Role of primary cilia and Hedgehog signaling in craniofacial features of Ellis-van Creveld syndrome.

Ellis-van Creveld syndrome (EvC) is an autosomal recessive genetic disorder involving pathogenic variants of EVC and EVC2 genes and classified as a ciliopathy. The syndrome is caused by mutations in the EVC gene on chromosome 4p16, and EVC2 gene, located close to the EVC gene, in a head-to-head configuration. Regardless of the affliction of EVC or EVC2, the clinical features of Ellis-van Creveld syndrome are similar. Both these genes are expressed in tissues such as, but not limited to, the heart, liver, skeletal muscle, and placenta, while the predominant expression in the craniofacial tissues is that of EVC2. Biallelic mutations of EVC and EVC2 affect Hedgehog signaling and thereby ciliary function, crucial factors in vertebrate development, culminating in the phenotypical features characteristic of EvC. The clinical features of Ellis-van Creveld syndrome are consistent with significant abnormalities in morphogenesis and differentiation of the affected tissues. The robust role of primary cilia in histodifferentiation and morphodifferentiation of oral, perioral, and craniofacial tissues is becoming more evident in the most recent literature. In this review, we give a summary of the mechanistic role of primary cilia in craniofacial development, taking Ellis-van Creveld syndrome as a representative example.

Trigeminal Traumatic Neuroma: a Comprehensive Review of the Literature Based On a Rare Case.

PURPOSE OF REVIEW: Traumatic neuromas in general, and trigeminal traumatic neuromas in particular, are relatively rare entities originating from a damage to a corresponding nerve or its branches. This manuscript is a comprehensive review of the literature on trigeminal traumatic neuromas based on an interesting and challenging case of bilateral intraoral lesions. RECENT FINDINGS: The diagnosis for this patient was bilateral trigeminal traumatic neuromas. It is possible that these patients have a genetic predisposition to the development of these lesions. It is a neuropathic pain condition and may mimic dental and other trigeminal pain entities. Topical treatment with lidocaine gel, utilizing a custom-made neurosensory stent, rendered the patient significant and sustained pain relief. Trigeminal traumatic neuromas present a diagnostic challenge even to a seasoned clinician, due to the complex clinical features that may mimic other entities. Topical medications such as local anesthetics may be a good viable alternative to systemic medications to manage the pain associated with the condition. Early identification of the lesion and the associated pain helps in the succinct management of symptomatic trigeminal traumatic neuromas.

Giant Cell Arteritis: A Case-Based Narrative Review of the Literature.

PURPOSE OF REVIEW: Giant cell arteritis (GCA) is a chronic, inflammatory condition, primarily affecting the medium and larger arteries. The purpose of this narrative review is to describe GCA in the context of headache and facial pain, based on a case and the available current literature. Understanding the etiology, pathophysiology, the associated conditions, and the differential diagnoses is important in managing GCA. RECENT FINDINGS: In a patient presenting with unilateral facial/head pain with disturbances of vision, GCA should be considered in the differential diagnosis. There is an association of GCA with several comorbid conditions, and infections including coronavirus-19 (COVID-19) infection. Management of GCA primarily depends upon the identification of the affected artery and prompt treatment. Permanent visual loss and other serious complications are associated with GCA. GCA is characterized by robust inflammation of large- and medium-sized arteries and marked elevation of systemic mediators of inflammation. An interdisciplinary approach of management involving the pertinent specialties is strongly recommended.

Monckeberg's Medial Sclerosis as a Cause for Headache and Facial Pain.

PURPOSE OF REVIEW: Mönckeberg's medial sclerosis (MMS) is a chronic, non-inflammatory degenerative condition affecting primarily the tunica media of muscular arteries resulting in their calcification. The purpose of this comprehensive review is to describe MMS as it appears in the literature, in the context of headache and facial pain. Understanding the etiopathology, the associated conditions, and the differential diagnoses is important in managing MMS. RECENT FINDINGS: Management of MMS primarily depends upon identification of its associated conditions and their treatment. Due to the rare incidence and inadequate literature on MMS presenting with headaches, the diagnosis of the pain and the entity itself is challenging. MMS is characterized by associated systemic conditions and absence of inflammatory markers. It can mimic giant cell arteritis (GCA) and other pain entities. An interdisciplinary approach involving appropriate specialties is recommended.

Nummular Headache - a Case Report of a Rare Entity.

PURPOSE OF REVIEW: The purpose of this manuscript is to shed light on systematic work-up of a diagnostic challenge such as nummular headache (NH), and to summarize the relevant literature on NH. RECENT FINDINGS: The specific nature of the shape of the pain site is usually characteristic of NH. Multiple modalities of investigation including succinct imaging are necessary to successfully rule out other similar conditions. A 26-year-old female patient of Asian-Indian origin presented to the clinic with the chief complaint of chronic persistent left parietal headache for more than 10 years, which has been worsening over the past few days with no specific identifiable trigger. The diagnosis of nummular headache is challenging and confusing due to the rare occurrence and inadequate references in the literature. Appropriate imaging was done, which revealed no pathology that could explain the headache presentation. These findings are consistent with our diagnosis of nummular headache, and helped in the successful management of the case.

Pro and anti-inflammatory cytokine levels (TNF-α, IL-1ß, IL-6 and IL-10) in rat model of neuroma.

Traumatic neuroma is neuronal tissue proliferation developed in a nerve injury site, often associated with increased sensitivity and spontaneous or evoked neuropathic pain. The mechanisms leading to the disorganized nerve proliferation are not completely understood, though inflammation in the injured nerve vicinity most likely has a role in the process. Inflammatory cytokines are also known to be involved in the maintenance and development of post-traumatic and neuropathic pain. The goal of this study was to quantify and compare pro and anti-inflammatory cytokines (TNF-α, IL-1ß, IL-6 and IL-10) levels in nerves that formed neuromas and nerves that did not, following sciatic nerve transection. A total of 30 rats were used in this study. Twenty rats underwent sciatic nerve transection and 10 underwent sham surgery. Six weeks post-surgery nerve sections were collected and histologically evaluated for neuroma formation. The samples were then classified as neuroma, non-neuroma and sham groups. TNF-α, IL-1ß, IL-6 and IL-10 levels were measured in the nerves employing ELISA. TNF-α levels were significantly higher in both neuroma and non-neuroma-forming injured nerves compared to the sham group. IL-1ß and IL-6 levels were significantly higher in the neuroma-forming nerves compared to the sham group. IL-10 levels were significantly higher in the non-neuroma group compared to the sham group. In conclusion IL-6, and IL-1 ß may have a role in the formation of traumatic neuroma while IL-10 may inhibit neuroma formation.

Burning mouth syndrome: Current concepts.

Burning mouth syndrome (BMS) is a chronic pain condition. It has been described by the International Headache Society as "an intra-oral burning or dysesthetic sensation, recurring daily for more than 2 h/day for more than 3 months, without clinically evident causative lesions." BMS is frequently seen in women in the peri-menopausal and menopausal age group in an average female/male ratio of 7:1. The site most commonly affected is the anterior two-thirds of the tongue. The patient may also report taste alterations and oral dryness along with the burning. The etiopathogenesis is complex and is not well-comprehended. The more accepted theories point toward a neuropathic etiology, but the gustatory system has also been implicated in this condition. BMS is frequently mismanaged, partly because it is not well-known among healthcare providers. Diagnosis of BMS is made after other local and systemic causes of burning have been ruled out as then; the oral burning is the disease itself. The management of BMS still remains a challenge. Benzodiazepines have been used in clinical practice as the first-line medication in the pharmacological management of BMS. Nonpharmacological management includes cognitive behavioral therapy and complementary and alternative medicine (CAM). The aim of this review is to familiarize healthcare providers with the diagnosis, pathogenesis, and general characteristics of primary BMS while updating them with the current treatment options to better manage this group of patients.

Knowledge and awareness about temporomandibular disorder among dentists in India: Questionnaire study and review.

AIM: Temporomandibular disorders (TMD) comprise ailments involving the jaw joint (temporomandibular joint) and its associated anatomical structures. The complexity involved in TMD is primarily due to its broad spectrum of conditions, clinical signs and symptoms variability, and multifactorial etiology. Considering the above, the present study was performed to help understand the prevailing knowledge and awareness of TMD among Indian dentists in the context of the new specialty "orofacial pain". SETTINGS AND DESIGN: Questinnaire study and review. MATERIALS AND METHODS: The questionnaire was distributed using a web-based portal nationwide among Indian dentists. Dentists were invited to participate, clearly stating that the intent and purpose of the questionnaire was to record the existing knowledge and awareness concerning temporomandibular disorders among Indian dentists. The questionnaire was segregated into three sections: pathogenesis, diagnosis, and management of TMDs. The questions were recorded using a Likert three-point scale (1=agree; 2=disagree; 3=not aware). 310 dentists participated in the survey, among which 105 were general dentists (BDS [Bachelor of Dental Surgery] graduates), and 205 were dentists with specialist training (MDS [Masters of Dental Surgery] graduates). STATISTICAL ANALYSIS USED: The results obtained from the study participants was used to calculate the percentage and frequency, following which tabulations were made based on graduate type and clinical experience. The values obtained from all three sections were recorded, and the responses were analysed using Pearson's Chi-Square test with statistical significance kept at P < 0.05. RESULTS: Results of the study disclosed that only 58.1% of general dentists and 46.8% of specialists were confident in handling temporomandibular disorder patients. Splint therapy was the preferred treatment modality for general dentists, whereas dentists with specialist training preferred occlusal rehabilitation. CONCLUSION: The results of the current survey indicate that Indian dentists lack sufficient training in dental schools on all three sections and face difficulty diagnosing and treating TMDs.

Sleep bruxism: The past, the present, and the future-evolution of a concept.

BACKGROUND: The concept of sleep bruxism (SB) has evolved exponentially over the past several decades. Many theories and hypotheses have been proposed as to the definition, pathophysiology, and management of SB, from the early 1960s through the present. The role of peripheral factors, such as dental occlusion, in the pathogenesis of SB has been discarded. TYPES OF STUDIES REVIEWED: The authors searched several electronic databases (ie, PubMed, Google Scholar, Web of Science, Embase, and Ovid MEDLINE) for studies on bruxism. The search was conducted from January 1961 through May 2023 and yielded 4,612 articles, of which 312 were selected for comprehensive review after eliminating duplicates and nonfocused articles. RESULTS: There has been an evident progressive shift from the role of peripheral factors, such as dental occlusion, to more central factors, such as the involvement of a central pattern generator as well as the autonomic nervous system, in the genesis of bruxing movements. There is continued robust interest in the dental community to elucidate the contributing factors involved in SB. CONCLUSIONS AND PRACTICAL IMPLICATIONS: The neurophysiology of SB appears to be leaning more toward central rather than peripheral factors. There is increasing evidence of the role of the autonomic nervous system, genetics, and comorbidities in the genesis of SB. The scientific literature seems to refute the role of dental occlusion in the causation of bruxing movements. As per the literature, there has been a paradigm shift in the definition and genesis of SB and its possible dental implications and management, which also highlights the need for succinct scientific studies in this regard.

Temporomandibular Disorders and Dental Occlusion: What Do We Know so Far?

Throughout the recorded history in the literature of temporomandibular disorders (TMD) there have been a variety of opinions as to its primary cause. Those supporting an occlusal basis of TMD opined that occlusal dysfunction is either the primary cause for or a significant etiopathogenic factor in the causation of TMD. Most of the current literature, however, points to evidence in another direction and questions the causal role of occlusion and occlusal disharmony in TMD etiopathogenesis. Recognition of this evidence-based literature is paramount in eliminating and preventing the chances of overtreatment of patients with TMD.

Temporomandibular Disorders Within the Context of Sleep Disorders.

This paper provided an overview of the knowledge on the relationship between temporomandibular disorders (TMDs) and the main sleep conditions and disorders of dental interest, namely, sleep bruxism (SB), sleep apnea, and gastroesophageal reflux disease (GERD). It emerged that although the topic of SB as a possible detrimental factor for the stomatognathic structures has been the most studied, evidence is growing that SB, obstructive sleep apnea, and GERD, all belong to a circle of mutually interacting sleep disorders and conditions that, in turn, may be associated with TMDs. The pathophysiology of the cause-and-effect relationships, if existing, has to be elucidated yet.

Temporomandibular Joint Disorder Comorbidities.

Comorbidity is a distinct additional condition that either existed or exists during the clinical course of a patient afflicted by the condition/entity in question. The clinician attempting to manage temporomandibular joint disorder (TMD) and TMD pain must realize that recognition and management of the comorbidities are essential to the successful management of the same with optimal pain control. When TMD presents with multiple comorbidities, the task for the clinician becomes more complex. It is the hope of the authors that this condensed version of TMD-associated comorbidities acts as a primer for understanding the significance of the same in pain management.

Expression of EGFR and survivin in ameloblastoma, odontogenic keratocyst and calcifying odontogenic cyst - An immunohistochemical study.

Context: Odontogenic lesions have diverse biological behaviour which is characterised by local invasiveness, and a high recurrence rate. EGFR and survivin was found to be involved in the aggressiveness, recurrences and metastasis of a variety of epithelial malignancies. Aims: To assess and compare the expression of EGFR and survivin in Ameloblastoma (AB), Odontogenic keratocyst (OKC) and Calcifying odontogenic cyst (COC). Settings and Design: The study's goal was to use immunohistochemistry to assess the qualitative and quantitative expression of EGFR and survivin and to correlate their expression patterns in AB, OKC and COC. Methods and Material: Study included 30 AB, 15 OKC and 10 COC. All the slides were immunohistochemically analysed for qualitative, quantitative and semi-quantitative data. In each group, the presence of EGFR and survivin was assessed in terms of stain localisation, intensity and percentage of positive cells. Statistical Analysis Used: Data were analysed using Chi-square test and one-way ANOVA, P value < 0.05 was considered statistically significant. Results: EGFR positivity was found in all cases. Survivin was found to be 96% positive in AB and 100% positive in OKC and COC. Both EGFR and survivin showed predominant cytoplasmic staining. All the slides that are stained with EGFR are also stained with survivin. The intensity varied significantly between the layers. OKC showed higher immunoreactive scores (IRSs). Conclusions: The current study provides insight into the role of EGFR and survivin in the pathogenesis of AB, OKC and COC. OKC appears to be more aggressive than ameloblastoma and COC, owing to its higher IRS.

Systemic Factors in Temporomandibular Disorder Pain.

The science of temporomandibular disorder (TMD) pain and its management has gone through significant changes during the last several decades. The authors strongly feel that the effect of systemic factors influencing TMD pain has been largely overlooked and poorly accounted for, even in established pain-management programs and protocols. The hope is that this article will act as a wake-up call for the pain management community to consider the importance of adequate knowledge of the systemic factors that affect the experience of TMD pain by the patient.

Temporomandibular Disorders: Implications in Restorative Dentistry and Orthodontics.

Over the past several decades, the science of restorative/reconstructive dentistry and orthodontics has evolved tremendously, following sound principles passed down from robust literature and scientific rationale. These principles have been solid and instrumental in enhancing dentistry, from a single tooth restoration to complex full-mouth rehabilitations. However, it must be noted that some of the principles and philosophies followed over these decades have been questioned based on the advances in science, technology, and evidence-based medicine. The scenario became complex when clinicians were faced with the question of guidance for optimum joint and muscle health as related to restorative dentistry and orthodontics.

Needlestick injuries in dentistry: Time to revisit.

BACKGROUND: Blood-borne pathogens (BBPs) are infectious microorganisms that are found in human blood and can cause diseases in humans. Health care workers such as physicians, surgeons, nurses, dentists, dental students, dental assistants, laboratory technicians, personnel handling infectious waste, and other health care employees are at increased risk of exposure to these pathogens. Percutaneous injuries from needles or other sharp objects are the major sources of BBPs in the workplace. Needlestick injuries (NSIs) have the most potential to transmit and have the easiest mode of transmission of BBPs. TYPES OF STUDIES REVIEWED: The authors searched electronic databases (PubMed, Web of Science, Google Scholar, Scopus, Embase, MEDLINE-Ovid) for studies and articles focused on the various aspects of NSIs, their possible causes, prevention, and management protocols. RESULTS: There is a lack of literature on the global prevalence of NSIs among dental practitioners and underreporting of NSIs by clinicians. The authors also found that dental students and inexperienced practitioners were the most vulnerable. They found apparent inconsistencies in guidelines and recommendations from various regulatory and statutory agencies in charge of limiting and managing NSIs. CONCLUSION: The most significant occupational risks for health care workers globally are NSIs. Dentists are recognized as one of the high-risk groups for exposure to NSIs. Although the reporting rate was noticeably low, the frequency of NSIs among dental students was alarmingly high. PRACTICAL IMPLICATIONS: Appropriate and succinct training of dental health care workers is crucial for prevention and management of NSIs. It is recommended that dentists familiarize themselves with recommendations from such agencies and organizations as the Center for Disease Control and Prevention, Occupational Safety and Health Administration, and American Dental Association.

The relation of temporomandibular disorders and dental occlusion: a narrative review.

OBJECTIVE: The term temporomandibular disorders (TMDs) encompasses a variety of disorders of the temporomandibular joint (TMJD) and the associated musculature (MMD). Occlusion and its role in the genesis of TMDs is one of the most controversial topics in this arena. The objective of the narrative review was to summarize the implications of TMDs and its relationship to dental occlusion in two scenarios: 1) TMD as an etiologic factor in dental occlusal changes; 2) The role of dental occlusion as a causative factor in the genesis of TMDs. DATA SOURCES: Indexed databases were searched from January 1951 to August 2021 using the terms TMJ, TMD, temporomandibular disorders, temporomandibular joint, and dental occlusion. CONCLUSION: There is lack of good primary research evaluating true association and showing the cause-and-effect relationship between dental occlusion and TMD. Systematic reviews suggest that the role of occlusion as a primary factor in the genesis of TMDs is low to very low. However, a variety of TMDs can lead to secondary changes in dental occlusion. Distinction between the two is paramount for successful management.

Facial pain and trigeminal neuralgia secondary to metastasis: A case report.

BACKGROUND AND OVERVIEW: Trigeminal neuralgia due to a metastatic lesion is a relatively rare occurrence. These cases pose a diagnostic challenge for the clinician due to a complex clinical presentation. CASE DESCRIPTION: The authors describe the case of a 65-year-old woman with left-sided facial pain and occasional numbness. The patient also reported autonomic features associated with facial pain, facial muscular weakness, and hearing and visual impairment. The patient's history of breast cancer, for which she was receiving treatment for bone metastasis, warranted diagnostic imaging. Magnetic resonance imaging with and without contrast was performed and revealed a possible metastatic lesion. CONCLUSIONS AND PRACTICAL IMPLICATIONS: Unusual clinical features must be identified, and prompt appropriate imaging is instrumental in making at an accurate diagnosis and management plan.

Dysgeusia: A review in the context of COVID-19.

BACKGROUND: Taste disorders in general, and dysgeusia in particular, are relatively common disorders that may be a sign of a more complex acute or chronic medical condition. During the COVID-19 pandemic, taste disorders have found their way into the realm of general as well as specialty dentistry, with significance in screening for patients who potentially may have the virus. TYPES OF STUDIES REVIEWED: The authors searched electronic databases (PubMed, Embase, Web of Science, Google Scholar) for studies focused on dysgeusia, ageusia, and other taste disorders and their relationship to local and systemic causes. RESULTS: The authors found pertinent literature explaining the normal physiology of taste sensation, proposals for suggested new tastes, presence of gustatory receptors in remote tissues of the body, and etiology and pathophysiology of taste disorders, in addition to the valuable knowledge gained about gustatory disorders in the context of COVID-19. Along with olfactory disorders, taste disorders are one of the earliest suggestive symptoms of COVID-19 infection. CONCLUSIONS: Gustatory disorders are the result of local or systemic etiology or both. Newer taste sensations, such as calcium and fat tastes, have been discovered, as well as taste receptors that are remote from the oropharyngeal area. Literature published during the COVID-19 pandemic to date reinforces the significance of early detection of potential patients with COVID-19 by means of screening for recent-onset taste disorders. PRACTICAL IMPLICATIONS: Timely screening and identification of potential gustatory disorders are paramount for the dental care practitioner to aid in the early diagnosis of COVID-19 and other serious systemic disorders.

Bilateral intraoral traumatic neuroma: Case report of a diagnostic challenge.

Oral traumatic neuromas (TrNs) are relatively rare lesions and they originate from a damaged nerve. They present a diagnostic challenge, due to the complex clinical features that may mimic odontogenic, musculoskeletal, and other neuropathic pain conditions. We describe an interesting and challenging case of painful bilateral intraoral lesions in a 56-year-old South Indian female patient who presented with clinical features consistent with TrN lesions bilaterally, in relation to different branches of the trigeminal nerve. The patient had undergone numerous aggressive dental treatments and interventions over the past three decades, with little or no pain relief. Topical treatment with lidocaine gel utilizing a custom-made neurosensory stent rendered the patient significant and sustained pain relief.