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Background: The etiology and treatment of complex mandibular defects in children differ markedly from those of adults, although treatment with free bone flaps is historical in both groups. While adult outcomes and complication rates are well known, few pediatric data exist, especially for patients with congenital deficiencies. This study reports early and late outcomes from a cohort of young, primarily syndromic patients undergoing microvascular mandibular reconstruction. Methods: This is a retrospective case series of patients who underwent microvascular mandibular reconstruction between 1995 and 2016. Results: Thirteen patients received a total of 13 fibula transfers and 1 medial femoral condyle transfer. Most patients carried a congenital diagnosis (77%), and the average age during surgery was 11.7 ± 5.7 years. The median (interquartile range) [IQR] length of follow-up was 6.3 (5.7) years. There was a 100% flap survival rate, although 86% of all patients experienced at least one complication. Half of all procedures resulted in an early complication. Nine patients (69%) developed late complications, of which temporal mandibular joint ankylosis was the most common (n = 5; 38%). Conclusions: This study is one of few detailing outcomes following mandibular reconstruction by free flap transfer in pediatric patients. These patients were primarily syndromic with appreciable complication rates higher than in other adult and pediatric studies. Some complications are manageable or self-resolving, but others lead to functional problems that may require late operative interventions to correct. Microsurgical treatment should be reserved for children with large, complex mandibular defects when other options are unavailable or have been exhausted.

Ocular anomalies may occur in craniofacial microsomia (CFM). The aim of this systematic review was to review the literature on ocular anomalies and their incidence, in order to estimate the need for ophthalmological screening in CFM patients. Online databases were searched, and data on the number of patients, type and incidence of ocular anomalies, and visual acuity were extracted. Four subgroups of ocular and adnexal anomalies were identified, to provide an overview of the different anomalies. Twenty-five papers analysing 1419 patients in total were included. Ocular anomalies were documented in 6.7–100% of patients. The most reported type I ocular anomalies were eyelid coloboma, lipodermoids, and orbital dystopia. The most reported type II ocular anomalies were epibulbar dermoid, microphthalmia, and anophthalmia. Ptosis a

Objective: To provide an overview on the prevalence and types of dental anomalies in patients with craniofacial microsomia (CFM). Eligibility criteria: Inclusion criteria were CFM and dental anomalies. The following data were extracted: number of patients, methodology, mean age, sex, affected side, severity of mandibular hypoplasia, dentition stage and dental a

Purpose: Obstructive sleep apnea (OSA) is a common problem in patients with craniofacial microsomia (CFM); however, the exact pathophysiology in patients with CFM remains unclear. The first aim of this study was to evaluate upper airway volume and morphology in patients with CFM. The second aim was to identify risk factors for the presence of OSA. Materials and Methods: A cross-sectional study was set up and 3 study groups were identified: 1) CFM with OSA, 2) CFM without OSA, and 3) control. Computed tomographic (CT) scans of the head and neck were included and used to create 3-dimensional models. The age-matched control group consisted of patients evaluated for traumatic head injury or epilepsy. Volumetric and morphologic parameters were measured. The results of patients with CFM were compared among the 3 study groups. Descriptive statistics were computed using the Pearson c2 test for categorical variables and nonparametric tests for continuous variables. A multiple variable regression model was used to identify risk factors for OSA. Results: In total, 79 patients with CFM were included, of which 25 patients were diagnosed with OSA. A total of 145 CT scans could be analyzed. In addition, a control population of 88 patients was identified. Oropharynx volume, mean cross-sectional area (CSA), minimal CSA, and minimal retropalatal area were found to be markedly smaller in patients with CFM compared with the control population. In contrast, in patients with CFM and OSA, minimal retroglossal area, sphericity, and uniformity markedly differed from those in patients without OSA. Sphericity was identified as the main predicting variable of OSA in patients with CFM.

Craniofacial microsomia (CFM) is characterized by underdevelopment of the structures derived from the first and second pharyngeal arches resulting in aesthetic, psychological, and functional problems including feeding and swallowing difficulties. The aim of this study is to gain more insight into swallowing difficulties in patients with CFM. A retrospective study was conducted in the population of patients diagnosed with CFM at three major craniofacial units. Patients with feeding difficulties and those who underwent video fluoroscopic swallow (VFS) studies were included for further analyses. The outcome of the VFS-studies was reviewed with regard to the four phases of swallowing. In our cohort, 13.5% of the 755 patients were diagnosed with swallowing difficulties. The outcome of the VFS-studies of 42 patients showed difficulties in the oral and pharyngeal phases with both thin and thick liquids. Patients with more severe mandibular hypoplasia showed more difficulties to form an appropriate bolus compared to patients who were less severely affected. This is the first study to document swallowing problems in patients with CFM. Difficulties were seen in both the oral and pharyngeal phases. We recommend routine screening for swallowing issues by a speech and language therapist in all patients with CFM and to obtain a VFS-study in patients with a type III mandible.