Practical and functional classification of the double urethra: A variable, complex and fascinating malformation observed in 20 patients.

INTRODUCTION: Urethral duplication is a rare and variable malformation of the urinary tract, with non-univocal and complex management. In addition, different classification schemes have been proposed, but none have considered all the possible variants. OBJECTIVE: To report experience with the management of 20 urethral duplication patients and propose a classification of this anomaly. MATERIALS AND METHODS: A retrospective analysis collecting information regarding 20 patients (Table) with urethral duplication treated at a single institution over the past 40 years. Three females and 17 males were treated: two had vesico-urethral duplication, eight had urethral duplication with a single bladder, and eight had 'λ' duplication. RESULTS: Immediate postoperative complications included urethral dehiescence (n = 1) and urethral stenosis (n = 2). The progressive augmentation by dilating the urethra (PADUA) technique was ineffective. During follow-up, the following were recorded: urinary incontinence (n = 2), urinary tract infection (n = 3), hypertension (n = 3) and erectile dysfunction (n = 1). All patients were satisfied with the aesthetic result. DISCUSSION: Urethral duplication is a rare anomaly with male preponderance. Four types of duplication were described, on the basis of anatomy and management: vesical and urethral duplication (type 1), urethral duplication with single bladder (type 2), 'λ-type' duplication (type 3) and 'miscellanea' (multiple urethral channels, spindle urethra, other female forms, type 4). A full description of the malformation and surgical approach was given for each type. The advantages of the classification were compared with the literature. CONCLUSIONS: The proposed classification should be a useful tool, based on the required surgical approach, even if surgery should be tailored to the patient. It is important to restore the anatomy and achieve urinary continence. Surgery can be challenging and a multi-step process, especially in cases of 'λ' duplications.

Long-term follow-up after esophageal replacement in children: 45-Year single-center experience.

PURPOSE: We evaluated the results of esophagocoloplasty (ECP) in children by performing a 45-year retrospective cohort study in a single center. MATERIALS AND METHODS: We collected all of the medical charts of patients who underwent ECP at our hospital from January 1969 to January 2014. We reviewed the medical history for each patient and then contacted the patients by phone to obtain responses to a questionnaire. The questionnaire evaluated the following four areas: overall general state, gastrointestinal function, dependence on the Health Sanitary System, and their aesthetic satisfaction. RESULTS: We contacted 96 patients, and 72 completed our questionnaire. There were 45 males and 27 female respondents, with a mean age of 34.5 years. The mean Karnofsky performance status index was 96.4%. There were excellent scores obtained from 55 patients and fairly good scores from 16 patients. There was 1 patient with a low score, and there were no scores reported below 70%. Most patients (58/72) reported regular bowel habits, and no patients required drugs or other measures to facilitate defecation. Gastroesophageal reflux was present in 51 patients, and 62.8% have bothersome symptoms. Twenty-five patients used pharmacological therapies. Forty-eight patients (66.6%) were satisfied with the aesthetic result of surgery (mean score in a scale from 0 to 5, is 3). CONCLUSIONS: ECP can be used in children who require esophageal substitution. The resulting long-term QoL is acceptable. However, the aesthetic outcomes remain a problem.

Incidence and correlates of complex ventricular arrhythmias during dobutamine stress echocardiography after acute myocardial infarction.

Although previous studies have confirmed the safety of dobutamine stress echocardiography, complex ventricular arrhythmias have been reported. Our aim was (1) to identify the markers of increased arrhythmic risk during dobutamine stress echocardiography and (2) to assess whether the occurrence of major ventricular arrhythmias during the test may represent a clinically useful marker of electrical instability. Three hundred and seventy-seven consecutive survivors from acute myocardial infarction, off cardioactive therapy, underwent dobutamine stress echocardiography 11.4 days after the acute event. Holter monitoring with assessment of heart rate variability and echocardiographic determination of left ventricular ejection fraction. In addition, exercise stress testing, signal averaged ECG and coronary angiography were carried out, respectively, in 357, 150 and 273 patients. Ten subjects showed complex ventricular arrhythmias (eight non-sustained and one sustained ventricular tachycardia and one ventricular fibrillation) during dobutamine stress echocardiography (group A), whilst 366 did not (group B). Complex ventricular arrhythmias were detected by Holter monitoring in 8/10 patients in group A and 45/367 patients in group B (odds ratio 28.6, 95% CI 5.4-92.2) and by exercise testing in 4/10 patients in group A and 33/347 patients in group B (odds ratio 6.3, 95% CI 1.4-27.2). Ejection fraction < 40% was present in 3/10 patients in group A and 50/367 in group B (odds ratio 2.7, 95% CI 0.3-12.2), whilst multivessel disease was present, respectively, in 8/10 and 176/263 patients (odds ratio 1.9, 95% CI 0.3-25.5). Reduced heart rate variability and the presence of late potentials on signal averaged ECG were found in, respectively, 40/367 and 13/140 patients in group B, but none were found in group A. A total of 61 events (35 CABG, 15 PTCA, four cardiac deaths and seven non-fatal reinfarctions) occurred during the follow-up (11.4 months, range 6 to 20): four in group A and 57 in group B. No documented major arrhythmic event was reported. We conclude that (1) complex arrhythmias during dobutamine stress may occur in patients early after acute myocardial infarction; (2) the preexisting evidence of frequent, as well as repetitive, arrhythmias represents a potential marker of increased risk in this connection and, finally, (3) dobutamine-induced arrhythmias seem to represent an uncommon, even though potentially dangerous, event but not a useful new "window' on electrical instability of post-MI patients.

Vasopressin release and water metabolism in patients with cirrhosis.

Water retention is a complication in many patients with cirrhosis, usually attributed to excessive release of arginine vasopressin. To investigate the responsiveness of arginine vasopressin to osmotic and non-osmotic stimuli and its relationship to free water excretion, we studied 19 patients with cirrhosis under three different conditions: 45 min with legs raised to 60 degrees, to expand the central blood volume; infusion of 1000 ml of 0.45% saline solution to reduce plasma osmolality; and rapid injection of 50 ml of 2 M NaCl to increase plasma osmolality. Both expansion of central blood volume and decrease of plasma osmolality significantly reduced plasma vasopressin levels (from 2.1 +/- 0.6 to 1.39 +/- 0.3 pg/ml, p < 0.04; and from 1.09 +/- 0.25 to 0.41 +/- 0.13 pg/ml, p < 0.0001). The changes in free water excretion differentiated two subgroups of patients during each test: excretors and non-excretors. In the excretors, increased free water excretion was associated with suppressed vasopressin levels (below 0.5 pg/ml) and normal renal function. In the non-excretors, inability to improve free water excretion was associated with high vasopressin levels or with reduced distal delivery of the glomerular filtrate, except in some cases where vasopressin levels had fallen below 0.5 pg/ml and renal function was normal. For these cases the presence of other vasopressin-independent antidiuretic mechanisms is conceivable. The injection of hypertonic saline solution caused significant rises in plasma osmolality (from 287 +/- 1.9 to 292 +/- 1.6 mmol/kg, p < 0.05) and in plasma vasopressin levels (from 1.13 +/- 0.29 to 2.86 +/- 0.52 pg/ml, p < 0.05). These results suggest that vasopressin release in patients with cirrhosis is normally responsive to osmotic and non-osmotic stimuli, although our results show a lower theoretical osmolar threshold for suppression of vasopressin release in non-excretors than in excretors (276 vs 284 mmol/kg).

Improvement in identification of multivessel disease after acute myocardial infarction following stress-recovery analysis of ST depression in the heart rate domain during exercise.

The demonstration of extensive coronary artery disease (CAD) after acute myocardial infarction (AMI) has important prognostic implications. Exercise-induced ST segment depression is commonly used for detecting the presence of CAD and evaluating its extension. However, even though there have been many attempts to increase its diagnostic yield, the accuracy of the electrocardiographic signal for identifying multivessel disease (MVD) is relatively low, particularly in post-MI patients. The aim of this study was to evaluate the ability of a simple index, combining information on the amount and kinetics of ST depression in the heart rate domain during exercise and recovery, to identify MVD after AMI. Seventy patients (mean age 53.4 years) underwent a bicycle, symptom-limited exercise stress test and coronary angiography 2-3 weeks and 6 weeks respectively, after uncomplicated AMI while cardioactive therapy was discontinued. After obtaining a computer-derived measurement of ST levels based on incremental averaging of normal complexes, the area subtended to baseline and limited by the ST trend against heart rate during both exercise (A1) and recovery (A2) was calculated. The difference (A1-A2) was defined as the 'Stress-Recovery Index' (SRI) and dichotomized, by means of receiver-operating characteristics curve analysis, at 5 mm x beats.min-1 to define an increased risk of MVD. The SRI of patients with MVD was significantly lower than that of patients with single vessel disease. The sensitivity of SRI < -5 mm x beats.min-1 (65%) for predicting MVD was significantly higher than that obtained by other conventional parameters, without appreciable loss of specificity (81%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Leiomyosarcoma of the rectum]. / Il leiomiosarcoma del retto.

Two cases of leiomyosarcoma of the rectum observed between 1980 and 1990 are reported. Both patients underwent abdominoperineal resection: one is still alive at three years from surgery, whereas the other died for neoplastic diffusion after three years. Epidemiological, clinical and therapeutic features of this rare tumor are discussed.