(1) Background: Despite cleft lips and palates belonging to the most common orofacial congenital anomalies, their morphopathogenesis is not yet fully understood. The study aimed to determine the distribution and relation of cytokines interferon-γ (IFN-γ), tumor necrosis factor-alpha (TNF-α), interleukin (IL)-2, IL-7, IL-12, and IL-13 in the cleft affected mucosa of the lip. (2) Materials and Methods: Twenty cleft lip (CL) mucosal samples and seven control tissues of oral cavity mucosa were included in the study. Specimen were obtained during reconstruction surgeries and processed by hematoxylin and eosin staining and immunohistochemistry for IFN-γ, TNF-α, IL-2, IL-7, IL-12, and IL-13. (3) Results: The distribution of cytokines was higher overall in the cleft affected epithelium compared to the connective tissue, with TNF-a, IL-2, and IL-12 displaying the highest number of immunopositive cells. With the exception of IL-2, CL specimen showed higher immunoreactivity. IFN-γ displayed only minor immunoreactivity, with no expression in the control epithelium. Correlation analysis was strongest between CL epithelial IL-13 and IFN-γ (z = 0.71, p < 0.0001). (4) Conclusions: The CLP affected epithelium displays high degrees of plasticity in expressing different cytokines, pointing towards the stimulation of a local adaptive immune response based on consistent inflammatory processes.

Patient: Female, 61Final Diagnosis: Pulmonary hypertensionSymptoms: DyspnoeaMedication: —Clinical Procedure: —Specialty: CardiologyObjective:Unusual setting of medical careBackground:Kyphoscoliosis is an anatomical deformity of the spine often accompanied by an array of respiratory complications, pulmonary hypertension being among the most severe ones. At present, evidence-based treatment options for kyphoscoliosis-related pulmonary hypertension remain limited to the correction of hypoxemia through ventilatory support and long-term oxygenation.Case Report:We report a case of a 61-year-old female with severe kyphoscoliosis-related pulmonary hypertension who was admitted to a university hospital in September 2018 due to progressive dyspnea and respiratory failure. She was diagnosed with pulmonary hypertension in 2016 and had been on endothelin receptor antagonist (ambrisentan) and oxygen therapy ever since. Upon admission, the patient presented with severe depression of peripheral oxygen saturation (SpO2 at 75%). The patient declined further treatment hours after hospitalization, despite optimized supportive oxygen therapy. Ambrisentan was discontinued and replaced by inhaled iloprost. Over the course of the next 4 days, the patient showed symptomatic improvement and was discharged on Day 5. Right heart catheterization follow-up in February 2019 showed no worsening in pulmonary hemodynamic parameters compared to the time of initial diagnosis.Conclusions:Managing the respiratory decline in kyphoscoliosis-related pulmonary hypertension can be challenging since these patients tend to deteriorate despite current treatment options. Our case reports on the use of vasoactive agents as a safe and effective treatment option in addition to established therapeutic regimen.

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the subgroup of pre-chiasmatic gliomas, intraorbital and intradural resection is a curative option after blindness. We present a two-center cohort using different surgical approaches. A retrospective analysis was performed, including 10 children. Mean age at surgery was 6.8 years. Interval between diagnosis and surgery was 1–74 (mean 24 ± 5.5, median 10) months. Indications for surgery were exophthalmos, pain, tumor progression, or a combination. Eight patients underwent an extradural trans-orbital-roof approach to resect the intra-orbital tumor, including the optic canal part plus intradural pre-chiasmatic resection. Gross total resection was achieved in 7/8, and none had a recurrence. One residual behind the bulbus showed progression, treated by chemotherapy. In two patients, a combined supra-orbital mini-craniotomy plus orbital frame osteotomy was used for intraorbital tumor resection + intradural pre-chiasmatic dissection. In these two patients, remnants of the optic nerve within the optic canal remained stable. No patient had a chiasmatic functional affection nor permanent oculomotor deficits. In selected patients, a surgical resection from bulb to chiasm ± removal of optic canal tumor was safe without long-term sequela and with an excellent cosmetic result. Surgery normalizes exophthalmos and provides an effective tumor control.

Introduction. Midline orofacial defects bear a high degree of morbidity for the affected individual, often associated with retardation in neonatal development and the need for surgical intervention. The etiology of the deformities, despite their high prevalence, remains, however, largely unknown. We report on the evaluation and quantification of proliferative and inflammatory markers in the cleft affected soft palate. Material and methods. This study included soft palate samples of eight cleft lip and palate affected individuals and a control group of six soft tissue specimens obtained during correctional surgery of hyperdentia. All samples were processed via immunohistochemistry for marker EGFR, NF-κB, Ki-67, Ma-1. The results were evaluated semi-quantitatively and statistically analysed by IBM SPSS 25.0. Results. Histopathological signs of inflammatory changes were continuous in cleft affected specimen. The quantitative distribution of the markers in the cleft affected group displayed a significant correlation between EGFR, Ki-67 and NF-κB (p< 0.05), along with a correlation among Ki-67 and NF-κB (p< 0.05). Immunoreactive structures in control group showed lower numbers in all evaluated specimen. A statistical significance between cleft affected and non-cleft tissue was observed in EGFR and Ma-1 (p< 0.05). Conclusion. Results are suggestive of a tissue phenotype modification in cleft affected palate. Observation of distribution and statistical correlation hint towards the involvement of epithelial growth inducer EGFR and inflammatory/ proliferative marker in epithelial changes.

INTRODUCTION: Optic pathway gliomas (OPG) in children carry significant morbidity and therapeutic challenges. The subgroup of pre-chiasmatic gliomas manifest with exopthalmus are a subgroup where, after blindness has occurred, an intraorbital and intradural resection is a curative option. We present a two-center cohort using two different surgical approaches and describe indication, technique, and long term surgical outcome. METHODS: A retrospective analysis in both centers was performed to included patients &lt; 18 years at diagnosis with a pre-chiasmatic intra-orbital glioma, in whom a resection from the bulb to the chiasm was performed. RESULTS: 11 patients were included. 4 had NF1. Mean age at surgery was 7.0 years. Interval between diagnosis and surgery was 1-74 (median 10) months. Two had prior chemotherapy, one radiation, one both, one prior intraorbital surgery. In all 5 progression occurred. Indications for surgery were exophthalmos, pain, tumor progression or a combination. 8 patients (Group A) underwent an extradural trans-orbital-roof approach to resect intra-orbital tumour including the optic canal part plus intradural pre-chiasmatic resection. In 3 patients (Group B) a combined supra-orbital mini-craniotomy plus orbital frame osteotomy was used for intraorbital tumour-resection, excluding the optic canal part, plus intradural pre-chiasmatic resection. GTR was achieved in 7/8 of Group A and none had a recurrence (mean-FU 42 month). One residual behind the bulbus showed progression, treated by chemotherapy. All residuals in Group B were remnants of the optic nerve within optic canal remained stable (mean FU 11.8 months). No patient had a chiasmatic functional affection or permanent oculomotor deficits. Two after prior radiotherapy developed slight enophthalmos. CONCLUSION: In these selected patients surgical resection from bulb to chiasm (± removal of optic canal tumor) is safe without long-term sequela and with excellent cosmetic result. Surgery removes immediately exophthalmos and provides an effective long-term tumor control. It should be considered therapy of choice.