Hiroshi Nishikawa

The increase of metopic synostosis: A pan-European observation

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, R2 = 0.578) as opposed to a nonsignificant increase in the percentage of sagittal synostosis (P > 0.05, R2 = 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.

Predictors of blood loss in fronto-orbital advancement and remodeling.

Fronto-orbital advancement and remodeling for craniosynostosis is extensive surgery and is associated with potential risks; the most significant of these is blood loss. We prospectively studied 116 consecutive patients undergoing fronto-orbital advancement by the same surgical team for a 5-year 6-month period to determine what factors are associated with blood loss and transfusion of blood products. The data collected on the calvarial sutures involved were whether the patient had a diagnosed syndrome, the age at operation, the length of the operation, the estimated blood volume lost during the perioperative course, the number of units of packed cells transfused (donor exposures), and the use of other blood products. The mean (SD) total blood volume lost was 116% (5.4) of the estimated preoperative volume. The median number of whole units of packed cells transfused was 2 units. Other blood products were given in 28% of the cases. There was significantly greater blood loss in those patients with recognized craniofacial syndromes, pansynostosis, an operating time longer than 5 hours, and an age of 18 months or younger at operation. The use of other blood products was associated with those patients losing a blood volume higher than the mean.

Venous malformations of the limbs: the Birmingham experience, comparisons and classification in children

The management of vascular anomalies in upper and lower limbs is complex. The current practice at Birmingham Childrens Hospital is based on a multidisciplinary approach, involving plastic surgeons, interventional radiologists, vascular surgeons, dermatologists and laser specialists. This study reviews the management strategies for peripheral venous malformations (VMs) and proposes a simple classification system to aid treatment. A retrospective review was undertaken involving all paediatric patients presenting with (VMs) of the upper and lower limbs, managed by the same multidisciplinary team over a period of 3 years. A total of 33 patients were identified, of whom 19 had lesions located in the upper limb. Treatment modalities included surgery, sclerotherapy, a combination of the two and conservative management. The indications for treatment included: (1) worsening pain, (2) increased swelling, (3) reduced function, (4) bleeding or ulceration and finally, (5) cosmetic deformity. Following treatment, outcome measures with regards to the symptoms were graded into (1) improved, (2) worsened and (3) unchanged. Based on magnetic resonance imaging, we were able to apply our classification to separate the lesions into Type 1a (superficial localised): nine, Type 1b (superficial diffused): five, Type 2 (Fascia/muscle infiltration): nine, Type 3 (Bone/joint infiltration): seven and Type 4 (Extensive whole-limb infiltration): three. In patients with upper limb VMs (n=19), eight lesions (42%) were superficial and localised (Type 1a) while the rest were diffused lesions. In contrast, in the lower limb (n=14), only one lesion (7%) was superficial while the rest were diffused lesions. Lower success rate for treatment was noted in lower limb malformations (p<0.05). In eight patients with recurrence of symptoms, six had Type 3 (intra-articular) lesions. There was one major and three minor complications following treatment. An outline of the management strategies for VMs in peripheral limbs is discussed in this article. An anatomical classification is described which aids in management and communication.

Is there a relationship between the severity of metopic synostosis and speech and language impairments

The occurrence of cognitive impairment and behavioral problems in patients with metopic synostosis has been described. The relationship between the severity of metopic synostosis and the incidence of speech and language delays has not been established. Twenty patients with nonsyndromic isolated metopic synostosis were evaluated. Five different preoperative measurements (metopic angle at the roof of the orbit, angle of lateral orbital wall at the sella, cranial indices, and distances between medial orbital walls and lateral orbital walls) were taken from computed tomography and compared with results from postoperative speech and language assessments. Frontal orbital advancement and remodeling were carried out by the same surgical team at a mean age of 1 year 4 months. Speech and language were assessed at 3 and 5 years. Six of the 20 patients had delayed speech and language developments. No consistent trend was observed linking the severity of frontal stenosis using the measured parameters with speech and language delays. Speech and language impairments in these patients cannot be explained by a physical concept, causing mechanical compression of the frontal lobes.

Percutaneous sclerotherapy of vascular malformations in children using sodium tetradecyl sulphate: The Birmingham experience

INTRODUCTION AND AIMS Sclerotherapy has become first line treatment for most venous malformations and some lymphatic malformations. We aimed to measure our sclerotherapy treatment success using 3% sodium tetradecyl sulphate (STD) and describe our experience. MATERIALS AND METHODS Retrospective five year review (Jan 04-09) of children with vascular malformations treated at our centre with 3% STD. Patients were classified using the Birmingham classification and treatment success was measured through case note review and pre- and post-treatment photographs. FINDINGS AND RESULTS: Forty-three (84.3%) of the 51 patients with vascular malformations (VM) who underwent sclerotherapy derived a benefit. Twelve patients (23.5%) had an excellent result, 31 (60.8%) were improved whilst eight (15.7%) were unchanged. Using Fishers exact test, there was a statistically significant difference in achieving complete resolution of superficial VMs compared to lesions involving the deeper layers of the head and neck. 17.6% of patients developed a complication with an overall complication rate of 12.2% per injection. There was one major complication with the remainder consisting of superficial skin necrosis that resolved conservatively. CONCLUSIONS Treatment with 3% STD sclerotherapy is effective in venous and some lymphatic vascular malformations. It should be considered an important treatment modality within a multi-disciplinary setting in these difficult problems.

Maxillary volume growth in childhood.

&NA; Nasomaxillary abnormalities in form, position, and development in children are often prominent features of craniosynostosis, and in particular, craniofacial dysostosis. While attempting to quantitatively assess the volumetric maxillary deficiency in these patients, it became apparent that there was no “normal” reference range for maxillary volumes throughout childhood that could be used for comparison. The aim of this study was to generate a model for measuring maxillary volume and subsequent changes throughout childhood. The technique of segmentation was applied to magnetic resonance images obtained in 55 healthy children (30 boys, 25 girls), aged 1 month to 184 months (15.33 years). Maxillary volumes were plotted against age for boys and girls to create a model for normal maxillary growth during the first 15 years of life. Maxillary volumes were larger in boys at all ages. However, the pattern of maxillary growth in boys and girls was similar and could be divided into three periods, each lasting approximately 5 years. During the first 5 years of life, there is a steady increase in maxillary volume, at the end of which the maxilla has reached 53 percent of the volume recorded at 15 years. There is an accelerated rate of growth between 5 and 11 years, which corresponds to the development and eruption of the permanent dentition. Thereafter, until the age of 15 years, the rate of growth of the maxilla plateaus. Maxillary volume in the first 12 months of life is, on average, 29 cm3 in boys and 25 cm3 in girls. By 15 years of age, it has increased to an average of 73.0 cm3 in boys and 59.4 cm3 in girls (an increase by a factor of 2.5 in boys and 2.4 in girls). The difference between the two sexes is statistically significant for the entire series (boys: mean maxillary volume = 56.55 cm3, SD = 24.61; girls: mean maxillary volume = 40.68, SD = 17.69, p = 0.009, one‐way analysis of variance). (Plast. Reconstr. Surg. 111: 1591, 2003.)

Changing referral patterns to a designated craniofacial centre over a four-year period.

Craniofacial conditions are mainly treated within England by four supra-regional centres. Due to a continuous increase in the number of cases referred to our service we audited the source and nature of these referrals. Data was prospectively collected over a four-year period from April 2004 to March 2008. The speciality of the referring clinicians was recorded, along with the diagnosis. A year-by-year increase in the number of referrals from 138 in 2004-2005 to 253 in 2007-2008 was seen. There was a 214% increase in the number of patients referred with single suture craniosynostosis, a 520% increase in patients with benign hyperplastic conditions such as fibrous dysplasia, neurofibromatosis and vascular anomalies and a 220% increase in patients treated elsewhere but now needing revision surgery. A 407% increase in referrals for positional plagiocephaly was recorded. Our referral pattern reflects the internationally accepted increase in the incidence of metopic synostosis and positional plagiocephaly. Due to the skill mix and experience present in a designated craniofacial service other benign hyperplastic and hypoplastic conditions are increasingly being referred. Additional referrals have come from a change in the referral pathway. To manage the increased workload we have established separate clinics to manage vascular anomalies and have adopted a policy of not reviewing patients with positional plagiocephaly.

Normal Fusion of the Metopic Suture

AbstractThere is some uncertainty as to when normal fusion of the metopic suture occurs. Existing studies have included relatively small numbers and have not used a statistical model to represent any variation in normality. In this study, a total of 337 head computed tomographic scans performed between 2006 and 2009 were retrospectively reviewed after strict exclusion criteria were met. Only patients aged younger than 18 months were included. Assessment was performed by analyzing axial slices of the bony window of the computed tomographic scan by 2 independent investigators. Two separate probit analyses were carried out to estimate the proportion of patients in whom the fusion process would have started and completed. Of 337 patients, 204 (60.5%) were male and 133 were female (39.5%). All patients older than 15 months and 23 days had completely fused metopic sutures. The estimated median age for the start of the fusion process was 4.96 months (95% confidence interval, 3.54–6.76 months), and the estimated median age for the completion of fusion was 8.24 months (95% confidence interval, 7.37–9.22 months). The fusion process completed between 2.05 and 14.43 months of age in 95% of the normal population. The difference between sexes was not significant. In conclusion, there was wide variation in the timing of normal fusion that can complete as early as 2 months of age.

Arteriovenous malformations of the head and neck: current concepts in management

Low-flow venous malformations are congenital lesions and they are the third most common vascular anomaly in the head and neck. In this paper, the third in a series of three educational reviews, we discuss current trends in their management, and include a summary of common sclerosant agents used in their control.

Role of parental risk factors in the aetiology of isolated non-syndromic metopic craniosynostosis

To try and identify potential parental risk factors for isolated non-syndromic metopic craniosynostosis, we did a telephone survey of parents of children who attended the craniofacial centre at Birmingham Childrens Hospital (BCH), UK, from 1995 to 2004. We calculated the prevalence of a number of potential risk factors and compared them with those of the general population. A total of 103 children with syndromic or non-syndromic isolated metopic craniosynostosis were seen, of which 81 (79%) had non-syndromic, isolated metopic craniosynostosis (M:F ratio 3:1). The response rate to the telephone survey was 72%. The prevalences of maternal epilepsy and the use of valproate, antenatal maternal complications (hypertension or pre-eclampsia, haemorrhage, and urinary tract infection), and fertility treatment in our sample were significantly higher than among the general population (p=0.01 or less in all cases). The likely roles of maternal epilepsy and taking valproate in the aetiology of isolated non-syndromic metopic craniosynostosis are further consolidated by our study. The finding that antenatal complications are possible risk factors for craniosynostosis has not to our knowledge been published previously.