Introduction
An uncontrollable bleeding case of a 22-year-old male patient was admitted by emergency. Before admission, the patient with a 22-year history of inborn cavernous hemangioma had undergone regional hemangioma resection of the left foot, but bleeding of the anastomotic stoma could not be controlled successfully after operation. The cavernous hemangioma involves the proximal and distal left lower limb as well as the perineal region, which presents with increasing swelling, pain, and bluish discoloration of overlying skin and superficial dilated veins (Fig. 1). These lesions or symptoms vary based on body position changes, particularly, lesions will be enlarged in erect position and reduced in decubitus.
The patient received hormone therapy, which worked well in the early childhood but failed several years later. No other special history was documented.
Hematological examination showed severe anemia, hypofibrinogenemia, and prolonged prothrombin time (PT) as well as activated partial thromboplastin time (APTT).
Magnetic resonance (MR) was used to detect and determine the location and extent of the cavernous hemangioma. The MR result indicated that cavernous hemangioma extensively invaded the muscular tissue (intramuscular hemangioma) of both lower limb (Fig. 2a) and perineal region (Fig. 2b). Magnetic resonance angiography (MRA) demonstrated that no abnormal gross vessels were found in the arterial phase (Fig. 2c), but considerable gross vessels and varicose small saphenous vein were observed in the postarterial phase and venous phase (Fig. 2d).
We failed to cease bleeding and heal the wound with symptomatic treatment (including rectification of anemia and abnormal clotting mechanism, and pressure therapy) and supportive care because of extensive surgical excision of hemangioma tissue that resulted in necrosis. Amputation was required to save the patient’s life, but selection of amputation plane as well as surgical approach became a challenging task because of extensive intramuscular lesions.
After scrupulous discussion, combination of superficial femoral artery ligation and supergenual thigh amputation was performed based on the location and extent of the lesions of the left lower limb. According to the MR result, muscles of supergenual thigh (1/3 of distal region to thigh) were less damaged by hemangioma.
Surgical techniques
The majority of the procedures were performed under general anesthesia. The patient lay in the supine position. Infra-inguinal incision was applied to expose the femoral artery and its branches prior to the amputation. We ligated the femoral artery and its branches with silk thread at the level of 1 cm below their junction and then closed the incision. After that, a fish-mouth incision of the supergenual thigh with equal anterior and posterior skin flaps was used. The skin and deep fascia were dissected off the shaft of femur. The distal ends of the femoral artery and vein were individually suture ligated. The femoral nerve was transected sharply under tension. The bone edges were smoothed with a rasp. The fascia and skin were closed in separate layers. In the course of amputation, every visible hemorrhagic spots, both artery derived and vein derived, was ligated strictly, although suture knots with high density scattered below the stoma of amputation.
Outcome
In this patient, pathological section examination for the removed lesions showed typical blood vessels and vascular sinusoids of hemangioma, which allowed classification of the lesion as a mixed (primarily cavernous) soft-tissue hemangioma (Fig. 3).
No massive bleeding occurred in both intraoperative and postoperative amputation, but complications of amputation wound disruption and delayed union appeared because of suture reaction. For skin transplantation that contributes to accelerating wound healing, which was refused by the patient, the stoma of amputation was completely closed and healed by 1 month dressing change (Fig. 4). Two-year follow-up revealed no other complications.
Discussion
Hemangiomas are benign vascular tumors, most often seen at birth or infancy. The incidence of these tumors in the extremities varies from 4.9% to 28.5% [
1]. They can be diffuse or localized, diffuse being more common [
2]. They do not spontaneously involute and may therefore require surgical intervention. Histologically, hemangiomas are subdivided into five categories, depending on the predominant type of vascular channel identified. These subdivisions include capillary, cavernous, arteriovenous, venous, and mixed variations [
3,
4]. Cavernous hemangiomas are composed of dilated, blood-filled spaces lined by flattened endothelium [
5,
6].
Intramuscular hemangiomas are uncommon, accounting for 0.8% of all benign vascular tumors. Shortly after birth, some grow rapidly, out of proportion to the growth of the child and often at a very alarming rate [
7]. When they are close to the skin surface, they are characteristically bluish in appearance and demonstrate a soft, nonpulsatile consistency. They can be actively emptied by manual compression or passively by elevation of the affected part. Most commonly, the age at presentation is 20-30 years, but patients may present earlier and have a wide anatomical distribution. It most commonly occurs in the extremities, and all patients present with a growing body position-related palpable mass, which may or may not be accompanied by pain or may present as pain without a mass [
8].
The appropriate imaging and consultations should be performed to arrive at the correct diagnosis and management, accordingly. For the diagnosis of intramuscular hemangioma, plain radiographs, MR, angiography, and positron emission tomography (PET) may be helpful. There has been concern in the past that MR imaging was not good at distinguishing edema from blood; however, as technology and imaging have been improved, MR imaging is now considered the modality of choice.
Optimal treatment is the complete surgical excision of hemangioma with a surrounding margin of the normal muscle based on rectification of abnormalities of the hematological system, which may occur in diffuse cavernous hemangioma [
9]. Incomplete removal almost always results in recurrence. Compression sclerotherapy using sclerosing agents has also been suggested as a treatment and may be useful in diffuse lesions that may not be excisable by surgery [
10]. As technological advancements allow easier and safer access to remote lesions, embolization is being used more frequently to treat difficult lesions [
11,
12]. Also, endovascular treatment is suggested to be an important component of the interdisciplinary management of hemangiomas [
13].
To avoid significant blood loss in perioperative period, surgery may be preceded by embolotherapy or sclerotherapy. Sclerotherapy in combination with surgical resection has shown excellent palliative results and is widely regarded as the preferred mode of therapy [
14].
In this case, we aimed at minimize blood loss by means of superior femoral artery ligation, which is a safe and effective treatment modality [
15-
18]. This case demonstrates that surgical measures even local resection for severe diffuse cavernous hemangioma of the limbs should be considered seriously to avoid uncontrollable bleeding that may endanger patients’ lives.
Preoperative blood tests and MR should be applied to determine if abnormal clotting mechanism exists and to identify the location and extent of the cavernous hemangioma [
19,
20], which is a factor in deciding on the amputation plane as well as the surgical approach. Femoral artery ligation should be adopted prior to performing amputation, in which every visible hemorrhagic spot, both artery derived and vein derived, was ligated strictly, aiming at minimizing blood loss, although delayed union may occur.
In conclusion, hemangioma of the limb is often difficult to manage. The role of surgery and intervention in current management is still not clear [
21]. In case of uncontrollable bleeding of severe diffuse cavernous hemangioma of the lower limbs, combination of superficial femoral artery ligation and supergenual thigh amputation is an effective and safe choice.
Higher Education Press and Springer-Verlag Berlin Heidelberg
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