Endoscopic Treatment of Craniosynostosis: Pediatric Endoscopic Neurosurgery 2018
With Dr. Mark Proctor · StayCurrentMD
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What the experts said
Human skull is made up of 5 major bones separated by growth plates called sutures.
Humans have very rapid brain growth in the first year of life, slowing considerably over the second year; past 2 years of age sutures play a very small role in skull or brain growth.
Virchow's law defines that skull growth is normally perpendicular to the sutures; if bone is closed, growth occurs parallel to sutures due to compensatory overgrowth in other areas.
Synostosis affects about 1 in every 2000 live births, with sagittal being by far the most common in about half of children.
In sagittal synostosis, the back of the head is the narrowest part (whereas normally it is the widest), with wide and bossed frontal region.
In unilateral coronal synostosis, there is orbital dystopia where the orbit on the affected side is higher and shallower, and the nose deviates toward the affected side.
Lambdoid synostosis is very rare, representing about 1 to 2% of all synostosis cases; Boston Children's sees about 100 new synostosis patients per year and averages 1 lambdoid case annually.
In lambdoid synostosis, the mastoid should be low (extended) on the affected side, which helps distinguish it from deformational changes.
Strip craniectomy results were poor historically, with about one-third of patients having bones fuse together before significant correction, leading to adoption of larger cranial vault reconstructions.
David Jimenez and Constance Barone in the mid-1990s pioneered endoscopic synostosis surgery with smaller incisions, less blood loss, and adjuvant helmet therapy.
Open surgery is a mechanical operation where bones are repositioned and fixed, but those bones don't grow normally over time; results at end of surgery aren't completely predictive of outcomes 5 or 10 years later.
Endoscopic surgery is a release procedure that relies on brain growth to move bones out over time or requires adjuncts like springs, distractors, or helmets to direct growth.
Conceptually, endoscopic surgery turns synostosis into a deformational problem by opening bones to make them malleable, then reshaping with a helmet.
Laparoscopic cholecystectomy was first reported in 1987 by a team in France and met significant early skepticism before becoming standard of care.
Only surgeons involved with open cholecystectomy and management of its potential complications should perform laparoscopic cholecystectomy (quote from historical article applied to craniosynostosis context).
CDC parameters of care for synostosis (2010–2012) consider endoscopic surgery a viable treatment option but stress the need for a very experienced team.
For sagittal synostosis endoscopic surgery, a 0-degree endoscope is used; Proctor avoids using high-quality neurosurgical scopes to prevent damage in this relatively blunt procedure.
Meticulous technique is necessary to keep blood transfusion rates down in endoscopic craniosynostosis surgery.
For sagittal synostosis, two incisions are made (anterior and posterior), burr holes created and expanded with Kerrison rongeurs to about a 2 cm gap.
Recent studies from Hopkins, DC, and Saint Louis show that narrow (2 cm) bone strips are just as effective as wide (6 cm) strips; most centers now use narrow strips with no barrel staves.
Helmets allow real-time adjustment (e.g., if top of head is getting flat, can adjust for more rounding), whereas springs or distractors cannot be adjusted.
Endoscopic treatment leads to sustained changes in cranial index over time, with results very similar to open operation from a cranial index perspective (compared by multiple groups).
In unilateral coronal synostosis treated endoscopically, neo-suture formation can occur, making it appear as if the patient never had a fused suture.
3D photogrammetry studies show facial asymmetry improved significantly more in the endoscopic group than in the frontal orbital advancement group, likely due to early release.
Astigmatism improved much better with endoscopic surgery compared to open surgery.
First 100 consecutive endoscopic cases (all synostosis types): mean surgical time 48 minutes, estimated blood loss 23 mL, 8 transfusions, median hospital stay 1 day.
Weight under 5 kg was a risk factor for transfusion; now waiting until over 5 kg for all patients, transfusion rates down to about 3%.
Cost of endoscopic treatment is 40% of open operation; three studies (Boston, Saint Louis, Midwest US) show consistent results.
Boston cost study included all hospital costs, home costs, and gas mileage for families traveling to orthotist appointments.
In experienced centers, both open and endoscopic craniosynostosis surgery should be very safe procedures.
Centers should have access to both minimally invasive and open techniques, and provide comprehensive care including surgery, neuropsych testing, and orthotist access.
Endoscope exposure to dura during dissection is very brief (20–30 seconds on average for sagittal synostosis); light source typically used at 70–75% (not 100%).
Endoscope appears to generate less heat than the drill; no injuries from scope heat have been observed.
Ideal surgical age for endoscopic craniosynostosis is about 10 to 12 weeks (3 months), when brain growth is most rapid.
Oldest patient treated endoscopically was 7 months of age (mild sagittal synostosis); result was reasonable but correction is less robust at older ages due to reduced brain growth.
Would not offer endoscopic surgery past 6 months for severe deformity (e.g., cranial index 0.62); may extend to 5–6 months for milder cases (cranial index 0.7 or 0.72).
Historical strip craniectomy had 20–30% rate of suture closing back before significant correction; current approach differs by using adjuvant therapy (helmets, springs, distractors) to direct growth.
In helmeted sagittal synostosis, the goal is 2:1 growth ratio (width to length) during helmet course; starting cranial index 0.75 reaches about 0.8 after 6 months.
Head circumference must be tracked closely during helmet therapy; do not want to see fall-off on growth curve; ideally should see a jump up with the operation.
Early endoscopic cases used two large IVs and an arterial line (treated like open cases); current standard is two IVs only, no A-line, no Foley catheter.
Some endoscopic craniosynostosis patients could truly go home the same day, though Proctor has never been bold enough to do so; most stay overnight (often as 23-hour observation rather than formal admission).
Average sagittal synostosis case is so classic that most surgeons can diagnose without imaging; imaging obtained if any question exists to avoid operating on open suture or missing a fused suture.
Well under 10–20% of craniosynostosis patients require imaging to make the diagnosis.
For sagittal synostosis, plain X-ray may suffice instead of CT scan; unilateral coronal can almost always be diagnosed on exam (nasal deviation, eye height, ear position).
Would never consider operating on lambdoid synostosis without a CT scan; it is a very difficult diagnosis to make clinically.
Ultrasound is increasingly used to show open versus closed sutures without radiation exposure; literature on this is growing.
MRI black-bone studies are a potential viable technique to show suture status and brain detail better than other modalities, though not yet adopted at Boston Children's.
For metopic synostosis, helmet can be stopped as soon as desired shape is achieved (as short as 3 months); there is essentially no regression.
For sagittal synostosis, there is definite regression; patients lose on average 0.02 cranial index between 1 and 2 years of age.
Average helmet duration for sagittal synostosis is 7 months from surgery; Proctor pushes closer to 1 year unless cranial index exceeds 0.82.
For unilateral coronal, there is no regression, but almost none are perfect at 1 year, so helmet is almost always continued to 1 year.
Jimenez now standardly helmets sagittal synostosis patients for 18 months; Proctor thinks loss between 1 and 2 years is so small that helmet value after 1 year is very small.
For coronal synostosis, bone removal is also about 1 to 2 centimeters, similar to sagittal.