Infant Monitoring Devices in Infants With or at Risk for Sturge–Weber Syndrome: Analysis of Clinical Questionnaire Supports Reduced Parent Anxiety

Diagnosis and treatment of Sturge–Weber syndrome (SWS) brain involvement in infants born with a port-wine birthmark who are at risk of seizures requires close neurological monitoring [1, 2]. In 90% of patients with SWS brain involvement, seizures will occur before 2 years of age [2]. Early seizures in patients with SWS are typically focal or partial, prolonged, and often associated with tachycardia. Less commonly, they may present with bradycardia and desaturations, which are often not detectable on seizure-specific devices [3]. There has been a notable increase in parents using infant monitoring devices (IMDs) in the Hunter Nelson Sturge–Weber Center. The present study analyzed parental experience using commercially available IMDs and assessed whether these devices may be useful for detecting clinically diagnosed seizures in patients with SWS. We sent a clinical questionnaire developed in Qualtrics via a secure email link to parents of patients < 5 y of age diagnosed or at risk for SWS and seen between August 31, 2021, and August 31, 2024 (N = 118) at Kennedy Krieger (KK). Twenty-two research-consented patients responded to the questionnaire. We shared the questionnaire with the Vascular Birthmarks Foundation (VBF), who posted it on SurveyMonkey on Facebook pages between February 7, 2025, and April 21, 2025 (N = 37 respondents). See Figures S1a,b for questionnaire and Tables S1a–S2b for demographic information and SWS characteristics. Of the 22 respondents, 10 reported using an IMD. Of the parents whose child had epilepsy and used an IMD, two said they were able to detect a seizure based on the alerts from the device (40%; Figure 1A). Patients who used an IMD were more likely to have known SWS brain involvement (p = 0.024). See Figure S2a,b for the combined and KK full consort diagram and Tables S3 and S4a for the combined parental Likert scale data and KK Fischer's exact results. Ninety percent of KK parents reported that having the reassurance of the device contributed to better sleep patterns for themselves and decreased their anxiety while their infant was sleeping. Most reported that they did not face any financial barriers when purchasing the devices and agreed that the device was easy to set up and would recommend it to another family with an infant at risk of or with SWS (Figure 2A). Of the 37 respondents, 28 reported using an IMD. Among these 28 infants, 18 were known to have SWS brain involvement, and 14 reported a history of epilepsy. Of the 14 parents whose child had a history of epilepsy and used an IMD, 5 said they were able to detect a seizure based on the alerts provided by the device (35.7%; Figure 2B). Among all IMD users, 89% (25/28) reported that the reassurance of the device contributed to better sleep patterns for themselves, and 89% (25/28) stated that the device decreased their anxiety. Only 4% (1/28) reported that the alarms were bothersome, and 68% (19/28) reported that they would recommend the device to another family. In addition, 47% (13/28) reported they faced no financial barriers when purchasing the device. See Figure 1B for VBF IMD consort diagram, Figure S2a,c for full combined and VBF consort diagrams, and Tables S3 and S4b for combined and VBF parental Likert scale data and VBF Fischer's exact results. While the data from the clinical site came from a closely followed patient population, the data from the advocacy site came from participants without a well-characterized clinical presentation. In addition, it is not certain whether the two groups could have generated multiple responses from the same individuals. Each site yielded the same conclusions, supporting broader generalization. Parents reported having an overall positive experience using an IMD. See Figure S4 for the combined parental Likert scale data. This initial study supports the use of IMDs to aid in decreasing anxiety in parents of infants with SWS brain involvement and epilepsy, and the need for future research, including larger and more diverse sample sizes and retrospective and prospective studies, to determine if the use of IMDs in these patients can detect epileptic seizures. Katharine E. Joslyn: writing – original draft, writing–review and editing, data visualization. Ashley N. Eisenberg and Veronica M. Lee: data curation, writing–review and editing. Linda Rozell-Shannon: conceptualization, data curation, writing–review and editing. Anne M. Comi: conceptualization, supervision, writing–review and editing. A.M.C. is an editorial board member of ACNS. The other authors declare no conflicts of interest. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.