Ann Pediatr Endocrinol Metab.  2015 Dec;20(4):235-237. 10.6065/apem.2015.20.4.235.

Turner syndrome with spinal hemorrhage due to vascular malformation

• Yu MK
• Jung MK
• Kim KE
• Kwon AR
• Chae HW
• Kim DH
• Kim HS

• KMID: 2354702
• DOI: http://doi.org/10.6065/apem.2015.20.4.235

Abstract

Turner syndrome (TS) is a relatively common chromosomal disorder and is associated with a range of comorbidities involving the cardiovascular system. Vascular abnormalities, in particular, are a common finding in cases of TS. However, dissection involving the vertebral arteries is rare. Here, we report the case of a 9-year-old girl with TS who had been treated with growth hormone replacement therapy for the past 3 years. She presented with weakness of both lower legs, and was ultimately diagnosed with spinal hemorrhage due to vascular malformation. We treated her with intravenous high dose dexamethasone (0.6 mg/kg) and she could walk without assistance after 6 days of treatment. In conclusion, when a patient with TS shows sudden weakness of the lower limbs, we should consider the possibility of spinal vessel rupture and try to take spine magnetic resonance imaging as soon as possible. We suggest a direction how to make a proper diagnosis and management of sudden vertebral artery hemorrhage in patients with TS.

Keyword

Turner syndrome; Vascular malformations; Vertebral artery dissection

MeSH Terms

Cardiovascular System
Child
Chromosome Disorders
Comorbidity
Dexamethasone
Diagnosis
Female
Growth Hormone
Hemorrhage*
Humans
Leg
Lower Extremity
Magnetic Resonance Imaging
Rupture
Spine
Turner Syndrome*
Vascular Malformations*
Vertebral Artery
Vertebral Artery Dissection
Dexamethasone
Growth Hormone

Figure

• 

  Fig. 1 T2-weighted magnetic resonance image taken on hospital day 2, shows acute intradural extramedullary spinal hemorrhage (white arrow) at T 11-12.

• 

  Fig. 2 Spinal angiography taken on hospital day 2, shows no remarkable evidence of spinal artery aneurysm or tumor staining (white arrow).

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  Fig. 3 Follow-up T2-weighted magnetic resonance image was taken 7 days after discharge, showing the resolving process of the subacute hemorrhage and the still existing suspicious cavernous malformation (white arrows) at T 11-12.

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