Facial Pain and Temporomandibular Joint Dysfunction Secondary to Acromegaly: Treatment with Manual Therapy, Neuromuscular Re-education - A Case Report

Abstract

Background and Purpose:

Acromegaly is a disorder typically caused by a benign pituitary adenoma resulting in hypersecretion of growth hormone. Common sequelae, including musculoskeletal changes and arthropathies can result in facial pain and temporomandibular disorder (TMD) that persist beyond primary treatment. Due to the unique etiology of facial pain and TMD in cases of acromegaly, the generalizability of established physical therapy (PT) literature for treatment of TMD cannot be assumed. The purpose of this case report was to illustrate an example of multimodal PT as a treatment strategy for facial pain and TMD secondary to acromegaly following treatment for benign pituitary macroadenoma.

Case Description:

48-year-old male patient with history significant for benign pituitary macroadenoma, presented with facial pain and TMD secondary to acromegaly. Patient-reported outcomes of pain, function, and quality of life were assessed utilizing the Gothenburg Trismus Questionnaire (GTQ) at baseline, eighth PT visit, and eight weeks following course of PT. Quantity and quality of jaw mobility were assessed at baseline and post-intervention using standard goniometric measurements and observation. A total of nine PT sessions were delivered over three months consisting of manual therapy, relaxation techniques, neuromuscular re-education, and therapeutic exercise.

Outcomes:

After eight PT visits, patient’s GTQ score improved from 81% to 67.6%, with a corresponding decrease in pain and improved symmetry of jaw mobility. Eight weeks following last PT visit, patient’s GTQ score further decreased from 67.6% to 61.3%.

Discussion:

Conservative management through multimodal PT may be effective in managing facial pain and TMD secondary to acromegaly following treatment for benign pituitary macroadenoma.

Background and Purpose

Acromegaly is a rare acquired disorder that usually begins during the third to fifth decades of life, and is caused by an adenoma of the pituitary gland in over 98% of cases.^1,2–4 Although pituitary adenomas are benign tumors, they can result in major health problems due to their proximity to other brain structures as well as their ability to alter systemic hormone levels.⁵ Most pituitary adenomas originate on the anterior aspect of the pituitary gland which is the area responsible for releasing growth hormone (GH) among other systemic hormones. The growth of a pituitary adenoma in this location often results in hypersecretion of GH leading to the development of acromegaly.⁵

Resulting from hypersecretion of GH and the subsequent impact on other systemic hormones, acromegaly is a condition with potential for multisystem morbidities, including diabetes mellitus, hypertension, hypertrophic cardiomyopathy, arthropathies, and bone/soft tissue overgrowth.^3,6 Musculoskeletal changes and arthropathies are common in cases of acromegaly secondary to altered hormone levels resulting in cartilage hypertrophy, tendon laxity, and osteophyte development, leading to mechanical joint damage.⁶ Acromegaly often causes disproportionate growth of the jaw and facial bones resulting in mandibular overgrowth, maxillary widening, tooth separation and jaw malocclusion.^1,7,8

Excessive growth may cease following surgical removal of the pituitary tumor and subsequent regulation of GH, but bony and arthropathic changes may persist resulting in facial pain, temporomandibular disorder (TMD), and increased disability.^1,4 Joint complaints have been shown to persist in as many as 77% of patients with biochemical remission of acromegaly.⁹

The current guidelines for the treatment of acromegaly, established in 2011 by the American Association of Clinical Endocrinologists, recommend pituitary surgery for patients who are appropriate candidates in order to optimize tumor burden and associated biochemical effects.⁶ The guidelines further recommend that arthropathies persisting following optimization of GH levels should be managed aggressively with physical therapy (PT), systemic or intra-articular anti-inflammatory medications, and consideration of joint replacement.⁶ The authors of the guidelines indicate that this recommendation regarding management of arthropathies is classified as a Grade C recommendation. By definition, a Grade C recommendation is based on low-level evidence, often with inconsistent findings that assert neither risk nor benefit of the interventions. Furthermore, this recommendation does not specifically address management of TMD or facial pain.

A literature search performed in PubMed using search terms “acromegaly” and the subsequent management of associated comorbidities, revealed a paucity of information regarding conservative management of facial pain and TMD. Current literature regarding treatment of facial pain and TMD secondary to acromegaly is focused only on surgical intervention. There is substantial literature regarding PT management of TMD, however not specific to the diagnosis of acromegaly. Due to the unique pathophysiology and resultant musculoskeletal changes that lead to facial pain and TMD in cases of acromegaly, the generalizability of established PT literature for treatment of TMD cannot be assumed.

The purpose of this case report was to present an example of multimodal PT as a treatment strategy for a patient with facial pain and TMD secondary to acromegaly following treatment for benign pituitary macroadenoma.

Case Description

The patient was a male who, at age 45, experienced progressive symptoms of acromegaly including enlarged feet, fingers, noticeable overbite, and sleep apnea. An MRI at that time revealed a benign pituitary macroadenoma. Further testing revealed increased systemic levels of GH. The patient’s past medical history was significant for prolactinoma, depression, and anxiety.

A transsphenoidal resection of the macroadenoma was performed to remove the tumor and to optimize systemic levels of GH. Post-operatively, patient was found to have residual tumor with ongoing GH excess, and subsequently underwent a second transsphenoidal resection. The patient’s GH levels improved, although they remained elevated, for which he was started on Sandostatin, a hormone therapy that mimics a natural chemical found in the body called somatostatin. Somatostatin is produced by the hypothalamus and stops secretion of GH from the pituitary gland.¹⁰ The patient demonstrated good response to the medication.

Following the two resections and eventual reduction in GH levels, the patient reported pain at bilateral temporomandibular joints (TMJ) associated with opening and closing his jaw. The patient’s symptoms were managed with oxycodone and intra-articular steroid injections at bilateral TMJ with partial alleviation of pain. Despite apparent benefit from steroidal injections, the patient was forced to discontinue this course of treatment due to concern for systemic steroids potentially interfering with the underlying pathophysiology of acromegaly.

Then, at age 48, two years following the second transsphenoidal resection, the patient was referred to outpatient physical therapy for education in self-management of pain and prevention of worsening symptoms according to physician order.

The PT evaluation consisted of goniometric measurement of mandibular active range of motion (AROM), qualitative description of mandibular AROM and patient’s posture, subjective pain levels using the Numeric Pain Rating Scale (NPRS), and subjective impact of the patient’s symptoms on his function and quality of life (QOL) using the Gothenburg Trismus Questionnaire (GTQ). The GTQ was utilized to monitor progress and to assess the impact of physical therapy interventions on the patient’s symptom burden and QOL.

The GTQ was selected as it is well-accepted by patients, with satisfactory compliance, and low rates of missing items. The GTQ was found to demonstrate good internal consistency (Cronbach’s alpha >0.70) as well as test-retest reliability of .97.¹¹ The GTQ contains 21 items; with 13 items divided into three domains with a one-week recall period: jaw-related problems; eating limitation; and muscular tension. The remaining eight items are retained as single items.¹¹ The domains and single items range from 0 – 100, where 100 indicates maximal amount of symptoms and 0 is equal to no symptoms.¹¹ The GTQ is suggested to be used as a screening tool as well as an endpoint in intervention and jaw physiotherapy/rehabilitation studies.¹¹ Although the GTQ has not been validated as a QOL measure, in this case, based on the patient’s stated impact of jaw-related issues on his overall wellbeing and QOL, the results of the questionnaire were utilized to make inference regarding the patient’s QOL.

At initial evaluation, the patient reported gradually worsening pain at bilateral TMJ, right side worse than left, constant in nature, rated 8/10 on average while at rest on the NPRS. The patient complained that pain often increased with sneezing, eating/chewing, and yawning, and he reported frequently awaking at night with 10/10 pain at the right TMJ. The patient’s composite score on the GTQ at initial evaluation was 81% and the patient reported “very severe” pain in his face and jaw during the last week (GTQ item 2), and “unbearable” pain during the last month (GTQ item 15). The patient’s mandibular AROM at initial evaluation is shown in Table 1.

Table 1:

Initial Evaluation and Outcomes

	Initial Evaluation	Visit 4, Week 4	Visit 8, Week 12
Maximum Mandibular Opening	42 mm	46 mm	42 mm
	Moderate anteroinferior translation of right temporal condyle noted with moderate mandibular deviation to left	Moderate deviation to left	No deviation observed
	8/10 pain*	6/10 pain*	0/10
TMJ Lateral Excursion to Right	8 mm	11 mm	10 mm
	9/10 pain*	6/10 pain*	5/10 pain*
TMJ Lateral Excursion to Left	15 mm	13 mm	11 mm
	9/10 pain*	6/10 pain*	5/10 pain*
Limitation eating solid food†	“Severe”	---	“Mild”
Limitation biting off†	“Very severe”	---	“Moderate”
Worst pain during the last month†	“Unbearable”		“Severe”
Average pain during the last month†	“Unbearable”	---	“Moderate”

^*Pain produced at right TMJ; rated on Numeric Pain Rating Scale (NPRS)

^†Individual responses from Gothenburg Trismus Questionnaire

Additional subjective questioning revealed that due to overgrowth of his fingers and subsequent decreased fine motor dexterity, the patient was unable to perform work duties as an optician or play the guitar, a hobby he enjoyed. Related to his symptoms of facial pain and TMD, the patient reported decreased social participation. These reports were felt to be of particular significance given patient’s history of depression and anxiety.

Soft tissue and musculature were assessed with fingertip palpation and revealed restricted soft tissue mobility throughout cranium, and around bilateral TMJ and cervical regions. Skin glides were performed in these regions in all directions to assess tissue extensibility, end feel, and recoil. This assessment revealed hardened end feel and decreased recoil, especially in caudal direction over bilateral TMJ. Palpation also revealed tenderness to palpation throughout bilateral muscles of mastication including masseter and temporalis muscles.

In the absence of imaging, assessment of underlying bony changes and potential impact on jaw occlusion and joint play was limited to observation and palpation. With mandibular opening, observation and fingertip palpation revealed hypermobility of right TMJ compared to left with excessive inferior and anterior displacement of mandibular condyle. This hypermobility, possibly due to hyperplasia of the right mandibular condyle resulting in joint instability, was felt to contribute to the asymmetry of mandibular opening and the higher level of pain located at the right TMJ.

The patient’s posture was assessed via observation. Patient demonstrated increased thoracic kyphosis, moderately forward head and neck, and rounded shoulders bilaterally.

Based on the results of soft tissue and TMJ palpation, observation of mandibular movement, observation of posture, as well as patient’s subjective reports of localized pain at face and bilateral TMJ, patient’s TMD was classified as a mixed disorder of TMJ and masticatory muscles with possible contribution of cervical spine dysfunction.¹²

Intervention

Overview of Intervention

To assist the patient in his goal of improved facial pain and TMJ function, the patient was treated with multimodal interventions including manual therapy^13–17, relaxation techniques^1,14–16, neuromuscular re-education^14–16, and therapeutic exercise^13–16

A licensed physical therapist conducted one-on-one treatment sessions in an outpatient setting for approximately forty-five minutes. A typical PT session consisted of a brief re-evaluation, manual therapy, therapeutic exercise and/or neuromuscular re-education, and instruction in a home exercise program (HEP).

Initial Approach

Based on the patient’s impaired mandibular opening, pain at bilateral TMJ, and restricted soft tissue mobility, initial goals of PT were to optimize TMJ and soft tissue mobility and to minimize pain.

As the TMJ functions bilaterally, symmetry of movement is crucial for optimal jaw occlusion and mandibular opening.¹⁶ To improve symmetry of joint mobility and mandibular movement, manual therapy techniques were initiated at the first PT visit following initial evaluation including soft tissue mobilization to skin, fascia, and bilateral masseter and temporalis muscles. Fascial manipulation has been shown to decrease subjective pain perception in cases of TMD, and the additional proposed rationale in this case was to minimize asymmetrical soft tissue tension impacting TMJ mobility.¹⁷ With the patient positioned in supine, skin and fascial mobilization was performed with multiple prolonged holds (30–60 seconds) over bilateral TMJ with emphasis in caudal direction based on direction of greatest restriction identified during evaluation. Skin and fascial mobilization was also performed at similar dosages over bilateral sides of cranium, forehead, and mandible. As mobilization to the cervical spine has been found to decrease pain intensity and sensitivity in patients with cervico-craniofacial pain of myofascial origin, soft tissue mobilization was performed throughout cervical musculature including bilateral upper trapezius, sternocleidomastoid, and submandibular muscles.¹⁸ Cervical spine passive range of motion sidebending was performed bilaterally with the patient positioned in supine (3 repetitions of 30 second holds).

Relaxation techniques have been shown to be effective in treatment of TMD and, considering patient’s history of depression and anxiety, were initiated at first PT visit following initial evaluation.^1,13–15 Relaxation techniques included diaphragmatic breathing (10 breaths) performed in supine, as well as education to increase patient’s awareness to habitual jaw clenching and subsequent relaxation of the muscles of mastication.

The patient was also educated regarding potential impact of posture on TMD, and was instructed in initiation of postural strengthening exercises including seated chin tucks and seated bilateral shoulder horizontal abduction with theraband resistance (2 sets of 10 repetitions each).^13,19,20

Home Exercise Program (HEP)

To maximize gains and improve carryover, the patient was instructed in a HEP at the first PT visit following initial evaluation. Initial HEP included relaxation techniques and postural re-education as noted above. To maintain soft tissue mobility gains and to encourage pain-free movement, gentle mandibular AROM exercises including opening (2 sets of 10 repetitions) and bilateral excursion (10 repetitions each way) in pain-free ranges were added to initial HEP. The HEP was prescribed to be performed once per day, and the patient was encouraged to utilize relaxation techniques for pain management as needed.

Progression of Therapeutic Techniques

At fourth PT visit, the patient demonstrated increased mandibular opening, however continued to demonstrate moderate lateral deviation to the left with maximum opening. This ongoing lateral deviation was felt to be due to ongoing soft tissue restrictions, asymmetry of joint mobility, as well as impaired neuromuscular control. The patient’s pain at bilateral TMJ improved but remained persistent, rated 6/10 on the NPRS upon maximum mouth opening.

At this time, to address the patient’s impaired motor control contributing to mandibular deviation, neuromuscular re-education exercises were initiated, utilizing a handheld mirror for visual feedback. The patient performed mandibular opening exercise (as previously prescribed) now with emphasis on maintaining neutral alignment throughout the movement.

Manual therapy techniques were also progressed to further address the patient’s pain and TMD. Intraoral soft tissue mobilization has been shown to be effective in treating myogenous TMD and was introduced including bilateral masseter release.²¹ TMJ mobilization was introduced for pain relief (bilateral TMJ grade 2 oscillatory mobilizations performed for 60 seconds) and to achieve improved symmetry of TMJ mobility (left TMJ grade 2 sustained mobilization performed for 60 seconds).

Progression of Home Exercise Program

The patient’s HEP was progressed to include neuromuscular re-education as described above as well as self-manual therapy including fascial release performed in an inferior direction at bilateral TMJ with palmar surfaces of fingers two through five, held for 30–60 seconds. He was instructed to perform updated HEP once per day, and to utilize self-manual therapy as pain management as needed.

Outcomes

Service Delivery

The patient was treated over a three-month period for a total of nine PT sessions. The initial recommendation for frequency and duration of PT sessions was two times per week for four weeks, however the patient preferred only one visit per week as he traveled a long distance to attend sessions. The course of PT was extended following re-evaluations at the fourth and eighth PT visits due to demonstrated progress and potential for further functional improvements. The course of PT was curtailed after the ninth visit due to an acute change in medical status unrelated to the course of PT.

Impairment, Functional Limitation, and QOL

Following intervention, the patient demonstrated improved symmetry of TMJ AROM, and decreased facial pain at rest, with mandibular movements (opening/closing, bilateral excursion), and with functional activities including eating, chewing, and yawning (Table 1).

At evaluation, the patient’s maximum mandibular opening (42 mm) was considered within functional limits, however patient’s high level of pain (8/10 on NPRS), and moderate lateral deviation were considered abnormal. At fourth PT visit, patient’s maximum opening increased to 46 mm, pain with mouth opening decreased to 6/10, however moderate deviation was still observed. At eighth PT visit, following increased emphasis on neuromuscular re-education, the patient’s maximum mandibular opening returned to 42 mm, however no deviation was observed, and patient reported no pain with opening.

The patient continued to report intermittent facial pain, however, according to the patient’s GTQ responses, average pain improved from “unbearable” at initial evaluation to “moderate” after eight sessions of PT. The patient’s GTQ score improved from 81% to 67.6% (Figure 1).

Figure 1.

Gothenburg Trismus Questionnaire Score Over Time

Eight weeks following final PT visit, patient was contacted for follow up and discharged due to inability to return. Patient was mailed a copy of the GTQ for final assessment. The patient reported ongoing compliance to HEP and subjectively reported that his pain remained at a bearable level. The patient’s GTQ score further improved from 67.6% to 61.3% (Figure 1).

Discussion

This case report provides an example of a multimodal conservative approach to facial pain and TMD in the setting of acromegaly. Although the techniques utilized in this case have been previously demonstrated in the literature to be effective in treating TMD, to the author’s knowledge, this is the first account of a multimodal approach utilizing these techniques with a patient with facial pain and TMD due to acromegaly.

Patients with a history of acromegaly may represent a unique subgroup of patients who experience facial pain and TMD because of the differences in underlying etiology. Common etiologies of TMD include muscular hypertonicity, degenerative joint changes, and internal derangement of the TMJ disc. In contrast, in cases of acromegaly, due to altered systemic hormone levels secondary to a benign pituitary tumor, etiologies of TMD more commonly include mandibular overgrowth and enlargement of oral tissues resulting in altered positioning of the mandible and loss of stable occlusion.^1,7 Though this case did not have supporting imaging to quantify overgrowth of bony and soft tissues, the patient’s clinical presentation was consistent with malocclusion and TMD secondary to mandibular overgrowth and soft tissue changes. The results of this case report suggest that patients with facial pain and TMD secondary to bony and soft tissue changes in the setting of acromegaly may benefit from a multimodal conservative approach with PT.

Following PT intervention, the patient’s GTQ score improved by 19.7%. The minimal detectable change (MDC) and minimally clinically important difference (MCID) have not been established for the GTQ, however, considering the chronicity of the patient’s symptoms and the patient’s individual responses on the questionnaire, the author considered this improvement significant. For example, patient reported “severe” limitation eating solid food and “very severe” limitation biting off while eating pre-treatment which improved to “mild” and “moderate” limitations respectively post-treatment. Additionally, patient initially reported average pain over the previous month as “unbearable” which improved to “moderate” following treatment. (Table 1). Although the GTQ is not validated as a QOL measure, the patient’s improved ability to eat and decreased average pain contributed to his improved QOL.

Alternative explanations should be considered for the patient’s functional and subjective improvements. The patient’s history of depression is significant in the setting of TMD as depression has been positively correlated with pain catastrophizing in patients with TMD.^22,23 Given the patient’s subjective high level of pain at initial evaluation despite mandibular opening AROM within normal limits, it may be considered that the patient’s pain and disability were not merely somatic in nature. The patient verbalized appreciation and relief throughout the course of PT for his symptoms being acknowledged and addressed. During the course of PT, the patient was also referred to occupational therapy to address his fine motor impairments which provided him compensatory strategies that he felt were useful in regaining his independence. The psychosomatic impact of the relaxation techniques and therapeutic services provided may offer an additional explanation for the patient’s improvements. Furthermore, the patient concurrently engaged in outside counseling which may have contributed to overall improvement and underscores the importance of a multidisciplinary approach in this population.

Additional explanation should also be considered for the increase in maximum mandibular opening noted at fourth PT visit. The smallest detectable difference for maximal mouth opening in healthy subjects has been found to be 3–5mm.²⁴ Although this may have limited generalizability to a case of acromegaly, as the patient demonstrated a 4mm increase, it must be acknowledged that this degree of change may be within measurement error. This would offer an explanation for the subsequent return to baseline for maximum mandibular opening. The corresponding improvement in pain and quality of movement, however, may suggest that these changes were attributable to improving soft tissue mobility, joint mechanics, and neuromuscular control.

As a critical aspect of this patient’s evaluation, treatment, and outcomes was soft tissue mobility, this case report and further investigations in this population would be strengthened by detailed objective assessment and outcomes regarding soft tissue extensibility and muscle balance.

As a result of the intervention, the patient was able to achieve an acceptable level of pain relief without use of pharmacological treatments. This was significant as the patient had previously been forced to discontinue use of steroid injections. This conservative intervention may be of particular benefit by providing an alternative pain management strategy while minimizing side effects of long-term steroid use. Additionally, as opioid use and reliance are being scrutinized on a national level, this case presented an example that may offer physicians an alternative to opioid prescription to treat facial pain and TMD secondary to acromegaly.

The initial recommendation of two sessions of PT per week for four weeks was based on the patient’s need for manual therapy with the intent of optimizing soft tissue and joint mobility. The patient achieved excellent results with a frequency of one session per week spread out over twelve weeks, however it may be feasible to achieve similar results with a shorter duration of PT services.

Based on the patient’s further improvement in GTQ score eight weeks following transition to independent HEP, he demonstrates continued improvement in pain, function and QOL. It is possible that if the patient’s course of PT was not curtailed by a change in medical status, his subjective and objective improvements would have continued to progress. Longer follow up would be required to determine the potential long-term benefit of PT interventions and independent HEP in this population.

The results of this case report suggest that conservative management through physical therapy may be beneficial as a treatment strategy for patients suffering from facial pain and TMD secondary to acromegaly following treatment for benign pituitary macroadenoma. Although rare, acromegaly may be encountered with increased frequency in rehabilitation settings as improved screening and treatment for benign pituitary tumors leads to improved monitoring, earlier referral, and intervention for associated symptoms. Continued research is needed to determine the generalizability of these results to other patients living with facial pain and TMD secondary to acromegaly.