Evaluation for Bleeding Disorders in Suspected Child Abuse Abstract

Guidance for the Clinician in Rendering Pediatric Care

CLINICAL REPORT Evaluation for Bleeding Disorders in Suspected Child Abuse

James D. Anderst, MD, MS, Shannon L. Carpenter, MD, MS, abstract Thomas C. Abshire, MD and the SECTION ON HEMATOLOGY/ Bruising or bleeding in a child can raise the concern for child abuse. ONCOLOGY and COMMITTEE ON CHILD ABUSE AND NEGLECT Assessing whether the findings are the result of trauma and/or KEY WORDS intracranial hemorrhage, inherited coagulation disorders, whether the child has a bleeding disorder is critical. Many bleeding bruising, nonaccidental trauma disorders are rare, and not every child with bruising/bleeding concern- ABBREVIATIONS ing for abuse requires an evaluation for bleeding disorders. In some aPTT—activated partial thromboplastin time instances, however, bleeding disorders can present in a manner sim- DIC—disseminated intravascular coagulation — ilar to child abuse. The history and clinical evaluation can be used to ICH intracranial hemorrhage ITP—immune thrombocytopenia determine the necessity of an evaluation for a possible bleeding dis- PFA-100—platelet function analyzer order, and prevalence and known clinical presentations of individual PT—prothrombin time bleeding disorders can be used to guide the extent of the laboratory VKDB—vitamin K deficiency bleeding VWD—von Willebrand disease testing. This clinical report provides guidance to pediatricians and This document is copyrighted and is property of the American other clinicians regarding the evaluation for bleeding disorders when Academy of Pediatrics and its Board of Directors. All authors child abuse is suspected. Pediatrics 2013;131:e1314–e1322 have filed conflict of interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any INTRODUCTION commercial involvement in the development of the content of this publication. Children often present for medical care with bleeding or bruising that The guidance in this report does not indicate an exclusive can raise a concern for child abuse. Most commonly, this occurs with course of treatment or serve as a standard of medical care. cutaneous bruises and intracranial hemorrhage (ICH), but other Variations, taking into account individual circumstances, may be presentations, such as hematemesis,1 hematochezia,2 and oronasal appropriate. bleeding can be caused by child abuse and/or bleeding disorders.3–7 When bleeding or bruising is suspicious for child abuse, careful consideration of medical and other causes is warranted. The inappropriate diagnosis of child abuse could occur,8–10 potentially resulting in the removal of a child from a home and/or the potential prosecution of an innocent person. Conversely, attributing an abusive injury to medical causes or accidental injury puts a child at risk for future abuse and possible death.11 Laboratory evaluations should be conducted with the understanding that the presence of a bleeding disorder does not rule out abuse as the etiology for bruising or bleeding.9 Similarly, the presence of a history of trauma (accidental or Accepted for publication Jan 23, 2013 nonaccidental) does not exclude the presence of a bleeding disorder www.pediatrics.org/cgi/doi/10.1542/peds.2013-0195 or other medical condition. This clinical report provides guidance to doi:10.1542/peds.2013-0195 pediatricians and other clinicians regarding the evaluation for All clinical reports from the American Academy of Pediatrics bleeding disorders when child abuse is suspected (Fig 1). automatically 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

e1314 FROM THE AMERICAN ACADEMY OF PEDIATRICS Downloaded from www.aappublications.org/news by guest on September 26, 2021 FROM THE AMERICAN ACADEMY OF PEDIATRICS

that their child “bruises easily.” These statements are difficult to assess dur- ing an evaluation for possible abuse, as they can be a sign of a bleeding disorder, a reflection of the child’s (fair) skin tone, or a fabrication to mask abuse. Children who are verbal and capable of providing a history should be interviewed away from potential offending caregivers, if possible. A thorough physical examination should include an evaluation of areas of bruising that have higher specificity for abuse,14 such as the buttocks, ears, and genitals. Any bleeding disorder can cause cutaneous bruising, and sometimes this bruising can be mild, can appear in locations that are considered suspicious for abuse,19 and can appear at any age. Given the extreme rarity of some bleeding disorders, it is not reasonable to perform extensive laboratory testing for bleeding disorders in every child. In some cases, the constellation of findings, taken in conjunction with the clinical history and physical examination, can be so strongly consistent with an abusive injury that further laboratory in- FIGURE 1 vestigation for medical conditions is Recommended pathway for evaluation of possible bleeding disorders when child abuse is suspected. not warranted. For instance, a child VWF, von Willebrand factor. with a patterned slap mark who describes being hit with an open hand does not require a laboratory evalua- ASSESSING THE NEED FOR excessive bleeding after dental proce- A LABORATORY EVALUATION FOR dures, increases the possibility of tion for a bleeding disorder. BLEEDING DISORDERS a bleeding disorder. Family history of In addition to bleeding disorders, the aspecific bleeding disorder or ethnic- possibility of other medical causes of The age and developmental capa- ity of a population with higher rates easy bruising or bleeding, such as bilities of the child, history of trauma, of a certain bleeding disorder (eg, Ehlers-Danlos syndrome, scurvy, can- the location and pattern of bruising, Amish) might necessitate testing for cer and other infiltrative disorders, and, in the case of ICH, findings on that condition. The child’smedications glutaric aciduria, and arteriovenous neuroimaging should be considered should be documented, because cer- malformations, should be assessed, when assessing children with bruising/ tain drugs can affect the results of as should a history of use of any – bleeding for possible abuse.12 18 Addi- some tests that might be used to medications or alternative therapies tionally, a medical history of symptoms detect bleeding disorders, such as that may increase bleeding/bruising. suggestive of a bleeding disorder, such the platelet function analyzer (PFA- Comprehensive descriptions of medi- as significant bleeding after a circum- 100; Siemens Healthcare Diagnostics, cal conditions that could be confused cision or other surgery, epistaxis, Tarrytown, NY) and platelet aggrega- with child abuse and alternative bleeding from the umbilical stump, or tion testing. Caregivers might state therapies that may predispose to

PEDIATRICS Volume 131, Number 4, April 2013 e1315 Downloaded from www.aappublications.org/news by guest on September 26, 2021 bleeding/bruising are beyond the scope In cases of bruising, the assessment of suffer ICH, such as a small subdural or of this report and can be found else- the need for an evaluation for bleeding an epidural hematoma underlying where.20,21 Results of the history, re- disorders should focus on the following: a site of impact, from a short fall; view of systems, physical examination, the specific history offered to ex- however, short falls rarely result in and, in the case of ICH, neuroimaging plain the bruising; significant brain injury.16 Birth trauma are generally adequate to exclude and some medical conditions can also the nature and location of bruis- these conditions. When there are con- result in ICH in infants. Consultation ing; and cerns that a medical condition might with a child abuse pediatrician should be the cause of bruising or bleeding, mobility and developmental status be considered in complex or con- the evaluation for the conditions in of the child. cerning cases. question should occur simultaneously The following factors generally exclude No studies have systematically com- with the evaluation for abuse. the need for an evaluation for pared the presentation, clinical find- a bleeding disorder: ings, patterns of ICH, or presence of Bruising the caregivers’ description of retinal hemorrhages found in children In the absence of independently wit- trauma sufficiently explains the with bleeding disorders with those nessed accidental trauma or a known bruising; found in children in whom abusive medical cause, any bruising in a non- the child or an independent wit- head trauma is diagnosed. However, mobile child is highly concerning for ness is able to provide a history bleeding disorders can cause ICH in abuse and necessitates an evalua- of abuse or nonabusive trauma any part of the cranial contents, and tion for child abuse.12–15 Additionally, that explains the bruising; or up to 12% of children and young adults bruising in a young infant could also abusive object or hand-patterned with bleeding disorders have had fi 22,23 be the rst presentation of a bleeding bruising is present. ICH at some time. Children with disorder.19 As such, a simultaneous ICH concerning for abuse require an The injury history offered by care- evaluation for bleeding disorders is evaluation for bleeding disorders. givers might be purposefully mis- recommended in these cases. In mo- Exceptions to required evaluation can leading if the caregivers have caused bile children, the locations and pat- include the following: the bruising by abusive means. terns of the bruising can be used to Independently witnessed or verifi- assess for the possibility of abuse In nonmobile infants, bleeding dis- able trauma (abusive or nonabu- (Table 1). orders can present with bruising or sive), petechiae in sites of normal handling fi or pressure. Examples of this include Other ndings consistent with the following: abuse, such as fractures, burns, TABLE 1 Suspicion of Child Abuse in or internal abdominal trauma. Ambulatory Children on the Basis petechiae at clothing line pressure 14,15,17 of Characteristics of Bruises sites; Less Suspicious More Suspicious Other Bleeding Symptoms for Child Abuse for Child Abuse bruising at sites of object pressure, such as in the pattern and Children with conditions such as Forehead Location hematemesis, hematochezia, or oro- Under chin Face location of infant seat fasteners; Elbows Ears and nasal bleeding as presenting symp- Lower arms Neck toms should be evaluated on a case- excessive diffuse bleeding if the Hips Upper arms by-case basis for possible abuse, Shins Trunk child has a severe bleeding disor- particularly child abuse in a medical Ankles Hands der. Genitalia setting. Medical conditions and/or Buttocks Absence of these examples does not child abuse can cause these findings. Anterior, medial thighs rule out a bleeding disorder; however, Pattern their presence might increase the Slap or hand marks BLEEDING DISORDERS AND Object marks probability of a bleeding disorder. Bite marks EXTENT OF EVALUATION Bruises in clusters ICH Bleeding disorders that can produce Multiple bruises of uniform shape Excepting obvious known trauma, ICH patterns of bruising or bleeding that Large cumulative size in a nonmobile child is highly con- mimic abuse include coagulation fac- of bruising cerning for child abuse. Children can tor deficiencies/abnormalities, fibrinolytic

e1316 FROM THE AMERICAN ACADEMY OF PEDIATRICS Downloaded from www.aappublications.org/news by guest on September 26, 2021 FROM THE AMERICAN ACADEMY OF PEDIATRICS defects, defects of fibrinogen, and diagnosis often requires additional platelet disorders, such as platelet platelet disorders. Table 2 contains testing, such as specific von Wille- storage pool disorders. If test results a listing of the most common bleeding brand testing or platelet aggregation; are abnormal or expanded/detailed disorders in children and character- therefore, many centers have de- testing is necessary or preferred, istics of potential testing strategies creased or ceased use of the PFA- consultation with a pediatric hema- for each disorder. Most factor defi- 100.25,26 Assessment of the results of tologist is recommended. ciencies can be detected by the pro- a PFA-100 and the need for further In many circumstances, children with thrombin time (PT) and activated testing are best accomplished in bruising that is suspicious for abuse partial thromboplastin time (aPTT); consultation with a pediatric hema- may be removed from a potentially however, von Willebrand disease tologist. dangerous setting where the abuse (VWD) and factor XIII deficiency are likely occurred. A thorough physi- not reliably detected by these screen- Vitamin K Deficiency cal examination performed in the ing tests. Additionally, mild deficiencies Vitamin K deficiency in infants can weeks after removal that reveals in factor VIII or factor IX (mild hemo- result in bleeding in the skin or from minimal bruising and/or bruising only philia) might not cause abnormalities mucosal surfaces from circumcision, in locations of common accidental in the aPTT but might still result in generalized ecchymoses, large in- bruises is supportive of abuse as the significant bleeding, including ICH, tramuscular hemorrhages, or ICH. cause of the original suspicious particularly after mild trauma. Fibri- Because of the widespread provision bruising. Each case must be evaluated nolytic defects can cause significant of vitamin K at birth, vitamin K de- individually, however, considering the bleeding/bruising but are extremely ficiency bleeding (VKDB) is rare; totality of findings, and with the un- rare and require specific testing. however, not all states require vitamin derstanding that the need for safety Defects of fibrinogen are also rare K to be administered at birth, and must be balanced with the emotional and can be detected by the fibrinogen some medical conditions predispose trauma of removing a child from his or concentration and thrombin time. to VKDB.24 In VKDB, there is a pro- her home. Bleeding disorders are The prevalence of mild platelet dis- longed PT and possibly aPTT for age. In generally permanent conditions that orders is unknown, and testing for patients who have already received do not result in abatement after mild platelet disorders is challenging. vitamin K, fresh-frozen plasma, or a change in caregivers. One exception fi to this is immune thrombocytopenia The most common clinical pre- speci c factor replacement as treat- (ITP), which is a transient, often self- sentations include bruising and mu- ment, measurement of proteins in- resolving bleeding disorder. Screen- cocutaneous bleeding. The prevalence duced by vitamin K absence can fi 27,28 ing for ITP (platelet count) is necessary of ICH in mild platelet disorders is con rm the diagnosis. at the time of presentation with unknown but is likely to be low. Platelet bruises. aggregation testing, best performed Coagulation Tests in Cases of by a pediatric hematologist, requires Bruising Determining the Need for a Test: a relatively large volume of blood, and The initial screening panel in a patient The Medical Probability interpretation of the test result who presents with bruising eval- Specific data regarding the prevalence requires a specialist.25 A PFA-100 can uates for conditions with a known of bleeding disorders in the population screen for many platelet function prevalence more common than 1 per of children with ICH or subdural he- disorders, including more severe 500 000 people, including idiopathic matoma is not available. However, types, such as Bernard Soulier syn- thrombocytopenic purpura, all factor there are data regarding the proba- drome and Glanzmann thrombasthe- deficiencies (except factor XIII de- bility of specific bleeding disorders to nia, as well as many types of VWD. ficiency), and VWD (Fig 1). It does not cause ICH. If the prevalence of a con- However, the PFA-100 is not an ef- evaluate for extremely rare condition and the frequency of a particu- fective screen for some types of VWD ditions, including factor XIII deficiency, lar presentation of that condition are and milder platelet abnormalities. defects of fibrinogen, and fibrinolytic known, a physician can construct the Individual patient characteristics, defects. This strategy also does not probability of that specific condition such as hematocrit, platelet count, screen for extremely rare platelet (bleeding disorder) resulting in the pregnancy, age, multisystem trauma, disorders, such as Glanzmann throm- specific presentation (ICH). The pres- sepsis, and medications, can affect basthenia, and more common but ence of “classic” bleeding symptoms, the results of the PFA-100. Accurate relatively more difficult to detect such as bleeding after circumcision,

PEDIATRICS Volume 131, Number 4, April 2013 e1317 Downloaded from www.aappublications.org/news by guest on September 26, 2021 e1318 TABLE 2 Common Testing Strategies for Bleeding Disorders Condition Frequency Inheritance Screening Tests Sn and Sp, % PPV and NPV,% Conﬁrmatory Test

RMTEAEIA CDM FPEDIATRICS OF ACADEMY AMERICAN THE FROM Factor abnormalities/deﬁciencies VWD type 1 1/1000 AD PFA-100 Sn = 79–96a PPV = 93.3 VWAgb VWF activity Sp = 88–96a NPV = 98.2 VW multimer analysis Factor VIII activity VWD type 2A Uncommon AD or AR PFA-100 Sn = 94–100a PPV = 93.3 VWAgb VWF activity Sp = 88–96a NPV = 98.2 VW multimer analysis Factor VIII activity

Downloaded from VWD type 2B Uncommon AD PFA-100 Sn = 93–96a PPV = 93.3 VWAgb VWF activity Sp = 88–96a NPV = 98.2 VW multimer analysis Factor VIII activity VWD type 2M Uncommon AD or AR PFA-100 Sn = 94–97a PPV = 93.3 VWAgb VWF activity a www.aappublications.org/news Sp = 88–96 NPV = 98.2 VW multimer analysis Factor VIII activity VWD type 2N Uncommon AR, or compound aPTT NA NA VWF-Factor VIII binding assay heterozygote VWD type 3 1/300 000–1 000 000 AR, or compound PFA-100 Sn = 94–100a PPV = 93.3 VWAgb heterozygote Ristocetin cofactor Sp = 88–96a NPV = 98.2 VWF multimer analysis Factor VIII activity Factor II deﬁciency (prothrombin) 26 reported cases, estimated aPTT, PT (may be normal) Sn = variable NA Factor II activity ± antigen levels 1/1–2 million

byguest on September26, 2021 Factor V deficiency 1/1 million AR aPTT, PT Sn = variable NA Factor V activity Combined Factor V/Factor VIII deficiency 1/1 million AR aPTT>PT Sn = variable NA Factor V and factor VIII activities Factor VII deficiency 1/300 000–500 000 AR PT Sn = variable NA Factor VII activity Factor VIII deficiency 1/5000 male births X-linked aPTT Sn = variable NA Factor VIII activity Factor IX deficiency 1/20 000 male births X-linked aPTT Sn = variable NA Factor IX activity Factor X deficiency 1/1 million AR aPTT, PT, RVV Sn = variable NA Factor X activity Factor XI deficiency 1/100 000 AR aPTT Sn = variable NA Factor XI activity Factor XIII deficiency 1/2–5 million AR Clot solubility Sn = variable NA Factor XIII activity Fibrinolytic defects α-2 antiplasmin deficiency ∼40 reported cases AR Euglobin lysis test Sn = variable NA α-2 antiplasmin activity PAI-1 deficiency Very rare AR Sn = variable NA PAI -1 antigen and activity Defects of fibrinogen Afibrinogenemia 1/500 000 AR PT, aPTT Sn = high NA Fibrinogen level Hypofibrinogenemia Less than afibrinogenemia PT, aPTT Sn = variable NA Thrombin time, fibrinogen activity Dysfibrinogenemia 1/million Thrombin time, fibrinogen Sn = variable NA Thrombin time, fibrinogen antigen level and activity level comparison, reptilase time Platelet disorders ITP Age-related NA CBC Sn = high NA Antiplatelet Ab (rarely needed) Glanzmann thrombasthenia Very rare AR PFA-100 Sn = 97–100 NA Platelet aggregation testing Flow cytometry FROM THE AMERICAN ACADEMY OF PEDIATRICS

umbilical stump bleeding, joint hem- not reasonable. Additionally, the initial orrhage, and excessive soft tissue screening panel evaluates for dis- bleeding, increase the probability for seminated intravascular coagulation a bleeding disorder; however, these (DIC). Because DIC can cause any type findings are neither sensitive nor of bruising/bleeding, including ICH, rmatory Test specific for bleeding disorders. the finding of DIC in the context of suspected child abuse could signifi-

cytometry cantly change the clinical approach to

Electron microscopy Molecular and cytogenetic testing Coagulation Tests in the Setting of ICH a patient. In children with DIC and bleeding symptoms as the only finding For bleeding disorders that cause ICH, concerning for abuse, consideration NA Platelet aggregation testing Flow the prevalence of the bleeding disor- must be given to the multitude of der and the prevalence of ICH in primary causes of DIC, including fi patients with each speci c bleeding trauma, sepsis, and primary bleeding disorder can be used to construct the 50 NA Platelet aggregation and secretion disorders, among many others. – probability of the specific bleeding disorder to cause ICH (Table 3). Some Many children with ICH suspicious for Sn = 27 Sn = 100 probabilities are so low as to pre- abuse, if they survive, are placed in safe clude calculation. Testing for these settings after hospital discharge. In conditions is likely not useful. Mild these cases, testing for bleeding dis- 24 hemophilia, which might be missed if orders can be deferred to a later date, only an aPTT test is ordered, can be with the exception of ITP. If blood fi products have been given to the patient, PFA-100 detected by measuring speci c levels as can happen in severe ICH, the de- Screening Tests Sn and Sp, % PPV and NPV,% Con fi of factor VIII and factor IX. Mild he- finitive evaluation for bleeding dis- PFA-100 mophilia can result in ICH, particularly after mild trauma, and because of the orders should be postponed until the relatively high prevalence of the con- transfused blood components are no dition, the probability of mild factor longer in the patient’ssystem(Table4). VIII deficiency causing or contribut- Assistance from a pediatric hematologist should be considered in address- Inheritance ing to ICH is 1 in 280 000 males. In variable AR populations with a high prevalence ing the possibility of factor deficiencies of factor XI deficiency, such as the after a transfusion has occurred. Ashkenazi Jewish population, it might be Many aspects of bleeding disorders reasonable to measure factor XI level. are under investigation, and thus, Clinical and historical information can changes in the understanding of the be used to determine the need for prevalence and severity of certain

Frequency testing in children with isolated ICH bleeding symptoms related to these concerning for abuse (Fig 1). The ini- disorders should be expected. For tial testing panel for ICH evaluates for example, although hemophilia A and B other platelet function disorders

Rare conditions for which the probability are X-linked diseases and, therefore, for the condition resulting in ICH is typically thought to affect only male greater than 1 per 5 million. The panel individuals, 25% to 50% of female includes testing for most factor defi- carriers of hemophilia report excess ciencies and afibrinogenemia. This bleeding; therefore, measurement of city; VW, von Willebrand; VWAg, von Willebrand antigen; VWF, von Willebrand factor Ab, antibody. fi screening panel does not test for factor VIII and IX levels in female factor XIII deficiency, VWD, fibrinolytic patients should be considered.29 In fi Condition defects, hypo brinogenemia, and dys- addition, the population prevalence fibrinogenemia. These conditions ei- and/or clinical effects of mild platelet Continued ther have not been associated with function disorders continue to be studied. ICH or they are so rarely the cause of In a patient with mucocutaneous symp- Bernard Soulier syndrome Platelet release/storage disorders Unknown, more common than May be reasonable to proceed directly to diagnostic testing depending on availability. See accompanying technical report for detailed discussion. Values derived from data before 2008 National Institutes of Health Consensus guidelines. Sn and Sp using current diagnostic cutoffs unknown but would be expected to have higher Sp with lower Sn.

TABLE 2 AD, autosomal dominant; AR, autosomal(test); recessive; Sn, CBC, complete sensitivity;a blood Sp, cell speci (count); NA, notb available or not applicable; NPV, negative predictive value; PAI-1, plasminogen activator inhibitor-1; PPV, positive predictive value; RVV, Russell viper venom ICH that testing for the conditions is toms, particularly if petechiae are

PEDIATRICS Volume 131, Number 4, April 2013 e1319 Downloaded from www.aappublications.org/news by guest on September 26, 2021 TABLE 3 Probabilities for Congenital Coagulopathies Causing ICHa Condition Prevalence of Condition, Prevalence of ICH, Upper Limits Probabilityb Upper Limits VWD 1/1000 Extremely rare Low Factor II deficiency 1/1 million 11% 1/10 million Factor V deficiency 1/1 million 8% of homozygotes 1/10 million homozygotes Combined factors V and VIII deficiencies 1/1 million 2% 1/50 million Factor VII deficiency 1/300 000 4%–6.5% 1/5 million Factor VIII deficiency 1/5000 males 5%–12% 1/50 000 males Factor IX deficiency 1/20 000 males 5%–12% 1/200 000 males Factor X deficiency 1/1 million 21% 1/5 million Factor XI deficiency 1/100 000 Extremely rare Low Factor XIII deficiency 1/2 million 33% 1/6 million α-2 antiplasmin deficiency 40 cases reported Not reported Low Plasminogen activator inhibitor-1 deficiency Extremely rare Common Low Afibrinogenemia 1/500 000 10% 1/5 million Dysfibrinogenemia 1/1 million Single case report Low a 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 which a 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. present, platelet aggregation testing cessity for further testing. Pro- Interpretation of Tests 25 should be considered. Finally, be- longation of the PTand aPTT because of It should be noted that the aPTT can be cause von Willebrand factor is an parenchymal damage has been noted falsely prolonged in certain circum- acute phase reactant, its levels can in abusive head trauma and should not stances, such as in the presence of vary in response to clinical status, automatically be interpreted as evi- a lupus anticoagulant, or can be pro- resulting in falsely elevated results. dence of a primary bleeding disor- longed and might not indicate a true Many times, testing must be repeated der.31 Additionally, consideration must bleeding disorder, such as in factor XII up to 3 times to ensure reliable be given to the likelihood of a preex- deficiency or other contact factor 30 fi results. If signi cant concern for isting bleeding disorder as the pri- deficiencies. In addition, patients who VWD exists, consultation with a pedi- mary cause of a child’s bleeding/ experience a traumatic brain injury atric hematologist is suggested. bruising. For example, given the rela- often have a transient coagulopathy tively high prevalence of VWD, it is that does not reflect an underlying When Testing Indicates a Possible inevitable that some children with congenital disorder.31,35 Coagulation Bleeding Disorder in the Context of VWD will be abused and present with tests are very sensitive to specimen an Abuse Evaluation bleeding/bruising symptoms. Deter- handling and should be performed in Positive laboratory test results require mining the causative factor in these laboratories experienced with these further evaluation for the possibility of situations is challenging. Bruising is assays. Inappropriate handling com- false-positive results and/or the ne- a common finding in VWD. If a child monly leads to false-positive results. has test results consistent with VWD and bruising concerning for abuse, CONCLUSIONS TABLE 4 Half-Lives of Coagulation Factors a short-term change in home setting Factor Half-Life Postinfusion, h Children who present with bleeding may be considered, understanding and bruising symptoms that are con- Fibrinogen 96–150 II 60 the cautions needed when using this cerning for abuse require careful V24approach. Only a few case reports evaluation for the potential of bleeding VII 4–6 have attributed ICH to VWD. Most disorders as a cause. No single panel VIII 11–12 IX 22 reported ICH in children with VWD of tests rules out every possible X35would not be confused with typical bleeding disorder. Given the rarity of XI 60 abusive ICH.32–34 Given the rarity of most bleeding disorders and the XIII 144–300 fi VWF 8–12 ICH in VWD, particularly spontaneous possible presence of speci c clinical VWF, von Willebrand factor. ICH, testing consistent with VWD does factors that decrease the likelihood of Reprinted with permission from Goodnight S, Hathaway not mean that ICH is definitively at- a bleeding disorder causing a child’s W. Disorders of Hemostasis and Thrombosis: A Clinical tributable to VWD, and abuse must findings, in many situations, exten- Guide. 2nd ed. New York, NY: McGraw-Hill Professional; 2001:497. still be considered. sive laboratory evaluation is not

e1320 FROM THE AMERICAN ACADEMY OF PEDIATRICS Downloaded from www.aappublications.org/news by guest on September 26, 2021 FROM THE AMERICAN ACADEMY OF PEDIATRICS necessary. If a laboratory evaluation is on the prevalence of the condition Gregory Hale, MD conducted, tests should be chosen on and potential of each specific con- Brigitta Mueller, MD fi fi Zora Rogers, MD the basis of the prevalence of the dition to cause the speci c ndings Patricia Shearer, MD condition, patient and family history, in a given child (Fig 1). Eric Werner, MD, Immediate Past Chairperson ease of testing, blood volume required 4. Laboratory testing suggestive or in- for testing, and, in the case of ICH, dicating the presence of a bleed- FORMER EXECUTIVE COMMITTEE MEMBERS probability of a bleeding disorder ing disorder does not eliminate causing ICH. Further consultation with Stephen Feig, MD abuse from consideration. In chil- Eric Kodish, MD a pediatric hematologist is recom- dren with bruising and laboratory Alan Gamis, MD mended if specific, expanded testing testing suggestive of a bleeding is necessary, if preliminary testing disorder, a follow-up evaluation af- LIAISONS — suggests the presence of a bleeding ter a change in home setting can Edwin Forman, MD Alliance for Childhood Cancer disorder, if testing to rule out a spe- provide valuable information re- fi ci c bleeding disorder is needed, or if garding the likelihood of a bleeding CONSULTANT testing for very rare conditions is disorder causing the concerning Shannon Carpenter, MD, MS preferred. findings. Thomas Abshire, MD 5. Children with ICH often receive GUIDANCE FOR PEDIATRICIANS STAFF blood product transfusions. It is Suzanne Kirkwood, MS In children who have bruising or suggested that screening for bleed- bleeding that is suspicious for abuse, ing disorders in these patients be COMMITTEE ON CHILD ABUSE AND – 1. Complete medical, trauma, and delayed until elimination of the NEGLECT, 2012 2013 transfused blood clotting elements. Cindy W. Christian, MD, Chairperson family histories and a thorough James Crawford-Jakubiak, MD physical examination are critical 6. The discovery of new information Emalee Flaherty, MD tools in evaluating for the possibil- regarding condition prevalence, John M. Leventhal, MD ity of abuse or medical conditions laboratory testing, and clinical pre- James Lukefahr, MD Robert Sege, MD PhD that predispose to bleeding/bruis- sentations of bleeding disorders ing. is to be expected. Close collabora- tion with a pediatric hematologist LIAISONS 2. In each case, careful considera- — is necessary to ensure the most Harriet MacMillan, MD American Academy of tion of the possibility of a medical Child and Adolescent Psychiatry condition causing the bleeding/ current evaluation and testing Catherine Nolan, MSW — ACSW, Administration bruising is essential. Specific ele- methods. for Children, Youth, and Families, Office on Child Abuse and Neglect ments of the history and character- LEAD AUTHORS Janet Saul, PhD — Centers for Disease Control istics of the bleeding/bruising can James D. Anderst, MD, MS and Prevention be used to determine the need for Shannon L. Carpenter, MD, MS a laboratory evaluation for bleed- Thomas C. Abshire, MD CONSULTANT ing disorders. James Anderst, MD, MS SECTION ON HEMATOLOGY/ONCOLOGY 3. If the evaluation indicates a need EXECUTIVE COMMITTEE, 2012–2013 STAFF for laboratory testing for bleeding Jeffrey Hord, MD, Chairperson Tammy Piazza Hurley disorders, initial testing is focused Gary Crouch, MD Sonya Clay

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e1322 FROM THE AMERICAN ACADEMY OF PEDIATRICS Downloaded from www.aappublications.org/news by guest on September 26, 2021 Evaluation for Bleeding Disorders in Suspected Child Abuse James D. Anderst, Shannon L. Carpenter, Thomas C. Abshire and the SECTION ON HEMATOLOGY/ONCOLOGY and COMMITTEE ON CHILD ABUSE AND NEGLECT Pediatrics 2013;131;e1314 DOI: 10.1542/peds.2013-0195 originally published online March 25, 2013;

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Downloaded from www.aappublications.org/news by guest on September 26, 2021 Evaluation for Bleeding Disorders in Suspected Child Abuse James D. Anderst, Shannon L. Carpenter, Thomas C. Abshire and the SECTION ON HEMATOLOGY/ONCOLOGY and COMMITTEE ON CHILD ABUSE AND NEGLECT Pediatrics 2013;131;e1314 DOI: 10.1542/peds.2013-0195 originally published online March 25, 2013;

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