Choanal Atresia (choanal + atresia)

Distribution by Scientific Domains
Medical Sciences100%

Selected Abstracts

A New Endoscopic Surgical Method for Unilateral Choanal Atresia

THE LARYNGOSCOPE, Issue 2 2001
Bálint Liktor MD
No abstract is available for this article. [source]

Perioperative care of a patient with Beare,Stevenson syndrome

PEDIATRIC ANESTHESIA, Issue 12 2005
SARA UPMEYER DO
Summary Beare,Stevenson syndrome is a craniofacial syndrome consisting of a specific pattern of craniosynostosis resulting in a cloverleaf skull deformity and hydrocephalus, down-slanting palpebral fissures, proptosis, hypertelorism, strabismus, dysmorphic ears, choanal atresia, cleft palate, cutis gyratum, acanthosis nigricans, and abnormal genitalia. Its primary cause has been identified as a single amino acid substitution in fibroblast growth factor receptor 2. Of primary importance to the anesthesiologist are issues related to airway management resulting from midface hypoplasia, choanal atresia, and airway abnormalities (tracheal stenosis). Additional issues affecting airway management include associated cervical spine and foramen magnum abnormalities. The authors present their experience caring for a patient with Beare,Stevenson syndrome and discuss the anesthesia care of these patients. [source]

Treacher Collins syndrome with choanal atresia: one way to handle the airway

PEDIATRIC ANESTHESIA, Issue 8 2004
Eva Nilsson MD DEAA
No abstract is available for this article. [source]

Successful Treatment of Laryngeal Stenosis in Laryngo-Onycho-Cutaneous Syndrome with Topical Mitomycin C

PEDIATRIC DERMATOLOGY, Issue 1 2006
M.R.C.S., P. Seamus Phillips B.M.
It affects the skin, nails, and larynx. Laryngeal involvement may cause lethal airway obstruction, and has in the past proved very difficult to treat. Mitomycin C is an antibiotic that acts as an alkylating agent, inhibiting DNA synthesis. It reduces fibroblast proliferation, and has previously been used to treat choanal atresia and laryngeal stenosis. We report an 18-year-old man with complete transglottic laryngeal stenosis secondary to laryngo-onycho-cutaneous syndrome. An airway was established by dissection with a bougie and sickle knife, and was initially maintained by the upper limb of a Montgomery T-tube. Laryngeal granulation tissue present on removal of the T-tube was treated with topical mitomycin C (2 mg/mL) applied for 4 minutes on two occasions with an interval of 1 month. A year later, the airway remained patent, with no granulation tissue. [source]

Paranasal Sinus Development: A Radiographic Study,

THE LARYNGOSCOPE, Issue 2 2003
Rahul K. Shah MD
Abstract Objective To demonstrate the development of the paranasal sinuses in a pediatric population by computed tomography scans. Study Design Radiology records at a tertiary care institution were reviewed for the computed tomography scans of the face, orbit, or paranasal sinuses in patients aged 0 to 12 years. Methods Computed tomography scans were reviewed by a head and neck radiologist and otolaryngologist for the development of the frontal, maxillary, ethmoid, and sphenoid sinuses. The size of the pneumatized paranasal sinuses was measured in two planes and graded on a scale of 0 to 3. Ossification of the maxillary crest and vomer, obliteration of the foramen cecum, and development of agger nasi cells, Haller cells, and the superior turbinate were studied. Patients with syndromes, nasal stenosis, choanal atresia, or cystic fibrosis were excluded from the study. Results In all, 91 computed tomography scans in 66 patients were studied. Serial development could be followed in 16 patients who underwent repeat scans. Patients were divided into six age cohorts based on their age at the time of the scan: 0 to 3 months (10%), 3 to 12 months (13%), 1 to 3 years (13%), 3 to 5 years (20%), 5 to 8 years (29%), and 8 to 12 years (16%). Ethmoid sinuses were the first to fully develop, followed sequentially by maxillary, sphenoid, and frontal sinuses. Each sinus has a rapid rate of development during specified age cohorts. Conclusion The results will aid the physician when correlating the clinical and radiographic findings of pediatric patients aged 0 to 12 years who are being evaluated for sinus disease and potential surgical intervention. [source]

T-Cell Immunodeficiency in CHARGE syndrome

ACTA PAEDIATRICA, Issue 2 2009
Charu Chopra
Abstract CHARGE syndrome comprises coloboma of the eye, heart defects, choanal atresia, growth and developmental retardation, genitourinary anomalies and ear and hearing defects. The association between CHARGE syndrome and T-cell immunodeficiency is recognized, but has not been reported widely in the literature. We report four patients meeting the diagnostic criteria for CHARGE syndrome, who had moderate or severe T-cell lymphopenia complicated by infections. The patients presented in Leicester, UK, between 2000 and 2007. All patients were negative for 22q11.2 deletions by FISH analysis, but mutations in the CHD7 gene were identified in three patients in whom the analysis was performed. Our cases indicate that patients with CHARGE syndrome may have a spectrum of T-cell immune deficiency, and that this association may be more common than has previously been appreciated. We recommend that all patients diagnosed with CHARGE syndrome should have lymphocyte subsets evaluated as part of their initial investigation. Conclusion: Thymic hypoplasia should be included in the clinical features associated with CHARGE syndrome. All patients with CHARGE syndrome should have lymphocyte subset analysis performed, to exclude T-cell immunodeficiency. [source]