results of early surgery for sagittal suture synostosis: long-term follow-up and the occurrence of...

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ORIGINAL PAPER Results of early surgery for sagittal suture synostosis: long-term follow-up and the occurrence of raised intracranial pressure Marie-Lise C. van Veelen & Oscar H. J. Eelkman Rooda & Tim de Jong & Ruben Dammers & Leon N. A. van Adrichem & Irene M. J. Mathijssen Received: 22 October 2012 / Accepted: 3 January 2013 / Published online: 20 January 2013 # Springer-Verlag Berlin Heidelberg 2013 Abstract Purpose Numerous techniques are used to correct sagittal synostosis. Although cosmetic results and operative compli- cations are well documented, little is known about function- al outcome. In our institution, the technique for extended strip craniectomy evolved over time. This study compares cosmetic results, complications, and signs of raised intracra- nial pressure (ICP) between the variants of the extended strip craniectomy. Methods Seventy-nine consecutive patients undergoing early extended strip craniectomy for scaphocephaly (20022008) were included. Four techniques were used: A, a simple bilat- eral parietal flap with out-fracturing of the bone flap; B, C, and D included remodeling of the parietal flap by adding triangu- lar cuts and bending or suturing the resulting fingers. In technique D, the sagittal strip was rotated and fixed between the parietal flaps. Data on head circumference (HC), skull X- ray, and fundoscopy were collected prospectively. Results For all patients, the average cranial index (CI) was 74 after 3 months and 72 after 2 years. Although technique D resulted in the best initial improvement, there was no significant percentage increase in CI after 24 months be- tween the four techniques. Postoperatively, 9 % of the patients developed papilledema, 42 % developed a fontanel bulge, and 57 % had diminished HC. Four patients were reoperated on because of raised ICP. Conclusions Postoperative CI is mainly determined by preoperative CI and hardly affected by type of extended strip craniectomy. Signs of raised ICP occurred more frequently than expected, therefore structural follow-up is required to detect such signs. Technique and timing of surgery should aim at creating sufficient intracranial volume. Keywords Scaphocephaly . Surgery . Fontanel bulge . Head circumference . Papilledema . Coronal suture fusion Introduction Scaphocephaly describes the skull deformation that occurs after early synostosis of the sagittal suture. The elongated skull, typically accompanied by a bulging forehead and prominent occiput, is mainly a cosmetic concern. However, if left uncorrected, 1020 % will develop increased intra- cranial pressure (ICP) [7, 17, 19, 40, 41, 48]. Patients have a normal IQ but minor delays in development of speech may be observed in 3040 %. This finding seems unrelated to timing and extent of surgery [7, 9, 10, 13, 14, 28, 29, 33, 44]. Numerous techniques to correct scaphocephaly have been described, starting in 1889 with a strip craniectomy Clinical trial registration This study was not subject to the Medical Research Involving Human Subjects Act (WMO) since this study does not involve any form of invasion of the study participants integrity. M.-L. C. van Veelen : O. H. J. Eelkman Rooda : R. Dammers Department of Neurosurgery, Dutch Craniofacial Centre Erasmus MC, Sophia Childrens Hospital, Rotterdam, The Netherlands T. de Jong : L. N. A. van Adrichem : I. M. J. Mathijssen Department of Plastic, Reconstructive and Hand Surgery, Erasmus MC, Sophia Childrens Hospital, Rotterdam, The Netherlands M.-L. C. van Veelen (*) Department of Pediatric Neurosurgery, Erasmus Medical Centre, PO Box 2060, 3000, Rotterdam, The Netherlands e-mail: [email protected] Childs Nerv Syst (2013) 29:9971005 DOI 10.1007/s00381-013-2024-9

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Page 1: Results of early surgery for sagittal suture synostosis: long-term follow-up and the occurrence of raised intracranial pressure

ORIGINAL PAPER

Results of early surgery for sagittal suture synostosis:long-term follow-up and the occurrence of raisedintracranial pressure

Marie-Lise C. van Veelen & Oscar H. J. Eelkman Rooda &

Tim de Jong & Ruben Dammers &

Leon N. A. van Adrichem & Irene M. J. Mathijssen

Received: 22 October 2012 /Accepted: 3 January 2013 /Published online: 20 January 2013# Springer-Verlag Berlin Heidelberg 2013

AbstractPurpose Numerous techniques are used to correct sagittalsynostosis. Although cosmetic results and operative compli-cations are well documented, little is known about function-al outcome. In our institution, the technique for extendedstrip craniectomy evolved over time. This study comparescosmetic results, complications, and signs of raised intracra-nial pressure (ICP) between the variants of the extendedstrip craniectomy.Methods Seventy-nine consecutive patients undergoing earlyextended strip craniectomy for scaphocephaly (2002–2008)were included. Four techniques were used: A, a simple bilat-eral parietal flap with out-fracturing of the bone flap; B, C, andD included remodeling of the parietal flap by adding triangu-lar cuts and bending or suturing the resulting fingers. Intechnique D, the sagittal strip was rotated and fixed betweenthe parietal flaps. Data on head circumference (HC), skull X-ray, and fundoscopy were collected prospectively.

Results For all patients, the average cranial index (CI) was74 after 3 months and 72 after 2 years. Although techniqueD resulted in the best initial improvement, there was nosignificant percentage increase in CI after 24 months be-tween the four techniques. Postoperatively, 9 % of thepatients developed papilledema, 42 % developed a fontanelbulge, and 57 % had diminished HC. Four patients werereoperated on because of raised ICP.Conclusions Postoperative CI is mainly determined bypreoperative CI and hardly affected by type of extendedstrip craniectomy. Signs of raised ICP occurred morefrequently than expected, therefore structural follow-upis required to detect such signs. Technique and timingof surgery should aim at creating sufficient intracranialvolume.

Keywords Scaphocephaly . Surgery . Fontanel bulge .Headcircumference . Papilledema . Coronal suture fusion

Introduction

Scaphocephaly describes the skull deformation that occursafter early synostosis of the sagittal suture. The elongatedskull, typically accompanied by a bulging forehead andprominent occiput, is mainly a cosmetic concern. However,if left uncorrected, 10–20 % will develop increased intra-cranial pressure (ICP) [7, 17, 19, 40, 41, 48]. Patients have anormal IQ but minor delays in development of speech maybe observed in 30–40 %. This finding seems unrelated totiming and extent of surgery [7, 9, 10, 13, 14, 28, 29, 33,44].

Numerous techniques to correct scaphocephaly havebeen described, starting in 1889 with a strip craniectomy

Clinical trial registration This study was not subject to the MedicalResearch Involving Human Subjects Act (WMO) since this study doesnot involve any form of invasion of the study participant’s integrity.

M.-L. C. van Veelen :O. H. J. Eelkman Rooda : R. DammersDepartment of Neurosurgery, Dutch Craniofacial CentreErasmus MC, Sophia Children’s Hospital, Rotterdam,The Netherlands

T. de Jong : L. N. A. van Adrichem : I. M. J. MathijssenDepartment of Plastic, Reconstructive and Hand Surgery,Erasmus MC, Sophia Children’s Hospital, Rotterdam,The Netherlands

M.-L. C. van Veelen (*)Department of Pediatric Neurosurgery, Erasmus Medical Centre,PO Box 2060, 3000, Rotterdam, The Netherlandse-mail: [email protected]

Childs Nerv Syst (2013) 29:997–1005DOI 10.1007/s00381-013-2024-9

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of the synostosed suture by Lannelongue and Lane [30,31]. Several implants were introduced to prevent the stripcraniectomy from early closing, such as polyethylenefilm [23], tantalum foil [45], or Zenker’s solution [5].The strip craniectomy was extended with additional cra-niectomies to correct parietal [11, 12, 24] but also frontaland occipital deformation [3, 20, 50]. In the 1970s,surgical techniques evolved into total cranial vaultremodeling [21, 34, 43, 46, 47]. Recently, the pendulumswung back to extended variants of the strip craniectomy,but with additional techniques like endoscopy, followedby helmet treatment or combined with distraction devices[15, 22, 25, 32].

The timing and extent of surgery continues to be amatter of debate. Lack of knowledge on the remaininggrowth potential and the minimally required intracranialvolume of the scaphocephalic skull prevent this debatefrom being settled. Limited interventions like strip cra-niectomy are performed early (≤6 months) and rely onremaining growth potential of the “released” skull. Totalcranial vault remodeling is performed around the age of1 year and aims at constructing a definitive skull shape,large enough to be independent of suture growth. Cos-metic results and operative complications have beenextensively evaluated, but little is known about func-tional outcome.

This study compares the variants of the extended stripcraniectomy used in our institution, focusing on cosmeticresults and complications, as well as on signs of stagnatingskull growth and raised ICP.

Patients and methods

This study included a consecutive series of patients withsagittal suture synostosis who underwent extended stripcraniectomy before the age of 6 months (corrected forprematurity) between 2002 and 2008 in the Dutch Cranio-facial Center.

Patient data were collected prospectively according to aprotocol including preoperative skull X-ray, 3DCT, fundo-scopy, digital imaging, and head circumference (HC). Post-operatively, skull X-ray, HC, fundoscopy, and grading of theoperative result by the surgeon was performed at 3 monthspostsurgery, at age 1, 2, 4, and 6 years, and then every3 years until age 18 years.

The postoperative result was graded by the surgeon asgood in case of no or slight residual signs of primaryabnormality, as acceptable in case of visible residual abnor-mality, such as frontal bossing, supraorbital narrowing oroccipital protuberance, and finally as unacceptable in caseof important and cosmetically disturbing abnormality of thehead shape. Postoperative frontal bossing and supraorbital

Fig. 1 Outline of the fourdifferent techniques (a–d): atechnique A (simple flap), btechnique B (fingers loose), ctechnique C fingers sutured,and d technique D (with bridge)

Table 1 Characteristics of the patient population

No. of patients 79

Surgery performed Between 2002 and 2008

Males 66

Females 13

Mean preoperative cranial index 64.7 (SD, 3.9; range, 65.1–73.7)

Mean age at surgery (months) 4.8 (range, 3–8)

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narrowing were independently scored by two surgeons on a3-point scale. Cranial index (CI) was measured on the skullX-ray as follows: (maximal cranial breadth/maximal craniallength)×100. Closure of the coronal suture was assessed onskull X-ray. The occurrence of a bulge at the level of theanterior fontanel was scored in the chart as small or largeand also assessed on skull X-ray. Information on the occur-rence of headache was retrieved from the clinical charts.Although follow-up information was not complete for allpatients, none had to be excluded.

Patients were operated on in prone position. All operationtechniques included removal of a bone strip 4 cm wide at thelevel of the synostosed sagittal suture and the creation of aparietal flap by a craniotomy just behind the coronal sutureand just in front of the lambdoid suture down to the squa-mosal suture. This was followed by out-fracturing of theparietal flap. Variations were introduced to remodel theparietal flap. In technique A, the flap was only shortenedat the anterior and posterior rim; in technique B, the con-cavity of the parietal flap was increased by adding darts(triangular cuts); in technique C, the distal ends of the“fingers” were sutured together to maintain concavity; andin technique D, the bone strip was rotated 90° and used toconstruct a widening bridge between the two remodeledparietal flaps. Figure 1 shows the craniotomy lines and thedifferent techniques. These technical variations were intro-duced over time in order to improve the surgical result.Surgical technique was independent of type of deformationwith, e.g., saddle, or prominent frontal bossing.

Statistical analysis was performed with SPSS 16.0. Tocompare CI and HC at different time points with one tech-nique, the Wilcoxon signed rank test was used. The Krus-kal–Wallis test was used to compare CI and HC betweentechniques at different time points. Fisher’s exact test wasused to analyze the relationship between the occurrence ofpapilledema and other parameters thought to be associatedwith increased ICP.

Results

Between 2002 and 2008, 79 patients with scaphocephalypresented at such a young age that they could undergo anextended strip craniectomy. Mean follow-up was 3.9 (range,0.2–9; standard deviation (SD), 2.3) years. Patient character-istics are shown in Table 1; the majority was male. Surgerywas performed at a mean age of 4.8 months (range, 3–8 months (not corrected for prematurity)). Table 2 shows

the distribution of patients by surgical technique and Table 3the surgical results. Operative complications were the samefor all techniques. Mean blood loss was 213 (50–400) cm3,two patients were reoperated on because of postoperativehemorrhage.

In the surgeons’ opinion, the cosmetic result was good in58 (76 %), acceptable in 16 (21 %), and unacceptable in 2(3 %) patients. One of these latter patients was reoperated on.

Photographs were obtained in 64 of the 79 patients. Scoresfor frontal bossing and supraorbital narrowing are listed inTable 3. About one third of the patients showed residualbossing or supraorbital narrowing. Frontal bossing becameless visible with age. Photographs made before age 4 yearsshowed more visible frontal bossing than photographs madeat age 4 years and older, i.e., 17 and 5 %, respectively.

Table 2 Numbers of proceduresaccording to surgical technique(A–D)

A (simple flap) B (fingers loose) C (fingers sutured) D (with bridge)

No. of procedures 19 12 36 12

Table 3 Surgical results

Surgical complications

Mean blood loss (cm3) 213 (range, 50–400)

Dural tear 0

Postoperative hemorrhage 2

Infection 0

Outcome

Surgeon’s opinion

Good 58 (76 %)

Acceptable 16 (21 %)

Unacceptable 2 (3 %, 1 patient was reoperated on)

Frontal bossing

None 35/64 (55 %)

Slight 23/64 (36 %)

Visible 6/64 (9 %)

Supraorbital narrowing

None 40/64 (63 %)

Slight 22/64 (34 %)

Visible 2/64 (3 %)

Cranial index

3 months 0.74

1 year 0.72

2 years 0.72

Adverse events during follow-up

Papilledema 7/79 (9 %)

Fontanel bulge 33/78 (42 %)

Decrease in head circumference(>1 SD)

36/63 (57 %)

Coronal suture fused at 1 year 14/69 (20 %)

Headache 13/69 (19 %)

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Supraorbital narrowing did not show this difference. For allpatients, the CI improved from 65 preoperatively to 74 at3 months postsurgery. CI decreased during the first year post-surgery and then remained stable at 72. Table 4 and Fig. 2present data on the pre- and postoperative CI of the surgicaltechniques. CI showed a significant improvement comparedwith preoperative CI immediately after surgery, as well as at24 months. Fingers sutured (technique C) and “simple flap”(technique A) had the best postoperative CI, but preoperativeCI was not evenly distributed. The preoperative CI of techni-ques A and C was significantly better compared with techni-ques B and D. Therefore, the data were normalized and wecalculated percentage changes in CI. Then, technique D (withbridge) resulted in the best initial improvement of CI, althoughthe difference was not significant. CI showed a significantdrop in most patients, and initial differences in percentageincrease of CI between techniques disappeared. At 24 monthspostsurgery, improvement in CI stabilised around 110 % andshowed no significant difference between the techniques. Infact, when analysed by linear regression, postoperative CI wasinfluenced more by preoperative CI (p=0.044) than by thetechnique (ns). Preoperative CI showed a linear relationshipwith postoperative CI with a R2 of 0.294.

Decrease of HC, occurrence of a bulge at the site of theanterior fontanel, fusion of the coronal suture and the occur-rence of papilledema and headache were adverse events thatoccurred in all techniques. Small differences between techni-ques were found but none of them were significant. We thenanalyzed these adverse events with respect to the presence ofpapilledema since papilledema is the most significant indica-tor of raised ICP (Table 7). All were more frequent in thepresence of papilledema, although only headache and earlyfusion of the coronal suture were statistically significant.

HC before surgery was +2.0 SD. After surgery HC showeda significant decrease, continued to decrease until 24 months,and then stabilised around 0.6 SD (Table 5; Fig. 3). This effectoccurred with all four techniques, although “fingers sutured”(technique C) showed the smallest decrease in HC.

We observed a bulge at the site of the anterior fontanel(Fig. 4) in 42 % of the patients; this occurred at ±1 yearpostsurgery. Figure 4 shows an example of a child presentingwith a nonsyndromic sagittal synostosis who developed a largebulge. Compared with small bulges, the proportion of largebulges increased over time (9 % at 1 year vs. 15 % at 2 yearspostsurgery), indicating that these bulges have a tendency togrow. Only rarely did a bulge disappear spontaneously. Thebulge occurred with all techniques, but less so (difference notsignificant) with technique C (fingers sutured). The occurrenceof a fontanel bulge was more frequent in patients with apostoperative decrease in HC of ≥1 SD (p=0.01) and inpatients with early closure of the coronal suture (p=0.021).

Of all patients, 20 % showed fusion of the coronal suturewithin 1 year postsurgery. Decrease in HC was 0.9 SD if thesuture was fused and 0.4 SD if the suture was patent.Papilledema was more likely to occur after early fusion ofthe coronal suture. Premature coronal suture closure oc-curred more frequently after techniques C and D (24 %)than after techniques A and B (17 %). However, this differ-ence was not significant.

Postoperatively, seven (9 %) patients developed papille-dema; in one of these patients, papilledema was transient

Table 4 Preoperative (preop) and postoperative (postop) cranial index (CI) according to the different techniques

Simple flap (SD) Fingers loose (SD) Fingers sutured (SD) With bridge (SD)

Preop CI 66.1 (3.7) 62.9 (2.9) 65 (4.0) 63.1 (3.8)

Postop CI (6–12 months) 72.2 (5.0) 72.4 (2.9) 74.4 (5.0) 73.3 (3.1)

CI (>24 months) 74.2 (4.8) 68.7 (4.2) 73.9 (5.3) 69.1 (2.6)

Preop CI 100 100 100 100

Postop CI (6–12 months) 110 (7.0) 113 (7.0) 113 (7.0) 115 (6.0)

p=0.003 p=0.068 p=<0.001 p=0.018

CI (>24 months) 110 (7.0) 108 (7.0) 110 (8.0) 110 (6.0)

p=0.046 p=0.028 p=0.008 p=0.012

Data are absolute numbers and percentage change

Fig. 2 Cranial index (CI) by type of surgery

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(Table 6). Six patients had an ICP measurement, of whomthree had to be reoperated on because of raised ICP. Thethree other patients had borderline raised ICP (baseline10–15 mmHg during overnight measurement). In one ofthem, papilledema resolved spontaneously, one is stillfollowed with visual evoked potentials and one was oper-ated on because of persistent papilledema and severeheadaches. Postoperative papilledema was found betweenages 2.5–9 years. TWIST and FGFR2 mutations weretested to rule out Saethre–Chotzen or Crouzon syndrome.However, none of the seven patients were positive forthese mutations. Papilledema occurred more frequently inpatients with headache, with bulges, and with a decreasein HC of ≥1 SD. Frequencies are listed in Table 7; thecorrelation with headache and with early closure of thecoronal suture was significant (p=0.01 and p=0.027,respectively).

Information on the occurrence of headache was noted in85 % of the charts. Frequent headache (≥1×/month) wasreported in 19 % of cases. Headache occurred more fre-quently in the presence of papilledema.

Discussion

Cosmesis is classically the least debatable reason to operateon sagittal synostosis.

However, objective and reproducible cosmetic outcomeparameters are difficult to define. The CI is the mostfrequently used outcome parameter for cosmetic results inscaphocephaly surgery. It is an objective but rather crudemeasure as it does not appreciate clinical signs likefrontal bossing, supraorbital narrowing, and occipital bul-leting. Some studies add a (subjective) grading of theseclinical signs on a 3- or 4-point scale [11, 37]. Antunezet al. developed additional indices for the frontal andoccipital reduction to improve the information that isprovided by simply calculating the CI [6]. Toma et al.used 3D photogrammetry and calculated the vertex height[49]. Both methods seem to add valuable information andit would be interesting to see whether these methodscorrelate with clinical gradings. In the present study,neither the surgeons’ grading of the postoperative result,nor the more detailed grading of frontal bossing andsuprorbital narrowing, showed any relation with CI.

The overall CI in our study is 74 at 3 months postsurgery,decreasing to 72 at 2 years. The range of CI after surgery forscaphocephaly is reported to be 71–79 [2, 4, 6, 18, 26, 27,34, 37, 39]. Most studies measure CI within 1-year postsur-gery; however, long-term studies show that the CI tends todrop after operative remodeling [2, 16]; our results confirmthese results. Compared with the preoperative CI, all ourtechniques resulted in a significant improvement of CI im-mediately after surgery and on the longer term. Modifica-tions of the technique for extended strip craniectomy, usedin our center, showed no further significant improvement inCI. Technique D (with the widening bridge) attained the bestimprovement of CI compared with preoperative CI; howev-er, this technique also showed the largest decline in CI. Thismay imply that introducing a bridge between the parietalflaps initially increases width but on the longer term restrictslateral growth of the skull. Alternatively, this may simplyillustrate the importance of preoperative CI. Although sur-gery results in a significant improvement in CI, the preop-erative CI remains the most important determinant of thepostoperative result on the long term. This finding impliesthat results of corrective surgery for scaphocephaly shouldbe reported as percentage increase of CI rather than absolutepostoperative CI values.

HC decreased from +2 SD preoperatively to less than +1SD at 12–24 months of age, after which HC stabilized.Fearon et al. [16] described a decline between early (6–12 weeks) and late (4.7 years; range, 3–11 years) postoper-ative measurements of HC of ≥1 SD. Toma et al. [49]reported that HC was significantly larger in patients with

Table 5 Head circumference(HC) before and after correctionfor scaphocephaly with differenttechniques

Data represent HC expressed asSD

Simple flap (SD) Fingers loose (SD) Fingers sutured (SD) With bridge (SD)

Preop HC 1.9 (0.8) 2.0 (1.1) 1.9 (0.8) 2.1 (1.1)

Postop HC(6–12 months)

1.1 (1.1) 0.9 (1.4) 1.5 (0.9) 1.3 (1.2)

HC (>24 months) 0.6 (1.1) 0.7 (1.3) 0.8 (1.0) 0.6 (1.3)

Fig. 3 A decrease in HC (in SD) after surgery

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sagittal synostosis than in the normal population, before andat (mean) 8 months after surgery, although the magnitude of

the difference was significantly reduced after surgery.Patients in the study of Fearon et al. were operated on at

Fig. 4 Postoperativelyoccurring fontanel bulge in onepatient. a Preoperative skull X-ray and CT scan. b Postopera-tive skull X-ray

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(mean) 8 months of age and underwent an occipital remod-elling; patients in the study of Toma et al. were operated onat (mean) 11 months and underwent a complete cranialremodeling [16, 49]. Therefore, it seems that the decline inHC we found is a common phenomenon and occurs afterdifferent types of surgery at different ages. To what extent adecline is acceptable in operated sagittal synostosis remainsunknown. After all, with the correction of the shape of theskull, more optimal dimensions are created for the brain,which allows a smaller HC.

In the present study group, a bulge occurred ±1 year aftersurgery at the level of the anterior fontanel. Although thisbulge is the same phenomenon as the vertex bulge describedby Marucci et al. [35], the site is shifted frontally, probablydue to differences in operating techniques. The vertex bulgeoccurs at the site were the remodeled skull finally closes,apparently under circumstances of raised ICP. Marucci et al.[35] were the first to describe the vertex bulge. In a series of 89patients, they found a bulge in seven patients (8 %), occurringat (mean) 8 months after surgery; five patients underwent ICPmonitoring, of which four had elevated ICP. We found 42 %bulges at 1 year after surgery. The occurrence of a fontanelbulge was associated with a decrease in HC of ≥1 SD (p=0.01). This finding supports the hypothesis that a bulge occursas a result of raised ICP. The correlation with early closure ofthe coronal suture (p=0.021) evokes a picture of oxycephaly.Vinchon et al. describe nine patients who developed oxyce-phaly after surgical correction of sagittal synostis in a studyincluding 447 patients [51]. However, in the present study,the high incidence of bulges and coronal suture closuresuggests a secondary effect to surgery rather than a latedevelopment of primary oxycephaly.

The coronal suture was not identifiable on skull X-ray in20 % of all patients within 1 year after surgery. Arnaud et al.[8] reported an incidence of premature closure of the coronalsuture in 10.4 % of patients with scaphocephaly operatedwith the H craniectomy compared with 1.2 % in a group of

nonoperated patients with scaphocephaly. They concludedthat early closure of the coronal suture does occur sponta-neously in patients with scaphocephaly but that surgerymust have an additional effect. It is, however, unclear howsurgery does affect the closure of the suture. Arnaud et al.[8] removed the coronal suture in addition to the H craniec-tomy in 24 patients but none of them subsequently devel-oped premature fusion of the coronal suture. If directsurgical insult to the suture such as complete removal ofthe suture does not influence closure of the suture thenanother aspect of the intervention has to be responsible.We suggest that perhaps surgical release of the skull isfollowed by a period of under pressure in which the incen-tive for the suture to stay patent is removed. This situation iscomparable to overshunting or microcephaly. The percent-age of early closure of the coronal suture is higher in thepresent study than in the study of Arnaud et al. The highincidence may be explained by differences in surgical tech-nique. In our study measures, were taken to increase con-cavity in two out of the four techniques and the fontanel wasnot covered with a piece of bone to prevent early closure atthe level of the sagittal suture. Both aspects might havecontributed to an under pressure early after surgery. In thestudy by Arnaud et al. [8] as well as in our study earlyclosure of the coronal suture is associated with the occur-rence of papilledema at a later age.

The occurrence of postoperative papilledema after cor-rection for scaphocephaly is rarely reported. Arnaud et al.[8] found papilledema in two patients in a group of 193patients who underwent H craniectomy for scaphocephaly.However, it is not reported at what age papilledema oc-curred and how many patients were routinely tested. Adamoet al. [1] retrospectively studied calvarial growth restrictionin a group of 164 patients. They found two patients whorequired second surgery at 23 and 36 months of age, respec-tively; both had papilledema. In the current study 9 % ofpatients developed papilledema postoperatively. This largernumber may be related to the fact that all patients underwentroutine fundoscopy until the age of 6 years and later onindication. In most studies, fundoscopy is not performedroutinely. The percentage of 9 % is particularly high whenjudged in view of the reported incidence of raised ICP in10–20 % of nonoperated cases [7].

In a previous study at our institute, 103 patients with asagittal suture synostosis born between January 2003 and

Table 6 Information on papilledema

Preoperatively 2 patients (2.5 %)

Postoperatively 7 patients (9 %)—1 transient and six 24-h ICPmonitoring (3 raised ICP>surgery and 3 borderline>1 signs resolved and 1 followed up by VEP)

Age at onset (2.2–9 years)

Table 7 Relation between pap-illedema and other variablespossibly related to raised intra-cranial pressure

Headache Bulge Decrease in headcircumference

Early closurecoronal suture

Technique D(bridge)

Papilledema (+) 67 % (4/6) 57 % (4/7) 71 % (5/7) 57 % (4/7) 29 % (2/7)

Papilledema (−) 14 % (9/63) 41 % (29/71) 55 % (31/56) 16 % (10/62) 14 % (10/72)

p=0.01 p=0.448 p=0.689 p=0.027 p=0.287

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July 2007 were evaluated for the presence of papilledema[17]. Of these patients, 62 had a total vault reconstructionbetween 9 and 12 months of age, and 1 of them (1.6 %)developed papilledema postoperatively. In the study of Flo-risson et al. [17], the follow-up was shorter compared withour study; however, this cannot account for the importantdifference between these percentages. This raises the ques-tion whether the limited corrections in the present study aresufficient to reach the primary goal for surgery, i.e., elimi-nating or significantly reducing the risk of raised ICP.

Other signs associated with increased ICP (e.g., decreasein HC, a fontanel bulge, early closure of the coronal suture)were frequent in our study group. This adds to the suspicionthat the early timing of our procedure and/or the restrictedextensiveness of the correction is not sufficiently effective.

This finding should be taken into consideration in thecurrent eagerness to perform minimally invasive cranio-plasties. Minimal incisions are a great improvement andcan be combined with extensive craniotomies [36, 38].However, minimal incisions reduce the possibilities forremodelation and necessitate the use of additional meas-ures. One might expect a helmet to be restricting, al-though a recent study by Ridgeway [42] shows increasedHC persisting until 3 years after surgery. Theoretically,spring-mediated cranioplasty could be a good solution,because of the additional distraction.

Conclusions

Early extended strip craniectomy significantly improves CI.A widening bridge does not further improve lateral skullgrowth. Preoperative CI remains the most important deter-minant of the postoperative CI on the long term. Signs ofraised ICP (like a fontanel bulge and papilledema) occurmore frequently than expected after early extended stripcraniectomy. Fundoscopy needs to be performed routinelyuntil the age of 6 years to identify patients with possibleraised ICP and later in case of clinical suspicion. Surgeryshould aim to avoid raised ICP by adjusting the techniqueand timing. Early biparietal expansion seems to be insuffi-cient to achieve this aim.

Disclosure The authors declare that they have no conflict of interest.

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