Abstract
Joubert Syndrome is a rare, autosomal recessive AR disorder characterized byneurologic findings as hypotonia, ataxia, physhcomotor developmental delay, abnormal respiratory pattern and abnormal eye movements. Molar tooth sign caused by cerebellar vermis hypoplasia is a neuroradiologic diagnostic criteria in cranial magnetic resonance imaging MRI . Scolisosis is common in these patients due to hypotonia during the early developmental period. In this article we presented our anesthesia management during scoliosis correction surgery in a patient with Joubert Syndrome
Keywords
Joubert syndrome scoliosis pediatric anesthesia neurophysiological monitoring neuromonitoring
References
- Joubert M, Eisenring JJ, Robb JP, Andermann F. Familial agenesis of the cerebellar vermis. A syndrome of episodic hyperpnea, abnormal eye movements, ataxia, and retardation. Neurology 2011;76:1837. [CrossRef]
- Maria BL, Quisling RG, Rosainz LC, Yachnis AT, Gitten J, Dede D, Fennell E. Molar tooth sign in Joubert syndrome: clinical radiologic, and pathologic significance. J Child Neurol 1999;14:368–76. [CrossRef]
- Saraiva JM, Baraitser M. Joubert syndrome: A review. Am J Med Gen 1992;43:726–31. [CrossRef]
- Maria BL, Boltshauser E, Palmer SC, Tran TX. Clinical features and revised diagnostic criteria in Joubert syndrome. J Child Neurol 1999;14:583–90. [CrossRef]
- Kumandas S, Akcakus M, Coskun A, Gumus H. Joubert syndrome: Review and report of seven new cases. Eur J Neurol 2004;11:505–10. [CrossRef]
- Brancati F, Dallapiccola B, Valente EM. Joubert Syndrome and related disorders. Orphanet J Rare Dis 2010;5:20. [CrossRef]
- Yang J, Huang Z, Shu H, Chen Y, Sun X, Liu W, et al. Improving successful rate of transcranial electrical motor-evoked potentials monitoring during spinal surgery in young children. Eur Spine J 2012;21:980–4. [CrossRef]
- Glover DC, Carling PN. Neuromonitoring for Scoliosis Surgery. Anesthesiology Clin 2014;32:101–14. [CrossRef]
- Padberg AM, Wilson-Holden TJ, Lenke LG, Bridwell KH. Somatosensory and motor-evoked potential monitoring without a wake-up test during idiopathic scoliosis surgery. An accepted standard of care. Spine 1998;23:1392–400. [CrossRef]
- Sloan TB, Heyer EJ. Anesthesia for intraoperative neurophysiologic monitoring of the spinal cord. J Clin Neurophysiol 2002;19:440–3. [CrossRef]
- Gibson PR. Anaesthesia for correction of scoliosis in children. Anaesth Intensive Care 2004;32:548–59.
- Castanelli DJ, Splinter WM, Clavel NA. Remifentanil decreases sevoflurane requirements in children. Can J Anesthesiology 2005;52:1064–70. [CrossRef]
- Jurgens S, Krishna M. Low dose remifentanil infusions for major spinal surgery. Anesthesia 2002;57:724–5. [CrossRef]
Abstract
Joubert Sendromu, hipotoni, anormal solunum paterni ve göz hareketleri, ataksi, psikomotor gelişme geriliği gibi nörolojik bulgularla karakterize, otozomal resesif geçişli nadir görülen nörolojik bir hastalıktır. Kranial magnetik rezonans görüntülemede serebellar vermis hipoplazisinin neden olduğu molar diş görüntüsü nöroradyolojik tanı kriteridir. Hastalarda erken gelişme dönemindeki hipotoni nedeni ile skolyoz sıklıkla görülür. Bu yazıda Joubert sendromlu bir hastada skolyoz düzeltme operasyonu sırasında anestezi yönetimimiz sunulmaktadır
Keywords
Joubert Sendromu skolyoz pediatrik anestezi nörofizyolojik monitörizasyon nöromonitörizasyon
References
- Joubert M, Eisenring JJ, Robb JP, Andermann F. Familial agenesis of the cerebellar vermis. A syndrome of episodic hyperpnea, abnormal eye movements, ataxia, and retardation. Neurology 2011;76:1837. [CrossRef]
- Maria BL, Quisling RG, Rosainz LC, Yachnis AT, Gitten J, Dede D, Fennell E. Molar tooth sign in Joubert syndrome: clinical radiologic, and pathologic significance. J Child Neurol 1999;14:368–76. [CrossRef]
- Saraiva JM, Baraitser M. Joubert syndrome: A review. Am J Med Gen 1992;43:726–31. [CrossRef]
- Maria BL, Boltshauser E, Palmer SC, Tran TX. Clinical features and revised diagnostic criteria in Joubert syndrome. J Child Neurol 1999;14:583–90. [CrossRef]
- Kumandas S, Akcakus M, Coskun A, Gumus H. Joubert syndrome: Review and report of seven new cases. Eur J Neurol 2004;11:505–10. [CrossRef]
- Brancati F, Dallapiccola B, Valente EM. Joubert Syndrome and related disorders. Orphanet J Rare Dis 2010;5:20. [CrossRef]
- Yang J, Huang Z, Shu H, Chen Y, Sun X, Liu W, et al. Improving successful rate of transcranial electrical motor-evoked potentials monitoring during spinal surgery in young children. Eur Spine J 2012;21:980–4. [CrossRef]
- Glover DC, Carling PN. Neuromonitoring for Scoliosis Surgery. Anesthesiology Clin 2014;32:101–14. [CrossRef]
- Padberg AM, Wilson-Holden TJ, Lenke LG, Bridwell KH. Somatosensory and motor-evoked potential monitoring without a wake-up test during idiopathic scoliosis surgery. An accepted standard of care. Spine 1998;23:1392–400. [CrossRef]
- Sloan TB, Heyer EJ. Anesthesia for intraoperative neurophysiologic monitoring of the spinal cord. J Clin Neurophysiol 2002;19:440–3. [CrossRef]
- Gibson PR. Anaesthesia for correction of scoliosis in children. Anaesth Intensive Care 2004;32:548–59.
- Castanelli DJ, Splinter WM, Clavel NA. Remifentanil decreases sevoflurane requirements in children. Can J Anesthesiology 2005;52:1064–70. [CrossRef]
- Jurgens S, Krishna M. Low dose remifentanil infusions for major spinal surgery. Anesthesia 2002;57:724–5. [CrossRef]
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | March 1, 2019 |
| Published in Issue | Year 2019Issue: 1 |