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Neuroradiology Case of the Week

Case 169

Sudhir Kathuria, MD, Henry Wang, MD, PhD, Ravinder Sidhu, MD,
Loris Cedeno, MD, and Per-Lennart Westesson, MD, PhD, DDS

Clinical Presentation: A 15-year-old male presented with long history of left cheek swelling.

Radiological Findings:

Figure 1: T2-weighted MR image showing diffusely infiltrative high-signal intensity lesion in the left masticator space with involvement of the masseter and pterygoid muscle.
Figure 2: Coronal fat saturated post-Gd T1 MR image showing intense enhancement in the lesion that is extending into the infratemporal fossa superiorly and is also involving the temporalis muscle.
Figure 3: Direct contrast medium injection into the malformation before sclerotherapy shows abnormal dilated venous channels draining into left jugular vein.
Figure 4: Droplets visible after injection of ethiodol mixed with dehydrated alcohol in the vascular malformation.
Figure 5: Direct contrast medium injection before the second sclerotherapy shows significant reduction in the dilated channels compared to initial angiogram in Figure 3.

Diagnosis: Facial venous vascular malformation treated with sclerotherapy

Discussion: Hemangiomas are benign tumors seen in children that are characterized by initial growth phase due to endothelial proliferation followed by involution phase with spontaneous slow regression. They are usually not present at birth, are clinically manifested within the first month of life, and exhibit a rapid growth phase in the first year. Majority of hemangiomas spontaneously regress to near-complete resolution by 5-7 years of age. However, at times involution can take several years and is incomplete that may cause scarring, deformity, and psychological trauma.
     Vascular malformation on the other hand are made up of ectatic venous, arteriovenous, or lymphatic channels that have a normal rate of endothelial cell turnover but continue to expand with the growth of the child, and do not undergo spontaneous involution. These can be classified as either high-flow or low-flow lesions. High-flow lesions are shunting malformations such as AV-malformations and congenital or acquired AV-fistulas. Low-flow lesions include venous malformations, lymphatic malformations, and mixed lesions.
     Medical or surgical management is indicated for lesions that become cosmetically deforming or those causing functional problems due to pressure effects.  Methods of management for such vascular malformations include compression, resection, and obliteration of the channel lumens by sclerosant injection or laser photocoagulation. Low flow vascular malformations are especially responsive to percutaneous sclerosant injection, such as ethanol and detergent sclerosant drugs. The goal of sclerotherapy is to obliterate the channel lumens by causing damage to the endothelium with subsequent inflammation and fibrosis. Good to excellent results are possible in majority of cases who undergo serial sclerotherapy.  Most adverse effects are manageable, but severe complications, such as skin necrosis, permanent nerve damage, and airway obstruction can result from the intravascular administration of ethanol. It is generally recommended that the treatment of such vascular malformations be performed by practitioners with appropriate training and support.

References:

  1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformation in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982 Mar;69(3):412-22. [Medline]
  2. Buckmiller LM. Update on hemangiomas and vascular malformations. [Review 67 refs]. Curr Opin Otolaryngol Head Neck Surg. 2004 Dec;12(6):476-87. [Medline]
  3. Burrows PE, Mason KP. Percutaneous treatment of low flow vascular malformations. [Review]. J Vasc Interv Radiol. 2004 May;15(5):431-45. [Medline]
  4. Conners JJ, Wojak JC. Interventional Neuroradiology: Strategies and Practical Technique. WB Saunders, Co.,1999: 327-337.
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