Slender Stalk with Combined Features of Saccular Limited Dorsal Myeloschisis and Congenital Dermal Sinus in a Neonate

Abstract

Limited dorsal myeloschisis (LDM) and congenital dermal sinus (CDS) originate from incomplete disjunction between the cutaneous and neural ectoderms. Some LDM stalks have been found to have elements of a CDS or dermoid cyst. We surgically treated a saccular lesion in the lumbosacral region of a 7-day-old male neonate. Although fetal magnetic resonance imaging (MRI) failed to reveal a stalk, postnatal MRI including three-dimensional heavily T2-weighted imaging demonstrated a stalk originating from the lumbar cord and extending caudally to enter the lumbosacral meningocele sac. During untethering surgery, we found that the stalk was slender, with a diameter of 0.7–0.8 mm, but otherwise appeared to be a typical LDM stalk. Histopathological examination revealed that the fibrocollagenous stalk contained glial fibrillary acidic protein-immunopositive neuroglial tissues and stratified squamous epithelium. The present report describes the first documented case of a stalk with combined features of saccular LDM and CDS in a neonate. Since cutaneous ectodermal tissue is likely to remain in the remnant stalk, this patient requires careful monitoring to detect the potential development of a dermoid cyst.

Neurosurgical Pathology and Management of Limited Dorsal Myeloschisis Associated with Congenital Dermal Sinus in Infancy

“…Only a few reports have discussed the incidence of the coexistence of LDM and CDS because the pre- and intraoperative diagnosis of the coexistence of LDM and CDS is difficult, especially in infants [9-11]. This difficulty was demonstrated in patients 2 and 3 of the present study.…”

“…a Preoperative 3D-hT2WI (slice thickness 1.0 mm) at birth reveals a slender stalk starting from the dorsal aspect of the conus at the L2 level and traveling down to the meningocele sac at the S1 to S2 level. This image is 1.0 mm lateral to the left side to the image in Figure 1d of our previous report [11]. b Follow-up 3D-hT2WI (slice thickness 1.25 mm) at the age of 6 months reveals two tumors enlarging in spherical shape at the remnant stalk.…”

“…We previously reported the clinicopathological findings at the initial surgery in patient 3, a boy with saccular LDM [11]; therefore, only the postoperative course is described in detail here. At birth, this boy was diagnosed with a slender stalk starting from the dorsal aspect of the conus at the L2 level and traveling down to the meningocele sac at the S1 to S2 level (Fig.…”

“…Histopathologically, however, stratified SE was present in the intra- and extradural portions of the stalk. At the proximal severed end of the stalk, GFAP-immunopositive neuroglial tissues were noted in parallel with the SE [11].…”

“…To establish the most appropriate surgical strategy for LDM associated with CDS in infancy, we herein highlight the detailed clinicopathological findings of the LDM stalk with CDS, especially the histopathological distribution of SE-lined CDS or dermoid cyst in the LDM stalk, in 3 patients. We described a case of LDM with CDS in a previous report [11], and our recent experience with 2 additional cases prompted us to report the comprehensive clinicopathological findings of all 3 cases.…”