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 Table of Contents  
Year : 2017  |  Volume : 2  |  Issue : 1  |  Page : 21-25

Missed diagnosis of acute stanford Type A aortic dissection presenting with abdominal pain in the setting of acute appendicitis

1 Department of Surgery, Wellstar Atlanta Medical Center, Atlanta, GA, USA
2 Wellstar Atlanta Medical Center, Atlanta, GA, USA

Date of Web Publication12-Dec-2017

Correspondence Address:
Jonathan Nwiloh
Department of Surgery, Wellstar Atlanta Medical Center, Atlanta, GA 30312
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njct.njct_3_17

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Acute Stanford Type A aortic dissection (ATAAD) has a high mortality rate, particularly with delayed diagnosis and treatment. A 30-year-old male Marfan patient presented to the emergency department with acute abdominal pain and underwent laparoscopic appendectomy for acute appendicitis about 15 h later. Postoperatively, the patient developed acute pulmonary edema and respiratory failure. Transthoracic echocardiogram revealed severe aortic insufficiency, 7.5 cm root aneurysm, severe left ventricular dysfunction, and probable ATAAD. Following computed tomography angiogram confirmation of ATAAD, he underwent emergency root and ascending aortic replacement with a composite mechanical valve graft 48 h after hospitalization. The patient had Escherichia coli pericarditis at time of sternotomy likely hematogenous from appendicitis and later also developed postoperative pneumonia. At 2 years follow-up, he was doing well without any evidence of prosthetic valve conduit infection. The simultaneous presentation of two unrelated pathologies with acute abdominal pain led to the initial missed diagnosis of ATAAD, which delayed treatment and increased risks for morbidity and mortality. Therefore, Marfan syndrome patients presenting with acute abdomen should be ruled out for acute aortic dissection despite any other obvious concomitant pathology, especially in younger patients.

Keywords: Acute abdomen, acute aortic dissection, Marfan syndrome

How to cite this article:
Nwiloh J, Chiegboka GT. Missed diagnosis of acute stanford Type A aortic dissection presenting with abdominal pain in the setting of acute appendicitis. Niger J Cardiovasc Thorac Surg 2017;2:21-5

How to cite this URL:
Nwiloh J, Chiegboka GT. Missed diagnosis of acute stanford Type A aortic dissection presenting with abdominal pain in the setting of acute appendicitis. Niger J Cardiovasc Thorac Surg [serial online] 2017 [cited 2020 Dec 1];2:21-5. Available from: http://www.nigjourcvtsurg.org/text.asp?2017/2/1/21/220489

  Introduction Top

Acute Stanford Type A aortic dissection (ATAAD) although relatively rare is the most common aortic emergency with an incidence of 2.9 in 100,000/year in the review by Mészáros et al.[1] It is a dreaded disease with preoperative mortality reportedly rising with each passing hour from onset of symptoms and up to 90% are estimated to die within 1 week without treatment.[2] Although patients typically show sudden onset of severe chest pain, the clinical presentation can be variable, and abdominal pain could sometimes be the first onset of symptoms.[3] Treatment is usually surgical to reduce mortality from complications.

  Case Report Top

A 30-year-old male presented to the emergency department (ED) with about 8 h acute onset of epigastric pain radiating to the back. Pain was described as moderate intensity, achy, sharp, constant, associated with nausea, vomiting, and aggravated by food. There was no fever, chills, or dysuria. He denied having similar symptoms in the past. The patient had 15 pack-years smoking history, occasionally used marijuana and cocaine and was a social drinker. Medical history was unremarkable, and family history was significant for atrial fibrillation in mother, with no history of Marfan syndrome. Physical examination revealed a young Caucasian male with Marfanoid features who was in obvious acute distress. Vital signs at presentation were blood pressure 146/60 mmHg, pulse 104/min, respiratory rate 24/min, temperature 97.3F, oxygen saturation 98% on room air, height 6 feet 8 inches, and weight 77.1 kg. Pertinent objective findings were epigastric and right lower quadrant tenderness on palpation, with no guarding or rebound tenderness. Laboratory results showed white blood cell (WBC) 14,000/uL hemoglobin 14.9 g/dL, hematocrit 44.6%, platelet 157,000/uL, potassium 3.9 mmol/L, glucose 124 mg/dl, blood urea nitrogen 20 mg/dL, creatinine 1.3 mg/dL, prothrombin time 13.3, international normalized ratio 1.17, partial thromboplastin time 29.2, and lactic acid 3.9 mmol/L. Electrocardiogram was nonspecific. Abdominal and pelvic computed tomography (CT) scan was reported by a radiologist as normal except for fluid-filled structure in the right lower abdominal quadrant most likely a prominent appendix with surrounding inflammatory changes consistent with probable early acute appendicitis. After evaluation by a general surgeon, the patient subsequently underwent laparoscopic appendectomy about 15 h after hospital admission. Subsequent histological examination confirmed diagnosis of acute appendicitis with marked neutrophilic infiltration involving the mucosa and muscular wall and mucosal necrosis near the distal end of the 9 cm appendix. However, in the postanesthesia recovery unit, the patient developed acute respiratory distress with chest X-ray showing fulminant acute pulmonary edema requiring intubation and ventilator support [Figure 1]. A transthoracic echocardiogram performed the following day in the Intensive Care Unit to investigate the cause of the acute pulmonary edema showed severe aortic insufficiency, 7.5 cm aortic root aneurysm with questionable intimal flap, and severe left ventricular dysfunction with an ejection fraction estimated at 20%–30%. Subsequent CT angiogram of the chest and abdomen confirmed root aneurysm with aortic dissection involving aortic arch, right brachiocephalic, and right common carotid arteries, with extension to the descending thoracic aorta and the proximal infrarenal aorta. The dissection flap extended minimally into the celiac artery, while the superior mesenteric and right renal arteries appeared to arise from the true lumen and the left renal artery from the false lumen [Figure 2], [Figure 3], [Figure 4]. The patient then underwent emergency aortic repair about 48 h after his hospitalization. At sternotomy, the patient was noted to have developed seropurulent pericardial effusion with fibrinopurulent exudate. A stat Gram stain performed showed many WBCs but no organisms. Arterial inflow was established through an 8 mm hemashield graft sewn to the right subclavian artery, with venous drainage through a two-stage right atrial cannula. On opening the ascending aorta, there was an intimal tear seen in the sinus of Valsalva just above the noncoronary cusp. The patient underwent a modified Bentall with a 29 mm St. Jude Valsalva valve conduit and reimplantation of the coronary arteries. The distal anastomosis was constructed during a brief period of profound hypothermic circulatory arrest at 24°C with bilateral antegrade cerebral perfusion. Sternal closure was delayed for 48 h due to the encountered mediastinal infection, which was then followed with continuous mediastinal irrigation with 1 L normal saline mixed with 3 cc betadine solution for 5 days. Postoperative course was complicated by atrial fibrillation and pneumonia, and the patient was eventually discharged home in stable condition after an extended subacute rehabilitation stay on day 40 of hospitalization and continues to do well 2 years postsurgery.
Figure 1: Chest X-ray postintubation.

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Figure 2: Computed tomography chest/abdomen sagittal view black arrows showing dissection extending from ascending to the infrarenal aorta.

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Figure 3: Computed tomography chest coronal view black arrows showing root aneurysm and dissection intimal flap in the ascending aorta and innominate artery.

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Figure 4: Computed tomography chest/abdomen three-dimensional reconstruction white arrows showing root aneurysm, dissecting flap in ascending aorta, innominate artery, and infrarenal aorta.

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  Discussion Top

ATAAD diagnosis generally requires emergency or urgent surgery to prevent life-threatening complications. While the classic presentation is usually sudden onset of severe chest and/or back pain, symptoms can sometimes be atypical and nonspecific leading to misdiagnosis in more than half of patients [1],[4] or delayed diagnosis of >24 h in almost 40% of patients [5] on presentation to the ED. One form of atypical presentation is isolated acute abdominal pain as initial symptom of acute thoracic aortic dissection. Although there have been several case reports of acute thoracic aortic dissection presenting as an acute abdomen,[6],[7],[8] it remains relatively rare as was reported by Upchurch et al.[3] with only 46 (4.6%) of 992 patients with acute thoracic aortic dissection presenting primarily with abdominal pain in a review of the International Registry of Acute Aortic Dissection from 1996 to 2001. Similarly, Slater and DeSanctis [9] found abdominal pain in only 6.3% (3/47) of patients with ATAAD in their series. To the best of our knowledge, there has been no prior publication in the English literature of ATAAD occurring concurrently with acute appendicitis. Our patient's presentation with epigastric pain, elevated WBC count, and the CT findings of inflamed appendix were all consistent with the initial ED diagnosis of acute appendicitis. Although the patient had Marfanoid features, there were no compelling reasons for the ED physician to suspect ATAAD. Due to the rare incidence of two causes of acute abdomen occurring simultaneously, the more life-threatening ATAAD was initially missed for several hours until the onset of acute pulmonary edema from wide open aortic insufficiency postoperatively after the appendectomy. A retrospective review of the initial ED abdominal CT scan, however, did show a dissecting flap in the abdominal aorta which was missed by the radiologist [Figure 5]. A chest X-ray which sometimes shows suggestive signs was not obtained in the ED although the CT abdomen scout film showed cardiomegaly with no other suspicious findings [Figure 6]. Although both pathologies presented concurrently, there was no causal relationship between them. Acute appendicitis is thought to usually occur from luminal obstruction by fecaliths or lymphoid tissue hypertrophy. The appendix has a rich blood supply and therefore not prone to ischemia unless there is severe compromise of the blood supply to the ileum and cecum. Although the CT scan showed both the superior mesenteric and right renal arteries arising from the compressed true lumen, there were no signs of mesenteric ischemia observed at laparoscopy. While the probability of both pathologies occurring simultaneously is extremely low, it is still possible especially in younger age groups. Khan and Nair [10] report an incidence of aortic dissection ranging from 5 to 30 per million people per year depending on the prevalence of risk factors in the study population. The incidence is obviously higher in Marfan syndrome, a known risk factor for acute dissection.[11] However, acute appendicitis has the highest incidence in persons aged 10–19 years with 23.3/10,000 population per year in the United States.[12] Marfan's patients life expectancy previously 2/3 that of unaffected individuals, with deaths mainly from cardiovascular causes in over 90%, has improved in recent decades.[13],[14] Given these statistics, this very rare simultaneous occurrence is therefore more likely in younger patients who have the highest incidence of acute appendicitis if they also have Marfan syndrome, a recognized risk for early acute aortic dissection. Both conditions are readily diagnosed by clinical findings and CT scans. Once ATAAD is diagnosed by CT scan and echocardiogram, cardiac catheterization is rarely required before surgery unless there are other specific indications.  Escherichia More Details coli purulent pericardial effusion found at sternotomy was probably a hematogenous spread from the appendicitis. The 30-h delay from onset of symptoms to appendectomy might be contributory to this rare appendicitis complication. Tan et al. reported two pediatric patients (12- and 13-year olds) over a 25-year period with multimicrobial including E. coli purulent pericarditis from complicated acute appendicitis.[15] Ku et al. reported a 14-year-old person with ruptured appendicitis complicated by a large pericardial effusion and also identified five other case reports of pericardial effusion complicating perforated acute appendicitis in their review of the English literature from 1946 to 2017.[16] Fortunately, despite the gross pericardial contamination and placement of a mechanical valve graft conduit, there was no observed postoperative prosthetic valve or graft infection up to 2 years of follow-up. The delayed chest closure for 48 h and dilute betadine normal saline continuous mediastinal irrigation for 5 days postoperatively, and broad-spectrum intravenous antibiotics might have contributed to this outcome. Although speculative, it is possible that earlier intervention with removal of the appendix might have prevented necrosis and subsequent hematogenous bacterial spread. However, a therapeutic dilemma might have arisen if the ATAAD had been diagnosed on admission along with acute appendicitis. Since ATAAD is more life-threatening, it would have taken precedence over acute appendicitis. A one-stage aortic repair followed by appendectomy if patient was hemodynamically stable might have been the best strategy. Although this might be associated with a higher risk of infecting the prosthetic valve conduit doing a contaminated case with a clean case. The alternative would have been a two-stage approach with aortic repair followed by appendectomy in 24 h if patient hemodynamically stable. This approach also has the downside risk of possible interval rupture of the appendicitis. With no previous reports of this rare clinical presentation for guidance, the surgeons faced with this scenario would have to make management decisions based on individual patients' circumstances and hemodynamic status. Despite several technical advances, operative mortality remains high with surgery for ATAAD, ranging from 15% to 32% in recent publications.[17],[18],[19] Even higher mortality ranging from 29% to 43.7% is reported with multiorgan malperfusion and as high as 75% in the subgroup with mesenteric ischemia.[20],[21],[22] This case presents an educational opportunity to increase physicians awareness, especially among those involved with emergency care services, on the possible atypical presentation of acute aortic dissection as primarily abdominal pain. Even after the patient's missed ED diagnosis of ATAAD, it took another 48 h before the correct diagnosis was finally made to enable appropriate surgical intervention. This patient also exemplifies the observations of Viljanen [5] of >24 h delayed diagnosis in 40% of patients with acute aortic dissection.
Figure 5: Computed tomography abdomen axial view black arrows showing dissecting flap in the thoracic and abdominal aorta.

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Figure 6: Scout film for computed tomography abdomen.

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  Conclusion Top

Marfan syndrome patients who present to the ED with acute abdominal pain should always be ruled out for acute aortic dissection regardless of any other obvious pathology to allow early intervention for best outcomes.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mészáros I, Mórocz J, Szlávi J, Schmidt J, Tornóci L, Nagy L, et al. Epidemiology and clinicopathology of aortic dissection. Chest 2000;117:1271-8.  Back to cited text no. 1
Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL, et al. The International Registry of Acute Aortic Dissection (IRAD): New insights into an old disease. JAMA 2000;283:897-903.  Back to cited text no. 2
Upchurch GR Jr., Nienaber C, Fattori R, Evangelista A, Oh J, Cooper JV, et al. Acute aortic dissection presenting with primarily abdominal pain: A rare manifestation of a deadly disease. Ann Vasc Surg 2005;19:367-73.  Back to cited text no. 3
Sullivan PR, Wolfson AB, Leckey RD, Burke JL. Diagnosis of acute thoracic aortic dissection in the emergency department. Am J Emerg Med 2000;18:46-50.  Back to cited text no. 4
Viljanen T. Diagnostic difficulties in aortic dissection. Retrospective study of 89 surgically treated patients. Ann Chir Gynaecol 1986;75:328-32.  Back to cited text no. 5
Lo C, Chen C, Hsueh C, Chin H, Feng N. Aortic dissection mimics acute abdomen in an older patient. Int J Gerontol 2012;6:140-3.  Back to cited text no. 6
Lin LF, Tung JN. An uncommon cause of acute abdomen: Aortic dissection complicated by superior mesenteric artery occlusion. J Dig Dis 2009;10:74-5.  Back to cited text no. 7
Besli GE, Durakbasa CU, Yildirim S, Acar M, Cakir B. Acute aortic dissection mimicking acute abdomen in a 14-year-old boy. Pediatr Int 2015;57:1169-71.  Back to cited text no. 8
Slater EE, DeSanctis RW. The clinical recognition of dissecting aortic aneurysm. Am J Med 1976;60:625-33.  Back to cited text no. 9
Khan IA, Nair CK. Clinical, diagnostic, and management perspectives of aortic dissection. Chest 2002;122:311-28.  Back to cited text no. 10
Larson EW, Edwards WD. Risk factors for aortic dissection: A necropsy study of 161 cases. Am J Cardiol 1984;53:849-55.  Back to cited text no. 11
Addiss DG, Shaffer N, Fowler BS, Tauxe RV. The epidemiology of appendicitis and appendectomy in the United States. Am J Epidemiol 1990;132:910-25.  Back to cited text no. 12
Silverman DI, Burton KJ, Gray J, Bosner MS, Kouchoukos NT, Roman MJ, et al. Life expectancy in the Marfan syndrome. Am J Cardiol 1995;75:157-60.  Back to cited text no. 13
Judge DP, Dietz HC. Marfan's syndrome. Lancet 2005;366:1965-76.  Back to cited text no. 14
Tan EC, Rieu PN, Nijveld A, Backx AP, Meis JF, Severijnen RS, et al. Pericarditis as complication of appendicitis. Ann Thorac Surg 2004;78:1086-8.  Back to cited text no. 15
Ku D, Cassey JG, Mejia R. Pericardial effusion as a rare complication of a perforated appendicitis. Int J Surg Case Rep 2017;35:98-100.  Back to cited text no. 16
Rampoldi V, Trimarchi S, Eagle KA, Nienaber CA, Oh JK, Bossone E, et al. Simple risk models to predict surgical mortality in acute type A aortic dissection: The International Registry of Acute Aortic Dissection score. Ann Thorac Surg 2007;83:55-61.  Back to cited text no. 17
Booher AM, Isselbacher EM, Nienaber CA, Trimarchi S, Evangelista A, Montgomery DG, et al. The IRAD classification system for characterizing survival after aortic dissection. Am J Med 2013;126:730.e19-24.  Back to cited text no. 18
Chikwe J, Cavallaro P, Itagaki S, Seigerman M, Diluozzo G, Adams DH, et al. National outcomes in acute aortic dissection: Influence of surgeon and institutional volume on operative mortality. Ann Thorac Surg 2013;95:1563-9.  Back to cited text no. 19
Pacini D, Leone A, Belotti LM, Fortuna D, Gabbieri D, Zussa C, et al. Acute type A aortic dissection: Significance of multiorgan malperfusion. Eur J Cardiothorac Surg 2013;43:820-6.  Back to cited text no. 20
Girdauskas E, Kuntze T, Borger MA, Falk V, Mohr FW. Surgical risk of preoperative malperfusion in acute type A aortic dissection. J Thorac Cardiovasc Surg 2009;138:1363-9.  Back to cited text no. 21
Nwiloh J. Surgery for acute Stanford Type A aortic dissection in an inner city community hospital: Single surgeon's experience. World J Cardiovasc Surg 2016;6:25-33.  Back to cited text no. 22


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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