Safety and benefit of modified mesohepatectomy in the treatment of bilobar involvement congenital biliary dilatation.

BACKGROUND: The diseased bile duct in bilobar congenital biliary dilatation is extensive and often requires major hepatectomy or liver transplantation associated with a higher risk. We aimed to evaluate the safety and benefit of modified mesohepatectomy, in comparison with trisectionectomy, to treat bilobar congenital biliary dilatation. METHODS: This study included 28 patients with type IV and V bilobar congenital biliary dilatation. An innovative mesohepatectomy comprising the hepatectomy technique beyond the P/U point and bile duct shaping was applied to 14 patients to address the extensively diseased bile duct and difficulty in hepaticojejunostomy. Another 14 patients received trisectionectomy. The perioperative and long-term outcomes of these patients were compared. RESULTS: The ratio of residual liver volume to standard liver volume in the mesohepatectomy group was higher (78.68% vs. 40.90%, p = 0.005), while the resection rate of the liver parenchyma was lower (28.25% vs. 63.97%, p = 0.000), than that in trisectionectomy group. The mesohepatectomy group had a lower severe complication (>Clavein III, 0% vs. 57.70%, p = 0.019) and incidence of posthepatectomy liver failure (7.14% vs. 42.86%, p = 0.038). No significant difference was observed in blood loss and bile leakage (p > 0.05). All the patients in the mesohepatectomy group achieved optimal results in the long-term follow-up. CONCLUSIONS: mesohepatectomy provides an efficient treatment option for bilobar congenital biliary dilatation and can achieve radical resection, retain more liver parenchyma, and reduce the difficulty of hepaticojejunostomy, especially for patients that are not eligible for major hepatectomy and liver transplantation.

Prediction of short- and long-term outcomes using pre-operative ventricular size in infants with post-hemorrhagic ventricular dilation.

PURPOSE: Post-hemorrhagic ventricular dilation (PHVD) leads to developmental delays in premature infants, yet the optimal timing of neurosurgical interventions is unknown. Neuroimaging modalities have emerged to delineate injury and follow the progression of PHVD. Fronto-temporal horn ratio (FTHR) is used as a marker of ventricular dilation and can be a standardized tool to direct the timing of neurosurgical intervention. Our study determined a pre-operative FTHR measurement threshold to predict short- and long-term outcomes. METHODS: This is a retrospective cohort study of premature infants with severe intraventricular hemorrhage (IVH) who developed PHVD requiring neurosurgical intervention and were treated in a level IV NICU between 2012 and 2019. Receiver operating characteristic (ROC) curve and area under the curve (AUC) analyses were performed to evaluate the accuracy of pre-operative FTHR for predicting developmental delay. In-hospital outcomes and developmental assessments were analyzed. RESULTS: We reviewed 121 charts of infants with IVH and identified 43 infants with PHVD who required neurosurgical intervention. We found FTHR measurements were an excellent predictor of cognitive and motor delay with an AUC of 0.89 and 0.88, respectively. An average pre-operative FTHR of ≥ 0.67 was also associated with worse lung and feeding outcomes. There was excellent inter-observer reliability of individual components of FTHR measurements. CONCLUSIONS: Early intervention for PHVD is ideal but not always practical. Identification of ventricular size thresholds associated with better outcomes is needed to direct timing of neurosurgical intervention.

Corneal pseudoectasia: a case series.

OBJECTIVE: To raise awareness of conditions that can tomographically mimic corneal ectasia and describe the actions required to avoid misdiagnosis. METHODS: We report a retrospective case series of seven patients presenting at two tertiary care centers in Israel with a presumed diagnosis of keratoconus or post-refractive ectasia. Upon further examination, the ectasia diagnosis was reconsidered and eventually ruled out. RESULTS: Included were ten eyes of seven patients. Cases included bilateral diffuse Salzmann's nodular degeneration, ophthalmoplegia with strabismus which precluded proper fixation during the acquisition of tomography images, two cases of incorrect Pentacam parameter settings, a patient with a history of hyperopic laser-assisted in situ keratomileusis (LASIK) treatment in one eye and myopic LASIK in the fellow eye, a case of old post-photorefractive keratectomy (PRK) stromal haze, and a patient with posterior polymorphous corneal dystrophy. CONCLUSIONS: Tomography patterns mimicking corneal ectasia can appear in patients without ectatic pathology. The comprehensive ophthalmologist should be aware of such cases as they may substantially alter the treatment course and prognosis of these patients.

Congenital segmental dilatation of the colon in a newborn: a radiologic perspective of an unusual entity.

A newborn with congenital segmental dilatation of the intestine affecting the colon is presented. This rare condition, unrelated to Hirschsprung's disease, may affect any portion of the bowel and is characterized by focal dilatation of a segment of bowel flanked by normal proximal and distal bowel. While reported in the surgical literature, congenital segmental dilatation of the intestine has not been reported in the pediatric radiology literature even though pediatric radiologists may be the first to encounter imaging suggesting the diagnosis. We therefore present the characteristic imaging findings, including abdominal radiographs and images from a contrast enema, and discuss the clinical presentation, pathology findings, associations, treatment, and prognosis of congenital segmental dilatation of the intestine to increase awareness of this unusual diagnosis.

Abdominal aortic aneurysm neck dilatation and sac remodeling in fenestrated compared to standard endovascular aortic repair.

OBJECTIVE: Contemporary commercially available endovascular devices for the treatment of abdominal aortic aneurysm (AAA) include standard endovascular aortic repair (sEVAR) or fenestrated EVAR (fEVAR) endografts. However, aortic neck dilatation (AND) can occur in nearly 25% of patients following EVAR, resulting in loss of proximal seal with risk of aortic rupture. AND has not been well characterized in fEVAR, and direct comparisons studying AND between fEVAR and sEVAR have not been performed. This study aims to analyze AND in the infrarenal and suprarenal aortic segments, including seal zone, and quantify sac regression following fEVAR implantation compared to sEVAR. METHOD: A retrospective review of prospectively collected data on 20 consecutive fEVAR patients (Cook Zenith® Fenestrated) and 20 sEVAR (Cook Zenith®) patients was performed. Demographic data, anatomic characteristics, procedural details, and clinical outcome were analyzed. Pre-operative, post-operative (1 month), and longest follow-up CT scan at an average of 29.3 months for fEVAR and 29.8 months for sEVAR were analyzed using a dedicated 3D workstation (iNtuition, TeraRecon Inc, Foster City, California). Abdominal aortic aneurysm neck diameter was measured in 5 mm increments, ranging from 20 mm above to 20 mm below the lowest renal artery. Sub-analysis comparing the fEVAR to the sEVAR group at 12 months and at greater than 30 months was performed. Standard statistical analysis was done. RESULTS: Demographic characteristics did not differ significantly between the two cohorts. The fEVAR group had a larger mean aortic diameter at the lowest renal artery, shorter infrarenal aortic neck length, increased prevalence of nonparallel neck shape, and longer AAA length. On follow-up imaging, the suprarenal aortic segment dilated significantly more at all locations in the fEVAR cohort, whereas the infrarenal aortic neck segment dilated significantly less compared to the sEVAR group. Compared to the sEVAR cohort, the fEVAR patients demonstrated significantly greater positive sac remodeling as evident by more sac diameter regression, and elongation of distance measured from the celiac axis to the most cephalad margin of the sac. Device migration, endoleak occurrence, re-intervention rate, and mortalities were similar in both groups. CONCLUSION: Compared to sEVAR, patients undergoing fEVAR had greater extent of suprarenal AND, consistent with a more diseased native proximal aorta. However, the infrarenal neck, which is shorter and also more diseased in fEVAR patients, appears more stable in the post-operative period as compared to sEVAR. Moreover, the fEVAR cohort had significantly greater sac shrinkage and improved aortic remodeling. The suprarenal seal zone in fEVAR may result in a previously undescribed increased level of protection against infrarenal neck dilatation. We hypothesize that the resultant decreased endotension conferred by better seal zone may be responsible for a more dramatic sac shrinkage in fEVAR.

High Astigmatism Secondary to Peripheral Ectasia Recurrence in Postpenetrating Keratoplasty Eyes Managed With Miniscleral Contact Lenses.

OBJECTIVES: After penetrating keratoplasty (PK) for keratoconus, vision can be impaired by high-degree astigmatism, particularly in those patients with recurrent peripheral ectasia. Scleral contact lenses (CLs) have long been used in the management of keratoconus both in treatment-naive corneas and those postcorneal transplants. We report the use of miniscleral CLs and their related visual and clinical outcomes in a series of patients with post-PK peripheral rim ectasia. METHODS: In this retrospective case series, 5 patients (7 eyes) presented because of reduced visual acuity with their spectacles/CLs and/or reduced comfort with their existing rigid gas-permeable lenses. All patients in this series underwent PK more than two decades ago for keratoconus (mean 28.7 years±7.2). All patients demonstrated characteristic thinning at the graft-host junction, with anterior chamber deepening. Central corneas had remained clear in all patients inferring high visual potential. Contact lenses used were No 7 Comfort 15 miniscleral and the Onefit MED scleral with 14.5 mm and 15.6 mm diameters, respectively. RESULTS: All eyes achieved a best-corrected visual acuity of 6/9 or greater. One case had difficulty with insertion and removal and has since discontinued wearing lens at this time. All others are successfully wearing the lenses regularly. CONCLUSION: Despite advances in CL design, surgical management is still required in some patients. Miniscleral CLs are effective in the refractive management of peripheral ectasia in keratoconic post-PK eyes and should be considered in such eyes before proceeding with repeat surgical intervention.

Factors predicting surgical difficulties in congenital biliary dilatation in pediatric patients.

BACKGROUND: The effects of disease classification and the patient's preoperative condition on the difficulty of performing a laparotomy for pediatric congenital biliary dilatation (CBD) have not been fully elucidated. METHODS: The present study retrospectively analyzed 46 pediatric CBD laparotomies performed at the study center between March 2010 and December 2021 and predictors of operative time. The patients were separated into a short operative time group (SOT) (≤ 360 min, n = 27) and a long operative time group (LOT) (> 360 min, n = 19). RESULTS: The preoperative AST and ALT values were higher, and the bile duct anastomosis diameter was larger, in the LOT. Correlation analysis demonstrated that the maximum cyst diameter, preoperative neutrophil-to-lymphocyte ratio, AST, ALT, AMY, and bile duct anastomosis diameter correlated positively with operative time. Multivariate analysis identified the maximal cyst diameter, preoperative AST, and bile duct anastomosis diameter as significant factors affecting surgical time. Postoperatively, intrapancreatic stones and paralytic ileus were observed in one patient each in the SOT, and mild bile leakage was observed in one patient in the LOT. CONCLUSIONS: The maximum cyst diameter, preoperative AST, and bile duct anastomosis diameter have the potential to predict the difficulty of performing a pediatric CBD laparotomy.

[Corneal ectasia following small-incision lenticule extraction: a case report].

A 22-year-old male presented with complaints of blurred vision in his right eye over the past 2 years following small incision lenticule extraction (SMILE) surgery conducted 4 years ago. Following a thorough ocular examination and evaluation, he was diagnosed with corneal ectasia in the right eye after the SMILE procedure. Subsequently, the patient underwent corneal cross-linking (CXL) treatment in the right eye to prevent the progression of the condition. After 3 months of treatment, the corneal ectasia remained stable. This article outlines the process of diagnosis and treatment, reviews the corneal conditions prior to the SMILE surgery, and analyzes the possible reasons behind the occurrence of postoperative corneal ectasia.

Indications for keratoplasty in management of corneal ectasia.

PURPOSE OF REVIEW: The current review aims to describe recent advancements in treatment of corneal ectasias and its effect on indications for corneal transplantation. RECENT FINDINGS: The majority of patients affected by ectatic corneal disease use contact lenses to correct resulting astigmatism. Patients who are intolerant of contact lenses or cannot achieve acceptable vision through conservative measures could consider keratoplasty. However, continuing advancements in both nonsurgical and surgical treatments are either reducing or delaying the need for keratoplasty in patients affected by ectatic corneal disease. SUMMARY: Corneal transplantation has been the mainstay of treatment for patients with advanced ectatic corneal disease. In the past decade, numerous improvements have been occurred to make contact lenses not only more effective for visual correction, but also more comfortable. Although corneal cross-linking is the only proven treatment known to prevent progression of disease, several other therapies show early potential for those in which cross-linking is contraindicated. Patients now have access to a wider range of therapies before considering keratoplasty.

Aortic Healing Appears to Occur Rapidly after Successful Endovascular Sealing of Blunt Thoracic Aortic Injury.

BACKGROUND: The traumatic nature of blunt thoracic aortic injury (BTAI) would suggest that healing of the aorta would occur once the injured area is shielded from aortic pressure. This would be in contrast to degenerative aortic diseases which often continue to degenerate despite coverage. We hypothesize that after successful thoracic aortic endografting (TEVAR) that the aorta rapidly heals itself leaving minimal to no trace of the residual injury. METHODS: BTAI that were successfully covered with TEVAR from 2006 to 2019 were collected. Those with failed sealing or a lack of follow-up scans were excluded. Centerline aortic diameters were measured at healthy aorta 1 cm above (D1) and below the injury (D3) and at the widest point of injury (D2) on preoperative and initial postoperative computed tomography (CT) scans. Postoperative CTs were examined for residual signs of aortic injury including residual periaortic hematoma, persistent thrombosed pseudoaneurysm, or thickened aortic wall. Diameter changes in the healthy and injured aortic segments were compared pre and post TEVAR. Aortic diameter changes were analyzed with the Student's t-test. RESULTS: Twenty four patients were identified with sealed BTAI. The mean graft diameter was 24.2 ± 3.2 mm with oversizing of 10.74 ± 6.1 % at D1 and 19.52 ± 10.22 % at D3. Postoperative CTs occurred at 61.25 ± 123.6 days with one outlier at 602 days. Injured aortic segments (D2) had significantly larger diameters compared to D1 (28.94 ± 5.08 mm vs. 22.14 ± 3.08 mm, P < 0.001). After TEVAR, 23/24 (95.8%) had no residual radiographic evidence of aortic injury by 2 months. One patient had a persistent thrombosed pseudoaneurysm likely due to more than 50% disruption of the aortic wall. Post TEVAR, there was a significant diameter reduction at D2 by 13.8% (29.10 ± 5.27 mm vs. 24.8 ± 4.2 mm, P < 0.001) which was within 2.45% of the mean stent graft diameter. The healthy aorta dilated to accommodate the graft by 9% at D1 (21.9 ± 3.0 vs. 23.7 ± 2.5 mm, P < 0.001) and 17% at D3 (20.6 ± 3.4 mm vs. 23.6 ± 3.2 mm, P < 0.001). CONCLUSIONS: TEVAR promotes rapid aortic healing in BTAI with no evidence of residual aortic injury suggesting that a long-term seal is not necessary. The healthy aorta dilates to the stent graft size, as expected, whereas the injured aortic segment heals around the stent graft and assumes its diameter as well. Massive disruption of the aortic wall may preclude early healing.

Bicuspid Aortic Valves: an Up-to-Date Review on Genetics, Natural History, and Management.

PURPOSE OF REVIEW: Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. It has a wide spectrum of clinical manifestations including aortic regurgitation (AR), aortic stenosis, and an associated aortopathy with a small but increased risk of aortic dissection. This review describes current knowledge of BAV, from anatomy and genetics to a discussion of multifaceted strategies utilized in the management of this unique patient population. This review will also highlight critical knowledge gaps in areas of basic and clinical research to enhance further understanding of this clinical entity. RECENT FINDINGS: The current knowledge regarding pathophysiologic mechanisms, screening, and surveillance guidelines for BAV and the associated aortopathy is discussed. We also discuss current management techniques for aortic valve repair versus replacement, indications for aortic surgery (root or ascending aorta), and the emergence of the Ross procedure as a viable management option not only in children, but also in adolescents and adults. The varied clinical phenotype of the BAV, resulting in its specific complex hemodynamic interactions, renders it an entity which is separate and distinct from the tricuspid aortic valve pathologies. While various aortic histopathologic and protein alterations in BAV patients have been described, it remains unclear if these changes are causal or the result of hemodynamic alterations imposed by sheer stress on the intrinsically dysfunctional BAV. Medical management for patients with BAV with AS, AI, or dilated aortic roots/ascending aortas remains challenging and needs further investigation. More than 50% of patients with BAV will undergo AVR during their lifetime, and more than 25% of patients with BAV undergo aortic surgery performed for dilation of the aortic root or ascending aorta, often concurrently with AVR. The search for the ultimate genetic or epigenetic cause of the different bicuspid phenotypes will ultimately be facilitated by the next-generation sequencing tools that allow for study of large populations at low cost. Improvements in diagnostic and stratification criteria to accurately risk assess BAV patients are critical to this process.

ACUTE CORONARY SYNDROME IN A PATIENT WITH MULTIPLY CORONARY ARTERY ECTASIA AND ASCENDING AORTIC ANEURYSM.

This report describes a case of multiply СAE presenting as a ST-elevation myocardial infarction in an adult patient with bicuspid aortic valve (BAV) and ascending aortic aneurysm. Coronary angiography revealed multiply ectasia in left anterior descending artery and right coronary artery. A computer tomographic aortogram confirmed the presence BAV with signs of calcification and an enlarged ascending aorta. The patient underwent coronary artery bypass grafting of left descending and circumflex coronary arteries and tube graft replacement of ascending aorta in 1 month after ACS in experienced centre. In patients with ACS due to CAE culprit, the emphasis is to restore flow. Medical treatment as well as lifestyle changes and risk factors control, and serial imaging assessment of aortic aneurysm constitute the second part of the management of these patients. We need prospective studies and registries to improve patient care and outcome.

Modified Antimesenteric Tapering Enteroplasty: An Alternative Technique for the Treatment of Dysfunctional Anastomosis in Patients With Short Bowel.

INTRODUCTION: Several techniques have been described to taper the dilated small bowel to improve intestinal motility and decrease complications related to overdilated small bowel, including longitudinal intestinal lengthening and tapering, serial transverse enteroplasty, and spiral intestinal lengthening and tailoring. We propose an alternative technique designed to optimize bowel function and minimize the effects of recurrent small-bowel bacterial overgrowth in patients with short or ultra-short gut syndrome and dysfunctional anastomosis with maintenance of the actual absorptive surface. TECHNIQUE: The dilated side-to-side anastomosis is identified, and the mesentery leaves from both the proximal and distal small-bowel loops are separated by using blunt dissection. The previous anastomosis is divided longitudinally with a GI stapler. Once the small-bowel transection is completed, 2 separate blind loops of intestine are created, each one with half the circumference of the dilated side-to-side anastomosis. The antimesenteric stapled line is then reinforced with an outer layer of running suture. The blind loops of the tapered small bowel are then trimmed and anastomosed in an end-to-end isoperistaltic fashion in 2 layers. RESULTS: There were no postoperative complications. The length of the tapering ranged from 10 to 23 cm, corresponding to approximately 16% (range, 13%-20%) of the remaining small-bowel length. Three of 4 patients presented significant improvement of their symptoms and were able to have their parenteral support discontinued. CONCLUSIONS: Modified antimesenteric tapering enteroplasty is an alternative technique to improve intestinal motility and treat patients with short-bowel syndrome and dysfunctional side-to-side anastomosis without the need for further small-bowel resection. This bowel-sparing technique represents a valuable option in the armamentarium of the surgeon who manages patients with intestinal failure.

Differences in post-operative complications after reconstruction for congenital biliary dilatation in a single institution-Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy.

BACKGROUND: The standard surgical procedure for congenital biliary dilatation (CBD) consists of excision of extrahepatic bile duct and Roux-en-Y hepaticojejunostomy (HJ). However, alternative reconstructive operations for CBD includes hepaticoduodenostomy (HD). We compared postoperative complications and therapeutic outcomes of these two operations at our institution. METHODS: From 1981 to 2009, there were 23 traceable patients who underwent operation for CBD, They were divided into an HJ Group (n = 15) and an HD Group (n = 8). Demographic and outcome data were compared. RESULTS: There were no significant differences in postoperative complications (cholangitis, pancreatitis, and anastomotic stenosis) and current blood test data (AST, ALT, Total Bilirubin, Direct Bilirubin, Amylase) between the two groups. Current abdominal pain and carcinogenesis were not observed in either group. Intrahepatic stones occurred in one patient in each group, both > 25 years post-operation. CONCLUSION: HD is considered to be an acceptable reconstruction method compared to HJ, based on our results. There has been a suggestion that inadequate diversion in HD might increase the risk of cholangiocarcinoma. To date, in this series, that has not happened.

Is Tapering Enteroplasty an Alternative to Resection of Dilated Bowel in Small Intestinal Atresia?

BACKGROUND: Intestinal atresia is a congenital defect resulting in intestinal discontinuity and can be associated with significant morbidity related to intestinal failure. The bowel proximal to the atresia is often significantly dilated and dysfunctional. The treatment approaches of this dilated bowel include resection with primary anastomosis versus tapering enteroplasty with preservation of bowel length. The purpose of this study was to compare these two approaches in regard to bowel function as characterized by the time to full enteral feeding. METHODS: A retrospective review was performed of intestinal atresia repair performed at a tertiary referral pediatric hospital from 2007 to 2017. Length of stay, time to full enteral feeds, and complications were assessed in patients who underwent repair with tapering enteroplasty (n = 8) and compared with those who underwent resection and anastomosis (n = 39). RESULTS: The median age at surgery, gender distribution, weeks gestational age (WGA), location of the atresia, and comorbidities were similar between the two groups. Overall, there was no statistically significant difference in length of stay and time to full enteral feeds between groups. Three of eight (38%) patients in the tapered group and five of 39 patients (13%; P = 0.12) in the nontapered group underwent further surgical exploration because of bowel dysmotility. Factors associated with longer length of hospital stay were abdominal reoperation and WGA, and factors associated with longer time to full enteral feeds were WGA, abdominal reoperation, and gastroschisis. CONCLUSIONS: Tapering enteroplasty at initial operation for intestinal atresias preserves bowel length and has statistically equivalent outcomes to resection and anastomosis in regard to the length of stay and time to full enteral feeds.

Endoscopic decompression of acute intestinal distension is associated with reduced mortality in critically ill patients.

BACKGROUND: Endoscopic placement of intestinal decompression tubes is a feasible technique for treatment of acute intestinal dilation. Given the heterogeneity of the underlying diseases leading to intestinal obstruction data on the significance of endoscopic procedures for treatment of these conditions are sparse. METHODS: In the study period from 2008 to 2019 all patients receiving a decompression tube were identified by retrospective chart review and analyzed. RESULTS: A total of 59 decompression tubes were placed in 50 patients. Technical success was achieved in 98% (58/59 tubes). As major complication one small bowel perforation occurred (1/59; 1.7%). Causes for impaired intestinal transit comprised tumor stenoses 22% (11/50), infections 18% (9/50), post-operative paralysis 14% (7/50), neurological diseases 8% (4/50), trauma 2% (1/50) and others 36% (18/50). Most patients (74%; 37/50) were critically ill and treated on intensive care unit. Treatment response after tube insertion was documented in 76% of patients (38/50) whereas 24% (12/50) did not fulfill response criteria. Patients with treatment response showed a significantly better outcome compared to non-responders. Responders had a median survival of 113 days (95% CI 41-186) compared to 15 days (95% CI 6-24) in non-responders (p = 0.002). Analysis of laboratory parameters after stratification in responders and non-responders to endoscopic therapy showed that non-responders had significantly higher levels of CRP and lower platelet count at baseline (CRP 262 mg/L (IQR 101-307) vs. 94 mg/L (IQR 26-153): p = 0.027; platelets 69 thsd/µL (IQR 33-161) vs. 199 thsd/µL (IQR 138-289): p = 0.009). CONCLUSIONS: Endoscopic decompression is a safe procedure for acute management of impaired intestinal transit even in critically ill patients. Response to therapy is associated with improved outcome and markers of inflammation and organ function such as CRP, platelet count and serum lactate have to be taken into account for therapy monitoring and evaluation of prognosis.

Optimizing outcomes for keratoplasty in ectatic corneal disease.

PURPOSE OF REVIEW: Advanced keratectasia often requires corneal transplantation for restoration of vision. This review discusses the surgical approach suitable for these cases including significant updates in the current literature. RECENT FINDINGS: With greater understanding of ectatic corneal disease and its associated operative challenges, several modifications in the surgical approach have been developed. Identified predictive factors for successful pneumatic dissection and intraoperative complications have informed surgical planning and decision-making. In the setting of failed pneumatic dissection, sequential use of alternative lamellar dissection techniques is a reasonable surgical strategy. Surgical refinements to optimize visual outcomes and postoperative interventions to correct residual refractive errors have improved visual rehabilitation after keratoplasty. SUMMARY: With evidence of its undisputed advantages, DALK has been widely recognized as the primary surgical option for ectatic corneal disease.

Reversible Focal Dystonia Secondary to Giant Perivascular Spaces.

Giant perivascular spaces (PVS) are rare, CSF-filled, dilated cavities lined by the pia mater that are most often asymptomatic radiological findings but can sometimes cause neurological symptoms. We present two exceptional cases of secondary focal dystonia induced by the mass effect on the basal ganglia caused by giant diencephalic PVSs. In both cases, we chose a surgical stereotactic drainage approach as a treatment strategy that allowed controlling the movement disorders. The beneficial therapeutic effect was sustained at long-term follow-up. We believe that similar cases could be addressed successfully with this method.

Correlation Between Total Repair Timing and Late Aortic Root Dilatation in Repaired Tetralogy of Fallot.

Aortic root dilatation is frequently encountered in adults with repaired Tetralogy of Fallot (TOF). The timing of total repair is known to have a significant correlation with late aortic root dilatation, but it is not well established. The objective of this study was to investigate the risk factors and correlation with timing of total repair for aortic root dilatation in adults with repaired TOF. An observational retrospective cohort study was conducted in 314 adults (≥ 18 years) with repaired TOF. Aortic root dilatation was defined when the absolute diameter of Sinus of Valsalva (SoV) was over 40 mm. SoV was measured by echocardiography in 110 patients, computed tomography angiography in 168 patients, and magnetic resonance image in 36 patients. Aortic root dilatation was observed in 65 patients (20.7%). Among them, 3 patients underwent Bentall operation due to acute aortic dissection or aortic root aneurysm. On multivariate logistic regression analysis, age at total repair was a significant risk factor for late aortic root dilatation (OR = 3.14; 95% CI 1.62-6.08; p = 0.001) and the cutoff value of age was 1.9 years. However, late aortic root dilatation was also observed in 10% of patients (10/91) who operated before 1 year of age. Late total repair was a significant risk factor for late aortic root dilatation in repaired TOF. However, early total repair did not always prevent late aortic root dilatation. These results suggest that congenital aortic pathology may also be a possible cause of aortic root dilatation along with longstanding hemodynamic stress.

Secondary tracheal distortion in an adult patient after therapy for diphtheria at childhood.

We report a rare case of difficult intubation because of trachea deformation after therapy for diphtheria and subsequent tracheostomy at childhood. The patient was admitted to be subjected to a Bentall procedure, because of ascending aorta dilatation. With a detailed preanesthetic assessment and simulation for intubation by using three-dimensional technology, he was successfully intubated and the operation was accomplished uneventfully. 3D-printed models of the difficult airway enhance imaging of difficult anatomy, assist in simulation and management of potentially problematic intubation, and can be a valuable tool when dealing with airways with potential anatomical malformations.