• Title/Summary/Keyword: primary congenital

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INCONTINENTIA PIGMENTI (BLOCH-SULZBERGER SYNDROME) WITH DENTAL PROBLEMS : CASE REPORT (색소실조증 환아의 증례보고)

  • Lee, Sang-Yup;Kim, Dae-Eop;Lee, Kwang-Hee
    • Journal of the korean academy of Pediatric Dentistry
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    • v.32 no.4
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    • pp.682-686
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    • 2005
  • Incontinentia pigmenti(IP), so called Block-Sulzberger syndrome is a rare genodermatosis that occurs almost in female infant; usually lethal in males, X-linked dominantly inherited disorder. IP is characterized by abnormalities of mesodermal and ectodermal tissues including eye, tooth, skin, nail, breast and hair as well as neurological deficiencies. Dental problems are congenital missing of teeth, delayed eruption, abnormal crown shape and so on. Here is a case of 6 year-old female with IP. She had congenital missing of primary and permanent teeth, delayed eruption, maxillary deficiency and extra cusps, resulting in unstable occlusion. Systemically, she had a history of operating eyes due to problem of retina and hyperpigmented macules on her trunk and extremities as typical character of IP.

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DENTAL CHARACTERISTICS OF A PATIENT WITH INCONTINENTIA PIGMENTI : A CASE REPORT (색소실조증 환자의 치과적 특성 : 증례보고)

  • Song, Jihyeo;Lee, Koeun;Song, Je Seon;Lee, Jaeho;Choi, Hyung-Jun
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.14 no.2
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    • pp.88-91
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    • 2018
  • Incontinentia pigmenti(IP), or Block-Sulzberger syndrome is a rare X-linked dominant genodermatosis that affects almost in female infant and is usually lethal for males in utero. IP is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. Dental problems are congenital missing of teeth, delayed eruption, abnormal crown shape. We reported a case of 5 year-old female with IP. She had congenital missing of multiple primary and permanent teeth, accessory cusp and cone-shaped crowns. Systemically, she had a problem of retina and hyperpigmented macules on her trunk and extremities as typical character of IP. As the syndrome involves many different medical problems and needs comprehensive consideration. Dental care should be performed in a multidisciplinary consultation system.

Reconstruction Techniques for Tissue Defects Formed after Preauricular Sinus Excision

  • Lee, Myung Joon;Yang, Ho Jik;Kim, Jong Hwan
    • Archives of Plastic Surgery
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    • v.41 no.1
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    • pp.45-49
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    • 2014
  • Background Preauricular sinuses are congenital abnormalities caused by a failure of fusion of the primitive tubercles from which the pinna is formed. When persistent or recurring inflammation occurs, surgical excision of the infected tissue should be considered. Preauricular defects inevitably occur as a result of excisions and are often difficult to resolve with a simple suture; a more effective reconstruction technique is required for treating these defects. Methods After total excision of a preauricular sinus, the defect was closed by a plastic surgeon. Based on the depth of the defect and the degree of tension when apposing the wound margins, the surgeon determined whether to use primary closure or a posterior auricular flap. Results A total of 28 cases were examined. In 5 cases, including 2 reoperations for dehiscence after primary repair, reconstruction was performed using posterior auricular transposition flaps. In 16 cases of primary closure, the defects were closed using simple sutures, and in 7 cases, closure was performed after wide undermining. Conclusions If a preauricular defect is limited to the subcutaneous layer and the margins can be easily approximated, primary closure by only simple suturing may be used to perform the repair. If the defect is deep enough to expose the perichondrium or if there is tension when apposing the wound margins, wide undermining should be performed before primary closure. If the extent of the excision exposes cartilage, the procedure follows dehiscence of the primary repair, or the tissue is not sufficiently healthy, the surgeon should use a posterior auricular flap.

A Clinicopathologic Analysis of Neck Masses (경부 종괴의 임상 및 병리학적 고찰)

  • Km Jeong-Ho;Oh Sang-Hoon;Kim Sang-Hyo
    • Korean Journal of Head & Neck Oncology
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    • v.13 no.1
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    • pp.51-57
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    • 1997
  • A mass appearing in the anterior or lateral side of neck often can be a diagnostic challenge. Differential diagnosis of the neck mass covers a broad spectrum of diseases and the proper evaluation and management of a neck mass requires an impressive amount of anatomic and pathologic information. Because improper diagnosis and management may convert a potentially curable malignant metastasis into incurable disease, a differential diagnosis must be considered in all patients who present with a neck mass. Authors reviewed 2,148 cases of neck mass who were diagnosed by surgical resection, biopsy or aspiration during the period between October 1982 to December 1993, excluding those with thyroid and parathyroid disease. The evaluated characteristics were age, sex, site of lesion, and pathologic diagnosis. The results were as follows: Of 2,148 cases of neck mass, the overall ratio of benign to malignant tumor was 3 : 1. In 1,603 cases of benign mass lesion, the most common disease was lymphadenitis(non-specific and tuberculosis) showing 53% incidence, the second was salivary gland tumor(13%), and the third was congenital lesion(12%). The minor problems such as lipoma and sebaceous cyst were 21 %. In the age distribution of benign lesion, tuberculous lymphadenitis showed peak incidence in second decade, non-specific lymphadenitis was main disease of childhood, salivary gland tumor was peak in fourth decade, and most of congenital lesions were diagnosed at the age below 15. In 545 malignant tumors, the most common lesion was metastatic cancer to cervical lymph nodes yielding 71 % incidence(head and neck primary 52%, infraclavicular primary 42%, unknown primary 5%), the second common disease was lymphoma(19%), and the third was salivary gland cancer(9%). In the age incidence of malignant tumor, 60% of them developed in the fifth and sixth decade, head and neck primary was more common in the fifth decade than sixth, however lymphoma showed higher incidence in sixth decade. In the analysis of mass location according to lymph node level grouping(I - V), lymphadenitis developed mostly in level V nodes, the next common occurring site was level IV in tuberculous lymphadenitis and level II in non-specific lymphadenitis. The majority of metastatic cancers were found in level IV and III, and common occurring site of lymphoma was in level II and IV. Pathologic diagnosis of neck masses were made by fine needle aspiration cytology 80 cases, incisional biopsy 533 cases, excisional surgery 1,399 cases, and neck dissection 116 cases. For the proper management of neck mass, a proper diagnostic modality should be selected from imaging techniques, cytology, biopsy or neck dissection, with the consideration of patient's age, history and clinical findings. The scapel biopsy could be used freely in the inflammatory disease or inoperable metastatic cancer, but it should be reserved in the curable metastatic cancer or clinically possible malignancy.

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EARLY DEVELOPMENT OF THE TOOTH IN THE STAGED HUMAN EMBRYOS AND FETUSES (한국인 배자 및 태아에서 유치 발생의 조직학적 변화)

  • Lim, Hee-Sik;Park, Hyoung-Woo;Oh, Hyeon-Joo;Kim, Hee-Jin;Choi, Byung-Jai
    • Journal of the korean academy of Pediatric Dentistry
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    • v.25 no.2
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    • pp.383-399
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    • 1998
  • Tooth development is usually described in four stages such as bud stage, cap stage, bell stage and crown stage. Exact time of appearance of tooth primordia is different among reports, and up to now there is no timetable regarding initial tooth development. To understand the congenital malformations and other disorders of the orofacial region, there is a need to establish a standard timetable on early tooth development. Till now, studies on the tooth development were mainly on later fetuses, and only few reports on early stage. Also, there were no reports on the time when bud stage turns to cap stage, and cap stage to bell stage. In this study, external morphology of face and the early development of the tooth, and transition of bud stage to cap stage, cap stage to bell stage were studied using 27 staged human embryos and 9 serially sectioned human fetuses. The results are as follows: 1. Mandibular region was formed by union of both mandibular arch at stage 15, and maxillary region by union of maxillary arch, medial nasal prominence, and intermaxillary segment at stage 19. 2. Ectodermal thickening which represents the primordia of tooth appeared in mandibular region at stage 13, and maxillary region at stage 15. 3. Bud stage began from mandibular primary central incisor at stage 17, and maxillary primary central incisor at stage 18. And the sequence of appearance was in the mandibular primary lateral incisor at stage 19, maxillary primary lateral incisor at stage 20, mandibular primary canine at stage 22, maxillary primary canine and primary first molar at stage 23, madibular primary first molar and maxillary primary second molar at 9th week, and mandibular primary second molar at 10th week of development. 4. Cap stage began from the primary anterior teeth at 9th week, and primary second molar still had the characteristics of cap stage at 12th week of development. 5. Transition to bell stage started from the primary anterior teeth at 12th week, and primary second molar started at 16th week of development. 6. Trnasition to crown stage started from primary anterior teeth at 16th week, and primary second molar at 26th week of development.

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COMPOUND ODONTOMA WITH CONGENITAL MISSING OF THE PERMANENT TEETH: CASE REPORTS (영구치의 선천적 결손을 동반한 복합 치아종)

  • Kim, Ha-Na;Kim, Jae-Gon;Baik, Byeong-Ju;Han, Ji-Hye;Yang, Yeon-Mi
    • Journal of the korean academy of Pediatric Dentistry
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    • v.34 no.4
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    • pp.679-684
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    • 2007
  • Odontoma is the most common benign odontogenic tumors, and have been defined as mixed odontogenic tumor composed of epithelial and mesenchymal cells. Odontoma is believed to be hamartomatous rather than neoplastic in nature. The classification by WHO divides odontoma into 2 groups such as complex odontoma and compound odontoma. Compound odontoma comprises dental tissues, resembling the morphology of a tooth and has predilection for the anterior maxilla. In contrast, complex odontoma has unorganized mass, not resembling the normal tooth and has predilection for the posterior mandible. Odontoma is almost asymptomatic, so it is usually found on routine radiographic examination. Common presenting symptom is impacted or unerupted permanent teeth and retained primary teeth, but coexistent odontoma and congenital missing of permanent teeth is a very rare condition. The recommended treatment for an odontoma is conservative surgical excision, with care taken to remove the surrounding soft tissue. This report presents 2 patients with compound odontoma of the mandible who have congenital missing of the permanent teeth.

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Primary Pulmonary Hypertension in a Maltese Dog (말티즈 견에서 발생한 원발성 폐동맥 고혈압증)

  • Moon, Hyeong-Sun;Lee, Seung-Gon;Choi, Ran;Park, In-Chul;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.24 no.4
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    • pp.613-617
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    • 2007
  • A 1-year-old castrated male Maltese dog(weighing 2.4 kg) was presented with primary complaints of occasional coughing, dyspnea and exercise intolerance. Based on diagnostic findings including paradoxical split S2, diastolic regurgitant murmur, marked dilation of pulmonary artery, right ventricular eccentric hypertrophy with thickening of ventricular septum, severe tricuspid and pulmonic regurgitation(5.4 m/sec and 3.4 m/sec, respectively) and the absence of any congenital intracardiac shunting, obstructive pulmonary diseases and systemic diseases associated with right ventricular pressure overload or pulmonary hyperperfusion, the case was tentatively diagnosed as primary pulmonary hypertension. The dog was treated with furosemide, aspirin and oxygen supplementation. This case report described a rare case of primary pulmonary hypertension in a Maltese dog.

Surgical Treatment for Primary Lymphedema: A Systematic Review of the Literature

  • Miguel Angel Gaxiola-Garcia;Joseph M. Escandon;Oscar J. Manrique;Kristin A. Skinner;Beatriz Hatsue Kushida-Contreras
    • Archives of Plastic Surgery
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    • v.51 no.2
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    • pp.212-233
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    • 2024
  • This is a retrospective review of surgical management for primary lymphedema. Data were extracted from 55 articles from PubMed MEDLINE, Web of Science, SCOPUS, and Cochrane Central Register of Controlled Trials between the database inception and December 2022 to evaluate the outcomes of lymphaticovenous anastomosis (LVA) and vascularized lymph node transfer (VLNT), and outcomes of soft tissue extirpative procedures such as suction-assisted lipectomy (SAL) and extensive soft tissue excision. Data from 485 patients were compiled; these were treated with LVA (n = 177), VLNT (n = 82), SAL (n = 102), and excisional procedures (n = 124). Improvement of the lower extremity lymphedema index, the quality of life (QoL), and lymphedema symptoms were reported in most studies. LVA and VLNT led to symptomatic relief and improved QoL, reaching up to 90 and 61% average circumference reduction, respectively. Cellulitis reduction was reported in 25 and 40% of LVA and VLNT papers, respectively. The extirpative procedures, used mainly in patients with advanced disease, also led to clinical improvement from the volume reduction, as well as reduced incidence of cellulitis, although with poor cosmetic results; 87.5% of these reports recommended postoperative compression garments. The overall complication rates were 1% for LVA, 13% for VLNT, 11% for SAL, and 46% for extirpative procedures. Altogether, only one paper lacked some kind of improvement. Primary lymphedema is amenable to surgical treatment; the currently performed procedures have effectively improved symptoms and QoL in this population. Complication rates are related to the invasiveness of the chosen procedure.

Therapeutic Effect of OK-432 for Lymphangioma in Children (OK-432를 사용한 소아 림프관종 치료효과)

  • Jung, Sung-Eun;Kim, Dae-Yeon;Shim, In-Bo;Lee, Seong-Cheol;Park, Kwi-Won;Kim, Woo-Ki
    • Advances in pediatric surgery
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    • v.4 no.2
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    • pp.131-136
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    • 1998
  • Lymphangioma is a congenital malformation of the lymphatic system that expands into the surrounding tissues in a manner similar to a malignant tumor. Surgical excision has been the treatment of choice, but the results are often unsatisfactory due to incomplete resection, fluid collection and recurrences. Intralesional injection of OK-432(lyophilized incubation mixture of Group A Streptococcus pyogenes of human origin) was employed in treating 24 patients with lymphangiomas between 1992 and 1997. The method was used in 21 patients as a primary therapy, and in 3 patients for unresectable or recurrent cases as a secondary treatment. The results were excellent in 8 patients (33.3%), good in 7 patients (21.8 %) and poor in 7 patients (21.8 %). The side effects were fever under $39^{\circ}C$ and local inflammation. However these symptoms subsided in a few days without serious sequelae. These results suggest that intralesional injection of OK-432 is safe and effective treatment for primary lymphangioma as well as a secondary therapy for unresectable or recurrent cases.

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Mucoperiosteal Flap Necrosis after Primary Palatoplasty in Patients with Cleft Palate

  • Rossell-Perry, Percy;Cotrina-Rabanal, Omar;Barrenechea-Tarazona, Luis;Vargas-Chanduvi, Roberto;Paredes-Aponte, Luis;Romero-Narvaez, Carolina
    • Archives of Plastic Surgery
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    • v.44 no.3
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    • pp.217-222
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    • 2017
  • Background The prevalence of flap necrosis after palatoplasty in patients with cleft palate. The prevalence of mucoperiosteal flap necrosis after palatoplasty remains unknown, and this complication is rare. This event is highly undesirable for both the patient and the surgeon. We present here a new scale to evaluate the degree of hypoplasia of the palate and identify patients with cleft palate at high risk for the development of this complication. Methods In this case series, a 20-year retrospective analysis (1994-2014) identified patients from our records (medical records and screening day registries) with nonsyndromic cleft palate who underwent operations at 3 centers. All of these patients underwent operations using 2-flap palatoplasty and also underwent a physical examination with photographs and documentation of the presence of palatal flap necrosis after primary palatoplasty. Results Palatal flap necrosis was observed in 4 cases out of 1,174 palatoplasties performed at these centers. The observed prevalence of palatal flap necrosis in these groups was 0.34%. Conclusions The prevalence of flap necrosis can be reduced by careful preoperative planning, and prevention is possible. The scale proposed here may help to prevent this complication; however, further studies are necessary to validate its utility.