Infant Pulmonary Function Tests in Children with Airway Anomalies and Correlation with Bronchoscopy Findings.

OBJECTIVES: To evaluate the role infant pulmonary function tests (Tidal Breathing Flow Volume Loops, TBFVL) in children with airway anomalies and to correlate the TBFVL so obtained with bronchoscopy findings. METHODS: In this prospective cohort study, we enrolled children aged 0-2 years with airway anomalies and performed TBFVL and bronchoscopy. The primary outcome measure was graphic pattern of TBFVL in laryngomalacia. Secondary outcome measures were types of TBFVL results in various airway anomalies and controls. RESULTS: Out of 53 children enrolled, 28 (52.3%) had laryngomalacia. Pattern 3 (fluttering of inspiratory limb) was commonest TBFVL pattern in laryngomalacia. Among TBFVL parameters, the ratio of inspiratory time to expiratory time (Ti/Te) and tPTEF/tE was significantly high in children with isolated laryngomalacia compared to controls. At six months of follow-up, TBFVL pattern 1 (normal) became the commonest pattern. CONCLUSION: A particular type of airway anomaly may have a characteristic graphic pattern in TBFVL and TBFVL pattern may indicate improvement in airway anomalies in follow-up.

THE BRONCHOPULMONARY SYSTEM DAMAGE UNDER THE INFLUENCE OF IONIZING RADIATION EXPOSURE AND RADIONUCLIDES INHALATION IN THE CONDITIONS OF THE CHORNOBYL CATASTROPHE (SUMMARIZING OF 35 YEARS RESEARCH EXPERIENCE). / URAZhENNIa BRONKhOLEGENEVOÏ SYSTEMY VNASLIDOK VPLYVU IONIZUIuChOGO OPROMINENNIa TA INGALIaTsIINOGO NADKhODZhENNIa RADIONUKLIDIV V UMOVAKh ChORNOBYL'S'KOÏ KATASTROFY (UZAGAL'NENNIa DOSVIDU 35 ROKIV DOSLIDZhEN').

The review is devoted to the summarizing of 35 years of research of ionizing radiation exposure and radionuclidesinhalation influence on the bronchopulmonary system of clean-up workers of the Chornobyl NPP accident. Radiationand hygienic preconditions for the formation of chronic respiratory pathology are considered, taking into accountthe dosimetric data of irradiation of the bronchopulmonary system.The main clinical symptoms, features of disorders of pulmonary ventilation capacity and endoscopic forms of lesionsof the bronchopulmonary system of participants in the liquidation of the accident were determined.On the basis of pathomorphological, microbiological and immunological researches the pathomorphosis of chronicnonspecific lung diseases in the conditions of the Chernobyl catastrophe is proved.It is proved that under combined influence of external irradiation and inhalation of a fragmentary mixture ofradionuclides in the condition of the Chernobyl catastrophe, the bronchopulmonary system has become one of themain «targets¼-tissues, of realization of stochastic and nonstochastic effects.

Etiologies of apnea of infancy.

BACKGROUND: To date there are limited data in the literature to guide the initial evaluation for etiologies of apnea in full-term infants born at greater than or equal to 37 weeks conceptional age (apnea of infancy [AOI]). Pediatricians and pediatric pulmonologists are left to pursue a broad, rather than targeted and a stepwise approach to begin diagnostic evaluation. METHODS: We performed a retrospective chart review of 101 symptomatic full-term infants (age under 12 months) diagnosed with apnea with an inpatient multichannel pneumogram (six channels) or a fully attended overnight pediatric polysomnogram in our outpatient sleep center accredited by American Academy of Sleep Medicine (AASM), scored using the standards set forth by the AASM. The infant was diagnosed as having AOI if the apnea hypopnea index (AHI) was greater than 1 (AHI is defined as the number of apnea and hypopnea events per hour of sleep). The final diagnosis/etiology was determined based on physician clinical assessment and work up. We then determined the frequency for each diagnosis. RESULTS: We found that the three most common etiologies were gastroesophageal reflux disease (GERD) (48/101), upper airway abnormalities/obstruction (37/101), and neurological diseases (19/101). There were significant numbers of infants with multiple etiologies for AOI. CONCLUSION: Based on the frequencies obtained, pediatric practitioners caring for full-term infants with apnea of unknown etiology are advised to begin with evaluation of more likely causes such as GERD and upper airway abnormalities/obstruction before evaluating for less common causes.

Clinical presentation and outcome in congenital pulmonary malformation: 25 year retrospective study in Thailand.

BACKGROUND: Congenital pulmonary malformations (CPM) are a group of rare abnormal lung development lesions that can have various presentations. The aim of this study was to define the differences in the clinical presentations of CPM in neonates, infants, and children, and to review the outcomes. METHODS: A retrospective study was conducted at a tertiary care hospital in southern Thailand between 1992 and 2016. RESULTS: Fifty-four patients were diagnosed with CPM, and the median age at onset was 1.7 months (IQR, 0.03-10 months). There were 33 cases (61.1%) of congenital pulmonary airway malformations, two (3.7%) of bronchogenic cyst, eight of (14.8%) congenital lobar emphysema, seven of (13.0%) pulmonary sequestrations, and four of (7.4%) congenital lung cysts. Twenty patients under 1 month old and 16 patients who were 1-12 months old had symptoms of respiratory distress. In contrast, 13 patients >1 year old had symptoms of pulmonary infection. There were significant differences in the numbers of patients who had cyanosis (P = 0.006), cough (P < 0.001), and fever (P < 0.001) between the three age groups. Thirty-eight patients (70%) required surgical treatment involving lobectomy (78.9%). Median follow-up duration was 28.1 months (IQR, 3.7-9.4 months). Nine of 10 patients had abnormal lung function tests, and 80.6% of patients had no subsequent limitations in physical activities. CONCLUSIONS: Respiratory distress is the important clinical feature in neonates and infants, whereas the signs of pulmonary infection usually occur in children >1 year old. Good outcomes usually occur after surgery but need long-term follow up including lung function assessment.

Effect of lung hypoplasia on the cardiorespiratory transition in newborn lambs.

Newborns with lung hypoplasia (LH) commonly have limited respiratory function and often require ventilatory assistance after birth. We aimed to characterize the cardiorespiratory transition and respiratory function in newborn lambs with LH. LH was induced by draining fetal lung liquid in utero [110-133 days (d), term = 147d, n = 6]. At ~133d gestation, LH and Control lambs (n = 6) were instrumented and ventilated for 3 h to monitor blood-gas status, oxygenation, ventilator requirements, and hemodynamics during the transition from fetal to newborn life. Lambs with LH had significantly reduced relative wet and dry lung weights indicating hypoplastic lungs compared with Control lambs. LH lambs experienced persistent hypercapnia and acidosis during the ventilation period, had lower lung compliance, and had higher alveolar-arterial differences in oxygen and oxygenation index compared with Control lambs. As a result, LH lambs required greater respiratory support and more supplemental oxygen. Following delivery, LH lambs experienced periods of significantly lower pulmonary artery blood flow and higher carotid artery blood flow in association with the lower oxygenation levels. The detrimental effects of LH can be attributed to a reduction in lung size and poorer gas exchange capabilities. This study has provided greater understanding of the effect of LH itself on the physiology underpinning the transition from fetal to newborn life. Advances in this area is the key to identifying improved or novel management strategies for babies with LH starting in the delivery room, to favorably alter the fetal-to-newborn transition toward improved outcomes and reduced lifelong morbidity.NEW & NOTEWORTHY Current clinical management of newborns with lung hypoplasia (LH) is largely based on expert opinion rather than scientific evidence. We have generated physiological evidence for detrimental effects of LH on hemodynamics and respiratory function in newborn lambs, which mimics the morbidity observed in LH newborns clinically. The unfavorable consequences of LH can be attributed to a reduction in lung size and poorer gas exchange capabilities.

Apnea-Hypopnea Event Duration Predicts Mortality in Men and Women in the Sleep Heart Health Study.

RATIONALE: Obstructive sleep apnea is a risk factor for mortality, but its diagnostic metric-the apnea-hypopnea index-is a poor risk predictor. The apnea-hypopnea index does not capture the range of physiological variability within and between patients, such as degree of hypoxemia and sleep fragmentation, that reflect differences in pathophysiological contributions of airway collapsibility, chemoreceptive negative feedback loop gain, and arousal threshold. OBJECTIVES: To test whether respiratory event duration, a heritable sleep apnea trait reflective of arousal threshold, predicts all-cause mortality. METHODS: Mortality risk as a function of event duration was estimated by Cox proportional hazards in the Sleep Heart Health Study, a prospective community-based cohort. Gender-specific hazard ratios were also calculated. MEASUREMENTS AND MAIN RESULTS: Among 5,712 participants, 1,290 deaths occurred over 11 years of follow-up. After adjusting for demographic factors (mean age, 63 yr; 52% female), apnea-hypopnea index (mean, 13.8; SD, 15.0), smoking, and prevalent cardiometabolic disease, individuals with the shortest-duration events had a significant hazard ratio for all-cause mortality of 1.31 (95% confidence interval, 1.11-1.54). This relationship was observed in both men and women and was strongest in those with moderate sleep apnea (hazard ratio, 1.59; 95% confidence interval, 1.11-2.28). CONCLUSIONS: Short respiratory event duration, a marker for low arousal threshold, predicts mortality in men and women. Individuals with shorter respiratory events may be predisposed to increased ventilatory instability and/or have augmented autonomic nervous system responses that increase the likelihood of adverse health outcomes, underscoring the importance of assessing physiological variation in obstructive sleep apnea.

Does endoscopic thoracic sympathectomy through clipping procedure have early effects on electrocardiographic parameters?

BACKGROUND & OBJECTIVES: Hyperhidrosis is a pathologic condition of excessive sweating in amounts greater than physiologic needs. Endoscopic thoracic sympathectomy (ETS) is a globally accepted treatment modality for primary palmar and axillary hyperhidrosis. ETS also has distinctive effects on the heart, circulatory and respiratory systems. In this study early effects of ETS on electrocardiographic (ECG) parameters of hyperhidrosis patients were evaluated. METHODS: Twelve-lead ECGs were performed on 72 patients who were free from cardiovascular, metabolic, neurological and pulmonary diseases and underwent planned ETS because of hyperhidrosis, before and after the procedure within the first 24 h. Heart rate (HR), PR, QT, corrected QT (QTc), QTc/Tpeak-Tend (TpTe) intervals, P-wave and QTc/TpTe dispersions were compared by ECG. RESULTS: A total of 72 patients (24.1±6.0 yr, 17 female) were included in the study. The pre-operative HR of patients was significantly higher than post-operative HR of patients (73.8±12.8 vs. 68.1±12.6 beats/ min; P<0.001). The QTc dispersion (QTcd) durations of pre-operative patients were significantly longer than those of post-operative patients (51.5±6.3 vs. 44.9±5.6 msec; P<0.01). The TpTe dispersion value of pre-operative patients was significantly (P<0.001) higher than that of post-operative patients. INTERPRETATION & CONCLUSIONS: Our study showed that ETS through clipping procedure had positive effects on the mechanisms of arrhythmia by reducing HR, QTcd, TpTe and TpTe dispersion parameters of ECG in early periods in hyperhidrosis patients.

Exercise capacity is not decreased in children who have undergone lung resection early in life for congenital thoracic malformations compared to healthy age-matched children.

PURPOSE: The purpose of this study was to compare (i) the exercise capacity and (ii) lung function prior to and immediately post cardiopulmonary exercise tests (CPET) of children who underwent early life lung resection for Congenital Pulmonary Airway Malformations (CPAM) to healthy control children. METHOD: Eight children with CPAM (four males, age 9.6 ± 1.8 years) and eight control children without respiratory disease (three males, age 9.4 ± 1.4 years) performed a CPET on a cycle ergometer, during which maximal oxygen consumption (V̇O2max ) and heart rate were measured. Prior to and immediately post CPET, lung function measures including Nitrogen Multiple Breath Washout (MBW) and spirometry were performed. RESULTS: There were no significant between group differences in pre CPET lung function (P > 0.05) or maximal exercise capacity (V̇O2max CPAM: 39.4 mL. kg-1. min-1 , Control: 40.5 mL. kg-1. min-1 ). Post CPET, FEV1 was significantly lower in the CPAM group, with two participants diagnosed subsequently with exercise induced bronchospasm based on post-CPET spirometry and follow-up clinical investigations. CONCLUSION: Early life lung resection for CPAM does not appear to have negative implications for exercise capacity later in childhood. Clinicians should be aware that dyspnoea following exercise may be due to asthma rather than residual effects of CPAM in these children.

Hemilaryngeal Microsomia: An Anatomic Variant.

OBJECTIVES: This study aims to describe a congenital laryngeal structural variant, hemilaryngeal microsomia (HLM), and to correlate identification on physical examination with computerized tomography scan (CT) and laryngoscopy findings. METHODS: The study was conducted at a tertiary care center. Six patients presenting with hoarseness were admitted to a tertiary care otolaryngology office. These patients had asymmetrical thyroid cartilage prominence on palpation during physical examination. A diagnosis of HLM was made. All patients underwent laryngostroboscopy and CT scan. Four control patients with normal thyroid cartilage anatomy on physical examination, CT, and stroboscopy results were included for comparison. RESULTS: Disparities in thyroid cartilage angles correlated with documented physical examination findings for six out of six HLM patients. On CT scan, the average difference in left and right thyroid laminar angles was 30.2° ± 18.3° in HLM patients vs 4.00° ± 1.63° in control patients (P = 0.023). Strobosocopic findings also correlated with HLM. The arytenoid cartilage was anteriorly or medially displaced on the microsomic side in all six HLM patients. Three patients had anterior placement of the vocal process resulting in shortening of the vocal fold on the microsomic side of the larynx. CONCLUSIONS: HLM is a congenital structural anomaly of the larynx that may be palpated on physical examination. HLM found on physical examination can be correlated with asymmetries found on CT scan and endoscopy. There is no evidence that the structural features of HLM were causally related to voice symptoms, but the findings on HLM may lead to misdiagnosis. A larger study is indicated to confirm laryngeal structural differences between patients with HLM on physical examination and the general population. Whether or not HLM affects clinical or surgical outcomes remains to be studied.

Early Diagnosis of Respiratory Abnormalities in Asbestos-Exposed Workers by the Forced Oscillation Technique.

BACKGROUND: The current reference test for the detection of respiratory abnormalities in asbestos-exposed workers is spirometry. However, spirometry has several shortcomings that greatly affect the efficacy of current asbestos control programs. The forced oscillation technique (FOT) represents the current state-of-the-art technique in the assessment of lung function. This method provides a detailed analysis of respiratory resistance and reactance at different oscillatory frequencies during tidal breathing. Here, we evaluate the FOT as an alternative method to standard spirometry for the early detection and quantification of respiratory abnormalities in asbestos-exposed workers. METHODOLOGY/PRINCIPAL FINDINGS: Seventy-two subjects were analyzed. The control group was composed of 33 subjects with a normal spirometric exam who had no history of smoking or pulmonary disease. Thirty-nine subjects exposed to asbestos were also studied, including 32 volunteers in radiological category 0/0 and 7 volunteers with radiological categories of 0/1 or 1/1. FOT data were interpreted using classical parameters as well as integer (InOr) and fractional-order (FrOr) modeling. The diagnostic accuracy was evaluated by investigating the area under the receiver operating characteristic curve (AUC). Exposed workers presented increased obstruction (resistance p<0.001) and a reduced compliance (p<0.001), with a predominance of obstructive changes. The FOT parameter changes were correlated with the standard pulmonary function analysis methods (R = -0.52, p<0.001). Early respiratory abnormalities were identified with a high diagnostic accuracy (AUC = 0.987) using parameters obtained from the FrOr modeling. This accuracy was significantly better than those obtained with classical (p<0.001) and InOr (p<0.001) model parameters. CONCLUSIONS: The FOT improved our knowledge about the biomechanical abnormalities in workers exposed to asbestos. Additionally, a high diagnostic accuracy in the diagnosis of early respiratory abnormalities in asbestos-exposed workers was obtained. This makes the FOT particularly useful as a screening tool in the context of asbestos control and elimination. Moreover, it can facilitate epidemiological research and the longitudinal follow-up of asbestos exposure and asbestos-related diseases.

Outcome of infants operated on for congenital pulmonary malformations.

INTRODUCTION: Patients operated on for congenital pulmonary malformations (CPM) have excellent survival rates, but little is known about long-term morbidity. Our aim is to report the sequelae in patients operated on for CPM in infancy and to define factors that may influence their outcome. METHODS: All patients operated on for major congenital anomalies are followed in a dedicated outpatient program and evaluated at 6, 24, and 48 months of life (corrected for gestational age) and at school age at 4, 6, 8, and 12 years of life. The data are prospectively collected. Patients operated on for CPM and enrolled in the follow-up clinic between January 2004 and December 2010 are compared with a control group of term infants operated on for inguinal hernia, without other major congenital or acquired abnormalities. The two groups were compared for auxological, respiratory, and orthopedic outcome. RESULTS: In the study period, 76 consecutive patients with CPM attended our dedicated follow-up clinic. Eight non-operated patients were excluded from the study. Age at follow-up was 82.0 (56.1-103.7) months in CPM patients and 83.5 (75.2-90.4) months in controls (P = 0.79). Fifty-three patients with CPM (78%) had one or more clinical or radiological abnormality versus six (16%) control patients (OR [95%CI] 16.5 [5.8-47.2]; P < 0.0001). CONCLUSIONS: Over 50% of patients with CPM present long-term sequelae, regardless type of malformation. Therefore, long-term follow-up of patients operated on for CPM is recommended. Further studies are needed to define if, in asymptomatic patients, surgery may modify the natural history of CPM. Pediatr Pulmonol. 2016;51:1367-1372. © 2016 Wiley Periodicals, Inc.

Respiratory system involvement in Costello syndrome.

Costello syndrome (CS) is a multisystem disorder caused by heterozygous germline mutations in the HRAS proto-oncogene. Respiratory system complications have been reported in individuals with CS, but a comprehensive description of the full spectrum and incidence of respiratory symptoms in these patients is not available. Here, we report the clinical course of four CS patients with respiratory complications as a major cause of morbidity. Review of the literature identified 56 CS patients with descriptions of their neonatal course and 17 patients in childhood/adulthood. We found that in the neonatal period, respiratory complications are seen in approximately 78% of patients with transient respiratory distress reported in 45% of neonates. Other more specific respiratory diagnoses were reported in 62% of patients, the majority of which comprised disorders of the upper and lower respiratory tract. Symptoms of upper airway obstruction were reported in CS neonates but were more commonly diagnosed in childhood/adulthood (71%). Analysis of HRAS mutations and their respiratory phenotype revealed that the common p.Gly12Ser mutation is more often associated with transient respiratory distress and other respiratory diagnoses. Respiratory failure and dependence on mechanical ventilation occurs almost exclusively with rare mutations. In cases of prenatally diagnosed CS, the high incidence of respiratory complications in the neonatal period should prompt anticipatory guidance and development of a postnatal management plan. This may be important in cases involving rarer mutations. Furthermore, the high frequency of airway obstruction in CS patients suggests that otorhinolaryngological evaluation and sleep studies should be considered. © 2016 Wiley Periodicals, Inc.

Biomarker as a research tool in linking exposure to air particles and respiratory health.

UNLABELLED: Some of the environmental toxicants from air pollution include particulate matter (PM10), fine particulate matter (PM2.5), and ultrafine particles (UFP). Both short- and long-term exposure could result in various degrees of respiratory health outcomes among exposed persons, which rely on the individuals' health status. METHODS: In this paper, we highlight a review of the studies that have used biomarkers to understand the association between air particles exposure and the development of respiratory problems resulting from the damage in the respiratory system. Data from previous epidemiological studies relevant to the application of biomarkers in respiratory system damage reported from exposure to air particles are also summarized. RESULTS: Based on these analyses, the findings agree with the hypothesis that biomarkers are relevant in linking harmful air particles concentrations to increased respiratory health effects. Biomarkers are used in epidemiological studies to provide an understanding of the mechanisms that follow airborne particles exposure in the airway. However, application of biomarkers in epidemiological studies of health effects caused by air particles in both environmental and occupational health is inchoate. CONCLUSION: Biomarkers unravel the complexity of the connection between exposure to air particles and respiratory health.

Management of perinatal lung malformations.

This review uses the most up-to-date literature to help guide obstetrical providers through the diagnosis and management of perinatal lung malformations. These lesions, which include congenital pulmonary airway malformation [CPAM, formerly congenital cystic adenomatoid malformation (CCAM)] and bronchopulmonary sequestration (BPS), are relatively rare but are becoming increasingly common because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment throughout pregnancy remains the norm rather than the exception. Perinatal management strategies can differ based on the sonographic characteristics and dynamic growth patterns of lung masses. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, the importance of echocardiography and utility of maternal steroids have been recognized in cases of non-immune hydrops. Fetal surgery is now rarely performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for lung resection on an elective basis. In the vast majority of cases, the overall prognosis remains excellent.

Congenital and acquired developmental problems of the upper airway in newborns and infants.

AIM: To review the current knowledge on congenital and acquired developmental problems of the upper airway in newborns and infants. DATA SYNTHESIS: Causes of airway obstruction include problems with the nasal airway (choanal atresia), craniofacial syndromes (Apert syndrome, Crouzon syndrome), problems with facial/tongue anatomy (Pierre-Robin syndrome), the tongue (Down syndrome), or the larynx (laryngomalacia, vocal cord palsy, subglottic stenosis, subglottic hemangioma), along with lower developmental problems (tracheo/bronchomalacia). After establishing a safe airway, a detailed assessment and appropriate management are necessary. Treatment may involve simple observation, conservative management, chest physiotherapy, CPAP ventilation, and surgery, urgently or in a second phase. CONCLUSION: Upper airway diseases in neonates and infants may be life threatening, or challenging regarding diagnosis and management. There should be a very low threshold for referring these children, after establishing a safe airway, for a specialist opinion and care in a tertiary unit, if local facilities are limited or unavailable.

Cystic fibrosis: the role of the small airways.

Cystic fibrosis (CF) infants are born with normal airway anatomy, and dilatation of mucus glands in smaller airways has been described as the earliest histological changes in the lung of patients. This and other evidence has led to the concept that the small airways may be the region demonstrating initial pathology. Studies clarifying this have been challenged by the lack of a clear definition of what constitutes "small airways" and the difficulties to find accurate measures to quantify and track abnormalities in this region of the lung. Dynamic lung function test are not ideally suited to capture small airway abnormalities as the overall surface area of the small airways is about 40 times greater than that of central airways. Measures that are linked to airway size therefore often fail to capture abnormalities in a disease such as cystic fibrosis that is unevenly distributed throughout the lung. This review summarizes our current understanding of the role of small airways in disease development in cystic fibrosis patients and describes the spectrum of diagnostic tools available to diagnose and follow small airway disease and highlight the opportunities as well as challenges of targeting small airways via aerosol therapy in CF patients.

The normal and the challenging pediatric airway.

Management of a child's airway is one of the main sources of stress for anesthetists who do not routinely anesthetize children. Unfortunately, trainees are gaining less experience in pediatric airway management than in the past, which is particularly difficult at a time when some beliefs about airway management are being challenged and airway management is less standardized. Fortunately, most children have an easily managed, normal airway. Nevertheless, it is of vital importance to teach our trainees the basic airway skills that are probably the most important skill in an anesthetists' repertoire when it comes to a difficult airway situation. This review focuses on the airway management in children with a normal and a challenging airway. Different choices of airway management in children, and their advantages and disadvantages are discussed. Furthermore, the three broad causes of a challenging airway in children and infants are highlighted - the difficulty obtaining a mask seal, difficulty visualizing the vocal cords, and the third cause in which the larynx can be visualized but the difficulty lies at or beyond that level. Guidelines are given how to deal with these patients as well as with the feared but rare scenario of 'cannot ventilate, cannot intubate' in children.

Pulmón en herradura con drenaje venoso pulmonar normal / Horseshoe lung with normal pulmonary venous return

Antecedentes: El pulmón en herradura es una rara anomalía congénita caracterizada por un istmo medio de parénquima pulmonar que une los segmentos posterobasales de ambos pulmones por detrás del corazón, acompañándose de una hipoplasia pulmonar unilateral. En el 80% de los casos se asocia con un síndrome de la cimitarra, consistente en drenaje venoso pulmonar derecho anómalo, hipoplasia pulmonar derecha y perfusión arterial sistémica a alguna porción del pulmón. Caso clínico: Niña de 6 años que desde el nacimiento presenta infecciones respiratorias bajas de repetición y escoliosis. Rx de tórax, angio-TC y RM. Hipoplasia pulmonar derecha, dextrocardia e istmo pulmonar que une las bases de ambos pulmones por detrás del pericardio. El pulmón derecho hipoplásico tiene un pequeño suministro sistémico que proviene de la aorta abdominal. La arteria pulmonar derecha es hipoplásica. El drenaje venoso de las venas pulmonares derechas es normal. Conclusiones: Se presenta un pulmón en herradura sin drenaje venoso anómalo (AU)

Background: Horseshoe lung is a rare congenital anomaly characterised by a midline isthmus of pulmonary parenchyma connecting the posterior basal segments of the lungs behind the heart in conjunction with unilateral pulmonary hypoplasia. Of all cases, 80% are associated with scimitar syndrome, consisting of right anomalous pulmonary venous drainage, pulmonary hypoplasia of the right lung and systemic arterial perfusion to some lung areas. Case: A six year old girl who had recurrent lower respiratory infections since birth. Chest Rx, angioCT and MR showed: hypoplasia of the right lung, dextrocardia and pulmonary isthmus bridging both lungs behind the pericardium. The right hypoplastic lung had little systemic supply coming from the abdominal aorta. The right pulmonary artery was hypoplastic. The right pulmonary venous drainage was normal. Conclusions: We present a case of horseshoe lung without abnormal venous drainage (AU)