Neurofibromatosis (NF1) is an autosomal dominant disorder
primarily affecting the development and growth of nerve cell tissues. We report
a rare case of pregnancy with giant plexiform neurofibroma involving the genital
tract with adverse perinatal outcome. A 26-year-old female presented at term in
labour with NF1 and a huge plexiform neurofibroma arising from sacral region, gluteal
region, pelvis and involving left labium majus. She delivered by cesarean section
and the baby had congenital NF1. The baby expired on day 4 of life. To the best
of our knowledge, this large size neurofibroma involving this site in pregnancy
has never been reported in English literature. Both pregnancy and NF1 have reciprocal
adverse effects on each other. Congenital neurofibromatosis can be fatal. Hence,
all pregnancies associated with NF1 should be considered as high risk and monitored
carefully.
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