Artikel
Ideopathic spinal cord herniation – diagnostic and therapeutic aspects
Die idiopathische spinale durale Myelonherniation – diagnostische und therapeutische Aspekte
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Veröffentlicht: | 30. Mai 2008 |
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Gliederung
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Objective: Ideopathic spinal cord herniation (ISCH) is a rare condition which, in untreated cases, leads to progressive paraparesis. Misdiagnosing the lesion as an arachnoidal cyst might lead to delayed treatment and therefore increased morbidity. With a series of 3 patients strategies for diagnosis and therapy are described and hypothesis to etiology of the disease are discussed.
Methods: Three female patients (n=3), with a mean age of 58.3 years (50 – 65 years) were operated on thoracic ISCH between 2005 and 2007. The average period between onset of symptoms and surgery was 7.2 years (2 – 16 years). Preoperatively two patients showed an apparent Brown-Séquard-syndrom, one patient complained about rapidly progressive incontinence. Surgical treatment included laminotomy or hemilaminotomy, intradural untethering from a lateral angle, repositioning the spinal cord hernia, and closure of dura mater using an artificial dura patch.
Results: Neurological examination took place preop, postop, and in average 15.2 months after surgery (5.1 – 23.2 months). Two patients presented clear improvement of the motor status. The incontinence present in one patient was completely regressive. Sensory deficity showed only modest improvement in all patients, a CSF-leak occurred in none of the patients.
Conclusions: ISCH is a rare condition, which is often misdiagnosed. With the ventral fixation of the medulla it is often assumed that there is a dorsal arachnoidal cyst. Saggital MRI with a clear indication of focal tethering and ventral irregularity is indicative of the condition. The basic difference is that between a ventral “pull” vs. a dorsal compression, leading to completely different surgical approaches. Timely treatment is desirable as with delayed surgery complete rehabilitation is not likely. Etiology of ISCH is still unknown, dysraphic developmental anomaly, traumatic genesis as well as local inflammatory processes are discussed. In the majority of patients, including our own, no underlying cause is found. A central register may be useful to gain more knowledge about the genesis of ISCH.