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.

GERD surgery in non-neurologic patients: Modified Laparoscopic Hill-Snow Repair is a valid alternative to Nissen fundoplication. Results of a 20 years of follow-up.

Nowadays laparoscopic Nissen fundoplication represents the gold standard in surgical treatment of complicated Gastro-Esophageal-Reflux Disease (GERD), above all in cerebral palsy patients. In non-neurological patients without gastrostomy Nissen fundoplication can create some problems (gas bloat syndrome, dysphagia). Laparoscopic Hill-Snow repair is an established surgical alternative, but it is reported only in adult population. We describe our modification of Hill-Snow technique and our experience in a large series of non-neurological children in order to report its effectiveness and applicability in pediatric patients affected by complicated GERD. Between 2000 and 2022, 319 children underwent surgical correction of gastro-esophageal reflux at our Department. All were affected by complicated gastro-esophageal reflux unresponsive to PPI (Proton Pump Inhibitors). 251 underwent laparoscopic Nissen fundoplication; 68 non-neurological patients underwent laparoscopic Hill-Snow repair. Of these 68 children 48 were males (71%) and 20 females (29%); median age was 5years (3 months-11 years). Weight range was 4-37kg. 52 patients (76.5%) presented the following symptoms: retrosternal pain, dysphagia, regurgitation, coughing, failure to thrive, persisting reflux esophagitis. 16 (23.5%) had chronic respiratory problems (aspiration, apneic-spells, dysphagia, coughing, choking, gagging). For 8 (11.8%) symptoms were expression of chronic recurrent gastric volvulus. All underwent modified-laparoscopic-Hill-Snow repair. Contrast study showed sliding hiatal hernia in 55 patients (81%), while endoscopy demonstrated 16 cases of histologically severe esophagitis (23.5%) and 52 of mild esophagitis (76.5%). No intraoperative/postoperative complications were recorded. 60patients had a complete follow-up (range 1-20 years). 60/68 patients were evaluated with barium-swallow-study at 6-12 months; 40/68 patients with upper-gastrointestinal-endoscopy at 12months. No relapse was reported. 50 patients (73.7%) were symptom-free. 18 (26.3%) referred occasional epigastric pain, associated with vomit in 2 cases. 64 (94.1%) referred ability to vomit; 4 temporary difficulty to swallow (average 30 days). All patients reported being able to burp. 3(4.5%) presented episodes of gas-air-bloat during the first 2 months with spontaneous resolution. No case of dumping syndrome was recorded. This technique's modification yields excellent results in term of relapse and side effects at long-term follow-up. We reported the first and largest pediatric series in non-neurological children with encouraging results.

Pediatric Gastric Volvulus: Is Laparoscopic Hill-Snow-Modified Gastropexy the Effective Long-Term Minimally Invasive Solution?

Introduction: Gastric volvulus (GV) is a rare and life-threatening condition if not promptly diagnosed and treated. Extreme rotation can cause obstruction cutting off blood supply to stomach and distal organs, leading to ischemia and necrosis. It is a clinically significant cause of acute/recurrent abdominal pain and chronic vomiting in children. GV can be classified into the following: primary, secondary, organoaxial, mesenteroaxial, or combined, acute, or chronic. Materials and Methods: Six neonates (5 males-1 female) were admitted to our department for recurrent postprandial vomiting associated with paleness, hyporeactivity, transitory abdominal distension, failure to thrive, and respiratory infections (only-1). Median age: 6 months (range 2-9 months). Patients metabolic and neurological evaluations were normal. A barium-swallow-study (upper gastrointestinal [UGI]) allowed definitive diagnosis. Results: UGI and esophagogastroduodenoscopy (EGDS) showed a gastric abnormal dilatation (gastric fundus), hiatal hernia (HH) in 5 patients, a large/small curvature discrepancy in 3 patients. Four were chronic-organoaxial-GV, 2 chronic-mesenteroaxial-GV. All underwent Laparoscopic-Hill-Snow-gastropexy (LHSG) with HH correction. No intraoperative/postoperative complications were recorded. Refeeding started in first postoperative day. Average hospital stay: 6-days. At long-term follow-up, all patients were symptoms free (able to vomit/burp); 2 referred occasionally epigastric pain sometimes with vomit. At 10-12 years follow-up UGI and EGDS confirmed correct gastric position revealing gastroduedenitis. Discussion: GV is a rare clinical entity in children considering etiology and management with an important morbidity and mortality rate. Its diagnosis could be missed simply. Vomiting or HH on imaging studies should suggest GV regardless patient's stable appearance. Conclusions: Chronic GV can manifest with atypical chest, abdominal, and gastrointestinal symptoms. Early diagnosis and treatment reduce the risk of developing acute form. LHSG is a long-term effective and safe solution with a very low complication rate, and no symptoms correlated to Nissen procedure.

Retroperitoneoscopic Nephrectomy in Pediatric Patients.

Minimally invasive surgery (MIS) in pediatric population is becoming more and more popular among pediatric urologists and now it can be considered the best treatment for nephroureterectomy that is one of the most frequent urological procedures. The main indications to MIS ureteronephrectomy in children are congenital or acquired benign conditions such as nonfunctional and/or damaging kidney, renal dysplasia, and destroyed kidneys due to obstructive or refluxing uropathy. Since ever in open urological surgery, the lateral retroperitoneal approach represents the typical way to approach this kind of surgery. Although different surgical approaches can be chosen (i.e., posterior retroperitoneal, lateral retroperitoneal, laparoscopic transperitoneal, and robotic approach), the lateral retroperitoneal approach represents the typical way to perform this kind of surgery, also with minimally invasive techniques. In this article, we illustrate the technique of retroperitoneoscopic nephroureterectomy in infants and children, reporting our experience with this technique in the past 20 years.

Endoscopic balloon dilatation of primary obstructive megaureter: method standardization and predictive prognostic factors.

The management of congenital primary obstructive megaureter (POM) is usually conservative, especially during the first year of life. Endoscopic high-pressure balloon dilatation (EHPBD) is indicated when symptoms, increasing dilatation and progressive renal damage are recorded, particularly in children younger than one year of age. We identified and described endoscopic prognostic factors predicting the success or failure of endoscopic dilatation. Thirty-eight patients (33 M;5 F) with POM from 2005-2018 were included. Diagnosis was based on US distal ureter dilatation (>7 mm), obstructive MAG-3 diuretic renogram and absence of vesicoureteral reflux (cystography). 24 patients were under 1 year of age. All patients underwent cystoscopy and high-pressure balloon dilatation with 3,5 Fr dilating balloon, after ascending pyelography. Median follow-up was of 6.5 years. We identified characteristics with poor prognosis: stenotic punctiform ureteral ostium and/or ostium located in a bladder diverticulum (9 pts) and stenotic tract longer than 1 cm (5 pts). The patients with a stenotic tract shorter than 1 cm (18 pts) were divided into two groups: <5 mm (5 pts) and between 5 and 10 mm (13 pts) showed a good response to dilatation. Endoscopic evaluation of ureteral ostium with pneumatic dilatation when possible is a useful diagnostic and therapeutic solution for POM treatment, especially under one year of age. EHPBD is effective in short stenotic tracts (<5 mm). It may also be repeated with good results in intermediate stenotic sections (5 mm-1 cm). According to our preliminary results, the procedure is more effective if performed earlier (3-7 months of life). Greater cohort and longer follow-up are needed to verify the stability of these results.

The Use of Endoclips in Thoracoscopic Correction of Esophageal Atresia: Advantages or Complications?

Background: Thoracoscopic correction of esophageal atresia (EA) with tracheoesophageal fistula (TEF) has been increasingly widespread, but is still one of the most advanced pediatric surgical skills. This procedure has a challenging learning curve, and usually initially requires a longer operative time than the open approach; furthermore to perform this intervention, the surgeon must be very experienced in endocorporeal knotting. In our opinion, standardization of the technique and the application of "tricks" (including the use of titanium endoclips for TEF closure) to make surgical steps easier, faster, and safe would be useful to the surgeon and to the patient above all. Materials and Methods: We present our experience in thoracoscopic treatment of EA/TEF over the past 12 years; during this period, we have treated 32 neonates. We reviewed all patient clinical records evaluating demographics, surgical technique, postoperative period, and long-term follow-up. Results: Thirty-one patients were affected by type C EA (five presented with a long-gap defect); one by type E EA. Mean gestational age was 36 + 5 weeks (29-41). Mean weight at surgery was 2340 g (990-3715 g). Through a transpleural thoracoscopic approach, after Azygos vein division, TEF was closed by sutures (silk/polydioxanone [PDS]) in 4 patients while in the remaining 28 two 5 mm titanium endoclips were applied. Esophageal anastomosis was then performed with 8-12 interrupted 5/0 absorbable sutures. We had two intraoperative complications in endoclips application (migration and misplacement), which were immediately resolved. After a contrast study on sixth to seventh postoperative day (average eighth), in the absence of leakage, oral feeding was started and chest tube removed. Four to six weeks after surgery, patients underwent endoscopic evaluation. At a follow-up of 9 years, we had no postoperative complications due to endoclips: neither TEF recurrence, nor problems due to clips dislocation. Conclusions: Although the benefits of thoracoscopic correction of EA/TEF are still discussed, we agree with this part of recent literature that considers thoracoscopic approach as a feasible, safe, and advantageous alternative to the traditional open approach. We also want to emphasize that in our experience, TEF closure by titanium endoclips is fast, reducing operative time, and effective, with no reported long-term complications in our case series. Overall success rate after clips application is, in our series, 100%. The only limit we have found, above all for low-weight patients, is the diameter of the clip applier which needs a 5 mm access even if the surgeon uses 3 mm operative instruments.

Duodenal Atresia: Open versus MIS Repair-Analysis of Our Experience over the Last 12 Years.

Objective. Duodenal atresia (DA) routinely has been corrected by laparotomy and duodenoduodenostomy with excellent long-term results. We revisited the patients with DA treated in the last 12 years (2004-2016) comparing the open and the minimally invasive surgical (MIS) approach. Methods. We divided our cohort of patients into two groups. Group 1 included 10 patients with CDO (2004-09) treated with open procedure: 5, DA; 3, duodenal web; 2, extrinsic obstruction. Three presented with Down's syndrome while 3 presented with concomitant malformations. Group 2 included 8 patients (2009-16): 1, web; 5, DA; 2, extrinsic obstruction. Seven were treated by MIS; 1 was treated by Endoscopy. Three presented with Down's syndrome; 3 presented with concomitant malformations. Results. Average operating time was 120 minutes in Group 1 and 190 minutes in Group 2. In MIS Group the visualization was excellent. We recorded no intraoperative complications, conversions, or anastomotic leakage. Feedings started on 3-7 postoperative days. Follow-up showed no evidence of stricture or obstruction. In Group 1 feedings started within 10-22 days and we have 1 postoperative obstruction. Conclusions. Laparoscopic repair of DA is one of the most challenging procedures among pediatric laparoscopic procedures. These patients had a shorter length of hospitalization and more rapid advancement to full feeding compared to patients undergoing the open approach. Laparoscopic repair of DA could be the preferred technique, safe, and efficacious, in the hands of experienced surgeons.

Horseshoe kidney and uretero-pelvic-junction obstruction in a pediatric patient. Laparoscopic vascular hitch: A valid alternative to dismembered pyeloplasty?

Horseshoe kidney (HSK) is a congenital defect of the urinary tract that occurs in 0.25% of the general population. Laparoscopic Vascular Hitch (LVH) according to Hellstrom-Chapman represent an alternative approach in treatment of extrinsic hydronephrosis by crossing vessels (CV) in pediatric age. In our Department from 2006 to 2016, 36 children with extrinsic-Uretero-Pelvic-Junction (UPJ)-Obstruction (UPJO) underwent laparoscopic vessels transposition. Over the last 4years, we have treated three patients with extrinsic hydronephrosis in HSK; two males and one female respectively of 6, 7 and 8years. The side affected was the left in all patients; symptoms of onset: recurrent abdominal pain, vomiting with associated intermittent hydronephrosis at ultrasonography. The preoperative examinations performed were: ultrasound/Doppler scan, MAG3-renogram, functional-magnetic-resonance-urography (fMRU). Mean operative time was 120'; median hospital stay 3- days. Intraoperative diuretic-test (DT) confirmed an extrinsic-UPJO in all patients. No JJ-stents and drain were used and there were no perioperative complications. Clinical and ultrasound follow-up (18 months-4 years) show resolution of symptoms and decrease in hydronephrosis grade in all patients. Our series is the largest in pediatric population by a revision of the literature. We believe that LVH is feasible in patients with symptomatic hydronephrosis by CV in HSK. Intraoperative-DT and the correct selection of patients are crucial to the success of the technique. According to us, this procedure is appropriate in those cases where the UPJ-anatomy is disadvantageous to a resection/re-anastomosis between ureter and renal pelvis. Our initial results are encouraging, although long-term follow- up and a more significant patient sample are required.

Ureteropelvic junction obstruction in children by polar vessels. Is laparoscopic vascular hitching procedure a good solution? Single center experience on 35 consecutive patients.

PURPOSE: We report the results of laparoscopic vascular hitching (LVH) in a series of children with ureteropelvic junction obstruction (UPJO) owing to aberrant lower polar crossing vessels (CV). Our aim is to confirm if LVH associated with intraoperative diuretic test (DT) represents a good procedure to treat extrinsic-UPJO by CV. In order to confirm the relief of the obstruction we suggest performing an intraoperative DT. MATERIALS AND METHODS: In our department from 2006 to 2014, 120 patients were treated for both extrinsic and intrinsic-UPJO. 85 (30 females, 55 males) presented an intrinsic obstruction and underwent dismembered pyeloplasty (AHDP), 61 open, 16 laparoscopic, 8 retroperitoneoscopic. 35 (23 males, 12 females) were studied for a suspected extrinsic-UPJO: 30 were treated with LVH (modified Hellström vascular hitch). Intraoperative-DT was performed in all patients before and after vessel transpositions confirming the UPJO and eventual relief after the procedure. We included in the study only patients with suspicion of vascular extrinsic-UPJO. Average age at surgery was 7.5years. Symptoms of presentation were recurrent abdominal/flank pain and hematuria. All patients presented ultrasound (US) detection of hydronephrosis. Preoperative diagnostic studies include: US/doppler scan, MAG3 renogram, urography, functional magnetic resonance urography (fMRU) and CT scan. RESULTS: 28 out 35 patients had a correct preoperative diagnosis, and the remaining needed an intraoperative diagnosis confirmation. All 35 patients had an intraoperative-DT: 30 patients underwent LVH (positive-DT); 3 patients (negative-DT) underwent laparoscopic-AHDP for intrinsic-UPJO; two with positive-DT and nonobstructive CV, had no surgical treatment. Median operating time was 95min; mean hospital stay was 4days. At 12-84months follow-up 29 patients remained symptoms-free, one needed after two years a laparoscopic-AHDP. CONCLUSIONS: According our experience, LVH associated with intraoperative-DT may be considered a safe procedure to treat extrinsic-UPJO by CV in carefully selected patients. In particular, the very low incidence of relapse suggest that in suspicion of extrinsic-UPJO, performing intraoperative-DT after CV transposition allows to exclude intrinsic-UPJO confirming that the LVH-procedure has relieved the pelvic obstruction, precluding the need for AHDP.

Amnioexchange for fetuses with gastroschisis: is it effective?

BACKGROUND/PURPOSE: Amniotic fluid of fetuses with gastroschisis (GS) contains inflammatory mediators, gastrointestinal, and urinary waste products. Dilution and removal of such harmful substances have been advocated to prevent damage to the herniated intestine. We evaluated the effectiveness of serial amnioexchange procedures in 8 consecutive fetuses with GS. METHODS: Amnioexchange was performed bimonthly during the third trimester. Amniotic fluid collected before each procedure was tested for pH, osmolarity, urea, creatinine, cystatin-C, proteins, albumin, bilirubin, biliary salts, pancreatic amylase, serum amyloid A, C-reactive protein, alanine transaminase (ALT), alcaline phosphatase (ALP), gamma-glutamyl transpetidase (gammaGT), tumor necrosis factor alpha, interleukin 2, interleukin 6, epidermal growth factor, transforming growth factor beta, and myeloperoxidase. RESULTS: A total of 25 samples (median, 3 per fetus) were examined. Biochemical or inflammatory markers did not correlate with gestational age, nor was any trend observed in values from individual patients during the course of amnioexchange treatment. There was no correlation between biochemical or inflammatory markers and clinical outcome, including time to full enteral feeding. CONCLUSIONS: Serial amnioexchanges did not modify the biochemical or inflammatory status of amniotic fluid nor appeared to prevent injury to the herniated gut. Because repeated amnioexchanges may carry some risks, their use in fetuses with GS is not recommended outside the setting of a prospective randomized trial.

Role of platelet activation in catheter-induced vascular wall injury.

PURPOSE: To investigate the role of smooth muscle cell (SMC) response and platelet activation in peripheral venous catheterization using a model of catheter injury associated with thrombocytopenic treatment. METHODS: Silicon elastic catheters were inserted into New Zealand White rabbit external jugular veins from 24 hours to 60 days. Immunocytochemical procedures with antibodies to differentiation markers specific for SMCs, myofibroblasts, and endothelial cells were used to ascertain the phenotypic features of injured venous SMCs and the tissue sleeve formed around the catheter. Thrombocytopenia was induced in rabbits by busulfan treatment and the effect on catheter injury development examined after 15 days. The putative direct effect of this drug on the venous SMC proliferation, migration, and differentiation was assayed in vitro for 48 hours. RESULTS: Catheter injury is characterized by the progressive formation of (1) a neointima, containing differentiating SMCs, which are derived from the media and adventitial layer, and (2) by the organizing thrombus formed around the catheter, which contains myofibroblasts. In busulfan-treated thrombocytopenic animals, there was no evidence for either neointimal development or thrombus formation. A direct role of this drug in the unresponsiveness of vascular wall can be excluded by the unchanged proliferation and migration pattern of cultured venous SMCs treated with busulfan compared to control cultures. CONCLUSIONS: In our model, accumulation of differentiated SMCs in the neointima and myofibroblast appearance in the thrombus are linked, although distinct, events regulated by platelet activation, which is able to furnish the appropriate microenvironment for vascular SMC recruitment from the media/adventitial layer.