ORIGINAL PAPER

Neurodevelopmental and esthetic results in childrenafter surgical correction of metopic suture synostosis: a singleinstitutional experience

Mathias Kunz & Markus Lehner & Alfred Heger &

Lena Armbruster & Heike Weigand & Gerson Mast &Aurelia Peraud

Received: 30 May 2013 /Accepted: 2 December 2013# Springer-Verlag Berlin Heidelberg 2013

AbstractIntroduction Metopic suture synostosis leading totrigonocephaly is considered the second most frequent typeof craniosynostosis. Besides esthetic results, we present 25consecutive pediatric cases operated upon metopic suturesynostosis with a focus on the child’s motor, speech, andneurocognitive development.Methods Twenty-five children (aged 6 to 33 months; median9.2 months) with trigonocephaly were operated upon between2002 and 2012 with fronto-orbital advancement includingfrontal bone cranioplasty and fronto-orbital bandeau remodel-ing. Neurodevelopmental deficits were evaluated by a stan-dardized questionnaire including gross motor function, man-ual coordination, speech, and cognitive function performed byindependent pediatric/developmental neurologists before sur-gery and at 6 and 12 months of time interval postoperatively.Results Twenty-one (84 %) boys and four (16 %) girls wereincluded in this study. Mean follow-up period was 33±28 months. Outcome analysis for esthetic results showed a

high degree of satisfaction by the parents and treating physi-cians in 23 cases (92 %). Preoperative evaluation revealedneurodevelopmental deficits in 10 children (40 %; six mild,four moderate degree). Twelve children (48%)were proven tohave a normal preoperative neuropediatric development. Mildor moderate developmental restraints were no longer apparentin 6/13, improved but still apparent in 3/13, and stable in 4/13,6 months after cranial vault reconstruction. At 12 months offollow-up, deficits were no longer present in 9/13 and improvedin 4/13. Apart from this cohort, two children were diagnosedwith a syndromic form, and one child had a fetal valproatesyndrome. In these three children, neurodevelopmental deficitswere more pronounced. Neurocognitive progress was obvious,but was comparably slower, and major deficits were still appar-ent at last follow-up. All children with proven mild/moderate/severe deficits received intensive physiotherapy, logopedic, orneurobehavioral support.Conclusions As shown in a single-center observation, surgicalcorrection of metopic suture synostosis not only refines estheticappearance but also might improve neurodevelopmental out-come if deficits are apparent, even in syndromic forms of thedeformity under additional physiotherapy, logopedic, or neuro-behavioral support.

Keywords Metopic suture . Premature closure . Synostosis .

Trigonocephaly . Fronto-orbital advancement . Estheticresults . Neuropediatric development

Introduction

The patency of open cranial sutures allows the skull to moldduring delivery and enables the developing brain to expandand to grow. Premature closure of the cranial sutures is calledcraniostenosis and provokes characteristic skull deformities

M. Kunz : L. Armbruster :A. Peraud (*)Department of Neurosurgery, Klinikum Großhadern,Ludwig-Maximilians-University Munich, Marchioninistrasse 15,81377 Munich, Germanye-mail: Aurelia.Peraud@med.uni-muenchen.de

M. Lehner :A. HegerDepartment of Pediatric Surgery, Ludwig-Maximilians-UniversityMunich, Lindwurmstrasse 4, 80377 Munich, Germany

H. WeigandDepartment of Pediatric Neurology, Ludwig-Maximilians-UniversityMunich, Lindwurmstrasse 4, 80377 Munich, Germany

G. MastDepartment of Craniomaxillofacial Surgery,Ludwig-Maximilians-University Munich, Lindwurmstrasse 2a,80377 Munich, Germany

Childs Nerv SystDOI 10.1007/s00381-013-2340-0

depending on the suture involved. A premature closure of themetopic suture between both frontal bones consequently leadsto trigonocephaly with narrowing of the orbits and the fore-head. Apart from the esthetic aspect with shallow temples andhypotelorism, impaired skull growth in the frontal region canlead to increased intracranial pressure and thereby impairednormal development. The main purpose of craniostenosisrepair is to prevent developmental delay and severe deformi-ties in an esthetic relevant area.

The range of incidence of metopic synostosis has beenreported to be rather wide with an increasing rate over therecent decades to 25.5 % [1]. Kweldam et al. reported on acurrent incidence of 1:5,200 newborns [2].

Metopic suture synostosis leading to trigonocephaly wasconsidered the third most frequent single-suturecraniostenosis/-synostosis after scaphocephaly andplagiocephaly. However, according to increasing incidenceover the last few decades, trigonocephaly is now the secondmost frequently seen type of craniosynostosis [1, 3–5]. Thenormal growth in the sutures is perpendicular to the orienta-tion of the suture. A premature closure of a single suture isaccompanied by compensatory growth in other sutures and byappositional growth in other parts of the skull [6, 7]. Intrigonocephaly, the skull becomes prominent in the parieto-occipital region, while it remains very short and narrow in theforehead with ridging of the metopic suture. In addition,development of the midface is impaired with narrow orbitsand a small midface. Apart from the esthetic aspects of ametopic synostosis, normal intellectual development of a childdepends significantly on the ability of the growing brain toexpand. Pressure on the developing cerebral cortex may havea detrimental effect on the child’s intelligence, although un-equivocally raised intracranial pressure is found only in about50 % of the patients, even with multiple suture fusions [8].

We present the clinical and esthetic results in 25 consecu-tive pediatric cases operated uponmetopic suture synostosis atthe Pediatric Neurosurgical Unit of the Ludwig-Maximilians-University, Munich, with special focus on the child’sneurocognitive, motor, and speech development.

Material and methods

Patient selection

All pediatric patients presenting with the clinical and radio-logical signs for a premature closure of the metopic suture andwho had craniostenosis repair between August 2002 and May2012 were included in the present study. Informed consentabout the developmental evaluation was obtained for allpatients.

The typical clinical appearance included a narrow foreheadwith ridging of the metopic suture, hypotelorism, fleeing

eyebrow region, and shallow temples. This was confirmedon a low-dose spiral CT scan with 3D reconstruction or morerecently on MR reconstructions. Fundoscopy was performedroutinely to rule out increased intracranial pressure. In addi-tion, patients underwent thorough neurodevelopmental examsby pediatricians or pediatric neurologists and, in suspectedcases, a genetic work-up for a syndromic form oftrigonocephaly.

The surgical correction of this complex three-dimensionalgrowth restriction caused by metopic synostosis was per-formed by a team consisting of a pediatric neurosurgeon(AP, MK, LA), pediatric surgeon (ML, AH), and a cranio-maxillo-facial surgeon (GM). The operative techniques aswell as the postoperative clinical course and complicationsare described.

Patient evaluation

Clinical and esthetic follow-up examination was performed 3,6, and 12months after surgery, followed by yearly intervals upto 5 years. Date of last follow-up was October 2012. Apartfrom standard measurements of the skull circumference, es-thetic results were evaluated by AP, GM, MK, and ML withinthe follow-up period to determine whether the metopic ridgingand width of the forehead were satisfactorily corrected. Es-thetic results have been photo documented by AP, GM, andML from three different positions (anterior, lateral, and supe-rior view) and compared to the preoperative shape and exten-sion of the forehead. In addition, at the time of follow-upinvestigations, parents were interviewed about their subjectivesatisfaction with esthetic results as well as the postoperativedevelopmental achievements of their children.

Due to the fact that the neurodevelopmental assessment ofchildren at the age of 12 months or younger is difficult, astandardized questionnaire including gross motor function(e.g., trunk control, head elevation, standing, walking), man-ual coordination (grasping), speech (e.g., intentional crying,vocalization), and cognitive function (e.g., observation, hand–mouth–eyes exploration) was applied and conducted by inde-pendent pediatric/developmental neurologists before surgeryand at 6 and 12 months of time intervals postoperatively [9,10]. Neuropediatric development was evaluated according tothe achievement of motor, speech, and cognitive milestonesthat have been reached/not reached at the corresponding age.The applied standardized questionnaire is shown in Table 1.

Preoperative developmental deficits were regarded as mild,moderate, or severe, if the corresponding motor, speech, andcognitive milestones achieved by each child was comparable/equivalent to children of 3 (mild), 6 (moderate), or more than6 (severe) months younger in age. At time intervals of 6 and12 months postoperatively, any functionally relevant changesin neurological development as compared to the preoperativestatus was classified as improvement if deficits changed from

Childs Nerv Syst

Tab

le1

Neuropediatricdevelopm

entatthe

ageof

0to

5years

Age

(months)

36

912

1518

2436

4860

Gross

motor

function

Headelevation

inprone

position,

basedon

the

forearms

Whenslow

lybe

pulledup

tositting

positio

n,thearmsarebent;

head

followstrunk

Free

sitting

inuprightposition

oftheback

and

steady

control

ofhead

Steadystanding

whileholding

onwallsor

furnitu

res

Steady

walking

whileholding

onparents’

hands,walls

orfurnitures

Self-contained

walking

with

good

balance

control

Steady

running

with

dodging

obstacles

Jumping

onboth

legs

downfrom

botto

mstep

Well-coordinated

pedalingand

controlling

atricycleor

similar

A/descendingstairs

freehand

with

legexchange

Manual

coordination

Hands

andfingers

arebrought

together

inthe

middle

Item

sandtoys

are

transferredfrom

1hand

totheother;

grasping

with

wholehand

Item

sareheld

in1

orboth

hands,

intensivetactile

exploration

Pincergrip

with

thum

band

indexfinger

2blocks

canbe

placed

on1

anotherupon

request

Objectsheldin

the

hand,belent,

putinaboxor

takenouto

nrequest

Bookpagesare

turned

individually,

sweetsare

unwrapped

skillfully

Smallo

bjectsare

takenaccurately

with

thefingertip

sandplaced

onanothersite

Crayonisheld

correctly

inthe

hand

Scissorscanbe

used

correctly,

simple

handicraft,

coloring

templates

accurately

Speech

Intentionalcrying

(hunger,

discom

fort,

pain)

Spontaneous

and

varyingrange

ofvocalizing

Vocalizingwith

repeating

“a”-lutes

(wa-wa-ra-ra)

Syllabledoubling

with

“a”(m

ama,

papa,dada)

Imitationof

speech

Figurativespeech

(wau-w

au)

1-to

2-word

sentences

3-to

5-wordsentences:

“i”,“you”,plural;

speaks

foritself

whileplaying

Experienced

things

aretold

chronologically

andlogically

correct

Accurate

pronunciation,

butsim

ple

gram

matical

structures

Cognitio

nMovingobjects

aretracked

with

theeyes

Objectstransferred

from

1hand

tothe

otherandstuck

into

themouth;

activities

inthe

immediatesurrounding

aremonitored

closely

Intensivetactile,

oralandvisual

exploration

ofobjects

Finds

anobject

againquickly,

which

was

hidden

underhiseyes

Objectsare

manipulated

andchecked

fortheir

simpleusability

Buildsatower

of2–4blocks;

likes

tolook

atage-appropriate

picturebooks;

pointsto

familiar

objects

Smallroleplay

(doll,teddy

bear);

approaches

toself-initiated

constructiveplay

Drawingof

“cephalopods”,

commentson

what

was

painted;

objectsare

abstracted

into

themeaning

and

thus

used

ingames

Wh-questions;

listening

while

readingand

explaining;

understands

roleplay

more

sophisticated

butstillo

nits

own

Intensifydetailed

roleplay

with

otherchildren;

constructive

games

with

/without

templates

Astandardized

neurodevelopmentalq

uestionnaire

includinggrossmotor

functio

n,manualcoordination,speech,and

cognition

[2,11]

was

appliedbefore

operationandat6and12

monthsof

follo

w-up

investigationby

independentp

ediatric/developmentaln

eurologists

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mild to no longer apparent (n.a.), from moderate to mild/n.a.,and from severe to moderate/mild/n.a., or as deterioration incase of worsening or new appearance of deficits. In all othercases, the postoperative status was evaluated to be stable.

Results

Between 2002 and 2012, 25 consecutive cases oftrigonocephaly were treated with fronto-orbital advancementincluding frontal bone cranioplasty and fronto-orbital bandeauremodeling. There were 21 (84 %) boys and four (16 %) girls.Median age at the time of surgery was 9.2 months (range, 6–33 months). Mean follow-up period was 33±28 months. Twochildren (8 %) were diagnosed later on with a syndromic formof metopic suture synostosis. In one syndromic child, a mu-tation in the TWIST1 gene could be identified. Despite inten-sive investigation, a definitive mutation in the othersyndromic child could not be detected, but clinically, a syn-drome had to be suspected due to severe retardation andtypical stigmata such as low ear position, auricle abnormali-ties, and syndactyly.

On preoperative work-up, fundoscopy was normal in allpatients. Preoperative CT or MR images showed prematurefusion solely of the metopic suture even in the syndromiccases.

Surgical procedure is to some extent standardized, and wemention in the following only surgical aspects that have beenmodified at our institution.

After careful positioning and limited hair shaving, abicoronal skin incision was tailored in multiple soft curvesfor optimal esthetic results. Using a monopolar needle, thelayer between galea and periosteum was dissected down to-wards the supraorbital bar with preservation of the supraorbit-al nerve. Bifrontal craniotomy was performed, and the supra-orbital bandeau was released with an oscillating mini-saw,remodeled by an open wedge osteotomy, internally fixed withabsorbable plates and screws. To smoothen the bony edgesand to improve the contour of the temples, the periosteum andthe temporal muscles were reattached to the bandeau. Thebifrontal bone flap was rotated and incised in order to modela symmetrical and anatomical well-contoured forehead. Fixa-tion was accomplished with absorbable plates and screws aswell as sutures. At the level of the bandeau, the plates andscrews were placed on the inner side to prevent visible degra-dation reactions in the facial area.

Perioperative complications

Although the surgical procedure was performed at a youngage with a mean body weight of 8.8±1.1 kg, patients toleratedsurgery very well, and there was no 30-day morbidity. Due tothe large area of exposed, well vascularized dura and bone

(still participating in hematopoesis), the limited blood volumeat this age and the length of the procedure of 3 to 4 h, the meanblood loss was 291±137 ml [21–49 % of circulating bloodvolume]. Blood transfusion (a mean of 177±126 ml) wasnecessary in all but two children, both of which were olderthan 1 year. Prophylactic antibiotics (third-generationcephalosporin) were given for the first 5 days. All childrendeveloped periocular swelling to a varying degree that re-solved completely during the hospital stay. Mean hospital staywas 7 days.

Esthetic results

Esthetic results showed a high degree of parents’ satisfactionin 23 cases (92 %) at follow-up investigations. The parents ofone boy had very high expectations and were not completelyhappy because they assumed the forehead still is too narrowdespite his perfect developmental progress. Another patienthad, in addition to his trigonocephaly, a positionalplagiocephaly, and this was most likely the reason why theparents of this child were not absolutely satisfied despite theperfect shape of the forehead also on MR scans. Estheticresults have been photo documented by AP, GM, and MLfrom three different positions (anterior, lateral, superior view)and compared to the preoperative shape and extension of theforehead.

No reoperation due to recurrent skull deformity of theforehead or temporal hollowing was necessary in any of thecases.

In three children, a transient swelling around the absorb-able plates and screws was observed around 12 to 18 monthsafter surgery. No revision was necessary, and the granuloma-tous reaction disappeared within the subsequent 6 months.

Neuropediatric development

Twelve children (48 %) were found to have a normal preoper-ative neurological development. In 10 children (40 %) standard-ized neuropediatric evaluation revealed neurodevelopmentaldeficit of a mild (n =6) and moderate (n=4) degree (Table 2).Deficits consisted of a mild or moderate impairment with regardto crawling or sitting due to hypotony/low tone of trunkmusclesor deficits in manual coordination in six and two children,respectively; an additional delay in speech development in twoand in cognitive development in one of these children. Twochildren presented with deficits in speech development in a mildand moderate degree in one child each without any otherlimitations.

Three children (12 %) showed more pronounced develop-mental delay with lack of head control, delayed motor func-tion of the hands, and with additional restraints in speech andcognition. Two of the latter group had a syndromic form, andthe other child had a known fetal valproate syndrome.

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During the postoperative course, the 12 unaffected childrencontinued to develop within the normal time and achieved alldevelopmental milestones (Table 2). Children with a mildform of delay in motor function (n =6) caught up with thoseof the same age under intensive physiotherapy in four casesand persisted in two cases at 6 months of follow-up. However,the respective delay was no longer apparent in all patients at12 months of follow-up. Moderate delay in motor functionwas obvious in two children. At 6 months of follow-up,deficits improved to a mild degree in one child and moderatelypersisted in the other. At 12 months of follow-up, both chil-dren had improved with physiotherapy, but still exhibited amild degree of motor delay. In eight children with provenmildand moderate delay in motor development, deficits were nolonger apparent in four, improved, but were still apparent inone and were unchanged in three children at 6 months offollow-up. At 12 months of follow-up, motor deficits wereno longer detectable in six and improved, but were still ap-parent in two children. Children with preoperative delay inspeech development (n =4), moderate deficits improved to amild but still apparent deficit at 6 and 12 months of follow-upin one child, and mild deficits (n =3) were no longer apparent

in two cases at 6 months of follow-up and in all three cases at12 months of follow-up. All of these children receivedlogopedic therapy. Cognitive delay was evident to a moderatedegree in one child and showed steady improvement underbehavioral therapy at 6 and 12 months of follow-up.

Mild or moderate developmental restraints were no longerapparent in 6/13, improved, but were still apparent in 3/13 andwere stable in 4/13 6 months after cranial vault reconstruction.At 12months of follow-up, deficits were no longer apparent in9/13 and improved, but were still apparent in 4/13 (Table 2).

The two syndromic children and the child with fetalvalproate syndrome also improved with regard to theirpreexisting severe motor, speech, and cognitive deficits underintensive physiotherapy, logopedic, and neurobehavioral sup-port, but neurocognitive progress was considerably slower. At6 months of follow-up, severe deficits (n =8) improved in onlyhalf of the deficits; at 12 months of follow-up, an improve-ment was obvious in six deficits, but were still apparent in amoderate degree.

In the patient cohort, no deterioration or appearance of newneurodevelopmental deficits occurred at 6 and 12 months offollow-up.

Table 2 Neurodevelopmental status and postoperative progress

shtnom21U/Fshtnom6U/FstluserevitarepoerPF/U

Motor Speech Cognition Motor Speech Cognition Motor Speech Cognition

1 Moderate Mild − Moderate n.a. − Mild n.a. − 38

2 Mild − Moderate n.a. − Mild n.a. − Mild 48

3 Mild Mild − n.a. Mild − n.a. n.a. − 50

4 − Moderate − − Mild − − Mild − 24

5 − Mild − − n.a. − − n.a. − 91

6 Moderate − − Mild − − Mild − − 12

7 Mild − − n.a. − − n.a. − − 4

8 Mild − − Mild − − n.a. − − 18

9 Mild − − Mild − − n.a. − − 86

10 Mild − − n.a. − − n.a. − − 19

11 Severe Severe Severe Severe Moderate Moderate Moderate Moderate Moderate 66

12 Severe Severe Moderate Moderate Severe Moderate Moderate Severe Moderate 24

13 Severe Severe Severe Moderate Severe Severe Moderate Moderate Severe 8

A detailed analysis of 13 children with preoperative deficits in motor, speech, or neurocognitive development and the respective postoperative course at 6and 12 months is provided. According to the standardized neurodevelopmental questionnaire, children’s performance was categorized in mild, moderateor severe, and n.a. (deficits not longer apparent). Among the severely delayed children, two had a syndromic form of metopic suture craniostenosis, andone had a known fetal valproate syndrome. Their performance had improved postoperatively but to amuch lesser degree than those in the non-syndromiccases. Right column displays the total follow-up in months

Childs Nerv Syst

Discussion

Due to the increased incidence over the last few decades,metopic synostosis (trigonocephaly) has been shown to bethe second most frequently seen type of craniosynostosis [1,3–5].

Whether the marked increase in occurrence oftrigonocephaly stated by several authors is due to a higherawareness of this disease among the medical community andpublic, due to improved diagnostic techniques or due to ge-netic or environmental or even mechanical or pharmacologi-cal factors, is still not resolved [12]. As was seen in our series,a male predominance in trigonocephaly is generally observed.

In the present study, esthetic results were very satisfactoryin almost all children of our cohort evaluated by the parentsand treating physicians at 3, 6, and 12 months of follow-upinvestigations and up to the age of 5 years. However, thisevaluation may be biased from both sides and prospectivethree-dimensional digital measurement of the forehead pre-and postoperatively will be performed in the future.

Furthermore, no complications have been observed despitethe extent of surgery in this very young age group with arelatively high rate of blood loss observed (mean blood loss291±137 ml; 21–49 % of circulating blood volume). Bloodtransfusion became necessary in all but two children. Theseresults are in accordance to the literature with a medianaverage amount of blood loss of less than 255 ml, rangingfrom 80 to 600 ml and blood transfusion in all cases [13].

No reoperations due to forehead deformities or temporalhollowing were necessary in all cases during follow-up. Re-ported complications in the literature are infection, hematoma,seroma, cerebrospinal fluid leakage, and recurrent bitemporalnarrowing requiring repeated surgical interventions. In theseries of Hormozi et al., eight revisions in 60 patients aftertrigonocephaly repair were reported [14]. In the literature, areoperation rate of up to 15% due to temporal hollowing is notunusual [15, 16].

While some authors report on mental impairment in chil-dren with craniostenosis [17], there is growing evidence thatsingle-suture craniostenosis do affect normal neurologicaldevelopment [18–23]. Neurodevelopmental delays in metopicsynostosis are reported in the literature ranging from 15 % toas high as 61 % [1, 24–26]. In comparison to sagittal suturestenosis, neurobehavioral studies on children affected bymetopic synostosis are far less common and less precise.There is some evidence of learning and/or behavioral deficits.Shimoji et al. [27, 28] theorized that even mild forms ofmetopic ridging can be associated with significant develop-mental delay, language problems, and hyperactivity.Mendonca and coworkers reported about 6 out of 20 patientsundergoing frontal orbital advancement and remodeling ex-hibit speech and language problems with no consistent trendobserved linking the severity of frontal stenosis using the

measured parameters with speech and language delays [11].In a recent study, Da Costa and coworkers evaluated theneurodevelopmental functioning of infants with untreatedsingle-suture craniosynostosis during early infancy [26]. Theyassumed that abnormal sutural fusion during the gestationaland perinatal phase could compromise brain development andconcomitant cognitive development. Boterro et al. evaluated76 children with metopic synostosis: 32 % of the operatedchildren showed developmental delay, while the incidence innonoperated children with mostly milder forms oftrigonocephaly was 23% [18]. Their results support the theorythat the developmental delay in metopic synostosis primarilyderives from the brain and might not be a direct result of thecraniosynostosis acting as a growth restrictor. The positivedevelopment in children treated for trigonocephaly at ourinstitution reported by their parents was the reason to performthis study and to include the neurodevelopmental progressapart from the esthetic results. Despite parents even reportinga slight acceleration in the development of their childrenwithin the first weeks after operation, we had to face theproblem that neurodevelopmental progress after surgery re-ported by the parents is not objective. Due to the fact thatneurodevelopmental assessment and quantification of deficitsin children at the age of 12 months or younger is difficult, astandardized questionnaire including gross motor function,manual coordination, speech, and cognitive developmentwas applied (Table 1) [9, 10] and conducted by independentpediatric/developmental neurologists before surgery and at 6and 12 months of time interval postoperatively. The intentionof the present study was to analyze the early effects of metopicsuture stenosis repair on the achievement of developmentalmilestones.

In the current study, 52 % of the children showedneurodevelopmental deficits: 40 % of the children exhibiteda moderate (i.e., corresponding milestones achieved compa-rable to children of 6 months younger in age) or mild (com-parable to children of 3 months younger in age) delay inmainly motor function (n =8), but also in speech development(n =4) and rarely in cognitive function (n =1). Apart from thiscohort, three children (12 %) suffered from a severe (compa-rable to children of >6 months younger in age) degree ofneurodevelopmental delay. Of these, two children weresyndromic, and one child was known to suffer from fetalvalproate syndrome. The authors admit that these three chil-dren compose a completely different cohort and are not com-parable to the other ten children due to other underlyingcauses.

Despite the fact that these children underwent a majorsurgical procedure at a very young age, an improvement wasobserved to some degree even at 6 months of follow-up. But at12 months of follow-up, motor deficits were no longer appar-ent in 75 % and improved, but were still present in 25 %.Comparable results after 12 months were observed in children

Childs Nerv Syst

with deficits in speech development. Even in severeneurodevelopmental deficits in two syndromic children andin one child with fetal valproate syndrome, an improvement toa moderate degree could be achieved in half of the deficits at6 months of follow-up. However, improvement was compa-rably slower despite intensive supportive therapy, but never-theless promising results could be observed at 12 months offollow-up with an amelioration of initially severe to moderatedeficits. Requirements for this relatively high improvementrate in all children were constant and intensive physiotherapy,logopedic, and neurobehavioral training irrespective of thedegree of deficits. One major limitation in the present studyis the relatively small number of patients treated at a singleinstitution over a 10-year period. Furthermore, we have toadmit that our series did not include children with mild formsof trigonocephaly and possible neurodevelopmental deficitsthat were surveyed without fronto-orbital advancement. Inthese cases, children might have improved under intensivetraining, too. Nevertheless, improvement rates at 6 monthsafter cranial vault remodeling imply some evidence that theseeffects are somehow related to the decompression of the brainand not solely attributable to the natural course of child’sdevelopment under intensified physiotherapy and logopedictherapy. Among the pediatric subspecialties dealing with chil-dren suffering from craniostenosis, there is a growing aware-ness that standardized parameters of care for craniostenosispatients and their caregivers as well as the medical communityare necessary. In this respect, Warren and coworkers haverecently published a reasonable reporting system [29]. Theindividual number of cases treated per year even in a veryactive pediatric neurosurgical unit is limited due to the lowincidence of this deformity. It would be therefore desirable toinclude all patients within a national or even internationalregistry applying general preoperative parameters as well aspostoperative outcome scales evaluating both esthetic resultsby three-dimensional digital measurements of the foreheadand standardized neurodevelopmental status and progress ina prospective fashion.

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