Wilkie Syndrome - Causes, Symptoms, Diagnosis, and Treatment

Introduction:

The superior mesenteric artery (SMA), also known as Wilkie's syndrome or cast syndrome, is a rare condition that can result in obstruction by compressing the third section of the duodenum between the SMA and the aorta. The proximal intestinal blockage caused by SMA syndrome is unusual and usually affects young individuals who have lost a significant amount of weight. A thorough medical history, as well as imaging results, is recommended to diagnose SMA syndrome. Several problems, including electrolyte imbalance, catabolic wasting, peritonitis, and gastrointestinal perforation, could arise from delaying this diagnosis. To restore the mesenteric fat pad and widen the aortomesenteric angle, conservative therapy mostly involves weight gain that is done orally or parenterally. Surgery may be used as a last resort if this non-invasive strategy is unsuccessful, with duodenojejunostomy being the currently recommended course of action.

What Is Wilkie Syndrome?

The third and last segment of the duodenum is crushed between the abdominal aorta (AA) and the overlaying superior mesenteric artery is called superior mesenteric artery (SMA) syndrome, a gastro-vascular condition. It is an uncommon, potentially fatal disease. It is frequently characterized by an angle between the AA and the SMA of 6 to 25 degrees, as opposed to the normal range of 38 to 56 degrees (mesenteric fat). However, patients with low BMI (body mass index), particularly children, have been reported to have a narrow SMA angle without exhibiting any signs of SMA syndrome. A narrow SMA angle alone cannot be used to diagnose SMA syndrome. However, it is possible to be diagnosed with both disorders. It is separate from nutcracker syndrome, the entrapment of the left renal vein between the AA and the SMA. The other synonyms of SMA syndrome are Wilkie's syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus, and intermittent arterio-mesenteric occlusion.

What Are the Causes of Wilkie Syndrome?

Typically, the duodenum is cushioned by retroperitoneal fat and lymphatic tissue to prevent compression by the SMA. Hence, any situation having a thin cushion and a small mesenteric angle causes SMA syndrome. The occurrence of SMA is broadly classified into:

• Chronic: Depending on the severity of duodenal compression, patients with the chronic, congenital type of SMA syndrome typically have a long or even lifetime history of stomach problems with sporadic exacerbations.

• Acute: After traumatic events that forcefully hyperextend the SMA across the duodenum, creating an obstruction or unexpected weight loss, result in the acute type of SMA syndrome. Long-term supine bed rest, scoliosis surgery, left nephrectomy, and ileoanal pouch surgery may also lead to SMA.

What Are the Symptoms of Wilkie Syndrome?

The major symptoms of Wilkie Syndrome are:

• Nausea.

• Vomiting.

• Severe postprandial abdominal pain, which is "stabbing" in nature and caused by both the duodenal compression and the compensatory reversed peristalsis.

• External abdominal hypersensitivity.

• Abdominal pain or distortion.

• Abdominal tenderness.

• Reflex.

• Heartburns.

• Feeding is more challenging in newborns with SMA so it may lead to poor weight gain.

A common symptom of the chronic type of SMA syndrome is a fear of food. Many people get relief from their symptoms while in the prone position (face down), left lateral decubitus, or the chest position. A Hayes maneuver elevates the root of the SMA and slightly loosens the constriction by applying pressure in a cephalad and dorsal manner below the umbilicus. While facing up or slanting to the right, symptoms may become worse.

What Is the Pathology Related to Wilkie Syndrome?

When a huge weight is lost, the cushion around the SMA will be reduced. This can result in a reduction in the distance between the artery and the aorta. A few conditions which can result in weight loss is

• Anorexia nervosa.

• Malabsorption.

• Burns, surgery, malignancy.

• Cognitive heart failure.

What Is the Clinical Presentation of Wilkie Syndrome?

The person who is affected with Wilkie syndrome will exhibit acute presentation of chronic symptoms, such as

• Symptoms related to duodenal obstruction.

• Chronic cases may exhibit abdominal symptoms, anorexia, and recurrent abdominal pain, along with vomiting.

How to Diagnose Wilkie Syndrome?

SMA syndrome is only considered after patients have thoroughly examined their gastrointestinal tract, including upper endoscopy, and after being checked for various malabsorptive, ulcerative, and inflammatory intestinal conditions with a higher diagnostic frequency. Since they may try to adjust to the condition by gradually reducing their food intake or naturally gravitating towards a lighter and more digestible diet, patients with the earlier stages of SMA syndrome frequently do not realize they have the illness until significant harm to their health has been done.

Some diagnostic criteria of Wilkie Syndrome are:

• X-ray: X-ray shows duodenal dilation followed by sudden constriction close to the surrounding SMA and a 4 to 6-hour transit delay across the gastroduodenal area.

• Computed Tomography: Upper gastrointestinal series (UGI), abdominal and pelvic computed tomography (CT) scan with oral and intravenous contrast, and, for complex cases, hypotonic biodemography are common diagnostic procedures.

• Vascular Imaging Tests: Contrast angiography and ultrasound may be performed to show a narrower SMA angle or increased blood flow velocity via the SMA.

What Are the Treatment Options for Wilkie Syndrome?

The Wilkie Syndrome is initially treated by medical treatment involving the insertion of a nasogastric tube for duodenal and gastric decompression, mobilization into the prone or left lateral decubitus position, reversal or removal of the precipitating factor with appropriate nutrition, replacement of fluid and electrolytes, or peripherally inserted central catheter (PICC line) administration of total parenteral nutrition (TPN). When reverse peristalsis persists, or recovered fat refuses to build within the mesenteric angle, symptoms could improve after weight gain. Despite their medical history, most patients benefit from nutritional support with hyperalimentation.

Surgical intervention is necessary only when medical treatment is unsuccessful. The most common surgical procedure is duodenojejunostomy. This procedure can be done openly or laparoscopically to connect the duodenum with the jejunum, circumventing the compression that the AA and the SMA cause. SMA syndrome can occasionally coexist with a serious, life-threatening disease like cancer or AIDS (acquired immune deficiency syndrome). Nonetheless, even in these situations, SMA syndrome treatment can result in a decrease in symptoms and an improvement in quality of life.

Conclusion:

Strong clinical suspicion is crucial, especially in patients with significant weight loss and signs of stomach distension. A higher level of awareness is indicated for early identification to prevent the patient from needless suffering. Stress from surgery should also be taken into account as a cause of SMA syndrome. Additionally, it is necessary to draw attention to the challenge of making an exact diagnosis of SMA syndrome in an unusual clinical situation as what occurred in our case.