Temporomandibular Joint Prosthesis in a Patient with Congenital Infiltrating Lipomatosis of the Face with Bony Ankylosis of the Temporomandibular Joint: A Case Report.

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.

Management of pediatric blunt abdominal trauma in a Dutch level one trauma center.

PURPOSE: Most children with intra-abdominal injuries can be managed non-operatively. However, in Europe, there are many different healthcare systems for the treatment of pediatric trauma patients. Therefore, the aim of this study was to describe the management strategies and outcomes of all pediatric patients with blunt intra-abdominal injuries in our unique dedicated pediatric trauma center with a pediatric trauma surgeon. METHODS: We performed a retrospective, single-center, cohort study to investigate the management of pediatric patients with blunt abdominal trauma. From the National Trauma Registration database, we retrospectively identified pediatric (≤ 18 years) patients with blunt abdominal injuries admitted to the UMCU from January 2012 till January 2018. RESULTS: A total of 121 pediatric patients were included in the study. The median [interquartile range (IQR)] age of patients was 12 (8-16) years, and the median ISS was 16 (9-25). High-grade liver injuries were found in 12 patients. Three patients had a pancreas injury grade V. Furthermore, 2 (1.6%) patients had urethra injuries and 10 (8.2%) hollow viscus injuries were found. Eighteen (14.9%) patients required a laparotomy and 4 (3.3%) patients underwent angiographic embolization. In 6 (5.0%) patients, complications were found and in 4 (3.3%) children intervention was needed for their complication. No mortality was seen in patients treated non-operatively. One patient died in the operative management group. CONCLUSIONS: In conclusion, it is safe to treat most children with blunt abdominal injuries non-operatively if monitoring is adequate. These decisions should be made by the clinicians operating on these children, who should be an integral part of the entire group of treating physicians. Surgical interventions are only needed in case of hemodynamic instability or specific injuries such as bowel perforation.

Pathophysiology and Individualized Treatment of Hypothalamic Obesity Following Craniopharyngioma and Other Suprasellar Tumors: A Systematic Review.

The development of hypothalamic obesity (HO) following craniopharyngioma (CP) and other suprasellar tumors leads to reduced patient quality of life. No treatment algorithms are currently available for management of HO. Depending on which hypothalamic nuclei are destroyed, the pathophysiologic mechanisms and clinical symptoms that contribute to HO differ among patients. Herein, we review the contribution of the hypothalamus to the pathophysiologic mechanisms and symptoms underlying CP-associated HO. Additionally, we performed a systematic search of MEDLINE and Embase to identify all intervention studies for weight management in patients with CP or other suprasellar tumors published until September 2017. The search yielded 1866 publications, of which 40 were included. Of these 40 studies, we identified four modalities for intervention (i.e., lifestyle, dietary, pharmacotherapeutic, or surgical) within six clinical domains (i.e., psychosocial disorders, hyperphagia, sleep disturbances, decreased energy expenditure, hyperinsulinemia, and hypopituitarism). We used the findings from our systematic review, in addition to current knowledge on the pathophysiology of HO, to develop an evidence-based treatment algorithm for patients with HO caused by CP or other suprasellar tumors. Although the individual effects of the HO interventions were modest, beneficial individual effects may be achieved when the pathophysiologic background and correct clinical domain are considered. These two aspects can be combined in an individualized treatment algorithm with a stepwise approach for each clinical domain. Recently elucidated targets for HO intervention were also explored to improve future management of HO for patients with CP and other suprasellar tumors.