Nontraumatic Head and Neck Injuries: a Clinical Approach. Part 2 183

Radiología. 2017;59(3):182---195

www.elsevier.es/rx

UPDATE IN RADIOLOGY

Nontraumatic head and neck injuries: A clinical

ଝ approach. Part 2

B. Brea Álvarez , L. Esteban García, M. Tunón˜ Gómez, Y. Cepeda Ibarra

Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, Spain

Received 3 May 2016; accepted 16 February 2017

KEYWORDS Abstract Nontraumatic emergencies of the head and neck represent a challenge in the ﬁeld of

Emergencies; neuroradiology for two reasons. As explained in the ﬁrst part of this update, these entities affect

Neck injuries; an area where the thorax joins the cranial cavity and can thus compromise both structures;

Orbital diseases; second, they are uncommon, so they are not well known.

Diseases of the Maintaining the same approach as in the ﬁrst part, focusing on the clinical presentations in the

paranasal sinus; emergency department rather than on the anatomic regions affected, we will study the entities

Sialadenitis; that present with two patterns: those that present with a combination of cervical numbness,

Cellulitis; dysphagia, and dyspnoea and those that present with acute sensory deﬁcits. In the latter group,

Diagnostic imaging; we will speciﬁcally focus on visual deﬁcits, because this is the most common symptom that calls

Computed for urgent imaging studies.

Magnetic resonance

imaging

PALABRAS CLAVE Urgencias no traumáticas de cabeza y cuello: aproximación desde la clínica. Parte 2

Urgencias;

Resumen Las urgencias no traumáticas de cabeza y cuello son un reto en el campo neurorra-

Lesiones del cuello;

Enfermedades diológico por los motivos referidos en la primera parte: su área de afectación, en la encrucijada

orbitarias; del tórax y la cavidad craneal, y su baja incidencia en la urgencia, lo que supone que sean poco conocidas.

Enfermedades del

Manteniendo el mismo enfoque que en la actualización previa, a partir de las formas clínicas

seno paranasal;

Sialadenitis; de presentación en el ámbito de la urgencia, en lugar de la división por regiones anatómicas

Please cite this article as: Brea Álvarez B, Esteban García L, Tunón˜ Gómez M, Cepeda Ibarra Y. Urgencias no traumáticas de cabeza y

cuello: aproximación desde la clínica. Parte 2. Radiología. 2017;59:182---195. ∗

Corresponding author.

E-mail address: [email protected] (B. Brea Álvarez).

Nontraumatic head and neck injuries: A clinical approach. Part 2 183

estudiaremos las entidades que se presentan con patrones que combinan tumefacción cervical,

Cellulitis; disfagia y disnea, y los déﬁcits agudos de los sentidos. Dentro de este último grupo, el síntoma

Imagen diagnóstica; al que haremos referencia especíﬁca será el déﬁcit visual, puesto que es el que de forma más

Tomografía frecuente requiere estudio radiológico urgente.

Resonancia Magnética

Introduction performing one cervical computed tomography (CT) scan

including the carina and even the whole thoracic cav-

ity. The anatomic continuity of retropharyngeal spaces

Nontraumatic head and neck emergencies take over a small

(retropharyngeal space and danger space) up to the lower

portion of the routine radiological day; however, they are

1 mediastinum allow the spread of cervical processes towards

very important. As Brucker et al. claim, this anatomic

the thorax. The use of one single X-ray might be applica-

region contains few structures that can be dispensable. After

ble in cases of young individuals with sudden onsets of this

analyzing the images and taking the clinical data into con-

clinical combination and associated cervical crepitus to be

sideration, the role of the radiologist should be deﬁning the

able to rule out a clinical manifestation as uncommon as

location and spread of the processes, identifying all those

spontaneous emphysema.

radiological signs indicative that the patient might be vitally

In sensory deﬁcits only the visual deﬁcit is included. Taste

compromised, or that might condition his/her treatment,

and smelling deﬁcits will not be studied here because they

and establishing differential diagnosis.

are not as invalidating as the visual deﬁcit, and for the

As we said in Part 1, in most papers, emergencies are

patient they are not as alarming as to go to the emergency

classiﬁed according to the region affected by the patholog-

room. Sudden hearing loss usually occurs within a 12 hour-

ical process, but since the patient presents to the hospital

span and implies the loss of ≥30 dB as conﬁrmed in at least

with clinical symptoms or signs, an approach from this point

three frequency tests conducted during the ﬁrst 72 h. It has

of view is interesting. In this review, we will be dealing with

a powerful impact in the patient’s life, above all in associa-

how radiological management is accomplished, and with the

tion with tinnitus and vertigo, occurring in 90 and 20---60% of

clinical characteristics of those entities that present with

cases, respectively. Its study in the acute phase requires

combined clinical manifestations of cervical tumefaction,

performing one otoscopy and one audiometry in order to

dysphagia, and diarrhoea, and we will also be dealing with

rule out any underlying causes and conﬁrm its diagnosis,

those patients who present to the hospital with acute sen-

and all this is the responsibility of the ENT unit. Although

sory deﬁcits.

included in the diagnostic armamentarium of this entity,

However, this paper will not deal with all the possible

the magnetic resonance imaging (MRI) is never performed

emergency entities that have such clinical manifestations.

in emergent situations.

This is why we chose the most common ones, tried to estab-

lish differential diagnoses, set the record straight on some

concepts and, same as we did in Part 1, emphasized the

Cervical tumefaction and dysphagia-dyspnoea

clinical manifestations that are less common but with char-

acteristic radiological images that will help the radiologist

Abscesses of the oral cavity and the mouth ﬂoor

achieve a correct diagnosis yet despite the rarity of such

clinical manifestations.

Most head and neck infections have an odontogenic origin.

The dental inﬂammatory disease may have an endodontal or

Nosological division and imaging modalities periodontal origin. The origin of endodontal infections may

be found in one dental cavity that progressively destroys

There are four clinical situations in nontraumatic head and the dentine, then the dental pulp, and ultimately the den-

neck emergencies: cervical tumefaction cervical, dyspha- tal nerve canal. This is how the infection reaches the dental

gia, dyspnoea, and sensory deﬁcit. The causes responsible apex while making up one granuloma or apical abscess. In

for these clinical presentations may be of inﬂammatory- the periodontal disease, the infection starts as gingivitis that

infectious, tumour or vascular origin. In this Part 2 we will progresses along the periodontal ligament while making up

be looking into those clinical manifestations that combine one periodontal abscess. From the radiological standpoint,

facial tumefaction, dysphagia, and dyspnoea, as well as sen- apical or periodontal abscesses look like radiolucent cavities

sory deﬁcits. surrounding the tooth. The abscess may rupture the max-

The combination of cervical tumefaction, dysphagia, illary or mandibular cortical bones and depending on the

and dyspnoea is not rare. It has been reported in oral dental piece affected, progress following variable location

cavity-mouth ﬂoor infections, Ludwig’s angina, necrotiz- and spread.

ing fasciitis, in collections of retropharyngeal abscesses, Infections that originate in the maxillary bone may

and spontaneous emphysemas. These situations require spread towards the mouth, the masticator space or the

184 B. Brea Álvarez et al.

Figure 1 Abscesses of the oral cavity. Abscess in the oral vestibule space. (A) Computed tomography (CT) scan, axial cut recon-

structed in bone window. (B) CT scan, sagittal reconstruction in bone window. (C) CT scan, axial cut reconstructed in soft tissues.

Female with clinical manifestations of neuralgia and anterior facial tumefaction. The CT scan shows periodontal affectation in the

premaxillary region (arrows in A and B), dental pieces #21 and #22---showing metallic material due to prior endodoncy---in the form

of periapical abscess with phlegmonous affectation of the oral space and the vestibule (short arrows in C). Submandibular abscess.

(D) CT scan, coronal cut with intravenous (IV) contrast. (E) CT, oblique axial reconstruction in bone window. (F) CT scan, axial

with IV contrast. Sixty-two year old-male with left submandibular pain. In the CT scan there was one collection with peripheral

enhancement (abscess) in the left mouth ﬂoor (asterisk in D and F). The infectious focus was located in dental piece #38 (arrow in

E) showing one periapical abscess with dehiscence of the lingual cortex.

parapharyngeal space, depending on whether the patho- Dental Federation (WDF) that numbers teeth as 11---18,

logical pieces are located in the pre-maxillary region, or 21---28, 31---38 and 41---48, the corresponding pieces to the

the second or third molars, respectively. In infections of right and left maxillary dental arches, and the left and right

mandibular origin, the inﬂammatory processes of the ante- mandibular dental arches, respectively, starting from the

rior dental pieces are found in the sublingual space, and ﬁrst incisive until the last molar in each of the quadrants.

when infections affect the second and third molars, the Nomenclature from the World Dental Federation (WDF)

inﬂammatory processes will be found in the submandibular numbers dental pieces from 1 to 32, starting from the right

space, since the roots of these molars are located under- upper arch third molar and continuing successively with the

neath the mylohyoid line. remaining quadrants following the WDF system.

Other than for dental abscess identiﬁcation, CT scans

also conﬁrm the presence of ﬂuid collections with periph-

eral enhancement. The location and spread of these ﬂuid Ludwig’s angina

collections should appear in the radiological report since

this is essential to guide surgical drainage. Also, the It is a speciﬁc and serious type of cellulitis occurring in the

affected dental piece should be identiﬁed (Fig. 1). The mouth ﬂoor and spreading bilaterally towards the soft tis-

nomenclature used to deﬁne the affected tooth speaks sues of the oral cavity and deep spaces of the neck, and at

of its name (incisive, canine, premolar, and molar) and the same time it implicates the muscles located between the

location at the quadrant of the mouth (upper: right or mouth ﬂoor and the larynx (Fig. 2). Clinically, it presents

left, lower: right or left). Following one numerical pattern, as pain and tumefaction at the mouth ﬂoor and can asso-

the most widely used method in Spain is that of the World ciate chest pain. Before the era of antibiotics, the spread

Figure 2 Ludwig’s angina. (A) Computed tomography (CT) scan, coronal cut with intravenous contrast. (B) CT scan, axial cut with

IV contrast. Eighteen-year old patient who presented with odynophagy and (dis) thermal sensation of three day duration. During

the medical examination, he had difﬁculties opening his oral cavity and showed over-elevation of his tongue. The image conﬁrmed

an increase in the fat density of submandibular and parapharyngeal spaces (arrows in A and B). (D---H) CT scan with IV contrast in

a different patient (C) who presented to the hospital with cervical tumefaction. (D) Sagittal cut. (E and G) Axial cuts. (F) Coronal

cut. (H) Oblique sagittal reconstruction. At the beginning (C---E), the CT scan showed an increase of fat density and trabeculation in

the submandibular space (arrow) associated with small collections in the mouth ﬂoor (arrowheads). The patient is admitted to the

hospital with a diagnosis of Ludwig’s angina and IV antibiotic therapy. The patient worsens and another CT scan is performed (F-H)

showing ﬂuid collections (long arrows) in the mouth ﬂoor with clear oversize with respect to the initial study. Affectation spread

along the digastric muscle posterior stomach (curved arrow in G and dotted line in H), which was oversized (compare with the left

side one, black curved arrow). Drainage showed seropurulent content.

of this process towards the mediastinum was far more com- In the CT scan, the radiological signs that help us estab-

mon and, therefore, responsible for the denomination given lish its diagnosis are the evidence of cellulitis, fasciitis,

to this entity. It may affect the airways and require tra- myositis, multiple collections, reactive adenopathies, and,

cheostomy more commonly than other cervical abscesses. on some occasions, septic thrombosis (Fig. 3). The presence

The CT scan is used to assess the spread of inﬂammatory of gas (in the absence of recent surgery or radiotherapy) is a

process, the presence of blood collections, or the formation highly suggestive ﬁnding of this entity, though this does not

12,13 17

of abscesses that can occur in some cases. occur in all cases.

Necrotizing fasciitis Spontaneous emphysema

Necrotizing fasciitis is one aggressive polymicrobial infec- The spontaneous cervical emphysema is deﬁned as the

tion of the superﬁcial and deep soft tissues of the neck presence of gas in cervical and mediastinal spaces that

usually occurring in patients with comorbid situations like is produced spontaneously in young adults and that is of

diabetes or immunosuppression. Infection can occur in the self-limiting an benign character. Clinically, it appears as

skin, the mucosa or the teeth, progress rapidly and reach the an intense sudden retrosternal pain associated with dysp-

mediastinum and compromise the life of the patient. Clin- noea of sudden onset. There is no relation whatsoever with

ically, patients show symptoms of cervical tumefaction and iatrogenia, trauma or infection. The underlying physiopa-

dysphagia-dyspnoea associated with high fever. There are thological mechanism is pressure increase in alveoli (caused

certain lab indicators of risk for developing necrotizing fasci- by multiple etiologies: cough, Valsalva, etc.) that causes its

itis based on routine lab tests. Such indicators are used to rupture. The air progresses in a centripetal motion towards

distinguish this entity from other less aggressive infections the mediastinum since there is less pressure there. From

of soft tissues in order to be able to early implement the the radiological standpoint, there are air bubbles of ran-

treatment of choice: emergent surgical debridement. dom distribution both in the different cervical spaces and

Figure 3 Necrotizing fasciitis. (A) Sagittal computed tomography (CT) with IV contrast. (B---D) Axial CT scan with IV contrast at

different cervical levels. Seventy-four year old-patient who presents to the ER with clinical manifestations of dyspnoea, odynophagy,

and fever. The patient is then referred to the ENT who conﬁrms saturation levels at 87% and triage and retractions. The image

shows thickening of the skin and the subcutaneous cellular tissue---celulitis (arrow in D), thickening and enhancement of superﬁcial

and deep fascias---fascitis (arrowhead in D), thickening and enhancement with ﬂuid collections of prelaryngeal muscles---miositis

(discontinuous arrow in D), swelling of the pharyngeal mucosal space (black arrows in B), and swelling of the whole retropharyngeal

space (dashed line in A and B) spreading towards the mediastinum (asterisk in C). Also, there was presence of gas (curved arrow in

D) and right pleural effusion (arrow in C).

the mediastinum (Fig. 4A and B). We should distinguish here the upper mediastinum. There is another virtual space,

between Boerhave’s syndrome and esophageal rupture sec- posterior to the retropharyngeal one, called danger space

ondary to vomit --- one clinical manifestation associated with that connects to the posterior mediastinum and descends

greater morbimortality that requires surgical management. towards the lower part of the thoracic cavity. The distinc-

The spontaneous emphysema should not be confused with tion between these spaces at cervical level is not possible

the presence of small quantities of air in the cervical spaces and it is its caudal spread that deﬁnes if the inﬂamma-

that can be incidentally observed in CT scan (Fig. 4C). It has tory process occurs in one or the other (spread beyond

been reported in 0.034% of CT scans of the brain performed T3 would be indicative of danger space affectation). The

in the emergency room to patients with one peripheral implication of any of these spaces is usually secondary to

route considered the air access point. The location of an infection originated in the upper aerodigestive tract,

the air bubbles happens at random; it is more common in whose pattern of lymphatic drainage is in the nodes of

the orbital veins, the masticator space, or the cavernous the retropharyngeal space. The infectious process promotes

sinuses. Patients with this ﬁnding do not have a history of adenitis and inﬂammatory changes in the adjacent tissue.

trauma or other relevant data. Adenitis manifests itself as an oversized lymphatic node.

The inﬂammatory affectation of the adjacent soft parts or

cellulitis appears radiologically as a symmetrical increase

Retropharyngeal and pre-vertebral infection of the retropharyngeal space that is occupied by a layer

The retropharynx or posterior space to the pharynx is a vir- of ﬂuid that is usually no thicker than a few millime-

tual space that spreads from the base of the skull towards tres (Fig. 5A---C). Poorly treated adenitis usually suppurates,

Figure 4 Spontaneous cervical emphysema. (A) Simple lateral X-ray. (B) Computed tomography (CT) scan with coronal recon-

struction. (C) Axial CT scan. (A and B) Spontaneous neumomediastinum in one young patient who presents to the ER with clinical

manifestations of retrosternal pain, sensation of dyspnoea, and odynophagy after choking. There is presence of cervical emphysema

(arrows) spreading towards the mediastinum---a very recognizable ﬁnding in the simple X-ray. The CT scan was performed in order

to distinguish it from Boerhave’s syndrome. (C) CT scan of the brain of one patient with suspicion of stroke who is a carrier of

one peripheral route. In the image there was presence of air in the masticator space (white arrows), in the anterior fascial vein

(arrowhead), in the epidural space, in anterior portion of the foramen magnum (discontinuous arrows).

ruptures and originates abscesses. In this situation, there is X-ray, the pathognomonic signs, and after observing in one

a greater ﬂuid collection that happens to be asymmetric and CT scan, the presence of dense image calciﬁcations (not

with variable parietal enhancement (Fig. 5D and E). Suppu- bony) in the pre-vertebral-retropharyngeal space at C1---C2

rated adenitis represents a relatively common complication level, associated with soft tissue swelling, that spreads from

of secondary formation of retropharyngeal abscesses in cervical level of C1 to C4.

children.

The pre-vertebral space is that space deﬁned by the

deep cervical fascia where pre-vertebral musculature can Sensory deﬁcit

be found. The infectious process of the spine, spondylodisci-

tis, may reach this space and are usually responsible for The acute loss of eyesight is studied by taking two factors

its affectation. Spondylodiscitis occurs due to direct into consideration: for how long has the loss been hap-

inoculation (traumatic or surgical), spread of an adjacent pening, and what is the segment responsible for the visual

infection or due to the hematogenous dissemination of system.

pathogen germs. The most common causal agent is Staphy- The duration of the visual loss may be transient or persis-

lococcus aureus and it is usually located in the thoracic tent. The transient duration is deﬁned as a visual loss lasting

or lumbar regions. The MRI is the most sensitive imaging less than 25 h and usually due to one vascular occlusion

modality for its study since it accurately deﬁnes spinal disc affecting the eyeball or the visual cortex, or being secondary

affectation and all possible pre-vertebral or epidural collec- to epileptic seizure or migraine. Persistent visual losses last

tions (Fig. 6). more than 24 h and are usually due to transient ischaemias.

There exists a rare entity, probably because it is misdi- Whichever the duration of the visual loss is, it should be

agnosed, called calciﬁc tendonitis of the longus colli muscle assessed by one ophthalmologist, who, if necessary, will

(Fig. 7) that can also implicate the pre-vertebral space too. be conducting imaging modalities for diagnostic purposes.

It is caused by the deposit of calcium hydroxyapatite in the The transient visual loss or unilateral amaurosis fugax is

tendon of the long muscle of the neck (longus colli mus- suspicious of a carotid origin as the cause for the arterial

cle). This anomalous deposit originates one inﬂammatory occlusion. In this case, we should conduct one Doppler ultra-

response to foreign bodies. The diagnosis of this clinical sound, one angio-CT scan, or one angio-MRI. The loss of

manifestation is achieved after observing, in one simple bilateral vision, above all in the elderly, is associated with

Figure 5 Infection of the retropharyngeal space. (A) Magnetic resonance imaging (MRI), sagittal cut T2-weighted imaging with fat

saturation. (B) MRI, axial T2-weighted imaging. C) Computed tomography (CT) scan, axial cut. (D) CT scan, sagittal reconstruction

after the administration of IV contrast. (E) CT scan, axial cut after the administration of IV contrast. (A---C) Female with cervical

pain, fever, and odynophagia. In the MRI we saw hypertrophy of the mucosal space of pharyngeal tonsils---amigdalitis (white arrows

in A and B), one left retropharyngeal adenitis (black arrow in B), and retropharyngeal swelling (celulitis) (dashed line in A and

C). Swelling grows bilaterally in the retropharyngeal space and barely distends to space of a few millimetres. (D and E) Fifteen

year old-female diagnosed with mononucleosis who presents with high fever, signiﬁcant odynophagia, and trismus. The CT scan

conﬁrmed the presence of one asymmetric ﬂuid collection with peripheral enhancement in the retropharyngeal space, suggestive

of an abscess (dashed line arrow), that was conﬁrmed after aspiration and drainage.

vertebrobasilar symptoms and requires performing one CT common neural visual patterns in our daily radiological rou-

scan and/or one MRI for the assessment of the encephalic tine which, though rare, can be interpreted to then maybe

parenchyma and the vascular structures of posterior circu- establish the correct diagnosis (Table 1).

lation.

If we study the visual segment responsible for the visual

loss, we should remember that the luminous stimulus should Inﬂammatory optic neuritis and acute ischaemic optic

run across the different components of the eyeball, continue neuritis

along the optic nerve, then the intracranial visual pathways, The clinical manifestations of inﬂammatory optic neuritis

to eventually reach the occipital cortex. Therefore, this (ION) and acute ischaemic optic neuritis (AION) are well

condition may be divided into three different segments: the established. The ION is the most common cause of optic

eyeball (except for the retina), the retina and neural visual nerve disease in young adults, while the AION is the most

patterns. rare aetiology in the elderly.

The alterations in the eyeball and the retina are assessed ION is characterized by a progressive loss of visual acuity,

by ophthalmologists, and such alterations do not usually and can occur in a matter of hours or days, and the follow-

require any additional images. It is the issues in the neural ing are very characteristic presentations: dyscromatopsia,

patterns that may require additional radiological studies. reduced sensitivity to contrast, and pain with eye move-

Setting the amaurosis fugax aside (except for the image ments during the ﬁrst 5 days. Visual acuity improves in most

of ischaemic optic neuritis to be able to distinguish it from cases during the ﬁrst month. Patients are usually young

inﬂammatory optic neuritis), we wanted to use this review women in their twenties or forties, and in many times it

24---26

to talk about all those clinical manifestations of the most is associated with multiple sclerosis.

Figure 6 Spondylodiscitis. (A and B) Lateral X-ray of the spine. (C) Magnetic resonance imaging (MRI), T2-weighted sagittal cut.

(D) MRI, T1-weighted sagittal cut enhanced with gadolinium. (E and F) MRI, T1-weighted axial cuts enhanced with gadolinium at

C2 level (E) and C6 level (F). Patient presents with neck pain and undergoes ones lateral X-ray (A). The X-ray shows reduction of

intervertebral spaces and mild erosion of C5---C6 and C6---C7 discs that is interpreted as degenerative changes. Twelve (12) days

later the patient presents to the hospital with more intense pain. Another X-ray is performed (B) that conﬁrms clear progression

with loss of height in vertebral bodies C5 and C6 and greater enhancement of pre-vertebral space (double arrow in B). Since

spondylodiscitis was suspected, one MRI is performed that conﬁrms such condition as well as the existence of one pre-vertebral

collection (long arrows in C) with intense enhancement after the administration of gadolinium (arrowheads in D and F). There was

also one collection in the epidural space (dashed line in D). In the location of greater affectation, that is segment C5---C6, there was

an oversized pre-vertebral musculature with increased uptake (black curved arrows in F) that was not present in upper levels like

C2 (white curved arrows in E).

AION is characterized by a sudden loss of visual acuity or In the arteritic variants of AION, we should rule out

by a campimetric defect, usually painless with eye move- giant cell arteritis (GCA). This disease is the most com-

ments. Forty two per cent of patients notice some visual mon systemic vasculitis. It may cause anterior or posterior

defect when they wake up. The loss of visual acuity is affectation of the optic nerve. The visual loss usually hap-

maintained though time, though it may improve during the pens in one eye and is transient, yet in 15---20% of the

ﬁrst 6 months. These patients are usually above 50 years old cases it can be permanent or bilateral; the latter clini-

and associate cardiovascular risk factors. AIONs are divided cal manifestations are more common if diagnosis is not

into anterior, those affecting the optic nerve papilla, poste- achieved or early treatment with corticoids is adminis-

rior or retrobulbar. They may be of arteritic or nonarteritic tered. This entity is suspected in elderly patients, with

origin. visual loss in one eye that characteristically associate clau-

However, even though there are different clinical mani- dication of the jaw, rheumatic polymyalgia, and elevated

festations between both entities, there are times that these values of C-protein and speed of sedimentation. From the

characteristics overlap, and then additional imaging modali- pathogenic standpoint, this condition affects the aorta, the

ties are necessary to help us achieve the correct diagnosis. supra-aortic vessels, and the epicranial superﬁcial arteries

In the ION an enlarged optic nerve may be seen in the emer- like the superﬁcial temporal artery and the occipital artery.

gent/urgent CT scan of the brain, although there is no The biopsy with histopathological analysis conﬁrming the

doubt that the MRI is the most sensitive imaging modality presence of one granulomatose and lymphocytic inﬂamma-

to help us distinguish between these two entities. Added tory affectation of the arterial wall is the best diagnostic

to the possible enlargement of the optic nerve, this modal- test. However, there are studies that show that the high-

ity may also show contrast uptake in the T1-weighted image resolution MRI with contrast can be a promising noninvasive

with gadolinium, and hypersignal and poor deﬁnition in the diagnostic technique. With this modality we can see thick-

T2-weighted image of the optic nerve in the ION (Fig. 8A and ening and enhancement after the administration of contrast

B). In the AION, the optic nerve shows normal thickness, yet at parietal-periadventitial level in the superﬁcial and occip-

it may show hypersignal diffusion at optic nerve papilla level ital temporal arteries. Affectation of intracranial arteries

(in the anterior variants) more commonly than in the cases has also been reported, such as affectation of the inter-

of ION (Fig. 8C---E). nal carotid artery and vertebral arteries, but compared to

Figure 7 Calciﬁc tendonitis of the longus colli. (A) Magnetic resonance imaging (MRI), sagittal cut T1-weighted imaging. (B) MRI,

sagittal cut T2-weighted imaging. (C) MRI, axial cut T2-weighted imaging with fat saturation. (D) Computed tomography (CT) scan,

sagittal reconstruction in bone window. Patient presents with neck pain. One MRI was performed that conﬁrmed pre-vertebral space

swelling (arrow in B and dashed silhouette in C). Also, there was signiﬁcant hyposignal anterior to the odontoid apophysis (dashed

arrow in A and B) that in the CT scan was conﬁrmed as a deposit of calcium (dashed arrow in D).

Table 1 Differential diagnosis of the entities responsible for visual deﬁcit.

Entities Epidemiology Clinical Findings Progression

manifestations

ION ♀ 20---40 years old Loss of visual acuity Oversizing and Improvement

in hours or days. alteration of optic during the ﬁrst

Dyscromatopsia. Pain nerve month. 25% MS

AION >50 years old 42---73% show visual Optic nerve of normal Persistent visual

Cardiovascular risk defects when waking size loss (>6 months)

factors up. Absence of pain Reduced ADC

OID >50 years old Early signs of pain Inﬂammatory Improves with

and diplopia affectation of the cortocoids. Assess

covers/layers of the possible IgG4

eye, orbital fat, disease

lacrimal gland

Scleritis, uveitis At any age Pain and visual loss ULT: Identiﬁes an It may progress

increased into

echogenecity of the endophtalmitis

covers/layers of the

Infectious orbital At any age Swelling, headache Sinus occupation Antibiotics or

disease surgery: this is

how most cases

resolve

ADC: apparent diffusion coefficient; ULT: ultrasound; OID: orbital idiopathic inflammatory disease; MS: multiple sclerosis; ION: inflam-

matory optic neuritis; AION: acute ischaemic optic neuritis.

Figure 8 Optic neuritis. (A) Computed tomography (CT) scan of the brain, axial cut. (B) Magnetic resonance imaging (MRI), coronal

cut, T2-weighted imaging with fat saturation. Twenty-three year old-female who presents to the hospital with visual loss and pain

in her right eye. The CT scan conﬁrms an increased calibre of the optic nerve (arrow in A). The MRI better deﬁnes oversized optic

nerves showing hypersignal and poor deﬁnition of bulbar covers (arrow in B). Acute ischaemic optic neuritis. (C) CT scan of the

brain, axial cut. (D) MRI, axial cut T2-weighted imaging with fat saturation. (E) MRI, axial cut diffusion-weighted imaging. Sixty-two

year old patient presents to the ER with visual loss in left eye. The CT scan of the brain did not show any signiﬁcant alterations at

encephalic parenchyma or orbital levels. In the MRI, the optic nerve showed normal thickness and deﬁnition. The diffusion-weighted

imaging, however, showed hypersignal in the left optic nerve papilla (arrow in E).

the high sensitivity and speciﬁcity of extracranial vessels, this entity: serum IgG4 levels >135 mg/dL, over 40% IgG4

sensitivity to intracranial affectation is low. positive plasma cells, and presence of over 10 cells per

high-magnification field in the biopsy sample. In the field of

Orbital idiopathic inﬂammatory disease neuroradiology, at orbital level it is expressed as an over-

Like we mentioned in Part 1, under this epigraph we ﬁnd sized lacrimal gland or inﬂammatory pseudotumor, or as

conditions causing acute orbital inﬂammation usually asso- hyperthrophic pachymeningitis. As an inﬂammatory pseudo-

ciated with pain with no infectious aetiology. Hence, in this tumor it can give rise to coronal or extracoronal masses,

nosological group we ﬁnd entities such as Wegener’s granulo- but we should not forget that most inﬂammatory pseudo-

matosis, GCA, inﬂammatory pseudotumor, and IgG4-related tumors are not part of the clinical spectrum of IgG4. The

disease. They appear as inﬂammatory or oversized soft tis- oversized calibre of trigeminal nerve branches, especially

sues masses and poor deﬁnition of the eye structures, which the infraorbital nerve, is one piece of information that is

poses differential diagnosis with other conditions such as typical of this entity. There are studies that claim that

orbital cellulitis or dysthyroid orbitopathy (Table 2). inﬂammatory pseudotumors as somehow associated with the

IgG4-related disease is one relatively recent entity IgG4-related disease of the trigeminal nerve in the form of

denomination. It is characterized by elevated concentra- soft tissue masses that are homogeneously enhanced and

tions of serum IgG4 and tissue tumefaction or inﬁltration affect the base of the skull along the trigeminal V2 and V3

due to IgG4 positive plasma cells. In the histological study divisions.

we can see several IgG4 positive plasma cells, ﬁbrosis, and The diagnostic criteria of the different entities respon-

obliterative ﬂebitis. It is an autoimmune disease that usu- sible for orbital idiopathic inﬂammatory disease (OID) are

ally progresses during long periods of time, affecting one established based on clinical, analytical and anatomopatho-

or several organs, and usually responding to corticoids. In logical data. In images, inﬂammatory injuries with more

2010, Umehara et al. established the diagnostic criteria of or less characteristic locations depending on the entities,

Figure 9 Orbital idiopathic inﬂammatory disease due to giant cell arteritis. (A) Computed tomography (CT) scan of the brain,

axial cut. (B, E and F) Magnetic resonance imaging (MRI), axial cut T1-weighted imaging with fat saturation after the administration

of gadolinium. (C) MRI, coronal cut T1-weighted imaging with fat saturation after the administration of gadolinium. (D) MRI, axial

cut T2-weighted imaging. Sixty eight year old patient (A) who presents to the hospital with visual loss in left eye and diagnosed

with acute ischaemic optic neuritis. In the CT scan there was presence of thickening in both optic nerves (short arrow in A) and

bulbar covers (long arrow in A). Yet despite the fact that nor the clinical data or the biopsies were not conclusive, giant cell arteritis

was diagnosed and with this suspicion in mind it was treated with corticoids. Four (4) months later the patient presents to the

hospital again (B---F) with new clinical manifestations in the right eye. One PET (positron emission tomography) scan is performed

(not shown) that conﬁrms Meckel’s cave and right orbital vertex uptake. The MRI conﬁrms orbital vertex affectation (thick arrow in

B) and shows thickening and hyper-uptake of Meckel’s cave dura mater (arrowhead in E) and right petroclinoid ligament (arrowhead

in B), the latter being hypointense in the T2-weighted imaging (black arrow in D). Also, there was thickening and hyper-uptake of

the basilar and vertebral arterial wall (arrows in C and F).

but without any clear signs as to be able to establish the and damages the optic nerve, or when the inﬂammatory

exact etiological diagnosis. That is, as radiologists we cannot component compromises the orbital vertex.

deﬁne this entity based on images only, but we will need to Fungal sinusitis is one relatively rare sinus infec-

know the clinical parameters as well. Also, some times the tious disease. It may be categorized into invasive and

diagnostic clinical criteria for these entities are not fully noninvasive. The invasive variant is deﬁned by the pres-

met. In these cases, in view of the radiological ﬁndings, for ence of fungi outside the sinus air cavity and occurs in

its management we could use a more general term such as immunosuppressed individuals, while clinically it presents

orbital idiopathic inﬂammatory disease (Fig. 9). itself acute or chronically. In acute cases, the individ-

ual presents to the hospital with a fever, rhinorrhea, and

Infectious orbital disease mucosal ulceration. In the chronic cases, symptom onset

The infectious orbital disease usually manifests itself as sep- is insidious and is characterized by the progressive inva-

tal and retroseptal cellulitis whose origin is usually pyogenic sion of paranasal sinus structures, like the orbital vertex,

sinusal affectation or, more rarely, fungal affectation. It can which clinically translates into reduced visual acuity and eye

associate loss of visual acuity when the infection spreads motility.

Figure 10 Orbital apex syndrome due to fungal sinusitis. (A) Computed tomography (CT) scan of the brain, axial cut. (B) CT

scan of paranasal sinus structures, axial cut. (C and D) Magnetic resonance imaging (MRI), axial cut T1-weighted imaging with fat

saturation after the administration of gadolinium. Patient presents to the ER with visual loss in left eye. One CT scan of the brain

is performed (A) that conﬁrms presence of an oversized optic nerve (arrow in A), complete opaciﬁcation of the sphenoid sinus that

showed thickened walls, very thick intrasinus calciﬁcations (black arrow in B), and soft tissue increase in the apex (asterisk in B).

Due to suspicion of invasive fungal sinusitis, one MRI is performed (C and D), that conﬁrms soft tissue increase in the orbital apex

(asterisk in C) and that the swelling has spread towards adjacent soft tissues (white arrows in D) in both pterygopalatine fossae.

Table 2 Clinical-radiological differential diagnosis of the most common orbital inﬂammatory entities.

Data Pseudotumor Cellulitis Dysthyroid

orbitopathy

Pain Intense and with Intense Mild

movements

Vision Usually normal Late onset Late onset

Motility Limited: in the Limited: in the Restricted: targeted

opposite direction to opposite direction to at the affected

the affected muscle the affected muscle muscle

Onset Acute Acute Gradual

Eyelids Ptosis, swelling Swelling Retraction

Clinical data Good response to Fever, sinus High levels of

corticoids affectation antibodies, hormonal

alterations

Imaging Unilateral: Unilateral: Gradual Bilateral asymmetric:

Inﬂammatory masses inﬂammatory Oversized extrinsic

No respect for muscle affectation: preseptal ocular extrinsic

insertion to postseptal. muscles

Sometimes presence Respect for muscle

of abscesses insertion

In the CT scan we can see sinus occupation, pres- Conﬂicts of interests

ence of dense tortuous calciﬁcations, and poor deﬁnition

of orbital vertex structures secondary to the presence of

The authors declare no conﬂict of interests associated with

inﬂammatory-fungal tissue. The MRI is a better imaging

this article whatsoever.

modality to deﬁne orbital affectation and the scope of the

spread process (Fig. 10).

In patients with radiological data of chronic rhinosinusitis

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