Case Report: Rare Case of NF2 in Pregnancy with Favorable Maternal and Perinatal Outcome, Under General Anesthesia Caesarean Section

Abstract

Background: Neurofibromatosis type 2 (NF2) is an autosomal dominant disease due to chromosome 22 (22q12.2) mutation with a prevalence of 1:33,000-40,000. As many as 50% of cases are familial, 30-60% are genetic mosaic, de novo mutations. Tumor growth in NF often becomes more rapid in pregnancy. Pregnancy with NF2 has a risk of complications of hypertension, preeclampsia, cardiovascular problems, and IUGR (Intrauterine growth restriction). Case Reports: A 26-year-old woman, 29/30 weeks gestational age complaint of visual impairment, hearing loss, and multiple body masses. The results of MRI examination of the Head and whole Spine with contrast showed bilateral vestibular schwannoma, meningiomatosis, cervical schwannoma (C1-C5), which were features of NF2. Routine clinical evaluation, laboratory tests, fetal growth examination, and multidisciplinary management were performed during the antenatal care. There were no signs of increased intracranial pressure, hydrocephalus, and complications of preeclampsia, fetal abnormalities, or IUGR. A termination at 37 weeks of gestation with Caesarean Section and General Anesthesia  was decided to reduce the increase of intracranial pressure during labor. A male infant was born with a birth weight of 2600 grams, without any abnormalities.  Conclusion: Pregnancy in NF2 patient has a potential risk of tumor growth progression, preeclampsia, and IUGR,  therefore it is necessary to monitor and evaluate the mother and fetus multidisciplinary during the pregnancy process to get good maternal and fetal outcomes. The mode of delivery in neurofibromatosis is determined mainly by obstetric indications.