Dual-energy CT hybridation and kernel processing effects on the estimation of bone mineral mass and density: a calcination study on ex vivo human femur.

INTRODUCTION: Recent technological advances with dual-energy quantitative computed tomography (DEQCT) allow to combine two images of different level of energy to obtain simulated mono-energetic images at 60 keV (SIM60KeV-QCT) with improved image contrast in clinical practice. This study includes three topics: (1) compare bone mineral content (BMC), areal and volumetric bone mineral density (aBMD, vBMD) obtained with SIM60KeV-QCT, single-energy QCT (SEQCT), and dual X-ray absorptiometry (DXA); (2) compare ash density and weight with respective vBMD and BMC assessed on SIM60KeV-QCT, SEQCT, and DXA; and (3) compare the influence of reconstruction kernels on the accuracy of vBMD and BMC using ash density and ash weight as the reference values. METHODS: DXA, SEQCT, and DEQCT acquisitions were performed ex vivo on 42 human femurs. Standard kernel (SK) and bone kernel (BK) were applied to each stack of images. Ten diaphyses and 10 femoral necks were cut, scanned, and reconstructed using the techniques described above. Finally, the bone specimens were calcined to obtain the ash weight. RESULTS: QCT analysis (SEQCT, SIM60KeV-QCT) underestimated BMC value compared to DXA. For femoral necks, all QCT analyses provided an unbiased estimate of ash weight but underestimated ash density regardless of the kernel used. For femoral diaphysis, SEQCT BK, SIM60KeV-QCT BK, and SK underestimated ash weight but not ash density. CONCLUSION: BMC and vBMD quantifications with the SIM60KeV-QCT gave similar results as the SEQCT. Further studies are needed to optimize the use of SIM60KeV-QCT in clinical situations. SK should be used given the effect of kernels on QCT assessment.

Risk Factors for the Incident Decline of Physical Performance in Older Men: The Prospective Strambo Study.

Risk factors of physical performance decline in older men remain uncertain. We assessed risk factors of incident physical performance deterioration in older men followed up prospectively. In a cohort of 821 men aged 60-87, physical performance was assessed by four tests (five chair stands, standing with closed eyes, forward and backward tandem walk) at baseline, 4 and 8 years. Various predictive biological measurements were performed at baseline. Serum creatinine/ cystatin C (Cr/CysC) ratio was used as an index of muscle mass. In multivariate models, higher age, higher fat mass index (FMI = fat mass/height2), low physical activity, prior stroke and fracture were associated with poor physical performance at baseline. Higher age, low physical activity, low calcium intake, prior non-vertebral fractures, low apparent free testosterone concentration and poor health status were associated with higher risk of loss to follow-up. Low grip strength, Parkinson's disease and stroke were associated with higher risk of incident inability to do five chair stands. Low Cr/CysC ratio and high FMI were associated with high risk of incident inability to perform forward and backward tandem walk. Sarcopenic obesity (co-occurrence of lower tertile of Cr/CysC and upper tertile of FMI) was associated with higher risk of incident inability to perform forward (OR = 3.31, 95% CI 1.88-5.84, p < 0.001) and backward tandem walk and of incident inability to perform more than one test (OR = 5.82, 95% CI 1.29-26.27, p < 0.001). In conclusion, sarcopenic obesity and poor health are associated with higher risk of incident severe decline of physical performance.

Bone Microarchitecture Decline and Risk of Fall and Fracture in Men With Poor Physical Performance-The STRAMBO Study.

CONTEXT: High fracture risk in individuals with low muscle strength is attributed to high risk of falls. OBJECTIVE: This work aims to study the association of muscle mass and physical performance with bone microarchitecture decline and risk of fall and nonvertebral fracture in men. METHODS: A prospective, 8-year follow-up of a cohort was conducted among the general population. A total of 821 volunteer men aged 60 and older participated. Hip areal bone mineral density (aBMD) and appendicular lean mass (ALM) were assessed at baseline by dual x-ray absorptiometry. Lower-limb relative ALM (RALM-LL) is ALM-LL/(leg length)2. The physical performance score reflects the ability to perform chair stands and static and dynamic balance. Bone microarchitecture was assessed by high-resolution peripheral quantitative computed tomography (HR-pQCT) at baseline and after 4 and 8 years. Statistical analyses were adjusted for shared risk factors. Outcome measurements included the rate of change in the HR-pQCT indices, incident falls, and fractures. RESULTS: Cortical bone loss and estimated bone strength decline were faster in men with low vs normal RALM-LL (failure load: -0.74 ±â€…0.09 vs -0.43 ±â€…0.10%/year; P < .005). Differences were similar between men with poor and those with normal physical performance (failure load: -1.12 ±â€…0.09 vs -0.40 ±â€…0.05%/year; P < .001). Differences were similar between men having poor performance and low RALM-LL and men having normal RALM-LL and performance (failure load: -1.40 ±â€…0.17 vs -0.47 ±â€…0.03%/year; P < .001). Men with poor physical performance had a higher risk of fall (hazard ratio [HR] = 3.52; 95% CI, 1.57-7.90, P < .05) and fracture (HR = 2.68; 95% CI, 1.08-6.66, P < .05). CONCLUSION: Rapid decline of bone microarchitecture and estimated strength in men with poor physical performance and low RALM-LL may contribute to higher fracture risk.

Is Laparoscopic Pyeloplasty for Ureteropelvic Junction Obstruction in Infants Under 1 Year of Age a Good Option?

Purpose: Laparoscopic pyeloplasty in children younger than 1 year of age is still debatable due to its supposed technical difficulties and failure rate. We present our experience and outcome in infants. Materials and Methods: A retrospective study was conducted in 3 Departments of Pediatric Surgery. We reviewed the records of the children under 1 year of age operated on for ureteropelvic junction obstruction (UPJO), between 2007 and 2017. Anderson-Hynes laparoscopic transabdominal dismembered pyeloplasty was performed. Patients' demographics, results of preoperative and postoperative exams, perioperative details, complications, hospital stay, and long-term follow-up results were analyzed. Results: Sixty cases were operated on during this period (49 boys, 11 girls). Mean age at operation was 4.5 months (1-12 months). Mean operating time was 140 min (80-240 min). There was no conversion in this group. There were four early complications: 1 ileus, 1 hypertension immediately post-operatively requiring medical treatment, 1 omental herniation through a drain orifice, and 1 percutaneous transanastomotic stent migrated intra-abdominally. The two last children had to be reoperated. Mean hospital stay was of 2 days (1-10 days). Late complications: two patients (3.4%) presented a recurrence of UPJO, one had been re-operated 15 months later and for the patient with persistent hypertension, nephropexy was performed for malrotated kidney, 1 year after pyeloplasty. Long term follow-up with a mean of 2.8 years (1-10 years) showed that surgery improved mean pelvic dilatation from 31.8 mm (13-63 mm) preoperatively to 15.3 mm (4-40 mm) postoperatively (P < 0.0001). The renal function slightly improved, from a mean of 35.7% (5-55%) it passed to 40.5% (0-54%), p = 0.137. In three cases the operated kidney became finally non-functional and atrophic. Conclusions: Laparoscopic transperitoneal pyeloplasty is feasible and safe in children younger than 1 year of age. Nevertheless, it requires experience and good intra-abdominal suturing skills. Laparoscopic pyeloplasty has a success rate comparable with open treatment but with less morbidity and better cosmetic results.

BCL-XL directly modulates RAS signalling to favour cancer cell stemness.

In tumours, accumulation of chemoresistant cells that express high levels of anti-apoptotic proteins such as BCL-XL is thought to result from the counter selection of sensitive, low expresser clones during progression and/or initial treatment. We herein show that BCL-XL expression is selectively advantageous to cancer cell populations even in the absence of pro-apoptotic pressure. In transformed human mammary epithelial cells BCL-XL favours full activation of signalling downstream of constitutively active RAS with which it interacts in a BH4-dependent manner. Comparative proteomic analysis and functional assays indicate that this is critical for RAS-induced expression of stemness regulators and maintenance of a cancer initiating cell (CIC) phenotype. Resistant cancer cells thus arise from a positive selection driven by BCL-XL modulation of RAS-induced self-renewal, and during which apoptotic resistance is not necessarily the directly selected trait.

Malpractice in paediatric minimally invasive surgery - a current concept: Results of an international survey.

This study aimed to assess malpractice in paediatric minimally invasive surgery (MIS), and attitudes, prevention strategies and mechanisms to support surgeons while they are under investigation. An observational, multicentric, questionnaire-based study was conducted. The survey questionnaire was sent via mail, and it comprised four sections. Twenty-four paediatric surgeons (average age 54.6 years), from 13 different countries, participated in this study. The majority had >15 years of experience in MIS. Three (12.5%) surgeons reported a total of five malpractice claims regarding their MIS activity. The reasons for the claims were a postoperative complication in 3/5 (60%) cases, a delayed/failed diagnosis in 1/5 (20%) cases and the death of the patient in 1/5 (20%) cases. The claims concluded with the absolution of the surgeon in all cases, and monetary compensation to the claimant in two (40%) cases. Eleven (45.8%) surgeons were invited as expert counsels in medico-legal actions. Medico-legal aspects have a minimal impact on the MIS activity of paediatric surgeons. In this series, claims concluded with the absolution of the surgeon in all cases, but they had a negative effect on the surgeon's reputation and finances. A key element in supporting surgeons while they are under investigation is always to choose a surgeon who is an expert in paediatric MIS as legal counsel. A constant update on innovations in paediatric MIS and appropriate professional liability insurance may also play a key role in reducing medico-legal consequences.

Intestinal Malrotation and Volvulus in Neonates: Laparoscopy Versus Open Laparotomy.

BACKGROUND: Intestinal malrotations with midgut volvulus are surgical emergencies that can lead to life-threatening intestinal necrosis. This study evaluates the feasibility and the outcomes of laparoscopic treatment of midgut volvulus compared with classic open Ladd's procedure in neonates. MATERIALS AND METHODS: The medical records of all neonates with diagnosis of malrotation and volvulus, who underwent surgery between January 1993 and January 2014, were reviewed. We considered the group of neonates laparoscopically treated (Group A, n = 20) and we compared it with an equal number of neonates treated with the classical open Ladd's procedure (Group B, n = 20). RESULTS: The median age at surgery was 8.4 days and the mean weight was 3.340 kg. The suspicion of volvulus was documented by plain abdominal radiograph, upper gastrointestinal contrast study, and/or ultrasound scanning of the mesenteric vessels. All the patients were treated according to the Ladd's procedure. Conversion to an open procedure was necessary in 25% of the patients. The mean operative time was 80 minutes (28-190 minutes) in Group A and 61 minutes (40-130 minutes) in Group B (P = .04). The median time to full diet (P = .02) and hospital stay (P = .04) was better in Group A. Rehospitalization because of recurrence of occlusive symptoms occurred in 30% of patients in Group A (n = 6) and in 40% of patients in Group B (n = 8). Among these, all the 6 patients of Group A underwent redo surgery for additional division of Ladd's bands or debridement; instead in Group B, 4 of 8 patients underwent open redo surgery. CONCLUSIONS: Laparoscopic exploration is the procedure of choice in case of suspicion of intestinal malrotation and volvulus. Laparoscopic treatment is feasible and safe even in neonatal age without additional risks compared with classical open Ladd's procedure.

Technical standardization of laparoscopic repair of Morgagni diaphragmatic hernia in children: results of a multicentric survey on 43 patients.

BACKGROUND: This study aimed to standardize the surgical correction technique of congenital Morgagni diaphragmatic hernia (CMDH), analyzing the results of an international multicentric survey. METHODS: The medical records of 43 patients (29 boys, 14 girls) who underwent laparoscopic repair of CMDH in 8 pediatric surgery units in a 5-year period were retrospectively reviewed. Their average age was 3.3 years. Ten patients (23.2%) presented associated malformations: 9 Down syndrome (20.9%) and 1 palate cleft (2.3%). Thirty-five patients (81.4%) were asymptomatic, whereas 8 patients (18.6%) presented symptoms such as respiratory distress, cough or abdominal pain. As for preoperative work-up, all patients received a chest X-ray (100%), 15/43 (34.8%) a CT scan, 8/43 (18.6%) a barium enema and 4/43 (9.3%) a US. RESULTS: No conversion to open surgery was reported. Average operative time was 61.2 min (range 45-110 min). In 38/43 (88.3%) patients, a trans-parietal stitch was positioned in order to reduce the tension during the repair. In 14/43 cases (32.5%), the sac was resected; in only 1/43 case (2.3%) a dual mesh of goretex was adopted to reinforce the closure. Average hospital stay was 2.8 days. The average follow-up was 4.2 years, and it consisted in annual clinical controls and chest X-ray. We recorded 2 complications (4.6%): one small pleural opening that required no drain and one recurrence (2.3%), re-operated in laparoscopy, with no further recurrence. CONCLUSIONS: To the best of our knowledge, this is the largest series published in the literature on this topic. Laparoscopic CMDH repair is well standardized: The full-thickness anterior abdominal wall repair using non-resorbable suture with interrupted stitches is the technique of choice. Postoperative outcome was excellent. Recurrence rate was very low, about 2% in our series. We believe that children with CMDH should be always treated in laparoscopy following the technical details reported in this paper.

Prevalence of Barrett Esophagus in Adolescents and Young Adults With Esophageal Atresia.

OBJECTIVE: To study the prevalence of Barrett esophagus (BE) (gastric and/or intestinal metaplasia) in adolescents treated for esophageal atresia (EA). SUMMARY OF BACKGROUND DATA: EA patients are at high risk of BE. METHODS: This multicenter prospective study included EA patients aged 15 to 19 years. All eligible patients were proposed an upper endoscopy with multistaged esophageal biopsies under general anesthesia. Histological suspicion of metaplasia was confirmed centrally. RESULTS: One hundred twenty patients [mean age, 16.5 years (±1.4)] were included; 70% had been treated for gastroesophageal reflux disease (GERD) during infancy. At evaluation, 8% were undernourished, 41% had received antireflux surgery, and 41% presented with GERD symptoms, although only 28% were receiving medical treatment. Esophagitis was found at endoscopy in 34% and confirmed at histology in 67%. BE was suspected after endoscopy in 37% and was confirmed by histology for 43% of patients (50 gastric and 1 intestinal metaplasia). No endoscopic or histological anomalies were found at the anastomosis site. BE was not significantly related to clinical symptoms. In multivariate analysis, BE was associated with EA without fistula (P = 0.03), previous multiple antireflux surgery (P = 0.04), esophageal dilation (P = 0.04), suspicion of BE at endoscopy (P < 0.001), and histological esophagitis (P = 0.02). CONCLUSIONS: Patients with EA are at high risk of persistent GERD and BE. The development of BE is related to GERD history. Long-term systematic follow-up of the esophageal mucosa including multistaged biopsies is required, even in asymptomatic patients. (NCT02495051).

Twenty years' experience for reduction of ileocolic intussusceptions by saline enema under sonography control.

BACKGROUND: Ultrasonography is a well-established efficient diagnostic tool for ileocolic intussusceptions in children. It can also be used to control hydrostatic reduction by saline enemas. This reduction method presents the advantage of avoiding radiations. Parents can even stay with their children during the procedure, which is comforting for both. The purpose of this study was to present our 20 years' experience in intussusception reductions using saline enema under ultrasound control and to assess its efficiency and safety. MATERIAL AND METHODS: This retrospective single center study included patients with ileocolic intussusceptions diagnosed by ultrasound between June 1993 and July 2013. We excluded the data of patients with spontaneous reduction or who underwent primary surgery because of contraindications to hydrostatic reduction (peritonitis, medium or huge abdominal effusion, ischemia on Doppler, bowel perforation). A saline enema was infused into the colon until the reduction was sonographically confirmed. The procedure was repeated if not efficient. Light sedation was practiced in some children. RESULTS: Eighty-tree percent of the reductions were successful with a median of 1 attempt. Reduction success decreased with the number of attempts but was still by 16% after 4 attempts. The early recurrence rates were 14.5%, and 61.2% of those had a successful second complete reduction. Forty-six patients needed surgery (11 of them had a secondary intussusception). Sedation multiplies success by 10. In this period, only one complication is described. CONCLUSION: Ultrasound guided intussusception reduction by saline enema is an efficient and safe procedure. It prevents exposure of a young child to a significant amount of radiation, with similar success rate. We had very low complication rate (1/270 cases or 3‰). The success rate could be increased by standardized procedures including: systematic sedation, trained radiologists, accurate pressure measurement, and number and duration of attempts.

Laparoscopic modified Orr-Loygue mesh rectopexy for rectal prolapse in children.

AIM: We present an operating technique inspired from the Orr-Loygue mesh rectopexy adapted for laparoscopy, and detail the technical steps that differ from laparoscopic posterior suture rectopexy more commonly described in the paediatric literature. METHOD: We present a retrospective study of all children who underwent a modified Orr-Loygue procedure for recurrent complete rectal prolapse from 1999 to 2012 after failure of conservative treatment. Pathological conditions, technical details of the procedure (excision of the Douglas pouch, use of a prerectal non-absorbable mesh to suspend the rectum to the presacral fascia and promontory avoiding any tension on the rectal wall) and postoperative results were reviewed. RESULTS: Eight patients were included, median age 6.5 years (range, 2-17). Median symptoms duration before surgery was 14 months (range, 6-24). Four patients presented with associated pathological conditions: 1 neurological impairment (Williams-Beuren syndrome), 1 severe malnutrition (mental anorexia), 1 solitary rectal ulcer with frequent bleeding, 1 syringomyelic cavity in the spinal cord. All procedures were completed laparoscopically with a median operative time of 98 minutes (range, 80-125). Median hospital stay was 3.5 days (range, 2-5). No postoperative constipation or recurrence was reported during the median follow-up period of 6 years (range 2-13). CONCLUSION: The laparoscopic modified Orr-Loygue mesh rectopexy is a simple operating technique, reproducible and efficient as surgical treatment of nonresolving recurrent complete rectal prolapse in children. To avoid postoperative constipation, it is important to perform a tension-free rectopexy which can be achieved by the use of a mesh to simply suspend and not "fix" the redundant rectosigmoid. Nonetheless, a greater number of patients as well as colorectal electromyography or anorectal manometry would be necessary to prove the absence of postoperative deleterious functional disorder.

Work-related upper limb musculoskeletal disorders in paediatric laparoscopic surgery. A multicenter survey.

BACKGROUND: Surgeons are at risk for developing work-related musculoskeletal symptoms (WMS). The present study aims to examine the physical factors and their association with WMS among pediatric laparoscopic surgeons. METHODS: A questionnaire consisting of 21 questions was created and mailed to 25 pediatric laparoscopic surgeons (LG). 23/25 surgeons (92%) completed the survey. The questionnaire was analyzed and then split into 2 groups. Group 1 (LG1) included surgeons with greater laparoscopic experience, and group 2 (LG2) included surgeons with less important laparoscopic experience. In addition, we constructed and sent to the same surgeons a similar questionnaire focused on WMS after an open procedure (OG) with the aim to compare results of LG with OG. RESULTS: The prevalence rate of WMS with shoulder symptoms was 78.2% in surgeons that performed laparoscopy for more than 10 years, with 60.8% also reporting other pain. In 66.6% this pain is evident only after a long-lasting procedure. Forty-four percent of these surgeons require painkillers at least twice a week. Fifty percent of these surgeons also suffer at home. Fifty-five and one half percent of surgeons indicate that this pain is related to their laparoscopic activity. Forty-three and a half percent think that laparoscopy is beneficial only for the patient but has a bad ergonomic effect for surgeons. Sixty-five and two-tenths percent think that robotic surgery can be helpful to improve ergonomics. Comparing the groups, WMS occur more frequently in LG (78.2%) than in OG (56.5%), but this difference was not statistically significant (χ(2)=0.05). In addition, WMS occur more frequently in LG1 (84.6%) than in LG2 (70%), but this difference was not statistically significant (χ(2)=0.05). CONCLUSIONS: These results confirmed a strong association between WMS and the number of laparoscopic procedures performed. Skilled laparoscopic surgeons have more pain than less skilled laparoscopic surgeons. WMS in the same group of surgeons are more frequent after laparoscopy than after open procedures. The majority of surgeons refer to shoulder symptoms.

Congenital diaphragmatic hernia: an evaluation of risk factors for failure of thoracoscopic primary repair in neonates.

PURPOSE: Publications aiming to prove the feasibility and safety of thoracoscopic CDH-repair in neonates grow in numbers. Some teams use selection criteria, but none have proven statistical evidence. The aim of this study is to detect risk factors for failure of thoracoscopic primary closure of CDH in neonates. METHODS: In 8 centers performing minimal access surgery (MAS), complete prenatal, postnatal, and operative data were evaluated for a retrospective study concerning patients with thoracoscopic congenital diaphragmatic hernia (CDH) repair. Most of the selection criteria and risk factors mentioned in the literature were analyzed. Two groups were defined: Group A - neonates who tolerated thoracoscopic primary repair, and Group B - neonates who required conversion or presented with major complications after thoracoscopic repair. Univariate and multivariate logistic regressions were used to compare these two groups. RESULTS: From 2006 to 2010, thoracoscopy was performed in 40 neonates: Group A consisting of 28 neonates, and Group B 9 patients. Three patients were excluded because of insufficient data or major associated malformations. Significant statistical differences were found in Group B for postnatal PaCO2 >60 mmHg, need of iNO during postnatal stabilization, intrathoracic position of the stomach, pulmonary hypertension signs on the postnatal cardiac ultrasound, and preoperative OI >3.0. On multivariate analysis, only an OI >3.0 was significantly associated with conversion or major post-operative complication of thoracoscopic primary repair. CONCLUSION: CDH can be safely repaired in the neonatal period by thoracoscopy. The limiting factor for thoracoscopic CDH repair is PPHN. The best preoperative indicator for PPHN is OI. Prospective studies are nonetheless necessary to prove the effectiveness of using these risk factors as selection criteria to help design surgical management protocols for neonates presenting CDH.

Outcome of alimentary tract duplications operated on by minimally invasive surgery: a retrospective multicenter study by the GECI (Groupe d'Etude en Coeliochirurgie Infantile).

BACKGROUND: Alimentary tract duplications (ATD) are a rare cause of intestinal obstruction in childhood. There are many case reports but few series about laparoscopy or thoracoscopy for ATD. The aim of our study was to report the outcome of minimally invasive surgery (MIS) for ATD. METHODS: This was a retrospective multicenter study from the GECI (Groupe d'Etude en Coeliochirurgie Infantile). We reviewed the charts of 114 patients operated on by MIS for ATD from 1994 to 2009. RESULTS: Sixty-two patients (54 %) had a prenatal diagnosis. Forty-nine patients (43 %) were symptomatic before surgery: 33 of those patients (63 %) with postnatal diagnosis compared to 16 (25 %) with prenatal diagnosis (P < 0.01). In this last group, the median age at onset of symptoms was 16 days (range = 0-972). One hundred and two patients had laparoscopy (esophageal to rectal duplications) and 12 patients had thoracoscopy for esophageal duplications. The mean operative time was 90 min (range = 82-98). There were 32 (28 %) resection anastomoses, 55 (48 %) enucleations, and 27 (24 %) unroofings. The conversion rate was 32 %, and in a multivariate analysis, it was significantly higher, up to 41 % for patients weighing <10 kg (P < 0.01). Ten patients (8 %) had unintentional perioperative opening of the digestive tract during the dissection. Eight patients had nine postoperative complications, including six small bowel obstructions. The median length of hospital stay was 4 days (range = 1-21) without conversion and 6 days (range = 1-27) with conversion (P = 0.01). The median follow-up was 3 months (range = 1-120). Eighteen of the 27 patients who underwent partial surgery had an ultrasound examination during follow-up. Five (18 %) of them had macroscopic residue. CONCLUSION: This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.

Herpes simplex virus encephalitis in a patient with complete TLR3 deficiency: TLR3 is otherwise redundant in protective immunity.

Autosomal dominant TLR3 deficiency has been identified as a genetic etiology of childhood herpes simplex virus 1 (HSV-1) encephalitis (HSE). This defect is partial, as it results in impaired, but not abolished induction of IFN-ß and -λ in fibroblasts in response to TLR3 stimulation. The apparently normal resistance of these patients to other infections, viral illnesses in particular, may thus result from residual TLR3 responses. We report here an autosomal recessive form of complete TLR3 deficiency in a young man who developed HSE in childhood but remained normally resistant to other infections. This patient is compound heterozygous for two loss-of-function TLR3 alleles, resulting in an absence of response to TLR3 activation by polyinosinic-polycytidylic acid (poly(I:C)) and related agonists in his fibroblasts. Moreover, upon infection of the patient's fibroblasts with HSV-1, the impairment of IFN-ß and -λ production resulted in high levels of viral replication and cell death. In contrast, the patient's peripheral blood mononuclear cells responded normally to poly(I:C) and to all viruses tested, including HSV-1. Consistently, various TLR3-deficient leukocytes from the patient, including CD14(+) and/or CD16(+) monocytes, plasmacytoid dendritic cells, and in vitro derived monocyte-derived macrophages, responded normally to both poly(I:C) and HSV-1, with the induction of antiviral IFN production. These findings identify a new genetic etiology for childhood HSE, indicating that TLR3-mediated immunity is essential for protective immunity to HSV-1 in the central nervous system (CNS) during primary infection in childhood, in at least some patients. They also indicate that human TLR3 is largely redundant for responses to double-stranded RNA and HSV-1 in various leukocytes, probably accounting for the redundancy of TLR3 for host defense against viruses, including HSV-1, outside the CNS.

A true laparoscopic herniotomy in children: evaluation of long-term outcome.

Thirteen years of experience with a laparoscopic technique mimicking, as closely as possible, the classical open technique are presented in this report. Consecutive laparoscopic herniotomies in 385 children (178 girls and 207 boys; age 0.13 to 16.8 years) with a total of 525 hernia repairs were included into a retrospective review. Overall, 95% of children were treated on a day-care basis; all operations were completed laparoscopically. Mean operation time was 32.1 minutes (26.2 for unilateral hernias and 34.5 for bilateral). In total, 10.1% of children presented with clinically bilateral hernias, whereas 29.2% of clinical unilateral hernias had a patent contralateral processus vaginalis. We discovered femoral hernias in 2.6% and direct inguinal hernias in 0.5% of the cases. In 79 cases, a concomitant of umbilical herniorraphy was performed. There were no cases of testicular malposition or atrophy. We had a 1% hernia recurrence rate. Cosmetic outcome was excellent. Laparoscopy provides the surgeon with a superior diagnostic tool, facilitating the diagnosis of any kind of inguinal hernia or controlateral patent processus vaginalis and its repair in the same session; surgery- and anesthesia-related stress is minimized. This makes it an interesting alternative to the open repair of inguinal hernias in children, providing an excellent view on the cord structures and leaving them, as well as the testis, untouched.

A new minimally invasive technique for the repair of femoral hernia in children: about 13 laparoscopic repairs in 10 patients.

BACKGROUND: Femoral hernias in children are rare and often misdiagnosed. The classic treatment is through an open anterior approach. Since the advent of laparoscopic treatment of inguinal hernia in children, laparoscopy has been proposed to offer an accurate diagnosis and treatment, especially in case of recurrent hernia or bilateral disease. This review was undertaken to report our experience with the primary laparoscopic diagnosis and treatment of pediatric femoral hernias and to investigate its safety and feasibility. METHODS: All cases of pediatric femoral hernia in a consecutive series of children treated laparoscopically for groin hernias in a single institution over a 7-year period (2001-2007) were identified and studied for patient characteristics, presentation, pre- and perioperative findings, details of the operative repair, and postoperative outcome. RESULTS: Out of a prospectively studied series of 462 laparoscopic pediatric inguinal hernia repairs in 389 patients, 13 femoral hernias were treated in 10 patients (6 boys), with a mean age of 71/2 years (range, 1.7-12). The preoperative diagnosis of femoral hernia was accurate in 7 patients. Seven femoral hernias were exclusively right sided; 3 were bilateral. All 13 femoral hernias were successfully treated by a standardized transabdominal laparoscopic approach with the use of three 3.5-mm trocars. All patients were treated in a day care setting. No postoperative complications occurred. No recurrences were seen until the present time, with a mean follow-up of 31/2 years. CONCLUSIONS: Laparoscopy provides a straightforward, accurate diagnosis for the rare and often missed pediatric femoral hernias. The new technique described offers a safe and efficient minimally invasive anatomical repair of the crural orifice in children, even when not suspected preoperatively. The laparoscopic diagnosis of 13 femoral hernias from a cohort of 462 laparoscopic groin hernia repairs (2.8%) may suggest a higher prevalence rate of this unusual type of hernia in children than earlier described in literature.

Neonatal minimally invasive surgery for congenital diaphragmatic hernias: a multicenter study using thoracoscopy or laparoscopy.

BACKGROUND: Minimally invasive surgery (MIS) for late-presenting congenital diaphragmatic hernia (CDH) has been described previously, but few neonatal cases of CDH have been reported. This study aimed to report the multicenter experience of these rare cases and to compare the laparoscopic and thoracoscopic approaches. METHODS: Using MIS procedures, 30 patients (16 boys and 14 girls) from nine centers underwent surgery for CDH within the first month of life, 26 before day 5. Only one patient had associated malformations. There were 10 preterm patients (32-36 weeks of gestational age). Their weight at birth ranged from 1,800 to 3,800 g, with three patients weighing less than 2,600 g. Of the 30 patients, 18 were intubated at birth. RESULTS: The MIS procedures were performed in 18 cases by a thoracoscopic approach and in 12 cases by a laparoscopic approach. No severe complication was observed. For 20 patients, reduction of the intrathoracic contents was achieved easily with 15 thoracoscopies and 5 laparoscopies. In six cases, the reduction was difficult, proving to be impossible for the four remaining patients: one treated with thoracoscopy and three with laparoscopy. The reasons for the inability to reduce the thoracic contents were difficulty of liver mobilization (1 left CDH and 2 right CDH) and the presence of a dilated stomach in the thorax. Reductions were easier for cases of wide diaphragmatic defects using thoracoscopy. There were 10 conversions (5 laparoscopies and 5 thoracoscopies). The reported reasons for conversion were inability to reduce (n = 4), need for a patch (n = 5), lack of adequate vision (n = 4), narrow working space (n = 1), associated bowel malrotation (n = 1), and an anesthetic problem (n = 1). Five defects were too large for direct closure and had to be closed with a patch. Four required conversion, with one performed through video-assisted thoracic surgery. The recurrences were detected after two primer thoracoscopic closures, one of which was managed by successful reoperation using thoracoscopy. CONCLUSIONS: In the neonatal period, CDH can be safely closed using MIS procedures. The overall success rate in this study was 67%. The indication for MIS is not related to weeks of gestational age, to weight at birth (if >2,600 g), or to the extent of the immediate neonatal care. Patients with no associated anomaly who are hemodynamically stabilized can benefit from MIS procedures. Reduction of the herniated organs is easier using thoracoscopy. Right CDH, liver lobe herniation, and the need for a patch closure are the most frequent reasons for conversion.

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.