1/6. Disappearance of hemifacial spasm after ventriculoperitoneal shunting in a patient with achondroplasia--case report.A 15-year-old boy with achondroplasia developed right hemifacial spasm associated with headache, vomiting, and hearing disturbance. Computed tomography showed hydrocephalus. A ventriculoperitoneal shunt was placed. His hydrocephalus subsequently resolved, the hemifacial spasm and headache disappeared, and his hearing disturbance improved. The episodes of hemifacial spasm were probably related to a small posterior cranial fossa volume, the so-called crowding of the posterior fossa. Increased intracranial pressure due to hydrocephalus apparently contributed to further reduction in the posterior cranial fossa volume and led to the hemifacial spasms. In addition, his hearing disturbance may have been the result of dysfunction of the cochlear nerve due to the increase in intracranial pressure caused by hydrocephalus.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/6. The role of endoscopic third ventriculostomy in the treatment of triventricular hydrocephalus seen in children with achondroplasia.The authors of this paper describe two patients with achondroplasia in whom triventricular hydrocephalus has been successfully treated using endoscopic third ventriculostomy (ETV). The authors performed ETVs in two children with achondroplasia involving a moderate macrocranium and enlarged lateral and third ventricles with intermittent clinical evidence of increased intracranial pressure. The endoscopic procedure revealed a significant deformation of the floor of the third ventricle in both patients caused by longstanding hydrocephalus. Because of the anatomical variations such as empty sella and the persistence of the infundibular recess, the ETV procedure became technically demanding. The standard ventriculostomy on the tuber cinereum was not possible. Ventriculostomies were performed on the part of the third ventricular floor that was hanging ventral to the pons. Both patients became symptom free postoperatively, and phase-contrast cine magnetic resonance (MR) imaging studies confirmed the patency and effectivity of the ventriculostomy sites. The authors suggest that ETVs may constitute an alternative for ventriculoperitoneal shunt therapy as the treatment of choice for patients with achondroplasia who have triventricular hydrocephalus and clinical symptoms. attention should be paid to the shape of the floor of the third ventricle, however, especially in cases of longstanding hydrocephalus. An association of the empty sella or the persistence of the infundibular recess must be carefully evaluated using MR imaging studies before attempting ETVs in such patients.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
3/6. hydrocephalus in achondroplasia: the possible role of intracranial venous hypertension.The significance and cause of ventriculomegaly in achondroplasia was investigated in five achondroplastic children. The intraventricular pressure (IVP) was monitored over 24 hours, followed by intraventricular injection of radionuclide alone or in combination with water-soluble contrast material. The IVP was elevated and the reabsorption of cerebrospinal fluid (CSF) into the sagittal sinus was slow in all cases, but there was no obstruction to CSF flow. The spinal subarachnoid space was well seen in all patients. Jugular venograms with pressure monitoring were obtained in four patients (bilaterally in one). These studies confirmed a narrow jugular foramen in all patients with a significant venous pressure gradient (3 to 10 mm Hg) obtained while the catheter was being pulled back from the sigmoid sinus through the foramen. A second gradient was found in the jugular vein in two patients at the level of the upper thoracic aperture. This gradient was 6 and 14 mm Hg, respectively. Identical venograms and monitoring of the venous pressure in a control group showed no pressure gradients across the jugular foramen and smaller gradients (2 to 5 mm Hg) across the thoracic inlet. It is concluded from these studies that ventriculomegaly in achondroplastic children represents hydrocephalus, which is likely secondary to raised intracranial venous pressure due to hemodynamically significant stenosis of the jugular foramen and, in some cases, the jugular vein in the thoracic aperture.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
4/6. Therapy-resistant papilledema in achondroplasia.A 20-year-old achondroplastic dwarf developed severe papilledema over a short period of time. Since the age of 3 he had a ventriculoperitoneal shunt for presumed hydrocephalus. Severe ventriculomegaly was never documented, neither in the past, nor at presentation. intracranial pressure was high, and the shunt was obliterated. Despite prompt shunt revision and subsequent bilateral optic nerve sheath decompression the patient developed postpapilledema optic atrophy with bilateral blindness. The pathophysiology of increased intracranial pressure in achondroplasia is discussed.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/6. hydrocephalus and chronically increased intracranial pressure in achondroplasia.Two achondroplastic children with ventriculomegaly are reported. The patients had no signs of increased intracranial pressure, but in one blindness had previously been detected by the parents. Neuroradiological examinations showed ventriculomegaly in both. intracranial pressure remained at high levels (20-55 mmHg) preoperatively; ventriculo-peritoneal shunting was performed in both cases, and postoperatively levels fell to normal (5-16 mmHg). The need for the treatment of ventriculomegaly seen in achondroplastic children is analyzed on the basis of these two patients, with a brief review of literature.- - - - - - - - - - ranking = 3keywords = pressure (Clic here for more details about this article) |
6/6. Jugular foraminal stenosis in Crouzon syndrome.We describe a 4-year-old boy with Crouzon syndrome, with associated acanthosis nigricans and Chiari-I malformation, who developed increased intracranial pressure necessitating posterior cranial expansion. Postoperatively, an arteriovenous fistula appeared over the mastoid region. cerebral angiography demonstrated bilateral atresia of the jugular veins and occlusion of the left sigmoid and right transverse sinuses. We propose that increased intracranial venous pressure, secondary to bilateral jugular foraminal stenosis, caused hydrocephaly and venous dilation in the scalp vasculature. The latter set the stage for a traumatic arteriovenous fistula of the scalp, probably resulting from laceration of an adjacent artery and vein. Jugular atresia is a basilar malformation common to achondroplasia and certain eponymous syndromic craniosynostoses. Our patient has a mutation in fibroblast growth factor receptor 3, a different locus in the same gene mutated in achondroplasia.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |