Arch Craniofac Surg.  2012 Apr;13(1):54-56. 10.7181/acfs.2012.13.1.54.

Dandy-Walker Syndrome with Submucous Cleft Palate: A Case Report

Affiliations
  • 1Department of Plastic and Reconstructive Surgery, Wonkwang University School of Medicine, Iksan, Korea. ycnadr@hanmail.net

Abstract

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.

Keyword

Dandy-Walker syndrome; Submucous cleft palate; Double opposing Z-plasty

MeSH Terms

Airway Obstruction
Ambulatory Care Facilities
Brain
Cleft Palate
Dandy-Walker Syndrome
Female
Follow-Up Studies
Fourth Ventricle
Humans
Hydrocephalus
Live Birth
Magnetic Resonance Imaging
Necrosis
Palate, Hard
Pediatrics
Physical Examination
Physical Therapists
Respiration
Uvula
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