背景：先天性小耳畸形（Congenital Microtia）是一种因胚胎期第一、二鳃弓及第一腮沟发育异常而导致的外耳与中耳畸形，主要临床特征表现为耳廓形态学改变。皮肤扩张法自体肋软骨外耳再造术是治疗先天性小耳畸形的有效手段。鉴于外耳再造术Ⅰ期的治疗持续时间长、照护内容复杂繁琐、手术对象年龄小，需家长监护，以及患儿及父母的心理负担重等原因，为接受此类患儿及家长提供合适的照护极为重要。基于当前庞大的病例基数和有限的医疗资源，如何统一规范照护内容，如何开展满足大基数需求的随访工作，帮助患儿家属完成院外照护，是家属与医务人员亟待解决的问题。因此，本研究旨在构建先天性小耳畸形患儿皮肤扩张法外耳再造术Ⅰ期以家庭为中心的照护路径，在临床中实施此路径并评价其应用效果，以期改善患儿健康结局与治疗效率、提高患儿父母照护能力以及以家庭为中心的照护质量。

目的：

1. 基于循证总结，并结合临床现状和专家意见，形成规范详细的先天性小耳畸形患儿皮肤扩张法外耳再造术Ⅰ期以家庭为中心的照护路径。

2. 在临床实践中应用该照护路径，以验证路径的有效性。

方法：

1. 构建照护路径

系统地检索国内外有关先天性小耳畸形患儿照护的相关文献，并对纳入文献进行分析和质量评价，初步制定《先天性小耳畸形患儿皮肤扩张法外耳再造术Ⅰ期以家庭为中心的照护路径》。采用德尔菲专家函询法，选择整形外科医生、临床护理专家、临床护理管理专家、护理教育专家共14人，结合专家的知识和经验对初始方案中的条目进行评价，根据专家意见进行修改，直至专家意见相对统一，最终形成《先天性小耳畸形患儿皮肤扩张法外耳再造术Ⅰ期以家庭为中心的照护路径》终稿。

2. 实施照护路径并评价效果

此部分为干预验证阶段，通过类实验研究，纳入行皮肤扩张法外耳再造术Ⅰ期的先天性小耳畸形患儿及其父母。对照组接受常规照护；路径组的干预方法遵循所形成的照护路径提供照护，比较对照组和路径组患儿的健康结局（Ⅰ期手术并发症发生率、超重比例、非计划入院率）、治疗效率（整个Ⅰ期治疗时间）、患儿父母照护能力及焦虑状态、以家庭为中心的照护质量评价。

结果：

1. 形成照护路径                                      

文献分析部分经筛选最终纳入8篇文献，形成了有关住院期间（入院、术前、术后、出院），出院期间（注水及养皮期）多个阶段的20条相关证据，以此为依据构建照护路径初稿。德尔菲函询，专家整体权威系数为0.93，两轮专家的积极系数分别为82.35%和100%；第二轮的各条目变异系数均值均＜0.25；第一轮重要性和可行性评分的Kendall’ s W系数分别为0.130、0.134，第二轮重要性和可行性评分的Kendall’ s W系数分别为0.095、0.141，显著性检验均为P＜0.01。结合专家意见对照护路径进行修改，最终形成了照护路径终稿。最终方案包括住院期间和出院期间两个干预阶段：住院期间包括建立多学科团队协作，充分阐明手术相关信息，全面评估患儿及家庭特点，围术期提供专科护理以及健康宣教；出院期间包括定期随访患儿扩张皮瓣与伤口情况，协助管理患儿体重，提供同伴与心理支持平台。

2. 照护路径的临床应用效果

本研究纳入符合标准的先天性小耳畸形患儿及父母104例，其中路径组（n=52），对照组（n=52）。两组患儿一般情况比较显示差异无统计学意义（P＞0.05）。①在整个Ⅰ期治疗期间中，对照组患儿手术并发症的发生率（17.6%）高于路径组（4.2%），两组间差异具有统计学意义（P=0.033）。②在Ⅰ期入院当天、Ⅰ期出院当天、注水结束当天和Ⅱ期入院当天，对照组与路径组患儿的超重比例均无显著差异（P＞0.05）。③对照组非计划入院率为7.8%，路径组为2.1%，两组间差异无统计学意义（P＞0.05）。④对照组患儿整个Ⅰ期治疗天数平均为（119.79±18.39）天，路径组整个Ⅰ期治疗天数平均为（120.64±16.45）天，两组间差异无统计学意义（P＞0.05）。⑤在Ⅱ期入院当天测量的两组父母照护能力得分比较显示，对照组总均分（69.13±17.09分）低于路径组（77.78±12.32分），差异具有统计学意义（P=0.006）。⑥两组患儿父母焦虑情绪比较显示，注水结束时，对照组父母SAS得分高于路径组，且差异具有统计学意义（P=0.001）。⑦相比对照组，路径组患儿父母对照护质量的评价得分在住院期间和注水期间更高，差异具有统计学意义（P＜0.05），但在养皮期间无明显差异。

结论：

1. 本研究构建的《先天性小耳畸形患儿皮肤扩张法外耳再造术Ⅰ期以家庭为中心照护路径》包括住院期间和出院期间两个干预阶段。住院期间涵盖了入院、术前、术后、出院四个阶段，包括多学科合作、全面评估、围术期护理、健康宣教在内的具体照护要点；出院期间（注水及养皮期）包括定期随访患儿扩张皮瓣与伤口情况，协助管理患儿体重，提供同伴与心理支持平台3个方面。该照护路径具体、可操作性强。

2. 构建的照护路径可降低皮肤扩张法外耳再造术Ⅰ期患儿手术并发症的发生率，提高患儿父母照护能力，提高患儿父母对照护质量评价，缓解患儿父母焦虑情绪。

Background: Congenital microtia, an abnormality of the outer and middle ear, is caused by developmental anomalies of the first and second branchial arches and first cleft during the embryonic stage. Autologous rib cartilage auricular reconstruction with skin expansion is an effective treatment. Given the lengthy duration, complex care, young patient age, parental involvement, and psychological burdens, providing proper care for patients and parents is crucial. With a large number of cases and limited medical resources, standardizing care and conducting follow-ups to assist families with post-discharge care are urgent issues. This study aims to develop a family-centered care pathway for the first stage of skin expansion autologous rib cartilage auricular reconstruction in children with congenital microtia, implement it clinically, and evaluate its effectiveness to improve health outcomes, treatment efficiency, parental care ability and emotions, and family-centered care quality.

Objectives:

1. Formulate a detailed, evidence-based, family-centered care pathway for the first stage of skin expansion autologous rib cartilage auricular reconstruction in children with congenital microtia, integrating clinical realities and expert opinions.

2. To apply this care pathway in clinical practice to verify its efficacy.

Methods:

1. Pathway Development:

Systematically review domestic and international literature on congenital microtia care, analyze and quality-assess included studies, and preliminarily formulate the care pathway. Use the Delphi method with 14 experts (plastic surgeons, clinical nursing experts, clinical nursing management experts, and nursing education experts) to evaluate and refine the initial pathway until consensus is reached, forming the final version.

2. Pathway Implementation and Evaluation:

In this quasi-experimental study, include children with congenital microtia undergoing the first stage of skin expansion autologous rib cartilage auricular reconstruction and their parents. The intervention group follows the care pathway, while the control group receives routine care. Compare health outcomes (complication rates, overweight rates, unplanned readmissions), treatment efficiency (total first-stage treatment time), parental care ability, anxiety levels, and family-centered care quality between the two groups.

Results:

1. Formation of the Care Pathway

From 8 included literature, extract 20 evidence items across hospitalization (admission, preoperative, postoperative, discharge) and post-discharge (inflation and skin expansion) phases to build the pathway. In the Delphi survey, Expert authority coefficient is 0.93. First-round expert response rate is 82.35%, second-round is 100%. Second-round mean variation coefficient for items is below 0.25. Kendall’s W coefficients for importance and feasibility in the first and second rounds are 0.130, 0.134, 0.095, and 0.141, respectively, all with P < 0.01. Final pathway includes two stages: hospitalization (multidisciplinary collaboration, surgery information explanation, family assessment, perioperative care, health education) and post-discharge (regular follow-ups on skin flaps and wounds, weight management, peer and psychological support platforms).

2. Clinical Application Effect of the Care Pathway

A total of 104 children with congenital microtia and their parents who met the criteria were included in this study, including 52 cases in the pathway group and 52 cases in the control group. On the time of admission in the first stage, the time of discharge in the first stage, the end of injection, and the time of admission in the second stage day of admission, there were no significant differences in the proportion of overweight children between the control group and the pathway group. (P > 0.05). ① In the whole first stage, the complication rate of the control group (17.6%) was higher than that of the pathway group (4.2%), and the difference between the two groups was statistically significant (P = 0.033). ② There was no significant difference in the overweight proportion between the control group and the pathway group (P > 0.05). ③ The unplanned admission rate of the control group was 7.8%, and that of the pathway group was 2.1%, and the difference between the two groups was not statistically significant (P > 0.05). ④The average treatment time of the whole first stage in the control group was (119.79 ± 18.39) days, and that in the pathway group was (120.64 ± 16.45) days, and the difference between the two groups was not statistically significant (P > 0.05). ⑤At the time of admission in the second stage day of admission, there was a significant difference in the comparison of the parents' care ability scores between the two groups. The total mean score of the control group (69.13 ± 17.09 points) was lower than that of the pathway group (77.78 ± 12.32 points), and the difference was statistically significant (P = 0.006). ⑥ In the comparison of the parents' anxiety levels between the two groups, at the end of injection, the SAS score of the control group's parents was higher than that of the pathway group, and the difference was statistically significant (P = 0.001). ⑦ Compared with the control group, the scores of the children and their parents' evaluation of the care quality in the pathway group were higher during the hospitalization and injection periods, and the differences were statistically significant (P < 0.05), but there was no significant difference during the skin - nurturing period.

Conclusions:

1. The developed care pathway for the first stage of skin expansion autologous rib cartilage auricular reconstruction includes two stages: hospitalization (covering admission, preoperative, postoperative, discharge phases with multidisciplinary collaboration, assessment, perioperative care, health education) and post-discharge (inflation and skin expansion phases with regular follow-ups, weight management, peer and psychological support platforms). It is specific and highly operational.

2. The pathway reduces complication rates, enhances parental care ability, improves care quality evaluations, and alleviates parental anxiety.