Cranial and Spinal Dysraphisms

A Rare Case of Basioccipital Encephalomeningocele with Tethered Dermoid Presenting as Cranial Nerve Palsy: A Case Report

“…Other uncommon types of basal encephaloceles include sphenopharyngeal, spheno-orbital, sphenomaxillary, sphenoethmoidal, transethmoidal, trans-sphenoidal or basioccipital [2]. There have been very few case reports about spheno-orbital, transethmoidal and trans-sphenoidal [4] basal encephaloceles and meningoencephaloceles, but many cases of posterior convexity occipital encephaloceles which are associated with Chiari III malformation.…”

“…The development of the normal brain and its coverings requires a sequential process of neurulation. The mesenchymal tissue forming the chondrocranium for the skull base is postulated to be derived from cells of neural crest origin [2]. Defects at the sites of convergence of the branchial arches, the chondral and membranous cranium lead to the formation of basal encephaloceles.…”

“…Spinal dysraphisms are ten times more common than cranial dysraphisms. The majority of encephaloceles and meningoencephaloceles are seen in the occipital region and they constitute almost 85% of encephaloceles [2]. Here, we report a 7-month-old infant presenting with right 6th and 7th cranial nerve palsy and found to have basioccipital (clival) encephalocele on neuroimaging.…”

Neurological disease of Şehzade Cihangir in the Ottoman history: spinal dysraphism

“…Pathological point of view, it is possible that there was an abnormal fusion of the midline embryonic neural, vertebral, and mesenchymal structures. Basically, it can be broadly divided to into two different pathological entities: open spinal dysraphism (meningocoele, myelomeningocele) and closed spinal dysraphism (dorsal dermal sinus, lipomyelomeningocele, diastematomyelia, neurenteric cyst, thickened filum terminale) [3]. In fact, history of Şehzade Cihangir helps us understand the complexity of the neurological disease because it has a full impact on humanity.…”

“…In clinical practice, some types of spinal dysraphism do not provide neurological findings, while others might result in complete loss of muscular strength and sensation and even sphincter dysfunction [3]. Today, it also is known that a deteriorated life quality and psychological problems due to associated congenital anomalies of cardiovascular and urinary systems are widespread especially among children and young adults with serious types of spinal dysraphism.…”