Intravenous Tranexamic Acid is Associated With a Clinically Significant Reduction in Blood Loss in Craniosynostosis Surgery.

ABSTRACT: Blood loss is a potential cause of morbidity and mortality in craniosynostosis surgery. Recent reports have suggested that the use of tranexamic acid (TXA), an antifibrinolytic agent, mitigates this blood loss. A retrospective cohort study of patients undergoing craniosynostosis surgery at a tertiary craniofacial hospital in Sydney was undertaken. Primary outcomes were blood loss and transfusion requirements. Two groups were compared: those who received intravenous prophylactic TXA and those who underwent surgery without TXA. Statistical analysis was performed with Student t test and the Mann-Whitney U test for nonparametric results. We identified 206 patients who underwent craniosynostosis surgery over an 8 year period; 78 control patients and 128 patients that received TXA. Tranexamic acid was found to result in a weight-adjusted calculated blood loss mean difference of 9.6 ml/kg across all procedures (P = 0.0332 95% confidence interval 0.7734-18.4266). The actual blood loss reduction achieved with TXA was 6.7 ml/kg in spring cranioplasties, compared to 15.2 ml/kg in fronto-orbital remodeling procedures. There was a statistically and clinically significant reduction in postoperative transfusion incidence, with transfusions required in 27% of controls and 6% of TXA patients (P < 0.0001). The number needed to treat to prevent giving a unit of blood postoperatively was 4.8. There were no incidences of TXA-specific complications. This study found that TXA is a safe and effective method of decreasing blood loss and transfusion requirements in patients undergoing craniosynostosis surgery. The clinical benefit of TXA is particularly evident in the more invasive craniosynostosis surgeries.

Clinical Features and the Use of Three-Dimensional Imaging for Unilateral Lambdoid Synostosis.

INTRODUCTION: True isolated lambdoid craniosynostosis is rare. It requires corrective surgery to prevent intracranial pressure and aesthetic stigma by significant dyscrania. We summarize our case series for lambdoid craniosynostosis outlining the pathophysiology, clinical findings and surgical approaches and outcomes. METHODOLOGY: A retrospective analysis of our data from 2010 to 2020 summarized our cases of true lambdoid synostosis. We have used the medical notes and the radiological findings from computed tomography scans to summarize a case series of isolated lambdoid synostosis. RESULTS: Our case series demonstrated 7 patients with true isolated lambdoid craniosynostosis. In most cases surgical intervention in the form of posterior cranial vault remodeling utilizing a bandeau, based on occipital advancement techniques, has demonstrated the most consistently favorable aesthetic outcome. CONCLUSIONS: Surgical referral to a craniofacial center should be sought early in difficult to diagnose cases. Although rare, surgical intervention is indicated to correct potentially increased intracranial pressure and to ameliorate cranial dyscrania.

Tranexamic Acid in Craniosynostosis Surgery: A Systematic Review and Meta-Analysis.

ABSTRACT: Blood loss is a potential cause of morbidity and mortality in craniosynostosis surgery. Recent reports have suggested that the use of tranexamic acid (TXA), an antifibrinolytic agent, mitigates this blood loss. A comprehensive systematic review and subsequent meta-analysis was undertaken, with the view to clarify the effectiveness of TXA in reducing blood loss and transfusion requirements in craniosynostosis surgery. Medline and PubMed databases were searched using the preferred reporting items for systematic reviews and meta-analyses technique, and 7003 articles were assessed based on predefined selection criteria. Seven trials were identified, of which 2 were randomized controlled trials and the remainder retrospective cohort studies. All trials were assessed using the Jadad and strengthening the reporting of observational studies in epidemiology scores. The meta-analysis found a clear statistical reduction in blood loss in those patients who received TXA perioperatively, with a combined blood loss reduction of 7.06 ml/kg (95% confidence interval -8.97 to -5.15, P < 0.00001). The blood loss reduction was found to extrapolate to a reduction in perioperative transfusion requirements by 8.47 ml/kg in this cohort (95% confidence interval -10.9 to -6.04, P < 00001). There were no TXA-related adverse outcomes recorded in the 258 patients who received TXA across all trials. The trials included in this meta-analysis were limited by underpowered population sizes and significant heterogeneity in blood loss recording techniques. Further, there was significant heterogeneity amongst operations performed. The current literature appears to support the use of TXA in craniosynostosis surgery, but further high quality randomized controlled trials are indicated, ideally including a subgroup analysis between the operations performed.

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.

A Simple Bedside Technique for Quantifying Volumetric Defects Prior to Hydroxyapatite Cranioplasty.

Contour irregularities following pediatric craniofacial surgery are common. Hydroxyapatite cranioplasty is a successful technique for optimizing the aesthetic outcome in these patients. We describe a simple technique that can be carried out at the bedside to calculate the volume of hydroxyapatite needed and therefore optimize the preoperative planning for hydroxyapatite cranioplasty.

Quantification of Head Shape and Cranioplasty Outcomes: Six-compartment Volume Method Applied to Sagittal Synostosis.

BACKGROUND: Premature fusion of the sagittal (midline) suture between 2 parietal bones is the most common form of craniosynostosis. Surgical correction is mandated to improve head shape and to decrease the risk of raised intracranial pressure. This study evaluated the utility of 3-dimensional (3D) imaging to quantify the volumetric changes of surgical correction. Currently there is no standardized method used to quantify the outcomes of surgery for craniosynostosis, with the cranial index (width: length ratio) being commonly used. METHODS: A method for quantification of head shape using 3D imaging is described in which the cranium is divided up into 6 compartments and the volumes of 6 compartments are quantified and analyzed. The method is size invariant, meaning that it can be used to assess the long-term postoperative outcomes of patients through growth. The method is applied to a cohort of sagittal synostosis patients and a normal cohort, and is used to follow up a smaller group of synostotic patients 1, 2, and 3 years postoperatively. RESULTS: Statistical analysis of the results shows that the 6-compartment volume quantification method is more accurate in separating normal from synostotic patient head shapes than the cranial index. CONCLUSIONS: Spring-mediated cranioplasty does not return head shape back to normal, but results in significant improvements in the first year following surgery compared with the preoperative sagittal synostosis head shape. 3D imaging can be a valuable tool in assessing the volumetric changes due to surgery and growth in craniosynstosis patients.

Auricular reconstruction.

External ear abnormalities are common. These may affect ear shape, size, prominence and degree of development. They may also be associated with hearing loss. The early identification and management of hearing loss is essential. There are several options for reconstruction of the external ear using both autologous and non-autologous techniques. The aim of this article is to outline the different reconstructive options.

Epidemiology and Outcome of Squamous Cell Carcinoma in Epidermolysis Bullosa in Australia and New Zealand.

We investigate the epidemiology and outcomes of squamous cell carcinoma (SCC) in recessive dystrophic epidermolysis bullosa (RDEB) from the Australasian EB registry cohort. Seventeen out of 49 (34.6%) RDEB patients developed at least one SCC. Data detailing SCC was obtainable from 16/17 RDEB-SCC patients. A total number of 161 primary SCCs occurred in 16 RDEB-SCC patients with a mean of 10 SCCs per person. The earliest age of first SCC development was 16 years. Eleven out of 16 RDEB-SCC patients eventually developed metastatic SCCs. The majority of the tumours were well and moderately differentiated. The cumulative risk of SCC development by age 35 was 76.1% for RDEB-Generalized Severe (RDEB-GS) and 10% for RDEB-Generalized Intermediate (RDEB-GI). Amongst those who developed SCCs, their median time to death after first SCC was 5 years for RDEB-GI and 4 years for RDEB-GS. This is the first retrospective study of RDEB-SCC in Australasia.

Unilateral Pterional Polycraniosynostosis Treated with Craniectomy and Helmet Therapy.

Craniosynostosis is a condition in which one or more of the cranial sutures have fused prematurely, affecting the growth pattern and contours of the infant skull. The pterion is the junction of temporal, frontal, parietal, and sphenoid bones of the skull. We present a case of unilateral pterional craniosynostosis, which was treated with strip craniectomy and helmet therapy.

Threatened limb from stingray injury.

We present the case of a 43-year-old female who suffered a stingray injury to her left ankle. The sting caused occlusion of the dorsalis pedis artery, causing dry gangrene of the medial forefoot. A below knee amputation was recommended but she was transferred for a second opinion. A Prostaglandin E1 infusion was commenced, resulting in alleviation of pain and improvement in perfusion. Amputation of great and second toes was performed, with the head of the first metatarsal preserved and covered via a cross-over skin flap raised from the contralateral leg. Achilles tendon lengthening was then performed to return the foot to a functional position. This case serves to highlight the utility of prostaglandin infusion, and the requirement for a multidisciplinary approach to critical limb ischemia in order to avoid major amputation.

Frontoethmoidal mucocele following pediatric craniofacial surgery.

BACKGROUND: Mucoceles occur as a result of accumulation and retention of mucous secretions in a paranasal sinus and are uncommon in the pediatric age group. Persistent or intermittent closure of its ostium through a variety of causes, including previous surgery, is implicated in etiology. The authors report 2 cases of frontoethmoidal mucocele that followed box osteotomies for the treatment of orbital dystopia, with medical literature review and discussion of possible causal factors and events. METHODS: Case histories and radiological imaging are presented on 2 patients presenting with frontoethmoidal mucoceles following craniofacial surgery. Both had transcranial craniofacial techniques where all orbital walls and globe are moved en bloc as a "box." RESULTS: Patient 1, a 12-year-old male patient with Crouzon syndrome, developed mucoceles within 18 months of monobloc distraction surgery and box osteotomies. This was successfully marsupialized with a combined external and endoscopic surgical approach. The second patient, a 15-year-old boy with previously corrected right-sided facial cleft, developed mucocele 9 years following box osteotomies; this was successfully managed by endoscopic drainage. Of 3 other patients having similar box osteotomies in our unit, no other mucoceles were noted as complications. CONCLUSIONS: Mucoceles are a rare complication of craniofacial surgery, and literature review confirms a paucity of reports. Only 1 case has previously been alluded to of mucocele complicating box osteotomy for orbital dystopia. Our 2 cases illustrate and highlight a successful management approach in a multidisciplinary craniofacial unit.

Bisphosphonate-related osteonecrosis of the jaw: tip of the iceberg.

The World Health Organization predicts that people aged older than 65 years will comprise 20% of the world's population by 2030. One of the most commonly prescribed medications for the elderly are the bisphosphonates, which have been shown to significantly reduce debilitating and fatal fractures by preserving bone density and consequently saving governments billions of dollars annually. Despite rigorous testing, 190 million prescriptions worldwide and US$8000 million in revenue, there is a serious adverse effect called bisphosphonate-related osteonecrosis of the jaw, which is poorly described and difficult to treat. The difficulty is compounded by the inability of medical personnel to recognize and adequately refer these patients or take adequate precautions before instituting bisphosphonate therapy. A myriad of differentials and a lack of consensus on how to definitively treat these patients have made this new presentation a worrying precursor for millions of other consumers who will reach the 5-year oral half life of bisphosphonates, which is when they generally start to present. In this paper, we explore historical parallels and provide the most comprehensive review to date in the literature about the presentation, diagnosis, treatment, pathophysiology, oncogenic associations, and best practice guidelines. Legal action pursuant to bisphosphonate-related osteonecrosis of the jaw is underway on 3 continents, and we believe that every health care professional should become au fait with this condition for which our growing case series represents merely the tip of the iceberg.

Ocular advancement in monobloc distraction.

BACKGROUND: Monobloc distraction has been demonstrated to be an efficacious, safe, and stable method of providing functional and aesthetic improvement in children with syndromic craniosynostosis. Although the movement of bony structures following monobloc distraction has been quantitatively assessed before, objective analysis of the movement of soft tissues, such as the globe, has not been published previously. The authors present a method that assesses globe movement following monobloc distraction, using computed tomographic scan data. METHODS: The preoperative and postoperative computed tomographic scans of 10 patients with Pfeiffer's and Crouzon's syndromes, undergoing monobloc distraction, were assessed. Scan data were loaded into voxel image-rendering software that produced a three-dimensional reconstruction. Direct measurements between preoperative and postoperative images were taken. RESULTS: An average upper face osseous distraction distance of 12.94 mm, with an average lower face distraction of 14.19 mm, was achieved in all 10 children. The authors measured average globe movements on three planes and calculated an average left eye forward movement of 5.28 mm and average right eye forward movement of 6.33 mm. In all, globe advancement was approximately two fifths of the distance advanced by the surrounding bone. CONCLUSIONS: Forward movement of the globe following monobloc distraction has not previously been described. Although there is no current evidence for optic nerve stretch and compromise in monobloc surgery, the functional implications of these findings should be formally assessed.

Unilateral isolated frontosphenoidal craniosynostosis causing frontal plagiocephaly.

Synostotic frontal plagiocephaly is most commonly caused by frontoparietal (unicoronal) synostosis, but may be caused by other fusions along the coronal hemiring. Frontosphenoidal synostosis is a rare cause of frontal plagiocephaly, with only five previously reported cases. We present the case of an 8-month-old male infant with frontal plagiocephaly caused by unilateral isolated frontosphenoidal synostosis mimicking frontoparietal (unicoronal) synostosis. The clinical and radiological features included flattened ipsilateral forehead, retruded ipsilateral maxilla, a midline chin, contralateral deviation of the nasal root and endocranial base, and anomalies of the bony orbit. Unilateral isolated frontosphenoidal synostosis may be misdiagnosed as unicoronal synostosis. Careful physical and radiological assessment is necessary to recognise this synostotic disorder of the coronal hemiring.

Raised intracranial pressure in Apert syndrome.

BACKGROUND: Raised intracranial pressure is a well-known complication of Apert syndrome. The current policy in the authors' unit is to monitor these patients and only perform surgery when raised intracranial pressure has been diagnosed. The authors present their experience with this protocol, as it allows a more accurate picture of the natural history of raised intracranial pressure in Apert syndrome. METHODS: The records of 24 patients, aged between 7 and 14 years, with Apert syndrome who had been managed expectantly (i.e., with no routine "automatic" early surgery) were reviewed. Data were collected on the incidence, timing, and management of raised intracranial pressure. RESULTS: Twenty of 24 patients (83 percent) developed raised intracranial pressure. The average age of the first episode was 18 months (range, 1 month to 4 years 5 months). Raised intracranial pressure was managed with surgery in 18 patients, including two patients who underwent shunt procedures for hydrocephalus. Two patients had their raised intracranial pressure treated successfully by correcting coexisting upper airway obstruction alone. Seven of the 20 patients (35 percent) developed a second episode of raised intracranial pressure, on average 3 years 4 months later (range, 1 year 11 months to 5 years 9 months). CONCLUSIONS: In Apert syndrome, there is a high incidence of raised intracranial pressure, which can first occur at any age up to 5 years and may recur despite initial successful treatment. Causes of raised intracranial pressure include craniocerebral disproportion, venous hypertension, upper airway obstruction, and hydrocephalus. Careful clinical, ophthalmologic, respiratory, and radiologic monitoring will allow raised intracranial pressure to be diagnosed accurately when it occurs and then treated most appropriately.

Implications of a vertex bulge following modified strip craniectomy for sagittal synostosis.

BACKGROUND: Modified strip craniectomy is a common treatment for early isolated sagittal synostosis. The authors assessed the significance of the development of a progressive vertex bulge following strip craniectomy as a predictor of raised intracranial pressure or multiple suture synostosis. METHODS: All cases of sagittal synostosis treated by modified strip craniectomy (removal of the sagittal suture with lateral barrel staving) at the authors' institution were reviewed. Eighty-nine patients with isolated sagittal synostosis were treated by modified strip craniectomy, usually before 6 months of age, between 1995 and 2005. Seven patients were noted to have developed a progressive vertex bulge. The vertex bulge was noted an average of 8 months postoperatively (range, 2 to 25 months). The clinical records of these seven patients were evaluated with regard to their clinical course, radiologic investigations, genetics testing, intracranial pressure monitoring, and the need for further surgery. RESULTS: Computed tomographic scanning demonstrated new synostosis involving other calvarial sutures in five patients. Five patients underwent intracranial pressure monitoring, and this was elevated in four patients. One patient required a ventriculoperitoneal shunt for hydrocephalus. All patients underwent genetic screening, and two were found to have fibroblast growth factor receptor (FGFR) mutations (one FGFR2 and one FGFR3 mutation). All patients required reoperation (calvarial remodeling) for either raised intracranial pressure, deteriorating head shape, or both. CONCLUSIONS: A progressive vertex bulge after modified strip craniectomy is a sign of possible raised intracranial pressure, the development of progressive multiple suture synostosis, or both. It is an indication for genetic testing for FGFR mutations.

The role of the nasopharyngeal airway for obstructive sleep apnea in syndromic craniosynostosis.

Syndromic craniosynostosis is known to be associated with obstructive sleep apnea (OSA), which can often present in infancy. Although multifactorial, a predominant contributing factor is midface hypoplasia. Nasal continuous positive airway pressure has proven to be an effective treatment modality but may be poorly tolerated in certain cases. This study looks at the effectiveness of bypassing midface obstruction with a nasopharyngeal airway (NPA). Twenty-seven children with syndromic craniosynostosis with confirmed moderate to severe OSA were initially treated with an NPA. The mean age of NPA insertion was 12.3 months (range, 0.5-48 mo). Seventeen had severe OSA, and 10 had moderate OSA preinsertion. Post-NPA insertion, 26 of 27 children (96%) demonstrated an improvement in sleep severity scores, resulting in 3 with moderate OSA and 24 with mild OSA. There was a significant improvement in mean oxygen saturation, mean number of saturation dips greater than 4% per hour, percentage time spent less than 90% SpO2, and number of pulse rate rises per hour. There were no significant differences in mean pulse rate. The NPA was well tolerated by this patient group, with 24 of 26 children retaining it for at least 6 weeks. We believe that an NPA is therefore an effective first-line treatment modality in the management of OSA in children with syndromic craniosynostosis. It is well tolerated by the patient and may obviate the need for continuous positive airway pressure or tracheostomy.