High Variability in the Reported Management of Hepatic Veno-Occlusive Disease in Children after Hematopoietic Stem Cell Transplantation

Abstract

Veno-occlusive disease (VOD) is a potentially fatal complication of hematopoietic stem cell transplantation (HSCT). Patients with VOD are often critically ill and require close collaboration between transplant physicians and intensivists. We surveyed members of a consortium of pediatric intensive care unit (PICU) and transplant physicians to assess variability in the self-reported approach to the diagnosis and management of VOD. An internet-based self-administered survey was sent to pediatric HSCT and PICU providers from September 2014 to February 2015. The survey contained questions relating to the diagnosis and treatment of VOD. The response rate was 41% of 382 providers surveyed. We found significant variability in the diagnostic and management approaches to VOD in children. Even though ultrasound is not part of the diagnostic criteria, providers reported using reversal of portal venous flow seen on abdominal ultrasound in addition to Seattle criteria (70%) or Baltimore criteria to make the diagnosis of VOD. Almost 40% of respondents did not diagnose VOD in anicteric patients (bilirubin < 2 mg/dL). Most providers (75%) initiated treatment with defibrotide at the time of diagnosis, but 14%, 7%, and 6% of the providers waited for reversal of portal venous flow, renal dysfunction, or pulmonary dysfunction, respectively, to develop before initiating therapy. Only 50% of the providers restricted fluids to 75% of the daily maintenance, whereas 21% did not restrict fluids at all. Albumin with diuretics was used by 95% of respondents. Platelets counts were maintained at 20,000 to 50,000/mm(3) and 10,000 to 20,000/mm(3) by 64% and 20% of the respondents, respectively. Paracentesis was generally initiated in the setting of oliguria or hypoxia, and nearly 50% of the providers used continuous drainage to gravity, whereas the remainder used an intermittent drainage approach. Nearly 73% of the transplant providers used VOD prophylaxis, whereas the remainder did not use any medications for VOD prophylaxis. There was also considerable variation in the management strategies among the transplant and critical care providers. We conclude that there is considerable self-reported variability in the diagnosis and management of VOD in children. The practice variations reported in this study should encourage the development of standard practice guidelines, which will be helpful in improving the outcome of this potentially fatal complication.

The role of continuous renal replacement therapy in the management of acute kidney injury associated with sinusoidal obstruction syndrome following hematopoietic cell transplantation

“…Skeens et al found significant variability in the diagnosis and management of SOS between HCT and Critical Care providers. The current work presents our institutional experience with collaborative efforts between Pediatric HCT, Nephrology, and Critical Care providers using a clinical evidence‐based algorithm for fluid management and early CRRT initiation in pediatric HCT patients with SOS, AKI, and FO (Figure ).…”

“…Severe SOS requires a multidisciplinary approach for optimal patient outcomes . The Pediatric HCT, Nephrology, and Critical Care teams developed institutional guidelines for managing AKI and FO in HCT patients.…”

“…Severe SOS requires a multidisciplinary approach for optimal patient outcomes. 5,7 The Pediatric HCT, Nephrology, and Critical Care teams developed institutional guidelines for managing AKI and FO in HCT patients. This report describes the index case that initiated the development of fluid management protocol attempting to standardize a clinical approach for five more patients who underwent CRRT as a supportive care modality in managing their severe SOS.…”

Cost-Effectiveness of Defibrotide in the Prophylaxis of Veno-Occlusive Disease after Pediatric Allogeneic Stem Cell Transplantation

“…Defibrotide (DF) with or without high-dose methylprednisolone is increasingly being used to treat moderate-tosevere VOD [7][8][9]. Improved supportive care, less toxic conditioning regimens, and the therapeutic use of DF may have contributed to the reduced incidence and severity of VOD.…”

The role of peritoneal drainage in veno-occlusive disease in pediatric patients post hematopoietic stem cell transplant

“…The treatment of SOS/VOD in children is highly variable, as noted in a recent survey [8]. Until recently, management included supportive care only, as thrombotic agents were not effective and also increased the risk of bleeding.…”