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.

Congenital malformations potentially affecting respiratory function: multidisciplinary approach and follow-up.

BACKGROUND AND AIM: Congenital malformations such as oesophageal atresia (OA) and tracheoesophageal fistula (TOF), congenital pulmonary airway malformations (CPAMs), congenital diaphragmatic hernia (CDH) and vascular rings (VRs) can affect lung development and respiratory function. This observational study describes our multidisciplinary approach and respiratory follow-up of children with such congenital malformations. METHODS: Clinical data of children followed at the Pediatric Respiratory Unit of Parma University Hospital (Italy) between January 2015 and January 2020 were collected.  Results. Twenty-three patients with congenital malformation affecting lung development were identified. Almost half of our patients were diagnosed with fetal ultrasound.  Children attended the clinic at a mean age of 3 (3.7) years and follow-up visits were scheduled every 6 months average. More than half of our patients were hospitalized for lower respiratory tract infections. Six out of 9 children able to perform spirometry showed anomalies in lung function. Chest physiotherapy was recommended especially in children with OA. CONCLUSIONS: Children with congenital malformations affecting lung development are at risk of short and long-term respiratory complications, especially in the first years of life. OA was the malformation more associated to respiratory problems. Multidisciplinary approach and appropriate personalized follow-up are recommended for the best management of these children.   Abstract word count: 186.

Megarectosigmoid in anorectal malformations: the role of laparoscopic resection.

Surgical treatment for anorectal malformations may lead to chronic constipation or stool incontinence. The first condition is mostly linked to an abnormal dilation of rectum and sigma and it is primarily managed with medical therapy (laxatives, diet and enemas). When medical therapy fails to improve the symptoms, a surgical resection of the dilated colon is advocated. When performing the procedure it is mandatory to consider all the previous operations the patient undergone. We present a laparoscopic left emicolectomy for an extremely dilated megarectosimoid after posterior sagittal anorectoplasty in childhood for a recto-urethral fistula.

Surgical Experience and Learning Points in the Management of Foregut Duplication Cysts.

BACKGROUND: Foregut duplication cysts (FDC) have unpredictable natural course and rarely remain asymptomatic. We present our large cohort of FDC to highlight surgical learning points and outcome. MATERIALS AND METHODS: Review of 41 children with FDC (April 1997-April 2015) included demographics, clinical presentation, preoperative investigations, surgical management, post-operative complications, and overall outcome. RESULTS: Forty-one children (26 girls/15 boys; male/female [M/F] ratio 1:1.7) had 43 FDC (two cases >1 cyst) consisting of antenatally diagnosed 16 (39%), late with symptoms 21 (51.2%), and incidental 4(9.8%) cases. FDC locations were cervical (3), mediastinal (35), and subdiaphragmatic (5). During all cervical FDC resection, recurrent laryngeal nerve was required to be dissected off the cyst wall. Thoracoscopic resection was feasible in 27 cases, with 4 conversions related to infections and adhesions or accidental injury to trachea/esophagus. Complete resection was achieved in all cases with no recurrence. There was no mortality and morbidity included, chylothorax (1) esophageal injury (1), phrenic nerve injury (1), and tracheal injury (2). The minimally invasive surgery and surgery on asymptomatic lesions significantly affect the outcome by reducing need for chest drain (p = 0.01) and length of hospital stay (p = 0.04). CONCLUSION: This retrospective study confirms the efficacy and safety of thoracoscopic surgery; important learning points were association of common wall with trachea in some superior mediastinal cysts and association of recurrent laryngeal nerve in cervical FDC.

Chronic inguinal pain in children.

PURPOSE: To present our experience in the management of chronic groin pain in children. METHODS: We report 4 patients (age range 0-18 years old) who presented with history of chronic groin pain (April 2010 January 2017). After failure of all conservative treatments they underwent surgical management as ultima ratio. RESULTS: Currently, there is no consensus on treatment of 'Sportsman's Hernia' and literature on paediatric population is still poor. There are no appropriate randomised controlled trials supporting a standardized management of chronic groin pain. Initial approach should be conservative [Physical Medicine and Rehabilitation (PM&R) follow-up for 3-6 months] and surgical treatment proposed after failure of conservative therapies. It is advisable to consider surgery at least months after clinical onset. More recently, some studies suggested surgery as a first line treatment. Most of surgical cases manage to recover to full activity without pain, as reported in our experience. Surgical approach to the groin can be anterior (open) or posterior (laparoscopic), both these procedures have shown good results. Surgeons having different opinions on the aetiology of 'Sportsman's Hernia' may sustain the suitability of different surgical techniques. In our experience, pain was localized at the pubic tubercle therefore we preferred the 'open' approach ensuring a successful release of ilioinguinal and genitofemoral nerves. CONCLUSIONS: Children with chronic groin pain can benefit of surgical treatment. In our experience, surgery always proved to be successful for treating chronic groin pain in paediatric age. Further multicentric studies are needed to support these results. KEY WORDS: Bernhardt-Roth syndrome, Children, Chronic groin pain, Chronic inguinal pain, Meralgia Paraesthetic, Sportsman's Hernia.

Endoscopic treatment of vesicoureteric reflux in paediatric patient: results of a long-term study.

BACKGROUND AND AIM OF THE WORK: Vesicoureteric reflux is the most common uropathy in paediatric age. It may be treated with open surgery, antibiotic therapy or endoscopic injection. The purpose of this work is to determine outcomes and experiences of parents with children who underwent endoscopic treatment for VUR. MATERIALS AND METHODS: In a period of 5 years (from 2004 to 2009) 48 patients (20 boys and 28 girls, mean age 5,5, range 1-17) underwent endoscopic treatment for VUR. Sample included 31 cases of primary reflux (64,6%), 11 complex cases of VUR (6 duplex system, 3 duplex system with ureterocoele, 2 bladder exstrophy) and 6 children (12,5%) with VUR secondary to neurogenic bladder. All procedures were performed by the same surgeon. A questionnaire assessing experiences with endoscopic treatment was administered to all families. RESULTS: Follow-up lasted from a minimum of 6 months to 5 years. Overall cure rate was 68,7% (33/48) per child after a single injection, a second injection performed in cases with VUR recurrence raised it to 81,2% (39/48 patients). Overall cure rate per grade of VUR was 60% (3/5) for grade I, 94,1% (16/17) for grade II, 86,9% (20/23) for grade III, 64,7% (11/17) for grade IV and 85,7% (6/7) for grade V. Minimal postoperative complications were recorded: 2,1% urinary tract obstruction, 12,5% macro-haematuria, 6,2% lumbar pain, 4,1% urinary retention or strangury. The results of the survey given to families were encouraging. CONCLUSIONS: Endoscopic treatment for VUR seems to be a feasible procedure as primary intervention.