Hearing Loss Detection and Early Intervention Strategies in Kenya.

Background: Thirty-four million children globally have disabling hearing loss, with the highest prevalence in low- and middle-income countries (LMICs). Early identification and management is crucial, yet barriers to screening and treatment of hearing loss are extensive in LMICs. Unaddressed hearing loss negatively impacts individuals and communities. The WHO's 2021 World Report on Hearing urges the development of Ear and Hearing Care (EHC) programs to improve access to all aspects of care, including screening, diagnostics, management, and developmental support. A joint Nairobi- and Seattle-based group convened in 2021 to discuss strategies for program development in Kenya, as presented in this paper. Findings: Developing a national EHC program must include the necessary support services for a child with a diagnosed hearing loss, while simultaneously promoting engagement of family, community, and healthcare workers. Existing government and healthcare system policies and priorities can be leveraged for EHC programming. Strategies for success include strengthening connections between policymakers at national, county, and municipal levels and local champions for the EHC agenda, with a concurrent focus on policy, early detection and intervention, habilitation, and family-centered care. Updates to health policy and funding to support the accessibility of services and equipment should focus on leveraging national healthcare coverage for hearing technologies and services, strengthening referral pathways, training to bolster the workforce, and metrics for monitoring and evaluation. Additional strategies to support forward progress include strategic engagement of partners and leveraging local partners for phased scale-up. Conclusions and Recommendations: Recommendations to strengthen EHC within the Kenyan health system include concurrent leverage of existing health policies and priorities, partner engagement, and strengthening referral pathways, monitoring and evaluation, and training. These strategies may be generalized to other countries too.

Hallermann-Streiff Syndrome in Concordant Monozygotic Twins With Congenital Cataracts, Exudative Retinal Detachments, and One Case of Corneal Perforation Requiring Keratoplasty.

PURPOSE: We describe the management of Hallermann-Streiff syndrome in monozygotic female twins with congenital cataracts, exudative retinal detachments, and 1 case of corneal descemetocele with associated dellen and subsequent perforation. METHODS: This study was a case report and review of the literature. RESULTS: Twins 1 and 2 exhibited all 7 cardinal characteristics of Hallermann-Streiff syndrome, presenting with spontaneous lenticular resorption, anterior uveitis, and glaucoma. They underwent bilateral cataract extraction with near total capsulectomy. Both twins experienced recurrent glaucoma, for which twin 1 underwent successful endocyclophotocoagulation in both eyes and twin 2 in the left eye alone. The fellow eye developed 2 sites of perilimbal corneal descemetoceles with associated dellen at the inferotemporal limbal corneal junction leading to spontaneous perforation of 1 site, requiring a full-thickness corneal graft. Both twins developed recurrent bilateral exudative retinal detachments unresponsive to oral prednisolone. Twin 1's last best-corrected visual acuity with aphakic spectacles was 20/260 in the right eye and 20/130 in the left eye at age 4 years and 8 months. Twin 2's last best-corrected visual acuity was 20/130 in each eye at age 4 years and 11 months, over a year after right eye penetrating keratoplasty. CONCLUSIONS: We describe 2 rare cases of Hallermann-Streiff syndrome in monozygotic twins complicated by corneal perforation requiring penetrating keratoplasty in 1 eye of 1 twin. Although corneal opacities have been described in this condition, this is the first case of corneal descemetocele in Hallermann-Streiff syndrome. The cornea was stabilized with a relatively favorable visual outcome over 1 year later.

POLR1A variants underlie phenotypic heterogeneity in craniofacial, neural, and cardiac anomalies.

Heterozygous pathogenic variants in POLR1A, which encodes the largest subunit of RNA Polymerase I, were previously identified as the cause of acrofacial dysostosis, Cincinnati-type. The predominant phenotypes observed in the cohort of 3 individuals were craniofacial anomalies reminiscent of Treacher Collins syndrome. We subsequently identified 17 additional individuals with 12 unique heterozygous variants in POLR1A and observed numerous additional phenotypes including neurodevelopmental abnormalities and structural cardiac defects, in combination with highly prevalent craniofacial anomalies and variable limb defects. To understand the pathogenesis of this pleiotropy, we modeled an allelic series of POLR1A variants in vitro and in vivo. In vitro assessments demonstrate variable effects of individual pathogenic variants on ribosomal RNA synthesis and nucleolar morphology, which supports the possibility of variant-specific phenotypic effects in affected individuals. To further explore variant-specific effects in vivo, we used CRISPR-Cas9 gene editing to recapitulate two human variants in mice. Additionally, spatiotemporal requirements for Polr1a in developmental lineages contributing to congenital anomalies in affected individuals were examined via conditional mutagenesis in neural crest cells (face and heart), the second heart field (cardiac outflow tract and right ventricle), and forebrain precursors in mice. Consistent with its ubiquitous role in the essential function of ribosome biogenesis, we observed that loss of Polr1a in any of these lineages causes cell-autonomous apoptosis resulting in embryonic malformations. Altogether, our work greatly expands the phenotype of human POLR1A-related disorders and demonstrates variant-specific effects that provide insights into the underlying pathogenesis of ribosomopathies.

Characterization of hearing status in children under 3 years of age with cleft palate.

OBJECTIVES: To describe the hearing thresholds of children under 3 years of age with cleft palate with or without cleft lip. METHODS: This retrospective study from a tertiary care children's hospital included children with cleft palate ± cleft lip born between January 01, 2008, and December 31, 2015. All patients who had the initial palate repair at our institution and at least one behavioral audiogram were included. Behavioral audiograms collected either in the sound field or under insert earphones showed results from the better hearing ear. We collected demographic information, cleft type, parent report of newborn hearing screen, syndrome diagnosis, comorbid conditions, and dates of tympanostomy tube placement and palatoplasty. The main outcome measure was hearing status as measured by behavioral audiograms, and, for a subset of patients, brainstem auditory evoked responses (BAER). Results were grouped by test type (behavioral vs BAER) and characteristics at time of testing (pre- or post-palatoplasty, with or without tympanostomy tube placement) to evaluate the range of severity of hearing loss. RESULTS: Three hundred and sixty patients were included in the cohort. The cohort had two groups: Group 1 included 37 patients who had BAER results prior to surgery and behavioral audiogram results post-surgery; Group 2 included 322 patients who had behavioral audiograms pre-surgery (n = 206) and/or post-surgery (n = 215). The median age of patients at time of BAER in Group 1 was 3.00 months [IQR: 1.00,3.75]; pre-surgery BAER results showed mild (45.9%, n = 17) or moderate hearing loss (29.7%, n = 11) by this age. Patients in Group 2 with pre-surgery behavioral audiograms had a median age of 12.00 months [IQR: 11.00, 14.00] at time of audiogram. Most patients had a mild (33.5%, n = 69) or moderate hearing loss (31.1%, n = 64). Post-surgery, patients had a median age of 13.00 months [IQR: 11.00, 15.00] at time of hearing assessment and typically had normal hearing (86.0%, n = 185). In most patients, hearing improved after palate repair with concurrent tympanostomy tube placement. CONCLUSIONS: Most patients with cleft palate ± cleft lip had evidence of mild or moderate hearing loss prior to cleft palate repair, and hearing loss was evident from early infancy. Hearing improved after concurrent tympanostomy tube placement and palatoplasty. Further research is needed to understand the clinical significance of untreated transient hearing loss during the first year of life in this patient population.

Multidisciplinary Care Considerations for Patients with Craniosynostosis.

Infants and children with craniosynostosis require multidisciplinary care, and this is best accomplished when care is provided on a craniofacial team. Most patients with craniosynostosis will have non-syndromic presentations; however, longitudinal care remains critical to ensure appropriate growth and development throughout childhood. In patients with syndromic craniosynostoses, coordinated longitudinal care becomes even more paramount because of the high level of complexity across many different specialties or disciplines. Care delivery that includes perspective and expertise from multiple disciplines is important to help patients reach their full potential and optimal outcomes.

Epidemiology, Genetics, and Pathophysiology of Craniosynostosis.

Craniosynostosis, the premature fusion of the infant cranial skulls, can be recognized by characteristic head shape differences that worsen with head growth. Craniosynostosis can be syndromic or nonsyndromic and can involve one suture or multiple sutures. Timely cranial vault surgery is recommended to expand and reshape the skull, with a goal of preventing increased intracranial pressure and providing sufficient space for brain growth. Several gene variants and environmental exposures are known to increase the risk of single suture craniosynostosis (SSC), including in utero constraint, exposure to specific toxins and medications, and medical conditions such as thyroid dysregulation and metabolic bone disorders.

Health-Related Quality of Life and Depression Symptoms in a Cross Section of Patients with Advanced Lung Cancer before and during the COVID-19 Pandemic.

Background: Adults with advanced lung cancer experience reduced health-related quality of life (HRQOL) and psychological symptoms at diagnosis. Objective: This study aimed to evaluate whether the COVID-19 pandemic worsened HRQOL among patients recently diagnosed with cancer. Design: We analyzed baseline data from two randomized controlled trials of early palliative care to compare HRQOL and depression symptoms among those enrolled during the pandemic (January 2020 to January 2021) versus prepandemic (March 2018 to January 2019). Setting/Subjects: This cohort included patients recently diagnosed with advanced lung cancer in two multisite studies. Measurements: We used analysis of covariance to calculate adjusted mean differences between groups with the timeframe as an independent variable and HRQOL (using the Functional Assessment of Cancer Therapy-General) and depression symptoms (using the Patient Health Questionnaire-9) as dependent variables, adjusting for age, gender, relationship status, performance status, symptoms, and time since diagnosis. We tested for an interaction between the COVID-19 timeframe and relationship status. Results: Neither HRQOL (adjusted mean difference -1.78; p = 0.137) nor depression symptoms (0.06; p = 0.889) differed between patients enrolled pre-COVID-19 (n = 665) relative to those enrolled during COVID-19 (n = 191) in adjusted analyses. Relationship status moderated the effect of the COVID-19 timeframe on HRQOL; unmarried patients experienced worse HRQOL during COVID-19 (adjusted mean difference: -5.25; p = 0.011). Conclusions: The COVID-19 pandemic did not further reduce HRQOL or increase depression symptoms among patients recently diagnosed with lung cancer, but did worsen HRQOL for unmarried patients in moderation analysis. Psychosocial evaluation and supportive care are important for all patients, particularly those with limited social support. Clinical trial registration numbers: NCT03337399 and NCT03375489.

Randomized Trial of a Palliative Care Intervention to Improve End-of-Life Care Discussions in Patients With Metastatic Breast Cancer.

BACKGROUND: Studies show that early, integrated palliative care (PC) improves quality of life (QoL) and end-of-life (EoL) care for patients with poor-prognosis cancers. However, the optimal strategy for delivering PC for those with advanced cancers who have longer disease trajectories, such as metastatic breast cancer (MBC), remains unknown. We tested the effect of a PC intervention on the documentation of EoL care discussions, patient-reported outcomes, and hospice utilization in this population. PATIENTS AND METHODS: Patients with MBC and clinical indicators of poor prognosis (n=120) were randomly assigned to receive an outpatient PC intervention (n=61) or usual care (n=59) between May 2, 2016, and December 26, 2018, at an academic cancer center. The intervention entailed 5 structured PC visits focusing on symptom management, coping, prognostic awareness, decision-making, and EoL planning. The primary outcome was documentation of EoL care discussions in the electronic health record (EHR). Secondary outcomes included patient-report of discussions with clinicians about EoL care, QoL, and mood symptoms at 6, 12, 18, and 24 weeks after baseline and hospice utilization. RESULTS: The rate of EoL care discussions documented in the EHR was higher among intervention patients versus those receiving usual care (67.2% vs 40.7%; P=.006), including a higher completion rate of a Medical Orders for Life-Sustaining Treatment form (39.3% vs 13.6%; P=.002). Intervention patients were also more likely to report discussing their EoL care wishes with their doctor (odds ratio [OR], 3.10; 95% CI, 1.21-7.94; P=.019) and to receive hospice services (OR, 4.03; 95% CI, 1.10-14.73; P=.035) compared with usual care patients. Study groups did not differ in patient-reported QoL or mood symptoms. CONCLUSIONS: This PC intervention significantly improved rates of discussion and documentation regarding EoL care and delivery of hospice services among patients with MBC, demonstrating that PC can be tailored to address the supportive care needs of patients with longer disease trajectories. ClinicalTrials.gov identifier: NCT02730858.

Accuracy and Reliability of 4D-CT and Flexible Laryngoscopy in Upper Airway Evaluation in Robin Sequence.

OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiber-optic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day time points, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 32 sets of 4D-CT visualizations and FFL videos (dynamic modalities) and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1 = none to 5 = complete) and noted confidence levels of each rating. Intraclass correlation and Krippendorff alpha were used to assess intra- and interrater reliability, respectively. Accuracy was assessed by comparing clinician ratings with quantitative percentage constriction (QPC) ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: There was similar intrarater agreement (moderate to substantial) with 4D-CT and FFL, and both demonstrated fair interrater agreement. Both modalities underestimated UAO severity, although 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (-1.06 and -1.46 vs QPC ratings, P = .004). Overall confidence levels were similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, P < .0001). CONCLUSION: Although 4D-CT may be more accurate in assessing the degree of UAO in patients with RS, 4D-CT and FFL assessments demonstrate similar reliability. Additionally, 4D-CT may be interpreted with greater confidence by nonotolaryngologists who care for these patients.

Home Language Environment of Children With Orofacial Clefts as a Target for Intervention.

OBJECTIVE: To evaluate the home language environment (HLE) in children with orofacial clefts as a potential modifiable target for language and literacy intervention. DESIGN: Feasibility study examining longitudinal trends in HLE and responses to parent-focused literacy intervention. SETTING: Tertiary care children's hospital. PARTICIPANTS: HLE data were collected for 38 children with orofacial clefts between ages 7 and 23 months. Twenty-seven participants received parent-focused literacy intervention. INTERVENTIONS: Reach Out and Read, a literacy intervention, was introduced during a clinic visit. To assess response, participants were randomized to age at intervention (9, 18, or 24 months). MAIN OUTCOME MEASURES: Primary outcome measures included measurements from recordings in the home language environment of adult word count, child vocalizations, and conversational turns. RESULTS: Baseline (preintervention) results showed lower adult word count and conversational turns for caregivers and children with cleft lip and palate, as well as for those from lower socioeconomic groups. After the literacy intervention was introduced, this cohort showed increasing measures of child and caregiver vocalizations, particularly when introduced at 18 months. CONCLUSIONS: Although these results are preliminary, findings suggest that HLE characteristics vary as a function of children's cleft type as well as family socioeconomic status. Further, our caregiver-focused literacy intervention was feasible and resulted in short-term improvements in HLE. This is the first study to document HLE as a target for intervention in children with oral clefts. These findings support further research on HLE and caregiver-focused intervention to improve language/literacy outcomes for children with oral clefts.

Patient-Reported and End-of-Life Outcomes Among Adults With Lung Cancer Receiving Targeted Therapy in a Clinical Trial of Early Integrated Palliative Care: A Secondary Analysis.

CONTEXT: Targeted therapy has revolutionized lung cancer treatment and markedly increased survival, though data are lacking on patient-reported and end-of-life (EOL) outcomes among patients receiving targeted therapy. OBJECTIVES: This study aimed to compare quality of life (QOL), symptoms, prognostic communication, and EOL care between patients receiving targeted therapy and patients with lung cancer without targetable mutations. METHODS: In this secondary analysis of a randomized trial of early palliative care in advanced lung cancer (n=154), we compared change in QOL and symptoms (per the Functional Assessment of Cancer Treatment [FACT]-Lung scale) over 24 weeks among patients with lung cancer receiving targeted therapy versus those without targetable mutations using linear mixed effects models, adjusted for receipt of palliative care, age and gender. We also compared prognostic communication and decedents' EOL health care utilization using logistic regression, adjusted for palliative care. RESULTS: Compared to individuals without targetable mutations, patients receiving targeted therapy (n=35) reported greater improvements in QOL (FACT-General B=0.46; 95% CI=0.19, 0.73) and symptoms (FACT-Lung Cancer Subscale B=0.12; 95% CI=0.03, 0.20) over time, independent of palliative care. Patients receiving targeted therapy were also more likely to report they rarely discussed prognosis with their clinicians (OR=2.59, 95% CI=1.01, 6.63) and were more likely to receive cancer-directed therapy in their last 14 days of life (OR=14.98, 95% CI=4.08, 54.96). CONCLUSIONS: Relative to patients without targetable mutations, patients with lung cancer who receive targeted therapy experience improved QOL and symptoms, are less likely to discuss prognosis early in their illness course, and more likely to continue treatment until death and die in the hospital.

Psychological Distress in Bereaved Caregivers of Patients With Advanced Cancer.

CONTEXT: Individuals caring for patients with advanced cancer (caregivers) experience psychological distress during the patient's illness course. However, data on the prevalence of bereaved caregivers' psychological distress and its relationship with the quality of patient's end of life (EOL) care are limited. OBJECTIVES: To describe rates of depression and anxiety symptoms in bereaved caregivers of patients with advanced cancer and to understand the relationship between these outcomes and patient distress at the EOL. METHODS: We conducted a secondary analysis of 168 caregivers enrolled in a supportive care trial for patients with incurable lung and gastrointestinal cancers and their caregivers. We used the Hospital Anxiety and Depression Scale to assess caregivers' depression and anxiety symptoms at three months after the patient's death. Caregivers also rated the patient's physical and psychological distress in the last week of life on a 10-point scale three months after the patient death. We used linear regression adjusting for caregiver age, sex, randomization, and cancer type to explore the relationship between bereaved caregivers' depression and anxiety symptoms and their ratings of physical and psychological distress in patients at the EOL. RESULTS: Of the 168 bereaved caregivers, 30.4% (n = 51) and 43.4% (n = 73) reported clinically significant depression and anxiety symptoms, respectively. Caregiver ratings of worse physical (B = 0.32; P = 0.009) and psychological (B = 0.50; P < 0.001) distress experienced by the patient at the EOL were associated with worse depression symptoms in bereaved caregivers. Only caregiver rating of worse psychological distress experienced by the patient at the EOL (B = 0.42; P < 0.001) was associated with worse bereaved caregivers' anxiety symptoms. CONCLUSION: Many bereaved caregivers of patients with advanced cancer experience symptoms of depression and anxiety, which are associated with their perceptions of distress in their loved ones at the EOL.

Catel-Manzke syndrome without Manzke dysostosis.

Catel-Manzke syndrome is characterized by hand anomalies, Robin sequence, cardiac defects, joint hyperextensibility, and characteristic facial features. Approximately 40 patients with Catel-Manzke have been reported, all with the pathognomonic bilateral or unilateral hyperphalangy caused by an accessory bone between the second metacarpal and proximal phalanx known as Manzke dysostosis. Here we present the first case of molecularly confirmed Catel-Manzke syndrome with Robin sequence but without Manzke dysostosis.

Neurodevelopmental and Academic Outcomes in Children With Orofacial Clefts: A Systematic Review.

CONTEXT: Children with orofacial clefts (OFCs) are reported to have worse neurodevelopmental outcomes than unaffected peers, although study methodologies and findings are highly variable and trends in outcomes by age remain unexplored. OBJECTIVE: To examine the strength of the evidence and explore trends in neurodevelopment by age. DATA SOURCES: A systematic review was conducted of studies published from January 1, 1980, through November 3, 2017. STUDY SELECTION: Studies were independently screened by the authors and included in the review if they met predetermined eligibility criteria: (1) children and/or youth (<25 years) with OFCs were studied, and (2) neurodevelopmental or academic outcomes were included. DATA EXTRACTION: The authors independently evaluated study quality and extracted outcome data. RESULTS: Thirty-one studies involving 10 143 patients with OFCs and 2 017 360 controls met eligibility criteria. Although the quality of the studies varied, patients with OFCs consistently performed worse than their peers on neurodevelopmental and academic measures. In infancy, differences were observed on multiple developmental outcomes (eg, cognition, motor skills, and language), and in later childhood and adolescence, differences were manifest on several indicators of academic achievement (eg, use of special education services, grades, and scores on standardized measures). LIMITATIONS: Heterogeneity in study designs, methods, and outcomes prevented statistical pooling and modeling for meta-analysis. CONCLUSIONS: Children with OFCs exhibit neurodevelopmental and academic deficits compared with their unaffected peers. Although the nature of these deficits changes with development, differences are observed from infancy through adolescence. Clinicians should monitor neurodevelopment in children with OFCs and support them appropriately.

An Algorithm for the Assessment of Facial Asymmetry in Children With Focus on Etiology and Treatment.

BACKGROUND: Facial asymmetry is a common referral indication for craniofacial teams but has a wide range of causes. Prompt identification of etiology is critical to treatment, as medical and surgical interventions vary depending on the cause of asymmetry in each patient. SOLUTION: A standardized diagnostic algorithm. WHAT WE DID THAT IS NEW: We developed an algorithm to assist in the diagnostic evaluation of facial asymmetry with a focus on next steps for medically actionable causes.

Defining the Elements of Early Palliative Care That Are Associated With Patient-Reported Outcomes and the Delivery of End-of-Life Care.

Purpose We describe the key elements of early palliative care (PC) across the illness trajectory and examine whether visit content was associated with patient-reported outcomes and end-of-life care. Methods We performed a secondary analysis of patients with newly diagnosed advanced lung or noncolorectal GI cancer (N = 171) who were randomly assigned to receive early PC. Participants attended at least monthly visits with board-certified PC physicians and advanced practice nurses at Massachusetts General Hospital. PC clinicians completed surveys documenting visit content after each encounter. Patients reported quality of life (Functional Assessment of Cancer Therapy-General) and mood (Hospital Anxiety and Depression Scale and Patient Health Questionnaire-9) at baseline and 24 weeks. End-of-life care data were abstracted from the electronic health record. We summarized visit content over time and used linear and logistic regression to identify whether the proportion of visits addressing a content area was associated with patient-reported outcomes and end-of-life care. Results We analyzed data from 2,921 PC visits, most of which addressed coping (64.2%) and symptom management (74.5%). By 24 weeks, patients who had a higher proportion of visits that addressed coping experienced improved quality of life ( P = .02) and depression symptoms (Depression subscale of the Hospital Anxiety and Depression Scale, P = .002; Patient Health Questionnaire-9, P = .004). Patients who had a higher proportion of visits address treatment decisions were less likely to initiate chemotherapy ( P = .02) or be hospitalized ( P = .005) in the 60 days before death. Patients who had a higher proportion of visits addressing advance care planning were more likely to use hospice ( P = .03). Conclusion PC clinicians' focus on coping, treatment decisions, and advance care planning is associated with improved patient outcomes. These data define the key elements of early PC to enable dissemination of the integrated care model.

Evaluating Trends in Headache and Revision Surgery following Cranial Vault Remodeling for Craniosynostosis.

BACKGROUND: The recommended treatment for craniosynostosis, is cranial vault expansion to prevent increased intracranial pressure and optimize developmental outcomes. Some patients complain about postoperative headaches and occasionally require revision to treat increased intracranial pressure. This study examines whether specific factors are associated with an increased risk of postoperative headaches or intracranial hypertension. METHODS: This retrospective cohort included patients with craniosynostosis from 1995 and 2010. Primary outcomes included headaches and delayed intracranial hypertension. Logistic regression was used to evaluate the associations with clinical characteristics. RESULTS: The cohort included 383 patients, of whom 127 (33 percent) complained of headaches. The positive predictive value of a headache indicating intracranial hypertension was only 9.4 percent among all patients and 6.7 percent among patients with nonsyndromic craniosynostosis. Headaches occurring in the morning, more than once per week, and associated with nausea, vomiting, or decreased activity level were most likely to be associated with delayed intracranial hypertension. Only 21 patients (6 percent) required revision surgery, and these patients were more likely to have syndromic craniosynostosis (OR, 5.6; 95 percent CI, 2.1 to 14.9), Chiari malformation (OR, 5.8; 95 percent CI, 1.7 to 19.5), or secondary craniosynostosis (additional sutures fused on the 2-year postoperative CT scan) (OR, 5.4; 95 percent CI, 2.2 to 13.5). CONCLUSIONS: Headaches are common after cranial vault remodeling but are not very predictive of who will need revision surgery for intracranial hypertension. Patients with specific headache characteristics, syndromic craniosynostosis, secondary synostosis, and Chiari malformations have the highest risk of developing delayed intracranial hypertension. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Differential effects of early palliative care based on the age and sex of patients with advanced cancer from a randomized controlled trial.

BACKGROUND: Early palliative care interventions enhance patient outcomes, including quality of life, mood, and coping, but it remains unclear whether certain subgroups of patients are more likely to benefit from early palliative care. We explored whether age and sex moderate the improved outcomes seen with early palliative care. METHODS: We performed a secondary analysis of data from a randomized trial of 350 patients with advanced lung and non-colorectal gastrointestinal cancer. Patients received an early palliative care intervention integrated with oncology care or usual oncology care alone. We used linear regression to determine if age (older or younger than 65) and sex moderated the effects of the intervention on quality of life (Functional Assessment of Cancer Therapy-General (FACT-G)), depression symptoms (Patient Health Questionnaire 9 (PHQ-9)), and coping (Brief COPE) within lung and gastrointestinal subgroups. RESULTS: At 24 weeks, younger patients with lung cancer receiving early palliative care reported increased use of active coping ( B = 1.74; p = 0.02) and decreased use of avoidant coping ( B = -0.97; p = 0.02), but the effects of early palliative care on these outcomes were not significant for older patients. Male patients with lung cancer assigned to early palliative care reported better quality of life (FACT-G: B = 9.31; p = 0.01) and lower depression scores (PHQ-9: B = -2.82; p = 0.02), but the effects of early palliative care on these outcomes were not significant for female patients. At 24 weeks, we found no age or sex moderation effects within the gastrointestinal cancer subgroup. CONCLUSION: Age and sex moderate the effects of early palliative care for patients with advanced lung cancer. Early palliative care may need to be tailored to individuals' unique sociodemographic and clinical characteristics.

Sarcopenia Is Associated with Quality of Life and Depression in Patients with Advanced Cancer.

BACKGROUND: Patients with advanced cancer often experience muscle wasting (sarcopenia), yet little is known about the characteristics associated with sarcopenia and the relationship between sarcopenia and patients' quality of life (QOL) and mood. MATERIALS AND METHODS: As part of a randomized trial, we assessed baseline QOL (Functional Assessment of Cancer Therapy-General [FACT-G]) and mood (Hospital Anxiety and Depression Scale [HADS]) in patients within 8 weeks of diagnosis of incurable lung or gastrointestinal cancer, and prior to randomization. Using computed tomography scans collected as part of routine clinical care, we assessed sarcopenia at the level of the third lumbar vertebra with validated sex-specific cutoffs. We used logistic regression to explore characteristics associated with presence of sarcopenia. To examine associations between sarcopenia, QOL and mood, we used linear regression, adjusted for patients' age, sex, marital status, education, and cancer type. RESULTS: Of 237 participants (mean age = 64.41 ± 10.93 years), the majority were male (54.0%) and married (70.5%) and had lung cancer (56.5%). Over half had sarcopenia (55.3%). Older age (odds ratio [OR] = 1.05, p = .002) and education beyond high school (OR = 1.95, p = .047) were associated with greater likelihood of having sarcopenia, while female sex (OR = 0.25, p < .001) and higher body mass index (OR = 0.79, p < .001) correlated with lower likelihood of sarcopenia. Sarcopenia was associated with worse QOL (FACT-G: B = -4.26, p = .048) and greater depression symptoms (HADS-depression: B = -1.56, p = .005). CONCLUSION: Sarcopenia was highly prevalent among patients with newly diagnosed, incurable cancer. The associations of sarcopenia with worse QOL and depression symptoms highlight the need to address the issue of sarcopenia early in the course of illness. IMPLICATIONS FOR PRACTICE: This study found that sarcopenia, assessed using computed tomography scans acquired as part of routine clinical care, is highly prevalent in patients with newly diagnosed, incurable cancer. Notably, patients with sarcopenia reported worse quality of life and greater depression symptoms than those without sarcopenia. These findings highlight the importance of addressing muscle loss early in the course of illness among patients with incurable cancer. In the future, investigators should expand upon these findings to develop strategies for assessing and treating sarcopenia while striving to enhance the quality of life and mood outcomes of patients with advanced cancer.