Prehepatic portal hypertension with aneurysm of the portal vein: unusual but treatable malformative pattern.

INTRODUCTION: Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. METHODS: Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. RESULTS: Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. CONCLUSIONS: These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.

Role of three-phase bone scintigraphy in paediatric osteoid osteoma eligible for radiofrequency ablation.

OBJECTIVE: The objective of the study was to underline the importance of three-phase bone scintigraphy at the time of diagnosis in children with suspected osteoid osteoma (OO) who are eligible for radiofrequency ablation. METHODS: Fifty-three patients (13 girls; mean age 7.2 years, 20% younger than 10 years of age) who underwent bone scintigraphy for suspected OO between 2005 and 2010 were included in the study, of whom 46 underwent a radiography at diagnosis. Computed tomography-guided biopsy was performed in all patients after bone scintigraphy, and radiofrequency ablation was performed following biopsy in patients with OO; ablation efficacy was confirmed by MRI at 1, 3, 12 and 18 months. RESULTS: The radiographic results were negative in 27/46 patients and was unclear in 19. Bone scintigraphy showed lesions in 53/53 patients, of whom 51 patients had a typical pattern of osteoma and nine patients required an additional scan with a pinhole collimator. Histological examination showed OO in 51/53 patients (3/51 intramedullary), Ewing's sarcoma in 1/53 patients, and chronic osteomyelitis in 1/53 patients. CONCLUSION: Any child with recurrent nocturnal pain and/or limb swelling should undergo radiography of the involved skeletal segment, which is the first-choice diagnostic method in the clinical suspicion of OO. In the event of ambiguous or negative radiographic results, bone scintigraphy is needed to exclude other pathologic conditions and to confirm the diagnosis. In children with recurrent but not well-localized bone pain in which OO is strongly suspected for signs and symptoms, a bone scan can help detect the lesion. The diagnostic accuracy of the bone scan, particularly for the appendicular skeleton, can be improved by pinhole collimator acquisition.

Selective arterial embolization of giant renal tuberous sclerosis.

Different minimally invasive methods have been proposed to treat renal manifestation of tuberous sclerosis complex (TSC), in order to preserve kidney parenchyma and avoid nephrectomy. The case of a boy with bleeding giant angiomyolipoma treated by selective arterial embolization is discussed. The extensive renal lesions resulted significantly decreased, with resolution of arterial hypertension and abdominal symptoms. Differential renal function increased from 26.6% to 32.6%. Renal involvement in TSC requires careful parenchymal-sparing procedures. Selective embolization of the main renal lesions should be considered as first line therapy.

Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review.

Congenital intrahepatic portosystemic shunts are rare hepatic vascular anomalies that often lead to severe secondary conditions. A 6-year-old boy was referred for assessment of severe hypoxia, and a large liver mass was diagnosed with such a malformation and was managed by direct closure of the venous fistula by interventional radiology. Follow-up assessment shows normalization of the respiratory condition and a progressive reduction of the vascular liver lesion. Review of literature suggests that radiologic interventions are currently the criterion standard for managing these conditions, with surgery reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation.

Particular imaging features and customized thermal ablation treatment for intramedullary osteoid osteoma in pediatric patients.

OBJECTIVE: To report on the particular imaging features and high success rate of cold mode radio-frequency thermal ablation (RFTA) as the treatment of choice for intramedullary osteoid osteoma. MATERIALS AND METHODS: The study population consisted of 51 patients (39 males, 12 females; mean age 7.2 years; 11 patients under 6 years of age, including 7 males and 4 females) who underwent RFTA for osteoid osteoma and were retrospectively observed. The affected sites were the tibia (n = 22, 43%), femur (n = 13, 25%), pelvis (n = 5, 10%), anklebone (n = 3, 6%), humerus (n = 2, 4%), sacrum (n = 2, 4%), heel, radium, patella ,and rib (n = 1, 2%), respectively. Three patients had tibial intramedullary osteoid osteoma (14% of the tibial lesions, 6% of all cases). Cold mode RFTA was performed for these three patients to obtain a large ablation area without positioning two probes. The noncooled mode was used to treat cortical and subperiosteal lesions. RESULTS: Following RFTA, all patients were pain-free and in good clinical condition. In the intramedullary osteoid osteoma group, no recurrences were observed during the 24-month follow-up period, but one patient, who was affected by cortical osteoid osteoma, required two RFTA treatments to heal completely. CONCLUSION: Children less than 6 years of age with recurrent nocturnal pain and limb swelling should be investigated for intramedullary osteoid osteoma. Once confirmed, CT-guided RFTA should be the first treatment for intramedullary osteoid osteomas because of the high success rate and reduced invasivity, especially with cold mode RFTA. The outcome is related to the disappearance of pain, and the efficacy may be checked shortly after treatment with MR imaging to evaluate the absence of lesion in the ablation area.

Radiofrequency thermoablation in chest wall mesenchymal hamartoma of an infant.

We report on an infant presenting with a chondroid hamartoma managed with a combined conservative surgical treatment and radiofrequency thermoablation.

Blind and ultrasound-guided percutaneous liver biopsy in children.

BACKGROUND: Percutaneous biopsy of the liver is the most commonly used procedure to obtain tissue for histopathological assessment of liver disease. Although, intuitively, image-guided liver biopsy might be expected to reduce the risk of bleeding, haematoma caused by a penetrating injury of a branch of the hepatic artery or portal vein, and puncture of the gallbladder, no trial has been large enough to show reduced mortality or morbidity with US guidance, and the mechanisms by which the use of US can reduce the risk of bleeding remain speculative. OBJECTIVE: To compare the mortality and morbidity of blind liver biopsy with that of US-guided liver biopsy. MATERIALS AND METHODS: A retrospective review of our experience of 140 procedures over a 16-month period. RESULTS: In the blind group, biopsy was unsuccessful in ten children (95% CL 7.3-25.4); no tissue was obtained in eight children and an inadequate sample was obtained in two. Three children (95% CL 9.2-14.7) suffered significant haemorrhage (indicated by a drop in haemoglobin of >20 g/l) with intrahepatic ( n=1) and subcapsular ( n=2) haematomas detectable by US after biopsy. An adequate sample was obtained in all children in the US-guided group. There were no complications requiring treatment in either group. CONCLUSIONS: Our results showed a significant difference in the complication rate between liver biopsy undertaken with US guidance and liver biopsy performed blind ( P=0.002).

Aneurysmal bone cysts: treatment with direct percutaneous Ethibloc injection: long-term results.

PURPOSE: To assess the efficacy and long-term results of Ethibloc treatment in aneurysmal bone cysts (ABC). METHODS: Thirteen patients with ABC were treated with direct percutaneous Ethibloc injection. Four patients had only one injection and the other nine patients from two to four injections. No severe complications were observed; in two patients a local leakage of Ethibloc from the cyst into the soft tissues occurred but it was temporary and the consequent inflammation self-healed without residua and sequelae. Imaging follow-up lasted from 6 to 67 months and included conventional radiology (CR) and magnetic resonance imaging (MRI), both used in the presurgical phase. RESULTS AND CONCLUSIONS: All images demonstrated a remarkable shrinkage of the cystic lesion and bone cortex thickening. In all patients, circumscribed areas of lucency persisted at radiography, corresponding to residual cystic areas without fluid-fluid levels at MRI. Pain, which was present in all the patients before treatment, was relieved within a month. According to our experience, direct percutaneous Ethibloc injection is effective in the treatment of ABC and can be recommended as the first-choice treatment. Due to its higher sensitivity MRI must be included either in the pretreatment phase to study the multilocular structure or in the imaging follow-up to evaluate the efficacy of Ethibloc in persistently non-responsive areas.