|Year : 2015 | Volume
| Issue : 1 | Page : 75-77
Large facial hemangioma causing kasabach-merritt syndrome, treated with percutaneous endovascular embolization
Afnan F Almuhanna, Bandar F Aldhafiri
Department of Radiology, King Fahd Hospital of the University, Al Khobar, University of Dammam, Saudi Arabia
|Date of Web Publication||20-Jan-2015|
Afnan F Almuhanna
P.O. Box 12432, Dammam 31473
This is a case of an infant that had a large right facial hemangioma and subsequently developed thrombocytopenia. He was admitted to the intensive care unit, then transferred to the interventional radiology for further management. He was successfully treated with endovascular embolization.
تعنى هذه الحالة بطفل رضيع يعاني من ورم كبير في الجهة اليمنى من الوجه أدى إلى نقص في الصفائح الدموية. ادخل المريض العناية المركزة وتم تشخيص حالته عن طريق الرنين المغناطيسي والوعائي، كورم وعائي دموي ومن ثم تم علاجه في قسم الأشعة بنجاح بواسطة الانصمام وبعد عام من العلاج أصبح عدد الصفائح طبيعيًا واختفى الورم كليًا.
Keywords: Endovascular embolization, hemangioma, Kasabach-Merritt syndrome, magnetic resonance imaging
|How to cite this article:|
Almuhanna AF, Aldhafiri BF. Large facial hemangioma causing kasabach-merritt syndrome, treated with percutaneous endovascular embolization. Saudi J Med Med Sci 2015;3:75-7
|How to cite this URL:|
Almuhanna AF, Aldhafiri BF. Large facial hemangioma causing kasabach-merritt syndrome, treated with percutaneous endovascular embolization. Saudi J Med Med Sci [serial online] 2015 [cited 2019 Jun 20];3:75-7. Available from: http://www.sjmms.net/text.asp?2015/3/1/75/149693
| Introduction|| |
Kasabach-Merritt Syndrome (KMS) is the association of a hemangioma, thrombocytopenia, and hypofibrinogenemia. This phenomenon was first described in 1940 by Kasabach and Merritt, who took care of an infant with a giant capillary hemangioma and thrombocytopenic purpura.  KMS is a rare disorder that can affect infants from the time of birth, or may appear later in infancy as the vascular malformation grows. Diagnosis of KMS is made based on the constellation of a vascular lesion, thrombocytopenia, consumptive coagulopathy and microangiopathic hemolytic anemia. Unlike true capillary hemangiomas that regress in childhood and are a cosmetic nuisance, the lesions in KMS are distinctive vascular tumors, that include tufted angiomas and kaposiform hemangioendotheliomas. 
| Case report|| |
A 1-month old neonate was found to have facial bluish mass measuring 5 cm × 8 cm on the right side of the face extending over the cheek at lateral side of the neck and over his eye preventing its opening and causing high risk of amybliopia [Figure 1]. Magnetic Resonance Imaging (MRI)/Magnetic Resonance Angiography (MRA) were performed and revealed a large hemangioma on right side of the face, extending to the upper neck with a big feeder vessel from the external carotid artery with no extension to the brain [Figure 2] and [Figure 3].
|Figure 2: Magnetic resonance imaging-T2-weighted fat-suppression showing a well-defined large right facial mass of heterogeneous signal intensity, predominantly increase signal with multiple signal void foci.|
Click here to view
|Figure 3: Magnetic resonance imaging-T1-weighted fat-suppression post-gadolinium showed avidenhancement of the previously described right facial mass.|
Click here to view
Patient was intubated and admitted to Surgical Intensive Care Unit and he received 4 units of packed Red Blood Cells, 2 units of Fresh Frozen Plasma, 2 units of Platelets and one cryoprecipitate. Patient was kept on methylprednisolone 2.5 mg and Vitamin K 1 mg intravenously for 1 month and Gamma IV Immunoglobulin 1.6 g every 2 weeks for 2 months. Later patient developed thrombocytopenia (platelet count was 6000).
The diagnosis of KMS was made based on the constellation of a vascular lesion, thrombocytopenia and consumptive coagulopathy. Patient was referred to interventional radiology for further management.
Patient underwent carotid angiography, selective catheterization of the right external carotid artery, with super-selective catheterization of the right superficial temporal artery [Figure 4] and embolization of the feeder artery with four coils [Figure 5]. The tumor started to regress in size slowly within few days after embolization [Figure 6].
|Figure 4: Lateral angiogram of the right external carotid artery and superficial temporal artery, showing a significant vascular tumor blush.|
Click here to view
|Figure 5: Four arterial coils were deployed; post embolization arteriogram revealed complete occlusion of the feeding artery.|
Click here to view
A year after therapy, platelets counts and coagulation profile were normal and the tumor was hardly visible [Figure 7].
| Discussion|| |
Hemangiomas are most frequently encountered vascular soft tissue abnormality.  They constitute 7% of oral benign soft tissue tumors.  They are the most common soft tissue neoplasm of infancy.  Hemangiomas arise in a variety of locations, including the skin, subcutaneous tissue, muscle and synovium.
Magnetic resonance imaging is the modality of choice for demonstrating the relationship between hemangioma and the adjacent anatomic structures. Hemangiomas appear as well-defined hyperintense mass on T2- [Figure 2] weighted images because of the presence of the cavernous or cystic vascular spaces containing stagnant blood. Fluid/fluid levels or low signal intensity areas (corresponding to fibrous tissue, fast flow within vessels, foci of calcification, or areas thrombosis) may also be seen.  On T1-weighted images, the lesions display a signal intensity intermediate between that of muscle and fat. Dynamic post contrast MRI can help differentiating hemangioma from other vascular malformations and to distinguish between low- and high-perfusion lesions. 
KSM syndrome is an uncommon complication of large hemangiomas, in which there is thrombocytopenia and purpura. It is a coagulopathy consisting of intravascular coagulation, clotting, and fibrinolysis within the hemangioma. 
The pathophysiology is believed to be consumption of platelets and fibrinogen by intralesional thrombosis.  The lesions are typically superficial and solitary, but may involve internal structures such as the liver. Cardiac failure may result from high-volume arteriovenous shunting. Shock, intracranial bleeding, or other internal hemorrhages may result in mortality rates as high as 30%.  Our patient had infantile KMS marked by multiple complications.
The treatment objective of KMS is to prevent bleeding from the consumptive coagulopathy. KSM syndrome shows wide variation in its response to different treatment modalities. 
Currently, there are no known treatment guidelines. Different interventions are recommended including compression, embolization, use of interferon and steroids, laser therapy, sclerotherapy, chemotherapy, radiation or surgery. 
The treating physician must decide the most suitable treatment to achieve maximum involution of the lesion and preservation of organ function and should consider the available facilities.
| Conclusion|| |
KSM syndrome is not an uncommon complication of large hemangioma for which, different therapeutic options are available. The use of embolization, seems to be safe and very effective, resulting in hematological cure and involution; it should be considered early in the course of the disease in preparation for excision.
| References|| |
Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: Report of a case. Am J Dis Child 1940; 59:1063-70.
Beutler E, Lichtman MA, Coller BS, Williams WJ. Williams Hematology. 6 th
ed. New York: McGraw-Hill; 2001.
Vilanova JC, Barceló J, Smirniotopoulos JG, Pérez-Andrés R, Villalón M, Miró J, et al.
Hemangioma from head to toe: MR imaging with pathologic correlation. Radiographics 2004;24:367-85.
Allen PW, Enzinger FM. Hemangioma of skeletal muscle. An analysis of 89 cases. Cancer 1972;29:8-22.
Teo EL, Strouse PJ, Hernandez RJ. MR imaging differentiation of soft-tissue hemangiomas from malignant soft-tissue masses. AJR Am J Roentgenol 2000;174:1623-8.
Larsen EC, Zinkham WH, Eggleston JC, Zitelli BJ. Kasabach-Merritt syndrome: Therapeutic considerations. Pediatrics 1987; 79:971-80.
Balaci E, Sumner TE, Auringer ST, Cox TD. Diffuse neonatal hemangiomatosis with extensive involvement of the brain and cervical spinal cord. Pediatr Radiol 1999;29:441-3.
Abass K, Saad H, Kherala M, Abd-Elsayed AA. Successful treatment of kasabach-merritt syndrome with vincristine and surgery: a case report and review of literature. Cases J 2008;1:9.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
|This article has been cited by|
||Successful transarterial embolization with cellulose porous beads for occipital haemangioma in an infant with Kasabach-Merritt syndrome
| ||Zaw Aung Khant,Toshinori Hirai,Osamu Ikeda,Eiji Furukoji,Yoshihito Kadota,Minako Azuma,Norihiro Shinkawa,Keiji Kitatani,Yoichi Mizutani,Kimihiko Endo,Yasuyuki Yamashita |
| ||BJR|case reports. 2017; : 20170004 |
|[Pubmed] | [DOI]|