保守疗法治疗先天性气管狭窄的效果:一项循证长期随访研究

Background/Purpose:Surgery has been the management of choice for severe congenital tracheal stenosis (CTS). The role of conservative management of CTS however is not clear. The aim of this study is to characterize the natural history of CTS,review the radiologic evidence of tracheal growth,and evaluate the clinical outcome and selection criteria of conservative management of CTS. Methods:A retrospective study was carried out on 22 consecutive children with symptomatic CTS admitted into a single institution between 1982 and 2001. The patients were categorized into operation (n = 11) and observation(n = 11) groups. Six patients of the observation group were followed up with serial computed tomography scan. Their tracheal growth was compared with that of healthy children of the same age. Results:The mortality rates of observation and operation groups were 9%and 27%,respectively,although the latter group consisted of more severely affected patients. The pathologic categorization of the CTS influenced the survival rates (P = 0.046,χ2),with the long segment type having the worst prognosis (67%). Serial computed tomography scans of 6 conservatively managed patients revealed that all stenotic tracheas continued to grow (P = .039,2-tailed paired Student’s t test). Of the 6 stenotic tracheas,5 grew at a faster-than normalrate,and the stenotic tracheal diameters approached those of normal diameters by the age of 9 years. Conclusions:The management of patients with symptomatic CTS should be individualized. A selected group of patients with CTS can be safely managed nonoperatively.

高危神经母细胞瘤强化治疗方案中的外科问题

Background: High- risk neuroblastoma (NB; age, > 1 year; INSS stage 4) is associated with a poor outcome. At our institution, the current dose- intensive high- risk Children’ s Oncology Group protocol for advanced NB appears to have a higher surgical complication rate as compared with previous protocols. Methods: All stage 4 patients (n = 51) entered in high- risk protocols between 1995 and 2005 were analyzed. Patients in the current high- risk protocol, Children’ s Oncology Group A3973 (n = 22), were compared with those in the 2 previous protocols, CCG 3891 and POG 9341 (n = 29). Results: Patients were comparable in their mean age and tumor markers, including Shimada histology, MYCN amplification, 1p deletion, tumor origin, and extent of metastasis. However, transfusion requirement (86% vs 45% ; P = 0.0019), postoperative infection rate (32% vs 3% ; P = 0.02), and other postoperative issues including nutritional support (45% vs 3% ; P = 0.0001) were significantly higher with the current protocol. No perioperative mortality was noted in either group, and the extent of resectability and margins were similar. Importantly, with the current protocol, the survival rate was higher (P = 0.0022) and the recurrence rate was significantly lower (P = 0.0003). Conclusions: Despite higher surgical morbidity associated with the current high- risk protocol (2.59 vs 0.86 complications/person; P < 0.01), the recurrence rate is lower and interim survival rate is improved for patients with high- risk NB. Therefore, the higher surgical complication rates associated with the current high- risk protocol are acceptable.

10年来在气管软化和(或)支气管软化的处理中应用气管/支气管腔内支架术是否优于主动脉固定术

We compared the efficacy and clinical outcomes of aortopexy versus tracheal stents in the management of tracheomalacia. Methods: A retrospective analysis of 25 consecutive patients undergoing aortopexy (n = 11; 8 boys, 3 girls) or tracheal stents (n = 14; 9 boys, 5 girls) between 1993 and 2003 was performed. Results: Both treatment groups, aortopexy versus stents, were comparable in their mean age of diagnosis, timing of intervention, surgical indications (“ dying spell” or failed extubation), and previous underlying conditions. The operative time (190 vs 72 minutes) and blood loss (26 vs 0 mL) were significantly greater in aortopexy group (P < . 01). There were no perioperative deaths in either group. Interestingly, 4 of 11 patients in the aortopexy group developed pericardial effusion (P <. 01). With stents in place for a mean of 15 (range 2- 41) months, 3 of 8 patients with stent removal had significant granulation tissue requiring further dilatation. No death was observed in aortopexy group, whereas 1 stentrelated death and 1 cardiac arrest requiring median sternotomy occurred during stent removal in 44 and 32 months’ follow-up, respectively. Conclusion: Both aortopexy and tracheal stents are effective treatment modalities in the management of tracheomalacia. However, although aortopexy is associated with early perioperative complications, tracheal stents are associated with higher failure rate and more severe stent-related morbidity and mortality.

临床表现明显的血管环伴先天性气管狭窄患儿的长期预后

Aim: Congenital tracheal stenosis (CTS) associated with vascular rings (VRs) is considered a secondary condition,but relative indications for selective VRs or combined VR and CTS repairs are unclear. Here,we report long-term outcomes after VR repair associated with tracheal narrowing (TN). Method: Retrospective analysis of patients admitted to a single institution with VR from 1982 to 2004. Of 131 patients with VR (Cardiac Registry Database),29 (19 males,10 females; age range,0 days-4 years) associated with symptomatic TN. Patients were followed until age 18 years. Results: One hundred thirty-one patients with VR were categorized into 3 treatment groups. There were 102 patients with VR with no symptomatic TN treated by VR repair (4 deaths,4% mortality). Of the 29 patients with VR + TN symptoms,16 were treated by selective VR repair (mean age,5.3 months; range,17-288 days) with 3 deaths (19% mortality) and 13 underwent combined VR and CTS repairs (mean age,11 months; range,1 day-4 years) with 6 deaths (46% mortality),for an overall mortality of 31% (P < 0.01,χ 2 test). Of the 102 patients with VR,17 had intracardiac anomalies with 3 deaths (18% mortality). In contrast,7 of 29 patients with VR with TN and intracardiac anomalies died 4(57% mortality); 3 patients in combined cardiac and CTS repairs (2 deaths,67% mortality) and 4 in selective VR repairs (2 deaths,50% mortality). Conclusions: Not all cases of TN associated with VR require combined repairs. The presence of TN and/or intracardiac anomalies in patients with VR,however,increases mortality risk to the repair of VR. Surgical decision requires individualized clinical pathway.