Fig. 23.1
Superior mesenteric artery and the aorta form an acute aorto-mesenteric angle (a). In superior mesenteric artery syndrome, the angle is markedly narrowed resulting in compression of the third portion of the duodenum (b, c)
Although patient demographics and presenting symptoms are similar between superior mesenteric artery syndrome and megaduodenum, the former is postulated to be a mechanical obstruction without underlying myopathy ruled out by duodenal biopsies whereas the latter is a hereditary motility disorder [4, 6, 14]. SMA syndrome often remains an ambiguous diagnosis as it can be overshadowed by co-exisitingmedical conditions involving severe malnutrition, psychosocial eating- related disorders and substance abuse [14, 22]. Surgeons tend to be consulted for SMA syndrome when medical therapy and conservative management fail and generally focus on the mechanical plausibility of the diagnosis based on imaging. The indications for surgery remain a challenge since the diagnosis of SMA syndrome is typically made clinically since there is much variation in the interpretation and significance of imaging studies. It should be noted that imaging studies do not rule in the diagnosis of SMA syndrome, most often they rule it out. For these reasons, the diagnosis of SMA syndrome often remains ambiguous at best with the diagnosis confirmed when patients symptoms improve following surgery. Results of surgical outcomes are by and large incomplete and thus should be viewed with caution.
In general the diagnosis of SMA syndrome is suspected when patients can no longer maintain their weight without exogenous nutritional support, display symptoms suggestive of duodenal obstruction, and have had all other potential causes ruled out. It is good practice to first have patients screened in an eating disorders clinic by a specialist including a dietician. Once the possibility of an eating disorder is ruled out, the diagnosis is considered when an upper gastrointestinal contrast study and CT angiogram are together suggestive of SMA syndrome. Median arcuate ligament syndrome, which can cause symptoms similar to SMA syndrome, should also be ruled out, as well as any endoluminal or extrinsic obstructive cause of duodenal obstruction.
The radiologic criteria for the diagnosis of SMA syndrome can be highly subjective and a comprehensive review of the literature is beyond the scope of this review. In general, upper barium study should be performed by an experienced radiologist who is familiar with the diagnosis. Additionally a CT angiogram should confirm that there is narrowing of the aortomesenteric window. Surgeons considering intervention should realize that there is much variability in the measurement of the aortomesenteric window from one radiologist to another and much subjectivity in the interpretation of the upper barium study. While the degree of angulation at the aortomesenteric site is used as criteria with specific numerical cutoffs, there is no consensus among radiologists as to how the angle is measured. Consideration of surgery should involve clear communication between the radiologist and surgeon as to the findings on imaging.
Once conservative measures have failed and the patient can no longer maintain their weight within a healthy range, surgery should be considered. Surgical options include enteric bypass (side-to-side duodenojejunostomy or gastrojejunostomy) or mobilization of the duodenum at the ligament of Treitz (Strong’s procedure).
Search Strategy
A literature search of English language publications from 1921 to 2014 was used to identity published data on surgical treatment of superior mesenteric artery syndrome. Databases searched were PubMed, Ovid, and GoogleScholar. Terms used in the search were “Superior Mesenteric Artery Syndrome”, “Wilkie’s Syndrome”, “Cast Syndrome”, “Duodenal Ileus”, “Aortomesenteric Compression Syndrome”, “Duodenojejunostomy” AND (“Open” OR “Laparoscopic”), “Gastrojejunostomy” AND (“Open” OR “Laparoscopic”), “Strong’s Procedure” AND (“Open” OR “Laparoscopic”), and “Duodenal Mobilization” AND(“Open” OR “Laparoscopic”). Reference lists of the retrieved publications were manually reviewed for additional publications. We noted that majority of large, comprehensive series dated back to 1960–1980s, while most recently only small case series and case reports are available. The data was classified using the GRADE system (Table 23.1).
Table 23.1
PICO table
P (patients) | I (intervention) | C (comparator) | O (outcomes) |
|---|---|---|---|
Patients with superior mesenteric artery syndrome | Duodenojejunostomy | Duodenal mobilization (Strong’s procedure) | Symptom resolution |
Results
Clinical Results of Duodenojejunostomy
Duodenojejunostomy was first described by Bloodgood in 1907 and performed by Stavely in 1908 (Fig. 23.2). It was shown to be a successful treatment option for SMA syndrome by Wilkie in 1921 [1]. Since, it has been the most frequently utilized operative procedure for treatment of this condition, having a published success rate of around 80 % [23–25]. In 1978 Lee and Mangla published a review of 146 patients surgically treated for SMA syndrome, concluding that duodenojejunostomy had superior outcomes to both Strong’s procedure and gastrojejunostomy [26]. Their quoted success rate was 90 % in terms of symptomatic relief. In 1984, Gustafsson et al. published a 100 % success rate in ten patients treated with duodenojejunostomy [3]. In 1989, a case series of 16 operative patients showed the opposite results; only one patient achieved complete symptom resolution, while the only significant improvement was decreased frequency of vomiting in the others [4]. The first successful laparoscopic duodenojejunostomy was described in 1998 by Gersin and Heniford [27]. More recently in 2009, Merrett et al. described eight patients treated with duodenojejunostomy with duodenal division, reporting 100 % success rate demonstrating no evidence of obstruction on imaging and weight gain in all eight patients post-operatively; however, the details of post-operative assessments and symptom resolution were omitted [14]. In 2010, Munene et al. published a literature review of nine case reports of patients with SMA syndrome treated with laparoscopic duodenojejunostomy reporting a 100 % success rate for the operation in ten patients. However, follow-up data was lacking and the criteria used for determination of operative success was missing [28]. In 2012, Lee at al. published a 100 % success rate for eight patients who underwent laparoscopic duodenojejunostomy and a 100 % success rate for two patients who underwent open duodenojejunostomy [22]. Retrospective review by Pottorf et al. of 12 cases of SMA treated with laparoscopic duodenojejunostomy report 92 % success in symptom improvement [29]. Most published studies suffer from a very small sample size, short follow-up, and lack of information regarding the criteria used to determine long term success (Table 23.2). Other small case reports consisting of one or two patients, revealed similar conclusions and suffer from the same lack of objective preoperative assessment tools compared to blinded postoperative assessment in the long term [30, 31, 35].
Fig. 23.2
Duodenojejunostomy
Table 23.2
Quality of follow up data in more recently published studies
Reference | Number of patients | Intervention | 6 month outcome reported | Details of follow up | Quality of follow up (0–3 points) |
|---|---|---|---|---|---|
Gustafsson et al. [3] | 10 | Open duodeno-jejunostomy | Yes – successful in 10/10 | Minimal | 1 |
Ylinen et al. [4] | 16 | Open duodeno-jejunostomy | Yes – successful in 3/16 only | Detailed | 3 |
Gersin et al. [27] | 1 | Laparoscopic duodeno-jejunostomy | No | Lacking | 0 |
Richardson et al. [29] | 2 | Laparoscopic duodeno-jejunostomy | No | Lacking | 0 |
Kim et al. [30] | 2 | Laparoscopic duodeno-jejunostomy | Yes – successful in 2/2 | Minimal | 1 |
Merett et al. [14] | 8 | Open duodeno-jejunostomy | Yes – successful in 8/8 | Minimal | 1 |
Singaporewalla et al. [31] | 1 | Laparoscopic duodeno-jejunostomy | No | Lacking | 0 |
Munene et al. [28] | 1 | Laparoscopic duodeno-jejunostomy | No | Lacking | 0 |
Lee et al. [22] | 8 | Laparoscopic duodeno-jejunostomy | Yes – successful in 8/8 | Minimal | 1 |
4 | Open duodeno-jejunostomy | Yes – successful in 4/4 | Minimal | 1 | |
2 | Open gastro-jejunostomy | Yes – successful in 1/2 | Minimal | 1 | |
Pottorf et al. [29] | 12 | Laparoscopic duodeno-jejunostomy | No | Lacking | 0 |
Massoud [32] | 4 | Laparoscopic duodenal mobilization | Yes – successful in 3/4 | Detailed | 2 |
Villalba et al. [33]
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