Vascular rings are congenital anomalies that lead to variable degrees of respiratory problems or feeding difficulties by forming a complete or partial ring compressing the trachea, the bronchi, and the esophagus. The clinical diagnosis of vascular rings is often challenging for the pediatrician because the clinical manifestations are heterogeneous and nonspecific. Symptoms can vary from wheezing, stridor, dyspnea, and/or dysphagia to life-threatening conditions; however, they may not be present. The aim of this study is to review the recent literature on this subject and describe new developments in diagnostics and imaging.
The pediatric ultrasound measurement of the inferior vena cava (IVC) and aorta (AO) with the study of the collapsibility index (CI) and of IVC-to-AO ratio (IVC/AO) can provide clinicians in the acute care setting with information on abnormal volume status but one of the major limitations is a lack of reference normal values by body surface area (BSA) and age. The aim of this study was to provide reference ranges for the sonographic measurement of IVC, AO, and IVC/AO ratio in healthy Caucasian Italian children. Methods: We enrolled prospectively 516 healthy Caucasian Italian children aged between 1 month and 16 years. Echocardiographic IVC and AO diameters were collected and presented separately for children aged 1 year and for children aged over 1 year. For children >1 year we categorized subjects into 3 years classes. CI and IVC/AO for the systolic aortic diameter were then calculated. For children over 1 year, age reference ranges were age-related or BSA-related; for children of 1 year, reference ranges were determined with their 90% confidence intervals regardless of age and of BSA. Results: Tables and charts with reference ranges for all the echocardiographic measurements are presented for children aged >1 year according to age and BSA. The equations to obtain percentile and Z-score for each echocardiographic measurement are provided. The reference ranges for children aged 1 year are shown considering the small 90% confidence intervals for upper and lower limits. CI was 30% (SD 17%) in children >1 year and 36% (SD 16%) in children <1 year. IVC/AOs showed agedependent values from 0.83 (SD 0.20) age <1 year to 1.22 (SD 0.31) in older subjects. Conclusions: We report reliable reference ranges for echocardiographic measurement of IVC, AO, CI, and IVC/AO for a Caucasian Italian healthy pediatric population.
Ovarian tumors associated with hormonal changes of the peripheral iso-sexual precocious puberty are of common presentation. We describe here a rare case of juvenile granulosa cell tumor in a female with central precocious puberty (CPP). An 8-year old girl with CPP presented with vaginal bleeding four months after the diagnosis and before starting treatment with gonadotropin-releasing hormone (GnRH)-analogs. Suppression of basal follicle-stimulating hormone (FSH) level, elevation of serum estradiol, progesterone and Cancer Antigen-125 were documented. Abdominal ultrasound examination (US) and magnetic resonance imaging showed a pelvic mass affecting the left ovary. A left salpingo-oophorectomy was performed and the mass was totally resected. Juvenile granulosa cell ovarian tumor was diagnosed. One month post surgery, estradiol and progesterone decreased to values of the first evaluation and FSH increased; Cancer Antigen-125 resulted normal while ultrasound pelvic examination showed absence of pelvic masses. In our patient, the tumor had grown very quickly since hormonal data demonstrated a CPP without any evidence of ovarian mass on US only four months before diagnosis. The overstimulation of the FSH or aberrant activation of FSH receptors may have contributed to the development of the mass.
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