• The Journal of Korean Academy of Prosthodontics
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• v.29 no.1
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• pp.249-254
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• 1991

Two male patients of hereditary ectodermal dysplasia with oligodontia aged 12years and 10years 6months were recalled after 6 years and treated by reconstructing overdentures according to the growth. Patients were improved functionally and cosmetically. Periodic recall is reguired and denture should be reconstructed according ato their growth.

• The Journal of the Korean dental association
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• v.22 no.4 s.179
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• pp.329-332
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• 1984

The author observed a patient of ectodermal dysplasia with oligodontia. The treatments were done as follows: Vital pulpotomy and restorative treatment were done as usual method. Removable type space maintainer was set on the area of primary teeth removed. Follow-up checking were required for the observation of growth and development of the permanent teeth and the jaw.

• Journal of the korean academy of Pediatric Dentistry
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• v.50 no.4
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• pp.483-494
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• 2023

This report presents two cases concerning strategies for improving denture retention in pediatric patients with oligodontia caused by hypohidrotic ectodermal dysplasia (HED). Both patients presented with multiple missing teeth, conical canines, alveolar bone atrophy, and a skeletal Class III tendency. In the first case, a modified form of conical-crown-retained denture was used to cover the canines. This approach was carried out without tooth extraction or coping. In the second case of severe alveolar bone resorption and a distally tilted lower left canine, the tooth was restored with a hybrid ceramic crown. It was subsequently converted into a clasp-retained removable partial denture, utilizing a suction mechanism. Both patients are currently receiving regular check-ups for the maintenance of their prosthetic appliances and the evaluation of their growth patterns. This study presents innovative prosthetic treatment methods for pediatric patients with HED who have inadequate denture retention.

• 대한치과보철학회:학술대회논문집
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• 1999.04a
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• pp.85.2-86
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• 1999

• The Journal of the Korean dental association
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• v.36 no.4 s.347
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• pp.246-248
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• 1998

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.2
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• pp.180-187
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• 2015

Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.

• Imaging Science in Dentistry
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• v.41 no.4
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• pp.167-170
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• 2011

Ellis-van Creveld syndrome is a rare congenital genetic disorder having autosomal recessive inheritance. It is a syndrome affecting the Amish population of Pennsylvania in USA with prevalence rate of 1/5,000 live at birth. In non-Amish population, the birth prevalence is 7/1,000,000. The syndrome is characterized by bilateral postaxial polydactyly of the hands, chondrodysplasia of long bones resulting in acromesomelic dwarfism, ectodermal dysplasia affecting nails as well as teeth and congenital heart malformation. There were very rare reports of this syndrome in dentistry. The present case focuses on the striking and constant oral findings of these patients, which are the main diagnostic features of this syndrome. Since the oral manifestations affect the esthetic, speech, and jaw growth of the child, the dentists have an important role to play in proper management of such case.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.3
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• pp.270-274
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• 2015

Although there are a number of studies on the treatment planning and the prognosis of dental implants, limited studies provide the information on the implant placement on growing adolescents. The aim of this review is to discuss the use of dental implants in growing patients and the impact of skeletal and dentoalveolar growth on the long-term stability of implants. The general information regarding skeletal growth of maxilla and mandible would be briefly reviewed and the general treatment options would be discussed.

• Neonatal Medicine
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• v.28 no.1
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• pp.48-52
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• 2021

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by skin defects involving the epidermis, dermis, subcutaneous tissue, bone, and sometimes dura. It commonly affects the scalp in approximately 70% of cases, but the neck, trunk, and the extremities can also be affected. ACC can occur either as an isolated condition or associated with other anomalies and congenital syndromes, and it can be acquired either genetically or sporadically. Morbidity and mortality are associated with the defects of skull bone, dura, and other multiple anomalies. We herein report the case of a female infant, with a large scalp defect accompanied by a skull defect noted at birth, who developed mental retardation in the preschool years.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.4
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• pp.636-643
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• 1999

Ectodermal dysplasia is a genetic birth defect in which at least abnormally develop two structures derived from the ectoderm. It is usually inherited in autosomal dominant or autosomal recessive pattern. Oral manifestations are oligodontia, anodontia, dysmorphic teeth(conical shape), decreased occlusal vertical dimension and alveolar bone. Extraoral signs may include decreased or absent sweat glands, sparse and fine hair, saddle nose, hearing loss and decreased production of body fluids including saliva. Most affected children require extensive dental treatment to restore their appearance and help the development of a positive self image. The patient's overclosed profile was due to a decreased vertical dimension. The use of overdenture is to preserve erupted teeth, to accomodate the newly constructed occlusal plane, to improve retention and stability of denture and to maintain the remaining alveolar bone. The restoration of vertical dimension improved the child's speech, swallowing, and eating. Growth continue until the age of approximately 18. As child grows, replacement dentures will have to be fabricated primarily to accomodate increasing vertical dimension and changing dentition. Implants may be indicated later if the alveolar bone is adequate. Periodic recall visits are advised, to monitor the dentures during periods of growth and development, and eruption of the permanent teeth.