Case Report
Treatment of Chronic Chest Wall Pain in a Patient With Loeys-Dietz Syndrome Using Spinal Cord Stimulation

https://doi.org/10.1111/j.1525-1403.2010.00298.xGet rights and content

Objectives

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 eight-electrode 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.

Section snippets

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., 2., 3.). Diagnosis of LDS

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

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

DISCUSSION

Spinal cord stimulation is based on the principles enunciated in the “gate-control theory” of pain proposed by Melzack and Wall 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

CONCLUSION

We present a patient with LDS and chronic postoperative chest wall pain, which was successfully treated with SCS. This technique may be a 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

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., Resch B.E., Karasev S.A. 2010. Treatment of Chronic Chest Wall Pain in a Patient With Loeys-Dietz Syndrome Using Spinal Cord Stimulation. Neuromodulation 2011; 14: 27–29

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