Three patterns of fronto-orbital remodeling for metopic synostosis: comparison of cranial growth outcomes.

BACKGROUND: The authors compared cranial growth across three patterns of fronto-orbital remodeling for metopic synostosis. METHODS: The authors reviewed all patients who underwent fronto-orbital remodeling for isolated metopic synostosis between 2006 and 2009. Inclusion criteria consisted of patients with preoperative, short-term postoperative (4 to 12 months), and long-term postoperative (>36 months) three-dimensional photographs. Patients were categorized by fronto-orbital remodeling pattern: group 1, retrocoronal; group 2, partial coronal; and group 3, precoronal. Head circumference, minimum frontal breadth (ft-ft), and maximum cranial length were measured by three-dimensional photographs, converted to standard Z scores, and compared. RESULTS: Thirty-one patients met inclusion criteria (group 1, n=12; group 2, n=10; and group 3, n=9). Group 1 presented with the greatest phenotypic severity. From preoperative to short-term postoperative assessment, head circumference Z scores rose for group 1 but dropped for groups 2 and 3, and the three groups demonstrated equivalent increases in minimum frontal breadth Z scores. From short-term to long-term postoperatively, the three groups demonstrated similar stability in head circumference Z scores but decreased minimum frontal breadth Z scores. From preoperatively to long-term postoperatively, head circumference Z scores rose for group 1 but fell for groups 2 and 3 (change in Z score, 0.5, -0.5, and -0.7, respectively; p=0.06) and the three groups demonstrated equivalent drops in minimum frontal breadth Z scores. Across preoperative to short-term postoperative and preoperative to long-term postoperative assessment, group 1 displayed the least drop in maximum cranial length Z scores. CONCLUSIONS: Retrocoronal patterns of fronto-orbital remodeling provide long-term gains in head circumference percentile and the least growth impairment in cranial length. Irrespective of osteotomy design, expansion in frontal breadth relapses significantly over time. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Total cranial vault remodeling for isolated sagittal synostosis: part I. Postoperative cranial suture patency.

BACKGROUND: Total vault reconstruction addresses all phenotypic aspects of scaphocephaly. The clinical implications of remodeling across open cranial sutures, however, remain unclear. The purpose of this study was to assess patency of unaffected sutures following total vault remodeling for isolated sagittal synostosis. METHODS: The authors reviewed routine postoperative computed tomographic scans of patients who underwent total vault remodeling for isolated sagittal synostosis between 2004 and 2008. Sutural patency was scored by a single reviewer as follows: 0 = closed, 1 = partial, and 2 = open. Individual suture scores were tallied for a total sutural patency score. Computed tomographic scans were stratified by postoperative time and craniofacial surgeon. RESULTS: Forty-two patients met the inclusion criteria. Individual sutural closure rates were 42.6, 38.3, 74.5, and 74.5 percent for right coronal, left coronal, right lambdoidal, and left lambdoidal sutures, respectively. Lambdoidal sutures had a significantly higher rate of closure than coronal sutures (OR(Closure), 4.3; 95 percent CI, 2.3 to 8.0; p < 0.001); lambdoidal patency significantly changed over time (χ2 = 9.9, p = 0.04). Across craniofacial surgeons, coronal and lambdoidal patency were equivalent. The total sutural patency score did not significantly correlate with postoperative time, surgical age, preoperative cephalic index, or craniofacial surgeon. CONCLUSIONS: Total vault remodeling for isolated sagittal synostosis results in a high degree of secondary craniosynostosis. Lambdoidal sutures are especially prone to closure, with their patency diminishing over time. At this time, radiographic fusion of adjacent sutures following surgery has not been related to any difference in head shape. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Longitudinal study of the neurodevelopmental characteristics of treated and untreated nonsyndromic craniosynostosis in infancy.

PURPOSE: Nonsyndromic craniosynostosis (NSC) are a group of congenital disorders sharing premature fusion of one or more of the cranial sutures that restricts and distorts growth of the skull and underlying brain. This study examined the neurodevelopmental sequelae of NSC both prior to and following reconstructive cranial surgery. METHODS: Sixty-four consecutive referrals with mixed forms of untreated NSC aged 4 to 16 months (M = 8.9, SD = 2.9) comprised the pre-operative cohort. Forty-four of these patients aged 6 to 32 months (M = 21.2, SD = 4.5) underwent post-operative developmental evaluation. Neurodevelopmental function was assessed with the mental (Mental Development Index) and motor (Psychomotor Development Index) scales of the Bayley Scales of Infant Development-2nd edition. RESULTS: Children with untreated NSC displayed significantly lower mental (M = 97.5) and motor (M = 87.7) scores than normative expectations, with the distribution of scores also differing significantly from the normative distribution. Post-operatively, children continued to display significantly lower mental (M = 89.5) and motor (M = 88.0) abilities, with mental abilities falling significantly lower than pre-operative levels. An increased prevalence of severe motor delay was found, and no child displayed accelerated development. Subgroup comparisons revealed no differences in mental or motor skills between the primary diagnostic subtypes (sagittal and metopic synostosis) both prior to and following corrective surgery. CONCLUSIONS: NSC is associated with an increased incidence of developmental delay in both treated and untreated conditions. Timing of surgery appears unrelated to developmental outcome.

Delayed sagittal sinus tear: a complication of spring cranioplasty for sagittal craniosynostosis.

Spring cranioplasty is now a well-established surgical technique in the treatment of sagittal craniosynostosis. It is widely regarded as a less invasive modality compared with operations such as cranial vault remodeling. Indeed, very few complications have been described in the literature in association with spring cranioplasty. We present a case of delayed sagittal sinus tear with hemorrhage following spring cranioplasty in a 4-month-old patient with sagittal craniosynostosis. Likely causes of the injury are discussed highlighting sagittal sinus injury as a potential risk of spring cranioplasty.

Analysis of morbidity and mortality in surgical management of craniosynostosis.

Multidisciplinary care involving plastic surgery and neurosurgery is generally accepted as optimal to manage craniosynostosis to avoid complications and to identify patients at risk. We conducted a retrospective 30-year review of craniosynostosis surgery at a single major craniofacial institute to establish the rate and predictors of complications. Medical records of 796 consecutive patients who underwent primary surgery for craniosynostosis from 1981 to 2010 at our institute were analyzed for complications. Complications were defined as intraoperative and postoperative adverse events requiring changed management. Reoperation was defined as a repeat transcranial procedure. Multivariate logistic regression was used to identify predictors for complications or revision. Across the years, the procedures evolved from technically simple to complex, which increased complications but better outcomes. Complications occurred in 111 patients (14%), and 33 (5.4%) needed major revision. Multivariate analysis identified multisuture and syndromic craniosynostosis, more recent surgeries, younger age (<9 months), spring-assisted cranioplasty, longer surgery, and greater transfusion as predictors of complications. Patients with syndromic and multisutural craniosynostosis and those operated on younger than 9 months had increased risk of major revision surgery for regression to phenotype. Our experience over 30 years indicates that pediatric transcranial craniosynostosis surgery can be safely carried out in our tertiary referral center. There were no deaths from primary surgery, and complication and reoperation rates mirror those of other published studies. Syndromic and complex craniosynostosis predicted both complications and need for major revision. Spring cranioplasty was associated with higher complications. Overall results support a recommended age for craniosynostosis surgery between 9 and 12 months.

Changing epidemiology of nonsyndromic craniosynostosis and revisiting the risk factors.

Recent studies in Europe and the United States report increased incidence of metopic synostosis. Whether a similar trend had occurred in Australia remains unknown. This research aimed to determine changes in incidence and subtypes of craniosynostosis in Victoria and to identify perinatal risk factors. A retrospective audit of patients (n = 522) presenting to the Royal Children's Hospital in Melbourne with nonsyndromic craniosynostosis from 1982 to 2008 was undertaken. Perinatal data were sourced from the Victorian Perinatal Data Collection. The changes in incidence of craniosynostosis subtypes were calculated based on Poisson regression, and risk factors for craniosynostosis and subtypes were analyzed by univariate logistic regression analysis. The prevalence of nonsyndromic craniosynostosis was 3.1 in 10,000 live births in Victoria. On average, the incidence of nonsyndromic craniosynostosis increased by 2.5% per year among Victorian live births. Over 25 years, metopic synostosis incidence significantly increased by 7.1% per year in the population of Victoria, outpacing other subtypes. The risk factors for metopic synostosis include being male, multiple births (ie, twins), preterm gestation, low birth weight, high maternal age, and emergency cesarean birth. This study revealed a true increase in incidence of metopic synostosis in Victoria, which could be a result of increased frequency of multiple births, preterm gestation, low birth weight, and high maternal age in the Victorian population from 1982 to 2008. The incidence of other nonsyndromic craniosynostoses, which include sagittal, unicoronal, and multisutural craniosynostoses, however, has remained unchanged.

Neurodevelopmental functioning of infants with untreated single-suture craniosynostosis during early infancy.

PURPOSE: Single-suture craniosynostosis (SSC) is a congenital craniofacial disorder, in which premature fusion of one of the skull sutures restricts and distorts growth of the cranium and underlying brain. This disorder of prenatal onset occurs during a critical phase of rapid growth and development of the immature brain. Craniosynostosis carries a known risk of developmental impairment. The neurodevelopmental sequelae of SSC prior to treatment remains however incompletely understood. This study sought to determine the neurodevelopmental sequelae of untreated single-suture craniosynostosis during early infancy. METHODS: Fifty-six consecutive patients with unoperated SSC (sagittal, metopic and unicoronal) comprised the sample cohort. Patients were aged between 4 and 16 months (M = 8.9 months, SD = 2.9 months). Neurodevelopmental functioning was assessed with the mental (Mental Development Index) and motor (Psychomotor Development Index) scales of the Bayley Scales of Infant Development, second edition. RESULTS: Children with SSC displayed significantly lower mean mental (M = 97.7, SD = 6.7, p < 0.05) and motor (M = 87.7, SD = 13.0, p < 0.001) scores than normative population averages. The distribution of these scores also differed significantly from the normative distribution; an increased rate of significant motor developmental delay was found, and none of the children displayed accelerated development. Subgroup comparisons between the primary diagnostic subtypes in this sample revealed no significant differences in mental or motor skill functioning. CONCLUSIONS: Untreated SSC is associated with an increased incidence of developmental delay during early infancy, with motor skills appearing the most vulnerable to impairment during this developmental phase.

Measuring cranial vault volume with three-dimensional photography: a method of measurement comparable to the gold standard.

The gold standard for measuring cranial vault volume is a computed tomography (CT) scan. Computed tomography scans in the pediatric population carry the significant risk of inducing a malignancy later in life. In a novel study, we show that a three-dimensional (3D) photograph can be used to measure and track cranial vault volume changes in craniosynostosis patients. In a study of adequate power as determined by pre hoc analysis, we demonstrate a greater than 0.91 correlation coefficient between volume as measured by CT and 3D photograph in more than 70 patients presenting to a tertiary craniofacial center (P < 0.001). Volume above a plane running through the lateral canthus (ex) and tragus (t) sag-ex-t on the digital 3D photograph differs from CT by a mean percentage difference of 31.9% (SD, 14.0%; 95% confidence interval, P < 0.0001). A linear regression model was used to determine the equation describing the relationship between volume as measured by 3D photograph and CT; with this equation, the absolute volume of the cranial vault is easily determined using only the 3D image. Interrater reliability is high (>0.99, ICC). The ability to measure cranial vault volume from the 3D photograph adds an important dimension to the complete and objective analysis of skull growth and postoperative change.

Quantitative morphometric outcomes following the Melbourne method of total vault remodeling for scaphocephaly.

The Melbourne method of total vault remodeling was developed at The Royal Children's Hospital (Melbourne) to address all phenotypic aspects of scaphocephaly. To quantitatively evaluate this technique, a retrospective analysis was performed on 33 consecutive patients who underwent the Melbourne procedure between October 2004 and June 2007. To monitor outcomes, three-dimensional digital surface photography was used, obtaining 4 anthropometric measurements (cranial length, cranial width, head circumference, auricular head height) preoperatively and postoperatively. To assess the effect on continuing cranial vault development, cranial volume was calculated with computed tomography. Anthropometric measurements were obtained in 27 patients (81.8%), with a mean postoperative follow-up of 8.9 months. Cranial index (width/length) demonstrated an 11.1% improvement. The mean head circumference in the scaphocephaly group preoperatively remained larger than the normative population postoperatively, although the magnitude of difference was decreased, whereas the mean auricular head height demonstrated a 10.5% increase postoperatively, remaining higher than the normative population. Cranial volume was calculated in 30 patients (91%), with a mean postoperative follow-up of 7.9 months. This demonstrated that the mean intracranial volume was significantly higher in the scaphocephaly group preoperatively, and this difference was maintained postoperatively. These results support our belief that the Melbourne procedure is a technique that may be used to correct all phenotypic aspects of scaphocephaly, with no apparent evidence for a detrimental effect on cranial growth.

Complete correction of severe scaphocephaly: the Melbourne method of total vault remodeling.

BACKGROUND: A new method of total calvarial remodeling for correcting significant scaphocephaly is described, and experience with the first 30 consecutive patients is presented. METHODS: Thirty consecutive patients (22 boys and eight girls) with moderate to severe scaphocephaly who underwent total calvarial remodeling using the Melbourne technique were analyzed retrospectively. Mean age at surgery was 7.5 months (range, 4 to 18 months). All had significant frontal and occipital deformities, bitemporal narrowing, a low cranial index, an anteriorly located vertex, and a narrow posteroinferiorly sloping posterior cranium. Clinical photographs, computed tomographic imaging, and cranial index were evaluated preoperatively and postoperatively. A sufficient follow-up period in 17 patients enabled the measurement of changes in cranial indices. RESULTS: A dramatic improvement in head shape was confirmed subjectively by clinical examination and photographs and, in 17 patients, objectively by analyses of the cranial indices. In these 17 patients, mean preoperative cranial index was 64.6 percent preoperatively (range, 56 to 69.3 percent) and increased to a mean of 77.5 percent postoperatively (range, 72 to 81 percent). The mean increase in cranial index was 12.9 percent (range, 9.5 to 19 percent). Among the 30 patients, overall complications were major in one patient (3.3 percent) (presumed air embolus) and minor in three (10 percent). CONCLUSIONS: The Melbourne technique of total vault remodeling consistently achieves a virtually normal head shape. After analysis of the first 30 cases, the authors recommend this as their technique of choice for severe scaphocephaly when the full constellation of deformities is present.

Aplicações da fotografia em três dimensões na cirurgia plástica / Three dimensional photography applications in plastic surgery

Sistemas digitais sofisticados de fotografia em três dimensões melhoraram muito a captura de imagens do corpo humano. A introdução de um sistema rápido, não-invasivo e sem radiação ionizante, e esses avanços possibilitam novas aplicações da imagem em três dimensões na cirurgia plástica. Desde 2004, o Royal Children's Hospital (RCH) de Melbourne utiliza o sistema de fotografia digital em três dimensões 3dMD, que foi montado num centro exclusivamente dedicado (Centro de imagens da Batten Foundation). Desde então, uma equipe multidisciplinar trabalha para desenvolver técnicas mais apuradas para coletar e analisar informações para que se possa titrar pleno proveito dessa poderosa tecnologia. Este artigo descreve uma série de aplicações clínicas onde a captura de dados em três dimensões e sua análise têm sido úteis.

Long-term outcomes of surgical tongue reduction in Beckwith-Wiedemann syndrome.

BACKGROUND: Surgical tongue reduction is often performed in Beckwith-Wiedemann syndrome when macroglossia results in abnormal tongue function or cosmesis; however, no published studies have examined the long-term outcomes of this procedure. METHODS: Patients older than 18 years with Beckwith-Wiedemann syndrome who had previously undergone surgical tongue reduction at the Royal Children's Hospital in Melbourne underwent assessment of speech and tongue function, mobility, sensation, and cosmesis. These assessments were performed by questionnaire on 11 subjects and by formal medical and speech pathology review in four of these 11 subjects. RESULTS: Eleven patients aged 19 to 31 years completed responses by means of questionnaire. The most common self-reported abnormalities were continued disproportionate tongue bulk (91 percent), abnormal tongue appearance (82 percent), specific speech sound errors (73 percent), and a short tongue tip (55 percent). Formal assessments investigated speech, swallowing, taste, and tongue mobility in four quite different subjects. One of these four subjects had no detectable deficits in tongue function. All 11 patients were of normal intelligence and did not report significant difficulties in their day-to-day life that were attributable to their macroglossia or tongue reduction surgery. CONCLUSIONS: Pediatric patients with symptomatic macroglossia requiring surgical tongue reduction may not achieve complete normality in tongue function and appearance in adulthood. It is important that surgical tongue reduction addresses the global nature of the macroglossia and aims to retain a tapered tongue tip with length sufficient to permit normal tongue movements.

Prospective analysis of the outcome of surgical management of drooling in the pediatric population: a 10-year experience.

BACKGROUND: Sialorrhea is a common problem in the neurologically impaired pediatric patient and surgery is the mainstay of treatment for severely affected patients refractory to conservative or medical treatment. METHODS: The results of the surgical protocol used since 1993 at the Royal Children's Hospital in Melbourne, Australia, are reported. Seventy-two patients underwent bilateral submandibular duct transposition combined with bilateral sublingual gland excision. Patients were analyzed prospectively and reviewed at 1, 6, and 12 months, then at 2 and 5 years postoperatively. RESULTS: Of 67 patients with 2 years of complete follow-up, the median score for the frequency of drooling fell from 4.0 to 2.9 (p < 0.001) and the median severity-of-drooling score decreased from 4.8 to 3.0 (p < 0.001). There was a fall in the number of clothing/bib changes from a median of four per day to zero (p < 0.0001). Of 41 patients followed to 5 years, both the median frequency and severity of drooling scores remained at 3. Overall the majority of patients benefited from surgery. Thirteen patients (18 percent) experienced complications which were major in 9 percent. CONCLUSION: We have found bilateral sublingual gland excision in combination with bilateral submandibular duct transposition to be superior to bilateral submandibular duct transposition plus parotid duct ligation. The surgical treatment of sialorrhea continues to be a rewarding experience for our multidisciplinary team.

Early postoperative efficacy of fibrin glue in face lifts: a prospective randomized trial.

Fibrin glue is increasingly finding use in plastic surgery at the clinical and basic science level. The authors conducted a prospective, nonblinded, randomized, controlled trial in 30 patients undergoing face lifts to examine the efficacy of fibrin glue in reducing postoperative wound drainage, hematomas, and, in particular, the degree of ecchymosis and edema at 24 hours and at 8 days. Patients were their own controls and were randomized to have the glue on one side of their face only to compare the glued and unglued sides. The patients ranged in age from 42 to 72 years (mean age, 60 years). There was one major hematoma requiring surgical evacuation. In the remaining 29 patients, the mean drainage on the glued side was 26 ml, compared with 33.5 ml on the control, unglued side. This difference was statistically significant numerically (p = 0.037) but was not thought to be surgically significant. Comparing scores among grades of hematomas, ecchymosis, and edema, there were minimal differences between the glued and unglued sides. This study suggests that fibrin glue may not be as beneficial as previously thought in reducing ecchymosis and edema in the early postoperative period after face lifts, and its future role is discussed.

Complications related to cranial vault surgery.

Craniofacial surgery is currently firmly established as a subspecialty, and operations previously regarded as exceptional are considered routine. A range of complications still can occur, however, from minor to devastating in nature. As transcranial surgery is more frequently performed, craniofacial units must take steps to prevent such problems, recognize them early, and treat them expeditiously. Infection and uncontrollable bleeding are the two most feared complications. Craniofacial teams should maintain accurate audit data on morbidity and mortality and clinical outcomes to monitor complication rates. This article reviews the literature on craniofacial complications and makes recommendations for their avoidance and treatment based on the 23-year experience with transcranial pediatric surgery at the Royal Children's Hospital, Melbourne, Australia.