Split spinal cord malformations in children

Abstract

<jats:p>The authors reviewed and analyzed information on 74 patients with split spinal cord malformations (SSCMs) treated between January 1, 1980 and December 31, 1996 at their institution with the aim of defining and classifying the malformations according to the method of Pang, et al.</jats:p> <jats:p>Computerized tomography myelography was superior to other radiological tools in defining the type of SSCM. There were 46 girls (62%) and 28 boys (38%) ranging in age from less than 1 day to 12 years (mean 33.08 months). The mean age (43.2 months) of the patients who exhibited neurological deficits and orthopedic deformities was significantly older than those (8.2 months) without deficits (p = 0.003). Fifty-two patients had a single Type I and 18 patients a single Type II SSCM; four patients had composite SSCMs. Sixty-two patients had at least one associated spinal lesion that could lead to spinal cord tethering. After surgery, the majority of the patients remained stable and clinical improvement was observed in 18 patients.</jats:p> <jats:p>The classification of SSCMs proposed by Pang, et al., will eliminate the current chaos in terminology. In all SSCMs, either a rigid or a fibrous septum was found to transfix the spinal cord. There was at least one unrelated lesion that caused tethering of the spinal cord in 85% of the patients. The risk of neurological deficits resulting from SSCMs increases with the age of the patient; therefore, all patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations.</jats:p>

Myelomeningocele Associated With Split Cord Malformation Type I -Three Case Reports-

“…Therefore, common embryologic errors may result in both MMC and SCM. 1,2,4,5,7,11,14,15) Other central nervous system anomalies, such as Chiari malformation, hydrocephalus, and syrinx, are also common in patients with SCM and MMC, so management of these disorders may be difficult. 3,9,10) Here we describe three difficult cases of SCM and MMC associated with various concomitant anomalies.…”

Duplication of spine with hemi-lipomyelomeningocele

“…It is generally argued that all patients with SCM should be surgically treated due to the concerns regarding the clinical consequences of the tethering of the cord, which is common in all of these syndromes [7,13]. However, none of the asymptomatic cases of duplication of the spinal cord reported in the literature undergo surgical management [1,5,8,10].…”

Fatty filum with different histological features. Case report

“…The unified theory proposes that all SCMs originate from one basic ontogenetic error occurring around the time when the primitive neurenteric canal closes 5 . This basic error is the formation of an "accessory neurenteric canal" through the midline embryonic disc that maintains communication between yolk sac and amnion and enables continued contact between ectoderm and endoderm within the canal.…”