UNASSIGNED: Hyperplasia of the coronoid process is a rare condition, potentially leading to a mechanical mouth opening restriction. Diagnostic workup and treatment will be discussed based on 5 cases. This article presents 5 cases of true coronoid process hyperplasia. In addition, we reviewed accessible literature on the topic with special attention to pathophysiologic theories, surgical approach, and postoperative physiotherapy. The improvement in the maximal intercuspidal opening ranged from 4 and 31 mm. Greater maximal intercuspidal opening improvement was connected to compliant patients, while poor outcome occurred in the case of a patient that neither followed the recommendations for physical therapy nor showed up for his follow up appointments. The success of the therapy is defined by a long-lasting and stable improvement of the mouth opening compared to the preoperative situation. In the presented cases, the outcome was strongly dependent on the patients\' postoperative compliance. Based on the cases described, we conclude that a good outcome is accomplishable for patients using the methods presented, as long as patients cooperate well after surgery.Diagnostic workup in patients with trismus should be thorough to correctly diagnose rare entities such as coronoid hyperplasia. If treated correctly this condition has a good outcome, as long as the compliance of the patient is adequate.

Lateral palatal synechiae are rare congenital adhesions running from the free borders of the cleft palate to the lateral parts of the tongue or the oral cavity floor, typically found in cleft palate lateral synechiae syndrome. We present a case of congenital lateral palatal synechia associated with a cleft palate that we treated and followed up for 10 years. We present the long-term prognosis. We also discuss variations in intraoral synechiae associated with cleft palate and the etiology of lateral palatal synechiae through a literature review.

Syngnathia is a rare congenital anomaly that presents as fusion of the jaws. The aims of this study were to review the clinical profile of reported cases of syngnathia available in the literature with a view to suggesting a classification that may simplify the understanding of syngnathia and to propose an appropriate management protocol. A PubMed database search of articles published in English was conducted. Selected articles were analyzed according to year of publication, type of article, number of cases per article, patient biodata, description of syngnathia, and associated anomalies. Data were analyzed using IBM SPSS version 19; the level of statistical significance was set at P≤ 0.05. Cases were categorized according to a proposed classification, and a review of the management of congenital syngnathia from 153 previously reported cases (in 110 articles) was done to propose a treatment protocol based on the proposed classification model. The findings are presented under three headings: pre-surgical considerations, surgical (intraoperative) considerations, and post-surgical considerations. The proposed classification categorizes syngnathia into soft tissue union (type 1) and hard tissue union (type 2). These two types were found to have similar frequencies in the literature, while their management differed in terms of mode of anaesthesia and surgical approach.

In rare instances, cleft lip and palate occur in association with synechia, intraoral fibrous bands connecting the maxilla and mandible. The main concern in synechia is a restricted mouth opening that leads to airway and feeding problems. This study reports our experience in the treatment of 3 cases and includes a review of the literature. Three patients with intraoral fibrous bands received treatment and follow-ups in our center. Division of the bands and reconstruction of the lip and palate were successfully performed. All reported cases in the literature were collected for a summary of clinical presentations and as references for management. Early management of the synechia improves the mouth opening and facilitates the treatment of associated anomalies.

BACKGROUND: Mulvihill-Smith syndrome is a rare sporadic condition that was first recognized in 1975. A total of 11 cases have been described in the literature. The aim of this study was to describe the orofacial signs and dental anomalies, their frequency, and the relationship between Mulvihill-Smith syndrome and other progeroid syndromes via a review of the literature.
METHODS: A systematic PubMed search was performed to retrieve articles published between 1975 and the present day that described patients affected by Mulvihill-Smith syndrome. The search identified 14 articles, and data on 11 patients were extracted from the selected articles.
RESULTS: A total of 7 patients (63.6%) affected by Mulvihill-Smith syndrome were described as having a typical \"bird\" face. Dental abnormalities, including irregular shape, enamel defects, hypodontia, and taurodontism, were described in 6 patients (54.5%). All patients (100%) had multiple pigmented nevi on the face and a lack or thinning of subcutaneous tissue around the neck and face. Three patients with Mulvihill-Smith syndrome exhibited early onset of tumors of the gastrointestinal tract, including the tongue.
CONCLUSIONS: Mulvihill-Smith syndrome is a clinically complex disease that may be caused by a single gene mutation. Numerous different tissues of the body are affected. This analysis of the orofacial signs may help clinicians to diagnose this rare pathology.

The evidence to date regarding corticosteroid exposure in pregnancy and select pregnancy and birth outcomes is limited and inconsistent. The authors provide a narrative review of published literature summarizing the findings for oral clefts, preterm birth, birth weight, preeclampsia, and gestational diabetes mellitus. Whenever possible, the results are limited to oral or systemic administration with a further focus on use in autoimmune disease. Although previous studies of corticosteroid exposure in pregnancy reported an increased risk of oral clefts in the offspring, more recent studies have not replicated these findings.

BACKGROUND: Orofacial clefts (OFCs) are common human birth defects in China. However, studies on the prevalence of OFCs present inconsistent results. The overall prevalence and geographic distribution of OFCs are poorly described in China. Thus, we conducted a systematic review and meta-analysis to estimate the prevalence of OFCs.
METHODS: The systematic review and meta-analysis were conducted on the basis of an established protocol (PROSPERO 2015: CRD42015030198). We systematically searched for articles in four electronic databases, including Embase, PubMed, Wanfang Database, and China National Knowledge Infrastructure (CNKI) to identify relevant studies about prevalence of OFCs in China. Meta-analysis, including subgroup analysis, was conducted to estimate the pooled prevalence.
RESULTS: A total of 41 studies published between 1986 and 2015 were included in our analysis. The sample size ranged from 2,586 to 4,611,808 live births. The random-effects model of meta-analysis showed that the overall prevalence of OFCs in China was 1.4 per 1000 live births (95% confidence interval [CI], 1.1-1.7). In subgroup analysis based on geographic regions, we found that OFC prevalence in Southwest (2.3 per 1000 live births, 95% CI, 1.1-4.7) was higher than that in other regions of China. There were no significant time trends of OFCs during the study period (p-value = 0.47).
CONCLUSIONS: The overall prevalence of OFCs in China was 1.4 per 1000 live births. No significant secular trend of prevalence has been found in this analysis. Further studies need to be conducted to explore the etiology of OFC to better control the risk of this common birth defect. Birth Defects Research 109:1011-1019, 2017. © 2017 Wiley Periodicals, Inc.

BACKGROUND: We conducted this systematic review and meta-analysis to address the open question of a possible association between the socioeconomic level of the neighborhoods in which pregnant women live and the risk of Congenital Heart Defects (CHDs), Neural Tube Defects (NTDs) and OroFacial Clefts (OFCs).
METHODS: We searched MEDLINE from its inception to December 20th, 2015 for case-control, cohort and ecological studies assessing the association between neighborhood socioeconomic level and the risk of CHDs, NTDs and the specific phenotypes Cleft Lip with or without Cleft Palate (CLP) and Cleft Palate (CP). Study-specific risk estimates were pooled according to random-effect and fixed-effect models.
RESULTS: Out of 245 references, a total of seven case-control studies, two cohort studies and two ecological studies were assessed in the systematic review; all studies were enrolled in the meta-analysis with the exception of the two cohort studies. No significant association has been revealed between CHDs or NTDs and neighborhood deprivation index. For CLP phenotype subgroups, we found a significantly higher rate in deprived neighborhoods (Odds Ratios (OR) = 1.22, 95% CI: 1.10, 1.36) whereas this was not significant for CP phenotype subgroups (OR = 1.20, 95%CI: 0.89, 1.61).
CONCLUSIONS: In spite of the small number of epidemiological studies included in the present literature review, our findings suggest that neighborhood socioeconomic level where mothers live is associated only with an increased risk of CLP phenotype subgroups. This finding has methodological limitations that impede the formulation of firm conclusions, and further investigations should confirm this association.

BACKGROUND: Congenital intraoral synechias are adhesions of intraoral structures involving soft tissue. These conditions and another anomaly called ankyloblepharon filiforme adnatum, which is partial fusion of the eyelid margins by bands of tissue, are rarely reported in the literature. The association of both anomalies of the craniomaxillofacial region is an even more unusual finding.
OBJECTIVE: This article adds more information to the literature by reporting a case of a neonate with a rare association of bilateral synechia between the jaws, fusion of buccal mucosa to the ridges and between the tongue and palate, and ankyloblepharon filiforme adnatum in both eyes, without any other facial or systemic malformation.
METHODS: A literature review of similar cases published in PUBMED/MEDLINE was conducted followed by a discussion and comparison to the new case.
CONCLUSIONS: The rare association of these congenital defects, without the presence of syndromic symptoms, could be diagnosed and treated easily with good prognostic evolution.

OBJECTIVE: Children with ankyloglossia, an abnormally short, thickened, or tight lingual frenulum, may have restricted tongue mobility and sequelae, such as speech and feeding difficulties and social concerns. We systematically reviewed literature on feeding, speech, and social outcomes of treatments for infants and children with ankyloglossia.
METHODS: Medline, PsycINFO, Cumulative Index of Nursing and Allied Health Literature, and Embase were searched. Two reviewers independently assessed studies against predetermined inclusion/exclusion criteria. Two investigators independently extracted data on study populations, interventions, and outcomes and assessed study quality.
RESULTS: Two randomized controlled trials, 2 cohort studies, and 11 case series assessed the effects of frenotomy on feeding, speech, and social outcomes. Bottle feeding and social concerns, such as ability to use the tongue to eat ice cream and clean the mouth, improved more in treatment groups in comparative studies. Supplementary bottle feedings decreased over time in case series. Two cohort studies reported improvement in articulation and intelligibility with treatment. Other benefits were unclear. One randomized controlled trial reported improved articulation after Z-frenuloplasty compared with horizontal-to-vertical frenuloplasty. Numerous noncomparative studies reported speech benefits posttreatment; however, studies primarily discussed modalities, with outcomes including safety or feasibility, rather than speech. We included English-language studies, and few studies addressed longer-term speech, social, or feeding outcomes; nonsurgical approaches, such as complementary and alternative medicine; and outcomes beyond infancy, when speech or social concerns may arise.
CONCLUSIONS: Data are currently insufficient for assessing the effects of frenotomy on nonbreastfeeding outcomes that may be associated with ankyloglossia.