Pediatric Bilateral Vocal Cord Immobility: New Treatment With Preservation of Voice.

OBJECTIVES: Pediatric bilateral vocal cord immobility (BVCI) represents a severe life-threatening condition that often causes severe dyspnea. Endoscopic arytenoid lateral abduction (EALA) is a relatively new, secure, minimal-invasive surgical technique. The present prospective observational study aims to evaluate the effects of EALA in terms of respiratory function, voice quality, and swallowing capabilities. METHODS: Twenty-one pediatric patients with BVCI underwent EALA. Eleven out of 21 patients had tracheostomy at the time of surgery. Pre and postoperative functional assessments included endoscopic evaluation, maximum phonation time, pediatric Voice Handicap Index (pVHI), GIRBAS Scale criteria, and Montreal Children's Hospital Feeding scale (MCH-Feeding scale). peak tidal inspiratory flow or peak inspiratory flow (PIF) and number of desaturations/hour (ODI/h) were evaluated in patients without tracheostomy. RESULTS: Postoperative endoscopy showed glottic airway improvement in all patients. Average time for decannulation was 4.6 weeks. One patient has not yet been decannulated. No major complications occurred. In patients without tracheostomy, we observed a significant improvement of ODI/h and PIF after surgery (p < 0.05) as expected. PVHI, MCH-Feeding scale, and GIRBAS score significantly worsened 1 month after surgical intervention (p < 0.05). One year after surgery, however, all values, except for B and A parameters of the GIRBAS score, returned to levels comparable to those preoperative. CONCLUSIONS: EALA represents a simple, safe and effective solution in pediatric patients with BVCI, avoiding tracheostomy, allowing early decannulation, preserving swallowing function, and maintaining good quality voice. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:2325-2332, 2023.

Management of Upper Airway Infantile Hemangiomas: Experience of One Italian Multidisciplinary Center.

Airway infantile hemangiomas (IHs) can represent a life-threatening condition since the first months of life. They may be isolated or associated to cutaneous IHs, and/or part of PHACES syndrome. Diagnosis, staging, and indication to treatment are not standardized yet despite the presence in the literature of previous case series and reviews. The diagnosis might be misleading, especially in the absence of cutaneous lesions. Airway endoscopy is the gold standard both for diagnosis and follow-up since it allows evaluation of precise localization and entity of obstruction and/or stricture. Proliferation of IH in the infant airways manifests frequently with stridor and treatment is required as soon as possible to prevent further complications. The first line of therapy is oral propranolol, but duration of treatment is not yet well-defined. All considered, we report the experience of our multidisciplinary center from 2009 to date, on 36 patients affected by airway IHs, and successfully treated with oral propranolol. Thus, the authors propose their experience for the management of airway IHs, specifically early diagnosis, when to perform endoscopy, how to interpret its findings, and when to stop the treatment.

Prevention of recurrent respiratory infections : Inter-society Consensus.

Recurrent respiratory infections (RRIs) are a common clinical condition in children, in fact about 25% of children under 1 year and 6% of children during the first 6 years of life have RRIs. In most cases, infections occur with mild clinical manifestations and the frequency of episodes tends to decrease over time with a complete resolution by 12 years of age. However, RRIs significantly reduce child and family quality of life and lead to significant medical and social costs.Despite the importance of this condition, there is currently no agreed definition of the term RRIs in the literature, especially concerning the frequency and type of infectious episodes to be considered. The aim of this consensus document is to propose an updated definition and provide recommendations with the intent of guiding the physician in the complex process of diagnosis, management and prevention of RRIs.

Surgical Options for Pediatric Bilateral Vocal Cord Palsy: State of the Art.

Management of pediatric bilateral vocal cord palsy (BVCP) is a controversial and challenging topic. It may represent a severe obstructive condition usually associated with respiratory distress, and, in such condition, tracheostomy has been considered the gold standard for a long time. Many surgical options have been described and used to increase the glottic space in BVCP (1), with ongoing research of less invasive techniques. The challenge and current trend in our department and in many major pediatric centers is to avoid tracheotomy through an early treatment. Many techniques introduced in the last decade reduced the number of tracheotomies and increased the decannulation rate. Furthermore, we observed a recent increase in attention to preserve the quality of the voice with new techniques, such as endoscopic arytenoid abduction lateropexy which is in our opinion an important innovation to improve glottic space with satisfactory voice results. We present a review of the literature about the evolution of the treatment options for pediatric BVCP during the years.

International Pediatric Otolaryngology Group (IPOG): Juvenile-onset recurrent respiratory papillomatosis consensus recommendations.

OBJECTIVES: To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. RESULTS: Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP. CONCLUSIONS: Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.

Updated Guidelines for the Management of Acute Otitis Media in Children by the Italian Society of Pediatrics: Treatment.

BACKGROUND: New insights into the diagnosis, treatment and prevention of acute otitis media (AOM) have been gained in recent years. For this reason, the Italian Paediatric Society has updated its 2010 guidelines. METHODS: A literature search was carried out on PubMed. Only pediatric studies published between January 1, 2010 and December 31, 2018 in English or Italian were included. Each included study was assessed according to the GRADE methodology. The quality of the systematic reviews was assessed using AMSTAR 2. The recommendations were formulated by a multidisciplinary panel of experts. RESULTS: Prompt antibiotic treatment is recommended for children with otorrhea, intracranial complications and/or a history of recurrence and for children under the age of 6 months. For children 6 months to 2 years of age, prompt antibiotic treatment is recommended for all forms of unilateral and bilateral AOM, whether mild or severe. Prompt antibiotic treatment is also recommended for children over 2 years with severe bilateral AOM. A watchful-waiting approach can be applied to children over 2 years with mild or severe unilateral AOM or mild bilateral AOM. High doses of amoxicillin, or amoxicillin-clavulanic acid for patients with a high risk of infection by Beta-lactamase producing strains, remain the first-line antibiotics. CONCLUSIONS: AOM should be managed on a case-by-case basis that takes account of the child's age, the severity of the episode and whether it is unilateral or bilateral. In patients under 2 years, prompt antibiotic treatment is always recommended.

Decannulation in children affected by congenital central hypoventilation syndrome: A proposal of an algorithm from two European centers.

RATIONALE: Long-life ventilatory assistance is necessary for survival in pediatric patients with congenital central hypoventilation syndrome (CCHS). Invasive mechanical ventilation (IMV) through tracheostomy is the most used method, especially in the first years of life when the central nervous system is maturing. Nevertheless, IMV via tracheostomy is not ideal because tracheostomy in children is associated with impaired speech and language development, also frequent infections of the lower airway tract occur. OBJECTIVE: Only few reports describe the transition from IMV to the noninvasive method, ending with decannulation in CCHS affected patients. We aim to provide our experience regarding decannulation program in CCHS affected children and to describe a proposal of an algorithm concerning transition from invasive to noninvasive ventilation (NIV) in CCHS patients. METHODS: The study has been conducted retrospectively. Four children from two European centers underwent tracheostomy removal and decannulation, upon request of patients and their families. RESULTS: All children were trained to carry out tracheostomy capping before decannulation and underwent endoscopic assessment of upper and lower airway. Subsequently they started training to NIV at mean age of 106.25 months (±40.7 SD). Decannulation occurred 12 months after and no patients needed the reintroduction of tracheal cannula in either short or long term follow up. CONCLUSIONS: our study shows that effective liberation from IMV, the transition to NIV and decannulation are possible in CCHS affected children and offers a proposal of an algorithm which can be applied in selected centers.

Vocal cord paralysis appears to be an acquired lesion in children with repaired esophageal atresia/tracheoesophageal fistula.

OBJECTIVES: Determine whether vocal cord paresis or paralysis (VCP/P) following surgical repair of congenital esophageal atresia/tracheoesophageal fistula (EA/TEF) is generally a primary anomaly, or is secondary to EA/TEF repair. METHODS: We carried out a retrospective study based on a recently published protocol, which included the systematic performance of a laryngo-tracheo-bronchoscopy before and after EA/TEF repair. RESULTS: There were 105 patients with EA/TEF referred for multidisciplinary follow-up, between 2010 and 2015. Sixty-four of the 105 patients included in the study underwent EA/TEF repair at the referral center and had pre-operative bronchoscopy; the others had their initial surgery elsewhere. No included patient had VCP/P detected pre-operatively. Six patients (4 initially managed at the referral center) were diagnosed with VCP/P during the follow-up period (6/105, 5.7%). CONCLUSION: VCP appears to be an acquired lesion in this population.

Dehiscence of Bronchial Anastomosis After Lung Transplantation: A Successful Unconventional Treatment.

Anastomotic airway complications, including the dehiscence of the bronchial anastomosis, are a severe cause of morbidity after lung transplantation. We present a case of dehiscence treated by placing an uncovered metal stent into the main bronchus. We usually use this procedure for bronchial stenosis, but in this case, the stent favored the growth of granulation tissue and so the closure of the dehiscence. This procedure was minimally invasive and may be an alternative to an open repair, without precluding open repair in case of failure.

Bronchial Mismatch as a Predictor of Respiratory Failure After Congenital Tracheal Stenosis Repair.

BACKGROUND: The purpose of this study was to identify predictors of postoperative respiratory failure after surgical tracheoplasty for congenital tracheal stenosis. METHODS: Between February 2011 and April 2017, 16 infants underwent congenital tracheal stenosis repair. Preoperative computed tomography evaluation of the trachea and mainstem bronchi was performed. The primary outcome was midterm freedom from postoperative respiratory failure defined as persistent need for mechanical ventilation or surgical tracheobronchial reoperation. Bronchial mismatch, defined as [1 - (smaller bronchus diameter / larger bronchus diameter)] × 100, was analyzed in relation to the primary outcome. RESULTS: Median age was 106 days (range, 1 to 406) and median weight was 5.3 kg (range, 2.6 to 8 kg). Four patients were neonates (25%) and 6 had genetic abnormalities (37.5%). There were no early nor late deaths. Median ventilation time was 5.5 days (range, 3 to 45). Mean follow-up time was 2.2 years (range, 0.1 to 4.5). Four patients with bronchial mismatch greater than 20% had postoperative respiratory failure (p = 0.002). Two of them underwent tracheostomy and were discharged with ventilation home care support. One underwent successful reoperation consisting of bilateral bronchial plasty with autologous cartilage rib grafts, and the other underwent successful right bronchial and tracheal reconstruction. One patient with bilateral bronchial hypoplasia underwent slide tracheoplasty associated with preemptive bilateral bronchial plasty and made a full recovery. CONCLUSIONS: Surgical treatment of congenital tracheal stenosis in neonates and infants portends a good outcome. Bronchial mismatch greater than 20% can identify a subset of patients at increased risk for surgical reintervention and chronic respiratory failure. Slide tracheoplasty with preemptive bronchial reconstruction may prevent postoperative respiratory failure.

Unusual cause of dyspnoea.

A 4-year-old boy was admitted to our department with fever, cough and dyspnoea, unresponsive to salbutamol and antibiotic therapy. He had previously contracted bronchiolitis at 20 days of life, followed by intermittent episodes of wheeze that never required hospitalisation and responded to short inhaled corticosteroid cycles. He had an atopic family history. On examination, he had dyspnoea, persistent cough with bronchospasm but normal oxygen saturations. Bloods showed elevated eosinophils (2004 µL), a slightly elevated C-reactive protein (1.5 mg/dL) and total IgE (326 kU/L), and specific IgE was raised for various inhalant allergens (box). A chest X-ray was performed (figure 1).BoxPositive inhalant allergens Anthoxanthum odoratumCynodon dactylonDactylis glomerataDermatophagoides farinaeDermatophagoides pteronissimusHolcus lanatusPoa pratensisPhleum pratense edpract;103/6/300/F1F1F1Figure 1Chest X-ray of the patient. QUESTIONS: 1. What does the chest X-ray in figure 1 show? interstitial pneumoniapneumothoraxlung atelectasis with mild mediastinal shiftdiffuse air trappingenlargement of right hilar lymph nodes 2. Given the clinical picture and the chest X-ray, what would your differential diagnosis include from the following? plastic bronchitis (PB)mycoplasma infectiontuberculosisforeign body aspirationlung perforation 3. Are any of these conditions not associated with a specific type of cast/PB? Fontan procedurehaemophilialymphatic abnormalitiesasthma and other allergic disorderssickle cell disease.

Laryngotracheal Abnormalities in Esophageal Atresia Patients: A Hidden Entity.

Importance: Presence of laryngotracheal abnormalities is associated with increased morbidity and higher mortality rate in esophageal atresia patients. Objective: Determine the prevalence of laryngotracheal abnormalities (LTA) in a prospectively collected cohort of patients treated for esophageal atresia and/or tracheoesophageal fistula (EA/TEF). Analysis of the impact of those airway anomalies in early post-operative outcomes was performed. Patients and Methods: This was a review of a prospectively collected database, including patients from January 2008 to December 2017. Patients enrolled in the present study were treated in a high-volume referral center. Present study included all newborn-infants consecutively treated for EA/TEF. All patients were evaluated by flexible laryngotracheoscopy performed under local anesthesia in spontaneous breathing. In case of airway malformation suspected during flexible endoscopy, a rigid endoscopy was performed to complete airway assessment. If post-operative respiratory symptoms (noisy breathing, respiratory difficulty, failure to extubate, or difficulty feeding) were noted, a second laryngotracheoscopy was performed. Primary study outcome was to evaluate the prevalence of LTA in EA/TEF infants, characterizing of LTA, and their impact on early post-operative outcomes. Those primary study outcomes were planned before data collection began. Results: During the study period 207 patients with EA/TEF were treated. LTA had a period prevalence of 40.1% (83/207). Although no differences were recorded in terms of demographics and clinical presentation, LTA+ infants more frequently required tracheostomy (12/52, 23% vs. 0/124, 0%; p 0.0001) and were at increased risk of death (12/83, 14% vs. 5/124, 4%; p 0.009) in comparison with EA/TEF without LTA. Conclusions: Present data suggest a high prevalence of congenital LTA in patients affected by EA. Most of the abnormalities are congenital and a high proportion of patients with LTA require a tracheostomy. Mortality significantly correlates with the presence of LTA. Systematic airway endoscopic preoperative evaluation has to be pushed forward to minimize LTA-related morbidity and mortality.

Respiratory problems in children with esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care. METHODS: A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015. RESULTS: 69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%). CONCLUSIONS: Our study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.

Cervical repair of congenital tracheoesophageal fistula: Complications lurking!

AIM: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) consist of a spectrum of rare congenital abnormalities. Although EA surgical treatment is well established, the outcome of EA with proximal fistula (type B and D EA) or isolated H-type fistula (type E EA) is poorly explored. These forms of EA shared a common surgical step: the need of a cervical approach to close the fistula. Therefore, the aim of present study is to evaluate postoperative outcomes of patients treated for Gross type B-D and E EA, on regards of their cervical surgery. MATERIALS AND METHODS: A retrospective case series analysis of all patients affected by type B-D and E EA, and admitted to our tertiary care center between January 2003 and December 2014 was performed. All patients underwent preoperative flexible laryngo-tracheobronchoscopy (LTBS) as part of our standardized preoperative diagnostic assessment to define the diagnosis, evaluate preoperative vocal cord motility and to cannulate the fistula when required. Fistula closure was always performed through a right cervical access. Analysis of all cases and comparison between type B-D and E EA were performed. Mann-Whitney test, Chi-squared test and unpaired t test were used as appropriate; p<0.05 was considered significant. RESULTS: During the study period, 180 EA newborns were treated. Proximal or isolated TEF was found in 18 patients (10%): 7 type B, 11 type E EA. Patients affected by type B and E EA/TEF frequently present associated major malformations (27%), and major cardiac abnormalities (44%). Major postoperative complications were: vocal cord paralysis (5 patients), bilateral in 2 infants requiring tracheostomy, cerebral ischemia (1 patient), and cardiac failure (1 patient). CONCLUSION: Patients affected by type B and E EA have a high rate of associated abnormalities, and risk of possible sequelae. Postoperative complications are common, with possible transient vocal cord dismotility, but in some cases persistent paralysis may require tracheostomy. Therefore, both preoperative and postoperative LTBS is highly recommended to evaluate the presence of a proximal fistula, and vocal cord motility, even in asymptomatic patients, to rule out any possible intraoperative "surprise" and any vocal cord abnormality and to possibly define its pathogenesis (congenital vs. iatrogenic).

Development of an algorithm for the management of cervical lymphadenopathy in children: consensus of the Italian Society of Preventive and Social Pediatrics, jointly with the Italian Society of Pediatric Infectious Diseases and the Italian Society of Pediatric Otorhinolaryngology.

UNLABELLED: Cervical lymphadenopathy is a common disorder in children due to a wide spectrum of disorders. On the basis of a complete history and physical examination, paediatricians have to select, among the vast majority of children with a benign self-limiting condition, those at risk for other, more complex, diseases requiring laboratory tests, imaging and, finally, tissue sampling. At the same time, they should avoid expensive and invasive examinations when unnecessary. The Italian Society of Preventive and Social Pediatrics, jointly with the Italian Society of Pediatric Infectious Diseases, the Italian Society of Pediatric Otorhinolaryngology, and other Scientific Societies, issued a National Consensus document, based on the most recent literature findings, including an algorithm for the management of cervical lymphadenopathy in children. METHODS: The Consensus Conference method was used, following the Italian National Plan Guidelines. Relevant publications in English were identified through a systematic review of MEDLINE and the Cochrane Database of Systematic Reviews from their inception through March 21, 2014. RESULTS: Basing on literature results, an algorithm was developed, including several possible clinical scenarios. Situations requiring a watchful waiting strategy, those requiring an empiric antibiotic therapy, and those necessitating a prompt diagnostic workup, considering the risk for a severe underling disease, have been identified. CONCLUSION: The present algorithm is a practice tool for the management of pediatric cervical lymphadenopathy in the hospital and the ambulatory settings. A multidisciplinary approach is paramount. Further studies are required for its validation in the clinical field.

Postpneumonectomy syndrome in a newborn after esophageal atresia repair.

INTRODUCTION: Postpneumonectomy syndrome (PPS) is an ominous complication, caused by mediastinal shift following massive lung resection. PRESENTATION OF THE CASE: A neonate with oesophageal atresia and tracheo-oesophageal fistula developed acute respiratory distress shortly after surgery, despite mechanical ventilation. The patient was found to have an associated oesophageal right lung that collapsed after oesophageal atresia repair and a left pulmonary artery sling causing left main bronchus stenosis mimicking a postpneumonectomy syndrome. DISCUSSION: We will describe the diagnostic work-up and the therapeutic measures used both in the acute phase and as definitive treatment in this challenging case. CONCLUSIONS: Neonatologists and paediatric surgeons should be aware of this rare association that may cause acute life threatening and worsening of patient's clinical status. Prompt realignment of the mediastinum in the normal position is critical to obtain rapid improvement of the patient's clinical conditions.

Pediatric BAHA in Italy: the "Bambino Gesù" Children's Hospital's experience.

The "Bambino Gesù" Children's Hospital offers the largest Italian cohort of children using a BAHA. This paper retrospectively reviews the center's experience with pediatric BAHA from its start in 1995 to December 2009, especially focusing on most debated issues, all the way from candidate selection to functional outcome and complications. A total of 47 children (21 females and 26 males) were implanted. From 1995 to 2000, a two-stage procedure was adopted in 100% (n = 22) cases. From 2001 onwards, one-stage surgery was chosen for 10 patients. Implant loss occurred in two patients (4.2%) after a two-stage surgery. Overall, the local complication rate was not different in the one-stage and in the two-stage group. The functional gain was significantly better with BAHA than conventional bone-conduction hearing aids and there was no significant difference in terms of functional outcome between the seven patients receiving a BAHA at an age younger than 5 years and the rest. The study results suggest that children presenting an indication to auditory rehabilitation with BAHA should be referred to specialized tertiary care centers. Only in such settings, a complete, multidisciplinary approach to associated comorbidities and syndromes is possible, and implantation of children at an age younger than five and one-stage surgery can be conducted safely and effectively.

Genetic prothrombotic factors in children with otogenic lateral sinus thrombosis: five case reports.

Lateral sinus thrombosis (LST) is an uncommon, but life-threatening complication of both acute and chronic otitis media. There is some evidence that acquired or hereditary prothrombotic disorders are risk factors for LST. The aim of this work was to evaluate the role of thrombotic screening, anticoagulant therapy or prophylaxis in patients with either acute or chronic otitis media and LST. The medical records of five children hospitalized at Pediatric Hospital Bambino Gesù of Rome because of acute or chronic otitis media complicated by mastoiditis and LST were reviewed. All children underwent laboratory workup for hypercoagulability. All the five children were found to be heterozygote for the C677T MTHFR mutation and a child presented also heterozygosity for factor V Leiden mutation. They have been successfully treated with anticoagulant therapy without sequels. Children with acute or chronic otitis media may have a prothrombotic tendency that becomes clinically evident because of the inflammatory state. Patients with a family and/or personal history of thrombosis and/or thrombophilic conditions need anticoagulant prophylaxis also in the absence of clear signs of LST. Treatment with low molecular weight is successful in patients with LST.