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BACKGROUND: Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Müllerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting. CASES PRESENTATION: two cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance (MR) imaging technology to achieve the correct diagnosis. CONCLUSIONS: MR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome.
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Imagen por Resonancia Magnética , Anomalías Urogenitales/diagnóstico , Adolescente , Diagnóstico Diferencial , Femenino , Humanos , Riñón/anomalías , Conductos Paramesonéfricos/anomalías , Síndrome , Útero/anomalíasRESUMEN
Sister Mary Joseph nodule is a nodule localized in the umbilicus which represents a cutaneous metastasis from a primary cancer, mostly localized in the abdomen. We report the case of an 82-year-old woman who presented with an ulcerated umbilical nodule. Imaging identified it as a Sister Mary Joseph nodule, representing the only distant metastasis from a silent caecal adenocarcinoma. When an umbilical nodule is found at clinical examination imaging can play a crucial role in determining underlying pathology and in determining therapeutic strategy.
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AIM: To prospectively investigate a new high resolution MRI technique for dynamic evaluation of the enhancement kinetics of bowel parietal layers and to correlate it with CDAI, CRP, endoscopic activity and histologic features. METHODS: About 16 consecutive patients with proven diagnosis of CD underwent ileocolonoscopy with biopsy and serial bowel dynamic contrasted-MRI (D-CE-MRI) evaluated in blind fashion. Quantitative analysis of bowel wall enhancement kinetics was performed basing on signal to noise ratio (SNR) of inner parietal layers (Mucosa-Submucosa, M-SM) and outer parietal layers (Muscular-Serosa, Ms-S). Disease activity was defined by CDAI > 150, serum CRP > 5 mg/dL and histologic results. RESULTS: About 9 patients showed a layered enhancement of bowel wall (8 active, 1 inactive), whereas inactive (7 cases) group presented a homogeneous pattern. In active patients we found a significant difference in parietal layered enhancement curves (M-SM vs. Ms-S, P < 0.03) not observed in inactive disease and controls (intra-group analysis). M-SM and Ms-S enhanced curves in clinically active patients were significantly different respect to those of patients with inactive CD (P < 0.001) (inter-group analysis). Parietal D-CE-MRI pattern well correlated with histologic features (r = 0.8; P < 0.001, Spearman test). CONCLUSIONS: D-CE-MRI can be a useful tool for clinical follow-up and in the treatment strategies in CD patients.
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Enfermedad de Crohn/diagnóstico , Íleon , Imagen por Resonancia Magnética/métodos , Adulto , Medios de Contraste , Diagnóstico Diferencial , Endoscopía Gastrointestinal , Femenino , Gadolinio DTPA , Humanos , Masculino , Estudios Prospectivos , Estadísticas no ParamétricasRESUMEN
Portomesenteric vein gas can occur owing to a variety of interraleted factors such as loss of mucosal integrity and intraluminal overpressure, and the most common and serious cause is bowel ischemia, which requires urgent laparotomy. Nevertheless, when portal venous gas is caused by nonischemic causes, surgery is not required and it can be treated conservatively. So, its features should be carefully evaluated at CT scan, together with clinical findings. The authors report a case of an old male with portomesenteric venous system gas after CT colonography, without evidence of pneumatosis intestinalis or colonic perforation. A CT scan without enema was required after 24 hours in absence of worsened patient conditions, revealing the disappearance of gas in mesenteric vein and in the portal venous system.
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Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy caused by intrauterine arrest of compaction of the myocardial fibres and meshwork, an important process in myocardial development. ILVNC is clinically accompanied by depressed ventricular function, arrhythmias, and systemic embolization. We reported a case of ILVNC with basal ECG-tracing strongly suggestive for type-2 Brugada syndrome (BrS). Up to now, this is the first report investigating the association between ILVNC and this particular ECG pattern.
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BACKGROUND: The developmental origin theory of coronary heart disease proposes that undernutrition in utero permanently changes body functions and metabolism leading to an increased risk of coronary artery diseases (CAD) in adult life. Some studies support this theory but others suggest that birth weight (BW) is not a major risk factor for cardiovascular diseases. Gender differences concerning the association between BW and risk factors for CAD have been reported in some studies but not in others.In this paper we have analyzed the effect of gender and diabetes on the relationship between BW and CAD in the White population of Rome. MATERIAL AND METHODS: 226 subjects admitted to the Hospital for non fatal CAD from the White population of Rome were studied. 395 consecutive newborn infants studied in the same population in the years 1968-1972 were considered for comparison. RESULTS: Among subjects with CAD, reliable information on BW was obtained in 127 subjects. The distribution of BW in CAD depends on gender (p=0.009). In females with CAD there is a tendency toward low BW, while in males with CAD there is a tendency toward high BW. These associations are very marked in non-diabetic subjects with CAD (p=.001), while no significant association is observed in diabetic subjects (p=0.557). CONCLUSION: Our data confirm the association between BW and CAD and suggest that the association depends on gender and is influenced by diabetes.
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Peso al Nacer , Enfermedad de la Arteria Coronaria/epidemiología , Diabetes Mellitus/epidemiología , Anciano , Presión Sanguínea/fisiología , Enfermedad de la Arteria Coronaria/etiología , Femenino , Humanos , Recién Nacido , Masculino , Factores de Riesgo , Ciudad de Roma/epidemiología , Caracteres SexualesRESUMEN
The most important systemic etiological factors for portal thrombosis are the thrombophilic conditions associated with myeloproliferative disorders (MPDs), either clinically manifest or so-called 'occult'. Latest studies have revealed a strong association between MPDs and a somatic point mutation (V617F) of the tyrosine kinase Janus kinase2. The 'pseudo-cholangiocarcinoma sign' is a well described radiological finding related to portal cavernoma. We describe the case of a 48-year-old man, presenting with cavernomatous transformation of an extrahepatic thrombotic obstruction of the portal vein mimicking a neoplastic mass of the hepatic hilum, who did not present any other cause for thrombosis except for the V617F Janus kinase2 mutation, suggesting an underlying MPD.