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The term intussusception refers to the invagination of the part of the intestine into the adjacent segment. It is one of the most common causes of acute abdomen in children. Idiopathic intussusception is more frequent within the first 2 years of age with most cases presenting at around 6 months of age. Symptomatic intussusception with a pathologic lead point can occur at any age, especially in older patient. Idiopathic intussusception seems to be related to an intestinal dysmotility after weaned or gastrointestinal/respiratory infections. The main symptoms are colic abdominal pain, vomit, and “red currant jelly stool.” Ultrasounds (US) are the first-level investigation for the diagnosis: the intussuscepted tract appears as two rings of low echogenicity with an intervening hyperechoic ring similar to a donut or a target. Contrast enema confirms the diagnosis and can be curative allowing progressive reduction of intussusception. In case of failure of the conservative treatment, a surgical approach is necessary. Classical operation is performed by laparotomy (small right-sided transverse incision). The intussusception mass is palpated and is brought outside the wound, and the reduction is attempted by exerting gentle and persistent pressure at its distal end. Laparoscopic reduction has been described as an effective alternative with reduced scarring, adhesions, pain, and hospitalization length although its role is still questioned. Delay in diagnosis is the main factor related to morbidity and mortality.

Summary Introduction Urethral duplication is a rare and variable malformation of the urinary tract, with non-univocal and complex management. In addition, different classification schemes have been proposed, but none have considered all the possible variants. Objective To report experience with the management of 20 urethral duplication patients and propose a classification of this anomaly. Materials and methods A retrospective analysis collecting information regarding 20 patients (Table) with urethral duplication treated at a single institution over the past 40 years. Three females and 17 males were treated: two had vesico-urethral duplication, eight had urethral duplication with a single bladder, and eight had ‘λ’ duplication. Results Immediate postoperative complications included urethral dehiescence (n = 1) and urethral stenosis (n = 2). The progressive augmentation by dilating the urethra (PADUA) technique was ineffective. During follow-up, the following were recorded: urinary incontinence (n = 2), urinary tract infection (n = 3), hypertension (n = 3) and erectile dysfunction (n = 1). All patients were satisfied with the aesthetic result. Discussion Urethral duplication is a rare anomaly with male preponderance. Four types of duplication were described, on the basis of anatomy and management: vesical and urethral duplication (type 1), urethral duplication with single bladder (type 2), ‘λ-type’ duplication (type 3) and ‘miscellanea’ (multiple urethral channels, spindle urethra, other female forms, type 4). A full description of the malformation and surgical approach was given for each type. The advantages of the classification were compared with the literature. Conclusions The proposed classification should be a useful tool, based on the required surgical approach, even if surgery should be tailored to the patient. It is important to restore the anatomy and achieve urinary continence. Surgery can be challenging and a multi-step process, especially in cases of ‘λ’ duplications. Table . Type Sex (n) Symptoms Surgery Immediate postoperative complications 1 F (n = 1), M (n = 1) Incontinence, asymptomatic Bladder unification + ectopic urethra removal + urethroplasty (n = 1); epispadic urethra and abortive bladder removal (n = 1) Urethral dehiscence (n = 1) 2A M (n = 4) Asymptomatic (n = 3), mucous discharge (n = 1) Conservative (n = 2); accessory urethra removal (n = 2) – 2D F (n = 1) Urge incontinence Vulvar urethrostomy and posterior channel endoscopic closure 2B M (n = 2) Mucous discharge Termo-ablation (n = 1), accessory channel removal (n = 1) None 2C M (n = 2) Incontinence, UTI Urethroplasy, ectopic urethra removal (n = 2) Urethral stenosis (n = 1) 3 M (n = 8) Anal voiding (n = 6), incontinence (n = 1), UTI (n = 1), anuria (n = 1) ASTRA + urethroplasty with BMFG (n = 3); ASTRA + urethroplasty (n = 2); perineal resection of the ectopic channel + urethroplasty (n = 1); vesicostomy (n = 1); perineal urethrostomy and PADUA (n = 2) Urethral stenosis (n = 3); terminal renal failure (n = 1) 2B F (n = 1) Double urinary stream Vulvar and clitoridoplasty, ectopic urethra removal None ASTRA, anterior sagittal transrectal approach; BMFG, bladder mucosa free graft; PADUA, progressive augmentation by dilating the urethra anterior; UTI, urinary tract infections.

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.

As the number of young girls subjected to chemo and radiotherapy is steadily increasing it is our duty to try to preserve their future fertility and ovarian function. A possibility is to collect ovarian samples, cryopreserve them and reimplant the tissue when the treatments are over. We report our 11 year experience with the use of laparoscopy to collect ovarian tissue samples. In particular we focus on the details of the surgical technique. The procedure was performed in 54 patients affected by malignancies or pathologies requiring chemo or radiotherapy, with good results. Cryopreservation seems to be a good option to preserve future fertility although it is still an experimental method.

Scimitar syndrome (SS) is a variety of congenital vascular abnormalities known as "partial anomalous pulmonary venous connections"(PAPVCs).In this anomaly, the venous return of a lung(more frequently the right one)drains into the inferior caval vein, the ipsilateral atrium, the coronary sinus, the azygos vein, portal vein or hepatic vein. SS is frequently associated with other anomalies:right lung hypoplasia(almost always present)with lobation defects, dextroposition of the heart,right pulmonaryartery hypoplasia(60%), sy stemic arterial blood supply to the lung(60%), atrial septal defect (ASD) (40%overall,80-90% in yhe infantile form), right posterior diaphragmatic hernia (15%)and horseshoe lung. The main clinical problem is the progressive development of pulmonary hypertension that may lead to heart failure. Definitive surgical treatment consists of rerouting the venous drainage. Many techinique have been proved but they all present technical difficulties and a high rate of postoperative complications such as stenosis of the scimitar vein. All medical and surgical measures aimed at controlling pulmonary hypertension can therefore be used to allow patient's grow and delay or avoidcardiac surgery. Among these,the closure of systemic arterial blood supply may constitute a valid and mininvasive opportunity. We report our initial experience in 4 pediatric cases. The first two cases were treated with percutaneous embolization and other two with thoracoscopic closure of the aberrant artery. In one case (treated with embolization), the patient dead for major cardiac malformation, while the other cases demonstrate hemodinamic stebility at follow-up.

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.

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.

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.

INTRODUCTION: We describe our experience with the use of a modified technique for laparoscopic internal inguinal ring closure (peritoneal incision and double "N" stitch placement). We evaluate the technique in terms of feasibility, efficacy, and safety. MATERIALS AND METHODS: From November 2003 to March 2014 we performed 1700 herniorraphies by laparoscopy. We selected 123 patients treated with the "double N" technique by the same surgical team, and we reviewed their notes analyzing demographic data, operative times, intra- and postoperative complications, and recurrence rate. The technique used is a modification of the Schier technique. Our technique consists of a partial lateral peritoneal 180° incision around the internal inguinal ring and in the placement of a double stitch. The first stitch is used to approximate the muscles with the inguinal ligament, and the second one is used to close the peritoneum above them. RESULTS: The female to male ratio was 22:101. Mean age at surgery was 4.3 years (range, 1 month-12 years). Mean operative time was 30 minutes (range, 20-50 minutes). There were 60 bilateral cases. There were no intraoperative complications. Two patients had reactive hydrocele treated conservatively, and 1 patient developed umbilical infection. We did not identify any recurrence. CONCLUSIONS: Our early results suggest that the "double N" laparoscopic technique to close the internal inguinal ring in children is safe and efficient. We therefore suggest using this approach in children with a patent internal inguinal ring of >1 cm. The recurrence rate is low, but it should be better assessed by studies with longer follow-up.

In questa pubblicazione, indirizzata alla consultazione del pediatra e dei giovani chirurghi pediatri, è raccolta l’esperienza maturata nella gestione delle malformazioni polmonari congenite. Queste malformazioni rappresentano uno spettro di anomalie la cui conoscenza si è ampliata negli anni grazie allo sviluppo di tecniche di imaging prenatale e di terapia fetale e postnatale. La valutazione prenatale permette di fare considerazioni prognostiche, di stabilire la necessità di intervento e di programmare il parto in centri di terzo livello. Dopo la nascita il trattamento chirurgico in molti casi è dilazionato ed è proposto frequentemente per via toracoscopica.

The article reports the case of an 11-year-old boy who presented with abdominal symptoms following the ingestion of three magnets. The history, the radiologic and endoscopic pictures gave the opportunity for some considerations about the peculiarities of the management of such dangerous foreign bodies. Surgery showed that the three magnets - one in the esophagus and the other two in the gastric fundus - attracted one another just above the esophagogastric junction causing an esophageal pseudodiverticulus. The magnets were removed without any further complication. Some recent statements on the management of ingested magnets are briefly discussed.

Minimally invasive surgery (MIS) in pediatric urology has gained a great interest in the past few years. Early uses of laparoscopy had diagnostic purposes and were related to the management of non-palpable testes (1970s). MIS is now used for more complex and challenging procedures, and it accounts for 10–15 % of all pediatric laparoscopic surgeries performed by urologists. The evolution of pediatric MIS is the result of recent advances in camera technology and instrumentation and of the recognition of its safety. The benefits of laparoscopic surgery include reduced postoperative pain and hospital stay, quicker recovery, reduced complication rate, and excellent cosmetic results. MIS achieves good outcomes and high acceptance between parents despite the fact that there are still controversies.

After the introduction of maternal US in the 1970s, many urinary tract anomalies are found in utero. On one hand this permits early diagnosis and treatment, avoiding debilitating sequelae to little children. On the other hand, there are many anomalies with no clinical significance and destined to disappear that are labeled as dangerous, creating a discomfort to the child and to the mother as well. The goal of the prenatal diagnosis is not only to obtain a differential diagnosis between pathologies that have similar elements on prenatal US but also to define the prognosis. The collaboration between specialists is fundamental for this purpose. In this context, the chapter takes into consideration various aspects of the prenatal diagnosis and perinatal management of urinary malformations.

The term cryptorchidism refers to the condition of abnormal testicular descent (TD). In this situation the testis is “hidden” and it cannot be identified in its normal position that is inside the scrotum. In fact the term “cryptorchidism” literally means “hidden testicle” and is often used interchangeably with the term “undescended testicle”. A normal testicular function is guaranteed by its full descent into the scrotum. Undescended testes carry the risk of malignant transformation and loss of fertility. It is thus mandatory to recognize this condition in order to plan the surgical correction.

Purpose We evaluated the results of esophagocoloplasty (ECP) in children by performing a 45-year retrospective cohort study in a single center. Materials and Methods We collected all of the medical charts of patients who underwent ECP at our hospital from January 1969 to January 2014. We reviewed the medical history for each patient and then contacted the patients by phone to obtain responses to a questionnaire. The questionnaire evaluated the following four areas: overall general state, gastrointestinal function, dependence on the Health Sanitary System, and their aesthetic satisfaction. Results We contacted 96 patients, and 72 completed our questionnaire. There were 45 males and 27 female respondents, with a mean age of 34.5years. The mean Karnofsky performance status index was 96.4%. There were excellent scores obtained from 55 patients and fairly good scores from 16 patients. There was 1 patient with a low score, and there were no scores reported below 70%. Most patients (58/72) reported regular bowel habits, and no patients required drugs or other measures to facilitate defecation. Gastroesophageal reflux was present in 51 patients, and 62.8% have bothersome symptoms. Twenty-five patients used pharmacological therapies. Forty-eight patients (66.6%) were satisfied with the aesthetic result of surgery (mean score in a scale from 0 to 5, is 3). Conclusions ECP can be used in children who require esophageal substitution. The resulting long-term QoL is acceptable. However, the aesthetic outcomes remain a problem.

The persistence of cloacal and urogenital sinus remains one of the most challenging problems for pediatric urologist. In this chapter we will describe the characteristics of these malformations in terms of diagnosis, clinical presentation, surgical treatment, and complications.

The Anderson–Hynes pyeloplasty is still the gold standard for the surgical treatment of the ureteropelvic junction obstruction in children. Laparoscopic, retroperitoneoscopic, and robotic approaches have been proposed as alternatives in order to reduce postoperative hospitalization, pain, and scars. We describe a modification of the retroperitoneoscopic technique that combines the advantages of a minimally invasive technique with the high success rate of standard dismembered pyeloplasty. We called the procedure OTAP (one-trocar-assisted pyeloplasty).

An anterior congenital diaphragmatic hernia (CDH) is a diaphragmatic defect that allows the passage of abdominal organs into the thorax. It is typically asymptomatic (the diagnosis is incidental) and it requires surgical correction. In this paper we present a 6 year-old girl affected by spinal muscular atrophy (SMA) who was diagnosed with anterior CDH. Four years after laparoscopic closure of the defect by interrupted suture the girl returned for hernia recurrence. Another laparoscopic procedure was performed and the defect was closed using a GORE-TEX patch. We postulate a mechanism of altered respiratory dynamic and increased abdominal pressure related to scoliosis favouring CDH recurrence in patients with neuromuscular pathologies such as SMA. In these patients patch interposition should be considered in the first place in order to reduce tension over margins. Laparoscopy is a safe and feasible procedure for CDH correction also in case of recurrence and when the interposition of a patch is required.

We report the case of a 4 year old patient affected by congenital bronchogenic cyst who underwent thoracoscopic surgery for cyst removal. A new 5 mm stapler device was used for the first time at our institution: its characteristics allowed us to safely perform the procedure with the advantages of using a 5 mm trocar.

Introduction. Jejuno-ileal atresia is the most common cause of congenital intestinal occlusion in newborns and the duodenal atresia is the most common cause of high bowel obstruction in the neonatal period. Nowadays the minimally invasive approach has been widely diffused and these surgical options are possible: circumbilical incision, the video-assisted and totally laparoscopic treatment. We present our experience with the video-assisted approach for the correction of intestinal atresias. Material and Methods. Seventeen patients with bowel atresia were treated by video-assisted procedure at our Institution. Surgical procedures consisted of identifying and exteriorization of the affected tract followed by traditional bowel atresia correction outside the abdomen. Results. All cases were successfully completed. There were no conversions to open surgery. One patient required a second procedure for an incomplete distal web that hadn’t been previously identified and another one experienced occlusion for intra-abdominal adhesions. Discussion and Conclusions. Our study supports the value of laparoscopy in the management of small bowel atresia providing a definitive diagnosis and directing subsequent surgical approach. The video assisted technique for the correction of small bowel atresia is safe, effective, and adds the advantages of the classic laparotomic procedure to the laparoscopic ones.

Pulmonary lymphangiectasia (PL) is a rare condition characterized by dilatation of the lymphatic vessels. Post-neonatal PL is usually associated with pleural effusion and should therefore be suspected in the presence of chylothorax. We describe a post-neonatal manifestation of PL in a 7-year-old boy presenting chylothorax. Radiological examinations included thorax X-ray, ultrasound, and computed tomography scans. After the failure of conservative management (maintenance of the chest tube, total parenteral nutrition, administration of somatostatin synthetic analogues) we performed a thoracoscopic massive ligation of the thoracic duct's collateral along with a lung biopsy. Histology was compatible with type 1 congenital pulmonary lymphangectasia. One month after surgery a thoracoscopic pleurodesis was required for persistent chylothorax. The boy is now doing well 1 year after surgery.

Mullerian duct anomalies include a wide spectrum of genital tract defects resulting from a development alteration of the genital tract. An 11-year-old girl with a hybrid septate variety uterus with left hemi-obstruction was identified. Beside preoperative imaging evaluation done with ultrasound, magnetic resonance, and hysterosalpingography, a combined laparoscopic and hysteroscopic procedure was fundamental for the diagnosis. The surgical procedure also allowed us to perform a successful and safe treatment. We propose that the American Fertility Society classification of these anomalies should be revised and that hybrid entities should be considered in the differential diagnosis of genital tract malformations.

Congenital lobar emphysema (CLE) is a pulmonary congenital malformation characterized by hyperinflation. After birth, a dysfunctional valve mechanism (air enters the alveoli thanks to negative pressure but cannot be released because the tracheobronchial tree has collapsed) leads to air trapping. CLE has all the features of obstructive emphysema: compression of lung parenchyma; mediastinal shift, herniation of the emphysematous lobe across the anterior mediastinum; and reduced respiratory reserve due to lowering of the diaphragm [1, 2].

Foregut duplications are rare cystic (by far the most frequent) or tubular masses, representing 20% of mediastinal masses [1]. These duplications include a wide spectrum of anomalies, usually enteric cysts or bronchogenic malformations.

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. 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 mullerian 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. 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. 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.

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.

Minimally invasive surgery (MIS) is a relatively new surgery comprising various procedures performed with special miniaturized instruments and imaging reproduction systems. Technological advances have made MIS an efficient, safe, and applicable tool for pediatric surgeons with unquestionable advantages. The recent introduction of three-dimensional (3D) high definition systems has been advocated in order to overcome some of the problems related to standard MIS visual limitations. This short paper recapitulates the necessity to minimize MIS visualization limitations and reports the characteristics of new laparoscopic 3D systems.

The application of laparoscopy in pediatric age with description of the main surgical procedures performed with this minimally invasive sugery. It also includes an introduction of instruments commonly used and a part related to pdiatric anesthesiology.