Background The fully endoscopic expanded endonasal approach (EEA) has been shown to be safe
and efficacious in pediatric patients. However, in the very young patient (≥ 6), the
anatomical challenge of working through a small nasal corridor is problematic. The
ability to repair the skull base and use a nasoseptal flap (NSF) has also been called
into question. The objective of this study was to review skull base resections, patient
age, tumor pathology, extent of resection, postoperative cerebrospinal fluid (CSF)
leaks, and complications in patients aged 6 years and younger.
Methods A retrospective chart review was conducted on pediatric patients aged 6 years and
younger who underwent EEA skull base resections over a 3-year period (June 2014 through
June 2017).
Results: Eight children aged 6 years and younger (range = 1−6 years, mean = 4.3 years) underwent
an EEA for resection of their skull base tumors, with a mean follow-up of 1.45 years.
The tumor pathology included one craniopharyngioma (age 4 years), one Rathke cleft
cyst (age 16 months), one encephalocele (age 6 years), one hamartoma (age 13 months),
two chordomas (both age 6 years), one dermoid cyst (age 4 years), and one neuroblastoma
(age 6 years).
A GTR was obtained in the craniopharyngioma, dermoid, neuroblastoma, and hamartoma
cases. The chordoma cases were both addressed with multiple staged resections, and
resulted in residual tumor adjacent to the carotid and brainstem that were addressed
with postoperative proton therapy. The Rathke cleft cyst was opened and drained. The
encephalocele was completely resected and paracribiform skull base repaired. There
are no tumor recurrences to date.
A high-flow intraoperative CSF leak was encountered in the craniopharyngioma case,
and both chordoma cases. All CSF leaks were repaired with NSFs that were able to reach
and fully cover the defect. There were no postoperative CSF leaks. The patient with
a craniopharyngioma suffered from postoperative hypopituitarism with TSH and ADH deficiencies.
The patient with the hamartoma had recurrent epistaxis secondary to nasal dryness
from turbinate destruction by the tumor. The neuroblastoma patient expired 8 months
postresection secondary to metastatic disease. There were no orbital complications
in any case.
Conclusion In the very young patient, age 6 years and less, the fully endoscopic EEAs for skull
base tumors is a safe and effective surgical technique. Skull base defects are able
to be effectively addressed with typical repairs including NSFs.
