Should asymptomatic patients with congenital lung malformations undergo surgery? A decision analysis.

BACKGROUND: Congenital lung malformations (CLMs) are being detected more frequently during pregnancy. There is controversy regarding the optimal treatment for an asymptomatic child with prenatally diagnosed CLMs. Due to the paucity of information from clinical trials, we developed decision analytic models to compare two treatment strategies for such patients-elective surgical resection versus expectant management. METHODS: We built decision analytic models stratified by lesion size. We used data from English language literature identified through PubMed searches along with estimates from expert opinions of surgical colleagues. We analyzed results for two hypothetical asymptomatic 6-month-old children with CLMs; one has a large lesion occupying more than 50% of the involved lobe, while the other has a small lesion occupying less than 50% of the involved lobe. We used quality-adjusted life years (QALYs) to measure effectiveness. RESULTS: For an asymptomatic child with a small or large lesion, expectant management resulted in a small gain of 0.09 or 0.15 QALYs, respectively. Sensitivity analyses showed that surgical resection would be preferred if the probability of remaining asymptomatic was low. CONCLUSIONS: In contrast to current practice, expectant management may be a better alternative for asymptomatic children born with CLMs. More longitudinal studies are required to improve the accuracy of the model.

A clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities.

OBJECTIVES: To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans. MATERIALS AND METHODS: A systematic literature search was conducted for articles describing CT-scan abnormalities of congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE) and bronchogenic cyst (BC). A structured report using objective features of CLA was developed after consensus between a pediatric pulmonologist, radiologist and surgeon. RESULTS: Of 1581 articles identified, 158 remained after title-abstract screening by two independent reviewers. After assessing full-texts, we included 28 retrospective cohort-studies. Air-containing cysts and soft tissue masses are described in both CPAM and BPS while anomalous arterial blood supply is only found in BPS. Perilesional low-attenuation areas, atelectasis and mediastinal shift may be found in all aforementioned abnormalities and can also be seen in CLE as a cause of a hyperinflated lobe. We have developed a structured report, subdivided into five sections: Location & Extent, Airway, Lesion, Vascularization and Surrounding tissue. CONCLUSIONS: CT-imaging findings in CLA are broad and nomenclature is variable. Overlap is seen between and within abnormalities, possibly due to definitions often being based on pathological findings, which is an unsuitable approach for CT imaging. We propose a structured assessment of CLA using objective radiological features and uniform nomenclature to improve reporting.

Thoracoscopic Resection of Congenital Lung Malformation: Looking for the Right Preoperative Assessment.

INTRODUCTION: Consensus on the best postnatal radiological evaluation of congenital lung malformations (CLMs) is still lacking. In recent years, the interest on magnetic resonance imaging (MRI) has grown, but its role is still unknown. AIM: The aim of the study was to identify the best preoperative diagnostic assessment for CLM. MATERIALS AND METHODS: All patients with a prenatal suspicion of CLM between January 2014 and February 2018 were studied. Asymptomatic newborns underwent MRI, during spontaneous sleep without contrast. Patients with a positive MRI were scheduled for computed tomography (CT) within the fourth month of life. Thoracoscopic resection was performed in cases with a pathological CT. MRI, CT, and surgical findings were compared based on dimension, localization, and features of the CLM using the Cohen's kappa test (K). RESULTS: A total of 20 patients were included (10 males). No difference was found in the diameter and site of the lesions always localized in the same side (K = 1) and in the same pulmonary lobe (K = 1). Infants who underwent thoracoscopic resection included: three congenital pulmonary airway malformations (CPAMs), five extralobar and eight intralobar sequestrations (bronchopulmonary sequestrations [BPSs]), three bronchogenic cysts, and one congenital emphysema. The concordance between MRI and CT and between radiological investigations and pathology was satisfactory for the greatest part of the studied variables. MRI showed sensitivity of 100%, specificity of 82%, positive predictive value of 50% and negative predictive value of 100% for CPAM and 77, 100, 100, and 80% for BPS, respectively. CONCLUSION: MRI proved to be a reliable diagnostic investigation for CLM with high sensitivity and specificity. Early MRI in spontaneous sleep without contrast and preoperative contrast CT scan is a valuable preoperatory assessment.

Antenatal assessment and postnatal outcome of fetal echogenic lung lesions: a decade's experience at a tertiary referral hospital.

INTRODUCTION: Fetal echogenic lung lesions (ELL) are the commonest pulmonary pathology diagnosed on antenatal sonography, and include congenital pulmonary airway malformations (CPAMs) and bronchopulmonary sequestrations. This study aimed to evaluate the predictive utility of the CPAM volume ratio (CVR) at presentation in a series of fetuses with ELLs at a tertiary Australian referral hospital. MATERIAL AND METHODS: Retrospective cohort study of all pregnancies with a prenatal diagnosis of an isolated fetal echogenic lung lesion managed at the Royal Women's Hospital, Victoria, Australia, between 2005 and 2015. Data were obtained from electronic ultrasound image databases and medical records. RESULTS: Sixty-five cases were included in the final analysis. The mean gestation at presentation was 22 weeks and 6 d, and the mean CVR was 0.66. Hydrops was evident in four cases at presentation, and did not develop subsequently in any other case. Significant perinatal concerns - fetal/neonatal demise, hydrops, requirement for neonatal intubation/ventilation, or surgery in the first year of life - did not occur with or following a CVR at presentation of <0.45. The survival rate at 1 year was 95%. DISCUSSION: The CVR is a potentially useful tool to assess all fetal ELLs, and not just those presumed to be CPAMs. A CVR at presentation of <0.45 was associated with favourable outcomes.

Pediatric acquired subglottic stenosis: Associated costs and comorbidities of 7,981 hospitalizations.

OBJECTIVES: Numerous risk factors have been characterized for acquired subglottic stenosis (ASGS) in the pediatric population. This analysis explores the comorbidities of hospitalized ASGS patients in the United States and associated costs and length of stay (LOS). METHODS: A retrospective analysis of the Kids' Inpatient Database (KID) from 2009 to 2012 for inpatients ≤ 20 years of age who were diagnosed with ASGS. International Classification of Diseases, Clinical Modification, Version 9 diagnosis codes were used to extract diagnoses of interest from 14, 045, 425 weighted discharges across 4179 hospitals in the United States. An algorithm was created to identify the most common co-diagnoses and subsequently evaluated for total charges and LOS. RESULTS: ASGS was found in 7981 (0.06%) of total discharges. The mean LOS in discharges with ASGS is 13.11 days while the mean total charge in discharges with ASGS is $114,625; these values are significantly greater in discharges with ASGS than discharges without ASGS. Patients with ASGS have greater odds of being co-diagnosed with gastroesophageal reflux, Trisomy 21, other upper airway anomalies and asthma, while they have lower odds of being diagnosed with prematurity and dehydration. Aside from Trisomy 21 and asthma, hospitalizations of ASGS patients with the aforementioned comorbidities incurred a greater LOS and mean total charge. CONCLUSION: Our analysis identifies numerous comorbidities in children with ASGS that are associated with increased resource utilization amongst US hospitalizations. The practicing otolaryngologist should continue to advocate interdisciplinary care and be aware of the need for future controlled studies that investigate the management of such comorbidities.

Resection of pediatric lung malformations: National trends in resource utilization & outcomes.

PURPOSE: We sought to determine factors influencing survival and resource utilization in patients undergoing surgical resection of congenital lung malformations (CLM). Additionally, we used propensity score-matched analysis (PSMA) to compare these outcomes for thoracoscopic versus open surgical approaches. METHODS: Kids' Inpatient Database (1997-2009) was used to identify congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS) patients undergoing resection. Open and thoracoscopic CPAM resections were compared using PSMA. RESULTS: 1547 cases comprised the cohort. In-hospital survival was 97%. Mortality was higher in small vs. large hospitals, p<0.005. Survival, pneumothorax (PTX), and thoracoscopic procedure rates were higher, while transfusion rates and length of stay (LOS) were lower, in children ≥3 vs. <3months (p<0.001). Multivariate analysis demonstrated longer LOS for older patients and Medicaid patients (all p<0.005). Total charges (TC) were higher for Western U.S., older children, and Medicaid patients (p<0.02). PSMA for thoracoscopy vs. thoracotomy in CPAM patients showed no difference in outcomes. CONCLUSION: CLM resections have high associated survival. Children <3months of age had higher rates of thoracotomy, transfusion, and mortality. Socioeconomic status, age, and region were independent indicators for resource utilization. Extent of resection was an independent prognostic indicator for in-hospital survival. On PSMA, thoracoscopic resection does not affect outcomes.

Ultrasonographic assessment of laryngohyoid position as a predictor of dorsal displacement of the soft palate in horses.

Dorsal displacement of the soft palate is an important cause of poor performance in racehorses, yet its etiology is not fully understood. Diagnosis requires treadmill videoendoscopy, which is not widely available. The relationship of the larynx, the hyoid apparatus, and the remainder of the skull may be important in predisposing horses to dorsal displacement of the soft palate. We hypothesized that this relationship could be accurately assessed in unsedated horses through ultrasonographic examination. Fifty-six racehorses presented for evaluation of poor performance were subjected to treadmill videoendoscopy and resting ultrasonography. Using ultrasound-assisted percutaneous measures of laryngo-hyoid position, the relationship between selected anatomic structures and the occurrence of dorsal displacement of the soft palate was evaluated. A significant relationship was found between the depth of the basihyoid bone at rest and the occurrence of dorsal displacement of the soft palate at exercise (P = 0.03). Other measures of laryngohyoid position were not found to be associated with dorsal displacement of the soft palate. Thus, there is an association between the occurrence of dorsal displacement of the soft palate at exercise and the resting position of the basihyoid bone, whereby on average a more ventral location of the basihyoid bone is present in horses with dorsal displacement of the soft palate. The pathophysiologic implications of this finding are not fully understood but, based on our findings, ultrasound examination is of value in assisting in the diagnosis of dorsal displacement of the soft palate.

Racing performance following the laryngeal tie-forward procedure: a case-controlled study.

REASONS FOR PERFORMING STUDY: The laryngeal tie-forward procedure (LTFP) is becoming widely used for correction of dorsal displacement of the soft palate (DDSP) despite the absence of an evidence-based assessment of its efficacy. HYPOTHESES: The LTFP returns racing performance to preoperative baseline levels and to that of matched controls; and post operative laryngohyoid position is associated with post operative performance. DESIGN AND POPULATION: Case-controlled study of racehorses undergoing a LTFP for dorsal displacement of the soft palate at Cornell University between October 2002 and June 2007. METHODS: The presence of at least one post operative start and race earnings ($) were used as outcome variables. Controls were matched by age, breed and sex from the third race prior to surgery. A novel radiographic reference system was used to determine laryngohyoid position pre- and post operatively. Data for definitively and presumptively diagnosed cases were analysed separately. RESULTS: During the study interval, 263 racehorses presented, of which 106 were included in the study; 36 had a definitive diagnosis of DDSP and 70 a presumptive diagnosis. Treated horses were equally likely to race post operatively as controls in the equivalent race. Treated horses had significantly lower earnings in the race before surgery than matched controls. The procedure moved the basihyoid bone dorsally and caudally and the larynx dorsally and rostrally. A more dorsal post operative basihyoid position and more dorsal and less rostral laryngeal position were associated with an increased probability of racing post operatively. CONCLUSIONS: Horses undergoing a LTFP are as likely to race post operatively as matched controls. The procedure restores race earnings to preoperative baseline levels and to those of matched controls. POTENTIAL RELEVANCE: This study provides strong evidence supporting the use of the LTFP in racehorses. Further work is needed to determine the relationship between laryngohyoid conformation and nasopharyngeal stability in horses.

The use of race winnings, ratings and a performance index to assess the effect of thermocautery of the soft palate for treatment of horses with suspected intermittent dorsal displacement. A case-control study in 110 racing Thoroughbreds.

REASONS FOR PERFORMING THE STUDY: There have been no reports of the efficacy of thermocautery of the soft palate (TSP) assessed objectively as a treatment of intermittent dorsal displacement of the soft palate (DDSP). OBJECTIVE: To compare: racing performance of horses that underwent thermocautery of the soft palate with matched controls; and 'Racing Post ratings' (RPR) with prize money won (RE) and a performance index (PI) for each of the horses in the study. HYPOTHESIS: Thermocautery of the soft palate has no beneficial effect on racing performance and the 3 measures of performance are significantly related. METHODS: The inclusion criteria were fulfilled by 110 horses and each was matched with 2 controls. Changes in performance were compared statistically. RPR, RE and PI were analysed using a regression model. RESULTS: The percentage of horses that improved in performance following the procedure was 28-51% for the 3 measures of performance, compared to 21-53% for the matched controls. There was no significant effect of the procedure on the changes in RPR or RE. There was a significant effect of the procedure on the change in PI (P=0.015) with more treated horses achieving an improved PI and fewer acquiring a worse PI than matched control horses. The measures of performance showed significant correlation. CONCLUSIONS: Thermocautery of the soft palate alone may not be the most efficacious treatment of DDSP. POTENTIAL RELEVANCE: Production of a reliable measure of racehorse performance may be possible.

Effect of head position on radiographic assessment of laryngeal tie-forward procedure in horses.

OBJECTIVE: To assess the effect of head position on relative position of the larynx and hyoid apparatus in horses with palatal dysfunction, and to define a standard position for radiographic assessment of laryngeal tie-forward. STUDY DESIGN: Prospective clinical study. ANIMALS: Adult horses (n=9) with palatal dysfunction. METHODS: Left lateral radiographs of the larynx were obtained pre and postoperatively for 3 different head positions (flexed=90 degrees ; neutral=100 degrees ; extended=115 degrees ). Distance between thyrohyoid bone and thyroid cartilage was measured. Data were analyzed to investigate differences between head positions, and to compare differences between pre- and postoperative measurements. RESULTS: Head position had a significant effect on relative position of the larynx and hyoid apparatus preoperatively. There was no significant difference in postoperative measurements of the 3 head positions. A significant difference between pre- and postoperative measurements was found with the head in the neutral or extended position whereas there were no significant differences between pre- and postoperative measurements using the flexed position. CONCLUSIONS: Head position affects the relationship between the hyoid apparatus and larynx preoperatively, with greatest distance occurring when the head is extended. In a flexed position, it is not possible to ascertain whether laryngeal position has changed postoperatively. CLINICAL RELEVANCE: A standardized head position is necessary when assessing the laryngeal tie-forward procedure radiographically. An extended head position is most useful for this assessment.

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.