Treatment of Chronic Chest Wall Pain in a Patient With Loeys-Dietz Syndrome Using Spinal Cord Stimulation
Alexander E. Yakovlev, MD* † , Beth E. Resch, APNP* † , Sergey A. Karasev, MD* †
Objective: Spinal cord stimulation (SCS) has been used with increased frequency for the treatment of intractable pain including chronic chest pain.
Methods: The patient with a history of Loeys-Dietz syndrome who underwent repair of pectus excavatum with subsequent chronic chest wall pain underwent an uneventful SCS trial with percutaneous epidural placement of two temporary eightelectrode leads placed at the level of T3-T4-T5.
Results: After experiencing excellent pain relief over the next two days, the patient was implanted with permanent leads and rechargeable generator four weeks later and reported sustained pain relief at 12-month follow-up visit.
Conclusion: SCS provides an effective, alternative treatment option for select patients with Loeys-Dietz syndrome who underwent repair of pectus excavatum with subsequent chronic chest wall pain who have failed conservative treatment. SCS may provide pain relief with advantages over conservative treatments and more invasive techniques.
Keywords: Electric stimulation, intractable chest wall pain, Loeys-Dietz syndrome, pain, spinal cord stimulation
Conflict of Interest: The authors reported no conflicts of interest.
INTRODUCTION
Loeys-Dietz syndrome (LDS) is an autosomal dominant genetic disorder that affects the connective tissue in the body first observed and described in 2005 by Dr. Bart Loeys and Dr. Hal Dietz at the Johns Hopkins University School of Medicine (1). This genetic syndrome has many features similar to Marfan syndrome, but differs from Marfan syndrome in that it is caused by mutations in the genes
encoding transforming growth factor beta receptor 1 (TGFBR1) or 2 (TGFBR2) (1–3). Diagnosis of LDS is made through use of genetic testing to determine if there is a mutation in TGFBR1 or TGFBR2. Patients with LDS exhibit various medical findings of the cardiovascular, musculoskeletal, and integumentary systems (4). The incidence and prevalence of this disease is unknown. There is no association of increased incidence among any racial, cultural, or gender group (3).
Although the etiology of LDS differs from Marfan syndrome, patients with each of the diseases share the potential for similar diverse presentations and potential causes of pain. For example, patients with either of these diseases may have scoliosis. Compared with patients with idiopathic scoliosis, patients with Marfan syndrome tend to have scoliosis that progresses at a faster rate and is more resistant to bracing; undergo scoliosis surgery complicated by greater blood loss, pseudarthrosis, and additional curvature; and have more frequent occurrences of dural ectasia, which may cause headaches, leg pain, or perineal pain. Protrusio acetabuli may result in hip joint arthritis and may require valgus osteotomy or total hip arthroplasty (5).
MATERIALS AND METHODS
A 31-year-old gentleman with a history of LDS and chronic chest wall pain presented to our clinic for pain management.The patient had musculoskeletal manifestations common in LDS and Marfan syndrome including pectus excavatum, pes planus, increased joint hypermobility, positive wrist and thumb signs bilaterally, a long history of chest wall pain since childhood and two manifestations common only in LDS: bifid uvula and hypertelorism. He had underwent reconstruction of pectus excavatum deformity repair three separate times at the ages of 4, 10, and 21 years, the last being a mesh repair with placement of two pectus metal bars. At the age of 22 years, he developed pneumonia and increased pain thought to be aggravated by the hardware, which was subsequently removed while mesh was left in place. He reported no significant increase in pain following this procedure. Onset of increased pain began four months before presenting to our clinic, without mechanism of injury or aggravating incident and had been off work since that time. He was a non-smoker and had no history of substance abuse. Conservative treatment following surgery included gabapentin, which he did not tolerate due to sedation, TENS unit with minimal relief, intercostal nerve blocks, which made the pain worse, thoracic epidural steroid injections with no benefit, opioid pain medications including hydrocodone and oxycodone, antidepressants, NSAIDS, and Tylenol.The patient had not tried lidocaine patches or Lyrica. He reported his pain worsened with moving, coughing, and sneezing. The patient describes his pain as constant burning and stabbing and rated his pain at the worst a 10 on the visual analog scale (VAS) and at best a 7 on the VAS. His chronic pain medication regimen consisted of oxycodone/APAP 5/500 mg up to 8 per day.
Surgical opinion regarding reexploration and repair was sought by the patient, but not recommended by two different surgeons because of the likelihood that the patient would need further surgery in the future to replace his valves and aorta and he already had four surgeries. He continued to follow with cardiologists, thoracic surgeons, and his primary care providers. When the patient presented to our clinic, we changed his pain medication regimen to long-acting morphine sulfate 30 mg by mouth every 12 hours and immediate release morphine sulfate 15 mg up to twice daily for breakthrough pain. The patient reported improved pain on this medication regimen but reported side-effects including somnolence, irritability, and constipation. We offered the patient spinal cord stimulation (SCS) therapy. The patient had been recommended to see one more thoracic surgeon, who did reexploration and mesh removal. Following this surgery the patient had improved pain and was able to discontinue long-acting and shortacting morphine for four months. Pain then returned to previous quality and intensity without mechanism of injury or aggravating incident. He restarted long-acting and short-acting morphine and was counseled on treatment options including continuing with current treatment or SCS therapy. Pertinent positives in the physical exam of the patient included well-healed sternal incisions and obvious chest wall deformity with severe allodynia over the chest wall from below the clavicles down to the diaphragm and between the mid-clavicular lines bilaterally. The patient elected to pursue SCS therapy.
The patient underwent a successful two-day trial of percutaneous placement of two eight-electrode epidural leads (Medtronic Inc., Minneapolis, MN, USA) after passing a psychological evaluation for an implantable device. Epidural access was gained with C-armguidance at the T11/T12 or T12/L1 intervertebral space with two 14 gauge Tuohy needles after local infiltration of 1% lidocaine. Two eight-electrode standard Octad Leads (Medtronic Inc.) were inserted through 14 gauge Tuohy needles with final lead placement in the posterior epidural space at T3-T4-T5 level. Both leads were connected to atemporary external stimulator via an extension cord. During the two-day SCS trial the patient reported greater than 50% improvement in pain. Four weeks later the patient underwent implantation with permanent leads and Restore Ultra (Medtronic Inc.) rechargeable generator. The postoperative courses were uneventful. The initiation of SCS after implantation was uneventful.
The implanted stimulator was programed using a guarded electrode configuration with a pulse width of 450 microseconds and a rate of 40 Hz. The amplitude use ranged from 3.0 to 3.5 volts. The patient reported that the stimulation covered 100% of his painful area following the initial programing. One month after implant the patient began weaning off long-acting morphine. Three months after implant the patient was no longer using any opioid pain medications. Twelve months after implant the patient continued to report 100% coverage of chest wall pain and continued to use no opioid pain medications.
RESULTS
No complications were reported during the SCS trial, permanent implantation, and postoperative period. At a 12-month follow-up visit, the patient reported significant pain relief (>50% reduction in VAS) with the permanent stimulator. He reported using the SCS 24 hours per day, adjusting stimulation intensity for changes in intensity of pain with good pain relief. He was able to discontinue use of pain medications but did continue on the antidepressant. He also reported other positive outcomes including the ability to return to social and educational activities.
DISCUSSION
Spinal cord stimulation is based on the principles enunciated in the “gate-control theory”of pain proposed by Melzack andWall in 1965 (6), which postulates that analgesia SCS stimulates large-diameter afferent fibers. This stimulation, in effect, “closes the gate” to pain transmission. While the mechanism of action of SCS continues to evolve and numerous theories are being explored, it is thought that SCS blocks the pain by stimulating the dorsal columns, which may inhibit transmission through the pain-conducting spinothalamic tract as well as increase activity in descending antinociceptive pathways (7,8). By placing the SCS electrode array over different segments of the spinal cord, stimulation with resultant analgesia alters the patient’s pain perception. Percutaneous leads in the epidural space or surgical lead placement over the epidural space following a laminectomy procedure are the two methods by which SCS is achieved.
As theories continue to evolve, so does the use of SCS for various chronic pain conditions. Since its first use more than four decades ago when electrodes were placed subdurally over the dorsal columns of the spinal cord, SCS has been further refined, and multiple studies have demonstrated its efficacy in the treatment of intractable, chronic pain with a variety of causes (9,10). SCS has been used to successfully treat chronic pain in patients with failed back syndrome (11,12), ischemic limb pain (13), angina pectoris (14,15), painful peripheral neuropathies (16,17), visceral abdominal pain
syndrome (18,19), chronic non-alcoholic pancreatitis, generalized abdominal pain, abdominal wall neuromas, and post-traumatic splenectomy (20–25).
Spinal cord stimulation provides a safe, effective, and convenient treatment option for patients suffering from chest wall pain. SCS has many advantages over conservative treatments as well as more
invasive techniques. There are no side-effects created by SCS as there are with many medications. There is a high rate of success with the permanent implant due to the fact that a trial is performed during which the patient evaluates the efficacy of the device. The therapy is completely reversible if for some reason therapy becomes contraindicated or is no longer needed. Patient programmers permit patients to control the leve lof stimulation they feel based on their degree of pain. This enables patients to take a more active role in their pain management.
CONCLUSION
We present a patient with LDS and chronic postoperative chest wall pain, which was successfully treated with SCS. This technique may bea safe and effective treatment for patients who have failed to find relief with more conservative measures or who are not appropriate candidates for opioid pain medications, more invasive interventional pain procedures, or surgical procedures based on their
comorbid health conditions. In our opinion, SCS offers a safe and effective treatment method that is completely reversible should a patient lose its pain-alleviating effect. These case studies provide support for SCS as an alternative treatment option for patients with chest wall pain and will hopefully inspire interest in prospective studies comparing SCS with other therapies.These preliminary data from the group of patients with intractable chest wall pain will provide groundwork for potential prospective studies including comparison with other less invasive interventions to control the pain.
Authorship Statement
Alexander E. Yakovlev conducted the study design and data analysis. Sergey Karasev assisted with the study design and data analysis. Beth Resch drafted the manuscript. All authors approved the submitted version of the manuscript.
How to Cite this Article:
Yakovlev A.E., ReschB.E., KarasevS.A. 2010. Treatmentof Chronic Chest Wall Pain in a Patient With Loeys-Dietz Syndrome Using Spinal Cord Stimulation. Neuromodulation 2011; 14: 27–29
REFERENCES
1. Loeys BL, Schwarze U, Holm T et al. Aneurysm syndromes caused by mutations in theTGF-beta receptor. N Engl J Med 2006;355:788–798.
2. LeMaire SA, Pannu H, Tran-Fadulu V, Carter SA, Coselli JS, Milewicz DM. Severe aortic and arterial aneurysms associated with a TGFBR2 mutation. Nat Genet 2007;4:167–171.
3. Loeys BL, Chen J, Neptune ER et al. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2.Nat Genet 2005;37:275–281.
4. Dean JC. Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet 2007;15:724–733.Epub 2007 May 9.
5. Shirley ED, Sponseller PD. Marfan syndrome. J Am Acad Orthop Surg 2009;17:572–581.
6. Melzack RA,Wall PD.Pain mechanisms:a new theory.Science 1965;150:971–979.
7. Stiller CO, Linderoth B, O’Connor W et al. Repeated spinal cord stimulation decreases the extracellular level of gamma-aminobutric acid in periaqueductal grey matter of freely moving rats. Brain Res 1995;699:231–241.
8. Linderoth B, Gazelius B, Franck J, Brodin E. Dorsal column stimulation induced release of serotonin and substance P in the cat dorsal horn. Neurosurgery 1992;31:289–296.
9. Yakovlev AE, Ellias Y. Spinal cord stimulation as a treatment option for intractable neuropathic cancer pain.Clin Med Res 2008;6:103–106.
10. Cameron T. Safety and efficacy of spinal cord stimulation for the treatment of chronic pain: a 20-year literature review.J Neurosurg 2004;100:254–267.
11. Struijk JJ, Holsheimer J, Spincemaille GH, Gielen FL, Hoekema R.Theoretical performance and clinical evaluation of transverse tripolar spinal cord stimulation. IEEE Trans Rehabil Eng 1998;6:277–285.
12. Ohnmeiss DD, Rashbaum RF, Bogdanorffy GM.Prospective outcome evaluation of spinal cord stimulation in patients with intractable leg pain. Spine 1996;21:1344–1350.
13. Ghajar AW, Miles JB. The differential effect of the level of spinal cord stimulation on patients with advanced peripheral vascular disease in the lower limbs.Br J Neurosurg 1996;12:402–408.
14. Hautvast RW, DeJongste MJ, Staal MJ, van Gilst WH, Lie KI. Spinal cord stimulation in chronic intractable angina pectoris: a randomized controlled efficacy study. Am Heart J 1998;136:1114–1120.
15. Mesa J, Yakovlev AE. Treatment of intractable angina pectoris utilizing spinal cord stimulation. Rev Cardiovac Med 2008;9:70–74.
16. Kumar K, Toth C, Nath RK. Spinal cord stimulation for chronic pain in peripheral neuropathy. Surg Neurol 1996;46:363–369.
17. Tesfaye S, Watt J, Benbow SJ, Pang KA, Miles J, McFarlane IA. Electrical spinal-cord stimulation for painful diabetic peripheral neuropathy. Lancet 1996;348:1698–1701.
18. Krames E, Mousad DG. Spinal cord stimulation reverses pain and diarrheal episodes of irritable bowel syndrome: a case report. Neuromodulation 2004;7:82–88.
19. Khan YN, Raza SS, Kahn EA. Application of spinal cord stimulation for the treatment of abdominal visceral pain syndromes: case reports. Neuromodulation 2005;8:14–27.
20. Tiede JM, Ghazi SM, Lamer TJ, Obray JB. The use of spinal cord stimulation in refractory abdominal visceral pain: case reports and literature review. Pain Pract 2006;6:197–202.
21. Ceballos A, Cabezudo L, Bovaira M, Fenollosa P, Moro B. Spinal cord stimulation: a possible therapeutic alternative for chronic mesenteric ischaemia. Pain 2000;87:99–101.
22. Kapur S, Mutagi H, Raphael J.Spinal cord stimulation for relief of abdominal pain in two patients with familial Mediterranean fever.Br J Anaesth 2006;97:866–868.
23. Kapural L, Narouze SN, Janicki TI, Mekhail N.Spinal cord stimulation is an effective treatment for the chronic intractable visceral pelvic pain.Pain Med 2006;7:440–443.
24. Jackson M, Simpson KH. Spinal cordstimulation in a patient with persistent oesophageal pain. Pain 2004;112:406–408.
25. Yakovlev AE, Resch BE. Treatment of intractable abdominal pain patient with Bannayan-Riley-Ruvalcaba syndrome using spinal cord stimulation. WMJ 2009;108:35–38.
COMMENTS
The case report documents SCS-related improvement of chronic pain caused by skeletal deformity in a patient with rare genetic disorder. The main learning point of this case is that severe disabling pain may be relieved by non-destructive procedure even if it persisted for many years.
It is not uncommon for physicians to quote lower success rate for patients with chronic severe pain. As the matter of fact, it was determined that duration of pain inversely correlated with SCS effectiveness. 1 Based on the experience of the authors of this report, it appears that SCS may provide significant and lasting improvement in pain that persisted for 10 years or longer and did not respond to less invasive approaches in the past.
Konstantin V. Slavin, MD
Professor
Neurological Surgery—CS
University of Illinois at Chicago
Chicago, IL, USA
Reference:
1. Kumar K. Epidural spinal cord stimulation for treatment of chronic pain—some predictors of success. A 15-year experience. Surgical Neurology 1998;50:110–21.
***
Patients with Loeys-Dietz syndrome, a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections should undergo complete aortic imaging at initial diagnosis and 6 months thereafter to establish if enlargement is occurring. (ACCF/AHA Guideline-Hiratzka etal).
Patients with Loeys-Dietz syndrome should have yearly magnetic resonance imaging from the cerebrovascular circulation to the pelvis. These patients develop aneurysms of other vessels (53 %).
It was not mentioned if the diagnosis was confirmed by mutational analysis of the TGFBR1 or TGFBR2 genes.
Liong Liem, MD
St. Antonius Hospital
Nieuwegein, The Netherlands
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