The effect of soft palate reconstruction with the da Vinci robot on middle ear function in children: an observational study.

Cleft palate is associated with a high prevalence of middle ear dysfunction, even after palatal repair. The aim of this study was to evaluate the effects of robot-enhanced soft palate closure on middle ear functioning. This retrospective study compared two patient groups after soft palate closure with a modified Furlow double-opposing Z-palatoplasty technique. Dissection of the palatal musculature was performed using a da Vinci robot in one group and manually in the other. Outcome parameters were otitis media with effusion (OME), tympanostomy tube use, and hearing loss during 2 years of follow-up. At 2 years post-surgery, the percentage of children with OME had reduced significantly to 30% in the manual group and 10% in the robot group. The need for ventilation tubes (VTs) decreased significantly over time, with fewer children in the robot group (41%) than those in the manual group (91%) needing new VTs during postoperative follow-up (P = 0.026). The number of children presenting without OME and VTs increased significantly over time, with a faster increase in the robot group at 1 year post-surgery (P = 0.009). Regarding hearing loss, significantly lower hearing thresholds were recorded in the robot group from 7 to 18 months postoperatively. To conclude, beneficial effects of robot-enhanced surgery were recorded, suggesting a faster recovery when the soft palate was reconstructed using the da Vinci robot.

Non-primary CMV infection not always innocent. A case-report and literature review.

OBJECTIVES: Cytomegalovirus (CMV) is the most common infectious cause of congenital malformations. CMV infections are frequently symptomatic in case of a primary infection during pregnancy. Generally, maternal immunity protects the newborn against a symptomatic course of the CMV infection. METHODS: We present clinical information and medical images of a neonate with non-primary congenital CMV infection. RESULTS: We report the case of a severe congenital infection in a newborn clinically presenting with diffuse petechia, facial dysmorphisms, respiratory distress, hepatomegaly and hypotonia. The girl was born to a mother with CMV immunity. Blood results demonstrated thrombocytopenia and elevated transaminases. Brain MRI revealed ventricular dilatation and germinolytic cysts, compatible with CMV infection. Auditory brain stem response testing was abnormal. CMV culture of saliva was positive. This led to the diagnosis of a severe congenital CMV infection due to a non-primary maternal infection. Antiviral treatment with valganciclovir was initiated immediately and continued for 6 months. CONCLUSION: Our case illustrates that, even when the mother was demonstrated CMV immune, congenital CMV infection is still an important differential diagnostic consideration in neonates presenting with congenital cerebral abnormalities, thrombocytopenia and/or hearing loss. ABBREVIATIONS: ABR: auditory brainstem responses; CMV: Cytomegalovirus; CPAP: Continuous Positive Airway Pressure; CRP: C-Reactive Protein; dBnHL: Decibel Above Normal Adult Hearing Level; IV: intravenously; MRI: Magnetic Resonance Imaging; SGA: Small for Gestational Age; SNHL: Sensorineural Hearing Loss.

Effect of body weight on upper airway findings and treatment outcome in children with obstructive sleep apnea.

OBJECTIVE/BACKGROUND: Surgical interventions for obstructive sleep apnea (OSA) are less effective in obese than in normal-weight children. However, the mechanisms that underpin this relationship are not fully understood. Therefore, this study aimed to explore how body weight influences upper airway collapse and treatment outcome in children with OSA. METHODS: We conducted a retrospective analysis of prospectively collected data on polysomnography, drug-induced sleep endoscopy (DISE), and treatment outcome in otherwise healthy children with OSA. Associations between body mass index (BMI) z-score and upper airway collapse during DISE were assessed using logistic regression modelling. Treatment success was defined as obstructive apnea-hypopnea index (oAHI) < 5 events/hour and cure as oAHI < 2 events/hour with obstructive apnea index < 1 event/hour. RESULTS: A total of 139 children were included [median (Q1─Q3); age 4.5 (3.1─8.4) years; BMI z-score 0.3 (-0.8 to 1.4); oAHI 10.8 (6.8─18.0) events/hour]. Twenty-five of them were overweight and 21 were obese. After adjusting for age and history of upper airway surgery, BMI z-score was significantly correlated with circumferential upper airway collapse during DISE (odds ratio 1.67; 95% confidence interval 1.12─2.65; P = 0.011). Outcome of DISE-directed treatment was similar in normal-weight (success: 91.4%; cure: 78.5%), overweight (success: 88.0%; cure: 80.0%), and obese (success: 90.5%; cure: 76.5%) children. Children with circumferential collapse responded better to continuous positive airway pressure than to (adeno)tonsillectomy. CONCLUSION: Increasing body weight is associated with circumferential upper airway collapse during DISE and, accordingly, may require treatment strategies other than (adeno)tonsillectomy.

Positional obstructive sleep apnea in children: prevalence and risk factors.

PURPOSE: Positional (supine dependent) obstructive sleep apnea (POSA) affects about 55% of adults with obstructive sleep apnea (OSA). We aimed to study the prevalence and risk factors for POSA in children. METHODS: Cross-sectional analysis of data obtained in 171 children with moderate to severe OSA confirmed by polysomnography (PSG) performed over a 2-year period. POSA is defined by an obstructive apnea-hypopnea index (oAHI) in the supine position ≥ 2× oAHI in the non-supine position. RESULTS: The overall prevalence of POSA was 18.7%. Children with POSA were significantly older (p < 0.001), had a higher prevalence of obesity (p = 0.04), a lower tonsil score (p = 0.049), and less severe OSA (lower oAHI) (p = 0.02) compared to children without POSA, while age was the only significant independent predictor of POSA. The ratio AHI supine to AHI non-supine was not significantly higher during REM than during NREM sleep in children with POSA. CONCLUSIONS: POSA is less common in children compared to adults and the prevalence of POSA increases with age. Although OSA worsens during REM sleep, this was not observed for POSA. Future studies should investigate the prevalence of POSA in specific subgroups and upper airway characteristics of POSA in children.

Treatment of sleep-disordered breathing with positional therapy: long-term results.

PURPOSE: The aim of the present study was to assess the efficacy of a sleep position trainer (SPT) in patients with an established diagnosis of positional obstructive sleep apnea and to evaluate the adherence after 1-year follow-up. METHODS: Polysomnography (PSG) was performed at baseline and after 1 year of SPT use. Patients received questionnaires to assess treatment satisfaction and subjective adherence. Data on objective adherence and number of vibrations initiated by the SPT were collected from the SPT device. RESULTS: Nine out of 58 patients stopped using the SPT during the first year of treatment (16%). Thirty-four middle-aged and overweight patients underwent a PSG after 1 year of SPT use (male/female ratio, 28/6; overall apnea/hypopnea index (AHI), 16/h). A significant reduction in overall AHI to 6/h was observed using treatment (p < 0.001). The median percentage of supine sleep decreased significantly to 1% with SPT (p < 0.001). The mean objective SPT use in 28 patients was 7.3 ± 0.9 h/night and 69 ± 26% of the nights. Furthermore, 75% of the patients reported a better sleep quality since the start of SPT treatment. CONCLUSIONS: Long-term treatment with the SPT was found to be effective in reducing overall AHI. Time spent sleeping in supine position was reduced to almost zero in the continuing users. Patient satisfaction was high when using the SPT.

Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study.

BACKGROUND: The prevalence and causes of sensorineural hearing loss (SNHL) in children with Down syndrome (DS) are poorly delineated. OBJECTIVE: To describe the prevalence, severity, laterality and underlying etiology of SNHL in a cohort of children with DS. METHODS: A cross-sectional study was performed among all children with DS followed at the multidisciplinary Downteam of the Antwerp University Hospital. Patients' characteristics, risk factors for hearing loss, audiometric data and results of an etiological work-up were collected. RESULTS: Among 291 patients in follow-up, 138 patients (47.4%) presented with hearing loss. In the majority this was caused by middle ear effusion and only 13 patients (4.5%) had sensorineural hearing loss, 7 boys and 6 girls with a mean age of 14.4 ±â€¯7.4 years. Hearing loss was bilateral in 8 cases. Hearing loss severity was graded as mild in 38.5%, moderate in 30.8% and profound in 30.8% of the patients. An etiological work-up was completed in 9 children. Four patients presented with single sided deafness due to cochlear nerve deficiency. One patient had a genetic cause and in 2 patients the hearing loss was attributed to excessive noise exposure. The etiology of hearing loss was unknown in 6 patients. CONCLUSION: Sensorineural hearing loss is uncommon in children with DS with a prevalence of 4.5%. Etiological work-up may allow identifying a specific underlying cause. Cochlear nerve deficiency was found in 4 children with DS and single sided deafness.

The Optiflow™ interface for chronic CPAP use in children.

Continuous positive airway pressure (CPAP) is being increasingly used in children of all age ranges. The limited number of commercially available masks especially in infants and young children may complicate its use and compliance. In this report, we describe our experience with the use of the Optiflow™ (Fisher and Paykel Healthcare) Nasal Cannula attached to a regular CPAP device in the setting of chronic CPAP use. This interface consists of a nasal cannula and was originally designed for the delivery of high-flow oxygen therapy. We could show an objective improvement in breathing parameters in several children selected for CPAP mainly because of obstructive sleep apnea syndrome (OSAS). However, this interface cannot be used for bilevel non-invasive ventilation due to insufficient triggering.

Sleep disorders during childhood: a practical review.

Sleep disorders are a common problem during childhood. The consequences are variable, and sleep disorders can influence medical, psychological and developmental aspects of the growing child. It is important to recognize sleep disorders and to treat them correctly. We discuss common sleep disorders during childhood using the 3rd edition of the International Classification of Sleep Disorders. We analyze the different sleep disorders from a clinical approach and provide an overview of adequate treatment options.Conlusion: This review discusses common sleep disorders during childhood using the 3rd edition of the International Classification of Sleep Disorders. We analyze the different sleep disorders from a clinical approach and provide an overview of adequate treatment options. What is known: • Sleep disorders are a common problem during childhood. • The consequences are variable, and sleep disorders can influence medical, psychological, and developmental aspects of the growing child. What is new: • Pediatricians should routinely screen for sleep and sleep disorders. • It is important to recognize sleep disorders and to treat them correctly.

Drug-induced sedation endoscopy in surgically naïve infants and children with obstructive sleep apnea: impact on treatment decision and outcome.

PURPOSE: Adenotonsillectomy (AT) is the first-line treatment for obstructive sleep apnea (OSA) in children irrespective of clinical upper airway (UA) findings. We aimed to investigate whether drug-induced sedation endoscopy (DISE) changes treatment decision and outcome in otherwise healthy children and infants with OSA. METHODS: Retrospective analysis of prospectively collected data on polysomnography, DISE, and treatment in surgically naïve, otherwise healthy infants (n = 34) and children (n = 75) with OSA. Treatment success is defined as post-treatment obstructive apnea/hypopnea index (oAHI) < 5 h-5-1, and cure is defined as oAHI < 2 h-1. RESULTS: Based upon UA findings during DISE, AT was performed in 22 infants and 57 children. oAHI improved from 16.5 h-1 (8.1-28.3) to 0.8 h-1 (0.3-4.2) (p = 0.01) in infants and from 28.6 h-1 (23.4-34.9) to 0.7 h-1 (0.4-1.8) (p < 0.001) in children. AT was successful in 84.2% of infants and 91.4% of children. A cure was obtained in 68.4% of infants and 78.7% of children. DISE changed the treatment decision in 1/3rd of infants and 1/4th of children, and they did not undergo AT. In the non-AT group, isolated adenoidectomy/tonsillectomy or non-surgical treatment was successful in 86.6% of children and in 100% of infants. Cure was achieved in 66.6% of children and 75% of infants. CONCLUSIONS: DISE performed in otherwise healthy and surgically naïve infants and children with OSA altered the therapeutic decision making in up to 1/3rd to 1/4th of the cases and resulted in comparable treatment outcomes as standard treatment by AT. The present data suggest that DISE may provide individually tailored treatment of OSA in otherwise healthy infants and children.

Drug-induced sedation endoscopy in children <2 years with obstructive sleep apnea syndrome: upper airway findings and treatment outcomes.

Few data are available about the pattern of upper airway (UA) obstruction in children <2 years with obstructive sleep apnea syndrome (OSAS). Also, the role of adenoidectomy versus adenotonsillectomy (AT) is poorly defined in this age group. We performed drug-induced sedation endoscopy (DISE) in young OSAS children to investigate the pattern of UA obstruction and the value of DISE in therapeutic decision making. Retrospective analysis of ≤2-year-old children undergoing DISE-directed UA surgery. OSAS severity and the treatment outcomes were documented by polysomnography. Data are available for 28 patients, age 1.5 years (1.3-1.8), BMI-z score 0.5 (-0.7 to 1.3) with severe OSAS, obstructive apnea/hypopnea index (oAHI) 13.8/hr (7.5-28.3). All but 3 had (>50%) obstruction at the level of the adenoids, and all but 5 had (>50%) tonsillar obstruction. DISE-directed treatment consisted of adenoidectomy (n = 4), tonsillectomy (n = 1), and AT (n = 23). There was a significant improvement in respiratory parameters. Twenty children (71.4%) had a postoperative oAHI <2/hr. None had palatal or tongue base obstruction. Five children had a circumferential UA narrowing (hypotonia), 2 of them had residual OSAS. DISE showed a collapse of the epiglottis in 6 and late-onset laryngomalacia in 4. These findings did not affect surgical outcome. Adenotonsillar hypertrophy is the major cause of UA obstruction, and DISE-directed UA surgery was curative in 71,4% of children ≤2 years. We suggest that DISE may be helpful in surgical decision making. Circumferential UA narrowing may result in less favorable surgical outcomes.

Adenotonsillectomy to treat obstructive sleep apnea: Is it enough?

Although adenotonsillectomy is the first line treatment for children with obstructive sleep apnea syndrome (0SAS),1 improvement in objectively documented outcomes is often inadequate and a substantial number of children have residual disease. Early recognition and treatment of children with persistent OSAS is required to prevent long-term morbidity. The management of these children is frequently complex and a multidisciplinary approach is required as most of them have additional risk factors for OSAS and comorbidities. In this paper, we first provide an overview of children at risk for persistent disease following adenotonsillectomy. Thereafter, we discuss different diagnostic modalities to evaluate the sites of persistent upper airway obstruction and the currently available treatment options. Pediatr Pulmonol. 2017;52:699-709. © 2016 Wiley Periodicals, Inc.

Stridor caused by a blue laryngeal mass in an infant: case report.

Stridor caused by.a blue laryngeal mass in an infant: case report. We describe an infant who had inspiratory stridor that began a few weeks after birth. Flexible upper airway endoscopy while the infant was awake showed a large blue mass at the posterior glottis, above the esophageal inlet. The lesion was only visible upon crying or coughing; it was undetectable when we examined the infant in the supine position during spontaneous breathing and did not respond to systemic treatment with propranolol. Based upon these typical features, we established a diagnosis of pressure- dependent postcricoid mass.

Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic.

UNLABELLED: Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency. CONCLUSION: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients. WHAT IS KNOWN: • Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates. • Little data are available on the prevalence and risk factors for ANSD in healthy newborns. What is new: • The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births. • In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.

Drug-induced sedation endoscopy in pediatric obstructive sleep apnea syndrome.

AIM: To describe the pattern of upper airway (UA) obstruction during drug-induced sedation endoscopy (DISE) and to evaluate the outcome of DISE-directed treatment. METHODS: Prospective study of DISE in surgically naive obstructive sleep apnea syndrome (OSAS) children without syndromic comorbidity or craniofacial abnormalities. Treatment was individually tailored according to UA findings during DISE and polysomnographic data. Reported values are median (lower-upper quartile). RESULTS: Thirty-seven children aged 4.1 years (2.1-6.0), with body mass index z-score 0.3 (-0.9 to 0.9), and obstructive apnea-hypopnea index (oAHI) 9.0/h (6.1-19.3) were included. Adenotonsillar obstruction was found in 33 cases (89%) as an isolated entity or as part of a multi-level obstruction. These children were treated with adenotonsillectomy (n = 28), adenoidectomy (n = 3), or tonsillectomy (n = 2). The remaining four patients received non-surgical treatment. Pre-postoperative polysomnographic data in 22 patients showed a significant improvement in oAHI from 8.6/h (6.7-20.7) to 1.0/h (0.6-2.0) (P = 0.001). Only two of these 22 children had residual OSAS (oAHI ≥ 5/h), indicating a success rate of 91%. CONCLUSIONS: Based on UA findings during DISE, a non-surgical treatment was proposed for 11% of children. A 91% success rate was obtained in those treated with (adeno)tonsillectomy. These data suggest that DISE may be helpful to identify patients most likely to benefit from UA surgery.

Nasal symptoms in patients with obstructive sleep apnea and their impact on therapeutic compliance with continuous positive airway pressure.

Continuous positive airway pressure (CPAP) is the first line treatment for moderate to severe obstructive sleep apnea syndrome. Despite the high effectiveness of this treatment, its use is often limited by suboptimal compliance and/or intolerance. Nasal side effects are considered a major cause of low therapeutic compliance. At present, there are no data to predict which patients will develop CPAP intolerance caused by nasal symptoms and only few studies looked at the effect of treatment of nasal symptoms on CPAP compliance. The first section of this review focuses on the impact of nasal symptoms (pre-existing or as a side effect of CPAP) on CPAP compliance/intolerance. The second section deals with the effect of nasal symptom treatment on CPAP compliance.

Intracranial complications of acute otitis media and Gradenigo's syndrome.

We describe the case of a 12-year-old girl with acute otitis media complicated by acute mastoiditis, epidural empyema, thrombosis of the sigmoid sinus and paralysis of the abducens nerve. The patient underwent a mastoidectomy on the left side combined with drainage of the epidural empyema through an extended burr holl and received intravenous antibiotics for 6 weeks and anticoagulation for 12 weeks. This report discusses the intracranial complications of acute otitis media, which were a common problem before the advent of adequate antibiotic drugs but have become rare since their introduction.

Functional respiratory imaging as a tool to assess upper airway patency in children with obstructive sleep apnea.

OBJECTIVE: We aim to investigate if anatomical and functional properties of the upper airway using computerized 3D models derived from computed tomography (CT) scans better predict obstructive sleep apnea (OSA) severity than standard clinical markers. METHODS: Consecutive children with suspected OSA underwent polysomnography, clinical assessment of upper airway patency, and a CT scan while awake. A three-dimensional (3D) reconstruction of the pharyngeal airway was built from these images, and computational fluid dynamics modeling of low inspiratory flow was performed using open-source software. RESULTS: Thirty-three children were included (23 boys; mean age, was 6.0±3.2y). OSA was diagnosed in 23 patients. Children with OSA had a significantly lower volume of the overlap region between tonsils and the adenoids (median volume, 1408 mm compared to 2173 mm; p=0.04), a lower mean cross-sectional area at this location (median volume, 69.3mm(2) compared to 114.3mm2; p=0.04), and a lower minimal cross-sectional area (median volume, 17.9 mm2 compared to 25.9 mm2; p=0.05). Various significant correlations were found between several imaging parameters and the severity of OSA, most pronounced for upper airway conductance (r=-0.46) (p<0.01) for correlation between upper airway conductance and the apnea-hypopnea index. No differences or significant correlations were observed with clinical parameters of upper airway patency. Preliminary data after treatment showed that none of the patients with residual OSA had their smallest cross-sectional area located in segment 3, and this frequency was significantly lower than in their peers whose sleep study normalized (64%; p=0.05). CONCLUSION: Functional imaging parameters are highly correlated with OSA severity and are a more powerful correlate than clinical scores of upper airway patency. Preliminary data also showed that we could identify differences in the upper airway of those subjects who did not benefit from a local upper airway treatment.

Auditory neuropathy: a challenge for diagnosis and treatment.

In current terminology, auditory neuropathy spectrum disorder (ANSD) is a disease involving the disruption of the temporal coding of acoustic signals in auditory nerve fibres, resulting in the impairment of auditory perceptions that rely on temporal cues. There is debate about almost every aspect of the disorder, including aetiology, lesion sites, and the terminology used to describe it. ANSD is a heterogeneous disease despite similar audiological findings. The absence of an auditory brainstem response (ABR) and the presence of otoacoustic emissions (OAE) suggest an ANSD profile. However, to determine the exact anatomical site of the disorder, more in-depth audiological and electrophysiological tests must be combined with imaging, genetics and neurological examinations. Greater diagnostic specificity is therefore needed to provide these patients with more adequate treatment.

An unusual tumour causing neonatal respiratory distress.

PROBLEM: We present the case of a term neonate referred shortly after birth because of breathing and feeding difficulties. METHODOLOGY: Fiber-endoscopic examination of the nasal cavity showed a pendulating mass in the nasopharynx. RESULTS: A complete surgical resection was performed and the baby recovered completely. Microscopic examination of the mass showed an overlying non-keratinized squamous cell lining with an atypical cell population in some fragments. Histological features were compatible with a high-grade epithelial tumour like a midline carcinoma, but a final diagnosis of a salivary gland anlage tumour was established. CONCLUSION: Flexible fiber endoscopy is the method of choice for examining the nasal passages and oropharynx in neonates with respiratory distress. Congenital salivary gland anlage tumour is a rare cause of neonatal nasal obstruction; it is benign and complete excision results in a cure. Histologically, it may mimic a malignant tumour owing to the high mitotic index.

Specific medical and surgical treatment for chronic inflammatory diseases in children.

Treatment for chronic inflammatory conditions in children should take into account the specific pathophysiological and clinical processes underlying these disorders. These guidelines provide a framework for both the medical and surgical treatment of chronic inflammatory diseases such as otitis media, allergic rhinitis and chronic rhinosinusitis, chronic inflammation of tonsils and adenoids, and laryngitis. In addition, the role of vaccinations and immunomodulatory therapies is discussed. Whenever possible, the evidence levels for specific treatments comply with the Oxford Levels of Evidence.