Index
1. What is Wilkie syndrome?
2. Prognosis of the disease
3. Symptoms of Wilkie syndrome
4. Medical tests for Wilkie syndrome
5. What are the causes of Wilkie syndrome?
6. Can it be prevented?
7. Treatments for Wilkie syndrome
Which specialist treats it?
What is Wilkie syndrome?
Wilkie syndrome or superior mesenteric artery syndrome (SAMS) is a pathology caused by extrinsic compression of the third portion of the duodenum between the aorta and the superior mesenteric artery (SMA).
The SMA originates in the aorta at the level of the first lumbar vertebra. The normal aorto-mesenteric angle in adults is between 25-50º. Any factor that attenuates this angle and places it around 7-22º will cause the typical picture of high intestinal obstruction presented by these patients.
Patients present with the typical picture of high intestinal obstruction.
Prognosis of the disease
It is a rare disease. It affects 0.013 – 0.3% of the population and has a higher incidence in women and young adults, between 10 and 30 years of age.
When there is an early diagnosis the prognosis is excellent, as long as it is accompanied by an adequate treatment for each patient. On the other hand, late diagnosis can lead to complications such as gastric pneumatosis, gas accumulation in the portal vein, progressive dehydration or oliguria; in the most severe cases it can result in death due to electrolyte disturbances or gastric perforation.
Symptoms of Wilkie syndrome
The main symptoms include:
– Nausea and vomiting.
– Weight loss.
– Sensation of full stomach.
– Abdominal bloating after meals.
– Pain in the middle region of the abdomen, colicky, relieved by standing or lying on the left side.
– Small bowel obstruction.
Medical tests for Wilkie syndrome
In order to diagnose Wilkie syndrome, a physical examination can be performed, where abdominal rigidity, cachexia or hyperesthesia in the epigastrium, among others, can be detected. However, this evaluation is non-specific and other tests should be performed. It is also necessary to perform serial radiographic examinations of the upper gastrointestinal tract and simple abdominal X-rays. Through computed axial tomography (CT) we can verify the decrease in intra-abdominal fat, the aorto-mesenteric angle, the aorto-mesenteric distance and duodenal dilatation.
Other tests to diagnose the syndrome are ultrasound or arteriography, although the latter is a technique that carries some risks such as allergic reaction to the contrast substance, acute renal failure, vascular perforation, bleeding or embolic events.
What are the causes of Wilkie syndrome?
Wilkie syndrome is usually associated with recent weight loss, surgery to correct scoliosis, and rapid growth. The syndrome may also have other causes such as feeding disorders, severe trauma, congenital defects such as short Treitz ligament or spinal deformities and celiac trunk compression syndrome (in some cases they have both syndromes at the same time).
Sometimes a genetic predisposition, several affected family members or twins have been reported. In other cases the cause has not been found, it is idiopathic.
Can it be prevented?
Wilkie syndrome tends to affect thin patients and, above all, women. Therefore, if we suspect intestinal obstruction or prolonged abdominal pain, we should go to a specialist for a diagnosis.
If we have undergone a corrective surgery of the lateral curvature of the spine, rapid weight loss or we have the syndrome of compression of the celiac trunk, it is very important that we do a follow-up in time and we are attentive to the appearance of possible symptoms. It is also essential to maintain a balanced diet.
Treatments for Wilkie syndrome
Treatments are aimed at alleviating the symptoms that cause the syndrome. They are divided into two types: conservative or non-invasive treatments and surgical or invasive treatments.
Conservative or non-invasive treatment:
– Improve the patient’s nutritional status. Depending on the case, a feeding tube will be necessary for enteral nutrition or when parenteral nutrition through a central line is not tolerated. As the patient gains weight and symptoms subside, solid foods should be gradually introduced.
– Adopt appropriate positions after meals: lie on the left side, face down or sit with the knees to the chest.
– Administer metoclopramide, a medication to treat nausea and vomiting.
Surgical or invasive treatment:
Although the clinical picture is generally managed conservatively, surgery is indicated in the following cases:
– Failure of conservative medical treatment.
– Long evolution disease with progressive weight loss and duodenal dilatation.
– Complicated peptic ulcer disease secondary to bile stasis and reflux.
There are several techniques described for the treatment of this syndrome:
– Strong’s procedure: involves sectioning the ligament of Treitz to mobilize the duodenum and reposition it.
– Gastrojejunostomy: it consists of making an anastomosis or union between the stomach and the jejunum distal to the duodenal obstruction.
– Duodenojejunostomy: in this case an anastomosis is performed between the second duodenal portion and the first loop of jejunum, bypassing the intestinal obstruction. Today it is the procedure of choice and has a success rate of about 90%.
Which specialist treats it?
Wilkie syndrome is treated by a specialist in General Surgery.