While colostomy is the standard stoma in patients with anorectal malformations (ARM), indications for ileostomy remain poorly defined. Our aim was to evaluate indications and outcomes of ileostomy in patients with ARM. A retrospective study was conducted selecting patients from ARM-Net Consortium registry who received ileostomy between 2007 and 2024. Additionally, a literature review was conducted to contextualize findings. Among 2,994 patients, 21 (0.7

The purpose of this paper was to define the specturm of management for Parapneumonial Pleural Effusion/Pleural Empyema (PE/PPE) in children in Italy. We conducted an online survey, distributed by the SIVI committee to 54 Italian pediatric surgery centers. A total of 23/54 (43%) Italian pediatric surgery centers responded. All responders (100%) required an anteroposterior chest radiograph (CXR) as the first imaging approach to suspect PPE, and chest Ultrasound (US) was routinely used in 96% of centers. A preoperative CT scan was routinely performed in 70% of centers. An etiological diagnosis was obtained in more than 80% of patients in 13% of centers, between 40% and 80% of cases in 61% of centers and in less than 40% of patients in 26% of centers. Empirical antimicrobial therapy with 2 antibiotics is the most commonly used therapeutic scheme (78% of centers) and targeted antibiotic therapy was used in 82% of enrolled centers. The majority of centers (57%) approached advanced stage pleural empyema with pleural drain placement and fibrinolysis (PDF); 26% of centers preferred to execute upfront Video-Assisted Thoracoscopic Debridement (VATD), and in 13% of centers, both fibrinolysis and VATD were used. In all cases (100%), urokinase was the fibrinolytic agent of choice. Broncho-Pleural Fistula (BPF) was treated conservatively with prolonged Pleural Drainage (PD) and antibiotics in 82% of centers, while the remaining 18% proposed early surgical treatment. 82% of centers proposed prolonged antimicrobial therapy for the treatment of Lung Abscess (LA), while 18% of centers preferred to execute upfront ultrasound-guided or thoracoscopic positioning of a pig-tail drain. As expected, we observed a lack of homogeneity in the treatment between the different centers: most of these have a preference for fibrinolysis over the use of primary VATD, with urokinase being the only fibrinolytic agent used in all centers. It would be desirable to involve as many centers as possible for the drafting of shared national guidelines in the treatment of PPE in children in Italy.

To compare functional bowel outcomes between patients followed-up at two referral centers in a high-income country and a low/middle-income country after anorectal malformation repair (ARM). Cross-sectional study using Krickenbeck, Rintala and Baylor Continence Scale questionnaires. Patients below three years of age, with developmental delay, or incomplete questionnaires were excluded. Associated malformations, surgical notes, toilet training, treatment and toilet setting were considered. Results were analyzed according to age and type of ARM. 68 patients from Milan and 65 from Johannesburg were included. Johannesburg population was younger (mean age 10.7 ± 6.1 years old vs 7.1 ± 4.7, p < 0.001). Perineal fistula was the most common ARM type in Milan (32/68, 47

Background: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. Methods: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010–2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). Results: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day–18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. Conclusions: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

This study aimed to compare techniques and outcomes of robotic-assisted varicocelectomy (RAV) and laparoscopic varicocelectomy (LV). The medical records of 40 patients, who received RAV and LV over a 2-year period, were retrospectively analyzed. Palomo lymphatic-sparing varicocelectomy using ICG fluorescence was adopted in all cases. Three 5-mm trocars were placed in LV, whereas four ports, three 8-mm and one 5-mm, were placed in RAV. The spermatic vessels were ligated using clips in LV and ligatures in RAV. The two groups were compared regarding patient baseline and operative outcomes. All patients, with median age of 14 years (range 11–17), had left grade 3 varicocele according to Dubin–Amelar. All were symptomatic and 33/40 (82.5

PurposeProne cross-table lateral x-ray (CTLxR) and colostogram aid surgical planning for anorectal malformations (ARMs) without perineal fistulas. We suggest objective imaging tools to classify ARMs.MethodsThree observers prospectively evaluated CTLxR and colostograms of male ARM patients (2012-2022) without perineal fistulas. The level of the rectal pouch was estimated with pubococcygeal (PC) and ischiatic (I) lines. On CTLxR, we described the "pigeon sign", defined as the rectal pouch ending with a beak-like image, suspicious for a rectourinary fistula. ARM was defined as rectobulbar when the rectal pouch was below the I line, rectoprostatic when between PC and I lines, and rectovesical when above the PC line. Concordance was assessed with Fleiss' kappa. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the "pigeon sign" were calculated.ResultsThirteen patients were included in this study. The interobserver agreement on CTLxR was 69.2% (k = 0.54) on pouch ending, 84.6% (k = 0.69) on the "pigeon sign", and 76.9% (k = 0.69) on diagnosis; concordance between observers and intraoperative diagnosis was 66.6% (k = 0.56). The "pigeon sign" had 75% sensitivity, 100% specificity, 100% PPV, and 50% NPV. The interobserver agreement on colostograms was 84.6% (k = 0.77) on pouch ending and 89.7% (k = 0.86) on diagnosis; concordance between observers and intraoperative diagnosis was 92.3% (k = 0.90).ConclusionPC and I lines and the "pigeon sign" are useful tools in examining CTLxR and colostograms. Adequate CTLxR interpretation may modify surgical strategy.

Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion that usually remains asymptomatic during the fetal and neonatal period. However, it can occasionally cause prenatal cardiocirculatory failure and fetal hydrops, requiring a thoraco-amniotic shunt (TAS) placement. In other cases, it can also cause symptoms at birth (such as respiratory distress) and may require urgent surgical intervention. Thoracoscopic lobectomy for neonates is rarely reported. Here, we report a case of right macrocystic CPAM causing fetal hydrops at 27 weeks of gestation. The fetus was treated with a TAS placement that successfully resolved the hydrops. At 39 weeks of gestation, a male neonate was born (weight 2,850 g). The TAS spontaneously displaced during delivery, causing an open pneumothorax (PNX), initially treated with a drainage. His condition gradually worsened, requiring ventilatory support. Computed tomography (CT) scan showed different giant cysts in the context of the right lower lobe, left mediastinal shift, and compression of the rest of the lung. An urgent surgical management was required. A thoracoscopic right lower lobectomy was performed at 10 days of life (weight 2,840 g). The postoperative course was uneventful; the child remained totally asymptomatic and showed a good recovery. To the best of our knowledge, this is the first reported case of open iatrogenic PNX following TAS positioning and the second of neonatal thoracoscopic lobectomy in a newborn weighting less than 3 kg. The purpose of this report is to indicate that minimally invasive surgery is feasible, safe, and effective for the resection of CPAM, even in small newborns.

This study aimed to report a multicentric national experience about the outcomes of pediatric endoscopic pilonidal sinus treatment (PEPSiT). The medical records of all pediatric patients, aged up to 18 years, who underwent PEPSiT in the period 2019–2021, were retrospectively reviewed. Patients’ demographics, operative details, and post-operative outcomes were assessed. A total of 294 patients (182 boys), with median age of 14 years (range 10–18), receiving PEPSiT in the study period, were enrolled. Pilonidal sinus disease (PSD) was primary in 258 (87.8

Background: The management of asymptomatic congenital lung malformations (CLM) is debated, and pulmonary function of patients with CLM is seldom discussed. Short-term respiratory outcome in patients operated for CLM was assessed in order to better define surgical impact.Methods: A retrospective study on patients operated for CLM between 2012 and 2021 was performed. Respiratory function was assessed with tidal breath analysis (TBA) in spontaneous sleep within 2 years of life. Patients with comorbidities affecting pulmonary function were excluded. Three variables were studied: tPTEF/tE (time to peak tidal expiratory flow/total expiratory time), tV (tidal volume), RR (respiratory rate).Results were assessed as z-score (normal +/- 1.64) and expressed as median (range). Pre- and post-operative results were compared, as well as post-operative results of disease and surgery type subgroups. Results: Thirty-nine patients were included. Median pre- and post-operative tPTEF/tE were normal (pre: -0.32 (-2.12; +1.56); post: -0.18 (-1.62; +2.43)). Patients with extralobar sequestration had preoperative high median RR, which improved after sequestrectomy (pre: +1.99 (-2.49; +7.43); post -0.22 (-3.01; +1.18)). All groups had reduced pre-operative median tV, which restored after surgery (pre: -2.15 (-9.75; +0.91); post: -0.35 (-6.65; +3.14)). Patients undergoing lobectomy and patients with intralobar sequestration showed greater improvement. Median post-operative TBA results were normal for all sub-groups except for reduced tV in patients operated with thoracotomy (-2.27 (-6.12; +5.26)).Conclusions: Patients with clinically asymptomatic CLM can have pathologic pulmonary function tests, which improve after surgery. These results add to the elements in favor of an interventional approach. Level of evidence: III, Treatment study.

Follow-up of children born with esophageal atresia (EA) is mandatory due to high incidence of comorbidities. We evaluated endoscopic findings at follow-up of EA patients performed at our Centre according to ESPGHAN-NASPGHAN 2016 guidelines. A retrospective observational study was performed using data from January 2016 to January 2021. We included EA patients (age range: 1–18 years) who were offered a program of endoscopic and histological high gastrointestinal (GI) tract examinations as per ESPGHAN-NASPGHAN 2016 guidelines. Clinical, surgical, auxological, endoscopic, and histological data were reviewed; variables as polyhydramnios, EA type, surgical type, enteral feeding introduction age, growth data, and symptoms were correlated to endoscopic and histological findings. The population included 75 patients (47 males), with mean age of 5 ± 4 years. In 40/75 (53.3%) patients, we recorded oral feeding problems, and upper gastrointestinal or respiratory symptoms suspicious of gastroesophageal reflux. Eosinophilic esophagitis (EoE) incidence was 9/75 (12%), significantly higher than in general population (p < 0.0001), and 10/75 (13.3%) presented non-specific duodenal mucosal lesions. EoE represents a frequent comorbidity of EA, as previously known. EA is also burdened by high, never-described incidence of non-specific duodenal mucosal lesions. Embedding high GI tract biopsies in EA endoscopic follow-up should be mandatory from pediatric age.

Cerebral near infrared spectroscopy (NIRS) monitoring has been extensively applied in neonatology and in cardiac surgery, becoming a standard in many pediatric cardiac centers. However, compensatory physiological mechanisms favor cerebral perfusion to the detriment of peripheral tissue oxygenation. Therefore, simultaneous measurement of cerebral and somatic oxygen saturation has been advocated to ease the differential diagnosis between central and peripheral sources of hypoperfusion, which may go undetected by standard monitoring and not mirrored by cerebral NIRS alone. A clinical algorithm already exists in cardiac surgery, aimed to correct intraoperative cerebral oxygen desaturations. A similar algorithm still lacks in noncardiac pediatric surgery. The goal of this paper is to propose a clinical algorithm for the combined use of cerebral and somatic NIRS monitoring during anesthesia in the pediatric population undergoing noncardiac surgery. A panel of experienced pediatric anesthetists developed the algorithm that is based on the clinical experience and intraoperative observations. It aims to lessen the current variability in interpreting NIRS measurement. Multisite NIRS monitoring was achieved applying one pediatric sensor to the forehead for cerebral tissue perfusion reading and a second one to the decumbent lumbar region for recording somatic renal tissue perfusion. The algorithm describes a sequence of acts aimed to identify the putative cause of intraoperative organ tissue desaturation and suggests clinical interventions expected to restore adequate tissue perfusion. It is composed of two arms: the main arm includes patients with an observed decrease in cerebral perfusion (CrO2), the second one includes those with a stable CrSO2 with declining RrSO2. Described also are five clinical cases of infants and neonates in whom pathological alterations of organ perfusion were detected using intraoperative multisite NIRS monitoring, portrayed in the accompanying figures (Annex).

BACKGROUND:Short bowel syndrome is the most common cause of intestinal failure (IF) in infants. We aimed to evaluate growth, nutrition intakes, and predictors of weaning from parenteral nutrition (PN) of infants with IF. METHODS:Clinical parameters, nutrition intakes, body weight and length z-scores were compared monthly from the 1st to 12th and at 18 and 24 months among infants receiving PN and those weaned. Logistic regression analysis was conducted to explore the predictors of weaning. RESULTS:We included 23 infants (10/23 weaned). Median [range: minimum; maximum] birth weight and gestational age were 1620 [590; 3490] g and 31 [24; 39] weeks, respectively. All infants showed growth retardation with similar median delta weight z-score from birth to discharge: -1.48 [-1.92; -0.94] in not-weaned and -1.18 [-2.70; 0.31] in weaned infants (P = 0.833) and a subsequent regain after the discharge: 0.20 [-3.47; 3.25] and 0.84 [-0.03; 2.58], respectively (P = 0.518). No differences in length z-score were found. After the sixth month, infants weaned from PN received lower PN energy and protein intakes compared with those not-weaned. Infants weaned from PN showed lower PN dependency index (PNDI%) from 5 months onward (45% for weaned and 113% for not-weaned infants at 5 months: P < 0.001). The Belza score, a predictor of enteral autonomy computed at 6 months, is associated with being weaned from PN within 24 months (odds ratio: 1.906; P = 0.039). CONCLUSION:Infants weaned and not-weaned showed similar growth patterns. Our findings support the clinical relevance of Belza score and PNDI% as predictors of weaning from PN.

Purpose Evaluate whether parents have ever discussed sexuality with their children with anorectal malformations (ARM), which sexuality issues they think should be addressed and who, in their opinion, should address these issues. Methods Parents from the Italian Parents’ and Patients’ Association for Anorectal Malformation participated in meetings organized by the Association together with local Pediatric Surgical Units and were asked to fill in a questionnaire. Results 103 parents participated. Overall, 66% of parents had never talked about sex with their children. Children’s age was marginally correlated with occasions to talk about sexuality ( r = .202, p = 0.53) indicating that the older were the children, the more the parents talked about sexuality. The majority of parents reported that their children should have the possibility to talk about sex with them (72%), psychologists (57%), gynecologists/andrologists (47%), pediatric surgeons (33.5%), surgeons specialized in ARM (39.8%), friends (28%), nurses (24.7%) and teachers (20.4%), respectively. The most important topic they thought their children should address was handling serene sexuality, although the most common topic effectively discussed with them was the conception. Conclusion Psychologists, gynecologists/andrologists, and pediatric surgeons are seen as key resources for talking about sexuality. A great number of parents express the wish that their children had more opportunities to discuss sexual topics with pediatric surgeons.

Background:The coronavirus disease 2019 (COVID-19) time exacerbated some of the conditions already considered critical in pediatric health assistance before the pandemic. A new form of pediatric social abandonment has arisen leading to diagnostic delays in surgical disorders and a lack of support for the chronic ones. Health services were interrupted and ministerial appointments for pediatric surgical healthcare reprogramming were postponed. As a result, any determination to regulate the term "pediatric" specificity was lost. The aim is, while facing the critical issues exacerbated by the COVID-19 pandemic, to rebuild future perspectives of pediatric surgical care in Italy. MethodsEach Pediatric Society, including the Italian Society of Pediatric Surgery (SICP), was asked by the Italian Federation of Pediatric Associations and Scientific Societies to fill a questionnaire, including the following the main issues: evaluation of pre-pandemic criticalities, pediatric care during the pandemic and recovery, and current criticalities. The future care model of our specialty was analyzed in the second part of the questionnaire. ResultsChildren are seriously penalized both for surgical treatment as well as for the diagnostic component. In most centers, the pediatric surgical teams have been integrated with the adult ones and the specificity of training the pediatric operating nursing is in danger of survival. "Emotional" management of the child is not considered by the general management and the child has become again an adults patient of reduced size. ConclusionA new functional pediatric surgical model needs to be established in general hospitals, including activities for day surgery and outpatient surgery. To support the care of the fragile child, a national health plan for the pediatric surgery is required.

Extralobar bronchopulmonary sequestration (eBPS) is a congenital lung malformation characterized by non-functional lung tissue with no connection with the bronchial tree, receiving systemic blood supply and covered by its own visceral pleura. It is mostly asymptomatic, however it can rarely get complicated with infections and neoplastic degeneration. Its management is still debated: many authors suggest observation for asymptomatic cases and intervention only in case of symptoms, others advocate surgery even in asymptomatic patients. Pleuropulmonary blastoma (PPB) is a rare malignant sarcomatous neoplasm, representing the most common primary lung malignancy in children. Typically, PPB is associated with congenital pulmonary airway malformation (CPAM). eBPS is rarely associated with PPB. We report a case of a previously healthy three-year-old boy presented to our emergency room with massive hemothorax after a low-energy chest trauma. Imaging revealed active thoracic bleeding by an anomalous artery arising from the celiac tripod, and an irregular mass in the lower right hemithorax. After performing biopsy, pathologic examination revealed a PPB. The patient underwent four cycles of chemotherapy before surgery. The operative findings revealed a right supradiaphragmatic extralobar mass, with no communication with the bronchial tree, strictly adherent to the right middle lobe. After mass excision, histological analysis confirmed the diagnosis of PPB type 2. The patient underwent postoperative chemotherapy. No recurrence nor metastasis were noted during the current follow-up. DICER-1 research revealed biallelic mutation on the tumor and on the surrounding normal tissue. We reviewed the literature concerning PPB and BPS. Based on these data, we would recommend intervention even in case of asymptomatic eBPS.

Introduction Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. We present our experience and provide a literature review. Methods Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed. Results Five cases were treated at our center, all presenting with severe hydrothorax. Four met the criteria for fetal hydrops. Four cases underwent postnatal computed tomography (CT) scan: in one case, there was no evidence of persistent bronchopulmonary sequestration. The other three underwent thoracoscopic resection, in two, a viable sequestration was found. Including our series, 57 cases have been reported, with no mortality and a success rate of 94.7%. Mean gestational age (GA) at the procedure was 28 +/- 3.4 weeks and mean GA at birth and birth weight (BW) were 38.6 +/- 2.3 weeks and 3,276 +/- 519.8 g, respectively. In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration. Conclusion Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops. Authors believe that this procedure should aim to reverse fetal distress and allow pregnancy continuation, and it should not be considered a definitive treatment. The currently available data do not support changes of the common postnatal management.

Introduction: Thoracoscopy represents the most challenging area of pediatric minimally invasive surgery due to its technical difficulty. A standardized training program would be advisable. The aim of this study is to evaluate the results of our surgical training. Materials and Methods: A retrospective, single-center, cohort study was performed. The following four-step program was tested: (1) theoretical part; (2) experimental training; (3) training in centers of reference; (4) personal operative experience. Particular attention was focused on the choice of mentor. Times and modality of adherence to the program were evaluated. The effectiveness and safety of the training were evaluated according to the surgical results of esophageal atresia (EA/TEF) repair and resection of congenital lung malformations (CLM). The study was conducted from January 2014 to May 2020. Attending surgeons with previous experience in neonatal and pediatric laparoscopy were selected for the training program after being evaluated by the head of Department. Results: The training program was fully completed in 2 years. Twenty-four lobectomies, 9 sequestrectomies, 2 bronchogenic cyst resections and 20 EA/TEF repair were performed. Thoracoscopy was always feasible and effective, with no conversion. The operative times progressively decreased. Only three minor complications were recorded, all treated conservatively. Conclusions: A standardized training program is highly desirable to learn how to safely perform advanced pediatric thoracoscopy. The 4-steps design seems a valid educational option. The choice of the mentor is crucial. An experience-based profile for pediatric surgeons who may teach thoracoscopy is advisable.

Background: This study aimed to report a European multi-institutional experience about thoracoscopic management of children with congenital lung malformations. Methods: The records of 102 patients (49 girls and 53 boys) with median age at surgery of 1 year (range 6 months-1.5 years), who underwent thoracoscopic lobectomy in five European Pediatric Surgery units, were retrospectively collected. Indications for surgery included congenital pulmonary airway malformation (CPAM) (n = 47), intra- and extralobar pulmonary sequestration (n = 34), hybrid lesion (CPAM/intralobar sequestration) (n = 2), severe bronchiectasis (n = 9), congenital lobar emphysema (n = 8), and others (n = 2). The condition was asymptomatic in 77/102 (75.5%), whereas symptoms such as recurrent pneumonia and/or respiratory distress were present in 25/102 (24.5%). Results: Surgical procedures included 18 upper, 20 middle, and 64 lower lobe resections. No conversions to open were reported. A 3 mm sealing device and 5 mm stapler were adopted in the last 48/102 patients (47%). The median operative time was 92.2 minutes (range 74-141). The median operative time significantly decreased in patients in whom the vessel division and bronchial sealing were performed using sealing devices (75.5 minutes) compared with suture ligations (118.9 minutes) (P = .001). The median hospital stay was 3.7 days (range 2-6.2). Three/102 patients (2.9%) developed postoperative complications, including air leakage requiring pleural drainage (n = 1) (Clavien IIIb) and respiratory infection (n = 2) (Clavien II). A reoperation was required in one patient with residual pleuropulmonary blastoma (0.9%). All symptomatic patients reported resolution of symptoms postoperatively. Conclusions: Thoracoscopic lobectomy is a safe and effective procedure with excellent cosmetic outcome, in expert hands. Based upon our experience, we strongly recommend surgery in patients with congenital lung malformations by the first year of life, to reduce the risk of infection and make the procedure technically easier, despite the small patients' size. Surgeon's experience and use of miniaturized instruments and sealing devices remain key factors for successful outcome.

Data sharing is not applicable to this article as no new data were created or analyzed in this study.

We aim to debate the topic of early ultrasound guided percutaneous liver biopsy (US-PLB) in former preterm infants.