Abstract
Background and Purpose Orofacial pain disorders are relatively uncommon and pose a substantial diagnostic challenge. This case report documents the diagnosis and management of hemifacial pain in a patient who was referred to an interdisciplinary pain medicine unit. The purpose of this case report is twofold. First, it presents complex regional pain syndrome (CRPS) as a potential differential diagnosis in cases of facial pain. Second, it describes the successful adaption of contemporary management approaches for distal-extremity CRPS to treat people with CRPS of the facial region.
Case Description The patient had hemifacial pain and concomitant motor and autonomic symptoms after a small laceration of the eyelid. Extensive specialist medical evaluations were undertaken to exclude an underlying structural pathology. After detailed clinical assessments by a physical therapist, pain physician, and clinical psychologist, a diagnosis of CRPS involving the face was made.
Outcomes The patient's pain was largely unresponsive to pharmacological agents. A modified graded motor imagery program, together with desensitization and discrimination training, was commenced by the physical therapist and clinical psychologist. A positive clinical response was indicated by a decrease in allodynia, normalization of motor control, and regained function in activities of daily living.
Discussion Complex regional pain syndrome is an infrequently reported differential diagnosis that can be considered in patients with persistent facial pain. This case report highlights how careful examination and clinical decision making led to the use of an innovative therapeutic strategy to manage a challenging condition.
Orofacial pain disorders are rare, with an estimated incidence rate of 38.7 per 100,000 person-years.1 Because orofacial pain can herald various musculoskeletal, dental, neurological, vascular, auditory, and ocular disorders, it poses a unique diagnostic challenge for clinicians; meticulous assessment is vital and ideally should be conducted within an interdisciplinary environment.
The differential diagnoses in patients with orofacial pain include trigeminal neuralgia, temporomandibular disorder, cervicogenic headache, and a dental disorder. In the absence of specific pathological findings, a diagnosis of atypical facial pain is most likely to be made. Atypical facial pain is a diagnosis of exclusion and may encompass several pathophysiologically heterogeneous syndromes that are united only by the presence of persistent pain. Unfortunately, research into the pathophysiology and treatment of atypical facial pain is limited.
Complex regional pain syndrome (CRPS) is a rarely made differential diagnosis in cases of persistent facial pain. This condition is characterized by intractable pain, together with multiple sensory and motor phenomena.2 The pathophysiology of CRPS is incompletely understood, but CRPS has been associated with local and systemic proinflammatory immune activity2,3; changes in somatosensory and motor cortex function in the brain4,5 that may, in part, reflect disuse6; and autonomic dysregulation.2 Treatment of CRPS is targeted at these presumed contributory pathophysiological mechanisms and aims to minimize pain and to restore function. Treatment includes pharmacological agents that target pain; functional rehabilitation strategies to improve function and reverse the presumed pathological brain changes; and psychological therapies to address any contributions from impaired mood, psychosocial issues, and problematic behavioral responses.
Although no large-scale studies have specifically investigated facial CRPS, one review of several case reports has been published.7 Management in these case reports focused on invasive or pharmacological monotherapies. Although an interdisciplinary approach, including the use of graded motor imagery (GMI), is recommended for CRPS of the extremities, its use in cases of facial CRPS has never been reported in the literature.
This case report describes the successful use of an interdisciplinary approach in a patient who was diagnosed with facial CRPS. Specifically, we propose that a diagnosis of CRPS should be considered in cases of atypical facial pain and that a rehabilitation program including GMI can be used to help manage this condition. This case report is important because, to our knowledge, it is the first to describe the use of GMI in a case of facial CRPS. This case report also highlights the importance of using a goal-oriented approach in designing a treatment regimen and in evaluating its clinical outcomes.
Patient History and Review of Systems
A 23-year old white woman presented to an emergency department 3 days after sustaining a small laceration of her left eyelid. Her medical history included childhood ear infections, irritable bowel syndrome, one arthroscopic knee procedure, one tooth extraction, and corrective sternal surgery. The patient was diagnosed with a left tear duct infection and provided with oral antibiotics and chloramphenicol eyedrops. Over the next 2 weeks, she developed periorbital bruising and severe pain over the left side of her face. During a medical review, periorbital cellulitis was suggested but was ruled out on clinical grounds. Systemic signs of infection were absent, including normal C-reactive protein levels (<1.0 mg/L).
The patient subsequently presented to the pain medicine unit with a 3-month history of unilateral facial pain secondary to now-resolved local eye trauma and infection. She was assessed by an interdisciplinary team that included a pain physician, a clinical psychologist, and a physical therapist. As part of standard practice at the pain medicine unit, we obtained measures of pain severity, neuropathic pain components, and mood using validated tools. The clinical outcome data are shown in the Table. The patient reported her pain as severe on an 11-point numerical rating scale,8 and her painDETECT questionnaire score indicated a possible neuropathic or central sensitization component to the pain.9 However, the distribution of her pain did not follow clear anatomical or neurological boundaries. The Hospital Anxiety and Depression Scale indicated mild anxiety (score=9) and depression (score=10).10 Psychological assessment identified social phobia arising from physical changes related to the pain condition but did not identify any formal thought or personality disorder. The patient's general and social functioning was severely impaired as a result of the pain; she was not able to wash her face or apply makeup because of tactile allodynia, and she reported a very low ability to function on the 11-point Patient-Specific Functional Scale.11
Clinical Outcomes at Initial Assessment (Baseline), Before Commencement of GMI, and at Dischargea
Only a limited physical assessment was performed because of the patient's fear of her face being touched. She had moderately limited control over left-side facial movements and could not actively open her left eye, which was kept permanently closed throughout the day. She also kept the left side of her face covered with her hair. Examination revealed tactile allodynia, increased skin temperature, swelling, and hyperhidrosis over the left side of the face, extending from the hairline to the jaw. She indicated that her main goals were to obtain pain relief, return to work, minimize medication use, and resume social activities.
Clinical Impression 1
Although the patient had been thoroughly assessed before referral, the team remained concerned that her symptoms could be related to a serious underlying pathology. In the presence of the significant sensory changes, reduced hemifacial motor control, and pain with neuropathic descriptors, we considered differential diagnoses such as trigeminal neuralgia and intracranial tumors (which can cause symptoms such as facial pain, sensory changes, and headaches12) and other ophthalmological and neurological conditions. So that an underlying pathology could be ruled out, the patient was referred for multiple structural imaging studies and to an ear, nose, and throat surgeon, an ophthalmology surgeon, and multiple neurologists. While the patient was undergoing the medical reviews, we reviewed her condition regularly in an attempt to find a pharmacological agent that would reduce her pain.
Examination
The results of the imaging studies of the patient's head and face (computerized tomography, magnetic resonance imaging, positron emission tomography, and bone scanning) were normal, revealing only a normal variant in the osseous structure of the left sphenoid bone. The ear, nose, and throat specialist found the patient to have normal acoustic reflexes, normal tympanography results, intact cranial nerves, and normal otoscopy results. Her hearing was also tested with an audiogram, which revealed normal pure tone with type A tympanometry. The ophthalmologic surgeon found the patient's visual acuity to be 6/7.5 on the right and 6/20 on the left, levator palpebrae muscle function was normal, and funduscopy and slit lamp examination results were normal. During the ophthalmology examination, the patient also showed evidence of ocular inflammation, including a purulent discharge, which was diagnosed as viral conjunctivitis. This acute viral episode resolved quickly, but the previously noted sensory and motor symptoms did not abate. The patient also was assessed by several neurologists, all of whom agreed that there was no evidence of central or peripheral nervous system pathology. During this diagnostic process, multiple analgesic medications were tried, but they did not deliver a sustained clinical benefit.
Clinical Impression 2
At the end of this diagnostic workup, the patient was again reviewed by pain clinic staff. Now, in the presence of ongoing pain for a duration of 7 months and the absence of any indication of structural pathology, we considered the diagnoses of atypical facial pain and CRPS. Both are diagnoses of exclusion and encompass facial pain that does not fit into any other clinical diagnostic criteria.
Examination
During the preceding 4 months, the patient experienced no improvement in her symptoms. She continued to report severe ongoing pain, skin hypersensitivity, intermittent skin color and temperature changes, intermittent swelling, increased sweating, and poor control of her eye on the affected side. She also now reported that simply viewing her face in a mirror increased her level of pain. During this clinical assessment, we noted extreme tactile and conflict allodynia (a painful response to normal light touch and to the threat of imminent touch, respectively),13 erythema, and hyperhidrosis around the left cheek and eyebrow; an inability to actively open her left eye; and reduced movement of the left side of her mouth and face during our interaction and on specific testing of her facial muscles. Her ability to detect sharp or blunt sensation was normal, although painful. She could accurately feel light touch, but approximately 20% of the time, she incorrectly attributed left-side stimulation to the right side of her face. Such somatosensory allochiria or hemispatial neglect has been reported in cases of parietal lobe damage,14 which had been ruled out by the neurologists, and in CRPS.15
Overall, the findings suggested that the patient's sensory function was grossly intact. Interestingly, we also noted that when her left index finger was stroked or pressed, she reported severe pain in the left side of her face (Fig. 1). It is possible that this unusual phenomenon is related to the functional cortical changes that are thought to occur during conditions such as CRPS.4,5 Specifically, these symptoms may suggest a change in the relationship between the juxtaposed representations of the hand and the face in the primary somatosensory cortex. However, we could not confirm this notion because we did not perform functional imaging studies. After this clinical assessment, the patient reported that her pain increased from 7/10 to 8/10 or 8.5/10.
Referred sensation (gray shading) and pain (x) in the face when the patient's left index finger was stroked or pressed. (Top) Pattern of referral before graded motor imagery was commenced. (Bottom) Pattern of referral at discharge.
Clinical Impression 3
On the basis of our examination findings and the absence of demonstrable nerve damage, we made a diagnosis of CRPS type 1 involving the left side of the face. According to the most commonly accepted diagnostic criteria, CRPS is diagnosed in the presence of persistent pain together with symptoms from at least 3 of the following 4 categories: sensory (hyperesthesia or allodynia), vasomotor (temperature or skin color asymmetry), sudomotor/edema (edema or abnormal sweating), and motor/trophic (reduced range of motion, weakness, tremor, dystonia, or hair/skin/nail changes).16 The patient also must demonstrate at least one of these signs at the time of assessment.16 On the basis of the extensive clinical workup, the extreme scarcity of published cases of facial CRPS, and our innovative and successful approach to therapy, we considered reporting this case to be worthwhile.
Intervention
The treatment and the measurement of its success were designed on the basis of the patient's main issues: severe pain, anxiety, and limited function. Our main treatment outcomes were improvements in the patient's pain scores (numerical rating scale), mood (Depression Anxiety Stress Scales), and function (Patient-Specific Functional Scale and subjective self-reported social functioning and vocational functioning). Because it has been suggested that the restoration of normal somatosensory function parallels a clinical improvement in CRPS,17 we also used the relationship between index finger pressure and facial pain as an outcome measure; we predicted that a decrease in the referred sensation would parallel a reduction in pain and an improvement in function. Finally, we used exercise-specific improvements as an outcome measure (eg, intensity and symptom response).
Because of the patient's severe pain and mechanical allodynia, she was not able to engage in a traditional exercise or desensitization program. Therefore, we instituted a 3-pronged therapeutic approach: first, we tried to reduce her pain with medication; second, we tried to help her deal with anxiety by involving the clinical psychologist and by educating the patient about her condition and its treatment; and third, we decided to implement a functionally oriented exercise program that avoided exacerbating her symptoms. The patient's condition was reviewed approximately every 1 or 2 weeks, alternating between the clinical psychologist and the physical therapist.
To address the patient's pain and to facilitate her rehabilitation, we tried dothiepin hydrochloride (25 mg) daily; this medication temporarily improved her pain, but this gain was not maintained, even with a subsequent dosage increase to 150 mg. Next, the following medications were tried, without success: pregabalin, carbamazepine, indomethacin, and tramadol. The patient received no benefit from complementary therapies, including naturopathy. To facilitate an interdisciplinary and active approach to her rehabilitation, we enrolled the patient in an interdisciplinary pain management program that followed the STEPS (Self-Training Educative Pain Sessions) model, which was developed at our pain medicine unit.18 The program provides education about pain and its management in accordance with the “explain pain” approach.19 In brief, patients attend a 2-day interactive workshop and learn about the neurophysiology of pain and its relationship to their symptoms and rehabilitation. Through this program, we aimed to improve the patient's understanding of her condition and to encourage her to become a motivated and active participant in her treatment.
To address the patient's pain and anxiety and to help improve her function, we also instituted complementary interventions by a clinical psychologist and a physical therapist. Because the patient was diagnosed with CRPS, we broadly formulated these interventions on the basis of the GMI approach.20 Graded motor imagery has shown some promise in the management of CRPS of the extremities,21,22 but its use for facial pain has not been reported.
Graded motor imagery involves graded exposure to movement. First, patients perform implicit motor imagery by making left and right judgments of pictured body parts (eg, “Is this a left or a right hand?”).23,24 Second, they perform explicit motor imagery (imagined movements of the affected body part). Third, they perform movements of both limbs while watching the reflection of their healthy limb in a mirror (a version of “mirror therapy”).25 The Graded Motor Imagery Handbook26 provides a full description and discussion of GMI.
We modified the GMI protocol for use with facial pain. For the first stage, the patient used photographs in magazines and an in-house computer program to identify whether the left side or the right side of the face was more visible in a series of photographs. She was instructed to do this for about 5 minutes, as close to hourly as possible. During this stage, the clinical psychologist reviewed the patient's condition every 3 or 4 weeks to teach her relaxation-based responses to pain and to use graded-exposure techniques to address her social phobia; the patient was provided with substantive homework tasks to practice these skills. The physical therapist reviewed the patient's condition 1 or 2 times weekly to assess progress and modify the program when appropriate targets had been achieved.
Initially, the patient's accuracy on the computer-based side recognition task was 65%, and the exercise caused severe pain that prevented her from continuing the program. Because the patient had an extremely low tolerance to physical touch and movement, she was not able to even engage in the first stage of the GMI program. In an attempt to address this problem, the clinical psychologist theorized that the addition of hypnosis might help the patient control her response to the program. The patient commenced relaxation training and a desensitization program27 under hypnosis; this training was aimed at reducing pain-related fear and involved the visualization of graduated movement and touch in the affected area. Interestingly, when—under hypnosis—the patient imagined viewing herself in a mirror, she reported a complete absence of the left side of her face. This finding appears to be consistent with the perceptual neglect–like symptoms that have been reported in CRPS.2,28 After about 2 weeks of the desensitization program, the patient was able to re-engage in the first stage of the traditional GMI program and tactile desensitization training, which involved gradual exposure to skin stimuli with a cotton swab. She reported a generally high level of adherence to the set tasks.
After 4 weeks, the patient's accuracy on the left/right judgment task improved to 100%, she reported little pain after the sessions, and she progressed to the second stage of the GMI program. In this stage, the patient imagined moving the affected area. She was asked to mentally execute specific facial expressions that were presented in digital photos. In the third (and final) stage, the patient used a mirror while exercising the unaffected side to create the illusion of normal movement on the affected side. The arrangement used is shown in Figure 2. After the completion of these stages, the patient was moved to a standard rehabilitation program with the aim of improving function and attaining personal goals. Specifically, we provided her with a series of exercises for her facial muscles and advised her to continue using skills such as desensitization, relaxation techniques, and activity pacing.
Mirror setup for the “mirror therapy” component of the graded motor imagery program. Two mirrors were set up at approximately a right angle to each other. The patient placed the affected side of her face outside the field of view of the mirrors. Small adjustments of the angle of the mirrors allowed the unaffected side to be reflected as a whole face.
Outcome
The patient successfully progressed through the GMI program and sensory discrimination training.28 We believe that an improved understanding of pain mechanisms, modification of the exercise in a way that the patient found interesting, and a goal-oriented approach were particularly important factors underlying her high level of adherence to the intensive program schedule. After 1 month of the program (12 months since the injury), the patient reported improved sleep and increased confidence in exposing the left side of her face in social situations. After 4 months of therapy (15 months since the injury), she no longer met the criteria for a diagnosis of CRPS. Her pain and function were greatly improved; her facial appearance was normal (absence of swelling and hyperhidrosis); her sensitivity to touch was nearly normal; stimulation of the hand no longer exacerbated her facial pain; she demonstrated spontaneous eye opening of approximately 5 mm; and her social phobia was improved. Interestingly, even though she reported an overall improvement in her pain and a dramatic reduction in allodynia, her pain ratings on the numerical rating scale did not change significantly over the course of treatment (Table). However, on the 10-point Patient-Specific Functional Scale, she indicated that her ability to engage in important tasks was almost normal. Furthermore, she was no longer taking any medication. These ostensibly incongruent outcomes highlight the complex ways in which people assess their pain and the importance of using more than one outcome measure.
The patient was discharged with advice to continue the program independently and invited to return for a consultation, if needed. We contacted the patient approximately 12 months after she had commenced therapy; she reported ongoing pain but thought that it had continued to diminish. She had regained almost normal eyelid and ocular function, although she still found that her eyelid drooped when she was fatigued. She did not take medications or use any health care services, and her social functioning was normal.
Discussion
Pain in the face that does not accompany a clear organic pathology is usually diagnosed as atypical facial pain and is often resistant to treatment.29 In this case report, we describe how CRPS is an important differential diagnosis when patients meet the requisite diagnostic criteria. Furthermore, we propose that the current best-practice management approaches for the treatment of CRPS can be tried in cases of facial CRPS. Finally, we describe a novel hypnosis-assisted imagined sensorimotor rehabilitation stage of GMI. To our knowledge, this approach has never been reported, and we believe that it can be an important therapeutic strategy for clinicians working with patients who have CRPS and whose symptoms are so severe that they cannot tolerate even the side recognition task (first stage) or any tactile stimulation. This case report is one of few published reports of facial CRPS and, to our knowledge, is the first to describe its treatment with an interdisciplinary approach based on the GMI paradigm.
On the basis of this case, we make several recommendations. First, a diagnosis of CRPS should be considered in patients with atypical symptom clusters that meet the diagnostic criteria, regardless of the bodily location of those signs and symptoms. In this case, making the diagnosis of CRPS led us to try the GMI approach with a patient who was not otherwise able to engage in any form of exercise. However, we also caution clinicians that for structurally complex bodily regions such as the face, the diagnostic process needs to include assessments by an interdisciplinary specialist team, even though the time spent is significant. In this case, had we considered the diagnosis of CRPS earlier, our patient might have achieved greater and earlier gains.
Second, the significant time investment required as part of the GMI approach demands a committed team and a highly motivated patient, regardless of the bodily location. Patients with complex problems surrounding their chronic pain require treatment by an interdisciplinary team. A lack of proper and thorough assessment and treatment by the appropriate specialists may prevent the identification of important clinical variables and, in turn, slow progress. In this case, the interdisciplinary approach directly resulted in the development of the treatment strategy, including a novel stage of GMI, which we believe was instrumental in helping the patient progress.
Third, we recommend that clinical outcomes include symptoms and function-oriented targets that are in agreement with the patient's goals. In this case, the patient achieved many of her clinical goals, including reduced medication intake and improved physical function, mood, and symptoms. However, although the patient reported significant reductions in pain and improvements in sensitivity and satisfaction with the therapeutic gains after treatment, her objective pain scores did not change. That lack of pain relief is not altogether surprising, considering the lack of strong evidence of analgesia with drug or nondrug treatments of CRPS.30 The lack of reduction in pain is relevant to questions about the mechanisms of treatment effects. It is possible that we simply improved the patient's confidence in undertaking tasks despite pain.
Finally, we took great care to document assessment, treatment, and response; therefore, we have confidence in our data. However, we acknowledge that a single case report does not constitute evidence of treatment efficacy; we could not exclude natural history, even though the patient's condition was chronic and had been extensively treated, nor could we identify any nontreatment factors that might have contributed to the positive response. Our hope is that this case report triggers further empirical exploration of complex clinical presentations and facilitates collaborative clinical reasoning among practitioners.
Footnotes
Dr Parkitny, Dr Wand, Mr Graham, and Dr Quintner provided concept/idea/project design. All authors provided writing. Dr Parkitny, Dr Wand, and Mr Graham provided data collection. Dr Parkitny and Dr Wand provided data analysis. Dr Parkitny, Mr Graham, and Dr Quintner provided project management, patients, and facilities/equipment. Dr Parkitny and Dr Wand provided consultation (including review of manuscript before submission).
Dr Parkitny was supported by an International Association for the Study of Pain Fellowship, funded by the Scan|Design Foundation by Inger and Jens Bruun. Dr Moseley was supported by National Health and Medical Research Council Fellowship 1045322.
- Received April 5, 2015.
- Accepted November 10, 2015.
- © 2016 American Physical Therapy Association
References
Issue highlights
