• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.601-608
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• 1996

Robinow syndrome or fetal face syndrome is a rare inherited disorder characterized by short stature, mesomelic brachymelia, hypoplastic genitalia, and a typical facial appearence ("fetal face") with frontal bossing, hypertelorism, ear abnormalities, a short upturned nose, long philtrum, micrognathia, and macrocephaly. Intraoral features have included quite a few dental cavities, crowding, hypoplastic uvula, cleft lip or/and cleft palate, gingival hyperplasia, alveolar hyperplasia, enamel hypoplasia, delayed eruption, and congenital missing of the permanent teeth. We report on a 10 years old girl with Robinow syndrome. The patient had most of the typical anomalies of the syndrome and negative family history but, in addition, had mental retardation, hearing loss, and serous otitis media. Intraoral findings included dental cavities, crowding, hypoplastic uvula, repaired cleft palate, and mouth breathing. Dental treatment and V-tube insertion(by dept. of ENT) were performed under general anesthesia. In all cases of Robinow syndrome, thorough evaluation and united treatments with medical specialists should be performed.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.1
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• pp.124-129
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• 2010

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine growth retardation and post-natally by dwarfism, severe microcephaly, bird-headed profile with receding chin, prominent nose, joint defects, clubfoot, sparse hair, malformation of genitourinary tract and rectum, mental retardation and hematological disorders. There is also a reduction in the number of blood cells. Dental anomalies of Seckel syndrome are crowded teeth with malocclusion, enamel hypoplasia, absence of some teeth and taurodontism. This report described the oral and maxillofacial manifestations of children associated with Seckel syndrome. Children with Seckel syndrome have several dental and skeletal irregularities. The purpose of this study was to report the dental and medical characteristics of the patient and review the literatures of Seckel syndrome.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.49 no.1
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• pp.21-29
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• 2023

Objectives: Dental or maxillofacial emergencies are uncommon during pregnancy, but if they occur, they are challenging to treat due to potential risks. The mother should not be denied necessary medical or dental care because of pregnancy. The aim of the study is to observe outcomes of pregnancy in patients requiring emergency minor oral surgical procedures during gestation and to determine the safety of the pregnant woman undergoing the procedure and the fetus. Materials and Methods: The study was conducted on 52 pregnant women requiring emergency oral surgical procedures. A standard treatment protocol for treatment of specific entities was followed. Close monitoring and observation were the primary goal of treatment. All patients were followed postoperatively until complete recovery from the surgical procedures and then until birth of the baby. A control group of 52 healthy pregnant patients who did not require oral surgical procedures was considered for statistical analysis. The measurements to calculate observation were fetal loss (spontaneous abortion), preterm birth, low-birth weight, or incidence of any congenital anomalies in the baby and its association with surgical procedures. Results: No fetal loss occurred in any of the cases. However, four patients experienced preterm birth and seven neonates exhibited low birth weights. No congenital abnormalities were discovered. In one instance, a patient who underwent surgery for a mandibular symphysis fracture under general anesthesia in the 31st week of pregnancy experienced labor pain on the fourth postoperative day, requiring an emergency Caesarean section. Conclusion: The results of our study demonstrate that, compared to the control group, minor emergency surgeries performed during pregnancy have no discernible negative effects on the fetus. These procedures can safely be performed by adhering to our described protocols.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.118-121
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• 2012

Down syndrome (DS) is a genetic disease known as trisomy 21. Congenital cardiac anomalies are present in about 40% of DS patients. Dental anomalies are also common among DS patients. In DS patients, canine impaction is 10 times more frequent and transposition of maxillary canine and first premolar is 50 times more common than in general population. A female DS patient with congenital heart disease was diagnosed as having a transposed impacted maxillary canine. Sectional fixed appliance with Nance holding arch was used for the orthodontic treatment. After space was regained for the eruption of the canine, orthodontic button was attached using flap operation with closed technique. Traction and alignment of the tooth followed. To prevent endocarditis, prophylactic antibiotics were prescribed for the recommended dental procedures. Total treatment time was 25 months and no complication was found.

• Proceedings of the KACD Conference
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• 2001.05a
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• pp.247-251
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• 2001

;A dental developmental anomaly is defined as an isolated aberration in tooth form, caused by a disturbance or abnormality which occurred during tooth development. There are numerous types of dental anomalies, and a considerable variation in the extent of the defects occurs with each type. Teeth with these anomalies pose unique challenges. Since the defects are not always apparent clinically, they can confuse diagnosticians investigating the etiology of pulpal pathosis. When endodontic treatment is required, the defects often hinder access cavity preparation and canal instrumentation. Treatment planning also becomes more challenging, since the defects can create complicated periodontal problems, and the malformed teeth can be difficult to restore, particularly those weakened by endodontic therapy. Fusion is defined as the joining of two developing tooth germs resulting in a single large tooth structure. The incidence of fusion is < 1% in the Caucasian population, and it is believed that physical force or pressure produces contact of the developing teeth. Clinically and radiographically, a fused tooth usually appears as one large crown with at least partially separated roots and root canals. There may be a vertical groove in the tooth crown delineating the originally separate crowns. Dens invaginatus is a deep surface invagination of the crown or root that is lined by enamel. Teeth in both maxillary and mandibular arches may be affected, but the permanent maxillary lateral incisor is the tooth most commonly involved. Studies have revealed an incidence ranging from 0.25% to as high as 10%. The invagination ranges from a slight pitting to an anomaly occupying most of the crown and root. The invagination frequently communicates with the oral cavity, allowing the entry of irritants and microorganism either directly into pulpal tissues or into an area that is deparated from pulpal tissues by only a thin layer of enamel and dentin. This continuous ingress of irritants and the subsequent inflammation usually lead to necrosis of the adjacent pulp tissue and then to periapical or periodontal abscesses. If the invagination extends from the crown to the periradicular tissue and has no communication with the root canal system, the pulp may remain vital. Recommended treatment of fused tooth and dens invaginatus has been reported in the endodontic literature. This case report describes the endodontic treatment of a maxillary laterl incisors having fused crown and dens invaginatus.natus.

• Korean Journal of Cleft Lip And Palate
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• v.7 no.1
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• pp.1-16
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• 2004

In the recent time, early correction of the congenital anomalies has become the focus of contemporary cleft lip care, The reason of that is mostly psychologic factor of children are respected, Although the propound object of lip adhesion is not esthetic improvement, that able to satisfy sufficiently parents of cleft children, In the unilateral or bilateral clefts, a preliminary lip adhesion has been advocated as a mean of narrowing the cleft at an early phase, of improving the nasal contour, of molding the alveolar arch, and of easing and improving the result of a definitive lip repair. The present study was earned out to investigate the effect of lip adhesion. We performed the Millard's high-half underminded adhesion and Seibert's lip adhesion followed by modified Millard's cheiloplasty for five infants had unilateral complete cleft lip. The lip adhesion reduced the actual deformity by molding the maxillary alveolar segments into better relationship and allows a easy cheiloplasty so that led to more perfect final lip result, Both Millard's high-half underminded adhesion and Seibert's lip adhesion were available methods to adhere a wide cleft lip, Especially, Seibert's lip adhesion had more advantages such as enhancement of the force of adhesion, correction of the deviated columella and acquirement of the esthetic upper lip continuity. These results suggest that the lip adhesion followed by cheiloplasty for wide unilateral complete cleft lip patients provide more favorable final result by molding the maxillary alveolar segments into better relationship.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.2
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• pp.136-143
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• 2004

The purpose of this study was to investigate the relationship between morphology and position of deciduous double teeth, and the occurrence of other dental anomalies in the same subject. Four morphological types were indentified according to Ailing's classification: type I, bifid crown-single root; type II, large crown-large root; type III, two fused crowns-single root; type IV, two fused crowns-two fused roots. Fifty-four double teeth were found in a total of 1,803 children, who had visited Wonkwang university hospital for dental treatment from January 1, 2003 to September 30, 2003. All of these children were examined clinically and intra-oral radiographs were taken. The results were as follows; 1. 49 children(2.7%) had more than one double teeth, 5 of these children had two double teeth on the bilateral side. And one child showed triple teeth which has three crowns and three roots. 2. Double teeth were predominantly situated in the anterior region, with a preference for the mandible. The ratio of cases involving central incisor and lateral incisor was higher than other cases. 3. There were 25 cases(46.2%) of missing successors among 54 cases of the double teeth. And, prevalence of the missing teeth was highest in the cases involving maxillary central incisor and lateral incisor. 4. In the case of type II(large crown-large root) had more missing successors.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.492-498
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• 1999

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.1
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• pp.34-39
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• 2014

Nevoid basal cell carcinoma syndrome(NBCCS) is a autosomal dominant disorder, and its major manifestations are multiple basal cell carcinoma, keratocystic odontogenic tumor, rib anomalies, palmer and plantar pits, calcification of the falx cerebri. Keratocystic odontogenic tumor(KCOT) is defined as intraosseous tumor of odontogenic origin with a characteristic lining of parakeratinized stratified squamous epithelium and potential aggressive behavior. We report a case of a 3-year-old patient with nevoid basal cell carcinoma syndrome who initially presented with unilocular keratocystic odontogenic tumor in maxillary canine region. Keratocystic odontogenic tumor was treated by enucleation, and periodic follow-up check will be required for early diagnosis of additional diseases related with this syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.4
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• pp.667-672
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• 2003

Rieger syndrome is a rare, autosomal dominant genetic disorder characterized by malformation of the anterior chamber of the eye(goniodysgenesis) coincident with hypodontia. It may also be accompanied by a spectrum of dental, craniofacial and somatic anomalies. Mutations in paired-like homeodomain transcription factor2(PITX2) are associated with the syndrome, and its frequency in the general population has been estimated to be 1 : 200,000. In the present case, the patient, 4 year 7 month-old female, had posterior embryotoxon and polycoria. The maxilla was retrusive in cephalometric radiography. She had congenital missing on #52, #62 and some tooth germs of permanent tooth were not detected in panoramic radiography. The purpose of this paper is to report the dental and craniofacial findings and review the pertinent literature through this case.