Author + information
- Katsushi Amemiya1
Patient initials or identifier number
Relevant clinical history and physical exam
She is a 40 years-old woman. At 35; aortic dissociation (Stanford A) developed and ascending and archre placement was done. At 41 year-old, TEVAR and EVAR for enlargement of residual dissection were performed. In August 2016, she suffered from severe pain in her right calf, followed by swelling. She visited our hospital and CT showed an aneurysm in the branch of right peroneal artery with leakage of contrast media. Emergency angiography planned.
Relevant test results prior to catheterization
Laboratory findings showed mild anemia (Hb11.4g/dl), normal renal function (Cr 0.79 mg / dl, eGFR 63.5 mL/min) and CK (60 U/L). But elevated D-dimer (24.6 ug/ml) and CRP (7.55 mg/dl). 3D CT showed swelling of the right calf and aneurysmal formation with extravasation, status after ascending/arch replacement and stent graft in the descending thoracicaorta, abdominal aorta and the right iliac artery. Large residual false lumen with typeII endoleak was seen. Renal artery was stented.
Relevant catheterization findings
The right lower limb angiography revealed a huge pulsating aneurysmal formation (8.5 × 11.7 mm) with extravasation in the branch of peroneal artery. The left lower limb angiography revealed irregularity of the vessel of superficial femoral artery and a small aneurysmal formation in the small branch of peroneal artery.
1. A 4.5 Fr sheath guide was introduced via the right common femoral artery antegradely.
I planned coil embolization for this aneurysm.
2. We advanced a 0.014 guide wire and a micro catheter with support of a 4Fr multi purpose catheter. Two micro coils (2/20 mm) were deployed in feeding artery proximal to the aneurysm.
3. Next shot demonstrated a tiny collateral from the posterior tibial artery leading to the aneurysm. We added coil embolization with 2 micro coils (2.0/5.0 mm) using the same system. Then a final angiography showed complete disappearance of the aneurysm.
4. 5 days later, we performed angiography of the left lower limbs artery because a small aneurysm was also suspected in CT.
5. A 4Fr sheath inserted in the left common femoral artery and we confirmed a small aneurysm existed in the small branch of the peroneal artery.
6. A 4Fr multipurpose catheter advanced to the proximal of the left tibioperoneal trunk. It was difficult to isolate the small branch arising from the very proximal part of peroneal artery.
7. A 0.014 guide wire and a micro catheter were advanced to the target branch and deployed 2 micro coils (2/20 mm). The aneurysm was successfully excluded.
8. Although other very tiny aneurysms were visualized, we did not performed additional coil embolization because they were too small.
We finally diagnosed her as Vascula Ehlers-Danlos syndrome (EDS) based on the diagnostic criteria. Vascular EDS is a rare disease with one of 50,000-250,000 people with autosomal dominant inheritance. The prognosis was poor; average expected life spam is 48 year-old and 90% of them develop fatal complications (arterial dissection, rupture, uterus rupture,etc). Tissue and vessel are so fragile that invasive examination like angiography should be avoided in patient with EDS. Therefore, we believe this is reportable case because she survived after several invasive treatments through artery and this time we succeeded to fix the aneurysmal rupture by coil embolization.