Background and objective There are five headache disorders composing the trigeminal autonomic cephalalgias (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua). Little is known about these disorders in the pediatric population. The objectives of this study are to report the full age ranges of pediatric trigeminal autonomic cephalalgias and to determine if pediatric-onset trigeminal autonomic cephalalgias display similar signs and symptoms as adult onset. Methods Search criteria in Medline Ovid, Embase, PsycINFO, and Cochrane Library were created by a librarian. The remainder of the steps were independently performed by two neurologists using PRISMA guidelines. Inclusion criteria for titles and abstracts were articles discussing cases of trigeminal autonomic cephalalgias with age of onset 18 or younger, as well as any epidemiological report on trigeminal autonomic cephalalgias (as age of onset data was often found in the results section but not in the title or abstract). Data extracted included age of onset, sex, and International Classification of Headache Disorders criteria for trigeminal autonomic cephalalgias (including pain location, duration, frequency, autonomic features, restlessness) and some migraine criteria (photophobia, phonophobia, and nausea). Studies that did not meet full criteria for trigeminal autonomic cephalalgias were examined separately as “atypical trigeminal autonomic cephalalgias”; secondary headaches were excluded from this category. Results In all, 1788 studies were searched, 86 met inclusion criteria, and most ( 56 ) examined cluster headache. In cluster headache, onset occurred at every pediatric age (range 1–18 years) with a full range of associated features. Autonomic and restlessness features were less common in pediatric patients, while migrainous features (nausea, photophobia, and phonophobia) were found at similar rates. The sex ratio of pediatric-onset cluster headache (1.8, 79 male and 43 female) may be lower than that of adult-onset cluster headache. Data for other trigeminal autonomic cephalalgias, while more limited, displayed most of the full range of official criteria. The data for atypical trigeminal autonomic cephalalgias were also limited, but the most common deviations from the official criteria were abnormal frequencies and locations of attacks. Conclusions Trigeminal autonomic cephalalgias can start early in life and have similar features to adult-onset trigeminal autonomic cephalalgias. Specifically, pediatric-onset cluster headache patients display the full range of each criterion for cluster headache (except maximum frequency of six instead of eight attacks per day). However, cranial autonomic features and restlessness occur at a lower rate in pediatrics. Additional information is needed for the other trigeminal autonomic cephalalgias. As for expanding the ICHD-3 criteria for pediatric-onset trigeminal autonomic cephalalgias, we have only preliminary data from atypical cases, which suggests that the frequency and location of attacks sometimes extend beyond the official criteria. Trial Registration: This study was registered as a systematic review in PROSPERO (registration number CRD42020165256).
Cluster headache: Epidemiology, pathophysiology, clinical features, and diagnosis
