OVERVIEW
Dieulafoy’s disease, also known as Dieulafoy’s lesion, is composed of submucosal constant diameter arterioles and superficial mucosal erosions, and is one of the causes of gastrointestinal hemorrhage, especially upper gastrointestinal hemorrhage, which can occur anywhere in the gastrointestinal tract, with the proximal stomach being the most common. The main clinical manifestations of Diuraphua disease are recurrent episodes of blood vomiting and tarry stools, and in severe cases, hemorrhagic shock; there is no obvious epigastric discomfort or pain before bleeding, and there is no history of peptic ulcer or family history of inheritance.
Etiology
Under normal circumstances, the blood supply of the gastric wall mainly comes from the short gastric artery, which enters the gastric wall and its branches gradually become thinner and thinner, eventually forming a capillary system in the gastric mucosa, but in patients with Dierafua disease, the branches of short gastric artery enter the gastric mucosa and maintain a constant diameter, which is thus called the constant diameter artery.
It is believed that the constant diameter artery is a congenital developmental abnormality, and there is a special relationship between the constant diameter artery and the mucosa. Under normal conditions, the submucosal laxity of the mucosa allows the mucosa on the surface of the artery to move freely, but in patients with Dierafowicz’s disease, the Wanken fiber bundles fix the artery and the mucosa and form a specific area of mucosal vulnerability, and the relationship of the Wanken fiber bundles with the artery and the mucosa is likely to be a result of congenital origin. Mucosal damage occurs in the vulnerable zone when stimulated by external factors and causes rupture of the submucosal constant diameter arteries; with age, the arterial diameter expands and the mucosa atrophies, making this weak environment even more susceptible to damage.
A variety of factors can promote gastric mucosal erosion and constant diameter artery rupture, such as heavy drinking, smoking, bile reflux can cause gastric mucosal erosion; gastric peristalsis constant diameter artery pressure, elongation, peristalsis generated by the cutting force or mechanical damage can also cause vascular rupture. Constant diameter artery is not a sudden corrosion rupture, but due to the wall gradually thinning, expansion and rupture, rupture is often preceded by thrombosis. Some studies have found varying degrees of atherosclerosis in the blood vessels at the site of Diuraphua disease, with the hardening of the blood vessels making rupture more likely, a phenomenon that may explain the older age of onset of Diuraphua disease.
Symptoms
The main clinical manifestations of Diuraphua disease are recurrent episodes of vomiting blood and tarry stools, and in severe cases, hemorrhagic shock; there is no obvious epigastric discomfort or pain prior to the hemorrhage, and there is no history of peptic ulcers or family history of the disease.
Examination
1. Positive fecal occult blood test.
2. Routine blood test
Decrease in total hemoglobin.
3. Endoscopy
The endoscopic manifestations of Diehlafuwa disease are different, the main features are focal defects of gastric mucosa in cardia area with jet-like bleeding; superficial depression of gastric mucosa with blood vessels walking in the middle of the defects and blood clots adhering to the surface; occasionally, small blood vessels can be seen protruding out of the surface of the normal mucosa with pulsatile bleeding.
4. Selective angiography
The diagnostic rate of Dierafowicz’s disease is 20% to 30%. The angiographic features of Dierafowicz’s disease are: the contrast agent enters the proximal part of the stomach through the left gastric artery, and rapidly enters the gastric lumen from the mucosal punctate vesicular area; the arterial morphology is normal, and there is no aneurysm formation or arteriovenous shunt. However, selective abdominal arteriography must be performed in the presence of active bleeding to successfully indicate the site of bleeding, and if the bleeding stops, consider leaving the catheter in the vessel for 24 hours, and imaging as soon as there is bleeding can be expected to obtain a diagnosis. It is generally recognized that in patients with Diehlafuerzia who remain negative after multiple endoscopic examinations, selective abdominal arteriography may be used to make a definitive diagnosis.
5. Nuclide examination
The use of 99mTc-erythrocyte tracer technique is helpful in detecting bleeding points.
Diagnosis
Due to the lack of specificity of the clinical manifestations of Dierafowicz’s disease, endoscopy, selective angiography, nuclear tracing and other examination methods can help preoperative diagnosis of Dierafowicz’s disease and provide an important basis for surgical treatment, and the diagnosis of some patients can only be obtained at the time of caesarean section and post-mortem pathologic examination.
Intraoperative diagnosis: Dierafowicz’s disease is mostly diagnosed during emergency surgical exploration. As long as the special location and pathological features of Dierafowicz’s disease are fully recognized, a definite diagnosis can be obtained during intraoperative exploration.
Differential diagnosis
In the diagnosis of Diehlafua disease, care should be taken to differentiate it from Malloy-Weiss tear and hemorrhagic gastric vasodilatation.
Treatment
Diehlafua disease has a high morbidity and mortality rate, with hemorrhagic shock and multiorgan failure being the main causes of death. Early diagnosis and effective treatment is essential. Endoscopic treatment, selective left gastric artery embolization and surgical treatment are available.
1. Surgical treatment
In the past, surgery was considered to be the treatment of choice for Diuraphua disease. With the advancement of endoscopic treatment, surgical treatment has tended to be preceded by endoscopic treatment, and decisive surgery should be performed if endoscopic treatment is ineffective.
2. Other therapies
(1) Endoscopic treatment The majority of patients with Diehl-Alfa disease can be successfully treated with endoscopic therapy. The success rate of endoscopic hemostasis has been reported to be 96% in the literature. The methods of endoscopic treatment include injection therapy, thermal probe, microwave, high-frequency electrocoagulation, laser and other thermal therapy, and hemostatic clips, loopers and other instrumentation.
(2) Embolization therapy Selective abdominal arteriography and embolization for Diehlafua disease have been reported less frequently. It should be a particularly useful treatment for those who have failed endoscopic therapy and cannot tolerate surgery.
Prevention
Early detection, early diagnosis and effective treatment are essential.