The challenges of diagnosis and management of pregnancy associated with neurofibromatosis and Becker nevus syndrome. Report of a unique clinical case.

Introduction. Neurofibromatosis is a multiorgan affecting genetic disorder with multiple implication in a pregnancy course. Most frequent pregnancy-related complications in this background are first-trimester spontaneous abortion, stillbirth, intrauterine growth restriction and a high rate of caesarean section. Becker nevus syndrome is typically characterized by the presence of circumscribed cutaneous hamartoma, hyperpigmented and hypertrichotic, along with unilateral breast hypoplasia, muscle, skin and/or skeletal abnormalities. The association between these two disorders is an extremely rare event. Moreover, pregnancy in the context of these two disorders overlapped is an exceptional entity. Case presentation. We present the case of a 24-year-old primiparous gravida, 25 weeks of gestation, who was admitted to our clinic for a multidisciplinary approach in the context of severe deformation of the cervical-thoracic-lumbar spine, in “C” shape, associated with multiple café-au-lait spots, suggestive for neurofibromatosis type 1, and an asymptomatic large hyperpigmented and hypertrichotic lesion in the lumbar region, clinically indicating Becker nevus syndrome. Conclusions. The course of this case was clearly a challenge, being a unique case in terms of reports in the specialized literature, both nationally and internationally. [ABSTRACT FROM AUTHOR]