Abstract: Introduction: Neurofibromatosis is an Autosomal dominant disease characterized by different types of mutations of the NF-1 gene1, 2. approximately 50% of the NF-1 gene mutations result from de novo mutations. The reported incidence of neurofibromatosis (NF) in pregnancy varies from 1:2500-1:3500.
Case Report: A 31-year-old G3P1L1A1,31+5 weeks of gestation, Prev caesarean, hypothyroidism, breech, known case of neurofibromatosis 1 presented with complaints of headache to the emergency with a BP of 160/98 mm of Hg. USG showed fetoplacental insufficiency with doppler changes showing a cerebroplacental ratio <1. Blood pressure was not controlled on IV antihypertensives and MgSO4.
Decision of emergency caesarean section was taken in view of uncontrolled hypertension, IUGR, doppler changes.
Conclusion: These patients are at higher incidence of miscarriages, stillbirths, pre-eclampsia, IUGR, oligohydramnios, preterm labour, pulmonary stenosis, valvular disease septal defects, coarctation of aorta, cardiomyopathy due to neurocutaneous changes. Associated phaeochromocytoma contribute to hypertension not responding to treatment Patients with neurofibromatosis are advised to undergo genetic, prenatal, and antenatal counselling.
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