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Isolated thoracic (D5) intramedullary epidermoid cyst without spinal dysraphism: A rare case report.

Mishra SS, Satapathy MC, Deo RC, Tripathy SR, Senapati SB - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Contrast magnetic resonance imaging (MRI) characteristics clinched the presumptive diagnosis.Postoperatively, he improved significantly with a gain of motor power sufficient to walk without support within a span of 6 months.Spinal IECs, without any specific clinical presentation, are often diagnosed based upon intraoperative and histopathological findings, however early diagnosis is possible on complete MRI valuation.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, S. C. B. Medical College, Cuttack, Odisha, India.

ABSTRACT
Spinal epidermoid cyst, congenital or acquired, is mainly congenital associated with spinal dysraphism, rarely in isolation. Intramedullary epidermoid cysts (IECs) are rare with less than 60 cases reported so far; isolated variety (i.e., without spinal dysraphism) is still rarer. Complete microsurgical excision is the dictum of surgical treatment. A 14-year-old boy presented with 4-month history of upper backache accompanied with progressive descending paresthesia with paraparesis with early bladder and bowel involvement. His condition deteriorated rapidly making him bedridden. Neurological examination revealed upper thoracic myeloradiculopathy probably of neoplastic origin with sensory localization to D5 spinal level. Digital X-ray revealed no feature suggestive of spinal dysraphism. Contrast magnetic resonance imaging (MRI) characteristics clinched the presumptive diagnosis. Near-total microsurgical excision was done leaving behind a small part of the calcified capsule densely adhered to cord. Histopathological features were confirmative of an epidermoid cyst. Postoperatively, he improved significantly with a gain of motor power sufficient to walk without support within a span of 6 months. Spinal IECs, without any specific clinical presentation, are often diagnosed based upon intraoperative and histopathological findings, however early diagnosis is possible on complete MRI valuation. Complete microsurgical excision, resulting in cessation of clinical progression and remission of symptoms, has to be limited to sub-total or near-total excision if cyst is adherent to cord or its confines.

No MeSH data available.


Related in: MedlinePlus

Intraoperative images (a) intramedullary pearly white, avascular, soft, flaky, cheese-like suckable material being expulsed, (b) image after evacuation of cystic contents, and (c and d) histopathological images showing desquamated epithelium surrounded by keratin-producing squamous epithelium suggestive of epidermoid cyst
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Figure 4: Intraoperative images (a) intramedullary pearly white, avascular, soft, flaky, cheese-like suckable material being expulsed, (b) image after evacuation of cystic contents, and (c and d) histopathological images showing desquamated epithelium surrounded by keratin-producing squamous epithelium suggestive of epidermoid cyst

Mentions: We proceeded for D4–D5 laminectomy aiming for complete microsurgical excision. Dura was tense and bulging. Cord appeared thickened and expanded. Slightly lateral to midline the lesion was bulging and had thinned out the overlying cord. On fine dissection over the most bulging part, a pearly white, avascular, soft, flaky lesion within the cord substance was observed with gushing out of dense, cheese-like suckable material. Near-total excision of the lesion was done leaving behind the part of the capsule calcified and densely adhered to cord antero-medially [Figure 4a and b]. After hemostasis, watertight dural closure was done with wound closure layer by layer. Intraoperative diagnosis of intramedullary epidermoid cyst (IEC) was confirmed on histopathology [Figure 4c and d]. Postoperatively, with physiotherapy, and rehabilitation he had gradual progressive improvement of the preoperative neurological deficits. Follow-up MRI of spine at 1-month revealed very minimal residual [Figures 2d–f and 3d–f]. By 6 months follow-up he regained his power of both LLs to 4+/5 with minimal spasticity, and is now able to walk with support.


Isolated thoracic (D5) intramedullary epidermoid cyst without spinal dysraphism: A rare case report.

Mishra SS, Satapathy MC, Deo RC, Tripathy SR, Senapati SB - J Pediatr Neurosci (2015 Apr-Jun)

Intraoperative images (a) intramedullary pearly white, avascular, soft, flaky, cheese-like suckable material being expulsed, (b) image after evacuation of cystic contents, and (c and d) histopathological images showing desquamated epithelium surrounded by keratin-producing squamous epithelium suggestive of epidermoid cyst
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489056&req=5

Figure 4: Intraoperative images (a) intramedullary pearly white, avascular, soft, flaky, cheese-like suckable material being expulsed, (b) image after evacuation of cystic contents, and (c and d) histopathological images showing desquamated epithelium surrounded by keratin-producing squamous epithelium suggestive of epidermoid cyst
Mentions: We proceeded for D4–D5 laminectomy aiming for complete microsurgical excision. Dura was tense and bulging. Cord appeared thickened and expanded. Slightly lateral to midline the lesion was bulging and had thinned out the overlying cord. On fine dissection over the most bulging part, a pearly white, avascular, soft, flaky lesion within the cord substance was observed with gushing out of dense, cheese-like suckable material. Near-total excision of the lesion was done leaving behind the part of the capsule calcified and densely adhered to cord antero-medially [Figure 4a and b]. After hemostasis, watertight dural closure was done with wound closure layer by layer. Intraoperative diagnosis of intramedullary epidermoid cyst (IEC) was confirmed on histopathology [Figure 4c and d]. Postoperatively, with physiotherapy, and rehabilitation he had gradual progressive improvement of the preoperative neurological deficits. Follow-up MRI of spine at 1-month revealed very minimal residual [Figures 2d–f and 3d–f]. By 6 months follow-up he regained his power of both LLs to 4+/5 with minimal spasticity, and is now able to walk with support.

Bottom Line: Contrast magnetic resonance imaging (MRI) characteristics clinched the presumptive diagnosis.Postoperatively, he improved significantly with a gain of motor power sufficient to walk without support within a span of 6 months.Spinal IECs, without any specific clinical presentation, are often diagnosed based upon intraoperative and histopathological findings, however early diagnosis is possible on complete MRI valuation.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, S. C. B. Medical College, Cuttack, Odisha, India.

ABSTRACT
Spinal epidermoid cyst, congenital or acquired, is mainly congenital associated with spinal dysraphism, rarely in isolation. Intramedullary epidermoid cysts (IECs) are rare with less than 60 cases reported so far; isolated variety (i.e., without spinal dysraphism) is still rarer. Complete microsurgical excision is the dictum of surgical treatment. A 14-year-old boy presented with 4-month history of upper backache accompanied with progressive descending paresthesia with paraparesis with early bladder and bowel involvement. His condition deteriorated rapidly making him bedridden. Neurological examination revealed upper thoracic myeloradiculopathy probably of neoplastic origin with sensory localization to D5 spinal level. Digital X-ray revealed no feature suggestive of spinal dysraphism. Contrast magnetic resonance imaging (MRI) characteristics clinched the presumptive diagnosis. Near-total microsurgical excision was done leaving behind a small part of the calcified capsule densely adhered to cord. Histopathological features were confirmative of an epidermoid cyst. Postoperatively, he improved significantly with a gain of motor power sufficient to walk without support within a span of 6 months. Spinal IECs, without any specific clinical presentation, are often diagnosed based upon intraoperative and histopathological findings, however early diagnosis is possible on complete MRI valuation. Complete microsurgical excision, resulting in cessation of clinical progression and remission of symptoms, has to be limited to sub-total or near-total excision if cyst is adherent to cord or its confines.

No MeSH data available.


Related in: MedlinePlus