Retroperitoneal liposarcoma and craniosynostosis: possible genomic relationship, case report, and literature review.

Based on a case report, this review explores the genomic landscape for patients with liposarcomas and possible relationships with gene mutations related to craniosynostosis. We describe the case of a 40-year-old man, known for a surgical correction of craniosynostosis before the age of 1 year, who underwent a radical resection of a voluminous retroperitoneal liposarcoma; histopathological analysis revealed a low-grade well-differentiated, mostly sclerosing, liposarcoma. A genetic analysis searching for mutations in blood DNA was performed and did not detect any specific mutation. A literature review was also conducted. Several tumors related to syndromic and non-syndromic craniosynostosis are mentioned in the literature; no specific link with retroperitoneal liposarcoma is established but the FGFR3 mutation is detected in dedifferentiated liposarcomas. To date, no case has been reported in the literature demonstrating a genetic relationship between craniosynostosis and low-grade differentiated retroperitoneal liposarcoma. We conclude that further studies for gene complex mutations should be conducted to show a possible genetic relationship between retroperitoneal liposarcoma and craniosynostosis.

Micron-sized PFOB liquid core droplets stabilized with tailored-made perfluorinated surfactants as a new class of endovascular sono-sensitizers for focused ultrasound thermotherapy.

The purpose of this study was to develop micron-sized droplet emulsions able to increase the heat deposition of high intensity focused ultrasound (HIFU), aiming to accelerate the tumour ablation in highly perfused organs with reduced side effects. The investigated droplets consisted of a perfluorooctyl bromide (PFOB) core coated with a biocompatible fluorinated surfactant called F-TAC. The novelty of this work relies on the use, for this application, of a high boiling point perfluorocarbon core (142 °C), combined with an in-house fluorinated surfactant to formulate the emulsion, yielding quasi-reversible strong interactions between the HIFU beam and the droplets. In order to fine-tune the emulsion size, surfactants with different hydrophobic/hydrophilic ratios were screened. Different concentrations of PFOB droplets were homogeneously embedded in two different MRI compatible materials, exhibiting either ultrasound (US) absorbing or non-absorbing properties. For the US absorbing TMM, the speed of sound at each droplet concentration was also assessed. These TMM were sonicated by 1 MHz HIFU with acoustical power of 94 W at two different duty cycles. The temperature elevation was monitored accurately by MRI proton shift resonance frequency in near real-time. The presence of sono-sensitive droplets induced a significant increase of the HIFU thermal effect that persisted under repeated sonication of the same locus. Optimal enhancement was observed at the lowest concentration tested (0.1%) with an additional temperature rise at the focal point of approximately 4 °C per applied kJ of acoustic energy corresponding to one order of magnitude augmentation of the thermal dose. Furthermore, no deformation of the heating pattern pre- or post-focal was observed.

Endovascular Treatment of Arterial Complications After Liver Transplantation: Long-Term Follow-Up Evaluated on Doppler Ultrasound and Magnetic Resonance Cholangiopancreatography.

PURPOSE: To evaluate long-term arterial patency and abnormalities of bile ducts in patients that had endovascular treatment for arterial complications after liver transplantation (LT). MATERIALS AND METHODS: Between 2004 and 2014, 1048 LTs were consecutively performed in our institution and 53 patients (42 men; age range 19-69) were diagnosed and treated by endovascular techniques for arterial complications such as stenosis, thrombosis, dissection or kinking of the hepatic artery (HA). Radiological and surgical data were retrospectively analyzed, and survivors were contacted to undergo follow-up Doppler ultrasound (DUS) of the HA and magnetic resonance cholangiopancreatography. RESULTS: The primary technical success of endovascular treatment was 94% (n = 50). The patency rate of HA at 5-year was 81%. After a median follow-up of 58 months, 17 patients (32%) developed radiological features of ischemic cholangiopathy (IC), including 7 patients with abnormal DUS and 10 with normal DUS. Patients who presented with complications of the HA in the first 3 months after LT developed IC more frequently (42%) than others (12%) (p = 0.028). No other factor was associated with the development of IC. CONCLUSION: IC was more often observed when HA complication occurred within the first 3 months after LT. The presence of IC was not excluded by a normal DUS during follow-up.

Ethylene vinyl alcohol copolymer for occlusion of specific portal branches during preoperative portal vein embolisation with n-butyl-cyanoacrylate.

OBJECTIVES: To evaluate the safety and efficacy of ethylene vinyl alcohol copolymer (EVOH) injection for selective occlusion of portal branches considered at risk for non-target embolisation during preoperative portal vein embolisation (PVE). METHODS: Twenty-nine patients (mean age, 57 ± 17 years) submitted to PVE with n-butyl-cyanoacrylate (NBCA) and additional EVOH for selected portal branches were retrospectively analysed. Indications for the use of EVOH and the selected portal branches were evaluated. Degree of hypertrophy of the future liver remnant (FLR) and kinetic growth were assessed by CT volumetry performed before and 3-6 weeks after PVE. Clinical outcome and histopathological analysis of portal veins occluded with EVOH were reviewed. RESULTS: EVOH was indicated intraoperatively for embolisation of selected portal branches that the operator reported at risk to provoke non-target embolisation with NBCA. Indications for the use of EVOH were embolisation of segment IV (n = 21), embolisation of segmental portal branches with early bifurcation (n = 7) and PVE in a 1-year-old girl with cystic hamartomas. All targeted portal branches were successfully embolised. There were no cases with non-target embolisation by EVOH. The degree of hypertrophy of the FLR was 14.3 ± 8.1% and the kinetic growth rate was 2.7 ± 1.8% per week. CONCLUSION: EVOH is safe and effective for embolisation of selected portal vein branches considered at risk for non-target embolisation. KEY POINTS: • EVOH is another effective liquid embolic agent for preoperative PVE. • EVOH is relatively simple to handle with a minimal risk of non-target embolisation. • During PVE, some portal branches considered complicated to occlude with NBCA may be efficiently embolised with EVOH.

Evaluation of port site hernias, chronic pain and recurrence rates after laparoscopic ventral hernia repair: a monocentric long-term study.

OBJECTIVE: The aim of this study was to evaluate hernia appearance at the trocar site after laparoscopic treatment of primary or incisional ventral hernias using an intraperitoneal prosthetic mesh. Chronic pain at the trocar site and primary hernia recurrence were also evaluated. PATIENTS AND METHODS: Two-hundred and twenty-six consecutive patients who underwent a standardized laparoscopic hernia repair for primary or incisional ventral hernia at our centre between January 2000 and December 2008 were included. All patients had clinical and radiological examinations. Primary end points were port site hernia and the occurrence of chronic trocar site pain. Secondary end point was primary hernia recurrence. RESULTS: Seventy-eight patients were excluded: 6 declined to participate, 48 were unreachable, and 24 did not meet the inclusion criteria (nine underwent a single site laparoscopic approach, ten died of unrelated disease, three were unable to visit the hospital and two had relocated). After exclusion, 148 remained in our study. Mean follow-up was 49 ± 12.6 months. Mean age at the time of surgery was 60 years (range, 28-83) In total, 504 port sites were clinically and radiologically evaluated, and only one (0.02%) had secondary herniation. Three patients (2.0%) had a recurrent hernia, and 14 (9.5%) had developed chronic pain at time of assessment. Nine patients (6.1%) were re-operated for the recurrent hernia before the follow-up evaluation. The overall recurrence rate is, therefore, 8.1%. Only two minor complications and no major complications occurred after surgery. No mortality was observed. CONCLUSION: Laparoscopic repair for primary or incisional ventral hernias is a safe surgical approach, with low rates of hernia recurrence and a low morbidity rate. When fascial closure is maintained for 10 mm port sites, the incidence of port site hernias is very low. Five millimetre ports do not require closure.

[Multidisciplinary management of localized rectal cancer]. / Prise en charge multidisciplinaire du cancer localisé du rectum.

Rectal cancer remains a frequent pathology, with a good prognosis, according to a proper management. During the last decades, we have been confronted with important improvements, notably regarding the diagnosis and the treatment. In the era of highly specialized medicine, it is clear that the management must be multidisciplinary, incorporating not only the surgeon, the oncologist and the radiation oncologist, but also the radiologist, the gastroenterologist, and the pathologist. We aim to review the recent concepts and the future developments in the management of rectal cancer.

Le cancer du rectum demeure une pathologie fréquente, dont le pronostic est heureusement bon. Ces dernières décennies, nous avons été confrontés à plusieurs avancées importantes, que ce soit au niveau du diagnostic ou du traitement. Sa prise en charge fait partie intégrante de la médecine hautement spécialisée, et il est devenu clair que l'approche se doit d'être multidisciplinaire, incorporant aussi bien le chirurgien, l'oncologue et le radio-oncologue, que le radiologue, le gastroentérologue et le pathologue. Dans cet article, les concepts récents ainsi que les perspectives futures sont analysés.

Liver volume is a prognostic indicator for clinical outcome of patients with alcoholic hepatitis.

PURPOSE: To evaluate the prognostic value of abdominal computed tomography (CT) in patients with alcoholic hepatitis (AH). METHODS: This ancillary study was based on data collected during a previous randomized controlled trial in patients with AH. Clinical response was defined as the improvement of the baseline MELD score ≥3 points at 3 months. All patients underwent contrast-enhanced CT of the abdomen. The following parameters were measured: (1) liver density, spleen density, and liver-to-spleen density ratio; (2) liver-to-body weight (LBW) ratio; and (3) subcutaneous fat, visceral fat, and muscular content. Improvers and non-improvers were compared with univariate, multivariate, and ROC analyses. Results were compared with a validation cohort of patients. RESULTS: Fifty-eight patients (mean age, 56 years) were analyzed, including 34 (59 %) improvers. On multivariate analysis, LBW ratio (OR = 3.73; 95 % CI, 1.65-8.46; p = 0.002) and subcutaneous fat (OR = 1.01; 95 % CI, 1.00-1.02; p = 0.022) were associated with clinical response, with AUROC curves of 0.78 ± 0.06 (p < 0.001) and 0.66 ± 0.07 (p = 0.043), respectively. LBW ≥2.4 % predicted response with 88 % sensitivity and 63 % specificity. In the validation cohort (n = 42, 64 % improvers), the same cut-off value predicted response with 93 % sensitivity and 60 % specificity. CONCLUSIONS: In patients suffering from AH, the liver volume appears to be a major positive prognostic factor.

Peribiliary Gland Dilatation in Cirrhosis: Relationship with Liver Failure and Stem Cell/Proliferation Markers.

BACKGROUND AND AIMS: Dilated peribiliary glands (PBG) in patients with cirrhosis are often an incidental finding although their significance and physiopathology remain unclear. We aimed to identify clinical factors associated with dilated PBG and to perform a detailed morphometric assessment of dilated PBG in cirrhotic patients undergoing liver transplantation (LT). METHODS: All consecutive cirrhotic patients undergoing LT at our institution between October 2006 and October 2011 were assessed for inclusion. Ten non-cirrhotic patients were included as controls. We performed morphometrical assessment of PBG, assessed baseline clinical factors associated with dilated PBG, immunohistochemistry staining with CK-19, MiB-1 and EpCAM, and radiological assessment of all available cases. RESULTS: Seventy-one patients met the inclusion criteria, 24% had PBG dilatation of >1000 µm. On multivariable analysis, MELD (OR 1.11 per unit increase in MELD, p = 0.004) was the only significant factor associated with dilated PBG. Compared to PBG < 1000 µm, large PBG had a higher proportion of EpCAM-positive (69 vs. 28%, p < 0.001) and MiB-1-positive lining cells (2.8 vs. 0.55%, p = 0.036). Computed tomography and magnetic resonance imaging had high specificity but low sensitivity for the diagnosis of dilated PBG > 1000 µm (specificity 90-100%, sensitivity 25-29%). CONCLUSIONS: Dilated PBGs are a common finding in explants of cirrhotic subjects undergoing LT and are associated with liver failure although diagnostic performance of cross-sectional imaging is inconstant. The high number of proliferative and EpCAM-positive cells lining the PBG may suggest a role of PBG in organ repair during liver failure.

Hereditary haemorrhagic telangiectasia: to transplant or not to transplant - is there a right time for liver transplantation?

BACKGROUND & AIMS: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. METHODS: Herein, we report the case of a 65-year-old female patient with a 'double Osler syndrome' consisting of hereditary haemorrhagic telangiectasia (HHT) and type I hereditary angioedema diagnosed at the age of 25 and 22 years respectively. RESULTS: Hereditary angioedema was treated with danazol for several decades until multiple hypoechogenic liver masses were detected. Albeit danazol treatment was replaced by C1 esterase inhibitor infusions, hepatocellular carcinoma was diagnosed at the age of 64 and the patient was listed for liver transplantation. HHT was marked by recurrent epistaxis until the age of 63 when severe intestinal bleeding occurred. At the age of 65, severe dyspnoea (NYHA class IV) developed and rapidly progressive high-output cardiac failure was diagnosed. Despite argon plasma coagulation to control bleeding from intestinal angiodysplasia, and treatment with bevacizumab to inhibit angiogenesis, the patient died from severe gastrointestinal bleeding associated with cardiogenic shock at the age of 66 before being transplanted. CONCLUSION: The indication to list this patient for liver transplantation was debated several times before the diagnosis of hepatocellular carcinoma because of good general condition and low MELD score. Precise guidelines for screening and management of patients with hepatic HHT need to be better defined.

[Giant hernias with loss of domain: what is the best way to prepare patients?]. / Hernies géantes avec perte de droit de cité: comment preparer au mieux les patients?

Giant hernias with loss of domain induce physiological modifications that impair quality of life and make more complex their surgical management. A good preparation of patients before surgery is the key to an eventless postoperative course. The progressive pre-operative pneumoperitoneum (PPP) is one of the described abdominal augmentation protocols which can help patients to tolerate hernia content reintegration and avoid components separation technique during hernia repair. This article describes the management of these complex patients. We also report the case of a patient who follows successfully a PPP protocol.

Sequential transarterial chemoembolization and portal vein embolization before resection is a valid oncological strategy for unilobar hepatocellular carcinoma regardless of the tumor burden.

OBJECTIVE: To investigate the long-term oncological outcome of patients with resectable hepatocellular carcinoma (HCC) undergoing sequential transarterial chemoembolization (TACE) and portal vein embolization (PVE). METHODS: Analysis of all Child A HCC patients who underwent TACE-PVE before major liver resection from 2006 to 2012 was performed according to whether or not they underwent surgical resection as planned. RESULTS: 54 patients (50 men, 93% median 69-years (range 44-87)) were included. Thirty-nine (72%) patients underwent resection, including 19/25, 16/23, and 4/6 of patients with BCLC A, B, and C (p = 0.839). Twenty-two (56%) had tumor recurrence (median delay 10 months) including 9/19, 11/16, and 2/4 of the patients with BCLC A, B, and C (p = 0.430). Survival was significantly better in resected patients as compared to those who were not resected (median overall survival (OS): 44 vs. 18 months; p < 0.001). Recurrence was associated with a poorer prognosis as compared to patients without recurrence (median OS 43 months vs. not reached; p < 0.001). BCLC stage did not influence survival (p = 0.13). CONCLUSION: In patients with large unilobar HCC, TACE-PVE leads to resection in most patients, with a good oncological outcome regardless of the tumor burden. When this strategy fails, patients can be managed with TACE despite prior PVE.

Emergency Computed Tomography: What is Missed at First Reading?

OBJECTIVES: The aim of this study was to evaluate the accuracy of preliminary computed tomography (CT) interpretations made by radiology residents in the emergency department. METHODS: For 4 periods of 20 consecutive days, attending radiologists prospectively recorded any disparities between their own CT interpretations (reference standard) and the preliminary interpretations of emergency radiology residents. Misinterpretations were defined as major when related to a potentially life-threatening pathology if not immediately managed after CT. The rate of disparities was calculated for all CT examinations, separately for body and neuroradiological cases, and for working and on-call hours. RESULTS: A total of 3044 emergency CT examinations were performed during the survey: 1568 (51%) body scans and 1476 (49%) neurological scans. Disparities were reported in 145 (4.8%) of all CT examinations, with 0.8% (24/3044) defined as major misinterpretations. There were 100 (6.3%) of 1568 disparities in body CT versus 45 (3.0%) of 1476 in neurological CT examinations (P = 0.001). No significant differences were found between disparities recorded during regular working hours (47/1083 [4.3%]) versus on-call hours (98/1961 [5.0%], P = 0.47). CONCLUSIONS: There were typically more disparities between resident and attending interpretations in emergency body CT. Major disparities represented only a small proportion of all misinterpretations. The detailed analysis of our data enables benchmarking over time and offers a reference for optimizing the training of residents in emergency radiology.

Cone Beam Computed Tomography (CBCT) in the Field of Interventional Oncology of the Liver.

Cone beam computed tomography (CBCT) is an imaging modality that provides computed tomographic images using a rotational C-arm equipped with a flat panel detector as part of the Angiography suite. The aim of this technique is to provide additional information to conventional 2D imaging to improve the performance of interventional liver oncology procedures (intraarterial treatments such as chemoembolization or selective internal radiation therapy, and percutaneous tumor ablation). CBCT provides accurate tumor detection and targeting, periprocedural guidance, and post-procedural evaluation of treatment success. This technique can be performed during intraarterial or intravenous contrast agent administration with various acquisition protocols to highlight liver tumors, liver vessels, or the liver parenchyma. The purpose of this review is to present an extensive overview of published data on CBCT in interventional oncology of the liver, for both percutaneous ablation and intraarterial procedures.

Improved liver function after portal vein embolization and an elective right hepatectomy.

BACKGROUND: Portal vein embolization (PVE) is used before extensive hepatic resections to increase the volume of the future remnant liver within acceptable safety margins (conventionally >0.6% of the patient's weight). The objective was to determine whether pre-operative PVE impacts on post-operative liver function independently from the increase in liver volume. METHODS: The post-operative liver function of patients who underwent an anatomical right liver resection with (n = 28) and without (n = 53) PVE were retrospectively analysed. Donors of the right liver were also analysed (LD) (n = 17). RESULTS: Patient characteristics were similar, except for age, weight and American Society of Anesthesiologists (ASA) score that were lower in LD. Post-operative factor V and bilirubin levels were, respectively, higher and lower in patients with PVE compared with patients without PVE or LD (P < 0.05). Patients with PVE had an increased blood loss, blood transfusions and sinusoidal obstruction syndrome. The day-3 bilirubin level was 40% lower in the PVE group compared with the no-PVE group after adjustment for body weight, chemotherapy, operating time, Pringle time, blood transfusions, remnant liver volume, pre-operative bilirubin level and pre-operative prothrombin ratio (P = 0.001). CONCLUSIONS: For equivalent volumes, the immediate post-operative hepatic function appears to be better in livers prepared with PVE than in unprepared livers. Future studies should analyse whether the conventional inferior volume limit that allows a safe liver resection may be lowered when a PVE is performed.

Portal Vein Embolization Before Extended Hepatectomy in a Toddler With Mesenchymal Hamartoma.

Portal vein embolization is widely used to induce hypertrophy of the future liver remnant before extended hepatectomy, decreasing the risk of postoperative liver failure. However, this percutaneous procedure has not been previously reported in a young child. The present report describes the case of a 14-month-old patient with a large multifocal mesenchymal hamartoma of the entire right liver, successfully resected after induction of future liver remnant hypertrophy by portal vein embolization.

Hepatic lymphatics: anatomy and related diseases.

The liver normally produces a large amount of lymph. It is estimated that between 25% and 50% of the lymph received by the thoracic duct comes from the liver. In normal conditions, hepatic lymphatics are not depicted on cross-sectional imaging. They are divided in lymphatics of deep system (lymphatics following the hepatic veins and the portal tract) and those of superficial system (convex surface and inferior surface). A variety of diseases may affect hepatic lymphatics and in general they manifest as lymphedema, lymphatic mass, or cystic lesions. Abnormal distended lymphatics are especially seen in periportal spaces as linear hypoattenuations on CT or strong linear hyperintensities on heavily T2-weighted MR imaging. Lymphatic tumor spread as in lymphoma and lymphangitic carcinomatosis manifests as periportal masses and regional lymph node enlargement. Lymphatic disruption after trauma or surgery is depicted as perihepatic fluid collections of lymph (lymphocele). Lymphatic malformation such as lymphangioma is seen on imaging as cystic spaces of variable size.

Embolization of a Jejunal Artery Pseudoaneurysm via Collateral Vessels.

Visceral artery pseudoaneurysms are rare and only few cases have been reported. They are considered to be life threatening in case of rupture. Rapid treatment is mandatory and endovascular procedure is recommended as the treatment of choice. Occasionally, endovascular approach is difficult to achieve, owing to unusual vascular anatomy. Whenever it is the case, an alternative method has to be considered. We report the case of a jejunal artery pseudoaneurysm that required an access via collateral vessels to accomplish complete occlusion in a 34-year-old woman who presented with a sudden epigastric pain 14 days after a cephalic duodenopancreatectomy.

Acute hand ischemia after unintentional intraarterial injection of drugs: is catheter-directed thrombolysis useful?

Unintentional intraarterial injections are rare but may have devastating consequences. No consensus on treatment has been established owing to the wide variety of possible injected substances, incomplete understanding of the underlying pathophysiology, and the absence of case-controlled, prospective human studies. The aim of the present study and literature review was to evaluate the benefit of intraarterial thrombolysis combined with systemic anticoagulation therapy when an artery of the upper extremity is accidentally punctured and ischemia of the hand ensues.