Cadilhac C, Fenoll B, Peretti A, Padovani J P, Pouliquen J C, Rigault P
Service de Chirurgie Pédiatrique, Hôpital Morvan, 29200 Brest.
Rev Chir Orthop Reparatrice Appar Mot. 2000 Oct;86(6):575-80.
Congenital pseudarthrosis of the clavicle is rare, only 200 cases having been reported. Based on 25 personal cases and an overview of the literature, we try to explain the etiology of this condition and the different kinds of treatment.
A retrospective analysis was performed on twenty-five children (16 females, 9 males, mean age at the end of the follow up - 11.5 yrs) from three different centers. We assessed the outcome of surgical and nonsurgical procedures, based on pain, functional ability, cosmetic results, and x-ray examination.
A family background was noted in three children. The lesion always involved the right side. Twenty patients presented a bump over the middle third of the clavicle, thirteen a foreshortened shoulder girdle, three complained of discomfort. In two cases, palpation of the clavicular area was painful. No neurovascular compressive syndrome was reported. None of the patients complained of a decrease in the range of motion or in the strength of the upper limb. X-rays showed a middle third defect. In five cases we found abnormal first ribs. Seventeen patients underwent surgery, at a mean age of 6 years and 4 months. The procedure always included excision of the pseudarthrosis at both ends and internal fixation with a wire or a plate. In only eight cases a bone graft was used. Healing was achieved in fourteen patients. Three patients needed a second surgical procedure. In these 3 cases we had not used bone grafting. All patients had a normal range of shoulder motion, except a twelve year old girl who complained of discomfort of the right upper limb. The cosmetic result was good in eleven cases, one surgical wound was noted as hypertrophic, and one developed a keloid. An asymmetry of the trunk was still noted in seven cases. The x-rays showed symmetric clavicles in ten cases. Eight patients were treated conservatively. All of them had a normal range of motion of the shoulder, six had a good cosmetic result and two cases a poor one.
According to Alldred, the anomaly results from the failed coalescence of the two primary ossification centers of the clavicle. The overview of the literature and our findings (in one case) confirm that the cartilage which covers both ends of the bone is made of growth cartilage. However, the true mechanism of the nonunion is still unknown. The three familial cases of our work suggest a possible genetic transmission of the disease. The diagnosis is based on the following criteria: right side lesion, found in infancy, without previous fracture, increasing size with growth, without major functional consequences, without neurofibromatosis or cleidocranial dysostosis symptom. X-rays or histologic examination will confirm the diagnosis showing the usual findings described above. Complications of the pseudarthrosis of the clavicle are rare and late. Conservative management appears to give good results as seen with our eight patients. However surgical treatment ensures symmetrical shoulder girdles and good function with few complications. Therefore, we recommend performing an excision of the cartilaginous caps, followed by an iliac bone graft and an internal fixation with wire. Surgical management will be preferred in symptomatic patients, in the case of major or increasing deformity, or on parental request.
先天性锁骨假关节较为罕见，仅报道过200例。基于25例个人病例及文献综述，我们试图解释该病症的病因及不同的治疗方法。
对来自三个不同中心的25名儿童（16名女性，9名男性，随访结束时的平均年龄为11.5岁）进行回顾性分析。我们基于疼痛、功能能力、外观效果及X线检查评估手术及非手术治疗的结果。
3名儿童有家族背景。病变总是累及右侧。20例患者在锁骨中1/3处有肿块，13例有肩带缩短，3例主诉不适。2例锁骨区域触诊疼痛。未报告有神经血管压迫综合征。所有患者均未主诉上肢活动范围或力量下降。X线显示中1/3处缺损。5例发现第一肋骨异常。17例患者接受了手术，平均年龄为6岁4个月。手术总是包括两端假关节切除及钢丝或钢板内固定。仅8例使用了骨移植。14例患者实现愈合。3例患者需要二次手术。这3例中我们未使用骨移植。除一名12岁女孩主诉右上肢不适外，所有患者肩部活动范围正常。11例外观效果良好，1例手术伤口为肥厚性瘢痕，1例形成瘢痕疙瘩。7例仍有躯干不对称。X线显示10例锁骨对称。8例患者接受保守治疗。他们均肩部活动范围正常，6例外观效果良好，2例效果不佳。
根据奥尔德雷德的观点，该异常是由于锁骨的两个主要骨化中心未能融合所致。文献综述及我们的发现（1例）证实覆盖骨两端的软骨由生长软骨构成。然而，骨不连的真正机制仍不清楚。我们研究中的3例家族病例提示该病可能有遗传传递。诊断基于以下标准：右侧病变，在婴儿期发现，无既往骨折史，随生长增大，无重大功能后果，无神经纤维瘤病或锁骨颅骨发育不全症状。X线或组织学检查将通过显示上述常见表现来确诊。锁骨假关节的并发症罕见且出现较晚。如我们的8例患者所示，保守治疗似乎效果良好。然而手术治疗可确保肩带对称且功能良好，并发症较少。因此，我们建议切除软骨帽，随后进行髂骨移植及钢丝内固定。对于有症状的患者、存在严重或逐渐加重的畸形时或应家长要求，手术治疗将为首选。
