OVERVIEW
是一种以胆囊的黏膜和肌层增生为主的胆囊非炎性的良性病变
临床表现主要有消化不良、腹痛、恶心、呕吐、腹泻等
与退行性变、胆囊排空障碍、慢性炎症、神经肌肉紊乱等有关
根据病情采取非手术治疗、手术治疗等
Definition
Adenomyosis of the gallbladder is an acquired, noninflammatory, and nonneoplastic benign disease characterized by chronic hyperplasia of the gallbladder glands and muscularis propria with hypertrophy of the gallbladder mucosal epithelial cells and invagination into the muscularis propria interstitium, followed by the formation of Rokitansky-Aschoff sinuses (RAS).
Staging
Based on the general features of gallbladder adenomyosis, it is categorized into limited, segmental and diffuse types:
Limited type: commonly found at the base of the gallbladder, hence the name basal type, the lesion is limited and thickened in a hemispherical shape and bulges into the gallbladder cavity, similar to a polyp.
Segmental type: the wall of the gallbladder is thickened in a circular pattern toward the lumen, forming a circular stenosis in the body or at the junction of the body and neck, resembling a gourd or hourglass.
Diffuse type: diffuse thickening of the gallbladder wall, with scattered distribution of hyperplastic, dilated ro- a sinus.
Pathogenesis
Adenomyosis of the gallbladder occurs in adults over 50 years of age and is complicated by gallbladder stones in more than 50% of ADM cases [1].
With the development and popularization of abdominal imaging techniques, gallbladder adenomatosis is increasingly being detected, with prevalence rates ranging from approximately 1.0% to 9.0%, with large autopsy series reporting a prevalence of 7.0% [1].
25% to 95% of gallbladder adenomyosis is combined with gallbladder stones and 33% with cholesterol deposition [2].
Etiology
Causes
The cause of adenomyosis of the gallbladder is not well established and may be related to the following factors:
Degenerative changes
It is generally accepted that gallbladder adenomyomatosis is not a congenital disease but a degenerative change.
Impaired emptying of the gallbladder
As a result of increased pressure in the gallbladder lumen, the mucosa can eventually protrude from the muscularis propria fissura and is most often found distal to the gallbladder.
Chronic inflammation
Long-term stimulation by gallbladder stones and chronic inflammation of the gallbladder is an important factor leading to adenomatous hyperplasia of the gallbladder.
Neuromuscular dysfunction
Abnormal proliferation of nerve fibers in the gallbladder wall and neuromuscular dysfunction lead to the development of gallbladder adenomyosis.
Risk factors and predisposing factors
People with any of the following risk factors are at high risk of developing adenomyosis of the gallbladder.
Eating greasy food.
People with diseases of the biliary system such as cholecystitis, gallbladder stones, cholesterol deposition.
Symptoms
Main Symptoms
Dyspepsia
If gallbladder adenomyosis is not accompanied by stones, the onset of the disease is usually insidious and the course of the disease is slow, and the main manifestations may include lack of appetite, a feeling of fullness in the middle and upper abdomen, and belching.
Abdominal pain
Adenomyosis of the gallbladder is often associated with stones, and usually occurs with a vague pain, distension or typical biliary colic in the right upper abdomen when eating fatty meals.
Nausea and vomiting
Nausea and vomiting of varying degrees are often present in acute attacks, which are associated with hypermotility and spasm of the lower esophagus, stomach and duodenum.
Diarrhea
Due to the strong contraction of the gallbladder, bile can be rapidly discharged into the duodenum, stimulating increased intestinal peristalsis and diarrhea.
Other symptoms
Some patients are asymptomatic, usually found incidentally during physical examination or cholecystectomy for other reasons.
Certain patients without stones may have specific symptoms or may present with unexplained fever.
In the presence of choledochal adenomyosis or papillary adenomyosis, obstructive jaundice may occur due to narrowing and obstruction of the bile ducts.
Elderly patients may develop the so-called “three highs syndrome”, i.e., high gallbladder concentration, high agitation, and high emptying, resulting in epigastric colic, dyspepsia, and depressed mood [3].
Consultation
Department of Medicine
Department of Hepatobiliary Surgery
When symptoms such as biliary colic, nausea and vomiting occur, or when physical examination reveals adenomatous hyperplasia of the gallbladder, it is recommended that prompt medical attention be sought.
Gastroenterology
When symptoms such as dyspepsia, lack of appetite, or a feeling of fullness in the middle or upper abdomen occur, it is recommended that you consult a doctor promptly.
Preparation
Consultation: Registration, Preparation of documents, Frequently Asked Questions
Tips for seeking medical treatment
Try to keep a record of symptoms, duration, and previous treatments for your doctor’s reference.
Take pictures of abnormal skin conditions such as jaundice.
Preparation Checklist
症状清单
Pay particular attention to the time of onset of symptoms, special manifestations, etc.
Are there symptoms of indigestion such as belching and loss of appetite?
Are there symptoms of abdominal pain, diarrhea, nausea, vomiting?
What is the location, frequency and degree of abdominal pain?
Are there any color changes in the skin?
Are there any aggravating or relieving factors for the above symptoms?
How long have the above symptoms lasted?
病史清单
Is there any history of biliary system disease such as cholecystitis or gallbladder stones?
检查清单
Test results in the last six months that can be carried to the doctor’s office
Laboratory tests: liver function.
Imaging tests: abdominal ultrasound, CT, MRI, magnetic resonance cholangiopancreatography (MRCP).
Diagnosis
Diagnosis is based on
Medical history
Past history of biliary system diseases such as cholecystitis and gallbladder stones is possible.
Clinical manifestations
Most patients have no clinical signs and symptoms, while some may present with vague pain and distension in the right upper abdomen, nausea, vomiting, loss of appetite and diarrhea.
Some patients may also have yellow coloring of the skin and sclera, and fever.
Laboratory tests
Routine blood tests and liver function tests are often performed. The results are mostly without abnormality. If accompanied by jaundice, serum bilirubin and alkaline phosphatase are elevated.
Imaging examination
腹部超声
Diffuse, ovoid or beaded intramural diverticula (1-10 mm) in the affected portion of the gallbladder wall are characteristic of adenomyosis of the gallbladder.
In most cases, these features are not always present, especially when the ro-ar sinus is relatively small (<3 mm).
腹部CT
CT abdomen is advantageous in identifying cases with predominantly thickened gallbladder wall and is helpful in differentiating ADM from gallbladder cancer.
The CT manifestations of ADM are focal or diffuse, non-specific gallbladder wall thickening and enhancement. In limited ADM, a focal mass at the base of the gallbladder can be seen, with marked enhancement of the mucosal layer and submucosa in the arterial phase of the lesion, and the thickened wall of the gallbladder can show the characteristic “candida sign”, due to the thin, reinforced epithelium lined with the RAS, and surrounded by the hyperextended hypertrophic muscularis propria. The characteristic “rosary sign” can be seen.
On enhanced CT, faint gray enhancement points can be seen on the outer border of the dotted enhancement layer in the thickened gallbladder wall, showing the “cotton ball sign”.
腹部MRI
Thickening of the gallbladder wall and RAS in ADM can be easily visualized, and RAS with high T2 and low T1 signals can show the characteristic “pearl necklace sign” on MRI [1].
磁共振胰胆管造影（MRCP）
MRCP can show the RAS more clearly, as well as the thickness of the gallbladder wall, whether the gallbladder wall is smooth and whether there is calcification, and whether the liver-biliary junction is clear, which can significantly improve the diagnostic accuracy.
During MRCP examination, the characteristic “pearl necklace sign” can be demonstrated.
Differential diagnosis
Adenomyosis of gallbladder should be differentiated from thickened gallbladder cancer and gallbladder polyps [5]:
Thickened gallbladder cancer
Similarities: both may present with symptoms such as dyspepsia, loss of appetite and belching.
Differences:
增厚型胆囊癌表现为胆囊壁不均匀增厚，局部形成肿块向腔内或腔外突出，边缘轮廓常显示不清，CT增强扫描呈明显不均匀强化，常侵犯邻近肝脏组织。
胆囊腺肌增生病表现为胆囊壁增厚相对均匀，常可见小囊状RAS与胆囊腔相通，CT增强扫描具有渐进性向浆膜面扩展的强化特点。
Gallbladder polyps
Similarities: both can present with symptoms such as biliary colic, nausea, and vomiting.
Differences:
胆囊息肉表现为胆囊腔内结节状软组织影，多发生于胆囊体部，密度/信号均匀，边界清晰，CT增强扫描可呈轻度或明显强化。
胆囊腺肌增生病局限型多发生于胆囊底部，呈帽状、结节状或乳头状增厚凸向腔内或腔外，边缘光滑。
Treatment
Treatment objective: to relieve symptoms, control disease progression, prevent and reduce complications.
Treatment principle: according to its typing, adopt individualized and differentiated treatment strategy, the treatment of gallbladder adenomyosis is mainly surgical treatment.
Non-surgical treatment
When the gallbladder function is shown to be good and there is no stone, the attack can be controlled by restricting oily diet and anti-inflammatory and choleretic.
Surgical treatment
Although it is inconclusive whether adenomyosis of the gallbladder has a malignant tendency, it is believed that ADM is a risk factor for gallbladder cancer, especially segmental ADM. it has been reported that it is difficult to distinguish ADM from early gallbladder cancer, so some scholars advocate surgical resection of ADM once it is diagnosed.
Surgical resection alone is the most important treatment.
Confined adenomyosis of the gallbladder
Surgery is not recommended for asymptomatic limited ADM [1].
Follow-up of asymptomatic limited ADM is needed, with close observation of lesion size and growth rate.
Symptomatic limited ADM with or without gallbladder stones should be treated surgically.
Other causes of abdominal pain must be excluded preoperatively, and laparoscopic cholecystectomy is preferred in the absence of contraindications, which can lead to complete resolution of symptoms after surgery [1].
Segmental adenomyosis of the gallbladder
Segmental ADM and gallbladder cancer may have some correlation, and it is difficult to identify when coexisting gallbladder cancer, and some scholars believe that patients with segmental ADM should be treated surgically regardless of whether they are symptomatic or not, and regardless of whether they are combined with gallbladder stones or not [1].
Diffuse adenomyosis of the gallbladder
Patients with asymptomatic diffuse ADM should be evaluated individually and comprehensively for age, gender, concomitant gallbladder stones, ethnicity, family history of tumors, and tumor risk factors such as pancreaticobiliary confluence abnormalities.
In order to make a definitive diagnosis of ADM, there is a rationale for prophylactic cholecystectomy for diffuse ADM, especially when gallbladder cancer cannot be excluded [1].
Adenomyosis of the gallbladder that cannot be definitively diagnosed preoperatively
Cholecystectomy is justified when there is any diagnostic suspicion of gallbladder cancer.
The surgeon should incise the gallbladder specimen in the operating room for careful observation and initial gross judgment, and should inform the pathologist of the relevant clinical situation in order to provide rapid and definitive histologic results.
If the gallbladder wall thickening is caused by ADM, no further treatment is necessary, but if it is caused by GBC, radical surgery should be immediately rescheduled.
Patients who undergo cholecystectomy for other benign diseases of the gallbladder and have postoperative pathologic findings of ADM do not require subsequent treatment.
Adenomyosis of the gallbladder combined with abnormal pancreaticobiliary confluence
Whether symptomatic or asymptomatic, with or without primary cystic dilatation of the bile ducts, ADM with an abnormal pancreaticobiliary confluence is a known risk factor for GBC, and requires prophylactic cholecystectomy, if necessary, based on the patient’s condition and evaluation.
Prognosis
Cure
Adenomatous hyperplasia of the gallbladder is often underdiagnosed due to its lack of recognition in the past and is rarely considered clinically.
The prognosis of all diagnosed patients is good after surgical treatment [6].
Daily
Daily management
Dietary management
Avoid eating greasy food, prefer small meals, light and easy to digest, and avoid oversatiety.
Eat more foods high in vitamins and rich in dietary fiber, fresh fruits and vegetables.
Life Management
Reasonable arrangement of work and rest time, combination of work and rest, avoid overwork and high mental tension.
Psychological support
Maintain mental relaxation, avoid irritability, anxiety and nervousness.
Encourage and support the patient to take active treatment, give the patient enough psychological comfort, and guide the patient to face the disease correctly.
Follow-up
Generally, it is recommended that the patient be followed up every 3 to 6 months so that the doctor can assess the changes in the patient’s condition.
Examinations needed for follow-up: abdominal ultrasound, CT and MRI may be needed if necessary.
Prevention
Regular physical examination, early prevention and treatment of biliary system diseases such as cholecystitis and gallbladder stones.
Eat regularly, avoid overeating and eat lightly; eat less greasy food.
参考文献
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何潇,徐智. 胆囊腺肌增生症的研究进展[J]. 中国微创外科杂志，2016，16（6）：562-565.
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陈孝平，汪建平，赵继宗. 外科学.人民卫生出版社，2018.
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