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

LEVEL OF EVIDENCE: III.

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. METHODS: A retrospective review was carried out including all SA-PVE cases performed at GOSH between 2008 and 2020. Demographic and clinical data were recorded including genetic diagnosis, craniofacial surgical history, surgical indication and assessment, age at time of surgery (spring insertion and removal), operative time, in-patient stay, blood transfusion requirements, additional/secondary (cranio)facial procedures, and complications. RESULTS: Between 2008 and 2020, 200 SA-PVEs were undertaken in 184 patients (61% male). The study population consisted of patients affected by syndromic (65%) and non-syndromic disorders. Concerns regarding raised intracranial pressure were the surgical driver in 75% of the cases, with the remainder operated for shape correction. Median age for SA-PVE was 19 months (range, 2-131). Average operative time for first SA-PVE was 150 min and 87 for spring removal. Median in-patient stay was 3 nights, and 88 patients received a mean of 204.4 ml of blood transfusion at time of spring insertion. A single SA-PVE sufficed in 156 patients (85%) to date (26 springs still in situ at time of this analysis); 16 patients underwent repeat SA-PVE, whilst 12 underwent rigid redo. A second SA-PVE was needed in significantly more cases when the first SA-PVE was performed before age 1 year. Complications occurred in 26 patients with a total of 32 events, including one death. Forty-one patients underwent fronto-orbital remodelling at spring removal and 22 required additional cranio(maxillo)facial procedures. CONCLUSIONS: Spring-assisted posterior vault expansion is a safe, efficient, and effective procedure based on our 12-year experience. Those that are treated early in life might require a repeat SA-PVE. Long-term follow-up is recommended as some would require additional craniomaxillofacial correction later in life.

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. DESIGN: An interpretive description qualitative approach was followed. Semistructured interviews were conducted, transcribed verbatim, and coded using a line-by-line approach. Qualitative analysis led to the development of a conceptual framework of outcomes important to patients. SETTING: Interviews were conducted in Canada and the United Kingdom at home, by telephone, or in the hospital. PARTICIPANTS: Participants (N = 72) were recruited between May and June 2014 from craniofacial clinics at the Hospital for Sick Children (Toronto) and Great Ormond Street Hospital (London). Participants included anyone with a visible and/or functional facial difference aged 8 to 29 years and fluent in English, excluding patients with a cleft. The sample included 38 females and 34 males, with a mean age of 13.9 years, and included 28 facial conditions (11 facial paralysis, 18 ear anomalies, 26 skeletal conditions, and 17 soft tissue conditions). RESULTS: Analysis led to identification of important concepts within 4 overarching domains: facial appearance, facial function, adverse effects of treatment, and health-related quality of life (psychological, social, and school function). CONCLUSIONS: Our study provides an understanding of concepts important to children and young adults with facial differences.

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. Here, we present findings from 16 unrelated probands with ERF-related craniosynostosis, with additional data from 20 family members sharing the mutations. Most of the probands exhibited multisutural (including pan-) synostosis but a pattern involving the sagittal and lambdoid sutures (Mercedes-Benz pattern) predominated. Importantly the craniosynostosis was often postnatal in onset, insidious and progressive with subtle effects on head morphology resulting in a median age at presentation of 42 months among the probands and, in some instances, permanent visual impairment due to unsuspected raised intracranial pressure (ICP). Facial dysmorphism (exhibited by all of the probands and many of the affected relatives) took the form of orbital hypertelorism, mild exorbitism and malar hypoplasia resembling Crouzon syndrome but, importantly, a Class I occlusal relationship. Speech delay, poor gross and/or fine motor control, hyperactivity and poor concentration were common. Cranial vault surgery for raised ICP and/or Chiari-1 malformation was expected when multisutural synostosis was observed. Variable expressivity and nonpenetrance among genetically affected relatives was encountered. These observations form the most complete phenotypic and developmental profile of this recently identified craniosynostosis syndrome yet described and have important implications for surgical intervention and follow-up.

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. We used 3DMA to examine the relationship between cranial lacunae and IH in children with Crouzon and Apert syndromic craniosynostosis. METHODS: Patients were divided into IH and non-IH groups defined on an intention-to-treat basis. Pre-operative CT scans were converted into 3D skull models and processed to quantify lacunae as a percentage of calvarium surface area (LCP). This was done on individual bone and whole skull basis. RESULTS: Eighteen consecutive children with Crouzon's syndrome and 17 with Apert syndrome were identified. Median age at CT scan was 135 days (range 6-1778). Of the 35 children, 21 required surgery for IH at median age of 364 days (range 38-1710). Of these 21 children, 14 had lacunae with mean LCP of 3% (0-28%). Of the 14 non-IH children, 8 had lacunae with mean LCP of 2% (0-8%). LCP was not significantly different between IH and non-IH groups. Parietal bones were most likely to show lacunae (IH 14/21, non-IH 9/14), followed by occipital (IH 8/21, non-IH 3/14), and frontal (IH 6/21, non-IH 2/14). CONCLUSION: Results suggest that cranial lacunae, measured using quantitative 3DMA, do not correlate with IH, in agreement with evidence from qualitative plain skull radiograph studies.

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. Retrospective chart review was used to record demographics, blood transfusion frequency and volumes, operating time, length of stay, clinical outcome, and complications. Eleven underwent modified Melbourne procedure between July 2015 and March 2017; 9 of 11 were male. All had a diagnosis of nonsyndromic sagittal synostosis. Mean age at surgery was 29 months. Mean surgical time was 6 hours. All patients required blood transfusion with a mean volume transfused of 29 mL/kg (range 13-83 mL/kg). For those 5 patients where preoperative and postoperative measurements were available, there was an increase in mean cephalic index (CI) from 0.64 to 0.75. All postoperative patients had a CI of over 0.70. Three-dimensional shape analysis indicated head shape change addressing all phenotypic aspects of scaphocephaly. In the 5 patients in which analysis could be undertaken, the mean intracranial volume increased from 1481 cm preoperatively to 1671 cm postoperatively, a mean increase in intracranial volume of 14%. The postoperative intracranial volume was higher than preoperative in all 5 patients. There were 4 minor and no major complications. Modified Melbourne procedure is safe and effective for the treatment of severe scaphocephaly in sagittal synostosis.

Ear Aesthetics: Investigation by the Use of an Online Viral Survey.

INTRODUCTION: The ear is a key facial feature and yet few studies have previously assessed ear aesthetics. This study aimed to assess the anatomical components of the ear that have the greatest impact on the perception of ear aesthetics. MATERIALS AND METHODS: Three photographs of a male adult ear (close-up, lateral, posterior) were digitally manipulated such that in each, 1 anatomical element of the ear was either enlarged or reduced. A complete set of 16 photographs including a repeat of the original ear as a control were randomized and entered into an online survey that required respondents to rate the attractiveness of each ear on a scale of 1 (least attractive) to 10 (most attractive). The survey was disseminated using email and social media. RESULTS: A total of 248 responses were received, 155 women and 92 men. Respondents were grouped by demographics of age and occupation. Reducing (R) or enlarging (E) the helix (R, P = 0.0256; E, P = 0.003), concha (R, P = 0.0002; E, P = <0.0001) and lobule (R, P = 0.0006; E, P < 0.0001) had a significant effect on ratings of attractiveness. Altering the tragus had no significant effect (R, P = 0.448; E, P = 0.201). In lateral view, raising the height of the ear had a significant effect (P = <0.0001) but not in lowering the height (P = 0.3038). Increasing and decreasing the projection of the ears both had a significant effect (P < 0.0001). CONCLUSIONS: The results of this study have provided useful evidence regarding the anatomical components of the ear that have the greatest effect on ear aesthetics. Furthermore, this study has proven the usefulness of conducting research using viral online surveys.

Design, Manufacturing, and In Vitro Testing of a Patient-Specific Shape-Memory Expander for Nose Reconstruction With Forehead Flap Technique.

Forehead skin is widely acknowledged as a good donor site for total nasal reconstruction, thanks to its matching color, texture, and abundant vascularity. The forehead flap technique uses an axial pattern flap forehead skin to replace missing nasal tissue. To increase the amount of available tissue and reduce the size of the tissue defect after flap mobilization, tissue expanders may be used. Although this is a relatively established technique, limitations include reduced moldability of the forehead skin (which is thicker than the nasal skin), and the need for multiple sessions of expansion to achieve a sufficient yield to close the forehead.Shape-memory metals, such as nitinol, can be programmed to "remember" complex shapes. In this work, the methodology for producing a prototype of nitinol tissue expander able to mold the skin in a predetermined patient-specific skin shape is described. A realistic nose mold was manufactured using metal rapid prototyping; nitinol sheet and mesh were molded into nose-shape constructs, having hyperelastic as well as shape-memory capability. Computed tomography scanning was performed to assess the ability of the structure to regain its shape after phase transformation upon cooling within 2% of initial dimensions. The prototypes were implanted in a pig forehead to test its ability to impose a nose shape to the forehead skin.The shape-memory properties of nitinol offer the possibility of producing bespoke tissue expanders able to deliver complex, precisely designed skin envelopes. The hyperelastic properties of nitinol allow constant preprogrammed expansion forces to be generated throughout the expansion process.

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. We found a seven-fold increase in SJS/TEN since the COVID pandemic. This increase correlates with an increase in COVID infections and vaccination rates. We explore the immunopathological relationships between COVID and SJS/TEN and propose theories for possible associations.

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. Wound healing (≥95% wound epithelialization) and pain were measured. Twenty-one burns were reviewed on ten patients. Per the initial protocol, six patients were reviewed every 3 to 5 days, with removal of only secondary dressings, until days 13 to 14, when the alginate dressings were removed. One patient was reviewed every 3 to 5 days until day 10, when a clinic nurse removed the alginate dressing. Restrictions on movement during the COVID pandemic necessitated a protocol change, with only one review at approximately day 14 for removal of alginate and secondary dressings; three patients were reviewed in this manner. Burns on all patients were 100% epithelialized at the time of final review and there were no complications, such as scarring, infection, or need for grafting. Following initial debridement and dressings, patients reported minimal pain. Dressing costs appeared to be significantly decreased. This protocol may be particularly useful for patients managed in rural and remote locations, with telemedicine support if required.

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. This study analyzes infection rates before and after its implementation. METHODS: The protocol was designed around the needs of patients undergoing FFS and consists of three checklists covering their preoperative, intraoperative, and postoperative care. Compliance required the completion of each checklist. All patients undergoing FFS between 1999 and 2019 were studied retrospectively, and infections occurring before and after the implementation of the protocol were analyzed. RESULTS: One hundred three patients underwent FFS (60 monobloc and 36 facial bipartition) before the implementation of the protocol in August of 2013, and 30 patients underwent FFS after its implementation. Compliance with the protocol was 95%. After implementation, there was a statistically significant reduction in infections from 41.7% to 13.3% ( P = 0.005). CONCLUSIONS: Although no specific cause for a cluster of postoperative infection had been identified, the implementation of a bespoke protocol consisting of preoperative, perioperative, and postoperative checklists covering measures known to reduce infection risk was associated with a significant reduction in postoperative infections in patients undergoing FFS. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

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). PARTICIPANTS: All adult patients in New South Wales with self-inflicted burn injuries between 1st January 2015 and 31st December 2020. OUTCOME MEASURES: Demographic information, precipitating factors, burn severity, morbidity and mortality outcomes. RESULTS: We found18 episodes of self-immolation in 2020, compared to an average of 10 per year previously. Burn size significantly increased (43% total body surface area vs 28%) as did revised Baux score (92 vs 77). Most patients had a pre-existing psychiatric illness. Family conflict and acute psychiatric illness were the most common precipitating factors. CONCLUSION: 2020 saw an increase in both the frequency and severity of self-inflicted burn injuries in New South Wales, with psychiatric illness a major factor.

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. METHODS: The tragus is placed on two cartilaginous bars (L-shaped), which are fixed to the framework base block creating a complete ring. These increase tragal projection and conchal depth. The tragus is carved angled posteriorly to have a shadowing effect on the absent auditory canal. Aesthetic outcome was assessed, at least 6 months after the second stage, on a 5-point ordinal scale (1-5) using the scoring system published in the UK microtia care standards and agreed on by the International Society for Auricular Reconstruction (ISAR). RESULTS: A total of 406 auricular reconstructions were performed in 363 patients (206 right, 114 left, 43 bilateral). After excluding cases who did not have second stage and those without complete photographs, 290 cases were assessed. The described modification in the framework carving was performed in 255 ears out of these 290 ears. The mean aesthetic score before and after the modification was 2.4 and 3.09 respectively for the tragus (p < 0.001), 2.2 and 2.95 for the intertragal notch (p < 0.001) and 2.77 and 3.49 for the concha (p < 0.001). CONCLUSIONS: This technical refinement has resulted in increased permanence and definition of the tragus and deeper and more aesthetic concha.

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. OBJECTIVE: The aim of this article is to provide an understanding of seasonal variation in foot burns for high-risk groups and how to manage them appropriately. This article provides a comprehensive analysis on epidemiology, pathology and management, with the aim of providing guidance for general practitioners (GPs). DISCUSSION: The management of foot burns is multidisciplinary, involving GPs, allied health specialists, nurses, medical and surgical specialties. The epidemiological variations of these injuries have shown differences in clinical outcomes among high-risk groups such as the elderly and people with diabetes. Education and prevention are crucial in reducing the rate of injury in the rising number of patients in high-risk groups.

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. Our aim is to analyse epidemiological trends of foot burns and examine the differences between diabetic and non-diabetics at Concord hospital from 2014 to 2019. METHODS: A retrospective audit from 2014-19 at Concord General Repatriation Hospital Burns Unit summarised patient demographics, burn injury, diabetic status, operations and length of stay. All foot burn injuries from 2014-19 of all ages and gender that attended Concord burns hospital were included in this study. RESULTS: We treated 797 patients who presented with foot burns, of which 16.2% were diabetic. The average age was higher in diabetics (60.72 years) than non-diabetics (39.72 years) and more males suffered burns compared to females in both groups (p < 0.001). There was a larger portion of elderly patients (greater than 65 years old, 15.1% of total) who sustained foot burns in the diabetic group compared to the non-diabetic group (p < 0.001). The most affected season was summer (27.0%), but diabetic patients were 1.7 times more likely to sustain injury in winter than non-diabetics. Diabetics were 3.8 times more likely to have contact burns compared to non-diabetic patients (p < 0.001). In a multivariable linear regression analysis, factors that contributed to increased length of stay included elderly status, place of event, diabetic status, number of operations, ICU admission, wound infection, amputation, and admission [F (16, 757 = 41.149, p < 0.001, R2 = 0.465]. CONCLUSIONS: With the increase of diabetes, our multidisciplinary approach to diabetic foot care should include nursing, medical and surgical disciplines to identify patients at risk. The data highlights that a focus on prevention and education for diabetes is central to optimize glycaemic control and burn management, whilst providing a multidisciplinary network on discharge.

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. METHODS: Data were collected between October 2016 and December 2019 from 2132 patients aged 8 to 29 years with noncleft facial conditions. Rasch measurement theory (RMT) analysis was used to examine Differential Item Function (DIF) by comparing the original CLEFT-Q sample and the new FACE-Q craniofacial sample. Reliability and validity of the scales in a combined cleft and craniofacial sample (n=4743) were examined. RESULTS: DIF was found for 23 CLEFT-Q items when the datasets for the two samples were compared. When items with DIF were split by sample, correlations between the original and split person locations showed that DIF had negligible impact on scale scoring (correlations ≥0.995). In the combined sample, RMT analysis led to the retention of original content for ten CLEFT-Q scales, modification of the Teeth scale, and the addition of an Eating/Drinking scale. Data obtained fit with the Rasch model for 11 scales (exception School, p=0.04). Person Separation Index and Cronbach alpha values met the criteria. CONCLUSION: The scales described in this study can be used to measure outcomes in children and young adults with cleft and noncleft craniofacial conditions.

FACE-Q craniofacial module: Part 2 Psychometric properties of newly developed scales for children and young adults with facial conditions.

BACKGROUND: The FACE-Q Craniofacial Module is a patient-reported outcome measure designed for patients aged 8 to 29 years with conditions associated with a facial difference. In part 1, we describe the psychometric findings for the original CLEFT-Q scales tested in patients with cleft and noncleft facial conditions. The aim of this study was to examine psychometric performance of new FACE-Q Craniofacial Module scales. METHODS: Data were collected between December 2016 and December 2019 from patients aged 8 to 29 years with conditions associated with a visible or functional facial difference. Rasch measurement theory (RMT) analysis was used to examine psychometric properties of each scale. Scores were transformed from 0 (worst) to 100 (best) for tests of construct validity. RESULTS: 1495 participants were recruited with a broad range of conditions (e.g., birthmarks, facial paralysis, craniosynostosis, craniofacial microsomia, etc.) RMT analysis resulted in the refinement of 7 appearance scales (Birthmark, Cheeks, Chin, Eyes, Forehead, Head Shape, Smile), two function scales (Breathing, Facial), and an Appearance Distress scale. Person separation index and Cronbach alpha values met criteria. Three checklists were also formed (Eye Function, and Eye and Face Adverse Effects). Significantly lower scores on eight of nine scales were reported by participants whose appearance or functional difference was rated as a major rather than minor or no difference. Higher appearance distress correlated with lower appearance scale scores. CONCLUSION: The FACE-Q Craniofacial Module scales can be used to collect and compare patient reported outcomes data in children and young adults with a facial condition.

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. We aimed hereby to obtain and validate a skull material model for SAC outcome prediction. Computed tomography data relative to 18 patients were processed to simulate surgical cuts and spring location. A rescaling model for age matching was created using retrospective data and validated. Design of experiments was used to assess the effect of different material property parameters on the model output. Subsequent material optimization-using retrospective clinical spring measurements-was performed for nine patients. A population-derived material model was obtained and applied to the whole population. Results showed that bone Young's modulus and relaxation modulus had the largest effect on the model predictions: the use of the population-derived material model had a negligible effect on improving the prediction of on-table opening while significantly improved the prediction of spring kinematics at follow-up. The model was validated using on-table 3D scans for nine patients: the predicted head shape approximated within 2 mm the 3D scan model in 80% of the surface points, in 8 out of 9 patients. The accuracy and reliability of the developed computational model of SAC were increased using population data: this tool is now ready for prospective clinical application.

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. Arhinia requires extensive management in early life in order to ensure airway patency and protection by means of tracheostomy, and to allow adequate pharyngeal and feeding function to the child. Aesthetic issues are managed with reconstructive surgery or an external prosthesis. There is no previous description in Literature of internal prosthetic devices used to sequentially shape soft tissues in complex reconstruction. We present an example of design and manufacturing of a bespoke nose implant produced by means of 3D printing and directly assessed on-table by means of 3D surface scanning.