Advanced Respiratory Failure Requiring Tracheostomy-A Marker of Unfavourable Prognosis after Heart Transplantation.

Advanced respiratory failure with tracheostomy requirement is common in heart recipients. The aim of the study is to assess the tracheostomy rate after orthotopic heart transplantation and identify the subgroups of patients with the highest need for tracheostomy and these groups' association with mortality at a single centre through a retrospective analysis of 140 consecutive patients transplanted between December 2012 and July 2018. As many as 28.6% heart recipients suffered from advanced respiratory failure with a need for tracheostomy that was performed after a median time of 11.5 days post-transplant. Tracheostomy was associated with a history of stroke (OR 3.4; 95% CI) 1.32-8.86; p = 0.012), previous sternotomy (OR 2.5; 95% CI 1.18-5.32; p = 0.017), longer cardiopulmonary bypass time (OR 1.01; 95% CI 1.00-1.01; p = 0.007) as well as primary graft failure (OR 6.79; 95% CI2.93-15.71; p < 0.001), need of renal replacement therapy (OR 19.2; 95% 2.53-146; p = 0.004) and daily mean SOFA score up to 72 h (OR 1.50; 95% 1.23-1.71; p < 0.01). One-year mortality was significantly higher in patients requiring a tracheostomy vs. those not requiring one during their hospital stay (50% vs. 16%, p < 0.001). The need for tracheostomy in heart transplant recipients was 30% in our study. Advanced respiratory failure was associated with over 3-fold greater 1-year mortality. Thus, tracheostomy placement may be regarded as a marker of unfavourable prognosis.

Debunking the Puzzle of Eyelid Apraxia: The Muscle of Riolan Hypothesis.

PURPOSE: Apraxia of eyelid opening (AEO) has been defined by the presence of an intermittent nonparalytic bilateral loss of the volitional ability to open the eyes or to maintain the eyelids in a sustained elevated position. It is not known whether the condition represents an apraxia, a dystonia, or a freezing phenomenon, and several different nomenclatorial terms have been suggested for this condition including the so-called AEO (scAEO), blepahrocolysis, focal eyelid dystonia, and so on. The primary goal of this review is to attempt to clarify the pathogenetic mechanisms underlying scAEO as a clinical phenomenon. This review also addresses the issue of whether scAEO is part of the spectrum of blepharospasm (BSP) which includes BSP, dystonic blinks and other dystonic eyelid conditions, or whether it is a separate phenomenologically heterogeneous disease with clinical features that merely overlap with BSP. METHODS: A literature review was conducted in PubMed, MEDLINE, PubMed Central (PMC), NCBI Bookshelf, and Embase for several related keywords including the terms "apraxia of eyelid opening," "pretarsal blepharospasm," "blepharocolysis," "eyelid freezing," "eyelid akinesia," "levator inhibition," "blepharospasm-plus," as well as "blepharospasm." The clinical findings in patients with scAEO who fulfilled the classic diagnostic criteria of the disease that were originally set by Lepore and Duvoisin were included, while patients with isolated blepharospasm or dystonic blinks (DB) were excluded. In addition, electromyographic (EMG) studies in patients with scAEO were reviewed in detail with special emphasis on studies that performed synchronous EMG recordings both from the levator muscle (LPS) and the pretarsal orbicularis oculi muscle (OO). RESULTS: The apraxia designation is clearly a misnomer. Although scAEO behaves clinically as a hypotonic freezing phenomenon, it also shares several cardinal features with focal dystonias. The authors broadly categorized the EMG data into 3 different patterns. The first pattern (n = 26/94 [27.6%]) was predominantly associated with involuntary discharges in the OO muscle and has been termed pretarsal blepharospasm (ptBSP). The commonest pattern was pattern no. 2 (n = 53/94 [56.38%]), which was characterized by involuntary discharges in the OO muscle, together with a disturbed reciprocal innervation of the antagonist levator muscle and is dubbed disturbed reciprocal innervation (DRI). This EMG pattern is difficult to discern from the first pattern. Pattern no. 3 (n = 15/94 [15.9%]) is characterized by an isolated levator palpebrae inhibition (ILPI). This levator silence was observed alone without EMG evidence of contractions in the pretarsal orbicularis or a disturbed reciprocal relation of both muscles. CONCLUSION: EMG evidence shows that the great majority (84%) of patients show a dystonic pattern, whereas ILPI (16%) does not fit the dystonic spectrum. The authors propose that a spasmodic contraction of the muscle of Riolan may be the etiological basis for levator inhibition in patients with ILPI. If this is true, all the 3 EMG patterns observed in scAEO patients (ptBSP, DRI, and ILPI) would represent an atypical form of BSP. The authors suggest coining the terms Riolan muscle BSP ( rmBSP ) for ILPI, and the term atypical focal eyelid dystonia ( AFED ) instead of the term scAEO, as both terms holistically encompass both the clinical and EMG data and concur with the authors' theorem.

Prognostic significance of serum lactate following cardiac transplantation.

Aim: Hyperlactatemia is common post-heart transplantation. Lactate measurements in the first 24 h were analyzed with respect to mortality. Methods: A total of 153 consecutive cardiac transplant patients were reviewed. Recipients of organs maintained in a state of ex vivo perfusion were included. Results: A total of 143 heart recipients were included. Hyperlactatemia (>2 mmol/l) was present in all patients. Despite maximum lactate and lactate clearance being significantly higher in nonsurvivors (p = 0.002, p = 0.004), neither receiver operator curve analysis nor multivariate logistic regression showed association with 1-year mortality. In comparison, the minimum lactate was significantly associated with mortality (area under the curve 0.728 [p < 0.001]; odds ratio 1.28 [95% 1.01-162; p = 0.04]). Conclusion: The minimum lactate, a surrogate of persistent hyperlactatemia, was demonstrated to be superior compared with maximum lactate and lactate clearance in determining patient prognosis.

Orbital Vascular Anomalies: A Nomenclatorial, Etiological, and Nosologic Conundrum.

PURPOSE: Vascular anomalies are a heterogeneous group of disorders that frequently present in the periorbital region. They encompass 2 broad entities: vascular tumors, which possess a proliferative endothelium, and vascular malformations, which are basically localized defects of vascular morphogenesis. The primary goal of this review was to address inaccurate or controversial terminology in the oculoplastic literature concerning orbital and periorbital vascular anomalies and to categorize these lesions in an abridged and simplified hierarchical list that adheres as much as possible to the most recent (2018) iteration for the classification of vascular lesions proposed by the International Society for the Study of Vascular Anomalies (ISSVA). The secondary goal of this review was to review and update information regarding the genetic underpinnings of vascular anomalies and the downstream signaling pathways that are subsequently affected as a result of these genetic errors. METHODS: A literature review was conducted in PubMed, MEDLINE, PubMed Central, National Center for Biotechnology Information Bookshelf, and Embase for several related keywords including "vascular anomalies, vascular malformations, vascular tumors, and cavernous venous malformation," both with and without adding the keywords "eyelid," "orbital," and "periorbital." In addition, a detailed search was conducted for controversial or obsolete keywords like "cavernous hemangioma," "lymphangioma," and "varices," again in their systemic and orbital/periorbital context. RESULTS: Crucial issues in the 2018 ISSVA classification regarding the proper categorization of orbital vascular anomalies, particularly venous lesions, were critically evaluated and revised, and a regional, simplified, and abridged modification of the ISSVA 2018 classification was proposed. CONCLUSIONS: Interdisciplinary and intradisciplinary dialogue concerning orbital vascular anomalies is seriously compromised due to the lack of a unanimous agreement on terminology and the absence of a unified classification concept system. The authors recommend that oculoplastic surgeons adopt ISSVA terminology whenever technically possible and scientifically sound. However, they also propose modifying the ISSVA 2018 classification specifically to adapt to the peculiarities of vascular anomalies in the periorbital region. At present, the simplified classification proposed here is a preliminary first step towards managing patients with orbital vascular anomalies with greater diagnostic and therapeutic precision, until such time in the future when the entire genetic makeup of orbital vascular anomalies is more completely elucidated. Optimistically, this could pave the way for a more robust classification and the ultimate therapeutic cure.

The Ruptured Globe, Sympathetic Ophthalmia, and the 14-Day Rule.

PURPOSE: Prophylactic enucleation of a ruptured globe with no light perception within 14 days of injury to prevent sympathetic ophthalmia (SO) has been an established dictum in academic teaching for more than 100 years. This treatment strategy was originally based on observation, speculation, and careful thought, but there was never any scientific proof. This review summarizes and updates the current state of our knowledge about globe rupture and SO, examines the origin and validity of the 14-day rule, and emphasizes the importance of trying to save the traumatized eye whenever possible. METHODS: A comprehensive literature review of SO and globe rupture was performed. RESULTS: SO is a rare disorder that may potentially occur following traumatic globe rupture as well as following a variety of other intraocular surgeries. Vitreoretinal surgery may be a more common cause than trauma according to some studies. SO may still occur despite having the eye removed within 14 days of the trauma. A variety of new medications including biologic agents are now available to treat SO with improved efficacy in suppressing the associated ocular inflammation and allowing retention of some useful vision. Removing the traumatized, blind eye may have other important psychological consequences associated with it that require consideration before eye removal is carried out. Retaining the blind, phthisical, disfigured eye avoids phantom vision and phantom pain associated with enucleation as well as providing a good platform to support and move an overlying prosthetic eye. Data on the occurrence of SO following evisceration and enucleation with and without predisposing factors confirms the exceedingly low risk. CONCLUSION: Most civilian open globe injuries can be successfully repaired with modern, advanced microsurgical techniques currently available. Because of the exceedingly low risk of SO, even with the severity of open globe trauma during military conflicts being more devastating as a result of the blast and explosive injuries, today every attempt is made to primarily close the eye rather than primarily enucleate it, providing there is enough viable tissue to repair. The 14-day rule for eye removal after severe globe ruptures is not scientifically supported and does not always protect against SO, but the safe time period for prophylactic eye removal is not definitively known. In the exceptional cases where SO does occur, several new medications are now available that may help treat SO. We advocate saving the ruptured globe whenever possible and avoiding prophylactic enucleation to prevent the rare occurrence of SO. When an eye requires removal, evisceration is an acceptable alternative to enucleation in cases that do not harbor intraocular malignancy.

Clinical characteristics and outcomes of critically ill patients with COVID-19 admitted to an intensive care unit in London: A prospective observational cohort study.

BACKGROUND: Cohorts of severely ill patients with COVID-19 have been described in several countries around the globe, but to date there have been few published reports from the United Kingdom (UK). Understanding the characteristics of the affected population admitted to intensive care units (ICUs) in the UK is crucial to inform clinical decision making, research and planning for future waves of infection. METHODS: We conducted a prospective observational cohort study of all patients with COVID-19 admitted to a large UK ICU from March to May 2020 with follow-up to June 2020. Data were collected from health records using a standardised template. We used multivariable logistic regression to analyse the factors associated with ICU survival. RESULTS: Of the 156 patients included, 112 (72%) were male, 89 (57%) were overweight or obese, 68 (44%) were from ethnic minorities, and 89 (57%) were aged over 60 years of age. 136 (87%) received mechanical ventilation, 77 (57% of those intubated) were placed in the prone position and 95 (70% of those intubated) received neuromuscular blockade. 154 (99%) patients required cardiovascular support and 44 (28%) required renal replacement therapy. Of the 130 patients with completed ICU episodes, 38 (29%) died and 92 (71%) were discharged alive from ICU. In multivariable models, age (OR 1.13 [95% CI 1.07-1.21]), obesity (OR 3.06 [95% CI 1.16-8.74]), lowest P/F ratio on the first day of admission (OR 0.82 [95% CI 0.67-0.98]) and PaCO2 (OR 1.52 [95% CI 1.01-2.39]) were independently associated with ICU death. CONCLUSIONS: Age, obesity and severity of respiratory failure were key determinants of survival in this cohort. Multiorgan failure was prevalent. These findings are important for guiding future research and should be taken into consideration during future healthcare planning in the UK.

Perioperative Prophylactic Antibiotics in 1,250 Orbital Surgeries.

PURPOSE: Intravenous antibiotic prophylaxis is used for many clean-contaminated surgeries or clean surgeries with an implant, but its value for clean orbital surgery has not been determined. This study investigated infection risks and adverse effects related to antibiotics in patients undergoing orbital surgery. METHODS: A prospective, nonrandomized comparative case series of all patients undergoing orbital surgery with participating surgeons between October 1, 2013, and March 1, 2015. Types of surgery, antibiotic regimens, corticosteroid use, antibiotic side effects, and surgical site infections (SSIs) were entered into an electronic database and subsequently analyzed. Cases in which patients received postoperative oral antibiotics were analyzed separately. RESULTS: Of 1,250 consecutive orbital surgeries, 1,225 met inclusion criteria. A total of 1208 patients were included in the primary analysis: 603 received no antibiotic prophylaxis (group A), and 605 received a single dose of intravenous antibiotic (group B). Five patients (0.42%) developed an SSI, 3 in group A and 2 in group B. The difference in SSI rates was not statistically significant between the 2 groups (p = 0.66). Antibiotic prophylaxis, alloplastic implants, paranasal sinus entry, and corticosteroid use were not associated with differences in SSI rates. All SSIs resolved on a single course of oral antibiotics; an implant was removed in 1 case. There were no complications associated with a single dose of intravenous prophylaxis. However, 12% of 17 patients (group C) who received 1 week of oral postoperative prophylactic antibiotics developed antibiotic-related complications (diarrhea, renal injury), yielding a number needed to harm of 8.5. CONCLUSIONS: In this large series, antibiotic prophylaxis does not appear to have reduced the already low incidence of SSI following orbital surgery. Given the detriments of systemic antibiotics, the rarity of infections related to orbital surgery, and the efficacy of treating such infections should they occur, patients undergoing orbital surgery should be educated to the early symptoms of postoperative infection and followed closely, but do not routinely require perioperative antibiotics.

Lessons of the month 1: Fatal oesophago-pericardial fistula with cerebral air embolism after elective atrial fibrillation ablation.

Oesophago-pericardial fistula following any electrophysiological procedure is a rare, and potentially, life-threatening condition. Initial presentation can easily be misdiagnosed, as symptoms vary and are not specific. Echocardiography is an invaluable tool to diagnose and rule out complications. We present the case of a 68-year-old patient who developed an oesophago-pericardial fistula complicated with purulent pericarditis, sepsis and cerebral air embolism. In conclusion, this case report encourages physicians to use strategies that may help with early diagnosis and lead to potential lifesaving interventions.

An unusual case of idiopathic calcinosis of the eyelid.

Idiopathic subepidermal calcinosis, also known as idiopathic calcinosis cutis, is a rare condition, which typically presents as a small, hard, subepithelial mass in the absence of systemic disease and trauma. We report on an atypical case of idiopathic calcinosis, which appeared in an otherwise healthy 61-year-old female along the tarsus of her right upper eyelid. The purpose of this case report is to demonstrate idiopathic calcinosis in an unusual location that is deep to the dermis and to discuss the vast differential diagnosis of this entity.

Embryologic and Fetal Development of the Human Orbit.

PURPOSE: To review the recent data about orbital development and sort out the controversies from the very early stages during embryonic life till final maturation of the orbit late in fetal life, and to appreciate the morphogenesis of all the definitive structures in the orbit in a methodical and timely fashion. METHODS: The authors extensively review major studies detailing every aspect of human embryologic and fetal orbital morphogenesis including the development of extraocular muscles, orbital fat, vessels, nerves, and the supportive connective tissue framework as well as bone. These interdisciplinary studies span almost a century and a half, and include some significant controversial opposing points of view which the authors hopefully sort out. The authors also highlight a few of the most noteworthy molecular biologic studies regarding the multiple and interacting signaling pathways involved in regulating normal orbital morphogenesis. RESULTS: Orbital morphogenesis involves a successive series of subtle yet tightly regulated morphogenetic events that could only be explained through the chronological narrative used by the authors. The processes that trigger and contribute to the formation of the orbits are complex and seem to be intricately regulated by multifaceted interactions and bidirectional cross-talk between a multitude of cellular building raw materials including the developing optic vesicles, neuroectoderm, cranial neural crest cells and mesoderm. CONCLUSIONS: Development of the orbit is a collective enterprise necessitating interactions between, as well as contributions from different cell populations both within and beyond the realm of the orbit. A basic understanding of the processes underlying orbital ontogenesis is a crucial first step toward establishing a genetic basis or an embryologic link with orbital disease.

Can the use of an age-adjusted D-dimer cut-off value help in our diagnosis of suspected pulmonary embolism? .

Safe exclusion of pulmonary embolism (PE) is a common problem in acute medicine. Common care pathways usually involve the use of a pre-test probability score with a D-dimer test to aid clinical decision-making. Unfortunately, the specificity of many D-dimer assays decreases with age. This study investigates the role of an age-adjusted D-dimer of 5 x patient's age when the conventional cut-off of the assay is 230 ng/mL. Data was collected retrospectively over a 12-month period from patients who went on to have either computed tomography pulmonary angiography (CTPA) or pulmonary ventilation/perfusion (V/Q) imaging. D-dimers in patients with low or moderate Wells score were analysed for both conventional and age-adjusted cut-offs. The use of an adjusted D-dimer showed a sensitivity of 0.97 (95% CI 0.9-1.0) while the specificity increased from 0.07 (95% CI 0.04-0.11) for the conventional cut-off to 0.32 (95% CI 0.27-0.38) for the age-adjusted cut-off. Using a 5 x patient's age-adjusted D-dimer cut-off is both safe and showed an increased specificity comparable to those published previously on other D-dimer assays.

Anatomic Considerations in Thyroid Eye Disease.

PURPOSE: To review and summarize the clinical findings in thyroid eye disease (TED) related to the unique anatomical structures of the eyelids and orbit. METHODS: A PubMed search was performed searching for anatomical relationships between eyelid and orbital anatomy and the clinical findings of TED. RESULTS: The major clinical findings associated with TED are varied. They typically involve both the eyelid and the orbit. In the eyelid, usual findings include upper eyelid retraction, contour abnormalities, eyelid edema, prolapsed orbital fat, conjunctival injection and chemosis, caruncular edema, and meibomian gland dysfunction. The exact causes of these changes remain a matter of controversy, but numerous hypotheses have been proposed, most with limited experimental support. In the orbit, inflammation and congestion characterize the acute active phase, with tissue expansion and fibrosis persisting into the chronic inactive phase. All of these findings result, at least in part, from the unique anatomy of the eyelids and orbit and their interaction with the immunologic processes underlying TED. Here we review these major characteristics of TED, with special reference to their anatomic relationships. CONCLUSIONS: The major findings characterizing TED are related to unique anatomic features in the eyelid, including Müller muscle, the levator palpebrae superioris muscle, and suspensory ligaments of the conjunctiva. In the orbit, Graves-related remolding results from the special physiologic features of the extraocular muscles, the structure of the orbital walls and their relations to adjacent paranasal sinuses, and the reaction of orbital fat to immunologic challenges.

Soft Tissue Metrics in Thyroid Eye Disease: An International Thyroid Eye Disease Society Reliability Study.

PURPOSE: To determine the reliability of 3 scales for assessing soft tissue inflammatory and congestive signs associated with thyroid eye disease. METHODS: This was a multicentered prospective observational study, recruiting 55 adults with thyroid eye disease from 9 international centers. Six thyroid eye disease soft tissue features were measured; each sign graded using 3 scales (presence/absence [0-1], 3-point scale [0-2], and percentage [0-100]). Each eye was graded twice by 2 independent raters. Accuracy (fraction of agreement) was calculated between the 2 trials for each rater (intrarater reliability) and between raters for all trials (interrater reliability) to determine the most sensitive scale for each feature that maintained a threshold of agreement greater than 0.70. Trial, intrarater reliability, and interrater reliability were determined by accuracy measurement of agreement for each inflammatory/congestive feature. RESULTS: Fifty-five patients had 218 assessments for 6 thyroid eye disease metrics. The intrarater reliability for each feature was consistently better than the interrater reliabilities. Using an agreement of 0.70 or better, for the interrater tests, conjunctival and eyelid edema could be reliably measured using the 0-1 or 0-2 scale while conjunctival and eyelid redness could only be reliably measured with the binary 0-1 scale. Caruncular edema and superior conjunctival redness could not be measured reliably between 2 raters with any scale. The percentage scale had poor agreement unless slippage intervals of >20% were allowed on either side of the measurements. CONCLUSIONS: Of the specific periocular soft tissue inflammatory features measured between raters in the Clinical Activity Score and Vision, Inflammation, Strabismus, Appearance scales, edema of the eyelids and conjunctiva could reliably be measured by both 0-1 and 0-2 scales, erythema of the eyelid and bulbar conjunctiva could reliably be measured only by the 0-1 scale, and the other parameters of superior bulbar erythema and caruncular edema were not reliably measured by any scale.

Seminoma Metastatic to the Orbit.

PURPOSE: Seminomas are solid tumors in young men, but which rarely metastasize to the orbit. The authors review the known literature on seminoma metastatic to the orbit, and describe an additional case in a 33-year-old man. METHODS: A literature search was performed on the MEDLINE database using keywords "seminoma," "testicular germ-cell tumors," "testicular cancer," "testicular neoplasm," "orbital metastasis," and "germ-cell neoplasms." RESULTS: Malignant neoplasms of the testis account for only 1% of cancers in men. None-the-less, testicular germ cell seminoma is the most common solid tumor found in young men between the ages of 15 and 39. Only seven previous cases have been mentioned in the literature. The pathogenesis remains unclear although genetic, environmental, and maternal factors may play a role. The number of cases is too few to determine the best treatment options, but surgical excision and adjunctive orbital radiotherapy appear to be most appropriate. CONCLUSIONS: Although metastases to the orbit are rare, seminoma should be considered in the differential diagnosis of all young men with proptosis.