Association between orofacial granulomatosis and Crohn's disease in children:Systematic review

AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.

Orofacial Human Bite: A Six Year Review of Cases from Komfo Anokye Teaching Hospital

Background: The orofacial region of the human body is usually not protected during a fight, making it prone to several injuries including human bite. Patients with human bite injury are often either intoxicated or are known to their assailant, and this makes the process of obtaining a reliable history especially about the aetiology difficult. In 2002, a study estimated the rate of infection secondary to human bite to be about 10%. Aim/Objective: The aim of the current study was to have a general overview of orofacial human bites seen at our unit including the aetiology, presentations, anatomic location, treatment and the treatment outcome. Results: Total number of cases was 127 over the six year period. Age range for females was 15 - 61 years with an average of 29.9. Age range for males was from 17 to 60 years and an average of 30.2 years. There were 31 males and 96 females, giving a male to female ratio of approximately 1:3. All of the reported cases resulted from a fight. Most of the offenders are known to the patients. Majority of the cases, except those infected at the time of presentation, were treated on the same day of presentation under local anaesthesia. Relationship of victim to the offender: Spouse/sexual partners were 21, rivals formed 70, known persons to the victim were 26 and strangers were 10. Sex distribution of offenders and victims: Females who bit females were 86 followed by females who bit males (24), males who bit females (10) and males who bit males (7). Conclusion: Most of the offenders are known to the patients. Majority of the cases, except those infected at the time of presentation, were treated on the same day of presentation under local anaesthesia after they had received antibiotics and anti-tetanus prophy-laxis. Early repair is now the recommended mode of treatment for human bites of the orofacial region, accompanied with good oral hygiene instructions and broad spectrum antibiotics and metro-nidazole.

Clinicopathologic Analysis of Biopsied Orofacial Lesions Seen in Children in a Tertiary Health Centre in Port Harcourt: An 11-Year Review

Background: Orofacial lesions in children and adolescents are diverse and show variation in prevalence from one region to another. Previous Nigerian studies on orofacial lesions in this age group have focused on tumours and tumour-like lesions, with the exclusion of cysts and some inflammatory/reactive lesions. The aim of this study was to describe the demographic characteristics of all biopsied orofacial lesions seen in children and adolescents aged 16 years and below. Materials and Methods: This retrospective study reviewed histopathology records over an 11-year period for histologically diagnosed lesions in patients aged 16 years and below. All such cases were extracted, and the age, gender, site and histopathologic diagnosis were recorded for each case. Lesions were categorized into three groups: inflammatory/reactive, cystic and neoplastic, with the neoplastic lesions sub-divided into benign and malignant. Patients were categorized into three age groups: 0 - 5 years, 6 - 12 years and 13 - 16 years. Data analysis was done using SPSS version 23. Results: A total of 109 biopsied lesions were seen in children ≤ 16 years during the period under review, representing 20.8% of all biopsied lesions during the same period. The patients’ age ranged from 4 months to 16 years, with a mean age of 10.4 ± 4.1 years and the lesions were encountered most frequently in the 6 - 12 years age group (45.9%). There was no gender predilection and the mandible (30.3%), maxilla (20.2%) and gingiva (22.9%) were the most frequently involved sites. Neoplastic, Inflammatory/reactive and Cystic lesions constituted 52.3%, 35.8% and 11.9% of cases respectively. The most frequent histopathologic diagnoses were pyogenic granuloma (16.5%) and unicystic ameloblastoma (12.8%). The most common inflammatory/reactive lesion was pyogenic granuloma (46.2%) and it was significantly associated with the gingiva (p = 0.000). Unicystic ameloblastoma was the most common neoplastic lesion, while dentigerous cyst was the most frequently encountered cystic lesion. Conclusion: Most orofacial lesions in children aged 16 years or below are either benign neoplasms or inflammatory/reactive lesions, with the three most common diagnoses being pyogenic granuloma, unicystic ameloblastoma and dentigerous cyst. The mandible is the most commonly affected site.

An audit of Paediatric Orofacial Lesions at the Kilimanjaro Christian Medical Centre in Moshi, Tanzania

Objective: To audit and categorize pathological lesions and conditions that occurred in the orofacial region among children aged up to 15 yrs. Setting: Kilimanjaro Christian Medical Centre, Moshi, Tanzania. Design: Retrospective cross-sectional audit based on archival records and material between 1985 to 2005. Results: Biopsy results were generated into 11 categories whence most common lesions encountered were in the categories of soft tissue benign neoplasms (35.1%) and soft tissue malignant neoplasms (21.8%). Remarkably, Burkitt’s lymphoma (BL) in the category of malignant soft tissue neoplasia constituted 11.8% of all the lesions biopsied while haemangiomas and tuberculous adenitis comprised 8.1% and 3.8% respectively. The age groups revealed the highest burden (37.1%) among the 0 to 5-year-olds followed by the 11 to 15-(34.5%) and 6 to 10-year-olds (28.4%). The orofacial site distribution among the 211 biopsied cases included 62.1% in the mandibular 29.9% in the maxillary region and 8% in the tongue areas. Malignant neoplasms of the bone were rare and all were diagnosed in the mandible. Overall, malignant neoplasms of soft tissue were significantly more in the age group of 6 - 10 years as well as in males than females. On the other hand, significantly more benign soft tissue neoplasms occurred in females than in males. Main Outcome Measure: There is great diversity and preponderance of soft tissue than skeletal orofacial lesions on the present audit. Significantly, clinicians should maintain high index of suspicion regarding the remarkably high frequency of diagnosing BL and tuberculous lymphadenitis in such a population in this era of HIV infection/AIDS.

Non-Random Distribution of Human Orofacial Clefts in Ghana: Gene-Environment Interactions

Human orofacial clefts (OFCs) are congenital anomalies that result from the breakdown of normal mechanisms that regulate the formation of the human face. They could be syndromic or non-syndromic, with a global incidence of 1:700 per live births. Environmental and genetic factors are thought to play various roles in the aetiology of OFCs. This study seeks to establish the diversity, distribution and pattern of inheritance of OFCs as well as environmental and other risk factors associated with OFCs in a Ghanaian population. A family-based, descriptive cross-sectional study that employed an interview-based survey questionnaire was used to obtain information from 467 families with history of OFCs. We employed chi-square statistics to analyse the data and used graphs to interpret the data. All previously reported subphenotypes of OFCs were observed by the present study. Clinically, about 12% of clefts in the study population were syndromic. The most common syndromic forms observed were Pierre Robin Sequence, cleft-with-club foot abnormalities and Van der Woude Syndrome. Only about 5% of clefts in the study cohort were familial. The study also established that lower level of education of parents, poverty, late antenatal care and dietary folate deficiency are major environmental factors associated with clefts in the Ghanaian population. In conclusion, OFCs are non-randomly distributed in Ghana and folate deficiency could likely be a source of genetic mutations and “epimutations” that cause OFCs, since folate is essential for DNA methylation, replication and repair as well as histone modification.

Orofacial pain and fibromyalgia pain:Being aware of comorbid conditions

Orofacial pain originating from myofascial pain of temporomandibular disorders is the second most common source of pain, after tooth pain. However, diagnosis of myofascial pain is challenging due to its characteristic referral pattern. Furthermore, pain arising from structures in the orofacial region may be a presentation of fibromyalgia and treatment directed at temporomandibular disorders fails to alleviate the pain. Similarly, patients with fibromyalgia may present with pain in the orofacial region. The physician in this case should be aware of temporomandibular disorders, its characteristic findings and treatment approaches that might be included in the treatment plan.

Serotonin 2C Receptor Alternative Splicing in a Rat Model of Orofacial Neuropathic Pain

Abnormal serotonin 2C receptor (5HTR2C) alternative splicing and RNA editing are involved in the etiology of pain disorders. Functional 5HTR2C can only be generated when alternative exon Vb is included within the mRNA;the small nucleolar RNA RBII-52 is complementary to exon Vb and promotes its inclusion. The expression of HBII-52 (the human equivalent of RBII-52) is reduced in Prader-Willi syndrome, patients of which have a high pain threshold. Here, we measured the pain threshold in a rat model of orofacial neuropathic pain and related it to the expression levels of wild-type and variant 5HTR2C and RBII-52. We generated an infraorbital nerve loose ligation model of neuropathic pain in rats and measured the pain threshold of the animals using mechanical stimulation with von Frey filaments. We then sacrificed the animals and examined the RNA levels of 5HTR2C and RBII-52 in the cervical spinal cord by real-time PCR. On post-injury day 28, pain threshold values in injured rats were significantly lower than in sham-operated or na?ve animals. The levels of total and exon Vb-skipped 5HTR2C mRNA were significantly lower in injured rats than in that sham-operated or na?ve rats, and the ratio of exon Vb-skipped 5HTR2C to total 5HTR2C was significantly higher. There were no significant differences in RBII-52 expression among the groups. Our data suggest that neuropathic pain induces serotonergic dysfunction mediated by 5HTR2C alternative splicing. 5HTR2C might be subject to complicated and fine regulation both by RNA editing and by alternative splicing.

Orofacial Cysts at Komfo Anokye Teaching Hospital, Ghana

Background: Information about orofacial cysts from African populations is scarce and there are only a few studies available regarding the prevalence of these lesions in the West African sub-region. The purpose of the present study is to determine the distribution and prevalence of all histologically diagnosed orofacial cysts in Kumasi, Ghana. Aim: To determine prevalence, sex, age and anatomic distribution of orofacial cyst seen at the oral and maxillofacial unit in Komfo Anokye Teaching hospital (KATH). Method: This is a retrospective study, which examined histologically diagnosed lesions including orofacial cysts. The study duration was from 1999 to 2010 September inclusive. Results: There were 37 odontogenic cysts constuting 6.5%, of all orofacial lesions. There were 18 non-odontogenic cysts i.e. 3.1% of all lesions diagnosed during the study period. The odontogenic cysts comprised 19 (51.4%) developmental cysts and 18 (48.6%) inflammatory cysts. Male-to-female ratio for the orofacial cysts was 1:1 and the mean age was 36.7 years. Conclusion: There is low prevalence of the odontogenic cysts, which is consistent with findings from other African studies. Although radicular cysts accounted for the majority of orofacial cysts in this study, the prevalence of radicular cysts is low compared to reports from developed countries.

Short-term effects of repetitive transcranial magnetic stimulation on sleep bruxism-a pilot study

The purpose of this study was to investigate the effects of repetitive transcranial magnetic stimulation （rTMS） on patients with sleep bruxism （SB）. Twelve patients with SB were included in an open, single-intervention pilot study, rTMS at 1 Hz and an intensity of 80% of the active motor threshold was applied to the ‘hot spot＇ of the masseter muscle representation at the primary motor cortex bilaterally for 20 rain per side each day for 5 consecutive days. The jaw-closing muscle electromyographic （EMG） activity during sleep was recorded with a portable EMG recorder at baseline, during rTMS treatment and at follow-up for 5 days. In addition, patients scored their jaw-closing muscle soreness on a 0-10 numerical rating scale （NRS）. Data were analysed with analysis of variance. The intensity of the EMG activity was suppressed during and after rTMS compared to the baseline （P = 0.04; P = 0.02, respectively）. The NRS score of soreness decreased significantly during and after rTMS compared with baseline （P 〈 0.01）. These findings indicated a significant inhibition of jaw-closing muscle activity during sleep along with a decrease of muscle soreness. This pilot study raises the possibility of therapeutic benefits from rTMS in patients with bruxism and calls for further and more controlled studies.

Burning Mouth Syndrome

Most clinicians dread seeing the patient presenting with a primary complaint of a burning pain on one or more oral mucosal surfaces. Unlike most other clinical conditions presenting in a dental office, burning mouth syndrome is poorly understood with few evidence based remedies. More recently, advances have been made towards clarifying the possible etiology of the disorder and testing the possible therapeutic modalities available. This article attempts to summarize the ＂state of the art＂ today.

Accurate diagnosis of prenatal cleft lip/palate by understanding the embryology

Cleft lip with or without cleft palate(CP) is one of the most common congenital malformations. Ultrasonographers involved in the routine 20-wk ultrasound screening could encounter these malformations. The face and palate develop in a very characteristic way. For ultrasonographers involved in screening these patients it is crucial to have a thorough understanding of the embryology of the face. This could help them to make a more accurate diagnosis and save time during the ultrasound. Subsequently, the current postnatal classification will be discussed to facilitate the communication with the CP teams.

Melkersson-Rosenthal syndrome

Melkersson-Rosenthal syndrome(MRS) is a rare, noncaseating granulomatous disorder of unknown etiology and undefined diagnostic criteria. The classical triad of recurrent orofacial edema, relapsing facial paralysis, and fissured tongue, is not frequently seen in its complete form, and many patients remain misdiagnosed or undiagnosed for years. The purpose of this study is to review the findings in the literature describing theMelkersson-Rosenthal syndrome with aim to identify its clinical and histopathological characteristics and correlate them with definitive diagnostic criteria and effective treatment modalities. A systematic review and analysis of more than 100 publications met eligibility criteria performed by the authors. Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Its coexistence with of facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Many investigators suggest cheilitis granulomatosa as a monosymptomatic form of MRS, while patients with facial palsy and fissured tongue, without orofacial edema, should not be considered having MRS.Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment.

Epidemiology, Clinical Aspects and Management of Cleft Lip and/or Palate in Burkina Faso: A Humanitarian Pediatric Surgery-Based Study

Background: Cleft lip and/or palate are the most common orofacial malformations. Many studies, especially in developed countries have been conducted on this malformation, but in Burkina Faso, data are scarce and they are not specific to children. The aim of this study was to report the epidemiological, clinical and therapeutic aspects of cleft lip and/or palate in children in a low-income country. Materials and Method: The authors conducted a retrospective descriptive study based on data of three humanitarian missions of pediatric reconstructive facial surgery which took place in 2007, 2010 and 2014 at Clinique El Fateh-Suka in Ouagadougou, Burkina Faso. All children of 0 - 14 years of age, presenting with cleft lip and/or palate, were included in the study. Results: A total of 185 cases of cleft lip and/or palate were seen during these three humanitarian surgery missions. There were 100 boys and 85 girls. The average age of the children was 2.4 ± 3.2 years [0 - 12 years];there were 8.7% newborns. The commonest type of cleft was cleft lip and palate (49.7%) followed by isolated cleft lip (48.7%) and isolated cleft palate (1.6%). The left side was the most affected (49.2%). In 21.1% of cases, clefts were associated with other congenital malformations. In total, 150 of 185 (81.1%) children underwent surgery and there were no postoperative complications reported. Conclusions: Epidemiological and clinical characteristics of cleft lip and/or palate observed in this study are not very different from those described elsewhere in Africa. However, in our conditions, there are circumstances and structural factors which hinder the diagnosis and constitute challenges that must be addressed for adequate management of this congenital, highly disfiguring malformation.

Meige Syndrome: An Eternal Diagnostic Confusion

Meige syndrome is an idiopathic dystonia characterized by combination of blepharospasm and involuntary movements of the lower facial and/or masticatory (jaw) muscles. The condition is rare and has a variety of clinical presentations which often lead to its misdiagnosis. We report a case of Meige syndrome repeatedly misdiagnosed and treated unsuccessfully as conversion disorder.

Non-odontogenic toothache revisited

Although pain of dental origin is the most common orofacial pain, other non-odontogenic pains can af-fect the orofacial region and occasionally mimic den-tal pain. These non-odontogenic pains may pose a diagnostic dilemma for the dental practitioner who routinely diagnoses and treats dental pain. Knowledge of the various non-odontogenic pains will ultimately prevent misdiagnosis and the delivery of incorrect and sometimes irreversible and invasive procedures to patients. The purpose of this article is to review the clinical presentations of the various types of non-odontogenic pains which may be mistaken as dental pain: myofascial, cardiac, sinus, neurovascular, neuropathic, neoplastic and psychogenic pain.

Treatment of mouth and jaw diseases with intralesional steroid injection

Many lesions of the oral region are treated with surgical methods such as curettage and resection. Chemotherapy and radiation therapy with or without surgical intervention can be used as an adjunct in some cases. Intralesional steroid injection is a conservative procedure which is already used in various regions of the body andjoints. This technique is used also for a number of mouth and jaw lesions. Localized langerhans cell histiocytosis, central giant cell granuloma, oral submucous fibrosis, oral lichen planus, lichen sclerosus of the oral mucosa, lymphatic malformations and orofacial granulomatosis can be considered among these diseases. The purpose of this review is to investigate the effects of intralesional steroid injections in the treatment of oral diseases.

Service Evaluation of a New Physiotherapy Management Service for Patients with Chronic Temporomandibular Dysfunction

Aims: This service evaluation explored and reported findings from a new multi-disciplinary service where physiotherapists were incorporated into an orofacial team who managed patients with chronic Temporomandibular Dysfunction (TMD). Methods: We collected data before the physiotherapists’ involvement and on discharge from physiotherapy management. Outcomes were patient-reported pain (numerical rating scale (NRS 0 to 10)), patient specific functional score (PSFS), health-related quality of life (EQ-5D) and range of mandibular depression (mm). Exploratory analyses compared baseline to follow-up scores on discharge from physiotherapy. Results: 79 patients (mean age 40 (SD 18);female 87%) received physiotherapy. At discharge, there was a mean reduction in pain: 2.8 (95% CI 2.0 to 3.6), improvement in function: ﹣4.0 (95% CI ﹣4.7 to ﹣3.3), improvement in health: ﹣0.139 (95% CI ﹣0.196 to ﹣0.082) and increase in mandible depression: ﹣6.6. (95% CI ﹣9.1 to ﹣4.1). Conclusions: The results provide early, exploratory evidence that patients with chronic temporomandibular dysfunction treated by physiotherapists achieve clinically important changes in pain, function and health in the short term.

Machine Learning Models for Genetic Risk Assessment of Infants with Non-syndromic Orofacial Cleft

The isolated type of orofacial cleft, termed non-syndromic cleft lip with or without cleft palate(NSCL/P), is the second most common birth defect in China, with Asians having the highest incidence in the world. NSCL/P involves multiple genes and complex interactions between genetic and environmental factors, imposing difficulty for the genetic assessment of the unborn fetus carrying multiple NSCL/P-susceptible variants. Although genome-wide association studies(GWAS)have uncovered dozens of single nucleotide polymorphism(SNP) loci in different ethnic populations, the genetic diagnostic effectiveness of these SNPs requires further experimental validation in Chinese populations before a diagnostic panel or a predictive model covering multiple SNPs can be built. In this study, we collected blood samples from control and NSCL/P infants inHan and Uyghur Chinese populations to validate the diagnostic effectiveness of 43 candidate SNPs previously detected using GWAS. We then built predictive models with the validated SNPs using different machine learning algorithms and evaluated their prediction performance. Our results showed that logistic regression had the best performance for risk assessment according to the area under curve. Notably, defective variants in MTHFR and RBP4, two genes involved in folic acid and vitamin A biosynthesis, were found to have high contributions to NSCL/P incidence based on feature importance evaluation with logistic regression. This is consistent with the notion that folic acid and vitamin A are both essential nutritional supplements for pregnant women to reduce the risk of conceiving an NSCL/P baby. Moreover, we observed a lower predictive power in Uyghur than in Han cases, likely due to differences in genetic background between these two ethnic populations.Thus, our study highlights the urgency to generate the HapMap for Uyghur population and perform resequencing-based screening of Uyghur-specific NSCL/P markers.

Clinical outcome of dental implants placed in fibula-free flaps for orofacial reconstruction

Background Functional reconstruction of the jaw defect due to tumor resection poses a challenging problem in maxillofacial surgery. The osteocutaneous fibula free flap in combination with simultaneous or second stage insertion of dental implants has exhibited growing popularity for such reconstructions. This study was aimed at evaluating the clinical status and the success rates of dental implants inserted in fibula-free flaps for orofacial reconstruction following ablation of tumors. Methods We conducted a clinical follow-up study based on 29 patients after oral tumor surgery, who received vascularized fibula bone grafts and endosseous implants for functional jaw reconstruction during a 5-year period. The follow-up protocol included clinical examination and radiological evaluation. The clinical records of the patients were reviewed retrospectively. Information on treatment modalities, dentition, implant parameters, and prostheses was collected and analyzed. Results In general, a high primary stability for implants placed into the free fibula grafts was achieved. The 1-year and 5-year cumulative survival rates of the implants were 96% and 91%, respectively, using the Kaplan-Meier method. The 1-year and 5-year cumulative success rates of implants respectively. The main reasons for failure of the dental proliferation. The fibula flap presents many advantages for implant-supported prosthetic rehabilitation difficult. placed into the fibula bone grafts were 95% and 87%, replants were infection, tumor recurrence and soft tissue implant placement, but its limited height sometimes makes Conclusions Vascularized fibula bone grafts provide a firm basis for the placement of dental implants in jaw reconstruction. Implants placed in fibula bone grafts were shown to integrate normally. The double-barrel technique, or increasing the height of the fibula flap by vertical distraction osteogenesis before implant placement in the mandible, is desirable from a functional and esthetic point of view.

Connexin 36 Mediates Orofacial Pain Hypersensitivity Through GluK2 and TRPA1

Trigeminal neuralgia is a debilitating condition,and the pain easily spreads to other parts of the face.Here,we established a mouse model of partial transection of the infraorbital nerve(pT-ION)and found that the Connexin 36(Cx36)inhibitor mefloquine caused greater alleviation of pT-ION-induced cold allodynia compared to the reduction of mechanical allodynia.Mefloquine reversed the pT-IONinduced upregulation of Cx36,glutamate receptor ionotropic kainate 2(GluK2),transient receptor potential ankyrin 1(TRPA1),and phosphorylated extracellular signal regulated kinase(p-ERK)in the trigeminal ganglion.Cold allodynia but not mechanic al allodynia induced by pT-ION or by virusmediated overexpression of Cx36 in the trigeminal ganglion was reversed by the GluK2 antagonist NS 102,and knocking down Cx36 expression in Nav1.8-expressing nociceptors by injecting virus into the orofacial skin area of Nav1.8-Cre mice attenuated cold allodynia but not mechanic al allodynia.In conclusion,we show that Cx36 contributes greatly to the development of orofacial pain hypersensitivity through GluK2,TRPA1,and p-ERK signaling.