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Surgery for Craniosynostosis Treatment & Management

  • Author: John A Jane, Jr, MD; Chief Editor: Brian H Kopell, MD  more...
 
Updated: Jan 17, 2014
 

Medical Therapy

At some institutions, patients with positional posterior plagiocephaly, when severe, are treated with plastic caps. The caps are fitted externally on the head and can gradually manipulate the shape of the skull. No clear benefit has been identified, and they are not always tolerated well. Most cases are mild forms and do not require treatment.

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Surgical Therapy

Early surgical treatment of craniosynostosis at the end of the 19th century included mostly linear craniectomies and excision of the affected sutures (suturectomies). At the turn of the century, Cushing observed that the complexity of the disorders in patients with craniosynostosis was such that linear craniectomies were hardly addressing the underlying cause.[46] The results of such procedures were unsatisfactory for most types of craniosynostosis, particularly when involving the coronal suture complex. Head shape and exophthalmos did not improve, and further operative treatment was commonly required. For sagittal synostosis, the pi procedure described by Jane and Park is still effective when performed in the first few months of life. In older children presenting late with untreated sagittal synostosis, complex cranial vault reconstruction is performed if the severity of the deformity merits treatment.

For other forms of craniosynostosis involving coronal or metopic sutures, linear craniectomies have been abandoned in favor of more complex cranial expansion and remodeling.[47, 48] The techniques of fronto-orbital advancement were pioneered in Paris in the late 1960s by Tessier[11] and later modified by Marchac and Renier.[49] In earlier years, the tendency was for monobloc facial advancement, including the forehead and midface in one osseous block. Monobloc facial advancement procedures have now waned in popularity because they constitute extensive surgery with considerable morbidity and less than superior results.

Most cranial expansion procedures performed in children with craniosynostosis constitute variations of fronto-orbital advancement. The general principle of these procedures is the independent mobilization of the supraorbital bar with a series of facial osteotomies in the appropriate sites of the medial, superior, and lateral orbital walls and the frontal bone. Subsequent advancement and stabilization of the supraorbital bar in a new, more anterior position results in expansion of the floor of the anterior fossa and the roof of the orbits. A new forehead is reconstructed with frontal bone flaps designed appropriately. With this technique, the connection of the coronal suture complex with the skull base is disrupted. This standard technique is used for most types of bilateral coronal synostosis, both isolated and syndromic.

Several variations of this technique have been described, differing mainly on the alternative possibilities of fixation of the lateral ends of the supraorbital bar on the adjacent temporal or zygomatic bones. The 2 most frequently used techniques are the "floating forehead" and the "tongue-in-groove" (see the image below).

Plain skull radiograph of 11-month-old child with Plain skull radiograph of 11-month-old child with Apert syndrome. Prominent feature is bilateral coronal synostosis. Prematurely closed coronal sutures are seen as white sclerotic bands. This resulted in shallow anterior fossa and shallow orbits.

The former completely disconnects the supraorbital bar and forehead from the temporal bones, with the goal of allowing complete freedom of growth of the forehead from the skull base. The latter purposely attaches the supraorbital bars to the adjacent temporal bones with internal fixation, with the goal of maintaining synchrony of growth between the realigned forehead and the skull base.

Although positive claims have been made from proponents of both techniques, the clinical results are comparable. With these methods, the patients may undergo postoperative relapse of the hypoplastic periorbital deformity owing to lack of a supportive base for the advanced supraorbital rim. As a technical note, an estimation by the surgeon is required to plan for an overcorrection that then allows proper positioning of the supraorbital rim as an adult.

More recently, Patel et al describe the “tilt” procedure to correct supraorbital rim deformity in patients with nonsyndromic metopic, unilateral coronal, or bilateral coronal synostosis.[50] The technique builds on the canthal advancement method by using a modified resorbable plate. After the orbital bar is detached superiorly and laterally, it is tilted forward, maintaining its inferior and medial blood supply. The posterior aspect of the advanced rim is held in place by the plate, while the posterior aspect of the plate, wedged in the squamous temporal bone, is held in place owing to its U shape. This may prevent recurrent hypoplasia while allowing for future growth; objective long-term data are being collected.[50]

For different types of craniosynostosis, appropriate modifications are made to the main technique of fronto-orbital advancement. In those with metopic synostosis, the ridge of the prematurely fused suture is excised and the forehead is reconstructed with suitably designed frontal flaps and supraorbital bars. In those with unilateral coronal synostosis, earlier views favored unilateral frontal advancement. Although only one coronal suture is prematurely fused, in fact, the deformity is bilateral because the normal side is attempting to compensate; bilateral forehead correction is usually necessary.

All variations of fronto-orbital advancement usually achieve satisfactory cosmetic results, with good forehead appearance and satisfactory cover of the orbits (see the image below). A problem often encountered and difficult to correct is persistent narrowing in the temporal regions, observed after any type of fronto-orbital advancement. In addition, abnormalities related to abnormal skull growth, such as turricephaly, brachycephaly, low-set ears, and orbital dystopia, usually persist after successful surgery.

CT (bone windows) images of 11-month-old patient w CT (bone windows) images of 11-month-old patient with Apert syndrome. Bilateral coronal synostosis has resulted in shallow anterior fossa, brachycephaly, and increased biparietal distance. Orbits are shallow. Ventricular system has normal configuration (same patient).

Although fronto-orbital advancement provides satisfactory correction of exorbitism and cosmetic improvement of the forehead appearance, the principle of fronto-orbital advancement does not seem to have any scientific basis. Many studies have indicated that this surgical technique is overcorrecting head volume, producing supranormal head volumes. This explains observations of a high incidence of frontal extradural collections at the site of the advancement after a successful surgery. Similarly, following successful correction of unilateral coronal synostosis, the previously compressed brain does not reexpand; instead, cerebrospinal fluid (CSF) occupies the newly created space. In the absence of another, better alternative, fronto-orbital advancement continues to prevail. It appears to have a cosmetic nature in most cases, excluding children with threatening exorbitism.

In a small, selected group of patients, despite classic syndromic appearances, the predominant problem found after careful appraisal of radiologic images is constriction of the posterior aspect of the skull. Recent attention to the posterior skull has demonstrated that posterior skull release can produce satisfactory results, often obviating the need for frontal advancement.

Syndromic patients with midface hypoplasia may require midface advancement later in life, after age 5 years. Midface advancement can be achieved with either a Le Fort III osteotomy and advancement in one operation or midface distraction osteogenesis.

The latter is gaining popularity because, although it confines the patient to wearing an external frame for several weeks, it appears to achieve a better, long-lasting result. In the last 5 years, midface distraction has been developed and refined and is regarded as the technique of choice for many patients. Meazzini et al recently evaluated patients with craniofacial synostosis who underwent distraction osteogenesis with Le Forte III and rigid external distraction (RED).[51] They found that although advancement of the midface was significant, the lack of maxillary-zygomatic sutural growth, coupled with the physiological remodeling process, caused re-expression of exorbitism in the long-term.

Internal distraction devices have also been used in concert with anterior cranial vault remodeling. The internal distractors are used after performing a Le Forte III in accordance with midface growth vectors.[52]

Wiberg et al recently described posterior distraction osteotomies to treat syndromic craniosynostosis. There was an improvement in turricephaly and intracranial pressure (ICP) postoperatively, with possible benefits of reduced tonsillar herniation.[53]

Patients with hindbrain herniation are commonly asymptomatic and do not require surgery, but a small minority may require surgical management if they develop symptoms or syringomyelia (cavitation and CSF collection inside the spinal cord).

Whenever the issue of possible intracranial hypertension arises, ICP monitoring is usually performed over 24 hours using an invasive bolt. Using an invasive bolt requires a small operation. In some institutions, routine measurement of ICP is performed in all syndromic cases, although this is not a universal policy.

Spring-assisted surgery has gained popularity. It originated in 1997 in Sweden with Dr. Lauritzen.[54] Dr. Lauritzen’s group in Gothenburg preferentially uses spring cranioplasty in children up to age 6 months and the modified pi-plasty in older children.[55] Its initial use has focused on single-suture craniosynostosis, particularly sagittal synostosis. MacFarlane uses the spring cranioplasty for children younger than 9 months who have sagittal synostosis with minimal frontal bossing. Their springs are custom made in Christchurch, New Zealand and have a distraction force of 12-14 N. Spring cranioplasty was compared with strip craniectomy with bioabsorbable cross-struts and was found to decrease the operative time from 122 minutes to 66 minutes.[55]

Massimi and Di Rocco recently described a minimally invasive technique to reduce the visibility of the surgical scar and reduce perioperative morbidity. Instead of a bicoronal incision, the team uses 2-6 incisions to gain access to the sagittal, coronal, and lambdoid sutures. The endoscope is used for the procedure and patients selected for the technique are younger than 8 months, owing to the less malleable skull in children older than 1 year. The sagittal suture is removed first, followed by the coronal and lambdoid sutures. Barrel-stave osteotomies may also be performed. Two limitations of the technique are the limited ability to correct severe frontal bossing and performing linear craniectomies without direct visualization.[56]

Jimenez and Barone recommend the endoscopic-assisted technique for unilateral coronal synostosis.[57] The condition leads to vertical dystopia and midsagittal plane deviation. Using the endoscope, the suture can be removed and approximately half of the patients treated with this method have full resolution of their vertical dystopia and another two thirds of the remaining patients have 80% correction. Advantages of the endoscopic method include decreased blood loss with minimal morbidity and mortality.[58]

General principles of surgical repair

Metopic synostosis

Metopic synostosis is characterized by trigonocephaly. The forehead appears ridged, and the patient has hypotelorism and proptosis. This condition is repaired by advancing the orbital rims, which are noted to be recessed, in addition to removing the fused metopic suture. The forehead requires careful reconstruction. Some institutions perform an endoscopic strip suturectomy through a small incision and then helmet the child to reshape the head as the child grows.

Sagittal synostosis

In sagittal synostosis, the skull is long and narrow. Correction requires reconstruction of the skull so that it is shorter and wider. One factor that must be taken into account during preoperative planning and repair is compensatory growth, which can be anterior, posterior, or both. Frontal bossing can be quite significant, particularly when compensatory growth is anterior. Surgical goals are to shorten and widen the skull. Additionally, a bifrontal craniotomy is required to correct the frontal bossing. In similar fashion, if the compensatory growth involves the occipital bone, then an occipital craniotomy is required. If compensation involves both the frontal and occipital bones, then surgery often needs to be performed in a modified prone position and should include both a bifrontal and occipital craniotomy.

Unilateral coronal synostosis

The unilateral coronal synostosis produces a forehead that is typically bossed on one side and recessed on the other. In this case, a bifrontal craniotomy is required with reconstruction of the frontal bone. In particular, the bossed area needs to be recessed and reduced. An orbital rim advancement is also required.

Bilateral coronal synostosis

The bilateral coronal synostosis produces a skull that is excessively tall and short. The surgery to correct this should produce a skull that is longer in the anterior-posterior dimension and shorter in the superior-inferior dimension. As with the unilateral coronal synostosis case, an orbital rim advancement is required.

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Preoperative Details

Surgery to correct craniosynostosis is known to result in extensive blood loss. Bleeding is the main cause of mortality after surgery to correct craniosynostosis. Meticulous hemostasis and early transfusion can mitigate the results of blood loss.

Recently, tranexamic acid (TXA) has been described in the literature as an adjuvant for reducing blood loss and transfusion requirements.[59] A double-blind, placebo-controlled trial was performed with TXA during correction of craniosynostosis. Patients were loaded with 50 mg/kg of TXA after induction of anesthesia, before incision, which was followed by a 5-mg/kg/h infusion during surgery. These patients were compared with the placebo and found to have lower perioperative mean blood loss, 65 mL/kg versus 119 mL/kg, and lower perioperative mean blood transfusion 33 mL/kg versus 56 mL/kg.[59]

Another group, in a randomized double-blind study, pretreated patients with erythropoietin (600 U/kg) once a week for 3 weeks leading up to surgery. The volume of packed erythrocytes transfused was reduced by 85% intraoperatively and by 57% throughout the study period.[60] Other studies in children undergoing cardiac surgery or spinal surgery for scoliosis have found similar benefits.

The mechanism by which TXA works is not well understood; moreover, the studies have been criticized for the heterogenous populations and attenuated power of the studies.[61] Research is ongoing in this area, in particular with regard to TXA dosing.

Controlled hypotension to reduce blood loss during fronto-orbital advancement was studied by in 2012. Mean arterial pressure (MAP) and calculated blood loss were evaluated. An inverse relationship between MAP and calculated blood loss was discovered; however, on further evaluation, it was found that blood loss was the cause of changes in MAP.[62]

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Intraoperative Details

Sagittal craniosynostosis repair

A preoperative photograph can be seen below. Permanent marker is used to outline the areas of frontal bossing and to delineate the planned incision line. The incision line is planned in a curved manner to allow a more cosmetically pleasing result as the child ages.

Sagittal craniosynostosis (scaphocephaly). Sagittal craniosynostosis (scaphocephaly).

As the image below illustrates, the bicoronal incision is made and Raney clips are placed on both sides of the wound to maintain meticulous hemostasis. One of the more significant risks to an infant undergoing cranial vault reconstruction is blood loss. The surgical team and anesthesiology team must work together to reduce risks and treat blood loss quickly to avoid complications.

Bicoronal incision with placement of Raney clips. Bicoronal incision with placement of Raney clips.

The image below depicts full exposure of the skull with scalp flaps retracted. Note the periorbita anteriorly.

Full view of skull from patient's right. Full view of skull from patient's right.

Permanent marker is used to outline the areas of frontal bossing as seen by the circles on the anterior portion of the skull in the image below. The margins of the planned cuts to remove the sutures with the craniotome are also drawn with marker.

Intraoperative planning. Intraoperative planning.

The anterior fontanelle must be teased away from the surrounding skull before the craniotome can be used to create the cuts that will remove the sutures of interest. Care must be taken not to disturb the superior sagittal sinus.

Separation of anterior fontanelle from surrounding Separation of anterior fontanelle from surrounding skull.

Using the craniotome, the cuts to remove the suture are made, as seen in the image below. The sutures are then easily removed and placed aside to use as part of the final construct.

Removing sutures with craniotome. Removing sutures with craniotome.

After the sutures are removed, the skull is assessed to determine how best to use the sutures in the final construct, as seen in the image below.

After suture removal. After suture removal.

The area of frontal bossing is removed with the craniotome and a high-speed saw. The bone is then reshaped to remove the bossing effect for a more cosmetic result, as seen in the image below.

Reshaping bossed frontal bone. Reshaping bossed frontal bone.

The frontal bone demonstrating bossing has been removed, split, and reshaped, and it is being evaluated for best orientation, as seen in the image below. The frontal bone is placed for best cosmetic result independent of the original orientation.

Evaluating placement of reshaped frontal bones. Evaluating placement of reshaped frontal bones.

The final configuration is seen in the image below. The frontal bones have been reshaped and reoriented for best cosmetic result. The sagittal suture and coronal sutures have been removed and cut into smaller pieces. They are reconfigured into the final construct, attaching them to the skull with absorbable plates and sutures.

Final configuration secured in place. Final configuration secured in place.

Final result after closure can be seen below. Note the reduced anterior-posterior length. In addition, the frontal bossing noted in the preoperative photo is absent.

Final result after closure. Final result after closure.

Unilateral coronal craniosynostosis repair

In the image below, notice the bossed right forehead (child's right) and the recessed left forehead.

Left unilateral craniosynostosis. Left unilateral craniosynostosis.

Reflecting the scalp, one can appreciate the recessed left forehead and a bossed right forehead with much more clarity, as seen below. The goal of correction is to bring the left forehead forward and the right forehead back slightly, in relation to each other, so that as the child grows, the forehead will be even when viewed from above. The superior orbital rim will be advanced on the left as well, with use of some autologous bone from the suturectomies.

Left unilateral craniosynostosis. Left unilateral craniosynostosis.

The frontal bones are removed with the craniotome, as seen below.

Removing frontal bones. Removing frontal bones.

The superior orbital rim is removed with a combination of a high-speed saw and osteotomes, as seen below. The periorbita is protected with a malleable retractor. Once the superior orbital rim is removed, it is remodeled.

Removing superior orbital rim. Removing superior orbital rim.

The image below shows a view of the superior orbital rim prior to remodeling.

Superior orbital rim. Superior orbital rim.

The superior orbital rim is remodeled in this case by extending the left side with autologous bone and absorbable plates, as seen below. A high-speed drill is also used to achieve a better cosmetic result.

Remodeling superior orbital rim. Remodeling superior orbital rim.

The superior orbital has been remodeled to correct the recessed left side and bossed right side. It is ready to be reattached with absorbable plates to the surrounding bones, including the frontal bones, which have already been reshaped.

Superior orbital rim remodeled. Superior orbital rim remodeled.

The corrected frontal bones and superior orbital rim have been reattached with absorbable plates. The left frontal region is no longer recessed and the right frontal region is no longer bossed.

Completed correction of left unilateral coronal cr Completed correction of left unilateral coronal craniosynostosis.

With the closure complete, the symmetry of the frontal bones is more obvious. The advancement of the left side and partial recession of the right frontal region can be appreciated.

Closure is complete. Closure is complete.

These 3 images are taken from a sagittal synostosis repair. Note the gyral pattern on the interior surface of the frontal bone and orbital bar. The reconstruction was performed with absorbable plates.

Craniosynostosis. Inner surface of frontal bone de Craniosynostosis. Inner surface of frontal bone demonstrating gyral impressions.
Craniosynostosis frontal bone and orbital bar, pri Craniosynostosis frontal bone and orbital bar, prior to plating.
Craniosynostosis repaired with plates; note gyral Craniosynostosis repaired with plates; note gyral pattern on interior face of frontal bone. Orbital bar is attached to frontal bone with absorbable plates.
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Postoperative Details

Postoperative imaging is used to demonstrate the new arrangement of the bony architecture of the cranial and facial skeleton. Radiographs obtained early in the postoperative period can be used as references for further assessment. The rate of reossification can be assessed in the regions where dura was left uncovered by calvaria as a result of the advancement.

The evolution of radiological appearances can be particularly useful if the issue of recurrent deformity arises. Although some loss of the advancement is expected in the first few years after operation, other features are also seen in cases of recurrent craniostenosis, such as a localized or generalized copper-beaten appearance and sclerotic hyperdense bands of bone in the calvaria, representing recurrent synostosis.

Recently, the issue of the migration of fixation screws, plates, and wires has gained increasing attention. As the child grows, the skull bones continuously remodel according to natural forces. As a result, the screws used to stabilize the mobilized segment end up buried in the skull or even in the intracranial cavity. Postoperative CT scan images are particularly helpful for monitoring this situation. Although the clinical significance is not known, identification and follow-up of the problem provides an opportunity for appropriate action to be taken. The recent development of bioabsorbable plates and screws may eradicate the problem of migration.

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Follow-up

Typically, each patient has early postoperative plain radiographs, a CT scan in the first few months after operation, and, in syndromic cases, MRI scans at yearly intervals to evaluate for the development of a hindbrain hernia. In patients with maxillary hypoplasia, yearly plain radiographs may be needed to help assess the progress of the deformity when the issue of possible midface advancement is considered.

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Complications

Complications are rare after craniofacial surgery. Hypovolemic shock can occur if significant intraoperative blood loss has not been replaced in a timely manner. Blood loss during surgery has been shown to increase with longer operative times, particularly in excess of 5 hours. Additionally, recognized craniofacial syndromes and pansynostosis have also been associated with increased blood loss during surgery.[63]

Intraoperative dural tears that remain unrecognized can cause postoperative cerebrospinal fluid leaks and resultant infection or subgaleal fluid collections. Epidural or subdural hematoma can occur because of surgical trauma.

Almost all patients develop facial swelling postoperatively, more prominently around the eyes, which rarely causes problems; however, parents and caregivers should be counseled appropriately. Wound infections are generally rare, even after midface procedures, which involve operating in the oral cavity. The frequency rate of these complications is less than 10%.

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Outcome and Prognosis

Most patients respond very well to surgical treatment of craniosynostosis.[64] The improvement in head shape is appreciated almost immediately after the operation. After the first few weeks, the facial swelling and orbital bruising settles, and most parents are pleased with the cosmetic result.[65] In a minority of patients, the deformity recurs after a few years and requires reoperation.

Children with syndromic deformities require careful follow-up into adulthood because they may develop recurrent stenosis and/or raised intracranial pressure from other causes (eg, hydrocephalus, hindbrain hernia). Papilledema, if present initially, may herald a recurrence if seen on follow-up.

Children with syndromic forms of craniosynostosis (eg, Crouzon or Apert syndrome) can be affected by a variable degree of developmental delay. Long-term care and surveillance by an integrated neurosurgical and craniofacial multidisciplinary team is essential.

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Future and Controversies

With increasing awareness by pediatricians and family doctors, craniosynostosis is usually diagnosed in the first few months of life. Sagittal synostosis is effectively treated with strip craniectomy when performed in the first 3 months of life. For craniosynostosis affecting the coronal sutures, considerable debate still exists regarding the timing of cranial expansion surgery. Some physicians prefer to perform surgery soon after birth, while others defer surgery until the child is aged 12 months.

Proponents of early surgery believe that cranial expansion at an early age is taking advantage of the effect the growing brain applies to the skull, while minimizing the risk of mental impairment due to restricted brain growth by relieving intracranial hypertension early.

Papilledema is the most consistent finding associated with elevated intracranial pressure (ICP). The relationship between increased ICP and craniofacial abnormalities is complex and not well-understood; however, a child with such an abnormality is at risk for developing increased ICP. This belief stems from the view that the skull grows in response to the growth of the brain, which is regarded as the main driving force of head growth.

Interestingly, craniosynostosis has been described after shunting. Patients with single-suture craniosynostosis have a 4-14% rate of developing increased ICP, whereas those with multiple-suture synostosis have an incidence of 47-67%. After surgery to repair the craniosynostosis by cranial vault reconstruction, the incidence of increased ICP has been reported at 6-36%.

Most cases of raised ICP present before age 3 years, and rarely after age 6 years.[66] Syndromic cases and those with multiple-suture synostosis who develop increased ICP have been shown to have increased rates of narrowing of the jugular foramina and sigmoid sinus–jugular vein complex, which can result in hypertension of the venous system.[66]

Some authors define elevated ICP as greater than 15 mm Hg, whereas, others count the number of ICP elevations above 20 mm Hg in order to guide surgical intervention. For ICP in the borderline range of 10-15 mm Hg, an elevation above 15 mm Hg for at least 1 minute during slow-wave sleep is diagnostic of increased ICP.[67, 68, 59]

A significant problem associated with early corrective surgery is a higher risk of recurrent deformity. In most series published from centers at which frontal advancement is performed in the first few months of life, a high reoperation rate is reported. This high rate could be explained by the observation that in the first months of life, craniosynostosis is still active; therefore, the effect of cranial expansion is less likely to be retained.

This hypothesis is reflected by the observation that fronto-orbital advancement performed at such an early age needs to include some overcorrection in order to achieve good late results. Often, several operations are required in the first few years of life to achieve satisfactory results. Although it may be socially acceptable among those who seek perfect shape, these procedures constitute major surgery with a small but appreciable risk of morbidity and mortality.

The published evidence in favor of early surgery is biased by preferences of the reporting authors, and no clear data exist comparing early versus late surgery. Some indirect evidence in favor of early surgery has been reported based on measurements of ICP and intelligence quotient. These reports are based on retrospective observations, use crude measures of outcome, and have not conclusively analyzed the effect of the different variables involved in psychomotor development.

Proponents of late surgery believe that delaying surgery until most of the skull growth has been completed reduces the risk for recurrent deformity. This view is supported by an observed lower reoperation rate in children and infants who were initially treated when older than 9 months. Critics of delayed surgical treatment point out that the final results of such policies are often not as attractive cosmetically. In addition, the delay in surgical decompression of the brain may impair the mental and psychological outcome of these children. This may be particularly important because a high proportion of patients with syndromic forms are likely to have intracranial hypertension.

Although significant indirect evidence indicates that late surgery (occurring in children >12 mo) in all types of craniosynostosis, even scaphocephaly, is associated with poor psychomotor development, the issue is complicated by many interrelated parameters. In attempts to define the optimal surgical time, a few studies have reported measurements of skull volumes in children with craniosynostosis. Some studies concluded that intracranial volume in children with craniosynostosis is within normal limits and that children with Apert syndrome tend to have higher than normal intracranial volumes.

Studies have attempted to correlate skull volume in craniosynostosis with ICP. No correlation has been identified between ICP and intracranial volume. Despite a normal head volume, intracranial hypertension may exist. A recent study that analyzed the change of intracranial volume with age showed that patients with craniosynostosis start their life with an intracranial volume smaller than their healthy peers. By the time the infants are aged 6-9 months, their intracranial volume has reached normal levels and continues to grow normally. If any restriction of brain growth by slow skull growth occurs, it is only operative in the first 6 months of life.

The maximum constrictive effect of craniosynostosis appears to occur at birth when the difference in intracranial volume between healthy neonates and neonates with craniosynostosis is maximal. After birth, the constrictive effect gradually declines during the first few months and stops in infants aged 6-9 months. Therefore, this would appear to be the optimal time for definitive corrective surgery because any alteration made on the cranial skeleton is likely to remain. Such a policy would not apply to patients with overriding reasons dictating early operation, such as severe intracranial hypertension or exorbitism.

A major factor implicated in the timing of surgery for craniosynostosis is the development of intracranial hypertension. Papilledema is rarely seen in children with craniosynostosis, even in the presence of intracranial hypertension. Among patients with verified intracranial hypertension, only a small proportion (16-25%) have papilledema.[69] If papilledema is present, however, it is 100% sensitive for increased ICP in patients older than 8 years and is only 22% sensitive in those younger than 8 years.[70] On the other hand, a late presentation of craniosynostosis may lead to irrecoverable optic atrophy and visual failure due to sustained intracranial hypertension.[71] The presence of a copper-beaten appearance of the skull on the plain skull radiograph suggests increased ICP, but this does not correlate well with the level of ICP.[72]

Interesting work by Schaller et al evaluated patients preoperatively with single-photon emission computed tomography (SPECT) studies and found regional asymmetry of cerebral perfusion. The patients ranged from those with scaphocephaly (50%) to those with syndromic forms of craniosynostosis (9%). The asymmetry correlated to hypoperfusion in the area underlying the fused suture. The same patients scanned 3 months postoperatively with SPECT, demonstrated improved perfusion defects and normalization of cerebral perfusion. Performing surgery within the first 6-8 months may give the best opportunity for decreasing cognitive sequelae.[73]

Recent work has indicated that the tympanic membrane could be used for ICP measurement.[74] The current techniques of measuring ICP are invasive, using subdural or intracerebral transducers and having a small but appreciable complication rate.

A significant proportion of patients with craniosynostosis (25-35%) are likely to have intracranial hypertension. The incidence of intracranial hypertension varies among different types of craniosynostosis and is higher in syndromic forms. As many as 60% of patients with Crouzon syndrome and 45% of patients with Apert syndrome have intracranial hypertension. Percentages vary for other forms of craniosynostosis. Of patients with nonsyndromic coronal synostosis, 30% have intracranial hypertension. Of patients with sagittal synostosis, 18% have intracranial hypertension. Of patients with unilateral coronal synostosis, 12% have intracranial hypertension.

ICP has been found at higher rates in children presenting with craniosynostosis when older than 12 months. This may imply a rise in ICP with age in patients with craniosynostosis. Children with late-presenting craniosynostosis constitute a rare and poorly studied group in which different considerations may apply with respect to head growth. In addition, intracranial hypertension has been reported to be able to persist despite cranial expansion surgery. This raises the question of whether poor psychomotor development and the high incidence of intracranial hypertension seen in patients with syndromic craniosynostosis could be due to a genetically predetermined tendency for adverse brain development.

The cause and mechanism of intracranial hypertension in children with craniosynostosis is not well understood. Intracranial hypertension may be due to other causes and/or unrelated to skull constriction, such as abnormal venous drainage in the skull base causing venous hypertension, hydrocephalus, and obstruction of the upper airways. The role of venous hypertension has been better appreciated in recent years and new research is being conducted with SPECT studies and underlying areas of cerebral hypoperfusion.

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Contributor Information and Disclosures
Author

John A Jane, Jr, MD Professor of Neurosurgery and Pediatrics, Neurosurgery Residency Program Director, Department of Neurosurgery, University of Virginia School of Medicine; Director of Pediatric Neurosurgery Program, Co-Director of Neuroendocrine Program, Neurosurgeon, University of Virginia Health System

John A Jane, Jr, MD is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, Endocrine Society, North American Skull Base Society, Congress of Neurological Surgeons, Southern Neurosurgical Society

Disclosure: Nothing to disclose.

Coauthor(s)

M Sean McKisic, MD Resident Physician, Department of Neurosurgery, University of Virginia Health System

M Sean McKisic, MD is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Christian Medical and Dental Associations, North American Skull Base Society, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ryszard M Pluta, MD, PhD Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Polish Society of Neurosurgeons, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from St Jude Neuromodulation for consulting; Received consulting fee from MRI Interventions for consulting.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor Spyros Sgouros, MD, FRCS, to the development and writing of this article.

References
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Normal anatomic suture configuration
Metopic craniosynostosis (trigonocephaly).
Sagittal craniosynostosis (scaphocephaly).
Unilateral coronal craniosynostosis (plagiocephaly).
Lambdoid craniosynostosis (posterior plagiocephaly).
Girl aged 11 months with Apert syndrome, with brachycephaly and turricephaly. Low-set ears and flat eyebrows are indicative of deformed skull base. Maxillary hypoplasia is seen clearly, even at this age, and will become more obvious later on in life.
Plain skull radiograph of 11-month-old child with Apert syndrome. Prominent feature is bilateral coronal synostosis. Prematurely closed coronal sutures are seen as white sclerotic bands. This resulted in shallow anterior fossa and shallow orbits.
CT (bone windows) images of 11-month-old patient with Apert syndrome. Bilateral coronal synostosis has resulted in shallow anterior fossa, brachycephaly, and increased biparietal distance. Orbits are shallow. Ventricular system has normal configuration (same patient).
Three-dimensional CT scan of 11-month-old patient with Apert syndrome. 3D visualization of skull anatomy offers more realistic interpretation of pathologic process of craniosynostosis. Anterior fossa is shallow, and prematurely fused coronal suture is seen as prominent ridge in inner surface of skull. Also, posterior fossa is shallow. Facial skeleton and relation of facial skeleton to skull base can also be appreciated.
MRI of 11-month-old patient with Apert syndrome. Abnormal configuration of brain parenchyma is seen. Multiple-suture synostosis has resulted in oxycephaly, with corresponding distortion of corpus callosum and ventricular system. Posterior fossa is shallow, and some hindbrain herniation is present.
Fronto-orbital advancement techniques are indicated in any form of bilateral coronal synostosis. On left, lines indicate osteotomy sites. Fused coronal sutures are removed (shaded area), and free supraorbital bar is created and moved forward to enlarge anterior fossa. Resulting free frontal bone flap is used to reconstruct forehead, often rotated 180°. In middle figure, "floating forehead" technique is shown. Forehead is disconnected from coronal suture system and skull base by extending bone removal in infratemporal fossa. Supraorbital bar is fixed to zygomatic bone. On right, "tongue-in-groove" technique is shown. Supraorbital bar is fixed to adjacent temporal bone in purposefully created groove, the goal of minimizing bone defect under temporalis.
Postoperative photograph of 4-year-old girl with Apert syndrome. She had fronto-orbital advancement when aged 12 months. Orbits are well covered as result of advancement, but ears remain low-set and turricephaly has not changed significantly. Cosmetic appearance is satisfactory.
Sagittal craniosynostosis (scaphocephaly).
Bicoronal incision with placement of Raney clips.
Full view of skull from patient's right.
Intraoperative planning.
Separation of anterior fontanelle from surrounding skull.
Removing sutures with craniotome.
After suture removal.
Reshaping bossed frontal bone.
Evaluating placement of reshaped frontal bones.
Final configuration secured in place.
Final result after closure.
Left unilateral craniosynostosis.
Left unilateral craniosynostosis.
Removing frontal bones.
Removing superior orbital rim.
Superior orbital rim.
Remodeling superior orbital rim.
Superior orbital rim remodeled.
Completed correction of left unilateral coronal craniosynostosis.
Closure is complete.
Craniosynostosis repaired with plates; note gyral pattern on interior face of frontal bone. Orbital bar is attached to frontal bone with absorbable plates.
Craniosynostosis frontal bone and orbital bar, prior to plating.
Craniosynostosis. Inner surface of frontal bone demonstrating gyral impressions.
 
 
 
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