Meulen, J.J.N.M. van der

Unilateral versus bilateral correction of unicoronal synostosis: An analysis of long-term results (Article)

2013-05-01T00:00:00Z

Introduction: Hollowing of the temporal region is a common problem after cranioplasty for unicoronal synostosis. In this study, first, the development of temporal hollowing pre- and postoperatively is evaluated. Second, the origin of temporal hollowing is investigated by comparing two operative techniques. Ultimately, the relation between timing of surgery and the development of hollowing is investigated. Methods: From 1979 to 2010, 194 patients with unicoronal synostosis were operated at our center. Patients were treated with a unilateral or bilateral correction of the supraorbital rim. A total of 48 patients qualified for the present study. Mean age at follow-up was 7.5 years. Cephalic landmarks were identified on radiographs prior to and after surgery to determine the growth of the forehead. For visual analysis, two independent observers evaluated normal photographs for the presence and severity of temporal hollowing. Results: Preoperative osseous asymmetry improved significantly after surgery. A total of 21 patients show an increase of temporal hollowing on photographs after surgery (46%). In 35 out of 48 patients, postoperative temporal hollowing was noted (73%). Bilaterally treated patients showed more severe temporal hollowing compared to unilaterally treated patients, though not significantly (23% vs. 6%, p = 0.229). Timing of surgery (before or after the age of 1 year) did not influence the occurrence of severe temporal hollowing. Conclusions: Fronto-supraorbital advancement was unable to achieve normal growth in the temporal region in a large proportion of patients, although more symmetry was achieved. The operative technique itself did not seem to influence the occurrence of temporal hollowing, nor did the timing of surgery.

The incidence of craniosynostosis in the Netherlands, 1997-2007 (Article)

2011-05-01T00:00:00Z

Objects: The first aim of this study was to determine the incidence of craniosynostosis, metopic synostosis and sagittal synostosis in the Netherlands from 1997 to 2007. The second aim was to study whether a shift in the proportion of different subtypes of craniosynostosis had taken place in the Netherlands during the study period. Methods: Patients of all six units performing craniofacial procedures in the Netherlands that met the inclusion criteria participated in the study (n = 927). A logistic regression in a weight sample was used to assess the differences in both the proportion and the incidence of different subtypes of craniosynostosis each year. The angle of the metopic ridge was compared using a regression analysis. Results: The incidence of craniosynostosis increased from 2.6 per 10 000 live births in 1997 to 6.4 in 2007. The incidence of metopic synostosis showed a significant increase of 6% each year (p = 0.029). We observed a significant change in the proportion of metopic synostosis, with a mean percentage of 20% during 1997-2000 and 27% during 2001-2007 (p = 0.046). The incidence of sagittal synostosis annually increased by 3% (p = 0.89). We did not find a significant change regarding the proportion of sagittal synostosis during 2000-2001. No significant difference in the severity of metopic synostosis was found between 1997-2000 and 2001-2004. Conclusion: The incidence of craniosynostosis, metopic synostosis and sagittal synostosis is 6.4; 1.9; and 2.8 per 10,000 live births, respectively. Both the incidence and the proportion of metopic synostosis have significantly increased over the study period, concluding that metopic synostosis is on the rise.

Long-term functional outcome in 167 patients with syndromic craniosynostosis; Defining a syndrome-specific risk profile (Article)

2010-10-01T00:00:00Z

Objective: Little is known about the long-term prevalence of elevated intracranial pressure (ICP), obstructive sleep apnoea (OSA), level of education, language and motor skills, impaired sight and hearing in craniosynostosis syndromes. The objective of this study was to define the prevalence per syndrome of elevated ICP, OSA, impaired sight and impaired hearing. Methods: A retrospective study was undertaken on 167 consecutive patients diagnosed with Apert, Crouzon, Pfeiffer, Muenke or Saethre-Chotzen syndrome, aged 1-25 years and treated between 1983 and 2008. The mean age at time of referral and review was 1 years and 2 months and 10 years and 3 months, respectively. Results: Patients with Apert and Crouzon/Pfeiffer syndromes had the highest prevalence of elevated ICP (33% and 53%, respectively) and OSA (31% and 27%, respectively), while Saethre-Chotzen syndrome was also associated with a fair risk for elevated ICP (21%). The prevalence of impaired sight (61%) and hearing (56%) was high in all syndromes. Conclusion: Based on these data, a syndrome-specific risk profile with suggestions for screening and treatment is presented.

Internal carotid dissection after Le Fort III distraction in Apert syndrome: A case report (Article)

2010-03-18T00:00:00Z

A 10-year-old girl with Apert syndrome underwent a Le Fort III osteotomy with the positioning of internal and external distraction devices. The operation was straightforward with no intraoperative complications. Very soon after completion of surgery an anisocoria (unilateral dilation of a pupil) was noticed. This was followed by intracranial oedema which was fatal. The aetiology was dissection of the right internal carotid artery is reported. The complications of Le Fort osteotomies are discussed regarding patients with complex syndromal craniosynostosis and midface hypoplasia, such as Apert syndrome.

Papilledema in isolated single-suture craniosynostosis: Prevalence and predictive factors (Article)

2010-01-01T00:00:00Z

The purpose of this retrospective study was to assess the prevalence of papilledema in patients with isolated craniosynostosis. Second, we wanted to assess if the presence of ventricular dilatation on computed tomography (CT) scan is a predictive factor for the occurrence of papilledema.We included 205 consecutive children with an isolated single-suture craniosynostosis, who had at least 1 fundus examination. Preoperative CT scans of the brain were examined for the presence of ventricular dilatation.Papilledema developed in 14 of 205 patients: 10 developed papilledema before surgery and 4 during the follow-up period. Ten of the patients with papilledema had a synostosis of the sagittal suture, and 4 of the metopic suture. Prevalence of papilledema in scaphocephaly was 9.7%, and in trigonocephaly, 5.6%. Based on evaluation of all CT scans, ventricular dilatation seemed not to be a predictive factor for papilledema in children with isolated craniosynostosis.The incidence of papilledema in almost 10% of scaphocephaly patients is remarkably higher than expected. Therefore, we recommend routine preoperative screening, especially for patients with scaphocephaly, but also for patients with trigonocephaly. Postoperative screening is recommended in all patients when there is any uncertainty.

On Trigonocephaly (Doctoral Thesis)

2009-12-08T00:00:00Z

The risk of psychopathology in children with craniosynostosis (Article)

2009-12-01T00:00:00Z

Background: The purpose of this study was to assess the prevalence of behavioral and emotional problems in patients with craniosynostosis and to determine the prospective association of a beaten-copper pattern before 18 months of age with behavioral and emotional problems in patients with craniosynostosis. Methods: The authors performed a follow-up study of 115 craniosynostosis patients at the Erasmus Children's University Hospital in Rotterdam. Behavioral and emotional problems were assessed with the Child Behavior Checklist at a mean age of 8 years. The presence of beaten-copper pattern before the age of 18 months was assessed on presurgical radiographs. Analyses were adjusted for intelligence quotient. Results: Whereas craniosynostosis patients with intelligence quotients of 85 or greater did not differ from children in the normal group, craniosynostosis patients with intelligence quotients less than 85 had a higher risk of behavioral and emotional problems. However, these results were comparable to the findings of other studies assessing psychopathology in children with lower intelligence levels. Type of craniosynostosis (single suture versus complex) and a beaten-copper pattern before the age of 18 months did not affect the risk for behavioral and emotional problems in children with craniosynostosis. Conclusion: When intelligence is taken into account, craniosynostosis is not associated with an increased risk of behavioral and emotional problems, nor is type of craniosynostosis or a beaten-copper pattern before the age of 18 months. Copyright

On the origin of bitemporal hollowing (Article)

2009-05-01T00:00:00Z

INTRODUCTION: Long-term results after cranioplasty for trigonocephaly often show bitemporal hollowing and a residual hypotelorism. Both findings fuel the perception that the growth of the periorbital region and the forehead as a whole continues to be restricted, even after correction. The aim of this study was to evaluate the growth process of the periorbital region after correction for trigonocephaly in the long term. MATERIALS AND METHODS: From 1972 to 2004, 184 patients underwent a cranioplasty for the correction of nonsyndromatic trigonocephaly. Cephalometric analysis was performed in 33 of these patients who had their radiographs taken on the same day as the photograph, at least 1 year postoperative and before the age of 6 years. Cephalic landmarks were used to analyze the growth of the forehead. Because of the lack of standardized cephalograms, growth ratios were used instead of absolute measurements. For visual analysis, normal anteroposterior photographs were used, which were taken on the same day as the radiograph. Two observers evaluated the anteroposterior photographs for the presence and level of temporal hollowing. A score of 0 (normal), 1 (moderate deformity), or 2 (severe deformity) was assigned to each of the photographs. RESULTS: A significant relation was found between a severe deformation seen at postoperative photographic evaluation and a lower growth ratio. The preoperative photo score was not of predicting value for the postoperative growth ratio and therefore, indirectly, for the postoperative photo score. The mean preoperative photo score dropped 5% after surgery. The age at operation had no influence on this postoperative photo score. The experience of the surgeon, however, was a significant contributing factor. CONCLUSIONS: Temporal hollowing seems to be of bony origin and can be explained by skeletal growth inhibition in the affected area. When present immediately after operation, they seem to persist through the years, which makes surgical skill another factor of importance.

The increase of metopic synostosis: A pan-European observation (Article)

2009-03-01T00:00:00Z

Metopic synostosis is thought to have an incidence of about 1 in 15,000 births. Traditionally, this makes it the third most frequent single-suture craniosynostosis, after scaphocephaly (1 in 4200-8500) and plagiocephaly (1 in 11,000). Our units have, independently from each other, noted a marked increase in the number of metopic synostosis over the recent years. This is a pan-European, retrospective epidemiological study on the number of cases with metopic synostosis born between January 1, 1997, and January 1, 2006. This number was compared to the prevalence of scaphocephaly, the most frequently seen craniosynostosis. In the 7 units, a total of 3240 craniosynostosis were seen from 1997 until 2006. Forty-one percent (n = 1344) of those were sagittal synostosis, and 23% (n = 756) were metopic synostosis. There was a significant increase of the absolute number as well as of the percentage of metopic synostosis over these years (regression analysis, P = 0.017, R = 0.578) as opposed to a nonsignificant increase in the percentage of sagittal synostosis (P > 0.05, R = 0.368). The most remarkable increase occurred around 2000-2001, with the average of metopics being 20.1% from 1997 to 2000 and 25.5% from 2001 to 2005 (independent t-test, P = 0.002). The sagittal synostosis showed a smaller and nonsignificant increase in the same years: from 39.9% in 1997-2000 leading up to 42.5% in 2001-2005 (independent t-test, P > 0.05). The number of metopic synostosis has significantly increased over the reviewed period in all of our units, both in absolute numbers as in comparison to the total number of craniosynostosis.

Unblocking the nose by unplugging the ear: The use of foam ear defenders as a splint following secondary cleft rhinoplasty (Article)

2009-01-01T00:00:00Z

Objective: The functional and aesthetic result from secondary cleft rhinoplasty is commonly impaired by a bulge in the lateral nasal vestibule, which can relapse in the postoperative period despite careful intraoperative correction. We aim to Improve our results by using a foam ear defender as a postoperative splint to prevent relapse. Design: Case series of 10 patients with photographs of a typical clinical case. Interventions: The postoperative nasal pack was exchanged with a foam ear defender on day 5. The splint was worn continuously for 3 to 4 weeks, then nightly for 3 months. The splint was changed daily by the patient. Main Outcome Measures: The results were assessed clinically by the senior author. Results: The patients had little or no recurrence of the lateral vestibular bulge following nasal splintage. Conclusions: Foam ear defenders are a simple, cheap, and comfortable method to provide splintage to the nasal vestibule following secondary cleft rhinoplasty.

The frontosphenoidal suture: Fetal development and phenotype of its synostosis (Article)

2008-04-01T00:00:00Z

Background: Isolated synostosis of the frontosphenoidal suture is very rare and difficult to diagnose. Little has been reported on the clinical presentation and fetal development of this suture. Objective: To understand the development of the frontosphenoidal suture and the outcome of its synostosis. Materials and methods: We studied the normal fetal development of the frontosphenoidal suture in dry human skulls and the clinical features of four patients with isolated synostosis of the frontosphenoidal suture. Results: The frontosphenoidal suture develops relatively late during the second trimester of pregnancy, which explains the mild phenotype when there is synostosis. This rare craniosynostosis results in a deformity that causes recession of the lateral part of the frontal bone and supraorbital rim, with minimal facial asymmetry. Three-dimensional CT is the best examination to confirm the diagnosis. Conclusion: Isolated frontosphenoidal synostosis should be considered in patients with unilateral flattening of the forehead at birth that does not improve within the first few months of life.

Early beaten-copper pattern: Its long-term effect on intelligence quotients in 95 children with craniosynostosis (Article)

2008-01-01T00:00:00Z

Object. The aim of this study was to analyze the presence of beaten-copper patterns (BCPs) in children with craniosynostosis before 18 months of age and its association with their IQ at a later age. Methods. The authors conducted a retrospective analysis of 538 cephalograms (obtained at a mean patient age of 1.16 years) from 95 patients. The BCP location and percentage of brain surface area covered were related to patient IQ scores obtained by the same psychologist using the Snijders-Oomen Nonverbal Intelligence Test-Revised, 51/2-17, and the Wechsler Preschool and Primary Scale of Intelligence-Revised. Results. As much as 71.6% of patients presented with a BCP before 18 months of age (mean surface area of BCP 20.3%, 93% of patients presented with bilateral BCPs). The mean IQ was 95 ± 21.3 (range 50-136) at a mean patient age of 8.4 ± 2.59 years. There was a significant increase in the surface area covered by BCPs in the first 3 years of life (p < 0.001) and a significant difference in IQs between syndromic (30 cases, mean IQ 88.9) and nonsyndromic craniosynostosis cases (54 cases, mean IQ 98.9, p = 0.03). No significant correlation was found between IQ and the appearance of BCPs on presurgery radiographs (Pearson correlation coefficient = 0.143, p = 0.19) or their location (Spearman rank correlation coefficient = 0.091, p = 0.45). The BCPs appeared predominantly in the occipital region (41.1%). Conclusions. Although the radiographic appearance of a BCP before the age of 18 months is an uncommon finding in healthy children, a craniosynostosis study group showed a preoperative BCP incidence of 71.6% and an increased incidence during the period of rapid brain expansion in the first 3 years of life. Note, however, that the presence of such a pattern had no significant long-term effect on patient intelligence levels.