We report a rare case of portal vein thrombosis (PVT) secondary to idiopathic hypercoagulability leading to non-cirrhotic portal hypertension and cavernous transformation. The patient had a history of acute PVT and superior mesenteric vein thrombosis, which was initially managed successfully with anticoagulation therapy. However, the discontinuation of treatment precipitated a transition to chronic PVT and subsequent cavernous transformation. This condition manifested clinically as esophageal and gastric varices, posing a significant bleeding risk. Attempts to mitigate portal hypertension through medical management and endoscopic interventions had limited success. The anatomical complexities presented an insurmountable challenge to transjugular intrahepatic portosystemic shunt (TIPS) placement, and thus alternative treatment strategies were considered. A splenectomy markedly improved the patient’s condition. Over a 2-year follow-up period, with the aid of direct oral anticoagulants (DOACs), the patient remained stable; further endoscopic procedures were not required, and the patient did not experience a recurrence of thromboembolic or hemorrhagic events. This case underscores the complexity of PVT management and highlights the need for individualized treatment approaches in the face of anatomical and therapeutic challenges.

non-cirrhotic portal hypertension portal vein thrombosis splenectomy direct oral anticoagulants

Chronic extrahepatic portal vein obstruction is a vascular liver disorder characterized by the blockage and cavernomatous transformation of the portal vein that can affect the intrahepatic portal vein, splenic vein, or superior mesenteric vein. Portal vein thrombosis is an uncommon condition in individuals without liver disease1, 2. The causes can be related to primary and secondary hypercoagulable states. Acute treatment involves anticoagulation and addressing the underlying hypercoagulability3. If portal vein thrombosis progresses to a chronic condition, complications of portal hypertension, including variceal bleeding, can arise. The management of these complications is similar to that of cirrhotic portal hypertension, including pharmacotherapy and endoscopic intervention3. However, endoscopic treatment has various limitations, particularly for gastric varices4. Transjugular intrahepatic portosystemic shunt (TIPS) is a suitable option, but anatomical variations can make it challenging5. We present a case of a patient with non-cirrhotic portal hypertension with chronic portal vein thrombosis treated with splenectomy and long-term anticoagulation, demonstrating the efficacy and safety of the treatment over a 2-year follow-up period.

Figure 1

Figure 2

Table 1

	Results	Reference range
ANA	0.54	S/Co < 0.8
Anti ds-DNA	9.58	< 25 IU/mL
C3	114.5	80-170 mg/dL
C4	32.3	15-45 mg/dL
Lupus anticoagulant screen	Negative	Negative
Lupus anticoagulant confirm	1.11	0.80-1.19 IU/mL
Anti beta 2 glycoprotein	2.9	< 7 IU/mL
Anti cardiolipin	0.72	< 12 MPL/mL
Protein C	82	70-140%
Protein	93	55-124%
Antithrombin III	79	83-128%
Platelet count before splenectomy	204	150-400 G/L
Platelet count after splenectomy	854

Table 2

Timeline	April 2019	May 2021	March 2022	August 2023
Diagnosis	Acute thromboembolism at portal and superior mesenteric veins	- Total chronic portal vein thrombosis with carvernous transformation - Partial chronic superior mesenteric vein - Severe easophageal and gastric varices	- Total chronic portal vein thrombosis with carvernous transformation - Partial chronic superior mesenteric vein - Easophageal and gastric varices at risk of rupture	- Total chronic portal vein thrombosis with carvernous transformation - Easophageal and gastric varices without rupture risk - Post-splenectomy
Treatment	Heparin and VKA	- Carvedilol, ISMN - Prophylatic ligation and slerotherapy via endoscopy - DOACs	- Splenectomy - Carvedilol - DOACs	- Carvedilol - DOACs
Notes	Cancelled checkup after 3 months follow-up	Refractory varices despite repetitive endoscopic intervention	- Early resolution of varices - No more endoscopic intervention	6-month to 1-year endoscopic follow-up

A 46-year-old female patient had a history of acute superior mesenteric and portal vein thrombosis successfully treated with medical therapy at age 44 (i.e., in 2019). During outpatient follow-up, the patient was prescribed long-term vitamin K antagonists (VKAs) for 3–6 months and was scheduled for a re-evaluation of the thrombotic condition and underlying hypercoagulability. However, after 3 months of treatment, the patient, having no symptomatic recurrence, chose to discontinue anticoagulation therapy and did not return for further follow-up until May 2021 when she was admitted to the hospital with epigastric pain. During this hospitalization, the patient underwent gastroduodenal endoscopy, revealing multiple gastric ulcers accompanied by grade III esophageal and gastric varices with red signs (Figure 1 A series). The gastric appearance suggested portal hypertension-related gastropathy, and the ulcers were considered the cause of the gastric pain. A subsequent contrast-enhanced computerized tomography (CT) scan identified chronic thrombosis and cavernomatous transformation of the portal vein extending to intrahepatic distal branches and other splanchnic veins, along with esophageal and gastric varices (Figure 2 A series). Partial thrombosis was noted in the superior mesenteric vein.

The treatment strategy was discussed during the first multidisciplinary consultation, which involved gastroenterologists, cardiologists, radiologists, interventionists, and surgeons. TIPS was deemed unfeasible because of the altered anatomy of the portal and hepatic veins. The medical board opted for the prophylaxis of portal hypertension-related bleeding, including medical management to reduce portal pressure and a combination of endoscopic variceal band ligation and sclerotherapy of gastric varices. The patient was subsequently treated with non-selective beta-blockers, isosorbide mononitrate (ISMN), and gastrointestinal endoscopic intervention. The maximum tolerated doses of carvedilol and ISMN were achieved. Furthermore, the patient received VKAs and was transitioned to DOACs after excluding antiphospholipid syndrome-associated hypercoagulability. The patient was screened for inherited thrombophilia and malignant causes; however, the tests yielded negative results, suggesting idiopathic etiology (Table 1 ).

After 3 months of follow-up, a repeat endoscopy revealed slightly improved esophageal varices but worsening gastric varices, prompting prophylactic intervention. Despite repeated ligation and sclerotherapy, two subsequent 3-month endoscopic assessments did not show adequate improvement. A second multidisciplinary consultation led to the decision for splenectomy after carefully assessing portal venous circulation via CT and preoperative liver function, which was within the normal range. The patient underwent endoscopic splenectomy with low-molecular-weight heparin (LMWH) bridging for anticoagulation. The procedure proceeded smoothly, without bleeding or thromboembolic complications. Postoperatively, the patient was switched to DOACs and discharged after 5 days. Portal pressure-lowering medications were continued. Postoperatively, gastrointestinal endoscopy and CT scans showed significant improvement in esophageal and gastric varices (Figure 1 B series, Figure 2 B series).

The patient was followed for 2 years and remained on medical treatment, including DOACs and carvedilol. Repeated 3-month endoscopic assessments showed sustained improvement and stability characterized by the significant regression of varices, and no high-risk features were observed. Remarkably, the gastric varices almost completely disappeared (Figure 2 C series). A follow-up CT scan after 2 years also indicated drastically improved gastric and esophageal varices. No changes in portal vein morphology were observed, but no mesenteric thrombosis was detected (Figure 2 C series). Notably, the patient did not experience any incidents of thromboembolism or hemorrhaging. The follow-up D-dimer test showed no elevation. The comprehensive diagnostic and treatment details are presented in Table 2.