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Hemifacial hyperplasia (HFH) is a rare congenital disorder characterized by marked unilateral overgrowth of the facial tissues. A subtype of HFH is congenital infiltrating lipomatosis of the face (CIL-F). This disease is characterized by unilateral diffuse infiltration of mature adipose cells in the facial soft tissue and is associated with skeletal hypertrophy. This work aims to report a case of a CIL-F patient with right facial asymmetry and progressive growth at adolescent age, causing mandibular asymmetry due to signs of concomitant unilateral condylar hyperplasia. At the age of seventeen, a condylectomy was performed to stop the progression of asymmetric mandibular growth. Five years later, the patient developed CIL-F-associated temporomandibular joint ankylosis, manifesting as progressive restricted mouth opening along with temporal facial pain. In this CIL-F patient, a TMJ reconstruction with an alloplastic total joint prosthesis was successfully performed with optimal maximal mouth opening, complete alleviation of temporal facial pain, and stable dental occlusion one year postoperatively. A TMJ reconstruction with a complete alloplastic total joint prosthesis proved to be a predictable, stable, and safe treatment option in a patient with CIL-F-associated TMJ ankylosis who was previously treated with condylectomy due to progressive mandibular asymmetry. [ABSTRACT FROM AUTHOR]

Objectives : To (1) appraise current international classification and clinical management strategies for craniofacial microsomia (CFM) and microtia, and (2) to assess agreement with the European Reference Network "European Guideline Craniofacial Microsomia" recommendations on screening and monitoring. Design : This was a cross-sectional online survey study. The survey consisted of 44 questions on demographics, diagnostics and classification, obstructive sleep apnea, feeding difficulties, speech and language development, hearing, ocular abnormalities, visual development, orthodontic screening, genetic counselling, psychological wellbeing, and extracraniofacial anomalies. Participants : Respondents were participants of 3 international cleft and craniofacial conferences, members of the American Cleft Palate and Craniofacial Association and members of the International Society for Auricular Reconstruction. Respondents were requested to complete 1 questionnaire per multidisciplinary team. Results : Fifty-seven responses were received from 30 countries (response rate ∼3%).The International Consortium for Health Outcomes Measurement diagnostic criteria were used by 86% of respondents, though 65% considered isolated microtia a mild form of CFM. The Orbit, Mandible, Ear, Facial Nerve and Soft Tissue classification system was used by 74% of respondents. Agreement with standardized screening and monitoring recommendations was between 61% and 97%. A majority of respondents agreed with screening for extracraniofacial anomalies (63%-68%) and with genetic counselling (81%). Conclusions : This survey did not reveal consistent agreement on the diagnostic criteria for CFM. Respondents mostly supported management recommendations, but frequently disagreed with the standardization of care. Future studies could focus on working towards international consensus on diagnostic criteria, and exploring internationally feasible management strategies. [ABSTRACT FROM AUTHOR]

Objective : Measuring the impact of orthognathic surgery on quality of life is of significant importance in patients with cleft deformities. Standardized tools such as patient-reported outcome measures (PROMs) are needed to fully comprehend patients' needs and perceptions. Therefore, the availability of reliable, valid, and comprehensive questionnaires for patients is essential. The aim of this study is to identify PROMs measuring the impact of orthognathic surgery on quality of life in patients with cleft deformities and to evaluate the identified PROMs. Methods : A systematic search of the literature was performed according to the preferred reporting items for systematic reviews and meta-analysis guidelines. All validated PROMs, regarding the impact of orthognathic surgery on quality of life in patients with cleft deformities, were identified and assessed according to the quality criteria proposed for measurement properties of health status questionnaires. Results : An electronic search yielded 577 articles. After a full-text review of 87 articles, 4 articles met the inclusion criteria, comprising 58 PROMs. Of these 58 PROMs, 1 PROM (the CLEFT-Q) has been validated to measure the impact of orthognathic surgery on patients with a facial cleft. Evaluation of methodological quality of the included articles and assessment of the measurement properties of the CLEFT-Q show that the CLEFT-Q scores relatively good for all available measurement properties, making it suitable for immediate use. Conclusion: The CLEFT-Q was found to be the only valid instrument so far to measure the impact of orthognathic surgery on the quality of life in patients with cleft deformities. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to validate the Dutch version of the Orthognathic Quality of Life Questionnaire (OQLQ-NL) for cleft patients who received orthognathic surgery., Methods: To compare the OQLQ-NL with the CLEFT-Q, we used a convenience sample of thirty-two cleft patients. Using the Cronbach's alpha coefficient for multiple item scales, internal reliabilities of the OQLQ-NL were evaluated. The OQLQ-NL was repeated at a two-week interval and the intraclass correlation coefficient was calculated, to establish of the test-retest reliability. The construct validity of the OQLQ-NL was evaluated by using Spearman's correlation to test its correlation with the CLEFT-Q., Results: Thirty-two patients filled in the OQLQ-NL and CLEFT-Q. The OQLQ-NL had excellent results in internal reliability and test-retest reliability. The vast majority of the correlations between the domains and scales of the questionnaires were as expected. Data from this study and previous studies confirm the construct validity of the OQLQ-NL., Conclusion: Our results suggest the OQLQ-NL is a valid and reliable instrument for measuring quality of life in cleft patients who have received orthognathic surgery in the Netherlands., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: The severity of craniofacial microsomia (CFM) is generally classified using the Orbit, Mandible, Ear, Soft tissue, Nerve (OMENS) classification score. The global assessment of the Phenotypic Assessment Tool for Craniofacial Microsomia (PAT-CFM), is a pictorial modification of the OMENS classification. The aim of this study was to assess the interrater reliability of the PAT-CFM global assessment score., Design: In this prospective cohort study, three clinicians completed the global assessment form of the PAT-CFM. The mandible was classified based on orthopantomogram- and/or computed tomography images., Participants: Consecutive patients with CFM or microtia., Interrater agreement was calculated using the weighted Krippendorff alpha (α), with 95% confidence intervals (CI)., Results: In total, 53 patients were included (106 hemifaces). The reliabilities of the main classification components ranged from high for the mandible (α = 0.904 [95% CI 0.860-0.948]) and ear (α = 0.958 [95% CI 0.934-0.983]) subscales, to tentative for the orbital summary score (α = 0.682 [0.542-0.821]), and nerve summary score (α = 0.782 [0.666-0.900]) subscales., Conclusions: The reliability of the ear and radiographic mandible scales of the PAT-CFM global classification were high, while the orbit, facial nerve and soft tissue subscales may have limited reliability. Research focusing on radiographic severity scores for hypoplasia of the orbits and soft tissues, as well as objective measures for overall facial hypoplasia using non-ionizing forms of imaging for early classification, are warranted., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

To (1) appraise current international classification and clinical management strategies for craniofacial microsomia (CFM) and microtia, and (2) to assess agreement with the European Reference Network "European Guideline Craniofacial Microsomia" recommendations on screening and monitoring., This was a cross-sectional online survey study. The survey consisted of 44 questions on demographics, diagnostics and classification, obstructive sleep apnea, feeding difficulties, speech and language development, hearing, ocular abnormalities, visual development, orthodontic screening, genetic counselling, psychological wellbeing, and extracraniofacial anomalies., Respondents were participants of 3 international cleft and craniofacial conferences, members of the American Cleft Palate and Craniofacial Association and members of the International Society for Auricular Reconstruction. Respondents were requested to complete 1 questionnaire per multidisciplinary team., Fifty-seven responses were received from 30 countries (response rate ∼3%).The International Consortium for Health Outcomes Measurement diagnostic criteria were used by 86% of respondents, though 65% considered isolated microtia a mild form of CFM. The Orbit, Mandible, Ear, Facial Nerve and Soft Tissue classification system was used by 74% of respondents. Agreement with standardized screening and monitoring recommendations was between 61% and 97%. A majority of respondents agreed with screening for extracraniofacial anomalies (63%-68%) and with genetic counselling (81%)., This survey did not reveal consistent agreement on the diagnostic criteria for CFM. Respondents mostly supported management recommendations, but frequently disagreed with the standardization of care. Future studies could focus on working towards international consensus on diagnostic criteria, and exploring internationally feasible management strategies.

Measuring the impact of orthognathic surgery on quality of life is of significant importance in patients with cleft deformities. Standardized tools such as patient-reported outcome measures (PROMs) are needed to fully comprehend patients' needs and perceptions. Therefore, the availability of reliable, valid, and comprehensive questionnaires for patients is essential. The aim of this study is to identify PROMs measuring the impact of orthognathic surgery on quality of life in patients with cleft deformities and to evaluate the identified PROMs., A systematic search of the literature was performed according to the preferred reporting items for systematic reviews and meta-analysis guidelines. All validated PROMs, regarding the impact of orthognathic surgery on quality of life in patients with cleft deformities, were identified and assessed according to the quality criteria proposed for measurement properties of health status questionnaires., An electronic search yielded 577 articles. After a full-text review of 87 articles, 4 articles met the inclusion criteria, comprising 58 PROMs. Of these 58 PROMs, 1 PROM (the CLEFT-Q) has been validated to measure the impact of orthognathic surgery on patients with a facial cleft. Evaluation of methodological quality of the included articles and assessment of the measurement properties of the CLEFT-Q show that the CLEFT-Q scores relatively good for all available measurement properties, making it suitable for immediate use., The CLEFT-Q was found to be the only valid instrument so far to measure the impact of orthognathic surgery on the quality of life in patients with cleft deformities.