Case Report

Management of Chronic Pain in a Patient With AutosomalDominant Polycystic Kidney Disease by Sequential Celiac

Plexus Blockade, Radiofrequency Ablation, and SpinalCord Stimulation

Nathaniel Walsh, BS, BA,1 and Jose E. Sarria, MD2

Chronic pain frequently is associated with autosomal dominant polycystic kidney disease and is a significantcause of morbidity. The classic approach to treat pain in patients with this disease starts with nonpharmaco-logic therapy and progresses to high-dose opioid therapy and more invasive procedures, including surgery. Wepresent the case of a 43-year-old white woman presenting in our clinic with poorly controlled chronic left flankand epigastric pain secondary to autosomal dominant polycystic kidney disease despite high-dose opioids andmultiple cyst decompression procedures. After temporarily successful management with celiac plexus neuroly-sis and intercostal nerve radiofrequency ablations for years, the next more permanent step was dorsal columnneurostimulation, affording excellent analgesia with significantly improved quality of life to this day.Am J Kidney Dis. 59(6):858-861. © 2012 by the National Kidney Foundation, Inc.

INDEX WORDS: Autosomal dominant polycystic kidney disease; chronic pain; spinal cord stimulation.

Pain is a common symptom in autosomal domi-nant polycystic kidney disease (ADPKD).1 Pain

may be acute due to cyst hemorrhage, infection, ornephrolithiasis, but also may become chronic anddetrimentally affect the patient’s quality of life.2 Formost patients, pain can be controlled effectively withconservative management, but a small percentage willneed a multidisciplinary approach, including surgeryand/or pain management (Fig 1). These patients fre-quently are on high-dose opioid therapy and experi-ence the negative effects of such a regimen, includingopioid-induced hyperalgesia, cognitive dysfunction,hypogonadism, constipation, nausea, psychosocial dys-function, depression, and generally low quality oflife.3 The following case report describes the interven-tional pain management of a patient with chronicintractable disabling pain from ADPKD who experi-enced temporary success in treatment with celiacplexus neurolysis and intercostal nerve radiofre-quency ablation, but ultimately underwent implanta-tion of a dorsal column neurostimulator with long-lasting success.

From the 1University of South Florida College of Medicine; and2Anesthesiology, Interventional Pain Service, Moffitt Cancer Cen-ter and Research Institute, Tampa, FL.

Received July 13, 2011. Accepted in revised form December 17,2011. Originally published online February 24, 2012.

Address correspondence to Jose E. Sarria, MD, 12902 Magno-lia Dr, Ste 12557, Tampa, FL 33612. E-mail: jose.sarria@moffitt.org

© 2012 by the National Kidney Foundation, Inc.0272-6386/$36.00

doi:10.1053/j.ajkd.2011.12.018

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CASE REPORTA 43-year-old white woman was referred to our clinic with a

long-term history of pain secondary to ADPKD localized to theepigastrium, dating back to June 2004, described as severe, dif-fuse, and colicky and often accompanied by nausea. She alsoreported a constant dull nagging sensation in the left flank andlumbar areas, often radiating to the abdominal wall in a T11dermatomal fashion that was aggravated by inspiration, withfrequent episodes of lancinating pain in the same areas. The mostsevere occurrences lasted hours and were scored at 10 of 10 on anumerical rating scale.

Upon initial evaluation in November 2006, she was on high-dose opioid therapy, including morphine sulfate at 100 mg/d plusoxycodone/acetaminophen 7.5 mg/325 mg at an average of 30-60mg of oxycodone per day. Quality of life was poor, and the patientwas incapacitated by the pain to the point that activities of dailyliving were difficult to complete at times. Additionally, the patientexperienced opioid side effects, including persistent somnolenceand constipation, along with feelings of depression and helpless-ness. Although complete outside records were not able to beobtained, computed tomography–guided cyst aspirations andsurgical cyst reduction operations had been performed. Further-more, abdomino-pelvic computed tomographic scans from 2006and 2007 showed bilateral renal cysts with evidence suggestiveof hemorrhage along with areas of prior infection and scarringin the left kidney. The largest left renal cyst was located in theperipelvic area (Fig 2). Findings from an outside intravenouspyelogram were normal. Finally, laboratory workup showedpreserved kidney function with normal urinalysis results, serumcreatinine levels ranging from 0.7-0.9 mg/dL (61.88-79.56�mol/L), and serum urea nitrogen levels ranging from 8-20mg/dL (3-7 mmol/L).

On physical examination, we corroborated the presence of 2pain generators: a parietal component (superficial, localized, andreproducible with inspiration) responsible for the left flank painwith radiation to the abdominal wall and a visceral component(deep and poorly localized) responsible for the epigastric pain (Fig3). Alleviation of the latter was priority for her, so we chose toproceed with a diagnostic fluoroscopy-guided celiac plexus blockby retrocrural approach followed with alcohol neurolysis. About

90% relief of the epigastric pain was achieved in less than 1 week

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Management of Chronic Pain in ADPKD

with no side effects. She required 6 repeated blocks every 4-6months during the next 27 months. These eventually were discon-tinued due to 2 incidences of temporary numbness and weakness inher lower extremities, a known complication that occurs fromspread of the alcohol into the spinal canal.

Concurrently, the patient’s left flank pain also was addressed. Aleft T11 and T12 intercostal nerve diagnostic local anestheticinjection provided 100% pain relief for more than 4 hours, justify-ing subsequent thermal radiofrequency ablation of these nerves.This procedure was very successful at temporarily relieving her

Figure 1. Pain management algorithm for polycystic kidney ddal anti-inflammatory drugs. Modified and reproduced from Bajw

Figure 2. Computed tomography of the abdomen-pelvis with

cysts with evidence of hemorrhage and scarring. Slices 43 (left panel)

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flank pain, and repeated radiofrequency ablations were needed atconsistent 4-month intervals. A total of 7 radiofrequency ablationswere performed. Of note, while undergoing these procedures, thepatient was able to wean the opioid dose to once- or twice-dailyhydrocodone/acetaminophen 7.5/500 mg. Furthermore, she wasable to return to work, and her overall quality of life dramaticallyimproved.

Ultimately, due to these complications and only temporary painrelief, neurostimulation was considered and the patient underwent a1-week trial of dorsal column stimulation. After obtaining �80%

e. Abbreviations: COX-2, cyclooxygenase 2; NSAIDs, nonsteroi-l,1 with permission of Macmillan Publishers Ltd.

without contrast from August 2006 shows multiple bilateral renal

and and 45 (right panel) of 101 are shown.

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Walsh and Sarria

analgesia of both the visceral and parietal components, she underwentpermanent implantation with an uncomplicated recovery. On fol-low-up visits, she has continued to report excellent results with thespinal cord stimulator. She stated her pain was a 1 of 10 and continuesto be extremely satisfied with the results up to the time of this writing.

DISCUSSION

ADPKD is an important source of morbidity and oneof the leading causes of end-stage renal disease. Pain is avery prevalent symptom (60%) that often leads to thediagnosis of this condition.4 The most common ana-tomic locations of the pain are the low back (71.3%) andabdomen (61.4%), with pain intensities ranging frommild to severe in a normal distribution.5

The pathogenesis of pain can be attributed to me-chanical effects of enlarging cysts on nearby struc-tures and distension of the renal capsule.1 We believethat 2 neural pathways are relevant in conveying painsensations from this area. First, a visceral pathway, inwhich afferent fibers from the renal capsule, paren-chyma, and vasculature travel to the neuraxis through

Figure 3. Innervation of kidney. Abbreviation: n., nerve. Re-produced from Bajwa et al1 with permission of Macmillan Publish-ers Ltd.

sympathetic nerves, passes through the prevertebral

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ganglia (most notably aorticorenal and celiac ganglia)to join the lesser and least splanchnic nerves. Thesenerves then travel cranially along the retrocrural spaceto gain access to the T10-T12 and L1 spinal levelsthrough the respective paravertebral ganglia and ramicommunicans. Second, part of the renal capsule andadjacent musculoskeletal structures in the retroperito-neum are served by somatic nerves, more specificallythe intercostal nerves, typically corresponding to der-matomal levels T7-T12. Convergence of both path-ways at the spinal cord accounts for referred pain andthe difficulty localizing the pain generators (Fig 3).

Chronic pain results not only from sustained periph-eral impulses of the target injured tissues, but alsofrom neuroplastic changes that occur as a result.Classic animal models of chronic pain describe theneurophysiologic, neurochemical, and anatomical phe-nomena that occur in the afferent fibers at the dorsalhorn level that contribute to amplification and perpetu-ation of the sensation of pain. These processes arecollectively called central and peripheral sensitiza-tion.6,7 Analgesic regimens tend to fail when thisoccurs and in the case of opioids, may aggravate theproblem because it now is believed that chronic expo-sure to opioids contributes to perpetuation of centralsensitization.8

Surgical management of intractable pain in ADPKDhas shown good results, but the long-term benefit is un-clear.9 Even after nephrectomy, chronic pain in patientswithADPKD may not subside. Interventional pain manage-ment, at least according to the literature, is a rarely exploredroute. However, with the basis of an anatomical and neuro-physiologic understanding of the pathways involved, suc-cessful pain management is possible.

In our case, most described therapies for the pa-tient’s chronic pain had failed. Consequently, otherapproaches with a good safety record and well vali-dated for other indications seemed a reasonable alter-native. Celiac plexus neurolysis to interrupt the vis-ceral pathway and intercostal nerve radiofrequencyablation to interrupt the somatic pathway initiallywere undertaken. Ultimately, a more definitive solu-tion seemed necessary and neurostimulation was cho-sen for its efficacy in localized opioid-resistant pain,particularly if neuropathic changes have occurred.

Spinal cord stimulation is an analgesic techniquewith tremendous potential for pain relief. Originallydescribed in a patient with cancer in 1967,10 dorsalcolumn stimulation was the original system based onthe “gate control theory” by Melzack and Wall11

proposed only 2 years before. The basic concept ofspinal cord stimulation revolves around the fact that inspinal cord pathways, when a sensory modality isapplied to an increased level, it can “close the gate” to

other modalities, thus modulating the traffic of these

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Management of Chronic Pain in ADPKD

signals to higher centers. More specifically, electricalstimulation of the dorsal column has a neuromodula-tory effect on the activity of the ascending painpathways. Our patient received a spinal cord stimula-tion implant and has had continued analgesia andimproved quality of life. She now is working, travel-ing, and performing activities of daily living on mini-mal doses of opioids 2 years after implantation of thespinal cord stimulation.

To our knowledge, there is only an anecdotal men-tion of spinal cord stimulation for ADPKD in thereview article by Hogan and Norby.9 However, spinalcord stimulation has been described for other painfulabdominal conditions, such as chronic pancreatitisand parietal axial pain.12-14 Higher level evidence isdifficult to produce in pain medicine, but evaluationof all the mentioned techniques for the growing num-ber of indications is necessary. The cost associatedwith the procedures can be significant, and the rare,but certain, possibility of complications also must beconsidered when making the decision to proceed withinterventional pain management.

ACKNOWLEDGEMENTSSupport: None.Financial Disclosure: The authors declare that they have no

relevant financial interests.

REFERENCES1. Bajwa ZH, Gupta S, Warfield CA, Steinman T. Pain manage-

ment in polycystic kidney disease. Kidney Int. 2001;60(5):1631-

1644.

Am J Kidney Dis. 2012;59(6):858-861

2. Heiwe S, Bjuke M. “An evil heritage”: interview study ofpain and autosomal dominant polycystic kidney disease. PainManage Nurs. 2009;10(3):134-141.

3. Ballantyne JC, Mao J. Opioid therapy for chronic pain.N Engl J Med. 2003;349(20):1943-1953.

4. Gabow PA. Autosomal dominant polycystic kidney disease—more than a renal disease. Am J Kidney Dis. 1990;16(5):403-413.

5. Bajwa ZH, Sial KA, Malik AB, Steinman TI. Pain patterns inpatients with polycystic kidney disease. Kidney Int. 2004;66(4):1561-1569.

6. Kidd BL, Urban LA. Mechanisms of inflammatory pain. Br JAnaesth. 2001;87(1):3-11.

7. Mantyh PW, Clohisy DR, Koltzenberg M, Hunt SP. Molecu-lar mechanisms of cancer pain. Nat Rev Cancer. 2002;2(3):201-209.

8. Lee M, Silverman SM, Hansen H, Patel VB, Manchikanti L.A comprehensive review of opioid-induced hyperalgesia. PainPhysician. 2011;14(2):145-161.

9. Hogan MC, Norby SM. Evaluation and management of painin autosomal dominant polycystic kidney disease. Adv ChronicKidney Dis. 2010;17(3):e1-e16.

10. Shealy CN, Mortimer JT, Reswick JB. Electrical inhibitionof pain by stimulation of the dorsal columns: preliminary clinicalreport. Anesth Analg. 1967;46(4):489-491.

11. Melzack R, Wall PD. Pain mechanisms: a new theory.Science. 1965;150(3699):971-979.

12. Kapural L, Rakic M. Spinal cord stimulation for chronicvisceral pain secondary to chronic non-alcoholic pancreatitis.J Clin Gastroenterol. 2008;42(6):750-751.

13. Kapural L, Nagem H, Tlucek H, Sessler DI. Spinal cordstimulation for chronic visceral abdominal pain. Pain Med. 2010;11(3):347-355.

14. Yakovlev AE, Resch BE, Karasev SA. Treatment of cancer-related chest wall pain using spinal cord stimulation. Am J Hosp

Palliat Care. 2010;27(8):552-556.

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