Distal Dilatation Research Articles

An Unusual Radiologic Image of Extensive Tumor Mass Infiltrating Hepatic Hilum without Signs of Cholestasis-A Case Report and a Literature Review of Non-Cancerous Lesions Mimicking Intrahepatic Cholangiocarcinoma.

Mass-forming intrahepatic cholangiocarcinoma (mICC) is the most frequent type of ICC. In contrast-enhanced computed tomography, mICC is visualized as a hypodense lesion with distal dilatation of intrahepatic bile ducts. The presented case illustrates the unusual manifestation of mICC in a 71-year-old male patient, where despite the extensive tumor mass and the hilar infiltration, the dilatation of intrahepatic bile ducts and cholestasis were not noted. A literature review on PubMed was performed. Primarily, 547 records were identified, and the titles and abstracts were systematically searched. Regarding the inclusion and exclusion criteria, 31 papers describing the non-cancerous liver lesions mimicking ICC were included in the further analysis. In 41.9% of the analyzed non-cancerous lesions, the obstruction of the bile ducts was not noted, similar to our patient. A significant cholestasis has been found in 30.03% of analyzed patients. The invasion of the liver hilum was noted in one-third of the patients. Atypical radiological features in lesions suspected of ICC, such as the absence of intrahepatic bile-duct dilation, are common in benign lesions. In the case of radiologically atypical lesions suspected of ICC, the diagnostic imaging needs to be correlated with clinical data, and the diagnosis should be confirmed with a pathological examination.

Dermoid sinus with distal dilation, meningocele and spina bifida in an English bulldog

AbstractA 1‐year‐old, male, entire English bulldog presented for progressive paraparesis. Magnetic resonance imaging and computed tomography revealed a dermoid sinus with distal dilation, meningocele and spina bifida. The lesion was successfully surgically resected, and histopathology confirmed the diagnosis of a dermoid sinus enveloped with dura mater. This case adds to the literature on dermoid sinuses and demonstrates that there can be variations within the classically described types of dermoid sinus.

The fate of the downstream aorta after total arch replacement.

The goal of this study was to evaluate the fate of the downstream aorta following total arch replacement. Between October 1999 and March 2018, a total of 740 patients underwent total arch replacement. After excluding connective tissue disease, previous descending or thoracoabdominal aortic surgery, patients without adequate preoperative images or operative mortality, late outcomes consisting of additional surgery for distal dilation and distal aortic events were evaluated in 623 survivors (240 aortic dissections, including 139 patients with acute dissection and 383 with a non-dissection aneurysm). The mean follow-up was 5.0 ± 4.0 years. The mean preoperative maximum diameter of the descending aorta was 36.9 ± 8.0 mm. An elephant trunk was inserted in 232 patients, including 183 patients with aortic dissection. Freedom from additional surgery for distal dilation was 88.5% at 5 years and 80.2% at 10 years. Freedom from distal aortic events was 81.9% at 5 years and 70.5% at 10 years. Multivariable regression analysis demonstrated that the preoperative diameter of the descending aorta was a significant risk factor for unfavourable distal aortic events. Computed tomography evaluation demonstrated a significant increase in the descending aortic diameter over time (P < 0.001). Positive aortic remodelling was observed in the proximal descending (P < 0.001) to mid-descending (P < 0.001) aorta exclusively in patients with acute aortic dissection. The diameter of the descending aorta increased significantly after total arch replacement, particularly in the distal descending aorta. The preoperative descending aortic diameter portended a significant risk for unfavourable distal aortic events.

A Novel Solution for Distal Dilation of Chronic Dissection After Repair Involving Visceral Branches: The Road Block Strategy.

AimNotwithstanding that unprecedented endovascular progress has been achieved in recent years, it remains unclear what is the best strategy to preserve the blood perfusion of abdominal visceral arteries and promote positive aortic remodeling in patients with distal dilatation of chronic aortic dissection in abdominal visceral part (CADAV) after aortic repair. The present study developed a Road Block Strategy (RBS) to solve this conundrum.Methods and ResultsThis prospective single-center clinical study included patients suffering from symptomatic distal dilatation of CADAV after aortic repair treated with RBS from January 2015 to December 2019 and followed up regularly for at least 2 years. Stent grafts were implanted first to cover distal tears and expand the true lumen. Device embolization was performed to induce proximal and distal segmental false lumen thrombosis (FLT) apart from the level of the ostia of vital branches. Successful RBS was performed in 13 patients. Significant differences were found in maximum true lumen diameter (p < 0.05), blood flow area in false lumen (FL) (p < 0.001), and the ratio of blood lumen to FL area (p < 0.05) between the pre-procedure and the latest follow-up results. No aortic rupture, vital branches occlusion, thoracic and abdominal pain, or death occurred during hospitalization and follow-up.ConclusionsOur findings suggest that RBS is feasible in treating distal dilatation of chronic aortic dissection after prior proximal repair, inducing false lumen thrombosis, preventing deterioration of aortic dissection, and maintaining the patency of abdominal visceral arteries.

PNEUMATOCELE FORMATION DUE TO SARS-COV2: A CASE REPORT

PNEUMATOCELE FORMATION DUE TO SARS-COV2: A CASE REPORT

Study of role of computerized tomography (CT) in evaluation of groove pancreatitis

Background: Groove pancreatitis is a rare form of chronic pancreatitis affecting the groove between the pancreatic head, duodenum and common bile duct. The exact cause of the disease is not known, although there are strong associations with long term alcohol abuse, functional obstruction of duct of Santorini and brunner gland hyperplasia. The purpose of this study was to describe the imaging findings of groove pancreatitis (GP) on Contrast enhanced CT Abdomen. Material and Methods: Present study was retrospective study conducted, with help of medical records of 16 patients with a final diagnosis of Groove pancreatitis. CT, MRI and MRCP findings were analysed. Statistical analysis was done using descriptive statistics. Results: In present study, two types of groove pancreatitis (GP) as pure type (50%) and segmental type (50%) were noted. Other important findings were focal duodenal wall thickening (62.5%) and cysts in the duodenal wall itself or in groove between the pancreatic head and the duodenum (37.5%), CBD dilatation and distal smooth tapering (62.5%) including all the segmental types and 2 of the pure type leading to intra- and extra-hepatic biliary system dilatation. MRI and MRCP were available in 6 patients in our study. There was a CT similarity regarding the sheet of tissues within the pancreaticoduodenal groove. These were seen expressing T1 hypo-intense and T2 slightly hyperintense signal in 3 patients with depiction of mild enhancement in the delayed phases in three of them (50%). On the MDCT examinations hypodense sheet at the PD groove was seen in 12 patients with modest enhancement identified in delayed phase seen in 6 of the them. Duodenal wall thickening was seen in 10 patients while associated cysts within the duodenal wall or in PD groove were seen in 6 patients. Pancreatic head enlargement with diffuse enhancement was seen in 8 patients. Mild pancreatic duct dilatation was seen in 8 patients while dilatation of the CBD was seen in 10 patients with distal tapering and intra-hepatic biliary dilatation. Conclusion: Groove pancreatitis (GP) is a disease that should be considered in the list of differential diagnosis of masses implicating the pancreatic head and medial duodenal wall. Imaging findings that are suggestive of GP include chronic inflammatory changes with fibrosis in the PD groove with or without implication of the nearby head of the pancreas, duodenal medial mural thickening with luminal stenosis and cysts at the PD groove or within the duodenal wall.

Possibility of hybrid surgery for haemodynamic correction in a child with left heart hypoplasia syndrome: Clinical observation

Owing to the development of prenatal diagnosis and interventional cardiology, newborns can survive complex palliative surgical procedures. Modern methods make it possible to change the principles of treatment for children undergoing haemodynamic correction, the last surgery being Fontan operation, and also stimulate the development of hybrid interventions (catheter procedures and surgical operations in the neonatal period). This article presents the dynamic monitoring of a child diagnosed with left heart hypoplasia syndrome, who underwent Norwood surgery at the age of 2 weeks, followed by restenosis of the distal anastomosis and balloon dilatation of neo-aorta restenosis with installation of a cobalt-chromium balloon-expandable stent (Andrastent XL) along with pararectal left-side access to the infrarenal section of the aorta at 11 months in order to reduce surgical risk and prepare for the next stage of correction (Glenn and Fontan operations).Received 18 March 2020. Revised 10 April 2020. Accepted 17 April 2020.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.

Dilatation of Aortic Neck and Common Iliac Arteries after Open Repair of Abdominal Aortic Aneurysms: Long-Term Follow-Up According to Aortic Reconstruction Configuration

Several studies in the literature report continued proximal aorta and distal iliac artery dilatation after surgical correction of an abdominal aortic aneurysm (AAA). The purpose of this study is to evaluate these findings, in a South American population, and relate them to the type of configuration of the open procedure aortic reconstruction. This is a retrospective review of ultrasonographic follow-up of patients submitted to open repair of AAA from 1989 to 2013, reporting proximal aorta dilatation (≥3cm) and distal iliac artery dilatation (≥1.5cm). A total of 155 patients were included. Life-table freedom at the intervals 11<15years and ≥15years were 47% and 23% for proximal dilatation and 63% and 38% for distal iliac arteries dilatation, respectively. There were more proximal and distal dilatations in patients submitted to more extensive aortic reconstructions (aorto-aortic 13% and 22% vs aorto-bilateral common iliacs 27% and 8% vs aorto-unilateral or bilateral external iliacs 27% and 32% and aorto-femoral 67% and 0%) P<0.0001. Juxtarenal anastomosis was also correlated with more proximal dilatations (42% vs 21%, P=0,046). There were two proximal and three distal anastomosis pseudoaneurysms. The presence of more extensive degenerative disease at the time of operation, requiring juxtarenal or more distal iliac reconstructions, may pose an increased risk of proximal aorta and iliac artery dilatation during follow-up. This study corroborates that significant changes are found after 7 to 10years of the operation, reinforcing the need for long-term monitoring.

Is the frozen elephant trunk technique justified for chronic type A aortic dissection in Marfan syndrome?

Chronic type A aortic dissection (cTAAD) in Marfan syndrome (MFS) is rare. Surgical experience is limited and the role of frozen elephant trunk (FET) technique remains undefined. We seek to evaluate the safety and efficacy of the total arch replacement (TAR) and FET technique for cTAAD in MFS. The clinical data of sixty-eight patients with MFS undergoing FET and TAR for cTAAD were analyzed. Mean age was 35.8±9.7 years and thirty-nine were male (57.4%). Operative mortality was 10.3% (7/68). Stroke occurred in one (1.5%), re-exploration for bleeding in five (7.3%), low cardiac output in four (5.9%), and acute renal failure in two (2.9%). Follow-up was complete in 100% (61/61) at mean 7.3±4.0 years. The false lumen was obliterated in 73.5% across FET and 50.0% in unstented descending aorta (DAo). Distal dilation occurred in twenty patients, six of whom underwent thoracoabdominal aortic replacement, one abdominal aortic replacement and one thoracic endovascular aortic repair (TEVAR). Late death occurred in five. At ten years, 59.8% were free from distal aortic dilation, and the incidences were 23.2% for death, 14.4% for distal reoperation, and 62.4% for reoperation-free survival. Predictors for operative mortality were extra-anatomic bypass [odds ratio (OR), 229.592; P=0.036], preoperative maximal size (DMax) of aortic sinuses (mm) (OR, 1.134; P=0.032) and cardiopulmonary bypass (CPB) time (minute) (OR, 1.061; P=0.041). Risk factors for aortic dilatation included patent false lumen at diaphragmatic hiatus [hazard ratio (HR), 5.374; P=0.008], preoperative DMax (mm) of proximal DAo (HR, 1.068; P=0.001) and renal arteries (HR, 1.102; P=0.005) which also predicted distal reoperation (HR, 1.149; P=0.001). The time from onset to operation (day) (HR, 1.002; P=0.004) and CPB time (minute) (HR, 1.032; P=0.036) predicted late death. This study shows that the TAR and FET technique is a safe and durable approach to cTAAD in patients with MFS. The operation should be performed as early as possible to optimize clinical outcomes.

V07-12 DEVELOPMENT OF A HIGH FIDELITY 3-PIECE INFLATABLE PENILE PROSTHETIC (IPP) MODEL USING 3D-PRINTING AND HYDROGEL MOLDING

You have accessJournal of UrologyLower-Tract Reconstruction II: ED/Infertility & Transgender Surgery (V07)1 Apr 2020V07-12 DEVELOPMENT OF A HIGH FIDELITY 3-PIECE INFLATABLE PENILE PROSTHETIC (IPP) MODEL USING 3D-PRINTING AND HYDROGEL MOLDING Patrick Saba, Rachel Melnyk, Michael Witthaus*, Gareth Warren, Divya Ajay, Alexander Cranwell, Ricardo Munarriz, and Ahmed Ghazi Patrick SabaPatrick Saba More articles by this author , Rachel MelnykRachel Melnyk More articles by this author , Michael Witthaus*Michael Witthaus* More articles by this author , Gareth WarrenGareth Warren More articles by this author , Divya AjayDivya Ajay More articles by this author , Alexander CranwellAlexander Cranwell More articles by this author , Ricardo MunarrizRicardo Munarriz More articles by this author , and Ahmed GhaziAhmed Ghazi More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000000897.012AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: This video demonstrates a high-fidelity, anatomical, full procedural simulation for placement of a 3-piece IPP. METHODS: Anatomical penile models consisting of a male pelvis, penis, urethra, corporal bodies, inguinal canals, iliac vessels and relevant adjacent structures were created using 3D-printed injection molds. The molds are filled with a poly-vinyl alcohol, a hydrogel that can be modified to replicate realistic tissue consistency. Relevant procedural steps: corporal exposure, dilation, measurement, scrotal pump placement, reservoir placement, prosthesis assembly and closure can be simulated using both an infrapubic or penoscrotal approach. The model has the ability to detect surgical errors including perforation of corpora during dilation, damage to iliac vessels or urethra and improper prosthesis placement. Twelve surgeons: three experts (>250 caseload), three low volume faculty (>50 caseload) and six novices (residents), completed an IPP insertion on the model under operating room conditions, after which a survey assessing realism and usefulness as a training tool was completed. RESULTS: When compared to live surgery, over 83.3% of the participants agreed that the model realistically replicated the skin retraction, placement of corporal stay sutures, corporotomy, distal dilation, prosthesis measurement, and IPP placement (Figure 1). Skin incision, proximal dilation, reservoir placement, corporal closure and skin closure was found to be realistic in above 66.7% of participants. On average experts agreed that the model could function as a training tool, assessment tool, prerequisite for IPP accreditation, and a requirement prior to live surgery. Experts stated ideally, they would have their trainees complete an average of 3.33 models before live surgical training. CONCLUSIONS: This video presents a high-fidelity, anatomical, hydrogel simulation platform for 3 piece IPP placement. With the ability to replicate surgical errors, its educational effectiveness as a training tool is further supported. This platform requires no specialized facilities and offers the basis for future male genital reconstruction simulations such as correction of Peyronie’s disease and artificial urinary sphincter insertions. Source of Funding: None © 2020 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 203Issue Supplement 4April 2020Page: e646-e646 Advertisement Copyright & Permissions© 2020 by American Urological Association Education and Research, Inc.MetricsAuthor Information Patrick Saba More articles by this author Rachel Melnyk More articles by this author Michael Witthaus* More articles by this author Gareth Warren More articles by this author Divya Ajay More articles by this author Alexander Cranwell More articles by this author Ricardo Munarriz More articles by this author Ahmed Ghazi More articles by this author Expand All Advertisement PDF downloadLoading ...

Extended repair for acute type A aortic dissection: long-term outcomes of the frozen elephant trunk technique beyond 10 years.

Long-term data are scarce regarding the efficacy of extended repair for acute type A aortic dissection (ATAAD) using the frozen elephant trunk and total arch replacement (FET + TAR) technique. We seek to evaluate our single-center experience with the FET + TAR technique in patients with ATAAD, focusing on early and long-term survival and reoperation. The early and long-term outcomes of FET + TAR were analyzed for 518 patients with ATAAD operated on between April 2003 and December 2012. Mean age 46.2±10.5 years and 426 were male (82.2%). The mean time from symptomatic onset to surgery was 4.8±3.7 days. Malperfusion occurred in 66 (12.7%) and Marfan syndrome (MFS) in 51 (9.8%). Bentall procedure was performed in 153 (29.5%), aortic cusp resuspension in 82 (15.8%), root remodeling (uni- or bi-Yacoub) in 19 (3.7%), ascending aortic replacement in 22 (4.2%) and extra-anatomic bypass in 15 patients (2.9%). The times of cardiopulmonary bypass (CPB), cross-clamp and selective antegrade cerebral perfusion were 201±50, 112±34, and 26±10 minutes, respectively. Operative mortality rate was 7.5% (39/518). Spinal cord injury occurred in 2.5% (13/518), stroke in 2.9% (15/518), re-exploration for bleeding in 2.5% (13/518) and acute kidney injury in 4.6% (24/518). Early reintervention with thoracic endovascular aortic repair (TEVAR) was performed in 3 (0.6%). Follow-up was complete in 98.7% (473/479) at mean 9.0±4.8 years (range 0.2-16.2). Late death occurred in 74, distal dilation in 31 and distal new entry in 9 patients. Late reoperation was performed in 31 patients, including TEVAR in 12, thoracoabdominal aortic replacement in 9, abdominal aortic replacement in 2, and anastomotic leak repair in 5. Survival and freedom from distal reoperation were 77.3% (95% confidence interval [CI] 72.9-81.1%) and 69.8% (95% CI 63.4-75.3%), and 92.9% (95% CI 89.9-95.0%) and 92.9% (95% CI 89.9-95.0%) at 10 and 15 years, respectively. Competing risks analysis showed that at 12 years, the incidence was 28.0% for death, 8.5% for distal reoperation, and 63.5% of patients were alive without reoperation. Multivariable analyses found that CPB time (in minutes) (odds ratio [OR], 1.011; 95% CI 1.006-1.017; P<0.001) and malperfusion syndrome (binary) (OR 2.291; 95% CI 1.283-6.650; P=0.011) were predictive of operative mortality, while multiple malperfusion predicted late death (hazard ratio, HR 6.815; 95% CI 2.447-18.984; P<0.001). Risk factors for late death and distal reoperation included MFS (HR, 1.824; 95% CI 1.078-3.087; P=0.025) and malperfusion (HR, 1.787; 95% CI 1.042-3.064; P=0.035). In this large series of patients with ATAAD, the FET + TAR technique has achieved favorable early and long-term survival and freedom from reoperation up to 15 years. Marfan syndrome and malperfusion syndrome were risk factors for early and late mortality and distal reoperation. This study adds long-term evidence supporting the use of the FET + TAR technique in patients with ATAAD involving the arch and descending aorta.

1291 Pancreatico-Biliary Stricture by Benign Hepatic Cysts

INTRODUCTION: Hepatic cysts are frequently identified incidentally, and usually do not require any follow up. We present a case of benign, large hepatic cysts which were extrinsically compressing on biliary ductal system and caused a fusiform-dilitation of the hepatic ducts, distal dilation of the common bile duct (CBD) and a narrowing of the pancreatic duct. CASE DESCRIPTION/METHODS: A 77-year-old male presented to our facility for drainage of a large liver cyst. Initial discovery was incidental, but follow up imaging showed increased solid component in one of the cysts. At the time of presentation he was completely asymptomatic and laboratory studies were all within normal range. Computed Tomography (CT)-guided drainage of the hepatic cyst yielded 250 cc of brown fluid. The cytology was benign. Patient then presented to one-month follow up appointment with painless jaundice, and elevated total bilirubin of 6.7 mg/dL. Magnetic Resonance Cholangiopancreatography (MRCP) was performed and showed filling defects of the CBD and bilateral hepatic ducts. Elective Endoscopic Retrograde Cholangiopancreatography (ERCP) a few days later showed dilated CBD and strictures of biliary duct at the bifurcation and pancreatic duct at the neck. The stricture appeared to be from extrinsic compression. We were concerned about infected cyst, and repeated cyst drainage. Previsouly drained cyst yielded 200 cc for purulent fluid; the adjacent cyst was also drained and it yielded &gt;1 L of clear fluid. At this time, patient had leukocytosis of 13.7 K, total bilirubin 6.2 mg/dL, ALP 433 IU/L. Patient was admitted post-procedure and was placed on broad-spectrum antibiotics. Culture grew Actinomyces species, and patient was discharged to home on long term oral antibiotics. ERCP was repeated two weeks later, and it showed fusiform dilation of intrahepatic duct, though stricture of the biliary tract and pancreatic duct were now resolved. There was no communication between the biliary tract and the hepatic cysts. CT of the abdomen showed persistent fluid collections, the patient ultimately underwent laparoscopic resection of hepatic cyst with partial hepatectomy, cyst fenestration and cholecystectomy. Pathology confirmed that it was indeed a benign cyst. DISCUSSION: It appears that due to close abutment of two large cysts caused compression of the ductal system. This unusual presentation of benign liver cyst leading to stricture of bile and pancreatic duct by extrinsic compression is worth a report.

Can Frozen Elephant Trunk Cure Type I Dissection Confined to Thoracic Aorta in Marfan Syndrome?

This study sought to evaluate the long-term impact of frozen elephant trunk (FET) on the distal aorta of patients with Marfan syndrome (MFS) who had type I dissection confined to the thoracic aorta (above the diaphragmatic hiatus). Between 2003 and 2016, 42 patients with MFS (Ghent or revised Ghent criteria) (age 33.3 ± 8.9 years; 27 men; 64.3%) sustaining type I dissection above the diaphragmatic hiatus involving the aortic arch (22 acute; 52.4%) underwent total arch replacement and FET. Dissection extended distally to the mid-descending aorta in 32 (76%) and to above the diaphragmatic hiatus in 10 (24%) patients. Operative mortality was 4.8% (2 of 42). Follow-up was 100% at 6.3 ± 3.0 years. Maximal aortic sizes at the mid-descending aorta, diaphragmatic hiatus, renal arteries, and largest segment of abdominal aorta were 22.8, 21.1, 19.1, and 19.9 mm preoperatively and 23.1, 22.0, 19.8, and 22.4 mm on the latest computed tomographic angiography. Dilation and complete remodeling of the distal aorta occurred in 10.0% (4 of 40) and 90% (36 of 40) of patients, respectively. One late death occurred, and 3 distal reoperations were performed. Preoperative abdominal aortic maximal aortic size was predictive of distal dilatation (mm) (hazard ratio, 1.78; P= .021) and reoperation (≥25 mm vs <25 mm) (hazard ratio, 12.88; P= .037). At 10 years, freedom from dilation, reoperation, and death were 69.8%, 78.1%, and 90.0%, respectively. At 8 years, the rates of death, reoperation, and reoperation-free survival were 10%, 11%, and 79%, respectively. The FET technique has a positive remodeling impact on type I dissection confined to the thoracic aorta in patients with MFS. This study adds evidence supporting the safety and durability of this extended approach for type I aortic dissection in MFS.

Predicting Underlying Neoplasms in Appendiceal Mucoceles at CT: Focal Versus Diffuse Luminal Dilatation.

OBJECTIVE. The purpose of this study was to determine whether a novel morphologic characteristic of appendiceal mucoceles at CT-focal distal appendiceal dilatation with a segment of morphologically normal appendix proximally-could predict an underlying neoplastic pathologic abnormality before surgery and histopathologic assessment. MATERIALS AND METHODS. A retrospective study was performed that assessed CT cases from 2012 through 2014. Cases showing morphologic features of a mucocele were identified and categorized into two subgroups: focal distal dilatation with a segment of normal appendix proximally and generalized appendiceal dilatation. The underlying histopathologic diagnosis for each case was assessed and categorized as neoplastic or nonneoplastic. Several additional morphologic findings were also assessed. RESULTS. Forty-nine cases with confirmed histopathologic diagnoses were identified. Of those, 20 of 23 (87.0%) cases with the finding of focal distal dilatation had an underlying neoplastic cause, whereas 14 of 26 (53.8%) cases with generalized dilatation had an underlying neoplastic cause (p = 0.012). The findings of periappendiceal fat stranding (p = 0.004), mural calcification (p = 0.006), and degree of luminal dilatation (p = 0.002) also reached statistical significance. When seen in combination with focal distal dilatation, the positive predictive value for underlying neoplasm approached or reached 100%. CONCLUSION. Our study shows that isolated focal distal appendiceal dilatation with a segment of morphologically normal appendix proximally is significantly associated with an underlying neoplastic histopathologic cause. When seen in combination with mural calcification, a diameter of more than 2 cm, and absence of periappendiceal stranding, an underlying neoplastic cause is strongly suggested.

Thoracic endovascular repair of a rare case of leaking aortic arch intramural hematoma secondary to Giant cell arteritis

BackgroundTraditionally thoracic aortic aneurysms (TAA) secondary to Giant Cell Arteritis (GCA) were treated with resection and open repair. However no prior studies have reported an aortic intramural hematoma (IMH) as a presentation of GCA or outcome of thoracic endovascular aortic repair (TEVAR) in TAA or IMH secondary to GCA.Case presentationA 59 year old female, nonsmoker, non-hypertensive, non-diabetic with a known history of GCA, temporal arteritis on prednisone presented with shortness of breath & chest pain. Chest CT revealed aortic arch IMH and large left hemothorax. CTA confirmed distal aortic arch focal dilation, a focal intimal irregularity in the distal aortic arch and extensive IMH without any active extravasation or signs of aortitis. Patient underwent an urgent TEVAR without oversizing the aortic landing zones. Post TEVAR aortogram showed exclusion of the site of IMH origin and dilated aortic arch segment by the stent and absence of active extravasation. One month post-TEVAR CTA showed patent stent graft with resolution of IMH and hemothorax. One year after TEVAR, patient remained asymptomatic.ConclusionGCA can present as an IMH secondary to underlying chronic vasculitis. When endovascular repair is considered, great care should be taken not to grossly oversize aortic landing zones.

The fate of the downstream aorta after open aortic repair for acute DeBakey type I aortic dissection: total arch replacement with elephant trunk technique versus non-total arch replacement†

The aim of this study was to evaluate the fate of the downstream aorta following open aortic repair for acute DeBakey type I aortic dissection comparing total arch replacement (TAR) with the elephant trunk (ET) technique versus non-total arch replacement (non-TAR). From October 1999 to December 2016, 267 patients underwent open repair for acute DeBakey type I aortic dissection. A tear-oriented strategy was mainly used to determine the extent of graft replacement. Hospital mortality was 10.0% (12/120 patients) in the TAR group and 17.0% (25/147 patients) in the non-TAR group (P = 0.070). Late outcomes were compared in 230 hospital survivors (TAR: n = 108 and non-TAR: n = 122). Mean follow-up was 6.5 ± 4.6 years. The aortic diameters were measured at 4 levels, across 6 time points using computed tomography. Freedom from additional aortic surgery for distal dilation was significantly better in the TAR group than the non-TAR group (TAR: 97.5 ± 1.8% at 5 years and non-TAR: 88.2 ± 3.4% at 5 years, P = 0.045). Freedom from a distal aortic event was also significantly better in the TAR group compared with the non-TAR group (TAR: 97.2 ± 1.6% at 5 years and non-TAR: 80.7 ± 4.2% at 5 years, P = 0.013). In the non-TAR group, the aortic arch diameter significantly increased (P < 0.001). Significant aortic remodelling occurred at the proximal descending aorta in the TAR with ET group (P < 0.001). The TAR with ET reduced the need for additional distal aortic repair compared to non-TAR. TAR with ET prevented unfavourable aortic growth in both the aortic arch and the proximal descending aorta.

Endoscopic vacuum therapy for a large esophageal perforation after bariatric stent placement.

Endoscopic vacuum therapy for a large esophageal perforation after bariatric stent placement.

Clinical diagnosis of Larsen syndrome, Stickler syndrome and Loeys-Dietz syndrome in a 19-year old male: a case report

BackgroundLarsen syndrome is a hereditary disorder characterized by osteochondrodysplasia, congenital large-joint dislocations, and craniofacial abnormalities. The autosomal dominant type is caused by mutations in the gene that encodes the connective tissue protein, filamin B (FLNB). Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by arterial aneurysms, dissections and tortuosity, and skeletal, including craniofacial, manifestations. Mutations in five genes involved in the transforming growth factor beta (TGF-β) signaling pathway cause five types of LDS. Stickler syndrome is a genetically heterogeneous arthro-ophthalmopathy caused by defects in collagen, exhibiting a wide specter of manifestations in connective tissue. A rare case is reported that was diagnosed with all these three hereditary connective tissue disorders.Case presentationA 19 year-old, Norwegian male with a clinical diagnosis of Larsen syndrome and with healthy, non-consanguineous parents attended a reference center for rare connective tissue disorders. Findings at birth were hypotonia, joint hypermobility, hyperextended knees, adductovarus of the feet, cervical kyphosis, craniofacial abnormalities, and an umbilical hernia. From toddlerhood, he required a hearing aid due to combined conductive and sensorineural hearing loss. Eye examination revealed hyperopia, astigmatism, and exotropia. At 10 years of age, he underwent emergency surgery for rupture of an ascending aortic aneurysm. At 19 years of age, a diagnostic re-evaluation was prompted by the findings of more distal aortic dilation, tortuosity of precerebral arteries, and skeletal findings. High throughput sequencing of 34 genes for hereditary connective tissue disorders did not identify any mutation in FLNB, but did identify a de novo missense mutation in TGFBR2 and a nonsense mutation in COL2A1 that was also present in his unaffected father. The diagnosis was revised to LDS Type 2. The patient also fulfills the proposed criteria for Stickler syndrome with bifid uvula, hearing loss, and a known mutation in COL2A1.ConclusionLDS should be considered in patients with a clinical diagnosis of Larsen syndrome, in particular in the presence of arterial aneurysms or tortuosity. Due to genetic heterogeneity and extensive overlap of clinical manifestations, genetic high throughput sequencing analysis is particularly useful for the differential diagnosis of hereditary connective tissue disorders.

Effectiveness of the proximal optimization technique for longitudinal stent elongation caused by post-balloon dilatation.

To evaluate the effectiveness of the proximal optimization technique (POT) to prevent longitudinal stent elongation. The mechanism of stent elongation, which occurs after post-balloon dilation, is still unclear. A total of 103 lesions treated with optical coherence tomography guided coronary intervention between May 2013 and November 2017 were retrospectively analyzed. Lesions were divided according to the circumferential degree of malapposition at the stent edge immediately after deployment into well-apposed group (<180°) and malapposed group (≥180°). Post-dilation was performed from distal to proximal within the stent until August 2016 (non-POT cohort), and POT was applied thereafter (POT cohort). In the POT cohort, post-dilation was done at the proximal portion of the stent with sufficiently large balloon to minimize malapposition followed by distal dilatations. Stent elongation length was defined as the change in stent length from stent deployment to after post-dilatation. In the non-POT cohort, 72 lesions, including 54 lesions in the well-apposed group and 18 in the malapposed group were analyzed. Stent elongation length was significantly longer in the malapposed group than in the well-apposed group (1.51 ± 1.34 mm vs 0.13 ± 0.84 mm, P < 0.01). In the POT cohort, 31 lesions including 21 in the well-apposed group and 10 in the malapposed group were analyzed. Stent elongation length was not significantly different between the groups (-0.09 ± 0.91 mm vs 0.30 ± 0.99 mm, P = 0.29). Malapposition of the stent edge is responsible for longitudinal stent elongation caused by post-dilatation. POT appeared to effectively prevent longitudinal stent elongation.

Good recovery of subarachnoid hemorrhage concomitant with ischemia due to anterior cerebral artery dissection by conservative treatment: A case report.

The present report describes the case of a 57-year-old woman presenting with subarachnoid hemorrhage (SAH) and acute ischemic stroke (AIS) due to anterior cerebral artery (ACA) dissection, which exhibited severe stenosis at the origin with distal dilatation of the A2 segment and occlusion of the A3 segment. In this case, computed tomography (CT) revealed SAH in right superior frontal sulcus and the interhemispheric fissure. Magnetic resonance imaging demonstrated acute infarct in the territory of the right ACA. Brain digital subtraction angiography showed severe stenosis at the origin of the A2 segment with distal dilatation and occlusion at the origin of the A3 segment of the right ACA, suggesting a diagnosis of dissection. Only treatment with atorvastatin, her clinical condition subsequently improved. The stenosis and dilatation of A2 segment were ameliorated as demonstrated by a follow-up CT angiography 5 months after onset. SAH concomitant with ischemia caused by ACA dissection is rare. Conservative treatment may be a safe and effective choice for patients with SAH concomitant with AIS due to ACA dissection.