Superior mesenteric artery syndrome

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BACKGROUND

Superior mesenteric artery (SMA) syndrome (aortomesenteric compression, AMC) is a rare mechanical obstruction of the proximal small intestine [1]. It develops when the third part of the duodenum is compressed between the superior mesenteric artery and the abdominal aorta. The Austrian pathologist Carl von Rokitansky first documented this condition in 1861. Nearly seven decades later, in 1927, British surgeon David Wilkie provided a comprehensive clinical description, including the syndrome’s manifestations, potential etiological mechanisms, and therapeutic approaches [2]. The eponymous term «Wilkie syndrome» is widely used in the international literature to refer to aortomesenteric compression (AMC) [3]. The incidence of aortomesenteric compression (AMC) ranges from 0.013% to 0.78%, with young and adolescent women being most commonly affected. By 2023, 730 articles have been published in the PubMed database, reporting 2,400 cases of this syndrome management. The etiological and diagnostic aspects of the condition have been thoroughly described, and indications for its surgical correction have been formulated [4]. However, the choice of the optimal surgical technique remains unclear. Clinical practice features diverse approaches to AMC management, yet lacks clear criteria for selecting the optimal method in individual cases. Surgery for Wilkie syndrome serves as part of a multimodal treatment plan to restore normal duodenal food transit. Its success directly influences digestive function and the patient’s rehabilitation timeline. We report a case of successful AMC management in a young patient, with equal contributions from the nutritionist, physiotherapist, intensivist, and surgical teams proving critical to the positive outcome.

CASE PRESENTATION

Patient Profile

Patient R., 18 years of age, was admitted to the National Medical Research Center of Surgery with complaints of nausea, vomiting of ingested food, nasogastric drainage of 2.0–2.5 L/day, weakness, and weight loss of 20 kg over the past 6 months.

In 2021, the patient initially presented to a local gastroenterologist with complaints of abdominal pain occurring after food intake. Comprehensive evaluation failed to reveal any organic pathology affecting the gastrointestinal tract. Anthropometric measurements at that time showed a body weight of 86 kg and a height of 192 cm, corresponding to a normal BMI of 23 kg/m². Despite the absence of structural disease, the patient’s symptoms persisted. A vicious cycle developed: anxiety related to the anticipation of postprandial pain led to progressive loss of appetite. This, in turn, resulted in an almost complete self-imposed dietary restriction and the development of progressive asthenia.

The patient’s condition deteriorated progressively: despite significant emaciation, dyspeptic symptoms intensified. In January 2025, he was admitted to a local hospital for further evaluation. Multidetector computed tomography (MDCT) with dual contrast administration was performed to assess the gastrointestinal anatomy. The scan demonstrated critical narrowing of the aortomesenteric space: the distance between the aorta and superior mesenteric artery at the LII vertebral level was 4.11 mm; the aortomesenteric angle was 7.7°. These radiological findings confirmed the diagnosis of aortomesenteric compression syndrome (AMC). Conservative therapy proved ineffective, necessitating surgical intervention. The patient underwent division of the ligament of Treitz. However, the early postoperative period was marked by complications, including recurrent vomiting and return of abdominal pain, indicating incomplete resolution of the underlying pathophysiology (Fig. 1).

 

Fig. 1. MSCT with double contrast: (a) the distance from the aorta to the superior mesenteric artery; (b) compression of the duodenum between the aorta and the superior mesenteric aorta.

 

On postoperative day 6, the patient’s condition showed no improvement with conservative measures. Consequently, a second surgical intervention was performed, involving the creation of a gastroenteroanastomosis (GEA) on a Braun loop to bypass the obstructed segment. Despite the procedure, postoperative recovery was complicated by persistent gastrointestinal dysfunction: gastostasis, nausea, episodes of intermittent vomiting. Fluoroscopic examination confirmed delayed gastric emptying, with impaired transit through the newly formed gastroenteroanastomosis. Given the persistence of gastro- and duodenostasis and the lack of therapeutic response to the performed surgical interventions, a telemedical consultation was convened. The multidisciplinary team concluded that the patient required advanced care and recommended transfer to the National Medical Research Center of Surgery for further management. On admission to the National Medical Research Center of Surgery, the patient presented in a state of undernutrition. Anthropometric assessment revealed a body mass index of 13 kg/m², indicating severe underweight (Fig. 2).

 

Fig. 2. Photo of the patient at admission: (a) front view; (b) side view.

 

Enteral nutrition was administered via a nasojejunal feeding tube. Simultaneously, gastric decompression was maintained using a separate nasogastric tube to relieve gastric distension.

Results of Clinical Examination

Laboratory findings revealed anemia (hemoglobin 121.1 g/L, hematocrit 32.8%, erythrocytes 3.86 × 10¹²/L), impaired water-electrolyte balance (potassium 3.1 mmol/L, sodium 130 mmol/L, chloride 96 mmol/L), hypoproteinemia (total protein 55.9 g/L, albumin 28.8 g/L), elevated liver enzymes (AST 97.5 U/L, ALT 75.0 U/L), and hypoglycemia (glucose 3.93 mmol/L).

Esophagogastroduodenoscopy (EGD) findings: the stomach was enlarged and overdistended, easily dilating upon insufflation; a large amount of stagnant liquid contents was present in the lumen. The mucosal folds were prominent and tortuous. Peristalsis was weakened but traceable along all walls up to the pylorus.

The accessible portion of the gastric and duodenal mucosa exhibited diffuse edema and hyperemia, suggesting active mucosal inflammation. The pyloric sphincter appeared structurally intact, without deformity or stenosis. It opened freely to a diameter of 16–18 mm during the examination, indicating preserved sphincteric function. The duodenal bulb maintained its normal oval shape, without evidence of ulceration or scarring. In the vertical (descending) segment of the duodenum: the lumen remained anatomically patent, without structural deformity. A substantial amount of mixed mucus and bile was present within the lumen, likely reflecting biliary reflux. Peristaltic activity was preserved and actively traceable along the full circumference of the wall. The mucosa had a pink, velvety texture, consistent with a non-atrophic, although inflamed, mucosa. However, progression of the endoscope was halted at the inferior horizontal (third) portion of the duodenum. Here, the lumen was significantly narrowed, preventing further advancement of the instrument. This finding suggests a possible stricture, extrinsic compression, or luminal obstruction at this level.

Barium radiography findings: the stomach was enlarged; the gastric sinus was located below the level of the iliac crests. Evacuation of the barium suspension through the gastroenteroanastomosis (GEA) began after 30 minutes, while passage through the duodenum was not traceable. After 1 hour, all contrast remained in the stomach with smudges in the loops of the GEA (Fig. 3a); most of the contrast was evacuated from the stomach through the GEA only after 12 hours (Fig. 3b).

 

Fig. 3. X-ray examination with water-soluble contrast: (a) gastro-stasis; (b) partial evacuation through the GEA after 12 hours.

 

Clinical management

Two weeks following hospital admission, the patient underwent a reconstructive laparotomy.

The procedure included disconnection of the interintestinal anastomosis and gastroenteroanastomosis (GEA), division of the duodenojejunal flexure, and transposition of the inferior horizontal part of the duodenum anterior to the superior mesenteric vessels. A terminal duodenojejunoanastomosis was then formed — a procedure known as Robinson’s operation (Fig. 4).

 

Fig. 4. Robinson’s scheme of surgery: (a) separation of the gastroenteroanastomosis and the intercellular anastomosis; (b) the jejunum is placed in an orthotopic position, the duodenal bend is crossed to the left of the BBA trunk; (c) a duodenoid anastomosis is formed anteriorly from the BBA in the window of the mesocolones.

 

During the laparotomy, the following anatomical findings were confirmed: the stomach was significantly enlarged, with an estimated volume of up to 3 liters, indicating chronic gastric distension. A pre-existing retrocolic gastroenteroanastomosis (GEA) was identified on the anterior gastric wall, positioned close to the greater curvature. Approximately 35 cm distal to the GEA, a wide Brown’s anastomosis (interintestinal bypass) was observed. The surgical team then proceeded with the following reconstructive steps: disconnection of pre-existing anastomoses. Both the gastroenteroanastomosis and the enteroenteroanastomosis were carefully divided. The duodenum was found to be markedly dilated, with a diameter of up to 6.0 cm. It was fully mobilized along its entire course, including the vertical and inferior horizontal portions.

The horizontal (third) portion of the duodenum was visibly compressed by the trunk of the superior mesenteric artery (SMA), confirming the diagnosis of SMA syndrome. Distal to the site of compression, the intestinal lumen was markedly collapsed, with an internal diameter of up to 1.5 cm, indicating chronic obstruction and stasis. The surgical team carried out a transection of the duodenum during the procedure.

The duodenum was carefully divided at the level of the duodenojejunal junction. Special attention was paid to preserving the first pair of jejunal vessels to ensure adequate blood supply to the distal bowel segment. The proximal jejunum was mobilized and repositioned anteriorly, to the left side of the SMA trunk. Subsequently, a hand-sewn, two-layer end-to-end duodenojejunoanastomosis was formed within the window of the mesocolon using interrupted sutures (Fig. 5).

 

Fig. 5. Intraoperative photo: (a) the stage of formation of the duodenoid anastomosis; (b) the completed reconstruction.

 

Dynamics and outcomes

Persistent gastroparesis was observed in the postoperative period. Over a two-week period, comprehensive therapy was administered to restore gastric motor activity, including proton pump inhibitors, prokinetics (intravenous erythromycin), antispasmodics, physiotherapy, and therapeutic exercise. Physiotherapy included iontophoresis with neostigmine (proserin) applied to the area of the greater curvature of the stomach (4 procedures), which yielded no clinical effect. Subsequently, 12 sessions of sinusoidal modulated current (SMT) therapy were administered, with each session lasting 20 minutes. The treatment was performed using the Amplipulse device. Constant modulation (Mode I, or I RR) at a frequency of 50 Hz was applied, followed by a combination of carrier current pulses and pauses (Mode II, or II RR) up to 100 Hz. The series of impulses exerted an excitatory effect on the nerve fibres, inducing rhythmic contractions of smooth muscle and enhancing both arterial circulation and venous outflow. Concurrently, therapeutic physical exercises (TPE) were performed according to a peristalsis-stimulating regimen, which also included general strengthening exercises tailored to the patient’s asthenia. Sessions were conducted 1–3 times daily, each lasting 15 minutes. Treatment efficacy was assessed using subjective criteria (nausea, frequency of vomiting, frequency and volume of food intake) and by monitoring the dynamics of X-ray findings. The patient received enteral tube feeding and supportive parenteral nutrition.

Two weeks after surgery, mild anemia persisted (hemoglobin 125,3 g/L, hematocrit 36,8%, erythrocytes 3,75×10¹²/L). The water-electrolyte balance was within normal limits (potassium 4 mmol/L, sodium 140 mmol/L, chloride 103 mmol/L). Blood protein fractions and liver enzyme levels remained within reference ranges (total protein 67 g/L, albumin 36,2 g/L, AST 14,3 U/L, ALT 25,7 U/L).

On follow-up contrast radiography, timely portioned evacuation of the barium suspension into the duodenum (D1) and through the duodenojejunal anastomosis into the downstream segments of the small intestine was observed (Fig. 6).

 

Fig. 6. Radiographs of the patient at discharge: (a) contrast agent in the stomach and lower branch of the duodenum after 10 minutes; (b) contrast agent in the lower horizontal branch of the duodenum and jejunum after 30 minutes; (c) contrast agent in the colon after 60 minutes.

 

The patient was allowed fractional oral nutrition and discharged from the clinic in a satisfactory condition on postoperative day 14 (POD 14).

Six months after discharge, the patient was examined at the local hospital. He reported no complaints and was in a satisfactory condition. No long-term complications were detected. The patient follows a regular diet (45 meals per day) — and has gained 7 kg (BMI: 14,9 kg/m²). He attends school, spends his free time with peers, and enjoys fishing.

DISCUSSION

The etiological factors of aortomesenteric compression syndrome (AMC) development are divided into congenital and acquired.

Congenital factors include a short or hypertrophied Treitz ligament, anomalies in the branching pattern of the aortic vessels, and spinal deformities (such as scoliosis, Marfan syndrome, et.). Acquired causes encompass diseases associated with significant weight loss and leading to depletion of the adipose tissue between the superior mesenteric artery (SMA) and the aorta.

Progressive weight loss may be caused by trauma, burns, prolonged bed rest, or weight-loss diets [5–7]. In 30% оf patients, predisposing factors for aortomesenteric compression syndrome development include eating disorders, use of illicit drugs, and anorexia nervosa [8].

The clinical manifestations of aortomesenteric compression syndrome are nonspecific and include a symptom complex of dyspeptic disorders: postprandial pain (59%), nausea (40%), vomiting (50%), early satiety (32%), asthenia, and cachexia (32%) [9]. A characteristic feature of aortomesenteric compression syndrome is the worsening of clinical symptoms in the upright position and their improvement when the patient is lying on the side, as the tension on the superior mesenteric artery decreases [10]. Among neurological symptoms, irritability, insomnia, and depressive episodes may occur.

Frequent vomiting after meals forces patients to avoid eating, which subsequently leads to progression of aortomesenteric compression syndrome [11].

In males, Wilkie’s syndrome is sometimes associated with varicocele. Compression of the left renal vein in the aortomesenteric interval leads to increased pressure, causing dysfunction of the valves in the internal spermatic vein and the formation of compensatory bypass renocaval blood flow. As a result, venous blood flows through the spermatic vein into the pampiniform plexus, leading to varicocele [12].

The diagnostic standard for aortomesenteric compression is multislice computed tomography (MSCT), which measures: the angle between the aorta and the superior mesenteric artery (SMA) — normal range: 28°–65°; the distance between the aorta and SMA at the LII–LIII level — normal range: 10–34 mm [2]. A diagnosis of compression of the inferior horizontal duodenal segment is valid when the angle is less than 25° and the distance between the aorta and SMA is 10 mm or less [13].

Conservative therapy for aortomesenteric compression is symptomatic and effective in most cases. It aims to identify and address the underlying cause of aortomesenteric compression. Surgical treatment is indicated for progressive forms of aortomesenteric compression with impaired duodenal patency, cholangitis, perforation, and duodenal wall necrosis [14]. According to S. Wan et al. (2020), surgical intervention for aortomesenteric compression is objectively indicated in only 11.5% of cases [15].

The surgical arsenal for correcting aortomesenteric compression includes:

Strong’s operation (division of the ligament of Treitz with mobilization of the horizontal part of the duodenum and its repositioning into the intraperitoneal space), various bypass anastomoses involving the stomach and duodenum on a Brown loop or Roux-en-Y loop, Gregory-Smirnov duodenojejunostomy, bilateral exclusion of the duodenum from the food passage, placement of nitinol stents at the level of compression, infrarenal transposition of the superior mesenteric artery, Robinson’s operation, which involves transposition of the duodenum with formation of a duodenojejunal anastomosis anterior to the superior mesenteric artery [16–18].

The advantages of Strong’s operation lie in preserving gastrointestinal continuity and avoiding anastomoses, thus eliminating the risk of anastomotic leakage. However, its efficacy is 75% [3]. Various bypass gastroenteroanastomosis (GEA) techniques also demonstrate low efficacy. Moreover, exclusion of the duodenum from the food passage leads to dumping syndrome, afferent loop syndrome, and peptic ulcers at the anastomotic sites [19; 20]. Any bypass duodenojejunostomy preserves the physiological passage of food, but due to the antiperistaltic orientation of the loop segment, it leads to the development of the so-called vicious circle syndrome. This condition impairs portioned evacuation and is accompanied by retrograde reflux of contents into the duodenum and stomach [21].

The physiological mechanism is as follows. The pyloric sphincter (PS) opens in response to increased pressure in the antral part of the stomach and is purely reflex in nature [22]. In this process, the circular muscle fibers relax, while the longitudinal fibers contract and stretch the base of the duodenal bulb, much like the suspension lines of a parachute.

The peristaltic wave generates a threshold pressure within the intestinal lumen, and as it fills, the pyloric sphincter contracts again. This mechanism is responsible for the portioned evacuation of chyme from the stomach into the duodenum. Subsequently, hydrochloric acid exerts an irritative effect on the duodenal mucosa, triggering contraction of the sphincters of Ochsner and Kapandji and release of secretin and cholecystokinin by the duodenal epithelium. These hormones regulate the volume and rate of bile and pancreatic juice secretion [23]. Contraction of the sphincters ensures pendular movements of the duodenal contents and mixing of chyme with digestive juices. When the pH of the contents rises to a certain level, the sphincter of Ochsner opens, allowing food to pass into the jejunum. In aortomesenteric compression, retrograde reflux from the duodenum into the stomach occurs in response to gastric reflex contractions. The stomach enlarges in size and loses its tone and contractility. Over time, reflex impulses in the pyloric sphincter weaken, and the motor function of the entire digestive system becomes impaired. Gastrointestinal motility disorders are accompanied by complex psychosomatic disorders in 67,3% of cases [24].

There are a few case reports of superior mesenteric artery transposition in aortomesenteric compression with a positive clinical outcome [16, 25]. However, this surgery is feasible only in specialized vascular centers equipped with X-ray endovascular diagnostic and treatment techniques, which limits its application in general clinical practice [16].

The global experience with anterior transposition of the horizontal part of the duodenum (Robinson’s operation) is also limited due to the technical complexity and labor-intensiveness of the procedure [26]. In our opinion, Robinson’s operation is a radical and relatively safe method for correcting aortomesenteric compression, allowing relief of duodenal compression and preservation of physiological food passage.

In the presented clinical case, an initial strategy of minimal invasiveness was adopted. Instead of the classic Strong’s operation, the decision was made to limit the intervention to a simple division of the ligament of Treitz. However, this did not fully relieve the compression of the horizontal part of the duodenum, and the created gastroenteroanastomosis did not result in clinical improvement under these conditions. Given that this was already the third surgical intervention, we opted for the most radical yet physiological method — Robinson’s operation (resection of the duodenojejunal junction with anterior transposition of the duodenum in front of the superior mesenteric artery, which achieved a satisfactory clinical outcome.

CONCLUSION

Clinical manifestations of aortomesenteric compression are nonspecific, and symptoms are variable. Timely diagnosis enables early prevention of severe digestive disorders and helps avoid surgical treatment. In progressive aortomesenteric compression, Robinson’s operation remains the most physiological surgical option. Given the rarity of the disease, treatment is advisable at specialized clinics. A multidisciplinary team approach to managing this syndrome enables achieving a positive outcome with minimal consequences for the patient and ensures prompt social and occupational rehabilitation.

ADDITIONAL INFORMATION

Author contributions: V.A. Kozlov, data collection and analysis, manuscript writing; D.V. Ruchkin, surgical procedure performance, manuscript editing, overall supervision, and approval of the final manuscript; Sh.Kh. Karimova, K.P. Lukyanchenko, surgical procedure performance, manuscript writing, literature review and analysis; E.A. Glebova, N.B. Kovalerova, manuscript editing, literature review and analysis. Thereby, all authors provided approval of the version to be published and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Consent for publication: Written informed consent was obtained from the patient for the publication of information regarding their treatment, including photographs and study data (dated July 25, 2025). The amount of published data is agreed with the patient.

Funding sources: The study had no sponsorship.

Disclosure of interests: The authors declare that they have no competing interests.

Statement of originality: The authors did not use previously published information (text, illustrations, data) while conducting this work.

Data availability statement: The editorial policy regarding data sharing is not applicable to this work, the data may be published in the public domain.

Generative AI: Generative AI technologies was not used for the preparation of this manuscript.

About the authors

Dmitry V. Ruchkin

A.V. Vishnevsky National Medical Research Center of Surgery

Email: ruchkindmitry@gmail.com
ORCID iD: 0000-0001-9068-3922
SPIN-code: 2587-8568

MD, PhD

Russian Federation, Moscow

Valentin A. Kozlov

A.V. Vishnevsky National Medical Research Center of Surgery

Author for correspondence.
Email: kozipan@mail.ru
ORCID iD: 0000-0002-4926-116X
SPIN-code: 5917-6510

MD, PhD

Russian Federation, Moscow

Shakhlo Kh. Karimova

A.V. Vishnevsky National Medical Research Center of Surgery

Email: sh.karim79@mail.ru
ORCID iD: 0009-0000-4028-6785
SPIN-code: 7624-5393
Russian Federation, Moscow

Elena A. Glebova

A.V. Vishnevsky National Medical Research Center of Surgery

Email: glebova@ixv.ru
Russian Federation, Moscow

Natalia B. Kovalerova

A.V. Vishnevsky National Medical Research Center of Surgery

Email: kovalerova.nat@gmail.ru
ORCID iD: 0000-0002-6951-1816
SPIN-code: 2525-9338
Russian Federation, Moscow

Kirill P. Lukyanchenko

A.V. Vishnevsky National Medical Research Center of Surgery

Email: lukyanchenko94@list.ru
ORCID iD: 0009-0006-5996-7170
Russian Federation, Moscow

References

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