Intracranial hemorrhage in children with congenital factor deficiencies

Turkan Patiroglu1, Mehmet Akif Ozdemir, Ekrem Unal

  • 1Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Talas, Kayseri, Turkey.

Insights

Intracranial hemorrhage (ICH) in children with congenital factor deficiencies (CFD) is serious. Prompt recognition and treatment are key to survival and preventing long-term deficits.

Area of Science:

  • Pediatric Hematology
  • Neurology
  • Genetics

Background:

  • Intracranial hemorrhage (ICH) poses a significant threat to children.
  • Congenital factor deficiencies (CFD) are a known cause of ICH, sometimes presenting as the first symptom.
  • Prompt diagnosis and management are crucial.

Purpose of the Study:

  • To evaluate the incidence, causes, and outcomes of ICH in children with CFD.
  • To identify risk factors and optimal management strategies for ICH in this pediatric population.

Main Methods:

  • A retrospective review of 107 children with CFD from 2000-2010.
  • ICH episodes were identified through clinical evaluation and neuroimaging (CT/MR).
  • Management strategies, surgical interventions, and patient outcomes were analyzed.

Main Results:

  • 18 patients (16.8%) experienced 21 ICH episodes, with a mean age of 42.1 months.
  • Trauma was the leading cause (57.1%), followed by non-trauma related events (42.9%). Epidural hematoma was most common.
  • All patients survived, though four developed intellectual capacity decrease and motor deficits.

Conclusions:

  • Optimal management of ICH in pediatric CFD requires immediate recognition and prompt replacement therapy.
  • Adequate surgical intervention is essential for hemostatic balance and improved outcomes.
  • Early intervention can mitigate long-term neurological sequelae.
Abstract

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