[Impact of the acquisition of a flexible ureteroscope on the management of upper urinary tract stones in children]. / Impact de l'acquisition d'un urétéroscope souple sur le traitement des calculs du haut appareil urinaire chez l'enfant.

INTRODUCTION: Firstly reported in the early 1990s for the treatment of upper urinary tract stones in adult patients, flexible ureteroscopy (F-URS) has been used in children during the past 10 years and is now considered as a viable, but still second-line alternative to extracorporeal shockwave lithotripsy in these patients (ESWL). The aim of this study was to assess the impact of the acquisition of a F-URS on the management of upper urinary tract stones in children. PATIENTS AND METHODS: Data of all ESWL, F-URS and percutaneous nephrolithotomy performed for upper urinary tract stones in children from 0 to 18 years old in a single center from 2000 to 2014 have been collected retrospectively. Patients have been divided into two groups: group 1 before the acquisition of the F-URS (2000-2008) and group 2 after the acquisition of the F-URS (2008-2014). Preoperative data and peri-operative outcomes were compared between both groups using the χ(2) test and Fisher exact test for discrete variables and the Mann-Whitney test for continuous variables. RESULTS: Thirty-seven children have been treated during the first era and 32 during the second one. The two groups were similar in terms of age (7.2 years vs 8.1 years; P=0.54), size of the largest stone (15 mm vs 16.2mm; P=0,56) and number of stones per patient (1.4 vs 2; P=0,07) but the sum of stone diameters was higher in group 2 (16.9 mm vs 24.2mm; P=0,048). The stone-free rates were comparable in both groups (28.1% vs 32.2% after the first procedure; P=0.72), as were the mean number of procedures per patient (2.4 vs 2.5; P=0.78), the total length of stay (2.7 days vs 2.9 days; P=0.77), and the number of patients who experienced at least one complication (37.8% vs 40.6%; P=0.87). CONCLUSION: The acquisition of a F-URS allowed the treatment of more complex stones with a similar efficacy and without increasing morbidity. Further studies are needed to define the role of F-URS in the management of upper urinary tract stones in children.

Preliminary experience with the use of an autologous platelet-rich fibrin membrane for urethroplasty coverage in distal hypospadias surgery.

OBJECTIVE: Platelet-rich fibrin (PRF) has been shown to have structural and biological properties that promote tissue healing. This prospective study evaluated the feasibility, safety, and efficiency of using autologous PRF membrane for urethroplasty coverage in distal hypospadias. MATERIALS AND METHODS: We prospectively included 33 patients with distal hypospadias operated on between June 2010 and September 2011. Urethroplasties were performed using the Duplay technique. During surgery, 5-10 ml of patient's blood was collected and immediately centrifuged. A PRF clot was transformed into a dense fibrin membrane with a particular cell content and architecture. This membrane was applied and sutured over the urethroplasty. The perioperative course and complications were recorded. Outcomes were compared with those in a control group of children undergoing the same procedure, but with another mean of coverage. RESULTS: With a median follow-up of 8 months (range, 6-18 months), urethral fistula occurred in 2/33 patients. No other complication was noted. No complication related to the blood sampling was reported. There was no statistically significant difference with the control group (p = 0.65). CONCLUSION: The PRF patch seems to be a safe and efficient covering technique. Thus, procedure is an additional approach to coverage for hypospadias surgery, and may help to reduce the incidence of postoperative complications when coverage healthy tissue is not available.

Long-term evaluation of respiratory status after esophageal atresia repair.

RATIONALE: Esophageal atresia (EA) is a congenital malformation. Nowadays, its initial prognosis is excellent thanks to improvements in neonatal and surgical management. However, the assessment of long-term respiratory outcome has become necessary in affected children and was thus performed in this study. The benefits of cardiopulmonary function testing were also examined. METHODS: The medical records of 77 children operated on for EA between 1990 and 2004 were reviewed. The results of respiratory function testing and cardiopulmonary response to effort were collected, together with neonatal and anthropometric data. RESULTS: Acceptable measurements were obtained in 31 children with EA. These children were comparable to the ones lost during follow-up. The results of pulmonary function tests (PFTs) were abnormal in 21 cases (68%). A poor ventilatory response was detected in 14 children (45%) by cardiopulmonary function testing. Ten children who had abnormal results on PFTs were not under any anti-asthmatic treatment. CONCLUSIONS: Impaired lung function was noted in children with repaired EA. Indeed, cardiopulmonary function tests results correlated with standard spirometric parameters and revealed minimal clinical symptoms. Moreover, many children with EA had a limited ventilatory reserve (VR). These results indicate that respiratory symptoms are often neglected in children with repaired EA and reinforce the need to provide adequate treatment.

[Gliomatosis peritonei, an unusual abdominal carcinomatosis: report of two cases]. / La gliomatose péritonéale: un aspect inhabituel de carcinomatose: à propos de deux cas.

We report on 2 cases of ovarian teratoma associated with gliomatosis peritonei in 2 young girls aged 9 and 14 years. Gliomatosis peritonei is an unusual miliary-like peritoneal carcinomatosis of glial tissue. In our experience, follow-up was important to detect abdominal recurrence of glial implants, which had to be removed by iterative surgery. We report a 14-year follow-up and an unusual pleural metastatic disease.

Technical problems and complications of a transanal pull-through for Hirschsprung's disease.

BACKGROUND: The purpose of this study was to evaluate the technical difficulties and complications of a transanal pull-through for Hirschsprung's disease. MATERIAL AND METHODS: This report was based on a multicentric retrospective study of 65 cases. Pull-through procedures were transanal Swenson or Soave procedures in 26 and 39 cases, respectively. RESULTS: Evaluation of the aganglionic level, peri-rectal dissection, and anastomosis were the three steps in the procedure where surgeons encountered difficulties. Such difficulties led to serious complications in 3 cases. A patient with a colon biopsy before the pull-through procedure had a postoperative pneumoperitoneum requiring a second laparoscopy for suture and washing. Another patient had peritonitis due to anastomotic leakage. Finally, a difficult rectal dissection in a neonate led to a urethral injury requiring secondary urethral repair. Only 41 of the 65 patients had no abdominal scars (63 %). CONCLUSION: We considered the transanal pull-through for Hirschsprung's disease to be a reliable technique. Nevertheless, it requires an urethral stent, precise dissection, careful anastomosis and selected indications in order to avoid major complications.

[Endoscopic therapy for pancreatitis in children]. / Endoscopie interventionnelle dans les pancréatites de l'enfant.

Authors report on 3 cases of children treated by therapeutic endoscopic retrograde cholangiopancreatography for pancreatitis (ERCP). The first child presented with familial pancreatitis: he was treated by sphincterotomy and lithiasis extraction. The second child presented with pancreatitis secondary to pancreas divisum: she was treated by sphincterotomy and stunt of small caroncula. The third child presented with severe pancreatitis with pseudocyst: after drainage she was treated by sphincterotomy, Wirsung dilatation and lithiasis extraction. ERCP is a beneficial treatment and may be alternative to surgery in complicated pancreatitis.

Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia.

The authors report Kasabach-Merritt syndrome (KMS) in a patient with thrombocytopenia and splenic hemangioma. A 13-month-old boy with a history of anemia, thrombocytopenia, and abdominal mass was admitted to the hospital. The scintigraphic studies showed that a large mass contiguous to the spleen was responsible for the platelet uptake. After partial splenectomy, the platelet count returned to normal. This report of KMS in a child with splenic hemangioma suggests that the scintigraphic studies are mandatory to confirm diagnosis. Indium-111-labeled platelets are useful in identifying hemangiomatous sequestration of platelets in patients with thrombocytopenia.

Detoxifying activity in pig livers and hepatocytes intended for xenotherapy.

BACKGROUND: Both livers and hepatocytes from pigs have been proposed for the treatment of end-stage liver diseases, as an alternative to allogeneic liver transplants. However, little is known of the capability of porcine hepatocytes to fulfill the biotransformation pathways of toxic compounds, including those released from livers in acute failure. We have studied the activity and expression of detoxifying enzymes in porcine livers and in cultured hepatocytes and their induction by phenobarbital. METHODS: Cytochromes P450 (CYP) 1A, 2B, and 3A and GST-like activities were tested with the following specific substrates: 7-ethoxyresorufin, 7-pentoxyresorufin, nifedipine, testosterone, 1-chloro-2,4-dinitrobenzene, 1,2-dichloro-4-nitrobenzene, and ethacrinic acid. CYP 1A1/2-, 2B1/2-, 2E1- and 3A4-related and GSTalpha proteins were analyzed by Western blotting and CYP 1A1/2, 2B1/2, 2C6, 2E1, and 3A4, aldehyde dehydrogenase, epoxide hydrolase, and GSTalpha-like RNA by Northern blotting. RESULTS: Enzymatic activities reflecting the expression of CYP 1A-, CYP 2B-, CYP 2E1-, and CYP 3A-like genes, that is, ethoxyresorufin-O-deethylase, pentoxyresorufin-O-deethylase, nifedipine oxidase and testosterone 6beta-hydroxylase, and chlorzoxazone 6-hydroxylase, were identified in pig livers. CYP 1A and CYP 2E1, GSTalpha-like proteins, CYP 1A, 2C, and 2E, epoxide hydrolase, aldehyde dehydrogenase, and GST like RNA were expressed in vivo and in vitro. CYP 2B and CYP 3A RNA and proteins, and their associated activities were induced by phenobarbital. CONCLUSIONS: Porcine hepatocytes express the most important biotransformation enzymes and their corresponding activities and RNA. Thus, livers and hepatocytes from pigs can detoxify a large spectrum of exogenous and endogenous compounds, which makes them a convenient substitute for allogeneic transplants for patients with liver failure.

[Urinary lithotripsy in children. Multicenter study of the Pediatric Urology Study Group]. / Lithotritie urinaire de l'enfant. Etude multicentrique du Groupe d'Etudes en Urologie Pédiatrique (GEUP).

The authors present the results of a survey conducted among French paediatric urologists belonging to the Groupe d'Etudes en Urologie Pédiatrique (GEUP) (Paediatric Urology Study Group). This study, based on 122 cases observed in 13 centres, is not exhaustive, but is nevertheless statistically significant. The preoperative assessment confirms the usual findings of urinary stones in children: pyelonephritis, haematuria and abdominal pain, the usual presenting complaint, concomitant malformative uropathy (10% of cases) and a predominance of calcium stones. More than 200 stones were treated, larger than 10 millimeters in diameter in one-third of cases. Renal stones, mainly caliceal (more than 50%), included 11 staghorn calculi. This study also included 22 ureteric stones, mainly in the pelvic ureter, and 2 bladder stones. Lithotripsy was ultrasound-guided in 2/3 of cases and required general anaesthesia in about 3/4 of cases. Ureteric catheterization was required in 19 infants preoperatively, but in only 2 infants (stein strasse) postoperatively. One or two lithotripsy sessions were sufficient in most cases, but 4 sessions were necessary in 5 patients, to the same kidney in 1 case. The mean hospital stay was 2 to 3 days, but the procedure was performed on an outpatient basis in 15 cases. The immediate postoperative course was uneventful and asymptomatic. This survey revealed about 10% of complete failures, corresponding to solitary caliceal stones in 2/3 of cases; 29 partial failures were essentially due to lower caliceal stones and staghorn calculi; 84 successes (stone-free), mainly pelvic or simple caliceal stones. Scintigraphy did not reveal any immediate postoperative impairment of renal function. This study reported a success rate of about 70%, regardless of the type of apparatus used. Assessment of the results of ESWL requires sufficient follow-up both concerning the outcome of fragmented stones and evaluation of possible functional repercussions. This survey defines the main indications: although ESWL can be applied to most stones, some stones constitute poor indications (cystine stones, stenotic malformative uropathy) or dubious indications: small lower caliceal stones, densely calcified staghorn calculi in older children. This study confirmed the efficacy and low morbidity of ESWL in children. A prospective study needs to be conducted according to a rigorous protocol in order to refine the technique and indications while reducing the possible long-term risks.

Tracheobronchial ruptures from blunt thoracic trauma in children.

BACKGROUND/PURPOSE: Tracheobronchial ruptures in blunt thoracic trauma in children are rare. The aim of this study was to suggest the means of an early diagnosis and a conservative management as often as possible. METHODS: Sixteen cases of tracheobronchial ruptures by blunt thoracic trauma were observed over 26 years in 9 regional pediatric centers. RESULTS: There were 12 boys and 4 girls, from ages 1 hour to 17 years. Nine children presented with associated lesions. Fibroscopy established the following diagnosis: 8 tracheal wounds and 8 bronchial wounds. Six children were operated on within 18 hours (on average) after installation of a thoracic drainage. Two lobectomies, 3 ideal tracheal sutures, and 1 bronchial suture were performed. Seven children were treated exclusively by thoracic drainage. Two of them were intubated through the lesion, leading to a transitory endoprothesis accompanied or not by an external thoracic drainage. One infant recovered spontaneously. There were no deaths in this series. Two recurrent postoperative nerve injuries were noted, one of which was a transitory spontaneously resolutive scar bud and one a granuloma treated by laser. Three times, a stenosis occurred after a conservative management. Two were operated on. CONCLUSIONS: Tracheobronchial ruptures in children are rare. An early fibroscopy holds an important place in the approach of this pathology. Treatment is variable, based on thoracic lesions, their tolerance by the child, and associated lesions. Surgery is not the only therapy because conservative treatment by simple thoracic drainage or lesion intubation has proved effective.

[Hepatocytes in cell therapy]. / Hépatocytes en thérapie cellulaire.

Cell-based therapy could represent an alternative treatment to orthotopic liver transplantation in acute liver failures and for the correction of genetic defects of various enzymatic functions. Several recent studies indicate that hepatocytes injected either in the spleen or in portal vein can restore liver-specific function(s) in animal model systems. Alternatively, an extracorporal hybrid bioartificial liver might provide liver-specific functions, maintain the patient alive and allow spontaneous recovery of the patient's own liver, or act as a bridge toward liver transplantation in acute liver failures. Various drawbacks of devices such as flat culture substrates, hollow-fiber bioreactors or microcarriers led us to develop a reliable extracorporeal bioartificial liver based on alginate-entrapped hepatocytes. This system was used successfully for the correction of the Gunn rat genetic defect which results in the lack of bilirubin conjugation. The development of this system for clinical purposes requires large yields of functional hepatocytes. We isolated porcine hepatocytes by collagenase perfusion of the liver and cells were immobilized within alginate beads which were subsequently inoculated in a bioreactor. Porcine hepatocytes expressed liver-functions at high levels, particularly those involved in detoxification and biotransformation processes; they were immunoisolated from immunoglobins and could be cryopreserved. This system represents a promising tool for the design of an extracorporeal bioartificial liver in human beings.

Mesenteric adenitis caused by Yersinia pseudotuberculosis presenting as an abdominal mass.

Infection by Yersinia pseudotuberculosis has become of increasing pathological importance. Patients normally present with symptoms similar to those of appendicitis, due to mesenteric adenitis. We present the case of 3 patients infected by Yersinia pseudotuberculosis who in addition to fever and abdominal pain had a palpable abdominal mass, so great was the enlargement of the mesenteric nodes. In 2 patients a laparotomy was carried out, followed by biopsy of a mesenteric lymph node. The diagnosis of Yersinia infection was confirmed by bacterial culture of the biopsied material and also by serology. In the third patient, serological studies and ultrasonic imaging of the abdomen led to early diagnosis and surgery was avoided. We suggest that a diagnosis of mesenteric adenitis due to Yersinia pseudotuberculosis should now be considered in all patients presenting with an abdominal mass, and in whom there is an appropriate clinical and epidemiological history. The diagnosis should be confirmed by abdominal ultrasound (alternatively Computerised Axial Tomography or Magnetic Resonance Imaging) and serological studies. In this way, unnecessary surgery can be avoided.

Intestinal vascular anomalies in children.

Vascular anomalies are an uncommon cause of gastrointestinal bleeding in childhood. Confusing nomenclature has made objective comparisons of published cases difficult and has interfered with an established consensus regarding diagnosis and therapeutic modalities. The purpose of this study was to clarify the situation by reviewing the records of all children who had intestinal vascular anomalies who were referred to our institution from 1975 to 1995. Thirteen lesions were identified in nine children (five boys and four girls). The median age at clinical onset was 8 years. Only two patients presented with a complex syndrome (Klippel-Trenaunay, 1; Osler-Rendu-Weber, 1). Diagnosis, location, and extension of these anomalies was only possible by angiography, which indicated that seven patients had isolated venous malformations and two had arteriovenous malformations. Because the lesions did not involve the serosa, intraoperative localization was a major problem. The main findings were a few slightly dilated mesenteric veins. Treatment was conservative in four children and surgical in five. Pathological findings on resected bowel demonstrated dilated and abnormal veins in the mucosa and submucosa. Selective angiography should not be delayed in patients with gastrointestinal bleeding if results of all other investigations are negative. Because these lesions are rarely recognizable on operative inspection, precise preoperative angiographic localization of intestinal vascular anomalies is essential to allow for a safe and limited resection of the involved bowel segment. Based on a better understanding of the natural history of these lesions, a classification of vascular anomalies of intestines in children is proposed.

Prenatal detection of a congenital pancreatic cyst and Beckwith-Wiedemann syndrome.

We report a case of congenital pancreatic cyst detected prenatally by ultrasound in a fetus with evidence for a diagnosis of Beckwith-Wiedemann syndrome (BWS). Neonatal hypoglycaemia was prevented. The cyst was managed by internal drainage. This is the second reported case of BWS associated with pancreatic cystic dysplasia and the first time that this association has been detected prenatally. Differential diagnosis of cystic abdominal lesions occurring in utero should take pancreatic cysts into consideration. This case suggests that pancreatic cysts should be included in the BWS phenotype.

Survival, proliferation, and functions of porcine hepatocytes encapsulated in coated alginate beads: a step toward a reliable bioartificial liver.

Orthotopic liver transplantation is the most effective treatment for fulminant hepatic failure. As an alternative treatment, an efficient extracorporeal bioartificial liver should contain a large yield of functional hepatocytes with an immunoprotective barrier, for providing temporary adequate metabolic support to allow spontaneous liver regeneration or for acting as a bridge toward transplantation. Survival, proliferation, and functions of porcine hepatocytes were evaluated in primary cultures and after embedding in alginate beads, which were subsequently coated with a membrane made by a transacylation reaction between propylene glycol alginate and human serum albumin. Disruption of total pig livers by collagenase perfusion/recirculation allowed the obtention of up to 10(11) hepatocytes with a viability greater than 95%. Hepatocytes in conventional cultures or embedded in coated alginate beads survived for about 10 days, secreted proteins, particularly albumin, and maintained several phase I and II enzymatic activities, namely ethoxyresorufin-O-deethylase, oxidation of nifedipine to pyridine, phenacetin deethylation to paracetamol, glucuroconjugation of paracetamol, and N-acetylation of procainamide. Typical features of mitosis and [3H]thymidine incorporation indicated that porcine hepatocytes proliferated in both conventional cultures and alginate beads. The efficacy of the membrane surrounding alginate beads for protecting cells from immunoglobulins was tested by embedding HLA-typed human lymphocytes, which were subsequently incubated with specific anti-HLA immunoglobulin G and complement. These data show that large yields of porcine hepatocytes that are embedded in coated alginate beads remain functional and are isolated from large molecular weight molecules, such as immunoglobulins. This system represents a promising tool for the design of an extracorporeal bioartificial liver, containing xenogeneic hepatocytes, to treat acute liver disease in humans.