皮瓣凹陷综合征：Sinking skin flap syndrome（Trephined综合征）

本期病例选自AuntMinnie.com

Our appreciation is extended to Dr. Anthony K. Guzman, University of Pennsylvania Department of Radiology, for contributing this case.

病史、CT图像

病史

A 62-year-old man with a history of right craniectomy presents with headache, poor concentration, left-sided weakness, and facial droop.

62岁女性，右侧颅骨切除病史。现表现为头痛、注意力不集中、左侧肢体乏力、面部下垂。

1.A mass effect is present.

患侧可见占位效应。

True

False

2.There is midline shift.

中线结构有移位。

True

False

3.CT shows evidence of an acute ischemic event.

CT可见急性缺血性改变。

True

False

更多问题……

4.Psychiatric symptoms are commonly seen with this condition.

 该类患者常出现精神症状。

True

False

5.Which of the following have been implicated in the pathophysiology of this condition?

 该病可能与以下哪种病理生理改变有关？

Reduction of cerebral blood flow 脑血流减少

Alteration of cerebrospinal fluid flow dynamics 脑脊液流动情况改变

Alteration in glucose metabolism 葡萄糖代谢发生改变

Direct compression of neurons causing local dysfunction 神经元直接受压导致局灶性功能异常

All of the above

6.What is the preferred mode of management?

  下列哪项是最合适的处理方法？

Establishment of a ventriculoperitoneal shunt 建立脑室-腹腔分流

Early cranioplasty 早期颅骨成形术

Conservative management 保守治疗

Late cranioplasty 晚期颅骨成形术

7.Cranioplasty has not been shown to correct abnormal cerebral perfusion.

 颅骨成形术不能改善脑血流灌注的异常。

True

False

选择题答案：

1. True

2. True

3. False

4. True

5. All the above

6. Early cranioplasty

7. False

影像表现与诊断

CT表现

• Prior right craniectomy

  右侧颅骨切除术后改变

• Marked concavity of the overlying skin flap

  颅骨缺如处覆盖的皮瓣明显凹陷

• Mass effect and partial effacement of the underlying cerebral sulci

  可见占位效应，脑沟局限性变浅

• Minimal leftward midline shift

  中线结构轻度移位

鉴别诊断

From a clinical standpoint, the syndrome may mimic an alternate etiology of the neurological deficit, such as an intracranial infection, stroke, toxic-metabolic disorder, seizure, or hydrocephalus.

从临床的角度来说，需要与其他可能引起神经功能障碍的疾病相鉴别，包括感染、卒中、代谢性疾病、癫痫、或脑积水。

诊断

Sinking skin flap syndrome (syndrome of the trephined)

皮瓣凹陷综合征（Trephined综合征）

诊断要点

病理生理

• The forces of atmospheric pressure and gravity overwhelm intracranial pressure after the removal of a portion of overlying cranium. Loss of volume and pressure in the air-tight closed cranial cavity is an additional factor creating pressure imbalance.

  颅骨板部分切除后，大气压力和重力作用超过了颅内压。密闭颅腔内的容积和压力减小是颅内外压力失衡的另一原因。

• Reduction of cerebral blood flow, dysregulation of postural blood flow, and alteration of cerebrospinal fluid flow dynamics also are contributory; these changes may be reversed with cranioplasty.

  脑血流量的减少、体位性血流异常、脑脊液流动状态异常等情况也可引起Trephined综合征。但这些异常在颅骨成形术后可以得到恢复。

• Orthostatic positioning has been described to exacerbate the condition.

  据报道，直立位时症状加重。

• Some authors have suggested that cerebral blood flow dysregulation also alters cerebral glucose metabolism.

  部分作者认为：脑血流灌注失调可以使大脑的葡萄糖代谢发生改变。

临床表现

• Patients present with nonspecific neurologic symptoms, including contralateral hemiparesis, headache, vertigo, tinnitus, fatigue, poor concentration, memory loss, dysphagia, and apraxia.

  患者通常表现为非特异性神经系统症状，包括轻偏瘫、头痛、眩晕、耳鸣、疲劳、注意力不集中、记忆力丧失、吞咽困难、精神性失用。

• Visual disturbances, such as field defects, blurry vision, and diplopia, are common.

  视觉障碍也很常见，如视野缺损、视物模糊、复视等。
  

• Psychiatric symptoms, such as depression and anxiety, may be seen.

  偶尔也可出现精神症状，如抑郁、焦虑等。

• Severe cases may present with seizure and a comatose state.

  重症患者可表现为癫痫发作或昏迷。

CT表现

• Prior craniectomy

  颅骨部分切除

• Marked concavity of the overlying skin flap

  皮瓣明显凹陷

• Mass effect and effacement of the underlying cerebral sulci

  占位效应，局部脑沟变浅

• Midline shift

  中线移位

治疗

• Early cranioplasty typically results in normalization of the underlying pressure imbalance and recovery of neurologic function.

  早期颅骨成像术可以可以使颅内外压力平衡，神经功能症状恢复。

• Improvements in memory, motor function, and concentration seen within the first week postoperatively.

  通常术后一周即可有记忆力、运动功能、注意力的改善。

• Cranioplasty also improves cerebral perfusion.

  颅骨成形术可改善脑血流灌注。

来源：双语学影像

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