Meso-Rex bypass for extrahepatic portal vein obstruction in children.

BACKGROUND: Meso-Rex bypass (MRB) and portosystemic surgical shunt (PSS) are both used to treat extrahepatic portal vein obstruction (EHPVO) in children. The aim of this study was to analyse the outcome of MRB and PSS to select patients who could benefit from a prophylactic MRB. METHODS: This single-centre retrospective study of children who underwent either MRB or PSS for EHPVO was conducted between 1996 and 2010. Details of patient demographics and preoperative evaluation were collected. Success rates, defined as shunt patency after a minimum of 6 months and clearance of varices or symptoms, were compared. Determinants of outcomes were explored. RESULTS: Sixty-nine patients underwent a MRB or PSS. Median (interquartile range, i.q.r.) age at surgery was 6.6 (4.0-10.6) years. Twenty-four patients (35 per cent) had had a neonatal umbilical catheter (NUC) placed previously and 47 (68 per cent) had experienced an upper gastrointestinal bleed. Imaging assessment of the intrahepatic left portal vein was considered favourable in 40 patients. Of 43 MRBs attempted, 11 failed during surgery and four patients had persistent thrombosis after a median of 55 (i.q.r. 18-107) months. The success rate of MRB was 60 per cent (26 of 43) compared with 100 per cent (26 of 26) for PSS (P < 0.001). It was lower among patients in whom a NUC had been used (2 of 10 versus 24 of 33; P = 0.004), for procedures undertaken early in the series (6 of 16 versus 20 of 27; P = 0.020) and when the imaging pattern was unfavourable (0 of 5 versus 26 of 38; P = 0.006). On multivariable analysis, only a previous history of NUC predicted failure (P = 0.016). CONCLUSION: Prophylactic MRB seems a good treatment option for EHPVO in children, but should be done only by an experienced team in patients with favourable imaging and without a previous history of NUC.

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.

Pancreatic resections for solid or cystic pancreatic masses in children.

OBJECTIVES: The aim of the study was to assess the diagnosis and management of solid pancreatic neoplasm in children and the type of surgical treatment, focusing on short- and long-term outcomes. METHODS: We retrospectively reviewed the charts of all children who had undergone pancreatic resection for suspicion of pancreatic tumor in Kremlin Bicêtre Hospital, Paris, between 1986 and 2008. We studied the symptoms at diagnosis, the type of surgery, and the short- and long-term morbidity and mortality. RESULTS: Of 18 patients identified, there were 7 pseudopapillary tumors, 3 neuroblastomas, 2 rhabdomyosarcomas, 1 acinar cell carcinoma, 1 endocrine cell carcinoma, 1 renal angiomyolipoma, and 3 pancreatic cysts. Symptoms at diagnosis were abdominal trauma, abdominal mass, and jaundice. Operative procedures were duodenopancreatectomy (11), mid-pancreatic resections (2), splenopancreatectomy (2), distal pancreatectomy (1), and tumorectomy (2). There were no deaths related to surgery. The postoperative morbidity rate was 45%, including 2 cases of fistula (11%) occurring after a mid-pancreatic resection and a pancreaticoduodenectomy. The median follow-up was 4.2 years (range 2-11). There was no diabetes mellitus, but there was 1 case of fat diet intolerance requiring pancreatic enzyme substitution. All of the children had a growth curve within normal limits. CONCLUSIONS: In this experience, pancreatic resections have proven to be a safe and efficient procedure, with low long-term morbidity, for the treatment of tumoral and selected nontumoral pancreatic masses.

Hepatic hydrothorax in a child and its management.

Hepatic hydrothorax is a rare complication of portal hypertension. The optimal treatment for this condition is liver transplantation. Liver transplantation is significantly more manageable in children who weigh more than 8 kg. Here, an implantable pleural access device was used in a 5-month-old infant for painless iterative punctures to relieve respiratory symptoms, while waiting for liver transplantation and the patient's growth. The patient underwent successful transplantation 3 months later with a more optimal weight.

[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.

Imaging features of atypical bleeds in young patients with hemophilia.

Hemarthroses and muscle bleeds are well-known and well-documented complications in pediatric and young adult hemophilia patients. In contrast, deep bleeds in atypical locations can be a diagnostic challenge, since clinicians and radiologists are often unfamiliar with their clinical and radiological features. Some atypical bleeds, however, can be life-threatening or severely disabling, highlighting the need for prompt, accurate diagnosis. Rare bleeds include central nervous system bleeds (including intracranial and spinal hematomas), urogenital bleeds, intra-abdominal bleeds (mesenteric and gastrointestinal wall hematomas) and pseudo tumors in unusual locations like the sinonasal cavities. Because clinical assessment can be difficult, clinicians and radiologists should be aware of the possibility of these rare complications in their hemophilia patients, so that they can avoid unnecessary invasive diagnostic and surgical procedures and institute prompt, appropriate treatment. The purpose of this review is to illustrate the imaging features of bleeds that occur in rare locations in young (i.e., children and young adults) patients with hemophilia to make the reader more familiar with these conditions.

[Retroperitonal fibrosis and lymphoma in a 15-year-old boy]. / Fibrose rétropéritonéale et lymphome chez un enfant de 15ans.

Retroperitoneal fibrosis (RF) is a rare disease in children. We report the case of a 15-year-old boy who presented with a 2-month history of dorsal pain and a 2-week history of fever. The erythrocyte sedimentation rate and serum C-reactive protein value were high. Magnetic resonance imaging revealed a large heterogeneous retroperitoneal mass, suggestive of RF. Percutaneous biopsy of inguinal lymph node provided the diagnosis of anaplastic large cell lymphoma. This is the first report of RF revealing malignancy in childhood. It emphasizes that malignancy must be carefully searched for in children with unexplained RF.

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.

[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.

[Cyclic filling cystourethrography in the study of febrile urinary tract infection in children]. / Cystographie à remplissages répétés dans l'exploration des infections urinaires fébriles chez l'enfant.

PURPOSE: To assess the value of cyclic voiding cystourethrography (VCUG) for the detection of reflux in a large population of children with urinary tract infection. MATERIALS AND METHODS: 234 patients (67% less than 3 years of age) underwent VCUG with two fillings in 214 cases and three fillings in 139 cases. Appearance or increase in the grade of reflux compared with previous filling was expressed as "modification of the radiology report" (MRR). The amount of contrast material, the duration of fluoroscopy and the number of films were recorded. RESULTS: The rate of reflux was 18.4% at the first filling, 16% at the second filling with 9.8% MRR and 14.7% at the third filling with 10% MRR. These results were the same for children younger and older than 3 years. For children under three years, if one considered reflux grade higher than 1, the MRR was 9.6% at the second filling and 7.5% at the third while it was 5.8% and 3% for older children. On average, the use of contrast material increased 50% with a third filling, number of films was not modified and the time of fluoroscopy increased by 6 sec per filling. CONCLUSION: VCUG is recommended in all children. The detection of reflux higher than grade 1 is more frequent with multiple fillings in children under 3 years. The increase in radiation exposure and cost seems negligible.

Ultrasound elastography: advantages, limitations and artefacts of the different techniques from a study on a phantom.

Ultrasound elastography is a technique currently under development. Its use in clinical practice is complicated because of the wide range of techniques used by the different manufacturers and the parameters proposed to characterise tissues. A comparative analysis on five ultrasound diagnostic systems has been performed on a calibrated elasticity phantom and demonstrated that: (1) all systems tested are reliable for simple qualitative analysis: is a nodule present and is it harder or softer than neighbouring tissues? (2) the deformation or hardness ratios between two regions are usually, however, not proportional to the theoretical ratios and only a binary analysis greater than 1 (harder) and less than 1 (softer) is reliable and could be used as a negative predictive value (NPV) for malignant lesions, as has been suggested by some authors; (3) finally, quantitative analysis using shear wave techniques performed variably, reliable measurements being obtained with only one of the systems. Measurements produced by these different systems must not be compared in clinical practice to monitor a patient and the threshold values proposed in the literature must only be used in an analysis carried out with the same system and same probe.