Retained medullary cord with sacral subcutaneous meningocele and congenital dermal sinus

Morioka, Takato; Murakami, Nobuya; Kanata, Akiko; Tsukamoto, Haruhisa; Suzuki, Satoshi

doi:10.1007/s00381-019-04301-1

Cited by 20 publications

(34 citation statements)

References 11 publications

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“…3a-3, b-2, c(1), (2), (3), (4)) and continuing to the nonfunctional portion of the C-LS (Fig. 3a-3, b-2, c(5), (6), (7)) [12]. The C-LS was severed immediately caudal to the exact border and resected.…”

Section: Case Reportmentioning

confidence: 99%

“…Because RMC is thought to be caused by the late arrest of secondary neurulation [8, 9], a concurrent RMC and CDS cannot be explained embryologically. However, we recently reported an RMC case with sacral subcutaneous meningocele and CDS, and stressed the possibility of a coexistent CDS in RMC that extended out to the extraspinal canal [12]. We operated on an additional case with CDS elements in each tethering stalk of a coexisting LDM continuous from the tail-like cutaneous appendage at the thoracic region and an RMC from the dimple at the lumbosacral region.…”

Section: Introductionmentioning

confidence: 99%

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Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord

et al. 2020

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Introduction: The embryogenesis of limited dorsal myeloschisis (LDM) likely involves impaired disjunction between the cutaneous and neural ectoderms during primary neurulation. Because LDM and congenital dermal sinus (CDS) have a shared origin in this regard, CDS elements can be found in the LDM stalk. Retained medullary cord (RMC) is a closed spinal dysraphism involving a robust, elongated, cord-like structure extending from the conus medullaris to the dural cul-de-sac. Because the RMC is assumed to be caused by impaired secondary neurulation, concurrent RMC and CDS cannot be explained embryologically. In the present article, we report a case in which CDS elements were noted in each tethering stalk of a coexisting LDM and RMC. Case Presentation: A 2.5-month-old boy with left clubfoot and frequent urinary and fecal leakage had 2 tethering tracts. The upper tract, which ran from the thoracic tail-like cutaneous appendage, had CDS elements in the extradural stalk and a tiny dermoid cyst in the intradural stalk immediately after the dural entry. In the lower tract, which ran from the lumbosacral dimple, the CDS as an extradural stalk continued to the RMC at the dural cul-de-sac. Both stalks were entirely resected through skip laminotomy/laminectomy at 1 stage to untether the cord and resect the CDS elements. Conclusion: Surgeons should be aware that CDS elements, in addition to LDM, may coexist with RMC that extends out to the extradural space.

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Section: Case Reportmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord

et al. 2020

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“…The prognosis of RMCs seems to be good. No patients have demonstrated new neurological deficits or progression after surgery in the literature 8,11,[15][16][17]19) . Since there is not much information available on RMC, it will be meaningful to compare the clinical outcomes of wide exposure with excision of the whole non-functional cord and those of limited exposure with only untethering.…”

Section: Clinical Outcomementioning

confidence: 99%

“…An RMC can, by default, be accompanied by other secondary neurulation anomalies such as presacral cysts or sacral agenesis. In the literature, caudal lipomas, limited dorsal myeloschisis, congenital dermal sinuses, subcutaneous meningoceles, and split cord malformations can be associated with RMCs 8,11,16,19) .…”

Section: Neuroimagingmentioning

confidence: 99%

Secondary Neurulation Defects-1 : Retained Medullary Cord

Kim

Lee

Wang

2020

J Korean Neurosurg Soc

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Retained medullary cord (RMC) is a relatively recent term. Pang et al. newly defined the RMC as a late arrest of secondary neurulation leaving a non-functional vestigial portion at the tip of the conus medullaris. RMC, which belongs to the category of closed spinal dysraphism, is a cord-like structure that is elongated from the conus toward the cul-de-sac. Because intraoperative electrophysiological confirmation of a non-functional conus is essential for the diagnosis of RMC, only a tentative or an assumptive diagnosis is possible before surgery or in cases of limited surgical exposure. We suggest the term 'possible RMC' for these cases. An RMC may cause tethered cord syndrome and thus requires surgery. This article reviews the literature to elucidate the pathoembryogenesis, clinical significance and treatment of RMCs.

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“…In mixed lesions of LDM and CDS, the widely accepted hypothesis for CDS is that the cutaneous ectoderm is “dragged down” toward the neural tube, whereas LDM supposedly forms by the “pulling up” of the neuroectoderm in the opposite direction toward the skin (Fig. 1d, upper row) [1-3, 9-12].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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• Because of the shared origin of limited dorsal myeloschisis (LDM) and congenital dermal sinus (CDS), CDS elements may be found within the LDM stalk. • The recommendation for excision of the entire length of the LDM stalk in all infants should be more carefully made. Novel Insights • Once the histological diagnosis of coexistent CDS is made in the resected end, the entire length of the remnant stalk should be excised as soon as possible.

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Retained medullary cord with sacral subcutaneous meningocele and congenital dermal sinus

Cited by 20 publications

References 11 publications

Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord

Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord

Secondary Neurulation Defects-1 : Retained Medullary Cord

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

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