Biomedical subjects

Stephen F Conley

Publications and source records attributed to Stephen F Conley.

12 recordsLinked to original sources

PubMed2006

Botulinum toxin as treatment for a unique case of subcutaneous emphysema.

A developmentally delayed 11-year-old male developed extensive spontaneous subcutaneous emphysema 6 weeks after a laryngotracheal separation. Computed tomography demonstrated a small amount of air at the distal end of the laryngeal stump and significant esophageal air. Aerophagia was diagnosed with air presumed diverted through the laryngeal stump due to cricopharyngeal hypertension. Cricopharyngeal botulinum toxin injection was coordinated with a minimal neck dissection to drain the subcutaneous emphysema. The subcutaneous emphysema gradually improved and the patient was discharged home. His subcutaneous emphysema has not returned after four total botulinum toxin injections every 3 months.

Aerophagy↗

PubMed2006

Bilateral epistaxis in children: efficacy of bilateral septal cauterization with silver nitrate.

OBJECTIVE: To evaluate the rate of control of epistaxis in patients with bilateral complaints using silver nitrate and to assess short-term complications from this therapy. STUDY DESIGN AND SETTING: Retrospective chart review with prospective incident case identification in a tertiary referral pediatric otolaryngology practice. SUBJECTS AND INTERVENTIONS: Forty-six consecutive patients presenting to a single pediatric otolaryngologist with complaints of bilateral epistaxis received bilateral silver nitrate cauterization to the anterior septum and were seen in follow-up 2 months later to assess for control of epistaxis and complications from this therapy. RESULTS: Ninety-three percent (43/46) of patients followed-up at 2 months after therapy. About 93% (40/43) had total or near-total resolution of their epistaxis. Five percent (2/43) of patients returned to the clinic after initial successful control for a second cauterization during the period of study. No significant complications of infection, perforation, long-term crusting, tattooing or mucocutaneous or allergic reactions were identified. CONCLUSIONS: Bilateral epistaxis can be treated at a single visit with bilateral silver nitrate therapy in children. This treatment is highly effective, well-tolerated, and appears to have a low rate of complications.

Adolescent↗

PubMed2006

Four mutations in Epidermodysplasia verruciformis 1 (EVER1) gene are not contributors to susceptibility in RRP.

OBJECTIVE: Epidermodysplasia verruciformis is a skin disease characterized by abnormal susceptibility to human papilloma viruses. Recently four mutations in the Epidermodysplasia verruciformis 1 gene (EVER1, also known as TMC6) have been associated with the disease. Because of the phenotypic similarity between Epidermodysplasia verruciformis and recurrent respiratory papillomatosis, we decided to investigate whether any of these mutations accounts for the susceptibility to human papilloma viruses in subjects with recurrent respiratory papillomatosis (RRP). METHODS: Allele-specific PCR and restriction fragment length polymorphisms (RFLPs) were employed for genotyping a cohort of 101 patients with recurrent respiratory papillomatosis. RESULTS: None of these four mutations were found in the studied subjects. CONCLUSION: The absence of these mutations in RRP patients might indicate that EVER 1 alleles are not associated with susceptibility to RRP, or that other, as yet unidentified, mutations in the Epidermodysplasia verruciformis 1 gene, might account for the susceptibility to RRP.

Female↗

PubMed2006

Ofloxacin otic drops vs neomycin-polymyxin B otic drops as prophylaxis against early postoperative tympanostomy tube otorrhea.

OBJECTIVES: To evaluate the incidence of tympanostomy tube (TT) sequelae, tube otorrhea, and tube obstruction immediately postoperatively in patients receiving TT for otitis media and to compare patients receiving postoperative otic drops with controls. DESIGN: Blinded randomized control trial. SETTING: A tertiary pediatric otolaryngology practice. SUBJECTS: The study population comprised 306 patients undergoing TT placement. INTERVENTIONS: The 306 patients were enrolled into the following 3 groups: (1) those receiving no postoperative otic drop prophylaxis (control group), (2) those receiving ofloxacin otic drops (FLOX group), and (3) those receiving neomycin sulfate-polymyxin B sulfate-hydrocortisone otic drops (COS group). RESULTS: Overall otorrhea rates postoperatively were 14.9% for the control group, 8.1% for the FLOX group, and 5.5% for the COS group. When controlling for disease severity, the rate of otorrhea was significantly higher for the control group than for both the FLOX (P = .04) and COS (P = .01) groups. Nonpatent, plugged, tube rates were added to otorrhea rates for a TT failure analysis postoperatively. The control group demonstrated a significantly greater failure rate (29.9%) than both the FLOX (12.1%) and COS (7.7%) groups. The only differences between the patients in the 2 groups receiving drops were that ofloxacin was more well liked by patients (P = .04) and caused less pain (P = .004). CONCLUSIONS: Nonpatency and otorrhea are the most frequent sequelae immediately following TT placement. Few studies have compared different treatment regimens in a randomized controlled trial. These results demonstrate that otic drops clearly provide benefit postoperatively in preventing TT plugging and otorrhea but primarily in patients who have middle ear fluid at the time of TT placement. In addition, consideration of drop choice should be based on patient tolerance and medication safety profiles.

Administration, Topical↗

PubMed2006

Unique challenges of obtaining regulatory approval for a multicenter protocol to study the genetics of RRP and suggested remedies.

OBJECTIVE: Investigations that seek to generalize findings or to understand uncommon diseases must be conducted at multiple centers. This study describes the process of obtaining regulatory approval for a minimal risk genetic study in a multi-center setting as undertaken by the Recurrent Respiratory Papillomatosis (RRP) Task Force. STUDY DESIGN AND SETTING: Sequential cohort of American children's hospitals. A single protocol was submitted to each Institutional Review Board (IRB). RESULTS: Documentation was prepared for 14 IRBs over 2.5 years. The median time between enlistment and approval at the first 8 sites was 15 months. Institutions varied considerably in their requirements and in the issues that were raised. Protocols were submitted sequentially and accumulated experience was used in the preparation of applications to subsequent IRBs. Nevertheless, there was no correlation between the accumulated experience and the number of issues that were raised. CONCLUSION: Despite uniform federal standards, all local IRBs required unique and individualized submissions. For multicenter studies, investigators should seriously consider the establishment of cooperative authorization agreements. On a simpler level, a standardized format for applications needs to be adopted nationwide. EBM RATING: B-3b.

Clinical Protocols↗

PubMed2006

Recurrent respiratory papillomatosis in children: masquerader of common respiratory diseases.

BACKGROUND: Recurrent respiratory papillomatosis in children is an uncommon but potentially life-threatening benign tumor of the respiratory tract with laryngeal predilection. The diagnosis of recurrent respiratory papillomatosis may be challenging unless there is a high index of suspicion and awareness of the variable presentations. METHODS: We reviewed the medical charts of children with recurrent respiratory papillomatosis treated at a tertiary children's hospital. The presentation of recurrent respiratory papillomatosis is illustrated by a series of case reports. We provide a paradigm to assist in the early diagnosis of children with recurrent respiratory papillomatosis. RESULTS: Five patients, aged 2 to 6 years, were erroneously diagnosed with recurrent croup, asthma, laryngeal hemangioma, and tracheomalacia after presenting with variable degrees of chronic dyspnea, cough, stridor, dysphonia, weak cry, and syncope. Once the diagnosis of recurrent respiratory papillomatosis was made, recurring surgical ablation of papillomata was initiated. CONCLUSIONS: Any child presenting with a voice disturbance with or without stridor is recommended to have diagnostic flexible fiber-optic laryngoscopy. Recurrent respiratory papillomatosis should be considered in children when other common pediatric airway diseases either do not follow the natural history or do not respond to treatment of the common disorder.

Child↗

PubMed2004

Pediatric myasthenia gravis and velopharyngeal incompetence.

OBJECTIVE: To determine the clinical course of velopharyngeal incompetence in children with myasthenia gravis (MG). METHODS: A 30-year retrospective study was performed using the medical records of 538 children who presented with velopharyngeal inadequacy (VPI) to a tertiary care academic pediatric center. Children with velopharyngeal incompetence due to myasthenia gravis were identified and their clinical courses were reviewed. RESULTS: Four children were identified with velopharyngeal incompetence associated with myasthenia gravis. All four children required intervention for improvement of speech intelligibility. A speech prosthesis was the uniform intervention. CONCLUSION: Neonatal myasthenia gravis patients should be followed long-term as symptoms may recur as speech impairment. In addition, a high index of suspicion for this entity is required for early diagnosis due to the highly variable presentation and clinical course.

Adolescent↗

PubMed2004

Foreign body granuloma of the external auditory canal.

External auditory canal polyps are most commonly inflammatory in nature but may also manifest more severe disease. Prolonged conservative therapy may delay the correct diagnosis and appropriate intervention. A case is presented of a child with chronic otorrhea treated for 4 months with topical drops and antibiotics. On referral, a large external auditory canal polyp was confirmed to represent a foreign body granuloma covering a large electrical cap, with erosion approaching the facial nerve. External auditory canal polyps that fail to respond promptly to conservative medical therapy warrant a computed tomography scan and surgical exploration with biopsy.

Child↗

PubMed2003

Office-based lower airway endoscopy in pediatric patients.

BACKGROUND: Office-based evaluation of the lower airway in adults with only topical anesthetics has been well documented. This study was performed to assess the feasibility of performing office-based lower airway endoscopy in a pediatric population. DESIGN: One hundred five consecutive pediatric patients requiring flexible laryngoscopy were studied. All received only a topical anesthetic-decongestant applied nasally. After flexible laryngoscopy, the endoscope was passed below the vocal folds to visualize the subglottis, trachea, and carina. All evaluations were videotaped for later review. SETTING: Academic pediatric otolaryngology practice. MAIN OUTCOME MEASURES: All 105 patients were studied for complications and agreement between office endoscopy and operative endoscopy when necessary (performed in 20 patients). A subset of 24 consecutive patients were studied for ease of performing the lower airway evaluation, rated on a 3-point scale: 1, unable to perform; 2, performed with some difficulty; and 3, performed without difficulty. The ability to view the subglottis, trachea, and carina were also rated on a 3-point scale. RESULTS: There were no complications for any of the procedures. Office endoscopy correlated with operative endoscopy in all cases. In the subset of 24 patients, the mean score for ease of endoscopy was 2.83. The mean scores for visualizing the lower airway were 2.91 for the subglottis, 2.80 for the trachea, and 2.24 for the carina. CONCLUSION: With the use of only topical anesthesia, flexible endoscopy of the lower airway in children can be performed in the office setting and can be used effectively to evaluate abnormalities of the lower airway.

Chi-Square Distribution↗

PubMed2003

Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair.

Plastic bronchitis is a rare disorder characterized by the formation of branching mucoid bronchial casts. Several pathophysiologic conditions are associated with development of these intrabronchial casts, including congenital heart defects. The management of plastic bronchitis presents an unusual and interesting bronchoscopic challenge. We describe a patient who underwent a Fontan procedure for correction of a congenital heart defect and subsequently developed respiratory distress secondary to plastic bronchitis on two occasions. In both cases, endoscopic intervention was required to remove these casts. A review of the literature, including the proposed etiologies, diagnosis, and current medical and surgical management, is also undertaken.

Bronchitis↗

PubMed2003

Anterior lingual thyroglossal cyst: antenatal diagnosis, management, and long-term outcome.

OBJECTIVE: To describe the long-term outcome of a large anterior lingual thyroglossal duct cyst, diagnosed antenatally by ultrasound that completely obstructed the oral cavity and had almost completely replaced the anterior two-thirds lingual musculature. METHODS: Longitudinal observation. RESULTS: Anticipated airway obstruction at birth did not occur. Complete cyst removal occurred at day 2 of life with a rim of lingual muscle observed present only on the left side. Complete and safe oral feeding was achieved by day 11 of life. Progressive muscle mass development and function with mild asymmetry was observed during sequential assessments. Vigorous speech therapy was started at age 7 months achieved 80-100% intelligible speech with minimal misarticulations at age 33 months. CONCLUSION: This case demonstrates the remarkable compensatory ability of the tongue to achieve almost normal function with minimal anterior musculature that is critical to deglutition and articulation. Early speech therapy appears key to improving functional outcomes of speech. A strong central nervous system basis for suckle development is suggested by the rapid development of a safe and effective suckle and swallow soon after cyst resection and in spite of in utero tongue fixation.

Humans↗

PubMed2002

Pyriform sinus malformations: a cadaveric representation.

BACKGROUND/PURPOSE: The most important aspects in management of pyriform sinus malformations are awareness of the diagnosis, familiarity with the clinical manifestations, and complete surgical excision of the entire tract. Pyriform sinus anomalies are the least common branchial apparatus malformations and present anatomically as sinus tracts with or without cystic dilatation. The clinical presentations can include lateral neck mass, thyroid abscess, suppurative thyroiditis, retropharyngeal abscess, neonatal airway obstruction, and even carcinoma. Recurrent symptoms after surgery suggest incomplete identification and excision of the tract. METHODS: Cadaveric dissections were performed to show both the proposed embryologic course and clinical manifestations of third and fourth branchial apparatus pyriform sinus anomalies. RESULTS: Illustrations and digital camera images of the cadaveric models are presented to explain the course of pyriform sinus fistula tracts. CONCLUSIONS: The authors discuss 3 case presentations of pyriform sinus anomalies with emphasis on their proposed embryologic origin and anatomic basis for surgical management. Surgical excision is the mainstay of therapy. Understanding the embryologic basis for pyriform sinus malformations aids in recognition of the diagnosis despite the myriad of clinical presentations. Laryngoscopy with sinus cannulation facilitates removal of the entire sinus tract with preservation of the recurrent and superior laryngeal nerves.

Abscess↗