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.

Should patients with autosomal dominant polycystic kidney disease be screened for cerebral aneurysms?

Autosomal dominant polycystic kidney disease is a genetic disorder affecting 1 in 1000 people worldwide and is associated with an increased risk of intracranial aneurysms. It remains unclear whether there is sufficient net benefit to screening this patient population for IA, considering recent developments in imaging and treatment and our evolving understanding of the natural history of unruptured aneurysms. There is currently no standardized screening protocol for IA in patients with ADPCKD. Our review of the literature focused on the above issues and presents our appraisal of the estimated value of screening for IA in the setting of ADPCKD.

Cervical epidural arteriovenous fistula presenting with radiculopathy: transvenous embolization using Onyx.

We present an uncommon case of cervical epidural arteriovenous fistula presenting with radiculopathy that was successfully treated with transvenous embolization using Onyx.

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.

Cerebrospinal fluid leak treated by aspiration and epidural blood patch under computed tomography guidance.

BACKGROUND AND OBJECTIVES: Cerebrospinal fluid (CSF) leakage secondary to surgery of the spine is usually treated by drainage of CSF through a subarachnoid catheter or surgical repair of the dural tear. We present 2 cases in which the pseudomeningocele was treated by aspiration of the leaked CSF and blood patch under computed tomography (CT) guidance. CASE REPORT: Two patients had headache after spine surgery. Physical examination showed a bulging accumulation of fluid at the laminectomy site. Aspiration of the fluid followed by injection of the patients' blood was performed aseptically under CT guidance. The patients had resolution of their headache, and follow-up showed no recurrence of the CSF leak. CONCLUSIONS: CSF leak secondary to a surgical tear of the dura can be successfully treated by aspiration of the fluid followed by injection of the patient's blood. CT guidance is recommended to assess the extent of the CSF leakage, determine the degree of evacuation of the leaked CSF, and to confirm the injection of the blood into the epidural space and the space created by the pseudomeningocele.

The properties of retention intervals and their affect on retaining prospective memories.

Five experiments were conducted to explore how the character of the retention interval affected event-based prospective memory. According to the canons of retrospective memory, prospective performance should have been worse with increasing delays between intention formation and the time it was appropriate to complete an action. That result did not occur. Rather, prospective memory was better with increasing retention intervals in Experiments 1A, 1B, and 3. In manipulating the nature of the retention interval, the authors found that there were independent contributions of retention interval length and the number of intervening activities, with more activities leading to better prospective memory (Experiments 2 and 3). The identical retention intervals did not improve retrospective memory in Experiment 4. Theoretical explanations for these dissociations between prospective and retrospective memory are considered.

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.

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.

Persistence of the notochordal canal: MR and plain film appearance.

We report a case of an unusually prominent persistent notochordal canal involving the T12-L5 vertebrae. This rare anatomic variation was discovered as an incidental finding in a patient with lymphoma undergoing MR imaging for evaluation of back pain. MR images showed a vertically oriented canal contiguous with the intervertebral disks traversing the anterior aspect of each affected vertebral body. Plain films showed a sclerotic rimmed central channel that flared at each vertebral endplate to merge with the disk spaces.

Spontaneous intracranial hypotension: spinal MR findings.

We report three patients with spontaneous intracranial hypotension in whom spinal MR imaging revealed ventral extradural fluid collections that were centered at the cervicothoracic junction in two patients and extended throughout the entire spine in the third patient. These spinal fluid collections most likely resulted from the accumulation of CSF at the site of dural leakage. Knowledge of this association can be helpful in the selection of imaging studies to facilitate diagnosis and treatment.

Imaging of angiographically occult cerebral vascular malformations.

The angiographically occult vascular malformations represent an important cause of cerebral pathology. The propensity for significant yet often limited symptomatology provides for frequent challenge in clinical management. Understanding in diagnostic evaluation of occult vascular lesions is often limited by varying application of pathologic diagnosis and classification. This article attempts to clarify issues of classification while also discussing imaging evaluation and its role in clinical management.