CYSTOID MACULAR EDEMA AFTER FOUR-POINT SCLERAL FIXATION OF INTRAOCULAR LENS.

PURPOSE: To assess the incidence of cystoid macular edema (CME) associated with 4-point Gore-Tex suture intraocular lens (IOL) scleral fixation, before and after institution of routine intravitreal triamcinolone acetonide prophylaxis and long-term topical nonsteroidal anti-inflammatory drug usage. METHODS: Consecutive patients were included after IOL implantation with concurrent pars plana vitrectomy for spontaneous IOL dislocation due to pseudoexfoliation syndrome. We compared short-term prophylactic nonsteroidal anti-inflammatory drugs only (Group A) to prophylactic intravitreal triamcinolone acetonide and long-term nonsteroidal anti-inflammatory drugs (Group B). RESULTS: Twenty-six eyes of 26 patients with pseudoexfoliation syndrome and spontaneous IOL dislocation were studied. Mean logMAR visual acuity improved from 1.27 ± 0.80 (20/375 Snellen equivalent) preoperatively to 0.46 ± 0.39 (Snellen 20/43) postoperatively (P < 0.001). Visual outcomes were similar for Groups A and B. In Group A, 10/16 eyes had CME, 4/16 had chronic CME longer than 6 months, and 1 longer than 12 months. In Group B, 1/10 had CME (which was both chronic and refractory). CONCLUSION: In eyes with pseudoexfoliation syndrome and spontaneous IOL dislocation, 4-point Gore-Tex suture IOL ab externo fixation yielded good visual outcomes, although CME was observed more than reported elsewhere. Prophylactic intravitreal triamcinolone acetonide and long-term nonsteroidal anti-inflammatory drugs seem to reduce the risk of postoperative CME.

Surgical Implications of the Anteriorly Displaced Segments of the Anterior Cerebral Artery in the Management of Frontoethmoidal Meningoencephalocele.

BACKGROUND: Preservation of the anterior cerebral arteries (ACAs) is important in the surgical management of frontoethmoidal meningoencephalocele (FEME). This would avoid complications related to the loss of blood supply to the part of the brain supplied by the ACA. Previous reports have identified hydrocephalus, microcephaly, cerebral dysplasias amounting to a 15% to 20% prevalence of brain anomalies in patients with FEME. What has not been previously reported are cerebral vasculature changes in the frontal region in FEME and how these may impact on the surgical correction and clinical outcome. METHODS: Two patients of FEME that demonstrate cautionary radiologic findings in relation to the ACAs and anterior fossa anatomy are discussed. RESULTS: The ACA in a 4-year-old boy with FEME was displaced anteriorly with the long A1 segments that extend into the defect. In the 2nd patient, a 4-year-old girl, we report on the complications related to the injury of ACAs from a previously partially resected FEME. CONCLUSION: The importance of the anterior cerebral vasculature around the FEME during surgery is crucial to prevent complications resulting from damage to a looping A1 segment of the ACA.

Central nervous system and cervical spine abnormalities in Apert syndrome.

PURPOSE: Apert syndrome characterized by acrocephalosyndactyly is a rare autosomal dominant congenital malformation with a prevalence of 1/65,000 births. With an extensive range of phenotypic and developmental manifestations, its management requires a multidisciplinary approach. A variety of craniofacial, central nervous system (CNS), and cervical spine abnormalities have been reported in these patients. This study aimed to determine the incidence of these CNS abnormalities in our case series. METHODS: Retrospective review of Australian Craniofacial Unit (ACFU) database for Apert patients was performed. Data collected that included demographics, place of origin, age at presentation, imaging performed, and images were reviewed and recorded. Where available, developmental data was also recorded. RESULTS: Ninety-four patients seen and managed at the ACFU had their CNS and cervical spine abnormalities documented. The main CNS abnormalities were prominent convolutional markings (67 %), ventriculomegaly (48 %), crowded foramen magnum (36 %), deficient septum pellucidum (13 %), and corpus callosum agenesis in 11 %. Major C-spine findings were present in 50.8 % of patients and included fusion of posterior elements of C5/C6 (50 %) and C3/4 (27 %). Multilevel fusion was seen in 20 %. Other abnormalities were C1 spina bifida occulta (7 %) and atlanto-axial subluxation (7 %). CONCLUSION: Multiple CNS and cervical spine (c-spine) abnormalities are common in Apert syndrome. The significance of these abnormalities remains largely unknown. Further research is needed to better understand the impact of these findings on growth, development, and treatment outcomes.

The Management of Nonsyndromic, Isolated Sagittal Synostosis.

AIM: Isolated sagittal synostosis is the commonest form of craniosynostosis. The reasons for surgery are to normalize the head shape and to increase the cranial volume, thus reducing the risk of raised intracranial pressure and allowing for normal brain development. It has been suggested that sagittal synostosis may impair neuropsychological development. This systematic review appraised the literature on the management of sagittal synostosis. METHODS: A literature search was performed with the assistance of a professional librarian. Studies selected had to satisfy the criteria set by PICO (patients, intervention, comparison, and outcome). Cranial index and neuropsychological outcome were used as outcome measures. MINORS was used to assess the methodological quality of the selected articles. A score of 75% was deemed to be of satisfactory quality, and the quality of the evidence from the selected studies was graded using the GRADE system. RESULTS: One hundred forty-eight articles were initially identified. Only 6 articles fulfilled the PICO criteria and scored a minimum of 75% on MINORS. Four studies compared 1 technique to another with documented cranial indices. Two studies compared 1 group to another and assessed the neuropsychological development. According to GRADE, the quality of evidence was deemed to be very low. CONCLUSIONS: This systematic review assessed cranial index and neuropsychological outcome following surgery for isolated, nonsyndromic sagittal synostosis. The quality of the evidence in the published literature was noted to be of very low quality. There is a need for better-designed, prospective studies to guide surgeons involved in management of sagittal synostosis.

The Craniofacial and Upper Limb Management of Nager Syndrome.

INTRODUCTION: Nager syndrome is a rare condition characterized by craniofacial and upper limb abnormalities. It is commonly mistaken for Treacher Collins syndrome, with which it shares the same craniofacial phenotype. However, patients with Treacher Collins do not exhibit hand anomalies, which are seen in patients with Nager syndrome. This paper reviews the multidisciplinary management of patients with Nager syndrome who were treated at the Australian Craniofacial Unit, Adelaide and the Erasmus Medical Centre, Rotterdam. METHODS: The database of both units was scrutinized and the case-notes of the patients with Nager syndrome were reviewed. Data was collected on patient demographics, surgical management, complications, and outcome. RESULTS: Nine patients (6 M:3 F) were identified with Nager syndrome, with a mean age at presentation of 3.7 years (range 8 days to 11.8 years). The mean follow-up time was 2.2 years (2 months to 19 years). SF3B4 mutation was noted in 2 patients and 1 patient had an X:9 translocation. Seven (77.8%) had obstructive sleep apnoea, with 5 patients diagnosed as severe obstructive sleep apnoea. Four patients had pollicization of their index, 2 patients had excision of extra radial digits and 1 patient underwent thumb duplication correction. Craniofacial surgery included mandibular advancement in 5 patients, temporo-mandibular joint reconstruction in 2 patients, and a genioplasty in 1 patient. CONCLUSION: Nager syndrome is a rare acrofacial dysostosis syndrome that is best managed within the realms of a multidisciplinary team. The authors would advocate early pollicization in patients with thumb anomalies to prevent any impairment in manual dexterity.

Apert Syndrome: Outcomes From the Australian Craniofacial Unit's Birth to Maturity Management Protocol.

The complex, progressive, multisystem nature of Apert syndrome presents many challenges to managing surgeons. Based on the pioneering work of Paul Tessier, the senior author developed a multidisciplinary birth to maturity management protocol for Apert syndrome. Between 1975 and 2014 the Australian Craniofacial Unit has treated 174 Apert syndrome patients and 28 have completed full protocol management. This paper reviews the scientific contribution made to the management of Apert syndrome by the Australian Craniofacial Unit, the development and evolution of the protocol and presents comprehensive data on the surgical and nonsurgical craniofacial interventions, and outcomes for the 28 patients who have completed the programme; 26 had normal visual acuity, 22 had normal hearing, 20 achieved normal or nearly normal speech, 24 a functional class I occlusion, 18 completed mainstream schooling of whom at least 8 went on to tertiary education, at least 13 gained employment and 15 developed good social groups. These outcomes equal or exceed those presented by other authors and provide compelling evidence of the value of protocol management in clinical outcomes, in addition to their value in international collaboration, and scientific development of future therapeutic strategies for the management of Apert syndrome.

Long-term visual outcomes in patients with orbitotemporal neurofibromatosis.

BACKGROUND: The study aimed to review the presentation and long-term visual outcomes of patients with orbitotemporal neurofibromatosis. DESIGN: Retrospective case series. PARTICIPANTS: Patients with orbitotemporal neurofibromatosis presenting from 1981 to 2009. METHODS: Demographic data, examination findings, causes of vision impairment and interventions performed were recorded for each patient from presentation through subsequent follow-up encounters. Visual impairment was defined as an ipsilateral Snellen acuity of <6/12. MAIN OUTCOME MEASURES: The proportion of patients with visual impairment or enucleation, the rate of new vision loss during follow up; and causes for vision loss or enucleation. RESULTS: Thirty-seven patients (17 female) were included. Median presenting age was 15 years (range 2-45) with an average follow up of 7.4 years (range 0.5-20.3). Visual impairment occurred in 54% of patients at presentation. Causes were amblyopia (13 of 37), optic atrophy (4 of 37), previous enucleation/evisceration (2 of 37), and optic nerve glioma (1 of 37). At presentation, 76% of patients had ptosis, and 51% had strabismus. Thirty-one patients had surgery, with an average of two procedures per patient. At final follow up, 62% had visual impairment. The rate of visual decline was 2% per patient-years. Causes of visual decline were two patients with optic nerve atrophy, one with exposure keratitis and one whose cause was unknown. Five blind patients had enucleation. CONCLUSIONS: The first series of orbitotemporal neurofibromatosis to focus on visual outcomes was presented. Vision loss is common, with a high prevalence of amblyopia. Close monitoring from an early age is needed to prevent visual impairment.

Neuropsychologic outcomes in patients treated for complex maxillofacial trauma.

Complex fractures of the craniofacial skeleton are caused most commonly, in Australia, by motor vehicle accidents, falls, and interpersonal violence. Significant force is required to fracture the facial skeleton, and the long-term effect these forces have on higher brain function is unclear. The study aim was to assess long-term neuropsychologic changes associated with complex fractures of the facial skeleton. Patients managed for complex fractures of the facial skeleton by the Australian Craniofacial Unit, South Australia, between 2002 and 2011, with at least 1-year follow-up, were assessed using the European Brain Injury Questionnaire. This questionnaire has previously published control data to which results were compared. Of the 2077 patients treated for facial fractures, 46 were identified as having complex fractures of the facial skeleton. Of the 46 patients, 13 were able to be contacted and assessed using the European Brain Injury Questionnaire. Changes in personality and ability to socialize and undertake executive function were noted in approximately 30% of the patients. In addition, approximately 50% of the patients' family members reported significant changes in the patients' life after the accident, yet this was only recognized by approximately 30% of the patients. This study shows that, despite the "crumple zone" of the facial skeleton providing some level of protection to the brain, patients having complex fractures of the facial skeleton have long-term neuropsychologic changes that affect both their own and their family's quality of life.

From birth to maturity: midline tessier 0-14 craniofacial cleft patients who have completed protocol management at a single craniofacial unit.

INTRODUCTION: The rare craniofacial clefts form an important component of craniofacial pathology, but little has been written regarding the definitive management of affected patients. This report describes the presentation, management, and outcomes in a group of patients who have completed their protocol management for treatment of midline Tessier 0-14 clefts. METHODS: A retrospective review of the clinical, photographic, and radiographic records of all midline cleft patients treated at a single center was performed. Data describing each patient's presenting features, surgical management, and final outcomes are presented. RESULTS: Four patients were identified as having completed protocol management for Tessier 0-14 midline clefting at the unit. The age range at the most recent follow-up was 19.3 to 36.3 years. Three patients had entered protocol management during infancy, and the remaining patient presented to the unit at 13.8 years of age. The surgical management regimen is described in detail. Outcomes for development, hearing, speech, and vision at maturity were all acceptable. Three patients attained a respectable educational and social status. With respect to facial aesthetics scores, the only significant difference after management was a significant worsening of deformity in the region of the orbits. The Whitaker grade for repeat surgery improved after management (3.25 before to 2.63 postmanagement), but this improvement was not statistically significant. CONCLUSION: Presented are the results of the first cohort of midline Tessier 0-14 cleft patients to have completed protocol management at a single craniofacial unit. As more patients complete their management in the future, further refinements to the protocol could be made.

Association of Perioperative Antibiotics with the Prevention of Postoperative Fistula after Cleft Palate Repair.

Background: There is debate amongst surgeons regarding the use of antibiotics to prevent fistulae after palatoplasty. Prescribing should be evidence based, as antibiotic stewardship is integral to reducing antibiotic resistance. Our aim was to determine whether differing perioperative regimens affect the prevalence of postoperative fistulae. Methods: The sample comprised participants from the Cleft Collective who had undergone palatoplasty. Participants were recruited across all 16 UK cleft centers between 2013 and 2021. The exposure was perioperative antibiotic regimen prescribed at the time of palatoplasty. The primary outcome was the presence of palatal fistula. Results: Fistula data were available for 167 participants when exploring antibiotic regimen and for 159 when exploring antibiotic agent. There was no evidence to suggest a difference in fistula rate between those receiving antibiotics on induction only versus as an inpatient or up to 7 days postoperatively (χ2 = 4.57; P = 0.10). There was no evidence to suggest a difference in fistula rate between those who received co-amoxiclav and those who had an alternative antibiotic (χ2 = 0.16; P = 0.69). Postoperative fistulae increased with the extent of the cleft (χ2 = 20.39; P < 0.001). When adjusting for cleft type, no evidence of an association between antibiotic regimen and fistulae was found (inpatient antibiotics: OR 1.36; 95% confidence interval, 0.53-3.51; antibiotics up to 7 days postoperatively: OR 0.68; 95% confidence interval, 0.26-1.80). Conclusions: The choice of antibiotic and dosing regimen does not influence the formation of postoperative fistulae. These results should be supported by interventional trials.

Management of isolated fractures of the medial orbital wall.

The purpose of the present study was to retrospectively review the management and outcome of patients treated by the Australian Craniofacial Unit (ACFU) for isolated fractures of the medial orbital wall. A retrospective medical record review of patients treated between 2008 and 2012 was performed. Ethical approval was granted by the ethics committee of the Women's and Children's Hospital. Patient demographics, causes of injury, physical examination findings, management (conservative or surgical), and findings at follow-up were recorded. Computed tomographic scans were reviewed, and values for fracture area and volume of displaced tissue were calculated. Twenty-four patients with this injury were treated by the ACFU between 2008 and 2012. Eighteen were male, and 6 were female. Assault was the most common cause (15/24 patients). Fifteen patients were managed conservatively, and 9 were treated surgically. In those patients managed conservatively, the mean fracture area was 1.44 cm (0.47-2.47 cm), and the mean volume of displaced tissue was 0.48 mL (0.03-1.15 mL). In patients treated surgically, the mean fracture surface area was 2.32 cm (0.07-3.43 cm), and mean volume of displaced tissue was 0.94 mL (0.00-1.47 mL). No patients were found to have clinically significant enophthalmos at follow-up examination. The current practice of managing isolated fractures of the medial orbital wall at the ACFU has been successful in preventing significant enophthalmos. The thresholds for fracture area and, in particular, volume of displaced tissue, proposed by Jin et al (J Oral Maxillofac Surg 2000;58:617-620), show merit as a tool for determining patient management.

Facial aesthetics and perceived need for further treatment among adults with repaired cleft as assessed by cleft team professionals and laypersons.

The objectives of this study were to compare the ratings of professionals and laypeople with and without a cleft regarding the facial aesthetics of adult patients previously treated for orofacial clefting. The necessity for further treatment, as perceived by the respective groups, is also compared. The design of the study was a cross-sectional study. Professionals (two plastic surgeons, one dentist, one orthodontist, and one psychologist) and laypeople (one male and one female adult without a cleft and one male and one female adult with a cleft) were recruited to rate photographs of 80 non-syndromic cleft patients treated by the Australian Craniofacial Unit from 1975 to 2009. Facial aesthetics were measured by a visual analogue scale (VAS; 0-100 mm). High values indicated good aesthetics. Necessity for further treatment was also measured by a VAS (0-100 mm). High values indicated high perceived need for further treatment. The professionals rated facial aesthetics significantly lower and had a lower perception of need for further treatment than the raters with and without a cleft. The laypeople with a cleft rated facial aesthetics significantly higher and had a lower perceived need for further treatment than laypeople without a cleft. The non-surgical professionals rated facial aesthetics significantly lower and had a lower perceived need for further treatment than the surgical professionals. Differences exist in the facial aesthetics ratings and perceived need for further surgery between professionals and laypeople with and without a cleft. This should be considered when managing cleft treatment expectations.

Syndromes associated with Robin sequence: a national prospective cohort study.

OBJECTIVES: To determine the prevalence of syndromic Robin sequence (RS) in the UK and if this group of patients had an increased need for airway and feeding management compared with a non-syndromic RS cohort. DESIGN: A prospective national multicentre study of cases submitted to the Cleft Collective cohort studies. SETTING: Specialist cleft services in the UK. PATIENTS: 259 participants who fulfilled the diagnosis of RS. This group was compared with 548 participants with cleft palate only (CPO). MAIN OUTCOME MEASURES: The primary outcome measure was the presence of a syndrome in patients with RS and CPO. Secondary outcome measures included the use of airway and feeding adjuncts. RESULTS: An associated syndrome was seen in 28% of patients with RS and 14% of patients with CPO. The most common syndrome for the RS group was Stickler syndrome (27%). Syndromic status was significantly higher among patients with RS compared with those with CPO (OR 2.36, 95% CI 1.65 to 3.39; p<0.001). Patients with syndromic RS have an increased reliance on airway adjuncts compared with the patients without syndromic RS (OR 2.02, 95% CI 1.13 to 3.64; p=0.018). There was no evidence of a difference in the use of feeding adjuncts between syndromic and non-syndromic RS groups (OR 2.43, 95% CI 0.78 to 7.58; p=0.126). CONCLUSION: The presence of a syndrome has implications for management of patients with RS. Early identification of a syndrome may help prevent the consequences of a missed syndromic diagnosis. Routine ophthalmological and genetic screening for Stickler syndrome should be mandatory for all patients with RS.

Binder syndrome.

UNLABELLED: Patients with chondrodysplasia punctata (CDP) usually present with Binder-type features, and often CDP is misdiagnosed as Binder syndrome. This study reviewed the management and outcome of patients with Binder syndrome and CDP in a multidisciplinary setting. METHODS: The notes and radiographs of the patients managed at the Australian Craniofacial Unit with a multidisciplinary setting since 1976 were reviewed, and data were collected on patient demographics, associated medical and surgical problems, subsequent management, and complications. RESULTS: Seventy-seven patients were treated over the 30-year period (5 patients were lost to follow-up); of the remaining 72 patients, 60 (83%) had Binder syndrome, and 12 (17%) were patients with CDP. Forty were males, and 32 were females, with an age range of 6 months to 47 years. Thirteen patients (18%) had a strong family history, and 65 patients (90%) have so far undergone surgical correction, and of those, 35 (54%) have completed their treatment, the longest follow-up time being 18 years. The mean number of surgical procedures was 2.4, and 18 patients (28%) had postoperative complications, which included partial necrosis of the maxilla, osteomyelitis of the mandible, facial nerve and inferior alveolar nerve neuropraxia, nasal bone graft exposure, and cellulitis. DISCUSSION: Because of the phenotypic characteristics shared by both Binder syndrome and CDP, it is most likely that Binder syndrome is not a syndrome, nor is it an entity, but most likely to be an "association." We would advocate that these patients should be managed in a multidisciplinary setting.

Cervical spine in Treacher Collins syndrome.

Treacher Collins syndrome is a congenital syndrome with characteristic craniofacial malformations, which are well described in the literature. However, the presence of cervical spine dysmorphology in this syndrome has been minimally described. This study reviews cervical spine radiographs of 40 patients with Treacher Collins syndrome. In this sample, 7 of 40 patients displayed cervical spine anomalies, with 3 of these patients displaying multiple cervical spine anomalies. The patterns of spinal anomalies were variable, suggesting that the underlying genetic mutation has variable expressivity in cervical spine development as it does elsewhere in the craniofacial skeleton.

General health-related quality of life and oral health impact among Australians with cleft compared with population norms; age and gender differences.

OBJECTIVE: To evaluate general health-related quality of life (HRQoL) and oral health impact among adults treated for cleft to determine age and gender differences, and to compare against population norms. DESIGN: Cross-sectional prospective study. PARTICIPANTS/SETTING: Nonsyndromic cleft patients treated by the Australian Craniofacial Unit from 1975 to 2009 were recruited (n  =  112). Response rate was 79% (n  =  88). Main outcome measures : HRQoL was measured by the Short Form (SF)-36 questionnaire. Oral health impact was measured by the Oral Health Impact Profile (OHIP)-14 questionnaire. State-based and national norms were used for comparative purposes. RESULTS: There were no significant age or sex differences in the cleft sample's SF-36 and OHIP-14 scores. When compared against South Australian 2002 state-level norms, cleft participants scored higher on physical function and physical role function but lower on vitality and mental health. The prevalence of having experienced one or more of OHIP-14 items "fairly often" or "very often" was 2.7 times higher than national-level estimates, while extent was 2.8 times and severity 1.7 times higher. CONCLUSIONS: The oral health impact among cleft patients included in our study was poor compared with population-level estimates. The HRQoL showed mixed results, with the vitality and mental health components being poorer in the cleft group compared with population-level estimates. These results indicate that treatment for orofacial clefting does not entirely remove the factors contributing to poor HRQoL and oral health.

Cardiometabolic risk factors and hypovitaminosis D in adolescents with overweight from a sunny region in northeast Brazil: a cross-sectional study.

INTRODUCTION: Background: obesity can influence vitamin D levels, which in turn might be associated with cardiometabolic risk factors. Objectives: this study assessed the association between 25-hydroxyvitamin D [25(OH)D] levels and cardiometabolic risk factors in adolescents with overweight living in a region of northeastern Brazil. Material and methods: a cross-sectional study was carried out by non-probabilistic sampling in adolescents diagnosed with overweight or obesity. The subjects were divided according to their 25(OH)D status into two groups: sufficient vitamin D and hypovitaminosis D. Biodemographic, lifestyle, cardiometabolic, and biochemical factors were evaluated. A logistic regression model was applied to determine the predictors of hypovitaminosis D. Results: we found a high frequency of hypovitaminosis D (45.6 %) in adolescents. Weekly sun exposure was negatively associated with hypovitaminosis D (OR = 0.96; 95 % CI: 0.92-0.99), while significant positive associations were observed between hypovitaminosis D and blood pressure above the 95th percentile (OR = 4.00; 95 % CI: 1.19-13.37), body weight (OR = 1.04; 95 % CI: 1.01-1.07), and fasting insulin (OR = 1.13; 95 % CI: 1.05-1.22). Conclusion: hypovitaminosis D showed a high prevalence in adolescents with overweight living in a sunny region of northeastern Brazil, and cardiometabolic risk factors such as systemic arterial hypertension, high body weight, and hyperinsulinemia are predictors of hypovitaminosis D.

INTRODUCCIÓN: Introducción: la obesidad puede influir en los niveles de vitamina D, lo que a su vez podría estar asociado con factores de riesgo cardiometabólico. Objetivos: este estudio evaluó la asociación entre los niveles de 25-hidroxivitamina D [25(OH)D] y los factores de riesgo cardiometabólico en adolescentes con sobrepeso que viven en una región del noreste de Brasil. Material y métodos: se realizó un estudio transversal mediante muestreo no probabilístico con adolescentes diagnosticados de sobrepeso u obesidad. Los sujetos se dividieron según su estado de 25(OH)D en dos grupos: suficiente vitamina D e hipovitaminosis D. Se evaluaron factores biodemográficos, de estilo de vida, cardiometabólicos y bioquímicos. Se aplicó un modelo de regresión logística para determinar los predictores de la hipovitaminosis D. Resultados: encontramos una alta frecuencia de hipovitaminosis D (45,6 %) en los adolescentes. La exposición semanal al sol se asoció negativamente a la hipovitaminosis D (OR = 0,96; IC 95 %: 0,92-0,99), mientras que se observaron asociaciones positivas significativas entre hipovitaminosis D y presión arterial por encima del percentil 95 (OR = 4,00; IC 95 %: 1,19-13,37), peso corporal (OR = 1,04; IC del 95 %: 1,01-1,07) e insulina en ayunas (OR = 1,13; IC del 95 %: 1,05-1,22). Conclusión: la hipovitaminosis D mostró una alta prevalencia entre los adolescentes con sobrepeso que viven en una región soleada del noreste de Brasil, y los factores de riesgo cardiometabólico, como hipertensión arterial sistémica, peso corporal elevado e hiperinsulinemia, son predictores de hipovitaminosis D.

A new syndrome with craniosynostosis and cleft lip and palate.

Two unrelated girls with craniosynostosis and bilateral cleft lip and palate who also had developmental delay and umbilical herniae are presented. We propose that these patients have the same condition, and that their combination of features may constitute a new syndrome. Management of the patients is discussed.

Sagittal Synostosis and Its Association With Cognitive, Behavioral, and Psychological Functioning: A Meta-analysis.

Importance: Findings on the cognitive, behavioral, and psychological functioning of individuals with sagittal synostosis (SS) are highly disparate, limiting their clinical utility. Objective: To identify and review research on individuals with SS and to determine whether, and to what extent, they experience cognitive, behavioral, and psychological difficulties compared with their healthy peers or normative data for each measure. Data Sources: PubMed, Scopus, Embase, and PsycINFO were searched through January 2021 with no date restrictions. Scopus citation searches and manual checks of the reference lists of included studies were conducted. Study Selection: Studies included participants of any age who had received a diagnosis of single-suture (isolated or nonsyndromic) SS or scaphocephaly and who had been assessed on cognitive, behavioral, and psychological outcomes. Data Extraction and Synthesis: Data were independently extracted by 2 reviewers. Case-control outcomes (individuals with SS vs healthy peers or normative data) were compared using random-effects models with 3 effect sizes calculated: weighted Hedges g (gw), odds ratios (ORs), and mean prevalence rates. This study follows the Meta-analysis of Observational Studies in Epidemiology (MOOSE) reporting guidelines. Main Outcomes and Measures: Findings were categorized by surgical status (conservatively managed, presurgery, postsurgery, or combined); domain (eg, general cognition); type of cognitive, behavioral, or psychological measure (objective or subjective); and source of comparison data (peers or normative data). Results: Data from 32 studies, involving a pooled sample of 1422 children and adults with SS (mean [SD] age at assessment, 5.7 [6.6] years; median [interquartile range] age, 3.3 [0.5-10.3] years), were analyzed. Data on sex were available for 824 participants, and 642 (78%) were male. Individual study results varied substantially. Objective tests identified significant moderate group differences on 3 of 16 examined domains: presurgical motor functioning (3 studies; gw = -0.42; 95% CI, -0.67 to -0.18; P < .001), postsurgical short-term memory (2 studies; gw = -0.45; 95% CI, -0.72 to -0.17; P < .001), and postsurgical visuospatial ability (6 studies; gw = 0.31; 95% CI, 0.18 to 0.44; P < .001). Prevalence estimates and ORs varied widely, with 15 studies showing prevalence estimates ranging from 3% to 37%, and 3 studies showing ORs ranging from 0.31 (95% CI, 0.01 to 6.12) for processing speed in the conservatively managed sample to 4.55 (95% CI, 0.21 to 98.63) for postsurgical visuospatial abilities. Conclusions and Relevance: In this meta-analysis, findings for the functioning of participants with SS were highly disparate and often of low quality, with small samples sizes and control groups rarely recruited. Nonetheless, the findings suggest that some individuals with SS experience negative outcomes, necessitating routine assessment.