CASE 25: 77 y/o female patient with altered mental status

Patient with previous semi-vegetative state (alertness only), had sudden loss of consciousness and apparently partial complex seizures. She had progressive deterioration of her mental status and locomotion during a 2 year period.

An MR study was recommended. Here are the relevant images:

DWI were negative for restricted diffusion.

Diferentials please...

DX: Superior Mesenteric Artery (Wilkie's) Syndrome

Wilkie's syndrome has some peculiarity becasue of its rareness and also because of the important role of the radiologist to identify it for proper and rapid surgical approach.

Here are the findings:

CT revealed gas within a duodenal loop wich is also dilated but apparently there is no intra or extraluminal compression. We have to consider that in a normal setting, there is no gas in duodenum.Upper GI series confirmed the obstruction in the 3rd segment (horizontal) and dilation of the 2nd segment (vertical) of the duodenum. CT also showed a narrowing between abdominal aorta and superior mesenteric artery right in the passage of the duodenum consistent with Wilkie's. She underwent suergery that confirmed the duodenal impingement.

Superior mesenteric artery (SMA) syndrome is a very rare, life-threatening gastrovascular disorder characterized by a compression of the third portion of the duodenum by the abdominal aorta (AA) and the overlying superior mesenteric artery. The syndrome is typically caused by an angle of 6°-25° between the AA and the SMA, in comparison to the normal range of 38°-56°, due to a lack of retroperitoneal and visceral fat. In addition, the aortomesenteric distance is 2-8 millimeters, as opposed to the typical 10-20.

 It is also known as Wilkie's syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arterio-mesenteric occlusion.It is distinct from Nutcracker syndrome, which is the entrapment of the left renal vein between the AA and the SMA.

Until next time!..

http://en.wikipedia.org/wiki/Superior_mesenteric_artery_syndrome
http://emedicine.medscape.com/article/932220-overview#a0104

CASE 24: 24 y/o female patient with acute abdominal pain

This case was provided from the renowned Centro Medico Hospital in Guatemala City.

Here is the brief history. Patient had some chronic digestive problems and developed malnutrition and low body weight consequently. She could not eat an entire meal or solids. Parents thought it was an anorexic problem. One day ago she suffered from an acute abdominal pain in epigastrium associated with nausea and vomiting so they decided to bring her to the ED.

She underwent a CT scan. Here is the CT spot image:

With this finding, they suggested an upper GI series (shown below):

Findings and diagnosis?

DX: Xanthogranulomatous Cholecystitis

This was a biopsy proven case that wasn't on my first diagnosis arsenal as a differential.

US gave me the impression  that a bile stone was "impacted" in gallbladder.

CT findings were: focal gallbladder wall thickening with pseudo-impingement at fundus, with no adjacent stones only near the neck. It had IV contrast enhancement. No liver lesions.

Imaging characteristics were not as conclussive as wanted but there was a neoplasic component for sure. I reccomended to correlate with tumor markers especially Carbohydrate Antigen 19-9 (CA 19-9). Results were abnormal so, Gallbladder Carcinoma was the clinical-radiological diagnosis.

She went to surgery and a Cholecytectomy was performed. Biopsy of the specimen was reported as Xanthogranulomatous Cholecystitis (XGC).

XGC is a rare inflammatory disease of the gallbladder characterized by a focal or diffuse destructive inflammatory process, with accumulation of lipid laden macrophages, fibrous tissue, and acute and chronic inflammatory cells. In 1970, it was known by the descriptive term fibroxanthogranulomatous cholecystitis, but in 1981 the name xanthogranulomatous cholecystitis was proposed in a review of 40 cases from the Armed Forces Institute of Pathology. Its importance lies in the fact that it is a benign condition that may be confused with carcinoma of the gallbladder, which is associated with a poor prognosis.

XGC was initially described as a variant of chronic cholecystitis. However, while the latter is usually regarded as a benign condition with questionable clinical significance, xanthogranulomatous cholecystitis is an active and destructive process that can lead to significant morbidity as the inflammatory process usually extends into the gallbladder wall and adjacent structures. Thus, it should be considered a distinct clinical entity.

The pathogenesis of XGC is thought to be related to extravasation of bile into the gallbladder wall from rupture of Rokitansky-Aschoff sinuses or by mucosal ulceration. This event incites an inflammatory reaction in the interstitial tissue, whereby fibroblasts and macrophages phagocytose the biliary lipids in bile, such as cholesterol and phospholipids leading to the formation of xanthoma cells.

Gallstones may have an important role in the pathogenesis, since they appear to be present in all patients. It has been suggested that xanthogranulomatous cholecystitis is analogous to xanthogranulomatous pyelonephritis, which results from obstruction and stasis due to renal calculi.

 A few recent reports have shown a possible association of this disease with carcinoma of the gallbladder.
The inflammatory process often extends into neighboring organs, such as the liver, omentum, duodenum, and colon. The clinical importance of XGC lies in the fact that it can be confused radiologically with a gallbladder carcinoma.

Several reports demonstrated the radiological features of XGC. However, as some are nonspecific, it is often difficult to distinguish XGC from gallbladder carcinoma by the conventional imaging techniques of  ultrasonography, CT and MRI. Moreover, the fact that XGC can infrequently be associated with gallbladder carcinoma makes the differentiation more difficult.

http://www.uptodate.com/contents/xanthogranulomatous-cholecystitis
http://www.ajronline.org/content/148/4/727.long
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2721248/pdf/WJG-15-3691.pdf
http://www.surgpath4u.com/caseviewer.php?case_no=660&view=yes&h=&w=&fs=



Hope it was helpful as it was to me..until next time...

CASE 23: 55 y/o female with RUC pain

Patient came to the ED with history of acute abdominal pain, suspected (+) Murphy's sign and mild jaundice. She had previous ultrasound showing gallstones several months ago. Calculous Cholecystitis was suspected so an ultrasound was conduced.

Bloodwork showed an obstructive pattern on billirubin serum levels but also high in Lactate Dehydrogenase and  mild elevation of Alanine Transaminase and Aspartate Transaminase levels. This was a concern to me on hepatic parenquimal integrity.

Here are the US gallbladder (VB) images: (soory the poor quality but click to enlarge)

The suitable exam to better characterize findings is a CT:

I wanted to see first a non contrast phase due to some suspected areas shown in ultrasound that produced posterior acoustic shadowing.

I wanted also a late phase (120 segs) to see the demeanor of the lesion.

Findings?..Differentials?..


Soon the conclusion..

DX: Left Carotid Artery Loop or Coil. Vertebral artery artifact

Though this case is incomplete and had technical deficiencies (such as: 1 Tesla Magnet; TOF sequence; no arteriogram; and lack of showing aortic arch and carotid and subclavian proximal segments). All of this issues makes diagnosis a little difficult.

First of all, TOF sequence itself always has its ups and downs because of low sensitivity. In addition, 3D reconstruction predisposes more artifacts. Many of these artifacts can cause pseudostenosis or segmental blurring. This has to be expected when looking at this sequence. To be assured, lets see the raw data previous 3D reformat.

There is an irregular trajectory of the left carotid artery in the upper cervical portion previous to petrous segment. This makes the diagnosis of a Carotid "Loop" more plausible. Clinical symptoms support this posibility. On the other hand, we did not see an abnormal left vertebral artery, that was shown in the 3D virtual reconstruction.

In this case, and arteriogram had to be done so it was reffered to another institution. (Finantial issues).

Carotid Loop or Coil:

The association between kinking or coiling of the internal carotid artery (ICA) and cerebrovascular insufficiency was first noted in 1951. Since then several reports have dealt with the clinical relationship between carotid elongation and kinking and cerebrovascular disease. Although conclusive evidence linking the two is still lacking, certain inferences can now be drawn.

Looping and kinking of the ICA has been observed in infants and even in fetuses. The cause of these loops is related to embryological development. The vessel is formed from the third aortic arch and from the dorsal aorta; hence, in the embryo it is normally kinked.
Straightening occurs when the fetal heart and large vessels recede in the thoracic cavity. If the embryological state persists, it produces different kinds of undulations, loops, and kinks. This anomaly generally does not become symptomatic until later in life.

There appears to be no relationship between the severity of the kinking and either rising blood pressure or increasing age. The role of degenerative changes in the vessel wall remains uncertain. If this factor were important, then kinks should be more severe in older individuals because degenerative changes increase with age; however, this is not generally the case. At best the cause for looping and kinking of the carotid artery may be ascribed partly to embryological development and partly as secondary to atherosclerotic changes.

http://www.crcnetbase.com/doi/abs/10.1201/9780203912904.ch54
http://stroke.ahajournals.org/content/6/6/649.full.pdf
http://bubbasoft.org/carotid_collaterals/carotid_loops.htm


All coments are welcome...have a nice day.