Experiences in Performing Posterior Calvarial Distraction.

The use of posterior calvarial distraction (PCD) for the management of craniosynostosis is well recognized. The advantages of using this technique include increased cranial volume, decreased intracranial pressure, relief of posterior fossa crowding, improved cerebrospinal fluid (CSF) circulation at the cranio-cervical junction with cessation, and possible resolution of syrinx.The authors retrospectively review their first 50 patients who have undergone PCD under the senior author's care in our unit.The demographics, diagnoses, intraoperative approach with techniques in distractor placement and outcomes of each patient were obtained through an electronic craniofacial database and written patient records. Analysis of complication rates (bleeding, distraction problems, CSF leaks, and infection) was included.A total of 31 boys and 19 girls underwent the procedure between October 2006 and September 2015 with a median age was 17.7 months (range 4 months to 19 years). Of those 50 children, 34 of the cohort were proven to be syndromic by genetic testing.The median length of inpatient stay was 9.4 days (range 3-43 days). Average distraction distance was 24 mm.Complications including CSF leaks, bleeding, distractor problems, and severe complications (recorded in 3 patients) are discussed. Our overall complication rate was 50%.Favorable outcomes included resolution of Chiari, syrinx, and raised intracranial pressure in the majority of patients where distraction was successful.The authors recommend that PCD should be considered the primary treatment for increasing calvarial volume. The authors discuss our experiences and technical innovations over the past decade.

Empowerment of Parents of Children With Craniosynostosis by Objective Scoring of Patient Information Websites.

INTRODUCTION: Parental anxiety may be caused by inappropriate information on the world wide web regarding craniosynostosis. The aim of this study was to study the correlation between the first 100 websites ranked on the Google search engine and those ranked based on objective scoring with patient information scoring tool. The results of these findings can then be used to recommend websites based on the quality of information that may not initially appear first on a search engine. METHODS: The DISCERN patient information scoring tool was chosen as a scientific way of measuring the quality of patient information based on previous research by the Picker Institute in Oxford. The word "craniosynostosis" was entered into the Google internet search engine. Patient information provided by the first 100 websites was studied and scored using the DISCERN scoring tool. From this score, each website was ranked with the highest scoring website at the top and worse scoring website at the bottom. This ranking based on the DISCERN tool score was correlated against the ranking of the website by Google. Positive correlation between DISCERN and Google ranking would be demonstrated if a website high in the ranking using DISCERN was also at the top of the first 100 websites on Google. RESULTS: No correlation could be found between those websites ranked highly for patient information on craniosynostosis using the DISCERN tool to the ranking provided by Google. DISCERN scores ranged from 17 to 72 (the lowest possible DISCERN tool score being 15 and the highest 85). The website ranking highest in terms of quality appeared only 50th in the Google rank. CONCLUSION: High-quality patient information on craniosynostosis does exist on the world wide web but may be difficult to find due to the complexity of factors used to rank websites on internet search engines. This results in some high-quality websites not appearing at the top of an internet search. Therefore, parents risk missing useful information relevant to their child's diagnosis. Healthcare professionals can use objective scoring of patient information websites to empower their patients to seek higher quality information.

Isolated Unilateral Frontosphenoidal Suture Synostosis in Six Patients: Lessons Learned in Diagnosis and Treatment.

INTRODUCTION: Due to the rarity of isolated frontosphenoidal suture synostosis clinical diagnosis can be challenging. This study of 6 patients aims to review the clinical, radiological findings, and operative techniques used to correct the underlying pathology. METHODS: Patients with isolated frontosphenoidal suture craniosynostosis were selected from a retrospective review of 88 patients with unicoronal synostosis treated during a 3-year period. Two-dimensional photography of patients' soft tissue morphology from the vertex view allowed assessment of the following morphology: frontal bossing, brow depression, nasal tip deviation, and ear position. Quantitative measure of the extent of bony deformity was measured using various angles measured from two-dimensional axial views of computerized tomography scans. Last, technical variations in correction of isolated frontosphenoidal craniosynostosis were collected from operative notes. RESULTS: On the side of isolated frontosphenoidal craniosynostosis, contralateral bossing and ipsilateral brow depression was present in all 6 patients. Ipsilateral nasal tip deviation was seen in 3 out of the 6 patients. Ear position was symmetrical in the cranial-caudal and anterior-posterior axes. No radiological evidence of harlequin deformity was seen on skull X-ray in all 6 patients, but computerized tomography scans demonstrated isolated frontosphenoidal suture craniosynostosis. The angle drawn between the foramen magnum, sella turcica, and anterior cribriform plate in 3 of 6 patients showed deflection of the anterior cranial fossa opposite to the side of isolated frontosphenoidal suture craniosysnotosis. There was no difference in the angle between the petrosal pyramid and the midline. In all patients, operative technique involved taking a deeper fronto-orbital bandeau to capture and reshape the pathological suture. CONCLUSIONS: In isolated frontosphenoidal suture craniosynostosis, contralateral bossing and ipsilateral flattening of the forehead were the most consistent clinical features with nasal tip deviation away from the side of pathology less consistent. Ear position is unaffected. Measurements of various angles of the skull base were not consistent. A deeper vertical osteotomy at the site of isolated frontosphenoidal suture craniosysnotosis on removing the fronto-orbital bandeau was 1 operative technical variation.

The Quality of Life in Adult Patients With Syndromic Craniosynostosis From Their Perspective.

INTRODUCTION: Clinical intuition may perceive those adults with syndromic craniosynostosis to have a lower quality of life (QOL) compared with the normative population. Classification of facial difference; standardization of cognitive capacity and selection of an appropriate QOL measurement tool provides a less intuitive and more evidence-based method of assessing QOL in this particular group of patients. METHODS: Adults with syndromic craniosynostosis treated by the same surgeons underwent Whittaker Classification for facial difference by an independent observer. Neuropsychology screening ensured cognitive ability in patients for independent answering of a World Health Organization QOL postal questionnaire. Data analysis using descriptive and z test statistics allowed comparison to nonsyndromic adult United Kingdom data provided by the World Health Organization. RESULTS: Forty adult patients met authors' inclusion criteria. Whittaker Classification of facial difference ranged from I (31 patients) to II (8 patients) and III (1 patient). Quality of life showed no correlation to facial difference. Quality of life was better in the physical, psychological, and environmental domains compared with the normative adult UK population. However, no statistical difference was found in the social domain. Female Apert syndrome patients had a worse QOL than males in the social domain. CONCLUSIONS: The counterintuitive findings show that adult syndromic patients with similar cognitive capacity perceive their quality of life as being above that experienced in a normative UK nonsyndromic population with no correlation to the degree of facial difference.

Amelioration of Chiari type 1 malformation and syringomyelia following posterior calvarial distraction in Crouzon's syndrome--a case report.

Multisutural craniosynostosis as seen in Crouzon's syndrome can result in raised intracranial pressure, Chiari malformation (CM) and syringomyelia. Posterior calvarial distraction (PD) is a technique for addressing cranio-cephalic disproportion, and this case report describes the reversal of both CM and syrinx in a 6-year-old child who underwent PD initially for raise intracranial pressure.

Calvarial remodelling for sagittal synostosis: does fibrin glue (Tisseel) reduce post-operative blood transfusion requirements?

INTRODUCTION: Calvarial remodelling for sagittal synostosis is extensive surgery and is associated with potential risks; the most significant of these is blood loss. MATERIALS AND METHODS: We studied 16 patients undergoing calvarial remodelling by the same surgical team over a 12-month period to determine whether scalp closure with fibrin glue (Tisseel) could decrease post-operative bleeding and the need for blood transfusion. In the last 5 months of the period studied, fibrin glue (Tisseel) was used and six out of the 16 patients had their wound closure assisted by this means. Data was prospectively collected on age at surgery, the estimated peri-operative blood loss, the volume of blood transfused intra-operatively, the volume drained in the first 8 h post-operatively, the total post-operative drainage and the volume of any post-operative blood transfusion required. RESULTS AND DISCUSSION: The two groups were comparable with a similar mean age at surgery, estimated peri-operative blood loss and intra-operative blood transfusion requirements. The volume drained in the first 8 h post-operatively was 172 ml in the treated group compared to 246 ml in the untreated group (p < 0.02) and the total post-operative drain volume was 301 ml compared to 441 ml (p < 0.01). None out of the six patients treated with fibrin glue required post-operative transfusion compared to two out of ten in the untreated group. The use of fibrin glue has enabled us to reduce post-operative bleeding and the need for post-operative blood transfusion.

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

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

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

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

The role of alcohol in maxillofacial trauma: a comparative retrospective audit between the two centers.

Alcohol abuse and maxillofacial trauma, particularly that due to interpersonal violence, have a well-established relationship in the literature. We present a retrospective audit comparing the role of alcohol in maxillofacial trauma between Selly Oak Hospital in Birmingham, United Kingdom and Westmead Hospital in Sydney, Australia, and the association between alcohol involvement and patient demographics, including age, sex, marital status, and employment status. Also presented are the differences between the two centers in terms of mechanisms and types of injuries and the locations where these injuries were sustained. Alcohol was involved in 34.78 and 30.77% of patients at Westmead and Birmingham, respectively. A multiple logistic regression analysis revealed a reduced likelihood of alcohol involvement in episodes of maxillofacial trauma where patients were unemployed (P=.04), and where injuries were sustained secondary to mechanisms other than assault (P<.001) and in locations other than pubs and nightclubs (P=.024). There appeared to be no statistically significant contribution to the likelihood of alcohol involvement by treating center, marital status, patient sex, or age. Alcohol continues to be a strong driving factor in cases of maxillofacial trauma, particularly those due to alleged assault, with a typical patient demographic of the gainfully employed frequenting drinking establishments being most at risk for alcohol-related trauma, most commonly sustaining their injuries secondary to assault. Identifying patient groups most at risk is a key step in developing public health strategies aimed at prevention, and our findings would appear to suggest this group of patients as being the most worthwhile to target with measures aimed at reducing alcohol-fueled maxillofacial trauma.

Management of the giant frontal sinus--a simple method to improve cosmesis.

Frontal bossing can be caused by many craniofacial syndromes, however an enlarged frontal sinus is a rare cause. Because of this the management of this condition is variable. One option is to remove the anterior wall of the frontal sinus and dividing this into segments which are then fixed in the desired position using miniplates.This however may prove difficult in thin bone and in these cases the periosteum can be left intact to splint the fragments which are then repositioned using resorbable sutures.