1Abeer Faisal Aldhawi, 1Mohammed Hussain Almetlag, 1Saeed Bin Nihayah
1King Fahd Medical City
In 1982 Mulliken and Glowacki suggested a classification of vascular abnormalities based on endothelial properties into hemangioma and vascular malformations basis on clinical and histopathological features (1). Vascular malformations are always present at birth, whereas hemangiomas frequently appear within the first month of life and are not commonly discovered at birth. Furthermore, vascular malformations expand in proportion to the baby growth and never diminish on their own. (2). Congenital vascular malformation remains a complicated and perplexing diagnostic and clinical entity due to the vast diversification of clinical manifestations, site, unexpected clinical course, variable response to therapy, and a high chance of recurrence. Besides that, the lack of standard nomenclature makes differentiation difficult (3, 4). Venous malformations are the the most prevalent type of vascular abnormality in children and young adults that appear as solitary lesion 90 % with most prevalent n in the head and neck around 40 – 48 % (5-6-7).Besides slow growth, with time the venous malformation appears as low flow, sluggish development, localized or diffuse appearance, and compressible lesion (8). A multidisciplinary team must undertake a diagnostic and treatment plan (9). Diagnostic and interventional radiologists play an important part in a vascular anomaly team, they must be informed of the clinical history and findings to make the correct diagnosis and provide the appropriate treatment alternatives (10). Ultrasound scan is non-invasive, low-cost, and freely accessible, so it demonstrates that venous malformations are compressible, display mixture echotexture in comparison to adjoining subcutaneous tissue, and Doppler in addition to grey scale is important in distinguishing between low – and high flow malformations. (11).CT rule is restricted; it aids in the definition of bone involvement and phlebitis if present (12). T2-weighted or STAIR images in MRI provide a high signal intensity and best define the entire extent of the lesion and its relationship to neighbouring essential structures. Post gadolinium, will rise to enhance homogeneously or heterogeneously. (13). For a long time, surgical excision has been the preferred treatment strategy for congenital lymphatic and vascular malformations currently sclerotherapy is a preferred first-line treatment for venous malformation, it can reduce the size or eliminate the lesion (14). A variety of pharmaceuticals have been used to treat venous malformations,the most widely utilized agents are ethanol, bleomycin, and sodium tetracyclic sulfate (STS) (2). Sodium tetracyclic sulfate has been broadly utilized in the past for the sclerosis of oesophagal varices and varicose veins, and it is also being used more frequently in the therapy of vascular malformations, it has been observed to be effective in eliminating channels of VMs (15). Bleomycin is an anticancer agent; however, some recent attempts have been made to use it as a sclerosing agent in patients with congenital lymphatic malformations (16). Complications are rare included skin blistering, ulceration and nerve injury (17).We present the case of a 3 -year-old boy who had a lip and cheek venous malformation that delayed diagnosis and was effectively treated with sclerotherapy.
Material(s) and Method(s):
A male child, 3.5 years old baby, during infancy period the parents observed painless swelling at the right upper lip, upper gingival area, and right buccal areas accompanied with discolouration which increased with time and became noticeable with crying and fever. They went to primary care and were referred to a pediatric clinic because of the swelling and discolouration otherwise he is medically free and had no family history of vascularmalformation. The systemic examination was unremarkable, at that time his lesion was clinically diagnosed as a hemangioma. An ultrasound performed at the site of the local swelling revealed a 3.3 x 3 x 2.2 cm lesion mislabeled as hemangioma (Figure 1). The patient was seen by dermatologists, and an MRI revealed that the lesion extended from tthe lateral aspect of the right upper lip into the submucosal oral cavity; the ipsilateral orbital region was intact, and the lesion again was misdiagnosed as a hemangioma (Figure 2).At the age of two years and a half, the swelling and discolouration were still present with increase facial deformity associated with discomfort felling (Figure 3A) then the patient was evaluated by a multidisciplinary team consisting of a plastic surgeon, dermatologist, paediatrician, pediatric oncologist, and pediatric interventional radiologist.A pediatric interventional radiologist performed an ultrasound that revealed venous malformation and agreed to start sclerotherapy (Figure 4). After explaining the treatment plan to the patient’s parents and obtaining written consent, the patient had three sclerotherapy treatments under general anaesthesia and sterile technique. All sessions were carried out with the aid of ultrasonography and fluoroscopic guidance.During the first sclerotherapy session in November 2018, the right cheek and upper lip venous malformations were punctured at six different locations then perform a venogram.Sclerotherapy was administered via three catheters and 13 cc of 3 % sodium tetradecyl sulphate (Figure 5A). The second session took place in March 2019 and were using 2 ml of sodium tetradecyl sulphate foam and 1.5 cc (1.5 mg) of bleomycin (Figure 5b). The third session was held in August2019 with a total of 3 ml of 3 % sodium tetradecyl sulphate foam and 1 ml bleomycin (Figure5C), with the same three locations used in the first session.
The duration between sessions was not ideal, however, owing to a bed crisis and admission difficulties, the time between sessions was prolonged.The patient endured all the sessions with no acute or long-term complications. Both the swelling and symptoms are fade over time after sclerotherapy. The family and clinicians were satisfied with the results in both medical and cosmetic aspects . The last ultrasound obtained after the third session shows a considerable reduction in venous malformation (Figure 6)
Sclerotherapy treatment was found to be an excellent option as a first-line treatment in terms ofsize reduction as well as safety profile. Follow-up ultrasound showed a significant reduction in size and with a minor remnant venousmalformation, the symptoms vanished totally, even with triggersThe diagnosis and differentiation of vascular malformations may overlap; this case highlightsthe importance of a multidisciplinary team in establishing an accurate diagnosis and selecting the best treatment strategy, as well as the importance of general radiologists being familiar with the various types of vascular malformations to minimize misinterpretation