Bow Hunter's Syndrome with Rotational Atlantoaxial Instability: A Rare Association

Bow Hunter's syndrome (BHS) is a very rare condition in which there is rotational vertebral artery (VA) insufficiency. The association of BHS with rotational atlantoaxial instability is extremely rare. We are reporting a case of pediatric BHS who presented with features of VA insufficiency on neck rotation. Careful evaluation revealed rotational C1-C2 instability. Provocative digital subtraction angiography and dynamic neck computed tomography were the mainstay of our diagnostic armamentarium. Our case emphasizes the fact that VA abnormalities need special consideration in young patients with craniovertebral junction instability and a high degree of suspicion is necessary in most instances for accurate diagnosis.