ERF-related craniosynostosis and surgical management in the paediatric cohort.

INTRODUCTION: ERF mutation is one of the most recently identified genetic aberrations associated with syndromic craniosynostosis. Data on the pattern of craniosynostosis, surgical management of ERF-related craniosynostosis and outcomes is limited. We report on our single-centre experience in paediatric cohort of patients with syndromic craniosynostosis secondary to ERF mutation. METHODS: A retrospective review of all paediatric craniofacial cases was performed over an 8-year period (2014-2022). All patients with genetically confirm ERF-related craniosynostosis were identified, and clinical parameters including, age, sex, pattern of craniosynostosis, associated tonsillar herniation and follow-up period were further analysed from electronic clinical and imaging systems. All patients were selected and discussed in multidisciplinary craniofacial meeting (composed of neurosurgical, maxillofacial, plastics and genetics teams) prior to any surgical intervention. RESULTS: Overall, 10 patients with ERF-related craniosynostosis were identified with a male-to-female ratio of 4:1 with mean age at the time of surgery of 21.6 months with a mean follow-up period of 5.2 years. ERF-confirmed cases led to variable craniosynostosis pattern with multi-sutural synostosis with concurrent sagittal and bilateral lambdoid involvement as the most common pattern (7/10). No patient pre-operatively had evidence of papilloedema on ophthalmological assessment. Eight out of 10 patients had associated low-lying tonsils/hind brain hernia pre-operatively. Eight out of 10 patients required surgery which included 2 fronto-orbital advancement, 3 calvarial remodelling, 2 posterior calvarial remodelling/release and 1 insertion of ventriculoperitoneal shunt. CONCLUSION: Involvement of sagittal and lambdoid sutures is the most common pattern of craniosynostosis. ERF-related craniosynostosis can have variable pattern of suture fusion, and management of each patient requires unique surgical planning and execution based on clinical needs for the optimal outcomes.

Extensive Post-Traumatic Surgical Emphysema Exacerbated by Cocaine Insufflation.

Surgical emphysema (SE) is characterized by air in the soft tissues causing a crackling sensation on palpation. In oral and maxillofacial surgery, it might occur using conventional air-driven dental hand pieces, as a complication of trauma and with cocaine insufflation.The subcutaneous air may travel through tissue planes causing cervicofacial emphysema, pneumothorax, and pneumomediastinum. It may carry bacteria and potentially lead to cellulitis or necrotizing fasciitis.The SE is usually a self-limiting entity requiring analgesia, close observation of the airway, occasionally prophylactic antibiotics, and rarely steroids. Although, the consensus on antibiotic and corticosteroid therapy is unclear.Here presented an unusual and extensive presentation of surgical emphysema. A 29 years old male presented with emphysema following a left orbital-zygomatic complex fracture and following intranasal cocaine insufflation. It extended from the temple to the parotid region, down into the neck and into the mediastinum. No surgical intervention was required. The patient was discharged after 24 hours of observation with a week-long course of co-amoxiclav antibiotics and no corticosteroids administration.Typical instructions following facial bone fractures are to avoid nose blowing because of the risk of SE, however, avoidance of drug habits is rarely considered. This case report highlights the importance of tailored advice to this particular patient cohort.

Custom-Made Three-Dimensional Models for Craniosynostosis.

The use of computer-aided design and computer-aided manufacturing in oral and maxillofacial surgery is an ever-growing field.The availability of 3D models, cutting guides, and customised surgical instruments gives surgeons the opportunity to modify and improve their surgical procedures.Here, we discuss the use of computer-aided design-computer-aided manufacturing to improve the management of a case of nonsyndromic metopic synostosis through the construction of: A cutting guide for the cranium, custom-made orbital protectors, a 3D model of the predicted postoperative meninges to allow off the table bone recontouring, and a template frontal bar to allow more specific recontouring of the frontal bar.

A review of drug-induced acute angle closure glaucoma for non-ophthalmologists.

Acute angle closure glaucoma is an ophthalmic emergency and can lead to blindness if left untreated. Several types of drugs have the potential to precipitate acute angle closure glaucoma. These include adrenergic, cholinergic and anticholinergic, antidepressants, anticoagulants and sulfa-based agents. This article provides a basic overview of the risk factors and pathophysiologic mechanisms involved in angle closure glaucoma and focuses on drug-induced angle closure glaucoma for the non-ophthalmologist. A PubMed search limited to the English language was conducted to find relevant literature for the purpose of this article. Most attacks occur in subjects unaware that they are at risk due to innately narrow iridocorneal angles. Clinicians should always review medications in patients presenting with symptoms of acute angle closure glaucoma. The aim of this article is to bring this ophthalmic condition to the attention of clinicians, particularly those outside the field of ophthalmology who commonly prescribe these medications or see these patients prior to referring to ophthalmologists.

Extensive subcutaneous emphysema secondary to endodontic treatment.

Subcutaneous emphysema is a relatively rare phenomenon, in the context of dentistry related to iatrogenic injury, with only 32 cases reported between 1993 and 2008. This article aims to discuss the relevance and importance of this issue to dentists, including how to identify the condition and its management.