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

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

Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study.

OBJECTIVE: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. STUDY DESIGN: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. RESULTS: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01). CONCLUSIONS: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population.

Predictors of the Performance of Early Antireflux Surgery in Esophageal Atresia.

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia.

Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia?

OBJECTIVE: Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA. STUDY DESIGN: We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year. RESULTS: Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.

Portal vein thrombosis after laparoscopic splenectomy during childhood.

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

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

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

Outcome of long gap esophageal atresia at 6 years: A prospective case control cohort study.

BACKGROUND DATA: EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years. METHODS: A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed. RESULTS: Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group. CONCLUSIONS: Long gap strongly influenced digestive morbidity at age 6 years.

Can donor liver left lateral sector weight be predicted from anthropometric variables?

Most of the pediatric LT grafts consist in a LLS. Liver graft size matching is one of the major factors determining a successful outcome. The aim of our study was to assess whether anthropometric parameters can be used to estimate the LLS weight. A total of 122 donors (48F/74M) from two transplantation centers were retrospectively reviewed. Eighteen were living related donors (LRLT) and 104 DDs. The donor age was 28.2 yr (range 15-63). The BW and height were, respectively, 70.1 kg (range 45-111) and 172.7 cm (range 152-197). The WLW (n = 66) was 1462 g (range 921-2340), and the liver-to-BW ratio was 2% (range 1.45-2.8%). The LLS graft weight was 313 g (range: 183-537 g). The ratio between LLS and BW was 0.452% (range 0.27-0.74). The LLS represented 22.3% of the WLW with a large variability (range 15.4-31.3%). None of the developed models (linear, nonlinear, or multiple) was clinically usable. The LLS weight is highly variable and is not predictable using simple anthropometric variables. When available, we propose that ultrasonographic estimation of the liver volume should be performed when a liver splitting is considered.

Segmental ABO-incompatible liver graft from a donor after cardiac death in neonatal acute liver failure.

Segmental liver grafts from DCD in pediatric LT have been safely used even in acute liver failure situations. Furthermore, despite the risk of antibody-mediated acute rejection, some studies have also demonstrated the safety of ABO incompatible LT in infants. The use of such grafts can be beneficial by reducing the time on the transplant waiting list but they are more susceptible to initial dysfunction and there is a lack of enthusiasm to consider their use especially for an emergency LT as a life-saving procedure. In this short article, we describe the use and successful outcome in a neonate with fulminant acute liver failure secondary to neonatal hemochromatosis who received an ABO-incompatible reduced-size DCD graft.

Feeding disorders in children with oesophageal atresia: a cross-sectional study.

INTRODUCTION: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale. The secondary aim was to determine conditions associated with PFD. METHODS: The Feeding Disorders in Children with Oesophageal Atresia Study is a national cohort study based on the OA registry from the French National Network. Parents of children born with OA between 2013 and 2016 in one of the 22 participating centres were asked to complete the French version of the Montreal Children's Hospital Feeding Scale. RESULTS: Of the 248 eligible children, 145 children, with a median age of 2.3 years (Q1-Q3 1.8-2.9, min-max 1.1-4.0 years), were included. Sixty-one children (42%) developed PFD; 13% were tube-fed (n=19). Almost 40% of children with PFD failed to thrive (n=23). The presence of chronic respiratory symptoms was associated with the development of PFD. Ten children with PFD (16%) had no other condition or OA-related complication. CONCLUSION: PFD are common in children with OA, and there is no typical profile of patients at risk of PFD. Therefore, all children with OA require a systematic screening for PFD that could improve the care and outcomes of patients, especially in terms of growth.

Nutritional status at age 1 year in patients born with esophageal atresia: A population-based, prospective cohort study.

Objective: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure. Study design: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model. Results: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year (P < 0.05). Neither EA type nor surgical treatment was associated with growth failure. Conclusion: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies.

ABO-incompatible pediatric liver transplantation in very small recipients: Birmingham's experience.

Liver transplantation (LT) for very small recipients is challenging but in experienced centres, good results can be achieved. Despite the risk of antibody-mediated acute rejection, some studies have demonstrated the safety of ABO incompatible liver transplantation (ILT) in children and particularly in infants. The aim of our study was to describe the outcome of liver transplantation in infants <5 kg and the safety of using ILT in this group. All LT performed between 1991 and 2010 in children <5 kg were reviewed. Twenty-nine patients were included, five of whom had an ILT. Acute liver failure was encountered in 20 cases. The recipient age and weight at transplantation were respectively 63 days (range: 14-268 days) and 4 kg (range: 2.4-5 kg). The graft-to-recipient ratio was 6.1% (range 2.3-9%). An aortic conduit and delayed abdominal closure were used respectively in 76% and 81% of the procedures. The ABO compatible liver transplantation (CLT) and ILT groups were similar regarding recipient's demographics, graft types or technical transplantation data. The one- and five-yr patient and graft survival were respectively 62%, 62% and 62%, 57.9% with a median follow-up of 95 months. Vascular complications occurred in six cases (21.4%) and biliary complications were encountered in five patients (17%). Acute and chronic rejection developed respectively in 37% and 26% of the recipients. The five patients undergoing ILT are all alive without graft lost after a median follow-up of 34 months (range 7-55 months). When compared with the CLT group, no significant differences were found regarding patient or graft survival, vascular or biliary complications and rejection rates. In our experience, ILT in small infants has short and long term outcomes comparable to ABO-compatible grafts and excellent results can be achieved with a standard immunosuppressive protocol. To avoid mortality on the waiting list for neonatal recipients, ABO-incompatible liver grafts can be used safely.

Esophageal Atresia and Respiratory Morbidity.

BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.

Back-table reconstruction of the donor replaced right hepatic artery prior to liver transplantation: what is the real impact on arterial complications?

BACKGROUND/AIMS: The aim of this study was to analyze the technique of reconstruction, prior to liver transplantation, of donor replaced right hepatic arteries and to study its impact on graft outcome. METHODOLOGY: Two groups of liver grafts were retrospectively examined. The first group (group 1) did not require any arterial reconstruction prior to liver transplantation (n=507). The second one (group 2) included grafts with right replaced hepatic arteries which required reconstruction (n=75). Patients' data were analyzed to evaluate the effect of the arterial reconstruction on the incidence of posttransplant mortality, arterial and biliary complications on one hand and patient and graft survivals on the other. We also made a comprehensive literature search to analyze the different approaches described to reconstruct such variation. RESULTS: The two groups showed no statistical sig nificant difference for early posttransplant mortality and arterial complications. Hepatic arterial thrombosis occurred in two cases in the group 2 (2.6%) and in four cases in group 1 (0.7%); p=0.17. Patient and graft survivals over the whole follow-up period were not significantly different between the two groups. The analysis of the literature found few publications dealing with reconstruction of right replaced hepatic arteries, though because various techniques were reported and the samples of patients were small, a preferred method could not clearly be identified. CONCLUSIONS: Graft arterial reconstruction of a right replaced hepatic artery using a safe and rigorous technique does not enhance the risk of arterial complications or graft loss and the technique using the GDA stump could be recommended for routine use.

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.

Thoracoscopic Rib Resection in Children.

OBJECTIVE: The authors present a multicenter retrospective series of different benign rib lesions in children operated on using thoracoscopy. MATERIALS AND METHODS: Between 2005 and 2015, 17 rib resections were performed thoracoscopically, in four French departments of pediatric surgery. Of these 17 cases, 13 exostoses, 2 endochondromas, 1 synostosis, and 1 Cyriax's syndrome were noted. Inclusion criteria were benign tumors or rib anomalies such as synostosis, in children younger than the age of 18 years, and thoracoscopy. Open surgery and malignant tumors were excluded. Thoracoscopy was put forward using one optical port as well as one or two operative ports. RESULTS: Ten patients presented with chest pain, dyspnea, or unexplained cough. Six tumors were incidentally diagnosed. One patient presented with a chest wall deformation. Single-lung ventilation was required in 2 cases. In 1 case of endochondroma, a segmental rib resection was performed, leaving a part of the periosteum and the intercostal vessels and nerve. In this case, rib resection was performed using an endoscopic shaver drill system. As for the other cases, a simple resection of the tumor or of the bridge between two ribs (synostosis) was performed. In these cases, a Codman Kerrison laminectomy rongeur was used. There was no complication during or after surgery. Nevertheless, 2 years after surgery, pain did not disappear in 1 case, probably due to a definitive intercostal nerve damage. CONCLUSION: Benign rib lesions in children are rare. Thoracoscopy may be offered to reduce the functional deleterious consequences of an open surgery. It may be put forward especially in case of hereditary multiple exostoses where redo procedures may be required.

Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.

[Surgery of hypospadias complications]. / Chirurgie des complications de l'hypospade.

The very large number of surgical techniques described and the inadequate long-term results published for each of them illustrate the difficulty of objective evaluation of hypospadias surgery [9]. Although the majority of surgeons define a satisfactory result as a straight penis without excess skin, regular scars, an apical urethral meatus with a "good" urinary stream, evaluation of these criteria remains very subjective due to the lack of precise, reliable, and standardized methods. The surgeon's criteria of success of this surgery differ significantly from the patient satisfaction criteria [53]. The complication rate reported in the literature therefore varies from 5% to 70% depending on the technique used and the surgeon's honesty.

[Surgical management of congenital adrenal hyperplasia in young girls]. / Prise en charge chirurgicale de l'hyperplasie congénitale des surrénales (HCS) chez la fille.

Congenital adrenal hyperplasia (CAH) is the commonest cause of virilization in genetically female children. Although no consensus has been reached concerning the indications, surgical management of these patients has improved considerably over the last twenty years. The authors describe the main genitoplasty techniques currently used and report their results, although they are difficult to interpret in view of the small number of published series.