In comparison to 1947, Bauersfeld first case report, SISMAD has recently been frequently reported due to the increased use of advanced technology in diagnostic imaging studies. Technological development, particularly in imaging diagnosis, lets us believe in the significant number of cases reported every year. With this fact in mind, there is a need to establish a universal treatment regimen based on evidence-based findings in clinical practice with a significant number of patients. Many authors reported gender difference in the distribution of this disease mainly affecting males in the mean age of fifties of there life (
13,
16). The patients who underwent endovascular treatment in our study were 27 male and four female with the mean age of 52.9 years that correlated with many findings.
SISMAD presentation could be described as symptomatic or asymptomatic according to abdominal pain. Abdominal pain is often associated with nausea, vomiting and sometimes diarrhea and passing bloody stool. In this study, all 31 patients presented with abdominal pain that presented as either localized abdominal pain or radiating to the back, but we did not find any patients with intestinal ischemia that makes us believe the dissection itself particularly the length of dissection may had been the significant source of pain as it was reported in other findings where there was association of dissection and inflammation through stimulation of visceral nerve plexus.
Apart from intestinal ischemia, many other factors may play a role in abdominal pain such as aberrant hemodynamic strength due to the convex morphology of the SMA particularly at 1.5 cm 3 cm from the origin which may cause abdominal pain (
17). This area of SMA is very important, especially in deciding the deployment of the stent. Similar to many vascular diseases, SISMAD is associated with risk factors such as smoking, diabetes, atherosclerosis, medial cystic necrosis, fibromuscular dysplasia, abnormalities of elastic fiber (Marfan syndrome and Ehlers-Danlos syndrome), trauma, as well as untreated hypertension (
1,
4,
9). Like another study, our patients presented with relevant associated vascular risk factors including hypertension, history of smoking, atherosclerosis and diabetes mellitus. No patients had an identifiable genetically related vascular disorder. In our findings, we hypothesize that both atherosclerosis and hypertension contribute significantly to the pathogenesis of dissection through destruction of vascular wall collagen and elastic fiber which in turn causes wall stiffness that results in dissection.
Currently, CTA is the preferred imaging modality in detecting and assessing SISMAD. Mural clot formation, intramural bleeding, intimal flap and enhanced attenuation around the SMA are a significant sign of SISMAD on CTA (
18). The pathognomonic finding of SISMAD is the presence of intimal flap in cross-sectional imaging. In general, CTA is more accurate, non-invasive and will be able to diagnose quickly especially in most cases of acute abdominal pain. On the other hand, catheter angiography is more superior in assessing collateral circulation and the relationship of the lesion to branching vessels. Nevertheless, angiography may fail to show the lesion in case of SMA dissection in patients with a complete thrombosed false lumen (type III). Angiography is an invasive procedure; hence, this procedure should be preserved and used only to those patients with worsening symptoms, who need endovascular or surgical treatment. Our final patient diagnosis was based on CTA, and the confirmatory study was done during angiography imaging.
Based on angiographic findings found in the study conducted by Yun et al. (
15), our patients belong to type IIA 11 and type IIB 17 with no type I or type III found. Solis et al. (
8) hypothesis stated that usually dissection begins between 1.5 cm and 3 cm from the origin of the SMA, hence sparing the proximal origin of the artery. Our findings are similar to the hypothesis above whereby the mean distance of SMA ostium to the beginning of SMA dissection was 2.5 cm equally to the findings of the study performed by Solis et al. (
8). Based on this, we highly recommend that in lesions that occur at this specific region, the stent should extend up to the origin and protect the convex curvature force that may cause stent migration. In case the lesion is further extended distally and the primary stent is not enough, the overlapping stent comes to play. The mean length of the SMA dissection was 4.79 cm.
The treatment regimens are still not well-established; there is a different approach such as conservative, endovascular and open surgical treatment (
6,
19-
23). Conservative approach includes use of antiplatelet drugs, anticoagulants, control blood pressure and bowel rest. However recently risks and failures related to conservative treatment have been reported (
24,
25) whereby there is report of patients developing recurrent clinical features and conditions worsening secondary to failure of the non-operative approach. These findings illustrate the treatment approach need close follow-up. Though it does not prevent disease progression but should be considered as an option for some asymptomatic patients (
26,
27). Endovascular and open surgery treatment generally are reserve options for the cases that abdominal pain does not subside, and there is clear evidence of signs indicative of bowel ischemia. In 2000, Leung et al. (
28) reported the first successful case of SISMAD who was treated by endovascular stent placement using a wall stent. Subsequently, Froment et al. (
29) came up with the recommendation in which endovascular stent treatment was proposed as a preferred treatment of choice. Their proposition was a result of the study they conducted in which they reported a failure rate of 38.5% among 13 asymptomatic patients who received conservative treatment, which increases to 50% in symptomatic patients. Eventually, several authors started reporting stent placement as a safe, effective, and successful treatment in the management of symptomatic SISMAD. Recently, endovascular stent placement is reported as the first treatment choice with good clinical outcomes for the management of symptomatic patients or as a secondary treatment after conservative management failure (
9,
12). Furthermore, the indications for endovascular treatment should not only be based on the presenting symptoms or percentage of true lumen occlusion but angiographic findings and the presence of collateral circulations through SMA side braches (
30). In China, where our study originated, endovascular stent placement is considered as the first line of management for symptomatic patients. Hypothetically, in recent years, endovascular treatment has provided additional advantages compared to open surgery. Endovascular treatment is less invasive because of the reduced time of the healing process, the reduced time needed for immobilization and reduced infections. Overall, it is more sufficient in treating symptomatic patients with severe co-morbidities who are unfit for open surgery (
6,
16,
21,
22).
In our experience, the endovascular treatment was successful in all patients. We selected a flexible bare self-expanding stent with a less radial force. This type of stent is suitable for the weak vascular wall and original curved site (
31). A bare stent is sufficient enough in opening the true lumen and allowing normal flow through the distal part of the SMA and endothelialization of the stent with the formation thrombus in a false lumen. In this study, it was found that both bare stents alone (straight or tapered), overlapping stent or stent-assisted coiling showed significant success outcomes with long-term patency in aneurysm lesions. Furthermore, all clinical findings resolved rapidly following endovascular treatment. In our findings, we did not encounter any case of restenosis or stent migration and this shows the best way in selecting the stent is basically to use both straight stents in straight vessels and tapered stents in tapered vessels, as well as overlapping and coil, assisting stent in cases of long dissection and aneurysm dissection, respectively.
Our study emphasized that SISMAD should be included as one of the differential diagnosis among patients presenting with acute or chronic abdominal pain associated with nausea, vomiting or diarrhea who are in the 5th decade of their life. CTA is an ideal imaging modality in the investigation of these conditions. It would be effective in establishing the location of the entry site, dissection length, and presence of pseudo-aneurysm. The radiologist should consider the presence of intimal flap in cross-sectional modalities as the key finding in establishing the presence of dissection. Endovascular stent placement is recommended in symptomatic patients especially in severe co-morbidity patients who are considered unfit for open surgery. The ideal choice of the stent should be a soft self-expandable stent, though it is more expensive. Radiological imaging morphology should dictate endovascular treatment especially whether to use a straight or tapered stent, overlapping stent or coil assisting.
Our study had several limitations. The study was a retrospective clinical case review with patients who received endovascular treatment. In addition, it was a single-institute study with a relatively small number of patients for conducting and analyzing statistical tests. Prospectively randomized clinical studies with a larger number of patients in collaboration will determine whether it is significantly different in the endovascular outcome between a patient with acute symptoms and those with chronic symptoms.
In conclusion, SISMAD is a rare vascular disease that presents with abdominal pain, nausea, vomiting, diarrhea or it may be asymptomatic. Recently it has become frequently reported due to the increased use of advanced technology in diagnostic imaging studies. Endovascular stent placement is a safe, effective, and successful treatment in the management of symptomatic SISMAD. Appropriate endovascular procedures to treat patients based on medical imaging results is a key point especially in patients with tapered vessels, a longer dissection lesion, and dissection aneurysm.