Congenital Absence of Tracheal or Bronchial Rings.

OBJECTIVE: Congenital airway stenosis secondary to absent tracheal or bronchial rings is a rare congenital anomaly that is difficult to manage both clinically and surgically. This typically manifests as severe segmental tracheomalacia, and only isolated cases with short-term follow-up have been previously described. We aim to describe a series of children with absent tracheal or bronchial rings who underwent surgical management and had long-term follow-up. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. METHODS: Patients with absent tracheal or bronchial rings from 2002 to 2016. Electronic and paper medical records were queried to obtain demographics, age at diagnosis and surgery, pre- and postoperative symptoms, location of absent rings, procedure performed, length of follow-up, and adjunctive procedures performed. RESULTS: Nine subjects were identified who underwent slide tracheoplasty for correction of congenital absent tracheal or bronchial rings. Age at diagnosis ranged from 10 days to 5 years of age (median, 4 weeks). Age at surgery ranged from 3 weeks to 5 years of age (median, 5 weeks). Six out of 9 subjects were extubated on postoperative day 1. Only 1 subject required additional intervention, which included balloon dilation, tracheobronchial stenting, and aortopexy to alleviate the obstruction. Mean follow-up time was 5.89 years. CONCLUSIONS: This is the largest series of children with absent tracheal rings who underwent slide tracheoplasty with long-term follow-up presented to date. Slide tracheoplasty is an effective surgical intervention for the treatment of absent tracheal or bronchial rings in infants and young children.

Congenital Bronchial Stenosis Presenting as Neonatal Respiratory Distress: A Case Report.

BACKGROUND: Neonatal respiratory distress has a broad differential that includes cardiac, pulmonary, anatomic, and infectious etiologies. Congenital stenotic lesions of the trachea and bronchus are rare and can occur anywhere along the tracheobronchial tree. Patients with tracheobronchial stenosis typically present in the neonatal period with respiratory distress. CASE REPORT: We present a case of a 10-day-old term female who presented to the emergency department (ED) with tachypnea and increased work of breathing. She was found to have congenital bronchial stenosis of her right mainstem bronchus. She was stabilized in the ED and remained in the neonatal intensive care unit until successful slide tracheoplasty was performed. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Congenital bronchial stenosis is a rare etiology of respiratory distress in a neonate. Anatomic lower airway abnormalities are an important cause of neonatal tachypnea and must remain on the differential. In addition to respiratory stabilization with noninvasive or invasive support, evaluation should be directed at determining the location and anatomic characteristics of the area of stenosis.

Complex rhinobronchial dystrophy and immunodeficiency: Chance association or exceptional congenital syndrome?

INTRODUCTION: We report a case of an exceptional syndromic association of apparently congenital rhinobronchial dystrophy associated with congenital anosmia and common variable immunodeficiency in a twelve-year-old girl. CASE SUMMARY: This young girl, born in 2000, consulted for the first time in 2012 for recurrent respiratory tract infections, refractory to all forms of treatment, starting in early childhood, associated with congenital anosmia and severe atrophic rhinitis as well as common variable immunodeficiency. The laboratory work-up essentially revealed IgG4 deficiency and imaging demonstrated bronchiectasis (lingula), multiple tracheobronchial diverticula, atrophic rhinitis and congenital anosmia with agenesis of the olfactory bulbs and sulci. DISCUSSION: After eliminating a number of differential diagnoses, we were left with the problem of the aetiology, the possible links between these various symptoms and the genetic basis for this apparently congenital complex rhinobronchial disease associated with common variable immunodeficiency. Do these various symptoms correspond to a chance association or an exceptional congenital syndrome that has not yet been identified in the literature? CONCLUSION: A review of the clinical and genetic literature did not enable us to propose a single diagnosis for these symptoms or this complex syndrome.

Descending Aortic Translocation and Right Pulmonary Artery Reimplantation for Midline Descending Aorta and Crossed Pulmonary Arteries in an Infant.

Left mainstem bronchial compression by a midline descending thoracic aorta is a rare anatomic variant. Translocation of the descending thoracic aorta to the ascending aorta has recently been described to treat this condition. We performed an aortic translocation and right pulmonary artery reimplantation in a 4-month-old infant with severe pulmonary hypertension secondary to right pulmonary artery stenosis and left bronchial compression by a midline descending thoracic aorta. The procedure was successful in ameliorating the patient's left mainstem bronchial compression and pulmonary hypertension. Descending aortic translocation should be considered when the left bronchus is compressed causing respiratory symptoms.

Predictors of early lobectomy after birth in prenatally diagnosed congenital pulmonary airway malformation.

PURPOSE: The purpose of this study was to clarify the relationship between congenital pulmonary airway malformation volume ratio (CVR) of bronchial atresia (BA), CVR of congenital cystic adenomatoid malformations (CCAM), and time of surgery after birth. METHOD: We retrospectively analyzed data of 36 BA and CCAM cases, prenatally diagnosed as CPAM from 2009 through 2014. RESULTS: Within 2 h after birth, 12 neonatal patients underwent emergent (EMG) lobectomy. Five cases of lobectomy were performed urgently (UG) from 12 to 48 h after birth. Four cases of lobectomy were required within 30 days after birth (early = EAG). We performed lobectomy in 15 other patients at 11 months after birth (late = LG). Of the EMG cases, 11 were macrotype CCAM (maximal CVR >2.0), and 4 of 5 UG cases were microtype CCAM (CVR >2.0). Of the EAG cases, 3 of 4 were macrotype CCAM with CVR of <1.5. Of 15 LG, 13 were BA and showed a CVR of 0.13-3.0 (median, 0.78). The CVR of the cases operated on within 48 h after birth was significantly larger than that of the cases operated on after 2 weeks (p = 0.001). CONCLUSION: EMG or UG lobectomy was usually required after birth in CCAM, indicating maximal CVR >2.0. By contrast, elective surgery was performed in most BA cases. LEVEL OF EVIDENCE: IV.

An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.

BACKGROUND/PURPOSE: Differences in clinical features between congenital pulmonary airway malformation (CPAM) and bronchial atresia (BA) have not yet been clearly described. METHODS: We retrospectively reviewed 112 patients with a pathological diagnosis of CPAM or BA. The clinical parameters were statistically analyzed between these diseases. RESULTS: Seventy-one patients received prenatal diagnosis and 41 received postnatal diagnosis. The percentage of prenatal diagnosis was significantly higher in CPAM patients (84% vs 50%, p < 0.001). Among patients with prenatal diagnosis, the backgrounds were not different between the two diseases except for the number of Caesarean sections (81% vs 9%, p < 0.0001). The numbers of patients that underwent fetal interventions and emergent neonatal surgery were higher in CPAM (51% vs 15%, p < 0.01 and 76% vs 12%, p < 0.0001), although there was no statistical difference in survival rate (86% vs 97%, p = 0.2). In patients receiving postnatal diagnosis, pneumonia was the primary symptom in most BA patients, whereas respiratory distress was the major symptom in patients with CPAM. Age at presentation of the primary symptom was significantly older in BA patients (4.2 years vs 1.2 years, p < 0.005). CONCLUSION: CPAM and BA have distinct clinical features in terms of therapeutic and natural history. Careful imaging evaluation and pathological analysis can lead to an accurate diagnosis of BA. TYPE OF STUDY: Prognostic study. LEVEL OF EVIDENCE: Level II. This study is categorized as a "Prognostic Study" with LEVEL III of Evidence.

Congenital right bronchial stenosis with high bifurcation: Successful management with side to side tracheobronchial anastomosis.

Congenital bronchial stenosis is a rare entity [1,2]. While there are some reports of congenital bronchial anomalies and their repair in the thoracic literature this is first report in the literature of a bronchial take off just below the larynx managed with surgical success. Here we present a case of a former 33-weeker born with a tracheoesophageal fistula, Tetralogy of Fallot, and a high bifurcation of the right mainstem bronchus with a concomitant long segment bronchial stenosis. Preoperative planning included printing 3-D reconstructed models of the airway to analyze various treatment options. At 3 months of age the patient was taken to the operating room for surgical repair of the Tetralogy of Fallot and side-to-side tracheobroncheoplasty. The patient was extubated on postoperative day 3 and was discharged home without need for any ventilatory support.

Surgery for congenital bronchial atresia.

A 22-year-old woman presented with a feeling of chest tightness. Chest computed tomography showed a dendritic shadow in the left segment 1 + 2c, surrounded by hyperlucent emphysematous changes. Bronchoscopy revealed loss of the orifice of bronchus 1 + 2c. Thus a diagnosis of bronchial atresia was made, and a left upper division segmentectomy was performed. Bronchial atresia should be considered in the differential diagnosis of young patients with an abnormal chest shadow. It can be diagnosed based on the characteristic imaging and bronchoscopic findings. Surgery is indicated for symptomatic bronchial atresia and can provide complete amelioration of the condition.

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Congenital right intermediate bronchial stenosis with carina trifurcation: successful management with slide tracheobronchial plasty.

Congenital bronchial stenosis is rarely described and is difficult to manage. Here we report two cases of right intermediate bronchial stenosis (stenotic orifice with complete cartilage rings). Both cases were associated with ventricular septal defects, and a "trifurcation" pattern was found in both carinas. Both patients underwent surgical repair of the ventricular septal defects but could not be separated from ventilator support despite successful cardiac operations. Slide tracheobronchial plasty was applied to the right intermediate bronchus and lower trachea. After correction of the bronchial stenosis, both patients could be extubated and live without supplementary ventilation support.

Estenosis bronquial congénita, papel de las imágenes en el diagnóstico: reporte de un caso / Congenital bronchial stenosis, the role of imaging in the diagnosis: a case report

En este artículo se reporta el caso de un lactante menor de 2 meses de edad que a los 15 días de nacido presentó estridor y dificultad respiratoria, por lo que consultó a una clínica de primer nivel en donde se le realizó radiografía de tórax, en la cual se documentó la presencia de una hiperinfiación del campo pulmonar derecho. Se realizó una fibrobroncoscopía óptica (FBO) que fue reportada como normal y una gammagrafía de ventilación/perfusión que reportó una hipoplasia pulmonar derecha. Fue manejado con oxígeno suplementario y terapias respiratorias sin mejoría clínica, motivo por el cual fue trasladado a nuestra institución para manejo especializado. Con el fin de obtener un mapa vascular pre quirúrgico, se realizó una angiografía pulmonar por tomografía (Figura 2) reconstrucciones con mínima intensidad de proyección (Figura 3) y broncoscopía virtual (BV) multicorte (Figuras 4 y 5) documentándose una estenosis del bronquio fuente derecho, producida por un repliegue de la pared bronquial y con efecto de válvula que producía una hiperinsufiación secundaria del pulmón derecho. Estos hallazgos fueron confirmados en la cirugía correctiva.

This article describes the case of an infant under 2 months old, who at 15 days of age presented stridor and respiratory distress, a chest X-ray was performed which reported the presence of a hyperinflation of the right lung field. A fiberoptic bronchoscopy (FBO) was performed which resulted normal and a ventilation/perfusion gammagraph showed a right lung hypoplasia. The infant was treated with additional oxygen and respiratory therapy without clinical. In order to obtain a pre-surgical vascular map, a multislice CT angiography and virtual bronchoscopy were performed, documenting a right bronchial stenosis produced by a fold of the bronchial wall and with valve effect which produced a secondary hyperinsufflation of the right lung. These findings were confirmed in corrective surgery.

Modified slide tracheoplasty in a newborn with bronchial and carinal stenosis.

Congenital tracheal stenosis (CTS) is a life-threatening condition that is associated with significant morbidity and mortality particularly when symptomatic presentation occurs in the newborn period. The most challenging form of CTS is long segment congenital tracheal stenosis (LSCTS) with compromise of the carina and main stem bronchi. We report the case of a newborn with severe distal tracheal stenosis with carinal and main stem bronchial involvement who was managed successfully with a modified slide and autologous rib graft tracheoplasty. The patient was discharged from hospital without ventilator support or oxygen requirement at 2 months of age. The details of this case and the description of the surgical procedure are presented and the related literature is reviewed.