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Investigation for Bleeding Disorders in Suspected Non-Accidental Intracranial Haemorrhage
Petrisse E Seeley1, Paul Monagle1,2, Lydia Garside1
1Sydney Children's Hospital, Randwick, Randwick, New South Wales, Australia.
Insights
In children with intracranial hemorrhage, inherited bleeding disorders are rare but crucial to identify. Initial screening with a coagulation panel, including activated partial thromboplastin time (APTT), is recommended.
Area of Science:
- Pediatric Hematology
- Child Abuse and Neglect
- Forensic Pathology
Background:
- Abusive head trauma is a leading cause of death in non-accidental injury (NAI) cases among children.
- Intracranial hemorrhage (ICH) in children can rarely stem from inherited bleeding disorders.
- Evaluating ICH in suspected NAI cases requires assessing for bleeding disorders, yet evidence guiding this is limited.
Purpose of the Study:
- To determine the prevalence of inherited bleeding disorders in children with ICH and suspected NAI.
- To identify hematological tests with the highest diagnostic yield in this population.
Main Methods:
- Retrospective cohort study of 120 children with ICH referred to Sydney Children's Hospital (2011-2020).
- Analysis of baseline coagulation screens (Full Blood Count, Prothrombin Time, Activated Partial Thromboplastin Time) performed within 72 hours of presentation.
Main Results:
- Three children (2.5%) were diagnosed with inherited bleeding disorders (hemophilia A, hemophilia B, von Willebrand disease).
- All three identified patients had a prolonged APTT on initial testing.
- Two of the three patients with bleeding disorders were confirmed as NAI.
Conclusions:
- Initial hematological screening for ICH in suspected NAI should focus on FBC, PT/APTT/fibrinogen, unless specific bleeding risk factors are present.
- Abnormal APTT on initial screening is a key indicator for further hematological investigation.
- A consistent approach to hematological evaluation is needed to effectively diagnose inherited bleeding disorders in this vulnerable population.
Background:
Abusive head trauma is the most common cause of death in children suffering non-accidental injury (NAI). Intracranial haemorrhage can (rarely) be caused by inherited bleeding disorders. Evaluation of children with suspected NAI and intracranial bleeding involves diagnosis or exclusion of a bleeding disorder; however, there is a paucity of evidence to guide haematological evaluation in these patients.
Objectives:
To determine the prevalence of inherited bleeding disorders in children with intracranial haemorrhage suspected of NAI and determine which tests have the highest diagnostic yield.
Methods:
We conducted a retrospective cohort study of children referred to the Child Protection Unit at Sydney Children's Hospital, Australia, between 2011 and 2020 with intracranial haemorrhage. Descriptive analyses of the data were completed.
Results:
A total of 120 children were included in the cohort. Eighty-seven (73%) had a baseline coagulation screen (FBC, PT and APTT) performed with initial pathology testing within 72 h of presentation. Three children (2.5%) were identified to have an underlying inherited bleeding disorder, all of whom (100%) had a prolonged APTT on initial testing.
Conclusion:
An extensive array of haematological investigations was performed, but with a lack of consistency. Three patients were identified to have an inherited bleeding disorder, including haemophilia A, haemophilia B and von Willebrand disease, two of whom were confirmed NAI regardless. All three had abnormal APTT on the initial coagulation screen. We propose initial haematological screening with FBC, PT/APTT/fibrinogen only, unless bleeding risk factors are identified. If an abnormality is detected, subsequent factor levels and further haematological investigations are recommended.
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