[See: Palestine > Al-Gazali et al. 1996].

Koul et al. (2000) reported the diagnosis of neurofibromatosis type 1 in monozygotic twins with Dandy-Walker syndrome. The twins, both males, were born at term to non-consanguineous parents. At delivery they were noted to be monozygotic twins as there was a single placenta. There were no antenatal or perinatal complications. As motor and mental developmental delay was noticed by the parents, these children at the age of 32-months were referred to the authors' care for neurological evaluation, which revealed marked motor delay (head control at nine months, sitting at one year, and just standing with support when seen) and no speech development. Examination revealed head circumference at the 50th percentile but both weight and height were below the third percentile. Both children had dysmorphic features which were not descriptive of any syndrome, including prominent forehead, hypotelorism, low set ears and prominent occiput. In addition to these features, they had café-au-lait spots seen more on their trunks with few of 5 cm2 in size, along with few depigmented spots. A diagnosis of neurofibromatosis type 1 was made on the bass of the presence of the features of café-au-lait, microcephaly and short stature. Routine investigations, including blood count, liver and renal function tests, nerve conduction studies and brainstem auditory evoked potential, and karyotyping were normal. In both children, CT scan of the brain revealed typical features of Dandy-Walker syndrome which were cerebellar aplasia, cystic dilatation of the fourth ventricle and enlarged posterior fossa. In addition, one twin had hydrocephalus as well. Koul et al. (2000) suggested an underlying genetic basis for this unique association of neurofibromatosis type 1 with Dandy-Walker syndrome in monozygotic twins.

Al-Gazali et al. (1996) reported a child, born to unrelated parents of Palestinian-Jordanian origin, with typical features of Meckel syndrome in whom both encephalocele and Dandy Walker malformation (DWM) existed. This female child, as well as two previous females born to the family, were born with posterior encephalocele, polycystic kidneys and no polydactyly. Both died a few hours after birth. At birth, the propositus was noted to also havewide sutures with an occipital encephalocele. CT scan of the brain showed hypoplastic cerebellar hemispheres, an absent vermis and a very large posterior fossa cyst communicating with the fourth ventricle, and high attachement of the tentorium. Renal ultrasound showed hugely enlarged hyperchogenic kidneys on both sides with loss of corticomedullary differentiation. The baby died on the second day of life.

El Awad (1992) calculated the overall incidence of infantile hydrocephalus in the south-western region of Saudi Arabia as 0.81/1000 between January 1988 and December 1990. Five of these infants showed Dandy-Walker malformation (8.2%).

Ohaegbulam and Afifi (2001) studied the incidence of DWS in a defined Saudi Arabian population of military personnel and their dependants during an 11-year period (1989-1999) from a cohort of 45,274 live births. Ohaegbulam and Afifi (2001) recorded an incidence of 1.0 DWS case per 100,000 live births per year. The incidence by sex per 100,000 live births per year was 1.24 for males and 0.78 for females. DWS formed 3.5% of studied cases of infantile hydrocephalus.

Alorainy (2006) reviewed and analyzed the MRI studies on 808 pediatric patients (aged 3 days to 15 years) over a 3-year period.  A total of 114 congenital cerebral malformations were identified in 86 of these patients via MRI.  Three patients were identified with Dandy Walker malformation.

Kurdi et al., (2009) described a 2-month old boy with a rare Dandy Walker malformation associated with progressive heart failure secondary to hypertrophic cardiomyopathy.  The child died at the age of 7 months.