Hemoperitoneo espontáneo masivo en paciente puérpera afecta de neurofibromatosis tipo 1 / Massive spontaneous haemoperitoneum in a post-partum patient with neurofibromatosis type 1

La neurofibromatosis tipo 1 o enfermedad de von Recklinghausen es una enfermedad genética, progresiva y multisistémica que afecta predominantemente a la piel y el sistema nervioso. La alteración vascular es poco frecuente pero puede tener resultados catastróficos. Las pacientes gestantes afectas de esta enfermedad necesitan un seguimiento exhaustivo y multidisciplinar con el objetivo de controlar las posibles alteraciones vasculares sobre todo renales por ser las más frecuentes. Presentamos el caso de una paciente diagnosticada de NF-1 que presentó un hemoperitoneo masivo espontáneo en el postoperatorio tardío de una cesárea electiva. El sangrado espontáneo de grandes vasos es una rara pero potencial complicación letal que puede producirse en pacientes afectos de neurofibromatosis I y que puede precisar de tratamiento quirúrgico urgente

Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease is a genetic, progressive, multi-system disease that predominantly affects the skin and nervous system. Vascular involvement is rare, but can have catastrophic results. Pregnant patients with this disease need careful, multidisciplinary follow up in order to control possible vascular alterations, which usually affect the kidneys. We present the case of a patient diagnosed with NF-1 who debuted with massive spontaneous hemoperitoneum in the late postoperative period of an elective Cesarean section. Spontaneous bleeding from large vessels is a rare but potentially lethal complication that can occur in patients with NF-1, and may require urgent surgical treatment

Massive spontaneous haemoperitoneum in a post-partum patient with neurofibromatosis type 1. / Hemoperitoneo espontáneo masivo en paciente puérpera afecta de neurofibromatosis tipo 1.

Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease is a genetic, progressive, multi-system disease that predominantly affects the skin and nervous system. Vascular involvement is rare, but can have catastrophic results. Pregnant patients with this disease need careful, multidisciplinary follow up in order to control possible vascular alterations, which usually affect the kidneys. We present the case of a patient diagnosed with NF-1 who debuted with massive spontaneous hemoperitoneum in the late postoperative period of an elective Cesarean section. Spontaneous bleeding from large vessels is a rare but potentially lethal complication that can occur in patients with NF-1, and may require urgent surgical treatment.