Hudgins R J, Boydston W R
Division of Neurosurgery, Scottish Rite Children's Medical Center, Atlanta, Ga., USA.
Pediatr Neurosurg. 1995;23(6):323-7. doi: 10.1159/000120979.
The Chiari malformation is the leading cause of death in infants with myelodysplasia. While controversial, early operative decompression may halt or reverse the progression of symptoms. If symptoms recur after initial improvement, attention is directed to assuring shunt patency and the absence of hydromyelia and not to reassessment of the area of bone decompression. Three infants are presented who initially improved after Chiari decompression only to have recurrence of symptoms several months after surgery. In each case, regrowth of bone at the site of previous decompression was documented radiographically and at surgery. In one case, regrowth of bone occurred twice. Each child stabilized or improved following further decompressive surgery. A theory of bone regrowth and a possible means of prevention are discussed. If recurrence of Chiari symptoms occurs after decompressive surgery in infants, thin section CT with bone windows through the operative site should be performed to exclude bone regrowth and recurrent compression.
Chiari畸形是脊髓发育不良婴儿的主要死因。尽管存在争议,但早期手术减压可能会阻止或逆转症状进展。如果症状在初始改善后复发,应关注确保分流管通畅以及不存在脊髓空洞症,而不是重新评估骨减压区域。本文介绍了3例婴儿,他们在Chiari减压术后最初症状有所改善,但在术后数月症状复发。在每例病例中,影像学检查及手术均证实先前减压部位有骨再生。其中1例病例发生了两次骨再生。每个孩子在进一步减压手术后病情稳定或有所改善。本文讨论了骨再生理论及一种可能的预防方法。如果婴儿减压手术后出现Chiari症状复发,应进行通过手术部位的带骨窗薄层CT检查,以排除骨再生和复发性压迫。