Giovanni Maltese

Spring-assisted surgery in the treatment of sagittal synostosis: A systematic review

Abstract Background: Premature sagittal synostosis (SS) can be surgically corrected using extensive cranioplasties or using less invasive methods, e.g. spring-assisted surgery (SAS). Aim: The aim of the present study was to perform a proper systematic review of springs in the treatment of SS. Methods: A literature search was performed with the assistance of a professional librarian in the databases PubMed, EMBASE, and The Cochrane Library between 1997 and September 2013. Studies that fulfilled the PICO (patients, interventions, controls, outcome) criteria were included. All studies were graded for methodological quality according to MINORS, and all retrospective studies were assessed according to a scale developed for retrospective studies in paediatric surgery. The quality of evidence was rated according to GRADE. Results: A total of 241 abstracts were extracted in the literature search. Five studies met the PICO criteria. Two of these five were considered as preliminary reports and excluded. Assessment according to MINORS showed a mean score of 21, i.e. fair quality. The clinical outcome regarding cephalic index did not differ between the surgical techniques, but the quality of evidence, according to GRADE, that SAS was equally efficient, was very low. Clinical outcome regarding operation time, blood loss, ICU stay and hospital stay was in favour for SAS, but the quality of evidence was low. Conclusions: This systematic review has revealed that the level of evidence for SAS being an equally efficient surgical method as more extensive cranioplasties for SS is low or very low. The results point out the need for well-designed prospective studies within craniofacial surgery.

A new computer tool for systematic evaluation of intracranial volume and its capacity to evaluate the result of the operation for metopic synostosis

Abstract The aim of this project was to develop a tool for systematic evaluation of volumetric changes after surgery for craniosynostosis. A computer program using MATLAB was developed to measure total intracranial volume and frontal volume, anterior to the coronary sutures, by multiplying the area with slice thickness of each slice from just above foramen magnum to just beneath the vertex in CT examinations. The ratio between frontal volume and total volume was used for evaluation of the clinical result in 12 patients operated on for metopic synostosis. In 0.625 and 5 millimetre slices the coefficients of variation were 0.00049 and 0.00058, respectively, for measurements of total volume. The highest coefficient of variation was found in postoperative measurements of the frontal volume and was 0.014 in 0.625 millimetre slices. Measurements in 5 millimetre slices resulted in 3.8% ± 1.5% (mean ± SD) lower total volumes and 5.8% ± 5.3% lower frontal volumes than measurements in 0.625 millimetre slices. In patients operated on for metopic synostosis the ratio between frontal volume and total volume increased 25% ± 16% for patients operated on with cranioplasty in combination with a spring (n = 6) and 20% ± 13% for patients operated on with cranioplasty in combination with a bone transplant (n = 6). In summary, this study has developed a tool that can determine frontal and total intracranial volume with little variation. This tool can be used for systematic evaluation of the result of the operation for metopic synostosis.

Intracranial volume is normal in infants with sagittal synostosis

Abstract Premature sagittal synostosis results in an elongated, narrow skull shape, scaphocephaly. It has been unclear whether the intracranial volume (ICV) of these children is different from that of normal children. The aim of the present study was to precisely determine the ICV in a large cohort of children with premature sagittal synostosis and to compare it to the ICV of a sex- and age-matched control group. All patients (n = 143) with isolated sagittal synostosis registered in the Göteborg Craniofacial Registry until the end of 2012 with a preoperative CT examination were identified. For each case, a sex- and age- (±30 days) matched control was identified from children who had undergone CT for post-traumatic or neurological reasons. The ICV was measured in a semi-automatic MATLAB program with functions such as region growing, watershed, and thresholding in axial CT slices. The ICV was calculated using the Cavalieri principle. The mean (± SEM) values of ICV for children with sagittal synostosis and for corresponding controls were 866 ± 13 ml and 870 ± 15 ml, respectively. The mean ages of these groups were 173 ± 8 days and 172 ± 8 days, respectively. Subgroup analysis of sex and age at CT (≤180 days and >180 days) did not reveal any differences in ICV between cases and controls. Precise determination of ICV in addition to the use of adequate controls has made it possible to conclude that children with premature isolated sagittal synostosis have a normal ICV.

Spring-assisted cranioplasty for bicoronal synostosis.

Background Numerous surgical techniques for cranial reconstruction of patients with bicoronal synostosis have been suggested. The outcome is, however, still often suboptimal. Methods Since 2005, we have, at the Craniofacial Unit, Sahlgrenska University Hospital, Gothenburg, used a standardized surgical technique with advancement and remodeling of the forehead combined with spring distraction of the occipital area. The aim of the current study was to evaluate this operative technique. Eighteen consecutive patients (9 boys and 9 girls) with bicoronal synostosis operated on using this technique were identified. Sixteen patients had syndromic bicoronal synostosis, and 2 had nonsyndromic bicoronal synostosis. Cephalic index was obtained from three-dimensional computed tomography scans, and photographs were analyzed for aesthetic evaluation. Results The preoperative calvarial shape was hyperbrachycephalic in all subjects. Postoperatively, the calvarial shape was, in general, much closer to the norm. The reduction in the mean cephalic index from the preoperative stage (94) to the 3-year follow-up (82) was statistically significant (P < 0.0001). The mean duration of surgery was 155 (SD, 32) minutes, with a mean perioperative bleeding of 237 (SD, 95) mL. The mean hospital stay was 6.3 (SD, 1.5) days, of which the mean intensive care unit stay was 1.6 (SD, 1.2) days. In 2 patients, one of the springs had to be reinserted because of postoperative dislocation. No other major complications were observed. Conclusions Spring-assisted cranioplasty for bicoronal synostosis is a safe technique, is less invasive than many other cranioplasties, and results in marked improvement in the calvarial shape.

Children with Apert syndrome as adults: a follow-up study of 28 Scandinavian patients.

Background: Apert syndrome (acrocephalosyndactyly type 1) includes craniofacial deformities, malformations of the extremities and the central nervous system, and often mental retardation. The aim of this study was to investigate the life situation of adult patients with Apert syndrome. Methods: Thirty-three patients with Apert syndrome born before 1990 were identified in the Göteborg craniofacial registry. The authors used a questionnaire mainly dealing with education, employment, social relations, and quality of life. A matched control group was created for comparison. Results: Five of the patients had died. Twenty-four of the remaining patients answered the questionnaire. The level of education was lower in patients than in controls (p = 0.007), but there was no significant difference in the extent of employment between the two groups. The difference in housing was significant (p < 0.001) and the majority of patients lived with their parents. Patients were less often married (p < 0.001), had fewer friends (p < 0.001), and had less experience with sexual relationships (p < 0.001). The somatic health was lower in patients [e.g., they had more hearing problems (p < 0.001) and more epilepsy (p = 0.005)]. Depressive mood periods were more common in patients (p < 0.001), but there was no difference between the groups regarding a generally positive attitude toward life. Conclusions: This study shows that patients with Apert syndrome manage relatively well in adulthood. The discrepancy in social relations between the Apert patients and the healthy control group indicates that further improvement of the treatment is desirable.

New objective measurement of forehead symmetry in unicoronal craniosynostosis – comparison between fronto-orbital advancement and forehead remodelling with a bone graft

Abstract Patients with unicoronal synostosis (UCS) present with ipsilateral forehead flattening, contralateral frontal bossing, and rotation of the facial midline. Uni- or bilateral fronto-orbital advancement (FOA) techniques are the most common surgical approaches for correction of UCS. The purpose of this study was to objectively evaluate the surgical outcome in patients for UCS, using a new MATLAB computer tool programmed to measure the symmetry of the two halves of the forehead.Files were reviewed from a consecutive series of patients treated for UCS at the unit, from 1979–2008. The patients were grouped according to the method of operation used. The computer tool evaluated preoperative and postoperative cephalograms and CT scans. Eighty-eight patients were included. The male-to-female ratio was 1:2.4. Forty-six patients had been operated on with FOA and 42 with forehead remodelling using a calvarial bone graft. Forehead symmetry was significantly improved by both techniques (p < 0.001 for both), but the postoperative forehead symmetry was significantly better after forehead remodelling (p = 0.025). The reoperation rate was much lower for the second group (6.5 vs 37.2%, p < 0.001). It is concluded that forehead remodelling with a calvarial bone graft creates a more symmetrical forehead than FOA and may, therefore, be a better alternative for treatment of unicoronal 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 ± 127 mL (mean ± SD) and 70.1 ± 4.0 to 1300 ± 158 mL and 73.1 ± 3.3, respectively. The corresponding values for controls were 796 ± 136 mL and 83.6 ± 7.3 preoperatively and 1334 ± 136 mL and 80.0 ± 4.5 at 3 years of age. Pi-plasty increased the ICV and CI from 1014 ± 115 mL and 69.7 ± 3.3 to 1286 ± 122 mL and 74.1 ± 2.6, respectively. Corresponding values for controls were 1043 ± 153 mL and 83.4 ± 7.0 preoperatively and 1362 ± 122 mL and 79.6 ± 3.9 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 in 15 children with bilateral coronal craniosynostosis.

Background: Intracranial volume (ICV) growth in patients with bilateral coronal craniosynostosis (BCS) is not well described. It is therefore important to evaluate the consequences of cranial surgery in children with this condition. The aim of the present study was to evaluate ICVs in patients operated on for BCS. Methods: A consecutive series of patients with BCS were operated on using spring-assisted cranioplasty, with computed tomography scans in 0.6-mm slices, were included. A MATLAB-based computer program capable of measuring ICV was used. Patients were compared with an age- and gender-matched control group of healthy children. Student’s t test was used for statistical analysis. Results: Fifteen patients (7 girls and 8 boys) with 43 computed tomography scans were identified. The diagnoses were 13 syndromic BCS (3 Apert, 1 Crouzon, 6 Muenke, and 3 Saethre-Chotzen) and 2 nonsyndromic BCS. The mean preoperative volume at the age of 5 months (n = 15) was 887 mL (range, 687–1082). Mean volume at follow-up at the age of 3 years (n = 13) was 1369 mL (range, 1196–1616). In comparison, the mean ICVs for controls at the ages of 5 months (n = 30) and 3 years (n = 26) were 854 mL and 1358 mL, respectively. The differences were not statistically significant (P > 0.05). Conclusions: Patients with BCS were operated on with spring-assisted cranioplasty seem to maintain their age-related ICV at 3 years of age when compared to normal children.

Conservative Healing of an 11 9-cm Aplasia Cutis Congenita of the Scalp with Bone Defect

Objectives Aplasia cutis congenita is a rare congenital condition, and it is difficult to find scientific support for optimal treatment strategies. In addition, these may vary due to defect size, tissue layers involved, contemporary malformations, and the physiologic status of the affected child. Clinical Presentation This case report describes complete skin coverage in 20 weeks and uneventful healing of a large 11 × 9-cm defect of the vertex, involving both skin and skull bone, using conservative treatment. To prevent infection and promote healing, the defect was kept moist and covered at all times, and it was treated with surgical debridement when necessary. For infection control, ionized silver-coated dressings were used in addition to prophylactic antibiotics over the first 3.5 weeks. Follow-up was 2 years. Conclusion Surgical treatment is usually preferred for larger aplasia cutis congenita defects, but it is accompanied with potential risks and will exacerbate secondary reconstruction of alopecia or skull bone defects. This case shows that even very complex defects may be treated conservatively.