Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome: a literature search and report of two cases.

SUMMARY: Patients with Klippel-Trenaunay-Weber syndrome present with venous varices, cutaneous capillary malformations, and tissue hypertrophy, usually involving an extremity. A small but important subset also harbors arteriovenous malformations (AVMs) of the spine. We report 2 such cases, 1 with 3 concurrent spinal arteriovenous fistulas. These cases and our review of the literature emphasize the importance of screening the spine for AVMs. In addition, it is also important to investigate for the presence of multiple spinal AVMs.

Autosomal Dominant Polycystic Kidney Disease and Intracranial Aneurysms: Is There an Increased Risk of Treatment?

BACKGROUND AND PURPOSE: Autosomal dominant polycystic kidney disease is associated with an increased risk of intracranial aneurysms. Our purpose was to assess whether there is an increased risk during aneurysm coiling and clipping. MATERIALS AND METHODS: Data were obtained from the National Inpatient Sample (2000-2011). All subjects had an unruptured aneurysm clipped or coiled and were divided into polycystic kidney (n = 189) and control (n = 3555) groups. Primary end points included in-hospital mortality, length of stay, and total hospital charges. Secondary end points included the International Classification of Diseases, Ninth Revision codes for iatrogenic hemorrhage or infarction; intracranial hemorrhage; embolic infarction; and carotid and vertebral artery dissections. RESULTS: There was a significantly greater incidence of iatrogenic hemorrhage or infarction, embolic infarction, and carotid artery dissection in the patients with polycystic kidney disease compared with the control group after endovascular coiling. There was also a significantly greater incidence of iatrogenic hemorrhage or infarction in the polycystic kidney group after surgical clipping. However, the hospital stay was not longer in the polycystic kidney group, and the total hospital charges were not higher. Additional analysis within the polycystic kidney group revealed a significantly shorter length of stay but similar in-hospital costs when subjects underwent coiling versus clipping. CONCLUSIONS: Patients with polycystic kidney disease face an increased risk during intracranial aneurysm treatment, whether by coiling or clipping. This risk, however, does not translate into longer hospital stays or increased hospital costs. Despite the additional catheterization-related risks of dissection and embolization, coiling results in shorter hospital stays and similar mortality compared with clipping.

Granulocytic sarcoma is associated with the 8;21 translocation in acute myeloid leukemia.

PURPOSE: Since the only three cases of granulocytic sarcoma among patients with acute myeloid leukemia (AML) seen at our institution during the last 12 years were each associated with the 8;21 translocation [t(8;21)], we sought to determine if this association is specific and more frequent than previously recognized. PATIENTS AND METHODS: We report three patients with AML and t(8;21) who developed granulocytic sarcomas, and review the world literature. RESULTS: Between 1980 and 1992, 53 cases of AML French-American-British (FAB) M2 were identified at our institution. Eight (15%) patients had t(8;21). Three of these eight patients (38%) developed granulocytic sarcoma. All three of our patients received conventional intensive antileukemic chemotherapy yet had short relapse-free survival durations. Several series of patients with t(8;21) report that granulocytic sarcomas occur in approximately 18% of this population, which is four times the expected incidence in AML. Thirty-seven cases have been previously reported. Although karyotype analyses were not reported in many cases of granulocytic sarcoma in the literature, the vast majority of abnormal karyotypes in patients with AML involved t(8;21). Recent work with a cell line derived from a patient with t(8;21) indicates that such cells are unusually adherent to culture bottles and are aggregable CONCLUSION: Our data suggest that this association is more common than generally recognized and may be specific. Patients with t(8;21) should be observed closely for signs and symptoms of granulocytic sarcoma. These patients may have a less favorable prognosis than other patients with t(8;21). Cooperative oncology groups should retrospectively identify patients with AML and t(8;21) who had a poor outcome to determine if they had a disproportionate incidence of granulocytic sarcoma. If so, aggressive therapy such as bone marrow transplantation may be warranted early in the therapeutic strategy.

Spinal cord venous infarction following endoscopic sclerotherapy for esophageal varices.

The authors report an unusual case of venous infarction of the spinal cord associated with endoscopic sclerotherapy for esophageal varices. MR imaging findings included signal abnormalities and abnormal enhancement of the affected spinal cord and T-8 vertebral body. A review of the hemodynamic changes associated with portal hypertension and the normal venous drainage of the spinal cord is presented.

Current approaches to imaging of the sellar region and pituitary.

Recent advances in MR imaging have enabled the radiologist to view the pituitary gland in its normal and diseased states to a greater extent than ever before. The techniques for obtaining quality images of the sellar region and the normal appearance of the pituitary gland are discussed. This article also discusses the imaging of several pituitary disease processes, with emphasis on pitutiary adenomas and recent advances in diagnosis and follow-up. Current controversies also are addressed.

Safety and efficacy of sodium and meglumine ioxaglate (Hexabrix) and Hypaque M60% in contrast-enhanced computed cranial tomographic scanning. A double-blind clinical study.

The safety and efficacy of sodium and meglumine ioxaglate (Hexabrix, Mallinckrodt, Inc., St. Louis, MO), a new low-osmolality contrast agent, and Hypaque Meglumine 60% (Wintrop-Breon, Inc., New York, NY) were compared for contrast-enhanced computed cranial tomographic scanning. Fifty-two patients with possible enhancing lesions of the brain received a total of 59 injections of either Hexabrix or Hypaque M60 in a double-blind, randomized fashion. Hexabrix was superior to Hypaque M60 in both subjective and objective patient tolerance. A lower incidence of heat and pain in patients receiving Hexabrix was believed to be related to its lower osmolality. Lesion enhancement with both Hexabrix and Hypaque M60 was excellent. There was a statistically significant increase in the degree of enhancement immediately after injection with Hexabrix. Hexabrix had less effect on the cardiovascular system than Hypaque M60. No neurologic complications or adverse reactions requiring treatment occurred in either group. Hexabrix is a suitable agent for intravenous contrast-enhancement for cranial CT.

T cell-depleted autologous hematopoietic stem cell transplantation for multiple sclerosis: report on the first three patients.

Multiple sclerosis (MS) is a disease of the central nervous system characterized by immune-mediated destruction of myelin. In patients with progressive deterioration, we have intensified immunosuppression to the point of myeloablation. Subsequently, a new hematopoietic and immune system is generated by infusion of CD34-positive hematopoietic stem cells (HSC). Three patients with clinical MS and a decline of their Kurtzke extended disability status scale (EDSS) by 1.5 points over the 12 months preceding enrollment and a Kurtzke EDSS of 8.0 at the time of enrollment were treated with hematopoietic stem cell (HSC) transplantation using a myeloablative conditioning regimen of cyclophosphamide (120 mg/kg), methylprednisolone (4 g) and total body irradiation (1200 cGy). Reconstitution of hematopoiesis was achieved with CD34-enriched stem cells. The average time of follow-up is 8 months (range 6-10 months). Despite withdrawal of all immunosuppressive medications, functional improvements have occurred in all three patients. We conclude that T cell-depleted hematopoietic stem cell transplantation can be performed safely in patients with severe and debilitating multiple sclerosis. Stem cell transplantation has resulted in modest neurologic improvements for the first time since onset of progressive disease although no significant changes in EDSS or NRS scales are evident at this time.

Activity of amoxycillin against enterococci and synergism with aminoglycoside antibiotics.

Amoxycillin was more active in vitro than ampicillin or benzylpenicillin against clinical isolates of enterococci. All 55 strains tested were sensitive to the three penicillins but 15 strains showed a high level of resistance to streptomycin and two of these were also insensitive to kanamycin. All strains were sensitive to gentamicin, which was the most active of the aminoglycoside antibiotics. The penicillins showed pronounced bactericidal activity against the enterococci but failed to sterilise cultures of these organisms. Combinations of penicillins and aminoglycosides invariably produced synergistic bactericidal effects which resulted in sterilisation of cultures of these organisms. Combinations of penicillins and aminoglycosides invariably produced synergistic bactericidal effects which resulted in sterilisation of cultures of enterococci provided that the strain was sensitive to the aminoglycoside moiety of the antibiotic combination. Synergism was not observed with a combination of a penicillin and an aminoglycoside when the enterococcus was resistant to the aminoglycoside. The data reported suggest that amoxycillin may have certain advantages, compared with ampicillin or benzylpenicillin, for the treatment of enterococcal infections.

Functional angiography of the head and neck.

The basic concepts of the territorial approach to angiography and intravascular embolization of vascular lesions of the head and neck are reviewed. Superselective arteriographic injections are performed in sequences and projections tailored to the suspected location of the vascular lesion. The resulting studies are then reviewed as a whole, so that a complete angiographic map of the tissue territory involved is considered. From this map the interventionalist selects the best and safest route for embolization, taking into account developmental and acquired constraints to blood flow and the presence of any potentially dangerous collateral flow toward normal nervous tissue. Case studies are presented to illustrate the most frequently encountered anatomic variants that may, by their presence within the circulation to be embolized, increase the risk of complication. Attention is directed toward the probability of intraprocedural changes in hemodynamic balance and flow direction, which also may increase risk and which may be modified by catheter techniques and minimized by meticulous attention to detail. This article should support the contention that functional angiography and careful attention to the resulting angiographic map are essential for efficacious and safe head and neck embolization.

High-resolution CT of mastoid sinus pneumocele with external auditory canal stenosis.

A 34-year-old man presented with tinnitus and conductive hearing loss. CT demonstrated an expansile, air-containing cavity contiguous with mastoid air cells, narrowing the external auditory canal. This is a case of symptomatic pneumocele resulting in an air collection beneath external canal lining, possibly related to an abnormality in mastoid fusion.

Spontaneous hemorrhage from a cerebral venous malformation related to thrombosis of the central draining vein: demonstration with angiography and serial MR.

A cerebral venous malformation ruptured after acute thrombosis of its central draining venous channel, leading to intracerebral hemorrhage. The CT, MR, and angiographic studies unequivocally demonstrated subsequent progressive recanalization of the malformation, providing proof that venous malformations can spontaneously bleed and that venous thrombosis may be associated with, and perhaps produce, the hemorrhage.

Osteocartilaginous tumors in the parapharyngeal space arising from bone exostoses.

Three osteocartilaginous tumors that arose from bone stalks were diagnosed by the demonstration, on multiplanar cross-sectional CT and MR images, of the site of stalk attachment to the adjacent mandible (one), skull base (one), and cervical vertebra (one). All three patients presented with signs and symptoms related to mass effect in the parapharyngeal region. Mass effect was more pronounced in the two cases of malignant degeneration (osteosarcoma, chondrosarcoma) than in the single case of benign osteocartilaginous exostosis. Differentiation of benign from malignant osteocartilaginous tumor may be accomplished by appreciating characteristic CT and MR features of the cartilage cap. The differential diagnosis of mass lesions of the parapharyngeal space should include tumors of osteocartilaginous origin.

Ocular ischemic syndrome during carotid balloon occlusion testing.

The use of a double-lumen balloon catheter for temporary occlusion testing of the internal carotid artery permits simultaneous perfusion of the distal internal carotid artery (and ophthalmic artery) with heparinized saline. If saline is infused too rapidly, the result may be total or partial replacement of oxygenated blood within the ophthalmic artery. This replacement may produce the signs and symptoms of ocular ischemic syndrome. These include ipsilateral orbital pain and progressive uniocular visual loss. Simple technical adjustments in the performance of the occlusion test can prevent the development of this syndrome. Failure to recognize the cause of the observed visual loss may produce the false impression of a positive occlusion test or may falsely suggest that a thromboembolic complication has occurred. Awareness of the occurrence of this syndrome should prevent confusion concerning the predictive result of provocative carotid occlusion testing.

Pituitary hypoplasia in patients with a mutation in the growth hormone-releasing hormone receptor gene.

BACKGROUND AND PURPOSE: Several anatomic abnormalities of the pituitary gland have been described as occurring in association with congenital growth hormone deficiency, including hypoplasia of the adenohypophysis, truncation of the pituitary stalk, and ectopia of the neurohypophysis. Their pathogenesis, however, is obscure. Normal pituitary development is dependent on the sequential expression of a series of ontogenetic factors. Growth hormone-releasing hormone (GHRH) is known to stimulate somatotroph proliferation, and a dwarf mouse model with a mutant GHRH receptor, the "little mouse," has a small anterior pituitary due to hypoplasia of the somatotrophs. We recently described the human homolog of the little mouse (dwarfism of Sindh), caused by a homozygous nonsense mutation in the GHRH receptor gene in a Pakistani kindred. We investigated MR imaging characteristics to gain information regarding the potential role of GHRH in human pituitary organogenesis. METHODS: MR images of the head were obtained of four affected male patients (age range, 22-29 years). Maximal anterior pituitary dimensions were determined from sagittal and coronal images, and pituitary volumes were estimated from cubic and ellipsoid formulae. The measurements were compared with normative values matched for age and sex. RESULTS: The adenohypophysis was small in each of the four patients. The maximal height for the anterior pituitary was 3 mm in three patients and 2 mm in one (mean +/- SD, 2.75 +/- 0.5 mm), which is significantly (P < .001) less than the expected height of 5.6 +/- 1.0 mm for men in this age group. Estimates of anterior pituitary volume in the patients ranged from 75 to 124 mm3 (104 +/- 21 mm3), which corresponds to 35% to 52% of the normal mean volume corrected for small head size (P < .005). No other cranial abnormalities were identified. CONCLUSION: We describe significant hypoplasia of the adenohypophysis occurring in four dwarfs with a nonsense mutation in the GHRH receptor. In addition to isolated growth hormone deficiency and severe dwarfism, affected patients have anterior pituitary hypoplasia, presumably due to somatotroph maldevelopment. Resistance to GHRH explains the hypoplasia of the adenohypophysis--a feature that contributes to growth hormone deficiency in this syndrome. This is one of the few instances in which the molecular basis of pituitary dysmorphogenesis has been identified.

Osteochondroma of the thoracic spine: an unusual cause of spinal cord compression.

A 24-year-old man with hereditary multiple exostoses had numbness of the lower extremities and difficulty walking. CT displayed a calcified extradural mass lesion within the spinal canal at T-8 causing cord compression. MR imaging showed it to be contiguous with the upper endplate of T-8, suggesting the diagnosis of osteochondroma, a rare cause of cord compression, and distinguishing the lesion from a calcified disk fragment.

Axial CT recognition of anteroposterior displacement of fourth ventricle.

The recognition of anterior or posterior displacement of the fourth ventricle on axial computed tomography (CT) has proven to be difficult because the apparent position of this structure is variable and dependent on scanning angle. In most cases direct visualization of a lesion and its relationship to normal anatomic structures allows for the correct assessment. However, in some instances it would be advantageous for lesion localization to be able to identify relatively subtle displacements of the fourth ventricle. This is possible on CT by determining the position of this structure relative to Twining's line (the line between the tuberculum sellae and torcula). The position of the fourth ventricle, tuberculum sellae, and torcula relative to an arbitrary fixed point can be established in virtually all cases, and thus the position of the fourth ventricle relative to Twining's line can be determined. In a control group of 100 patients with normal CT examinations, the ratio of the distance from the tuberculum sellae to the center of the fourth ventricle and the distance from the tuberculum sellae to torcula (Twining's line) was between 0.47 and 0.53. In 66% of the cases the ratio was 0.49-0.51. THere were 54 posterior fossa masses evaluated by this technique. Determination of fourth ventricular position by this method proved to be of particular value in recognizing brainstem glioma, and in determining the site of origin of laterally placed posterior fossa masses.

Falx and interhemispheric fissure on axial CT: II. Recognition and differentiation of interhemispheric subarachnoid and subdural hemorrhage.

Interhemispheric hyperdensity or unenhanced computed tomography was originally considered a sign of subarachnoid hemorrhage, the "falx sign." It has since been identified as a normal feature and has also been seen with interhemispheric subdural hemorrhage. To determine the differential features of interhemispheric hemorrhage, 50 patients with subarachnoid hemorrhage and 32 patients with interhemispheric subdural hematomas were reviewed. Subarachnoid hemorrhage produced anterior interhemispheric hyperdensity only, with a zigzag contour and extension from the calvarium to the rostrum of the corpus callosum. Interhemispheric subdural hematomas produce unilateral crescentic hyperdensities that are largest in the posterior superior part of the fissure, behind and above the splenium of the corpus callosum. Interhemispheric hyperdensity in children is more complex. Because the anterior part of the fissure is narrow in younger patients, subarachnoid hemorrhage may go undetected. Likewise, interhemispheric subdural hematomas in children are smaller and more difficult to recognize. They produce asymmetric thickening of the falx shadow with extension over the tentorium. They are, however, of great significance since they are generally seen in abused patients and carry a poor prognosis.

MR imaging of the normal and abnormal clivus.

We retrospectively reviewed 330 T1-weighted sagittal images, 80 T2-weighted sagittal images, and 83 gadopentetate-dimeglumine-enhanced scans of normal adults to determine the MR appearance of the normal adult clivus. MR images of 21 patients with an abnormal clivus (19 with tumor invasion and two with marrow reconversion) were also evaluated restrospectively and compared with those of the control group to assess MR features distinguishing the two groups. Our study revealed that a normal adult clivus consisted of low- and high-intensity portions mixed in various proportions on T1-weighted images. The low-intensity portion was isointense or hyperintense relative to the pons and always contained foci of bright signal intensity. The low-intensity tumor of a pathologic clivus tended to be hypointense relative to the pons (17/19), and was completely devoid of foci of bright signal intensity. The normal adult clivus was approximately isointense relative to the pons on T2-weighted images. Clival tumors were grossly hyperintense relative to the pons on T2-weighted images in 11 of 17 patients. In the remaining six patients, either a portion of or the entire lesion was isointense relative to the pons and, therefore, was not detectable on T2-weighted images. A normal adult clivus can enhance to some degree (19/83). Clival tumors were found to enhance intensely. A clivus of very low signal intensity (signal void) on T1- or T2-weighted images was always abnormal. The clivus with marrow reconversion was uniformly hypointense relative to the pons on T1-weighted images and isointense relative to normal marrow on T2-weighted images.(ABSTRACT TRUNCATED AT 250 WORDS)

Location of the central sulcus via cortical thickness of the precentral and postcentral gyri on MR.

PURPOSE: To determine whether relative cortical thickness measurements of the precentral and postcentral gyri can be used to differentiate the central sulcus from adjacent cortical sulci. METHODS: Turbo inversion-recovery MR imaging of the entire brain was done with scans parallel to the anterior commissure-posterior commissure line. Cortical thickness was measured in each hemisphere with a jeweler's eyepiece with 0.1-mm gradations. Three measurements were obtained perpendicular to the central, precentral, and superior frontal sulci, as determined by means of established anatomic methods. The ratios of cortical thickness on both sides of the central, precentral, and superior frontal sulci were calculated and compared. RESULTS: The mean ratio of precentral/postcentral gyri was 1.64 for the right hemisphere and 1.53 for the left hemisphere. The mean cortical thickness ratios were as follows: 1.01 for the right hemisphere and 3.01 for the left hemisphere across the precentral sulcus, and 1.03 for the right hemisphere and 0.99 for the left hemisphere across the superior frontal sulcus. CONCLUSION: Cortical thickness measurements across the central sulcus provide a method for locating the primary motor (precentral gyri) and primary somatosensory (postcentral gyri) cortices. The higher mean cortical thickness ratio across the central sulcus corresponds with known cytoarchitectonic relationships.

Contrast-enhanced MR of cerebral arteritis: intravascular enhancement related to flow stasis within areas of focal arterial ectasia.

The authors present a case of probable amphetamine-induced cerebral arteritis in a 31-year-old man with AIDS, testicular carcinoma, and recurrent pneumocystis pneumonia. MR enhancement was demonstrated in areas of focal arterial ectasia, presumably due to slow flow. CT, MR, and angiographic findings were strikingly confirmed postmortem. Angiography remains essential in the diagnosis of cerebral arteritis.