• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.568-574
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• 2009

Tongue-tie is a congenital anomaly characterized by an abnormally short lingual frenum, which may restrict tongue tip mobility, In the neonate and infant, tongue-tie was said to cause difficult breast-feeding, In the older infant, toddler and young child, poor speech has been frequently listed because movement of tongue tip is limit ed. Also as a abnormal tongue position, various orthodontic problems, later mechanical and social manifestations could be developed. Treatment options such as observation, speech therapy, frenotomy and frenectomy have been suggested. The optimal timing for the surgery has not been determined, but early intervention may be appropriate for the children with significant tongue-tie who has the significant potential to speech difficulties and later social and mechanical problems. Sedation is an effective method for incapable of cooperative and the handicapped children, necessary to early intervention. We report three cases, using oral sedation for the frenectomy in young children with severe tongue-tie. After treatments, we could find out sufficient tongue movement and improved speech ability.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.569-577
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• 1986

Between October 1979 and June 1986, modified Fontan procedures have been performed on 22 patients by the Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine. Twelve patients had tricuspid atresia; one, congenital tricuspid stenosis; five, single ventricle; two, I-transposition of the great vessels; one, double outlet right ventricle, and one, pulmonary atresia with an intact ventricular septum. There were 9 operative deaths [mortality; 40.9%]. The causes of death were right heart failure in six patients and pulmonary venous hypertension in one who misdiagnosed preoperatively. Another two were deceased due to sepsis and cerebrovascular accident at postoperative 35 and 34 days in each. There were 7 patients below 4 years of age at the time of operation and among them 4 patients were deceased. The operative death was not related with patients` age above and below 4[p=0.211]. The relation between operative death of tricuspid anomaly and another cardiac malformations was statistically significant [p=0.048]. The operative procedures with or without valved conduit [woven dacron] was related significantly [p=0.043] in the case of the 21 of the patients, but the modified Fontan operation with a valved conduit was performed early stage in this series. Since 1982, we operated on 4 patients, doing a right atrium-right ventricle anastomosis without a conduit. All survived and remained in functional class I [NYHA]. The right atrial pressure [RAP] was elevated significantly after operation [mean 9.9$\pm$4.8 ~16.9$\pm$3.6 mmHg, p<0.001]. The relation between the postoperative RAP of the survival group [16.5$\pm$4.3 mmHg] and the group who died [17.4$\pm$2.2 mmHg] was statistically significant [p=0.047]. There was no relation between any operative death and any previous palliation. All patients were followed for 4 months to 80 months, except one who was lost to follow up at 2 months following surgery [mean 11.4 months, 238 patient. months]. All were in functional class I with 5 on medications and 7 not. One was reoperated at 70 months following the first operation, due to conduit stenosis. She was moderately impaired in activity, with hepatomegaly after the second operation.

• Journal of Chest Surgery
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• v.21 no.2
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• pp.211-222
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• 1988

Tetralogy of Fallot is a cyanotic congenital heart disease characterized by large ventricular septal defect[VSD] and stenosis of right ventricular outflow tract[RVOT] and the degree of RVOT stenosis and the state of pulmonary arteries are the major determinant of prognosis of this anomaly after operation. The sum of blood flow through RVOT and collateral flow from systemic arteries determine the total pulmonary blood flow and it is drained to left atrium and left ventricle. Therefore the degree of development of left ventricle not only reflects pulmonary blood flow and the status of peripheral pulmonary arteries but also affects postoperative prognosis as a systemic ventricle. In this article, left ventricular volume and its influence on postoperative cardiac function in tetralogy of Fallot were studied in 34 patients operated on at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital in 1985. Mean age of the patients was 5 1/12*3 9/12 years[range 9/12 - 14 8/12 years], mean body surface area[BSA] 0.65*0.20m2[range 0.38 - 1.22m2], mean body weight 15.6k6.48kg[range 7.0 - 36kg]and mean hematocrit 50.6*9.77%[range 32.0 - 73.5%]. Left ventricular end-diastolic volume[LVEDV] of them were from 11.2 to 113cc and there was a good linear correlation between BSA[m2, X]and LVEDV[cc, Y][Y= - 20.0+923x, r= 0.84, p < 0.005]. Mean LVEDV/m2 was [57.6 * 18.3 cc / m2[range 28.7 - 95.8 cc / m2] and there was a significant reduction of volume compared with normal value. As body surface increases, there was a increasing tendency in LVEDV/m2 but there was no statistical significance. Mean total amount of postoperatively infused dopamine in these 33 patients[except one who expired postoperatively] was 65.6*74.5mg / kg and it was 40.6*44.0mg / kg in routine RVOT widening group [Group I] and 205*49.3mg / kg in transannular RVOT widening group[Group II]. There was a statistically significant difference between two groups. In group I patients there was a good linear inverse correlation between dopamine total amount[mg / kg, Y] and LV volume[cc / m2, X] [Y = 150 - 1.89 X, r = - 0. 77, p < 0.005]. But there were no correlations between dopamine total amount and Hct, cardiopulmonary bypass time and aorta cross clamp time. In conclusion, the patient with small preoperative left ventricular volume required more amount of dopamine as an inotropic agent for the maintenance of a cardiac function in postoperative period. But this is a result of immediate postoperative period and does not reflect the long term effect of left ventricular volume in tetralogy of Fallot. There must be more study for the evaluation of its long term effect.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• 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.

• Advances in pediatric surgery
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• v.16 no.2
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• pp.126-133
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• 2010

Malrotation is a congenital anomaly that becomes symptomatic more frequently during infancy. The indication for surgical treatment at that age is straightforward. In older children, the diagnosis may be more difficult because of chronic and vague complaint. The aim of this study is to compare the symptoms, rate of volvulus and surgical findings in children younger and older than one year. A retrospective study of 40 patients in a a single medical center diagnosed with malrotation from April 1996 to May 2010 was performed. There were 20 (50 %) boys and 20 (50 %) girls. At the time of operation, 27 (67.5 %) patients were younger and 13 (32.5 %) were older than 1 year. Vomiting was seen in 20 cases (74.1 %) of the younger group compared to 2 cases (15.4 %) of the older group. Abdominal sonography and upper gastrointestinal series showed a sensitivity of 100%. Operative findings: 12 (44.4 %) of the younger group presented with volvulus compared to none of the older group. The Ladd's procedure was routinely performed with appendectomy in all cases and bowel resection was requires when volvulus included bowel necrosis or other anomalies were found. After definite procedures, surgical correction for adhesive obstruction was necessary in 5 menbers (18.5 %) of the younger group and 1 patient (7.7 %) in the older group. There was 1 death due to respiratory failure and pneumonia. Abdominal pain was more frequent symptom and bilious vomiting was less frequent. Volvulus did not occur in the older group. Malrotation should be diagnosed promptly in children over 1 year of age by upper gastrointestinal series and abdominal ultrasonography even though symptoms are not as clear cut as in infants.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.127-134
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• 2007

Meckel's diverticulum is the most common congenital anomaly of gastrointestinal tract in children. The incidence of complicated Meckel's diverticulum is about 4 %. The major complications of Meckel's diverticulum are bleeding, intussusception, obstruction and perforation. The aim of this study was to investigate the clinical manifestations and the role of laparoscopic surgery in complicated Meckel's diverticulum in children. We retrospectively reviewed the medical records of 19 patients with complicated Meckel's diverticulum who underwent operation at Asan Medical Center between Jan. 1990 and Apr. 2007. Male to female ratio was 11:8, and median age was 1 year (1 day-13 years). The most frequent symptom was hematochezia (68%), followed by irritability or abdominal pain (16%), vomiting (11%), and abdominal distension (5%). Two operative procedures were performed; small bowel resection with anastomosis (68%) and diverticulectomy (32%). The operation proven complications of the Meckel's diverticulum were bleeding (68%), intussusception (16%), perforation (11%) and obstruction (5%). Ectopic tissues found by postoperative pathologic examination were gastric (84%) and pancreatic (11%). Hospital stay after laparoscopic operation for bleeding Meckel's was 5 days (median) and average first postoperative feeding was 1.5 days. On the contrary, hospital stay for open surgery was 7 days and first feed was 3 days. In summary, the most common compliation of Meckel's diverticulum in children was bleeding and ectopic gastric tissues were present in 84%. Laparoscopic procedure seemed to be useful for diagnosis as well as for definitive treatment.

• Archives of Plastic Surgery
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• v.34 no.1
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• pp.52-59
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• 2007

Purpose: The venous flap is used as an alternative method to the standard free flap for the management of small and thin soft tissue defects. Especially, the venous flap has advantages of being thin, easy harvest and various donor sites, as well as it having lower morbidity. Yet their clinical applications have been limited by their unstable postoperative course and also by their complications such as partial necrosis. The aim of this study is to extend the clinical indications of venous flaps with using various methods. Methods: From May 2005 to March 2006, total of 19 patients(21 cases) underwent various venous flaps for soft tissue defects as a result of trauma(15 cases), facial skin cancer(3 cases), chronic ulcer(1 case) and surgical wound for congenital anomaly(2 cases). The arterialized venous free flap were applied in 18 cases, the pure venous free flap was applied in 1 case and the pure venous pedicled flap were applied in 2 cases. Among them, two flow-through arterialized venous free flaps were applied that used the great saphenous vein to reconstructed major arteries as well as the injured skin and soft tissues in the arm. All the flap were harvested from the volar wrist(11 cases), dorsum of foot(5 cases), thenar(2 cases), and medial thigh(3 cases). Results: The sizes of the flap ranged from $0.75cm^2$ to $264cm^2$(mean size: $40.06cm^2$). The follow-up period ranged from two to twelve months. In the majority of cases, we obtained satisfying results, which was the excellent reconstruction of skin and soft tissue defects and especially in the case of limb salvage, replantation and cancer reconstruction. However, there were 5 cases of partial necrosis and 2 cases of complete failure. The donor sites were closed primarily in 7 cases and wound closure with skin graft were in 14 cases. Conclusion: We conclude that the venous flap will not only be useful for reconstruction of small defect in the hand and foot, but also be useful for various other clinical indications.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.71-78
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• 2010

Purpose : Hydronephrosis is found about 30% of children with urinary tract infection (UTI). It can be caused by various conditions, although most childhood hydronephrosis is congenital. This study was performed to investigate the relationship between febrile UTI and hydronephrosis. Methods : We retrospectively reviewed the medical charts of 183 patients diagnosed as UTI between January 2007 and May 2009 at Korea University Guro Hospital. Inclusion criteria were as followings; 1) fever more than $37.5^{\circ}C$ measured in the axilla, 2) positive urine culture, 3) no history of urinary tract anomaly on antenatal sonography and urinary tract infection. We classified the enrolled children into two groups of patients with hydronephrosis (HN) and those without hydronephrosis (NHN). Results : The 80 patients were HN and 103 patients NHN. Hydronephrosis was found in 58 patients with left kidney, 8 right and 14 both kidneys. Most of hydronephrosis were of low grade. Compared with NHN group, initial renal cortical defects on DMSA scan significantly increased in HN group (HN 37.5%, NHN 16.5%, P<0.05). The incidence of VUR was not different between the two groups (HN 22%, NHN 12.1%). White blood cell counts and C-reactive protein were not different between the two groups. Follow-up DMSA scan (about 6 months later after UTI) showed no difference of renal scarring in both two groups. Conclusion : Our data suggests that hydronephrosis in febrile UTI patients is clinically useful for detecting renal cortical defects, but is not associated with follow-up renal scar.