Ailawadhi Pankaj, Mahapatra A K
Department of Neurosurgery, Cardio-Neuro Centre, AIIMS, New Delhi, India.
Pediatr Neurosurg. 2011;47(5):372-5. doi: 10.1159/000336752. Epub 2012 May 25.
Composite-type split spinal cord malformation (SCM) is very rare and results from 2 separate foci of ectoendodermal adhesions and endomesenchymal tracts leading to the development of SCM with an intervening normal cord in the same patient. Posterior spurs are even rarer. We report a unique case of composite SCM, where the patient had 3 posterior bony spurs and 1 fibrous spur at different levels. To our knowledge, this is the first reported case of a patient having composite-type SCM with posterior spurs at 3 different levels.
A 3-year-old girl presented to us with progressively worsening scoliosis. Neurological examination was unremarkable except for mild weakness in the left lower limb. Three-dimensional CT imaging demonstrated posterior bony spurs located at T(6), T(12) and L(3) levels. The patient underwent T(11)-L(5) laminoplasty and T(5)/T(6) laminectomy and excision of SCM type 1 with division of the thickened filum terminale. All the bony spurs arose from the inner surface of the lamina, where the base of the spur was located.
The unique point of this case report is the rarity of such a presentation. Careful planning and knowledge of the occurrence of such conditions can make surgery in these children safe, and all the levels can be treated in a single setting.
复合型脊髓纵裂畸形（SCM）非常罕见，由外胚层内胚层粘连和内间质束的两个独立病灶引起，导致同一患者出现脊髓纵裂畸形且中间有一段正常脊髓。后突更为罕见。我们报告了一例独特的复合型SCM病例，该患者在不同节段有3个后骨突和1个纤维突。据我们所知，这是首例报道的在3个不同节段有后突的复合型SCM患者。
一名3岁女孩因进行性加重的脊柱侧弯前来就诊。除左下肢轻度无力外，神经系统检查无异常。三维CT成像显示后骨突位于T(6)、T(12)和L(3)节段。患者接受了T(11)-L(5)椎板成形术、T(5)/T(6)椎板切除术以及1型脊髓纵裂畸形切除术，并切断增厚的终丝。所有骨突均起自椎板内表面，即突的基部所在位置。
本病例报告的独特之处在于这种表现罕见。仔细规划并了解此类情况的发生，可使这些儿童的手术安全进行，且所有节段可在一次手术中得到治疗。
