Continuing Education Column Dong Seok Kim, MD Department of Neurosurgery, Yonsei University College of Medicine E - mail : dskim33@yuhs.ac J Korean Med Assoc 2009; 52(1): 78-90 Abstract Spinal dysraphism is a common birth defect that causes different kinds of secondary impairments, including joint deformities, reduced mobility, and bowel or bladder dysfunction. Various dysraphic spinal abnormalities result in tethered cord syndrome, a progressive form of neurological deterioration that results from spinal cord tethering. The surgery and management of children who have spinal dysraphism require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of spinal dysraphism in infancy. Keywords: Spinal dysraphism; Neural tube defect; Spina bifida 78
A B C D E F G Figure 1. Diagram of spina bifida and MR axial view. (A, E): normal, (B): spina bifida occulta, (C, F): spina bifida cytica-meningocele, (D, G): spina bifida cytica-myelomeningocele. Table 1. Congenital neurologic malformation depending on postovulatory age Stage (s) Postovulatory age (days) Normal development Malformation 8 18 Neural plate Craniorachischisis 9 20 Neural folds Craniorachischisis 10 22 First fusion of neural folds Craniorachischisis 11 24 Closure of rostral neuropore Anencephaly 12 26 Closure of caudal neuropore Myelomeningocele 13~23 28~56 Secondary neurulation and Skin-covered lesions retrogressive differentiation such as lipomyelomeningocele *Neurosurg Clin North Am, 1995: p184 79
Kim DS Table 2. Congenital spinal malformation due to abnormal early neural development Pathogenesis Incomplete disjunction of ectoderm Abnormal closure of neural tube Early disjunction of neural tube while primary neurulation Abnormal gastrulation Abnormal secondary neurulation Malformation Dermal sinus Anencephaly,myelomeningocele,myeloschisis Dorsal, transitional lipomyelomeningocele Ant, post complex spina bifida, spondylosis,neurenteric cyst, small intestine malformation Caudal myelocystocele, caudal lipomyelomeningocele A B C Figure 2. Spina bifida, open. (A) Myeloschisis, (B) Spina bifida cystica-meningocle, (C) Spina bifida cystica-myelomeningocle. Figure 3. Various dermatologic feature in closed spina bifida. (A) Lipoma, (B) Skin depression, (C) Capillary telangiectasis. A B C 80
Figure 4. Location of conus medullaris. (A) Postovulation 8 wks, (B) Postovulation 24 wks, (C) Birth, (D) Normal adult. 81
Kim DS A B Figure 5. (A) Dorsal lipomyelomeningocele, (B) Transitional lipomyelomeningocele. 82
Figure 6. Caudal lipomyelomeingocele Preop (left) and postop (right). 83
Kim DS A B Figure 7. Fetal sonography (left) and fetal MRI (right) showing mylelomeningocele at post gestation 22wks. Figure 8. Spina bifida cystica-myelomeningocele. 84
A B Figure 9. (A) Initial incision follows the circumferential white line formed by the junction of the arachnoid and skin, (B) Cross section of A. 85
Kim DS Table 3. Criteria for shunt Rapid progressive enlargement of ventricular size or head circumference Stridor, apnea or GE reflex due to hindbrain dysfunction CSF leakage at closure site Developmental delay Cosmetically unpleasant head shape 86
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