Endoscopic endonasal skull base surgery in the pediatric population

Clinical article

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Object

The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies.

Methods

A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed.

Results

A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eighty-five patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up.

Conclusions

Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population.

Abbreviations used in this paper:ACTH = adrenocorticotropic hormone; CN = cranial nerve; EEA = endoscopic endonasal approach; EES = endoscopic endonasal surgery; GTR = gross-total resection; RCC = Rathke cleft cyst; SIADH = syndrome of inappropriate antidiuretic hormone.

Article Information

Address correspondence to: Elizabeth C. Tyler-Kabara, M.D., Ph.D., Department of Pediatric Neurosurgery, Children's Hospital of Pittsburgh, 4401 Penn Avenue, 4th Floor Faculty Pavilion, Pittsburgh, Pennsylvania 15224. email: Elizabeth.Tyler-Kabara@chp.edu.

A portion of this work was presented in abstract form at the 22nd Annual Meeting of the North American Skull Base Society, Las Vegas, Nevada, February 2012.

Please include this information when citing this paper: published online December 14, 2012; DOI: 10.3171/2012.10.PEDS12160.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Gross-total resection of a juvenile nasopharyngeal angiofibroma in a 16-year-old boy who presented with nasal obstruction and snoring. Upper: Preoperative coronal and axial T1-weighted MR images obtained after contrast administration, showing an enhancing mass that occupies the left nasal cavity, maxillary sinus, and pterygopalatine fossa and extends into the sphenoid sinus and pharynx. Lower: Postoperative coronal and axial T1-weighted MR images obtained after contrast administration, demonstrating the total resection of the mass 3 months after EES.

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    Gross-total resection of a craniopharyngioma in a 14-year-old girl with headache, visual impairment, hypopituitarism, and diabetes insipidus. Upper: Preoperative coronal and sagittal T1-weighted MR images obtained after contrast administration, showing a suprasellar heterogeneous lesion that extends to the third ventricle and the interpeduncular cistern. Enlargement of the temporal horns and the body of the right lateral ventricle is also seen. Lower: Postoperative coronal and sagittal T1-weighted MR images obtained after contrast administration, demonstrating GTR of the lesion 29 months after EES. The linear contrast-enhanced area (arrow) represents the vascularized nasoseptal flap that was used for reconstruction of the bony defect. The artifacts on the coronal image are from the ventriculoperitoneal shunt that was placed for the developed hydrocephalus. Postoperatively, the patient's vision normalized; she remained on a regimen of hormonal replacement therapy.

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