Hao-Fountain syndrome and genital disorders: report of a new possible association.

BACKGROUND: Hao-Fountain syndrome is a neurodevelopmental disorder characterized by global developmental delay, variably impaired intellectual development with significant speech delay and, in some males patients, it has been reported an association with hypogonadism. At present less than 50 cases are reported in literature. CASE PRESENTATION: We report a case of this rare syndrome in a young female with isolated tubal torsion; our patients had different hospitalizations without treatment but during the last episode we decide to perform an abdominal surgical explortion. This is the first case in Literature with a new USP7 mutation. CONCLUSIONS: This case opens new perspective in this rare syndrome and a review approach to isolated tubal torsion. These symptoms should be always well checked.

Testicular Torsion: Preliminary Results of In Vitro Cell Stimulation Using Chorionic Gonadotropin.

Testicular torsion is a pathology that occurs in young males generally before the age of 25. Despite surgery representing the only effective approach, there is still a need to identify a marker that can predict whether a preserved testicle will be functional. In addition, no therapeutic approach is currently considered in the post-operative phase. Through an approach based on the in vitro culture of a tissue strictly linked to the testicle, the gubernaculum, we defined the healthy state of the organ and the possible responsiveness to a therapy used in the andrology field, chorionic gonadotropin (hCG). Firstly, we optimized a protocol to obtain viable cells starting from a small piece of gubernacular tissue harvested during surgery with the aim to amplify cells in vitro. Intriguingly, only for a patient whose testicle had been removed during surgery due to an excessive necrotic area, gubernacular cells were not able to grow in culture. These data support the possibility of exploiting the gubernaculum to evaluate the healthy state of the testicle. Then, as we demonstrate that gubernacular cells express a luteinizing hormone receptor, to which hCG is specific, we analyzed the cellular response to hCG treatment on in vitro cultured cells derived from patients affected by testicular torsion. Our study opens the way for the possibility of evaluating testicle wellbeing after derotation through in vitro culture of a small piece of gubernaculum together with predicting the response to the treatment with hCG, which can have a positive effect on cell proliferation and vascularization.

Adolescent male genitalia dissatisfaction: a surgical perspective.

Genital dissatisfaction is well known in female and adults. Less is known about male adolescents and their genital satisfaction. The aim of this study was to investigate and report the role of surgery in male adolescents to improve the evaluation of their genitalia. We considered all patients treated for external genital pathology in the period of adolescence. Inclusion and exclusion criteria were created. Patients underwent an evaluation test before and after surgery. During the study period, 137 patients were treated, and at the end of the study, 98 cases were considered for analysis. The most frequent pathologies were webbed penis and penile curvature. A postoperative score improvement was noted and patients with concealed penis and webbed penis showed a better postoperative outcome. Overweight was considered an important factor associated with a worse preoperative score. Evaluation of the external genitalia is important in adolescents, and it is an understudied problem. Overweight may be associated with a worse evaluation of one's genital and should be clinically considered to avoid related social problems in adulthood. Therefore, cosmetic genital surgery should be considered even in male adolescents.

Twenty-year experience with macro-area school screening for andrological disease in paediatric age.

Varicocele, phimosis and undescended testes are the most frequent andrological diseases in paediatric age; varicocele and undescended testes are primary causes of male infertility and the interests of research about these conditions have changed in the last years. The aim of the study was to report our experience after 20 years of macro-area school screening between 2000 and 2020. Data about school screening were reviewed and analysed. Subjects aged between 11 and 14 years underwent andrological visit. During the study period, three main andrological screenings were performed into our macro-area. The distribution of cohorts was different among the screenings. Among andrological diseases, varicocele diagnosis increased especially in the last 10 years. Phimosis was diagnosed less respect the first screening (2000-2001), while at present there were no cases of undescended testes. Our experience reported some interesting data, especially for the higher incidence of varicocele detected on two consecutive school screening; our results demonstrate also the importance and the preventive role of andrological check-up also in paediatric age and adolescence, to reduce the incidence of those diseases affecting the fertility potential.

Hypertrophic Pyloric Stenosis: 10 Years' Experience with Standard Open and Laparoscopic Approach.

PURPOSE: Hypertrophic pyloric stenosis (HPS) is the most common cause of gastric obstruction in newborns. Extra-mucosal pyloromyotomy can be performed through a small laparotomy or laparoscopy. The aim of this study was to compare the two surgical techniques. We also analyzed the incidence of HPS in infants in the last 10 years in relation to the demographic trend of our province. METHODS: We analyzed all the cases of HPS treated at our Unit between January 2010 and December 2019. The data were obtained from operating systems. Data about the demographic trends, in particular, the number of births and the population residing in the province of Verona from 2010 to 2019, were also retrieved. RESULTS: During the study period, 60 patients were treated for HPS and met the inclusion criteria. Of these, 56 males and 4 females with an average age of 38±14 days at surgery were included. No differences were found in terms of the duration of surgery, post-operative complications, duration of hospitalization, and weight at the time of surgery. The only statistically significant data was the chlorine level in cases with and without post-operative vomiting (97±3.5 vs. 102±3.3 mmol/L, p<0.05). There was a lower incidence of HPS from 2014 to 2019; however, there was no significant evidence regarding the correlation between this and the reduced birth rate recorded in the province of Verona during the same period. CONCLUSION: Although laparoscopic pyloromyotomy is a highly complex procedure, it is a feasible alternative to the classic open technique.

Correction: Quarantine and Appendicitis: A Macro-Area Experience.

[This corrects the article on p. 75 in vol. 24, PMID: 33505896.].

Histology and immunohistochemical evaluation of phimotic prepuce: The role of steroid therapy.

Phimosis is one of the most frequent andrological diseases in paediatric age. Steroids are useful to treat phimosis. Through a retrospective study of histological and immunohistochemical analysis, we evaluated the effectiveness of topical steroid treatment in patients undergoing circumcision. Cases of patients treated for phimosis were selected during the two-year study period. All patients underwent circumcision and were divided into four groups: groups A (religiously circumcised patients), B (phimotic patients not undergoing steroid treatment), C (phimotic patients who do not respond to cortisone treatment) and D (hypospadic patients undergoing urethroplasty). An histological evaluation of the degree of fibrosis and an immunohistochemical evaluation of collagen IV and tenascin were carried out. Study results demonstrate that the grade of fibrosis is age-related. On histological and immunohistochemical evaluation, fibrosis was found to be lower in patients receiving steroids; higher degrees of fibrosis were found in older patients (p < .05). Different degrees of fibrosis have also been found in hypospadic patients. We can conclude that study results correlated with the clinical history of the patients. The success rate of medical therapy seems to be age-related.

Varicocele in paediatric age: Is the scientific community on the correct pathway?

Varicocele is the primary cause of male infertility and the interests of research about varicocele have changed in the last years. The aim of the study was to analyse how topics of interests about varicocele have changed in the last two decades. Literature about paediatric varicocele between 2000 and 2018 was reviewed, and the subjects of the articles were analysed, selecting the main and secondary topics of each article. A study of their prevalence over the years was performed. In the research, 625 articles were found but only 169 provided data in the paediatric age. The total percentage of exclusively paediatric works was 27.04%, being above average in Europe, North and South America. The main subject both for Europe and North America was surgical technique, while for Asia it was videolaparoscopy. The main issue in South America was fertility; Africa and Oceania do not have enough publications to make statistics. Paediatric articles account for less than one-third of overall articles. The first interest of research is surgical technique. In Europe, the topics seem to exclude fertility potential.

Quarantine and Appendicitis:A Macro-Area Experience / 대한소아소화기영양학회지

Purpose@#Appendicitis is the most frequent urgency in pediatric age; the aim of this study was to investigate the association of quarantine for severe acute respiratory syndrome coronavirus 2 pandemic and the incidence of pediatric appendicitis in a specific macro area. @*Methods@#We retrospectively analyzed the medical records of consecutive patients who underwent surgical exploration for acute appendicitis in the period March–April since 2014. This specific quarantine period was divided into two phases as indicated by National government. Patient data, demographics, characteristics and outcomes were studied and evaluated comparing patients treated during quarantine especially phase 1 vs. phase 2 (March–April 2020). @*Results@#After reviewing medical charts following the inclusion and exclusion criteria, 155 patients were studied; focusing on the final outcome, it is possible to show a decreased amount of appendicitis during phase 1 and a progressive increase during phase 2; respect to previous years, there was a statistical increase in severity of appendicitis during quarantine (gangrenous vs. phlegmonous appendicitis). @*Conclusion@#During this specific quarantine there was a reduction in appendicitis and a progressive increase during phase 2. These results offer new perspective among disease incidences during lockdown.

Quarantine and Appendicitis:A Macro-Area Experience / 대한소아소화기영양학회지

Purpose@#Appendicitis is the most frequent urgency in pediatric age; the aim of this study was to investigate the association of quarantine for severe acute respiratory syndrome coronavirus 2 pandemic and the incidence of pediatric appendicitis in a specific macro area. @*Methods@#We retrospectively analyzed the medical records of consecutive patients who underwent surgical exploration for acute appendicitis in the period March–April since 2014. This specific quarantine period was divided into two phases as indicated by National government. Patient data, demographics, characteristics and outcomes were studied and evaluated comparing patients treated during quarantine especially phase 1 vs. phase 2 (March–April 2020). @*Results@#After reviewing medical charts following the inclusion and exclusion criteria, 155 patients were studied; focusing on the final outcome, it is possible to show a decreased amount of appendicitis during phase 1 and a progressive increase during phase 2; respect to previous years, there was a statistical increase in severity of appendicitis during quarantine (gangrenous vs. phlegmonous appendicitis). @*Conclusion@#During this specific quarantine there was a reduction in appendicitis and a progressive increase during phase 2. These results offer new perspective among disease incidences during lockdown.

Post-operative use of human chorionic gonadotrophin (u-hCG) inpatients treated for intrabdominal unilateral undescended testes.

OBJECTIVE: To report our experience with post-operative use of human chorionic gonadotrophin to achieve higher testicular volume and function, respect to untreated patients. MATERIALS AND METHODS: A prospective study was done using subjects who underwent orchidopexy between Sptember 2010 and September 2016 for unilateral intrabdominal undescended testes. All patients were treated by the same surgeon with laparoscopic one-stage Fowler-Stephens technique. After surgery (2 weeks) those patient parents who accepted to use hormonal therapy, had to follow a 6 weeks scheme. Patients received subcutaneous 500 UI (Gonasi-HP) weekly. A follow-up was performed at the end of therapy and 6 months later. Testicular volume was measured at each visit by ultrasound and by sonoelastography and compared with the untreated ones. RESULTS: Forty-five patients were enrolled and treated with a mean age of 18.0±9.7 months. 32 patients received post-operative hormonal therapy. There were no cases of adverse effects nor droupout. All patients completed follow-up. There were no cases of testicular atrophy in both groups. At 6 months among treated patients 26 (81%) subjects achieved normal testicular size while the other had still smaller volume. Among untreated patients, 6 (46%) subject achieved normal testicular size (P < 0.05). CONCLUSION: Despite the role of hormonal therapy is still under discussion, especially for post-operative treatment, our results suggest that it is safe and useful to improve testicular volume and morphology; treated testes have also a good stiffness respect to untreated testes.

Semen analysis in patients treated for varicocele in pediatric age: are surgical outcomes enough to preserve the fertility potential?

Preserving the fertility potential of adolescent patients with varicocele is still the main outcome of pediatric and adolescent urologysts; the aim of this study is to assess the fertility potential of patients treated for varicocele with testicular hypotrophy in absence of post-operative complications, with complete ipsilateral testicular catch-up growth and normal hormonal values. METHODS: The Authors collected data from adolescents with varicocele aged between 10 and 16 years treated at their Institution between September 2010 and September 2015. Inclusion and exclusion criteria were created; all patients were followed clinically and with hormonal tests before and after surgery and then re-evaluated after semen analysis. Semen quality was correlated with hormonal status, surgical complications and testicular volume. RESULTS: 133 subjects without recurrences and with testicular catch-up growth were enrolled; at surgery 52 patients had grade III varicocele, 80 had grade II varicocele and 1 patient had grade I varicocele. Surgical complications at 18 months were 6 hydroceles (2 in grade III and 4 in grade II varicocele). Semen analysis was performed on 41 patients: 75% had a good semen quality, 9.7% fairly good semen quality and 14.6% poor semen quality. Considering grade III patients (12 subjects), 7 (58%) had good quality, 3 (25%) fairly good quality and the remaining 2 (16.6%) poor semen quality. In grade II patients, 23 (79.3%) had good quality, 4 (13.7%) fairly good quality and 2 (6.8%) had poor quality (P>0.05). There was no correlation between semen quality, hormonal status and pre-operative grade of varicocele and post-operative testicular volume. CONCLUSIONS: The results demonstrate that varicocelectomy alone in well-studied and fully treated pediatric patients does not wholly preserve their fertility potential.

National survey for bariatric procedures in adolescents: Long time follow-up.

INTRODUCTION: The role of bariatric surgery and its role in adolescent is still under discussion worldwide. The aim of this study is to report an Italian survey for bariatric procedures in adolescents and the outcome with a medium and long-term follow-up. MATERIALS AND METHODS: We retrospectively analyzed consecutive data added into the Italian register of the society for bariatric surgery(period 2000-2010). We evaluated all patients treated in a 10-year period with a mean follow-up of 3 years. Inclusion and exclusion criteria were created. All patients were aged between 13 and 18 years. We evaluated and compared clinical data. RESULTS: After reviewing medical charts, 173 patients were considered for the study; 85 patients were treated with adjustable gastric band (AGB), 47 with intragastric balloon (IB), 26 with sleeve gastrectomy (SG) and other 15 patients with malabsorptive techniques (MT). Among clinical data, there was a statistical difference in terms of %excess weight loss (%EWL) between techniques only after 1 year post-procedure; at 5 years, considering the percentage of patients studied, sleeve gastrectomy had the best %EWL respect to other non malabsorptive techniques (p<0.05); at 5 year more than 90% resolved their comorbidities especially hypertension, dyspnea, orthopedic problems and dyspnea. CONCLUSIONS: This study is the first reporting a national survey in adolescent; more than 80% of patients are followed until 5 years post-op but only few patients (less than 5%) until 10 years. Our results demonstrated that sleeve gastrectomy in adolescent is safe and had a better %EWL respect to other non-malabsorptive bariatric procedures. LEVEL OF EVIDENCE: level III.

Pediatric Ovarian Torsion and its Recurrence: A Multicenter Study.

STUDY OBJECTIVE: To report results of a retrospective multicentric Italian survey concerning the management of pediatric ovarian torsion (OT) and its recurrence. DESIGN: Multicenter retrospective cohort study. SETTING: Italian Units of Pediatric Surgery. PARTICIPANTS: Participants were female aged 1-14 years of age with surgically diagnosed OT between 2004 and 2014. INTERVENTIONS: Adnexal detorsion, adnexectomy, mass excision using laparoscopy or laparotomy. Different kinds of oophoropexy (OPY) for OT or recurrence, respectively. MAIN OUTCOME MEASURES: A total of 124 questionnaires were returned and analyzed to understand the current management of pediatric OT and its recurrence. The questionnaires concerned patient age, presence of menarche, OT site, presence and type of mass, performed procedure, OPY technique adopted, intra- and postoperative complications, recurrence and site, procedure performed for recurrence, OPY technique for recurrence, and 1 year follow-up of detorsed ovaries. RESULTS: Mean age at surgery was 9.79 ± 3.54 years. Performed procedures were open adnexectomy (52 of 125; 41.6%), laparoscopic adnexectomy (25 of 125; 20%), open detorsion (10 of 125; 8%), and laparoscopic detorsion (38 of 125; 30.4%). Recurrence occurred in 15 of 125 cases (12%) and resulted as significant (P = .012) if associated with a normal ovary at the first episode of torsion. Recurrence occurred only in 1 of 19 cases after OPY (5.2%). Ultrasonographic results of detorsed ovaries were not significant whether an OPY was performed or not (P = 1.00). CONCLUSION: Unfortunately, oophorectomy and open technique are still widely adopted even if not advised. Recurrence is not rare and the risk is greater in patients without ovarian masses. OPY does not adversely affect ultrasonographic results at 1 year. When possible OPY should be performed at the first episode of OT.

The Role of Sonoelastography in the Evaluation of Testes With Varicocele.

OBJECTIVE: To evaluate the role of elastosonography in the evaluation of testicular elasticity as a predictive sign of testicular damage in patients with varicocele. MATERIALS AND METHODS: Between December 2010 and December 2014, we evaluated patients with varicocele by sonoelastography (SE) of the testes. We created 3 groups: group A included patients with untreated varicocele; group B, patients treated with the same technique; and group C, healthy age-matched patients without varicocele. All patients underwent SE for the evaluation of testicular stiffness and results were graded from 1 to 3 following the color scale grading. RESULTS: During the study period, 36 boys (9-16 years old) with untreated varicocele, 47 treated patients, and 24 age-matched healthy subjects underwent control visit for varicocele and SE. All right testes of all groups were scored as 1, whereas testes with varicocele were stiffer than normal; all hypotrophies were scored as 3, whereas not all testes that were scored 3 were associated with testicular hypotrophy. There was a significant and statistical recovery rate of the testicular volume and the sonoelastographic score after surgery. CONCLUSION: Testes with varicocele are significantly stiffer than normal ones. All testes with testicular hypotrophy had grade 3 sonoelastographic scores, but not all patients with a grade 3 score have testicular hypotrophy or continuous spermatic vein reflux. Our results show that sonoelasography can play a significant role in the evaluation of testicular elasticity as a predictive sign of testicular damage.

Wandering spleen with a ten-time twisted vascular pedicle.

Torsion of a wandering spleen is a rare cause of acute abdomen in children, usually diagnosed with color-Doppler ultrasonography and enhanced computed tomography. We report a pediatric case of torsion of wandering spleen.

Hypospadias anatomy: Elastosonographic evaluation of the normal and hypospadic penis.

BACKGROUND: Hypospadias is one of the most common congenital anomalies in childhood. The aim of this study is to apply elastosonography on normal and hypospadic penis to verify the structural differences in tissues composition and stiffness. MATERIALS AND METHODS: We analyzed medical chart of patients treated at our Institution for hypospadias during the period December 2005 and December 2014 (group 1). Other two groups were enrolled for this study: group 2- patients with hypospadias waiting for surgery and group 3-patients without hypospadias. Inclusion and exclusion criteria were created; all patients underwent penile ultrasound and elastosonography. Elastographic index of elasticity was defined as soft, medium-hard or hard. We assigned the value 1 to soft tissue, 2 and 3 to medium-hard and hard respectively. RESULTS: During the study period 294 patients were treated for hypospadias. After reviewing medical chart 115 patients were considered for analysis (group 1). 22 patients were enrolled in group 2 and 38 patients were enrolled in group 3. Group 1: 7 proximal hypospadias, 29 penile hypospadias, 79 distal hypospadias. Patients with hypospadias had malformation also at corpus spongiosum and cavernosum respect to controls. Elastography showed a corpus spongiosum stiffness defined as medium-hard or hard in all cases of the pathologic group and soft in all the subjects of the control group (p < 0.05). CONCLUSIONS: Elastosonography showed how the hypospadia anatomy is deeply altered, even in an anatomical area far from meatal abnormality: corpus spongiosum in hypospadic penis seems to be globally stiffer and less elastic and cavernous corpora are less developed.

Ureteral rupture after blunt abdominal trauma in a child with unknown horseshoe kidney.

More than 90% of renal injuries in children result from blunt abdominal trauma. A 10-year-old female had a blunt abdominal trauma with macro-hematuria. The computed tomography scan revealed the presence of a horseshoe kidney and a 3rd grade renal lesion and contrast leakage from the right ureter. The ureteral rupture was confirmed by cystoscopy and ascendant pyelography and than a double J-stent was implanted. The stent was removed one month later. Non-surgical management has become the standard of care for both ureteral and renal lesions in children. Non-surgical treatment is a safe procedure for renal trauma with ureteral rupture in children.