Chapter: Orthopedic problems associated with vascular malformations
Article: 8 of 9
Update: Feb 24, 2021
Author(s): Kertai, Michael Amir
Patients with vascular anomalies should in principle be assessed as chronic pain patients. Their complaints should be evaluated in this context.
For example, pain in the feet may be indicative of an abnormal load due to an existing contracture, thrombophlebitis in the area of the vascular anomaly, or complex regional pain syndrome (CRPS). It is often a combination of all the factors.
This must always be evaluated individually and the goal of surgical therapy must be determined together with the patient, since a definitive cure is not possible in most cases.
Furthermore, the affected body part is often viewed as inferior by the patient. This means in the area of the legs, for example, that surgical interventions to the affected extremity are usually approved, but the same intervention on the “healthy” side is rejected. This can become relevant in the case of leg length discrepancy if the unaffected side has to be slowed down in its growth.
Before surgical intervention in the area of a vascular anomaly, the possibilities of interventional therapy should be fully exhausted in order to minimize the risk of bleeding.
Likewise, diagnostics and history with regard to a possible coagulation imbalance must be analyzed, as many patients with large-volume venous malformations have consumptive coagulopathy and LIC or DIC.
The use of a tourniquet is recommended on the extremities. Surgical hemostasis, as is usually performed, is difficult or futile, especially in the case of venous and capillary malformations. In such cases, it is advisable to perform the operation as a bloodless procedure with coagulation of the largest atypical vessels. Bleeding after opening of the tourniquet can be stopped by compression bandages. In some cases, clot-supporting drugs that can be introduced into the wound are used. These should be kept on hand in any case.
Most patients with vascular anomalies wear compression garments on the affected extremities. These often cannot be used in the initial postoperative phase due to pain and swelling after the procedure with an increase in circumference. Alternatively, elastic bandages can be used temporarily to prevent thrombophlebitis, thrombosis and edema. All patients, regardless of age, require postoperative anticoagulation until full mobility is regained.
Treating patients with vascular anomalies can be difficult and frustrating. Complications are much more common than in the usual patient population and complete recovery can rarely be achieved.
When initiating therapy, one must be prepared for a long course, sometimes with repetition and modification of procedures. Pre- and postoperative planning, diagnostics and therapy are complex. Intraoperatively it can be very demanding as well. A Z-shaped Achilles tendon lengthening is normally pure routine for an orthopedic surgeon; however, if the Achilles tendon is surrounded by dysplastic vessels, some of which exceed the diameter of the tendon itself, it is no longer a routine procedure.
On the other hand, these patients often encounter ignorance and unwillingness during their visits to the doctor, so they are grateful to meet a surgeon who takes care of their problem and are usually happy to go the long and often winding road with such a surgeon.