Right paraduodenal hernia accompanying superior mesenteric vein thrombosis: a rare case
- Nail Omarov ,
- İbrahim Halil Özata and
- Emre Balık
- General Surgery Department, Koç University Hospital, Istanbul, Turkey
- Correspondence to Dr Nail Omarov; nomarov@kuh.ku.edu.tr
Abstract
A 59-year-old man with abdominal pain was admitted to the emergency department. Investigations had revealed a right-sided paraduodenal hernia and superior mesenteric vein (SMV) twisting around the superior mesenteric artery in rotation, the ‘whirlpool sign’. Owing to the increasing severity of abdominal pain and the presence of SMV thrombosis complicated with strangulated paraduodenal herniation associated with high mortality rates, diagnostic laparoscopy was performed. Resection of the intestines was not needed and paraduodenal hernia was repaired. The patient was uneventfully discharged.
Background
Acute mesenteric venous thrombosis (MVT) is a rare, potentially fatal form of mesenteric ischaemia with a 20%–30% mortality rate.1 It accounts for 5%–15% of all mesenteric ischaemia cases. It was first described in the literature by Elliot2 in 1895 as ‘thrombosis of the portomesenteric venous system’. However, in 1935, Warren and Eberhard3 described MVT as a distinct clinical entity. Diagnosis is usually made based on contrast-enhanced CT.4 As in all thrombotic cases, the aetiology of superior mesenteric vein (SMV) thrombosis may be primary (idiopathic) or secondary. It is caused by thrombophilia, trauma or local inflammation in 90% of cases; however, pancreatitis and diverticulitis may be the underlying cause of SMV thrombosis.5 Patients with MVT may present with a wide range of symptoms from an asymptomatic state to life-threatening complications.6
Paraduodenal hernias are the most common internal abdominal herniation type, accounting for 50% of all internal hernia cases.7 Internal abdominal herniation is an uncommon cause of obstruction of the small intestine. Left-sided paraduodenal hernias are three times more likely to occur than right-sided ones.8 Prompt diagnoses and treatment of intestinal obstruction can be life-saving approaches, regardless of the underlying cause.
It is rare for a patient who presents with abdominal pain to be suffering from a right paraduodenal hernia accompanied by SMV thrombosis.
Case presentation
A 59-year-old man with abdominal pain was admitted to our emergency department for the past 4 days. Physical examination revealed a blood pressure of 110/70 mm Hg, a pulse rate of 90 beats/min, a respiratory rate of 18 breaths/min, a body temperature of 36.8°C and oxygen saturation on room air of 96%. His consciousness was clear and body mass index was 27.7 kg/m2. There was pain in the upper abdominal quadrant on deep palpation, but no defence or rebound was observed. He had severe abdominal distension. The laboratory test results were as follows: haemoglobin 14.4 g/dL, white cell count 10.07×109/L, C reactive protein 148 mg/L. Liver and kidney function test results were normal. On blood gas analysis, the pH value was 7.3 and the lactate level was 0.8 mmol/L. The patient’s medical history revealed no nausea or vomiting, no known comorbidity, previous surgery or concomitant medication use. There was a discharge of gas without discharge of faeces for the past 2 days. He had a history of spontaneous deep vein thrombosis 20 years ago, and he had an uneventful follow-up course; however, he was lost to follow-up for medical care.
Differential diagnosis
He underwent a plain abdominal X-ray at the time of admission due to persistent and increasing abdominal pain. Radiographs showed a large amount of gas and air–fluid, compatible with the signs of ileus. Oral and intravenous contrast-enhanced CT of the abdomen revealed findings compatible with a right-sided paraduodenal hernia and SMV twisting around the superior mesenteric artery (SMA) in rotation, the ‘whirlpool sign’. We observed herniation of the jejunal loops into this area, increasing the wall thickness of an about 15 cm loop (figure 1). This loop’s wall thickness was found to increase up to 10 mm with an appearance of SMV thrombosis extending until the confluence with the portal vein (figure 2). The free intraperitoneal fluid was noted without perforation signs. Once the pain became unbearable, a nasogastric tube was inserted for decompression, and a Foley catheter was placed for urinary drainage. Treatment with low-molecular-weight heparin (LMWH) 0.6 mL two times per day and a broad-spectrum antibiotic (piperacillin/tazobactam 4.5 g three times per day) was initiated.
Contrast-enhanced CT of the abdomen showing findings compatible with a right-sided paraduodenal hernia and superior mesenteric vein twisting around superior mesenteric artery in rotation (whirlpool sign).
Contrast-enhanced CT of the abdomen showing increased wall thickness of the intestinal loop with an appearance of superior mesenteric vein thrombosis extending until the confluence with the portal vein.
Treatment
Owing to the increasing severity of abdominal pain and the presence of SMV thrombosis complicated with strangulated paraduodenal herniation associated with high mortality rates, diagnostic laparoscopy was performed. Written informed consent was obtained from the patient. During the operation, severely dilated small intestinal loops along with multiple adhesions were visualised. Adhesions may have developed due to asymptomatic inflammatory bowel disease. As the intestine was fragile and enlarged with a high bleeding pattern, total exploration was avoided. A lower midline incision (ie, below the umbilicus) was performed. Adhesions were resolved by making adhesiolysis. The small intestines herniating into the paraduodenal recess were mobilised. Resection was abandoned due to reduced ischemia following the loops’ compression with decreased blood flow using warm physiological saline (figure 3). Paraduodenal hernia was repaired. Postoperatively, the patient was followed by nasogastric tube decompression, LMWH and antibiotherapy. After haematological testing, thrombophilia panel, mutation analysis, leucocyte formula analysis and genetic testing were performed. According to the leucocyte formula analysis, a heterozygous factor V Leiden mutation was detected. In this case, acute MVT may have developed due to paraduodenal hernia pressure on chronic MVT due to factor V Leiden deficiency. The fluorescence in situ hybridisation revealed no reciprocal translocation of chromosomes 9 and 22 and no JACK2 V617F mutation.
An intraoperative view of exploratory laparotomy. The small intestines herniating into the paraduodenal recess were mobilised.
Outcome and follow-up
The patient was uneventfully discharged on day 4 of the operation.
Discussion
MVT affects two or three in every 100 000 individuals annually.9 Acute portomesenteric venous thrombosis facilitates the formation of varices by increasing the portal vein pressure and results in variceal bleeding, which is the initial sign of MVT in the majority of cases. If thrombosis originates from the SMV alone or along with the portomesenteric vein, infarction typically involves the small intestines.10 The clinical presentation may vary depending on the location, progression rate and degree of extension of thrombosis. There is no intestinal necrosis in nearly half of the portomesenteric thrombosis cases. Also, some patients with MVT may be asymptomatic. Extensive acute MVT may lead to massive intestinal fluid accumulation, hypovolemia and hemoconcentration.11 It also induces vasoconstriction of the intestinal artery by increasing the portal pressure, resulting in impaired intestinal perfusion and haemorrhagic intestinal infarction. The breach of the intestinal wall integrity may lead to translocation of the intestinal microorganisms and, eventually, multiple organ failure.10 11
A thorough medical history is a basis for diagnosis. Early recognition of MVT can be challenging due to non-specific physical examination and laboratory test findings. Clinical suspicion is needed for prompt diagnosis. Abdominal pain, which becomes increasingly severe, is the most common manifestation of MVT. Time from MVT development to symptom onset may vary between 24 and 72 hours, and patients with subacute MVT may experience symptoms over days to weeks.12 13 The other symptoms include nausea/vomiting, hematochezia, the lack of gas and stool discharge and diarrhoea. On physical examination, abdominal tenderness is seen in 80%, peritonitis findings in 10% and blood in rectal examination in 23% of the cases.13 14 The typical laboratory test findings include leukocytosis, metabolic acidosis and elevated lactate and D-dimer levels.15 Despite sophisticated diagnostic modalities currently, delayed diagnosis is the main reason for high mortality rates of 15%–40%.16 17 This case was admitted to our emergency department with progressive abdominal pain for the past 4 days. Physical examination did not reveal an acute abdomen. He had only nausea and the inability to defecate. Laboratory testing showed no metabolic acidosis, leukocytosis or lactate elevation.
Imaging modalities are of paramount importance for the diagnosis of MVT. The contrast-enhanced CT is the standard imaging modality for MVT’s delineation; however, CT angiography is the most sensitive method.18 Despite negative laboratory testing, MVT complicated by a right-sided paraduodenal hernia increases the suspicion of intestinal ischaemia. In case of a right-sided paraduodenal hernia, the herniated content is located in the right half of the transverse mesocolon and behind the ascending mesocolon.19 The SMA and middle colic artery lie adjacent to the neck of the hernia sac. In such cases, herniated intestinal loops apply pressure on neighbouring arteries, and the blood flow is disrupted, thereby leading to ischaemia of these intestinal loops. The hernia sac is typically located on the right side of the midline and presents with rotation abnormalities. The small intestines and, rarely, large intestines are entrapped in the sac.20 21 If left undiagnosed or untreated, mortality is high. The main treatment options include anticoagulation, hydration, antibiotherapy, thrombolytic therapy, surgical thrombectomy and intestinal resection. Heparinisation is the first-line treatment of choice, and early heparinisation has been shown to slow disease progression and reduce recurrence significantly.22
In a case report, Peru et al 23 performed an emergency laparotomy in a patient with a suspected right paraduodenal hernia and found that the right paraduodenal hernia was located behind the right mesocolon and duodenum with the first jejunal loop situated in the right, indicating midgut malrotation. The CT showed acute proximal obstruction of the small bowel without any ischaemia. At the small bowel’s standard anatomical position with the release of the common mesentery, herniation was primarily repaired. In another report, Cui and Kirkby24 described a patient diagnosed with SMV thrombus extending into the portal vein as evidenced by CT. The patient was closely followed in the intensive care unit with conservative treatment using anticoagulants and discharged uneventfully on a postoperative day 17. No peritonitis findings were observed during regular follow-up. Also, Kim et al 15 retrospectively analysed 66 patients with acute SMV thrombosis. Of the patients, 15 (23%) underwent bowel resection. The authors concluded that the extent of thrombus and aetiology were correlated with the severity of acute SMV thrombosis. Consistent with the literature, we applied heparinisation with LMWH to our case due to MVT accompanied by paraduodenal herniation, and we performed emergency diagnostic laparoscopy. We avoided intestinal resection due to the lack of total ischaemia or perforation, despite decreased blood flow to the intestines. The main goal of surgical treatment is to protect the intestines from infarction or limit the infarction area. Emergency exploratory surgery should be performed in all patients suspected to have localised or diffuse peritonitis.
Patient’s perspective
I suffered from severe abdominal pain for a few days before I visited the emergency room. I always thought that this pain was temporary and insignificant. However, I learnt from my doctors that the diagnosis was more complicated than I thought. I am very thankful to my surgeons, who saved my life and relieved my unbearable pain.
Learning points
This is a rare case. It is not often that a patient presenting with abdominal pain is diagnosed with a right paraduodenal hernia accompanying a superior mesenteric vein thrombosis.
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The presence of superior mesenteric vein thrombosis with a right paraduodenal hernia increases the possibility of intestinal necrosis.
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We may have to resect the necrotic intestines.
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We have to be careful in this situation, because if we intervene late it can be fatal.
Footnotes
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Contributors NO: conception of the case report and acquisition of patient’s data. NO and İHÖ: literature search. NO and İHÖ: wrote the first draft of the manuscript. EB: final approval of the version to be published. All authors read and approved the final manuscript.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
References
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