A Case of EHPVO

Dr.Shobika,DrAnupa Thomas ,Dr.Bhanu Vikraman pillai ,DrRekha Hari  

Dr.C Jayakumar

AIMS KOCHI 

One year old developmentally normal and immunized female child presented with vomiting of 1 day followed black tarry stools,with blood clots 

As per outside health care facility  Hb dropped from 11 to 7 so PRBC was given 

.USG abdomen-multiple small bowel loops seen in right upper quadrant showing diffuse mural thickening and surrounded marked echogenic fat planes and thin rim of free fluid s/o bowel inflammatory process 

 Laparotomy done -intraluminal blood noted in ileum,squeezed into caecum.

she was treated with Iv prednisolone,iv cefuroxime,iv.metrogyl and other supportives.

After discharge from outside hospital  she was asymptomatic for 1 week after which she developed hematemesis and dark coloured stools with reduced oral Intake and admitted here for further management

Labs:CRP-9.95,

WBC-15,N-44,L-45,

Hb-7.8g/dl,PT/INR-19.2/14.7/1.33,APTT-26/30.5

USG abdomen- suspicious for extra hepatic portal venous obstruction with Portal cavernoma formation.

CECT abdomen done Features of EHPVO with multiple large para esophageal, perigastric,peri rectal and retroperitoneal varices,reformed portal collaterals.large retroperitoneal collateral draining into Superior mesenteric vein

 Attenuated splenic vein.

Splenomegaly(9cm).

Diagnosed as EHPVO.

The child was started on Inj.Taxim,Inj.Octreotide,Inj.Vit K,T.Beta blocker  and other supportives

After obtaining fitness the child was taken up for OGD Scopy

There were Grade 3 varices and 3 columns of Grade 2 varices noted in esophagus

Blood clots in stomach were noted in stomach,no gastric varices.Duodenum till D2 was normal.

Endoscopic sclerotherapy was done.

Hb dropped and PRBC was given.

She improved symptomatically with no post operative complications.she was discharged with the advise to regular follow up with hemodynamically stable vitals.

Extra hepatic portal venous obstruction 

Is a vascular disorder of liver which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein,splenic vein or superior mesenteric vein.

Inicidence: usually 10 – 14 years

Portal hypertension:

Pre hepatic – portal or splenic vein thrombosis

Intrahepatic- cirrhosis

Post hepatic- Budd Chiara syndrome

Symptoms:

Blood in vomit

Bloated abdomen

Swelling over legs and feet

Complications:

Ascites

GI bleeding

Hypersplenism

Kidney failure

Diagnosis:

CBC

USG

Endoscopy

Management  :

Endoscopic sclerotherapy

Medications:Beta blockers,vasoconstrictors

Transjugular intrahepatic porto systemic shunt (TIPS )

Distal splenic renal shunt (DSRS)

Take home message – EHPVO in infancy is quite rare .They should be a team work required for follow up and treatment and standardization of portal hypertension in pediatric age group.

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