什么是胸腔功能不全综合征？
胸腔功能不全综合征是胸壁畸形that interferes with lung function and development. Children with thoracic insufficiency syndrome are born with the condition, which tends to get worse as they grow. In most cases, they are also born with先天性脊柱侧弯。较少的胸腔功能不全综合征与Kybhosis或诸如Jeune综合征等疾病。许多患有胸腔功能不全综合征的儿童缺少肋骨或肋骨融合在一起。
胸腔功能不全综合征is rare. Fewer than 4,000 children are born with the condition in the U.S. each year. However, it is a serious condition that requires close monitoring and treatment.
What causes thoracic insufficiency syndrome?
大多数胸腔功能不全综合征病例与先天性脊柱侧弯有关。严重的儿童脑盲症也会扭曲胸壁的形状并干扰肺部。
Other, less common causes include:
- skeletal dysplasiaorachondroplasia，，，，also known as dwarfism or short stature
- 引起脊柱和胸部畸形的神经肌肉疾病，例如肌营养不良症
- 脊柱裂，一种影响脊柱和脊髓的先天缺陷
是否与脊柱侧凸或条件nother problem, children with thoracic insufficiency have small chest cavities and usually lean significantly to one side due to the associated scoliosis. Because their lungs do not have enough room to function, children with thoracic insufficiency syndrome have a hard time breathing. Further, their small chest cavities do not give their lungs space to develop as they should.
胸腔功能不全综合征的症状是什么？
Symptoms of thoracic insufficiency syndrome usually appear in early childhood and get worse over time. Symptoms include:
- shortness of breath
- getting winded from easy activities
- fast, shallow breathing, even at rest
- 呼吸问题引起的睡眠破坏
How is thoracic insufficiency syndrome diagnosed?
胸腔功能不全综合征是lmost always diagnosed when children are younger than 5 years old. The physician will start with a medical history, asking about your child’s symptoms and family health history. During a physical exam, the physician will assess your child’s spine and the size and shape of their chest, checking for visible signs of deformity. The physician may also order a series of tests to confirm the diagnosis.
- CT或CAT扫描（计算机断层扫描）使用成像和计算机技术的组合来产生骨骼，肌肉，脂肪和器官的详细横截面图像。
- MRI（磁共振成像）使用大磁铁，无线电频率和计算机的组合来制作体内器官和结构的详细图像。MRI所需的时间比CT扫描更长，但产生更详细的图像，可以帮助识别或排除骨骼异常。
- 一个pulmonary function test在灵感和到期期间测量空气和氧气的流动。
How is thoracic insufficiency syndrome treated?
如果您的孩子由于胸腔功能不全综合症而呼吸困难，可以通过鼻输送或通风给予氧气。但是，长期治疗需要手术。
The most common surgical treatment is垂直可膨胀钛肋骨称为VEPTR，是胸腔功能不全综合征儿童的相对较新选择。该处理涉及将钛膨胀装置附加到孩子的肋骨笼子上，以增加其胸腔腔的大小。由于它们是可扩展的，因此随着孩子的成长，VEPTR设备可以延长。
VEPTR处理
VEPTR治疗涉及多年来发生的一系列手术。
- 在第一次手术期间，外科医生会扩大孩子的胸部，并将钛肋骨固定在畸形上方和下方的健康肋骨上。植入设备和放置的数量取决于胸部的形状。植入后，肋骨将支撑您孩子的胸壁，以便他们的肺有起作用和发育的空间。
- 您的孩子每六个月左右就需要进行一次后续手术，以使VEPTR延长。这些手术比第一次手术不那么侵入性。通常，您的孩子在医院一晚后可以回家。
- 在您的孩子在后期至后期停止生长之后，他们将进行另一项手术，以切除钛肋骨。
经过VEPTR手术后，一些孩子有脊柱融合手术to permanently support their spine in an upright position. Your child will need to return for regular check-ups with their orthopedic surgeon throughout their teen years and into adulthood. The doctor will examine your child to ensure their chest and spine remain strong and balanced.
胸腔功能不全综合症儿童的长期前景是什么？
With VEPTR and spinal fusion surgery, many children with thoracic insufficiency syndrome are able to stand up straighter, breathe easier, and gain a level of mobility that would not be possible without treatment.
我们如何照顾波士顿儿童医院的胸腔功能不全综合症欧宝彩票平台
波士顿儿童医院的脊柱分区是美国最大，最繁忙的欧宝彩票平台儿科脊柱中心。我们是该国最早使用VEPTR来纠正胸腔功能不全综合征的计划之一。当世界各地的家庭无法在其他任何地方找到答案时，他们就会来治疗复杂的脊柱问题。
Treating thoracic insufficiency syndrome typically requires the expertise of a variety of specialties. As part of a large teaching hospital, our orthopedic surgical specialists collaborate regularly with top practitioners inpulmonary medicine，，，，神经肌肉疾病，，，，neurology，，，，and advanced nursing practice, to name a few. Thanks to the depth of our knowledge and access to the extensive resources at Boston Children’s, we successfully treat children with thoracic insufficiency syndrome, as well as many other complex spine issues.