Spinal lipoma
Classification according to ICD-10 | |
---|---|
Q06.8 | Other specified congenital malformations of the spinal cord |
D17.9 | Benign new formation of adipose tissue, unspecified |
ICD-10 online (WHO version 2019) |
A spinal lipoma is a very rare congenital developmental disorder with a mass of fat and connective tissue in connection with the skin of the spinal cord ( leptomeninx ) or the spinal cord , which often leads to a tethered cord .
distribution
The frequency of congenital spinal lipomas is given as 0.4-0.8 / 100,000. Both sexes are affected equally often, the mean age at the time of an operation is 5 years.
These lipomas are the most common form of spina bifida occulta.
root cause
This is due to an embryonic developmental disorder between the 25th and 48th day of pregnancy. This results in a temporally disturbed separation and closure of the skin ectoderm and neuroectoderm , whereby mesenchyme gets into a temporary opening of the neural tube and thereby forms fatty tissue.
Classification
Depending on the localization, the following forms can be distinguished:
- Lipomeningomyelocele (84%) (associated with spinal dysraphia)
- Filum terminale fibrolipoma (12%)
- Lipomyelocele, purely intradural
Clinical manifestations
Depending on the location and extent of the changes, the timing and type of clinical manifestation can vary considerably.
The decisive factor is whether dysraphy or adipose tissue infiltration inhibits the ascension of the spinal cord with a tethered cord.
At the time of surgery, 2/3 had neurological failures.
diagnosis
A fat-containing mass can be detected by means of sonography shortly after birth if there are suspicions. The more precise extent is determined by means of magnetic resonance imaging or computed tomography .
therapy
The treatment, especially the indication for an operation, requires interdisciplinary coordination.
literature
- A. Accogli, M. Pavanello, P. Accorsi, P. De Marco, E. Merello, M. Pacetti, P. Nozza, C. Fiorillo, L. Pinelli, A. Cama, A. Rossi, M. Catala, V. Capra: Spinal lipoma as a dysembryogenetic anomaly: Four unusual cases of ectopic iliac rib within the spinal lipoma. In: Birth defects research. Part A, Clinical and Molecular Teratology. Vol. 106, No. 7, July 2016, pp. 530-535, doi: 10.1002 / bdra.23489 , PMID 27087621 .
- G. Tamura, H. Ogiwara, K. Uematsu, N. Morota: Alteration in radiological subtype of spinal lipoma: case report. In: Child's nervous system: ChNS: official journal of the International Society for Pediatric Neurosurgery. Vol. 29, No. 10, October 2013, pp. 1957-1959, doi: 10.1007 / s00381-013-2121-9 , PMID 23636146 .
- A. Singh, V. Gupta, H. Singh, K. Chand: Nondysraphic intradural spinal lipoma. In: Journal of pediatric neurosciences. Vol. 4, No. 1, January 2009, pp. 55-56, doi: 10.4103 / 1817-1745.49116 , PMID 21887183 , PMC 3162845 (free full text).
Individual evidence
- ↑ a b A. J. Barkovich: Pediatric Neuroimaging. 2nd edition, Raven 1995, ISBN 0-7817-0179-1 , p. 494
- ↑ A. Pierre-Kahn, M. Zerah, D. Renier, G. Cinalli, C. Sainte-Rose, A. Lellouch-Tubiana, F. Brunelle, M. Le Merrer, Y. Giudicelli, J. Pichon, B. Kleinknecht, F. Nataf: Congenital lumbosacral lipomas. In: Child's nervous system: ChNS: official journal of the International Society for Pediatric Neurosurgery. Vol. 13, No. 6, June 1997, pp. 298-334, PMID 9272285 (review).
- ↑ C. Xenos, S. Sgouros, R. Walsh, A. Hockley: Spinal lipomas in children. In: Pediatric neurosurgery. Vol. 32, No. 6, June 2000, pp. 295-307, doi: 10.1159 / 000028958 , PMID 10971191 .
- ↑ a b A. Kumar, AK Mahapatra, GD Satyarthee: Congenital spinal lipomas: Role of prophylactic surgery. In: Journal of pediatric neurosciences. Volume 7, number 2, May 2012, pp. 85-89, doi: 10.4103 / 1817-1745.102562 , PMID 23248681 , PMC 3519090 (free full text).
- ↑ Finn MA, Walker ML: Spinal lipomas: clinical spectrum, embryology, and treatment. In: Neurosurgical focus. Vol. 23, No. 2, 2007, p. E10, doi: 10.3171 / FOC-07/08 / E10 , PMID 17961016 (review).
- ↑ DG McLone, TP Naidich: myelocystocele terminal. In: Neurosurgery. Vol. 16, No. 1, January 1985, pp. 36-43, PMID 3883218 .
- ↑ JL Emery, RG Lendon: Lipomas of the cauda equina and other fatty tumors related to neurospinal dysraphism. In: Developmental medicine and child neurology. Supplement. Volume 20, 1969, pp. 62-70, PMID 4905064 .
- ↑ Radiopaedia.org
- ↑ M. Zieger, U. Dörr, RD Schulz: Pediatric spinal sonography. Part II: Malformations and mass lesions. In: Pediatric radiology. Vol. 18, No. 2, 1988, pp. 105-111, PMID 3281107
- ↑ W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 . Vol. I, p. 530
- ↑ K. El-Ali, R. Slator, G. Solanki, A. Hockley, H. Nishikawa: Multidisciplinary management of spinal lipoma. In: Journal of plastic, reconstructive & aesthetic surgery: JPRAS. Vol. 62, No. 7, July 2009, pp. 964-968, doi: 10.1016 / j.bjps.2007.10.078 , PMID 18468502 .
Web links
- Lipomeningomyelocele. In: Orphanet (Rare Disease Database).