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The aim of this study is to present our preliminary experience in robotics and a comparative analysis with conventional Minimally- Invasive Surgery (MIS). Cases operated by da Vinci Xi® System from February 2016 to October 2017 are reviewed retrospectively through demographics, diagnosis-procedure and short-term outcome parameters. A comparison with a matching conventional MIS population was also conducted. 40 robotic procedures were carried out and 112 (out of 3705) non-robotic procedures met inclusion criteria for comparison. Among robotic patients we observed: an average age of 143.5 months, weight of 42.9 Kg, operative and anaesthesia induction time respectively of 116.8 and 34.8 minutes. Furthermore, we observed a 6.1-day length of stay, 2.5% conversion rate and no complications. From the comparison between the groups, no statistical difference emerged in the length of stay, in conversion rates or in complications. A statistical significance was observed in terms of operative time in favour of non-Robotic- System. Our experience has meant to introduce the System in our surgical environment, comparing to the conventional MIS (an already established approach routinely performed at our center). Results have shown comparable safety and feasibility.

Bochdalek hernia (BH) is a major malformation that consists of a postero-lateral diaphragmatic defect through which abdominal contents can migrate into the thorax. It is most commonly a neonatal pathology associated with significant morbidity and mortality. Rarely, this type of congenital diaphragmatic hernia (CDH) presents later in life, some even in adulthood. Indications for surgery are not clear even though the majority of authors, being aware of the possible severe complications, recommend surgical correction in all cases. Many surgical approaches have been tried for this pathology, both open (laparotomic or thoracotomic) and minimally invasive (laparoscopic or thoracoscopic). We report a case of a late-onset BH corrected with a robot-assisted thoracoscopic procedure. The use of robotic technology for CDH has never been described to date.

Paediatric foreign body aspiration can be related to a high morbidity/mortality rate, especially in young children, and pneumothorax can be a severe associated issue. We describe a case of a 13-year-old girl with recurrent pneumothorax in bilateral bullous lung, twice previously treated at our institution by robotassisted bullectomy, and once again showing same signs and symptoms. At conventional thoracoscopy an inorganic foreign body was found to be located pointing upwards the apex. We believe this may have acted as a contributory cause in the persistence of the clinical picture, despite the surgical removal of the underlying pathology.

Purpose: The spectrum of male urethral duplication is heterogeneous and it includes the Y-duplication. The malformation is rare and there is only a few case series reported in the literature. The management of Y-forms remains challenging for the surgeon and the long-term follow-up is still scarcely investigated. We report our 40-year experience in the management of patients with Y-duplication., Materials and Methods: We conducted a restrospective analysis collecting information of patients with urethral Y-duplication treated at our department from April 1975 to April 2015. We investigated long-term effects of surgery by using a questionnaire., Results: Ten male patients with Y-duplication came to our attention. One was treated conservatively, seven underwent surgery and two were lost. Surgery consisted of removal of the ectopic branch (via perineal or ASTRA/anterior sagittal trans-rectal approach approach) and reconstruction of the orthotopic urethra. Post-operative complications included stenosis and infections. Long-term results are influenced by associated anomalies and significant problems (incontinence, urinary tract infections and orchiepididimitis) have been reported., Conclusions: Y-duplication (or λ-duplication, as we prefer calling it) is a particular form of urethral duplication. The management of patients should be based upon the identification of the functional channel. The removal of the ectopic channel with ASTRA approach is safe and feasible. On the other hand, the reconstruction of the anterior urethra (when steno-atresic) is more challenging and justifies the need for many procedures. The P.A.D.U.A. (progressive augmentation by dilating the anterior urethra) technique was not effective. Skin tube grafts were responsible for infections ("hairy urethra"). BMFG (bladder mucosa free graft) urethroplasty is a good alternative, although associated with well-known complications. Associated anomalies influence long-term outcomes. Clinical study with type IV level of evidence., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Persistent urogenital sinus (UGS) is a developmental anomaly. It represents one of the most complex problems that a pediatric surgeon may deal with. We report the case of a patient with UGS treated at 3 years of age by anterior sagittal transrectal approach and en bloc sinus mobilization. The procedure was performed with the patient prone with the initial idea of performing an anterior sagittal transrectal approach. The described approach allows an excellent anatomical view with a midline muscle sparing incision, along with an easy identification of the vaginal confluence with the benefit of avoiding dissection between the urethra and vagina.

Duplications of the urinary collecting system and pyelo-ureteral junction obstruction (PUJO) are common, but the simultaneous presence of both anomalies is rarely encountered. In incomplete duplicated systems, PUJO usually affects the lower moiety. We present the case of a 5-year old boy with left bifid renal pelvis and hydronephosis of the lower moiety treated by robot-assisted pyeloureterostomy.

Choledochal cyst (CDC) is a congenital dilatation of the extra and/or intrahepatic bile ducts and it is a rare condition in western countries. Classical treatment consists of cyst excision and hepaticojejunostomy. The first case of a laparoscopic CDC excision was described in 1995 and since that time an increasing number of institutions have adopted this technique, with good success. We describe our early experience of 3 cases of CDC treated with laparoscopic approach. We used a 10 mm umbilical port for the camera, and four 3-5 mm operative ports. We performed the laparoscopic removal of the cyst and gallbladder, videoassisted preparation of the Roux-en-Y loop and laparoscopic hepaticjejunostomy. No post-operative complications occurred. Laparoscopic excision of CDCs has been supposed to give better observation, a better cosmetic result, potentially less postoperative pain, and a shorter recovery. The main argument for performing an extracorporeal anastomosis is that it decreases the operative time. We recommend caution to prevent injury to the pancreatic duct and biliary structures during dissection and anastomosis. Lifelong surveillance is mandatory, even after resection of the choledochal cyst.

Purpose: To report our 14 years experience with the laparoscopic-assisted anorectal pull-through (LAARP) for the treatment of male neonates with high imperforate anus., Materials and Methods: We reviewed all medical charts of patients who underwent LAARP from January 2001 to January 2015 collecting information regarding demographic data, associated anomalies, type of fistula, pelvic floor muscles tropism, surgery (operative time, conversion to open technique, and complications), and follow-up. Follow-up data were obtained through the use of validated questionnaires that aim to assess the intestinal function in terms of constipation and continence., Results: At the Department of Pediatric Surgery of the University of Bologna, 13 male patients were operated in the study period (our protocol consisted of colostomy at birth followed by delayed LAARP). Mean age at operation was 4.75 months (range 1-14). There was one conversion to open technique due to a strong tension from the colostomy (this patient was excluded). Functional results were acceptable at a minimum 2-year-long follow-up., Conclusions: LAARP is a good choice for the correction of the high imperforate anus. Short-term outcomes are similar to those after posterior sagittal anorectoplasty (PSARP). Long-term outcomes should be better assessed.

Introduction: Ureteropelvic junction obstruction (UPJO) is the most common pediatric obstructive uropathy. We report our 8-year experience with the use of a retroperitoneal laparoscopic-assisted pyeloplasty (one-trocar-assisted pyeloplasty [OTAP])., Materials and Methods: This is a retrospective analysis of 88 patients treated in 8 years. Surgery was performed on the basis of standard indications and it consisted of 88 OTAP (the ureteropelvic junction is identified through a retroperitoneoscopic inspection and it is exteriorized to perform a traditional dismembered pyeloplasty). Operative time, hospital stay, complication, success of the technique, and aesthetical results are analyzed., Results: In the 8-year period we performed 88 OTAP. Mean age was 19 months. Prenatal diagnosis was the most common presentation (n = 73), followed by occasional identification (n = 8), and symptomatic (n = 7). Mean operating time was 139 minutes (range 60-225 minutes). Conversion was required in seven cases. The success rate was 87.5% (reduction of the severity of hydronephrosis on ultrasound) and 100% among symptomatic patients (symptoms resolution). Aesthetical results were excellent., Discussion and Conclusions: OTAP is safe, feasible, and efficacious. Operative times are similar or even shorter than those of other minimally invasive techniques and complication rate agrees with the findings of literature. OTAP can be considered a reasonable alternative., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: Gastrointestinal stromal tumors (GIST) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract, accounting for 0.1% to 3% of all GI malignancies. Paediatric GIST have an annual incidence of 0.02 per million children, have a female predisposition, are usually located in the stomach (50-60%) and in up to 85% of cases CD117-cKit or PDGFRA mutation is absent, resulting in a decreased efficacy of the target therapy., Case Description: We report the case of an incidentally diagnosed gastric GIST in a 14-year-old boy with multiple malformations. Genetic tests and Kariotype resulted negative. Recently, an abdominal US visualized an hypoechoic heterogeneous abdominal mass. The common tumor markers resulted negative and the abdominal CT-scan confirmed the presence of a solid round lesion (42×36mm) in contact with the stomach and the pancreas. Laparoscopy allowed the recognition and the removal of the nodular mass at the posterior margin of the stomach. The histopathologic and the molecular biology findings were consistent with a kit-wilde type GIST. Surgical margins were microscopically free of tumor cells. These results justify the decision not to add other surgical or medical therapy. However, for high risk of recurrence and metastasis, a close follow-up was started., Discussion and Evaluation: GIST are asymptomatic in 10% to 30% of patients or present nonspecific symptoms and signs. These tumors present usually irregular, lobulated and ulcerated. CT-scan of the abdomen and pelvis or magnetic resonance imaging (MRI) are mandatory in the diagnostic work-up. The final diagnosis is based on histology and immunohistochemistry. Surgery is the first-line treatment in patients with localized disease., Conclusion: Guidelines for the management of pediatric GIST are not presently available for the paucity of reports and data. However it is widely accepted that surgery is the first-line treatment and gross resection with negative microscopic margins can be considered therapeutic and lead to full remission of the pathology. Laparoscopy is a safe surgical approach for the exploration of the abdominal cavity, the evaluation of the disease and the complete removal of the tumor.

Introduction: Partial splenectomy in children is a good surgical option for hematological diseases and focal splenic tumors because it allows the preservation of the spleen's immunological function. Furthermore, it can be performed by laparoscopy in children as it is a safe procedure, offering the benefits of a minimally invasive approach., Materials and Methods: The software VR-render LE version 0.81 is a system that enables the visualization of bidimentional 3D images with magnification of anatomical details. We have applied this system to five cases of non-parasitic splenic cysts before laparoscopic partial splenectomy., Results: The images obtained with VR rendering software permitted the preoperative reconstruction of the vascularization of the splenic hilum, allowing the surgeon safe vessel control during laparoscopic procedures. All five partial splenectomies were carried out with no complications or major blood loss., Conclusions: Laparoscopic partial splenectomy should be a first choice procedure because it is feasible, reproducible, and safe for children; furthermore, it preserves enough splenic tissue thereby preventing post-splenectomy infections. Volume rendering provides high anatomical resolution and can be useful in guiding the surgical procedure., (© 2013.)

Purpose: Congenital lung malformations (CLM) are traditionally treated by pulmonary lobectomy. The foremost indication for surgery is that these lesions predispose to respiratory tract infections and to malignancy. Owing to the improvement of prenatal diagnosis, most patients are operated in a timely manner and prophylactically. In this context, lung-sparing surgery (LS) has gained interest as a lung preservation strategy, especially for asymptomatic patients. In the present study, we evaluated both thoracoscopic and open lung-preserving resections as an alternative to lobectomy for CLM resection., Methods: We retrospectively reviewed all patients who underwent lung-sparing resection (segmentectomy and atypical resection) for CLM from 2001 to 2010. Data were collected regarding preoperative diagnostic workup, type of intervention, and follow up., Results: Fifty-four patients received LS for CLM during the study period. Twenty-six were approached thoracoscopically, with 18 cases requiring open conversion for a complete resection. There were six postoperative complications: three asymptomatic pneumothoraces that resolved without intervention, one tension pneumothorax that required replacement of a drainage catheter, and three instances of intra-operative bleeding requiring blood transfusion. Mean duration of follow-up was 65.2 months. Two patients experienced pneumonia during the follow-up period. A third patient had a cystic lung lesion on postoperative computed tomography (CT) which required a second-look surgery., Conclusions: LS for CLM is a safe and effective means of lung parenchymal preservation in pediatric patients. Complication rates are comparable to that of traditional lobectomy. In our experience, this type of lung surgery does not carry a higher risk of residual disease and recurrence if accurately planned in selected patients, i.e., those with small asymptomatic lesions. The complication rate is acceptable and apparently not affected by preoperative symptoms. The thoracoscopic approach is recommended, although open conversion should be advocated to avoid too long operative times., (Copyright © 2013. Published by Elsevier Inc.)

Introduction: Pectus excavatum is the most common congenital malformation of the anterior chest wall. The purpose of this study is to assess the role of thoracic ultrasound studies in the preoperative workup of patients affected by pectus excavatum and to identify the dynamics of the chest wall., Materials and Methods: An observational study was carried out between January and September 2011. Patients between 4 and 14 years of age were divided into 5 study groups. Group A: healthy patients without pectus excavatum; Group B: healthy patients with different grades of untreated pectus excavatum; Group C: patients with pectus excavatum treated with a Nuss bar; Group D: patients surgically treated with removed bar; Group E: patients surgically treated with different techniques., Results: Patients with deeper anatomical depression showed a differential value between maximum inspiration and forced expiration lower than healthy patients or patients with shallower depression (p<0.05) in any age range considered. A depression deeper than 2.8 cm was associated with lower elasticity of the chest wall., Conclusions: Study results demonstrate that the ultrasound is useful in patients with PE. Patients with pectus excavatum have altered chest dynamics when compared to healthy patients. The study also demonstrate that between the 4th and the 6th ribs there is the great dynamicity of the chest wall., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Although it is virtually impossible to formulate a scheme that can satisfactorily collect all different types of vaginal malformations, a simple classification would be of a considerable value and would permit logical operative decisions. Many classifications of anomalies of uterus and vagina have been proposed: we find them unsatisfactory and confusing, being either too simple or too complex. We propose a new classification, focused only on vagina and based on embryological, anatomical, clinical and surgical criteria., Methods: In over 30 years, 167 females with vaginal malformations have been observed in our department. Intersex cases were excluded. The encountered anomalies have been divided into six types and ten subtypes: type I vaginal agenesis (IA associated with uterine agenesis/17 Pts, IB isolated/1 Pt); type II vaginal atresia (IIA proximal/1 Pt, IIB distal/4 Pts); type III vaginal atresia with urethrovaginal fistula-urogenital sinus (IIIA proximal fistula-high sinus/42 Pts, IIIB distal fistula-low sinus/55 Pts); type IV vaginal atresia with transverse septum (IVA transverse septum/6 Pts, IVB imperforate hymen/17 Pts); type V disorders of müllerian ducts fusion (VA vaginal duplication/4 Pts, VB longitudinal septum/4 Pts); type VI cloaca/16 Pts. Of each type and subgroup of malformation the appropriate surgical correction is reported. Types of malformation and surgical treatment are analysed: IA vaginal reconstruction using a sigmoid conduit, IB vaginal reconstruction using a sigmoid conduit, atresic cervical resection and uterus-new vagina anastomosis according to Schmid; II perineal vaginal pull-through; IIIA anterior sagittal transanorectal vaginal pull-through, IIIB perineal flap vaginoplasty; IVA excision with abdominovaginal approach, IVB hymen incision; VA tubularization, VB septectomy via perineal approach; VI posterior sagittal anorectal-vaginal-urethroplasty., Results: Most of the patients had good aesthetic and functional results. Type III showed relatively more complications: four redo operations (IIIA), four revisions of the vaginoplasty (IIIB). One patient is still waiting for definitive surgical correction., Conclusions: An early diagnosis is desirable to correct adequately vaginal malformations, which becomes mostly evident around puberty. The better timing for surgery is early age, to obtain better results and to avoid many of the psychological problems that arise at a later age.

Background: Laparoscopic nephrectomy in children has gradually become a reasonable alternative to open nephrectomy and, besides, a retroperitoneal approach seems more logical than transperitoneal approach to perform nephroureterectomy for benign disease, as in open surgery. To further reduce the access-related complications, we propose a retroperitoneal one trocar-assisted nephrectomy (OTAN). We report our experience with minimally invasive OTAN for the treatment of benign renal disease., Methods: A total of 27 OTANs were performed at our institution between 2003 and 2009. The median patient age was 7.6 (range, 2-32 months). Indications for unilateral nephrectomy were multicystic dysplastic kidney (MCDK) in 23 cases (85.2%), dysplastic kidney in 3 cases (11.1%), and reflux nephropathy in 1 case (3.7%)., Results: The median operative time from the initial incision to skin closure was 60 min. Perioperative transfusion was not required in any cases. No major perioperative complications developed. Conversion to open surgery was necessary in four cases (14.8%): in three cases for a small working space arising from a peritoneal perforation, and in one case for a difficult visualization of the parenchyma (renal fusion not detected by preoperative ultrasound evaluation). Most patients were allowed oral intake on postoperative day 1. The median hospital stay was 2 (range, 2-3) days; the cosmetic results were excellent. Convalescence was uneventful in all patients., Conclusions: Although the indications for a nephrectomy in case of benign disease remain limited, when a little child has small, poorly functioning kidneys that must be removed, a one trocar-assisted approach for nephrectomy is usefully a safe and effective treatment choice. The procedure can be easily performed through a small incision with minimal morbidity, comparable operative time, and excellent cosmesis without excessive postoperative pain issues, allowing early discharge home.

Background/purpose: The absence of vagina is rare in the pediatric population. It can occur as a result of congenital malformations such as an aplasia of mullerian ducts (46,XX Mayer-Rokitansky-Küster-Hauser syndrome) or a complete androgen insensitivity syndrome (46,XY testicular feminizing syndrome). Intersex patients, who underwent reassessment of a female sex, need a genital reconstruction toward a feminine phenotype. Patients with congenital adrenogenital syndrome with high urogenital sinus could have a severe hypoplastic vagina. In all these cases, a vaginal replacement is required. We reviewed our experience of vaginal replacement using a sigmoid conduit., Methods: In 34 years, we evaluated 47 patients. The observation period was from 1 to 34 years (mean: 12 years). The preoperative diagnosis was Mayer-Rokitansky-Küster-Hauser syndrome in 17 cases, androgen insensitivity syndrome in 24 cases, adrenogenital syndrome with high urogenital sinus in 5 and 1 patient was affected by penile agenesis. Forty-six patients were treated with vaginal reconstruction by interposition of sigmoid colon. Only in 1 case we performed a vaginal construction with an ileal loop: in this case, the sigmoid colon was extremely dilated by a chronic constipation secondary to a high anorectal malformation corrected at birth., Results: The outcome for 47 patients is excellent: 18 are sexually active and 4 are married. Only 1 patient with adrenogenital syndrome died of endocrine problems. Complications occurred in 17 cases: in 1 patient a necrosis of the replaced vagina occurred, thus requiring vaginal exeresis; now she is waiting for a second operation. Another patient had an abdominal abscess, which was surgically treated. In 12 cases a second procedure was required: 6 had stenotic new-vaginal introitus, 4 had new-vaginal prolapse, and 2 had intestinal obstruction., Conclusions: We believe that the preferable technique for vaginal replacement is the use of intestinal conduit. The sigmoid colon is the best intestinal tract to be used owing to its size, location and preserved blood supply. Our experience leads us to believe that the sigmoid segment is the segment of choice, although we consider ileal loop as a good alternative when the sigmoid colon is not available., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: Fetal and neonatal hepatic arteriovenous fistulas are rare and associated with a high mortality rate; they can be prenatally detected by ultrasonography. Management of these malformations can be a challenge for pediatric surgeons., Methods: Two patients with a prenatal diagnosis of intrahepatic arteriovenous shunts were treated at our institution in the last 2 years. A hepatic complex arteriovenous malformation fed respectively by prominent branches of the hepatic artery and of the celiac trunk rising from dilated suprarenal aortae and draining into suprahepatic veins was detected. In the first case, an embolization was performed; in the second, the surgical resection of the vascular malformation was the treatment of choice., Results: The first patient died after embolization and before surgery for hemodynamic complications. The second patient, at a follow-up of 16 months, is alive and doing well., Conclusion: Hepatic resection is the treatment of choice for localized intrahepatic arteriovenous malformation. Theoretically, embolization could be curative or reduce the size of a malformation, making consequent hepatic resection feasible. Results do not support this theory because of the high rate of complications recorded that brought in every case, ours included, to the death of the child.

The risk of sudden death in patients with Brugada syndrome (BS) is still unclear. Moreover, particular clinical conditions may have a confounding effect on the diagnostic and therapeutic approach. We report the case of a 27-year-old man with a clinical history of suspected neurally mediated syncope and typical ECG features of BS. The tilt table test showed a type I, mixed, positive response. The electrophysiological study (EPS) disclosed a peculiar ventricular irritability with the induction of a life-threatening arrhythmia. After the implantation of a cardioverter-defibrillator an episode of ventricular fibrillation during sleep at night was correctly identified and treated by the device. The association between neurally mediated susceptibility and the typical ECG abnormalities of BS is not an unexpected event in young subjects. The misjudgment of the pathophysiological mechanism of syncopal episodes may lead, on one hand, to overlook the risk of sudden death and, on the other, to pursue inappropriate therapeutic measures. The application of a tailored diagnostic work-up based on currently available guidelines may be useful to overcome the clinical and therapeutic dilemma.

Purpose: Müllerian duct remnants (MDRs) are present in a male pseudohermaphroditic form characterized by failure of the müllerian duct to regress due to insufficient production or peripheral action of müllerian inhibiting substance. The MDR can be asymptomatic but it often results in infections, stones and voiding troubles. Furthermore, it may develop into a neoplasm. Therefore, surgery is mandatory for large MDRs and symptomatic patients. Laparoscopic removal is described., Materials and Methods: Six males were treated from February 1998 to February 2003. Age at surgery was between 3 and 18 years (mean 8.6). All patients showed severe hypospadias and 2 had mixed gonadal dysgenesis with ambiguous genitalia. Three patients presented with urogenital infections and all had a large MDR. Laparoscopic procedures, which were preceded by cystoscopy, were performed using a 10 mm umbilical trocar for the camera and 3, 5 mm trocars for instruments placed in the suprapubic region and iliac fossa bilaterally. The remnants were ligated with endoscopic loops or an endoscopic GIA stapler and cut., Results: Mean operative time was 2 hours. We noted no complications. In 2 cases there was deferential ectopia and in another of mixed gonadal dysgenesis bilateral gonadectomy was performed because of the risk of degeneration. Feeding started on postoperative day 1 and the patients were discharged home on day 5. After a followup of 8 months to 4 years all boys were healthy., Conclusions: Multiple approaches are used in traditional surgery, often leading to complications. Laparoscopy improves the view, decreases surgical risk and operative time, avoids large scars and allows more rapid hospital discharge.