Outcomes and indications for intervention in non-operative management of paediatric liver trauma: a 5 year retrospective study.

AIM: To determine the applicability of accurate computed tomography (CT) evaluation and embolization as non-operative management for liver trauma in a paediatric population. MATERIAL AND METHODS: A retrospective observational study of 37 children (mean age 10.5 years) with hepatic trauma (28 blunt, 9 penetrating) admitted to a trauma referral centre over a 5 year period. All patients were evaluated with CT and scored with an Association for the Surgery of Trauma score. Inpatient information was reviewed for demographics, associated injuries, modes of management, efficacy and complications of management, and outcome.Statistical analysis was performed. RESULTS: There were seven contusions, two grade I, two grade II, nine grade III, and 17 grade IV liver lacerations. Only two patients (grade IV, penetrating) underwent surgery for the management of bowel perforation. All children had non-surgical treatment of their liver trauma: three cases (grade IV) had primary angiography due to CT evidence of active bleeding and embolization was performed in two of these. Seven patients (two grade III, five grade IV)had angiography during the follow-up for evidence of a complicating pseudoaneurysm and embolization was performed in six of them. Embolization was successful in all the children; one minor complication occurred (cholecystitis). Endoscopic retrograde cholangiopancreatography (ERCP) plus stenting was performed in two cases for a bile leak. All 37 children had a positive outcome. CONCLUSION: The present study demonstrates that non-operative management of hepatic trauma is applicable to children and may have a higher success rate than in adults.

Hepatic splenosis presenting as arterialised liver lesion in a patient with NASH.

INTRODUCTION: Splenosis represents the heterotopic autotransplantation of splenic tissue after a traumatic splenic rupture and splenectomy. It is not a rare condition and it is estimated to occur in up to 67% of patients with traumatic splenic rupture. CASE REPORT: We report one case of patient, affected by non alcoholic steatohepatitis (NASH), with a hypervascularised liver lesion, that the final histological examination revealed hepatic splenosis. This is a rare condition that may be misinterpreted as adenoma or hepatocellular carcinoma (HCC). Imaging techniques and features that might contribute to the diagnosis and may avoid invasive treatment are also discussed. Although hepatic splenosis is a rare condition, this diagnosis should be considered in patients with previous history of abdominal trauma and then the diagnosis of splenosis may be confirmed by Tc-99m-DRBC scintigraphy, avoiding biopsy or further surgery.

Manganese-enhanced MRI predicts the histological grade of hepatocellular carcinoma in potential surgical candidates.

AIM: To evaluate the role of manganese-enhanced magnetic resonance (Mn-MRI) in predicting tumour differentiation prior to liver transplant or resection for hepatocellular carcinoma (HCC). MATERIALS AND METHODS: The inclusion criteria were patients with HCC who underwent Mn-MRI prior to transplantation or resection from 2001-2008. T1-weighted MRI images were acquired at 0 and 24h after manganese dipyridoxal diphosphate (MnDPDP) intravenous contrast medium and reviewed prospectively. Manganese retention at 24h was correlated with tumour differentiation and disease-free survival. RESULTS: Eighty-six patients underwent Mn-MRI (transplantation 60, resection 26); 114/125 lesions (91%) that were arterialised as evidenced at computed tomography (CT) and had manganese uptake on MRI were HCC. There were 11 false positives (9%) that were regenerative nodules. Ten of fourteen non-manganese-retaining HCC (71%) were poorly differentiated, compared with only 13/114 manganese-retaining HCC (11%) (p<0.0001). Sensitivity, specificity, positive and negative predictive values of non-retention of MnDPDP in predicting poorly differentiated tumours were 0.43, 0.96, 0.71 and 0.88. Median disease-free survival of patients with non-manganese-retaining HCC was less than for patients with manganese-retaining HCC (14±5 months versus 39±3 months, log rank p=0.025). CONCLUSION: Non-manganese-retaining HCCs are likely to be poorly differentiated and have a poor prognosis. Manganese-enhanced MRI appears to have a role in preoperative assessment of HCC and warrants further evaluation.

Diagnosis of Alagille syndrome-25 years of experience at King's College Hospital.

OBJECTIVE: The aim of the study was to study the clinical and histological features of Alagille syndrome (AGS) at presentation comparing the value of the various modalities before the implementation of genetic diagnosis. PATIENTS AND METHODS: We performed a retrospective analysis of the records of 117 children diagnosed as having AGS after referral to King's College Hospital between 1980 and 2005. RESULTS: Cholestasis was seen in 104 of 117 (89%), characteristic facies in 91 of 117 (77%), posterior embryotoxon in 72 of 117 (61%), butterfly vertebrae in 44 of 117 (39%), heart disease (most often peripheral pulmonary stenosis) in 107 of 117 (91%), and renal disease in 27 of 117 (23%). Serum cholesterol levels of >5 mmol/L were seen in 52 of 86 (60.4%). Liver biopsy showed characteristic features of paucity of interlobular bile ducts in 59 of 77 (76.6%) children younger than 16 weeks of age, in 10 of 14 (71.4%) between 16 weeks and 1 year of age, and in 8 of 12 (66.66%) older than 1 year of age. Other biopsy findings were those of nonspecific hepatitis and biliary features. Iminodiacetic acid scans showed no excretion of isotope into the bowel after 24 hours in 21 of 35 (60%), and small/no gallbladder on ultrasound was seen in 29 of 104 (27.8%). Eleven of 117 (9.4%) had a diagnostic laparotomy and operative cholangiography, 2 proceeding to Kasai portoenterostomy before referral to our unit. CONCLUSIONS: Clinical features of AGS are not as consistently informative as suggested in the literature. Hypercholesterolaemia is nonspecific but may be a helpful pointer. Histology is not characteristic in 25%; hepatobiliary iminodiacetic acid scan and ultrasound may suggest a false diagnosis of biliary atresia in 60% and 28%, respectively, supporting the concept that infants with liver disease warrant early referral to a specialist centre. The advent of genetic diagnosis will redefine the syndrome with likely effects on the prognosis of the defined group.

Predictive value of bile duct dimensions measured by ultrasound in neonates presenting with cholestasis.

OBJECTIVES: : The significance of extrahepatic bile duct dilatation on ultrasound examination in jaundiced infants is often uncertain. We wished to clarify the diagnostic and prognostic significance of the present finding in neonatal conjugated hyperbilirubinaemia. PATIENTS AND METHODS: : We retrospectively enrolled all of the infants younger than 3 months with extrahepatic biliary dilatation > or =1.2 mm (nonfasting ultrasound) who presented during the study period. We reviewed clinical, radiological, and laboratory data to determine mode of presentation, diagnosis, interventions, and long-term outcome. RESULTS: Seventy-six infants (41 male) were identified, all of whom were referred with conjugated hyperbilirubinaemia. Median gestational age was 39 weeks (range 24-42 weeks). Inspissated bile was the most common diagnostic category, whereas congenital choledochal malformation was the diagnosis made in 13% infants. Dilatation was an incidental finding in 9% of the infants. Seventeen percent of infants had required either surgical or radiological intervention by the time of follow-up. Overall, 41% infants had spontaneous resolution of bile duct dilatation, including 8% who had "grown into" an unchanged duct size rather than involution of dilatation. The median size of bile duct at presentation for those who required intervention was 4.7 versus 2 mm for the remainder (P < 0.001). Of those who resolved spontaneously, the median size of duct at presentation was 1.8 mm. CONCLUSIONS: : Bile duct dilatation <3 mm (nonfasting ultrasound) with neonatal cholestasis is unlikely to be of significance whereas >4 mm is likely to be associated with choledochal malformation or need for intervention. The intermediate group is likely to be associated with inspissated bile syndrome following resolution of which innocent biliary dilatation may persist.

Role of imaging in the management of pancreatic mass.

Pancreatic cancer is the second commonest malignant gastrointestinal neoplasm. Modern imaging techniques have greatly increased sensitivity in diagnosing and staging pancreatic cancers. Multidetector CT in particular, plays a critical role in local staging and determining the resectability of pancreatic tumours. MR and endoscopic ultrasound are valuable in those groups of patients in whom CT findings alone are inconclusive in tumour characterisation and local staging, particularly vascular involvement. In this article we review the current established concepts and the role of imaging in the multidisciplinary management of pancreatic tumours together with a comprehensive review of the literature.

Cerebral manifestations in liver disease and transplantation.

Patients with liver disease frequently develop cerebral complications. The brain may be affected directly by the pathological process or agent responsible for the liver disease, as a consequence of the metabolic and haemodynamic derangement caused by hepatic dysfunction, or as a complication of the treatment of liver disease. Computed tomography (CT) and magnetic resonance imaging (MRI) can be helpful in the diagnosis and management of these complications, and advanced techniques such as diffusion weighted imaging (DWI) and magnetic resonance spectroscopy (MRS) are providing new insights into the underlying pathophysiological processes. We present a radiological overview of the cerebral manifestations of liver disease, categorized as acute presentations, chronic presentations, and conditions occurring in the context of liver transplantation.

A novel treatment of congenital hepatoportal arteriovenous fistula.

Congenital hepatoportal arteriovenous fistula is a rare cause of portal hypertension in young children. Unlike the acquired form, which is usually isolated and can be cured by hepatic artery (HA) embolization, recurrence of portal hypertension often occurs with congenital hepatoportal arteriovenous fistula after embolization and/or HA ligation because of early, rapid collateralization and the presence of multiple arterioportal fistula. Although long-term outcome after embolization is not known, liver transplantation has been proposed as the only option for this condition. However, portal vein and hepatic arterial anastomoses are made difficult because of the presence of portal vein arterialization and previous HA ligation, with a significantly increased risk of vascular complications. We report a case where resolution of portal hypertension has been achieved by an end-to-side portocaval shunt, to preserve the portal vein and HA for future liver transplantation, should it be required.

Vanishing liver tumours.

Spontaneous resolution of liver tumours is a rare, but recognized entity that has been reported to occur within the spectrum of benign and malignant liver tumours occurring in both adult and paediatric population. The aetiology of this unusual phenomenon is not clearly understood. In this article we present case examples of various benign and malignant liver tumours that have regressed spontaneously without treatment together with a review of the literature, and a summary of the current understanding of the pathogenesis of these tumours.

Hepatocyte transplantation followed by auxiliary liver transplantation--a novel treatment for ornithine transcarbamylase deficiency.

We report the first successful use of hepatocyte transplantation as a bridge to subsequent auxiliary partial orthotopic liver transplantation (APOLT) in a child antenatally diagnosed with severe ornithine transcarbamylase (OTC) deficiency. A total of 1.74 x 10(9) fresh and cryopreserved hepatocytes were administered intraportally into the liver over a period of 6 months. Immunosuppression was with tacrolimus and prednisolone. A sustained decrease in ammonia levels and a gradual increase in serum urea were observed except during episodes of sepsis in the first 6 months of life. The patient was able to tolerate a normal protein intake and presented a normal growth and neurological development. APOLT was successfully performed at 7 months of age. We conclude that hepatocyte transplantation can be used in conjunction with APOLT as an effective treatment for severe OTC-deficient patients, improving neurodevelopmental outcomes.

Colocutaneous fistula complicating therapeutic mesenteric embolisation.

Percutaneous embolotherapy has now assumed an important role in the management of massive colonic haemorrhage. However, this therapeutic option is associated with a significant risk of irreversible segmental colonic ischaemia. We present a case where distal segmental ischaemia led to a colocutaneous fistula, a complication not reported so far in the literature.

Parametric mapping of the hepatic perfusion index with gadolinium-enhanced volumetric MRI.

The purpose of this study was to adapt the hepatic perfusion index (HPI) methodology previously developed for MRI to derive 3D parametric maps of HPI, and to investigate apparent differences in HPI maps between a group of colorectal cancer patients and controls. To achieve this, a new and simpler approach to HPI calculation which does not require measurements from the aorta or portal vein is introduced, and assessed with large liver regions of interest (ROIs) in patients and controls. Several example HPI maps showing localized variation are then presented. The subject group consisted of 12 patients with known colorectal metastases, and 13 control subjects referred for routine contrast-enhanced spine imaging with no history of neoplastic disease. HPI was evaluated from serial T1 volume acquisitions acquired over the course of a Gd-DTPA bolus injection. Regions of abnormal perfusion were visible on the HPI maps derived for the patient group, manifested as areas of locally increased HPI extending around the visible margins of known metastases evident on the conventional contrast-enhanced images. This method for MR voxel-based parametric mapping of HPI has the potential to demonstrate regional variations in perfusion at the segmental and subsegmental level.

Peribiliary cysts can mimic Caroli's disease: a case report.

Peribiliary cysts, otherwise known as cystic dilatation of the peribiliary glands, are uncommon, and are usually discovered incidentally at autopsy, or in explants following liver transplantation. Preoperative diagnosis is often difficult owing to their asymptomatic nature and small size. Exclusion of a premalignant or malignant cystic condition is mandatory. We report a case of peribiliary cysts, initially thought to represent Caroli's disease, and briefly discuss the management of this condition.

Comparison of the hepatic perfusion index measured with gadolinium-enhanced volumetric MRI in controls and in patients with colorectal cancer.

The aim of the study was to adapt the methodology established for dynamic CT measurements of the hepatic perfusion index (HPI) to MRI, and to assess the potential role of MRI measurements of the HPI in detecting regional alterations in liver perfusion between patients with colorectal liver metastases and normal controls. The HPI was evaluated from serial T(1) volume acquisitions acquired over the course of a Gd-DTPA bolus injection. Time-course data from regions of interest in the liver, spleen and aorta were used to calculate the HPI; and HPI data from control subjects were compared with data from patients with known colorectal metastases. Significant differences were found between the relative portal perfusion and hepatic perfusion indices calculated for the patient and control groups (p<0.005). These results suggest that hepatic perfusion indices can be derived using MRI-based methods, and that these perfusion indices are sensitive to differences in liver perfusion associated with established metastatic liver disease on imaging. This technique may contribute to the early detection of liver metastases, allowing early surgical intervention and improved patient survival.

Hepatic artery stenosis following liver transplantation: significance of the tardus parvus waveform and the role of microbubble contrast media in the detection of a focal stenosis.

PURPOSE: To evaluate the role of microbubble ultrasound contrast media in detecting stenosis of the post-liver transplant extrahepatic hepatic artery (HA) in the presence of the tardus parvus spectral Doppler waveform of the intrahepatic HA. MATERIALS AND METHODS: All post-liver transplant patients with a prolonged systolic acceleration time (SAT>0.08s) and/or a reduced resistant index (RI<0.50) of the HA (the tardus parvus waveform) on colour Doppler ultrasound (CDUS), were assessed with microbubble contrast medium for a focal arterial stenosis. Following microbubble contrast-enhanced CDUS, patients underwent arteriography or follow-up CDUS. RESULTS: A total of 2038 examinations were performed in 529 liver transplant recipients; 16 (3.02%) tardus parvus waveforms were identified. The median SAT of the intrahepatic HA was 0.18s (range 0.11-0.38s) and the RI 0.47 (range 0.22-0.58). No extrahepatic elevated peak systolic velocity (PSV), defined as above 1.00m/s, was detected on the baseline examinations. Following the administration of microbubble contrast, medium, PSV in the extrahepatic HA was elevated in 14 of 16 patients, (median=2.15m/s, range=1.44-3.10m/s); flow was not identified in two patients. Arteriography was performed in 10 patients and confirmed stenosis in eight (median grade of stenosis 93%, range 60-99%) and occlusion in two. The measured median PSV at contrast-enhanced CDUS in the stenosis group was 2.03m/sec (range 1.44-2.71m/sec). Repeat CDUS in six patients not undergoing arteriography showed resolution in four; one underwent re-transplantation before arteriography and one patient maintains a tardus parvus waveform. In transplant recipients undergoing arteriography during the study period (n=55), no hepatic artery stenosis without a tardus parvus waveform was seen. CONCLUSION: The tardus parvus waveform pattern is an excellent screening test for the presence of post-liver transplantation hepatic artery stenosis. There is only a limited role for microbubble ultrasound contrast agent in the presence of a tardus parvus waveform. It could be used following equivocal colour Doppler ultrasound, but arteriography will still be necessary.

Accuracy of investigations for asymptomatic colorectal liver metastases.

PURPOSE: The aim of this study was to prospectively assess the accuracy of the most promising imaging and tumor marker tests in liver metastasis diagnosis on follow-up of asymptomatic colorectal cancer patients during a median of 57 months after primary tumor resection. METHODS: One hundred patients, who were considered free of liver metastases after primary colorectal cancer resection and conventional follow-up, were screened for liver metastases by computerized tomography, magnetic resonance and ultrasound scans, ultrasound Doppler and isotope assessment of changes in hepatic arterial and portal venous flow, and serum estimation of carcinoembryonic antigen. Patients were followed up during a median of 41 months to identify those who developed liver metastases. RESULTS: The most sensitive technique was computerized tomography (sensitivity 0.67, specificity 0.91). Computerized tomography and magnetic resonance but not ultrasound were 100 percent accurate in differentiating liver metastases from other hepatic lesions. Techniques based on changes in hepatic arterial and portal venous flow had lower diagnostic accuracies (Doppler perfusion index, sensitivity 0.58, specificity 0.57; hepatic perfusion index, sensitivity 0.50, specificity 0.55), whereas ultrasound scanning identified only 43 percent (sensitivity 0.43, specificity 0.96) and serum carcinoembryonic antigen 33 percent (sensitivity 0.33, specificity 0.81) of patients with asymptomatic liver metastasis. Sensitivity could be improved by using tests in combination but this reduced specificity. CONCLUSIONS: Computerized tomography was the most sensitive test for asymptomatic colorectal liver metastases, but only 67 percent of affected patients were identified.

Living related adult-to-adult liver transplantation: meeting the donor shortage.

Experience in the Cromwell Hospital London, Adult-to-Adult Living Donor Liver Transplantation programme is described with particular reference to the results obtained in the first five recipients. The first two of these received a left lobe graft and the remaining three a right lobe graft. Three sons/daughters, and two siblings were the donors. Four of the five recipients survived and did well. The one recipient who died was a complicated retransplant procedure. The donors showed rapid recovery of liver function with normal tests by the 10th day and with evidence of regeneration on follow-up CT volume evaluation. The value of the procedure for patients who have little chance of obtaining a cadaver organ is undoubted, but critical assessment of the recipient's clinical state is essential if success is to be obtained with a small graft and at all times the safety of the donor must remain of paramount concern, as reports to date indicate instances of donor death in adult programme.

Hepatic artery pseudoaneurysms following liver transplantation: incidence, presenting features and management.

AIM: Hepatic artery pseudoaneurysm (PA) is an uncommon complication of liver transplantation. We report a series of 13 patients, the largest published review including outcome. The presenting features, risk factors and role of radiology in the diagnosis and management of this frequently fatal complication are discussed.

Survival and outcome after hepatic artery thrombosis complicating pediatric liver transplantation.

BACKGROUND/PURPOSE: Hepatic artery thrombosis (HAT) represents a significant cause of graft loss and mortality after pediatric orthotopic liver transplantation (OLT). The incidence and etiology of this complication have been investigated in detail but relatively little is known about outcome. METHODS: A review was conducted of all children with confirmed HAT complicating OLT during a 10-year period (1990 through 1999) in a single center. HAT was established by angiography or at operation in all cases. RESULTS: From a consecutive series of 400 pediatric OLTs, there were 31 (7.8%) instances of HAT in 29 children of median age 3.8 years (range, 8 days to 16 years). Twenty-four (83%) are alive after a median follow-up of 3.6 years. Fourteen cases occurred after transplantation of whole grafts and 17 after reduced or split livers. Of the 18 episodes resulting in retransplantation, there were 5 deaths and 2 second episodes of HAT; surviving children are alive with good graft function. Of the 13 episodes managed without retransplantation, 4 patients underwent attempted early revascularisation of the graft, which was successful in 2, and the remainder initially were treated conservatively. All 13 children are alive after a median follow-up of 4.1 years (range, 0.6 to 5.8), but 5 required radiologic or surgical intervention for biliary or septic complications; biochemical liver function is normal in 8, mildly abnormal in 3, and poor in 2. Retransplantation was less likely in those who had received reduced or split grafts (7 of 17) compared with those who had received whole grafts (11 of 14), but this difference just failed to reach statistical significance (chi(2) = 3.01, 0.1 > P > .05). CONCLUSIONS: Using a selective policy of retransplantation, revascularisation, and conservative treatment, 83% of children survived HAT complicating OLT. Approximately 40% of children with HAT survived without retransplantation. J Pediatr Surg 36:888-891.