Surgical histopathology of limited dorsal myeloschisis with flat skin lesion

Abstract

Immunopositivity for GFAP in the LDM stalk was observed in as few as 50% of our patients, despite the relatively extensive histopathological examination. We confirm that the clinical diagnosis of LDM should be made based on comprehensive histopathological examination as well as clinical manifestations. The profuse network of peripheral nerve fibers in every stalk and the high incidence of melanocyte accumulation associated with dermal melanocytosis might assist the histopathological diagnosis of LDM.

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

“…In our patient, the typical histopathological finding of an LDM stalk was noted at the most rostral side of the intradural stalk. The presence of peripheral nerve fibers in the fibrocollagenous stalk and arachnoid tissue in the meningocele sac strongly supports the diagnosis of LDM [4, 5]. Another notable histopathological finding is that almost the entire stalk, except for the stalk-sac attachment, contained stratified squamous epithelium.…”

“…The central histopathological feature of an LDM stalk is GFAP-immunopositive neuroglial tissue in the fibrocollagenous band, which is a hallmark of the stalk’s origin from the incompletely disjoined neuroectoderm [1-5, 17]. In our patient, the typical histopathological finding of an LDM stalk was noted at the most rostral side of the intradural stalk.…”

“…Its hallmark is a fibroneural stalk containing glial fibrillary acidic protein (GFAP)-immunopositive neuroglial tissues, causing tethering on the dorsal spinal cord rostral to the conus [1-5]. Treatment involves relatively straightforward resection of the LDM stalk from the spinal cord [1, 2].…”

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

“…When conservative follow-up is selected, postoperative MRI including 3D-hT2WI is needed for the detection of newly developed dermoid cysts. Although the 2- to 5-mm-thick sections provided by conventional MRI are unable to demonstrate sufficiently detailed imaging, 3D-hT2WI can provide high-resolution images with excellent contrast between solid structures and the CSF [6, 7, 16-18]. The present study revealed that 3D-hT2WI with a slice thickness of 0.50–1.25 mm clearly demonstrated the spherical enlargement of the stalk, which is the characteristic finding of development of a dermoid cyst, on the contrast with CSF [18].…”

“…Saccular LDM is characterized by a skin-based cerebrospinal fluid (CSF) sac topped by a squamous epithelial dome. Flat LDM is characterized by squamous epithelium (SE) with a flat surface or sunken crater or pit, typically called “cigarette-burn” skin lesion [6, 7].…”