• Journal of Chest Surgery
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• v.45 no.5
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• pp.308-315
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• 2012

Background: Long-term surveillance comparing satisfaction between the early experience of Nuss procedure vs. Ravitch procedure. Materials and Methods: A total of 100 patients that underwent surgical correction of a pectus excavatum between 2001 and 2004 and were followed for ${\geq}2$ years were included. Surveillance on the degree of satisfaction was performed using five-levels of the Likert scale and self-assessment scoring. Results: Nuss or Ravitch surgery was performed in 63 and 37 patients, respectively. The Nuss procedure required a shorter operation time and shorter hospital stay than the Ravitch procedure (p<0.001). The surveillance demonstrated that 17.6% of the Nuss group and 35.7% of the Ravitch group were not satisfied with the outcome of the surgery (p=0.072). The most common causes of dissatisfaction were redepression in the Nuss group (n=5) and incomplete correction in the Ravitch group (n=7). The multivariate analysis showed that reoperation and a high postoperative pectus index were significant risk factors for a low satisfaction score. Conclusion: The Nuss procedure had several advantages over the Ravitch procedure in the immediate postoperative period. However, the long-term satisfaction was determined by a complete correction without recurrence or need for re-intervention rather than by the operation type.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.524-529
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• 2004

Background: Since the Nuss procedure for the correction of pectus excavatum is in its early stage, there have been problems that need to be solved. We examined complications in a single-institute experience of the Nuss technique in order to develop possible solutions to prevent them. Material and Method: 335 consecutive patients, who underwent the modified Nuss procedure between August 1999 and October 2002, were studied retrospectively. Median age was 8 years (range 1 to 46). 264 patients (78.8%) were in pediatric group (age$\leq$15) and 71 patients (21.2%) were in adult group (age> 15). 193 patients (57.6%) had symmetric and 142 patients (42.4%) had asymmetric pectus configurations. Risk factors predicting postoperative complications were analyzed using multi-variate logistic regression. Result: Postoperative complication rates were 18.9% (61/335) in total patients. Frequent complications were pneumothorax 24 (7.5%), bar displacement 11 (3.4%), and wound seroma 10 (3.1%) in order. Early complications (within a month, 49 cases, 15.2%) were pneumothorax (n=23, 6.9%), wound seroma (n=12, 3.6%), and bar displacement (n=8, 2.4%). Late complications (after a month, 12 cases, 3.7%) were pericarditis and pericardial effusion (n=5, 1.5%), bar displacement (n=4, 1.2%), and hemothorax (n=3, 0.9%). Techniques were modified to prevent complications especially in bar shaping and fixation, which led to decrease complication rate in later experience (Operation Date 1: 15/51 (29.4%) vs Operation Date 2: 34/284 (12.0%), p=0.004). Grand Canyon type (eccentric long canal type) showed higher complication rate than other types (GC type: 12/30(40%) vs Others: 37/305 (12.1%), p<0.001). Major risk factors are severity of pectus (OR=2.88, p=0.038), Grand Canyon type (OR=2.82, p=0.044), and Op. Date 1 (OR=4.05, p=0.001). Conclusion: Major complications were related to severe eccentric type of pectus configuration (Grand Canyon type) and lack of surgeon's experience (Op. Date 1). Com-plication rate was reduced with accumulation of experience and advancement of surgical techniques. The Nuss procedure can be performed at a low risk of complications with our current technique.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1247-1250
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• 1997

A 13-year-old boy presented with anterior chest wall depression and dyspnea on exertion(NYHA II). He underwent Ravitch operation for pectus excavatum 7 years ago. A preoperative echocardiographic study revealed secundum atrial septal defect. He had no other abnormality of laboratory test, except FVC and FEVI were decreased into 2.03 L(7 %) and 1.82 L(71 %). He underwent repair of secondary anterior chest wall deformity and secundum atrial septal defect. We used unique method, raising sternum at right angle to secure good operative field for open heart surgery. Acute respiratory insufficiency was developed on postoperative day 1. Mechanical ventilation was applied which could be weaned on postoperative day 6 and thereafter hospital course was uneventful without any other sequale. He was discharged on postoperative day 19.

• Journal of Chest Surgery
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• v.39 no.11 s.268
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• pp.868-871
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• 2006

Nuss operation as a method for correction of pectus excavatum is safe and satisfactory, but its complication presents pneumothorax, displaced bar, wound infection, pericarditis, pleural effusion, hemothorax, cardiac injury etc. We report a rare case of acute ascending aortic rupture after displaced and adhered Nuss bar removal. The patient was a 30-years old man who received Nuss operation 3 years ago. Nuss bar was removed without resistance but massively bled at both operation wound, so immediately femoro-femoral CPB and median sternotomy was done and repaired proximal aortic arch under deep hypothermic total circulatory arrest. The patient was discharged without complication at postoperative 13 day.

• Journal of Genetic Medicine
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• v.17 no.1
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• pp.43-46
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• 2020

The Shprintzen-Goldberg syndrome (SGS) is an extremely rare genetic disorder caused by heterozygous variant in SKI. SGS is characterized by neurodevelopmental impairment with skeletal anomaly. Recognition of SGS is sometimes quite challenging in practice because it has diverse clinical features involving skeletal, neurological, and cardiovascular system. Here we report a case of a 6-month-old boy who initially presented with developmental delay and marfanoid facial features including prominent forehead, hypertelorism, high arched palate and retrognathia. He showed motor developmental delay since birth and could not control his head at the time of first evaluation. His height was above 2 standard deviation score. Arachnodactyly, hypermobility of joints, skin laxity, and pectus excavatum were also noted. Sequencing for FBN1 was negative, however, a novel missense variant, c.350G>A in SKI was identified by sequential whole exome sequencing. To our knowledge, this is the first case with SGS with phenotypic features of SGS overlapping with those of the Marfan syndrome, diagnosed by next generation sequencing in Korea.