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.

Midbrain and thalamic haemorrhage as first presentation of intracerebral glioma.

A case of spontaneous intracerebral haemorrhage (midbrain and thalamic, with intraventricular extension) as the first presentation of an anaplastic astrocytoma is presented. Multiple CT scans and cerebral angiography failed to identify any vascular or neoplastic cause for the haemorrhage, and a presumptive diagnosis of hypertensive haemorrhage was made. Shunting of hydrocephalus was followed by early clinical improvement. However, delayed progressive deterioration necessitated MRI scan, which demonstrated a mass lesion in the basal ganglia and midbrain. This was subsequently found to be anaplastic astrocytoma on biopsy. The literature regarding this uncommon presentation of spontaneous intracerebral haemorrhage from an occult brain tumour is reviewed. The need for investigation and close follow-up of presumed hypertensive haemorrhage is emphasised by this case.

Coexistence of aneurysmal subarachnoid haemorrhage and pituitary apoplexy: Case report and review of the literature.

A case of aneurysmal subarachnoid haemorrhage with associated haemorrhagic infarction of a growth hormone secreting pituitary macroadenoma is presented. The subarachnoid haemorrhage was not identifiable on CT, but was apparent on MRI. Angiography revealed a 7mm right posterior communicating aneurysm, a 3mm left A1 segment anterior cerebral aneurysm, and vasospasm. Surgery was performed through a right pterional/subfrontal approach, clipping both aneurysms and debulking the tumour. The left A1 aneurysm was the site of subarachnoid haemorrhage. There was evidence of haemorrhagic infarction of the pituitary tumour. Although rupture of an aneurysm into a pituitary tumour has been previously reported, this is the first case reported of aneurysmal subarachnoid haemorrhage with coexisting pituitary apoplexy where the aneurysm had not bled directly into the pituitary tumour. The literature regarding the association between pituitary tumours and aneurysm is reviewed.