Outcome and Prognosis
Most patients respond very well to surgical treatment of craniosynostosis.17 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 happy with the cosmetic result.18
In a small 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 ICP from other causes (eg, hydrocephalus, hindbrain hernia). They may need surgery on their midface.
Children with syndromic forms of craniosynostosis (eg, Crouzon or Apert syndrome) can be affected by a variable degree of developmental delay, which becomes apparent later on in life.
Future and Controversies
With the increasing awareness of 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. Views vary widely. 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 push that the growing brain applies to the skull, while minimizing the risk of mental impairment due to restricted brain growth by relieving intracranial hypertension early. 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.
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 this 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.
All the published evidence in favor of early surgery is colored by prejudice and preference of the reporting authors, and no comparative study of early versus late surgery has ever been conducted. 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 very crude measures of outcome, and have not conclusively analyzed the effect of the different variables involved in psychomotor development; therefore, they cannot be regarded as definitive evidence.
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 and no conclusive evidence has been produced. Conversely, in patients with sagittal synostosis, a 30% incidence of speech and language developmental delay has been observed, which cannot be explained on the basis of sustained intracranial hypertension because the condition is associated with a low incidence of intracranial hypertension and all children undergo the operation early in life.
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.
Two studies have attempted to correlate skull volume in craniosynostosis with ICP. No correlation has been identified between ICP and intracranial volume. Intracranial hypertension can exist in the presence of normal head volume. 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. After the infant is older than 6 months, the effect of craniosynostosis becomes exhausted or burnt out.
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. No reliable indirect indicators exist for 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. On the other hand, a late presentation of craniosynostosis can lead to irrecoverable optic atrophy and visual failure due to sustained intracranial hypertension. The presence of a copper-beaten appearance of the skull on the plain skull radiograph does not correlate well with the level of ICP.
No satisfactory noninvasive technique exists for measuring ICP. Previous studies have demonstrated the relative value of transcranial Doppler. The practicalities of performing such an examination are so complex that they prevent the routine use of Doppler in very young patients.
Recent work has indicated that the tympanic membrane could be used for ICP measurement. 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.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor, Spyros Sgouros, MD, FRCS, and M Sean McKisic, MD, to the development and writing of this article.
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Further Reading
Clinical guideline
Prevention and management of positional skull deformities in infants.
American Academy of Pediatrics - Medical Specialty Society. 2003 Jul. 4 pages. NGC:003254
Clinical trials
Endoscopic Treatment for Isolated, Single Suture Craniosynostosis
Osteogenic Profiling of Tissue From Children With Craniosynostosis
Clinical Study of Muenke Syndrome (FGFR3-Related Craniosynostosis)
Tranexamic Acid for Craniofacial Surgery
Related eMedicine topics
Apert Syndrome
Congenital, Synostoses
Craniosynostosis Management
Craniosynostosis (Neurology)
Craniosynostosis (Radiology)
Crouzon Syndrome
Keywords
craniosynostosis, craniostenosis, craniofacial dysostosis, craniofacial defect, craniofacial surgery, cranial sutures, cosmetic facial deformity, cranial deformity, cranial malformation, intracranial hypertension, abnormal skull shape, abnormal head shape, skull growth, Crouzon syndrome, Apert syndrome, Crouzon's syndrome, Apert's syndrome, scaphocephaly, sagittal synostosis, plagiocephaly, unilateral coronal synostosis, posterior plagiocephaly, lambdoid synostosis, trigonocephaly, metopic synostosis, brachycephaly, oxycephaly, turricephaly, cloverleaf skull deformity, triphyllocephaly, pansynostosis, Saethre-Chotzen syndrome, Pfeiffer syndrome, Pfeiffer's syndrome, cranial expansion, mid-face advancement, midface advancement
