Quality Improvement of Pediatric Airway Emergency Carts: Standardization, Streamlining, and Simulation.

Objective Pediatric airway emergencies are amongst the most tenuous scenarios faced by on-call providers, requiring quick access to the appropriate equipment and a timely response. In the present study, we report on the testing and improvement of pediatric airway carts at our institution. The primary objective was to optimize our pediatric airway emergency carts to improve response times. Secondarily, we aimed to implement a training scenario to improve providers' familiarity and confidence in attaining and assembling equipment. Methods Surveys of airway cart configuration at our hospital and others were used to identify differences. Volunteer otolaryngology physicians were tasked with responding to a mock scenario using an existing cart or one modified based on the survey. Outcomes included (1) time to arrival of the provider with the appropriate equipment, (2) time from arrival to complete assembly of equipment, and (3) time for re-assembly of the equipment. Results The survey revealed differences in cart equipment and location. The inclusion of a flexible bronchoscope and a video tower, as well as the placement of the carts directly within the ICU, resulted in improved time to arrival by an average of 181 seconds, and improved equipment assembly time by an average of 85 seconds. Discussion Standardization of pediatric airway equipment on the cart and location near critically ill patients improved response efficiency. Simulation led to improved confidence and reduced reaction time among providers at all levels of experience. Conclusion The present study provides an example for the optimization of airway carts, which can be adapted by healthcare systems to their local milieu.

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.

Congenital laryngeal webs: surgical course and outcomes.

OBJECTIVES: We compare the success of different surgical options in the treatment of laryngeal webs. METHODS: We performed a retrospective study spanning the years 1980 to 2005. RESULTS: Eighteen patients were identified. The average age at diagnosis was 6 months (range, 1 day to 2.5 years). The presenting symptoms included weak cry, stridor, airway obstruction, and difficulty breathing. Associated cardiac defects consistent with the diagnosis of 22q-syndrome were present in 7 patients. Webs were classified as grade I (5 patients), grade II (2 patients), grade III (10 patients), or grade IV (1 patient) according to the Cohen classification. In 5 patients, only endoscopic lysis was required. The remaining 13 patients underwent open procedures; 9 patients in this group required tracheotomy. An average of 1.3 open airway procedures was necessary to achieve a decannulation rate of 89%. After operation, 34% of patients had residual webbing and 20% had a weak or aphonic voice. CONCLUSIONS: Management of laryngeal webs is dependent on the severity of airway obstruction. Grade I and II webs can be treated endoscopically; more severe laryngeal webs usually require tracheotomy and open airway reconstruction.

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.

Inherited plasminogen deficiency presenting as ligneous vaginitis: a case report with molecular correlation and review of the literature.

Type 1 plasminogen deficiency is an inherited and potentially life-threatening systemic disease in which patients develop pseudomembranous lesions of mucosal surfaces exposed to minor trauma. It is most commonly clinically encountered as ligneous conjunctivitis. We report the case of a 39-year-old woman with extensive involvement of the female genital tract. Microscopically, the vagina, cervix, endometrium, ovaries, and parametrial tissues showed innumerable deposits of paucicellular hyaline material with adjacent inflammation. Histochemical, immunofluorescent, and electron microscopic analyses revealed the amorphous material to be fibrin and collagen. In the plasma, functional plasminogen and plasminogen antigen levels were markedly decreased. Sequencing showed the patient to be a compound heterozygote for a missense and nonsense mutation in the plasminogen gene. Histologically, deposits in ligneous vaginitis are easily confused with amyloid or fibrinous debris. Recently, replacement therapy with plasminogen has been shown to significantly improve systemic symptoms, making ligneous mucositis a serious but treatable condition.

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.

Polyoma virus infection of renal allografts: relationships of the distribution of viral infection, tubulointerstitial inflammation, and fibrosis suggesting viral interstitial nephritis in untreated disease.

Whether polyoma virus (PV) infection of renal allografts induces an antiviral or antigraft immune reaction is unclear. By examination of the relationships of tubular PV to graft inflammation and scarring, this study sought histological evidence of viral interstitial nephritis in allograft biopsies with untreated PV infection and compared the inflammatory indices to controls with acute rejection (AR). Morphological features including viral cytopathic changes (VCCs) and modified Banff 97 histological indices were evaluated in sections of 28 diagnostic biopsies from a group of patients receiving prednisone, tacrolimus, and mycophenolate mofetil at constant dosage before biopsy. Two-micrometer paraffin sections were stained for PV large T antigen (TAg) and for C4d, by immunohistochemistry. Tubular profiles with 1 or more nuclei expressing TAg per x200 field were scored using an interval scale (0-10; none to 91-100%) by 2 observers. Controls with AR (n = 38, TAg negative) were matched for time after transplantation and severity of Banff 97 interstitial inflammation (i) and tubulitis (t) scores. Median t scores for tubules with VCC or TAg or both exceeded scores for tubules without VCC or TAg (3 versus 0, P = .001). Tubular TAg score correlated with i score (r = 0.58, P < .01) and sum ct + ci score (r = 0.61, P < .001). Atrophic tubules in scars had persistent VCC and/or TAg. Interstitial plasma cells (75% versus 21%) and neutrophils (32% versus 0%) were more frequent, and interstitial fibrosis was more severe (ci >1 in 54% versus 21%) in polyoma virus nephropathy (PVN) than in the group with AR (P < .01). Intimal arteritis (0% versus 35.7%), peritubular capillary C4d (0% versus 47.4%), and interstitial hemorrhage (4% versus 37%) were almost exclusively found in AR (P < .01). Tubular inflammation in untreated PVN involves infected tubular profiles with greater severity than those without evidence of infection. The extent of tubular PV infection is proportional to interstitial inflammation and scarring. Tubulointerstitial inflammation in PV infection has significant qualitative differences from AR. Observations in these examples of untreated PVN suggest that the allograft inflammatory reaction may exhibit features of viral tubulointerstitial nephritis distinct from AR.

Implications of immunohistochemical detection of C4d along peritubular capillaries in late acute renal allograft rejection.

BACKGROUND: Immunohistochemical detection of the C4d complement product along peritubular capillaries (PC) may indicate humoral rejection of renal allografts. We examined the frequency of PC C4d expression in renal-allograft biopsies with acute rejection (AR) arising more than 6 months after transplantation and the impact of this finding. METHODS: C4d was detected by immunoperoxidase in 2-micron paraffin sections of consecutive biopsies obtained over a 3-year period. The extent was classified as diffuse (> or =50% PC C4d+), focal (<50% C4d+), and negative (C4d-). Clinical data were obtained by retrospective chart review. Fifty-five AR episodes with Banff 97 types 1A (n = 13), 1B (n = 26), 2A (n = 11), 2B (n = 3), and 3 (n = 2) met inclusion criteria. RESULTS: PC C4d expression was diffuse in 23 (42%), focal in 9 (16%), and negative in 23 (42%) biopsies. AR episodes with focal and diffuse C4d expression had higher proportionate elevation of serum creatinine at biopsy and 4 weeks after diagnosis (P< or =0.05). Biopsies with diffuse PC C4d had interstitial hemorrhage (56.5%) and plasmacytic infiltrates (52%) more frequently than C4d- biopsies (22% and 16%), P = 0.02, but had no other distinctive histologic features. Graft loss was greater in diffuse (65%) compared with focal C4d+ (33%) and C4d- (33%) groups 1 year after diagnosis, P = 0.03. Other clinical and pathologic parameters did not differ significantly, including treatment received for AR. CONCLUSION: Evidence of acute cellular with occult humoral rejection is identified in more than 40% of late AR episodes. Late acute humoral rejection may be associated with interstitial hemorrhage and plasma cells and contributes significantly to graft loss.

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.

Endoscopic sinus surgery in cystic fibrosis: effects on pulmonary function and ideal body weight.

There is some question as to whether surgical treatment of sinus disease in patients with cystic fibrosis improves pulmonary function or helps patients achieve their ideal body weight. To investigate this matter, we retrospectively studied 17 children with cystic fibrosis who had undergone endoscopic sinus surgery at the University of Kentucky College of Medicine over a 15-year period. All patients had advanced sinus disease at the time of surgery. Although there was an overall increase in mean pulmonary function values, which peaked 1 month postoperatively, no statistically significant improvements were maintained at 6 and 12 months. Nor was there any significant change in these patients' percentage of ideal body weight.

Platelets and capillary injury in acute humoral rejection of renal allografts.

Platelet accumulation in glomerular capillaries (GC) and peritubular capillaries (PC) has pathogenetic importance in antibody-mediated hyperacute renal allograft rejection. CD61 is expressed constitutively by platelets, by platelet microparticles arising from platelet activation, and is readily detectable by immunohistochemistry. This study examined the immunohistochemical localization of CD61 in acute humoral rejection (AHR) of renal allografts to explore the relationship of platelet accumulation to antibody-mediated rejection. Two observers graded the extent of CD61 staining in PC and GC from 0 (none) to 2+ (>50%) in 15 renal allograft biopsy specimens with AHR and compared these with tissues from allografts with acute cellular rejection (ACR) (n = 23); acute calcineurin inhibitor toxicity (ACIT) (n = 21) with thrombotic microangiopathy (TMA) (n = 11) and tubular toxicity only (n = 10); acute tubular necrosis (ATN) (n = 16); acute renal vein thrombosis (RVT) (n = 4); and histologically unremarkable native kidneys (n = 26). Selected tissues were examined by electron microscopy and stained for CD34 by immunohistochemistry. Histologically unremarkable native kidney tissues had CD61 only in scattered small lumenal granules in GC and PC. Mural and occlusive lumenal CD61 deposits (>0.5+) were observed in 13 of 13 (100%) allograft tissues with GC thrombi due to AHR (1) and ACIT TMA (9) and RVT (3). Twenty-seven of 66 allografts (40.9%) without glomerular thrombi had >0.5+ GC CD61 in AHR (60%), ACR (26%), tubular ACIT (60%), and ATN (44%). More than trace (>0.5+) PC mural and lumenal CD61 deposits were seen only in AHR (53.3%) and ACR (30%). PC CD61 correlated with interstitial hemorrhage (r = 0.64), neutrophilic capillaritis (r = 0.47), and interstitial inflammation (r = 0.47) (P <0.001 for each). PC CD61 was observed in 11 of 11 foci of necrosis due to AHR, RVT, and ischemia. In AHR, capillaries with CD61 deposits had few platelets, numerous microvesicles and membrane fragments, severe endothelial injury seen on electron microscopy, and reduced capillary CD34 expression. CD61 detection by immunohistochemistry revealed products of capillary platelet activation in allograft biopsy specimens without light microscopic thrombi. Observations in this study suggest that intracapillary platelet activation occurs in response to graft capillary injury from many causes and may not be specific for antibody-mediated rejection.

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.