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.

Agreement between magnetic resonance imaging and computed tomography in the postnatal evaluation of congenital lung malformations: a pilot study.

OBJECTIVES: To compare postnatal magnetic resonance imaging (MRI) with the reference standard computed tomography (CT) in the identification of the key features for diagnosing different types of congenital lung malformation (CLM). METHODS: Respiratory-triggered T2-weighted single-shot turbo spin echo (ss-TSE), respiratory-triggered T1-weighted turbo field echo (TFE), balanced fast field echo (BFFE), and T2-weighted MultiVane sequences were performed at 1.5 T on 20 patients prospectively enrolled. Two independent radiologists examined the postnatal CT and MRI evaluating the presence of cysts, hyperinflation, solid component, abnormal arteries and/or venous drainage, and bronchocele. Diagnostic performance of MRI was calculated and the agreement between the findings was assessed using the McNemar-Bowker test. Interobserver agreement was measured with the kappa coefficient. RESULTS: CT reported five congenital pulmonary airway malformations (CPAMs), eight segmental bronchial atresias, five bronchopulmonary sequestrations (BPS), one congenital lobar overinflation, one bronchogenic cyst, and three hybrid lesions. MRI reported the correct diagnosis in 19/20 (95%) patients and the malformation was correctly classified in 22/23 cases (96%). MRI correctly identified all the key findings described on the CT except for the abnormal vascularization (85.7% sensitivity, 100% specificity, 100% PPV, 94.1% NPV, 95% accuracy for arterial vessels; 57.1% sensitivity, 100% specificity, 100% PPV, 84.2% NPV, 87% accuracy for venous drainage). CONCLUSIONS: MRI can represent an effective alternative to CT in the postnatal assessment of CLM. In order to further narrow the gap with CT, the use of contrast material and improvements in sequence design are needed to obtain detailed information on vascularization, which is essential for surgical planning. KEY POINTS: • Congenital lung malformations (CLMs) can be effectively studied by MRI avoiding radiation exposure. • Crucial features of CLM have similar appearance when comparing CT with MRI. • MRI performs very well in CLM except for aberrant vessel detection and characterization.

Antenatal Determinants of Postnatal Renal Function in Fetal Megacystis: A Systematic Review.

Introduction: To evaluate the clinical usefulness of demographic data, fetal imaging findings and urinary analytes were used for predicting poor postnatal renal function in children with congenital megacystis. Materials and methods: A systematic review was conducted in MEDLINE's electronic database from inception to December 2023 using various combinations of keywords such as "luto" [All Fields] OR "lower urinary tract obstruction" [All Fields] OR "urethral valves" [All Fields] OR "megacystis" [All Fields] OR "urethral atresia" [All Fields] OR "megalourethra" [All Fields] AND "prenatal ultrasound" [All Fields] OR "maternal ultrasound" [All Fields] OR "ob-stetric ultrasound" [All Fields] OR "anhydramnios" [All Fields] OR "oligohydramnios" [All Fields] OR "renal echogenicity" [All Fields] OR "biomarkers" [All Fields] OR "fetal urine" [All Fields] OR "amniotic fluid" [All Fields] OR "beta2 microglobulin" [All Fields] OR "osmolarity" [All Fields] OR "proteome" [All Fields] AND "outcomes" [All Fields] OR "prognosis" [All Fields] OR "staging" [All Fields] OR "prognostic factors" [All Fields] OR "predictors" [All Fields] OR "renal function" [All Fields] OR "kidney function" [All Fields] OR "renal failure" [All Fields]. Two reviewers independently selected the articles in which the accuracy of prenatal imaging findings and fetal urinary analytes were evaluated to predict postnatal renal function. Results: Out of the 727 articles analyzed, 20 met the selection criteria, including 1049 fetuses. Regarding fetal imaging findings, the predictive value of the amniotic fluid was investigated by 15 articles, the renal appearance by 11, bladder findings by 4, and ureteral dilatation by 2. The postnatal renal function showed a statistically significant relationship with the occurrence of oligo- or anhydramnion in four studies, with an abnormal echogenic/cystic renal cortical appearance in three studies. Single articles proved the statistical prognostic value of the amniotic fluid index, the renal parenchymal area, the apparent diffusion coefficient (ADC) measured on fetal diffusion-weighted MRI, and the lower urinary tract obstruction (LUTO) stage (based on bladder volume at referral and gestational age at the appearance of oligo- or anhydramnios). Regarding the predictive value of fetal urinary analytes, sodium and ß2-microglobulin were the two most common urinary analytes investigated (n = 10 articles), followed by calcium (n = 6), chloride (n = 5), urinary osmolarity (n = 4), and total protein (n = 3). Phosphorus, glucose, creatinine, and urea were analyzed by two articles, and ammonium, potassium, N-Acetyl-l3-D-glucosaminidase, and microalbumin were investigated by one article. The majority of the studies (n = 8) failed to prove the prognostic value of fetal urinary analytes. However, two studies showed that a favorable urinary biochemistry profile (made up of sodium < 100 mg/dL; calcium < 8 mg/dL; osmolality < 200 mOsm/L; ß2-microglobulin < 4 mg/L; total protein < 20 mg/dL) could predict good postnatal renal outcomes with statistical significance and urinary levels of ß2-microglobulin were significantly higher in fetuses that developed an impaired renal function in childhood (10.9 ± 5.0 mg/L vs. 1.3 ± 0.2 mg/L, p-value < 0.05). Conclusions: Several demographic data, fetal imaging parameters, and urinary analytes have been shown to play a role in reliably triaging fetuses with megacystis for the risk of adverse postnatal renal outcomes. We believe that this systematic review can help clinicians for counseling parents on the prognoses of their infants and identifying the selected cases eligible for antenatal intervention.

Minimal Access in Pediatric Surgery: An Overview on Progress towards Dedicated Instrument Developments and Anesthesiologic Advances to Enhance Safe Completion of Procedures.

Surgical techniques are evolving in Pediatric Surgery, especially in the area of minimal access surgery (MAS) where indications for applications are expanding. Miniaturization of instruments, using natural orifices, single incisions, or remotely controlled robot-assisted procedures, promises to increase the benefits of MAS procedures in pediatrics. Many pediatric pathologies are rare, and specialized surgical and anesthesiologic instruments are necessary to manage them, defined as "orphan devices", for which development and dissemination on the market are slowed down or sometimes hindered by regulatory standards and limiting financial conflicts of interest. In pediatric surgery, it is of utmost importance to work in a multidisciplinary way to offer a surgical path that is safe and supported by technological advances. For this reason, optimizing pediatric anesthesia is also a crucial factor where technological advances have made monitoring more precise, thereby enhancing safety in the operative room. The development of customized instruments and technologies should be supported by pediatric research and should be adapted to the individualities of the small patient. This overview outlines the importance of dedicated instruments developed for the safe completion of MAS procedures in pediatrics.

Efficacy of Three Different Injection Techniques for the Endoscopic Treatment of Vesicoureteral Reflux (VUR) in Children: A Review of 10 Years of Experience.

Aim: To review our 10 years of experience with the endoscopic treatment of vesicoureteral reflux (VUR) in children, emphasizing the long-term efficacy of the "combined STING-HIT" technique. Materials and Methods: A retrospective study was performed including all children with symptomatic VUR undergoing the cystoscopic injection of bulking agents from January 2013 to December 2022 in our pediatric tertiary referral center. Three different endoscopic techniques were adopted: the "combined STING-HIT" technique, the STING technique, and the HIT technique. Treatment success was defined as symptom remission and VUR resolution on the voiding cystourethrogram (VCUG) performed at the 3-month follow-up. Results: In the study period, 140 (F:M = 64:76) patients and 228 ureters were treated at a median patient age of 3 (2.0-6.0) years. After a single endoscopic treatment, VUR resolved in 203 (88%) ureters. The VUR resolution rate after a single endoscopic treatment was 95% (n=70/74) in case of I-II VUR, 88% (n=87/99) in case of III VUR; 83% (n=38/46) in case of IV VUR; 89% (n=8/9) in case of V VUR (p-value: 0.174). Overall, one or two endoscopic treatments succeeded in 219 (96%) ureters. The overall VUR resolution rate following one or two endoscopic treatments was 100% (74/74) in case of I-II VUR, 93% (n=92/99) in case of III VUR; 96% (n=44/46) in case of IV VUR; 100% (n=9/9) in case of V VUR (p-value: 0.083). Despite not being statistically significant, the VUR resolution rate was higher for the "combined STING-HIT" technique, both after one (92%: n=110/119; versus 85%; n=62/73 versus 86%; n=31/36; p-value: 0.225) or two (98%: n=116/119; versus 95%; n=69/73 versus 94%; n=34/36; p-value: 0.469) endoscopic treatments. Conclusion: The endoscopic approaches were highly successful for the treatment of VUR in children. The "combined STING-HIT" technique was a safe and effective procedure, being associated with the higher resolution rate.

Lung Function Evaluated By Structured Light Plethysmography in Children After Lung Surgery: A Preliminary Analysis.

Background: Structured light plethysmography (SLP) is a novel light-based method that captures chest wall movements to evaluate tidal breathing. Methods: Thirty-two children who underwent lung surgery were enrolled. Their clinical history was collected along with spirometry and SLP. Results: Median age of surgery was 9 months (interquartile range 4-30). Most frequent diagnosis was congenital pulmonary airway malformation (14/32), then pulmonary sequestration (9/32), tumor (5/32), and bronchogenic cyst (4/32). The most frequent surgical approach was lobectomy (59%), segmentectomy (38%), and complete resection (3%). More than 80% had surgery when younger than 3 years of age. Eight patients had short-term complications (pleural effusion was the most frequent), while long-term effects were reported in 15 patients (19% recurrent cough, 13% thoracic deformities, 13% airway infections, 9% wheezing, 6% reduced exercise tolerance, and 3% columnar deformities). Spirometry was normal in 9/22 patients. Nine patients had a restrictive pattern, while 4 showed a mild bronco-reactivity. Ten patients did not perform spirometry because of young age. SLP revealed the presence of obstructive pattern in 10% of patients (IE50 > 1.88) and showed a significant difference between the two hemithorax in 29% of patients. Discussion: SLP may be a new method to evaluate lung function, without collaboration and radiation exposure, in children who underwent lung resection, also in preschool age.

Validation of the Clavien-Madadi Classification for Unexpected Events in Pediatric Surgery: A Collaborative ERNICA Project.

BACKGROUND: The Clavien-Madadi classification is a novel instrument for the assessment and grading of unexpected events in pediatric surgery, based on the Clavien-Dindo classification. The system has been adjusted to better fit the pediatric population in a prospective single-center study. There is a need now to validate the Clavien-Madadi classification within an international expert network. METHODS: A pediatric surgical working group created 19 case scenarios with unexpected events in a multi-staged process. Those were circulated within the European Reference Network of Inherited and Congenital Anomalies (ERNICA) and surgeons were instructed to rate the scenarios according to the Clavien-Madadi vs. Clavien-Dindo classification. RESULTS: 59 surgeons from 12 European countries completed the questionnaire. Based on ratings of the case scenarios, the Clavien-Madadi classification showed significantly superior agreement rates of the respondents (85.9% vs 76.2%; p < 0.05) and was less frequently considered inaccurate for rating the pediatric population compared to Clavien-Dindo (2.1% vs 11.1%; p = 0.05). Fleiss' kappa analysis showed slightly higher strength of agreement using the Clavien-Madadi classification (0.74 vs 0.69). Additionally, intraclass correlation coefficient was slightly higher for the Clavien-Madadi compared to the Clavien-Dindo classification (ICCjust 0.93 vs 0.89; ICCunjust 0.93 vs 0.89). More pediatric surgeons preferred the Clavien-Madadi classification for the case scenarios (43.0% vs 11.8%; p = 0.002) and advantages of the Clavien-Madadi were confirmed by 81.4% of the surgeons. CONCLUSION: The Clavien-Madadi classification is an accurate and reliable instrument for the grading of unexpected events in pediatric surgery. We therefore recommend its application in clinical and academic pediatric surgical practice. LEVEL OF EVIDENCE: III.

Magnetic resonance urography and laparoscopy in paediatric urology: a case series.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment. This pictorial essay draws on our experience; it illustrates some specific MRU findings and highlights the usefulness of MRU for the diagnosis of upper urinary tract pathology in children. It also offers some examples of the potential additional diagnostic information provided by laparoscopy as well as its therapeutic role.

Familial Poland anomaly revisited.

Poland anomaly (PA) is a pectoral muscle hypoplasia/aplasia variably associated with ipsilateral thoracic (TA) and/or upper limb anomalies (ULA). PA is usually sporadic and sometimes familial, making recurrence risk an issue in genetic counseling. Multidisciplinary evaluation of 240 PA patients was carried out, including physical examination of patients and their parents in 190 PA (subjects of the study). Familial conditions were classified into three groups. Group1: true familial PA (F-PA): pectoral muscle defects with familial recurrence: 8(4.2%). Group2: familial Poland-like anomaly families (F-PLA): PA index case and ≥1 relative(s) showing normal pectoral muscles but ULA and/or TA common in PA: 16(8.4%). Group3: sporadic PA (S-PA): 166(87.4%). F-PA indicated a stronger male (87.5%) and left side (62.5%) prevalence, but fewer ULA (37.5%) compared to the other two groups. Maternal transmission (6/8) was more common in F-PA. Statistical significance was not reached due to the small number of F-PA and F-PLA. Karyotyping and array-comparative genomic hybridization were performed in 13 families. Three maternally inherited copy number variants were identified in three patients: 1p31.1 deletion, Xp11.22 duplication, and 16q23.1 duplication. Interestingly, the proband's mother carrying the 16q23.1 duplication displayed moderate breast and areola asymmetry, but normal pectoral muscles on ultrasound. Though there is no recent review discussing recurrence of PA, we reviewed 31 published PA families. On the basis of our study and previous reports, familial PA is not uncommon. Nonetheless, no information can be derived either regarding a molecular basis or clinical tools with which to identify cases with recurrence risk.

Video-Assisted Thoracoscopy for Vertebral Body Tethering of Juvenile and Adolescent Idiopathic Scoliosis: Tips and Tricks of Surgical Multidisciplinary Management.

VATS (video assisted thoracoscopic surgery) is routinely and successfully performed in minor and major complex thoracic procedures. This technique has been recently introduced for the treatment of severe forms of idiopathic scoliosis (IS) with the aim to repair the deformity, reduce morbidity and to prevent its progression in patients with skeletal immaturity. This study aims to present VATS in anterior vertebral body tethering (AVBT) approach to support the pediatric orthopedic surgeons during vertebral body fixation. Surgical and anesthesiologic tips and tricks are reported to assure a safe procedure. The study includes preadolescents with IS and a grade of scoliosis >40° that had a high probability of deterioration due to remaining growth (December 2018 to April 2021). Skeletal immaturity of enrolled patients was assessed by Sanders classification and Risser sign. Patients had a Risser score between 0 and 1 and a Sanders score >2 and <5. AVBT technique using VATS was performed by a senior pediatric surgeon assisting the pediatric orthopedic surgeon. Twenty-three patients have been submitted to VATS AVBT in the period of study (age range 9-14 years). The patients had a classified deformity Lenke 1A or B convex right and all types of curves were treated. In all patients, the vertebrae submitted to tethering surgery ranged from D5 to D12; mean curve correction was 43%. Three postoperative complications occurred: one late postoperative bleeding requiring a chest tube positioning on 12th postoperative day; one screw dislodged and needed to be removed; one child showed worsening of the scoliosis and needed a posterior arthrodesis. Initial results of VATS AVBT in growing patients with spinal deformities are encouraging. An appropriate selection of patients and a pediatric dedicated multidisciplinary surgical approach decrease intraoperative complications, time of operation and postoperative sequelae and guarantee an optimal outcome.

Congenital Esophageal Atresia Long-Term Follow-Up-The Pediatric Surgeon's Duty to Focus on Quality of Life.

Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon's management and needs to be performed in each phase of a child's development.

Congenital Lung Malformations: Clinical and Functional Respiratory Outcomes after Surgery.

Congenital lung malformations (CLMs) involve anomalies of the lungs and respiratory tree such as congenital pulmonary airway malformation (CPAM), pulmonary sequestration (PS), bronchogenic cysts, congenital lobar emphysema, and bronchial atresia (BA). Although symptomatic lesions require surgical resection, the appropriateness of surgery for patients with asymptomatic malformations is a matter of ongoing debate. Limited data are available concerning the long-term follow-up of affected subjects. In this study, we sought to evaluate the long-term clinical and functional respiratory outcomes in children with CLMs who underwent surgical resection. We carried out a retrospective analysis of 77 children with CLMs who underwent pulmonary resection with at least one year of follow-up. The most common diagnoses were CPAM (50.65%), hybrid lesions (25.97%), lobar emphysema (11.69%), and PS (5.19%). The most common surgical approaches were lobectomy (61.3%), segmentectomy (10.7%), and pneumonectomy (5.3%). Acute post-surgery complications occurred in 31.2% of children. In addition, 73.7% experienced long-term complications, and we found no correlation between the presence of these complications and the sex of the patients, their age at time of surgery, the type of surgery undergone, the presence of symptoms prior to intervention, or acute complications after surgery. Pulmonary function tests revealed FEV1 Z-scores of <−2 SDs in 16 patients, and we found a significant correlation between pneumonectomy and the development of lung function deficit (p = 0.031). In conclusion, clinical and functional respiratory complications may occur in children with CLMs who undergo surgical resection. Long-term monitoring is needed to improve the management of asymptomatic patients.

Utility of Functional Lumen Imaging Probe in Long-Term Follow-Up of Children with Esophageal Atresia: A Single-Center Retrospective Study.

Long term follow-up of patients with esophageal atresia (EA) may be hampered by esophageal dysmotility, which affects quality of life and might lead to late complications. The endoluminal functional lumen imaging probe (EndoFlip™ Medtronic, Crospon Inc.) is an innovative diagnostic tool that assesses esophageal distensibility. Our aim was to report the use of EndoFlip™ in an EA follow-up, in order to describe distensibility patterns and to determine its possible role for functional evaluation of patients. We retrospectively collected data of EA patients, with a minimum follow-up of 9 years, who required endoscopic evaluation and underwent EndoFlip™. An adaptation of the Medtronic EF-322 protocol was applied and distensibility data were compared to those reported by Pandolfino et al. Nine patients (median age 13 years) were included in the study. The median minimum distensibility was 2.58 mm2/mmHg. Signs of peristalsis were observed in three patients. In one case, the esophagogastric junction (EGJ) after Toupet fundoplication showed low distensibility. EGJ distensibility values of 2.58 mm2/mmHg (median) confirmed both good esophagogastric continence and compliance. Esophagitis and absent peristalsis were found in one patient together with partial stenosis of the fundoplication, confirming the importance of surgical adaptation. Esophageal body distensibility was higher than that of the EGJ. Considering the presence of symptoms, the EndoFlip™ results seem to correlate better with the clinical picture. EndoFlip™ use was safe and feasible in children. It allowed for the measurement of esophageal distensibility and diameter and the acquisition of indirect information on motility with clinical implications. The routine use of EndoFlip™ could be part of EA follow-up, although considerable research is needed to correlate Endoflip™ system measurements to EA patient outcomes.

Developing Virtual Reality Head Mounted Display (HMD) Set-Up for Thoracoscopic Surgery of Complex Congenital Lung MalFormations in Children.

Video assisted thoracoscopic surgery (VATS) has been adopted in pediatric age for the treatment of congenital lung malformations (CLM). The success of VATS in pediatrics largely depends on the surgeon's skill ability to understand the airways, vascular system and lung parenchyma anatomy in CLM. In the last years, virtual reality (VR) and 3-dimensional (3D) printing of organ models and VR head mounted display (HMD) technologies have been introduced for completion of preoperative planning in adult patients. To date no reports about the use of VR HMD technologies in a pediatric setting are available. The aim of this report is to introduce a VR HMD model in VATS procedure to improve the quality of care in children with CLM. VR HMD set-up for planning thoracoscopic surgery was performed in a series of pediatric patients with diagnosis of CLM. The preoperative VR HMD evaluation allowed a navigation into the malformation with the aim to explore, interact, and make the surgeon more confident and skilled to answer to the traps. A development of surgical simulations models and teaching program dedicated to education and training in pediatric VATS is suitable among the pediatric surgery community. Further studies should demonstrate all the benefits of such technology in pediatric patients submitted to VATS procedure.

Postoperative Osteopathic Manipulative Treatment in Children with Esophageal Atresia: Potential Benefits on the Anthropometric Parameters.

Esophageal atresia (EA) is a congenital malformation that affects the normal esophageal development. Surgical treatment, although restoring the integrity of the alimentary tract, may lead to long-term sequelae-like developmental abnormalities and musculoskeletal deformities. We evaluated the effects of osteopathic manipulative treatment (OMT) on the recovery of the range of the right upper limb movement and on the rise of the auxological parameters. A case series of five children affected by type C EA were described. Six OMT sessions were performed over a 4-month period. At each treatment, height, weight, body mass index (BMI) and range of motion (ROM) in elevation of the right upper limb were assessed. OMT was applied to improve scar, larynx, rib cage, and sternum mobility. An average change of 2.3 cm in height and an average increase of 8° in the ROM of the upper limb in the period of study were detected. Additionally, OMT could improve the anthropometric data and the mobility of the right upper limb of children surgically treated for EA. Further studies that evaluate the effectiveness of OMT in post surgical treatment of congenital malformations of the thorax can be considered in the future.

Epidemiological and Clinical Profile of Pediatric Burns in the COVID-19 Era: The Experience of a Reference Center.

Pediatric burns represent a significant public health problem. We analyzed the characteristics of pediatric burns in a reference center, in order to identify better strategies for prevention and care. Burn patients admitted to the pediatric departments of our hospital from January 2020 to June 2022 were retrospectively evaluated. Age, gender, the etiology of injuries, the total burn surface area (TBSA), the degree of burns, the length of hospital stay (LOS), concomitant SARS-CoV-2 infection, and burn surface microbial colonization were analyzed. Forty-seven patients were included in the analysis (M:F = 1:0.67). Most of the cases involved patients between 0 and 4 years of age (83%). Hot liquid burns accounted for 79% of cases, flame burns for 9%, thermal burns for 6%, scald burns for 4% and chemical burns for 2%. Mean TBSA was 14 ± 11%. A second-degree lesion was detected in 79% of patients and third-degree in 21%. Mean LOS was 17 days. No additional infection risks or major sequelae were reported in patients with SARS-CoV-2 infection. Fifteen different species of bacteria plus C. parapsilosis were isolated, while no anaerobic microorganisms were detected. In the light of our experience, we recommend a carefully planned and proactive management strategy, always multidisciplinary, to ensure the best care for the burned child.

Colonic Volvulus in Children: Surgical Management of a Challenging Condition.

Colonic volvulus (CV) is a rare but potentially life-threatening condition with unclear etiopathogenesis. To date, less than 80 pediatric cases have been described. Hirschsprung's disease (HD) is associated with CV in 17% of cases, representing a significant risk factor. Non-HD CV is an even more complex entity. The aim of this study is to describe a series of patients with CV to accentuate some peculiar aspects of this disease. We performed a retrospective study (period: 2012-2021) collecting information of patients with CV. Data analyzed included: demographics, medical history, presenting symptoms and radiological and surgical details. Eleven patients (12.5 ± 2.8 years; 7F/4M) had CV (eight sigmoid, two transverse colon, one total colon). Five patients had associated anomalies and three had HD. A two-step approach with volvulus endoscopic/radiological detorsion followed by intestinal resection was attempted in eight cases (one endoscopic approach failed). Three patients required surgery at admission. At follow-up, two patients developed recurrent intestinal obstruction, one of whom also had anastomotic stenosis. Colonic volvulus is a challenging condition that requires prompt patient care. A missed diagnosis could lead to severe complications. The evaluation of the patient should include a careful histological examination (searching for HD and alpha-actin deficiency), immunologic and metabolic screening, neurological tests and detection of chronic intestinal pseudo-obstruction (CIPO). Lifelong follow-up is mandatory for the early recognition and treatment of progressive diseases involving the proximal gastrointestinal tract.

Nephrectomy for multicystic dysplastic kidney and renal hypodysplasia in children: where do we stand?

OBJECTIVES: Little is reported in literature regarding correct management of benign lesions of the kidney. The aim of our study is to present a series of total and partial nephrectomies performed in the last 5 years and to discuss indications. MATERIALS AND METHODS: Patients with benign lesions who underwent nephrectomy and partial nephrectomy at our institution in the period 2003-2008 were retrospectively included in the study. Notes were carefully reviewed and demographic data, symptoms onset, preoperative diagnosis, investigations, medical and/or surgical treatment, postoperative complications and definitive histological reports were collected. RESULTS: Forty procedures were performed. Twelve patients were preoperatively diagnosed of having multicystic dysplastic kidney (MCDK), which was confirmed in 10, whereas the remaining 28 patients of having severe dysplasia or hypodysplasia. Thirty-four patients underwent total nephrectomy, six underwent partial nephrectomy. Histopathological analysis confirmed segmental or complete abnormalities of the involved kidney in all cases. No malignancies were detected. DISCUSSION: Our study confirmed the extremely low malignancy rate of MCDK and hypodysplastic kidneys. The 20% mismatch of pre- and post-operative diagnosis suggests a common aetiology and shared therapeutic strategies for MCDK and hypodysplasia. At present, there is no consensus regarding correct indications for nephrectomy in paediatric age. As nephrectomy seems not to provide any advantage over preservation, but surgical and anesthesiological risks, we should be prudent in preserving every asymptomatic poorly or non-functioning kidneys maintaining a strict follow-up. Randomised controlled studies on larger multicentric series are strongly warranted to define this topic.

The Challenges of a Children's Hospital during the COVID-19 Pandemic: The Pediatric Surgeon's Point of View.

During the coronavirus disease of 2019 (COVID-19) emergency, in the pediatric surgical setting, it has been essential to avoid and contain infections as well as to protect both the patients and the surgical team. During this emergency, procedures and workflow were adapted to provide the safest possible environment for both the surgical team and the patients. Pediatric surgical activities were reorganized during the COVID-19 pandemic at the "Vittore Buzzi" Children's Hospital, which is a pediatric/maternal hospital located in Milan (Lombardy Region), Italy. Resources were optimized in order to maintain high levels of care and quality of assistance. During the COVID-19 emergency, the pediatric surgical department at the "Vittore Buzzi" Children's Hospital became an acute care surgical service. For the reorganization of surgical activities, institutional protocols were adapted in order to preserve the pediatric-specific characteristics of our service; five crucial points were specifically addressed. The pediatric surgical procedures carried out during the initial two months of the Italian lockdown are also reported. Continuity of care was maintained for children affected by severe diseases, such as tumors and neurosurgical conditions, whose treatment could not be deferred. Telemedicine and telecommunication were adopted as quick-support modalities for pre- and post-operative care. This reorganization allowed us to preserve the "pediatric specificity" and all care-related procedures offered at this high-quality/high-volume surgical care referral center.

Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence).

BACKGROUND: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. MAIN BODY: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. CONCLUSION: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.