Extended Cranial Ultrasound Views in Infants with Acute Brain Stem/Infratentorial Lesions: Diagnosis of a Progressive Midline Glioma in a 6-Week-Old Infant

Central nervous system (CNS) tumors are the most common solid tumors in children and adolescents. However, in neonates and children aged younger than a year, they are very rare. Clinical presentation in neonates is often subtle and nonspecific. When neurological symptoms are apparent at this age, cranial ultrasound (CUS) is often done as the initial evaluation, with a standard approach through the anterior fontanel (AF), followed by further imaging, such as magnetic resonance imaging (MRI), if necessary. We report the first neonatal case of a rapidly progressive diffuse midline glioma positive for histone H3 K27M mutation (World Health Organization [WHO] grade IV) in which using extended (transmastoid) CUS studies through the mastoid fontanelle (MF) in the second month of life defined the lesion in the brainstem.

Congenital Implantation Cholesteatomas of the Occipitoparietotemporal Junction

Two cases of cranial cholesteatomas centered at the occipitoparietotemporal junction are presented, and 12 similar cases reported as diploic cholesteatomas involving the temporal bone are reviewed. Among the reported diploic cholesteatomas, 3 involved the occipitoparietotemporal junction and had the same clinical characteristics as the ones presented here. These 5 lesions did not produce expansion of the cranial tables, they had a predominantly intracranial growth, and their matrix was bonded to the dura, so that their complete excision was prevented. It is proposed that cranial cholesteatomas involving the occipitoparietotemporal junction arise, not from within the tables of the skull, but from squamous cells trapped during closure of the mastoid fontanelle and formation of the suture. Hence, they should be recognized as having an origin different from that of diploic lesions, and termed congenital implantation cholesteatomas.