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Anesthesia and perioperative management for an patient with Ulrich syndrome undergoing surgery for scoliosis

Wim Vandenberghe (UGent) , TOM JACOBS (UGent) , Frank Plasschaert (UGent) , J Willems, Nadia Den Blauwen (UGent) , HUGO VEREECKE (UGent) and Patrick Wouters (UGent)
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Abstract
Abstract : Ullrich syndrome is a rare congenital hypotonic-sclerotic muscular disorder in which affected children develop a slowly progressive scoliosis and contractures and limpness of joints. The disease causes increasingly invalidating contractures and hardening of the muscles of the neck and trunk. While this neuromuscular type of scoliosis is progressive, patients rarely attain the point of surgery due to their compromised general medical condition. This may explain the current lack of outcome data and the paucity of information on perioperative management for patients with Ullrich syndrome undergoing major surgery. The purpose of this report was therefore to describe our first experience with the perioperative and anesthetic management of a 15-year old boy presenting with Ullrich syndrome and a secondary invalidating scoliosis. The specific challenges of this condition characterized by severe restrictive lung disease and a challenging airway abnormality are discusse
Keywords
scoliosis, hypotonic-sclerotic muscular dystrophy, Ullrich, perioperative managament.

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Chicago
Vandenberghe, Wim, TOM JACOBS, Frank Plasschaert, J Willems, Nadia Den Blauwen, HUGO VEREECKE, and Patrick Wouters. 2010. “Anesthesia and Perioperative Management for an Patient with Ulrich Syndrome Undergoing Surgery for Scoliosis.” Acta Anaesthesiologica Belgica 61 (1): 43–47.
APA
Vandenberghe, Wim, JACOBS, T., Plasschaert, F., Willems, J., Den Blauwen, N., VEREECKE, H., & Wouters, P. (2010). Anesthesia and perioperative management for an patient with Ulrich syndrome undergoing surgery for scoliosis. ACTA ANAESTHESIOLOGICA BELGICA, 61(1), 43–47.
Vancouver
1.
Vandenberghe W, JACOBS T, Plasschaert F, Willems J, Den Blauwen N, VEREECKE H, et al. Anesthesia and perioperative management for an patient with Ulrich syndrome undergoing surgery for scoliosis. ACTA ANAESTHESIOLOGICA BELGICA. 2010;61(1):43–7.
MLA
Vandenberghe, Wim, TOM JACOBS, Frank Plasschaert, et al. “Anesthesia and Perioperative Management for an Patient with Ulrich Syndrome Undergoing Surgery for Scoliosis.” ACTA ANAESTHESIOLOGICA BELGICA 61.1 (2010): 43–47. Print.
@article{1006729,
  abstract     = {Abstract : Ullrich syndrome is a rare congenital
hypotonic-sclerotic muscular disorder in which affected
children develop a slowly progressive scoliosis and
contractures and limpness of joints. The disease causes
increasingly invalidating contractures and hardening of
the muscles of the neck and trunk. While this neuromuscular type of scoliosis is progressive, patients rarely attain the point of surgery due to their compromised
general medical condition. This may explain the current
lack of outcome data and the paucity of information on
perioperative management for patients with Ullrich
syndrome undergoing major surgery. The purpose of
this report was therefore to describe our first experience
with the perioperative and anesthetic management of a
15-year old boy presenting with Ullrich syndrome and a
secondary invalidating scoliosis. The specific challenges
of this condition characterized by severe restrictive lung
disease and a challenging airway abnormality are
discusse},
  author       = {Vandenberghe, Wim and JACOBS, TOM and Plasschaert, Frank and Willems, J and Den Blauwen, Nadia and VEREECKE, HUGO and Wouters, Patrick},
  issn         = {0001-5164},
  journal      = {ACTA ANAESTHESIOLOGICA BELGICA},
  keyword      = {scoliosis,hypotonic-sclerotic muscular dystrophy,Ullrich,perioperative managament.},
  language     = {eng},
  number       = {1},
  pages        = {43--47},
  title        = {Anesthesia and perioperative management for an patient with Ulrich syndrome undergoing surgery for scoliosis},
  volume       = {61},
  year         = {2010},
}