Purpose or Case Report: Benign macrocrania (BM) is primarily diagnosed by ultrasound (US). Many children with BM have developmental delay which triggers neurosurgical evaluation and CT or MR brain imaging due to concerns for communicating hydrocephalus. The purpose of this study was to determine if CT and/or MR imaging changed the diagnosis or management in those children whose primary complaint was macrocephaly with or without developmental delay.
Methods & Materials: IRB approval was obtained for this retrospective study. The radiology database was searched to identify patients diagnosed with BM via head US between 2006 and 2013. Those with follow up CT or MR imaging were identified and compared to initial US studies to identify change in diagnosis. Chart review was performed to determine the neurologic and clinical status of each patient and the consequent clinical management.
Results: Initial search identified 467 patients (68.6 % male) with a head US and diagnosis of BM (mean age of 6.47 +/- 2.43 months). In this group, 81 (17.5%) went on to have head CT or MR imaging done. 2/81 (2%) had neurological findings with additional significant findings on MRI. 6/79 (7%) had a stable diagnosis of BM with incidental findings including Chiari I (3), small subdural bleeds (2), arachnoid cyst (1), frontal bone dermoid (1) and nonspecific white matter disease (1), stable over two studies. None of these cases resulted in a change in management. 64.6% of all patients had neurosurgical evaluations, none requiring intervention for communicating hydrocephalus. Of the 177 infants with available developmental follow up records, 94 (53.1%) had speech, fine motor or gross motor delay. At the time of the last available clinical follow up 58/94 (61.7%) still showed some delay (average age at last follow-up was 38.0 +/- 2.0 months).
Conclusions: Our study suggests that patients diagnosed with BM with or without developmental delay and without other focal neurological findings do not require subsequent brain CT/MR imaging or neurosurgical evaluation. Routine developmental surveillance by primary care providers with referral to appropriate developmental therapy services is the best clinical management. This change in practice would decrease costs incurred by unnecessary imaging, would free up clinic appointments for neurosurgeons, and avoid risks associated with radiation and sedation.