Paediatric ulcerative colitis surgery: Italian survey.

BACKGROUND AND AIMS: Recent epidemiological studies showed an increase in ulcerative colitis among children, especially in its aggressive form, requiring surgical treatment. Although medical therapeutic strategies are standardized, there is still no consensus regarding indications, timing and kind of surgery. This study aimed to define the surgical management of paediatric ulcerative colitis and describe attitudes to it among paediatric surgeons. METHODS: This was a retrospective cohort study. All national gastroenterology units were invited to participate. From January 2009 to December 2013, data on paediatric patients diagnosed with ulcerative colitis that required surgery were collected. RESULTS: Seven units participated in the study. Seventy-one colectomies were performed (77.3% laparoscopically). Main surgical indications were a severe ulcerative colitis attack (33.8%) and no response to medical therapies (56.3%). A three-stage strategy was chosen in 71% of cases. Straight anastomosis was performed in 14% and J-pouch anastomosis in 86% of cases. A reconstructive laparoscopic approach was used in 58% of patients. Ileo-anal anastomosis was performed by the Knight-Griffen technique in 85.4% and by the pull-through technique in 9.1% of patients. Complications after colectomy, after reconstruction and after stoma closure were reported in 12.7, 19.3 and 35% of cases, respectively. CONCLUSIONS: This study shows that there is general consensus regarding indications for surgery. The ideal surgical technique remains under debate. Laparoscopy is a procedure widely adopted for colectomy but its use in reconstructive surgery remains limited. Longer follow-up must be planned to define the quality of life of these patients.

Prevalence of cerebral aneurysms in patients treated for left cardiac myxoma: a prospective study.

AIM: To estimate the prevalence of cerebral aneurysms in patients previously treated for left cardiac myxoma (LCM). MATERIALS AND METHODS: This prospective institutional review board-approved study included patients treated for LCM. All patients treated at our institution (IRCCS Policlinico San Donato, Italy) were telephoned and those enrolled underwent unenhanced brain magnetic resonance imaging (MRI) using sagittal T1-weighted turbo spin-echo (TSE); axial T2-weighted TSE; axial fluid-attenuated inversion-recovery; axial echo-planar diffusion-weighted; and three-dimensional time-of-flight angiographic sequences. RESULTS: Seventy-six patients were telephoned, and data regarding their clinical history since tumor resection were obtained for 49 patients (64%). Four of the 49 (8%) patients were deceased, one due to a cerebral hemorrhage from a ruptured cerebral aneurysm 8 years after tumor resection. One patient had a pacemaker preventing MRI. Of the remaining 44 patients, 31 refused MRI and 13 were enrolled (10 females; mean age 64 years). Three of the 13 (23%; two females; 59-78 years) were diagnosed with a cerebral aneurysm, from 2 mm to 4-5 mm in diameter, involving the right middle cerebral artery (n = 2) or the right internal carotid artery (n = 1). Including the deceased patient, the resulting prevalence was 4/14 (29%). CONCLUSION: From this preliminary study, one-third of patients treated for LCM may present with a cerebral aneurysm. Longitudinal large studies are needed to further clarify this matter.

Bone mineral density differences between femurs of scoliotic patients undergoing dual-energy X-ray absorptiometry.

AIM: To investigate whether a difference exists in the bone mineral density (BMD) between femurs in scoliotic patients undergoing dual-energy X-ray absorptiometry (DXA) and whether this difference is related to spine convexity. MATERIALS AND METHODS: Of 1080 consecutive patients who underwent DXA, 127 had lumbar scoliosis seen at DXA. Further, after excluding 30 patients with previous osteoporotic fractures, metallic/image artefacts, soft-tissue calcifications, the BMD differences between femurs of 97 scoliotic patients (94 females; mean age 67 ± 11 years) were analysed. Femurs were classified as ipsilateral or contralateral to the spine convexity. Least significant change was used as a threshold of measurement precision. Differences between femoral neck BMD in respect of and regardless of spine convexity were calculated for each patient. Student's t-test and Wilcoxon's signed-rank test were used to assess significance. RESULTS: Fifty-nine of 97 patients (61%) had left-sided scoliosis. Osteoporosis was found in 32/97 patients (33%), osteopenia in 54/97 (56%), and 11/97 (11%) had a normal T-score. Of 97 patients, 46 (47%) had ipsilateral BMD < contralateral BMD. Regardless of spine convexity, 66/97 patients (68%) had different BMD values between femurs. Among them, variation of densitometric diagnosis was seen in 29/66 patients (44%), and in 29/97 patients overall (30%). CONCLUSION: Differences in the femoral neck BMD exist between femurs of scoliotic patients undergoing DXA. Thus, bilateral femoral DXA acquisition is recommended.

Contrast-enhanced MR imaging of the breast: association between asymmetric increased breast vascularity and ipsilateral cancer in a consecutive series of 197 patients.

PURPOSE: This study was done to estimate the diagnostic performance of an asymmetric increase in breast vascularity (AIBV) for ipsilateral cancer. MATERIALS AND METHODS: A total of 197 patients without previous breast interventions underwent bilateral contrast-enhanced (gadoterate meglumine, 0.1 mmol/kg) magnetic resonance (MR) imaging. Vessels >-2 mm in diameter and ≥ 3 cm in length were counted on maximum intensity projections: a difference ≥ 2 in number between the two breasts was considered AIBV. Pathology or ≥ 1 year follow-up served as a reference standard. The difference in sensitivity of AIBV between invasive and ductal carcinoma in situ (DCIS) as well as the association between AIBV and the diameter of invasive lesions or the histological grade were evaluated using χ(2) test. RESULTS: Pathology revealed 82 malignancies and 20 benign lesions: 70 invasive carcinomas (57 ductal, nine lobular, three mucinous, one papillary) and 12 DCIS: 10 fibroadenomas, two papillomas, two atypical ductal hyperplasias and six other benign lesions. The remaining 95 patients were negative at follow-up. Sensitivity of AIBV was 74% (61/82), specificity 94% (108/115), accuracy 86% (169/197), positive predictive value 90% (61/68) and negative predictive value 84% (108/129). Sensitivity for invasive cancers (80%; 56/70) was significantly higher than that for DCIS (42%; 5/12) (p<0.001). For invasive cancers, sensitivity was 40% (2/5) for lesions ≤ 9 mm in diameter, 69% (9/13) for those 10-14 mm, 79% (15/19) for those 15-19 mm and 91% (30/33) for those ≥ 20 mm (p<0.001). The G3 lesion rate was 49% (27/55) among true positives and only 7% (1/14) among false negatives (p=0.009). CONCLUSIONS: An association between AIBV and ipsilateral cancer exists, particularly for invasive cancers ≥ 20 mm or with high pathologic grade.

Treatment with pamidronate for osteoporosis complicating long-term intestinal failure.

Long-term home parenteral nutrition (PN) is a potential risk for developing osteoporosis. Various attempts have been made to treat bone disease both by modifying the composition of PN and by administering hormones, such as calcitonin, parathyroid hormone, and sexual hormones. Bisphosphonates are recognized as a medication useful for the treatment of several bone disorders associated with excessive reabsorption. Nevertheless, there have been no paediatric studies on bisphosphonates use for intestinal failure-associated bone disease. Our study includes 6 paediatric patients receiving extremely long-term home PN (at least 3 years) who showed radiological and clinical signs of osteoporosis. Diagnosis of bone disease was made after a median period of 127.5 PN months. Treatment consisted in 2 cycles of intravenous pamidronate, 30 mg/m once per month for 6 months consecutively. They all showed a significant improvement in bone mineral density, evaluated after 6 and 12 months of pamidronate treatment. In our sample anthropometrical variables (weight, height, and body mass index) are not related with the z-score trend. Our patients had normal levels of calcium, phosphorus, and vitamin D, and proper nutrient intake. At the last follow-up, dual-energy x-ray absorptiometry scan showed that no patients had a z-score lower than -2.5; moreover, nobody developed bone fractures during the 108-month follow-up. The patients did not have any prominent adverse effect. Finally, in our experience, pamidronate is effective for improving bone mineral density and safe in patients with intestinal failure-associated bone disease.

Magnetic resonance imaging of painful total hip replacement: detection and characterisation of periprosthetic fluid collection and interobserver reproducibility.

PURPOSE: The purpose of our study was to demonstrate the diagnostic value of magnetic resonance imaging (MRI) when measuring and characterising periprosthetic fluid collections in patients with painful hip prosthesis and to provide an estimation of interobserver reproducibility. MATERIALS AND METHODS: Nineteen patients (mean age 59±13 years) with painful total hip replacement and clinical suspicion of infection underwent MRI. Images were reviewed blindly by two musculoskeletal radiologists with different levels of experience who evaluated the presence/absence of soft tissue oedema or fluid collection (when present, three-plane maximal diameters were measured; involvement of skin/subcutaneous/deep tissues or prosthesis were estimated; fluid was classified as serous/purulent/haematic according to signal behaviour). Interobserver agreement was calculated (Cohen's ). RESULTS: A total of 26 MRI studies were carried out (three patients underwent two and two patients underwent three MRI examinations). Both readers detected soft tissue oedema (13/26, 50%) or fluid collection (21/26, 81%) and characterised the fluid as serous (9/21, 43%), purulent (8/21, 38%) or haematic (4/21, 19%). The collection involved skin/subcutaneous tissues (16/21, 76%), deep soft tissues (19/21, 91%) or the implant (12/21, 57%). For all evaluations, interobserver agreement was complete (=1). No significant differences were found between the measurements of the collections (p>0.258). CONCLUSIONS: MRI is highly reproducible in detection, localisation, quantification, and characterisation of fluid collections when the presence of implant infection is clinically suspected.

Renal volume assessment with 3D ultrasound.

PURPOSE: This study was undertaken to determine the accuracy of 3D ultrasound (US) in assessing renal volume, with multislice computed tomography (MSCT) considered as the gold standard. MATERIALS AND METHODS: Forty-nine patients (30 men, 19 women; age range 30-82 years) underwent abdominal contrast-enhanced MSCT and 3D-US performed with a 3.5-MHz 3D/4D convex-array probe. The results of the two modalities were compared with the Wilcoxon test. Variability between the two measurements was determined with the Bland-Altman method and reported in terms of bias and coefficient of repeatability (CoR). RESULTS: Mean values obtained were 210 ml with MSCT and 192 ml with 3D-US (p<0.001). Analysis of variability per patient between MSCT and 3D-US showed a bias of 19 ml, a CoR of 47 ml and an accuracy of 78%, with an average 3D-US underestimation of 19 ml (9%). Analysis of variability per kidney showed a bias of 9 ml, a CoR of 34 ml and an accuracy of 80%. CONCLUSIONS: Three-dimensional US is a valuable technique for monitoring renal volume, whereas MSCT may be reserved for assessing renal anatomy and relationships with neighbouring organs.

Cardiac magnetic resonance: impact on diagnosis and management of patients with congenital cardiovascular disease.

AIM: To estimate the clinical impact of cardiac magnetic resonance (CMR) in patients with congenital cardiovascular disease (CCD). MATERIALS AND METHODS: Since 2003, 1.5T CMR was used at our university hospital to evaluate morphology, cardiac kinetics, aortic and pulmonary flow, and vascular anatomy in patients with CCD. The present study considered a consecutive series of these patients from 2003 to 2006. A paediatric cardiologist judged our reports as expected or unexpected and, secondarily, as not reliable (level 0), describing findings already known (level 1), not changing therapy/suggested lifestyle (level 2), changing therapy/suggested lifestyle (level 3) or changing diagnosis (level 4). RESULTS: CMR reports were judged to be expected in 187/214 (87%) and unexpected in 27/214 (13%). Less than 2% of CMRs were judged as levels 0 or 1, 66% as level 2, and 5% as level 4. During 2005-2006 the clinical impact improved toward higher impact levels (p<0.001, chi-square test). CONCLUSIONS: In patients with CCD, more than one in 10 CMR reports were unexpected to cardiologists and over seven in 10 prompted a change of diagnosis or therapy.

Myocardial delayed enhancement using a single dose (0.1 mmol/kg) of gadobenate dimeglumine: contrast resolution versus intraventricular blood and viable myocardium.

PURPOSE: This study was done to estimate delayed enhancement (DE) contrast resolution of infarcted myocardium (IM) relative to intraventricular blood (IB) and viable myocardium (VM) using gadobenate dimeglumine (Gd-BOPTA). MATERIALS AND METHODS: After approval from the Ethics Committee, we retrospectively evaluated 21 consecutive patients (61+/-10 years) with a healed myocardial infarction who underwent 1.5-T magnetic resonance (MR) imaging using an inversion-recovery-prepared turbo gradient-echo sequence 10 minutes after injection of 0.1 mmol/kg of Gd-BOPTA. Signal intensity (SI) was measured in arbitrary units (au) for IM, IB, VM, and outside the patient. Contrast-to-noise ratio (CNR) was calculated for IM to IB and IM to VM. Seven consecutive patients (59+/-6 years) with a healed myocardial infarction studied with similar technique but with 0.1 mmol/kg of gadoterate meglumine (Gd-DOTA) served as the control group. The Mann-Whitney U test was used to compare groups. RESULTS: Mean SI of IM was 44+/-16 au for Gd-BOPTA and 20+/-6 au for Gd-DOTA (p<0.001), that of IB 35+/-15 au and 14+/-5 au (p=0.016), and that of VM 7+/-3 au and 5+/-2 au (p=0.116), respectively. Mean IM to IB CNR was 10+/-7 for Gd-BOPTA and 8+/-5 for Gd-DOTA (p=0.836), that of IM to VM was 45+/-27 and 18+/-6, respectively (p=0.012). CONCLUSIONS: Gd-BOPTA at 0.1 mmol/kg produced a higher myocardial DE and an IM to VM CNR than a single dose of Gd-DOTA. No significant difference was observed for IM to IB CNR.

MR imaging of aortic coarctation.

Aortic coarctation accounts for 5%-10% of all congenital heart diseases and represents 7% of critically ill infants with heart disease. Magnetic resonance (MR) imaging allows the study of this disease with several advantages in comparison with conventional angiography, transesophageal echocardiography, and computed tomography. The MR protocol applied at our institution for both diagnosis and follow-up after surgical or endovascular treatment consists of four steps: morphologic study, cine MR study, flow analysis, and MR angiography (MRA). The first three sequences are acquired during breath-hold and with electrocardiographic gating. Anatomy is well depicted with dark-blood half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences. Cine true-fast imaging with steady-state precession (true-FISP) sequences show not only morphologic features but also blood-flow changes inside the aorta. Gradient-echo sequences for phase-velocity mapping allow flow analysis. Application of Bernoulli's equation--here briefly presented and discussed--allows for calculation of the pressure gradient caused by the coarctation. MRA, acquired with a breath-hold three-dimensional T1-weighted gradient-echo sequence and intravenous administration of paramagnetic contrast material, allows for optimal depiction of the aortic lumen, with a panoramic view of the whole aorta, its main branches and possible collateral circulation.

Deep sedation with propofol for upper gastrointestinal endoscopy in children, administered by specially trained pediatricians: a prospective case series with emphasis on side effects.

BACKGROUND AND STUDY AIMS: The need to administer procedural sedation to children has increased in recent years, as has experience in this field among nonanesthesiologists. Using propofol makes it easier to achieve sufficiently deep sedation. There is a considerable literature on the administration of propofol by nonanesthesiologists for gastroscopy in adults, but very few data are available on this issue in children. The aim of the present study was to assess the safety and efficacy of procedural sedation with propofol for gastroscopy in a pediatric ward with trained personnel and monitoring facilities. PATIENTS AND METHODS: A training protocol was developed to educate nurses and residents. Children requiring gastroscopy were included in the study prospectively and underwent procedural sedation with propofol administered by nonanesthesiologists. RESULTS: A total of 811 upper gastrointestinal endoscopies were carried out with procedural sedation. Sedation was achieved in all procedures, and all but three (0.4%) were conducted successfully. None of the patients required intubation. Stridor with signs of upper airway obstruction occurred in 14 of the 811 procedures (1.7%). Laryngoscopy was required to manage difficulties in introducing the gastroscope in 16 of the 811 procedures (2.0%). Major desaturation requiring a short course of ventilation occurred in six procedures (0.7%), and transient desaturation that resolved spontaneously occurred in 97 of the procedures (12%). CONCLUSIONS: Administration of propofol by nonanesthesiologists for gastroscopy examinations in children was successful in this study, but was associated with a small risk of potentially severe complications. Although the residents were generally able to administer procedural sedation alone, constant and immediate availability of anesthesiological support continues to be mandatory.

Treatment of juvenile idiopathic arthritis with intra-articular triamcinolone hexacetonide: evaluation of clinical effectiveness correlated with circulating ANA and T gamma/delta + and B CD5+ lymphocyte populations of synovial fluid.

OBJECTIVE: The aims of the study were to assess the effect of intra-articular treatment with triamcinolone hexacetonide (TH) in juvenile idiopathic arthritis (JIA) and to investigate whether treatment response correlates with the presence of antinuclear antibodies (ANA) in the serum and/or B CD5+ and T gamma/delta + lymphocytes in the synovial fluid. METHODS: A total of 37 patients (81% females, 56% ANA+) with oligoarticular JIA involving knees were treated with intra-articular injections of TH after failing to respond to NSAIDs for two months. Eighteen patients were treated within 6 months of onset, 19 were treated more than 6 months after onset. RESULT: Mean duration of remission was 13.9 months. Twelve patients (7 ANA+) had stable remission after a single injection; 13 patients (3 ANA+) experienced more than 6 months' remission but subsequently had a relapse; 12 patients (11 ANA+) had a relapse within six months of injection. Of 20 patients treated within 6 months of onset, 17 had stable remission whereas only 8 out of 17 who were treated during relapse attained stable remission (p = 0.03). The mean percentage of T gamma/delta + and of B CD5+ lymphocytes in synovial fluid was the same as in peripheral blood of normal subjects. CONCLUSION: Our data indicate that local treatment with slow-release steroids is very effective in oligoarticular JIA. Prolonged remission was less likely in the presence of ANA positivity, probably because the disease is immunologically more active. Finally, our data suggest that the earlier the treatment, the easier it is to obtain a protracted, and possibly permanent, response.

[Urethral ultrasonography: a new diagnostic approach in the evaluation of stenosis of the male urethra]. / Ultrasonografia uretrale: un nuovo approccio diagnostico nella valutazione della stenosi dell'uretra maschile.

Endoscopy and radiological investigations, using contrast medium, have been the only available methods in the study of urethral pathology. In this article we have suggested using the technique of urethral ultrasound to visual the urethra before using ultrasound. Our study included 16 patients ranging in age from 23 to 82 years with urethral stenosis already diagnosed or suspected on the basis of symptomatology at referral. When the urethra is distended by the gel it appears to be a black tube with a smooth surface which does not produce echoes. The gel determines the compression of the corpus spongiosa which is normally a rigid, inelastic tissue because of the prevalence of collagen fibres. The possibility of adding further gel inside the urethra to confirm the echo results even after the introduction of a standard quantity at the start of the procedure we feel is the second definitive advantage with this method. In fact it was possible in our survey by increasing the quantity of gel to currently differentiate between complete and partial stenosis. No patient suffered from a temperature or using retention after this test. No-one complained of discomfort during the procedure. The anterior and posterior urethra are separated by the external sphincter. Radiological images are better obtained from the anterior urethra than the posterior urethra. We have found that ultrasound is a reliable and possibly superior method. With this method it is possible to obtain more information about the peri-urethral scar tissue, the length of the stenosis, the degree of spongy fibrosis therefore allowing more precise urethrotomies to be performed. The test is comfortable for the patient, simple and reduces the risk of radiation. The disadvantages of this method are that the posterior urethra is poorly visualized. Examination of the posterior urethra using a trans-scrotal approach have given poor results. It is necessary to avoid excessive pressure on the probe which could give false positive results. To eliminate this problem it is necessary to scan longitudinally and transversally within the lumen at various stages of filling. It is important to note that air present within the gel could simulate urethral calculi, if the image has been frozen. During the test the presence of air helps to evaluate the degree of flow through the stenosis.

[Chronic idiopathic hypertransaminasemia]. / Ipertransaminasemia cronica idiopatica.

The elevation of aminotransferase serum levels is frequently encountered in pediatric practice. We have retrospectively evaluated the clinical patterns of 108 patients with chronic, so called "idiopathic", alterations of aminotransferases, by sending a questionnaire to 11 Italian Pediatric Centers. The average period of follow-up was 22 months. Patients, whose ages ranged through all pediatric ages, were mostly asymptomatic and with a rather insignificant physical examination. The average rise of aminotransferases value was generally limited within 2 times the upper normal level and the highest value, during the period of follow-up, never exceeded 5 times the upper normal level. Other liver function tests did not result generally altered significant. Just 25,9% of the patients normalized aminotransferases serum level during the follow-up period. All maintained good physical status with no clinical signs of liver disease. A muscular cause of hyper-transaminasemia was excluded in all the cases. Possible infective causes (HBV and HCV) autoimmune hepatitis, Wilson disease, alfa1 antitripsine deficiency and hyperammoniemia were excluded. Ultrasound investigation did not seem to be a sensitive investigation, resulting negative in 54/82. Histologic liver examination was more informative. This evaluation, performed in 46/108 patients, showed infarct metabolic alterations (steatosis, nucleus glucogenic degeneration, cytoplasmatic clarification) in 65% of cases and inflammatory findings in only 13% of cases. In conclusion, our results suggest the opportunity to enclose liver histologic study in the diagnostic approach of children with hepatic idiopathic chronic hypertransaminasemia. This approach may address the clinician, in a more aimed way, towards further investigations.

[Vascular hyperfragility in systemic lupus erythematosus treated with low doses of cortisone]. / Iperfragilità vascolare in corso di lupus eritematoso sistemico trattato con basse dosi di cortisone.

We report an unusual cutaneous manifestation of systemic lupus erythematosus (SLE) in a 15-year old female. The diagnosis was made on the basis of clinical symptoms, cutaneous hystology (positive "lupus band test") and on laboratory findings (hypocomplementemia, positive antinuclear antibodies and rheumatoid factor). Treatment with methylprednisolone (0.5 mg/kg/die) improved the clinical symptoms but, after 2 months, large ecchymotic lesions appeared on the lower legs below the knee extending as far as the ankles, likely triggered by minor local traumas. Coagulative function was normal, the lupic anti-coagulant factor (LAF) was negative, anticardiolipin antibodies were absent and there was no thrombocytopenia. There was only a slight increase in clotting times in vitro, in presence of ADP. The amount of cortisone was reduced and the type of treatment modified; satisfactory control of the disease was attained with deflazacort (0.3 mg/kg/die). The ecchymosis on the lower limbs never disappeared even though they became slightly smaller. Ecchymotic lesions are not usually included in the wide range of cutaneous manifestations associated with SLE. Moreover vascular fragility resulting from pressure and minor traumas is known to be a cutaneous complication of hypercorticism; nevertheless the doses of cortisone administered to this patient were rather low and other clinical signs of steroid hyper-dosing were absent although cortisolemia assay at base and after stimulus with ACTH was not performed. We would suggest that the negligible platelet binding defect (whether primary or SLE-associated) together with the low amounts of cortisone administered caused ecchymotic lesions to appear in this patient suffering from a disease (SLE), in which the small cutaneous vessels are favourite targets.

[Systemic lupus erythematosus: description of a pediatric series]. / Lupus eritematoso sistemico: descrizione di una casistica pediatrica.

We describe 11 cases of Systemic Lupus Erythematosus (SLE) with pediatric onset (10 females and 1 male). Mean age at onset was 10.9 years (range 3 to 16 years). Initial manifestations: cutaneous involvement in 7 cases, articular symptoms in 7 cases, renal involvement in 5 cases (proteinuria and/or microhematuria, or renal failure), pancytopenia in 3 cases. In 3 cases the onset of the disease was extremely sudden and severe: one patient had an intestinal infarct following mesenteric thrombosis associated with glomerulonephritis; another started with encephalopathy (deep coma, stage III); a third patient presented renal failure due to acute glomerulonephritis. At diagnosis all patients received systemic steroid therapy with the exception of one who had only a cutaneous involvement. The course of the disease is described. We underline that, in our series, it was rare for organs and systems, apart from the central nervous system, to be involved in exacerbations after initial onset of the disease. Six patients are presently asymptomatic or have only minor cutaneous and/or articular manifestations which are well controlled with low-dose cortisone therapy. Laboratory indices did not return to normal in any of the patients. In fact, in our series the disease doesn't appear to reach a complete remission, even many years after onset and no patient seems to be able to withdrawal the therapy at all. Our data confirm, according to other Authors, that the course of LES with paediatric onset is more severe than in adults.