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BACKGROUND: Objectively determining tissue loss in craniofacial morphea is challenging. However, 3-dimensional (3D) stereophotogrammetry is a noninvasive modality that may be a useful adjunct. OBJECTIVE: To prospectively evaluate 3D stereophotogrammetry in the assessment of craniofacial linear morphea. METHODS: Participants underwent clinical, quality-of-life, and 3D-stereophotogrammetry assessments. Traditional photographs and 3D-stereophotogrammetry images were rated as mild, moderate, or severe by 2 experts and 2 nonexperts. In addition, interrater and intrarater reliability (on delayed rescoring) were calculated. RESULTS: Of 23 patients with craniofacial morphea, 3D stereophotogrammetry detected pathologic asymmetry in 14 (20.6%) patients. Providers rated patients as more severely affected when using 3D stereophotogrammetry versus when using traditional photographs (19% severe on 3D stereophotogrammetry vs 0% severe on traditional photographs, P = .004). Qualitative ratings of both traditional and 3D images showed high inter- and intrarater reliability between experts and nonexperts alike. Physicians' Global Assessment of Damage scores correlated with mouth asymmetry (P = .0021), cheek asymmetry (P = .04), and 3D-stereophotogrammetry ratings (median, mild: 27.5 vs moderate: 46.5 vs severe: 64, P = .0152). Lower face asymmetry correlated with worse quality-of-life scores (P = .013). LIMITATIONS: Small sample size and cross-sectional design. CONCLUSION: 3D stereophotogrammetry can reliably detect and quantify asymmetry in craniofacial morphea with greater sensitivity than that observed with traditional assessment alone. 3D stereophotogrammetry may be a useful adjunct to clinical examination.
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Esclerodermia Localizada , Humanos , Esclerodermia Localizada/complicaciones , Esclerodermia Localizada/diagnóstico por imagen , Estudios Transversales , Reproducibilidad de los Resultados , Fotogrametría , CaraRESUMEN
This study used three-dimensional measurements to provide a nasolabial analysis of patients with unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), and controls across different races and ethnicities.A retrospective comparative study.Tertiary care pediatric institution.The study included 90 patients with UCLP, 43 patients with BCLP, and 90 matched controls. Patients are separated as self-identified Caucasian, Hispanic, or African American.Nasal length, nasal protrusion, columellar height, columellar width, tip width, alar width, alar base width, nasolabial angle, upper lip length, philtrum length, nostril height, and nostril width.All UCLP groups had significantly greater columella and tip widths and decreased nasolabial angles than controls. All BCLP groups had significantly greater columella width, tip width, nasolabial angle, and nostril widths. Upper lip length, philtrum length, and nostril height were significantly decreased in BCLP compared to controls. Across UCLP groups, African Americans had significantly decreased nasal protrusion and columella height and a significantly increased columella width compared to Caucasians and Hispanics. Alar and alar base widths were significantly different between all groups. Across BCLP groups, the Caucasian nostril width was significantly less than the African Americans.These findings suggest that when correcting nasolabial characteristics in patients with cleft lip, it is important to consider racial and ethnic differences to achieve a normal appearance. Specifically, goals for alar width, alar base width, nasal tip, and projection should be tailored to the patient's race and ethnicity.
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BACKGROUND: Sagittal craniosynostosis results in varying degrees of frontal bossing and bilateral temporal pinching. This study assessed the three-dimensional changes in these regions using curvature analysis and volumetric analysis before and 1 year after extended sagittal strip craniectomy (ESC) with postoperative helmet therapy. METHODS: A retrospective review of three-dimensional photographs of 50 subjects treated with ESC with postoperative helmet therapy and 50 age-matched controls was performed. Images were collected preoperatively and 1 year postoperatively. Forehead convexity and temple concavity were quantified. Computed tomographic scans of subjects with and without sagittal synostosis were analyzed to assess the percentage of total intracranial volume (ICV) in the anterior cranial fossa before and after ESC with postoperative helmet therapy. RESULTS: Forehead convexity in the ESC with postoperative helmet therapy group preoperatively (24.49 ± 3.16 m -1 ) was significantly greater than controls (22.48 ± 3.84 m -1 ; P = 0.005). Forehead convexity significantly decreased after ESC with postoperative helmet therapy (18.79 ± 2.43 m -1 ; P < 0.001) and did not differ from controls (19.67 ± 3.08 m -1 ; P = 0.115). The ESC group had more concave temples preoperatively (-10.27 ± 4.37 m -1 ) as compared with controls (-6.99 ± 3.55 m -1 ; P < 0.001). Temple concavity significantly decreased after ESC (-4.82 ± 3.17 m -1 ; P < 0.001) and did not differ from controls (-5.64 ± 3.27 m -1 ; P = 0.075). In the ESC group, the percentage ICV in the anterior cranial fossa decreased from 22.03% to 18.99% after surgery, whereas the anterior volume in controls was stable (17.74% to 16.81%). CONCLUSIONS: The ESC group had significantly greater forehead convexity, temple concavity and anterior cranial fossa volume compared with controls. One year after ESC with postoperative helmet therapy, forehead convexity, temple concavity, and percentage ICV in the anterior fossa were comparable to controls. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
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Craneosinostosis , Humanos , Lactante , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/cirugía , Huesos Faciales/cirugía , Craneotomía/métodos , Estudios Retrospectivos , Frente/diagnóstico por imagen , Frente/cirugíaRESUMEN
Breast cancer in trans women is rare. Only 21 cases have been reported worldwide. Multidisciplinary teams must balance oncologic treatment with patient goals. Here we describe a case of invasive ductal carcinoma in a transgender woman who was found to have a BRCA2 gene mutation. A shared decision-making process led to the patient undergoing bilateral nipple-sparing mastectomy with immediate tissue expander placement. Later findings prompted discussions about adjuvant chemotherapy and radiation. Additionally, we discuss the complexities associated with reconstructing a transfeminine chest.
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Signaling via the tropomyosin-related kinase receptor subtype B (TrkB) regulates neuromuscular transmission, and inhibition of TrkB kinase activity by 1NMPP1 in TrkBF616A mice worsens neuromuscular transmission failure (NMTF). We hypothesized that acute inhibition of TrkB kinase activity will impair the ability of the diaphragm muscle to produce maximal transdiaphragmatic pressure (Pdi) without impacting the ability to generate forces associated with ventilation, consistent with the greater susceptibility to NMTF in motor units responsible for higher-force nonventilatory behaviors. Adult male and female TrkBF616A mice were injected with 1NMPP1 (n = 8) or vehicle (DMSO; n = 8) 1 h before Pdi measurements during eupneic breathing, hypoxia/hypercapnia (10% O2/5% CO2), tracheal occlusion, spontaneous deep breaths ("sighs") and during maximal activation elicited by bilateral phrenic nerve stimulation. In the vehicle-treated group, Pdi increased from ~10 cmH2O during eupnea and hypoxia/hypercapnia, to ~35 cmH2O during sighs and tracheal occlusion, and to ~65 cm H2O during maximal stimulation. There was no effect of acute 1NMPP1 treatment on Pdi generated during most behaviors, except during maximal stimulation (~30% reduction; P < 0.05). This reduction in maximal Pdi is generally similar to the worsening of NMTF previously reported with TrkB kinase inhibition in rodents. Accordingly, impaired TrkB signaling limits the range of motor behaviors accomplished by the diaphragm muscle and may contribute to neuromuscular dysfunction, primarily by impacting fatigable, higher force-generating motor units.NEW & NOTEWORTHY TrkB signaling plays an important role in maintaining neuromuscular function in the diaphragm muscle and may be necessary to accomplish the various motor behaviors ranging from ventilation to expulsive, behaviors requiring near-maximal forces. This study shows that inhibition of TrkB kinase activity impairs maximal pressure generation by the diaphragm muscle, but the ability to generate the lower pressures required for ventilatory behaviors is not impacted.