The Dandy-Walker malformation

Abstract

Forty cases of Dandy-Walker malformation referred to the Hôpital Necker Enfants-Malades between 1969 and 1982 have been reviewed. The incidence of the malformation in hydrocephalus was 2.4%. There was a slight, statistically insignificant, female prevalence. Hydrocephalus should not be included in the definition of the syndrome. In 80% of the cases, it was actually a post-natal complication of the malformation and most often developed within 3 months after birth. In 80% of the cases, a communication, although insufficient, was found between the dilated 4th ventricle and the subarachnoid space. Since this communication is probably established through the foramina of Luschka, the definition of the Dandy-Walker malformation should only include atresia of the foramen of Magendie. Associated brain and systemic malformations were numerous. Among facial anomalies, facial angiomas were found in 10% of our cases. The association of facial and cardiovascular anomalies favors the hypothesis that the onset of the malformation occurs between the formation and the migration of the cells of the neural crest (that is, between the 3rd and the 4th post-ovulatory week, earlier than previously thought). Except in selected patients, membrane excision has a high rate of failure and should be abandoned. Cyst-peritoneal shunting avoids the risk of an entrapped fourth ventricle and is presently the best surgical procedure. The overall mortality in this series was 12.5%. Intelligence quotients were over 80 in 60% of the patients. Other studies will be necessary to understand why the communication between the fourth ventricle and the subarachnoid spaces, sufficient in utero, usually becomes insufficient for a normal cerebrospinal fluid (CSF) circulation in the first months following birth. Two hypotheses are discussed: a change in CSF circulation, or bleeding in the dilated fourth ventricle during delivery.

Dandy-Walker Malformation Presenting with Affective Symptoms

“…Psychomotor developmental delay and mental retardation have been mentioned frequently in DWM patients in the literature (7,8,9,10). Other psychiatric disorders accompanying this malformation have been rarely reported.…”

“…Although Hirsch et al (7) mentioned hydrocephalus as a frequent complication of DWM, they suggested that it is not necessary for the diagnosis.…”

“…The incidence of DWM is approximately 1 in 25,000 to 35,000 live births (7). Hydrocephalus is present in 91%, and objective neurologic signs are observed in 73% of patients with DWM.…”

A Newborn with Dandy-Walker Malformation and Aortic Coarctation

“…The malformation occurs in approximately 1 per 25.000 live births, more frequently in girls than in boys (3). Usually, it is diagnosed in the prenatal period or early infancy, rarely in adulthood (3). Systemic anomalies in DWM may include cleft lip and palate, polydactyly, ORCID ID: orcid.org/0000-0003-0181-1166…”

Dandy-Walker syndrome in adult mimicking myasthenia gravis

“…Several other structured lesions of the central nervous system, such as cerebrovascular disease, brain stem tumors and Chiari malformation were described as mimicking MG, due to involvement or compression of the brain stem, could easily lead to misdiagnosis and an inappropriate treatment, but the DWS have not been reported to date 1,2,[6][7][8][9][10][11][12] .…”