Author: Chang Xing, Yang Su, Tang Shanhong
Unit: Department of Gastroenterology, General Hospital of the Western Theater of Operations
Editor's note: In order to help clinical hepatologists broaden their horizons, enrich their practice, and cultivate their clinical thinking ability, International Liver Disease invited the team of Professor Tang Shanhong from the Department of Gastroenterology of the Western Theater General Hospital to jointly create a column of "Liver Difficult Cases". This column brings together the "classic cases" encountered by Professor Tang Shanhong's team in clinical diagnosis and treatment for many years, and will also regularly collect complex or rare clinical cases published in well-known academic journals, focusing on the diagnosis and treatment of various liver diseases, and providing valuable clinical reference for the majority of colleagues.
Thematic review
Gastrointestinal variceal bleeding should be considered to be caused by posterior sinus hypertension such as cirrhosis and Budd-Chiari syndrome, and presinus portal hypertension such as portal vein occlusion and stenosis. Superior mesenteric vein stenosis is a rare condition that can cause local intestinal blood flow reflux disorders, increased pressure, and hypermetabolic dynamic circulatory disorders, resulting in varicose veins in the regional intestine (mainly the small intestine and right colon), and severe variceal veins can rupture and gastrointestinal hemorrhage. In this paper, we report a rare case of severe varices in the ascending colon due to the stenosis of the superior mesenteric vein, which is called "left portal hypertension" according to the pattern of splenic vein stenosis-related disease, and we named it "right portal hypertension".
Summary of medical records
(1) A brief introduction to the medical history
The patient, a 42-year-old male, was admitted to the hospital on 2023-07-03 due to "recurrent blood in the stool for 3 years, aggravation for 3 months, and detection of colonic varices for 1 month".
3 years ago, the patient had blood in the stool after eating a spicy diet, the number of times per year was less, the specific is unknown, it is bright red blood on the surface of the stool, blood dripping after defecation, the amount is small, the stool is yellow, with acid reflux, no nausea, vomiting, no abdominal pain, abdominal distention, no hematemesis, black stool, it can be relieved after the treatment of "hemorrhoids suppository".
Before 3 months, the patient's blood in the stool was aggravated, without obvious causes, mainly blood dripping before or after defecation, bright red, large amount, with acid reflux heartburn, no nausea and vomiting, hematemesis black stool, dizziness and headache, chest tightness and chest pain, palpitations, frequent urination and urgency, bone and joint pain and other discomforts, 2023-04-14 Outpatient blood examination routine in our hospital: hemoglobin 62 g/L, blood in the stool improved after "traditional Chinese medicine treatment" (specifics unknown), no more blood in the stool, and hemoglobin increased on recheck (no report).
1 month ago, the patient had blood in the stool again, the symptoms were the same as before, (2023-05-27) Gastroscopy in Danling County People's Hospital: reflux esophagitis; Barrett's esophagus? Please combine pathology. It is recommended to perform NBI + ME after treatment. Colonoscopy: internal hemorrhoids; Varicose veins of the ascending colon (further investigation recommended).
The patient was admitted to the hospital for further treatment. Since the illness of the patient, the patient was in good spirits, normal physical strength, normal appetite, normal sleep, no significant change in weight, normal stool, normal urination, and was admitted to the hospital for further examination and treatment.
Patient was previously healthy; At the age of 9, he suffered from "tuberculosis", and was treated with drugs for half a year (the specific drug is unknown), at the age of 13, he suffered "a bruise on the back of the head" (the specific drug is unknown), and at the age of 20, he developed "abdominal pain", which improved after hospitalization (the specific cause and treatment are unknown), smoked for more than 20 years, 4~5 cigarettes/day, occasionally drank alcohol, and denied the history of metallurgical travel.
(2) Physical examination
Conscious, anemia, normal abdomen, abdominal wall veins inconspicuous, no intestinal shape and peristaltic waves. Abdominal muscles are soft, no tenderness, rebound tenderness in the abdomen, no fluid wave tremor, no palpable mass in the whole abdomen, no palpable subcostal liver and spleen, negative hepato-jugular venous reflux sign, no palpable abnormality in the gallbladder, Murphy's sign (-). Moving dullness (-), the upper boundary of the liver is located on the right midclavicular line and the flat 5th intercostal space, percussion pain in the hepatic region (-), and percussion pain in the bilateral renal region (-). Bowel sounds were normal, 4 bpm, and no vibrating water or bruit was heard.
(3) Auxiliary examinations
1
Laboratory tests
2023-07-03 Blood routine: white blood cell count: 4.13×109/L, red blood cell count: 3.89×1012/L, hemoglobin concentration: 78 g/L, platelet count: 256×109/L, neutrophil count: 2.9×109/L, mean corpuscular volume: 69.3 fL, mean corpuscular hemoglobin content: 19.9 pg, mean corpuscular hemoglobin concentration: 288 g/L;
Liver function: aspartate aminotransferase 14.2 U/L, alanine aminotransferase 23 U/L, direct bilirubin 2.1 μmol/L, prealbumin 247 mg/L, white globule ratio 1.71, albumin 41 g/L, total protein 65 g/L;
Hepatitis B virology: hepatitis B surface antibody (positive), the rest negative.
2
Electrocardiogram
2023-07-03ECG: (1) sinus rhythm, heart rate 92 beats/min; (2) Normal electrocardiogram.
3
Imaging tests
2023-07-04 320-slice CT aortogram: the main trunk of the superior mesenteric vein is close to the confluence with the splenic vein (marked in Figure 1-C), with a diameter of about 4.26 mm, no abnormal signals such as thrombosis or exogenous compression, and the upstream superior mesenteric vein and celiac branches, including the right colon and some areas of the small intestine, have obvious multiple changes and dilations, and the maximum diameter of the superior mesenteric vein is 8.79 mm, partially surrounding the head of the pancreas, suggesting superior mesenteric varices.
The blood vessels and blood supply on the right side of the abdomen in the portal phase were significantly more than those on the left side in the same period. Locally visible lateral branch circulation flows into the systemic circulation; Compared with the inferior mesenteric venous supply area on the left side of the abdomen, the lumen of the distal branch of the distal superior mesenteric artery on the right side of the abdomen was significantly thickened at the same time, and the duodenum and part of the jejunum in the blood supply range ran in the right upper quadrant region, and the casts were slightly thickened, and the reinforcement after enhancement was significantly stronger than that on the left side.
Figure 1. 320-slice CT aortogram
(A/B) Superior mesenteric vein and its branches are clearly varicose, and the communicating branches of varicose can be seen; (C) The entrance of the superior mesenteric vein and the portal vein is stenosis, about 4.26 mm at its narrowest point; (D) The superior mesenteric vein branch area was more prominently strengthened, and the superior mesenteric vein branch was significantly thicker than that of the inferior mesenteric vein in the same period.
2023-07-05Abdominal large vessel ultrasound: no abnormalities in hepatic veins and branches; The main trunk of the portal vein (about 11.9 mm in diameter) and the splenic vein (8.5 mm) run naturally, with smooth lumen and good blood flow. The superior mesenteric vein is about 12.3 mm in diameter at its widest point, and a slightly tortuous and dilated duct echo can be seen around it, and blood flow signals can be seen in the lumen. Findings: echo of tortuous dilated ducts around the superior mesenteric vein, thought to be varicose veins.
4
Endoscopy
2023-07-06Ultrasound gastroscopy: no abnormalities in pancreas and common bile duct.
2023-07-13Colonoscopy: (1) Varicose veins were seen in the ascending colon, with a maximum diameter of about 0.6 cm, and the local area was flexed into a clump, and no red signs were found; (2) Internal hemorrhoids, under the orthoscopic scope, hemorrhoidal nuclei are seen at 12 o'clock, 3-4 o'clock, and 7-8 o'clock on the oral side of the dentate line, and the surface mucosa is congested.
Figure 2. Colonoscopy: (A/B) varicose veins of the ascending colon with a maximum diameter of about 0.6 cm
Diagnosis and treatment process
The patient's main report is recurrent blood in the stool, and it is a drop of blood after defecation, which is considered to be lower gastrointestinal bleeding, because the patient has no abdominal pain, black stool, and drops of fresh red blood after eating a spicy diet, so the hemorrhoids are first considered to rupture and bleeding, and the preoperative examination and preparation are improved, 2023-07-13Colonoscopy suggests: internal hemorrhoids, under the orthoscopic system, hemorrhoids are seen at 12 o'clock, 3-4 o'clock, and 7-8 o'clock on the oral side of the dentate line, and the surface mucosa is congested, and the endoscopic endoscope is treated with multi-point banding with a banding device.
Because the patient's colonoscopy found moderate to severe varices in the ascending colon, it is thought to be related to local vascular hypertension caused by local blood return obstruction caused by narrowing of the main mesenteric vein. Due to the patient's hemoglobin concentration in our hospital on 2023-07-13: 78 g/L, moderate anemia, it is considered that the patient may also have intracolonic variceal vein rupture and bleeding in addition to hemorrhoidal blood loss, and the patient's ascending colon can see moderate to severe variceal veins, the disease may develop, and there is also a risk of bleeding in the later stage, so early intervention can be given.
After evaluation, it was difficult for the patient to enter the superior mesenteric vein stenosis through the TIPS route, and it was recommended to open the abdomen, and the corresponding vascular shunt should be performed in parallel to solve the problem of the main trunk stenosis of the superior mesenteric vein. The patient is instructed to pay close attention to the color of the stool, regularly review the colonoscopy and abdominal CT with contrast, and seek medical attention immediately if there is lower gastrointestinal bleeding. On September 13, 2023, the patient did not have gastrointestinal bleeding in 2 months after surgery, and the symptoms of anemia disappeared completely.
Case discussion
The hepatic portal vein is a short, thick venous trunk, about 6 cm long and 1.5 cm in diameter, formed by the superior mesenteric vein and the splenic vein at right angles, and the inferior mesenteric vein can be confluent into the splenic vein or superior mesenteric vein or directly to the hepatic portal vein. After entering the liver, the portal vein is divided into left and right branches, and then repeatedly branches in the liver, and finally forms an interlobular vein, which joins the hepatic blood sinusoids together with the branch interlobular artery of the hepatic artery, and then merges into the central vein and veins at all levels after material exchange, and finally merges into the inferior vena cava by the hepatic vein at the second hepatic portal and returns to the right atrium.
There is extensive collateral circulation between the portal vein and the superior and inferior vena cava system, and when the portal venous pressure increases, the lateral collateral circulation of the hepatic portal cava system is formed to relieve hepatic portal vein blood flow and hyperdynamic circulation. Common side side circulation in the presence of portal hypertension includes: (1) hepatic portal vein→ left gastric vein→ esophageal venous plexus→ azygous vein→ superior vena cava; (2) Hepatic portal vein→ splenic vein→ inferior mesenteric vein→ superior rectal vein→ rectal venous plexus→ internal iliac vein→ common iliac vein→ inferior vena cava; (3) Hepatic portal vein→ with umbilical vein→ periumbilical vein network → superior and inferior vena cava. In the presence of portal hypertension, the venous plexus or venous distension described above can lead to esophageal varices, hemorrhoids, and abdominal varices, which are the result of our common collateral hyperdynamic circulation.
In this case, the patient's colonoscopy showed ascending colonic varices, but the gastroscopy showed no esophageal and gastric varices, no obvious abnormalities in liver function after admission, and no obvious signs of liver cirrhosis on imaging, so the ascending colonic varices caused by cirrhosis portal hypertension were not considered for the time being. CT showed that the superior mesenteric vein and its celiac branches were more tortuous and dilated, and the lumen of the distal branches of the superior mesenteric artery was thickened, indicating that the varices and poor reflux of the ascending colonic arteries had affected the superior mesenteric artery and made it compensatorily thickened, and the intestinal casts in the right upper quadrant area of its blood supply range were slightly thickened, and the enhancement was more obvious than that on the left side after enhancement.
In order to clarify the cause of the superior mesenteric varices, abdominal large vessel color ultrasound showed that there were no obvious abnormalities in the inner diameter of the portal vein trunk and the inner diameter of the spleen vein, and the main trunk of the superior mesenteric vein was obviously stenotized before confluence with the spleen vein. There were no varices in the portal vein or splenic vein, and there were no obvious signs of thrombosis. Superior mesenteric varices and thickening of the lumen of the distal branches of the superior mesenteric artery due to stenosis of the superior mesenteric vein proximal to the portal vein (Fig. 1-B), resulting in local varices.
Due to the local stenosis of the superior mesenteric vein near the portal vein, which leads to the superior mesenteric varice, the ascending colonic vein upstream is also varice, and the blood stasis, so that the lumen of the distal branch part of the superior mesenteric artery is compensated for thickening. The physiological changes in cases of superior mesenteric vein stenosis are similar to those of hypermetabolic dynamic circulation after portal thrombosis. Due to the obstruction and stagnation of blood flow in the superior mesenteric vein, the pressure increases, the blood flow and blood volume of the superior mesenteric vein are increased, and the vascular branches are compensated for dilation. The superior mesenteric vein divides into the right colonic vein, and the right colonic vein emits small branches distributed in the upper 2/3 of the ascending colon and the right colon area, so the superior mesenteric vein stenosis can cause ascending colonic varices.
There are many causes of superior mesenteric vein stenosis, from benign strictures caused by pancreatitis or surgical injuries such as pancreaticoduodenectomy to malignant strictures caused by tumors such as pancreatic cancer or midgut carcinoid. The clinical manifestations of supramesenteric vein stenosis are focal mesenteric-venous hypertension with refractory ascites, gastrointestinal bleeding, portal vein thrombosis, and even congestive intestinal infarction [1,2]. Typical CT findings include intestinal wall thickening, intestinal dilation, mesenteric injection, and superior mesenteric venous lumen stenosis [3], which require further angiography to confirm the diagnosis.
It has been reported that blunt abdominal trauma can also cause isolated superior mesenteric vein injury [4], and a detailed history shows that the patient has no history of abdominal trauma, impingement, surgery, pancreatitis or cholangitis, abdominal tumors, etc., and excludes local stenosis caused by other factors, so the patient is judged to have idiopathic superior mesenteric varices.
It is widely recognized that splenic venous hypertension is referred to as "left-sided portal hypertension" [5] due to stenosis or obstruction of the splenic vein, which is caused by invasion or compression of the splenic vein, which causes splenic venous blood to flow into the portal trunk through collateral circulation. Similarly, we named this case of localized venous stenosis caused by stenosis of the superior mesenteric vein on the right side of the main trunk of the portal vein as "right-handed" portal hypertension.
Endoscopic surgical management (excision of the bleeding site of the lesion or reconstruction of the vascular bypass to bypass the stenotic vein) may be considered for branches with acute bleeding. For non-acute supramesenteric vein stenosis, the predominant route is the endovascular route (angioplasty or stent implantation) to obtain atresia/stenotic venous recanalization as a means of addressing venous hypertension. However, percutaneous supramesenteric stenting is more minimally invasive than open surgery.
A retrospective analysis of the technical and clinical success of percutaneous supramesenteric venous stenting in 6 patients was reported [6], with stenosis resulting in symptomatic ascites in 3 patients, mesenteric ischemia in 2 patients, and recurrent gastrointestinal bleeding in 1 patient. Stenting is performed using a percutaneous transhepatic approach using a self-expanding nitinol stent. Percutaneous supramesenteric venous stenting was successful and clinically successful in all patients. No interventional complications occurred. During the average follow-up period of 6 months (range 2~10 months), one patient developed early stent occlusion 2 weeks after stent placement.
Overall, the use of self-expanding nitinol stenting for symptomatic superior mesenteric stenosis is feasible and effective. The patient has undergone endoscopic banding therapy, and follow-up will continue, and if the patient has symptoms of gastrointestinal bleeding and hematochezia, percutaneous superior mesenteric venous stenting can be considered for further treatment.
Bibliography:
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[2] HELLMAN P, HESSMAN O, ÅKERSTRöM G, et al. Stenting of the Superior Mesenteric Vein in Midgut Carcinoid Disease with Large Mesenteric Masses [J]. World Journal of Surgery, 2010, 34(6): 1373-9.
[3] FURUKAWA A, KANASAKI S, KONO N, et al. CT Diagnosis of Acute Mesenteric Ischemia from Various Causes [J]. American Journal of Roentgenology, 2009, 192(2): 408-16.
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Writer
Chang Xing, attending physician of the Department of Gastroenterology, Master of Medicine, has been engaged in the diagnosis and treatment of digestive diseases for a long time.
Writer
Yang Su, Department of Gastroenterology, Western Theater General Hospital, Master's Degree Candidate.