RESUMEN
INTRODUCTION: Vascular malformation is associated with coagulopathies, especially when hemostasis is challenged. CASE PRESENTATION: We present the case of an 11-year-old Hispanic girl with Klippel-Trenaunay-Weber syndrome that developed disseminated intravascular coagulation after minor surgery, which was controlled by blood product transfusions and enoxaparin to address an ongoing consumptive coagulopathy. The patient, however, developed bacteremia and liver trauma that resulted in severe bleeding. To the best of our knowledge, we report here the first known instance of administering recombinant coagulation factor VIIa to control acute bleeding in a patient with Klippel-Trenaunay-Weber syndrome. CONCLUSIONS: This case illustrates the concept of enoxaparin maintenance to suppress an ongoing consumptive coagulopathy and the use of recombinant coagulation factor VIIa to control its potentially fatal severe bleeding episodes.
Asunto(s)
Infecciones por Virus de Epstein-Barr/complicaciones , Intestino Delgado/trasplante , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/cirugía , Preescolar , Humanos , Huésped Inmunocomprometido , Trastornos Linfoproliferativos/terapia , Masculino , Complicaciones Posoperatorias , Reoperación , Factores de Riesgo , Insuficiencia del Tratamiento , Resultado del TratamientoRESUMEN
We are reporting the first known case of sequential combined living donor liver/small bowel transplantation (LDL/SBT). A 2-year-old boy born with gastroschisis and intestinal malrotation lost his entire small bowel and colon shortly after birth. He underwent a living donor small bowel transplant at 1 year of age that was lost 4 months after implantation for posttransplant lymphoproliferative disease (PTLD). He recovered from PTLD but developed total parenteral nutrition (TPN)-induced liver failure. He received a combined left lateral liver and terminal ileum transplant that we chose to perform sequentially due to the presence of preformed antibodies against his mother's tissues. The mother had no complications and a cumulative hospital stay of 7 days. At 9 months postsurgery, the patient is on full enteral nutrition and has suffered neither technical complications nor rejection. The technique described here is reproducible and makes combined living donor LDL/SBT an alternative to combined cadaveric liver-small bowel transplant.