Endovascular Treatment of Congenital Portosystemic Shunt: A Single-Center Prospective Study.

Purpose: To design a prospective study on endovascular closure of congenital portosystemic shunts. The primary endpoint was to assess the safety of endovascular closure. The secondary endpoint was to evaluate the clinical, analytical and imaging outcomes of treatment. Methods: Fifteen patients (age range: 2 days to 21 years; 10 male) were referred to our center due to congenital portosystemic shunts. The following data were collected prior to treatment: age, sex, medical history, clinical and analytical data, urine trimethylaminuria, abdominal-US, and body-CT. The following data were collected at the time of intervention: anatomical and hemodynamic characteristics of the shunts, device used, and closure success. The following data were collected at various post-intervention time points: during hospital stay (to confirm shunt closure and detect complications) and at one year after (for clinical, analytical, and imaging purposes). Results: The treatment was successful in 12 participants, migration of the device was observed in two, while acute splanchnic thrombosis was observed in one. Off-label devices were used in attempting to close the side-to-side shunts, and success was achieved using Amplatzer™ Ductus-Occluder and Amplatzer™ Muscular-Vascular-Septal-Defect-Occluder. The main changes were: increased prothrombin activity (p=0.043); decreased AST, ALT, GGT, and bilirubin (p=0.007, p=0.056, p=0.036, p=0.013); thrombocytopenia resolution (p=0.131); expansion of portal veins (p=0.005); normalization of Doppler portal flow (100%); regression of liver nodules (p=0.001); ammonia normalization (p=0.003); and disappearance of trimethylaminuria (p=0.285). Conclusion: Endovascular closure is effective. Our results support the indication of endovascular closure for side-to-side shunts and for cases of congenital absence of portal vein.

Endovascular Closure Resolves Trimethylaminuria Caused by Congenital Portosystemic Shunts.

This study aimed to report three new cases of an association between two rare conditions, congenital portosystemic shunts (CPSS) and trimethylaminuria (TMAU), and the efficacy of endovascular closure of the CPSS for resolving TMAU. Between November 2014 and April 2017, 15 patients with CPSS were enrolled in this prospective study to assess the efficacy of percutaneous endovascular shunt closure. Three patients presented with clinical symptoms of TMAU that were confirmed by urine analysis of trimethylamine (TMA) and TMA n-oxide. One year after endovascular closure of the congenital portosystemic shunt, the same parameters were evaluated were obtained and the values were compared to the pretreatment values. The results indicated the disappearance of clinical symptoms of TMAU and normalization of the urine test parameters in two patients and no changes in one patient, who developed new portosystemic communications.

Validation of the Position of Injection Needles with Contrast-Enhanced Ultrasound for Dynamic Contract-Enhanced MR Lymphangiography.

PURPOSE: To assess the feasibility of validating the position of the needle in groin lymph nodes using injection of ultrasound (US) contrast prior to magnetic resonance (MR) lymphangiography. MATERIALS AND METHODS: In 28 patients (average age, 52 y; 15 women, 13 men), 25G needles were placed in bilateral groin lymph nodes using US guidance outside the MR suite. The confirmation of the position of the needles in lymph nodes and efferent lymphatic flow was performed by injecting 1 mL of the US contrast agent (Lumason) mixed with 2 mL of lidocaine and observing contrast enhancement of the efferent lymphatic ducts leading to the pelvis from the lymph node. The patients were then transferred to the MR suite, and MR lymphangiography was performed. RESULTS: In 6 patients, the needle was repositioned due to nonvisualization of the efferent lymphatic flow toward pelvis at the first attempt. MR lymphangiogram was then performed in all patients with good opacification of the central lymphatic system. No extravasation of gadolinium contrast agent was observed. CONCLUSIONS: Injection of US contrast to confirm the position of the needles inside the lymph nodes is a safe and effective technique. This technique can serve as a substitute for fluoroscopic confirmation of needle position, allowing performance of MR lymphangiography on any MR machine with a detachable table.

Mielolipoma gigante presacro / Giant presacral myelolipoma

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