Index Pollicization with Subsequent Development of Scaphoid AVN

Background: Preiser's disease, or idiopathic scaphoid avascular necrosis (AVN), is a rare ischemic event of the scaphoid occurring in patients with no prior history of wrist injury. Blood flow to the scaphoid originates distally from the radial artery and proximally; thus, many cases of Presier's disease present with proximal pole ischemia. Holt Oram syndrome is an autosomal dominant disease that presents with cardiac and upper appendicular skeletal deformities, namely of carpal and thenar origin. Carpal malformations include scaphoid hypoplasia and bipartite ossification, retention of the os centrale in utero, and the addition of extra carpal bones or other carpal anomalies, while thumb malformations present with triphalangeal, hypoplastic, or absent thumbs. Scaphoid AVN has only ever been reported in patients presenting with Holt Oram syndrome in cases of trauma. Case Information: A 38-year-old female with a history of index finger pollicization in 1983 for congenital thumb agenesis from Holt-Oram syndrome presented to the clinic for evaluation of radial and ulnar sided wrist pain for one year that bothered her daily. She presented with an MRI that demonstrated avascular necrosis of the scaphoid. Physical examination demonstrated intact pollicization of the index finger with well-healed incisions. Pain located to the scaphoid on flexion and extension of the wrist. Exam was positive for snuffbox tenderness. Patient demonstrated adequate range of motion in all digits. The patient elected to proceed with scaphoid excision and four-corner fusion after conservative therapies had been exhausted. After 2 months of short arm immobilization, fusion was demonstrated on X-ray, and the patient was pain-free on exam with adequate composite fist formation. Conclusions: For patients with thumb absence or hypoplasia, index pollicization is considered the ideal treatment, as it positions a functioning finger in place of the lacking thumb. There is no mention in the literature of scaphoid AVN as a long-term complication of index pollicization. Treatment of scaphoid AVN in this patient with index pollicization showed similar outcomes to those treated with isolated scaphoid AVN. To our knowledge, this is the first reported case of scaphoid AVN in a patient with a history of index finger pollicization. In this case, treating scaphoid AVN in the traditional fashion has led to a satisfactory outcome in regards to both pain control and function.