Three‐dimensional parametric modeling of bicuspid aortopathy and comparison with computational flow predictions

Bicuspid aortic valve (BAV)‐associated ascending aneurysmal aortopathy (namely “bicuspid aortopathy”) is a heterogeneous disease making surgeon predictions particularly challenging. Computational flow analysis can be used to evaluate the BAV‐related hemodynamic disturbances, which likely lead to aneurysm enlargement and progression. However, the anatomic reconstruction process is time consuming so that predicting hemodynamic and structural evolution by computational modeling is unfeasible in routine clinical practice. The aim of the study was to design and develop a parametric program for three‐dimensional (3D) representations of aneurysmal aorta and different BAV phenotypes starting from several measures derived by computed‐tomography angiography (CTA). Assuming that wall shear stress (WSS) has an important implication on bicuspid aortopathy, computational flow analyses were then performed to estimate how different would such an important parameter be, if a parametric aortic geometry was used as compared to standard geometric reconstructions obtained by CTA scans. Morphologic parameters here documented can be used to rapidly model the aorta and any phenotypes of BAV. t‐test and Bland–Altman plot demonstrated that WSS obtained by flow analysis of parametric aortic geometries was in good agreement with that obtained from the flow analysis of CTA‐related geometries. The proposed program offers a rapid and automated tool for 3D anatomic representations of bicuspid aortopathy with promising application in routine clinical practice by reducing the amount of time for anatomic reconstructions.     

The "sclerodermic hand": a radiological and clinical study

ObjectiveTo assess the clinical and radiographic features of hand involvement in patients with systemic sclerosis (SSc).MethodsForty-one unselected Sardinian SSc patients (32 women, 9 men; mean age 58.9, range 31–81 years; mean disease duration 11.8 years, range 1–36 years) were evaluated in this observational cross-sectional study. Twenty-six patients had diffuse scleroderma (dSSc) and 15 limited scleroderma (lSSc). Radiological examination of the hands was performed and the films were read by two independent rheumatologists blinded to the diagnosis using a classification system of four predefined radiological patterns (normal/minimal changes, articular degenerative, articular inflammatory and periarticular pattern). Correlations between radiological pattern, clinical and serological features were assessed.ResultsThe skeletal and articular involvement of the hand was frequent in SSc, being clinically evident in 30/41 (73%) and radiologically in 33/41 (80%) of patients. The periarticular pattern (defined as the occurrence of bone resorption of ungueal tufts, soft tissue calcifications and/or flexion deformities) was the most frequent pattern detected (14/41, 34.1%) and finger flexion contractures and bone resorptions were significantly associated with interstitial lung disease, reduced FVC, oesophagus involvement and prostacycline therapy. Calcinosis (29.2%) was found to be associated with erosions, suggesting a pathogenic link. An inflammatory pattern was also radiologically frequent (8/41, 19.5%), but erosions, with the exception of those localized at distal interphalangeal joints, were demonstrated mainly in patients with clinical picture of rheumatoid arthritis overlapped with SSc. We found no significant differences in terms of radiographic findings between lSSc and dSSc with the exception of calcinosis, which was more frequent in patients with lSSc.ConclusionThis cross-sectional study confirms that the skeletal and articular involvement of the hand is frequent in SSc.     

Tracheal agenesis without esophageal fistula: genetic, resuscitative, and pathological issues

An exceptional case of tracheal agenesis with no communication with the esophagus is described. This malformation needs surgical airway approach and is hardly classifiable. We analyzed the literature and our institutional data: this resulted to be the first case of such anatomical variant. Genetic and pathological issues are reviewed: recent genetic data seem to explain this malformation. We also reviewed the available literature about prenatal presentation. Because prenatal diagnosis is difficult to achieve and current guidelines for neonatal resuscitation do not provide any recommendation, the resuscitative team may not be prepared for managing such a case. Usefulness of uncommon resuscitative maneuvers is discussed: a promptly performed surgical tracheotomy is the only mean to ventilate such a baby.     

Small bowel tumours in emergency surgery: specificity of clinical presentation

BACKGROUND: Despite advances in diagnostic modalities, small bowel tumours are notoriously difficult to diagnose and are often advanced at the time of definitive treatment. These malignancies can cause insidious abdominal pain and weight loss, or create surgical emergencies including haemorrhage, obstruction or perforation. The aim of the present study was to describe the clinical presentation, diagnostic work-up, surgical therapy and short-term outcome of 34 patients with primary and secondary small bowel tumours submitted for surgical procedures in an emergency setting and to look for a correlation between clinical presentation and the type of tumours. METHODS: From 1995 to 2005, 34 consecutive surgical cases of small bowel tumours were treated at the Department of Emergency Surgery of St Orsola-Malpighi University Hospital, Bologna, Italy. Clinical and radiological charts of these patients were reviewed retrospectively from the department database. RESULTS: All patients presented as surgical emergencies: intestinal obstruction was the most common clinical presentation (15 cases), followed by perforation (11 cases) and gastrointestinal bleeding (eight cases). Lymphoma was the most frequent histologic type (nine patients), followed by stromal tumours (eight patients), carcinoids (seven patients), adenocarcinoma (seven patients) and metastasis (three patients). Of the nine patients with lymphoma, eight were perforated, all patients with stromal tumours had bleeding, and all carcinoids patients had bowel obstruction. There were two patients with melanoma metastasis, both had bowel intussusception. Resection of the neoplasm was carried out in 32 patients and two patients were deemed unresectable and received a palliative procedure. CONCLUSIONS: The present study shows that there is a correlation between small bowel tumours and clinical emergency presentation: gastrointestinal stromal tumours (GIST) mostly bleed; carcinoids make an obstruction; lymphomas cause a perforation; and melanoma metastasis causes intussusception.     

Liver Resection with Portal Vein Thrombectomy for Hepatocellular Carcinoma With Vascular Invasion

Introduction  Hepatocellular carcinoma (HCC) tends to invade the intrahepatic vasculature, especially the portal vein.1 The presence of portal vein tumor thrombus (PVTT) in patients with HCC is one of the most significant factors for a poor prognosis.2 ^– 5 The presence of macroscopic PVTT in patients with HCC is also a significant factor for poor prognosis, with a median survival of <3 months without treatment.1 In surgically resected series, in patients with gross PVTT (PVTT in the portal trunk, its first-order branch, or its second-order branch), the 3-year and 5-year survival rates are reportedly 15% to 28% and 0% to 17%, respectively.2 ^– 5 Methods  The patient was a 77-year-old woman with well-compensated hepatitis C virus–related cirrhosis (stage A6 according to Child-Pugh classification) who sought care at our department for vague abdominal discomfort. Triphasic spiral computed tomographic scan confirmed HCC 6 cm in diameter in the left lobe of the liver. In addition, portal vein tumor thrombosis of the left branch that extended to the right portal vein was present. Results  The procedure included left hepatectomy and en-bloc portal vein thrombectomy with clamping of both the common portal vein trunk and the right portal vein. The portal vein was incised at the bifurcation of the right and left portal veins, and the thrombus was extracted from the incision in the portal vein. With this procedure, we were able to examine under direct vision the exact extent of the portal vein thrombus, and we identified whether the tumor thrombus was adherent to the venous wall or was freely floating in the venous lumen. Portal clamping and length of operation were 16 and 330 minutes, respectively. Intraoperative blood loss was 550 mL. The patient was discharged on postoperative day 6, and she was free of disease at 15 months after surgery. Discussion  Liver resection should be considered a valid therapeutic option for HCC with PVTT. Electronic supplementary material  The online version of this article (doi:) contains supplementary video material, which is available to authorized users. Presented to Annual Meeting of the American Hepato-Pancreato-Biliary Association (AHPBA), Miami, Florida, USA, March 9-12, 2006.     

Outcome and management of isolated moderate renal pelvis dilatation detected at postnatal screening

The aim of this study was to evaluate the incidence and outcome of isolated moderate renal pelvis dilatation (RPD) [anterior–posterior diameter (APD) 10–15 mm] in an unselected population of 2-month-old infants prospectively followed for up to 12–14 months of life. Isolated moderate renal pelvis dilatation was detected in 282 of the 11,801 (2.4%), infants screened; 240 infants with normal renal ultrasound were enrolled as the control group. Resolution of RPD was considered when an APD ≤ 5 mm was found on two consecutive sonograms. Urological investigations were initiated if the RPD persisted or if urinary tract infection (UTI) occurred during follow-up, and antibiotic therapy was administered only when UTI occurred. The events of interest were resolution of the RPD, presence of uropathy and UTI. At follow-up, RPD persisted only in 18 infants; of these, four infants were diagnosed with vesicoureteral reflux (grade 1–3) and 14 with ureteropelvic junction obstruction. Of the 223 infants with RPD and 230 control infants who completed follow-up, UTI occurred in 3.6 and 2.5%, respectively. The incidence rate of UTI per 1000 person-months was 5.98 episodes in the patient group and 5.22 episodes in the control group. The rate ratio was 1.146 (95% confidence interval 0.389–3.54, p = 0.8). Our data suggest that isolated moderate RPD is essentially a self-limiting condition and that antibiotic prophylaxis for the prevention of UTI should not be performed. A non-invasive ultrasound scan performed during the follow-up is sufficient to diagnose a potentially dangerous and persistent RPD.     

Longitudinal evaluation of mycophenolic acid pharmacokinetics in pediatric kidney transplant recipients. The role of post-transplant clinical and therapeutic variables

Abstract:  This longitudinal study assessed the influence of post-transplant clinical and therapeutic variables in 50 kidney transplant recipients aged 2–19 yr receiving a triple immunosuppressive regimen consisting of cyclosporine microemulsion (CsA), steroids and MMF (300–400 mg/m² body surface area twice daily), the full pharmacokinetic profile (10 points) of which was investigated on post-transplant days 6, 30, 180 and 360. Total plasma MPA was measured by Enzyme Multiplied Immunoassay Technique. CsA therapeutic drug monitoring (TDM) was performed via C2 blood monitoring, while MPA TDM via C0. MPA Cmax, tmax, AUC0-12 and AUC0-4 pharmacokinetic profile changed significantly during the first post-transplant year. C0 was a poor predictor of the total MPA exposure [as measured by the area under the concentration-time curve AUC)], while a truncated AUC was a good surrogate of the 12-h profile (r = 0.91; p < 0.001) Graft function and cyclosporine therapy influenced MPA pharmacokinetics, as shown by the univariate and multivariate analyses. We conclude that because after transplantation MPA exposure varied over time, a strict TDM is advisable in the pediatric population.