Journal of Surgical Case Reports, 2023, 12, 1–3

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Case Report

Case Report
Successful management of portal hypertension with
splenomegaly and cavernous transformation of the
portal vein: a rare case report
1,
Simrah Sharjeel *, Muhammad Abdullah2
1 Medicine, Jinnah Sindh Medical University, Karachi, Pakistan
2 General Surgery, Indus Hospital and Health Network, Karachi, Pakistan
*Corresponding author. Jinnah Sindh Medical University, Karachi, Pakistan. E-mail: [email protected]

Abstract
Portal hypertension, often stemming from liver cirrhosis or vascular anomalies, can result in cavernous transformation of the
portal vein, a rare condition associated with biliary obstruction, variceal hemorrhage, and splenomegaly. This case report details
a unique occurrence of portal hypertension, splenomegaly, and cavernous transformation of the portal vein successfully managed
through splenectomy and spleno-renal shunt. A 30-year-old female with a history of portal hypertension, portal gastropathy, and
splenomegaly presented with left upper quadrant abdominal pain. She had previously undergone esophageal variceal ligation and
required intermittent blood transfusions. Additional complications included easy bruising, heavy menstrual bleeding, and a prior
episode of hematemesis. Clinical assessment confirmed splenomegaly, while a CT scan confirmed the diagnosis. A tailored surgical
approach was chosen, leading to splenectomy and spleno-renal shunt.

Keywords: extra hepatic portal venous obstruction; esophageal varices; spleno-renal shunt; non-cirrhotic portal hypertension; cavernous
transformation; case report

Introduction in infancy, which may have been a risk factor for her current
diagnosis. Apart from this, her family history, drug history, and
Portal hypertension, a complex condition characterized by
psychosocial history was unremarkable.
increased blood pressure within the portal venous system,
On examination, she appeared pale and lethargic but was alert
often can result from liver cirrhosis, portal vein thrombosis,
and oriented. Abdominal examination revealed an enlarged and
or vascular abnormalities [1]. One main consequence of portal
tense spleen extending 19 cm beyond the costal margin, while
hypertension is the cavernous transformation of the portal vein,
the liver was non-tender and palpable. The percussion note was
a rare condition with an insidious presentation. It often manifests
tympanic, with no signs of f luid accumulation. Cardiovascular
as biliary obstruction, variceal bleeding, and splenomegaly [2].
and respiratory exams were unremarkable.
Herein, we report a rare case of portal hypertension, splenome
The CT scan exhibited features consistent with portal hyper-
galy, and cavernous transformation of the portal vein treated with
tension and associated changes in the pre-portal venous system,
a splenectomy and spleno-renal shunt. This case report has been
liver, and spleen (Figs 1 and 2).
reported in line with the SCARE Criteria [3].
The patient’s complete blood count indicated pancytopenia,
while liver function tests showed no significant abnormalities.
Upper gastrointestinal endoscopy showed the presence of small
Case presentation esophageal varices and severe portal hypertensive gastropathy
A 30-year-old female with a history of portal hypertension, por- (Fig. 3).
tal gastropathy, and splenomegaly presented to the outpatient Based on the patient’s clinical history, examination findings,
department with pain and a dragging sensation in the left upper and imaging studies, the working diagnosis of cavernous trans-
quadrant of her abdomen. She had previously undergone multi- formation of the portal vein (complicated by portal hypertension
ple sessions of endoscopic band ligation for esophageal varices with splenomegaly, portal gastropathy, and suspected cirrhosis)
and occasionally required transfusions for red blood cells and was decided upon. The patient was informed about her condition
platelets. The patient also reported easy bruising and heavy men- and consented to a high-risk surgical procedure involving
strual bleeding. She had a history of melena and a single episode splenectomy and spleno-renal shunt. She underwent pre-
of hematemesis in the past. Notably, she had umbilical sepsis splenectomy vaccination.

Received: September 24, 2023. Accepted: October 15, 2023

Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2023.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which
permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
2 | Sharjeel and Abdullah

Figure 1. CT scan coronal view showing gross hepatosplenomegaly with

large collaterals suggestive of portal venous hypertension with portal
biliopathy. Chronic thrombus seen in SMV and portal conf luence.

Figure 2. CT scan axial view.

Figure 4. Proximal side-to-side spleno-renal shunt.

uneventful recovery and was discharged on the third day after

surgery (Fig. 4).
Post-operative follow-ups were conducted two and four weeks
after surgery. She has remained well and stable. Her quality
of life has improved significantly, as has her functional class.
Hematological tests done reveal a reversal of counts to normal
levels. A repeated upper GI scope performed after a month showed
complete resolution of gastric varices. She has been taking meat
regularly, but there are no alarming signs of encephalopathy. She
has been kept on regular surveillance at 3-month intervals.
Figure 3. Upper GI scopes showing portal hypertension gastropathy.

Discussion
The surgical procedure involved a Chevron incision. During the Extra Hepatic Portal Vein Obstruction (EHPVO) is a rare condi-
operation, an enlarged and firm spleen was encountered. Multiple tion with an uncertain prevalence. In India, it affects 0.84–13.6
dilated and tortuous varices were found around the spleen involv- individuals per 100 000, while in Western countries, portal venous
ing the splenocolic, phrenicocolic, and gastrocolic ligaments. The thrombosis accounts for two to five percent of all variceal bleeds.
splenic vein was also dilated and tortuous. Splenic vein dissection EHPVO predominantly affects young adults, primarily between
was affected proximal to the splenic hilum to preserve maximum the ages of 10 and 30, and men are more commonly affected,
length for the subsequent shunt. Following removal of the spleen, with a male-to-female ratio of 2:1 [4]. Therefore, there is limited
an end-to-side, central (non-selective) spleno-renal shunt was knowledge about risk factors, natural progression, and the long-
performed with polydioxanone 5/0. term outcomes of surgical interventions.
The patient was monitored for vital signs, pain, and f luid Historically, EHPVO has been treated with surgical shunts,
balance during the immediate post-operative period. She had an the largest series of which was conducted by Orloff et al., who
 Successful management of portal hypertension | 3

conducted two randomized controlled trials on emergency Conflict of interest statement

portocaval shunts. Their findings indicated that this procedure
The authors declare no conf lict of interest.
effectively stopped variceal bleeding with almost no chance of
re-occlusion [5].
Predisposing factors for EHPVO include intra-abdominal Funding
infections, peritonitis, hypercoagulability, and portal venous
None declared.
stenosis or atresia. EHPVO and non-cirrhotic portal fibrosis
are the leading causes of non-cirrhotic portal hypertension
[6]. It is essential to consider our patient’s history of neonatal Data availability
omphalitis, which may have contributed to the development of
All data related to the content of this case report is present within
portal hypertension in this case. The infection spread in such
the body of the manuscript.
instances may lead to portal pyemia, resulting in irreversible
venous wall damage, either through thrombogenic predisposition,
fibrotic narrowing, or both [7]. Author contributions
Due to its vast range of clinical manifestations, early EHPVO
Simrah Sharjeel and Dr Muhammad Abdullah, both contributed
diagnosis is challenging. Common symptoms include abdomi-
significantly toward the study concept, data collection and inter-
nal pain, splenomegaly, recurrent variceal bleeding, and ascites.
pretation, and writing the paper.
Splenomegaly causing hypersplenism is an important finding
seen in patients with EHPVO. Even Though medical and endo-
scopic management is recommended for EHPVO, shunt surgery is Ethical approval
indicated in rare cases such as portal biliopathy, hypersplenism, Patient approval has been given. This study is exempt from ethical
massive splenomegaly affecting the quality of life, growth retar- approval in our institution.
dation, and rare blood group [4].
Our patient had been undergoing endoscopic band ligation
due to esophageal variceal bleeding. Although endoscopic man- Consent
agement has temporarily alleviated variceal bleeding, it is not Written informed consent was obtained from the patient to pub-
treating the underlying cause, making it unsuitable as a long-term lish this case report and accompanying images.
treatment. Despite potential hazards due to collaterals, direct
surgical procedures on the biliary system are often performed for
EHPVO, providing a one-time solution for young patients with long
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