Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

28 July 2024: Articles  USA

Acute Aortic Dissection Presenting as Rectal Tenesmus

Unusual clinical course, Challenging differential diagnosis, Management of emergency care, Educational Purpose (only if useful for a systematic review or synthesis)

Adam D. Fratczak1EF, Jeffrey A. Nielson ORCID logo23EF*, Roy L. Johnson23EF

DOI: 10.12659/AJCR.943991

Am J Case Rep 2024; 25:e943991

0 Comments

Abstract

0:00

BACKGROUND: Acute aortic dissection (AAD) is a life-threatening medical emergency that requires a high index of clinical suspicion to be diagnosed promptly. The variability in the clinical presentation of AAD has historically made it difficult to identify in the acute setting. There remains significant inter-physician variability in the use of imaging. The median time to diagnosis in the Emergency Department is over 4 h and AAD has a mortality rate of 68% when diagnosis is delayed by over 48 h after onset of symptoms.

CASE REPORT: We discuss a case of a 69-year-old woman presenting with gastrointestinal symptoms in the Emergency Department who ultimately was found to have AAD. The patient had delayed presentation by 12 h due to misattribution of her rectal tenesmus to irritable bowel syndrome. However, after a thorough history and physical exam, the Emergency Medicine physician appropriately risk-stratified the patient and correctly diagnosed her with a Stanford Type A aortic dissection using a computed tomography study of the chest, abdomen, and pelvis with intravenous contrast.

CONCLUSIONS: AAD is an uncommon disease often requiring emergency intervention. We summarize the research and scoring systems and discuss the physical exam findings, comorbidities, imaging modalities, and risk stratification tools. Although imperfect, the Aortic Dissection Detection Risk Score with the addition of a D-dimer test is currently the best-validated tool and should be an important part of clinical decision making prior to performing computed tomography imaging.

Keywords: Abdominal Pain, Aneurysm, Dissecting, Diagnosis, Emergency Medicine

Introduction

Acute aortic dissection (AAD) results from a tear within the wall of the aorta and disruption of flow. This can be caused by abnormal blood flow, hemodynamic stressors, or genetic disorders [1,2]. Management is based on the Stanford classification into either Type A (TAAD) or Type B (TBAD). TAAD involves the ascending aorta, whereas TBAD occurs distal to the left subclavian artery.

Acute aortic dissection (AAD) is a rare but potentially fatal disease. The incidence rate ranges from 3.5 to 30 cases per 1 000 000 population per year [1,2], and mortality ranges from 13% to 27% [1,3], with rates increasing by 1–2% for every hour during which the disease is left untreated [4]. Unfortunately, AAD can be difficult to diagnose as it can have various presenting symptoms that often mimic other disease pathologies, including acute coronary syndrome, syncope, and intra-abdominal diseases. The median time between arrival in the Emergency Department and identification of AAD is 4.3 h. Untreated AAD has a reported cumulative mortality rate of 68% at 48 h after symptom onset [5], and misdiagnosis of the disease can lead to serious complications, including pericardial effusion, hemorrhagic shock, and death [6].

Case Report

A 69-year-old woman with a past medical history of anxiety, depression, fibromyalgia, irritable bowel syndrome, hypertension, hyperlipidemia, and COPD presented to the ED with a chief concern of abdominal pain and back pain. Prior to arrival, she had rectal tenesmus, an abrupt onset of “gas pains” along with abdominal distention, which were symptoms she would typically experience while constipated. Given her history of irritable bowel syndrome (IBS), she initially thought that she was experiencing symptoms related to her IBS and self-administered an enema, but she had no output and experienced poor relief of her symptoms. Her gastrointestinal symptoms persisted for 12 h. When the symptoms evolved and she began experiencing abdominal pain radiating to her back, she came the Emergency Department.

In the Emergency Department, her blood pressure was 170/68, but other vital signs were within normal limits. Pertinent risk factors included hypertension, chronic obstructive pulmonary disease (COPD), and first-degree relatives with aortic and brain aneurysms. She also noted that her pain began wrapping around her abdomen to her back and radiating up into her chest. Physical exam revealed only diffuse abdominal tenderness with no pulse discrepancies. Using the ADD-RS (Aortic Dissection Detection Risk Score), the patient was considered high risk and underwent computed tomography (CT) of the chest, abdomen, and pelvis with intravenous contrast. The imaging study revealed a Stanford Type A aortic dissection extending from the aortic root to the aortic bifurcation (Figures 1, 2). She was started on a nicardipine infusion with a systolic pressure goal of less than 120 and an esmolol infusion with a heart rate goal of less than 60. Cardiothoracic Surgery was consulted and she was taken to the operating room for emergent intervention. She spent 8 days in the hospital, during which she made a complete recovery and then was discharged.

Discussion

AAD is a severe, life-threatening diagnosis that remains challenging to identify and manage. Although the disease is rare, research has improved the process of diagnosis, management, and treatment. With the increased use of CT and recent advances in endovascular procedures, in-house mortality of TAAD decreased from 31% to 22% from 1995 to 2013 [7]. Registries such as the International Registry of Acute Aortic Dissections (IRAD) are the main sources for research on AAD. IRAD is a database comprising 58 international centers in 13 countries, which collect prospective data on the presentation, management, and outcomes of patients with aortic dissection. As of 2018, IRAD had collected data from more than 7300 individuals with aortic dissection [4]. We discuss IRAD’s conclusions together with the imaging modalities and the risk stratification tools that can be utilized.

The presentation of AAD can be highly variable and depends on the location, sex, and age of the patient [4], often misleading clinicians to an alternative, incorrect diagnosis. A small retrospective study of 66 patients who presented to a tertiary care center and were later diagnosed with AAD found that 39% of these patients were initially misdiagnosed, most commonly with acute coronary syndrome. This had severe implications, as they were given antithrombotic medications, leading to an increased number and severity of complications, including hemorrhagic pericardial effusion, hemorrhagic pleural effusions, hemodynamic instability, and ultimately, a significant increase of in-hospital mortality (27% in-house mortality rate for the misdiagnosed group vs 13% for the correctly diagnosed group) [6].

AAD, as classically taught, presents as a sudden, severe, ripping, or tearing chest pain/abdominal pain that radiates to the back. However, atypical presentations are common, which likely explains the frequency of misdiagnosis. IRAD showed that the most common presenting symptoms of AAD are chest pain (72.7%), followed by back pain (53.2%), abdominal pain (29.6%), and syncope (9.4%). The pain was most often described as “severe” (90.6%), “abrupt in onset” (84.8%), “sharp” (64.4%), “ripping or tearing” (50.6%), or “radiating” (28.3%) [8], with 6.3% of patients reporting no pain whatsoever. Syncopal episodes were associated with approximately 13% of cases and carried a higher mortality rate, as they typically indicated progression to cardiac tamponade or stroke [4]. We found no references to tenesmus in AAD in our literature search. We believe tenesmus is likely secondary to mesenteric malperfusion – an obvious part of the AAD pathology, although clearly a less common symptom.

Physical exam findings also varied in AAD. Most patients had hypertension (49.0%), many were normotensive (34.6%), and hypotension was noted in 16.4%. Other physical exam findings correlated poorly with AAD, although aortic insufficiency murmur was the most common (31.6%). Interestingly, a pulse deficit was only present in 15.1% of patients, and congestive heart failure was present in 6.6% of cases [8]. Overall, this highlights the poor diagnostic utility of physical exam findings.

A nationwide Danish 20-year cohort study evaluated the clinical characteristics of 2719 AAD patients to identify risk factors. Hypertension was the most common comorbidity in both TAAD and TBAD, with a prevalence of 55.2% and 64.1%, respectively. The other 2 common comorbidities for TAAD were thoracic aortic aneurysm (14.6%) and COPD (13.1%). For TBAD, they were aortic aneurysm (7.5–12%) and COPD (15.7%). Less common comorbidities for TAAD and TBAD included history of stroke (5.6%, 4.6%), atrial fibrillation (5.3%, 3.9%), acute myocardial infarct (4.6%, 3.4%), heart failure (2.5%, 2.5%), diabetes mellitus (2.3%, 3.4%), chronic kidney disease (1.4%, 2.8%), peripheral vascular disease (1.0%, 2.1%), and Marfan syndrome (0.9%, 0.5%). TAAD patients had a mean age of 63.5±12.9, whereas TBAD patients had a mean age of 67.5±12.2. Men accounted for 67.5% of TAAD cases and 67.0% of TBAD cases, whereas women accounted for 32.5% of TAAD cases and 33% of TBAD cases. Incidence was higher for men for both TAAD (2.9/100 000 vs 1.6/100 000) and TBAD (1.9/100 000 vs 1.1/100 000) [3].

The risk factors identified in the Danish cohort were similar to those in IRAD. Additional risk factors identified in the IRAD database included a history of atherosclerotic disease (27%), previous cardiac surgery (16%), and cocaine use (1.8%). Furthermore, patients <40 years of age that presented with AAD were more likely to have Marfan syndrome or bicuspid aortic valve (59%) as their prevailing risk factors.

Additionally, our literature review demonstrated that there is a lack of standardized protocol for the diagnosis of AAD. EKG, laboratory test results, and chest X-ray results are typical for chest pain patients presenting in the ED. Abnormal EKG findings are often nonspecific and may delay a proper diagnosis. Biomarkers are of limited use in diagnosing or excluding AAD. Utilizing a D-dimer with a cutoff level of 500 ng/mL has been used to rule out AAD, with a sensitivity ranging from 91% to 100%. However, there are conditions causing false-negative D-dimer results, such as age, onset of symptoms, thrombosed false lumens, and chronicity of disease [1]. Chest X-rays can be helpful in diagnosing TAD but can provide false reassurance when normal. Widening of the mediastinum or aortic knob is seen in only 40–60% of patients with AAD [3] because dissections can occur with a non-dilated aorta [7]. According to IRAD, only 60% of patients had a maximum aortic diameter greater than 5.5 cm, with 40% having a diameter less than 5.0 cm [4].

Although clinical suspicion is important, imaging studies play a crucial role in diagnosis of AAD. IRAD data showed that the initial diagnostic modality was CT (69%) followed by echocardiography (25%), magnetic resonance imaging (MRI) (4%), and aortography (2–3%) [4]. A meta-analysis of 16 studies involving a total of 1139 patients with AAD sought to identify the best imaging modality specifically for the diagnosis of TAD. The review compared the sensitivities, specificities, and positive and negative likelihood ratios of transesophageal echocardiography, CT, and MRI. All 3 modalities provided “clinically equally reliable diagnostic for confirming or ruling out thoracic aortic dissection.” However, it highlighted a key finding regarding the use of CT. Although conventional CT appears to be the most common modality for the identification of TAD, it has relatively poor sensitivity (83–94%) and specificity (87–100%). The newer, helical CT, was identified as the best imaging modality for low-risk patients (pre-test probability <5%) as it had a sensitivity of 100% and a specificity of 98%. With increased availability, improved sensitivity, and shorter scanning times for completion of the study, helical CT appears to be the imaging modality of choice for TAD and thus AAD when available [5].

Analysis of the IRAD database led to the creation of the Aortic Dissection Detection Risk Score (ADD-RS). In 2010, the American Heart Association, in conjunction with the American College of Cardiology, released a set of guidelines for risk-stratifying patients with potential AAD into low-risk (0 points), intermediate-risk (1 point), and high-risk categories (2–3 points). The system incorporates 12 factors divided into 3 categories: high-risk conditions, high-risk pain features, and high-risk exam features, with 1 point designated to each category [2]. This guideline was retrospectively validated the following year. In the case presented above, the patient was in the high-risk category based on her past medical history and clinical presentation.

In 2018, the ADvISED study, which was a multicenter prospective observational trial, incorporated the use of a D-dimer in conjunction with the ADD-RS in the hopes of improving diagnostic accuracy in the detection of AAD. The study discovered that an ADD-RS=0 with a negative D-dimer had a sensitivity of 99.6% and a negative predictive value of 99.7%. Additionally, an ADD-RS≤1 with a negative D-dimer had a sensitivity of 98.8% with a negative predictive value of 99.7% [9]. Through the analysis of the IRAD database, the ADvISED trial has illustrated that obtaining a thorough history, physical examination, and use of a D-dimer can be a very useful for ruling out AAD in clinical practice.

Conclusions

AAD is an extraordinarily fatal disease that can mimic many other pathologies in its presentation as demonstrated by this case of AAD with tenesmus. We examined common and uncommon risk factors, presenting symptoms, and physical exam findings associated with AAD. We suggest that clinicians implement the ADD-RS tool with a D-dimer test to improve diagnostic accuracy in patients presenting with atypical symptoms of AAD.

References:

1.. Diercks DB, Promes SB, Schuur JD, Clinical policy: Critical issues in the evaluation and management of adult patients with suspected acute nontraumatic thoracic aortic dissection [published correction appears in Ann Emerg Med. 2017;70(5):758]: Ann Emerg Med, 2015; 65(1); 32-42.e12

2.. Isselbacher EM, Preventza O, Hamilton Black J, 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines: Circulation, 2022; 146(24); e334-482

3.. Obel LM, Lindholt JS, Lasota AN, Clinical characteristics, incidences, and mortality rates for type A and B aortic dissections: A nationwide Danish population-based cohort study from 1996 to 2016: Circulation, 2022; 146(25); 1903-17

4.. Evangelista A, Isselbacher EM, Bossone E, Insights from the International Registry of Acute Aortic Dissection: A 20-year experience of collaborative clinical research: Circulation, 2018; 137(17); 1846-60

5.. Shiga T, Wajima Z, Apfel CC, Diagnostic accuracy of transesophageal echocardiography, helical computed tomography, and magnetic resonance imaging for suspected thoracic aortic dissection: Systematic review and meta-analysis. 2006: Database of abstracts of reviews of effects (DARE): Quality-assessed reviews [Internet], 1995, York (UK), Centre for Reviews and Dissemination (UK) Available from: https://www.ncbi.nlm.nih.gov/books/NBK72807/

6.. Hansen MS, Nogareda GJ, Hutchison SJ, Frequency of and inappropriate treatment of misdiagnosis of acute aortic dissection: Am J Cardiol, 2007; 99(6); 852-56

7.. Pape LA, Awais M, Woznicki EM, Presentation, diagnosis, and outcomes of acute aortic dissection: 17-year trends from the international registry of acute aortic dissection: J Am Coll Cardiol, 2015; 66(4); 350-58

8.. Hagan PG, Nienaber CA, Isselbacher EM, The International Registry of Acute Aortic Dissection (IRAD): New insights into an old disease: JAMA, 2000; 283(7); 897-903

9.. Nazerian P, Mueller C, Soeiro A de M, Diagnostic accuracy of the aortic dissection detection risk score plus D-dimer for acute aortic syndromes: The ADvISED prospective multicenter study: Circulation, 2018; 137(3); 250-58

In Press

Case report  China

Pathological Complete Response with Neoadjuvant Trastuzumab, Pertuzumab, and Chemotherapy Followed by Modif...

Am J Case Rep In Press; DOI: 10.12659/AJCR.943936  

Case report  USA

Uncommon Presentation of Hypersplenism in Adult Sickle Cell Disease Patients: A Rare Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.944693  

Case report  USA

A Rare Case of Idiopathic Reversible Cerebral Vasoconstriction Syndrome

Am J Case Rep In Press; DOI: 10.12659/AJCR.944273  

Case report  Poland

Coexisting Sacroiliac Arthritis and Chronic Nonbacterial Osteomyelitis in an Adolescent with Ehlers-Danlos ...

Am J Case Rep In Press; DOI: 10.12659/AJCR.943579  

Most Viewed Current Articles

07 Mar 2024 : Case report  USA 41,772

Neurocysticercosis Presenting as Migraine in the United States

DOI :10.12659/AJCR.943133

Am J Case Rep 2024; 25:e943133

0:00

10 Jan 2022 : Case report  Germany 32,260

A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...

DOI :10.12659/AJCR.935263

Am J Case Rep 2022; 23:e935263

21 Jun 2024 : Case report  China (mainland) 47,701

Intracranial Parasitic Fetus in a Living Infant: A Case Study with Surgical Intervention and Prognosis Anal...

DOI :10.12659/AJCR.944371

Am J Case Rep 2024; 25:e944371

0:00

23 Feb 2022 : Case report  USA 19,468

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923