Kasabach-Merritt syndrome in infants

N a n c y Burton Esterly, M . D .
Chicago, IL

Kasabach-Merritt syndrome consists of thrombocytopenia,

microangiopathic hemolytic anemia, and an acute or chronic
consumption coagulopathy in association with a rapidly enlarging
hemangioma. Although the potential for serious morbidity is great,
the condition remits when the hemangioma begins to involute. Four
infants with large congenital cavernous hemangiomas and
Kasabach-Merritt syndrome are described. All four received oral
prednisone for variable periods; two received aspirin and one
dipyridamole. Two patients required intravenous heparin because of
life-threatening disseminated intravascular coagulation and bleeding.
Two patients experienced almost total involution of their
hemangiomas by the age of 2 years, and one by the age of 13
months. The fourth patient showed signs of beginning resolution of
the hemangioma at the age of 8 months and hematologic values
returned to normal levels. No side effects from therapy were noted in
any of these infants. (J AM ACAD DERMATOL8:504-513, 1983.)

In 1940 Kasabach and Merritt I described a CASE REPORTS

young infant with a rapidly enlarging hemangioma
of the leg, thrombocytopenia, and a hemorrhagic
diathesis. That combination of findings has be-
come known as the Kasabach-Merritt syndrome
and, more recently, has been shown to be associ-
ated with a consumption coagulopathy and mi-
croangiopathic hemolytic anemia. 2-6 In some in-
stances, the hematologic findings have indicated a
chronic localized coagulopathy; however, in oth-
ers, the process has eventuated in an acute dissem-
inated type of intravascular coagulation (DIC).
Four infants are described with large hemangi-
omas and hematologic abnormalities requiring in-
tervention. All four responded to medical man-
agement.
From the Division of Dermatology, Department of Pediatrics,
Northwestern University Medical School.
Read before the 102nd Annual Meeting of the American Dermatolog-
ical Association, Inc., Hot Springs, VA, May 2-6, 1982.
Accepted for publication Aug. 26, 1982.
Reprint requests to: Dr. Nancy B. Esterly, Head, Division of Der-
matology, The Children's Memorial Hospital, 2300 Children's
Plaza, Chicago, IL 60614.
504
Case 1
A 7-week-old male infant was referred for evalua-
tion because of a cavernous hemangioma of the lower
portion of the right leg which was noted at birth and had
been increasing very gradually in size. A platelet count
obtained during the immediate neonatal period was
normal.
On physical examination he was an alert, active in-
fant with normal vital signs. The only abnormal physi-
cal finding was an enlarged right leg with a skin-
colored to violaceous compressible mass extending
from the ankle to just below the knee. No bruits were
heard. The hemoglobin was 10.8 gm and the platelet
count 87,000/mm a. A short course of prednisone was
advised in a dosage of 2 mg/kg/day.
He was next seen at Children's Memorial Hospital at
10 weeks of age. Prednisone had been started, but at a
dose lower than that suggested. In the interim, his leg
had enlarged several centimeters, become firm to pal-
pation, and, for the first time, ecchymoses and
petechiae were visible in the taut, shiny skin over the
surface of the hemangioma (Fig. 1, A). The right knee
was now bigger than the left, and a compressible 3-cm
inguinal mass had appeared on the right. He was
otherwise well, and there was no evidence of bleeding
Volume 8
Number 4
April, 1983
Fig. 1. Case 1. A, Hemangioma of the right leg at 10 weeks of age. B, Hemangioma of the
right leg at 8 months of age.
from the gastrointestinal or urinary tract. The labora-
tory data were as follows: hemoglobin, 8.9 gin; platelet
count, 9,000/ram3; fibrinogen, 85 rag% (normal, 150-
350 mg/dl); factor V, 64%; prothrombin time (PT),
15.8 second (11.7, control); partial thromboplastin
time (l:q'T), 52.8 second (30-45, normal). The predni-
sone dosage was doubled (3 mg/kg/day) and, when
seen 2 weeks later at 3 months of age, the leg edema
was significantly decreased, the petechiae had disap-
peared, and the groin mass was softer and smaller. The
platelet count was 35,000/mm a, fibrinogen, 51 mg/dl,
fibrin sprit products (FSP) >10, Frl', 16.5/11.3, and
PTT, 46.5.
By 4~A months of age, the hemangioma appeared
stable, his cardiac status remained normal, and the he-
moglobin and platelet counts were normal, although the
fibrinogen level was still low and the PT prolonged.
One month later the prednisone dosage was lowered
without incident. The prednisone was thereafter re-
duced at regular intervals and discontinued by 8 months
of age. All laboratory studies were normal at the
7-month visit. The groin mass had completely disap-
peared. The right leg, although larger and firmer than
the left, was less discolored than previously, and the
circumferential measurement was slowly decreasing
(Fig. 1, B).
When seen at 1 year of age, he was standing and
Kasabach-Merritt syndrome 505
walking alone despite the hemangioma, which was
continuing to regress. Throughout his first year his
weight remained at approximately the 50th percentile.
His length was at the 75th percentile at 3 months of
age, 25th at 6 months, 10th at 9 months, and between
the 75th and 90th at 1 year of age.
Case 2
This 5-week-old male infant was the product of a
normal pregnancy and delivery. At birth a large
hemangioma of the left forearm was noted, but he was
otherwise normal and did well in the immediate
postnatal period. At 2 weeks of age his platelet count
was 44,000/ram 3, at 4 weeks of age, 39,000/ram 3,
and, on the day of admission, 8,500/ram 3. There were
no bruises or petechiae evident nor was a history of
hematuria, hematemesis, or melena obtained.
On examination the most remarkable finding was a
5•215 cavernous hemangioma which encom-
passed almost the entire length of the left forearm (Fig.
2, A). There were no bruits. Additional findings o f note
were a pulse of 160/minute, a respiratory rate o f 75/
minute, a grade III/VI systolic ejection murmur, hepato-
megaly of 2 cm, and palatal ecchymoses. Laboratory
data included a hemoglobin of 10.6 gm, hematocrit,
32%, platelet count, 5,000/mm 3, reticulocyte count,
0.9%, fibrinogen, 103 mg/dl, FSP, 20 /zg/ml, and

506 Esterly
Fig. 2. Case 2. A, Hemangioma of the left forearm at 5 weeks of age. B, Hemangioma of
the left forearm at 13 months of age.
a normal PT and PTT. Chest film demonstrated 1+
cardiomegaly. Electrocardiogram and echocardiogram
showed biventricular hypertrophy felt to be due to a high
output state. Examination of urine and stool was nega-
tive for blood. Bone marrow examination demonstrated
increased numbers of platelets.
Prednisone therapy was initiated at a dosage of 2
mg/kg/day. A pressure bandage was applied briefly but
resulted in circulatory compromise and was discon-
tinued. The platelet count rose to 18,000/mm 3 6 days
later, at the time of discharge. Two weeks later the
platelet count was 175,000/mm 3, and the cardiac mur-
mur and hepatomegaly had disappeared. At 10 weeks
of age there was evidence of beginning fibrosis, al-
though the hemangioma was larger by measurement.
The prednisone dosage was reduced a month later
(platelet count, 502,000/mma), and alternate-day ther-
apy was instituted the following month. The prednisone
was gradually reduced and discontinued by 6 months of
age. By 13 months of age the hemangioma had invo-
luted considerably (Fig. 2, B). Development was nor-
real throughout his course. There was no apparent al-
teration in his growth curves while on medication.
Case 3
This 14-day-old male infant was admitted to the
hospital because of pallor, a hematocrit of 20%, and a
Journal of the
American Academy of
Dermatology
rapidly enlarging cavernous hemangioma of the left
axilla. The hemangioma had been present since birth.
At the time of admission it measured 8 cm in diameter,
was reddish in color, firm, nontender, and without
bruits. The general physical examination was unre-
markable. No petechiae or ecchymoses were noted, nor
was there evidence of cardiac failure. Pertinent labora-
tory data included a hemoglobin of 6.4 gm, hematocrit,
19.5%, white blood cell count of 16,000/mm 3 with a
normal differential, and a platelet count of 19,000/
mm s. Fragmented red blood cells (schistocytes) were
noted on smear.
A diagnosis of Kasabach-Merritt syndrome with
microangiopathic hemolytic anemia was made and
confirmed by a prolonged PT and PTT, decreased
fibrinogen levels, and elevated FSP. Multiple transfu-
sions administered during the first 10 days of hospital-
ization failed to correct the anemia. Numerous pete-
chiae and ecchymoses appeared and were particularly
concentrated over the hemangioma, which continued to
increase in size, elevating the arm and markedly distort-
ing the contour of the shoulder. By the seventh hospital
day the hemangioma encompassed the entire shoulder
girdle and the left anterior portion of the chest (Fig. 3,
A), and the platelet count reached a nadir of 1,900/
mm 3. Prednisone therapy in a dosage of 4 mg/kg/day
and intravenous heparin, 75 mg/kg every 4 hours, were
Volume 8
Number 4
April, 1983
Fig. 3. Case 3. A, Hemangioma of the upper left portion of the trunk at 3 weeks of age.
Note the ecchymoses and marked distortion of the shoulder. B, Considerable improve-
ment of the hemangioma by 8 weeks of age.
instituted at this time. Within a few days, the heman-
gioma ceased to enlarge and the platelet count stabi-
lized at about 20,000/mm ~. Heparin dosages were
monitored by maintaining the gY and PTT in the range of
11/~-2 times nomml. The infant remained hospitalized
for 5 weeks and was discharged on daily prednisone, 3
mg/kg/day, and intravenous heparin every 4 hours. At
the thne of discharge the hematocrit was 34% and the
platelet count 46,000/mma; the hemangioma was
somewhat smaller and softer than previously (Fig. 3, B).
During subsequent weeks he was followed closely in
the outpatient clinic. Two weeks after discharge (age, 9
weeks) the prednisone was switched to an alternate-day
regimen. Heparin was continued at a dosage of 75
mg/kg every 4 hours. The platelet count was 43,000/
mm z, hematocrit, 27%, reticulocyte count, 2.9%,
fibrinogen, 65 rag%, and FSP, 1:8. Fragmented red
blood cells were still noted on smear. By 11 weeks of
age the platelet count rose to 185,000/mm a, and the
hemangioma was gradually decreasing in size.
By 4 months of age the heparin was discontinued,
but the prednisone dose was maintained at 4 mg/kg/day
in a single early rooming dose. An oral iron supple-
ment, aspirin, 90 rag, and dipyridamole, 25 mg were
added to his regimen. His platelet count continued to
hover around the 50,000 mark, but there was no evi-
Kasabach-Merritt syndrome 507
dence of internal or cutaneous bleeding. His hematocrit
stabilized at 30%, with a reticulocyte count ranging
from 3% to 5%; a single platelet count reached
270,000/ram a but it subsequently dropped again to
57,000/mm 3 shortly thereafter. At 5 months of age the
prednisone dosage was reduced to 2 mg/kg/day, given
on an alternate-day basis along with aspirin and di-
pyridamole daily. His general condition remained sta-
ble, and he was developing normally. The left arm
appeared positionally rotated, with a limited range of
motion at the shoulder.
During the next several months the aspirin and
prednisone were further reduced in dosage. All medi-
cations were discontinued by 10 months o f age.
Throughout this period weights ranged from the 5th to
the 10th percentile, and lengths were below the fifth
percentile.
At approximately 14 months of age the platelet count
and hemoglobin rose to normal, and no further
hematologic problems were encountered. Development
was normal, height was in the 25th percentile, and
weight in the 5th percentile. The hemangioma was
considerably smaller (Fig. 4, A), but the mobility of the
left arm continued to be somewhat restricted. Physical
examination at 2 years of age revealed residual
hemangioma in the left axilla only (Fig. 4, B) and

508 Esterly
Fig. 4. Case 3. A, Hemangioma at 14 months of age. B, Hemangioma at 2 years of age.
limited range of motion of the left shoulder, attributed
to muscle atrophy from disuse. A roentgenogram of the
arm and shoulder was normal.
Height and weight are in the 75th percentile at 4
years of age. Hypomobility of the shoulder joint, due to
fibrosis with secondary muscle atrophy, requires surgi-
cal intervention to restore full range of motion.
Case 4
This term male infant was delivered by cesarean
section because of meeonium staining of the amniotic
fluid, late fetal deceleration, and failure to progress. At
birth he was noted to have a large cavernous heman-
gioma extending from the left hip and buttocks to the
knee. The entire leg was massively edematous, woody
in texture, and purplish in color, with some superficial
blebs noted in the overlying taut skin. The left midthigh
circumference was 10 cm greater than the right. Addi-
tional abnomaal findings included marked pallor,
tachypnea, tachycardia, a systolic ejection murmur,
and hepatosplenomegaly. Laboratory studies revealed a
hemoglobin of 8.5 gin, hematocrit, 24%, platelet
count, 9,000/mm ~, PT, 22.3/12.2, P I T , 122[36,
fibrinogen, 66 rag%, and FSP, 64/~g/ml. A diagnosis
of Kasabach-Merritt syndrome was made, and he was
treated immediately with a transfusion of whole blood
and a heparin drip, followed by a transfusion of fresh
platelets. Arteriography performed via an umbilical ar-
Journal of the
American Academy of
Dermatology
tery catheter showed a nomaal arterial supply to the left
leg and a massive hemangioma of the thigh. Because a
diagnosis of hemangiosarcoma was entertained, 150
rads were delivered to the left thigh on the second and
third days of life.
The hospital course was complicated by high output
failure which required digitalization and diuretics. In-
travenous heparin was discontinued at 6 days; aspirin
was begun in a dosage of 45 mg twice daily and in-
creased to a dosage of 75 mg twice daily. Numerous
transfusions of fresh frozen plasma, cryoprecipitate,
packed red blood ceils, and single-donor fresh platelet
concentrate were given during this period, but none had
any lasting effect on the platelet count or other
hematologic values.
By 2 weeks of age his leg was less swollen, his
cardiac condition stable, and the DIC resolving, al-
though the platelet count continued to drop precipi-
tously to below 10,000/ram 3 after every platelet trans-
fusion. A pressure bandage was applied and resulted in
marked swelling and purplish discoloration of the foot,
leg, and scrotum, followed by recrudescence of an
acute DIC state. At 3 weeks of age digitalis was dis-
continued and prednisone, 2 mg/kg/day in two divided
doses, was begun. By 5',~ weeks he was ready for
discharge; the hemangioma was slowly decreasing in
size and the platelet count remained stable at around
50,000/ram ~.
Volume 8
Number 4
April, 1983
Fig. 5. Case 4. A, Hemangioma of the left hip and thigh at 3% months of age. Note the
ecchymoses of the leg and genitalia. B, Appearance of the hemangioma at 11 months of
age.
By 31A months of age he had little difficulty except
for small amounts of blood passed in the stool. His left
leg was less edematous, but there were ecchymoses on
the leg, back, chest, penis, and scrotum (Fig. 5,A). He
had tachycardia, a systolic ejection murmur, and hepa-
tomegaly. Mild cardiomegaly was noted on chest film,
and the electrocardiogram showed combined ventricu-
lar hypertrophy. The cardiologist felt he was in impend-
ing failure and recommended digitalization. The plate-
let count remained at 50,000/mm a, his hematocrit at
30%, and the fibrinogen level at 47 mg/dl. The PT and
PTT were prolonged. He continued to improve gradu-
ally, although bruising was consistently a problem
since he was an extremely active infant. His weight
gain was normal, as was his developmental progress.
The prednisone dosage was switched to an alternate-
day regimen by 5~/2 months of age. Digitalis was dis-
continued at 8 months of age, by which time his platelet
count had stabilized at 90,000/ram 3. The prednisone
was reduced at the age of 9 months and finally discon-
tinued at 11 months, at which time his platelet count
was 465,000/mm a, his hematocrit 40%, and his PT,
PTT, and fibrinogen levels normal. On examination the
hemangioma appeared pale, less bulky, and quite soft
and compressible (Fig. 5, B). By 2 years of age the leg
appeared almost normal (Fig. 6). Weight and length
Kasabach-Merritt syndrome 509
consistently remained at the fifth percentile throughout
his entire course.
DISCUSSION
The four infants described in this report dem-
onstrated the findings of Kasabach-Merritt syn-
drome, a serious hematologic state that may com-
plicate the course of patients with rapidly growing
hemangiomas. The features of this syndrome in-
clude an enlarging hemangioma, thrombocyto-
penia, and anemia. The development of an acute
or chronic consumption coagulopathy (dissemi-
nated intravascular coagulation) may result in
serious coagulation defects, particularly decreased
fibrinogen levels, but also depressed levels of
several of the coagulation factors. Rapid onset and
progression of this state without intervention may
terminate in a frighteningly quick demise.
In a review of seventy-four cases of Kasa-
bach-Merritt syndrome, Shim r noted that the ma-
jority of the hemangiomas were large and occurred
on the limbs or on a portion of the tt'unk, with
involvement of the proximal adjacent limb. This
hematologic syndrome has also rarely been asso-
 Journal of the
510 Esterly American Academy of
Dermatology

Table I. Hematologic evaluation for

Kasabach-Merritt syndrome
Screening tests Expected result

Hemoglobin Often $
Hematocrit Often
Blood smear Fragmented RBCs
Prothrombin time Prolonged
Partial prothrombin time Prolonged
Platelet count < 150,000/ram a
Fibrinogen level < 150 mg/dl
Fibrin split products Elevated
Optional tests [ Expectedresult
Presence of soluble fibrin Positive
Levels of factors II, V, Reduced
VIII
Platelet and fibrinogen Shortened
survival

have occurred in all age groups, with mortality

Fig. 6. Case 4. Appearance of the leg at 2 years of age.
ciated with relatively small but rapidly growing
hemangiomas, s and with visceraP ,7'9'1~ and retro-
peritoneal hemangiomas. 5 Histopathologic exami-
nation of these lesions has demonstrated a variety
of patterns, including capillary and cavernous
hemangiomas, mixed hemangiomas, and heman-
gioendotheliomas.7.8
Kasabach-Merritt syndrome has its highest in-
cidence in young infants, usually during the first
few weeks of life. In Shim's 7 series, the median
age of admission to the hospital was 5 weeks. Nev-
ertheless, this problem is not exclusive of older
children and adults, and several patients with nonin-
voluting hemangiomas have experienced acute
onset of Kasabach-Merritt syndrome, sometimes
with disastrous results. 4'~'1~ In these patients,
the coagulopathy has been enhanced or precipi-
tated by surgical procedures 1a'14 and angiogra-
phy 1~ and, in gravid women, by delivery of a full-
term infant and by therapeutic abortion. 11 Deaths
figures in the range of 20% to 30% 7.14
If Kasabach-Merritt syndrome is suspected, an
immediate hematologic evaluation is indicated.
Findings that should elicit concern are pallor, spon-
taneous petechiae and ecchymoses, easy bruis-
ing, rapid change in size and appearance of the
hemangiomatous mass, prolonged bleeding from
punctures or abrasions, hematuria, hematochezia,
epistaxis, and oozing from the umbilicus or cir-
cumcision site in an infant. The vascular mass
may become tense and woody in texture, with
overlying taut, shiny, discolored skin; in some in-
stances, the appearance of these hemangiomas has
mistakenly suggested cellulitis. Petechiae and ec-
chymoses may concentrate initially in the skin
overlying and adjacent to the hemangioma and
then spread to involve other areas.
The appropriate studies for hematologic screen-
ing are listed in Table I. In addition to hematocrit
and hemoglobin determinations, examination of
the blood smear for fragmented red blood cells
(schistocytes) is a helpful screening test, although
abnormal red cell morphology cannot be relied
upon as an invariable indicator of DIC. 17.18 If
thrombocytopenia is present, clotting studies, in-
cluding a prothrombin time and/or partial throm-
boplastin time, fibrinogen level, and determina-
tion of FSP should be obtained for documentation
Volume 8
Number 4
April, 1983
of a consumption coagulopathy. Coagulation fac-
tor levels, particularly factors II, V, and VIII, are
reduced in DIC and should be assayed, if possible.
The identification of soluble fibrin and fibrinopep-
tide A in plasma also provides strong evidence for
a DIC state, j7,~8
The thrombocytopenia of Kasabach-Merritt syn-
drome has been attributed to trapping and in-
creased destruction of platelets within the vascular
mass. As might be expected, bone marrow exami-
nation demonstrates normal to increased numbers
of megakaryocytes. 18 Several pieces of indirect
evidence support sequestration as a pathogenetic
mechanism for the thrombocytopenia. Higher
platelet counts have been noted in blood removed
directly from the hemangioma as compared to pe-
ripheral bloodlg; following intravenous injection
of 51Cr-labeled platelets, the radioactivity has been
found localized to the tumor mass. ~'2~Preferential
accumulation of fibrinogen within the tumor has
also been demonstrated following administration
of Ilal-labeled fibrinogen. '5 Both platelets and
fibrinogen from patients with Kasabach-Merritt
syndrome have a shortened survival time. 6'15'16'2~
Some authors have suggested that a chronic
rather than acute consumption coagulopathy is
more common in patients with Kasabach-Merritt
syndrome. In a chronic coagulopathy, the levels of
platelets and coagulation factors represent a bal-
ance between rate of production and rate of con-
sumption. If production rates are increased to
compensate for loss, levels of these factors can be
normal or even elevated despite more rapid con-
sumption. For this reason, it is important to assay
for FSP as confirmatory evidence of a consump-
tion coagulopathy with secondary hyperfibrinoly-
sis. When the balance is disturbed, acute DIC may
supervene.
Treatment of this disorder is determined solely
by clinical criteria. Young infants may tolerate
moderate to severe thrombocytopenia without
bleeding because of their relative immobility. In
these instances, careful observation may be the
most appropriate management since, ultimately,
most hemangiomas involute spontaneously and
the hematologic abnormalities resolve without
therapy. 21,22 If the hemangioma is enlarging
rapidly and the platetet count dropping precipi-
Kasabach-Merritt syndrome 511
tously, prednisone therapy at a dosage of 2-4 mg/
kg/day may prevent further growth of the lesion
and effectively palliate the condition. 23-2s Several
authors have doubted the efficacy of systemic cor-
ticosteroids, and, indeed, for some patients with
Kasabach-Merritt syndrome this medication may
be relatively ineffective. 3'13 In some instances,
however, an inadequate dose or too brief a course
of steroids was given. 26 Generally a 2- to 4-week
period is necessary for an adequate trial.
The mechanism of action of corticosteroids on
hemangiomas is poorly understood. Increased
vascular sensitivity to vasoconstriction is one
mechanism that has been suggested. 27'28 Steroid-
induced thrombosis and inhibition of fibrinolysis,
resulting in obliteration of the vascular channels,
has been proposed as an alternative mechanism. 23
There is also no agreement as to whether the pri-
mary effect of the steroid in Kasabach-Merritt
syndrome is to reduce the vascular mass or to re-
store the integrity of the clotting system. It is
known, however, that prednisone increases plate-
let survival time. z'~
If the patient continues to deteriorate despite
oral corticosteroid therapy, additional therapeutic
measures are required. In the emergent situation
when thrombosis is evident and bleeding is a prob-
lem, it is necessary to bring the consumption
coagulopathy under control. The use of heparin is
controversial but has been recommended by some
authors in a dosage of 100 U/kg every 4 hours
until bleeding ceases, the platelet count is sta-
ble, and the coagulation defect is under con-
t r o l , 3'13'14'16'17 Following institution of heparin
therapy, the platelet and red cell survival times
should return to normal'6; heparin has also been
shown to prolong the survival of fibrinogen in
Kasabach-Merritt syndrome, a~ The restoration of
platelets, coagulation factors, and fibrinogen by
administration of platelet concentrates, fresh fro-
zen plasma, and cryoprecipitate may be ineffec-
tive until the patient is heparinized, since these
factors will simply be consumed at an even faster
rate and bleeding will be potentiated; replacement
therapy, however, can be very effective following
heparinization. 17,18
Two additional drugs have recently been advo-
cated in the treatment of Kasabach-Merfitt syn-

512 EsterIy
drome, but there is, as yet, scant literature pertain-
ing to their use. s'13'1~ These drags are aspirin and
dipyfidamole, both inhibitors of platelet func-
tion. 29'z1'32 No dosage guidelines for combined use
in Kasabach-Merritt syndrome are available for
infants and children.
Additional measures for the treatment of shock
and heart failure may be required for acutely ill
patients. Many infants require digitalization until
the high output state has been abolished. In situ-
ations in which medical management has failed,
extirpation of the hemangioma has been per-
formed successfully ~'7,8'~a'84 but is fraught with
complications. Splenectomy is never indicated, s Ir-
radiation has been administered, usually in com-
bination with other modalities, but the attendant
long-term risks make this modality less acceptable
as a treatment of choice. Compression bandaging
as adjuvant therapy has been successful in some
instances but may cause circulatory compromise. 21
Despite its potential for disaster, Kasabach-
Merritt syndrome is basically a self-limited condi-
tion, When possible, expectant observation is the
safest approach. The specific therapeutic regimen
chosen for a particular patient depends solely on
the patient's clinical status and hematologic eval-
uation. Consultation with a hematologist is always
warranted, particularly if anticoagulants and anti-
platelet drugs are being considered. Some patients
respond promptly to the measures outlined, but
others have a prolonged and difficult course. The
choices of surgical extirpation or radiation therapy
must take into account the risk/benefit ratio and the
skills and experience of the attendant physicians.
REFERENCES
1. Kasabach HH, Merritt KK: Capillary hemangioma with
extensive purpura, Am J Dis Child 59:1063-1070, 1940.
2. Wacksman SJ, Flessa HC, Glueck HI, Will JJ: Coagula-
tion defects and giant cavernous hemangioma. Am J Dis
Child 111:71-74, 1966.
3. Hagerman LJ, Czapek EE, Donnellan WL, Schwartz
AD: Giant hemangioma with consumption coagulop-
athy. J Pediatr 87:766-768, 1975.
~-. Watanabe M, Yuasa S, Taketa K, KonagaE, Okajima K:
A case of giant cavernous hemangioma of the liver
complicated by intravascular coagulopathy. Acta Med
Okayama 32:61-68, 1978.
5. Pearl GS,MathewsWH: C~176176 hem-
angioendothelioma with Kasabach-Merritt syndrome.
South Med J 72:239-240, 1979.
6. Propp RP, Scharfman WB: Hemangioma-thrombo-
Journal of the
American Academy of
Dermatology
cytopenia syndrome associated with microangiopathie
hemolytic anemia. Blood 28:623-633, 1966.
7. Shim WKT: Hemangiomas of infancy complicated by
thrombocytopenia. Am J Surg 116:896-906, 1968.
8. Martins AG: Hemangioma and thrombocytopenia. J Pe-
diatr Surg 5:641-648, 1970.
9. Maeda H, Matsuo T, Nagaishi T, Ikeda T, Tomonaga Y,
Moil H: Diffuse hemangiomatosis, coagulopathy and
microangiopathic hemolytic anemia. Acta Pathol Jpn
31:135-142, 1981.
10. Behar A, Moran E, Izak G: Acquired hypofibrino-
genemia associated with giant cavernous hemangioma of
the fiver. Am J Clin Pathol 40:78-82, 1963.
11. Phillippe M, Acker D, Frigoletto FD: Pregnancy compli-
cated by the Kasabach-Merritt syndrome. Obstet Gyne-
col 56:256-258, 1980.
12. Katz HP, Askin J: Multiple hemangiomata with throm-
bocytopenia. An unusual case with comments on steroid
therapy. Am J Dis Child 115:351-357, 1968.
13. Jona JZ, Kwaan HC, Bjelan M, Raffensperger JG: Dis-
seminated intravascular coagulation after excision of
giant hemangioma. Am J Surg 127:588-592, 1974.
14. Lang PG, Dubin HV: Hemangioma-thrombocytopenia
syndrome. Arch Dermatol 111:105-107, 1975.
15. Straub PW, Kessler S, Schreiber A, Frick PG: Chronic
intravascular coagulation in Kasabach-Merritt syndrome.
Arch Intern Med 129:475-478, 1972.
16. Rodriguez-Erdmann F, Button L, Murray JE, Moloney
WC: Kasabach-Merritt syndrome: Coagulo-analytical
observations. Am J Med Sci 261:9-15, 1971.
17. Corrigan JJ Jr: Disseminated intravascular coagulopathy.
Pediatrics 64:37-45, 1979.
18. Bell WR" Disseminated intravascular coagulation. Johns
Hopkins Med J 146:289-299, 1980.
19. Gilon E, Ramot B, Sheba C: Multiple hemangiomata
associated with thrombocytopenia: Remarks on the
pathogenesis of the thrombocytopenia in this syndrome,
Blood 14:74-79, 1959.
20. Kontras SB, Green OC, King L, Duran RJ: Giant
hemangioma with thrombocytopenia. Case report with
survival and sequestration studies of platelets labeled
with chromium 5 I. Am J Dis Child 105:188-195, 1963.
21. Wallerstein RO: Spontaneous involution of giant hem-
angioma. Am J" Dis Child 102:233-235, 1961.
22. Cartwright JD, Van Coller BM: Conservative manage-
ment of the Kasabach-Merritt syndrome (cavernous
haemangioma and thrombocytopenia). S Afr Med J
60"670-672, 1981.
23. Evans J, Batchelor ADR, Stark G, Uttley WS: Haeman-
gioma with coagulopathy. Sustained response to predni-
sone. Arch Dis Child 50:809-812, 1975.
24. Schneider HJ, Lascari AD: Consumption coagulopathy
in an infant with Kasabach-Merritt syndrome. Helv
Paediatr Acta 23:674-678, 1968.
25. Edgerton MT: The treatment of hemangiomas: With
special reference to the role of steroid therapy. Ann Surg
183:517-532, 1976.
26. Brown SH Jr, Neerhout RC, Fonkalsrud EW: Prednisone
therapy in the management of large hemangiomas in in-
fants and children. Surgery 71:168-173, 1972.
27. Fost NC, Esterly NB: Successful treatment of juvenile
hemangiomas with prednisone. J Pediatr 72:351-357,
1968.
Volume 8
Number 4 Kasabach-Merritt syndrome
April, 1983

28. Zarem HA, Edgerton MT: Induced resolution of cavern-
ous hemangiomas following prednisolone therapy, Plast
Reconstructr Surg 39:76-83, 1967.
29. Harker LA, Slichter SJ: Platelet and fibrinogen con-
sumption in man. N Engl J Med 287:999-1005, 1972.
30. Hillman RS, Phillips LL: Clotting-fibrinolysis in a
cavernous hemangioma. Am J Dis Child 113:649-653,
1967.
31. Didisheim P, Fuster V: Actions and clinical status of
platelet-suppressive agents. Semin Hematol 15:55-72,
1978.
32. Koeh-Weser J, Weiss I-IJ: Antiplatelet therapy. N Engl J
Med 298:1344-I347, 1978.
33. DeKlerk DJt, Northover RC: Giant hemangiomas of the
scalp. A report of 2 cases. S Afr Med J 55:59-62, 1979.
34. Inglefield JT Jr, Tisdale PD, Fairchild JP: A case of
hemangioma with thrombocytopenia in the newborn in-
fant treated by total excision. J Pediatr 59:238-241,
1961.

Complications of cutaneous cryosurgery

Richard F. Elton, M.D.
Detroit, MI

The complications of cutaneous cryosurgery may be divided into

technical problems, those involving patient selection, reactions of
the immediate and acute type, short-term reactions, and long-term
complications. Technical problems involve primarily the delivery
system and temperature monitoring. Proper patient selection is
essential for avoiding complications. Reactions to cryotherapy vary
widely from those which may be considered normal to hemorrhage,
severe systemic reactions in cold-sensitive individuals, full-thickness
skin necrosis, syncope, and sudden death. Short-term complications
include hemorrhage, infection, and granuloma pyogenicum.
Long-term reactions include pseudoepitheliomatous hyperplasia,
nerve damage, pigmentary problems, tissue defects, delayed healing,
scar formation, and the recurrence of benign and malignant lesions.
(J AM ACAD DERMATOL 8:513-519, 1983.)

A place for cryosurgery has been firmly estab-
lished in the armamentarium of many physicians,
and for many dermatologists it may be the treat-
ment of choice for a variety of benign and malig-
nant skin lesions. Not only must the cryotherapist
know which lesions to treat or not treat but also he
must know the consequences of such treatment. In
From Wayne State University Department of Dermatology.
Accepted for publication Aug. 26, 1982.
Reprint requests to: Dr. Richard F. Elton, 22250 Providence Dr.,
Suite 301, Southfield,MI 48075.
competent hands, cryosurgery and, more specifi-
cally, liquid nitrogen (LN2) therapy, has proved to
be a safe and effective therapy. There are, how-
ever, a number of possible complications with
which the cryotherapist should be familiar? This
work will review the known problems and will
discuss other problems which are theoretically
possible.
The complications of cryosurgery can, for
practical purposes, be divided into technical prob-
lems, those involving patient selection, reactions
of the immediate and acute type, short-term reac-
tions, and long-term complications.
513