Short-lasting unilateral neuralgiform headache attacks with conjunctival 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

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 carbamazepine 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 neuroimaging 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.

Introduction

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is classified as a trigeminal autonomic cephalalgia (TAC) in the International Classification of Headache Disorders, 3rd edition beta (ICHD-3 beta) (1). SUNCT is defined as at least 20 attacks of moderate or severe unilateral pain with an orbital, supraorbital, temporal or trigeminal distribution, lasting 1–600 seconds (1). These attacks must be associated with both 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 population is scant. The most common secondary causes are lesions in the posterior fossa and pituitary gland (3).

The differential diagnosis of SUNCT includes trigeminal neuralgia (TN) as another unilateral brief pain syndrome. 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 finding is vascular compression of the trigeminal nerve, usually 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 autonomic features, particularly in the presence of an anomalous vessel adjacent to the ipsilateral trigeminal nerve.

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, conjunctival injection, diaphoresis and eyelid edema. Touching the right periorbital region occasionally triggered an attack; there were no other triggers. There was no refractory 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.

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 stabs during attack	Moderate or severe pain	Severe pain
Location	Unilateral right-sided periorbital	Unilateral with orbital, supraorbital, temporal and/or other trigeminal distribution	Unilateral facial pain in one or more divisions of the trigeminal nerve, with 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 or in a saw-tooth pattern	Electric shock-like, shooting, stabbing or sharp
Associated symptoms	Immediate onset of conjunctival injection, lacrimation, rhinorrhea, eyelid edema, forehead and facial sweating and forehead and facial flushing	At least one cranial autonomic symptom or sign ipsilateral to pain: SUNCT – both of conjunctival injection and lacrimation	Mild autonomic symptoms such as lacrimation and/or redness of the eye may be present
Frequency	10–200 attacks per day	Frequency of at least one attack a day for more than half of the time when the disorder is active	Frequency of TN is not outlined in the ICHD-3 beta, but can be variable and dependent on exposure to triggers
Trigger/refractory period	Touching side of face No refractory period	SUNCT and SUNA are usually triggerable without a refractory period	Precipitated by innocuous stimuli to the affected side of the face Following a painful paroxysm, there is usually a refractory period, during which pain cannot be triggered
Associated (neurologic) deficit	Mild hyperesthesia right side of the face a	Miosis and/or ptosis may occur ipsilateral to pain	No clinically evident neurological deficits b

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.

Two years prior, he had experienced a similar bout of attacks lasting 6 weeks and resolving spontaneously before neurology assessment. He was diagnosed retrospectively with paroxysmal hemicrania and given a prescription of indomethacin for future use in case of recurrence. A non-contrast computed tomography (CT) scan performed at that time was reported as normal. Prior to presentation to hospital this time, the patient tried the previously prescribed indomethacin at adequate doses without benefit.

Contrast-enhanced magnetic resonance imaging (MRI) performed at admission revealed a small enhancing lesion in the right pons, consistent with capillary telangiectasia, and a large enhancing vascular mass abutting 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 inferior and medial to the root of the right trigeminal nerve with no sign of mass effect (Figure 1).

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).

In the emergency department, the patient did not respond to a 1–L bolus of normal saline, 10 mg metoclopramide and repeat administration of 50 mg indomethacin. 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 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 anomaly 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 pediatric neurology clinic, he reported no further severe headaches, 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 carbamazepine 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 carbamazepine 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 neurologic 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 contribution 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 trigeminal nerve (2). This was a more common finding in patients with chronic SUNCT or SUNA (100%) compared 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 compression (6). Among patients with neurovascular compression, 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 diagnosis (Table 1), which illustrates the overlap in characteristics between SUNCT and TN. Consequently, in order to help clarify the diagnosis, one must focus on the distinguishing clinical features, such as gender predilection (based on adult populations: SUNCT: male; TN: female), distribution of pain (SUNCT: first division 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 autonomic 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 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 preventive therapy he tried. However, if he had not responded, other medications for treating headaches with SUNCT (lamotrigine, topiramate, gabapentin and intravenous 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.