Posttraumatic Pseudoaneurysm of Extracranial Internal Carotid Artery Presenting as Massive Oropharyngeal Bleed with Shock and Hemiparesis in a 12-year-old Boy: A Case Report.

Aneurysm in the extracranial segment of the internal carotid artery is extremely rare in children. Traumatic aneurysms of the internal carotid artery are generally "false aneurysms" (pseudoaneurysms) with at least part of the aneurysm wall composed only of the adventitial layer, or even just by the hematoma. There are few reports in the world medical literature about its etiology, presenting features, and catastrophic consequences. The natural history of the disease has not been well-defined yet. However, the potential risk of embolism originating from the aneurysm or even its rupture indicates a need for early diagnosis and intervention. We present a 12-year-old previously healthy child with recurrent oropharyngeal hemorrhage leading to massive hematemesis and hypovolemic shock due to right-sided internal carotid artery pseudoaneurysm with hemorrhage and right middle cerebral artery territory infarct. The child required urgent endovascular stenting with decompressive craniectomy for life-threatening intracranial bleeding with herniation with near-complete recovery. Copyright:

INTRODUCTION

Aneurysm in the extracranial segment of the internal carotid artery is very rare and uncommon in the pediatric age group. Although any segment of the carotid artery (common, external, internal) can be involved, the internal carotid artery is most commonly involved. True aneurysms involving all layers of the carotid arterial wall and false aneurysms involve a single layer (tunica adventitia) and or they may be lined by blood clot only. Overall, extracranial carotid artery aneurysm accounts for less than 1% of all arterial aneurysms and approximately 4% of peripheral artery aneurysms.[123] Management of extracranial carotid artery aneurysm represents 0.2%–5% of all the carotid procedures depending upon the hospital performing it.[4] The usual causes related to an aneurysm in the extracranial internal carotid artery (EICA) in adults are atherosclerosis, arteritis, fibromuscular dysplasia, trauma, infections, and dissections.[5] Trauma and infection are the most common causes of these aneurysms, with congenital and collagen vascular disorders being the less common causes in children.[6] The most frequent signs at presentation are palpable pulsatile/non-pulsatile neck mass, neurological manifestations, mainly transient ischemic attacks are the commonest presentations.[17] They can assume the large size and have catastrophic complications of thromboembolism and hemorrhage. We present a 12-year-old child with recurrent oropharyngeal hemorrhage leading to massive hematemesis and shock. A distal embolism causing malignant ipsilateral cerebral infarct further complicated the management options. The aim of our case report was to increase awareness and inclusion of this rare pathology in the differential diagnosis of oropharyngeal hemorrhage which would facilitate the early diagnosis and prevent devastating complications.

CASE HISTORY

A 12-year-old boy was brought by parents with a history of vomiting blood for the last 8 days, started with –one to two times a day for initial 3 days, and was admitted to the civil hospital for the same complaints, discharged after 2 days with symptomatic treatment. Again, he developed similar complaints about the last 2 days but with an increase in quantity and frequency. There was a history of lifting heavyweight on the right shoulder 10 days back.

This child had massive upper gastrointestinal (GI) bleeding in the form of hematemesis with severe anemia with shock within a short duration without any prior history of liver disease or portal hypertension or any other significant illness except trauma. Complete blood picture showed moderate anemia (Hb 7 g%, white blood cells [WBCs] 16,500 cells/c.mm, platelet count 1.36 lakhs) with normal coagulation profile (PT 15.4/INR 1.14 APTT 36 s, Serum Fibrinogen 199, normal LFT, serum creatinine of 0.4 mg/dL, serum Sodium 142 meq/L, K level 3.8 meq/L, and Cl 102 meq/L). Emergency gastro-duodenoscopy by gastroenterologist demonstrated vascular telangiectasia in the antrum of the stomach, for which argon plasma coagulation (APC) was done. Within the next 24 h, the patient had second episode of massive hematemesis (around 2 L) leading to hemorrhagic shock with hemoglobin less than 4 g/dL and so, he was resuscitated, intubated, and ventilated. He was supported with fluids, blood product transfusions (4 units of packed cell red blood cell [RBC], 4 units of FFP, and 2 units of single donor platelet), and vasoactive medications.

Fresh bleeding was noted from the oral cavity in the cricopharyngeal region during intubation using the RSI technique. The patient became unconscious with subsequently developed left-sided hemiparesis and anisocoria. Immediate hyperventilation given with target PaCo2 between 30 to 40 mmHg was provided along with hyperosmolar therapy (3% saline, 5 mL/kg). After stabilization, A CECT brain and neck reported approximately 1.8 cm × 1.8 cm × 2.1 cm pseudoaneurysm from right distal cervical internal carotid artery with a neck measuring 7.3 mm aneurysm appeared bilobed with extension into the parapharyngeal region surrounding hematoma with compression of distal ICA in the neck [Figure 1].

Figure 1

CT angiography of neck and head showing distal ICA pseudoaneurysm at the level C2 level

CECT scan of the brain revealed right internal carotid artery (ICA) infarct with evolving infarct in right middle cerebral artery (MCA) territory with wedge-shaped hypodensity. The mass effect noted on the right lateral ventricle with a midline shift of approximately 1 cm and herniation [Figure 2]. Angiography of the carotid arteries in the cervical region confirmed an extracranial pseudoaneurysm near the skull base, at the level of C2 vertebrae [Figure 3].

Figure 2

CT brain with right MCA infarct, mass effect on right lateral ventricle with midline shift to left

Figure 3

Angiography neck showing right ICA pseudoaneurysm

An emergency endovascular stenting for pseudoaneurysm with decompressive craniectomy for malignant cerebral edema in a single sitting by a team of interventional radiologists and neurosurgeons was performed within 24 h of admission and within 2 h of developing Anisocoria. The patient had undergone right cervical ICA stenting using right femoral access: 6 mm × 60 mm, fluency stent graft, placed across the pseudoaneurysm.

Postprocedure, right ICA angiogram with CT neck revealed complete exclusion of pseudoaneurysm [Figure 4]. His sensorium improved within the next 2 days, extubated on day 5 of admission, and discharged with left-sided hemiparesis after 10 days of hospitalization. Oral anticoagulation therapy, Aspirin at the dose of 5 mg/kg/day was continued. But after 6 months on the OPD basis follow-up, He had minimal left-sided hemiparesis, with Glasgow Coma Scale of 15/15. He was able to walk without any gait disturbance and follow-up Doppler’s neck revealed the stented segment of right ICA with stable occlusion of pseudoaneurysm [Figure 5].

Figure 4

Post-stent repeat CT angiography neck revealing complete occlusion of pseudoaneurysm and stent inside the right ICA

Figure 5

Pre- and post-stent-graft digital subtraction angiography showing near-complete resolution of the pseudoaneurysm

DISCUSSION

In our patient, trauma due to carrying of heavyweight over neck and shoulder might have led to tear at a deep location of the carotid artery, developing a pseudoaneurysm at the site. The site of hemorrhage at the lateral pharyngeal wall correlates with the position of the patient’s developing PA which led to erosion of the oropharyngeal region culminating in hemorrhagic episodes. The most common presenting complaint is pulsatile or no pulsatile neck swelling. Less often, patients may experience embolic episodes (TIAs, amaurosis fugax), catastrophic hemorrhage, and embolic complications. The emboli may arise from mural thrombi within the pseudoaneurysm or from associated intimal tears.[5] Neck pain (carotidynia) is noticed frequently and it can be caused by the enlargement of the pseudoaneurysm, rupture of the aneurysm into the carotid sheath, or from an associated intramural dissection. Diagnosis is usually established by a combination of clinical and radiological findings.[57] Radiological confirmation can be achieved by a Duplex ultrasound scan (US), digital subtraction angiography (DSA), MRI and MRA, or CTA. The most serious consequences of ICA aneurysms are related to the high risk of thromboembolic events resulting in a stroke or transient ischemic attack [Figure 2]. Treatment of PAs is generally considered mandatory because of their unstable wall and tendency toward spontaneous expansion and rupture. The treatment approach depends on presenting complaints, etiology, and site of the carotid artery aneurysm. Observation is safe for some small, asymptomatic aneurysms, but the repair is indicated for enlarging asymptomatic aneurysms to prevent stroke and aneurysm rupture, and for all symptomatic aneurysms. Due to the limited number of large studies, the safety and efficacy in the surgical and interventional management of EICA are unclear. The optimum treatment has not been clearly defined.[7] In most series, open surgical repair is more often selected for true aneurysms, infected aneurysms, and larger aneurysms causing mass effects. But open surgical repair carries a high risk of rupture, and cranial nerve palsy.[89] The surgical repair can be tried in the pediatric age group but avoided in this case due to unfavorable anatomy and side effects of long-term anticoagulation. However, the present case admitted with massive ongoing bleeding and simultaneous complication of ipsilateral distal ICA infarct with tentorial herniation, and also, the location of pseudoaneurysm was high up reaching skull base at second cervical vertebrae level, which made the exposure difficult. Quick stenting and exclusion of pseudoaneurysm and decompressive craniectomy in the same sitting led to early and better recovery of the child while taking care of all the nuisances of the illness. Endovascular repair is more often chosen initially for patients with pseudoaneurysms due to traumatic mechanisms or as a result of carotid dissection.[1011] Endovascular stenting for the treatment of Extracranial carotid artery pseudoaneurysm is possible with acceptable acute and long-term clinical and radiologic outcomes.[1011] Endovascular means of treatment for pediatric neck aneurysms are relatively simple and safe.[6] Endovascular stenting is technically feasible with high procedure success and a relatively low complication rate in patients with EICA.[1011] Future real-world larger studies are required to further substantiate the safety and the long-term outcome of endovascular stent repairs in children.[11] Adequate anticoagulation should be initiated after stent implantation, with particular attention being paid to monitoring graft patency.

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