Piergiorgio Falappa

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.

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

ObjectiveThe 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. MethodsFifty-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. ResultsThe 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. ConclusionAny 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.

Diagnostic and therapeutic tricks in a rare case of pediatric ileal congenital arteriovenous malformation.

90 iovenous malformatio red initially in the di C ongenital arter ns (AVMs) are not usually conside agnostic workup of a pediatric melena (1). After excluding conditions requiring nonoperative management, conventional endoscopic evaluations and radionuclide scans are, in most patients, able to define the site of bleeding and address the type of operative treatment. Furthermore, capsule endoscopy or double-balloon enteroscopy can provide additional information in doubtful cases, even of pediatric age (2). Nevertheless, there are some cases in which the diagnosis remains difficult, setting up an ‘‘obscure melena’’ requiring additional investigations. AVMs are rare in children, usually small, single, predominantly located in the rectum and descending colon, and relatively uncommon in the small bowel (3). An exceedingly rare case of congenital AVM arising from the ileal branch of the ileocolic artery in a baby girl is described as well as the original method of angiographic wire placement, as an intraoperative locating mark, to help surgical resection planning.

Sclerotherapy of primary aneurysmal bone cysts: comment to Lambot-Juhan et al.

Sir, We read with great interest the article by Lambot-Juhan et al. [1] titled “Primary aneurysmal bone cysts in children: percutaneous sclerotherapy with absolute alcohol and proposal of a vascular classification.” We agree that alcohol can be safely used for percutaneous treatment of primary aneurysmal bone cysts, and we have performed this procedure for simple bone cysts nonresponsive to local corticosteroid injection since alcoholic zein solution (Ethibloc, Ethnor Laboratories/Ethicon, Norderstedt, Germany) was retracted from the market. However, we hereby respectfully express our reservations regarding some points in the manuscript: & Performance of an open biopsy of aneurysmal bone cysts before percutaneous sclerotherapy might not correspond to the criteria of a minimally invasive procedure [2]. Biopsy of the cyst might be obtained with a percutaneous procedure as well, using biopsy forceps introduced under fluoroscopic guidance through an 11-G bone biopsy needle, in order to obtain multiple specimens from the cyst wall. & Differential diagnosis between aneurysmal bone cysts and simple bone cysts (unicameral bone cysts in the Campanacci classification) [3] may also be done with MRI. In this respect a fluid level within the cyst, secondary to gross blood contained, may be demonstrated in aneurysmal bone cysts and may be absent in simple bone cysts [4, 5]. However, a fluid level discovered in a cystic bone lesion may also be related to a telangiectatic sarcoma, a highly malignant neoplasm with different prognostic and therapeutic features [6, 7]. For that reason, a histological definition of the lesion with adequate sampling before treatment is necessary. & Venous escapes from the cysts are often noted during contrast injection before sclerotherapy and their presence may be related to both the pressure and the volume of contrast material injected during the procedure, especially when the injection is performed with a single needle inserted into the cyst. & The role of MRI in follow-up studies after treatment must be emphasized, especially for patients such as infants and adolescents who can undergo the exam without sedation or anesthesia [4, 5]. Furthermore, the sensitivity of MRI is higher when compared to conventional radiographic study for the detection of recurrence of the aneurysmal bone cysts (blood-filled spaces, fluid levels) [4, 5], thus orientating physicians to other treatments. A. Crocoli (*) Department of Surgery and Transplantation, Division of General Surgery, Bambino Gesù Children Hospital IRCCS, Piazza S. Onofrio 4, 00165 Rome, Italy e-mail: alessandro.crocoli@opbg.net

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.