Dandy-Walker malformation: a rare association with hypoparathyroidism.

Dandy-Walker malformation is characterized by cystic dilatation of the fourth ventricle and an enlarged posterior cranial fossa with upward displacement of the tentorium, lateral sinuses, and torcular, with agenesis or hypoplasia of the cerebellar vermis. Dandy-Walker malformation occurs in approximately the 4th week of gestation and is associated with various abnormalities involving the cardiac, skeletal, genitourinary, and gastrointestinal systems. The parathyroid gland also forms in the 3rd and 4th gestational weeks. Reported here is the case of a male infant with Dandy-Walker malformation with ventricular and atrial septal defect, unilateral renal agenesis, and hypoparathyroidism. To our knowledge, this rare association with neural crest events during the development of Dandy-Walker malformation has not been reported previously.

Ventriculosubgaleal shunt procedure and its long-term outcomes in premature infants with post-hemorrhagic hydrocephalus.

OBJECTIVE: It is well known that 10-15% of hydrocephalus cases at childhood and 40-50% in premature infants, occur following Germinal matrix hemorrhage (GMH). Such hemorrhages are reported to arise due to the rupture of germinal matrix (GM) vessels as a result of cerebral blood flow changes among infants with <1500 g birth weight and <32 weeks old. Intraventricular hemorrhage (IVH) associated with GMH leads to a disruption in the cerebrospinal fluid (CSF) and ventricular dilatation. Ventriculosubgaleal shunt (VSGS) is preferred in those hydrocephalus cases because it is a simple and rapid method, precludes the need for repetitive aspiration for evacuation of CSF, establishes a permanent decompression without causing electrolyte and nutritional losses, and aims to protect the cerebral development of newborns with GMH. MATERIAL AND METHOD: The present study comprises 25 premature cases, subjected to VSGS and diagnosed with post-hemorrhagic hydrocephalus (PHH) arising from IVH associated with GM, and low birth weight (LBW) in the Neurosurgery Department of the Medical Faculty of Erciyes University between July 2002 and September 2006. VSGS surgery was performed on those cases, and their clinical and radiological prognoses were monitored with regard to several parameters. RESULTS: Mortality and morbidity results were found to be lower than those in PPH treatment methods. While prognosis of grade 4 GMHs was poor, grades 2 and 3 GMHs displayed a much better prognosis after VSGS along with complete recovery in some hydrocephalus cases.