Transjugular Intrahepatic Portosystemic Shunt for Very Severe Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome (VOD/SOS) after Unmanipulated Haploidentical Hematopoietic Stem Cell Transplantation with Post-transplantation Cyclophosphamide.

Hepatic veno-occlusive disease or sinusoidal obstruction syndrome (VOD/SOS) is a threatening complication after both autologous and allogeneic hematopoietic stem cell transplantation (HSCT), with high mortality rates despite early medical treatment, including the use of defibrotide (DF). We retrospectively analyzed 185 unmanipulated haploidentical (haplo-) HSCT with post-transplantation cyclophosphamide as graft-versus-host disease prophylaxis performed consecutively between 2011 and June 2019 in a single center. Seventeen patients (9.2%) were diagnosed with VOD/SOS. Based on revised European Society for Blood and Marrow Transplantation severity criteria, the VOD/SOS cases were classified as mild in 2 patients (11.7%), moderate in 2 (11.7%), severe in 2 (11.7%), and very severe in 11 (64.9%). Thirteen patients (76%) were treated with DF, including all patients with severe or very severe VOD/SOS, except 1 patient with CNS hemorrhage. Sixteen patients (94%) were alive at day +100 after HSCT. Seven patients (41%) with very severe VOD/SOS were treated with transjugular intrahepatic portosystemic shunt (TIPS) owing to rapid clinical or analytical deterioration or refractory hepatorenal syndrome despite medical treatment, including DF. TIPS insertion was performed at a median time since VOD/SOS diagnosis of 4 days (range, 1 to 28 days) without technical complications in any case. The median hepatic venous pressure gradient before and after TIPS treatment was 24 mmHg (range, 14 to 29 mmHg) and 7 mmHg (range, 2 to 11 mmHg), respectively, with a median drop of 16 mmHg (range, 9 to 19 mmHg). Following TIPS insertion, all patients showed clinical improvement with hepatomegaly, ascites, and renal failure resolution, and all showed analytical improvement with reduced alanine aminotransferase (ALT), creatinine, and international normalized ratio values, except for patient 2, whose indication for TIPS was refractory hepatorenal syndrome with a normal ALT level. The 6 patients who had initiated DF before TIPS insertion completed 21 days of treatment. All patients met the criteria for complete remission (CR) at a median of 8 days after TIPS insertion (range, 2 to 82 days). The 100-day overall survival was 100%. For patients with rapid progressive VOD/SOS, early TIPS insertion allowed completion of DF therapy. The use of TIPS together with DF resulted in CR and no associated complications with no VOD/SOS-associated mortality despite high severity. In our experience, timely and individualized use of TIPS significantly improves outcomes of very severe VOD/SOS after haplo-HSCT. Therefore, TIPS should be promptly considered in rapidly progressive cases.

Pancreatic regeneration after near-total pancreatectomy in children with nesidioblastosis.

BACKGROUND: Nesidioblastosis is often resistant to medical therapy and requires near-total pancreatectomy. There is little information on the postoperative imaging findings of these patients. OBJECTIVE: To demonstrate by US the late imaging findings in these patients. MATERIALS AND METHODS: Children diagnosed with nesidioblastosis and who had undergone 90-95% pancreatectomy received preoperative, immediate-postoperative (within 10 days of surgery) and long-term annual US examinations. In the preoperative study, three anterior-posterior (AP) measurements were obtained of the head, body and tail of the pancreas. In the postoperative and long-term follow-up US, AP and transverse measurements of the pancreatic remnant were obtained. Pancreatic echogenicity was also assessed. The results were compared with normal pancreatic dimensions as a function of age. Glucose metabolism and pancreatic enzymes were also analysed. RESULTS: The study group comprised 22 patients (aged 9 days to 2 years). The pancreas was normal in all preoperative US examinations. The first postoperative examination showed, in all patients, a remnant of the pancreatic head measuring 8-13 mm. The last follow-up US was similar to the first postoperative study in 6 patients, and 12 showed complete pancreatic regeneration (normal size, echogenicity and function), and 4 had incomplete regeneration with head and body normal in size, but lack of a pancreatic tail. All patients were asymptomatic and showed normal laboratory tests. CONCLUSIONS: US measurements indicated normal age-dependent growth after near-total resection in 54% of patients. The function and echogenicity of the regenerated pancreas indicate that the increase in organ size was due to normal pancreatic tissue.