When diastematomyelia is a malformation of the spinal canal, which consists in affected patients from birth. Diastematomyelia is very rare and manifests itself as a longitudinal division of some sections of the spinal cord. Diastematomyelia belongs to the category of dysraphias.
What is diastematomyelia?
The disease term diastematomyelia is derived from the Greek and is composed of the words for back and space. In diastematomyelia, a spur or septum cuts through the medulla of the back, creating two areas. The spur is made up of a substance that resembles bone and cartilage.
These structures divide the spinal canal lengthways into two areas. The division extends over at least one vertebral body, but often a larger number is affected. Diastematomyelia is a congenital malformation of the back and was scientifically described for the first time in 1827 by the physician Oliver. The similar concept of disease sometimes leads to a confusion between diastematomyelia and diplomyelia.
However, this is a disease in which the medulla of the back is sometimes doubled. In 1992, the doctor Pang and his colleagues proposed to designate diastematomyelia and diplomyelia with a uniform disease term, the so-called ‘split cord malformation’. Pang divides the disease into two types. Split cord type 1 has two dura tubes that are not connected to each other. In type 2 patients have a single dural tube.
Causes
Diastematomyelia is a congenital disease of the spinal cord. The exact causes for the formation of diastematomyelia are currently not yet known. Genetic causes may be responsible for the development of diastematomyelia in the patient. In addition, spontaneous malformations during pregnancy in the growing embryos are possible.
Due to the congenital malformation, the spinal cord is doubled in diastematomyelia. This makes the disease one of the dysraphic disorders. The two systems for the spinal cord are usually not identical and have separate skins. A septum or spur separates the spinal cord in the spinal canal. The spur usually arises from the posterior side of the vertebral bodies.
Symptoms, ailments & signs
Diastematomyelia is characterized by a typical combination of three symptoms. Patients with diastematomyelia suffer from scoliosis, have skin changes in the area above the spine and show abnormalities in the lower limbs. In the majority of cases, there is a special hair layer on the skin above the spine.
In addition, pigment nevi, a dermal sinus, hemangiomatosis, lipomas and pilonidal sinuses often occur in this area. In some patients, neurological changes develop in the feet as a result of diastematomyelia. The spine sometimes bends and the nerves usually fail on a single leg.
Diastematomyelia only rarely manifests itself for the first time in adult patients. Instead, the typical symptoms of diastematomyelia often develop in people at a young age. If the spinal cord is asymmetrical, neurological deficits can occur on the side of the body that is less developed. The corresponding deficits then occur regularly.
The patients with diastematomyelia also suffer from scoliosis, which is the result of incorrect posture and malformations. The medulla of the back is locally fixed, so that impairments arise on the ascensus during the growth phase. As a result, the typical neurological and orthopedic symptoms of diastematomyelia develop.
Diagnosis
The diagnosis of diastematomyelia is usually made by an orthopedic surgeon, who first takes a medical history with the patient regarding the symptoms and medical history. After the consultation with the patient, the specialist examines the physical complaints using a visual examination and various examination-technical procedures. By default, he uses x-ray methods to depict the spine.
The doctor focuses on the roots of the arches, vertebral bodies and the vertebral arch and examines the areas for abnormalities. In doing so, the specialist often discovers the bony spur that causes diastematomyelia. In order to show the changes in the spinal cord even more precisely, the doctor uses an MRI scan. This means that the cartilaginous structures in the area of the spine can also be clearly illustrated.
With the help of a myelography and a CT scan, the dural tube is clearly visible. In principle, diastematomyelia can be diagnosed prenatally using sonography. The same test method can also be used to detect diastematomyelia in newborn children and babies. The differential diagnosis of diastematomyelia excludes tethered cord syndrome and syringomyelia.
When should you go to the doctor?
If the symptoms of diastematomyelia cause increasing discomfort, a doctor should be consulted. If, for example, neurological changes in the feet or a curvature of the spine are noticed, a doctor must be called in. The same applies to sensory disorders and paralysis in the limbs. In general, the following applies: in the event of unusual symptoms that gradually seem to worsen, the responsible doctor should be informed.
Diastematomyelia usually progresses slowly and causes a wide variety of complaints that require clarification. In particular, bad posture and deformities that appear to appear overnight must be clarified – either by the family doctor or a specialist for the respective symptom. In addition, a therapist should be called in.
Patients who also suffer psychologically from the increasing physical stress must have this treated in any case. If a fall occurs due to a bad posture or sudden discomfort, the emergency services must be alerted. A longer hospital stay is then usually indicated, during which the symptoms of diastematomyelia are clarified again.
Treatment & Therapy
There are many options for treating diastematomyelia nowadays. The conservative therapeutic approach focuses on physiotherapeutic care of the patient with regular physiotherapy sessions. As part of the symptomatic treatment of diastematomyelia, spasmolytic therapy is also possible.
Medicines from the group of spasmolytics increase the tone of the muscles and thus support patients with diastematomyelia. Surgery for diastematomyelia is necessary if tethered cord syndrome is present.
Outlook & forecast
Diastematomyelia leads to various unpleasant complaints that can significantly limit the patient’s everyday life. For this reason, treatment is necessary in every case.
If there is no treatment for diastematomyelia, those affected suffer from various neurological deficits and continue to suffer from skin changes. Extreme hairiness on the skin can also occur. In some cases, the disease also leads to malformations, whereby the symptoms can worsen without treatment.
The child’s development is slowed down and restricted. Furthermore, the malformations can lead to bullying or teasing, which can lead to psychological complaints.
The individual complaints can usually be treated well, so that the diastematomyelia can be completely limited. The affected person is dependent on therapies and various exercises. In some cases, however, the use of medication or surgery is necessary.
However, a complete healing is also aimed for, which can usually also be achieved. There are no particular complications. Correct treatment of diastematomyelia also does not have a negative impact on the life expectancy of those affected.
Prevention
Diastematomyelia is congenital, which is why it is currently not possible to effectively prevent the disease. However, diastematomyelia can already be diagnosed by sonography in embryos in the womb.
Aftercare
In most cases of diastematomyelia, the patient has no direct follow-up measures available. The person concerned is first of all dependent on a full examination and treatment by a doctor in order to alleviate further symptoms and avoid complications. In the worst case, this leads to paralysis that can no longer be treated.
Since it is also a congenital disease, it cannot be treated causally. In most cases, diastematomyelia is treated by means of physiotherapy. The patients are dependent on regular exercises, whereby physiotherapy can also be carried out. In many cases, the exercises from these therapies can also be carried out in your own home, so that healing is accelerated somewhat.
Surgical interventions can sometimes be necessary in diastematomyelia. After such a procedure, the patient should rest and take care of the body. Exertion and physical strain are to be avoided in any case. Care and support from friends and family also have a positive effect on the course of diastematomyelia. Usually, diastematomyelia does not reduce the life expectancy of those affected.
You can do that yourself
In any case, diastematomyelia requires a comprehensive medical evaluation. However, those affected can do a few things themselves to alleviate the symptoms and ensure a speedy recovery.
First of all, a physiotherapeutic care should be taken. The physiotherapy can be at home supported by regular exercise and individual exercises. Basically, a healthy lifestyle with exercise, a balanced diet and avoidance of stress is recommended.
These measures reduce the discomfort and improve well-being and quality of life. Both of these are often limited in diastematomyelia, which can lead to mental illnesses later on. That is why the primary goal of self-therapy is to preserve the quality of life and at the same time to alleviate the individual symptoms.
If neurological changes occur, you must speak to your family doctor or a neurologist. Moles and other typical skin changes, if these are perceived as annoying, are best dealt with using appropriate clothing or cosmetic products.
Surgical intervention is also possible, but due to the risks it should not be done until the more severe symptoms have been treated. Finally, talk to relatives, friends or other people affected about the disease. An open approach makes a lot of things easier and often also gives a new perspective on life.
