Increased Serum Levels of Activated Caspases in Murine and Human Biliary Atresia.

In biliary atresia (BA), apoptosis is part of the pathomechanism, which results in progressive liver fibrosis. There is increasing evidence suggesting that apoptotic liver injury can be non-invasively detected by measuring the caspase activity in the serum. The purpose of this study was to investigate whether serological detection of caspase activation mirrors apoptotic liver injury in the infective murine BA-model and represents a suitable biomarker for BA in humans. Analysis showed increased caspase-3 activity and apoptosis in the livers of cholestatic BALB/c mice, which correlated significantly with caspase activation in the serum. We then investigated caspase activation and apoptosis in liver tissues and sera from 26 BA patients, 23 age-matched healthy and 11 cholestatic newborns, due to other hepatopathies. Compared to healthy individuals, increased caspase activation in the liver samples of BA patients was present. Moreover, caspase-3 activity was significantly higher in sera from BA infants compared to patients with other cholestatic diseases (sensitivity 85%, specificity 91%). In conclusion, caspase activation and hepatocyte apoptosis play an important role in experimental and human BA. We demonstrated that serological detection of caspase activation represents a reliable non-invasive biomarker for monitoring disease activity in neonatal cholestatic liver diseases including BA.

A novel approach in the intraoperative management of ovotesticular DSD.

Ovotesticular disorder of sex development (OTD) management remains challenging. In OTD, cautious gonadal evaluation and separation of ovarian and testicular components might be required to avoid virilization of a patient with female identity. Herein we report our minimal invasive approach in this very rare condition. The gonads are externalized under laparoscopic control through trocar openings. Intraoperative ovotesticular ultrasonography (US) is used for clear identification of ovarian and testicular tissue which can then be safely separated. We strongly promote the use of a minimal invasive approach in the management of these patients undergoing long term treatment and often multiple procedures.

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.

Clinical Outcome of Children With Antenatally Diagnosed Hydronephrosis.

Fetal renal pelvis dilation is a common condition, which is observed in 1-4. 5% of pregnancies. In many cases, this finding resolves spontaneously. However, sometimes it may be a signal of significant urinary tract pathologies. The main abnormalities found after birth are uretero-pelvic junction stenosis, primary vesicoureteral reflux, megaureter, duplex kidneys, and posterior urethral valves, with uretero-pelvic junction stenosis and primary vesicoureteral reflux accounting for most of the cases. Diagnosis, management, and prognosis at short and longer term of these conditions will be reviewed in this article.

Extravesical Ureteral Reimplantation Following Lich-Gregoir Technique for the Correction of Vesico-Ureteral Reflux Retrospective Comparative Study Open vs. Laparoscopy.

Introduction: The aim is to compare the outcome of open versus laparoscopic Lich-Gregoir technique in patients with vesicoureteral reflux. We report a retrospective multicenter comparative study between open and laparoscopic extra-vesical ureteral reimplantation (EVUR) following Lich-Gregoir (LG) technique for the correction of Vesico-Ureteral Reflux (VUR). Materials and Methods: Between January 2007 and December 2015, 96 patients with VUR (69 females and 27 males) and deterioration of the renal function, underwent EVUR following LG technique. Fifty patients (16 males and 34 females) were operated by open surgery (group A). The mean age was 4.22 years-old, (14-147 months). Laparoscopic approach (group B) was performed in 46 patients (11 males and 35 females). The mean age was 4.19 years-old (15-110 months). We compared the results in relation to degree of VUR, operative time, hospital stay, post-operative pain medications, recovery time, complications, successful rate, recurrence, and follow-up. Statistical analysis was done used Chi square test for categorical variables and the Student t-test for continuous variables. P < 0.05 was considered significant. Results: In both groups no correlation was identified between age or weight and operative time, length of stay or total analgesia used. The mean operative time for group A was 63.2 and 125.4 min for unilateral and bilateral VUR, respectively, and for the group B was 127.90 and 184.5 min, respectively. There was no conversion in the laparoscopic group. Perioperative mucosal perforation of the bladder occurred in 6 patients of group A and 4 patients of group B and was immediately repaired. One patient had to be reoperated for leakage in group B. The mean duration of Morphine, IV and PO analgesia was shorter in group B. The mean hospital stay was 5.46 days for group A and 1.54 days for Group B. The success rate was 98% in group A and 97, 8% in group B. The mean follow-up was 3.67 years for the open and 1.54 years for the laparoscopic group. Transitory voiding dysfunction occurred in bilateral EVUR in one case in each group. Conclusion: Laparoscopic or Open approach for the correction of VUR following Lich-Gregoir technique is effective in unilateral and bilateral VUR with similar results. Laparoscopic approach reduces significantly (p < 0.05 in each item) post-operative pain medication, hospital stay, and allows for a faster return to normal activity.

Pulmonary Myofibroblastic Tumour Involving the Pericardium and Left Atrium in an 18 Month Infant.

Inflammatory myofibroblastic tumor (IMT) is the most frequent primary lung tumor in children and it may be locally aggressive. The management of a locally advanced pulmonary IMT in an 18 month-old female child is presented.A left pulmonary mass was incidentally found on the computerized tomography (CT) scan of a child with persistent systemic inflammatory syndrome. Biopsy confirmed the diagnosis; after preoperative corticotherapy, left pneumonectomy was performed. The pericardium and left atrium were invaded and resected, requiring pericardial reconstruction. There is no relapse at four years of follow-up.Steroids play a role in tumor size reduction, but marginal resection is the gold standard. Extended approaches are feasible and often required in advanced cases.

Transverse comparisons between ultrasound and radionuclide parameters in children with presumed antenatally detected pelvi-ureteric junction obstruction.

PURPOSE: The main criteria used for deciding on surgery in children with presumed antenatally detected pelviureteric junction obstruction (PPUJO) are the level of hydronephrosis (ultrasonography), the level of differential renal function (DRF) and the quality of renal drainage after a furosemide challenge (renography), the importance of each factor being far from generally agreed. Can we predict, on the basis of ultrasound parameters, the patient in whom radionuclide renography can be avoided? METHODS: We retrospectively analysed the medical charts of 81 consecutive children with presumed unilateral PPUJO detected antenatally. Ultrasound and renographic studies performed at the same time were compared. Anteroposterior pelvic diameter (APD) and calyceal size were both divided into three levels of dilatation. Parenchymal thickness was considered either normal or significantly decreased. Acquisition of renograms under furosemide stimulation provided quantification of DRF, quality of renal drainage and cortical transit. RESULTS: The percentages of patients with low DRF and poor drainage were significantly higher among those with major hydronephrosis, severe calyceal dilatation or parenchymal thinning. Moreover, impaired cortical transit, which is a major risk factor for functional decline, was seen more frequently among those with very severe calyceal dilatation. However, none of the structural parameters obtained by ultrasound examination was able to predict whether the level of renal function or the quality of drainage was normal or abnormal. Alternatively, an APD <30 mm, a calyceal dilatation of <10 mm and a normal parenchymal thickness were associated with a low probability of decreased renal function or poor renal drainage. CONCLUSION: In the management strategy of patients with prenatally detected PPUJO, nuclear medicine examinations may be postponed in those with an APD <30 mm, a calyceal dilatation of <10 mm and a normal parenchymal thickness. On the contrary, precise estimation of DRF and renal cortical transit should be performed in patients with APD >30 mm, major calyceal dilatation and/or parenchymal thinning.

Predicting the clinical outcome of antenatally detected unilateral pelviureteric junction stenosis.

OBJECTIVE: To determine, in children with antenatally detected pelviureteric junction (PUJ) stenosis, what factors may be predictive for deterioration of differential renal function (DRF) in case of conservative treatment or improvement of DRF in case of pyeloplasty. METHODS: This study analyzed and compared the initial level of hydronephrosis, DRF, quality of renal drainage, and cortical transit with the late DRF outcome. We reviewed the medical charts of 161 consecutive children with antenatally diagnosed PUJ stenosis during a 10-year period (between 1997 and 2007). From this cohort, we retained 81 children with unilateral PUJ and strictly normal contralateral kidney, with a median follow-up of 67 months. Repeated ultrasounds, voiding cystourethrography, and radionuclide renograms were performed in all children. RESULTS: Fifty patients never underwent a surgical intervention (62%), whereas surgical repair (Anderson-Hynes dismembered pyeloplasty) was performed in 31 (38%). During conservative follow-up, DRF deterioration was observed in 11% of patients. After pyeloplasty, DRF improvement was observed in 25% of patients. Abnormal cortical transit was the only predictive factor of DRF deterioration in case of conservative approach, whereas the initial degree of hydronephrosis, or renal drainage, and the initial DRF level were not predictive. In children who were operated on, only impaired cortical transit was predictive of DRF improvement postoperatively. CONCLUSION: Conservative management of children with unilateral PUJ stenosis is a safe procedure. Impaired cortical transit although imperfect, seems the best criterion for identifying children for whom pyeloplasty is warranted.

Impact of concomitant thoracic interventions on feasibility of Nuss procedure.

BACKGROUND: This study aimed to analyze the feasibility of subsequent minimally invasive pectus repair, particularly modified Nuss procedure, combined with simultaneous thoracic procedures for different underlying intrathoracic diseases and conditions. METHODS: A total of 110 patients, who underwent minimally invasive pectus repair in Nuss technique over a 5-year period, were retrospectively analyzed concerning complications, cosmetic results, and satisfaction. Six patients (5%) underwent the Nuss procedure with concomitant thoracic interventions. Patients with prior cardiac surgery or planned redo pectus repair were not examined and were excluded. The mean age of 6 patients (3 male and 3 female) was 11 years (range, 5.5-17.2). Two patients with former left-sided transabdominal diaphragmatic hernia repair and 1 with former lobectomy of the left lower lobe underwent thoracoscopic adhesiolysis. Two underwent thoracotomy: one for closure of a recurrent left-sided diaphragmatic hernia with fundoplication owing to a large hiatal hernia, another for lobectomy of the right middle lobe owing to recurrent infections and bronchodysplasia. One patient presented with anterior mediastinal mass, which was suspected to be benign, and underwent thoracoscopic complete resection. All patients underwent a 1-stage procedure with subsequent simultaneous Nuss procedure. RESULTS: Simultaneous Nuss procedure was feasible without intraoperative complications in all patients (100%). Thoracoscopic adhesiolysis did not affect the feasibility of the Nuss procedure in 3 patients with former diaphragmatic hernia repair, particularly former lobectomy in one. Thoracotomy with middle-lobe lobectomy, as well as repair of recurrent diaphragmatic hernia and fundoplication in 2, did not affect modified Nuss technique and dispensed thoracoscopic guidance. Histopathologic analysis in one patient with a removed anterior mediastinal mass revealed Hodgkin lymphoma (stage IA), and the patient received multiagent chemotherapy. The postoperative course was uneventful in 5 of 6 patients. One patient required intermittent drainage of pleural effusion after simultaneous lobectomy. At follow-up (6 months-5 years), 6 patients had excellent cosmetic results and good quality of life confirmed by a questionnaire. There was no evidence of recurrent malignancy in one patient after 6 months. CONCLUSIONS: Thoracic surgery and subsequent Nuss procedure can be performed simultaneously. Underlying conditions, such as prior repair of congenital diaphragmatic hernia or diaphragmatic eventration, as well as former lobectomy, had no impact on feasibility. Open thoracotomy can be combined with Nuss procedure dispensing thoracoscopy.

Thoracoscopic thoracic duct sealing with LigaSure in two children with refractory postoperative chylothorax.

PURPOSE: Chylothorax is an uncommon complication of cardiac surgery. We report two cases of the thoracoscopic management of persistent postoperative chylothorax by thoracic duct thermofusion and section with the LigaSure device. MATERIALS AND METHODS: A 6-year-old boy and a 3-year-old girl developed persistent chylothorax following an aortic coarctation repair and a Fontan procedure, respectively. The former presented with a left chylothorax due to direct surgical injury, and the latter, a right chylothorax due to indirect lymphatic rupture secondary to increased venous pressure. In both patients, voluminous chylous drainage persisted for more than 3 weeks despite maximal medical treatment. Ultimately, a right thoracoscopic coagulation and section of the thoracic duct with the 5-mm LigaSure device was performed. The LigaSure is a computer-controlled bipolar diathermy system designed to seal blood vessels up to 7 mm in diameter, with no specific recommendations regarding the lymphatics. RESULTS: The lymph leakage ceased within 2 days in the first patient and immediately in the second one, with no recurrence after oral intake resumption on day 5. Both patients are free of recurrence at 1 year. CONCLUSION: Thoracoscopic coagulation and section of the thoracic duct above the diaphragm with the LigaSure device appears to be a simple, effective, and safe therapeutic option for treatment of refractory postoperative chylothorax in children.

Laparoscopic partial splenectomy using radiofrequency ablation for nonparasitic splenic cysts in two children.

Nonparasitic splenic cysts are uncommon lesions, for which partial splenectomy offers a definitive treatment while preserving splenic function. We report the use of radiofrequency energy for laparoscopic partial splenectomy to treat two children with nonparasitic splenic cysts by creating a 1-cm thick coagulated parenchyma resection plane, allowing bloodless splenic transection. This approach spares the need for blood transfusions, reduces hospital stay, and offers great promise for partial splenectomy in children.

Traumatic duodenal necrosis with avulsion of Vater's papilla in a child.

Blunt duodenal trauma with avulsion of the papilla of Vater is an extremely uncommon injury in children. The usual management of these injuries relies on complex reconstructive procedures and bypasses. We report the case of a 6-year-old girl with near total traumatic duodenal necrosis and avulsion of Vater's papilla managed by a simple physiological montage avoiding pancreatic resections and gastrointestinal bypasses.

[Atypical presentation of an intestinal duplication in a three month old child]. / Présentation atypique d'une duplication intestinale chez un enfant de trois mois.

Intestinal duplication is an uncommon congenital anomaly that often is diagnosed during childhood. Ultrasound diagnosis is based on the presence of a characteristic double-walled cystic mass. We report a case of duplication in a three Month old child presenting with small bowel obstruction. This case is unusual due to the presence of calcifications that are uncommon in intestinal duplication.

Achalasia of the cardia in Allgrove's (triple A) syndrome: histopathologic study of 10 cases.

Allgrove's syndrome, i.e., achalasia, addisonianism, alacrima (OMIM 231550) is an autosomal recessive disorder recently associated with the AAAS gene coding for the Aladin protein. However, the pathophysiology of achalasia in Allgrove's syndrome remains obscure. Here we investigated the histopathology of the cardia in Allgrove's syndrome. Myectomy specimens from 10 children with Allgrove's syndrome and four normal cardia were studied by routine staining and by immunohistochemistry for the pan-neuronal marker PGP9.5, neuronal NO synthase, interstitial cells of Cajal, and CD3+ lymphocytes. In the normal cardia, myenteric ganglia, intramuscular nerve fibers, and interstitial cells of Cajal were numerous, whereas myenteric fibrosis and lymphocyte infiltrates were absent. In Allgrove's syndrome, fibrosis of the intermuscular plane was prevalent in all patients. Myenteric ganglia were absent, decreased, or apparently normal in 1 of 10, 8 of 10, and 1 of 10, respectively. Neuronal NO synthase was absent in 7 of 10 and decreased in 3 of 10, whereas interstitial cells of Cajal appeared normal in 7 of 10 and decreased in 3 of 10. Lymphocytes infiltrating the myenteric plexus were present in 6 of 10. Pyloromyectomy specimens available for six patients showed normal histopathologic features. In conclusion, the lack of neuronal NO synthase and fibrosis of the intermuscular plane can be linked to the defective cardia relaxation. Other features were less constant and may reflect the variability of disease expression and progression among patients with Allgrove's syndrome.