Actualización y controversias en el tratamiento de las malformaciones congénitas de la vía aérea pulmonar / Update and disagreements in the treatment of congenital pulmonary airway malformations

Congenital pulmonary airway malformations (CPAM) are rare and occur due to a failure in lung embryological development. They are classified according to their pathological characteristics and their anatomical origin. They can occur from the antenatal period to adulthood, can be associated with hydrops fetalis, respiratory distress, recurrent infections, or in an otherwise asymptomatic patient. In this article we carry out a scope review of the literature to answer frequent questions of the clinical teams in charge of patients with CPAM, such as the antenatal approach, the different types of surgeries and the management of asymptomatic patients. Although the indication for surgery is clear in symptomatic patients, little is known about its natural history of this condition, including the possibility of spontaneous resolution and the development of complications or neoplasm. So, the treatment of asymptomatic patients continues to be controversial. Therapeutic decisions must be made by multidisciplinary teams with the informed participation of parents and patients. In our opinion, considering the excellent results of minimally invasive surgery, its low incidence of complications, and practically zero mortality when performed by experienced groups, it seems reasonable to consider elective resection of a MCVAP in asymptomatic patients.

Las malformaciones congénitas de la vía aérea pulmonar (MCVAP) son infrecuentes y ocurren debido a una falla en el desarrollo embriológico pulmonar. Se clasifican de acuerdo con sus características patológicas y a su origen anatómico. Se pueden presentar desde el periodo antenatal hasta la adultez, asociarse a cuadros de hidrops fetal, distrés respiratorio, infecciones recurrentes, o como un hallazgo en pacientes asintomáticos. En este artículo realizamos una revisión bibliográfica exploratoria para responder dudas frecuentes de los equipos clínicos a cargo de pacientes con MCVAP, como el enfrentamiento antenatal, los distintos tipos de cirugía y su abordaje, y el manejo de pacientes asintomáticos. Si bien la indicación de cirugía es clara en pacientes sintomáticos, poco se conoce acerca de su historia natural, incluyendo la posibilidad de resolverse de forma espontánea, de complicarse o de evolucionar hacia el desarrollo de una neoplasia, por lo que el tratamiento de pacientes asintomáticos continúa siendo controversial. Las decisiones terapéuticas deben ser tomadas por equipos multidisciplinarios con la participación informada de los padres y de los pacientes. En nuestra opinión, considerando los excelentes resultados de la cirugía mínimamente invasiva, su baja incidencia de complicaciones y prácticamente nula mortalidad, al ser realizada por grupos con experiencia, nos parece razonable plantear la resección electiva de una MCVAP en un paciente asintomático.

The Validity of the Modified Tal Score and Wang Respiratory Score Instruments in Assessing the Severity of Respiratory System Disorders in Children.

Nurses need to have a high awareness of children's respiratory status changes to prevent worse conditions such as respiratory failure. For this reason, nurses require a tool or instrument that can facilitate a comprehensive assessment of the severity of respiratory system disorders in children. The aim of this study was to identify the validity and reliability of the Modified Tal Score and Wang Respiratory Score instruments and their sensitivity and specificity for assessing the severity of respiratory system disorders in children. A descriptive-analytic research design and a cross-sectional approach were used in this research. A total of 48 children aged 0-2 years who were hospitalized in a top referral hospital in Jakarta with acute respiratory infections were assessed for the severity of respiratory system impairment using the Modified Tal Score and the Wang Respiratory Score instruments. The modified Tal Score had a strong correlation with SpO2 (r = -0.699; p = 0.0001; α = 0.05), as did the Wang Respiratory Score (r = -0.501; p = 0.0001; α = 0.05). The Cronbach's Alpha value for the Modified Tal Score instrument was 0.768, while that for the Wang Respiratory Score instrument was 0.68. Analysis using ROC curves gave an AUC value of 0.897 (95% CI 0.794; 1, p = 0.0001) for the Modified Tal Score instrument for predicting the severity of respiratory system disorders in children versus 0.815 (95% CI 0.681; 0.95, p = 0.0001) for the Wang Respiratory Score instrument. These results showed that the Modified Tal Score instrument has higher validity and reliability, as well as better sensitivity and specificity, than the Wang Respiratory Score instrument for assessing the severity of respiratory system disorders in children. A further study for instruments' improvement by classifying the children's respiratory rate according to their age, as well as adding an item related to the use of supplementary oxygen, is needed.

A review of congenital lung malformations with a simplified classification system for clinical and research use.

PURPOSE: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. RESULTS: The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. CONCLUSIONS: Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

Classification of congenital nasal deformities: a proposal to amend the existing classification.

Congenital nasal anomalies are rare malformations with a broad spectrum of defects. The only existing classification strictly relating to nasal anomalies was presented by Losee et al. (Plast Reconstr Surg 113(2):676-689, 2004). The aim of this paper is to propose some suggestions, based on our current knowledge and experience gained by treating our patients in the clinic, in creating a specification of patients with congenital nasal anomalies. All patients with congenital nose defects treated in our health center were selected for this study. The research was retrospective and included years from 1995 to 2015. Nasal anomaly associated with cleft lip and palate was excluded. Patients were classified into four categories of congenital nasal anomalies, according to Losee et al. CLASSIFICATION: In the period of 20 years, 191 patients with congenital nasal anomalies were treated in our health center. Type 1 defects were found in 124 patients, type 2 in 15, type 3 in 32 and type 4 in 20 patients. The nasal defect accompanying craniofacial syndrome is the most common type of nose malformation. The nose cleft is not always a part of craniofacial clefts; isolated forms of such malformations can occur. Vascular anomalies, due to different etiology, categorization and treatment, should not be recognized as nasal malformation.

Intra- and interobserver reliability estimates for identification and grading of upper respiratory tract abnormalities recorded in horses at rest and during overground endoscopy.

BACKGROUND: Previous studies support good intra- and interobserver agreements for endoscopic evaluation of various upper respiratory tract (URT) diseases in horses. However, these studies mainly assessed resting endoscopic examination videos and/or focussed on a single URT abnormality. OBJECTIVES: To estimate intra- and interobserver agreement for identification and grading of all URT abnormalities from resting and overground endoscopy (OGE) videos of Thoroughbreds. STUDY DESIGN: Blinded, fully crossed design. METHODS: Resting and OGE URT videos for n = 43 Thoroughbreds were retrospectively chosen based on identification of common URT disorders. The videos were randomly evaluated in duplicate by 4 raters blinded to all information including prior URT disorder(s) diagnosis. Abnormalities were graded using well-described ordinal scales. Intra- and interobserver agreements were estimated using Cohen's weighted κ and Krippendorff's α, respectively. RESULTS: Intraobserver agreement was perfect/nearly perfect for arytenoid symmetry at exercise, epiglottic entrapment and epiglottic retroversion, substantial for arytenoid asymmetry at rest, palatal dysfunction (PD), medial deviation of the aryepiglottic folds (MDAF), pharyngeal mucus and epiglottic grade at exercise and moderate for vocal fold collapse (VFC), ventromedial luxation of the apex of the corniculate process of the arytenoid (VLAC), nasopharyngeal collapse (NPC) and epiglottic grade at rest. Interobserver agreement was substantial for arytenoid symmetry at exercise and PD and moderate for arytenoid asymmetry at rest, MDAF, VLAC and epiglottic entrapment. It was only fair for VFC, epiglottic grade at exercise, epiglottic retroversion, pharyngeal mucus and NPC and poor for epiglottic grade at rest. MAIN LIMITATIONS: Sample size was insufficient to allow assessment of the effect of one abnormality on the grading of another abnormality. CONCLUSIONS: Observers were consistent in grading URT disorders. However, significant disparity in grading existed between observers for some conditions affecting reliability.

Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation.

Congenital pulmonary airway malformation (CPAM) is one of the most common lung lesions detected prenatally. Despite the research efforts made in the past few years, controversy and lack of clarity in the literature still exist regarding nomenclature, classification, pathogenesis and the management of CPAM. Therefore, it is of greatest importance to delineate the natural history of CPAMs and to create a consensus to guide the management and follow-up of these lesions. This review will focus on classification systems, highlighting the most recent advancements in pathogenesis, and current practice in the prenatal diagnosis of CPAM. Strategies of prenatal management and postnatal management will be reviewed. Long-term follow-up, including lung cancer risk, is discussed and an outcome perspective is presented.

Oculoauriculofrontonasal syndrome: case series revealing new bony nasal anomalies in an old syndrome.

Frontonasal Dysplasia (FND) and Oculo-auriculo-vertebral spectrum (OAVS) are two well-recognized clinical entities. With features of both FND and OAVS, the term oculoauriculofrontonasal syndrome (OAFNS) was coined in 1981. The OAFNS phenotype combines elements of abnormal morphogenesis of the frontonasal and maxillary process (derived from forebrain neural crest) with abnormal development of the first and second branchial arches (derived from hindbrain neural crest). We present a case series of 33 children with OAFNS ascertained from a comprehensive review of the literature and report an additional retrospective series of eight patients displaying features consistent with OAFNS. Notably, in a subset of our cases, we have observed abnormalities in nasal ossification and bony structures of the maxilla that have not previously described in OAFNS and are not seen in either FND or OAVS. We present the phenotype and novel naso-maxillary findings and explore potential etiologic and developmental pathways for OAFNS. We highlight the differences in phenotypic characteristics of OAFNS compared to OAVS and FND. These observations support the classification of OAFNS as a discrete syndrome. Further phenotypic refinements of OAFNS are needed to understand pathogenesis of this syndrome and the newly described nasal malformation may help identify the etiology.

Congenital pulmonary malformations in pediatric patients: review and update on etiology, classification, and imaging findings.

Congenital pulmonary malformations represent a heterogeneous group of developmental disorders affecting the lung parenchyma, the arterial supply to the lung, and the lung's venous drainage. In both asymptomatic and symptomatic pediatric patients with congenital pulmonary malformations, the diagnosis of such malformations usually requires imaging evaluation, particularly in cases of surgical lesions for preoperative assessment. The goal of this article is to review the current imaging techniques for evaluating congenital pulmonary malformations and their characteristic imaging findings, which can allow differentiation among various congenital pulmonary malformations in pediatric patients.

Nasal septal deformities in ear, nose, and throat patients: an international study.

PURPOSE: The purpose of this study was to investigate the incidence and characteristics of nasal septum deformities in ear, nose, and throat (ENT) patients in various geographic regions in the world. MATERIALS AND METHODS: Anterior rhinoscopy without nasal decongestion was performed in 17 ENT centers in 14 countries. The septal deformities were classified according to the classification system proposed by Mladina. RESULTS: A total of 2589 adult ENT patients (1500 males and 1089 females) were examined. Septal deformities were found in 89.2% of subjects. Left-sided deformities were slightly more prevalent than right-sided deformities (51.6% and 48.4%, respectively). The most frequent type of deformity was type 3 (20.4%). Straight septum was found in 15.4% of females and 7.5% of males. CONCLUSIONS: Almost 90% of the subjects showed 1 of the 7 types of septal deformity. There were no statistically significant differences in the incidence of their appearance among particular geographic regions. Type 3 was the most frequent type. Straight septum was twice as frequent in females than in males.

Type I posterior laryngeal clefts.

Posterior laryngeal clefts (PLCs) are described in the literature as rare laryngeal abnormalities. The authors believe type I clefts are much more common than previously reported. In two busy pediatric tertiary care centers, such clefts are the second most common congenital laryngeal finding at rigid endoscopy, second only to laryngomalacia. PLCs frequently present with symptomatology that can be attributed to other common disease processes and are often undiagnosed unless the surgeon maintains a high index of suspicion and specifically examines the posterior glottis by palpation during microlaryngoscopy. This report presents a series of 41 patients with type I PLCs, reviews their subtle and often confusing presenting signs and symptoms, and describes a simple yet reliable method of diagnosis.