Pediatric endoscopic airway management with posterior cricoid rib grafting.

OBJECTIVES/HYPOTHESIS: To confirm and extend reported successful treatment of posterior glottic stenosis in pediatric patients using endoscopic laser division of the posterior cricoid plate with augmentation using costal cartilage. STUDY DESIGN: A retrospective chart review and case series. METHODS: Medical records were examined to determine the surgical indications, outcomes, and postoperative complications of this procedure. RESULTS: Twelve patients underwent the procedure, six females and six males, with an average age of 7 years (range, 2-26 years). There were 8/12 (67%) patients successfully decannulated after being tracheostomy dependent. There were no consistent anatomic abnormalities or surgical findings predictive of failure to decannulate. Average hospital stay was 3.6 days (range, 2-9 days). There were no deaths or other major complications; one patient had extrusion. CONCLUSIONS: Endoscopic posterior cricoid grafting is a valuable surgical option for patients with posterior glottic stenosis. The procedure is associated with low morbidity and permits decannulation in the majority of patients.

EYA1 mutations associated with the branchio-oto-renal syndrome result in defective otic development in Xenopus laevis.

BACKGROUND INFORMATION: The BOR (branchio-oto-renal) syndrome is a dominant disorder most commonly caused by mutations in the EYA1 (Eyes Absent 1) gene. Symptoms commonly include deafness and renal anomalies. RESULTS: We have used the embryos of the frog Xenopus laevis as an animal model for early ear development to examine the effects of different EYA1 mutations. Four eya1 mRNAs encoding proteins correlated with congenital anomalies in human were injected into early stage embryos. We show that the expression of mutations associated with BOR, even in the presence of normal levels of endogenous eya1 mRNA, leads to morphologically abnormal ear development as measured by overall otic vesicle size, establishment of sensory tissue and otic innervation. The molecular consequences of mutant eya1 expression were assessed by QPCR (quantitative PCR) analysis and in situ hybridization. Embryos expressing mutant eya1 showed altered levels of multiple genes (six1, dach, neuroD, ngnr-1 and nt3) important for normal ear development. CONCLUSIONS: These studies lend support to the hypothesis that dominant-negative effects of EYA1 mutations may have a role in the pathogenesis of BOR.

H-type congenital tracheoesophageal fistula: University Of Iowa experience 1985 to 2005.

OBJECTIVES: We review the diagnostic workup, associated disorders, surgical technique, and postoperative course of patients who underwent repair of H-type tracheoesophageal fistulas. METHODS: We performed a retrospective chart review of patients who received a diagnosis of tracheoesophageal fistula at the University of Iowa. RESULTS: Seven patients with an H-type tracheoesophageal fistula and a single patient with a missed proximal fistula associated with esophageal atresia were identified. Their symptoms included coughing with feeding, recurrent pneumonia, and episodic cyanosis. A delay in diagnosis was seen in 4 patients and ranged from 2.5 months to 5.9 years. In all patients, the diagnosis was made with an esophagogram. The level of the fistulas was between C5 and T3, and all were successfully repaired via a right cervical approach. CONCLUSIONS: A high index of suspicion for an H-type tracheoesophageal fistula should be maintained in the presence of neonatal respiratory symptoms, as the condition can be associated with a delay in diagnosis. Repeat esophagograms and bronchoscopy may be required for diagnosis. In the postoperative period, airway obstruction is a potential risk; however, long-term difficulty with swallowing, respiration, and phonation was not observed.

EYA1 expression in the developing inner ear.

OBJECTIVES: We sought to determine the developmental anatomy and EYA1 protein distribution in the inner ear of Xenopus laevis. METHODS: Xenopus laevis embryos were stained with monoclonal antibodies and imaged with confocal microscopy. RESULTS: At stage 27, the otocyst fully forms, with strong tubulin staining of early sensory cells at its ventromedial aspect. Neuronal ingrowth follows at stage 33/34. At stage 50, the semicircular canals are complete. EYA1 localizes to the anterior aspect of the otocyst from stages 37 to 44. By stage 50, EYA1 distribution is localized primarily to the sensory maculae and the endolymphatic duct of the developing inner ear. CONCLUSIONS: Whole mount confocal imaging of the developing Xenopus inner ear delineates the exact timing of otic development, sensory cell differentiation, and innervation. EYA1 protein expression has a distinct distribution pattern at the anterior aspect of the developing otocyst in stages 41 and 44. Later stages have a more localized pattern, in which EYA1 is detected only in the sensory epithelium and endolymphatic duct. This specific pattern of expression indicates a possible role in the determination of the anterior-posterior orientation of the inner ear, as well as a later role in sensory cell differentiation.

Spastic diplegia and other motor disturbances in infants receiving interferon-alpha.

OBJECTIVE: To determine how frequently the use of -interferon (-IFN) is associated with the development of spastic diplegia. STUDY DESIGN AND METHODS: Meta-analysis of 600 English manuscripts published January 1991 to June 2002 reporting -IFN use in infants/children. We identified 3,113 children 18 years of age or younger and an estimated 3,055 children 12 years of age or younger who received -IFN therapy. Sixty-nine percent were treated for chronic hepatitis and 14% for vascular neoplasms. OUTCOME MEASURE: Neurologic examination to confirm spastic diplegia or a motor developmental disturbance other than spastic diplegia such as hyperactive deep tendon reflexes, gait disturbances, or impaired fine motor control. RESULTS: Including our index case, 11 of 441 children with vascular lesions developed spastic diplegia and an additional 16 of 441 developed a motor developmental disturbance. All of these children were less than 1 year of age at initiation of therapy. Mean age of initiation and duration of -IFN therapy were not significantly different between groups (P >.05); however, motor developmental disturbances improved with cessation of therapy, whereas spastic diplegia did not. No child receiving treatment for chronic hepatitis developed neurologic complications; however, only 49 children were less than 1 year of age at initiation of therapy. CONCLUSION: -IFN should not be used in infants under 1 year of age unless life-threatening conditions do not respond to any other form of treatment. If -IFN must be used, children should have monthly neurologic examinations. If a motor developmental disturbance is detected and -IFN therapy can be discontinued, it should be.

Pediatric airway reconstruction: principles, decision-making, and outcomes at the University of Iowa hospitals and clinics.

Pediatric airway stenosis challenges the treating surgeon, from the initial evaluation and decision-making process to the final postoperative care and follow-up setting. As our approach to these cases evolves, we must critically evaluate our outcomes in reference to our stated goals. We describe our process in treating this population and critically examine our outcome data from 1990 to the present. Emphasis is placed on selection of procedure and postoperative management.

Tracheal spindle cell tumor in a child.

Tracheal tumors in the pediatric population are rare neoplasms that have devastating consequences if not evaluated and diagnosed in a timely fashion. As illustrated by this case, the tumors typically do not become symptomatic until marked airway obstruction occurs, and patients may present with acute respiratory distress. Radiologic advances, including 3-D CT tracheal reconstruction, are invaluable in the diagnosis of and surgical planning for tracheal neoplasms.

Upper airway abnormalities in Canavan disease.

OBJECTIVE: To describe upper airway anatomical abnormalities associated with Canavan disease. METHODS: Retrospective case report. RESULTS: Physical examination and laryngoscopy demonstrated oropharyngeal narrowing, macroglossia, and bronchial asymmetry in a child with Canavan disease. Tracheostomy decreased problems with chronic aspiration and obstructive sleep apnea. CONCLUSIONS: Oropharyngeal obstruction and bronchial asymmetry are previously undescribed upper airway abnormalities found in an individual with Canavan disease. Tracheostomy is an effective method of managing chronic aspiration and obstruction in these patients.

Biofilm formation on tracheostomy tubes.

An increased awareness of biofilms and their mechanisms has led to a better understanding of bacterial infections that occur following the placement of tracheostomy tubes and other implanted devices and prostheses. One aspect of biofilm formation that is still subject to debate is whether the specific material that is used to manufacture a tube has any bearing in the incidence of infection. We conducted a test of four different tube materials--polyvinyl chloride, silicone, stainless steel, and sterling silver--to ascertain how bacterial biofilms form on tracheostomy tubes and to determine if there is a material-dependent difference in biofilm formation. Scanning electron microscopy demonstrated that Pseudomonas aeruginosa and Staphylococcus epidermidis both formed bacterial biofilms on tracheostomy tubes in vitro. We also found that there was no difference in susceptibility to biofilm formation among the four tube materials tested.

Free-radical damage: a possible mechanism of laryngeal aging.

We conducted a study of lipid peroxidation as a marker of age-related free-radical damage in the human larynx--the first study of its kind. A colorimetric assay for malondialdehyde (MDA) and 4-hydroxy-2-nonenal (4-HNE) was performed on extracts taken from thyroarytenoid muscle harvested from fresh cadaveric laryngeal specimens. Levels of MDA and 4-HNE were measured by spectrophotometry. Correlation studies were performed by linear regression analysis. We found that MDA levels in human thyroarytenoid muscle appeared to increase with age while 4-HNE levels showed a slight decrease with age. Our findings are consistent with those of previous studies of other organ systems and indicate that there is a need for further study of free-radical damage and the effects of aging on the human larynx and on voice production.

Complications of intranasal prescription narcotic abuse.

The abuse of drugs via an intranasal route is an increasingly prevalent pattern of behavior. In the past year, a number of patients received care at our institution for complications resulting from the previously unreported phenomenon of intranasal prescription narcotic abuse. This report describes the clinical manifestations of this form of drug abuse in 5 patients. Their symptoms consisted of nasal and/or facial pain, nasal obstruction, and chronic foul-smelling drainage. Common physical findings were nasal septal perforation; erosion of the lateral nasal walls, nasopharynx, and soft palate; and mucopurulent exudate on affected nasal surfaces. In addition, 2 of the 5 patients had invasive fungal rhinosinusitis, which appears to be a complication unique to intranasal narcotic abuse.