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 CASE REPORT
Year : 2016  |  Volume : 11  |  Issue : 4  |  Page : 361-363

Russell–Silver syndrome associated with low conus medullaris


1 Department of Neurosurgery, Sisli Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey
2 Department of Neurosurgery, Haseki Training and Research Hospital, Istanbul, Turkey
3 Department of Neonatology, Sisli Florence Nightingale Hospital, Istanbul Bilim University, Istanbul, Turkey

Correspondence Address:
Gokhan Canaz
Department of Neurosurgery, Haseki Training and Research Hospital, Fatih, Istanbul 34087
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.199482

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Russell–Silver syndrome is a rare heterogeneous disorder mainly characterized by intrauterine and postnatal growth retardation, craniofacial disproportion, clinodactyly, variation in urogenital development, and skeletal asymmetry. It is rare to come across tethered cord-associated Russell–Silver syndrome. We report a rare case of Russell–Silver syndrome associated with low conus medullaris in a 2-year-old patient with demonstrative phenotype. Magnetic resonance imaging indicated a low conus medullaris at the inferior border of the L3 vertebral body. Urodynamic study revealed detrusor-sphincter dyssynergia and detrusor overactivity. A decision to follow-up the patient was made because of the suspicion of tethered cord syndrome. Even though tethered cord syndrome is not a common finding in Russell–Silver syndrome, it is important to consider tethered cord syndrome to avoid scoliosis and other long-term complications.






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