Paranasal mucoceles in children with cystic fibrosis: Management of a not so rare clinical condition.

PURPOSE: Paranasal mucocele (PM) is reported as a complication in children with cystic fibrosis (CF) in up to 4% of patients. The objective of this study was to identify PMs in the personal large series of children with CF and to assess their diagnosis and treatment. MATERIAL AND METHODS: Medical records of children with CF and PM who were admitted and treated by means of endoscopic nasal surgery between 2004 and 2020 were retrospectively reviewed. RESULTS: Thirty-four patients were included in the study (mean age 7.7 years). CT scan of sinuses showed a total of 53 PMs. Nasal endoscopic findings suggestive for PM were present in almost 80% of patients. PMs were located in the maxillary, ethmoid, and sphenoid sinuses in 29/34 (85.3%, bilateral in 17 cases), 4/34 (11.8%) and 1/34 (2.9%) patients, respectively. Marsupialization of PMs was performed in all patients using an endoscopic transnasal approach. No complications were observed. Resolution of symptoms and normalization of the endoscopic nasal picture was evident in all patients. After a mean follow-up of 85 months, no recurrences were observed. CONCLUSIONS: To the best of our knowledge, this is the largest series of CF patients with PMs. Even if not frequently reported in the literature, PMs should not be considered an uncommon finding in patients affected by CF. Routine nasal endoscopy is mandatory to favor early diagnosis. Endoscopic transnasal marsupialization represents the gold standard of care for patients with CF and PM(s).

Case report: pharyngolaryngeal stenosis in a child due to caustic ingestion treated with transoral CO2 laser microsurgery.

Accidental caustic ingestion occurs mainly in the 2- to 3-year-old age group. Up to 33% of patients develop long-term complications that principally involve the gastroesophageal tract, whereas their occurrence at the level of pharyngeal and laryngeal structures is less frequent. When present, strictures are the main disorders that can be observed. In this pathological situation, surgery is the treatment of choice, and several procedures have been described. We report the history of a 3-year-old boy affected by pharyngolaryngeal stenosis due to accidental caustic ingestion. After careful diagnosis, the child underwent surgery by transoral CO2 laser. The patient had immediate improvement and restarted oral feeding 1 day after the surgical procedure. An analysis of diagnosis and treatment of this long-term complication is also presented.

A conservative treatment for plastic bronchitis in pediatric age.

Plastic bronchitis is a rare disorder in pediatric age. This disease can cause life-threatening episodes. Broncoscopy plus bronchial lavage is considered the gold standard therapeutic technique. Knowledge of this disease is mandatory to perform correct diagnosis and provide prompt treatment. The authors report the history of a 5-year-old girl affected by plastic bronchitis who was successfully treated by a conservative therapy avoiding the traditional more invasive management.

The Role of Bronchoscopy in the Management of Children With Cystic Fibrosis.

BACKGROUND: Currently, no consensus guidelines recommend routine bronchoscopy procedure in cystic fibrosis (CF), as no evidence is available concerning its use as either a diagnostic or therapeutic tool. Its efficacy is controversial, and no randomized controlled prospective trials are available to check its effectiveness. The aims of the present study were to evaluate the effectiveness of bronchoscopy as a diagnostic/therapeutic tool in CF children and adolescents; and to verify the effect of serial bronchoscopy on lung disease progression in subjects with CF not responding to a single procedure. METHODS: Data of patients who received bronchoscopy at 2 Italian CF centers were collected. Bronchoalveolar lavage was performed during the procedure including airway clearance with mucolytics, inhaled antibiotics, and/or surfactant instillation. RESULTS: A total of 16 patients in center 1 and 17 in center 2 underwent, respectively, 28 and 23 bronchoscopic procedure in the study period. Five patients in each center underwent >1 procedure. All procedures were generally well tolerated. No patient required admission to the pediatric intensive therapy unit. In 19.6% of bronchoalveolar lavages, growth of Aspergillus fumigatus was evident, although not detected by sputum analyses. After the procedure, an increase in mean percent predicted forced expiratory volume in the 1 second >10% was observed, and a significant decrease in pulmonary exacerbations yearly was evident. CONCLUSION: Based on the results, we suggest bronchoscopy is not to be considered an obsolete tool, and it remains useful in CF management, although in selected cases. We encourage to support longitudinal observational studies to standardize the procedure, focusing on the choice of drugs to be instilled, modalities and timing of serial bronchoscopy and subsequent follow-up in selected severe clinical conditions.

Management of chronic rhinosinusitis.

: Chronic rhinosinusitis (CRS) in children is difficult to treat, with resultant frequent recurrences and failures. There are controversies in the treatment, mirroring the debate over the exact etiology of this disorder. The available medical treatments are antibiotics, topical nasal corticosteroids, and nasal lavage with saline solutions; though, there is no general agreement on the efficacy of the latter. The new technique of balloon sinuplasty allows ventilation to the sinuses to be restored with minimal risk and trauma to the tissues, and initial outcome seems promising, being successful in most treated children. Concerning the surgical approach, adenoidectomy is among the most frequent surgical procedures performed on children, but its therapeutic effect is controversial, because randomized studies have failed to prove that adenoidectomy alone is sufficient in curing CRS. Instead, functional endoscopic sinus surgery is a minimally invasive technique which restores the sinus ostia patency and can re-establish ventilation and drainage through the natural pathways. It is important that the effectiveness of any treatment is also evaluated by patient-reported outcomes (PROs) that refer to all health-related reports coming from the patients, without any involvement or interpretations by physician or others. Among PROs, health-related quality of life (HRQL) is the one most widely known and used. HRQL can be measured by means of validated questionnaires, which provide scores proportional to the degree of well-being perceived by patients. Concerning diseases of the upper airway including RS, there are numerous instruments specifically designed for children and caregivers, which allow to assess the effects of treatments in a more extensive and complete manner.

Ciliary aplasia associated with hydrocephalus: an extremely rare occurrence.

Ciliary aplasia is a rare congenital disease that alters the normal function of the mucociliary apparatus in several organs. Patients generally present with severe recurrent and chronic infections of the airways. A high suspect of this disorder is mandatory to perform correct diagnosis and provide prompt treatment. The authors describe the history of two siblings affected by primary ciliary aplasia that was associated with hydrocephalus in one case. A careful description of diagnostic procedures and treatment of this extremely rare disorder is also presented.

Spontaneous Abscess of the Posterior Nasal Septum: An Unusual Cause of Nasal Obstruction in Children.

INTRODUCTION: Nasal septal abscess is a rare disorder in pediatric patients and is mostly diagnosed as a complication of trauma or secondary to dental or sinonasal infection. CASE PRESENTATION: A 10-year-old girl presented with acute nasal obstruction, otalgia, and headache. Medical history was negative; physical examination and anterior rhinoscopy did not reveal signs of acute rhinosinusitis or septal abscess. MANAGEMENT AND OUTCOME: A thorough nasal endoscopy was performed to rule out a posterior nasal infection, revealing a bilateral posterior septal bulging, in the absence of purulent discharge. CT scan and contrast-enhanced MRI were performed, confirming the diagnosis of a spontaneous posterior septal abscess. Trans-nasal endoscopic-assisted drainage was conducted under general anesthesia. Haemophilus influenzae was detected on culture. DISCUSSION: Although rare, nasal septal abscess in the pediatric age is typically anterior and secondary to local trauma or infection. Accurate medical history and anterior rhinoscopy are usually sufficient to make the diagnosis. In cases where the clinical presentation is consistent with a nasal septal abscess, with no history of recent local trauma or infection, or signs of anterior septal bulging, nasal endoscopy should be performed to rule out spontaneous posterior septal abscess. Delay in diagnosis and treatment could potentially cause the rapid onset of life-threatening complications, including intracranial abscess, meningitis, and cavernous sinus thrombosis.

Endoscopic treatment of tracheocele in pediatric patients.

Acquired tracheal pouch known also as tracheocele is a rare air-filled diverticulum of tracheal pars membranacea. This disease may be due to esophageal or tracheal surgery, orotracheal intubation, or increased intralunimal pressure through a weak area of tracheal wall. When symptomatic and medical therapy is insufficient, this disorder must be treated surgically. Several surgical methods ranging from open neck or thoracic surgery to endoscopic managements have been reported. We report the case history of a 7-year-old boy affected by recurrent pneumonia due to tracheal pouch. The patient underwent successful brushing of tracheocele plus fibrin glue application by rigid tracheobroncoscopy. Furthermore, particular emphasis on endoscopic treatments of tracheal diverticulum is also presented.

Treatment of congenital nasolacrimal duct cyst: the role of endoscopic marsupialisation.

OBJECTIVE: Congenital nasolacrimal duct cyst (NLDC) is a rare disorder, which can present with ophthalmological and nasal signs and symptoms. The authors analyse their personal experience to identify diagnostic criteria for NLDC, which were treated by endoscopic transnasal procedure. METHODS: Clinical records of patients with a diagnosis of NLDC were retrospectively reviewed. All patients underwent rhinoscopy and ophthalmologist evaluation before surgery, whereas imaging was performed in selected cases. All neonates underwent transnasal endoscopic marsupialisation after failure of conservative medical therapy. RESULTS: Five patients were included in the study. One patient presented bilateral NLDC. In 3 cases, CT scan of the sinus was carried out. A total of 6 marsupialisation procedures were performed and a bi-canalicular lacrimal stent was positioned in 1 case. Complete remission of symptoms was observed in all cases. CONCLUSIONS: Nasal endoscopy is mandatory to diagnose NLDCs, and, in some cases, it can be complemented by radiological procedures. When symptoms persist after systemic and topical therapy, nasal endoscopic marsupialisation is the treatment of choice. This surgical procedure is effective, safe and can be repeated if needed.

Endoscopic outcomes of resorbable nasal packing after functional endoscopic sinus surgery: a multicenter prospective randomized controlled study.

Nasal packings can aid in control of postoperative bleeding and healing following functional endoscopic sinus surgery (FESS), but traditional non-resorbable stents have several inherent drawbacks. We performed a randomized, controlled, multicenter clinical trial to assess efficacy of resorbable nasal packing in patients undergoing FESS for chronic rhinosinusitis. A total of 66 patients for 88 nasal cavities were randomized to receive either hyaluronan resorbable packing (MeroGel) or standard non-resorbable nasal dressing after FESS. All underwent preoperative rhinoscopy, CT of sinuses, and, after surgery, were reassessed by rhinoscopy at 2, 4, and 12 weeks in blinded fashion. A total of 44 nasal cavities (MeroGel-group) received resorbable packing, whereas the remaining 44 were packed with non-resorbable nasal dressing. At follow-up endoscopic visit, the presence of nasal synechia was evaluated as primary outcome. Moreover, the tolerability and surgical handling properties of MeroGel and its comfort were assessed by surgeons and patients. Preoperative severity of rhinosinusitis was similar in both groups. No significant adverse events were observed in all patients. Follow-up endoscopy showed a lower proportion of nasal adhesions in MeroGel-group at both 4 (P = 0.041) and 12 weeks (P < 0.001). Moreover, an improvement of other endoscopic nasal findings such as re-epithelialization, presence of granulation tissue, and appearance of nasal mucosa of nasal cavities after FESS was observed in the MeroGel-group. Tolerability and surgical handling properties of MeroGel were positively rated by clinicians and the overall patient judged comfort of MeroGel was favorable. In conclusion, MeroGel can be considered a valid alternative to standard non-resorbable nasal dressings. It is safe, well-accepted, well-tolerated, and has significant advantage of being resorbable. Moreover, it may favor improved healing in patients undergoing FESS and reduce formation of adhesions.

Sensorineural hearing loss in primary antibody deficiency disorders.

To evaluate the hearing function in patients affected by primary antibody deficiency disorders. Forty-seven patients, 25 of whom were affected by X-linked agammaglobulinemia and 22 of whom were affected by common variable immunodeficiency were evaluated with audiologic tests that included pure tone audiometry, acoustic immittance assessment and auditory brainstem-evoked response. Eighteen patients (38%), 7 with X-linked agammaglobulinemia and 11 with common variable immunodeficiency, showed sensorineural hearing loss, bilateral in 12 and unilateral in 6. Our data underline the high frequency of hearing loss in patients with antibody deficiency and suggest that a systematic audiologic evaluation should be part of the clinical care of these patients.

Long-term follow-up of children undergoing topical intranasal steroid therapy for adenoidal hypertrophy.

BACKGROUND: Encouraging results concerning chronic obstructive nasal symptoms due to adenoid in pediatric population were reported by use of intranasal steroid for short period. Up to now, no data are available about outcomes of such therapy after long-term follow-up. We evaluated the utility of mometasone furoate aqueous nasal spray in children with adenoidal hypertrophy in long-term maintenance therapy. METHODS: All children affected by adenoidal hypertrophy and undergoing successful mometasone treatment for 4 months in a preceding trial were reassessed after a mean follow-up of 28 months. Based on the duration of maintenance therapy and eventual adenoid surgical treatment, three subgroups were identified: (1) children voluntarily suspending maintenance therapy and requiring surgery (Group A); (2) children continuing maintenance therapy but undergoing surgery (Group B); and (3) children continuing maintenance therapy but not undergoing surgery (Group C). Clinical and endoscopic evaluation in each group was compared to the assessment performed after the first 4 months of treatment. RESULTS: Six patients (Group A) voluntarily suspended maintenance therapy and underwent adenoidectomy. Either the overall symptom score or choanal obstruction of this group worsened compared to the previous trial. Adenoidal surgery was performed in another three children (Group B) for ear disease. Chronic obstructive nasal symptoms and adenoid size were unchanged compared to the preceding study. Finally, 12 patients were in Group C. The overall symptom score and choanal obstruction of this group showed a further significant improvement at this stage. CONCLUSIONS: This study describes the first long-term follow-up of children undergoing mometasone furoate aqueous nasal spray treatment for adenoidal hypertrophy. Voluntary suspension of maintenance therapy favors surgery of this disorder, whereas its regular administration may lead to successful results.

Sialoendoscopy for treatment of juvenile recurrent parotitis: The Brescia experience.

OBJECTIVE: To evaluate the role of sialoendoscopy associated with steroid irrigation for juvenile recurrent parotitis (JRP) at a tertiary referral hospital. METHODS: Clinical records of patients affected by JRP and treated with operative sialoendoscopy between June 2011 and April 2017 were retrospectively reviewed. Data on demographics, number of acute episodes per year before and after surgery, characteristics of the surgical procedure, hospitalization time, and rate of complications were collected. The outcome of the procedure was measured by comparing the number of episodes of parotid swelling before and after salivary endoscopic treatment. RESULTS: Twenty-three patients for a total of 34 operative sialoendoscopies were included in the study. Before the surgical endoscopic procedure, the mean number of parotid swelling was 10 episodes per year. At sialoendoscopy, typical endoscopic findings such as mucous plugs, stenosis of the duct, intraductal debris, and pale ductal appearance were evident. All patients were discharged on the first postoperative day. A significant decrease in the number of swelling episodes per year was observed compared to the preoperative rate (p = .0004). Complete resolution of the disorder was obtained in 35% of patients. CONCLUSIONS: Operative sialoendoscopy with steroid irrigation can be considered a valid therapeutic treatment for JRP. The technique is conservative, effective, safe, and, potentially repeatable. Short hospitalization time, rapid recovery, absence of peri-operative complications, and a high rate of good outcomes are the main advantages of this treatment.

Inhaled foreign bodies in pediatric patients: review of personal experience.

BACKGROUND: Foreign bodies (FBs) are a life-threatening event in children that require early diagnosis and prompt successful management. The ideal means of FB removal is rigid bronchoscopy under general anesthesia, although the choice between spontaneous or controlled breathing and the type of drug used are still subjects of discussion. We made a review of the literature and report our experience on FB inhalation, nature and location of FB, diagnostic method, prediction, perioperative complications, type of anesthesia, ventilation and total duration of the surgical procedure. METHODS: Forty-six children undergoing rigid bronchoscopy for suspect FB aspiration were retrospectively assessed. Relevant clinical and radiological findings were retrieved. During endoscopic procedures induction and maintenance of anesthesia were performed by intravenous or volatile drugs associated with topical airway lidocaine under spontaneous breathing. RESULTS: The most common symptoms were cough and dyspnea. Radiological examination was beneficial in 34 patients. At bronchoscopy, organic and inorganic FBs were located largely in bronchial tree and removed in 40 of the 46 children. All patients maintained spontaneous ventilation using volatile and intravenous anesthesia in 22 and 24 children, respectively. The mean surgical time was 79 min. Perioperative complications such as bronchospasm, bleeding and desaturation were observed in five patients. CONCLUSIONS: FB inhalation is an uncommon life-threatening event in pediatric patients that can manifest with various symptoms. Rigid bronchoscopy is the procedure of choice for diagnosis and management of FB inhalation in pediatric patients. Spontaneous ventilation can be considered safe, using either volatile or intravenous agents. Perioperative complications were not correlated with either the choice of agent (volatile or intravenous) or the duration of surgery. A close collaboration between anesthesiologists and otorhinolaryngologists and a long-standing experience in pediatric airway emergencies are the key factors for obtaining good results.

Acute Scedosporium apiospermum Endobronchial Infection in Cystic Fibrosis.

Fungi are known pathogens in cystic fibrosis patients. A boy with cystic fibrosis boy presented with acute respiratory distress. Bronchoscopy showed airways obstruction by mucus plugs and bronchial casts. Scedosporium apiospermum was identified as the only pathogen. Bronchoalveolar lavage successfully resolved the acute obstruction. Plastic bronchitis is a new clinical picture of acute Scedosporium endobronchial colonization in cystic fibrosis patients.

Endoscopic surgery for fibrous dysplasia of the sinonasal tract in pediatric patients.

Fibrous dysplasia (FD) is a non-neoplastic, expansile lesion of unknown origin. In about one-fourth of cases this disorder affects the head and neck area, where the mandible and maxilla are the most frequently involved sites. Its localization to the ethmoid is a rare event. Since the disease slowly progresses, its management is delayed until significant clinical symptoms or non-well-tolerated aesthetic deformities are present. When required, surgery is the treatment of choice. Several external procedures have been used to manage the lesion, but recently, more conservative transnasal approaches have been proposed. We report the history of a 6-year-old boy with fibrous dysplasia of the ethmoid labyrinth that underwent successful transnasal endoscopic removal. Furthermore, an analysis of the literature is presented with particular emphasis on clinical picture, diagnosis, and treatment of this rare illness.

Tympanic membrane perforation in children: Endoscopic type I tympanoplasty, a newly technique, is it worthwhile?

OBJECTIVES: To evaluate the results of a newly introduced technique to our Department of endoscopic assisted transcanal myringoplasty applied in tympanic membrane perforation in children of any age and compare them to that of the previously standard microscopic assisted myringoplasty technique. METHODS: A retrospective study of myringoplasties performed between January 2005 and June 2014 in children suffering from chronic otitis media with perforation. In microscope-assisted cases, a transcanal approach was applied when the anterior tympanic annulus was completely visible through the ear speculum, and a postauricular approach was used in all other cases. A transcanal approach was used in all endoscopic-assisted cases. RESULTS: Between January 2005 and December 2010 and January 2011 and June 2014, 23 and 22 myringoplasties were performed by means of an operative microscope and an endoscope, respectively. Patient age varied from 5 to 16 years. Median duration of microscopic and endoscopic approaches was 90 min and 80 min (P=0.3), respectively. Hospital stay after surgery was significantly longer in the microscope group than the endoscope group (P<0.001). The intact graft success rate was 82.6% in microscopic and 90.9% in endoscopic approaches. Median postoperative air-bone gap of microscopic and endoscopic approaches was 6.2 dB and 6.6 dB, respectively (P=0.9). Neither intra- nor postoperative complications were observed. CONCLUSION: Endoscopic transcanal myringoplasty is an alternative surgical approach to traditional technique. This surgery is more conservative than microscopic approach and can be performed in all pediatric cases independently from age. Moreover, it offers comparable anatomical and functional results to the traditional surgery, and grants better comfort for the child.

Different endoscopic surgical strategies in the management of inverted papilloma of the sinonasal tract: experience with 47 patients.

OBJECTIVE: To demonstrate the potentials and limitations of three different endoscopic procedures employed for treatment of inverted papilloma (IP) of the sinonasal tract. STUDY DESIGN: Retrospective analysis of a cohort of patients treated at two University hospitals. METHODS: From January 1992 to June 2000, 47 patients with IP underwent endoscopic resection. Preoperative workup included multiple biopsies of the lesion and imaging evaluation by computed tomography or magnetic resonance imaging. Massive skull base erosion, intradural or intraorbital extension, extensive involvement of the frontal sinus, abundant scar tissue caused by previous surgery, or the concomitant presence of squamous cell carcinoma were considered absolute contraindications for a purely endoscopic approach. Three types of resection were used: ethmoidectomy with wide antrostomy and sphenoidotomy (type 1) for IPs confined to the middle meatus, medial maxillectomy with ethmoidectomy and sphenoidotomy (type 2) for IPs partially invading the maxillary sinus, and a Sturmann-Canfield operation (type 3) for IPs involving the mucosa of the alveolar recess or of the anterolateral corner of the maxillary sinus. All patients were followed by periodic endoscopic evaluations. RESULTS: Type 1, 2, and 3 resections were performed in 26, 15, and 6 patients, respectively. No recurrences were observed after a mean follow-up of 55 (range 30-132) months. One patient, who underwent a type 2 resection, developed a stenosis of the lacrimal pathways requiring endoscopic dacryocystorhinostomy. CONCLUSIONS: Our experience confirms that endoscopic surgery is an effective and safe method of treatment for most IPs. The availability of different endoscopic techniques allows the entity of the dissection to be modulated in relation to the extent of disease. Strict application of selection criteria, meticulous use of subperiosteal dissection in the involved areas, and regular follow-up evaluation are key elements for success.