Revista espanola de cirugia ortopedica y traumatologia · 2014
[Occipital-cervical instability in Morquio disease: a presentation of 2 cases and a review of the literature].
Клинический случайСтатьяОбзорНазвание и аннотация: Перевод в очереди
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Ruiz-Picazo D, Díez-Ulloa M A
Аннотация
INTRODUCTION: Morquio syndrome is caused by an inherited autosomal recessive enzyme deficiency. It presents with numerous musculoskeletal anomalies, among which atlantoaxial instability is highlighted, due it being life-threatening. Occipital-cervical arthrodesis surgery (and decompression) is indicated in these cases.
OBJECTIVE: The cases of 2 patients with Morquio syndrome that required this type of surgery are presented, along with a review of the literature.
PATIENTS AND METHODS: Two patients: one boy and one girl, with Morquio syndrome and high cervical instability, with neurological signs and symptoms, who were subjected to surgery using decompression and instrumented occipital-cervical arthrodesis and followed up for 6 months and one year, respectively.
RESULTS: The neurological problems of both patients improved, and are currently having a normal life for their age.
CONCLUSION: Occipital-cervical arthrodesis with decompression provides a safe biomechanical environment that prevents neurological involvement. It should be indicated before the appearance of symptoms or mechanical instability.
OBJECTIVE: The cases of 2 patients with Morquio syndrome that required this type of surgery are presented, along with a review of the literature.
PATIENTS AND METHODS: Two patients: one boy and one girl, with Morquio syndrome and high cervical instability, with neurological signs and symptoms, who were subjected to surgery using decompression and instrumented occipital-cervical arthrodesis and followed up for 6 months and one year, respectively.
RESULTS: The neurological problems of both patients improved, and are currently having a normal life for their age.
CONCLUSION: Occipital-cervical arthrodesis with decompression provides a safe biomechanical environment that prevents neurological involvement. It should be indicated before the appearance of symptoms or mechanical instability.