SINDROME DANDY WALKER PDF

Dandy-Walker malformation affects brain development, primarily development of the cerebellum, which is the part of the brain that coordinates movement. In individuals with this condition, various parts of the cerebellum develop abnormally, resulting in malformations that can be observed with medical imaging. The central part of the cerebellum the vermis is absent or very small and may be abnormally positioned. The right and left sides of the cerebellum may be small as well.

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The Dandy-Walker malformation is a congenital present at birth defect affecting the cerebellum, the back part of the brain that controls movement, behavior, and cognitive ability. Dandy-Walker can cause obstruction of the normal drainage of cerebrospinal fluid CSF , resulting in a build-up of CSF and a condition called hydrocephalus.

The Dandy Walker malformation is part of a spectrum of conditions that include the classic form and other mild variants.

It is important to make a correct diagnosis because the different conditions may have very different outcomes. The classic form of Dandy Walker malformation has certain key features:. In addition, most children will develop hydrocephalus CSF buildup in the brain before their first birthday.

Experts still do not fully understand the causes of classic Dandy-Walker malformation and its variants. In many cases, a specific cause cannot be identified.

Potential causes include:. Hydrocephalus complicates the majority of Dandy-Walker malformations. Symptoms of hydrocephalus include:. Your physician may detect Dandy-Walker malformation in your baby with a fetal high-resolution level II ultrasound during the second or third trimester.

Tests may include:. Obtaining a MRI scan and an expert fetal neurological opinion is important because the long-term outcome for conditions resembling Dandy-Walker malformations may be much better than in cases of Dandy-Walker malformation. The Fetal Brain Program of our Prenatal Pediatrics Institute has a team of experts including a prenatal radiologist, prenatal neurologist and prenatal neurosurgeon.

This multidisciplinary team is most qualified to make this determination. The main course of treatment for Dandy-Walker malformation is to manage any complications that may arise. Treatment options may include:. The neurologists and neurosurgeons in the Fetal Brain Program at our Prenatal Pediatrics Institute have extensive experience in diagnosing and managing Dandy Walker malformations. We develop plans that ensure continuity of care from the early stages of pregnancy, after birth, and across childhood.

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Coronavirus Update: What patients and families need to know Dismiss Alert. Pediatric Dandy-Walker Malformation. What is the Dandy-Walker Malformation? The classic form of Dandy Walker malformation has certain key features: Enlarged back compartment posterior fossa of the brain Malformed or missing cerebellar vermis part of the cerebellum Large cyst in the fourth ventricle of the brain In addition, most children will develop hydrocephalus CSF buildup in the brain before their first birthday.

What Causes Dandy-Walker Malformation? Potential causes include: Chromosomal defects that affect fetal brain development Certain viral infections in the mother that pass to the developing baby Exposure of the unborn baby to certain toxins or medications Maternal diabetes Symptoms of Dandy-Walker Malformation Symptoms of Dandy-Walker malformation usually appear by age 1.

These symptoms may include: Developmental delays in motor and language skills such as sitting up, walking, and talking Poor muscle tone, balance, and coordination Problems with eye movement, mainly jerky eye movement Vision and hearing impairment Seizures Hydrocephalus complicates the majority of Dandy-Walker malformations.

Tests may include: Fetal MRI to help confirm the diagnosis and rule out other conditions e. Treatments for Dandy-Walker Malformation The main course of treatment for Dandy-Walker malformation is to manage any complications that may arise. Treatment options may include: Medications to control seizures Speech therapy to help with speech and language development Physical therapy to improve muscle strength and coordination Surgical insertion of a ventriculoperitoneal shunt in cases of severe or worsening hydrocephalus Occupational therapy to help build self-care and mobility skills such as eating, getting dressed, and walking Special education as necessary for cognitive and learning problems The neurologists and neurosurgeons in the Fetal Brain Program at our Prenatal Pediatrics Institute have extensive experience in diagnosing and managing Dandy Walker malformations.

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Dandy-Walker Syndrome Information Page

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Pediatric Dandy-Walker Malformation

Instead, a more detailed anatomic description is recommended It is known as the most common posterior fossa malformation Clinical presentation is dependent on the severity of the abnormality. In a classic Dandy-Walker malformation, patients usually manifest in the first year of life with symptoms of hydrocephalus and associated neurological symptoms. Despite severe cerebellar abnormalities, cerebellar signs are not common. Many have a sporadic inheritance with some possibly having an autosomal dominant or X-linked inheritance.

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Dandy-Walker malformation

The Dandy-Walker syndrome: considerations on four cases. Gilberto Machado de Almeida. Dandy-Walker syndrome is a particular form of hydrocephalus whose main feature is the transformation of the IV ventricle into a large cyst, which occupies almost the whole of the posterior fossa; the latter, owing to the lifting of the tentorium cerebelli and bulging of the occipital bone, is enlarged. The pathogenesis is incertain; it is generally agreed that it is originated from a developmental disorder brought about by the non-opening of the foramina of Luschka and Magendie. In the literature were found 60 cases reported. Four additional cases are reported, two of which underwent the ventriculo-jugular shunt by Spitz-Holter's technique. The main clinical signs, laboratory data and therapy are discussed.

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Dandy–Walker malformation

The Dandy-Walker malformation is a congenital present at birth defect affecting the cerebellum, the back part of the brain that controls movement, behavior, and cognitive ability. Dandy-Walker can cause obstruction of the normal drainage of cerebrospinal fluid CSF , resulting in a build-up of CSF and a condition called hydrocephalus. The Dandy Walker malformation is part of a spectrum of conditions that include the classic form and other mild variants. It is important to make a correct diagnosis because the different conditions may have very different outcomes. The classic form of Dandy Walker malformation has certain key features:. In addition, most children will develop hydrocephalus CSF buildup in the brain before their first birthday.

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