Pediatric Endoscopic Skull Base Surgery: Collaborative Approaches and Techniques
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What the experts said
Endoscopic skull base surgery was slow to propagate into the pediatric world despite evolving as an adult specialty.
Many pediatric neurosurgeons felt there wasn't enough room and hadn't had enough training in endoscopic skull-based techniques.
Strong collaboration between pediatric neurosurgery and adult endoscopic skull base surgery is the key to program success.
Children are not just little adults - there are unique special things about the pediatric population that adult surgeons may not be aware of or able to manage as well as pediatric neurosurgeons.
Age 4 and above is a comfortable threshold after which most endoscopic skull base procedures can be performed, though younger is possible.
In the pediatric population, the conchal type of sphenoid sinus is most common, and this enlarges over time.
Even with a conchal sphenoid sinus, much of the bone in the middle of the sphenoid is very soft and can be easily drilled out with a diamond drill to create a pneumatized sphenoid sinus.
After age 4, most conchal sphenoid sinuses are eliminated based on radiographic studies.
Inter-carotid distance increases from 12-13 millimeters at age 5 to about 15 millimeters at age 15, but this 2-3 millimeter difference doesn't make a dramatic difference to surgical procedures.
100% of encephaloceles are now done using an endonasal approach, even fairly large ones, whereas traditionally these would be removed with a bifrontal craniotomy.
For encephalocele repair, intrathecal fluorescein is given, a lumbar drain is placed, and all encephalocele tissue must be resected until gone, with cauterization of the edges.
The closure technique for encephaloceles involves an inlay of Duragard on the inside, then an onlay of fat and a nasal septal flap.
For encephaloceles, a lumbar drain is left in for a couple of days postoperatively because some patients have increased intracranial pressure, allowing pressure decrease while the closure tightens.
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.
In pediatric patients, a small rim of craniopharyngioma tumor is often intentionally left on the hypothalamus to avoid causing morbid obesity and cognitive maldevelopment.
Craniopharyngiomas in the pediatric age group are mostly adamantinomatous type, more likely in the sella, and more likely calcified, while adults have both adamantinomatous and papillary types.
Papillary craniopharyngiomas with BRAF mutation are more likely suprasellar and less likely calcified.
The endonasal view for craniopharyngiomas provides better visualization of the stalk, third ventricle, and hypothalamus with less retraction on optic nerves and brain compared to transcranial approaches.
For craniopharyngiomas, the surgical goal is to save the pituitary stalk as long as possible, but if the stalk is completely infiltrated, it will be sacrificed to achieve cure.
Superior hypophyseal arteries should be preserved during craniopharyngioma surgery because they feed superiorly to the chiasm, and their sacrifice could cause visual loss.
The endonasal approach for craniopharyngiomas results in no retraction of the brain, so there is no FLAIR signal in the brain after tumor removal.
Comparing endonasal to transcranial surgery for craniopharyngiomas, the extent of resection for endonasal surgery is greater than for transcranial surgeries.
Visual outcome after endonasal surgery for craniopharyngiomas is better than after transcranial surgery because the approach is from below without manipulation of optic nerves.
In a series of 85 craniopharyngioma patients, gross total resection rate was 86%, greater than 95% resection achieved in 95% of patients, visual improvement in 78%, and CSF leak rate 2.4%.
For craniopharyngiomas, diabetes insipidus and panhypopituitarism are common when pursuing gross total resection.
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, visual function was stable or improved in over 70%, and all children except one returned to an academic environment, with 10 in a grade appropriate for age.
Other than age less than 4, there are no significant anatomical concerns in the pediatric population for endoscopic skull base surgery beyond the perceived limitations of limited nares size and non-pneumatized sinuses.
The same instruments, scope holder, and scopes used in adult endoscopic skull base surgery are used in pediatric cases.
When nostril width is a concern, the irrigation sheath can be removed from the scope, reducing its diameter from 4 millimeters to 2.7 millimeters.
For encephalocele repair, the approach is to first repair the encephalocele and see if it holds; shunting is only performed for patients with recurrent encephaloceles or clear hydrocephalus.
Patients are placed on acetazolamide for about 2 weeks after encephalocele repair to ensure healing.
Out of approximately 40 encephalocele cases, 3 or 4 patients required shunting postoperatively.
Intrathecal fluorescein protocol uses a low dose of 0.3mL of 10% fluorescein diluted in 10cc of CSF, with pretreatment using Decadron and Benadryl.
Literature has shown adverse effects to high doses of intrathecal fluorescein, but with low-dose protocol and steroid pretreatment, no allergic reactions or arachnoiditis have been observed.
The long-term implications of arrested skull base growth in a child with lack of pneumatization 10 years down the road are unknown, though followed patients have not shown long-term problems.