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 adulth adulthood ood.. Congen Congenita itall heart heart diseas diseasee can be catego categorize rized d as cyanot cyanotic ic or acyano acyanotic tic.. 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 develo developme pmenta ntall defect: defect: an abnorm abnormal al anteri anterior or and cephal cephalad ad displa displacem cement ent 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 malalig malalignme nment nt of the interv intervent entricu ricular lar septum septum,, (2) subval subvalvul vular ar pulmon pulmonic ic stenos stenosis is   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 heart diseas diseasee in childr children en after after ventric ventricula ularr septal septal defct, defct, atrial atrial septal septal defect 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

sinuse sinuses, s, orbit, orbit, face, face, scalp, scalp, penetr penetrati ating ng skull skull injury injury,, commin comminute uted d skull skull fractur fracturee 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

sinuse sinuses, s, orbit, orbit, face, face, scalp, scalp, penetr penetrati ating ng skull skull injury injury,, commin comminute uted d skull skull fractur fracturee 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

Tetra Tetralo logy gy of Fall Fallot ot is a cong congen enit ital al card cardia iacc malf malfor orma mati tion on that that cons consis ists ts 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 common cyanotic heart defect, and the most common cause of blue of  blue baby syndrome. syndrome . As such, by definition, tetralogy of fallot involves exactly four  heart malformations which present 3 : 1. Vent Ventri ricu cula larr Sept Septal al Defe Defect ct The interventricular communication found in Tetralogy of Fallot exists because of  the the ante anterio riorr and and ceph cephal alad ad mala malali lign gnme ment nt of the the outl outlet et porti portion on of the the musc muscul ular  ar  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 those approp appropria riately tely descri described bed as a malalig malalignme nment nt defect defect.. A hole hole betwee between n 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. Pulm Pulmon onar ary y Steno Stenosi siss 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 (hypertrophy of the septoparietal septoparietal trabeculae), however the events leading leading to the formation of the overriding aorta are also believed to be a cause. The pulmonic sten stenos osis is is the the majo majorr caus causee of the the malf malfor orma mati tion ons, s, with with the the othe otherr asso associ ciat ated ed malformations acting as compensatory mechanisms to the pulmonic stenosis. [9] The degr degree ee of sten stenos osis is vari varies es betw betwee een n indi indivi vidu dual alss with with TOF, and and is the the prim rimary ary determinant of symptoms and severity. This malformation is infrequently described as

  sub-pulmonary stenosis or   subpulmonary obstruction. 3. Right Right Ventri Ventricul cular ar Hiperth Hiperthrop ropy y The right ventricular is more muscular than normal, causing a characteristic bootshap shaped ed (coe (coeu ur-en r-en-s -sab abot ot)) appe appear aran ance ce as seen seen by ches chestt X-ra X-ray. y. Due Due to the the misarrangement of the external ventricular septum, the right ventricular wall increases 3

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.

2.3.

4

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

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 1.

6

:

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 rightto-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 10

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  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

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

12

distinct murmur related to the VSD, because it is typically large and thus generate little turbulence. 8

b. Diagnostic Work-Up -

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 “bootshaped” hert. Pulmonary vaskular markings are typically diminshed because of  decreased flow through the pulmonary circulation.

9

13

-

ECG The ECG shows right ventricular hyperthrophy with right axis deviation.

-

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

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

15

•

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 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

16

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

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

17

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 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

18

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 rightto-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

Etiology12

Predisposing Factor

Causative

 Neonate

Proteus spp

Citrobacter spp

Enterobacter spp

Immunocompromised host

 Nocardia spp

19

Fungi

Mycobacterium tuberculosis

CHD

S.Viridans

Microaerophilic streptococci

Haemophillus spp

Infection

Middle ear 

Streptococci (aerobic & anaerobic)

Enterobacteriaceae

Pseudomonas spp

Sinus

Streptococci (aerobic & anaerobic)

S. aureus

Enterbacteriaceae

Oral cavity

Mixed anaerobic flora

Streptococci (aerobic & anaerobic)

S. aureus

Enterbacteriaceae

Post traumatic

S. aureus

20

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

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, 21

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, broadspectrum 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 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. 22

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

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.

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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

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

24

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 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 re pair of  TOF are at risk for  sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult

25

congenital cardiologist is recommended to monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary.

CHAPTER 3

MEDICAL REPORT

3.1. Objective 26

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 28 th 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.

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.

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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 (-) 28

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 33333

55555

55555

Genitalia: Male, within normal range Lab Result

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Complete Blood

Results

Normal Value

Count Hemoglobin (Hb)

21,80 g %

10,7 – 17,1g %

Erytrocyte (RBC)

8,01 x 106/mm3

3,75 – 4,95 x10 6/mm3

Leukocyte (WBC)

15,22 x10 3/mm3

6 – 17,5 x10 3/mm3

Hematocrite

62,6 %

38 – 52 %

Trombocyte (PLT)

296 x10 3/mm3

217 – 497 x10 3/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 10 3/µL

1,9 – 5,4 x10 3/µL

Limfosit absolute

3,53 x 10 3/µL

3,7 – 10,7 x10 3/µL

Monosit absolute

1,76 x 10 3/µL

0,3 – 0,8 x10 3/µL

Eosinophil absolute

0,06 x 10 3/µL

0,2 – 0,5 x10 3/µL

Basophil absolute

0,06 x 10 3/µL

0 – 0,1 x10 3/µL

61,20 mg/dl