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We report a mother and daughter who have hypertelorism, mild limb defects, umbilical hernia/omphalocele, natal teeth and minor craniofacial anomalies. These individuals represent the third family with Teebi hypertelorism syndrome, a rare autosomal dominant syndrome.
A 20-day-old black male infant with two rare posterior natal teeth was referred for examination and care. Oral radiographs suggested that the teeth were the right and left maxillary primary molars. The hypermobile teeth and associated suspicious soft tissue masses were removed. Histologic examination revealed irregular tooth structures and infiltrate consistent with inflamed dental follicles.
Described are four oral anomalies--natal teeth, palatal cyst, bilateral lymphangiomas of the alveolar ridge, and median alveolar notch--found in a newborn. Immediate and long-term treatment considerations are outlined.
Natal teeth, defined as those that are the mouth at birth, are rare: one case in 2,000 to 3,500 births. In this case, that of a nine-month-old Japanese boy, it is reasonable to infer that the tooth-like structure, described as a hard-tissue conglomerate with a bone-like appearance, originated in the remnant of the dental papilla and Hertwig's sheath of the natal tooth. Both dentin and root formation were thereby induced.
Two male infants with a pseudo-hydrocephalic progeroid syndrome with natal teeth are compared with two very similar female cases reported in the literature and interpreted as congenital progeria. All these cases may represent a separate entity, a previously unrecognized genetic progeroid syndrome.
We report on two brothers, their mother, and 18 other relatives of five generations presenting an apparent newly recognized syndrome involving natal teeth, trichodystrophy, prominent interdigital folds, simian-like hands with transverse palmar creases, and ungual digital dystrophy, inherited as an autosomal dominant trait. In addition, the patients had hypoplasia of the first metacarpal and metatarsal bones and distal phalanges of the toes.
Oral examinations were performed of 1021 newborn Swedish children, of whom 101 were re-examined after 2-3 or 4-5 months. The most common findings, registered in 74.9% of the children, were of oral mucosal cysts situated either palatally or on the alveolar ridges. The majority of the palatal cysts disappeared shortly after birth, and some alveolar cysts appeared after birth. Ankyloglossia was found in 2.5% of the children, and Fordyce spots in 1.0%. No natal teeth were found. The upper labial frenum was attached to the crest of the alveolar ridge in 76.7% of the children, palatally in 16.7% and buccally in 6.7%. The relationship of the alveolar ridges was recorded: the anterior segment of the mandibular ridge was distal to the maxillary in 99% of cases, and, posteriorly, the mandibular ridges were lingual to the maxillary in 97.6%. An open bite was found in 39.8% of the children.
We studied 128 children who were transplacentally exposed to polychlorinated biphenyls and dibenzofurans in Taiwan, their parents and siblings who were directly exposed, and 115 control children. Direct exposure of the mothers stopped in 1979 and the children were born as late as 1985. At birth, exposed children had increased rates of hyperpigmentation, eyelid swelling and discharge, deformed nails, acne, natal teeth and swollen gums compared to controls. On examination, they had a much higher rate of dystrophic finger-nails and pigmented or dystrophic toe-nails than controls. They also had an increased rate of hyperpigmentation and acne. In addition they had more generalized itching, localized skin infections and hair loss. The findings seen in transplacentally exposed children differ from those seen in people directly exposed, particularly in the latter group in higher prevalence of acne.
This report concerned 2 cases (1-month and 8-day-old boy and 5-month and 27-day-old boy) of Riga-Fede's disease. The cases were treated with photopolymerized resin coverage of the incisal edges. Generally, the treatment methods for Riga-Fede's disease are discing and extraction of the traumatized teeth. The photopolymerized resin coverage proved to help in the rapid healing of the ulcerations. This method is useful because it is easy and is able to avoid a false pulp exposure of the hypoplastic natal and neonatal teeth by discing. 7 cases of natal and neonatal teeth were encountered at the Pedodontic Clinic of Niigata University Dental Hospital during 8 years from September 1979 to August 1987. 1) 7 cases were 3 boys and 4 girls, aging from 21 days after birth to 3 months 19 days. 2) Only one baby had a low birth weight. 3) The natal teeth accounted for 6 cases and the neonatal teeth accounted for 1 case. All were lower central primary incisors (5 on the right side, 1 on the left side and 1 bilateral). There were no supernumerary teeth. 4) 5 cases were of hypoplastic teeth. 5) Riga-Fede's disease was recognized in 3 cases. 6) The prognosis was 1 case of shedding, 2 cases extracted and 4 cases remaining. 7) Mesial inclination of the neighboring teeth and narrowness of the extracted space wee observed in the cases where extraction was performed at an early age. 8) Microradiogram and undemineralized specimens of the extracted tooth revealed that the tooth was very thin and mineralization was so poor and there was almost no root formation.
Infants born to mothers heavily exposed to polychlorinated biphenyls (PCBs) and dibenzofurans (PCDFs) have earlier been reported to have increased prevalences of natal and neonatal teeth. Some tendency toward higher prevalence figures of natal and neonatal teeth can be seen in the literature in normal child populations during the last 40 y. We therefore decided to determine the present prevalence of these teeth in a Finnish population and to evaluate whether infants with natal and neonatal teeth are more exposed to PCBs, PCDFs, and polychlorinated dibenzo-p-dioxins (PCDDs) than infants on average. A total of 34,457 infants born in 1997-2000 in four hospitals in southern Finland were examined for natal and neonatal teeth. The exposure of the infant to PCBs and PCDD/Fs was evaluated by measuring the levels of 17 most toxic PCDD/F and 36 PCB congeners in his or her mother's milk sample when the child was 4-8 wk old. A total of 34 infants had one or two natal (29 infants) or neonatal teeth (five infants). The milk analyses showed that the median level of PCDD/Fs as toxic equivalent (World Health Organization-recommended 2,3,7,8-tetrachlorodibenzo-p-dioxin equivalent quantity for PCDD/Fs in fat) was 11.9 pg/g in fat, and that of PCBs (World Health Organization-recommended 2,3,7,8-tetrachlorodibenzo-p-dioxin equivalent quantity for PCBs) was 7.24 pg/g in fat. These levels corresponded to the prevailing levels. The results showed that the prevalence of natal and neonatal teeth was 1:1000. No association was found between pollutant levels and occurrence of natal and neonatal teeth, indicating that the prevailing levels of PCDD/Fs and PCBs are likely to be below the threshold to cause perinatal eruption of teeth.
A natal tooth, the lower left central primary incisor, extracted from a twenty-eight-day-old boy was the subject of a clinical and ultrastructural (S.E.M., T.E.M., H.R.T.E.M.) study. The right one having been lost shortly after birth, the places of the natal teeth were taken by two hyperplastic structures which disappeared one year later, ejecting two little pearls of hard tissue. Scanning electron microscopic investigation of the extracted natal tooth showed a reduced enamel thickness corresponding to the development stage of the tooth as well as the absence of Hunter-Schreger bands and of an outer prism-free layer probably related with amelogenesis perturbations. Transmission electron microscopy disclosed characteristic ultrastructure of the enamel rods, the morphology of the enamel crystals evaluated by their width-to-thickness ratio taking logically place between fetal and adult enamel. A central dark line, which is thought to be in relation to the initial growth process, was observed in enamel crystals. Dentin aspects did not reveal significant perturbations compared to normal primary teeth.
Oral findings in 45 patients with Rubinstein-Taybi syndrome living in The Netherlands are compared with those from the literature. The main non-dental findings were thin upper lip, small oral opening, pouting lower lip, retro/micrognathia, and apparently higher arched, narrow palate. Cleft uvula, cleft palate, or, rarely, cleft upper lip can be part of the syndrome. Timing of the eruption of deciduous and permanent dentition is normal. Sixty-two percent of patients have malpositioned, crowded teeth. Marked caries was found in 36% and was possibly caused by problems in dental care due to the small opening of the mouth, malposition and malformation of the teeth, and non-cooperation of the patients. Hypodontia, hyperdontia, and natal teeth can be manifestations of the syndrome. In 73% of all patients and in 92% of all permanent dentitions, talon cusps were found. Two or more talon cusps are rarely found in the normal population or other syndromes. Therefore, this finding strongly supports the diagnosis of Rubinstein-Taybi syndrome in patients in whom this diagnosis is suspected.
The purpose of this report is to present two cases of natal and neonatal teeth including discussion on the various features concerning these conditions as well as the possible management strategies. The first case is a natal tooth in a newborn Saudi girl while the second case is two neonatal teeth in a Saudi boy.
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A four generation Scottish family with hidrotic ectodermal dysplasia affecting predominantly teeth, skin and hair is described. Hypo- or oligodontia of the secondary dentition by late adolescence was characteristic and two individuals had multiple natal teeth. Flexural acanthosis nigricans during childhood and early adolescence is a feature in some of the women. All affected individuals produced sweat, but heat tolerance was variable. Hypoplasia of the pilosebaceous units was found on light microscopy in one subject. Scalp hair was thin and slow growing (but adult females described much improved quality during pregnancy) and body hair was scanty. Scanning electron microscopy of hair samples showed abnormal cuticular appearances consistent with a primary defect affecting keratin structure. The nails were normal. Relative macrocephaly due to hyperostosis of the cranial vault was variably present. Short stature (5-10th centile) present in some cases is possibly a separate familial trait. The family demonstrates overlapping features with Clouston syndrome. In Clouston syndrome, however, alopecia can be severe, palmarplantar hyperkeratosis is usually present, and hypo/oligodontia is not a prominent feature.