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Case Study On Dandy Walker Syndrome Done by : Sajeda Moh’d
24

Case study dandy2

Apr 02, 2015

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Page 2: Case study dandy2

Objective

By the end of this presentation you will be able to:-

• Discuss the Child’s History, family history, and chief complain.

• Discuss the effect of disease on the growth and development of child.

• Define dandy walker syndrome

• Indentify the incidence of dandy walker syndrome.

• Identify the Etiologies and Predisposing Factors.

Page 3: Case study dandy2

Objectives

By the end of this presentation you will be able to:-

• Discuss the Pathophysiology of dandy walker syndrome.

• Identify the Clinical Manifestations

• Identify the Diagnostic Tests

• List the possible Complications of dandy walker syndrome.

• Discuss the Medical, Surgical, and nursing Management.

• Discuss a nursing care plan.

• Identify Health Education

Page 5: Case study dandy2

Background

S.Y 5 years old, known case of dandy walker syndrome with

ventriculoperitoneal shunt (VPS), admitted with abdominal pain,

vomits and fever since 2 weeks, her present weigh is 14.5. Her

vomits yellowish in color, complain of Flank pain which is at left

kidney side, and has history of painful urination which known as

dysuria.

Page 6: Case study dandy2

Family history

No family history of dandy walker syndrome or any brain

problems.

• Father family: has history of Hemophilia.

• Mother is a SCT.

• Child S.Y sisters have no disease.

Page 8: Case study dandy2

Definition of dandy walker syndrome

• In children with Dandy-Walker Syndrome, the cerebellum

which is the organ that regulates movements and

coordination, either partially or completely lacks the

cerebellar vermis, which is the segment that connects the

two hemispheres, or sides, of the cerebellum

Page 9: Case study dandy2

Incidence of Dandy walker syndrome

It is rare disorder that occurs in one for every 25,000 live births, mostly in females.

In Bahrain, the statistics didn’t specify the dandy walker syndrome and it shows congenital malformations, deformations and chromosomal abnormalities in general, which increase between the 1-9 ages to rich 245 cases in 2008.

Page 10: Case study dandy2

Etiology and pre-disposing factors

• Unknown cause.

• family (generic) occurrence.

• gestational exposure specifically at first trimester to

infection or taking Warfarin, and alcohol.

For S.Y the cause of her disease is unknown.

Page 11: Case study dandy2

Pathophysiology

during embryogenesis

cerebellar hemispheres and to the fourth ventricle changes

(unknown cause or nature)

cerebellar defect occur

results in blockage or atresia of the foramina of Magendie and Luschka

dilatation of the fourth ventricle & cysts formation which present in the back of the brain

Page 12: Case study dandy2

Clinical manifestations

• Vomiting.

• Convulsions.

• Irritability.

• Lack of coordination in movements.

• Head circumference enlarged and a bulge in the back of the cranium is

present.

• Hydrocephalus.

the mean clinical manifestations for S.Y are the head circumference

enlarged and present of a bulge in the back of the cranium, and

hydrocephalus.

Page 13: Case study dandy2

Diagnostic tests

Full blood count:- High WBC, indicates an inflammation and

infection. Low Red blood cells, hemoglobin, hematocrit and high red

cell size distribution indicate iron deficiency anemia and dehydration.

Manual differential: High Polymorphs, indicates infection and

inflammation.

Reticulocytes: High, in response to blood loss or treatment of iron

deficiency anemia.

Electrolytes: Normal.

Page 14: Case study dandy2

Cont.. Blood c-reactive protein: High, indicates an infection or

inflammation after surgery.

Blood cross match: O+

Fasting blood sugar: Normal.

U+E+CR: Normal

Liver function test: Normal.

CT brain: present of VP shunt at same location, and bilateral basal

ganglia calcification.

Renal Ultrasound test: present of cyst around the tip of up shunt in

the left upper abdominal quadrant.

Page 15: Case study dandy2

Complications

• The possible complications of dandy walker syndrome are:

• Raised intracranial pressure.

• Papilledema.

VPS hunt was applied to the S.Y, which is after birth, to

prevent the complication, farther enlargement of head, and

drain the CSF.

Page 16: Case study dandy2

Medical Management

1. IV.F 1250 ml ½ NS with Dextrose 5% / 24 hrs.

2. Keep NPO.

3. Maintain I/O Chart.

4. Medications: Rocephin, Gentamicin, Flagyl, Chloral hydrate,

Perfalgan, Panadol, Phenobarbitone, and Valium

Page 17: Case study dandy2

Surgical Management

o The doctors decided to apply the ventriculoperitoneal shunt (VPS)

to child S.Y after confirming the diagnosis with dandy walker

syndrome.

o Ventriculoperitoneal shunting is surgery to relieve increased pressure

inside the skull due to excess cerebrospinal fluid (CSF) on the brain.

Post operative complications

1. Infection.

2. VP shunt malfunction.

3. Increase intracranial pressure.

Page 18: Case study dandy2

VP shunt placement

Page 19: Case study dandy2

Nursing Management

Observe for sings of increase intracranial pressure, and if it happens

report the doctor immediately.

Administer prescribed medications to improve child condition.

Observe signs of infection and check vital signs Q4 hours.

Support the family by providing total information about their child.

.

Page 20: Case study dandy2

Nursing care plan:-

• Patient problem:-

Post Ventriculoperitoneal shunt complication which is infection.

• Nursing Diagnosis:-

Infection R/T ventriculoperitoneal shunt placement

• Immediate goal:-

At the end of the shift, the child will not manifest signs and symptoms

of infection such as an elevation in body temperature, and redness.

Page 21: Case study dandy2

Nursing intervention:

1. Monitor vital signs every 4 hours.

2. Maintain a Proper wound care & hygiene..

3. Make sure the dressing is clean & dry.

4. Advice the parent to give the child juices high in vitamins like

orange and lemon as she tolerate.

5. Give antibiotic such as IV Rocephin according to the Dr’s order

6. Report to Dr immediately if fever persists and wound has any signs

of infection such as redness, swelling, discharges.

Page 22: Case study dandy2

Health Education

• Observe for abdominal distention.

• Provide a balance vitamins rich diet to the child.

• The signs and symptoms of shunt failure are persistent headache,

emesis, , change in the neurologic exam, and visual changes and if it

occur inform the doctor immediately.

Page 23: Case study dandy2

• Instruct the child to avoid contact sports that may cause injury to the

shunt valve or head trauma. Instead, parents are advised to encourage

participation in low impact sports such as tennis or swimming.

• Observe signs and symptoms of bleeding and infection and if it

appear report immediately.

Page 24: Case study dandy2