Update in Management of Craniosynostosis | Journal CME Article-Journal CME Article: Update in Management of Craniosynostosis Video

Journal CME Article: Update in Management of Craniosynostosis Video

Video Transcription

This young lady presents to our hospital at the age of three with Crouzon syndrome and papilledema.  She had been treated at an outside hospital with a ventral peritoneal shunt at a young age. On presentation, her shunt did not appear to be functioning.  Therefore, our nursery team revised her shunt, and we proceeded with a frontal orbital advancement in order to help correct her cranioscerebral disproportion.  The papilledema resolved after the frontal orbital advancement, and we watched her grow uneventfully until the age of eight.  At this point, she started to develop some mild to moderate sleep apnea and lag of thalamus. Because of this, we decided to proceed with a monoblock osteotomy and advancement with  distraction osteogenesis in addition to a facial bi-partition in order to help correct orbital morphology.  We're here proceeding through her previous frontal orbital advancement scar and dissecting the scalp all the way down to the frontal bar.  Here we're performing the bi-frontal craniotomy in order to release the frontal bone. Now we're performing the lateral osteotomies, which goes from the infra-oblique fissure  intra-orbitally all the way up into the cranium.  We're performing the superior orbital cuts here, connecting them to the lateral orbital cuts that we previously made.  Now we're dissecting down to the zygomatic arch in order to cut the arch. Realize you do not have to dissect the whole temporalis muscle off the arch in order to perform this.  Now we're dissecting medially in order to osteotomize the medial orbital wall and as much of the orbital floor as we can.  An intraoral incision is performed in order to expose the pterygomaxillary region in order to perform our pterygomaxillary osteotomy and disjunction.  Realize this pterygomaxillary disjunction needs to be taken as superior as possible. Transconjunctival incision is performed in order to expose the infra-orbital rim on both  the right and the left orbits. Then the orbital floor osteotomies are performed with care to osteotomize the posterior maxillary  wall in order to help release the face from the cranial base. Same procedure is performed on the left side.  Realize that you are trying to connect all the orbital cuts from superior to lateral to medial so that you have a complete orbital disjunction.  Then our final osteotomy disjunction from the skull base is performed and osteotome is taken just posterior to the frontal orbital bar, just anterior to the cribriform plate  and we're aiming this to the posterior maxillary spine. Now that we have performed this, the disjunction is performed and the face should be freely mobile.  Please note that if it's not mobile, you need to repeat your osteotomies in order to make sure that your distraction is successful.  Via the intraoral approach, we are now splitting the palate in order to assist with our partition.  Our palatal split is right down the middle in between the central incisors and then we're taking a portion of bone out in the middle approximately 1 to 15 millimeters in order  to help achieve orbital rotation and correct the lateral slanting palpebral fissures. Here's our portion of bone removed from the central bit which will include nasal bones.  Therefore these nasal bones will be removed and replaced and replated so that we restore the nasal frontal area to its normal morphology.  Here's a plate spanning our bi-partition and holding our orbits into a more rotated position. Here's our frontal bone placed back.  Here our patient is with her distraction device on nicely and then here she is pre- and post-operatively  after her removal of her frame with good mid-face projection, improvement of the lateral slanting palpebral fissures, improvement in her airway, and no lag of thalamus.  The post-op pictures were taken during COVID so they had to be done via telemedicine. Thank you for your attention.

Video Summary

A three-year-old with Crouzon syndrome and papilledema was treated at our hospital after an initial non-functioning shunt. Her condition improved following a frontal orbital advancement. At age eight, she developed sleep apnea and lag of thalamus, prompting a monoblock osteotomy and facial bi-partition to correct orbital morphology. Surgical procedures included bi-frontal craniotomy, orbital and pterygomaxillary osteotomies, and palate splitting. Post-surgery, the patient showed improved mid-face projection, airway function, and orbital shape. The surgery's success was evident in telemedicine follow-ups during COVID.