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.

Isolated left hypoplasia of the pulmonary artery accompanied by lung hypoplasia and chronic bronchial disease in a cat.

A 2-year-old castrated Russian Blue cat presented with inappetence, depression, and labored respirations. Radiography findings suggested left lung atelectasis; however, the ultrasonography findings did not indicate lung atelectasis. The left pulmonary artery (PA) was abnormally small on echocardiography; further, there were no other cardiac anomalies. Computed tomography revealed an abnormally small left PA and left lung. Furthermore, bronchiectasis and tree-in-bud patterns were observed in the lung lobes. Based on these findings, the cat was diagnosed with isolated left PA hypoplasia, presumed left lung hypoplasia, and feline chronic bronchial disease. Early diagnosis of this disease is important because it can cause serious complications, including recurrent respiratory infection, bronchiectasis, massive hemoptysis, and pulmonary hypertension.

Failure of epidural analgesia converted to epidural anesthesia for cesarean delivery in a patient with bronchial atresia in labor.

Bronchial atresia is a rare anomaly characterized generally by obstruction in the bronchial system, mucus accumulation, emphysematous changes and bulla formation in the peripheral lung. Regional anesthesia is the choice for cesarean delivery in a parturient patient with this anomaly. We report a patient with a diagnosis of bronchial atresia in whom the conversion of epidural analgesia to epidural anesthesia for cesarean delivery failed during labor, needing the application of general anesthesia for a successful delivery.

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.

Hypoplastic left heart syndrome associated with large omphalocele and hypoplastic left bronchus.

We present a patient with the combination of hypoplastic left heart syndrome (HLHS), large omphalocele, and hypoplastic left bronchus who underwent a modified Norwood procedure and was successfully discharged home. The child required tracheostomy with home ventilation and we elected to delay the primary repair of the omphalocele. To our knowledge, this is the first reported case with this combination of anomalies surviving till hospital discharge. Our experience demonstrates the potential viability of patients with concurrent HLHS, large abdominal wall defects, and airway anomalies and may additionally advocate for delayed repair of large omphaloceles in patients with complex congenital heart disease.

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.

Anomalous ascending aorta causing severe compression of the left bronchus in an infant with ventricular septal defect and pulmonary atresia.

Tracheobronchial compression by cardiovascular structures complicates the course after surgery of congenital heart disease. A 2-month-old boy with ventricular septal defect, pulmonary atresia, and velocardiofacial syndrome had severe left main bronchus obstruction due to external compression by an anomalously oriented ascending aorta. The patient's hypoxemic episodes necessitated extracorporeal membrane oxygenation. We inserted a stent in the left bronchus to open the airway, but the stent was crushed by the anomalous aorta. We later surgically rerouted the aorta and finally restored the patency of the left main bronchus. However, the patient died of fungemia, without being weaned from extracorporeal membrane oxygenation. We conclude that surgery is necessary instead of stent implantation to relieve the external compression of the left bronchus from a vessel with systemic arterial pressure.

Two further cases supporting nonsurgical management in congenital lobar emphysema.

BACKGROUND: For decades, the well-established standard recommended treatment for patients with congenital lobar emphysema (CLE) and respiratory distress has been lobectomy of the affected lobe or lobes, whereas indications for conservative management have been controversially discussed. PATIENTS/METHODS: Description of the clinical courses including the results of diagnostic procedures and the resulting therapeutic strategies in 2 patients with congenital lobar emphysema. We review the literature on conservatively treated patients with congenital lobar emphysema. RESULTS: Considering that formerly asserted hypotheses postulating benefits of surgical treatment cannot unambiguously be corroborated from cases in literature, we could show that conservative treatment in patients with congenital lobar emphysema is appropriate in mildly to moderately symptomatic children. CONCLUSION: Conservative treatment of children with congenital lobar emphysema is an attractive option, whenever justifiable on medical grounds. Our cases may serve as paradigms in decision-making processes in similar cases and - together with the literature review - may be helpful to avoid unnecessary lobectomies in children. Patients treated conservatively will need a close follow-up, and further data on long-term follow-up courses are desirable.

Right tracheal bronchus in an infant with congenital diaphragmatic hernia.

Neonates with congenital diaphragmatic hernia (CDH) often have other major malformations. Anatomic airway anomalies associated with CDH include congenital stenosis, pulmonary hypoplasia, and abnormal bronchial branching. We describe an infant with CDH who, after developing recurrent upper-lobe atelectasis, was found to have a right tracheal bronchus. This clinically significant anomaly can have important and potentially preventable adverse consequences.

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.

The rare association of truncus arteriosus with a cervical double aortic arch presenting with left main bronchial compression.

Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4th embryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.

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.

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.

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.

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.

Tracheobronchial stenting in an infant with an anomalous right main bronchus.

Congenital anomalies of the right main bronchi have not been fully described. Bronchial anomalies are usually asymptomatic unless they coexist with other abnormalities. We describe a rare bronchial anomaly in a 2-month-old girl in which the right upper lobe, middle lobe, and lower lobe bronchi all originated at the same point as a result of a developmental teratogenic long right main bronchus and an absent bronchus intermedius. Furthermore, the left main bronchus contained a proximal segment of stenosis. This combination of anomalies, along with right upper lobe atelectasis and left-sided hyperinflation, resulted in a severe right mediastinal shift and respiratory distress. The mediastinum was returned to midline with endoscopic placement of an in-dwelling tracheobronchial stent into the left main bronchus. To the best of our knowledge, the specific developmental anomaly in our patient has not been previously described.