Pediatric Kasabach-Merritt Syndrome Treatment & Management

  • Author: Alexandra C Cheerva, MD, MS; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 1, 2012
 

Medical Care

  • Selected patients who have Kasabach-Merritt syndrome (KMS) with absent or mild thrombocytopenia and coagulopathy may be evaluated on an outpatient basis. However, as hemangiomas grow, these infants often require inpatient evaluation and treatment because most develop significant thrombocytopenia and disseminated intravascular coagulation (DIC).
  • Kasabach-Merritt syndrome management involves hastening hemangioma regression, interfering with platelet trapping within the lesion, and supporting the patient with transfusions.
  • Embolization, corticosteroids and alpha interferon have been successfully used in many patients.[16, 17, 18, 19, 20] Interferon alfa has been associated with neurologic problems in some patients.[21, 22]
  • Other medications, including pentoxifylline and dipyridamole have been reported to be beneficial in treatment of Kasabach-Merritt syndrome.[22, 23]
  • Chemotherapy, including vincristine, cyclophosphamide, and actinomycin D, has been used successfully in some patients.[24, 25, 26, 27, 28]
  • Treatment with intermittent pneumatic compression, either alone or in combination with other therapies, has helped some patients.[29] Compression is most useful when the hemangioma is located on an extremity. Encircle the hemangioma with a blood pressure cuff and gradually increase pressure to midway between the systolic and diastolic blood pressures. The cuff is intermittently inflated (ie, 90 s compression, 30 s rest). A cuff can be used for an extended period. Compression with surgical hose stockings may help. Compression treatment may be a helpful modality before attempting other potentially more toxic treatments.
  • Radiation therapy is indicated in patients with severe disease.[13, 30] Radiation has also been used with interferon therapy with some success.[31] Although low radiation doses usually have been used with some success, carefully weigh the long-term adverse effects of radiotherapy in very young children (eg, risk of malignancy, decreased bone growth in the radiated field) against the problems caused by the hemangioma.
  • Recently, propranolol has reportedly been used in two infants with severe hemangiomas.[32] Potential explanations of the therapeutic effect include vasoconstriction, decreased expression of VEGF and bFGF genes, and the triggering of apoptosis of capillary endothelial cells.
Next

Surgical Care

  • Complete surgical resection, when possible, is the most effective treatment of vascular lesions complicated by Kasabach-Merritt syndrome.[33] Thrombocytopenia and DIC resolve after the lesion is removed.
  • Depending on the location of the tumor, general surgery, thoracic surgery, or neurosurgery may be needed for excision or ligation of the vessels feeding the hemangioma. Wide local excision is recommended but may be difficult. The large size and infiltrative growth pattern of the vascular lesion may cause complications (eg, hemorrhage, obstruction, respiratory compromise).
  • Amputation may be necessary for intractable lesions involving a limb.
  • Some lesions are surgically inaccessible and require nonsurgical treatment modalities.
  • Interventional radiologic procedures use polyvinyl alcohol or absolute ethanol to embolize/sclerose the vessels of the hemangioma.[20, 34] Another technique involves injecting polyvinyl beads to stop feeder blood supply.
  • Treatment with the tunable dye laser (TDL) may help patients with diffuse cutaneous hemangiomas.[35]
Previous
Next

Consultations

  • Pediatric hematologists are needed to manage the complex hemostatic problems of patients with Kasabach-Merritt syndrome and to administer and manage many of the medications needed for the most fulminant cases.
  • Consultations with neonatal or pediatric intensive care specialists may be needed, based on the age of the patient.
Previous
Next

Diet

  • No special diet is required.
  • Depending on the medical conditions, infants may require IV nutrition.
Previous
Next

Activity

  • The location of the hemangioma and patient tolerance determine activity restrictions.
Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Alexandra C Cheerva, MD, MS  Associate Professor of Pediatrics, Division of Hematology/Oncology, Director of Pediatric Blood and Marrow Transplantation, University of Louisville School of Medicine; Attending Staff, Section of Pediatric Hematology and Oncology, Kosair Children's Hospital

Alexandra C Cheerva, MD, MS is a member of the following medical societies: American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, International Pediatric Transplant Association, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Salvatore Bertolone, MD  Director, Division of Pediatric Hematology/Oncology, Department of Pediatrics, Kosair Children's Hospital; Professor, University of Louisville School of Medicine

Salvatore Bertolone, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Education, American Association of Blood Banks, American Cancer Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Ashok B Raj, MD  Associate Professor, Section of Pediatric Hematology and Oncology, Department of Pediatrics, Kosair Children's Hospital, University of Louisville School of Medicine

Ashok B Raj, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Kentucky Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary R Jones, MD  Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Carlos Suarez, MD, to the development and writing of this article.

References

  1. Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child. 1940;59:1063-70.

  2. Kelly M. Kasabach-Merritt phenomenon. Pediatr Clin North Am. Oct 2010;57(5):1085-9. [Medline].

  3. Hatley RM, Sabio H, Howell CG, Flickinger F, Parrish RA. Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon. J Pediatr Surg. Oct 1993;28(10):1356-7; discussion 1358-9. [Medline].

  4. Martin MC, Harrington H, Wong WW. Massive congenital kaposiform hemangioendothelioma of the eyelid in a neonate. J Craniofac Surg. Nov 2011;22(6):e38-41. [Medline].

  5. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. Apr 1997;130(4):631-40. [Medline].

  6. Zukerberg LR, Nickoloff RJ, Weiss SW. Kaposiform Hemangioendothelioma of Infancy and Childhood. American Journal of Surgical Pathology. 1993;17(4):321-328.

  7. Szlachetka DM. Kasabach-Merritt syndrome: a case review. Neonatal Netw. Feb 1998;17(1):7-15. [Medline].

  8. Payne LG, Hayward CPM, Kelton JG. Destruction of red cells by the vasculature and reticuloendothelial system. In: Hematology of Infancy and Childhood. 1998:523-43.

  9. Berman B, Lim H. Concurrent cutaneous and hepatic hemangiomata in infancy: report of a case and a review of the literature. J Dermatol Surg Oncol. Nov 1978;4(11):869-73. [Medline].

  10. Arunachalam P, Kumar VR, Swathi D. Kasabach-Merritt syndrome with large cutaneous vascular tumors. J Indian Assoc Pediatr Surg. Jan 2012;17(1):33-6. [Medline]. [Full Text].

  11. Enjolras O, Riche MC, Merland JJ, Escande JP. Management of alarming hemangiomas in infancy: a review of 25 cases. Pediatrics. Apr 1990;85(4):491-8. [Medline].

  12. Tang JY, Chen J, Pan C, Yin MZ, Zhu M. Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child. World J Pediatr. Aug 2008;4(3):227-30. [Medline].

  13. Mitsuhashi N, Furuta M, Sakurai H, et al. Outcome of radiation therapy for patients with Kasabach-Merritt syndrome. Int J Radiat Oncol Biol Phys. Sep 1 1997;39(2):467-73. [Medline].

  14. Pampin C, Devillers A, Treguier C, et al. Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia. J Pediatr Hematol Oncol. May-Jun 2000;22(3):256-8. [Medline].

  15. Powell Julie. Update on hemangiomas and vascular malformations. Current Opinion in Pediatrics. 1999;11:457-463.

  16. Akyuz C, Emir S, Buyukpamukcu M, et al. Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome. Arch Dis Child. Jan 2003;88(1):67-8. [Medline]. [Full Text].

  17. Argenta LC, Bishop E, Cho KJ, et al. Complete resolution of life-threatening hemangioma by embolization and corticosteroids. Plast Reconstr Surg. Dec 1982;70(6):739-44. [Medline].

  18. Biban P. Kasabach-Merritt syndrome and interferon alpha: still a controversial issue. Arch Dis Child. Jul 2003;88(7):645-6. [Medline]. [Full Text].

  19. Ezekowitz RA, Mulliken JB, Folkman J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med. May 28 1992;326(22):1456-63. [Medline].

  20. Stanley P, Gomperts E, Woolley MM. Kasabach-Merritt syndrome treated by therapeutic embolization with polyvinyl alcohol. Am J Pediatr Hematol Oncol. Winter 1986;8(4):308-11. [Medline].

  21. Michaud AP, Bauman NM, Burke DK, et al. Spastic diplegia and other motor disturbances in infants receiving interferon-alpha. Laryngoscope. Jul 2004;114(7):1231-6. [Medline].

  22. de la Hunt MN. Kasabach-Merritt syndrome: dangers of interferon and successful treatment with pentoxifylline. J Pediatr Surg. Jan 2006;41(1):e29-31. [Medline].

  23. Wananukul S, Nuchprayoon I, Seksarn P. Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon. Int J Dermatol. Sep 2003;42(9):741-8. [Medline].

  24. Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol. Aug-Sep 2002;24(6):459-62. [Medline].

  25. Hu B, Lachman R, Phillips J, et al. Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D. J Pediatr Hematol Oncol. Nov-Dec 1998;20(6):567-9. [Medline].

  26. Hurvitz SA, Hurvitz CH, Sloninsky L, Sanford MC. Successful treatment with cyclophosphamide of life-threatening diffuse hemangiomatosis involving the liver. J Pediatr Hematol Oncol. Nov-Dec 2000;22(6):527-32. [Medline].

  27. Perez Payarols J, Pardo Masferrer J, Gomez Bellvert C. Treatment of life-threatening infantile hemangiomas with vincristine. N Engl J Med. Jul 6 1995;333(1):69. [Medline].

  28. Fernandez-Pineda I, Lopez-Gutierrez JC, Ramirez G, Marquez C. Vincristine-ticlopidine-aspirin: an effective therapy in children with Kasabach-Merritt phenomenon associated with vascular tumors. Pediatr Hematol Oncol. Nov 2010;27(8):641-5. [Medline].

  29. Aylett SE, Williams AF, Bevan DH, Holmes SJ. The Kasabach-Merritt syndrome: treatment with intermittent pneumatic compression. Arch Dis Child. Jul 1990;65(7):790-1. [Medline].

  30. Schild SE, Buskirk SJ, Frick LM, Cupps RE. Radiotherapy for large symptomatic hemangiomas. Int J Radiat Oncol Biol Phys. Aug 1991;21(3):729-35. [Medline].

  31. Hesselmann S, Micke O, Marquardt T, et al. Case report: Kasabach-Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha. Br J Radiol. Feb 2002;75(890):180-4. [Medline].

  32. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taieb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. Jun 12 2008;358(24):2649-51. [Medline].

  33. George M, Singhal V, Sharma V, Nopper AJ. Successful surgical excision of a complex vascular lesion in an infant with Kasabach-Merritt syndrome. Pediatr Dermatol. Jul-Aug 2002;19(4):340-4. [Medline].

  34. Yang YH, Lee PI, Lin KH, Tsang YM. Absolute ethanol embolotherapy for hemangioma with Kasabach-Merritt syndrome. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. Jan-Feb 1998;39(1):51-4. [Medline].

  35. Poetke M, Philipp C, Berlien HP. Flashlamp-pumped pulsed dye laser for hemangiomas in infancy: treatment of superficial vs mixed hemangiomas. Arch Dermatol. May 2000;136(5):628-32. [Medline].

  36. Ram SP. Kasabach-Merritt syndrome and Down's syndrome. J R Soc Med. Mar 1997;90(3):159-60. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.