Journal of Pediatric Surgery (2013) 48, 673–676

www.elsevier.com/locate/jpedsurg

Successful treatment of Kasabach–Merritt syndrome with

transarterial embolization and corticosteroids☆
Shao-yi Zhou, Hai-bo Li, Yue-ming Mao, Pei-ying Liu, Jing Zhang ⁎
Department of Interventional Radiology and Vascular Anomalies, Guangzhou Women and Children's Medical Center,
Guangzhou 510623, PR China

Received 21 August 2012; revised 22 November 2012; accepted 27 December 2012

Key words:
Abstract Kasabach–Merritt syndrome (KMS) refers to the combination of large neonatal vascular
Kasabach–Merritt
tumors and thrombocytopenic coagulopathy. However, a standard treatment regimen for KMS has not
syndrome;
yet been established. We report a case of a 6-week-old male infant with life-threatening KMS who was
Transarterial embolization;
successfully treated with transarterial embolization and corticosteroids. One week after initiating the
Corticosteroid;
corticosteroid treatment, his platelet counts recovered, and the lesion growth halted. The approach with
Infant
corticosteroid therapy resulted in an excellent response that was maintained long enough for us to
perform transarterial embolization therapy. The combination of transarterial embolization and
corticosteroid therapy should be considered as an option for Kasabach–Merritt syndrome.
© 2013 Elsevier Inc. All rights reserved.

Kasabach–Merritt syndrome (KMS) was first noted by
Kasabach and Merritt [1] in 1940 when they described a
newborn male baby with a rapidly enlarging capillary
hemangioma in association with increasing extensive
purpura of the skin and thrombocytopenia. KMS is
characterized by a rapidly enlarging vascular anomaly and
consumptive coagulopathy with thrombocytopenia, a pro-
longed prothrombin time, a partial thromboplastin time,
hypofibrinogenemia, and the presence of D-dimer and fibrin
split products, with or without microangiopathic hemolytic
anemia. KMS lesions are generally located in the trunk,
shoulder girdle, thigh, perineum, and head and neck, and
patients have a mortality rate of 20% to 30%, particularly for
☆ swelling on his right waist since birth. Cutaneous
Conflict of interest statement: The authors declare that there are no
conflicts of interest.
⁎ Corresponding author. Tel.: +86 20 133 0555; fax: + 86 20 8133 0526.
E-mail address: keavojoe@gmail.com (J. Zhang).
mediastinal and retroperitoneal tumors [2]. The treatment of
KMS is difficult, and several therapeutic regimens, including
corticosteroids, interferon, vincristine, platelet aggregation
inhibitors, radiotherapy and surgery, have been reported in
the literature [3]. We present a case of Kasabach–Merritt
syndrome in a male infant with extreme thrombocytopenia
who was successfully treated with transarterial embolization
and corticosteroid therapy.
1. Case report
A 19-day-old male infant was admitted to our depart-
ment for a rapidly expanding, red-purple, indurated tumor
examination showed a 10-cm × 8-cm, poorly circumscribed,
reddish-purple lesion with warm plaque and scattered
petechiae over both bilateral inguinal regions and the

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674
Fig. 1 Image showing a red-purple, poorly circumscribed lesion
covering both bilateral inguinal regions and the abdominal wall of
our patient. Note the giant hemangioma on the right waist. The
image was taken 19 days after birth.
abdominal wall (Fig. 1). Further physical examination
revealed no other abnormalities. The initial laboratory test
revealed that the platelet count was 25 × 10 9 /L and
hemoglobin was 92 g/L. Other laboratory results included
a prothrombin time of 19.20 s, partial thromboplastin time
of 45.94 s, and fibrinogen of 0.56 g/L. Additional color
Doppler ultrasound examination revealed a vascular tumor
in his right waist. All of these findings supported the
diagnosis of KMS.
The patient was started on oral prednisone at a dose of
3 mg/kg/day for 1 week with close monitoring. After the
Fig. 2 A: Abdominal aortography showing the tumor fed by the branches of 4 right lumbar arteries. B: Super-selective angiography showing
that one of the feeding vessels had a significant vascular tumor blush.
S. Zhou et al.
treatment with prednisone, the patient's platelet count was
maintained at a normal level, the lesion decreased in size, and
the petechiae resolved. The patient was discharged and
returned for weekly blood tests to ensure that his platelet
count was normal.
After 2 weeks of outpatient treatment, the patient was re-
admitted to the hospital due to increased petechiae around
the right waist. At this time, the patient was found to have a
platelet count of 8 × 109/L. Other laboratory results included
a hemoglobin of 86 g/L, prothrombin time of 15.80 s, partial
thromboplastin time of 40.04 s and fibrinogen of 1.24 g/L.
The patient was admitted and treated with intravenous
dexamethasone at 2 mg/kg/day. One week after initiating
therapy, his platelet counts recovered to 86 × 109/L, and the
lesion ceased to expand. No further improvement in the
platelets was observed, and the lesion size was small enough
to perform transarterial embolization treatment.
After his parents signed informed consent forms, the
patient was referred for angiography and possible emboli-
zation. The patient was placed in a state of general
anesthesia, and a 4-Fr introducer was placed in his right
femoral artery by percutaneous puncture. Abdominal
aortography, performed with a 4-Fr Cobra catheter (Glide-
cath; Terumo, Japan), showed an extensive hypervascular
tumor feeding from the branches of four right lumbar arteries
(Fig. 2A and B). After super-selective catheterization of each
feeding artery using a 2.7-Fr microcatheter over a micro-
guidewire (Progreat; Terumo, Japan), the microcatheter was
flushed with a total of 6 mL liquid embolic agent mix
including 2 mL iodinated oil (Lipiodol Ultra Fluide;
Guerbet, France), 3 mg bleomycin A5 hydrochloride
(Bolaipingyang; Laiboten, China) dissolved in 3 mL iohexol
(Omnipaque; GE Healthcare of Shanghai, China), 1 mL
dexamethasone (2 mg) (Cisen, Lukang, China), followed by
300–500 μm polyvinyl alcohol (polyvinyl alcohol particles;
COOK, American). Subsequent super-selective angiography
showed that one of the feeding arteries was embolized
Treatment of Kasabach–Merritt syndrome
Fig. 3 Super-selective angiography showing that one of the
feeding arteries was embolized completely and the liquid embolic
agent mix was deposited in the lesion.
completely, and the liquid embolic agent mix was deposited
in the lesion (Fig. 3). Injection was stopped after complete
obliteration of the tumor blush through the main guide
catheter. The patient tolerated the procedure well.
The following morning, the size of the lesion had
decreased to half its preoperative size. Laboratory findings
revealed a platelet count of 300 × 109/L and hemoglobin of
109 g/L. One week after the operation, the platelet count
had increased to 322 × 109/L. After that, consumption
coagulopathy completely recovered. In the time leading
up to the preparation of this article, up to 10 months after
the transarterial embolization, the patient remained well, the
red-purple skin color disappeared, and there was no sign of
thrombocytopenia (Fig. 4).
Fig. 4 Photographs taken 10 months after therapy showing the
disappearance of the red-purple skin color after transarterial
embolization therapy.
675
2. Discussion
Kasabach–Merritt syndrome (KMS) is a consumptive
coagulopathy that was originally described in association
with cutaneous hemangiomas in children. It is often a
frustrating condition to treat, and it carries a high mortality
rate. Some laboratory evidence has shown that the majority
of KMS cases are associated with disseminated intravascular
coagulopathy (DIC). KMS occurs in 0.3% of infants with
hemangioma. Treatment is aimed at reducing the tumor size
and preventing significant mortality and morbidity [4].
Several factors contribute to the bleeding tendency in
KMS. A possible mechanism for consumption coagulapathy
of KMS is platelet trapping by the abnormally proliferating
endothelium within the hemangioma [3]. The tumor is
responsible for platelet trapping which, from the abnormally
proliferating endothelium within the hemangioma, can result
in the activation of platelets with secondary activation of
coagulation cascades, eventually leading to the consumption
of various clotting factors [5]. An immunohistochemical
study using a monoclonal antibody against CD61, a marker
of platelets, and isotope studies using 111indium-labeled
platelets and 51Cr-labeled platelets support the possible role
of platelet trapping in the development of KMS [6].
The treatment objectives for KMS are to prevent
bleeding from thrombocytopenia and consumptive coagulo-
pathy and to induce vascular tumor regression. Although
the management adopted for KMS is variable, including
corticosteroids and steroids, radiation therapy, surgery,
embolization, interferon (IFN)-a, chemotherapy, platelet
aggregation, the use of fibrinolysis inhibitors, supportive
care and local therapies, there are no known treatment
guidelines for this disease. Moreover, the sporadic
incidence of the disease makes it difficult to study the
treatment modalities systemically [7]. In each case, the
treating physician must decide the most suitable treatment
to achieve maximum involution of the lesion with minimal
adverse effects. A major mainstay of the treatment of KMS
is surgical excision, but it is often limited by the tumor
location or size. This approach is recommended for single
cutaneous lesions or multiple lesions in the spleen
(splenectomy) or liver (wedge resection/hepatectomy)
[8,9]. However, the patient must be stabilized before each
surgical intervention. Conventional therapy with corticoste-
roids and steroids is most likely the most cost-effective
treatment option for patients with KMS. Corticosteroids or
steroids have been used as first-line therapy because they
are available at a relatively low cost. One study
demonstrated a variable response to prednisolone, ranging
from no efficacy to significant regression of the lesion size,
including complete remission [7], while the known adverse
effects of corticosteroid and steroid treatment include
hypertension, cushingoid appearance, and opportunistic
infections [10]. More recent reports consider chemotherapy
with vincristine, an inhibitor of endothelial proliferation, as
a first-line treatment option for KMS. Haisley-Royster et al.
676
[11] had encouraging findings with the use of vincristine in
the management of Kasabach–Merritt phenomenon (KMP).
In their study, 13 (87%) of 15 patients had significant
reductions in the size of their tumors. Thomson et al. [12]
successfully treated four patients with KMS using vincris-
tine as monotherapy or concurrent therapy. More recently,
there have been additional case reports about the efficacy of
vincristine chemotherapy [13,14]. However, neurotoxicity
is the dose-limiting side effect of vincristine, and peripheral
mixed sensory and motor neuropathy is the most common
side effect. Vincristine can also produce an autonomic
neuropathy that may result in abdominal pain, constipation
and ileus. Vincristine therapy requires close monitoring of
blood counts, electrolytes, and liver function tests. A larger
series of patients treated with vincristine chemotherapy will
be needed to demonstrate its efficacy. Interferon alpha
(IFN-α) most likely works as an antiproliferative/antiangio-
genic agent. Two forms of IFN-α, IFNα2a and IFNα2b,
which most likely have identical efficacy, have been used
as second-line therapy in KMS and life-threatening
hemangiomas of infancy at a dosage of 3 million IU/m2/
day with favorable results in most, but not all, reports.
Researchers also recommend the use of IFN-α when steroid
treatment fails [15]. However, life-threatening adverse
events may occur, and the cost of treatment is considerably
higher than that of the first-line drugs. In addition, flu-like
symptoms, neutropenia, mild anemia, mild elevation of
liver function tests, and, most deleteriously, spastic diplegia
in infancy are the frequently reported side effects of
interferon therapy [16]. Radiation therapy was not consid-
ered because of its long-term effects on bone growth arrest
and the risk of the development of a secondary malignancy,
particularly when administered in infancy.
In a previous report, Blei et al. [17] took a successful
multimodal therapeutic approach in the management of
Kasabach–Merritt phenomenon. In most recent case reports,
researchers have used the combination of two or more
treatment modalities [18], while other investigators recom-
mend a stepwise multimodal approach for the treatment of
this disease [19]. We did not use surgical excision to treat our
KMS case due to the age of the patient and the size of the
tumor; additionally, there is always the risk of concurrent
DIC. In the present case, the administration of dexametha-
sone helped stabilize the patient's general condition,
allowing for transarterial embolization. We achieved an
excellent outcome for our patient using transarterial
embolization with PVA, which dramatically improved our
patient's hematological parameters. Our case adds to the
growing body of literature suggesting that treatment
alternatives, particularly those involving transarterial embo-
lization, should be considered.
In conclusion, a patient with KMS was successfully
treated with transarterial embolization and corticosteroid
therapy. An excellent response was achieved after the
corticosteroid treatment, which was maintained long enough
to embolize the tumor. The combination of transarterial
S. Zhou et al.
embolization and corticosteroid therapy may be considered
as an option for KMS, although well-designed studies are
needed to quantify the benefits and risks of this treatment
combination. The use of embolization seems to be safe and
very effective, with both hematological cure and involution,
and should be considered early in the treatment course of
Kasabach–Merritt syndrome.
References
[1] Kasabach HH, Merritt KK. Capillary hemangioma with extensive
purpura: report of a case. Am J Dis Child 1940;59:1063-70.
[2] Tsang WY, Chan JK. Kaposi-like infantile hemangioendothelioma. A
distinctive vascular neoplasm of the retroperitoneum. Am J Surg
Pathol 1991;15:982-9.
[3] Hall GW. Kasabach–Merritt syndrome: pathogenesis and manage-
ment. Br J Haematol 2001;112:851-62.
[4] Freeman I, Ganesan K, Emmerson AJ. Kasabach–Merritt syndrome in
a term neonate. Arch Dis Child Fetal Neonatal Ed 2012;97:F139-40.
[5] Kim T, Roh MR, Cho S, et al. Kasabach–Merritt syndrome arising
from tufted angioma successfully treated with systemic corticosteroid.
Ann Dermatol 2010;22:426-30.
[6] Seo SK, Suh JC, Na GY, et al. Kasabach–Merritt syndrome:
identification of platelet trapping in a tufted angioma by immunohis-
tochemistry technique using monoclonal antibody to CD61. Pediatr
Dermatol 1999;16:392-4.
[7] Maguiness S, Guenther L. Kasabach–Merritt syndrome. J Cutan Med
Surg 2002;6:335-9.
[8] Drolet BA, Scott LA, Esterly NB, et al. Early surgical intervention in a
patient with Kasabach–Merritt phenomenon. J Pediatr 2001;138:756-8.
[9] Pasqual E, Bacchetti S, Gasparini D, et al. Embolisation of
arteriovenous intrahepatic fistulas associated with diffuse haemangio-
matosis of the liver. Report of a case in an adult and review of the
literature. Chir Ital 2007;59:701-5.
[10] Uysal KM, Olgun N, Erbay A, et al. High-dose oral methylprednis-
olone therapy in childhood hemangiomas. Pediatr Hematol Oncol
2001;18:335-41.
[11] Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach–Merritt
phenomenon: a retrospective study of treatment with vincristine. J
Pediatr Hematol Oncol 2002;24:459-62.
[12] Thomson K, Pinnock R, Teague L, et al. Vincristine for the treatment
of Kasabach–Merritt syndrome: recent New Zealand case experience.
N Z Med J 2007;120:U2418.
[13] Lopez V, Marti N, Pereda C, et al. Successful management of
kaposiform hemangioendothelioma with Kasabach–Merritt phenome-
non using vincristine and ticlopidine. Pediatr Dermatol 2009;26:365-6.
[14] Garcia-Monaco R, Giachetti A, Peralta O, et al. Kaposiform
hemangioendothelioma with Kasabach–Merritt phenomenon: suc-
cessful treatment with embolization and vincristine in two newborns. J
Vasc Interv Radiol 2012;23:417-22.
[15] 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 2003;88:67-8.
[16] Wananukul S, Nuchprayoon I, Seksarn P. Treatment of Kasabach–
Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole,
and interferon. Int J Dermatol 2003;42:741-8.
[17] Blei F, Karp N, Rofsky N, et al. Successful multimodal therapy for
kaposiform hemangioendothelioma complicated by Kasabach–Merritt
phenomenon. Pediatr Hematol Oncol 1998;15:295-305.
[18] Jiang RS, Hu R. Successful treatment of Kasabach–Merritt syndrome
arising from kaposiform hemangioendothelioma by systemic cortico-
steroid therapy and surgery. Int J Clin Oncol 2012;17:512-6.
[19] Shin HY, Ryu KH, Ahn HS. Stepwise multimodal approach in the
treatment of Kasabach–Merritt syndrome. Pediatr Int 2000;42:620-4.