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Published on: April 18, 2025
[Intracranial hypotension syndrome in a pediatric patient with Marfan syndrome]
Amelia Moreno Sánchez1, David Molina Herranz2, Armando Sánchez Andrés3
1Servicio de Pediatría. Sección de Neuropediatría. Hospital Universitario Miguel Servet.. ameliamorenosanchez111@gmail.com.
Insights
Intracranial hypotension syndrome (IHS) in children, often linked to cerebrospinal fluid (CSF) leaks, requires prompt diagnosis. This case highlights the importance of suspecting IHS in pediatric patients with orthostatic headaches, especially those with connective tissue disorders like Marfan syndrome.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Intracranial hypotension syndrome (IHS) is characterized by reduced cerebrospinal fluid (CSF) pressure, often presenting as orthostatic headache.
- IHS can be spontaneous or secondary to trauma, surgery, or systemic conditions.
- Marfan syndrome, a connective tissue disorder, may predispose individuals to conditions causing CSF leaks.
Observation:
- An 11-year-old boy with Marfan syndrome experienced orthostatic headache and vomiting after a fall.
- Magnetic resonance imaging revealed extradural fluid collections, indicative of a CSF leak.
- The patient had recurrent episodes of IHS despite initial treatment.
Findings:
- The case demonstrates a pediatric presentation of IHS secondary to a likely CSF leak following trauma in a patient with Marfan syndrome.
- Recurrent symptoms necessitated a delayed epidural blood patch for resolution.
- This case underscores the diagnostic challenge of IHS in children, particularly those with underlying connective tissue abnormalities.
Implications:
- Pediatric IHS should be considered in children presenting with orthostatic headaches, especially those with connective tissue disorders.
- Further research is needed to establish optimal management strategies for IHS in the pediatric population.
- This case contributes valuable data to the limited literature on pediatric IHS management.
Abstract:
Intracranial hypotension syndrome (IHS) is attributed to reduced cerebrospinal fluid (CSF) pressure. It may be spontaneous or secondary to a history of trauma or systemic disease. We present the case of an 11-year-old boy, with medical history of Marfan syndrome, with orthostatic headache and persistent vomiting (12 hours) following a fall on the sacrococcygeal region. Magnetic resonance showed extradural fluid collections at dorsal and lumbosacral levels, compatible with CSF leak. The condition was resolved with treatment, but the patient had two new episodes during the follow-up period. Thus, an epidural blood patch was performed two years after the first episode. Although HIS is uncommon in children, it should be suspected in patients with orthostatic headache, particularly if the patient presents a connectivopathy. Few studies have assessed the management of HIS in paediatric age. The case presented here and the reviewed available literature provides further data for these type of cases.
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