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Case No. 328
2014-01-01~2014-01-31
출제자 : 최진욱
Hit : 418
| Period | 2014-01-01~2014-01-31 |
|---|---|
| Diagnosis | Achondroplasia |
| Clinical information | M/7months 상기 환자 출생시 모양이상, hydrocephalus진단받고 V-P shunt 위해 입원함 진단은? |
| Discussion | Achondroplasia manifestation in craniovertebral junction, Cranium, and Brain Autosomal dominant disorder that is the most common cause of short limb dwarfism Inhibition of enchondral bone formation Disharmonic development of cranium - large skull with small base and stenosis of foramen magnum Compression of medullar and upper cervical cord - neurological and respiratory complication Imaging findings - Macrencephaly - Stenois of foramen magnum - Hydrocephalus, enlarged subarachnoid space, cortical venous and sinus dilatation and anastomotic channel - Obstruction of 4th ventricular outlet - Elevation of venous sinus pressure due to stenosis of jugular foramen - Upward displacement of brainstem , vertical course of straight sinus, increased tentorial and optic base angle |
| Correct answer |
Correct Answer
Semi-Correct Answer |