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Skin covered myelomeningoceles do not exist: What are these lesions?

Background

The author reports a clinical experience of congenital midline skin covered lesions and discusses the diagnostic approach, neuro imaging, operative management, and follow-up, with particular emphasis on patients with terminal myelocystocele.

Materials and methods

During the period of August 1977 through December 2004, the author evaluated children with skin covered spinal lesions, and made the diagnosis of: meningocele (6), lipomas and lipomeningoceles (42), hamartomas (9), teratomas (6), and terminal myelocystocele (8).

Neuro Imaging

The patients were initially diagnosed by either spinal high resolution ultrasonography, spinal computed tomography (CT), or spinal magnetic resonance imaging (MRI). All the patients with terminal myelocystocele underwent preoperative and postoperative spinal MRI.

Operative Findings

All patients underwent operative confirmation of the clinical diagnosis at the time of corrective surgery for the underlying disease. The patients with terminal myelocystocele underwent release of the tethered cord, radical but subtotal resection of the lipoma, and myelotomy and drainage of the syringocele with microlaser neurosurgery.

Results

Of the 8 patients with terminal myelocystocele, 4 had associated abdominal wall defects (i.e., bladder exstrophy) and 4 did not. Patients without abdominal wall defects were diagnosed earlier in life and underwent operative intervention sooner than those with abdominal defects. The former had better neurological outcome and less operative interventions. These findings will be presented.

Conclusion

There is a wide variety of congenital midline skin covered lesions in the dorsal medial embryo. Correct diagnosis enables an earlier and more effective management. Terminal myelocystoceles are a distinct clinical entity that is at risk of having a late diagnosis and consequently a delay in treatment.

Acknowledgements

This report has been supported in part by the Foundation for Pediatric and Laser Neurosurgery, Inc. and the Lucy B. Gooding Charitable Foundation Trust.

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Correspondence to Hector James.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/2.0 ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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James, H. Skin covered myelomeningoceles do not exist: What are these lesions?. Fluids Barriers CNS 2, S20 (2005). https://doi.org/10.1186/1743-8454-2-S1-S20

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Keywords

  • Teratoma
  • Lipoma
  • Subtotal Resection
  • Abdominal Wall Defect
  • Meningocele