American Journal of Emergency Medicine 32 (2014) 1152.e1–1152.e2
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Phantom organ pain syndrome, a ghostly visitor to the ED☆
Abstract Phantom organ pain syndrome is a phenomenon affecting individuals who have had an organ surgically removed. It occurs presumably as deafferentation due to surgical trauma to the incision site compromising deep somatic nerves and visceral sensory nerves innervating the missing organ. Although countless patients have had organs removed and an innumerable amount of patients have subsequently experienced pain, we are only able to ﬁnd a handful of cases of phantom organ pain syndrome described in the literature. We report a case of a young male who visited our emergency department (ED) with chronic left ﬂank pain despite ongoing therapy with hydrocodone. He had history of chronic pyelonephritis and staghorn calculus. He had a left-sided nephrectomy due to nearly fatal emphysematous pyelonephritis. The pain was described as “the same” as that he had experienced for years during pyelonephritis exacerbations. His numerous visits to primary care, specialty clinics, and other EDs to obtain pain killers had already triggered an alert by the Controlled Substances Prescription Monitoring Program as a potential drug diverter. He had extensive postsurgical workup including negative results from magnetic resonance imaging, computed tomography of the abdomen and pelvis with and without contrast, ultrasound, and abdominal x-rays. Despite a concern about drugseeking behavior, there was no evidence of aberrant behavior or drug diversion. With a gabapentin trial initiated in the ED and subsequent supportive psychotherapy offered in a pain medicine service clinic, his pain was successfully controlled with no opiates. Currently, he is on maintenance doses of gabapentin. A 43-year-old man with recurrent left pyelonephritis and staghorn calculus presented to our emergency department (ED) with 8-month history of left ﬂank pain. Six months before, he underwent left-sided nephrectomy. His pain was aching and dull with paroxysms of electricity and burning sensation. The average intensity of the pain was 7/10 on visual analog scale. There were no modifying factors, but the nightly symptoms were causing him a sleep disturbance. The pain was unchanged from what he had experienced during the pyelonephritis episodes. The patient was well known by the departments of surgery, nephrology, and family medicine and local EDs. He was prescribed oral opiates in most visits. Unsurprisingly, his name had been ﬂagged as a potential drug diverter. He had extensive negative postsurgical workup including abdominal magnetic resonance imaging, computed tomographic scans, ultrasound, and abdominal x-rays. In addition, urine and blood analyses were unremarkable. He did not use tobacco,
☆ No sources of support or prior presentations to report. 0735-6757/© 2014 Elsevier Inc. All rights reserved.
alcohol, or illicit drugs. He denied taking medications other than the prescribed hydrocodone. His vital signs were normal; the physical examination had only positive result for a well-healed nontender surgical scar at the left ﬂank. There was no indication for additional tests. Despite our concern about drug-seeking behavior, there was no evidence of aberrant behavior or drug diversion. Considering a diagnosis of phantom organ pain syndrome (POPS), we performed a literature search. Gabapentin was mentioned as a therapeutic alternative. A trial of oral 300 mg/d was started; he was also referred to the pain department. Four weeks later, we followed up with the consulting service, which conﬁrmed our diagnosis. The patient was then taking gabapentin 600 mg 3 times a day and receiving supportive psychotherapy. His pain was successfully controlled with no opiates. Chronic pain affects more than 70 million Americans . Survival of many conditions considered fatal in recent years contributes to the growth of the population with chronic pain. Emergency physicians (EPs) are affected by biases to treat chronic pain . Furthermore, the biggest obstacle is the lack of knowledge. Like POPS, many patients with chronic pain syndromes are misclassiﬁed as drug addicts or drug diverters. Unlike phantom limb pain, a familiar condition that occurs in 60% to 80% of patients after limb amputation , POPS is an uncommon condition; some authors even question its existence. The frequency and etiology still remain unclear . The current data of POPS are predominantly based on case reports including teeth, nose, tongue, breast, testis, male-to-female transsexual penises, anus, and a few visceral organs including appendix, bladder, and gall bladder [5,6]. No reports of POPS of the kidney were found. Multiple theories try to explain POPS pathophysiology; none have been supported to a great extent. The neuromatrix theory proposes that the conscious awareness of organs is generated in the brain via different perceptual inputs . These inputs like pain can create a neurosignature. The neurosignature of a speciﬁc body part determines how it is consciously perceived . This neurosignature may be the cause of organ pain even after its removal. Thereby, POPS may arise from abnormal reorganization in the neuromatrix to a preexisting pain state . A diagnosis criterion for POPS has not been clearly accepted; some consider it a rule out. However, POPS should be considered in patients who underwent organ removal and are experiencing pain of similar nature to the presurgical stage. The pain is typically a combination of visceral nociception (secondary to distension, overstretching, or inﬂammation) and neuropathic (continuous and/or paroxysmal with qualities including electric, burning or coldness, “pins and needles,” numbness or itching) . The treatment of POPS yields more questions than answers. A wide variety of pharmacologic agents have been used including Nmethyl-D-aspartate (NMDA) receptor-antagonists, gamma-
C.J. Roldan, J.S. Lesnick / American Journal of Emergency Medicine 32 (2014) 1152.e1–1152.e2
aminobutyric acid (GABA) analogs, anticonvulsants, antidepressants, and muscle relaxants. Interventional options include acupuncture, nerve blocks, neurolysis, sympathectomy, spinal cord stimulators, deep brain stimulators, and even cortical brain resection. Alternative therapies include rehabilitation and physical therapy, electromyographic and thermal biofeedback, psychological treatments, and hypnosis. Limitations of an ED make the diagnosis of some chronic pain syndromes an unrealistic task. However, the unavoidable exposure to such conditions is a challenge that EPs have to face. The EP has an important role in differentiating chronic pain syndromes from other conditions. As such, pseudoadiction (patient behavior that simulates true addiction occurs secondary to inadequate pain control)  and diversion (transfer of prescription drugs from a lawful to an unlawful distribution or use)  should be distinguished. In addition, the EP has an important role in referring suspected undertreated pain syndromes to a pain specialist. Phantom organ pain syndrome might be a rare condition, yet one should consider it in the patient with combined visceral and neuropathic pain after organ surgical removal. When suspected, patients should be referred to a pain service. Opiates pseudoadiction is clinical scenario commonly seen in the ED, and efforts should be made not to misclassify patients with chronic pain.
Carlos J. Roldan, MD Department of Emergency Medicine The University of Texas Health Science Center at Houston Houston, TX 77030, USA Department of Emergency Medicine The University of Texas MD Anderson Cancer Center Houston, TX 77030, USA
Joseph S. Lesnick, MD Department of Emergency Medicine The University of Texas Health Science Center at Houston Houston, TX 77030, USA E-mail address: [email protected]
References  Institute of Medicine. Report from the Committee on Advancing Pain Research, Care, and Education: relieving pain in America, a blueprint for transforming prevention, care, education and research. The National Academies Press, 2011. http://books.nap.edu/openbook.php?record_id=13172&page=1.  Todd KH, Ducharme J, Choiniere M, et al. Pain in the emergency department: results of the Pain and Emergency Medicine Initiative (PEMI) multicenter study. J Pain 2007;8(6):460–6.  Nikolajsen L, Staehelin T. Phantom limb pain. Curr Rev Pain 2000;2(4):166–70.  Dorpat TL. Phantom sensation of internal organs. Compr Psychiatry 1971;12:27–35.  Park KE, Cheon KS, Ok SH, et al. Phantom bladder pain. Korean J Anesthesiol 2012;63(4):376–7.  Ramachandran VS, Hirstein W. The perception of phantom limbs. The D. O. Hebb lecture. Brain 1998;121(9):1603–30.  Giummarra MJ, Gibson SJ, Georgiou-Karistianis N, et al. Central mechanisms in phantom limb perception: the past, present and future. Brain Res Rev 2007;54(1):219–32.  Bittar RG, Otero S, Carter H, et al. Deep brain stimulation for phantom limb pain. J Clin Neurosci 2005;12(4):399–404.  Melzack R. Phantom limbs. Sci Am 1992;266(4):120–6.  Bouhassiraa D, Attala N, Fermanianc J, et al. Development and validation of the Neuropathic Pain Symptom Inventory. Pain 2004;108:248–57.  Weissman DE, Haddox JD. Opioid pseudoaddiction—an iatrogenic syndrome. Pain 1989;36(3):363–6.  Inciardi JA, Surratt HL, Cicero TJ, et al. Prescription opioid abuse and diversion in an urban community: the results of an ultra-rapid assessment. Pain Med 2009;10(3):537–48.