INTRODUCTION: Sturge Weber Syndrome (SWS) is a rare congenital neurocutaneous disorder affecting capillary formation, classically presenting with a facial port-wine birthmark and seizures. Associated symptoms vary by individual but may include additional brain and eye manifestations such as cognitive impairment, headache, stroke-like episodes, and glaucoma or visual field defects. Because SWS results from a sporadic mutation, it does not carry specific contraindications to pregnancy regarding fetal risk. However, pregnancies in patients with SWS should be managed by an multidisciplinary team and closely monitored for acute neurological and ophthalmologic complications. In childbirth, previous case reports have favored Cesarean section over vaginal delivery to minimize increases in intracranial pressure on abnormal cerebral or ocular capillaries, potentially worsening vision.
CASE SUMMARY: Here, we present a 21-year-old primigravid woman with known SWS who received routine antepartum care in our OBGYN clinic. At 32 weeks gestation, she was found to have a new serous retinal detachment and an enlarging choroidal hemangioma in her only seeing eye, placing her at high risk for rapid, irreversible vision loss. Given the worsening detachment and her progressive visual symptoms, a multidisciplinary team involving Maternal–Fetal Medicine, Retina, and Ocular Oncology recommended emergent induction of labor at 34 weeks and 3 days to allow expedited postpartum ophthalmologic treatment. She progressed quickly during induction, with a five-minute second stage, and delivered a healthy but premature infant without complications. Outside of her ophthalmologic disease, her pregnancy remained uncomplicated. She was discharged on postpartum day one to attend same-day ophthalmology follow-up to plan radiation treatment not feasible during pregnancy.
CONCLUSION: Progressive choroidal hemangioma in pregnancy is uncommon and poorly described, and vaginal delivery has not yet been utilized as seen in the literature for these patients. Thus, patients with SWS and concurrent ocular pathology require coordinated care involving obstetrics, ophthalmology, and subspecialty teams. This case illustrates that vaginal delivery can be a safe and appropriate option for pregnant patients with vision-threatening retinal detachment and choroidal hemangioma when supported by close multidisciplinary management. This case also supports the potential role of propranolol as an adjunctive agent in reducing labor duration, consistent with reports suggesting it may shorten the second stage of labor.