Objective To describe our experience in the treatment of pectus excavatum(PE)using a modifed stemal elevation procedure.Methods From Oct.1986 to Dec.1997,171 patients with PE were admitted to the Department of Pediatr...Objective To describe our experience in the treatment of pectus excavatum(PE)using a modifed stemal elevation procedure.Methods From Oct.1986 to Dec.1997,171 patients with PE were admitted to the Department of Pediatric Surgery of the First Hospital of West China University of Medical Sciences.All patients were diagnosed through a history and physical examination.Cardiopulmonary function was assessed by M-mode echocardicgraphy and instrument of pulmonary function in 40 patients before and 4.2 years after surgery which was performed between 1989 and 1994.We performed the following three procedures in the stemal elevation:(1)forming the metal strut in a“arch”shape,(2)suturing the perichondrium into a“pipe”shape,and(3)encouraging patients to do chest expansion exercise after operation.All patients were followed up for 1 to 12 years.Results The normal contour of the costal cage was enlarged in all but one patient.Exercise tolerance was improved,and cardiac function recovered to the same level as in healthy children,while pulmonary function recovered very slowly after surgery.Conclusion The normal appearance of chest wall can be recovered and normal cardiopulmonary function can be restored by the modified stemal elevation procedure in children with PE展开更多
Introduction: The main indication for surgery of thoracic wall deformities (TWD) is psychological due to cosmetic complaints. The assumption is that appearances have a negative effect on?self-esteem and quality of lif...Introduction: The main indication for surgery of thoracic wall deformities (TWD) is psychological due to cosmetic complaints. The assumption is that appearances have a negative effect on?self-esteem and quality of life?(QoL). Correction should result in improvement. Methods: Prospective trial. QoL was assessed using the?CHQ and the WHOQOL-bref. Measurements were taken before surgery (T1) and 6 weeks thereafter (T2). Results: Forty-two patients were included.?WHOQOL-bref showed differences between pre-operative and?six weeks past surgery on facet body image (p = 0.003).?Self-esteem (CHQ) did not show a significant improvement at T2.?Concerning the scores on the single step questionnaire (SSQ),?33 patients were “very” to “extremely satisfied” with appearance and increased self-esteem (p < 0.001). Concerning the domain “pain and physical complaints”, CHQ did show a significant change (p < 0.001) with more complaints at T2. Conclusion: Six weeks after surgical correction of a TWD satisfaction with the “new” chest is good;pain seems to be a problem with possible negative influence on self-esteem.展开更多
Pectus excavatum (PE) and pectus carinatum (PC) are relatively common deformities involving the anterior chest wall, occurring in 1:1000 and 1:1500 live births, respectively. While the etiology remains an enigma, the ...Pectus excavatum (PE) and pectus carinatum (PC) are relatively common deformities involving the anterior chest wall, occurring in 1:1000 and 1:1500 live births, respectively. While the etiology remains an enigma, the association of pectus deformities with other skeletal abnormalities suggests that connective tissue disease may play a role in their pathogenesis. Clinical features of these deformities vary with severity, as determined by the Haller index and Backer ratio, but frequently include cardiac and respiratory abnormalities. Importantly, there exist profound psychosocial implications for children afflicted with these defofrmities, including but not limited to feelings of embarrassment and maladaptive social behaviors. These debilitating characteristics have prompted the development of novel medical and surgical corrective techniques. The correction of pectus deformities reduces the incidence of physiological complications secondary to chest wall malformation, while simultaneously improving body image and psychosocial development in the affected pediatric population. The Ravitch (open) and Nuss (minimally invasive) procedures remain the most frequently employed methods of pectus deformity repair, with no difference in overall complication rates, though individual complication rates vary with treatment. The Nuss procedure is associated with a higher rate of recurrence due to bar migration, hemothorax, and pneumothorax. Postoperative pain management is markedly more difficult in patients who have undergone Nuss repair. Patients undergoing the Ravitch procedure require less postoperative analgesia, but have longer operation times and a larger surgical scar. The cosmetic results of the Nuss procedure and its minimally invasive nature make it preferable to the Ravitch repair. Newer treatment modalities, including the vacuum bell, magnetic mini-mover procedure (3MP), and dynamic compression bracing (DCB) appear promising, and may ultimately provide effective methods of noninvasive repair. However, these modalities展开更多
Objective To assess whether and to what extent pulmonary function returns to normal after surgical correction for pectus excavatum Methods Twenty seven patients who could be examined in person at the outpatient de...Objective To assess whether and to what extent pulmonary function returns to normal after surgical correction for pectus excavatum Methods Twenty seven patients who could be examined in person at the outpatient department of our hospital were included in this study Of these patients, 24 were boys and 3 were girls, with age ranging from 3 to 16 years (mean: 8 67 years) The mean age at surgery was 4 years and mean years at follow up was 6 8 Pulmonary function measurements included inspiratory vital capacity (IVC), total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC), RV/TLC ratio, maximal voluntary ventilation (MVV), forced ventilatory capacity (FVC), forced expiratory volume in one second (FEV 1), maximal mid expiratory flow (MMEF), maximal expiratory flow at 75% vital capacity (V 75 ), maximal expiratory flow at 50% vital capacity (V 50 ), maximal expiratory flow at 25% vital capacity (V 25 ) and breathing reserve ratio (BR) Results TLC, FRC, MVV, MMEF, V 75 and V 50 were not different from normal values IVC, FVC, FEV 1 and V 25 were significantly decreased compared with normal values RV and RV/TLC were high in 87 5% cases Conclusions Preoperative symptoms improved substantially after operation Little airway obstruction was observed postoperatively, suggesting that patients with pectus excavatum should have surgery as early in life as possible, preferably by age 3展开更多
Background: Adolescents with pectus excavatum (PE) are often affected by their body shape. The aim of our approach was to quantify the patients’ individual psychological distress and to create a psychological indicat...Background: Adolescents with pectus excavatum (PE) are often affected by their body shape. The aim of our approach was to quantify the patients’ individual psychological distress and to create a psychological indication for treatment. Methods: 10 adolescents (8 male, median age 16 years, interquartile range 15 - 17 yrs.) with PE were examined at our psychological department. Using standardized psychological tests, projective tests and interviews psychologists validated the patients’ individual psychological status. All patients were offered psychological therapy and correction of the deformity. In addition, the children were followed-up by a telephone questionnaire (median follow-up after starting therapy 12.8 months (5.9 - 18.0). Results: No patient had a relevant physiological limitation. The median follow-up since presentation to our psychologists was 15.0 months (9.1 - 20.6). 8 patients (5 were operated, 2 used the vacuum bell, 1 will undergo surgery) had distinct psychological limitations especially concerning the dimensions attractiveness, self-esteem and somatisation. They demonstrated increased insecurity, anxiety and denegation of their body. Since all patients were within puberty the psychological distress due to the PE has to be interpreted as disadvantageous for their further development. 7 patients completed the follow-up questionnaire and reached a median score of 80.8% (76.4% - 86.8%), which indicates a good improvement in all patients. Conclusions: We conclude that the psychological indication for treatment is justified, since our results support this indication.展开更多
Background Thoracoscopic minimally invasive pectus excavatum repair （Nuss operation） features its little trauma, simple, short operation time, and good outcome compared with traditional treatment of pectus excavatum...Background Thoracoscopic minimally invasive pectus excavatum repair （Nuss operation） features its little trauma, simple, short operation time, and good outcome compared with traditional treatment of pectus excavatum surgery-sternal elevation （Ravitch operation） and sternal turnover. The effect of the operation on patients＇ heart and heart function remains unclear. This study aimed to understand the changes of electrocardiogram and cardiac function after Nuss procedure. Methods From 2008 January to 2013 July, thoracoscopic Nuss operation was performed in 217 patients with pectus excavatum. All the patients underwent the preoperative, postoperative detection of ECG and cardiac function in 3 months to 1 year after operation. Results After 3 months to 1 year follow-up, arrhythmias persisted in 46 out of 135 patients with preoperative symptoms （P 〈 0.05）; Stroke volume and cardiac output significantly increased （P 〈 0.05）; And cardiac parameters greatly improved （P 〈 0.05）. Conclusions Minimally invasive repair of pectus excavatum deformity can correct the chest malformation, alleviate arrhythmia, and improve cardiac function.展开更多
Background After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair fo...Background After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes. Data sources Literature was reviewed on PubMed with the terms 'pectus carinatum', 'minimal access repair', 'thoracos-copy' and 'children'. Results Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15.46 years met the inclusion criteria. Success rate of corrections wasn = 125, about 89% in cumulative reports, with seven articles reporting 100%. The complication rate was 39.28%. Since the pectus bar is placed over the sternum and has a large contact area, skin irritation was the most frequent morbidity (n = 20, 14.28%). However, within the complication group (n = 55), wire breakage (n = 21, 38.18%) and bar displacement (n = 10, 18.18%) were the most frequent complications. Twenty-two (15.71%) patients required a second procedure. Recurrences have been reported in four of twelve techniques. There were no lethal outcomes. Conclusions MARPC techniques are not standardized, as MIRPE are, so comparative analysis is difficult as the only common denominator is minimal access. Surgical morbidity is high in MARPC and affects > 2/3rd patients with about 15% requiring surgery for complication management.展开更多
Background:Brachydactyly,a developmental disorder,refers to shortening of hands/feet due to small or missing metacarpals/ metatarsals and/or phalanges.Isolated brachydactyly type E (BDE),characterized by shortened met...Background:Brachydactyly,a developmental disorder,refers to shortening of hands/feet due to small or missing metacarpals/ metatarsals and/or phalanges.Isolated brachydactyly type E (BDE),characterized by shortened metacarpals and/or metatarsals,consists in a small proportion of patients with Homeobox D13 (HOXD13) or parathyroid-hormone-like hormone (PTHLH) mutations.BDE is often accompanied by other anomalies that are parts of many congenital syndromes.In this study,we investigated a Chinese family presented with BDE combined with pectus carinatum and short stature.Methods:A four-generation Chinese family was recruited in June 2016.After informed consent was obtained,venous blood was collected,and genomic DNA was extracted by standard procedures.Whole-exome sequencing was performed to screen pathogenic mutation,array comparative genomic hybridization (Array-CGH) analysis was used to analyze copy number variations,and quantitative real-time polymerase chain reaction (PCR),stride over breakpoint PCR (gap-PCR),and Sanger sequencing were performed to confirm the candidate variation.Results:A 3.06-Mb deletion (chr12:25473650-28536747) was identified and segregated with the phenotype in this family.The deletion region encompasses 23 annotated genes,one of which is PTHLH which has been reported to be causative to the BDE.PTHLH is an important regulator of endochondral bone development.The affected individuals showed bilateral,severe,and generalized brachydactyly with short stature,pectus carinatum,and prematurely fusion of epiphyses.The feature of pectus carinatum has not been described in the PTHLH-related BDE patients previously.Conclusions:The haploinsufficiency of PTHLH might be responsible for the disease in this family.This study has expanded the knowledge on the phenotypic presentation of PTHLH variation.展开更多
Background:Pectus excavatum is the most common congenital chest wall deformity,with a high incidence in live births.This study aimed to evaluate the measured factors on CT images related to the number of pectus bars f...Background:Pectus excavatum is the most common congenital chest wall deformity,with a high incidence in live births.This study aimed to evaluate the measured factors on CT images related to the number of pectus bars for surgical correction.Methods:A total of 497 patients who had undergone minimally invasive repair between April 2007 and July 2011 were classified into single-bar(n=358)and doublebar(n=139)insertion groups.We measured eight distinct distances and one angle on CT scans to reflect quantitative assessment.Univariate analysis and multivariate logistic regression analysis were performed to detect statistically significant association between radiologic measurements and the pectus bars required.Results:After adjusting for age and gender,the transverse distance(T),the transverse distance of the depression area(A),the inclined distance of the depression area(B),the AP distance of the depression area(C),the depression angle(G),and the eccentric distance of deformity(E)were significantly correlated with double-bar insertion.The regression model showed that age(,P<0.0001),gender(P<0.0001),depression angle(G)(P<0.0001),direction of the depression(DD)(P<O.OOO1)and depression depth(D)(P<0.0001)were significantly associated with doublebar insertion.Conclusion:CT scan provides useful factors which can be of assistance in predicting the number of pectus bars for the surgical correction of pectus excavatum.展开更多
文摘Objective To describe our experience in the treatment of pectus excavatum(PE)using a modifed stemal elevation procedure.Methods From Oct.1986 to Dec.1997,171 patients with PE were admitted to the Department of Pediatric Surgery of the First Hospital of West China University of Medical Sciences.All patients were diagnosed through a history and physical examination.Cardiopulmonary function was assessed by M-mode echocardicgraphy and instrument of pulmonary function in 40 patients before and 4.2 years after surgery which was performed between 1989 and 1994.We performed the following three procedures in the stemal elevation:(1)forming the metal strut in a“arch”shape,(2)suturing the perichondrium into a“pipe”shape,and(3)encouraging patients to do chest expansion exercise after operation.All patients were followed up for 1 to 12 years.Results The normal contour of the costal cage was enlarged in all but one patient.Exercise tolerance was improved,and cardiac function recovered to the same level as in healthy children,while pulmonary function recovered very slowly after surgery.Conclusion The normal appearance of chest wall can be recovered and normal cardiopulmonary function can be restored by the modified stemal elevation procedure in children with PE
文摘Introduction: The main indication for surgery of thoracic wall deformities (TWD) is psychological due to cosmetic complaints. The assumption is that appearances have a negative effect on?self-esteem and quality of life?(QoL). Correction should result in improvement. Methods: Prospective trial. QoL was assessed using the?CHQ and the WHOQOL-bref. Measurements were taken before surgery (T1) and 6 weeks thereafter (T2). Results: Forty-two patients were included.?WHOQOL-bref showed differences between pre-operative and?six weeks past surgery on facet body image (p = 0.003).?Self-esteem (CHQ) did not show a significant improvement at T2.?Concerning the scores on the single step questionnaire (SSQ),?33 patients were “very” to “extremely satisfied” with appearance and increased self-esteem (p < 0.001). Concerning the domain “pain and physical complaints”, CHQ did show a significant change (p < 0.001) with more complaints at T2. Conclusion: Six weeks after surgical correction of a TWD satisfaction with the “new” chest is good;pain seems to be a problem with possible negative influence on self-esteem.
文摘Pectus excavatum (PE) and pectus carinatum (PC) are relatively common deformities involving the anterior chest wall, occurring in 1:1000 and 1:1500 live births, respectively. While the etiology remains an enigma, the association of pectus deformities with other skeletal abnormalities suggests that connective tissue disease may play a role in their pathogenesis. Clinical features of these deformities vary with severity, as determined by the Haller index and Backer ratio, but frequently include cardiac and respiratory abnormalities. Importantly, there exist profound psychosocial implications for children afflicted with these defofrmities, including but not limited to feelings of embarrassment and maladaptive social behaviors. These debilitating characteristics have prompted the development of novel medical and surgical corrective techniques. The correction of pectus deformities reduces the incidence of physiological complications secondary to chest wall malformation, while simultaneously improving body image and psychosocial development in the affected pediatric population. The Ravitch (open) and Nuss (minimally invasive) procedures remain the most frequently employed methods of pectus deformity repair, with no difference in overall complication rates, though individual complication rates vary with treatment. The Nuss procedure is associated with a higher rate of recurrence due to bar migration, hemothorax, and pneumothorax. Postoperative pain management is markedly more difficult in patients who have undergone Nuss repair. Patients undergoing the Ravitch procedure require less postoperative analgesia, but have longer operation times and a larger surgical scar. The cosmetic results of the Nuss procedure and its minimally invasive nature make it preferable to the Ravitch repair. Newer treatment modalities, including the vacuum bell, magnetic mini-mover procedure (3MP), and dynamic compression bracing (DCB) appear promising, and may ultimately provide effective methods of noninvasive repair. However, these modalities
文摘Objective To assess whether and to what extent pulmonary function returns to normal after surgical correction for pectus excavatum Methods Twenty seven patients who could be examined in person at the outpatient department of our hospital were included in this study Of these patients, 24 were boys and 3 were girls, with age ranging from 3 to 16 years (mean: 8 67 years) The mean age at surgery was 4 years and mean years at follow up was 6 8 Pulmonary function measurements included inspiratory vital capacity (IVC), total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC), RV/TLC ratio, maximal voluntary ventilation (MVV), forced ventilatory capacity (FVC), forced expiratory volume in one second (FEV 1), maximal mid expiratory flow (MMEF), maximal expiratory flow at 75% vital capacity (V 75 ), maximal expiratory flow at 50% vital capacity (V 50 ), maximal expiratory flow at 25% vital capacity (V 25 ) and breathing reserve ratio (BR) Results TLC, FRC, MVV, MMEF, V 75 and V 50 were not different from normal values IVC, FVC, FEV 1 and V 25 were significantly decreased compared with normal values RV and RV/TLC were high in 87 5% cases Conclusions Preoperative symptoms improved substantially after operation Little airway obstruction was observed postoperatively, suggesting that patients with pectus excavatum should have surgery as early in life as possible, preferably by age 3
文摘Background: Adolescents with pectus excavatum (PE) are often affected by their body shape. The aim of our approach was to quantify the patients’ individual psychological distress and to create a psychological indication for treatment. Methods: 10 adolescents (8 male, median age 16 years, interquartile range 15 - 17 yrs.) with PE were examined at our psychological department. Using standardized psychological tests, projective tests and interviews psychologists validated the patients’ individual psychological status. All patients were offered psychological therapy and correction of the deformity. In addition, the children were followed-up by a telephone questionnaire (median follow-up after starting therapy 12.8 months (5.9 - 18.0). Results: No patient had a relevant physiological limitation. The median follow-up since presentation to our psychologists was 15.0 months (9.1 - 20.6). 8 patients (5 were operated, 2 used the vacuum bell, 1 will undergo surgery) had distinct psychological limitations especially concerning the dimensions attractiveness, self-esteem and somatisation. They demonstrated increased insecurity, anxiety and denegation of their body. Since all patients were within puberty the psychological distress due to the PE has to be interpreted as disadvantageous for their further development. 7 patients completed the follow-up questionnaire and reached a median score of 80.8% (76.4% - 86.8%), which indicates a good improvement in all patients. Conclusions: We conclude that the psychological indication for treatment is justified, since our results support this indication.
基金supported by National Science & Technology Pillar Program during the 12th Fiveyear Plan Period(No.SQ2011SF12C03081)
文摘Background Thoracoscopic minimally invasive pectus excavatum repair （Nuss operation） features its little trauma, simple, short operation time, and good outcome compared with traditional treatment of pectus excavatum surgery-sternal elevation （Ravitch operation） and sternal turnover. The effect of the operation on patients＇ heart and heart function remains unclear. This study aimed to understand the changes of electrocardiogram and cardiac function after Nuss procedure. Methods From 2008 January to 2013 July, thoracoscopic Nuss operation was performed in 217 patients with pectus excavatum. All the patients underwent the preoperative, postoperative detection of ECG and cardiac function in 3 months to 1 year after operation. Results After 3 months to 1 year follow-up, arrhythmias persisted in 46 out of 135 patients with preoperative symptoms （P 〈 0.05）; Stroke volume and cardiac output significantly increased （P 〈 0.05）; And cardiac parameters greatly improved （P 〈 0.05）. Conclusions Minimally invasive repair of pectus excavatum deformity can correct the chest malformation, alleviate arrhythmia, and improve cardiac function.
文摘Background After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes. Data sources Literature was reviewed on PubMed with the terms 'pectus carinatum', 'minimal access repair', 'thoracos-copy' and 'children'. Results Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15.46 years met the inclusion criteria. Success rate of corrections wasn = 125, about 89% in cumulative reports, with seven articles reporting 100%. The complication rate was 39.28%. Since the pectus bar is placed over the sternum and has a large contact area, skin irritation was the most frequent morbidity (n = 20, 14.28%). However, within the complication group (n = 55), wire breakage (n = 21, 38.18%) and bar displacement (n = 10, 18.18%) were the most frequent complications. Twenty-two (15.71%) patients required a second procedure. Recurrences have been reported in four of twelve techniques. There were no lethal outcomes. Conclusions MARPC techniques are not standardized, as MIRPE are, so comparative analysis is difficult as the only common denominator is minimal access. Surgical morbidity is high in MARPC and affects > 2/3rd patients with about 15% requiring surgery for complication management.
文摘Background:Brachydactyly,a developmental disorder,refers to shortening of hands/feet due to small or missing metacarpals/ metatarsals and/or phalanges.Isolated brachydactyly type E (BDE),characterized by shortened metacarpals and/or metatarsals,consists in a small proportion of patients with Homeobox D13 (HOXD13) or parathyroid-hormone-like hormone (PTHLH) mutations.BDE is often accompanied by other anomalies that are parts of many congenital syndromes.In this study,we investigated a Chinese family presented with BDE combined with pectus carinatum and short stature.Methods:A four-generation Chinese family was recruited in June 2016.After informed consent was obtained,venous blood was collected,and genomic DNA was extracted by standard procedures.Whole-exome sequencing was performed to screen pathogenic mutation,array comparative genomic hybridization (Array-CGH) analysis was used to analyze copy number variations,and quantitative real-time polymerase chain reaction (PCR),stride over breakpoint PCR (gap-PCR),and Sanger sequencing were performed to confirm the candidate variation.Results:A 3.06-Mb deletion (chr12:25473650-28536747) was identified and segregated with the phenotype in this family.The deletion region encompasses 23 annotated genes,one of which is PTHLH which has been reported to be causative to the BDE.PTHLH is an important regulator of endochondral bone development.The affected individuals showed bilateral,severe,and generalized brachydactyly with short stature,pectus carinatum,and prematurely fusion of epiphyses.The feature of pectus carinatum has not been described in the PTHLH-related BDE patients previously.Conclusions:The haploinsufficiency of PTHLH might be responsible for the disease in this family.This study has expanded the knowledge on the phenotypic presentation of PTHLH variation.
基金supported by a grant from the Research Foundation of Korea University(K1220231)
文摘Background:Pectus excavatum is the most common congenital chest wall deformity,with a high incidence in live births.This study aimed to evaluate the measured factors on CT images related to the number of pectus bars for surgical correction.Methods:A total of 497 patients who had undergone minimally invasive repair between April 2007 and July 2011 were classified into single-bar(n=358)and doublebar(n=139)insertion groups.We measured eight distinct distances and one angle on CT scans to reflect quantitative assessment.Univariate analysis and multivariate logistic regression analysis were performed to detect statistically significant association between radiologic measurements and the pectus bars required.Results:After adjusting for age and gender,the transverse distance(T),the transverse distance of the depression area(A),the inclined distance of the depression area(B),the AP distance of the depression area(C),the depression angle(G),and the eccentric distance of deformity(E)were significantly correlated with double-bar insertion.The regression model showed that age(,P<0.0001),gender(P<0.0001),depression angle(G)(P<0.0001),direction of the depression(DD)(P<O.OOO1)and depression depth(D)(P<0.0001)were significantly associated with doublebar insertion.Conclusion:CT scan provides useful factors which can be of assistance in predicting the number of pectus bars for the surgical correction of pectus excavatum.
