75Saudi Journal of Medicine & Medical Sciences | Vol. 3 | Issue 1 | January 2015 | 75-77

Large Facial Hemangioma Causing Kasabach–Merritt Syndrome, Treated with Percutaneous Endovascular Embolization

Afnan F. Almuhanna, Bandar F. Aldhafi riDepartment of Radiology, King Fahd Hospital of the University, Al Khobar, University of Dammam, Kingdom of Saudi Arabia

Correspondence: Dr. Afnan Fahd Almuhanna, P.O. Box 12432, Dammam 31473, Kingdom of Saudi Arabia. E-mail: amuhanna@uod.edu.sa

CASE REPORT

INTRODUCTION

Kasabach–Merritt Syndrome (KMS) is the association of a hemangioma, thrombocytopenia, and hypofi brinogenemia. This phenomenon was fi rst described in 1940 by Kasabach and Merritt, who took care of an infant with a giant capillary hemangioma and thrombocytopenic purpura.[1] 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.[2]

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 [Figures 2 and 3].

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.

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DOI:10.4103/1658-631X.149693

A B S T R A C T

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.

Key words: Endovascular embolization, hemangioma, Kasabach–Merritt syndrome, magnetic resonance imaging

ملخص البحث: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 طبيعياً واختفى الورم كلياً.

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Almuhanna and Aldhafi ri: Large facial hemangioma causing KMS

Saudi Journal of Medicine & Medical Sciences | Vol. 3 | Issue 1 | January 201576

Figure 1: Large right facial hemangioma. Figure 2: Magnetic resonance imaging-T2-weighted fat-suppression showing a well-defi ned large right facial mass of heterogeneous signal intensity, predominantly increase signal with multiple signal void foci.

Figure 3: Magnetic resonance imaging-T1-weighted fat-suppression post-gadolinium showed avidenhancement of the previously described right facial mass. Figure 4: Lateral angiogram of the right external carotid artery and

superfi cial temporal artery, showing a signifi cant vascular tumor blush.

Figure 5: Four arterial coils were deployed; post embolization arteriogram revealed complete occlusion of the feeding artery.

Figure 6: 1 month after embolization.

Figure 7: A year after embolization.

Patient underwent carotid angiography, selective catheterization of the right external carotid artery, with super-selective catheterization of the right superfi cial 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].

A year after therapy, platelets counts and coagulation profi le were normal and the tumor was hardly visible [Figure 7].

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Almuhanna and Aldhafi ri: Large facial hemangioma causing KMS

77Saudi Journal of Medicine & Medical Sciences | Vol. 3 | Issue 1 | January 2015

DISCUSSION

Hemangiomas are most frequently encountered vascular soft tissue abnormality.[3] They constitute 7% of oral benign soft tissue tumors.[4] They are the most common soft tissue neoplasm of infancy.[1] 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-defi ned hyperintense mass on T2- [Figure 2] weighted images because of the presence of the cavernous or cystic vascular spaces containing stagnant blood. Fluid/fl uid levels or low signal intensity areas (corresponding to fi brous tissue, fast fl ow within vessels, foci of calcifi cation, or areas thrombosis) may also be seen.[3] 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.[5]

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 fi brinolysis within the hemangioma.[3]

The pathophysiology is believed to be consumption of platelets and fi brinogen by intralesional thrombosis.[6] The lesions are typically superfi cial 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%.[6] 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.[7]

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.[8]

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.

REFERENCES1. Kasabach HH, Merritt KK. Capillary hemangioma with

extensive purpura: Report of a case. Am J Dis Child 1940; 59:1063-70.

2. Beutler E, Lichtman MA, Coll er BS, Williams WJ. Williams Hematology. 6th ed. New York: McGraw-Hill; 2001.

3. 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.

4. Allen PW, Enzinger FM. Hemangioma of skeletal muscle. An analysis of 89 cases. Cancer 1972;29:8-22.

5. 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.

6. Larsen EC, Zinkham WH, Eggleston JC, Zitelli BJ. Kasabach-Merritt syndrome: Therapeutic considerations. Pediatrics 1987; 79:971-80.

7. 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.

8. 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.

How to cite this article: Almuhanna AF, Aldhafi ri BF. Large facial hemangioma causing Kasabach-Merritt syndrome, treated with percutaneous endovascular embolization. Saudi J Med Med Sci 2015;3:75-7.

Source of Support: Nil. Confl ict of Interest: None declared.

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