Feeding challenges at initiation and maintenance of the ketogenic diet in children with infantile epileptic spasms syndrome: 20 years single center experience

RATIONALE: Infants with Infantile Epileptic Spasms Syndrome (IESS) are at high risk for feeding challenges such as aspiration. These vulnerabilities may be exacerbated by the ketogenic diet (KD), an accepted second-line therapy. Hormonal therapy (ACTH or oral corticosteroids), the first-line IESS treatment, may transiently enhance appetite and improve oral feeding performance, and hence mask feeding challenges. METHODS: A retrospective chart review was conducted for infants with IESS who initiated KD between January 2005 and January 2025 at our center. Collected data included ages at diagnosis and KD initiation, comorbidities, clinical and video fluoroscopic swallow study (VFSS) assessments, timing relative to hormonal therapy, feeding method before and during KD initiation, and incidence of feeding concerns during KD. Feeding challenges included aspiration, confirmed by VFSS or occupational therapy clinical assessment of feeding (OCTA), or inadequate oral intake. RESULTS: Forty infants were enrolled. Twelve (30%) had previous aspiration reported in the medical charts or VFSS at baseline; nine were tube-fed prior to KD. Of the 31 (31/40, 77.5%) orally fed infants at initiation of KD, 23 were referred for feeding safety evaluation. Twelve of these had a feeding concern found on OTCA and/or VFSS, of which 3 presented with aspiration without a protective cough confirmed by VFSS. Nineteen babies were deemed safe to initiate KD orally; however, 5/19 (26%, 95% CI 11.8%-48.7%) went on to experience aspiration or aspiration pneumonia during KD treatment. CONCLUSION: Feeding challenges are frequent, dynamic, and under-recognized in IESS. Comprehensive feeding evaluation at KD initiation and maintenance is essential for safe treatment.