Children with Macrocrania: Clinical and Imaging Predictors of Disorders Requiring Surgery

Abstract

BACKGROUND AND PURPOSE: Macrocrania is a common pediatric clinical condition affecting up to 5% of the population. The purpose of this study was to determine clinical and imaging predictors that are useful in the differentiation of disorders requiring surgical treatment from those that can be treated medically in children with macrocrania.

METHODS: In a 3-year 7-month retrospective study, 88 patients (median age, 8 months; interquartile range, 5−13 months) with macrocrania and no known underlying neurologic disorder underwent imaging of the brain (sonography, n = 36; CT, n = 31; MR imaging = 21). The study was conducted in a pediatric tertiary care referral center. Clinical and imaging data were correlated to final diagnosis by means of logistic regression and receiver operating characteristic curves.

RESULTS: Sixteen (18%) of the patients had disorders requiring surgery: communicating hydrocephalus, n = 7; noncommunicating hydrocephalus, n = 3; hemorrhagic subdural collections, n = 3; neoplasm, n = 1; encysted cavum septi pellucidi, n = 1; and vein of Galen malformation, n = 1. Clinical predictors of disorders requiring surgery included vomiting (P = .007), labor instrumentation (P = .026), developmental delay (P = .008), and abnormal neurologic findings (P = .028). Imaging predictors of disorders requiring surgery included a focal space-occupying lesion (P < .0001) and moderate-to-severe ventriculomegaly (P < .0001). The diagnostic sensitivity of the combination of independent clinical and imaging predictors was higher than that of independent clinical predictors alone, being 100% (95% confidence interval = 96.9%, 100%) and 93.8% (95% confidence interval = 88.7%, 98.8%), respectively. A trend indicated that the area under the receiver operating characteristic curve for clinical plus imaging findings (0.95) was greater than that for clinical findings alone (0.85) (P = .09). An increase in the number of clinical and imaging predictors was highly correlated with an increased risk of a disorder requiring surgery (P < .0001).

CONCLUSION: Baseline neuroimaging is indicated for children with macrocrania because the combination of clinical and imaging predictors has the best diagnostic performance in determining the need for surgical versus nonsurgical management.