[MIM 600 920]

(Marden-Walker-like syndrome)

Rare. Recessive autosomic transmission.

Association of:

-        blepharophimosis

-        arachnodactyly and contractures

-        dysmorphy: maxillary hypoplasia, large ears, thin wings of the nose, arched or cleft palate,  everted lower lip

-        two cases of laryngeal anomalies have been described

Anesthetic implications:

risk of difficult intubation; preoperative ENT fibroscopy mostly in case of stridor ?

References :

-        Carr CW, Carron JD, Lachman RS, Abdul-Rahman OA.
Van Den Ende–Gupta syndrome: Laryngeal abnormalities in two siblings.
Am J Med Genet Part A 2007; 143A:2706–2711.

Updated: November 2016