Importance 

Neurocutaneous disorders have significant implications for care of the pregnant patient. As neurocutaneous disorders are uncommon, obstetricians may be unfamiliar with these disorders and with recommendations for appropriate care of this population.

Objective 

This review aims to summarize existing literature on the interaction between neurocutaneous disorders and pregnancy and to provide a guide for physicians caring for an affected patient.

Evidence Acquisition 

A PubMed, MEDLINE, and Google Scholar search was carried out with a broad range of combinations of the medical subject headings (MeSH) terms “pregnancy,” “Sturge -Weber,” “Neurofibromatosis Type 1,” “neurofibromatosis type 2,” “von Hippel Lindau,” “Tuberous Sclerosis,” “neurocutaneous disorder,” “treatment,” “congenital malformations,” “neurodevelopmental defects,” “miscarriage,” “breastfeeding,” “autoimmune,” “pathophysiology,” and “management.” References of included articles were searched to identify any articles that may have been missed after the above method was used.

Results 

Neurocutaneous disorders are associated with increased pregnancy-associated maternal and fetal/neonatal morbidity, largely surrounding hypertensive disorders, epilepsy, and medication exposure. Some features of neurocutaneous disorders may be worsened or accelerated by pregnancy. Neurocutaneous disorders can often be diagnosed prenatally. Therefore, directed assessment should be offered to affected individuals with a personal or family history of a neurocutaneous disorder.

Conclusion and Relevance 

Patients affected by neurocutaneous disorders who are pregnant or planning for future pregnancy should be carefully followed by a multidisciplinary team, which could include maternal-fetal medicine, neurology, and anesthesia, as well as other relevant subspecialists. Additional research is required regarding optimal counseling and management of these patients.

Target Audience 

Obstetricians and gynecologists, family physician.

Learning objectives 

After completing this activity, the learner will be better able to identify the most common neurocutaneous disorders seen in reproductive women and their implications in pregnancy; propose recommendations for genetic evaluation, diagnosis, management, and a differential diagnosis; describe treatment options including labor and delivery management, emphasizing multidisciplinary approach; and discuss potential maternal and fetal adverse outcomes related to neurocutaneous disorders.