J Korean Neurosurg Soc.  2008 Oct;44(4):240-244. 10.3340/jkns.2008.44.4.240.

The First Neurosurgical Analysis of 8 Korean Children with Sotos Syndrome

Affiliations
  • 1Department of Neurosurgery, Ajou University School of Medicine, Suwon, Korea. ee80@ajou.ac.kr

Abstract


OBJECTIVE
Sotos Syndrome is characterized by macrocephaly, overgrowth, and developmental delay, and more than 300 patients have been reported worldwide to date. The authors reviewed the clinical characteristics of 8 patients with Sotos Syndrome in Korea for a new understanding and treatment strategies.
METHODS
The medical records of a total of eight Korean children with Sotos Syndrome were reviewed. All patients underwent developmental checkup, lumbar punctures for measurement of intracranial pressure (ICP), brain and spine magnetic resonance imaging and computerized tomography.
RESULTS
All 8 patients showed macrocephaly and the characteristic craniofacial features of Sotos Syndrome. Other clinical characteristics shown were overgrowth (7/8), developmental delay (7/8), congenital heart defect (3/8), flat foot (8/8), scoliosis (4/8), spina bifida (8/8), hydrocephalus (4/8), cavum vergae (3/8), and increased subdural fluid collection (5/8). Mean ICP measured via lumbar puncture was 27.35+/-6.25 cm H2O (range 20 to 36 cm H2O). Two patients received ventriculo-peritoneal shunt, and 1 patient underwent subduro-peritoneal shunt with improvement. Spinal orthosis was applied to 4/5 patients with scoliosis and 4/8 children with flat foot were provided with foot orthosis.
CONCLUSION
In this first Korean study of 8 Sotos Syndrome patients we demonstrated the presence of spina bifida and increased ICP, which had not been previously described. The authors therefore suggest that all patients with Sotos Syndrome should undergo examination for the presence of spina bifida, and that shunt procedures would improve development and alleviate clinical symptoms.

Keyword

Sotos Syndrome; Spina bifida; Intracranial pressure; Hydrocephalus; Shunt

MeSH Terms

Brain
Child
Flatfoot
Foot Orthoses
Heart Defects, Congenital
Humans
Hydrocephalus
Intracranial Pressure
Korea
Macrocephaly
Magnetic Resonance Imaging
Medical Records
Orthotic Devices
Scoliosis
Sotos Syndrome
Spinal Dysraphism
Spinal Puncture
Spine
Ventriculoperitoneal Shunt
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