2022, Number 1
Neurofibromatosis type 1 or Von Recklinghausen´s disease
Language: Spanish
References: 7
Page: 1-9
PDF size: 293.91 Kb.
ABSTRACT
Introduction: Von Recklinghausen's disease or neurofibromatosis type I is a autosomal dominant inheritance condition with varied clinical expressions, its manifestations range from café-au-laittype spots on the skin to severe aesthetic and functional complications that can also affect other organs, bone and nerve tissues.Objective: To preport the aesthetic and functional results achieved with the surgical intervention of a case with neurofibromatosis type I with large lesions.
Case report: We report a case treated in Gaborone, Republic of Botswana by a multidisciplinary team including a plastic surgeon, a neurosurgeon and a pathologist, all from the Republic of Cuba. The patient was treated, on that occasion, for skin neurofibromas and she reported a family history of the disease. Bone changes were found due to an exaggerated syphosis and with no intellect damage. She underwent surgery for a major back injury.
Conclusions: The aesthetic and functional results of surgical treatment in extensive lesions, in the course of neurofibromatosis, can be successful if correct assessment is carried out by a multidisciplinary group. The group can choose the most appropriate surgical treatment regardless of the size of the lesion.
REFERENCES
Ramos Díaz N, Adefna Pérez R I, Izquierdo Lara FT, Pulido Prieto Y L, Lara Fernández N.Neurofibromatosis sin neurofibromas con tumor mediastinal como forma de debut. Rev. cuba. cir. 2017 [acceso 18/12/2021];56(3):1-7. Disponible en:http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-74932017000300009&lng=es
Javed F, Ramalingam S, Bashir Ahmed H, Gupta B, Sundar Ch, Qadri T, et al. Oralmanifestations in patients with neurofibromatosis type-1: A comprehensive literature review.Critical Rev Oncol Hematol. 2014 [acceso 18/12/2021];91(2). Disponible en:http://fawadjaved.com/wpcontent/themes/fawad/downloads/Javed%20et%20al%202014%20(NF%20&% 20Oral%20health).pdf