Spinal enterogenous cysts (SEC) are a rare congenital and developmental malformation, which are formed from ectopic mesodermal tissue derived from the foregut during embryonic development, causing innate diseases within the spinal canal. Clinically, they are relatively rare. According to Fortund's report, they account for 12% of spinal cystic diseases. In recent years, with the continuous development of imaging technology, there have been increasing reports on this disease in China, and effective treatment has been achieved. In the past, the naming was more complex, including gastrointestinal cell tumors, bronchogenic cysts, endodermal cysts, foregut-derived cysts, teratoma-like cysts, and so on. In recent years, the term 'neurenteric cyst' is more commonly used in literature. The WHO Central Nervous System Tumor Histological Classification (1993) names it 'enterogenous cysts' and defines it as 'cysts with an inner wall lined with epithelium similar to that of the gastrointestinal tract, which can secrete mucus'.
Wilkins and Odom classified SEC into three types based on the tissue source of the cyst wall:
Type I, the basal membrane of the cyst wall is a single or pseudostratified columnar or cuboidal epithelium (with or without villi), similar to gastrointestinal epithelium (accounting for 50%), respiratory epithelium (17%), or a mixture of both epithelia (33%).
Type II, similar to type I, with the following tissues: mucous glands, smooth muscle, fat, cartilage, bone, elastic fibers, lymphoid tissue, or ganglia.
Type III, similar to type I, with ependymal or glial tissue as inherent components, rather than simply surrounding the cyst.
More than 80% of simple cysts are type I, while the cyst walls of cysts with associated malformations often have mesenchymal or ectodermal derivatives. This may mean that the embryonic developmental disorder in patients with simple cysts appears later, and therefore, cell differentiation is better, and the cysts produced only have endodermal components. Therefore, there is a hypothesis that the cause of malformation is not the primordial germ cells, and the degree of lesion is related to the stage of embryonic development at which the error occurred. Some cyst lesions have hyaline degeneration, necrosis, and chronic inflammatory cell infiltration; immunocytochemical examination expresses CD68, leukocyte common antigen, human leukocyte antigen II-positive cells, and tumor necrosis factor.
1. What are the causes of enterogenous cysts?
The clinical manifestations of enterogenous cysts are diverse. Unlike those occurring in the spinal canal or intracranially, enterogenous cysts in the abdominal cavity have a later onset age due to the larger abdominal cavity volume and greater organ mobility compared to the spinal canal or intracranial cavity, hence symptoms caused by cyst compression appear later. The clinical manifestations of enterogenous cysts in the abdominal cavity vary with the size, location, and shape of the cysts. As the volume of the cysts continues to increase, complications mainly involving compression of adjacent organs appear. Non-specific gastrointestinal symptoms such as nausea, vomiting, abdominal distension, and abdominal pain, as well as painless abdominal masses, are often the initial manifestations of the disease. With the progression of the disease course, different locations of the cysts can cause symptoms such as intestinal obstruction, intussusception, and urinary tract obstruction. In addition, due to the presence of gastric mucosal tissue in some cysts, which can secrete a large amount of hydrochloric acid, this can cause ulcers, bleeding, and perforation in the cyst itself and adjacent tissues and organs. In this case, the non-specific gastrointestinal symptoms present a pathological process of pain, intermittent pain, and recurring pain, and this pain with a remission period may be related to the periodic rupture of the cyst, with the overflow of cyst fluid causing aseptic inflammation; the gradual enlargement of the mass is due to the continuous secretion of endothelial adenocytes in the cyst wall, which has lost its balance with the resorption function of the cyst wall. When the mass is small, there are no positive signs, and when the mass is large, the abdominal wall can be felt to have a smooth mass with clear boundaries.
3. What are the typical symptoms of intestinal cysts?
This disease is more common in children and adolescents, with the minimum age being 11 days after birth, and the older ones rarely exceed 40 years old. Generally, men are more than women, with a ratio of 2-3:1.
The initial symptoms are often radicular pain at the site of the cyst, with more bilateral neck pain, limited neck movement, and neck resistance. About half of the patients have recurrent symptoms, that is, there are periods of remission and exacerbation, and are accompanied by low fever. This remission and recurrence may be due to the periodic rupture of the cyst, or the leakage of cyst fluid may alleviate the symptoms, followed by an increase in the secretion of epithelial cells on the cyst wall, causing the cyst to gradually increase in size and compress the spinal cord again, thus reappearing the symptoms. Some patients present with an acute onset, with rapid progression of the disease, often appearing limb sensory and motor disorders and sphincter dysfunction in a short period of time. Particularly, motor disorders are more common, presenting with paraplegia or quadriplegia.
This disease often complicates with other congenital malformations, with the most common being malformations in the corresponding parts of the spine, such as cranial depression, atlanto-occipital malformation, vertebral fusion, spina bifida, hemivertebra, meningocele, scoliosis, etc. Additionally, there are also digestive and respiratory tract malformations, such as intestinal atresia, esophageal or intracranial diverticula, bronchial and mediastinal cysts, defects in the mediastinum or the squamous part of the occipital bone. In addition, in the high cervical segment, there may be malformation of the cerebellar tonsillar hernia, see the typical cases in this section.
4. How to prevent intestinal cysts?
Intestinal cysts are congenital diseases, and the main preventive measure is to start from the mother.
1. Pre-marital examination: The first line of defense in preventing the birth of congenital malformed children.
2. Prenatal examination: Establishing a comprehensive pre-pregnancy routine examination system.
3. Amniocentesis holds a very important position.
4. At the molecular level, fetal DNA is extracted from amniotic fluid cells for direct or indirect analysis or detection of a specific gene. This includes diagnosing conditions such as thalassemia, phenylketonuria, and progressive muscular dystrophy.
5, cytogenetic level. After collecting amniotic fluid, it can be cultured in vitro for genetic karyotype analysis, which is the most widely used diagnostic method at present. The amniotic fluid chromosomes have the following uses: (1) It has a diagnostic significance for more than 100 kinds of chromosomal abnormality syndromes such as Down syndrome and ambiguous sex; (2) It can determine the sex of the fetus early for fetuses with a family history of X-linked recessive inheritance diseases, such as hemophilia, color blindness, and pseudohypertrophic muscular dystrophy, and terminate pregnancy for male fetuses; (3) It can carry out selective breeding for pregnant women with a family history of chromosomal genetic diseases.
5, protein diagnostic level. By measuring specific proteins or proteases in amniotic fluid, a disease can be diagnosed, such as alpha-fetoprotein and cholinesterase in diagnosing fetal neural tube defects, omphalocele, anencephaly, stillbirth, congenital esophageal atresia, duodenal atresia, congenital nephrotic syndrome, severe Rh incompatibility pregnancy, and so on; measuring the activity of hexosaminidase A to diagnose familial amyotrophic lateral sclerosis; measuring enzymes in sugar and lipid metabolism to diagnose lipid metabolism disease, mucopolysaccharide deposition disease, amino acid metabolism disease, carbohydrate metabolism disease, and so on.
5. What kind of laboratory tests are needed for intestinal cysts
Firstly, the patient needs to undergo a B-ultrasound examination, which is a simple and non-invasive examination method, and can also distinguish liver flukes. X-ray examination: including abdominal flat film, selective abdominal aortic angiography, gastrointestinal contrast, CT examination, and nuclear magnetic resonance examination.
Liver cysts mainly rely on imaging examinations for diagnosis. In imaging diagnosis, ultrasound examination is the most important. In terms of the定性 of liver cysts, it is generally believed that ultrasound examination is more accurate than CT. However, when it comes to a comprehensive understanding of the size, number, location, and related organs around the liver, especially for patients with large liver cysts who need surgical treatment, CT examination plays a guiding role in surgery that is obviously superior to B-ultrasound.
In general, patients with liver cysts do not need to undergo color Doppler ultrasound and magnetic resonance imaging (MRI) examinations. Laboratory tests have little diagnostic value for liver cysts. Usually, liver cysts do not cause abnormal liver function. However, sometimes in order to differentiate diagnosis, certain blood tests are still necessary, especially the alpha-fetoprotein (AFP) blood test, to exclude primary liver cancer.
For patients with liver cysts with clear conditions, active treatment is needed. Heilongjiang Northeast Hepatobiliary Hospital implements a non-surgical treatment method for liver cysts under CT guidance, making it easy for patients with liver cysts to free themselves from pain.
6. Dietary taboos for patients with intestinal cysts
Patients with intestinal cysts should pay attention to the following points in diet:
1. Low-fat, low-fiber. Foods with too much fat are not easy to digest, and their lubricating bowel action often worsens diarrhea symptoms, so patients should not eat fried, fried, cold, and high-fiber foods.
Food can be chosen, such as easily digestible thin noodles, stewed noodles, wontons, tender green leaves, fish, shrimp, eggs, and soybean products, so that the intestines can rest.
When there is excessive exhaust, flatulence, it is advisable to eat less sucrose and foods that are easy to produce gas and fermentation, such as potatoes, sweet potatoes, white radish, pumpkin, milk, soybeans, and so on.
4. Most patients with intestinal cysts are physically weak and have poor resistance, so they should pay more attention to dietary hygiene, avoid eating raw and cold, hard, and deteriorated food, do not drink alcohol, and do not eat spicy and strong刺激性 flavoring
Product.
5. Apples contain tannic acid and fruit acids, which have astringent and anti-diarrheal effects. They are rich in vitamin C, which improves immunity. Patients with intestinal cysts can eat them regularly.
7. Conventional methods of Western medicine for the treatment of intestinal cysts
The laboratory examination of this disease can be completely normal, and individual abnormal indicators have no diagnostic value for the disease. Preoperative ultrasound examination and CT scans, although non-specific, can help clarify the location, size, nature, and adjacency relationship with surrounding organs. X-ray barium meal examination, barium enema, and contrast examination can all understand the degree and position of the compression of the cyst on surrounding organs. Intraperitoneal intestinal cysts are rare in clinical practice, with atypical symptoms and signs, and imaging examinations are often non-specific. Laboratory examinations are also often non-specific, and they are prone to misdiagnosis as other diseases. In this case, the preoperative misdiagnosis was schwannoma.
The final diagnosis depends on pathological examination, with the following pathological characteristics: under the microscope, the cyst wall epithelium can be single-layer flat, cuboidal, columnar, or stratified flat epithelium, and some can be seen with cilia and mucus-secreting cells. CEA staining is positive, and the cyst wall is surrounded by fibrous connective tissue. In this case, the pathological examination shows that the cyst wall is composed of smooth muscle tissue, the lining of the cyst is ciliated columnar epithelium, and some mucus glands can be seen. It is consistent with the pathological characteristics of congenital intestinal cysts.
Surgical resection is the first-line treatment for this disease, and surgery should be performed regardless of whether there are symptoms. The choice of surgical method should be based on the location of the cyst. Complete resection of the cyst wall is the key to surgical cure. For those who cannot be completely resected, the residual part can be burned and destroyed by an electric knife or iodine tincture to prevent recurrence. Patients with intraperitoneal intestinal cysts need close follow-up after surgery, especially those with incomplete resection of the cyst wall. If recurrence occurs, surgery can be performed again. In this case, the mass was completely resected, and there has been no recurrence so far after follow-up.
In summary, intraperitoneal intestinal cysts are clinically rare, with atypical symptoms and signs, and imaging examinations are often non-specific. Laboratory examinations are also often non-specific, and they are prone to misdiagnosis as other diseases. Clinical diagnosis requires clinical pathological diagnosis, and complete resection is the best treatment. The choice of surgery should be based on the location of the cyst. Complete resection of the cyst wall is the key to surgical cure. For those who cannot be completely resected, the residual part can be burned and destroyed by an electric knife or iodine tincture to prevent recurrence. Patients with intraperitoneal intestinal cysts need close follow-up after surgery, especially those with incomplete resection of the cyst wall. If recurrence occurs, surgery can be performed again. In this case, the mass was completely resected, and there has been no recurrence so far after follow-up.
