Background: VACTERL association is a rare condition that can present with vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. The most common limb deformities affect the upper limbs and may include shortened long bones, abnormal bone structure, and aplasia of bones. Although limb and spinal abnormalities related to VACTERL association have been documented, there are no current reports on the acute management of fractures caused by trauma. Fractures of the deformed bones can cause orthopedic challenges regarding fixation methods. We present a case of a patient with VACTERL association who sustained a right intraarticular distal humerus fracture involving the capitellum and a displaced olecranon fracture.
Case Presentation: A 42-year-old man with a known diagnosis of VACTERL association presented to the emergency department after sustaining a ground level fall. Imaging showed a right coronal shear-type capitellum distal humerus fracture and displaced olecranon fracture. Characteristics of VACTERL association were present, including an absent radius, absent carpal bones, and limb length abnormalities. A posterior approach was performed and ulnar nerve was identified and protected carefully as no retroepicondylar groove was present. The coronoid fragment was managed with two mini-fragment screws and the olecranon fracture was managed with a mini-fragment plate, contoured to fit the abnormal anatomy of the ulna, secured with non-locking screws. At three months post-op, radiographs demonstrated complete healing.
Conclusion: Patients with elbow fractures and VACTERL syndrome can be treated successfully with operative fixation. However, they do have some anatomic variation that requires attention including nerves and vasculature with atypical presentation. Specifically, the ulnar nerve does not sit within the retroepicondylar groove and the double coronoid process must be noted. Lastly, postoperative care may need to be modified given anatomic and functional differences.
Keywords: VACTERL; Olecranon fracture; Coracoid fracture
