Case Report: Complex Regional Pain Syndrome Involving the Head and Face After Severe Malignant Otitis Media and Mastoiditis

Abstract

Background

Complex regional pain syndrome (CRPS) is a rare condition characterized by intense chronic pain, vascular changes, swelling, and trophic changes leading to significant morbidity. It is often related to crush/mechanical injury or surgery and most commonly affects the limbs. There have only been a few rare documented cases affecting the head and face, and more scarce are CRPS cases of the head and face unrelated to trauma.

Case

We report a 79-year-old woman referred to Neurology after three months of severe, persistent left-sided head and face pain associated with discrete episodic swelling and localized autonomic symptoms, skin and hair changes in the affected area, following severe otitis media complicated by mastoiditis. Her history and neurologic exam did not meet a categorical diagnosis based on criteria in The International Classification of Headache Disorders-3rd edition and International Classification of Orofacial Pain, 1st edition but her symptoms and signs met the Budapest Criteria (BC) for a diagnosis of CRPS of the head and face. The patient had been on carbamazepine with only marginal benefit. After the initial Neurology evaluation, she started duloxetine and was referred for mirror visual feedback therapy. During an acute attack, she had a very good response to a sphenopalatine ganglion block and compounded intranasal and topical lidocaine-ketamine preparations were adjunctively added. Eleven months after initial symptom onset, she reported significant symptomatic improvement with markedly decreased disability and return to her premorbid quality of life.

Conclusion

Complex regional pain syndrome is difficult to recognize, there are no diagnostic tests, there is no definitive cure and management is symptomatic. Early identification and treatment may prevent significant morbidity and we recommend review and classification of such cases using the BC.

Introduction

Complex regional pain syndrome (CRPS) is a rare chronic pain syndrome with incidence 15–26 per 100,000 and more common in women than men (3.5:1), with highest incidence in women aged 60–70 years. ¹ The pain is characterized as out of proportion to the inciting injury and is a cause of significant morbidity and functional impairment. ² The pathophysiology is not clearly understood and is thought to be a pro-inflammatory response with multifactorial comorbidities involving dysregulation of the sympathetic and central nervous system pain regulatory response and possibly genetic and psychological components. ³ Complex regional pain syndrome is related to crush/mechanical injury or surgery and it is not uncommon for patients to have a history of psychiatric illness. Complex regional pain syndrome is difficult to diagnose because there are no diagnostic tests and it typically develops within 4–6 weeks after the initial injury. Complex regional pain syndrome is subdivided into two subtypes: CRPS-I and CRPS-II. Both share the same diagnostic categories, however, CRPS-I does not have a confirmed nerve injury. ⁴ Complex regional pain syndrome-I can occur without any known injury in which case, may be secondary to infection or other pathologies causing internal injury. ⁵ Complex regional pain syndrome can manifest anywhere in the body, most commonly the limbs, and only rare cases involving the head and face have been reported.^6,7 There is no established treatment for CRPS and management is symptomatic; individualized to the patient, with the most favorable outcomes achieved with earlier diagnosis and treatment.^1,2,6,8 To establish a diagnosis, the International Association for the Study of Pain (IASP) accepted the Budapest Criteria (BC) where the following must be met ² :

Continuing pain disproportionate to any inciting event.

Must report ≥1 symptom(s) in all four of the following categories:

○ Sensory: hyperesthesia and/or allodynia,

○ Vasomotor: temperature asymmetry and/or skin color changes and/or skin color asymmetry,

○ Sudomotor/edema: edema and/or sweating changes and/or sweating asymmetry,

○ Motor/trophic: decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (skin, hair, nails).

Must display evidence of ≥1 sign(s) at time of evaluation in ≥2 of the following categories:

○ Sensory: hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement),

○ Vasomotor: temperature asymmetry (>1 °C) and/or skin color changes and/or asymmetry,

○ Sudomotor/Edema: edema and/or sweating changes and/or sweating asymmetry,

○ Motor/Trophic: decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (skin, hair, nails).

No other diagnosis that better explains the symptoms and signs.

Case

A previously healthy 79-year-old woman, avid golfer, with a past medical history of anxiety and depression since losing her husband 1 year prior, presented with severe, persistent unilateral head and facial pain which evolved immediately after hospitalization for severe acute otitis media complicated by mastoiditis. Prior to hospitalization she initially developed left ear pain rapidly progressing over 3 days. The morning of day 4 she woke with severe left ear pain with hearing loss and a pink discharge on her pillowcase also dripping from her left ear. The Emergency Department evaluation showed a temperature of 38.3 °C, mastoid tenderness, and edematous left external auditory canal with purulent serosanguinous drainage. A computed tomography head showed opacified left mastoid air cells, no erosive changes or air cell coalescence. There was mucosal thickening and near complete opacification of the left external auditory canal obscuring visualization of the tympanic membrane (Figure 1). Ceftriaxone and vancomycin were immediately started; Otolaryngology was consulted and added otic ciprofloxacin drops and admission for continued IV antibiotic treatment of severe otitis media complicated by mastoiditis.

Figure 1.

There was mucosal thickening and near complete opacification of the left external auditory canal obscuring visualization of the tympanic membrane.

Emergency Department Labs: leukocytes 11.4, neutrophils 82.4%, CRP 218, ESR 46. Blood cultures drawn hours after antibiotics were started remained negative. The following day infectious disease discontinued ceftriaxone, vancomycin and started IV ampicillin–sulbactam and IV ciprofloxacin. The patient improved and was transitioned to the analogous oral agents and continued otic ciprofloxacin. On hospital day 4, she had very mild, dull, burning left-sided head pain and was discharged with an Otolaryngology follow-up appointment in 2 weeks. Otolaryngology noted hearing in her left ear returned; however, the left-sided head pain intensified with radiation anteriorly behind her left eye and down into her left cheek, maxilla, and upper molars. She had severe periodic exacerbations triggered by innocuous stimuli such as wind blowing on the left side of her head and sleeping with the left side of her head on the pillow. She described the exacerbations as intensely sharp, stabbing left side-locked pain emanating from the left parasagittal apex of her head radiating anteriorly into the forehead, anterior temple, periorbital, and maxillary regions. These regions would feel heavy and hot to her touch and developed an intense redness with swelling in the left periorbital, maxillary, and palpebral regions.

Otolaryngology reassured her that the headache was sequela from the infection, and would eventually resolve. Two months after symptom onset, she slowly developed mild left eyelid weakness partially obscuring her vision, and high-dose NSAIDs were no longer effective. Otolaryngology ordered MRI brain and neck without and with contrast and MRA head and neck were read as normal without damage to sympathetic chain or carotid artery. Repeat ESR, CRP, and mastoid aspiration cultures were normal, and infection was ruled out. Dental exam ruled out temporomandibular joint dysfunction and other dental etiology. She was concurrently evaluated by Rheumatology and Ophthalmology and no abnormalities were found. Eight weeks after symptom onset, her left ptosis worsened and Ophthalmology performed left superior palpebral blepharoplasty. Fifteen weeks after onset, the headaches became severely debilitating and did not respond to tricyclics, α2δ ligands, triptans, or CGRP targeting agents. Carbamazepine 600 mg twice daily was started which was only marginally effective and Otolaryngology referred the patient for emergent Neurology management of refractory trigeminal neuralgia.

Neurologic history: Head pain was characterized as unremitting burning, achy, and pressure-like with intensity 5/10 on the left side of her head and face extending from parasagittal apex of her head to the anterior left forehead, behind the left eye, and into left maxilla. Exacerbations were triggered by touch (including light touch), wind, and warm water to the left side of the head and face and characterized as a sharp, stabbing pain at the left parasagittal apex of her head with peak intensity “20/10” and shooting down to her left forehead, eye, anterior ear, and maxilla (Figure 2). There was an associated outward pushing sensation behind her left eye and the pain would last hours and described as “exponentially” worse compared to the pain from the initial ear infection. There was mild photophobia only without nausea, conjunctival injection, tearing, rhinorrhea, ptosis, or miosis; change in salivation, taste, significant changes in her vital signs other signs of dysautonomia during exacerbations. The pain would eventually return to the baseline persistent burning, achy sensation with intensity 5/10 which never subsided. The patient also complained of “hot flashes” in her left cheek with redness and swelling and associated heavy feeling. She noticed the affected area did not sweat, and she had lost her eyebrow hair and the hair around her forehead and anterior ear was shorter and did not grow much anymore (Figure 2). Exacerbations were so intense she stopped playing golf and had to sleep sitting up so her head did not rest on her left side.

Figure 2.

Stabbing pain at the left parasagittal apex of her head with peak intensity “20/10” and shooting down to her left forehead, eye, anterior ear and maxilla. Adapted from: Smith, C., Suen, J.Y. (2018). Applied Neuroanatomy of the Face and Head. In: Suen, J., Petersen, E. (eds) Diagnosis and Management of Head and Face Pain. Springer, Cham (p. 5). https://doi.org/10.1007/978-3-319-90999-8_1.

Neurologic examination: There was allodynia to both superficial and deep stimuli and hyperpathia with mild pinprick both elicited at the origin of her pain in the region of the left greater occipital nerve in line with the posterior edge of the helix of the ear and radiated anteriorly into the left supraorbital nerve distribution. Discrete erythema and edema were observed in the left zygomaticotemporal, zygomaticofacial, and infraorbital regions as compared to the right. There was a measured skin temperature difference of 1.3 °C between the affected and unaffected sides. There was dry friable skin in the left zygomaticotemporal, zygomaticofacial, and infraorbital nerve distributions and an iodine-starch test in bilateral identical areas in a hot sunny room showed clumping only on the right cheek. ⁹ There was markedly decreased hair growth in the left zygomaticotemporal, auriculotemporal, and inferior greater occipital and superior lesser occipital nerve territories (Figure 2). Pupils were round equal and reactive, extraocular movements were normal and painless. There were no other cranial nerve signs, and the rest of the exam was normal.

Symptoms did not meet International Classification of Headache Disorder-3rd edition (ICHD-3) criteria for migraine, cluster headache, or any of the major non-odontogenic syndromes of headache with facial pain (trigeminal neuralgia, trigeminal autonomic cephalalgias, post-herpetic neuralgia, persistent idiopathic facial pain), or paratrigeminal oculosympathetic syndrome. ¹⁰ The pain was not restricted to a specific nerve territory or dermatome and the constellation of symptoms met the BC for CRPS.

The patient had left-sided signs and symptoms with intermittent painful exacerbations disproportionate to the pain from the inciting infection in an area much larger than the primary site of injury, which no other diagnosis could better explain (signs and symptoms summarized in Table 1). ¹¹

Table 1.

Summary of the Patient's Symptoms and Signs by the Budapest Criteria Diagnostic Category.

Diagnostic categories	Symptoms	Signs
Sensory	Constant burning, achy and pressure-like pain. Intermittent exacerbations disproportionate to the pain from the inciting infection.	Hyperpathia to pinprick in affected area. Allodynia to light touch and deep pressure in affected area.
Vasomotor	“Hot flashes” on her left cheek.	Left cheek redness, temperature difference between affected and non-affected sides was +1.3 °C on the left.
Sudomotor/edema	Left cheek swelling and anhidrosis.	Dry, edematous skin of left cheek with anhidrosis.
Motor/trophic	Left ptosis status post blepharoplasty, decreased hair growth around her left ear.	Friable skin, markedly decreased hair growth on the left scalp in the zygomaticotemporal, auriculotemporal, greater and lesser occipital nerve regions.

She was started on duloxetine 60 mg twice daily, referred for mirror visual feedback and carbamazepine was decreased to 300 mg twice daily with modest improvement 4 weeks after initial presentation to Neurology. During an acute attack, she underwent a transnasal sphenopalatine ganglion block using a Sphenocath^® device with a lidocaine–ketamine mixture which reduced her pain from 10/10 to 3/10 within 10 minutes which was lower than her baseline 5/10 pain. Based on her response, a compounded lidocaine–ketamine nasal spray and a similar compounded topical cream applied to the affected skin provided significant additional benefit and the carbamazepine was discontinued. Treatment was well tolerated and there was a marked improvement in her pain with significantly decreased frequency, intensity, and duration of exacerbations. On her eight-month follow-up (11 months after initial symptom onset), she reported “very much improved” on the Patient Global Impression of Change questionnaire and marked improvement in her quality of life and return to her premorbid quality of life.

Discussion

Complex regional pain syndrome is a rare chronic pain condition, and the pathology is still not completely understood. There are no diagnostic tests that can quantify the symptoms. The ICHD-3 and the recently published first edition (International Classification of Orofacial Pain) do not include criteria to aid in the diagnosis of CRPS affecting the head or face and a diagnosis is made using the BC criteria established by the IASP which has a sensitivity of 0.99 and specificity 0.68 over older criteria.⁸ It is commonly due to crush, mechanical injury, or surgery though occasionally, it can develop without any known injury. Complex regional pain syndrome uncommonly affects the head and face and may very rarely be seen after a severe infection.^2,5,12 Complex regional pain syndrome causes morbidity adversely affecting quality of life and is a diagnosis which should be considered in unexplainable pain syndromes of the head and face meeting the BC and after a thorough examination does not reveal any other diagnosis. Though there is no single effective treatment, early diagnosis, and prompt multidisciplinary treatment may lead to decreased disability and improvement in quality of life.

Clinical Implications

• CRPS can present as a syndrome of headache with facial pain.

• CRPS can develop without any known mechanically related trauma.

• These cases should be reviewed and classified according to the BC as early diagnosis and treatment may improve symptoms and quality of life.