tof+brain abcess

CHAPTER I

INTRODUCTION

1.1. Background

Congenital heart disease are the most common from of the birth defects and

are the leading case of death from the birth abnormalties in the first year of life.

Although congenital heart disease are present at birth, milder defects may remain

inappreant for weeks, months, or years, and not infrequenly, escape detection until

adulthood. Congenital heart disease can be categorized as cyanotic or acyanotic.

Cyanotic refers to a blue-purple discoloration of the skin and mucous membranes

caused by an elevated blood concentration of deoxygenated hemoglobin (at least 4

g/dl, which corresponds to an arterial O2 saturation of approximately 80% to 85%). In

congenital heart disease, cyanotic results from defects that allow poorly oxygenated

blood from the right side of the heart to be shunted to the left side, bypassing the

lungs.

Tetralogy of Fallot is the most common cyanotic heart defect and the most

common cause of blue baby syndrome. Tetralogy of Fallot results from a single

developmental defect: an abnormal anterior and cephalad displacement of the

infundubular (outflow tract) portion of the interventricular septum. As a consequence,

four anomalies arise that charcterize this condition, as shown; (1) a VSD caused by

malalignment of the interventricular septum, (2) subvalvular pulmonic stenosis

because of obstruction from the infundubular septum, (3) an overriding aorta that

receives blood from both venricles, and (4) right ventricular hyperthrophy owing to

the high pressure load placed on the Right Ventricle by the pulmonic stenosis.1

In Indonesia, Tetrology Of Fallot is the fourth of the most frequent congenital

heart disease in children after ventricular septal defct, atrial septal defect, and

persistent ductus arteriosus, or approximately 10-15% of all congenital heart disease,

among cyanotic congenital heart disease, Tetralogy Of Fallot is 2/3.

One of the complication of Tetralogy Of Fallot is brain abscess. Brain abscess

is a serious life threatening infection of brain parenchyma.It results from spread of

infection from contiguous non-neuronal tissue, hematogenous seeding or a direct

introduction into the brain. Predisposing factors identified include congenital heart

disease with a right to left shunt, infections of the middle ear, mastoid, paranasal

1

sinuses, orbit, face, scalp, penetrating skull injury, comminuted skull fracture or

intracranial surgery including insertion of ventriculo-peritoneal shunts, dermal sinuses

and abnormal immune functions.2

1.2. Objective

This paper is done in order to complete the task in following the doctor's

professional education program in the department of pediatrics. In addition, providing

knowledge to the author and readers about tetralogy of fallot.

CHAPTER II

2

LITERATURE REVIEW

2.1 DEFINITION

Tetralogy of Fallot is a congenital cardiac malformation that consists of an

interventricular communication, also known as a ventricular septal defect, obstruction of the

right ventricular outflow tract, override of the ventricular septum by the aortic root, and right

ventricular hypertrophy. It is the most common cyanotic heart defect, and the most common

cause of blue baby syndrome. As such, by definition, tetralogy of fallot involves exactly four

heart malformations which present 3 :

1. Ventricular Septal Defect

The interventricular communication found in Tetralogy of Fallot exists because of

the anterior and cephalad malalignment of the outlet portion of the muscular

ventricular septum, or of its fibrous remnant should the outflow cushions fail to

muscularise during embryonic development. The resulting hole is one of a number of

those appropriately described as a malalignment defect. A hole between the two

bottom chambers (ventricles) of the heart. The defect is centered around the most

superior aspect of the ventricular septum (the outlet septum), and in the majority of

cases is single and large. In some cases thickening of the septum (septal hypertrophy)

can narrow the margins of the defect.

2. Pulmonary Stenosis

A narrowing of the right ventricular outflow tract and can occur at the pulmonary

valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis).

Infundibular pulmonic stenosis is mostly caused by overgrowth of the heart muscle

wall (hypertrophy of the septoparietal trabeculae), however the events leading to the

formation of the overriding aorta are also believed to be a cause. The pulmonic

stenosis is the major cause of the malformations, with the other associated

malformations acting as compensatory mechanisms to the pulmonic stenosis.[9] The

degree of stenosis varies between individuals with TOF, and is the primary

determinant of symptoms and severity. This malformation is infrequently described as

sub-pulmonary stenosis or subpulmonary obstruction.

3. Right Ventricular Hiperthropy

The right ventricular is more muscular than normal, causing a characteristic boot-

shaped (coeur-en-sabot) appearance as seen by chest X-ray. Due to the

misarrangement of the external ventricular septum, the right ventricular wall increases

3

http://en.wikipedia.org/wiki/Tetralogy_of_Fallot#cite_note-8

http://en.wikipedia.org/wiki/Heart




http://en.wikipedia.org/wiki/Blue_baby_syndrome

http://en.wikipedia.org/wiki/Cyanosis

in size to deal with the increased obstruction to the right outflow tract. This feature is

now generally agreed to be a secondary anomaly, as the level of hypertrophy

generally increases with age.

4. Overriding Aorta

An aortic valve with biventricular connection, that is, it is situated above the

ventricular septal defect and connected to both the right and the left ventricle. The

degree to which the aorta is attached to the right ventricle is referred to as its degree

of "override." The aortic root can be displaced toward the front (anteriorly) or directly

above the septal defect, but it is always abnormally located to the right of the root of

the pulmonary artery. The degree of override is quite variable, with 5-95% of the

valve being connected to the right ventricle.

2.2. EPIDEMIOLOGY

Tetralogy Of Fallot is the most common form of cyanotic congenital heart disease

after infancy, occuring in 3 of 10.000 live births, and is often associated with other cardiac

defects, including a right-sided aortic arch (25% of patients), ASD (10% of patients), and less

often anomalous origin of the left coronary artery. Tetralogy of Fallot is also accounts for 7–

10% of all congenital cardiac malformations.

In Indonesia, Tetrology Of Fallot is the fourth of the most frequent congenital heart

disease in children after ventricular septal defect, atrial septal defect, and persstent ductus

arteriosus, or approximately 10-15% of all congenital heart disease, among cyanotic

congenital heart disease, Tetralogy Of Fallot is 2/3. 4

2.3. ETIOLOGY

The etiology is multifactorial, includes endogen and exogen factors. Its cause is thought

to be due to environmental or genetic factors or a combination. The endogen factors are 5 :

- Chromosomal anomalies can include trisomies 21, 18, and 13, but recent experience

points to the much more frequent association of microdeletions of chromosome 22

and DiGeorge Syndrome.

- Untreated maternal Diabetes, Hyperthension, Phenylketonuria, and Intake of retinoic

acid

4

http://en.wikipedia.org/wiki/Aortic_valve

The exogen factors are :

- The history pregnancy : Join the family planning program KB oral or injection, taking

drugs without prescription (thalidomide, dextroamphetamine, aminopterin,

amethopterin, herbs)

- The mothers suffering from infectious disease : rubella

- The exposure of X-ray

2.4. PATHOPHYSIOLOGY

Fetal Circulation

There are 4 shunts in fetal circulation 6 :

• Placenta

5

• Ductus venosus

• Foramen ovale

• Ductus Arteriosus

Some important aspects of fetal circulation :

1. The placenta receives the largest amount of combined ventricular output (55%)

and has the lowest vascular resistance in the fetus.

2. Superior Vena Cava drains the upper part of the body, Inferior Vena Cava drains

the lower part of the body and placenta. O2 saturation in the Inferior Vena Cava

(70%) is higher than in the Superior Vena Cava (40%)

3. Most of Superior Vena Cava blood goes to the Right Ventricular. One third of the

Inferior Vena Cava blood is directed by the crista dividens to the Left Atrium

through the foramen ovale, the remaining two third enters the Right Ventricular

and Pulmonal Artery.

4. Less oxygenated blood in the Pulmonal Artery flows through the widely open

ductus arteriosus to the descending aorta and then to the placenta for oxygenation.

After Birth

6

As the umbilical cord is calmped or constricts naturally, the low-resistance placental flow

is removed from the arterial system, resulting in an increase in systemic vascular resistance.

Simultaneously, pulmonary vascular resistance falls for two reasons 6 :

1. The mechanical inflation of the lungs after birth stretches the lung tissues, causing

pulmonary artery expansion and wall thinning

2. Vasodilation of the pulmonary vascular occurs in response to the rise in blood oxygen

tension accompanying aeration of the lungs.

This reduction in pulmonary resistance results in a dramatic rise in pulmonary blood flow.

It is most marked within the first day after birth but continues for th next several weeks until

adult levels of pulmonary resistance are achieved.

7

As pulmonary resistance falls and more blood travels to the lungs through the pulmonary

artery, venous return from the pulmonary veins to the left atrium also increase, causig left

atrial pressure to rise. At the same time, cessation of umbilical venous flow and constriction

of the ductus venosus cause a fall in Inferior Vena Cava and right atrial pressures. As the

result, the left atrial pressure becomes greater than that in the right atrium, and the valve of

the foramen ovale is forced against the septum secundum, eliminating the previous flow

between the atrial.

With oxgenation now occuring in the newborn lungs, the ductus arteriosus becomes

superfluous and begins to constrict. During fetal life, a high circulating level of prostaglandin

E1 (PGE1) is generated in sponse to relative hypoxia, whch causes the smooth muscle of the

ductus arteriosus to relax, keeping it patent. After birth, PGE1 levels decline as the oxygen

tension rises and the ductus constricts. The responsiveness of the ductus to vasoactive

substances depends on the gestational age of the fetus.

With the anatomic separation of the circulatory paths of the right and left sides of the

heart now complete, the stroke volume of the Left Ventricular increases and that of the Right

Ventricular decrease, equalizing the cardiac output from both ventricles, the augmented

pressure and volume load placed on the Left Ventricular induces the myocardial cells of that

chamber to hyperthrophy, while th decreased pressure and volume loads on the Right

Ventricular results in gradual regression of Right Ventricular wall thickness.

Congenital Heart Disease

Congenital heart lesion can be categorized as cyanotic or acyanotic. Cyanosis refers to a

blue-purple discoloration of the skin and mucous membranes caused by an elevated blood

concentration of deoxygenated hemoglobin (at least 4 g/dl, which correcsponds to an arterial

O2 saturation of approximately 80% to 85%). In congenital heart disease, cyanosis results

from defects that allow poorly oxygenated blood from the right side of the heart o be shunted

to the left side, by passing the lungs. Acyanotic lessions include intracardiac or vascular

stenoses, valvular reguirgitation, and defcts that result in left-to-right shunting of blood.

Large left-to-right shunts at the atrial, ventricular, or great vessel level cause the pulmonary

artery volume ad pressure to increase and can be associated with the later development of

pulmonal arteriolar hyperthropy and increased resistance to flow. Over time, the elevated

pulmonary resistance may force the direction of the original shunt to reverse, causing right-

to-left flow to supervence, accompanied by the physical findings of hypoxemia and cyanosis.

8

The deveopment of pulmonary vascular disease as a result of a chronic large left-to-right

shunt is known as Eisenmenger syndrome and is described in greater detail in the final

section of the chapter. 6

Hemodynamic Acyanotic Hemodynamic Cyanotic

Tetralogy Of Fallot

9

Tetralogy Of Fallot results from a single developmental defect: an abnormal anterior

and cephalad displacement of the infundibular (outflow tract) portion of the interventricular

septum. As consequence, four anomalies arise that characterize this condition :

1. VSD caused by malalignmentof the interventricular septum

2. Subvalvular pulmonic stenosis because of obstruction from the infundibular

septum

3. An overriding aorta that receives blood from both ventricles, and

4. Right ventricular hyperthrophy owing to the hig pressure load placed on the Right

Ventricular by the pulmonic stenosis.

Increased resistance by the subvalvular pulmonic stenosis cause deoxygenated blood

returning from the systemic veins to be diverted from the Right Ventricular, through the

Ventricular Septal Defect, to the Left Ventricular , and into systemic circulation, resulting in

systemic hypoxemia and cyanosis. The magnitude of shunt flow across the Ventricular Septal

Defect is primarily a function of the severity of the pulmonary stenosis, but acute changes in

systemic an pulmonary vascular resistances can affect it as well.7

2.5. CLINICAL MANIFESTATIONS

The initial presentation of tetralogy of Fallot varies depending on the severity of the

obstruction of blood flow to the lungs. Most patients will present in the neonatal period with

mild-to-moderate cyanosis, but typically without respiratory distress. More uncommonly,

patients with very mild right ventricular outflow tract obstruction at birth may be diagnosed

at a couple months of age as the obstruction worsens resulting in newly noticed cyanosis and

a louder murmur. Because patients with tetralogy of Fallot have obstruction to pulmonary

blood flow, they will not present with signs of heart failure such as failure to thrive.

Children with tetralogy of fallot often experience dyspnea on exertion. “Spells” may

occur following exertion, feeding, or crying when systemic vasodilation resuts in an

increased rght to left shunt. Manifestations of such spells include irritability, cyanosis,

hyperventilation, and occasionally syncope or convulsions. Children learn to alleviate their

symptoms by squatiting down, which is thought to increase systemic vascular resistance by

“kinking” the femoral arteries, thereby decreasing the right to left shunt and directing more

10

blood from the Right Ventricular to the lungs. The primary symptom is low blood oxygen

saturation with or without cyanosis from birth or developing in the first year of life. If the

baby is not cyanotic then it is sometimes referred to as a "pink tet". 8

Clubbing fingers caused soft tissue growth under the nail bed as a consequence of central

cyanosis. The mechanism for soft tissue growth is unclear. Hypothesis :

Megakaryocytes present in the systemic venous blood may be responsible for the change. In

normal persons, platelets are formed from the cytoplasm of the megakaryocytes by

fragmentation during their passage through the pulmonary circulation. The cytoplasm of

megakaryocytes contains growth factors (platelet-derived growth factor and transforming

growth factor β). In patients with right-to-left shunts, megakaryocytes with their cytoplasm

may enter the systemic circulation, become trapped in the capillaries of the digits, and release

growth factors, which in turn cause clubbing. Clubbing usually does not occur until a child is

6 months or older, and it is seen first and is most pronounced in the thumb. In the early stage,

it appears as shininess and redness of the fingertips. When it is fully developed, the fingers

and toes become thick and wide and have convex nail beds .Clubbing is also seen in patients

with liver disease or subacute bacterial endocarditis and on a hereditary basis without

cyanosis. 8

11

http://en.wikipedia.org/wiki/Cyanosis

2.6. DIAGNOSIS

a. Anamnese and Physical Examination

Children with tetralogy of fallot and moderate pulmonary stenosis often have mild

cyanosis, most notaby of the lips, mucous membranes, and digits. Infants with severe

pulmonary stenosis may present with profound cyanosis in the first few days of life.

Chronic hypoxemia caused by the right-to left shunt commonly results in clubbing of

the fingers and toes. Right ventricular hyperthrophy may be appreciated on physical

examination as a palpable heave along the left sternal border. The S2 is single,

composed of a norma aortic componnt is soft and usually inaudible. A systolic

ejection murmur heard best at the upper-left sternal border is created by turbulent

blood flow through the stenotic right ventricular outflow tract. There is usually no

distinct murmur related to the VSD, because it is typically large and thus generate

little turbulence. 8

b. Diagnostic Work-Up

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- Antenatal Diagnostic

Tetralogy of Fallot can be diagnosed antenatally as early as 12 weeks of gestation.

In a population-based study, however, only half of the cases were detected during

routine obstetric ultrasonic screening. In general, patients who are referred for

foetal echocardiography with a suspicion of tetralogy of Fallot have the most

severe phenotype. Other reasons for referral for foetal echocardiography include

discovery of extra-cardiac malformations, or known chromosomal abnormalities.

As a result, patients referred for foetal echocardiography tend to have worse

outcomes when compared to patients who are diagnosed postnatally. The foetus

with tetralogy can be delivered vaginally, but efforts should be made for delivery

to occur in a centre where paediatric cardiologists are available to aid in the

postnatal care. 9

- Chest Radiograph

Chest Radiograph demonstrates prominence of the Right Ventricle and decreased

size of the main pulmonary artery segment, giving the apperance of a “boot-

shaped” hert. Pulmonary vaskular markings are typically diminshed because of

decreased flow through the pulmonary circulation. 9

- ECG

The ECG shows right ventricular hyperthrophy with right axis deviation.

13

- Echocardography

Diagnosis is confirmed with echocardiography. The severity of the subpulmonary

obstruction, its dynamic component, the size of the right and left pulmonary

arteries, and any additional sources of flow of blood to the lungs will all be

delineated. The degree of aortic override, the size of the interventricular

communication, as well as the presence of other associated lesions, will be

identified. Cardiac catheterisation is now rarely needed due to the high sensitivity

and specificity of echocardiographic images. 9

14

http://www.ojrd.com/content/4/1/2/figure/F6?highres=y

This still frame image of a parasternal short axis view of the

echocardiogram of a patient with tetralogy of Fallot demonstrates the

antero-cephalad deviation of the outlet septum into the right ventricular

outflow tract

2.7 DIAGNOSIS DIFFERENTIAL 9

- Pulmonary Atresia

- Double outlet right ventricle and pulmonary stenosis

- Transposisi of great arteri and pulmonary stenosis

2.8 TREATMENT

Management Of The Hypercyanotic Spell

Overcoming a hypercyanotic spell requires maneuvers to re-establish adequate

balance between the systemic and pulmonary flows. Treatment must focus on decreasing

pulmonary, and increasing systemic, vascular resistance, hence promoting left to right flow

across the ventricular septal defect and into the subpulmonary outlet.

Parents at home with a child suffering such spells are taught to place their child in the

knee-to-chest position in an effort to increase systemic vascular resistance and promote

15

systemic venous return to the right heart. This will theoretically increase intracardiac

shunting from left-to-right across the interventricular communication, as well as increase the

preload of the right ventricle.

Emergency services should be contacted immediately. Medical management will

consist of establishing immediate intravenous access to allow prompt administration of fluids,

which will improve right ventricular preload. Oxygen should be initiated to decrease

peripheral pulmonary vasoconstriction, and improve oxygenation once flow of blood to the

lungs is re-established. Subcutaneous morphine should be administered to decrease the

release of catecholamines. This will increase the period of right ventricular filling by

decreasing the heart rate, and promote relaxation of the infundibular spasm. If the patient

remains hypercyanotic after these measures, he or she should be paralysed and intubated,

with phenylephrine administered intravenously to increase systemic vascular resistance.

The long half-life, and potential side effects, such as hypotension and cardiac

dysfunction, of beta blockers precludes their routine use in the emergency situation.

Propranolol has been used in small doses in the chronic care of patients deemed to be at risk

for spells in an effort to minimise the infundibular spasm responsible for the episodes. Once a

patient requires prophylaxis by beta-blockade, surgical referral should occur to prevent the

potential tragic and unpredictable outcome of a hypercyanotic spell. 10

Management Of Palliation : Blalock-Tausig Shunt

16

Palliation, which frequently does not require cardiopulmonary bypass, establishes a

secure source of flow of blood to the lungs by placing a prosthetic tube between a systemic

and a pulmonary artery. The most common type of aorto-pulmonary shunt is known as the

modified Blalock-Taussig shunt. This consists of a communication between a subclavian and

pulmonary artery on the same side. A complete repair, always performed under

cardiopulmonary bypass, consists of closing the interventricular communication with a patch

channeling the left ventricle to the aortic root, relief of the subpulmonary obstruction, and

reconstruction, if necessary, of the pulmonary arteries. 10

Management Of Total Correction

Complete neonatal repair provides prompt relief of the volume and pressure overload

on the right ventricle, minimises cyanosis, decreases parental anxiety, and eliminates the

theoretical risk of stenosis occurring in a pulmonary artery due to a palliative procedure.

Patients who undergo a successful complete repair during the neonatal period will be unlikely

to require more than one intervention in the first year of life, but are not free from

reintervention. Concerns regarding such neonatal complete repairs include exposure of the

neonatal brain to cardiopulmonary bypass, and the increased need to place a patch across the

ventriculo-pulmonary junction when compared to older age at repair. Patches placed across

the ventriculo-pulmonary junction, so-called transannular patches, create a state of chronic

pulmonary regurgitation, which increases morbidity in young adults, producing exercise

intolerance and ventricular arrhythmias. If left untreated, this increases the risk of sudden

17

death. The effect of cardiopulmonary bypass on the neonatal brain, and the associated risk of

longer stay in hospital and the intensive care unit, is not trivial. Studies of

neurodevelopmental outcomes of neonates undergoing cardiopulmonary bypass compared to

older children have shown lower intelligence quotients in patients exposed to bypass as

neonates. Longer periods of bypass, and longer stays in the intensive care unit, have been

associated with an increased risk for neurological events and abnormal neurological findings

on follow-up. While some studies have not found cyanosis itself to be responsible for

cognitive problems in children with congenitally malformed hearts, others have implicated

chronic cyanosis as a factor contributing to impaired motor skills, decreased academic

achievement, and worsened behavioural outcomes. In the absence of randomised control

trials, assessing the risk and benefits of the two surgical strategies has been notoriously

difficult. 10

2.9. COMPLICATION

a. Brain Abscess

Defenition and Epidemiology

By definition, a brain abscess is an intraparenchymal collection of pus. The incidence

of brain abscesses is ∼ 8% of intracranial masses in developing countries, whereas in the

West the incidence is ∼ 1–2%. Patients with congenital cyanotic heart disease (with a right-

to-left shunt) are at risk for developing a brain abscess.] Cyanotic heart disease accounts for

12.8–69.4% of all cases of brain abscesses with identified risk factors in several series, with

the incidence being higher in children. In most series of patients from developed countries,

cyanotic heart disease is the most commonly identified risk factor for development of brain

abscess in immunocompetent patients. The incidence of brain abscess in patients with

cyanotic heart disease has been reported to range between 5 and 18.7%.11

Etiology 12

18

Predisposing Factor Causative

Neonate

Immunocompromised host

CHD

Infection

Middle ear

Sinus

Proteus spp

Citrobacter spp

Enterobacter spp

Nocardia spp

Fungi

Mycobacterium tuberculosis

S.Viridans

Microaerophilic streptococci

Haemophillus spp

Streptococci (aerobic & anaerobic)

Enterobacteriaceae

Pseudomonas spp


S. aureus

Enterbacteriaceae

19

Oral cavity

Post traumatic

Mixed anaerobic flora


S. aureus

Enterbacteriaceae

S. aureus

Streptococci spp

Enterobacteriaceae

Pathogenesis

The predisposition for brain abscesses may partially result from the fact that right-to-left

intracardiac shunts may bypass the normally effective phagocytic filtering actions of the

pulmonary capillary bed. This predisposition may also result from the fact that polycythemia

and the consequent high viscosity of blood lead to tissue hypoxia and microinfarction of the

brain, which are later complicated by bacterial colonization. The triad of symptoms of brain

abscesses are fever, headache, and focal neurologic deficit. 13

Diagnostic

Diagnostic is made by clinical presentasion, laboratorium examination and other

diagnostic examination. Plain head rontgen shows increase intra cranial pressure, also can

shows extra cerebral infection, but this examination can’t identification whether there is

abscess or not. EEG is important for localication of the abscess in the hemisfer.

Pnemoencephalography can identify abscess in cerebellum. Brain CT scan with technetium

radioisotope can identify the location of the abscess. The abscess area shows hipodense

compare to the normal area and surrounded by hiperdense layer. Nowadays MRI is the most

useful diagnostic imaging because of the accurate and fast result.14

20

Treatment

The initial management of a brain abscess includes prompt diagnosis and institution of an

antibiotic regimen that is based on the probable pathogenesis and most likely organism.

When the cause is unknown, the dual combination of a third-generation cephalosporin and

metronidazole is commonly used. If there is a history of head trauma or neurosurgery, a

combination of nafcillin or vancomycin with a third-generation cephalosporin and

metronidazole is given. The choice of antibiotics should be altered when the culture and

sensitivity results become available. An abscess resulting from a penetrating injury, head

trauma, or sinusitis should be treated with a combination of nafcillin or vancomycin,

cefotaxime or ceftriaxone, and metronidazole. Monotherapy with meropenem, which has

good activity against gram-negative bacilli, anaerobes, staphylococci, and streptococci,

including virtually all antibiotic-resistant pneumococci, is a reasonable alternative. In

contrast, the initial treatment of a lesion resulting from cyanotic heart disease is penicillin and

metronidazole. Abscesses secondary to an infected ventriculoperitoneal shunt may be initially

treated with vancomycin and ceftazidime. When otitis media, sinusitis, or mastoiditis is the

likely cause, vancomycin, because of the increasing incidence of penicillin resistance among

S. pneumoniae, in combination with a third-generation cephalosporin and metronidazole is

initially indicated until the culture results become available. When Citrobacter meningitis

(often in neonates) leads to abscess formation, a third-generation cephalosporin is used,

typically in combination with an aminoglycoside. In immunocompromised patients, broad-

spectrum antibiotic coverage is used, and amphotericin B therapy should be considered.

Surgical management of brain abscesses has changed since the advent of CT. In the

early stages of cerebritis or with multiple abscesses, antibiotics may be used alone. An

encapsulated abscess, particularly if the lesion is causing a mass effect or increased

intracranial pressure, should be treated by a combination of antibiotics and aspiration.

Surgical excision of an abscess is rarely required, because the procedure may be associated

with greater morbidity compared with aspiration of a cavity. Surgery is indicated when the

abscess is larger than 2.5 cm in diameter, gas is present in the abscess, the lesion is

multiloculated, the lesion is located in the posterior fossa, or a fungus is identified.

Associated infectious processes, such as mastoiditis, sinusitis, or a periorbital abscess, may

21

require surgical drainage. The duration of antibiotic therapy depends on the organism and

response to treatment, but it is usually is 4–6 wk.15

Surgery not only obtains pus for accurate bacteriological diagnosis but also decreases

the number of pathogens and amount of necrotic tissue present and, most importantly,

reduces the mass effect and intracranial pressure. There is a consensus that surgical treatment

is indicated for abscesses larger than 2.5 cm located in noneloquent areas and causing

significant mass effect. Freehand aspiration, stereotactic aspiration, endoscopic aspiration,

and craniotomy with excision are the surgical modalities used for treatment of brain

abscesses. The choice of treatment modality depends on the patient's status, the techniques

available, and the surgeon's experience; there is no significant difference in outcome between

aspiration and excision.

Aspiration is the gold standard for treatment of brain abscesses; it is simple and can

be easily performed via a bur hole even in critically ill patients at any stage of the abscess. In

recent series, aspiration is the most often selected method of surgical treatment. The only

contraindication for aspiration is coagulopathy. Moreover, aspiration can be repeated multiple

times.

b. Vascular Stroke

22

Vascular Stroke caused by embolization arising from thrombus in the cardiac chamber or

in the systemic veins may be associated with surgery or cardiac catheterization. Cerebral

venous thrombosis may occur, often in infants younger than 2 years who have cyanosis and

relative iron deficiency anemia. A possible explanation for these findings is that microcytosis

further exacerbates hyperviscosity resulting from polycythemia.

c. Polycthemia

Low arterial O2 content stimulates bone marrow through erythropoietin release

from the kidneys and produces an increased number of red blood cells. Polycythemia, with a

resulting increase in oxygen-carrying capacity, benefits cyanotic children. However, when

the hematocrit reaches 65% or higher, a sharp increase in the viscosity of blood occurs, and

the polycythemic response becomes disadvantageous, particularly if there is congestive heart

failure (CHF).

Some cyanotic infants have a relative iron deficiency state, with normal or lower than

normal hemoglobin and hypochromia on blood smear. A normal hemoglobin in a cyanotic

patient represents a relative anemic state. Although less cyanotic, these infants are usually

more symptomatic and improve when iron therapy raises the hemoglobin.

d. Bleeding Disorders

23

Disturbances of hemostasis are frequently present in children with severe cyanosis and

polycythemia. Most frequently noted are thrombocytopenia and defective platelet

aggregation. Other abnormalities include prolonged prothrombin time and partial

thromboplastin time and lower levels of fibrinogen and factors V and VIII. Clinical

manifestations: easy bruising, petechiae of the skin and mucous membranes, epistaxis, and

gingival bleeding. Red cell withdrawal and replacement with an equal volume of plasma tend

to correct the hemorrhagic tendency and lower blood viscosity.

e. Depressed Intelligent Quotient (IQ)

Children with chronic hypoxia and cyanosis have a lower than expected intelligence

quotient as well as poorer perceptual and gross motor functions than children with acyanotic

congenital heart defects, even after surgical repair of cyanotic heart defects.

f. Scoliosiss

Children with chronic cyanosis, particularly girls and patients with TOF, often have

scoliosis.

3.0. PROGNOSIS

Untreated, Tetralogy of Fallot rapidly results in progressive right ventricular

hypertrophy due to the increased resistance on the right ventricle.

- Approximately 25% of untreated patients with TOF and RVOT obstruction die

within the first year of life.

- 95% of patients die by 40 years.

- Delayed growth and development including puberty if untreated

This progresses to heart failure (dilated cardiomyopathy) which begins in the right heart and

often leads to left heart failure.

Patients who have undergone total surgical repair of Tetralogy of Fallot have

improved hemodynamics and often have good to excellent cardiac function after the

operation with some to no exercise intolerance (New York Heart Association Class I-II).

Surgical success and long-term outcome greatly depends on the particular anatomy of the

patient and the surgeon's skill and experience with this type of repair. Ninety percent of

patients with total repair as infants develop a progressively leaky pulmonary valve as the

heart grows to its adult size but the valve does not. Patients also may have damage to the

electrical system of the heart from surgical incisions if the middle cardiac nerve is

24

accidentally tapped during surgery. If the nerve is touched, it will cause abnormalities as

detected by EKG and/or arrhythmias.

Long-term follow up studies show that patients with total repair of TOF are at risk for

sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult

congenital cardiologist is recommended to monitor these risks and to recommend treatment,

such as interventional procedures or re-operation, if it becomes necessary.

25

CHAPTER 3

MEDICAL REPORT

3.1. Objective

The aim of this paper work is to report a case of a 13 years old boy diagnosed with

hemiparese ec brain abscess ec TOF.

3.2. Case

M.I., a boy aged 13 years old, came to Department of Child Health of Haji Adam

Malik General Hospital on 28th July 2011 at 18.07 pm with chief complaint weakness of the

right arm and right leg. This complaint had been experienced since 6 days ago. At the

beginning, patient felt weakness on his right arm and right leg when he went home and

walked by dragging his right leg. When he was admitted, the patient couldn’t stand and write.

Headache, experienced by the patient in 6 days, intermittent.

The patient experienced seizure 3 times on 24 July 2011 with interval 1 hour, the first

seizure last 5 minutes, the second and third seizure last 7 minutes, with characteristic of the

seizure is pounding of the right arm and leg, and the patient still alert when seizure happened.

The patient had been experienced shortness of breath since 3 years after doing activity

and took squatting position to relieve the symptom.

Blue on fingers and lips realized by parents since 3 years old, worsening when he did

activity like playing football and running.

26

The patient experienced difficulty in studies and had to retain 1 year in primary

school. The patient has not been developed secondary sex sign. His mother felt that his

growth development was slower than his friends.

The patient has one younger sister about 9 years old and now in healthy condition.

But his aunt has congenital heart disease.

History of Pregnancy :During pregnancy his mother never got fever and took drugs or

herbs. His mother said that she has never checked if she had diabetes mellitus and

hypertension. His mother checked the pregnancy regularly (every month) to the midwife.

According to the midwife, the pregnancy was okay.

History of Delivery : Patient was born spontaneously in term helped by midwife.

His body weight was 3,2 kg. His mother forgot the body length of the patient at birth.

According to his mother, the patient cry right after birth, and there was no cyanosis found.

History of Immunization : The mother said the immunization was complete but

she couldn’t remember what and how many times the immunization. The patient himself also

got immunization in school but he didn’t know what kind of immunization.

When he was 3 years old, his mother took him to see a cardiologist and was

diagnosed with leaky heart. The cardiologist suggested an operation but his mother refused

because of found issue. After that the patient controlled irregularly.

Patient was referred from Kumpulan Pane general hospital and has been treated there

for 4 days

History of previous disease : TOF

History of previous drug : -

BW: 28 kg, BL: 145 cm, BW/Age: 59,5%; BL/Age: 91%; BW/BL: 73,6%. Head

circumference: 49 cm

Physical examination

Presence status:

Sensorium: alert; temperature: 36,5 °C; BW: 28kg; BL: 145cm; BW/BL: 73,6%; Head

circumference 49 cm

Pale (-), Dyspnea (-), Icteric (-), Cyanosis (+), Oedematous (-)

27

Localized Status:

Head: Eye: Light reflexex (+/+), isochoric pupil, pale conjunctiva palpebra inferior (-/-),

icteric sclera (-/-)

Ear and nose: Within normal limit

Mouth: Cyanosis (+)

Neck: Lymph node enlargement (-), jugular vein pressure: R-2 cm H2O

Chest: Symetric, retraction (-)

HR: 106 bpm, regular, ejection systolic murmur grade 3/6 intercostal space II-III

linea parasternalis, pansystolic murmur grade 3/6 intercostal space IV-V linea mid

clavicularis sinisra

RR: 24 tpm, regular, rales (-)

Abdomen: Soepel, Peristaltic (+) normal, Hepar and lien: non palpable

Extrimities: Pols: 106 bpm, regular, adequate pressure/volume, warm, clubbing fingers

(+) on four extrimities, cyanosis (+)

Physiologic reflexes: (+) Normal

Patologic reflexes: -

Muscle strength: 33333 55555

33333 55555

Genitalia: Male, within normal range

Lab Result

28

29

Complete Blood

Count

Results Normal Value

Hemoglobin (Hb) 21,80 g % 10,7 – 17,1g %

Erytrocyte (RBC) 8,01 x 106/mm3 3,75 – 4,95 x106/mm3

Leukocyte (WBC) 15,22 x103/mm3 6 – 17,5 x103/mm3

Hematocrite 62,6 % 38 – 52 %

Trombocyte (PLT) 296 x103/mm3 217 – 497 x103/mm3

MCV 78,2 fL 93 – 115 fL

MCH 27,2 pg 29 – 35 pg

MCHC 34,8 g % 28 – 34 g %

RDW 13,5 14,9 – 18,7 %

MPV 10,5 fL 7,2 – 10 fL

PCT 0,31 %

PDW 12,5 fL

Neutrofil 64,4 % 37 – 80 %

Limfosit 23,2 % 20 – 40 %

Monosit 11,6 % 2 – 8 %

Eosinophil 0,4 % 1 – 6 %

Basophil 0,4 % 0 – 1 %

Neutrophil absolute 9,8 x 103/µL 1,9 – 5,4 x103/µL

Limfosit absolute 3,53 x 103/µL 3,7 – 10,7 x103/µL

Monosit absolute 1,76 x 103/µL 0,3 – 0,8 x103/µL

Eosinophil absolute 0,06 x 103/µL 0,2 – 0,5 x103/µL

Basophil absolute 0,06 x 103/µL 0 – 0,1 x103/µL

GLUCOSE Ad Random

Blood glucose 61,20 mg/dl <200 mg/dl

Chest X Ray

Head CT Scan with contrast Result (26/7/2011) RS Materna:

30

Conclusion: Intracranial SOL on left parietal area. Cystic astrocytoma, DD:Brain abscess

EEG Result (26/7/2011)

Conclusion: EEG showed diffuse irritative disorder

Working Diagnosis: Susp.Brain abscess + TOF

Therapy:

- IVFD D5% NaCl 0,45% 10gtt/I (micro)

- Inj. Cefotaxim 1gr/ 8 hour i.v. (skin test)

31

- Inj. Metronidazole LD 420mg/IV, 12 hours later MD 210mg/12 hours/ IV

- Paracetamol 3x500mg (if needed)

- Diet 1660 kkal + 56 gr protein

Diagnostic planning:

- ECG

- Echocardiography

Follow Up 29th July 2011

S : weakness of the right arm and leg, headache

O :

Sensorium: alert; temperature: 36,5 °C; BW: 28kg; BL: 145cm; BW/BL:

73,6%; Head circumference 49 cm


Localized Status:

Head: Eye: Light reflexex (+/+), isochoric pupil, pale conjunctiva palpebra

inferior (-/-), icteric sclera (-/-)


Mouth: Cyanosis (+)



HR: 100 bpm, regular, ejection systolic murmur grade 3/6 intercostal

space II-III linea parasternalis, pansystolic murmur grade 3/6 intercostal

space IV-V linea mid clavicularis sinisra



Extrimities: Pols: 106 bpm, regular, adequate pressure/volume, warm,

clubbing fingers (+) on four extrimities, cyanosis (+)


32



33333 55555


A: Hemiparese dextra ec Brain abscess ec TOF + moderate malnutrition


- Inj. Cefotaxim 1gr/ 8 hour i.v. (H2)

- Inj. Metronidazole 210mg/8 hours/ IV (H2)


- Dulcolax supp 1 time at night



- Consult to neurosurgery

- Consult to neurology

Follow Up 30th July 2011


O :




Localized Status:




Mouth: Cyanosis (+)

33













33333 55555


Answer from neurology:

A: Hemiparese dextra ec Brain abscess

Th: - Inj. Cefotaxime 1gr/ 6 hours/ IV

- Inj. Ampicillin 1 gr/ 6 hours/ IV

- Inj. Metronidazole 200 mg/ 6 hours/ IV

- Inj. Dexametasone 4,2 mg/ 6 hours for 2 days and continue oral prednisone

(2-2-2)



- Inj. Ampicilin 1gr/ 6 hours/ IV (skin test) (H1)



- Inj. Dexametasone 4,2mg/ 6 hours/iv for 2 days and continued by

prednisone 30mg/ days (2-2-2 tab) (H1)



34


- Echocardiography

Follow Up 31st July 2011


O :




Localized Status:




Mouth: Cyanosis (+)













33333 55555


35



- Inj. Ampicilin 1gr/ 6 hours/ IV (H2)



- Inj. Dexametasone 4,2mg/ 6 hours/iv for 2 days and continued by

prednisone 30mg/ days (2-2-2 tab) (H2)



Follow Up 1st August 2011

S : weakness of the right arm and leg

O :




Localized Status:




Mouth: Cyanosis (+)






36








33333 55555




- Inj. Ampicilin 1gr/ 6 hours/ IV(H3)



- Prednisone tab (2-2-2) (H1)




- Complete Blood

- Blood culture (ST)

Echocardiography result performed on 30 July 2011

Conclusion: TOF

Complete Blood Result (1 August 2011)

Hb: 21,20/ Ht: 61,20%/ Leu: 14.930/mm3/ T: 361.000/mm3

Follow Up 2nd August 2011


37

O :




Localized Status:




Mouth: Cyanosis (+)













44444 55555









38


Follow Up 3rd August 2011


O :

Sensorium: alert; temperature: 37 °C; BW: 28kg; BL: 145cm; BW/BL: 73,6%;

Head circumference 49 cm


Localized Status:




Mouth: Cyanosis (+)













44444 55555




39








- Consult to nutrition and metabolic

Follow Up 4th August 2011


O :




Localized Status:




Mouth: Cyanosis (+)









40





44444 55555


Bone Age Result:

Conclusion: Average boy










- Bone Age



O :




Localized Status:


41



Mouth: Cyanosis (+)













44444 55555


Bone Age Result:

Conclusion: Average boy

Lab result (5th August 2011)

Hb/Ht/Leu/Pla: 21,6/61,5/15410/375000

Random BG: 117mg/dL

Ureum/Creatinine/Uric acid: 23/0,48/3,5

Na/K/Cl: 138/4,7/106

Total Bil/Direct Bil/Alk.Phos/SGOT/SGPT: 1,23/0,16/225/13/19

PT/INR/APTT/TT: 17,5/1,49/30,5/14,1

Answer from Neurosurgery:

Aspiration of the abscess is planning for this patient on 8th August 2011




42





- Diet Regular food 1660 kkal + 56 gr protein


- Consult to tolerance operation

- Consult to anesthesia

- Whole Blood Count

- LFT/RFT

- Random Blood Glucose

- Electrolyte

- HST



O :




Localized Status:




Mouth: Cyanosis (+)





43




Extrimities: Pols: 84 bpm, regular, adequate pressure/volume, warm, clubbing

fingers (+) on four extrimities, cyanosis (+)




44444 55555


Lab Result: 6th August 2011

Hb/Ht/Leu/Pla: 22,1/64,6/23,1/397000

Tot.Bil/Dir.Bil/ALP/SGOT/SGPT: 0,71/0,28/182/25/19

Random BG: 90 mg/dL

Ur/Cre/Uric Acid: 38,9/0,51/3,6

Na/K/Cl: 137/4,1/103

PT/INR/aPTT/TT: 15/1,26/25,7/11,7


- IVFD D5% NaCl 0,45% 20 gtt/I (micro)






- Diet regular food 1660 kkal + 56 gr protein


- Whole Blood count, RFT, LFT, Random BG, Electrolyte

- HST

44



O :




Localized Status:




Mouth: Cyanosis (+)













44444 55555






45







O :




Localized Status:




Mouth: Cyanosis (+)













44444 55555

46


Follow up from neurosurgery: prepare the patient for aspiration of the abscess

on 9th august 2011











O :




Localized Status:




Mouth: Cyanosis (+)






47








44444 55555










Surgery was canceled because of the triage



O :




Localized Status:



48


Mouth: Cyanosis (+)













44444 55555










Surgery was canceled because of the condition of the patient


S : -

O :

49




Localized Status:




Mouth: Cyanosis (+)













55555 55555









50



S : -

O :




Localized Status:




Mouth: Cyanosis (+)













55555 55555




51


- Inj. Cefotaxim 1gr/ 8 hours i.v. (H16)






S :

O :




Localized Status:




Mouth: Cyanosis (+)












52


55555 55555











S :

O :




Localized Status:




Mouth: Cyanosis (+)






53








55555 55555










54

55

28/7/11 13 years 28 kg 145 cm

M.I.

CHAPTER IV

DISCUSSION AND SUMMARY

4.1 DISCUSSION

M.I, a 13-years-old boy, body weight of 28 kg, stature of 145 cm

Admitted to Infection Division of and Perinatology Adam Malik General Hospital,

Medan on 28th July 2011

Sign and Symptoms:

- weakness arm and leg

-seizure - blue on fingers and lips

- headache - tet spell

- clubbing fingers

The patient was diagnosed with : Hemiparese dextra ec Brain abscess ec TOF + moderate

malnutrition on clinical grounds and was treated with :

- Inj. Ampicilin 1gr/ 6 hours/ IV (skin test)

- Inj. Cefotaxim 1gr/ 8 hour i.v.

- Inj. Metronidazole 200mg/8 hours/ IV

- Inj. Dexametasone 4,2mg/ 6 hours/iv for 2 days and continued by prednisone 30mg/

days (2-2-2 tab)


56

Tetralogy of Fallot is a congenital cardiac malformation that consists of an

interventricular communication, also known as a ventricular septal defect, obstruction of the

right ventricular outflow tract, override of the ventricular septum by the aortic root, and right

ventricular hypertrophy.

In Indonesia, Tetrology Of Fallot is the fourth of the most frequent congenital heart

disease in children after ventricular septal defct, atrial septal defecr, and persstent ductus

arteriosus, or approximately 10-15% of all congenital heart disease, among cyanotic

congenital heart disease, Tetralogy Of Fallot is 2/3

The initial presentation of tetralogy of Fallot varies depending on the severity of the

obstruction of blood flow to the lungs. Most patients will present in the neonatal period with

mild-to-moderate cyanosis, but typically without respiratory distress. More uncommonly,

patients with very mild right ventricular outflow tract obstruction at birth may be diagnosed

at a couple months of age as the obstruction worsens resulting in newly noticed cyanosis and

a louder murmur.

Brain abscess is one of the complication of cyanotic heart disease. Cyanotic heart

disease accounts for 12.8–69.4% of all cases of brain abscesses with identified risk factors in

several series, with the incidence being higher in children.

The predisposition for brain abscesses may partially result from the fact that right-to-

left intracardiac shunts may bypass the normally effective phagocytic filtering actions of the

pulmonary capillary bed. This predisposition may also result from the fact that polycythemia

and the consequent high viscosity of blood lead to tissue hypoxia and microinfarction of the

brain, which are later complicated by bacterial colonization.

57

To diagnose brain abscess, first we need to anamneses the chief complain includes the

triad of brain abscess such fever, headache, and focal neurologic deficit. Then we can any

tests like Whole blood count, CT scan, MRI, and the gold standard is aspiration of the

abscess.

The initial management of a brain abscess includes prompt diagnosis and institution

of an antibiotic regimen that is based on the probable pathogenesis and most likely organism.

When the cause is unknown, the dual combination of a third-generation cephalosporin and

metronidazole is commonly used. The duration of antibiotic therapy depends on the organism

and response to treatment, but it is usually is 4–6 wks. Surgery not only obtains pus for

accurate bacteriological diagnosis but also decreases the number of pathogens and amount of

necrotic tissue present and, most importantly, reduces the mass effect and intracranial

pressure. There is a consensus that surgical treatment is indicated for abscesses larger than

2.5 cm located in noneloquent areas and causing significant mass effect. Freehand aspiration,

stereotactic aspiration, endoscopic aspiration, and craniotomy with excision are the surgical

modalities used for treatment of brain abscesses. The choice of treatment modality depends

on the patient's status, the techniques available, and the surgeon's experience; there is no

significant difference in outcome between aspiration and excision.

4.2 SUMMARY

It has been reported a case of a 13 years old boy diagnosed with hemiparese

dextra ec brain abscess ec TOF and moderate malnutrition. The diagnosis was

established based on history taking, clinical manifestation, and diagnostic tools.

Treatment for this patient was IVFD D5% NaCl 0,45% for his fluid maintenance;

cefotaxime, ampicillin, and metronidazole for eradicating of the pathogen

58

microorganism that caused the brain abscess; corticosteroid to reduce the

inflammation in the brain, paracetamol if the patient got fever; and regular food

with 1660kkal for his nutrition.

59

REFERENCES

1. World Health Organization. Tetralogy Of Fallot. 2010. Available from

http://www.who.int [accessed on July 20, 2011]

2. Mehnaz, Atiq, et. al. Clinical Features And Outcome Of Cerebral Abscess In

Congenital Heart Disease. J Ayub Med Coll Abbottabad 2006;18(2).

3. Alvaro, Z. And Mike, M. Tetralogy Of Fallot. Available from:

http:// www. emedicine health .com/ . Accessed on August 16, 2011

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Disease. Available from: http:// www. emedicine health .com/ . Accessed on July 22,

2011

5. Lily, Leonard. Pathophysiology of Heart Disease-Tetralogy Of Fallot. Philladelphia:

Lippincott Willians & Wilkins; 2007. P.390-394

6. Sherwood, Lauralee. Fisiology Manusia-Sirkulasi Janin. Jakarta: EGC; 2001. P.260-

261.

7. Wink, K. and Hutchins, G. The american Journal of Pathology Tetralogy Of Fallot.

Available from: http:// www.PudMed.com / . Accessed on July 22, 2011

8. Sierra, Jose. Tetralogy Of Fallot. Available from: http:// www.thefetus.net .com/ .

Accessed on July 22, 2011

9. Spektor, Mark. Tetralogy Of Fallot in Emergency Medicine. Available from:

http:// www. emedicine .medscape .com/ . Accessed on July 22, 2011

10. Maclean, D. Cerebral Abscess and Tetralogy Of Fallot. Available from:

http:// www.britishmedicaljournal. com/ . Accessed on August 16, 2011

11. Moorthy, R.K. and Rasjheksar, V. Management of Brain Abscess: An overview.

Avaiable from: http://www.medscape.com/viewarticle/581536_print. Accessed on:

13th August 2011.

12. Sheehan, J.P., Jane,J.A., Ray,D.K., and Goodkin, H.P. Brain Abscess in Children.

Journal of Neurosurgery: Pediatric. June 2008 Volume 24, Number 6

13. Park,M.K. Pediatric Cardiology for Practitioners. Philladelphia: Mosby Elsevier;

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14. Kamaluddin, M.T. Abses Otak. Cermin Dunia Kedokteran No. 89, 1993: 25-27

15. Haslam, R.H.A. Brain Abscess. In: Behrman,R.E., Kliegman,R.M., Jenson,H.B

Editors. Nelson Textbook of Pediatric 17th Ed. USA: Saunders; 2004.

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tof+brain abcess

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