Pediatric Endonasal Skull Base Surgery: Pediatric Endoscopic Neurosurgery 2018
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What the experts said
Age 4 and above is generally a comfortable threshold for pediatric endonasal skull base surgery, though procedures are possible at younger ages.
In pediatric patients, conchral (non-pneumatized) sphenoid sinuses can be converted to pneumatized sinuses by drilling out soft, vascular bone in the middle with a diamond drill until reaching harder bone over the carotid artery and planum.
After age 4, most conchral sphenoid sinuses are eliminated, with progressive pneumatization occurring over time.
The intercarotid distance increases from approximately 12-13 millimeters at age 5 to about 15 millimeters at age 15, but this 2-3 millimeter difference does not dramatically affect surgical feasibility.
Essentially 100% of encephaloceles are now managed using an endonasal approach rather than bifrontal craniotomy.
For encephalocele repair, intrathecal fluorescein is administered, a lumbar drain is placed, all encephalocele tissue is resected and cauterized back to the edges, and closure is performed with inlay Duragen, onlay fat, and nasal septal flap.
Lumbar drains are left in place for a couple of days after encephalocele repair because some patients have increased intracranial pressure, allowing pressure reduction while the closure tightens.
In pediatric craniopharyngioma surgery, a small rim of tumor is often intentionally left on the hypothalamus to avoid causing morbid obesity and cognitive maldevelopment.
Juvenile nasopharyngeal angiofibromas are very vascular tumors that are typically embolized beforehand but still bleed quite a bit during surgery.
Most juvenile nasopharyngeal angiofibromas arise from the pterygopalatine fossa, requiring wide exposure of the back wall of the maxillary sinus.
Craniopharyngiomas have two age peaks of presentation; pediatric cases are mostly adamantinomatous type, more likely in the sella, and more likely calcified, while adult cases include both adamantinomatous and papillary types.
Papillary craniopharyngiomas with BRAF mutation are more likely suprasellar and less likely calcified.
The endonasal view for craniopharyngioma provides better visualization of the stalk, third ventricle, and hypothalamus with less retraction on optic nerves and brain compared to transcranial approaches.
In craniopharyngioma surgery, the pituitary stalk is preserved when possible, but is sacrificed if completely infiltrated with tumor in favor of achieving a cure.
Superior hypophyseal arteries should be preserved during craniopharyngioma surgery because they feed the chiasm superiorly, which is critical for preventing visual loss.
After endonasal craniopharyngioma resection, there is no flare signal in the brain on postoperative imaging because there is no brain retraction during the procedure.
Extent of resection for craniopharyngioma is greater with endonasal surgery compared to transcranial approaches.
Visual outcomes after endonasal craniopharyngioma surgery are better than after transcranial surgery because the approach is from below without manipulation of the optic nerves.
In a series of 85 craniopharyngioma patients, gross total resection was achieved in 86%, with greater than 95% resection in 95% of patients.
Visual improvement occurred in 78% of craniopharyngioma patients after endonasal surgery.
The CSF leak rate in the craniopharyngioma series was 2.4%.
Diabetes insipidus and panhypopituitarism are fairly frequent outcomes when pursuing gross total resection of craniopharyngioma.
In 11 pediatric craniopharyngioma patients (average age 8 years), gross total resection rate was 45%, but intentional subtotal resection is often the goal in the pediatric population.
In the pediatric craniopharyngioma series, only 2 patients had greater than 9% increase in BMI, visual function was stable or improved in over 70%, and all children except one returned to an academic environment with 10 in grade-appropriate settings.
Complications in the pediatric craniopharyngioma series included 1 CSF leak, 1 visual loss, and 1 abscess.
When nostril width is limiting, the irrigation sheath can be removed from the endoscope, reducing the diameter from 4 millimeters to 2.7 millimeters.
The same instruments, scopes, and scope holders used in adult endonasal surgery are used in pediatric cases.
For encephaloceles associated with intracranial hypertension, the approach is to first repair the encephalocele and see if it holds, placing patients on acetazolamide for about 2 weeks postoperatively.
Shunting for encephaloceles is reserved for patients with clearly evident hydrocephalus or those with recurrent encephaloceles after initial repair.
Out of approximately 40 encephalocele cases, shunting was required 3 or 4 times.
Intrathecal fluorescein is administered at a low dose of 0.3 mL of 10% fluorescein diluted in 10 cc of CSF, with pretreatment using Decadron and Benadryl to prevent adverse effects.
Literature has shown adverse effects including allergic reactions and arachnoiditis with high doses of intrathecal fluorescein, but low-dose protocols with steroid pretreatment have not resulted in such problems in the speaker's experience.