Background

The meningocele is a developmental anomaly of meningothelial elements displaced into the skin and subcutaneous tissue. It is defect of the neural tube, the embryonic structure that gives rise to the spinal cord and vertebral column. This defect leads to protrusion of the spinal cord and its coverings through a defect in the skin.

The condition is often associated with disruption of the cerebrospinal fluid pathways with a resulting hydrocephalus. The degree of neurological deficit is directly relates to the level of the spinal cord defect and its extent. If only the bottom of the spinal cord is involved (conus), there may be only bowel and bladder dysfunction, while the most extensive lesions can result in total paralysis of the legs with accompanying bowel and bladder dysfunction.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

 

EPIDEMIOLOGY	CHARACTERISTICS
INCIDENCE	1/1000 live births

 

PATHOGENESIS	CHARACTERIZATION
Embryonic defect	Begins before the 4th week of gestation since neurulation (the normal closing process of the fetus's brain and spinal cord) is normally complete by that time Uncertain whether this disorder represents a failure of neurulation in the base of the spinal cord, or a rupture there after neurulation has become complete

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General
VARIANTS
Rudimentary meningocele	Arch Dermatol 2001;137:45-50 Appears to result from abnormal attachment of the developing neural tube to skin Majority of cases had no underlying bony defect or communication to the meninges
SKIN CHANGES
Skin Markers of Occult Spinal Dysraphism in Children A Review of 54 Cases David Guggisberg, MD; Smaïl Hadj-Rabia, MD; Caroline Viney, MD; Christine Bodemer, MD, PhD; Francis Brunelle, MD; Michel Zerah, MD, PhD; Alain Pierre-Kahn, MD; Yves de Prost, MD; Dominique Hamel-Teillac, MD	Arch Dermatol. 2004;140:1109-1115. Objectives  To verify the diagnostic value of lumbosacral midline cutaneous lesions in asymptomatic children to detect occult spinal dysraphism (OSD) and to propose a practical approach for clinical investigations with respect to the type of cutaneous lesions observed. Design  Retrospective study of 54 children referred to the Department of Pediatric Dermatology between 1990 and 1999 for congenital midline lumbosacral cutaneous lesions. Setting  The private or institutional practices of participating dermatologists and pediatricians. Main Outcome Measures  Evaluation of the diagnostic value of midline cutaneous lesions for the detec-tion of OSD. Association of skin examination findings with spinal anomalies detected by magnetic resonance imaging or ultrasound. Results  Occult spinal dysraphism was detected in 3 of 36 patients with an isolated congenital midline lesion and 11 of 18 patients with a combination of 2 or more different skin lesions. Conclusions  A combination of 2 or more congenital midline skin lesions is the strongest marker of OSD. Careful dermatologic examination is needed to detect suggestive markers and request a spinal magnetic resonance image, which is the most sensitive radiologic approach to detect an OSD.

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General
VARIANTS
Rudimentary meningocele	Arch Dermatol 2001;137:45-50 Meningeal tissue often simulates vascular or connective tissue Loose network of pseudovascular spaces Rare psammoma bodies may be present Meningocytes may encircle collagen bundles Decrease or increase in adnexal structures, similar to epidermal nevi or aplasia cutis Occasional syncytial giant cells caused by fusion of meningocytes

 

PROGNOSIS AND TREATMENT

CHARACTERIZATION

Multivitamin supplementation and risk of birth defects. Werler MM, Hayes C, Louik C, Shapiro S, Mitchell AA. Slone Epidemiology Unit, School of Public Health, Boston University, MA, USA.

Am J Epidemiol 1999 Oct 1;150(7):675-82 Abstract quote It is widely accepted that supplementation with folic acid, a B vitamin, reduces the risk of neural tube defects (NTDs). This case-control study tested the hypothesis that multivitamins reduce risks of selected birth defects other than NTDs. Infants with and without birth defects and aborted fetuses with birth defects were ascertained in the greater metropolitan areas of Boston, Philadelphia, and Toronto during 1993-1996. Mothers were interviewed within 6 months after delivery about a variety of factors, including details on vitamin use. Eight case groups were included: cleft lip with or without cleft palate, cleft palate only, conotruncal defects, ventricular septal defects, urinary tract defects, limb reduction defects, congenital hydrocephaly, and pyloric stenosis (n's ranged from 31 to 186). Controls were 521 infants without birth defects (nonmalformed controls) and 442 infants with defects other than those of the cases (malformed controls). Daily multivitamin supplementation was evaluated according to gestational timing categories, including periconceptional use (28 days before through 28 days after the last menstrual period). Odds ratios (ORs) below 1.0 were observed for all case groups except cardiac defects, regardless of control type. For periconceptional use, ORs with 95% confidence intervals that excluded 1.0 were estimated for limb reduction defects using both nonmalformed controls (OR = 0.3) and malformed controls (OR = 0.2) and for urinary tract defects using both nonmalformed controls (OR = 0.6) and malformed controls (OR = 0.5). Statistically significant ORs for use that began after the periconceptional period were observed for cleft palate only and urinary tract defects. These data support the hypothesis that periconceptional vitamin supplementation may extend benefits beyond a reduction in NTD risk. However, other than folic acid's protecting against NTDs, it is not clear what nutrient or combination of nutrients might affect risk of other specific defects.

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Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
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DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated September 21, 2004

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