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.

Effectiveness of Articulating Linear Stapler for Total and Partial Laparoscopic Splenectomy in Children.

Purpose: We performed a retrospective study to determine the effectiveness and feasibility of articulating linear stapler in laparoscopic total splenectomy (LTS) and laparoscopic partial splenectomy (LPS), focusing on technical laparoscopic skills that could help pediatric surgeons to avoid intra- and postoperative complications. Methods: Retrospective cohort study of children younger than 18 years who underwent laparoscopic spleen surgery between January 2008 and March 2020. Age, sex, indication for surgery, operative time (OT), intra- and postoperative complications, and postoperative length of hospital stay (LHS) were analyzed. Data from parenchymal resection and vessels sealing techniques were obtained. Results: Thirty patients, 19 LTS and 11 LPS, were included. The mean age of the patients was 10.9 years, and 16 patients were male and 14 were female. For hematologic diseases, LTS was the elective surgery, associated with cholecystectomy in 5 cases. LPS was the common procedure for splenic cysts. The stapler was used in LTS to close the hilum vessels and in LPS for parenchymal resection. No statistically significant differences in OT were observed comparing LTS and LPS. Two conversions occurred in LTS; none in LPS. The mean LHS was 6 days in both groups. No recurrence or major complications appeared in both groups at 1-12 years of follow-up. In particular for LPS, there are no relapse of cyst neither reduction in splenic function. Conclusions: This study shows the effectiveness, feasibility, and safety of mechanic stapler in splenic surgery both for hilum vessels sealing and for parenchymal resection. The use of this device can reduce risk of hemorrhagic recurrences or major surgical complications improving the safety of the operation.

Visceral plexiform schwannoma: A case series.

Plexiform schwannoma (PS) is a benign tumour of the peripheral nerve sheath that is typically found in the skin. Fewer than 15 cases of visceral PS have been reported to date in both adults and children. We herein discuss a series of 3 patients (2 male and 1 female) with abdominal PS, aged 10-16 years (mean age, 12 years). All the patients had an acute presentation with abdominal pain, which was associated with rectal bleeding in 1 case and with walking difficulties in 1 case. Radiological investigations included abdominal magnetic resonance imaging (MRI) and computed tomography, along with neurofibromatosis screening (cerebral MRI and dermatological evaluation). Complete removal of the mass was possible in 2 of the patients (in 1 case by laparoscopically assisted surgery). Follow-up was uneventful. Abdominal PS is a rare occurrence. Due to its possible association with neurofibromatosis, the diagnosis of PS should prompt an investigation for other manifestations of this disorder.

The First Reported Pediatric Case of Primary Myoepithelial Carcinoma Involving the Whole Lung: Surgical Radical Treatment and Prosthesis Implant.

Primary myoepithelial carcinoma of the lung (PMC-L) arising from the bronchial glands in lower respiratory tract is exceedingly rare. Thus far, few cases in adults and only one in a pediatric patient have been recorded. To our knowledge, this is the first report of PMC-L successfully removed in a child, focusing on the importance of multidisciplinary primary surgery for the treatment of this tumor. A 7-year-old girl was admitted for persistent cough and fever; she was unresponsive to oral antibiotics. Chest radiography showed loss of volume of left lung sustained by almost total atelectasis. After routine clinical investigations, she was referred for computed tomography scan and magnetic resonance imaging that documented the presence of a mass occupying the entire left upper lobe, infiltrating the pulmonary hilum (main bronchus, pulmonary artery, superior pulmonary vein, and pericardium). After multidisciplinary evaluation, the histopathologic diagnosis of PMC-L was established using ultrasonography-guided transthoracic core needle biopsy and bronchoscopic biopsies. She was then subjected to left pneumonectomy under extracorporeal circulation and positioning of a thoracic expander filled with 200 mL of saline solution. The postoperative course was uneventful. With TREP (very Rare Tumor in Pediatric Age) consent radiotherapy was performed (61.2 Gy). At the 10-month follow-up, the patient was alive, breathing normally without any oxygen support, without recurrence of PMC-L or metastasis, and without any chest deformity. To our knowledge, this is the first case where a pediatric patient was successfully operated for PMC-L involving the whole lung. Extracorporeal circulation enabled us to perform radical primary surgery. Prosthesis implant not only maintained normal chest expansion but also allowed focused radiotherapy, thus enabling us to prevent damage to vital organs.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the esophageal atresia.

Esophageal Atresia (EA) is defined as an interruption in esophageal continuity that results in a proximal tract that ends in a blind pouch in 98% of cases, and a distal tract that in 87% of cases arises via a Fistula from the Trachea (TEF). (...).

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the ureteropelvic-junction obstruction.

The hydronephrosis, characterized by the dilation of the renal pelvicalyceal system with possible functional damage to the renal parenchyma, is the most common congenital abnormality of the urinary system detected in utero through the prenatal ultrasound screening. (...).

Peripheral neuroblastic tumor of the kidney: case report and review of literature.

INTRODUCTION: Peripheral neuroblastic tumors (PNTs) account for 8%-10% of all pediatric tumors. Adrenal glands and sympathetic ganglia are the commonest site of tumor growth. In the clinicopathologic spectrum of PNTs, neuroblastoma and ganglioneuroma are the most primitive and the most mature tumor form, while ganglioneuroblastoma represents an intermediate state of maturation. Surgical resection is the therapy of choice in localized disease, but can lead to serious complications when performed in the presence of certain imaging-defined risk factors. CASE PRESENTATION: We present a rare case of primary intrarenal ganglioneuroblastoma diagnosed in a teenager who underwent conservative surgery and, despite this, developed upper pole renal ischemia without loss of parenchymal function. CONCLUSION: We underline the complex management of these extremely rare cases of neuroblastic tumors, which require a dedicated multidisciplinary team.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the esophageal atresia.

Not available.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the ureteropelvic-junction obstruction.

Not available.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of pediatric nephrectomy and partial nephrectomy.

Not available.

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.

Pulmonary Malformations: Predictors of Neonatal Respiratory Distress and Early Surgery.

OBJECTIVES: The objective of our study is to retrospectively analyze a single-centre series of antenatally detected pulmonary malformations (PM) and to evaluate their postnatal outcome. MATERIALS AND METHODS: We retrospectively reviewed all prenatally diagnosed PM patients referred to our Centre in the period between January 1999 and December 2014. All cases were diagnosed by one of our Maternal-Fetal Specialists by US examination. Congenital pulmonary airway malformation (CPAM) volume ratio (CVR), development of fetal complications, need for fetal therapy, need for neonatal resuscitation and timing of surgery were analyzed. RESULTS: A total of 70 fetuses were diagnosed with a PM in the period of study. An initial CVR higher than 1.6 was found in 16/70 patients (22.8%); 14/16 developed fetal complications (p less than .0001). Fifty-six fetuses (80%) did not develop any complications during pregnancy. To all complicated cases a prenatal treatment was offered, carried out in 12 (1 termination, 1 refusal). Survival rate was 100%. Sixty-three fetuses (90%) were asymptomatic at birth and did not require any neonatal resuscitation. Six patients submitted to fetal therapy and one untreated presented with neonatal respiratory distress, required mechanical ventilation at birth and early surgery in the neonatal period (7/70, 10%). CONCLUSION: CVR > 1.6 and the presence of fetal complications can be considered as predictors of respiratory distress at birth and of the need for early surgery. Nevertheless, the vast majority of PM are asymptomatic at birth and only a small group of fetuses require prenatal and postnatal treatment and support.

Effectiveness and properties of the biological prosthesis Permacol™ in pediatric surgery: A large single center experience.

INTRODUCTION: The use of prosthetic patches of non-absorbable materials represents a valid tool in the treatment of abdominal wall and diaphragmatic defects in pediatric age. In recent years research has developed biological dermal scaffolds made from a sheet of acellular matrix that can provide the desired support and reduce the occurrence of complications from non-absorbable implant. We present our experience and a systematic review to evaluate the use of biologic prosthesis for abdominal wall closure in pediatric patients. METHODS: The study from January 2009 to January 2015 involved 20 patients treated with Permacol™ implant. We observed postoperative complications only in patients treated for abdominal wall closure, which is the major indication for the use of Permacol™. We conducted a systematic review and meta-analysis (according to PRISMA) on PubMed/Medline, Scopus and EMBASE regarding the use of biological prosthesis in pediatric population considering the incidence of complications as the primary outcome. RESULTS: 3/20 patients experienced complications: 2 patients with skin necrosis healed conservatively and 1 of them developed laparocele. Thus only 1 patient with incisional hernia had significant surgery complication. In patients who were permanently implanted with Permacol™ it has not determined adverse reactions with optimal functional outcome. CONCLUSIONS: In accordance with the few data (case reports and case series) reported in literature about pediatric patients, our experience in different pathologies and applications has shown the effectiveness of Permacol™, in particular for the non-occurrence of infections, that often affect the use of prosthesis.

Long-term results of laparoscopic treatment of esophageal achalasia in children: a multicentric survey.

BACKGROUND: This report describes three Italian centers' experience in the treatment of children with esophageal achalasia. PATIENTS AND METHODS: Between June 2000 and June 2012, 31 children (13 girls and 18 boys, with a median age of 8.4 years) affected by esophageal achalasia were treated in three different institutions with an esophagomyotomy according to Heller's procedure via laparoscopy associated with a Dor antireflux procedure. Between 2000 and 2005 (for 14 patients) we used mono- or bipolar coagulation to perform myotomy; after 2005 (for 17 patients) we used the new hemostatic devices to perform it. RESULTS: Median length of surgery was 120 minutes. Median hospital stay was 4 days. We recorded eight complications in our series: 3 patients (9.6%) had a mucosal perforation, and 5 children (16.1%) presented dysphagia after surgery. When comparing the data before and after 2005, it seems that the new hemostatic devices statistically shortened the length of surgery (P<.01, Student's t test). CONCLUSIONS: On the basis of our experience, laparoscopic Heller's myotomy associated with an antireflux procedure is a safe and effective method for the treatment of achalasia in the pediatric population. Intraoperative complications were <10%, and they occurred mostly at the beginning of our experience. Residual dysphagia occurred in about 16% of cases. The use of the new hemostatic devices seems to reduce the length of surgery and intraoperative bleeding. Considering the rarity of this pathology, we believe that patients with achalasia have to be treated only at centers with a strong experience in the treatment of this pathology.

Esophageal impedance/pH monitoring in pediatric patients: preliminary experience with 50 cases.

This paper describes multiple intraluminal impedance (MII) in 50 children with typical and atypical gastroesophageal reflux (GER) symptoms and discusses the possible clinical significance of objective numeric data provided by MII computed analysis. Patients underwent 24-hr pH/MII monitoring. Reflux parameters were analyzed with relation to age and reported symptoms. Nonacidic MII events occurred as frequently as acidic ones. A Pathologic Bolus Exposure Index associated with a normal pH Reflux Index was detected in 26% of our series. Significant correlations were found regarding acid and bolus clearing times and their ratio. We conclude that the low rate of symptom occurrence in the pediatric population represents a limit on MII evaluation. Our study confirmed that nonacid GER is at least as frequent as acid GER. As MII provides interesting objective data that could be used in clinical practice, we suggest further research to define normal ranges in the pediatric population.