Recurrence of Ulnar Vascular Malformations Involving Ulnar Nerve Compression, a Case Series

Vascular malformations (VMs) of the ulnar artery are rare, with an unclear prevalence [1]. Most upper limb VMs can be managed conservatively [2], but treatment may be necessary when they lead to aneurysm, rupture, thrombosis, or nerve compression [1]. A prior review of the literature reported nine case reports of ulnar artery VMs, two of which involved ulnar nerve compression, with no recurrence after treatment [3]. However, our clinical experience suggests that recurrences are not uncommon. We present three cases of VM recurrence after treatment, each involving ulnar nerve compression. A 39-year-old woman was first seen at age 25 years for a painful swelling with a blue discoloration over the right dorsal and medial elbow region. Her medical history included prior surgery for olecranon bursitis. Magnetic resonance imaging (MRI) and duplex ultrasound (US) confirmed a VM near the ulnar nerve at the elbow. A year later, complete VM obliteration was achieved with sclerotherapy treatment using aethoxysklerol foam. This provided only partial symptom relief for a few years. At 29 years, she sought care again due to elbow pain, limited elbow flexion and extension, increasing swelling of the arm and hand, and handgrip weakness. Alcohol embolization of the entire VM was performed, but it resulted in sensory loss and muscle weakness in an ulnar distribution. Initially there was partial recovery of ulnar nerve function over several months, but symptoms worsened after 1 year, including progressive hand intrinsic muscle atrophy. An electrodiagnostic (EDx) study confirmed ulnar neuropathy localized to the site of previous VM, and US showed VM recurrence (Figure 1A). Surgical partial resection of the recurrent malformation with neurolysis was performed. Postoperatively, motor and sensory function improved. A year later, EDx studies showed reinnervation, with normalization of the ulnar nerve cross-sectional area on US (Figure 1B). There was no recurrence over a 9-year follow-up. This 21-year-old woman had been diagnosed with a VM of the medial left forearm since birth, for which she underwent surgery and repeated embolization at the age of 8 years, and had worn a compression stocking since. Genetic VM tissue testing showed a de novo phosphoinositide-3-kinase catalytic subunit alpha (PIK3CA) mutation. PIK3CA-related overgrowth syndrome spectrum is commonly associated with venous anomalies and results from mosaic somatic mutations [4]. At the age of 14 years forearm pain and functional impairment increased, and MRI showed an expansion of the VM surrounding the ulnar artery, with an intramuscular extension into the finger extensors. Embolizations at ages 17 and 19 years provided only temporary pain relief. An EDx study confirmed ulnar neuropathy, and US showed ulnar nerve thickening throughout the forearm (Figure 2A,B). The patient then underwent surgical release of the ulnar nerve; intraoperative visualization confirmed the VM had grown into the ulnar nerve (Figure 2C). Postoperatively, the patient developed thrombophlebitis which was successfully treated with anticoagulation. Pain dissipated, but recurred within a few months, radiating from the wrist to the forearm and fingers. Physical examination revealed weakness, allodynia and sensory loss in an ulnar distribution. Pulsed radiofrequency therapy alleviated pain initially but it recurred within months. The patient has declined additional neuromodulation treatment and was considering forearm amputation at the last follow-up. Patient 3, previously described in the literature [3], underwent surgical resection of an ulnar artery VM, which recurred after 6 months. A second surgical resection was performed, but the VM recurred again after 2 months. Our findings highlight the recurrence of VMs of the ulnar artery. Recurrence times in our patients ranged from 2 months to 6 years, with the interval shortening after each recurrence. Awareness of recurrence is crucial for patient counseling and this underscores the need to inform patients that VM recurrence is possible, regardless of the type of treatment (endovascular or surgical resection), and may occur months or years later. In cerebral VM's recurrence after angiographically confirmed complete surgical resection is 9.5% (range 2.8%–16%), occurring from several months to 16 years after initial resection in children, and much rarer in adults, with only 18 cases reported in the English literature in the last 30 years [5]. Future studies should establish the recurrence rate in peripheral VM's, which may differ from cerebral AVM's due to differences in etiology, symptoms and treatment, to assist with patient counseling. Clinicians must differentiate between pain caused by local pressure from the VM recurrence and pain due to ulnar nerve compression, as the treatments differ. Ultrasound can be a useful tool in this differentiation, as demonstrated in our cases. Galia V. Anguelova: conceptualization (equal), investigation (lead), writing – original draft (lead), review and editing (equal). Jordan VanderEnde: conceptualization (equal), review and editing (equal). Claire I. A. van Houdt: review and editing (equal). Nens van Alfen: conceptualization (equal), review and editing (equal). We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Nens van Alfen is an ultrasound instructor for Sonoskills and performs editorial services for Wiley Publishing; all payment goes to their employer. The data that support the findings of this study are available from the corresponding author upon reasonable request.

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