Surgical Treatment for Craniosynostosis

Surgical management for craniosynostosis is based on the suture(s) involved, the age of the child, and the individual needs for each patient.

Options for treatment that are most commonly recommended include:

Strip craniectomy

The common treatment approach at Children’s Hospital of Philadelphia (CHOP) includes a formal cranial vault expansion and reshaping procedure, but a strip craniectomy can be used as a preliminary procedure to reduce pressure in very young children (typically less than 6 months of age) with multiple sutures involved.

A strip craniectomy is typically performed in conjunction with a pediatric neurosurgeon. The procedure generally takes approximately two to three hours. After surgery, your child will stay at the Hospital for recovery and follow-up care. Most children stay for an average of three to five days.

Additional surgeries including vault expansion and reshaping procedures will be scheduled according to your child’s recovery and how well she responds to this preliminary procedure.

Sagittal springs

The use of sagittal springs with strip craniectomy may be recommended for sagittal synostosis if the child is younger than 5 months at the time of initial surgery. The surgery involves a strip craniectomy and placement of two to three stainless steel springs to help increase the amount of room for the brain to grow, improve the skull shape, and reduce the risk of the sagittal suture closing again. Springs are an effective “minimally invasive” surgery that can be done through small incisions in the scalp, limit blood loss such that only a minority of patients require blood transfusion, and usually only results in a one-day hospital stay. There is an additional surgery approximately three months later to remove the springs.

Patient pre- and post- cranial spring procedure

Pre-operative (top) and post-operative (bottom) comparison of sagittal synostosis corrected in infancy with cranial spring placement. Note improved width to skull shape and more typical roundedness of skull compared to progressive pattern of long and narrow head shape that is characteristic of sagittal synostosis.

Cranial molding helmet therapy may be used before surgery to reduce the progression of head shape changes that occur with sagittal synostosis, and possibly again after surgical spring removal to redirect skull growth to a more typical pattern.

Procedure demonstration of sagittal spring placement

Procedure demonstration of sagittal spring placement Demonstration of the bony cuts of the craniectomy and placement of sagittal springs. This procedure is used for correction of sagittal suture synostosis in early infancy.
From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Fronto-orbital advancement /reshaping

Fronto-orbital advancement may be used in the correction of metopic, coronal, or multi-suture craniosynostosis. It involves exposure of the upper eye socket and forehead through an ear-to-ear incision. The forehead bone is then removed with the assistance of the neurosurgeon. The misshapen upper eye socket is then cut free, reshaped, and replaced in a new position, held in place with resorbable plates and screws. The forehead is then reshaped and affixed to the upper eye socket.

Patient pre- and post- bilateral fronto-orbital advancement

Images of infant pre-operatively (upper) and post-operatively (lower) who underwent bilateral fronto-orbital advancement for premature closure of both coronal sutures. The bony gap seen in the post-operative CT scan will mostly fill in over time. Of note, this patient ultimately required further surgery to expand the posterior dimension of the skull.

This procedure offers eye protection and creates room for brain growth, and reshapes the upper eye socket and forehead.

For children with eye exposure issues, fronto-orbital advancement is a first-stage procedure. Otherwise it is delayed as long as possible after posterior vault distraction, and may be performed when your child is older depending on the other surgical procedures she undergoes. In some cases, the procedure may be avoided and the condition managed with a monobloc distraction, later in life. 

Patient pre- and post- bilateral fronto-orbital advancement

Example of metopic synostosis before surgery (top row) and more than three years after repair by bilateral fronto-orbital advancement. Note significant narrowing and triangular shape of forehead prior to surgical correction.

Fronto-orbital advancement surgery typically takes about four to five hours, followed by an average hospital stay of four to five days for monitoring and recovery. As your child grows and develops, she may need additional procedures.

Patient pre- and post- bilateral fronto-orbital advancement

Pre-operative (top) and post-operative (bottom) after bilateral fronto-orbital advancement for right unicoronal synostosis. Note improvement in forehead height and symmetry.

Procedure demonstration of fronto-orbital advancements

Demonstration of the bony cuts of a bilateral frontal orbital advancement Demonstration of the bony cuts of a bilateral frontal orbital advancement are shown in red (left). On the right is a demonstration of the fixation of the reshaped frontal bones by resorbable screws and plates. A slurry of cranial bone cells is placed in the large bony gap to help remodel the bony gap. This procedure is used most often for coronal synostosis and when the forehead is of typical shape.
From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Demonstration of the bony cuts of a bilateral frontal orbital advancement and broadenin Demonstration of the bony cuts of a bilateral frontal orbital advancement and broadening are shown in red (left). On the right is a demonstration of the fixation of the reshaped frontal bones by resorbable screws and plates. A slurry of cranial bone cells is placed in the large bony gap to help remodel the bony gap. Used for correction of metopic synostosis, this procedure requires a reshaping of the forehead shape and therefore the extra need for fixation at the center of the forehead.

From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Demonstration of the bony cuts of a unilateral frontal orbital advancement Demonstration of the bony cuts of a unilateral frontal orbital advancement, in this case left, are shown in red (left). On the right is a demonstration of the fixation of the reshaped frontal bones by resorbable screws and plates. A slurry of cranial bone cells is placed in the large bony gap to help remodel the bony gap. Though not used at CHOP, this procedure may be used for unilateral coronal synostosis and when the forehead deformation is not severe and when bilateral reshaping is not required.

From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Posterior vault remodeling and reconstruction

Cranial vault remodeling, also commonly called cranial vault reconstruction, involves the reshaping of the cranial bones in a single stage surgery.  This surgery is often used when more than one cranial suture is closed prematurely.  It involves a coronal (ear-to-ear) incision and then removal of the cranial bone by neurosurgery in the area that has been restricted in growth. This can be done regionally in the skull (e.g., posterior skull remodeling, middle vault expansion, or anterior cranial vault remodeling), and it can be done to the whole vault.

Patient pre- and post- posterior cranial vault remodeling and reconstruction

Pre-operative photos of infant with sagittal synostosis (upper) and after posterior vault reconstruction (lower). Note improvement in the skull shape.

After the bone is removed, it is reshaped and often expanded. The reshaped skull is bolstered with a cranial bone graft and secured with resorbable plates or sutures to provide more room for the brain to grow and for the head shape to be more typical. This surgery requires admission to the hospital for several days. A post-operative surgical drain is in place and is typically removed prior to discharge. Importantly, after cranial vault remodeling procedures, children do not require cranial molding helmets and can participate in sports when older.

Procedure demonstration of cranial vault reshaping and remodeling

Demonstration of the bony cuts of a posterior vault remodeling Demonstration of the bony cuts of a posterior vault remodeling (left). On the right is a demonstration of the fixation of the reshaped posterior vault by resorbable screws, plates and sutures. In this example, release and remodeling were for the treatment of premature closure of the lambdoid suture.
From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Demonstration of the bony cuts of a posterior vault remodeling Demonstration of the bony cuts of a posterior vault reshaping in a child with sagittal synostosis (left). On the right is a demonstration of the fixation of the reshaped posterior vault by resorbable screws, plates and sutures. Bilateral fronto-orbital advancement/reshaping may be required in a separate surgery if the front of the skull is affected.
From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Demonstration of the bony cuts of a total cranial vault reshaping Demonstration of the bony cuts of a total cranial vault reshaping (left). On the right is a demonstration of the direction of cranial vault expansion and shortening. Shown in this example is a patient with sagittal craniosynostosis, but variation of this technique may be applied to various other forms of synostosis.

From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Posterior vault distraction

Posterior cranial vault distraction is a surgical procedure that expands the back of the skull, by gradually stretching the bone and skin to expand the intracranial space and create new bone. CHOP has been a leader in instituting this procedure that has helped countless children.

Patient pre- and post- posterior vault distraction

Pre-operative photos of infant with coronal synostosis (upper) and while in consolidation (period of bone hardening) from posterior vault distraction (lower). Note improvement in the skull dimension from front to back. The distractors were uneventfully removed several weeks after these photos.

The distraction technique has often replaced the traditional fronto-orbital advancement procedure as the most common initial treatment used to expand the skull in patients with syndromic craniosynostosis. The posterior vault distraction offers several advantages. Distraction expands the soft tissue in addition to the bone, allowing for significantly greater expansion of your child’s skull. This is especially useful if your child has elevated intracranial pressure or a very abnormal head shape.

In the posterior cranial vault distraction procedure, a coronal (ear-to-ear) incision is made, the posterior skull is exposed, and cuts are made around the bone to be expanded. Metallic distractors are then placed along your child’s bone and the incision is closed. The distractors work to slowly stretch and expand the bone and surrounding tissues.

Starting three to five days after the procedure, your child’s surgeon will begin turning the distractors. Over the next two to three weeks, gradual expansion of the bone and soft tissue occurs.

The operation takes approximately two and three hours. You can expect your child to remain in the hospital post-surgery for an average of two to three days. The posterior cranial vault distraction is less invasive than a formal open vault expansion, and allows for more significant expansion of the bone and soft tissue.

A second procedure to remove the distractors is required after approximately three months.

Procedure demonstration of posterior vault distraction

Demonstration of the bony cuts and placement of posterior vault distractors Demonstration of the bony cuts and placement of posterior vault distractors. The green arrow represents the advancement that occurs with this procedure. This procedure is used for correction of syndromic craniosynostosis and gives improved shape and greater volume of expansion.

From AO Surgery Reference (www.aosurgery.org). Copyright by AO Foundation, Switzerland. Reprinted with permission.

Final facial contouring

When your child is done growing and all major osteotomies are complete, final facial contouring is performed to enhance the visual appearance and correct any remaining irregularities of the facial skeleton. Final contouring procedures include smoothing irregularities, reduction, adding bone grafts or bone substitutes, and re-suspending soft tissues.

Patient pre- and post- final facial contouring

Patient with repair of metopic synostosis in infancy with residual contour deficiencies (top) and after final contouring (bottom). Note improved appearance of fullness at both temples and of the forehead.

Images of Surgical Treatment of Craniosynostosis

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