The Role of Airway Management on Feeding Difficulties in Children With Pfeiffer Syndrome.

BACKGROUND: Pfeiffer syndrome is characterized by craniosynostosis, mid-face hypoplasia, broad thumbs, and often multilevel airway obstruction. Airway management is often required, including the use of positive airway ventilation, nasopharyngeal airway (NPA), or tracheostomy. OBJECTIVE: The objective of this study was to assess the impact an airway adjunct can have on feeding difficulties in children with Pfeiffer syndrome. METHODS: Retrospective review of patients diagnosed with Pfeiffer syndrome from January 1998 to January 2020 at one of England's 4 supraregional Craniofacial Units, Alder Hey Children's Hospital. Speech & Language Therapy case notes and medical notes were used to gather data, as well as the Oral Feeding Score component of the UK Craniofacial Outcome Score. RESULTS: Eleven patients were included. Six patients had no airway adjunct (55%): 3 had tracheostomy (27%) and 2 patients had NPA (18%). All patients with airway adjuncts were percutaneous endoscopic gastrostomy/percutaneous endoscopic jejunostomy fed. Those who did not require an airway adjunct had an Oral Feeding Score of 4.60 (SD: 0.49). The children who went on to have an airway adjunct had a mean preintervention Oral Feeding Score of 2.4 (SD: 0.8). The mean feeding score (postairway adjunct) in the NPA group was 2.0, compared with the tracheostomy group scoring 3.0. CONCLUSIONS: Children with Pfeiffer syndrome who require airway intervention have more significant feeding problems requiring feeding intervention. Although there were small numbers included in this study, there is a suggestion that airway adjuncts can contribute to feeding difficulties, particularly NPAs.

Variations in Paranasal Sinus Anatomy in Children With Apert Syndrome: A Radiological Analysis.

BACKGROUND: Apert syndrome is a genetic disorder characterized by craniofacial abnormalities and premature closure of the coronal sutures. The restriction of cranial development may have a subsequent effect on paranasal anatomy development. AIM: The aim of the study was to gain an understanding of paranasal sinus anatomical variations seen in children with Apert syndrome. MATERIALS AND METHODS: This was a retrospective review of computed tomography and magnetic resonance images of children with Apert syndrome from 2000 to 2020. Images were reviewed to identify anatomical variations in paranasal sinus anatomy. RESULTS: Twenty-one patients were included in the study. The most commonly seen variation was septal deviation in 86% of cases, with 60% of patients having a septal defect. The presence of protrusion or dehiscence of the infraorbital nerve, carotid canal and Vidian nerve, and presence of a concha bullosa were not observed in any patients. Keros type I was the most commonly observed olfactory fossa depth in 79% of patients, and type I Kuhn cells were observed in 83% of patients. CONCLUSIONS: To our knowledge, this is the first study which describes the prevalence of variations in paranasal sinus anatomy found in children with Apert syndrome. Septal deviation, type I Kuhn cells and Keros type I olfactory fossa depth were observed in a higher prevalence in our cohort than in the general population. As such, assessment for the presence of chronic rhinosinusitis and nasal obstruction should be evaluated as part of the multidisciplinary assessment.

Inner Ear Anomalies in Children With Apert Syndrome: A Radiological and Audiological Analysis.

INTRODUCTION: Apert syndrome is a multisystem genetic disorder typically characterized by craniosynostosis and syndactyly. Studies also report an increased incidence of hearing loss in children with Apert syndrome in comparison to the general population. The aim of this study was to gain an understanding of the inner ear radiological anatomical variations seen in children with Apert syndrome and correlate these with audiological outcomes. MATERIALS AND METHODS: This was a retrospective review of computed tomography imaging of patients with Apert syndrome. Radiological images were examined for anatomical variations in inner ear structures. These were correlated with audiological testing. RESULTS: Nineteen patients were included in the study. The most commonly observed anomaly was an absent bony window of the lateral semi-circular canal (SCC) in 11 patients (58%), followed by an enlarged lateral SCC in 12 patients (63%). This combination of anomalies was seen collectively in 42% of patients and together these give the appearance of a 'rectangular vestibular cavity'. Audiological results were available in 11 patients and 9 of these patients had a conductive hearing loss. CONCLUSION: To the authors' knowledge, this is the first study that reports radiological findings alongside audiological testing in Apert syndrome and describes the appearance of a 'rectangular vestibular cavity'.

Defining the role of surgical drainage in paediatric deep neck space infections.

OBJECTIVES: Surgical drainage is the traditional mainstay of treatment of paediatric deep neck space infection (DNSI), but recently non-operative management in selected cases has been advocated. We sought to identify any characteristics predictive of requirement for surgical intervention. DESIGN: Retrospective cohort study using receiver operating characteristics analyses. SETTING: Tertiary referral paediatric hospital. PARTICIPANTS: Children (≤16 years) with a radiologically confirmed diagnosis of retro- or parapharyngeal abscess over a ten-year period. MAIN OUTCOME MEASURES: Predictive value of clinical and radiological variables in determining the requirement for surgical intervention. Length of hospital stay (LoS) was also examined. RESULTS: Ninety-three children were studied, 15 (16.1%) of whom underwent immediate surgery, 42 (45.2%) of whom underwent delayed surgery following an initial period of conservative management, and 36 (38.7%) of whom were managed conservatively. Age, WCC and CRP were not predictive of the need for surgical drainage. Radiological abscess diameter, however, was predictive of requirement surgery (AUC = 0.85 [95% CI ± 0.09] P = 0.02), with a cut-off value of 2.5 cm determined assuming equal weight to sensitivity and specificity. All DNSIs were successfully treated with no adverse outcomes, and there was no significant difference in LoS between groups. In those managed surgically, outcome and LoS did not depend on yield of pus. CONCLUSIONS: Selected paediatric DNSIs can be successfully managed conservatively, with abscess diameter >2.5 cm a significant predictor of need for surgical intervention. Any benefit of surgery does not appear to depend on intra-operative yield of pus.

Management of the Airway in Apert Syndrome.

The management of the airway in Apert syndrome is complex and multidisciplinary. This rare syndrome, occurring in up to approximately 1 in 65,000 live births, results in airway compromise at various anatomic levels, in addition to abnormal central respiratory drive. Obstructive apneas arise because of decreased airway caliber, which may occur in the form of congenital bony nasal stenosis, choanal atresia, a deviated nasal septum, a narrowed nasopharynx, a thick long soft palate, lateral palatal swellings, and a tracheal cartilage sleeve. Central apneas in Apert syndrome arise because of raised intracranial pressure and/or Chiari malformations. The purpose of this study was to investigate our treatment methods and outcomes in optimizing the airway in this complex, rare and interesting cohort of patients who present with airway compromise. Patients with Apert syndrome were retrospectively evaluated during a period from 1990 to 2013. Treatments for obstructive apnea were dilatation of nasal airways and choanal atresia repair, adenoidectomy, tonsillectomy, early midface advancement, and noninvasive ventilation. The insertion of ventriculoperitoneal shunts, fronto-orbital advancement, and Chiari decompression aid in managing central apneas. The authors present our experience at Alder Hey Children's Hospital, Liverpool, one of the 4 Supraregional Craniofacial Units in the United Kingdom.

Management of Bell's palsy in children: an audit of current practice, review of the literature and a proposed management algorithm.

OBJECTIVE: We carried out a complete audit cycle, reviewing our management of paediatric patients with Bell's palsy within 72 h of symptom onset. Our protocol was published after the initial audit in 2009, and a re-audit was carried out in 2011. We aimed to improve our current practice in accordance with up-to-date evidence-based research on the use of steroids and antivirals. PATIENTS AND METHODS: A total of 17 patients were included in the first cycle, but only eight patients met our inclusion and exclusion criteria for the re-audit. We assessed documentation of House-Brackmann (HB) grade on presentation, initial treatment, follow-up and recovery. RESULTS: The first cycle revealed inconsistent management with steroids (41%), antivirals (6%), steroids and antivirals (6%) or nothing at all (47%). In addition, only 65% of patients were followed-up in the ear, nose and throat (ENT) clinic. Our management protocol was published in 2010, and a re-audit was completed. Our results showed 100% compliance with steroid treatment and 100% follow-up with the ENT team. A thorough literature review revealed some additional benefit from the use of antivirals. CONCLUSIONS: At present there is insufficient evidence to discount the use of steroids and antivirals. Therefore, with our new management protocol, we recommend the use of steroids in patients presenting within 72 h of symptom onset, and antivirals for patients with a HB grade of IV or higher.

Assessment of tympanic membrane perforation size using ImageJ software by ENT clinicians of different grades.

OBJECTIVE: To compare visual estimation versus ImageJ calculation of tympanic membrane perforation size in the paediatric population between clinicians of different experience. METHODS: Five images of tympanic membrane perforations in children, captured using an otoendoscope, were selected. The gold standard was the ImageJ results by one consultant otologist. Consultants, registrars and Senior House Officers or equivalent were asked to visually estimate and calculate the perforation size using ImageJ software. RESULTS: The mean difference in variation from gold standard between visual estimation and ImageJ calculation was 12.16 per cent, 95 per cent CI (10.55, 13.78) p < 0.05, with ImageJ providing a more accurate estimation of perforation. Registrars were significantly more accurate at visual estimation than senior house officers. There was no statistically significant difference in ImageJ results between the different grades. CONCLUSION: Using ImageJ software is more accurate at estimating tympanic membrane perforation size than visual assessment for all ENT clinicians regardless of experience.

Pediatric temporomandibular joint (TMJ) arthritis, an elusive complication of acute mastoiditis.

INTRODUCTION: Septic arthritis of the Temporomandibular joint (TMJ) is a rare complication of acute middle ear infection. Presentation is elusive and could be easily missed. Often diagnosis is made only with consequential development of TMJ ankylosis. This study intends to characterize patients and course of disease and suggest a diagnostic and therapeutic strategy. METHODS: Retrospective review of all children diagnosed with TMJ arthritis and/or TMJ ankylosis secondary to acute middle ear infection, treated in a tertiary pediatric medical center between the years 2005 and 2021. RESULT: Seven patients were identified with otogenic TMJ arthritis. Median age at presentation was 1.14 years (IQ range 1.1-1.5). All seven were diagnosed with acute mastoiditis. CT scans demonstrated TMJ related collections in 5/7 and intracranial complications in 3/7. Treatment included cortical mastoidectomy for 5/7. One patient had a concomitant surgical washout of the TMJ. Two patients had drainage only of subperiosteal collections. Six of the seven patients went on to develop TMJ ankylosis that presented within a median of 2.8 years (IQ range 2.6-3.9) after the episode of acute mastoiditis. All six patients presented with trismus and facial growth anomalies, and all but one required surgery to release the ankylosis. CONCLUSION: Otogenic TMJ arthritis develops mostly in young children with acute mastoiditis and intratemproal and/or intracranial suppuration. Imaging is helpful in making the diagnosis, with TMJ related collections on CT being the most common finding. TMJ ankylosis can develop within a few years and present with trismus and abnormal facial growth. Cortical mastoidectomy does not seem to prevent ankylosis. It remains unclear whether focused treatment to the TMJ or physiotherapy could be beneficial in that.

Clinical outcomes of sinogenic intracranial suppuration: the Alder Hey experience.

OBJECTIVES: The aim of this study was to present a series of cases of sinogenic intracranial suppuration in a pediatric tertiary referral center and to review the long-term outcomes of the patients. METHODS: We performed a retrospective review of the hospital database and the patient case notes. RESULTS: Over 10 years, 14 patients were identified. The clinical presentations at the time of admission tended to include headache, vomiting, pyrexia, limb weakness, and decreased level of consciousness, in decreasing order of frequency. Sinonasal symptoms such as discharge and obstruction were only present in 36% and 21% of cases, respectively. The most common intracranial complication was subdural empyema in the frontal lobe region. The mortality rate was 21% (3 of 14). The remaining 11 patients remained alive at latest follow-up. The average follow-up period after hospital discharge was 19 months (median, 15 months; range, 6 to 64 months). No significant complications were noted in 4 patients, who had returned to normal daily activities at 6 months of follow-up. A significant proportion of patients who survived have some form of neurologic sequelae, although 64% of cases became asymptomatic in the 12 months following hospital discharge. CONCLUSIONS: The significant risk of morbidity and mortality of this disease requires a multidisciplinary approach that is best delivered at a tertiary referral center.

Acute otitis externa: Consensus definition, diagnostic criteria and core outcome set development.

OBJECTIVE: Evidence for the management of acute otitis externa (AOE) is limited, with unclear diagnostic criteria and variably reported outcome measures that may not reflect key stakeholder priorities. We aimed to develop 1) a definition, 2) diagnostic criteria and 3) a core outcome set (COS) for AOE. STUDY DESIGN: COS development according to Core Outcome Measures in Effectiveness Trials (COMET) methodology and parallel consensus selection of diagnostic criteria/definition. SETTING: Stakeholders from the United Kingdom. SUBJECTS AND METHODS: Comprehensive literature review identified candidate items for the COS, definition and diagnostic criteria. Nine individuals with past AOE generated further patient-centred candidate items. Candidate items were rated for importance by patient and professional (ENT doctors, general practitioners, microbiologists, nurses, audiologists) stakeholders in a three-round online Delphi exercise. Consensus items were grouped to form the COS, diagnostic criteria, and definition. RESULTS: Candidate COS items from patients (n = 28) and literature (n = 25) were deduplicated and amalgamated to a final candidate list (n = 46). Patients emphasised quality-of-life and the impact on daily activities/work. Via the Delphi process, stakeholders agreed on 31 candidate items. The final COS covered six outcomes: pain; disease severity; impact on quality-of-life and daily activities; patient satisfaction; treatment-related outcome; and microbiology. 14 candidate diagnostic criteria were identified, 8 reaching inclusion consensus. The final definition for AOE was 'diffuse inflammation of the ear canal skin of less than 6 weeks duration'. CONCLUSION: The development and adoption of a consensus definition, diagnostic criteria and a COS will help to standardise future research in AOE, facilitating meta-analysis. Consulting former patients throughout development highlighted deficiencies in the outcomes adopted previously, in particular concerning the impact of AOE on daily life.