Developmental Considerations in Pediatric Skull Base Surgery

Abstract

Objectives To review developmental surgical anatomy and technical nuances related to pediatric skull base surgery. Design Retrospective, single-center case series with literature review. Setting MD Anderson Cancer Center. Participants Patients undergoing pediatric skull base surgery. Main Outcome Measures Review developmental anatomy of the pediatric skull base as it relates to technical nuance of various surgical approaches and insight gained from a 25-year institutional experience with this unique patient population. Results Thirty-nine patients meeting these criteria were identified over a 13-year period from 2003 to 2016 and compared to a previously reported earlier cohort from 1992 to 2002. The most common benign pathologies included nerve sheath tumors (11%), juvenile nasopharyngeal angiofibromas (9.5%), and craniopharyngiomas (4.8%). The most common malignancies were chondrosarcoma (11%), chordoma (11%), and rabdomyosarcoma (11%). Varied surgical approaches were utilized and were similar between the two cohorts save for the increased use of endoscopic surgical techniques in the most recent cohort. The most common sites of tumor origin were the infratemporal fossa, sinonasal cavities, clivus, temporal bone, and parasellar region. Gross total resection and postoperative complication rates were similar between the two patient cohorts. Conclusions Pediatric skull base tumors, while rare, often are treated surgically, necessitating an in depth understanding of the anatomy of the developing skull base.

Keywords: developmental anatomy; pediatric skull base; surgery.

Fig. 1

Sagittal and coronal computed tomography scans in a 3-year-old with clival chordoma. Note the lack of aeration of the sphenoid sinus and the multiple unerupted teeth. These anatomic findings may significantly impact the choice and difficulty of the operative approach.

Fig. 2

Axial magnetic resonance images of 16-year-old with left acoustic neuroma. This child had absence of functional hearing and a primarily intratemporal location of his acoustic neuroma. A translabyrinthine approach was utilized for complete tumor resection.

Fig. 3

( A ) Sagittal computed tomography scans in 14-year-old demonstrating the preoperative presence of platybasia. Following endoscopic resection of the chordoma, the craniocervical alignment remained stable. Three months later cranial settling was noted and there was evidence of asymptomatic but significant instability necessitating occipitocranial fixation. ( B ) These magnetic resonance images are from the same patient. Imaging reveals a gross total tumor resection. Note the increased kinking of the brainstem associated with the cranial settling.

Fig. 4

( A ) Preoperative and ( B ) postoperative axial computed tomography and coronal post-contrast magnetic resonance imaging of 8-year-old with primary right temporal bone osteoblastoma. A total resection was accomplished. An experienced neuro-otologist is critical to safely navigate the pediatric temporal bone (All images are courtesy of the University of Texas M.D. Anderson Cancer Center Department of Neurosurgery and are used with permission.)