BAB I

BEGINNER

There are a variety of known congenital neurological disorders. The disorders have
clinical manifestations and a multidisciplinary prognosis. One such congenital neurological
disorder is the dandy walker syndrome.

Dandy walker syndrome is a congenital aberration that occurs, about 1 per 25,000-
35,000 live births, which are about the cerebellum and ventricle IV. Dandy Walker Syndrome
is characterized by the presence of agenesis of the cerebelli vermis, cystic dilatation of the IV
ventricle as well as posterior fossa enlargement. This disorder can appear dramatically or
without significant symptoms.

Symptoms often appear early in development after birth, in the form of motor
development delays and progressive enlargement of the head. Other symptoms that may arise
are due to increased intracranial pressure (ICT) such as vomiting and anxiety as well as
impaired cerebellum function such as imbalance, lack of muscle coordination or involuntre
movement in the eye.

Treatment for patients with Dandy Wilker Syndrome is generally intended to address
the problems of the participant, if necessary. VP shunt installation (VP shunt) may be needed
to drain the excess of the cerebrospinal liquor (LCS), reduce ICT and help control the
enlargement of head arrhythmias.

The effect of DWM on the development of intelligence varies greatly: some have
normal intelligence while others have never experienced normal development of intelligence,
despite initial therapy. The magnitude of life expectancy is affected by the severity of this
condition and the presence or absence of other accompanying malformations. The presence
of multiple congenital abnormalities may shorten the patient's life expectancy. Although there
are authors who mention less than 25% of patients who died because of the disorder.

Computed tomography (CT) is used to diagnose and evaluate shunt function in shunt-
mounted patients. Head CT examination without the use of contrast material is sufficient to
determine the components of the dandy walker syndrome. Given the importance of the role of
radsiology in diagnosing, and given the rare cases and the implications of the diagnosis of the

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prognosis and management of patients with dandy walker syndrome, the knowledge of a
radiologist regarding this disorder is important

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 BAB II

LITERATURE REVIEW

2.1.Defenition
Dandy Walker Syndrome is a rare congenital anomaly that affects the
cerebellum and ventricle IV. This disorder is characterized by the presence of
agenesis or cerebelli vermis hypoplasia, cystic dilatation of the IV ventricles and
enlargement of the fossa. This disorder was first described by Blackfan and Dandy in
1914, but the new terminology was given by Benda in 1954.

2.2.Epidemology
The prevalence of Dandy Walker Syndrome is estimated to be about 1 per
25,000-35,000 live births and is 4-12% of all cases of hydrocephalus in infants. Men
are slightly more likely to develop this disorder than women.

2.3.Etiology and pathofiology

Malophageation occurs as a result of an obvious, yet clearly known, disorder
of the development of hemisphere and vertex during embryogenesis. There are several
theories about the etiology of the malformations. Predisposing factors for dandy
walker syndrome include exposure to rubella, cytomegalovirus, toxoplasmosis,
warfarin, alcohol and isotreinoin during the first trimester of pregnancy.
A theory says the cause of dandy walker syndrome is due to a blockage in the
foramina magendie or Luschka during embryogenesis. Other theories explain because
of a stagnation of hindbrain development in weeks 7 to 10 kehamimlan. Dandy wlaker
syndrome can also occur with regard to genetic inheritance disorders and genetic
disorders, such as partial deletions on chromosome 13, trisomy 13, 18 and 21. Other
authors mention that dandy walker syndrome is sporadically lowered and is thought to
be dominant or bound to the X chromosome.
Opportunities for the birth of a child with dandy walker syndrome in women
who have a history of childbearing children with this disease is 1-2%.

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2.4.Risk
Dandy walker syndrome can be inherited. If the parent has a dandy walker
syndrome, the child is at higher risk of the disease. Other risk factors have not been
identified.

2.5.Manifestation clinic
The clinical picture of dandy walker syndrome depends on the severity of the
disorder occurring. In the dandy walker's classic syndrome, manifestations appear in
the first year of life in the form of hydrocephalus symptoms and associated
neurological disorders. Ventriculomegaly occurs in 80% of cases and hydrocephalus
in 90%. In addition to providing neurological problems, recent studies indicate an
association between mild forms of dandy walker syndrome, dandy walker variant
(DWV), and psychiatric disorders, such as bipolar disorder and psychosis, especially
schizophrenia.
In dandy walker syndrome occurs ventricular cystic dilatation IV. This causes
the physical examination of this disease is obtained a large head shape with prominent
occipital parts

2.6.Therapy
Treatment in patients with dandy walker syndrome is generally intended to
address comorbid problems, when necessary. VP-shunt installation may be necessary
to drain the excess of the cerebrospinal liquor (LCS), decrease ICT and help control
head size enlargement.

2.7.Diagnosis
To diagnose Dandy Walker Syndrome, classic triads must be found: agenesis
or hypoplacy of cerebellar vermis, posterior fossa cysts that are directly related to IV
ventilation with cystic dilatation. Another picture that can be encountered is the small,
spaced hemispheric cerebellis, the deviation of the cerebelli hemispheres to the
anterolateral, the pons to the anterior deviation, the elevated transverse sinuses with
the bones in the posterior fossa thinning and prominent, the scaloping of the oss
petrosus pyramid and the assessment of Presence or absence of hydrocephalus
conditions.

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 In 80% of cases, the diagnosis is established in the first year of life.
Radiological characterization was initially based on the results of
pneumoencephalography. Magnetic resonance imaging (MRI) is the preferred
modality for diagnosis, but computed tomography (CY) is capable of showing
adequate features for diagnosis. Distinguishing cystic structures in the posterior fossa
is even more difficult with MRI than CT Scan. Because of the wide availability of the
pliers, CT Scan is mostly used to diagnose this disorder.
CT Scan is used to diagnose and evaluate shunt function in shunt-mounted
patients. There is no specific technique in visualizing Dandy Walker Syndrome on CT
Scan head examination and CT Scan head without the use of contrast material is
sufficient to determine the components of dandy walker syndrome. CT Scan is said to
be very good and has been able to distinguish dandy walker syndrome from other
posterior fossa abnormalities, but distinguishing the CT Scan image alone is difficult.
As a result of the agenesis or hypoplasia of the cerebelli vermis, the IV
ventricle appears to be directly related to cisterna magna. The ventricular feature of
IV which is directly related to the posterior fossa cyst (cisterna magna) is particularly
characteristic in dandy walker syndrome, but this picture can also be seen in CT scans
of axial fragments in the event of a cerebral vermis rotation toward the superior. This
makes observation on axial pieces alone susceptible to errors to diagnose dandy
walker syndrome. To avoid errors in diagnosing hypoplasi or resoresis of cerebellum
vermis, need to illustrate other areas (coronal or sagittal, or both) and caution on
observation of superior axial CT scans from ventricular IV, to avoid being trapped by
vermis cerebelli rotation to superior.

2.8.Differential Diagnosis
Dandy-Walker Variant (DWV), mega cisterna magna (MCM) and posterior
fossa arachnoid cyst (AC) along with Dandy Walker Malignancy (DWM) are a group
of disorders called Dandy Walker Complex (DWC).
DWV is a milder form of DWM. This disorder is characterized by a normal or
slightly enlarged posterior fossa, dilated by normal posterior or slightly enlarged
posterior fossa, mild to moderate dilation of the IV ventricle, normal cerebellar
hemispheres and hypoplasi instead of vermis cerebelli agenesis. There is very little
hydrocephalus in this disorder. In MCM, there is a large posterior fossa due to
enlargement of cisterna magna with normal cerebelli and ventricular IV vermis.

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 Arachnoid cyst (AC) is a benign cyst that occurs in the cerebrospinal to the
arachnoid membrane and is not associated with the ventricular system. The air
conditioner contains a very colorless colorless fluidproof LCS. True retrocerebellar
air conditioning will devalue the IV ventricles and the cerebellum toward the anterior.
Since operative therapy for AC and DVM is very different, distinguishing the two
entities is crucial.

2.9.Prognosis
The influence of the walker syndrome on intellectual development varies,
some children have normal cognitive and others never achieve normal intellectual
development even though hydrocephalus is premature and handled correctly. Survival
depends on the level of fear and malformation. An innate multi-organ disorder may
shorten the life span.

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 BAB III

CONCLUSION

Dandy Walker Syndrome is a rare congenital anomaly that affects the cerebellum and
ventricle IV. This disorder is characterized by the presence of agenesis or cerebelli vermis
hypoplasia, cystic dilatation of the IV ventricles and enlargement of the fossa. The prevalence
of Dandy Walker Syndrome is estimated to be about 1 per 25,000-35,000 live births and is 4-
12% of all cases of hydrocephalus in infants. Men are slightly more likely to develop this
disorder than women.

A theory says the cause of dandy walker syndrome is due to a blockage in the
foramina magendie or Luschka during embryogenesis. VP-shunt installation may be
necessary to drain the excess of the cerebrospinal liquor (LCS), decrease ICT and help
control head size enlargement. To diagnose Dandy Walker Syndrome, classic triads must be
found: agenesis or hypoplacy of cerebellar vermis, posterior fossa cysts that are directly
related to IV ventilation with cystic dilatation. Another picture that can be encountered is the
small, spaced hemispheric cerebellis, the deviation of the cerebelli hemispheres to the
anterolateral, the pons to the anterior deviation, the elevated transverse sinuses with the bones
in the posterior fossa thinning and prominent, the scaloping of the oss petrosus pyramid and
the assessment of Presence or absence of hydrocephalus conditions. Survival depends on the
level of fear and malformation. An innate multi-organ disorder may shorten the life span.

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 REFERENSES

1. Behrman R.E:et.al:Text Book of Pediatrics; Ed Nelson W.E, W.B Saunders

Philadelphia, 1983, p 378-381.
2. Geeraets W.J : Ocular Syndrome; 3rd Edition, Departement of Opthalmology,
Departement of Biophysics, Medical collage of virginia, Virginia Commonwealth
Universoty Richard, Virginia
3. Incesu L. Et.al: Dandy Walker Malformation: departement of Radiology, Oudokuz
Mayis University School of Medicine, Turkey. www.emedicine.com October 2003.