Bilateral Infraclavicular Block in a Pediatric Patient with Hurler Scheie Syndrome: A Successful Approach

Dear Editor, I am writing to share our experience with a pediatric patient diagnosed with Hurler Scheie syndrome who successfully underwent bilateral carpal tunnel surgery under bilateral infraclavicular block. This case underscores the utility of regional anesthesia as a viable and safe alternative in patients with difficult airways, particularly when general anesthesia poses significant risks. Hurler Scheie syndrome, a subtype of mucopolysaccharidosis, is an autosomal recessive metabolic disorder characterized by a deficiency in the lysosomal enzyme alpha-L-iduronidase. This condition results in progressive systemic involvement and high pediatric mortality (1,2). Patients often require surgical interventions, such as those for carpal tunnel syndrome, but airway abnormalities—including macrocephaly, restricted neck mobility, and short mandibular distances—make airway management challenging (1). The patient, a 13-year-old male weighing 40 kg, had a history of failed intubation attempts during a previous surgical procedure due to difficult airway features. Written and verbal informed consent was obtained from the patient and their parents prior to the procedure. For the current bilateral carpal tunnel surgery, we opted for an ultrasound-guided bilateral infraclavicular block to minimize the risks associated with general anesthesia. The procedure was conducted using a solution mixture of 0.25% bupivacaine (1 mg/kg) and 0.5% lidocaine (2 mg/kg) (Figure 1). The patient remained awake and pain-free throughout the surgery, which lasted 45 minutes. No complications were observedintraoperatively or postoperatively. Figure 1. Ultrasound view of the block (PM: Pectoralis major, Pm: Pectoralis minor, LA: Local anesthetic). This case highlights the critical role of regional anesthesia techniques in managing pediatric patients with difficult airways. Advances in ultrasound technology and nerve stimulation have significantly enhanced the safety and efficacy of regional anesthesia, particularly in children (3). Studies have shown that peripheral nerve blocks can be performed without sedation in select pediatric patients, further reducing risks associated with general anesthesia (4). We believe this case demonstrates the importance of considering regional anesthesia as a primary approach in patients with difficult airways or where general anesthesia is contraindicated. By sharing this experience, we hope to encourage further discussion and exploration of regional techniques in similar high-risk scenarios.