Scheimpflug-Based Tomographic Risk Factors for the Development of Acute Corneal Hydrops in Keratoconus.

PURPOSE: The purpose of this study was to assess the risk factors that predispose patients with keratoconus to develop acute corneal hydrops (ACH), including both clinical and tomographic risk factors. We additionally describe tomographic changes of the cornea over time after ACH. METHODS: We retrospectively reviewed patients with keratoconus who were followed at our institution from January 2015 to May 2023. Control eyes, defined as eyes with advanced keratoconus (stage IV Amsler-Krumeich classification on initial examination) were compared with eyes that developed ACH. Demographic, clinical, and tomographic factors were investigated. Visual acuity, keratometry, and corneal thickness were assessed at each follow-up visit to monitor progression over time. RESULTS: Twenty-three eyes of 19 patients developed ACH over the follow-up period. The incidence of known clinical associations including seasonal allergies, eye rubbing, snoring, asthma, and eczema was similar between the hydrops and control groups. There was a higher incidence of Down syndrome in the hydrops group (P = 0.04). Eyes that developed hydrops had similar best corrected visual acuity on initial examination, but had steeper keratometry (P = 0.003) and thinner corneas (P < 0.001) than controls at baseline. After hydrops, progressive corneal flattening and reduced maximum keratometry occurred over time. However, final best corrected visual acuity was worse compared with initial examination before hydrops (P = 0.03), as well as compared with control eyes (P < 0.001). CONCLUSIONS: Risk factors of developing ACH include steep keratometry and thin corneas as well as Down syndrome. Although corneal flattening will occur after resolution of acute corneal edema, visual acuity worsened after ACH.

Acute-onset surgically induced necrotizing scleritis after strabismus surgery.

Surgically induced necrotizing scleritis (SINS) is an uncommon but devastating complication that may occur days to years after ocular surgery. We report the case of a 32-year-old man who underwent uncomplicated strabismus surgery for large-angle exotropia and developed SINS characterized by painless scleral inflammation, choroidal exposure, and globe ectasia within days of surgery. Work-up revealed no associated infectious process or underlying systemic inflammatory condition. Clinical resolution occurred with oral immunosuppression alone, without need for graft.

Congenital Orbital Fibrosis With Spontaneous Regression of Orbital Tumor.

Congenital orbital fibrosis (COF) is a rare disorder characterized by an infiltrating orbital mass with secondary involvement of the extraocular muscles that may present with extraocular muscle dysfunction, and globe and eyelid abnormalities in infancy. This condition is thought to be a nonprogressive process and literature on longitudinal assessment of COF is limited. The authors describe a case of COF which was followed for 15 years. The patient had stable symptoms of ocular dysmotility and ptosis but was noted to have spontaneous regression of the orbital mass on serial MRI.

Structure-function analysis for macular surgery in patients with coexisting glaucoma.

PURPOSE: To develop methods to assess the effects of epiretinal membranes (ERM) and macular holes (MH) coexisting with glaucoma on pre-operative retinal structure and function and evaluate post-operative outcomes. METHODS: Seven eyes of 7 patients with glaucoma, 6 with ERMs and 1 with MH, were enrolled; 4 underwent vitrectomy for ERM and one for MH. Visual fields (VFs) and optical coherence tomography (OCT) scans were obtained pre- and post-operatively. The 10-2VF deviation map was overlayed on ganglion cell and inner plexiform layer (GCL + IPL) and retinal nerve fiber layer (RNFL) deviation maps derived from OCT macula and disc cube scans. Optic nerve circle scans were obtained to assess RNFL thickness, and OCT b-scans associated with VF defects were compared pre- and post-operatively. RESULTS: Examination of pre-operative VFs and OCT scans showed the importance of determining the extent to which glaucomatous damage contributed to VF loss; verifying automated segmentation of the GCL + IPL and RNFL; and assessing foveal anatomy. Evaluation of post-operative structure-function outcomes required correction of magnification changes in OCT scans and repeated follow-up visits to clarify the origin of VF changes. CONCLUSIONS: Pre-operative comparisons of VFs and OCT scans may be beneficial in guiding surgical planning, and evaluating outcomes, in eyes with glaucoma undergoing macular surgery.

Importance of surgical expertise in septal myectomy for obstructive hypertrophic cardiomyopathy.

OBJECTIVE: In 2011, a multidisciplinary hypertrophic cardiomyopathy (HCM) program with a dedicated myectomy surgeon was implemented at our institution. We hypothesized that a dedicated approach allows better identification and management of mitral regurgitation (MR) during septal myectomy (SM) for obstructive HCM with significant mitral regurgitation. METHODS: Between 2006 and 2018, 181 patients had SM at our institution. This study consists of 53 patients with preoperative moderate or greater MR associated with systolic anterior motion who underwent isolated SM with or without mitral intervention. Patients were divided into those who underwent SM by a dedicated myectomy surgeon (group D, n = 31) or by a non-dedicated surgeon (group ND, n = 22). Primary outcome of interest was rate of mitral valve replacement (MVR) at SM. Secondary outcomes include in-hospital mortality, need for permanent pacemaker, mitral valve reoperation, and residual MR and left ventricular outflow tract gradient on postoperative echocardiography. RESULTS: 12 patients (55%) had a concomitant MVR during septal myectomy in group ND compared to 2 patients (6%) in group D (p < 0.01). Among patients who did not undergo MVR, patients in group D less commonly had residual MR than patients in ND after SM (p < 0.01). Group D had 100% survival with NYHA class I in 94% patients at follow-up visit (p = 0.01). Reoperation for MVR was required in four patients in group ND vs. none in group D (p < 0.01). CONCLUSIONS: A dedicated surgeon is able to spare the mitral valve in patients undergoing SM. This study emphasizes the importance of surgical expertise in this cohort.

Impact of Septal Myectomy Volume on Mitral-Valve Replacement Rate in Hypertrophic Cardiomyopathy Patients.

OBJECTIVE: Mitral regurgitation (MR) induced by systolic anterior motion in patients with hypertrophic cardiomyopathy (HCM) can frequently be abolished with a proficient septal myectomy (SM) without the need for mitral-valve replacement (MVR). ACC guidelines stress the importance of volume in improving outcomes after SM, but there is a lack of data measuring the impact of volume on the need for MVR during SM. This study was designed to assess the impact of institutional volume on MVR rates using national outcomes data. METHODS: The Nationwide Inpatient Sample was queried from 1998 to 2011 and a total of 6,207 patients had a diagnosis of HCM and a procedure code for SM. Outcomes were compared between patients who underwent SM (group I) and SM and MVR (group II). Furthermore, patients were stratified into 3 groups based on the number of SMs at the performing institution: low experience (1-24 cumulative SMs), medium experience (25-49 SMs), and high experience (>50 SMs). These patients underwent multivariable analysis to determine the impact of institutional volume on MVR rate. RESULTS: The total MVR rate was 26%. Perioperative outcomes were worse, i.e., there were higher rates of mortality, kidney injury, and urinary complications, in group II than in group I. Only 37.6% of patients were operated on at institutions meeting the guideline criteria of >50 cumulative SMs. When compared to patients in the high-experience group, patients in the low- (OR 2.7, 95% CI 2.3-3.2, p < 0.05) and medium-experience (OR 3.0, 95% CI 2.5-3.6, p < 0.05) groups were more likely to undergo MVR. CONCLUSION: Compared to reports from SM reference centers, national data suggest that MVR rates are quite high at SM. Patients undergoing SM at centers that do not meet the guideline standard have >2.5× the odds of undergoing MVR compared to those operated on at guideline-endorsed centers.

Chondrogenic, hypertrophic, and osteochondral differentiation of human mesenchymal stem cells on three-dimensionally woven scaffolds.

The development of mechanically functional cartilage and bone tissue constructs of clinically relevant size, as well as their integration with native tissues, remains an important challenge for regenerative medicine. The objective of this study was to assess adult human mesenchymal stem cells (MSCs) in large, three-dimensionally woven poly(ε-caprolactone; PCL) scaffolds in proximity to viable bone, both in a nude rat subcutaneous pouch model and under simulated conditions in vitro. In Study I, various scaffold permutations-PCL alone, PCL-bone, "point-of-care" seeded MSC-PCL-bone, and chondrogenically precultured Ch-MSC-PCL-bone constructs-were implanted in a dorsal, ectopic pouch in a nude rat. After 8 weeks, only cells in the Ch-MSC-PCL constructs exhibited both chondrogenic and osteogenic gene expression profiles. Notably, although both tissue profiles were present, constructs that had been chondrogenically precultured prior to implantation showed a loss of glycosaminoglycan (GAG) as well as the presence of mineralization along with the formation of trabecula-like structures. In Study II of the study, the GAG loss and mineralization observed in Study I in vivo were recapitulated in vitro by the presence of either nearby bone or osteogenic culture medium additives but were prevented by a continued presence of chondrogenic medium additives. These data suggest conditions under which adult human stem cells in combination with polymer scaffolds synthesize functional and phenotypically distinct tissues based on the environmental conditions and highlight the potential influence that paracrine factors from adjacent bone may have on MSC fate, once implanted in vivo for chondral or osteochondral repair.

Virtual septal myectomy for preoperative planning in hypertrophic cardiomyopathy.

OBJECTIVE: Although septal myectomy (SM) is the preferred treatment for medication-refractory obstructive hypertrophic cardiomyopathy, the procedure remains subjective. We have developed a virtual myectomy (VM) technique using 3-dimensional reconstruction of gated cardiac computed tomography (CT) to assist intraoperative objective assessment of the adequacy of the resection. METHODS: We retrospectively reviewed patients 15 patients who underwent a SM guided by preoperative VM at our program between March 2016 and July 2017. Gated cardiac CT was performed to allow delineation of the left ventricular (LV) myocardium at end-diastole to replicate the cardioplegic myocardial arrest (90%-95% RR interval). SM was performed to attain resection volume predicted by VM. Retrospective, blinded VM also was performed with fixed parameters to determine relationship between ideal (VM1) and conservative (VM2) VM and actual resection. RESULTS: Mean patient age was 52.1 ± 10.6 years, 27% were male, and 80% had New York Heart Association class 3 or 4. Preoperative mean peak LV outflow tract gradient was 79 mm Hg (range 47-82). In-hospital mortality was 0%. Mean postoperative LV outflow tract gradient was 13 mm Hg (11-19). Gated cardiac CT was performed with mean phase 94% (86%-98%). Mean total LV myocardial volume was 226 cm3 (146-365) and volume of the asymmetric portion of the LV was 19 cm3 (5.2-48.8). Actual surgical resection volume was 6.2 ± 1.7 cm3. Retrospective VM1 and VM2 performed postoperatively blinded to surgical results were 12.8 cm3 (4.8-29.23) and 6.7 cm3 (3.5-13.2), showing a modest correlation (R1 = 0.44, R2 = 0.56) with actual myectomy. CONCLUSIONS: Three-dimensional CT and VM can be a viable addition to preoperative assessment of patients with obstructive hypertrophic cardiomyopathy.

Late outcomes of subcostal exchange of the HeartMate II left ventricular assist device: a word of caution.

OBJECTIVES: Previous studies have shown the usefulness of the subcostal exchange of the HeartMate II left ventricular assist device for device malfunction. However, long-term data are still limited. METHODS: Between March 2004 and July 2017, 41 of 568 (7.2%) patients who had received a HeartMate II implant at our institution had a device exchange via a subcostal incision. We summarized early and late outcomes. RESULTS: Forty-one patients had a total of 48 subcostal pump exchanges. Indications for device exchange included device thrombosis (n = 31, 76%), driveline infection (n = 2, 5%) and driveline injury (n = 8, 19%). All of the procedures were successful, and there were no in-hospital deaths. A Kaplan-Meier survival curve showed 30-day and 1-year survival rates after subcostal exchange of 100% and 94.6%, respectively. However, 10 (25%) patients had left ventricular assist device-related infections following subcostal exchange that included 7 pump pocket infections and 3 driveline infections. Freedom from left ventricular assist device-related infection at 1 year after subcostal exchange was 79.3%. Thirteen (32%) patients had device malfunction due to pump thrombosis that required a 2nd device exchange. Seven patients had recurrent thrombosis. Three (7%) patients had a stroke. Freedom from device thrombosis and from a stroke event at 1 year was 74.4%. CONCLUSIONS: Subcostal pump exchange can be safely performed. However, there is a substantial risk of infection and recurrent thrombosis. Careful follow-up for late complications is mandatory.

Role of computed tomography angiography for HeartMate II left ventricular assist device thrombosis.

INTRODUCTION: Device thrombosis is one of the most devastating complications of HeartMate II left ventricular assist devices. The purpose of this study is to assess the anatomical properties that the inflow and outflow computed tomography angiography provides in assessing for left ventricular assist device thrombosis, as well as their impact on clinical management and postoperative outcomes. METHODS: Between April 2010 and December 2016, 22 patients who received a HeartMate II left ventricular assist device implantation were readmitted for suspected device thrombosis and underwent a computed tomography angiography for workup. Left ventricular assist device-associated anatomy was assessed, including outflow abnormality on computed tomography angiography (contrast filling defect), inflow abnormalities on computed tomography angiography (space at inflow, M-I angle), and inflow abnormalities on chest X-ray (inflow angulation, pump pocket depth). RESULTS: Computed tomography angiography revealed an outflow filling defect in three patients (14%) resulting in change in surgical approach from subcostal pump exchange to resternotomy pump and outflow graft exchange. Inflow graft malpositioning was identified in four patients, with the inflow abutting the left ventricular wall and obstructing the cannula opening. On computed tomography angiography assessment, mean space at inflow was 5.3 ± 1.6 mL and M-I angle was 35.6° ± 6.6°. Chest X-ray evaluation revealed mean inflow angle and pump pocket depth of 75.7° ± 13.4° and 110.2 ± 26.6 mm, respectively. CONCLUSION: Computed tomography angiography provides a noninvasive assessment of the outflow graft and inflow cannulas in left ventricular assist device patients. Findings on computed tomography angiography reveal possible mechanical etiologies of thrombosis and may be useful for determining the surgical management of device thrombosis patients.