Enlargement of the Internal Auditory Canal and Associated Posterior Fossa Anomalies in PHACES Association

Area of Science:

• Neurology
• Pediatrics
• Radiology

Background:

• Posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, and sternal or supraumbilical defects (PHACES) association is a complex condition.
• Enlargement of the internal auditory canal (IAC) has been anecdotally noted in some patients with PHACES.

Purpose of the Study:

• To investigate the prevalence of enlarged IAC in children with PHACES.
• To identify associated imaging findings, including infantile hemangioma within the IAC.
• To explore the potential genesis of IAC enlargement in the context of PHACES.

Main Methods:

• Retrospective review of MR imaging studies in children diagnosed with PHACES association.
• Detailed analysis of IAC for enlargement and abnormal enhancement.
• Assessment for other posterior fossa abnormalities, such as cerebellar hypoplasia and calvarial deformity.

Main Results:

• Enlargement of the IAC was observed in a subset of patients with PHACES.
• Associated findings included cerebellar hypoplasia, petrous ridge prominence, and calvarial deformity.
• The possibility of infantile hemangioma within the IAC was considered.

Conclusions:

• Enlargement of the IAC may be associated with PHACES.
• This finding might indicate a generalized malformation of the posterior fossa in PHACES patients.
• Further research is warranted to elucidate the exact relationship and underlying mechanisms.