(2022) 22:488 Tetsuhara et al. BMC Neurology https://doi.org/10.1186/s12883-022-03022-4 Open Access CASE REPORT Quantitative indices for an intracranial aneurysm and subarachnoid hemorrhage in early childhood: a case report Kenichi Tetsuhara1,2,3, Noriyuki Kaku1,2* , Koichi Arimura4, Yasunari Sakai1 and Shouichi Ohga1 Abstract Background: Intracranial aneurysms (ICA) rarely occur in children under 3 years of age. Little is known for neuroimaging parameters that predict survival and clinical outcomes of patients with ICA in early childhood. Case presentation: A 2-year-old girl showed intracranial hemorrhage due to a rupture of aneurysm at the middle cerebral artery. Quantitative measurements of ischemic damages on the head computed tomography (CT) marked an extremely low score of 2 points with modified Alberta Stroke Program Early CT Score (mASPECTS). She died 15 days after admission. In publications from 2021 to 2022, we found 21 children who were under 3 years of age at onset of ICA. None of them died, but two of three patients who had mASPECTS scores 0–8 showed developmental delay and/ or epilepsy as neurological complications. Conclusion: Early CT findings are applicable for predicting survival and neurological outcomes of young children with intracranial hemorrhage. Keywords: Intracranial aneurysm, Modified Alberta stroke program early CT score (mASPECTS), Outcome, And children Background Intracranial aneurysm (ICA) is a rare condition in children under 3 years of age [1, 2]. Infections, posttraumatic, and specific genetic conditions are more frequently associated with ICA and ICA-related brain hemorrhages in childhood than those in adults [3]. However, only a few reports have demonstrated details in neuroimaging features and clinical outcomes of patients with ICA in early childhood [4, 5]. Two neuroimaging parameters, simplified gray matter attenuation-to-white matter attenuation ratio (sGWR) and modified Alberta stroke program early CT score (mASPECTS), are known to be *Correspondence: kaku.noriyuki.198@m.kyushu-u.ac.jp 2 Emergency and Critical Care Center, Kyushu University Hospital, 3‑1‑1, Maidashi, Higashi‑ku, Fukuoka 812‑8582, Japan Full list of author information is available at the end of the article useful for quantitatively analyzing parenchymal damages of the brain in children with cardiac arrest [6]. We thus asked whether these scoring systems might also provide critical values for the outcome of ICA in early childhood. We herein report a young child who had a rupture of ICA at the middle cerebral artery (MCA) and characterize the neuroimaging feature of this patient in comparison with previously reported children under age 3 years. Case presentation A 2-year-and-8-month-old girl was referred to the previous hospital because of altered consciousness. A head computed tomography (CT) indicated the urgent neurosurgical intervention for the intracranial hemorrhage with a midline shift. On arrival to our hospital, spontaneous breathing was absent, and Glasgow Coma Scale was evaluated to be E1V1M1. The contrastenhanced CT in our hospital confirmed the hemorrhage © The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://​creat​iveco​mmons.​org/​licen​ses/​by/4.​0/. The Creative Commons Public Domain Dedication waiver (http://​creat​iveco​ mmons.​org/​publi​cdoma​in/​zero/1.​0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Tetsuhara et al. BMC Neurology (2022) 22:488 extending to the subarachnoid space and disclosed an aneurysm of 21 × 13 × 12 mm at the right MCA (Fig. 1). We applied quantitative measurements of the sGWR and mASPECTS (range 0–24) for her neuroimaging data (Fig. S1). The modified sGWR scored 1.13, while mASPECTS gained only 2 points, both suggestive of poor prognosis [6]. Although decompressive surgery was immediately performed, her systemic conditions became unstable with uncontrollable pulmonary edema. She died on the 15th day after admission. The panel sequencing for COL3A1, FBN1, TGFBR1, TGFBR2, and RNF213 excluded the diagnosis of Ehlers-Danlos syndrome, Page 2 of 4 Marfan syndrome, Loeys-Dietz syndrome, and Moyamoya disease. Blood culture was negative throughout the treatment course. Systemic imaging studies excluded malformation of great arteries, renal cysts, and tumors. Through the literature search from 2021 to April 2022 in PUBMED (https://​pubmed.​ncbi.​nlm.​nih.​gov/), we found 103 publication records with search terms, “intracranial”, “aneurysms” and “pediatric”. Among them, 21 patients were reportedly under 3 years of age (12 females; 1 months to 2 years) at onsets (Table 1) [4, 5, 7–12]. Although variable degrees of neurological deficits were left, none of them died or failed to receive neurosurgical Fig. 1 Plain and contrast-enhanced CT scans of the aneurysm in the present case. A A plain CT on admission in the present case. Arrow indicates the massive hemorrhage and the devoid of hemorrhagic signal (asterisk) in the right MCA region. Arrowheads indicate the prominent midline shift to the left hemisphere. B A plain CT on admission. The hemorrhagic lesion was extended to the surrounding parenchyma involving the caudate and lentiform nuclei (arrows). C A contrast CT shows the presence of an aneurysm located at the right MCA region (asterisk) and subarachnoid hemorrhage (arrowheads). D A stereographic reconstitution of the contrast head CT depicting the fusiform structure of MCA aneurysm (arrow: 21 × 13 × 12 mm in size) Tetsuhara et al. BMC Neurology (2022) 22:488 Page 3 of 4 Table 1 Summary of patients with intracranial aneurysms less than 3 years of age (reports in 2021–2022) No Patient ID Age at ­onset1 Location, morphology Etiology mASPECTS2 Outcome3 Year Ref 1 Xu-1 1m L-cavernous, saccular Unknown – mRS 0 2021 5 2 De Aguiar-1 1.5 m R-MCA 10 mm, saccular Idiopathic – – 2022 8 3 Clarke-7 2m R-MCA 11 mm, saccular Idiopathic L DD, EP 2022 4 4 Clarke-3 3m R-MCA 9 mm, fusiform Idiopathic L DD 2022 4 5 Clarke-6 3m L-PCA 16 mm, saccular Idiopathic – DD 2022 4 6 Komuński-1 5m R-ICA, saccular Idiopathic M – 2021 10 7 Clarke-5 6m R-MCA 2 mm, saccular Idiopathic – Hemiparesis 2022 4 8 Clarke-9 6m L-MCA 14 mm, saccular Idiopathic – Hemiparesis, VD 2022 4 9 Clarke-8 7m R-ICA 5 mm, saccular Idiopathic – Hydrocephalus 2022 4 10 Sombo-1 8m Proximal basilar 8 mm, fusiform Post-infectious L – 2021 12 11 Xu-2 9m L-MCA, pseudo Unknown – mRS 2 2021 5 12 Barch-1 10 m R-MCA, multiple, fusiform Idiopathic H – 2021 7 13 Saraf-1 11 m L-MCA, multi-lobular, dissecting Post-traumatic H Hemiplegic gait 2021 11 14 Clarke-10 12 m R-PCA 10 mm, saccular Gaucher disease – Hemiparesis 2022 4 15 Xu-3 12 m L-MCA Unknown M mRS 1 2021 5 16 Clarke-1 1 y 2m L-MCA 4 mm, saccular Idiopathic – Minor spasticity 2022 4 17 Clarke-4 1 y 5m R-MCA 18 mm, saccular Idiopathic H Hemiparesis 2022 4 18 Xu-4 1 y 9m R-MCA, saccular Unknown M mRS 1 2021 5 19 Xu-5 1 y 10 m L-cavernous, giant Unknown – mRS 0 2021 5 20 Clarke-2 1 y 10 m R-ACA 3 mm Post-traumatic – No deficit 2022 4 2021 9 21 Demartini-1 2y R-ICA 5 mm, saccular Post-traumatic M Asymptomatic 22 Present case 2 y 8m R-MCA 21 mm Idiopathic L, 2 Died on day 15 1 Age represents years (y) and months (m) 2 The mASPECTS scores were estimated and classified into the following three groups according to the presented images: L (low, 0–8), M (moderate, 9–16) and H (high, 17–24). -, no image available 3 Comorbidities and neurological outcomes include epilepsy (EP) and measurements in modified Rankin scale (mRS) -, No complication or normal development; DD, developmental delay; VD, visual defects; mASPECTS, modified Alberta Stroke Program Early CT Score; Ref, references operations for their critical conditions. Thus, no clinical or neuro-imaging parameters were identified to predict the unfavorable outcome (death). Based on the neuroimaging data (n = 10), however, we estimated mASPECTS scores. We classified them into the three groups: L (low, mASPECTS 0–8), M (moderate, 9–16) and H (high, 17–24) (Table 1). Two (67%) of the three patients who belonged to the L group showed profound complications of developmental delay and/or epilepsy as neurological sequelae. On the other hand, two (29%) of seven patients with M and H scores showed hemiplegia. These data suggested that mASPECTS scores were useful for predicting postsurgical outcomes of ICA. Discussion ICA in pediatric age accounts for 10–15% of the whole patient populations [13]. Patients under 3 years of age are extremely rare in prevalence [14, 15]. While it is difficult to perform angiography for infants and young children during the critical period, pediatric patients with successful outcomes have been increasingly reported in recent years [3–5, 8, 11, 12]. Nevertheless, data have been less extensively analyzed for young children with unfavorable clinical courses. For this reason, the present case underscores the diagnostic value of neuroimaging findings for accurately detecting the ICA in early childhood. In our previous study, lower scores (sGWR < 1.14 and mASPECTS < 20) were correlated with worse outcomes [6]. The present case showed low values of sGWR (1.13) and mASPECTS (2) on admission. Although further studies are required, these data may compensate insufficient prognostic values for survival and neurological outcomes of pediatric patients with intracranial hemorrhage. Different patho-mechanisms have been considered to be involved in the development of ICAs in childhood and adults [1, 2, 4]. Childhood-onset ICAs are more frequently associated with trauma, infection, and particular genetic backgrounds than adult-onset ICAs [16]. In our patient, either the family information, past history, laboratory data or genetic analysis did not support evidence for common causes of ICAs. Thus, the patient was etiologically classified into the group of “idiopathic” ICA [1]. In conclusion, contrast-enhanced CT is a useful modality not only for detecting the source of hemorrhage, but Tetsuhara et al. BMC Neurology (2022) 22:488 also for predicting survival and neurological outcomes of young children with massive ICA. Accumulating clinical and quantitative neuroimaging data will further dissect critical findings for both groups of children with successful and unfavorable outcomes. Abbreviations ICA: Intracranial aneurysm; CT: Computed tomography; mASPECTS: Modified Alberta Stroke Program Early CT Score; sGWR​: Simplified gray matter attenuation-to-white matter attenuation ratio; MCA: Middle cerebral artery. Supplementary Information The online version contains supplementary material available at https://​doi.​ org/​10.​1186/​s12883-​022-​03022-4. Additional file 1. Acknowledgements We thank Kazusa DNA Research Institute (Kisarazu Chiba, 292-0818 Japan) for genetic analysis. Authors’ contributions KT and NK managed the patient, conceptualized this report, and drafted the paper; KA performed surgical interventions; YS and SO organized the grand design of this report. All authors have read and approved the manuscript. Funding This study was supported in part by JSPS KAKENHI grant number JP20K10832 (N. Kaku). The. funding body did not play any role in the design of the study and writing the text. Availability of data and materials The datasets supporting the conclusions of this article are all available in this manuscript. Declarations Ethics approval and consent to participate Genetic analysis was performed in a compliance with the institutional guideline of Kyushu University Hospital. Written informed consent was obtained from the patients’ parents. Consent for publication Written consent for publication was obtained from the patients’ parents. Competing interests The authors have no conflicts of interest to declare. Author details 1 Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3‑1‑1, Maidashi, Higashi‑ku, Fukuoka 812‑8582, Japan. 2 Emergency and Critical Care Center, Kyushu University Hospital, 3‑1‑1, Maidashi, Higashi‑ku, Fukuoka 812‑8582, Japan. 3 Present address: Department of Critical Care Medicine, Fukuoka Children’s Hospital, 5‑1‑1, Kashii‑Teriha, Higashi‑ku, Fukuoka 813‑0017, Japan. 4 Department of Neurosurgery, Kyushu University, 3‑1‑1, Maidashi, Higashi‑ku, Fukuoka 812‑8582, Japan. Received: 3 May 2022 Accepted: 10 December 2022 Page 4 of 4 References 1. Beez T, Steiger HJ, Hanggi D. Evolution of Management of Intracranial Aneurysms in children: a systematic review of the modern literature. J Child Neurol. 2016;31(6):773–83. 2. Sorteberg A, Dahlberg D. Intracranial non-traumatic aneurysms in children and adolescents. Curr Pediatr Rev. 2013;9(4):343–52. 3. Huang J, McGirt MJ, Gailloud P, Tamargo RJ. 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