Isolated Fallopian Tube Torsion in Children With Hydrosalpinx: Is Conservative Management an Option?

BACKGROUND: Isolated fallopian tube torsion (IFTT) is very rare gynecological emergency in pediatric population. Our objective is to assess treatment options and discuss outcome of a cohort of IFTT with a focus on the association between IFTT and hydrosalpinx (HSX). METHODS: A retrospective review was conducted. Pediatric patients with IFTT operated in the same center were included. RESULTS: Seventeen girls (aged: 11-16 years) were managed for acute abdominal pain between 2008 and 2018, with intraoperative diagnosis of IFTT. All patients underwent laparoscopic exploration, with laparoscopically fallopian tube detorsion in all patients. Based on the association of IFTT with HSX after fallopian tube detorsion, patients were divided into 2 groups: group 1 (IFTT without HSX; 12 girls) and group 2 (IFTT with HSX; 5 girls). During the same surgery, complementary surgical procedures were done. In group 1: salpingectomies (4), partial salpingectomies (2) and cystectomies (6) were done. In group 2: salpingectomy (1), salpingotomy (1), and cyst ablation (1). The treatment was called conservative when the tube was preserved.Follow-up was uneventful in group 1. In group 2, for all patients with initial fallopian tube preservation, further surgical procedures were necessary (1-4 surgeries/patient), and, finally, another 3 patients required salpingectomy. CONCLUSIONS: Conservative treatment with tube preservation of IFTT without HSX appeared to be beneficial compared to those with HSX, with no recurrence of torsion or symptoms during the follow-up. However, the same conservative treatment was not sufficiently effective for IFTT with HSX and required further procedures due to recurrence of torsion. LEVEL OF EVIDENCE: IV.

Ultrafast cadmium-zinc-telluride-based renal single-photon emission computed tomography: clinical validation.

BACKGROUND: One of the main limitations of 99mtechnetium-dimercaptosuccinic acid (DMSA) scan is the long acquisition time. OBJECTIVE: To evaluate the feasibility of short DMSA scan acquisition times using a cadmium-zinc-telluride-based single-photon emission computed tomography (SPECT) system in children. MATERIALS AND METHODS: The data of 27 children (median age: 4 years; 16 girls) who underwent DMSA SPECT were retrospectively analyzed. Both planar and SPECT DMSA were performed. SPECT images were analyzed using coronal-simulated planar two-dimensional images. A reduction in SPECT acquisition time was simulated to provide 4 series (SPECT-15 min, SPECT-10 min, SPECT-5 min and SPECT-2.5 min). A direct comparison of the planar and SPECT series was performed, including semi-quantification reproducibility, image quality (mean quality score on a scale of 0 to 2) and inter- and intra-observer reproducibility of the scintigraphic patterns. RESULTS: The overall image quality score (± standard deviation) was 1.3 (± 0.6) for the planar data set, 1.6 (± 0.5) for the SPECT-15 min data set, 1.4 (± 0.5) for the SPECT-10 min data set, 1.0 (± 0.5) for the SPECT-5 min data set and 0.6 (± 0.6) for the SPECT-2.5 min data set. Median Kappa coefficients for inter-observer agreement between planar and SPECT images were greater than 0.83 for all series and all readers except one reader for the SPECT-2.5 min series (median Kappa coefficient = 0.77). CONCLUSION: Shortening SPECT acquisitions to 5 min is feasible with minimal impact on images in terms of quality and reproducibility.

Early detection of ureteropelvic junction obstruction in neonates with prenatal diagnosis of renal pelvis dilatation using 1H NMR urinary metabolomics.

Renal pelvis dilatation (RPD) is diagnosed in utero on prenatal ultrasonography (US) and can resolve spontaneously. However, isolated RPD can also reflect ureteropelvic junction obstruction (UPJO), which requires surgical treatment to prevent progressive renal deterioration. The diagnosis of UPJO can only be confirmed after birth with repeat US and renal isotope studies. 1H Nuclear Magnetic Resonance spectroscopy (NMR) was performed on urine of newborns with prenatally diagnosed unilateral RPD and healthy controls to identify specific urinary biomarkers for UPJO. The original combination of EigenMS normalization and sparse partial-least-squares discriminant analysis improved selectivity and sensitivity. In total, 140 urine samples from newborns were processed and 100 metabolites were identified. Correlation network identified discriminant metabolites in lower concentrations in UPJO patients. Two main metabolic pathways appeared to be impaired in patients with UPJO i.e. amino acid and betaine metabolism. In this prospective study, metabolic profiling of urine samples by NMR clearly distinguishes patients who required surgery for UPJO from patients with transient dilatations and controls. This study will pave the way for the use of metabolomics for the diagnosis of prenatal hydronephrosis in clinical routine.

Pleuropneumonectomy as Salvage Therapy in Children Suffering from Primary or Metastatic Sarcomas with Pleural Localizations.

Pediatric sarcoma patients with pleuropulmonary lesions have a dismal prognosis because the impossibility to obtain local control. The aim of this study was to determine if pleuropneumonectomy (PP) could be a therapeutic option. We retrospectively reviewed nine patients who underwent salvage PP for pleuropulmonary localization of primary localized sarcoma or metastatic recurrence. Surgery and complications were analyzed, pulmonary function tests were conducted, and quality of life was determined with EORTC-QLQ-C30 questionnaire. At the time of PP age was between 9-17 years. Underlying disease included metastatic osteosarcoma (n = 5), Ewing sarcoma (two metastatic, one primary), and one primary undifferentiated sarcoma. Early complications occurred in three patients. Mean postoperative hospitalization stay was 14.5 days. Pulmonary function test showed 19-66% reduction of total lung capacity which led to mild exercise intolerance but did not affect daily life. Four patients died of multi-metastatic relapse <14 months after PP, one patient had a local recurrence, and four patients are in complete remission between 1.5 and 12 years after PP. In conclusion, in this small patient group treated with a pleuropneumonectomy for primary or metastatic lesions, outcome is variable; however, this extended surgical technique was generally quite well tolerated. Postoperative lung function seems well preserved, and it seems to lead to at least an extension of life with good quality and therefor can be considered as salvage therapy.

Tumoral and pseusotumoral processes of the vagina in the pediatric population: A 26-YEAR retrospective study.

BACKGROUND: Vaginal lesions are rare and of various types in children. Clinical presentation is generally undifferenciated. Histological examination is fundamental to ascertain the nature of the lesion. Regarding tumoral lesions, histological subtypes encountered are radically different from those seen in adults, dominated by stromal benign lesions. OBJECTIVE: The aim of this retrospective study was to describe characteristics and pathological aspects of pediatric vaginal lesions, diagnosed in a single pediatric experienced center. STUDY DESIGN: A database analysis was performed on all vaginal samples of patients under 18 years old received in a pediatric-specialized pathology laboratory of an academic hospital, over a 26-year period. RESULTS: Among 36 vaginal tissue samples reported, a total of 15 tumoral or pseudotumoral processes was recorded. Primitive malignant tumors included embryonal rhabdomyosarcoma (n = 3) and germ-cell tumors, yolk-sac type (n = 2). Benign tumoral or pseudotumoral processes included inflammatory stromal polyps (n = 8), epidermic cyst (n = 1), and benign Müllerian papilloma (n = 1). DISCUSSION: Over 15 primitive vaginal tumors, 1/3 was malignant with embryonal rhabdomyosarcoma being the most common. The remaining 2/3 specimens were benign, with stromal inflammatory lesions being the most commonly observed. Fibro-epithelial polyp is a debated entity, which covers a wide histological spectrum, with varying inflammation and stromal cellularity, raising sometimes the question of the differential diagnosis with rhabdomyosarcoma. Stromal cells morphology along with their immunohistochemical profile suggest their reactive myofibroblastic nature. Pseudotumoral inflammatory lesions display very similar histological findings with these entities. A common pathogenesis beginning with an inflammatory process, potentially accelerated by chronic traumatic factors, could be discussed. CONCLUSION: We confirmed the rarity and the diversity of vaginal lesions in children. Vaginoscopy and biopsy sample should be systematic, given the non-specific presentation of tumoral processes. Myogenin immunostain must be systematic in case of vaginal polypoid mass, in order to rule out malignancy.

Disorder of sex development with germ cell tumors: Which is uncovered first?

BACKGROUND: Disorders of sex development (DSD) are rare conditions. Although they are known to predispose to germ cell tumors (GCT), there is a paucity of information regarding the circumstances of DSD discovery. DESIGN/METHODS: All patients with DSD registered in two French pediatric GCT protocols (TGM95 and 13) were analyzed. RESULTS: Sixteen patients were identified among 276 ovarian, 160 testicular, and 24 mediastinal GCT. Eleven phenotypic females (median age 15 years) exhibited gonadal GCT, including 10 with a 46,XY karyotype and gonadal dysgenesis and one with 46XX,45X0 mosaicism. None had genital anomalies, seven had spontaneous pubertal changes, and one had spontaneous menarche. The tumors were bilateral in four cases. DSD was diagnosed after the GCT diagnosis in seven cases. The reasons for karyotyping were bilateral tumors (3), gonadoblastoma/streak gonad/absence of egg follicles (3), or systematic for GCT (1). The karyotyping was performed before the GCT diagnosis in four cases: for polymalformative syndrome (2) or primary amenorrhea (2). Four males (median age 14 years) exhibited mediastinal GCT (metastatic in two cases) indicative of Klinefelter syndrome, despite typical phenotypes in all cases. The remaining patient had severe hypospadias, leading to the discovery of 46,XY/45,X0 mosaicism before the diagnosis of testicular nonseminomatous GCT at 16 years of age. CONCLUSION: DSD are often uncovered at the time of GCT diagnosis (11/16 cases). This should prompt oncologists to rule out a DSD in patients with GCT, even in case of pubertal development. Earlier recognition of Klinefelter syndrome could potentially lead to GCT detection at an earlier stage.

[Atypical genital development and tumor risk]. / Atypie du développement génital et risque tumoral : synthèse de la réunion scientifique du 25 mai 2018 organisée par le « Centre de référence des maladies rares du développement génital du fœtus à l'adulte ¼.

Atypical genital development (AGD), also called disorders of sex development are a set of miscellaneous pathologies who have in common a morphological and/or functional abnormality of the internal and/or external genital organs. The Chicago classification identifies 3 major groups based on karyotype, hormone balance and genetic studies. Some AGD predispose to the occurrence of tumors, mainly malignant germ cell tumors. The tumor risk depends on many factors: the type of AGD, the position of the gonad, the age of the patient, the phenotype, the function of the gonad and the presence of germ cells in the gonad. AGD with the highest tumor risk are those with gonadal dysgenesis, implying an incomplete differentiation of the bipotential gonad (dysplasia). Monitoring of patients with AGD and indication of prophylactic gonadectomies should be individualized according to tumor risk.

Prognostic Value of Ultrasound Grading Systems in Prenatally Diagnosed Unilateral Urinary Tract Dilatation.

PURPOSE: We compared the prognostic value of anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and the Society for Fetal Urology grading system in children with prenatally diagnosed unilateral urinary tract dilatation. MATERIALS AND METHODS: All newborns with prenatally diagnosed unilateral urinary tract dilatation, normal bladder and anteroposterior intrasinus diameter 10 mm or greater on the first postnatal ultrasonography were prospectively enrolled from January 2011 to February 2015. Indications for surgery were recurrent febrile urinary tract infections and/or decrease of relative renal function more than 10% on serial isotope studies and/or increasing anteroposterior intrasinus diameter greater than 20% on serial ultrasounds. Sensitivity, specificity and ROC curves were calculated to evaluate the accuracy of anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology grading system in determining which children would need surgery within 24 months. RESULTS: A total of 57 males and 13 females were included. Of the patients 33 required surgery at a median age of 5 months (IQR 3.8 to 6.4). Urinary tract dilatation remained stable in 14 cases and decreased in 23 with a median followup of 42 months (IQR 25 to 67). Anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology scores were all correlated with the need for surgery. Anteroposterior intrasinus diameter with a threshold of 20 mm had the best prognostic value, with a sensitivity of 81.8% and a specificity of 91.7%. CONCLUSIONS: Our study confirms that the prognostic value was comparable between anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and Society for Fetal Urology grading system in newborns with prenatally diagnosed unilateral urinary tract dilatation. Anteroposterior intrasinus diameter and abnormal parenchymal thickness are the most important ultrasound criteria to identify children at risk for requiring surgery.

Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?

Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and "non-hormonal/non chromosomal" DSD. Questions are summarized for each DSD group with the support of literature and the feed-back of several world experts. Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46,XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.

Laparoscopic-assisted vaginal pull-through: A new approach for congenital adrenal hyperplasia patients with high urogenital sinus.

BACKGROUND: To open vaginal cavity to the pelvic floor is part of surgical treatment for urogenital sinus (UGS) in girls with congenital adrenal hyperplasia (CAH). For high UGS, this operative procedure can be challenging and may jeopardise urinary continence. Combined perineal and laparoscopic approaches could be useful to minimise perineal dissection and to facilitate the vaginal lowering. PATIENTS AND METHODS: We report the procedure of a laparoscopic-assisted vaginal pull-through for supra-sphincteric UGS in a 5-year-old girl with CAH. Laparoscopic dissection of the vagina from the posterior wall of the bladder and urethra, division of the confluence and vaginal pull-through to the perineum are described. DISCUSSION: The technique is derived from laparoscopic-assisted treatment for high ano-rectal malformations. Compared with current procedures for treatment for high UGS, laparoscopic-assisted approach allows mobilising vagina with minimal dissection of perineum and complete preservation of urethra. Another major advantage is to provide a direct vision for dissection of the space between rectum and urethra prior to vaginal pull-through. CONCLUSION: Laparoscopic-assisted vaginal pull-through appears to be an interesting approach for high UGS in CAH patients, reducing dissection and risk of urinary incontinence. This new approach needs to be strengthened by other cases.

Portal vein thrombosis after laparoscopic splenectomy during childhood.

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this complication is reported in 5-10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.10(9)/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.

Mini-invasive surgery for adrenocortical carcinoma in children: is it safe?

Laparoscopy in adrenocortical carcinoma is controversial due to high risk of local recurrence in case of per-operative spillage. We wanted to report the case of a successful laparoscopic transperitoneal adrenalectomy performed in a 3-year-old girl presenting a 5.5-cm adrenocortical carcinoma.

Transrectal small bowel evisceration after abdominal crush injury.

Transrectal small bowel evisceration (TSBE) is a rare entity that can occur after traumatic injuries. It has been described after impalement, sexual assault, blunt abdominal trauma, and swimming pool drain suction. The authors encountered such a case in a 4-year-old by following a crush abdominal injury. A laparotomy was performed. The eviscerated bowel was pulled inside the abdominal cavity, revealing the rectal perforation. A portion of devascularized ileum was resected, a primary bowel anastomosis was performed, and the longitudinal rectal laceration was repaired with a two-layered closure. There was no fecal soilage, and no protective colostomy was performed. The mortality and morbidity of TSBE are usually related with the severity and type of trauma. The mesenteric laceration and concomitant injuries can cause significant bleeding leading to a hemorrhagic shock and multiorgan failure.

Does androgen stimulation prior to hypospadias surgery increase the rate of healing complications? - A preliminary report.

OBJECTIVE: Androgens have a positive effect on penile growth in children, but they may also have a repressive effect on the healing process. The aim of this prospective study was to compare the outcomes of onlay urethroplasty with and without preoperative androgen stimulation in patients with severe hypospadias. PATIENTS AND METHOD: Of 300 severe hypospadias cases treated at a single institution, 126 operated on by the same surgeon had complete follow-up data, and 30 of these received preoperative androgen treatment (human chorionic gonadotrophin and/or systemic testosterone) 1-24 months before surgery. RESULTS: Thirty-five patients presented with a complication (27.7%) of whom 26 (20.6%) had a fistula or dehiscence. Among patients on androgen stimulation there was a 30% healing complication rate (9/30) whereas for those without this was 17.7% (17/96). When androgenic treatment was given > 3 months prior to surgery the healing complication rate was 21.7% (5/23), and when < 3 months prior to surgery the rate reached 57% (4/7). Mean follow up was 41 months (10-97). CONCLUSION: Although the numbers were too small in this series to reach statistical significance, the tissular interactions of androgens in the healing process reported by dermatologists should alert the hypospadiologists and lead to a further prospective study to define the optimal protocol for stimulation of the penis in specific cases without affecting outcome.

Urethral duplication in a 12-year-old child.

Urethral duplication is a rare congenital malformation affecting mainly boys. The authors report a case in a Cameroonian child who was diagnosed and managed at the Gynaeco-Obstetric and Paediatric Hospital, Yaounde. The malformation was characterized by the presence of an incontinent epispadic urethra and a normal apical urethra. We describe the difficulties faced in the management of this disorder in a developing country.

Modified technique of meso-Rex shunt in case of insufficient length of the jugular vein graft.

UNLABELLED: Meso-Rex shunt (MRS) can relieve portal hypertension and restore a physiological portal flow in patients with portal vein thrombosis. We describe a technical variant where the autologous internal jugular vein (IJV) was too short to bridge the superior mesenteric vein (SMV) and the Rex recessus. PATIENT: A 15-year-old boy with portal cavernoma had several episodes of gastrointestinal bleeding despite repeated sclerotherapy. Preoperative assessment, including retrograde transjugular portography, showed persistent esophageal and gastric varices, severe hypertensive gastropathy, obstructed portal vein, patent SMV and splenomesenteric confluence, patent intrahepatic portal branches, and normal transhepatic pressure gradient. An MRS was planned. The left IJV was retrieved from its infracranial part to its confluence with subclavian vein. After performing the Rex recessus to IJV graft anastomosis, the IJV graft proved to be too short for classical end-to-side anastomosis onto the SMV. After clamp testing showing good tolerance of the small bowel, the proximal jejunal branches of the SMV were tied, the proximal SMV was mobilized and transsected 4 cm below the pancreas, and an end-to-end anastomosis between SMV and IJV was performed. Portal pressure decreased from 23 to 13 mm Hg, and intraoperative Ultra Sound Doppler (US Doppler) showed good flows in the shunt. Postoperative course was uneventful, and 1 year after surgery, the child is clinically well, off medication, with a patent shunt, and no portal hypertension. CONCLUSION: This modified MRS technique may be useful when the autologous IJV graft is too short, avoiding the need for prosthetic conduits and prolonged postoperative anticoagulation.

Minimally invasive surgery for anorectal malformation in boys: a multicenter study.

INTRODUCTION: The laparoscopic pull-through for high imperforate anus in boys has recently been developed in France and Benelux. The purpose of this study was to evaluate technical difficulties and surgical complications of this approach. PATIENTS AND METHODS: This report was based on a retrospective study from January 2002 to December 2007,including 34 patients from 10 centers. The rectourinary fistula found in 33/34 cases was bulbar in 10, prostaticin 20, and cervical in 3. RESULTS: Patients were 3.7 months old (range, 1.6-10.4) at surgery. Fistula was cut without closure in 11 cases or divided and closed on the genitourinary side with a suture in 15, with clips in five and linear stapler in two.Median operative duration was 152 mm (range, 80-410). The main per-operative complication, occurring in five cases, was leakage of stools from the rectum after the fistula section, requiring in one case a second laparoscopic procedure for washing and for all five cases intravenous antibiotics. Median hospital stay was 6 days(range, 3-15). During the follow-up, patients experienced eight anal stenoses and three prolapses, requiring in three cases perineal redo surgery. CONCLUSION: The laparoscopic approach of high imperforate anus allowed an easy control of the fistula and helped to determine the accurate passage through the levator ani. Nevertheless, technical points have to be improved,such as coprostasis before fistula section to avoid stool leakage, rectum tacking to prevent prolapse,and calibration of muscle channel to avoid stenosis.

Early elimination dysfunction associated with cephalic anomalies: is there a link?

UNLABELLED: Elimination dysfunction in children can be related to three main aetiologies: 1) spinal cord anomalies, 2) social and environmental disorders, and 3) syndromic elimination disorders. From this last group, we report cases of a previously undescribed combination of elimination disorders and cephalic anomalies symptoms which may constitute a proper entity for which conventional treatments may fail. A comprehensive review of congenital elimination disorders is given. PATIENTS AND METHODS: Six patients (four boys, two girls) presenting with early elimination dysfunction associated with cephalic anomalies were assessed and treated between 1994 and 2005. None presented with identified lower urinary tract obstruction or spinal cord anomalies. Follow up ranged between 5.5 and 11.5 (mean 6.7) years. RESULTS: All six had early elimination disorders, represented by urine retention, urinary tract infections, constipation and soiling. All had facial dysmorphy and cerebral anomalies with developmental delay of varying severity. All had a dilated urinary tract, with severe vesicoureteral reflux in five and one megaureter without reflux. All had abnormal renal isotope scans, two associated with chronic renal failure. The family medical history was significant in some cases. Treatment included early urinary diversion, and there was a high failure rate for ureteral reimplantation. CONCLUSION: The combination of congenital elimination dysfunction with facial anomalies, developmental retardation, cephalic anomalies, abnormal urinary tracts, without identified spinal cord disorders or lower urinary tract obstruction, may represent a defined population of children. Identification may lead to early elimination support measures including temporary bladder diversion, Mitrofanoff diversion, alpha blockers and bowel transit medications.

Laparoscopic resection of abdominal neuroblastoma.

BACKGROUND: Since indications for laparoscopic adrenalectomy have progressively expanded to pediatric surgery, preliminary reports have studied the laparoscopic approach for abdominal neuroblastoma (NB). We aimed to report on the indications and the results of laparoscopic resection in a large series of abdominal NBs. METHODS: A retrospective multicenter study included 45 children with abdominal NBs (28 localized, 11 stage 4, 6 stage 4s) and laparoscopic resection of their abdominal primary tumor. Primary site of the tumor was the adrenal gland in 41 cases and retroperitoneal space in 4. The median age at surgery was 12 months (1-122); median tumor size was 37 mm (12-70). Resection was performed through transperitoneal laparoscopy (n = 38) or retroperitoneoscopy (n = 7). RESULTS: Complete macroscopic resection was achieved in 43 of 45 children (96%). The median duration of pneumoperitoneum was 70 min (30-160), and the length of hospital stay was 3 days (2-9). Four procedures (9%) were converted to open surgery, and tumor rupture occurred in three cases. Of the 28 children with localized disease, there was a 96% overall survival (OS) rate after a median follow-up of 28 months (4-94). There was one local relapse in this subgroup, with subsequent complete remission. For the entire 45-children cohort, four children died and three presented a recurrence resulting in OS, disease-free survival, and event-free survival rates of 84% +/- 8.1, 84% +/- 8.2, and 77% +/- 9.1 respectively. CONCLUSION: Laparoscopic resection of abdominal primary allows effective local control of the disease in a wide range of clinical situations of neuroblastoma, with an acceptable morbidity.

Management of severe hypospadias.

Many classifications of hypospadias have been published, mainly based on the position of the ectopic meatus, which is an insufficient criterion to define the severity of this malformation. What really marks the proximal landmark of this malformation is the level of division of the corpus spongiosum, which is always proximal to the ectopic meatus. In this article, we will focus on the most severe forms of hypospadias which include those with a proximal division of corpus spongiosum (below the midshaft), important chordee and a poor development of the ventral radius, reflecting a marked hypovirilization of the genital tubercle, and cripple hypospadias resulting from several previous failed surgical procedures. The principle of hypospadias surgery will be reviewed together with the outcome of the current surgical techniques. Furthermore, common complications will be outlined. There is no minor or major hypospadias and all forms require a solid experience of the surgeon, as minor looking hypospadias may turn out to be far more complex to repair than they appear once the ventral radius of the penis has been dissected.