Management of a fusiform brachial artery aneurysm associated with birth trauma
- 1 Pediatric Surgery, University Children’s Hospital Zürich, Zürich, Switzerland
- 2 Children’s Research Center (CRC), Universitats-Kinderspital Zurich, Zürich, Switzerland
- 3 Department of Pediatrics, Division of Emergency Medicine, Universitats-Kinderspital Zurich, Zürich, Switzerland
- 4 Department of Surgery, Division of Cardiovascular Surgery, Universitats-Kinderspital Zurich, Zürich, Switzerland
- 5 Department of Surgery, Division of Plastics and Reconstructive Surgery, Universitats-Kinderspital Zurich, Zürich, Switzerland
- Correspondence to Dr Eduardo Betancor Campos; edu.betancorcampos@gmail.com
Abstract
Brachial artery aneurysms in children are rare. Surgical treatment is generally recommended.
We present the case of a female toddler with a pulsatile swelling on the medial aspect of the right upper arm without history of recent trauma. Medical history revealed a traumatic birth with labour arrest. Postnatally diffuse trunk and arm haematomas as well as a temporary right arm discolouration were detected. Preoperative ultrasound revealed a true brachial artery aneurysm. A full-body MRI ruled out any accompanying lesions. Primary resection and end-to-end anastomosis were performed. Recovery was uneventful. 6-month and 12-month follow-up showed normal motor function and arterial patency; ultrasound also demonstrated harmonious growth of the anastomosed vessel segments.
No other publication has associated birth trauma with brachial artery aneurysm yet. Correct diagnosis and prompt curative surgery are key to prevent severe complications. Further reports and data on long-term outcome are needed to optimise clinical management.
Background
Arterial aneurysms are extremely rare in children and the true incidence is unknown. Only about 5% of peripheral aneurysms in children are located on the upper extremity. In the paediatric population, true aneurysms are typically associated with cardiac abnormalities and other conditions such as Marfan syndrome, Ehlers-Danlos syndrome, Turner syndrome, infection or various types of vasculitis (eg, giant cell arteritis, Kawasaki disease, polyarteritis nodosa).1–11 Most brachial artery aneurysms are pseudoaneurysms and are typically caused by arterial damage during diagnostic or therapeutic interventions.6 To the best of our knowledge, until today no published literature associates a true brachial artery aneurysm (BAA) with birth trauma as to be shown in this case report.
Case presentation
A toddler of British Asian descent presented to the emergency department of the University Children’s Hospital in Zürich, Switzerland in 2021 with a swelling on the right upper arm of sudden onset. The toddler was otherwise healthy, and no recent trauma was reported by the parents. On physical examination, there was a non-tender, pulsatile swelling on the proximomedial aspect of her right upper arm (figure 1). The overlying skin and the remaining upper limb examination including the hand trophic were normal and no functional impairments were observed. Patient age limited a more specific neurological examination.
(A, B) Initial presentation of the female toddler demonstrating a pulsatile tumour on the upper right arm.
We took an extended history from the parents, which revealed a traumatic birth with labour arrest due to occipital-posterior positioning of the baby leading to an emergency C-section at 40 2/7 weeks gestational age. No complications were reported. The newborn had a birth weight of 3220 g, a length of 53 cm and a head circumference of 33 cm. Initial physical examination revealed multiple haematomas on the trunk and extremities as well as discolouration of the right upper extremity starting distal to the place where the aneurysm was found months later (figure 2). As no swelling or persistent discolouration was noted at discharge 4 days after delivery and observed upper limb movements were normal, no further attention was given to these initial findings by the medical team.
(A, B) Photographic documentation after delivery showing diverse diffuse haematomas on the trunk and right upper extremity.
Investigations
Prompt duplex ultrasonography was performed and a rounded lesion of 27×20×15 mm with turbulent arterial inflow and outflow was described and led to the diagnosis of a BAA (figure 3). A full-body MRI ruled out other accompanying lesions (figure 3). Echocardiographic studies did not show any coronary aneurysms but demonstrated a small hemodynamically irrelevant patent foramen ovale with a non-concerning left to right shunt. Postoperative genetic testing did not reveal any pathologically modified sequences on genes usually affected in cases of Marfan syndrome, Ehlers-Danlos-Syndrome, Cutis laxa syndrome as well as in other directly associated genes. The chosen molecular-genetic method can identify 98–99% of mutations on the examined genes and is therefore highly sensitive. Consequently, a genetic disorder can be ruled out.
(A, B) Diagnostic findings: a full-body MRI shows the aneurysm of the right brachial artery. Same aneurysm during ultrasound examination measuring 26.9×20.5×15.4 mm.
Treatment
Following multidisciplinary team consultation with plastic and cardiovascular surgery, the operative resection of the aneurysm was planned to prevent complications such as thrombus or embolus formation, aneurysmal enlargement or rupture as well to avoid (further) nerve compression or hypoperfusion of the arm. The surgical plan included possible saphenous-vein interposition graft, if necessary.
Under general anesthesia, an 8 cm ‘lazy S’ incision over the aneurysm was used to expose the area of interest. Under surgical loupe magnification, meticulous dissection of the aneurysm and the neighbouring veins and peripheral nerves (V. basilica, V. brachialis, N. medianus, N. ulnaris and N. cutaneous antebrachii medialis) was performed (figure 4). A small arterial twig arising from the dorsal wall of the aneurysm was suture ligated. Following complete exposure of the aneurysm, 100 IU/kg body weight crystalline heparinisation was administered intravenously. Proximal and distal vascular control was achieved, and the aneurysm was incised. It demonstrated a significantly thickened wall. The true aneurysm was then resected with a total length of 4 cm. Because of distal arterial kinking and thorough mobilisation of the brachial artery, an oblique end-to-end anastomosis using interrupted 8-0 Prolene transmural sutures was performed (figure 5). Immediate and strong pulsatile flow over the anastomosis was documented. The total ischaemia time with the proximal and distal artery clipped was 55 min. Of note, during this time, a prompt capillary refill of the hand was noted, suggesting distal perfusion through collateral vessels.
(A, B) Intraoperative view of the aneurysm after meticulous preparation of neighbouring veins and nerves that are marked with vessel loops. From cranial to caudal, the median nerve, ulnar nerve and cutaneous nerve of the forearm. Line diagram explaining the anatomy. The line diagram was created by the principal author of the case report.
Intraoperative photographic documentation of the end-to-end anastomosis.
Postoperatively, the oxygen saturation on the right thumb was 100%, the capillary refill time was prompt and the distal pulses were strong at all times. Therapeutic anticoagulation with heparin was established 8 hours after surgery and regularly adjusted according to the measured anti-factor Xa values (goal: 0.35–0.7 IU/mL) for 5 days. On postoperative day (POD) 6, medication was switched to oral aspirin (5 mg/kg body weight). Repeated bedside duplex ultrasound measurements were performed in the early postoperative period and showed regular blood flow through the anastomosis. Ultrasound studies on POD 7 (figure 6) demonstrated a brachial artery with even calibre without signs of relevant turbulent flow. A bandage was used to limit upper arm excursions for the first 3 days. The parents were then instructed to avoid any shoulder abduction above 90°. On the day of discharge home (POD 7), the patient was in very good general condition with uneventful wound healing and normal limb perfusion and motor activity. Aspirin was continued in the same dosage for 6 months after surgery.
(A, B) Duplex ultrasound at 12-month follow-up demonstrating an arterial calibre of 2.31 mm without signs of turbulent flow as well as a harmonious growth of the proximal, distal and anastomosed vessel segments. Distal to the anastomosis, the peak systolic velocity (Vmax) is 51 cm/s.
Histological evaluation of the specimen confirmed a true aneurysm containing all three vascular layers with evidence of widened intimal layer, fragmentation of the internal elastic membrane, perimedial fibroplasia and strongly reduced smooth muscle cells on the tunica media.
Outcome and follow-up
Regular follow-up in our outpatient clinic at 2, 4 and 8 weeks, 3 months and thereafter at 6 and 12 months showed timely healing, normal motor skills and hand trophic as well as strong proximal and distal pulses without signs of recurrence. Duplex ultrasounds at 1, 2, 6 and 12 months showed a patent artery with adequate growth in vessel size and optimum remodelling of the anastomotic site. Furthermore, no signs of stenosis or turbulent flow were detected using Doppler. Aspirin therapy was stopped at 6 months.
Discussion
To the best of our knowledge, this is the first report in the medical literature on successful surgical treatment by resection and primary anastomosis of a true BAA-related to birth trauma.
We performed a primary resection with end-to-end anastomosis of a BAA in a toddler that was associated to birth trauma.
Establishing the correct diagnosis as well as prompt curative treatment are key to avoid complications. Enlargement and rupture of a BAA could lead to compression of neural structures, long-term hypoperfusion, thrombus formation and embolisation, bleeding and, in the worst-case scenario, limb loss.
True BAA are very rare in children. In total, only 16 cases of BAA of unknown aetiology have been reported to date; all other published cases were either associated with connective tissue disorders or direct vascular trauma. We did not find any cases associating birth trauma with the development of arterial aneurysm. In our case, labour arrest, emergency C-section and the immediate clinical signs on the first days of life with discolouration of the arm likely related to venous stasis suggest a strong correlation between birth trauma and the formation of the aneurysm. We hypothesise that during labour arrest, the uterine contractions damaged the brachial artery leading to aneurysm formation months later. Genetic testing ruled out a connective tissue disorder.
There is no standard of care for diagnostics. We advise using duplex ultrasound as the primary diagnostic method. It is an inexpensive, non-invasive and time-efficient tool that can help to define the characteristics of the lesion as well as study blood flow through the aneurysm. As there is a clear association of arterial aneurysm with connective tissue disease and cardiac anomalies, we propose a full-body MRI scan to rule out accompanying lesions. Intravenous contrast medium can be used in both methods to identify extravasation of blood as well as to differentiate a true aneurysm from pseudoaneurysm or arteriovenous malformation. We do not advise CT angiography since MRI can achieve more information including morphology as well as 4D flows, without exposing the child to harmful radiation.
Although the overall number of published cases is very limited, there seems to be a consensus among authors that surgical treatment is necessary and feasible in the paediatric population. The authors argue that the benefit of performing surgery significantly outweighs the risks of complications related with conservative management alone, that is, permanent nerve damage and limb hypoperfusion, particularly in children. Of note and to support the tendency towards operative treatment, we did not find any reports on sequelae of missed aneurysms or outcome of conservative treatment. In line with the published reports and in view of the size and location of the BAA in our patient, the involved multidisciplinary team of plastic and cardiovascular surgeons decided to proceed with surgical treatment to avoid long-term complications. Resection of the true aneurysm with primary end-to-end anastomosis was feasible and allowed healing without any sequelae at this stage of follow-up.
In 2008, Pagès et al reported on three infants with true BAA of unknown origin and one with a false aneurysm. One end-to-end anastomosis was performed; the three other patients received an autologous venous graft.11 In 2015, Gangopadhay et al published a case of a 7-month-old infant with a BAA of undescribed length and unknown origin that was resected and reconstructed using a saphenous-vein graft.5 A year later, a similar case was published by Ghazanfar et al. The group reported about a 2-year-old boy who presented with a 7 cm true brachial artery aneurysm on the distal end of the right brachial artery of unknown aetiology. Again, primary resection and a reversed great saphenous-vein graft were performed.6 The largest case series was published by Nurmeev et al who reported on five cases of upper limb aneurysms in children with a median age of 3.3 years. Two aneurysms were idiopathic and two were associated with connective tissue disorders. In one patient, a history of trauma was reported, but it was not stated if this was related to birth trauma. With respect to surgical treatment, one radial artery ligation was performed as good collateral blood flow ensured the blood supply of the hand. In one case, an end-to-end anastomosis was performed, and two patients received venous grafts. In one case, a polytetrafluoroethylene prosthesis was chosen for restoration. Revision was done on POD 5 due to thrombosis and dysfunction of the graft and an autogenous vein graft was required.9 The first report on BAA recurrence following resection was not published until 2020 by Kaplan et al. Primary resection and reconstruction with a saphenous-vein graft was performed in a 2-year-old boy with no medical history. Less than 4 months postoperatively, recurrence was documented on both ends of the graft. A second attempt of excision and saphenous-vein grafting was performed. On re-referral to the Children’s Hospital some time later (exact time frame not noted), ultrasonography confirmed a second recurrence showing two aneurysms 7 cm apart at the margins of the arterial reconstruction. The third surgical attempt involved a reversed saphenous-vein graft with microsurgical anastomosis and two grafts of acellular dermal matrix wrapped around the anastomotic sites for reinforcement. Three months postoperatively, ultrasound examination reported recurrent aneurysm versus pseudoaneurysm at the distal end of the vein graft. At 3 years of age, the excision and reconstruction with reversed saphenous-vein graft was repeated with additional reinforcement using a Gore-Tex conduit. Eighteen months postoperatively, there were no more signs of recurrence. The underlying reason for repeated recurrence remained unclear to the authors, although they suspected poor postoperative immobilisation or native brachial artery disease to be the underlying reason.8
While vein grafts are commonly used, primary end-to-end anastomoses is always an option if feasible without significant tension. The use of a primary, oblique interrupted end-to-end anastomosis increases the surface area of healthy arterial wall between the sutures and ensures no foreign or non-arterial material as well as a pulsatile anastomosis. This in turn allows the arterial wall to grow and increase its diameter as the patient ages. We find extensive mobilisation and primary end-to-end anastomosis to be a preferable option as there is no donor-site morbidity and as it only requires one anastomosis instead of two as in the situation of a vein graft. In addition, we can avoid the long-term risks associated with venous tissue in the arterial system when using a reverse vein interposition graft. We did not find data on the use and the long-term follow-up of arterial versus reversed venous grafts in brachial artery reconstructive surgery. Patency of reversed saphenous-vein grafts versus radial or internal mammary artery is very critical when used as coronary artery bypass graft, but the use of arterial free grafts for peripheral vascular aneurysm repair is regularly discussed in the literature but remains controversial. Even though some studies claim there is no significant benefit of using arterial grafts,12 other studies state that the patency rates are significantly better when an arterial graft is used.13–15
Unfortunately, most abovementioned reports on BAA in children lack long-term follow-up as well as more specific clinical details on, for example, preoperative clinical findings, histology and complications. There are no guidelines for clinical management and many associated topics remain open for discussion. To prevent thrombosis and stenosis at the anastomosis, some authors advocate anticoagulative therapy. The recommended duration of aspirin treatment after surgery varies between 311 and 6 months,2 which matches with the 6-month duration of aspirin therapy we prescribed in our case. Once the early vascular remodelling is completed, no further anticoagulation is needed and a harmonious vascular growth with time is ensured. Some authors advise to do life-long follow-up to prevent missed recurrences2; however, the ideal duration of follow-up remains unclear. We plan to monitor our patient until adulthood and screen for late stenosis and blood flow turbulences.
In summary, our report of successful repair of a true BAA in this young toddler significantly adds facts to the scarce field of data in the paediatric population. We would like to sensitise our colleagues that birth trauma may also be an aetiological factor. Surgical treatment is feasible and generally recommended to prevent severe complications. The earlier the diagnosis and treatment, the better are the chances of achieving a direct anastomosis. Treating physicians should continue to collect data and publish on this rare condition to expand our knowledge and improve the clinical management. As in our situation, interdisciplinary discussions and decisions are key to define the best options in such difficult and rare clinical scenarios.
Learning points
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Brachial artery aneurysms are extremely rare in children and the actual incidence is unknown.
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Among the known associations with connective tissue disorders, cardiac anomalies and various types of vasculitis, one should also consider birth trauma in young children as a potential cause of the condition.
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Establishing the correct diagnosis followed by prompt curative surgery is key to avoid long-term complications, such as enlargement of the aneurysm, compression of nerval structures, rupture and bleeding, long-term hypoperfusion, thrombus formation and embolisation and, in the worst-case scenario, limb loss.
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We advise performing a primary end-to-end anastomosis if feasible. Otherwise, we suggest using a reversed saphenous-vein graft.
Ethics statements
Patient consent for publication
Acknowledgments
I would like to express my special thanks of gratitude to all three co-writers, especially KN, for all the time she has invested in this case and her thorough correction of the report.
Footnotes
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Contributors I have been the principal author of this case report and the attending physician during the postoperative hospital stay. KN and DH were the attending surgeons. FD was the attending physician in the ED. All three were responsible for corrections.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.
References
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