Airway obstruction in children with congenital heart disease: assessment by flexible bronchoscopy.

We assessed the spectrum of airway disorders in children with congenital cardiac anomalies, and reviewed our experience in using flexible bronchoscopy for assessment of airway problems in this patient group. The clinical records, flexible bronchoscopic findings, and cardiac imaging results of pediatric cardiac patients who presented with either clinical or radiological signs of airway obstruction between 1992-1999 were reviewed. Flexible bronchoscopic assessment was performed with the patients under sedation and topical anesthesia, using one of two bronchoscopes, i.e., an Olympus BFN20 or Olympus BF3C20. Of a total of 52 patients, 33 had acyanotic cardiovascular lesions, the commonest being left-to-right shunts (61%), while 19 had cyanotic heart lesions, with right ventricular outflow obstruction being the commonest (63%). Twenty-seven patients had undergone either surgical or transcatheter interventions. The median age at bronchoscopic assessment was 6 months (range, 4 days to 6 years). None of the patients developed significant procedural complications. A definitive diagnosis was made in 48 (92%) patients, 8 of whom had abnormalities involving only the upper airways, 35 only the lower airways, and 5 both. Abnormalities of the upper airway included laryngomalacia (n = 6), subglottic stenosis (n = 3), pharyngeal collapse (n = 2), and 1 each of choanal stenosis and supraglottitis. Extrinsic compression was the commonest lower airway abnormality that was found in 27/40 patients (67%), with a predilection for the left main bronchus (18/27, 67%). The structures that caused extrinsic compression included dilated pulmonary arteries with or without left atrial dilation (n = 20), an anomalous aortic or pulmonary arterial course (n = 3), a dilated aorta (n = 1), and a shunt (n = 1), but were not obvious in 2 patients. Intrinsic lower airway abnormalities included bronchomalacia (n = 4), tracheal stenosis (n = 4), and one each of variant bronchial bifurcation and a pouch arising from the tracheal wall. Intraluminal mucus plugging of the lower airways occurred in the remaining 3 patients. Children with congenital heart disease are at risk of airway obstruction both before and after surgery. Flexible bronchoscopy, being safe and effective in diagnosing airway disorders in this patient group, should be considered as the first line of investigation.

MRI for the assessment of the malformed fetus.

Ultrafast MRI sequences have changed the use of MRI for evaluating fetal abnormalities. Currently, the best application is the demonstration of normal fetal brain development and the further definition of suspected brain abnormalities found on ultrasound. MRI differentiates well the various types of fetal ventriculomegaly. Superior posterior fossa visualization allows differentiation of Dandy Walker malformation from a large cisterna magna. Anomalies of the corpus callosum and some disorders of neuronal migration can be seen. MRI is also valuable in the evaluation of fetal giant neck masses for planning delivery of the baby and surgery for life threatening airway obstruction. In the chest, MRI differentiates masses such as diaphragmatic hernia, cystic adenomatoid malformation and sequestration, and aids in planning fetal surgery because MRI directly visualizes the position of the lung, liver and bowel. MRI defines abnormalities of the urinary tract by demonstrating dysplastic pathology in the renal cortex and dilation of the collecting systems. Oligohydramnios does not effect MRI.

Benefit of feeding assessment before pediatric airway reconstruction.

OBJECTIVES/HYPOTHESIS: To determine the utility of preoperative feeding assessments in children undergoing airway reconstruction, identifying parameters that correlate with functional deficits in swallowing and postoperative feeding difficulties. STUDY DESIGN: Prospective, sequential enrollment. METHODS: Two hundred fifty-five patients with structural abnormalities of the upper aerodigestive tract underwent endoscopic swallow studies. Classification of preoperative feeding abilities, specific feeding disorders, and abnormal endoscopic feeding parameters were used to predict the postoperative course of patients undergoing airway reconstruction. The relationships between diagnoses and functional feeding categories and postoperative outcomes and functional feeding categories were appraised by chi2 analysis. RESULTS: The median age of the study population was 2.5 years. Fifty-three percent of the patients were tracheotomy dependent. Only 13% of the patients had diagnoses limited to the airway, with 45% of patients having three or more diagnoses. Worse preoperative feeding abilities were associated with the presence of a tracheotomy, age 2 years or less, and multiple underlying diagnoses. Neurological diagnoses were associated with worse feeding abilities. Preoperative feeding assessments directly altered the course of management of 15% of operative patients, by recommending a delay in the surgical correction, the placement of a gastrostomy tube preoperatively, or a modification in the surgical reconstruction planned for the patient. Postoperative airway protection predictions were 80% accurate. Twelve percent of the predictions involved patients who developed unforeseen complications that required additional treatments or prolonged the hospital stay secondary to difficulties with airway protection. There was no correlation between the preoperative feeding abilities of the patients and their postoperative course after airway reconstruction. CONCLUSION: Transient dysphagia is common after laryngotracheal reconstruction. Preoperative feeding abilities do not correlate with the postoperative airway protection abilities of a patient. Feeding assessments before pediatric airway reconstruction provide a means of identifying patients with poor airway protection mechanisms that may compromise the patient after reconstruction. Findings on swallowing evaluations that predict poor airway protective mechanisms are 1) pooling of secretions in the hypopharynx, 2) poor oral motor skills, allowing premature spillage of material into the hypopharynx where it penetrates the larynx, and 3) residue that persists in the hypopharynx after multiple swallows. The integration of information generated from the preoperative swallowing assessment promotes the selection of operative procedures that are optimal for that patient and highlights specific therapy issues that may need to be addressed in the postoperative management of the patient that may not have been obvious without the study.