Whether the Spetzler-Martin grading scale is adequate to children with intracranial arteriovenous malformations?

Background: Intracranial arteriovenous malformation (ICAVM) is a rare and life-threatening disease. Clinical presentations of pediatric ICAVMs are variable and unpredictable. The Spetzler-Martin grading scale (SMGS) is a commonly used system for predicting prognoses and guiding therapeutic protocols. However, the application of this system to children is controversial. Objective: The purposes of this study were to retrospectively analyze clinical presentations of children with ICAVM and to investigate the relationship between the SMGS and the prognoses of children with ICAVM. Materials and Methods: Based on the International Classification of Diseases, 9 th revision, Clinical Modification (ICD-9-CM) code 747.81, we retrospectively reviewed eight children with ICAVMs admitted to a medical center from 1991 to 2012. The primary selection criterion was the diagnosis of the ICAVM, which was not irrelevant to traumatic injury or known coagulopathy. Patients′ outcomes were determined based on evaluation records of admission, discharge, and clinic visit 6-month after the ICAVM episode. For purposes of data analysis, outcomes were divided into "1 = death," "2 = persistent vegetative state," "3 = severe disability," "4 = moderate disability," and "5 = low disability." The relationship between the Glasgow outcome scale (GOS) and SMGS was analyzed using a scatter plot and Spearman′s correlation coefficient. Results: A total of eight patients with at least follow-up 6-month consisted of four males (50.0%) and four females (50.0%). Their ages ranged from 7 to 15 years, and the mean age was 12.63 years. Common sites of ICAVM nidus included basal ganglia (n = 3, 37.5%), and frontal-temporal region (25.0%). 62.5% of patients experienced bleeding on the right side of the brain. About 62.5% of patients complained of headache. 62.5% of patients showed loss of consciousness. About 37.5% of patients showed generalized seizures. The SMGS were Grade I in one patient (12.5%), Grade II in two patients (25.0%), Grade III in two patients (25.0%), and Grade IV in three patients (37.5%). The clinical outcomes for the overall series were 87.5% excellent or good (GOS score equal or above 4) and only one case dead. The correlation between SMGS and GOS, analyzed by Spearman′s correlation coefficient, was insignificant. Treatment, either by stereotactic radiosurgery (SRS) or surgery, was irrelevant to the prognosis of patient with ICAVM. Conclusion: Intracranial arteriovenous malformation is a life-threatening disease in children. Headache is a warning. Clinical presentations, including altered level of consciousness and generalized seizures in patient with ICAVM may suggest bleeding. The use of this system in children with ICAVM should be considered patients′ age for their better potential neuroplasticity and good healing capacities in higher grade of patients. Treatment, either by surgery or SRS, may be irrelevant to patients′ prognoses in this study