Neonatal Teeth Research Articles

Neonatal Riga-Fede disease: A systematic review of 84 cases collected over 120 years

Background: Traumatic ulceration of the tongue due to congenital teeth is known as Riga-Fede disease (RFD). The literature on RFD consists largely of anecdotal case reports, thereby lacking valid clinical recommendations. While managing a newborn with RFD complicated by Ludwig’s angina, this knowledge gap was recognized. Therefore, this systematic review was conducted to gain insight into the optimal management and the possible complications of RFD. Methods: A literature search was conducted using PubMed, Google Scholar, and Science Direct, supplemented with African Journals Online, Indmed, and manual searches. Papers published between 1900 and July 2023 in any language were retrieved. Neonates with natal or neonatal teeth and ulceration of the oral mucosa were included. Papers with incomplete data and review articles were excluded. Results: A total of 84 newborns with RFD from 62 published reports (including the case reported herein) were analyzed. Most of the reports were from the Indian subcontinent, and the frequency of reporting has increased since the year 2000. Tongue ulceration in all cases was due to mandibular incisors. There was no significant difference in the median duration of healing between the tooth extraction group (14 days) and the conservative management group (18 days). There was only a very weak positive correlation between the duration of symptoms and healing time, which was statistically insignificant. Conclusion: Healing of mucosal ulcers in RFD is often protracted and does not appear to be influenced by the duration of symptoms or by the modality of treatment. Extraction of the offending teeth is more common (75% of cases) than conservative management, such as grinding (ameloplasty) or resin capping of teeth. RFD is mostly a benign disorder; however, serious complications such as meningitis, Ludwig’s angina, and soft tissue abscesses can rarely occur with Staphylococcus aureus infections.

Case Report: A rare case of bilateral molar natal teeth in a term newborn

Reports of natal and neonatal teeth have been well documented in the literature, though the presence of natal primary molars remains rare, and a clear management strategy does not exist. This is a case study of a female newborn delivered at a gestational age of 41 weeks 1 day to a 31-year-old G1P1001 mother. Apgar scores were 8 and 9 at 1 and 5 min, respectively. The infant was delivered by cesarean section and was admitted to the Mass General Hospital Newborn Nursery, where she received routine care. The patient had two posterior molars, which warranted consultation for oral maxillofacial surgery. Due to the gross mobility of the natal teeth and the risk of aspiration in a small breastfeeding newborn, the decision was made to extract the natal teeth immediately. Understanding the management of natal teeth is important for the pediatrician.

Management of natal tooth of a 2 months old infant: a case report

Natal and neonatal teeth are the type of supernumerary tooth which have various myths and superstitions associated with it. Natal teeth are the teeth which appear at the time of the birth whereas neonatal teeth appear within one month of life. Presence of natal teeth often risks the aspiration of teeth by infant and the mother also has difficulty in breast feeding and comes with the complaint of the same. The present case report of 2-month-old infant describes the management of natal teeth present in mandibular arch with mobility. After the consent of parents, the extraction of the shell-like tooth was done immediately and hemostasis was achieved. The follow up was carried out after 1 week to examine the normal healing of extraction site.

A rare case report of natal tooth, neonatal tooth (dentitia praecox) and fibrous hyperplasia (Riga fede disease) of same newborn patient

The natal tooth is present at the time of birth and neonatal tooth erupts within first 30 days of birth. Both are considered as an anomaly of a child. Due to mechanical irritation of those teeth there may be a chance of traumatic ulceration on the ventral surface of teeth known as Riga Fede disease (RFD). The term is used for the lesion in case of child less than two years old. The age above two years the term coined as traumatic granuloma. It may lead to a serious condition where the child denies to take any food or to suck breast milk due to the painful condition. In the present case study 21 days old newborn came to the clinic with a complaint of ulcerative lesion on the ventral surface of the tongue which was interfering in suckling. On examination 8 X5 mm ulcer extending from anterior border of the tongue to lingual frenum was found along with two mandibular incisors. He was detected with natal tooth (at the time of birth) and neonatal tooth (erupted on 16th day) which caused RFD.The case report throws some light on a possible treatment approach for successful management of the condition.

Management of natal teeth: A twin case report

Child’s evolution and growth right from conception up to the first couple of years of life is dotted with several transitions. Tooth eruption is one such phenomena which follows a chronology corresponding to the age of the child at which the tooth appears in the mouth. The exfoliation timeline has been documented in the literature but may vary based on several factors including hormonal, hereditary, and environmental. However, sometimes the eruption sequence undergoes a more drastic alteration where the first teeth may be present in the mouth at the time of birth or erupt within the first thirty days after birth. This paper was written with the aim to discuss the etiologic factors, clinical features, complications, and management of such natal or neonatal teeth, as well as their role in sublingual ulcer formation and discomfort during breastfeeding. Two cases are discussed where newborns, a 10-day old male, and a 7-day old female with a mandibular incisor natal tooth had reported to the Department of Pedodontics and Preventive Dentistry at Kalinga Institute of Dental Sciences. The teeth were extracted as they were mobile and there was fear of aspiration and refusal to feed. Extraction of the natal tooth accelerated healing of ulcers and relief from discomfort during breastfeeding.

Riga-Fede Syndrome : A Rare Case Report With Literature Review

Riga-Fede syndrome is characterised as an ulcerative lesion that originates on the ventral surface of a neonate’s tongue due to vigorous back and forth movements of the tongue over the precociously erupted mandibular anterior teeth. This condition has been receiving discernible attention as a progressing condition with escalating case reports over the years. The aetiology of this issue is not precisely deduced; however, there have been multiple treatment options discovered that have secured success with higher precision. The purpose of this research paper is to exhibit various case reports and aetiologies responsible for this disease, as well as an explanation of different treatment plans depending on the status of the tooth, that is, the mobility of the tooth and the precarious chances of aspiration. Moreover, radiographic study to visualise if it is a supernumerary or a primary regular tooth and if it is associated with hindrance in breast-feeding causing soft tissue injuries and the state of the child’s health. A 28-day-old female infant was presented to the oral pathology clinic with a natal tooth exhibiting grade 2 mobility along with a lesion on the ventral surface of the tongue, leading to the clinical diagnosis of Riga-Fede disease. The treatment performed, consisting of the extraction of natal teeth, was selected as the treatment of choice over more conservative treatments for the rapid resolution of the lesion and the limited risk of inadequate nutrient intake since the tooth was interfering with the feeding process. Following the extraction of the tooth, it underwent microscopic studies to identify diverse changes associated with the erupted tooth. There was regular monitoring of the patient, and complete wound healing was observed after 4 weeks. The proposed treatment was successful, and the patient is still in follow-up without recurrence of the lesion after one year. This paper presents a concise review of the literature about neonatal teeth and their role in Riga-Fede disease.

Riga-Fede disease: report of a case in an infant

Riga-Fede disease (RFD) is a rare, benign condition found in infants, characterized by ulcers in the oral mucosa, around the tongue and gums. It is caused by repeated injuries, caused by movements of the tongue over the lower incisors. It usually coexists with neonatal teeth. Although it starts as a small ulcer, it can progress to a large fibrous lesion. Symptoms include pain, swelling and difficulty swallowing and sucking. Early diagnosis and treatment are important to prevent late complications, including insufficient food intake, growth retardation and deformity of the tongue, gums, lips and floor of the mouth. The diagnosis is usually made on the basis of medical history and clinical features. The following paper presents the case of a 9-month-old boy with a lump occurring in the fundus of the mouth, at the base of the tongue.

Management of Recurrence of Early Infancy Tooth Following Natal Teeth Extraction: An Unusual Case Report

Natal tooth are the teeth which can be present at birth whereas neonatal tooth appears within 30 days after birth which is an unusual and rare situation. The exact etiology behind this is unknown and various factors may be related to these teeth. Both natal and neonatal teeth are notably important for pediatric dentists and pediatricians. This case report describes the management of 24 days old infant with a mobile mandibular natal tooth related to Rigafede disease. After 45 days, there's a recurrence of early infancy tooth followed by extraction of natal tooth.

Riga-Fede disease: a review of the literature and a description of clinical cases

Relevance. Riga-Fede disease (RFD) is a rare pathology that develops as a result of trauma to the mucous membrane of the tongue ventral surface with sharp edges of natal, neonatal or primary teeth erupting in time and manifests in the form of erosion or sublingual fibrous lesion with an ulcerated surface.Purpose. To analyze the literature and present our own RFD clinical cases.Material and methods. A search was performed of articles published in 2001-2023 on the research topic in the main scientific literature databases (PubMed, E-library, Google Scholar, etc.). We analyzed the data from 68 publications and presented three of our own clinical cases of RFD in children aged 5, 6 and 9 months.Results. Analysis of literature data revealed different approaches to the diagnosis and treatment of RFD in children. The diagnosis of RFD should rely on the clinical picture and exclude additional trauma to children by histological examination and lesion excision. Pronounced tooth mobility, ineffective infant feeding and the presence of neurological diseases in patients were the main indications for the extraction of teeth, which caused RFD development. In the absence of tooth mobility, non-surgical treatment methods should be preferable. In the presented clinical cases, erupted primary teeth caused BRF. In two cases, the treatment consisted of smoothing the sharp edges of the teeth and restoration with glass ionomer cement; in one case, a child with a neurological disease experienced extraction of lower central incisors. In all cases, recovery was within 3-4 weeks.Conclusion. Once clinically diagnosed with RFD, treatment method choice should consider tooth mobility and the child's overall health.

Non syndromic hypodontia with retained natal teeth-a rare case report

Teeth that erupt prematurely have been designated with various terms such as natal teeth, neonatal teeth, congenital teeth, pre-deciduous teeth and dentitio praecox. Tooth agenesis is one of very common finding in a person. Developmental or congenital absence of one or more teeth excluding the third molars is a highly prevalent condition referred to as hypodontia. The incidence of hypodontia usually varies from 1.6-6.9% while the incidence of natal teeth on the other hand is noted to be approximately 1:2,000-1:3,000. Lower primary central incisors are the most affected tooth. The occurrence of retained natal teeth and that too with hypodontia is not found in the literature. This case report presents a rare occurrence of hypodontia with presence of retained natal teeth in a 14-year-old male child. Diagnosis made as non-syndromic hypodontia and retained natal teeth.

Research on natal and neonatal teeth in Africa: A systematic scoping review of empirical evidence.

In Africa, natal and neonatal teeth is a culture-bound phenomenon which is associated with several sociocultural connotations which might affect child survival. Different empirical studies have been conducted in Africa on natal and neonatal teeth; however, no known scoping review has been conducted to map the empirical evidence. This systematic scoping review (SSR) aims to document the available empirical evidence, research gaps, and hotspots on neonatal and natal teeth in Africa. The methodology of this SSR was informed by the Joanna Brigg's Institute guidelines for SSRs, and it was reported in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews. Eleven research databases were systematically searched to scooped out all literatures relevant to the scoping review question, after which they were screened for eligibility based on the review's selection criteria. Only the eligible literatures were included in the review. Data were extracted from the included literatures, after which the extracted data were collated, summarized, and presented as results. This review included only three journal articles. All the reviewed articles revealed knowledge gaps about natal/neonatal teeth. These articles also found misconceptions around natal/neonatal teeth; for example, nurses'and traditional birth attendants'beliefs about these teeth are contrary to scientifically known facts. Multiple myths associated with natal/neonatal teeth, as indicated in the reviewed studies, point to a poor understanding of the condition. Neonates and infants are highly vulnerable persons; they require care and safeguarding from dental myths that could threaten their survival. Educative information concerning natal/neonatal teeth should be included in public health education programs to address the observed knowledge gaps among African populations and correct wrong beliefs on neonatal and natal teeth.

Response to Tofacitinib in an Infant with Aicardi-Goutières Syndrome due to Biallelic Variants in RNU7–1

Aicardi-Goutières syndrome (AGS) is a severe, early-onset neuroinflammatory disease characterized by leukoencephalopathy resulting in developmental delays and neurologic regression. Patients can have extracranial findings including hematologic, cutaneous, and other organ manifestations. AGS is a recognized type I interferonopathy, with evidence of interferon activation in both the central nervous system and blood, leading to attempts to modulate disease using JAnus Kinase (JAK) inhibition.We present a now 1-year-old female who had suspected AGS despite nondiagnostic clinical genetic testing at birth, done for prematurity, failure to thrive requiring enteral tube feeding, leukodystrophy, hypotonia, seizures, hearing loss, and developmental delay. She also has dysmorphisms, including neonatal teeth, rib abnormalities and microcephaly. She was sequentially diagnosed with hepatitis (biopsy findings of mild macrovesicular steatosis, mild interface hepatitis and mildly increased fibrosis), hypertrophic cardiomyopathy, hyponatremia attributed, in part, to adrenal insufficiency, and chronic lung disease from microaspirations. Evaluation at 11 months revealed unremarkable immunoglobulin levels, vaccine titers, immunophenotyping, and proliferation to mitogens and Candida but not tetanus (despite protective titers). An elevated 6-gene interferon score (19.5) was detected in the blood.Given the devastating natural history of this syndrome, she was started on the JAK inhibitor tofacitinib age 8 months. After starting tofacitinib, she had notably decreased clinical symptoms, improved tone and milestones. Her transaminitis resolved. A repeat interferon score after 4 months of treatment showed improvement (15.9). She has tolerated common viral infections on immune suppression.Under the Undiagnosed Diseases Network, clinical trio genome sequencing was re-analyzed revealing compound heterozygous variants in the non-coding RNA RNU7–1, a newly identified cause of previously genetically unexplained AGS (AGS9). This results in impairment of U7 snRNP function leading to misprocessing of pre-mRNA and driving an interferon response. The mortality of AGS9 is higher than other AGS genotypes, with cardiac and liver involvement being more common.Revelation of RNU7–1 mutations in this case underscores that diagnostic workup for genetically unexplained AGS should include targeted evaluation of RNU7–1. The impact of early initiation of JAK inhibition in patients with AGS on long-term outcomes remains to be determined, but tofacitinib, uniquely available in liquid formulation, is one option.

FGF4 and FGF9 have synergistic effects on odontoblast differentiation.

The purpose of this study was to investigate whether fibroblast growth factor 4 (FGF4) and FGF9 are active in dentin differentiation. Dentin matrix protein 1 (Dmp1) -2A-Cre transgenic mice, which express the Cre-recombinase in Dmp1-expressing cells, were crossed with CAG-tdTomato mice as reporter mouse. The cell proliferation and tdTomato expressions were observed. The mesenchymal cell separated from neonatal molar tooth germ were cultured with or without FGF4, FGF9, and with or without their inhibitors ferulic acid and infigratinib (BGJ398) for 21days. Their phenotypes were evaluated by cell count, flow cytometry, and real-time PCR. Immunohistochemistry for FGFR1, 2, and 3 expression and the expression of DMP1 were performed. FGF4 treatment of mesenchymal cells obtained promoted the expression of all odontoblast markers. FGF9 failed to enhance dentin sialophosphoprotein (Dspp) expression levels. Runt-related transcription factor 2 (Runx2) was upregulated until day 14 but was downregulated on day 21. Compared to Dmp1-negative cells, Dmp1-positive cells expressed higher levels of all odontoblast markers, except for Runx2. Simultaneous treatment with FGF4 and FGF9 had a synergistic effect on odontoblast differentiation, suggesting that they may play a role in odontoblast maturation.

A Combined Case of Neonatal Teeth, Bohn’s Nodules and Eruption Haematoma in an Infant Presenting as a Diagnostic Dilemma

Abnormalities in the oral cavity of infants are not uncommon, but most are innocuous and resolve with age in the absence of treatment. Some of the most prevalent oral findings are oral inclusion cysts, vascular lesions, natal and neonatal teeth. The American Academy of Paediatric Dentistry (AAPD) recognises that perinatal and infant oral health are the basics upon which preventive education and dental care must be built to enhance the opportunity for a child to have a lifetime free from preventable oral disease This report describes the clinical presentation, diagnosis and suggestive management for a 32-day-old male child patient reported with neonatal teeth, Bohn’s nodules and further at the age of 18 months, with eruption haematoma in the maxillary alveolar region. “Bohn’s nodules” are keratin-filled cysts with prevalence of 47.4% with no gender predilection. Natal and neonatal teeth considered as critically important conditions as their presence can lead to numerous complications such as ulceration on the ventral surface of the tongue caused by the sharp incisal edge of the tooth and condition known as Riga-Fede disease or syndrome. “Eruption haematoma” is circumcoronal cystic cavity which contains blood usually associated with an erupting primary or permanent tooth which is in its soft tissue eruption phase. Therefore, a thorough clinical examination and knowledge of the various lesions is essential for precise diagnosis, management, as well as parental counselling.

Eruption Cyst of a Newborn and Review of the Management

Eruption cyst is a cyst associated with the unerupted tooth that is rarely found in a newborn or infant. We reported a case of a male infant who presented with an eruption cyst at birth in which has not caused any feeding or airway problem in him. A periodic monitoring was performed and spontaneous regression of the cystic lesion was observed within a month with the presence of a neonatal tooth .

A Rare Case of Natal Teeth

The presence of natal teeth (at birth) or neonatal teeth (within a month of delivery) is a rare condition. This report discusses a newborn female, at birth, with one mandibular incisor tooth. The teeth were mobile and were extracted because of the fear of aspiration and difficulty in feeding. The purpose of this report is to review the literature related to natal teeth and discuss their possible etiology and treatment. Keywords: natal teeth, aspiration

MANAGEMENT OF A RARE CASE OF AN INFANT WITH THREE NATAL TEETH.

Primary teeth normally start to sprout at 6 months of age. However, the eruption of teeth at or shortly after birth is rather rare. Neonatal teeth are those that erupt within the rst 30 days of life and are referred to as "natal" teeth if they are present at birth. Although the size and shape of these natal teeth may approximate that of the primary dentition, they frequently have hypoplastic enamel and dentin along with weak or nonexistent roots. They are also frequently smaller, conical, and yellowish. The baby may experience discomfort, including pain and difculties sucking, sublingual ulcers, lacerated breasts, and even the ability to aspirate teeth. In this situation, extraction is required. Additionally, no treatment is necessary if the tooth is generally asymptomatic and does not obstruct regular breastfeeding. Since there are negative cultural attitudes associated with natal teeth, good parental counselling and vigilant management in relation to child protection are required. As a result, both general practice dentists and pediatric dental specialists may be involved in the supervision or treatment of patients with natal and neonatal teeth. This report describes a rare instance in which a one-month-old baby appeared with three natal teeth that had to be removed to prevent aspiration risk and feeding disruption.

Natal and neonatal teeth a case series - from south India

Teeth eruption into the oral cavity follows a chronology. Small variations depending on endocrine, genetic and environmental factors can alter the chronology of teeth eruption. The occurrence of teeth at birth is called natal teeth and when teeth arise within 30 days of birth it is called neonatal teeth. The occurrence of natal and neonatal teeth is an uncommon anomaly. It is associated with a lot of superstition in various ethnic group. If the natal and neonatal teeth do not interfere with feeding nor are they causing any ulceration they can be left in place. Some natal and neonatal teeth cause injury to the mother on feeding and can cause tongue ulceration in the neonate. In such cases the teeth can be removed. If the natal and neonatal teeth are extremely mobile such that reattachment in unlikely and there is a danger of aspiration of the tooth, then they can be removed. We describe in this report 4 neonates with natal and neonatal teeth.

Riga Fede disease associated with a neonatal tooth

Riga Fede disease is defined as traumatic ulcerations on the ventral surface of the tongue in infants and neonates and most commonly occurs due to natal, neonatal teeth or erupting deciduous mandibular central incisors. The occurrence of natal or neonatal teeth is also a rare occurrence. This article represents a reported case of Riga Fede disease associated with a neonatal tooth in a 10 weeks old patient and its non-invasive management.

Inborn or acquired changes in children under three years of age – a review of the literature

This study aimed to discuss some inborn and acquired changes in the oral cavity in young children under three years of age. Based on current literature, eruption cyst, Riga-Fede disease, natal and neonatal teeth, congenital epulis, Epstein pearls, Bohn’s nodules, and gingival cyst of the newborns have been described. The symptoms, clinical presentation, and histological features of these changes, their epidemiology, prevalence, etiopathogenesis, differential diagnosis, and management were taken into consideration to present them accurately. Described oral normal findings and anomalies are not very common, so they require precise diagnosis and management properly. Having relevant knowledge on issues is important both for the pediatricians and pediatric dentists who always cooperate to distinguish the pathology or anomaly and start dealing with it. To our knowledge, this is the first literature review presenting comprehensive and thorough specification of abnormalities occurring in children’s mouth, basing on the most recent scientific literature between 1999 and 2022.