Henry W. Pribram

Computed tomography of Paget disease of the skull versus fibrous dysplasia

Abstract Objective. Radiologists are often challenged to review CT examinations of the skull without pertinent clinical information or plain radiographs. Skull lesions of fibrous dysplasia (FD) may often be confused with Paget disease (PD). The purpose of this article is to evaluate radiographic similarities and to find the signs that can differentiate PD from FD of the skull on head CT and to describe the CT imaging features of PD and FD. Design and patients. CT scans of the skull in eight cases of PD, 18 cases of FD (13 cases of skull and facial bones, five cases of only facial bones) and 10 normals were studied retrospectively. Results. Ten features were found to be similar in PD and FD and 10 other features were found to be dissimilar. The frequency of the 10 differentiating features was evaluated to determine their reliability in distinguishing one disorder from the other. The differentiating features in order of significance include: (1) “groundglass” appearance, (2) symmetry, (3) involvement of the paranasal sinuses, (4) thickness of the cranial cortices, (5) involvement of the sphenoid bone, (6) orbital involvement, (7) nasal cavity involvement, (8) presence of a soft tissue mass, (9) maxillary involvement, and (10) the presence of cyst-like changes. Conclusion. These 10 signs improve the radiologist’s skill in differentiating FD and PD.

A new method for treating carcinomatous biliary obstruction with intracatheter radium

We describe a new method for relieving biliary obstruction due to malignant solid tumors. The method consists of placement of radium needles in a Ring biliary drainage catheter for three days. After removing the radium needles, the catheter is left in place to allow for repair of irradiated tissues, then it is removed. The object is to leave the patient with a patent biliary tree without a biliary drainage prosthesis. The clinical course of six patients treated by this method is described, and the autopsy findings in three cases are outlined. Three patients died with a serum total bilirubin ranging from 1–4.5 mg/100 ml, two to three months after removing the drainage catheter. Two patients died before the bile drainage catheter could be removed. One patient is alive with a bile drainage catheter in place. Possible modifications in technique and catheters are being considered.

Fetal hydrocephalus and ear anomalies associated with maternal use of isotretinoin

Collins syndrome and may have resulted f rom defective migra t ion of neural crest cells f rom the first and second branchia l arches?. 2 Including this case, there are now 16 women retrospectively identified as having taken isotretinoin dur ing pregnancy. 3,4 Outcomes from these pregnancies include six spontaneous abortions, one normal infant , and nine infants with malformat ions . Of 18 women identified dur ing pregnancy who did not in ter rupt the pregnancy, the outcomes have included 13 spontaneous abort ions, four normal children, and one abnormal child. Therefore, in 15 pregnancies t ha t have come to term, five chi ldren were normal at b i r th and 10 were abnormal . O f the 10 abno rma l children, seven had microt ia with or wi thout agenesis of the external ear canal, nine had cent ra l nervous system abnormal i t ies , and four had congeni tal hear t defects. A l though the guidelines for using isotret inoin are established, each physician must decide whether the benefit of prescribing any retinoid outweighs the chance tha t the pa t ient will conceive while taking the d rug? Despite cur ren t warnings, with increased use of isotret inoin in women of chi ldbear ing age more f requent inadver ten t exposures have occurred. Unt i l the relat ionship between the dose and dura t ion of exposure with the likelihood and severity of a mal format ion is be t ter understood, the desirabil i ty of cont inuing the pregnancy af ter an exposure should be discussed. Physicians should document tha t sexually active female pat ients are not p regnant when the drug is begun, are using effective b i r th control methods, and are provided with wr i t ten informat ion about the teratogenic effects of this drug.

Xanthogranulomas of the third ventricle.

Two patients with xanthogranuloma of the 3rd ventricle are described. In each instance the tumor obstructed the interventricular foramina and caused dilatation of the lateral ventricles and signs of increased intracranial pressure; computerized tomography demonstrated the dense, nonenhancing lesion in the anterior 3rd ventricle. The pathogenesis of these rare tumors is discussed. These cases affirm the need to consider xanthogranuloma in the differential diagnosis of anterior 3rd ventricle tumors in adults.

Solitary osteochondroma with spinal cord compression

Osteochondromas are unusual in the spine, and they are very rarely present with compression of the spinal cord. Two cases are reported with delineation of the tumor by metrizamide myelography and computed tomography.

Septation in Syringomyelia and Its Possible Clinical Significance

A 3-year-old boy with a septated syrinx and several other dysraphic features is presented. The literature regarding the cause, diagnosis, and pathology of septation in syringomyelia is reviewed. This case raises two points. First, the septated pattern may be associated with an elevated pressure in the syrinx. Second, despite the septations, the syrinx may communicate freely.

Spinal Cord Infarction Following Vertebral Angiography: Clinical and Pathological Findings

Abstract Spinal cord damage from radiographic contrast material has been known to occur in both spinal and nonspinal angiographic procedures. Reported here is cervical spinal cord injury (SCI) during vertebral angiography. During the procedure, the patient displayed signs of acute cervical spinal cord irritation that should have been taken as a warning of impending injury. Autopsy 9 years later showed evidence of central cervical spinal cord necrosis. The pathological findings are similar to those seen in animal models of contrast media-induced SCI; and the pathophysiological mechanisms of such injury are discussed.

Spontaneous closure of a high-risk dural arteriovenous malformation of the transverse sinus

The case of a 44-year-old woman with tinnitus of her left ear is presented. An arteriogram demonstrated a posterior dural arteriovenous malformation (AVM) on that side with drainage via dilated cortical veins into the superior sagittal sinus. No intervention was undertaken. When angiography was repeated 7 years later, no dural AVM was seen.

Traumatic pseudoaneurysm of the cervical carotid artery: the value of arteriography

A patient who sustained a gunshot wound to the neck and subsequent blunt trauma is presented. No angiogram was performed after the initial injury. Subsequently, the patient developed a cerebral embolus from a thrombus dislodged from a pseudoaneurysm of the cervical carotid artery, resulting in complete occlusion of the distal internal carotid artery. After antiplatelet therapy, the distal internal carotid artery recanalized, and the pseudoaneurysm occluded spontaneously. We stress the need for the use of arteriography in the initial evaluation of penetrating injuries to the neck and the utility of repeated arteriograms for further treatment planning as these traumatic lesions can change with time.

FETAL HYDROCEPHALUS AND EAR ANOMALIES ASSOCIATED WITH THE MATERNAL USE OF ISOTRETINOIN

A 16 year old mother took 40 mg of isotretinoin (13-cis retinoic acid) daily for cystic acne during the first 130 days of pregnancy. An enlarged fetal head necessitated Caesarian section. The live born infant showed an increased cranialfacial ratio, low set and simplified auricles and an atretic ear canal A.D.. Neurological responses were depressed with poor visual tracking and minimal pupillary constriction to light. At age 5 months there had been no developmental progress. Brain stem evoked responses were abnormal on the right and non-elicitable on the left. An intravenous pyelogram was negative. The CT findings included aqueductal stenosis and an area of cerebral tissue loss in the right occipital region. The right hemicranium was enlarged compared to the left. In rats and rabbits, isotretinoin is similar to vitamin A in producing teratogenic effects involving the ears and brain. Taken together with our observation on the outcome in this human infant, it is suggested that sexually active women under treatment for acne not be given isotretinoin unless an effective form of contraception is utilized and the potential risk to the fetus is described. Should conception ensue, the physician and patient should discuss the desirability of continuing the pregnancy.