Long-term outcome of liver transplantation in childhood: A study of 20-year survivors.

We report the results of a study of survival, liver and kidney functions, and growth with a median follow-up of 24 years following liver transplantation in childhood. From 1988 to 1993, 128 children underwent deceased donor liver transplantation (median age: 2.5 years). Twenty-year patient and graft survival rates were 79% and 64%, respectively. Raised serum aminotransferase and/or γ-glutamyl transferase activities were present in 42% of survivors after a single transplantation. Graft histology (35 patients) showed signs of chronic rejection in 11 and biliary obstruction in 5. Mean total fibrosis scores were 4.5/9 and 3/9 in patients with abnormal and normal serum liver tests, respectively. Glomerular filtration rate was <90 mL·min-1 in 35 survivors, including 4 in end-stage renal disease who were undergoing dialysis or had undergone renal transplantation. Median final heights were 159 cm for women and 172 cm for men; final height was below the target height in 37 patients. Twenty-year survival after childhood liver transplantation may be close to 80%, and final height is within the normal range for most patients. However, chronic kidney disease or altered liver biochemistries are present in over one third of patients, which is a matter of concern for the future.

Congenital portosystemic shunts in children: recognition, evaluation, and management.

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

The long-term outcome of hepatic artery thrombosis after liver transplantation in children: role of urgent revascularization.

Hepatic artery thrombosis (HAT), one of the most severe complications of pediatric orthotopic liver transplantation (OLT), often compromises graft and/or child survival. Of 590 OLT performed in 516 children over a 20-year period, 45 were complicated by early HAT, during the first 2 weeks after transplantation. Systematic Doppler ultrasonographic detection of HAT allowed successful surgical revascularization in 19 instances, resulting in a 20-year graft survival rate of 77% versus 24% of cases when revascularization was not attempted or failed. A combination of surgical emergency revascularization, biliary interventional radiology, biliary surgery and/or retransplantation resulted in an 80% 20-year patient survival rate, identical to that of transplanted children who did not experience early HAT. The majority of long-term survivors with their initial graft had normal liver tests, no biliary dilation on ultrasonography and minimal or moderate fibrosis on liver histology. A failed attempt at revascularization did not significantly alter patient survival. Despite these encouraging results, for the children and their parents to overcome the entire process in terms of reoperations, repeated radiological interventions, number of hospitalizations and emotional stress, remains an ordeal of such magnitude that it justifies renewed efforts to progress in the prevention of this complication.

[Transrectal drainage of pediatric pouch of Douglas abscesses under suprapubic US guidance: technique and results]. / Drainage par voie transrectale sous contrôle échographique sus-pubien des abcès du douglas chez l'enfant: technique et résultats.

Pelvic abscesses are not always amenable to percutaneous drainage. Transrectal drainage of pouch of Douglas abscesses, performed blindly by surgeons for many years, is not familiar to radiologists. The purpose of this article is to describe our technique for the drainage of pouch of Douglas abscesses under suprapubic US guidance and report our results in a series of 7 pediatric patients with deep pelvic abscess not amenable to percutaneous drainage after failure of antibiotics. The procedure was performed under general anesthesia. All procedures were successful and without complication. The drainage catheter was well tolerated in all cases with short post-drainage hospital stay. After failure of medical management, this technique can be used irrespective of patient age or sex using US transducers and drainage catheters routiney available in any radiology department.

Acquired renal cystic disease after liver transplantation in children.

To our knowledge, the development of renal cystic disease that may contribute to kidney dysfunction has never been reported after liver transplantation. Herein we have reported on the fortuitous finding of renal cystic lesions upon computed tomographic scans (CT) in 33 (30%) of 108 pediatric liver transplant recipients who were the subjects of a prospective study evaluating long-term kidney dysfunction at 10 years after liver transplantation. The renal lesions had 2 different appearances: that of simple renal cysts and that of round lesions that were spontaneously hyperdense before contrast injection. These high-density lesions had a low signal on T2 weighted sequences, but 70% of them had been missed at ultrasonography. Their aspect upon CT and magnetic resonance favored cystic lesions filled with hemorrhagic or milk calcium content. Both types of cystic lesions were associated in 14 children. The renal lesions were significantly associated with moderate renal dysfunction, biopsy-proven chronic liver graft rejection, and thrombosis of the retrohepatic vena cava. The physiopathology of these lesions is undetermined. Two important questions need to be clarified with respect to the risk of progression of renal dysfunction associated with individual volume changes and/or increased number of renal cysts, as well as the risk of renal cancer as has been reported in dialyzed patients with acquired cystic kidney disease.

Organization in response to massive afflux of war victims in civilian practice - experimental feedback from the November 2015 Paris terrorist attacks.

The arrival of a large number of war-weapon casualties at a civilian trauma center requires anticipation. A plan defining the management principles and the respective roles of the involved physicians and nurses and their interaction with each other is essential. Uni-directional patient flow associated with adequate numbers of staff physicians and nurses under the leadership of a medical director is essential to prevent the overwhelming of the trauma center. Routine and regular interaction between the pre-hospital medical flow control system and the medical director, on one hand, and between surgical teams and the medical director, on the other, are necessary to know when to apply "damage control" surgical techniques. Based on the feedback of a level 1 trauma center that received 53 victims of the November 13, 2015 terrorist attack in Paris, we present the factors of success, and the stumbling blocks.

[Duodenal duplication revealed by acute pancreatitis]. / Duplication duodénale révélée par une pancréatite aiguë.

INTRODUCTION: Duodenal duplications are rare congenital malformations whose revealing signs are highly variable and nonspecific. OBSERVATION: We report the case of a female infant who presented with neonatal acute pancreatitis complicated by recurrent ascites, profound hypoalbuminemia responsible for pleural and pericardial effusions, revealing a duodenal duplication cyst. The unusual and original clinical presentation as well as the difficulty detecting the duplication radiologically delayed the diagnosis. A prolonged medical treatment with octreotide, albumin infusions, and exclusive parenteral nutrition led to an almost total disappearance of the ascites before surgery. The outcome was favorable after surgical removal of the duplication with 1 year of follow-up. CONCLUSION: The diagnosis of duodenal duplication can be difficult and it may be necessary to repeat the ultrasound examinations. Surgical resection is delicate, especially when there is an abundant pancreatic ascites. Therefore, an adequate prolonged medical treatment to reduce this ascites is recommended before the surgery.

[Pediatric liver tumor: What to do?]. / Conduite à tenir devant une tumeur hépatique de l'enfant.

Two-thirds of pediatric liver tumors are malignant, but pseudotumors such as abscesses or hematoma can simulate a tumor. The pediatrician is often the first to discover a hepatic mass in a child. The diagnostic gamut varies depending on the child's age. Before the age of three years, the main diagnoses are hepatoblastoma and hemangioma, while after the age of three, hepatocarcinoma, sarcoma, focal nodular hyperplasia, and adenoma are more frequent. The laboratory findings to search for are alpha-fetoprotein whatever the age (increased in hepatoblastoma and hepatocarcinoma), beta-hCG, and urinary catecholamines in infants. Liver function is usually normal. Ultrasonography is the first-line examination to request. It confirms the hepatic location of the mass, differentiates solid from cystic tumors (cystic mesenchymal hamartoma and undifferentiated sarcoma), hypervascular findings (hemangioma in the infant, focal nodular hyperplasia in the older child), portal or hepatic thrombosis suggesting a malignant tumor, and findings of portacaval fistula predisposing to focal nodular hyperplasia and adenoma. At the end of this clinical, biological, and ultrasound examination, the pediatrician will refer the patient to a specialized center for further investigation and management, which are at best performed by pediatric oncologists, surgeons, and radiologists. Diagnostic confirmation and extension work-up will require CT or MRI depending on the patient's age and clinical state and the availability of equipment.

Ovarian tumors in children and adolescents: a series of 41 cases.

OBJECTIVE: Pictorial review with a detailed semiological analysis of ovarian tumors in children and adolescents to provide a relevant diagnostic approach. PATIENTS AND METHODS: Retrospective study (2001-2011) of 41 patients under the age of 15 who underwent surgery for an ovarian mass with a definite pathological diagnosis. RESULTS: Sixty-two percent of the lesions were benign, 33% were malignant and 5% were borderline. Germ cell tumors were most frequent (77.5%), followed by sex cord stromal tumors (12.5%) and epithelial tumors (7.5%). Malignant tumors were more frequent in children between 0 and 2 years old. On imaging, calcifications and fat were specific for germ cell tumors; the presence of a mural nodule was predictive of a mature teratoma (P<0.001). Predictive factors for malignancy were clinical, including abdominal distension (P<0.01) or a palpable mass (P=0.05), biological, including increased hCG and/or AFP levels (P<0.001) and radiological, including tumors larger than 12 cm (P<0.05), tumoral hypervascularity (P<0.01) and voluminous ascites (P<0.01). CONCLUSION: This semiological analysis confirms the role of imaging in diagnosing the etiology of ovarian lesions in children and adolescents and emphasizes the importance identifying tumoral hypervascularity, which, in addition to classic criteria, is highly predictive of malignancy.

Postshunt encephalopathy in liver transplanted children with portal vein thrombosis.

BACKGROUND: Surgical portosystemic shunting has been reported to alleviate successfully portal hypertension in liver transplanted recipients with portal vein thrombosis. METHODS: We report two liver transplanted children with portal vein thrombosis who developed post-shunt acute encephalopathy. In one child, a mesocaval H-type shunt was created surgically because of bleeding related to Roux-en-Y loop varices at 3 months posttransplantation; in the other, a large spontaneous splenorenal shunt was discovered at the time of diagnosis of portal vein thrombosis on day 34 posttransplantation and was preserved. RESULTS: Post-shunt encephalopathy developed 6 months and 2.7 years after transplantation, causing death in one child. CONCLUSIONS: This report illustrates the risk and the possible dismal outcome of post-shunt encephalopathy in liver transplanted children. Therapeutic procedures other than portosystemic shunting that will restore an hepatopetal portal flow to the liver graft should be considered in liver-transplanted children with portal vein thrombosis.

Central nervous system lesions in hypomelanosis of Ito: an MRI and pathological study.

A severe form of hypomelanosis of Ito is reported, which presented as fetal macrocephaly and neonatal epileptic encephalopathy. Lymphocyte karyotypes were normal. MRI showed an absence of delineation between cortical grey matter and white matter. The prominent neuropathological finding was an abnormal cortical morphogenesis, with the co-existence of cells migrating normally and cells exhibiting arrêt en route or even the complete absence of migration. Intense astrocytic reaction with moderate dystrophic features was present. Juxtaposition of two migration behaviours in the neural cells paralleled the cutaneous findings and reinforced the hypothesis of a genetic chimerism.

Spontaneous perforation of the biliary tract in infancy: a series of 11 cases.

Eleven patients presenting with spontaneous perforation of the biliary tract were treated at Bicêtre Hospital between 1971 and 1993. Three groups were individualised, each with a different pattern of local presentation: generalised biliary peritonitis (n = 2), localised biliary peritonitis (n = 4), secondary biliary stenosis (n = 5). In each case, cholestatic jaundice developed after a postnatal symptom-free interval. Ten patients were operated on. Perforation was located in the cystic duct (n = 2), at the junction of the cystic and hepatic ducts (n = 4), in the common hepatic duct (n = 1) or common bile duct (n = 1). The site of perforation was no longer identifiable in two cases with stenosis. A cholecystectomy was performed in the 2 cases with cystic duct perforation; in the cases of lesions of the main duct, either simple external biliary drainage (n = 3) or biliary reconstruction (n = 5) was carried out. Postoperative complications included bile leak (n = 2), ascending cholangitis (n = 1), portal vein thrombosis (n = 2). Five patients were submitted to further surgery including biliary revision (n = 3), porto-systemic shunt (n = 1), and other procedures (n = 2). One infant died from postoperative sepsis; 2 were lost to follow-up, one of which probably did not survive; 4 are alive and well. Late sequelae are present in 4 children: portal hypertension (n = 1), mild residual bile duct dilatation without cholestasis (n = 1), and mild to moderate liver fibrosis (n = 2). Prompt diagnosis and appropriate treatment should improve the prognosis of this rare condition.

[Vascular malformations of the cecum: treatment by embolization. Apropos of 2 cases]. / Malformations vasculaires du caecum: traitement par embolisation. A propos de deux cas.

Two cases of arteriovenous malformations of the cecum treated by transcatheter embolization are presented. In both cases, treatment resulted in improvement of the clinical and biological signs. In general, there is a high risk of ischemic complications associated with this treatment. Embolization of arteriovenous malformations should be considered rarely and in the following cases: single lesion, high blood output, feasibility of superselective catheterization. The risk of colonic ischemia calls for preparation of the colon as if the patient were undergoing colonic surgery, as well as thorough clinical follow-up (for at least 10 days).

Fatal hemobilia after liver graft biopsy in a transplanted child.

Severe hemobilia after liver transplantation is a rare complication. We report one case of fatal hemobilia resulting from arterio-biliary fistula after a liver graft needle biopsy in a 7-year-old girl transplanted with a reduced-size graft. Arterial reconstruction was performed by an iliac conduit implanted in the aorta. The diagnosis of hemobilia was made by angiography but the hemorrhage could not be controlled by selective embolization of the bleeding arterial branch. The patient died from early and massive recurrent bleeding before surgery. The prevention of iatrogenic hemobilia and current therapeutic strategies are discussed.

[Pseudotumor nodules of the liver in cirrhotic patients. Study of 7 cases]. / Nodules pseudo-tumoraux du foie chez le cirrhotique. Etude de 7 cas.

Hepatocellular pseudotumor (HCP) occurs in cirrhotic liver and can resemble hepatocellular carcinoma. Liver ultrasonography shows a space-occupying lesion. The aim of this study was to describe the clinical, radiological and histologic features of HCP based on seven patients (3 women, 4 men), mean age 48 years (24 to 62), with histologically proven cirrhosis (alcoholic, 4 cases; autoimmune, 1 case; postnecrotic, 1 case, idiopathic, 1 case). Serum alphafetoprotein was below 16 ng/ml in 5 patients and remained over 120 ng/ml in the remaining 2. Desgammacarboxyprothrombin, performed in 3 cases, was below the upper limit of normal range. Real time ultrasonography of the liver showed a homogeneous parenchyma in 1 case and median size (20-48 mm) space-occupying nodular lesions in 6 cases. Sonography patterns of hepatocellular pseudotumor were as follows: anechoic lesions in 5 cases and mixed pattern (sonodense and hypoechogenic) in 1 case. Angiographic findings exhibited different patterns: hypervascular or hypovascular nodules, multinodular uptake after lipiodol bolus injection. Computed tomography showed iso- or hypodense space-occupying lesions. Lipiodol injection, performed in 3 cases, showed nodular lipiodol uptake. Fine needle biopsy always showed normal hepatocytes. At laparotomy, performed in 3 cases, an hyperplastic nodule was found in 1 case only. All patients were alive at 12 to 36 months. These findings are consistent with the fact that hepatocellular pseudotumor is a true entity. Differential diagnosis is difficult. Iodine oil nodular fixation on CT scan may be non specific for hepatocellular pseudotumor. Histologic data is mandatory before beginning a non surgical therapeutic regimen for suspected hepatocellular carcinoma.

[Sonography of the pediatric abdomen: liver and biliary tract]. / Echographie abdominale de l'enfant: foie et voies biliaires.

Ultrasound is a routine imaging modality that is quite valuable to assess the pediatric abdomen. The objective of this article is to describe the sonographic appearance of the liver and bile ducts in children. Our goal is to emphasize normal variants and specific pediatric diseases, even if some of them may be encountered in adults.

[Ultrasound and portal hypertension in children. Demonstration of gastro-oesophageal collaterals. Preliminary report (author's transl)]. / L'échotomographie dans l'hypertension portale de l'enfant. Etude des dérivations gastro-oesophagiennes. Note préliminaire.

The authors studied the value of ultrasonography in demonstrating oesophageal collaterals in portal hypertension in childhood. 22 children with portal hypertension underwent successively an ultrasound examination and an endoscopy looking for submucosal oesophageal varices. Oesophageal collaterals were said to be present when ultrasound showed vascular structures in the gastro-hepatic ligament above the coeliac trunk origin. Among those 22 patient 18 had a satisfactory ultrasound examination. 12 true positive, 3 true negative, 3 false negative ultrasound examinations were obtained. 4 ultrasound studies were inconclusive. The value of ultrasound in the pre and post surgical check-up of portal hypertension in children is emphasized.

[Acute gastric dilatation with superior mesenteric artery syndrome in a young girl with anorexia nervosa]. / Dilatation aiguë de l'estomac avec pince mésentérique chez une jeune fille anorexique.

BACKGROUND: Acute gastric dilatation is a rare complication of anorexia nervosa which may be fatal in case of gastric perforation. Superior mesenteric artery syndrome may be associated with gastric dilatation. CASE REPORT: A 14 year-old girl was admitted suffering from severe anorexia nervosa. Shortly after admission, she complained of abdominal pain related to a bulimic episode a few days before, followed by nausea and vomiting. Distended abdomen and tachycardia suggested acute small-bowel obstruction. A diagnosis of acute gastric dilatation with superior mesenteric artery syndrome was suspected on X-ray examination and confirmed by esophagogastrography after gastric evacuation. The patient improved rapidly under total parenteral nutrition. Upper gastrointestinal study repeated 10 days later showed normal stomach. CONCLUSIONS: Small-bowel obstruction in anorexia nervosa first evokes acute gastric dilatation, and a perforation has to be ruled out by esophagogastrography. Association with superior mesenteric artery syndrome is possible; it does not necessarily lead to surgery.