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Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study.

UNLABELLED: The clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient-control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome. DESIGN: Prospective clinical trial, multicentric, cross-sectional. SETTING: MRI of the lumbosacral spine. PATIENTS: Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls. OUTCOME MEASURES: Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine. RESULTS: DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6-8 years, P < 0.05). VBD related to body height showed a similar growth related increase in patients with proven or suspected Marfan syndrome and controls. DSD related to body height was elevated in patients with proven or suspected Marfan syndrome at different levels of the lumbar spine. DSD at levels L1, L5, and S1, and DSR at levels L5 and S1 of patients with proven Marfan syndrome were significantly higher (P < 0.05) than in controls. CONCLUSION: Even during childhood pathologic changes inside the lumbosacral spine of patients with Marfan syndrome can be observed. Dural ectasia, which occurs at different levels of the lumbar spine, can be detected at levels L5 and S1 in up to 40% of patients with Marfan syndrome.

Adolescent↗

Early detection of cardiac disease masquerading as acute bronchospasm: The role of bedside limited echocardiography by the emergency physician.

We report two cases in which the patients experienced dyspnea, cough, and acute bronchospasm. Pulmonary pathology was initially suspected. Failure to respond to an initial trial of inhaled bronchodilator prompted the use of bedside limited echocardiography by the emergency physician. The potential role of limited echocardiography by the emergency physician as a triage tool in facilitating early diagnosis and emergent therapy, reducing time to final discharge, and enhancing interaction between the pediatric emergency physician and cardiology consultants is highlighted.

Albuterol↗

[A clinical experience of upper urinary tract disease using an endourological technic].

Fifty-three patients with upper urinary tract disease were treated by using endourological technique between July, 1986 and July, 1987; percutaneous nephrolithotripsy (PNL) in 26 cases, transurethral ureterolithotripsy (TUL) in 11 cases, endopyelotomy for ureteropelvic obstruction in 3 cases, dilation of ureteral stenosis in 3 cases and percutaneous nephrostomy in 10 cases. The stones could be successfully removed in 24 cases (92%) of PNL and 10 cases (91%) of TUL. No severe complications were seen in this series. The many reports of PNL and TUL were investigated in detail, but other endourological approach will need further long-term followup.

Constriction, Pathologic↗

[Ultrasound diagnosis of gallbladder and bile duct diseases].

The paper is concerned with analysis of the potentialities of ultrasound introscopy in the diagnosis of various diseases of the gall bladder and biliferous ducts in 174 patients aged 19 to 76. The accuracy of the detection of calcium containing concrements of the gall bladder was 98.8%, that of choledocholithiasis--75%, noncalculous cholecystitis--88.4%, choledochodilatation--71.4%. In the absence of pathological changes in the gall bladder and biliferous ducts the reliability of echography was 27.7 and 93.1%, respectively. Causes of diagnostic errors in ultrasound tomography of the biliary system were discussed, methods of their prevention were proposed. A diagnostic algorithm of the sequence of use of ultrasound introscopy and other diagnostic methods to be employed in clinical practice was devised.

Adult↗

Corpus callosum size and very preterm birth: relationship to neuropsychological outcome.

Thinning of the corpus callosum (CC) is often observed in individuals who were born very preterm. Damage to the CC during neurodevelopment may be associated with poor neuropsychological performance. This study aimed to explore any evidence of CC pathology in adolescents aged 14-15 years who were born very preterm, and to investigate the relationship between CC areas and verbal skills. Seventy-two individuals born before 33 weeks of gestation and 51 age- and sex-matched full-term controls received structural MRI and neuropsychological assessment. Total CC area in very preterm adolescents was 7.5% smaller than in controls, after adjusting for total white matter volume (P = 0.015). The absolute size of callosal subregions differed between preterm and full-term adolescents: preterm individuals had a 14.7% decrease in posterior (P < 0.0001) and an 11.6% decrease in mid-posterior CC quarters (P = 0.029). Preterm individuals who had experienced periventricular haemorrhage and ventricular dilatation in the neonatal period showed the greatest decrease in CC area. In very preterm boys only, verbal IQ and verbal fluency scores were positively associated with total mid-sagittal CC size and mid-posterior surface area. These results suggest that very preterm birth adversely affects the development of the CC, particularly its posterior quarter, and this impairs verbal skills in boys.

Adolescent↗

[Anatomo-pathological changes in the biliary system caused by povidone iodide. Experimental study].

In this report, the possible effects of povidone iodide (PVP-I) on the biliary system are studied in an experimental study using dogs. We used 20 dogs divided in two study groups. Light microscopy and electronic scanning microscopy (SEM) were used. We demonstrated that in the tied choledochus, intermittent washings with PVP-I caused a notable injury pattern on the biliary system.

Animals↗

Megacystis-microcolon-intestinal hypoperistalsis syndrome.

The megacystis-microcolon-intestinal hypoperistalsis syndrome is a congenital disorder characterized by urinary bladder distension and hypoperistalsis throughout the entire gastrointestinal tract. We present a new case with the typical clinical, radiological, and pathological findings of the syndrome. The diagnosis should be suspected in a patient who present clinically with intestinal obstruction and urinary retention, and confirmed with imaging studies, including abdominal plain films, urinary tract ultrasonography, and contrast studies of the colon and the bladder. The prognosis is generally very poor. Our patient died secondary to sepsis on day 5 of life.

Abnormalities, Multiple↗

Ectasia after laser in situ keratomileusis.

Eighty-five cases of post laser in situ keratomileusis ectasia were reviewed and analyzed. Cases of keratoconus or forme fruste keratoconus were eliminated; many remaining case reports lacked key information. The current literature is unable to define a specific residual corneal thickness or a range of preoperative corneal thickness that would put an eye at risk for developing ectasia. The most logical cause for eyes without preexisting pathology to develop ectasia is a postablation stromal thickness that is mechanically unstable; this "minimal" thickness is probably specific to each eye. The preoperative and postoperative corneal thickness, measured flap thickness, and microkeratome and laser parameters used in a given case are required to determine the range of residual corneal thickness that puts the eye at risk for developing ectasia. Other as yet undetermined factors may play a role in the development of this complication.

Cornea↗

Experimental models of tracheobronchial stenoses: a useful tool for evaluating airway stents.

BACKGROUND: Stent implantation is a conservative alternative to open operation for treating benign tracheobronchial strictures. Most of the presently available stents were primarily designed for endovascular use. Their respiratory use entails a risk of iatrogenic complications. From a scientific and from an ethical point of view these risks justify preclinical evaluation of new respiratory stents in experimental models of central airway stenoses. Therefore, an attempt was made to develop such models in piglets and adult minipigs. METHODS: Tracheal stenoses were obtained by creating first a segmental tracheomalacia through extramucosal resection of cartilaginous arches. The fibrous component of the stenoses was then obtained through bronchoscopic application of a caustic agent causing progressive deep mucosal and submucosal injury. Stenoses of the main bronchi were created by topical application of the caustic agent only. RESULTS: These models demonstrated the typical features of benign fibromalacic tracheobronchial stenoses with constant recurrence after mechanical dilation. Preliminary experiments showed that short-term problems of tolerance of stent prototypes are easily demonstrable in these models. CONCLUSIONS: These experimental models, which simulate quite realistically human diseases, offer the opportunity to perfect new tracheobronchial stents specifically designed for respiratory use and to evaluate their long-term tolerance before their use in humans.

Animals↗

Reoperation for complications of renal artery reconstructive surgery undertaken for treatment of renovascular hypertension.

Seventy-two secondary operations for complications of prior renal artery reconstructive surgery were undertaken in 58 patients. This experience evolved from the management of 373 patients who underwent 425 primary operations for renovascular hypertension. Secondary operations were performed 10 times in pediatric patients following 42 primary procedures (24%); 44 times in adult fibrodysplastic patients following 199 initial operations (22%); and 18 times in atherosclerotic patients after 184 primary operations (10%). The overall reoperation rate was 15.5% (58 of 373 patients). Reoperation typically followed persistent or recurrent hypertension caused by graft thromboses, perianastomotic graft narrowing, or progressive nonanastomotic graft stenoses. Aneurysmal deterioration of vein grafts was an uncommon reason for reoperation. Secondary reconstructions included nephrectomy (31), bypass with vein grafts (15) or prosthetic grafts (8), angioplasty or reimplantation (12), thrombectomy (4), and operative dilation (2). Benefits regarding hypertension control were afforded 91% of these patients. One death occurred among the 72 reoperations, representing a 1.4% operative mortality rate. Reoperative renal artery reconstructive surgery for complications of renal revascularization may present formidable technical problems. Early diagnosis and prompt reoperation with exacting vascular surgical techniques are most likely to provide optimal results.

Adolescent↗

The role of major duct excision and microdochectomy in the detection of breast carcinoma.

BACKGROUND: The association of nipple discharge with breast carcinoma has resulted in numerous women undergoing exploratory surgery to exclude malignancy. The aim of this study was to determine whether pre-operative factors can identify those patients that are most at risk of carcinoma. METHODS: All patients over a 14-year period (1991-2005) who had a microdochectomy or subareolar exploration for the evaluation of nipple discharge were assessed. Patient characteristics, pre-operative imaging and pathological findings were analysed. RESULTS: Of the 211 patients included in this study, 116 patients had pathological (unilateral, uniductal serous or bloody) discharge. On excision, 6% (n = 7) of patients with pathological discharge and 2.4% (n = 2) of patients with non-pathological discharge were diagnosed with carcinoma. Overall, major duct excision resulted in the diagnosis of carcinoma in 4.3% (n = 9), ADH/LCIS in 4% (n = 8), papilloma in 39% (n = 83), and duct ectasia or non-specific benign disease in 53% (n = 111) of patients. In the patients determined to have malignancy, 44% (n = 4) were premenopausal. No patient with a non-bloody discharge in the total population analysed (28%; n = 59/211), or in the population with a pathological discharge (21%; n = 24/116) was found to have carcinoma upon excision. CONCLUSION: Microdochectomy or major duct excision performed for nipple discharge resulted in a low rate of malignancy on excision. Conservative management of non-bloody nipple discharge can be considered in patients with no other clinical or radiological signs of malignancy.

Adult↗

[Acute colectasia in patients on artificial respiration. 10 cases].

Acute colectasia may occur in patients under mechanical ventilation. Causative factors include haemodynamic changes, potassium loss, underlying pathology (chronic respiratory failure, cirrhosis) and especially morphine-like compounds used for sedation. Analysis of the results obtained with various treatments suggests that surgery is not justified: caecal perforation is extremely rare in a previous healthy colon; any surgical procedure is hazardous in this type of patient, and colectasia frequently regresses under appropriate medical treatment, even though mechanical ventilation is pursued.

Acute Disease↗

Granulomatous lobular mastitis.

The clinical and pathological features of nine cases of granulomatous mastitis were compared with those of 10 cases of duct ectasia/periductal mastitis (DE/PM), all of which were associated with active granulomatous inflammation. Granulomatous mastitis affects a younger age group, and although there is some overlap with DE/PM, it has distinctive pathological features, particularly a lobule centred distribution, for which the term "granulomatous lobular mastitis" is recommended. There is a strong tendency for persistence or recurrence. Our cases of granulomatous mastitis all occurred in parous women, five of them within three years of pregnancy. Awareness of this condition is important, because surgery does not offer the best treatment of recurrent disease, and trials of adequate drug treatment, including corticosteroids, are required.

Adolescent↗

[What is the contribution of echography in Crohn disease?].

The alimentary tract often contains some gas which may obstruct the passage of the ultrasonic beam. However, when a pathological process involves the intestinal wall, a "target" pattern or a "pseudo-kidney" pattern may be visualized by ultrasound. It is also possible to visualize by US examination some complications of Crohn's disease: intestinal obstructive lesion with distended loops, fistula, abscesses and renal or biliary lithiasis. Because sonography is non-invasive and simple to perform without radiation exposure, it should often be used in Crohn's disease as the initial diagnosis and as the following-up test.

Abscess↗

Dilation of difficult gastrointestinal strictures using a modified wire-guided technique.

We describe here 18 patients with gastrointestinal strictures through which a standard guide wire could not be passed, and which were dilated using a modified technique. After a 0.035" guide wire had been passed through a 5-Fr metal-tipped catheter, the narrow strictures were dilated using a 10-Fr biliary dilator. A modified Savary-Gilliard guide wire (olive-tipped with a hole) was passed over the 0.035" guide wire beyond the stricture. After removing the 0.035" guide wire, the strictures were dilated with Savary-Gilliard dilators passed over the olive-tipped guide wire. Seventeen patients had upper gastrointestinal strictures, and one had a sigmoid colon stricture. The strictures were caused by tumor in two, radiation therapy in six, esophagogastric resection for cancer in five, and a combination of two or more factors in five patients. The modified technique was successful in 17 patients, without any complications. Adequate symptomatic relief was achieved in 15 patients.

Adult↗

Fibroadenoma of the breast: analysis of associated pathological entities--a different risk marker in different age groups for concurrent breast cancer.

BACKGROUND: Fibroadenoma, one of the most common benign breast lesions, has a characteristic age-specific incidence and is associated with other pathological entities in 50% of cases. The clinical or imaging diagnosis of fibroadenoma may be erroneous, and in some cases is found to be invasive cancer. The clustering of such entities, their correlation with age, and the risk of synchronous breast malignancy are uncertain. OBJECTIVE: To explore the possibility of any significant clustering of fibroadenoma-associated benign breast diseases and to assess the possible risk of concomitant breast cancer. METHODS: We analyzed the pathological results of 147 women undergoing excisional biopsies for fibroadenoma diagnosed pre-operatively either by clinical examination and imaging (n = 17) or by radiology alone (n = 30). The inter-relationships among all entities associated with fibroadenoma were studied by hierarchical cluster analysis. The correlation of the various pathologies with the risk of invasive breast cancer in relation to the patient's age was also evaluated. RESULTS: Fibroadenoma-associated pathologies were found in 48% of the cases: sclerosing adenosis (23%), duct ectasia (17.7%), apocrine metaplasia (15.6%), florid fibrocystic disease (12.9%), duct papillomatosis (11.6%), infiltrating duct carcinoma (5.4%), duct carcinoma in situ (3.4%), and 1 case of lobular carcinoma in situ (0.6%). An orderly internal hierarchy and three significant clusters emerged: a) epithelial apocrine metaplasia, duct ectasia and sclerosing adenosis (similarity coefficients 16.0, 11.0 and 8.0 respectively); b) papillomatosis, florid fibrocystic disease and calcifications (similarity coefficients of 6.0, 4.0 and 2.0 respectively); and c) infiltrating duct carcinoma and duct carcinoma in situ (similarity coefficients of 1.8 and 1.6 respectively). Seven of the eight patients with breast cancer were older than 40 years. CONCLUSIONS: In about half of the cases fibroadenoma was associated with other pathological entities clustered in an orderly hierarchy. The rarity of synchronous breast cancer in the younger age group and its more common association with fibroadenoma in the older age groups dictate a different approach to each. The finding of fibroadenoma in women older than 40 indicates the need for surgical excision.

Adolescent↗

[Diagnostic evaluation of magnetic resonance urography in urinary tract dilatation and obstruction].

OBJECTIVE: To evaluate the diagnostic value of magnetic resonance urography (MRU). METHODS: Two hundred and one patients were examined, 92 patients with urinary tract dilatation using a modified, heavily T2-weighted fast spin-echo pulse sequence and fat-suppression pulse aid. Postprocedure processing was performed with a maximum-intensity-projection (MIP) algorithm. RESULTS: In the 92 patients with urinary tract dilatation, 86 had obstructive urinary tract dilatation 6 non-obstructive urinary tract dilatation. In the 86 patients, 14 had ureteral carcinoma, 19 calculus, and 13 benign stricture, 23 congenital ureteral stricture/or with anomalies, 7 ureteral obstructive caused by extrinsic pelvis disease, and 10 bladder carcinoma involved ureter. In the 6 patients, 5 had nervous bladder. MR urography provided the high-resolution image of the urinary tract and determined the presence of obstruction. CONCLUSION: MR urography is a reliable noninvasive method for depicting the urinary tract.

Adolescent↗

Prospective follow-up of patients with bile duct strictures secondary to laparoscopic cholecystectomy, treated endoscopically with multiple stents.

BACKGROUND/AIMS: The frequency of iatrogenic postoperative benign strictures has substantially increased in recent years and this is thought to be because of the widespread use of laparoscopic cholecystectomy. Our study was performed prospectively with the goal to investigate the short-term and long-term outcome for endoscopic treatment with insertion of multiple stents in patients with postoperative benign common bile duct stricture after laparoscopic cholecystectomy. METHODOLOGY: Overall 43 consecutive patients with history of laparoscopic cholecystectomy were enrolled and followed prospectively between December 1998 and December 2003. In all patients a maximum possible number of stents, in relation to the tightness of the stricture and diameter of the bile duct for a period of one year was inserted endoscopically. Patients entered in the follow-up phase after first ERCP procedure with insertion of a biliary plastic stent and continued to be followed after extraction of all stents. RESULTS: Successful endoscopic dilatation of benign biliary stricture after laparoscopic cholecystectomy with placement of multiple biliary plastic stents was achieved in all 43 patients (100%), with a mean follow-up of 16.0+/-11.1 months (range 1 to 42 months) after stent removal. The mean number of multiple plastic stents inserted in one patients with the goal to achieve maximum stricture dilation was 3.4+/-0.6 (range 3 to 5). No recurrence of biliary stricture during or at the end of follow-up was noticed in any patients (100% success rate). CONCLUSIONS: Endoscopic insertion of maximum number of stents in relation to the tightness of the stricture and diameter of the bile duct is highly effective and may improve long-term results for patients with biliary strictures secondary to laparoscopic cholecystectomy.

Bile Ducts↗