• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.54-56
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• 2012

Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

• Journal of Korean Neurosurgical Society
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• v.56 no.1
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• pp.61-65
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• 2014

Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.77-81
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• 2016

The Dandy-Walker syndrome is a relatively rare congenital malformation occurring about one in 25,000-35,000 pregnancies. It is characterized by hydrocephalus, cystic dilatation of the fourth ventricle, complete or partial absence of the cerebellar vermis, and other various extra-CNS malformations. We report a dental caries treatment of a 4-year old girl with Dandy-Walker syndrome and partial trisomy 9q. The patient visited Seoul National University Dental Hospital due to multiple caries. The cause of multiple caries is mainly presumed as patient's eating habit caused by her general condition. She was still using milk bottle because she was having difficulties swallowing solid food due to breathing problem while eating. The treatment was performed under general anesthesia considering patient's condition; delayed development, very poor cooperation and respiratory problem. The dental procedure was successfully conducted and there were no postoperative complications. In consideration of her eating habit and oral hygiene problem, regular check appointment was recommended.

• Journal of Life Science
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• v.15 no.4 s.71
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• pp.664-667
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• 2005

Hydrosyringomyelia is a dilation of the spinal cord central canal. In human it may be caused by congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as a result of infection, trauma or neoplasia. Hydrocephalus is an excessive accumulation of cerebrospinal fluid within the ventricles and occipital dysplasia is the dorsal extension of the foramen magnum. Hydrosyringomyelia and hydrocephalus can be confirmed by computed tomography or magnetic resonance imaging (MRI). A 3-year-old male maltese was presented with a history of long-term seizure. Blood examination was all unremarkable. On rostrodorsal-caudoventral oblique radiograph of the skull showed severe occipital dysplasia. On brain sonography through the persistent fontanelle, severe lateral ventriculomegaly was revealed. MRI examination revealed hydrocephalus and hydrosyringomyelia. Diuretic therapy didn't reduce clinical symptoms and surgical decompression was conducted. The dog responded well with ventriculo-peritoneal shunting. MRI is the most superior modality to diagnose hydrocephalus and hydrosyringomyelia, to plan therapy and to determine the prognosis.