06 May 2023: Articles
A 60-Year-Old Man with a 34-Year History of Chronic Exertional Compartment Syndrome and 3 Previous Surgical Fasciotomies, Successfully Treated with Injection of Botulinum Toxin
Unusual clinical course, Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease, Clinical situation which can not be reproduced for ethical reasonsYohan Sumathipala 1ABCDEF*, Shivani Khakhkhar1ABCDEF, Michael J. Suer 1ABCDE
Am J Case Rep 2023; 24:e939431
BACKGROUND: Patients with post-fasciotomy CECS recurrence can experience significant mobility issues at baseline that limit independent living. For these patients, a repeat fasciotomy is not ideal because they are older and post-surgical scar tissue will make the fasciotomy technically challenging. Therefore, post-fasciotomy patients with CECS recurrence require new, non-surgical treatment options. Recent studies show botulinum toxin injections can be effective for the initial management of chronic exertional compartment syndrome (CECS) prior to surgery, especially in young patients primarily experiencing pain on exertion with minimal lower-extremity symptoms at rest. However, the ability to treat CECS recurrence status after fasciotomy with botulinum toxin injections of the legs has not been studied.
CASE REPORT: We present the first case where botulinum toxin was applied to this patient population. Our patient was a 60-year-old man with a 34-year history of CECS who, 8 years after his third bilateral fasciotomy, progressively developed rest pain in his calves bilaterally, paresthesias, and difficulties when walking or descending stairs, with multiple near-falls due to his toes catching on stair steps. OnabotulinumtoxinA (BTX-A) injections into the posterior and lateral compartments resolved baseline symptoms: within 2 weeks, he was able to walk, negotiate stairs symptom-free, and enjoy an overseas vacation without complications.
CONCLUSIONS: Symptoms related to recurrent CECS status after multiple fasciotomies can successfully be treated with BTX-A injections. Our patient’s baseline mobility issues resolved within 2 weeks after the injection and remained that way for over 31 months. However, his exertional symptoms and rest pain recurred at 9 months, suggesting that BTX-A injections are not completely curative.
Keywords: Botulinum Toxins, Type A, Chronic Exertional Compartment Syndrome, chronic pain, fasciotomy, Activities of Daily Living
Chronic compartment syndrome, often referred to as chronic exertional compartment syndrome (CECS), is a condition of pain following exercises of repeated motions. It usually presents as anterior leg pain in runners or in the arms of baseball pitchers. CECS is a diagnosis of exclusion, although intracompartmental pressure monitoring is the criterion standard [1–4]. Initial treatment is conservative with exercise reduction, physical therapy, limb taping, and gait retraining . Classically, surgical decompression via fasciotomy is the next step if conservative measures fail; however, recent studies show botulinum toxin may be an effective alternative to surgery in this clinical context. Putatively regarded as definitive, surgery is rarely completely curative in practice, as CECS recurs in 50–80% of such patients in after a fasciotomy [6,7].
Patients with post-fasciotomy CECS recurrence have not been well-studied historically. A repeat fasciotomy is not ideal because the operation will be technically complicated, due to scar formation following the first operation; furthermore, they will have aged and potentially accrued more comorbidities after the initial operation [8,9]. If conservative measures fail, their post-fasciotomy recurrent CECS progresses until they develop significant baseline debilitation that warrants surgical intervention .
The absence of an evidence-based, non-surgical interventional therapy for post-fasciotomy CECS recurrence is responsible for the suffering and disease progression in these patients. Until 2 recent studies showed that intramuscular botulinum toxin injections provide symptom relief in surgically naïve patients, there has not been an evidence-based non-surgical therapy for CECS [10,11]. Both of those cohort studies focused only on the initial interventional CECS patients, who were all surgically naïve.
In this case report, we present the first known case of botulinum toxin being used to treat a patient with post-fasciotomy recurrent CECS. Our patient was a 60-year-old man with a 34-year history of CECS with 3 previous surgical fasciotomies (all were 4-compartment fasciotomies with fasciectomies) who presented with posterior calf pain (CECS typically affects the anterior and lateral leg compartments) and mobility issues that impacted his activities of daily living. Just 2 weeks after an intramuscular injection with onabotulinumtoxinA (BTX-A), his baseline mobility issues resolved and at 31-month follow-up he reported he was able to complete his activities of daily living without issues.
A 60-year-old male former triathlete presented in 2020 to our clinic with a 2-month history of bilateral posterolateral calf pain at rest refractory to conservative measures, tingling, and a cold sensation in both feet. He also had a history of multiple near-falls when descending stairs, which in conjunction with his pain, limited his ability to walk. His symptoms occurred in the setting of a 34-year history of CECS, 33 years after his first fasciotomy.
The patient was first diagnosed with CECS in 1986, when he presented with bilateral calf pain at rest that markedly worsened on exertion and returned to baseline within 10–20 minutes of rest. His symptoms did not improve with rest, ice, and non-steroidal anti-inflammatories. His presentation was most consistent with CECS, and was further supported by a negative workup for competing etiologies, including multiple sclerosis and peripheral artery disease.
After minimally-invasive management strategies – dry needle nerve denervation and intramuscular steroid injections – failed to improve symptoms, a bilateral 4-compartment open fasciotomy was performed in 1987 . As a post-op rehabilitation plan was not yet standard of care, he resumed his usual running regimen just 10 days later.
Six years later, his exertional symptoms returned (1993) and were attributed to post-fasciotomy CECS recurrence after intracompartmental pressures were found to be >15 mmHg at rest, per the Pedowitz criteria . A repeat bilateral 4-compartment fasciotomy was performed but was complicated by intraoperative bleeding with hematoma formation that required each leg to be operated on separately. Following a post-operative rehabilitation plan, he returned to recreational running and was asymptomatic until his symptoms returned in 2003. Over the next 8 years, they progressed to lifestyle-limiting resting pain. Surgery was considered on multiple occasions but deferred due to his hematoma complications and expected degree of post-surgical scarring. When his resting pain became intolerable in 2010, intracompartmental pressures were again found to be elevated (>15 mmHg at rest in all compartments bilaterally) and confirmed post-fasciotomy recurrent CECS. In early 2011, he underwent bilateral 4-compartment fasciotomy with bilateral partial fasciectomies in the anterolateral and posteromedial planes, superficial peroneal neurolysis, and release of the soleus from the tibia. His surgery and post-surgical rehabilitation were uncomplicated, and he returned to his normal activities.
His chronic exertional pain recurred in 2019 and progressed over the year. He presented to our clinic in 2020 with tingling in the anterior leg bilaterally, difficulty walking, and a history of multiple near-falls when descending stairs due to his toes catching the steps. On examination at rest, he had a non-antalgic gait with bilaterally soft calf compartments and palpable posterior tibialis pulses. Sensation to light touch was intact in both of his lower extremities. He had normal, 5/5 strength throughout both legs; however, he had hypertrophy of the gastrocnemius and experienced posterior calf cramping upon plantar flexion.
Given the exertional nature of his symptoms with now-persistent pain at rest, we surmised that he had progressive recurrent CECS, for which he was interested in non-surgical intervention [10,11,13–16]. A total of 150 units of BTX-A in a concentration of 100 units: 1 mL was injected into each leg, in 50-unit aliquots into the medial gastrocnemius, lateral gastrocnemius, and lateral compartment.
At his 9-month virtual follow-up with us, he reported that he was symptom-free and had no mobility issues during his vacation that took place 2 weeks after the injections. Furthermore, he reported that he continued to be symptom-free at rest and had no issues descending stairs during this 9-month interval. He also noted that he would be pain-free at a walking pace of about 2 miles per hour but would have posterior calf pain when walking at a faster pace of 4 miles per hour.
At a 31-month virtual appointment with an orthopedic surgeon in December 2022, he was still able to comfortably complete his activities of daily living. However, he noted a recurrence of his rest pain and decrease in the range of motion in both ankles that began about 9 months after the botulinum toxin injections. A tentative surgical plan was made for a gastrocnemius muscle release either endoscopically or as part of a larger repeat fasciotomy, pending an in-person evaluation in early 2023.
For post-fasciotomy CECS recurrence, onabotulinumtoxinA (BTX-A) is an effective treatment, as our case shows. Our patient’s presentation of posterior calf pain and mobility issues impacting activities of daily living are both atypical presentations of CECS but highlight the many ways this condition can present. The resolution of his mobility issues within 2 weeks, without the need for a complex post-procedural rehabilitation plan, shows that BTX-A injections have a rapid onset to symptom relief and can be an excellent treatment option for patients in need of immediate relief of debilitating symptoms. Furthermore, his mobility issues remain resolved 31 months after the injection, showing that this treatment modality provides long-term benefit of debilitating symptoms. However, the return of his rest and exertional pain at 9 months shows that BTX-A injections are not a permanent solution for recurrent CECS. Whether additional injections at the 9-month interval or a repeat fasciotomy would provide this patient greater benefit remains an open question, likely best answered by randomized clinical trials.
The approach to of CECS has changed little since the condition was first described in 1945 . Surgical decompression remains the criterion standard treatment upon failing conservative measures, consisting of activity restrictions, physical therapy, and gait retraining. Putatively, surgery is regarded as definitive; however, the data on long-term outcomes is limited. There are just 10 longitudinal trials tracking patients for over 1 year: 2 trials have 2-year follow-up [18,19], 5 have 4-year [20–24], 2 have 5-year [25,26], and 1 has 10-year . Across all those studies, the success rate of controlling all rest and exertional symptoms varies from 50% to 80% [6,7]. Surgery, therefore, is far from definitive management over a patient’s lifespan, particularly when patients are first diagnosed and operated on as young adults. Non-surgical alternatives are needed.
Botulinum toxin is one such treatment, and there is a limited but growing body of evidence to support its efficacy and safety. A PubMed search for “compartment syndrome botulinum toxin,” yielded 29 publications, only 5 of which were about chronic exertional compartment syndrome. Two were cohort studies which each had 16 patients [10,11], 5 were case reports of which 4 described leg injections [13–16], and 1 described a hand injection in a post-fasciotomy patient . Collectively, these 5 papers described a total of 37 patients, of whom 33 had leg symptoms and 4 had hand symptoms. Except for the case report of hand injections , the other 36 patients were surgically naïve, and botulinum toxin injections were used for the initial management of their symptoms. The primary symptoms for all 36 patients were exertional with negligible symptoms when not exercising. No patient was reported to have had mobility issues with activities of daily living, only upon exercising.
In all of these studies of botulinum toxin for the initial treatment of CECS, all patients began to experience some symptom relief within 1 month. Some patients – particularly younger patients with mild symptoms—had no recurrence of symptoms at 6 months . One case report showed complete symptom relief for up to 14 months – the longest follow-up interval recorded . However, in patients who experienced recurrent symptoms, recurrence began to occur about 4–6 months after the injection . Our patient’s experience – relief of his most debilitating symptoms within 2 weeks and return of exertional and resting pain symptoms at 9 months after injection – is similar to that reported in the literature. However, his symptoms of posterior calf pain and mobility issues at baseline make him truly unique, as none of the 36 patients previously studied had those symptoms. His positive response to BTX-A injections shows that this treatment modality suggests that BTX-A addresses the underlying pathophysiology of CECS, although the exact mechanism by which botulinum toxin alleviates CECS symptoms is not completely known .
To the best of our knowledge, this case report is the first to show the effectiveness of BTX-A in recurrent CECS of the legs many years after surgical treatment and may serve as a reference point for future physicians as a safe, alternative treatment between conservative and surgical intervention. Future work is needed to define optimal dosing strategies and timing of injections for a wider range of patients. While we caution against generalizing the results of a single case, our case highlights the potential for a safe, non-surgical treatment with long-term relief, warranting further investigation.
Currently, there are no published randomized controlled trials on the outcomes of botulinum toxin injections for CECS treatment. In the interim, while our institute is currently running such a trial with open enrollment, our case extends the literature by showing that BTX-A injections are therapeutic for patients with post-fasciotomy recurrent CECS.
For patients with post-fasciotomy recurrent CECS, botulinum toxin can treat debilitating mobility issues that impact a patient’s ability to perform activities of daily living. Symptom relief began just 2 weeks after the injection and persisted for over 31 months.
1.. Pedowitz RA, Hargens AR, Mubarak SJ, Modified criteria for the objective diagnosis of chronic compartment syndrome of the leg: Am J Sports Med, 1990; 18; 35-40
2.. Chandwani D, Varacallo M, Exertional Compartment Syndrome: StatPearls [Internet], 2022, Treasure Island (FL), StatPearls Publishing [cited 2022 Nov 15]. Available from:http://www.ncbi.nlm.nih.gov/books/NBK544284/
3.. Lindorsson S, Zhang Q, Brisby H, Rennerfelt K, Significantly lower intramuscular pressure in the posterior and lateral compartments compared with the anterior compartment suggests alterations of the diagnostic criteria for chronic exertional compartment syndrome in the lower leg: Knee Surg Sports Traumatol Arthrosc, 2021; 29(4); 1332-39
4.. Aweid O, Del Buono A, Malliaras P, Systematic review and recommendations for intracompartmental pressure monitoring in diagnosing chronic exertional compartment syndrome of the leg: Clin J Sport Med, 2012; 22(4); 356-70
5.. Buerba RA, Fretes NF, Devana SK, Chronic exertional compartment syndrome: Current management strategies: Open Access J Sports Med, 2019; 10; 71-79
6.. Ding A, Machin M, Onida S, A systematic review of fasciotomy in chronic exertional compartment syndrome: J Vasc Surg, 2020; 72; 1802-12
7.. Vogels S, Ritchie ED, van Dongen TTCF, Systematic review of outcome parameters following treatment of chronic exertional compartment syndrome in the lower leg: Scand J Med Sci Sports, 2020; 30; 1827-45
8.. Waterman CBR, Laughlin CM, Kilcoyne CK, Surgical treatment of chronic exertional compartment syndrome of the leg: Failure rates and postoperative disability in an active patient population: J Bone Joint Surg, 2013; 95; 592-96
9.. Gatenby G, Haysom S, Twaddle B, Functional outcomes after the surgical management of isolated anterolateral leg chronic exertional compartment syndrome: Orthop J Sports Med, 2017; 5 2325967117737020
10.. Isner-Horobeti M-E, Dufour SP, Blaes C, Intramuscular pressure before and after botulinum toxin in chronic exertional compartment syndrome of the leg: A preliminary study: Am J Sports Med, 2013; 41; 2558-66
11.. Charvin M, Orta C, Davy L, Botulinum toxin a for chronic exertional compartment syndrome: A retrospective study of 16 upper- and lower-limb cases: Clin J Sport Med, 2022; 32; e436-e40
12.. Detmer DE, Sharpe K, Sufit RL, Chronic compartment syndrome: Diagnosis, management, and outcomes: Am J Sports Med, 1985; 13; 162-70
13.. Baria MR, Sellon JL, Botulinum toxin for chronic exertional compartment syndrome: A case report with 14 month follow-up: Clin J Sport Med, 2016; 26; e111-e13
14.. Hutto WM, Schroeder PB, Leggit JC, Botulinum Toxin as a novel treatment for chronic exertional compartment syndrome in the U.S: Military. Mil Med, 2019; 184; e458-e61
15.. Berrigan WA, Wickstrom J, Farrell M, Botulinum toxin a for chronic exertional compartment syndrome evaluated with shear wave elastography: A case report: Clin J Sport Med, 2022; 32; e178-e80
16.. Jensen M, Lystrup RM, Jonas CE, Chronic exertional compartment syndrome treated with botulinum toxin-A yielding 36-month total symptom relief: A case Report: Mil Med, 2021 [Online ahead of print]
17.. Horn CE, Acute ischaemia of the anterior tibial muscle and the long extensor muscles of the toes: J Bone Joint Surg, 1945; 27; 615-22
18.. McCallum JR, Cook JB, Hines AC, Return to duty after elective fasciotomy for chronic exertional compartment syndrome: Foot Ankle Int, 2014; 35; 871-75
19.. Orlin JR, Øen J, Andersen JR, Changes in leg pain after bilateral fasciotomy to treat chronic compartment syndrome: A case series study: J Orthop Surg Res, 2013; 8; 6
20.. Slimmon D, Bennell K, Brukner P, Long-term outcome of fasciotomy with partial fasciectomy for chronic exertional compartment syndrome of the lower leg: Am J Sports Med, 2002; 30; 581-88
21.. Pasic N, Bryant D, Willits K, Assessing outcomes in individuals undergoing fasciotomy for chronic exertional compartment syndrome of the leg: Arthroscopy, 2015; 31; 707-13.e5
22.. Drexler M, Rutenberg TF, Rozen N, Single minimal incision fasciotomy for the treatment of chronic exertional compartment syndrome: Outcomes and complications: Arch Orthop Trauma Surg, 2017; 137; 73-79
23.. van Zantvoort APM, Setz MJM, Hoogeveen AR, Common peroneal nerve entrapment in the differential diagnosis of chronic exertional compartment syndrome of the lateral lower leg: A report of 5 cases: Orthop J Sports Med, 2018; 6 2325967118787761
24.. Barrera-Ochoa S, Haddad S, Correa-Vázquez E, Surgical decompression of exertional compartment syndrome of the forearm in professional motorcycling racers: Comparative long-term results of wide-open versus mini-open fasciotomy: Clin J Sport Med, 2016; 26; 108-14
25.. Packer JD, Day MS, Nguyen JT, Functional outcomes and patient satisfaction after fasciotomy for chronic exertional compartment syndrome: Am J Sports Med, 2013; 41; 430-36
26.. Gondolini G, Schiavi P, Pogliacomi F, Long-term outcome of mini-open surgical decompression for chronic exertional compartment syndrome of the forearm in professional motorcycling riders: Clin J Sport Med, 2019; 29; 476-81
27.. Almdahl SM, Samdal F, Fasciotomy for chronic compartment syndrome: Acta Orthop Scand, 1989; 60; 210-11
28.. Orta C, Petit J, Gremeaux V, Chronic exertional compartment syndrome in hands successfully treated with botulinum toxin-A: A case: Ann Phys Rehabil Med, 2018; 61; 183-85
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