Orbital Surgical Guidelines: Pediatric Considerations

Abstract

Pediatric orbital and skull base surgery comprises a wide array of tumors. An understanding of the location of the lesion, nature of the disease, and surrounding anatomy is paramount to surgical planning in these small spaces. The goals of pediatric skull base surgery are to avoid injury to the surrounding structures, minimize cosmetic deformities, and remove some or all of the tumors based on anticipated pathology and biologic cost of removal. Safe surgery on many of these tumors requires an understanding of the location of the lesion relative to the optic nerve or orbit. This is particularly challenging because the dimensions of the orbital confines change continuously as one navigates from rostral to caudal. Management of these tumors may require a multidisciplinary approach including orbital surgery, neurosurgery, otolaryngology, oral maxillofacial surgery, plastic surgery, and interventional neuroradiology.

Keywords: craniofacial; endoscopic orbital surgery; orbital approach; pediatric orbital surgery; pediatric orbital tumors; transcranial approach.

Fig. 1

Optic nerve glioma. ( a ) External photograph of left proptosis. ( b,c ) Sagittal and axial T1 magnetic resonance imaging (MRI) reveals an encased optic nerve glioma (*).

Fig. 2

Cavernous hemangioma. ( a ) Coronal T1 magnetic resonance imaging (MRI) of a left superomedial cavernous hemangioma (*). ( b ) A cryotherapy probe was used to engage the tumor.

Fig. 3

Cranio-orbital venous malformation. ( a ) External photograph of left orbital proptosis, complete ptosis, and ecchymosis. ( b,c ) Axial T1 and coronal T1 magnetic resonance imaging (MRI) of a congenital cranio-orbital venous malformation ( arrowheads ).

Fig. 4

Intraosseous hemangioma. ( a ) External photograph of left hyperglobus and proptosis. ( b,c ) Coronal computed tomography (CT) and magnetic resonance (MR) images of a highly vascular honeycomb intraosseous lesion within the maxilla eroding to the inferior orbit diagnostic of a cavernous hemangioma of the bone (*).( d ) Postoperative month 1 photograph following resection with repair using a custom 3D implant.

Fig. 5

Sickle cell infarction. ( a ) External photograph of a sickle cell disease hemoglobin SS patient with profound left orbital cellulitis and compressive optic neuropathy. ( b ) Coronal T1 magnetic resonance imaging (MRI) reveals an infarction of the marrow space of the greater wing of the sphenoid bone creating a large subperiosteal exudative effusion ( arrowhead s).

Fig. 6

Orbital osteoma. ( a ) External photograph of a right proptosis and medial fullness. ( b,c ) Axial and coronal computed tomography (CT) reveals a large osteoma extending from the right ethmoid sinus to the lateral orbit inferior to and displacing the optic nerve and extraocular muscles superiorly.

Fig. 7

Fibrous dysplasia. ( a ) External photograph of a teenage girl found to have Albright's syndrome. ( b ) Axial magnetic resonance imaging (MRI) reveals bilateral fibrous dysplasia (*) involving both orbits and narrowing the optic canals that underwent a series of bisphosphonate infusions.

Fig. 8

Chondrosarcoma. ( a ) External photograph of a patient with a right superolateral mass. ( b ) Axial computed tomography (CT) reveals a superior orbital lesion with areas of calcification (*) found to be a chondrosarcoma. ( c ) Postoperative photograph following an eyelid conjunctival sparing orbital exenteration with dermis fat graft to allow for an ocular prosthesis.

Fig. 9

Teratoma. ( a ) External photograph of a newborn with a large teratoma. ( b ) Axial computed tomography (CT) reveals a lesion extending from the anterior cranial vault through the superior orbital fissure into the lateral orbit and down into the pterygopalatine space and out of the mouth.