Surgery 5th year, 2nd lecture (Dr. Mazin)

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The lecture has been given on May 24th, 2011 by Dr. Mazin.

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Surgery 5th year, 2nd lecture (Dr. Mazin)

  1. 1. Congenital anomalies of the CNS<br />DR.MAZIN M.K. BOUJAN<br />2011<br />
  2. 2. INTRODUCTION<br />Phenotype: the outer look.<br />Genotype: the inner genetics.<br />1.Malformation=genetic=organ formation.<br />2.Deformity= environmental<br />-Internal (more sever, no spontaneous recovery) e.g.: Meningomyelocele=spastic muscles=club feet.<br />-External (might correct itself after the resolution of the force) e.g.: oligohydraminios(uterus force) leads to premature descent of the head and eventually coronal synostosis ).<br />3. disruption: tissue or organ destruction. (Hydrancephaly=necrosis).<br />4. a sequence= one primary defect leads to many secondary anomalies.<br />
  3. 3. Neuroembryology <br />Stages of embryonic development;<br />Human embryo has 23 stages of development (each of 2-3 days long).<br /> Stage 8 to 23 is related to CNS development.<br />
  4. 4. Stages of CNS development<br />
  5. 5. Types of CNS anomalies<br />Arachnoid cyst.<br />Neuroenteric cysts.<br />Craniosynostosis.<br />Encephalocele.<br />Chiari malformation.<br />Dandy-Walker malformation.<br />Aqueductal stenosis.<br />Neural tube defects.<br />Tethered cord syndrome.<br />Split cord syndrome.<br />Intracranial lipoma.<br />Hypothalamic hamartoma.<br />
  6. 6. 1.Arachnoid cyst<br />1.Intracranial:<br />Usually asymptomatic discovered incidentally.<br />Could be symptomatic.<br />Symptoms: Increased ICP, seizure, or hemiparesis. <br />Treatment: from shunting to excision.<br /> 2.intraspinal:almost always dorsal.<br />
  7. 7. 2.Neuroenteric cysts<br />Is a CNS cyst lined by endothelium resembling GIT or respiratory.<br />Intraspinal: is the main location, usually anterior.<br />Intracranial: few reported cases.<br />Symptom: pain or myelopathy, meningitis(in the fistula type).<br />Treatment: complete removal.<br />
  8. 8. 3.Craniosynostosis. <br />Congenital early closure of the sutures.<br />Might occur postnataly by abnormal positioning (lazy lambdoid).<br />Has many types according to the suture involved. Most well known:<br />Sagittal=scaphocephaly.<br />Unilateral Coronal= plegiocephaly. <br />Bilateral coronal = brachycephaly.<br />Treatment: indication for surgery is usually cosmetic.<br />
  9. 9. 4.Encephalocele<br />Is cranium bifidum usually a cyst in the occipital region.<br />If only contains meningies=meningocele.<br />If contain meningies and cerebral tissue = encephalocele. <br />Another group is called basal encephalocele appear at the base of the skull and could be mistaken for a nasal polyp.<br />Treatment: surgical excision and water-tight Dural suture. <br />
  10. 10. 5.Chiari malformation<br />Basically 4 types:<br />Chiari I: caudal descent of the cerebellar tonsils to the cervical canal.<br />Chiari II: caudal descent of the vermis, medulla and the 4th ventricle.<br />Chiari III: rare, cerebellar herniation + encephalocele.<br />Chiari IV: cerebellar hypoplesia. <br />Treatment: surgical decompression of the posterior fossa.<br />
  11. 11. 6.Dandy-Walker Malformation<br />Atresia of foramen Magendie and luschka.<br />Agenesis of the vermis.<br />4th ventricle communicated with a posterior fossa cyst.<br />Treatment: shunt the posterior fossa cyst. <br />
  12. 12. 7.Aqueduct stenosis<br />Triventricular hydrocephalus.<br />Normal size 4th ventricle.<br />Congenital: malformation.<br />Acquired: infection, neoplasm, arachnoids' cyst.<br />Treatment: shunting. <br />
  13. 13. 8.Spinal dysraphysm<br />Spina bifida: types;<br />Spina bifida occulta: an incidental finding of spinous process and lamina.<br />Meningocele: 1+ cystic distention of the meningies.<br />Myelomeningocele: 1+2+ spinal cord or cauda equina involvement.<br />Treatment : for 2 and 3 repair, with or without shunt operation.<br />
  14. 14. 9.Tethered cord syndrom<br />Thick filumterminale with an abnormal low level conusmedularis.<br />Presentation: Gait difficulty, visible muscle atrophy, sensory deficit, bladder dysfunction, scoliosis and kyphosis.<br />Treatment: division of the filum. <br />
  15. 15. 10.Split cord syndrom<br />Type 1: 2 cords with 2 covering dura.<br />Type 2(diplomyelia):2 cords within the same dura.<br /> treatment: untethering the cord<br />
  16. 16. 11.Intracranial lipoma<br />Usually in the midline above the corpus callosum.<br />Discovered insidentaly, or cause seizure or hydrocephalus.<br />Treatment : shunting the associated hydrocephalus. Direct surgery is rarely necessary.<br />
  17. 17. 12.Hypothalamic hamartoma<br />Masses of ectopic neural tissues.<br />Presentation: seizure, behavioral disturbance, precocious puberty, mental retardation, and visual impairment.<br />Surgical resection. <br />
  18. 18. Good luck<br />

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