Russell-Silver syndrome with cleft palate: a case report

Abstract

Background

Russell-Silver syndrome (RSS) is characterized by intrauterine growth retardation, short stature without postnatal catch-up growth, and an inverted triangular face with relative macrocephaly. There have been few case reports of RSS with cleft palate, in which perioperative problems such as difficult intubation due to trismus and impossibility to wear a mouth gag due to growth failure of the mandible were described. The case of a female RSS patient with cleft palate who underwent palatoplasty is reported.

Case presentation

Although her weight was particularly low (5920 g), palatoplasty was performed under general anesthesia at 3 years and 6 months of age. Despite limited mouth opening, intubation was relatively easy. Although her mandibular alveolar width was narrow, a Dingman mouth gag could be tightly fastened around her mouth. Postoperatively, the patient was transferred to the intensive care unit without extubation due to pharyngeal edema. On the following day, since the pharyngeal edema had improved, the endotracheal tube was extubated, and her respiratory status was subsequently stable.

Conclusions

In RSS patients with cleft palate, there have been a few reports of pharyngeal edema. Thus, the risk of pharyngeal edema must be considered in such patients.

https://ift.tt/2FXesXL

Styloid-stylohyoid syndrome: a rare cause of cranio-facial pain—a retrospective case series of 12 patients

Abstract

Purpose

Elongated styloid process results in severe cranio-facial/cervico-facial pain. The purpose of this study is to determine the efficacy of treatment outcomes using transcervical approach in the management of styloid-stylohyoid syndrome/styloid syndrome.

Study design

This is a retrospective cohort study. Subjects were enrolled from out-patient clinics reporting between the periods Jan 2016–Jan 2018.

Methods & methods

Twelve patients diagnosed with styloid syndrome based on history, thorough clinical workup, and assessment were included in the study. The primary outcomes—improvement in pain and regression of chief complaints following surgery were assessed. Elongated styloid was resected using transcervical approach under general anesthesia.

Results

The intra-operative lengths of the styloid process varied from 40 to 43 mm. No post-operative complications were encountered and the symptoms regressed completely after surgery in all the patients at follow-up of 6 months.

Conclusions

Transcervical styloidectomy is an effective treatment for exposure and resection of the styloid process with minimal complications in patients with stylo-stylohyoid syndrome.

https://ift.tt/2E5m6xy

Mesenchymal stem cells participate in oral mucosa carcinogenesis by regulating T cell proliferation

Publication date: Available online 4 December 2018

Source: Clinical Immunology

Author(s): Yichen Chen, Xi Wang, Juan Fang, Jingjing Song, Da Ma, Liqun Luo, Bailin He, Juan Xia, Vivian Wai Yan Lui, Bin Cheng, Zhi Wang

Abstract

Recent evidences suggested that Mesenchymal stem cells (MSCs) may be involved in tumor formation by modulating of the tumor microenvironment, but it is still unclear the potential of MSCs in the malignant transformation of oral mucosa. Using a chemically-induced oral carcinogenesis model by 4-nitroquinoline-1-oxide (4NQO), we generated precancerous lesions and cancerous lesions in the oral cavity of rats. Flow cytometric analysis on lesions derived single cell suspension revealed an increase in the proportion of MSCs and a decreased proportion of T cell during oral mucosa malignancy. Moreover, MSCs showed increased immunosuppression capacity on T cell proliferation during mucosa malignancy. At last, we demonstrated that higher frequency of lesions resident MSCs was correlated with more Ki67 expression in the lesion, which indicated higher cellular proliferative status in the lesions. Our study demonstrated that MSCs may play an important role in oral mucosa malignant transformation through regulating T cell proliferation.

https://ift.tt/2PhYhUr

Pediatric Nasal Septal Perforation

Publication date: Available online 4 December 2018

Source: International Journal of Pediatric Otorhinolaryngology

Author(s): Jesse J. Jennings, Amber D. Shaffer, Amanda L. Stapleton

Abstract

Objective

1.) Describe demographic and clinical characteristics of pediatric nasal septal perforations (NSP), 2.) Analyze efficacy of treatment modalities in symptom management and resolution of pediatric NSP, 3.) Describe the surgical technique of external rhinoplasty with vascularized nasal septal flaps in the treatment of nasal septal perforations.

Methods

IRB-approved retrospective chart review of pediatric patients ages 0-18 years with nasal septal perforations treated at a tertiary care pediatric otolaryngology practice.

Demographic and clinical characteristics including gender, age, race, and presenting symptoms, and location, size, and etiology of perforation were collected. Outcomes including persistence of perforation and symptoms at 1 year and most recent visit were recorded. A total of 20 patients were included. Statistical analysis used Fisher's t-test for categorical variables and Wilcoxon rank-sum for continuous variables.

Results

Median age was 167.5 months (1.5-221.0). The most common etiology was iatrogenic (40%), followed by button battery (20%). Thirty percent of patients underwent surgical repair. Fifty percent of patients who underwent surgical repair achieved closure of their perforation at most recent follow up.

Conclusion

Pediatric NSP is a challenging issue with limited literature to date. Iatrogenic causes (40%) and button batteries (20%) were the most common etiologies of nasal septal perforation in our study. We introduce an advancement in our center's surgical technique with a case illustration with repair via external rhinoplasty and bilateral vascularized nasal septal flaps. Future larger studies may further elucidate characteristics and treatment modalities associated with successful closure.

https://ift.tt/2Qy2bwV

Personalisierte Medizin in der Allergologie

Zusammenfassung

Hintergrund

Personalisierte Medizin eröffnet neue Optionen der Diagnostik und Behandlung, stellt aber auch erhöhte Anforderungen an den behandelnden Arzt.

Fragestellung

Betrachtet werden Entwicklungen, Potenziale und Risiken der personalisierten Medizin in der Allergologie.

Material und Methode

Es erfolgten ein Überblick, eine Auswertung und Diskussion des aktuellen Stands der Wissenschaft anhand ausgewählter Beispiele in der Allergologie.

Ergebnisse

Allergische Erkrankungen wie die Rhinitis allergica, das atopische Ekzem oder die Anaphylaxie lassen sich in verschiedene klinische Phänotypen einteilen, die pathophysiologisch auf unterschiedlichen immunologischen Endotypen basieren. Diese lassen sich durch eine Vielzahl an Omics-Technologien erfassen und klassifizieren. Die Identifikation endotypenspezifischer Biomarker offeriert vielversprechende Möglichkeiten für eine präzisere Diagnostik, den Einsatz neuer zielgerichteter Therapien oder die Entwicklung optimierter Präventionsstrategien. Eine besondere Herausforderung besteht jedoch in der individualisierten Gewichtung und Relevanzbewertung der erhobenen Parameter.

Schlussfolgerungen

Erkenntnisse der vielschichtigen Omics-Analytik müssen in umfassenden prospektiven Studien auf ihre klinische Bedeutung und Eignung für eine personalisierte Medizin in der Allergologie evaluiert werden.

https://ift.tt/2zH0DHv

Editorial Board

Publication date: September 2018

Source: Operative Techniques in Otolaryngology-Head and Neck Surgery, Volume 29, Issue 3

Author(s):

https://ift.tt/2Qy0v6R

Oncocytic cystadenoma and oncocytic cysts of the parotid gland occurring synchronously with a pleomorphic adenoma

Publication date: Available online 3 December 2018

Source: British Journal of Oral and Maxillofacial Surgery

Author(s): T.J. Cooper, M. Hardie, P. Ricciardo, A. Bobinskas

https://ift.tt/2E01BS2