ABSTRACT
Von Hippel-Lindau disease is a dominant autosomal genetic condition with variable penetrance and expressivity. It is characterized by hemangioblastomas in multiple organs but mainly in the retina and cerebellum. There is a predisposition to carcinoma. We report a cesarean section in a 28-year-old woman with von Hippel-Lindau disease. She had no neurologic symptoms at the time of the operation but a history of ocular and cerebellar involvement and several procedures to remove cerebellar hemangioblastomas. Epidural anesthesia was chosen given that there was no nervous system involvement at the time of surgery.
Subject(s)
Anesthesia, Epidural/methods , Anesthesia, Obstetrical/methods , Cesarean Section, Repeat , Pregnancy Complications , Pregnancy, High-Risk , von Hippel-Lindau Disease , Adult , Cerebellar Neoplasms/genetics , Cerebellar Neoplasms/surgery , Diabetes, Gestational , Female , Hemangioblastoma/genetics , Hemangioblastoma/surgery , Hemangioma/genetics , Hemangioma/surgery , Humans , Infant, Newborn , Laser Coagulation , Male , Pregnancy , Pregnancy Complications/genetics , Radiosurgery , Reoperation , Retinal Neoplasms/genetics , Retinal Neoplasms/surgery , Survivors , von Hippel-Lindau Disease/geneticsABSTRACT
La enfermedad de von Hippel Lindau es un transtornogenético autosómico dominante con penetranciavariable, caracterizado por hemangioblastomas multiorgánicosprincipalmente en la retina y cerebelo y con predisposicióna desarrollar carcinoma. Se comunica el casode una gestante a término de 28 años de edad, portadorade la enfermedad de von Hippel Lindau, sin clínicaneurológica en el momento de la cesárea, con historia deafectación ocular y cerebelosa, a quien se le habían extirpadohemangioblastomas cerebelosos en varias ocasiones.Se eligió anestesia epidural por no presentar afectacióndel sistema nervioso, en el momento de la cirugía(AU)
Von Hippel-Lindau disease is a dominant autosomalgenetic condition with variable penetrance and expre -ssivity. It is characterized by hemangioblastomas inmultiple organs but mainly in the retina and cerebellum.There is a predisposition to carcinoma. We report acesarean section in a 28-year-old woman with von Hippel-Lindau disease. She had no neurologic symptoms at thetime of the operation but a history of ocular and cerebellarinvolvement and several procedures to remove cerebellarhemangioblastomas. Epidural anesthesia was chosen giventhat there was no nervous system involvement at the timeof surgery(AU)
