Lower-Extremity Amputations For Unusual Causes

William E. Gazeley, M.D. Carl A. Paulsen, M.D.


By a strange coincidence recent issues of the Inter-Clinic Information Bulletin have dealt rather extensively with amputations deriving from a number of unusual circumstances. In the March 1969 issue, King, Marks, and Canaan4 described limb losses following meningococcemia in several cases and diarrhea in one. In May 1969, Hagen, Matthews, and Nixon3 reported a case of quadrimembral amputations as an aftermath of chicken pox; and in the July 1969 issue, Clark, Hoyt, and Fitzsimmons1 discussed another possible case of meningococcemia leading to gangrene and amputation.

The present article seeks to contribute to this growing literature on unusual amputation etiology by describing three cases seen at the Schenectady Child Amputee Clinic.

The first patient probably represents a case of diffuse deep vascular hamartomata of the extremities as described by Goidanich and Campanacci 2. These authors report that the condition is more common in females than in males.

The second case represents an instance of Volkmann's ischemic paralysis of the lower extremity. This condition is apparently and fortunately most uncommon in English-speaking countries; at least no similar case of apparent complete cessation of distal tibial endochondral growth following such an occurrence was discovered in the English literature.

The third case may represent a result of vascular insufficiency following vessel damage by venipuncture. However, hypercoagulability of blood with thromboembolic complications associated with prolonged large doses of corticosteroids has been described in the case. The administration of corticosteroids may have increased the potential for the type of vascular catastrophe that occurred to this child.

The case histories of these three children follow:

Case #1

R.W. was an 11 1/2-year-old boy first seen in the Amputation Clinic on June 21, 1967. A diagnosis of multiple arteriovenous shunts, cystic hygroma, and multiple hemangiomas of the whole leg was made. At birth, a diffuse swelling of the left leg and foot had been noted and at seven months of age the child was admitted to a medical center for evaluation. Surgery was deferred until he was 17 months of age. An arteriogram at that time showed multiple arteriovenous shunts.

Surgery

The surgical procedure that was undertaken was interrupted by a cardiac arrest. Successful resuscitation followed open chest cardiac massage.

At the age of two years and six months, further surgery was done without incident. Innumerable surgical procedures followed: these included excision of arteriovenous shunts, amputation of the second toe because of osteomyelitis, removal of the skin and subcutaneous tissue of the dorsum of the foot and replacement with split-thickness skin grafts, and removal of cystic hygroma.

Following these procedures, the patient's condition was described as congenital arteriovenous fistula of the left leg, with multiple cutaneous hemangiomas, several giant subcutaneous cavernous hemangiomas, a swollen left foot with stiff ankle, and multiple neurofibromas.

A later arteriogram failed to reveal the presence of arteriovenous fistula. The foot was enormous and the patient walked on his heel. The hemangiomatosis extended from the mid-thigh to the foot. There were several giant subcutaneous cavernous hemangiomas in the lateral aspect of the thigh, knee, and lateral calf. The foot was edematous and bulky.

The patient's cardiac evaluation revealed no evidence of organic heart disease. X-rays showed generalized decalcification and atrophy of the bones of the leg with 1.8 cm shortening. The psychological evaluation revealed a verbal I.Q. of 129 on the Qtis test indicating a superior intelligence. The boy was somewhat resigned to the prospect of losing his leg at this time.

The leg was amputated on November 14, 1967, and the boy's rehabilitation has presented no problems. A photograph of the foot prior to surgery is shown in Fig. 1 . The pathological diagnosis was that of extensive arteriovenous hygromas involving the thigh, lower leg, and foot.

Considerable discussion had revolved about the level of amputation. One large center had advised a hip-disarticulation; a mid-thigh amputation was finally done.

Case #2

This boy (K.B.) was ten years of age when admitted from another hospital for treatment of an open fracture of the right tibia and fibula in the middle third of the leg. He had been struck by an automobile and had been treated by debridement of his wound and the application of a plaster cylinder. Large doses of narcotics had failed to relieve his pain and he was, therefore, referred to this hospital. He continued to have severe pain until his cast was opened shortly after being admitted here. The leg was found to have little sensation below the fracture site and there was an evident ischemic paralysis. To close the wound itself required several split-thickness grafts and pedical grafts. Even though several full-thickness bone grafts were used, the union was not secure when the leg was finally amputated ten years later.

As time passed the leg, especially the segment below the fracture site, failed to grow and after ten years the foot was found to have markedly impaired sensation. The leg was 8 cm shorter than the left and the foot was several sizes smaller.

At this stage, a below-knee amputation was done. It is felt that this cessation of growth was an unusual sequela to a Volkmann's ischemic paralysis.

Case #3

C.S. is a 13-year-old female who was in good health until February 1960, when she developed albuminuria associated with the rapid onset of generalized edema following an upper-respiratory infection. In March 1960, the diagnosis of lipoid nephrosis was made at a nearby university medical center. A dramatic diuresis followed treatment with corticosteroids. Despite this diuresis she continued to demonstrate proteinuria and the edema worsened, even though the administration of the corticosteroids was maintained.

In June 1960, the girl was readmitted to the same center. Femoral venipuncture was used for admission laboratory studies and several days later acute right femoral artery blockade developed; endarterectomy and vascular prosthesis insertion were necessary. Gangrene of the foot and lower leg developed despite this treatment and on June 28, 1960, a below-knee amputation was performed and she was referred to this institution for rehabilitation.

She was successfully fitted with a patellar-tendon-bearing prosthesis and continues to utilize this device with an excellent gait. No treatment with corticosteroids has been necessary for the past six years. The etiology of the acute vascular insufficiency was not definitely established.

Summary

Three cases of unusual indications for lower-extremity amputation in children are presented. Included is a case of diffuse deep vascular hamartomata, a case of cessation of limb growth following Volkmann's ischemic paralysis, and a case possibly representing thromboembolism following corticosteroid therapy.

William E. Gazeley, M.D. and Carl A. Paulsen, M.D. are associated with the Sunnyview Hospital and Rehabilitation Center Schenectady, New York

References:
1. Clark, R.; Hoyt, W.Ak; and Fitzsimmons, L.: "Immediate Postsurgical Fitting of Prostheses for Bilateral Amputations," Inter-Clin. Information Bull., 8:1-8, July 1969. 
2. Goidanich, J.F., and Campanacci, M.: "Vascular Hamartomata and Infantile Angioectatic Osteohyperplasia of the Extremities. A Study of Ninety-four Cases," J. Bone and Joint Surg., 44-A:815-8A2, July 1962. 
3. Hagen, R.; Matthews, J.G.; and Nixon, J. J.: "Varicella Gangrenosa," Inter-Clin. Information Bull., 8:9-19, May 1969. 
4. King, R.E.; Marks, T.W.; and Canaan, R.: "Meningococcemia and Diarrhea With Gangrene," Inter-Clin. Information Bull., 8:1-8, Mar. 1969.