Misdiagnosed superior mesenteric artery syndrome due to diabetic gastroparesis. Case report and literature review

Abstract

Introduction

Superior mesenteric artery syndrome (SMAS) is a rare cause of upper intestinal obstruction. This occurs due to duodenal compression between the superior mesenteric artery and the aorta. Anatomical alterations, eating disorders, after some surgical procedures, and trauma are frequent causes of this rare syndrome. Diabetes is a highly prevalent disease that can cause gastroparesis in up to 12 %. Its association with SMAS is extremely rare and challenging to identify.

Case presentation

A 32-year-old man experienced nausea and vomiting after diagnosis and treatment for type II diabetes. He was treated for diabetic gastroparesis for 2 years without improvement until he lost 40 kg of weight. After imaging studies, a distance between the superior mesenteric artery and the aorta of 5.3 mm and an angle of 17 degrees were detected, corroborating the diagnosis of SMAS syndrome. Due to medical failure, surgical treatment via duodenojejunostomy was performed.

Discussion

Diabetes is a very prevalent disease in the world population that can cause gastrointestinal symptoms. In our patient, diabetic gastroparesis delayed the diagnosis of SMAS until severe symptoms of upper intestinal obstruction and significant weight loss occurred. In our patient, due to medical failure, surgical treatment significantly improved his symptoms and stopped his weight loss.

Conclusion

Superior mesenteric artery syndrome is a rare syndrome, and challenging to differentiate from diabetic gastroparesis. Delays in management may result in excessive weight loss. Surgical treatment can improve symptoms and weight loss.

Highlights

• •Superior mesenteric artery syndrome (SMAS) is a rare cause of upper intestinal obstruction.
• •Diabetes is a highly prevalent chronic disease, and its association with SMAS is exceptionally rare.
• •Diabetic gastroparesis makes the diagnosis of intestinal syndromes such as SMAS challenging.
• •Delaying the diagnosis of SMAS can lead to significant weight loss, and surgical treatment could be a good treatment option.
• •We present a rare case of SMAS in a type II diabetic patient misdiagnosed with gastroparesis with significant weight loss.

Abbreviations

CT

Computed tomography

DM

Diabetes mellitus

MRI

Magnetic resonance Imaging

POD

postoperative day

SMA

Superior mesenteric artery

SMAS

Superior mesenteric artery syndrome

WS

Wilkie syndrome

1. Introduction

Superior mesenteric artery syndrome (SMAS) or Wilkie syndrome (WS) is a rare cause of upper intestinal obstruction. This obstruction occurs due to compression of the third portion of the duodenum between the superior mesenteric artery and the aorta. Its frequency and incidence are rare, and only a few isolated cases and case series have been reported. Different causes have been identified that predispose SMAS. Among the most common causes are anatomical alterations after some surgical procedures, serious burns, eating disorders, and trauma, among others [[1], [2], [3]]. Its appearance in chronic degenerative diseases is extremely low. Diabetes is a highly prevalent disease, but its association with superior mesenteric artery syndrome, it has rarely been identified. In diabetic patients, the presence of diabetic gastroparesis and gastrointestinal dysmotility make the identification of intestinal syndromes such as superior mesenteric artery syndrome challenging [4].

Due to its extreme rarity and the difficulty in diagnosing this pathology, we reviewed the updated literature and presented a case of superior mesenteric artery syndrome misdiagnosed in a type II diabetes patient with gastroparesis. We present this case according to the updated 2023 Surgical CAse REports SCARE guidelines, as well as under written authorization from the patient [5,6].

2. Presentation of case

A previously healthy 32-year-old man began treatment based on diet and oral hypoglycemic agents with metformin 500 mg every 12 h due to a diagnosis of type II diabetes. He subsequently presented a decrease in appetite and gradual weight loss, which was attributed to diet, and medical treatment. Due to this and poor glycemic control, his treatment was changed and his management continued until he needed an insulin glargine regimen. One year after the patient was diagnosed and treated for diabetes he lost one-quarter of his original weight, and his condition began with early satiety, nausea, vomiting, oral intolerance, and gastroesophageal reflux. He underwent a medical evaluation and started medical management with prokinetics agents for diabetic gastroparesis without improvement. Subsequently, his condition worsened, and he presented clear signs of upper intestinal obstruction, which was the reason for his going to the emergency room on several occasions. In total, he went to the emergency department four times without improving his symptoms. Panendoscopy was performed, revealing mild gastroesophageal reflux, stomach, and duodenal dilation, and Helicobacter pylori infection, and medical management continued. The patient was started on nutrition through a nasogastric tube without improvement. After two years of symptoms initiation, he showed a 50 % decrease in his body mass, losing 42 kg of total weight. With a diagnosis of upper intestinal obstruction and severe malnutrition, the patient was referred to the general surgery service.

Initially, a gastrointestinal series was performed, which showed gastric distension, significant duodenal distention, and decreased passage of contrast medium into the small intestine (Fig. 1). With suspicion of superior mesenteric artery syndrome (SMAS), contrast-enhanced abdominal tomography was performed, revealing a distance between the superior mesenteric artery and the aorta of 5.3 mm (normal 10 to 20 mm) (Fig. 2, Fig. 3). The protocol was completed with angioresonance, where an angle between the superior mesenteric artery and the aorta of 17 degrees was observed (normal 25 to 60 degrees), corroborating the diagnosis of SMAS. (Fig. 4).

Fig. 1.

Intestinal transit. Gastric and duodenal dilation was observed, as abrupt narrowing at the level of the third duodenal portion (green arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 2.

Abdominal tomography in the arterial phase, axial section. A distance of 5.35 mm in the third duodenal portion was demonstrated between the superior mesenteric artery and the aorta (normal 10 to 20 mm).

Fig. 3.

Abdominal tomography in the arterial phase, sagittal section. An angle of less than 17 degrees was demonstrated between the superior mesenteric artery and the aorta (normal 25 to 60 degrees).

Fig. 4.

Angioresonance, 3D reconstruction. An angle between the superior mesenteric artery and the aorta of 17 degrees was observed (normal 25 to 60 degrees).

A complete nutritional protocol was carried out, and parenteral nutrition was started without improvement. Given the persistence of his symptoms and clear signs of upper intestinal obstruction with nausea, vomiting and oral feeding intolerance, surgical treatment was chosen. The intraoperative findings included gastric and duodenal distention, a significant decrease in all peritoneal fat, aortomesenteric obstruction at the level of the third duodenal portion, and significant intestinal hypotrophy. A manual duodenojejunostomy in two layers with a retrocolic Roux-en-Y was performed. Minimal bleeding, a surgical duration of 3 h, and no complications were reported. After POD-3, the patient tolerated a liquid diet and subsequently continued with a soft diet. At the fifth month of follow-up, the patient reported no discomfort or surgical complications, showed weight gain, recovery of 50 % of the lost body mass and control of his diabetes with an insulin regimen as well as return to his work activities.

3. Discussion

SMAS is a rare cause of upper intestinal obstruction. This syndrome occurs due to compression of the third portion of the duodenum between the superior mesenteric artery and the aorta. This syndrome has been named in different ways, of which the most frequent names are superior mesenteric artery syndrome, chronic duodenal ileus, duodenal aorto-mesenteric compression, Wilkie syndrome or cast syndrome [3]. The first reported case of this disease was made by Carl von Rokitansky in 1842, and later, Wilkie in 1927 described the first case series of this disease, giving it the eponym that this syndrome bears [7,8]. Due to the rarity of this disease, its incidence and prevalence are unknown. Lee et al., in a review, reported an incidence between 0.0024 and 0.34 %, and Merrett et al., reported an approximate incidence between 0.013 and 0.3 % [9,10].

The identified mechanism that produces duodenal obstruction in this syndrome is the decrease in fat between the superior mesenteric artery and the aorta with subsequent duodenal constriction. Different causes and risk factors that trigger this obstruction have been described in the literature; these can be grouped into anatomical or congenital causes and acquired causes. The most frequent congenital causes identified are high insertion of the Trietz ligament, a low origin of the superior mesenteric artery, pronounced lumbar lordosis, and intestinal malrotation. Among the most common acquired causes are eating disorders such as anorexia nervosa, malabsorption syndrome, trauma (head trauma, polytrauma), burns, and certain surgeries (Nissen fundoplication, spinal instrumentation, colon surgery, bariatric surgery, aneurysm surgery). Akin et al., in a case series of 10 patients, reported 40 % of their cases without apparent cause [2,3,11].

Diabetes mellitus is a disease with a high prevalence worldwide. This disease leads to gastroparesis and gastrointestinal disorders such as intestinal dysmotility in up to 12 % of patients. These disorders can lead to misdiagnosis of intestinal syndromes, such as SMAS, which are difficult to diagnose. The association between diabetes and SMAS is exceptionally rare. In our review, we found only 5 cases reported in the worldwide literature. In these patients, the initial management of diabetes, oral hypoglycemic medications, dysmotility, and eating disorders predispose to a decrease in mesenteric fat between SMA and the aorta, although the pathophysiology is not very well studied [12]. In the case we report, in addition to diagnostic delay and difficult management due to diabetes, SMAS was associated with a significant weight loss of more than 50 % of body mass, which has not been reported previously [4,13].

Most symptoms of this syndrome are nonspecific and vague and include nausea, vomiting, anorexia, feelings of fullness, postprandial abdominal pain, and weight loss. All of these symptoms occur equally in diabetic gastroparesis and make the differential diagnosis of superior mesenteric artery syndrome challenging. Weight loss is a characteristic that occurs very frequently in SMAS. The average weight loss reported in various case series is approximately 10.7 kg. In our case, the weight loss was 42 kg, which was much greater than that previously reported [9,14].

Diagnostic confirmation of this rare syndrome is based on imaging studies. Initially, the gastrointestinal series is recommended, where gastric distention, duodenal distention, and narrowing in the third portion of the duodenum are evident, as well as decreased emptying of the contrast medium. Tomography and resonance angiography are useful for detecting and demonstrating narrowing at the level of the superior mesenteric artery. The accepted diagnostic imaging criteria are a decrease in the angle between the aorta and the SMA of less than 22 to 25 degrees (normal 25–60 degrees) and a distance of less than 8 mm (normal 10–20 mm) [15,16].

Once the diagnosis is confirmed, the initial treatment consists of adequate nutritional assessment, treatment of eating disorders and initiation of nutritional management based on enteral or parenteral nutrition. Conservative management in these cases consists of nutritional support through hyperalimentation to increase the mesenteric fat pad, therefore increasing the aorto-mesenteric angle and improving symptoms. In our case, due to clear signs of obstruction, we opted for surgical treatment. In the case of medical treatment failure or persistence of symptoms, surgical management is indicated. Conservative therapy has been reported in several case reports and case series; however, to date, it has not been standardized, and these reports lack long-term follow-up. Furthermore, treatment with parenteral nutrition has major disadvantages, such as long hospital stays and a low success rate ranging from 14 to 71 % [9,12,17].

Given the persistence of symptoms, especially in the presence of intestinal obstruction with nausea, vomiting or oral intolerance, surgical treatment is the best option. Different techniques have been described to correct this pathology. The most reported procedures are the duodenojejunostomy, the release or division of the angle of Treitz, the gastrojejunostomy, and even vascular transposition [9,18,19].

Mobilization of the duodenum by releasing the Treitz ligament was first described by Edward Strong in 1958. This open or laparoscopic procedure has the advantage of not opening the intestine and has been reported with regular acceptance, with a 75 % success rate. As a major disadvantage, persistence and recurrence of symptoms associated with the need for long-term duodenojejunostomy have been reported [9,20]. Gastrojejunostomy is a less commonly used procedure. This treatment reduces gastric distention but is very inefficient at releasing and decompressing duodenal distention and obstruction [9,18].

Since Wilkie's reports, the use of duodenojejunostomy has been reported to be an effective treatment. This procedure is the most effective treatment for resolving the symptoms associated with duodenal obstruction in the long term. In a study with a 7-year follow-up, duodenojejunostomy improved gastrointestinal symptoms and helped in weight recovery [9,21]. This procedure is reported to have a low rate of complications and a morbidity of less than 2 %. Recurrence of the symptoms of this syndrome after surgical treatment with duodenojejunostomy is rare. In our review, we found only one patient, reported by Railes et al., with recurrence of symptoms after duodenojejunostomy, which was resolved with Roux-en-Y anastomosis [[22], [23], [24]]. In our case, due to the clear persistence of symptoms and signs of recurrent obstruction, we performed duodenojejunostomy anastomosis in Roux-en-Y to ensure long-term reduction of all symptoms and weight recovery. In our case, we prefer open surgery because we have more experience in this operation. In our experience, in this challenging case, this treatment has shown good acceptance by the patient, as well as a decrease in symptoms and gradual recovery of lost weight.

4. Conclusion

Superior mesenteric artery syndrome is a rare syndrome, and challenging to differentiate from diabetic gastroparesis. Delays in management may result in excessive weight loss. Surgical treatment can improve symptoms and weight loss.

Financing

The authors declare that they do not have funding to carry out this article.

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Declaration of competing interest

The authors declare that they have no conflict of interest.