Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

A new frontier in therapy personalisation based on in vitro studies to preserve fertility potential of men.

At present, there is still a lack of attention to male infertility and fertility impairment. Indeed, the pathologies affecting the reproductive area in man are derived from anatomical or functional alterations of neuroendocrine system; thus, the study of these dysfunctions is necessary for a correct aetiopathogenetic and therapeutic framing of infertile patients. In this article, we underline the importance of the study of the molecular mechanisms regulated by the most common therapy used to treat infertile men, with the aim to highlight the necessity to avoid the administration of the wrong posology or, even more important, the wrong therapy to the patient. Accordingly, we present some pioneer data obtained on primary testicular cells cultured in vitro and treated with human chorionic gonadotropin (hCG). These data pave the way on the possibility to preliminarily test the effectiveness of the therapy in vitro, in order to identify the responsiveness of patient-derived cells to the treatment and its effectiveness in each subject, in order to identify the correct dosage in a personalised way.

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.

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.

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.

Thromboses of the pampiniform plexi after subinguinal varicocelectomy.

PURPOSE: To present the Authors' experience with spermatic vein thrombosis after varicocelectomy. MATERIALS AND METHODS: The medical charts of patients treated for varicocele at the Authors' Institution between January 2008 and January 2013 were reviewed; inclusion and exclusion criteria were created. Data were analyzed focusing on the clinical diagnosis of spermatic vein thrombosis after varicocelectomy performed using two different techniques. RESULTS: After revision of the medical charts and in compliance with the inclusion criteria previously established, 188 patients underwent varicocelectomy: 112 with laparoscopic technique and 76 patients with subinguinal technique. A total of five cases of spermatic vein thrombosis (2.6%) were diagnosed between 6 and 12 days after surgery. All these patients had received the subinguinal technique (6.5%). All the patients were managed conservatively. DISCUSSION: Spermatic vein thrombosis after surgery is a rare complication but its onset should be considered as a possible event in patients with inguinal and scrotal pain. To manage this condition after diagnosis surgeons may opt for a surgical or clinical approach, either with drugs or local heat, rest and scrotal support.

Congenital fibrosarcoma of the bowel: sonographic description of a rare case of neonatal intestinal obstruction.

A case of localization in the bowel of congenital fibrosarcoma (a rare soft-tissue tumor usually occurring in the extremities) causing intestinal obstruction in a newborn girl is described. A focal thickening of the ileal wall with features mimicking intussusception was identified at sonography; the final diagnosis was achieved by means of molecular analysis after surgical removal of the mass.

Long time consequences after STARR procedure: Report of 15 years experience and medico-legal perspective.

INTRODUCTION: Stapled transanal rectal resection is the most surgical procedure used for obstructed defecation syndrome, rectal prolapse, rectocele and rectal intussusception worldwide. The aim of this study is to report our experience and long time consequences and to offer a new medico-legal perspective. MATERIALS AND METHODS: We retrospective review medical charts of patients treated between 2006 and 2021 â€‹b â€‹y the same team directed by the same senior surgeon. We consider major complications and long time sequelaeses as main object for the discussion. Inclusion and exclusion criteria were created. IRB approved the study. After revision a medico-legal perspective was done based on major complications. RESULTS: During the study period 1726 patients, ages between 18 and 71 years old, were treated with 1280 STARR procedures and 446 "Longo" [was stopped on 2012]; all procedures were performed by the senior surgeon and visited by the team at the same control visit at 7days, 30 days and 12 and 18 months after surgery. All patients had 100 â€‹% compliance at 30 days, while 85 â€‹% had long time visit (more than 18 months). During the study period 6 â€‹% (104 subjects) of patients had minor complications while 1 patient (42 â€‹yrs female) reported total fecal incontinence after 18 months (0,05 â€‹%). This patient had mental disorder treated with drugs unknown before surgery and long time mental disorder after surgery. We focused on this last case to discuss long time complication DISCUSSION: This survey reports some interesting clinical data; respect to standard complications minor complications such as pain, bleeding and anal discomfort represent less than 10 â€‹% of procedures that is a good results in this perineal surgery. For those working with rectal mucosal prolapse, obstructed defecation syndrome, rectocele or rectal intussusception is essential to distinguish these diagnosis to have a good counselling with patient before surgery (at least 1 month before). It is essential to check these patients with a close follow-up especially after surgery, to avoid any other mental discomfort related to fecal incontinence; long time fecal incontinence, without anatomical disorders as our case, could be associated and related to drugs consumption or mental disorder, or perineal insensitivity due to surgical procedure. In conclusion it is essential to have good clinical practice to suggest STARR procedure, having idea about different diseases, different surgical approaches and different long time complications.

Fertility Preservation in Pediatric Age: Future Perspective among Andrological Diseases.

Male infertility is a condition that has always been less studied and known than female infertility. Male infertility is increasingly present and increasingly diagnosed. Although several causes are known, to date about 40% of the causes are considered idiopathic. The worldwide denasality can only be slowed if awareness campaigns are implemented on all the diseases that can alter fertile potential, especially in young adolescents. Male infertility is, in addition, associated with several medical conditions. In particular, the association between infertility and testicular cancer, cardiovascular disease, autoimmune diseases, and genetic diseases is well known. For this reason, fertility preservation should not be proposed or be only oncological in nature, as there are several diagnosable pediatric pathologies that are associated with altered fertile potential to whose patients we could offer a gamete preservation pathway. In this paper we propose our experience on fertility preservation in pediatric andrological diseases.

Mitochondrial Dynamics as Potential Modulators of Hormonal Therapy Effectiveness in Males.

Worldwide the incidence of andrological diseases is rising every year and, together with it, also the interest in them is increasing due to their strict association with disorders of the reproductive system, including impairment of male fertility, alterations of male hormones production, and/or sexual function. Prevention and early diagnosis of andrological dysfunctions have long been neglected, with the consequent increase in the incidence and prevalence of diseases otherwise easy to prevent and treat if diagnosed early. In this review, we report the latest evidence of the effect of andrological alterations on fertility potential in both young and adult patients, with a focus on the link between gonadotropins' mechanism of action and mitochondria. Indeed, mitochondria are highly dynamic cellular organelles that undergo rapid morphological adaptations, conditioning a multitude of aspects, including their size, shape, number, transport, cellular distribution, and, consequently, their function. Since the first step of steroidogenesis takes place in these organelles, we consider that mitochondria dynamics might have a possible role in a plethora of signaling cascades, including testosterone production. In addition, we also hypothesize a central role of mitochondria fission boost on the decreased response to the commonly administrated hormonal therapy used to treat urological disease in pediatric and adolescent patients as well as infertile adults.

Single port surgery in pediatric age: report of first 300 cases.

BACKGROUND: In recent years, evolution of surgery has led to laparoscopy and then to single port surgery. In pediatric age, few papers have been published about single port procedures; in particular, no one has described the use of the Octoport device (Frankenman International Ltd., Suzhou, China). We present our experience using a new device. METHODS: A retrospective analysis of first 300 cases was performed collecting the data of all patients treated with Octoport device from October 2017 to September 2021. Epidemiological data, diagnosis, operative times, and complications were analyzed. Postoperative pain was compared with standard laparoscopy. RESULTS: A total of 300 procedures were performed during the study period. The age range was 1-17 years. The conversion rate was 3.6% (11 patients) including both conversion to traditional laparoscopy and to laparotomy. Pain management was comparable to traditional laparoscopy. The complication rate was 3.6%, in one case leading to re-do surgery. All the cases in our Unit were successfully completed, with complications mainly related to the original pathology rather than to the technique itself. CONCLUSIONS: The learning curve for Octoport use proved to be functional as for standard laparoscopy. In this study, surgical indications for the use of single port laparoscopy were defined, discerning favorable and unfavorable procedures. A proven superiority of this technique over traditional laparoscopy is yet to be defined, but Octoport has proved to be a safe and easy tool to reduce invasiveness of procedures in pediatric surgery with better cosmetic results.

Class III gynecomastia in pediatric age: a new modified surgical treatment.

BACKGROUND: Prepubertal gynecomastia is an extremely rare condition usually requiring surgical treatment, especially in case of class III gynecomastia. This study presents cases treated with a new surgical treatment. METHODS: From 2010 at our institution, gynecomastia is treated surgically using a modified technique of periareolar incision. Aesthetic results and possible complications of this technique are reported. RESULTS: Five patients were treated at the authors' unit for class III gynecomastia between January 2010 and December 2011. All patients were properly treated without the need of further surgery for skin resection. One patient developed seroma, treated with suction; one patient showed hematoma, treated with hot packs for 3 weeks. All patients and their parents were satisfied of the aesthetic results obtained. All patients resumed sport activities 4 weeks after surgery. It was not possible to observe intraoperative complications. CONCLUSION: The technique used meets safety and efficacy standards for the treatment of this type of pediatric gynecomastia. It also offers the best possible aesthetic results with minimum complications which can be treated without the need of further surgery.

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.

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.

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.

Quality of Life and Anorectal Malformations: A Single-Center Experience.

Purpose: The treatment and long term clinical outcomes of anorectal malformations (ARM) in children have always been the focus of pediatric surgeons. This study aimed at reporting our experience as far as long-term follow-up of ARM in children is concern. Methods: We enrolled patients treated between 1999 and 2019, and established selection criteria to choose appropriate subjects. A validated questionnaire was used to determine long-term quality of life outcomes. Results: Out of a total of 48 patients treated within the study period, 28 were enrolled in this study. Among the latter, more than 35% had at least one long-time complication, and more than 90% had a good lifestyle. Urinary and fecal continence was achieved in more than 95% of the patients using medical devices. Conclusion: This study aimed to bring up new concepts; taking into consideration all aspects of life in patients with ARM, from school life to sexuality, while evaluating fecal and urinary continence. This is essential for the improvement of the skills of the different specialists involved in the management of these patients, and for the implementation of strategies that can improve postoperative function. Most especially, it will also help improve communication between doctors to ensure an adequate transition of these children into adult life.

Pseudopapillary tumor in pediatric age: clinical and surgical management.

BACKGROUND: Solid pseudopapillary tumors of the pancreas are rare exocrine pancreatic tumors. Through a review of pediatric cases in a single Institution, we present the clinical and surgical management of this neoplasm. METHODS: We retrospectively reviewed the clinical charts of patients treated at our unit between 1995 and 2009 for SPT. Clinical and surgical management were analyzed and reported. RESULTS: During the study period 11 patients underwent surgery for pseudopapillary tumor. Five patients were treated with duodenum-preserving pancreatic head resection and six patients with splenopancreasectomy with a Roux-en-Y pancreatic jejunostomy. Patients did not show recurrence and are currently disease free. Blood tests, Ultrasound, Computed tomography and Magnetic Resonance Imaging were not useful to preoperatively identify the nature of the pancreatic masses. CONCLUSION: Solid pseudopapillary tumor is a rare condition that should be taken into account for the differential diagnosis of pancreatic masses in pediatric age. Due to its favourable prognosis, surgical removal should be planned and done following the intraoperative findings.