Cerebral salt wasting syndrome following atlantoaxial fracture dislocation in Down syndrome
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Published on: November 8, 2024
Mohamed El-Amin Abdel-Latif1, Patrick W-K Chan, Adrian Yu-Teik Goh
1Royal Hospital for Women, Department of Newborn Care, Barker Street, Randwick, 2031 New South Wales, Australia.
Insights
Cerebral salt wasting syndrome (CSWS) can occur in children with spinal cord disorders. Prompt diagnosis and fluid replacement with saline corrected hyponatremia and improved outcomes in a pediatric patient.
Area of Science:
- Pediatric Neurology
- Endocrinology
- Neurosurgery
Background:
- Spinal cord disorders in children can lead to complex electrolyte imbalances.
- Cerebral salt wasting syndrome (CSWS) is a recognized cause of hyponatremia.
- Down syndrome presents unique challenges in managing pediatric neurological conditions.
Purpose of the Study:
- To report a case of CSWS in a pediatric patient with Down syndrome and spinal cord injury.
- To highlight the importance of differentiating CSWS from other causes of hyponatremia (SIADH, DI) in this population.
- To emphasize the efficacy of specific treatment for CSWS.
Main Methods:
- Case report of a 5-year-old female with Down syndrome.
- Diagnosis of acute myelopathy secondary to atlantoaxial subluxation.
- Monitoring of electrolytes, urine output, and urine sodium concentration.
- Treatment with volume-for-volume replacement of urine loss using 0.9% sodium chloride.
Main Results:
- The patient presented with hyponatremia, excessive urine output, and elevated urine sodium.
- Treatment with 0.9% sodium chloride resulted in normalization of electrolytes and resolution of symptoms.
- This intervention led to an excellent clinical outcome.
Conclusions:
- CSWS should be considered in children with spinal cord disorders presenting with hyponatremia.
- Accurate differentiation from SIADH and DI is crucial for appropriate management.
- Timely and specific treatment of CSWS in pediatric spinal cord injury yields favorable outcomes.
Abstract:
We describe cerebral salt wasting syndrome (CSWS) in a 5-year-old female child with Down syndrome who had acute myelopathy secondary to chronic atlantoaxial subluxation and fracture dislocation of the odontoid process. The patient developed hyponatraemia associated with excessive urine output and elevated urine sodium concentration following her injury. An administered volume-for-volume replacement of urine loss with 0.9% sodium chloride resulted in an excellent outcome. This patient illustrates the importance of ascertaining CSWS in children with spinal cord disorders, in addition to the syndrome of inappropriate antidiuretic hormone (SIADH) secretion and diabetes insipidus (DI) commonly encountered following a central nervous system (CNS) injury, as the specific treatment approaches is clearly associated with an excellent outcome.
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