Microsurgical versus endovascular treatment of ethmoidal dural arteriovenous fistulas: systematic review and meta-analysis with a single-center case series.

OBJECTIVE: Ethmoidal dural arteriovenous fistulas (DAVFs) are often associated with cortical venous drainage (CVD) and a higher incidence of hemorrhage compared with DAVFs in other locations. They may be treated with open surgical disconnection or with endovascular treatment (EVT). In this systematic review and meta-analysis, the authors compare the outcomes of ethmoidal DAVFs treated with open microsurgery versus EVT and report four additional cases of ethmoidal DAVFs treated with open microsurgery in their institution. METHODS: A literature search of the PubMed and Scopus databases was conducted between December 2021 and May 2022 to identify relevant articles published between 1990 and 2021 using the PRISMA guidelines. References were reviewed and screened by two authors independently, and disagreements were resolved through consensus. Exclusion criteria included non-English-language studies, those with an incorrect study design, those reporting DAVFs in a nonethmoidal location, and studies whose outcomes were not stratified based on DAVF location. Inclusion criteria were any studies reporting on ethmoidal DAVFs treated by either microsurgery or EVT. A risk of bias assessment was performed using the Newcastle-Ottawa Scale. The authors performed a pooled proportional meta-analysis to compare patient outcomes. RESULTS: Twenty studies were included for analysis. Of 224 patients, 142 were treated with surgery, while 103 were treated with EVT. Seventy percent (148/210) of the patients were symptomatic at presentation, with hemorrhage being the most common presentation (48%). CVD was present in 98% of patients and venous ectasia in 61%. The rates of complete DAVF obliteration with surgery and EVT were 89% and 70%, respectively (95% CI -30% to -10%, p < 0.03). Twenty percent (21/103) of endovascularly treated fistulas required subsequent surgery. Procedure-related complications occurred in 10% of the surgical cases, compared with 13% of the EVT cases. The authors' case series included 4 patients with ethmoidal DAVFs treated surgically with complete obliteration, without any postoperative complications. CONCLUSIONS: The complete obliteration rates of ethmoidal DAVF appear to be higher and more definitive with microsurgical intervention than with EVT. While complication rates between the two procedures seem similar, patients treated with EVT may require further interventions for definitive treatment. The limitations of this study include its retrospective nature, the quality of studies included, and the continued evolving technologies of EVT. Future studies should focus on the association between venous drainage pattern and the proclivity toward venous ectasia or rate of hemorrhage at presentation.

Etiological classification and treatment strategies for secondary bile duct dilatation.

Secondary intra- and extrahepatic bile duct dilatation is a very common condition that can be caused by several diseases. However, it has been rarely discussed in the specialized literature. Moreover, no distinct etiology can be determined in some cases, which hampers the diagnosis and treatment. Here, we discuss the etiological classification and treatment strategies of secondary intra- and extrahepatic bile duct dilatation based on an extensive literature review, as well as our experimental research and clinical experience. The etiology of secondary intra- and extrahepatic bile duct dilatation can be classified in different ways. From a clinicopathological perspective, it can be classified into obstruction-, lesion-, and compression-induced dilatation. Treatment varies depending on the cause. For example, endoscopic dilation or stenting is used for biliary strictures, laparoscopic choledochectomy for stone removal, and resection for cholangiocarcinoma.

A Case of TAVR Complicated by Severe Functional Mitral Regurgitation.

Mitral regurgitation (MR) is a known complication of transcatheter aortic valve replacement (TAVR). We report a case of a 90-year-old man with severe symptomatic aortic stenosis who underwent elective TAVR. The procedure was complicated by severe functional MR from left ventricular stunning and dilatation caused by hypotension throughout the procedure. An Impella CP (Abiomed, Inc, Danvers, MA) was inserted to unload the left ventricle and decrease its size with subsequent improvement in MR severity, which was sustained after Impella CP removal. In conclusion, we present the first case of successful management of post-TAVR severe functional MR with an Impella CP.

Extracranial ectasia and embolic infarcts in HIV: two case reports and a clinical decision-making algorithm.

HIV is known to increase the risk of both ischemic and hemorrhagic strokes. There are many postulated mechanisms for this elevated risk including an HIV-induced vasculopathy and/or coagulopathy, opportunistic infections, and cardioembolic etiologies, among others. Regarding vasculopathy, prior reports have described the various changes to the arterial vasculature that can occur in the setting of HIV, yet the appropriate workup and management of this condition remains poorly defined. Here we describe two cases of patients with HIV presenting with large vessel intracranial occlusions in the setting of ectatic extracranial vasculature accompanied by intraluminal thrombus formation. One patient underwent thrombectomy, while the other improved after receiving IV-tPA. Inferring on these cases and the existing literature, a standardized workup and treatment algorithm is proposed, emphasizing the key management decisions that should be considered on a case-by-case basis.

Comparison of accelerated CXL alone, accelerated CXL-ICRS, and accelerated CXL-TG-PRK in progressive keratoconus and other corneal ectasias.

PURPOSE: To compare accelerated corneal crosslinking (CXL) alone, CXL with simultaneous intrastromal corneal ring segments (CXL-ICRS), and CXL with simultaneous topography-guided photorefractive keratectomy (CXL-TG-PRK) in progressive keratoconus, pellucid marginal degeneration (PMD), or laser in situ keratomileusis (LASIK)-induced ectasia. SETTING: The Kensington Eye Institute and Bochner Eye Institute, Toronto, Canada. DESIGN: Prospective nonrandomized interventional study. METHODS: Visual and topographical outcomes using a comparative analysis adjusting for preoperative maximum keratometry (Kmax) were evaluated 1 year postoperatively. RESULTS: Four hundred fifty-two eyes from 375 patients with progressive keratoconus, PMD, or LASIK-induced ectasia that underwent accelerated (9 mW/cm, 10 minutes) CXL alone (n = 204), CXL-ICRS (n = 126), or CXL-TG-PRK (n = 122) were included. Change in logarithm of the minimum angle of resolution uncorrected distance visual acuity was significant with CXL-ICRS (-0.31; 95% CI, -0.38 to -0.24) and CXL-TG-PRK (-0.16; 95% CI, -0.24 to -0.09), but not with CXL alone. No significant differences in change were found between the 3 groups. Change in corrected distance visual acuity (CDVA) was significant in all 3 groups: -0.12 (95% CI, -0.15 to -0.10) with CXL alone, -0.23 (95% CI, -0.27 to -0.20) with CXL-ICRS, and -0.17 (95% CI, -0.21 to -0.13) with CXL-TG-PRK. Improvement in CDVA was greater with CXL-ICRS than with CXL alone (-0.08 ± 0.02; P < .0001) and CXL-TG-PRK (-0.05 ± 0.02; P = .005). Change in Kmax was significant with CXL-ICRS [-3.21 diopters (D); 95% CI, -3.98 to -2.45] and CXL-TG-PRK (-3.69 D; 95% CI, -4.49 to -2.90), but not with CXL alone (-0.05 D; 95% CI, -0.66 to 0.55). CONCLUSIONS: CXL alone might be best for keratoconic patients who meet the inclusion criteria. CXL-ICRS might be more effective for eyes with more irregular astigmatism and worse CDVA and CXL-TG-PRK for eyes requiring improvements in irregular astigmatism but still have good CDVA.

Dural Ectasia in Neurofibromatosis 1: Case Series, Management, and Review.

BACKGROUND: The natural history and management of dural ectasia in Neurofibromatosis 1 (NF1) is still largely unknown. Dural ectasias are one of the common clinical manifestations of NF1; however, the treatment options for dural ectasias remain unstudied. OBJECTIVE: To investigate the natural history, diagnosis, management, and outcome of the largest case series of patients with NF1-associated dural ectasia to date. METHODS: Records from our NF1 clinic were reviewed to identify NF1 patients with computed tomography or magnetic resonance imaging evidence of dural ectasia(s) to determine their clinical course. Demographics, symptoms, radiographic and histopathologic findings, treatment, and clinical course were assessed. RESULTS: Thirty-four of 37 patients were managed without surgery. Of the 18 initially asymptomatic patients, 5 (27.8%) progressed to symptoms attributable to a dural ectasia (onset of 2.7% per patient-year). Three patients required surgical intervention because of extraspinal mass effect. All 3 initially improved but had symptom recurrence within 2 yr. Reoperation involved shunt placement for cerebrospinal fluid (CSF) diversion. On imaging review, 26 (76.5%) of the nonsurgical patients harbored an associated nearby plexiform neurofibroma. Pathology of one surgical case revealed dural infiltration by diffuse neurofibroma. CONCLUSION: Using the largest NF1-associated dural ectasia group to date, we report the first symptom-onset rate for nonsurgical patients. In the few cases requiring surgery for decompression, primary resection, and patching of ectasias failed, subsequently requiring CSF shunting. We demonstrate imaging evidence of nearby plexiform neurofibroma in a majority of cases, which, when combined with histopathology, provides a novel explanation for the formation of dural ectasias.

Effect of Sequential Intrastromal Corneal Ring Segment Implantation and Corneal Collagen Crosslinking in Corneal Ectasia.

PURPOSE: To assess the clinical efficacy of sequential intrastromal corneal ring segment (ICRS) implantation and corneal crosslinking (CXL) in corneal ectasia. METHODS: This retrospective case series included eight eyes in which both ICRS implantation and CXL had been performed. CXL was performed within 1 month after ICRS implantation. The clinical outcomes (visual acuity, refractive errors, keratometry, and topographic indices) of these patients were compared with those of patients who had undergone only ICRS implantation (eight eyes) or CXL (20 eyes). RESULTS: Greater improvement in uncorrected visual acuity was observed in the ICRS + CXL group than in the ICRS or CXL alone groups at both 6 (p = 0.008) and 12 months (p = 0.028). Refractive errors of sphere and spherical equivalent were significantly reduced in both the ICRS (p = 0.002 at 6 months, p = 0.004 at 12 months) and ICRS + CXL groups (p < 0.001 at both 6 and 12 months). Keratometric values including the maximum, minimum, and average were significantly reduced in all 3 groups at postoperative 6 and 12 months; however, the greatest reductions were observed in the ICRS + CXL group (all p < 0.001). CONCLUSIONS: ICRS implantation followed by CXL within 1 month seems to be effective, and may be superior to ICRS or CXL alone in improving visual acuity and reducing refractive errors and keratometric values.

Orthokeratology on patient with ectasic cornea / Ortoceratologia no paciente com córnea ectásica

Abstract A 26-year-old man, single, business student, reveals a ectasic cornea during corneal topography exam. Among some procedures, the patient chose Orthokeratology to do a corneal reshape and got successfully a good visual acuity, going against the most authors guidance.

Resumo Estudante de 26 anos, masculino, estudante de economia, apresentou ao exame topográfico de córneas, ectasia corneal. Dentre todos os procedimentos apresentados, optou pela ortoceratologia para o remodelamento corneal, e obteve sucesso com melhora da acuidade visual, indo contra a orientação da maioria dos autores.

Bentall's Procedure for Annuloaortic Ectasia and Severe Aortic Regurgitation in a Patient With Repaired Tetralogy of Fallot and Aortic Valvular Reconstruction.

A 21-year-old female patient with repaired tetralogy of Fallot and aortic valvular reconstruction with aneurysmal aortic root and severe aortic regurgitation underwent aortic root replacement. Intrinsic aortopathy in tetralogy of Fallot and its surgical importance are highlighted.

Abdominal distension after eating lettuce: The role of intestinal gas evaluated in vitro and by abdominal CT imaging.

BACKGROUND: Some patients complain that eating lettuce, gives them gas and abdominal distention. Our aim was to determine to what extent the patients' assertion is sustained by evidence. METHODS: An in vitro study measured the amount of gas produced during the process of fermentation by a preparation of human colonic microbiota (n = 3) of predigested lettuce, as compared to beans, a high gas-releasing substrate, to meat, a low gas-releasing substrate, and to a nutrient-free negative control. A clinical study in patients complaining of abdominal distention after eating lettuce (n = 12) measured the amount of intestinal gas and the morphometric configuration of the abdominal cavity in abdominal CT scans during an episode of lettuce-induced distension as compared to basal conditions. KEY RESULTS: Gas production by microbiota fermentation of lettuce in vitro was similar to that of meat (P = .44), lower than that of beans (by 78 ± 15%; P < .001) and higher than with the nutrient-free control (by 25 ± 19%; P = .05). Patients complaining of abdominal distension after eating lettuce exhibited an increase in girth (35 ± 3 mm larger than basal; P < .001) without significant increase in colonic gas content (39 ± 4 mL increase; P = .071); abdominal distension was related to a descent of the diaphragm (by 7 ± 3 mm; P = .027) with redistribution of normal abdominal contents. CONCLUSION AND INFERENCES: Lettuce is a low gas-releasing substrate for microbiota fermentation and lettuce-induced abdominal distension is produced by an uncoordinated activity of the abdominal walls. Correction of the somatic response might be more effective than the current dietary restriction strategy.

Thirty-month results after the treatment of post-LASIK ectasia with allogenic lenticule addition and corneal cross-linking: a case report.

BACKGROUND: To report a first case of lenticule addition and corneal cross-linking for post-LASIK ectasia with associated corneal thinning. CASE PRESENTATION: Lenticule addition followed by corneal cross-linking was performed on the left eye of a patient with post-LASIK ectasia. Postoperatively, the corneal power and elevation were stable with a remarkable improvement in corneal thickness, and the lenticule had merged with the adjacent corneal stroma at 30 months follow-up. The patient's corrected distance visual acuity gained two lines. CONCLUSIONS: This case provides a potential treatment option for patients with keratectasia and keratoconus in association with thin corneal thickness (less than 400 µm) and may provide the benefit of delaying or avoiding the need for keratoplasty, which has its own associated complications and is limited by the availability of donor corneas.

Olfactory cleft dilatation.

The surgical technique of olfactory cleft dilatation consists in transmucosal lateral fracture-dislocation of the lateral wall of each olfactory cleft (i.e., of the turbinate wall of the ethmoid, composed, from anterior to posterior, of the middle, superior and supreme turbinates), in order to get access to the recess hosting the human olfactory mucosa and to the roof of the olfactory cleft (i.e., cribriform plate), with minimal trauma to the mucosa. Olfactory cleft dilatation is indicated for dysosmia secondary to constitutional stenosis of the olfactory clefts due to abnormal development of the ethmoid. Constitutional stenosis of the olfactory clefts should be differentiated from inflammatory obstruction and other diseases of the olfactory clefts, and especially from respiratory epithelial adenomatoid hamartoma, which enlarges the olfactory clefts and must be treated by resection. The technique of olfactory cleft dilatation is illustrated by three surgical cases. There was clear improvement in dysosmia in all three cases, without any complications. The place of constitutional olfactory cleft stenosis needs still to be defined in both diagnosis and treatment of dysosmia.

Surgical "Fat Patch" Improves Secondary Intracranial Hypotension Orthostatic Headache Associated with Lumbosacral Dural Ectasia.

BACKGROUND: Secondary intracranial hypotension is a clinical syndrome associated with reduction of cerebrospinal fluid volume and dural continuity violation. The main symptoms are orthostatic headache associated with nausea, vomiting, diplopia, dizziness, and tinnitus. The treatment is usually nonspecific. CASE DESCRIPTION: A 37-year-old woman developed secondary intracranial hypotension caused by lumbosacral iatrogenic dural ectasia following detethering surgery. An orthostatic headache was the mainstay of her clinical picture, and it was confirmed by intracranial pressure monitoring. Conservative treatment including spinal blood patch improved symptoms for a limited amount of time (<1 month). Altered compliance of the dural spinal sac was suspected. Therefore thecal sac remodeling by placing autologous fat at the level of the dural ectasia was performed, improving the symptoms for 2 years. CONCLUSION: Volumetric reduction of the epidural space may be considered as a valuable therapeutic option in case of intracranial hypotension that is unresponsive to medical treatments and spinal blood patch, as well as when an altered compliance of the dural sac is hypothesized.

A case of bilateral megaducts: Diagnostic and treatment methods.

INTRODUCTION: The pathology of the saliva glands comprises both tumoral and obstructive disorders. The latter include lithiasis, stenosis and megaduct. In this paper, we describe a clinical case of bilateral megaduct, a rare pathology, using sialo-MRI imaging and a conservative diagnostic-cum-therapeutic technique, sialendoscopy with dilation followed by catheterization. CLINICAL CASE: Our female patient presented oversized parotids with an unsightly deformation of the face (parotid ducts visible beneath the skin) and itchy cheeks, from which she had suffered for several years. Sialo-MRI revealed bilateral hypertrophied parotid saliva glands. We opted to perform diagnostic sialendoscopy to explore the branches of the salivary gland system and found ducts shaped like strings of sausages associated with mucous plugs. The treatment procedure was combined with rinsing of both parotid ducts in physiological serum followed by initiation of antibiotic-corticotherapy within the saliva ducts and, lastly, by placement of transpapillary drains, which were left in place for 10 days. Immediately following the procedure, the patient felt a considerable improvement regarding both local discomfort and her cheek deformation. Postoperative control at 10 weeks by sialo-MRI confirmed the reduction of the dilation of the salivary ducts. At 3 months, the patient continued to display a marked clinical improvement despite her saliva retaining a thick consistency. She no longer suffered from pruritis or deformation of the cheeks. DISCUSSION: Sialendoscopy could become the reference treatment tool since it is both efficient and conservative. Duration of her postoperative catheterization remains to be defined.

Topography-Guided PRK and Crosslinking in Eyes with Keratoconus and Post-LASIK Ectasia.

Topography-guided photorefractive keratectomy (TG-PRK) combined with corneal collagen crosslinking (CXL) has been shown to potentially improve vision and stabilize progression in patients with keratoconus (KC). We attempted to reproduce the previously published results using a different laser platform (AMARIS 500E) in patients with KC and post-LASIK ectasia (PLE). All of the 9 included eyes showed improved topography (Kmax, Kmean, RMS HOA, vertical coma, cylinder; p < 0.05) and improved visual acuity (BCVA, UCVA; p < 0.05) after 18 months. Despite some still uncontrollable factors, the combination of TG-PRK and CXL may be a promising option to regularize and stabilize corneas with KC and PLE and improve visual acuity.

[Therapeutic mega-ureter primitive before one year of life, retrospective study of 20years]. / Stratégie thérapeutique des méga-uretères primitifs avant 1 an de vie, étude rétrospective de 20 ans.

INTRODUCTION: What is the proper way to manage complicated primary mega-ureter in infants under the age of one. This has already been discussed in the literature but the controversy remains. OBJECTIVE: Evaluate the long-term results of the management of mega-ureter based support under the age of one. MATERIAL AND METHODS: Single-center retrospective study from 1990 to 2010. All children under one year found were evaluated including clinical examination, ultrasound, scintigraphy and cystography. They were divided into two groups: group 1: children operated on before the age of one year, group 2 non-operated or operated children after the age of one year. We analyzed the long-term evolution of these children on the following criteria: reflux, pyelonephritis, changes in dilation, renal function, need for surgical revision or secondary surgery, and impact on bladder function. RESULTS: In total, 54 patients were included in group 1 and 56 patients in group 2. In a median follow-up of 12 years. A total of 101 boys and 9 girls (sex-ratio 11.22). There were 57 left MUP (52%), 22 right (20%) and 31 bilateral (28%). A total of 71% of antenatal diagnosis. No difference on the emergence of complications: 25 (group 1) versus 31 (group 2) OR=0.69; 95% (0.307; 1.574); P=0.44. No difference between secondary surgery and revision surgery: group 1=12, group 2=22, OR=0.45; 95% CI (0.17, 1.09); P=0.06. No difference for daytime incontinence: OR=1.04; 95% CI (0.14; 7.64); P=0.67. Seventy-six children (69%) were finally made, 12 children operated twice (10.9%) and 34 children (31%) never made. CONCLUSION: The main challenge of the MUP of management is the preservation of renal function. Sixty-nine percent of our children received surgery due to impaired renal function lower than 30% of urethral dilatation greater than 10mm associated with reflux or recurrent pyelonephritis. Clinical monitoring, regular ultrasound and isotopic testing are necessary and should be extended to adulthood. LEVEL OF EVIDENCE: 5.

Japanese clinical practice guidelines for congenital biliary dilatation.

Until now, there have been no practical clinical guidelines for congenital biliary dilatation (CBD). In this review article, the Japanese Study Group on Congenital Biliary Dilatation (JSCBD) propose to establish clinical practice guidelines for CBD. Because the evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 20 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs included the distinct aspects of CBD: (1) Concepts and Pathology (three CQs); (2) Diagnosis (six CQs); (3) Pancreaticobiliary Complications (three CQs); Treatments and Prognosis (eight CQs). Each statements and comments for CQs were made by the guidelines committee members. CQs were finally approved after review by members of the editorial committee and the guidelines evaluation board of CBD. These guidelines were created to provide assistance in the clinical practice of CBD management; their contents focus on clinical utility, and they include general information on CBD to make this disease more widely recognized.

Corneal collagen crosslinking for corneal ectasias: a review.

PURPOSE: To review the published literature on corneal collagen crosslinking (CXL). METHODS: Importance has been placed on seminal publications, systemic reviews, meta-analyses, and randomized controlled clinical trials. Where such evidence was not available, cohort studies, case-controlled studies, and case series with follow-up greater than 12 months were examined. RESULTS: Corneal collagen crosslinking with riboflavin and ultraviolet A (UVA) 370 nm radiation appears to be capable of arresting the progression of ectatic corneal disorders, with most studies reporting significant improvements in visual, keratometric, and topographic measurements. Its mode of action at the molecular level is undetermined. Follow-up is limited to 5-10 years but suggests sustained stability and enhancement in corneal shape with time. Nearly all published long-term data and comparative studies are with epithelium-off techniques. Epithelium-on investigations suggest some efficacy but less than with epithelium-off treatments and long-term data are unavailable. Accelerated techniques with higher UVA fluencies and shorter treatments times, delivering the same UVA energy dosage, are the subject of recent investigation, with some laboratory and clinical studies suggesting reduced efficacy compared to the standard 3 mW/cm2 for 30 minutes irradiation procedure. Combined methodologies of CXL with techniques such as photorefractive keratectomy and intrastromal rings show promise but long-term follow-up is indicated. Sight-threatening complications of CXL are rare. CONCLUSIONS: Studies of epithelium-off CXL with irradiation at 3 mW/cm2 for 30 minutes support its efficacy. Refinement in techniques may allow for safer and more rapid procedures with less patient discomfort but require further investigation.

Aortic Root Dilatation in Mucopolysaccharidosis I-VII.

The prevalence of aortic root dilatation (ARD) in mucopolysaccharidosis (MPS) is not well documented. We investigated aortic root measurements in 34 MPS patients at the Children's Hospital of Orange County (CHOC). The diagnosis, treatment status, age, gender, height, weight and aortic root parameters (aortic valve annulus (AVA), sinuses of Valsalva (SoV), and sinotubular junction (STJ)) were extracted by retrospective chart review and echocardiographic measurements. Descriptive statistics, ANOVA, and paired post-hoc t-tests were used to summarize the aortic dimensions. Exact binomial 95% confidence intervals (CIs) were constructed for ARD, defined as a z-score greater than 2 at the SoV. The patient age ranged from 3.4-25.9 years (mean 13.3 ± 6.1), the height from 0.87-1.62 meters (mean 1.24 ± 0.21), and the weight from 14.1-84.5 kg (mean 34.4 ± 18.0). The prevalence of dilation at the AVA was 41% (14/34; 95% CI: 25%-59%); at the SoV was 35% (12/34; 95% CI: 20%-54%); and at the STJ was 30% (9/30; 95% CI: 15%-49%). The highest prevalence of ARD was in MPS IVa (87.5%). There was no significant difference between mean z-scores of MPS patients who received treatment with hematopoietic stem cell transplantation (HSCT) or enzyme replacement therapy (ERT) vs. untreated MPS patients at the AVA (z = 1.9 ± 2.5 vs. z = 1.5 ± 2.4; p = 0.62), SoV (z = 1.2 ± 1.6 vs. z = 1.3 ± 2.2; p = 0.79), or STJ (z = 1.0 ± 1.8 vs. z = 1.2 ± 1.6; p = 0.83). The prevalence of ARD was 35% in our cohort of MPS I-VII patients. Thus, we recommend screening for ARD on a routine basis in this patient population.