Vascular anomalies of the upper limb.

Vascular anomalies of the upper extremity are a surgical challenge to the hand surgeons. The treatment modality varies with respect to the presentation, extent of the lesion, progression and their complications. Based on our experience in treating patients with vascular malformations, a protocol has been formulated for their management, which we have found to be very useful and successful. With the use of the tumescent technique and good planning, haemangiomas are best excised in infancy or early childhood. Investigations like contrast computed tomography and magnetic resonance imaging have been found to be a useful tool in the diagnosis and planning of surgery for venous malformations. Embolisation seems to be a safe option in arteriovenous malformations.

INTRODUCTION

In 1863, Virchow[1] first introduced microscopic evaluation of vascular lesions. Rudolf Virchow, Wegener[2] and Frazer[3] attempted an anatomico pathological classification, which does not help in clinical decision making. A classification based on embryologic characteristics of the vasculature helped to differentiate haemangiomas from arteriovenous malformations.

Mulliken described haemangiomas as true tumours that present in the first 4 weeks of life. Biological classification proposed by Mulliken and Glowacki in 1975[4] was based on the cellular features, and correlated the findings with physical examination and natural history. Further studies confirm that the system of classification is also based on radiological, haemodynamic and immuno histochemical characteristics.

Haemangiomas are characterised by early presentation in infancy followed by spontaneous regression in childhood.[5] Rapid growth during the neonatal period is the hallmark of haemangioma at a rate beyond the child's growth. Haemangiomas have a preference for pretty female babies, denoting probable hormonal dependence.

Haemangiomas never appear in the later part of life in contrast to vascular malformations, which result from anomalous development of the embryonic vascular system. Vascular differentiation occurs between the fourth and 10th weeks of intrauterine life, which becomes evident and symptomatic during later development. The characteristics that distinguish haemangiomas from vascular malformation during infancy and childhood are summarised in Table 1.

Table 1

Comparative features of haemangioma and vascular malformations

MATERIALS AND METHODS

Between 1990 and 2010, we have treated 104 patients with vascular anomalies of the upper limb. Of these, 62 were females and 42 were males. Haemangiomas were seen more commonly in female children aged less than 1 year. The common complaint was a florid lesion or ulceration. We have not come across a patient presenting to us with a bleeding lesion. Primary excision with closure was performed in 20 of 30 patients. Excision and skin grafting was carried out in seven patients out of 10 as a primary procedure.

Venous malformations presented in the age group of 15–20 years. The presentation was predominantly swelling and pain restricting the activity. The most common site of the lesion was the palm of the hand. Fifty-two patients of venous malformations were seen, and 30 of them involving the hand were primarily excised. Staged excision was carried out in eight cases. Patients with arteriovenous malformations can present themselves as late as in the age group of 30–40 years. They invariably present with trophic changes in the fingers. Embolisation was carried out in eight patients only out of 12 patients. One of these patients later required shortening and closure of his left thumb following gangrene.

Eight patients with lymphangiomas affecting the fingers were seen, and all were primarily excised. Only one female child with extensive port wine stain involving the left upper limb was seen.

All these patients were routinely investigated for blood coagulation profile. Beside this, specific investigations like plain X-ray were done to look for pheliboliths, calcification and skeletal changes. Contrast computed tomography (CT) or magnetic resonance imaging (MRI) was performed to delineate the extent and plane of the lesion and for post-operative evaluation. Although angiogram was performed in the earlier cases, contrast CT proved to be a very useful tool in arriving at the diagnosis. All cases of arteriovenous malformations underwent angiogram followed by embolisation.

DISCUSSION

Port wine stains persist throughout life, with gradual change in colour. However, lasers, as a modality, have widely been acclaimed to ablate these lesions. We have no experience in this modality. Scarification, tattooing and cosmetic cover-up have all been tried with variable results.

Primarily, inactivity may be the management strategy for haemangioma elsewhere in the body. On the other hand, in the case of upper extremity haemangiomas, early excision of the lesion was found to provide a better outcome as these lesions are exposed to the risk of injury and the tendency of the children to bite, scratch and, otherwise, meddle with these lesions. With tumescent infiltration, which reduces the risk of bleeding and need for blood transfusion, tourniquet usage becomes unnecessary [Figures 1–4].

Figure 1

Haemangioma – ulcerative

Figure 4

Haemangioma – After excision

Haemangioma –- after excision under tumescent infiltration – florid lesion

Haemangioma – florid lesion – after excision

Venous malformations, [Figures 5–10] present as soft compressible swelling, and phleboliths could be palpated or seen in the X-ray, and are pathognomonic of venous malformations. Involvement of the underlying tissue can be to a varying extent. Intramuscular lesions will enlarge with muscle contractions. Pain is a characteristic feature of these lesions. The skin overlying the venous malformation may appear normal, although, more often, there is a bluish hue. Calcification of these may happen following repeated trauma with bleeding leading to dystrophic calcification of the lesion.

Figure 5

Venous malformation – clinical

Figure 10

Venous malformation – function after excision

Venous malformation – magnetic resonance imaging

Venous malformation – intraoperative

Venous malformation – excision

Use of sclerosing agents in managing these lesions leads to occlusion of the arterial inflow, often causing necrosis of the overlying skin and adjacent tissues. Intralesional sclerotherapy by the direct percutaneous route is an old modality that presently appears to have an expanding role.

Intralesional Bleomycin injection is yet another effective treatment of haemangiomas and vascular malformations. Muir et al. reported complete resolution or significant improvement in 80% of the lesions, which comprised of haemangiomas, venous malformations, cystic hygromas and lymphatic malformations.[6]

The indications for surgery are pain, swelling or deformity. Excision is the treatment of choice for localised venous malformation. Venous malformations in the hand [Figure 9,10] were mostly localized and, in one-third of the cases, involvement of the small muscles of the hand was found. Careful planning and operative procedure in the salvage of the muscle is possible. In such cases, contrast CT or MRI will be of immense help to plan the surgery.

Figure 9

Venous malformation

Staged excisions will be useful and tendon transfer could manage the functional loss. Total excision of venous anomaly is usually extensive, and surgical removal must be restrained by anatomical considerations, both aesthetic and functional. Subtotal resection always carries a risk of post-operative expansion of the remaining malformed channels, often mistaken for “recurrence”. Sometimes, subtotal resection is employed for the purpose of:

Reducing the bulk

Improving the contour

Better function

The use of magnification makes a tremendous difference to the identification and preservation of normal neurovascular structures. The use of pneumatic tourniquet and complete exanguination enable the surgeon to visualize normal and abnormal structures clearly. Tumescent infiltration technique is of immense help in achieving a bloodless field and relief of post-operative pain. With extensive malformations, staged procedures should begin distally and progress proximally.

Embolisation is the preferred method of managing arteriovenous malformations, [Figures 11–14] a double-edged weapon, and needs an experienced team. It can be repeated. It is used alone as a single modality or as a pre-operative measure to devascularise the operative field, preferably 48–72 h before surgery. Radical excision is rarely feasible.

Figure 11

Arteriovenous malformation

Figure 14

Arteriovenous Malformation – After Embolization

Arteriovenous malformation – embolisation

Lymphangiomas usually present themselves as a painless swelling and keep increasing progressively in their size. Excision is the treatment of choice [Figure 15].

Figure 15

Lymphatic Malformation

The following lesions are to be differentiated from the vascular birth marks:

Traumatic arteriovenous fistula [Figure 16]

Figure 16

Pyogenic Granuloma

Pigmented tumours

Pyogenic granulomas have a history of recent-onset, often following an injury. The discharging granulation tissue and the history will prove the diagnosis [Figures 16–18].

Figure 18

False Aneurysm – Function after excision

False Aneurysm

Traumatic arteriovenous fistula will mimic an AV malformation in its colour and signs, like transmitted pulsation bruit, etc. Distal neurovascular deficit may be present. History of injury will pinpoint the diagnosis [Figure 19].

Figure 19

Post traumatic A.V.Fistula

CONCLUSION

Vascular anomalies need not be considered as a confounding problem. The challenges in the management of vascular anomalies of the upper limb have been overcome by proper clinical examination and diagnosis. Of all the investigations, we feel that the 64-slice CT scan and MRI will pinpoint the diagnosis and help in decision making and follow-up. Non-surgical management like radiation and injection of sclerosants have limited value. Surgical management is preferred. The tumescent technique helps in providing a good avascular plane for dissection and prevention of blood loss, in addition to effective post-operative pain relief. The various treatment modalities preferred for the different types of vascular birthmarks are summaried in Table 2.

Table 2

To sum up the treatment modalities in a nut shell

Treatment is optimized by a team approach that includes a plastic surgeon, interventional radiologist and anaesthesiologist. Expertise and infrastructure availability have a bearing on the final outcome.