ABSTRACT
BACKGROUND: The technique of « en bloc¼ liver and small bowel transplantation (L-BT) spares a biliary anastomosis, but does not protect against biliary complications. We analyze biliary and duodenal complications (BDC) in our pediatric series. METHODS: Between 1994 and 2020, 54 L-BT were performed in 53 children. The procurement technique included in situ vascular dissection and pancreatic reduction to the head until 2009 (group A). Thereafter, the whole pancreas was recovered (group B). RESULTS: Nine BDCs occurred in 8/53 (15%) patients (7 in group A and 1 in group B): leak of the donor's duodenal stump (2), stenosis of the extra-pancreatic bile duct (5), and intra-pancreatic bile duct stenosis (2). Median delay for diagnosis of stricture was 8 months (4-168). Interventional radiology was successful in one child only, the others required reoperations. Two patients died, of biliary cirrhosis or cholangitis, 15-month and 12-year post-L-BT. One was listed and liver re-transplanted 13 years post-L-BT. At last follow-up, two patients only had normal liver tests and ultrasound. CONCLUSION: BDC after L-BT can cause severe morbidities. Pancreatic reduction might increase this risk. Early surgical complications or chronic pancreatic rejection might be co-factors. Early diagnosis and treatment are key to the long-term prognosis.
Subject(s)
Biliary Tract Diseases/epidemiology , Duodenal Diseases/epidemiology , Intestine, Small/transplantation , Liver Transplantation , Postoperative Complications/epidemiology , Adolescent , Child , Child, Preschool , Female , France/epidemiology , Humans , Infant , Male , Pancreas/surgery , Retrospective StudiesABSTRACT
BACKGROUND: Hepatoblastoma tumor rupture is a high-risk criterion in the SIOPEL 3/4 protocol. Little is known about the outcome of these children. METHODS: Radiological signs of possible tumor rupture, defined as peritoneal effusion, peritoneal nodules, or hepatic subcapsular hematoma, were reported in 24 of 150 patients treated for hepatoblastoma in France from January 2000 to December 2014 after central radiological expert review. RESULTS: Twenty-two patients with available clinical data were included (nine PRETEXT-I/II, six PRETEXT-III, seven PRETEXT-IV, and five had lung metastases). Five patients had a subcapsular hematoma only, and 17 patients had intraperitoneal rupture (subcapsular hematoma and peritoneal effusion). A hepatic biopsy was performed in 19 patients. Intraperitoneal rupture occurred before biopsy in 12 and after biopsy in three (including one with prebiopsy subcapsular hematoma) (missing data: two). All patients were treated with chemotherapy, with high-risk regimens including cisplatin or carboplatin and doxorubicin in 19 and cisplatin or carboplatin alone in three. Liver surgery was performed in 20 patients (including three liver transplants). Fifteen patients (68%) achieved complete remission. With a median follow-up of 5.5 years, 11 events occurred (six progressions and three relapses, including three peritoneal progressions/relapses, one surgical complication, and one second cancer) and eight patients died. One of eight patients with no other high-risk criterion had a relapse. The three-year event-free survival and overall survival rates were 49.6% (95% CI = 30-69) and 68.2% (40-84), respectively. CONCLUSIONS: Tumor rupture is predictive of poor prognosis with risk of peritoneal progression/relapse. However, it should not be a contraindication for liver transplantation.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Hepatoblastoma/physiopathology , Liver Neoplasms/physiopathology , Rupture, Spontaneous/drug therapy , Adolescent , Carboplatin/administration & dosage , Child , Child, Preschool , Cisplatin/administration & dosage , Doxorubicin/administration & dosage , Female , Follow-Up Studies , France/epidemiology , Humans , Incidence , Infant , Male , Prognosis , Retrospective Studies , Risk Factors , Rupture, Spontaneous/epidemiology , Rupture, Spontaneous/pathology , Survival RateABSTRACT
BACKGROUND: Liver-transplanted, immunosuppressed pediatric patients undergoing repeated percutaneous transhepatic cholangiography (PTC) require optimized exposure to ionizing radiation. OBJECTIVE: To establish local diagnostic reference levels (DRL) for pediatric PTC and investigate the routine use of X-ray equipment. MATERIALS AND METHODS: The study retrospectively analyzed data collected between October 2016 and June 2018 from a single center performing PTC. We collected exposure parameters including kerma area product (PKA), air kerma at patient entrance reference point (Ka,r) and fluoroscopy time via a dose archiving and communication system. Local diagnostic reference levels were derived as the 50th percentile of the distributions while considering published recommended weight groups. We investigated exposure variability with procedure complexity and with technical parameters recovered from the radiation dose structured report. RESULTS: The analysis included 162 PTC procedures performed in 64 children: 58% male, average age 6 years (range 39 days to 16 years) and weight 24 kg (range 3-60 kg). Local DRLs for weight groups 0-5 kg, 5-15 kg, 15-30 kg, 30-50 kg and 50-80 kg were, respectively, 6 cGy.cm2, 22 cGy.cm2, 68 cGy.cm2, 107 cGy.cm2 and 179 cGy.cm2 in PKA. Local DRLs per weight group were also established for intermediate and complex procedures. Radiation dose structured report analysis highlighted good local practice with efficient collimation, low fluoroscopy pulse rate, no magnification and limited use of radiographic acquisitions. Meanwhile, table and detector positioning and tube projection could still be optimized. PKA correlated significantly with the number of acquisitions and tube-to-table distance. CONCLUSION: We established local DRLs for children undergoing PTC.
Subject(s)
Cholangiography/methods , Cholestasis/diagnostic imaging , Liver Transplantation , Postoperative Complications/diagnostic imaging , Adolescent , Child , Child, Preschool , Female , Fluoroscopy , Humans , Immunocompromised Host , Infant , Male , Reference Values , Retrospective StudiesABSTRACT
INTRODUCTION: The use of interventional radiology (IR) in the treatment of pediatric solid tumors has markedly increased over the last three decades. However, data on effectiveness of IR-techniques, such as embolization/ablation, are scarce. In this systematic review and meta-analysis, we examined the outcomes of IR-procedures in the treatment of solid tumors in children. MATERIALS AND METHODS: Using a defined search strategy, we searched for studies reporting the use of IR-techniques for pediatric solid tumors from 1980 to 2017. Reports with less than three patients, review, and opinion articles were excluded. The study was conducted under preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. We analyzed dichotomous and continuous variables by appropriate statistical methods. RESULTS: Of 567 articles screened, 21 papers met the inclusion criteria (12 retrospective, 7 prospective, and 2 randomized-control trials). Many of the analyzed papers described relatively small cohorts of patients. IR-guided procedures were mainly rescue procedures to treat primarily unresectable tumors, local recurrences, or metastases. Inclusion/exclusion criteria and success definition were not specified in most reports. Major side effects were documented in 17/286 (6%) infants, while minor side effects were self-limiting in most patients. Six studies had a comparison between tumor embolization (127 infants) to surgery or chemotherapy without IR-procedures (113 controls). The meta-analysis showed lower mortality (16 vs. 47%) and surgical time for resection (206 vs. 250 m), higher 2-year tumor-free survival (82 vs. 36%), and favorable histology in IR group (p < 0.001 for all). CONCLUSION: IR-guided techniques are promising in the treatment of pediatric solid tumors. Further prospective (randomized) trials are needed to clarify efficacy.
Subject(s)
Neoplasms/diagnostic imaging , Neoplasms/therapy , Radiology, Interventional , Child , Humans , Neoplasms/mortality , Survival Analysis , Treatment OutcomeSubject(s)
Echocardiography , Heart Diseases/etiology , Heart/diagnostic imaging , Liver Diseases/complications , Lung Diseases/etiology , Lung/diagnostic imaging , Magnetic Resonance Imaging , Radionuclide Imaging , Tomography, X-Ray Computed , Coronary Circulation , Female , Heart/physiopathology , Heart Diseases/diagnostic imaging , Heart Diseases/physiopathology , Hemodynamics , Humans , Liver Circulation , Liver Diseases/diagnosis , Liver Diseases/physiopathology , Lung/physiopathology , Lung Diseases/diagnostic imaging , Lung Diseases/physiopathology , Male , Middle Aged , Predictive Value of Tests , Prognosis , Pulmonary Circulation , Respiration , Risk FactorsABSTRACT
BACKGROUND: Regenerative medicine using stem cell technology is an emerging field that is currently tested for inborn and acquired liver diseases. OBJECTIVE: This phase I/II prospective, open label, multicenter, randomized trial aimed primarily at evaluating the safety of Heterologous Human Adult Liver-derived Progenitor Cells (HepaStem) in pediatric patients with urea cycle disorders (UCDs) or Crigler-Najjar (CN) syndrome 6 months posttransplantation. The secondary objective included the assessment of safety up to 12 months postinfusion and of preliminary efficacy. METHODS: Fourteen patients with UCDs and 6 with CN syndrome were divided into 3 cohorts by body weight and intraportally infused with 3 doses of HepaStem. Clinical status, portal vein hemodynamics, morphology of the liver, de novo detection of circulating anti-human leukocyte antigen antibodies, and clinically significant adverse events (AEs) and serious adverse events to infusion were evaluated by using an intent-to-treat analysis. RESULTS: The overall safety of HepaStem was confirmed. For the entire study period, patient-month incidence rate was 1.76 for the AEs and 0.21 for the serious adverse events, of which 38% occurred within 1 month postinfusion. There was a trend of higher events in UCD as compared with CN patients. Segmental left portal vein thrombosis occurred in 1 patient and intraluminal local transient thrombus in a second patient. The other AEs were in line with expectations for catheter placement, cell infusion, concomitant medications, age, and underlying diseases. CONCLUSIONS: This study led to European clinical trial authorization for a phase II study in a homogeneous patient cohort, with repeated infusions and intermediate doses.
Subject(s)
Crigler-Najjar Syndrome/drug therapy , Liver Transplantation , Liver/metabolism , Stem Cell Transplantation , Urea Cycle Disorders, Inborn/surgery , Adolescent , Age Factors , Child , Child, Preschool , Crigler-Najjar Syndrome/blood , Crigler-Najjar Syndrome/diagnosis , Crigler-Najjar Syndrome/physiopathology , Europe , Female , Humans , Infant , Liver/pathology , Liver/physiopathology , Liver Regeneration , Liver Transplantation/adverse effects , Male , Prospective Studies , Stem Cell Transplantation/adverse effects , Time Factors , Transplantation, Heterologous , Treatment Outcome , Urea Cycle Disorders, Inborn/blood , Urea Cycle Disorders, Inborn/diagnosis , Urea Cycle Disorders, Inborn/physiopathologyABSTRACT
Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.
Subject(s)
Diagnostic Imaging/methods , Portal Vein/abnormalities , Vascular Malformations/diagnostic imaging , Vascular Malformations/therapy , Humans , Portal Vein/diagnostic imaging , Portal Vein/surgery , Vascular Malformations/surgeryABSTRACT
BACKGROUND: Hepatic venous outflow obstruction after paediatric liver transplantation is an unusual but critical complication. OBJECTIVES: To review the incidence, diagnosis and therapeutic modalities of hepatic venous outflow obstruction from a large national liver transplant unit. MATERIALS AND METHODS: During the period from October 1992 to March 2016, 917 liver transplant procedures were performed with all types of grafts in 792 children. Transplants suspected to have early or delayed venous outflow obstruction were confirmed by percutaneous venography or surgical revision findings. Therapeutic intervention, recurrence and outcome were evaluated. RESULTS: Twenty-six of 792 children (3.3%) experienced post-transplant hepatic venous outflow obstruction. These patients had been diagnosed from 1 day to 8.75 years after transplantation. Six occurred during the early post-transplant period; in three of them, the graft was lost. Seventeen patients were initially treated by balloon angioplasty with success; 11 of these experienced recurrences. Four stents were implanted; one was complicated by definitive occlusion. Three of the five surgical revisions were successful. The initial stenosis involved the inferior vena cava in 10 grafts, in isolation or associated with hepatic vein involvement. Mean follow-up was 79 months after transplantation. Eight grafts were lost. CONCLUSION: Acute postoperative hepatic venous outflow obstruction was associated with poor prognosis. Diagnostic venography should be performed if there is any suspicion of venous outflow obstruction, even if first-line examinations are normal. Stenosis frequently involved the inferior vena cava. Angioplasty was a safe and efficient treatment for venous outflow obstruction despite frequent recurrence.
Subject(s)
Angioplasty, Balloon , Budd-Chiari Syndrome/diagnostic imaging , Budd-Chiari Syndrome/therapy , Liver Transplantation , Postoperative Complications/diagnostic imaging , Postoperative Complications/therapy , Stents , Adolescent , Budd-Chiari Syndrome/epidemiology , Child , Child, Preschool , Female , Graft Rejection/diagnostic imaging , Graft Rejection/epidemiology , Humans , Incidence , Infant , Male , Postoperative Complications/epidemiology , Retrospective StudiesABSTRACT
BACKGROUND: Rhabdoid tumors (RTs) of the liver are rare, aggressive and nonsecreting malignancies occurring mainly during the first year of life. Definition of RT relies on characteristic morphology and on the inactivation of the SMARCB1 tumor suppressor gene. The aim of this study was to analyze clinical data, treatments and outcomes in our patients. PATIENTS AND METHODS: 6 cases of patients treated in our institution for RT of the liver between January 2007 and January 2015 are reported. Variables examined included age at diagnosis, tumor stage, treatment and long-term survival. RESULTS: Median age at diagnosis was 5months (range: 4-23). Normal for age serum AFP levels was observed in all patients. No patient presented with metastasis at diagnosis. The diagnosis of RT based on the loss of SMARCB1 was made early in 4 patients. The 2 others were initially diagnosed as nonsecreting hepatoblastomas. Median follow-up was 6years (range: 2-9). All patients received chemotherapy, with variable regimens depending on initial diagnosis, followed by surgical resection. Three patients (50%) died of disease. Two of them were mistaken for nonsecreting hepatoblastomas at diagnosis and had recurrence shortly after completion of treatment. The third one presented a cardiac right atrium thrombus. Three patients (50%) are long-term survivors; they received multimodal therapy including chemotherapy according to protocol EpSSG NRSTS consisting of doxorubicin and surgical removal of the tumor performed within 3months after diagnosis. One patient had adjuvant radiotherapy. CONCLUSION: According to our results, search of SMARCB1 mutation or alternatively immunohistochemical assay for SMARCB1 in nonsecreting hepatoblastomas is mandatory to exclude RT. Chemotherapy according to EpSSG NRSTS protocol together with a surgical treatment seems justified to improve long-term survival. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level IV.
Subject(s)
Liver Neoplasms/diagnosis , Liver Neoplasms/therapy , Rhabdoid Tumor/diagnosis , Rhabdoid Tumor/therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Female , Humans , Infant , Liver/surgery , Liver Neoplasms/genetics , Male , Mutation , Neoplasm Recurrence, Local , Radiotherapy, Adjuvant , Retrospective Studies , Rhabdoid Tumor/genetics , SMARCB1 Protein/geneticsABSTRACT
OBJECTIVES: Angiogenic defects secondary to gene mutations of JAG1 and NOTCH2, causing arterial anomalies in Alagille syndrome (AGS), are well described in the literature. The study analyzes the frequency of abdominal arterial anomalies in children with AGS with an emphasis on outcomes following liver transplantation (LT). METHODS: Between 1988 and 2013, 242 children with AGS were treated at our institution. We performed a retrospective analysis of 55 who underwent LT during the study period. Preoperative abdominal arterial findings, operative reports, arterial reconstruction technique, and early as well as late complications following LT were reviewed specifically focusing on arterial thrombosis. RESULTS: Twenty-five patients had preoperative imaging available for analysis. Twelve of these patients showed celiac trunk stenosis (48.0%), 2, a superior mesenteric artery stenosis (8.0%) and one a stenosis of both renal arteries. Twenty patients (36.3%) underwent standard hepatic reconstruction using the native recipient hepatic artery. Thirty-five patients (63.7%) underwent aortic conduit reconstruction (ACR) from the infrarenal aorta using donor arterial conduits. Hepatic artery thrombosis occurred in 9 patients (16.3%). This number was higher in the standard arterial anastomosis group 7/20 (35.0%) than in those with ACR 2/35 (5.7%, Pâ=â0.0079). CONCLUSIONS: In this series, children with AGS pretransplant have a high prevalence of abdominal arterial anomalies. Preoperative abdominal vascular imaging makes it possible to anticipate whether or not a classical arterial revascularization can be performed or whether an ACR is required.
Subject(s)
Alagille Syndrome , Celiac Artery/abnormalities , Liver Transplantation , Mesenteric Artery, Superior/abnormalities , Renal Artery/abnormalities , Vascular Malformations , Adolescent , Alagille Syndrome/complications , Alagille Syndrome/diagnosis , Alagille Syndrome/epidemiology , Alagille Syndrome/surgery , Arterial Occlusive Diseases/diagnosis , Arterial Occlusive Diseases/epidemiology , Arterial Occlusive Diseases/etiology , Arterial Occlusive Diseases/surgery , Celiac Artery/surgery , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Mesenteric Artery, Superior/surgery , Prevalence , Renal Artery/surgery , Retrospective Studies , Treatment Outcome , Vascular Malformations/complications , Vascular Malformations/diagnosis , Vascular Malformations/epidemiology , Vascular Malformations/surgeryABSTRACT
OBJECTIVES: To describe and evaluate an additional sonographic sign in the diagnosis of biliary atresia (BA), the microcyst of the porta hepatis, in comparison with previously described signs. METHODS: Ultrasound performed in 321 infants (mean age 55 days) with cholestasis were retrospectively analyzed. BA was surgically confirmed in 193 patients and excluded in 128. US evaluated gallbladder type (1: normal; 2: consistent with BA; 3: suspicious), triangular cord sign (TCS), microcyst and macrocyst, polysplenia syndrome, portal hypertension, and bile duct dilatation. T test and Pearson χ2 test were used to compare US signs between the two groups, followed by univariate regression analysis. RESULTS: The highest specificity and sensitivity for BA (p < 0.001) were respectively obtained with non-visible gallbladder (100 %-13 %), macrocyst (99 %-10 %), polysplenia (99 %-11 %), microcyst (98 %-20 %), type 2 gallbladder (98 %-34 %), and TCS (97 %-30 %). Combination of signs (macro or microcyst; cyst and no bile duct dilatation; microcyst and/or TCS; type 2 gallbladder and/or cyst) provided better sensitivities (25-49 %) with similar specificities (95-98 %) (p < 0.001). On univariate analysis, the single US signs most strongly associated with BA were polysplenia (odds ratio, OR 16.3), macrocyst (OR 14.7), TCS (OR 13.4) and microcyst (OR 8). CONCLUSIONS: Porta hepatis microcyst is a reliable US sign for BA diagnosis. KEY POINTS: ⢠The porta hepatis microcyst is a specific sign of biliary atresia. ⢠It was found in 31 (16.1 %) of 193 patients with biliary atresia. ⢠Its specificity was 98 % (p < 0.001). ⢠High frequency transducer and color Doppler can show the porta hepatis microcyst.
Subject(s)
Bile Duct Diseases/diagnostic imaging , Biliary Atresia/diagnostic imaging , Cholestasis/diagnostic imaging , Cysts/diagnostic imaging , Gallbladder/diagnostic imaging , Heterotaxy Syndrome/diagnostic imaging , Hypertension, Portal/diagnostic imaging , Liver/diagnostic imaging , Bile Duct Diseases/complications , Biliary Atresia/complications , Cholestasis/complications , Cysts/complications , Dilatation, Pathologic/complications , Dilatation, Pathologic/diagnostic imaging , Female , Heterotaxy Syndrome/complications , Humans , Hypertension, Portal/complications , Infant , Infant, Newborn , Male , Retrospective Studies , Sensitivity and Specificity , UltrasonographyABSTRACT
BACKGROUND: Undifferentiated embryonal sarcoma of the liver is a rare malignant mesenchymal tumour occurring mostly in children ages 6-10 years. The discrepancy between its solid appearance on US and cystic-like appearance on CT has been described. OBJECTIVE: To study the imaging particularities and similarities among our cases of undifferentiated embryonal sarcoma and to report the errors in initial diagnoses. MATERIALS AND METHODS: We conducted a retrospective study of 15 children with undifferentiated embryonal sarcoma diagnosed or referred to our hospital during 1997-2015 and analysed the clinical, biological and imaging data. RESULTS: We identified eight boys and seven girls ages 9 months to 14 years. Ten children presented with abdominal pain. Alpha-fetoprotein was slightly increased in one. Initial US and CT had been performed for all, while additional MRI had been done in two children. Initial CT demonstrated a hypoattenuated mass in all. Rupture was seen in five and intratumoural bleeding in seven children. Tumour volumes reduced during neoadjuvant chemotherapy in 10 children. CONCLUSION: Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.
Subject(s)
Liver Neoplasms/diagnostic imaging , Magnetic Resonance Imaging , Neoplasms, Germ Cell and Embryonal/diagnostic imaging , Sarcoma/diagnostic imaging , Tomography, X-Ray Computed , Ultrasonography , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Liver/diagnostic imaging , Male , Retrospective StudiesABSTRACT
Neonatal sclerosing cholangitis (NSC) is a rare biliary disease leading to liver transplantation in childhood. Patients with NSC and ichtyosis have already been identified with a CLDN1 mutation, encoding a tight-junction protein. However, for the majority of patients, the molecular basis of NSC remains unknown. We identified biallelic missense mutations or in-frame deletion in DCDC2 in four affected children. Mutations involve highly conserved amino acids in the doublecortin domains of the protein. In cholangiocytes, DCDC2 protein is normally located in the cytoplasm and cilia, whereas in patients the mutated protein is accumulated in the cytoplasm, absent from cilia, and associated with ciliogenesis defect. This is the first report of DCDC2 mutations in NSC. This data expands the molecular spectrum of NSC, that can be considered as a ciliopathy and also expands the clinical spectrum of the DCDC2 mutations, previously reported in dyslexia, deafness, and nephronophtisis.
Subject(s)
Cholangitis, Sclerosing/genetics , Cilia/metabolism , Microtubule-Associated Proteins/genetics , Microtubule-Associated Proteins/metabolism , Mutation , Cholangitis, Sclerosing/metabolism , Cytoplasm/metabolism , Female , Humans , Male , Microtubule-Associated Proteins/chemistry , Mutation, Missense , Pedigree , Protein Domains , Sequence DeletionABSTRACT
PURPOSE: To evaluate the feasibility of using supersonic shear-wave elastography (SSWE) in children and normal values of liver stiffness with the use of control patients of different ages (from neonates to teenagers) and the diagnostic accuracy of supersonic shear wave elastography for assessing liver fibrosis by using the histologic scoring system as the reference method in patients with liver disease, with a special concern for early stages of fibrosis. MATERIALS AND METHODS: The institutional review board approved this prospective study. Informed consent was obtained from parents and children older than 7 years. First, 51 healthy children (from neonate to 15 years) were analyzed as the control group, and univariate and multivariate comparisons were performed to study the effect of age, transducer, breathing condition, probe, and position on elasticity values. Next, 45 children (from 1 month to 17.2 years old) who underwent liver biopsy were analyzed. SSWE measurements were obtained in the same region of the liver as the biopsy specimens. Biopsy specimens were reviewed in a blinded manner by a pathologist with the use of METAVIR criteria. The areas under the receiver operating characteristics curve (AUCs) were calculated for patients with fibrosis stage F0 versus those with stage F1-F2, F2 or higher, F3 or higher, and F4 or higher. RESULTS: A successful rate of SSWE measurement was 100% in 96 patients, including neonates. Liver stiffness values were significantly higher when an SC6-1 probe (Aixplorer; SuperSonic Imagine SA, Aix-enProvence, France) was used than when an SL15-4 probe (Aixplorer) was used (mean ± standard deviation, 6.94 kPa ± 1.42 vs 5.96 kPa ± 1.31; P = .006). There was no influence of sex, the location of measurement, or respiratory status on liver elasticity values (P = .41-.93), although the power to detect such a difference was low. According to the degree of liver fibrosis at liver biopsy, 88.5%-96.8% of patients were correctly classified, with AUCs of 0.90-0.98 (95% confidence interval [CI]: 0.8, 1.0). The AUC for patients with stage F0 versus stage F1-F2 was 0.93 (95% CI: 0.87, 0.99). CONCLUSION: SSWE allows accurate assessment of liver fibrosis, even in children with early stage (F1-F2) disease, and the choice of transducer influences liver stiffness values.
Subject(s)
Elasticity Imaging Techniques/methods , Liver Cirrhosis/diagnostic imaging , Liver/diagnostic imaging , Adolescent , Biopsy , Child , Child, Preschool , Feasibility Studies , Female , Humans , Infant , Liver/pathology , Liver Cirrhosis/pathology , Male , Prospective StudiesSubject(s)
Liver Neoplasms/genetics , Oncogene Proteins, Fusion/genetics , Proto-Oncogene Protein c-fli-1/genetics , RNA-Binding Protein EWS/genetics , Sarcoma, Ewing/genetics , Antineoplastic Combined Chemotherapy Protocols , Child, Preschool , Combined Modality Therapy , Female , Hepatectomy , Humans , In Situ Hybridization, Fluorescence , Liver Neoplasms/pathology , Liver Neoplasms/therapy , Reverse Transcriptase Polymerase Chain Reaction , Sarcoma, Ewing/pathology , Sarcoma, Ewing/therapy , Transcription, GeneticABSTRACT
AIM: Undifferentiated sarcoma of the liver (USL) is the third commonest malignant liver tumor in children. The aim of our study is to evaluate the outcome of this rare entity according to the quality of the surgical resection and the compliance to the European pediatric soft tissue sarcoma group guidelines. PATIENTS AND METHODS: We conducted a monocentric review of patients referred to our department with a definitive pathologic diagnosis of USL between 1997 and 2013. We looked at the diagnosis and management pitfalls, surgical technique, and outcomes. Results are expressed as median (range). RESULTS: There were 13 patients (M/F=7:6=1.1). Age at presentation was 8 years (range, 11 months-16 years). Of the 13 patients, 10 patients (69%) presented with abdominal pain, 5 (38%) with tumoral bleeding, and 2 (15%) with peritoneal rupture. All lesions were unique, nonmetastatic, and heterogeneous with cystic components measuring 14 (6-19) cm. Six (46%) patients had an upfront surgery: five because of wrong clinical diagnosis (three query mesenchymal hamartoma, one spontaneous peritoneal bleeding, and one cystic lymphangioma), and one because of rapid enlargement of the mass. Seven (54%) patients (including one with tumoral bleeding) received neoadjuvant chemotherapy, and had their tumor diameter decreasing by 40% (range, 0-60%). Final surgery consisted of seven right hepatectomies; one right extended hepatectomy; three mesohepatectomies; two left hepatectomies. There were three incomplete resection in the upfront surgery group versus none in the neoadjuvant chemotherapy group. The degree of tumor necrosis after chemotherapy ranged from 95 to 100%. Surgical complications included the following: liver transplantation (LT) following a Budd-Chiari syndrome after a mesohepatectomy, one biliary ducts injury treated by Roux-en-Y loop. All patients received the postoperative chemotherapy according to the European protocol. One of seven patients (14%) with neoadjuvant chemotherapy underwent radiotherapy for rupture at diagnosis versus three of six patients (50%) with upfront surgery: one for rupture at diagnosis and two for rupture during upfront surgery. One patient (17%) with upfront surgery had local recurrence at 2 years after initial surgery, and is in second complete remission 1 year after a redo surgery. All patients are alive at a median 34 months (range, 5-134) follow-up. CONCLUSION: USL presents with painful mixed cystic and solid liver mass. If misdiagnosed and mistreated (enucleation or unroofing), the usual good outcome of this malignancy could be impaired. Preoperative chemotherapy is recommended.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Hepatectomy , Intraoperative Care/methods , Liver Neoplasms/surgery , Neoadjuvant Therapy , Sarcoma/surgery , Adolescent , Antineoplastic Agents/administration & dosage , Chemotherapy, Adjuvant , Child , Child, Preschool , Female , Follow-Up Studies , Hepatectomy/methods , Humans , Infant , Liver Neoplasms/drug therapy , Male , Postoperative Complications , Retrospective Studies , Sarcoma/drug therapy , Treatment OutcomeABSTRACT
Childhood obliterative portal venopathy presents at any age and may be genetic in origin. We report 48 children with obliterative portal venopathy, based on strict histologic criteria, investigated between 1972 and 2011. Diagnosis requires histology and is suggested by ultrasonography findings. Portal hypertension is the main complication but is absent in some cases. Prognosis is relatively good, but the detection of cardiopulmonary complications is essential.
Subject(s)
Hypertension, Portal/complications , Liver/pathology , Portal Vein/pathology , Vascular Diseases/complications , Adolescent , Child , Child, Preschool , Female , Humans , Hypertension, Portal/diagnosis , Infant , Infant, Newborn , Male , Prognosis , Vascular Diseases/diagnosisABSTRACT
Various vascular and nonvascular hepatobiliary interventional radiology techniques are now commonly performed in children's hospitals. Although the procedures are broadly similar to interventional practice in adults, there are important differences in indications and technical aspects. This review describes the indications, techniques, and results of liver biopsy, hepatic and portal venous interventions and biliary interventions in children.