Annals of Clinical and Medical

Case Reports

Case Report ISSN 2639-8109 Volume 14

Endovascular Superior Vena Cava Stenting As Effective Therapy of A Life Threatening

Epistaxis in A Patient with Hereditary Hemorrhagic Telangiectasia
Pierre Yves Marcy (MD)1,*, Lacout Alexis (MD)2 and Mostfa El Hajjam (MD, Associate Professor)3
1
Department of Radiodiagnostics and Interventional Imaging, Polyclinics ELSAN Group, PolyClinics Les Fleurs, Quartier Quiez,
83189, Ollioules, France
2
Radiologist, Centre de diagnostic ELSAN, 15000 Aurillac, France
3
Pluridisciplinary HHT team, Ambroise Paré Hospital, 9- Avenue Charles de GAULLE; 92100 Boulogne Billancourt France, Groupe-
ment des Hôpitaux Ile-de-France Ouest, Assistance Publique - Hôpitaux de Paris Université de Versailles Saint Quentin en Yvelines,
France
Received: 26 Oct 2024 Copyright:
*
Corresponding author:
Accepted: 16 Nov 2024 ©2024 Pierre Yves Marcy. This is an open access article
Pierre Yves Marcy,
Published: 21 Nov 2024 distributed under the terms of the Creative Commons
Department of Radiodiagnostics and Interventional
J Short Name: ACMCR Attribution License, which permits unrestricted use, dis-
Imaging, Polyclinics ELSAN Group, PolyClinics
tribution, and build upon your work non-commercially
Les Fleurs, Quartier Quiez, 83189, Ollioules, France
Citation:
Pierre Yves Marcy (MD), Endovascular Superior Vena
Keywords: Cava Stenting As Effective Therapy of A Life
Superior vena cava stenting; Epistaxis; Hereditary Threatening Epistaxis in A Patient with Hereditary
hemorrhagic telangiectasia; Rendu Osler; Central Hemorrhagic Telangiectasia. Ann Clin Med Case Rep.
venous catheter, Superior vena cava occlusion 2024; V14(9): 1-6

1. Abstract in this elderly patient suffering from severe chronic anemia. In

Indwelling port catheters are nowadays the main etiology of be- August 2016, the venous device occluded and recanalization was
nign superior vena cava (SVC) syndrome. SVC obstruction increa- successfully achieved by using urokinase protocol. Patient subse-
ses the venous blood pressure upstream, and subsequently into the quently presented further exacerbating episodes of epistaxis re-
arterio-venous sac malformations of the nasal cavity, leading to the sulting in acute anemia (hemoglobin 7.7g/dL). In addition, arm
high risk of recurrent epistaxis in hereditary hemorrhagic telan- and facial swelling progressively developped with concomitant
giectasia (HHT) patients. In the present case, percutaneous SVC orthopnea that were consistent with clinical SVC syndrome. CT
recanalization made possible to deal successfully with epistaxis in scan showed SVC occlusion, staged II according to the Stanford
a HHT patient presenting with complete occlusion of SVC related classification [4]. CT scanning displayed a chronic and organized
to central venous catheter secondary misplacement into the azygos thrombus located at the distal tip of the venous catheter that was
venous arch. mislocated into the azygos vein arch (Figure 1A, B). Percutaneous
SVC recanalization was successfully performed through the right
2. Case Description
common femoral vein, using a steel Wallstent endoprothesis 18
A 87-year-old man (body mass index 30) with known HHT di- mm x 60 cm, a 11French introducer sheath, a 0.0035in guide wire.
sease presented with SVC syndrome, and worsening of epistaxis. Final angiography showed SVC lumen complete reopening (Figu-
The epistaxis had been treated by conventional therapy [1], bila- re 1C). Venous obstruction related symptoms, particularly epista-
teral surgical ligation of sphenopalatine arteries [2], several per- xis, resolved immediately and durably. The port catheter was sur-
cutaneous arterial embolization procedures [3] and laser therapy gically explanted two days after the endovascular treatment. The
in 1985 and 1986 respectively. In 2010, owing to the gravity of SVC stent was controlled patent at 6 months CT scan follow-up
epistaxis, an implantable venous device was placed into the SVC, (Figure 1D) and episodes of epistaxis never recurred clinically at a
through the right subclavian vein, to perform blood transfusion follow up of eight years.

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Figure 1A: Pre-Treatment Findings Chest X-Ray shows catheter tip hooked into azygos vein arch.

Chest front x- ray shows subclavian vein (SCV) port catheter tip mislocation into azygos arch. Key feature displays a retracted catheter tip inclined
medially to the SVC long axis (arrow). Secondary catheter tip displacement was likely due to severe coughing or vomiting efforts.

Figure 1B: Caval angiography displays SVC occlusion (Azygos Catheter tip, arrow) and dilated venous cervical collateral pathways (oblique arrows).

Percutaneous SVC recanalization was successfully performed through the right common femoral vein, using a 18mmx 60mm steel Wallstent endopro-
thesis, a 11French introducer sheath, a 0.0035in-guide wire.

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Figure 1C: Caval angiogram displays antegrade SVC flow, disappearance of collaterals after SVC stent placement.

Post- SVC stenting phlebography shows complete reopening of the SVC while disappearance of the collateral veins. SCV catheter will be retrieved
few days after the SVC stenting procedure.

Figure 1D: Enhanced – Chest CT control at 6months follow-up. SVC stent is patent

Chest CT scan follow-up at six months confirms durable SVC patency, without collateral vein opacification. SVC clot has completely disappeared.

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Volume 14 Issue 9 -2024 Case Report

Figure 1E: Scheme of arteriovenous sac malformation (AVM) sac, before SVC stenting. The dilation of the nasal AVM sac is due to increased blood
pressure and retrograde flow (red arrows) into the SVC, internal jugular veins, facial veins and the AVM’s sac dilated efferent vein. SVC clot (*) ex-
plains the SVC occlusion related to secondary catheter tip misplacement. Double black arrow stigmatizes the rupture of the dilated AVM’s sac, and
subsequent nose bleeding.

Figure 1F: Scheme of arteriovenous sac malformation, after SVC stenting. The AVM efferent vein flow has become antegrade thanks to the SVC
reopening (vertical arrow). AVM’s sac diameter has dramatically decreased, active nose bleeding definitely will stop.

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3. Discussion metallic 18 mm x 60mm Wallstent endoprothesis. Downsizing risk

SVC syndrome was first described in 1795 and is well documented is secondary cardiac embolization [9]. Patients with concerning
in the scientific literature. More than 80% of reported cases are due symptoms should have their stent surgically removed or retrieved
to malignant obstruction. Indwelling port catheters are nowadays by endovascular methods [10]. In the present case, SVC obstruc-
the main cause of benign SVC occlusion [5, 6]. Dedicated studies tion promoted facial and intracranial venous hyperpressure, thus
have shown that the risk of SVC occlusion is higher when the im- increasing pressure into the efferent veins of the nasal mucosae
planted catheter is too short, particularly when located in front of HHT malformation, secondary nose arterio-venous malformation
the azygos vein arch or at the confluence of the innominate veins, sac dilation and rupture, which explains the occurrence of the epis-
and in case of left sided insertion [7, 8]. As initial chest x- ray taxis. As arterio-venous malformation sac pressure dropped dra-
showed satisfactory implantation of the central venous catheter tip matically after successful SVC stenting, nosebleeds stopped even-
2cm above the atrial caval junction, we assume that secondary ca- tually, and SVC clinical symptoms completely resolved.
theter malposition into the azygos vein occurred for intrathoracic 4. Conclusion
hyperpressure reason. Thus we strongly advocate for 2cm – over- The use of central venous catheters is a common iatrogenic cause
trimming the central venous catheter when left-sided inserting or of SVC syndrome. In HHT patients, central venous occlusion not
in case of obese patients. Overweighted patients often present with only leads to a series of clinical complaints but also causes dys-
perivenous loose connective tissue and potential venous catheter function of the central venous catheter and jeopardizes the future
loops giving a higher elasticity reserve to the inserted catheter, of suitable vascular access in fragile patients who often require
thus a higher risk of secondary catheter displacement. blood transfusion in case of severe and chronic epistaxis. Stan-
Patients with benign SVC syndrome have a normal life expec- dard rules should be undertaken to prevent risks of catheter-related
tancy when compared to SVC patients presenting with malignant complications into the central venous system, particularly optimal
disease. Percutaneous SVC endoprothesis insertion is a safe and positioning of the catheter tip, namely when left sided (i.e. at or
efficient percutaneous procedure and definitely is the treatment of even below the cavo-atrial junction).
choice of benign SVC syndrome, allowing a long-term relief of 5. Funding
the clinical symptoms with a primary patency rate > 90% [5,6]. The authors declare that this study received funding from Associ-
However some life threatening SVC stenting complications have ation BonSens.org to cover the publication fees.
been reported to date, including the following: acute recurrence of
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