Anesthesia and Von Hippel-Lindau disease. Case report
Keywords:
multimodal general anesthesia, von Hippel-Lindau DiseaseAbstract
Introduction: the management of genetic diseases is challenging because of their low incidence, diverse etiology, coexisting diseases, and variability in responses to anesthetics. This case presentation provides new insight into how to approach multimodal general anesthetic management in patients with Von Hippel-Lindau disease.Patient information: a 49-year-old female patient is presented for orthopedic surgery with open tibial wound and history of bronchial asthma and Von Hippel-Lindau disease, operated for cerebellar hemangioblastoma and renal carcinoma, with current left meningeal and cerebellar hemangioblastoma with clinical and imaging follow-up, renal and pancreatic cysts, right adrenal nodular lesion of recent appearance (ruling out the possibility of pheochromocytoma) and recent migraine headache unrelated to endocranial hypertension despite the mass effect of the cerebellar tumor. Multimodal general anesthetic management was performed.
Conclusions: The possibility of performing multimodal minimally invasive techniques in the surgical intervention of a high surgical risk patient with Von Hippel-Lindau disease with open tibial wound can be considered in order to minimize transoperative morbidity and mortality and to avoid complications.
Downloads
References
1.García Balaguer S, Arteche Arnaiz I, Elgezabal Gomez M, García-Serrano Fuertes E, Lopez Maseda A, Garmendia Zabaleta A, Zapico Guillermo G. Enfermedad de Von Hippel-Lindau: sus manifestaciones más frecuentes y cómo reconocerlas. SERAM [Internet]. 2022 [citado 30/06/2022];1(1):[aprox. 12 p.]. Disponible en: https://piper.espacio-seram.com/index.php/seram/article/view/8820
2.Calle X, Jiménez-Gallegos D, Muñoz-Córdova F, Sánchez P, Lavandero S. Mecanismo sensor y de adaptación a los niveles de oxígeno y su implicancia en las enfermedades cardiovasculares: a propósito del Premio Nobel de Fisiología-Medicina 2019. Rev Chil Cardiol [Internet]. 2019 [citado 24/05/2021];38(3):225-235. Disponible en: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0718-85602019000300225&lng=es. http://dx.doi.org/10.4067/S0718-85602019000300225
3.MedlinePlus [Internet]. Bethesda: National Library of Medicine; 2021 [actualizado 25/04/2018; citado 24/05/2021]. Von Hippel-Lindau Disease; [aprox. 2 pantallas]. Disponible en: https://medlineplus.gov/vonhippellindaudisease.html
4.Instituto Nacional del Cáncer [Internet]. Bethesda: NCI; 2022 [actualizado 24/06/2022; citado 30/06/2022]. Enfermedad de Von Hippel-Lindau (PDQ®)–Versión para profesionales de salud; [aprox. 38 pantallas]. Disponible en: https://www.cancer.gov/espanol/tipos/rinon/pro/genetica-del-carcinoma-de-celulas-renales/enfermedad-de-vhl
5.Sala-Hernández Á, Montalvá-Orón EM, Pareja-Ibars E, Ballester-Pla N, López-Andújar R. Manejo del gastrinoma pancreático asociado a la enfermedad de Von Hippel-Lindau. A propósito de un caso. Rev Esp Enferm Dig [Internet]. 2017 [citado 24/05/2021];109(2):154-157. Disponible en: https://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082017000200015&lng=es&nrm=iso&tlng=es. https://dx.doi.org/10.17235/reed.2016.4224/2016
6.Salazar R, González-Castaño C, Rozas P, Castro J. Hemangioma capilar retiniano y enfermedad de von Hippel-Lindau: implicaciones diagnósticas y terapéuticas. Arch Soc Esp Oftalmol [Internet]. 2011 [citado 24/05/2021];86(7):218-221. Disponible en: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0365-66912011000700004&lng=es
7.Fons Martínez MR, España Gregori E, Aviñó Martínez JA, Hernández Pardines F. Angioma retiniano como síndrome mascarada de tumor del nervio óptico en la enfermedad de Von Hippel-Lindau. Arch Soc Esp Oftalmol [Internet]. 2006 [citado 24/05/2021];81(5):293-296. Disponible en: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0365-66912006000500009&lng=es
8.Villar Gómez de las Heras K. Descripción de la enfermedad de von Hippel-Lindau en tres familias españolas. An Med Interna (Madrid) [Internet]. 2002 [citado 24/05/2021];19(7):34-38. Disponible en: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0212-71992002000700005&lng=es
9.Vallejo Desviat P, Anguita Martínez G, Villar Banco I, Molina López-Nava P. Múltiples imágenes nodulares hipervasculares. Sanidad Mil [Internet]. 2018 [citado 24/05/2021];74(1):47-48. Disponible en: https://scielo.isciii.es/pdf/sm/v74n1/1887-8571-sm-74-01-00047.pdf
10.Regato Avilés M, Cimadevilla Calvo B, Rábago Moriyón JL, Ruiz Andrés N, Del Olmo Ruiloba P. Manejo anestésico de feocromocitoma en paciente con enfermedad de Von Hippel-Lindau. Rev Chil Anest [Internet]. 2022 [citado 30/06/2022];51(3):335-338. Disponible en: https://revistachilenadeanestesia.cl/PII/revchilanestv5122031222.pdf. https://doi.org/10.25237/revchilanestv5122031222
11.Ene CI, Marton RP, Ferreira M Jr, Sekhar LN, Kim LJ. Spontaneous hemorrhage from central nervous system hemangioblastomas. World Neurosurg [Internet]. 2015 [citado 30/06/2022];83(6):1180.e13-7. Disponible en: https://pubmed.ncbi.nlm.nih.gov/25727302/. https://doi.org/10.1016/j.wneu.2015.02.009
12.Shoseyov D, Cohen-Cymberknoh M, Wilschanski M. Ataluren for the treatment of cystic fibrosis. Expert Rev Respir Med [Internet]. 2016 [citado 30/06/2022];10(4):387-391. Disponible en: https://www.tandfonline.com/doi/full/10.1586/17476348.2016.1150181?scroll=top&needAccess=true. https://doi.org/10.1586/17476348.2016.1150181
13.Jito J, Nozaki K. Treatment strategies for cerebellar hemangioblastomas: simple or further studies? World Neurosurg [Internet]. 2014 [citado 30/06/2022];82(5):619-620. Disponible en: https://www.sciencedirect.com/science/article/abs/pii/S1878875014007396. https://doi.org/10.1016/j.wneu.2014.08.018
14.Richards S, Aziz N, Bale S, Bick D, Das S, Gastier-Foster J, et al. Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology. Genet Med [Internet]. 2015 [citado 30/06/2022];17(5):405–424. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4544753/. https://doi.org/10.1038/gim.2015.30
15.Binderup ML, Budtz-Jørgensen E, Bisgaard ML. Risk of new tumors in von Hippel-Lindau patients depends on age and genotype. Genet Med [Internet]. 2016 [citado 30/06/2022];18(1):89-97. Disponible en: https://pubmed.ncbi.nlm.nih.gov/25834951/. https://doi.org/10.1038/gim.2015.44
Downloads
Published
How to Cite
Issue
Section
License
Authors who have publications with this journal agree to the following terms:
- Authors will retain their copyright and assign to the journal the right of first publication of their work, which will simultaneously be subject to a Creative Commons License / Attribution-Noncommercial 4.0 International (CC BY-NC 4.0) that allows third parties to share the work as long as its author and first publication in this journal are indicated.
- Authors may adopt other non-exclusive license agreements for distribution of the published version of the work (e.g., depositing it in an institutional repository or publishing it in a monographic volume) as long as the initial publication in this journal is indicated.
- Authors are allowed and encouraged to disseminate their work through the Internet (e.g., in institutional telematic archives or on their web page) before and during the submission process, which can produce interesting exchanges and increase citations of the published work. (See The effect of open access).
15 junio 2025
