Three-dimensional computed tomographic evaluation of Le Fort III distraction osteogenesis with an external device in syndromic craniosynostosis.

There is little anteroposterior growth of the midface in patients with syndromic craniosynostosis who are followed up over time without intervention. A Le Fort III with distraction osteogenesis can be done to correct this. This is a controlled way in which to achieve appreciable stable advancement of the midface without the need for bone grafting, but the vector of the movement is not always predictable. The purpose of this study was to evaluate the 3-dimensional effect of Le Fort III distraction osteogenesis with an external frame. Ten patients (aged 7-19 years) who had the procedure were included in the study. The le Fort III procedure and the placement of the external frame were followed by an activation period and then a 3-month retention period. Computed tomographic (CT) images taken before and after operation were converted and loaded into 3-dimensional image rendering software and compared with the aid of a paired sample t test and a colour-coded qualitative analysis. Comparison of the CT data before and after distraction indicated that the amount of midface advancement was significant. Le Fort III distraction osteogenesis is an effective way to advance the midface. However, the movement during osteogenesis is not always exactly in the intended direction, and a secondary operation is often necessary. Three-dimensional evaluation over a longer period of time is necessary.

The incidence of craniosynostosis in the Netherlands, 1997-2007.

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.

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.

[Traumatic atlanto-occipital dislocation with minimal neurologic deficits]. / Traumatische atlanto-occipitale dislocatie met minimale neurologische uitval.

A 21-year-old woman presented with mild neck pain following a scooter accident. Conventional cervical radiographs showed a lateralisation of the dens axis to the left in relation to the foramen magnum. A subsequent CT with a multiplanar reconstruction revealed an atlanto-occipital dislocation that was repositioned under fluoroscopic control. The patient had an uneventful recovery; a mild loss of strength in the left arm remained. The atlanto-occipital dislocation without neurological deficit is rare.

Evaluation of five different incisions for correction of radial dysplasia.

The complications of the five different incisions used for centralization from 1970 to 1996 were evaluated. In five cases access was by an S- shaped incision, in one with a transposition flap. Sixteen centralizations were done using a radial Z-plasty in combination with an ulnar excision and four by using two opposite Z- plasties, preserving wrist mobility as much as possible. In 1995 and 1996 a bilobed flap was used in seven cases. No complications occurred with the primary procedures prior to 1995. In all seven cases where the bilobed flap was used venous congestion was seen. In four out of these seven superficial necrosis of parts of the flaps occurred. In all cases though wound healing occurred spontaneously by delayed primary healing after the standard 8 weeks of cast immobilization.