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Aim: To describe architecture and expression of myosin isoforms of the human cremaster muscle (CM) and to individuate changes in clinically differentiated abnormalities of testicular descent: cryptorchidism or undescended testis (UDT) and retractile testis (RT). Background: The CM is a nonsomitic striated muscle differentiating from mesenchyme of the gubernaculum testis. Morphofunctional and molecular peculiarities linked to its unique embryological origin are not yet completely defined. Its role in abnormalities of testicular descent is being investigated. Subjects and Methods: Biopsy samples were obtained from corrective surgery in cases of cryptorchidism, retractile testis, inguinal hernia, or hydrocele. Muscle specimens were processed for morphology, histochemistry, and immunohistology. Results and Conclusions: The CM differs from the skeletal muscles both for morphological and molecular characteristics. The presence of fascicles with different characterization and its myosinic pattern suggested that the CM could be included in the specialized muscle groups, such as the extrinsic ocular muscles (EOMs) and laryngeal and masticatory muscles. The embryological origin from the nonsomitic mesoderm is, also for the CM, the basis of distinct molecular pathways. In UDT, the histological alterations of CM are suggestive of denervation; the genitofemoral nerve and its molecular messengers directed to this muscle are likely defective. Compared with the other samples, RT has a distinct myosinic pattern; therefore, it has been considered a well-defined entity with respect to the other testicular descent abnormalities.

Introduction There is a need to develop sensitive markers to diagnose or monitor the severity of intestinal damage in necrotizing enterocolitis (NEC). Mitochondrial deoxyribonucleic acid (mtDNA) is increased in the intestine and blood of adults in response to intestinal ischemia and can trigger secondary organ damage. We hypothesize that mtDNA is increased during experimental NEC and that mtDNA levels are correlated to the degree of intestinal injury. Materials and Methods NEC was induced in C57BL/6 mice (n = 18) (approval: 44032) by gavage feeding with hyperosmolar formula, hypoxia, and lipopolysaccharide administration from postnatal day (P) 5 to 9. Breastfed pups served as control (n = 15). Blood was collected by cardiac puncture and terminal ileum was harvested on P9. Reverse transcription quatitative polymerase chain reaction was used to measure mtDNA (markers COX3, CYTB, ND1) and inflammatory cytokines (interleukin 6 [IL-6] and tumor necrosis factor-α[TNF-α]) in blood and ileum. Intestinal injury was scored blindly by four investigators and classified as no/minor injury (score 0 or 1) or NEC (score ≥2). Results mtDNA is significantly increased in gut and blood of NEC mice (p Conclusion Following NEC intestinal injury, mtDNA is released from the intestine into circulation. The blood level of mtDNA is related to the degree of intestinal injury. mtDNA can be a novel marker of intestinal injury and can be useful for monitoring the progression of NEC.

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.

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.

Background Urethral duplication associated with epispadias is a rare malformation. Few cases are described in Literature. We report the experience of two centers to add to the literature. Methods A retrospective study was conducted in two Italian Centers. All patients with urethral duplication associated with epispadias, treated from 1997 to 2017 were included. The preoperative work-up included renal-urinary ultrasonography and voiding cystourethrogram. All patients underwent surgery according to the Mitchell-Caione technique. Cosmetic result, urinary continence and satisfaction degree of patients at the last follow-up were evaluated as outcomes. Six male patients with urethral duplication in epispadias were included. Two patients presented penile epispadias and four penopubic epispadias. Only one patient had urinary incontinence as presenting symptomatology. The diagnosis of urethral duplication was accidental during preoperative evaluation in the remaining five patients. Results At last follow-up (mean 8.3 years) all patients but one presented good cosmetic result, one patient presented mild stress urinary incontinence, one presented nocturnal enuresis. The physical genital appearance was improved in all patients. Urethral duplication in association with epispadias is a rare urogenital abnormality. No classification is universally accepted. Conclusions Based on our experience, we believe that the presence of any duplication should be carefully searched during surgery for male epispadias.

Background: Congenital pouch colon (CPC) is an unusual malformation associated with anorectal malformations (ARMs) that are seen especially in Asia. The aim of this study was to analyse our series of CPC associated with ARM in our centre and report our experience based on the rarity of pathology. Materials and Methods: This is a retrospective study conducted at the Department of Paediatric Surgery in North Iraq. We identified patients from a prospectively kept database, including all patients managed between 1997 and 2014. Results: In total, 17 patients (3 females and 14 males) were included; all had CPC and high ARM; male:female ratio was 5.6:1. Two females had colo-vestibular fistula, 1 had a colo-cloacal fistula, all males had imperforate anus with colovesical fistula. Pre-operative diagnosis of CPC was made in eight patients (47%). Four patients (23.5%) had an incomplete pouch colon (Type III and IV), and 13 (76%) had complete pouch colon (Type I and II). All patients were managed with staged surgery. Mortality rate was 17.6% (3/17). Conclusions: CPC associated with ARM can be defined as 'Asian' complex malformation. Few cases are reported in Literature from Europe and USA. However, it is known that it is ever more frequent the collaboration between Asian and European surgeons (as in our study), so the suspicion should be considered in all patient high ARM who come from Asian region. We reviewed literature and report our experience of 15 years, after a retrospectively analysis, to share it and add our data to their reported.

Prune-belly syndrome is a rare congenital disorder characterized by three major features: deficient abdominal wall musculature, urinary tract anomalies, and bilateral cryptorchidism in males. Incidence is estimated between 1/30,000 and 1/50,000 live births and the 3–5% of known cases are females. The exact pathogenesis of prune-belly syndrome is not clearly known as yet. Three major theories are known, but none of them have universal acceptance because none completely explains the whole constellation of findings in the syndrome. No specific gene defect has been identified for PBS. Many authors tried to formulate classification systems, but the considered classification system to date is by Woodard. The main goal of treatment of patients with prune-belly syndrome is to preserve renal function. The prognosis and the postnatal management of these patients, indeed, are closely related to it. The spectrum of therapeutic possibilities range from a “wait and see” approach to immediate or delayed urologic surgery.

We present a very rare case of gastric duplication treated with complete excision with two surgical procedures. We decided to report our case to share our experience that confirm the difficult of preoperative/intraoperative diagnosis of gastric duplication in a pediatric patient. Finally we reviewed the literature to date.

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.

Summary Introduction Botulinum toxin-A (BtA) has been used for refractory idiopathic overactive bladder (IOAB) in children. Data on the optimum dose success rates, duration of effect, complications and medium-term outcomes are limited. This study aims to analyse the authors' experience to provide medium-term results of BtA in symptomatic refractory patients. Materials and methods Patients with refractory IOAB who were treated with BtA (Botox®) were retrospectively analysed. All patients had urodynamic study before treating with BtA. Group A had low-dose BtA (LDBtA) at 5 units/kg (maximum 150 units), and Group B had high-dose BtA (HDBtA) at 10 units/kg (maximum 300 Units). Post-BtA clinical response, functional bladder capacity (FBC) and postvoid residual (PVR) were assessed in addition to the duration of response. Results Thirty-nine patients, 11 male and 28 female, were analysed. Forty-six percentage had symptom improvement (73% of males and 36% of females [P = 0.072, ns]). The difference in response rates between LDBtA and HDBtA was not statistically significant (P = 0.684). Increase in total bladder capacity (TBC) was greater in those given HDBtA (P ≤ 0.001), but the increase in FBC was not different between the groups, due to greater PVRs in the HDBtA group. Nine patients (23%) developed UTI; however, six of these patients suffered with UTI pre-BtA as well. Only three were asymptomatic after a single treatment with BtA. The remainder required further BtA or oral anticholinergic therapy. At a median follow-up of 35.4 months (interquartile range [IQR] 25.2–46.6), 12 (31%) were asymptomatic and off all therapy, 18 (46%) were still symptomatic despite therapy and 9 (23%) had their symptoms controlled on continuing treatment. Discussion and conclusions Botulinum toxin-A improves symptoms in 46% of children after the first injection in refractory IOAB. Although HDBtA resulted in greater increase in bladder capacity, it conferred no advantage in terms of success rate or duration of response. Five units/kg may be an optimum dose to use as a first treatment with the understanding that some patients will require a higher dose. And, there will be a cohort of patients who need a dose lower than 5 units/kg. A higher dose is more likely to lead to PVR leading to urine stasis and UTIs. The success of BtA only lasts until its effect wears off, and the majority of this cohort (36/39) required continuing treatment with repeat BtA or anticholinergic agents. However, it remains a useful option in patients who are intolerant or unresponsive to anticholinergic medication with symptomatic resolution in 30% at medium-term follow-up. Download : Download high-res image (173KB) Download : Download full-size image Fig . Change in TBC, PVR and FBC with BtA therapy, according to dose.

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

The management of choledochal cysts has evolved during the last 3 decades. Laparoscopic surgery has revolutionized the treatment of choledochal cyst (CC). We report our case, 2-year old female, in which total excision of cyst and Roux-en-Y hepaticoenterostomy is done by laparoscopic approach. In the hands of experts, laparoscopic excision of the CC is safe and effective approach. This is our first experience and our outcome has been good to a follow-up of 6 months. In our opinion, laparoscopic surgery is a safe treatment of choledochal cyst with less postoperative morbidity, a shorter length of stay and a lower blood loss when compared with open approach. Laparoscopic surgery may become the first choice procedure for choledochal cyst.

Introduction To study the impact on adult’s fertility of serum inhibin B levels in adolescent patients with idiopathic varicocele after minimally invasive surgical correction and to compare fluctuation of pituitary-testis hormonal values and testicular volumes. Materials and Methods: A case-control study was carried out on a group adolescent patients (n=60) affected by idiopathic left varicocele (group V) and compared with control adolescents (n=40) in the Paediatric Surgery Section of Siena (from June 1993 till September 2013). Inhibin B levels and testicular volume before (T0) and after at 6 and 12 months from surgery (T1 and T2) were evaluated. Results: A positive correlation between testicular growth at T1 and T2 (P

Background Necrotizing enterocolitis (NEC) is the most common surgical emergency in newborns and it is still a leading cause of death despite the improvements reached in the management of the critically ill neonate. The purpose of this study was to evaluate risk factors, surgical treatments and outcome of surgical NEC. Methods We retrospectively evaluated a multicentric group of 184 patients with surgical NEC over a period of 5 years (2008-2012). Indications to operation were modified NEC Bell stages IIIA or IIIB. The main outcome was measured in terms of survival and postsurgical complications. Results Data on 184 patients who had a surgical NEC were collected. The majority of patients (153) had a primary laparotomy (83%); 10 patients had peritoneal drainage insertion alone (5%) and 21 patients had peritoneal drainage followed by laparotomy (12%). Overall mortality was 28%. Patients with lower gestational age (P=0.001), lower birth weight (P=0.001), more extensive intestinal involvement (P=0.002) and cardiac diseases (P=0.012) had a significantly higher incidence of mortality. There was no statistically significant association between free abdominal air on the X-ray and mortality (P=0.407). Mortality in the drainage group was 60%, in the laparotomy group and drainage followed by laparotomy group was of 23-24% (P=0.043). There was a high incidence of stenosis (28%) in the drainage group (P=0.002). On multivariable regression, lower birth weight, feeding, bradycardia-desaturation and extent of bowel involvement were independent predictors of mortality. Conclusions Laparotomy was the most frequent method of treatment (83%). Primary laparotomy and drainage with laparotomy groups had similar mortalities (23-24%), while the drainage alone treatment cohort was associated with the highest mortality (60%) with statistical value (P=0.043). Consequently laparotomy is highly protective in terms of survival rate. Stenosis seemed to be statistically associated with drainage. These findings could discourage the use of peritoneal drainage versus a primary laparotomy whenever the clinical conditions of patients allow this procedure.

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.

Introduction Burkitt’s lymphoma is a high grade B-cell tumor described for the first time by the Irish surgeon Dennis Burkitt in 1958 in Africa. The most frequent of the clinical variants, in which it is classified by the World Health Organization (sporadic, endemic, HIV-associated), is the sporadic one, which usually involves the abdomen, in particular the ileocecal tract. Thus, a common clinical presentation is that of a child suffering from abdominal pain with nausea and vomit, until the dramatic case of an intestinal occlusion by an intussusceptions. According to this, the surgeon is the first who diagnoses and treats this tumor, playing an important role for the treatment, in terms of reduction of the metabolic complications of the medical therapy and of improvement of survival rate. In this work we present a case of a child operated for intestinal occlusion by ileoileal intussusception, caused by a Burkitt’s lymphoma, as it was diagnosed by histological examination few days after surgical intervention. Case report A 12-years boy reached the emergency department for abdominal pain and vomit. Two weeks before he had a surgical intervention for a suspected appendicitis at another hospital. An ultrasound examination was performed and it revealed the presence of a complex mass in the right iliac fossa. The day after the patient felt worse and he had an episode of bilious vomit. An x-ray examination of the abdomen was performed and showed the presence of an intestinal obstruction. The patient underwent to surgical intervention. The obstruction was caused by an ileoileal intussusceptions, and it needed to perform a resection followed by anastomosis. Few day after surgical intervention, the result of histological examination indicated the presence of a Burkitt’s lymphoma within the tract resected. A CT scan was performed and showed the involvement of mesenteric lymph nodes. The bone marrow aspirate and the lumbar puncture showed no neoplastic presence. Then the patient started therapy according to protocol. Discussion and Conclusions Burkitt’s lymphoma represents 3-5% of all non-Hodgkin lymphomas, and 40% in pediatric population. Children have an excellent prognosis with contemporary treatment regardless of the disease stage. Patients with limited stage disease are curable with limited treatment, avoiding complications associated with more intensive therapies. Nevertheless surgery is important in the management of this disease, the role of the surgeon has usually been controversial. A surgical intervention can be resolving in case of limited disease, or, in case of a high stage disease, it can be diagnostic or helpful, through the debulking of the mass. However, apart in case of intussusception, the diagnoses of Burkitt can be challenging and the presence of disease can revealed after a story of recurrent abdominal pain or after surgical interventions for appendicitis. In this work we show how a timely diagnosis can be difficult and how it can be an obstacle for treatment. In this case the sudden worsening of clinical conditions permitted an early diagnosis with a complete resection of the ileum involved by Burkitt’s lymphoma has improved the prognosis and reduced therapy’s complications.

Background: In the last 30 years mini-invasive surgery (MIS) has been widely used becoming an important and irreplaceable method, safe and highly reliable, in both the diagnostic and therapeutic treatment, for a variety of conditions in both the child and the newborn. This has led to a significant increase of the use of this approach in the pediatric population under the age of 3 years, with results similar to the open techniques. Materials and Methods: At the Department of Surgery, Medicine and Neuroscience at the University of Siena we conducted a retrospective study of patients aged less than three years of life underwent minimally invasive procedures. The clinical information was extrapolated from a prospective database. They included all patients treated from June 2003 to October 2014. Were considered for each patient demographics, associated diseases, surgical procedure, MIS approach, the instruments used, the duration of surgery and hospitalization, intraoperative and postoperative complications (early and late). Results: From a total of 933 minimally invasive procedures, 150 (16%) were performed under the age of 3 years. The 76% of patients were males, 24% were females. Twenty-two patients (15%) presented associated diseases. 53 (35%) were diagnostic procedures, 97 (65%) were therapeutic procedures. We treated: 1 3 esophageal atresia, 1 diaphragmatic hernia, 1 congenital cystic adenomatoid malformation (CCAM), 8 stenosis of the ureteropelvic junction , 2 disorders of sexual differentiation (DSD), 20 inguinal hernias, 75 cases of non-palpable testis, 15 cases of Hirschsprung disease, 10 cases of fundoplication, 4 cases of ovarian tumors, 6 cases of nephrectomy, 4 cases of eminefrectomy and 1 case of thymoma. In total we performed 6 (4%) thoracoscopic procedures, 18 (12%) in retroperitoneoscopy and 126 (84%) in laparoscopy. 55 procedures (37%) were performed in "one-trocar" technique. Among the remaining 95 (67%), in 62 (41%) we used a “3 mm” instruments, in 18 (12%) “5 mm” instruments and in 15 (14%) they were mixed (3/5 mm). The duration of the procedure was an average of 59 minutes (range: 20-135 minutes) in diagnostic procedures and 184 minutes (range: 40-370 minutes) in the therapeutic procedures. We converted to an open technique in 14/150 (9%). We reported no postoperative complication and incidentaloma no intraoperative complications. The following is 100% survival. Conclusions:MIS is probably the most important change that has taken place in the field of Pediatric Surgery in the last 30 years. The optimum benefits are to be found in the lesser surgical stress, the lower cavity contamination, the magnification of details, the reduced hospital stay, reduced morbidity or the best aesthetic result.

Introduction Duodenal atresia Windsocks type is a rare condition of congenital bowel obstruction. Thanks to recent technological advancements of prenatal diagnosis it is possible to make a diagnosis of duodenal atresia with high degree of certainty through the radiological sign of “double bubble”, but up to date it is not yet possible to identify the type of duodenal atresia. We report the case of a patient with prenatal diagnosis of “double bubble”. The patient had no other concomitant malformations. Case Report The patient came to our attention after prenatal ultrasound that showed a picture of double bubble. At the 27th week of gestation we performed fetal MRI that confirmed the US pattern of double bubble but it did not identify with certainty the type of duodenal atresia. At birth the patient underwent GI rx examination that showed a picture of partial duodenal obstruction compatible with the Windsocks type. On the following day, we performed endoscopy which showed the presence of duodenal membrane, so the patient underwent surgical treatment with a longitudinal duodenal incision in order to treat the wind-sock membrane. After one month a further Upper-GI rx examination showed a regular transit of the contrast. Four months after the first operation the patient underwent new surgical treatment for bowel obstruction by adhesions. The operation was successful and the patient had a complete recover.Conclusions Patients with prenatal diagnosis of “double bubble” require a multidisciplinary approach for proper clinical management. Unfortunately it is not currently possible to identify with certainty by prenatal ultrasound the type of duodenal atresia, but in case of incomplete bowel occlusion , the possibility of an atresia Windsocks type should always be considered, especially for setting the right surgical approach.

Introduction: Hypospadias is one of the most common birth defects that affect the male urogenital tract. It can present as isolated anomaly, but sometimes can arise in the context of complex disorders of sex development (DSD). These malformations are characterized by a great variety of clinical manifestations and compromise the aesthetic appearance, but also functional and psychological impact that the malformation can determine the patient. Materials and Methods: We conducted a retrospective study of patients undergoing surgery for hypospadias from March 2000 to January 2015. The data was extrapolated from a prospective database. It was considered for each patient: demographics; type of hypospadias; surgical technique; average age for surgery; intraoperative and postoperative complications (early and late). Duckett’s classification was used. Results: 343 urethroplasties were performed. 320 (93%) were primary urethroplasties and 23 (7%) reoperations in patients who had performed many other surgical procedures. 7 patients with megameatus were excluded. The hypospadias have been ranked according to Duckett’s classification, 35 patients had associated diseases. In total were performed: 186 (55%) Snodgrass, 71(21%) Duckett,10 (3%) augmented Duckett, 42 (13%) Magpi, 16 (5%) Duplay, 1 (0,3%) Bracka, 1 (0,3%) was a Bianchi’technique and 5 (1,4%) were Standoli. In 4 patients (1%) were used mixed technique. There were no intraoperative complications. The mean age at surgery was 15 months (range 12-22 months). Postoperative complications were 12%. Long term follow up was done with uroflussimetrie at 3 and 6 months in those who had reached the continence and possible urethral calibrations in those who had submitted a stenosis in post-op.Conclusions: The improvement of surgical techniques, the use of optical amplification tools, the use of suture material (PDS) and the experience gained in recent years have enabled us to optimized the results. Though aware of the potential and actual complications that this type of microsurgical correction can lead to the results we have obtained are comparable to those of major international series and can be considered satisfactory, both from an aesthetic and functional.

Bladder exstrophy and epispadias are severe congenital anomalies associated with an open bladder and urinary sphincter. Despite modern reconstruction, there is a significant incidence of residual or recurrent urinary incontinence that impacts on quality of life (QoL) and self-esteem, which in turn limits social interaction (Figure). The present study involved 14 patients, mainly from a Middle Eastern country, and reported the early findings with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique for both urinary and faecal control.Fourteen children, with a median age of 8.1 years, with poor quality of life and low self-esteem because of urinary incontinence and small polypoidal open bladders of 5-15 ml volume, mostly after bladder exstrophy surgery, were managed with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique keeping an intact anal sphincter. The retrorectal pulled-through colon was anastomosed to the posterior wall of the rectum just above the external sphincter complex, thereby avoiding any possible injury to the anal sphincter. All patients had a normal colon and a competent anal sphincter without lumbosacral spinal or nerve anomalies.Ten children had a 5- to 10-year follow-up, one child had a 15-year follow-up, and three others, that were also continent, were excluded because of a5-year follow-up. There were no postoperative complications, and all were dry and odour-free by day within 2-4 weeks of surgery. Two children still had minor urinary loss at night. There were no UTIs and renal function remained unimpaired. Eleven years after surgery, one child underwent excision of a pedunculated benign inflammatory polyp from the tip of the left ureter because of recurrent torsion and bleeding, there was no recurrence at the 2-year follow-up. None of the rectal or ureteric biopsies from any of the children showed metaplasia or neoplasia; however, in view of the potential long-term risks, all children were placed on a lifelong 'proctoscopy and biopsy' protocol.The ability to be dry and odour-free, and to wear normal clothing had a striking impact on QoL and psychological well-being of the children and their families. This was reflected in their positive overall approach, voluntary school attendance, and enthusiastic participation in communal events. All agreed that their improved genital appearance markedly contributed to their better body image and increased self-esteem.These significant benefits, at a crucial time in the child's life, outweigh the potential risk of long-term neoplasia. Therefore, the Heitz-Boyer-Hovelacque rectal bladder technique is recommended with long-term proctoscopic follow-up.

Achalasia is a rare neuromuscular esophageal disorder in children. There are many surgical options to treatment including botulinum toxin (Botox) injections, oral pharmacologic therapies with nitrates and calcium channel blockers, pneumatic dilation (PD), and surgical myotomy (open surgery, endoscopy, laparoscopy and recently robotic approach). In pediatric age, usually, Heller's myotomy is the main choice. Laparoscopic approach is known and standardized. Few robotic have been published. We decided to report our first case to share our experience with scientific community. Keywords: Achalasia, Child, Dysphagia, Myotomy, Robotic surgery

The purpose of this study was to compare prognostic differentiation performances of the 7th and the 8th edition of American Joint Commission on Cancer (AJCC) staging system for gastric cancer (GC) patients.A total of 1,633 GC patients who underwent curative D2 resection followed by adjuvant chemotherapy alone (CA) or concurrent chemo-radiotherapy (CCRT) from 2004 to 2013 were included. Concordance index (c-index) was applied to compare the discriminatory ability.In the 8th edition, migration of stage was detected in 248 patients (15.2%). Among them, 121 patients were up-staged while 127 patients were down-staged. Overall, there was no statistically significant difference in the discriminatory ability between the 7th and 8th editions. The new edition of staging system, however, showed a trend of better prognostic performance not only in recurrence-free survival (c-index=0.734; 95% confidence interval [CI], 0.706 to 0.762 in the 7th edition vs. c-index=0.740; 95% CI, 0.712 to 0.768 in the 8th edition; p=0.14), but also in overall survival (c-index=0.717; 95% CI, 0.688 to 0.745 in the 7th edition vs. c-index=0.722; 95% CI, 0.694 to 0.751 in the 8th edition; p=0.19), especially in stage III. This finding was repeated in the subgroup analysis regardless of adjuvant CA or CCRT.Generally, the 8th edition of AJCC staging system had failed to show a superior discriminatory ability for curatively D2 resected GC patients than the 7th edition, although there was a trend of better prognostic performance of the new edition, regardless of adjuvant treatment method.

‘Caudal duplication syndrome’ was coined to describe the apparent duplication of organs derived from the hindgut, the neural tube and the adjacent mesoderm. Review of the anatomy suggests that the word ‘duplication’ may be a misnomer. This paper describes the management of 2 girls with caudal duplication syndrome who underwent multistage reconstructive surgery. Both had a large omphalocele and a severe diastasis of the pubic symphysis. The first patient also had an apparent duplication of the vulva, the perineum and the anus to either side of a wide midline. Each vulva contained a urethra, a hemi-clitoris with ipsilateral labium minor, and a hemi-vagina with hemi-uterus. The second child had an infrapubic sequestrated appendico-cecal duplication lying between two hemi-bladders each with ipsilateral ureter and urethra. The everted duplication split the single vulva longitudinally in the midline as far as the fourchette. To each side were a hemi-clitoris, and a hemi-vagina with hemi-uterus and ipsilateral fallopian tube. Analysis of our patients' anatomy and a literature review indicates for the most part ‘hemi’ organs on either side and suggests that the term ‘duplication’ is a misnomer such that caudal ‘split’ syndrome may be a more appropriate title.

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.

The appendix carcinoid, although rare (with an incidence of 0,08%), is the more frequent gastrointestinal tumor in children and teen. In most cases these tumors cannot be diagnosed or they can be incidentally found during a surgical exploration for acute abdomen. Only in few cases there are symptoms of a carcinoid’s syndrome (flushing, diarrhea and wheezing). In this work we present the manegement of a patient with an appendix carcinoid revealed during an appendicectomy performed for a peritonitis caused by a perforated appendicitis, underlining the importance of diagnosis and long term follow-up.

Introduction. Congenital diaphragmatic hernia (CDH) is still today considered a challenge from surgeons. Considerable progress in prenatal diagnosis, intensive care unit of neonates and surgical techniques, with the possibility to perform minimally access surgery, widely increased survival rates. The aim of this study is to analyze our series about long and short-term outcomes, also considering the progress made by minimally invasive techniques. Methods. The study was performed at Pediatric Surgery of Siena. It is a retrospective study that analyzed all patients with CDH (Bochdalek) treated in the last 14 years, from 2000 to 2013. Sex, side of the defect, presence of prenatal diagnosis, age of onset and symptoms, associate malformation, herniated organs, surgical technique and site of surgery, complications, recurrences, survival and followup were analyzed. Results. We included 23 patients. Five of them, were ruled out because of affected by diaphragmatic eventration or acquired diaphragmatic hernia. Forteen patients (77%) presented left CDH and 4 patients (23%) a right one. The male female ratio were 14:4. Prenatal diagnosis was performed in 5 patients (27,5%) at a mean gestational age of 29 weeks. Forteen patients (77%) had an early onset of symptoms (first day of life). Most common symptoms were respiratory distress and cyanosis; 4 patients (23%) had a late onset of symptoms, at a mean age of 9 months, and most common symptoms were failure to thrive and vomiting. Seven patients (39%) had associated malformation: common mesenterium (5 pts-71%). The colon was the most commonly herniated organ, present in 15 patients (83%), followed by small intestine in 13 patients (72%), stomach in 11 patients (61%), spleen in 9 patients (50%) and liver in 4 patients (23%). Seventeen patients (94,5%) underwent open surgery: 10 of them (59%) underwent a subcostal laparotomy approach, 7 of them (41%) underwent a supraumbelical laparotomy approach; 1 patient (5%) underwent minimally access surgery with thoracoscopy access. We performed performed surgery in the intensive care unit in 3 patients (16,5%). Six patients (33%) developed minor postoperatory complications. No patient had recurrence. Four patients died so we report a mortality rate of 23%. A follow-up investigation, with an average duration of 87 months, it is still going on in 5 patients (27,5%). Conclusions. The our survival rate was 77% and it reflects the encouraging reported data in the recent literature. These results are due to the reliability of the new resuscitation strategies, such as high-frequency oscillatory ventilation and the use of NO, the ability to perform surgery in the neonatal intensive care unit and, especially, to successfully perform minimally invasive surgery in newborn. The improvement of the survival showed the increasing of long-term morbidity end the requirement of a multidisciplinary followup. For these reasons, a multidisciplinary pathway for the management of young patients has been created, to follow them in a standardized way as early as the prenatal diagnosis.

Adrenocortical neoplasms (ACNs) are rare and poorly characterized in infants. The true incidence of ACNs is not well known and it appears to vary substantially across different geographical areas. ACNs are more common in females and two peaks of incidence have been identified: The first year of life and between the age of nine and 16 years. Due to the heterogeneity and rarity of ACNs, their pathological and prognostic classification is challenging. The current study describes the case of a seven-year-old male, who presented to the Department of Pediatric Surgery, University of Siena (Siena, Italy) with a feminization syndrome and increased somatic growth that was associated with a unilateral adrenal mass, which was diagnosed by magnetic resonance imaging. Surgical excision of the mass was performed and histological analysis determined that it was an ACN, with a low risk of malignity; however, the pathological classification of the tumor was challenging. At present, the future behavior of ACNs is unpredictable. Therefore, increasing the knowledge surrounding this type of tumor may aid in its diagnosis, treatment and prognosis. Due to the rarity of pediatric ACNs, no single pediatric oncology center has acquired extensive experience treating this type of tumor. Thus, the initiation of an international tumor registry may aid with the management of patients presenting with ACNs.

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).

Burkitt’s lymphoma (BL) is a high-grade lymphoma which represents 8-10% of all tumors in individuals younger than 15 years old. It may occur as an abdominal tumor, which in rare cases may include gastric mucosa, although in most cases, the tumor is located in the ileum or cecum. Primary gastric lymphoma constitutes as low as 1.48% of all gastric cancers in children. In this study, we described a case of gastric BL in a 4-year-old child, presenting discontinuous abdominal pain, weight loss, constipation and irritability. Despite its rarity in children, this tumor should be treated aggressively and long-term survival has been reported.

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.