining cosmesis and function while undergoing resection of a rare tumor involving a key facial structure-the zygoma. Involvement of oculofacial plastics and reconstructive surgery service allowed for advanced eyelid reconstruction techniques to limit any functional impairment to our patient with deliberate choice of implant material for well-adhered, durable, and aesthetically optimal reconstruction of the right malar eminence, lateral orbital rim, and orbital floor defect. The postoperative result through the multidisciplinary approach was a near symmetrical facial reconstruction without any associated eyelid or globe abnormalities.

2.
Cureus ; 16(4): e59206, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38807824

RESUMEN

Background Ophthalmology is a unique specialty with limited exposure during medical school. To improve the transition to ophthalmology residency, the Accreditation Council for Graduate Medical Education (ACGME) announced in 2017 that all ophthalmology residency programs would move to a combined post-graduate year (PGY) 1 year with mandatory integration by 2023. Currently, there are no standardized guidelines from the American Board of Ophthalmology (ABO) or the Accreditation Council for Graduate Medical Education (ACGME) to address ophthalmology resident competence prior to becoming the primary contact for inpatient and emergency room (ER) consultations as a PGY-2. Novice residents may not be equipped to accurately diagnose vision or life-threatening ocular conditions. A balance between resident autonomy and supervision is required for proper training without increasing patient morbidity and mortality. Objective This study's objective is to examine the diagnostic accuracy of PGY-2 ophthalmology non-integrated residents on call to standardize supervision requirements (through buddy-call) prior to initiating indirectly supervised calls. Methods All inpatient and ER ophthalmology consults for the first seven weeks of the year evaluated by PGY-2 (junior) residents were supervised and graded as "correct" or "incorrect" by PGY-4 (senior) residents. Results One hundred forty-eight consults were seen over 30 call days over a period of seven weeks (4.93 consults per call). The percentage of correct diagnoses increased with each successive week (R2 = 0.9581; correlation = 0.979). The greatest percent increase of correctly diagnosed encounters was between weeks 2 and 3 (19.14%) correlating to call numbers 10-16 and 45-68 patient encounters. The mean percent accuracy surpassed 70% during weeks 3-4, and improvement continued to week 7. High-acuity diagnoses were identified consistently 100% of the time from week 5 onward. Conclusion Our analysis indicated that diagnostic accuracy was greater than 70% between weeks 3 and 4 with high-acuity diagnostic accuracy reaching 100% at week 5. It can be postulated that optimal direct senior resident supervision is needed for at least 3-5 weeks before transitioning to indirectly supervised calls by PGY-2 residents. This standardization would allow junior residents to acquire sufficient clinical experience to accurately make a diagnosis and prevent patient morbidity. Further research nationally is necessary prior to creating a standardized call structure for PGY-2 residents especially with the newly mandatory integrated ophthalmology residency programs.

3.
Orbit ; : 1-7, 2024 May 30.
Artículo en Inglés | MEDLINE | ID: mdl-38815176

RESUMEN

PURPOSE: The purpose of this study is to determine the public's perception of the scope of practice for oculofacial plastic and reconstructive surgeons (OFPRS). METHODS: A 49-question survey was distributed by QualtricsⓇ to a panel similar to the US demographic composition. Responses collected underwent bivariate statistical analysis. RESULT: A total of 530 responses were obtained, with most respondents being white, female, over the age of 35, from the Midwest, and with at least a college education or above. Most respondents did not think ophthalmologists or optometrists were surgeons, and only 158 people (29.8%) knew the primary specialty of OFPRS was ophthalmology. Board certification was preferred by 98.87% of respondents, and 95.28% preferred ASOPRS-trained OFPRS. CONCLUSIONS: Our study highlights the gap in knowledge about OFPRS as a field, the qualifications and training required, and the scope of practice. Notably, even for OFPRS-specific procedures, PRS remained the leading subspecialist chosen for interventions such as orbital decompression (58.5% vs. 71.5%), orbital reconstruction (57.9% vs. 74.2%), enucleation/evisceration (48.1% vs. 53.4%), optic nerve-related surgery (39.8% vs. 43.4%), orbital cancer resection (42.8% vs. 46.8%), and tear duct surgery (41.9% vs. 52.5%). Additionally, most respondents did not feel that facial fillers, laser skin resurfacing, eyelid cancer removal, or cataract surgery were within the OFPRS scope of practice.

4.
Cureus ; 16(4): e58585, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38765324

RESUMEN

As the use of teprotumumab for thyroid eye disease (TED) becomes more prolific, there remains a scarcity of literature regarding the associated side effects and adverse events of teprotumumab use. The authors present a single-center retrospective, observational case review of TED patients who received at least a single dose of teprotumumab infusion at the oculofacial plastic surgery service between February 2020 and July 2023. The most predominant recollected side effects were fatigue, brittle nails, dry eye symptoms, hair loss, muscle spasms, and dry mouth. Significant adverse events were limited to two cases of a blood clot and a single case of pulmonary embolism. This is the first retrospective study of patient-reported side effects and adverse events experienced by a cohort of teprotumumab users.

5.
Orbit ; : 1-6, 2024 Feb 07.
Artículo en Inglés | MEDLINE | ID: mdl-38323601

RESUMEN

The incidence of penetrating orbital injuries from writing instruments continues to rise in the pediatric population. Such injuries can cause significant visual morbidity and have a lifelong psychosocial impact. While the description of graphite pencil-related orbital trauma management is well demonstrated with over 40 reported cases, a lack of consistent management protocol for colored pencil-related injuries. Here, we report an inadvertent penetrating orbital colored pencil injury with progressive mechanical ptosis and pre-septal cellulitis necessitating urgent orbitotomy, debridement, and washout to reduce inflammatory and infectious burden. The wooden body serves as a nidus for polymicrobial infection, and the unique composition of colored pencil cores may lead to inflammatory processes that require vigilant multidisciplinary surgical and medical management reflected in our literature review.

6.
Ophthalmic Plast Reconstr Surg ; 40(1): 34-38, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-37552488

RESUMEN

PURPOSE: The integrity of the orbit has a finite structural limit due to the compliance of its tissue. The authors investigate these limits to quantify them and inform the treatment of heightened ocular and orbital pressure. METHODS: Cadaveric study with 12 orbits being volumized before randomization of treatment for pathologic levels of ocular and orbital pressure. First-line and second-line treatment was chosen randomly (lateral cantholysis, superior septolysis, inferior septolysis). Prior to treatment, IOP, orbital compartment pressure, and axial globe projection was measured after delivery of each 1cc aliquot and surgical treatment. RESULTS: Orbital compartment pressure and IOP were well correlated (r = 0.99). The average reduction in IOP after treatment averaged a 56.2 mm Hg reduction in IOP. All treatments were statistically equivalent ( p < 0.01). Loss of compliance (P LOC ) was determined when the mean plus 1 standard deviation of change in IOP/ml volume was achieved with simultaneous change in exophthalmometry of <0.5 mm change/ml added volume, indicating an acceleration in pressure in the face of a steady volume. This criteria was met for 11 of 12 orbits. The 12th orbit missed this threshold by 1 mm Hg in IOP. P LOC occurred at an average IOP of 43.0 mm Hg (±5.8 mm Hg, 90% CI) and after an average injection of 13 ml (±1.4 mm, 90% CI). Additionally, lateral cantholysis, superior septolysis, and inferior septolysis were statistically equivalent in reducing IOP after P LOC . CONCLUSIONS: IOP and orbital compartment pressure are excellent proxies for each other in the authors' model. Orbital compliance is a mathematic phenomenon that can be quantified, as evident in this investigation. P LOC can inform timing for orbital decompressions in the presence of heightened IOP. Multiple procedures can be used to extinguish dangerously high orbital compartment pressure.


Asunto(s)
Presión Intraocular , Órbita , Humanos , Órbita/cirugía , Descompresión Quirúrgica/métodos
7.
J Clin Endocrinol Metab ; 109(1): 25-35, 2023 Dec 21.
Artículo en Inglés | MEDLINE | ID: mdl-37925673

RESUMEN

CONTEXT: Early inflammatory thyroid eye disease (TED) can lead to symptomatic chronic disease, including disabling proptosis. Teprotumumab, an insulin-like growth factor-1 receptor (IGF-1R) inhibitor, previously demonstrated efficacy in acute, high-inflammation TED trials. OBJECTIVE: We present data from the first placebo-controlled trial with teprotumumab in chronic/low disease activity TED. METHODS: This randomized double-masked, placebo-controlled trial, conducted at 11 US centers, enrolled adult participants with TED duration of 2 to 10 years, Clinical Activity Score (CAS) ≤ 1 or no additional inflammation or progression in proptosis/diplopia for ≥1 year, proptosis ≥3 mm from before TED and/or from normal, euthyroid/mildly hypo/hyperthyroid, no prior teprotumumab, and no steroids within 3 weeks of baseline. Patients received (2:1) intravenous teprotumumab or placebo once every 3 weeks (total 8 infusions). The primary endpoint was proptosis (mm) improvement at Week 24. Adverse events (AEs) were assessed. RESULTS: A total of 62 (42 teprotumumab and 20 placebo) patients were randomized. At Week 24, least squares mean (SE) proptosis improvement was greater with teprotumumab (-2.41 [0.228]) than with placebo (-0.92 [0.323]), difference -1.48 (95% CI -2.28, -0.69; P = .0004). Proportions of patients with AEs were similar between groups. Hyperglycemia was reported in 6 (15%) vs 2 (10%) and hearing impairment in 9 (22%) vs 2 (10%) with teprotumumab and placebo, respectively. AEs led to discontinuation in 1 teprotumumab (left ear conductive hearing loss with congenital anomaly) and 1 placebo patient (infusion-related). There were no deaths. CONCLUSION: Teprotumumab significantly improved proptosis vs placebo in longstanding/low inflammation TED, demonstrating efficacy regardless of disease duration/activity. The safety profile was comparable to that previously reported.


Asunto(s)
Exoftalmia , Oftalmopatía de Graves , Adulto , Humanos , Anticuerpos Monoclonales Humanizados/efectos adversos , Oftalmopatía de Graves/tratamiento farmacológico , Inflamación , Inhibidores de Proteínas Quinasas , Método Doble Ciego
8.
Arthrosc Sports Med Rehabil ; 5(1): e201-e206, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36866312

RESUMEN

Purpose: To evaluate the recurrent dislocation risk and patient-reported outcomes of peroneus longus allograft tissue for medial patellofemoral ligament (MPFL) reconstruction. Methods: Patients who underwent MPFL reconstruction with peroneus longus allograft at an academic center between 2008 and 2016 were identified. Record review and patient contact were used to identify any cases of recurrent patellar dislocation and collect patient-reported outcomes scores (Knee injury and Osteoarthritis Outcome Score [KOOS], Norwich Patellar Instability score, Marx activity scale). Patients with 1-year minimum follow-up were included. Outcomes were quantified and the proportion of patients reaching a previously defined patient acceptable symptom state (PASS) for patellar instability was determined. Results: Sixty-one patients (42 female and 19 male) underwent MPFL reconstruction with peroneus longus allograft during the study period. Forty-six patients (76%) with 1-year minimum follow up were contacted at a mean of 3.5 years postoperative. The mean age at time of surgery was 22 ± 7.2 years. Patient-reported outcomes data were available in 34 patients. Mean KOOS subscale scores were as follows: Symptoms 83.2 ± 19.1, Pain 85.2 ± 17.6, Activities of Daily Living 89.9 ± 14.8, Sports 75 ± 26.2, and Quality of Life 72.6 ± 25.7. The mean Norwich Patellar Instability score was 14.9% ± 17.4%. The mean Marx activity score was 6.0 ± 5.2. No recurrent dislocations were noted during the study period. Sixty-three percent of patients who underwent isolated MPFL reconstruction met PASS thresholds in at least 4 of 5 KOOS subscales. Conclusions: The use of a peroneus longus allograft in MPFL reconstruction in conjunction with other indicated procedures results in a low re-dislocation risk and a high proportion of patients meeting PASS criteria for patient-reported outcome scores 3 to 4 years postoperatively. Level of Evidence: IV, case series.

9.
Ophthalmic Plast Reconstr Surg ; 39(4): e128-e132, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36972111

RESUMEN

The authors report a penetrating orbitocerebral vape pen injury necessitating a primary enucleation and craniotomy to remove the foreign body fragments. A 31-year-old male presented with acute right vision loss after a modifiable vape pen explosion launched multiple projectile fragments into his right eye. CT revealed a deformed globe with multiple radiodense curvilinear fragments in the superior orbital roof and intracranial space. A right frontal craniotomy and orbitotomy with removal of vape pen fragments, reconstruction of the orbital roof, primary enucleation, and eyelid repair were performed in conjunction with neurosurgery. To the best of the authors' knowledge, this is the first reported penetrating globe injury from a vape pen explosion.


Asunto(s)
Lesiones Oculares , Cuerpos Extraños , Fracturas Orbitales , Vapeo , Masculino , Humanos , Adulto , Órbita/diagnóstico por imagen , Órbita/cirugía , Órbita/lesiones , Fracturas Orbitales/diagnóstico , Fracturas Orbitales/etiología , Fracturas Orbitales/cirugía
10.
Cureus ; 14(10): e30215, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36381938

RESUMEN

Orbital implant materials have evolved greatly over the past century and include but are not limited to metal, ceramic, polymer, silicone, and glass. Knowledge of historically used materials is clinically relevant to patient care as certain materials carry a greater risk of migration, extrusion, infection, and limitations for imaging modalities utilized to visualize adjacent structures. We report an unusual case of an 80-year-old male who presented to our community hospital with seizure-like activity. CT imaging of the brain revealed several white matter and cortex lesions with the largest lesion measuring 2.5 × 2 × 1.9 cm. The patient had a history of enucleation with placement of an orbital implant following a penetrating injury to the left eye at four years of age. Hounsfield scale analysis was read by radiology as being most consistent with a thin metallic shell surrounding the orbital implant. The potential for metallic material was consistent with the implant's age and time of placement. Few reviews on ocular implant materials from this period exist in the current medical literature. A single case report discussing a hollow metal orbital implant with similar-appearing imaging was identified. Due to concern for possible metal implant materials, the patient underwent implant exchange so MRI imaging could safely be performed. Intraoperatively, the implant was identified as a clear, hollow, non-metallic, non-porous polymer sphere. Following surgery, the patient was able to undergo appropriate neuroimaging with subsequent diagnostic biopsy. Current literature reviewing CT or photographic imaging of ocular implant devices prior to the 1940s is limited. This case highlights the importance of detailing materials historically used in orbital implants, their effects on clinical decision-making, and the utility of Hounsfield scale values to identify a material's radiodensity on CT imaging.

11.
Commun Biol ; 5(1): 1231, 2022 11 12.
Artículo en Inglés | MEDLINE | ID: mdl-36371461

RESUMEN

Cell-cell communication and physical interactions play a vital role in cancer initiation, homeostasis, progression, and immune response. Here, we report a system that combines live capture of different cell types, co-incubation, time-lapse imaging, and gene expression profiling of doublets using a microfluidic integrated fluidic circuit that enables measurement of physical distances between cells and the associated transcriptional profiles due to cell-cell interactions. We track the temporal variations in natural killer-triple-negative breast cancer cell distances and compare them with terminal cellular transcriptome profiles. The results show the time-bound activities of regulatory modules and allude to the existence of transcriptional memory. Our experimental and bioinformatic approaches serve as a proof of concept for interrogating live-cell interactions at doublet resolution. Together, our findings highlight the use of our approach across different cancers and cell types.


Asunto(s)
Transcriptoma , Neoplasias de la Mama Triple Negativas , Humanos , Microfluídica , Perfilación de la Expresión Génica/métodos , Regulación de la Expresión Génica
12.
Orthop J Sports Med ; 10(6): 23259671221104414, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35783469

RESUMEN

Background: Patella alta and elevated tibial tubercle-trochlear groove (TT-TG) distance can predispose patients to lateral patellar dislocations and recurrent instability. Their influence on patient-reported outcomes (PROs) after medial patellofemoral ligament (MPFL) reconstruction is less clear. Hypothesis: We hypothesized that neither moderately increased TT-TG distance nor patella alta would negatively affect PROs after isolated MPFL reconstruction in patients with relatively normal patellar tracking (no large J-sign). Study Design: Cohort study; Level of evidence, 3. Methods: We identified patients who underwent isolated MPFL reconstruction at a single institution between 2008 and 2016. The decision to perform an isolated MPFL reconstruction was at the discretion of the operating surgeon but was not performed in the setting of a large J-sign. Patient characteristics and surgical details were collected, and patients completed the Norwich Patellar Instability Score, Knee injury and Osteoarthritis Outcome Score, and Marx activity score. Patellar height (Caton-Deschamps Index [CDI]) was assessed on preoperative lateral radiographs, and TT-TG distance was measured on preoperative axial magnetic resonance imaging (MRI) scans. Patients were grouped based on CDI and TT-TG distance, and outcomes were compared. Linear regression modeling was performed to determine whether patella alta or elevated TT-TG distance was associated with poorer PRO scores. Results: Of 165 knees in 152 patients who underwent isolated MPFL reconstruction, 115 patients (125 knees; 76%) with minimum 1-year follow-up were contacted at a mean of 5.2 years after surgery. Recurrent dislocation occurred in 5 of 125 knees (4%). Preoperative radiographs were available in 111 knees (89%), and preoperative MRI scans were available in 89 knees (71%). Mean CDI was 1.13, and 35% had a CDI ≥1.20. Mean TT-TG distance was 17.5 mm, and 26% had a TT-TG distance >20 mm. After adjusting for patient age, sex, body mass index, and graft choice, we observed that neither patella alta nor elevated TT-TG distance were associated with poorer PROs. Conclusion: Isolated MPFL reconstruction in the setting of moderately elevated TT-TG distance or patella alta was not associated with worse PROs in this cohort with relatively normal patellar tracking (no large J-sign).

13.
Neurosurg Focus ; 53(1): E17, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-35901747

RESUMEN

OBJECTIVE: Head and neck arteriovenous malformations (AVMs) are complex lesions that represent a subset of vascular anomalies (VAs). The authors present an analysis of their institutional experience managing these lesions as a multidisciplinary team. METHODS: A retrospective chart review was performed of head and neck AVM patients treated at the authors' institution from 2012 to 2022. Recorded data included patient demographic characteristics, details of clinical presentation, Schöbinger clinical scale and Yakes AVM classification results, and details of all endovascular and surgical treatments. The primary outcome of the study was clinical response to treatment. Angiographic occlusion and complication rates were reported. Chi-square tests were used for comparative statistics. RESULTS: Sixteen patients (9 female, 56%) with AVMs of the head and neck presented from age 3 to 77 years. The Schöbinger stage was stage II in 56% of patients (n = 9) and stage III in 44% of patients (n = 7). The Yakes AVM classification was nidus type (2a, 2b, or 4) in 7 patients (43%) and fistula type (1, 3a, or 3b) in 9 patients (57%). The majority of patients (n = 11, 69.0%) were managed with embolization as the only treatment modality, with an average of 1.5 embolizations/patient (range 1-3). Surgical resection was employed in 5 patients (4 in combination with embolization). Symptom resolution and symptom control were achieved in 69% and 31% of patients, respectively, in the entire cohort. A radiographic cure was demonstrated in 50% of patients. There were no statistical differences in clinical outcomes or radiographic cure rates between patients treated with different modalities. CONCLUSIONS: Head and neck AVMs can be treated successfully with a primarily endovascular management strategy by a multidisciplinary team with the goal of symptomatic control.


Asunto(s)
Malformaciones Arteriovenosas , Embolización Terapéutica , Malformaciones Arteriovenosas Intracraneales , Adolescente , Adulto , Anciano , Malformaciones Arteriovenosas/diagnóstico por imagen , Malformaciones Arteriovenosas/epidemiología , Malformaciones Arteriovenosas/cirugía , Niño , Preescolar , Embolización Terapéutica/métodos , Femenino , Humanos , Malformaciones Arteriovenosas Intracraneales/cirugía , Persona de Mediana Edad , Cuello/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
14.
J Neurol Surg B Skull Base ; 83(3): 281-284, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35769794

RESUMEN

Objective Orbital roof fractures are more likely to occur in younger children, specifically younger than 7 years. Cranium to face ratio decreases with age; however, there is no definition for measurement of the neurocranium or face. We propose using the length of the orbital roof as a measurement of the neurocranium and length of the orbital floor as a tool to estimate midface size. The purpose of this study is to test this measurement as a correlation rate of orbital roof fractures within the pediatric population. Design This is a retrospective study. Setting This study was done at the LeBonheur Children's Hospital. Participants Sixty-six patients with orbital roof fractures were identified and stratified by gender and age, specifically younger than 7 years and 7 years or older. Main Outcome Measures The main outcome measures were orbital roof length, floor length, and ratio thereof. Results Mean orbital roof length was 43.4 ± 3.06 and 45.1 ± 3.94 mm for patients <7 and ≥7 years, respectively ( p = 0.02). Mean orbital floor length was 41.3 ± 2.99 and 47.7 ± 4.19 for patients <7 and ≥7 years, respectively ( p < 0.00001). The mean roof to floor ratio (RTFR) for patients <7 years was 1.051 ± 0.039 and for patients ≥ 7 years was 0.947 ± 0.031 ( p < 0.00001). Conclusion As children age, the relative length of the orbital roof decreases when compared with the orbital floor. The RTFR was more than 1.0 in children younger than 7 years. These differences were statistically significant when compared with children 7 years and older. This measurement shift follows the differences noted in orbital fracture patterns during childhood.

15.
J Inherit Metab Dis ; 45(5): 926-936, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35560233

RESUMEN

External quality assurance (EQA) is crucial to monitor and improve the quality of biochemical genetic testing. ERNDIM (www.erndim.org), established in 1994, aims at reliable and standardized procedures for diagnosis, treatment and monitoring of inherited metabolic disease (IMD) by providing EQA schemes and educational activities. Currently, ERNDIM provides 16 different EQA schemes including quantitative schemes for various metabolite groups, and interpretive schemes such as diagnostic proficiency testing (DPT). DPT schemes focus on the ability of laboratories to correctly identify and interpret abnormalities in authentic urine samples across a wide range of IMDs. In the DPT schemes, six samples each year are distributed together with clinical information. Laboratories choose and perform the tests needed to reach a diagnosis. Data were collected on 345 samples, distributed to up to 105 laboratories worldwide. Diagnostic proficiency (the % of total points possible for all participating laboratories within a scheme for analysis and interpretation) ranged widely: amino acid disorders (n = 20), range 33%-100%, mean 84%; organic acid disorders (n = 35), range 14%-100%, mean 84%; lysosomal storage disorders (n = 13), range 20%-97%, mean 73%; purine/pyrimidine disorders (n = 9), range 37%-100%, mean 70%; miscellaneous disorders (n = 8), range 17%-100%, mean 65%; no IMD, range 65%-95%, mean 85%. When a sample with the same disorder was distributed in a subsequent survey, performance improved in 75 cases with no improvement seen in 32, suggesting overall improvement of performance. ERNDIM diagnostic proficiency testing is a valuable activity which can help to assess laboratory performance, identify methodological/technical challenges, be informative during quality audits and contribute to a better clinical appreciation of diagnostic uncertainty.


Asunto(s)
Enfermedades por Almacenamiento Lisosomal , Enfermedades Metabólicas , Técnicas y Procedimientos Diagnósticos , Humanos , Laboratorios , Enfermedades Metabólicas/diagnóstico , Enfermedades Metabólicas/genética , Enfermedades Metabólicas/orina
16.
Am J Ophthalmol Case Rep ; 26: 101510, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35434419

RESUMEN

Purpose: To report an unusual case of spontaneous orbital hemorrhage in the setting of DIC and hepatic failure. Observations: A 33-year-old female presented to the Emergency Department (ED) with acute liver failure. During the first week of her hospital admission, she developed unilateral eyelid swelling and proptosis, prompting a consult to ophthalmology. Additional physical examination revealed significantly decreased visual acuity, extraocular muscle restriction, afferent pupillary defect, and increased intraocular pressure. Computed tomography (CT) and ultrasound confirmed the diagnosis of intraorbital hemorrhage. Aggressive management in the form of lateral canthotomy, cantholysis, and septolysis was unable to be performed due to the patient's multiple comorbidities outweighing the potential benefits. Conclusions and importance: This rare phenomenon is unique from previous existing literature in that the timing of the incident limited the therapeutic options for this patient, additional imaging in the form of ultrasound was utilized in the work-up, and to our knowledge this is the second case of spontaneous orbital hemorrhage as a complication of disseminated intravascular coagulation (DIC) caused by hepatic failure.

17.
Ophthalmology ; 129(4): 438-449, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-34688699

RESUMEN

PURPOSE: To evaluate teprotumumab safety/efficacy in patients with thyroid eye disease (TED) who were nonresponsive or who experienced a disease flare. DESIGN: The Treatment of Graves' Orbitopathy to Reduce Proptosis with Teprotumumab Infusions in an Open-Label Clinical Extension Study (OPTIC-X) is a teprotumumab treatment and re-treatment trial following the placebo-controlled teprotumumab Phase 3 Treatment of Graves' Orbitopathy (Thyroid Eye Disease) to Reduce Proptosis with Teprotumumab Infusions in a Randomized, Placebo-Controlled, Clinical Study (OPTIC) trial. PARTICIPANTS: Patients who previously received placebo (n = 37) or teprotumumab (n = 14) in OPTIC. METHODS: OPTIC nonresponders or those who flared (≥2-mm increase in proptosis, ≥2-point increase in clinical activity score [CAS], or both) during follow-up were treated for the first time (previous placebo patients) or re-treated with teprotumumab in OPTIC-X with 8 infusions over 24 weeks. MAIN OUTCOME MEASURES: Proptosis response and safety. Secondary outcomes included proptosis, CAS, subjective diplopia, and quality-of-life. RESULTS: Thirty-three of 37 placebo-treated OPTIC patients (89.2%) became proptosis responders (mean ± standard deviation, -3.5 ± 1.7 mm) when treated with teprotumumab in OPTIC-X. The responses were equivalent to the OPTIC study. In these responders, proptosis, CAS of 0 or 1, and diplopia responses were maintained in 29 of 32 patients (90.6%), 20 of 21 patients (95.2%), and 12 of 14 patients (85.7%), respectively, at follow-up week 48. The median TED duration was 12.9 months versus 6.3 months in those treated with teprotumumab in the OPTIC study. Of the 5 OPTIC teprotumumab nonresponders re-treated in OPTIC-X, 2 responded, 1 showed a proptosis reduction of 1.5 mm from OPTIC baseline, and 2 discontinued treatment early. Of the OPTIC teprotumumab responders who experienced flare, 5 of 8 patients (62.5%) responded when re-treated (mean proptosis reduction, 1.9 ± 1.2 mm from OPTIC-X baseline and 3.3 ± 0.7 mm from OPTIC baseline). Compared with published double-masked trials and their integrated follow-up, no new safety signals were identified. Mild hearing impairment was reported; 4 events occurred during the first course of treatment, and 2 events reoccurred after re-treatment. CONCLUSIONS: Patients with TED of longer disease duration responded similarly to those treated earlier in the disease course. Patients with an insufficient initial response or flare may benefit from additional teprotumumab therapy. No new safety risk was identified; however additional postmarketing pharmacovigilance is ongoing.


Asunto(s)
Exoftalmia , Oftalmopatía de Graves , Anticuerpos Monoclonales Humanizados/uso terapéutico , Diplopía , Oftalmopatía de Graves/tratamiento farmacológico , Humanos
18.
Orbit ; 41(5): 585-590, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34514944

RESUMEN

PURPOSE: To assess extended release/long acting (ER/LA) opioid prescribing patterns among ophthalmic plastic surgeons in the Centers for Medicare and Medicaid Services (CMS) Part D database. METHODS: A retrospective observational cohort study was conducted on oculoplastic surgeons in the CMS Part D database who prescribed ER/LA opioids from 2013 to 2017. American Society of Ophthalmic Plastics and Reconstructive Surgery (ASOPRS) and non-ASOPRS surgeons were analyzed as groups. Prescribers were also analyzed based on gender and practice experience. RESULTS: Oculoplastic surgeons (64 ASOPRS and 78 non-ASOPRS) were responsible for 1,177 ER/LA opioid prescriptions from 2013 to 2017. ASOPRS members accounted for 4.6% and non-ASOPRS members accounted for 7.5% of all ER/LA opioids prescribed by ophthalmologists over the study period (p= .02). The total number of ASOPRS and non-ASOPRS members prescribing ER/LA opioids decreased by 52% (p= .10) and 58% (p= .07) from 2013 to 2017 respectively. CONCLUSION: ER/LA opioids are indicated for treatment of chronic pain and may be appropriately prescribed by the oculoplastic surgeon in certain circumstances, however due to the higher risk of overdose injury, those circumstances must be defined and justified. While a relatively small number of oculoplastic surgeons (10.6% ASOPRS and 19.6% non-ASOPRS) prescribed ER/LA opioids from 2013 to 2017, non-ASOPRS oculoplastic surgeons wrote 23.5% more ER/LA opioid prescriptions over the study period. Over the 5-year study period there was a general decline in the prescribing of ER/LA opioids by oculoplastic surgeons. Reviewing the prescribing practices of oculoplastic specialists, regardless of professional affiliation, is necessary to understand the role of ER/LA opioids for all of ophthalmology.


Asunto(s)
Medicare Part D , Oftalmólogos , Cirujanos , Anciano , Analgésicos Opioides/uso terapéutico , Humanos , Pautas de la Práctica en Medicina , Estudios Retrospectivos , Estados Unidos
19.
J Craniofac Surg ; 33(1): 211-213, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-34292239

RESUMEN

ABSTRACT: The objective of this article is to compare the incidence of premature dislocation of silicone tubes and the effect on treatment success between monocanalicular (MCI) and bicanalicular (BCI) intubation in pediatric patients with simple congenital nasolacrimal duct obstruction. Retrospective comparative case series of 108 eyes of 78 pediatric patients with simple congenital nasolacrimal duct obstruction who underwent probing with either BCI (n = 38 eyes) or MCI (n = 70 eyes) from 2017 to 2020. Premature tube extrusion was defined as any tube removed prior to the 3 month postoperative appointment. Success was defined as resolution of tearing 3 months post tube removal. Ages ranged from 10 months to 5.35 years (mean, 1.95 years; Standard deviation (SD), 0.91). Premature tube extrusion occurred in 15 eyes with BCI and 29 eyes with MCI. Success rates were not significantly different regardless of intubation type between the planned tube removal (90.6%) and the premature tube extrusion cohorts (84.1%), P = 0.89. There was no significant difference in treatment success between the planned tube removal (92.7% MCI, 87% BCI) and the premature tube extrusion cohorts (86.2% MCI, 80% BCI). Complications included 2 infections (1 MCI, 1 BCI) and 2 cases of tube related keratopathy (1 MCI, 1 BCI) that all resolved with tube removal. There were 2 BCI patients that presented to the emergency department for premature tube extrusion. Silicone intubation regardless of stent type is an effective treatment for simple congenital nasolacrimal duct obstruction. There was no significant difference in treatment success between tubes that extrude prematurely, and tubes removed at term based on type of intubation.


Asunto(s)
Dacriocistorrinostomía , Obstrucción del Conducto Lagrimal , Conducto Nasolagrimal , Niño , Humanos , Lactante , Intubación , Obstrucción del Conducto Lagrimal/terapia , Conducto Nasolagrimal/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
20.
Optom Vis Sci ; 99(1): 31-34, 2022 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-34882610

RESUMEN

SIGNIFICANCE: This study aimed to determine the prescription rates and prescribing trends of opioids by optometrists in the Centers for Medicare & Medicaid Services (CMS) part D database from 2013 to 2017 and to assess opioid prescribing patterns of U.S. optometrists in the CMS part D database. METHODS: With internal review board approval, a retrospective observational cohort study was conducted on optometrists listed in the CMS part D database who prescribed opioids from 2013 to 2017. RESULTS: There was an average of 26,477 optometrists in the CMS database from 2013 to 2017, of which 5.9% prescribed opioids. Of those prescribing opioids, optometrists wrote an average of 5.9 opioid prescriptions per year. Those writing greater than 10 opioid prescriptions averaged 24.2 annually. Overall, of opioid prescribing optometrists, opioid prescriptions comprised 7% of prescriptions written per year. CONCLUSIONS: Most optometrists do not prescribe opioids, and the vast majority of those who do write few opioid prescriptions. The total number of optometrists prescribing opioids and the total number of opioid prescriptions declined from 2013 to 2017. Further investigation into the opioid prescribing practices by optometrists will help better understand specific pain needs, as opioid prescribing patterns may differ depending on patient population.


Asunto(s)
Medicare Part D , Optometristas , Anciano , Analgésicos Opioides/uso terapéutico , Prescripciones de Medicamentos , Humanos , Pautas de la Práctica en Medicina , Estudios Retrospectivos , Estados Unidos
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