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.

Pediatric endoscopic pilonidal sinus treatment (PEPSiT): report of a multicentric national study on 294 patients.

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%) and recurrent in 36 (12.2%). The median operative time was 36 min (range 11-120). The median VAS pain score was 0.86 (range 0-3) and the median duration of analgesic use was 27 h (range 12-60). The overall success rate was 95.2% (280/294) and the median time to full healing was 23.4 days (range 19-50). Six/294 (2.0%) patients developed Clavien 2 post-operative complications. The recurrence rate was 4.8% (14/294) and all recurrences were re-operated using PEPSiT. Redo-surgery for wound debridement was performed in one (0.3%) patient with late healing. On multivariate analysis, hirsutism and typology of sinus (pits ≥ 2, paramedian and more proximal to the anus) were predictors of PSD recurrence (p = 0.001). To date, this is the largest series of PEPSiT published in the pediatric population. The outcomes reported after a 3 years experience confirm that PEPSiT is a safe, effective, and real minimally invasive procedure to treat adolescents with PSD. It provides patients quick and painless recovery, satisfactory success, and high quality of life.

Uretero-pelvic junction obstruction in children: Is vascular hitch an effective and safe solutions in very long term outcome? Report of 25 years follow-up.

Vascular (VH) according to Hellstrom-Chapman technique is considered a safe and effective alternative approach to pure extrinsic Ureteropelvic Junction Obstruction (UPJO) with good results in short and medium term, but few data are available on long and verylong term outcomes. Our aim is to evaluate VH long and very-long term outcomes in patients treated in pediatric age focusing on relapse, development of hypertension and/or inferior polar kidney hypotrophy during puberty and adulthood. From 1990 to 2015 in our Department 76 children were treated by open or laparoscopic VH for pure extrinsic-UPJO. We were able to contact 54 of 76. 41 patients (25 males, 16 females) accepted to be studied. Mean follow- up time was 12.7 years (range 6-27 years); mean age at the assessment was 22.2 years. We excluded patients who were younger than 13 (if females) or 14 (if males) at the assessment (upper limits of physiological puberty onset). Patients were followed with US, MAG-3-scan and arterial blood pressure measurement. Collected data were compared with the preoperative ones by Student t-test. 95% of US images and MAG-3-scan reports were compatible with complete resolution of obstruction with good renal functionality. 87% of patients were completely healthy. We recorded 3 cases of hypertension (7%) not secondary to renovascular origin; 2 cases with recurrent flank pain (5%) with slightly dilated pelvis at the US and sub-obstructive pattern at MAG-3-scan with preserved renal function. Our experience confirms that VH, (open/laparoscopic) is a safe and effective procedure with good outcomes at very longterm follow-up. No patients at puberty and in adulthood required reoperation or presented polar hypotrophy and related vascular hypertension. VH is an alternative approach to pure extrinsic-UPJO. There were few data about long and very-long term outcomes in patients after this kind of surgery. We followed-up 41 patients confirming that VH (open/laparoscopic) is safe and effective with good long-term outcomes.

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.

Non-traumatic Myositis Ossificans as Unusual Cause of Neck Pain During COVID-19 Pandemic: a Case Report.

Myositis ossificans circumscripta (MOC) is a benign disease characterized by localized heterotopic bone formation within muscles or soft tissue, usually interesting great muscles of extremities. We report a rare case of unusual location in the neck not associated with previous trauma, mimicking a solid tumor, with well-documented diagnostic imaging features. During COVID-19 pandemic outbreak in Italy, in May 2020, a 14-year-old boy developed a progressive and persistent neck pain on the right side, without known history of trauma. Initial therapy with non-steroid anti-inflammatory drugs and physiokinetic therapy gave only a slight improvement. A neck ultrasound showed an inhomogeneous right neck mass, with posterior shadowing due to calcifications. Computed tomography and magnetic resonance imaging confirmed a huge right neck mass, located in the paravertebral space with peripheral calcifications and mild central contrast enhancement. After surgical excision of the lesion, pathology revealed the presence of muscular tissue mixed with fibroblastic/myofibroblastic proliferation and ossification areas consistent with myositis ossificans. A careful analysis of clinical and radiological features is very important to manage young patients showing progressive pain and swelling of the neck, since MOC can mimic soft tissue or bone tumors, and it should be suspected even in the absence of a known history of trauma.

Surgical management of large scalp infantile hemangioma in 30-month-old infant.

Infantile Hemangiomas (IH) are the most common benign tumor of infancy, occurring in over 10% of newborns. The head and neck is the most frequently affected area (60%), and the scalp is a typical site for such large lesions. Scalp-IHs are usually focal lesions that can be both disfiguring and may lead to complications such as ulceration and bleeding. We describe a case of a 30-months old female who presented a large scalp-IH at birth that rapidly grew in the first year of life. Topical and systemic treatments (with timolol ointment and oral propranolol, respectively) were not effective in reducing dimensions of the hemangioma. After vascular imaging study, the patient underwent surgical resection of the IH and primary closure with excellent cosmetic outcome. When medical therapy is ineffective or cosmetic and functional integrity is threatened, early surgery allows to completely removing large scalp-IHs, with good cosmetic results.

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.

Torsion of cecal appendix. Report of the first Italian case and review of the literature.

In pediatric patients appendicitis is the most common cause of abdominal pain and surgery. Torsion of vermiform appendix is a rare cause, clinically indistinguishable from appendicitis with usually an intraoperative diagnosis. The first description of vermiform appendix torsion was made by Payne in 1918. Clinical presentation is similar to acute appendicitis. Preoperative investigations play a minimal role. Etiology of this condition is unclear, but is possible to distinguish a primary and a secondary torsion. We report a case of 5-years-old boy who presented with right lower quadrant abdominal pain. His clinical signs, symptoms and investigations mimicked an acute appendicitis. Intraoperatively we found a 720° appendix torsion on its base with its mesentery rotated in counter-clockwise direction. The appendix was gangrenous in appearance. A video-assisted trans-umbilical appendectomy was performed. We describe clinical presentation and management of this rare condition reviewing the literature.

Thoracoscopic treatment of a rare bilateral extralobar lung sequestration in a 3-years old girl.

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is particularly appropriate in surgical treatment.

Guidelines of the Italian Society of Videosurgery in Infancy (SIVI) for the minimally invasive treatment of fetal and neonatal ovarian cysts.

In the last three decades, fetal ovarian cysts were diagnosed more frequently, due to technological improvement and the increasing use of prenatal screening ultrasound. Nonetheless, treatment uncertainties are still present, either prenatally or postnatally. Recently, significant innovations on diagnosis and treatment have been proposed and a more conservative, minimally invasive approach may be offered to the Pediatrician or the Surgeon who face with this condition during prenatal or neonatal age. (...).

Guidelines of the Italian Society of Videosurgery (SIVI) in Infancy for the minimally invasive treatment of Hypertrophic Pyloric Stenosis in neonates and infants.

The most appropriate treatment for the infantile Hypertrophic Pyloric Stenosis (HPS) is still debated. The non-surgical conservative treatment with oral or intravenous administration of atropine does not enjoy a widespread appreciation for several factors (...).

Endourologic and Retroperitoneoscopic Combined Mininvasive Management of Cystine Urolithiasis in an Infant of 13 Months of Life.

Background: There are limited data about urolithiasis in young infants, especially in class age younger than 2 years. Case presentation: We report the case of a child <2 years old (13 months) affected by metabolic urolithiasis (cystinuria), and renal hypertension. He was admitted to our ward from the pediatric emergency room for fever, lack of appetite, irritability, and abdominal pain crisis. Ultrasonography (US) described a huge stone (15 mm) in dilated left renal pelvis (20 mm) associated with distal ureteral ectasia (7 mm). Urine and blood diagnostic assessments were performed. Hydropenic therapy and urine alkalization were started without success. The child underwent an ureteroscopy (URS) with a 4.5-6.5F rigid ureteroscope aiming to reach the renal pelvis and perform holmium: yttrium-aluminum-garnet laser stone disintegration. During the procedure, the ureter presented two unexpected stones in the distal portion (missed on US). A laser ureteral lithotripsy was effectively performed extracting smaller stone fragments. Ureteral kinking, confirmed by ascending pyelography, made it impossible to reach renal pelvis with the ureteroscope. The child was further studied with a CT scan that revealed a massive stone involvement of the left excretory pathway. Metabolic and urine assessment revealed a metabolic disease: cystinuria. To remove renal stones, an operative retroperitoneoscopy (RP) was performed. During the procedure was identified a lesion of the pelvis (secondary to stone decubitus) with urine tearing and massive perirenal inflammation. The stone was removed and pelvis was sutured. Postoperatively, the patient was stone free without major complications. Conclusions: In particular cases (younger patients, ureteral kinking, and renal failure risk), a totally combinated minimally invasive access (URS, laser stone fragmentation, and RP) can be a valid alternative to micro percutaneous nephrolithotomy or to massive open treatment. In fact, rigid URS represents a valid minimally invasive approach either for examination or for laser treatment of pediatric urinary tract stones. With important ureteral kinking, RP must be considered by experienced pediatric urologists.

Feasibility of a Standardized Management for Primary Spontaneous Pneumothorax in Children and Adolescents: A Retrospective Multicenter Study and Review of the Literature.

Introduction: The management of primary spontaneous pneumothorax (PSP) in children still remains controversial. The current literature shows a lack of scientific evidence supporting a standardized management. Materials and Methods: A multicenter retrospective study was performed, patients admitted for PSP in five Pediatric Surgical Units over a 9-year period (from 2008 to 2017) were included. Patient data were reviewed for each case. Management and outcomes were statistically analyzed. Results: Overall 159 patients (135 males and 24 females) were included in this study. During the first hospital admission, 62/159 patients (39%) were conservatively managed with oxygen therapy; 95/159 patients (60%) required a chest drain insertion; 2/159 patients (1%) underwent surgery because of a bilateral pneumothorax. Thoracoscopy was performed in 75/159 (47%) patients after the second hospital admission or for persistent air leak during the first access. Postoperatively, 8/75 (11%) patients developed recurrent pneumothorax requiring chest drain insertion in 3/8 (37%) cases or aspiration in 1/8 (13%), whereas 4/8 (50%) patients were conservatively managed. All of the patients but one, were confirmed to be affected by emphysematous-like changes at histology. Conclusions: Despite the limitations of being a retrospective study, we suggest that the early surgical management in children with PSP is feasible and safe and it seems to significantly reduce the recurrence rate. To the best of our knowledge there are no pediatric guidelines for the management of PSP, therefore, we support the need for prospective studies to create the evidence-based pillars for correct and standardized management of this condition.

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.

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 pediatric nephrectomy and partial nephrectomy.

Throughout history, the pediatric laparoscopic nephrectomy was first described at the beginning of the Nineties by Erlich and colleagues in a child and by Koyle and colleagues in an unweaned patient. (...).

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. (...).