Cardiopulmonary function in adolescent patients with pectus excavatum or carinatum.

BACKGROUND: Pectus excavatum (PE) and pectus carinatum (PC) have generally been considered an aesthetic issue, although there is growing evidence of associated cardiopulmonary function (CPF) impairment, especially in PE patients. The study goal was to determine any correlation between pectus malformations and cardiopulmonary symptoms and function based on systematic assessment of CPF and thoracic measurements, such as Haller Index (HI) and sternal torsion angle (STA). METHODS: Data from 76 adolescent patients with PE (n=30) or PC (n=46) were retrospectively collected referred between January 2015 and April 2018. CPF measurements and thoracic imaging were performed in all patients. HI and STA correction indexes were measured in all patients. FINDINGS: Medical records from 76 patients (PE n=30; PC n=46) were analysed. Patients were predominantly male (>93.3%), and aged between 13 and 14½ old. PE was associated with airway obstruction, with a forced expiratory volume in 1 s value under the lower limit of normal in 13% of cases (p<0.001). Restrictive syndrome was observed in 23% of cases (p<0.001), with a Z score for total lung capacity under the lower limit of normal. In PC, pulmonary function was not affected. All patients showed slightly decreased values of left and right ejection fraction and cardiac index at rest, although values were within normal range. There were no significant correlations between pulmonary and cardiac functions or between low CPF and thoracic measurements. INTERPRETATION: Our results confirm the modest impact of pectus malformations on CPF at rest, without correlation with anamnestic dyspnoea on exertion, nor with chest pain or anatomical measurements. Validation of new correction indexes could be helping characterise these malformations and choose optimal therapeutic management.

Safely Decreasing Rigid Bronchoscopies for Foreign-Body Aspiration in Children: An Algorithm for the Emergency Department.

INTRODUCTION: Rigid bronchoscopy was traditionally performed in the management of foreign-body aspiration (FBA). More recently, since development of a less invasive method, flexible bronchoscopy has been proposed in some centers for the management of FBA. For the past few years, we have applied a decisional algorithm, privileging flexible bronchoscopy for diagnosis and, in some cases, for extraction of foreign body (FB). Our aims are first to analyze our current management of FBA and second to examine the bronchoscopic findings and complications. MATERIALS AND METHODS: Retrospective medical chart review of all patients with clinical suspicion of FBA who underwent bronchoscopy (flexible and/or rigid) from 2009 through 2014. RESULTS: An FB was found in 23 (33%) of the 70 patients included in the study (45 boys, 25 girls; median age: 21.5 months). Diagnosis of FBA was made on first intention in 22/23 (96%) and extraction was performed in 7/23 (30%) by flexible bronchoscopy. Rigid bronchoscopy was necessary for the extraction of the 16/23 (70%) remaining FBs. The rigid procedure was performed as first intention in only two (3%) patients, and one of the two was negative. Among the clinical signs of FBA, none were > 90% specific except for apnea (100%), but which was poorly sensitive (22%). Seven clinical and radiologic signs were found to be significantly different between FB+ and FB- groups: sudden choking, cyanosis, apnea, decreased breath sounds, atelectasis, mediastinal shift, and air trapping. Conversely, when none of these symptoms or signs and no clear history of sudden choking were present (in 15/70 patients), no FB was found. No life-threatening complications or death were observed. CONCLUSION: Our current management of FBA allows us to avoid almost all negative rigid bronchoscopies. In addition, we identified some symptoms and clinical and radiologic signs whose absence was highly predictive of negative bronchoscopy. We propose a novel algorithm for management of FBA that will help decrease the number of negative bronchoscopies.

Airway compression management in late-presenting absent pulmonary valve syndrome.

INTRODUCTION: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. METHODS: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. RESULTS: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months-11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries - two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. CONCLUSIONS: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.

Early blood exchange transfusion in malignant pertussis: a case report.

OBJECTIVE: To report early blood exchange transfusion in malignant pertussis and a favorable clinical outcome. SETTING: A pediatric intensive care unit in a tertiary hospital in Geneva, Switzerland. DESIGN: A descriptive case report. PATIENT: An 8-wk-old girl was diagnosed with malignant pertussis (extreme leukocytosis, seizures, pneumonia, and secondary severe hypoxic respiratory failure associated with pulmonary hypertension). After administration of a one-volume blood exchange transfusion, a rapid decrease in white blood cell count (from 119,000/mm(3) to 36,500/mm(3)) was observed and followed by clinical improvement and favorable outcome despite the initial presence of all described risk factors associated with a high mortality. CONCLUSION: The use of exchange blood transfusion early in the course of the disease might help to prevent a fatal outcome of malignant pertussis.

Bronchoscopic diagnosis of asymptomatic unilateral pulmonary vein atresia in an infant.

An eight-month-old boy with findings of persistent left pulmonary basal infiltrate was diagnosed with congenital unilateral pulmonary vein atresia by bronchoscopy. Cardiac catheterization documented slow left pulmonary venous return to atretic pulmonary veins. Conservative treatment was chosen because the child was asymptomatic and corrective surgery or percutaneous intervention was not technically possible. After a 3-year follow-up, the child still has no documented pulmonary hypertension. Early diagnosis of unilateral pulmonary vein atresia is important to anticipate potential threatening complications like pulmonary hypertension and hemoptysis. Surgical treatment of this entity might be drastic and complex and should be weighed against a conservative alternative and careful follow-up.

Nasal nitric oxide measurements to screen children for primary ciliary dyskinesia.

STUDY OBJECTIVE: To examine the usefulness of exhaled and nasal nitric oxide (NO) measurements to detect primary ciliary dyskinesia (PCD) in children. DESIGN AND METHODS: The study population consisted of 34 children with symptoms suggestive of PCD who were previously referred to our pediatric university respiratory disease clinic for a diagnostic workup including analysis of ciliary structure and function by respiratory mucosal biopsy. PCD was diagnosed in 17 of the 34 children according to the ciliary biopsy results. Measurements of nasal and exhaled NO were performed according to European Respiratory Society and American Thoracic Society guidelines in the patients with and without biopsy-proven PCD, and also in 24 healthy age-matched subjects. RESULTS: Nasal NO was significantly lower in those children with proven PCD (geometric mean; 13.7 parts per billion [ppb]), compared to those who had negative biopsy results (132.7 ppb) and healthy control subjects (223.7 ppb). The measurement of nasal NO in our study population showed, below a cut-off level of < 105 ppb, a specificity of 88% for PCD, and positive predictive value of 89%. Nasal NO above a cut-off level of 105 ppb excluded PCD with a 100% certainty. The lower levels of exhaled NO in patients with PCD did not reach statistical significance. CONCLUSION: The measurement of nasal NO appears to be a useful tool to screen children for PCD and to exclude this disease in those with high nasal NO levels.