• Title/Summary/Keyword: congenital malformation

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Abberant Root Morphology in the Permanent First Molars : Case Reports (제1대구치에서 관찰되는 비정형적 치근형태에 대한 증례보고)

  • Lee, Eunkyoung;Kim, Youngjin;Kim, Hyunjung;Nam, Soonhyeun
    • Journal of the korean academy of Pediatric Dentistry
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    • v.42 no.2
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    • pp.172-179
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    • 2015
  • The developmental mechanism of root formation is a complex process. Hereditary and environmental factors may affect the morphology of the developing root. A total of 12 cases was presented with permanent first molars with abberant root morphology. Clinically, these teeth appeared as a normal crown. However, radiographically, the root was slender, twisted and characterized by irregular lengths. In addition, root trunk length was shorter and pulp chamber was obliterated. In these cases, periapical radiolucency and loss of lamina dura were often observed. In 6 cases, an abnormal root of the primary second molars were also present, as well as root malformation of permanent first molars. In 3 cases, permanent central incisors also had a dysmorphic crown. These cases almost all had medical history, such as premature birth, brain infection or congenital heart disease in infants. The present paper describes cases of permanent first molars with an abnormal root that are rarely reported in literature. This case may intensify the variation in the permanent first molar and is intended to reinforce the clinician's awareness of rare morphology of the roots.

Treatment of Pulmonary Sequestration with Thoracoscopic Approach (폐분리증의 치료와 흉강경적 절제술의 경험)

  • Cho, Min-Jeng;Kim, Tae-Hoon;Kim, Dae-Yeon;Kim, Seong-Chul;Kim, In-Koo
    • Advances in pediatric surgery
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    • v.16 no.2
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    • pp.154-161
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    • 2010
  • Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The anomaly is characterized by absence of communication with the tracheobronchial tree and isolated blood supply from an anomalous systemic vessels. With the utilization of antenatal ultrasound, the diagnosis of asymptomatic neonatal PS has increased. Treatment options include observation, arterial embolization and surgical resection. The aim of the present study is to review the clinical course of PS and to share our experience with thoracoscopic resection. A total of 96 patients with PS were treated at Asan Children's Hospital between 1999 and 2010. The diagnosis of PS was established by CT in the cases managed by observation or embolization, and by tissue pathology in the surgical cases. Medical records and radiographic images were retrospectively reviewed. Thirty-nine patients were managed by embolization and 30 patients by surgery. The remaining 27 patients have been under observation without any procedures. Among 27 observation patients, 1 patient regressed completely and 10 patients were lost to follow up. Of the 39 embolizations patients, 2 had their lesion regress and sepsis was suspected after embolization. In 1 patient, the microcoil migrated to the iliac artery during the embolization procedure, and another patient developed renal abscess caused by renal artery embolization. Among 30 surgical cases, resection by thoracotomy was performed in 27 at the Department of Thoracic Surgery, and thoracoscopic resection in 3 at the Division of Pediatric Sugery. Only one wound complication ocurred. We conclud that surgical excision should be recommended for pulmonary sequestration, whether the sequestration is symptomatic or not because of the risk of infection, the low rate of natural regress, poor compliance, severe complications after embolization, and to exclude other pathology. In summary, thoracoscopic resection of the pulmonary sequestration is feasible, efficacious, safe and cosmetically superior even in neonatal period.

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DENTAL CARIES TREATMENT IN A PATIENT WITH DANDY-WALKER SYNDROME : A CASE REPORT (댄디워커 증후군 환아의 치아우식 치료 증례 보고)

  • Ryu, Jiyeon;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Young Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Kim, Chong-Chul;Lee, Sang-Hoon
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.12 no.2
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    • pp.77-81
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    • 2016
  • The Dandy-Walker syndrome is a relatively rare congenital malformation occurring about one in 25,000-35,000 pregnancies. It is characterized by hydrocephalus, cystic dilatation of the fourth ventricle, complete or partial absence of the cerebellar vermis, and other various extra-CNS malformations. We report a dental caries treatment of a 4-year old girl with Dandy-Walker syndrome and partial trisomy 9q. The patient visited Seoul National University Dental Hospital due to multiple caries. The cause of multiple caries is mainly presumed as patient's eating habit caused by her general condition. She was still using milk bottle because she was having difficulties swallowing solid food due to breathing problem while eating. The treatment was performed under general anesthesia considering patient's condition; delayed development, very poor cooperation and respiratory problem. The dental procedure was successfully conducted and there were no postoperative complications. In consideration of her eating habit and oral hygiene problem, regular check appointment was recommended.

Role of Trophobolast in Implantation and Placenta Development (착상 및 태반 발달과정에 따른 영양막세포의 역할)

  • Kim, Gi-Jin
    • Clinical and Experimental Reproductive Medicine
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    • v.37 no.3
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    • pp.181-189
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    • 2010
  • The placenta, which is a temporary organ derived from the fetus during pregnancy, is critical to support fetus development via optimal regulation between mother and fetus. Trophoblast as a major cell population of the placenta is one of the earliest to differentiate and shows an extensive proliferation or/and differentiation up to the formation of the placenta. The role of the trophoblast show dynamic changes from early embryo implantation to placentation during pregnancy. Implantation of the blastocyst into the endometrium of the maternal uterus is mediated by invasion of the differentiated trophoblast (e.g. syncytiotrophoblast) from the trophectoderm. During pregnancy, the unique role of the trophoblast is to invasion, eroding, and metastasizing in the placenta as well as to ensure appropriate bidirectional nutrient or waste flow required for growth and maturation of the embryo. The dysfunction of the trophoblast during pregnancy can result in several gynecological diseases including preeclampsia and congenital malformation in neonatal medicine. Therefore, trophoblasts act as a conclusive factor in placental and fetal development. This brief review outlines the classification of trophoblast and its function in the placenta during pregnancy. Also, we introduce the latest research in trophoblast for implantation and the placenta development, and the application potential of trophoblast for infertility and obstetrical diseases.

Diagnosis and Post-Therapeutic Evaluation of Arteriovenous Malformations in Extremities Using Transarterial Lung Perfusion Scintigraphy (경동맥 폐관류 신티그라피를 이용한 상하지 동정맥 혈관기형의 진단과 치료 평가)

  • Chung, Hyun-Woo;Choi, Joon-Young;Kim, Young-Wook;Kim, Dong-Ik;Do, Young-Soo;Lee, Eun-Jeong;Lee, Su-Jin;Cho, Young-Seok;Hyun, Seung-Hyup;Lee, Kyung-Han;Kim, Byung-Tae
    • Nuclear Medicine and Molecular Imaging
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    • v.40 no.6
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    • pp.316-321
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    • 2006
  • Purpose: Differential diagnosis between arteriovenous (AVMs) aud non-arteriovenous malformations (nAVMs) is important in patients with congenital vascular malformations, because AVMs can cause hemodynamic alteration and require immediate treatment. We investigated whether transarterial lung perfusion scintigraphy (TLPS) was useful for the diagnosis and post-therapeutic evaluation of AVMs in extremities. Materials and Methods: Fifty-seven patients (M:F=26:31, $21{\pm}13$ yr, 9 upper and 48 lower extremities) suspected of congenital vascular malformations in extremities underwent TLPS using $^{99m}Tc-MAA$ before embolization/sclerotherapy. Dose-corrected shunt fraction (SF) was calculated from time-activity curve of the lung. Final diagnosis of AVMs was determined by angiography. in patients with AVMs, follow-up TLPS was done for post-therapeutic evaluation. Results: Sixteen patients (8 upper and 8 lower extremities) had AVMs, while the remaining 41 had nAVMs (1 upper and 40 lower extremities). The mean SF of AVMs on TLPS was significantly higher than that of nAVMs ($66.4{\pm}25.8%\;vs.\;2.8{\pm}4.3%$), p=0.003). The sensitivity, specificity, and accuracy of TLPS (cut-off of SF = 20.0%) in diagnosis of AVMs before treatment were 93.8% (15/16), 100% (41/41) and 98.2% (56/57), respectively. The follow-up TLPS and angiography for post-therapeutic evaluation showed concordant results in 13 of 16 patients (81.3%) with AVMs. The mean SF of TLPS was significantly decreased after embolization/sclerotherapy ($69.5{\pm}24.0%\;vs.\;41.0{\pm}34.7%$, p=0.01). Conclusion: TLPS provides hemodynamic information of AVMs in extremities semiquantitatively. Furthermore, the results of TLPS showed a high concordance rate with angiographic findings. Therefore, TLPS is useful for the diagnosis and post-therapeutic evaluation of AVMs in extremities.

A Clinical Analysis of Neonatal Surgical Gastrointestinal Diseases in Daegu·Busan Area (대구·부산 지역에서 수술을 요하는 신생아 소화기 질환의 임상적 고찰)

  • Son, Seung Kook;Park, Jae Hong;Choi, Byung Ho;Choi, Kwang Hae;Lee, Kyoung Hoon
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.2
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    • pp.179-185
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    • 2004
  • Purpose: Perinatal mortality rates have been used as a summary statistic for evaluating child health and medical status. Neonatal mortality rates have decreased over the past 30 years in Korea. To understand the current status of neonatal surgical gastrointestinal diseases in Daegu Busan area, we have studied about neonatal gastrointestinal diseases with their clinical features, postoperative outcome, and mortality rates. Methods: A clinical analysis on 202 neonates who underwent neonatal surgery from January 1996 to July 2003 at Pusan National University, Kyungpook National University, Youngnam University, and Daegu Catholic University was carried out. Results: The main diseases of surgical conditions were anorectal malformation (23.8%), atresia/stenosis of midgut (13.4%) and pyloric stenosis (13.4%). The male to female ratio was 2.8 : 1. Thirty-five cases (17.0%) had one or more associated anomalies including congenital heart disease, cryptoorchidism, hydronephrosis, and chromosomal anomaly. Twenty cases (10.0%) were diagnosed by antenatal ultrasound. Patients with esophageal atresia had the longest hospitalization for 54.6 days. Postoperative complications occurred in 18 cases (8.9%). The main postoperative complications were wound infection (3.5%) and anastomotic leakage (2.5%). Overall mortality was 5.9%. Diaphragmatic hernia showed the highest mortality rate (37.5%), and esophageal atresia (28.6%) and omphalocele (20.0%) were followed. Conclusion: The current status of neonatal surgical gastrointestinal diseases in Daegu Busan area has improved because the disease categories are various, postoperative complications and mortality rates are decreased.

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결장루형성술 환자 간호를 위한 일 연구

  • 모경빈
    • Journal of Korean Academy of Nursing
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    • v.1 no.1
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    • pp.27-43
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    • 1970
  • This study is designed to find out proper nursing activities for the needs of the colostomy patients, i.e., mental and psychological as well as physical needs for rapid recovery, and to help them build up the follow-up care for proper social adjustment. The study is based on 268 cases out of 381 colostomy patient's records kept in Ewha Womans University Hospital, Yonsei Medical Center, and National Medical Center in between the period from Jan. 1953 to Jan. 1970. The items of study are mainly on etiology, sex, age, duration of hospitalization, mortality rate, seasonal frequency, time from the onset of illness to the admission of the hospital, signs and symptoms. 1. Frequency of onset by etiology: Neoplastic disease 112 cases (42%), Inflammatory disease 33 cases (12%), Congenital malformation 30 cases (11%), Intussusception 25 cases (9.3%), Trauma 24 cases (9%), Volvulus 17 cases (6.3%), and Crohn's disease 6 cases (2.2%). 2. By sex: male 167 cases (62.9%), and female 101 cases (37.1%). So the ratio of portion of male and female 2:1. 3. By age: under 1·year·old 27 cases (10.1%) highest, 41-50 yrs 54 cases (20.2%), 51-60 yrs 42 cases (15.5%), above 71 yrs 5 cases (1.9%). 4. Duration of hospitalization: the shortest is 2-days and the longest is 470 days. 1-20-days 52%, 40-60 days 14%. 5. Mortality rate: Under the 10-days-admission 19.5%, and the beyond 30-days-admission 3.9%. 6. Seasonal frequency: Higher in summer (32% ). 7. Signs and symptoms: abdominal pain (56%), abdominal distention (54%), vomiting (40%), bloody mucoid diarrhea (38%) , pain of anal region (18%), abdominal tenderness, anorexia, indigestion, constipation, disuria, tenesmus, high fever and chilling sensation, bile tingled vomiting. Nursing activities for the patient's physical needs are as follows: Skin care for colostomy region, Prevention of colostomy constriction and depression, Removal of an offensive odor, The use of colostomy bag-selection for, and demonstration of the use of inexpensive colostomy irrigation equipment, Personal hygiene, general skin care, care of hair, finger nails and toe-nails, Oral hygiene, sleep and rest, aquate, Daily activities, etc. Measures for regulation of bowl movement. Keeping the instruction of taking food, Preparing the meal and help for anorexia, Constipation and it's solution, Prevention of diarrhea, helping the removal of mucous, and stretch constricted steam as needed. Nursing activities for pt's socio-psychological needs are as follows; Help the patient to make decision for the operation, Remove pt's anxiety toward operation and anesthesia, To meet the pt's spiritual needs at his death bed, Help to establish family and friends cooperation, Help to reduce anxiety at the time of admission and it's solution, Help to meet religious need, Help to remove pt's anxiety for loosing his job and family maintenance, Follow-up studies for 7 cases have been done to implement the present thesis. The items of the personal interviews with the patients are as follows: Acceptability for artificial anus, The most anxious thing they had in mind at the time of discharge, The most anxious thing they hat·e in mind at present, Their friends and family's attitudes toward the patient after operation, Relations with other colostomy patients, Emotional damage from the operation, Physical problem of enema, irrigation, Control of diet, Skin care, Control of offensive odor, Patient's suggestions to nurses during hospital stay and after discharge. In conclusion, the follow-up care for colostomy patients shares equal weight or perhaps more than the post-operative care. The follow-up care should include the spiritual care for moral support of the patient, to drag him out of isolation and estrangement, and make him fully participate in social activities. It is suggested that the following measures would help to rehabilitate the colostomy patients (1) mutual acquaintance with other colostomy patients if possible form a sort of club for the colostomy patient to exchange their experiences in care (2) through the team work of doctor, nurse and rehabilitation specialists, to have a sort of concerted effort for betterment of the patient.

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Imaging Findings of Pneumothorax Caused by Bronchial Cartilage Hypoplasia in a Dog (개에서 기관지 연골 저형성에 의해 발생한 기흉의 영상학적 진단 증례)

  • Kim, Su-yeon;Kim, Seong-soo;Lee, Jeo-soon;Yun, Soo-kyung;Oh, Hyun-jung;Shon, Jung-min;Kim, Bo-eun;Kim, Wan-hee;Yoon, Jung-hee;Choi, Min-cheol
    • Journal of Veterinary Clinics
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    • v.32 no.5
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    • pp.469-472
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    • 2015
  • A 10-year-old, castrated poodle dog presented with a cough for 2 weeks, and the cough initially developed since very young age. On radiographs, pneumothorax was noticed by characteristics of radiolucent area without pulmonary markings along the thoracic wall and diaphragm, retracted lung lobes from the thoracic wall and severely decreased volume of the left cranial lung lobe with disconnected bronchus. Computed tomography (CT) findings identified several pulmonary air-filled cysts and collapsed lung with abnormal shape and non-tapered end of bronchus, bronchioles at the accessory lobe and left cranial lobe. Also, pneumothorax, pneumomediastinum and subcutaneous emphysema were found. Imaging diagnosis was the spontaneous pneumothorax caused by ruptured emphysematous bullae associated with congenital bronchial cartilage abnormality or bronchial tree malformation. On surgery, hypoplasia of the left cranial lobe, right middle lobe, and accessory lobe with a bulla where air was leaking was identified. The accessory lobe was partially resected and bronchial cartilage hypoplasia was confirmed by histopathologic examination.

The Effectiveness of Intravenous Immunoglobulin for Clinically Suspected Neonatal Sepsis (임상적으로 의심되는 신생아 패혈증에서 정맥용 면역 글로불린의 치료 효과)

  • Na, Hyun Jung;Kim, Ji Young;Lee, Gyeong Hoon;Lee, Jun Hwa;Choi, Eun Jin;Kim, Jin Kyung;Chung, Hai Lee;Kim, Woo Taek
    • Clinical and Experimental Pediatrics
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    • v.48 no.11
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    • pp.1187-1192
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    • 2005
  • Purpose : The purpose of this study is to determine the effectiveness of intravenous immunoglobuin (IVIG) administration in fullterm neonates having clinically suspected neonatal sepsis. Methods : Forty full-term neonates admitted to the neonatal intensive care unit with clinically suspected neonatal sepsis, who had at least two positive diagnostic criteria were enrolled. Twenty neonates were enrolled into the IVIG arm and 20 in the placebo arm. Neonates with a gestational age of less than 36 weeks and those with any major congenital malformation were excluded. The neonates were randomized to receive 1 g/kg of IVIG or equivalent amount of normal saline. The treatments including antibiotics and supportive care were administered. Results : The neonates in the therapy and placebo groups were comparable in terms of birth weight, gestational age, sex distribution, duration of antibiotics therapy and admission, elevation of serum IgG level, mortality rate, change of CBC, and serum level of acute phase reactants etc. Conclusion : Serum IgG values increased significantly 5 days after administration of IVIG in the IVIG-treated group and decreased significantly 5 days after administration of normal saline in the placebo group. However, there was no significant difference in the duration of antibiotics therapy and admission, or of mortality between the IVIG-treated and placebo groups. No adverse reactions to the IVIG infusions were noted during the study. Our preliminary observations suggest that the administration of 1 g/kg IVIG to neonates had some effect on augmentation of humural immune status in neonates with clinically suspected sepsis. But further study is needed to verify the benefit of IVIG infusion to neonatal sepsis.

Minimal Invasive Surgery: A National Survey of Its Members by the Korean Association of Pediatric Surgeons (최소 침습 수술: 대한소아외과학회 회원을 대상으로 한 전국조사)

  • Kim, Dae Yeon;Kim, I.S.;Kim, H.Y.;Nam, S.H.;Park, K.W.;Park, W.H.;Park, Y.J.;Park, J.H.;Park, J.Y.;Park, J.S.;Park, J.Y.;Boo, Y.J.;Seo, J.M.;Seol, J.Y.;Oh, J.T.;Lee, N.H.;Lee, M.D.;Jang, J.H.;Jung, K.H.;Jung, S.Y.;Jung, S.E.;Jung, S.M.;Jung, E.Y.;Jung, J.H.;Cho, M.J.;Choi, K.J.;Choi, S.J.N.;Choi, S.O.;Choi, S.H.;Choi, Y.M.;Hong, J.
    • Advances in pediatric surgery
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    • v.20 no.1
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    • pp.1-6
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    • 2014
  • Minimal invasive surgery (MIS) has rapidly gained acceptance for the management of a wide variety of pediatric diseases. A questionnaire was sent to all members of the Korean Association of Pediatric Surgeons. Thirty one members (25.4%) took part in the survey that included data for the year 2012: demographic details, opinion regarding minimal invasive surgery and robotic surgery, spectrum of minimally invasive operations, and quantity of procedures. 48.4% of the respondents had more than 10 years experience, 35.5% less than 10 years experience, and 16.1 % had no experience. The respondents of the recommend MIS and perform MIS for surgical procedures are as follow; inguinal hernia (61.3%), simple appendicitis (87.1%), complicated appendicitis (80.6%), reduction of intussusceptions (83.9%), pyloromyotomy (90.3%), fundoplication (96.8%), biopsy and corrective surgery of Hirschsprung's disease (93.5%/90.3%), imperforate anus (77.4%), congenital diaphragmatic hernia (80.6%), and esophageal atresia (74.2%). The MIS procedures with more than 70% were lung resection (100%), cholecystectomy (100%), appendectomy (96.2%), ovarian torsion (86.7%), fundoplication (86.8%), hiatal hernia repair (82.6%), and splenectomy (71.4%). The MIS procedures with less than 30% were congenial diaphragmatic hernia reapir (29.6%), esophageal atresia (26.2%), correction of malroatation (24.4%), inguinal hernia repair (11.4%), anorectal malformation (6.8%), Kasai operation (3.6%).