Brooks B S, El Gammal T, Hartlage P, Beveridge W
AJNR Am J Neuroradiol. 1981 Jul-Aug;2(4):319-23.
In the past, neurologic deficits found in association with sacral agenesis were thought to be unamenable to surgical therapy. Recent experience and a careful review of autopsy and case reports form the literature have demonstrated that this assumption is unwarranted. Four cases of sacral agenesis are reported with description of the myelographic findings of each case. Surgical confirmation was obtained in three of these patients. Dural sac stenosis treated with duraplasty resulted in striking improvement in the neurologic status of two patients, while in the third, a 2-month-old infant, adhesive arachnoidal bands in the distal thecal sac were found at surgery and a taut and thickened filum terminale was transected. The fourth patient has a low-lying spinal cord and a posterior meningocele. The myelographic findings appear to be divisible into two categories. One group of patients may have high termination of the subarachnoid space with a dural sac stenosis and will benefit from duraplasty, while in the other, findings may include a widened or normal subarachnoid space and low-lying tethered spinal cord. It is emphasized that treatment of dural sac stenosis, tethered cord, and intrathecal or extrathecal masses that occur in some of these patients may afford significant improvement in their neurologic condition. These children deserve careful baseline neurologic evaluation and follow-up and a more aggressive approach toward adequate myelographic assessment.
过去，人们认为与骶骨发育不全相关的神经功能缺损无法通过手术治疗。近期的经验以及对文献中尸检和病例报告的仔细回顾表明，这种假设是没有根据的。本文报告了4例骶骨发育不全病例，并描述了每例的脊髓造影结果。其中3例患者获得了手术证实。2例患者采用硬脑膜成形术治疗硬脊膜囊狭窄后，神经功能状况有显著改善；而第3例患者是一名2个月大的婴儿，手术中发现硬脊膜囊远端有粘连性蛛网膜带，并切断了紧张增厚的终丝。第4例患者有低位脊髓和后位脊膜膨出。脊髓造影结果似乎可分为两类。一类患者可能存在蛛网膜下腔高位终止伴硬脊膜囊狭窄，硬脑膜成形术对其有益；而另一类患者的表现可能包括蛛网膜下腔增宽或正常以及低位脊髓栓系。需要强调的是，治疗这些患者中出现的硬脊膜囊狭窄、脊髓栓系以及鞘内或鞘外肿物，可能会使其神经状况得到显著改善。这些儿童应接受仔细的基线神经学评估和随访，并采取更积极的方法进行充分的脊髓造影评估。
