CASE OF
DR. NILOY BANERJEE, PGT DR. S. K. DAS, ASSISTANT PROFESSOR DR. UDAS GHOSH, ASSOCIATE PROFESSOR DR. K. CHATTERJEE, PROFESSOR DEPT. OF MEDICINE R.G.KAR MEDICAL COLLEGE,KOLKATA
Patients
13 yrs Girl Hindu Student
particulars
CHIEF
COMPLAINTS
prior to admission
HISTORY ILLNESS
OF
PRESENT
abdomen started six
months ago - insidious in onset -gradually progressive -dragging in nature -associated with early satiety
Contd
There was history of one episode of
blood-vomiting seven days ago . No h/o weight loss, fever, jaundice, abdominal or pedal swelling, pruritus, skin rash, bleeding from any site, joint pain or swelling. No h/o hypertension or diabetes mellitus. Bladder and bowel habit-WNL.
PAST
HISTORY
surgical operation including neonatal umbilical vein catheterization. No history suggestive of intra-abdominal infection.
PERSONAL
HISTORY
Non - contributory
FAMILY
HISTORY
TREATMENT HISTORY
No H/O any regular drug intake
MENSTRUAL HISTORY
Menarche not achieved yet.
ON EXAMINATION:GENERAL EXAMINATION
Pt is conscious, alert and co-operative Nutrition - average A++ J0 Cy0 Cl0 Oe0 P.R.-84/m B.P.-110/70 mm Hg Temperature - normal R.R.-16/m. Neck vein-normal Lymph nodes- not palpable Skin - normal.
GASTROINTESTINAL SYSTEM
A. UPPER G. I. TRACT : WNL
B. EXAMINATION OF ABDOMEN :
No venous prominence over abdomen and back
OTHER
SYSTEMS
CHEST-WNL CVS-WNL
CNS-WNL
LYMPHO-RETICULAR SYSTEM- No bony
Summary:
13 yr old girl presented with left upper abdominal dragging pain with early satiety
for six months and history of one episode of hematemesis. Past , personal and family history were non-contributory. On examination, moderate pallor present, other parameters of general examinations were within normal limits. On systemic examination, massive splenomegaly was present. Other systemic examinations were within normal limits.
POSSIBILITIES???
DIFFERENTIAL DIAGNOSES
EXTRA-HEPATIC PORTAL VENOUS
OBSTRUCTION
NON-CIRRHOTIC PORTAL FIBROSIS CHRONIC HEMOLYTIC ANEMIA
MYELO-PROLIFERATIVE DISORDER.
LABORATORY
DATA
MCV-91.3 fl, MCH29.3 pg, MCHC32.3% FBS - 79 mg% Urea - 18 mg% Creatinine - 0.67mg% LFT - WNL PT 13.2 s Bone marrow studyWNL.
Hb- 8.38 g% TLC-3500/cu.mm N75 L22 E02 M01 B00 4/ Platelet-15x 10 cmm ESR- 15 mm/ h Malaria parasite- not found PCV- 28.5 Reticulocyte count 1.2% ;index 0.9
Contd
USG of abdomen : - Liver- normal in size and shape. normal
echotexture - Portal vein diameter -10 mm at porta - Huge splenomegaly. - No free fluid.
Contd
Coombs test -- negative
method) WNL
Contd
OESOPHAGO-GASTRO-DUODENOSCOPY: - Grade III oesophageal varices
Contd
SPLENO-PORTAL DOPPLER STUDY: - Massive splenomegaly - Enlarged splenic vein ( at confluence 8
Contd
CT SCAN OF ABDOMEN ( done 4 weeks
after spleno-portal doppler study ): Massive splenomegaly Portal vein diameter 12 mm Portal cavernoma formation Increased splenic collateral veins No free fluid.
Splenic
collateral
vein
Contd
COAGULATION PROFILE ( done after 6 wks of her admission): -homocysteine - 8.28 mmol/L (normal 3.7- 13.9 mmol/L ) -cardiolipin antibody IgM-2.8 MPL ( normal 0-15 MPL ) & IgG-3.2 GPL (normal 0-15 GPL ) -antithrombin III WNL -Factor V Leiden mutation not detected. -protein C activity-61% ( normal-67-195% ) -protein S activity-40% ( normal-55-123% )
FINAL
DIAGNOSIS
PORTAL HYPERTENSION DUE TO EXTRAHEPATIC PORTAL VENOUS OBSTRUCTION IN A CASE OF CHRONIC PORTAL VEIN THROMBOSIS DUE TO COMBINED DEFICIENCY OF PROTEIN C AND PROTEIN S
DISCUSSION
Protein C and protein S are both vitamin K
dependent plasma protein synthesized in liver. Protein S acts as a co-factor to activated Protein C while inhibiting coagulation factors Va and VIIIa.
MANAGEMENT
PROTOCOL
Non selective beta-blocker Endoscopic variceal ligation Low molecular weight heparin and
Summary:
13 yr old girl presented with left upper abdominal dragging pain with early satiety
for six months and history of one episode of hematemesis. Past , personal and family history were non-contributory. On examination, moderate pallor present, other parameters of general examinations were within normal limits. On systemic examinatio, massive splenomegaly was present. Other systemic examinations were within normal limits.
Contd
Investigations revealed low Hemoglobin. USG abdomen revealed huge splenomegaly .
Esophago-gastro-duodenoscopy showed grade III esophageal varices. Spleno-portal Doppler study revealed enlarged splenic vein and thrombus in right branch of portal vein. CT abdomen showed portal cavernoma formation with increased spleenic collaterals. Coagulation profile revealed decreased protein C and protein S activity.