Chapter: Orthopedic problems associated with vascular malformations
Article: 6 of 9
Update: Feb 24, 2021
Author(s): Kertai, Michael Amir
Scoliosis is a pathological curvature of the spine. It consists essentially of two components, first a rotation of the vertebral bodies, and secondly a lateral deviation of parts of the spine from the sagittal plane.
In a progressed case with a pronounced, progressive curvature, under the mechanical stress to which the spine is subjected, even more rapid progression of the scoliosis due to the resulting asymmetry sets up a vicious circle.
However, the correlation of the degree of scoliosis with symptoms has not been well established. Very severe scoliosis can lead to respiratory problems, but it is debatable whether mild curvatures of the spine cause back pain.
One indication for the treatment of scoliosis is therefore known as “back cosmetics”, i.e., improving the visual appearance. Although not a typical medical problem, scoliosis can be disfiguring and stigmatize affected patients accordingly.
The exact cause of the development of scoliosis has not been definitively determined. However, in connection with vascular malformations, two notable factors are prominent that can contribute to the development of scoliosis:
Secondary scoliosis may develop as a consequence of a pelvic obliquity, which in turn is due to a leg length discrepancy. It should be noted, however, that by no means every leg length discrepancy will lead to scoliosis. According to current studies, there is also no directly proportional relationship between the extent of the leg length discrepancy and the probability of developing a scoliosis. However, it is undisputed that the probability of scoliosis increases in principle from a significant (from about 2 cm) leg length difference.
On the other hand, hemihyperplasia or circumscribed overgrowth syndromes and asymmetric hyperplasia syndromes are often associated with scoliosis that may be due to asymmetric growth of the vertebral bodies, analogous to the development of leg length discrepancy. These are to be regarded as syndromic forms of scoliosis.
In CLOVES syndrome, scoliosis is considered a typical concomitant symptom and represents the “S” in the name of this disorder.
An essential diagnostic procedure that should be performed in all patients with vascular malformations of the lower extremity is a regular clinical orthopedic examination.
This is done for the spine with the patient standing and, if there is a difference in leg length, after compensating for this difference by placing wooden boards under the foot of the shorter limb.
The patient is viewed from behind, and attention is paid to possible asymmetry of the lumbar dimples, the alignment of the spine and the shoulder position, comparing the two sides. With regard to spinal alignment, particular attention should be paid to whether it is out of the perpendicular position. The easiest way to do this is to look at the prominent cervical vertebra C7 and check whether a plumb line can be dropped from this to the sacrum.
In addition, the patient is viewed from the side, where the shape of the double S-curve of the spine can be examined. As a rule, scoliosis leads to flattening of the double-S curve.
Finally, the so-called inclination test is performed, in which patients bend their back and let their arms hang down towards the floor. Now the examiner must bend his or her knees so that his or her eyes are level with the patient's spine. Thus, the examiner looks at the patient from behind and checks whether the height of the posterior torso wall is symmetric on both sides of the spine. If the torso wall is higher on one side than the other, the patient is said to have a rib or lumbar bulge (depending on the location) and thus a rotational scoliosis.
Only the detection of a rib or lumbar bulge enables the clinical diagnosis of scoliosis to be made. If the spine merely shows a deviation from the sagittal plane when the patient is standing upright, this alone is a sign of asymmetry but not of scoliosis. As a rule, if asymmetry is present in the inclination test, it is measured using a scoliometer. If it indicates an inclination of more than 5°, there is an indication for performing a spinal X-ray.
The radiograph must be taken in a standing position and usually as a posterior-anterior image of the entire spine on a composed X-ray (one image, whole spine).
Here, the Cobb angle, the rib angle, and the rotation of the vertebral bodies are assessed.
In addition, to analyze the prognosis of the future progression of the scoliosis, the expected spinal growth can be calculated with the aid of assessment of the iliac crest apophysis (so-called Risser stage). In this case, the iliac crest should additionally be imaged on the whole-spine radiograph.
In patients with vascular anomalies, diagnosis of scoliosis should usually be further evaluated by MR imaging of the spine to distinguish whether the spinal curvature is due to a vascular malformation in the spine itself (e.g., in CLOVES syndrome) or a possible leg length discrepancy. On the other hand, it may be an idiopathic scoliosis that is present in the patient independently of the vascular malformation.
In principle, the same options are considered in the treatment of patients with vascular anomalies and scoliosis as for idiopathic scoliosis. These are physiotherapy, brace treatment and surgical corrections. These are often supplemented by direct therapy of the vascular malformation.
The treatment of secondary scoliosis must, in principle, be carried out according to the same principles as those for idiopathic scoliosis.
Prophylactically, if there is an existing leg length discrepancy, this must of course be compensated for (see limb length discrepancy).
The treatment itself is essentially based on the scoliosis angle according to Cobb and the age of the patient.
Low-grade scoliosis is usually treated with special physiotherapy and exercises, while more severe cases of scoliosis are treated with a brace. Surgical therapy should be considered for Cobb angles of 50° and above.
The treatment options just mentioned are also available for cases of primary scoliosis, although their therapeutic success has not yet been demonstrated with sufficient evidence. In the case of scoliosis associated with vascular anomalies, this is due to their rarity and the varying individual degrees of involvement.
However, we know from other diseases with primary or syndromal scoliosis that non-operative measures show worse results than in idiopathic scoliosis.
In the context of vascular malformations, this is compounded by the fact that brace therapy is complicated by the sometimes painful vascular malformations.
As a rule, therefore, the treatment of such scoliosis cases is carried out on an individual basis. The willingness of the person treating the patient to tolerate certain degrees of spinal curvature without treating them will usually be greater than would be the case with idiopathic scoliosis.
In addition, the vertebral bodies themselves or their immediate surroundings are sometimes involved in the vascular malformation in terms of pathological vessels. This complicates surgical treatment of scoliosis considerably and requires prior interventional repair of these vessels (e.g., by sclerotherapy or embolization).