[Bourneville's tuberous sclerosis associated with double uterus and vagina (didelphic uterus)]. / Esclerosis tuberosa de Bourneville asociada a duplicidad uterovaginal (útero didelfo).

INTRODUCTION: We describe a case that fulfill the diagnostic criteria for the tuberous sclerosis (Bourneville disease), and presents a double uterus and double vagina (didelphic uterus). We have not found any reference ti this association in the literature (MEDLINE search from July 1986 to December 1996). CLINICAL CASE: A 38-year-old woman with mental retardation and seizures who has been with carbamazepine since age 14. In 1979 a double vagina was diagnosed and a resection of a vaginal thin wall was performed. Laparoscopy showed a double uterus and multiple granulations of mesothelial proliferation in the Douglas sac. She has a remarkable behavior disorder with conjugal and familiar problems as well as a chronic renal insufficiency. The examination show a patient with a mental retardation, cutaneous hypopigmentation, facial adenomas sebaceums of Pringle, and ataxia, the rest of exploration was normal. The cranial CT show multiples calcified subependymal nodules. An abdominopelvic CT revealed several lesions in the liver parenchymal, and the pancreas head, compatible with hamartomas or lipomas, big kidneys with bilateral cysts and angiomyolipomas, and didelphic uterus. The cytogenetic study showed a normal karyotype (46,XX) and the molecular cytogenetic study (Fluorescence in situ hybridization, FISH) of chromosomes 2, 3, 4, 9, 11, 12 and 16 showed no chromosomal reangement. CONCLUSIONS: Eventhough both Bourneville disease and didelphic uterus have individually been associated with chromosomal abnormalities, our cytogenetic studies show no chromosomal reangement or abnormality despite the coexistence of both disease in our patient. In the bibliographic search that we have performed we have not found any report of a case like the one we describe here.

[Cleidocranial dysostosis. Presentation of a case]. / Disostosis cleidocraneal. Presentación de un caso.

INTRODUCTION: Cleidocranial dysostosis is a syndrome defined by three characteristic findings: clavicular aplasia, retarded cranial ossification, and autosomic dominant hereditary transmission, with completed penetrance and full expression. However, the diagnosis cannot only be made based on those finding, because the polymorphism and extension of the lesions of this disease is important. Therefore, in this disease we can see upset in the second teething, short stature or dwarf, persistence of the biconvex appearance of vertebral body, bone hypoplastic iliac, retarded pubis branch ossification, wedge shape distal phalanges or with brachymesophalangia of the forefinger and fifth finger. CLINICAL CASE: We describe a 20 years old man, with cleidocranial dysostosis, without familiar antecedent (probable mutation), that come to our center for treatment of denture pathology with disabled eating, because anomalous distribution and eruption. He had clavicle agenesis, cranial ossification upset with wormian bones, vertebral bodies biconvex, superior maxillary hypoplastic, and dental packed in the superior maxillary and jawbone. CONCLUSIONS: Cleidocranial dysostosis is a hereditary disease, which can be of spontaneous apparition (mutation), has a grand polymorphism, affect the osseous development, predominate in the middle line membranous bone and is an entity of radiologic diagnosis.

[Subarachnoid hemorrhage associated to subhyaloid hemorrhage: "Terson syndrome"]. / Hemorragia subaracnoidea asociada a hemorragia subhialoidea 'síndrome de Terson'.

INTRODUCTION: The combination of subarachnoid hemorrhage (SAH) and subhyaloid hemorrhage is known as 'Terson syndrome'. Retinal hemorrhage is commonly observed clinically in the optic fundi of patients with SAH, however, subhyaloid hemorrhage of the globe in the setting of SAH has been rarely on CT of the brain. Several mechanisms of subhyaloid hemorrhage have been proposed: a. A sudden increase in intracranial pressure (ICP) forces blood from the subarachnoid space directly into the preretinal space. b. A sudden rise in ICP is thought to decrease venous return to the cavernous sinus from the veins draining the globe. The increased retinal venous pressure results in stasis followed by vessel rupture. c. A sudden rise in ICP obstructs both the retinochoroidal anastomoses and the central retinal vein due to a rapid effusion of CSF through the communication of the subarachnoid space with the optic nerve sheat. This produces an acute decrease in venous drainage from the retina and results in stasis and hemorrhage. CLINICAL CASE: A 35 year old man, with a history of a non controlled arterial hypertension, dilated cardiopathy and 'agitation episodes'. He had a spontaneous intracranial hemorrhage, consistent in a parenchymal hematoma ruptured into ventricles and subarachnoid space. The CT showed through optic nerve sheath this hemorrhage extended to subhyaloid space. The patient came in coma 'dépassé' and brain death. CONCLUSIONS: We report a case of Terson syndrome demonstrated by CT. This CT allow see the blood from the subarachnoid space erupt directly into the preretinal space through optic nerve sheath, confirming one the proposed mechanism for this syndrome.

[Trans-ethmoid meningocele diagnosed in adults. Description of one case]. / Meningocele transetmoidal. Diagnóstico en edad adulta: presentación de un caso.

The meningocele and encephalocele are extracranial herniation of single meninges or meninges with brain tissue, through cranial defect. This pathology can be classificated according to contain or localization. The trans-etmoidal encephalocele is the 5% of meningoencephaloceles, and they are the 8-19% of all neural tube dysraphism. We report a 54 year-old woman with a spontaneous rhinorrhea due to an trans-ethmoidal meningocele associate with a recurrent meningitis. The computed tomographic (CT) revealed a trans-ethmoidal meningocele and she was treated with surgery. In presence of a patient with recurrent meningitis is necessary value the possibility of rhinorrhea, and an exhausted radiology study will be fulfill for identify the opening in the skull through leak CSF, and offer the best treatment. The transetmoidal can be a cause of rhinorrhea. The CT scan study of anterior fosa is a good method for diagnostic of this pathology, however, the IRM is the election method.

[Partial bilateral agenesis of the posterior arch of the atlas]. / Agenesia parcial bilateral del arco posterior del atlas.

INTRODUCTION: Congenital abnormalities of the posterior arch of the atlas (C-1) are very uncommon and not widely known. Isolated partial agenesis of the posterior arch of the atlas was initially considered a benign variation without any clinical or pathological significance. There is, however, increasing evidence that neurological symptoms may occur after minor cervical trauma in patients with an isolated partial agenesis of the posterior arch of the atlas, specially the types 'C' and 'D' malformations described by Currarino et al. CLINICAL CASE: A 63 year old woman with cervicalgia and 'seasickness', was study with a plain cervical spine radiographs and CT with three-dimensional reconstruction, who showed a bilateral partial absence of the posterior arch of C-1 with persistent posterior tubercle, corresponded to type 'D' in the classification descrites by Currarino et al. CONCLUSIONS: The fact of this pathology can unchain an important neurological symptoms after minor cervical trauma, oblige to the physician (radiologist, neurologist, neurosurgeon, traumatologist, rehabilitation specialist) know this pathology, for to include this patients in a group of the risk, and indicate to them who should avoid contact sports and other strenuous athletic endeavors, and furthermore value the surgical treatment.

Direct non-invasive techniques for assessing coronary bypass graft patency.

Venous digital subtraction angiography (vDSA) and computed tomography (CT) are two non-invasive techniques that allow direct imaging of coronary grafts. Neither of them is 100% accurate when compared to selective angiography. We studied 52 patients with 107 coronary grafts (101 saphenous and 6 mammary artery grafts) by CT and vDSA. Fifty patients had control selective angiography and 2 had control digital arterial aortography. CT correctly diagnosed 88 of the 95 patent grafts and 8 out of the 12 occluded grafts (in 1 patient 3 patent grafts could not be well analyzed owing to artifacts from pacemaker wires). vDSA correctly identified 93 patent grafts and the 12 occluded grafts, allowing the correct diagnosis of the 11 non/or misdiagnosed CT grafts. CT allowed the correct identification of the 2 misdiagnosed vDSA grafts. These results show that when there was agreement between CT and vDSA there were no diagnostic errors and that the combination of these two non-invasive techniques may avoid selective angiography for studying coronary bypass graft patency.