cme management of...

CME

Management of CraniosynostosisJayesh Panchal, M.D., M.B.A., and Venus Uttchin, B.A.Oklahoma City, Okla.

Learning Objectives: After studying this article, the participant should be able to: 1. Review the etiopathogenesis ofcraniosynostosis and craniofacial anomalies. 2. Develop a basic understanding of the clinical manifestations and diagnosisof craniofacial anomalies. 3. Describe the surgical principles of managing craniosynostosis and craniofacial anomalies.

Craniosynostosis, or the premature closure of calvarialsutures, results in deformed calvaria at birth. Although theetiology of craniosynostosis is currently unknown, animalexperiments and a recent interest in molecular biologypoint toward interplay between the dura and the under-lying brain. This interaction occurs by means of a localalteration in the expression of transforming growth factor,MSX2, fibroblast growth factor receptor, and TWIST. Thefused suture restricts growth of the calvaria, thus leadingto a characteristic deformation, each associated with adifferent type of craniosynostosis. Uncorrected craniosyn-ostosis leads to a continuing progression of the deformity,and in some cases, an elevation of intracranial pressure.Clinical examination should include not only an exami-nation of the skull but also a general examination to ruleout the craniofacial syndromes that accompany cranio-synostosis. Because deformational plagiocephaly, or pla-giocephaly without synostosis, occurs secondary to sleep-ing in the supine position during the early perinatalperiod, the physician should be aware of this abnormality.Treatment for deformational plagiocephaly is conserva-tive when compared with treatment for craniosynostosis,which requires surgery. Appropriate investigations shouldinclude genetic screening, radiologic examination with acomputerized tomographic scan, and neurodevelopmen-tal analysis. Surgical intervention should be performedduring infancy, preferably in the first 6 months of post-natal life, to prevent the further progression of the de-formity and possible complications associated with in-creased intracranial pressure. The principles of surgicalintervention are not only to excise the fused suture butalso to attempt to normalize the calvarial shape. Long-term follow-up is critical to determine the effect of thesurgical outcome. (Plast. Reconstr. Surg. 111: 2032, 2003.)

The calvarial sutures serve two importantfunctions: (1) maintenance of head malleabil-ity during passage through the birth canal, and(2) continuance of separation of the calvarialbones during intrauterine and early perinatal

life. The sutures serve as growth sites wherenew bone is deposited in response to the con-tinuing separation of the osteogenic fronts be-tween the opposing bones.1 Premature closureof any of the calvarial sutures prevents separa-tion of the calvarial bones. Inevitably, this pro-duces a restriction on growth vectors, leadingto a morphologic change in calvarial shape.These changes are specific and characteristicfor every type of craniosynostosis.2–4 However,the sequence of events leading to prematureossification of sutures is unknown. Biome-chanical forces and genetically determined lo-cal expression of growth factors have been im-plicated in the etiology of craniosynostosis.4–10

Although Sommering11 proposed that thecalvarial suture was the primary locus of theabnormality, Virchow12 popularized the con-cept. Later, Moss13 conceptualized that the cra-nial base was the primary locus of the abnor-mality in children with craniosynostosis andthat the altered cranial base transmitted thetensile forces through the dura. This ultimatelyled to premature closure of the calvarial su-ture. To study the changes in the cranial base,Babler et al.14 performed suturectomy in rab-bits with and without dural transection. Theirfindings suggested that suturectomy and tran-section of the dura did not affect growth morethan suturectomy alone. This evidence indi-cated that the dura played a less important rolethan believed by Moss.13 Eaton et al.15 exam-ined the skulls of North Indian tribes whosemembers intentionally modified children’sskull shapes by head binding. They concluded

From the Oklahoma University Health Science Center. Received for publication March 6, 2002; revised September 13, 2002.

DOI: 10.1097/01.PRS.0000056839.94034.47

2032

that a deformity of the cranial vault, eithercongenital or intentional, alters the structureof the endocranial base and face. This sug-gested that the endocranial base might not bethe primary anomaly in bicoronal and sagittalcraniosynostosis.

Over the past decade, the role of the dura inmaintaining sutural patency has been exten-sively studied. Through a series of experi-ments, Opperman et al.7,16 –20 demonstratedthat the dura initially plays an inductive role.Later, it assumes a permissive role in maintain-ing sutural patency through various signalingfactors. Levine et al.21 studied the role of thedura in premature synostosis in rats and deter-mined that the dura generates abnormal sig-nals. These signals could be blocked by a Silas-tic sheet interposed between the dura and theoverlying suture. Most et al. documented anincreased level of transforming growth factor-beta and messenger RNA levels locally, indicat-ing an interaction between the dura and over-lying sutures at the time of closure.22 Later,Mooney et al.23 demonstrated that dura matertransplanted from beneath normally patent su-tures to sites of sutural fusion in a naturallyoccurring craniosynostotic rabbit model suc-cessfully kept these sites from re-obliterating.Though the mechanism is not understood, theregional dura mater somehow determines thefate of the overlying suture.6

Sutures continue to remain as growth siteswhere cells undergo proliferation before dif-ferentiating into osteoblasts. The calvarial su-tures produce new bone at the suture front inresponse to the expanding neurocranium. Asthe brain expands, the sutures respond to thisstimulus by adding new bone at the suturefront. The addition of this new bone helps thesuture width remain constant to accommodatethe increasing size of the brain.20 The interac-tion between the brain and patency of theoverlying sutures has been amply proved byclinical evidence showing the premature clo-sure of sutures in the presence of microceph-aly, and continuing patency in the presence ofhydrocephalus. Secondary craniosynostosis canalso develop following placement of a shunt ina child with hydrocephalus (shunt-induced cra-niosynostosis) because of a drop in intracranialpressure and diminution of neural thrust.4 Theneural thrust and patency of the overlying su-tures are closely integrated through the dura.The dura also serves as an intermediary sourceof signaling, which is mediated by transform-

ing growth factor, fibroblast growth factor re-ceptor, TWIST, and MSX2. Genetic studieshave now determined that mutations withinthese factors are responsible for various typesof craniosynostosis.8,24–26 Fibroblast growth fac-tor receptors 2 and 3 mutations were present inall patients with syndromic craniosynostosisand in 74 percent of children with unclassified“nonsyndromic” craniosynostosis.27 The spe-cific source generating these signals and geneamplification is not yet understood.

PATHOGENESIS

The neonate calvaria is composed of thefrontal, parietal, temporal, sphenoid, and oc-cipital bones. The metopic suture separates thefrontal bones, whereas the coronal suture isfound between the frontal and parietal bones.The squamosal suture lies between the parietaland the squamosal temporal bone. The lamb-doid suture is found between the parietal andoccipital bones, and, finally, the sagittal suturelies between the two parietal bones. The patentsutures allow continuing separation of the cal-varial bones. Growth vectors generated by theunderlying cortex dictate this separation. If asuture is prematurely fused, growth is re-stricted in a direction transverse to the fusedsuture. Thus, premature closure of the sagittalsuture results in continuing growth of the cal-varia in the ventral-dorsal direction and dimin-ished growth in the transverse direction, caus-ing scaphocephaly. A pathologically closedmetopic suture prevents the continuing trans-verse expansion of the frontal bones, leadingto a narrow frontal and fronto-orbital region.This anterior narrowing manifests as trigono-cephaly. The coronal suture could close pre-maturely, either unilaterally or bilaterally, re-sulting in unicoronal or bicoronal synostosis,respectively. Bilateral closure of the coronalsuture produces a diminished growth vector inthe ventral-dorsal direction, and compensatorygrowth takes place in the medial-lateral direc-tion. This results in brachycephaly and bilat-eral recession of the fronto-orbital bar. In se-vere cases, exorbitism can result. Unicoronalsynostosis results in diminished growth in theventral-dorsal direction on the ipsilateral sideand compensatory growth on the contralateralside. This asymmetry is known as frontal pla-giocephaly. In this case, the fronto-orbital baris recessed ipsilaterally and projects furtherventrally on the contralateral side. Prematureclosure of the lambdoid suture leads to occip-

Vol. 111, No. 6 / MANAGEMENT OF CRANIOSYNOSTOSIS 2033

ital plagiocephaly with ipsilateral flattening ofthe occipital region and contralateral bulging.The anterior cranial base deviates toward thefused suture in unicoronal synostosis. Similarly,the posterior cranial base deviates toward theipsilateral fused lambdoid suture.28 Becausethe face grows as a template off the cranialbase, uncorrected synostosis leads to asymme-try of the face and occlusal plane.

Closure of multiple sutures can limit theexpansion of a neonate’s calvaria and underly-ing cortex. The continuing expansile forces ofthe cortex against the fixed volume of the cal-varia leads to an increase in intracranial pres-sure. Blindness and possible mental retarda-tion are two problems associated withintracranial pressure. Diagnosing increased in-tracranial pressure in children with craniosyn-ostosis has been difficult. Gault et al.29 discov-ered the trend by measuring intracranialpressure overnight by using an epidural mon-itor in children with craniosynostosis. They re-garded pressure readings above 15 mmHg asconfirmatory for increased intracranial pres-sure, below 10 mmHg as normal, and between10 and 15 mmHg as borderline. Using thissystem, they found that 47 percent of childrenwith multiple-suture craniosynostosis, and 14percent of those with single-suture craniosyn-ostosis, demonstrated increased intracranialpressure. Thompson et al.,30 using a subduralcatheter to measure intracranial pressure,demonstrated that in children with craniosyn-ostosis, 17 percent had increased intracranialpressure, 38 percent had borderline pressure,and 45 percent had a normal reading. Renier31

also demonstrated that intracranial pressuredecreased following surgery. Consequently,there was a direct correlation between the ex-tent of increased intracranial pressure andmental function in a subgroup of 55 children.Because the precise range of intracranial pres-sure levels in “normal ” children is unknown,the true impact of the studies is still unclear.Regardless, Cohen and Persing32 offer a goodreview of techniques and recommendations formeasuring intracranial pressure.

CLINICAL EXAMINATION AND DIAGNOSIS

Although premature fusion of the sutures isa prenatal event, the diagnosis of craniosynos-tosis is not imminent in the neonatal period.The passage of the head through the birthcanal temporarily deforms the head and makesit difficult to ascertain whether it will be shaped

normally or abnormally. Either the parent orthe pediatrician will notice a persistent abnor-mal shape in early infancy, and the child isreferred to either a craniofacial surgeon or aneurosurgeon.

Because the characteristic calvarial deforma-tion is secondary to sutural fusion, clinical di-agnosis is made more easily in the later perina-tal months. Clinical history should include theprimary birth events (length of term, birthweight, and complications during and afterbirth). A detailed history of the perinatal sleep-ing position is also critical in differentiatingcraniosynostosis from plagiocephaly withoutsynostosis (vide infra). Syndromic craniosynos-tosis involves multiple systems (cardiac, genito-urinary, and musculoskeletal), and a family his-tory of abnormal head shape is critical in itsdiagnosis.

Clinical examination should include an ex-amination not only of the head and neck torule out torticollis, but it also should includethe digits, toes, and spine. Measuring the headcircumference aids in ruling out microcephalyand macrocephaly with associated hydroceph-alus. The calvarial shape is characteristic foreach type of sutural synostosis. An increase inanteroposterior length, or scaphocephaly, isassociated with sagittal synostosis (Fig. 1).Transverse narrowing of the anterior calvaria,or trigonocephaly, is associated with metopicsynostosis (Fig. 2). The distance between themedial canthi of the eyes (medial intermedialcanthus distance) is decreased, whereas thedistance between the centroids (center of eachocular globe) is normal. Metopic synostosis istherefore not associated with true orbital hypo-telorism, but the orbital rims may appear hypo-plastic. Bicoronal synostosis results in shorten-ing of the skull in the anteroposterior directionand widening at the bitemporal region. Thefronto-orbital rim is recessed bilaterally, anddepending on the severity of the case and ageof the child, this may lead to exorbitism with itsassociated complication of exposure keratitis(Fig. 3). Unilateral asymmetry, or plagioceph-aly, can result from coronal or lambdoid syn-ostosis. Unilateral coronal synostosis results ina recessed forehead and fronto-orbital rim andcontralateral bossing of the forehead andfronto-orbital rim. The eyebrow on the ipsilat-eral side is raised, and the palpebral fissure isincreased in its vertical height. The ocularglobe is sometimes raised, along with the pupil,resulting in vertical dystopia (Fig. 4). An older

2034 PLASTIC AND RECONSTRUCTIVE SURGERY, May 2003

child with uncorrected unicoronal synostosisdemonstrates asymmetry of the facial skeleton.This is associated with a reduction in height ofthe ipsilateral maxilla and mandible, resultingin an oblique occlusal cant.33 Lambdoid synos-tosis is rare and must be differentiated fromdeformational plagiocephaly, or plagiocephalywithout synostosis.

PLAGIOCEPHALY WITHOUT SYNOSTOSIS

(DEFORMATIONAL PLAGIOCEPHALY)

Plagiocephaly without synostosis, or defor-mational plagiocephaly, is a very commoncause for an asymmetrical head associated withan ipsilateral occipital flattening. The increasein this abnormality has been associated withthe “Back to Sleep” campaign to reduce theincidence of sudden infant death syndrome(SIDS).34 If left in the same position for a pro-longed duration of time, the skull of a newbornchild can easily become deformed because it is

so soft. In addition, infants have limited abilityto move their head in the first few months oflife because of their weak neck muscles. Torti-

FIG. 1. A child with sagittal craniosynostosis. (Above) Lat-eral view demonstrates frontal and occipital bossing and doli-chocephaly. (Below) Superior view demonstrates frontal boss-ing bilaterally and dolichocephaly.

FIG. 2. A child with metopic craniosynostosis. (Above) Su-perior view demonstrates trigonocephaly (narrowing of theanterior calvaria). (Below) Frontal view demonstrates ametopic ridge.

FIG. 3. A child with bicoronal synostosis exhibiting exor-bitism and a recessed frontal orbital bar.


collis, the tightening of neck muscles, may fur-ther complicate the situation by preventing theinfant or parent from repositioning the head.

The deforming force flattens the occipitalskull, and like a deflated ball, it preferentiallytends to lie in that position. The continuingforce flattens the occipital region and pushesthe forehead and ear forward. In extremecases, the cheekbones, jaw joints, and mandi-ble may be involved. This leads to a typicalparallelogram-shaped head, which differs fromunicoronal or unilambdoid synostosis (TableI). This abnormality is known as deformationalplagiocephaly, or plagiocephaly without synos-tosis.35 Other factors, such as prolonged labor,abnormal fetal position, and multiple birthshave also been incriminated in the origin ofplagiocephaly without synostosis. Intentionalchange in positioning or the use of positioningdevices, however, can improve head shape dur-ing this period. Keeping the child in the proneposition while awake also contributes to im-proving head shape. The use of a customized

molding helmet for 23 hours/day until thechild is 1 year of age is also very successful intreating this condition when conservative mea-sures have failed (Fig. 5). An early referral,preferably before the age of 6 months, is criti-cal because molding helmet therapy is not veryeffective after 1 year of age. It is also extremelyimportant to differentiate plagiocephaly with-out synostosis from craniosynostosis (Table I),because treatment of the former includes us-ing either positional therapy or molding hel-met therapy, whereas the latter requires surgi-cal intervention.

INVESTIGATIONS

The clinical diagnosis of craniosynostosis orplagiocephaly without synostosis can be sub-stantiated with radiologic investigations.

Radiography of the Skull

Skull radiography is a preliminary examina-tion to visualize whether or not the sutures arepatent. Although the test is simple, accuracy is

FIG. 5. Customized molding helmet for the treatment ofdeformational plagiocephaly.

FIG. 4. An older child with unicoronal synostosis showingasymmetry of the facial axis and progressive deformity of theasymmetry of the orbits.

TABLE ICharacteristics of Craniofacial Anomalies*

Right Deformational Plagiocephaly Right Unicoronal Synostosis Right Unilambdoid Synostosis

Frontal Ipsilateral R bossingContralateral L flattening

Ipsilateral R flatteningContralateral L bossing

No change

Occipital Ipsilateral R flatteningContralateral L bossing

No change Ipsilateral R flatteningContralateral L bossing

Palpebral fissure Ipsilateral R narrowing or no change Ipsilateral R widening No changeEars Ipsilateral R ventral translocation Ipsilateral R ventral translocation Ipsilateral R dorsal translocationMastoid process No change No change WidenedFace Ipsilateral R ventral translocation Ipsilateral R dorsal translocation No changeNose Deviated to contralateral L side Deviated to ipsilateral R side No changeChin Deviated to contralateral L side Deviated to ipsilateral R side No change

* R, right; L, left.


not perfect and differentiating between a lamb-doid synostosis and plagiocephaly without synos-tosis is sometimes difficult. Special views areneeded to better visualize and appreciate allsutures.

Ultrasonography

Ultrasonography, a noninvasive, nonradio-logic procedure, has recently been studied todetermine its utility in diagnosing a patent su-ture. Ultrasound is more accurate than a radio-graphic examination, but it requires a sonog-rapher who is willing to specialize ininterpreting the results.

Computed Tomographic Scan

A computed tomographic scan is the defini-tive standard for determining whether a sutureis patent or fused. Two-dimensional views allowfor the direct visualization of each suture, butgrasping the extent of the associated deformityremains challenging. This ultimately makespreoperative planning very difficult and affectsoutcome reliability. In contrast, three-dimen-sional computed tomographic scans allow com-plete visualization of the skull and clearly doc-ument the extent of the deformity (Figs. 6 and7). Scanning specific views (i.e., frontal, poste-rior (occipital), right and left profile, superior,and inferior ectocranial and endocranial) can

be used as a protocol to allow comparison be-tween preoperative, perioperative, and postop-erative computed tomographic scans. A radio-graphic scale should also be placed beside theimages to make approximate measurements ofthe various bony landmarks. This aids in pre-operative planning and assists in performing ameaningful outcome analysis. Sophisticatedsoftware has also played a role in determiningthe best option by allowing manipulation ofthe osteotomies on a graphic workstation.36

Sagittal Synostosis

When the sagittal suture is fused, trans-verse growth of the skull becomes restricted(Fig. 8), resulting in a reduction of the trans-verse diameter at the parietal eminencies (bi-parietal diameter) and an increase in theanteroposterior length of the skull. This ex-plains the frontal bossing and occipital pro-trusion associated with sagittal synostosis.The cephalic index (maximum transversewidth/maximum anteroposterior length) isalso reduced. Nomograms for cephalic indi-ces are available and are one way to evaluatethe severity of the scaphocephaly.37 Unfortu-nately, the cephalic index measures only twodimensions of the skull and does not allowassessment of either frontal bossing or occip-ital protuberance. Yet, it is the only readilyavailable method for quantitative assessment.

Metopic Synostosis

The fused metopic suture restricts thetransverse growth of the frontal bones, which

FIG. 6. Three-dimensional computed tomographic scanof a child with metopic craniosynostosis. Note the ridging atthe site of the fused metopic suture.

FIG. 7. Three-dimensional computed tomographic scanof a child with right unicoronal synostosis demonstratingasymmetry of the orbits. The right orbit is narrower and talleras compared with the contralateral side, and the facial axis ismildly deviated.


results in narrowing of the anterior cranialfossa and trigonocephaly (Fig. 6). The re-striction in the transverse direction also re-sults in reduction in the intermedial canthaldistance (distance between medial canthi)and interdacryon distance (distance betweenlacrimal crests), but true hypotelorism (dis-tance between the centroids of the globe)does not exist. The orbital rims may also behypoplastic, depending on the severity of themetopic craniosynostosis. The metopic su-ture is the first suture to close physiologi-cally. Previous studies documented this clo-sure to be in the first and second year ofpostnatal life. However, computed tomo-graphic scans taken in the neonatal periodshow that the metopic suture closes physio-logically at a much earlier age (between 3and 9 months postnatally).38 A physiologicclosure, however, is not associated with thesecondary deformity of narrowing of the an-terior cranial fossa and hypoplastic orbitalrims. The medial intercanthal distanceshould also be measured preoperatively andcompared with a nomogram.38

Bicoronal Synostosis

The bicoronal suture is fused, thus restrict-ing the anteroposterior growth of the anteriorcranial fossa. This results in an increased bi-temporal diameter and a decreased anteropos-terior dimension of the anterior cranial fossa.In addition, the fronto-orbital bar is recessedbilaterally. The supraorbitale (the most prom-inent point of the fronto-orbital rim) is nor-mally 2 mm ventral to the plane of the cornea.However, in a child with bicoronal synostosis,the fronto-orbital bar is recessed and the su-praorbitale is more dorsal (posterior) to thecorneal plane.39 In most circumstances, thisleads to exorbitism with its associated compli-cations. Therefore, the objective of surgery isto place the fronto-orbital bar 2 mm ventral tothe corneal plane.

Unicoronal Synostosis

Unicoronal synostosis is characterized by afused coronal suture. The ipsilateral fronto-orbital bar is recessed, whereas the contralat-eral fronto-orbital bar and frontal bone aremore ventral. These conditions are the resultof secondary deforming changes that occurbecause of the closed suture. When anteropos-terior growth is restricted across the closedcoronal suture, compensatory changes takeplace along the contralateral suture. This crit-ical event must be understood when correctingunicoronal synostosis, because correcting thecontralateral bones will require surgery. Thefused suture also restricts the growth of thesphenoid bone, resulting in a reduction of theanteroposterior dimension of the anterior cra-nial fossa. Consequently, the anterior endocra-nial base deviates toward the ipsilateral side.Similarly, the ectocranium also deviates to theipsilateral side. If uncorrected, deviation of theface and an occlusal cant may result. The pos-terior cranial base, however, remains unaf-fected. The roof of the orbit, formed by thegreater wing of the sphenoid, is raised suchthat the shape of the ipsilateral orbit becomesmore vertical (Fig. 7) compared with the morehorizontal orbit on the contralateral side.40

Lambdoid Synostosis

Lambdoid synostosis is characterized by anobliterated ipsilateral lambdoid suture that re-duces the anteroposterior dimension of theposterior cranial fossa. The protruding con-tralateral suture and bulging ipsilateral mas-

FIG. 8. Preoperative superior view of a child with sagittalcraniosynostosis demonstrating dolichocephaly.


toid process are distinguishing characteristicsof lambdoid synostosis. The posterior cranialbase will deviate to the ipsilateral side, whereasthe anterior base is unaffected. The greatestdifficulty lies in differentiating between defor-mational plagiocephaly (plagiocephaly withoutsynostosis) and lambdoid synostosis. Synostosissimply restricts growth on the ipsilateral side,but deformational plagiocephaly deforms byexerting force in a ventral direction. Thus,lambdoid synostosis involves the petrous por-tion of the temporal bone being pulled towardthe closed lambdoid suture. Correspondingly,the external auditory canal is also pulled to-ward the fused suture. In contrast, both thepetrous portion of the temporal bone and theexternal auditory canal are pushed anteriorlyin deformational plagiocephaly. Ipsilateralfrontal bossing occurs regularly in deforma-tional plagiocephaly but almost never in lamb-doid synostosis. Thus, diagnosing deforma-tional plagiocephaly, unlike in lambdoidsynostosis, is made by assessing secondarychanges instead of the suture itself. This elim-inates diagnosis of partial suture closures andlambdoid suture sclerosis or thickening.

MAGNETIC RESONANCE IMAGING

A magnetic resonance imaging scan helps todelineate the pattern of cortical gyri and sulciunderneath the fused suture. Whether the ab-normal pattern is secondary to the pressurefrom the overlying fused suture or is a primaryevent leading to a fused suture is often de-bated. A simple follow-up study comparing thepreoperative magnetic resonance imagingscans with the 1-year postoperative scans wouldoffer some insight. However, it has been verydifficult to objectively measure the extent ofgyri and sulci flattening, despite having usedsophisticated software from the National Insti-tutes of Health. Lack of a centralized databaseof magnetic resonance imaging scans fromnormal children of a specific age further com-plicates the study. The major limitation of themagnetic resonance imaging scan is that it onlyallows for anatomical delineation of the cortexand provides no information about corticalfunction.

NEURODEVELOPMENTAL ANALYSIS

The purpose of neurodevelopmental testingis to obtain information about the corticalfunctioning before and after cranial vault re-modeling. The Bayley Scales of Infant Develop-

ment–II are often used to procure this infor-mation.41 The greatest difficulty withneurodevelopmental testing is the lack of ac-curacy in measuring cortical function in aninfant 3 to 6 months of age. Therefore, a lon-gitudinal assessment is essential in a thoroughanalysis. These tests compare the child’s men-tal and psychomotor scales with a nomogram,thus helping to quantify whether the child isdevelopmentally delayed and if surgery willhelp reduce the severity of that delay. Childrenwith syndromic craniosynostosis, characterizedby multiple system involvement, are often de-velopmentally delayed. This delay can be se-vere and the child could be classified as men-tally retarded. Although children with single-suture craniosynostosis often do not seem tohave significant developmental delays,41–43 amore detailed assessment with sophisticatedanalyses has shown otherwise.44 Children withmetopic and sagittal craniosynostosis also haveminor delays in learning and speech, a prob-lem not previously recognized in this popula-tion.45,46 A study showed that children with de-velopmental delays at 12 months also exhibitedcognitive and motor deficiencies during theirpreschool years.41,47,48 Understandably, a3-month-old infant cannot be tested for lan-guage delays, but the Bayley Scales of InfantDevelopment–II is a comprehensive test thatincludes aspects of the child’s developmentalskills. For these reasons, this test is a reliabletool for assessing infants.

NEED FOR SURGERY

The aim of surgical intervention is to excisethe prematurely fused suture and correct theassociated deformities of the calvaria. Uncor-rected synostosis quite frequently is associatedwith an increase in intracranial pressure. Thishas been documented in the animal modeland in humans.29,49 If the synostosis goes un-corrected, the deformity progresses to involvethe facial skeleton, which is associated withasymmetry of the face and malocclusion. Asym-metry of the orbit leads to ocular dystopia andconsequent strabismus. Therefore, the surgicalgoal is to increase the intracranial volume, es-pecially under the fused suture, and preventany long-term complications. Normalization ofthe calvarial shape successfully achieves thisgoal.

Surgery is best performed in early infancybecause:


• Most brain growth occurs in the first year oflife. The deforming vectors of the continuallygrowing brain result in progression of thedeformity with increasing age.

• Few studies have demonstrated an increasein intracranial pressure in nonsyndromicsingle-suture craniosynostosis.29,49

• Osseous defects following surgery undergore-ossification more completely before 1year of age as compared with later.

• A delay in surgery beyond the first 9 to 12months of life leads to progressive deformityof the cranial base, resulting in abnormalfacial growth and asymmetry of the maxillaand mandible.50

• The calvaria in a child 3 to 9 months of ageis still malleable and, therefore, quite easy toshape.

For these reasons, there is now an increasingconsensus among craniofacial surgeons andneurosurgeons that surgery should be per-formed in early infancy.

SAGITTAL CRANIOSYNOSTOSIS

Sagittal craniosynostosis is characterized byan increased growth in the anteroposterior di-rection and a reduction in the transverse direc-tion (Fig. 8). This results in frontal bossing andoccipital protrusion. Consequently, the objec-tive of surgery is to correct the secondary de-formational changes in addition to excising thefused sagittal suture. Traditionally, the aim hasbeen to excise only the fused suture in thehope that the secondary changes would auto-matically correct themselves as the brain growsin the first year of postnatal life. This simpleobjective was achieved by removing the fusedsuture with a procedure called a strip craniec-tomy. Initial strip craniectomies were associ-ated with refusion, and various agents wereused to keep the sutures patent.51 Numerousmodifications have been made to strip craniec-tomies; currently, as many as 16 types of pro-cedures have been collated in an attempt toelucidate the rationale of performing surgeryfor sagittal craniosynostosis.52 Over the past de-cade, craniofacial surgeons have become moreaggressive in trying to correct the suture andthe associated deformities of frontal bossingand occipital protrusion, or bathrocephaly.The procedure of cranial vault remodeling in-volves excision of the frontal, parietal, and oc-cipital bones. These are trimmed, reshaped,and then relocated and affixed with absorbable

plates. The primary surgical objective is to re-lease the synostotic constraint and promotenormal calvarial growth. Reducing the antero-posterior length and increasing the transversewidth also improves calvarial contour.

A retrospective quantitative analysis (usingthe cephalic index) of 40 infants who under-went surgery for sagittal craniosynostosis wasconducted to determine whether any differ-ence in outcome could be associated with ei-ther the child’s age at surgery or the extent ofthe operation. Despite the extended stripcraniectomies performed before 4 months ofage, the study concluded that cranial vault re-modeling was more effective in normalizingthe cranial shape (Fig. 9).53

Recently, craniofacial surgeons and neuro-surgeons have been using an endoscope toperform extended strip craniectomies, fol-lowed by molding helmet therapy for 6 to 8months postoperatively.54 Although this tech-nique reduces intraoperative time, length ofhospital stay, and blood loss, it does not allowthe surgeon to intraoperatively alter the calvar-ial shape or cephalic index. After all, the pro-cedure remains a strip craniectomy despite itsbeing performed with an endoscope. Thoseauthors recently reported their results usingthe cephalic index for nine patients who hadundergone endoscopic strip craniectomy. Twopatients had cephalic indices that were belownormal, and seven had values within the nor-mal range. Unfortunately, the mean follow-upwas only 10 months after surgery. Also, nocomparison was made between the improve-ments of cephalic index with either technique.This ultimately makes it difficult for the readerto determine whether the combined endo-scopic strip craniectomy and molding helmettherapy is superior to cranial vaultremodeling.55

UNICORONAL SYNOSTOSIS

Preoperative Planning

Ventral advancement of the supraorbital bar. Inunicoronal synostosis, the ipsilateral supraor-bital rim is recessed and dorsal to the plane ofthe cornea.56 A two-dimensional long axis viewalong the apex of the orbit and the center of thecornea demonstrates the extent of the reces-sion. Nomograms have revealed that the su-praorbital rim is approximately 2 to 3 mm ven-tral to the vertical plane of the cornea. Themovement of the supraorbital rim is such that


it is 3 mm ventral to the vertical plane of thecornea. The extent of this movement varies be-tween 7 to 15 mm, depending on the severity ofthe unicoronal synostosis. However, additionalapproaches to advancing the supraorbital barhave been offered by Marchac and Renier57 andPosnick.58

Correcting orbital asymmetry. The ipsilateralroof of the orbit is higher than the contralat-eral orbit. The contralateral orbit, in contrast,is also wider than normal. The extent of the

correction is determined by using nomo-grams. Excising bone at the frontozygomaticand nasomaxillary sutures reduces the heightof the ipsilateral orbit. In addition, insertinga cranial bone graft into the supraorbital rimincreases the width of the ipsilateral orbit.Similarly, the height of the contralateral orbitis increased by elevating the supraorbital bar,and its width is reduced by excising a bonesegment from the supraorbital bar (Figs. 10through 12).

FIG. 9. (Above) Graphical representation of the normalization of cranial shape in childrenwho have undergone external strip craniectomy. Only 29 percent of the postoperative cephalicindexes were within normal range. (Below) Graphical representation of the cephalic indices ofchildren who have undergone subtotal calvarectomy and cranial vault remodeling; 66 percentof the patients had postoperative indices within normal range. Both panels from Panchal, J.,Marsh, J. L., Kauffman, B., Park, T.S., and Pilgram, T. Sagittal craniosynostosis: Outcomeassessment for two methods and timings of intervention. Plast. Reconstr. Surg. 103: 1574, 1999.


BICORONAL SYNOSTOSIS

In treating bicoronal synostosis, the surgicalobjective is to increase the anteroposterior di-mension of the calvaria by ventrally advancingthe frontoparietal bones and the fronto-orbitalbar. Advancement of the latter would increasethe projection of the supraorbital rim 2 mmbeyond the corneal plane. In addition, othertechniques for treating bicoronal synostosishave been proposed by Marchac and Renier.57

For treatment planning, appropriate soft-ware allowing delineation of the bone seg-ments and their advancements can be used.Most of these software programs are cumber-some and require prolonged duration of ma-nipulation before the appropriate position isobtained. Despite the most accurate preopera-tive planning, the current software fails to pro-vide intraoperative confirmation that the de-sired position is being reached. Intraoperative

confirmation of the desired position will prob-ably be available in the future because of therecent surge in frameless stereotacticneurosurgery.

A two-dimensional sagittal image is used forpreoperative calculations. This image traversesthrough the centroid (center of the ocularglobe) and the center of the orbital apex. Theposition of the supraorbitale is confirmed onthis view. The corneal plane is developed bypassing a ray connecting the infraorbitale tothe most prominent point of the cornea. Theray is extended so that it passes 2 mm ventral tothe cornea. This provides an estimate for ven-tral advancement of the fronto-orbital bar.

METOPIC SYNOSTOSIS

In treating metopic synostosis, the surgicalgoal is to increase the volume of the anteriorcranial fossa by performing a ventral advance-ment of the fronto-orbital bar. The reducedinterdacryon distance must be normalized byincreasing the transverse dimension betweenthe pterions to correct the abnormality. This isdone by placing a calvarial bone graft betweenthe two halves to correct the reduced inter-dacryon distance. The width of the bone graftshould be equal to the extent of correctionneeded to normalize the interdacryon dis-tance. If the bar is thick at the midline, a tenonmortise bone joint is used to inherently in-crease the stability of the bone graft. A T-shaped absorbable plate is then used to fix thegraft at the junction of the fronto-orbital barand the nasion.59 Cohen et al.60 provided alter-native techniques in treating metopicsynostosis.

FIG. 10. Graphical representation of the method of cor-rection of the orbital asymmetry. (Above) The right orbit iswider and shorter as compared with the left orbit, which isnarrower and taller. (Center) The right orbit is decreased inwidth by reducing the width of the frontal orbital bar on theright. The left frontal orbital bar is widened by 5 mm.The height of the left orbit is reduced by 3 mm. (Below) Thecorrected position of the right and the left orbit, held inposition by Lactosorb plates and screws. The 5-mm-widebone graft on the left orbit is also fixed by using Lactosorbplates and screws.

FIG. 11. Fronto-orbital bar demonstrating the correctiondescribed in Figure 10. Note the presence of an absorbableplate at the lateral aspect of the frontal orbital bar, which willallow rigid fixation and advancement of the left frontal orbitalbar at the pterion.


POSTOPERATIVE CARE AND FOLLOW-UP

After extubation, the child is transferred tothe pediatric intensive care unit for 24 to 48hours so that hemodynamic stability and levelof consciousness can be monitored. Parentsare informed about the considerable amountof swelling that occurs around the scalp andperiorbital areas, and they are reassured thatthe swelling will subside after a few days. It isnot unusual for the child to develop pyrexia of38°C for the first 72 hours. Continuing pyrexia,persistent swelling, and cellulitis should be fur-ther investigated. Another important point isthat the need for blood transfusions is basedon the hemodynamic stability of the childrather than on a specific hematocrit level.

The senior author performs a perioperativethree-dimensional computed tomographic

scan on the fifth postoperative day. This allowsan accurate method of documenting the os-teotomy sites. In addition, the surgeon canconfirm postoperatively whether the appropri-ate symmetry and ventral advancement wereachieved intraoperatively. The child is dis-charged after completion of the computed to-mographic scans.

Further clinical follow-up is done at 3 weeks,6 weeks, 3 months, 6 months, and 1 year post-operatively. A three-dimensional computed to-mographic scan and magnetic resonance imag-ing scan are also performed 1 year aftersurgery. These tests are valuable for document-ing whether the ventral movement and symme-try of the orbits has been maintained. There-after, annual visits are required until age 6years. Longitudinal follow-up by a craniofacialteam is also recommended to assess the child’sneuropsychologic development and craniofa-cial growth.

COMPLICATIONS

To reduce mortality and morbidity, this sur-gery should be performed in a children’s hos-pital equipped with a pediatric anesthesiologistand pediatric intensive care facilities. Recentseries have demonstrated only isolated cases ofmortality, which often involved insufficientblood replacement during the intraoperativeperiod. Therefore, blood loss should be mini-mized and replaced quickly when necessary.

The incidence of wound infection and dehis-cence is low.61 Cranial bone graft infection canbe minimized significantly by administering a48-hour prophylactic antibiotic regimen.62 Thecurrent authors frequently use nafcillin andgentamicin. Continuing cerebrospinal fluidleakage is also extremely rare if adequate pre-cautions are taken to ensure proper closure ofdural tears intraoperatively.

Long-term follow-up may reveal minor asym-metries of the supraorbital bar and forehead;these can be addressed by elevating the scalpflap and applying a hydroxyapatite paste to thesurface of the calvaria. Ossification within theorbit takes place quite rapidly and is completewithin 1 year after surgery.63 Persistent areas ofincomplete ossification, those larger than 2 cmin diameter, are also similarly treated with hy-droxyapatite paste. Major asymmetries may re-quire a revision osteotomy and re-advancementof the supraorbital bar. This, however, is rareunder current surgical standards.64–66

FIG. 12. Three-dimensional computed tomographicscans demonstrating right unicoronal synostosis. (Above) Inthe preoperative scan, the right orbit is narrower and talleras compared with the contralateral side. Note the deviationin the facial axis. (Below) In the 1-year postoperative scan,note the improvement in the position of the orbits and cor-rection of the deviation in the facial axis.


ABSORBABLE PLATES

Similar to the impact of titanium platingdevices for maxillofacial fixation, absorbableplating systems have revolutionized pediatriccraniofacial surgery by ensuring rigid fixationand a better outcome. The initial enthusiasmof using titanium-plating devices on a child’sgrowing calvaria was quickly followed by com-plications. Resorption of inner table and dep-osition of new bone on the outer table led to agradual movement of the titanium plates andscrews over the calvaria. This movement thenprogressed onto the dura, sometimes comingin direct contact with the brain.3,67–70

At the same time, absorbable plating tech-nology is becoming more dependable.71,72 Ab-sorbable plates are manufactured from a com-bination of polyglycolic and polylactic acids.The addition of polylactic acid increases the

resorption period of the plate. The resorptiontime, therefore, varies from 12 to 36 months,and resorption typically occurs by hydrolysis,which does not incite an inflammatory reac-tion. Studies have documented that the rigidityoffered by absorbable plating devices is equiv-alent to that of titanium devices.73,74 The onlydrawback to this system is that absorbablescrews must be tapped. This process adds timeto the surgery, but with increasing practice, thisis negligible.

USE OF HYDROXYAPATITE BONE SUBSTITUTE

Although hydroxyapatite has been usedquite extensively by orthopedic surgeons forjoint implants and by maxillofacial surgeonsfor chin augmentation, the compound hasbeen available only as blocks. Recently, thematerial has been produced as a powder that is

FIG. 13. Three-dimensional computed tomographic scans demonstrating (above, left) the osseous defect left in the calvariaafter compound comminuted fracture; (above, right) abnormality in the contour and osseous defect over the left temporoparietalskull; (below, left) postapplication of hydroxyapatite into the osseous defect, which is completely obliterated; and (below, right)postoperative superior view demonstrating perfect contour between the right and left temporoparietal regions followingapplication of hydroxyapatite.


mixed with sodium phosphate. The resultantpaste is used to fill osseous defects and to aug-ment frontal or temporal bones. Although thefirst products did not solidify for 24 hours,more recent products solidify in 10 minutes.These newer products give more reliable re-sults even in the presence of moisture andblood. Craniofacial surgeons currently use hy-droxyapatite quite extensively following cranialvault remodeling. For example, hydroxyapatiteis used for osseous defects that have failed toossify or to augment the temporal hollowingand minor asymmetry that may occur after cra-nial vault remodeling (Fig. 13).75,76

CRANIOFACIAL SYNDROMES

Apert syndrome, a rare craniofacial disorder,occurs sporadically in most cases but can betransmitted by an autosomal dominant trait insome families. The syndrome is also known asacrocephalosyndactyly because of the cranialand extremity involvement. It is characterizedby a bicoronal synostosis, turricephaly, severeexorbitism, midface hypoplasia, anterior openbite, and bilateral symmetrical complex syn-dactyly of the digits and toes (Figs. 14 and 15).In addition to developmental delays and hyper-activity, these children quite often suffer fromobstructive sleep apnea because of airway re-striction caused by the midface retrusion.

Crouzon syndrome, or craniofacial dysosto-sis, is a rare disorder with a prevalence of onein 25,000 in the general population. The syn-drome, transmitted as an autosomal dominanttrait, is characterized by exorbitism, midfaceretrusion, and brachycephaly because of thebicoronal synostosis. The midface is character-istically box-shaped and may be associated withhypertelorism. The mandible has normalgrowth but may be secondarily deformed bymaxillary retrusion and malposition (Fig. 16).

In 1964, Pffeifer described a syndrome con-sisting of craniosynostosis, broad thumbs,broad great toes, and, occasionally, partial soft-tissue syndactyly of the hand. The syndrome isinherited as an autosomal dominant trait withcomplete penetrance.

Carpenter syndrome is characterized by cra-niosynostosis in association with preaxialpolysyndactyly of the feet. Short fingers withclinodactyly and variable soft-tissue syndactylymay also be present. Carpenter syndrome istransmitted as an autosomal recessive trait.

Unlike Carpenter syndrome, Saethre-Chot-zen syndrome is inherited in an autosomal

dominant manner. Characteristics of Saethre-Chotzen include craniosynostosis, low-set hair-line, ptosis of the upper eyelids, facial asymme-try, brachydactyly, partial cutaneous syndactyly,and other skeletal anomalies.

The management of craniofacial syndromescan be summarized as follows:

• Step I: Correction of craniosynostosis be-tween the ages of 3 and 6 months.

FIG. 14. Three-month-old child with Apert syndromedemonstrating turricephaly, frontal bossing, and recessedfrontal orbital bar.

FIG. 15. Complex syndactyly of the left hand in a childwith Apert syndrome.


• Step II: Correction of syndactyly betweenthe ages of 1 and 2 years.

• Step III: Correction of midface retrusionwith distraction techniques by the age of 4 to5 years. Timing and progress of the distrac-tion may vary, depending on the severity ofobstructive sleep apnea, malocclusion, andpsychological disturbance.

• Step IV: Correction of hypertelorism andturricephaly, if present, at age 4 to 6 years.This may be done in conjunction with, orseparately from, step III.

• Step V: Await full maturity and perform LeFort I or Le Fort III procedure in conjunc-tion with mandibular osteotomy to normal-ize appearance and correct malocclusion.

Jayesh Panchal, M.D.Oklahoma University Health Science CenterWP 2220 Section of Plastic Surgery920 Stanton L. Young BoulevardOklahoma City, Okla. [email protected]

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Self-Assessment Examination follows onthe next page.


Self-Assessment Examination

Management of Craniosynostosisby Jayesh Panchal, M.D., M.B.A., and Venus Uttchin, B.A.

1. WHICH OF THE FOLLOWING STATEMENTS REGARDING DEFORMATIONAL PLAGIOCEPHALY ISCORRECT?A) It is often due to an infant sleeping in the prone position in the early perinatal period.B) It is associated with occipital flattening, ipsilateral frontal bossing, and ventral translocation of the ipsilateral ear.C) It is associated with occipital flattening, contralateral frontal bossing, and ventral translocation of the contralateral ear.D) It can be treated successfully with molding helmet therapy as late as 24 months of age.E) It is familial in most cases.

2. WHICH OF THE FOLLOWING FINDINGS IS MOST CONSISTENT WITH CROUZON SYNDROME?A) ExorbitismB) Unicoronal synostosisC) Complex syndactylyD) HypotelorismE) Mandibular hypoplasia

3. THE PRIMARY OBJECTIVE IN SURGICAL TREATMENT OF SAGITTAL CRANIOSYNOSTOSIS IS TO:A) Reduce the cephalic indexB) Alter the shape of the calvaria to attain a normocephalic shapeC) Decrease the transverse dimensions of the calvariaD) Resect the abnormal suture lineE) Increase cranial volume

4. SURGICAL TREATMENT FOR CRANIOSYNOSTOSIS IS BEST PERFORMED AT WHICH AGE?A) 1 to 6 weeksB) 1 to 6 monthsC) 1 to 2 yearsD) 2 to 4 yearsE) Later than 4 years

5. RADIOGRAPHIC FINDINGS ON COMPUTED TOMOGRAPHY IN A CHILD WITH UNICORONALCRANIOSYNOSTOSIS ARE BEST DESCRIBED BY WHICH OF THE FOLLOWING?A) Deviation of the cranial base to the contralateral sideB) Ipsilateral roof of the orbit lower than the contralateral sideC) Increased height of the orbit on the contralateral sideD) Deviation of the cranial base toward the ipsilateral sideE) Frontal bossing on the ipsilateral side

To complete the examination for CME credit, turn to page 2132 for instructions and the response form.

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