AN INTERESTING HEMATEMESIS

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

Resides in North 24 Parganas

CHIEF

COMPLAINTS

Left upper abdominal pain for last six

months with early satiety

One episode of blood-vomiting seven days

prior to admission

HISTORY ILLNESS

OF

PRESENT
abdomen started six

pain in left upper

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

No h/o previous hematemesis , jaundice. No history of blood transfusion or any

surgical operation including neonatal umbilical vein catheterization. No history suggestive of intra-abdominal infection.

PERSONAL

HISTORY

Non - contributory

FAMILY

HISTORY

No history of similar type of illness in any

of the family members.

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

Massive splenomegaly-20 cm along its axis

No free fluid No other organomegaly.

OTHER

SYSTEMS

CHEST-WNL CVS-WNL

CNS-WNL
LYMPHO-RETICULAR SYSTEM- No bony

tenderness. No lymphadenopathy. Massive splenomegaly present.

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

Hemoglobin electrophoresis ( by HPLC

method) WNL

ANA ( Hep-2)- negative at 1: 80 dilution

Contd
OESOPHAGO-GASTRO-DUODENOSCOPY: - Grade III oesophageal varices

Contd
SPLENO-PORTAL DOPPLER STUDY: - Massive splenomegaly - Enlarged splenic vein ( at confluence 8

mm, at hilum -7.2 mm )

- Thrombus in right branch of portal vein.

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.

Portal cavernoma formation

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

warfarin for 5 days

Warfarin - to maintain INR between 2 to 3.

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.