Head and neck sarcoma: three-year data from a tertiary referral centre.

INTRODUCTION: Sarcomas of the head and neck are neoplasms arising from the embryonic mesenchyme. They are rare and heterogeneous in nature and are associated with significant morbidity and mortality. This study evaluates patients referred to the Oxford Sarcoma Service, a tertiary referral centre. METHODS: Patients discussed over a three-year period were included. Medical records were analysed using the electronic patient record database. Data were acquired on a range of domains, including: demographics, histopathology, treatment modality, recurrence, mortality, survival, etc. RESULTS: Thirty-two eligible patients, 21 male and 11 female, were identified with a mean age of 58 years; 26 out of 32 patients had high-grade sarcomas. The commonest histological subtype was chondrosarcoma (8/32). Twenty-two underwent planned multidisciplinary team surgical resection after biopsy and staging: negative margins were noted in 9, with close and involved margins in 5 and 8, respectively. Local recurrence was noted in 13 and 6 had metastatic disease out of the 32 eligible patients. Mortality was noted in 10 out of 32 patients. Mean survival was 69.5 months. Five-year overall survival was 64%. Surgery demonstrated statistically significant improvement in survival (p=0.0095). There were no significant differences in survival, recurrence or marginal status between methods of adjuvant or neoadjuvant therapy. CONCLUSION: Outcomes of head and neck sarcomas are inferior compared with other types of sarcoma. The nature of the complex surrounding anatomy presents unique challenges in surgical management. This in turn affects rates of local recurrence and prognosis. Therefore, it is critical that they are managed in tertiary, specialist centres with a multidisciplinary approach.

Systematic review and meta-analysis of tracheostomy outcomes in COVID-19 patients.

A systematic review and meta-analysis of the entire COVID-19 Tracheostomy cohort was conducted to determine the cumulative incidence of complications, mortality, time to decannulation and ventilatory weaning. Outcomes of surgical versus percutaneous and outcomes relative to tracheostomy timing were also analysed. Studies reporting outcome data on patients with COVID-19 undergoing tracheostomy were identified and screened by 2 independent reviewers. Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were followed. Outcome data were analysed using a random-effects model. From 1016 unique studies, 39 articles reporting outcomes for a total of 3929 patients were included for meta-analysis. Weighted mean follow-up time was 42.03±26 days post-tracheostomy. Meta-analysis showed that 61.2% of patients were weaned from mechanical ventilation [95%CI 52.6%-69.5%], 44.2% of patients were decannulated [95%CI 33.96%-54.67%], and cumulative mortality was found to be 19.23% [95%CI 15.2%-23.6%] across the entire tracheostomy cohort. The cumulative incidence of complications was 14.24% [95%CI 9.6%-19.6%], with bleeding accounting for 52% of all complications. No difference was found in incidence of mortality (RR1.96; p=0.34), decannulation (RR1.35, p=0.27), complications (RR0.75, p=0.09) and time to decannulation (SMD 0.46, p=0.68) between percutaneous and surgical tracheostomy. Moreover, no difference was found in mortality (RR1.57, p=0.43) between early and late tracheostomy, and timing of tracheostomy did not predict time to decannulation. Ten confirmed nosocomial staff infections were reported from 1398 tracheostomies. This study provides an overview of outcomes of tracheostomy in COVID-19 patients, and contributes to our understanding of tracheostomy decisions in this patient cohort.

The presentation, clinical features, complications, and treatment of congenital dacryocystocele.

PurposeTo determine the incidence and presenting features of congenital dacryocystocele in the United Kingdom. To report on those cases complicated by dacryocystitis, respiratory compromise, and the treatment undertaken.MethodsA prospective observational study of cases of congenital dacryocystocele presenting in the United Kingdom between September 2014 and October 2015. Infants <3 months of age presenting with a cystic swelling in the medial canthal area were included. Cases were identified via the British Ophthalmology Surveillance Unit (BOSU) reporting system.ResultsA total of 49 cases were reported during the study period. This gives an incidence of 1 in 18 597 live births. There was a 71% response rate to the questionnaire. The average age at presentation was 16.94 days. Dacryocystoceles were unilateral in 91% of cases. Dacryocystitis was a complicating factor in 49% of patients and 17% had respiratory distress. Uncomplicated dacryocystocele responded well to conservative measures in 86%. Surgical intervention was required in 23% of patients. Those cases complicated by dacryocystitis (29%) and nasal obstruction (17%) were more likely to require surgical intervention compared to those with dacryocystocele alone (14%). Digital massage appears to reduce the likelihood of requiring surgical intervention. The mean time to resolution was 19 days.ConclusionsCongenital dacryocystocele is a rare presentation in the United Kingdom. Dacryocystitis and respiratory compromise commonly complicate a dacryocystocele. The use of digital massage as an early intervention is advocated and conservative measures may be sufficient in cases of uncomplicated dacryocystocele.

ABO incompatible renal transplantation following lung transplantation.

We present management strategies utilised for the first case of an urgent live-donor ABO incompatible B blood group renal transplant, in a patient with a prior A blood group lung transplant for cystic fibrosis. Three years on, renal function is excellent and stable, whilst lung function has improved.

A multicentre audit of GDPs knowledge of orthodontic retention.

OBJECTIVE: To determine GDP knowledge and willingness to supervise orthodontic retention and provide replacements retainers. DESIGN: An audit sampling GDPs from six centres within England (Bradford, Cambridge, Burton-Upon-Trent, Croyden, Norwich and Plymouth). A gold standard of 100% of GDPs should be aware of commonly used retainers and be able to provide replacements was selected. METHOD: Overall, 1,053 postal questionnaires were sent to local GDPs. The questions covered knowledge and provision of various retainers, practitioner background and education. GDP satisfaction with the information provided by the orthodontist at discharge was also explored. RESULTS: Five hundred and two questionnaires were received (response rate of 48%). The majority of GDPs (64%) were trained in the UK. Awareness of vacuum-formed, Hawley and fixed retainers was generally high. A significantly smaller number of GDPs were willing to prescribe, fit or review the retainers. The most common reasons for reluctance in provision were insufficient knowledge, financial and time constraints. Over two thirds (72%) of GDPs would like further training on retention. CONCLUSION: This audit highlights a need for increased training at undergraduate and postgraduate levels to update practitioners about contemporary retention practice. Better communication is required from orthodontists to GDPs to ensure that on discharge the dentist is aware of the retainer type and retention regime.

Evolving practice: X-linked agammaglobulinemia and lung transplantation.

X-linked agammaglobulinemia (XLA) is a rare primary humoral immunodeficiency syndrome characterized by agammaglobulinemia, recurrent infections and bronchiectasis. Despite the association with end-stage bronchiectasis, the literature on XLA and lung transplantation is extremely limited. We report a series of 6 XLA patients with bronchiectasis who underwent lung transplantation. Short-term outcomes were excellent however long-term outcomes were disappointing with a high incidence of pulmonary sepsis and chronic lung allograft dysfunction (CLAD).

Coeliac disease is more common in children with high socio-economic status.

AIM: There are a number of genetic and environmental factors that are associated with an increased risk of developing coeliac disease. Our aim was to determine whether socio-economic deprivation increases or reduces the development of the disease. METHODS: A cross-sectional study identified all children <16 years old diagnosed with coeliac disease in the same tertiary paediatric centre between January 1995 and December 2011. Data, including age at diagnosis and postcode, were collected and linked with the quintile rank of the Welsh Index of Multiple Deprivation score 2008, a measure of socio-economic status. RESULTS: We included 232 patients and identified a graded association between the prevalence of coeliac disease and socio-economic deprivation, which showed a higher rate in children living in more affluent areas. The largest difference was between the lowest deprivation level (rate/1000 = 1.16) and the highest deprivation level (rate/1000 = 0.49). CONCLUSION: In our population, coeliac disease was more common in children in the higher socio-economic groupings. The reasons for this are not clear, but perhaps both the 'hygiene hypothesis' and the health seeking behaviours of parents with high socio-economic status are possible factors in the more frequent diagnosis of coeliac disease in this group.

Oxygen therapy in preterm infants.

Despite being the most widely used and vital therapy in neonatology, optimal strategies for the use of oxygen in preterm infants remain controversial. Achieving the balance between attaining adequate tissue oxygenation and avoiding oxygen toxicity is challenging. There remains a paucity of clear evidence based guidance for clinicians on safe oxygen saturation targets. What does seem apparent is that these targets vary over time in the life of a preterm infant. This article summarises the evidence behind current practice of oxygen monitoring and administration from the first few minutes after birth, through to the acute neonatal and later convalescent periods. Finally, we review the use of home oxygen for preterm infants with bronchopulmonary dysplasia including administration and weaning from domically home oxygen.

Electromagnetic inductance plethysmography to measure tidal breathing in preterm and term infants.

Tidal breathing measurements which provide a non-invasive measure of lung function in preterm and term infants are particularly useful to guide respiratory support. We used a new technique of electromagnetic inductance plethysmography (EIP) to measure tidal breathing in infants between 32 and 42 weeks postconceptional age (PCA). Tidal breathing was measured in 49 healthy spontaneously breathing infants between 32 and 42 weeks PCA. The weight-corrected tidal volume (V(T) ) and minute volume (MV) decreased with advancing PCA (V(T) 6.5 ± 1.5 ml/kg and MV 0.44 ± 0.04 L/kg/min at 32-33 weeks, respectively; 6.3 ± 0.9 ml/kg and 0.38 ± 0.02 L/kg/min at 34-36 weeks; and 5.1 ± 1.1 ml/kg and 0.28 ± 0.02 L/kg/min at term, V(T) P < 0.001 and MV P < 0.01 for 32-33 weeks PCA vs. term; V(T) P = 0.016 and MV P = 0.015 for 34-36 weeks PCA vs. term). Respiratory frequency and the phase angle decreased significantly with advancing PCA but the flow parameter t(PTEF) /t(E) did not change significantly. Using a new technique to measure tidal breathing parameters in newborn infants, our data confirms its usability in clinical practice and establishes normative data which can guide future respiratory management of newborn infants.

Ca2+ activation of cytosolic calpain induces the transition from apoptosis to necrosis in neutrophils with externalized phosphatidylserine.

Physiologically, apoptotic neutrophils are ingested before they undergo necrosis. However, failure of ingestion will lead to necrosis of neutrophils and the unregulated release of neutrophil-derived pathogenic molecules, such as protease and hydrolases. Understanding the mechanism of postapoptotic necrosis is thus clearly important. Here, we monitored the apoptotic-to-necrotic transition in individual-aged human neutrophils in vitro by imaging fluorescent probes for externalized PS, cytosolic Ca(2+), and membrane integrity. This showed that prenecrotic-aged neutrophils with externalized PS had a significantly elevated cytosolic-free Ca(2+) level. A further unregulated Ca(2+) influx into PS-externalized neutrophils always preceded the necrotic transition. Ca(2+) elevation was not simply a consequence of aging, as PS externalization was not uniform in similarly aged neutrophil populations. PS-externalized neutrophils could be induced to undergo necrosis experimentally by simply elevating cytosolic Ca(2+) further with ionomycin. This effect was observed only in neutrophils that had externalized PS, and was independent of the time after their isolation from blood (i.e., in vitro age). As pharmacological inhibition of calpain-1 inhibition significantly reduced this CAIN, it was concluded that the apoptotic-to-necrotic transition was a consequence of uncontrolled calpain activation that resulted from Ca(2+) overload in PS-externalized neutrophils.

Antenatal and postnatal management of congenital cystic adenomatoid malformation.

Congenital thoracic malformations (CTMs) are a heterogeneous group of rare disorders that may involve the airways or lung parenchyma. The authors have focused on the condition that causes the most controversy, namely, congenital cystic adenomatoid malformation (CCAM). The reported incidence is 3.5 and 0.94 per 10,000 live births for CTMs and CCAMs respectively. Ultrasound is the antenatal imaging modality of choice for screening for CCAMs whilst magnetic resonance imaging is complimentary for morphological and volumetric evaluation of the foetal lung. Most CCAMs are detected antenatally with only a small proportion presenting postnatally. Only a few CCAMs cause foetal problems, with foetal hydrops being the best predictor of death. Although many CCAMs regress during pregnancy, most remain detectable postnatally by CT scans. Surgical excision of symptomatic lesions is relatively straightforward, but management of asymptomatic lesions is controversial. Some surgeons adopt a "wait and see" approach operating only on those patients who develop symptoms, but others operate on asymptomatic patients usually within the first year of life. Due to the potential of malignant transformation, children should have long term follow up. There is an urgent need to delineate the natural history of antenatally detected CCAMs to guide future management.

Congenital diaphragmatic hernia.

Infants with congenital diaphragmatic hernia (CDH) have significant mortality and long-term morbidity. Only 60-70% survive and usually those in high-volume centres. The current Task Force, therefore, has convened experts to evaluate the current literature and make recommendations on both the antenatal and post-natal management of CDH. The incidence of CDH varies from 1.7 to 5.7 per 10,000 live-born infants depending on the study population. Antenatal ultrasound scanning is routine and increasingly complemented by the use of magnetic resonance imaging. For isolated CDH, antenatal interventions should be considered, but the techniques need vigorous evaluation. After birth, management protocols are often used and have improved outcome in nonrandomised studies, but immediate intubation at birth and gentle ventilation are important. Pulmonary hypertension is common and its optimal management is crucial as its severity predicts the outcome. Usually, surgery is delayed to allow optimal medical stabilisation. The role of minimal invasive post-natal surgery remains to be further defined. There are differences in opinion about whether extracorporeal membrane oxygenation improves outcome. Survivors of CDH can have a high incidence of comorbidities; thus, multidisciplinary follow-up is recommended. Multicentre international trials are necessary to optimise the antenatal and post-natal management of CDH patients.

Estimation of tidal ventilation in preterm and term newborn infants using electromagnetic inductance plethysmography.

Tidal volume (VT) measurements in newborn infants remain largely a research tool. Tidal ventilation and breathing pattern were measured using a new device, FloRight, which uses electromagnetic inductive plethysmography,and compared simultaneously with pneumotachography in 43 infants either receiving no respiratory support or continuous positive airway pressure (CPAP).Twenty-three infants were receiving CPAP (gestational age 28 ± 2 weeks, mean ± SD) and 20 were breathing spontaneously (gestational age 34 ± 4 weeks). The two methods were in reasonable agreement, with VT (r2 = 0.69) ranging from 5 to 23 ml (4­11 ml kg−1) with a mean difference of 0.4 ml and limit of agreement of −4.7 to + 5.5 ml. For respiratory rate, minute ventilation,peak flow and breathing pattern indices, the mean difference between the two methods ranged between 0.7% and 5.8%. The facemask increased the respiratory rate (P < 0.001) in both groups with the change in VT being more pronounced in the infants receiving no respiratory support. Thus, FloRight provides an easy to use technique to measure term and preterm infants in the clinical environment without altering the infant's breathing pattern.

Role of pulmonary infection in the development of chronic lung disease of prematurity.

We studied the role of ante- and post-natal infection in the development of chronic lung disease (CLD) of prematurity. 192 newborn infants (61 term and 131 pre-term of <34 weeks gestation: 88 with respiratory distress syndrome, 35 developed CLD and eight died) were recruited. 16S ribosomal RNA (rRNA) genes were identified by PCR of DNA isolated from 840 gastric and lung fluid samples. Ureaplasma spp. were also cultured. Presence of 16S rRNA genes (OR 1.6, 95% CI 1.2-2.2) and Ureaplasma spp. (OR 3.6, 95% CI 1.7-7.7) was significantly associated with the development of CLD. This association remained if the 16S rRNA genes and Ureaplasma spp. were first identified within the first 3 days of life (OR 2.4 (95% CI 1.4-4.1) and 3.8 (95% CI 1.4-10.0), respectively) or if first identified after 3 days of age (OR 1.7 (95% CI 1.1-2.8) and OR 5.1 (95% CI 1.3-19.8), respectively). Peak lung fluid interleukin (IL)-6 and IL-8 were significantly associated with presence of microbes (p<0.0001 and p=0.0001, respectively) and development of CLD (p=0.003 and 0.001, respectively). Both early and late microbial presence in neonatal lung fluid samples was significantly associated with the development of CLD suggesting that both ante- and post-natal infection play a role in the development of CLD.

Non-resolving periapical inflammation: a malignant deception.

AIM: To report a case of oral non-Hodgkin's lymphoma with a delayed diagnosis. SUMMARY: Non-Hodgkin's lymphoma of the oral cavity is an uncommon but important condition. Early diagnosis is complicated when the presenting signs and symptoms are similar to those of odontogenic infections. This report describes the case of a 38-year-old female patient who presented to her dentist complaining of pain in her upper jaw. Subsequent dental treatment, including extraction, root canal treatment and apicectomy including biopsy were carried out by the patient's dentist and local dental hospital. Nine months elapsed before a more extensive surgical exploration established a diagnosis of lymphoma.

Paediatrics in Berlin.

The aim of this report is to describe the highlights of the European Respiratory Society annual congress in Berlin, Germany. The best abstracts in asthma and allergy, cystic fibrosis, respiratory infection, paediatric and neonatal intensive care, paediatric investigative techniques (in particular respiratory physiology and bronchoscopy) and respiratory epidemiology are presented and set in the context of the current literature.

The viral aetiology of croup and recurrent croup.

BACKGROUND: Historically croup was subdivided into classic "viral" croup with associated viral upper respiratory tract infections, and recurrent or spasmodic croup where asthma and allergies were thought more important. METHODS: All children admitted to the University Hospital of Wales with croup in 2003 were eligible. Baseline demographics including croup score were recorded and per-nasal swabs taken for virus detection by RT-PCR. Recurrent croup was defined as at least one other admission for croup in the preceding or following 3 years. RESULTS: Sixty (29.4%) children entered the study, and a viral pathogen was detected in 41 (68%). There was no significant difference in the rate of virus detection between those with single episode croup and recurrent croup. CONCLUSIONS: The aetiologies of viral and recurrent croup appear similar.

Multiple self-healing squamous cell carcinomas of the face.

We report the case of a 73-year-old woman who developed multiple self-healing squamous cell carcinomas over a 6-year period. Some were excised and others resolved spontaneously.

A four year retrospective study of 1,062 patients presenting with maxillofacial emergencies at a specialist paediatric hospital.

AIMS: To examine the nature of maxillofacial injuries that presented to the Birmingham Children's Hospital according to aetiology, incidence and characteristics of patients. METHODS: The maxillofacial unit at Diana Princess of Wales Birmingham Children's Hospital serves a catchment area of 5.2 million. We examined all children who presented with maxillofacial trauma from 2002 to 2006. Details including characteristics of patients and aetiology and nature of injury, were entered prospectively into a database. RESULTS: The total number of patients who presented was 1062. The ratio of boys to girls was 2:1. The main reason for attendance was a fall (70%). 149 (17%) patients presented after interpersonal violence. Soft tissue injuries accounted for 70% of injuries and 14% presented with a maxillofacial fracture. CONCLUSIONS: Our data indicate that the range and mechanism of presenting injuries have not changed within the time frame. Despite public perception of increasing interpersonal violence, this was not reflected in our series of patients.