Jordan Terner

The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis.

Background: The relationship between surgical age and long-term neuropsychological outcomes in sagittal-suture craniosynostosis remains equivocal. Whole-vault cranioplasty and strip craniectomy are performed at various times in individuals with sagittal-suture craniosynostosis. This study used comprehensive neurological testing to examine the relationship between age at time of surgery and long-term neuropsychological function. Methods: Seventy sagittal-suture craniosynostosis patients who had previously undergone either whole-vault cranioplasty or strip craniectomy were examined divided into three groups: treatment before 6 months (n = 41), between 6 and 12 months (n = 21), and after 12 months (n = 8). To examine long-term cognitive functioning, participants between the ages of 5 and 25 years underwent neurodevelopmental tests to evaluate intelligence, achievement, and learning disabilities. Results: Compared with those treated between 6 and 12 months and after 12 months, patients who underwent surgery before 6 months demonstrated higher full-scale IQ (p < 0.01) and verbal IQ (p < 0.01). Patients who received surgery before 6 months also demonstrated superior abilities in word reading (p < 0.01), reading comprehension (p < 0.01), spelling (p < 0.01), and numerical operations (p < 0.05) relative to those who had surgery between 6 and 12 months old. A statistically significant higher percentage of patients treated after 6 months had one or more reading-related learning disabilities as compared with those undergoing earlier surgery. Conclusions: This study suggests that surgery before 6 months old results in improved long-term neurological outcomes. Future studies should examine how the technique of surgery impacts these neuropsychological measures. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

An analysis of mandibular volume in treacher collins syndrome.

1. Steinbacher DM, Bartlett SP. Relation of the mandibular body and ramus in Treacher Collins syndrome. J Craniofac Surg. 2011;22:302–305. 2. Kaban LB, Mulliken JB, Murray JE. Three-dimensional approach to analysis and treatment of hemifacial microsomia. Cleft Palate J. 1981;18:90–99. 3. Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects Orig Artic Ser. 1969;1:120–129. 4. Steinbacher DM, Gougoutas A, Bartlett SP. An analysis of mandibular volume in hemifacial microsomia. Plast Reconstr Surg. 2011;127:2407–2412. 5. Terner JS, Travieso R, Chang C, Bartlett SP, Steinbacher DM. An analysis of mandibular volume in Treacher Collins syndrome. Plast Reconstr Surg. 2011;129:751e–753e.

A range of condylar hypoplasia exists in Treacher Collins syndrome.

PURPOSE Temporomandibular joint malformation is a complex deformity in Treacher-Collins syndrome (TCS); however, it is not well characterized. This study aimed to better clarify this pathology by volumetrically assessing the mandibular condyle in patients with TCS compared with normal controls and the relative contribution of the condyle to hemimandibular volume. MATERIALS AND METHODS A retrospective, cross-sectional analysis of pediatric patients with TCS and unaffected controls was performed. The study sample was comprised of Treacher Collins patients. The predictor variable in this study was disease status (TCS diagnosis vs control), and the outcome variable was condylar volume. Demographic information was collected, and 3-dimensional computed tomographic data were analyzed by computerized segmentation (Materialise). Volumes were obtained for TCS condyles and compared with age-matched controls using the Student t test. RESULTS Three-dimensional computed tomographic scans were identified in 10 patients with TCS (20 sides) and 14 control subjects (28 sides). The TCS group included 4 female and 6 male patients (age, 0.3 to 213 mo; average age, 66.5 mo). The control cohort included 7 female and 7 male subjects (average age, 68.8 mo). Evaluation of the mandibular condyle showed that patients with TCS had a significantly smaller condylar volume than control patients (TCS, 178.28 ± 182.74 mm(3); control, 863.55 ± 367.20 mm(3); P < .001). Additional intragroup analysis showed no significant differences between the left and right condylar volumes in the TCS group (P = .267). In addition, the condyle for patients with TCS represented a smaller proportion of hemimandibular volume compared with controls (1.37% vs 4.19%, respectively; P < .001). CONCLUSIONS The results of the this study suggest that condylar volumes are significantly smaller in patients with TCS compared with age-matched controls, and the condyle represents a smaller fraction of the total mandibular volume for patients with TCS than in unaffected children. In addition, there is considerable variability of condylar size in patients with TCS. These facts portend treatment decisions because a functional temporomandibular joint is necessary and may need to be reconstructed as a first stage before effective implementation of distraction procedures.

On Bernard Sarnat's 100th birthday: pathology and management of craniosynostosis.

Abstract The focus on nonsyndromic craniosynostosis, the most common type of isolated craniosynostosis, is sagittal, followed by unilateral coronal, bilateral coronal, metopic, and lambdoid, in order of decreasing frequency. Certain forms of craniosynostosis display a sex predilection. For example, boys outnumber girls in a 4:1 ratio in sagittal synostosis, but girls outnumber boys in a 3:2 ratio in unilateral coronal synostosis. Other forms, such as metopic, lambdoid, and bilateral coronal synostosis, demonstrate no sex dominances tract.

The Perichondrium in Auricular Melanomas: Implications for Surgical Management

Objective Auricular melanomas are now considered less aggressive than originally thought, but those located on the conchal bowl and posterior ear can create particular challenges for reconstruction. Given the paucity of cases, no standardized recommendations exist for tumor resection. In this study, we provide a pathologic basis in support of conservative tumor resection along the perichondrial plane. Study Design Case series with review of pathology and medical records. Setting Academic tertiary referral center. Subjects and Methods We retrospectively reviewed all ear melanomas from the archives of Yale Dermatopathology and Surgical Pathology laboratories between 1987 and 2009. Cases of melanoma in situ or malignant melanoma of the earlobe were excluded. Results Fifty-one cases were included in the study. Patients’ age ranged from 26 to 94 years, with a mean (SD) of 58.9 (17.5) years. The male to female ratio was of 5.4:1. Melanomas were distributed similarly between right and left ears, at 52% and 48%, respectively. The most common location was the helix (74.5%). The Breslow depth ranged from 0.19 to 11 mm, with a mean (SD) of 1.64 (1.6) mm. The lesion-perichondrium distance ranged from 0 to 8.12 mm, with a mean (SD) of 1.11 (1.1) mm. Notably, the perichondrium was not invaded in any of the cases. Conclusion Based on the histopathologic characteristics of our cases, the perichondrium can be considered an effective barrier and therefore a biological plane for wide local resection in some cases of auricular melanomas, allowing surgeons to achieve negative margins without necessarily sacrificing underlying cartilage.

Combined metopic and sagittal craniosynostosis: is it worse than sagittal synostosis alone?

OBJECT Combined metopic and sagittal craniosynostosis is a common variant of the nonsyndromic, multiplesuture synostoses. It is unknown whether this combined form causes reduced intracranial volume (ICV) and potentially more brain dysfunction than sagittal synostosis alone. This study is a volumetric comparison of these 2 forms of craniosynostosis. METHODS The authors conducted a retrospective chart and CT review of 36 cases of isolated sagittal synostosis or combined metopic and sagittal synostosis, involving patients seen between 1998 and 2006. Values were obtained for the intracranial compartment, brain tissue, CSF space, and ventricular volumes. Patients with craniosynostosis were then compared on these measures to 39 age- and sex-matched controls. RESULTS In patients with isolated sagittal synostosis and in those with combined metopic and sagittal synostosis, there was a trend toward smaller ICV than in controls (p < 0.1). In female patients older than 4.5 months of age, there was also a trend toward smaller ICV in patients with the combined form than in those with sagittal synostosis alone (p < 0.1), and the ICV of patients with the combined form was significantly smaller than the volume in controls in the same age group (p < 0.05). Brain tissue volume was significantly smaller in both patient groups than in controls (p < 0.05). Ventricular volume was significantly increased (compared with controls) only in the patients with isolated sagittal synostosis who were younger than 4.5 months of age (p < 0.05). Overall CSF space, however, was significantly larger in both patient groups in patients younger than 4.5 months of age (p < 0.05). CONCLUSIONS These findings raise concerns about intracranial and brain volume reduction in patients with sagittal and combined metopic and sagittal synostoses and the possibility that this volume reduction may be associated with brain dysfunction. Because the ICV reduction is greater in combined metopic and sagittal synostosis in patients older than 4.5 months of age than in sagittal synostosis in this age group, the potential for brain dysfunction may be particularly true for these younger infants.

Direct brain recordings reveal impaired neural function in infants with single-suture craniosynostosis: a future modality for guiding management?

BackgroundPatients with single-suture craniosynostosis (SSC) are at an elevated risk for long-term learning disabilities. Such adverse outcomes indicate that the early development of neural processing in SSC may be abnormal. At present, however, the precise functional derangements of the developing brain remain largely unknown. Event-related potentials (ERPs) are a form of noninvasive neuroimaging that provide direct measurements of cortical activity and have shown value in predicting long-term cognitive functioning. The current study used ERPs to examine auditory processing in infants with SSC to help clarify the developmental onset of delays in this population. MethodsFifteen infants with untreated SSC and 23 typically developing controls were evaluated. ERPs were recorded during the presentation of speech sounds. Analyses focused on the P150 and N450 components of auditory processing. ResultsInfants with SSC demonstrated attenuated P150 amplitudes relative to typically developing controls. No differences in the N450 component were identified between untreated SSC and controls. ConclusionsInfants with untreated SSC demonstrate abnormal speech sound processing. Atypicalities are detectable as early as 6 months of age and may represent precursors to long-term language delay. Electrophysiological assessments provide a precise examination of neural processing in SSC and hold potential as a future modality to examine the effects of surgical treatment on brain development.

Brain electrophysiology reveals intact processing of speech sounds in deformational plagiocephaly.

Background: The prevalence of deformational plagiocephaly has risen dramatically in recent years, now affecting 15 percent or more of infants. Prior research using developmental scales suggests that these children may be at elevated risk for developmental delays. However, the low positive predictive value of such instruments in identifying long-term impairment, coupled with their poor reliability in infants, warrants the development of methods to more precisely measure brain function in craniofacial patients. Event-related potentials offer a direct measure of cortical activity that is highly applicable to young populations and has been implemented in other disorders to predict long-term cognitive functioning. The current study used event-related potentials to contrast neural correlates of auditory perception in infants with deformational plagiocephaly and typically developing children. Methods: Event-related potentials were recorded while 16 infants with deformational plagiocephaly and 18 nonaffected controls passively listened to speech sounds. Given prior research suggesting their association with subsequent functioning, analyses focused on the P150 and N450 event-related potential components. Results: Deformational plagiocephaly patients and normal controls showed comparable cortical responses to speech sounds at both auditory event-related potential components. Conclusions: Children with deformational plagiocephaly demonstrate neural responses to language that are consistent with normative expectations and comparable to those of typical children. These results indicate that head shape deformity secondary to supine sleep is not associated with impairments in auditory processing. The applicability of the current methods in early infancy suggests that electrophysiologic brain recordings represent a promising method of monitoring brain development in children with cranial disorders. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Improved correction of supraorbital rim deformity in craniosynostosis by the "tilt" procedure.

AbstractOrbital rim deficits are a feature of metopic, unilateral coronal, and bilateral coronal craniosynostosis. Several procedures have been developed to address this issue, but relapse to the preoperative hypoplastic deformity and stunted growth of the fronto-orbital region are common. The authors describe a technique modification of the conventional lateral canthal advancement referred to as the orbital rim “tilt” procedure, which aims to preserve inferior bony support for the orbital rim and create projection with optimal proclination of the fronto-orbital complex.

The Severity of Deformity in Metopic Craniosynostosis Is Correlated with the Degree of Neurologic Dysfunction

Background: In recent years, metopic synostosis has represented up to 25 percent of all nonsyndromic craniosynostosis cases, manifesting with varying degrees of trigonocephaly. It is unknown whether the degree of severity of anterior dysmorphology is proportionate to neurologic dysfunction. This knowledge is essential to guide future surgical treatment recommendations. Given the reported correlation of abnormal auditory processing with future language dysfunction and poorer academic performance in craniosynostosis patients, the present study aims to evaluate auditory processing in preoperative patients with moderate and severe metopic synostosis, and in control infants, to discern the neurologic risk carried by different severities of trigonocephaly. Methods: Thirty-eight infants underwent evaluation of auditory processing: nine patients with severe metopic synostosis, seven with moderate metopic synostosis, and 22 controls. Brain activity was recorded by electroencephalography while the participants listened to language-based stimuli. Electroencephalographic data were analyzed to extract event-related potentials evoked by the speech sounds. Results: Severe metopic synostosis patients demonstrated significantly attenuated event-related potential responses in the left frontal scalp region overlying the left frontal lobe compared with controls (p < 0.05). The moderate metopic synostosis patients did not show significantly different language processing compared to the control infants or the severe metopic synostosis patients in the frontal scalp region. Conclusions: The results of this study suggest that untreated severe metopic synostosis is associated with reduced language response in the frontal cortex. Less severe (moderate) forms are indeterminate, and mild forms do not show calculable irregularity by the event-related potential method of analysis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.