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Spontaneous Regression Of Lipomyelomeningocele Associated With Terminal Syearsingomyelia In A Child

Klein & Thompson, 2007Other/Unknown

Klein, O., & Thompson, D. (2007). spontaneous regression of lipomyelomeningocele associated with terminal syearsingomyelia in a child. Case report. Journal of neurosurgery, 107(3 Suppl), 244–247. https://doi.org/10.3171/PED-07/09/244

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Abstract

The authors present the case of a 3-month-old girl who presented with lipomyelomeningocele (LMM) associated with a low-lying conus and a terminal syrinx. At the time of presentation, the girl had a mild neurological, but congenital, deficit: weakness of the right foot and calf as well as absence of the right ankle reflex. Bladder assessment was normal, and it was decided to monitor this child closely, without performing surgery. Results of further magnetic resonance imaging performed 1 year after diagnosis and when the girl was 28 months old, showed a significant regression of LMM and associated syrinx. Interestingly, the child remained neurologically unchanged. Although described in the literature, spontaneous regression of LMM is a very unusual situation, and very few cases have been reported. This case represents the first report of concomitant regression of an associated syrinx. Close follow-up of nonsurgically treated children is mandatory, as the risk of late deterioration remains.

Case Details

Disease Location

Spinal cord

Personal Characteristics

3 months female

Clinical Characteristics

Clinical exam revealed a lumbosacral subcutaneous lipoma. There was a dimple over the lipoma with an intact epithelium and was not at all typical of a dermal sinus tract lipomyelomeningocele (lmm) associated with low-lying conus and a terminal syearsinx found via MRI. No evidence of chiari malformation or hydrocephalus at the time of presentation, there was a mild neurological but congenital deficit: weakness of the right foot and calf as well as absence of the right ankle reflex bladder assessment was normal first follow-up) bladder function assessment performed at 1 year of age was satisfactory, renal ultrasonography showed normal appearance of both kidneys second follow-up) 6 months later revealed a stable neurological state. The right foot was still weaker, follow-up MRI showed significant improvement with both regression of lipoma and decreased size of syearsinx third follow-up) at 2 years and 4 months old, able to walk but right leg slightly rotated externally. Stable neurological findings, no pain in back or lower limbs, no uti. MRI showed stable appearance of the lmm and syearsingomyelia (fig 4 in article).

Remission Characteristics

MRI imaging a year after diagnosis (28 months) showed a significant lmm and associated syearsinx regression, the child remained neurological unchanged.

Treatment & Mechanisms

Proposed Remission Mechanisms

Major debate, some people suggested shunting large cysts, others suggested myelotomy, drainage and/or shunt inserightion nothing major mentioned

Clinical Treatment

None reported

Non-Clinical Treatment

None reported