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.

Gallbladder Stones in Pediatric Age: An Emerging Problem: The Risk of Difficult Cholecystectomy and the Importance of a Preoperative Evaluation.

The need for cholecystectomy during pediatric age has significantly increased in the last two decades. As biliary pathology increases, the probability of complicated cholecystectomies increases too. The aim of this article is to analyze our experience with difficult laparoscopic pediatric cholecystectomy, focusing on the importance of an accurate pre-operative imaging study. We retrospectively analyzed all patients affected by cholelithiasis who underwent laparoscopic cholecystectomy at the Pediatric Surgery Department of San Camillo Forlanini hospital of Rome and Santa Maria alle Scotte University Hospital of Siena from 2017 to 2022. Demographic data, body mass index (BMI), recovery data, laboratory tests, imaging exams, surgical findings, post operative management and outcome were taken into account. Overall, 34 pediatric patients, with a mean age of 14.1 years (6-18 years) were included, with a mean BMI of 29. All patients underwent abdominal ultrasonography and a liver MRI with cholangiography (cMRI). We identified five cases as "difficult cholecystectomies". Two subtotal cholecystectomies were performed. Cholecystectomy in pediatric surgery can be difficult. The surgeon must be able to find alternative strategies to total cholecystectomy to avoid the risk of possible bile duct injury (BDI). Pre-operative imaging study trough ultrasound and especially cMRI is crucial to recognize possible difficulties and to plan the surgery.

Ovarian torsion in the pediatric population: predictive factors for ovarian-sparing surgery-an international retrospective multicenter study and a systematic review.

STUDY OBJECTIVE: Ovarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making. DESIGN: We conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010-2020). PARTICIPANTS: Patients aged 0-18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions. RESULTS: Ninety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary. CONCLUSIONS: Our results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.

Surgical management of ovarian teratomas in childhood: a multicentric study on 110 cases and a literature review.

AIM OF THE STUDY: To compare a multicentric surgical experience on ovarian teratomas in childhood with the current management trends. DESIGN: A retrospective multicentric pediatric ovarian teratomas surgically treated between January 2000 and August 2020 at four Italian institutions. PubMed database was used to search for Reviews and Systematic Reviews published between January 2010 and August 2020: 15 manuscripts reported 3633 ovarian neoplasms in pediatric age, 1219 (33,5%) of which were ovarian teratomas. RESULTS: A hundred-ten patients with a mean age at diagnosis of 11.8 years were enrolled. Mature cystic teratomas accounted for the 90% of the masses. At surgery, 78 were oophorectomies and 32 were ovary sparing surgeries. Laparoscopy occurred in 16.3% of the surgeries.As regarding the current management trends, the mean age at diagnosis was 11.9 years and 80.5% of the cases were represented by mature teratomas. Of 430 procedures, 331 were oophorectomies while 99 were ovary sparing surgeries and laparoscopy was performed in 23.8% of cases. CONCLUSIONS: Ovary-sparing surgery with laparoscopic approach is increasingly offered as standard treatment for benign masses that fit the criteria for mature teratomas, in the attempt to achieve the best compromise between the preservation of fertility and the prevention of recurrences. Awareness should be raised among pediatric surgeons to reduce unnecessary radical surgery.

Can contrast enhanced ultrasound (CEUS) be useful in the diagnosis of ovarian torsion in pediatric females? A preliminary monocentric experience.

PURPOSE: To describe contrast enhanced ultrasound (CEUS) characteristics of ovarian torsion in pediatric females, assessing the potential diagnostic advantages of method as well as its limitations. MATERIALS AND METHODS: A retrospective study design was used. Between January 2018 and December 2020 we analyzed all pediatric females who underwent explorative surgery with a suspected diagnosis of ovarian torsion, and who were previously evaluated by conventional ultrasound (US), color-Doppler ultrasound (CDUS) and CEUS. RESULTS: We examined twenty pediatric females with a median age of 12 years. US identified 9/20 ovarian complex masses against 13/20 by CEUS. At US abdominal free fluid was shown in 13/20 patients and in 18/20 cases with CEUS. In our case series the evaluation of contrast enhancement at CEUS in the detection of ovarian torsions revealed a sensitivity of 94.1%, a specificity of 100% and an overall accuracy of 95%. CONCLUSION: Although there is a known ultrasound semeiotics suggestive of ovarian torsion, it is not always possible to obtain a diagnosis of certainty with conventional US even with CDUS. The study reports that complementation with CEUS showed excellent agreement with surgery obtaining a diagnosis in almost all the pediatric females examined.

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

Relapsed papillary urothelial neoplasm of low malignant potential (PUNLMP) of the young age: a case report and a review of the literature.

BACKGROUND: Papillary Urothelial Neoplasm of Low Malignant Potential (PUNLMP) are exceptionally rare in the first decade of life (mostly if multifocal) and there is a lack of standardized recommendations for the pediatric age. CASE PRESENTATION: We describe the case of a 9-year-old boy with a diagnosis of PUNLMP, who underwent to cystoscopic lesion removal and later to endoscopic lesion removal and intra-bladder Mitomycin-c (MMC) instillations for relapsed disease. Follow-up investigations at five years showed disease negativity. CONCLUSIONS: Intra-bladder MMC instillation may allow obtaining the complete remission with bladder-sparing for paediatric patients with a high-risk relapsed PUNLMP.

The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association?

BACKGROUND: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia]. METHODS: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes. RESULTS: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean. CONCLUSIONS: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.

A rare case of accessory spleen torsion in a child diagnosed by ultrasound (US) and contrast-enhanced ultrasound (CEUS).

INTRODUCTION: Accessory spleen, also known as supernumerary spleen or splenunculum, is a congenital anomaly of the spleen due to a fusion defect during the embryogenesis. Usually it is detected casually during an ultrasound (US) examination of the abdomen and it is asymptomatic. CASE REPORT: RESULTS: We present a case of a 12 years old male patient, with 2-days history of left abdominal pain, without fever, gastrointestinal or genitourinary symptoms. The day before our observation, the patient had gone to another hospital, from where he had been discharged with medical analgesic therapy, without any benefit. Blood tests were normal, the Ultrasound abdominal examination showed normal aspect of abdominal organs, but the presence in the left side of a small round parenchymal structure surrounded by hyperechogenic mesenteric fat. We interpreted this image as an accessory spleen, complicated by torsion. As the torsion of accessory spleen is a quite rare occurrence, we carried out a contrast enhanced ultrasound (CEUS) to get more information. CEUS showed the absence of enhancement of the nodular formation, suggestive for a complete lack of vascularization; the spleen was normally enhanced. While the management in case of accessory spleen torsion is non-operative, in this case the patient underwent surgical exploration, due to the persistence of abdominal pain despite the medical therapy, with clinical signs of peritoneal reaction, mimicking an acute abdomen. Surgery confirmed the diagnosis of accessory spleen torsion. DISCUSSION AND CONCLUSIONS: In conclusion, US is the first diagnostic tool in pediatric abdominal pain and allows to direct the diagnosis; the use of CEUS helps to clarify the US reports, without leaving doubts about the parenchymal vascularization of the abdominal organ involved.

Multimodal treatment of pediatric patients with Askin's tumors: our experience.

BACKGROUND: We report our experience and outcomes about the management of Askin's tumors [AT], which are rare primitive neuroectodermal tumors (PNETs) that develop within the soft tissue of the thoracopulmonary region, typically in children and adolescents. METHODS: We retrospectively analyzed the charts of 9 patients affected by AT (aged 6-15 years), treated at the Paediatric Oncology Unit of Gemelli University Hospital in Rome between January 2001 and December 2016. RESULTS: All nine patients underwent to biopsy followed by neoadjuvant chemotherapy. At the end of the neoadjuvant chemotherapy, they underwent to surgical removal of the residual tumor. Five patients with positive tumor margins and/or necrosis< 90% received local radiotherapy. Two patients with metastasis received an intensified treatment, with the addition of high dose adjuvant chemotherapy followed by peripheral blood stem cells rescue. No statistically significant correlation was found between outcome and gender; the presence of any metastasis and the radiotherapy. The overall survival was 65.14 months (95% confidence interval [95%CI], 45.81-84.48), and the 5 years survival was 60%, at a median follow-up of 53.1 months. CONCLUSION: Our study confirms that a multimodal treatment with surgery, chemotherapy, and radiotherapy may increase the survival in AT pediatric patients.

Non-Coding RNAs and Endometrial Cancer.

Non-coding RNAs (ncRNAs) are involved in the regulation of cell metabolism and neoplastic transformation. Recent studies have tried to clarify the significance of these information carriers in the genesis and progression of various cancers and their use as biomarkers for the disease; possible targets for the inhibition of growth and invasion by the neoplastic cells have been suggested. The significance of ncRNAs in lung cancer, bladder cancer, kidney cancer, and melanoma has been amply investigated with important results. Recently, the role of long non-coding RNAs (lncRNAs) has also been included in cancer studies. Studies on the relation between endometrial cancer (EC) and ncRNAs, such as small ncRNAs or micro RNAs (miRNAs), transfer RNAs (tRNAs), ribosomal RNAs (rRNAs), antisense RNAs (asRNAs), small nuclear RNAs (snRNAs), Piwi-interacting RNAs (piRNAs), small nucleolar RNAs (snoRNAs), competing endogenous RNAs (ceRNAs), lncRNAs, and long intergenic ncRNAs (lincRNAs) have been published. The recent literature produced in the last three years was extracted from PubMed by two independent readers, which was then selected for the possible relation between ncRNAs, oncogenesis in general, and EC in particular.

Correction to: Multimodal treatment of pediatric patients with Askin's tumors: our experience.

[This corrects the article DOI: 10.1186/S12957-018-1434-2.].

Obstetric brachial plexus palsy: a population-based retrospective case-control study and medicolegal considerations.

AIMS: The aim of this study was to examine 24 cases of obstetric brachial plexus palsy (OBPP) in 41,002 deliveries occurred at San Camillo-Forlanini Hospital in Rome, during the period 2000-2012. MATERIALS AND METHODS: A population-based retrospective case-control study was designed and the database of the hospital was searched; for each case, maternal and fetal records were examined and some risk factors were evaluated. RESULTS: A statistically significant association between the 24 cases OBPP and the following risk factors: primiparity (p < .014), birth weight (p < .002), maternal age (p < .02), diabetes (p < .03) and shoulder dystocia (p < .003) was found, moreover all the OBPP cases were recorded only in vaginal deliveries. CONCLUSIONS: The absence of OBPP cases in cesarean deliveries highlighted in this study supports the option of proposing an elective cesarean in the presence of known risk factors after a full disclosure with the mother of risks and benefits in order to obtain a valid consent. Furthermore, when cases of OBPP occur, communication between the physician and the parents of newborns is crucial and it may represent a valid risk-management tool to reduce malpractice lawsuits.

A novel approach in the treatment of neonatal gastroschisis: a review of the literature and a single-center experience.

Gastroschisis is a congenital abdominal wall defect and its management remains an issue. We performed a review of the literature to summarize its evaluation, management and outcome and we describe a new type of surgical reduction performed in our center without anesthesia (GA), immediately after birth, in the delivery room. Between January 2002 and March 2013, we enrolled all live born infants with gastroschisis referred to the third-level Division of Obstetrics and Gynecology "San Camillo" of Rome. Two groups of infants were identified: group 1 in which gastroschis reduction was performed by the traditional technique and group 2 in which reduction was immediately performed after birth in the delivery room without GA. Twelve infants were enrolled in group 1, and seven infants in group 2. Statistical significance was observed between the groups regarding the hospital stay, for the duration of parenteral nutrition and full oral feeds (p = .004). Survival was similar between two groups. The reduction without GA performed immediately after birth in a delivery room encourages the relationship between the mother and her child and appears to be a safe and feasible technique in a selected group of patients with simple gastroschisis defect; for this reason, it could represent a valid alternative to traditional approach.

Role of Non-Coding RNAs in the Etiology of Bladder Cancer.

According to data of the International Agency for Research on Cancer and the World Health Organization (Cancer Incidence in Five Continents, GLOBOCAN, and the World Health Organization Mortality), bladder is among the top ten body locations of cancer globally, with the highest incidence rates reported in Southern and Western Europe, North America, Northern Africa and Western Asia. Males (M) are more vulnerable to this disease than females (F), despite ample frequency variations in different countries, with a M:F ratio of 4.1:1 for incidence and 3.6:1 for mortality, worldwide. For a long time, bladder cancer was genetically classified through mutations of two genes, fibroblast growth factor receptor 3 (FGFR3, for low-grade, non-invasive papillary tumors) and tumor protein P53 (TP53, for high-grade, muscle-invasive tumors). However, more recently scientists have shown that this disease is far more complex, since genes directly involved are more than 150; so far, it has been described that altered gene expression (up- or down-regulation) may be present for up to 500 coding sequences in low-grade and up to 2300 in high-grade tumors. Non-coding RNAs are essential to explain, at least partially, this ample dysregulation. In this review, we summarize the present knowledge about long and short non-coding RNAs that have been linked to bladder cancer etiology.

Labia minora hypertrophy: causes, impact on women's health, and treatment options.

INTRODUCTION AND HYPOTHESIS: We provide a review of the literature about the onset and development of hypertrophy of the labia minora, together with some expert opinions on the appropriateness of labiaplasty. METHODS: We searched PubMed and used popular search engines, with a greater emphasis on the physiology and hormone-mediated metabolism of these structures, and less emphasis on their surgical treatment. RESULTS: We describe major embryological, cytological, and biochemical features of this anatomical part and summarize the clinical aspects of its hypertrophy, evaluating types of discomfort reported by women and the medical treatments available. Also, based on what is known about the artificial elongation and spontaneous hypertrophy of the inner labia, we illustrate and discuss the main biological factors that may trigger this medical condition. There are not enough data identifying a clear inheritance of inner labia hypertrophy in the absence of other pathological conditions; instead, we found indirect evidence for an association with transient episodes of local inflammation either before birth or during puberty. We also analyze the role played by estrogen receptors and other factors with regard to the onset of this condition and highlight the importance of their timing in determining the size of women's labia minora. Remarkably, most cases of enlarged labia minora should be considered as outliers that are within the physiological range of size variation described for these structures. CONCLUSIONS: We generally advise against surgical treatment of labia minora, especially in young, pre-pubertal girls, unless specific medical conditions are also present and/or the psychological impact on the patient is deemed particularly negative.

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.

Wandering Spleen and Organoaxial Gastric Volvulus after Morgagni Hernia Repair: A Case Report and Review of the Literature.

Wandering spleen and gastric volvulus are two rare entities that have been described in association with congenital diaphragmatic hernia. The diagnosis is difficult and any delay can result in ischemia and necrosis of both organs. We present a case of a 13-year-old girl, previously operated on for anterior diaphragmatic hernia and intrathoracic gastric volvulus, that presented to our service for a subdiaphragmatic gastric volvulus recurrence associated with a wandering spleen. In this report we reviewed the literature, analyzing the clinical presentation, diagnostic assessment, and treatment options of both conditions, in particular in the case associated with diaphragmatic hernia.

Fetal intra-abdominal cysts: accuracy and predictive value of prenatal ultrasound.

OBJECTIVE: The finding of a fetal intra-abdominal cyst is relatively common and it can be due to a wide variety of clinical conditions. The aims of this study were to determine the accuracy of the prenatal ultrasound (US) in identifying the etiology of fetal intra-abdominal cysts and to describe the neonatal outcomes. METHODS: All cases of fetal intra-abdominal cystic lesion referred to our center between 2004 and 2012 were reviewed. Cysts of urinary system origin were excluded. Prenatal and postnatal data were collected. Our cohort was divided into subgroups according to the prenatal suspected origin of the lesion (ovarian, mesenteric, gastro-intestinal and other locations). For each subgroup, sensitivity, specificity, positive predictive value, false-positive rate and accuracy of fetal US were calculated. RESULTS: In total 47 fetuses (10/37 M/F) were identified. The mean gestational age at the time of diagnosis was 33 ± 4.9 weeks. Our cohort comprised of 25 ovarian cysts (10 simplex and 15 complex), 3 GI duplication, 6 mesenteric lesions, 4 meconium pseudocyst and 9 lesions of other origin. Surgery was performed in 38 cases (81%) at birth. The prenatally established diagnosis was exactly concordant with post-natal findings in 34 cases (72.3%). Sensitivity, specificity, positive predictive value and false-positive rate of US were 88.1%, 95.5%, 71% and 5.4%, respectively. Long-term outcome was good in almost 90% of the cases. CONCLUSIONS: The etiology of fetal intra-abdominal cysts can be prenatally diagnosed in about 70% cases. Despite the high risk of surgery at birth, the long-term outcome was good in most of the cases.

Antenatal ultrasonographic features associated with segmental small bowel dilatation: an unusual neonatal condition mimicking congenital small bowel obstruction.

Segmental dilatation of the intestine (SDI) is an unusual condition presenting during the neonatal period, with symptoms of obstruction. Late diagnosed cases are also reported. The clinical polymorphism, and the lack of specificity of radiological investigations, make diagnosis difficult. Prenatal detection of abdominal cystic lesions or bowel dilatation has occasionally been reported to be associated with SDI. We herein report two cases of SDI, with a prenatal ultrasonographic suspicion of intestinal abnormality. In both infants, a dilatation of the ileum was found at surgery, without any evident site of obstruction or abnormal histology. SDI must be taken into consideration when a prenatal alert of possible bowel obstruction is not followed by postnatal clinical signs.