Descemet-Stripping Automated Endothelial Keratoplasty.

PURPOSE: To evaluate the speed of visual recovery in 16 consecutive patients with corneal endothelial dysfunction who received Descemetstripping automated endothelial keratoplasty (DSAEK). METHODS: This is a retrospective study of a novel method for smallincision endothelial transplantation (DSAEK). Endothelial replacement was accomplished with Descemet stripping of the recipient and insertion of a posterior donor tissue that had been prepared with a microkeratome. Best spectacle-corrected visual acuity (BSCVA) by manifest refraction, endothelial counts, and dislocation rates were measured up to 12 months after DSAEK. RESULTS: Sixteen consecutive patients underwent uncomplicated DSAEK. Three patients had known optic nerve or macular disease precluding vision better than 20/200. Of the remaining 14 patients, 11 had BSCVA of 20/40 by postoperative week 12 (7 by week 6). The remaining 2 were 20/50 by weeks 6 and 12. All 14 patients were 20/40 or better at 1 year. One patient had a primary graft failure, and surgery was repeated with 20/40 BSCVA at 1 year. The dislocation rate was 25%. The average cell count between 7 and 10 months was 1714. The average pachymetry was 682. CONCLUSION: DSAEK surgery allows rapid, excellent BSCVAvisual recovery. The rate of visual recovery is more rapid than usually found with penetrating keratoplasty.

Recovery of Contrast Sensitivity After Descemet Membrane Endothelial Keratoplasty.

PURPOSE: To study the change in contrast sensitivities in eyes with Fuchs endothelial dystrophy and bullous keratopathy after Descemet membrane endothelial keratoplasty (DMEK). METHODS: In this prospective study, 50 pseudophakic eyes of 50 patients who received DMEK surgery at the Charité-Universitätsmedizin Berlin were included. Visual acuity; contrast sensitivity using OPTEC 6500 at spatial frequencies of 1.5, 3, 6, 12, and 18 cycles/degree in photopic and mesopic light with and without glare; central corneal thickness (CCT); and anterior and posterior corneal aberrations were measured preoperatively and at 3 and 12 months postoperatively. RESULTS: Best-corrected visual acuity (preoperative 0.67 ± 0.46 and after 12 months 0.19 ± 0.16 LogMAR, P < 0.001) and photopic and mesopic contrast sensitivities with and without glare improved significantly, whereas CCT decreased significantly (preoperative 677 ± 114 µm, after 12 months 527 ± 29 µm, P < 0.001). Preoperative CCT correlates significantly with preoperative photopic contrast sensitivity (correlation coefficient -0.462, P = 0.002), and postoperative total anterior aberrations correlates with postoperative photopic contrast sensitivity (correlation coefficient -0.361, P = 0.006). CONCLUSIONS: Photopic and mesopic contrast sensitivities, especially with glare, are impaired in patients with Fuchs endothelial dystrophy and bullous keratopathy. The extent of the corneal thickening seems to mainly influence the contrast sensitivity preoperatively. DMEK surgery improves the contrast sensitivity significantly. However, higher postoperative anterior corneal aberrations limit the postoperative contrast sensitivities.

Techniques, Outcomes, and Complications of Preloaded, Trifolded Descemet Membrane Endothelial Keratoplasty Using the DMEK EndoGlide.

PURPOSE: To describe 2 insertion techniques, outcomes, and complications of preloaded, trifolded Descemet membrane endothelial keratoplasty (DMEK) cases using the DMEK EndoGlide inserter. METHODS: This retrospective, consecutive case series analyzed the first 35 cases using the DMEK EndoGlide performed between October 2018 and October 2019 at a single center. Preloaded, trifolded DMEK tissues were delivered through a fluid-injected or pull-through technique. To inject the tissue, a burst of fluid was delivered into the lumen of the injector with a second instrument. Postoperatively, best-spectacle corrected visual acuity (BSCVA), pachymetry, graft survival, and complications were assessed. RESULTS: Thirty-five eyes of 29 patients underwent DMEK alone (n = 11), with cataract surgery (n = 21), or with additional surgeries (n = 3). Of these, 19 (54.3%) grafts were injected. Video analysis revealed a median time of 3.5 minutes from graft insertion to opening for gas insertion. Median preoperative BSCVA of 0.398 logMAR improved to 0.097 logMAR (P = 0.02) at 9 months. Median pachymetry decreased from 619 µm to 551 µm (P = 0.03) at 9 months. Median donor endothelial cell count of 2890.5 cells/mm2 reduced to 2123 cells/mm2 (26.6% endothelial cell loss; P = 0.008) 6 months postoperatively. One (2.9%) graft failed due to inverted marking at the eye bank and subsequent reverse implantation. CONCLUSIONS: Pre-loaded, tri-folded tissues can be implanted with acceptable levels of endothelial cell loss. We describe a no-touch method of injecting pre-loaded, tri-folded tissue and highlight incorrect marking as a potential complication. This may not be identifiable intraoperatively due to lack of scroll formation.

Treatment strategy for primary lung cancer in a lung highly compressed by giant emphysematous bullae: A case report.

Lung cancer sometimes develops on the wall of a giant emphysematous bulla (GEB). Herein, we describe a rare case in which lung cancer developed in lung tissue compressed by GEBs. A 62-year-old man underwent a computed tomography (CT) scan that revealed two right GEBs. A tumor was suspected in the highly compressed right upper lobe. Since the right bronchus was significantly shifted toward the mediastinum, it was difficult to perform a bronchoscopy. We inserted thoracic drains into the GEBs, and a subsequent CT scan revealed re-expansion of the remaining right lung and a 3.3 cm tumor in the right upper lobe. The shift of the right bronchus was improved, and bronchoscopy was performed. The tumor was diagnosed as non-small cell lung cancer (NSCLC). Additionally, the GEBs were found to have originated from the right lower lobe. We performed a right upper lobectomy, mediastinal lymph node dissection, and bullectomy of the GEBs via video-assisted thoracoscopic surgery. In preoperative evaluation of a GEB, assessing re-expansion and lung lesions of the remaining lung is important, and intracavity drainage of a GEB may be useful. KEY POINTS: Significant findings of the study Cancer that develops in lung tissue highly compressed by a giant emphysematous bulla is difficult to diagnose. In the preoperative evaluation of a giant emphysematous bulla, assessing re-expansion and lung lesions of the remaining lung is important. What this study adds After performing intracavity drainage of a giant emphysematous bulla, the remaining lung re-expands, and the bronchial shift improves; subsequently, bronchoscopy makes it possible to diagnose lung cancer in the remaining lung.

Osteoporosis in Skin Diseases.

Osteoporosis (OP) is defined as a generalized skeletal disease characterized by low bone mass and an alteration of the microarchitecture that lead to an increase in bone fragility and, therefore, an increased risk of fractures. It must be considered today as a true public health problem and the most widespread metabolic bone disease that affects more than 200 million people worldwide. Under physiological conditions, there is a balance between bone formation and bone resorption necessary for skeletal homeostasis. In pathological situations, this balance is altered in favor of osteoclast (OC)-mediated bone resorption. During chronic inflammation, the balance between bone formation and bone resorption may be considerably affected, contributing to a net prevalence of osteoclastogenesis. Skin diseases are the fourth cause of human disease in the world, affecting approximately one third of the world's population with a prevalence in elderly men. Inflammation and the various associated cytokine patterns are the basis of both osteoporosis and most skin pathologies. Moreover, dermatological patients also undergo local or systemic treatments with glucocorticoids and immunosuppressants that could increase the risk of osteoporosis. Therefore, particular attention should be paid to bone health in these patients. The purpose of the present review is to take stock of the knowledge in this still quite unexplored field, despite the frequency of such conditions in clinical practice.

Secondary spontaneous pneumothorax and bullous lung disease in cannabis and tobacco smokers: A case-control study.

BACKGROUND: The notion that smoking cannabis may damage the respiratory tract has been introduced in recent years but there is still a paucity of studies on this subject. The aim of this study was to investigate the relationship between cannabis smoking, pneumothorax and bullous lung disease in a population of operated patients. METHODS AND FINDINGS: We performed a retrospective study on patients operated on for spontaneous pneumothorax. Patients were divided into three groups according to their smoking habit: cannabis smokers, only-tobacco smokers and nonsmokers. Cannabis lifetime exposure was expressed in dose-years (1d/y = 1 gram of cannabis/week for one year). Clinical, radiological and perioperative variables were collected. The variables were analyzed to find associations with smoking habit. The impact of the amount of cannabis consumption was also investigated by ROC curves analysis. Of 112 patients, 39 smoked cannabis, 23 smoked only tobacco and 50 were nonsmokers. Median cannabis consumption was 28 dose/years, median tobacco consumption was 6 pack/years. Cannabis smokers presented with more severe chronic respiratory symptoms and bullous lung disease and with a higher incidence of tension pneumothorax than both tobacco smokers and nonsmokers. Cannabis smokers also developed a larger pneumothorax, experienced prolonged postoperative stay and demonstrated a higher incidence of pneumothorax recurrence after the operation than nonsmokers did. The risk of occurrence of chronic respiratory symptoms and bullous lung disease in cannabis smokers was dose-related. CONCLUSIONS: Cannabis smoking seems to increase the risk of suffering from respiratory complaints and can have detrimental effects on lung parenchyma, in a dose-dependent manner. Cannabis smoking also negatively affected the outcome of patients operated for spontaneous pneumothorax. A history of cannabis abuse should always be taken in patients with pneumothorax. There may be need for a specific treatment for pneumothorax in cannabis smokers.

Epidermolysis Bullosa Patients' Perception of Surgical Wound and Scar Healing.

BACKGROUND: There is limited evidence to suggest patients with epidermolysis bullosa (EB) have more postoperative wound complications than the general population. Despite this, the authors have noted reluctance among some surgeons to operate on these patients. OBJECTIVE: A cross-sectional study was designed to investigate postoperative wound and scar healing outcomes in patients with EB. METHODS: Patients were asked to complete the "Surgical Wound and Scar Healing in EB" questionnaire, and data gathered were analyzed. RESULTS: Forty-six patients completed the questionnaire for a total of 94 different surgical procedures. Five patients reported blistering at the surgical wound site. All 5 had generalized forms of EB. Four patients reported wound infections, and 1 patient reported wound dehiscence. The postoperative scar healed with keloid or hypertrophic scarring after 26% of the reported surgical procedures. CONCLUSION: Blistering at the postoperative site seems to be uncommon and particularly unlikely to occur in localized forms of EB. Postoperative wound infections and dehiscence are uncommon. Patients with EB may have a propensity to develop keloid or hypertrophic scarring. With these data, the authors hope clinicians have greater confidence in referring patients with EB for surgery, and surgeons more reassured about postoperative wound healing.

Photo Rounds: Painful blisters on fingertips and toes.

Our patient had visited the emergency department for painful blisters on her fingertips and toes. A follow-up visit to our clinic unearthed the cause.

Longitudinal Changes in Bleb Height, Vascularity, and Conjunctival Microcysts After Trabeculectomy.

OBJECTIVE: The objective of this article was to study longitudinal changes in bleb morphology (presence of microcysts and bleb height and vascularity) in subjects enrolled in the Singapore 5-Fluorouracil (5-FU) trabeculectomy study. STUDY DESIGN: Retrospective subgroup analysis of a randomized controlled trial. MATERIALS AND METHODS: Following trabeculectomy with either 5-FU or placebo, subjects were reviewed at day 1, weeks 1, 2, and 3, and months 3, 6, 9, 12, 16, 20, 24, 28, 32, and 36 and intraocular pressure (IOP), presence of microcysts and bleb height and vascularity graded and recorded. Statistical analyses were used to investigate baseline factors (demographics, eye drops use, IOP, treatment type, and comorbidities) that might account for long-term bleb changes and assess the influence of bleb morphology on mean, maximum, minimum, and variation in IOP following trabeculectomy. RESULTS: One hundred fifty-nine (61.2%) subjects completed follow-up and 71 subjects (44.7%) received 5-FU. Proportion of subjects with microcysts increased posttrabeculectomy till weeks 3 and 6 in 5-FU and placebo-treated eyes, respectively, before decreasing. Presence of microcysts at day 1 and week 2 was associated with lower mean (P=0.027 to 0.033), maximum (P=0.040), and minimum IOP (P=0.010). Mean bleb height decreased over 36 months and at months 20 and 36 was associated with maximum (P=0.031) and minimum IOP (P=0.032), respectively. Mean bleb vascularity decreased till month 9 and remained fairly constant after. CONCLUSIONS: Microcysts seen early posttrabeculectomy is associated with lower IOP. Blebs reduce in height as they remodel and the relationship between bleb vascularity and IOP is complex.

Acute COPD exacerbation presenting with pronounced intrabullous haemorrhage and haemoptysis.

A 54-year-old man with history of chronic obstructive pulmonary disease (COPD) presented with subacute onset of chest pain, shortness of breath, productive cough with haemoptysis and night sweats. There were no fever or recent weight loss reported. The chest radiograph showed right upper lobe bullae with adjacent opacification and an emphysematous lung. Due to worsening haemoptysis and persistent chest pain, CT of the chest with contrast was performed, which revealed moderate to severe emphysema and numerous blood-filled bullae. Cardiac work-up for chest pain was negative for myocardial ischaemia and for aortic dissection. Further infectious work-ups for mycobacterial and invasive fungal infection were negative. The patient was treated for acute COPD exacerbation and responded well to the antibiotics with the resolution of haemoptysis. Follow-up CT of the chest revealed the gradual resolution of the haemorrhage, while the patient remained asymptomatic.

Suction blister lesions and epithelialization monitored by optical coherence tomography.

BACKGROUND/PURPOSE: Our objective was to assess epithelialization of suction blister lesions by optical coherence tomography (OCT) and benchmark it to histology using epidermal thickness (ET) as the primary outcome. METHODS: Thirty-two healthy volunteers were recruited to Study 1 and 2. One 10-mm suction blister was raised on each buttock, and the blister roof was excised. Lesions were covered with moisture-retaining dressing. In Study 1, the lesions were OCT-scanned on day 0 (D0), D2 and D4 and excised for histological examination. In Study 2, the progress of epithelialization and skin barrier function were monitored to D14. RESULTS: ET increased from D0 to D2 by 46 µm (P<.001) and from D2 to D4 by 19 µm (P=.004). Compared with histology, OCT overestimated the presence of the epithelium (P<.0001) and ET on D4. Reliable measurements were obtained when the ET of the lesions reached the ET of the normal epidermis from D5-D7 and onwards. The ET development was reflected in decreased transepidermal water loss. CONCLUSION: We found that the OCT technique was poorly discriminative with respect to the neoepithelium and the moist lesion surface material in the early postinjury period. In the later stages, OCT seemed valuable for estimating the advancement of epithelialization.

A Missed Late Presentation of a Congenital Pulmonary Airway Malformation as a Large Infected Bulla.

A congenital pulmonary airway malformation (CPAM) is a rare cystic anomaly that may occur during development of the fetal airways. The vast majority of CPAMs are detected in neonates; as such, it is unusual for diagnosis to occur in adulthood. We report a 21-year-old male patient who presented to the emergency department of the Hospital Ampang, Kuala Lumpur, Malaysia, in 2015 with chest pain, breathlessness and tachypnoea. Based on an initial chest X-ray, the patient was misdiagnosed with pneumothorax and underwent urgent chest tube insertion; however, his condition deteriorated over the course of the next three days. Further imaging was suggestive of infected bullae or an undiagnosed CPAM. The patient therefore underwent video-assisted thoracoscopic surgery, during which a large infected bulla was resected. A diagnosis of an infected CPAM was confirmed by histopathological examination. Following the surgery, the patient recovered quickly and no bullae remnants were found at a one-month follow-up.

Current status of the treatment of blood blister-like aneurysms of the supraclinoid internal carotid artery: A review.

Currently, the treatment of blood blister-like aneurysms (BBAs) of the supraclinoid internal carotid artery (ICA) is challenging and utilizes many therapeutic methods, including direct clipping and suturing, clipping after wrapping, clipping after suturing, coil embolization, stent-assisted coil embolization, multiple overlapping stents, flow-diverting stents, covered stents, and trapping with or without bypass. In these therapeutic approaches, the optimal treatment method for BBAs has not yet been defined based on the current understanding of BBAs of the supraclinoid ICA. Therefore, in this study, we aimed to review the literature from PubMed to discuss and analyze the pros and cons of the above approaches while adding our own viewpoints to the discussion. Among the surgical methods, direct clipping was the easiest method if the compensation of the collateral circulation of the intracranial distal ICA was sufficient or direct clipping did not induce stenosis in the parent artery. In addition, the clipping after wrapping technique should be chosen as the optimal surgical modality to prevent rebleeding from these lesions. Among the endovascular methods, multiple overlapping stents (≥3) with coils may be a feasible alternative for the treatment of ruptured BBAs. In addition, flow-diverting stents appear to have a higher rate of complete occlusion and a lower rate of retreatment and are a promising treatment method. Finally, when all treatments failed or the compensation of the collateral circulation of the intracranial distal ICA was insufficient, the extracranial-intracranial (EC-IC) arterial bypass associated with surgical or endovascular trapping, a complex and highly dangerous method, was used as the treatment of last resort.

Amino acid duplication in the coiled-coil structure of collagen XVII alters its maturation and trimerization causing mild junctional epidermolysis bullosa.

The function and stability of collagens depend on the accurate triple helix formation of three distinct polypeptide chains. Disruption of this triple-helical structure can result in connective-tissue disorders. Triple helix formation is thought to depend on three-stranded coiled-coil oligomerization sites within non-collagenous domains. However, only little is known about the physiological relevance of these coiled-coil structures. Transmembrane collagen XVII, also known as 180 kDa bullous pemphigoid antigen provides mechanical stability through the anchorage of epithelial cells to the basement membrane. Mutations in the collagen XVII gene, COL17A1, cause junctional epidermolysis bullosa (JEB), characterized by chronic trauma-induced skin blistering. Here we exploited a novel naturally occurring COL17A1 mutation, leading to an in-frame lysine duplication within the coiled-coil structure of the juxtamembranous NC16A domain of collagen XVII, which resulted in a mild phenotype of JEB due to reduced membrane-anchored collagen XVII molecules. This mutation causes structural changes in the mutant molecule and interferes with its maturation. The destabilized coiled-coil structure of the mutant collagen XVII unmasks a furin cleavage site that results in excessive and non-physiological ectodomain shedding during its maturation. Furthermore, it decreases its triple-helical stability due to defective coiled-coil oligomerization, which makes it highly susceptible to proteolytic degradation. As a consequence of altered maturation and decreased stability of collagen XVII trimers, reduced collagen XVII is incorporated into the cell membrane, resulting in compromised dermal-epidermal adhesion. Taken together, using this genetic model, we provide the first proof that alteration of the coiled-coil structure destabilizes oligomerization and impairs physiological shedding of collagen XVII in vivo.

A New Method for Revision of Encapsulated Blebs after Trabeculectomy: Combination of Standard Bleb Needling with Transconjunctival Scleral Flap Sutures Prevents Early Postoperative Hypotony.

PURPOSE: A simple needling procedure is the standard method for restoring the function of an encapsulated bleb after trabeculectomy. However, postoperative hypotony represents a possible hazard. This study describes a new surgical approach for treating encapsulated blebs with reduced risk of early postoperative hypotony: bleb needling combined with transconjunctival sutures tightening the scleral flap directly. METHODS: The study included two groups of 23 patients with failing bleb following trabeculectomy: "Group 1" underwent simple needling revision of the filtering bleb and served as a control group, while "Group 2" received needling revision with additional transconjunctival scleral flap sutures, if intraoperatively the intraocular pressure was estimated to be very low. Intraocular pressure (IOP), postoperative management and complications were analyzed over a follow-up period of 4 weeks postoperatively. Results were compared using t-test or Mann-Whitney U-tests. RESULTS: Adverse effects occurred with a higher frequency after sole needling of the bleb (5 cases of choroidal effusion and 1 case of choroidal hemorrhage) than after the combined method with additional scleral sutures (1 case of choroidal effusion). The IOP on the first postoperative day was significantly lower in group 1, with 9.43 ± 9.01 mm Hg vs. 16.43 ± 8.35 mm Hg in group 2 (P = 0.01). Ten patients with ocular hypotony (IOD of 5 mmHg or lower) were found in group 1 and only two in group 2. One week and one month after surgery the intraocular pressure was similar in both groups (P>0.05). CONCLUSIONS: This new needling technique with additional transconjunctival scleral flap sutures appears to reduce postoperative hypotony, and may thus protect from further complications, such as subchoroidal hemorrhage.

Detection of apoptosis in pemphigus vulgaris by TUNEL technique

Abstract: Background: Pemphigus is part of a group of blistering diseases that affect the skin and mucous membranes. Based on its autoimmune origin, autoantibodies develop in pemphigus that are directed toward cell surface components of keratinocytes. However, some data cannot be explained, such as the lack of a relationship between autoantibody levels and the severity of clinical manifestations, treatment resistance, the presence of inflammatory infiltrates and the potential occurrence of apoptosis as determinants of vesicle formation. Objective: To examine the presence of apoptosis in pemphigus vulgaris by TUNEL technique. Methods: In this cross-sectional study, we selected 15 paraffin-embedded tissues from subjects who were diagnosed with pemphigus vulgaris by hematoxylin and eosin staining. The samples were subjected to TUNEL assay and examined under an Olympus BX61 fluorescence microscope. Positivity was categorized dichotomously, and the statistical analysis was performed using the X2 test. Results: Positivity was observed in basal layer cells in 14 (93.3%) cases. In 13 (86.7%) of the positive cases, we noted espinosum and granular layers that formed the blister roof, and in 12 cases (80%), positive acantholytic cells were observed. Conclusions: TUNEL positivity was observed in pemphigus vulgaris, implicating apoptosis in the pathophysiology of this condition, which can help guide the development of apoptotic blockers as therapeutics.