临床小儿外科杂志  2019, Vol. 18 Issue (1): 13-17
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Nuss手术治疗漏斗胸患儿合并特发性脊柱侧弯的临床研究
曹隽 , 张学军 , 曾骐 , 陈诚豪 , 于洁 , 郭东 , 白云松 , 祁新禹 , 姚子明 , 冯磊     
首都医科大学附属北京儿童医院(北京市,100045)
摘要目的 了解漏斗胸患儿合并特发性脊柱侧弯的发病情况及经Nuss手术治疗后脊柱侧弯的改变情况。方法 以2011至2013年于首都医科大学附属北京儿童医院接受胸外科手术治疗并符合纳入标准的171例漏斗胸患儿为研究对象,患儿入院后行CT检查了解漏斗胸情况,并分别于Nuss手术前及取出钢板后行胸部及脊柱X线检查了解脊柱侧弯情况;对比脊柱侧弯与非侧弯患儿的胸骨旋转度、胸廓旋转度、Haller指数等指标,同期对比胸廓对称与非对称患儿的脊柱侧弯改变情况。结果 171例漏斗胸患儿中合并脊柱侧弯的比例为21.1%(36/171),平均Cobb角16.9°;侧弯患儿与非侧弯患儿Haller指数无统计学差异(P>0.05),但胸骨旋转度及胸廓旋转度均显著大于非侧弯患儿(P < 0.05);侧弯患儿根据弯度给予相应治疗(侧弯 < 25°的患儿继续随诊观察,侧弯在25° ~45°且骨骼发育未成熟的患儿要求佩戴支具,侧弯>45°的患儿予以手术治疗);中位随访时间为35.73个月,随访过程中,13例原脊柱正常患儿出现脊柱侧弯,14例患儿原脊柱侧弯消失;胸廓不对称患儿脊柱侧弯出现变化的比例显著高于胸廓对称的患儿(P < 0.05)。结论 漏斗胸患儿易合并特发性脊柱侧弯,Nuss手术治疗后其脊柱侧弯程度可能发生改变,需要小儿胸外科医生与小儿骨科医生共同协作治疗。
关键词儿童; 漏斗胸; Nuss手术; 特发性脊柱侧弯
Clinical study of correcting scoliosis plus pectus excavatum with Nuss procedure in children
Cao Jun , Zhang Xuejun , Zeng Qi , Chen Chenghao , Yu Jie , Guo Dong , Bai Yunsong , Qi Xinyu , Yao Ziming , Feng Lei     
Department of Surgery, Affiliated Children's Hospital, Capital Medical University, Beijing 100045, China
Abstract: Objective To evaluate the prevalence and progression of idiopathic scoliosis plus pectus excavatum after Nuss procedure. Methods A total of 171 pectus excavatum cases were retrospectively reviewed.Chest and spinal radiographic films were taken before Nuss procedure and after bar removal.Several parameters including thoracic kyphosis, sternum rotation, thoracic rotation, thoracic asymmetric ratio and Haller index were compared between scoliosis and non-scoliosis groups.Also the changes of scoliosis were compared between symmetric and asymmetric chest groups. Results The prevalence of scoliosis plus pectus excavatum was 21.1% (36/171) with an average Cobb's angle of 16.87°.In scoliosis group, there were higher values of sternal and thoracic rotation (P < 0.05) and no difference existed between Haller index.The scoliosis patients were treated as observation/brace/surgery due to their curve angle.The follow-up period was 35.73 months.After bar removal, 14 cases improved to normal while another 13 normal cases progressed to scoliosis.Scoliosis change occurred more likely in asymmetric chest patients (P < 0.05). Conclusion There is a high prevalence of idiopathic scoliosis in pectus excavatum patients.And scoliosis may change after Nuss procedure.Pediatric chest and orthopedic surgeons should cooperate closely.
Key words: Child; Funnel Chest; Nuss Procedure; Idiopathic Scoliosis

漏斗胸是一种常见的儿童先天性前方胸廓畸形,发病率为1‰ ~3‰,男性发病率高于女性[1]。典型表现为前方胸骨及肋软骨局限性凹陷[2]。除外观上的异常,凹陷的胸壁还会压迫下方的心脏和肺脏,并影响其生理功能和发育过程[3-5]。除了影响心肺功能外,漏斗胸患儿还可合并其他畸形[6]。据报道,在需要手术治疗的漏斗胸患儿中,合并特发性脊柱侧弯者占16% ~23%[7-10]。脊柱侧弯会造成双肩不等高、躯干倾斜、一侧背部突出等外观异常[11]。更重要的是,弯曲的脊柱也会压迫胸廓和肺脏,加重漏斗胸对肺功能的影响[12]

Nuss医生报道并推广的微创漏斗胸修补手术(Nuss手术)是漏斗胸治疗领域的一个重要里程碑[13-17]。而特发性脊柱侧弯的治疗则比较复杂,需要根据患儿年龄、侧弯类型、进展速度选择非手术或手术治疗方法,总体治疗原则是在控制并矫正畸形的同时保留脊柱的生长潜能[18]。由于漏斗胸和特发性脊柱侧弯的伴发比例相对较高,且二者可能在病因学上存在交互作用,对患儿的远期预后带来不利影响。本研究旨在了解漏斗胸患儿合并特发性脊柱侧弯经过Nuss手术治疗后的脊柱侧弯进展情况,为儿童漏斗胸患儿合并特发性脊柱侧弯的诊治提供临床依据。

材料与方法 一、研究对象

以2011至2013年于首都医科大学附属北京儿童医院接受胸外科手术治疗的漏斗胸患儿为研究对象。纳入标准:①漏斗胸未经特殊治疗者;②本次入院行Nuss手术治疗者;③门诊检查与手术时间间隔不超过1个月者;④无先天性肋骨畸形者。排除标准:①非特发性脊柱侧弯(包括先天性脊柱侧弯、神经肌肉型脊柱侧弯以及综合征型脊柱侧弯)者;②有脊柱手术病史者;③2年内行手术取出钢板者;④存在先天性肋骨融合畸形者;⑤患儿未行取钢板手术或取钢板手术与首次手术间隔小于2年者。最终共171例患儿纳入本研究,其中男132例,女39例,平均年龄6.94岁(3.92~12.5岁)。

二、研究方法

入选患儿均行胸部CT检查,根据CT检查结果测量Haller指数评估漏斗胸严重程度;测量胸骨旋转角度(轴位胸骨与椎体横轴成角)、胸廓旋转角度(胸骨后方至椎体前缘正中连线与椎体纵轴成角)、胸廓对称度(两侧胸廓最长纵径比值,当两侧最长胸廓纵径差异>10 mm或不对称指数<0.9时可判定为不对称胸廓)。手术指征为:①Haller指数>3.2;②存在心肺受压的影像学表现或临床症状。站立位胸部X线正侧位检查了解胸廓、肋骨和脊柱情况,测量冠状位脊柱侧弯Cobb角及矢状位胸椎后凸角,如Cobb角>10°认为存在脊柱侧弯,此时需进一步行脊柱正侧位X线检查了解脊柱情况。患儿行Nuss手术出院后定期门诊随诊,手术后2~3年取出钢板,取出钢板第2天行站立位胸部/脊柱正侧位X线检查及胸廓CT评估漏斗胸矫正效果。

脊柱侧弯的治疗方案根据Cobb角大小确定:<25°时继续观察;25° ~45°时佩戴脊柱支具治疗;>45°时行后路融合手术(如骨骼发育未成熟则行后路生长棒手术,并定期行再撑开手术控制侧弯进展,至骨骼发育成熟后再行后路融合手术)。如脊柱侧弯与Nuss手术均需要手术治疗,则先行Nuss手术,6个月后再行脊柱侧弯手术。

三、统计学处理

采用SPSS17.0进行统计分析。采用均数、标准差、率、构成比对结果进行统计学描述;根据入院时患儿是否存在脊柱侧弯分为侧弯组和非侧弯组,根据胸廓对称度将患儿分为胸廓对称组和胸廓非对称组,采用两独立样本t检验比较两组术前及术后的漏斗胸Haller指数、胸骨旋转度、胸廓旋转度、胸廓对称度等指标有无差异;采用χ2检验分析胸廓不对称组与对称组侧弯的人数分布及改变情况。以P<0.05为差异有统计学意义。

结  果

171例患儿中,首次就诊时有36例诊断为脊柱侧弯(21.1%), 其中男7例,女29例;32例为单胸弯,2例为双胸弯,2例为胸腰弯;21例侧弯凸向左侧,13例凸向右侧,2例为双胸弯;侧弯组Cobb角、胸骨旋转度、胸廓旋转度、胸廓不对称比例显著高于非侧弯组,胸椎后凸角度则显著低于非侧弯组(P < 0.05)。详见表 1

表 1 侧弯组与非侧弯组漏斗胸患儿影像学指标差异(x±s) Table 1 Variations in imaging parameters between scoliosis and non-scoliosis pectus excavatum patients(x±s)

经过平均35.73个月(24~40个月)的随访后,患儿经手术取出漏斗胸矫正钢板并行X线检查,其中35例发生脊柱侧弯,发生率为20.5%,平均侧弯角度为14.8°。14例于进入研究队列时伴脊柱侧弯者经治疗后脊柱侧弯角度减小到10°以内,另有13例原无侧弯的患儿新发脊柱侧弯,均为胸弯,凸向左侧7例,右侧6例。新出现的脊柱侧弯患儿Cobb角均<25°,因此均随诊观察。

Nuss手术前不对称胸廓组患儿共37例,胸廓对称组与不对称组患儿的年龄、Cobb角、胸椎后凸、胸廓旋转度及随访时间均无统计学差异(P>0.05),但胸廓不对称组胸骨旋转度更高(P < 0.05)。胸廓不对称的脊柱侧弯患儿中,有51.4%(19/37)出现病情变化(由无侧弯变为有侧弯或原有侧弯消失),胸廓对称的患儿中仅有6.0%(8/134)出现病情变化,差异有统计学意义(P < 0.05)。详见表 2表 3。典型病例见图 1图 2

表 2 胸廓不对称组与对称组影像学指标差异(x±s) Table 2 Variations in imaging parameters between symmetric and non-symmetric chest cage groups(x±s)

表 3 胸廓不对称组与对称组侧弯分布及改变情况[%(n/N)] Table 3 Scoliosis distributions and change variations between between symmetric and non-symmetric chest cage groups[%(n/N)]

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图 1 A.男性漏斗胸患儿,5.2岁,X线片示术前无脊柱侧弯;B.X线片示Nuss手术后出现胸段右凸侧弯,Cobb角11°;C.3年后取出钢板,X线片示侧弯进展Cobb角为17°;D.CT提示为不对称漏斗胸,左侧凹陷 Fig. 1 A.A 5.2-year-old boy with pectus excavatum and there was no preoperative scoliosis; B.after Nuss procedure, there was an onset of right convex thoracic scoliosis with a Cobb's angle of 11°; C.after 3 years, upon bar removal, scoliosis progressed with a Cobb's angle of 17°; D.CT showed asymmetric pectus excavatum with left cave-in

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图 2 A.男性患儿,4.8岁,X线片示漏斗胸存在胸段脊柱侧弯,Cobb角18°;B.X线片示Nuss手术后脊柱弯度略减小,Cobb角减少到4°;C.3年后取出钢板,X线片示侧弯Cobb角减小至2°;D.CT提示为不对称漏斗胸,左侧凹陷 Fig. 2 A.A 4.8-year-old boy with pectus excavatum and there was thoracic scoliosis with a Cobb's angle of 18°; B.after Nuss procedure, spinal curvature declined slightly and Cobb's angle decreased to 4°; C.after 3 years, upon bar removal, Cobb's angle of scoliosis decreased to 2°; D.CT showed asymmetric pectus excavatum with left cave-in
讨  论

漏斗胸曾被认为与纵膈的牵拉密切相关,但近些年的研究显示,肋软骨的过度生长也参与了畸形的形成过程[19]。过度生长的肋软骨造成前方胸廓的凹陷,使得整体躯干发生扭转,从而造成胸骨的倾斜和胸廓的不对称[20]。而特发性脊柱侧弯除表现出冠状位侧弯和矢状位的胸椎后凸减小以外,另一个重要表现就是水平位上的椎体旋转。5岁以内的幼年及儿童期出现的早发性脊柱侧弯可能影响胸廓发育,包括先天性、神经肌肉性、综合征性和特发性4类[18, 21]。前3类脊柱侧弯的进展主要取决于其病因特点:先天性脊柱侧弯主要由于椎体发育畸形,导致脊柱畸形并不断进展;神经肌肉性脊柱侧弯的进展主要源于神经控制异常或肌肉力量不平衡;综合征性脊柱侧弯则与肌肉软组织松弛(马凡综合征)或骨骼发育异常有关。

本研究中,漏斗胸患儿合并脊柱侧弯的比例为21.1%,与既往文献报道结果相近[7-10]。脊柱侧弯中21例凸向左侧,13例凸向右侧,且与漏斗胸固定器放置无明显关联。有报道称漏斗胸患儿合并脊柱侧弯多凸向右侧,并认为其主要的影响因素为机械性因素,但本研究中未发现此现象[9]。分型方面多为胸弯,胸腰弯与双胸弯较少,未观察到腰弯,这与漏斗胸累及胸廓的特点一致。侧弯组患儿胸椎后凸较非侧弯组小,符合特发性脊柱侧弯平背畸形的特点。

除去经手术和支具治疗的患儿,原随访观察的脊柱侧弯患儿中有14例脊柱侧弯消失,另有13例原脊柱无侧弯的患儿出现了轻度侧弯,其中大部分出现病情变化的患儿都未接受过任何脊柱侧弯的相关治疗。这说明漏斗胸患儿合并的脊柱侧弯不是恒定的,在某些因素的作用下脊柱侧弯情况会发生变化。进一步分析发现,病情发生改变的患儿大多数为非对称性漏斗胸,有文献报道过类似的结果[22],也有文献提出这类患儿存在不同程度的椎体旋转[23]。结合本研究中胸廓旋转在脊柱侧弯患儿中明显高于非侧弯患儿的特点,推测可能是由于非对称漏斗胸在矫正过程中,两侧胸廓前壁受力不平衡导致胸廓扭转,而扭转力通过肋骨及肋椎关节传递到椎体,使得脊柱侧弯发生变化。

在脊柱侧弯的治疗方面,绝大多数患儿的侧弯程度较轻,仅需随诊观察;少部分患儿因侧弯严重采取了支具佩戴联合手术治疗的方案。随诊观察的病例并不影响漏斗胸的治疗,且经Nuss手术后一部分脊柱侧弯自发消失;需要佩戴支具的患儿则需先行Nuss手术后再佩戴支具,以避免漏斗胸手术后胸廓外形改变导致支具不适合其生理结构位置。本研究中,3例通过支具治疗的患儿经3年随访取出漏斗胸钢板后,侧弯均得到控制;需手术的患儿则均先行漏斗胸手术,6个月后再行脊柱手术,漏斗胸手术能够改善心肺受压状况,且置入的钢板能够支撑胸廓,有效避免了患儿俯卧位情况下胸部受压。2例行生长棒手术的患儿未发生漏斗胸钢板移位或脊柱手术并发症,目前已取出钢板,但仍需定期行脊柱撑开手术。Nuss手术后,一部分原脊柱正常的患儿出现脊柱侧弯,但其侧弯程度较轻,其远期预后状况的评估还需进一步随访观察。漏斗胸和脊柱侧弯两者的治疗并不冲突[13],在本院就诊的此类患儿中,两种疾病中任何一种严重至符合手术指征,即可行相应手术;但当两者都严重到需要手术时,我们倾向于先进行可有效改善患儿心肺功能的漏斗胸手术。

本研究的局限性包括如下几点:首先,首次就诊时采用站立位胸片对脊柱侧弯进行初步检查,确认脊柱侧弯后再拍脊柱X线片,这可能会漏诊一部分腰段脊柱侧弯患儿,但鉴于腰段侧弯与胸廓功能的关联相对较小,因此未对其进行深入分析;其次,在随访过程中,末次随访时选择去除钢板的第2天进行影像学检查,但此时患儿仍未完全恢复,对研究结果可能造成一定影响;最后,本研究仅针对不对称胸廓畸形与脊柱侧弯之间关联性进行了初步分析,但其具体作用机制还需通过相关的实验研究来进一步验证。

参考文献
1
Fokin AA, Steuerwald NM, Ahrens WA, et al. Anatomical, histologic, and genetic characteristics of congenital chest wall deformities[J]. Semin Thoracic Cardiovasc Surg, 2009, 21(1): 44-57. DOI:10.1053/j.semtcvs.2009.03.001.
2
Brochhausen C, Turial S, Müller FK, et al. Pectus excavatum:history, hypotheses and treatment options[J]. Interact Cardiovasc Thorac Surg, 2012, 14(6): 801-806. DOI:10.1093/icvts/ivs045.
3
Shamberger RC. Congenital chest wall deformities[J]. Current Problems in Surgery, 1996, 33(6): 469-542. DOI:10.1016/S0011-3840(96)80005-0.
4
Ravitch MM. Pectus excavatum and heart failure[J]. Surgery, 1951, 30(1): 178.
5
Jr KR, Lawson ML, Paidas CN, et al. Pectus excavatum in a 112-year autopsy series:anatomic findings and the effect on survival[J]. Journal of Pediatric Surgery, 2005, 40(8): 1275-1278. DOI:10.1016/j.jpedsurg.2005.05.010.
6
Park JM, Varma SK. Pectus excavatum in children:diagnostic significance for mitral valve prolapse[J]. Indian Journal of Pediatrics, 1990, 57(2): 219-222. DOI:10.1007/BF02722092.
7
Waters P, Welch K, Micheli LJ, et al. Scoliosis in children with pectus excavatum and pectus carinatum[J]. J Pediatr Orthop, 1989, 9(5): 551-556. DOI:10.1097/01241398-198909010-00009.
8
Niedbala A, Adams M, Boswell WC, et al. Acquired thoracic scoliosis following minimally invasive repair of pectus excavatum[J]. Am Surg, 2003, 69(6): 530-533.
9
Tardie GB, Dorsey DA, Kaeferlein BH. Evaluation of unexplained dyspnea in a young athletic male with pectus excavatum[J]. J Sports Sci Med, 2005, 4(3): 323-331.
10
Yeh YC, Yang CC, Tai CL, et al. Characterization of a novel caudal vertebral interbody fusion in a rat tail model:An implication for future material and mechanical testing[J]. Biomedical Journal, 2017, 40(1): 62-68.
11
Goldberg CJ, Fogarty EE, Moore DP, et al. Scoliosis and developmental theory:adolescent idiopathic scoliosis[J]. Spine, 1997, 22(19): 2228. DOI:10.1097/00007632-199710010-00006.
12
Jr CR, Smith MD, Mayes TC, et al. The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis[J]. Journal of Bone & Joint Surgery American Volume, 2003, 85(3): 399.
13
Kelly RE, Goretsky MJ, Obermeyer R, et al. Twenty-one years of experience with minimally invasive repair of pectus excavatum by the Nuss procedure in 1215 patients[J]. Annals of Surgery, 2010, 252(6): 1072. DOI:10.1097/SLA.0b013e3181effdce.
14
Hoksch B, Kocher G, Vollmar P, et al. Nuss procedure for pectus excavatum in adults:long-term results in a prospective observational study[J]. EurJ Cardiothorac Surg, 2016, 50(5): 934-939. DOI:10.1093/ejcts/ezw130.
15
Sacco Casamassima MG, Gause C, Goldstein SD, et al. Patient Satisfaction After Minimally Invasive Repair of Pectus Excavatum in Adults:Long-Term Results of Nuss Procedure in Adults[J]. Annals of Thoracic Surgery, 2016, 101(4): 1338-1345. DOI:10.1016/j.athoracsur.2015.09.102.
16
Zhang DK, Tang JM, Ben XS, et al. Surgical correction of 639 pectus excavatum cases via the Nuss procedure[J]. Journal of Thoracic Disease, 2015, 7(9): 1595. DOI:10.3978/j.issn.2072-1439.2015.09.30.
17
Rainey JW, David F, Sunil S. Systematic review of surgical treatment techniques for adult and pediatric patients with pectus excavatum[J]. Journal of Cardiothoracic Surgery, 2014, 9(1): 25. DOI:10.1186/1749-8090-9-25.
18
Tis JE, Karlin LI, Akbarnia BA, et al. Early onset scoliosis:, modern treatment and results[J]. Journal of Pediatric Orthopaedics, 2012, 32(7): 647. DOI:10.1097/BPO.0b013e3182694f18.
19
Park CH, Kim TH, Haam SJ, et al. Asymmetric Pectus Excavatum Is Associated with Overgrowth of Ribs Rather Than Cartilage[J]. Thoracic & Cardiovascular Surgeon, 2014, 63(5): 427-432. DOI:10.1055/s-0034-1395394.
20
Raso VJ, Lou E, Hill DL, et al. Trunk distortion in adolescent idiopathic scoliosis[J]. J Pediatr Orthop, 1998, 18(2): 222. DOI:10.1097/00004694-199803000-00017.
21
Redding GJ, Mayer OH. Structure-Respiration Function Relationships Before and After Surgical Treatment of Early-onset Scoliosis[J]. Clin Orthop Relat Res, 2011, 469(5): 1330-1334. DOI:10.1007/S11999-010-1621-0.
22
Nagasao T, Noguchi M, Miyamoto J, et al. Dynamic effects of the Nuss procedure on the spine in asymmetric pectus excavatum[J]. J Thorac Cardiovasc Surg, 2010, 140(6): 1294-1299. DOI:10.1016/j.jtcvs.2010.06.025.
23
Tomaszewski R, Wiktor Ł, Machała L. Evaluation of thoracic vertebrae rotation in patients with pectus excavatum[J]. Acta Orthop Traumatol Turc, 2017, 51(4): 284-289. DOI:10.1016/j.aott.2017.03.005.