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

Morioka, Takato; Murakami, Nobuya; Suzuki, Satoshi; Takada, Akira; Tajiri, Seiji; Shimogawa, Takafumi; Mukae, Nobutaka; Iihara, Koji

doi:10.1159/000507867

Cited by 8 publications

(7 citation statements)

References 18 publications

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“…The exact incidence of coexisting LDM and RMC cannot be determined because of the different ages and clinical profiles among the populations; however, around 10% of LDM stalks may have CDS elements [4, 5, 7, 21]. In mixed LDM and CDS lesions, the widely accepted hypothesis for a 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 [1-5, 7].…”

Section: Discussionmentioning

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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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: Discussionmentioning

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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“…Intraoperatively, the C-LS was swollen further than estimated from the findings of the last preoperative MRI, indicating exacerbation of the infection during the waiting period for surgery. The diffusion-weighted images used for the MRI diagnosis of abscesses and dermoid cysts are often not useful for small intraspinal lesions in infants,[ 10 ] which were the case in the present study.…”

Section: Discussionmentioning

confidence: 77%

“…A congenital dermal sinus (CDS) consists of a tract lined by stratified squamous epithelium, which is thought to result from a focal premature disjunction between the neuroectoderm and cutaneous ectoderm during primary neurulation. [ 8 , 10 ] Although seemingly innocuous, a CDS may provide a pathway for infections. This can cause discharging sinus, local erythema, and induration as well as repeated meningitis or neurological dysfunction associated with infected inclusion tumors such as dermoid or epidermoid cysts.…”

Section: Introductionmentioning

confidence: 99%

“…This can cause discharging sinus, local erythema, and induration as well as repeated meningitis or neurological dysfunction associated with infected inclusion tumors such as dermoid or epidermoid cysts. [ 1 , 2 , 7 , 10 , 19 ] Spinal intramedullary abscesses are the rarest but most serious complication of a CDS, which may lead to permanent and serious neurological deficits. [ 1 , 2 , 7 , 10 , 19 ] Fifty cases of intramedullary abscesses due to a CDS in the pediatric age group were reported in 2019.…”

Section: Introductionmentioning

confidence: 99%

“…[ 1 , 2 , 7 , 10 , 19 ] Spinal intramedullary abscesses are the rarest but most serious complication of a CDS, which may lead to permanent and serious neurological deficits. [ 1 , 2 , 7 , 10 , 19 ] Fifty cases of intramedullary abscesses due to a CDS in the pediatric age group were reported in 2019. [ 19 ]…”

Section: Introductionmentioning

confidence: 99%

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Intramedullary abscess at thoracolumbar region transmitted from infected dermal sinus and dermoid through retained medullary cord

Matsubara

Murakami

Kurogi

et al. 2022

Surgical Neurology International

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Background: A retained medullary cord (RMC) is a relatively newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during secondary neurulation. A congenital dermal sinus (CDS) may provide a pathway for intraspinal infections such as repeated meningitis. Intramedullary abscesses are the rarest but most serious complication of a CDS. Case Description: We treated a female infant with an intramedullary abscess in the thoracolumbar region, which was caused by infection of the CDS. Surgery revealed that the cord-like structure (C-LS) started from the cord with the intramedullary abscess, extended to the dural cul-de-sac, and further continued to the CDS tract and skin dimple. The boundary between the functional cord and the non-functional CL-S was electrophysiologically identified, and the entire length of the C-LS (the RMC) with an infected dermoid cyst was resected. As a result, the abscess cavity was opened and thorough irrigation and drainage of the pus could be performed. Histopathological examination of the C-LS revealed an infected dermoid cyst and abscess cavity with keratin debris in the fibrocollagenous tissue. The abscess cavity had a central canal-like ependymal lined lumen (CCLELL), with surrounding glial fibrillary acidic protein (GFAP)-immunopositive neuroglial tissues. Conclusion: We demonstrated that the transmission of an infection through the RMC was involved in the development of the intramedullary abscess. A good postoperative outcome was obtained because a terminal ventriculostomy for pus drainage could be achieved by excising the nonfunctional RMC.

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Limited dorsal myeloschisis without extradural stalk continuity to coexisting congenital dermal sinus

Kawamoto

Harada²,

Fujinaga³

et al. 2022

Childs Nerv Syst

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

Cited by 8 publications

References 18 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

Intramedullary abscess at thoracolumbar region transmitted from infected dermal sinus and dermoid through retained medullary cord

Limited dorsal myeloschisis without extradural stalk continuity to coexisting congenital dermal sinus

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