Wilkie syndrome. What is this
M. Stamatakos, K. Kontzoglou, Ch. Stefanaki, S. Tsaknaki, R. Iannescu, A. Manta, M. SafioleasGeneral reports, no. 1, 2009
* 2nd Department of Propedeutic Surgery, School of Medicine, Athens University, Laiko Hospital, Greece
* 2nd Department of Propedeutic Surgery
* 4th Surgical Department of Surgery, School of Medicine, Attikon General Hospital, Greece
Introduction
Superior mesenteric artery syndrome (SMA syndrome, also known as Wilkie's syndrome,) is a rare condition first described in 1861, by Rokitansky (1). It occurs when the third (transverse) portion of the duodenum gets entrapped under the superior mesenteric artery. Anatomically, the duodenum passes across the abdominal aorta just below the origin of the SMA. In some cases, various structural anomalies change the angle between the superior mesenteric artery and the aorta (which is normally 45°). This change creates pressure to the duodenum, resulting in to obstruction of its lumen. In the English-language medical literature, there are over 400 cases of SMA syndrome reported, constituting this an unusual cause of upper intestinal obstruction. Some researchers and physicians however still doubt the syndrome's validity (2-6).
Pathophysiology
The SMA usually forms an angle of approximately 45° (range, 38-56°) with the abdominal aorta, and the third part of the duodenum crosses caudaly to the origin of the SMA, coursing between the SMA and aorta. The SMA usually arises from the anterior aspect of the aorta at the level of the L1 vertebral body. It is enveloped in fatty and lymphatic tissue and extends in a caudal direction at an acute angle into the mesentery. In the majority of patients, the angle between the SMA and the aorta is about 25 to 60 degrees, due in part to the mesenteric fat pad. In the SMA syndrome, the angle is reduced to as low as six degrees due to the loss of the mesenteric fat pad, allowing the SMA to compress the duodenum against the aorta. Any factor that sharply narrows the aortomesenteric angle to approximately 6-25° can cause entrapment and compression of the third part of the duodenum as it passes between the SMA and aorta, resulting in SMA syndrome (7-10).
In addition, the aortomesenteric distance in SMA syndrome is decreased to 2-8 mm (normal 10-20 mm). Alternatively, other causes implicated in SMA syndrome include high insertion of the duodenum at the ligament of Treitz, a low origin of the SMA, and compression of the duodenum due to peritoneal adhesions (11).
Such causes as mentioned above are:
1. Constitutional factors: thin body build, curvature of the spine or laxity of the abdominal walls with a drop in the position of the internal organs,
2. Rapid or severe weight loss in conditions such as cancer or extensive burn injuries, prolonged bedrest, anorexia, or malabsorption syndromes (12-17).
3. Disease deformity, or trauma to the spine or anatomical variants (18-22).
4. Use of a body cast in the surgical treatment of scoliosis or vertebral fractures.
SMA cases after corrective spine surgery, are due to the result of spinal elongation, which decreases the superior mesenteric/aortic angle. Postoperative weight loss is an important factor for the development of SMA syndrome. Although the use of Harrington rods for corrective surgery commonly used in the 1950s and 1960s was an important contributory factor for development of SMA syndrome, newer derotation/translation corrective techniques can also be rarely associated with this disease entity (23-27), as rapid growth without proportional weight gain (particularly in teenagers) (28).
Other rare but recognized causes include trauma or aneurysm of the SMA, and familial predisposition toward SMA syndrome (29,30).
Furthermore anatomic anomalies as abnormally high and fixed position of the ligament of Treitz with an upward displacement of the duodenum or unusually low origin of the SMA.
Also, other unusual causes: traumatic aneurysm of the SMA after a stab wound, familial SMA syndrome and Recurrent SMA syndrome (30-34). Finally, SMA syndrome has also been described in pregnancy, when the gravid uterus reduces in abdominal volume (30).
Epidemiology
SMA syndrome is quite rare. The precise incidence of this entity is unknown. In a review of the literature, approximately 0.013-0.3% of the findings from upper GI tract barium studies support a diagnosis of SMA syndrome. No racial differences have been identified. The SMA syndrome usually occurs in older children and adolescents. In one report by Geer, 75% of the cases occurred in patients aged 10-30 years. Due to the general nature of the symptoms, it is difficult to estimate the frequency of cases in the general population. More females are affected by SMA syndrome. In one large series of 75 patients with SMA syndrome, two thirds of the cases involved women, with an average age of 41 years; one third of cases involved men, with an average age of 38 years. Thus, it does not seem to be more common in any particular race, however seems to strike women about twice as often as men. The syndrome usually occurs in older children and teenagers. About 3/4 of all cases occur between the ages 10 and 30 (33,35-40).
Presentation and Clinical features
The presentation of SMA syndrome can be chronic, intermittent or acute, and can result in either complete or partial duodenal obstruction. Patients often present with chronic symptoms related to the upper abdomen.
These may include: epigastric (upper mid-abdominal) pain, nausea, eructation (belching), vomiting of large quantities of bilious or partially digested material, discomfort after meals, early satiety (loss of appetite) and subacute small bowel obstruction.
These symptoms are often relieved by lying down on the left side, prone (face down), or with the knees to the chest; they can be aggravated in the supine (face up) position (41-45).
Diagnosis
Physical findings in SMA syndrome are often vague or generalized and the diagnosis of this condition often comes by exclusion. About eight out of ten patients are thin and sickly (asthenic habitus). They often suffer from peptic ulcer disease (25-45%), or hypersecretion of stomach acid (hyperchlorhydria). The presentation can mimic that of subacute small bowel obstruction. Examination can reveal tenderness or pain, fullness, or a succussion splash. Pressure from below the navel upward and toward the back (Hayes maneuver) can lift the SMA, releasing the obstruction and cause relief of both subjective and objective signs. Repositioning during the examination may also afford symptomatic relief.
As it was previously reported the definitive diagnosis of SMA syndrome is difficult. Xray and other imaging studies that may be helpful include (46-51).
Upper GI study with barium contrast - can show dilatation of the first and second portions of the duodenum with a sudden cutoff around the midline.
Computed tomography (CT scan) of the abdomen is useful in the diagnosis of the SMA syndrome and can provide diagnostic information including the aorta-SMA distances and duodenal distension. It can also be used to assess intra-abdominal and retroperitoneal fat. Another diagnostic tool is fluoroscopy with contrast.
Upper GI endoscopy (esophogastroduodenoscopy) may be necessary to exclude mechanical causes of duodenal obstruction. However, the diagnosis of SMA syndrome may be missed with this study.
Finally, abdominal ultrasonography may be helpful in measuring the angle of the SMA and the aortomesenteric distance.
Differential diagnoses
Differential diagnoses for SMA syndrome include eating disorders such as anorexia nervosa and bulimia and other metabolic conditions including diabetes and collagen vascular disease. There are other causes of obstruction and pseudo-obstruction that should also be considered (7, 39, 42).
Treatment
Initially, conservative treatment including intravenous and oral fluids, nutrition, decompression, and proper positioning after food intake is recommended. Enteral feeding by nasogastric tube can be helpful as part of the treatment of rapid or severe weight loss. Metoclopramide, a pro-motility agent, may be beneficial. It stimulates motility of the GI tract. GI smooth muscle is regulated by autonomic innervation, local reflexes, and hormones. Peristalsis is induced and causes the gut contents to move, encouraging digestion. Medical management of SMA syndrome works best in cases which present acutely. Reversing or removing the precipitating factor is usually successful in a patient with acute SMA syndrome. Conservative initial treatment is recommended in all patients with SMA syndrome; this includes adequate nutrition, nasogastric decompression, and proper positioning of the patient after eating (ie, left lateral decubitus, prone, or knee-to-chest position). Enteral feeding using a double lumen nasojejunal tube passed distal to the obstruction under fluoroscopic assistance is an effective adjunct in treatment of patients with rapid severe weight loss and also eliminates the need for intravenous fluids and the risks associated with total parenteral nutrition. In some instances, both enteral and parenteral nutritional support may be needed to provide optimal calories. The patient's weight should be monitored daily. Subsequently, the patient can be started on oral liquids followed by slow and gradual introduction of small and frequent soft meals as tolerated. The patient should be encouraged to stay prone or in the left lateral decubitus position after any oral intake to facilitate passage of intraluminal material past the obstruction. Symptoms often resolve once weight is regained and the fat pad is reconstituted. Finally, regular solid foods are introduced. Metoclopramide treatment may be beneficial.
Surgical intervention is indicated when conservative measures are ineffective, particularly in patients with a long history of progressive weight loss, pronounced duodenal dilatation with stasis, and complicating peptic ulcer disease (52-55). In conclusion, treatment includes an attempt at feeding to improve nutrition. If this fails, various surgical procedures have been advocated. Duodenojejunostomy, during which the compressed portion of the duodenum is released and an anastomosis created between the duodenum and jejunum anterior to the SMA, is the definitive procedure of choice and is successful in about 90% of cases. However, other procedures including gastrojejunostomy, Roux-en-Y duodenojejunostomy and anterior transposition of the third part of the duodenum have been reported (56-62). The use of laparoscopic surgery (63) that involves lysis of the ligament of Treitz and mobilization of the duodenum has been reported as the duodenal-jejunal junction has been fully mobilized, the jejunum is passed behind the SMA and positioned to the right of the SMA so it does not lie in the acute angle between the aorta and the SMA; however, laparoscopy is being used investigationally.
Natural course
Delay in the diagnosis of acute or chronic SMA syndrome can result in malnutrition, dehydration, electrolyte disorders, and death. The acute form if untreated can result in dehydration and even intestinal perforation.
Prognosis
With proper conservative treatment in acute cases, and appropriate medical or surgical management in more chronic cases, the success rate in treating SMA syndrome is high. It is imperative to discover the underlying cause of the condition and treat that as well.
Conclusions
SMA syndromeis a rare benign disease,which responds positively in both therapies medical and surgical if properly used.
References
1. Rokitansky, C. - Handbuch der Pathologischen Anotomie. Vienna: Branmiller and Siedel 1842.
2. Baltazar, U., Dunn, J., Floresguerra, C. - Superior mesenteric artery syndrome: an uncommon cause of intestinal obstruction. South Med. J., 2000, 93:606.
3. Welsch, T., Buchler, M., Kienle, P. - Recalling Superior Mesenteric Artery Syndrome Digestive Surgery, 2007, 24:149.
4. Wilson-Storey, D., MacKinlay, G.A. - The superior mesenteric artery syndrome. J. Roy Coll. Surg. Edin., 1986, 31:175.
5. Wilkie, PD. - Chronic duodenal ileus. Am J Med Sci, 1927, 173:643.
6. Plesa, A., Constantinescu, C., Crumpei, F., Cotea, E. - Superior Mesenteric Artery Syndrome: An unusual cause of intestinal obstruction. J. Gastrointest. Liver. Dis., 2006, 15:69.
7. Niaz, Z., Chaudhary, F., Gardazi, J. - Superior mesenteric artery syndrome: An uncommon cause of upper intestinal obstruction. JCPSP, 2006, 16:666.
8. Raissi, B., Taylor, B., Traves, D. - Recurrent superior mesenteric artery (Wilkie's) syndrome: a case report. Can. J. Surg., 1996, 39:410.
9. Ylinen, P., Kinnunen, J., Hockerstedt, K. - Superior mesenteric artery syndrome. J. Clin. Gastroenterol., 1989, 11:386.
10. Wilkie, PD. - Chronic duodenal ileus. Am. J. Med. Sci., 1927, 173:643.
11. Cohen, L.B., Field, S.P., Sachar, D.B. - The superior mesenteric artery syndrome: the disease that isn't or is it? J. Clin. Gastroenterol., 1985, 7:113.
12. Barnes, J.B., Lee, M. - Superior mesenteric artery syndrome in an intravenous drug abuser after rapid weight loss. South Med. J., 1996, 89:331.
13. Pentlow, B.D., Dent, R.G. - Acute vascular compression of the duodenum in anorexia nervosa. Br. J. Surg., 1981, 68:665.
14. Milner, E.A., Cioffi, W.G., McManus, W.F., Pruitt, B.A. Jr. - Superior mesenteric artery syndrome in a burn patient. Nutr. Clin. Pract., 1993, 8:264.
15. Roy, A., Gisel, J.J., Roy, V., Bouras, E.P. - Superior mesenteric artery (Wilkie's) syndrome as a result of cardiac cachexia. Gen Intern Med., 2005, 20:958.
16. Barnes, J., Lee, M. - Superior mesenteric artery syndrome in an intravenous drug abuser after rapid weight loss. South Med. J., 1996, 89:331.
17. Mehler, P.S., Weiner, K.L. - Use of total parenteral nutrition in the refeeding of selected patients with severe anorexia nervosa. Int. J. Eat. Disord., 2007, 40:285.
18. Altiok, H., Lubicky, J.P., DeWald, C.J., Herman, J.E. - The superior mesenteric artery syndrome in patients with spinal deformity. Spine, 2005, 30:2164.
19. Crowther, A., Webb, P.J., Eyre-Brook, I.A. - Superior mesenteric artery syndrome following surgery for scoliosis. Spine, 2002, 27:528.
20. Hutchinson, D.T., Bassett, G.S. - Superior mesenteric artery syndrome in pediatric orthopedic patients. Clin. Orthop., 1990, 250:250.
21. Munns, S.W., Morrissy, R.T., Golladay, E.S. - Hyperalimentation for superior mesenteric-artery (cast) syndrome following correction of spinal deformity. J. Bone Joint. Surg. Am., 1984, 66:1175.
22. Shah, M.A., Albright, M.B., Vogt, M.T., Moreland, M.S. - Superior mesenteric artery syndrome in scoliosis surgery: weight percentile for height as an indicator of risk. J. Pediatr. Orthop., 2003, 23:665.
23. Shapiro, G., Green, D.W., Fatica, N.S., Boachie-Adjei, O. - Medical complications in scoliosis surgery. Curr. Opin. Pediatr., 2001, 13:36.
24. Vitale, M.G., Higgs, G.B., Liebling, M.S., Roth, N., Roye, D.P. Jr. - Superior mesenteric artery syndrome after segmental instrumentation: a biomechanical analysis. Am. J. Orthop., 1999, 28:461.
25. Zhu, Z.Z., Qiu, Y. - Superior mesenteric artery syndrome following scoliosis surgery: It's risk indicators and treatment strategy. World Journal of Gastroenterol., 2005, 11:3307.
26. Hutchinson, D.T., Bassett, C.S. - Superior mesenteric artery syndrome in pediatric orthopedic patients. Clin. Orthop., 1990, 250:250.
27. Munns, S.W., Morrissy, R.T., Golladay, S., McKenzie, C.N. - Hyperalimentation for superior mesenteric-artery (cast) syndrome following correction of spinal deformity. J Bone Joint Surg Am., 1984, 66(8): pag.1175-1177.
28. Smith, S.J., Cooney, R.N. - Superior mesenteric artery syndrome in a tube-fed patient. Nutr. Clin. Pract., 1994, 9:151.
29. Laffont, I., Bensmail, D., Rech, C., Prigent, G., Loubert, G., Dizien, O. - Late superior mesenteric artery syndrome in paraplegia: case report and review. Spinal Cord., 2002, 40:88.
30. Ortiz, C., Cleveland, R.H., Blickman, J.G. - Familial superior mesenteric artery syndrome. Pediatr. Radiol, 1990, 20:588.
31. Raissi, B., Taylor, B.M., Taves, D.H. - Recurrent superior mesenteric artery (Wilkie's) syndrome: a case report. Can. J. Surg., 1996, 39:410.
32. Ortiz, C., Cleveland, R., Blickman, J., Jaramillo, D., Kim, S. - Familial superior mesenteric artery syndrome. Pediatric Radiology, 1990, 20:588.
33. Sianesi, M., Soliani, P., Arcuri, M.F., Bezer, L., Iapichino, G., Del Rio, P. - Dunbar's syndrome and superior mesenteric artery syndrome: a rare association. Dig. Dis. Sci., 2007, 52:302.
34. Pentlow, B.D., Dent, R.G. - Acute vascular compression of the duodenum in anorexia nervosa. Br. J. Surg., 1981, 68:665.
35. Altman, D.H., Puranik, S.R. - Superior mesenteric artery syndrome in children. Am. J. Roentgenol Radium Ther Nucl Med., 1973, 118:104.
36. Shetty, A.K., Schmidt-Sommerfeld, E., Haymon, M.L. - Radiological case of the month. Superior mesenteric artery syndrome. Arch. Pediatr. Adolesc. Med., 1999, 153:303.
37. Veysi, V., Humphrey,G., Stringer, M. - Superior Mesenteric Artery Syndrome Presenting With Acute Massive Gastric Dilatation. Journal of Pediatric Surgery, 1997, 32:1801.
38. Gamblin, T.C., Stephens, R.E., Johnson, R.K., Rothwell, M. - Adult malrotation: a case report and review of the literature. Curr. Surg., 2003, 60:517.
39. Samdani, P., Samdani, V., Goel, A. - Superior Mesenteric Artery Compression Syndrome. Indian J. Pediatr., 2006, 73:523.
40. Lundell, L., Thulin, A. - Wilkie's syndrome - a rarity? Br. J. Surg., 1980, 67:604.
41. Mackowiak, P., Arentz, M., Kirkpatrick, B., Casey, A. - Vomiting and rapid weight loss in a man with newly diagnosed HIV infection. Clinical Infections diseases, 2005, 41:1196.
42. Fromm, S., Cash, J. - Superior mesenteric artery syndrome: an approach to the diagnosis and management of upper gastrointestinal obstruction of unclear etiology. S D J Med., 1990, 43:5.
43. Lippl, F., Hannig, C., Weiss, W., Allescher, H.D., Classen, M., Kurjak, M. - Superior mesenteric artery syndrome: diagnosis and treatment from the gastroenterologist's view. J. Gastroenterol., 2002, 37:640.
44. Payawal, J., Cohen, A., Stamos, M. - Superior mesenteric artery syndrome involving the duodenum and jejunum Emergency Radiology, 2004, 10:273.
45. Gockel, I., Graupe, F., Hain, J., Lorenz, D. - Superior mesenteric artery (Wilkie) syndrome: different faces. J. Gastroenterol. Hepatol., 2004, 19:720.
46. Khoo, L.A., Belli, A.M. - Superior mesenteric artery stenting for mesenteric ischaemia in Sneddon's syndrome. British Journal of Radiology, 1999, 72:607.
47. Wiesner, W., Khurana, B., Ji, H., Ros, P. - CT of Acute Bowel Ischemia. Radiology, 2003, :635.
48. Flynn, J., Latif, O., Stewart, R., Feldman, L. -Superior Mesenteric Artery Syndrome. Clinical Images in Medicine, 2006, 6:44.
49. Geer, D. - Superior mesenteric artery syndrome. Mil. Med., 1990, 155:321.
50. Santer, R., Young, C., Rossi, T., Riddlesberger, M.M. - Computed tomography in superior mesenteric artery syndrome. Pediatr. Radiol., 1991, 21:154.
51. Neri, S., Signorelli, S.S., Mondati, E., Pulvirenti, D., Campanile, E., Di Pino, L., Scuderi, M., Giustolisi, N., Di Prima, P., Mauceri, B., Abate, G., Cilio, D., Misseri, M., Scuderi, R. - Ultrasound imaging in diagnosis of superior mesenteric artery syndrome. J. Intern Med., 2005, 257:346.
52. Ylinen, P., Kinnunen, J., Hockerstedt, K. - Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J. Clin. Gastroenterol., 1989, 11:386.
53. John, A.S., Tuerff, S.D., Kerstein, M.D. - Nonocclusive mesenteric infarction in hemodialysis patients. J. Am. Coll. Surg., 2000, 190:84.
54. Orihashi, K., Matsuura, Y., Sueda, T., Shikata, H., Morita, S., Hirai, S., Sueshiro, M., Okada, K. - Abdominal aorta and visceral arteries visualized by trans-gastric echocardiography: technical considerations.Hiroshima J. Med. Sci., 1997, 46:151.
55. Coletti, G., Torracca, L., La Canna, G., Maisano, F., Sebastiano, P., Fucci, C., Berra, P., Alfieri, O. - Diagnosis and management of cerebral malperfusion phenomena during aortic dissection repair by transesophageal Doppler echocardiographic monitoring. J. Card. Surg., 1996, 11:355.
56. Eugster, T., Obeid, T., Gurke, L., Wolff, T., Stierli, P. - Acute supramesenteric thrombosis of an abdominal aortic aneurysm with deleterious embolism: a case report. Ann. Vasc. Surg., 2005, 19:411.
57. Goldberg, M., Kim, H. - Treatment of Acute Superior Mesenteric Vein Thrombosis with Percutaneous Techniques. AJR, 2003, 181:1305.
58. Arbell, D., Gross, E., Koplewitz, B., Vromen, A., Barr-Jin, J., Udassin, R. - Superior Mesenteric Artery Syndrome masquerading as recurrent billiary pancreatitis. IMAJ, 2006, 8:441.
59. Rappaport, W.D., Hunter, G.C., McIntye, K.E., Ballard, J.L., Malone, J.M., Putnam, C.W. - Gastric outlet obstruction caused by traumatic pseudoaneurysm of superior mesenteric artery. Surgery, 1990, 108:930.
60. Strong, E.K. - Mechanics of arteriomesenteric duodenal obstruction and direct surgical attack upon etiology. Ann. Surg., 1958, 148:725.
61. Ylinen, P., Kinnunen, J., Hockerstedt, K. - Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J. Clin. Gastroenterol., 1989, 11:386.
62. Schroeppel, T.J., Chilcote, W.S., Lara, M.D., Kothari, S.N. - Superior mesenteric artery syndrome after laparoscopic Roux-en-Y gastric bypass. Surgery, 2005, 137: 383.
63. Massoud, W.Z. - Laparoscopic management of superior mesenteric artery syndrome. Int. Surg., 1995, 80:322.
* 2nd Department of Propedeutic Surgery
* 4th Surgical Department of Surgery, School of Medicine, Attikon General Hospital, Greece
Introduction
Superior mesenteric artery syndrome (SMA syndrome, also known as Wilkie's syndrome,) is a rare condition first described in 1861, by Rokitansky (1). It occurs when the third (transverse) portion of the duodenum gets entrapped under the superior mesenteric artery. Anatomically, the duodenum passes across the abdominal aorta just below the origin of the SMA. In some cases, various structural anomalies change the angle between the superior mesenteric artery and the aorta (which is normally 45°). This change creates pressure to the duodenum, resulting in to obstruction of its lumen. In the English-language medical literature, there are over 400 cases of SMA syndrome reported, constituting this an unusual cause of upper intestinal obstruction. Some researchers and physicians however still doubt the syndrome's validity (2-6).
Pathophysiology
The SMA usually forms an angle of approximately 45° (range, 38-56°) with the abdominal aorta, and the third part of the duodenum crosses caudaly to the origin of the SMA, coursing between the SMA and aorta. The SMA usually arises from the anterior aspect of the aorta at the level of the L1 vertebral body. It is enveloped in fatty and lymphatic tissue and extends in a caudal direction at an acute angle into the mesentery. In the majority of patients, the angle between the SMA and the aorta is about 25 to 60 degrees, due in part to the mesenteric fat pad. In the SMA syndrome, the angle is reduced to as low as six degrees due to the loss of the mesenteric fat pad, allowing the SMA to compress the duodenum against the aorta. Any factor that sharply narrows the aortomesenteric angle to approximately 6-25° can cause entrapment and compression of the third part of the duodenum as it passes between the SMA and aorta, resulting in SMA syndrome (7-10).
In addition, the aortomesenteric distance in SMA syndrome is decreased to 2-8 mm (normal 10-20 mm). Alternatively, other causes implicated in SMA syndrome include high insertion of the duodenum at the ligament of Treitz, a low origin of the SMA, and compression of the duodenum due to peritoneal adhesions (11).
Such causes as mentioned above are:
1. Constitutional factors: thin body build, curvature of the spine or laxity of the abdominal walls with a drop in the position of the internal organs,
2. Rapid or severe weight loss in conditions such as cancer or extensive burn injuries, prolonged bedrest, anorexia, or malabsorption syndromes (12-17).
3. Disease deformity, or trauma to the spine or anatomical variants (18-22).
4. Use of a body cast in the surgical treatment of scoliosis or vertebral fractures.
SMA cases after corrective spine surgery, are due to the result of spinal elongation, which decreases the superior mesenteric/aortic angle. Postoperative weight loss is an important factor for the development of SMA syndrome. Although the use of Harrington rods for corrective surgery commonly used in the 1950s and 1960s was an important contributory factor for development of SMA syndrome, newer derotation/translation corrective techniques can also be rarely associated with this disease entity (23-27), as rapid growth without proportional weight gain (particularly in teenagers) (28).
Other rare but recognized causes include trauma or aneurysm of the SMA, and familial predisposition toward SMA syndrome (29,30).
Furthermore anatomic anomalies as abnormally high and fixed position of the ligament of Treitz with an upward displacement of the duodenum or unusually low origin of the SMA.
Also, other unusual causes: traumatic aneurysm of the SMA after a stab wound, familial SMA syndrome and Recurrent SMA syndrome (30-34). Finally, SMA syndrome has also been described in pregnancy, when the gravid uterus reduces in abdominal volume (30).
Epidemiology
SMA syndrome is quite rare. The precise incidence of this entity is unknown. In a review of the literature, approximately 0.013-0.3% of the findings from upper GI tract barium studies support a diagnosis of SMA syndrome. No racial differences have been identified. The SMA syndrome usually occurs in older children and adolescents. In one report by Geer, 75% of the cases occurred in patients aged 10-30 years. Due to the general nature of the symptoms, it is difficult to estimate the frequency of cases in the general population. More females are affected by SMA syndrome. In one large series of 75 patients with SMA syndrome, two thirds of the cases involved women, with an average age of 41 years; one third of cases involved men, with an average age of 38 years. Thus, it does not seem to be more common in any particular race, however seems to strike women about twice as often as men. The syndrome usually occurs in older children and teenagers. About 3/4 of all cases occur between the ages 10 and 30 (33,35-40).
Presentation and Clinical features
The presentation of SMA syndrome can be chronic, intermittent or acute, and can result in either complete or partial duodenal obstruction. Patients often present with chronic symptoms related to the upper abdomen.
These may include: epigastric (upper mid-abdominal) pain, nausea, eructation (belching), vomiting of large quantities of bilious or partially digested material, discomfort after meals, early satiety (loss of appetite) and subacute small bowel obstruction.
These symptoms are often relieved by lying down on the left side, prone (face down), or with the knees to the chest; they can be aggravated in the supine (face up) position (41-45).
Diagnosis
Physical findings in SMA syndrome are often vague or generalized and the diagnosis of this condition often comes by exclusion. About eight out of ten patients are thin and sickly (asthenic habitus). They often suffer from peptic ulcer disease (25-45%), or hypersecretion of stomach acid (hyperchlorhydria). The presentation can mimic that of subacute small bowel obstruction. Examination can reveal tenderness or pain, fullness, or a succussion splash. Pressure from below the navel upward and toward the back (Hayes maneuver) can lift the SMA, releasing the obstruction and cause relief of both subjective and objective signs. Repositioning during the examination may also afford symptomatic relief.
As it was previously reported the definitive diagnosis of SMA syndrome is difficult. Xray and other imaging studies that may be helpful include (46-51).
Upper GI study with barium contrast - can show dilatation of the first and second portions of the duodenum with a sudden cutoff around the midline.
Computed tomography (CT scan) of the abdomen is useful in the diagnosis of the SMA syndrome and can provide diagnostic information including the aorta-SMA distances and duodenal distension. It can also be used to assess intra-abdominal and retroperitoneal fat. Another diagnostic tool is fluoroscopy with contrast.
Upper GI endoscopy (esophogastroduodenoscopy) may be necessary to exclude mechanical causes of duodenal obstruction. However, the diagnosis of SMA syndrome may be missed with this study.
Finally, abdominal ultrasonography may be helpful in measuring the angle of the SMA and the aortomesenteric distance.
Differential diagnoses
Differential diagnoses for SMA syndrome include eating disorders such as anorexia nervosa and bulimia and other metabolic conditions including diabetes and collagen vascular disease. There are other causes of obstruction and pseudo-obstruction that should also be considered (7, 39, 42).
Treatment
Initially, conservative treatment including intravenous and oral fluids, nutrition, decompression, and proper positioning after food intake is recommended. Enteral feeding by nasogastric tube can be helpful as part of the treatment of rapid or severe weight loss. Metoclopramide, a pro-motility agent, may be beneficial. It stimulates motility of the GI tract. GI smooth muscle is regulated by autonomic innervation, local reflexes, and hormones. Peristalsis is induced and causes the gut contents to move, encouraging digestion. Medical management of SMA syndrome works best in cases which present acutely. Reversing or removing the precipitating factor is usually successful in a patient with acute SMA syndrome. Conservative initial treatment is recommended in all patients with SMA syndrome; this includes adequate nutrition, nasogastric decompression, and proper positioning of the patient after eating (ie, left lateral decubitus, prone, or knee-to-chest position). Enteral feeding using a double lumen nasojejunal tube passed distal to the obstruction under fluoroscopic assistance is an effective adjunct in treatment of patients with rapid severe weight loss and also eliminates the need for intravenous fluids and the risks associated with total parenteral nutrition. In some instances, both enteral and parenteral nutritional support may be needed to provide optimal calories. The patient's weight should be monitored daily. Subsequently, the patient can be started on oral liquids followed by slow and gradual introduction of small and frequent soft meals as tolerated. The patient should be encouraged to stay prone or in the left lateral decubitus position after any oral intake to facilitate passage of intraluminal material past the obstruction. Symptoms often resolve once weight is regained and the fat pad is reconstituted. Finally, regular solid foods are introduced. Metoclopramide treatment may be beneficial.
Surgical intervention is indicated when conservative measures are ineffective, particularly in patients with a long history of progressive weight loss, pronounced duodenal dilatation with stasis, and complicating peptic ulcer disease (52-55). In conclusion, treatment includes an attempt at feeding to improve nutrition. If this fails, various surgical procedures have been advocated. Duodenojejunostomy, during which the compressed portion of the duodenum is released and an anastomosis created between the duodenum and jejunum anterior to the SMA, is the definitive procedure of choice and is successful in about 90% of cases. However, other procedures including gastrojejunostomy, Roux-en-Y duodenojejunostomy and anterior transposition of the third part of the duodenum have been reported (56-62). The use of laparoscopic surgery (63) that involves lysis of the ligament of Treitz and mobilization of the duodenum has been reported as the duodenal-jejunal junction has been fully mobilized, the jejunum is passed behind the SMA and positioned to the right of the SMA so it does not lie in the acute angle between the aorta and the SMA; however, laparoscopy is being used investigationally.
Natural course
Delay in the diagnosis of acute or chronic SMA syndrome can result in malnutrition, dehydration, electrolyte disorders, and death. The acute form if untreated can result in dehydration and even intestinal perforation.
Prognosis
With proper conservative treatment in acute cases, and appropriate medical or surgical management in more chronic cases, the success rate in treating SMA syndrome is high. It is imperative to discover the underlying cause of the condition and treat that as well.
Conclusions
SMA syndromeis a rare benign disease,which responds positively in both therapies medical and surgical if properly used.
References
1. Rokitansky, C. - Handbuch der Pathologischen Anotomie. Vienna: Branmiller and Siedel 1842.
2. Baltazar, U., Dunn, J., Floresguerra, C. - Superior mesenteric artery syndrome: an uncommon cause of intestinal obstruction. South Med. J., 2000, 93:606.
3. Welsch, T., Buchler, M., Kienle, P. - Recalling Superior Mesenteric Artery Syndrome Digestive Surgery, 2007, 24:149.
4. Wilson-Storey, D., MacKinlay, G.A. - The superior mesenteric artery syndrome. J. Roy Coll. Surg. Edin., 1986, 31:175.
5. Wilkie, PD. - Chronic duodenal ileus. Am J Med Sci, 1927, 173:643.
6. Plesa, A., Constantinescu, C., Crumpei, F., Cotea, E. - Superior Mesenteric Artery Syndrome: An unusual cause of intestinal obstruction. J. Gastrointest. Liver. Dis., 2006, 15:69.
7. Niaz, Z., Chaudhary, F., Gardazi, J. - Superior mesenteric artery syndrome: An uncommon cause of upper intestinal obstruction. JCPSP, 2006, 16:666.
8. Raissi, B., Taylor, B., Traves, D. - Recurrent superior mesenteric artery (Wilkie's) syndrome: a case report. Can. J. Surg., 1996, 39:410.
9. Ylinen, P., Kinnunen, J., Hockerstedt, K. - Superior mesenteric artery syndrome. J. Clin. Gastroenterol., 1989, 11:386.
10. Wilkie, PD. - Chronic duodenal ileus. Am. J. Med. Sci., 1927, 173:643.
11. Cohen, L.B., Field, S.P., Sachar, D.B. - The superior mesenteric artery syndrome: the disease that isn't or is it? J. Clin. Gastroenterol., 1985, 7:113.
12. Barnes, J.B., Lee, M. - Superior mesenteric artery syndrome in an intravenous drug abuser after rapid weight loss. South Med. J., 1996, 89:331.
13. Pentlow, B.D., Dent, R.G. - Acute vascular compression of the duodenum in anorexia nervosa. Br. J. Surg., 1981, 68:665.
14. Milner, E.A., Cioffi, W.G., McManus, W.F., Pruitt, B.A. Jr. - Superior mesenteric artery syndrome in a burn patient. Nutr. Clin. Pract., 1993, 8:264.
15. Roy, A., Gisel, J.J., Roy, V., Bouras, E.P. - Superior mesenteric artery (Wilkie's) syndrome as a result of cardiac cachexia. Gen Intern Med., 2005, 20:958.
16. Barnes, J., Lee, M. - Superior mesenteric artery syndrome in an intravenous drug abuser after rapid weight loss. South Med. J., 1996, 89:331.
17. Mehler, P.S., Weiner, K.L. - Use of total parenteral nutrition in the refeeding of selected patients with severe anorexia nervosa. Int. J. Eat. Disord., 2007, 40:285.
18. Altiok, H., Lubicky, J.P., DeWald, C.J., Herman, J.E. - The superior mesenteric artery syndrome in patients with spinal deformity. Spine, 2005, 30:2164.
19. Crowther, A., Webb, P.J., Eyre-Brook, I.A. - Superior mesenteric artery syndrome following surgery for scoliosis. Spine, 2002, 27:528.
20. Hutchinson, D.T., Bassett, G.S. - Superior mesenteric artery syndrome in pediatric orthopedic patients. Clin. Orthop., 1990, 250:250.
21. Munns, S.W., Morrissy, R.T., Golladay, E.S. - Hyperalimentation for superior mesenteric-artery (cast) syndrome following correction of spinal deformity. J. Bone Joint. Surg. Am., 1984, 66:1175.
22. Shah, M.A., Albright, M.B., Vogt, M.T., Moreland, M.S. - Superior mesenteric artery syndrome in scoliosis surgery: weight percentile for height as an indicator of risk. J. Pediatr. Orthop., 2003, 23:665.
23. Shapiro, G., Green, D.W., Fatica, N.S., Boachie-Adjei, O. - Medical complications in scoliosis surgery. Curr. Opin. Pediatr., 2001, 13:36.
24. Vitale, M.G., Higgs, G.B., Liebling, M.S., Roth, N., Roye, D.P. Jr. - Superior mesenteric artery syndrome after segmental instrumentation: a biomechanical analysis. Am. J. Orthop., 1999, 28:461.
25. Zhu, Z.Z., Qiu, Y. - Superior mesenteric artery syndrome following scoliosis surgery: It's risk indicators and treatment strategy. World Journal of Gastroenterol., 2005, 11:3307.
26. Hutchinson, D.T., Bassett, C.S. - Superior mesenteric artery syndrome in pediatric orthopedic patients. Clin. Orthop., 1990, 250:250.
27. Munns, S.W., Morrissy, R.T., Golladay, S., McKenzie, C.N. - Hyperalimentation for superior mesenteric-artery (cast) syndrome following correction of spinal deformity. J Bone Joint Surg Am., 1984, 66(8): pag.1175-1177.
28. Smith, S.J., Cooney, R.N. - Superior mesenteric artery syndrome in a tube-fed patient. Nutr. Clin. Pract., 1994, 9:151.
29. Laffont, I., Bensmail, D., Rech, C., Prigent, G., Loubert, G., Dizien, O. - Late superior mesenteric artery syndrome in paraplegia: case report and review. Spinal Cord., 2002, 40:88.
30. Ortiz, C., Cleveland, R.H., Blickman, J.G. - Familial superior mesenteric artery syndrome. Pediatr. Radiol, 1990, 20:588.
31. Raissi, B., Taylor, B.M., Taves, D.H. - Recurrent superior mesenteric artery (Wilkie's) syndrome: a case report. Can. J. Surg., 1996, 39:410.
32. Ortiz, C., Cleveland, R., Blickman, J., Jaramillo, D., Kim, S. - Familial superior mesenteric artery syndrome. Pediatric Radiology, 1990, 20:588.
33. Sianesi, M., Soliani, P., Arcuri, M.F., Bezer, L., Iapichino, G., Del Rio, P. - Dunbar's syndrome and superior mesenteric artery syndrome: a rare association. Dig. Dis. Sci., 2007, 52:302.
34. Pentlow, B.D., Dent, R.G. - Acute vascular compression of the duodenum in anorexia nervosa. Br. J. Surg., 1981, 68:665.
35. Altman, D.H., Puranik, S.R. - Superior mesenteric artery syndrome in children. Am. J. Roentgenol Radium Ther Nucl Med., 1973, 118:104.
36. Shetty, A.K., Schmidt-Sommerfeld, E., Haymon, M.L. - Radiological case of the month. Superior mesenteric artery syndrome. Arch. Pediatr. Adolesc. Med., 1999, 153:303.
37. Veysi, V., Humphrey,G., Stringer, M. - Superior Mesenteric Artery Syndrome Presenting With Acute Massive Gastric Dilatation. Journal of Pediatric Surgery, 1997, 32:1801.
38. Gamblin, T.C., Stephens, R.E., Johnson, R.K., Rothwell, M. - Adult malrotation: a case report and review of the literature. Curr. Surg., 2003, 60:517.
39. Samdani, P., Samdani, V., Goel, A. - Superior Mesenteric Artery Compression Syndrome. Indian J. Pediatr., 2006, 73:523.
40. Lundell, L., Thulin, A. - Wilkie's syndrome - a rarity? Br. J. Surg., 1980, 67:604.
41. Mackowiak, P., Arentz, M., Kirkpatrick, B., Casey, A. - Vomiting and rapid weight loss in a man with newly diagnosed HIV infection. Clinical Infections diseases, 2005, 41:1196.
42. Fromm, S., Cash, J. - Superior mesenteric artery syndrome: an approach to the diagnosis and management of upper gastrointestinal obstruction of unclear etiology. S D J Med., 1990, 43:5.
43. Lippl, F., Hannig, C., Weiss, W., Allescher, H.D., Classen, M., Kurjak, M. - Superior mesenteric artery syndrome: diagnosis and treatment from the gastroenterologist's view. J. Gastroenterol., 2002, 37:640.
44. Payawal, J., Cohen, A., Stamos, M. - Superior mesenteric artery syndrome involving the duodenum and jejunum Emergency Radiology, 2004, 10:273.
45. Gockel, I., Graupe, F., Hain, J., Lorenz, D. - Superior mesenteric artery (Wilkie) syndrome: different faces. J. Gastroenterol. Hepatol., 2004, 19:720.
46. Khoo, L.A., Belli, A.M. - Superior mesenteric artery stenting for mesenteric ischaemia in Sneddon's syndrome. British Journal of Radiology, 1999, 72:607.
47. Wiesner, W., Khurana, B., Ji, H., Ros, P. - CT of Acute Bowel Ischemia. Radiology, 2003, :635.
48. Flynn, J., Latif, O., Stewart, R., Feldman, L. -Superior Mesenteric Artery Syndrome. Clinical Images in Medicine, 2006, 6:44.
49. Geer, D. - Superior mesenteric artery syndrome. Mil. Med., 1990, 155:321.
50. Santer, R., Young, C., Rossi, T., Riddlesberger, M.M. - Computed tomography in superior mesenteric artery syndrome. Pediatr. Radiol., 1991, 21:154.
51. Neri, S., Signorelli, S.S., Mondati, E., Pulvirenti, D., Campanile, E., Di Pino, L., Scuderi, M., Giustolisi, N., Di Prima, P., Mauceri, B., Abate, G., Cilio, D., Misseri, M., Scuderi, R. - Ultrasound imaging in diagnosis of superior mesenteric artery syndrome. J. Intern Med., 2005, 257:346.
52. Ylinen, P., Kinnunen, J., Hockerstedt, K. - Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J. Clin. Gastroenterol., 1989, 11:386.
53. John, A.S., Tuerff, S.D., Kerstein, M.D. - Nonocclusive mesenteric infarction in hemodialysis patients. J. Am. Coll. Surg., 2000, 190:84.
54. Orihashi, K., Matsuura, Y., Sueda, T., Shikata, H., Morita, S., Hirai, S., Sueshiro, M., Okada, K. - Abdominal aorta and visceral arteries visualized by trans-gastric echocardiography: technical considerations.Hiroshima J. Med. Sci., 1997, 46:151.
55. Coletti, G., Torracca, L., La Canna, G., Maisano, F., Sebastiano, P., Fucci, C., Berra, P., Alfieri, O. - Diagnosis and management of cerebral malperfusion phenomena during aortic dissection repair by transesophageal Doppler echocardiographic monitoring. J. Card. Surg., 1996, 11:355.
56. Eugster, T., Obeid, T., Gurke, L., Wolff, T., Stierli, P. - Acute supramesenteric thrombosis of an abdominal aortic aneurysm with deleterious embolism: a case report. Ann. Vasc. Surg., 2005, 19:411.
57. Goldberg, M., Kim, H. - Treatment of Acute Superior Mesenteric Vein Thrombosis with Percutaneous Techniques. AJR, 2003, 181:1305.
58. Arbell, D., Gross, E., Koplewitz, B., Vromen, A., Barr-Jin, J., Udassin, R. - Superior Mesenteric Artery Syndrome masquerading as recurrent billiary pancreatitis. IMAJ, 2006, 8:441.
59. Rappaport, W.D., Hunter, G.C., McIntye, K.E., Ballard, J.L., Malone, J.M., Putnam, C.W. - Gastric outlet obstruction caused by traumatic pseudoaneurysm of superior mesenteric artery. Surgery, 1990, 108:930.
60. Strong, E.K. - Mechanics of arteriomesenteric duodenal obstruction and direct surgical attack upon etiology. Ann. Surg., 1958, 148:725.
61. Ylinen, P., Kinnunen, J., Hockerstedt, K. - Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J. Clin. Gastroenterol., 1989, 11:386.
62. Schroeppel, T.J., Chilcote, W.S., Lara, M.D., Kothari, S.N. - Superior mesenteric artery syndrome after laparoscopic Roux-en-Y gastric bypass. Surgery, 2005, 137: 383.
63. Massoud, W.Z. - Laparoscopic management of superior mesenteric artery syndrome. Int. Surg., 1995, 80:322.