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Hypertrophic pyloric stenosis (HPS) is the most important cause of non-bilious vomiting during infancy and it is usually idiopathic. It is very rare in older children and adolescents, in which gastric outlet obstruction (GOO) is more typically secondary to other conditions. Gastrointestinal malformation are frequently associated with Down syndrome and some of them (especially duodenal abnormalities) can be detected quite late. In infants the standard treatment of HPS is represented by a Ramstedt pyloromyotomy, while in adults a distal gastric resection is more indicated and it is usually associated to a Billroth I or II reconstruction. Case report We present an unusual case of a 12-year-old girl with Down syndrome, diagnosed with late-onset HPS. She came to our attention with periodic non-bilious vomiting and growth delay. She underwent multiple imaging examinations, which highlighted the presence of an HPS. Based on patient's age and the thickness of the pyloric muscle, we decided to perform a partial gastrectomy with a Roux-en-y reconstruction. Patient was discharged on the X day. She is now on follow up, gains weight and presents good conditions. Discussion HPS is rare but possible during childhood and adolescence. Diagnosis is usually performed through ultrasounds (US) and upper gastrointestinal radiological series (UGI), but sometimes further examinations are required (abdominal MRI, esophagogastroduodenoscopy). In older children since less invasive techniques are not effective, we have to consider partial gastrectomy. Based on our experience, Roux-en-y reconstruction is a valid option, associated with poor complication and good quality of life.

Introduction Juvenile myasthenia gravis (JMG) is a rare debilitating and potentially fatal autoimmune disease, with unclearify pathogenesis. Surgical immunomodulation with thymectomy has been repeatedly demonstrated to be a safe and effective treatment for JMG in both adult and pediatric patients. In the last few years, minimally invasive approach and above all robotic assisted thoracoscopy, replaced sternotomy which is widely used in adult patients. We report our experience in a case of robot-assisted thoracoscopic thymectomy approach in a 12 years old boy for juvenile myasthenia gravis (JMG). Case report Procedure was performed with the Da Vinci surgical robot (Xi) using left-sided approach. Left lung was kept out from mechanic ventilation. A 8 mm port for the 3-D camera was introduced on the V intercostal space on the anterior axillary line. Others two 8mm thoracic ports were placed under vision after the induction of a low-pressure pneumothorax, respectively on the midaxillary line on the III intercostal space and on the parasternal space on the V intercostal space. Bulk resection of gland was made using Maryland grasper and Harmonic scalpel, starting at level of the left pericardiophrenic angle and continuing cranially. Thymus was unstick from the posterior face of the sternum until the right pleura releasing lower thymic horns. After that, controlateral right-side thymectomy was continued into the neck to include the upper horns and finally it was removed with an endocatch bag. Histopathological examination showed a benign thymic hyperplasia. There were no perioperative and postoperative complications. The discharge was on IV post-operative day. After thymectomy, patient reported an improvement in symptoms and stopped medical therapy with pyridostigmine. Conclusions VATS and Robotic thoracoscopic thymectomy have increasingly taken hold in recent years. The surgical treatment offered to patient an improvement in clinical status. Surgery by robotic assistance has demonstrable advantages, including three-dimensional visualization and articulating instruments. Left lateral approach provided excellent visualization of the thymic veins, anonymous vases and phrenic nerves. Three-dimensional visualization as well as articulating arms greatly facilitated the dissection compared with standard thoracoscopic technique. In Literature very few series of robotic approach for surgical treatment of JMG in children are reported, for these reasons further studies are needed.

In the last decade, the applicability of robotic surgery has been demonstrated in many interventions, expanding the indications of minimally invasive surgery also to pediatrics. The aim of the study is to evaluate postoperative pain to demonstrate better control following robotic procedures compared to thoraco-laparoscopic surgery. An observational, retrospective, multicentre study was performed involving 204 children undergoing robot-assisted surgery and thoraco/laparoscopic surgery at the Istituto Giannina Gaslini in Genoa and the Siena University Hospital (2013-2017): 83 children underwent robotic-assisted surgery and 121 thoracic-laparoscopic surgery. Personal data and type of intervention were assessed, dividing the patients into four categories: thoracic, gastrointestinal, hepatobiliary and urological surgeries. We analyzed the anesthetic risk according to ASA classification by type of intervention, the type of anesthesia used, the anesthetic drugs used during surgery and in the postoperative period. Both the problems that occurred during the procedures and the number of interventions converted into open during robotic surgery and laparoscopic thoracic surgery were analyzed. Pain was measured on the 1st, 2nd and 3rd day (FLACC or NRS scales). By comparing the two groups (robotics-non-robotics), the analysis shows that postoperative pain does not change with the chosen approach, but always maintains very low values, typical of minimally invasive surgery. The pain score is significantly higher in patients undergoing thoracic surgery, either robotic or thoracoscopic, compared to those undergoing gastrointestinal surgery (P corrected according to Bonferroni: 0.0006) and those undergoing urological intervention (P corrected according to Bonferroni: 0.04). In conclusion, no significant change in the intensity of postoperative pain between the two groups was found, while it is seen that the pain in patients undergoing thoracic interventions (robotic/thoracoscopic) is more intense than that reported for other types of interventions.

Lymphangioma accounts for 5% of benign pediatric tumors and less than 1% is located in the abdomen. Indeed mesenteric cystic lymphangioma is a rare benign abdominal mass and its pathogenesis is still unknown. Mesenteric cystic lymphangioma is often diagnosed incidentally. Nevertheless, in some cases its clinical presentation can mimic several common surgical pathologies. Here we describe a case of mesenteric cystic lymphangioma mimicking an ileo-colic intussusception.

Torsion of an epididymal cyst is an extremely rare cause of acute scrotum in children and in young boys. Its reported incidence is 5-20%. The treatment is usually conservative. Many cases (up to 60%) regress spontaneously, especially if below 3 cm. Few cases have been reported in Literature (7 cases/2018). We add our patient as eighth case. He was a 13-year-old boy who was admitted for acute scrotum. Ultrasound excluded torsion of the testis and he was managed for 5 day conservatively. Based on clinical history and physical exam, we decided to perform a prompt surgery that revealed a torsion of epididymal cyst that was confirmed by histological exam.

Purpose To evaluate and compare the feasibility and short-term results of laparoscopic and robotic total oesophago-gastric dissociation (TOGD) with a Roux-en-Y oesophago-jejunostomy. Minimal invasive surgery has multiple advantages in neurologically impaired patients. Robotic approach has overcome disadvantages linked to laparoscopy, in particular, referring to the surgeon fatigue. Methods A retrospective study comparing five laparoscopic and five robotic TOGD was conducted between February and October 2016 in Giannina Gaslini Children's Hospital and Section of Pediatric Surgery of Siena. Neurologically impaired children scheduled for TOGD were included. Age, sex, weight, symptomatology, presence of epilepsy, and preoperative X-ray contrast were considered. Operative time, hospital stay, postoperative complications, redo surgery, nutrition rehabilitation, and X-ray contrast study after 5 days and after 1 month from the intervention were recorded. Results In our series, there were no intraoperative complications, no conversions to open surgery, and no vagal lesions. In two of five robotic cases, a pyloroplasty was necessary. The median operative time was statistically longer in the robotic group. One dehiscence in the robotic group was recorded, and no dumping episodes occurred. No statistical differences in terms of complications were detected. Conclusion TOGD is feasible both with laparoscopic and robotic-assisted surgery with similar results. Robotic approach is considered feasible. At the same time, high laparoscopic skills allow to reach the same results as robotic approach with shorter operative time.

Persistent pulmonary interstitial emphysema (PPIE) is a rare condition that occurs in both preterm and term infants. It is thought to arise from a disruption of the basement membrane of the alveolar wall allowing air entry into the interstitial space. The characteristic CT scan appearance of PPIE can be used to differentiate it from other congenital cystic lesions that may present similarly. The management of infants suffering from diffuse persistent interstitial pulmonary emphysema varies according to severity and stability of the patient, being either conservative treatment or aggressive surgical treatment by pneumonectomy. We report a case of an unstable patient with diffuse persistent interstitial pulmonary emphysema successfully treated by lobectomy as a form of conservative surgical approach.

Background Esophageal replacement in children is an option that is confined to very few situations including long-gap esophageal atresia and esophageal strictures unresponsive to other therapies (peptic or caustic ingestion). The purpose of our work was to describe the experience of gastric transposition in three Italian centers. Methods This is a retrospective study. The data were extrapolated from a prospective database. We included all patients who had undergone gastric transposition in the last 15 years. Results In the 15-year period, eight infants and children (3 males and 5 females) underwent gastric transposition for esophageal replacement. Six patients had long-gap esophageal atresia, and two had caustic esophageal stenosis. There were no deaths in the series. Three patients had an early postoperative complication: two had a self-limited salivary fistula at three weeks, and one (a patient with jejunostomy) had a jejunal perforation treated surgically. One late complication, anastomotic stricture, was recorded that required two endoscopic dilatations. The median follow-up was 60 months (range: 18-144 months). At final clinical follow-up, six patients had no eating problems, and two patients had some difficulties with eating (jejunostomy in situ), but they underwent logopedic therapy with improved outcomes. All patients had an increase in body weight and height postoperatively. Conclusion Our small study reports the clinical experience of three Italian centers in which gastric transposition was performed with excellent results, both in terms of surgical technique (simplicity, reproducibility, complication rate) and clinical follow-up (good oral feeding of young patients, normal social life and regular growth curves).

Background: Phimosis is a condition in which the foreskin cannot be portrayed on the glans. It is a physiological and common condition in the pediatric age. The pathological form derives from an inflammatory or traumatic lesion. Circumcision is the most common surgical treatment of phimosis but it is a controversial practice, especially in occidental world. Methods: We enrolled 61 patients with pathological phimosis (22/balanoposthitis, 18/painful erection, 21/ urinary discomfort) between 2015–2017. Results: All patients underwent preputialplasty. Conclusions: Various alternatives to circumcision have been described, as manual retraction therapy, topical steroid therapy, and many types of preputialplasty. We report our technique.

Duplications of the alimentary tract are rare malformations, and ileum is the most involved intestinal tract. During fetal life, they are usually occasional findings and may be asymptomatic until adulthood. We present the clinical case of volvulus on ileal cystic duplication diagnosed by ultrasound during prenatal follow-up. Case report: Diagnosis of cystic ileal duplication was made by ultrasound at our center at 23 weeks of gestational age, which was confirmed with fetal MRI. During follow-up, at 36 + 4 weeks, the presence of dilated loops with reduction of peristalsis was shown by ultrasound, so that, despite stable fetal parameters, an emergency caesarean delivery was programmed for suspected volvulus. After being stabilized, the patient underwent to laparotomy that revealed ileal volvulus at 2 cm from the ileo-cecal valve on a cystic duplication. Cyst and necrotic loop (10 cm) was resected and was performed an anastomosis. Patient started feeding on VII post-operative day, and was discharged on the X day. Patient is on follow up and present good conditions. Discussion: Diagnosis of intestinal cystic mass in prenatal period requires a close follow-up because the risk of a volvulus. According to the literature, even in the presence of stable fetal parameters, the presence of dilated loops and the absence of peristalsis are signs that must suspect the presence of a mechanical occlusion for which timely treatment is necessary to reduce mortality and morbidity. Keywords: Prenatal ultrasound, Volvulus, Ileal duplication

Background Ewing Sarcoma (ES) and Neuroblastoma (NB) belong to a family of tumours of primitive neuroectodermal origin (PNET) that occurs in both bone and soft tissue. Notwithstanding ES and NB are two distinct malignant tumours, sometimes there could be a link between them. Case report We describe a case of an extraosseous ES localized in the retrosternal region and the upper lobe of the right lung, which had been previously treated for NB in a 6 years old female. We treated this case with a clamshell approach which allows, in a one-step surgery, a complete excision of the mass reconstructing the hemysternectomy with a non-crosslinked matrix. Conclusion the clamshell approach is therefore useful to achieve the retrosternal space and the lung with a single surgical access. According to our experience, we consider appropriate to use a non-crosslinked matrix for sternal reconstruction.

More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.

Congenital cystic lung lesions are a rare but clinically significant group of anomalies, including congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, congenital lobar emphysema (CLE) and bronchogenic cysts. Despite the knowledge of these lesions increasing in the last years, some aspects are still debated and controversial. The diagnosis is certainly one aspect which underwent many changes in the last 15 years due to the improvement of antenatal scan and the introduction of 3-D reconstruction techniques. As it is known, a prompt diagnosis has an essential role in the management of these children. The new imaging studies as 3D Volume rendering system are the focus of this paper. We describe our preliminary experience in a case of hybrid lung lesion, which we approached by thoracoscopy after a preoperative study with 3D VR reconstruction. Our final balance is absolutely positive.

Highlights • We describe a case of a 3 years old girl, with all features of Herlyn–Werner–Wunderlich syndrome (HWWS) who came to our attention for lower abdominal mass. • Only six cases are reported in Literature with early onset of this syndrome under 5 years. • The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. • The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. • An error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. • Our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cisto-vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis., Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis [1,2]. Most authors reported cases of Herlyn–Werner–Wunderlich syndrome with prepuberal or postpuberal onset with cyclical abdominal pain and a vaginal mass (3–8). Only six cases are reported in Literature with early onset of this syndrome under 5 years (9–14). Our case is about 3 years old girl, with all the features of this syndrome who came to our attention for lower abdominal mass. The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. For all these reasons, our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cystoscopy and vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

Objective. Over the past decade, multiple factors have changed the pattern of neonatal surgical emergencies. An increase in prenatal screenings and the development of neonatal tertiary care centres have changed the clinical approach to these kids. Materials and methods. Between 1995 to 2011 were retrospectively reviewed 34 patients with diagnosis of uncommon rare neonatal surgical emergencies at our institute. We analyzed: sex, gestational age, weight at birth, primary pathology, prenatal diagnosis, associated anomalies, age and weight at surgery, clinical presentation, start of oral feeding and hospitalization. The follow-up was performed at 6,12, 24 and 36 months. Results. There were 21 male and 13 female. The gestational age ranged between 28 and 36 weeks. The weight at birth ranged between 700 and 1400 grams. Oral feeding was started between 4th and 10th postoperative day. The average hospitalization was about 70.47 days. To date, all patients have finished the followup. They are healthy. Conclusion. The outcome of the patients with uncommon surgical emergencies is different based on the etiology. Overall survival is generally good but is influenced by the associated anomalies.

The aim of this study is to present a preliminary series of six children affected by an anterior mediastinal mass, resulting in a thymoma. We treated this pediatric population by a video-assisted thoracoscopic thymectomy.We performed a retrospective study from January 2000 to January 2012 of all children affected by an anterior mediastinal mass, resulting in a thymoma. Data included sex, age at surgery, clinical and radiological features, surgical procedure, follow-up, and complications.Video-assisted thoracoscopic thymectomy was performed in six children (five boys and one girl). Four patients presented with dyspnea and/or thoracic pain treated with analgesic and corticosteroid therapy without any benefits. Two patients were asymptomatic. Preoperatively, exams included radiological imaging, blood, and immunological test. All 6 patients were treated with a thoracoscopic approach; of these patients, 5 were treated with a thoracoscopic right-side thymectomy and 1 with a left-side approach. Patients were treated by a three-trocar technique, and thymectomy was performed using Valleylab (now Covidien) (Boulder, CO) LigaSure™. The mean operative time was 120 minutes. The mean hospital stay was 5 days. In all cases histological findings revealed no malignancy. The mean follow-up was 38 months.Thoracoscopic thymectomy facilitated the goal of early thymectomy. The right-side approach is preferred because it gains a good visualization of the venous anatomy for dissection. The left side allows a good extended resection of both the thymus and perithymic fat. Thoracoscopic thymectomy should be consider a valid, less invasive alternative to the most radical open approach.

Congenital diaphragmatic hernia (CDH) is a defect of development of the pericardioperitoneal canals. In general, it occurs at 6–7 weeks of gestation. It is responsible for herniation of the abdominal organs into the thoracic cavity. CDH is delimited anteriorly by the diaphragm, sideways by the diaphragmatic pillars and posteriorly by the costal margin. CDH causes lung compression on the side of the diaphragmatic defect, precluding normal development of the lung (“lung hypoplasia”) [1, 2]. CDH has an incidence of 1 case per 3,000–5,000 in live births with a male predominance. CDH is not a hereditary disease. CDH is on the left side in 86% of cases (Figs 19.1 and 19.2).

Background: Vesico-ureteral reflux (VUR) is a dynamic event in which a retrograde flow of urine is present into the upper tracts. VUR may occur isolated or in association with other congenital abnormalities or as part of syndromic entities. We present a patient with a bilateral primary VUR, syndromic disease caused by a large deletion of 18q (18q21.3-qter) and terminal duplication of 1p (1p36.32-p36.33). Case report: The patient was 8 years old female with a disease including moderate growth retardation, psychomotor retardation, facial dysmorphism, single umbilical artery, umbilical hernia, urachal remnant, bilateral congenital clubfeet and renal-urinary disease. Chromosomal analysis and Array-CGH revealed two heterozygous chromosomal rearrangements: 1p terminal duplication and de novo 18q terminal deletion. She referred to our clinic to evaluation of bilateral hydronephrosis and right renal cortex thinning. Voiding cystourethrography demonstrated bilateral grade IV VUR and dimercaptosuccinic acid renal scintigraphy confirmed right renal cortex thinning and showed a cortical uptake of 75% of the left kidney and 25% of the right kidney. The patient underwent ureterovesical reimplantation after failure of 3 endoscopic submeatal Deflux injections with VUR resolution. Conclusions: This is the first report involving a patient with 18q-syndrome and contemporary presence of 1p chromosomal terminal duplication. The coexistence of two chromosomal rearrangements complicates the clinical picture and creates a chimeric disorder (marked by characteristics of both chromosomal anomalies). Kidney problems, primarily VUR is reported in 15% of patients affected by 18-q syndrome and no cases is reported in the literature regarding a correlation between VUR and 1p36 chromosomal duplication.

Neonatal testicular tumors are rare and should be considered in the differential diagnosis of newborn scrotal masses. Juvenile granulosa cell tumor (JGCT) accounts for about 5% of all prepubertal testis tumors. As a benign neoplasm, radical orchiectomy is sufficient for treatment. We report a case of a newborn with a prenatal diagnosis of scrotal mass. After surgery, the histological diagnosis was juvenile granulosa cell tumor. To date the patient is healthy.