Multidisciplinary management of pregnancy complicated by extensive Sturge–Weber syndrome with previously unrecognized spinal vascular involvement: a case report
This case report highlights the critical importance of comprehensive multidisciplinary evaluation and individualized neurovascular assessment, including unexpected spinal imaging, to guide safe anesthetic and delivery planning for pregnant women with extensive Sturge–Weber syndrome and previously unrecognized spinal vascular involvement.
Original paper licensed under CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). This is an AI-generated explanation of the paper below. It is not written or endorsed by the authors. For technical accuracy, refer to the original paper. Read full disclaimer
Pregnancy is a time of profound physical change, where the body adapts to support a growing life. For most, these shifts are manageable, but for those with rare vascular disorders, the added blood flow and pressure can turn a routine event into a high-stakes medical challenge. One such condition is Sturge–Weber syndrome, a rare disorder present from birth where abnormal blood vessels form on the face, inside the skull, and sometimes elsewhere in the body. These vessels are fragile and prone to bleeding or causing seizures. While doctors understand how to manage this condition in children and adults, there is very little guidance on how to safely guide a woman with this syndrome through childbirth. The core question for medical teams is not just how to deliver the baby, but how to do so without triggering a dangerous bleed in the mother's brain or spine, or causing a seizure that could harm both.
A team of doctors at Shandong University recently shared the story of a 27-year-old woman who faced this exact dilemma. She carried the full weight of extensive Sturge–Weber syndrome, with a network of abnormal blood vessels covering her face, mouth, nose, trunk, and limbs. She also had a history of seizures, which were well-controlled with medication, and had lost sight in one eye due to complications from the disorder. Her pregnancy had proceeded smoothly, with no seizures and a healthy baby, but as her due date approached, the medical team faced a critical uncertainty. They knew her brain contained abnormal vessels, but they did not know if similar hidden dangers existed in her spine. This uncertainty was the pivot point for her entire care plan.
In standard obstetric care, a woman in labor might receive an epidural, a form of anesthesia injected into the lower back to numb pain. For a woman with Sturge–Weber syndrome, this procedure carries a specific risk: if abnormal blood vessels are present in the spine, the needle could puncture them, causing a dangerous bleed or nerve damage. Because this woman's condition was so widespread, the doctors could not assume her spine was safe based on her brain scans alone. To solve this, a large group of specialists—including experts in pregnancy, anesthesia, brain surgery, eye care, and intensive care—gathered to map out a strategy. They decided to perform a full scan of her entire spine, a step that is not routine for every patient but was deemed necessary here due to the extent of her visible vascular issues.
The scan revealed a surprise that changed everything. Hidden deep within her lower back, extending from the fourth lumbar vertebra down to the sacral canal, was a dense cluster of blood vessels. This finding meant that the spine was not safe for an epidural or spinal anesthesia. The risk of causing a hemorrhage or neurological injury was too high. This discovery forced the team to abandon the idea of a vaginal delivery with pain relief from the back, which is common for many women. Instead, they planned a scheduled cesarean section, a surgical delivery, to be performed under general anesthesia, where the patient is fully asleep and the airway is managed directly.
The surgery took place when the woman was 40 weeks and one day pregnant. The medical team prepared meticulously, anticipating that her facial and nasal blood vessel abnormalities might make breathing difficult during the procedure. They used invasive monitoring to watch her blood pressure and heart function closely, ensuring that no sudden spikes occurred that could stress her fragile vessels. The operation went smoothly. A healthy baby boy weighing 3000 grams was born, scoring perfectly on the standard newborn health checks. The mother remained stable throughout, with no seizures, no bleeding from the airway, and no sudden changes in her blood pressure. She spent a few days in the intensive care unit for close observation before recovering fully.
Six weeks after the birth, the mother and baby were both doing well. The mother had not experienced any seizures, and her neurological status remained stable. This successful outcome was not a matter of luck but of careful, individualized planning. The case demonstrated that for women with extensive vascular disorders, the diagnosis alone does not dictate the best path forward. Instead, the specific location and spread of the abnormal vessels must guide the decision. In this instance, the unexpected discovery of spinal involvement ruled out the most common pain relief methods and pointed toward a surgical delivery under general anesthesia.
The doctors noted that while this approach worked for this patient, it does not mean every woman with this syndrome needs a spinal scan or a cesarean section. The key takeaway is that when a patient has widespread vascular issues that go beyond the typical patterns, a thorough check of the entire nervous system is essential before delivery. By bringing together experts from many fields and using advanced imaging to look for hidden risks, the team was able to navigate a complex situation that had no clear textbook answer. This case serves as a reminder that in medicine, especially with rare conditions, the safest path is often found by looking deeper than the obvious symptoms and tailoring the plan to the unique anatomy of the individual.
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