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Open Craniosynostosis Repair
WHY ARE THE SKULLS OF INFANTS SUBJECT TO DEFORMATION?
- The skull's plasticity of the newborn makes it receptive to external pressure in the womb.
- The newborn's immobility and any positional neck preference can make infants susceptible to extrinsic skull deformities.
- Craniosynostosis or genetic transmission can result from intrinsic abnormalities.
- Deformational forces that frequently affect the occiput. Though, in severe cases the frontal bones and face may be affected.
- At birth, about 24% of babies have a noticeable skull deformity, but at 4 months of age it reduces to about 20%.
- If the baby spends most of the day on the back against a hard surface of carriers or other holding devices, then the abdormal shap may persist.
WHAT ARE THE TYPES OF HEAD SHAPES?
Deformational Plagiocephaly is the most common type of infant skull deformity, and is normally noticed at about 6 to 10 weeks of age. It is characterized by an asymmetrical skull shape and has a unilateral occipital flattening. The ear is positioned more anterior on the occipital flattening side. Also, there may be facial asymmetry and the forehead may be asymmetrical with an anterior positioning. Deformational Plagiocephaly could be accompanied by torticollis, limited neck range of motion, weakness and preferential head positioning.
Deformational Brachycephaly has central occipital flattening and a high and sloped skull (the head is excessively wide for its length). This skull deformity could be accompanied by a prominent, bossed forehead.
Deformational Brachycephaly with Asymmetry is a combination of brachycephalic and plagiocephalic characteristics. The shape is disproportionately wide and asymmetrical. Though, there may not be asymmetries to the forehead and facial structure.
Deformational Scaphocephaly is a very elongated head shape that in width is excessively long. This skull deformity is uncommon and is caused by extrinsic forces. It is sometimes seen in premature infants who are often positioned side lying (such as NICU infants). Scaphocephaly caused by extrinsic positioning can be confused with sagittal synostosis (which is a more serious deformity that usually requires surgery).
Craniosynostosis is the premature closure of one or more cranial sutures. Treatment of craniosynostosis usually requires surgical intervention. Craniosynostosis causes restriction of growth at the suture and abnormal growth perpendicular to the fused suture. There are different types of craniosynostosis, which include: sagittal, coronal, metopic and lambdoid. Infants diagnosed with craniosynostosis usually require surgery, and early diagnosis is vital. Endoscopic surgeries are often recommended before 3 months of age.
Sagittal Synostosis is the most common type of single suture craniosynostosis and results in an elongated head shape.
- Sagittal suture is usually ridged.
- Frontal and occipital bones have a pinched/boxy shape.
- The skull becomes progressively more abnormal and does not improve with repositioning.
Lambdoid Synostosis is the least common type of craniosynostosis and can be confused with plagiocephaly.
- Significant occipital asymmetry without anterior ear shift or frontal changes.
- Mastoid bossing on the side of occipital flattening.
- Contralateral parietal bossing.
Other types of synostosis include metopic, uni-coronal, bi-coronal and synostoses that are manifested in syndromal disorders like Apert's and Crouzon's (Cranial Center of Excellence).
Deformational Plagiocephaly can be difficult to differentiate from Lambdoid Craniosynostosis because both conditions present with asymmetry.
- Ipsilateral frontal bossing.
- Ipsilateral anterior ear displacement.
- Occipital flattening with contralateral bossing.
- No ipsilateral frontal bossing.
- Ipsilateral posterior ear displacement (usually).
- Ipsilateral mastoid bossing.
- Significant occipital flattening that worsens over time.
Craniosynotosis Multisutural Open Vault
Endoscopic Sagittal Craniosynotosis
Endoscopic Sagittal Craniosynotosis
Left-sided craniofacial microsomia with combined maxillary and mandibular hypoplasia.
The surgical operation was performed with LeFort-I osteotomy, bilateral sagittal split osteotomy, nasal endoscopy and internal fixation of both osteotomies. VSP Orthognathics (a computerized modeled plan and computerized custom modeling) was used for the staging and creation of the two separate custom oral splints for this procedure.