Extra hepatic portal vein obstruction (EHPVO) is the commonest cause of portal hypertension in children. EHPVO along with thrombosis of splenic vein (SV) and superior mesenteric vein (SMV) is an uncommon condition causing extensive varices formation in the oesophagus, stomach and in other parts of gastrointestinal tract including rectal varix as well as splenomegaly and associated hypersplenism. Most commonly the child presents with hematmesis and due to extensive varices it is difficult to obliterate the varices using endoscopic therapy. Due to thrombosed SMV and SV shunt surgery is not possible. We describe here a case of EHPVO with SMV and SV thrombosis with bleeding gastric varix that underwent gastro- oesophageal devascularisation with splenectomy and oesophageal transection to prevent recurrent bleed from gastric varices.
Extra Hepatic Portal Vein Obstruction (EHPVO) with Extensive Mesenteric Venous Thrombosis in a Child
BOSIS IN A
EXTRA HEPATIC PORTAL VEIN OBSTRUCTION (EHPVO) WITH EXTENSIVE
Rakesh Rai*, ST Gopal*,Suresh Singhvi*, Radhakrishna Hedge# and Anand Alladi**
*Senior Consultant Surgeon Institute of Liver & Pancreatic Disorder and Solid Organ Transplantation,
#Senior Consultant, Department of Pediatric Medicine,**Senior Consultant, Department of Pediatric Surgery,
Correspondence to: Dr Suresh Singhvi, Senior Consultant (Surgery), Institute of Liver & Pancreatic Disorder and
Solid Organ Transplantation, Apollo Hospital, Bannerghatta Road, Bangalore 560 076, India.
Extra hepatic portal vein obstruction (EHPVO) is the commonest cause of portal hypertension in children.
EHPVO along with thrombosis of splenic vein (SV) and superior mesenteric vein (SMV) is an uncommon
condition causing extensive varices formation in the oesophagus, stomach and in other parts of
gastrointestinal tract including rectal varix as well as splenomegaly and associated hypersplenism. Most
commonly the child presents with hematmesis and due to extensive varices it is difficult to obliterate the
varices using endoscopic therapy. Due to thrombosed SMV and SV shunt surgery is not possible. We
describe here a case of EHPVO with SMV and SV thrombosis with bleeding gastric varix that underwent
gastro- oesophageal devascularisation with splenectomy and oesophageal transection to prevent
recurrent bleed from gastric varices.
Key words: Extra hepatic portal vein obstruction, Mesenteric venous thrombosis, Portal hypertension,
MESENTERIC VENOUS THROMBOSIS IN A CHILD
Bannerghatta Road, Apollo Hospital, Bangalore 560 076, India.
Extra hepatic portal venous obstruction (EHPVO) is the
commonest cause of portal hypertension in the developing
world accounting for 70% of pediatric patients with portal
hypertension and is second only to cirrhosis in the West. It is
also the most common cause of upper gastrointestinal
bleeding in children . Classical presentation in these
children is with painless hematmesis with splenomegaly
without hepatic decompensation . The predisposing
factors are thought to be – direct injury to vessels, rare
congenital portal vein anomalies, sepsis, dehydration,
multiple exchange transfusion and hypercoagulable state .
The management of these patients usually involves
variceal banding or sclerotherapy. Surgical options include
different types of porto-systemic shunts. In small
percentage of patients with extensive mesenteric venous
thrombosis with SMV and SV thrombosis only surgical
option is to do a gastro oesophageal devascularisation and
oesophageal transection to control active bleeding or to
prevent recurrent hematmesis.
A 3-years old male child presented with history of three
episodes of hematmesis in the past. All three episodes
required hospital admission but were treated conservatively
in different hospitals. Parents also provided history of
umbilical sepsis at the age of 6 months.
The child underwent upper gastrointestinal (UGI)
endoscopy which showed grade III oesophageal varices
extending upto the middle third of oesophagus as well as
had extensive gastric varices involving cardia and fundus
(Fig 1). The patient underwent ultrasound (US) doppler as
Fig 1 Grade III oesophageal varices with extensive gastric
varices involving cardia and fundus.
Apollo Medicine, Vol. 7, No. 4, December 2010 310
well as computerized tomography (CT) which showed
presence of thrombosis in right and left portal vein branch
as well as thrombosis of main portal vein (MPV), superior
mesenteric vein (SMV) and splenic vein (SV) and
splenomegaly. Liver was normal on US and CT. Blood tests
of the patient showed normal liver function. Patient
underwent detailed thrombophilia study which revealed
protein C and protein S deficiency.
To prevent recurrent bleeding from gastric and
oesophageal varices different options were considered.
Sclerotherapy or banding was not possible in this case as
had extensive cardiac and fundal varices. In view of
thrombosed SMV and SV shunt surgery was not possible.
Hence, the patient underwent elective gastro-oesophageal
devasculari-sation through an abdominal incision including
lower oesophageal transection and splenectomy. During
surgery, a liver biopsy was also carried out. The patient
made an uncomplicated recovery and was discharged
home on 8th post operative day. The histology of liver was
Classical presentation of children with EHPVO is with
splenomegaly and repeated episodes of painless, massive
hematemesis. The etiology of EHPVO may not be obvious
in many cases but a detailed history to rule out causes like
severe dehydration and omphalitis must be taken. A
thrombophilia profile is also mandatory to rule out
hereditary or acquired thrombophilia. Our patient had
Protein C and protein S deficiency. Other Indian and
Western studies have shown that protein C deficiency is the
second most common cause of inherited thrombophilia in
patients with portal vein thrombosis (PVT) .
Amarapurkar, et al showed that protein C deficiency
was the commonest hereditary risk factor (26%) in a study
on 28 patients with mesenteric venous thrombosis .
Protein C was also the commonest risk factor (38%
patients) in a series of 16 patients with mesenteric venous
thrombosis reported by Harward, et al .
Children with EHPVO presenting with hematmesis are
usually treated with variceal banding and sclerotherapy.
Patients who fail endoscopic therapy are considered for
surgical intervention. Shunt surgery including newer shunt
procedures like Rex shunt ( mesentrico – left portal shunt)
can result in resolution of symptom in majority of patients
. Gastro-oesophageal devascularisation is usually
reserved for patients in whom emergency surgery is
required to control the bleeding. However 33 - 50% of
patients may have extensive thrombosis of portal and
splenic veins making them unsuitable for shunt surgery .
The unshuntable portal hypertension is a challenge to
treat. These patients require frequent hospital admissions
for gastro intestinal bleed and require massive blood
transfusion. Endoscopic therapy is usually not effective as
majority of patients bleed from large fundic varices
.The patient also had large fundal varices. Cyanoacrylate
glue has also been used to control fundic varices but in case
of extensive fundic varices it may not be effective and
recurrence rate of bleeding is high .
These patients are suitable for oesophageal-gastric
devasularisation. The Sugiura’s procedure has achieved
great success in the treatment of EHPVO in Japan as well as
outside . But this involves thoracotomy causing
significant morbidity. The modification of Sugiura’s
procedure has been described which involves abdominal
incision and oesophageal stapling through a gstrostomy
. In our case we carried out a similar procedure of
gastro-oesophageal devascularisation with splenectomy
and oesophageal transaction through abdominal incision.
In recent years quality of life (QOL) has become an
established endpoint of medical care in patients with
EHPVO . It has been observed that splenomegaly and
growth retardation are independent contributing factors
that adversely affect the QOL in children with EHPVO
. As gastro – oesophageal devascularisation with
splenectomy corrects problem with splenomegaly it might
improve the QOL in long term follow up.
EHPVO with SV and SMV is a challenging problem to
treat. These patients need detailed investigation to rule out
thrombophilia. Shunt surgeries are not possible in this
group of patients and bleeding from fundic varices is
difficult to manage with endoscopic therapy. To prevent
recurrent bleed from gastro-oesophageal varices elective
gastro – oesophageal devascularisation should be
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