Accurate diagnosis requires the evaluation of relevant clinical, neurological, and neurophysiological findings along with the careful consideration of other possible diagnoses. High success rates of 44% to 91% are reported after operative treatment. The results are better in idiopathic than in posttraumatic cases. If surgery fails, re-operation is indicated only in patients with inadequate release.
Cubital tunnel syndrome (CuTS) is the second most common peripheral nerve compression syndrome. In German-speaking countries, cubital tunnel syndrome is often referred to as sulcus ulnaris syndrome (retrocondylar groove syndrome). This term is anatomically incorrect, since the site of compression comprises not only the retrocondylar groove but the cubital tunnel, which consists of 3 parts: the retrocondylar groove, partially covered by the cubital tunnel retinaculum (lig. arcuatum or Osborne ligament), the humeroulnar arcade, and the deep flexor/pronator aponeurosis. According to Sunderland , cubital tunnel syndrome can be differentiated into a primary form (including anterior subluxation of the ulnar nerve and compression secondary to the presence of an anconeus epitrochlearis muscle) and a secondary form caused by deformation or other processes of the elbow joint. The clinical diagnosis is usually confirmed by nerve conduction studies. Recently, the use of ultrasound and MRI have become useful diagnostic tools by showing morphological changes in the nerve within the cubital tunnel. A differential diagnosis is essential in atypical cases, and should include such conditions as C8 radiculopathy, Pancoast tumor, and pressure palsy. Conservative treatment (avoiding exposure to external noxes and applying of night splints) may be considered in the early stages of cubital tunnel syndrome. When nonoperative treatment fails, or in patients who present with more advanced clinical findings, such as motor weakness, muscle atrophy, or fixed sensory changes, surgical treatment should be recommended. According to actual randomized controlled studies, the treatment of choice in primary cubital tunnel syndrome is simple in situ decompression, which has to be extended at least 5-6 cm distal to the medial epicondyle and can be performed by an open or endoscopic technique, both under local anesthesia. Simple decompression is also the therapy of choice in uncomplicated ulnar luxation and in most post-traumatic cases and other secondary forms. When the luxation is painful, or when the ulnar nerve actually "snaps" back and forth over the medial epicondyle of the humerus, subcutaneous anterior transposition may be performed. In cases of severe bone or tissue changes of the elbow (especially with cubitus valgus), the anterior transposition of the ulnar nerve may again be indicated. In cases of scarring, submuscular transposition may be preferred as it provides a healthy vascular bed for the nerve as well as soft tissue protection. Risks resulting from transposition include compromise in blood flow to the nerve as well as kinking of the nerve caused by insufficient proximal or distal mobilization. In these cases, revision surgery is necessary. Epicondylectomy is not common, at least in Germany. Recurrence of compression on the ulnar nerve at the elbow may occur. This review is based on the German Guideline "Diagnose und Therapie des Kubitaltunnelsyndroms" ( www.leitlinien.net ).
Not only truncating but also missense mutations of the KCC3 gene are associated with Andermann syndrome. Different types of KCC3 mutations may determine different clinical phenotypes.
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