CLINICAL REPORT

Evaluation for Bleeding Disorders in Suspected ChildAbuse

abstractBruising or bleeding in a child can raise the concern for child abuse.Assessing whether the findings are the result of trauma and/orwhether the child has a bleeding disorder is critical. Many bleedingdisorders are rare, and not every child with bruising/bleeding concern-ing for abuse requires an evaluation for bleeding disorders. In someinstances, however, bleeding disorders can present in a manner sim-ilar to child abuse. The history and clinical evaluation can be used todetermine the necessity of an evaluation for a possible bleeding dis-order, and prevalence and known clinical presentations of individualbleeding disorders can be used to guide the extent of the laboratorytesting. This clinical report provides guidance to pediatricians andother clinicians regarding the evaluation for bleeding disorders whenchild abuse is suspected. Pediatrics 2013;131:e1314–e1322

INTRODUCTION

Children often present for medical care with bleeding or bruising thatcan raise a concern for child abuse. Most commonly, this occurs withcutaneous bruises and intracranial hemorrhage (ICH), but otherpresentations, such as hematemesis,1 hematochezia,2 and oronasalbleeding can be caused by child abuse and/or bleeding disorders.3–7

When bleeding or bruising is suspicious for child abuse, carefulconsideration of medical and other causes is warranted. The in-appropriate diagnosis of child abuse could occur,8–10 potentiallyresulting in the removal of a child from a home and/or the potentialprosecution of an innocent person. Conversely, attributing an abusiveinjury to medical causes or accidental injury puts a child at risk forfuture abuse and possible death.11 Laboratory evaluations should beconducted with the understanding that the presence of a bleedingdisorder does not rule out abuse as the etiology for bruising orbleeding.9 Similarly, the presence of a history of trauma (accidental ornonaccidental) does not exclude the presence of a bleeding disorderor other medical condition. This clinical report provides guidance topediatricians and other clinicians regarding the evaluation forbleeding disorders when child abuse is suspected (Fig 1).

James D. Anderst, MD, MS, Shannon L. Carpenter, MD, MS,Thomas C. Abshire, MD and the SECTION ON HEMATOLOGY/ONCOLOGY and COMMITTEE ON CHILD ABUSE AND NEGLECT

KEY WORDSintracranial hemorrhage, inherited coagulation disorders,bruising, nonaccidental trauma

ABBREVIATIONSaPTT—activated partial thromboplastin timeDIC—disseminated intravascular coagulationICH—intracranial hemorrhageITP—immune thrombocytopeniaPFA-100—platelet function analyzerPT—prothrombin timeVKDB—vitamin K deficiency bleedingVWD—von Willebrand disease

This document is copyrighted and is property of the AmericanAcademy of Pediatrics and its Board of Directors. All authorshave filed conflict of interest statements with the AmericanAcademy of Pediatrics. Any conflicts have been resolved througha process approved by the Board of Directors. The AmericanAcademy of Pediatrics has neither solicited nor accepted anycommercial involvement in the development of the content ofthis publication.

The guidance in this report does not indicate an exclusivecourse of treatment or serve as a standard of medical care.Variations, taking into account individual circumstances, may beappropriate.

Accepted for publication Jan 23, 2013

www.pediatrics.org/cgi/doi/10.1542/peds.2013-0195

doi:10.1542/peds.2013-0195

All clinical reports from the American Academy of Pediatricsautomatically expire 5 years after publication unless reaffirmed,revised, or retired at or before that time.

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).

Copyright © 2013 by the American Academy of Pediatrics

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ASSESSING THE NEED FORA LABORATORY EVALUATION FORBLEEDING DISORDERS

The age and developmental capa-bilities of the child, history of trauma,the location and pattern of bruising,and, in the case of ICH, findings onneuroimaging should be consideredwhen assessing children with bruising/bleeding for possible abuse.12–18 Addi-tionally, a medical history of symptomssuggestive of a bleeding disorder, suchas significant bleeding after a circum-cision or other surgery, epistaxis,bleeding from the umbilical stump, or

excessive bleeding after dental proce-dures, increases the possibility ofa bleeding disorder. Family history ofa specific bleeding disorder or ethnic-ity of a population with higher ratesof a certain bleeding disorder (eg,Amish) might necessitate testing forthat condition. The child’s medicationsshould be documented, because cer-tain drugs can affect the results ofsome tests that might be used todetect bleeding disorders, such asthe platelet function analyzer (PFA-100; Siemens Healthcare Diagnostics,Tarrytown, NY) and platelet aggrega-tion testing. Caregivers might state

that their child “bruises easily.” Thesestatements are difficult to assess dur-ing an evaluation for possible abuse,as they can be a sign of a bleedingdisorder, a reflection of the child’s(fair) skin tone, or a fabrication tomask abuse. Children who are verbaland capable of providing a historyshould be interviewed away from po-tential offending caregivers, if possible.A thorough physical examinationshould include an evaluation of areasof bruising that have higher specificityfor abuse,14 such as the buttocks, ears,and genitals.

Any bleeding disorder can cause cu-taneous bruising, and sometimes thisbruising can be mild, can appear inlocations that are considered suspi-cious for abuse,19 and can appear atany age. Given the extreme rarity ofsome bleeding disorders, it is notreasonable to perform extensive lab-oratory testing for bleeding disordersin every child. In some cases, theconstellation of findings, taken inconjunction with the clinical historyand physical examination, can be sostrongly consistent with an abusiveinjury that further laboratory in-vestigation for medical conditions isnot warranted. For instance, a childwith a patterned slap mark whodescribes being hit with an open handdoes not require a laboratory evalua-tion for a bleeding disorder.

In addition to bleeding disorders, thepossibility of other medical causes ofeasy bruising or bleeding, such asEhlers-Danlos syndrome, scurvy, can-cer and other infiltrative disorders,glutaric aciduria, and arteriovenousmalformations, should be assessed,as should a history of use of anymedications or alternative therapiesthat may increase bleeding/bruising.Comprehensive descriptions of medi-cal conditions that could be confusedwith child abuse and alternativetherapies that may predispose to

FIGURE 1Recommended pathway for evaluation of possible bleeding disorders when child abuse is suspected.VWF, von Willebrand factor.

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bleeding/bruising are beyond the scopeof this report and can be found else-where.20,21 Results of the history, re-view of systems, physical examination,and, in the case of ICH, neuroimagingare generally adequate to excludethese conditions. When there are con-cerns that a medical condition mightbe the cause of bruising or bleeding,the evaluation for the conditions inquestion should occur simultaneouslywith the evaluation for abuse.

Bruising

In the absence of independently wit-nessed accidental trauma or a knownmedical cause, any bruising in a non-mobile child is highly concerning forabuse and necessitates an evalua-tion for child abuse.12–15 Additionally,bruising in a young infant could alsobe the first presentation of a bleedingdisorder.19 As such, a simultaneousevaluation for bleeding disorders isrecommended in these cases. In mo-bile children, the locations and pat-terns of the bruising can be used toassess for the possibility of abuse(Table 1).

In cases of bruising, the assessment ofthe need for an evaluation for bleedingdisorders should focus on the following:

� the specific history offered to ex-plain the bruising;

� the nature and location of bruis-ing; and

� mobility and developmental statusof the child.

The following factors generally excludethe need for an evaluation fora bleeding disorder:

� the caregivers’ description oftrauma sufficiently explains thebruising;

� the child or an independent wit-ness is able to provide a historyof abuse or nonabusive traumathat explains the bruising; or

� abusive object or hand-patternedbruising is present.

The injury history offered by care-givers might be purposefully mis-leading if the caregivers have causedthe bruising by abusive means.

In nonmobile infants, bleeding dis-orders can present with bruising orpetechiae in sites of normal handlingor pressure. Examples of this includethe following:

� petechiae at clothing line pressuresites;

� bruising at sites of object pres-sure, such as in the pattern andlocation of infant seat fasteners;and

� excessive diffuse bleeding if thechild has a severe bleeding disor-der.

Absence of these examples does notrule out a bleeding disorder; however,their presence might increase theprobability of a bleeding disorder.

ICH

Excepting obvious known trauma, ICHin a nonmobile child is highly con-cerning for child abuse. Children can

suffer ICH, such as a small subdural oran epidural hematoma underlyinga site of impact, from a short fall;however, short falls rarely result insignificant brain injury.16 Birth traumaand some medical conditions can alsoresult in ICH in infants. Consultationwith a child abuse pediatrician shouldbe considered in complex or con-cerning cases.

No studies have systematically com-pared the presentation, clinical find-ings, patterns of ICH, or presence ofretinal hemorrhages found in childrenwith bleeding disorders with thosefound in children in whom abusivehead trauma is diagnosed. However,bleeding disorders can cause ICH inany part of the cranial contents, andup to 12% of children and young adultswith bleeding disorders have hadICH at some time.22,23 Children withICH concerning for abuse require anevaluation for bleeding disorders.Exceptions to required evaluation caninclude the following:

� Independently witnessed or verifi-able trauma (abusive or nonabu-sive),

� Other findings consistent withabuse, such as fractures, burns,or internal abdominal trauma.

Other Bleeding Symptoms

Children with conditions such ashematemesis, hematochezia, or oro-nasal bleeding as presenting symp-toms should be evaluated on a case-by-case basis for possible abuse,particularly child abuse in a medicalsetting. Medical conditions and/orchild abuse can cause these findings.

BLEEDING DISORDERS ANDEXTENT OF EVALUATION

Bleeding disorders that can producepatterns of bruising or bleeding thatmimic abuse include coagulation fac-tor deficiencies/abnormalities, fibrinolytic

TABLE 1 Suspicion of Child Abuse inAmbulatory Children on the Basisof Characteristics of Bruises14,15,17

Less Suspiciousfor Child Abuse

More Suspiciousfor Child Abuse

Forehead LocationUnder chin FaceElbows EarsLower arms NeckHips Upper armsShins TrunkAnkles Hands

GenitaliaButtocksAnterior, medial thighs

PatternSlap or hand marksObject marksBite marksBruises in clustersMultiple bruises of

uniform shapeLarge cumulative size

of bruising

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defects, defects of fibrinogen, andplatelet disorders. Table 2 containsa listing of the most common bleedingdisorders in children and character-istics of potential testing strategiesfor each disorder. Most factor defi-ciencies can be detected by the pro-thrombin time (PT) and activatedpartial thromboplastin time (aPTT);however, von Willebrand disease(VWD) and factor XIII deficiency arenot reliably detected by these screen-ing tests. Additionally, mild deficienciesin factor VIII or factor IX (mild hemo-philia) might not cause abnormalitiesin the aPTT but might still result insignificant bleeding, including ICH,particularly after mild trauma. Fibri-nolytic defects can cause significantbleeding/bruising but are extremelyrare and require specific testing.Defects of fibrinogen are also rareand can be detected by the fibrinogenconcentration and thrombin time.

The prevalence of mild platelet dis-orders is unknown, and testing formild platelet disorders is challenging.The most common clinical pre-sentations include bruising and mu-cocutaneous bleeding. The prevalenceof ICH in mild platelet disorders isunknown but is likely to be low. Plateletaggregation testing, best performedby a pediatric hematologist, requiresa relatively large volume of blood, andinterpretation of the test resultrequires a specialist.25 A PFA-100 canscreen for many platelet functiondisorders, including more severetypes, such as Bernard Soulier syn-drome and Glanzmann thrombasthe-nia, as well as many types of VWD.However, the PFA-100 is not an ef-fective screen for some types of VWDand milder platelet abnormalities.Individual patient characteristics,such as hematocrit, platelet count,pregnancy, age, multisystem trauma,sepsis, and medications, can affectthe results of the PFA-100. Accurate

diagnosis often requires additionaltesting, such as specific von Wille-brand testing or platelet aggregation;therefore, many centers have de-creased or ceased use of the PFA-100.25,26 Assessment of the results ofa PFA-100 and the need for furthertesting are best accomplished inconsultation with a pediatric hema-tologist.

Vitamin K Deficiency

Vitamin K deficiency in infants canresult in bleeding in the skin or frommucosal surfaces from circumcision,generalized ecchymoses, large in-tramuscular hemorrhages, or ICH.Because of the widespread provisionof vitamin K at birth, vitamin K de-ficiency bleeding (VKDB) is rare;however, not all states require vitaminK to be administered at birth, andsome medical conditions predisposeto VKDB.24 In VKDB, there is a pro-longed PT and possibly aPTT for age. Inpatients who have already receivedvitamin K, fresh-frozen plasma, orspecific factor replacement as treat-ment, measurement of proteins in-duced by vitamin K absence canconfirm the diagnosis.27,28

Coagulation Tests in Cases ofBruising

The initial screening panel in a patientwho presents with bruising eval-uates for conditions with a knownprevalence more common than 1 per500 000 people, including idiopathicthrombocytopenic purpura, all factordeficiencies (except factor XIII de-ficiency), and VWD (Fig 1). It does notevaluate for extremely rare con-ditions, including factor XIII deficiency,defects of fibrinogen, and fibrinolyticdefects. This strategy also does notscreen for extremely rare plateletdisorders, such as Glanzmann throm-basthenia, and more common butrelatively more difficult to detect

platelet disorders, such as plateletstorage pool disorders. If test resultsare abnormal or expanded/detailedtesting is necessary or preferred,consultation with a pediatric hema-tologist is recommended.

In many circumstances, children withbruising that is suspicious for abusemay be removed from a potentiallydangerous setting where the abuselikely occurred. A thorough physi-cal examination performed in theweeks after removal that revealsminimal bruising and/or bruising onlyin locations of common accidentalbruises is supportive of abuse as thecause of the original suspiciousbruising. Each case must be evaluatedindividually, however, considering thetotality of findings, and with the un-derstanding that the need for safetymust be balanced with the emotionaltrauma of removing a child from his orher home. Bleeding disorders aregenerally permanent conditions thatdo not result in abatement aftera change in caregivers. One exceptionto this is immune thrombocytopenia(ITP), which is a transient, often self-resolving bleeding disorder. Screen-ing for ITP (platelet count) is necessaryat the time of presentation withbruises.

Determining the Need for a Test:The Medical Probability

Specific data regarding the prevalenceof bleeding disorders in the populationof children with ICH or subdural he-matoma is not available. However,there are data regarding the proba-bility of specific bleeding disorders tocause ICH. If the prevalence of a con-dition and the frequency of a particu-lar presentation of that condition areknown, a physician can construct theprobability of that specific condition(bleeding disorder) resulting in thespecific presentation (ICH). The pres-ence of “classic” bleeding symptoms,such as bleeding after circumcision,

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TABLE2

Common

TestingStrategies

forBleeding

Disorders

Condition

Frequency

Inheritance

ScreeningTests

SnandSp,%

PPVandNPV,%

Confirm

atoryTest

Factor

abnorm

alities/deficiencies

VWDtype

11/1000

ADPFA-100

Sn=79–96

aPPV=93.3

VWAg

b

VWFactivity

Sp=88–96

aNPV=98.2

VWmultim

eranalysis

Factor

VIIIactivity

VWDtype

2AUncommon

ADor

ARPFA-100

Sn=94–100a

PPV=93.3

VWAg

b

VWFactivity

Sp=88–96

aNPV=98.2

VWmultim

eranalysis

Factor

VIIIactivity

VWDtype

2BUncommon

ADPFA-100

Sn=93–96

aPPV=93.3

VWAg

b

VWFactivity

Sp=88–96

aNPV=98.2

VWmultim

eranalysis

Factor

VIIIactivity

VWDtype

2MUncommon

ADor

ARPFA-100

Sn=94–97

aPPV=93.3

VWAg

b

VWFactivity

Sp=88–96

aNPV=98.2

VWmultim

eranalysis

Factor

VIIIactivity

VWDtype

2NUncommon

AR,orcompound

heterozygote

aPTT

NANA

VWF-Factor

VIIIbindingassay

VWDtype

31/300000–1000000

AR,orcompound

heterozygote

PFA-100

Sn=94–100a

PPV=93.3

VWAg

b

Ristocetin

cofactor

Sp=88–96

aNPV=98.2

VWFmultim

eranalysis

Factor

VIIIactivity

Factor

IIdeficiency

(prothrombin)

26reported

cases,estim

ated

1/1–2million

aPTT,PT(m

aybe

norm

al)

Sn=variable

NAFactor

IIactivity

±antigen

levels

Factor

Vdeficiency

1/1million

ARaPTT,PT

Sn=variable

NAFactor

Vactivity

CombinedFactor

V/Factor

VIIIdeficiency

1/1million

ARaPTT>PT

Sn=variable

NAFactor

Vandfactor

VIIIactivities

Factor

VIIdeficiency

1/300000–500000

ARPT

Sn=variable

NAFactor

VIIactivity

Factor

VIIIdeficiency

1/5000

malebirths

X-linked

aPTT

Sn=variable

NAFactor

VIIIactivity

Factor

IXdeficiency

1/20

000malebirths

X-linked

aPTT

Sn=variable

NAFactor

IXactivity

Factor

Xdeficiency

1/1million

ARaPTT,PT,RVV

Sn=variable

NAFactor

Xactivity

Factor

XIdeficiency

1/100000

ARaPTT

Sn=variable

NAFactor

XIactivity

Factor

XIIIdeficiency

1/2–5million

ARClot

solubility

Sn=variable

NAFactor

XIIIactivity

Fibrinolyticdefects

α-2antiplasm

indeficiency

∼40

reported

cases

AREuglobin

lysistest

Sn=variable

NAα-2antiplasm

inactivity

PAI-1

deficiency

Very

rare

ARSn

=variable

NAPAI-1antigen

andactivity

Defectsof

fibrinogen

Afibrinogenem

ia1/500000

ARPT,aPTT

Sn=high

NAFibrinogen

level

Hypofibrinogenem

iaLess

than

afibrinogenem

iaPT,aPTT

Sn=variable

NAThrombintim

e,fibrinogen

activity

Dysfibrinogenem

ia1/million

Thrombintim

e,fibrinogen

level

Sn=variable

NAThrombintim

e,fibrinogen

antigen

andactivity

levelcomparison,

reptilase

time

Platelet

disorders

ITP

Age-related

NACBC

Sn=high

NAAntiplateletAb

(rarelyneeded)

Glanzm

annthrombasthenia

Very

rare

ARPFA-100

Sn=97–100

NAPlatelet

aggregationtestingFlow

cytometry

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umbilical stump bleeding, joint hem-orrhage, and excessive soft tissuebleeding, increase the probability fora bleeding disorder; however, thesefindings are neither sensitive norspecific for bleeding disorders.

Coagulation Tests in the Setting ofICH

For bleeding disorders that cause ICH,the prevalence of the bleeding disor-der and the prevalence of ICH inpatients with each specific bleedingdisorder can be used to construct theprobability of the specific bleedingdisorder to cause ICH (Table 3). Someprobabilities are so low as to pre-clude calculation. Testing for theseconditions is likely not useful. Mildhemophilia, which might be missed ifonly an aPTT test is ordered, can bedetected by measuring specific levelsof factor VIII and factor IX. Mild he-mophilia can result in ICH, particularlyafter mild trauma, and because of therelatively high prevalence of the con-dition, the probability of mild factorVIII deficiency causing or contribut-ing to ICH is 1 in 280 000 males. Inpopulations with a high prevalenceof factor XI deficiency, such as theAshkenazi Jewish population, it might bereasonable to measure factor XI level.

Clinical and historical information canbe used to determine the need fortesting in children with isolated ICHconcerning for abuse (Fig 1). The ini-tial testing panel for ICH evaluates forconditions for which the probabilityfor the condition resulting in ICH isgreater than 1 per 5 million. The panelincludes testing for most factor defi-ciencies and afibrinogenemia. Thisscreening panel does not test forfactor XIII deficiency, VWD, fibrinolyticdefects, hypofibrinogenemia, and dys-fibrinogenemia. These conditions ei-ther have not been associated withICH or they are so rarely the cause ofICH that testing for the conditions is

not reasonable. Additionally, the initialscreening panel evaluates for dis-seminated intravascular coagulation(DIC). Because DIC can cause any typeof bruising/bleeding, including ICH,the finding of DIC in the context ofsuspected child abuse could signifi-cantly change the clinical approach toa patient. In children with DIC andbleeding symptoms as the only findingconcerning for abuse, considerationmust be given to the multitude ofprimary causes of DIC, includingtrauma, sepsis, and primary bleedingdisorders, among many others.

Many children with ICH suspicious forabuse, if they survive, are placed in safesettings after hospital discharge. Inthese cases, testing for bleeding dis-orders can be deferred to a later date,with the exception of ITP. If bloodproducts have been given to the patient,as can happen in severe ICH, the de-finitive evaluation for bleeding dis-orders should be postponed until thetransfused blood components are nolonger in the patient’s system (Table 4).Assistance from a pediatric hematolo-gist should be considered in address-ing the possibility of factor deficienciesafter a transfusion has occurred.

Many aspects of bleeding disordersare under investigation, and thus,changes in the understanding of theprevalence and severity of certainbleeding symptoms related to thesedisorders should be expected. Forexample, although hemophilia A and Bare X-linked diseases and, therefore,typically thought to affect only maleindividuals, 25% to 50% of femalecarriers of hemophilia report excessbleeding; therefore, measurement offactor VIII and IX levels in femalepatients should be considered.29 Inaddition, the population prevalenceand/or clinical effects of mild plateletfunction disorders continue to be studied.In a patient with mucocutaneous symp-toms, particularly if petechiae areTA

BLE2

Continued

Condition

Frequency

Inheritance

ScreeningTests

SnandSp,%

PPVandNPV,%

Confirm

atoryTest

BernardSouliersyndrome

Rare

ARPFA-100

Sn=100

NAPlatelet

aggregationtestingFlow

cytometry

Platelet

release/storagedisorders

Unknow

n,morecommon

than

otherplatelet

functiondisorders

variable

PFA-100

Sn=27–50

NAPlatelet

aggregationandsecretion

Electron

microscopy

Molecular

andcytogenetic

testing

AD,autosom

aldominant;AR,autosom

alrecessive;CBC,completebloodcell(count);NA,not

availableor

notapplicable;NPV,negativepredictivevalue;PAI-1,plasm

inogen

activator

inhibitor-1;PPV,positivepredictivevalue;RVV,Russellviper

venom

(test);S

n,sensitivity;S

p,specificity;VW,von

Willebrand;VWAg,von

Willebrand

antigen;VWF,vonWillebrand

factor

Ab,antibody.

aValues

derivedfrom

data

before

2008

NationalInstitutesof

Health

Consensusguidelines.S

nandSp

usingcurrentdiagnosticcutoffs

unknow

nbutwould

beexpected

tohave

higher

Spwith

lower

Sn.

bMay

bereasonable

toproceeddirectlyto

diagnostictestingdependingon

availability.Seeaccompanyingtechnicalreport

fordetaileddiscussion.24

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present, platelet aggregation testingshould be considered.25 Finally, be-cause von Willebrand factor is anacute phase reactant, its levels canvary in response to clinical status,resulting in falsely elevated results.Many times, testing must be repeatedup to 3 times to ensure reliableresults.30 If significant concern forVWD exists, consultation with a pedi-atric hematologist is suggested.

When Testing Indicates a PossibleBleeding Disorder in the Context ofan Abuse Evaluation

Positive laboratory test results requirefurther evaluation for the possibility offalse-positive results and/or the ne-

cessity for further testing. Pro-longation of the PTand aPTT because ofparenchymal damage has been notedin abusive head trauma and should notautomatically be interpreted as evi-dence of a primary bleeding disor-der.31 Additionally, consideration mustbe given to the likelihood of a preex-isting bleeding disorder as the pri-mary cause of a child’s bleeding/bruising. For example, given the rela-tively high prevalence of VWD, it isinevitable that some children withVWD will be abused and present withbleeding/bruising symptoms. Deter-mining the causative factor in thesesituations is challenging. Bruising isa common finding in VWD. If a childhas test results consistent with VWDand bruising concerning for abuse,a short-term change in home settingmay be considered, understandingthe cautions needed when using thisapproach. Only a few case reportshave attributed ICH to VWD. Mostreported ICH in children with VWDwould not be confused with typicalabusive ICH.32–34 Given the rarity ofICH in VWD, particularly spontaneousICH, testing consistent with VWD doesnot mean that ICH is definitively at-tributable to VWD, and abuse muststill be considered.

Interpretation of Tests

It should be noted that the aPTT can befalsely prolonged in certain circum-stances, such as in the presence ofa lupus anticoagulant, or can be pro-longed and might not indicate a truebleeding disorder, such as in factor XIIdeficiency or other contact factordeficiencies. In addition, patients whoexperience a traumatic brain injuryoften have a transient coagulopathythat does not reflect an underlyingcongenital disorder.31,35 Coagulationtests are very sensitive to specimenhandling and should be performed inlaboratories experienced with theseassays. Inappropriate handling com-monly leads to false-positive results.

CONCLUSIONS

Children who present with bleedingand bruising symptoms that are con-cerning for abuse require carefulevaluation for the potential of bleedingdisorders as a cause. No single panelof tests rules out every possiblebleeding disorder. Given the rarity ofmost bleeding disorders and thepossible presence of specific clinicalfactors that decrease the likelihood ofa bleeding disorder causing a child’sfindings, in many situations, exten-sive laboratory evaluation is not

TABLE 3 Probabilities for Congenital Coagulopathies Causing ICHa

Condition Prevalence of Condition,Upper Limits

Prevalence of ICH, Upper Limits Probabilityb

VWD 1/1000 Extremely rare LowFactor II deficiency 1/1 million 11% 1/10 millionFactor V deficiency 1/1 million 8% of homozygotes 1/10 million homozygotesCombined factors V and VIII deficiencies 1/1 million 2% 1/50 millionFactor VII deficiency 1/300 000 4%–6.5% 1/5 millionFactor VIII deficiency 1/5000 males 5%–12% 1/50 000 malesFactor IX deficiency 1/20 000 males 5%–12% 1/200 000 malesFactor X deficiency 1/1 million 21% 1/5 millionFactor XI deficiency 1/100 000 Extremely rare LowFactor XIII deficiency 1/2 million 33% 1/6 millionα-2 antiplasmin deficiency 40 cases reported Not reported LowPlasminogen activator inhibitor-1 deficiency Extremely rare Common LowAfibrinogenemia 1/500 000 10% 1/5 millionDysfibrinogenemia 1/1 million Single case report Lowa The probability of having a specific bleeding disorder increases in the setting of a family history of that specific named bleeding disorder or if the patient is from an ethnicity in whicha specific bleeding disorder is more common (eg, Ashkenazi Jewish people and factor XI deficiency).b“Probability” indicates the probability that an individual in the general population would have the following specific coagulopathy causing an ICH.

TABLE 4 Half-Lives of Coagulation Factors

Factor Half-Life Postinfusion, h

Fibrinogen 96–150II 60V 24VII 4–6VIII 11–12IX 22X 35XI 60XIII 144–300VWF 8–12

VWF, von Willebrand factor.Reprinted with permission from Goodnight S, HathawayW. Disorders of Hemostasis and Thrombosis: A ClinicalGuide. 2nd ed. New York, NY: McGraw-Hill Professional;2001:497.

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necessary. If a laboratory evaluation isconducted, tests should be chosen onthe basis of the prevalence of thecondition, patient and family history,ease of testing, blood volume requiredfor testing, and, in the case of ICH,probability of a bleeding disordercausing ICH. Further consultation witha pediatric hematologist is recom-mended if specific, expanded testingis necessary, if preliminary testingsuggests the presence of a bleedingdisorder, if testing to rule out a spe-cific bleeding disorder is needed, or iftesting for very rare conditions ispreferred.

GUIDANCE FOR PEDIATRICIANS

In children who have bruising orbleeding that is suspicious for abuse,

1. Complete medical, trauma, andfamily histories and a thoroughphysical examination are criticaltools in evaluating for the possibil-ity of abuse or medical conditionsthat predispose to bleeding/bruis-ing.

2. In each case, careful considera-tion of the possibility of a medicalcondition causing the bleeding/bruising is essential. Specific ele-ments of the history and character-istics of the bleeding/bruising canbe used to determine the need fora laboratory evaluation for bleed-ing disorders.

3. If the evaluation indicates a needfor laboratory testing for bleedingdisorders, initial testing is focused

on the prevalence of the conditionand potential of each specific con-dition to cause the specific findingsin a given child (Fig 1).

4. Laboratory testing suggestive or in-dicating the presence of a bleed-ing disorder does not eliminateabuse from consideration. In chil-dren with bruising and laboratorytesting suggestive of a bleedingdisorder, a follow-up evaluation af-ter a change in home setting canprovide valuable information re-garding the likelihood of a bleedingdisorder causing the concerningfindings.

5. Children with ICH often receiveblood product transfusions. It issuggested that screening for bleed-ing disorders in these patients bedelayed until elimination of thetransfused blood clotting elements.

6. The discovery of new informationregarding condition prevalence,laboratory testing, and clinical pre-sentations of bleeding disordersis to be expected. Close collabora-tion with a pediatric hematologistis necessary to ensure the mostcurrent evaluation and testingmethods.

LEAD AUTHORSJames D. Anderst, MD, MSShannon L. Carpenter, MD, MSThomas C. Abshire, MD

SECTION ON HEMATOLOGY/ONCOLOGYEXECUTIVE COMMITTEE, 2012–2013Jeffrey Hord, MD, ChairpersonGary Crouch, MD

Gregory Hale, MDBrigitta Mueller, MDZora Rogers, MDPatricia Shearer, MDEric Werner, MD, Immediate Past Chairperson

FORMER EXECUTIVE COMMITTEEMEMBERSStephen Feig, MDEric Kodish, MDAlan Gamis, MD

LIAISONSEdwin Forman, MD — Alliance for ChildhoodCancer

CONSULTANTShannon Carpenter, MD, MSThomas Abshire, MD

STAFFSuzanne Kirkwood, MS

COMMITTEE ON CHILD ABUSE ANDNEGLECT, 2012–2013Cindy W. Christian, MD, ChairpersonJames Crawford-Jakubiak, MDEmalee Flaherty, MDJohn M. Leventhal, MDJames Lukefahr, MDRobert Sege, MD PhD

LIAISONSHarriet MacMillan, MD— American Academy ofChild and Adolescent PsychiatryCatherine Nolan, MSW — ACSW, Administrationfor Children, Youth, and Families, Office onChild Abuse and NeglectJanet Saul, PhD — Centers for Disease Controland Prevention

CONSULTANTJames Anderst, MD, MS

STAFFTammy Piazza HurleySonya Clay

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