Neurocutaneous Disorders in Pregnancy

Obstet Gynecol Surv. 2023 Oct;78(10):606-619. doi: 10.1097/OGX.0000000000001202.


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.

Publication types

  • Review

MeSH terms

  • Female
  • Humans
  • Infant, Newborn
  • Neurocutaneous Syndromes* / complications
  • Neurocutaneous Syndromes* / diagnosis
  • Neurocutaneous Syndromes* / therapy
  • Neurofibromatosis 1* / complications
  • Pregnancy
  • Tuberous Sclerosis* / complications
  • Tuberous Sclerosis* / diagnosis
  • von Hippel-Lindau Disease* / complications
  • von Hippel-Lindau Disease* / diagnosis