Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:435 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size

Table of Contents   
Year : 2015  |  Volume : 20  |  Issue : 4  |  Page : 194-196

Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in a neonate of life- and limb-threatening nature: A case report

1 Department of Paediatric Surgery, Kanchi Kamakoti Childs Trust Hospital, Nungambakkam, Chennai, Tamil Nadu, India
2 Department of Dermatology, Kanchi Kamakoti Childs Trust Hospital, Nungambakkam, Chennai, Tamil Nadu, India
3 Department of Paediatrics, Kanchi Kamakoti Childs Trust Hospital, Nungambakkam, Chennai, Tamil Nadu, India

Date of Web Publication2-Sep-2015

Correspondence Address:
Dr. Namasivayam Selvarajan
Department of Paediatric Surgery, Kanchi Kamakoti Childs Trust Hospital, 12A Nageswara Road, Nungambakkam, Chennai - 600 030, Tamil Nadu
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.154671

Rights and Permissions



Kaposiform hemangioendothelioma involving whole of a leg in a neonate with Kasabach-Merritt phenomenon causing limb and life-threatening situation has not been reported. One such case and its successful management is presented in this case report. Literature review is made.

Keywords: Infant-Newborn, Kaposiform hemangioendothelioma, Kasabach-merritt phenomenon, vincristine

How to cite this article:
Subash A, Senthil GK, Ramamoorthy R, Appasamy A, Selvarajan N. Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in a neonate of life- and limb-threatening nature: A case report. J Indian Assoc Pediatr Surg 2015;20:194-6

How to cite this URL:
Subash A, Senthil GK, Ramamoorthy R, Appasamy A, Selvarajan N. Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in a neonate of life- and limb-threatening nature: A case report. J Indian Assoc Pediatr Surg [serial online] 2015 [cited 2023 Feb 1];20:194-6. Available from: https://www.jiaps.com/text.asp?2015/20/4/194/154671

   Introduction Top

Kaposiform Hemangioendothelioma (KHE) is a rare congenital vascular tumor arising from vascular endothelial cell lining and 60% of it occurs in neonates. Kasabach-Merritt phenomenon (KMP) includes thrombocytopenia, microangiopathic hemolytic anemia and mild consumptive coagulopathy, and occurs in KHE, Kaposiform lymphatic anomaly (KLA) and tufted angioma (TA). KHE with KMP has a high mortality rate of up to 50%. [1],[2] Although complete excision is the treatment of choice, due to its size, site and permeation of multiple tissue planes, KHE can rarely be resected safely. Hence the treatment of KHE with KMP is primarily medical. Treatment options include Corticosteroid, [3],[4] Propranolol, [5] Interferon, [6] Sirolimus, [7] Vincristine, [3],[7],[8] VAT (Vincristine, Aspirin and Ticlopidine), [7] Radiotherapy [1] and Embolization. [5] No treatment is uniformly effective or relapse- and complication-free. Because of the rare nature of the disease, management decisions are based on anecdotal opinion and isolated case reports and series without guidance from prospective trials. [5] The case presented is a KHE with KMP in a neonate of limb- and life-threatening nature due to the involvement of entire leg, ulceration, and recurrent local and systemic sepsis. As drugs such as Prednisolone and Propranolol failed to contain the disease, Vincristine was started and KHE with KMP, ulceration and sepsis resolved, and the limb was saved.

   Case Report Top

One-month-old female baby presented with a circumferential diffuse swelling of entire right leg with reddish purple discoloration noticed since birth, which developed an abscess, ulceration and purulent discharge and sepsis. There was gross edema of right foot and thigh with inguinal adenitis [Figure 1]a. Hematological investigations showed anemia and thrombocytopenia. A diagnosis of KMP was made and a biopsy was carried out after controlling the sepsis, which showed features of KHE. Immunohistochemistry was positive for Vimentin, CD31 and CD34, negative for Cytokeratin and GLUT 1 and Ki67 was 5-10% suggestive of KHE. X-ray of right lower limb did not show any bony involvement or osteomyelitis. MRI showed T2 hyperintense ill-defined circumferential mass involving the entire right leg and lower thigh. Underlying bones and joints were normal [Figure 2]. Doppler correlation of the lesion showed increased vascularity. Arterial feeders were from arteries around the knee joint and the venous drainage was into the tibial vein. The arteries from the external iliac to popliteal artery showed high resistance flow. Distal tibial vessels showed reduced but high resistance flow due to severe external compression. The baby was started on high dose Prednisolone regimen (40 mg orally on alternate days tapered to half the dose every 20 days till a dose of 2.5 mg on alternate days was completed). [9] The KHE and KMP did not show any resolution and Propranolol 2 mg/kg daily orally in two divided doses was added. [5] There was no improvement for five months since admission and the child required frequent hospitalizations for increasing size of the lesion, foul smelling discharge from the fungating ulcer over the lesion, the proximally spreading violaceous discoloration on to the thigh, increasing distal foot edema and life- and limb-threatening systemic and local sepsis. Hence, Vincristine was administered at weekly dose of 0.025 mg/kg [10] for four weeks. The lesion resolved with local wound care and compression stockings gradually over the next 10 months [Figure 1]b]. The hematological changes due to KMP also got corrected. Currently, the child is on follow-up.
Figure 1: KHE of right leg before (a) and after (b) Vincristine therapy

Click here to view
Figure 2: MRI of KHE of right leg

Click here to view

   Discussion Top

The prevalence of KHE is 0.91 per 100,000 children. The male: Female incidence is 1.33:1. KHE can be diagnosed in prenatal ultrasound. KHE is classified as superficial when its involvement is restricted to dermis through subcutaneous tissue and deep fascia. KHE is designated as deep when it involves muscle, bone, retroperitoneal or intrathoracic sites, and forms 83% of the cases. KHE is usually unifocal, but multifocal KHE have also been reported. Biopsy proven KHE of liver has never been reported. [2] Seventy-five percentage of cases progressively enlarge during infancy. The superficial component of KHE cause a red-purple discoloration with surrounding ecchymosis. [8]

KMP is hypothesized to be due to abnormal platelet activation and aggregation secondary to interaction with abnormal tumor endothelium resulting in localized trapping of platelets and consumption of clotting factors or due to turbulent blood flow that results from the architecture of the small convoluted capillaries of KHE. Infants with KMP are at risk for intracranial pleural, pulmonary, peritoneal, and gastrointestinal hemorrhage and musculocutaneous pain and decreased function. [2] Thrombocytopenia is unresponsive to platelet transfusion due to intralesional trapping. [8] KMP is less likely to occur in KHE less than 8 cm in size and its incidence increases when KHE is of deep type. [8] The mortality rate of KHE with KMP with life-threatening vasculopathy can go up to 30-50%. [1]

X-ray may show osteolysis due to bony involvement of KHE. MRI of KHE reveals ill-defined, hypo or isointense lesion in T1-weighed images and hyperintense mass with reticular standing in T2-weighed images. Feeding and draining vessels are small relative to tumor size. Doppler studies correlate these vascular changes of the lesion. [8] Histopathology of KHE reveals aggressive infiltration of normal tissues by sheets or lobules of oval or spindle endothelial cells, dilated lymphatic channels and slit-like vascular spaces filled with hemosiderin and compacted erythrocytes suggesting stasis. [8] There is characteristic absence of mitosis or nuclear atypia. There is focal immunopositivity for lymphatic endothelial markers. [2] The children with KHE with KMP who showed resolution require a close follow-up, as KMP can recur latter. [1] Authors believe that publication of number cases of this rare lesion may help to evolve a uniform treatment policy.

   References Top

Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB, et al. Kaposiform hemangioendothelioma: Atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013;162:142-7.  Back to cited text no. 1
Malhotra Y, Yang CS, McNamara J, Antaya RJ. Congenital kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon successfully treated with low-dose radiation therapy. Pediatr Dermatol 2014;31:595-8.  Back to cited text no. 2
Fahrtash F, McCahon E, Arbuckle S. Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine. J Pediatr Hematol Oncol 2010;32:506-10.  Back to cited text no. 3
Arunachalam P, Kumar VR, Swathi D. Kasabach-Merritt syndrome with large cutaneous vascular tumors. J Indian Assoc Pediatr Surg 2012;17:33-6.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
Chiu YE, Drolet BA, Blei F, Carcao M, Fangusaro J, Kelly ME, et al. Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma and Kasabach-Merritt phenomenon. Pediatr Blood Cancer 2012;59:934-8.  Back to cited text no. 5
Acharya S, Pillai K, Francis A, Criton S, Parvathi VK. Kasabach merritt syndrome: Management with interferon. Indian J Dermatol 2010;55:281-3.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
Fernandez-Pineda I, Lopez-Gutierrez JC, Chocarro G, Bernabeu-Wittel J, Ramirez-Villar GL. Long-term outcome of vincristine-aspirin-ticlopidine (VAT) therapy for vascular tumors associated with Kasabach-Merritt phenomenon. Pediatr Blood Cancer 2013;60:1478-81.  Back to cited text no. 7
Kulungowski AM, Fishman SJ. Vascular anomalies. In: Coran AG, Scott Adzick N, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, editors. Pediatric Surgery. 7 th ed. Philadelphia: Elsevier; 2012;2:1613-43.  Back to cited text no. 8
Edgerton MT, Morgan RF. Hemangiomas: Congenital hamartomas. In: Welch KJ, Randolph JG, Ravitch MM, O′Neill JA, Rowe MI, editors. Pediatric Surgery. 4 th ed. Chicago: Year Book Medical Publishers INC; 1986;2:1511-8.  Back to cited text no. 9
Guthrie SO, Rhodes M, Janco R, Stein SM, Jabs K, Engelhardt B. An infant with Kasabacjh-Merritt syndrome with associated renal hematoma and intussusception. J Perinatol 2005;25:143-5.  Back to cited text no. 10


  [Figure 1], [Figure 2]

This article has been cited by
1 Kaposiform Hemangioendothelioma of the pancreas requiring biopsy for diagnosis
Jasmine D. Lee, Barrett P. Cromeens, Megan A. Coughlin
Journal of Pediatric Surgery Case Reports. 2023; 88: 102532
[Pubmed] | [DOI]


Print this article  Email this article


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (604 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded95    
    Comments [Add]    
    Cited by others 1    

Recommend this journal

Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer | Privacy Notice

  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05