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 YouTube videos addressing thoracoscopic sympathectomy using the LAP-VEGaS guidelines.

Introduction: The study aims to evaluate the quality of videos addressing thoracoscopic sympathectomy on YouTube® using the LAParoscopic surgery Video Educational GuidelineS (LAP-VEGaS) criteria. Methods: YouTube was searched using the following keyword: "thoracoscopic sympathectomy" on August 22, 2021. The first 50 videos were analyzed and classified for baseline characteristics and conformity to the LAP-VEGaS checklist. Results: Duration ranged from 19 s to 22 min. The mean number of likes was 14.8 (range 0-80). The mean number of dislikes was 2.5 (range 0-14). The mean number of comments was 8.5 (range 0-67). Nineteen videos did not meet our criteria and were excluded. Regarding the remaining 31 videos, none contained all 16 points of the LAP-VEGaS essential checklist (mean 5.4 points, range 2-14 points), with almost all neglecting preoperative information and outcomes. The mean percentage of conformity was 37% (range 12%-93%). The most viewed videos were not associated with higher conformity to LAP-VEGaS criteria showing only 4/16 points (25%). Conclusions: The quality of videos addressing TS on YouTube®, based on the LAP-VEGaS checklist may be considered not acceptable. Experienced surgeons and surgeons in trainees should be aware of this when using it as a learning resource in their clinical practice.

Effectiveness of unilateral sequential video-assisted sympathetic chain blockage for primary palmar hyperidrosis in children and adolescents.

Introduction: Primary palmar hyperhidrosis (PPH) is a severely debilitating condition that can affect patients of any age. Thoracoscopic sympathectomy provides a definitive treatment for PPH. Aim of this study is to investigate the effectiveness of unilateral sequential video-assisted thoracic sympathetic chain clamping (VATSCC) by clips application in pediatric population. Methods: The surgical procedure was done in the semi-sitting position, under general anesthesia with orotracheal intubation. Mean operation time was 23 ± 6 min (range 12-45). Two 5 mm ports were inserted at the level of the middle axillary line in the second and fourth intercostal space respectively. The sympathetic chain was identified, and two clips were applied, the first one at the level of the third and the second one, at the level of the fourth rib. No chest tube was used. Resolution of symptoms, complications, recurrence rate, onset and duration of compensatory hyperhidrosis were analyzed. Results: From August 2017 to September 2021, 58 patients (male:female ratio 32:26), mean age 16.5 years (range 14-19), with PPH underwent unilateral sequential VATSCC by clips application, starting on the dominant hand. The contralateral side was operated 2 months after. All patients except one (transient pneumothorax) were discharged on the first post-op day. No immediate or late complications have been recorded. Mean follow-up was 32 months (range 6-41). All patients except one (1,7%), affected by Raynaud's disease, showed a complete resolution of the symptom. Seven patients (12%) developed transient moderate compensative hyperhidrosis (CH) that spontaneously disappeared in the postoperative period. Conclusions: Unilateral sequential thoracoscopic sympathetic chain clamping for PPH in pediatric patients is a safe and very effective procedure with a low complication rate and low incidence of postoperative CH that, in our experience, resolved spontaneously in the postoperative period, after the second surgery leading to an improvement in the quality of life.

Robotic Excision of the Vagina in a 46 XX DSD Male Patient. First Pediatric Report.

The Disorders of Sex Differentiation (DSD) represent a wide range of congenital anomalies of the genitalia. Surgical treatment of these cases may become a challenge. We present a case of a 16-year-old boy with 46 XX DSD, SRY negative, presented with persistent dribbling incontinence, recurrent UTI and perineal pain. Past medical history included right orchiectomy, laparoscopic excision of uterus, fallopian tubes and left streak gonad at another institution at the age of 2 years. The native vagina was left in place. VCUG confirmed the presence of the residual vagina (8 cm in maximum length), connected with the bulbar urethra. Robotic-assisted laparoscopy of the vagina was performed with satisfying short and long-term results.

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.

Subtotal functional sialoadenectomy vs four-duct ligation for the treatment of drooling in neurologically impaired children: Long-term follow-up.

OBJECTIVES: The best surgical option to treat drooling in neurodisabilities is still under debate. The aim of this study was to describe the technique of subtotal functional sialoadenectomy (SFS) (ie four-duct ligation (4-DL) together with bilateral sublingual gland excision) and its long-term outcomes, in comparison with 4-DL. DESIGN: Retrospective observational cohort study. SETTING: Unit of Pediatric Surgery of Bambino Gesù Children's Hospital (Rome). PARTICIPANTS: Seventy-five patients surgically treated for drooling between 2002 and 2012, with at least five years of follow-up, divided into two groups: 4-DL group (19 patients) underwent four-duct ligation, and SFS group (56 patients) underwent subtotal functional sialoadenectomy. MAIN OUTCOME MEASURES: Primary end points were the evaluation of drooling improvement after surgery (parameters: Drooling Severity and Frequency Scale, DSFS; no of bibs/day; no of shirts/day; no of pneumonia/year; use of antidrooling drugs) and the comparison between two different surgical techniques. RESULTS: Median age at surgery was 10 years (1-35). Long-term outcomes showed significant improvement in DSFS and in no of shirts/day in both groups. Significantly better results were found in the SFS group than in the 4-DL group as far as DSFS (P value .045), no of bibs/day (P value .041), no of shirts/day (P value .032) are concerned. Reoperation rate for recurrence was 42% in the 4-DL group and 0% in the SFS group (P value < .0001). Six patients (8%; 2 in the 4-DL group and 4 in the SFS group) experienced perioperative complications, while 4 patients (5%; 2 in the 4-DL group and 2 in the SFS group) recorded long-term complications, with no difference between groups neither need for surgical treatment. No surgery-related mortality was recorded. CONCLUSIONS: In our experience, subtotal functional sialoadenectomy ensured significantly greater long-term effects than four-duct ligation for drooling treatment in neurologically impaired child, with equal complication rate.

Totally implantable venous access devices in children with medical complexity: preliminary data from a tertiary care hospital.

INTRODUCTION: Children with special health-care needs are an emerging and consistent population. In a subset of children with medical complexity (CMC) a continuous access to the central vascular system is advisable to eliminate unnecessary pain and stress and to improve home management and palliative care. METHODS: The surgical registry of a tertiary hospital was checked in order to identify CMC who underwent totally implantable venous access device (VAD) placement. Medical records were reviewed. RESULTS: From October 2009 to August 2014, a totally implantable VAD was placed in 10 children. Seven out of 10 patients were affected by cerebral palsy while 3 presented a genetic syndrome. The median duration of the indwelling catheter was 31 months (range 5 to 77 months). Six catheters are still in place since the first placement. Infectious complications were observed in two patients, respectively, a Candida albicans and a Staphylococcus aureus colonization; in both cases the VAD was removed. In another two cases, removal was planned for reservoir dislodgment within the subcutaneous tissue. No other major complications were observed during the procedure and the follow-up period. Emergency admissions decreased from a median value of 0.4/month (range 0-1.5/month) to 0.2/month (range 0-0.6/month) after the VAD placement. CONCLUSIONS: A totally implanted VAD in CMC is safe and manageable. As expected, infection seems to be the major complication with no infection-related death. Malnutrition and musculoskeletal deformities, which are frequent comorbidities in CMC, should be considered to reduce the risk of dislodgment/migration.

The Obalon swallowable intragastric balloon in pediatric and adolescent morbid obesity.

Background and study aims Incidence of morbid obesity has grown dramatically in the last half century and this phenomenon affects with particular severity the pediatric population. Dietary restrictions and careful programs to improve lifestyle are often ineffective to manage this particular group of patients, due to poor compliance typical of the adolescence. The aim of this study was to evaluate the effectiveness of a new intragastric balloon for treatment of morbidly obese children. Patients and methods A new swallowable intragastric balloon (Obalon) has been used for the first time in 17 obese children in order to assess its safety and effectiveness in terms of reduction in excess weight. In 9 of 17 children a second balloon was placed 30 to 40 days after the first insertion. All devices were endoscopically removed after a mean time of 18 weeks. Results In the group of 16 patients who completed the study (1 patient still under treatment) mean weight decreased from 95.8 ±â€Š18.4 Kg to 83.6 ±â€Š27.1 (P < 0.05). Mean body mass index (BMI) decreased from 35.27± 5.89 (range 30.4 - 48) to 32.25 ±â€Š7.1 (range 23.5 - 45.7) (P > 0.05); mean excess weight, calculated according to Cole's curves for pediatric populations, decreased from 36.2 ±â€Š15.9 to 29.4 ±â€Š18.3 Kg (P = 0.14), with an %EWL of 20.1 ±â€Š9.8 (range 2.3 - 35.1). Waist circumference decreased from 109 ±â€Š12.3 cm to 99 ±â€Š10.5 cm (P < 0.05). Conclusions Obalon can be administered easily without complications, inducing an appreciable weight loss with a statistically significant reduction in BMI and an improvement in associated comorbidities.

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.