One-bone forearm procedure for Hajdu-Cheney syndrome: a case report

Area of Science:

• Orthopedic Surgery
• Genetics
• Reconstructive Surgery

Background:

• Longitudinal forearm instability presents a significant challenge, often requiring salvage procedures.
• Hajdu-Cheney syndrome is a rare genetic disorder causing generalized ligamentous laxity and skeletal abnormalities.
• One-bone forearm (OBF) surgery is a reconstructive option for severe instability.

Purpose of the Study:

• To evaluate the long-term outcomes of one-bone forearm surgery in a patient with Hajdu-Cheney syndrome.
• To assess the efficacy of OBF as a salvage procedure for rare genetic etiologies of forearm instability.

Main Methods:

• A 23-year-old patient with Hajdu-Cheney syndrome underwent one-bone forearm surgery.
• The patient's functional outcome and satisfaction were assessed over an eight-year follow-up period.
• Overuse shoulder pain was managed conservatively post-surgery.

Main Results:

• The patient reported no pain and complete satisfaction eight years after OBF.
• A Quick Disabilities of the Arm Shoulder and Hand (QDASH) score of 21 was recorded at eight years.
• No additional procedures were required during the eight-year follow-up.

Conclusions:

• One-bone forearm surgery can be a viable and durable salvage option for forearm instability, even in rare genetic conditions like Hajdu-Cheney syndrome.
• Careful patient selection and consideration of OBF in unique circumstances can lead to predictable and acceptable long-term results.
• OBF offers a potentially effective solution for complex forearm instability cases refractory to other treatments.