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Nose and Sinuses

Topic: The Genetic, Hormonal, and Molecular Determinants of Juvenile Nasopharyngeal Angiofibroma:  A Systematic Review
Principal Investigator: Dr. Michael Cunningham
Summary: We present a systematic review of the molecular and somatic genetic determinants of JNA, including a qualitative and, where appropriate, quantitative meta-analysis of pooled small sample sized studies. We highlight areas of particular clinical relevance, including potential points of therapeutic intervention with respect to tumorigenesis and vasculogenesis. Additionally, we explore areas of controversy in pathogenesis, including the histological origin of JNA and the relationship of JNA to familial adenomatous polyposis (FAP) as related to the APC/B-catenin pathway. Lastly, we examine the potential clinical implications of a deeper genomic understanding of JNA, including the role for hormonal therapy and the capability to include genetic or molecular markers in staging criteria.

Topic: Endoscopic Radiofrequency Ablation Assisted Resection of Juvenile Nasopharyngeal Angiofibroma: Comparison with Traditional Endoscopic Technique
Principal Investigator: Dr. Michael Cunningham and Dr. Eelam Adil
Summary: Given the vascular nature of juvenile nasopharyngeal angiofibroma (JNA), the ability to achieve hemostasis and tissue ablation with a single device is theoretically beneficial during tumor resection. The purpose of this study was to assess the feasibility of radiofrequency ablation assisted (RFA) resection of JNA.  We also aimed to compare endoscopic RFA resection of JNAs to age and stage matched controls who underwent endoscopic resection using traditional endoscopic (TE) instrumentation technique. 

No statistical advantage of using a radiofrequency ablation device compared to traditional endoscopic instrumentation was identified with respect to operative time, intraoperative blood loss, transfusion rate, hospital length of stay, or recurrence rate. RFA instrumentation, however, does offer the surgeon an alternative option for tumor debulking during resection of these highly vascularized tumors.

Topic: Sinus CT Imaging in Pediatric Cystic Fibrosis: Added Value?
Principal Investigator: Dr. Michael Cunningham and Dr. Eelam Adil
Summary: In the pediatric CF population, the need for frequent imaging increases the potential risk for significant cumulative lifetime doses of ionizing radiation given the multisystem involvement of this disease.  The aims of this study were to 1) review the overall prevalence of CT imaging in our pediatric CF population with a focus on paranasal sinus imaging indications and outcomes 2) evaluate changes in Lund-Mackay (LM) scores over time and 3) estimate radiation exposure secondary to sinus CT imaging. 

All patients with CF have relatively high LM scores regardless of clinical context.  Routine imaging for disease evaluation is probably not necessary and represents an area where radiation exposure can be decreased in these patients.  Clinicians should limit paranasal sinus imaging to the preoperative context or the evaluation of suspected sinusitis complications. In this context, otolaryngology consultation should be considered prior to imaging.  In addition, LM scores for individual patients do not change significantly with time or intervention.  If there has been no prior surgical anatomical disruption and the child is of an age in which significant developmental changes between imaging studies is unlikely, then repeat paranasal sinus imaging can also be avoided.

Topic: Electron Microscopy of Nasal and Tracheobronchial Biopsies in the Diagnosis of Primary Ciliary Dyskinesia: A Systematic Review and Meta-analysis
Principal Investigators: Dr. Michael Cunningham and Dr. Eelam Adil
Summary: In patients with suspected PCD, the most widely accepted methods for confirming the diagnosis involve biopsy of ciliated respiratory mucosa followed by ultrastructural electron microscopic (EM) evaluation, high speed videomicroscopy, or genetic testing for known mutations. Nasal and tracheobronchial biopsy sites have both been described with no consensus as to the most effective biopsy site or method.  The purpose of this systematic review and meta-analysis is to analyze the literature regarding the efficacy of alternative biopsy sites and methods for the diagnosis of PCD.
There is currently no single definitive diagnostic test for patients with PCD.

Ultrastructural EM evaluation of ciliary epithelium provides valuable information that is complementary to clinical history and alternative diagnostic methods in individuals suspected to have PCD.  Nasal ciliary biopsy should be considered prior to tracheobronchial biopsy in patients who can tolerate an office-based procedure.  Scrape biopsy may be superior to brush and forceps techniques, but a more definitive study taking into account the training and experience of the physician performing the biopsy is necessary to confirm this finding.

Topic: Orbital Complications of Acute Pediatric Sinusitis
Principal Investigator: Dr. Eelam Adil
Summary: While acute sinusitis is a relatively common condition in children, it has the potential to spread to the adjacent orbit and brain. The majority of complications involve the orbit, and Oxford and McClay report that a remarkable 91% of their pediatric patients with complicated acute sinusitis had orbital involvement (2005). Chandler's classification of orbital complications include I. periorbital cellulitis, II. orbital cellulitis, III. subperiosteal abscess, IV. orbital abscess, V. cavernous sinus thrombosis. With this study, we hope to take a deeper look at these complications and their course of treatment. We will review all pediatric patients with acute sinusitis seen at Boston Children's Hospital over the past 10 years, and we will categorize the patients into 4 groups: 1) patients admitted with acute sinusitis who have no orbital or intracranial complications; 2) patients admitted with acute sinusitis who have an orbital complication who are treated medically and eventually discharged; 3) patients admitted with acute sinusitis who are initially treated medically with IV antibiotics but fail to improve and eventually require surgery; 4) patients with an orbital complication who undergo immediate surgical intervention. We will compare groups based on age, gender, ethnicity, past medical and surgical history, prior sinusitis and treatment history, duration of symptoms prior to presentation, extent of sinusitis and infection, oral antibiotic use, type of orbital complication, presence of fever and fever duration, laboratory studies (e.g. white blood cell count), IV antibiotic therapy, ophthalmology findings, nasal endoscopic findings, imaging findings, microbiology and sensitivities, permanent complications, length of hospitalization, surgical intervention, readmission, and duration of antibiotic therapy. We are hopeful that this retrospective case control study will better inform treatment decisions and improve our understanding of how orbital complications impact the clinical outcome of acute sinusitis.

Topic: Nasal septal perforation in children: presentation, etiology, and management
Principal Investigator: Dr. Eelam Adil
Summary: Pediatric nasal septal perforation is a rare clinical condition. Symptoms of septal perforation include nasal whistling, recurrent epistaxis, crusting, and discomfort. While there is a significant amount of literature on adult septal perforation, the literature regarding pediatric septal perforation is limited to a few small series, each with 3 or fewer patients. We propose a comprehensive retrospective review of our clinical experience with the diagnosis and management of septal perforation at Boston Children's Hospital. We are interested in studying patient demographics, symptomology, etiology, clinical presentation, treatment, and surgical repair of this condition. We believe this will improve our understanding of not only the etiology of pediatric nasal septal perforation, but also the most effective management techniques.

Topic: Endoscopic sinus surgery in pediatric cystic fibrosis patients
Principal Investigator: Dr. Eelam Adil, Dr. Michael Cunningham
Summary: The purpose of this study is to describe and document the Boston Children’s Hospital experience with endoscopic sinus surgery in pediatric patients with cystic fibrosis. Specifically, we want to look at whether endoscopic sinus surgery affects pulmonary function for these patients. We are analyzing the data from pulmonary function tests of cystic fibrosis patients both prior to surgery and for up to a year after surgery. By looking at pre- and post-operative data, we hope to determine whether endoscopic sinus surgery is beneficial from a pulmonary perspective. 

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