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Objectives: To report the clinical spectrum of genital defects diagnosed before birth, identify predictive factors for severe phenotypes at birth, and determine the rate of associated malformations., Patients and Methods: A retrospective study (2008-2017) of 4580 fetuses, identified prenatally with abnormalities evaluated by our Reference Center for Fetal Medicine, included cases with fetal sonographic findings of abnormal genitalia or uncertainty of fetal sex determination. Familial, prenatal and postnatal data were collected via a standardised questionnaire., Results: In all, 61 fetuses were included. The positive predictive value (PPV) of the prenatal diagnosis of genital defects was 90.1%. Most cases were 46,XY-undervirilized boys, 42 cases (68.8%), which included 29 with mid-penile or posterior hypospadias, nine with anterior hypospadias, and epispadias, micropenis, scrotal transposition, and buried penis (one each). In all, 46,XX-virilized girls were identified in seven cases (11.5%), which included four with congenital adrenal hyperplasia, two with isolated clitoromegaly, and one with ovotestis. Other defects included prune belly syndrome and persistent cloaca (six cases). Early detection during the second trimester (58.1% vs 18.8%, P = 0.03), intra-uterine growth restriction (IUGR) (45.2% vs 9.1%, P = 0.06), and curvature of the penis (38.7% vs 0%, P = 0.02), were more frequently related to severe defects in male newborns. Associated malformations (14 cases, 22.9%) and genetic defects (six) were frequent in undervirilized boys., Conclusion: Prenatal imaging of genital defects leads to a wide range of phenotypes at birth. Its PPV is high and extra-urinary malformations are frequent. Early diagnosis during the second trimester, associated IUGR, and curvature of the genital tubercle, should raise suspicion of a severe phenotype and may justify delivery near a multidisciplinary disorders/differences of sex development team., (© 2019 The Authors BJU International © 2019 BJU International Published by John Wiley & Sons Ltd.)

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.)

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.

Percutaneous suprapubic catheterization is an alternative when placement of a urethral catheter is contraindicated or unsuccessful. We report the case of a 3-day-old newborn, who presented anuric acute renal failure secondary to placement of a suprapubic catheter. An excessive length of catheter had been inserted into the bladder and was obstructing the vesicoureteral junctions. Several measures can be taken to prevent this complication, such as systematic measurement of the length inserted into the bladder, use of a catheter with 1cm gradations or recourse to radiopaque material., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: Thoracoscopic treatment of esophageal atresia type 3 has been previously reported to be feasible but no study clearly showed the benefits of thoracoscopy compared to open procedure. The aim of this study was to compare the outcome of esophageal atresia type III treated by thoracoscopic or open procedure., Material and Method: From january 2000 to december 2006, 31 children were operated, 17 by thoracotomy (weight range from 1750 to 4020 g) and 14 by thoracoscopy (weight range from 2110 to 4160 g). Neonatal deaths from an independent condition (3 cases in thoracotomy group) were excluded from the study and we analyzed 14 children in each group. Analyzed data included length of surgery, length of post operative assisted ventilation, length of pleural drainage, length of stay in ICU, delay before oral feeding, length of morphine analgesia, length of hospitalization and rate of complication., Results: Length of morphine analgesia was higher in thoracotomy group than in thoracoscopic group (mean 6.6 days versus 5.3 days, p = 0.16). Length of hospitalization was also higher in thoracotomy group (mean 22.6 days versus 19.1 days, p = 0.3). The rate of complication with thoracoscopy was not higher need of oesophageal dilatation (21% in thoracoscopic group versus 14% in thoracotomy group), need of Nissen fundoplication (21% in thoracoscopic group versus 28% in thoracotomy group)., Conclusion: Thoracoscopic treatment of esophageal atresia type III reduces the need of morphine analgesia and the length of stay without increasing the risk of postoperative complications.

The authors present an early laparoscopic treatment in a newborn with biliary atresia. They describe the technical details of the Kasai laparoscopic procedure. A 10-day-old girl, weight 2.4 kg, was admitted with a history of jaundice and fecal acholia since birth, with elevated total bilirubin and abnormal hepatic test. Abdominal ultrasound showed a small gallbladder with hyperechogenicity in porta hepatis and absence of biliary principal duct. Other metabolic and hematological tests were normal. The procedure was performed at 20-day-old by laparoscopy. The cholangiography confirmed the biliary atresia and Kasai's procedure was continued by laparoscopy and transumbilical extracorporeal Roux-Y approach. The duration of the procedure was 220 min, with good tolerance of pneumoperitoneum due to the laparoscopy. Feedings of breast milk began on the third day postoperative, presenting normal colored stools, with normalization of the hepatic test. A 20 months follow-up was without complications.

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: 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.

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: 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.

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.

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.)

The aim of this study was to assess the role of nitric oxide (NO) and endothelin (ET)-1 in the pathophysiology of persistent pulmonary hypertension of the newborn in fetal lambs with a surgically created congenital diaphragmatic hernia (CDH). The pulmonary vascular response to various agonists and antagonists was assessed in vivo between 128 and 132 days gestation. Age-matched fetal lambs served as control animals. Control and CDH lambs had similar pulmonary vasodilator responses to acetylcholine, sodium nitroprusside, zaprinast, and dipyridamole. The ET(A)-receptor antagonist BQ-123 caused a significantly greater pulmonary vasodilatation in CDH than in control animals. The ET(B)-receptor agonist sarafotoxin 6c induced a biphasic response, with a sustained pulmonary vasoconstriction after a transient pulmonary vasodilatation that was not seen in CDH animals. We conclude that the NO signaling pathway in vivo is intact in experimental CDH. In contrast, ET(A)-receptor blockade and ET(B)-receptor stimulation significantly differed in CDH animals compared with control animals. Imbalance of ET-1-receptor activation favoring pulmonary vasoconstriction rather than altered NO-mediated pulmonary vasodilatation is likely to account for persistent pulmonary hypertension of the newborn in fetal lambs with a surgically created CDH.

This study evaluated long-term clinical and radiologic results of Dunn's osteotomy carried out in 50 consecutive cases for severe slipped capital femoral epiphysis. The results of all the patients were analyzed with 4 years 6 months of follow-up. The clinical results were very good and good in 90% of the patients and were fair or poor in 10% of the patients. Seven patients had an important complication (total necrosis or chondrolysis), and the clinical evolution was studied. Dunn's procedure seems to be adequate to correct severe slips.

Described for the first time in 1848 by Bochdalek, congenital diaphragmatic hernia is still a hot topic. How can it be that a simple defect of the diaphragm still has a mortality rate reaching 50% in 1997, and this despite continuous progress in neonatal intensive care? If some problems remain unsolved, experimental studies over the past 30 years have raised some questions concerning the pathogenesis, and have shed some light into the pathophysiology of congenital diaphragmatic hernia. This article reviews the recent knowledge about the aetiology, pathogenesis and pathophysiology of this complex malformation.