Limited Dorsal Myeloschisis and Congenital Dermal Sinus: Comparison of Clinical and MR Imaging Features

Editor’s Choice

These investigators retrospectively reviewed the clinical and MR imaging findings of 12 patients with limited dorsal myeloschisis and 10 patients with congenital dermal sinus. A crater covered with pale epithelium was the most common skin lesion in limited dorsal myeloschisis (83%). Infectious complications were common in congenital dermal sinus (60%), but not found in limited dorsal myeloschisis. They show that limited dorsal myeloschisis has distinct MR imaging features including a visible intrathecal tract with dorsal tenting of the cord at the tract-cord union.

Abstract

Figure 3 from paper
CDS in a 12-month-old girl who presented with fever and quadriplegia. Sagittal T2-weighted (A) and contrast-enhanced T1-weighted (B) MR images show ring-enhancing mass lesions nearly filling the entire intrathecal space (arrows), accompanied by spinal cord expansion and intramedullary thick enhancement (open arrows). There is no discernable tract in the intrathecal region on MR imaging (classified into “poorly visible”). These enhancing mass lesions (arrows) and intramedullary enhancement (open arrows) were infected epidermoid tumor and intramedullary abscess at the operation, respectively. C, Photograph obtained during the operation shows a yellowish material, representing infected epidermoid tumor.

BACKGROUND AND PURPOSE

While limited dorsal myeloschisis is a distinctive form of spinal dysraphism, it may be confused with congenital dermal sinus. The aim of this study was to describe clinical and MR imaging findings of limited dorsal myeloschisis that can distinguish it from congenital dermal sinus.

MATERIALS AND METHODS

We retrospectively reviewed the clinical and MR imaging findings of 12 patients with limited dorsal myeloschisis and 10 patients with congenital dermal sinus. Skin abnormalities, neurologic deficits, and infectious complication were evaluated on the basis of clinical information. We evaluated the following MR imaging features: visibility of the tract along the intrathecal course, attachment site of the tract, level of the conus medullaris, shape of the spinal cord, and presence of intradural lesions such as dermoid/epidermoid tumors.

RESULTS

A crater covered with pale epithelium was the most common skin lesion in limited dorsal myeloschisis (10/12, 83%). Infectious complications were common in congenital dermal sinus (6/10, 60%), whereas none were found in limited dorsal myeloschisis (P = .003). The following MR imaging findings were significantly different between the 2 groups (P < .05): 1) higher visibility of the intrathecal tract in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), 2) the tract attached to the cord in limited dorsal myeloschisis (12/12, 100%) versus various tract attachments in congenital dermal sinus, 3) dorsal tenting of the cord in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), and 4) the presence of dermoid/epidermoid tumors in congenital dermal sinus (6/10, 60%) versus none in limited dorsal myeloschisis.

CONCLUSIONS

Limited dorsal myeloschisis has distinct MR imaging features: a visible intrathecal tract with dorsal tenting of the cord at the tract-cord union. Limited dorsal myeloschisis was not associated with infection and dermoid/epidermoid tumors.

 

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Limited Dorsal Myeloschisis and Congenital Dermal Sinus: Comparison of Clinical and MR Imaging Features
jross
Jeffrey Ross • Mayo Clinic, Phoenix

Dr. Jeffrey S. Ross is a Professor of Radiology at the Mayo Clinic College of Medicine, and practices neuroradiology at the Mayo Clinic in Phoenix, Arizona. His publications include over 100 peer-reviewed articles, nearly 60 non-refereed articles, 33 book chapters, and 10 books. He was an AJNR Senior Editor from 2006-2015, is a member of the editorial board for 3 other journals, and a manuscript reviewer for 10 journals. He became Editor-in-Chief of the AJNR in July 2015. He received the Gold Medal Award from the ASSR in 2013.

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