181/256. Dandy-Walker malformation and associated cardiac anomalies. 2 patients with Dandy-Walker malformations (DWM) and cardiac anomalies are presented. The types of cardiac defects with septal involvement are suggestive of an early embryonic abnormality occurring before 6 weeks' gestational age. This correlates with the postulated timing of the development of the DWM and suggests a complex developmental anomaly. awareness of the associated systemic and central nervous system anomalies in patients with the DWM in regard to management and prognosis is emphasized. ( info) |
182/256. A case of holoprosencephaly: with possible association of Dandy-Walker cyst. The authors described a case of alobar holoprosencephaly with a huge cyst in the posterior fossa, possible representing a Dandy-Walker cyst, and marked cerebellar agenesis in the absence of median facial dysmorphias that has recently been encountered at our clinic. ( info) |
183/256. Pathogenesis of congenital midline porencephaly. A recent report in this journal described 5 children with holoprosencephaly, parietal scalp anomalies and midline intracranial cysts. The authors coined the term 'congenital midline porencephaly' for the clinical syndrome and described the cysts as diverticula of hydrocephalic lateral ventricles. This report describes 3 further children with the same syndrome. The author proposes that the midline cyst is simply a variant of the dorsal cyst reported in other cases of holoprosencephaly. It is further concluded that the cyst arises from primary dysgenesis of cerebral midline structures and is not the result of hydrocephalus. The monoventricle and cyst in these children have the CT scan appearance of the head of a primitive arrow. This 'arrowhead' sign may be helpful in the diagnosis of holoprosencephaly with congenital midline porencephaly. ( info) |
184/256. A fetus with upper limb amelia, "caudal regression" and Dandy-Walker defect with an insulin-dependent diabetic mother. We describe the fetus delivered to an insulin-dependent diabetic woman who had had a previous large, stillborn, non-malformed male infant and a normal female infant. The present fetus had a most unusual combination of malformations which to date had not been described in diabetic embryopathy. The anomalies include: upper limb amelia, "caudal regression" with bilateral absence of the fibulae, unilateral absence of a femur and ipsilateral oligodactyly; undescended testes; atrial septal defect; multiple vertebral and rib anomalies with cervical scoliosis and right webbed neck; left cleft lip and cleft palate; severe micrognathia; left microtia with atresia of the ear canal; and central nervous system defects including hydrocephalus with the Dandy-Walker malformation, asymmetry of the lateral ventricles, abnormal frontal gyral formation, and ependymal and ganglion cell heterotopias of the spinal cord. The pathogenesis of diabetic embryopathy is discussed. ( info) |
185/256. adult-onset presentation of Dandy-Walker variant in siblings. BACKGROUND: The dandy-walker syndrome and Dandy-Walker variant usually present as isolated cases of hydrocephalus in pediatric patients. methods AND RESULTS: THis paper consists of a case report of the adult onset of symptoms in two sisters having Dandy-Walker variant. Such an occurrence has never before been reported in the medical literature. Both patients presented with headaches and progressive neurologic deficit. On computed tomography (CT scan) of the head, both were found to have hydrocephalus, with hypoplasia of the inferior vermis. Both patients were treated successfully with ventriculoperitoneal shunting. A third sister, with a similar history, elected not to undergo CT scanning or surgical treatment. CONCLUSIONS: Variants of the dandy-walker syndrome may occasionally present clinically in the adult age group. Such an occurrence in siblings is consistent with an underlying genetic etiology. ( info) |
186/256. Brainstem tethering in dandy-walker syndrome: a complication of cystoperitoneal shunting. Case report. Treatment of the dandy-walker syndrome has included placement of a ventriculoperitoneal shunt alone or in combination with a posterior fossa cystoperitoneal shunt. Complications in shunting are common and are usually related to malfunction or infection. The authors present a case in which the patient developed headaches and focal cranial nerve deficits following infection caused by a cystoperitoneal shunt. magnetic resonance imaging showed tethering of the brainstem. A posterior fossa craniotomy with microsurgical untethering and cyst fenestration achieved two goals: improvement of the focal cranial nerve deficits and elimination of the cystoperitoneal shunt. ( info) |
187/256. Transtentorial herniation of the fourth ventricle. Transtentorial herniation (TTH) of the fourth ventricle is the result of a progressive enlargement of an isolated fourth ventricle which herniates through the tentorial incisure into the middle cranial fossa. The characteristic CT-MR and neurosonographic findings are described. ( info) |
188/256. Sudden unexpected death in patients with Dandy-Walker malformation. An uncommon but well-recognized occurrence in patients with Dandy-Walker malformation is sudden unexpected death. The mechanism of demise has not been established. We report three patients with Dandy-Walker malformation that experienced sudden unexpected death without uncal or tonsillar herniation, the mechanism usually proposed for demise in such situations. Our findings suggest the possibility of vascular compromise as the cause of the sudden unexpected death in these patients. Early and effective relief of the pressure in the posterior fossa may prevent the occurrence of this catastrophic complication. ( info) |
189/256. L-dopa resistant parkinsonism in an adult woman with a cyst in the posterior fossa. We report a case of a woman with L-Dopa resistant asymmetrical parkinsonism with a posterior fossa cyst compressing the lower brainstem on MR. She did not show improvement in any of her symptoms after cysto-cardiac derivation. It was not possible to delineate if this was a case of a new malformative syndrome or the coincidence of two different disorders. ( info) |
A boy was born with dandy-walker syndrome associated with a giant occipital meningocele, cleft lip, and cleft palate. The meningocele was actually a component of the giant posterior fossa cyst which communicated with the fourth ventricle. A cyst-peritoneal shunt achieved a considerable decrease in the size of the meningocele, but decubital ulcers developed due to restricted head movement caused by the occipital lesion. Cranioplasty removed a wide area of the inferior occipital bone, and the boundary between the superior occipital and parietal bones was thinned to allow free bending of the bone flap. The meningocele was removed totally in the third operation, but infection of the wound and pneumonia developed, causing death. The coexistence of dandy-walker syndrome and occipital meningocele, together with midline facial anomalies, may suggest a later pathogenesis of dandy-walker syndrome than previously believed. ( info) |