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.

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.

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.

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.

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.

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.

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.

Varicocele and varicocelectomy: Which news from the past?

To the Editor, In 1952, after many centuries, the varicocele was treated to resolve infertility. From Celsus to modern surgical techniques, over the centuries, many surgeons have proposed numerous treatment options, some very traumatic others more "physiological" [...].

Laparoscopic and open approach for inguinal hernia in pediatric age: report of 8 years.

BACKGROUND: Ongoing innovation in laparoscopy lead pediatric surgeons to consider a mini-invasive approach for inguinal hernia correction. We review cases and evolution of surgical technique in a pediatric center. METHODS: A retrospective study included patients that underwent inguinal hernia repair between 01/01/2011 and 30/06/2019. Surgical techniques are compared. Outcomes considered: surgery duration, intraoperative and postoperative complications. RESULTS: 664 patients were included. 187 patients underwent laparoscopy(group A), 477 underwent open surgery(group B). Throughout time from 2011 to 2019, there has been an increase in laparoscopy, accounting in 2019 for more than 60% of overall surgeries. In 151 patients of group A initial diagnosis was of monolateral hernia; in 25.8% contralateral side was corrected at the same time because of intraoperative finding of open internal inguinal ring. Surgery duration in group B is shorter than group A;difference loses significance in bilateral corrections. Complications : 2.9% short term: prematurity related as well as to duration in group B. 1.7% relapses, regardless of technique. 5.2% metachronous hernias, related with age and open surgery. 0.6% secondary criptorchidism, unrelated to technique. CONCLUSIONS: There is not an evident superiority of laparoscopy over open repair. Laparoscopy should be preferred in case of doubt about bilaterality and in case of emergency surgery.

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.

Varicocele in adolescents: new findings and certainties among volume, hormones and semen analysis. Open perspectives.

Varicocele and varicocelectomy, and the clinical indications to treat it in pediatric age are still under discussion. Through the study of a recent work on the association between varicocele, hormones, and semen analysis, we comment on new results related to the management of varicocele in pediatric age In this new study, it has been reported that there is a correlation between abnormal semen analysis and FSH value, an association between abnormal semen analysis and other hormonal values (i.e. inhibin B) and an association between abnormal semen analysis and testicular volume. If these data are confirmed it is possible to suggest new diagnostic and therapeutic strategies in the treatment of pediatric varicocele.

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.].

Gender specific data in patients with acute appendicitis: a single center perspective.

BACKGROUND: The influence of sex, socioeconomic status and other factors on outcomes from acute illness has been found recently with an increasing interest; acute appendicitis is the most frequent urgency in pediatric age. The aim of this study is to show any gender differences. MATERIALS AND METHODS: We retrospectively analysed the medical records of consecutive patients who underwent surgical exploration for acute appendicitis. Inclusion and exclusion criteria were created. Patient data, demographics, characteristics and outcomes were studied and evaluated on a gender perspective. RESULTS: After reviewing medical charts following the inclusion and exclusion criteria, 364 patients were studied; focusing on final outcomes, it was possible to add news from a gender perspective of appendicitis: gangrenous appendicitis was associated with younger female and older female were more likely to have false positive appendicitis; female had less post-operative pain respect to agematch male for all appendicitis and males had more symptoms respect to females especially for phlegmonous and gangrenous appendicitis. CONCLUSIONS: There is a gender difference in appendicitis; as for adults girls appear to be affected less, have fewer symptoms and have better postoperative results.