Swallowing impairment in older persons following burn injury: Validation of incidence and predictive factors for dysphagia.

Background: Early retrospective data identify that dysphagia is common in older persons with burn injury, suggesting a rate of 47 %, and that it is associated with medical, burn, and nutritional outcomes.

Aims: To prospectively (1) explore the incidence, (2) describe associations, and (3) evaluate risk factors for dysphagia in patients ≥ 75 years old hospitalised with burn injury.

Methods: All patients > 75 years old admitted to Concord-Repatriation-General-Hospital with burn injury over 4 years (2019-2023) were assessed for dysphagia on presentation and were continually monitored throughout their admission.

The seven-fold rise in incidence of Stevens-Johnson syndrome & toxic epidermal necrolysis: Associations with COVID-19 and the vaccine.

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) is a rare, potentially life threatening mucocutaneous hypersensitivity reaction resulting in desquamation of the skin and mucosa. These patients are managed on burns units due to the widespread desquamation. We report the largest case series of participants developing SJS/TEN in the setting of recent COVID infection or vaccination.

Frontofacial Surgery: Reducing Infection with the Development and 6-Year Outcome of a Frontofacial Protocol.

Background: Frontofacial surgery (FFS) creates a communication between the cranial and nasal cavities and is associated with significant infection risk. After a cluster of infections affecting patients undergoing FFS, a root cause analysis of index cases was undertaken, but no specifically remedial causes were identified. Basic principles incorporating known risk factors for the prevention of surgical-site infection were then applied to the creation of a perioperative management protocol.

Does the Mutation Type Affect the Response to Cranial Vault Expansion in Children With Apert Syndrome?

III.

Alginate Dressings Continuously for 14 Days on Uncontaminated, Superficial, Partial Thickness Burns.

Calcium alginate dressings are commonly used on split-thickness skin donor sites, where they are typically removed after 14 days. Alginates have been used previously on superficial, partial thickness burns, but changed every 3 to 5 days. In this study, alginates were applied to superficial, partial thickness burns on adults within 36 hours of injury, then left intact for up to 14 days.

Increased self-immolation frequency and severity during the COVID-19 pandemic.

Objective: To determine whether the increased restrictions, isolation and stressors associated with COVID-19 led to an increase in rates or severity of self-immolation burn injuries.

Design: Retrospective review of a prospectively-collected database of New South Wales burn patients, comparing 2020 data with the preceding 5 years.

Setting: Both adult units in the New South Wales Statewide Burn Injury Service (Concord Repatriation General Hospital and Royal North Shore Hospital).

Spring-assisted posterior vault expansion-a single-centre experience of 200 cases.

Purpose: Children affected by premature fusion of the cranial sutures due to craniosynostosis can present with raised intracranial pressure and (turri)brachycephalic head shapes that require surgical treatment. Spring-assisted posterior vault expansion (SA-PVE) is the surgical technique of choice at Great Ormond Street Hospital for Children (GOSH), London, UK. This study aims to report the SA-PVE clinical experience of GOSH to date.

Management of foot burns with an emphasis on high-risk groups.

Background: Burns injuries are dynamic and evolve over time. Burn injuries to the feet present with seasonal variation, affecting clinical outcomes for certain high-risk groups. Although they affect a very small percentage of the body, burn injuries to the feet can affect mobility, morbidity and rehabilitation, particularly in patients with diabetes.

FACE-Q Craniofacial Module: Part 1 validation of CLEFT-Q scales for use in children and young adults with facial conditions.

Background: The CLEFT-Q includes 12 independently functioning scales that measure appearance (face, nose, nostrils, teeth, lips, jaws), health-related quality of life (psychological, social, school, speech distress), and speech function, and an eating/drinking checklist. Previous qualitative research revealed that the CLEFT-Q has content validity in noncleft craniofacial conditions. This study aimed to examine the psychometric performance of the CLEFT-Q in an international sample of patients with a broad range of facial conditions.

Modification of the cartilaginous framework for autologous ear reconstruction: Construction of a stable complete ring framework with grander highs and lows.

Background: Successful microtia reconstruction involves fabrication of a framework with well-defined ear subunits. Tragal definition and deep conchal bowl are key elements to produce a natural well-defined and contoured ear. We describe a modification in the cartilage framework with the purpose of increasing framework stability, tragal definition and conchal bowl depth.

Concepts Important to Patients With Facial Differences: A Qualitative Study Informing a New Module of the FACE-Q for Children and Young Adults.

Objective: The concepts important to children and young adults who undergo treatments for facial differences are not well-defined. Measurement of treatment outcomes from the patient's perspective is necessary to ensure goals of treatment are met. We aimed to identify concepts important to children and young adults with facial differences through a qualitative study.

Foot burns: A comparative analysis of diabetic and non-diabetic patients.

Introduction: Foot burns represent a small part of the body with many challenges. The impact of diabetes on clinical outcomes adds further issues in management that clinicians must consider in their management. These factors have serious implications on morbidity and long term sequelae.

Design and manufacturing of a patient-specific nasal implant for congenital arhinia: Case report.

Arhinia (congenital absence of the nose) is a congenital rare disease, which has been reported in less than 60 cases in the literature. It consists of the absence of external nose, nasal cavities and olfactory apparatus and is generally associated with midline defects, microphthalmia, blepharophimosis and hypotelorism. Aesthetic problems as well as associated functional anomalies can potentially impact on the development and interpersonal relationships of the child at a later stage in life.

A population-specific material model for sagittal craniosynostosis to predict surgical shape outcomes.

Sagittal craniosynostosis consists of premature fusion (ossification) of the sagittal suture during infancy, resulting in head deformity and brain growth restriction. Spring-assisted cranioplasty (SAC) entails skull incisions to free the fused suture and insertion of two springs (metallic distractors) to promote cranial reshaping. Although safe and effective, SAC outcomes remain uncertain.

In search of a single standardised system for reporting complications in craniofacial surgery: a comparison of three different classifications.

Comparing complication rates between centres is difficult due to the lack of unanimous criteria regarding what adverse events should be defined as complications and how these events should be compiled. This study analysed all adverse events in a cohort of craniofacial (CF) operations over a 10-year period and applied three different scales (Clavien-Dindo, Leeds and Oxford) for systematic comparison. A total of 1023 consecutive CF procedures in 641 patients was identified.

Syndromic Craniosynostosis: Complexities of Clinical Care.

Patients with syndromic craniosynostosis have a molecularly identified genetic cause for the premature closure of their cranial sutures and associated facial and extra-cranial features. Their clinical complexity demands comprehensive management by an extensive multidisciplinary team. This review aims to marry genotypic and phenotypic knowledge with clinical presentation and management of the craniofacial syndromes presenting most frequently to the craniofacial unit at Great Ormond Street Hospital for Children NHS Foundation Trust.

ERF-related craniosynostosis: The phenotypic and developmental profile of a new craniosynostosis syndrome.

Mutations in the ERF gene, coding for ETS2 repressor factor, a member of the ETS family of transcription factors cause a recently recognized syndromic form of craniosynostosis (CRS4) with facial dysmorphism, Chiari-1 malformation, speech and language delay, and learning difficulties and/or behavioral problems. The overall prevalence of ERF mutations in patients with syndromic craniosynostosis is around 2%, and 0.7% in clinically nonsyndromic craniosynostosis.

The turricephaly index: A validated method for recording turricephaly and its natural history in Apert syndrome.

Introduction: We present the CT scan-derived turricephaly index (TI) as a quotient of the maximal occipito-frontal length of the skull to the distance from the centre of the sella to the highest point on the vertex as a validated tool for assessing turricephaly and evaluating surgical techniques aimed at reducing it.

Materials And Methods: Measurements taken from CTs of non-operated children with Apert syndrome and age-matched controls were analysed using Centricity PACS system (from the lateral scout image) and the thick-sliced Osirix tool. CTs from non-operated children with Apert syndrome were used to investigate the natural history of their turricephaly both as a group and individually.

Lack of association of cranial lacunae with intracranial hypertension in children with Crouzon syndrome and Apert syndrome: a 3D morphometric quantitative analysis.

Purpose: Cranial lacunae (foci of attenuated calvarial bone) are CT equivalents of "copper beating" seen on plain skull radiographs in children with craniosynostosis. The qualitative presence of copper beating has not been found to be useful for the diagnosis of intracranial hypertension (IH) in these patients. 3D morphometric analysis (3DMA) allows a more systematic and quantitative assessment of calvarial attenuation.

Development of a New Patient-reported Outcome Measure for Ear Conditions: The EAR-Q.

Background: Patient-reported outcome measures are widely used to improve health services and patient outcomes. The aim of our study was to describe the development of 2 ear-specific scales designed to measure outcomes important to children and young adults with ear conditions, such as microtia and prominent ears.

Methods: We used an interpretive description qualitative approach.

Intracranial Volume and Head Circumference in Children with Unoperated Syndromic Craniosynostosis.

Background: When analyzing intracranial volume gain resulting from operative intervention in craniosynostosis, it is necessary to understand the underlying growth. The authors sought to create comprehensive intracranial volume and occipitofrontal circumference growth charts, as measured on unoperated craniosynostotic children, and aimed to investigate whether intracranial volume and occipitofrontal circumference could act as proxy measures for each other.

Methods: All preoperative Great Ormond Street Hospital patients with a diagnosis of Apert, Crouzon-Pfeiffer, or Saethre-Chotzen syndrome from the year 2004 onward were considered for this study.

Results Following Adoption of a Modified Melbourne Technique of Total Scaphocephaly Correction.

The Melbourne technique was described in 2008 as a novel method for complete correction of scaphocephaly. Since 2015, it has become our operation of choice for children with sagittal synostosis who are too old at presentation for minimally invasive techniques. Our modifications were 2-position (initially supine then prone) technique and undertaking a formal fronto-orbital remodeling to correct forehead contour.

Mosaic RAS/MAPK variants cause sporadic vascular malformations which respond to targeted therapy.

Background: Sporadic vascular malformations (VMs) are complex congenital anomalies of blood vessels that lead to stroke, life-threatening bleeds, disfigurement, overgrowth, and/or pain. Therapeutic options are severely limited, and multidisciplinary management remains challenging, particularly for high-flow arteriovenous malformations (AVM).

Methods: To investigate the pathogenesis of sporadic intracranial and extracranial VMs in 160 children in which known genetic causes had been excluded, we sequenced DNA from affected tissue and optimized analysis for detection of low mutant allele frequency.