Ruptured visceral artery aneurysms in a patient of neurofibromatosis type 1 (NF-1) successfully treated by endovascular treatment
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Neurofibromatosis type 1 (NF-1) is an autosomal dominant disease and arteriovenous abnormalities are a well-recognized complication. There are several case reports of ruptured aneurysms; however, among them, reports of superior pancreaticoduodenal artery (PDA) and superior mesenteric artery (SMA) aneurysms are rare. We experienced the case of ruptured PDA and SMA aneurysms in a patient of neurofibromatosis type I successfully treated by endovascular treatment.
A 55-year-old woman with NF-1 came to our hospital with abdominal pain and vomiting. Enhanced abdominal computed tomography revealed a hematoma in the retroperitoneum and an aneurysm in the head of the pancreas. Angiography was performed, and a ruptured aneurysm was suspected the periphery of the PDA, and we embolized it using coils. However, on postoperative day 2, the hemoglobin level decreased, and a branch of the SMA was ruptured. She underwent embolization using coils again and discharged on postoperative day 27 without any further hemorrhage.
To our knowledge, this is the first successfully treated case of ruptured SMA and PDA aneurysms in a patient with NF-1.
KeywordsVisceral artery aneurysms Neurofibromatosis type 1 Endovascular treatment
Neurofibromatosis type 1
Superior pancreaticoduodenal artery
Superior mesenteric artery
Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder affecting one in 3000 people which is characterized by café au lait spots and various features in the bones, eyes, and nervous system. Vascular abnormalities such as arterial stenosis, aneurysms, arterial compression, and arteriovenous abnormalities have also been known [1, 2], of which involvement of renal arteries aneurysms is the most common. However, aneurysms of intracranial, cranial, vertebral, subclavian, intercostal, and visceral arteries are also reported, and spontaneous rupture may occur with fragile vessel walls . We herein report a case of ruptured visceral artery aneurysms in a patient with NF-1 successfully treated by endovascular treatment and review the literature.
NF-1 is a multisystem genetic disorder caused by the mutation of a gene on chromosome 17. Common symptoms include cutaneous findings, most notably café au lait spots and neurofibromas, skeletal dysplasia, tumors of the central and peripheral nervous system, and vision disorders . NF-1 is also associated with vascular abnormalities, which may be complicated by spontaneous rupture . The causes of rupture are reported as follows: (1) Neurofibromatous infiltrates directly into the tunica media, and the blood vessel wall becomes weak. (2) Neurofibromatous compress the vasa vasorum of the large artery tissue and the blood vessel wall becomes weak. (3) Tunica media becomes weak because of the smooth muscle proliferation in tunica intima and the elastic membrane becomes weak [1, 6].
Most lesions become evident by age 50 years . Most patients with NF-1 vasculopathy are reported to be asymptomatic, but the patients with ruptured artery aneurysms have an acute presentation with hemodynamic instability and loss of consciousness with localized pain and swelling which could be fatal [3, 5]. In the current case, hemodynamic was stable, but she had a sudden abdominal pain.
Three cases of the rupture of SMA and PDA aneurysm
Mendonça et al.
Huffman et al.
Serleth et al.
Treatment for ruptured aneurysm includes surgery and endovascular treatment, but there is no consensus. It depends on the type, patient’s age, and the location of the lesion. The advantage of endovascular treatment is that it is less invasive, excellent in organ preservation, and rapid recovery [11, 12]. Recently, there have been some cases for which hemorrhage was successfully terminated by endovascular treatment, which seems to be increasingly performed. David et al. reported that endovascular treatment even in hemodynamically unstable patients at all ages appears to be a safe approach . However, there are problems such as difficulty in the procedure, possibility of intestinal necrosis, and report of vascular damage from coils and catheters. To avoid vascular injury due to the fragility of the vessel wall, it is necessary to select appropriately sized coils that match the diameter of the blood vessel or aneurysm. Additionally, a softer coil should be selected as we used in the current case. In particular, it is important to be careful with the trouble of the insertion site in NF-1 because of the weakening of the vessel wall [10, 11]. Open surgery may be required in some cases. In the current case, we chose endovascular treatment because of its minimum invasiveness, and the ruptured aneurysms were successfully treated without intestinal ischemia. Although the follow-up period is not decided, the visceral aneurysms in patients with NF-1 are more likely to rupture, and it is necessary to perform enhanced CT at least 1 year. In the current case, if the jejunal aneurysms are getting bigger, the treatment, such as the coil embolization, will be required again.
We experienced the case of ruptured PDA and SMA aneurysms in a patient of neurofibromatosis type I successfully treated by endovascular treatment. To our knowledge, this is the first successfully treated case of ruptured PDA and SMA aneurysms in a patient with NF-1.
NF wrote this article. HA performed the preoperative management and supervised the writing of the manuscript. MO and KY approved the final submission of the manuscript. ST and KM performed angiography. All authors read and approved the final manuscript.
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The authors declare that they have no competing interests.
- 12.Morita Y, Hasegawa T, Hanawa M. Transcatheter arterial embolization for the pancreaticoduodenal artery aneurysms. Intervent Radiol. 1999;14:334–42.Google Scholar
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