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Submitted: 03 December 2014 Modified: 16 September 2015
HERDIN Record #: R07-CVHRDC-14120308510869

Arthrogryposis multiplex congenita : A case report

1Frederick  G. Sarmen
Department of Orthopedic Surgery - Vicente Sotto Memorial Medical Center

Abstract

Background: Arthrogryposis multiplex congenita, or multiple congenital contractures, is a non progressive syndrome characterized by deformed, rigid joints. This case report as to its etidogy, clinical, pathophysiological, diagnostic, therapeutic, as well as prognostication purposes.


Methods: A 12-year old female, child admitted at Department of Orthopaedic Surgery, Vicente Sotto Memorial Medical Center was made as the subject of this case report. History, physical examination and diagnostic work-up were reviewed and we found out that the data of the subject was consistent with Arthrogryposis.


Results: Arthrogryposis multiplex congenita is a nonprogressive syndrome characterized by deformed, rigid joints. the incidence of Arthrogryposis is 1 in 3000 live births. The involved muscles or muscle groups are atrophied or absent. The involved extremities appear cylindrical, fusiform, or cone-shaped and have diminished skin creases and subcutaneous tissue. Contracture of the joint capsule and is common, especially of the hip and knee. Sensation and intellect are normal. Deformities may result from neurogenic, myogenic, skeletal, or environmental factors, most are neurogenic in origin. Diagnosing Arthrogryposis is made based on clinical features of this disorder. There is no known medical treatmetn noted that would treatment noted that would modify this disease entity however surgical intervention is the one recommended.


Conclusion: Even though we seldom encounter Arthrogryposis, this should always be included as differential diagnosis in patients presenting and radio graphic studies is the gold standard in diagnosing this disorder. Management would include physiotherapy improving the range of movement, however, surgery is still the mainstay with the goal of improving the function. The prognosis is good. With proper management, most patients live independently and hold useful jobs.

1.
Publication Type:
Research Report
Date:

Objectives

The objective of this case report is to discuss Arthrogrypsis as to its etiology, clinical, pathophysiologic, diagnostic, therapeutic and pragnostic features.

LocationCall NumberAvailable FormatAvailability
Vicente Sotto Memorial Medical Center Fulltext Print Format
1. Tachjian, , Mihran . Pediatric Orthopedic. Wol 3 2nd ed.. WB Saunder, 1990.
2. Broughton, , Nigel . Pediatric Orthopaedics 1st ed.. WB Saunder, 1997.
3. Canale,, ST Campbell . Operative Orthopaedics 19 th ed.. Mosby, St Louis: 1998.
4. Miller , Mark D.. Neuromuscular Disorders: Arthrogrypotic Syndromes.. Review of Orthopaedics 3rd ed. Philadelphia: WB Saunders., 1992.
5. Brown,, L.M , Robson, M.J . "The pathophysiology of arthrogryposis multiplex congenita neurological." J Bone Joint Surg 62, 291, 1980
6. Brooks, D.M , Seddon, H.J . "Pectoral transplantation for paralysis of the flexor of the elbow. A new technique." J Bone Joint Surg 41, B 36, 1989