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Background: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years., Material and Methods: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences., Results: Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03)., Conclusions: SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option., (© 2016 Wiley Periodicals, Inc.)

Mesoblastic nephroma is by far the most frequent intrarenal fetal tumor. To the best of our knowledge, we report the first case of a newborn with an intrarenal neuroblastoma that was discovered prenatally. An intrarenal echogenic and homogenous mass was observed on routine prenatal ultrasonography, corroborated by magnetic resonance imaging, in a 30-week gestation fetus. A male weighing 3280 g was born with elevated blood pressure and cardiac failure. Postnatal ultrasound confirmed a left intrarenal tumor with microcalcifications and perirenal adenopathy. An open total left nephrectomy by laparotomy was performed. The pathologic study reported that the mass was an intrarenal neuroblastoma with local and regional invasion. Immediate postoperative urine analysis revealed a high level of vanillylmandelic acid, and blood samples showed high levels of normetanephrine. The purpose of this report is to demonstrate that prenatal intrarenal neuroblastoma can clinically and radiologically mimick a mesoblastic nephroma. High blood pressure, calcifications, and lymphadenopathy on ultrasound should raise the index of suspicion for a possible malignant process. Preoperative measurement of urinary vanillylmandelic acid (VMA) and metanephrines should be performed if the diagnosis is in doubt., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Introduction: Intraabdominal cystic lymphangioma is a rare benign lesion with a variable clinical presentation. The aim of this study was to evaluate the natural outcome of this lesion and to propose a decision-making protocol for antenatal suspected intraabdominal lymphangioma. We also would like to emphasize the role of laparoscopy in treatment., Materials and Methods: This retrospective study examined 14 cases of intraabdominal cystic lymphangioma in children underwent to our observation, at the Pediatric Surgery Department in Lapeyronie Hospital, Montpellier between 1996 and 2007., Results: Of the 14 patients, 12 were operated on (7 by laparoscopy and 5 in open surgery). In 2 patients we didn't perform intervention because the lesion was localized in the mesenteric root: we observed a regression of the lesion on US-scan., Conclusion: The intraabdominal cystic lymphangioma is a rare lesion which is now sometimes suspected on antenatal US scan; consequently we observed at the present time an increasing number of lesion potentially asymptomatic: in these case the management has to be established and a risk-benefit balance has to be made before a surgical intervention. Our experience is limited to a small number of cases, and it is too premature to describe any final conclusion; however our suggestion is that clinical course of cystic abdominal lymphangioma is unknown, and we propose that if no complication occurs, a clinical and ultrasonographic monitoring should be done in case of mesenteric root localisation.

Background: Nonoperative treatment is the gold standard approach to treat blunt liver trauma (BLT) in hemodynamically stable children. The purpose of this study was to evaluate the incidence, risk factors, timing for appearance, diagnostic modalities, management and outcome of hepatic complications secondary to such approach., Methods: This retrospective study included children admitted at Montpellier University Hospital for BLT over a 10-year period. All hemodynamically stable children were initially conservatively treated., Results: A total of 51 children underwent nonoperative treatment for BLT during the study period. The success rate was 94.1% (48/51). Three patients (5.9%) presented 13 complications related to hepatic injuries and required secondary surgical treatment. These 3 patients presented grade 3 or higher liver lesions. Others factors identified as predictive of complications included initial hemodynamic instability (responding to the first resuscitative measures), presence of peritoneal irritation at first examination, severe hemoperitoneum, an initially low haemoglobin level (< 8.5 g/dl) and need for important transfusions during the first 48h (> 10 cc/kg). The median interval for appearance of complications was 19 days after injury (range 1-60 days). Complications were successfully treated using minimally invasive techniques in 69.2% of cases (9/13). The rest of the complications (30.8% : 4/13) were surgically treated. All children included in our study had favourable outcomes (follow-up 1-54 months)., Conclusions: This series fully validates conservative approach of BLT in hemodynamically stable children. Complications of such approach are uncommon; many can be successfully treated using minimally invasive techniques with very satisfying results.

Aims: To analyze the results of treatment of major renal injuries according imaging data in order to determine their function after follow-up., Patients and Methods: This is a retrospective study of 22 cases of fracture of the kidney (grade V) in two pediatric surgical services that were reviewed over a period of 16 years. After initial conservative treatment in 19 patients (86.5%), a scan and/or Uro-MRI were realized in all patients in monitoring evolving. Three children with vascular injury were treated by interventional radiology. The morphology and functional evolution of the injured kidney were determined., Results: A DMSA scan investigation was performed in 21 patients (95.5%) associated with Uro-MRI in two cases; one patient was only explored with Uro-MRI. A complete restitution of the renal parenchyma was confirmed in 10 children (45.5%), we noted an atrophy of the upper pole in 30%, a lower pole atrophy in 4.5%, two complete renal atrophy in 9%. An urinoma was present in six patients (27%) that required drainage in five cases and declined during the surveillance in one case. Normal function of the injured kidney was noticed in half of grade V (11 of 22 patients) with a mean follow-up of 19 months. None of our patients did present hypertension., Conclusion: Non-operative conservative treatment in severe renal trauma was efficient, morphological and functional sequelae were present in 50% on scintigraphy and/or Uro-MRI., (Copyright © 2011. Published by Elsevier Masson SAS.)

Aim of the Study: To evaluate the impact of prenatal diagnosis on the epidemiology and outcome of children with posterior urethral valves (PUV), considering that today termination of pregnancy may be proposed in the most severe cases., Patients and Methods: Forty-three cases of patients with PUV were diagnosed between 1998 and 2007 in the Languedoc-Roussillon region. In this study, we detailed the prenatal data and postnatal outcome of those patients with a mean follow-up period of 7.6 years., Results: Medical interruption of pregnancy was performed in 15 severe cases. One death in utero was also observed. Twenty-seven patients were managed postnatally and one child died during the neonatal period because of lung hypoplasia. For the 26 remaining patients at the end of the follow-up period, 5 had mild renal insufficiency with a creatinine clearance calculated with the Schwartz formula of less than 80 ml/min/1.73 m2. One reached end-stage renal failure at the age of 7 years. Proteinuria and hypertension were rare., Discussion and Conclusion: Today, because of the increase in medical termination of pregnancy in the most severe cases, most of the children born alive with PUV experienced a better renal prognosis when compared with previous data. Prenatal diagnosis and therapeutic progress significantly modified the epidemiology and outcome of this disease. Nevertheless, the best predictor of renal outcome remains renal function at 1 year of age.

Purpose: This retrospective study reports our experience in laparoscopic approach after failure of antireflux surgery. It evaluates the results and circumstances of failure of the initial procedure to understand indications of refundoplication., Methods: Four hundred seventeen patients were operated on for a gastroesophageal reflux disease (GERD) by laparoscopy in our unit from August 1993 to February 2005. Thirty redo procedures (7.19%) were performed. The indications were 24 (80%) recurrent reflux resistant to the medical treatment and 6 (20%) severe dysphagia resistant to iterative dilatations. The average age was 57.6 months. Nineteen patients (63%) were males and 11 patients (37%) were females. The time between the first and the redo procedure was an average of 16 months. Ten (33%) of them were neurologically impaired (NI); in 7 patients, a percutaneus gastrostomy was also associated. The techniques previously used were 13 Nissen, 7 Nissen-Rossetti, and 10 Toupet., Results: The redo procedure was performed by laparoscopy in 27 cases. A conversion was necessary in 3 children because of a difficult dissection. In the 24 cases of recurrent reflux, we realized a valve disassembly, reconstruction of hiatus and Nissen refundoplication. In 3 cases of dysphagia, the release of the hiatus needed a complete valve redo. The mean operative time was 140 minutes (110 to 240 minutes). The sole complication was a pleural perforation. All patients were fed on the first day. The mean duration of hospitalization was 3.1 days. The follow-up was from 48 months to 12 years. A new recurrent reflux occurred in 6 patients; 5 of them were NI., Conclusion: The failure rate of the antireflux laparoscopic surgery is similar to the conventional surgery. The redo procedure is possible by laparoscopic with a success rate similar to the open redo surgery. The rate and morbidity of complications are acceptable and decrease with experienced surgeons. The indications of redo procedures should be similar to conventional surgery.

Objective: To determine the genetic cause of primary amenorrhea in a 46,XY adolescent girl., Design: Case report., Setting: Pediatric endocrinology and gynecologic unit of an academic hospital., Patient(s): A 16-year-old adolescent referred for primary amenorrhea., Intervention(s): Endocrine and surgical investigation, SRY mutational analysis., Main Outcome Measure(s): Plasma gonadotropin levels, estradiol and testosterone levels, and pathologic findings., Result(s): We report a new mutation of the SRY gene in a 46,XY sex-reversed patient. We observed two unusual features. First, partial pubertal development has rarely been described in association with SRY gene mutation. Second, the location of the mutation was in the HMG box region of the SRY gene, in contrast to the other partial cases of 46,XY gonadal dysgenesis. In addition, the presence of a gonadoblastoma underlines the necessity of removing the gonads quickly in 46,XY sex-reversal cases, and raises several questions about the role of the SRY gene in the development of such tumors., Conclusion(s): Partial pubertal development in a 46,XY sex-reversed patient does not exclude SRY gene mutation.

Purpose: Controversy persists concerning the management of post-appendectomy intra-abdominal abscesses. We hypothesised that most of these abscesses can be successfully managed by antibiotic treatment alone, avoiding the complications of surgical treatment., Methods: Hospital records of children treated in our unit for intra-abdominal post-appendectomy abscesses over a 6-year period were reviewed retrospectively., Results: This study investigates a series of 26 children from 2 to 15 years of age presenting with one or more post-appendectomy intra-abdominal abscesses. After an average delay of 7 days after initial surgery, 23 children had developed an isolated abscess, while 3 children had multiple abscesses. Twenty-two patients (84.8 %) were treated conservatively by intravenous triple antibiotic therapy alone. Complete clinical, radiological and biological resolution of the abscesses was obtained in all of these children after a mean hospitalisation of 8 days. Four children (15.2 %) were treated surgically: three children with a stable patient status and one child with septic shock requiring urgent surgery., Conclusion: The results suggest that intravenous triple antibiotic therapy alone is an efficacious first-line treatment in children developing intra-abdominal abscesses following appendectomy. Surgical intervention is rarely necessary except in patients with an alarming patient status or with signs of septic shock.

Background: Complex procedures for managing congenital abnormalities are reported to be feasible. However, neonatal videosurgery involves very specific physiologic constraints. This study evaluated the safety and complication rate of videosurgery during the first month of life and sought to determine both the risk factors of perioperative complications and the most recent trends in practice., Methods: From 1993 to 2005, 218 neonates (mean age, 16 days; weight, 3,386 g) from seven European university hospitals were enrolled in a retrospective study. The surgical indications for laparoscopy (n = 204) and thoracoscopy (n = 14) were congenital abnormalities or exploratory procedures., Results: Of the 16 surgical incidents that occurred (7.5%), mainly before 2001, 11 were minor (parietal hematoma, eventration). Three neonates had repeat surgery for incomplete treatment of pyloric stenosis. In two cases, the incidents were more threatening (duodenal wound, diaphragmatic artery injury), but without further consequences. No mortality is reported. The 26 anesthetic incidents (12%) that occurred during insufflation included desaturation (<80% despite 100% oxygen ventilation) (n = 8), transient hypotension requiring vascular expansion (n = 7), hypercapnia (>45 mmHg) (n = 5), hypothermia (<34.9 degrees C) (n = 4), and metabolic acidosis (n = 2). The insufflation had to be stopped in 7% of the cases (transiently in 9 cases, definitively in 6 cases). The significant risk factors for an incident (p < 0.05) were young age of the patient, low body temperature, thoracic insufflation, high pressure and flow of insufflation, and length of surgery., Conclusion: Despite advances in miniaturizing of instruments and growth in surgeons' experience, the morbidity of neonatal videosurgery is not negligible. A profile of the patient at risk for an insufflation-related incident emerged from this study and may help in the selection of neonates who will benefit most from these techniques in conditions of maximal safety.

Purpose: Intravaginal or extravaginal spermatic cord torsion is a diagnostic challenge for the surgeon and radiologist. Color Doppler sonography can be inaccurate, leading to dangerous false-negative results. To date, no single reliable test has been able to provide 100% diagnostic accuracy. The direct visualization of the twisted cord during emergency high resolution ultrasonography has been proposed to avoid systematic and abusive surgical exploration. The aim of this multicenter study was to assess the validity and reproducibility of high resolution ultrasonography based management of acute scrotum in children., Materials and Methods: A total of 919 patients from 11 European university hospitals underwent color Doppler sonography and high resolution ultrasonography for acute scrotum between 1992 and 2005. The spermatic cord was studied along its complete length to detect a spiral twist. The surgical findings were correlated with the preoperative results., Results: A total of 208 patients had spermatic cord torsion proved at surgery. Intratesticular vascularization was absent in the affected testis in only 158 cases (76%). In contrast, high resolution ultrasonography detected the twist as a snail shell-shaped mass, measuring 7 to 33 mm, in 199 patients (96%). High resolution ultrasonography revealed a linear cord for all other causes of acute scrotum (711 patients) with a specificity of 99%. The radiologist training level was the best predictive factor of high resolution ultrasonography reliability (p <0.05)., Conclusions: High resolution ultrasonography based management of acute scrotum is reliable and reproducible. Thanks to its high sensitivity and specificity for the diagnosis of spermatic cord torsion, high resolution ultrasonography can significantly improve the management of acute scrotum in children.

Purpose: The indications for thoracoscopy remain imprecise in cases of pleural empyema. This study aimed to identify preoperative prognostic factors to help in the surgical decision., Methods: From 1996 to 2004, 50 children with parapneumonic pleural empyema underwent thoracoscopy either as the initial procedure (n = 26) or after failure of medical treatment (n = 24). Using multivariate analysis, we tested the prognostic value of clinical and bacteriological data, the ultrasonographic staging of empyema, and the delay before surgery. Outcome measures were technical difficulties, postoperative complications, time to apyrexia, duration of drainage, and length of hospitalization., Results: The clinical and bacterial data did not significantly predict the postoperative course. Echogenicity and the presence of pleural loculations at ultrasonography were not independent significant prognostic factors. A delay between diagnosis and surgery of more than 4 days was significantly correlated (P < .05) with more frequent surgical difficulties, longer operative time, more postoperative fever, longer drainage time, longer hospitalization, and more postoperative complications, such as bronchopleural fistula, empyema relapse, and persistent atelectasia., Conclusion: The main prognostic factor for thoracoscopic treatment of pleural empyema is the interval between diagnosis and surgery. A 4-day limit, corresponding to the natural process of empyema organization, is significant. The assessment of loculations by ultrasonography alone is not sufficient to predict the postoperative course.

Objective: To evaluate the feasibility and results of thoracoscopy in various types of esophageal atresia (EA)., Materials and Methods: From April 2001 to August 2002, 5 patients with EA were treated by thoracoscopy. Their mean gestational age was 38 weeks and mean birth weight was 2700 g. Two patients had short-gap atresia with tracheo-esophageal fistula (type III according to Ladd's classification). Three had long-gap atresia: 2 with low fistula to the carina (type IV) and 1 without fistula (type I). Patients were placed in a prone position with the right side elevated at 80 degrees . Four intrapleural ports were necessary. The fistula when present was dissected and sutured with intrathoracic knots and esophageal anastomosis performed in the same manner., Results: Positive airway pressure increased in all patients after insufflation but was kept in a safe range to prevent lung injury. An esophageal anastomosis was performed in 3 cases (2 short gaps and 1 long gap). Oral feeding started on day 6, and their mean length of hospital stay was 14 days. For one child with type IV EA, the anastomosis was impossible because of a long gap confirmed by an immediate thoracotomy. The ends were just approximated. A "spontaneous" fistula developed, and normal feeding was possible 2.5 months later. For the child with type I EA, the pouches could be only approximated at 2 months of age. A spontaneous fistula developed with a stenosis. A redo anastomosis by open surgery allowed for normal feeding., Conclusion: The thoracoscopic repair of an esophageal atresia is a reasonable choice for experienced surgeons treating patients, including those with long gaps.

Objectives: Video-surgery in neonates is recent. Data on the respiratory, hemodynamic, and thermic effects during the first month of life are still sparse. This study aimed to evaluate the tolerance of video-surgery in neonates and to determine the risk factors of per-operative complications., Methods: From 1994 to 2004, 49 neonates (mean age: 11 days; weight: 3285 g) underwent 50 video-surgical procedures. Indications for laparoscopy were duodenal atresias, volvulus with malrotation, pyloric stenosis, gastroesophageal reflux, cystic lymphangiomas, ovarian cysts, biliary atresia, and congenital diaphragmatic hernias; indications for thoracoscopy were esophageal atresias and tracheoesophageal fistula., Results: Median operative time was 79 minutes. Mean insufflation pressure was 6.7 mm Hg (range: 3-13). Oxygen saturation decreased, especially with thoracic insufflation or high-pressure pneumoperitoneum. Systolic arterial pressure, which decreased in 20% of the patients, was controlled easily with vascular expansion. Thermic loss (mean postoperative temperature: 35.6 degrees C) was proportional to the duration of insufflation. No surgical incident was noted. Ten anesthetic incidents occurred (20%), 3 of which required temporary or definitive interruption of insufflation (O2 saturation <70%). Risk factors for an incident were low preoperative temperature, high variation of end-tidal pressure of CO2, surgical time >100 minutes, thoracic insufflation, and a high oxygen or vascular expansion requirement at the beginning of insufflation., Conclusion: The neonate's high sensitivity to insufflation is an important limiting factor of video-surgery. The described profile of the neonate at risk may help to reduce the frequency of adverse effects of this technique and improve its tolerance.

Objective: The transverse outer preputial (TOP) island flap, a simple procedure inspired by the double-faced island flap, is proposed as a substitute for the Byars repair to provide skin coverage for most of the current reconstructive techniques for hypospadias., Patients and Methods: From 1996 to 2003, 108 consecutive children (aged 1-8 years) had a primary repair of various forms of hypospadias with different degrees of chordee; the TOP island flap was used in 66. The surgical technique is based on the use of a dorsal transverse skin flap mobilized with its own vascular pedicle from the outer foreskin, then ventrally rotated longitudinally after removing the inner part of the prepuce., Results: Partial necrosis of the lateral border of the flap was infrequent (<2%) and did not induce urethral complications. There was no total necrosis in the series. The rate of fistula was comparable with children operated using other techniques (8%). The cosmesis was good, with no ventral bulkiness or hypertrophic scarring., Conclusions: The TOP island flap is a safe method for covering a new urethra and penile skin defects, with good cosmetic results.

Introduction: Cavitary necrosis remains a rare complication of bacterial pneumonia in children. Conservative medical treatment and radical surgical treatment with lung resection are the current therapeutic choices. Evaluation of thoracoscopy for this pathology has not yet been reported. We describe 3 cases., Materials and Methods: Between January 2001 and January 2002, 3 children (1, 2, and 3 years old) were admitted to our institution with necrotizing pneumonia. The diagnosis was based on injected computed tomography (CT) scan showing pulmonary condensation, intra-parenchymal bullae, and hypovascularization. In addition to an adapted antibiotic therapy, a thoracoscopic approach was decided on within 24 hours of diagnosis, with extensive decortication, ablation of superficial necrotic debris, irrigation, and drainage., Results: No conversion to open thoracotomy or lung resection was needed. Admission to the intensive care unit was unnecessary. On average, apyrexia was reached on postoperative day (POD) 2 and tube drainage was removed on POD 15. Mean follow-up at 16 months showed excellent lung re-expansion with no relapse., Conclusion: On the condition that the decision is made quickly, thoracoscopy may be a valuable treatment option in childhood necrotizing pneumonia, as it hastens recovery and avoids lung resection. Injected CT scan allows an early diagnosis and we propose the first 24 hours after diagnosis as the optimal period for thoracoscopy because of the rapid natural course of lung gangrene.

Background: This study aimed to evaluate the optimal conditions for laparoscopic management of neonatal subacute volvulus with malrotation., Methods: Between 1994 and 2002, 13 neonates with midgut volvulus and malrotation entered the authors' institution. Five of these neonates met the eligibility criteria for laparoscopy: good hemodynamic parameters, no gut perforation, and no severe ischemic distress of the bowel shown on preoperative ultrasonography., Results: The results were excellent for only three infants, associated with a shorter postoperative course. One underwent reoperation for a residual duodenal band, and one conversion to open laparotomy was necessary. The predictors of success were no difficulty identifying the abnormal anatomy, no important chylous stasis, and the learning curve of the surgeon., Conclusions: Despite its previously reported feasibility, neonatal laparoscopy for volvulus with intestinal malrotation is appropriate only for a small number of patients. Strict selection criteria with a clear understanding of the optimal conditions for success may improve the outcome.

H-type tracheoesophageal fistula (H-TEF) without esophageal atresia makes up 4% to 5% of esophageal congenital abnormalities. The authors present the thoracoscopic treatment of a 2.47-kg newborn boy with a fistula between the second and third thoracic vertebrae diagnosed by esophagography. Four trocars were used for fistula closure, and tracheal and esophageal suturing were accomplished without intraoperative incident. Five days after surgery, results of a barium swallow excluded anastomotic leaks. The chest tube was removed, and oral feeding was initiated.

Purpose: Many surgeons advocate systematic exploration for acute scrotum rather than risking a misdiagnosis of testicular torsion. Study of testicular vascularization with color Doppler sonography (CDS) can be inaccurate, leading to dangerous false-negative results. We determine whether direct visualization of the twisted cord during emergency high resolution ultrasonography (HRUS) is a reliable sign to diagnose the torsion and whether its absence can dispense with unnecessary surgery., Materials and Methods: From 1993 to 2002 an average of 35 patients per year presented with acute scrotum, and 44 patients had spermatic cord torsion. CDS and HRUS were performed in all cases. Transversal and longitudinal scans on both sides of the scrotum permitted comparison of testicular echogenicity, size and vascularization. The spermatic cord was studied along its complete length to detect spiral twist. The surgical findings were correlated with the preoperative results., Results: Spermatic cord torsion at surgery was confirmed in all 44 cases. The time lost by the examination was never more than 30 minutes. Intratesticular blood flow was absent in the affected testis in 31 cases, and CDS was unreliable in the others. In all cases, regardless of CDS findings, HRUS succeeded in detecting the twist as a snail shell-shaped mass measuring 11 to 33 mm., Conclusions: The finding of a twisted spermatic cord is a highly reliable sign for the diagnosis of testicular torsion. Whereas normal intratesticular perfusion does not dispense with emergency exploratory surgery, direct and complete visualization of a nontwisted spermatic cord strongly indicates that surgery is unnecessary. Strict conditions are required, including time spent on HRUS should not exceed 30 minutes, which generally can only be achieved by a senior pediatric radiologist.

Although cryptorchidism is probably the most frequent genital anomaly encountered in boys, this pathology remains largely unexplained and its long-term prognosis is still uncertain. Despite the relative easiness of this diagnosis, attention should be paid to some clinical traps, which can mislead the sexual identity. Indeed, beside the isolated common cryptorchidism, many troubles of the testicular migration can be associated with various malformative syndromes and can be named as "cryptorchidism symptoms". Most of these syndromes are obvious, but some can be suspected only by the way of an abnormal descent of testis. Cryptorchidism, which is systematically screened out during any pediatric consultation, should play the role of clinical marker for various endocrine or genetic disorders. Among them, the intersex states should be underlined to avoid the harmful effects of misdiagnosis. We dealt with this subject through a clinical and practical approach.

Objective: Outpatient surgery in pediatrics is attractive for several reasons. Although 6-8 months has been generally acknowledged as the lower age limit, no minimal age has been clearly established. This study evaluated the outcomes and limits of outpatient surgery in infants from 4 months to 1 year of age., Material and Methods: Between 1996 and 2003, 935 patients were admitted to our hospital for outpatient surgery; of these, 152 were between 4 months and 1 year (mean: 6 months). The indications were essentially surgery to the external genitalia and surgery to remove hernias. The following were exclusion criteria: age below 4 months, ASA scores equal to or above 3, and premature birth with either respiratory distress syndrome or a near-miss of sudden death., Results: More than 98% of the infants met the criteria for outpatient surgery. Cancellations (1.97 vs. 0.51%, P > 0.05), conversions to classic hospitalization (0.65 vs. 0.37%, P > 0.05), postoperative complications (0.65 vs. 0.25%, P > 0.05), rehospitalization (0.65 vs.0.25%, P > 0.05) and relapse of the initial pathology (0.65 vs. 0.63%, P > 0.05) were not more frequent in these infants than in infants over 1 year., Conclusion: Pediatric outpatient surgery can be proposed from the age of 4 months without increasing the postoperative risk, either anesthetic or surgical, included for selected former premature infants.

Background: Thoracoscopy may be required for resistant empyema in children. This study aimed to determine the advantages of thoracoscopy performed soon after diagnosis and its ideal timing., Methods: Between 1996 and 2002, 21 children who had undergone thoracoscopy as an initial procedure or after failure of medical treatment were retrospectively reviewed. The study compared outcome of early thoracoscopy (fewer than 4 days after diagnosis) and that of later surgery. The 4-day limit was chosen for physiopathologic reasons (organization of pleuresia in 72 h)., Results: In the early thoracoscopy group, the findings showed shorter operative time (p = 0.03) and postoperative hospital stay (p < 0.05), fewer technical difficulties, fewer complications, and no recourse to other surgical procedures., Conclusions: Early thoracoscopy is greatly beneficial for children with empyema by shortening disease progression. An initial short trial of medical treatment for nonorganising empyema may be attempted on the condition that thoracoscopy not be delayed more than 4 days.

Ultrasonography has radically changed the approach of these rare malformations, still diagnosed with difficulties in spite of modern iconography. Bladder anomalies may easily be discovered when associated with otherwise obvious malformations of genitalia. The rest must be suspected for non-specific signs as micturition troubles, multiple urinary tract infections, chronic renal failure. The abnormal bladder is the only common feature of a polymorphic spectrum of diseases including complete or partial duplication or septum, congenital diverticula, vesicovaginal fistula, agenesis and urachal anomalies. Treatment must be accorded to each case and it needs perfect knowledge of open and endoscopic bladder surgery.

Ambiguous genitalia in the newborn need immediate and rational management. This complex situation requires a strategy of clinical, hormonal, genetic, molecular, and radiographic investigation to determine the etiology of the intersex state and orient the therapeutic approach. Physical examination is key to diagnosis. Careful palpation to locate gonads at the genital folds or in the inguinal region provides the first element for diagnostic orientation. If gonads are absent, a diagnosis of female pseudohermaphroditism seems advisable; if gonads are palpated, a diagnosis of male pseudohermaphroditism is more appropriate. Karyotyping is systematic while polymerase chain reaction (PCR) analysis of the SRY gene provides information about the presence of a Y chromosome within 1 day. Hormonal investigation should be based on clinical and genetic orientation. Substantially elevated plasma 17-OH progesterone will confirm the diagnosis of congenital adrenal hyperplasia due to deficiency in 21-hydroxylase. Testicular stimulation with human chorionic gonadotropin (hCG) will determine the functional value of testicular tissue. Exploration of the genitourinary axis is principally carried out by ultrasound and genitography. By the end of these investigations, the medical team should be able to give a precise diagnosis. Female pseudohermaphroditism may be due to excess fetal androgens (congenital adrenal hyperplasia), increased androgen production of maternal origin, or placental androgen excess. In male pseudohermaphroditism, if testosterone rises normally after hCG stimulation, androgen resistance is indicated. If it does not rise after this test, either testicular dysgenesis or disturbance in testosterone biosynthesis may be responsible. The assignment of sex for rearing must be guided by the etiology of the genital malformation, the anatomic condition, and family considerations. In cases of female pseudohermaphroditism, the newborn should always be declared to be of female sex at birth. In cases of male pseudohermaphroditism, great care should be taken in the declaration of male sex: the potential for reconstructive surgery and the pubertal "programmed" response of the external genitalia to endogenous and exogenous testosterone are determinant. Management of ambiguous genitalia in the newborn requires an entire multidisciplinary team in every step of the diagnostic procedure, the choice of sex assignment, and the treatment strategy.

Background: Little attention has been given to abdominal hollow viscus injuries in pediatric literature. The purpose of this report is to describe their main features, and the current methods of diagnosis and treatment., Methods: We reviewed our experience with 16 cases of blunt injuries involving the gastrointestinal (GI) and biliary tract in the last seven years., Results: Male to female ratio was 0.68 and mean age 9.4 years. The site of injury was the stomach in 2 cases, duodenum in 5, jejunum or ileum in 7, caecum in 2, left mesocolon in 2, and gallbladder in 1. Diagnosis of the 8 perforations was clinically suspected in 6, and confirmed by radiography in 6 or surgery in 2, with a mean delay of 0.42 days. Diagnosis of the 11 hematomas was clinically suspected in 6, and confirmed by radiography in 9 or surgery in 2, with a mean delay of 12.6 days. Hematomas were managed nonoperatively, and perforations were surgically or laparoscopically closed. Mortality was 1/16 by pancreatic injury, and morbidity was 4/16., Conclusion: Incidence of blunt hollow viscus injuries is low in children. Hematomas are easily recognized by modern radiography, and simply managed nonoperatively. Diagnosis of perforations still remains difficult. This results in dangerous delays for lesions which need prompt repair.

Purpose: This retrospective study describes the 5-year experience of a single surgeon with 142 consecutive laparoscopic fundoplications in children and analyzes the results in terms of the surgeon's learning curve and the choice of technique., Methods: The patients were 44 girls (40%) and 98 boys (60%) with an age range of 3 months to 18 years (mean, 5.35 years). Indications for surgery included medically refractory reflux associated with vomiting, pneumopathy, otorhinolaryngologic pathology, failure to thrive, esophagitis, apnea and bradycardia, or anemia. The laparoscopic surgery used 5 trocars of 5 mm or 3 mm, with a 30 degrees telescope., Results: Laparoscopic fundoplication was successful in 139 cases. The authors performed 13 Nissen techniques without short gastric vessel division (SGVD), 47 Toupets without SGVD, 9 Toupets with SGVD, and 70 Nissens with SGVD. Mean operating time was 105 minutes (range, 45 to 300). Mean time was 125 minutes for the first 60 cases and 93 minutes for the other 79 cases). Intraoperative and postoperative complication rates were 0.5% and 2%, respectively, and the complications occurred in the first 60 cases. Mean postoperative hospital stay was 3 days (range, 2 to 14)., Conclusions: The rate of complication caused by the laparoscopic procedure was acceptable, and it decreased with the surgeon's experience. Complications and conversions to open technique occurred only in the beginning of the operator's experience. Dysphagia, epigastric pain, gas bloating, and early reflux recurrences were noted among the first 60 cases but seemed to be consecutive to the fact that the gastric vessels were not divided. J Pediatr Surg 36:921-926., (Copyright 2001 by W.B. Saunders Company.)

To gain further information concerning the regulation by androgen of AR mRNA expression in cultured genital skin fibroblasts (GSF), we first developed a quantitative reverse transcription-competitive polymerase chain reaction (RT-PCR). This method used an ethidium bromide stain analysis of the PCR products for the accurate quantitation of low levels of human androgen receptor (hAR) mRNA in GSF. To control for variations due to sample preparation, and to minimize the disparity of the reverse transcriptase efficiency between samples after the RT procedure, we produced an initial PCR for the glyceraldehyde-3-phosphate dehydrogenase (GAPDH) gene, and then adjusted the amount of cDNA to that of this housekeeping gene. Competitive PCR for hAR was then immediately performed on normalized cDNA with a competitor DNA that exhibited a 13 bp deletion as compared to the 163 bp for the target fragment, and the PCR products were easily separated by 3.5% agarose gel electrophoresis. This quantitation procedure involved no additional steps, such as enzymatic cleavage of the PCR products, nor the use of radioactivity. In GSF from individuals, we found that the normal amount of AR mRNA was 5.6 attomoles/microg RNA, (+/-1.0, s.e.m.) with an intra- and an inter-assay of 8.4 and 14.7%, respectively. We observed a biphasic pattern of AR mRNA expression in normal human GSF in the presence of physiological concentration of androgen. Quantitative RT-PCR of AR mRNA may be useful for studying AR mRNA expression in experimental or clinical conditions.

From January 1986 to February 1994, 198 children were operated on for hypertrophic pyloric stenosis (HPS). Postoperative follow-up have been carried out in 194 cases. The children were divided into two groups: group A (n = 134; 69.1%): without any postoperative diet troubles (n = 52) or simple regurgitations (n = 82), and group B (n = 60; 30.9%) presenting more significant vomiting requiring medical treatment (n = 52) or a prolongation of parental nutrition (n = 8). A retrospective study of the different factors which can possibly explain this postoperative vomiting was carried out. The criteria having an influence are: the age (44.5 days in group A; 35.7 days in group B; (p < 0.001) the weight at the time of the operation (3921) g in group A; 3647 in group B; p = 0.01) the thickness of the pylorus at the pre-operative ultrasound scan (5.2 mm in group A; 47 in group B; p < 0.015). The other studied criteria (prematurity, birth weight, delay in diagnosis, weight loss, hydroelectrolytic abnormalities, surgical approach way-subcostal or umbilical-, surgical difficulties and operation duration) are not statistically significant. The young age (and therefore the low weight) at the time of the pyloromyotomy can easily explain the post-operative vomiting through the physiological immaturity of the lower sphincter of the esophagus. It is more paradoxical to note that these difficulties are all the more frequent because the pyloric tumor is less thick at the ultrasound scan. But this criterion is also directly related to the child's age (average thickness of 4.5 mm before the age of one month and 5.8 mm after the age of two months; p < 0.0001). These data suggest the importance of systematic medical treatment to prevent postoperative vomiting in high-risk children, in order to decrease hospital stay (4.14 days in group A; 5.20 days in group B; p < 0.0001).

Objective: We explored the possibility of a genetic anomaly in the sex determining region of the Y chromosome, SRY gene, known to be equated to the testis determining region., Patients: Four patients with bilateral congenital anorchia, absence of testicular tissue, elevated FSH and a lack of testosterone response to human chorionic gonadotrophin stimulation tests were studied., Measurements: Amplification by polymerase chain reaction of the SRY gene and direct double stranded DNA sequencing were performed with the same primers., Results: The expected 648 basepairs band of SRY was detected in the four DNA samples from patients with bilateral congenital anorchia. Direct sequencing did not show any difference with the previous published sequence., Conclusions: These data suggest that, in the four patients, bilateral congenital anorchia is not related to an anomaly of the opening reading frame sequence of the SRY gene.

In a multicentre trial we compared the clinical efficacy of amoxicillin/clavulanate used as a single-agent therapy with that of the three-agent combination usually prescribed in the post-operative period for appendicular peritonitis in children. Only bacteriologically documented peritoneal infections were included. Sixty-four patients were randomly distributed between two groups: Group A (29 cases) treated with amoxicillin/clavulanate, first administered iv (100 mg/kg/d), followed by conversion to the oral route (50 mg/kg/d) once the patient had been afebrile for 48 hours; Group B (35 cases) first treated by the iv route with benzylpenicillin (100,000 IU/kg/d) plus netilmicin (5 mg/kg/d) plus metronidazole (30 mg/kg/d), followed by conversion to the oral route for metronidazole (30 mg/kg/d). In both groups, the total duration of parenteral and oral treatment was not less than 5 days. One hundred and seventy nine bacterial strains were recovered from peritoneal fluid samples obtained during surgery; 86% of these were sensitive to amoxicillin/clavulanate. Clinical efficacy, assessed on the basis of time until return to normal temperature and gut transit and duration of hospitalization, was identical in both groups, with follow-up monitoring on day 30 showing recovery in all cases. Cure was obtained without any problems of infection in 25/29 patients in group A and in 34/35 patients in group B (non significant difference). Tolerance was excellent and identical in the two groups with the exception of three cases of thrombophlebitis which occurred in group B. The results of this study suggest that amoxicillin/clavulanate may be useful as single-agent therapy as a first-line curative treatment for appendicular peritonitis in children.

The authors report the case of a tetralogy of Fallot associated with a neuroblastoma secreting large quantities of noradrenaline. Anoxic decompensation of the tetralogy of Fallot occurred early at the age of 4 months with cyanotic crises associated with an impression of abdominal pain. A systemic-pulmonary anastomosis was performed and the cyanosis regressed but the crises persisted and investigations showed the presence of a neuroblastoma. The tumour was treated by surgery and chemotherapy and has not recurred after 2 years' follow-up. Complete repair of the tetralogy of Fallot was performed secondarily at the age of 2. In this case, the high concentration of circulating catecholamines was probably a factor of early decompensation of the tetralogy of Fallot by infundibular spasm. A review of the literature revealed 26 cases of cardiac disease with a peripheral neurogenic tumour. Two pathogenic mechanisms are suggested: an embryological abnormality of cardiac cell migration from the neural crest and chronic stimulation of neuroblastic cells by chronic hypoxia.

During the past ten years, we have treated 26 children with closed renal injuries. Hematuria appeared in 19 cases, but was not detected in 7 other cases, 6 of which corresponded to severe, especially pedicular, lesions. The diagnostic means for the lesion included IVP in 24 cases, ultrasonography in 23, angiography in 8 and computed tomography in 7. The treatment was medical for 18 children and surgical for 8. Observation revealed 4 cases of renal atrophy, including 1 of general atrophy without residual arterial hypertension. On the basis of this series and of a review of the literature, we study the evolution of the diagnosis and treatment of this condition with the development of new medical imaging techniques, especially of CT.

During the past ten years, we have treated 26 children with closed renal injuries. Hematuria appeared in 19 cases, but was not detected in 7 other cases, 6 of which corresponded to severe, especially pedicular, lesions. The diagnostic means for the lesion included IVP in 24 cases, ultrasonography in 23, angiography in 8 and computed tomography in 7. The treatment was medical for 18 children and surgical for 8. Observation revealed 4 cases of renal atrophy, including 1 of general atrophy without residual arterial hypertension. On the basis of this series and of a review of the literature, we study the evolution of the diagnosis and treatment of this condition with the development of new medical imaging techniques, especially of CT.

The authors report the use of high frequency ultrasound (7.5-10 MHz probes) for the detection of intestinal complications in 14 children with Henoch-Schönlein purpura. Ultrasound gave information of 3 levels: (1) It explained the acute abdominal pain by showing in all cases the oedematous haemorrhagic infiltration of the intestinal wall, which appeared thickened (3-11 mm): lesions were diffuse (6 cases) or focal (duodenal in 5 cases, jejunal in 2 cases and ileal in one). (2) It followed the evolution of the disease: extension of lesions (5 cases) or resolution (progressive decrease of parietal thickening, reexpansion of small bowel lumen, reappearance of peristalsis). (3) It always detected surgical complication: ileo-ileal intussusception (3 cases), perforation (1 case).

This review deals with the various anatomo-clinical features and the embryogenesis of utero-vaginal malformations. Three anatomo-clinical groups are distinguished from the vaginal malformations: vaginal aplasia, vaginal septa and hydrometrocolpos. Similarly, there are 3 types of uterine malformations, each corresponding to a chronologically different embryopathological anomaly: mullerian aplasia, most often responsible for utero-vaginal aplasia, mullerian fusion defects leading to bicornuate uterus, and resorption defects of the mullerian septum resulting in uterine septum. Only the utero-vaginal malformations with a vaginal component, mainly neonatal hydrometrocolpos, can really be detected during infancy and childhood, provided that the examination of the external genitalia is systematically and carefully performed. Due to their usual clinical latency during childhood, most malformations are only discovered by the end of puberty or after menarche, the main clinical signs being primary amenorrhea, cryptomenorrhea, unilateral dysmenorrhea or isolated dyspareunia. After the clinical gynecological examination, ultrasound diagnosis is very useful to explore the internal genitalia and to search for a frequently associated anomaly of the urinary tract.

Three techniques have been developed for single-stage repair of posterior hypospadias: free skin grafts, free bladder mucosal grafts, and pedicle preputial grafts. This multicenter retrospective study of 178 children who underwent surgery for posterior hypospadias was designed to compare the results achieved with these procedures. Free skin grafts (15 cases) resulted in the most frequent complications, and in particular the most severe strictures; in our opinion this technique should be abandoned. Pedicle preputial grafts (133 cases) gave the greatest number of successes from the outset, and should be preferred whenever the dimensions of the prepuce are sufficient for urethroplasty. Results with bladder mucosal grafts (30 cases) were not as good as with preputial grafts, but this technique remains the only solution when the meatus is in a very posterior position, and for children who have already undergone multiple operations.

Neonatal obstruction is a radio-clinical syndrome, common to a wide range of malformative anomalies which alter the normal anatomy or function of the digestive tract at any level. The main etiologies are reviewed with emphasis on specificity due to embryology, topography and clinical presentation. It is relatively easy to diagnose neonatal obstruction and relate it to a definite cause. The successful treatment of such obstruction requires a pluridisciplinary collaboration of specialized teams.