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Inherited disorder of the connective tissue, characterized mainly by brachycephaly, arachnodactyly, widespread dysostoses, increased ligament laxity, receding lower jaw (micrognathia), and joint laxity of the hands and feet.

First described in 1902 by Emile Charles Achard, a French internist.

Autosomal dominant trait.

Based on the clinical findings of brachnodactyly, micrognathia, and broad and brachycephalic skull.

Despite similarities, Achard syndrome and Marfan syndrome are different entities. Achard syndrome is a well-defined clinical entity characterized by widespread dysostoses most consistently affecting the tubular bones of the hands and feet, mandible, and calvaria, but it can occur anywhere in the skeleton. Ligament laxity manifests as hypermobility and/or subluxations of the joints (most commonly hands and feet) and increased lateral excursion of the patella. The skull is broad and brachycephalic, the lower jaw is small and receding, and the hands and feet show arachnodactyly. Body proportions are normal; however (in contrast to Marfan Syndrome), most of these patients are known for not being tall. Patients with Achard syndrome lack the eye and heart abnormalities and the subnormal subcutaneous fat.

Assess the airway for signs of difficult management.

Micrognathia may make airway management difficult. Spontaneous ventilation should be maintained until the airway has been secured. Careful patient positioning is required to avoid (sub)luxations of the joints.

No known specific implications with this condition.

Marfan Syndrome: Familial disorder of generalized connective tissue abnormalities leading to connective tissue weakness associated with hyperextensible joints, dislocation of the lens, increased risk of valvular heart disease, risk of dissecting aortic aneurysm, and spontaneous pneumothorax.

Parish JG: Heritable disorders of connective tissues with arachnodactyly. Proc R Soc Med 53:515, 1960.  [PubMed: 14430455]
Duncan PA: The Achard syndrome. Birth Defects Orig Artic Ser 11:69, 1975.  [PubMed: 1201353]

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