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During spring-assisted cranioplasty, the spring transmits forces through adjacent cranium. We have previously demonstrated that the ectocranial-endocranial thickness of cranial sutures increases significantly over time in the presence of continuous spring forces. We wished to investigate if cranial bone showed similar adaptational responses.New Zealand white rabbits were randomized into a treatment group [a spring was placed across a posterior frontal suture (PFS) suturectomy and a control group (PFS suturectomy)]. Animals (n = 6) were euthanized from each group at 4, 7, and 10 weeks, respectively. A sham group (n = 6) was euthanized at 10 weeks. Frontal bone thickness was recorded at five reproducible anatomical points on the frontal bone. Histological analysis of the bone architecture was performed.Frontal bone thickness was significantly greater than controls at all five sites at weeks 7 and 10. There were multiple significant differences between the 4-, 7-, and 10-week groups with each site progressively thickening over time. Histological analysis revealed a uniform increase in thickness of the endocranial and ectocranial cortical bone in the treatment groups.Cranial bone adapts to the presence of continuous spring cranioplasty forces by progressively thickening over time. This property is beneficial in craniosynostosis cases with very thin and poor quality bone and may partly explain the observed lack of spring erosion through bone.

BACKGROUND: Expansile cranial springs are used to treat selected cases of sagittal synostosis. After sagittal suturectomy, springs are placed perpendicular to the line of the synostosis. Normal growth across the coronal suture is approximately perpendicular to the suture line. To allow cranial expansion in the parietal direction, it is hypothesized that the normal growth vectors across the coronal sutures are altered. METHODS: Ten New Zealand White rabbits underwent suturectomy of the midline posterior frontal suture followed by insertion of an expansile spring. Radiologic markers were placed on either side of the normal coronal sutures (n = 20) perpendicular to the released suture. Serial radiology was performed up to 7 weeks. Growth vectors across the coronal suture were compared with those of 10 control rabbits. RESULTS: Dorsal cranial expansion was predominantly in an anterolateral direction in controls. The normal growth vector across the coronal suture occurred at an angle of -1 degree to the midline. A spring altered this growth vector to 63 degrees. Over 7 weeks, the cranial width increased 5.1 mm in rabbits with springs compared with 0.2 mm in the control group (p < 0.01). The increase in cranial length across the coronal suture was 1.82 mm in controls and 0.93 mm in rabbits with springs (p = 0.019). CONCLUSIONS: Application of a spring across a midline cranial suture alters the growth vector of the normal adjacent sutures. The spring greatly increases cranial width at the expense of some of the normal increase in cranial length over this period. This would be beneficial in correcting the cranial index in sagittal synostosis.

Metopic synostosis, apart from the pointed forehead, typically is characterized by hypotelorism with egg-shaped orbits on cephalography and the frontoorbital axis parallel or even converging superiorly. The frontoorbital axis angle is a novel parameter for analyzing and describing the orientation of the orbits. Current methods of surgery often result in undercorrection of the almost ever-present hypotelorism. The present study was performed to analyze a new technique, capable in this respect, using steel wire springs in conjunction with a cranioplasty.A retrospective study of 23 metopic synostosis patients operated on between 1999 and 2004 was conducted. A strip midline craniectomy and frontal reshaping were combined with the insertion of a steel wire spring across the midline craniectomy, forcing lateral displacement of the orbits. Preoperative and postoperative follow-up cephalograms were obtained, and the bony medial interorbital distance was measured and compared with the bony medial interorbital distance of a control group. Perioperative data and complications were noted.Preoperative mean bony interorbital distance was 10.6 mm (range, 7.7 to 13.2 mm). It increased to 15.7 mm (range, 10.4 to 22 mm) at 1.5 months postoperatively and to 16.2 mm (range, 10.9 to 24.5 mm) 5 months postoperatively. Results as judged clinically ranged from little effect to a definitive overcorrection. The frontoorbital axis was improved in every case. Average frontoorbital axis was -4 degrees (range, -33 to 23 degrees) preoperatively and 28 degrees (range, 11 to 46 degrees) postoperatively.It was concluded that a spring used together with a cranioplasty is a powerful tool for the correction of both hypotelorism and orbital shape in trigonocephaly.

The Saethre-Chotzen syndrome is an autosomal, dominantly inherited craniosynostosis caused by mutations in the basic helix-loop-helix transcription factor gene TWIST1. This syndrome has hitherto not been associated with an increased risk of cancer. However, recent studies, using a murine breast tumor model, have shown that Twist may act as a key regulator of metastasis and that the gene is overexpressed in subsets of sporadic human breast cancers. Here, we report a novel association between the Saethre-Chotzen syndrome and breast cancer. In 15 Swedish Saethre-Chotzen families, 15 of 29 (52%) women carriers over the age of 25 had developed breast cancer. At least four patients developed breast cancer before 40 years of age, and five between 40 and 50 years of age. The observed cases with breast cancer (n = 15) are significantly higher than expected (n = 0.89), which gives a standardized incidence ratio (SIR) of 16.80 (95% CI 1.54–32.06). Our finding of a high frequency of breast cancer in women with the Saethre-Chotzen syndrome identifies breast cancer as an important and previously unrecognized symptom characteristic of this syndrome. The results strongly suggest that women carriers of this syndrome would benefit from genetic counseling and enrolment in surveillance programs including yearly mammography. Our results also indicate that the TWIST1 gene may be a novel breast cancer susceptibility gene. Additional studies are, however, necessary to reveal the mechanism by which TWIST1 may predispose to early onset breast cancer in Saethre-Chotzen patients. © 2007 Wiley-Liss, Inc.

In the 10-year period 1986-1996, 85 patients were admitted to our unit with craniofacial injuries, 56 of whom had orbital fractures. These were studied with respect to the type of injury, type and location of fracture, presence of ocular and intracranial injury, and associated injuries to the head and body, as well as operative techniques used. Both the patient's and the surgeon's opinion on the aesthetic result were noted. The patients were also given a questionnaire about their quality of life after the injury. Road traffic crashes accounted for 31 (55%) of the injuries, falls for 9 (15%), and horse-riding for 6 (11%). The Injury Severity Score (ISS) ranged from 4 to 41 (mean 18). Twelve also had eye injuries, which resulted in complete blindness in one eye in 4 (7%). Thirty patients had 41 neurological injuries (54%), frontal contusions being the most frequent diagnosis (n = 15). Exact repositioning with rigid fixation included bone grafting to the orbit in 11 patients, and the dominating bone graft was split calvarium (n = 5). Forty-two patients completed a questionnaire, 26 of whom (64%) had no aesthetic complaints. Seven of the 42 were too disabled to work one year after the injury. Re-exploration was infrequent and the aesthetic outcome, both in the surgeon's and the patient's opinion, was good. However, the older the patient, the worse the outcome.

The aim of the present study was to evaluate the possibility of mobilizing calvarial bone with a fully implantable and bioresorbable device. The animal model used was the New Zealand white rabbit (n = 12). An island bone flap attached to the dura mater was created in the parietal region and amalgam markers were placed in this bone flap and in the ipsilateral frontal bone. In one group of six rabbits (group 1), a specially processed contractile 70L/ 30D,L polylactic acid plate, 15 X 6 X 0.6 mm, was attached to the island flap by one extremity, and to the fixed ipsilateral frontal bone by the other. In group 2 (control), no plate was added. Bone marker movement was followed with serial radiography. In group 1, there was a progressive reduction in mean marker distance over the first 48 hours, and stability thereafter. In group 2 (control), mean marker distance remained stable until the second postoperative week, after which time there was a slight increase until the end of the experimental period. At 4 weeks, the mean marker separation differed significantly between group 1 (mean, -3.62 mm; SD, 0.79 mm) and group 2 (mean, 0.34 mm; SD, 0.14 mm; p

Object. Brachycephaly is a characteristic feature of Apert syndrome. Traditional techniques of cranioplasty often fail to produce an acceptable morphological outcome in patients with this condition. In 1996 a new surgical procedure called “dynamic cranioplasty for brachycephaly” (DCB) was reported. The purpose of the present study was to analyze perioperative data and morphological long-term results in patients with the cranial vault deformity of Apert syndrome who were treated with DCB. Methods. Twelve patients have undergone surgery performed using this technique since its introduction in 1991 (mean duration of follow-up review 60.2 months). Eleven patients had bicoronal synostosis and one had a combined bicoronal—bilambdoid synostosis. Perioperative data and long-term evolution of skull shape visualized on serial cephalometric radiographs were analyzed and compared with normative data. Changes in mean skull proportions were evaluated using a two-tailed paired-samples t-test, with differences being considered significant for probability values less than 0.01. The mean operative blood transfusion was 136% of estimated red cell mass (ERCM) and the mean postoperative transfusion was 48% of ERCM. The mean operative time was 218 minutes. The duration of stay in the intensive care unit averaged 1.7 days and the mean hospital stay was 11.8 days. There were no incidences of mortality and few complications. An improvement in skull shape was achieved in all cases, with a change in the mean cephalic index from a preoperative value of 90 to a postoperative value of 78 (p = 0.000254). Conclusions. Dynamic cranioplasty for brachycephaly is a safe procedure, yielding high-quality morphological results in the treatment of brachycephaly in patients with Apert syndrome.

A prospective developmental assessment was performed on 26 patients operated on with dynamic cranioplasty for sagittal synostosis. Because this technique entails the application of compressive force, it was of great concern to assess the effect of surgery on development and mental status. The surgical technique used was a modified pi procedure. Perioperative variables were recorded. Six patients underwent preoperative intracranial pressure (ICP) measurements. To evaluate objectively the developmental outcome, the Griffiths' Mental Development Scales was used for analysis before and after surgery. A parental questionnaire was used for subjective outcome measurement. Preoperative ICP recordings during sleep ranged from 12.8 to 22.8 mmHg (mean, 16.1 mmHg). The mean age at the time for surgery was 6.9 months (range, 4-16 months; standard deviation [SD], 2.32 months). The surgical technique included shortening of the anteroposterior diameter of the skull by a mean of 16.6 mm. The mean global development quotient (GDQ) preoperatively was 104.5 (range, 82-144; SD, 12.4) and the mean GDQ postoperatively was 101.4 (range, 62-129; SD, 13.6). Mean age at follow-up was 16.3 months (range, 9-40 months; SD, 4.04 months). There was no significant correlation between the amount of intraoperative shortening and mental development. In comparison of means, the GDQ preoperatively did not differ significantly from the GDQ postoperatively. The modified pi procedure is safe and efficient. When surgery was performed before 1 year of age, no significant (p = 0.33) effect on mental development-either detrimental or beneficial-was demonstrated.

We followed up 10 patients whose non-syndromal bicoronal synostosis had been operated on with a dynamic cranioplasty technique developed by this craniofacial unit in 1992. With this technique, the growth of the brain is redirected in an anteroposterior direction as wire-mediated compression and restraint are exerted on the transverse and vertical dimensions of the skull. The mean operating time was 160 minutes (range 120-275) and mean stay in the intensive care unit was 36 hours (range 23-58). There was no operative mortality and few complications. The surgical results were assessed objectively by analysis of cephalometric tracings. The mean (SD) cephalic index was 87.6 (4.9) preoperatively and 77.7 (1.8) postoperatively (p = 0.001). The modified Whitaker scale was used as a subjective outcome measurement, and nine patients were classified as Whitaker grade 1 (no additional surgery). One patient required additional intracranial surgery. A questionnaire was sent to all families to obtain an additional subjective measurement of outcome. Parents' satisfaction was high. We conclude that dynamic cranioplasty is a safe and efficient operation for treatment of brachycephaly.

With the inception of craniofacial surgery elaborate surgical procedures were introduced for the treatment of craniosynostosis. Recently the use of implantable springs to aid simple strip craniectomies for these conditions has been described. Having shown the feasibility of using springs for dynamic skull reshaping, several questions remain to be answered, all of them about how to control the spring action clinically. One of the most important questions concerns force. The purpose of this study was to evaluate experimentally the effect of two springs of different strengths on the growth of rabbit calvaria after strip craniectomy. Thirty-two 6-week-old rabbits were randomised into one of four groups: sham-operation where only amalgam markers were inserted after subperiostal dissection; strip craniectomy of the sagittal suture, no expansion; strip craniectomy of the sagittal suture and insertion of an expander element made of titanium molybdenum alloy (TMA); and strip craniectomy sagittal suture and insertion of an expander element made of stainless steel (SS). SS springs delivered a range of forces from 2.42-2.18 N, whereas the TMA expander elements delivered a range from 1.39-1.09 N. The parietal bone marker separation after 12 weeks was 4.9 mm in the TMA group and 7.4 mm in the SS group. This resulted in a significant increase of the calvarian height compared with control groups. Histological examination showed intramembranous bone formation in the bone gap in all groups.

Clinically diagnosed trigonocephaly have been treated by a standard surgical technique developed at Göteborg University Craniofacial Unit. To evaluate the technique we designed a study to include both subjective and objective assessments. Of 30 patients operated on between 1988-1997, 15 patients with at least three years postoperative follow-up have been included. An evaluation of the surgical outcome of the bitemporal width, the forehead contour, and the intercanthal distance was made both subjectively using a visual analogue scale (VAS) and objectively with analyses of cephalograms taken preoperatively and postoperatively at 3 and 5 years of age. For the subjective evaluation a control group of 10 randomly selected children from a Child Welfare Centre were selected. For the objective evaluation the control group consisted of cephalograms from children born with cleft lip and palate who were matched regarding sex and age. Both cephalometric analysis and subjective studies of the outcome indicated improvement. When the forehead contour was investigated the number of patients who had been improved or corrected completely was higher when evaluated subjectively. When interorbital distance on cephalograms and intercanthal distance scores on VAS were evaluated, again the subjective study indicated improvement in more subjects.

We have developed a new technique for reconstruction of full thickness eyelid defects. A composite three-layer skin-cartilage-mucosal unit is manufactured and tailored three-dimensionally. Use of this sandwich graft in our patients proved to be simple and safe for eyelid reconstruction and has given good results without complications in four patients.

1976, P. 569-580). Both syndromes follow an autosomal dominant mode of transmission. Most cases of Crouzon’s syndrome are familial, whereas the majority of cases with Apert’s syndrome are sporadic cases representing new mutations. The development of advanced and refined meth- ods for craniofacial surgery has renewed interest in patients with cranial and craniofacial malforma- tions. New surgical methods have made it possible to improve both function and cosmetic appearance in patients with severe malformations such as those seen in Crouzon’s and Apert’s syndromes. This sur- gery is very specialized and is only performed in a few centres in the world. One such centre is locali- zed in Goteborg, Sweden. The patients treated here come from the whole of Scandinavia. This centralization gives the unique opportunity to per- form large-scale studies on different pathogenetic aspects of craniosynostosis. Very few chromosome studies by banding technique on patients with cra- niosynostosis syndromes have been reported. In a survey of six patients with chromosome 7 short- arm-terminal deletions involving the 7p2 region, MCPHERSON et al. (1976) and DHADIAL and SMITH (1979) found five patients with craniosynostosis. These findings led them to suggest the existence of a 7p- syndrome typical of craniosynostosis. They also proposed that the amount of missing material from chromosome 7 was related to the variability in the phenotypic expression. This hypothesis will be tested in the present report, which deals with

A new technique of using implantable springs as an adjunct after corrective surgery for craniofacial malformations is presented. A 6-month-old boy with multiple premature craniosynostoses and extreme turricephaly underwent surgery of limited extensiveness but supplemented with a set of indwelling springs for gradual postoperative skull reshaping. At spring removal three months later the skull was normalised both clinically and on cephalogram. A 5-year-old boy with Apert syndrome, severe midface retrusion, exorbitism, and sleep apnoea underwent a monobloc full face disjunction without repositioning, but was fitted with two springs for postoperative facial advancement. Three months postoperatively cephalometric analysis revealed 14 mm advancement at incisor level and at least 16 mm in the frontal region. There was no more exorbitism or clinically noticeable midface retrusion. Sleep studies revealed that the sleep apnoea was significantly improved, meaning complete cure except when sleeping flat on the back. It was concluded from these first clinical applications of spring assisted craniofacial distraction that springs hold significant promise for the future in many respects.

In craniofacial surgery, the most common techniques for treatment of brachycephaly have been either to let the forehead float on the brain or to fix it in an advanced position. Since neither of these techniques renders acceptable results with enough consistency, we have developed a different way of addressing the problem. In principle, the design of the operation is to restrict upward and transverse growth of the cranium but to allow anterior and posterior expansion. This is accomplished by producing transverse tension across the skull and letting it expand anteriorly by means of a superiorly hinged fronto-orbital flap and posteriorly by an inferiorly based occipital flap. To prevent upward expansion at the squamosal sutures when still open, these junctions are bridged with miniplates. This surgical technique has brought definite improvement to the results even in some Apert syndrome children. During a 2-year period, we have treated 14 infants with this technique and followed 10 of them with roentgencephalometry, 3 for more than 1 year, and 4 for more than 6 months. The diagnoses were the following : nonsyndromal bicoronal synostosis (4), Apert (7), bicoronal synostosis with midline cleft, Saethre-Chotzen, and Antley-Bixler (1 each). The mean age of surgery was 6.6 months (range 3 to 16 months). There were no major complications.

The main objective of surgical treatment of scaphocephaly is to improve the shape of the patient's skull. Since 1989 we have used a modified pi-plasty method, and the aim of this report was to study how effective surgery had been. A sample of 25 patients was investigated. All patients were operated on during their first year of life (mean: 6 months). They had roentgencephalometric records preoperatively as well as at a follow-up at age 3 years. Certain measurements were taken from the cephalograms and compared with normative data. It was found that the average skull length had decreased from a preoperative 114% of normal to 107% at follow-up. The skull width had improved from 92% to 98% of normal. The cephalic index had improved as well but was still not within the normal range. The cephalograms were also classified according to the occurrence of digital markings. Before surgery at 6 months none had these signs, but at follow-up every second patient displayed evolution of digital markings. It was concluded that the modified pi-plasty method improves skull morphology considerably but does not completely normalize the shape of the average patient's skull. The cranial length, in particular, needs further improvement.

Introduction Spring cranioplasty is used in selected cases of craniosynostosis. A rabbit model was used to determine (1) if cranial biomechanics modify the expected rate of spring expansion, (2) the residual spring force in situ after cranial expansion, and (3) if the spring weakens during clinical use. Materials and methods Twenty-seven New Zealand white rabbits were divided into 3 groups: the treatment group that underwent posterior frontal suturectomy and spring expansion (n = 15) and the control (n = 6) and sham groups (n = 6) that underwent suturectomy and incision only, respectively. Cephalography measured cranial expansion for 7 weeks. Spring force-deflection curves were measured in a dynamometer before and after use. Results Significant cranial expansion was observed for 8 hours (15% of the total expansion). The rate of expansion decreased significantly between 48 and 96 hours followed by a second period of rapid expansion from 96 hours to 7 days (P = 0.001). Approximately 80% of expansion was achieved by 2 weeks and 90% by 3 weeks. Initial spring force was 9.4 N (range, 7.2-10.7). Once the spring had achieved 90% of its eventual expansion, it retained 40% of its original force. At the completion of cranial expansion, the residual spring force was 2.6 N (range, 1.4-4.0) or 28% of the original spring force. All springs maintained identical load-compression curves after use. Conclusions The craniofacial structures are mechanically complex and modify the uniform expansion curve expected as the spring force decays. Significant force is maintained within the spring at the completion of cranial expansion. This may have implications for possible relapse if the springs are removed early.

Spring-assisted cranioplasty transmits forces throughout the craniofacial complex that can be measured as strain. The strain magnitude in relation to normal background physiologic strains and the distribution of these strains are currently unknown.Twenty New Zealand White rabbits were randomized into two groups: the treatment group, which included posterofrontal suture removal and spring insertion (n = 10); and the control group, which consisted of posterofrontal suture removal (n = 10). Strain gauges were placed across the interfrontal suture, both coronal sutures, and the frontal bone. Continuous strain recordings were made for 15 minutes after spring insertion.Physiologic strains caused by dural pulsation and intracerebral swelling measured were higher within sutures (40 to 50 microepsilon) than in bone (10 to 20 microepsilon). Spring activation produced large increases in strain across all sutures and bone. Compared with controls, tensile strains were 20 times higher in the frontal bone (mean, 341 microepsilon; p = 0.000), 15 times higher in the interfrontal suture (539 microepsilon; p = 0.000), and 21 times higher in the coronal suture (700 microepsilon; p = 0.000). Compressive strain in the left coronal suture confirmed a shear force at these sutures (-503 microepsilon; p = 0.000). The variability of background physiologic strain was not dampened by spring loading.Spring-assisted cranioplasty produces supraphysiologic strain in adjacent cranial bone and suture tissue. Mechanotransduction converts these forces into the biological processes that modulate calvarial morphology. Contrary to expectations, bone lateral to the spring insertion is subjected to tensile strain on its ectocranial surface. A compressive strain on its endocranial surface is likely because of bone flexion. This has implications for subsequent calvarial morphology.

Background: The mechanical properties of the pediatric craniofacial complex allow dissipation of spring cranioplasty forces. Springs do not fully expand in situ and continue to transmit a continuous force until removal. The authors wished to investigate whether ongoing forces altered the biomechanical characteristics of cranial sutures. Methods: Thirty New Zealand White rabbits were divided into five groups: spring expansion for 4, 7, and 10 weeks; early spring removal at 4 weeks followed by monitoring for 3 weeks; and a control group (n = 6 each). Cranial expansion was monitored using cephalometry. The left coronal suture then underwent load-displacement testing in a dynamometer. Results: Relapse of cranial expansion was observed following early spring removal (mean, 6 percent; p = 0.017). Cranial suture thickness was significantly correlated to the length of spring insertion. Load displacement curves of sutures in all groups initially exhibited classic viscoelastic behavior. The treatment group developed intrasutural weakening before failure that was not observed in controls. The peak load before failure as a percentage of that observed in controls was 31 percent in the 4-week group (p = 0.001), 35 percent in the 7-week group (p = 0.000), and 45 percent in the 10-week group (p = 0.023). Conclusions: Cranial suture compliance is modified in the presence of continuous spring cranioplasty forces. Thickening of the coronal sutures, which have been expanded in a shear-like manner, increases their three-dimensional surface area and may contribute to the relative lack of relapse observed after early spring removal.

Spring-assisted cranioplasty has been demonstrated to correct hypotelorism associated with metopic synostosis. In addition, the fronto-orbital axis rotates toward a more normal orientation. We postulated that spring-induced shear forces and subsequent displacement across the frontobasal sutures are the primary initial mechanisms for change in cranio-orbital morphology.Sixteen consecutive patients (32 orbits) with trigonocephaly operated on between 1999 and 2004 were studied retrospectively. After frontal remodeling, a spring was placed across the released metopic suture. Preoperative and 6-week postoperative cephalograms were used to measure the relative translation of the medial orbital wall from the midline at fixed vertical distances above and below the frontoethmoidal suture (FES). The vertical height of the spring above the frontonasal suture and the patient's age were analyzed with respect to the increase in bony interorbital distance (BIOD).There were significant increases in movement of the medial orbital wall above the FES at each measurement point (P0.001). There was no significant postoperative movement of the medial orbital wall below the FES at any measurement point. The relative movements above the suture suggest a degree of plastic deformation adjacent to the suture.There was no significant correlation between the age of patient or the height of the spring above the frontonasal suture and the increase in BIOD.Tensile spring forces are transmitted directly to the frontoethmoid suture. Most initial increases in BIOD and altered intraorbital morphology are due to distraction of cranial base sutures rather than plastic deformation.

UNLABELLED: Expansile cranial springs are used in selected cases of craniosynostosis. The spring exerts moderate force against the relatively thin skull. We investigated whether the spring erodes through the bone and the clinical significance of any erosion relative to the cranial expansion achieved. METHODS: New Zealand white rabbits (n = 10) underwent sagittal suturectomy and spring insertion. Amalgam markers were placed perpendicular to the expected direction of suture erosion. A control group (n = 10) underwent suturectomy. Radiological evaluation was performed for 7 weeks to check for migration of the spring foot. RESULTS: The mean cranial thickness was 1.4 mm at the site of spring insertion. This compared with 1.8 mm in 7 children undergoing spring cranioplasty. The mean spring force was 9.4 N. In sagittal synostosis, the mean spring force used is 7 to 10 N.The cranial width increased 11.02 mm in the spring treatment group compared with 0.23 mm in the control group (P < 0.001). Spring erosion occurred in 4 (20%) of the 20 spring ends. Mean spring erosion for the treatment group was 0.18 mm. This was 3.2% of the mean increase in cranial width. The maximum percentage spring erosion versus cranial expansion in an individual rabbit was 14.17%. There was no statistical difference in cranial expansion between the rabbits in whom bone erosion did and did not occur. CONCLUSIONS: The degree of spring force required to effectively expand the cranium may cause bone erosion in some individuals. This degree of spring erosion was of minimal clinical significance in this animal model.

Ventricular shunt overdrainage may cause cranial vault collapse, deformation, and secondary craniosynostosis. There is a paucity of information in the literature about this condition or successful low morbidity methods to reshape the cranial vault. Spring-assisted cranioplasty was developed in the Gothenborg Craniofacial Unit and is part of our routine protocol for the treatment of selected patients with craniosynostosis. The same treatment principles were applied to skull deformity secondary to ventricular shunt-associated deformity. Four patients with scaphocephaly secondary to ventricular shunting were treated using expansile springs. Three patients had a secondary sagittal synostosis requiring osteotomy. A further patients' sagittal suture was still patent, and this was expanded without osteotomy. Near normalization of the cranial index and significant aesthetic improvement of the cranial vault were noted in all cases. The mean preoperative cranial index increased from 0.60 to 0.71 within 4 months after surgery. The limited dissection and short operative time enabled minimal morbidity and blood loss compared with that expected with traditional craniofacial reshaping techniques. Patients with ventricular shunt-associated cranial deformity often have associated medical problems. Cranial bone is often in short supply and its quality less than optimal. In this situation, spring-assisted surgery is straightforward and offers significant advantages over traditional craniofacial reshaping techniques.

Facial deformity in Crouzon syndrome is characterized by exophthalmos, exorbitism, mild hypertelorism, and maxillary hypoplasia with a Class III occlusion. The Göteborg craniofacial unit corrects this deformity in selected patients by the 2-stage procedure of "spectaclesplasty" followed by a Le Fort I maxillary osteotomy at skeletal maturity. "Spectacles" is a reference to the bilateral circumferential periorbital bony skeleton, and a spectaclesplasty is a differential rotation and advancement of this complex. Spectaclesplasty has been integrated as part of our protocol in managing Crouzon syndrome from birth to maturity. All patients who have undergone spectaclesplasty were retrospectively reviewed. A composite scoring system analyzing periorbital anatomy and aesthetics was used to compare the preoperative and most recent postoperative photographs. Complications and the need for ancillary procedures were recorded. Twenty-one patients have undergone spectaclesplasty since this technique was introduced in 1984. Mean follow-up time is 5 years 10 months. Compared with their preoperative aesthetics, the improvement was rated as excellent for 8 patients (38%), very good for 7 patients (33%), good for 5 patients (24%), and minimal for 1 patient (5%). No patient was rated as having no improvement. Mean perioperative blood loss was 111% of estimated red cell mass. Mean operative time was 6.4 hours. The mean duration of stay in the intensive care unit was 28 hours, and the mean hospital stay was 11 days. There were few complications and no incidences of mortality. Spectaclesplasty yields high-quality aesthetic results in most cases. It is our impression that spectaclesplasty en bloc rotation advancement of the periorbital bony skeleton can be safely performed before skeletal maturity of the lower face. Correction of the bony periorbital anatomy in early adolescence is important in alleviating psychosocial distress in this age group. In our hands, spectaclesplasty produces a more normal anatomic position of the periorbital soft tissues facilitating both function and aesthetics.

The use of springs in craniofacial surgery originated at Sahlgrenska University Hospital in 1997 as a way of remodeling the cranial vault postoperatively.The hospital records of the first 100 operations involving spring placement were analyzed retrospectively. Demographic, perioperative, and postoperative data were recorded.Two hundred forty-six springs were used in 96 patients. Results for sagittal, metopic, bicoronal, multiple synostoses, and midface surgery are presented. In total, five patients (5 percent) required further surgery because of undercorrection. There were no major complications. Spring dislodgement (5 percent) was the most common complication in early cases. Raised intracranial pressure resulted in a protocol change with the use of compressive springs. The data compare favorably with those of standard craniofacial procedures performed in the same unit.This therapeutic modality in craniofacial surgery has allowed minimization of the extent of surgery without compromising clinical outcomes. Springs have now become part of the authors' treatment protocol for craniosynostosis and midface surgery. The authors have shown the use of these techniques to be safe and, in selected situations, to offer significant advantages over other methods of treatment.

Spring-assisted cranioplasty (SAS) has been used for the treatment of selected cases of sagittal synostosis at our unit routinely since 1998. In order to assess the long-term outcomes of this procedure, we compared the clinical data and morbidity with the pi-plasty technique, our previous standard procedure for the treatment of such children. The first 20 consecutive patients who underwent SAS for isolated sagittal synostosis with complete records, and who were 3 years old at the time of this study, were included. Twenty patients with a pi-plasty performed in the period immediately preceding the spring group acted as a control group. Cephalograms (preoperative, 1-year and 3-year), clinical examination, medical record data, medical photography, and a questionnaire (spring-group only) were used to evaluate and compare these two groups. The mean age of the spring group was 3.5 months (2.5-5.5) and the pi-plasty group 7.1 months (4-15.5) of age at surgery. There were no deaths in either group. There was a higher rate of complications in the pi-plasty group. The skull morphology was similar preoperatively in both groups but slightly different at the 3-year follow-up. The mean cephalic index (CI) in the spring group was 72 at 1 year of age and 71 at 3 years of age, indicating a minor relapse. The pi-plasty group had a mean CI of 73 at 3 years of age. The length was the same in both groups however the pi-plasty group had a lower height (mean 2 mm) and wider biparietal distance (mean 5 mm). All parents of the spring group were highly satisfied with the aesthetic results achieved, would undergo the operation again, and would recommend it to others with scaphocephaly. It was concluded that the two groups of surgery resulted in a quite similar morphologic outcome. The pi-plasty group had a cephalic index marginally closer to the normal range at 3 years of age. The spring group was superior with respect to blood loss, transfusion requirements, operative time, ICU time, recovery time, and total hospital stay.

Craniofacial parameters were studied clinically and by cephalometry in 11 trigonocephalic patients from infancy to 4 years of age. Six of the most severe patients had surgery between 6 and 18 months of age. Analysis of morphology indicated that bony interorbital distance was reduced in patients selected for surgery and hypotelorism persisted at final examination. All patients demonstrated orbital width measurements above the mean for the norm, while orbital height was essentially normal. All but one of the patients had a variably prominent forehead bony ridge that was eliminated or reduced as a result of surgery and/or growth. However, the forehead of most patients, whether operated or not, was too narrow when compared to normal skulls. Thus, although some of the striking features of trigonocephaly are eliminated, minor characteristics of the anomaly still persevere at 4 years of age.

The aim of this study was to compare the safety, morphological outcome, and degree of parental satisfaction of the new spring-mediated cranioplasty with those of the modified pi-plasty in the management of sagittal synostosis. Ten patients with non-syndromic sagittal synostosis treated with the spring-mediated cranioplasty were followed prospectively. A control group of 10 sex-matched patients operated on with the modified pi-plasty procedure was chosen. Cephalometric radiographs were obtained preoperatively and postoperatively at 1 year of age. Cephalic index, axial width ratio, length ratio, width ratio and height ratio were used as objective measures of outcome. Parents were sent a questionnaire to obtain a subjective aesthetic assessment of outcome. Significantly less blood replacement was required (p = 0.003), and shorter duration of postoperative anaesthesia (p = 0.030) and postoperative hospital stay (p = 0.013) were found in the spring-mediated cranioplasty group. There were no complications or deaths in either group. Also significant was the inter-group difference in the postoperative change in the height ratio (p = 0.030), the most change being seen in the spring group. The change in the subjective parental aesthetic evaluation of skull shape was significant in both groups. In conclusion, the spring-mediated procedure was morphologically more effective than the modified pi-plasty procedure in the management of sagittal synostosis with the additional benefits of less blood transfusion needed and shorter duration of hospital stay.

We conducted sleep studies in a consecutive series of 21 children with craniofacial malformations and suspected obstructive sleep apnoea. Thirteen had sleep apnoea, and one patient with a tracheostomy could not be tested. Twelve children were admitted for initiation of treatment with nasal continuous positive airway pressure (nCPAP). Ten patients accepted nCPAP, two did not. Seven children use home treatment successfully. Obstructive sleep apnoea in most children is completely eliminated with nCPAP. The treatment is safe, effective, and leads to a reduction in the number of operations done for children with craniofacial malformations. Aesthetic operations can be delayed until facial growth is complete.

Reconstruction of calvarium after tumor resection may present several technical difficulties. The authors reused the resected calvarial bone in four patients after submitting the bone to a lethal dose of gamma radiation. The authors conclude that resected, irradiated, tumorous bone can be reused for the reconstruction of its own defect. This provides a simple method of reconstruction. Partial bone resorption should be anticipated but further reconstruction, if needed, will be facilitated.

Craniofacial surgery in craniofacial dysostosis on airway obstruction was studied retrospectively in a consecutive series of patients. The records of 76 patients were reviewed, 27 with Apert syndrome, 47 with Crouzon's syndrome, and two with Pfieffer's syndrome. Of 172 operations, 148 were done for cosmetic reasons, hydrocephalus, or papillary oedema, and 24 were done for airway distress. Forty patients (23%) were children less than 13 years of age, and 22 underwent midface advancement procedures. Only 13 of these operations had been done for airway distress. Two were cured by operation and seven improved. After operation had failed to relieve the airway obstruction, a nasal continuous positive airway pressure device (nCPAP) was fitted to seven patients. The nCPAP relieved or improved airway obstruction recorded by a sleep study. As midface advancement in childhood rarely results in lasting improvement in breathing or aesthetics, it may well be advisable to postpone operation until the early teens. We conclude that with nCPAP operation can in most cases be deferred until a time when a permanent result can be achieved.

Thirteen bone tumours that were invading the craniofacial skeleton were operated on by intracranial procedures. The resected tumorous bone was autoclaved and put back. Follow up of no less than one year included 122mTc scanning, computed tomography, radiography, bone biopsy and clinical examination, in every case, when rigidly fixed, most of the autoclaved bone was gradually revitalised by invading new and normal bone. We conclude that autoclaved bone will be replaced by normal bone, and that the present technique is justified for reconstruction of complicated structures or large areas of bone after operations for tumours invading the craniofacial skeleton.

An experimental model to study the effects of temperature, ischemia and pressure on microcirculation and wound healing in rabbit skin flaps is described. An island skin flap is inserted into a plexiglass chamber and a transverse segment at the middle of the flap is compressed with a plastic cuff. When the cuff is inflated the flap is divided into three segments, one used as a control, one being subjected to pressure and ischemia and one being ischemic only. The method has been tested on two groups of rabbits; in one group a pressure of 200 mmHg was applied for 2 hours while in the other the same pressure was applied for 4 hours. The temperature was kept constant at 36°C in both groups. The microcirculation was observed with a vital microscope in all segments during and after release of the pressure. After the acute experiments the flaps were resutured. Eight days later the influence of the pressure/ischemia was assessed using microangiography and by checking wound healing of the different flap segments. T...

A standardized method of surgical dismantelling of the face for access to the periorbital area is described in detail. Its use in 11 patients with periorbital tumors is described and illustrated by selected case reports. It was concluded that many tumors in the periorbital area that previously were considered inoperable with craniofacial disassembling techniques can be adequately treated without sacrificing eyes or mutilating the face.

The microvascular sleeve anastomosis, where one vessel is inserted into the other, was compared with the conventional end to end anastomosis. In an experimental study in rat femoral arteries 42 anastomoses of each kind were examined. Operating time, patency rates, flow rates and strength of the anastomoses were recorded at various postoperative time intervals. It is concluded that the sleeve anastomosis is quicker to perform and that the two types of anastomoses in biomechanical respects are similar. Flow rates are slightly decreased immediately postoperatively in the sleeve anastomosis but this is of a temporary nature.

A long-term study of 5 sleeve anastomoses in rabbit renal arteries was performed. Six months postoperatively angiography, light microscopy and electron microscopy was carried out. Good long-term function of the anastomoses without any stenoses or aneurysm formation was obtained. It was concluded from this study in rabbits that the sleeve technique is suitable for microvascular anastomosis and that good long-term function can be obtained.

The cause of maldistributed capillary perfusion in hemorrhagic shock was investigated in a combined in vivo and in vitro study. The cat tenuissimus muscle was observed by vital microscopy before and after withdrawal of 45% of the blood volume. The induced shock conditions reduced the number of perfused capillaries about 50%. Close examination of the scattered, nonperfused capillaries showed that they remained open and almost invariably contained one or several leukocytes. These cells were usually located at the bulgings of endothelial cell nuclei, and when occasionally dislodged, recirculation immediately occurred. Platelet or erythrocyte aggregates were not seen in the microvasculature in shock. To test whether the trapping of leukocytes reflected an impaired deformability of these cells in shock or was merely a pressure dependent phenomenon, deformability studies were performed on leukocytes in vitro. Central venous blood was drawn before and 2 h after bleeding and leukocytes were separated by sedimentation. The passage-time of single leukocytes through a glass capillary stenosis at constant driving pressures was used as an index of cell deformability. No qualitative changes in stiffness were found after shock. The distribution of cell passage-times indicated, however, that the stiffest portion of the leukocyte population was removed from the circulation in shock.

A microvascular sleeve anastomosis, inserting one vessel into another, using 3 extraluminal sutures, was performed on rat femoral vessels. In 21 arterial and 21 venous anastomoses the intimal healing and the vascular wall rebuilding were studied by scanning and transmission electron microscopy. The patency rate of the anastomoses was 21/21 in the arteries and 19/21 in the veins. Functional recovery of the endothelium took place within a week.

A program for preoperative workup and post-operative follow-up in children with craniofacial anomalies is described. Objective measurements were made with cephalometry and computed axial tomography. Improvement in length of the anterior cranial base and shape of the calvaria after surgery and growth was demonstrated in this manner in serial follow-ups. It was concluded that both cephalometry and computed tomography were essential for the diagnosis, surgical planning and follow-up for this group of patients.

Craniofacial surgical techniques have yielded better methods of treating severe craniofacial trauma. In this paper surgical techniques are discussed in principle and specifically presented through case reports. We conclude that the most important factors for successful reconstruction after craniofacial trauma are to do as much as possible the first time, to obtain wide exposure, to ensure rigid fixation of bone pieces and grafts, and to make use of a workbench procedure where bone fragments are assembled on a side table for subsequent reattachment to the head.

Our results of bone grafting to the alveolar process during the mixed dentition were investigated in 55 consecutively treated patients (66 clefts). The amount of remaining bone and gingival retraction at the tooth mesial to the cleft after 3 and 12 months was measured and correlated with the following anatomical conditions present during surgery: width of the cleft, rotation of the adjacent incisor, stage of eruption of the tooth distal to the cleft. It was also considered if any deciduous lateral incisor or canine was extracted during surgery and if any flap dehiscence took place postoperatively. It was found that flap dehiscence resulted in significantly less bone at 3 months and at 1 year after surgery. Furthermore, extraction of a deciduous tooth was found to be significantly correlated to less bone 1 year after surgery, in which cases there were also persisting gingival retractions. The other factors had no significant influence on the outcome of surgery.

Premature sutural synostoses are currently being treated at the Craniofacial Center at the Department of Plastic Surgery, University of Göteborg. A leading principle has been to release tethered parts of the skull and the facial skeleton in order to let the expanding brain mould the skeletal parts to an acceptable position. This can be achieved within the first year of life. When operating in later stages, the skeletal parts are wired in a semi-static position by a single osteosynthesis, still allowing expansion by the growing brain.

In an experimental pilot study polyglycolic acid tubes in the rat were used as vessel grafts. 8 femoral arteries and 12 femoral veins were grafted in 15 animals, and followed up to 4 months. No artery was patent upon examination, while 6 out of 12 veins stayed open. A neointima was formed in the patent vessels and the vascular wall seemed to regenerate, while the tube graft was absorbed. It was concluded that, further developed, the polyglycolic acid tubes hold definite promise as instant and absorbable microvascular grafts.

A prospective double blind study using Varidase® or Trypure® on 2nd degree local burns in 18 patients was performed. No difference in cleaning or healing effect was revealed. In 6 out of 11 Trypure®-treated and 1 out of 12 Varidase®-treated patients treatment had to be discontinued because of intolerable pain. This difference was statistically significant.

Cephalometric radiographs and dental study casts were analyzed in a group of 23 seven-year-old cleft lip and palate patients, 16 with unilateral and 7 with bilateral cleft. The patients' primary surgical procedures had been completed except for closure of the cleft in the hard palate. For comparison, similar records from another group of patients, 18 with unilateral and 8 with bilateral cleft lip and palate, were studied. In these cases the cleft of the hard palate had been repaired in infancy, using a vomer flap procedure. The results indicated that midfaciai growth and dental occlusion of the unilateral cleft sample was significantly better in patients whose closure of the hard palatal cleft had been delayed to the stage of mixed dentition than where repair had been performed with a vomer flap in infancy. No differences were found, however, between similar subgroups with bilateral cleft lip and palate.

One hundred and sixty-five patients with localized cancer of the lower lip were excised and reconstructed over a 25-year period using Bengt Johanson's step technique. Eighty-eight percent of the tumors were less than 2 cm in size and 65% were of high histopathological differentiation. Fifty-six percent were reconstructed with bilateral step flaps. Nine local recurrences appeared in 5 patients, none of whom died of lip cancer. Eight patients later developed regional metastases and 3 of these patients died of lip cancer. The 5-year survival rate was 98%. The step technique is recommended for reconstruction of lip defects of up to 2/3 of the lower lip and may, in larger resections, be combined with either a fan flap or an Estlander flap. The outstanding functional results are due to the use of adjacent tissue for the reconstruction which preserves the normal arrangement of muscles, vessels and nerves.

Clinical use of the microvascular sleeve anastomosis in 6 elective free tissue transfers is reported. Arterial and venous anastomoses were performed. It was found that with the exception of the distal anastomosis of a vein graft to artery the sleeve anastomosis was simple and efficient. It was concluded that when the vessels to be anastomosed have suitable length and diameter the sleeve anastomosis may well be used. By its use time and effort can be saved, especially in cases requiring multiple anastomoses.

Craniofacial growth after surgery in children is not completely understood. We have therefore formed a programme for pre- and postoperative studies with both roentgencephalometric- and computed tomographic investigations. This regimen results in overlapping information. Thus, an assessment of the advantages and disadvantages of the two methods was made and exemplified in four patients. In our experience both cephalometry and CT are essential for the diagnosis, surgical planning and follow-up of surgically treated children with craniofacial anomalies. To minimize the radiation doses and the diagnostic procedures which in these cases often includes general anesthesia, patients with isolated, asymmetrical conditions such as plagiocephaly, might be followed with CT only, since roentgencephalometry can not add much information.