Neurenteric cysts are rare, non-cancerous, fluid-filled sacs that can develop in the body. These cysts originate during early embryonic development due to an unusual connection between the primitive gut and the developing nervous system.
Understanding Neurenteric Cysts
Neurenteric cysts are congenital, meaning they are present from birth, and are characterized by a lining of cells similar to those found in the digestive tract. These cysts are considered benign formations. Their unique origin explains their name, as “neurenteric” points to an abnormal interaction between the notochord, a precursor to the spine, and the endoderm, which forms the lining of the digestive tract, during the third week of embryonic development. This abnormal separation or remnant allows endodermal cells to become misplaced near the developing nervous system.
These cysts most frequently appear along the spine, specifically within the spinal canal. They are commonly located in the cervical (neck) or thoracic (mid-back) regions, typically situated in the intradural, extramedullary space. While less common, neurenteric cysts can also form within the brain, particularly in the posterior fossa, or in other unusual locations such as the abdomen or mediastinum.
Recognizing the Signs
The symptoms associated with neurenteric cysts vary significantly, depending on the cyst’s size, exact location, and whether it is pressing on nearby structures like nerves, the spinal cord, or the brain. Symptoms can manifest at any age, from infancy through adulthood.
Common neurological symptoms include pain in the back, neck, or limbs, along with weakness, numbness, or tingling sensations. Issues with bladder and bowel control, such as incontinence or difficulty urinating, can also arise. If a cyst is located in the brain, symptoms might include headaches, seizures, or problems with cranial nerves, leading to facial weakness or double vision. In infants, a cyst in the upper spine or neck region might cause respiratory distress or feeding difficulties.
Diagnosis and Treatment Approaches
Magnetic Resonance Imaging (MRI) is the primary imaging method for identifying these cysts, as it provides clear visualization of the cyst and its relationship to surrounding neural structures. Computed Tomography (CT) scans can serve as a supplementary tool, particularly for identifying any associated bony abnormalities, which are frequently observed with spinal neurenteric cysts. Myelography can also help delineate the cyst’s margins or determine if it communicates with the cerebrospinal fluid space. These imaging techniques help differentiate neurenteric cysts from other spinal or intracranial lesions that might present similarly.
Surgical removal is the main and most effective treatment for neurenteric cysts. The primary goal of surgery is to achieve complete removal of the cyst to alleviate symptoms by decompressing affected neural structures and to prevent recurrence. However, achieving complete removal can be challenging if the cyst adheres closely to vital neurological structures, such as the spinal cord or cranial nerves. In such cases, partial removal may be performed to avoid causing neurological damage. If complete surgical removal is not feasible or only partially successful, ongoing management of symptoms may be necessary.
Long-Term Outlook
The long-term outlook for individuals with neurenteric cysts is generally favorable, particularly when complete surgical removal is achieved. This often leads to the resolution of symptoms or significant improvement in neurological function. However, recurrence of the cyst is possible, especially if the cyst capsule could not be entirely removed due to adherence to surrounding structures. Recurrence rates of up to 37% have been reported with incomplete resection.
Regular medical follow-up, including repeat MRI scans, is important to monitor for any signs of recurrence or the development of new symptoms. While surgical intervention often yields positive outcomes, there is a possibility of long-term neurological deficits if significant damage occurred due to cyst compression before treatment or if the cyst recurs and causes further compromise.