Detubularized Ureterosigmoidostomy for the Creation of Continent Neobladder in Children: Cases Report and Review of the Literature.

Introduction: To report our experience in continent urinary diversions, we describe two cases we treated performing detubularized ureterosigmoidostomy. In children, in the case of malformations or neoplastic diseases affecting the bladder, the need for a cystectomy is not so frequent. When cystectomy becomes mandatory, there is a need to create a continent bladder diversion. Mainz pouch II and Cologne pouch are procedures that utilize a detubularized sigma as a reservoir in order to build up a continent neo-bladder. Materials and methods: This is a retrospective study performed at the Pediatric Surgical Unit of the Salesi Children's Hospital. In this work, we reviewed data about two patients who underwent surgery for the creation of a sigmoid neo-bladder by the Mainz pouch II and Cologne pouch techniques. Results: In our experience, we treated a girl who was affected by a bladder's rabdomiosarcoma and a girl born with a bladder exstrophy and treated at birth abroad. In both patients, a complete cystectomy was performed and a continent neo-bladder was created by a detubularized ureterosigmoidostomy. In the first case, we performed the Mainz pouch II technique and in the second, the Cologne pouch technique. Discussion: Different techniques have been developed with the main goal of the creation of an orthotopic neo-bladder, which has to be a low pressure reservoir with a continent sphincteric mechanism. Detubularized ureterosigmoidostomy is a good choice in pediatric patients. Our study, according to other works, shows that these procedure are safe with good long-term outcomes.

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

Isolated fallopian tube torsion in pediatric age: An Italian multicenter retrospective study.

PURPOSE: The purpose of this study was to quantify the experience of five Italian centers on the diagnosis and management of isolated fallopian tube torsion (IFTT) in children. METHODS: We retrospectively reviewed the data of 20 patients aged 1-16 years of age with surgically diagnosed IFTT between 1991 and 2017 from five Italian centers of pediatric surgery. We analyzed common presenting signs, symptoms, and radiographic findings, as well as surgical interventions to describe management offering further insight into the diagnosis and treatment of this rare entity. RESULTS: Twenty cases of IFTT were collected. Median age was 13.1 years. Menarche was present in 14 cases. A clinical history of abdominal pain was present in 13 patients, whereas in 7 patients the clinical picture was an acute abdomen. Ultrasonography was the first diagnostic examination in 16 cases. Surgical approach was by laparoscopy in 16 cases and laparotomy in 4 cases. In 11 patients IFTT was associated with another pathologic condition. In 7 of the remaining 9 IFTT without pathologic association, the girls played sports. Salpingectomy was performed in 13 patients and de-torsion in 7. CONCLUSIONS: IFTT is a rare condition that seems to occur in younger adolescents. Vague clinical presentation contributes to low preoperative suspicion. IFTT should be considered in girls with abdominal pain who practice sports with sudden body movements. Preoperative suspicion may be increased based on radiographic findings of an enlarged tubular/cystic structure with adjacent normal ovary. Conservative management is controversial but could be preferred in order to provide the best option for future fertility of these girls. TYPE OF STUDY: Treatment Study (Retrospective Study) - Level IV.

Prospective evaluation of lung function in children with parapneumonic empyema.

RATIONALE: Prospective studies that evaluated the outcome of childhood empyema are limited. OBJECTIVE: To compare the outcome of pulmonary function in children with empyema. PATIENTS AND METHODS: Children discharged with a diagnosis of empyema underwent a longitudinal study including measurement of pulmonary function and radiographic imaging. RESULTS: The population consisted of 39 patients, 24 males, and 15 females; with a median age of 4.6 years. Etiology was defined in 20/39 patients, and predominant microorganism was Streptococcus pneumoniae (19/20 isolates). Chest tube drainage with or without fibrinolytic agents was the primary intervention in 25 children. Video-assisted thoracoscopic surgery was performed in 14 and 5 children as primary and secondary intervention, respectively. Thirty-five children completed the lung function follow-up. At first follow-up visit, 5 out of 17 children able to perform spirometry (initially collaborating children) had normal tests, and 12 had mild-to-moderate defects of lung function that returned to normal over 2-57 months. Eighteen children unable to perform spirometry at first follow-up visit (initially non-collaborating children) had normal tests when they were evaluated 5-78 months postdischarge. At the end of the follow-up, all patients had normal lung function. Time to normalize did not differ between groups receiving different treatments (initially collaborating children, P = 0.064; initially non-collaborating children P = 0.223). Three previously healthy children had recurrent cough, and all children had normal chest radiographs aside from pleural thickening. CONCLUSIONS: The respiratory outcome in children with empyema is generally good and is not influenced by the type of intervention.

How replace the gastrostomy tube in children with extremely compromised general conditions.

The percutaneous endoscopic gastrostomy is the best technique to use in case of children requiring long-time enteral nutrition. The first replacement of the tube is easy in most of the patient, but this step can be extremely problematic in patients with compromised general conditions. The authors report their experience in some cases. Two boys with endoscopic gastrostomy had a deterioration in the general conditions, when replacing the probe, these conditions contraindicated anaesthesia for which the "cut-and-push" technique was adopted in a patient with heart malformation and a local anaesthetic in a neuropathic patient. Both the boys died a few days after this procedure. In our opinion, in children who are not using anaesthesia to reposition the gastrostomy probe, it is best to continue feeding through the nasogastric probe.

The isolated tubal torsion: an insidious pediatric and adolescent pelvic urgency.

Isolated tubal torsion (ITT) is a rare cause of acute abdominal pain. Preoperative diagnosis is difficult because of a lack of specific signs. Surgery is recommended to preserve the integrity of the tube. Seven patients of median age of 13 years (range 9 to 15) came to our observation for worsening abdominal pain, nausea and vomiting. On admission, all girls had blood tests and ultrasound. Laparoscopy was performed for diagnosis in all cases. The girls had one-month and one-year ultrasound and clinic follow up. In all cases diagnosis was delayed, median 66 hours after the onset of symptoms and laparoscopy showed necrosis of the Fallopian tube. In five girls a laparoscopic salpingectomy was performed. In the other two, an open salpingectomy was necessary because of pelvic adhesions. Histology showed a hemorrhagic infarction of the Fallopian tubes. At follow up all patients were asymptomatic with normal ovaries, but one ovarian cyst. In the differential diagnosis of acute abdominal pain in children or female adolescents the possibility of ITT should be considered for a conservative treatment. Laparoscopy allows for definitive diagnosis and treatment.

Guidelines on pediatric day surgery of the Italian Societies of Pediatric Surgery (SICP) and Pediatric Anesthesiology (SARNePI).

The Italian Society of Pediatric Surgery (SICP) together with The Italian Society of Pediatric Anesthesia (SARNePI) through a systematic analysis of the scientific literature, followed by a consensus conference held in Perugia on 2015, have produced some evidence based guidelines on the feasibility of day surgery in relation to different pediatric surgical procedures. The main aspects of the pre-operative assessment, appropriacy of operations and discharge are reported.

Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center.

OBJECTIVE: The authors present their experience in the management of pediatric patients with Herlyn-Werner-Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation. METHODS: All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices). RESULTS: Six patients were treated in the study period. The mean age was 9 years (2 months-15 years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18 months all patients were symptoms free. CONCLUSIONS: Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary.

Retroperitoneoscopic One-Trocar-Assisted Pyeloplasty in Children: An Age-Related Evaluation.

INTRODUCTION: The aim of this study was to evaluate the efficacy of the retroperitoneoscopic one -trocar -assisted pyeloplasty (OTAP) in children of different ages. MATERIALS AND METHODS: Clinical data of all children who underwent OTAP for ureteropelvic junction (UPJ) obstruction at our Institution, between 2006 and 2014, were reviewed by focusing on demographics, surgical management, and outcomes. Data were analyzed by dividing patients into three groups according to age at operation: patients younger than 2 years (group 1), between 2 and 6 years (group 2), and older than 6 years (group 3). RESULTS: During the study period, 70 children (50 males) underwent OTAP (45 left and 25 right). A 10-mm ballooned trocar was inserted under the 11-12th rib and pneumoretroperitoneum was induced (12 mmHg pressure). Through an operative scope, the UPJ was carefully mobilized and exteriorized to the skin level to perform conventional Anderson-Hynes dismembered pyeloplasty. Mean age was 48 months (range, 1 month-14 years). Thirty-six patients (mean age 8.8 months) were in G1, 18 patients (mean age 4.2 years) in G2, and 16 patients (mean age 10.2 years) in G3. There were no intraoperative complications. Mean operative time was 137 minutes: 128 minutes (range, 85-213) in G1, 118 minutes (range, 90-215) in G2 (P > .05 versus G1), and 154 minutes (range, 95-215) in G3 (P < .05 versus G1; P < .05 versus G2). Conversion (an extension of the subcostal incision) rate was 27.1%: 16.6% in G1, 22.2% in G2 (P > .05 versus G1), and 56.25% in G3 (P < .05 versus G1; P < .05 versus G2). At a mean follow-up of 29 months (range, 12 months-5 years), recurrence was observed in 3 patients (2 G1, 1 G3). CONCLUSIONS: Our results show that OTAP is a good alternative in children younger than 6 years. OTAP is more challenging in older children because of the thickness of the abdominal wall and the higher incidence of aberrant crossing vessels.

Gastric transposition as a valid surgical option for esophageal replacement in pediatric patients: experience from three Italian medical centers.

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

Transumbilical Laparoscopic-Assisted Appendectomy in the Treatment of Acute Uncomplicated Appendicitis in Children.

Transumbilical laparoscopic-assisted appendectomy (TULAA) is increasingly being performed worldwide. The authors report their experience in the treatment of acute uncomplicated appendicitis in children with TULAA. From January 2008 to December 2012 all types of acute appendicitis were divided, according to the clinical and ultrasonographic findings, into complicated (appendiceal mass/abscess, diffuse peritonitis) and uncomplicated. Complicated appendicitis was treated by open appendectomy (OA). All patients with the suspicion of uncomplicated appendicitis were offered TULAA by all surgeons of the team. Conversion to open or laparoscopic appendectomy (LA) was performed in case of impossibility to complete TULAA, depending on the choice of surgeon. The histopathologic examination of appendix was always performed. 444 children (252 males) with acute appendicitis were treated. The mean age was 9.2 years (range, 2 to 14 years). Primary OA was performed in 144 cases. In 300 patients a transumbilical laparoscopic-assisted approach was performed. TULAA was completed in 252 patients. Conversion to OA was performed in 45 patients and to LA in 3. Conversion was related to the impossibility to adequately expose the appendix in 47 patients and bleeding in 1. The mean operative time for TULAA was 42 minutes. Histopathologic examination of the appendix removed by TULAA showed a phlegmonous/gangrenous type in 92.8% of cases. Among the 252 TULAA there were 11 cases of umbilical wound infection. TULAA is a feasible and effective procedure for uncomplicated appendicitis in children. It combines the advantages of open and laparoscopic technique (low operative time, low complications rate, and excellent cosmetic results).

A possible association between sports and isolated fallopian tube torsion in children and adolescent females.

Isolated fallopian tube torsion (IFTT) is a rare occurrence characterized by the rotation of the fallopian tube on its own axis, without twisting the ipsilateral ovary. Pre-operative diagnosis is difficult due to the lack of specific clinical symptoms and radiological signs. The etiology is to date unknown and only some pre-disposing factors have been reported. The history, clinical presentation, laboratory findings and surgical treatment of five patients aged 12-16 with IFTT were evaluated. All patients came to be examined for worsening abdominal pain, nausea or vomiting. 75% of them practiced sports involving sudden changes in body position. They all underwent blood tests, ultrasound and Magnetic Resonance and then laparoscopy for the persistence of acute symptoms. Laparoscopic salpingectomy was performed in three patients. Another two needed a conversion to open salpingectomy due to the presence of pelvic adhesions. During the follow-up period, every girl was fine. The cause of IFTT is undetermined. In this series, a close association between IFTT and sports causing rapid body movements was found. The possibility of IFTT should be considered in the differential diagnosis of acute abdominal pain in children or adolescent females, especially if they practice sports.

Laparoscopic approach to Meckel's diverticulum.

AIM: To retrospective review the laparoscopic management of Meckel Diverticulum (MD) in two Italian Pediatric Surgery Centers. METHODS: Between January 2002 and December 2012, 19 trans-umbilical laparoscopic-assisted (TULA) procedures were performed for suspected MD. The children were hospitalized for gastrointestinal bleeding and/or recurrent abdominal pain. Median age at diagnosis was 5.4 years (range 6 mo-15 years). The study included 15 boys and 4 girls. All patients underwent clinical examination, routine laboratory tests, abdominal ultrasound and technetium-99m pertechnetate scan, and patients with bleeding underwent gastrointestinal endoscopy. The abdominal exploration was performed with a 10 mm operative laparoscope. Pneumoperitoneum was established based on the body weight. Systematic overview of the peritoneal cavity allowed the ileum to be grasped with an atraumatic instrument. The complete exploration and surgical treatment of MD were performed extracorporeally, after intestinal exteriorization through the umbilicus. All patients' demographics, main clinical features, diagnostic investigations, operative time, histopathology reports, conversion rate, hospital stay and complications were registered and analyzed. RESULTS: MD was identified in 17 patients, while 1 had an ileal duplication and 1 a jejunal hemangioma. Fifteen patients had painless intestinal bleeding, while 4 had recurrent abdominal pain and exhibited cyst like structures in an ultrasound study. Eleven patients had a positive technetium-99m pertechnetate scan. In the patients with bleeding, gastrointestinal endoscopy did not name the source of hemorrhage. All patients were subjected to a TULA surgical procedure. An intestinal resection/anastomosis was performed in 14 patients, while 4 had a wedge resection of the diverticulum and 1 underwent stapling diverticulectomy. All surgical procedures were performed without conversion to open laparotomy. Mean operative time was 75 min (range 40-115 min). No major surgical complications were recorded. The median hospital stay was 5-7 d (range 4-13 d). All patients are asymptomatic at a median follow up of 4, 5 years (range 10 mo-10 years). CONCLUSION: Trans-umbilical laparoscopic-assisted Meckel's diverticulectomy is safe and effective in the treatment of MD, with excellent results.

Spermatogenesis and cryptorchidism.

Cryptorchidism represents the most common endocrine disease in boys, with infertility more frequently observed in bilateral forms. It is also known that undescended testes, if untreated, lead to an increased risk of testicular tumors, usually seminomas, arising from mutant germ cells. In normal testes, germ cell development is an active process starting in the first months of life when the neonatal gonocytes transform into adult dark (AD) spermatogonia. These cells are now thought to be the stem cells useful to support spermatogenesis. Several researches suggest that AD spermatogonia form between 3 and 9 months of age. Not all the neonatal gonocytes transform into AD spermatogonia; indeed, the residual gonocytes undergo involution by apoptosis. In the undescended testes, these transformations are inhibited leading to a deficient pool of stem cells for post pubertal spermatogenesis. Early surgical intervention in infancy may allow the normal development of stem cells for spermatogenesis. Moreover, it is very interesting to note that intra-tubular carcinoma in situ in the second and third decades have enzymatic markers similar to neonatal gonocytes suggesting that these cells fail transformation into AD spermatogonia and likely generate testicular cancer (TC) in cryptorchid men. Orchidopexy between 6 and 12 months of age is recommended to maximize the future fertility potential and decrease the TC risk in adulthood.

Versatility of one-trocar surgery in children.

BACKGROUND/PURPOSE: One-trocar surgery (OTS) includes all video-surgical techniques performed using a single 10-mm port and an operative scope. These techniques can be completely endoscopic or endoscopic assisted. Since 1997, OTS has become the approach of choice in our institution for a variety of laparoscopic, retroperitoneoscopic, and thoracoscopic operations. We report our experience with this technique. METHODS: Four hundred fifty-eight patients (age range, 3 months to 17 years) underwent OTS from October 1997 to December 2008. The procedures were transumbilical laparoscopic-assisted (TULA) appendectomy (182 patients), TULA small bowel resection (14 patients), TULA intestinal biopsies (7 patients), laparoscopic adhesiolysis (6 patients), laparoscopic-assisted liver biopsies (5 patients), laparoscopic revision of peritoneal dialysis catheter (3 patients), retroperitoneoscopic varicocelectomy (202 patients), retroperitoneoscopic-assisted renal biopsies (4 patients), retroperitoneoscopic drainage of posttraumatic urinoma (1 patient), retroperitoneoscopic-assisted pyeloplasty (15 patients), and thoracoscopic pleural debridement and decortication for empyema (19 patients). RESULTS: The procedure was completed using only one trocar in 399 cases (87.1%). All conversions to multitrocar or open surgery were elective and regarded the retroperitoneoscopic approach during the learning curve (28 of 222, 12.6%; 21 varicocelectomies and 7 pyeloplasties) and the TULA appendectomy because of the appendix mobilization failure (31 of 182, 17%). There were no intraoperative or postoperative complications related to OTS. Wound infection was observed after two TULA appendectomies (1.3%). CONCLUSIONS: According to our experience, OTS is a feasible and versatile technique in pediatric surgery, providing a safe, effective, and the least invasive treatment for several different diseases.

Immature teratoma of the mediastinum arising in ectopic thyroid tissue: a case report.

BACKGROUND: Papillary carcinoma can arise from ectopic thyroid tissue, but teratoma have not been described. Differentiation into thyroid follicles does not occur in mediastinal teratomas. CASE: A case of upper mediastinal immature teratoma occurred in an 18-day-old male newborn. The histologic examination revealed the presence of a discontinuous rim of compressed thyroid follicles on the outer aspect of the tumor capsule. This finding is consistent with the origin of the teratoma in ectopic thyroid tissue, and it has not been previously described in the literature. The patient was free of disease after 22 months, in accordance with the benign behavior of immature teratoma in infancy. CONCLUSION: Ectopic thyroid tissue can undergo the same pathologic changes as the thyroid gland, including the rare occurrence of teratoma.

Delayed upper gastrointestinal bleeding after laparoscopic treatment of forme fruste choledochal cyst.

UNLABELLED: Delayed upper gastrointestinal (UGI) bleeding after surgery is a catastrophic event with high mortality unless diagnosed early. In this paper, report a case of massive UGI bleeding 1 month after the laparoscopic treatment of a forme fruste choledochal cyst (FFCC). CASE REPORT: A 8-year-old girl presented at our attention because of acute pancreatitis. Ultrasound and magnetic resonance cholangiopancreatography diagnosed an FFCC. Once serum amylase and lipase were normal, a laparoscopic extrahepatic bile duct excision (EHBD) with a Roux-en-Y hepaticojejunostomy was performed without intraoperative complication. One month later, the patient had massive UGI bleeding, and laparotomic treatment of duodenal bleeding was necessary because of hemodynamic instability. Despite intravenous omeprazole and somatostatin, 1 week later, a new massive UGI bleeding occurred during hospitalization and an antral gastric resection with gastrojejunostomy (Billroth II) was performed. The patient was discharged 3 weeks later and she is well at 18-months of follow-up. EHBD excision with a Roux-en-Y hepaticojejunostomy is the treatment of choice for FFCC; laparoscopic approach is feasible and effective in children, too. The severe complication reported seems not related to the minimal invasive approach; in fact, it can occur after pancreatic or biliary surgery.