Spontaneous intracranial hemorrhage as the presenting sign of hemophilia B in a 3-month-old infant

Intracranial hemorrhage (ICH) is an uncommon complication of hemophilia in the 1st year of life and most often is reported after head trauma or birth trauma. Spontaneous ICH unrelated to birth or head trauma is rare at any age, especially in the 1st year of life. We describe a 3-month-old infant who presented to the emergency department (ED) with a spontaneous ICH as the presenting sign of hemophilia B. We review the literature and discuss the ED evaluation and management of hemophiliacs with ICH.     

Intracranial hemorrhage in newborns with hemophilia: the role of screening radiologic studies in the first 7 days of life

In newborns with hemophilia, intracranial hemorrhage (ICH) is a potentially life-threatening event and in those who survive, there is a risk of long-term neurologic sequelae. A single-center retrospective chart review was performed to look at the utility of central nervous system screening of babies who were diagnosed with moderate or severe hemophilia in the first 7 days of life. Twenty of the twenty-two eligible babies had either a head ultrasound or computerized tomography of the head performed as soon as possible after the diagnosis of hemophilia was confirmed. ICH was found in 3 of the 20 newborns. All patients with ICH had instrument-assisted deliveries with extracranial signs of bleeding/trauma. On the basis of this series we found screening to be safe and effective in recognizing ICH. As a result of this approach, all ICHs were found when they were radiographically small and before the babies had neurologic symptoms.     

Neonatal hemophilia B with intracranial hemorrhage. Case report

It is uncommon for infants with hemophilia to have excessive bleeding during the neonatal period. Even if bleeding occurs, it rarely becomes life-threatening, such as in intracranial hemorrhage (ICH). We here report a case of a 4-day-old boy who had intracranial hemorrhage as the first complication of hemophilia B. Computerized axial tomography (CT scan) and ultrasonography were very useful for early diagnosis. Only a few cases of neonatal hemophilia with intracranial hemorrhage have been reported, but the occurrence of this complication is probably more frequent. We reviewed seven cases (including our case) with intracranial hemorrhage as the first manifestation of neonatal hemophilia. Although these infants showed good prognosis as to survival, permanent residual neurological deficits remained in all of them. It is emphasized that intracranial hemorrhage due to hemophilia may occur in neonates even without a family history. Urgent neuroimaging and coagulation studies are necessary for an early and adequate diagnosis.     

Effect of intracranial bleeds on the health and quality of life of boys with hemophilia

OBJECTIVES: To compare the health, physical function, and quality of life (QoL) of boys with hemophilia with and without a history of intracranial hemorrhage (ICH). STUDY DESIGN: Of 172 patients with hemophilia A or B, 18 (10%) had at least one episode of ICH. For outcome assessments, 16 of 18 (89%) boys with ICH and 32 controls, matched (1:2) for age and severity of hemophilia, were available. The outcome measures were neurologic function, physical function, and QoL. RESULTS: The median age of the boys at the first ICH was 5.9 months (range, 1 day to 2.7 years). Boys with ICH had a higher incidence of inhibitors and lower mean household income. Neurologic examination was abnormal in seven of 16 (44%) boys with ICH and nine of 32 (28%) controls (P=.3). The mean physical function in boys with ICH was lower (82%+/-25%) compared with controls (93.5%+/-12%, P=.045). The QoL was decreased in boys with ICH compared with controls (6.8+/-3.2 vs 8.5+/-1.4, P=.02), whereas health-related QoL was not significantly different between groups. CONCLUSION: The poorer long-term outcomes of boys with hemophilia appropriately treated for ICH, especially in the domain of QoL, suggest that new strategies to prevent ICH and to manage ICH effectively in this population are needed.     

重型血友病患儿治疗方式与颅内出血的关系

目的回顾性分析近10年我院门诊重型血友病患儿的治疗方案及其发生颅内出血(ICH)的临床资料,了解重型血友病患儿治疗方式与ICH的关系。方法 2014年4月1日收集并回顾性分析1993年1月1日-2012年12月31日出生的重型血友病患儿治疗方案和发生ICH的相关临床资料。结果共收血友病患儿病例197例,157例为血友病A,40例为血友病B。血友病A患儿中4.5%(7/157)以ICH为首发症状,8.2%(11/134)按需治疗患儿发生ICH,0%(0/16)预防治疗患儿出现ICH;血友病B患儿中10%(4/40)以ICH为首发症状,13%(4/32)的按需治疗患儿发生ICH,0%(0/4)的预防治疗患儿出现ICH。ICH近5年累积发生率为13.2%(26/197),42%(11/26)以ICH为首发症状,73%(19/26)的患儿发生ICH时年龄小于3岁,69%(18/26)的患儿发生ICH有明确外伤史。结论目前我院随诊的重型血友病患儿以ICH为首发症状约占40%,多发生于婴幼儿。预防治疗组患儿无ICH发生,提示及早诊断和积极开展预防治疗可以降低重型血友病患儿ICH的发生。     

Intracranial and extracranial hemorrhages in newborns with hemophilia: a review of the literature.

PURPOSE: Intracranial hemorrhage (ICH) and extracranial hemorrhage (ECH) in newborns with hemophilia were reviewed with respect to incidence, anatomic location, clinical presentation, and relationship to the mode of delivery and type of hemophilia. MATERIALS AND METHODS: A MEDLINE search from 1964 to 1996 of all reports of neonatal hemophilia and head bleeds in children from birth to 1 month old was performed. ICH was defined as any bleed occurring within the cranial cavity, and ECH was defined as hemorrhage occurring outside of the cranial cavity, including subgaleal and cephalhematoma. The mode of delivery, type and severity of hemophilia, and clinical presentation were also noted. RESULTS: One hundred two newborns with hemophilia and cranial bleeds were described in 33 publications. The cumulative incidence of ICH and ECH was 3.58% in 5 studies that reported the total newborn population or that examined birth records. The type of hemophilia was reported for 40 of 102 newborns and was hemophilia A in 87%. The mode of delivery was recorded in 46% (47 of 102) of the patients. Of these, 13% had cesarean delivery, and 87% were delivered vaginally (40% had spontaneous vaginal delivery, and 47% had vaginal delivery with vacuum extraction or forceps). There were 109 episodes of ICH and ECH (65% were ICH, 35% were ECH, and 5.8% were a combination of both). Common clinical features of ICH and ECH included anemia, hypotension, shock, and lethargy. However, only patients with ICH were reported to have neurologic deficits (15%) and late neurologic sequelae (38%). CONCLUSION: In neonates with hemophilia and cranial bleeds, ICH occurs more often and is often associated with late neurologic deficits.     

Computerized tomography in hemophiliacs with head trauma

Intracranial hemorrhage secondary to head trauma is a major cause of morbidity and mortality in patients with bleeding disorders. Indications for head computerized tomographic scanning (CT scan) on patients with bleeding disorders who sustain head trauma are not well established. We retrospectively reviewed the medical records and head CT scan results of 21 patients with bleeding disorders. Five patients had more than one episode of head trauma. The severity of head trauma per episode was classified according to objective clinical findings as minor in 12 episodes, moderate in 12, and severe in four. In three of four patients with severe head trauma, the CT scan showed evidence of intracranial hemorrhage. In this series, all 17 patients with 24 episodes of moderate or minor head trauma had normal head CT scans. We conclude that a larger prospective study is needed to further evaluate the diagnostic value of head CT scan in hemophilia patients with minor or moderate head trauma, as defined in this study.     

Perinatal factors associated with early-onset intracranial hemorrhage in premature infants. A prospective study

Serial ultrasound examinations were performed on 40 consecutive newborn infants less than 35 weeks' gestational age. Fifteen of 17 infants with intracranial hemorrhage (ICH) had evidence of hemorrhage on the first ultrasound examination (mean age, 1.9 +/- 0.2 hours post partum). Comparing the clinical course of these 15 infants with age- and weight-matched non-hemorrhage controls showed a significant association between the occurrence of early ICH and the pattern of labor. There was no correlation between ICH and the mode of delivery, the use of sodium bicarbonate, volume administration, or the initial BP. In nine of the 15 infants with early-onset ICH, the hemorrhage progressed in severity during the first three postpartum days in association with increasing ventilatory requirements. The results of this study suggest that the course of labor may be a precipitating factor in the onset and evolution of early ICH.     

Comparison of intracranial computed tomographic (CT) findings in pediatric abusive and accidental head trauma

Background. Child abuse specialists rely heavily on diagnostic neuroimaging. Objectives. Study objectives were: (1) to compare the frequencies of six specific intracranial CT abnormalities in accidental and non-accidental pediatric head trauma, and (2) to assess interobserver agreement regarding these CT findings. Materials and methods. Three pediatric radiologists blindly and independently reviewed cranial CT scans of pediatric patients who sustained closed head trauma between 1991 and 1994. All patients were less than 4 years of age. Study cases included thirty-nine (50 %) with non-accidental head trauma and thirty-nine (50 %) with accidental head trauma. Each scan was evaluated for the presence or absence of the following six intracranial findings: (1) interhemispheric falx hemorrhage, (2) subdural hemorrhage, (3) large (non-acute) extra-axial fluid, (4) basal ganglia edema, (5) posterior fossa hemorrhage, and (6) frontal-parietal shearing tear(s). Interobserver agreement was calculated as the percentage of total cases in which all reviewers agreed a specific CT finding was present or absent. Diagnosis required independent agreement by all three pediatric radiologists. The frequencies of these six intracranial CT abnormalities were compared between the two study groups by Chi-square analysis and Fisher's exact test. Results. Interobserver agreement between radiologists was greater than 80 % for all lesions evaluated, with the exception of frontal-parietal shearing tear(s). Interhemispheric falx hemorrhage, subdural hemorrhage, large (non-acute) extra-axial fluid, and basal ganglia edema were discovered significantly more frequently in non-accidental trauma (P≤ .05). Conclusion. Although not specific for child abuse, discovery of these intracranial CT abnormalities in young patients should prompt careful evaluation of family and injury circumstances for indicators of non-accidental trauma. Received: 8 November 1996 Accepted: 10 April 1997     

Auditory brainstem response in neonates with asphyxia and intracranial haemorrhage

Auditory brainstem response (ABR) was used to assess possible brainstem damage in 76 neonates with asphyxia and intracranial haemorrhage (ICH). Fifty-eight neonates had ICH, 52 had neonatal asphyxia and 34 of these patients had both. Eighty-nine percent of the patients with neonatal asphyxia showed some abnormal patterns in response, the major one being an increase in the threshold of wave V. In the ICH group, abnormal patterns were observed in 62.5%, among whom the prolongation of the I–V interpeak latency (IPL) and of wave V latency was seen more frequently than the increase of threshold of wave V. In the case of neonatal asphyxia associated with ICH, both the prolongation of the latency and the increase of threshold were observed equally. These abnormalities of ABR were associated with worsening clinical condition and conversely normalized gradually following the improvement of the underlying disease. Especially the I–V IPL, wave V latency and the threshold of wave V could serve as indicators of the treatment.Abbreviations ABR auditory brainstem response - ICH intracranial haemorrhage - IPI interpeak latency - NICU neonatal intensive care unit - AFD appropriate-for-date - SFD small-for-date - SDH subdural haemorrhage     

Lack of evidence for a causal relationship between hypoxic-ischemic encephalopathy and subdural hemorrhage in fetal life, infancy, and early childhood.

It has been asserted that hypoxic-ischemic encephalopathy (HIE) with cerebral swelling in the absence of marked trauma may be responsible for subural hemorrhage in the young. As this may have considerable implications in determining both the mechanism of death and the degree of force required to cause injury in certain cases of inflicted head injury in infancy, clarification is required. A retrospective study of 82 fetuses, infants, and toddlers with proven HIE and no trauma was undertaken from forensic institutes in Australia, the United Kingdom, Germany, Denmark, and the United States. The age range was 35 weeks gestation to 3 years, with a male to female ratio of 2:1. All cases had histologically confirmed HIE. Causes of the hypoxic episodes were temporarily resuscitated sudden infant death syndrome with delayed death (N = 30), drowning (N = 12), accidental asphyxia (N = 10), intrauterine/delivery asphyxia (N = 8), congenital disease (N = 6), aspiration of food/gastric contents (N = 4), inflicted asphyxia (N = 3), epilepsy (N = 1), dehydration (N = 1), drug toxicity (N = 1), complications of prematurity (N = 1), and complications of anesthesia (N = 1). The initiating event was not determined in 4 instances. In no case was there macroscopic evidence of subdural hemorrhage. In this study no support could be given to the hypothesis that HIE in the young in the absence of trauma causes subdural hemorrhage.     

Behavioral characteristics and early temperament of premature infants with intracranial hemorrhage

Behavioral characteristics of 12 full-term and 44 premature infants with and without intracranial hemorrhage (ICH) were studied. Cranial ultrasonography prospectively documented Grade I-II ICH in 14, Grade III-IV in 19 and no ICH in 11 premature infants. Examination at corrected age of 40 +/- 2 weeks using the Neonatal Behavioral Assessment Scale showed that infants in the ICH groups had lower levels of arousal and more abnormal reflexes than full-terms. Infants with ICH III-IV displayed less optimal motor responses than full-term infants and diminished orientation responses, especially to visual stimuli. Thus, lower level of arousal, immature motoric processes, and poor visual orientation differentiated premature with ICH from full-term infants, although premature infants without these sequelae, did not differ significantly from full-term infants. The above may represent early manifestations of visual-perceptual and motor problems noted in the follow-up of ICH infants. Further, neonatal behavior was found to affect parent ratings of infant temperament (via the Bates Infant Characteristics Questionnaire) at 3 months corrected age, and the relationships between neonatal behavior and parental ratings differed depending upon the infant's gestational age and severity of hemorrhage. We conclude that neonatal behaviors are less optimal in premature infants, and least optimal in premature infants with severe intracranial hemorrhage when compared to fullterm infants.     

儿童重症监护病房急性颅内出血病因和预后分析

目的调查儿童重症监护病区(PICU)急性颅内出血的病因和预后。方法对2000年1月~2004年12月我院PICU危重病患儿中,急性颅内出血患儿临床资料进行回顾性病因和预后分析。结果近5年来共收治急性颅内出血患儿58例,病因是迟发性维生素K(VitK)缺乏症28例,颅脑外伤16例,脑血管畸形6例,病毒性脑炎2例,血友病、血小板减少症、脑肿瘤破裂各1例,不明原因3例。其中15例放弃治疗,9例并发继发性脑梗塞。8例死亡(病死率13.8%)。结论迟发性VitK缺乏症和颅脑外伤是PICU小儿颅内出血的主要原因,预防性应用VitK或改变喂养方式可能降低婴儿早期颅内出血发病率。颅内出血预后较差,重在预防。     

Cerebral infarct secondary to traumatic carotid artery dissection

Internal carotid artery dissection is an important cause of ischemic stroke in children and young patients. Children presenting with gross neurological abnormalities after blunt trauma to the head or neck should be considered to have sustained injury to the carotid arteries until proven otherwise. Treatment options include observation, anticoagulation and endovascular stenting, and aggressive surgical repair of the carotid artery injury. We present the case of a 7-year-old boy who had a dissection of his right internal carotid artery after a dangerous position of head upside down from a water slide.     

Factors associated with the development of intracranial hemorrhage in patients treated with extracorporeal membrane oxygenation

Intracranial hemorrhage (ICH) is a major cause of morbidity and mortality during extracorporeal membrane oxygenation (ECMO) and has been associated with prematurity and hypertension. The purpose of this study was to determine whether factors present before the institution of ECMO would predict the development of ICH. Data from the national ECMO registry were collected on all patients who received ECMO over a 2-year period in established centers. Stepwise multivariate analysis was used to determine the relative contribution of different factors in the development of ICH. Data on 972 infants were evaluated; the overall incidence of ICH was 11%. There was a progressive increase in incidence with prematurity, acidosis, and hypercarbia. The best positive predictive value was 60% using all available factors. The premature as well as the term infant with severe metabolic acidosis and hypercarbia has the highest risk to develop ICH on ECMO. Conditions present prior to institution of ECMO therapy have a significant impact on outcome. Although the best predictive ability is only 60%, patients at greatest risk to develop ICH can be identified. Prompt referral and institution of bypass in these patients, when indicated, may modify the subsequent course.The opinions expressed are those of the authors and do not reflect those of the United States Air Force or the Department of Defense.     

Fatal cerebral edema and intracranial hemorrhage associated with hypernatremic dehydration

We report neuroimaging findings of intracranial hemorrhage and cerebral edema in an infant with obtundation and seizures, initially suspected to be secondary to non-accidental trauma but finally attributed to hypernatremic dehydration. Neuroimaging findings due to hypernatremic dehydration have not been previously described in the radiologic literature. Hypernatremia should be included in the differential diagnosis of intracranial hemorrhage in the infant without evidence of nonaccidental trauma. Received: 21 February 1997 Accepted: 25 April 1997     

Traumatic low attenuation subdural fluid collections in children younger than 3 years

OBJECTIVE: To determine the time course for the development of posttraumatic nonhemic subdural fluid collections in infants and young children. DESIGN: Retrospective consecutive case series during 16 years.Patients Fifty-five head trauma patients younger than 3 years with low attenuation subdural fluid on computed tomography. MAIN OUTCOME MEASURE: Time after head trauma when low attenuation fluid first becomes visible. SETTING: Regional pediatric medical center. RESULTS: The initial visualization of low attenuation subdural fluid was within 4 days of the trauma for 44 of the patients. The mean +/- SD size of the subdural fluid collections when first identified was 4.6 +/- 2.0 mm (range, 2-12 mm), and the maximum observed size was 7.7 +/- 3.5 mm (range, 3-21 mm). The mean +/- SD time after injury until the maximum observed size was 16 +/- 18 days (range, 0-87 days). Low attenuation subdural fluid and high attenuation intracranial hemorrhage coexisted on at least 1 computed tomographic study during the first week after the trauma in 42 (81%) of the 52 patients with hemorrhage. CONCLUSION: Low attenuation subdural fluid collections (distinct from clotted blood) in infants and young children with head injuries most often develop during the first week after the traumatic event.     

Intracranial hemorrhage in children younger than 3 years: prediction of intent

OBJECTIVE: To determine whether certain computed tomographic imaging patterns in infants and young children with intracranial hemorrhage help predict intentional compared with unintentional injuries. DESIGN: Retrospective consecutive case series over a 10-year period. PATIENTS: Two hundred ninety-three children younger than 3 years with intracranial hemorrhage. MAIN OUTCOME MEASURES: The sensitivity and specificity of computed tomographic imaging patterns for intentional head injury. SETTING: Regional pediatric medical center. RESULTS: Four variables used in the multiple logistic regression analysis for predicting intentional head injury were statistically significant (P<.05): subdural hematoma located over the cerebral convexities, hematoma within the interhemispheric subdural space, hygroma (nonhemic subdural fluid) with intracranial hemorrhage, and absence of a skull fracture with intracranial hemorrhage. The prediction model for the diagnosis of intentional head trauma using combinations of these 4 variables and a.45 probability cutoff point indicated a sensitivity of 84% (95% confidence interval, 78%-90%) and a specificity of 83% (95% confidence interval, 74%-89%). CONCLUSION: Computed tomographic imaging patterns of intracranial hemorrhage in children younger than 3 years help predict whether the injury was intentional.     

新生儿血友病A 11例病例系列报告

目的 探讨新生儿血友病A的临床表现、诊治及预后。方法 对复旦大学附属儿科医院2016年2月1日至2019年6月30日收治的11例新生儿血友病A临床资料进行回顾性分析。结果 11例新生儿血友病患儿均为男性,3例有明确血友病家族史。2例无出血表现,2例仅表现为皮肤瘀点瘀斑,7例有出血表现。出血部位包括硬膜下、颅内、皮下、消化道。11例活化部分凝血酶时间(APTT)均延长,均为凝血因子Ⅷ缺乏,中间型9例,重型和轻型各1例。5例行基因检测者证实FⅧ基因突变,缺失2例,点突变3例。血友病A确诊后予静脉输注Ⅷ因子。随访至2019年6月9~10日,1例失访,4例无出血表现,1例有踝关节自发出血表现,5例表现为外伤后皮肤瘀点/皮下血肿。结论 新生儿期多次凝血功能异常,以APTT延长为主,特别是延长＞3倍者有或无出血表现均应考虑血友病可能,应行凝血因子活性水平测定及基因检测及早确诊,早期诊断和预防性输注凝血因子可改善预后。     

半导体激光配合神经内窥镜手术治疗小儿脑积水

目的　探讨微创神经外科技术在治疗脑积水中的应用。方法　应用半导体激光配合神经内窥镜对32例脑积水患儿 ,其中 15例梗阻性脑积水行第三脑室底造瘘术 ,11例交通性脑积水行脉络丛电灼凝固术 ,6例室间孔闭塞性脑积水行透明隔造瘘术。结果　随访 31例 ,随访时间 6个月～ 1年 ,头部动态CT或MRI复查 ,2 9例脑积水明显好转 ,2例出现脑积水加重 ,改用内窥镜辅助下脑室 腹腔分流术 (V P术 ) ,症状改善。术后并硬膜外血肿、脑室内少量出血各 1例 ,无感染、心肺功能异常等其他并发症。结论　半导体激光配合神经内窥镜技术治疗脑积水 ,创伤小 ,安全 ,术后并发症少 ,效果确切