Pediatric leiomyoma of the glans: a case report.

OBJECTIVE: The leiomyoma is a benign mesenchymal tumor originating from smooth muscle cells therefore its location is ubiquitous. The genitourinary system is not a common site and the glans localization in pediatric age has been described only three times in the literature to date. CASE REPORT: We describe a case of an 11-year-old boy who presented with a painless, non-bleeding or itchy tumor of the glans. The surgical procedure consisted in the total removal of the mass. The histological study showed spindle cells with an eosinophilic cytoplasm while the immunohistochemical studies proved cells stained strongly positive for smooth muscle actin. The clinical follow-up for more than 5 years after surgery demonstrates the absence of recurrence and discomfort for the patient and a good aesthetic appearance of the glans. RESULTS: Leiomyoma is a benign tumor that can originate anywhere there is smooth muscle. However, localization at the level of the glans can be treated with a total excision due to the presence of a cleavage plane with the surrounding tissues that allows a good reconstruction of the glans itself. CONCLUSIONS: We propose that leiomyoma ought to be considered in the differential diagnosis of any glans mass in children.

Distal hypospadias repair using the needle point bipolar cutting-coagulation forceps.

INTRODUCTION: To determine the outcome of distal hypospadias repairs performed using bipolar diathermy (BD) for all the dissection. METHODS: Retrospective review of 310 patients undergoing distal hypospadias (264 subcoronal/distal penile and 46 coronal/glanular) over a 11-year period. Median age at surgery was 2.0 years (range 9 months-15 years). Caudal anesthesia was performed in all patients. All children underwent an in situ tubularization of the urethral plate, which was combined with midline incision of the plate in 30 (10%). Preputial reconstruction was performed in 303 (98%) patients. BD forceps was used for coagulation and for all the dissection including skin incision, elevation of glans wings, separation of the corpus spongiosum from the corpora cavernosa, and urethral plate incision, when deemed appropriate. The authors assessed surgical complications and cosmetic results. The latter using the hypospadias objective score (HOSE), with a score ≥ 14 considered as acceptable. RESULTS: The average operative time was 70 min. There was no postoperative bleeding or hematomas that required surgical intervention. There were no wound infections or necrosis. Complications occurred in 37 patients (11.9%). Urethral fistula formation was the commonest (n = 18). Postoperative persistent preputial swelling occurred in 3.5% of cases. Two hundred twelve patients (90.6%) had a HOSE score ≥ 14, and no patient required revision surgery for skin problems after a median follow-up of 8.1 (range 1.2-13.1) years. DISCUSSION: The study is limited by its retrospective nature and by the fact that a number of other pre-operative, intra-operative, and postoperative variables can affect outcome. CONCLUSIONS: Bipolar diathermy can be safely used for distal hypospadias repairs. It allowed careful control of intra-operative bleeding and also clear visualization of tissue planes. Complication rate overall compares favorably with the literature, and cosmetic results were satisfactory.

Tethered cord in patients with anorectal malformation: preliminary results.

BACKGROUND: A tethered cord (TC) has been reported in as much as 50% of the patients affected by anorectal malformation (ARM). No guidelines for timing and modality of diagnosis and treatment have been established. We present the preliminary results of a multidisciplinary protocol carried out at our center. METHODS: Seventy-four ARM patients underwent spinal magnetic resonance imaging (MRI). All TC patients underwent videourodynamic (UD), somatosensory-evoked potentials (SEPs), and neurological examination at baseline and, if normal, at 5 and 10 years of age. Conversely, when UD or SEP abnormalities were detected the follow-up was individually tailored at shorter time. RESULTS: 25/74 patients had a neuroradiological TC (33.7%). Based on the results of UD, SEP, and neurological status, four patients were untethered, eight are possible candidates, nine are stable, and four were excluded because of incomplete data. DISCUSSION: Tethered cord is frequent in ARM patients. Because neurological deficits secondary to TC can contribute to neurological disability, we recommend routine MRI examination and a multidisciplinary program of follow-up in cases of TC. Preliminary results suggest the combined use of SEPs and UD could represent a useful adjunct to clinical examination in patients in whom a "wait and see" approach is preferred to the prophylactic surgery.

EBM II: How to perform a literature search.

The present article is a second part related to evidence based medicine (EBM) in a series of five by the European society for paediatric urology (ESPU) research committee. It will present the different databases/search engines available to clinicians and researchers and describe strategies to focus the search to one's particular needs. Indeed, databases/search engines used and search strategy should vary according to the goal of the research. If the aim is to address a clinical problem, the search should allow to identify a small number of most pertinent articles (high specificity); if the search is for research purposes, instead, it should ensure no meaningful articles are overlooked (high sensitivity).

Evidence-based medicine 1: Background.

The Research Committee of the European Society of Pediatric Urology will present a series of short educational communications related to evidence-based medicine. The aim of the series is to emphasize the importance of grading evidence, thereby determining the best clinical practice for patients. The goal of this series is to guide the clinician in using tools for setting up a clinical question, finding appropriate information, searching appropriate databases, and evaluating the results in relation to the patient in mind. This first part will serve as an introduction or background. Following publications will cover the topics of hierarchy of evidence, information acquisition, clinical appraisal tools, and applications in clinical practice [3].

Evidence-based medicine III: level of evidence.

The present article is the third installment in a five-part series related to evidence-based medicine (EBM) provided by the European Society for Paediatric Urology Research Committee. It will present the different levels of evidence (i.e. systematic review, randomized controlled trial, cohort study) available to clinicians and researchers and describe the strengths of each study type. While EBM provides a valuable construct to aid in medical decision-making, it remains imperative that this information be interpreted and applied in the clinical context with a good dose of common sense.

Evidence Based Medicine IV: how to find an evidence-based answer to a clinical question? Make a critically appraised topic!

This manuscript is the fourth in a five part series related to evidence based medicine (EBM) provided by the European society of pediatric urology (ESPU) research committee. It will present a way to come to a quick and critical appraisal of available evidence on a specific topic: a CAT (critically appraised topic). The way how to write a cat is described for interventions to be compared to a control group, and for other, more generalized clinical questions. While systematic reviews provide a throughout overview of all evidence available, a CAT provides a shorter way to come to quick insights based on EBM.

Pyeloplasty vs. nephrectomy for ureteropelvic junction obstruction in poorly functioning kidneys (differential renal function <20%): a multicentric study.

INTRODUCTION: Management of poorly functioning kidneys with ureteropelvic junction obstruction (UPJO) is controversial, with some recommending direct nephrectomy and others direct pyeloplasty, and others temporary diversion. This study aimed to determine whether pyeloplasty is associated with higher complication rate than nephrectomy under these circumstances and whether it allows for functional recovery. METHODS: A retrospective review of 77 patients undergoing surgery for UPJO associated with a differential renal function (DRF) < 20% at 4 centres between January 2000 and December 2015 was conducted. Short- and long-term complications between pyeloplasties (n = 47) and nephrectomies (n = 16) and the changes in the DRF after pyeloplasty were compared. RESULTS: Patients undergoing nephrectomy had significantly lower median pre-operative DRF (p < 0.001) and were significantly more likely to undergo a minimally invasive approach (p = 0.002) than those undergoing pyeloplasty. No postoperative variable was statistically different between groups. After a mean follow-up of 63 (10-248) months, no statistically significant difference was found in intra-operative, early, late, and overall complications between pyeloplasty and nephrectomy. Pyeloplasty failed in 3% (2/62) of cases. Of the patients undergoing successful pyeloplasty, 36 had a pre-operative and a postoperative renogram, and functional recovery >5% was observed in 13 (36%), whereas the DRF remained unchanged in 16 (45%). Only postnatal diagnosis was associated with a significantly higher chance of functional recovery (odds ratio [OR] = 4.13, p = 0.047). DISCUSSION: Nephrectomy was more commonly performed using a minimally invasive approach and required less-intense follow-up than pyeloplasty. Moreover, complications after pyeloplasty, although occasional, required additional surgery which was never required after nephrectomy. Moreover, it should be considered that conservative treatment might be a third option in some of these patients. CONCLUSIONS: In the study patients, pyeloplasty was not associated with significantly higher morbidity than nephrectomy. Need for deferred nephrectomy seems exceptional in decompressed kidneys even though renal function remains poor. Of the pyeloplasty cases not requiring additional surgery, one-third showed an improvement in DRF and a postnatal diagnosis was the only predictive factor for renal functional recovery.

Comprehensive laparoscopic approach to pediatric varicocele based on preoperative color Doppler ultrasound assessment.

BACKGROUND: This study aimed to assess whether laparoscopic treatment for any kind of varicocele is possible after preoperative identification of refluxing veins by color Doppler ultrasound (CDUS). METHODS: At the authors' institution, 98 patients with a median age of 11.3 years (range, 7.1-16 years) were evaluated for a left varicocele. Preoperatively, all the patients underwent ultrasound scan assessment of testicular volume and CDUS to rule out reflux into the internal spermatic vein (ISV), deferential vein, or cremasteric vein. In all the patients, laparoscopic division of the spermatic artery and veins was performed as close as possible to the internal inguinal ring. The other vessels were coagulated and divided if shown to be refluxing on CDUS. RESULTS: Color Doppler ultrasound showed reflux only in the ISV in 87 cases (88.7%), but in both the ISV and the deferential in the remaining 11 cases (11.2%). During a median follow-up period of 18 months (range, 6-49 months), none of the authors' patients experienced varicocele recurrence either clinically or according to CDUS scanning. The median left testicular volume increased significantly postoperatively. CONCLUSION: The proposed technique based on laparoscopic interruption of the ISV and testicular artery very close to the internal inguinal ring, meticulous CDUS assessment to rule out reflux in the deferential vein, and coagulation of refluxing deferential veins allows successful laparoscopic treatment of most varicoceles.

[Duodenal perforation due to an abdominal drain placed after appendectomy in a child.

Insertion of abdominal drainage after appendectomy is controversial. We report on a case of duodenal perforation due to a silastic open drainage tube placed in the right para-colic gutter after appendectomy for perforated appendicitis. This case offers a clue against too liberal usage of abdominal drainage after appendectomy. Conservative management of bowel perforations secondary to abdominal drainages should be attempted in the absence of generalised peritonitis.

Two-stage repair in hypospadias.

We provide the reader with a nonsystematic review concerning the use of the two-stage approach in hypospadias repairs. A one-stage approach using the tubularized incised plate urethroplasty is a well-standardized approach for the most cases of hypospadias. Nevertheless, in some primary severe cases, in most hypospadias failures and in selected patients with balanitis xerotica obliterans a two-stage approach is preferable. During the first stage the penis is straightened, if necessary and the urethral plate is substituted with a graft of either genital (prepuce) or extragenital origin (oral mucosa or postauricular skin). During the second stage, performed around 6 months later, urethroplasty is accomplished by graft tubulization. Graft take is generally excellent, with only few cases requiring an additional inlay patch at second stage due to graft contracture. A staged approach allows for both excellent cosmetic results and a low morbidity including an overall 6% fistula rate and 2% stricture rate. Complications usually occur in the first year after the second stage and are higher in secondary repairs. Complications tend to decrease as experience increases and use of additional waterproofing layers contributes to reduce the fistula rate significantly. Long-term cosmetic results are excellent, but voiding and ejaculatory problems may occur in as much as 40% of cases if a long urethral tube is constructed. The procedure has a step learning curve but because of its technical simplicity does not require to be confined only to highly specialized centers.

Is perioperative cholangiography necessary in children undergoing elective laparoscopic cholecystectomy?

BACKGROUND: The necessity of carrying out pre- or intra-operative imaging of the biliary tree to rule out a possible anatomical abnormality or the presence of common bile duct (CBD) stones in patients undergoing laparoscopic cholecystectomy (LC) is debated. We prospectively assessed the risk of developing symptoms related to bile duct injury or CBD stones after LC in children not receiving peri-operative cholangiography. MATERIALS AND METHODS: All patients

Rectal adenocarcinoma in patients with anorectal malformations: report of two cases and a review of the literature.

AIM: Anorectal malformation (ARM) is a rare congenital disorder of the anus and rectum. In the last 30 years virtually all patients born with ARM have survived and surgeons from adult care may be called to deal with new and long-term sequelae, including tumors of the pulled-through anorectum. Two new cases of colorectal carcinoma in young adults born with ARM and a review of the literature is reported to emphasize the importance of a multidisciplinary follow-up. METHODS: A man and a woman, with previous history of ARM, presented at 34 years of age with symptoms of intestinal occlusion and a large pelvic mass. Both patients had no familial history of colorectal carcinoma. RESULTS: The patients underwent biopsies (mucinous rectal adenocarcinoma) and stadiation (T4N0M0). In one case the microsatellite instability showed a stable profile. Despite maximal treatments, including surgery, chemo- and radio-therapy, they both died a few years after diagnosis for progression of disease. CONCLUSION: Case studies are too limited to suggest guidelines for prevention and treatment of such complications, but the life-long follow-up is mandatory in the framework of a well-established network between pediatric and adult surgeons. The risk of tumor development in these patients should not be neglected and colleagues from adult care should be aware of the possibility this occurs in their practice.

Bladder urothelial neoplasms in pediatric age: experience at three tertiary centers.

INTRODUCTION: Urothelial bladder neoplasms (UBN) typically occur in patients in their sixth or seventh decade of life while they are infrequent in children and young adults. They occur in 0.1-0.4% of the population in the first two decades of life. Their management is controversial and paediatric guidelines are currently unavailable. OBJECTIVE: To further expound the available data on the outcome of patients younger than 18 year old diagnosed with UBN. STUDY DESIGN: We retrospectively reviewed the files of all the consecutive paediatric patients with UBN treated in three tertiary paediatric urology units from January 1999 to July 2013. Lesions were classified according to the 2004 WHO/ISUP criteria as urothelial papillomas (UP), papillary urothelial neoplasm of low malignant potential (PUNLMP), low-grade urothelial carcinoma (LGUC), and high-grade urothelial carcinoma (HGUC). RESULTS: The table shows the results. Management after TURB varied among centres. One centre recommended only follow-up US at increasing intervals whereas another follow-up US plus urine cytologies and endoscopies, every three months in the first year, and at increasing intervals thereafter. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed disease recurrence or progression. DISCUSSION: UBN is an uncommon condition in children and adolescents and, unlike in adults, its incidence, follow-up and outcome still controversial. Paediatric guidelines are currently lacking and management varies among centres. Gross painless haematuria is the most common presenting symptom. Therefore, this symptom should never be underestimated. US is generally the first investigation and additional imaging seems unnecessary. TURB often allows for complete resection. Lesions are generally solitary, non-muscle invasive, and low-grade (mainly UP and PUNLMP). Ideal follow-up protocol is the most controversial point. Reportedly, recurrence or progression during follow-up is uncommon in patients under 20 years, recurrence rate 7% and a single case of progression reported so far. Accordingly, a follow-up mainly based on serial US might be considered in this age group compared to adults where also serial endoscopies and urine cytologies are recommended. In the selection of the follow-up investigations, it should also be taken into consideration that urine cytology has a low sensibility in the detection of low-grade lesions while cystoscopy in young patients requires a general anaesthesia and hospitalization, and carries an increased risk of urethral manipulation. CONCLUSION: UBN is a rare condition in children. Ultrasound is generally accurate in order to visualize the lesion, and TURB can treat the condition. Lesions are generally low-grade and non-muscle invasive, but high-grade lesions can also be detected. In present series, after TURB, follow-up US monitoring at increasing intervals was used at all centres, follow-up cystoscopies were added in two centres, but with different schedules. Urine cytologies were considered only at one centre. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed recurrence or progression of the disease.

Optimizing the surgical management of lung nodules in children with osteosarcoma: thoracoscopy for biopsies, thoracotomy for resections.

BACKGROUND: The goal of this study was to assess the role of thoracoscopy (TS) and thoracotomy (TT) in the management of lung nodules in children with osteosarcoma. METHODS: Charts of 16 osteosarcoma patients undergoing surgery for lung nodules were retrospectively analyzed for a correlation between nodule localization at CT scan, findings at surgery, and pathology. RESULTS: Fourteen TSs were performed in 10 children, eight of which were converted: two for technical problems, and six for inconsistency between CT scan and intraoperative findings. In three converted cases, TT allowed detection of more nodules than CT scan and/or TS. Eight TTs were performed as primary intention in seven children, in one as secondary surgery after a previous TS. In three cases, TT detected more nodules than CT scan. Overall, TT detected more nodules than CT scan in seven of 16 cases (sensitivity, 56.2%), six of whom had a predicted bilateral involvement. Neoplastic tissue was present in lung samples of all but three patients (86.4%). CONCLUSIONS: Lung nodules in osteosarcoma patients are usually metastases. CT scan is unreliable in detecting all the nodules, especially in the case of predicted bilateral involvement. If excision of all metastases is considered the goal of surgery, a TT approach should be chosen in patients with more than one thoracic nodule.

Outcome of laparoscopic Nissen-Rossetti fundoplication in children with gastroesophageal reflux disease and supraesophageal symptoms.

BACKGROUND: The role of surgery is debated for children with gastroesophageal reflux disease (GERD), particularly when they show atypical symptoms. This study was designed to evaluate the safety and outcome of laparoscopic Nissen-Rossetti fundoplication performed in a selected population of children with gastroesophageal reflux and atypical supraesophageal symptoms. METHODS: This prospective study included 595 patients younger than 14 years with GERD who reported recurrent respiratory symptoms and had no benefit from standard medical treatment. Surgery was performed for 48 patients with anatomic anomalies, life-threatening events, or respiratory complications after ineffective medical treatment. The subjective and objective outcomes were evaluated. RESULTS: No major intraoperative complications were experienced, and there was no recurrence of gastroesophageal reflux during a postoperative follow-up period of 12 months. The parents' final subjective evaluation of the outcomes 12 months after surgery was positive in 44 cases and negative in 4 cases. CONCLUSIONS: Children with difficult-to-treat chronic respiratory symptoms must be evaluated for GERD, even if the need for surgery is low (8%), because complete eradication of reflux is mandatory. Radical treatment of GERD allows the pulmonologist to perform correct respiratory treatment and to prevent the development chronic and life-threatening complications.

Anesthesiologic aspects of laparoscopic fundoplication for gastroesophageal reflux in children with chronic respiratory and gastroenterological symptoms.

INTRODUCTION: During the past three decades laparoscopy has significantly improved. As fundoplication extensively benefits by the great advantages of the minimally invasive approach, many surgeons chose laparoscopic fundoplication for the treatment of gastroesophageal reflux in adults and children as well. Pneumothorax, cardiovascular collapse, hypoxia, and hypercarbia are some of the anesthesiologist's principal fears during carbon dioxide insufflation. Thus, monitoring cardiovascular and respiratory status is mandatory to early detect any complication and to maintain a proper balance during pneumoperitoneum. MATERIALS AND METHODS: At Gaslini Children's Hospital we performed a prospective nonrandomized study aimed at describing the main cardiorespiratory changes produced by pneumoperitoneum in 33 pediatric patients operated on by laparoscopic fundoplication between January 2000 and June 2001. Patients were divided into two groups; namely, group A and group B. Group A included 14 patients with chronic respiratory symptoms, and group B included 19 children who preoperatively mainly emphasized gastrointestinal symptoms. We monitored intraoperative cardiorespiratory status, timed length of surgery, and described intraoperative complications. RESULTS: No significant cardiovascular changes occurred during carbon dioxide insufflation. Partial oxygen saturation remained still in all the patients. End tidal CO2, meanly higher in group A children, increased in all the patients after pneumoperitoneum creation, but never exceeded 45 mmHg. Similarly, peak inspiratory pressure increased in all the patients, but was always maintained within acceptable values. Finally, group B patients required a harder and slower surgery, whose length seems to be negatively influenced by age. No intraoperative complication occurred. CONCLUSIONS: Carbon dioxide insufflation does not impair cardiovascular function, if intraabdominal pressure is maintained lower than 10 mmHg nor does it interfere with gas exchanges. Pneumoperitoneum slightly reduces ventilatory function, mainly in respiratory patients with various degrees of underlying bronchopulmonary impairments, but this effect is easily correctable. Thus, laparoscopic fundoplication is feasible and safe in both respiratory and gastroenterological patients, although surgery is easier and faster if periesophagitis is less evident.

Effectiveness of esophageal manometry in predicting the outcome of children with primary GER after floppy Nissen-Rossetti wrap.

BACKGROUND: Surgical treatment of gastroesophageal reflux (GER) can result in many postoperative problems because of an incorrect indication or an unsuitable fundoplication. Many preoperative tests have been suggested to perform a "tailored fundoplication," but there is no clear evidence as to which is the best. The aim of our study was to define the effectiveness of esophageal manometry in predicting the outcome of children who need fundoplication because of refractory primary gastroesophageal reflux. METHODS: Thirty-two children were included in the study. Patients with gastroenterologic and respiratory symptoms numbered 10 (31%) and 22 (69%), respectively. The preoperative motility pattern was (1) inappropriate relaxations alone in nine patients (28%) and associated with esophageal body dysmotility in one patients (3%); (2) low-pressure lower esophageal sphincter (LES), alone in 13 patients (41%) and associated with body dysmotility in eight (25%). Motility pattern of the esophageal body was abnormal in nine children. Manometric anomalies were absent only in one case. RESULTS: Low pressure of the LES associated with atypical esophageal motility disorders was significantly higher in the gastroenterological group and in the older patients. Low pressure of the LES associated with good esophageal activity was significantly higher in the respiratory group and in the patients younger than 24 months. There was no correlation between motility pattern and outcome, and no statistically significant differences were found between pressure values at the different levels and symptoms, outcome, and age groups. CONCLUSIONS: Esophageal manometry is not mandatory to predict the outcome of patients undergoing laparoscopic Nissen-Rossetti fundoplication for refractory primary GER treatment.

Results of the laparoscopic Heller-Dor procedure for pediatric esophageal achalasia.

BACKGROUND: Esophageal achalasia is not a frequent disorder in children and different treatments have been proposed during past decades. This study reviews the results of the laparoscopic Heller-Dor procedure performed in pediatric patients in two different surgical units. METHODS: We included the patients aged <14 years with a minimum follow-up of 6 months operated on in the period 1994-2001. A single longitudinal anterior esophageal myotomy (Heller) and a 180 degrees anterior gastropexy (Dor) were laparoscopically performed. The patients were checked to detect intra- or postoperative complications and recurrence. RESULTS: Twenty children were operated on. Mean follow-up was 45 months (range 6-102). Postoperative clinical score was Visick 1 in 15 cases and Visick 2 in five. CONCLUSIONS: As complication and recurrence rates are very low we consider modified Heller myotomy and Dor gastropexy through a laparoscopic approach our first choice to treat esophageal achalasia in the pediatric population.

IgG antibodies to papillomavirus genus-antigens in adult men with cancer of the urinary bladder.

Forty-five sera from men with bladder cancer were examined in a micro solid-phase enzyme-linked immunosorbent assay (ELISA) and in a Western-blotting (WB) assay for the presence of IgG antibodies to papillomavirus (PV) genus-antigens of bovine origin. The ELISA detected PV antibodies in 75.6% of cancer patients. This antibody frequency was significantly higher than that found in both healthy males (22.7%) and patients with urological disorders (24%). A similar correlation among the PV antibody frequencies of the three groups was found with WB assay: 60% of the neoplastic group showed PV antibodies versus 17.3% in healthy males and 32.6% in non-neoplastic patients. Within the same group, 78% to 87% sera showed the same reactivity to both assays. Of these concordant sera, PV positive sera were 55.6% in cancer patients, 13.3% in healthy adults and 19.6% in patients with urological disorders. ELISA PV antibody level in the cancer group was higher than in each of the two control groups. The meaning of the humoral response to PV genus-antigens in men with bladder cancer is discussed.