Psychosomatics 48:355-358, July-August 2007
doi: 10.1176/appi.psy.48.4.355
© 2007 Academy of Psychosomatic Medicine
Factitious Aortic Dissection Leading to Thoracotomy in a 20-Year-Old Man
Elise Chambers, M.D., Capt. USAF,
Joel Yager, M.D.,
William Apfeldorf, M.D., Ph.D., and
Eduardo Camps-Romero, M.D.
Received May 2, 2005; revised September 26, 2006; accepted October 3, 2006. From the 35th Aerospace Medicine Squadron, Misawa Air Base, Japan, and the Dept. of Psychiatry, Univ. of New Mexico School of Medicine, Albuquerque, NM. Send correspondence and reprint requests to Joel Yager, M.D., Professor and Vice Chair for Education and Academic Affairs, Dept. of Psychiatry/MSC09 5030,Univ. of New Mexico School of Medicine, 1 Univ. of New Mexico, Albuquerque, NM 87131-0001. e-mail: jyager{at}unm.edu
© 2007 The Academy of Psychosomatic Medicine
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ABSTRACT
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A 20-year-old man presented to an emergency department with dramatic, sudden-onset, tearing chest pain. He also claimed to have been previously diagnosed with Ehler-Danlos syndrome and a previous Type I aortic dissection (intimal tear of ascending aorta), rapidly increasing his treating physician’s suspicion of an emergent aortic dissection. The patient was quickly transferred to a large university hospital, where he underwent a median sternotomy and thoracotomy, with no aortic pathology found on operation and biopsy. After the patient’s postoperative recovery, he was treated at a mental health facility, where he remained ambivalent about his psychiatric condition and did not respond well to treatment. This case report describes a unique case of factitious disorder that led to a serious operative intervention and subsequent psychiatric care and assesses factors that might have contributed to his hospital course.
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Case Report
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"Mr. MH," a 20-year-old man, presented to a small community hospital complaining of sudden-onset, severe, ripping chest pain radiating to the back. He also claimed to have been diagnosed several years previously with Ehler-Danlos syndrome and some category of Type I aortic dissection. The patient stated that the pain he was experiencing resembled that of his previous dissection. He was traveling through the state and had no medical records available for the evaluating physicians. Because of the grave prognosis for an aortic dissection without treatment, surgeons at the community hospital and medical center with which they consulted decided that the patient should be immediately transferred, via fixed-wing transport, to a large, university hospital, where surgeons would be waiting to operate. Upon arrival at our hospital, he again presented the same dramatic complaints of severe chest pain radiating to the back, "interscapular area," and right neck.
The medical records from that initial encounter indicated that he had a past medical history of Ehler-Danlos syndrome and Type I dissection and a surgical history of multiple central-line placements (because of highly sensitive arm veins); his medications included labetolol, clonidine, and nimopidine; he was allergic to gadolinium, iodine, prochlorperazine, and morphine; his family history included the fact that his father had died from a ruptured thoraco-abdominal aneurysm, and both his mother and grandmother had died of aneurysms. He indicated that he was a minister by profession, and he just happened to be traveling through the state. Significant findings on physical exam included multiple old scars secondary to central-line placements (internal jugular and subclavian). Cardiac examination revealed normal S1–S2 and no murmurs.
Mr. H was dramatic in his complaints of pain to physicians and staff. In the Emergency Department, he was periodically tachycardic, but normotensive, and he was breathing at a regular rate, without an oxygen requirement. It was also noted that the patient repeatedly requested meperidine for pain because of his morphine allergy. An ECG showed normal sinus rhythm, nonspecific I–V conduction delay, and prolonged Q–T interval. He was mildly anemic (Hemoglobin/Hematocrit: 9.9/30), and his white-cell count was 10.7. Electrolytes and cardiac enzymes were within normal limits.
The cardiothoracic surgery team operated emergently. They were highly concerned about the risk of aortic rupture and an acute extension of an intimal flap in the ascending aorta. The team had received information from the transferring physician (based on the patient’s account) that he had been seen previously in at least four hospitals in surrounding states for similar complaints. The transferring physician also thought that recent MRIs and echocardiograms at some of these institutions had been suspicious for or confirmed an intimal flap of the aorta. An MRI from the day before at a different institution had been read to show an intimal flap.
The patient underwent left femoral-artery cannulation, sternotomy, cardiopulmonary bypass, aortotomy, and biopsy of the aortic wall. The surgeons found entirely normal anatomy. The aorta was normal in size, without aneurysm, and its wall was thick (atypical for the thin-walled aortas of Ehler-Danlos and Marfan-syndrome patients). There was no evidence of intimal flap or tear of the aorta. A full-thickness biopsy of the aortic wall showed no acute or chronic pathology.
During his postoperative recovery, for several days, the patient adamantly refused to have his staples, central line, and Foley catheter removed, despite the surgeons’ warnings of the dangers of leaving these lines in place. Although he eventually complied, the patient was extremely uncooperative and also refused to get out of bed and ambulate despite multiple conversations with the surgeons and physical therapists about its importance. He continually asked to receive only meperidine in very large doses for pain, and, on several occasions, he threatened to leave against medical advice.
During his postoperative course, all possible previous medical records were obtained. Including this hospitalization, the patient had been seen and admitted to three hospitals in our state and four hospitals in surrounding states in the previous 90 days. He presented with consistent complaints of severe chest pain, a history of Type I aortic dissection, Ehler-Danlos and sometimes Marfan’s syndrome, and hemophilia, with multiple severe drug allergies. In fact, 3 days before his transfer to our Emergency Department, he had been seen and admitted to our own cardiology service. That team was suspicious of the patient’s history, suggesting possible factitious disorder in their admission plan. However, they believed that he was worthy of further work-up because of the gravity of aortic dissections. At that time, a trans-esophageal echocardiogram performed by the attending cardiologist was negative for dissection. The cardiology team did not find further abnormalities on CT scan, and he was discharged the next day.
The patient was eventually deemed stable and was transferred to the University Psychiatric Center. The team’s initial treatment plan included a multidisciplinary approach with a behavioral plan and, because of an initial diagnosis of "probable psychosis," antipsychotic medications (which the patient refused).
At first, the team did not want to confront the patient’s defenses "head-on." Eventually, the staff managed to identify and contact the patient’s stepmother and biological mother, each of whom gave spotty histories of the patient’s having been on haloperidol and hearing voices in the past. They were both extremely worried and knew he had been fabricating illnesses and traveling the country.
On the unit, the patient had to be watched constantly because he began picking at his surgical wounds. He continued to complain of severe chest pain and was worried that he might have pericarditis. Each time he complained, he was thoroughly evaluated, and no abnormalities were detected on exam. On one occasion, he complained dramatically of severe headaches and told the night-float resident he had been previously diagnosed with a brain aneurysm. He received an MRI of the brain, which was normal. He continued to insist that he really did have an aortic dissection up until a few days before discharge, at which point he finally expressed understanding that if the condition was not found at surgery, it probably was not there. He continued to refuse all psychiatric medications and did not improve psychiatrically with the psychosocial therapies made available to him. He was discharged after 10 days at the mental health center and was provided with a bus ticket to his mother’s house, several states away.
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Discussion
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We conducted an exhaustive literature search to inquire whether any previously published reports described patients undergoing emergency open-heart surgery for factitious symptoms. The databases searched were PubMed, PsychInfo, SciSearch, and Google Scholar (Beta). Although factitious disorder is not uncommon in surgical settings, (indeed, one study identified 41 cases of surgical-ward patients in a major teaching hospital1 over a 2-year period), we could identify no previous cases of factious disorder actually leading to major cardiovascular surgeries. With respect to factitious cardiovascular disease, reported cases have described what has become known as the "cardiac Munchausen syndrome," or "cardiopathica fantastica," involving patients who feign chest pain, arrhythmias, and hypertension.2 One reported a patient who feigned angina over a period of many months and who underwent multiple balloon catheterizations and eventual bypass surgery.3 However, no previous cases have reported emergency thoracotomy due to factitious aortic dissection.
DSM–IV describes the essential features of factitious disorder: intentional production of physical or psychological signs or symptoms; motivation for the behavior being to assume the sick-role; and external incentives for the behavior being absent.4 In the most dramatic form of factitious disorder, known as Munchausen’s syndrome, first described by Asher in 1951,5 core elements include recurrent hospitalization, peregrination (travel), and pseudologica fantastica. These patients have been described in past literature as "peregrinating problem patients and ‘hospital hoboes’ ... with psychopathic personalities and hospital-addiction syndrome."6 In a series of 42 cases described in 1983 by Carney and Brown,7 most were described as single, separated, or divorced; emotionally deprived; and psychopathic. The authors described several distinct subgroups, including wanderers and multiple-system patients, among others. Our patient demonstrated an extreme form of factitious illness, feigning his way through multiple medical facilities and lying about his profession and family history, until he was subjected to one of the most invasive surgeries in modern medicine.
The differential diagnosis for such patients usually includes conversion disorders on Axis I and the Axis V code, Malingering. According to DSM–IV-TR, in conversion disorder, the symptoms are not "intentionally" feigned, as they are in factitious disorder or malingering, and, in factitious disorder, the key motivation is to assume the sick-role and to obtain medical evaluation and treatment. By contrast, in malingering, more obvious goals, such as financial compensation, avoidance of duty, evasion of criminal prosecution, or obtaining drugs, are apparent. Taking all of these features and our patient’s history into account, factitious disorder is most likely. Admittedly, these labels are limited in what they actually tell us about the patient.
A full understanding of the patient’s Axis II diagnosis is problematic, since, as is the case with many patients with factitious disorder, he did not remain in treatment long enough and did not willingly participate in a complete assessment. His lying and wandering qualities suggest antisocial features. Desiring repeatedly to assume the "sick-role" suggests dependent features. Seeking out and seemingly craving invasive and mutilating procedures suggests masochistic and self-mutilating elements, which might have sexual-fetishistic elements. His lack of connection or attachment and possible "micropsychotic" features (it remains unclear as to whether he intermittently experienced hallucinations) suggest a severe personality disorder, at best, perhaps in the schizoid or schizotypal group, or borderline personality organization.
Sorting out his medical history, to include where he received what type of care, proved to be extremely challenging. Munchausen patients are often extremely bright and well-read on the medical illnesses they feign, "often with a mastery of medical terms."8 This patient was extremely well-informed about connective-tissue diseases and their complications. He seemed to know exactly what history and symptoms to describe, all designed to ensure that he would be subjected to extremely invasive procedures.
No effective treatment options for patients with factitious disorders and Munchausen’s syndrome exist at this time. Complicating management efforts are the often strong, negative countertransferance feelings emerging in care-providers.9 Such negative reactions were evident among nearly all of our patient’s staff, including physicians, nurses, and social workers. One daily note described the patient as an "outright psychopath." Many noted that the patient was splitting staff. Remission rates are high, and prognosis is poor for these patients, who are usually impervious to all efforts at therapy.1,10 One study reported that only 1 out of 10 patients with factitious disorder would ever admit to feigning their symptoms.10 Not surprisingly, our patient was discharged without having made much progress in understanding his true illness.
Instructive aspects of this case bear particular mention. As noted by the admitting cardiology team, factitious disorder was on the initial differential-diagnosis list for MH. Unfortunately, because of the early-morning hours of his presentation before the surgery and the grave prognosis of untreated aortic dissections, the cardiothoracic surgical staff found it impossible to sort out the many details of MH’s history within the rapid time-frame in which they felt compelled to act. This dilemma underscores the need for improved communication early-on between medical centers faced with patients like MH. Easily-accessible electronic medical records within and shared among medical centers might have helped to avert this unnecessary surgery. Clearly, he was well-known at multiple facilities. However, the fact that a few studies read in the past were said to suggest that he might have had an intimal flap further confused physicians later in the patient’s course. Nevertheless, he had never actually been officially diagnosed with Ehler-Danlos or Marfan syndrome, nor had he ever produced any pertinent medical records or given names of previous physicians who had treated him for these diseases.
Although patients like MH are rare, such patients require optimal efforts to communicate with other hospitals at which they have been seen. Also, efforts should be made to contact family members as soon as possible from the emergency department. We were able to do so only after the surgery, once the previous medical records were reviewed. Certainly, this patient’s condition and course of treatment were financially and emotionally costly for all involved in his care. Improving communication among hospitals and with family members might potentially prevent other Munchausen patients from enduring similar catastrophic encounters.
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REFERENCES
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- Reich P, Gottfried LA: Factitious disorders in a teaching hospital. Ann Intern Med 1983; 99:240–247[Abstract/Free Full Text]
- Park TA, Borsch MA, Dyer AR, et al: Cardiopathica fantastica: the cardiac variant of Munchausen syndrome. Southern Med J 2004; 97:48–52[CrossRef][Medline]
- Mumford M, Tobis J: A case of Munchausen’s syndrome masquerading as unstable angina. J Natl Med Assoc 1981; 73:661–664[Medline]
- American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th Edition. Washington, DC, American Psychiatric Association, 2000, pp 513-517
- Asher R: Munchausen’s syndrome. Lancet 1951; 1:339–341[CrossRef][Medline]
- Yassa R: Munchausen’s syndrome: a successfully treated case. Psychosomatics 1976; 19:242–243
- Carney MW, Brown JP: Clinical features and motives among 42 artifactual-illness patients. Br J Med Psychol 1983; 56:57–66[Medline]
- Bursten, B: On Munchausen’s syndrome. Arch Gen Psychiatry 1965; 13:261–267[Abstract/Free Full Text]
- Feldman JM, Feldman MD: Tangled in the web: countertransference in the therapy of factitious disorders. Intl J Psychiatry Med 1995; 25:389–399[Medline]
- Sutherland AJ, Rodin GM: Factitious disorders in a general-hospital setting: clinical features and review of the literature. Psychosomatics 1990; 4:392–398
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ABSTRACT
Case Report
