• Archives of Plastic Surgery
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• v.37 no.4
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• pp.481-484
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• 2010

Purpose: Congenital dermoid cysts develop during the fusion of the embryo when the ectodermal tissue gets trapped in the line of fusion. Dermoid cysts of the head are rare lesions comprised of epidermal and mesodermal elements. Furthermore, dermoid cysts in the occipital area are extremely rare. Only a few cases of dermoid cysts in the posterior scalp have been reported. Especially, A bilateral, synchronous presentation in this location has not been reported previously in the literature. Methods: All 5 cases had a gradually enlarging mass of the posterior aspect of the scalp. The cysts were mobile, noncompressible, and non-tender, without evidence of an associated sinus tract, skin dimpling, discoloration, or communication with adjacent structures. The CT scan displayed a hypodense cystic lesions about -87 to +24 HU (Housefield units, average +3.2 HU) with hypodense capsule and no postcontrast enhancement. All tumors were found just under the skin, and were well encapsulated, so they were completely removed the mass with adjacent periosteum. Results: On gross findings, all tumors were oval-or round-shaped, and when the cystic tumor was cut open it presented a greasy and caseous substance. Histologically, all specimens contain desquamated squamous epithelium and keratin in the lumen and are encapsulated and lined by keratinized stratified squamous epithelium. And, all cases of posterior mass are the presence of adnexal structures. Conclusion: Appropriate diagnosis requires not only an index of suspicion for this rare tumor a very careful history and search for skin changes. Especially, CT can reveal the exact location of the cyst, its relationship with the adjacent structures. We think that occipital dermoids divide into superficial and deep type. In our cases, because they did not have intra-cranial involvement or fistula formation, they are superficial type. This report describes the clinical and operative aspects of the superficial dermoid cysts and provides a review of the literatures.

• Journal of The Korean Dental Society of Anesthesiology
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• v.7 no.1
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• pp.22-26
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• 2007

상악과 하악이 선천적으로 붙어서 태어나는 선천성 유합증은 드문 선천성 기형으로 단순히 점막이 붙은 점막유합증에서 악골이 붙은 골유합증까지 다양하게 나타난다. 이중 상악골과 하악골의 골자체가 붙는 골유합증은 아주 드물어서 현재까지 26증례만 보고되고 있는데, 보고된 대부분의 증례는 편측에만 발생하는 불완전형으로 알려져 있다. 7세 된 여아환자가 인디아의 GSR 병원에 입이 벌어지지 않는다는 주소로 내원하였는데 환아의 턱은 완전히 움직이지 않았으며, 2-3 mm 정도 벌어지는 앞니부위에서는 2.5 cm 폭경의 3.0 mm 두께의 단단한 치조점막이 관찰되었다. 전기메스로 전방부의 부착성 섬유밴드를 잘라준 후 즉각적인 개구정도는 16 mm 정도까지 가능하여 구강으로의 기관삽관이 가능하였다. 삽관후 양쪽 후방부 협측점막의 두꺼운 밴드들을 모두 제거하여 개구량을 33 mm까지 증진시킨 후 수술을 종결하였다. 환아의 보호자에게 거즈 블록과 설압자를 이용하여 개구 연습을 능동적으로 시키도록 강조하여 교육하였으며 술후 16개월 경과시까지 특별한 합병증이나 개구량 감소는 관찰되지 않았다. 독립적으로 발생한 선천성 치조점막 유합증 환자에서 비정상적으로 커져있는 과두와 설골이 관찰되었는데, 설-하악 구조의 비정상적인 발육에 기인하여 지속적인 비정상적 운동으로 인한 이차적인 치은과 협점막의 섬유성 부착이 생긴 것으로 추측되었다. 이에 마취과와의 효율적인 협진으로 기관절개술 등의 부가적인 마취방법 없이 효과적으로 치료할 수 있었다.

• Korean Journal of Cleft Lip And Palate
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• v.4 no.1
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• pp.13-26
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• 2001

Cleft lip and/or palate is the congenital orofacial malformation most commonly occurred in humans, The disease is multifactorial and is probably caused by genetic and/or environmental factors, So, there are many problems in research concerning etiology and in treatment of the disease, Even the most practiced and sophisticated methods of surgical repair are necessarily followed by scar contraction and fibrosis, which result in skeletal defects, dental abnormalities, cosmetic disfigurement, and speech impairment, As a result, Fetal surgery can be considered but practiced rarely when the deformity is not fatal to life, And treatment of cleft palate is performed in the form of medicine projection into uterus in animal experiments, Many studies show that growth factor and its receptor emerge from the developing palate; and the epidermal growth factor receptors have a important role in craniofacial development and in palatal fusion, The palatal morphogenesis of the avine is different from the mammal's; it takes the form of physiologic cleft palate, Recently, cleft palate fusion experiment was performed when the avine were in the period of palate formation through the exogenous TGF-β3 addition, and it showed that the exogenous TGF-β3 makes fusion of divided palate through certain process when cleft palate is occurred in palatal formation, In this study, I had the conformation of the fusion of cleft palate through the addition of TGF-β in case of chicken embryo, and observed the effect of TGF-β in EGF receptor distribution, And the following is the results of this study, 1. In case of the TGF-βl and β3 addition group, there was the decrease of EGFR(Epidermal Growth Factor Receptor) immunoreactivity in mesenchymal cells beneath the medial edge epithelium and also in epithelial mesenchymal interface which is between medial edge epithelium and nasal septum in 72 hours, 2, The immunoreactivity of the control group resembles that of normal chicken embryo palate in development, 3. In the view through fluorescence confocal microscopy, there was confluence in TGF-β3 addition group, This shows that the confluence induced by exogenous TGF-β3 is related to EGFR expression in palate of chicken embryo, which is a physiologic cleft palate model.

• Journal of Veterinary Clinics
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• v.26 no.5
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• pp.467-471
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• 2009

This report describes Neural Tube Defects (NTDs) with Abdominal Wall Defects (AWDs) on the sibling of Yorkshire terriers. The NTDs and AWDs are rare serious congenital defects. The NTDs are neurulation abnormality that results from to failed transformation of the neurual tube by the incomplete closure of the embryonic neural plate. These dysraphic states range form mild to severe according to developmental malformation that include fusion defects of skull (crania bifida; CB) and fusion defects of vertebrae (spina bifida; SB). The AWDs are genetic defects that results from to failed formation of abdominal wall and cavity. These dysraphic states are omphalocele and gastroschisis. The 12-month dam was delivered by caesarian section and 4 littermate had obvious malformations. One male dead stillbirth fetus (L1) was revealed the extruded abdominal viscera, omphalocele. One female fetus (L2) was died within 1 hour after birth with defects of abdominal muscle upper umbilicus, gastroschisis. 3rd fetus (L3) was died within 36 hours after parturition and revealed a copious dermal and vertebral defects on the midline thorax, upper SB asperta. 4th fetus (L4) is still growing well now at 6 months but at the 2 week age, appears hairy nevus on the frontal cranium and dorsal thoracic portion. The radiograph of L1 and L2 are shown decrease bony density of calvarium and L3 was shown defect of spinose processes of the T9-T13. On our knowledge, this is first report of the SB and CB in Yorkshire terrier. And also sibling of NTDs with AWDs that has not previously been reported in the dog.

• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.697-705
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• 1996

Ankyloglossia, or tongue-tie, is a congenital condition which occurs as a result of fusion between the tongue and the floor of the mouth. Ankyloglossia often results in malocclusion with an anterior "open bite" deformity, early prognathism, swallowing problem, speech disorder, and periodontal problem. Generally lingual frenectomy is used for treatment of ankyloglossia, but incomplete operation and simple frenectomy may produce a scar contracture resulting in a more deformed ankyloglossia than was present initially. The Z-plasty is used for the correction of scar contractures and the replacement of missing tissue and this procedure is ideally suited for the treatment of an ankylosed frenum. Most authors advise postponement of any decision for surgical correction of tongue-tie until the age of 4 years, unless the child is having much difficulty with sucking or swallowing. We treated 4 patients with ankyloglossia using Z-plasty technique. As a result, we found out that it was effective for correction of movement limitation of tongue, prevention of relapse. Further, periodic check ups are needed for evaluation of relapse, improvement of speech, and other functions of the tongue.

• Journal of Korean Medicine Rehabilitation
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• v.25 no.2
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• pp.175-180
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• 2015

The Klippel-Feil Syndrome (KFS) is characterized by congenital fusion of two or more vertebrae with hypermobile normal segment. According to this, a patient with KFS can be at risk of severe neurological symptoms after manipulation treatment. We had a KFS patient who suffered from neck pain and limited range of motion at cervical after manipulation treatment. The patient, 49-year-old woman was diagnosed as KFS through Cervical X-ray and MRI. The patient was treated by acupuncture therapy and SCENAR therapy. We measured neck pain by visual analog scale (VAS) and neck disability index (NDI) and checked range of motion at cervical before and after the treatments. After 8 times treatments, the patient's pain decreased and the range of motion increased. From this case, we can recognize the risk of Chuna manipulation treatment for KFS patient. So, we suggest that radiological examination is needed before cervical Chuna manipulation treatment to avoid adverse reactions.

• Journal of Korean Foot and Ankle Society
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• v.27 no.1
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• pp.24-29
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• 2023

Macrodactyly of the toe is a rare congenital anomaly characterized by the overgrowth of a digit/digits in the foot and is one of the most difficult conditions to treat. Since the condition alters functionality and appearance, the treatment goal is to restore function and cosmetically enhance the appearance. Various surgical techniques are used for toe macrodactyly, including amputation, debulking, and epiphysiodesis. Herein, we present a case of a six-year-old patient with a second toe macrodactyly who was successfully treated with a two-stage operation over a seven-year period. We initially performed an ostectomy of the middle phalanx with a fusion of the proximal and distal phalanges and then performed a soft tissue debulking procedure.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.1
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• pp.44-50
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• 2002

Apert syndrome is a kind of congenital-acrocephalosyndactyly syndrome which was first reported by Apert in 1906 and characterized by its acrocephaly and syndactyly. Clinical characteristic features are cone-shaped skull morphology due to early fusion of coronal suture, fusion of fingers of hands and toes of feet. It is an autosomal dominant-heritable syndrome. Due to hypo-development of midface region, Apert syndrome patients have a tendency to have ocular proptosis, hypertelorism, maxillary deficiency. High palate and soft palate cleft are common findings in these patients. In general, mandibular growth pattern is normal, but relative maxillary deficiency exaggerates mandibular forward position, so relative mandibular prognathism is inevitable. Narrow maxillary and mandibular dental arch worsen teeth alignment and crowding. Skeletal malocclusion and open bite are also common. This is a case report of a Korean 3 year 1 month male Apert syndrome child referred by department of plastic surgeon for the possibility of orthodontic treatment. General features of Apert syndrome, patient's medical history, radiographic evaluation, clinical examination, orthodontic and surgical treatment planning are discussed in this report.

• Tuberculosis and Respiratory Diseases
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• v.48 no.1
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• pp.45-53
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• 2000

Background: The literature on variations of rib is limited. Very little has been written in the radiological journal of this country on the subject. It seemed of interest to investigate the nature and incidence of congenital variations in a series of routine chest roentgenograms. The topic of rib variations has not been covered extensively in the radiological journals in Korea. This has presented an opportunity to investigate the nature, type, shape and incidences of congenital rib variations in normal Korean adults from a series of routine roentgenograms. Methods: Chest radiographs of 5,000 adults, who visited our hospital for a routine check-up or for employment physical examinations from January 1996 to September 1998, were consecutively reviewed. The sex distribution consisted of 2,827 male males and 2,173 female females(ratio of 1.3:1) with the age range aged between 19 and 65 years(mean age: 34.6 years) were included. The chest PAs were analyzed for the presence, type, location, and shape of the rib variations. From this data the incidence of each type of variations was calculated. Results: Seventy-six of the 5,000 adults(1.52%), 63 male(2.23%) and 13 female(0.6%), showed 88 cases of rib variation(Table 1). The most common variation was the bifid rib(n=35), followed by hypoplasia of the rib(n=22), flaring of the rib(n=18), bridging of the ribs(n=7), cervical ribs(n=3), and fusion of between ribs(n=3)(Table 2). The bifid rib(Table 1) was found most frequent in the right fourth rib(12/35, 34.3%), followed by the left fifth rib(6/35, 17.1%) and right third rib(6/35, 17.1%). Hypoplasia of the rib was common in first rib(20/22, 90.9%). Flaring of the rib was common at fourth rib(8/18, 44.4%, right and left combined), and bridging between ribs was common between first and second rib(3/7, 42.9%). Conclusion : The percentage of incidence of rib variations in adults was 1.52%. Bifid rib was the most common variation, followed by hypoplasia, flaring, bridging, cervical rib, and fusion of ribs, in decreasing order.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.245-252
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• 2009

The congenital missing of teeth is common, which takes place since the proliferation and differentiation are not allowed in that tooth bud fail to start development. The purpose of this study is to research incidence rate, number, and missing part of congenital missing teeth, and to study whether a person who has missing teeth has other abnormality of teeth or not. For this study, 1,520 subjects(aged 2.9$\sim$17) who had visited pediatric dentist department of Chonbuk national university dental hospital within 2 years were examined with an panoramic radiograph; exempting third molar missing state. The obtained results are as follows. 1. 8.88% among total subjects show missing teeth; male 9.05%, female 8.64% 2. The most frequently missing permanent teeth were the mandibular second premolars(22.3%). The most frequently missing primary teeth are mandibular lateral incisors(50%). 3. 43.3% patients have one permanent missing tooth, 34.3% have two, and 10.4% have more than six, respectively. In primary teeth, 86.7% patients have one missing tooth, and 13.3% have two missing teeth. 4. 18 patients(13.3%) have missing teeth as well as hyperdontia, while some patients have microdont, ectopic eruption, and fusion teeth.