A 2 years old girl presented

with cyanosis & clubbing

Presented by -

Dr. Fouzia Zaman

Assistant Registrar
Department of Pediatrics
BSMMCH,Faridpur
Particulars of patient

•Name : Shamima
•Age : 2 years
•Sex : Female
•Father's name : Rashed Haque
•Mother's name : Amena Begum
•Address : Nagarkanda, Faridpur
•Date of admission: 26.10.2022
•Date of examination: 26.10.2022
•Informant: Mother
Presenting complaints

1.Breathlessness for 4 hours

2.Bluish discoloration of lips,tongue,fingers & toes since 1 year of age.

History of present illness

• According to the statement of informant mother, her child was

reasonably well 4 days back. Then she developed sudden onset of
respiratory distress .The distress was not associated with cough and
allergic manifestations. Mother also complaints of bluish
discoloration of lip, tongue, finger & toes since her 1 year of age
which was worsen during feeding, crying, laughing and activities
initially but now symptoms deteriorate even after minimal exertion.
Contd…
• She had also history of not growing well compared to other children of
her age. She had history of same type of attacks previously which usually
occurs at morning awaking from sleep & relieved by taking squatting
position. On query mother gave history of frequent consumption of
dirt and sand for past few months .
contd….
• She had no h/o fever, cough, convulsion, headache, visual impairment,
unconsciousness & limb weakness. For these problems she consulted
with local physician and took some medications the name of which
mother cannot mentioned with no significant improvement .With these
above complaints she got admitted to BSMMCH for proper
management and evaluation.
History of Past Illness
• Nothing significant.
Birth history
• She was delivered by NVD at hospital at term with average birth
weight and had uneventful peri-natal period .
Developmental history
• Age appropriate.
Immunization history
• She was immunized as per EPI schedule.
Family history

•2nd issue of non consanguineous parents.

•Other siblings are healthy.

Feeding history
• She was on EBF ,then on complementary feeding and now on family
diet which is adequate in amount, frequency and nutritive value. For
last few months she has increased affinity for dirt and sand according
to her mother .
Socioeconomic history

•She is from low socioeconomic background.

• Lives in tin shed house , drinks tube-well water and use sanitary latrin.

• her father is an auto driver and his monthly income is about 20000 tk.
General examination
•Appearance : Ill looking ,anxious,dyspneic
• Anemia : Absent,
• Jaundice : Absent
• Cyanosis : present
• Clubbing : Present
• Koilonychia : Absent
• Leuconychia : Absent
• Edema : Absent
• Dehydration : Absent
• Bony tenderness : Absent
•Examination of eyes : Sub conjunctival congestion
Contd…
•Lymph node : Not palpable
• Skin survey : Reveals normal and BCG mark present
• Pulse : 85 / min
• BP : 75/ 45 mmHg (lies btn 50th & 75th centile)
• R/R : 50/min
• Temperature : 98 F
• Spine : Normal
• Anthropometry : Height - 74 cm (lies beiow 5th centile) ,
Weight- 6 kg (lies below 5th centile)
•BMI : 11 (lies below 5th centile)
•WAZ : -5.5
•HAZ : -2.6
Systemic examination
Cardiovascular System
Precordium
-Inspection : Precordium was not bulged
Palpation : Apex beat palpable in left 4th intercostal space, tapping in
character
-Left parasternal heave absent
- Systolic thrill absent
- palpable P2 absent
Contd…
Auscultation
-First & second heart sound audible in all 4 areas

- Ejection systolic murmur present at pulmonary area. Grade 3/6

Salient feature
• Shamima, a 2 years old girl, 2nd issue of non consanguineous parents,
immunized as per EPI schedule, belongs to low socio economic
background hailing from Nagarkanda, Faridpur got admitted at
BSMMCH on 26.10.2022 with the complaints of respiratory distress
for 4 hrs & bluish discoloration of lip,tongue,finger & toes for 1 year.
Contd..
• The distress was not associated with cough and any allergic
manifestations. Bluish discoloration developed since her 1 yr of age,
which was worsen during feeding,crying, loughing & activities initially
but now symptoms are appearing even after minimum effort.
Contd…
• She had also history of not growing well compared to other children of
her age. She had history of same type of attacks previously which
usually occurs at morning awaking from sleep & relieved by taking
squatting position. She had no h/o fever
cough,convulsion,unconsciousness, headache, visual disturbance &
limb weakness.
Contd….
• She was on EBF then complementary feeding was started & now on
appropriate family diet with special affinity for dirt and mud for last
few months.
• On examination patient was ill looking and anxious, dyspneic. Central
cyanosis present, clubbing & sub conjunctival congestion present.
Vital signs were within normal limit & was moderately stunted &
severely wasted. BMI -11 (which lies below 5th centile).
Contd…
On examination of CVS, precordium was not bulged,apex beat palpable
at left 4th intercostal space at midclavicular line, Systolic thrill
absent.1st and 2nd heart sound was audible in all 4 areas.There was an
ejection systolic murmur best heard over pulmonary area,grade 3/6.
Other systemic examination reveals no abnormality.
Provisional diagnosis
Congenital cyanotic heart disease most
probably Tetralogy of fallot with blue spell with Pica with FTT.
Differential Diagnosis
• Pulmonary stenosis
Investigations

•CBC Neutrophils 78%

Hb : 14.7 gm/ dl Eosinophil 02%

ESR: 02 mm/ 1st hr Lymphocytes 16%

Total count of WBC: 22,100/ cmm Basophil 00%

Differential count: Monocytes 04%

Platelet count : 300000/cmm

Contd..
Chest Xray P/A view
- Boot shaped heart
- Oligamic lung field
Echocardiography :
-large VSD
-Right ventricular hypertrophy
-pulmonary infundibular and vulvular stenosis
Final Diagnosis
• Tetralogy of fallot with blue spell with Pica with FTT.
Treatment
At first I have managed the blue spell with-

• Placement of the baby in knee-elbow position

• Oxygen inhalation : 3-4 l /min by face mask

• IV fluid : 10ml/kg (60 ml) normal saline

•Propranolol 1 mg/kg 6 hourly

Contd..

Supportive
•Counseling was done to parents about the disease,it's
treatment,prognosis
•High calory diet was advised
•Adequate fluid intake
•Iron supplementation : ½ TSF 12 hourly.
•Multivitamin supplementation
F/U on 2nd day (27-10-2022)
Subjective Objective Assesment Plan
No new • App- normal improving • Discharge
complain • HR - 84 with Iron and
• RR - 40 Multivitamin
• Cyanosis - supplementa
present tion
• Clubbing - • Tab
present propranolol
• Heart – • Advice for
ejection total
systolic corrective
murmur surgery as
present early as
• Lungs - clear possible
• Specific treatment :
• Surgery
- Total correction
- Palliative surgery(Blalock Taussig Shunt)