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Background Children with neuromuscular diseases (NMDs) often display respiratory muscle weakness which increases the risk of postoperative pulmonary complications (PPCs) after general anaesthesia. Non-invasive ventilation (NIV) associated with mechanical insufflation-exsufflation (MI-E) can reduce the incidence and severity of PPCs. The aim of this study was to report our experience with a shared perioperative protocol that consists in using NIV combined with MI-E to improve the postoperative outcome of NMD children (IT-NEUMA-Ped). Method We conducted a multicentre, observational study on 167 consecutive paediatric patients with NMDs undergoing anaesthesia from December 2015 to December 2018 in a network of 13 Italian hospitals. Results We found that 89% of the 167 children (mean age 8 years old) were at high risk of PPCs, due to the presence of at least one respiratory risk factor. In particular, 51% of them had preoperative ventilatory support dependence. Only 14 (8%) patients developed PPCs, and only two patients needed tracheostomy. Average hospital length of stay (LOS) was 6 (2-14) days. The study population was stratified according to preoperative respiratory devices dependency and invasiveness of the procedure. Patients with preoperative ventilatory support dependence showed significantly higher intensive care unit (ICU) admission rate and longer hospital LOS. Conclusion Disease severity seems to be more related to the outcome of this population than invasiveness of procedures. NIV combined with MI-E can help in preventing and resolve PPCs.

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.

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.

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.

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.

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.

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 Yshaped), 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.

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.

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.

Narrowing of the left mainstem bronchus(LMSB)may produce severe respiratory symptoms andairway stenting may represent a conservative treatment. We described 7 cases(3 boys,median age:0.5yrs, age range:0-13yrs)in which a left obstruction was treated with metallic balloon expandable( J e OSTENT,Abbott ®)and only a follow-up permitted to manage stent-related potentially fatal complications with low risk interventions. After a median time of 2.5 yrs(range:0.25-5yrs)from the stenting, all children showed major complications(3 complete ovalization, 3 breakage and 1 haemoptysis)due to a novel close proximity or collision to adjacent vessels. After a CTA scan showing vascular collision, 2 of the children with stent ovalization were treated with a posterior aortopexis(PA), while a watchful approach was planned in the third child. One of the children with stent breakage was submitted to PA, while a silicone stent(Dumon, Novatech) overlapping the metallic one was chosen for the second and a surgical expansion of the chest has been scheduled for the third patient who was affected by jeune syndrome. Tranexamic acid instillation together with a posterior aortopexis was successfully carried on in the child with haemoptysis. The presence of satisfactory anatomical relationships at the time of stent placement in LMSB is not by itself a guarantee of the final device. Only a radiological study before and eventually after stenting and a close follow-up by bronchoscopy can highlight in real time potentially dangerous complications secondary to the well known “mass effect”. The use of a permanent device must take account that clinical sequelae may become catastrophic.

Objectives We reviewed the role of posterior aortopexy for left mainstem bronchus compression in infants and children. Methods Eighteen children with respiratory symptoms were enrolled between 2005 and 2015 for surgical decompression of the left mainstem bronchus. The children were managed from diagnosis to follow-up by a dedicated tracheal team. Primary outcomes were the complete relief of symptoms or improvement with respect to preoperative clinical status. Results The median age was 4 years (0.3-15.4) and the median weight was 13.2 kg (3, 1-40). Symptoms or indications for bronchoscopy included difficult weaning from mechanical ventilation (n = 3, 17%), difficult weaning from tracheotomy (n = 4, 22%), recurrent pneumonia (n = 4, 22%), wheezing (n = 3, 17%), atelectasis (n = 1, 5.5%), bitonal cough (n = 1, 5.5%) and stridor (n = 2, 11%). Associated malformations were present in 88.7%. The diagnosis was made by bronchoscopy and computed tomography. Indication for surgery was the presence of pulsations and reduction in the diameter of the left mainstem bronchus compression of more than 70%. Surgery was performed by left posterolateral thoracotomy. Aortopexy was done under bronchoscopic control. No early or late deaths were observed, nor were reoperations necessary. Residual malacia was observed in 8 children (44%). Median follow-up was 4.1 years (0.1-7.1). At last follow-up, 17/18 (94.4%) children showed adequate airway patency. Conclusions The intrathoracic location of the left mainstem bronchus predisposes it to compression. Vascular anomalies represent the most frequent causes. Aortopexy has been advocated as a safe and useful method to relieve the compression, and our results confirmed these findings. Management of these patients is challenging and requires a multidisciplinary team.

Objectives To analyze the outcome of a new endoscopic approach for the treatment of pediatric subglottic stenosis. Study design Case series. Setting Tertiary care center. Material and methods Eighteen pediatric cases of grade II to IV subglottic stenosis (8 congenital and 10 acquired) consecutively treated at our institutions by Endoscopic Anterior Cricoid Split (EACS) and balloon dilation between 2006 and 2010. Treatment protocol encompassed systematic postoperative laryngeal stenting (7 days of intubation or 1 month of Montgomery T-tube in previously tracheotomized patients) and endoscopic controls with possible additional balloon dilation every 15 days for at least 2 months. Results Patients’ ages ranged from 1 to 101 months. Postoperative follow-up ranged from 4 to 45 months (median value ± SD: 15.3 ± 11.9). The mean duration of the endoscopic procedure was 35.2 ± 13.2 min. The number of days spent in PICU during the perioperative period varied between 2 and 15. Four patients (22.2%) needed one and 14 patients (77.7%) required several (from 4 to 7) additional balloon dilations during the postoperative endoscopic controls. No incident was observed during or immediately after EACS. Treatment was efficient in 83% of cases (n = 15), with no residual respiratory symptoms and grade 0 to 1 SGS at the end of follow-up. Conclusion EACS is a safe and efficient technique to treat pediatric subglottic stenosis, regardless of their grade and length, provided to associate it with postoperative laryngeal stenting and regular endoscopic follow-up with possible additional balloon dilations. In our teams, it has become the first line treatment for most grades II to IV SGS. Its indications can be extended to congenital stenosis with cartilaginous involvement and to long-lasting acquired stenosis with firm fibrosis.

1Department of Emergency, Pediatric Intensive Care Unit, Catholic University of the Sacred Heart, Rome, Italy 2Division of Pediatric Surgery, Department of Pediatrics, “S. Camillo-Forlanini” Hospital, Rome, Italy 3Division of Neonatology, Department of Pediatrics, Catholic University of the Sacred Heart, Rome, Italy 4Division of Neonatology, Department of Pediatrics, “S. Camillo-Forlanini” Hospital, Rome, Italy 5Department of Intensive Care, “A. Meyer” Children Hospital, Florence, Italy

L’endoscopia pediatrica ha acquisito un ruolo diagnostico- terapeutico ufficiale solo negli anni ’70, grazie alla disponibilita di strumenti adeguatamente miniaturizzati e sofisticati. Oltre alla disponibilita delle apparecchiature, questo approccio strumentale richiede specifica esperienza da parte dell’endoscopista e un adeguato supporto anestesiologico- rianimatorio intra e postoperatorio. Com’e noto, le vie aeree del paziente pediatrico presentano peculiarita anatomo-funzionali, particolarmente evidenti nel neonato, soprattutto prematuro; pertanto gli approcci diagnostici e strumentali endoscopici risultano di grande impegno e rischio e le indicazioni vanno opportunamente selezionate.

Il termine stent deriva da Charles B. Stent, dentista britannico vissuto alla fine del XIX secolo. Stent crO un materiale che originariamente veniva utilizzato per le impronte dentali e, piu tardi, per supportare gli innesti cutanei. Il primo stent fu inserito nell’albero tracheobronchiale da Brunings ed Albrecht [1], mentre la prima protesi metallica (in argento) riportata in letteratura fu utilizzata da Canfield e Norton in un bambino di due anni con una stenosi laringotracheale [2]. Al giorno d’oggi, con il termine stent si intende il materiale utilizzato per impedire ad alcune strutture di collassare. In particular modo, gli stents rappresentano dei devices in grado di mantenere pervio il lume di organi tubulari. e stato Montgomery, nel 1965, a descrivere per primo l’uso di protesi in silicone per lo stenting tracheale nell’adulto (Fig. 1).

Per la comprensione delle patologie che interessano le vie aeree in eta pediatrica, spesso di natura malformativa, ci e sembrato opportuno ricordare brevemente alcune note embriologiche sulla formazione del’apparato respiratorio.

In questi ultimi anni si e assistito ad un notevole aumento del numero di endoscopie respiratorie eseguite in eta pediatrica presso centri specializzati.

Per tracheomalacia (TM) o discinesia tracheale s’intende un indebolimento congenito o acquisito della parete tracheale che collabisce con gli atti del respiro. La trachea malacica presenta sia una debolezza intrinseca degli anelli cartilaginei che appaiono meno consistenti e plicabili, sia un ridotto tono muscolare della pars membranacea, che sono causa del suo collasso durante il ciclo respiratorio.

OBJECTIVE: Acute renal failure is common in septic patients. Fenoldopam, a dopamine-1 receptor agonist, increases renal blood flow and may, therefore, reduce the risk of acute renal failure in such patients. Accordingly, we sought to determine the safety and efficacy of fenoldopam for the prevention of acute renal failure in septic patients. DESIGN: Prospective, double-blind, placebo-controlled trial. SETTING: Three multidisciplinary intensive care units at a university hospital. PATIENTS: Three hundred septic patients with baseline serum creatinine concentrations 150 micromol/L, during study drug infusion. MEASUREMENTS AND MAIN RESULTS: The incidence of acute renal failure was significantly lower in the fenoldopam group compared with the control group (29 vs. 51 patients; p = .006). The odds ratio of developing acute renal failure for patients treated with fenoldopam was estimated to be 0.47 (p = .005). The difference in the incidence of severe acute renal failure (creatinine >300 mumol/L), however, failed to achieve statistical significance (10 vs. 21; p = .056). The length of intensive care unit stay in surviving patients was significantly lower in the fenoldopam group compared with the control group (10.64 +/- 9.3 vs. 13.4 +/- 14.0; p < .001). There were no complications of fenoldopam infusion. A direct effect of treatment on the probability of death, beyond its effect on acute renal failure, was not significant (odds ratio = 0.68, p = .1). CONCLUSIONS: Compared with placebo, low-dose fenoldopam resulted in a smaller increase in serum creatinine in septic patients. The clinical significance of this finding is uncertain. A large multiple-center trial is now needed to confirm these findings.

Cranio-orbital fibrous dysplasia usually causes proptosis, visual loss, ocular motility impairment and cosmetic deformity. The goals of cranial fibrous dysplasia management are to preserve neurological functions and improve cosmesis. Strict neurological and ophthalmological follow-up and early cranio-facial surgery enables non-aggressive treatment without significant morbidity. Furthermore, good results are achieved thanks to new techniques and materials especially in children and adolescents. The aim of the present paper is to discuss surgical indications, techniques and outcomes of cranio-facial-orbital fibrous dysplasia on the basis of personal experience of 14 patients who underwent surgery.