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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

Multiseptate gallbladder (MSG) is a rare congenital anomaly. MSG may be accompanied with other abnormalities of the biliary tree. MSG can be asymptomatic and most cases are detected incidentally. Nevertheless, some patients present with biliary pain or colicky abdominal pain due to biliary stasis and calculi. We report a case of MSG in a girl who presented with recurrent abdominal pain and normal laboratory work-up recovered after laparoscopic cholecystectomy. A pediatric literature review of this rare anomaly is also performed. Keywords: Multiseptate gallbladder, Laparoscopic surgery, Children

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.

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.

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.

Anxiety in children undergoing surgery is characterized by feelings of tension, apprehension, nervousness and fear which may manifest differently. Postoperative behavioural changes such as nocturnal enuresis, feeding disorders, apathy, and sleep disturbances may stem from postoperative anxiety. Some Authors pointed out that over 60% of children undergoing surgery are prone to developing behavioural alterations 2 weeks after surgery. Variables such as age, temperament and anxiety both in children and parents are considered predictors of such changes.1 Studies were published describing how psycho-behavioural interventions based on play, learning and entertainment in preparing children for surgery, may reduce preoperative anxiety. Clown-therapy is applied in the most important paediatric facilities and has proved to diminish children’s emotional distress and sufferance, as well as consumption of both analgesics and sedatives and to facilitate the achievement of therapeutic goals. The aim of our study was to evaluate the efficacy of clown-therapy during the child’s hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the child’s overall life quality.

We report our series of selected patients with complete incontinence in whom the appendix was divided and utilized for creating two continent catheterizable stomas. All patients were treated for urinary and fecal incontinence by split appendix technique. The appendix was divided into two different parts preserving adequate perfusion and used for creating an appendicocecostomy (ACE) and an appendicovesicostomy at the same time. After a clinical and radiological follow up, our patients referred a good acceptance and an easily management of the stomas in order to stay dry for all day from urine and feces with improving of their quality of life. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry.

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.

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.

In this study we want to demonstrate the effectiveness of the esophagogastric dissociation as a first level operation in treatment of the gastroesophageal reflux in severe neurologically impaired children, in term of a reduction of reoperation rate.We divided patients operated from 1998 to 2005 in a group A, composed by children treated with fundoplication, and in a group AR, composed by the patients of group A who had a recurrence of reflux and that was treated with esophagogastric dissociation. Patients operated from 2005 to 2013 were selected on the basis of the severity of the neurological impairment and were divided in a group B, treated with fundoplication, and in a group C of more severe impaired children, treated with esophagogastric dissociation. Data regarding the complications of the A and C groups were analyzed with Fisher's test.We evaluated 63 patients: 34 (54%) in group A, 11 in group AR, 15 (23.6%) in group B, 14 (22.4%) in group C. The Fisher's test showed a non significant difference with a p value of 0.2.Despite of statistic result we believe that TOGD is a useful procedure as the first choice of surgical management in severe neurological impaired children affected by gastroesophageal reflux.

Mycobacterium triplex was first described in 1996. This nontuberculous Mycobacterium causes a severe pulmonary disease in immunocompromised patients but it can involve also healthy patients. A literature search was made on the PubMed database and it produced only few cases of children with cervical lymphadenitis due to this Mycobacterium Triplex. We are describing a case of M. triplex cervical lymphadenitis in an immunocompetent child.

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.