Patient prioritisation of impact items to develop the patient-reported impact of dermatological diseases (PRIDD) measure: European Delphi data.

BACKGROUND: The Global Research on the Impact of Dermatological Diseases (GRIDD) project is developing a patient-reported measure of the impact of dermatological disease on the patient's life called Patient Reported Impact of Dermatological Diseases (PRIDD). We developed a list of 263 potential impact items through a global qualitative interview study with 68 patients. We next conducted a Delphi study to seek consensus on which of these items to prioritize for inclusion in PRIDD. This study aims to explore patterns in demographic (e.g. country) and clinical variables (e.g. disease group) across the impacts ranked as most important to European dermatology patients. METHODS: We conducted a modified, two rounds Delphi study, testing the outcomes from the previous qualitative interview study. Adults (≥18 years) living with a dermatological disease were recruited through the International Alliance of Dermatology Patient Organizations' (GlobalSkin) membership network. The survey consisted of a demographic questionnaire and 263 impact items and was available in six languages. Quantitative data were collected using ranking scales and analysed against a priori consensus criteria. Qualitative data were collected using free-text responses and a Framework Analysis was conducted. European data were obtained, and descriptive statistics, including multiple subgroup analyses, were performed. RESULTS: Out of 1154 participants, 441 Europeans representing 46 dermatological disease from 25 countries participated. The results produced a list of the top 20 impacts reported by European patients, with psychological impacts accounting for the greatest proportion. CONCLUSION: This study identified what patients consider to be the most important issues impacting their lives as a result of their dermatological disease. The data support previous evidence that patients experience profound psychological impacts and require psychological support. The findings can inform research, clinical practice and policy by indicating research questions and initiatives that are of most benefit to patients.

Reduced growth performance in gilt progeny is not improved by segregation from sow progeny in the grower-finisher phase.

Gilt progeny (GP) are born and weaned lighter than sow progeny (SP) and tend to have higher rates of mortality and morbidity. This study quantified the lifetime growth performance differences between GP and SP and, additionally, evaluated whether segregating GP and SP in the grower-finisher period compared to mixing them within common pens reduced this variation. It was hypothesised that GP would be lighter than SP at every stage and segregation would improve growth performance of both GP and SP. All piglets born to 61 gilts (parity 1) and 47 sows (parities 2 to 7; mean 3.5 ± 0.2) were allocated to four treatments at 10 weeks of age: (i) GP housed together (GG), (ii) GP mixed (M) with SP (GM), (iii) SP housed together (SS) and (iv) SP mixed with GP (SM). The GM and SM pigs were housed together in common pens after movement into the grower-finisher facility. Individual live weight of all progeny was recorded at birth, weaning (WWT), 10 weeks of age (10WT) and sale (SWT). Individual hot carcass weight (HCW), fat depth at the head of the last rib (P2) and dressing percentage were measured at slaughter. Gilt progeny were lighter at birth (P = 0.038), weaning (P < 0.001) and through to sale (P = 0.001) than SP. Nursery and grower-finisher performance differences in GP were highly attributable to their lower WWT compared to SP (P < 0.001 when fitted as a covariate). Segregation of GP and SP increased grower-finisher average daily gain (ADG) in SP but decreased ADG and SWT in GP (P < 0.10). Segregated SP had increased average daily feed intake but only in males (P = 0.007); HCW (P < 0.001) and P2 fat depth (P = 0.055) were higher in mixed female GP, but there was no difference (P > 0.10) in female SP, or in males. In conclusion, GP were lighter at every stage than SP and differences after weaning were highly related to the lighter WWT of GP. Under the conditions of this study, overall segregation of GP and SP showed no consistent advantages in growth performance for both groups and differed significantly between males and females.

The multi-disciplinary management of complex congenital and acquired tracheo-oesophageal fistulae.

AIM OF THE STUDY: Complex tracheo-oesophageal fistulae (TOF) are rare congenital or acquired conditions in children. We discuss here a multidisciplinary (MDT) approach adopted over the past 5 years. METHODS: We retrospectively collected data on all patients with recurrent or acquired TOF managed at a single institution. All cases were investigated with neck and thorax CT scan. Other investigations included flexible bronchoscopy and bronchogram (B&B), microlaryngobronchoscopy (MLB) and oesophagoscopy. All cases were subsequently discussed in an MDT meeting on an emergent basis if necessary. MAIN RESULTS: 14 patients were referred during this study period of which half had a congenital aetiology and the other half were acquired. The latter included button battery ingestions (5/7) and iatrogenic injuries during oesophageal atresia (OA) repair. Surgical repair was performed on cardiac bypass in 3/7 cases of recurrent congenital fistulae and all cases of acquired fistulae. Post-operatively, 9/14 (64%) patients suffered complications including anastomotic leak (1), bilateral vocal cord paresis (1), further recurrence (1), and mortality (1). Ten patients continue to receive surgical input encompassing tracheal/oesophageal stents and dilatations. CONCLUSIONS: MDT approach to complex cases is becoming increasingly common across all specialties and is important in making decisions in these difficult cases. The benefits include shared experience of rare cases and full access to multidisciplinary expertise.

Oral propranolol in the treatment of proliferating infantile haemangiomas: British Society for Paediatric Dermatology consensus guidelines.

BACKGROUND: Infantile haemangiomas (IH) are the most common vascular tumours of infancy. Despite their frequency and potential complications, there are currently no unified U.K. guidelines for the treatment of IH with propranolol. There are still uncertainties and diverse opinions regarding indications, pretreatment investigations, its use in PHACES (posterior fossa malformations-haemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe) syndrome and cessation of treatment. OBJECTIVES: To provide unified guidelines for the treatment of IH with propranolol. METHODS: This study used a modified Delphi technique, which involved an international treatment survey, a systematic evidence review of the literature, a face-to-face multidisciplinary panel meeting and anonymous voting. RESULTS: The expert panel achieved consensus on 47 statements in eight categories, including indications and contraindications for starting propranolol, pretreatment investigations, starting and target dose, monitoring of adverse effects, the use of propranolol in PHACES syndrome and how to stop treatment. CONCLUSIONS: These consensus guidelines will help to standardize and simplify the treatment of IH with oral propranolol across the U.K. and assist in clinical decision-making.

Validation of the Airway-Dyspnoea-Voice-Swallow (ADVS) scale and Patient-Reported Outcome Measure (PROM) as disease-specific instruments in paediatric laryngotracheal stenosis.

OBJECTIVE: To validate the Airway-Dyspnoea-Voice-Swallow (ADVS) instrument as a disease-specific Patient-Reported Outcome Measure in paediatric laryngotracheal stenosis. DESIGN: Prospective observational study. SETTING: A quaternary referral centre for complex airway disease. PARTICIPANTS: Forty-eight patients (30 males) with a mean age of 49 ± 49 months who underwent laryngotracheal surgery or microlaryngoscopy and bronchoscopy (MLB) following laryngotracheal surgery. MAIN OUTCOME MEASURES: Airway-Dyspnoea-Voice-Swallow summary scale and Patient-Reported Outcome Measure (PROM), Paediatric Quality of Life (PedsQL) scale, Paediatric Voice Handicap Index (pVHI) and Lansky performance scale were administered to patients before and 6-8 weeks following airway examination/surgery. RESULTS: Most patients (73%) had intubation-related subglottic stenosis, and 60% of patients had prior airway treatments. The majority of patients (77%) had more than one major chronic morbidity, and the commonest procedures were diagnostic MLB (49%), followed by airway dilation (29%). Cronbach-α value for the ADVS PROM was 0.71 overall and 0.85, 0.86 and 0.64 for the dyspnoea, voice and swallow domains, respectively. Rank correlations between Dyspnoea, Voice and Swallow summary scale and PROM scores were 0.83, 0.71 and 0.81, respectively (P < 0.0001). For those patients undergoing diagnostic MLB, pre- and post-examination scores were highly correlated (intraclass correlations >0.75). There was a significant rank correlation between ADVS PROM score and Lansky performance score (r = -0.68; P < 0.0001). There were significant correlations between PROM score and PedsQL (r = -0.57; P < 0.0001) and between voice domain of the PROM and pVHI (r = 0.78; P < 0.0001). There were strong correlations between Myer-Cotton stenosis severity and dyspnoea scale and PROM score (r = 0.68; P < 0.0001). There were significant differences in voice and swallow ADVS scales and PROM scores between patients with and without concomitant laryngeal/oesophageal pathology. Patient age and presence of high dyspnoea and swallowing PROM scores were independently associated with poorer quality of life and performance status. CONCLUSIONS: These series of observations validate the ADVS instrument as a disease-specific outcome measure for paediatric laryngotracheal stenosis. Dyspnoea and swallowing dysfunction appear to have the greatest impact on quality of life. More widespread adoption of the ADVS instrument could help create a shared language for outcomes communication and benchmarking for children with this complex condition.

Tissue-Engineered Tracheal Replacement in a Child: A 4-Year Follow-Up Study.

In 2010, a tissue-engineered trachea was transplanted into a 10-year-old child using a decellularized deceased donor trachea repopulated with the recipient's respiratory epithelium and mesenchymal stromal cells. We report the child's clinical progress, tracheal epithelialization and costs over the 4 years. A chronology of events was derived from clinical notes and costs determined using reference costs per procedure. Serial tracheoscopy images, lung function tests and anti-HLA blood samples were compared. Epithelial morphology and T cell, Ki67 and cleaved caspase 3 activity were examined. Computational fluid dynamic simulations determined flow, velocity and airway pressure drops. After the first year following transplantation, the number of interventions fell and the child is currently clinically well and continues in education. Endoscopy demonstrated a complete mucosal lining at 15 months, despite retention of a stent. Histocytology indicates a differentiated respiratory layer and no abnormal immune activity. Computational fluid dynamic analysis demonstrated increased velocity and pressure drops around a distal tracheal narrowing. Cross-sectional area analysis showed restriction of growth within an area of in-stent stenosis. This report demonstrates the long-term viability of a decellularized tissue-engineered trachea within a child. Further research is needed to develop bioengineered pediatric tracheal replacements with lower morbidity, better biomechanics and lower costs.

Nasal dermoids in children: a proposal for a new classification based on 103 cases at Great Ormond Street Hospital.

OBJECTIVES: Nasal dermoids are rare developmental anomalies seen in children. This study reports the largest case series of 103 patients seen in a quaternary specialist unit over a 10-year period. We report the surgical and radiological findings and propose a new classification system, which clearly describes the extent of the lesions, thus allowing better surgical planning. METHODS: A retrospective review of case notes was conducted. Data collection included demographics, initial presentation, site of lesion, pre-operative CT and MRI imaging, surgical procedure, intraoperative findings (including depth of lesion), complications and recurrence. Surgical findings were correlated with radiological findings. RESULTS: A total of 103 patients were included in the study. The mean age at presentation was 29 months. 89% of children presented with a naso-glabellar or columellar lesion and 11% had a medial canthal lesion. All the patients underwent preoperative imaging and were treated with surgical excision. 58 children had superficial lesions, 45 had subcutaneous tracts extending to varying depths. Of these, 38 had intraosseous extension into the frontonasal bones, eight extended intracranially but remained extradural and two had intradural extension. There was good correlation between radiological and surgical findings. The superficial lesions were locally excised. The lesions with intraosseous tracts were removed via open rhinoplasty and the frontonasal bones drilled for access. Intracranial extension was approached either via a bicoronal flap and frontal craniotomy or the less invasive anterior small window craniotomy. CONCLUSIONS: This report describes the largest published cases series of nasal dermoids. The cases demonstrate the presenting features and the variable extent of the lesions. The new proposed classification; superficial, intraosseous, intracranial extradural and intracranial intradural, allows precise surgical planning. In the presence of intracranial extension, the low morbidity technique of using a brow incision and small window anterior craniotomy avoids the more invasive and commonly used bicoronal flap and frontal craniotomy.

Anterior tongue reduction surgery for paediatric macroglossia.

OBJECTIVE: Anterior tongue reduction is indicated when macroglossia causes problems with oral hygiene, airway compromise, deglutition, articulation or orthognathic complications. Causes of macroglossia include hypothyroidism, mucopolysaccharide and lipid storage disease, lymphangioma, haemangioma, neurofibroma, and muscular macroglossia. This paper presents an 11-year experience of anterior tongue reduction at Great Ormond Street Hospital. METHOD: Retrospective study of patient medical records identified from the hospital ENT database. Anterior wedge resection was the preferred technique. RESULTS: Anterior tongue reduction was performed on 18 patients, due to cystic hygroma with tongue involvement (nine patients), Beckwith-Wiedemann syndrome (eight) and Down's syndrome (one). Anterior wedge resection was preferred, using electrocautery in the majority, except for four cases involving CO2 laser. All but one patient had a good surgical outcome (i.e. tongue in mouth at rest). One patient subsequently required multiple laser procedures for recurrent macroglossia. CONCLUSION: Anterior tongue reduction can be a safe procedure, with limited post-operative morbidity, consistently resulting in good surgical outcomes and improvement in macroglossia symptoms. Speech development does not appear to be adversely affected.

Single stage laryngotracheal reconstruction using endoscopic posterior grafting in isolated posterior glottic stenosis in children - how we do it.

OBJECTIVE: Posterior glottic stenosis is a well recognised though rare condition. A wide range of treatment options have been proposed including endoscopic laser techniques and open techniques with grafting. The objective of this study was to present our experience of single stage laryngotracheal reconstruction with endoscopic technique of placement of posterior graft in isolated posterior glottic stenosis. METHODS: This study was conducted at the Department of Paediatric Otolaryngology at Great Ormond Street Hospital, London. The senior authors (ALT, DMA) were involved in the management of two children with isolated posterior glottic stenosis operated on as single stage reconstruction. RESULTS: The two children in this small series had isolated posterior glottic stenosis. The aetiology in both the cases was previous prolonged intubation. Both patients underwent a successful placement of the posterior graft endoscopically, though it was a challenging task because of space constraints. None of these children required an intra-operative or post-operative tracheostomy. There were no complications in these patients. At follow up endoscopic examinations, both these patients have had their airway widened successfully. CONCLUSION: Single stage laryngotracheal reconstruction with endoscopic placement of posterior graft in cases with isolated posterior glottic stenosis is a good alternative to open surgical techniques, although is technically a challenging procedure.

Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children.

OBJECTIVE: There has been much recent focus on sclerotherapy treatment of lymphatic malformations with OK432. Surgical treatment however can have a number of advantages, including complete curative excision. The aim of this study was to evaluate the results of surgical excision as the primary (first) treatment for this condition. This group includes a number of children with very extensive disease as well as some with smaller lesions. METHODS: Prospectively collected database with additional information from medical records of children with cervicofacial lymphangiomas treated over 10 years at a tertiary paediatric referral centre. For this study only children who underwent surgical excision as the primary treatment modality were included. RESULTS: Total of 118 children with lymphatic malformations were treated under the care of the senior author over a 10 year period. Of these 53 patients, who underwent surgical excision as the primary treatment modality for cervicofacial lymphatic malformations were included in this study. Forty-one patients who underwent sclerotherapy as the initial treatment were excluded. Also excluded were 6 patients who underwent thoracic surgery and 18 who were treated conservatively. The majority of the patients (41, 77.3%) underwent only a single surgical procedure (36 - surgical excision, 5 - laser excision). At the first follow up after the primary surgery, the result was complete resolution of symptoms in 29 patients, near complete resolution in 13 patients (together 79.3%) and partial response in 11 (19.7%) patients. Twenty-three patients with disease localised only to the neck, all (100%) had a complete or near complete resolution of the disease after the primary surgery. Complete/near complete response was achieved in 98% cases with macrocystic disease, regardless of the location. Minor complications occurred in 11.3% patients. No permanent nerve weaknesses occurred. CONCLUSION: Cervicofacial lymphatic malformations in children should be managed in a multidisciplinary setting. Surgery remains a very important treatment modality. The majority of patients (80%) in this study had complete or near complete resolution with one surgical procedure. Isolated neck lesions have the best outcomes (100% resolution in this study). Patients with macrocystic disease, achieved complete or near complete resolution in 97% of cases, regardless of the location. Some children with extensive disease will need multiple treatments. Surgical excision as the primary treatment modality in selected cases is safe and reliable technique and has good aesthetic and functional outcomes in experienced hands.

The follow-up of patients with head and neck cancer: an analysis of 1,039 patients.

In all cancer specialities, there has been much debate about the best follow-up regime. The provision of a service that meets high standards whilst being cost-effective is increasingly pertinent. The objectives of the study were to examine: whether routine follow-up facilitates early diagnosis and recurrence; whether there is a cohort of patients who require a more intensive follow-up regime; whether follow-up should be customised to individual patients. A total of 1,039 consecutive outpatient consultations were prospectively analysed in a multicentre study. All adult patients who had undergone multidisciplinary, multimodality management for head and neck cancer were included. The case mix was representative of all head and neck tumour sites and stages. Suspicion of recurrence was noted in 10% (n = 96/951) of patients seen routinely. This rose to 68% (n = 60/88) for the subset of patients who had requested an appointment. Most recurrences were found within the first follow-up year (n = 64/156, 54%). Only 0.3% (n = 3/1,039) of asymptomatic patients attending routine appointments were suspected of having a recurrence, and two (0.2%) were found to have an actual recurrence following investigation. Of the total number of patients reporting a new suspicious symptom, recurrence was suspected in 56% (n = 152/270). Patients thus had a 98.1% sensitivity to raising suspicion for a recurrence based on the reporting of new symptoms with a 99.6% negative predictive value. Our data show that the efficiency of the current follow-up regime at detecting suspected recurrence of head and neck cancer is low, suggesting the need for a customised, more focused follow-up regime, tailored to individual cases. Patient education and close relationships with clinicians and allied health-care professionals are essential for early diagnosis and management of cancer recurrence. Follow-up regimes within the first year should be most intensive as recurrence is most likely within this time, and it serves to alleviate patient anxiety in the early post-treatment period. More research needs to be carried out to investigate the role of patient self-reporting and surveillance of cancer recurrence.

Adult nasal glioma presenting with visual loss.

OBJECTIVES: We report a rare case of a nasal glioma found incidentally in an adult, presenting with visual loss, optic nerve oedema and proptosis. CASE REPORT: A 41-year-old woman presented with bilateral proptosis, impairment in visual acuity (6/60 bilaterally) and loss of colour vision. Computed tomography and magnetic resonance imaging showed proptosis, bilateral optic nerve swelling and a heterogeneous mass occupying the left nasal cavity and extending through a skull base defect into the anterior cranial fossa. Biopsy confirmed a nasal glioma. Treatment with intravenous dexamethasone resolved the proptosis, and the patient's visual acuity recovered to 6/9 bilaterally. At the multidisciplinary team meeting, it was felt that the nasal glioma probably represented an incidental finding and was not directly responsible for the patient's proptosis and transient visual loss. CONCLUSION: To our knowledge, this is the first report in the English language literature of adult nasal glioma presenting with visual loss. The management of nasal gliomas in adults is contentious and the relevant literature is reviewed. This case was managed conservatively with regular follow up.

Nonlinear vortex development in rotating flows.

We present the results of a combined experimental and numerical investigation into steady secondary vortex flows confined between two concentric right circular cylinders. When the flow is driven by the symmetric rotation of both end walls and the inner cylinder, toroidal vortex structures arise through the creation of stagnation points (in the meridional plane) at the inner bounding cylinder or on the mid-plane of symmetry. A detailed description of the flow regimes is presented, suggesting that a cascade of such vortices can be created. Experimental results are reported, which visualize some of the new states and confirm the prediction that they are stable to (mid-plane) symmetry-breaking perturbations. We also present some brief results for the flows driven by the rotation of a single end wall. Vortex structures may also be observed at low Reynolds numbers in this geometry. We show that standard flow visualization methods lead to some interesting non-axisymmetric particle paths in this case.

The palmar locking compression plate is biomechanically comparable to the dorsal pi plate for dorsally comminuted, intraarticular wrist fractures.

A clinically appropriate fracture model and testing regimen were used to test the null hypothesis that a palmarly applied locking plate was inferior to a dorsally applied Pi plate in the stabilisation of dorsally comminuted intraarticular wrist fractures. Sixteen standardised fractures of Synbone models of the radius were stabilised using either a palmar locking compression T plate (the experimental group) (n=8) or a dorsally applied Pi plate (the control group) (n=8). The constructs were tested on an Instron materials testing machine. Deformation was monitored during 500 loading cycles to 200 N. The mean permanent deformation and stiffness favoured the palmar locking compression T plate over the dorsal Pi plate (P=0.036). However, the absolute difference was only 0.5 mm. Such a small difference is unlikely to be clinically detectable and, therefore, we conclude that there is no clinically significant difference between the two types of fixation.

The bisphosphonate acute phase response: rapid and copious production of proinflammatory cytokines by peripheral blood gd T cells in response to aminobisphosphonates is inhibited by statins.

The bisphosphonates are a novel class of drug that have been registered for various clinical applications worldwide. Bisphosphonates, and in particular the aminobisphosphonates (nBPs), are known to have a number of side-effects including a rise in body temperature and accompanying flu-like symptoms that resemble a typical acute phase response. The mechanism for this response has been partially elucidated and appears to be associated with the release of tumour necrosis factor (TNF)alpha and interleukin (IL)6, although the effector cells that release these cytokines and the mechanism of action remain enigmatic. Here, we show that the nBP-induced acute phase response differs from the typical acute phase response in that CD14+ cells such as monocytes and macrophages are not the primary cytokine producing cells. We show that by inhibiting the mevalonate pathway, nBPs induce rapid and copious production of TNFalpha and IL6 by peripheral blood gammadelta T cells. Prior treatment with statins, which inhibit 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase, blocks nBP-induced production of these proinflammatory cytokines by gammadelta T cells and may offer a means of avoiding the associated acute phase response. In addition, our findings provide a further mechanism for the anti-inflammatory effects attributed to inhibitors of HMG CoA reductase.