Korean J Perinatol.  2014 Dec;25(4):307-310. 10.14734/kjp.2014.25.4.307.

Glial Choristoma Accompanied with Severe Bony Defect in Orbit in a Newborn

Affiliations
  • 1Department of Pediatrics, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea. piena81@hotmail.com
  • 2Department of Radiology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea.
  • 3Department of Otorhinolaryngology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea.

Abstract

Glial choristoma is a mass-like lesion composed of otherwise normal, mature brain tissue that is isolated from the spinal cord and cranial cavity. In generally, this choristoma involves extracranial midline structures, especially the nose, as well as the nasopharynx, oropharynx and cleft. Glial choristomas rarely involve extracranial non-midline structures such as the scalp, orbit, middle ear or mastoid and account for approximately 10% of all glial choristomas. Moreover, such glial choristoma presentations are usually seen in adults, most of whom have predisposing factors such as trauma, surgery, or chronic inflammation. Here, we present a case of a glial choristoma that invaded a neonate's the nasopharynx, oropharynx, oral cavity and orbit.

Keyword

Brain; Choristoma; Heterotopia; Orbit; Newborn

MeSH Terms

Adult
Brain
Causality
Choristoma*
Ear, Middle
Humans
Infant, Newborn*
Inflammation
Mastoid
Mouth
Nasopharynx
Nose
Orbit*
Oropharynx
Scalp
Spinal Cord

Figure

  • Fig. 1. Gross photographs taken prior to surgery. A mass at the right buccal area (A) and a mass in the oral cavity with mouth was opened (B).

  • Fig. 2. The patient’s radiologic findings. Magnetic resonance imagine (MRI) showing a mixed cystic and solid lesion approximately 3.1x 2.4 cm with a bony defect in the right inferior orbital wall (A). MRI showing a multiloculated cystic mass approximately 2.5x2.2 cm with peripheral enhancement in the right masticator and buccal space as well as connected with cystic lesion in right foramen lacerum on MRI (B) and a computed tomography scan showing a bony defect in the right orbit floor (C).

  • Fig. 3. Patient’s pathologic findings. The mass is composed of mature glial tissue and choroid plexus-like area with papillary formation (A, Hematoxylin-and-eosin stain, x100). Both the glial and choroid plexus-like area showing a strong positive reaction for glial fibrillary acidic protein (B, x100).


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