Brief Report

Cephalalgia
0(0) 1–5
Short-lasting unilateral neuralgiform ! International Headache Society 2016
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DOI: 10.1177/0333102416657148
cep.sagepub.com
injection and tearing-like attacks in
a pediatric patient found to have a
pontine capillary telangiectasia and
developmental venous anomaly: A case
report exploring the root of the problem

Natalia A Liapounova1, Juliana H VanderPluym1,

Ravi Bhargava2 and Hanna H Kolski1

Abstract
Introduction: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)-
like attacks are rarely reported in the pediatric population and may remain undiagnosed and under-investigated as a
result.
Case presentation: We present a case of a 15–year-old male with intermittent, episodic, right-sided brief headaches
most in keeping with SUNCT, initially diagnosed as paroxysmal hemicrania, but with no response to indomethacin. The
pain was likewise not responsive to typical migraine treatments or steroids.
Management and outcome: Contrast-enhanced magnetic resonance imaging demonstrated a right pontine capillary
telangiectasia with an associated developmental venous anomaly that was adjacent to the root of the right trigeminal
nerve. Differential diagnosis included first division trigeminal neuralgia with autonomic features. The patient’s pain was
partially alleviated by oxygen administration and responded well to carbamazepine; he remained pain free on carba-
mazepine a year later.
Conclusion: This case highlights the diagnostic dilemma of differentiating SUNCT from trigeminal neuralgia with
autonomic features, both of which are rare diagnoses in pediatric patients, and the importance of appropriate neuroima-
ging to rule out secondary causes in patients presenting with trigeminal autonomic cephalalgias, recognizing that
abnormalities identified on neuroimaging, such as vessels adjacent to the trigeminal nerve, may not be causative findings.

Keywords
SUNCT, trigeminal neuralgia, pediatric, developmental venous anomaly, capillary telangiectasia
Date received: 16 March 2016; revised: 6 May 2016; accepted: 31 May 2016

Introduction
Short-lasting unilateral neuralgiform headache attacks 1
Department of Pediatric Neurology, University of Alberta, Edmonton,
with conjunctival injection and tearing (SUNCT) is AB, Canada
classified as a trigeminal autonomic cephalalgia 2
Department of Radiology and Diagnostic Imaging, University of Alberta,
(TAC) in the International Classification of Headache Edmonton, AB, Canada
Disorders, 3rd edition beta (ICHD-3 beta) (1). SUNCT
is defined as at least 20 attacks of moderate or severe Corresponding author:
Natalia A Liapounova, Department of Pediatric Neurology, University of
unilateral pain with an orbital, supraorbital, temporal Alberta, 3-574A ECHA 11405-87 Avenue, Edmonton, AB, Canada T6G
or trigeminal distribution, lasting 1–600 seconds (1). 1C9.
These attacks must be associated with both Email: liapouno@ualberta.ca
2 Cephalalgia 0(0)

conjunctival injection and lacrimation, distinguishing
SUNCT from short-lasting unilateral neuralgiform
headache attacks with cranial autonomic symptoms
(SUNA) (1). SUNCT has a reported prevalence in
adults of 6.6 per 100,000 of the population (2).
Literature describing SUNCT in the pediatric popula-
tion is scant. The most common secondary causes are
lesions in the posterior fossa and pituitary gland (3).
The differential diagnosis of SUNCT includes trigem-
inal neuralgia (TN) as another unilateral brief pain syn-
drome. In comparison to SUNCT, TN consists
of recurring attacks of severe shock-like or stabbing
pain in one or more divisions of the trigeminal nerve
(usually the second or third), lasting for a fraction of a
second to 2 minutes (1). The most common imaging find-
ing is vascular compression of the trigeminal nerve, usu-
ally by the superior cerebellar artery (1). Like SUNCT,
TN is rare in pediatric patients and should be considered
symptomatic until proven otherwise.
We present a case of a 15–year–old male with
SUNCT-like headaches who was found to have a right
pontine capillary telangiectasia and developmental
venous anomaly. This case highlights the diagnostic
dilemma of differentiating SUNCT from TN with auto-
nomic features, particularly in the presence of an anom-
alous vessel adjacent to the ipsilateral trigeminal nerve.

Table 1. Headache characteristics of our patient in comparison to International Classification of Headache Disorders, 3rd edition
(beta version) diagnostic criteria for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and
trigeminal neuralgia.

Our patient SUNCT TN (classical)

Number of attacks Over 200 At least 20 attacks At least 3 attacks

Pain severity Background 8/10 with 10/10 Moderate or severe pain Severe pain
stabs during attack
Location Unilateral right-sided periorbital Unilateral with orbital, Unilateral facial pain in one
supraorbital, temporal or more divisions of the
and/or other trigeminal trigeminal nerve, with
distribution no radiation beyond the
trigeminal distribution
Duration 5–50 seconds 1–600 seconds Fraction of a second to 2
minutes
Pattern Saw-tooth Single stabs, series of stabs Electric shock-like, shooting,
or in a saw-tooth pattern stabbing or sharp
Associated symptoms Immediate onset of conjunctival At least one cranial auto- Mild autonomic symptoms such as
injection, lacrimation, rhinor- nomic symptom or sign lacrimation and/or redness of the
rhea, eyelid edema, forehead ipsilateral to pain: eye may be present
and facial sweating and fore- SUNCT – both of
head and facial flushing conjunctival injection
and lacrimation
Frequency 10–200 attacks per day Frequency of at least one Frequency of TN is not outlined
attack a day for more than in the ICHD-3 beta, but can be
half of the time when the variable and dependent on
disorder is active exposure to triggers
Trigger/refractory Touching side of face No SUNCT and SUNA are usually trig- Precipitated by innocuous stimuli
period refractory period gerable without a refractory to the affected side of the
period face Following a painful parox-
ysm, there is usually a refractory
period, during which pain cannot
be triggered
Associated (neuro- Mild hyperesthesia right side of Miosis and/or ptosis may occur No clinically evident
logic) deficit the facea ipsilateral to pain neurological deficitsb
Mandatory ICHD-3 beta diagnostic criteria are in bold. Italicized data are found in the comments of the ICHD-3 beta.
a
As opposed to persistent pain characterizing TN with concomitant persistent facial pain, which responds poorly to conservative treatment and is less
likely to be triggered by innocuous stimuli (1).
b
There is no obvious underlying medical disease/provocation prompting consideration of painful trigeminal neuropathy.
ICHD-3 beta: International Classification of Headache Disorders, 3rd edition (beta version); SUNA: short-lasting unilateral neuralgiform headache
attacks with cranial autonomic symptoms; SUNCT: short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing; TN:
trigeminal neuralgia.
Liapounova et al.
Case report
A 15-year-old otherwise healthy and developmentally
normal Caucasian male was seen in the emergency
department regarding a 2–day history of brief, frequent
painful attacks involving the right periorbital region.
Table 1 outlines his headache characteristics. The attacks
lasted between 5 and 50 seconds and consisted of stabs of
10/10 pain on a background of 8/10 pain in a saw-tooth
pattern; he was pain free between attacks for periods of 5–
60 minutes. At worst, he was having approximately 200
attacks per day. During attacks, he felt severe stabbing
pain in the right periorbital region, accompanied by
immediate and profound ipsilateral lacrimation, conjunc-
tival injection, diaphoresis and eyelid edema. Touching
the right periorbital region occasionally triggered an
attack; there were no other triggers. There was no refrac-
tory period. The patient was significantly disabled by
these events; he was unable to eat, sleep or go to school
for the 2 days prior to presenting to hospital. Full physical
and neurological examinations were otherwise normal.
Two years prior, he had experienced a similar bout
of attacks lasting 6 weeks and resolving spontaneously
before neurology assessment. He was diagnosed retro-
spectively with paroxysmal hemicrania and given a pre-
scription of indomethacin for future use in case of
recurrence. A non-contrast computed tomography
(CT) scan performed at that time was reported as
3
normal. Prior to presentation to hospital this time,
the patient tried the previously prescribed indometh-
acin at adequate doses without benefit.
Contrast-enhanced magnetic resonance imaging
(MRI) performed at admission revealed a small enhan-
cing lesion in the right pons, consistent with capillary
telangiectasia, and a large enhancing vascular mass abut-
ting the right pons, consistent with a developmental
venous anomaly. MRI with SPACE (Sampling
Perfection with Application optimized Contrasts using
different flip angle Evolution) sequence repeated 4
months later demonstrated no change to the lesion and
showed that the draining vein exited immediately infer-
ior and medial to the root of the right trigeminal nerve
with no sign of mass effect (Figure 1).
In the emergency department, the patient did not
respond to a 1–L bolus of normal saline, 10 mg metoclo-
pramide and repeat administration of 50 mg indometh-
acin. He also failed intravenous methylprednisolone
(1 g divided every 6 hours). Because the patient remained
in significant distress, low-flow oxygen by nasal cannula
was provided. This unexpectedly improved the severity
of his attacks. Based on this observation, the patient was
provided with high-flow 100% oxygen (12 L/minute) by
non-rebreather mask to be used at his discretion for
longer and more frequent attacks; the patient reported
improvement in both severity (decrease in pain from

Figure 1. (a) Contrast-enhanced T1 magnetic resonance imaging (MRI): faintly enhancing lesion in the right pons, consistent with
capillary telangiectasia (black arrow), and a large enhancing serpiginous vascular lesion arising along the right lateral aspect of the
capillary telangiectasia and extending into the right cerebellopontine angle, consistent with a developmental venous anomaly (white
arrow). (b) MRI Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) sequence:
draining vein (thin white arrow) of the above demonstrated developmental venous anomaly abutting the lateral aspect of the right
trigeminal nerve (thick white arrow).
4 Cephalalgia 0(0)
8/10 to 6/10) and length of attacks with the oxygen
inhalation. Despite the improvement in headaches with
oxygen inhalation, it was not an absolute response, such
as is seen with cluster headaches; thus, he was started on
a prophylactic therapy. Though lamotrigine is accepted
as the first choice for the treatment of SUNCT, it was
felt that the risk of potential side effects (such as Steven–
Johnson syndrome) from the relatively rapid titration
was too high. Carbamazepine was therefore chosen as
a literature-supported therapy in TN, as there was a
question of possible compression of the vascular anom-
aly of the trigeminal root. Initially, the patient did not
tolerate oral intake due to the severity of his pain, so he
was administered a single intravenous dose of 400 mg
phenytoin, given the presumed similar mechanisms of
action of phenytoin and carbamazepine; the patient
reported a decrease in pain severity from 6/10 to 4/10
following this intervention. He was subsequently started
on carbamazepine 200 mg twice daily with rapid titration
up to a final dose of 400 mg twice daily. The patient
experienced initial somnolence with carbamazepine,
which resolved prior to discharge from hospital.
Three days later, he was having infrequent episodes of
minimal-intensity pain and was thus discharged home on
carbamazepine. At the 6–month follow-up at the pediat-
ric neurology clinic, he reported no further severe head-
aches, but noted infrequent unprovoked episodes of brief
(less than 5–second), severe, stabbing right periorbital
pain with no associated autonomic symptoms. One
month prior to follow-up, he discontinued carbamaze-
pine on his own. He was advised to restart carbamazepine
(400 mg twice daily) as he was approaching the time of
year when his severe headache bouts usually occurred. At
follow-up over 1 year later, he was compliant on carba-
mazepine and reported no further bouts of headache.
Discussion
Literature describing SUNCT in pediatric patients is
scarce (4). Our case contributes another patient to
this limited number of reported cases. Besides the
patient’s age, this case is novel for two other reasons:
(i) pontine capillary telangiectasia with associated
developmental venous anomalies is rare (5); and (ii)
this case illustrates the difficulty in distinguishing
SUNCT from TN with autonomic features.
Our patient had abnormal MRI findings despite a
previous normal non-contrast CT scan, normal neuro-
logic physical examination and typical SUNCT-like
headache. This emphasizes the need for appropriate
neuroimaging with MRI, including dedicated views of
the pituitary gland, trigeminal nerves and vasculature,
in all patients presenting with TAC-like headaches.
Secondary SUNCT has been described with pituitary
gland and posterior fossa lesions of varying etiology,
including neoplastic, vascular, traumatic, infectious,
inflammatory and structural (3). In studies looking at
SUNCT and vascular compression of the trigeminal
nerve, the vessels of interest were mainly arterial, in
contrast to our patient, who had a venous anomaly
adjacent to his trigeminal nerve (2,6). It is important
to note that the vein in our patient exited immediately
inferior and medial to the root of the right trigeminal
nerve, and did not show any signs of mass effect.
Therefore, one must be cautious in interpreting the con-
tribution of this vein to his headaches, and as such, we
cannot ascribe a causative relationship.
Williams and Broadley examined MRIs in patients
with SUNCT and SUNA and found that 88% of MRIs
with dedicated views of the trigeminal nerve showed an
aberrant arterial loop that was in contact with the tri-
geminal nerve (2). This was a more common finding in
patients with chronic SUNCT or SUNA (100%) com-
pared to episodic SUNCT or SUNA (38%), suggesting
a possible etiological contribution. Another recent
study that reviewed 222 published cases of SUNCT
and SUNA found that 16.9% had neurovascular com-
pression (6). Among patients with neurovascular com-
pression, 47% had microvascular decompression of
the trigeminal nerve, with effectiveness in 75% (6).
The reported number of aberrant vessels is lower in the
Favoni et al. study (6) than that reported by Williams
and Broadley (2); this difference may be due to the fact
that the MRIs in the Williams and Broadley study
included more dedicated views of the trigeminal nerve.
SUNCT and TN can demonstrate several similar
clinical features, including severity of pain, quality of
pain, unilaterality and even cutaneous triggers, likely
due to the activation of the trigeminal nerve as a
shared final common pathophysiological pathway (7).
Our patient’s pain severity, location, duration, pattern
and cutaneous triggers met the criteria for either diag-
nosis (Table 1), which illustrates the overlap in charac-
teristics between SUNCT and TN. Consequently, in
order to help clarify the diagnosis, one must focus on
the distinguishing clinical features, such as gender pre-
dilection (based on adult populations: SUNCT: male;
TN: female), distribution of pain (SUNCT: first div-
ision of trigeminal nerve or outside trigeminal nerve
distribution; TN: second or third division of trigeminal
nerve), duration of attacks (TN: more commonly under
10 seconds), intensity of autonomic symptoms
(SUNCT: severe; TN: mild), time of onset of auto-
nomic symptoms (SUNCT: early; TN: late), presence
of a refractory period (TN: present) and response to
therapy (TN: responsive to carbamazepine), which are
valuable in helping differentiate these disorders (7).
When applying these distinguishing features to our
patient, his diagnosis fits best with SUNCT, while
recognizing that there are some outstanding features
Liapounova et al. 5

to his case, such as his exquisite responsiveness to
carbamazepine, which would be more supportive of a
diagnosis of TN (8). Finally, our patient had a good
response to carbamazepine, which was the first prevent-
ive therapy he tried. However, if he had not responded,
other medications for treating headaches with SUNCT
(lamotrigine, topiramate, gabapentin and intraven-
ous lidocaine) (9) or TN (oxcarbazepine, baclofen,
gabapentin and lamotrigine) features could have been
tried (10).

Clinical implications
. There is an overlap between the clinical presentations of short-lasting unilateral neuralgiform headache
attacks with conjunctival injection and tearing and trigeminal neuralgia, illustrating that when diagnoses
share a final common physiological pathway (the trigeminal nerve), the clinical pictures may be difficult to
distinguish.
. It is important to recognize that young patients can experience headache disorders outside of the common
diagnosis of migraine, such as trigeminal autonomic cephalalgias, and must be considered in cases of
unusual characteristics of headaches in the young.
. Advanced imaging of the posterior fossa with a focus on vascular structures (e.g., magnetic resonance imaging
with gadolinium) may uncover previously unreported findings, such as Developmental Venous Anomaly
(DVA) or capillary telangiectasia, which may help identify associations but cannot necessarily speak to
causation.

Declaration of conflicting interests 4. Sekhara T, Pelc K, Mewasingh LD, et al. Pediatric

The authors declared no potential conflicts of interest with
respect to the research, authorship and/or publication of this
article.
Funding
The authors received no financial support for the research,
authorship and/or publication of this article.
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