Spring-Assisted Surgery of Unilambdoid Craniosynostosis.

Craniosynostosis is traditionally treated with extensive cranial vault reconstructions (CVRs). Although less invasive techniques, such as endoscopic strip craniectomy with postoperative helmet therapy, have been successful, they also present difficulties. An alternative method is distraction osteogenesis using either manually controlled devices or specially designed springs. In this study, the authors provide the first comparison of spring-assisted surgery (SAS) with CVR for the treatment of unilambdoid synostosis (ULS). Fourteen consecutive patients (8 CVR and 6 SAS) treated for ULS at Sahlgrenska University Hospital between 2005 and 2018 were included. Skull shape and deviations were evaluated using previously defined measurement points on 3-dimensional computed tomography scans preoperatively, at spring removal, and at 3 years of age. Posterior and middle cranial fossa (PCF and MCF, respectively), skull-base cant, facial twist, and mastoid bulge (MB) were measured, and clinical data were obtained from chart reviews. The results indicated that at the 3-year follow-up, PCF, MCF, and MB improved in both groups, with no significant difference in outcome observed between methods. In the SAS group, duration of operation [61±27 min (mean±SD)] and perioperative bleeding (3.5±2.8 mL/kg body weight) were both significantly lower relative to the CVR group (P<0.05). These findings showed that both SAS and CVR resulted in similar improvements in treating ULS, although neither produced complete normalization of skull shape. The results suggest that early diagnosis and operation allow less extensive SAS to be performed without adversely affecting the results.

Correction of Unicoronal Synostosis With Springs: Two Patients With Improved Facial Symmetry.

OBJECTIVE: Surgical correction of unicoronal synostosis (UCS) entails extensive cranioplasties which do not address facial scoliosis. This paper presents the first results with springs that motivated the shift from extensive cranioplasties to dynamic techniques for surgical correction of UCS. METHODS: Two cases of UCS were operated with a linear osteotomy combined with springs. The deviation in facial symmetry (orbital dystopia angle) and skull base angles were measured on pre and postoperative computed tomography scans until 3 years of age. RESULTS: The facial scoliosis was corrected. At spring removal, the orbital dystopia angle had gone from a 9.2 to 13.2-degree deviation preoperatively to a 0.5 to 0.9-degree overcorrection compared with the ideal 0-degree deviation. Also, the skull base deviation improved. CONCLUSION: Linear osteotomy combined with springs corrects the facial scoliosis in UCS. These cases indicate that dynamic methods may be beneficial for improving the results of surgical correction of UCS.

Ethical Considerations in Surgery for Single-suture Craniosynostosis.

Singe-suture craniosynostosis (SSC) describes the premature fusion of one cranial suture, which restricts cranial growth and consequently results in unaffected regions presenting a compensatory expansion. Surgery can redistribute intracranial volume, reduce the risk of elevated intracranial pressure, and improve head shape, potentially leading to improved neurocognitive function and social acceptance. However, there is limited evidence that surgery for SSC improves neurocognitive function and social acceptance. Given the inherent surgical risks and uncertainty of outcomes, the conditions under which this surgery should be allowed remain uncertain. Here, we discuss ethical questions regarding the permissibility of surgery, value of neurocognitive function and social acceptance, research ethics associated with SSC, patient autonomy and parental roles, and the process of recommending surgery and obtaining consent. Because surgery for SSC has become a routine procedure, its practice now presents a relatively low risk of complications. Furthermore, having acquired an understanding of the risks associated with this surgery, such knowledge fulfils the principle of non-maleficence although not beneficence. Thus, we advocate that surgery should only be offered within Institutional Review Board-approved research projects. In these situations, decisions concerning enrollment in scientific research involves health care providers and parents or guardians of the child, with the former acting as gate-keepers upon recognition of a lack of coping skills on the part of the parent or guardian in dealing with unforeseen outcomes. To minimize associated surgical risks and maximize its benefits, there exists a moral obligation to refer patients only to highly specialized centers.

Incidence of Non-Syndromic and Syndromic Craniosynostosis in Sweden.

ABSTRACT: Premature craniosynostosis is a rare condition, with a wide range of incidence estimations in the literature. The aim of this study was to establish the current incidence among the Swedish population. Since the surgical care for these children is centralized to the 2 centers of Sahlgrenska University Hospital and Uppsala University Hospital, the 2 craniofacial hospital registries were examined for surgically treated children, all having a computed tomography verified diagnosis. Results show an incidence of 7.7 cases per 10,000 live births, including 0.60/10,000 syndromic craniosynostosis. Due to information programs among health care staff and a system for early diagnosis through rapid communication, these results seem to mirror the true incidence of craniosynostosis in the Swedish population. The updated incidence data will facilitate healthcare planning and make future studies of possible changes in craniosynostosis incidence more accurate.

Comparisons of Intracranial Volume and Cephalic Index After Correction of Sagittal Craniosynostosis With Either Two or Three Springs.

ABSTRACT: In this retrospective study, the authors determined changes in intracranial volume (ICV) and cephalic index (CI) in patients with sagittal craniosynostosis and operated with craniotomy combined with either 2 or 3 springs. The authors included patients (n = 112) with complete follow-up that had undergone surgical correction for isolated sagittal craniosynostosis with craniotomy combined with springs between 2008 and 2017. All patients underwent computed tomography examination preoperative, at the time of spring extraction, and at 3 years of age. Intracranial volume was measured using a semiautomatic MATLAB program, and CI was calculated as the width/length of the skull. The authors found that craniotomy combined with 2 springs increased the ICV from a preoperative value of 792 ±â€Š113 mL (mean ±â€Šstandard deviation) to 1298 ±â€Š181 mL at 3 years of age and increased the CI from 72.1 ±â€Š4.1 to 74.6 ±â€Š4.3, whereas craniotomy combined with 3 springs increased the ICV from 779 ±â€Š128 mL to 1283 ±â€Š136 mL and the CI from 70.7 ±â€Š4.3 to 74.8 ±â€Š3.7. The relative increase in ICV was 65 ±â€Š21% in the two-spring group and 68 ±â€Š34% in the three-spring group (P value = 0.559), and the relative increase in CI was 3.6 ±â€Š3.3% in the two-spring group as compared with 6.0 ±â€Š5.0% in the three-spring group (P = 0.004). These findings demonstrated that use of 3 springs resulted in additional absolute and relative CI-specific effects as compared with 2 springs during the time when the springs were in place, with this effect maintained at 3 years of age.

Simplified Postoperative Care After Spring-assisted Strip Craniotomy for Sagittal Synostosis: A Prospective Before-and-After Study.

ABSTRACT: Since the development of spring-assisted techniques for corrective craniofacial surgery, routine postoperative admission to intensive care units (ICUs) has been questioned. However, close monitoring is necessary if the continuous infusion of morphine is used as recommended for better pain relief. In this study, the authors evaluated a simplified postoperative protocol without continuous morphine infusion and no indwelling urinary catheter following spring-assisted surgery (SAS) for sagittal synostosis. Ten children were cared for according to a standard protocol with postoperative intravenous (i.v.) infusion of morphine and an indwelling urinary catheter, and 11 consecutive children were treated according to a simplified protocol with pain relief based on intermittent injections of morphine and clonidine [according to Face, Legs, Activity, Cry, Consolability (FLACC) scores >4] without the indwelling catheter. A Mann-Whitney U test was used for comparison of distributions between the two groups. The results revealed no differences between groups regarding the proportion of FLACC scores >4, total amount of administered i.v. morphine and clonidine, total volume of buffered glucose infused, time to first feeding on breast milk or substitute, or the length of stay. Despite the inherent limitations of our small observational study, the authors concluded that at our institution, it was possible to exclude a standard continuous i.v. infusion of morphine and an indwelling urinary catheter from our postoperative care protocol without decreasing the quality of pain relief in children submitted to SAS for sagittal synostosis. This finding supports downgrading the level of care from the ICU to a regular ward after limited immediate postoperative observation.

Craniosynostosis: A Reversible Pathology?

The formation of the cranial sutures, in utero, occurs when the ossification of the skull bones reaches predestined positions around gestational week 15 to 20. Craniosynostosis, and the consequent skull shape deformities, is treated with surgery including osteotomies of the fused sutures. The occasional appearance of a new suture in the osteotomy lines has previously been described as sporadic events. In this retrospective study, a 4-year consecutive series of osteotomies combined with springs for craniosynostosis were systematically analysed regarding the appearance of neosutures. In total, 84 patients were included and in 16 patients (19%) a new radiologically normal suture appeared in a part of the suture that was completely closed preoperatively. Additionally, in 7 patients (8%) a new suture appeared in a part of the suture that had a discernible suture prior to surgery.In conclusion, in this consecutive and well-defined patient cohort operated for craniosynostosis, the formation of a neosuture is not a rare, and speculatively not a random, event. The appearance of a new suture long after the normal time period for suture formation in utero indicates that the craniosynostosis may just as well be caused by disturbed formation of the suture as actual premature closure.

Spring-Assisted Surgery in the Treatment of Complex Craniosynostosis.

Multisutural nonsyndromic craniosynostosis is a rare group of malformations, whose frequency has been reported between 3% and 7% of all craniosynostosis. The clinical diagnosis can be difficult and computed tomography is usually required. Surgical treatment is challenging and staged procedures are performed in up to 80% of patients. The aim of the present study was to determine the reoperation rate and to evaluate the surgical outcomes by measuring intracranial volume (ICV) preoperatively and at follow-up, and comparing it to a control group. Perioperative variables and reoperation rate were recorded. Fifty-one patients presented with a complex pattern of synostosis without a recognizable syndrome (5% of cases of total patients evaluated). Fifteen patients have been treated with spring-assisted surgery, either alone or in combination with a foreheadplasty. The mean follow-up was 6.2 years. The mean preoperative ICV of the patients was smaller, but not significantly, than in the normal population (P = 0.13). Postoperatively, the mean ICV was similar to that of the control group at 1 year (P = 0.92), while at 3 years it was appreciably smaller, although not significantly different (P = 0.06). Five patients (33%) went through a secondary skull expansion for either raised intracranial pressure or cosmetic reasons. Spring-assisted surgery seems to temporarily expand ICV in children with complex synostosis and lower the reoperation rate, thus reducing the need for a second procedure. A longer follow-up would be necessary to further investigate the effects of springs over time.

Cranioplasty without Periosteal Dissection Reduces Blood Loss in Pi-Plasty Surgery for Sagittal Synostosis.

BACKGROUND/AIMS: Cranioplasty is often accompanied by a substantial oozing bleeding from the bone surface and bone edges. Our aim was to measure if strict subgaleal dissection without any periosteal release reduces blood loss during pi-plasty surgery for sagittal synostosis. METHOD: A group of 32 children who underwent pi-plasty surgery at the Sahlgrenska University Hospital between 2010 and 2014 for premature sagittal synostosis with traditional subgaleal dissection combined with incision and release of the periosteum adjacent to the osteotomy lines was compared to a group of 7 children who underwent pi-plasty with strict subgaleal dissection and osteotomy through the bone with the periosteum attached. Information about blood loss and body weight was extracted from medical records. RESULTS: The blood loss in the group of 7 children with strict subgaleal dissection was 102 ± 86 mL (mean ± SD) (10 ± 7 mL/kg) compared to 320 ± 119 mL (32 ± 12 mL/kg) in the control group with traditional periosteal release (p < 0.001). CONCLUSION: Intact periosteum at the osteotomy lines significantly reduces blood loss in pi-plasty surgery for sagittal synostosis. The mechanism is likely because of preserved veins between the bone surface and periosteum.

The Degree of Surgical Frontal Volume Correction in Metopic Synostosis Determines Long-Term Outcomes.

Metopic synostosis results in a keel-shaped forehead, reduced frontal intracranial volume (ICV), and lower frontal to total volume ratio. The ratio improves with cranioplasty, but at 3 years of age, the ratio is still not normalized when compared to that in normal children. The aim of the present study was to investigate whether a low frontal to total ICV ratio at 3 years of age was due to relapse or insufficient correction.All children surgically treated for metopic synostosis in combination with a spring at Sahlgrenska University Hospital with subsequent spring extraction between 2002 and 2008 (n = 20) were included. A MATLAB program was used to measure frontal and total ICV.Preoperatively, the frontal to total ICV ratio was 9.8 ±â€Š1.3% (mean ±â€Šstandard deviation). At spring removal, 6 months after cranioplasty, the ratio had increased to 11.8 ±â€Š2.4%. At 3 years of age, the ratio was 11.6 ±â€Š1.9%. In age-matched normal children, the ratio was 14.4 ±â€Š1.9% preoperatively, 15.3 ±â€Š2.2% at time of spring extraction, and 13.4 ±â€Š1.4% at 3 years of age.Cranioplasty thus improved the frontal to total ICV ratio, but did not normalize it. The ratio did not change from 6 months after the cranioplasty to 3 years of age. These results indicate that a more pronounced frontal volume correction during cranioplasty is necessary to achieve a normalized distribution of ICV in metopic synostosis.

Craniotomy of the Fused Sagittal Suture Over the Superior Sagittal Sinus Is a Safe Procedure.

INTRODUCTION: Spring-assisted cranioplasty to correct sagittal synostosis is based on midline craniotomy through the closed sagittal suture, over the superior sagittal sinus (SSS). The aim of the present study was to evaluate the perioperative safety of this technique. MATERIALS AND METHODS: This is a retrospective study of all patients operated with median craniotomy and springs from 1998 to the end of 2015. For comparison, all Pi-plasties performed during the same time interval were also evaluated. The safety measures were evaluated based on incidence of damage to SSS, incidence of dural tears, perioperative blood loss, operative time, and hospital stay. RESULTS: In the group that had undergone midline craniotomy combined with springs (n = 225), 4 perioperative damages to SSS and 1 dural tear were seen. The perioperative blood loss was 62.8 ±â€Š65.3 mL (mean ±â€Šstandard deviation). The operative time was 67.9 ±â€Š21.5 minutes and the hospital stay was 4.8 ±â€Š1.1 days. In the group that had undergone pi-plasty (n = 105), no damages to SSS but 3 dural tears were seen. The perioperative blood loss was 352.8 ±â€Š174.4 mL. The operative time was 126.0 ±â€Š31.7 minutes and the hospital stay was 7.1 ±â€Š1.4 days. CONCLUSION: Craniotomy SSS in sagittal synostosis is a safe procedure with low morbidity in terms of damage to the SSS. Midline craniotomy combined with springs has significantly lower preoperative blood loss, operative time, and hospital stay (P < 0.001 for all) compared to pi-plasty.

Temporal Deformity Objectively Measured Before and After Surgery for Metopic Synostosis: Retrusion Rather than Hollowing.

The temporal contour deformity typical of metopic synostosis is often referred to as temporal hollowing, but has not been quantitatively defined. This deformity is present before surgery and remains to a varying extent at long-term follow-up. The present study aimed to objectively evaluate the degree of this contour deformity in metopic synostosis before and after surgical correction.All children surgically treated for metopic synostosis at Sahlgrenska University Hospital between 2002 and 2014 (n = 120) with appropriate computed tomography scans (n = 160) performed preoperatively and/or at follow-up at 3 years of age were included. Depending on age, 1 of 2 surgical techniques was used. Children presenting before the age of 6 months were treated with frontal remodeling in combination with a spring (S group), whereas children older than 6 months were treated with a bone transplant (BT group). The bony temporal deformity was measured with a semiautomatic MATLAB program and patients were compared to sex- and age-matched controls.The deformity was significantly reduced in both groups (P < 0.001). In the S group, it was reduced from a mean ±â€Šstandard deviation of 3.6 ±â€Š1.9% to 1.0 ±â€Š1.2% and in the BT group, it was reduced from 3.3% ±â€Š1.4% to 1.1% ±â€Š0.8%.The contour deformity in metopic synostosis is present both before and after surgery and should therefore be termed temporal retrusion (TR). This assessment method enables objective comparison of TR before and after surgical correction and is a potential tool to evaluate TR in metopic synostosis.

Comparison of Intracranial Volume and Cephalic Index After Correction of Sagittal Synostosis With Spring-assisted Surgery or Pi-plasty.

BACKGROUND: The aim of the current study was to determine the intracranial volume (ICV) and cephalic index (CI) in patients operated for sagittal synostosis, and to compare the outcome of 2 different surgical techniques: craniotomy combined with springs and modified pi-plasty. METHODS: The authors studied all patients who had been operated for isolated sagittal synostosis and registered in the Gothenburg Craniofacial Registry until the end of 2012 and who had undergone a preoperative and/or postoperative (at 3 years of age) computed tomography examination. Sex- and age-matched controls were identified from children who had undergone computed tomography for other reasons. RESULTS: Craniotomy combined with springs increased the ICV and CI from 802 ±â€Š127 mL (mean ±â€ŠSD) and 70.1 ±â€Š4.0 to 1300 ±â€Š158 mL and 73.1 ±â€Š3.3, respectively. The corresponding values for controls were 796 ±â€Š136 mL and 83.6 ±â€Š7.3 preoperatively and 1334 ±â€Š136 mL and 80.0 ±â€Š4.5 at 3 years of age. Pi-plasty increased the ICV and CI from 1014 ±â€Š115 mL and 69.7 ±â€Š3.3 to 1286 ±â€Š122 mL and 74.1 ±â€Š2.6, respectively. Corresponding values for controls were 1043 ±â€Š153 mL and 83.4 ±â€Š7.0 preoperatively and 1362 ±â€Š122 mL and 79.6 ±â€Š3.9 at 3 years of age. CONCLUSIONS: There was no significant difference between craniotomy combined with springs in children younger than 6 months and pi-plasty in older children regarding the efficacy of improving ICV and CI. Neither of the techniques fully normalized the head shape.

Comparison Between Two Different Isolated Craniosynostosis Techniques: Does It Affect Cranial Bone Growth?

INTRODUCTION: Craniosynostosis is a premature closure of a cranial suture. Cranioplasty is indicated to correct skull deformity, relieve increased intracranial pressure, and promote homogenous cranial growth. Different techniques have been adopted to achieve optimal outcomes. Although surgical benefits are widely accepted, this intervention might also affect cranial skeletal growth. METHODS: The authors conducted a retrospective case-control study including patients operated for isolated metopic or sagittal synostosis. These patients had undergone a computed tomography (CT) scan before surgery and/or at 3 years of age postoperatively. These were operated between 2002 and 2012. Intracranial volume was measured using a MATLAB application. The control group was age and sex-matched individuals who had CT scans for trauma or neurological indications. All results with P value <0.05 were considered statistically significant. RESULTS: A total of 185 patients were included in the study with a preoperative CT scan (143 sagittal synostosis and 42 metopic synostosis). Postoperatively 160 patients were identified including 103 sagittal synostosis and 57 metopic synostosis. These patients were compared to 414 controls. There was a statistically significant reduction in intracranial volume (ICV) in patients operated for metopic synostosis with both techniques. It also demonstrated a statistically significant difference in ICV in patients with sagittal synostosis operated with Pi-plasty and a nonsignificant difference in ICV in patients operated with strip craniotomy and springs. CONCLUSION: In conclusion, these measurements of ICV have revealed that extensive cranioplasties for premature craniosynostosis can lead to minor but significant growth restriction and reduced ICV at long-term follow-up.

Intracranial volume before and after surgical treatment for isolated metopic synostosis.

Metopic synostosis results in a keel-shaped forehead, hypotelorism, and an increased interparietal width. This study aimed to measure the frontal and total intracranial volume in patients with metopic synostosis before and after surgery and to compare the effect of 2 different operation methods. All patients operated for isolated metopic synostosis between 2002 and 2008 at Sahlgrenska University Hospital who had undergone preoperative and/or postoperative computed tomographic examination (at 3 y of age) were included. The patients were grouped according to operation method: (1) forehead remodeling in combination with a bone graft or (2) forehead remodeling in combination with a spring. Sex- and age-matched controls were identified. A previously developed MATLAB computer program was used to measure the frontal and total intracranial volumes. Sixty patients and 198 controls were included. Preoperatively, the patients with metopic synostosis had significantly lower frontal volumes than those of the controls (P < 0.001) but equal total intracranial volumes. The operations redistributed the intracranial volume and resulted in an improved, frontal-total intracranial volume ratio. However, at 3 years of age, the frontal volume (P < 0.001), total intracranial volume (P ≤ 0.002), and ratio between the 2 (P < 0.001) were significantly lower in the patients than in the controls. The 2 operation methods were equally efficient in creating an improved frontal-total ratio. Surgery for metopic synostosis improves the distribution of the intracranial volume but does not result in normal total intracranial volume or frontal volume at 3 years of age.

Improved Facial and Skull-Base Symmetry following Osteotomy and Distraction of Unilateral Coronal Synostosis.

BACKGROUND: Unilateral coronal synostosis (UCS) results in a surgically demanding deformation, as the deformity is asymmetric in the calvaria but also presents with facial scoliosis and orbital dystopia. Traditional cranioplasties correct the forehead but have little effect on the face and orbits. In this article, the authors describe a consecutive series of patients operated on for UCS with osteotomy of the fused suture combined with distraction osteogenesis. METHODS: Fourteen patients (mean age, 8.0 months; range, 4.3 to 16.6 months) were included in this study. The authors measured and compared the orbital dystopia angle, anterior cranial fossa deviation, and anterior cranial fossa cant between preoperative computed tomography results and those at distractor removal. RESULTS: Blood loss was 6.1 mL/kg (range, 2.0 to 15.2 mL/kg), and length of stay was 4.4 days (range, 3.0 to 6.0 days). The authors observed significant improvements in the median orbital dystopia angle from 9.8 degrees (95% CI, 7.0 to 12.6 degrees) to 1.1 degrees (95% CI, -1.5 to 3.7 degrees) ( P < 0.001), anterior cranial fossa deviation from 12.9 degrees (95% CI, 9.2 to 16.6 degrees) to 4.7 degrees (95% CI, 1.5 to 7.9 degrees) ( P < 0.001), and anterior cranial fossa cant from 2.5 degrees (95% CI, 1.5 to 3.5 degrees) to 1.7 degrees (95% CI, 0.0 to 3.4 degrees) ( P = 0.003). CONCLUSIONS: Osteotomy combined with a distractor for UCS straightened the face and relieved orbital dystopia by affecting the nose angle relative to the orbits, correcting the deviation of the cranial base in the anterior fossa, and lowering the orbit on the affected side. Furthermore, this technique demonstrated a favorable morbidity profile with low perioperative bleeding and a short inpatient period, suggesting its potential to improve the surgical treatment of UCS. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Springs Produce Favorable Morphologic Outcomes Relative to H-Craniectomy According to a Two-Center Comparison of Matched Cases.

BACKGROUND: Sagittal synostosis is the most common type of premature suture closure, and many surgical techniques are used to correct scaphocephalic skull shape. Given the rarity of direct comparisons of different surgical techniques for correcting craniosynostosis, this study compared outcomes of craniotomy combined with springs and H-craniectomy for nonsyndromic sagittal synostosis. METHODS: Comparisons were performed using available preoperative and postoperative imaging and follow-up data from the 2 craniofacial national referral centers in Sweden, which perform 2 different surgical techniques: craniotomy combined with springs and H-craniectomy (the Renier technique). The study included 23 pairs of patients matched for sex, preoperative cephalic index, and age. Cephalic index, total intracranial volume (ICV), and partial ICV were measured before surgery and at 3 years of age, with volume measurements compared against those of preoperative and postoperative controls. Perioperative data included operation time, blood loss, volume of transfused blood, and length of hospital stay. RESULTS: Craniotomy combined with springs resulted in less bleeding and lower transfusion rates than H-craniectomy. Although the spring technique requires 2 operations, the mean total operation time was similar for the methods. Of the 3 complications that occurred in the group treated with springs, 2 were spring-related. The compiled analysis of changes in cephalic index and partial volume distribution revealed that craniotomy combined with springs resulted in superior morphologic correction. CONCLUSION: The findings showed that craniotomy combined with springs normalized cranial morphology to a greater extent than H-craniectomy based on changes in cephalic index and total and partial ICVs over time. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Spring-assisted posterior vault expansion in children over 2 years of age with craniosynostosis.

BACKGROUND: This study evaluated spring-assisted posterior vault expansion (SA-PVE) in children aged > 2 years with craniosynostosis and signs of high intracranial pressure (ICP). METHODS: We retrospectively analysed all consecutive patients aged > 2 years and operated with SA-PVE between 2018 and 2020 at the Craniofacial Center at Sahlgrenska University Hospital, Sweden. During the procedure, a circumferent occipital bone flap extending below the torcula was created and remained attached to the dura. Intracranial volumes (ICVs) were calculated from computed tomography (CT) images, and demographic data and information regarding symptoms and signs of high ICP were collected. RESULTS: The study included eight patients [Crouzon/Pfeiffer (n = 4), multiple craniosynostosis (n = 3), and secondary synostosis (n = 1)]. Median age at SA-PVE was 3.8 years (range: 2.5-12.8 years), and springs were removed after a median of 5.5 months (range: 2.3-8.3 months). The median operating time was 164 min (range: 102-221 min), and estimated blood loss was 4.5 mL/kg body weight (range: 1.4-59.1 mL/kg body weight), with 50% of patients receiving a blood transfusion. The median increase in ICV was 206 cm3 (range: 122-344 cm3) representing an 18.7% increase (range: 7.9-24.1%; p = 0.01). We observed no major perioperative complications, and symptoms related to high ICP were improved or absent at clinical follow-up. CONCLUSION: These results demonstrated that SA-PVE involving creation of a large occipital bone flap including the torcula as a safe and effective surgical treatment in children aged >2 years with craniosynostosis and elevated ICP.

A novel quantitative image-based method for evaluating cranial symmetry and its usefulness in patients undergoing surgery for unicoronal synostosis.

BACKGROUND: Unicoronal synostosis presents with cranial asymmetry. Fixed points are difficult to identify; surgical results are therefore difficult to evaluate. The aim of this study was to develop a computer-based method for evaluation of forehead symmetry to enable evaluation of surgical results in unicoronal synostosis. METHODS: The MATLAB tool was programmed to segment computed tomographic images, leaving the outermost contour. Cephalometric images were segmented manually due to lower contrast. A center-point (O) and an end-point were manually defined in the midline of the forehead and at the nonfused coronal suture, respectively. The program then found a point (p) on the fused side, at the same distance from the O as the end-point. The contours of the left and right side of the forehead were thereafter superimposed, and the position of minimal area mismatch of the sides was identified. To correct for growth between preoperative images and follow-up, the number of mismatching pixels was related to the area outlined by the contour of the forehead, the end-point and p. Two quantities, the relative symmetry change and the absolute symmetry change, were defined and evaluated by repeated measurements on spherical and elliptical phantoms and 15 patients. RESULTS: Measurements with the MATLAB program were reliable with an SD of 0.26% to 5.39% for the expected range of differences. The SD was lower for measurements on computed tomographic images than for measurements on cephalometric images. The SD was also lower in patients with large surgical improvement than in patients with little improvement. The results support the use of relative symmetry change to evaluate surgical results. CONCLUSIONS: Our new computer-based method is capable of measuring forehead symmetry with good precision. This method can be used for systematic evaluation of surgical outcome for unicoronal synostosis and other asymmetric skull deformities.

Health-related quality of life of children treated for non-syndromic craniosynostosis.

Health-related quality of life (HRQoL) allows the acquisition of the subjective perspective of patients regarding their health and function; yet a very few studies have been evaluated HRQoL of patients treated for craniosynostosis (CS). In this retrospective, descriptive cohort study, school-aged children (7-16 years) treated for non-syndromic CS were assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales. Seventy-three patients and their parents responded to the PedsQL (response rate: 80.2%). Patients generally estimated average HRQoL with no difference compared to the normal population sample. Further, no difference in HRQoL was found between treated sagittal (SS) or metopic synostosis. In the SS group, surgical methods involving spring-assisted surgery and pi-plasty were unrelated to HRQoL outcomes. Additionally, HRQoL was highly correlated with intelligence quotient (IQ, r = 0.42; p = 0.0004) and adaptive behavior skills (ABAS, r = 0.57; p = 0.0001). Furthermore, differences were observed in estimated physical function (p = 0.002) and school function (p = 0.012) between self- and proxy reports (i.e. parents estimated child HRQoL as higher than did the children). Children treated for CS have a generally average HRQoL, and neither CS type nor surgical method influenced HRQoL outcomes. Moreover, children and parents estimated HRQoL differently, suggesting the importance of using both self- and proxy reporting in patient-reported measures. HRQoL was strongly related to IQ and ABAS, indicating that the PedsQL can be used as a screening instrument to identify craniofacial patients in need of further psychological assessment.