Encephalocele
Description of an Encephalocele
An Encephalocele, which may be found anywhere in the craniofacial region, forms when the brain tissue pushes through a bony defect of the skull. This skull defect can be caused by abnormal skull growth and may be part of various syndromes. An Encephalocele usually occurs in the fronto-orbital region or as a herniation located between the orbits, but it also occurs on the top of the head or skull at any location or in the base of the skull. When it presents in the intraoral cavity, it causes clefting of the palate.
An Encephalocele most frequently occurs in the posterior skull, but it may be found in the fronto-orbital region or as a herniation located between the orbits. This skull defect can be caused by abnormal skull growth and may be part of various syndromes. When it presents in the interoral cavity, it causes clefting of the palate.
Expectations and Treatment
In some situation, this deformity is surgically corrected upon diagnosis to prevent infections. However, the ideal time is between age 1 and 2 years, or from 1 to 5 for definitive correction, depending on the deformity.
Case Studies
Frontonasal Encephalocele
Preoperative frontal view of a 2-year-old patient with frontonasal encephalocele. Postoperative frontal view 1 year after surgical correction.
Posterior Encephalocele with Cranial Vault Deformity
Preoperatuve lateral view of a 2-year-old patient with a marked cranial-vault abnormality due to a posterior encephalocele. Postoperative right lateral view 2 weeks after surgical correction. Three-dimensional CT shows the cylindrical cranial-vault distortion by the posterior encephalocele. Postoperative lateral roentgenogram. Split cranial bone was reshaped and recontoured for reconstruction.For a Child with an Encephalocele
Having a child with an encephalocele, while cosmetically unacceptable, doesn’t have to be a tragedy. An encephalocele is a birth defect where a portion of the brain pushes between a defect in the skull and bulges through the defect. An encephalocele often occurs in the area of the forehead, between the eyes or in the back of the head. Encephaloceles generally push through suture lines in the skull. Encephaloceles are congenital and the defect is usually present at birth. If your child has an encephalocele, you may notice it at birth or perhaps several weeks later. If an encephalocele occurs without a normal covering of skin, it must be repaired immediately, before infection around the brain occurs.
Encephalocele surgery may need to be done at birth or up to 1-2 years of age. Because the child is growing, encephalocele repair may need to be done more than once, until the suture lines begin to fuse. Children with an encephalocele may develop a hydrocephalus or extra fluid around the brain.
Treating an Encephalocele
An encephalocele, if improperly treated, can cause meningitis or infection around the brain. If it’s covered with skin, the encephalocele may not need immediate surgical repair. If the encephalocele is at risk for hydrocephalus or infection, urgent encephalocele repair is necessary. Your doctors will tell you when the proper time will be for repair of the encephalocele.
Your doctors will consult with you prior to encephalocele surgery. The risks and benefits of encephalocele repair will be explained to you. One of our doctors will examine the encephalocele to see if the encephalocele repair needs to be done immediately or if it can be delayed. When the time comes for encephalocele repair, your child’s appearance will be much improved over how he or she looked prior to encephalocele surgery.
