The underlying pathomechanism in a symptomatic LGS is the widespread electrographic expression of a focal pathology developing early in life through the rich anatomical networks of a developing brain.
Congenital brachial artery aneurysms in the upper extremity are very rare(1).Although most brachial artery aneurysms are associated with systemic diseases, they can present as an isolated finding. These lesions can be asymptomatic masses or can present with pain, ischemia, or nerve compression.
They must be differentiated from pseudoaneurysm, hematoma, vascular malformation, and other vascular lesions that can occur in the upper limb. We present a case of right axillary/brachial artery aneurysm in a 11 weeks-old male infant with Acute Limb Ischaemia, and the literature is reviewed.
An 11 week old male baby was brought to the Emergency Department by his mother with complaints of sudden onset swelling in the right axilla and excessive crying with inability to move the right upper limb that had lasted 1 day. There was no history of Trauma/previous history of any significant complaint.
Family history was not contributory. On examination, the child was alert, active and crying and the right upper limb was flail and colder and pale from hand to mid arm compared to the left upper limb. On palpation, there was a warm, tender nodular pulsatile swelling felt in the axilla and upper medial arm. The skin was normal over the swelling.
The brachial and radial artery pulses were not palpable. The left upper limb and both lower limbs were normal.
Capillary Refilling Time was prolonged and Spo2 in fingers was 81% Doppler Study of the Right Upper limb – A tubular lesion in the upper arm extending to the axilla in close proximity to the major arteries showing significant arterial flow pattern in the proximal part.
CT Angio RT Upper Limb
The right distal subclavian artery showed fusiform irregularity with the entire axillary artery and proximal brachial artery appearing thrombosed with fusiform enlargement of these vessels of size 4.8cm x 1.3 cm. The distal brachial artery appeared to be reformed with attenuated flow imaged in the brachial artery in the elbow and also in the radial and ulnar arteries in the forearm. The rest of the proximal subclavian artery appeared to be normal. An enlarged reactive axillary lymph was noted within the axilla. The rest of the aortic arch vessels appeared normal.
Operative Procedure executed
Exploration and Revascularisation of Right Upper Limb with PTFE Graft from Distal Subclavian Artery to Mid Brachial Artery
Fusiform Aneurysmal Dilatation of Axillary Artery extending to distal Brachial Artery with Intraluminal thrombus in the aneurysm adherent to surrounding nerves and soft tissues.
Under aseptic Precautions parts painted and draped. Right axilla and upper arm explored through longitudinal incision extending across axilla to pectoral region and wound opened
in layers. Above findings confirmed and the vessel dissected out in its entirety until normal flow was seen proximally and distally until normal caliber vessel was seen. Median and ulnar nerves released and dissected out. Vascular clamps applied and the proximal and distal normal vessels cut and prepared with adventitiectomy for anastamosis. GSV explored in left lower limb and there was a significant size mismatch and hence size 3 PTFE Artery graft was used for revascularisation from distal subclavian artery to distal brachial artery and anastamosed with 8-0 ethilon intermittent sutures on proximal and distally. Flow established satisfactorily. The aneurysmal sac ligated at both ends and left undisturbed and hemostasis achieved and wound closed in layers with a single corrugated drain.
In both children and adults, peripheral artery aneurysms are much less common than central aneurysms. Of peripheral aneurysms, only about 5% are located in the upper extremity.
Within the upper extremity, subclavian involvement is most common, and more distal lesions are rare. In the pediatric population, true aneurysms have been described in the axillary, brachial, ulnar, and radial arteries. Unlike adults, in whom aneurysms are usually related to hypertension and atherosclerosis, pediatric aneurysms are typically associated with cardiac anomalies (aortic aneurysms) and other conditions, such as the Marfan Syndrome, the Ehlers– Danlos Syndrome, the Turner Syndrome, infection (mycotic), and various types of vasculitis such as Giant Cell Arteritis, the Kawasaki Disease, and Polyarteritis Nodosa.(1) Since the 1950s, 14 cases of brachial artery aneurysm in children younger than 12 years have been reported.(1–13) In most cases (9 of 14), other associated aneurysms, both central and peripheral, were present. In 5 cases, the brachial artery aneurysm was solitary. In just over half (8 cases), an associated systemic diagnosis was identified, with giant cell arteritis being the most commonly associated diagnosis. Other associated diagnoses were polyarteritis nodosa, Kawasaki arteritis, and the Ehlers–Danlos syndrome. Cases with multiple aneurysms were not necessarily associated with an identified systemic diagnosis. In some cases, the presence of a solitary brachial artery aneurysm was followed by the subsequent development of an aneurysm at another location, either central or peripheral. In 1 case, a second aneurysm (abdominal aorta) developed 9 years after the brachial artery aneurysm was treated, suggesting that continued long-term surveillance of these children is important.(5)
Brachial artery aneurysms may be initially confused with hematoma, vascular malformation, or arteriovenous fistula. Doppler ultrasonography, computed tomography angiography (CTA), or MRA can be performed to define the characteristics of the lesion. Ultrasonography has less sensitivity and specificity than CTA or MRA and should be used as a screening study in these patients. Although both CTA and MRA expose the child to an intravenous dye load, they can also help differentiate among pseudoaneurysm, true aneurysm, and arteriovenous fistula. They also provide additional information regarding extravasation of blood from the lesion, evidence of thrombosis, and the status of adjacent structures. Althoughrupture,thrombosis,occlusion,and embolization are theoretical concerns,only 1 case of thrombosis of a brachial artery aneurysm in a child has been described,(6) and no cases of embolization or rupture have been reported. Because of this, an asymptomatic brachial artery aneurysm does not necessarily require urgent surgical intervention.(1) It may be reasonable to observe small asymptomatic aneurysms in very young children until the child is larger, and small aneurysms associated with inflammatory conditions can potentially be treated with prednisone, intravenous immuno- globulin, or other immune modulators to prevent progression. Moderate or large aneurysms, enlarging aneurysms, or aneurysms causing neurologic or vascular symptoms should be treated surgically. Definitive surgical treatment involves resection of the aneurysm with arterial repair or reconstruction. For small lesions, end-to-end anastomosis may be performed after resection. For larger lesions, an interposition vein graft is required. In infants and small children, microsurgical techniques are required.
In summary, the diagnosis of brachial artery aneurysms in pediatric patients involves a screening ultrasound, followed by further evaluation with CTA or MRA. Early surgical intervention including resection and microsurgical reconstruction should be performed for moderate or large lesions, expanding lesions, or aneurysms that are causing neurologic or vascular symptoms.
Small asymptomatic aneurysms may potentially be observed until the child is larger, and lesions associated with systemic inflammatory conditions (polyarteritis nodosa, giant cell arteritis, etc.) may benefit from immunosuppression. A rheumatologist and geneticist should be involved to help screen for associated conditions. Because of the frequent association with aneurysms at other locations, full-body arterial evaluation is important. Because a subsequent second central or peripheral aneurysm can develop many years later, children with solitary brachial artery aneurysms should be followed long-term with screening studies.
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