Paola Serio

Tracheobronchial obstruction: follow-up study of 100 children treated with airway stenting

OBJECTIVE We described a cohort of 100 children with a wide variety of airway obstruction who underwent stent positioning in the last 7 years. The study examined the outcomes of this treatment in the largest series of paediatric patients reported in the literature with special concern over safety and clinical effectiveness. METHODS We performed a retrospective analysis of 100 consecutive paediatric patients who underwent stent insertions between January 2005 and May 2012. Statistical analysis was performed and exact likelihood was used. RESULTS A total of 235 stents were placed for severe airway obstruction. One hundred and twelve silicone stents (cylinder, hourglass or Y-shaped), 120 metallic stents (covered Nitinol stents, expandable coronary and vascular stents) and 3 biodegradable polydioxanone (PDS) stents were used. Eighty patients presented clinical improvement after stent insertion, 17 were weaned off mechanical ventilation and 3 showed no significant clinical improvement [95% confidence interval (CI) 0.1-8.0%]. Complications were different according to stent type. In our cohort, no fatal stent-related complications have been observed. At follow-up (median 41.4 months, range 1.1-145.4) complete resolution was registered for 60 patients (66%; 95% CI 55-76%), 17 are still under treatment, 9 were lost to follow-up, 8 underwent surgery and 6 died of causes not stent related. CONCLUSION Airway stenting represents a conservative treatment before more invasive surgical procedures and can be very effective when performed in selected children and in specialized centres by physicians experienced in rigid and flexible bronchoscopy.

Residual tracheobronchial malacia after surgery for vascular compression in children: treatment with stenting†.

OBJECTIVES: Surgery for vascular anomalies can occasionally fail to relieve symptoms, especially when severe tracheobronchial malacia persists. We studied outcomes in children who underwent airway stenting for severe post-surgical airway malacia and tested known clinical and surgical prognostic factors. METHODS: Among 257 children evaluated for tracheobronchial vascular compression, we reviewed the clinical charts for the 59 patients (23%) who underwent surgery. After surgery, children in whom severe malacia and respiratory symptoms persisted underwent airway stenting. RESULTS: Among the 59 patients (boys: 58.1%, median age: 6.4 months, age range: 0.1–182.8 months), 79.7% had major comorbidities, 39% additional upper airway anomalies and 15.2% abnormal bronchial anatomy. Diagnostic imaging identified seven vascular anomalies: innominate artery compression 27.1%, left bronchial compression from the descending aorta and left pulmonary artery 20.3%, lesions associated with right aortic arch 22.0%, double aortic arch 13.6%, bovine arch type 10.2%, aberrant right subclavian artery 5.1% and pulmonary artery sling 1.7%. For severe residual malacia and severe persistent respiratory symptoms, 20 patients (33.9%) had silicone or metallic stents inserted. The most frequent indication for stenting was double aortic arch (P = 0.02 by chi-square test). A clinical prognostic factor for permanent stenting was left bronchial compression from the descending aorta and left pulmonary artery [odds ratio (OR): 14.667, 95% confidence interval (CI): 2.881–74.659], particularly if associated with congenital heart disease (OR: 30.00, 95% CI: 4.349– 206.927). All silicone stents but one were successfully removed; metallic uncovered stents were patent and completely re-epithelialized. CONCLUSIONS: When surgery leaves severe airway obstruction and respiratory symptoms unchanged, children with tracheobronchial malacia can safely undergo airway stenting.

Tracheal surgery in children: outcome of a 12-year survey

OBJECTIVES Despite the fact that team management has improved the results in recent years, perioperative deaths and complications remain high in paediatric tracheal surgery. We reviewed our institutional experience by comparing our results with those in the literature. METHODS Between 2005 and 2017, 30 children underwent surgery for tracheal disease. Fifteen were boys and fifteen were girls (50% vs 50%). The median age at operation was 7 months (15 days-9.6 years), and the median weight was 5.2 kg (2.8-34 kg). Congenital tracheal stenosis was diagnosed in 25 children (83.3%), and 5 (16.7%) had acquired lesions. The mean internal diameter in congenital tracheal stenosis was 1.5 mm, with complete tracheal rings present in all patients. Associated malformations were bronchopulmonary in 11 cases (36.7%) and cardiovascular in 16 (53.3%). RESULTS No in-hospital deaths occurred in our data set. Overall mortality was 4 of 30 cases (13.3%). Twenty-four endoscopic reinterventions were required in 19 children (63%) and consisted of stent positioning in 13 (43.3%), balloon dilatation in 5 (16.7%), granulation removal in 4 (13.3%) and tracheostomy in 2 (6.7%). Of the survivors (26 of 30, 86.7%), 11 children (42.3%) did not require further examination on adequate tracheal diameter for age and absence of symptoms after a median follow-up period of 3.5 years. CONCLUSIONS The result of paediatric tracheal surgery depends on several factors. The number of cases treated at a particular centre is an important one, but our experience, although limited, can be compared with that at centres with a higher volume of cases. We emphasize the need for applying a multidisciplinary approach to master the surgical command of different reconstructive tracheal procedures, to manage associated defects, particularly cardiovascular defects, and to manage complications under endoscopic guidance. These can be considered the mainstays of building a successful tracheal programme.

Outcome of long-term complications after permanent metallic left bronchial stenting in children

OBJECTIVES We describe the way we treated 7 children with critical long-term complications after metallic balloon-expandable stenting in the left mainstem bronchus. METHODS Endoscopic follow-up included a first bronchoscopy 3 weeks after stenting, then monthly for 3 months, every 4-6 months up to 1 year and at scheduled times to calibrate stent diameter up to final calibration. When major complications occurred, patients underwent chest computed tomographic angiography. RESULTS In 1 of the 7 children (median age 2.8 years), metallic left bronchial stenting served as a bridge to surgery. After a median 4-year follow-up, all 7 children experienced recurrent stent ovalizations with stent breakage in 3 and erosion in 1. In 4 children, computed tomographic angiography showed abundant peribronchial fibrous tissue, in 2 left mediastinal rotation and in 1 displacement along the left bronchus after pulmonary re-expansion as the cause of stent-related complication. Of the 7 children, 6 underwent surgery (5 posterior aortopexy and 1 section of the ligamentum arteriosus) and 3 required nitinol stents placement within the metallic ones. One patient completed the follow-up, and 1 patient was lost to follow-up. All 5 remaining children still have permanent bronchial stents in place, patent and re-epithelialized after a median 10.5-year follow-up. There were no deaths. CONCLUSIONS Satisfactory anatomical relationships when children have stents placed in the left mainstem bronchus alone do not guarantee the final success. Several mechanisms intervene to cause critical stent-related complications in children during growth. Permanent metallic stents should be used carefully, and only in selected patients.

Airway stenting in a child with spondyloepiphyseal dysplasia congenita: 13-Year survival

We describe the case of a boy with spondyloepiphyseal dysplasia congenita. At birth, he experienced severe respiratory distress necessitating tracheotomy. Endoscopy done because mechanical ventilation failed to resolve desaturations disclosed severe tracheo-bronchomalacia. A Polyflex silicone stent was placed in the trachea (replaced by Y-Dumon stent) and 2 Palmaz metallic stents in the mainstem bronchi (overlapped with 2 Jomed stents 5 years later). Airway stenting guaranteed a suitable respiratory status and allowed a child who was expected to die at birth, to reach 13.5 years old in good conditions.

ERS statement: interventional bronchoscopy in children

Paediatric airway endoscopy is accepted as a diagnostic and therapeutic procedure, with an expanding number of indications and applications in children. The aim of this European Respiratory Society task force was to produce a statement on interventional bronchoscopy in children, describing the evidence available at present and current clinical practice, and identifying areas deserving further investigation. The multidisciplinary task force panel performed a systematic review of the literature, focusing on whole lung lavage, transbronchial and endobronchial biopsy, transbronchial needle aspiration with endobronchial ultrasound, foreign body extraction, balloon dilation and occlusion, laser-assisted procedures, usage of airway stents, microdebriders, cryotherapy, endoscopic intubation, application of drugs and other liquids, and caregiver perspectives. There is a scarcity of published evidence in this field, and in many cases the task force had to resort to the collective clinical experience of the committee to develop this statement. The highlighted gaps in knowledge underline the need for further research and serve as a call to paediatric bronchoscopists to work together in multicentre collaborations, for the benefit of children with airway disorders. Interventional bronchoscopy in children: evidence at present, clinical practice and areas for further investigation http://ow.ly/Z7Rv30fuPV6

Double Tracheoesophageal Fistula and Azygos Lobe

Figure 1 Chest computed tomography image showing the azygos fissure (AF) outlining the azygos lobe (AL). On Day 3 of life, a term female neonate, whose mother had gone through normal pregnancy and delivery, developed dyspnea and cyanosis during feeding. Chest X-ray demonstrated infiltration in the right upper lobe. Aspiration pneumonia was diagnosed, then antibiotic therapy was initiated, and enteral feeding was discontinued. After the feeding bottle was reintroduced, regurgitation, cyanosis, and coughing reappeared. Esophageal pH monitoring excluded gastroesophageal reflux and video-fluorographic swallowing study revealed no motor alterations. However, after recurrent episodes of aspiration pneumonia, a computed tomography (CT) scan was performed. CT revealed several right lung pulmonary opacities and a distinguished azygos lobe (Figure 1). To rule out the possibility of tracheal abnormality, a flexible bronchoscopy was performed and no pathology was found. Subsequent rigid bronchoscopy (Karl Storz, Tuttlingen, Germany; Ø 3.0) with a Hopkins Forward-Oblique Telescope 30 and positive pressure ventilation under general anesthesia detected two isolated H-type tracheoesophageal fistulas (TEFs) in the cervical trachea (Figure 2).

Airway stenting as a salvage procedure in a child with a lethal spondyloepiphyseal dysplasia congenita: 13 years follow-up

We describe the case of a term infant with broad and protruding forehead, micrognathia, wide cleft palate, short neck and stocky body with small thoracic cage, short limbs, bilateral clubfeet and triventricular hydrocephalus. A G313S mutation in the gene for type II-collagen(COL2A1)was demonstrated. Soon after birth, the infant had respiratory distress with repeated obstructive apnea, leading to nasotracheal intubation. Despite mechanical ventilation(MV)with high positive pressure, he showed desaturations, leading to tracheotomy. An endoscopy showed severe tracheo-bronchomalacia. At 5 months a polyflex self-expanding silicone stent was placed in the trachea(10x30, replaced by12x20 and removed after 4 years), with two stainless steel stents in the mainstem bronchi(Palmaz 104right and Palmaz 128left). After 5 years 2 stents were overlapped to the previous in each of the mainstem bronchus(2 Jomed 12-10/12). At 10.5 years a Y Dumon stent(11-7-7) has been placed in the trachea and removed after 2 years. To date he is a 13 year old child, he has a tracheotomy(4.5mm cuffed Shiley with speech valve)and occasionally needs of night ventilatory support. He attends the secondary school with fairly good performance. G313S mutation has been described in a lethal case with respiratory insufficiency at birth, with spondyloepiphyseal dysplasia congenita. In our case, the airway malacia has been promptly treated by stenting with immediate improvement of the airway ventilation. Stenting, although is a palliative treatment given the cage narrowness, has allowed a child who was expected to die at birth, to reach adolescence in good condition and with a certain degree of autonomy.

The role of endoscopy in the treatment of IV type laryngotracheal cleft complications after surgical repair

Laryngotracheal cleft (LTC) is a rare congenital anomaly. Severe forms of LTC are usually treated surgically using extracorporeal circulation. Despite tremendous improvement of surgical techniques, postoperative complications are still frequent. We report a comprehensive description of an endoscopic approach to diagnosis, surgical repair and endoscopic treatment of complications after correction in a type IV LTC.

ALCAPA and massive pulmonary atelectasis: How a stent in the airway can be life-saving

Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital anomaly in which left coronary artery arises from the pulmonary artery resulting in progressive myocardial ischemia and dysfunction of the left ventricle. We report a case of ALCAPA with severe cardiac and respiratory failure and huge heart dilation compressing the left main bronchus and preventing from an effective ventilation. Emergency bronchial stenting allowed to improve left lung atelectasis, reduce pulmonary hypertension, resume anterograde left coronary artery perfusion and stabilize cardiovascular conditions to undertake a successful surgical correction.