Journal of Medical Evidence

CASE REPORT
Year
: 2021  |  Volume : 2  |  Issue : 2  |  Page : 182--184

Giant splenic artery aneurysm with extrahepatic portal vein obstruction – A rare entity: Case report with literature review


Nisanth Puliyath1, Nirjhar Raj2, Sumit Sanyal2, Debendra Kumar Tripathy3, Rohit Gupta4,  
1 Department of General Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Surgical Gastroenterology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
3 Department of Anesthesiology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
4 Department of Medical Gastroenterology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Correspondence Address:
Dr. Nirjhar Raj
Department of Surgical Gastroenterology, All India Institute of Medical Sciences, Level 3, Block C, Rishikesh, Uttarakhand
India




How to cite this article:
Puliyath N, Raj N, Sanyal S, Tripathy DK, Gupta R. Giant splenic artery aneurysm with extrahepatic portal vein obstruction – A rare entity: Case report with literature review.J Med Evid 2021;2:182-184


How to cite this URL:
Puliyath N, Raj N, Sanyal S, Tripathy DK, Gupta R. Giant splenic artery aneurysm with extrahepatic portal vein obstruction – A rare entity: Case report with literature review. J Med Evid [serial online] 2021 [cited 2021 Dec 8 ];2:182-184
Available from: http://www.journaljme.org/text.asp?2021/2/2/182/324978


Full Text



 Introduction



Splenic artery aneurysms (SAA) are rare clinical entities which account for 0.77% of all abdominal aneurysms, and 60% of all visceral aneurysms, thus making them the most common type of visceral artery aneurysm.[1] They are prone to rupture and life-threatening haemorrhage, especially in women of childbearing age and during pregnancy, as it is four times common in them owing to endocrinal changes.[2] Most of the reported SAA are small in size (≤2 cm) and involve middle and distal thirds of the splenic artery. Only a handful of cases of giant SAA (with a diameter >2.5 cm) have been reported so far.[2],[3],[4]

 Case Report



A 30-year-old female patient presented to our outpatient department with the chief complaints of epigastric pain, progressive jaundice and itching for 2 weeks associated with high-grade fever and chills for 2 days. On retrospective analysis, she was a diagnosed case of extrahepatic portal vein obstruction (EHPVO) 3 years back but had lost to follow-up.

At the time of presentation, she was 8-month primigravida. On examination, she was icteric and febrile with tachycardia. The abdomen was distended with uterine enlargement corresponding to her period of gestation.

Her investigations showed elevated total leucocytes (27,000/mm3) with neutrophilia (95%), anaemia (haemoglobin of 9.4 g/dl) and a decreased platelet count (37,000/mm3). Liver function test was found to be deranged in the form of elevated total bilirubin (17.7 mg/dl), conjugated bilirubin (7.7 mg/dl) and alkaline phosphatase (547 U/L). Other investigations were within their normal limits.

On ultrasound abdomen, the portal vein was not visualised and was replaced by multiple tortuous venous channels, which were suggestive of portal cavernoma and portal hypertension along with altered liver echotexture. Gall stones and small hepatic duct stones along with bi-lobar intrahepatic biliary radical dilatation were also noted. These observations were consistent with magnetic resonance cholangiopancreatography (MRCP) finding [Figure 1]. The spleen was enlarged (>18 cm), and the splenic vein was dilated (15 mm) with multiple peri-splenic and peripancreatic collaterals. Foetal well-being was assessed on ultrasonography (USG), which revealed a single live intrauterine foetus of 29 weeks ± 2 weeks.{Figure 1}

A diagnosis of obstructive jaundice with cholangitis in the background of EHPVO with portal biliopathy was made. Percutaneous transhepatic biliary drainage (PTBD) was done for biliary decompression along with antibiotics. She improved clinically. During the hospital stay, she developed a massive bout of haematemesis. An emergency upper gastrointestinal (GI) endoscopy revealed oozing fundal varix which was controlled by glue injection therapy. Meanwhile, foetal bradycardia developed followed by the intrauterine demise of the baby, confirmed by obstetric USG assessment. A tri-phasic contrast-enhanced computed tomography abdomen was done which was suggestive of splenic artery showing fusiform dilatation (3.9–5.5 cm) and tortuosity throughout its course [Figure 2].{Figure 2}

In view of portal biliopathy with fundal varices with SAA, the patient was taken up for lienorenal shunt with excision of splenic artery aneurysm. The intraoperative findings were a giant splenic artery aneurysm, of size 5–6 cm in diameter [Figure 3], along the entire course of the artery with perianeurysmal adhesions to the distal pancreas. The other findings were splenomegaly and collateral vessel formation at the peri-oesophageal, peri-gastric, peri-splenic and retroperitoneal areas. An attempt was made to establish a plane beneath the aneurysm that led to massive bleeding. Hence, the plan of shunt surgery was abandoned and proceeded with en masse excision of the distal pancreas, splenic artery aneurysm [Figure 4] with splenectomy and para-oesophagogastric devascularisation. Distal pancreatectomy was also carried out because of dense adhesions between the SAA and distal pancreas. The post-operative period was uneventful, and she was discharged on the post-operative day 12.{Figure 3}{Figure 4}

 Discussion



SAAs, commonly an incidental finding, have a reported occurrence of 0.01%–0.2%.[5] Although studies show an increased risk of SAAs in multipara which are commonly located at the distal third, in our case, the patient was a primigravida, with the involvement of the entire length of the artery. SAA associated with EHPVO is extremely rare, with only a few cases reported till date.[2],[3],[6],[7] A giant SAA associated with both EHPVO and portal biliopathy has not been reported so far in the literature.

The pathogenesis of EHPVO in SAA proposed in previous case reports is that focal SAA will be causing either splenic vein compression or compression over splenic vein–portal vein confluence that leads to thrombus formation, thus causing sinistral portal hypertension or full-blown picture of EHPVO.[3],[6],[7] Treatment options available for SAA include endovascular techniques such as transcatheter embolisation using coils and glue,[8] percutaneous thrombin injection[9] and laparoscopic surgery.[10] An open surgical approach is used mainly in an emergency setting like a ruptured aneurysm. The treatment modality in a case of sinistral portal hypertension with distal SAA is splenectomy with excision of the aneurysm, and in EHPVO with SAA is splenectomy and excision of the aneurysm with or without devascularisation or shunt surgery. In our case, the splenic artery was dilated throughout its course, with collaterals and portal biliopathy. Hence, it is safe to assume that our patient was a pre-existing case of EHPVO, who later would have developed an aneurysm during her pregnancy owing to the underlying endocrine factors.

Most cases reported in the literature so far had symptoms of GI bleed without any portal biliopathy as the presenting symptom and hence their management required splenectomy with aneurysectomy alone. What makes our case special is the unique picture of portal biliopathy and existence of dense collaterals in the porta, which demands an additional procedure of shunt surgery. Thus, the patient was planned for a shunt surgery, but ended up in en masse excision of the distal pancreas, splenic artery aneurysm with splenectomy and para-oesophagogastric devascularisation due to uncontrolled haemorrhage from the aneurysm.

Hence, based on our experience, a pre-operative endovascular intervention, such as embolisation, could have been done in this case. This would have reduced the chance of the torrential bleed that occurred during the surgery, thus avoiding the need for an en masse excision of the distal pancreas with SAA. The patient could be taken for definitive shunt surgery on a later date.

Based on our experience and literature review, we here propose a new management algorithm for SAA with EHPVO [Figure 5].{Figure 5}

 Conclusion



SAAs, although rare, can have fatal consequences including massive bleed associated with rupture. They are primarily managed surgically with aneurysectomy and splenectomy, however newer endovascular approaches are on the rise.

A case of giant SAA with a combination of EHPVO and portal biliopathy is a clinical rarity, and pre-operative endovascular management of splenic artery aneurysm followed by a surgical procedure is advised to minimise intraoperative events such as torrential bleeding.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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