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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.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.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.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.4 Laryngoscope, 2022.

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.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.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.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.

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. Supplementary Information The online version contains supplementary material available at 10.1186/s13052-021-01150-0.

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.

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.

Background: reports suggest that congenital esophageal atresia/tracheoesophageal fistula (EA/TEF) is associated with a number of upper airway anomalies [1,2]. Aims: it remains unclear whether vocal cord paresis or paralysis (VCP) is usually a primary anomaly, or is secondary to EA/TEF repair. Methods: a retrospective study was carried out in 105 patients with EA/TEF referred to the Department of Pediatric Medicine, Bambino Gesu Children’s Hospital (BGCH) for multidisciplinary follow-up following EA/TEF repair, between 2010 and 2015. Results: 64 of the 105 patients included in the study underwent EA/TEF repair and had pre-operative bronchoscopy in BGCH; the remaining patients had their initial surgery elsewhere. No patient included in the study had VCP detected pre-operatively. Six patients were diagnosed with VCP during the follow-up period (6/105, 5.7%), of whom 4 were surgically managed at the BGCH, and two were managed elsewhere. Four children with bilateral VCP initially required tracheostomy. The other two patients were treated with posterior cordotomy by CO2 laser, and one received a tracheostomy. Conclusion: we did not observe VCP in pre-operative bronchoscopy in patients undergoing EA/TEF repair, but observed VCP in 5.7% of patients during a post-operative re-evaluation, suggesting that VCP is an acquired lesion in TEF patients. Future studies are needed to determine whether VCP is present in some patients prior to EA/TEF repair, and if so, what the prevalence is. References: 1. Ann Otol Rhinol Laryngol. 2015; 124: 808-13. 2. Laryngoscope. 2015; 125: 469-74.

Object The term ‘vocal cord immobility’ (VCI) encompasses both vocal cord paralysis and crico-arytenoid joint ankylosis (CAJA). Bilateral VCI represents an emergency condition characterized by stridor and respiratory distress sometimes requiring tracheostomy to ensure an adequate and safe airway. The aim of this study is to describe the diagnostic and therapeutic management of a rare case of congenital bilateral CAJA in a patient without pregnancy complications, perinatal traumas, or other comorbidities. Materials and Methods The patient was born full term by C-section after a healthy pregnancy (BW 3270 g, APGAR 9 at 5'). After birth she presented severe stridor with respiratory distress. She was admitted to our hospital when she was 3 months old. The diagnostic assessment was performed with airway endoscopy, pulmonary function tests (PFT), sleep study, echocardiogram, neurological evaluation, chest computed tomography (CT) scan, and brain magnetic resonance imaging (MRI). Laryngeal electromyography (LEMG) with endoscopic placement of Hookwire electrodes was carried out for the differential diagnosis between paralysis and ankylosis and the subsequent choice of the treatment. PFT and sleep study were repeated after each endoscopic procedure. Results The first airway endoscopy showed bilateral VCI in paramedian position and palpatory evidence of bilateral crico-arytenoid joint fixation. PFT highlighted inspiratory obstruction at the flow/volume and flow/time curves and tidal volume reduction. Sleep study was indicative of mild–moderate obstructive apnea. Echocardiogram, neurological evaluation, brain MRI, and chest CT scan did not detect anomalies. LEMG showed continuous low-amplitude basal activity in all analyzed muscles, in the absence of spontaneous neurotonic activations. Motor evoked potentials (MEP) denoted normal left response and minimum right delay. Two glottic dilations were performed with 7 and 8 mm balloons determining the decrease of stridor and good respiratory balance confirmed by PFT. Endoscopy showed a slight recovery of laryngeal motility. Conclusion The management of this rare clinical case points out the crucial role of a careful and complete endoscopic examination including the palpation of the crico-arytenoid joints. Moreover LEMG represents an important instrument for the correct differential diagnosis in VCI. In the future the use of LEMG could be mandatory in pediatric patients in order to avoid tracheotomy in favor of more conservative procedures.

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.

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 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).

Objectives Laryngopharyngeal reflux (LPR) is a complex problem in pediatric population: diagnosis and clinical presentation are still controversial. Classic white light endoscopy shows some pathognomonic signs of LPR in children, such as thickening of pharyngo-laryngeal mucus, the cobblestoning aspect of pharyngeal mucosa, arytenoid edema/hyperemia, nodular thickening/true vocal cord edema, hypertrophy of the posterior commissure, subglottic edema. The NBI (Narrow Band Imaging) technology, generally used in oncology, allows to study neoangiogenesis and hypervascularization of the mucosa, common aspects in both chronic inflammation and neoplastic transformation. The aim of our study was to evaluate the added value of this technology in identifying the main laryngopharyngeal reflux sign in a pediatric population. Methods We evaluated at the Otolaryngology Unit of the “Fondazione Policlinico A. Gemelli” hospital and the Airway Surgery Unit of the “Bambino Gesu” Children's Hospital 35 patients aged from 2 months to 16 years divided into two groups in the period between November 2017 and May 2018. Group A included patients with clinical suspicion of LPR after gastroenterologist evaluation and Group B included patients who underwent an endoscopic evaluation for the assessment of recurrent respiratory symptoms such as stridor, recurrent croup, wheezing and persistent cough. We performed an endoscopic evaluation by white light and NBI for each patient, comparing the results of both methods to evaluate signs of pharyngo-laryngeal reflux and to calculate the value of reflux finding score (RFS). Results The analysis of the data showed: for Group A an average value of RFS with white light of 11,84 (range 8–17, standard deviation 2,52 ± 0,57) and with NBI of 13,63 (range 10–17, standard deviation 2,13 ± 0,49); for Group B the analysis of the data showed an average value of RFS with white light of 10,06 (range 8–14, standard deviation 2,32 ± 0,58) and with NBI of 12,50 (range 9–18, standard deviation 2,63 ± 0,65). The comparison between the two methods resulted significant. Furthermore evaluation by NBI allowed to highlight other signs of pharyngo-laryngeal reflux, characteristic of pediatric age and not included in RFS, in particular cobblestone aspect of the hypopharingeal mucosa, phlogosis of the tonsillar crypts and adenoid surface, hyperemia and hypervascularization of subglottic and tracheal mucosa. Conclusion Although still preliminary our results represent an interesting starting point for further studies, because they underline the potentiality of NBI endoscopy in LPR evaluation and how this technology could improve the identification of reflux signs.

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.

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.

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.

Vascular lesions are the most common congenital and neonatal abnormalities. The aim of this work is to point out differences between various vascular anomalies, in order to define accurate diagnosis, and to present different therapeutic options now used for the treatment of the vascular lesions in children. According to biological classification described by the work of Mulliken and Glowacki there are two major types of vascular abnormality: haemangioma and vascular malformation. Haemangioma is a distinct biologic tumour entity characterised by rapid endothelial proliferation shortly after birth. The lesion is absent at birth and growth in early infancy, followed by a spontaneous resolution in childhood. Vascular malformations are structural anomalies that have a normal growth rate and endothelial turnover. According to the morphology of the vessels and the flow rate we distinguish: slow-flow and fast-flow vascular malformation. The authors document their personal experience in diagnosis, clinical evaluation, treatment and follow-up of the vascular lesions.

In September 2005, the 3rd OPBG/Mayo Clinic Joint Conference was held in Rome to highlight current views in paediatric medicine. After an overview of the various pathologies, the Otolaryngology Unit of the Bambino Gesu Children's Hospital of Rome described their own experience with endoscopic surgery for the treatment of upper airway diseases between 2002 and 2004. During this period, 1500 paediatric endoscopies were performed for obstructive upper airway disease. A total of 80% of patients were under 2 years of age. The aetiological causes of upper airway obstruction most frequently cited were: foreign bodies (the most frequent reason for urgent endoscopic treatment), choanal atresia, severe laryngomalacia, laryngeal cysts, neurological lesions (vocal fold paralysis), laryngeal clefts, laryngeal haemangiomas, recurrent respiratory papilloma, tracheoesophageal fistula, tracheal granuloma and tubercular adenobronchial fistula. In all the patients, stridor disappeared following surgery. No complications, such as aspiration or supraglottic stenosis, were identified and no revision surgeries were required. Stenting was necessary in only three cases. This study described the OPBG management of paediatric airway obstruction and outcomes during a 3-year period and was the starting point for reflection on this topic.

Highlights • After esophageal atresia repair, if postpneumonectomy syndrome develops, an associated esophageal lung must be considered. • Contrast esophagography, with the patient lying on the side of collapsed lung, is the optimal diagnostic study. • In infants with postpneumonectomy syndrome, infusion of normal saline in the hemithorax on the side of collapsed lung is a simple, rapid, and effective method to obtain mediastinum realignment in emergency with immediate improvement of clinical conditions., 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.

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). Box Positive inhalant allergens Anthoxanthum odoratum Cynodon dactylon Dactylis glomerata Dermatophagoides farinae Dermatophagoides pteronissimus Holcus lanatus Poa pratensis Phleum pratense Questions 1. What does the chest X-ray infigure 1show? interstitial pneumonia pneumothorax lung atelectasis with mild mediastinal shift diffuse air trapping enlargement 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 infection tuberculosis foreign body aspiration lung perforation 3. Are any of these conditions not associated with a specific type of cast/PB? Fontan procedure haemophilia lymphatic abnormalities asthma and other allergic disorders sickle cell disease.

Francesca Petreschi (FP): A 9.8 year–old-girl was first admitted in our unit for a persistent bronchospasm, poorly responsive to salbutamol, and progressive onset of respiratory distress after spontaneous expectoration of a bronchial cast (figure 1A). Her clinical history was uneventful until she was 7 years old, when she suffered from two subsequent bouts of pneumonia, but no admission was required. In the following years, she presented few episodes of bronchospasm, clinically diagnosed, with good response to bronchodilator and no limitation on her daily activities. Her family history was positive for allergic disease. On physical examination at her arrival, she appeared quite unwell with persistent wet cough, bilateral respiratory wheeze but normal levels of saturation. The remaining examination was normal. Figure 1 (A) Bronchial cast. (B) Bronchial fistula secreting milky material under pressure. (C) CT scan of the thorax demonstrating left lower lobe opacity and pleural effusion. (D) Lung biopsy compatible with pulmonary lymphangiectasia. (E) CT scan showing chylopericardium. Renato Cutrera (RC): We have a previously healthy young girl with positive family history for allergies and personal history of few lower respiratory infections and intermittent episodes of wheezing. Patient's history and the spontaneous expectoration of a bronchial cast during an acute episode of bronchospasm, together with her physical examination characterised by intense cough and diffuse wheeze are suggestive of a case of plastic bronchitis (PB), already described as possible complication of allergic asthma. The differential diagnosis, though, should always include more inflammatory diseases of the lung, such as cystic fibrosis, pulmonary infections and acute chest syndrome in sickle-cell anaemia.1 Nicola Ullmann (NU): Patient was afebrile; blood exams were normal, including: haemoglobin, c-reactive protein, immunoglobulins and lipid values. Allergy tests were positive to house dust mites and few pollen, but sweat tests and nasal brushing for ciliary dyskinesia were negative. Spirometry showed mainly …

Heterotopic neuroglial tissue is a rare lesion, occurring more frequently in the nasal cavities. Other rare locations are the orbit, the scalp, the palate, the pharynx, the parapharyngeal space and the lungs. They are usually detected occasionally because they are often asymptomatic, but sometimes they might present with dyspnoea, feeding difficulty, snorting and nasal flaring. Respiratory symptoms occur when heterotopic neuroglial tissue is located in the parapharyngeal space. We report a case of an infant affected by Pierre Robin sequence (PRS) who was admitted to our Institution for a worsening respiratory distress that was not explainable only by PRS.

Objective: Most of the studies on the surgical treatment of severe laryngomalacia have aimed to describe the efficacy of the treatment in terms of improvement of clinical symptoms or anatomical findings. The aim of this study is to analyze the changes in breathing patterns following aryepiglottoplasty for severe laryngomalacia.Method: A series of retrospective cases at a tertiary referral children’s hospital. Between 2005 and 2010, 40 infants with severe laryngomalacia were studied before (T1) and after surgery (T2). Breathing patterns were studied using an ultrasonic flow-meter measuring: tidal volume, respiratory rate, time to peak and mean expiratory flow/expiratory time ratio.Results: The surgical procedure was well tolerated in all the infants and no perioperative and long-term complications were present. In T1, the breathing pattern is characterized by low tidal volume and high flow/expiratory time ratio (tPTEF/Te) and mean expiratory/mean inspiratory flow ratio (MEF/MIF), suggesting a severe reduct...

Objective: Present a rare case of a massive posttraumatic nasal swelling hiding a complication of an undiagnosed nasal dermoid sinus cyst in a 3-year-old boy. Emphasize that the clinical history, careful assessment, and especially accurate imaging are essential for accurate diagnosis and the proper treatment to be applied.Method: A 3-year-old boy was admitted to our hospital because of swelling of the fronto-nasal regions following a nasal trauma occurring 5 days earlier. X-rays of the nasal bones in the lateral side were negative for fracture. Given the noteworthy facial deformation we opted for a hospitalization for further study.Results: Bone-image CT and 3D-CT revealed bone defects within the frontal skull base. MRI demonstrates that a dermoid cyst centered in a bone defect was in contact with the dura of the frontobasal area, and a dermal sinus extending to the frontonasal skin could also be detected. Only then we observed the midline pit on the nasal dorsum initially attributed to the outcomes of re...

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 Gesu 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.

The “Bambino Gesu” 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.

Training through Research Application Italian iNitiative (TRAIN) is a mobility program financed under the EU action called “Cofinancing of regional, national and international programs” (COFUND) of the European Commission Seventh Framework Program (FP7) – People, and has been designed to encourage the promotion and development of international programs of research through mobility at various stages of research careers. The aim of TRAIN is to improve translational skills in the field of cancer by promoting a three-year international mobility program assigning a total of 51 fellowships subdivided into incoming, outgoing and reintegration fellowships. The TRAIN proposal has been submitted in February 2009 to the European Commission in reply to the 2008 FP7-PEOPLECOFUND call and has been successfully evaluated. TRAIN is addressed to postdoctoral scientists or scientists who have at least four years' full-time equivalent research experience and who wish to improve their careers spending one year abroad. The mobility program is open also to non-Italian experienced scientists wishing to spend one year in an Italian research center or private company. Part of the scheme is targeted to experienced Italian scientists who have completed at least three years of research in a foreign country and are interested in returning to Italy. TRAIN is part of an overall Italian strategy outlined by the International Program of the Italian Cancer Network “Alleanza Contro il Cancro” to promote Italian participation in the building of the European Area for translational cancer research and to enhance the interaction between academy and industry.

Objectives To describe the prevalence and pathogenesis of symptomatic vocal cord paresis/paralysis (VCP) in patients treated for esophageal atresia (EA), tracheo-esophageal fistula (TEF) or both. Study design Retrospective study of all patients treated for EA/TEF in our center (1995 to 2009). Patients with and without symptomatic VCP were compared for gestational age, birth weight, associated anomalies, referrals, long-gap EA (>3cm or 3 vertebral bodies), cervical esophagostomy, anastomotic leakage, length of ventilation, and major cardiac surgery. Prevalence or median (IQR) is reported. Results Of 174 patients, 7 (4%) had symptomatic VCP. Prevalence of referrals (5/7 versus 21/167; P = .0009), long gap (5/7 versus 41/167; P = .0146), previous cervical esophagostomy (5/7 versus 7/167; P P = .0097) was higher, and ventilation longer (8.5 days [7.0 to 15.5] versus 6.0 days (5.0 to 7.0); P = .0072) in patients with VCP. Conclusions In infants treated for EA/TEF, VCP should be ruled out in case of persistent respiratory morbidity or, when present, cautiously monitored. Surgical risk factors should be actively controlled. Further studies are needed to define the prevalence of acquired and congenital VCP in patients with EA/TEF.

Background/Purpose: Despite surgical refinements, perioperative use of tracheobronchoscopy (TBS) as part of surgical approach to esophageal atresia (EA) is still controversial. The purpose of this study was to evaluate the influence of preoperative TBS in newborns with EA in preventing complications and improving diagnosis and surgical treatment. Methods: In the period ranging from 1997 to 2003, 62 patients with EA underwent preoperative TBS. The procedure was carried out with flexible bronchoscope maintaining spontaneous breathing. When a wide carinal fistula was found, this was mechanically occluded by Fogarty catheter and cannulated with rigid bronchoscopy. Type of EA, surgical procedure variations caused by TBS, and associated anomalies not easily detectable were recorded. Results: Before TBS, the Gross classification of the 62 patients was as follows: type A, 9 patients; type B, none; type C, 51 patients. At TBS, however, 3 of 9 type A patients had an unsuspected proximal fistula (type B). These 3 patients, plus the 2 with H-type fistula, were repaired through a cervical approach. In 4 patients, previously undetected malformations of the respiratory tree (2 aberrant right upper bronchus and 2 hypoplastic bronchi) were found at TBS. Carinal fistulas in 14 type C patients were occluded by Fogarty catheter to improve ventilation during repair. No complications were observed. Overall, TBS was clinically useful in 28 (45.2%) of 62 patients, including 15 (24.2%) of 62 infants in whom it was crucial in modifying the surgical approach. Conclusion: Tracheobronchoscopy is a useful and safe procedure and should be recommended in tertiary centers for babies with EA before surgical repair.

We report a case of worsening respiratory distress associated with opisthotonus secondary to tracheomalacia, a rather unique pathophysiological phenomenon. A 2-month-old male baby was referred to our hospital for respiratory distress syndrome with a noticeable opisthotonus. Examination and investigation confirmed the presence of an aberrant innominate artery compressing the trachea. The infant underwent aortopexy and made a dramatic post-operative recovery. Of special note, the opisthotonus vanished soon after the operation. Opisthotonus is not always related to neurological impairment and may be a warning sign of mediastinal overcrowding in patients with respiratory distress syndrome secondary to vascular compression.

The aim of this study is to assess the frequency and clinical importance of mediastinal tubercular lymphadenitis and adenobronchial fistulas (TABF) and to evaluate the role of fiberbronchoscopy and surgical bronchoscopy associated with antimicrobical chemotherapy. 136 cases of primary pulmonary TBC, admitted to the Unit of Infectious Diseases, Bambino Gesu Children Hospital in Rome, between 1980 and 2001, were enrolled in the study. We considered 56 patients with clinical and radiological evidence of mediastinal tubercular lymphadenitis and 28 patients with adenobronchial fistulas (TABF). The incidence of TABF was 20,58% of primary pulmonary TBC. All patients were treated by medical therapy combined with local endobronchial surgery. TABF emerges as a complication of pediatric primary pulmonary TBC. We suggest a clinical and radiological survey to decide the utility of a diagnostic and therapeutic surgical bronchoscopy

A 13-year-old girl underwent surgery at the Ear Nose and Throat Department of the Children's Hospital and Scientific Research Institute, Bambino Gesu, Rome, because of an acinic cell carcinoma of the parotid gland. Two years after total parotidectomy with preservation of the facial nerve, metastasis occurred in a lateral cervical lymph node. Acinic cell tumors are uncommon in childhood; in children under the age of 16 years only 35 cases have been reported. There are too few acinar cell carcinomas occurring in children to yield an idea of their natural course. The neoplasm is also referred to as ‘acinar cell tumor’, in contrast to acinar cell carcinoma, because the neoplasm may be benign or malignant. Unfortunately, there are no histological distinguishing features that permit the pathologist to determine which neoplasm will behave in an aggressive fashion.

Summary Two rare cases of benign dysontogenetic neoplasms of the thyroid gland in pediatric age are presented, which were observed at the E.N.T. department of the Bambino Gesu Hospital of Rome, and successfully operated. The first case turned out to be a dysembryoma, classified as monodermic because of its origin from derivatives of only one germinal layer, the entoderm. The second case, a teratoma, presented a high seric concentration of AFP as the consequence of the synthetic activity of the share of embryonal epatic tissue present in the neoformation. Criteria to distinguish thyroid from cervical teratomata and to classify the different types of dysontogenetic neoplasms are discussed. The usual clinical and pathological manifestations of teratomas of the neck in infancy are mentioned. The necessity of a prompt surgical removal and of pre- and postoperative thyroid function studies is mentioned.

Ninety-three cases of infantile chronic recurrent parotitis are recorded. Fifty-three cases were treated with parasympathectomy which was carried out at 2 levels. Good results were achieved in 79.1 % of patients. The paper describes the use of the Doppler examination to study the effects of operation on the physiology of the parotid.