OSPE Peads Medicine: Tetralogy of Fallot

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OSPE PEADS MEDICINE

Case 1
The child presented with history of cyanosis since birth.
Look at the picture & answer the questions.
1. What is this condition called?
2. In which disease it is seen?
3. How will you treat the attack seen in the picture?

OSPE Peads Medicine SurgicoMed.com
Key
1. Tet Spell (Paroxysmal Hypercyanotic Spell)

2. Tetralogy of Fallot (TOF)
3. Treatment plan is as;
Hospitalization
Place the child in lateral knee-chest position
Oxygen inhalation
Subcutaneous Morphine sulphate
Propranolol
I/V Na-Bicarbonate to correct acidosis
Note
1. Tet spell is a problem during first 2 years of life in TOF. The infant becomes hyperapneic
& restless. Cyanosis increases and grasping respiration occurs. If severe may lead to
Syncope. The spell mostly occurs when child wakes up in the morning or after sleep or
during crying. During the attack, systolic murmur disappears.
2. Cardio-protective shunts (improve oxygenation if along TOF) like ASD, PDA.
Case 2
The child with known case of Tetralogy of Fallot (TOF) developed severe dyspnea &
respiratory rate more than 42/min. On examination, there was marked cyanosis & child was
restless.
1. What is your diagnosis?

2. Give its emergency management.
Key
1. Tet Spell (Paroxysmal Hypercyanotic Spell)

2. The emergency management is;
Hospitalization
Place the child in lateral knee-chest position
Oxygen inhalation
Subcutaneous Morphine sulphate
Propranolol
I/V Na-Bicarbonate to correct acidosis
Case 3
1. Give findings on above x-ray?

3. Give 3 complications of this disease?
Key
1. The findings on x-ray are;

Boot shaped heart (with up tilted apex due to RVH)
Oligemic lung fields (diminished pulmonary vascular markings)
Pulmonary artery bay
3. The complications of this disease are;
Cerebrovascular accident (CVA)
Brain abscess
Bacterial endocarditis
Polycythemia
Relative anemia
Failure to thrive
Psychological problems
Case 4
Picture of childs hand who presented with history of cyanosis since 12 hours after birth is
shown here;
1. What is obvious findings in above photograph?

2. Give your most likely diagnosis?
3. Name 3 congenital cyanotic heart diseases?
Key
1. Clubbing of fingers (Hippocratic fingers)

3. Congenital cyanotic heart diseases are
Tricuspid atresia
Transposition of great arteries (TGA)
Truncus arteriosus
Pulmonary atresia
Tetralogy of Fallot (TOF)
Note
In Tetralogy of Fallot (TOF);
1. Harsh ejection systolic murmur along left sternal border in 3rd intercostal space
2. Left parasternal heave due to RVH
3. Failure to thrive is there
Case 5
1. Which anomaly is obvious in figure B?

2. Give its two risk factors?
3. Name the murmur heard in this disease?
4. At which site in the precordium, the murmur would be best heard?
Key
1. Patent ductus arteriosus
2. Risk factors are;
Maternal rubella infection
Premature infant
Female sex
3. Continuous (Machinery murmur)
4. Left infra-clavicular area & radiating to the back
Case 6
A 5 month girl is brought to the office by her mother, who states that the girl had an episode
following feeding during which she began to breathe deeply, became blue & then lost
consciousness. The mother states that she picked her up & held her & the infant regained her
usual color & become alert. Physical examination revealed a harsh systolic murmur. The
remainder of physical examination is unremarkable.

2. Enumerate the components of this disease?
3. Name the palliative surgery used in the treatment of this disease?
Key
2. The features of this disease are;
Pulmonary infundibular stenosis
Ventricular septal defect (VSD)
Overriding aorta
Right ventricular hypertrophy (RVH)
3. Blalock Taussing shunt
Case 7
A 5 years old girl from Okara is brought with a 3 day history of fever & intermittent joint pain.
She is generally healthy but according to her mother, she had a cold about a month ago. On
physical examination, her temperature is 103.2 0F, B.P is 94/60 mmHg, pulse is 114/min &
respiration is 22/min. There is a grade III/6 murmur best heard at the apex. Multiple fine pink
macules are noted on her trunk. The macules are blanching in the middle.

2. What ECG findings will you find in this patient?
3. How will you manage this case?
4. How will you prevent this disease?
Key
1. Acute rheumatic fever
2. Reversible prolongation of R-R interval
3.
Management of Disease
General measures Strict bed rest
Salt restriction
Medical measures Salicylates
Benzathine penicillin
Corticosteroids
4.
Prevention of Disease
Primary prophylaxis Treat streptococcal pharyngitis by
benzathine penicillin for 10 days
Secondary prophylaxis Prevent recurrence by continuous
chemoprophylaxis
Case 8
A 4 months old boy gets cyanosed on crying. Examination revealed single soft 2nd heart sound
and an ejection systolic murmur close to left upper sternal border. X-ray chest reveals reduced
vascular shadows in the lungs bilaterally.
1. What does the heart sound & murmur indicate?

2. What is most likely diagnosis?
3. How will you confirm your diagnosis?
Key
1. These indicate pulmonary stenosis as 2nd heart sound is soft & there is ejection systolic
murmur.
3.
Confirmation of Diagnosis
Blood count Hb RBCs
Chest X-ray Boot shaped heart

Oligemic lung fields
ECG Right axis deviation & RVH
Echocardiography RVH, pulmonary stenosis, VSD,

overriding aorta
Case 9
A 7 days old pre-term baby is admitted in the nursery. He is receiving intravenous fluids. Being
previously well, he developed bilateral fine crepitation and hepatomegaly. A continuous
machinery murmur is heard at the left upper sternal margin.
1. What is most likely diagnosis?

2. Give one non-invasive investigation to confirm the diagnosis?
3. Enlist three management steps?
Key
1. Patent ductus arteriosus (PDA) / large one

2. Trans-thoracic echocardiography
3.
Steps of Management
Medical measures to close the duct Indomethacin (0.2 mg/kg IV 3 doses every 8-
12 hours)
RBCs increased
Treat Cardiac Failure Restrict fluids
Diuretics (furosemide)
Surgery 1 year of age using Coil or occlusion device via
cardiac catheter
If < 1 year, ligation and division
Note
Choice of surgery;
Small PDA: close to prevent endocarditis

Moderate PDA: close to prevent CCF & pulmonary disease
Case 10
A 2 years old boy is brought into the emergency room with complaint of fever for 6 days &
the development of the limp. On examination, he is found to have an erythematous macular
exanthema over his body as shown in the image. Bilateral conjunctivitis, dry & cracked lips, a
red throat & cervical lymphadenopathy. There is grade 2/6 vibratory ejection murmur at the
lower left sternal border. WBCs & differential show predominant neutrophils with increased
platelets. He later developed desquamation of skin of palms & sole around the finger tips with
edema.

2. Give the diagnostic criteria of this disease?
3. How will you manage this case?
Key
1. Kawasaki disease
2. Fever for at least 5 days + 4 of following 5 criteria
Steps of Management
Mucosal involvement Dry cracked lips, strawberry tongue, injected pharynx
Hands & Feet Edema, desquamation, redness
Eye Non-purulent bilateral conjunctivitis
Adenopathy Unilateral cervical lymphadenopathy > 1.5 cm
Rash Polymorphous truncal erythematous scarlitiform rash

3.
Supportive
Take blood culture to rule out other bacterial infections
IV gamma globulin high dose
High dose aspirin until platelet count start to decrease
Long term aspirin + Dipyridamole for coronary abnormalities
If myocardial infarction or ischemia present, do early cardiac catheterization & bypass surgery
Note
1. Kawasaki disease is the vasculitis of all blood vessels especially coronary arteries.
2. Diagnostic criteria is remembered with pneumonic My HEART
Mucosal involvement
Hands & Feet
Eyes
Adenopathy
Rash
Temperature
3. Complications include myocarditis, pericarditis, coronary aneurysm & coronary vasculitis
4. Cause of death is rupture of coronary aneurysm & myocardial infarction due to vasculitis
5. ESR, WBC & Platelets es while Hb on blood work up.
6. Echocardiography shows coronary aneurysm
Case 11
A 6 years old boy presents with colicky abdominal pain, bloody diarrhea & pain in the knee
joints. History reveals upper respiratory tract infection one week ago. On examination, a
maculopapular, non-blanching rash was observed on the legs as shown here.

2. Which renal complication can develop in this child?
Key
1. Henoch Schnlein Purpura

2. Following renal complication may occur;
Glomerulonephritis / IgA nephropathy
Nephrotic syndrome
Note
1. IgA forms in response to infections (upper respiratory) etc. & gets deposited in small
blood vessels & kidneys leading to vasculitis & glomerulonephritis.
2. Involvement of GIT blood vessels leads to colicky pain & bloody diarrhea & sometimes
intussusception.
3. In adults, skin & joint involvement and severe kidney diseases are more likely but GIT
symptoms are less. Also myocardial involvement is more common in adults.
4. Skin biopsy will show leckocytoclastic vasculitis with IgA and C3.
5. Palpable purpura is the most common and earliest feature. Later petechial lesions,
cutaneous nodules, sub-cutaneous edema and arthritis.
Case 12

2. What is genetic basis of this disease?
3. Which malignancy is predisposed if a child has this disease?
Key
1. Downs syndrome
2. Genetic basis of this disease is;
Meiotic non-disjunction (95%): Trisomy 21
Robertsonian translocation (4%)
Mosaicism due to meiotic non-disjunction during embryogenesis (1%)
3. Leukemia especially acute lymphocytic leukemia (ALL)
Note
Down syndrome may lead to following in the later life;
Hearing loss
Visual impairment
Leukemia
Atlantoaxial instability
Hypothyroidism
Celiac disease
Epilepsy
Alzheimers disease
Case 13
A 28 years old mother gave birth to a child. On physical examination, the child is alert and
moves all extremities well but is hypotonic. Abdominal examination shows umbilical hernia.
Karyotype is shown here;

2. Give 5 physical clinical features of this disease?
3. Name the abnormalities of other organs most likely found in this disease?
Key
1. Down syndrome
2. Clinical features in this disease are;
Hypotonia Small ears

Epicanthic folds Flat occiput
Upslanted palpebral fissures Simian crease & incurved 5th finger
Brush field spots in iris Wide scandal gap b/w 1st & 2nd
Flat face toes.
Protruded tongue Short neck with excessive skin fold
3.
Abnormalities in Other Organs

Heart (40%) Endocardial cushion defects
VSD
Tetralogy of Fallot (TOF)
Intestine Duodenal atresia

Hirschsprung disease
Endocrine Hypothyroidism
Eye Cataract
Strabismus
Ears Hearing loss
Case 14
At the time of delivery, a woman is noted to have a large volume of amniotic fluid. At 6
hours of age, her baby begins regurgitating small amount of mucous & bile stained fluid.
Abdominal x-ray is shown here;

2. Which syndrome is associated with this disorder?
3. Give two pre-natal screening methods to identify this syndrome during gestational
period?
Key
1. Duodenal atresia
2. Down syndrome
3. Pre-natal screening methods
Pre-natal Screening Methods

Chromosomal Analysis Amniocentesis
Chorionic villi sampling & FISH
Ultrasound (1st trimester) Shows increased nuchal translucency

Case 14
1. Give findings in above photograph?

3. What is most common genetic abnormality found in this disease?
Key
1. Following findings are there;
Flat nasal bridge
Up slanting palpebral fissure
Lower set ears
Short & broad hands
Single transverse palmer crease (Simian crease)
Epicanthic folds
2. Down syndrome
3. Meiotic non-disjunction leading to trisomy 21
Case 15
A mother brings her two & half week infant to you and tells that he sleeps most of the day
and he does not feed well. On physical examination, the infant has normal weight and length
but has enlarged head and enlarged tongue. Heart rate is 70 beats per minute. The child is
still jaundiced and has wide anterior and posterior fontanels, distended abdomen and
umbilical hernia.

2. How will you investigate?
3. How will you treat this child?
Key
1. Congenital hypothyroidism (Cretinism)
2. Investigation are in the table;
Investigations
Screening Thyroid functions tests
TSH increased
T4 decreased
Imaging X-ray Long Bones Absence of epiphyseal center
Epiphyseal dysgenesis
Large fontanelle
Wormian bone
Thyroid scan Difference b/w agenesis & ectopic thyroid
Endocrine Hypothyroidism
ECG Decreased P, T & QRS
EEG Low voltage
3.
Lifelong thyroxine replacement
Use T4 only (Sodium L-Thyroxine)
Single daily dose
Neonate: 10-15 ug/kg/day
Child: 4 ug/kg/day
Case 17
This child was born at 42nd weeks of gestation and presented with acrocyanosis and
prolonged jaundiced after birth.

2. Give etiology of this disease?
3. What do you mean by Pendred syndrome?
Key
2.
Dysgenesis or aplasia of thyroid gland
Dyshormonogenesis (Autosomal recessive)
Ectopic thyroid gland
Iodine deficiency (Endemic goiter)
End organ resistance to thyroxine
Trans-placental passage of drugs
Use of propylthiouracil during pregnancy
Maternal autoimmune thyroid disease
3. It is an autosomal thyroid dyshormonogenesis defect having defect in Organification

and is associated with Sensorineural hearing loss.
Note
For congenital hypothyroidism;
1. History: Prolonged jaundice or poor feeding

2. Symptoms: Lethargy and constipation
3. Examination: Larger anterior & posterior fontanelle, umbilical hernia
4. Commonest enzyme affected in dyshormonogenesis is Thyroid Peroxidase.
Case 18
This child was presented with constipation. History revealed poor feeding and prolonged
physiological jaundice after birth.

2. Name the drug used to treat this disease?
3. How this drug is given to child?
4. Give two important complications in untreated baby?
Key
2. L-thyroxine tablet
3. After adjusting the dose, the tablet is crushed & given to child in small amount of water
or milk.
4.
Mental retardation Epiphyseal dysgenesis
Impaired physical growth Short stature
Case 19

2. Name two diseases which might be associated with it?
3. What are its possible complications?
Key
1. Umbilical hernia
2.
Congenital hypothyroidism
Down syndrome
Mucopolysaccharide storage disease
Beckwith Wiedemann syndrome
3.
Obstruction of bowel loops Necrosis
Strangulation Sepsis
Ischemia Hemorrhage
Case 20
A 15 years old girl is referred for evaluation of short stature. Her bowel habits are not
satisfactory and she has constipation off and on. She is not performing well in school.
Menarche has not yet started. Weight is 30 kg & height is 135 cm. Her neck seems short as
her low hairline posteriorly.
1. What other three clinical findings you will look for in this girl?
2. What is your clinical diagnosis?
3. Enlist three investigations you will carry out to confirm your diagnosis?
Key
1.
Webbed neck and redundant skin on nape of neck
Shield check and widely spaced nipples
Cubitus valgus (increased carrying angle)
Multiple pigmented nevi
Signs of coarctation of aorta (Radio-femoral delay, notching of ribs)
Streaked ovaries on ultrasound
2. Turner syndrome
3.
Hormonal levels (FSH, LH, TSH) FSH raised
Chromosomal analysis (Karyotyping)
X-ray wrist (Bone age = chronological age)
Echocardiography (for coarctation of aorta)
Pelvic ultrasound scan (to see uterus etc.)
Case 21
A 12 years old girl who has short stature and delayed puberty presents to your clinic. After
doing physical examination, tests were done to identify the karyotype which is shown here;

2. What are the chromosomal abnormalities in this karyotype?
3. Give five features of this disease?
Key
1. Turner syndrome
2. X0
3.
Short stature
Low posterior hairline
Webbed neck
Shield chest
Widely spaced nipples
Cubitus valgus (increased carrying angle)
Swelling of hands and feet (Lymphedema)
Steaked ovaries
Multiple pigmented nevi
Signs of Coarctation of aorta (Radio-femoral delay, notching of ribs)
Redundant skin at the nape of the neck
Renal abnormalities (Horse shoe kidneys)
Case 22
1. Name the apparatus shown in the photograph?

2. Give indications for its use?
3. Give three complications of this procedure?
Key
1. Phototherapy machine
2.
Neonatal Pathological Unconjugated Hyperbilirubinemia
Prophylactically for low birth weight babies & severely bruised babies
In hemolytic disease of newborn
3.
Loose stools
Erythematous macular rash
Hyperthermia
Dehydration
Bronze baby syndrome
Retinal damage
Masking of cyanosis
Case 23
1. Name the apparatus shown in the photograph?

2. What its mechanism of action?
3. At which level of indirect bilirubin, this procedure is recommended?
4. What do you mean by Breast Milk jaundice?
Key
1. Phototherapy machine
2. Blue light (425-475 nm) is used which;
Converts unconjugated bilirubin into water soluble Lumirubin excreted in urine
Converts 4Z and 15Z unconjugated bilirubin by E-isomerization into water soluble
compounds.
3.
Term infants: 16-18 mg/dl
Pre-term: at somewhat low level
4. Inhibitors of bilirubin conjugation (glucuronidase, pregnanediol, free fatty acids, and
steroids) are present in some breast milk and it causes unconjugated
hyperbilirubinemia during second week of life.
Note
1. 1 g Hb produces = 34 mg of Bilirubin
2. Physical evidence of jaundice becomes visible when bilirubin = (5-10) mg/dl
Physiological Jaundice Pathological Jaundice

Appears on 2nd to 3rd day 1st day
Peak bilirubin
Term: 10-12 mg/dl Greater
Pre-term:15 mg/dl
Rate of bilirubin
< 5 mg/dl per day > 5 mg/dl per day
3. Kernicterus occurs if bilirubin levels are;

Term > 25 mg/dl
Pre-term, sepsis, hemolysis, asphyxia, hypoglycemia, sulfa drugs: < 20 mg/dl
Case 24
1. Which Neonatal reflex is being elicited in this picture?

2. Name different components of this reflex?
Key
1. Moro Reflex
2. Different components are;
Abduction Flexion
Extension Opening of hands
Adduction
Note
1. Moro reflex is obtained after 28 weeks of life
2. It disappears after 4-6 months
3. If Absent: Sedation, asphyxia, hypotonia, < 28 weeks delivery
4. If asymmetrical: Erbs paralysis
5. If exaggerated: Cerebral palsy/irritability
Case 25
1. Identity the instrument?

2. What is it use?
Key
1. Harpenden infantometer
2. To measure the length of an infant
Case 26
1. Give the positive findings in the above photograph of an infant?

2. How would you differentiate it from Caput succedaneum?
3. Should it be drained?
Key
1. Cephalhematoma
2.
Cephalhematoma Caput succedaneum

Sub-periosteal hematoma Bruising and edema of presenting part
Confined to sutures lines & one part of the skull Irregular, not confined
3. Drainage is contra-indicated as it may lead to infection.
Note
Causes of Cephalhematoma: Birth injury, coagulation and platelet disorder
Complications of Cephalhematoma: Anemia, jaundice, kernicterus
Case 27
1. Identify the obvious findings in this photograph?

2. Which vitamins deficiency leads to this condition?
Key
1. Angular Stomatitis
2.
Vitamin B2 (Riboflavin)
Vitamin B6 (Pyridoxine)
Case 28
1. Give findings in this photograph?

3. X-ray of which part of the body is very helpful in establishing diagnosis in this disease?
Key
1. Rachitic Rosary (beading of ribs, prominent costo-chondral junction)

2. Rickets
3. Wrist x-ray
Case 29
1. Give findings in this photograph?

3. Give two complications of this disease?
Key
1. Harrisons sulcus (depression above the sub-costal margin at the site of attachment of
diaphragm)
2. Rickets
3.
Respiratory infections (bronchitis, bronchopneumonia)
Pulmonary atelectasis
Anemia
Case 30
6 years old boy with the history of frequent admission due to cough and breathing difficulty.
1. Name the abnormality obvious in this photograph?

2. Name two diseases in which this abnormality can be seen?
Key
1. Harrisons sulcus (depression above the sub-costal margin at the site of attachment of
diaphragm)
2.
Rickets
Asthma
Case 31

2. Give x-ray findings?
3. Name important investigations for this disease?
Key
1. Rickets
2. X-ray of wrist showing;
Cupping
Widening
of distal ulna and ulna
Fraying
Flaring
3. Important investigations are;
Serum calcium
Serum phosphate
Plasma alkaline phosphate
25-hydroxyvitamin D (< 20 ng)
Case 32
1. Identify the disease?

2. Which nutrient is deficient in this disease?
3. Name one skin lesion found in this disease?
Key
1. Kwashiorkor
2. Protein deficiency
3. Skin lesions found are;
Flaky paint dermatitis
Ulcers
Signs of vitamin deficiency
Hypo or hyperpigmentation of covered areas
Case 33
Case 35
Case 36
Case 37
Case 38
Case 39
Case 40
Case 41
Case 42
Case 43
Case 44
Case 44
Case 45
Case 46
Case 47
Case 48
Case 49
Case 50
Case 51
Case 50
Case 52

OSPE Peads Medicine: Tetralogy of Fallot

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OSPE Peads Medicine SurgicoMed.

OSPE PEADS MEDICINE

Look at the picture & answer the questions.

1. What is this condition called?

2. In which disease it is seen?

3. How will you treat the attack seen in the picture?

1. Tet Spell (Paroxysmal Hypercyanotic Spell)

1. What is your diagnosis?

1. Tet Spell (Paroxysmal Hypercyanotic Spell)

1. Give findings on above x-ray?

1. The findings on x-ray are;

1. What is obvious findings in above photograph?

1. Clubbing of fingers (Hippocratic fingers)

In Tetralogy of Fallot (TOF);

1. Which anomaly is obvious in figure B?

1. What is your diagnosis?

1. What is your diagnosis?

1. What does the heart sound & murmur indicate?

Chest X-ray Boot shaped heart

Echocardiography RVH, pulmonary stenosis, VSD,

1. What is most likely diagnosis?

1. Patent ductus arteriosus (PDA) / large one

Small PDA: close to prevent endocarditis

1. What is your diagnosis?

Eye Non-purulent bilateral conjunctivitis

Adenopathy Unilateral cervical lymphadenopathy > 1.5 cm

Rash Polymorphous truncal erythematous scarlitiform rash

1. What is your diagnosis?

1. Henoch Schnlein Purpura

1. What is your diagnosis?

Down syndrome may lead to following in the later life;

1. What is your diagnosis?

2. Clinical features in this disease are;

Hypotonia Small ears

Abnormalities in Other Organs

Intestine Duodenal atresia

1. What is your diagnosis?

Pre-natal Screening Methods

Ultrasound (1st trimester) Shows increased nuchal translucency

1. Give findings in above photograph?

1. What is your diagnosis?

1. What is your diagnosis?

1. Congenital hypothyroidism (Cretinism)

3. It is an autosomal thyroid dyshormonogenesis defect having defect in Organification

1. History: Prolonged jaundice or poor feeding

1. What is your diagnosis?

1. What is your diagnosis?

1. What is your diagnosis?

1. Name the apparatus shown in the photograph?

1. Name the apparatus shown in the photograph?

Physiological Jaundice Pathological Jaundice

3. Kernicterus occurs if bilirubin levels are;

1. Which Neonatal reflex is being elicited in this picture?

1. Identity the instrument?

1. Give the positive findings in the above photograph of an infant?

Cephalhematoma Caput succedaneum

1. Identify the obvious findings in this photograph?

1. Give findings in this photograph?

1. Rachitic Rosary (beading of ribs, prominent costo-chondral junction)

1. Give findings in this photograph?

1. Name the abnormality obvious in this photograph?

1. What is your diagnosis?

1. Identify the disease?