scholarly journals Anesthetic Considerations for Sjogren-Larsson Syndrome

2020 ◽  
Vol 30 (12) ◽  
pp. 1390-1395
Author(s):  
Marcellene H. Franzen ◽  
Michelle M. LeRiger ◽  
Kaitlyn P. Pellegrino ◽  
Jane A. Kugler ◽  
William B. Rizzo

2020 ◽  
Vol 24 (1) ◽  
pp. 105-107
Author(s):  
Sedighe Shahhosseini ◽  
Reza Aminnejad ◽  
Amir Shafa ◽  
Mehrdad Memarzade

Carvajal syndrome is a rare genetic disorder. Patients reporting for surgery pose some difficulties in anesthesia management. In this case report we present the case of a 12-year-old boy, who was a known case of Carvajal syndrome, referred for surgical resection of perianal condyloma. Close monitoring of hemodynamic status is the mainstay of anesthetic considerations in such patients. As in any other challenging scenario, it should be kept in mind that ‘there is no safest anesthetic agent, nor the safest anesthetic technique; there is only the safest anesthesiologist’. Citation: Shahhosseini S, Aminnejad R, Shafa A, Memarzadeh M. Anesthesia in Carvajal syndrome; the first case report. Anaesth pain intensive care 2020;24(1):___ DOI: https://doi.org/10.35975/apic.v24i1.


2021 ◽  
Vol 56 (2) ◽  
pp. 163-165
Author(s):  
George Georgoulis ◽  
Argyrios Dinopoulos ◽  
Emmanouil Gkliatis

Introduction: Study of muscle tone in individuals with severe spasticity (Modified Asworth Scale – MAS:3) under general anesthesia can confirm or rule out the eventual necessity of the impending spasticity relieving ablative neurosurgery by observing the hypertonia reduction and passive range of motion expansion. Therefore, what we measure under muscle relaxants is practically a fixed deformity. Case Presentation: The study was performed on a girl with Sjögren-Larsson syndrome, presenting with icthyosis and spastic diplegia. Proposed intervention was Dorsal Rhizotomy. Under general anesthesia, with and without muscle relaxants, hypertonia was significantly reduced (MAS:1), but the angle of motion did not increase much. Conclusion: We decided not to perform such a neurosurgical procedure. In ambiguous situations, the proposed study can help in decision-making for spasticity treatment.


Author(s):  
Montserrat Fontanals ◽  
Glenn Merritt ◽  
Plinio Sierra ◽  
Gastón Echaniz

2006 ◽  
Vol 89 (1-2) ◽  
pp. 111-115 ◽  
Author(s):  
J GLOERICH ◽  
L IJLST ◽  
R WANDERS ◽  
S FERDINANDUSSE

2012 ◽  
Vol 28 (10) ◽  
pp. 1259-1265 ◽  
Author(s):  
Kathleen Davis ◽  
Kenton R. Holden ◽  
Dana S’Aulis ◽  
Claudia Amador ◽  
M. Gisele Matheus ◽  
...  

2002 ◽  
Vol 2 (4) ◽  
pp. 223-232
Author(s):  
Arvind Rup Singh ◽  
Jai Rup Singh ◽  
Harshinder Kaur ◽  
Gurpal Singh Sachdeva ◽  
Anupam Kaur ◽  
...  
Keyword(s):  

1973 ◽  
Vol 17 (4) ◽  
pp. 354???355
Author(s):  
M. LlPPMANN ◽  
P. H. LORHAN

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