541/632. Intraethmoidal encephalomeningocele presenting with spontaneous cerebrospinal fluid rhinorrhea in an elderly man. Case report. The case of a 63-year-old patient with spontaneous cerebrospinal fluid rhinorrhea from a intraethmoidal encephalomeningocele is presented. The patient was asymptomatic until the old age. The preoperative coronal CT-scan and the preoperative and postoperative MRI are shown. The rarity of this onset in an elderly man and the surgical indications of this disease are debated. ( info) |
| Irradiation of the central nervous system may cause significant morbidity, including endocrine dysfunction and intellectual impairment. The authors report a case of bilateral temporal bone encephaloceles in a 21-year-old man who had received prophylactic central nervous system irradiation for acute lymphocytic leukemia in early childhood. Endaural encephaloceles are uncommon, and most occur as a complication of mastoid surgery. The etiology, clinical features, radiological diagnosis, and surgical treatment of temporal bone encephaloceles are discussed. ( info) |
| The authors report a 32-year-old woman who had undergone repair of an occipital encephalocele in infancy and who experienced a 20-year history of progressive hearing loss and intermittent vertigo. After parturition, she developed a rapidly progressive quadriparesis and brain-stem dysfunction associated with persistent intraventricular and subarachnoid hemorrhage. Serial magnetic resonance (MR) images showed progressive deposition of hemosiderin along the surface of the brain, brain stem, and spinal cord, and enhanced thickened membranes at the site of the original encephalocele repair. Posterior fossa exploration disclosed hemorrhagic membranes, which were resected; despite removal of this tissue, the patient deteriorated and died. Postmortem examination confirmed iron-containing pigment along the meninges, cerebral hemispheres, brain stem, spinal cord, and cranial nerves accompanied by atrophy of the superficial cerebellar cortex. It is concluded that superficial siderosis may accompany encephalocele repair. This is believed to be the first report in the literature of superficial siderosis of the central nervous system to correlate in vivo MR images with autopsy results. ( info) |
544/632. Nasopharyngeal gliomas: diagnostic and treatment considerations. Nasopharyngeal masses in newborns may produce life-threatening airway obstruction. Distinction between encephaloceles and gliomas is important for surgical planning, but cannot always be accurately assessed on the basis of high-resolution computed tomography or magnetic resonance imaging. Preoperative or intraoperative nuclide imaging or contrast injection studies may assist with selection of appropriate surgical approach. ( info) |
545/632. Orbital meningoencephalocele manifesting as a conjunctival mass. Orbital meningoencephalocele is a rare congenital abnormality caused by a defect of the cranio-orbital bones that usually manifests soon after birth as a soft, cystic fullness in the superomedial canthal area with associated exophthalmos. We managed an unusual case of congenital orbital meningoencephalocele that manifested as a cystic conjunctival mass without proptosis or periorbital changes. Preoperative computed tomographic scans failed to demonstrate a bone defect. After suture ligature of the posterior stalk, excision of the lesion yielded an ependymal cyst surrounded by neuroglial and meningeal tissue and filled with cerebrospinal fluid. The patient had normal results of ophthalmic and neurologic examinations after transconjunctival resection of the lesion after three years of follow-up. An orbital approach may be appropriate for a few selected cases in which no bone defect is found on computed tomography. Orbital meningoencephalocele should be included in the differential diagnosis of isolated congenital conjunctival cystic masses. ( info) |
546/632. Spontaneous temporal encephalocele. Case report. The authors report a 36-year-old woman with a 23-year history of simple and complex partial seizures who was treated surgically for an anteroinferior temporal encephalocele, with resolution of the seizure disorder. This patient's presentation, findings, and response to treatment are typical of those associated with anteroinferior temporal encephalocele, and different from the clinical patterns of four other types of spontaneous temporal encephalocele. ( info) |
547/632. Brain herniation into the mastoid in a normal hearing ear. A cluster of right temporal lobe abscesses in a 40-year-old male originated from an endaural brain hernia affecting the mastoid only, without direct contact with the ossicular chain. In infancy, antrotomy had been performed. Otologic signs were sparse, hearing was normal. Computed tomography of the temporal bone showed homogenous clouding of the retrotympanal spaces and a defect of the tegmen antri. Treatment consisted of transmastoid debridement, reduction and duraplasty with fascia lata. ( info) |
548/632. Identical twins with Weissenbacher-Zweymuller syndrome and neural tube defect. Neurologic abnormalities have been described only once previously in a child with Weissenbacher-Zweymuller syndrome (WZS), a rare skeletal dysplasia, evident neonatally. We report on identical twin male infants with skeletal findings typical of WZS, including small size at birth, proximal limb shortness, mid face hypoplasia, and myopia. In addition, twin B had a parieto occipital encephalocele while twin A had a meningocele at the same location. Twin B has had significant delays in development and hearing loss. ( info) |
549/632. Diagnosis of descending transtentorial herniation by cranial computed tomography. Descending transtentorial herniartion (DTH) secondary to a supratentorial mass effect can be detected by CT. Early signs of uncal herniation include encroachment on the suprasellar cistern, displacement of the brain stem, enlargement of the ipsilateral crural subarachnoid space, and compression of the contralateral cerebral peduncle. Increasingly severe combined uncal and hippocampal DTH results in progressive obliteration of the suprasellar and interpeduncular cisterns, elongation or compression of the brain stem, and inferior or posterior displacement of the basilar artery. ( info) |
| Two unrelated families are presented with repeated occurrences of a congenital syndrome of which the main stigmata were polycystic kidneys and occipital encephalocele (Meckel syndrome). prenatal diagnosis, followed by interruption of pregnancy, was performed in one case. The diagnosis was based on an increase of amniotic alpha-fetoprotein (AFP), and on the mode of growth and cell types of cultured amniotic cells. In another similarly examined case the diagnosis was suspected, but the parents did not wish the pregnancy to be interrupted. The child was stillborn and malformed. AFP values are presented and discussed in relation to the observed malformations. neural tube defects are associated with an increase of AFP in amniotic fluid, but, as in normal pregnancies, the values decrease with increasing gestational age. On the other hand, kidney malformations seem to be associated with AFP values which remain high or even increase with increasing gestational age. ( info) |