Chronic respiratory disorders due to aberrant innominate artery: a case series and critical review of the literature.

BACKGROUND: Tracheal compression (TC) due to vascular anomalies is an uncommon, but potentially serious cause of chronic respiratory disease in childhood. Vascular slings are congenital malformations resulting from abnormal development of the great vessels; in this group of disorders the most prevalent entity is the aberrant innominate artery (AIA). Here we provide a report on diagnosis and treatment of AIA in nine children with unexplained chronic respiratory symptoms. We describe the cases, perform a literature review, and provide a discussion on the diagnostic workup and treatment that can help manage AIA. METHODS: Clinical history, diagnostic procedures and treatment before and after the AIA diagnosis were retrospectively reviewed in nine children (5 boys and 4 girls), who were referred for recurrent-to-chronic respiratory manifestations over 10 years (2012-2022). We performed a comprehensive report on the ongoing clinical course and treatment as well as an electronic literature search on the topic. RESULTS: Diagnoses at referral, before AIA was identified, were chronic dry barking cough associated with recurrent pneumonia (n = 8, 89%), lobar/segmental atelectasis (n = 3, 33%), atopic/non atopic asthma (n = 3, 33%); pneumomediastinum with subcutaneous emphysema complicated the clinical course in one case. When referred to our Unit, all patients had been previously treated with repeated antibiotic courses (n = 9, 100%), alone (n = 6, 67%) or combined with prolonged antiasthma medications (n = 3, 33%) and/or daily chest physiotherapy (n = 2, 22%), but reported only partial clinical benefit. Median ages at symptom onset and at AIA diagnosis were 1.5 [0.08-13] and 6 [4-14] years, respectively, with a relevant delay in the definitive diagnosis (4.5 years). Tracheal stenosis at computed tomography (CT) was ≥ 51% in 4/9 cases and ≤ 50% in the remaining 5 subjects. Airway endoscopy was performed in 4 cases with CT evidence of tracheal stenosis ≥ 51% and confirmed CT findings. In these 4 cases, the decision of surgery was made based on endoscopy and CT findings combined with persistence of clinical symptoms despite medical treatment. The remaining 5 children were managed conservatively. CONCLUSIONS: TC caused by AIA may be responsible for unexplained chronic respiratory disease in childhood. Early diagnosis of AIA can decrease the use of expensive investigations or unsuccessful treatments, reduce disease morbidity, and accelerate the path toward a proper treatment.

Metastatic choriocarcinoma with hemorrhagic complications and spontaneous ovarian hyperstimulation syndrome: A case report.

Gestational choriocarcinoma is a malignant trophoblastic tumor arising from any gestational event, even with a long latency period, generally in the reproductive female. It is associated with a high level of beta-human chorionic gonadotropin. Its primary site is usually the uterus but not all patients have a detectable lesion in this site. Regression of the primary tumor after it has metastasized is not uncommon, and one-third of cases manifest as complications of metastatic disease. In this report we present an uncommon case of gestational choriocarcinoma with lung, liver and jejunal metastases at the time of diagnosis without evidence of pelvic disease, in 34-year-old woman. The main points of interest of our case were the development of the ovarian hyperstimulation syndrome with massive multicystic ovarian enlargement induced by high level of beta-human chorionic gonadotropin and the bleeding of jejunal and liver metastases, due to the high vascularity of the tumor tissue, a condition known as "Choriocarcinoma Syndrome". We will focus on the radiological findings of metastases, bleeding complications and ovarian hyperstimulation syndrome.

Cerebellum and cognition in Friedreich ataxia: a voxel-based morphometry and volumetric MRI study.

BACKGROUND: Recent studies have suggested the presence of a significant atrophy affecting the cerebellar cortex in Friedreich ataxia (FRDA) patients, an area of the brain long considered to be relatively spared by neurodegenerative phenomena. Cognitive deficits, which occur in FRDA patients, have been associated with cerebellar volume loss in other conditions. The aim of this study was to investigate the correlation between cerebellar volume and cognition in FRDA. METHODS: Nineteen FRDA patients and 20 healthy controls (HC) were included in this study and evaluated via a neuropsychological examination. Cerebellar global and lobular volumes were computed using the Spatially Unbiased Infratentorial Toolbox (SUIT). Furthermore, a cerebellar voxel-based morphometry (VBM) analysis was also carried out. Correlations between MRI metrics and clinical data were tested via partial correlation analysis. RESULTS: FRDA patients showed a significant reduction of the total cerebellar volume (p = 0.004), significantly affecting the Lobule IX (p = 0.001). At the VBM analysis, we found a cluster of significant reduced GM density encompassing the entire lobule IX (p = 0.003). When correlations were probed, we found a direct correlation between Lobule IX volume and impaired visuo-spatial functions (r = 0.58, p = 0.02), with a similar correlation that was found between the same altered function and results obtained at the VBM (r = 0.52; p = 0.03). CONCLUSIONS: With two different image analysis techniques, we confirmed the presence of cerebellar volume loss in FRDA, mainly affecting the posterior lobe. In particular, Lobule IX atrophy correlated with worse visuo-spatial abilities, further expanding our knowledge about the physiopathology of cognitive impairment in FRDA.

Machine learning applications in prostate cancer magnetic resonance imaging.

With this review, we aimed to provide a synopsis of recently proposed applications of machine learning (ML) in radiology focusing on prostate magnetic resonance imaging (MRI). After defining the difference between ML and classical rule-based algorithms and the distinction among supervised, unsupervised and reinforcement learning, we explain the characteristic of deep learning (DL), a particular new type of ML, including its structure mimicking human neural networks and its 'black box' nature. Differences in the pipeline for applying ML and DL to prostate MRI are highlighted. The following potential clinical applications in different settings are outlined, many of them based only on MRI-unenhanced sequences: gland segmentation; assessment of lesion aggressiveness to distinguish between clinically significant and indolent cancers, allowing for active surveillance; cancer detection/diagnosis and localisation (transition versus peripheral zone, use of prostate imaging reporting and data system (PI-RADS) version 2), reading reproducibility, differentiation of cancers from prostatitis benign hyperplasia; local staging and pre-treatment assessment (detection of extraprostatic disease extension, planning of radiation therapy); and prediction of biochemical recurrence. Results are promising, but clinical applicability still requires more robust validation across scanner vendors, field strengths and institutions.

Synchronous mucinous colonic adenocarcinoma and multiple small intestinal adenocarcinomas: report of a case and review of literature.

With the wide use of diagnostic imaging modalities, multiple primary malignancies frequently occur; different associations of malignancies have been reported. We describe the case of a primary mucinous adenocarcinoma of large bowel synchronous with three primary poorly differentiated adenocarcinomas of ileum. This type of association has not been described yet; since computed tomography increasingly is proving to be highly accurate in detection of colon cancer, this technique is recommended in such patients.

Radiofrequency-assisted partial nephrectomy for metanephric adenoma: a case report and literature review.

Metanephric adenoma (MA) is a rare benign tumor, diagnosis of which is often carried out after surgical treatment. In case of peripheral lesions, a partial nephrectomy (PN)--either open or laparoscopic may be preferred--and, furthermore, a radiofrequency (RF)-assisted procedure may facilitate adequate hemostasis. In November 2010, the authors performed a RF-assisted PN, according to Habib's technique, using a 4-needle bipolar device, on a woman affected by a small exophytic MA of the right kidney. Fibrin glue was applied on the cut surface. Postoperative course was uneventful, and discharge was on postoperative day 4. MA is an extremely rare benign tumor with a favorable prognosis. In case of a preoperative cytological diagnosis, a careful follow-up has to be considered. PN represents the standard of care for small exophytic MA, and RF-assisted procedures allow an excellent hemostasis and a rapid conservative resection, with very low morbidity.

A case of valproic acid-induced acute pancreatitis in tuberous sclerosis coexisting with end-stage renal disease.

Tuberous sclerosis complex (TCS) is a genetic disorder with a variable clinical presentation. It is commonly characterized by seizures, mental retardation and cutaneous angiofibromas. Renal manifestations frequently include angiomyolipomas and cysts which lead to chronic kidney disease. We report a case of valproic acid-induced acute pancreatitis in a dialysis patient affected by TCS. The case demonstrates the importance of assessing antiepileptic drug treatment in dialysis patients.

Malignant fibrous histiocytoma of the stomach.

MFH is a kind of sarcoma, normally located in the soft tissues of the extremity and retroperitoneal space. The involvment of the gastroenteric tube and particularly of the stomach are rather rare. Our case shows some differences from the others described in literature: the sight of mass, the age of the patient, the extension at the moment of the diagnosis.

La embolización arterial en patología tumoral / Arterial embolization in tumoral pathology

Los autores refieren las principales indicaciones y limitaciones de la embolización de tumores resultantes de la experiencia obtenida en varios cientos de pacientes. 70% de las embolizaciones fueron de tipo paliativo y de ellas el 60% para reducir el dolor o el volumen tumoral, cerca de 40% con fines hemostáticos y el resto para controlar secreciones tumorales. Las embolizaciones curativas se asociaron en general a cirugía. Describen limitaciones en pacientes con cirugía o radioterapia previa y las consecuencias de la necrosis y liberación secundaria de substancias vasoactivas