• 제목/요약/키워드: malformation

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Effects of Gamma-Irradiation on the Sterilization of Primordial Germ Cells in Quail (메추리 원시생식세포 감소를 위한 감마선 조사의 효과)

  • Park, Kyung-Je;Kim, Tae-Min;Lee, Hyung-Chul;Jang, Hyun-Jun;Song, Gwon-Hwa;Han, Jae-Yong
    • Korean Journal of Poultry Science
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    • v.37 no.2
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    • pp.139-143
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    • 2010
  • Quail is a very useful animal model for studying vertebrate development because of its small body size and unique reproductive traits. This species is also ideal model for producing germline chimeras via transferring exogenous primordial germ cells (PGCs) into the recipient embryo. To increase the contribution efficiency of donor PGCs into recipients' tissues, decreasing the population of endogenous PGCs has been rate-limiting factor. We therefore conducted this study to investigate if gamma ($\gamma$)-irradiation depletes endogenous PGCs in developing quail embryo. Firstly, freshly laid stage X quail embryos were irradiated with various output of $\gamma$-irradiation and its teratogenic effect on the embryo was evaluated. Although a dose-dependent increase in the number of embryo showing malformation was found as the output increased (0, 250, 500, 750, and 1,000 rads), only a maximum of 10.1% of embryos were abnormal in 1,000 rads. Immunocytochemical analysis using the QCR1 antibody, which is specific marker for quail PGCs, was conducted to analyze the effect of sterilization. As results, $\gamma$-rays at a dose-rate of 500 rads/73 sec onto undeveloped stage X embryo significantly reduced the number of germ cells to an average of 75.55 % and 82.03 % in male and female embryos, respectively. We conclude that $\gamma$-ray selectively targets PGCs while affects minimally to the somatic development in quail embryo. Our results will not only provide important data for germline chimera production but can be used for analyzing the effect of ionized rays on the differentiating germ cells in various stages during animal development.

Bivalvation Valvuloplasty for Common Atrioventricular Valve Regurgitation in Functional Single Ventricle; Early and Mid-term Results (기능적 단심설에서 공통방실판 역류의 판막성형술; 판막 이분성형술(bivalvation)의 조기 및 중기 결과)

  • Chang, Yun-Hee;Sung, Si-Chan;Kim, Seon-Hee;Lee, Hyoung-Doo;Ban, Ji-Eun
    • Journal of Chest Surgery
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    • v.42 no.5
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    • pp.597-603
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    • 2009
  • Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

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%).

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.

Effects of Gestation Length and Birth Weight on Survival Rate in Cloned Korean Native Calves (복제 송아지의 임신 기간과 생시체중이 출생 후 생존율에 미치는 영향)

  • Yang, Byoung-Chul;Im, Gi-Sun;Kim, Dong-Hoon;Ko, Yeoung-Gyu;Hwang, Seong-Soo;Nho, Whan-Gook;Kim, Myung-Jick;Yang, Boh-Suck;Lee, Sang-Jin;Seong, Hwan-Hoo
    • Reproductive and Developmental Biology
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    • v.32 no.1
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    • pp.51-58
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    • 2008
  • This study was performed to investigate the relation between birth weight and survivability on the production of cloned Hanwoo calves. The 580 cloned embryos were transferred into the 293 recipients. The pregnancy rate of the cloned embryos was 72.3% at 50 days after embryo transfer, and then the rate was dramatically decreased. The mean gestation lengths were 287 days in both clone (range of$279{\sim}295$ days) and artificial insemination (AI, range of $255{\sim}293$ days) calves, respectively. The mean birth weight of cloned calves (30.3kg) was significantly higher compared to that of AI calves (23.7kg) (p<0.05). Among the cloned calves, the birth weight was not different in both normal delivery (n=17, 29.9kg) and caesarean section (n=14, 32.3kg). The weight, however, was significantly higher in the clones (n=18, 32.8kg) dead within 175 days than that of the clones (n=11, 28.3kg) alive more than 175 days after birth (p<0.05). Interestingly, all cloned calves weighed <15kg (n=5) or >35kg (n=9) at birth have been dead within 175 days from the date of birth. The causes of death in the cloned calves were premature birth (n=2, 10.0%), abnormal function of lung and liver (n=2, 10.0%), abnormal function of lung (n=4, 20.0%), malformation (n=4, 20.0%), unknown (n=4, 20.0%), and sudden death syndrome (n=4, 20.0%), respectively. Our findings suggest that normal birth weight is one of the most important factors to survive more than 6 months in cloned calves.

Anorectal Malformations In Korea - A Clinical Survey by the Korean Association of Pediatric Surgeons, 1999 - (한국의 항문-직장 기형 -1999년도 대한소아외과학회 회원 대상 전국 조사-)

  • Lee, M.D.;Kim, S.Y.;Kim, W.K.;Kim, I.K.;Kim, J.U.;Kim, J.C.;Kim, H.H.;Park, K.W.;Park, W.H.;Song, Y.T.;Oh, S.M.;Yoo, S.Y.;Lee, D.S.;Lee, S.K.;Lee, S.C.;Chang, S.I.;Chung, S.Y.;Jung, S.E.;Chung, U.S.;Jung, P.M.
    • Advances in pediatric surgery
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    • v.6 no.2
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    • pp.106-123
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    • 2000
  • The purpose of the survey was to clarify the clinical features and management of the anorectal malformations (ARM) in Korea. Twenty-seven members of the Korean Association of Pediatric Surgeons were surveyed. The members completed a case registration form for each patient during the two years period of 1996-1997, and a questionnaire that contained their management preferences for primary and complicated patients. Twenty-four members of the 22 institutions registered 295 cases of the ARM, and 27 out of 34 members (77.1 %) responded to the questionnaire. The patients were classified by the Wingspread classification of ARM(1984). The case registration form was a modification of Wingspread workshop for the postoperative assessment and case registration. The male to female ratio was 1.7 : 1. The average number of patients per surgeon was 6.1 cases per year. Prematurity(< 36 weeks) was present in 1.9 % of cases and low birth weight (< 2.6 Kg) in 12.1 %. Among 187 male patients, 62 were high(H) type, 29 intermediate(l) type, 88 low(L), and 8 unclassified. In female, there were 2 cases of cloacal anomalies(C), 4 H type, 30 I type, 66 L type and 6 unclass ified. For male, 87.9 % of H and I type were operated by the Pena procedure, but only 7.9 % in L types. In female, all of C, H and I types, and 40.9 % of L type were done by the Pena procedure. One case expired (MR; 0.003%) as a result of surgical complication. Over-all complication rate was 12.5 %. Among 6 cases of reoperation, 4 were failed Pena procedures. Among 140 colostomies sigmoid colon was utilized in 75.7%, and loop colostomy was commonly used. Ten surgeons prefered primary maturation of the stoma. In conclusion, posterior sagittal anorectoplasty is popular for high types of ARM in this country, but considerable number of patients are still suffering from failed operations. For better understanding and analysis, necessity of prospective study by new classification was discussed.

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TOOTH MOVEMENTS TO THE SITE OF ALVEOLAR BONE GRAFT (구순구개열 환아에서의 치조골이식)

  • Cho, Hae-Sung;Park, Jae-Hong;Kim, Gwang-Chul;Choi, Seong-Chul;Lee, Keung-Ho;Choi, Yeung-Chul
    • Journal of the korean academy of Pediatric Dentistry
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    • v.34 no.1
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    • pp.140-149
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    • 2007
  • Cleft lip and palate are congenital craniofacial malformation. Reconstruction of dental arch in patient with alveolo-palatal clefts is very important, because they have many problems in functions and esthetics. Malnutrition, poor oral hygiene, respiratory infections, speech malfunctions, maxillofacial deformity, and psychological problems may be occured without proper treatment during the long period of management of the cleft lip and palate. So the treatment should be managed with a multidisciplinary approach. Bone grafting is a consequential step in the dental rehabilitation of the cleft lip and palate patient A complete alveolar arch should be achieyed of the teeth to erupt in and to form a stable dentition. And the presence of the cleft complicate the orthodontic treatment. Therefore bone grafting in patients with cleft lip and palate is a widely adopted surgical procedure. Grafted bone stabilizes the alveolar process and allows the canine or incisor to move into the graft site. After the bone grafting, orthodontic closure of the maxillary arch has become a common practice for achieving dental reconstruction without any prosthodontic treatment. Various grafting materials have been used in alveolar clefts. Iliac bone is most widely fovoured, but tibia, rib, cranial bone, mandible have also been used. And according to its time of occurrence, the bone graft may be divided into primary, early secondary, secondary, late secondary. Bone grafting is called secondary when performed later, at the end of the mixed dentition. It is the most accepted procedure and has become part of treatment of protocol A secondary bone graft is performed preferably before the eruption of the permanent canine in order to provide adequate periodontal support for the eruption and preservation of the teeth adjacent to the cleft. In this report, we report here on a patient with unilateral cleft lip and palate, who underwent iliac bone graft. The cleft was fully obliterated by grafted bone in the region of the alveolar process. The presence of bone permitted physiologic tooth movement and the orthodontic movement of adjacent tooth into the former cleft area. Satisfactory arch alignment could be achieved in by subsequent orthodontic treatment.

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Preliminary Results of Stereotactic Radiosurgery Using Stereotactic Body Frame (정위 체부 고정틀을 이용한 체부 방사선수술의 예비적 결과)

  • Ahn Seung Do;Yi Byong Yong;Choi Eun Kyung;Kim Jong Hoo;Nho Young Ju;Shin Kyung Hwan;Kim Kyoung Ju;Chung Won Kyun;Chang Hyesook
    • Radiation Oncology Journal
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    • v.18 no.4
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    • pp.251-256
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    • 2000
  • Purpose : To evaluate efficacy and complication of stereotactic radiosurgery using stereotactic body frame. Methods and Materials :From December 1997 to June 1999, 11 patients with primary and metastatic tumors were treated with stereotactic radiosurgery using stereotactic body frame(Precision TherapyTu). Three patients were treated with primary hepatoma and seven with metastatic tumor from liver, lung, breast, trachea and one with arteriovenous malformation on neck. We used vacuum pillow for immobilization and made skin marker on sternum and tibia area with chest marker and leg marker. Diaphragm control was used for reducing movement by respiration. CT-simulation and treatment planning were peformed. Set-up error was checked by CT-Simulator before each treatment. Dose were calculated on the 80$\~$90$\%$ isodose of isocenter dose and given consecutive 3 fractions for total dose of 30 Gy (10 Gy/fraction). Results :Median follow-up was 12 months. One patient (9$\%$) showed complete response and four Patients (36$\%$) showed partial response and others showed stable disease. Planning target volumes (PTV) ranged from 3 to 111 cc (mean 18.4 n). Set-up error was within 5 mm in all directions (X, Y, Z axis). There was no complication in all patients. Conclusion :In Primary and metastatic tumors, stereotactic body frame is very safe, accurate and effective treatment modality.

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Clinical Manifestations of 15 Cases of Pulmonary Sequestration (폐격리증 15예의 임상양상에 관한 고찰)

  • Park, Kwang-Joo;Kim, Eun-Sook;Kim, Hyung-Jung;Chang, Joon;Ahn, Chul-Min;Kim, Sung-Kyu;Lee, Won-Young;Kim, Sang-Jin;Lee, Doo-Yun
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.2
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    • pp.401-408
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    • 1997
  • Background : Pulmonary sequestration is a rare congenital malformation, which is manifested by formation of nonfunctioning lung tissue lacking normal communication with the tracheobronchial tree. The preoperative diagnostic rate has been relatively low, and without consideration of pulmonary sequestration, unexpected bleeding from aberrant vessels may be a serious problem during the operation. The purpose of our study is to describe the clinical features of pulmonary sequestration based on a review of 15 cases treated by operation. Method : Fifteen patients with pulmonary sequestration who had undergone surgical treatment from 1991 through May 1996 at Yongdong Severance Hospital and Severance Hospital were reviewed retrospectively. Results : The mean age of the patients was 22.5 years (range 5~57), and male to female ratio was 9 : 6. Clinical presentations varied from recurrent respiratory infections such as fever, cough, and sputum or chest pain to no symptom. The chest simple X-rays showed multicystic shadow(10/15) and solid mass-like shadow(5/15). The chest CT scans, done in twelve cases, showed multicystic lesion with or without lung infiltration(8/12), solid mass-like lesion(4/12), The chest MRIs, done in three cases, revealed the aberrant arteries originating from descending aorta(2/3). Aortograms, done in four cases, showed the aberrant arteries originating from descending thoracic aorta(2/4), abdominal aorta(I/4), and intercostal artery(1/4). and the venous returns were via the pulmonary veins. Pulmonary sequestration was considered preoperatively in six patients of fifteen. Other preliminary diagnosis were lung tumor(3/15), lung abscess(21/15), bronchiectasis(2/15), and mediastinal tumor(2/15). In the operative findings, twelve cases were of intralobar type and three cases of extralobar type. The left lower lobe was most often affected(9/15) and one extralobar sequestration was in the pericardium. The aberrant arteries originated from descending thoracic aorta(6/15), abdominal aorta(1/15), internal thoracic arteries (2/15), intercostal artery(1/15), pericardiophrenic artery(1/15), but in four cases, the origins could not be defined. There was no mortality or complication postoperatively. Conclusion : In our study, preoperative diagnostic rate was relatively low, and clinical features were similar to previous reports. Preoperative vigorous diagnostic approach including aortography is strongly advocated not only for its diagnostic value, but also for accurate localization of the aberrant vessels, which is major concern to surgical procedure.

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Differentiation of Medial or Lateral Temporal Lobe Epilepsy by F-18-Fluorodeoxyglucose Positron Emission Tomography: Comparative Study with Magnetic Resonance Imaging in 113 Surgically and Pathologically Proven Patients (F-18-Fluorodeoxyglucose 양전자단층촬영을 이용한 내외측 측두엽간질의 감별: 수술과 병리 소견으로 확진한 113예에서 자기공명영상과 비교 분석)

  • Lee, Dong-Soo;Lee, Sang-Kun;Chang, Ki-Hyun;Chung, Chun-Kee;Choi, Ki-Young;Chung, June-Key;Lee, Myung-Chul
    • The Korean Journal of Nuclear Medicine
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    • v.33 no.2
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    • pp.111-119
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    • 1999
  • Purpose: As mesial temporal lobe epilepsy (TLE) shows hypometabolism of medial and lateral temporal lobe, we investigated whether symmetric uptake of F-18-FDG in medial temporal lobes can differentiate mesial from lateral TLE. Materials and Methods: In 113 patients (83 mesial TLE, 30 lateral TLE) who underwent anterior temporal lobectomy and/or corticectomy with good surgical outcome, we performed F-18-FDG PET and compared F-18-FDG uptake of medial and lateral temporal lobes. All the patients with mesial TLE had hippocampal sclerosis except one congenital abnormal hippocampus. Patients with lateral TLE revealed cerebromalacia, microdysgenesis, arteriovenous malformation, old contusion, and cortical dysplasia. Results: Sensitivity of F-18-FDG PET and MR for mesial TLE was 84% (70/83) and 73% (61/83), respectively. Sensitivity of F-18-FDG PET and MR for lateral TLE was 90% (27/30) and 66% (20/30), respectively. Twelve patients were normal on F-18-FDG PET. 101 patients had hypometabotism of lateral temporal lobe. Among 88 patients who showed hypometabolism of medial temporal lobe as well as lateral temporal lobe, 70 were mesial TLE patients and 18 were lateral TLE on pathologic examination. Positive predictive value of medial temporal hypometabolism for mesial TLE was 80%. Among 13 patients who showed hypometabolism of only lateral temporal lobe, 4 were mesial TLE and 9 were lateral TLE. Positive predictive value of hypometabolism of lateral temporal lobe for the diagnosis of lateral TLE was 69% (9/13). Normal MR findings stood against medial TLE, whose negative predictive value was 66%. Conclusion: Lateral temporal lobe epilepsy should be suspected when there is decreased F-18-FDG uptake in lateral temporal lobe with normal uptake in medial temporal lobe.

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