Zinner syndrome in pediatric patients: rare disease leading to challenging management.

Introduction: Zinner syndrome (ZS) is the association of seminal vesicle cysts, ipsilateral ejaculatory duct obstruction, and ipsilateral renal agenesis. This condition is very rare in children and both diagnosis and treatment may be challenging. We reviewed the clinical presentation and treatment describing our experience with a series of three patients. Methods: From January 2016 to January 2021, three patients (patients 1, 2, and 3) with symptomatic ZS, aged 2, 15, and 17 years, respectively, were diagnosed and treated. All three patients were symptomatic, manifesting pelvic pain and dysuria. The diagnosis was made by physical examination, ultrasonography, and abdominopelvic MRI. Patient 1 underwent open surgery, while for patients 2 and 3, laparoscopic excision was performed. Results: The renal agenesis regarded the left side in patients 1 and 3, and the right side in patient 2. In all cases, the cystic complex was excised. The mean operating time was 4 h and the mean hospitalization time was 5 days (range 4-6 days). The mean follow-up period was 5 years (range 2-5 years). Patients 1 and 3 showed a complete resolution of the symptoms during postoperative follow-up. In patient 2, clinical symptoms relapsed because of the persistence of a 9 mm cyst requiring a redo laparoscopic excision. Conclusions: Seminal vesicle cyst with ipsilateral renal agenesis, even if rare in pediatric age, should be suspected in young male patients presenting with pelvic cystic masses, pelvic pain, dysuria, and ipsilateral renal absence. Conservative management should be reversed to asymptomatic patients. Surgical treatment is mandatory in symptomatic cases and the preferred approach is minimally invasive surgery to magnify the operating field to spare anatomical structures, primarily the contralateral vas deferens. Radicality is crucial to avoid the persistence of symptoms and the need for reintervention.

Evaluation of educational value of YouTube videos addressing robotic pyeloplasty in children.

BACKGROUND/INTRODUCTION: Ureteropelvic junction obstruction (UPJO) is the most frequent obstructive uropathy of the upper urinary tract in children. Video-sharing platforms have become a significant source of visual information for health care providers. Among these platforms, YouTube® (www.youtube.com), contains a high number of videos free of charge and represents one of the most important and known websites of video-sharing. YouTube® is a widely used open-access video sharing website that allows us to watch an unlimited number of video content, and to upload an infinitive number of videos. OBJECTIVE: This study aims to evaluate the educational quality of videos related to robotic pyeloplasty in pediatric age because an increasing number of videos addressing these procedures is now available on YouTube®. STUDY DESIGN: We performed a search on YouTube® by using the following keyword: "robotic pyeloplasty in children" on July 9, 2020. The first 50 videos were analyzed. The videos were classified according to the source in 1) academic (author/s was/were affiliated with a university), 2) physician (author/s who was/were not affiliated with a university), 3) patient, 4) commercial. All the videos were evaluated also according to the content in 1) surgical technique, 2) information about the surgery and disease 3) patient personal experience 4) advertisement. Duplicated videos and videos not in English were excluded. The search for videos was done based on the website's default settings in order of the proposed relevance. The reliability was evaluated using DISCREN and JAMA scores. The Global Quality Score (GWS) was used to assess the educational value. Time since upload, run time, like, dislike and number of views were recorded. RESULTS: The first 50 videos were analyzed. Seven videos (14%) did not meet our criteria and were excluded (three videos were duplicated while 4 out of seven were not in English). The mean DISCERN was 32.47 ± 12.24 (range 15-78). The mean JAMA Score was 2.1 ± 0.9 (range 0-4). Mean GQS was 2,12 ± 0.9. DISCERN and JAMAS and GQS scores of academic/physician sourced videos were significantly higher than the patient sourced videos (p = 0.037, p = 0.023, p = 0.017 respectively). Regarding content, the surgical technique had significantly higher DISCERN, JAMAS and GQS scores than videos based on patient experience (p = 0.012, p: 0.021, p = 0.023 respectively). CONCLUSIONS: Videos uploaded by Physicians and Academic Institutions show higher DISCERN and JAMAS and GQS compared to other sourced videos and should be considered more suitable for teaching respect to those originating from patients or non-physicians.

Individualized robotic organ-sparing surgery in a paediatric case of renal perivascular epithelioid cell tumour.

BACKGROUND: Perivascular epithelioid cell tumours (PEComas) represent a rare group of mesenchymal tumours with an unpredictable outcome. They are usually observed in middle-aged women whilst they are very rare in children, with limited cases described. Due to the rare anatomical location in kidneys, a correct diagnosis and treatment is really challenging. METHODS: We observed and treated a case of kidney's PEComa in a 14-year-old boy. RESULTS: An individualized approach based on anatomical (3D CT-reconstructions) and histopathological (US-guided preliminary biopsy) features lead us to perform a successful robotic-sparing surgery enabling the preservation of two-thirds of the kidney involved. CONCLUSIONS: A meticulous preoperative planning in selected patients can lead to a minimally invasive approach even in some paediatric kidney's neoplasms.

A Simplified Management of Transverse Testicular Ectopia in Patients with Persistent Mullerian Duct Syndrome.

Persistent müllerian duct syndrome (PMDS) in the majority of cases is discovered during surgery for inguinal hernia or cryptorchidism. A transverse testicular ectopia (TTE) with cryptorchidism may be very rarely associated to PMDS. Assuming that müllerian remnants have a very low malignant degeneration potential if compared to the malignancy risk of an undescended and not relocated testis, we describe a simplified surgical technique of orchiopexy that avoids an extensive anatomical dissection, in this way minimizing the risk of losing the deferential blood supply to the testis.

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.

Enterobius Vermicularis as a Cause of Intestinal Occlusion: How To Avoid Unnecessary Surgery.

Enterobius vermicularis may cause infections of the gastrointestinal tract and occurs approximately in 4% to 28% of children worldwide. It is most common in children aged 5 to 14 years.The most commonly reported symptoms are pruritus in the perianal region, abdominal pain, urinary tract infection, insomnia, irritability, salpingitis, and appendicitis, whereas intestinal obstruction is a very rare but would be considered to perform the right instrumental examination avoiding unnecessary surgical exploration.We report a case of an 8-year-old boy with an intestinal occlusion due to a colonic intussusception by Enterobius vermicularis managed conservatively.

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. 

YouTube and pediatric surgery. What is the danger for parents?

Internet became one of the most important sources of public health informations especially for relatives and/or caregivers of sick children. Use of YouTube as a source of information in pediatric surgery has not been evaluated. In this study, we want to evaluate the use of YouTube as a source of information about one of the most frequent surgical urgency in pediatric patients, the acute appendicitis, to evaluate the risks for patients and parents.

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.

Analysis of readability and quality of web pages addressing both common and uncommon topics in pediatric surgery.

INTRODUCTION: The quality medical information on Internet is highly variable. The aim of this study is to determine if Web pages addressing four common pediatric surgical topics (CT) and four uncommon pediatric surgical topics (UT) differ significantly in terms of quality and/or characteristics. MATERIALS AND METHODS: We performed an Internet search regarding four CT, addressing more frequent clinical conditions with an incidence≤1:1.500 children (inguinal hernia, varicocele, umbilical hernia, and phimosis) and four UT addressing less frequent clinical conditions with an incidence≥1:1.500 children (anorectal malformation, intestinal atresia, gastroschisis, and omphalocele), using a popular search engine (Google). We evaluated readability with the Flesch reading ease (FRE) and the Flesch-Kincaid grade (FKG) and quality of content using the site checker of the HON Code of Conduct (HON code) for each website. RESULTS: In this study, 30/40 websites addressing CT versus 33/50 addressing UT responded to our criteria. No differences statistically significant in advertisements between the two groups were found (15 vs. 16%) (p>0.05). No differences were found in terms of time from last update, owner/author type, financial disclosure, accreditation, or advertising. CT had higher quality level according to the HON code (6.54±1.38 vs. 5.05±1.82) (p<0.05). Mean FRE was 47.38±14.27 versus 46.24±14.56, respectively, for CT and UT (p>0.05). The mean FKG was 8.1±1.9 for CT versus 8±1.9 for UT (p>0.05). CONCLUSIONS: Websites devoted to pediatric surgical topics have higher readability and quality information for disease diagnosis and natural history. Otherwise, the quality of pediatric surgical information on the Internet is high for CT and UT. A high reading level is required to use these resources.

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.

The impact of perinatal urinoma formation on renal function: our experience and review of the literature.

INTRODUCTION: A urinoma is a fluid mass consisting of extravasated urine in the perirenal space. Its impact on renal function was analysed. METHODS: All cases of prenatal and neonatal urinoma reported in the literature were analysed as well as two cases recently observed in our department. RESULTS: A review of all prenatally diagnosed urinomas revealed 25 cases, for a total of 30 renal units. The cause of obstruction was: lower urinary tract (LUT) obstruction in 16 cases (62.5%), upper urinary tract (UUT) obstruction in seven cases (29.5%) and unknown in two cases (8%). A preserved renal function was found in only 30% of all cases. Furthermore, prognosis for renal function was better in posterior urethral valve (PUV) patients than in uretero-pelvic junction obstruction (UPJO) patients. In addition, we analysed 35 published cases of urinoma with a neonatal onset, without prenatal diagnosis, and secondary to LUT obstruction in 27 cases (77%), UUT obstruction in seven cases (20%), while the underlying cause was unknown in one cases (3%). A preserved renal function was observed in 84% of cases with UUT obstruction and in 80% of cases presenting a LUT obstruction. CONCLUSIONS: Prognosis concerning renal function seems to be mainly related to two factors: age at presentation and underlying diagnosis. Decompression of the urinary tract by urine extravasation produces the best results in terms of preservation of the renal function in LUT obstruction (75% in prenatal and 84% in postnatal cases); UUT obstruction however is associated with a good prognosis in the neonatal period (80% of preserved renal function) but with a severe impairment (only 20% of preserved renal function) in prenatally detected cases.

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.