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Current Pain and Headache Reports

https://doi.org/10.1007/s11916-022-01018-w

UNCOMMON AND/OR UNUSUAL HEADACHES AND SYNDROMES (J AILANI, SECTION


EDITOR)

Trigeminal Traumatic Neuroma: a Comprehensive Review


of the Literature Based On a Rare Case
Davis C. Thomas1   · Saranya Devatha Mallareddy2 · Jeffrey P. Okeson3 · Josna Thankachan4 ·
Priyanka Kodaganallur Pitchumani5 · Reshmy Chellam Pichammal2

Accepted: 24 January 2022


© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022

Abstract
Purpose of Review  Traumatic neuromas in general, and trigeminal traumatic neuromas in particular, are relatively rare
entities originating from a damage to a corresponding nerve or its branches. This manuscript is a comprehensive review of
the literature on trigeminal traumatic neuromas based on an interesting and challenging case of bilateral intraoral lesions.
Recent Findings  The diagnosis for this patient was bilateral trigeminal traumatic neuromas. It is possible that these patients
have a genetic predisposition to the development of these lesions. It is a neuropathic pain condition and may mimic dental
and other trigeminal pain entities. Topical treatment with lidocaine gel, utilizing a custom-made neurosensory stent, rendered
the patient significant and sustained pain relief.
Summary  Trigeminal traumatic neuromas present a diagnostic challenge even to a seasoned clinician, due to the complex
clinical features that may mimic other entities. Topical medications such as local anesthetics may be a good viable alternative
to systemic medications to manage the pain associated with the condition. Early identification of the lesion and the associ-
ated pain helps in the succinct management of symptomatic trigeminal traumatic neuromas.

Keywords  Traumatic neuroma · Oral · Neuropathic pain · Tinel’s sign · Neurosensory stent · Topical medication ·
Trigeminal nerve

Introduction cells, and fibroblasts, in a dense matrix of connective tissue


[1, 2•]. A consensus on the definition of the lesion is lack-
Traumatic neuroma (TrN) is a non-neoplastic condition ing. Pain associated with trigeminal TrN is highly varied,
secondary to trauma (surgical or incidental), characterized including absence of it [3•] and may be provoked or spon-
by the presence of proliferating nerve fibers, inflammatory taneous. The gross appearance of the lesion is described as
a smooth, palpable mass that could be tender or non-tender
This article is part of the Topical Collection on Uncommon and/or on palpation [3•, 4, 5].
Unusual Headaches and Syndromes This comprehensive review is based on a case of bilat-
eral trigeminal TrN occurring in a middle-aged female
* Davis C. Thomas patient. The interesting feature of this case was that the
davisct1@gmail.com
lesions appeared bilaterally, but on different divisions of the
1
Center for TMD and Orofacial Pain, Rutgers School trigeminal nerve, and temporally not related to each other.
of Dental Medicine, Newark, NJ, USA We attempt to discuss the epidemiology, clinical features,
2
Ragas Dental College and Hospital, Chennai, India pathophysiology, investigations, differential diagnosis, and
3
College of Dentistry, University of Kentucky, Lexington, KY,
management modalities of trigeminal TrN based on an
USA exhaustive literature review that we performed.
4
Department of Diagnostic Sciences, Rutgers School
of Dental Medicine, New Jersey, NJ, USA
5
The Ohio State University College of Dentistry, Columbus,
OH, USA

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Current Pain and Headache Reports

Case Report With regards to the mandibular pain, she described bursts
of pain intensity of seven on the same scale.
History of Presenting Illness
Quality
A 56-year-old Indian female patient presented with chief
complaints of chronic pain in the distribution of the second The background pain had a quality of “dull aching” in both
division (maxillary, V2) of the left trigeminal nerve and the arches, and the exacerbated pain had “pulsating” on the
the third division (mandibular, V3) of the right trigemi- maxillary site, and “shooting” on the mandibular site.
nal nerve. Her medical history was positive for diabetes
mellitus and hypercholesterolemia, both managed well Temporal Characteristics
with medications. The pain has been existing for the past
approximately 30 years. These two pains had started sub- Over a time period of approximately 30  years, the pain
sequent to different surgical procedures, separated by a few evolved to a slow chronic progressive type.
years. The quality of the pain had changed over the past 15
to 30 years; the intensity was variable, and she was una-
ble to achieve any meaningful pain relief, although going Aggravating Factors/Triggers
through multiple dental procedures in an attempt to do so.
The details of the characteristics of the pain appear below. The pain worsened upon chewing, “after a meal,” acid foods
(such as citrus fruits, cheese, tamarind), and left lateral
recumbent sleep position.
Onset
Relieving Factors
The pain was described as of slow onset.
None reported.
Location
Response to Past Treatment
The reported sites of pain were maxillary left buccal gin-
giva in relation to upper molar tooth (left V2), and man- Since the onset of the pain, the patient underwent several
dibular right buccal gingiva in relation to the molar tooth dental procedures without any pain relief. The patient also
(right V3). reported change in the pain characteristics, sometimes for
the worse, every time another dental procedure was done.
Medications that were “tried” included but were not limited
Frequency to (varying dosages of) non-steroidal anti-inflammatories
(NSAIDs), anti-epileptic medications, nortriptyline, benzo-
The patient reported continuous “background” pain on the diazepines, and vitamin supplementations. These medica-
left V2 division, and occasional exacerbations of approxi- tions were relatively ineffective.
mately twice a week on the same area, and continuous pain
on the right V3 division with nocturnal exacerbations. Associated Features

None reported.
Duration

The maxillary left dull background pain was continuous Radiation/Referral


(hours); the exacerbated pain at the same site of four to 5-h
duration; and the mandibular right quadrant pain as continu- The exacerbated pain radiated to the temple, eyebrow, preau-
ous (hours) with nocturnal exacerbations. ricular, and postauricular areas.

Association with Sleep
Intensity
The patient reports a reduced quality of sleep in general. She
The intensity of the background pain on the maxillary site is tired during the day and takes sleep aid medications. The
was five to six on a visual analog scale (VAS) of zero to ten. pain does not wake her up from sleep. She does wake up at
Exacerbated pain was described as ten on the same scale. her routine waking time and realizes she has pain.

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Current Pain and Headache Reports

Clinical Examination Findings Discussion

Painful trigger zones in relation to the gingiva supplied by Definition


the maxillary and mandibular divisions of the trigeminal
nerve were identified. These areas were approximately 3 mm Traumatic neuroma is defined as a non-neoplastic repara-
in diameter and looked relatively paler color compared to tive/reactive [6–8] lesion of an injured nerve, made up of
the healthy gingiva surrounding them. There were no signs rapidly proliferating axons, inflammatory cells, Schwann
of inflammation or any pathology in or around these well- cells in a dense fibrous connective tissue stroma [2•, 9,
circumscribed trigger zone areas. The patient’s familiar 10].
pain was reproduced by eliciting Tinel’s sign (pain upon
percussion of the lesion). Provocation by light touch did
not reproduce the pain from the trigger zone areas. Local Classification
infiltration of a small amount (0.5 cc) of 3% mepivacaine
without epinephrine, in a close proximity around the trigger Although no universally accepted official classification
zone (approximately 3–5-mm radius), rendered the patient exists, most of the literature is in agreement of two types of
complete pain relief. TrNs, neuroma in continuity (NIC), and nerve end neuroma
(NEN). The obvious morphological difference between the
two is that there is a continuity of the nerve maintained in
Management NIC, whereas NEN has the distal portion separated and most
likely disintegrated [11].
The TrN was managed effectively with a 5% topical lidocaine
gel, which was retained on the site of the paler color area in
a thermoformed neurosensory stent. The technique of filling Etiology/Pathophysiology
in a minimal amount of the anesthetic in the neurosensory
stent was shown to the patient, and she was prompted to TrN is a lesion that results from an attempted repair by
repeat the same during the same visit. The at-home instruc- the nerve [12]. Hypotheses on the proposed stages of TrN
tions were to repeat the same twice daily over a period of development have been published in the literature [13].
2 months, meanwhile having weekly evaluations at the clinic. However, to date, there is no absolute confirmation on the
Local anesthetic injection (0.5 cc of 3% mepivacaine without exact sequence or the precise mechanism of development
epinephrine) was administered at every re-evaluation visit. of TrN. It has been proposed that a combination of nerve
The symptoms improved gradually over 2 months, and the repair, contraction of wound, and scarring (which are a part
patient was completely pain-free at the end of the third month of the healing process of the wound), and the accompanying
re-evaluation visit. Concurrently, all the chief complaints neuronal proliferation contribute to the formation of TrN
associated with the pain completely dissipated. At the six- [12, 13]. TrN develops subsequent to an incidental or surgi-
month re-evaluation, the pain associated with the two sites cal trauma [14]. It has also been proposed that lesions that
had almost completely disappeared, with a possible exception are not “planned” surgically are incidental, traumatic, or
of “occasional twinges” of pain localized to original trigger “unclean” have a propensity to develop TrN [2•, 8]. The type
zone, upon applying pressure. of force such as compressive and traction has been hypoth-
esized to be of etiological importance in TrN formation [15].
Pro-inflammatory cytokines TNF- α, IL-6, and IL-1β
Prognosis were shown to be present in significant concentrations in
traumatic neuroma lesions of the sciatic nerve in rats [2•].
With the consecutive anesthetic injections, and employ- It is not clear from the literature whether these inflamma-
ment of the neurosensory stent with topical anesthetic, the tory cytokines play a role in the formation or sustenance
patient reported approximately 90 percent pain relief from of the lesion, or are a result of the ongoing pathology
her chronic familiar pain. Utilizing the non-invasive modali- within the lesion. Histologically, TrN lesions consist of
ties such as neurosensory stent with topical anesthetic, the a disorganized cluster of proliferating axons, inflamma-
prognosis of this pain condition was good. At the 1-month tory and formative cells, embedded in a dense connective
and 3-month re-evaluation visits, the patient had almost tissue matrix [16, 17]. Earlier studies had indicated that
complete pain relief on the affected areas. It is indefinite the most common procedure implicated in the etiology of
and probably unknown as to how many months she may trigeminal TrN formation was extraction of teeth [14, 18,
have to continue the neurosensory stent (lack of consensus 19]. There are isolated reports of oral TrNs secondary to
guidelines). overextended dental prosthesis [20]. In the head and neck

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Current Pain and Headache Reports

region, almost all surgical procedures have been shown to Epidemiology


induce TrN formation [21–24].
Removal of the dental pulp tissue and incision and Incidence and Prevalence
drainage of the dental abscess has also been identified
as causative for trigeminal TrN [19]. Some of the newer Prevalence of TrN has been reported with a wide range.
literature contradicts this and states that dental surger- Some studies put the prevalence of oral (trigeminal) TrN at
ies (causing intrabony lesions), rather than extractions, approximately 0.3% [6, 34]. TrN of the facial nerve is a rare
form the main etiological factor for TrN formation [18]. occurrence with a reported approximate prevalence of 0.002
Orthognathic surgeries including chin augmentation [25] to 0.8% [35]. In the head and neck region, the reported inci-
and sagittal split osteotomy have been shown to cause TrN dence of TrN is high following parotidectomy (trigeminal
[26–29] of the affected nerves. The damage to the nerve and glossopharyngeal nerve distribution), and neck dissec-
could be caused by the use of scalpels, chisels, burs, or by tions [34, 36].
the compressive forces [25, 27]. There are rare reports in
the literature of TrN being caused by miscellaneous etiol- Site Predilection
ogy such as a burn [6]. Surgical decompression procedures
involving the orbit have been shown to cause TrN of the Painful TrNs constitute one-fourth of all oral TrN cases
divisions of the maxillary nerve (V2), causing continu- [20, 28, 37]. The available literature on head and neck TrNs
ous excruciating pain [30, 31]. There is also evidence of alludes to the site predilection, with regards to the affected
infraorbital TrN due to various causes other than surgery, nerve, as inferior alveolar, lingual, and greater auricular
like bomb blast injury [32]. There are case reports of TrN nerves, in the descending order of occurrence [6]. Site pre-
occurrence secondary to facial trauma [12]. Rarely, cases dilection has been variably reported by different authors as
have been reported of unusual occurrences of trigeminal mental foramen [28] and intraosseous lesions [18]. Alter-
TrN with such procedures as a halo fixation device [33]. natively, a slightly different site preponderance referring to
The possible etiology and neuroanatomy of trigeminal TrN the tissue appears in the literature, as the submental area,
are summarized in Fig. 1. tongue, and labial mucosa [4, 38–40].

Fig. 1  Trigeminal traumatic neuroma: etiology, appearance, and gross neuroanatomy of traumatic neuroma pain

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Genetics lesions occurring on the mental nerve are reportedly most


painful, [3•] whereas some authors describe the lower lip
Recent studies done using nerve injury models have related and tongue as the most common painful sites [14, 28, 34].
certain proteins and their associated genes as either causing The specific set of symptoms depends mainly on such
TrN formation or being responsible for the pain associated variables as the nerve affected, and the location of the
with TrN [41–43]. lesion along the course of the nerve [13, 28]. The symp-
toms may include both sensory and motor, especially when
Clinical Features specific sites such as the infraorbital nerve are involved
with TrN [32]. Pain from TrN can cross the midline [3•].
In cases of trigeminal TrNs that could be palpated, and/or Pain associated with TrN has been described as lancinat-
observed, the clinical description appearing in the litera- ing, electric, burning, dull aching, or stabbing quality, [3•,
ture is that of a well-circumscribed lesion, that is neither 19, 20, 46] similar to neuralgias occurring in the distribu-
ulcerated, nor encapsulated, with a smooth surface [34, tion of the trigeminal nerve [31]. There have been rare
38]. TrN can clinically manifest with a variety of features, reports of tingling sensation in the area as well [23]. Some
often confusing the clinician. These include, but are not articles refer to sudden cessation of TrN pain, similar to
limited to, significant variations in the frequency, dura- spontaneous remission seen in cases of trigeminal neu-
tion, intensity, quality, radiation/referral, and associated ralgia [34]. However, what is unclear in these articles is
features. Tinel’s sign, defined as pain along the nerve whether the diagnosis was trigeminal neuralgia, to begin
distribution upon percussion of the lesion, is described with. The neuroanatomic pathways of pain of trigeminal
as one of the cardinal features of typical TrN [44, 45]. origin are summarized in Fig. 2. Common aggravating fac-
However, when the lesion is clinically difficult to palpate tors are applying local pressure, [6, 14, 25] percussion, and
and localize, such as in many intraoral sites, Tinel’s sign palpation of the lesion [18, 47]. Trigeminal TrN can also
may be a difficult clinical feature to elicit. TrN lesions be associated with symptoms including neurological (such
may or may not elicit pain [3•]. The incidence of pain in as anesthesia, paresthesia, and hyperpathia), autonomic
oral TrN is reported to be approximately 20–30% of the (such as redness and swelling), and others (such as dizzi-
cases [34, 38]. With regards to pain of intraoral TrNs, the ness and nausea) [7, 14, 19, 20, 48]

Fig. 2  Trigeminal traumatic neuroma: pain pathways

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Current Pain and Headache Reports

Histopathology a cotton bud or digital pressure may be of diagnostic value


[19, 27].
TrN contain multiple cut axon ends, Schwann cells, inflam-
matory cells, and dense connective tissue/collagen matrix Prevention
[2•, 35]. There are also special markers including S-100
protein, that specifically stains Schwann cells, indicating Since the inferior alveolar nerve (mandibular nerve) has a
presence of multiple myelinated axons [14, 34]. Also present relatively higher incidence of TrN occurrence, alterations
are Schwann cells and proliferating axons within dense scar and modifications of surgical techniques have been recom-
tissue [23, 49]. TrN lacks encapsulation [38, 50]. mended to minimize trauma to these nerves [29, 56–59].
It has been suggested that, when surgical removal of man-
Investigations dibular impacted wisdom teeth is planned, removal of the
crown part of the tooth (a procedure called coronectomy)
Although the confirmatory test is histopathology, the size reduces the risk of nerve trauma [56, 60, 61]. The accurate
and location of the lesion, many a time, preclude a biopsy determination of the anatomical position of the tooth/dental
procedure. There is always the inherent risk of resection implant with respect to the underlying nerve, prior to sur-
of a TrN, ending up in the formation of a new TrN lesion, gery, utilizing imaging modalities such as cone beam com-
which may be even deeper in the tissue to locate. Conse- puted tomography (CBCT) and magnetic resonance imaging
quently, reproduction of the patient’s familiar pain, elicit- (MRI) or other prudent digital imaging, can help prevent
ing the Tinel’s sign, and subsequent successful diagnostic nerve injuries [62–65]. The adjunctive techniques such as
anesthetic blocking of the lesion [46, 51, 52] may be the best diathermy and electrical coagulation have been reported
investigative method. The use of imaging such as ultrasound to prevent traumatic neuroma formation, while managing
[22] and MRI [53–55] is also a valuable diagnostic aid for peripheral nerve injuries [6, 66]. However, it must be noted
larger lesions in anatomic areas other than the trigeminal that the literature confirming the efficacy of these procedures
distribution. in TrN prevention is scanty. In addition, diathermy has also
been used successfully to guide and facilitate the desired
Differential Diagnosis regeneration of the injured nerve [67].

Based on the clinical features, pain characteristics, and other Management


features of trigeminal TrN, the most plausible differential
diagnoses include trigeminal neuralgia, painful post-traumatic The overall management of trigeminal TrNs may be divided
trigeminal neuropathy, tumor lesions that can mimic TrN, and into conservative, pharmacologic, and surgical approaches.
other neuropathic pain entities. The diagnosis is largely by As alluded to earlier, trigeminal TrN may become a difficult
exclusion, and specific clinical features of TrN, such as Tinel’s entity to manage, especially when the lesion cannot be local-
sign. The paradigm followed for diagnosis of trigeminal TrN ized sufficiently. Further, although surgical management has
is given below. been proposed as the most reliable method for managing
TrN afflicting the larger peripheral nerves (such as the sciatic
Diagnosis nerve), this method could prove extremely difficult and often
impossible when the smaller branches of trigeminal nerve
Suggested clinical criteria for TrN include post-surgical pain are involved with the lesion. In addition, there is an inher-
at the site; neurogenic type of pain; increased pain from trig- ent risk of a re-occurrence of the lesion at the newly cut end
ger zones at the site; and pain relief upon local anesthetic post-surgical excision. The currently accepted modalities
injection [19, 46]. Local anesthetic block of the lesion has of management of trigeminal TrN and of the closest most
been proposed as a rapid and cost-effective tool to aid in the common differential diagnoses are summarized in Table 1.
diagnosis and confirmation of TrN [52]. Since there is a lack
of consensus on the exact diagnostic criteria, classification, Surgical Management
and management of TrN, diagnosis becomes one of exclu-
sion of similar entities that may simulate TrN in terms of The current modalities available for the management of
clinical features. In this regard, Tinel’s sign is an important trigeminal TrN are limited and include local steroid injection
parameter. It must be noted that description of Tinel’s sign [3•, 29], local anesthetic (infiltration and topical) [114], and
involves “percussion” of the lesion, which may be difficult a combination of these and systemic medications, and surgi-
or even impossible in trigeminal nerve territories, especially cal resections [3•, 29, 127, 128]. The use of custom fabri-
in the hard to access intraoral sites. Therefore, a modified cated neurosensory stent has been proposed [114, 119, 120].
version of Tinel’s sign employing palpation/pressure with There is evidence of successful management of identifiable

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Table 1  Trigeminal traumatic neuroma: differential diagnoses and management
Management modalities Neuropathic pain in general Trigeminal neuropathic pain Traumatic neuroma in general Trigeminal traumatic neuroma

Evidence on prevention - Modification of surgical techniques - Avoidance of trigeminal nerve - Monopolar diathermy and electrical - Administration of systemic steroids
[68] injuries by: coagulation [6, 66, 71] [29]
    1. Not using high concentrations - Capping of the cut nerve end [72, - Adequately stabilized wiring
of local anesthetics especially the 73] without rigidity [29, 59]
ones that can be neurotoxic [69] - Implantation of cut nerve into the - Absolute mobilization of proximal
    2. Not employing multiple nerve adjacent muscle [72] fragments [29, 57, 59]
blocks at the same site [70••] - Local administration of chemicals - Use of external cortical plate wire
    3. Appropriate imaging to prevent such as alcohol, phenol, steroids at osteosynthesis [29, 58]
Current Pain and Headache Reports

nerve injury [69] the site of surgical trauma - Use of thin spatula osteotome [29,
- Use of lasers [72] 58]
- Triamcinolone and 5-fluorouracil
[74]
Surgical management - Neuromodulation via motor cortex - Microvascular decompression, - Surgical excision [77–79] - Surgical resection [3•, 12, 14, 25,
stimulation (MCS) and deep brain Percutaneous rhizotomy, Gamma - Microsurgery, use of nerve grafts 29]
stimulation (DBS) [75] knife radiosurgery, Neuromodulation [80, 81]
via motor cortex stimulation (MCS) - Neuromodulatory or reconstructive
and deep brain stimulation (DBS) modalities [82]
[75] - Regenerative peripheral nerve
- Radiofrequency thermocoagulation, interface (RPNI) [83–86]
Subcutaneous peripheral nerve - Targeted muscle reinnervation
field stimulation [76] (TMR) [83, 87, 88]
- Cap assisted resection [89]
Pharmacological management: - Tricyclic antidepressants (TCA) / - Steroids [97] - Antidepressants [107] Antiepileptic drugs (carbamazepine
Systemic medications Serotonin- - TCA/SNRI antidepressants [10, - Anticonvulsants [108] and gabapentin) and pregabalin
Norepinephrine Reuptake Inhibitors 97] - Calcitonin [82, 108] [114]
(SNRI) [90–92] - Anticonvulsants (gabapentin and - Calcium channel blockers [82]
- Anticonvulsants (gabapentin and pregabalin) [10, 75] - Beta-blockers [82]
pregabalin) [90, 91, 93, 94] - Botulinum toxin type A [75, - Perineural/neuraxial analgesia with
- Botulinum toxin type A [93, 95] 98–106] local anesthetic and morphine
- Opioids [91, 93] [109–111]
- Anti-arrhythmics [96] - TNF-a blockers (etanercept) [112,
113]
Topical: - Oromucosal cannabinoids [93] - Local anesthetics or 8% capsaicin - Local injections of steroids [117, - Use of neurosensory stent [114, 119,
- Capsaicin [93] patch [97] 118••] 120]
- Topical lidocaine [90, 91, 93] - Topical lidocaine [70••, 116] - Local steroid infiltration [3•]
- Topical anesthetics and
combination cream [115]

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trigeminal TrNs using surgical excision of the lesion [25,


29]. Surgical excision of the lesion may predispose to the
development of a new TrN at the fresh-cut end. Systemic
Trigeminal traumatic neuroma

percussion, ultrasound [14] medications may help manage residual pain after surgical
- Stereotactic radiosurgery,

excision [28].

Pharmacological Management

Systemic medications that belong to several classes of drugs


have been used to manage the pain of trigeminal TrN with
limited efficacies. These medications include antiepileptic
- Ultrasound-guided steroid injections

drugs (carbamazepine and gabapentin) and pregabalin [31,


114, 117, 129]. The use of custom fabricated neurosensory
neurolysis and radiofrequency
Traumatic neuroma in general

stent has been proposed [114, 119, 120].


- Ultrasound-guided alcohol
- Psychological, cognitive-behavioral - Electrostimulation [112]

Complications and Prognosis
ablation [126]

Chronic pain, often intractable, can be a complication sig-


nificantly affecting the quality of life of patients with TrN.
[125]

Many of these patients, as is in our current case, go through


multiple procedures (including surgical) resulting in either
more chronic pain and/or complications from the proce-
dures themselves [14, 130]. Irreversible dental treatments
approaches and hypnosis [97]

performed by clinicians lacking knowledge about trigeminal


Trigeminal neuropathic pain

neuropathic pain, in general, are not indicated and may often


lead to unfortunate results in many cases. Further, although
surgical excision may be one of the more prominent man-
agement modalities proposed in the literature, it should be
borne in mind that there is a significant inherent risk of a
recurrence, especially when there is a possible genetic predi-
lection, as is apparent in our current case [3•, 14, 131]. With
early diagnosis, and management by a competent clinician,
relaxation, pressure, yoga, exercise

well versed in management of neuropathic pain, trigeminal


hypnosis, biofeedback, massage,

TrN can be thought of as having a fair to good prognosis.


- Physical therapy, acupuncture,
- Cognitive-behavioral therapy,

- Spinal cord stimulation [123]


Neuropathic pain in general

- Transcutaneous magnetic
mirror therapy [91, 121]

- Electrotherapy [96, 122]

- Counseling [70••, 96]

Case Discussion in the Context


stimulation [124]

of the Literature

Clinical Presentation
[96]

Bilateral TrN lesions of the trigeminal nerve, the diagnosis


in the current patient, are rarely reported in the literature.
Ultrasound-guided phonophoresis,

The lesion is classically described as unilateral (second-


Alternative therapies (physical
therapy, massage Cognitive

ary to trauma) [132]. The time elapsed from initial onset of


symptoms to diagnosis in our current case is approximately
Management modalities

30 years, which is unusual and inconsistent with the known


behavioral therapy,
Table 1  (continued)

literature of similar lesions in other anatomic locations [107,


iontophoresis)

133–135]. The fact that the patient had developed the lesion
after surgical procedures is consistent with the available lit-
erature [18, 25–28] The pain characteristics of the current
case, as they relate to the literature, are given below.

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Onset is inconsistent with the published literature of TrN, which


reports a higher frequency of shooting, tingling, electric, and
Many authors report the onset of symptoms to be ranging burning types of pain [3•, 18, 29, 112].
from soon after the trauma of a surgical event has occurred,
to several weeks after the same [5]. In our present case,
Temporal Characteristics
there was no such definite temporal pattern reported by the
patient. TrN of other anatomic sites have been reported to
Although the slow chronic progressive nature of this
become symptomatic months following the surgical event
patient’s case is consistent with much of the neuropathic
[136].
pain literature, the 30-year chronicity of this pain is unusual
[19, 28].
Location

Consistent with the literature, this case exemplifies TrN of Aggravating Factors/Triggers
the terminal branch of V2 and V3 divisions of the trigem-
inal nerve [6]. However, the occurrence of two anatomi- The triggering of the pain in the present case, by functions
cally independent lesions in the same patient (left and right such as chewing, talking, and jaw movements, is consistent
sides and differing branches of the trigeminal nerve) is a with the current literature [18, 19, 28, 29, 112, 138]. How-
unique feature of this case that is largely inconsistent with ever, the aggravation of pain by acidic foods, citrus fruits,
the established TrN literature. Considering the variability and sleep position is rather unusual.
of clinicians and procedures between the two sites/sides,
we the authors strongly feel that there might be a genetic
Relieving Factors
predisposition in this patient to TrN formation. Genetic pre-
disposition to neuropathic pain has been published in the
The lack of relieving factors for the background pain (as
literature [137].
was in this case), and relief achieved by complete rest to the
affected areas (thereby avoiding triggers), and by the use of
Frequency topical anesthetics are consistent with the current literature
on trigeminal TrN [120].
We could not find pertinent focused literature on the nature
of the background pain or frequency of pain associated with
trigeminal TrN. Response to Past Treatment

The fact that our current patient had no relief from pain
Duration
even after multiple dental procedures is consistent with the
published literature [14, 130]. The lack of response to mul-
The continuous nature (“hours”) of the background pain in
tiple drugs, although part of published literature, the specific
this particular case is in agreement with past literature on
absolute lack of pain relief upon trial of anti-seizure and
similar TrNs in other anatomic areas of the body [28, 107].
SNRI medications (gabapentin, carbamazepine, nortriptyl-
ine) seems consistent with the literature [117].
Intensity

Intensity of the pain varying from five to six (moderate) Associated Features
on a numerical pain scale of zero to ten is at par with the
literature for oral and extraoral sites of TrN [19]. The seven As different from this case, associated features of skin/
out of ten pain (severe) for the exacerbated episodes is also mucosal changes, paresthesias, dysesthesias, and auto-
consistent with much of the published literature for TrN in nomic features have been reported in the literature [14, 19,
trigeminal and other nerve distributions [28, 29, 107]. 29, 139–141].

Quality Radiation/Referral

The description of dull aching background pain goes along Referral pattern in this patient’s case goes along with the
with the published literature in the oral and extraoral sites of published literature of pain in the distribution of the affected
TrN [29]. However, the pulsating quality of exacerbated pain nerve [8, 29, 142, 143].

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Association with Sleep trigeminal TrN has been established, the clinician should opt
for conservative modalities such as topical pharmacotherapy,
The lack of association with sleep disturbances, as observed possible systemic pharmacotherapy, and adjunct modalities,
in this case, is not consistent with the current literature on thereby avoiding aggressive surgical treatments and possibil-
neuropathic pain [19]. ity of recurrence.

Investigations
Conclusion
The size of the TrN lesion in this case as elicited clinically is
consistent with the literature on trigeminal TrN [34]. Repro- Traumatic neuromas in general, and trigeminal TrNs in
duction of patient’s familiar pain from the local TrN lesion particular, present with varied clinical features, and may
is one of the clinical methods to confirm the location of pose a diagnostic challenge to the clinician. TrN is a neuro-
the lesion. Specifically, eliciting a Tinel sign is considered pathic pain condition that results from attempted repair by
one of the clinical diagnostic features of TrN in the other an injured nerve. When TrN occurs in the trigeminal nerve
anatomic areas of the body [44, 45, 121]. The pain relief distribution, they present as small, non-encapsulated, often
obtained in this case by both topical and infiltration of local non-palpable lesions making it difficult to palpate. The diag-
anesthetic goes along with the published literature [46, 52]. nosis of this neuropathic pain entity is often by exclusion of
other similarly behaving entities and eliciting classic features
Management such as Tinel’s sign. Surgical resection, although may be a
choice, may have limited value due to the relative inability
Specific guidelines for the management of TrN lesions in to locate the lesion and probably running the risk of a recur-
general, and trigeminal TrN lesions in particular, are lacking. rence of the entity. Systemic medications may help; how-
TrN is considered a neuropathic pain entity [2•, 107, 144]. ever, topical medications form the choice of management,
Conceivably, management modalities that work for neuro- due to reduced risk of side effects and better efficacy from
pathic pain in general may work very well for TrN lesions localized application. Further, the use of custom fabricated
as well. One of the medications used in management of neu- delivery methods, such as a stent, further increases the effi-
ropathic pain conditions similar to TrN is topical lidocaine cacy by successfully retaining the medication locally for a
[145, 146]. In an attempt to retain the topical medications longer time. There may be a genetic predilection for patients
such as lidocaine at the site of the lesion, a custom fabricated to form TrNs subsequent to incidental or surgical trauma.
dental stent may be utilized [114]. There is a necessity for consensus amongst the pioneer pain
management organizations as to diagnostic criteria, manage-
Clinical Pearls ment protocols, and classification of TrNs.

For clinicians who manage pain, there are certain take-home


pearls from the case and the review. We strongly believe that Author Contribution  Davis C. Thomas: conceptualization, investi-
awareness and education for all clinicians (dental, medical, gation, methodology, writing/reviewing/editing, analysis. Saranya
Devatha Mallareddy: writing/reviewing/editing, literature search.
allied health, and specialists) are paramount in the early rec-
Jeffrey P. Okeson: writing/reviewing/editing, literature search. Josna
ognition and succinct management of trigeminal TrN. This Thankachan: writing/reviewing/editing, literature search. Priyanka
definitely would help improve quality of life for the patient, Kodaganallur Pitchumani: writing/reviewing/editing, literature search.
help avoid unnecessary procedures, and bring optimal pain Reshmy Chellam Pichammal: writing/reviewing/editing, literature
search, investigation, methodology, medical illustrations.
relief to the patient. An interdisciplinary team consisting
of physicians, pain management specialists, dentists, neu-
rologists, and orofacial pain specialists is the best hope for Compliance with Ethical Standards 
patients with difficult-to-diagnose entities such as trigemi-
Conflict of Interest Davis C Thomas, Saranya Devatha Mallareddy,
nal TrN. The clinician should also understand that there Priyanka Kodaganallur Pitchumani, Reshmy Chellam Pichammal,
are other entities that may mimic signs and symptoms of Josna Thankachan, and Jeffrey P Okeson declare no conflict of interest.
trigeminal TrN, and the diagnosis, many a times, is by exclu-
Human and Animal Rights and Informed Consent  This article does not
sion. The astute clinician should recognize the entity, and
contain any studies with human or animal subjects performed by any
make a timely, prompt, appropriate referral to the specialist of the authors.
for management of trigeminal TrN. After the diagnosis of

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Current Pain and Headache Reports

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