Wilkie‘s Syndrome with Successful Nonsurgical Conservative Treatment in a Young Man: A Case Report

Introduction

Nausea and vomiting are 2 of the most common symptoms in the emergency and outpatient departments of a general surgical ward. Their causes can vary widely and range from harmless events, such as general gastrointestinal infections, to severe illnesses, most commonly intestinal obstructions.

In most cases, clinical examination, complete blood count, or diagnostic imaging leads to proper diagnosis. However, challenging cases are often associated with rare causes of symptoms.

A rare diagnosis causing nausea and vomiting and that is often undetected at first is superior mesenteric artery (SMA) syndrome, also known as “Wilkie’s syndrome”. The source of this syndrome is an unusual angle of the SMA. This results in the compression of the structures crossing between the abdominal aorta and the SMA, which is the third portion of the duodenum and the left renal vain. Symptoms such as postprandial stomachache and vomiting can appear, and male patients also can exhibit varicoceles as a result of the compression [1–4].

Wilkie’s syndrome is defined as compression of the duodenum between the SMA and the abdominal aorta, due to a smaller angle between these 2 vessels (<22°), leading to the symptoms of a high gastrointestinal obstruction.

The origin of Wilkie’s syndrome has been widely discussed. Descriptions range from a more distal branching of the SMA, hyperlordosis of the lumbal spine, to a loss of the fatty tissue that embeds the SMA. Rapid weight loss is the most described cause of Wilkie’s syndrome. Due to the obstruction caused by the smaller angle, patients with Wilkie’s syndrome will lose even more weight because of their postprandial symptoms. This creates a vicious circle [3,5].

Therapy approaches range from conservative methods, such as additional nutritional supply, to surgical approaches, such as duodenojejunostomy or lowering of the duodenum by dividing the ligament of Treitz [6]. All of these surgical options involve massive upper gastrointestinal surgeries, which cause long-term effects on the patients’ health, lower health-related quality of life, and are normally linked to lower life expectancy. In these cases, the patients would have to undergo a duodenal resection in order to fully restore intestinal continuity. This might again result in intestinal malabsorption. Therefore, the surgical approach should be avoided as long as possible when diagnosing Wilkie’s syndrome, especially in young patients. The first choice should always be the conservative method of additional nutritional supply to reach a target body mass index (BMI) and increase visceral adipose tissue, thereby reaching a higher angle between the SMA and abdominal aorta, due to the increased peritoneal fatty tissue.

Case Report

A 21-year-old male patient was admitted to an outpatient department of a peripheral hospital and was transferred to our surgical department for further therapy planning. The young man exhibited postprandial nausea and vomiting. The symptoms had started about a year ago and became aggravated, when he started to vomit more often, leading to the referral. Thorough anamnesis revealed that psychological stress had induced the symptoms about a year ago. As the symptoms lasted, he had lost about 10 kg, leading to a BMI of 24.2, from an initial BMI of 27.3. He also started inhalant drugs at the same time the symptoms started to occur, due to the described psychological alteration. However, treatment for the described depression was not administered nor sought. The patient underwent a computed tomography (CT) scan and blood testing. The CT scan showed a dilated stomach and a dilated duodenum, with an 8-cm diameter in the pre-stenotic area. An obstruction of the SMA was shown, with an angle of the SMA of 7.5° (Figure 1), thereby creating a maximum distance of 5.1 mm for the duodenum to pass (Figure 2).

The patient was transferred to our tertiary referral center. A gastroscopy was performed, until the lower part of the duodenum had enough elasticity for the duodenum to widen sufficiently. Then, the patient received interdisciplinary treatment with nutritional support, high caloric diet, and analgesic therapy. A psychiatric consultation was performed to evaluate and improve the current psychological wellbeing of the young man, and therapy was administered.

The symptoms improved daily during hospitalization, so that discharge could be initiated on day 6. He was enrolled in regular biweekly follow-up care in our outpatient department. These checkups showed the success of the conservative treatment, with weight gain (BMI discharge, 24 kg/m2; BMI outpatient, 27 kg/m2) and freedom of symptoms since the initiated therapy, suggesting sufficient intestinal passage. The last follow-up of the symptom-free patient was in February 2024.

Discussion

Wilkie’s syndrome, with an incident from 0.013% to 0.3%, is a rare etiology that leads to obstruction of the third portion of the duodenum. It mostly affects female patients with a history of resent weight loss. It is caused by an unusual angle of the SMA to the abdominal aorta below 25° (usually from 38° to 65°), thereby creating a narrow space for the duodenum to pass, below 10 mm [1,2]. A rapid loss of the fatty tissue embedding the SMA is postulated to be the main reason for the small angle leading to the obstruction. Clinical symptoms of nausea, vomiting, and post-prandial stomachache are the logical consequences in this case.

Diagnosis of Wilkie’s syndrome is often delayed due to unspecific clinical findings. A high clinical suspicion and a process of exclusion of other possible causes is necessary to come to the right diagnosis. Even though imaging methods, such as ultrasound and CT, are most likely to identify the obstruction, a gastroscopy should be performed prior to the ultimate diagnosis, to exclude other more severe illnesses [3].

Treatment should be decided individually and should depend on the suspected reason for the syndrome. Conservative therapy should be used prior to a surgical one, especially because of the severity of the surgical approaches [7]. Most patients eventually have to undergo surgical resections or surgical treatment, despite the known long-term effects. The effectiveness of the conservative nutritional approach is very promising, and, if the patient continues to follow the dietary recommendations, a full recovery without surgery is possible [8,9].

In our case, we present a young man who experienced post-prandial nausea and vomiting for about a year before finally being diagnosed. Initially, he had a weight loss due to psychological stress, which probably caused the symptoms and thereby led to even more weight loss.

In an interdisciplinary meeting, the decision for a conservative therapy approach was made. Under nutritional supply, high caloric diet, and sufficient analgesic therapy, the symptoms alleviated, and the patient was discharged and enrolled in regular follow-up in our outpatient department. We could see the success of our therapy by the gained weight and the absence of the symptoms.

We would like this case to be an example to show that the combination of high clinical suspicion to find the right diagnosis and patience with a noninvasive therapy can avoid major surgical approaches and their potential complications.

Conclusions

Wilkie’s Syndrome is a rare entity that greatly affects a patient’s wellbeing. As various symptoms can be experienced by the patient, diagnosis is often delayed. Abdominal imaging is the only mainstay for correct diagnosis. Surgical treatment is often considered, but it is not without consequences. Patients who have to undergo treatment, especially at a young age, face deleterious adverse effects, such as malabsorption, shortened life expectancy, and abdominal discomfort. We present a case in which non-surgical treatment was successful and led to a good recovery, resulting in the sustained amelioration of the patient’s symptoms.