Arianna Bertocchini

Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: revisiting the role of adjuvant therapy

Inflammatory myofibroblastic tumor is an uncommon lesion, also called pseudotumor, with a variable natural course from benign with spontaneous regression to mimicking malignant tumors. We report a case of diffuse peritoneal and omental pseudotumor in a 10-year-old boy characterized by aggressive behavior at the onset followed by stability after subtotal resection and chemotherapy. Total excision was not possible because of the tumor dissemination over the whole peritoneal surface. Adjuvant antiinflammatory drug (ketorolac tromethamine) and chemotherapy (methotrexate-vinblastine followed by ifosfamide-adriamycin and ifosfamide alone) were helpful to obtain rapidly complete resolution of clinical symptoms and anatomic stability of the residual lesions. Long-term evolution, in the absence of continued therapy, has been characterized by progressive involution and reduction of the residual masses.

Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: anatomy and clinical relevance.

BACKGROUND Children with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not. METHODS All children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography. Venous anatomy was categorized into five subtypes (A to E), depending on the presence of thrombosis in the Rex recessus, or not, and its extension within the intrahepatic portal venous system. RESULTS Eighty-nine children entered the study. Previous umbilical vein catheterization is usually associated with Rex thrombosis, while the Rex recessus and the intrahepatic portal venous system are patent in idiopathic cases, thus allowing for the performance of a Meso-Rex Bypass with a good outcome. CONCLUSIONS Wedged hepatic venous portography is a very effective tool for detailed preoperative assessment and identification of children being considered for Meso-Rex Bypass surgery. An anatomic-radiological classification is useful in selecting patients for Meso-Rex Bypass with anticipation of a high rate of success.

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.

Ultrasound-guided percutaneous sclerosis of congenital splenic cysts using ethyl alcohol 96% and minocycline hydrochloride 10%: A pediatric series

INTRODUCTION The management of congenital splenic cysts continues to evolve. In the past the standard treatment was splenectomy, but increased knowledge about the spleens immunologic function has led most pediatric surgeons to preserve splenic tissue. A great number of studies using sclerosing substances have been published, but to date reports in children have been limited. Our study concerns a group of 15 children with congenital splenic cysts treated with percutaneous drainage and sclerosis with alcohol. We performed the procedure under general anesthesia and checked radiologically for possible leakage. METHODS In 2000 our group started managing pediatric patients with splenic cysts. During the first eight years surgery was the treatment of choice. From April 2008 to December 2014, a prospective study was conducted on 15 consecutive patients treated with percutaneous sclerotherapy. The outcomes regarding cystic dimensional variations before and after treatment were analyzed. RESULTS In 20% of patients complete disappearance of the cystic lesion was achieved. In 67% of the patients the maximum diameter of the cyst was reduced to below 50mm. CONCLUSION Our results should encourage the use of this treatment because it is a valid and safe option in childhood. The high success rate achieved with percutaneous drainage and sclerotherapy of cystic lesions supports our results.

Meso-Rex bypass to manage prehepatic portal hypertension after the failure of an intrahepatic portosystemic stent shunting

A cavernomatous transformation of the extrahepatic portion of the portal vein is a common cause of chronic portal hypertension in children. A few attempts at radiological interventions have been reported, but have rarely been successful. In this report, a surgical Meso-Rex bypass was performed to treat complicated prehepatic portal hypertension, after the insertion of an intrahepatic stent for portosystemic shunting had failed. The review of this case nicely illustrates how differently effective are these two shunting procedures—in terms of restoring hepatopetal flow, managing portal hypertension, and establishing—or not—portosystemic connections.

Caval replacement strategy in pediatric retroperitoneal tumors encasing the vena cava: a single-center experience and review of literature

BACKGROUND Complete encasement of the inferior vena cava by retroperitoneal tumors is rare. Although replacement of the vena cava has been considered for various conditions in adults, it is rarely used in children except for challenging resections and as a last chance approach - often aiming more at debulking than cure. MATERIALS AND METHODS From January 2009 to February 2017, 4 patients (2 adrenal neuroblastomas, 1 renal cell carcinoma, 1 infantile fibrosarcoma) underwent elective en-bloc resection of tumor and of the infrahepatic portion of the inferior vena cava (IVC), with planned IVC prosthetic replacement. In three cases a portion of the left renal vein had to be resected as well, with the vein reanastomosed onto the prosthesis, and a concomitant auto-transplantation of the right kidney was associated in one neuroblastoma patient. RESULTS All patients had an uncomplicated postoperative course. In one patient, the prosthetic conduit is patent at long-term (43 months), while the middle portion of the prosthesis did eventually thrombose at mid-term after surgery in the three others - with no related symptoms. Interestingly, all renal venous reconstructions remain patent. Three patients (2 neuroblastomas and 1 infantile fibrosarcoma) are alive and disease-free at 43, 74 and 108 months after surgery, respectively. One patient with renal cell carcinoma died of recurrence of the disease 21 months after surgery. CONCLUSION Resection and reconstruction of the vena cava, including the renal vein portion, can be considered and planned electively in case of tumoral encasement. This strategy is associated with good tolerance of the operation, low morbidity and satisfactory long-term function, even in cases with progressive and/or secondary partial thrombosis. LEVEL OF EVIDENCE IV.