Early Open Extensive Suturectomy for the Severe Multisuture Synostosis.

Neonates born with severe multisuture synostosis can present as an emergency. The severe craniocerebral disproportion with or without underlying hydrocephalus and retruded midface can result in raised intracranial pressure and airway compromise within the first few days or weeks of life. This presents a challenging multidisciplinary condition. There is no international consensus on management. There are limited publications available describing the approach to treatment. In our unit, children who present in the neonatal period with severe multisuture synostosis are offered early open extensive suturectomy within the first few months of life. The goals are; reduction in raised intracranial pressure, improvement in head shape and bone formation, and avoidance of a ventriculoperitoneal shunt. This is performed as an adjunct, not a replacement of other traditional skull vault procedures. We describe the technique and postoperative care without the need for a helmet that leads to excellent skull-shape outcomes and avoidance of a ventriculoperitoneal shunt.

Part II: Blood Transfusion and Donor Exposure in the Surgical Management of Trigonocephaly Patients: A Protocol From Alder Hey Craniofacial Unit.

Trigonocephaly is a craniofacial malformation caused by premature fusion of the metopic suture. Surgical correction frequently results in the need for blood transfusion. Transfusion complications include transfusion-transmitted infections (TTIs), immune-mediated reactions, and volume overload. Donor exposure (DE) describes the number of blood products from unique donors with increasing DE equating to an increased risk of TTI. We evaluate data on 204 trigonocephaly patients covering 20 years of practice with respect to blood transfusions and DE. This represents the largest series from a single unit to date. A protocol based on our experiences has been devised that summarizes the key interventions we recommend to minimize blood transfusions and DE in craniofacial surgery. Patients operated on between 2000 and 2020 were included. DE and a range of values were calculated including estimated red cell loss (ERCL) and estimated red cell volume transfused (ERCVT). Groups were established by relevant interventions and compared using the Mann-Whitney U test. Mean DE fell from 1.46 at baseline to 0.85 ( P <0.05). Median allogenic transfusion volume fell from 350 mL at baseline to 250 mL ( P <0.05). Median ERCL fell from 15.05 mL/kg at baseline to 12.39 mL/kg and median ERCVT fell from 20.85 to 15.98 mL/kg. Changes in ERCL and ERCVT did not reach statistical significance. DE can be minimized with the introduction of key interventions such as a restrictive transfusion policy, preoperative iron, cell saver, tranexamic acid, and use of a matchstick burr for osteotomies.

Malrotation and Fibroblast Growth Factor Receptor-2-Associated Craniosynostosis: An Underrecognized Association and Management Pathway.

INTRODUCTION: Feeding difficulties are common and multifactorial in children with Fibroblast Growth Factor Receptor-2 (FGFR-2) mutations. Intestinal rotation anomalies have been demonstrated to occur more frequently in animals with FGFR-2 mutations. This study aims to describe intestinal rotation anomalies, surgical management, and feeding assistance in children with FGFR-2 mutations who have undergone upper gastrointestinal (UGI) contrast studies. METHODS: Retrospective data were collected of children born between 1988 and 2020 in a UK quaternary craniofacial unit with FGFR-2-associated craniosynostosis. A consultant survey of approach to malrotation was undertaken. RESULTS: Thirty-four children were included, 17 (50%) female. Six (18%) had UGI symptoms, which included bilious vomiting (n=2), nonbilious vomiting (n=5), retching (n=1), feed intolerance (n=3), and failure to thrive (n=3). Nine had a gastrostomy in situ. Intestinal rotation anomalies occurred in 4 (12%) children, 3 of whom underwent a Ladd procedure and two third required gastrojejunal feeding postoperatively. Consultants agreed that all children with FGFR-2 mutation and UGI symptoms should undergo UGI contrast study, as should children requiring a gastrostomy. DISCUSSION: Intestinal rotation anomalies in children with FGFR-2 mutations occur more frequently than the general population. Prompt consideration of UGI contrast in symptomatic children with FGFR-2 mutation is recommended to enable early surgical management of children with malrotation.

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

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

Patient Tailored Surgery in Saethre-Chotzen Syndrome: Analysis of Reoperation for Intracranial Hypertension.

Saethre-Chotzen syndrome (SCS) is a syndromic craniosynostosis with pathogenic variants in the TWIST1 gene showing a broad phenotypic spectrum. Controversies exist in the literature regarding surgical management with single one-stage versus patient-tailored surgery and the related reoperation rate for intracranial hypertension of up to 42%. At our center, SCS patients are offered patient-tailored surgery with single-stage fronto-orbital advancement and remodeling or fronto-orbital advancement and remodeling and posterior distraction in an individually determined order. The authors' database identified 35 confirmed SCS patients between 1999 and 2022. Involved sutures in craniosynostosis were left unicoronal (22.9%), bicoronal (22.9%), sagittal (8.6%), bicoronal and sagittal (5.7%), right unicoronal (2.9%), bicoronal and metopic (2.9%), bicoronal, sagittal and metopic (2.9%), and bilateral lambdoid (2.9%). There was pansynostosis in 8.6% and no craniosynostosis in 14.3% of the patients. Twenty-six patients, 10 females, and 16 males were operated on. Mean age at the first surgery was 1.70 years, and 3.86 years at the second surgery. Eleven of 26 patients had invasive intracranial pressure monitoring. Three patients presented with papilledema before the first surgery and 4 afterward. Four of the 26 operated patients were operated initially elsewhere. The other 22 patients were initially referred to our unit and underwent patient-tailored surgery. Nine of these patients (41%) had a second surgery, and 3 (14%) of them were because of raised intracranial pressure. Seven (27%) of all operated patients had a complication. Median follow-up was 13.98 years (range, 1.85-18.08). Patient-tailored surgery in a specialized center and long-term follow-up allow for a low reoperation rate for intracranial hypertension.

The Paediatric Vault Score (PVS)-A Novel Scoring Tool for Prioritisation of Surgical Management of Craniosynostosis Patients.

Craniosynostosis is the premature fusion of the skull sutures, resulting in abnormal skull shape and volume. Timely management is a priority in avoiding raised intracranial pressure which can result in blindness and neurodevelopmental delay. Due to the COVID-19 pandemic, theater access was reduced. A risk stratification scoring system was thus devised to score patients attending surgery and aid in prioritization according to surgical need. The authors present the Paediatric Vault Score (PVS), which can also be customized to each unit's individual protocols. Ten patients on the waiting list were randomly selected and their clinical information was summarized in uniform anonymized reports. Six craniofacial consultants were selected as assessors and given 1 week to independently rank the patients from 1 to 10. Each scorer's ranking was verified against the PVS template and concordance was analyzed using the Kendall tau correlation coefficient (KT). Three cycles of the scoring process were carried out. Improvements were made to the scoring tool following cycle 1. Cycle 1 revealed 2 clinicians to be concordant with the PVS system and 4 to be discordant. Cycle 2 revealed all 6 clinicians to be concordant, with a mean KT score of 0.61. The final cycle revealed all 6 clinicians to be concordant, with a mean KT score of 0.70. Four scorers increased their concordance once the scoring sheet was introduced. Kendall's correlation of concordance calculated the interrater reliability to be 0.81. The PVS is the first known vault scoring system to aid in risk stratification and waiting list prioritization.

An Esthetic Scoring System for Scaphocephaly Assessment and Outcomes: A Pilot Study.

Scaphocephaly is the commonest form of craniosynostosis with a varied presentation consisting of many morphological components and a range of possible surgical interventions. However, with regard to esthetic assessment, there is no universally applied assessment system. The aim was to develop a simple assessment tool encompassing multiple phenotypic components of scaphocephaly. This was done by piloting a red/amber/green (RAG) scoring system to judge esthetic outcomes following scaphocephaly surgery using photographs and experienced observers. Standard photographic views of 20 patients who had undergone either passive or anterior 2/3 vault remodelling were scored by 5 experienced assessors. Using a RAG scoring system before and after scaphocephaly correction according to 6 morphological characteristics: visual impression of cephalic index, calvarial height, bitemporal pinching, frontal bossing, posterior bullet, and displacement of the vertex. All 5 assessors were asked to score the preoperative and postoperative views independently. The RAG scores were each assigned a number (1-3) and added to give a composite score (range 6-18) and these were averaged between the 5 assessors. There was a highly statistically significant difference between both preoperative and postoperative composite scores ( P <0.0001). A subgroup analysis of the postoperative composite score between the 2 surgical techniques showed no significant difference ( P =0.759). The RAG scoring system can be used to assess esthetic change following scaphocephaly correction and it provides both a visual analogue and a numerical indicator of change. This assessment method needs further validation but is a potentially reproducible way to score and compare esthetic outcomes in scaphocephaly correction.

Review of prophylactic prescribing of antibiotics during the management of fractured mandibles.

OBJECTIVES: To provide evidence based guidance on the optimum prophylactic antibiotic prescribing regimens in the treatment of fractured mandibles to protect against surgical site infections. MATERIAL AND METHODS: OVID and Pubmed databases were searched for articles published between 1946 and 2020. Inclusion criteria was for articles to be in English, involve adult patients aged 14 and over, and involve patients treated with oral or IV antibiotics preoperatively, perioperatively or postoperatively during treatment of open or closed fractures of the mandible. Exclusion criteria included infected fractures on presentation, immunocompromised patients, fractures resulting from gunshot and pathological fractures. RESULTS: A number of retrospective and prospective, randomised, double blind placebo-controlled trials were identified as suitable for inclusion. The age range within these trials was 14-77 years old. The numbers of patients contained within each trial ranged from 30 to 642. The most commonly prescribed antibiotics were penicillin, administered orally or intravenously. Duration of administration ranged from hospital admission to five days postoperatively. Analysis of these studies failed to demonstrate a statistical difference on the number of surgical site infections and the duration of antibiotic course. CONCLUSIONS: The available evidence reveals no statistical difference in infection rates whether antibiotics are prescribed pre, peri, or postoperatively. The duration of antibiotics therapy also appears not to be important. Current evidence does not support the recommendation of an optimum antibiotic prescribing regimen. Additional prospective studies looking at the duration and timing of antibiotics during the management of fractured mandibles are required to identify the optimum prescribing regimen.

Part 1: Surgical Correction in 231 Trigonocephaly Patients - The Alder Hey Experience.

ABSTRACT: Isolated metopic synostosis presents with a range of severity, from a palpable ridge as the sole presenting feature to a constellation of features resulting in trigonocephaly. At our unit, patients on the moderate to severe end of the phenotypic spectrum of trigonocephaly are offered fronto-orbital advancement and remodeling. The authors present our series of trigonocephaly patients who have undergone surgical correction. From January 2000 to January 2020, the authors operated on 231 patients with trigonocephaly. The average age at surgery was 18 months, with an average follow-up of 77.4 months. Seventy-nine percent of patients had no comorbidity. Ten percent of patients sustained a dural tear with no long-term consequences. The total early complication rate was 12.1%. The most common early complications were wound infection and wound dehiscence at 7.4% and 3.9% respectively. The total reoperation rate was 6.5%. The introduction of infection prevention and control measures over the 2 decades at our unit reduced the reoperation rate to 1.1%. The most common late complication was temporal recession in 20.8% of patients, none of whom required aesthetic correction. The recurrence rate of a metopic ridge was 2.3% with no patients requiring further surgery. None of our patients required calvarial remodeling for raised intracranial pressure after the primary fronto-orbital advancement and remodeling. There were no life-threatening complications or mortalities in our cohort. The authors present recommendations which include an infection control care bundle, cessation of surgical drains, and practice adjustments to reduce risks of infection and risk of requiring further calvarial remodelling for raised intracranial pressure.

Isolated Frontosphenoidal Craniosynostosis: The Alder Hey Experience and a Novel Algorithm to Aid Diagnosis.

ABSTRACT: Unilateral synostotic frontal plagiocephaly is most commonly due to a premature fusion of the frontoparietal suture. However, the coronal ring comprises of major and minor sutures and these sutures in isolation or in combination can result in similar clinical presentations which can make diagnosis challenging and result in a delay in referral to a craniofacial surgeon for timely management. Isolated frontosphenoidal craniosynostosis is a rare clinical entity with only 49 cases reported in the English literature to date. The authors present our series of 4 patients to add to this cohort of patients and describe key characteristics to distinguish frontoparietal from isolated frontosphenoidal synostosis and introduce a means of differentiating these 2 diagnoses from posterior deformational plagiocephaly and unilateral lambdoid synostosis. All previous case reports have been diagnosed after radiological imaging but the authors have devised a novel algorithm to aid the clinician in diagnosis of craniosynostosis before any radiological imaging.

Pseudomeningocele With Orbital Extension as a Complication of Fronto-Orbital Advancement and Remodeling in Craniosynostosis.

AIM: The authors present a series of patients who developed a pseudomeningocele following fronto-orbital advancement and remodeling (FOAR), describing clinical presentation, investigations, and management. Risk factors are identified and preventative strategies suggested. MATERIALS AND METHODS: From 2002 to 2012, all patients who underwent FOAR at our unit were identified. Those who developed a pseudomeningocele were selected and case notes, scan imaging and photographs were reviewed. RESULTS: Two hundred thirty-six FOAR operations were carried out over 12 consecutive years. Sixty-one of these patients were syndromic. A pseudomeningocele occurred in 6 patients. All affected cases were syndromic. Clinical features of presentation with orbital pseudomeningocele included orbital swelling, ptosis, proptosis, and/or hypoglobus. Raised intracranial pressure (ICP) was managed before pseudomeningocele repair in 2 patients, at the time of pseudomeningocele repair using an extra-ventricular drain (EVD) or lumbar drain in 4 patients. Decompression of the pseudomeningocele with excision and duraplsty was carried out in 5 patients, 1 patient required excision of gliotic brain and obliteration of dead space. Four patients had a calvarial graft to manage the bony defect and a further 2 had a titanium mesh. None of the patients had a recurrence of the pseudomeningocele or any long-term ocular or aesthetic complications. CONCLUSION: Pseudomeningocele has not previously been described in FOAR, but in a large series of consecutive patients, we have identified a 2.5% incidence. This incidence increases to 10% in the syndromic population of patients undergoing FOAR. The risk factors include a diagnosis of syndromic craniosynostosis, dural tear, hydrocephalus or raised ICP, infection, persistent cerebrospinal fluid (CSF) leak, or presence of dead space. Preventative strategies include CSF management before or post-FOAR. The ultimate treatment of the pseudomeningocele and growing fracture involves surgical decompression of the collection, a duraplasty, reconstruction of the orbital roof, and temporary CSF diversion.

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

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

Gender differences in patients with severe dental infections presenting to hospital.

Introduction Gender differences have been documented in prevalence and morbidity of caries, gingivitis and oral health, but not previously in cervicofacial infection. Identification and minimisation of gender inequalities is a World Health Organisation priority, and there are physiological, behavioural and cultural reasons to suspect that sex and gender differences may be present.Methods Analysis was carried out of the MTReC National Snapshot audit of cervicofacial infections. This database was created by oral and maxillofacial surgery trainees in 2017 and records over 400 variables in 1,002 individual patients admitted to hospital with severe odontogenic infection.Results Records were available for 1,002 patients with cervicofacial infection (456 females and 546 males). There were significant differences between recorded gender in those presenting with airway compromise (male 7% vs female 2%, p = 0.001), severe inflammatory response syndrome (male 60% vs female 39%, p = 0.007) and requirement for awake fibre-optic intubation on admission (male 4% vs female 1%, p = 0.014).Discussion These results suggest that male patients access healthcare later in their disease than female patients, and with more severe systemic compromise. This may be due to prevalent cultural and behavioural norms. As equality of access is the responsibility of the administrator, we discuss methods which might improve timely presentation in males with cervicofacial infections.

Systematic review on the incidence of bisphosphonate related osteonecrosis of the jaw in children diagnosed with osteogenesis imperfecta.

OBJECTIVES: To conduct a systematic review of epidemiological literature to determine the incidence of bisphosphonate related osteonecrosis of the jaw occurring either spontaneously or after dental surgery, in children and adolescents diagnosed with osteogenesis imperfecta. MATERIAL AND METHODS: MEDLINE, HMIC and EMBASE were used to search for English-language articles published from 1946 - 2013. Inclusion criteria consisted of population based studies of children and adolescents (24 years and younger) diagnosed with osteogenesis imperfecta, only studies which included a dental examination, and patients treated with intravenous bisphosphonates were included. Articles were excluded if patients had any other co-morbidity which could affect osteonecrosis of the jaw, and those which treated patients with oral bisphosphonates only. RESULTS: Five studies consisting of four retrospective cohort studies and one case series were identified. Study populations ranged from 15 to 278 patients and number of subjects with osteogenesis imperfecta ranged from 15 to 221. Mean duration of intravenous bisphosphonate use ranged from 4.5 to 6.8 years. All patients were clinically examined and no patients were found to have osteonecrosis of the jaw. CONCLUSIONS: There is no evidence to support hypothesis of causal relationship between bisphosphonates and osteonecrosis of the jaw in children and adolescents with osteogenesis imperfecta. More prospective studies on bisphosphonate use in osteogenesis imperfecta needs to be carried out.

Oncological screening for Bilateral Breast Reduction: a survey of practice variations in UK Breast and Plastics surgeons 2009.

INTRODUCTION: Bilateral Breast Reduction (BBR) is a common procedure performed by Breast and Plastic surgeons in the UK. No consensus exists regarding preoperative screening for malignancy or for selective criteria for such screening. Preoperative BBR screening practices among UK Breast and Plastic surgeons are unknown. AIMS: Ascertain the preoperative and postoperative BBR screening practices of UK Breast and Plastic surgeons. MATERIALS AND METHODS: A questionnaire was posted to all 434 Breast and 335 Plastic surgeons in the UK. All results were analysed with relevant statistical methods. KEY RESULTS: 64% of Breast surgeons and 72% of Plastic surgeons responded. 40% of Breast surgeons and 91% of Plastic surgeons perform BBR. Routine radiological screening: 92% Breast 41% Plastic (p < 0.05). Routine breast examination prior to BBR: 98% Breast 91% Plastic. Routine histology for BBR specimens: 96% Breast 90% Plastic. Selective screening of patients aged 30-40 years old: Breast 38% Plastic 10%. Selective screening of patients aged 40-50: Breast 78%, Plastic 53%. Selective screening of patients with strong family history of breast cancer: Breast 72%, Plastic 91%. Selective screening of patients with previous breast cancer: Breast 77%, Plastic 93%. CONCLUSIONS: There are significant differences in practice between UK Breast surgeons and Plastic surgeons in preoperative oncological screening for BBR. The large discrepancy in preoperative radiological screening, reflects a ubiquitous pro-screening ideology among Breast surgeons not prevalent among Plastic surgeons. These results will provoke debate towards the direction of consensus to ultimately reflect best practice.

Sturge-Weber syndrome and dermatomal facial port-wine stains: incidence, association with glaucoma, and pulsed tunable dye laser treatment effectiveness.

BACKGROUND: Laser therapy is the optimal approach for treating port-wine stains, but whether it is effective for patients with facial dermatomal port-wine stains and Sturge-Weber syndrome is undetermined. This project aimed to verify the incidence of Sturge-Weber syndrome and glaucoma within the population, compare the response in syndromic and nonsyndromic patients with facial dermatomal port-wine stains to laser, and determine the treatment response in relation to dermatome and color. METHODS: The authors retrospectively reviewed 874 patients with facial port-wine stains (203 displayed a dermatomal pattern). Pretreatment and posttreatment photographs were assessed clinically and recorded on a statistically reliable percentage gradient. RESULTS: Sturge-Weber syndrome was diagnosed in 30 patients. Twelve patients had glaucoma, with the port-wine stains involving the ophthalmic (V1) division of the trigeminal nerve. The highest proportion of patients with glaucoma came from those with involvement of the maxillary (V2) and/or mandibular (V3) division of the trigeminal nerve. No patients had Sturge-Weber syndrome with V3 port-wine stains alone. After laser therapy, only 45 percent of syndromal patients and 55 percent of nonsyndromic patients had a satisfactory outcome (>50 percent) in color and size reduction of dermatomal port-wine stains. CONCLUSIONS: The incidence of Sturge-Weber syndrome was 3 percent in patients with a facial port-wine stain. There was an increased risk of Sturge-Weber syndrome with involvement of V1 port-wine stains and no risk with involvement of V3 port-wine stains alone. Laser treatment produced unsatisfactory outcomes in patients with facial dermatomal port-wine stains. V3 port-wine stains responded best and V2 worst to laser.