A case of Sotos syndrome with subduroperitoneal shunt.
The authors present a case of Sotos syndrome with increasing severity of subdural hygroma from the age of 5 months, which was managed with a subduroperitoneal shunt at 10 months of age. The patient had been followed up until 30 months of age with continuing improvement of symptoms. The patient initially presented with dolichocephaly accompanied by macrocrania, early tooth development, repeated pneumonia infections and developmental retardation concerning crawling, sitting, walking and speaking at 5 months of age. Magnetic resonance imaging (MRI) demonstrated partial hypoplasia of the corpus callosum and bifrontal subdural hygroma. The patient underwent subduroperitoneal shunting at 10 months of age with partial improvement of symptoms. At 18 months of age, the patient showed increased irritability and sweating, and development of spinal kyphosis, which resulted from shunt malfunction as shown in the shuntogram. The appearance of cervical syringomyelia was also seen in the MRI. After shunt revision, the irritability, sweating and kyphosis improved along with disappearance of the syringomyelia. The authors describe a case of Sotos syndrome with subduroperitoneal shunt that showed syringomyelia which developed with shunt malfunction but disappeared after shunt revision. We emphasize the importance of active management such as subduroperitoneal shunting to drain the cerebrospinal fluid in the Sotos syndrome.
Park
Seoung Woo
SW
Department of Neurosurgery, Kangwon National University College of Medicine, Chunchon, Korea.
Park
Moon Sung
MS
Hwang
Jin Soon
JS
Shin
Yong Sam
YS
Yoon
Soo Han
SH
D061085:Agenesis of Corpus Callosum; D003337:Corpus Callosum / Q000473:pathology; D019465:Craniofacial Abnormalities / Q000175:diagnosis / Q000601:surgery*; D004868:Equipment Failure; D019066:Facies; D005500:Follow-Up Studies; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011183:Postoperative Complications / Q000601:surgery*; D012086:Reoperation; D013353:Subdural Effusion / Q000151:congenital* / Q000601:surgery*; D013355:Subdural Space; D013577:Syndrome; D013595:Syringomyelia / Q000175:diagnosis / Q000601:surgery; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt / Q000295:instrumentation*