Masses, Fistulas and Cutaneous Anomalies of the Back Minimal Spinal Dysraphism, Mild Forms of Spinal Dysraphism (Spina Bifida Occulta) |
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Incidence | |
Together, anencephaly, encephalocele and myelomeningocele have a total incidence of one in 1000 births in Switzerland; the incidence of minimal spinal dysraphism is at least one forth of the above mentioned CNS anomalies. | |
Clinical significance | |
1. In contrast to the common dysraphism, the incidence of which is decreasing due to a preconceptional prophylaxis with folic acid and mainly due to prenatal ultrasound, minimal spinal dysraphism is of major clinical significance today. | |
Etiology | |
The etiology is almost the same as in myelomeningocele, with a combination of familial predisposition and external injury during early pregnancy by noxious factors. | |
Pathology, anatomical types | Illustrations |
a) Lipomeningocele. Isolated intraspinal lipoma. | show details |
Pathophysiology | |
The primary malformation leads to a chronic progressive or acute neurological deficit during growth or following a trauma due to compression and/or traction of the conus. |
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Clinical presentation (history, findings, clinical skills) | Illustrations |
In more than 2/3 of cases, recognizable findings in the lumbosacral region are observed; these cutaneous malformations may indicate mild forms of spinal dysraphism, needing further evaluation. | show details |
Natural history | |
With increasing age most patients become symptomatic either following the neonatal period at the earliest, or during adulthood at the latest. | |
Differential diagnosis | Illustrations |
The differential diagnosis depends on the externally recognizable findings at the back, and the neurological deficits. | show details |
Work-up examinations | Illustrations |
In case of externally recognizable findings in the lumbosacral region, a precise history of micturation and defecation, and orthopedic and neurological clinical examinations are necessary, as well as x-rays of the vertebral column and sacrum. | show details |
Therapy | Illustrations |
If the diagnosis of minimal spinal dysraphism without/with neurological deficit is confirmed, a surgical revision is indicated: Removal of the anomalies leading to compression and traction (tethering) of the spinal cord, and of the other anomalies, e.g. dermal sinus and dermoid cyst with the danger of ascending meningitis and intradural abscess. | show details |
Prognosis | |
It seems that asymptomatic infants operated on in the first three months of life remain so. In symptomatic cases the findings remain stable in 2/3 of the cases following surgery, and 1/3 either ameliorate or become worse.
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