0000000000075747
AUTHOR
Cinzia Chiaramonte
Unusual case of continuous urinary dribbling in a young female due to unknown complex urogenital malformation: magnetic resonance features
Congenital anomalies of kidney and urinary tract (CAKUT) occur in up to 3.2% of infants, and in almost one third of cases they are associated to genital aberrations. DMSA renal scintigraphy is considered the gold standard in CAKUT patients, to assess renal function, depict and locate ectopic kidney and to guide eventual surgical management. Recent cases have shown the noticeable limit of scintigraphy in detecting poorly functional renal moieties and associated genital anomalies; thus leading to a substantial delay in therapy, and, in females, to severe uro-gynecological complications. We herein describe a case of a young girl with unusual urinary dribbling, where DSMA scintigraphy was not d…
Early intestinal perforation secondary to congenital mesenteric defects
Abstract Gastrointestinal perforation (GIP) in preterm neonates may be idiopathic, due to necrotizing enterocolitis (NEC), or mechanical obstruction. The predominant cause of GIP in the neonatal period is NEC. Differential diagnosis with congenital malformations, including mesenteric defects leading to internal hernias, is mandatory if the onset is early. We describe two newborns with trans-mesenteric herniation resulting in GIP, and we discuss the presence of possible additional risk factors such as prematurity and predisposing vascular disruption in connective tissue disorders (Ehlers-Danlos syndrome), twinning, and use of assisted reproductive technologies. These cases prompted us to rev…
Bladder Mucosal Graft Vaginoplasty: A Case Report
Abstract Background Female vaginoplasty reconstruction, by choice, is usually performed with adjacent tissue. However in some clinical conditions such as high urogenital confluence sinus, cloacal malformation with extreme vaginal hypoplasia, local tissue may not be available. When vaginal replacement is performed in pediatric patients intestinal segments is preferred to non-operative procedures that require continuative dilations. However mucus production, malignant transformation risk and diversion colitis are important side effects. Technique We present a nouvel technique for vaginoplasty in a female child presenting with an isolated urogenital sinus malformation without virilization. The…
Splenogonadal Fusion: A Genetic Disorder?—Report of a Case and Review of the Literature
Splenogonadal fusion is a rare congenital anomaly in which there is fusion between the spleen and gonad, epididymis or vas. We treated a patient with scrotal mass. A discontinuous fusion between the spleen and epididymis was found: after frozen section mass was excised sparing testis. Although rare, the splenogonadal fusion should be considered in the differential diagnosis of scrotal masses in children, and orchiectomy should be avoided. Moreover, examination of siblings showed a brother affected from accessory spleen. This finding, not reported previously to our knowledge, suggests a possible familiarity of this disorder. Keywords: Splenogonadal fusion, Testis, Accessory spleen