Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect

Pang, Dachling; Zovickian, John; Wong, Sui-To; Hou, Yong; Moes, Greg S.

doi:10.1007/s00381-013-2189-2

Cited by 96 publications

(118 citation statements)

References 21 publications

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“…In contrast to flat LDM, untethering surgery during the postnatal period is often indicated for saccular LDM because the CSF-filled mass can be a major hindrance to proper handling of the baby [1, 2, 4, 8]. In such cases, saccular LDM should be distinguished from a myelomeningocele and simple meningocele during the perinatal period [8-10].…”

Section: Discussionmentioning

confidence: 99%

“…LDM is categorized as saccular or nonsaccular (flat) based on the external skin manifestations [1, 2]. In contrast to flat LDM, untethering surgery during the postnatal period is often indicated for saccular LDM because the CSF-filled mass can be a major hindrance to proper handling of the baby [1, 2, 4, 8].…”

Section: Discussionmentioning

confidence: 99%

“…To avoid the possibility of later formation of an intradural dermoid or epidermoid cyst in the remnant stalk, previous researchers have recommended following the stalk to its attachment with the spinal cord [1, 2, 6] or, at least, performing intraoperative serial frozen sections of the stalk until no cutaneous ectodermal tissue is present in the cut stalk [7]. In the present case, we did not expose the stalk-cord attachment because we considered intraoperatively that resection of the stalk, which appeared to be a typical LDM stalk, at the L2 level near its attachment with the cord was sufficient for cord untethering and that additional laminotomy of L1 would be too invasive for a 7-day-old infant.…”

Section: Discussionmentioning

confidence: 99%

“…Limited dorsal myeloschisis (LDM) originates from incomplete disjunction between the cutaneous and neural ectoderms during primary neurulation [1, 2]. 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].…”

Section: Introductionmentioning

confidence: 99%

“…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]. …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2019

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2019

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Concepts in the neurosurgical care of patients with spinal neural tube defects: An embryologic approach

Blount

George

Koueik

et al. 2019

Birth Defects Research

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Background: The neural tube defects (NTDs) are a heterogeneous group of structural birth defects that arise from a complex array of multiple genetic and environmental factors and adversely affect the structure and function of the brain and spinal cord. Spinal NTDs are clinically more common than cranial NTDs. There remains a significant gap in linking the multiple NTD phenotypes to current genomic understanding. Methods: This article summarizes the neurosurgical clinical approach to spinal NTDs by correlating each step of embryonic development of the human nervous system with key management concepts for defects that arise at that step. Results: The NTDs are broadly classified as open or closed. Open defects include myelomeningocele (MMC), encephalocele, and anencephaly. Closed defects are also known as occult spinal dysraphism and are characterized by intact skin over the spinal defect. They are more common and often cause neurologic decline from tethered cord syndrome. Failure of primary neurulation gives rise to open myelomeningocele (MMC). Surgical closure of an open MMC focuses on realigning the tissue layers that failed to separate during neurulation. In utero closure is a promising recent technique. Chronic neurosurgical management largely focuses treating hydrocephalus. The Chiari II malformation is uniformly present in MMC patients and may cause brainstem dysfunction. Tethered spinal cord may progressively impair normal neurologic function but typically responds well to surgical untethering. Conclusions: Surgical closure of MMC centers on approximated realignment of embryologically disordered neural tissue. Clinical surgical management decisions in the spinal NTDs remains challenging but standardized principles have emerged. K E Y W O R D S hydrocephalus, myelomeningocele, neurosurgery, spina bifida, spinal lipoma, tethered spinal cord

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A case of prenatally diagnosed limited dorsal myeloschisis with good prognosis

Lafitte¹,

Blouet

Belloy

et al. 2017

J of Clinical Ultrasound

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Diagnosis of fetal spinal dysraphism is a challenge. It is difficult to distinguish between a meningocele, myelomeningocele, and a recently described entity called limited dorsal myeloschisis (LDM). Although myelomeningocele is associated with a poor prognosis, LDM can have a good outcome. We present a case of prenatally diagnosed LDM. Because sonographic examination revealed a round, cystic, septated cervical mass without associated cerebral anomalies, the lesion was initially considered an isolated meningocele. Fetal MRI contributed to correct the diagnosis. A diagnostic error can lead to the wrong surgical support or even the termination of pregnancy. Therefore, we highlight the importance of fetal MRI in such cases, particularly when no cerebral abnormalities are observed on sonographic examination.

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Limited dorsal myeloschisis: a not-so-rare form of primary neurulation defect

Abstract: LDM is a distinctive clinicopathological entity and a tethering lesion with characteristic external and internal features. We propose a new classification incorporating both saccular and flat lesions.

Cited by 96 publications

References 21 publications

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

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

Concepts in the neurosurgical care of patients with spinal neural tube defects: An embryologic approach

A case of prenatally diagnosed limited dorsal myeloschisis with good prognosis

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