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Quantitative indices for an intracranial aneurysm and subarachnoid hemorrhage in early childhood: a case report

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.

Peer Review reports

Background

Intracranial aneurysm (ICA) is a rare condition in children under 3 years of age [1, 2]. Infections, post-traumatic, 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 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 contrast-enhanced CT in our hospital confirmed the hemorrhage 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, 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.

Fig. 1
figure 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)

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, 7,8,9,10,11,12]. Although variable degrees of neurological deficits were left, none of them died or failed to receive neurosurgical 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.

Table 1 Summary of patients with intracranial aneurysms less than 3 years of age (reports in 2021–2022)

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 [3,4,5, 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 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.

Availability of data and materials

The datasets supporting the conclusions of this article are all available in this manuscript.

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

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Acknowledgements

We thank Kazusa DNA Research Institute (Kisarazu Chiba, 292-0818 Japan) for genetic analysis.

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.

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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.

Corresponding author

Correspondence to Noriyuki Kaku.

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Genetic analysis was performed in a compliance with the institutional guideline of Kyushu University Hospital. Written informed consent was obtained from the patients’ parents.

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Written consent for publication was obtained from the patients’ parents.

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The authors have no conflicts of interest to declare.

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Tetsuhara, K., Kaku, N., Arimura, K. et al. Quantitative indices for an intracranial aneurysm and subarachnoid hemorrhage in early childhood: a case report. BMC Neurol 22, 488 (2022). https://doi.org/10.1186/s12883-022-03022-4

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Keywords

  • Intracranial aneurysm
  • Modified Alberta stroke program early CT score (mASPECTS)
  • Outcome
  • And children