Life-threatening mediastinal-retroperitoneal hemorrhage in a child with moderate hemophilia A and high inhibitor titer: successful management with recombinant activated factor VII

The authors describe an 11-year-old boy with hemophilia A and high titer inhibitor who developed a life-threatening mediastinal-retroperitoneal hemorrhage. Chest CT showed a large hematoma beginning in the retrotracheal area, filling the mediastinum, compressing the carina, and extending retroperitoneally up to the kidneys. As the surgical approach has a high mortality rate, the authors chose a more conservative approach initially and obtained excellent bleeding control with recombinant activated factor VII without the need for surgical intervention. As reported in other patients, the authors also showed a decrease in the factor III inhibitor while this patient was successfully treated with bypassing agents.     

Intracranial hemorrhage in a newborn with hemophilia following elective cesarean section.

A newborn infant with severe hemophilia developed an intracranial hemorrhage despite a delivery by elective cesarean section. This case shows that hemophiliacs can have spontaneous central nervous system bleeding during the perinatal period. Newborns at risk for hemophilia should receive prompt evaluation whatever the mode of delivery.     

Extra hepatic portal vein thrombosis in a child associated with lupus anticoagulant

The presence of lupus anticoagulant has been implicated in venous as well as arterial thrombosis. We report here a 10-year-old boy who presented to us with hematemesis, malaena and splenomegaly. An ultrasound showed a recanalized portal vein with collaterals suggestive of portal vein thrombosis. He had grade IV esophageal varices. The liver function tests were normal. Investigations for prothrombotic factors showed that tests for PNH and for APC resistance were negative. Levels of anti-thrombin II and protein C were normal. There was a prolonged activated partial thromboplastin time with a normal prothrombin time. Presence of lupus anticoagulant was confirmed with dilute Russell viper venom time and platelet neutralization test. Repeat tests after 10 weeks showed persistence of the lupus anticoagulant. ELISA test for anti-phospholipid antibody was negative. The association of lupus anticoagulant with portal vein thrombosis in the pediatric age group is very rare.     

Deep cerebral venous thrombosis in thalamo-ventricular hemorrhage of the term newborn

Unilateral thalamic bleeding with associated intraventricular hemorrhage is reported in three full-term neonates. The first presented within 48 hours from birth with early onset streptococcal meningitis, persistent pulmonary hypertension, tonic seizures and a tense fontanelle. The second presented 6 days after birth with irritability, opisthotonus, a tense fontanelle and tonic seizures. The third was admitted three days after birth with seizures and a tense fontanelle. In the latter two infants NMR and CT imaging documented thrombosed superficial and deep cerebral veins. The etiopathogenesis of intracranial venous thrombosis in the neonate is diverse: asphyxia, dehydration, polycythemia, sepsis-meningitis and difficult delivery are the main causes. In one of our patients jugular vein compression by the collar of a negative-pressure ventilation chamber probably initiated the intracranial events. More than half of the survivors sustain severe neurological impairment.     

Transient lupus anticoagulants associated with hemorrhage rather than thrombosis: The hemorrhagic lupus anticoagulant syndrome

Lupus anticoagulants (LAs) represent a diverse group of antibodies directed against phospholipids. Patients with LAs may be free of symptoms but can have thrombotic complications including stroke, placental infarction, and fetal loss. Rarely hemorrhagic symptoms have been reported. We describe six previously healthy children who were first seen with clinical bleeding and prolonged activated partial thromboplastin time. Laboratory evaluation revealed positive results on mixing studies and evidence of phospholipid dependence of the anticoagulant, suggesting LAs. Four of six patients had anticardiolipin antibodies, and all four who were tested had reduced factor II activity levels. In all patients, bleeding symptoms resolved spontaneously within 3 months, and laboratory findings returned to normal within 6 months. The hemorrhagic LA syndrome should be considered in previously healthy children with new-onset bleeding and prolonged activated partial thromboplastin time. This clinical entity probably represents a pathogenic mechanism distinct from thrombotic LA syndromes. (J Pediatr 1997;130:998-1000)     

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。     

Intradiploic hemangioma with repeated hemorrhage in a child with hemophilia

Intraosseous hemangioma is an uncommon benign vascular tumor, which is most frequently found in middle-aged female patients. The clinical course is usually insidious and the outcome excellent after total resection. The authors report a case of a calvarial hemangioma in a child with hemophilia who experienced a catastrophic postoperative hematoma and discuss the mechanism, clinical features, and treatment of this condition.     

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 neonates with unrecognized hemophilia A: a persisting problem

Hemophilia is a rare disorder, and an uncommon cause of intracranial hemorrhage in neonates. We present 2 patients with hemophilia A, who presented with massive subdural hemorrhages on day 5 and day 4 postpartum. Both were taken urgently to surgery without a diagnosis of hemophilia being established. Neither patient had a family history of hemophilia, and both were born following difficult deliveries. The activated partial thromboplastin time (APTT) was normal in patient No. 1 (subsequent factor VIII level 10%). In patient No. 2, the APTT was slightly prolonged, but initially interpreted as being within the normal range for age (subsequent factor VII level of < 1%). Patient 1 rebled, required a second operation, and had a poor outcome. Patient 2 was given prophylactic fresh frozen plasma, and made a good recovery. Factor VIII assay should be performed in all term male babies presenting with intracranial hemorrhage. In urgent circumstances, prophylactic clotting therapy should be administered during surgery to prevent postoperative bleeding in an undiagnosed hemophiliac.     

Thalamic hemorrhage with intraventricular hemorrhage in the full-term newborn

Intraventricular hemorrhage is an uncommon problem in the full-term newborn. In a review of 19 full-term infants with intraventricular hemorrhage diagnosed on computed tomography prior to 1 month of age, thalamic hemorrhage associated with the intraventricular hemorrhage was documented in 12 infants. Thus, thalamic hemorrhage appears to the most common source of intraventricular hemorrhage in this age group, particularly in infants who had uneventful birth histories and in whom clinical abnormalities (signs of increased intracranial pressure, seizures, altered level of consciousness) developed after the first week of life. The majority of these infants had predisposing factors for cerebral venous infarction such as sepsis, cyanotic congenital heart disease, and coagulopathy. The clinical appearance and outcome for infants with thalamic hemorrhage/intraventricular hemorrhage were similar to those in infants with intraventricular hemorrhage originating from other sites, except for an increased incidence of cerebral palsy in infants with thalamic hemorrhage/intraventricular hemorrhage. Definitive diagnosis was made on the basis of characteristic radiologic abnormalities.     

Life-threatening intracranial bleeding in a newborn with congenital cytomegalovirus infection: late-onset neonatal hemorrhagic disease

The authors present a case of a 36-day-old infant with intracranial and intramuscular hemorrhage due to vitamin K deficiency bleeding, who received intramuscular vitamin K prophylaxis at birth. In this case, laboratory tests showed anemia, liver dysfunction with cholestasis, and coagulopathy, consistent with vitamin K deficiency abnormality. Serological analyses showed that cytomegalovirus immunoglobulin (Ig)M and IgG avidity were both positive. The infant was treated successfully with intravenous ganciclovir and blood products. This case suggests that it is imperative to meticulously investigate the etiology in neonates with late-onset hemorrhagic disease of the newborn. Cholestatic liver disease caused by congenital cytomegalovirus infection should be in mind in term infants who presented with late-onset hemorrhagic disease.     

Posterior fossa hemorrhage in the newborn — diagnosis and management

Two cases of posterior fossa hemorrhage in full-term newborns are presented. Because of possible sonographic misinterpretations computer tomographic (CT) scans are advocated for a reliable diagnosis. Regarding the management of the primary hemorrhage as well as the posthemorrhagic hydrocephalus a conservative approach is recommended.