Can Dandy Walker Syndrome Be Cured

Danielle Fletcher
• Wednesday, 25 November, 2020
• 7 min read

The key features of this syndrome are an enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord), a partial or complete absence of the area of the brain between the two cerebellar hemispheres (cerebellar dermis), and cyst formation near the lowest part of the skull. Symptoms, which often occur in early infancy, include slow motor development and progressive enlargement of the skull.

dandy walker syndrome malformation brain ultrasound vermis cerebellar posterior fossa symptoms scan ventricle normal hypoplasia head fourth fetal cerebellum patient
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In older children, symptoms of increased intracranial pressure such as irritability and vomiting, and signs of cerebellar dysfunction such as unsteadiness, lack of muscle coordination, or jerky movements of the eyes may occur. Other symptoms include increased head circumference, bulging at the back of the skull, problems with the nerves that control the eyes, face and neck, and abnormal breathing patterns.

A surgical procedure called a shunt may be required to drain off excess fluid within the brain. The FINDS conducts and supports a wide range of studies that explore the complex mechanisms of normal brain development.

Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs. Instead, treatment will focus on managing certain brain abnormalities and symptoms.

Extra fluid can cause increased pressure and swelling in the brain. Some children will require physical therapy and educational help.

CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

dandy walker syndrome symptoms causes prognosis characteristics treatments interventions treatment
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I was pre-diagnosed when my mother was 20 weeks pregnant, and MRI after birth confirmed my syndrome. I joined this group in the hope to raise awareness.

My son was born with Esophageal amnesia (now repaired). A week later we found out he also had dandy walker variant, lissencephaly and spina bifida occult.

My son Sebastian was born in December 1994, in late summer 1995 he started having seizures. After a few EEG's and a brain MRI they said he has Dandy Walker Malformation.

He was a bit behind meeting his milestones and the doctors had told me not to to... It is characterized by the neuropathologic triad of (1) complete or partial agenesis of the cerebellar dermis, (2) cystic dilatation of the fourth ventricle; and (3) an enlarged posterior fossa with high position of the terrorism, lateral sinus and circular .

It has an estimated prevalence of approximately 1 per 30,000 births, and it is found in 4% to 12% of cases of infantile hydrocephalus . Dandy Walker Malformation is associated with genetic syndromes because approximately 50% of cases diagnosed prenatally were caused by a chromosome abnormality, however; the etiology is unknown, though attempts have been made to produce it in laboratory animals .

dandy walker syndrome chiari banana sign lemon head bifida
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Cerebellar brain malformations have a high prevalence of neurological, developmental, and functional disabilities involving motor, cognitive, and social behavioral deficits that correlate in general with the degree of reduction in cerebellar volume. Lesions of the cerebellar dermis are independent predictors of expressive language and gross motor delays .

Case Report A Polish 5-year-old boy, who was diagnosed with Dandy Walker Malformation at the age of 1.0 by the pediatric neurologist, was born in the 39th week of pregnancy by C-section with weight of 3650 g and 7/7/9 Appear/. The structural deformation of oral cavity (the high palate) caused difficulty with sucking and breastfeeding.

The USG during the pregnancy showed the increase of fluid spaces near the head of fetus. The USG of peripheral nervous system at the age of 4 and 5 months revealed the increased cranioventricular index (35% and 43%).

Since the age of 3 months the has been rehabilitated due to reduce muscle tension. Frontal-central site of the right hemisphere consists of synchronous sharp-waves of 300 UV in amplitude and a single sharp slow wave.

The boy (Figures 2 and 3) was diagnosed at the age of 4.1 by speech and language pathologist. Other structural changes in the organs of speech, i.e. tongue (Figures 5 and 6) and lingual and inferior labial frenulum, do not occur.

syndrome dandy walker case radiopaedia radiology cases
(Source: radiopaedia.org)

Communication functions, i.e. behavioral regulations, social interactions, joint attentions, are initiated with physical manipulations, giving, pointing, showing, gaze shifting, head nodding/head shaking, vocalizations, creating one-word (nouns). The boy is not able to negotiate, refuse, resolve own conflicts or ask for help in a socially accepted way.

), personal and possessive pronouns are understood, however, simple commands, requests and following verbal instructions are not executed. There are problems with vocabulary, naming, producing simple sentences, counting 1-10.

Discussion The aim of speech and language treatment is the development of verbal communication skills and social competence. In the first 8 appointments was performed the anamnesis, the speech and communication skills assessment and the observations of child in the natural environment (home and nursery school).

It should be stressed that the child is praised not only for the effects of activity, but also attempts to complete the task. It enables the occurrence of expressive speech acts and communicates intent before speaking first words.

i.e./too!/to express pleasure, excitement or astonishment/AAA!/to comprehension or confirmation/EEE!/to negation; onomatopoeia words to name objects or actions. The child is instructed to look at the therapist’s face in order to remember correct production of speech sounds.

dandy walker syndrome treatment
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Additionally, the hearing perception and phonological awareness is increased during the active listening training. A CD, which consists of recorded consonants in initial , medial and final position, is listened for 20 minutes/day by the child.

The treatment of expressive language disorders is effective, but the skills are still not solid. In order to increase social interaction, school performance and feeling of self-worth, the Augmentative and Alternative Communication is introduced and the Picture Exchange Communications System is applied.

The taught vocabulary is connected with everyday objects, actions, events, person, natural phenomena, quantities, material; then verbs and adjectives, few adverbs, numerals and prepositions of place, movement and time. The material is based on the rules of morphology, syntax and speech sounds which occur in the typical phonological development.

The exercises include visual discrimination, matching, distinguishing, classifying, memory, attention, and motor planning and switch activities. Additionally, the child learned how to develop positive relationships and work in team.

Conclusion The DandyWalkerSyndrome cannot be cured or repaired; therefore, the treatment should focus on individual needs, symptoms and difficulties. The aims and objectives of speech-language treatment for individuals with DandyWalkersyndrome depend on the nature of speech and language disorders; however, it should focus on developing the communication skills.

dandy walker ct syndrome agenesis vermis cerebellar
(Source: www.slideshare.net)

Berg DA, McMahan JP, Pretorium DH, Pile G (2003) Diagnostic Imaging of Fetal Anomalies. James DK, Steer CP, Water BG (2011) High Risk Pregnancy: Management Options.

Doublet PM, Benson CB (2003) Atlas of Ultrasound in Obstetrics and Gynecology: A Multimedia Reference. In: Levine MI, Frank AC (eds) Fetal and Neonatal Neurology and Neurosurgery.

Citation: Witch T, Kusama K (2018) The Speech and Language Treatment for Individuals with Dandy Walker Malformation: A Case Report. DandyWalkerSyndrome is a rare genetic, congenital brain defect affecting the cerebellum.

Its symptoms may include developmental delays in motor and language skills, poor muscle tone, problems with balance and coordination, problems with eye movement, vision and hearing impairment. The speech and language treatment should be established on the analysis of medical documents, the detailed interview questions for parents, speech and language assessment and the observation of child in the natural environment.

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