Sir,

Congenital vascular malformations are a heterogeneous group of lesions that may cause significant morbidity in patients. In 2018, the International Society for the Study of Vascular Anomalies had differentiated between vascular tumors and malformations, vascular tumors dominated by hemangiomas, which are proliferations of endothelial cells, and vascular malformations, which are hypo or hyperplastic vascular dysplasias, which include capillary, venous lymphatic and arteriovenous malformations [1].

Herein, we report a two-year-old child from a consanguineous marriage with a well-attended pregnancy and a medical delivery by vaginal route who presented, since the age of nine months, a swelling on the left lower limb, which had been progressively increasing in size. On clinical examination, we noted a tumor extending from the lower third of the left thigh to the left foot, with a slightly bluish surface (Fig.1a) and on the external surface of the left knee an agglomeration of vesicles with both hemorrhagic and translucent content (Fig. 1b), with a lagoon-like pattern on dermoscopy made up of hemorrhagic lagoons, an erythematous background and short linear vessels (Fig. 2). The patient benefited from Doppler ultrasound imaging and MRI, which revealed the presence of an extensive venous malformation in the left lower limb. Then, a diagnosis of a venous malformation of the left lower limb associated with a superficial microcystic lymphatic malformation of the same limb was established. The decision in consultation with the interventional radiologist was to put the patient under symptomatic treatment based on compression and antiplatelet agents since the patient was not eligible for surgery or sclerotherapy, and to perform ablative laser for the superficial microcystic lymphatic malformation.

	Figure 1: (a) Tumefaction of the left lower limb. (b) Erythematous plate on the external face of the thigh.
	Figure 2: Dermoscopic aspect in a lagoon-like pattern of hemorrhagic lagoons, an erythematous background, and short linear vessels.

Venous malformations are soft tissue masses resulting from congenital disruption of normal venous development. Inappropriate communication between aberrant venous channels creates these lesions, which tend to expand through progressive accumulation of static blood and decreased vascular elasticity. The connections of the normal vessels to the venous malformations supply blood that is trapped in the network of dysplastic veins, ultimately creating a lesion of abnormal veins with ill-defined boundaries that infiltrate normal tissue. Over time, slow dilation of the veins and progressive vascular ectasia occur. If left untreated, venous malformations continually develop, form thrombi, become painful, and interfere with normal function and aesthetics [2]. Lymphatic malformations are rare, slow-flowing, congenital malformations consisting of abnormal cystic dilatations, and although congenital, they are not always apparent at birth. Approx. 90% of lesions are diagnosed before the age of five and may be subject to inflammatory flare-ups in response to local infectious or traumatic events. A distinction is made between superficial and deep microcystic lymphatic malformations and macrocystic lymphatic malformations [3].

Medical treatment of venous malformation is limited to the prevention of venous stasis, vascular expansion, and thrombus formation. Local intravascular coagulation (LIC) is present in venous malformation and causes acute or chronic pain due to local thrombus and phlebolith formation. Elevated D-dimer levels and reduced fibrinogen levels are often found in these patients and may be a diagnostic biomarker for VD [4].

Medical treatment of venous malformations should be initiated at the first onset of pain. For extensive venous malformations localized to the limbs, grade 2 elastic compression is considered the most effective treatment. This external compression may be considered a replacement therapy, which compensates for the lack of smooth muscle in the walls of the abnormal veins. By reducing the tension on the venous malformation, the pain is rapidly reduced. In addition, this restraint prevents blood stagnation in the venous lakes, which may reduce the potential risk of phlebothrombosis that may cause pain. The frequency, duration, and timing of wearing this support depends on the patient’s symptoms. If the pain is felt only during exercise (sports), the brace should be worn only then. If the patient experiences pain during prolonged standing or sitting, he or she is advised to wear the brace all day upon rising. Finally, for large and intramuscular venous malformations that may cause pain at the end of the night and upon awakening, nighttime wear of the support may be advised. For children, the support must be custom-made and modified according to their growth. It is important to ensure that the entire deformity is properly compressed to avoid painful swelling downstream of the bandage, which may lead to treatment failure. Compression is contraindicated during acute episodes of phlebothrombosis, which cause a highly painful inflammatory reaction for about ten days and require the administration of nonsteroidal anti-inflammatory drugs or even aspirin or low molecular weight heparin to thin the blood. Painful venous defects associated with localized intravascular coagulation (LIC) with high levels of D-dimer may be relieved by daily injections of subcutaneous low molecular weight heparin (LMWH). Some people may experience consistent relief after as little as one month of treatment. However, others may require several months of treatment to avoid a recurrence of pain. For the latter, other treatments may be considered to reduce the size of the lesion and alleviate symptoms [5].

There are numerous therapeutic modalities that have been reported for superficial microcystic lymphatic malformations, and these include surgery, sclerotherapy, electrocoagulation, and ablative laser [6].

REFERENCES

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2.  Fowell C, Verea Linares C, Jones R, Nishikawa H, Monaghan A. Venous malformations of the head and neck:Current concepts in management. Br J Oral Maxillofac Surg. 2017;55:3-9.

3.  Lorette G, Vaillant L, Wiersbicka E, Herbreteau D. [Localized cystic lymphatic malformation (or cutaneous lymphangiomas)]. Presse Med. 2010;39:1309-14.

4.  Richter GT, Braswell L. Management of venous malformations. Facial Plast Surg. 2012;28:603-10.

5.  Boon LM, Vanwijck R. [Medical and surgical treatment of venous malformations]. Ann Chir Plast Esthet. 2006;51:403-11.

6.  Puri N. Treatment options of lymphangioma circumscriptum. Indian Dermatol Online J. 2015;6:293-4.