Bernard-Soulier syndrome (BSS), a rare autosomal recessive disorder first identified in 1948, is characterized by excessive and prolonged bleeding due to thrombocytopenia and platelet dysfunction with increased platelet size and deformability. The primary defect in BSS involves the glycoprotein (GP) Ib-IX-V complex, which is important in initiating platelet aggregation and thrombosis after vascular injury by facilitating the adhesion of platelets to von Willebrand factor. The coagulation defect in BSS can lead to significant bleeding during traumatic injury or surgical intervention. We present a 17-year-old adolescent with BSS who presented for posterior spinal fusion for idiopathic scoliosis. Previous reports of perioperative care of patients with BSS are reviewed and options for anesthetic care including perioperative control of the coagulation defect are presented.

Initial clinic evaluation among referred patients and factors limiting treatment initiation are not well characterized. We conducted a retrospective review of referrals to our outpatient pain clinic to identify intake visits and factors associated with treatment initiation among adolescents with chronic pain. We identified adolescents aged 13 to 18 years at the time of referral to clinic (2010-2016). Factors associated with completion of visits were evaluated using logistic regression. Patients who completed visits more frequently had private insurance than public or no insurance ( P = .053). The most common reasons for caregiver decision not to attend the pain clinic included use of another pain clinic, that services were not wanted or no longer needed, and that their child was undergoing further testing. The current study demonstrated that patients with head pain were more likely to complete an intake visit, while there was a trend showing that lack of private insurance decreased this likelihood.