Combined Surgical and Endoscopic Approach for Ring-Sling Complex.

BACKGROUND: Left pulmonary artery (LPA) sling (PAS) is a vascular ring, which is frequently associated with long-segment tracheal stenosis (TS). Mortality rate in operated children is still high, especially in cases of severe tracheal hypoplasia and/or associated congenital heart defects (CHDs). We report our experience of treatment and follow-up in a pediatric cohort of patients affected by PAS with severe tracheobronchial involvement. METHODS: From 2005 to 2017, we enrolled 11 children diagnosed with PAS and congenital TS requiring surgical intervention. Echocardiography, computed tomography, and bronchoscopy were performed in all patients. Associated CHD were present in 5 (45%) patients. Tracheal reconstruction techniques included slide tracheoplasty (7/11; 63%), slide tracheoplasty and costal cartilage graft (2/11; 18%), and Hazekamp technique (2/11; 18%).Nine patients underwent LPA direct reimplantation and concomitant tracheoplasty; concomitant surgical repair for CHD was performed in three children. RESULTS: Over a mean follow-up of 30 months (range: 3-75 months), a late mortality of 18% was registered; no early death occurred. Good flow through LPA could be documented in all patients. Ten children required operative bronchoscopies (mean: 16/patients) aimed at stent positioning/removal, treatment of granulomas, and tracheobronchial dilatation. CONCLUSIONS: Severe tracheobronchial stenosis and associated CHD were the main determinants for hospitalization time, intensive assistance, and repeated endoscopic procedures.Patients affected by PAS/TS complex require a careful management at high-specialized centers providing multidisciplinary team.Respiratory endoscopy may play a central role both in preoperatory assessment and in postoperative management of patients showing severe tracheobronchial involvement.

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.

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.

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.

The role of posterior aortopexy in the treatment of left mainstem bronchus compression.

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.

Efficacy of ketamine in refractory convulsive status epilepticus in children: a protocol for a sequential design, multicentre, randomised, controlled, open-label, non-profit trial (KETASER01).

INTRODUCTION: Status epilepticus (SE) is a life-threatening neurological emergency. SE lasting longer than 120 min and not responding to first-line and second-line antiepileptic drugs is defined as 'refractory' (RCSE) and requires intensive care unit treatment. There is currently neither evidence nor consensus to guide either the optimal choice of therapy or treatment goals for RCSE, which is generally treated with coma induction using conventional anaesthetics (high dose midazolam, thiopental and/or propofol). Increasing evidence indicates that ketamine (KE), a strong N-methyl-d-aspartate glutamate receptor antagonist, may be effective in treating RCSE. We hypothesised that intravenous KE is more efficacious and safer than conventional anaesthetics in treating RCSE. METHODS AND ANALYSIS: A multicentre, randomised, controlled, open-label, non-profit, sequentially designed study will be conducted to assess the efficacy of KE compared with conventional anaesthetics in the treatment of RCSE in children. 10 Italian centres/hospitals are involved in enrolling 57 patients aged 1 month to 18 years with RCSE. Primary outcome is the resolution of SE up to 24 hours after withdrawal of therapy and is updated for each patient treated according to the sequential method. ETHICS AND DISSEMINATION: The study received ethical approval from the Tuscan Paediatric Ethics Committee (12/2015). The results of this study will be published in peer-reviewed journals and presented at international conferences. TRIAL REGISTRATION NUMBER: NCT02431663; Pre-results.

Ketamine in refractory convulsive status epilepticus in children avoids endotracheal intubation.

OBJECTIVE: The purpose of this study was to report on the efficacy and safety of intravenous ketamine (KE) in refractory convulsive status epilepticus (RCSE) in children and highlight its advantages with particular reference to avoiding endotracheal intubation. METHODS: Since November 2009, we have used a protocol to treat RCSE including intravenous KE in all patients referred to the Neurology Unit of the Meyer Children's Hospital. RESULTS: From November 2009 to February 2015, 13 children (7 females; age: 2 months-11 years and 5 months) received KE. Eight patients were treated once, two were treated twice, and the remaining three were treated 3 times during different RCSE episodes, for a total of 19 treatments. Most of the RCSE episodes were generalized (14/19). A malformation of cortical development was the most frequent etiology (4/13 children). Ketamine was administered from a minimum of 22 h to a maximum of 17 days, at doses ranging from 7 to 60 mcg/kg/min, obtaining a resolution of the RCSE in 14/19 episodes. Five patients received KE in lieu of conventional anesthetics, thus, avoiding endotracheal intubation. Ketamine was effective in 4 of them. Suppression-burst pattern was observed after the initial bolus of 3mg/kg in the majority of the responder RCSE episodes (10/14). CONCLUSIONS: Ketamine is effective in treating RCSE and represents a practical alternative to conventional anesthetics for the treatment of RCSE. Its use avoids the pitfalls and dangers of endotracheal intubation, which is known to worsen RCSE prognosis. This article is part of a Special Issue entitled "Status Epilepticus".

Ground zero: not asthma at all.

Upper airway obstruction is commonly misdiagnosed as asthma. We report on four children with recurrent respiratory symptoms who had been erroneously diagnosed as having asthma and who received anti-asthma medication for several years. The evaluation of spirometry tracing was neglected in all cases. Subglottic stenosis, tracheomalacia secondary to tracheo-esophageal fistula, double aortic arch, and vocal cord dysfunction were suspected by direct inspection of the flow-volume curves and eventually diagnosed. The value of clinical history and careful evaluation of spirometry tracing in children with persistent respiratory symptoms is critically discussed.

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.

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.

Efficacy and safety of ketamine in refractory status epilepticus in children.

OBJECTIVE: To evaluate the efficacy and safety of ketamine (KE) in the management of refractory convulsive status epilepticus (RSE) in children. METHODS: In November 2009, we started using KE for treating all children consecutively referred for RSE. Clinical and treatment data were analyzed. RESULTS: Between November 2009 and June 2011, 9 children with RSE received IV KE. In 8 patients, SE had persisted for more than 24 hours (super-refractory RSE), with a median of 6 days (mean 8.5 ± 7.5; range 2-26 days). Prior to KE administration, conventional anesthetics were used, including midazolam, thiopental, and propofol in 9, 5, and 4 patients each. Median dose of KE in continuous IV infusion was 40 gamma(µg)/kg/min (mean 36.5 ± 18.6 gamma[µg]/kg/min; range 10-60 gamma[µg]/kg/min). Midazolam was administered add-on to prevent emergence reactions. The use of KE was associated with resolution of RSE in 6 children. None of the patients experienced serious adverse events. Among the 3 individuals who did not respond to KE, 2 were cured by surgical removal of epileptogenic focal cortical dysplasia. CONCLUSION: In this small, open-label, unblinded series with no concurrent control group, KE appears effective and safe in treating RSE in children. Larger, randomized studies are needed to confirm data emerging from this preliminary observation. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that IV KE can be effective in treating children with RSE (no statistical analysis was done).

Successful management of congenital bronchial stenosis using an expandable stent.

Congenital bronchial stenosis is a very rare cause of neonatal dyspnea. Surgical management remains challenging in small children. We report successful implantation of a bronchial stent in a 3-month-old female infant presenting with congenital right bronchial stenosis and 18 months of follow-up. Use of stents in children remains controversial because of the problem of size mismatch as the child grows. Nevertheless, expandable stent implantation could be an interesting alternative to complex surgery for localized bronchial stenosis in neonates.

Successful simultaneous correction of complex congenital tracheal stenosis and tetralogy of fallot.

Congenital tracheal stenosis is frequently associated with heart malformations. Simultaneous correction of both anomalies has been advocated by several authors. We describe our experience with a premature neonate with congenital tracheal stenosis and tetralogy of Fallot. The anomalies were corrected during the same surgical procedure with the aid of extracorporeal circulation. The implications of the operative and postoperative courses, concerning both the cardiac anomaly and the tracheal anomaly are discussed.

Endoscopic anterior cricoid split and balloon dilation in pediatric subglottic stenosis.

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.

Neonatal liver abscesses due to Candida infection effectively treated with caspofungin.

UNLABELLED: Candidiasis is relatively frequent in neonatal and pediatric intensive care units (ICUs), particularly in preterm infants less than 28 weeks of gestational age. Neonatal candidiasis shows high mortality and is often associated to poor neurodevelopmental prognosis in survivor patients. Amphotericin B and fluconazole are the first choice drugs for the treatment of neonatal candidiasis. Caspofungin is an alternative antifungal agent, which is recommended for invasive candidiasis in adults, but has been poorly experienced in neonates and infants as far as now. We report the first two infants with Candida liver abscesses treated with caspofungin. In the first infant bloodstream and liver lesions were cleared by combination therapy with fluconazole, liposomal amphotericin and caspofungin, while in the second one by caspofungin alone. CONCLUSION: Our observations confirm the efficacy and tolerability of caspofungin in the treatment of neonatal candidiasis refractory to conventional antifungal drugs. More extensive data are recommended in order to asses a specific neonatal schedule.

Assessment of cardiac output in children: a comparison between the pressure recording analytical method and Doppler echocardiography.

OBJECTIVE: To assess cardiac output in pediatric patients with the pressure recording analytical method (PRAM) and the Doppler echocardiography method. PRAM derives cardiac output from beat-by-beat analysis of the arterial pressure profile (systolic and diastolic phase) in the time domain. DESIGN: A prospective observational study. SETTING: Pediatric intensive care unit at a tertiary care children's hospital. PATIENTS: Forty-eight patients between the ages of 1 month and 18 yrs. INTERVENTIONS: Femoral or radial artery catheterization and mechanical ventilation. MEASUREMENTS AND MAIN RESULTS: Cardiac output was simultaneously estimated by Doppler echocardiography and PRAM. Cardiac output values obtained by Doppler echocardiography (2.7 +/- 1.6 L/min, range 0.92-8.20) were significantly correlated with those estimated by PRAM (2.6 +/- 1.7 L/min, range 0.89-7.48; r2 = .99, p < .01). The mean difference between the two estimates was 0.12 +/- 0.27 L x min(-1) (95% confidence interval, -0.54 to 0.77 L x min(-1)). CONCLUSIONS: In the range of ages evaluated, PRAM provides reliable estimates of cardiac output when compared with noninvasive techniques.

The importance of a multidisciplinary approach in a child with major abdominal penetrating trauma.

We present a case of a 2-year-old girl who had a lawn mower accident with subtotal gut evisceration, multiple ischemic intestinal lesions, hepatic and gastric wounds, amputation of the left forearm, and hypovolemic shock. Prompt and adequate management was carried out in tertiary level institution, based upon quick evaluation of the lesions, fluid resuscitation, surgical repair, and postoperative admission to the pediatric intensive care unit.

Glandular hamartoma of the larynx: report of a case.

Glandular hamartoma is an extremely rare congenital malformation of the larynx. Presenting symptoms result from airways obstruction and may include slowly rising respiratory distress, stridor, changes in voice, eating and activity levels. Management consists in local mass excision with a good functional result and prognosis. Recurrences are usually associated with incomplete removal. We present a 3-month-old infant with a history of stridor and respiratory distress caused by a firm 0.4cm wide and 1.4cm long mass arising in the supraglottic region and detected with endoscopic approach. The lesion was excised endoscopically with an uneventful postoperative course and an excellent long-term prognosis. Histopathologically the lesion consisted of mature tissues with abnormal growth and disorganized architecture, chiefly composed of mature glandular structures, smooth muscular fibers, mature fat, surrounded by fibrous stroma and covered by typical squamous epithelium. The aim of our report is to underline how this condition must be considered by physicians, paediatricians and anaesthetists as an important cause of airway obstruction.