Cephalic Vein Aneurysm in the Distal Forearm Managed with Sclerotherapy—A Rare Case Report and Literature Review

Abstract

Venous aneurysm of upper extremities is rare entity. We report a rare case of large cephalic vein (CV) aneurysm in a 41-year-old man who presented as slowly growing painless swelling at distal forearm along the lateral aspect. Duplex ultrasound showed aneurysm originating from the anterior wall of the CV with venous waveform pattern on Doppler. It was managed successfully with percutaneous sclerotherapy.

Venous aneurysms unlike the arterial aneurysms are rare entity. The incidence has been reported to be around 0.1% in general population. Venous aneurysms are the focal dilatation of venous segments which communicate with the normal vein. They are at least two to three times the normal diameter and are not contained within the varicose segment. It must have also no association with arteriovenous malformation or pseudoaneurysm. ¹ The first venous aneurysm was described by Harris in 1928. ² Hilscher ³ first suggested the term venous aneurysm, similar to arterial aneurysm. The most common locations of venous aneurysm are seen in internal jugular vein, popliteal vein, superior vena cava, and the portal vein. Upper limb venous aneurysms are extremely rare entity and are often mimic as soft tissue masses. Only few cases have been reported in the literature. ⁴ We report a rare case of large cephalic vein (CV) aneurysm at distal forearm managed with percutaneous sclerotherapy along with review of literature on CV aneurysm and their management.

Case Presentation

A 41-year-old male presented to us with complaints of swelling in the right distal forearm along the lateral aspect. The swelling was asymptomatic for the past 5 years but was increasing in size slowly. There was also history of trauma to the distal forearm 5 years back following which he started noticing small swelling over the local area. On examination, the swelling was soft in consistency and measuring ∼2 × 1.5 cm in size ( Fig. 1A , B ). There was no tenderness or local rise of temperature, and the swelling was compressible without any palpable bruit or thrill over it. When the manual compression was applied, it almost completely disappeared; however, it immediately reappeared after removing the local manual compression. The arterial pulses were well appreciated in the affected forearm. The swelling became more prominent on keeping the forearm in dependent position, and it shrank on elevation of forearm. An initial clinical diagnosis of slow flow venous malformation or soft tissue cystic mass was made. A duplex ultrasonography (USG) scan of the mass was performed which showed heteroechoic, compressible 1.7 × 1.2-cm cystic lesion along the lateral aspect of the distal forearm located superficially ( Fig. 1C ). The lesion was arising from the anterior wall of superficial CV with gradually filling on color Doppler without any evidence of thrombus ( Fig. 2A , B ). Focal disruption of CV wall continuing with the lesion was also noted ( Fig. 2C ). There was also evidence of internal echoes within the cystic lesion which was artifactual due to slow flow within the aneurysm. On applying the compression with USG probe, there was complete regression of lesion. On color and spectral Doppler, there was characteristic venous waveform pattern ( Fig. 2D ). There was no evidence of any arteriovenous fistula. We concluded the diagnosis of venous aneurysm of the CV. Under local anesthesia, sclerotherapy was performed with 2 mL of 0.5% polidocanol. During the injection, the CV is compressed to prevent the passage of sclerosing foam into the CV. After injection, compression was applied over the site of injection for 24 hours. Postsclerotherapy treatment, there was complete regression of swelling noted with residual pigmentation of skin and erythema in surrounding localized area ( Fig. 3 ). The postoperative course was uneventful, and the patient was discharged next day. Patient was advised clinical follow-up after 4 weeks; however, the patient was lost to follow up.

Fig. 1.

Swelling over the lateral aspect of distal forearm ( A, B ). Sonography shows heteroechoic cystic lesion measuring 1.7 × 1.1 cm ( C ).

Fig. 2.

Aneurysm arising from the anterior wall of superficial cephalic vein (CV) (arrow in A ) which shows gradually filling on color Doppler without any evidence of thrombus ( A, B ). Focal disruption of the CV (arrow) which is continuous with the adjacent aneurysm ( C ). Color and spectral Doppler shows venous waveform pattern ( D ).

Fig. 3.

Postsclerotherapy treatment, complete regression of the swelling noted. Postsclerotherapy pigmentation is also noted.

Discussion

Venous aneurysm can be classified into primary and secondary based on the etiology. The natural history of these primary venous aneurysms is poorly understood and remains idiopathic. The secondary venous aneurysms are more commonly secondary to trauma, inflammation, iatrogenic, and presence of arteriovenous malformation and underlying chronic venous insufficiency. ⁴ We hypothesized that trauma might has been the etiological factor in our case. Primary venous aneurysm commonly occurs in children, while secondary aneurysm usually presents in adulthood. They are equally common in men and women. However, they have been more commonly observed in individuals with higher body mass indices. Depending on location, venous aneurysm can be either superficial or deep in location. Most superficial venous aneurysms are palpable and easily compressible. They are usually diagnosed by inspection and palpation. There is no palpable bruit or thrill over it unlike the arterial aneurysm as in our case. Differential diagnosis of superficial venous aneurysm includes other slow flow vascular lesions, benign adipose, neurogenic and melanocytic mass. ⁵ Superficial venous aneurysms are usually asymptomatic swelling but may compress the adjacent neurovascular structures. In our case, they presented as slowly growing swelling with no neurovascular compromise. There have been the reports of radial and ulnar nerve compression by the venous aneurysm. ^{4 6} They expand and flatten depending on the position of the extremity and also expand on performing Valsalva maneuver. In contrast, deep venous aneurysms may sometimes present as painful or swollen soft tissue masses and may be associated with deep venous thrombosis or pulmonary embolism. Many of the venous aneurysms have been misdiagnosed as cystic masses, inguinal hernia, soft tissue masses including lipoma, and solid and neurogenic tumors. ^{4 5 7} Venous aneurysms of the upper and lower limbs involve both superficial and deep venous systems. Upper limb venous aneurysms are very rare, and only few cases have been reported. We did a PubMed literature search of cephalic venous aneurysms unrelated to arteriovenous fistulas, and total number of 14 cases have been reported till date ( Table 1 ). ^{1 4 6 7 8 9 10 11 12 13 14 15} The most common presentation was slowly growing swelling or pain. There were two patients who presented with symptoms of nerve compression. ^{4 6} All the reported cases of CV aneurysm were treated with surgical excision; however, we did a percutaneous sclerotherapy in our case. Previously, no case report of CV aneurysm treated with percutaneous sclerotherapy has been reported in the literature. Plain radiographs are helpful in the evaluation of any unknown masses by assessing the presence of mineralization and underlying bony involvement. It helps in narrowing the differential diagnosis. USG is the investigation of choice which enables the confirmation of the diagnosis of all forms of venous aneurysm of the extremities. It is noninvasive and quick imaging tool and provides all the relevant information such as lesion morphology, localization, feeding vessel, presence of thrombi, and communication. It also helps in planning the surgical and endovascular procedure. USG will show a compressible anechoic structure or sometimes with floating internal hyperechoic thrombus. On Doppler examination, venous aneurysms may show bidirectional blood flow, no flow in case of complete thrombosis, and partial flow in case of partial thrombosis. USG can also be helpful in the diagnosis of venous aneurysm in neck and abdomen. The dynamic enhanced computed tomography (CT) and magnetic resonance imaging (MRI) are more accurate in delineating the morphology and extent of lesion. However, the use of other imaging tools such as phlebography, CT, and MRI should be reserved for the diagnosis of thoracic and abdominal venous aneurysms. ^{1 6} On CT scan, venous aneurysm can show variable attenuation depending on the patency of the aneurysm and internal thrombus age. On MRI scan, it can show variable internal signal intensity depending on the internal thrombus. Contrast-enhanced images show enhancement pattern similar to the other venous structures if there is no internal thrombosis. The contiguity of aneurysm with vein on all the imaging modalities is critical in the diagnosis. ¹ In our case, we performed duplex USG and found a venous aneurysm communicating with a defect in the wall of CV. There are many treatment options of venous aneurysm which include aneurysmectomy, endovenous ablation and lateral venorrhaphy, aneurysmectomy and end-to-end anastomosis, and aneurysmectomy with interposition graft repair. ⁴ In most of the cases, simple excision of the aneurysm is adequate. Sclerotherapy can be a nonsurgical alternative to achieve better cosmetic results and to avoid injury to the surrounding structures. Few cases of superficial venous aneurysm treated with sclerotherapy have been reported in the literature. ^{16 17 18} The potential complications associated with sclerotherapy (percutaneous injection of polidocanol) include superficial erythema, induration of the skin, and hyperpigmentation. ^{19 20} In our case, patient developed local erythema and hyperpigmentation after the procedure for which we prescribed topical depigmenting agent. Venous aneurysms can also be left to observation in truly asymptomatic cases. ¹ To the best of our knowledge, sclerotherapy treatment of CV aneurysm has never been reported in the literature.

Table 1. Reported case of cephalic vein aneurysm in the literature.

Author's name (y)	Age/sex	Clinical presentation	Location	Management
Ekim et al (1995)	17 y/F	Expansion, pain	Right forearm	Excisional surgery
Goto et al (1998)	41 y/F	Expansion	Lower third of the left arm	Excisional surgery
Wang et al (2001)	67 y/M	Expansion	Dorsal aspect of the right wrist	Excisional surgery
Wang et al (2001)	53 y/M	Expansion	Volar aspect of the right wrist	Excisional surgery
Kassabian et al (2003)	43 y/M	Weakness	Antecubital fossa	Excisional surgery
Ekim et al (2004)	ND	Asymptomatic	ND	Excisional surgery
Ekim et al (2004)	ND	Symptomatic	ND	Excisional surgery
Faraj et al (2007)	19 y/M	Expansion	Left forearm	Excisional surgery
Min et al (2008)	13 y/M	Expansion	Right upper arm	Excisional surgery
Cakıcı et al (2014)	61 y/F	Pain, expansion	Right wrist	Excisional surgery
Naqvi et al (2016)	35 y/F	Expansion, numbness	Flexor aspect of the right mid-forearm	Excisional surgery
Kobata et al (2018)	38 y/F	Pain, expansion	Left upper arm	Excisional surgery
Weeks et al (2018)	54 y/M	Pain, expansion	Volar radial aspect of his left wrist	Excisional surgery
Antonopoulos et al (2019)	67 y/M	Expansion, burning sensation	Left wrist	Excisional surgery
Present case (2021)	41 y/M	Expansion	Right distal forearm	Sclerotherapy

Abbreviations: F, female; M, male, ND, not described.

Conclusion

CV aneurysm should be considered in patients with compressible, nonpainful masses in distal forearm and wrist. Preoperative diagnosis of CV aneurysm with Doppler USG can be done. Sclerotherapy with polidocanol can be simple, effective minimally invasive treatment of CV aneurysm alternative to surgical excision.