We present a case of epignathus (oral teratoma) with midline intracranial associated malformations. This is a rare report of epignathus diagnosed in utero with fetal MRI description of these midline brain malformations, including duplication of the pituitary gland. This case is an example of how these intracranial findings led to the correct diagnosis of a small fetal oral mass.

Case Report. Isolated carotid artery originating from the pulmonary trunk is an exceedingly rare anomalous origin of head and neck vessels. We present this finding, along with a persistent embryonic trigeminal artery, in a male infant with multiple cardiac defects and other congenital anomalies associated with CHARGE syndrome. After extensive investigations, cardiac catheterization revealed the anomalous left common carotid artery arising from the cranial aspect of the main pulmonary artery. There was retrograde flow in this vessel, resulting from the lower pulmonary pressure, essentially stealing arterial supply from the left anterior cerebral circulation. The persistent left-sided trigeminal artery provided collateral flow from the posterior circulation to the left internal carotid artery territory, allowing for safe ligation of the anomalous origin of the left common carotid artery, thereby reversing the steal of arterial blood flow into the pulmonary circulation and resulting in a net improvement of cerebral perfusion. Conclusion. The possibility of this vascular anomaly should be considered in all infants with CHARGE syndrome. Surgical repair or ligation should be tailored to the specific patient circumstances, following a careful delineation of all sources of cerebral perfusion.

Juvenile pilocytic astrocytoma, the most common pediatric central nervous system (CNS) neoplasm, characteristically displays an indolent growth pattern and rarely demonstrates metastatic dissemination. Reports of infections mimicking CNS metastatic disease are also rare and can impact treatment. We report the youngest known case of a child with a CNS Nocardia farcinica infection who had a known cerebellar pilocytic astrocytoma, review other infections that may masquerade as CNS neoplasms, and discuss N. farcinica CNS infections.

Incidentally noted ICA calcifications are a common finding on temporal bone CT in children, most likely a physiologic response to turbulent flow at natural bends in the artery rather than secondary to underlying disease predisposing to early atherosclerotic calcification.

BACKGROUND: There are no standard practice guidelines for cerebrospinal (CSF) diversion for diffuse intrinsic pontine glioma (DIPG), nor clear understanding of potential for palliation and life-prolongation. We evaluated CSF diversion characteristics in children with DIPG to determine incidence, indications, symptom effects, and survival. METHODS: Data were extracted from subjects registered in the International DIPG registry (IDIPGR). Univariable analyses was performed using the Fisher’s exact test or Wilcoxon rank sum test. Survival was estimated using the Kaplan-Meier method. RESULTS: Evaluable patients (n=542) met criteria for DIPG diagnosis by central radiologic review; of those, 126 (23%) had permanent CSF diversion. Median time from diagnosis to diversion was 0.5 months (IQR 0.1-4.5 months). Those with permanent diversion were significantly younger (median 5.4 years vs 7.0 years, p<0.001) and had higher incidence of hydrocephalus at diagnosis (65.3% vs 11.9%, p<0.001). Permanent CSF diversion did not significantly impact overall survival (OS) (p=0.4), even amongst the 124 patients with hydrocephalus at presentation (p=0.20). Those with permanent diversion prior to radiation therapy demonstrated longer median OS than those in whom diversion was placed after radiation (14.3 vs 9.6 months, p=0.001). Patients reported significantly less headache and vomiting at last follow up after permanent CSF diversion compared to pre-diversion (p<0.0001 and p=0.001, respectively), however steroid use was also significantly higher at last follow-up after CSF diversion (p<0.001). CONCLUSIONS: Amongst an international cohort, DIPG patients who had permanent CSF diversion were significantly younger and had higher rates of hydrocephalus at initial presentation than those without permanent diversion. Symptoms of increased intracranial pressure improved in those with CSF diversion, although a direct effect may be confounded by increased steroid use. Permanent CSF diversion did not prolong overall survival in this large cohort of patients, even amongst those who presented with hydrocephalus.