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Arthrogryposis multiplex congenita: a case report.

Author

Cherryl T. Yrag

Related Institution

Department of Anesthesia - Vicente Sotto Memorial Medical Center

Publication Information

Publication Type
Research Report
Date

Abstract

Significance :


    Knowing the pathophysiology, anatomy and the clinical features of the disease will lead anesthesiologist to proper treatment and management should a need for an operation arise. Alternative anesthetic plan could also be made possible if well equipped and prepared for the procedure.


Result :


     Preoperative evaluation of the patient was carefully done. Subarachnoid block was indicated in this patient because of the site of operation. General anesthesia was an alternative although not the proposed plan due to the limitation of temporomandibular  joint movement and the small mouth opening of the patient. The patient was stable throughout the operation. No complication was encountered during the entire procedure.


Conclusion: 


     Arthrogryposis multiplex congenita comprises nonprogressive conditions characterized by multiple joint contractures found throughout the body. This is used in connection with a heterogeneous group of disorders with a common feature of multiple congenital joint contractures. Severity of the disease varies widely and depends mainly on etiology (i. e. cartilaginous, nervous system, constraint). Regardless of the cause, anesthesia can be difficult because vascular access often is restricted, intubation may pose problems for patients with a small underdeveloped jaw, limited movement of the temporomandibular joint, and narrow airway. Osseous hypoplasia, which is associated with decreased mechanical use in developing bone, is prone to fracture at multiple sites. Anesthesia should be undertaken carefully, since patients who have a rare form of  arthrogryposis are prone to having malignant hypertermia.


Recommendations:


     In order to prevent possible difficulties intraoperatively, it is recommended, especially for anesthesiologists to do proper physical examination and evaluation of the case preoperatively. Arthrogryposis is rare, occurring in 1: 3,000 live births, but poses several complications if not assessed properly.


 


 


 

Objectives

     To present a case of a 23 year old patient with arthrogryposis multiplex congenita having distal tibial osteotomy procedure, to understand the pathogenesis, clinical features, diagnosis and management of the disease.

References

1. Barash, et al. . Clinical Anesthesia. Philadelphia: Lippincott Williams & Wilkins, 2001. (4th ed.)
2. Morgan, et Al. . Clinical Anesthesiology. New York: Appleton and Lange, 2002. (3rd ed.)
3. Chen, Harold . (). . Retrieved from: www.emedecine.com
4. Israel, Alfonso , Papazian, Oscar , Paez, Julio Cesar , Grossman, John A.I.. (). Arthrogryposis multiplex congenita : A review article.. www.internationalpediatrics.com.

Physical Location

LocationLocation CodeAvailable FormatAvailability
Vicente Sotto Memorial Medical Center Fulltext Print Format

 
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