Clinical Meeting on Neonatal CHolestasis

1. Dr. Shubhra Prakash Paul
MD (Ped.) Part III
Bangladesh Institute of Child Health

2. Name Ishrat
Age 5 months
Sex Female
Residence Daudkandi, Comilla
Date of Admission 08/03/2017
Date of Examination 12/03/2017
Informant Mother
Particulars of the Patient

3. • Swelling of right cheek for 11 days
• Passage of occasional pale stool from
3rd day of life
• Yellow coloration of eyes from 2nd week
of life
Presenting Complaints

4. Ishrat was admitted with the complaints
of swelling on right side of cheek for last
11 days with high grade fever without
any history of trauma. Her mother
complained of yellow coloration of both
eyes from 2nd week of life. She also
noticed passage of pale colored stool
with occasionally yellow stool from that
time. She had history of passage of high
color urine from the 2nd week of life.
History of Present Illness

5. Her mother also noticed gradual
abdominal distention for 3 months. For
these complaints Ishrat was treated with
herbal medications up to her 2 months of
age rendering no improvement. For these
problems she got admitted to DSH at her
2 months of age for evaluation and
management. In DSH she underwent for
diagnostic workup and treated with oral
medication and discharged after 2 weeks.
History of Present Illness(contd. ..)

6. From the time of discharge her condition
was static until this episode of swelling.
She had no history of delayed passage of
meconium, constipation, fever, vomiting,
malena, convulsion or family history of
such type of illness.
History of Present Illness(contd. ..)

7. There is no significant past illness.
History of Past Illness(contd. ..)

8. Birth History
Antenatal
Mother was on irregular antenatal
checkup. There was no history of
maternal fever with rash, jaundice, UTI,
PROM, PIH, GDM. Her blood group
was A positive.
Natal
Ishrat was delivered by LSCS at
term due to maternal
oligohydramnios. Birth weight was
2.3 kg.

9. Birth History (contd..)
Postnatal
Ishrat passed meconium on 1st day of
life but from 3rd day onward she
started passing occasional pale stool.
She developed Jaundice from 2nd
week. There was no history of
convulsion.

10. Feeding History
She is on exclusive breast feeding till
date.
Immunization History
Immunization is ongoing as per EPI
Schedule.
Milestone of Development
Developmentally appropriate for age.

11. Treatment History
Ishrat was treated with herbal medication
up to her 2 months of age. Then she was
admitted in DSH in December 2016. She
stayed 2 weeks in hospital and treated
with different oral medications and
advised to come for follow up. On this
admission she received injectable and
oral drugs.

12. Family History
She is the 2nd issue of her
consanguineous parents. Other family
members are healthy.
Socioeconomic History
Her father is a worker, lives abroad and
mother is a housewife. Monthly income of
the family is about 30,000/-. Family
consist of 4 members.

13. Ishrat / 5 months/ Female

14. Patient’s Stool Stool Color Card

15. Physical Examination
General Examination
Appearance Alert, playful.
Anemia Mildly pale.
Jaundice Present.
Cyanosis
Edema
Dehydration Absent
Koilonychia
Leukonychia

16. General Examination (contd.)
Skin Survey  BCG mark Present
 A soft, tender, reddish
swelling found on right side
of cheek measuring about
2cm X 2cm. Temperature
over the swelling raised.
Lymph nodes Not palpable
E.N.T. Normal
Fontanelle Open, not bulged.
Physical Examination (contd.)

17. Physical Examination (contd…)
General Examination (contd.)
Vitals
Pulse 100/min.
BP 80/55 mm of Hg (50th
centile)
Resp. Rate 20/min.
Temperature 990F

18. Physical Examination (contd…)
General Examination (contd.)
Anthropometry
Length 65 cm
Weight 6 kg
OFC 44 cm
Weight for Age (-) 1.3
Length for Age 0.28 SD
Weight for Length (-) 0.25 SD

19. Physical Examination (contd.)
Ishrat

20. Systemic Examination
Examination of Alimentary System
Mouth and Normal. No bleeding
bleeding spot was
seen
Abdomen
Inspection
Abdomen was distended.
Umbilicus centrally placed,
inverted.

21. Systemic Examination
Examination of Alimentary System (contd.)
Abdomen (contd.)
Palpation
Abdomen was soft, non tender.
Liver Enlarged in size measuring about
7 cm along right mid clavicular line
from costal margin with upper border
border of liver dullness on 5th
intercostal space, non tender, firm in
consistency, sharp margin, smooth
surface.

22. Systemic Examination
Examination of Alimentary System (contd.)
Abdomen (contd.)
Palpation
Spleen Enlarged about 4 cm along its
its long axis having smooth
surface, firm consistency, sharp
margin, notch present.
Kidneys Not ballotable.
Urinary bladder Not palpable.

23. Systemic Examination
Examination of Alimentary System (contd.)
Abdomen (contd.)
Percussion
Tympanitic.
Auscultation
Bowel sound present. There was no
hepatic or splenic bruit.

24. Systemic Examination
Examination of other systems revealed
normal findings.

25. Ishrat a 5 months old female infant, 2nd
issue of consanguineous parents was
presented with right sided cheek swelling
for 11 days along with jaundice,
intermittent passage of pale stool and
passage of high color urine from 2nd
week of life. She was delivered at term
without any history of delayed passage of
meconium. She had no history of
convulsion, constipation, similar family
history.
Salient Features

26. She was mildly pale, icteric with normal
vitals and mild wasting. A soft, tender
swelling with raised local temperature
over right cheek. She had
hepatosplenomegaly without ascites.
Salient Features (contd.)

27. Neonatal Cholestasis due to Neonatal
Hepatitis Syndrome with abscess on right
cheek.
Provisional Diagnosis

28. • Biliary Atresia with abscess on right cheek
• Choledochal cyst with abscess on right
cheek
Differential Diagnosis

29. Complete Blood Count
Hemoglobin 8.3 gm/dL
Total count of WBC 17,800 /cu mm
Diff. count of WBC
N -
61%
L –
31%
M -
04%
E –
Platelet count 4,80,00
0
/cu mm
Film
RBC – Anisocytic, anisochromic.
Laboratory Investigations

30. Biochemical
Prev.
Adm.
10/03/17
Serum Bilirubin (Total)
(mg/dL)
10.8 7.4
Direct Bilirubin 3.4 2.2
Indirect Bilirubin 7.4 5.2
Serum Alk. Phosphatase
(U/L)
520 287
SGPT (U/L) 350 287
SGOT (U/L) 479 375
Laboratory Investigations

31. Coagulation Profile
12/03/1
7
Prev.
Adm.
Bleeding Time (min.) 3
Clotting Time (min.) 6
Prothrombin Time (sec.)
Patient 13 11
Control 12 12
Laboratory Investigations
Blood group & Rh type
A positive

32. Others
RBS 4.5 mmol/L
TORCH screening Negative
Urine RE Normal
Urine for Reducing
substance
Absent
Laboratory Investigations

33. USG of Hepatobiliary System
(on 26/12/2016) [Previous admission]
Laboratory Investigations
Liver Liveris enlarged 7.8 cm, with
raised parenchymal echo.
Intrahepatic biliary trees are
not dilated.
Gallbladder Gallbladder is visualized. Wall
is thickened. No organic lesion
seen.
CBD Common bile duct is not
dilated.

34. USG of swelling on right cheek with
Doppler study (on 08/03/2017)
Laboratory Investigations
 Right Face swelling- Contains
irregular contour, hypoechoic
fluid suggestive of abscess
 Thickened echogenic
tissue due to cellulitis
 No flow is seen within the mass.

35. Cholescintigraphy (reported on 26/12/2017)
Findings:
Liver appears to be enlarged in size
showing fairly uniform tracer
concentration. Persistence of cardiac
pool activity was seen up to 32 minutes
images. Gall bladder and bile ducts
were visualized. Tracer activity within
the bowel loops was observed.
Laboratory Investigations

36. Cholescintigraphy (contd.)
Impression:
Persistent cardiac blood pool suggesting
parenchymal insufficiency.
Laboratory Investigations

37. Laboratory Investigations
Liver Biopsy
Microscopic Examination
 Section reveals focal degeneration of
hepatocytes and prominent
parenchymal cholestasis as well as
few multinucleated giant cells.
 The portal tract shows chronic
inflammatory cellular infiltrate
predominantly lymphocytes.
 No malignancy is seen.

38. Laboratory Investigations
Liver Biopsy (contd.)
Impression
Liver tissue (core biopsy):
Consistent with neonatal hepatitis.

39. Neonatal Cholestasis due to
Neonatal Hepatitis Syndrome with
abscess on right cheek
Final Diagnosis

40. Management
Counseling
Supportive treatment
 Diet Breast feeding on demand.
Medium chain triglycerides (mixed with
expressed breast milk)
 Inj. Cefuroxime (150 mg/kg/day tds.)
250mg IV 8 hourly
 Inj. Flucloxacillin (100 mg/kg/day qds.)
125 mg IV 6 hourly

41. Management
Supportive treatment
 Syrup Ursodeoxycholic acid (15-
30mg/kg/day) 3 mL
once daily
 Syrup Phenobarbitone ( 5mg/kg/day)
3 mL 12 hourly
 Cap. Vitamin A (10,000-15,000 IU/day)
2 drops/day
 Vitamin D (Cholecalciferol) (400 - 1200
IU/day)
2ml 12 hourly
 Cap. Vitamin E (50 - 400 IU/day)

42. Management
Supportive treatment
 Inj. Vitamin K
2 mg I/V every alternate day
 Multivitamin drop
5 drops 12 hourly
 Syrup Zinc (2mg/kg/day)
2.5mL 12 hourly
 Tab. Folic Acid (0.2 mg/kg/day)
1/4 tablet daily
 Calcium Tablet (250mg) (50mg/kg/day)
1tablet daily

43. Follow up
Clinical Laboratory
Vital signs
Liver size
Stool color
Urine color
 S. Bilirubin
 SGPT
 S. Alkaline
Phosphatase
 Prothrombin Time