Ullrich-Turner Syndrome and Tumor Risk: Is There Another Chance to Early Gonadectomy in Positive TSPY and SRY Patients?

The presence of the Y chromosome in the karyotype of patients with disorders of sex differentiation is significantly associated with an increased risk to develop specific types of malignancies, predominantly type II germ cell tumors (GCTs). Gonadoblastoma in the gonads without an obvious testicular differentiation and intratubular germ cell neoplasia of unclassified type in testicular tissue are the precursor lesions of most GCTs. Gonadal dysgenesis, the characteristic feature of Ullrich-Turner syndrome (UTS), further contributes to increase this tumor risk. The reported incidence of Y chromosome material in UTS is 6 to 8% and in these cases an early gonadectomy is strongly recommended to prevent the risk of a malignancy. The aim of this work was to retrospectively analyze the clinical outcome and the histopathological and cytogenetic findings of our UTS patients who underwent gonadectomy to establish strict selection criteria aimed at promoting an organ-sparing surgery.

Changing concepts in microsurgical pediatric varicocelectomy: is retroperitoneal approach better than subinguinal one?

PURPOSE: To compare and to assess two different microsurgical "lymphatic-sparing" techniques (subinguinal/inguinal vs. retroperitoneal) used for the treatment of a pediatric and adolescent varicocele in terms of success rate, complications and mean operative time. MATERIALS AND METHODS: A retrospective study included 54 consecutive patients affected by a varicocele and treated with a microsurgical (loupes--operating microscope) magnification. Thirty-four out of 54 (group 1) underwent subinguinal ligation with the help of loupes magnification (× 3); 20 out of 54 (group 2) underwent retroperitoneal (Palomo like) ligation with preservation of lymphatics and with the help of an operating microscope (× 6 to 10). The two groups were homogeneous in terms of mean age, clinical and color Doppler ultrasound grade of disease. Pre- and post-operative testicular volume was measured in all cases. All the procedures were performed under general anesthesia and in an outpatient basis. RESULTS: Mean post-operative follow-up time was 23.6 months. In group 1 we observed 3 (8.8%) early complications (wound's infection, transient hydrocele), 2 (5.8%) recurrences and 1 (2.9%) major complication (atrophy of the testis). Mean operative time was 45 +/- 6 min. In group 2 we did not observe complications and/or varicocele recurrence and mean operative time was 38 +/- 7 min. Comparison of mean operative time between the two groups resulted statistically significant differences (P < .05) such as the difference in testicular "catch-up" growth volume between pre- and post-operative evaluations. CONCLUSION: Retroperitoneal microsurgical "lymphatic-sparing" varicocelectomy is safe and effective method. In our experience, it is preferable, in the pediatric and adolescent patient, to the subinguinal/inguinal approach in terms of success rate, complications and operative time duration. 

Cobb's collar: a rare cause of urinary retention.

Cobb's collar is a membranous stricture of the bulbar urethra, often misdiagnosed, potentially leading to acute urinary retention, upper urinary tract dilations, enuresis, infectious diseases, poor streaming and haematuria. A 14-year-old boy with a history of previous hospitalisations came to our attention. A Cobb's collar was diagnosed and successfully managed with a videoendoscopic one-step approach. Although extremely uncommon, paediatric urologists must be aware of this congenital anomaly. A correct and timely diagnosis and endoscopic management may affect positively the clinical onset of these patients avoiding major urethral surgery and preserving renal and bladder function.

Management and follow-up of pediatric asymptomatic testicular microlithiasis: are we doing it well?

PURPOSE: To define timing and methods for a balanced follow-up of testicular microlithiasis (TM) in pediatric age. MATERIALS AND METHODS: We retrospectively reviewed medical records of 21 pediatric asymptomatic patients (42 testicular units) diagnosed with TM and without associated risk factors. Microliths were found bilaterally on ultrasonography in all the patients. Distribution of microliths (focal or diffuse) inside the parenchyma was evaluated as well as its eventual variation over time. Every six months, each patient underwent clinical and ultrasonography evaluation, as well as serum chemistry markers (α-fetoprotein and ß-human chorionic gonadotropin) measurement to detect potential malignancy. In the interval between the follow-ups, parents and/or patients themselves were asked to control eventual enlargement of the gonads or scrotal swelling. Testicular biopsy was not performed in any of our subjects. RESULTS: Of 21 patients, 6 had unilateral undescended testis, 4 varicocele, and 1 patent processus vaginalis with scrotal swelling while 10 patients did not show associated anomalies. The distribution pattern of microliths on ultrasonography remained unchanged in all follow-ups in every patient, showing a predominance of diffuse pattern in the undescended testis series. Tumor markers remained within normal limits. In no subject, we observed a shift toward a malignant condition. CONCLUSION: In the pediatric population with an incidentally diagnosed TM and without any associated risk factor, a slight follow-up is suggested, consisting of clinical evaluation every 6 months, without any justifiable recommendation to perform a testis biopsy and a measurement of serum tumor markers.

Endoscopic management of a type IV syringocele causing massive perirenal urinoma in an infant.

The finding of a urinoma in the fetal and neonatal period is almost always related to an obstruction distal to the collection itself. Syringocele is a congenital or acquired dilation of a bulbourethral Cowper's gland. An exceedingly rare case of neonatal anterior urethral obstruction caused by a syringocele and manifesting itself with a dramatic clinical onset is presented. Clinical and radiologic findings are discussed and minimally invasive therapeutic strategies emphasized.