Peculiarities of coagulopathy in children with vascular tumors and vascular malformations

Authors

  • I. M. Benzar Національний медичний університет імені О. О. Богомольця

DOI:

https://doi.org/10.11603/2414-4533.2016.3.6806

Abstract

Coagulopathy associated with vascular malformations and vascular tumors are often mistakenly named Kasabach–Merritt syndrome although they differ on the pathogenesis, clinical manifestations and treatment options. The objective was to study the coagulation disorders in children with vascular tumors and vascular malformations. The study enrolled 41 children with vascular anomalies during 2011–2015. Patients were divided in two groups. First group enrolled 18 children with venous malformations (VM) aged from 2 month to 17 years. Second group enrolled 23 children aged from 10 days to 8 month with vascular tumor more than 10 sm2 in size. Vascular tumors are the infantile hemangiomas (IH) (78.3 %), congenital hemangiomas (13.1 %), tufted angioma (4.3 %), and kaposiform hemangioendothelioma (KHE) (4.3 %). Children with vascular tumors represent maximum clinical symptoms in age 4–5 months whereas children with VM can be symptomatically in wide age diapason from 1 month to 17 years. In children with VM the size of solitary lesion or the sum of multiple lesions size was more than 10 sm2 in 66.7 %, phleboliths are reviled in 27.8 %, elevated D-dimer levels occur in 83.3 %. Elevated D-dimer levels are associated with low fibrinogen levels in 16.5 % patients, and with phleboliths in 80.0 % patients. In children with high D-dimer levels the size of VM was more than 10 cm2. Children with IH haven’t any coagulation disorders. Mild thrombocytopenia (70–80 × 109/L) was in 8 month girl with tufted angioma, severe thrombocytopenia (6–8 × 109/L) diagnosed in newborn girl with KHE. Low-molecular-weight heparin was used to treat the coagulation disorders in children with VM. Lowering of D-dimer level was in 5–7 days of treatment start. Thrombocytopenia was diagnosed in children only of first year of life with locally aggressive tumors. Elevated of D-dimer levels was diagnosed in 83.3 % of children with VM. The increasing of coagulopathy risk in children with VM is associated with extensive (more than 10 cm2) VM and phleboliths. Low-molecular-weight heparin is effective in treatment of coagulopathy in clildren with VM.

References

ISVI-IUA consensus document diagnostic guidelines of vascular anomalies: vascular malformations and hemangiomas / B. B. Lee, P. L. Antignani, V. Baraldini [et al.] // Int. Angiol. – 2015. – Vol. 34(4). – P. 333–374.

Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach–Merritt syndrome / E. Mazoyer, O. Enjolras, C. Laurian [et al.] // Clin. Lab. Haematol. – 2002. – Vol. 24 (4). – R. 243–251.

Kasabach H. H. Capillary hemangioma with extensive purpura report of a case / H. H. Kasabach, K. K. Merritt // Am. J. Dis. Child. – 1940. – Vol. 59(5). – P.1063–1070.

Thrombocytopenic coagulopathy (Kasabach-Merritt pheno-menon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma / M. Sarkar, J. B. Mulliken, H. P. Kozakewich [et al.] // Plast. Reconstr. Surg. – 1997. – Vol. 100. – P. 1377–1386.

Association of Localized Intravascular Coagulopathy With Venous Malformations / A. Dompmartin, A.Acher, P. Thibon [et al.] // Arch. Dermatol. – 2008. – Vol. 144(7). – R. 873–877.

Dasgupta R. ISSVA classification / R. Dasgupta, S. J. Fishman // Seminars in Pediatric Surgery. – 2014. – Vol. 23. – P. 158–161.

Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies / M. Wassef, F. Blei, D. Adams [et al.] // Pediatrics. – 2015. – Vol. 136 (1). – P. e203–e215.

Lee B. B. Hemangioma and venous/vascular malformation are different as an apple and orange! / B. B. Lee, J. Laredo // Acta. Phlebol. – 2012. – Vol. 13. – R. 1–3.

Zukerberg L. R. Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis / L. R. Zukerberg, B. J. Nickoloff, S. W. Weiss // Am. J. Surg. Pathol. – 1993. – Vol. 17. – P. 321–328.

Kasabach–Merritt phenomenon: a single centre experience / C. Ryan, V. Price, P. John [et al.] // Euerop. J. of Haematol. – 2010. – Vol. 84 (2). – P. 97–104.

Vascular malformations in the extremities: emphasis on MR imaging features that guide treatment options / L. M. Fayad, T. Hazirolan, D. Bluemke [et al.] // Skeletal Radiol. – 2006. – Vol. 35. – R. 127–137.

Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas / O. Enjolras, M. Wassef, E. Mazoyer [et al.] // J. Pediatr. – 1997. – Vol. 130(4). – P. 631–640.

Weibel L. Vascular anomalies in children / L. Weibel // Vasa. – 2011. – Vol. 40. – P. 439–447.

Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals / S. E. Croteau, M. G. Liang, H. P. Kozakewich [et al.] // Pediatr. – 2013. – Vol. 162(1). – P. 142–147.

Consensusderived practice standards plan for complicated Kaposiform hemangioendothelioma / B. A. Drolet, C. C. Trenor 3rd, L. R. Brandão [et al.] // J. Pediatr. – 2013. – Vol. 163(1). – P. 285–291.

A Newborn Girl with a Large Cutaneous Lesion, Thrombocytopenia, and Anemia / J. B. Mulliken, S. Anupindi, R. A. B. Ezekowitz [et al.] // N. Engl. J. Med. – 2004. – Vol. 350. – P. 1764–1775.

Coagulation disorders in patients with venous malformation of limbs and trunk: s study in 118 patients / E. Mazoyer, O. Enjolras, A. Bisdorff [et al.] // Arch. Dermatol. – 2008. – Vol. 144 – R. 861–867.

Fishman S. J. Slow-flow vascular malformations / S. J. Fishman, A. E. Young: In Mulliken & Youngʺs Vascular Anomalies Hemangiomas and Malformations // J. B. Mulliken, P. E. Burrows., S. J. Fishman – 2-nd ed., Oxford University press, 2013. – P. 562–594.

MRI phenotypes of localized intravascular coagulopathy in venous malformations / K. S. Koo, C. F. Dowd, E. F. Mathes [et al.] // Pediatr. Radiol. – 2015. – Vol. 45(11). – P. 1690–1695.

Venous malformations of skeletal muscle / K. D. Hein, J. B. Mulliken, H. P. Kozakewich [et al.] // Plast. Reconstr. Surg. – 2002. – Vol. 110(7). – P. 1625–135.

Extensive venous/lymphatic malformations causing life-threatening haematological complications / J. Mazereeuw-Hautier, S. Syed, R. I. Leisner, J. I. Harper // Br. J. Dermatol. – 2007. – Vol. 157. – R. 558–562.

Martin L. Chronic localized intravascular coagulation complicating multifocal venous malformations / L. Martin, S. Russell, O. Wargon // Austral. J of Dermatol. – 2009. – Vol. 50 – P. 276–280.

Published

2016-11-02

How to Cite

Benzar, I. M. (2016). Peculiarities of coagulopathy in children with vascular tumors and vascular malformations. Hospital Surgery. Journal Named by L.Ya. Kovalchuk, (3). https://doi.org/10.11603/2414-4533.2016.3.6806

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