Disease Information for Dandy-Walker anomaly/syndrome: Definition

  • Dandy Walker Malformation; DWM; Dandy-Walker Cyst; Dandy-Walker Deformity; Dandy-Walker Syndrome; Hydrocephalus, Internal, Dandy-Walker Type; Hydrocephalus, Noncommunicating, Dandy-Walker Type; Luschka-Magendie Foramina Atresia; Dandy-Walker Malformation is a rare malformation of the brain that is present at birth (congenital); It is characterized by an abnormally enlarged space at the back of the brain (cystic 4th ventricle) that interferes with the normal flow of cerebrospinal fluid through the openings between the ventricle and other parts of the brain (foramina of Magendia and Luschka); Excessive amounts of fluid accumulate around the brain and cause abnormally high pressure within the skull, swelling of the head (congenital hydrocephalus), and neurological impairment; Motor delays and learning problems may also occur; Dandy-Walker Malformation is a form of "Obstructive" or "Internal Noncommunicating Hydrocephalus," meaning that the normal flow of cerebrospinal fluid is blocked resulting in the widening of the ventricles; [NORD 2005]--------------------------------.Dandy-Walker syndrome; also known as:

    Dandy-Walker disease; Dandy-Walker anomaly; Dandy-Walker cyst; Atresia of the foramen of Magendie, atresia of the foramen of Luschka and Magendie, obstructed Sylvius aqueduct, Luschka-Magendie foramina atresia, internal hydrocephalus, and noncommunicating hydrocephalus;

    A variant of Arnold-Chiari syndrome; A congenital brain malformation involving the fourth ventricle and cerebellum, marked by partial or complete absence of the cerebellar vermis, and posterior fossa cyst continuous with the fourth ventricle; Congenital hydrocephalus is caused by blockage of the foramina of Magendie and Luschka (the foramina of the fourth ventricle of the brain), through which the cerebrospinal fluid passes; Because of the blockage, the cerebrospinal fluid cannot pass into the subarachnoid space and in consequence accumulates in the ventricles;Symptoms are vomiting, hyperirritability, convulsion ; There is progressive enlargement of the head, congested veins in the scalp, bulging of anterior fontanelle, separated cranial sutures; Other complications may include papilledema, blepharoptosis, bradycardia, bradypnea and sixth cranial nerve paralysis; Develops in utero; etiology uncertain; In some rare cases the disease is a dominant or recessive inheritable trait; It is now recognised that the anatomical abnormality of the foramina that leads to internal hydrocephalus can be a component of several distinct syndromic entities; [Whonamedit.com 2005]