A Case of Intractable Left Forearm Congenital Arteriovenous Fistula Ending with Amputation: Importance of New Medical Information Obtained via the Internet

Abstract

Objective: The aim of the present study was to consider the importance of medical information obtained via the Internet for difficult cases in hospitals, especially in those located in rural areas. We report here a case of congenital arteriovenous fistula (AVF) in the upper extremities.

Patient: A 30-year-old lady was transported to our hospital by ambulance due to massive bleeding in her left hand. She was seen by our current cardiovascular surgery team for the first time, although she had been diagnosed with congenital AVF of the left arm 9 years previously. Because it was asymptomatic, she was followed up by observation. During 5 years of observation, symptoms such as cyanosis, pain, and refractory ulcers gradually developed. When she was 26 years old, she was referred to a university hospital in Akita, but surgery had already been judged to be impossible. When she was 30 years old, traumatic bleeding in her left hand and hemorrhagic shock led her to be taken to our hospital by ambulance. Using the Internet, we found an institution that had treated a large number of cases of AVF. After controlling the bleeding, we referred her to that institution. However, she could not be treated without an above-elbow amputation.

Conclusion: Congenital AVF in the upper extremities is a rare vascular anomaly and has been generally accepted to be an extremely difficult disease to treat. Treatment should be started as early as possible before the presence of any symptoms. When a specialist is not available near the hospital, precise information must be found using the Internet and the patient should be referred without any delay.

Introduction

Congenital arteriovenous fistula (AVF) represents one of the most difficult diseases to diagnose and treat despite medical advances. AVF was first described in early 1758 by William Hunter¹⁾. It is characterized by an abnormal communication between an artery and a vein, which might be congenital in origin or even acquired²⁾. It is a rare anomaly that may occur anywhere in the body. Those in the upper limb account for 30–60% of all cases³⁾. They can be found incidentally without any symptoms or show multiple symptoms such as compression of the adjacent nerves, life-threatening hemorrhage, high output heart failure, ischemic necrosis or even psychiatric problems. Computed tomography (CT), Doppler ultrasound (US) imaging, magnetic resonance imaging (MRI), and angiography are used to evaluate the disease⁴⁾. In most cases, conservative treatment works for a while, but surgery should be considered during the asymptomatic period. Complete removal of an AVF has been demonstrated to be the only way for a radical cure⁵⁾. Because surgery techniques are difficult and surgery alone might result in life-threatening bleeding during the operation, most surgeons recommend that embolo/sclerotherapy also be used⁶⁾. As AVF is a rare and difficult-to-treat disease, the number of specialists is quite limited. The main aim of this report was to discuss how to diagnose and treat a difficult case like AVF in a hospital located in a rural area, far from institutions in which specialists work.

Patient

A 30-year-old lady was transferred to our hospital by ambulance due to continuous massive bleeding in her left hand. Our current cardiovascular team saw her in the emergency room for the first time. When she was 21 years old, she had had a thorough examination because of a palpable mass and vascular murmur in her left forearm found incidentally in a health examination. She was diagnosed with congenital AVF. Because it was asymptomatic at that time, she was followed up in our outpatient clinic. When she was 24 years old, symptoms of cyanosis and pain in the second finger of her left hand developed. The cardiovascular surgery team at that time prescribed analgesics for pain control but did not consider surgery. Thereafter, some other symptoms developed gradually, such as severe pain and recurrent formation of ulcers (Fig. 1). For further examination and extensive treatments, she was referred to a university hospital in Akita. After thorough examinations, comprehensive discussions were conducted with plastic surgeons for any possibility of surgical treatment of her limb. Although the precise reason is unknown now, they concluded that surgery was already impossible and that there was no other option but to continue observation and conservative treatments at the university hospital. After that, symptoms such as necrosis of fingers even developed gradually. After admission this time, she recovered from hemorrhagic shock after repeated blood transfusion. Hemostasis was achieved by local compression. (Fig. 2). Her AVF was reassessed by angiography, CT, and MRI (Fig. 3). Fortunately, a high-speed Internet system was already available despite the very rural location of our hospital at that time (December 2007). We found a surgeon with experience treating AVF through his review article⁷⁾. We referred the patient to this expert for a second opinion via e-mail, but his office was 1000 km from our hospital. After discussion with her and the surgeon, we sent her to a hospital at which one of the surgeon’s colleagues was working. It was a 90-min trip by airplane for her to the hospital. She had two surgeries to try to salvage her forearm. Unfortunately, she had to have a third surgery to amputate her arm above the elbow. She recovered well, and her mental condition is stable now that she no longer experiences severe pain and fear of lethal bleeding.

Figure 1.

Photo taken 4 years after her forearm AVF was found. Cyanosis and recurrent formation of ulcers with severe pain developed (2004).

Figure 2.

Photo taken 9 years after her forearm AVF was found. All of her left fingers already showed severe necrosis (2007).

Figure 3.

Her forearm AVF was reassessed by angiography (upper panel), MRI, and CT (lower panel) after hemostasis was achieved (2007).

Discussion

AVF in the extremities displays various clinical presentations and an unpredictable clinical course. Certain conditions such as trauma, hormonal effects, or incomplete treatment can lead to explosive growth. Massive bleeding and wide tissue necrosis are life threatening. Although diagnostic techniques now give us precise information, treatment of AVF in the extremities is still limited and has a high recurrence rate. Surgical eradication is considered to be the key treatment method³⁾. Catheterization and embolization comprise a valuable therapy for AVF and should be used as the primary technique for treatment in most cases⁸⁾. The optimum treatment strategy involves surgical resection, transcatheter embolization, or a combination of the two^{9, 10)}. However, because AVF is relatively rare, there were very few specialists in Japan.

Treatment of an AVF in an extremity should be considered when the lesion is not symptomatic yet, such as when there is no ischemia, compression of adjacent structures, bleeding, or heart failure. It is essential not to lose the best treatment timing; otherwise, amputation is the only choice. Our case obviously missed the appropriate timing for surgery. When we saw her the first time, all of her left fingers were already showing necrosis (Fig. 2). We checked her medical records but could not find any record of consultation of any expert beyond the borders of our prefecture. One of the reasons for the delay of surgery was the lack of new medical information and excessive trust/dependency on the university hospital. Information quality depends on development of communication networks such as optical fiber networks for high-speed Internet. She should have had appropriate surgery by 2002 (Fig. 1), but we could find no trace of any searches for medical knowledge via Internet in her medical record in 2002. A high-speed Internet system became available at our hospital in 2003. It is possible that the cardiovascular surgery team at that time did not know how to find and get new medical articles or had no idea how to consult an expert via the Internet. During conservative follow-up at the university hospital, however, reassessment of any possibility of surgery should have been considered. The precise reason for this irresponsible follow-up is unknown. Follow-up by multiple teams, lack of communication among teams, and lack of a responsible person commanding teams may have contributed to the delay of surgery. In our hospital, we now have a comfortable system for searching of new medical articles and can download most medical journals. Even in a very rural area, we can not excuse a lack of knowledge concerning new medical information. We can learn good lessons from this case. New medical knowledge is absolutely necessary for daily clinical practice. It is now possible to ask an expert for a second opinion via the Internet when we encounter a difficult case.