Vascular Amputation in Children
FRANCIS J. TROST, MD
When one thinks of vascular amputees, the elderly person with arteriosclerosis or diabetes most frequently comes to mind. Although they are still small in number compared to adults, it has been our impression that we have seen pediatric vascular amputees more frequently over the past several years. We questioned the reason for this apparent increase in amputations in children due primarily to vascular causes and reviewed eight of these children. It is worthwhile to call attention to this problem because the literature does not seem to have any large series and the problem may be preventable or at least treatable if approached with a high index of suspicion and prompt, aggressive treatment.
Martini is given credit for reporting the first child amputated for vascular causes in 1828. Weller reported the first case of gangrene in the upper extremity in 1899.
Since then, the literature has been confined to periodic reports of this phenomenon due to varying causes and to articles reviewing treatment or advocating a particular diagnostic or therapeutic approach.
Many causes for limb ischemia have been documented, based on case reports. Maternal diabetes and premature rupture of the membranes producing a "dry" labor have been cited as predisposing causes. More recently arterial thrombosis and embolism have been seen most frequently among infants cared for in an intensive care nursery where venous puncture, arterial puncture, angiography and indwelling catheters are commonly used.
Abnormal fetal position, arterial occlusion associated with the closure of the ductus arteriosus or the umbilical artery, developmental abnormalities of the arterial walls, thrombocytopenia and polycythemia producing hypercoagulability, sepsis, congenital heart disease, disseminated intravascular coagulation, and inadvertent intravascular injections have all been indicated as causes of dysvascular amputation in newborns and children. The actual definitive cause of this phenomenon, however, remains somewhat obscure.
Mucocutaneous lymph node syndrome (Kawasaki disease), necrotizing facsciitis, and purpura fulminans must be differentiated from these other causes because they run a more violent course with higher mortality and require prompt vigorous treatment somewhat different from the treatment of the previously mentioned entities.
The diagnosis, therefore, is made by having a high index of suspicion especially in children at risk to develop these problems or in an otherwise healthy child who suddenly develops a pulseless cyanotic limb.
Treatment until the 1960's was primarily supportive. It consisted of adequate hydration, close clinical monitoring and measures to prevent sepsis and further trauma to the extremity. Other measures, such as warming of the extremity, anticoagulation, vasodilators, hyperbaric oxygen and lumbar sympathetic blocks, have all been tried with varying results.
The 1960's saw something of a shift toward more aggressive treatment of this problem. In 1963, Sapin and associates described transumbilical angiography in the newborn. Also in 1963, Fogarty and associates reported the development of the balloon catheter thrombectomy. In the future thrombolytic drugs, laser treatment, and bypass surgery may play a part in the treatment of this condition.
Angiography can confirm the diagnosis, demonstrate the level of the occlusion and indicate the degree of collateral blood supply to the extremity. It should be performed to determine the site of the occlusion, the possible etiology of the occlusion (intraluminal thrombosis versus external compression) and whenever operative intervention is anticipated.
Surgical intervention should be considered whenever there are severe or progressive ischemic changes despite adequate conservative care or in the presence of a functional motor defect. Once the decision to operate has been made, it should be carried out promptly and without delay since procrastination compromises the eventual result.
Because of the size of the children and the vessels involved, technical problems with appropriately small instruments and with finding surgeons who have an expertise in pediatric, vascular and microsurgery can influence the outcome.
Finally, amputation may be necessary but should be delayed as long as possible, unless complications (such as sepsis) develop, to allow the part to revascularize as much as possible and therefore limit the amount ablated. Ultimately, survival of the limb depends on the reversal or correction of the process and the development of collateral circulation.
We studied ten children who had undergone amputation because of vascular problems. None were amputated or received primary care at our hospital. The five girls and five boys were all amputated at infancy or early childhood. Four had arterial or venous lines installed for various reasons. The sites included umbilical, antecubital and femoral vessels. One child had a steroid injection which may have thrombosed the femoral and iliac arteries; this patient had a family history of hypercoagulability. Another patient being treated for bums developed a staphylococcal infection and endocarditis with mitral insufficiency which led to a valve replacement; he developed thromboembolism of the femoral artery. Another was born with gangrenous changes in both lower limbs; the child, a twin, had twin-to-twin exchange transfusion. The other twin died. One patient was the product of a difficult pregnancy with right shoulder dystocia, traumatic delivery, and maternal spotting, diabetes of pregnancy, and post partum seizures. In one child a vascular thrombosis of the right arm developed, the cause of which was unknown. One child had hemophilic influenza, meningitis and septicemia and probable septic emboli.
The vascular insults were accompanied by numerous other medical problems including prematurity, intestinal perforation, retrolental fibroplasia, cardiac defects, and laryngeal stenosis.
Ultimately, the children underwent three upper limb and six lower limb amputations. One child had bilateral amputations. There was nothing remarkable about the amputations and they seemed to have ablated where they demarcated.
Prosthetic fitting was relatively routine. Children did not develop any particular prosthetic complications or problems with the residual limb.
Initial treatment was difficult to judge because it was not generally well documented in our charts. Surgery was not very successful in preventing amputation in these cases, but the timing of surgery and the indications were not apparent from the records. In one case, fasciotomies were done which have not been of much benefit in treating this problem.
In summary, we have taken a look at the interesting, but uncommon, problem of amputation in newborns and children due to vascular causes. This was prompted by the clinical impression that this phenomenon has become more frequent in recent years.
The history, etiology, treatment, and prognosis have been reviewed and we have taken a closer look at ten of our patients who have undergone amputation for this reason. We are suspicious of distressed births, children with predisposing problems or any otherwise healthy infant or child who suddenly develops the signs of vascular compromise. We perform angiography and surgery for any child who develops a progressive problem despite adequate supportive treatment or who develops functional motor complications. Having made a decision to treat the problem surgically we feel this should be done promptly and without further delay.
New technology in vascular surgery may offer an improved prognosis for these problems.
Shriners Hospital for Crippled Children, 2025 East River Road, Minneapolis, MN 55414
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