A total of 182 records were identified and reduced to 116 after removing duplicates. After screening and the application of the eligibility criteria, 22 articles (10, 11, 21–40) met the inclusion criteria. Figure 1 presents the flow diagram of included and excluded articles of our review according to PRISMA (41).
Table 1 provides an overview of the patients’ characteristics and outcome measures. The included studies consist of 5 case series and 17 case reports. There were no cohort or case-control studies, or clinical trials in this regard. Overall, the studies represent 38 children suspected of PH, however, we excluded 3 cases (11, 39) for the final analysis, since they had no concomitant autonomic symptoms. These 3 cases are discussed in the discussion section.
The first pediatric case of PH was reported by Kudrow in 1989 (10). Among previous studies in the pediatric population, CPH, EPH, and not-specified-PH were reported in 23, 7, and 5 patients, respectively. PH has been described among children and adolescents in various parts of the world including the United States (USA) (10, 23, 27, 35), Canada (29, 39), United Kingdom (UK) (21), Germany (22, 25, 30, 31), France (40), Italy (26, 32, 38), Austria (34), Estonia (36, 37), Brazil (24), Portugal (11), Japan (28), and Korea (33), but the races were not specified in most of the studies. Unless one study, the gender of all cases was reported (10). We found a male to female sex ratio of 1.125:1 (18 males vs 16 females) in our review of all pediatric PH cases. The youngest age of onset was reported by Almeida et al. They reported a 10-year old girl who complained of left unilateral headache attacks since she was 1-year-old (24). After excluding the cases with unknown ages of diagnosis (10, 21, 34, 35), the mean age of onset among reported cases in children and adolescents was 6.30 years, while the mean age of diagnosis was 7.96 years. The shortest course of pediatric PH was reported by Ishii et al in an 11-year-old boy from Japan, who underwent treatment with Indomethacin within two days of his first referral (28), in contrast, the longest course was reported in a 10-year old girl who have suffered since she was a 1-year-old baby. The most frequent attacks were attributed to a 14-year old girl with 32-48 attacks/day (21) and the least frequent attacks were described in a 10-year old girl with 1 attack per week(24). The duration of attacks varied between 2 minutes (21) to 40 minutes (24). The pain is characteristically unilateral but can alternate sides between attacks (39) and rarely can be bilateral (33). The left-sided pain was approximately reported twice more than the right-sided headaches (11 left-sided vs 6 right-sided). The characteristics of the pain in PH is usually severe in intensity (lead to crying/screaming, awakening the child at night, and wishing to die) in almost all cases and has been described by patients as sudden, sharp, stabbing, shooting, throbbing, and excruciating.
Attacks of PH invariably occur in association with ipsilateral cranial autonomic features in all cases except three children which we noted above (11, 39). Migrainous features including nausea (40), vomiting (26, 29, 33, 38), phonophobia (32, 40), photophobia (29, 32, 34, 38), and osmophobia (33, 38) were reported in 1, 4, 2, 4 and 2 children, respectively. Patients were often restless or agitated during an attack. While the majority of attacks are spontaneous, common triggers include heat waves (40), tobacco smell (40), stress such as school-attributed problems (29), exercise (31), lack of sleep (33), bouncing on a trampoline (34), and quick movements of the head (34). Overall, the physical examination and electroencephalography (EEG) were normal. The brain magnetic resonance imaging (MRI) in all the reported cases was completely normal except for two cases with an incidental pineal cyst (31) and a previous ipsilateral occipital infarction (23). Past medical history for Kawasaki’s disease (31), ipsilateral occipital infarction (23), asthma (28), sinusitis (33), otitis media and tonsillitis (29), migraine without aura (34), septoplasty surgery (34), and mild obesity (32) were reported in children and adolescents with PH. In these 35 cases that we systematically reviewed, there was no evidence of PH in the family history of children and adolescents. Whereas, we found 8, 5, 1, and 1 cases with positive family history for migraine (26, 33–36, 38) and unspecified headaches (29, 31, 34, 35), breath-holding (36), and bipolar disorder (23), respectively. Almost all patients benefited with Indomethacin and showed complete response to treatment (23, 25–28, 31, 33, 34, 36–38, 40), while some needed combination treatment of Indomethacin with other medications such as combination of Topiramate and Sumatriptan (21), Verapamil (24, 35), Sodium valproate (38) and Topiramate (38). There is also one successful report on Aspirin prophylaxis in pediatric PH(10). In addition, Shabbir et al. represented two cases with effective treatment of PH by Verapamil monotherapy(35). One study suggested using a headache diary as both an idea for diagnosis and an initial non-pharmacological therapeutic intervention in children with PH (29). In addition, a recent study describes the first child treated by occipital nerve stimulation for CPH as a new treatment option for children with refractory CPH (40).
Table 2
Diagnostic criteria of paroxysmal hemicranias based on ICHD-3
A. At least 20 attacks fulfilling criteria B-E
B. Severe unilateral orbital, supraorbital and/or temporal pain lasting 2-30 minutes
C. Either or both of the following:
1. at least one of the following symptoms or signs, ipsilateral to the headache:
• – conjunctival injection and/or lacrimation
• – nasal congestion and/or rhinorrhea
• – eyelid edema
• – forehead and facial sweating
• – miosis and/or ptosis
2. a sense of restlessness or agitation
D. Occurring with a frequency of >5 per day1
E. Prevented absolutely by therapeutic doses of indomethacin2
F. Not better accounted for by another ICHD-3 diagnosis.
|
References |
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14. Myers KA and Smyth KA. Preadolescent Indomethacin-Responsive Headaches Without Autonomic Symptoms. Headache: The Journal of Head and Face Pain 2013; 53: 977-980. |
15. Raieli V, Cicala V and Vanadia FJNS. Pediatric paroxysmal hemicrania: a case report and some clinical considerations. 2015; 36: 2295-2296. |
16. Rho YIJKJoP. Bilateral paroxysmal hemicrania with autonomic features in a child: A case report. 2009; 52: 619-621. |
17. Seidel S, Wöber CJHTJoH and Pain F. Paroxysmal hemicrania with visual aura in a 17-year‐old boy. Headache 2009; 49: 607-609. 2009/04/07. DOI: 10.1111/j.1526-4610.2008.01252.x. |
18. Shabbir N, McAbee GJHTJoH and Pain F. Adolescent chronic paroxysmal hemicrania responsive to verapamil monotherapy. Headache 1994; 34: 209-210. 1994/04/01. DOI: 10.1111/j.1526-4610.1994.hed3404209.x. |
19. Talvik I, Koch K, Kolk A, et al. Chronic paroxysmal hemicrania in a 3-year, 10-month-old female. Pediatr Neurol 2006; 34: 225-227. 2006/03/01. DOI: 10.1016/j.pediatrneurol.2005.08.026. |
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22. Vieira J, Salgueiro A and Alfaro M. Short-lasting headaches in children. Cephalalgia 2006; 26: 1220-1224. 2006/09/12. DOI: 10.1111/j.1468-2982.2006.01205.x. |
Risk of bias and heterogeneity
Table S-3 show the results of the quality assessment of the included studies. Following the results of the JBI tools for case reports and case series studies, almost all studies had an overall high quality, except Vieira et al study (11).