Sushila Ladumor, MD

Case History: A 36-year-old male presented with weak stream and pain.

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Case History: 36-year-old male presented with weak stream and pain.

36 y/o Male, Weak Stream, Pain

 A 42 year-old male patient referred to rule out intestinal obstruction; history of appendectomy and laparotomy.

 There are surgical staples in the right hypochondriac region indicating previous resection side. There is obvious retroperitoneal predominately low density mass of about 12 cm x 8 cm with multiple cystic areas with the thick peripheral rim with anterior displacement of the second part of the duodenum and mesenteric vessels with distended stomach with persistence of oral contrast in gastric lumen even in delayed series suggestive of partial outlet obstruction Proximal small gut loops are distended and there is obvious narrowed segment in the distal small gut with associated bowel wall thickening in right lower quadrant, suggestive of distal intestinal obstruction.

Paragangliomas are rare tumors that arise from extra adrenal chromaffincells. During embryogenesis, neural crest cells migrate to diverse locations in the body and differentiate into chief and sustentacular cells, which are designated as paraganglia.

Extra luminal perigastric masses are frequently considered to be gastrointestinal stromal tumors (GISTs) or other soft tissue sarcomas, when histological confirmation is not possible. Paragangliomas may have a similar radiographic appearance. In the absence of histological diagnosis and symptoms of catecholamine excess, paragangliomas may be mistaken for GISTs. Perigastric abdominal mass presumed to be a GIST after nondiagnostic biopsy, is presented.

On abdominal CT, there are no unique imaging characteristics specific for paragangliomas. Consequently, these tumors may be mistaken for other primary epithelial or mesenchymal abdominal tumors. For instance, GIST, the most common sarcoma of the gastrointestinal tract, may have a similar radiographic appearance. They are often large (84 percent N 5 cm) tumors arising from the stomach with heterogeneous enhancement (84 percent) and central necrosis (37 percent). Retroperitoneal paragangliomas affect mainly adults in the fourth or fifth decade of life, with no sex predilection.

While size and location help to differentiate GISTs from paragangliomas, correlation with clinical symptoms is needed. A presumed GIST considered to be resectable does not necessarily warrant preoperative biopsy, and thus further diagnostic workup is not pursued.

Articles

Paraganglioma

CASE 1: An elderly male patient with long term history of right inquinal swelling presented to emergency department with acute pain in abdomen along with nausea, vomiting, distension of abdomen and increase in swelling and tenderness in right inquinal region.

Frontal erect abdominal X-ray shows only two dilated air-fluid level on right side. Air and fecal matter in colon. In view of history CT scan of abdomen recommended.

Axial CT with contrast images of abdomen shows hugely distended stomach and dilated small bowel.

Continuous axial CT with contrast images of abdomen shows dilated small bowel with right inguinal hernia containing dilated mildly thick wall small bowel and some fluid.

Continuous axial CT with contrast images of abdomen shows right inguinal hernia containing dilated mildly thick wall small bowel and some fluid. Patient has penile prosthesis.

Coronal reformatted images of abdomen shows hugely distended stomach, dilated small bowel and right inguinal hernia containing dilated mildly thick wall small bowel and some fluid represent incarcerated hernia causing small bowel obstruction.

Saggital and coronal reformatted images of abdomen shows hugely distended stomach, dilated small bowel and right inguinal hernia containing dilated mildly thick wall small bowel and some fluid represent incarcerated hernia causing small bowel obstruction.

Findings represent incarcerated hernia. Emergency physician informed immediately for further management.

CASE 2: An elderly male patient with previous history of abdominal surgery and long term swelling at the site of surgical scar at left side of lower abdomen and now presented to emergency with acute abdominal pain and distension along with nausea, vomiting and increase in swelling with tenderness at the site of swelling.

Supine X-ray abdomen shows dilated small bowel indicating small bowel obstruction.

Erect X-ray abdomen shows multiple dilated air-fluid level in small bowel, indicating small bowel obstruction.

Axial images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect, distal bowel loops are not dilated represent incarcerated hernia.

Axial images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect with transitional zone, distal bowel loops are not dilated represent incarcerated hernia.

Axial images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect with transitional zone, distal bowel loops are not dilated represent incarcerated hernia. Multiple non complicated colonic diverticulum.

Reformatted images of CT abdomen with oral and intravenous contrast shows dilated proximal small bowel with defect in left side of abdomen containing omental fat in upper aspect and bowel loop with some fluid and fat stranding in lower aspect with transitional zone, distal bowel loops are not dilated, no pneumatosis, represent incarcerated hernia. Multiple non complicated colonic diverticulum.

Emergency physician informed immediately for further management.

Definition: A hernia is the protrusion of an organ or the fascia of an organ through the wall of the cavity that normally contains it. There are different kinds of hernia, each requiring a specific management or treatment.

Indirect hernia - An indirect inguinal hernia follows the tract through the inguinal canal. This results from a persistent process vaginalis. The inguinal canal begins in the intra-abdominal cavity at the internal inguinal ring, located approximately midway between the pubic symphysis and the anterior iliac spine. The canal courses down along the inguinal ligament to the external ring, located medial to the inferior epigastric arteries, subcutaneously and slightly above the pubic tubercle. Contents of this hernia then follow the tract of the testicle down into the scrotal sac.

Direct hernia - A direct inguinal hernia usually occurs due to a defect or weakness in the transversalis fascia area of the Hesselbach triangle. The triangle is defined inferiorly by the inguinal ligament, laterally by the inferior epigastric arteries, and medially by the conjoined tendon.

Femoral hernia - The femoral hernia follows the tract below the inguinal ligament through the femoral canal. The canal lies medial to the femoral vein and lateral to the lacunar (Gimbernat) ligament. Because femoral hernias protrude through such a small defined space, they frequently become incarcerated or strangulated.

Umbilical hernia - The umbilical hernia occurs through the umbilical fibromuscular ring, which usually obliterates by 2 years of age. They are congenital in origin and are repaired if they persist in children older than age 2 to 4 years.

Richter hernia - The Richter hernia occurs when only the antimesenteric border of the bowel herniates through the fascial defect. The Richter hernia involves only a portion of the circumference of the bowel. As such, the bowel may not be obstructed, even if the hernia is incarcerated or strangulated, and the patient may not present with vomiting. The Richter hernia can occur with any of the various abdominal hernias and is particularly dangerous, as a portion of strangulated bowel may be reduced unknowingly into the abdominal cavity, leading to perforation and peritonitis.

Incisional hernia - This iatrogenic hernia occurs in 2 percent to 10 percent of all abdominal operations secondary to breakdown of the fascial closure of prior surgery. Even after repair, recurrence rates approach 20 percent to 45 percent.

Spigelian hernia - This rare form of abdominal wall hernia occurs through a defect in the spigelian fascia, which is defined by the lateral edge of the rectus muscle at the semilunar line (costal arch to the pubic tubercle) The two subtypes are interstitial and subcutaneous, which are best defined using CT and assist with optimizing the surgical approach when indicated.

Obturator hernia - This hernia passes through the obturator foramen, following the path of the obturator nerves and muscles. Obturator hernias occur with a female-to-male ratio of 6:1, because of a gender-specific larger canal diameter and predominately in the elderly. Because of its anatomic position, this hernia presents more commonly as a bowel obstruction than as a protrusion of bowel contents.

Hernia symptoms and signs: The signs and symptoms of a hernia can range from noticing a painless lump to the severely painful, tender, swollen protrusion of tissue that you are unable to push back into theabdomen (an incarcerated strangulated hernia).

- It may appear as a new lump in the groin or other abdominal area.

- It may ache but is not tender when touched.

- Sometimes pain precedes the discovery of the lump.

- The lump increases in size when standing or when abdominal pressure is increased (such as coughing).

- It may be reduced (pushed back into the abdomen) unless very large.

- It may be an occasionally painful enlargement of a previously reducible hernia that cannot be returned into the abdominal cavity on its own or when you push it.

- Some may be chronic (occur over a long term) without pain.

- An irreducible hernia is also known as an incarcerated hernia.

- It can lead to strangulation (blood supply being cut off to tissue in the hernia).

- Signs and symptoms of bowel obstruction may occur, such as nausea and vomiting.

- This is an irreducible hernia in which the entrapped intestine has its blood supply cut off.

- Pain is always present, followed quickly by tenderness and sometimes symptoms of bowel obstruction (nausea and vomiting).

- The affected person may appear ill with or without fever.

- This condition is a surgical emergency.

Sushila Ladumor, MD, FRCR, consultant radiologist with multi-modality imaging experience, working in Medical Imaging Department, King Abdulaziz Medical City, Riyadh, Saudi Arabia

Incarcerated hernia: X-ray and CT scans evaluate inquinal swelling and and long-term swelling in elderly males.

Incarcerated Hernia

Clinical History: A 33-year-old female with abdominal distension and suspected ovarian mass.
CT scan of abdomen and pelvis done with oral and intravenous contrast with multiple axial sections along with coronal and sagittal reformation.

Findings: Large abdomino-pelvic cystic mass with thin septations. No enhancing nodule. Right ovary is seen and appears within normal limit. Features are suggestive of benign mucinous cyst adenoma of left ovary.

Multi-planner, multi-sequential MRI of abdomen and pelvis performed with and without contrast. Large multi-loculated cystic lesion arising from the left adnexa measuring maximum dimension of 30 x 11 x 27 cm in craniocaudal, AP, and mediolateral dimension.

Post gadolinium T1 images shows no enhancement of mass or septa.
 

The lesion demonstrated multiple internal thin septations. The mass exerts significant mass effect on the bladder and the uterus which is shifted to the left side. The right adnexum appears unremarkable. No free fluid identified in the pelvis. No obvious retroperitoneal lymph nodes seen. No obvious carcinomatosis identified. The appearance of the lesion is mostly in keeping with a benign epithelial ovarian neoplasm. Given the size of the mass and the presence of multiple septations, the appearance is most likely in keeping with mucinous cystadenoma.

Post-op pathological diagnosis: Mucinous cystadenoma.

Overview: Mucinous cystadenoma is a type of tumor in the cystadenoma grouping. There are four major categories of ovarian tumors:

1. Epithelial tumors (65 percent to 75 percent) - serous or mucinous cystadenoma/carcinoma, clear cell carcinoma, Brenner tumor
2. Germ cell tumors (15 percent) - dysgerminoma, embryonal cell cancer, choriocarcinoma, teratoma
3. Sex-chord-stromal tumors (5 percent to 10 percent) - granulosa cell tumor, thecoma, fibroma
4. Metastatic tumors (10 percent) - uterine, stomach, colon, breast, lymphoma

Mucinous cystadenomas make up 15 percent to 20 percent of all ovarian tumors. They often become very large and can extend up into the abdomen. Pseudomyxoma peritonei can result if the tumor ruptures and spills its contents into the abdomen.

These tumors are usually evaluated using ultrasound, CT scan, or MRI. Findings on imaging studies are nonspecific. These ovarian tumors are usually multi-septated, cystic masses with thin walls. They also contain varying amounts of solid tissue which consists of proliferating stromal tissue, papillae, or malignant tumor cells.

Mucinous cystadenomas are divided into three categories: benign, borderline, and malignant.
Survival is largely dependent on the histology of the tumor, with a 10-year survival rate of 100 percent for benign tumors, 60 percent for borderline tumors, and only 34 percent for the malignant subtype. There is some difference in ages of the peak incidence for the different subtypes with considerable overlap as described below. In general, benign tumors tend to present earlier, while malignant tumors are often seen later in life.

Benign mucinous cystadenomas compose 80 percent of mucinous ovarian tumors and 20 percent to 25 percent of benign ovarian tumors overall. The peak incidence occurs between 30 to 50 years of age. Benign tumors are bilateral in 5 percent to 10 percent of cases. Borderline mucinous cystadenomas make up about 10 percent of mucinous ovarian neoplasms and are bilateral in 10 percent of cases.

Malignant mucinous cystadenoms are rare, and encompass 10 percent of mucinous ovarian tumors and 5 percent to 10 percent of primary malignant ovarian neoplasms overall. They are bilateral in 15 percent to 30 percent of cases and have a peak incidence between 40 to 70 years of age. It can present in the ovary.

http://emedicine.medscape.com/article/255865-overview

http://www.annclinlabsci.org/content/33/4/465.abstract

http://journals.lww.com/intjgynpathology/Abstract/2005/01000/Mucinous_Tumors_of_the_Ovary__A_Review.3.aspx

http://en.wikipedia.org/wiki/Mucinous_cystadenoma

Mucinous cystadenom: 33-year-old female with abdominal distension and suspected ovarian mass. CT scan of abdomen and pelvis done with oral and intravenous contrast with multiple axial sections along with coronal and sagittal reformation.

Mucinous Cystadenoma

Clinical History: A 45-year-old man with abdominal pain receives a CT angiogram. 

Imaging: CT angiogram with three dimensional reformation demonstrates acute angulation (kinking) and narrowing of the proximal celiac axis. There is minimal poststenotic dilatation, which creates a “hooked” appearance.

Diagnosis: Median arcuate ligament syndrome

Discussion: The median arcuate ligament is a fibrous arch that unites the diaphragmatic crura on either side of the aortic hiatus. The ligament usually passes superior to the origin of the celiac axis. In 10 percent to 24 percent of people, however, the ligament may cross anterior to the artery; in some of these individuals, the ligament may actually compress the celiac axis, compromising blood flow and causing symptoms.

Although the diagnosis of celiac artery compression is traditionally made by using conventional angiography, the use of thin-section multidetector computed tomography (CT) and of 3D imaging techniques has greatly improved the ability to obtain detailed images of the mesenteric vessels noninvasively. Compression of the celiac axis by the median arcuate ligament produces characteristic findings visible at CT angiography, also referred to as celiac artery compression syndrome.

The median arcuate ligament syndrome (or celiac artery compression syndrome) was first described in 1963 by Harjola. The definition of the syndrome relies on a combination of both clinical and radiographic features. It typically occurs in young patients (20 to 40 years of age) and is more common in thin women, who may present with epigastric pain and weight loss. The abdominal pain may be associated with eating, but not always. At physical examination, an abdominal bruit that varies with respiration may be audible in the midepigastric region. Symptoms are thought to arise from the compression of the celiac axis, resulting in a compromise in blood flow.

Median arcuate ligament syndrome is a controversial entity. It is clear that the abnormally low insertion of the median arcuate ligament can be found in normal asymptomatic people. In a small subset of patients, however, the compression of the celiac axis can cause symptoms that may be relieved after surgical decompression. CT angiography can play a role in the diagnosis of this condition by demonstrating the characteristic focal narrowing of the celiac artery in patients presenting with the appropriate clinical symptoms.

http://radiographics.rsna.org/content/25/5/1177.full

http://radiology.rsna.org/content/228/2/437.full

Sushila Ladumor, MD, FRCR, consultant radiologist with multi-modality imaging experience, working in Medical Imaging Department, King Abdulaziz Medical City, Riyadh, Saudi Arabia. 

A 45-year-old man with abdominal pain receives a CT angiogram. CT angiogram demonstrates acute angulation and narrowing of the proximal celiac axis.

Median Arcuate Ligament Syndrome

A 73-year-old man with known case of liver cirrhosis receives a CT scan. A triphasic contrast study of the liver is performed.

Portal vein thrombosis (arrow), splenomegaly, collaterals at splenic hilum, ascitis.

Findings: Liver shows cirrhotic profile with low density area in precontrast phase. In arterial phase there is multifocal diffuse enhancement and wash out in portal phase seen involving both the lobe of liver consistent with multifocal hepatocellular carcinoma (HCC), portal vein thrombosis (tumor thrombus), splenomegaly, collaterals at splenic hilum, ascitis

Hepatocellular carcinoma (HCC), which is the sixth most common malignancy worldwide, usually develops in patients with liver cirrhosis. HCC is the most common primary hepatic tumor, and is a primary malignancy of hepatocellular origin.

Hepatocellular carcinoma frequently arises in the setting of cirrhosis, appearing 20 to 30 years following the initial insult to the liver. However, 25 percent of patients have no history or risk factors for the development of cirrhosis. The extent of hepatic dysfunction limits treatment options, and as many patients die of liver failure as from tumor progression. Tumors are multifocal within the liver 75 percent of the time. Late in the disease, metastases may develop in the lung, portal vein, periportal nodes, bone, or brain.

Patients generally present with symptoms of advancing cirrhosis.
• Pruritus
• Jaundice
• Splenomegaly
• Variceal bleeding
• Cachexia
• Increasing abdominal girth (portal vein occlusion by thrombus with rapid development of ascites)
• Hepatic encephalopathy 
• Right upper quadrant pain (uncommon)

Preferred examination: Cross-sectional imaging with computed tomography scanning and magnetic resonance imaging (MRI) is most commonly used to detect hepatocellular carcinoma. CT is frequently the first examination; however, MRI has superior contrast resolution and may better detect lesions less than 1 cm in diameter.

Ultrasonography can be sensitive in detecting HCC and, depending on the operator, can detect small lesions. US can evaluate for vascular invasion of the portal and hepatic veins through color Doppler imaging. Nuclear medicine imaging, angiography, and plain films are less useful.

Limitations of techniques: Plain films are nonspecific but may show a mass in the upper abdomen if the HCC is large. Nuclear medicine provides relatively nonspecific findings. The HCC may present as a "cold" defect on a sulfur-colloid study or may demonstrate uptake of radiopharmaceuticals if the mass produces bile. Gallium uptake is seen in 90 percent of HCCs.

The US appearance of HCC varies; it may be hyperechoic or hypoechoic. A small hyperechoic HCC may be confused with hemangioma. Perform CT scanning in hepatic arterial, portal venous, and delayed phases. Similarly, if MRI is used, precontrast, arterial, venous, and delayed phases are essential. Enhancement patterns of regenerative, dysplastic, and HCC nodules overlap; therefore, nodules of cirrhosis may not be differentiated from small HCCs. Angiography study may show increased vascularity of other hepatic tumors, including benign masses.

http://emedicine.medscape.com/article/282814-clinical

http://emedicine.medscape.com/article/369226-overview#a20

http://radiology.rsna.org/content/263/2/590.full

http://radiology.rsna.org/content/219/2/445.full

Hepatocellular Carcinoma

Clinical History: A 69-year-old male patient presented with a three-week history of right upper quadrant abdominal pain, pyrexia.

Plain X-Ray of the abdomen shows gas-filled structure in the right upper quadrant, most likely air filled gall bladder; for clinical correlation and further investigation.

Ultrasound shows the gallbladder is not fluid-filled and appears filled with air showing posterior shadowing. Some air in IHBR. 

CT confirms the findings on plain film and ultrasound. CT images show gallbladder is filled with air and minimal fluid. Air in intra hepatic ducts represents gangrenous cholecystitis.

Discussion: Gangrenous cholecystitis (GC) is a serious complication of acute cholecystitis. It is the result of marked distension of the gallbladder causing increased tension in the gallbladder wall. Associated inflammation leads to ischemic necrosis of the wall, with or without associated cystic artery thrombosis.

It is more common in men and in patients with co-existing cardiovascular disease and leukocytosis - white cell count (WCC) greater than 17 × 109/L. Other associated factors include diabetes, critical illness and a high C-reactive protein (CRP) level. Pre-operative diagnosis of this condition may prove difficult. Once suspected, patients with GC generally undergo emergency cholecystectomy in order to avoid life-threatening complications.

GC is the last stage of gallbladder inflammation and, in spite of its grave prognosis, its diagnosis can be elusive, both clinically and on laboratory investigation. The incidence of GC ranges from 2 percent to 29.6 percent in all patients with acute cholecystitis, in various surgical series, and generally occurs in older patients. Many factors have been implicated in its formation. Fagan et al. demonstrated that nine variables were associated with GC, but Contini et al. showed that there is no single clinical or laboratory finding, apart from a high WCC, predictive of severe inflammation of the gallbladder.

Contini et al. showed that the time of hospitalization delay plays a crucial role in the formation of GC. The time between the onset of symptoms and hospital admission was significantly longer in patients with GC. The patient's history (timely or delayed admission) and physician's attitude (general practitioner and/or surgeon) are likely to play a role in the progression towards a severe necrosis of the gallbladder wall.

Ultrasonography usually serves as the first-line imaging modality for the evaluation of patients with clinically suspected acute cholecystitis. However, CT can play an important role in evaluation of these patients if sonography is inconclusive. The hallmark on sonography of GC is the presence of heterogeneous or striated thickening of the gallbladder wall, which is often irregular with projections into the lumen and pericholecystic fluid collections. The presence of intra-luminal membranes representing desquamative gallbladder mucosa is a specific finding but it is less common. The accuracy of pre-operative ultrasound in diagnosing GC remains uncertain. Twenty-eight percent of patients with GC had ultrasound reports that failed to show any evidence of acute inflammation. This was mainly due to the absence of sonographic Murphy's sign and gallbladder walls of less than 3 mm, both important radiological signs of acute inflammation of the gall bladder.

The CT findings most specific for acute GC are gas in the wall or lumen, intra-luminal membranes, an irregular wall and pericholecystic abscess. GC is associated with a lack of mural enhancement, pericholecystic fluid and a greater degree of gallbladder distension and wall thickening. GC has a mortality rate of up to 22 percent and a complication rate of 16 percent to 25 percent. Complications associated with GC include perforation, which has been reported to occur in as many as 10 percent of cases of acute cholecystitis. Perforation of the gall bladder can then lead to abscess formation or peritonitis. Hence, in contrast to acute cholecystitis, it is important both to diagnose and surgically treat GC prior to complication and/or perforation to avoid its high morbidity and mortality rate.

Conclusion: When dealing with patients with acute cholecystitis, a high index of suspicion is essential for the early diagnosis and treatment of GC. The possibility of a patient, especially an elderly patient with acute cholecystitis, progressing to GC should always considered, even in an apparently improving patient and in spite of the absence of any firm clinical or laboratory findings. The radiological investigations may not be conclusive. There is a need for an early (if not urgent) surgical intervention in acute cholecystitis (whether laparoscopic or open surgery) in order to decrease the time elapsed from the start of symptoms to admission and treatment.

http://www.jmedicalcasereports.com/content/5/1/199

http://www.ajronline.org/content/178/2/275

http://www.belsurg.org/uploaded_pdfs/106/106_545_549.pdf

http://radiology.rsna.org/content/148/1/219.full.pdf+html

Sushila Ladumor, MD, FRCR, consultant radiologist with multi-modality imaging experience, working in Medical Imaging Department, King Abdulaziz Medical City, Riyadh, Saudi Arabia. 

A 69-year-old male patient presented with a three-week history of right upper quadrant abdominal pain, pyrexia.

Gangrenous Cholecystitis

First case: A 98-year-old male with multiple co-morbidities presented with abdominal pain, fever and distension, r/o free air.

X-ray of the abdomen: Supine and sitting; Lamellated relatively large opacity in pelvis with dilated small and large bowel indicating obstruction, most likely from gallstone ileus with gallbladder stone in distal left colon.

Ultrasound: Gallbladder is not well distended and shows irregular outline. Multiple hypoechoiec lesions in liver, likely metastasis, for further work-up.

CT scan of abdomen and pelvis done without contrast for intestinal obstruction, as patient had high creatinine.

CT abdomen without contrast, shows collapse gallbladder with air in IHBR with stone in sigmoid. Thickening of sigmoid beyond stone, worrisome for malignancy. Liver lesions seen by ultrasound, cannot be evaluated without contrast. Bilateral pleural effusion, Rt>LT. Patient lost follow-up.

Second case: Elderly female patient with right upper quadrant pain.

First plain film:Radio density in right upper quadrant, most likely due to gallstone. No signs of bowel obstruction.

Second film after one year with acute presentation: Previously seen density in right upper quadrant is not seen and opacity noted pelvis with distal small bowel obstruction and air in IHBR indicating Rigler’s triad from gallbladder stone ileus.

CT of the abdomen and pelvis: Coronal reconstruction; Confirms the findings of gallstone ileus.

Third case: A 50-year-old female with acute abdominal pain, r/o obstruction.

Plain film: Multiple dilated small bowel air-fluid level, air in IHBR, fain opacity left side of L-L4 (shown by arrow), indicating gallbladder stone ileus (Rigler’s triad).

CT confirms the findings of plain films and it is consistent with gallstone ileus.

Coronal reconstruction of CT abdomen shows gallstone ileus and it is in proximal ileal loop.

Gallbladder stone ileus is an unusual complication of chronic cholecystitis, impaction of gallstone in ileum after passing through a biliary-enteric fistula. Sixty percent are cholecystoduodenal fistulae; others are cholecystocolonic and cholecystogastric fistulae. The average age of the patient is 70; there is a delay in diagnosis and a mortality of 15 percent to 20 percent.

Strong clinical suspicion, timely use of combined diagnostic imaging modalities (mainly, abdominal plain film and CT scan) and early surgical intervention are of paramount importance to achieve a favorable outcome. It accounts for 10 percent of obstruction in those over 65 and accounts for 25 percent of obstruction in the elderly. It is more common in females. It forms part of the Bouveret syndrome (when obstruction occurs in the duodenum).

Recurrence rate is 5 percent to 10 percent, and 40 percent present with pneumobilia. Stones over 2.5 cm will cause obstruction. Mortality rates are high and surgical intervention is required. Small bowel obstruction can lead to thiamine deficiency which may result in Wernicke's encephalopathy.

Rigler's triad, which should not be confused with Rigler’s sign, consists of three findings seen in gallstone ileus:

• gallstone, usually in the right iliac fossa

• The differential is essentially that of each component of Rigler's triad.

• Lower abdominal/pelvic calcification

• Small bowel obstruction (other causes)

http://www.patient.co.uk/doctor/Gallstone-Ileus.htm

http://www.uptodate.com/contents/gallstone-ileus

http://www.isrjem.org/april%202009.caglayan-gallstoneileus.pdf

Three cases: A 98-year-old male with multiple co-morbidities presented with abdominal pain, fever and distension; Elderly female patient with right upper quadrant pain; A 50-year-old female with acute abdominal pain, r/o obstruction.

Gallbladder Ileus

Clinical history: An 11-year-old boy with frequent partial seizures and lip twisting for four years. Initial CT of brain was done without contrast as patient presented to ER.

Low density lesion in medial aspect of right temporal lobe


There is hypodensity at the medial aspect of right temporal lobe. The patient needs further evaluation by MRI to rule out focal mass.

Heterogeneous enhancing cortial based right medial temporal lesion with low signal in  T1,high  signal inT2 & FLAIR  images with minimal perilesional edema.

An MRI of the brain was done with and without contrast. There is heterogeneously enhancing lesion at the most medial part of right temporal lobe. It measures 2.5 cm by 2.4 cm by 2 cm in maximum craniocaudal, transverse and AP dimensions. There is minimal white matter edema. It is causing minimal mass effect and prominence of right temporal pole over the suprasellar cistern. It has low T1, high T2, FLAIR signal intensity.

The differential would include dysembyoplastic neuroepithelial tumor, gangliocytoma / glioma.  Most likely ganglioglioma. The differential includes dysembyoplastic neuroepithelial tumor, but it is less likely.

Ganglioglioma is a tumor that arises from ganglion cells in the central nervous system.
 A ganglioglioma is an uncommon central nervous system tumor which accounts for around 2 percent (from 0.4 percent to  3.8 percent) of all primary intracranial tumors; and up to 10 percent of primary cerebral tumors in children. They are however the most frequent of the neuronal-glial central nervous system neoplasms.

They are of low grade (WHO Grade I or II). Typical occurrence is in the temporal lobes, although they have been described in all parts of the central nervous system.

Children and young patients are usually affected, and no gender predominance is recognized.

The most common presentation is with temporal lobe epilepsy, presumably due to the temporal lobes being a favored location.

Ganglioglioma was first described by Courville in 1930 as a central nervous system neoplasm containing both astrocytic and neuronal components. Gangliogliomas may occur anywhere in the central nervous system but are not encountered commonly.

Similar to other brain tumors, imaging techniques define the location of gangliogliomas and their relationship to adjacent structures. Because of the relative rarity and nonspecific appearance of these lesions, gangliogliomas are only infrequently considered a presurgical diagnosis.

The differential diagnosis is very broad and should be restricted by location.

If in the temporal lobe consider:
 •	pleomorphic xanthoastrocytoma (PXA)
 •	Ganglioglioma
 •	ganglioneuroma
 •	pilocytic astrocytoma
 •	dysembyoplastic neuroepithelial tumour (DNET)

Temporal lobe tumor differential diagnosis
 If in the spinal cord consider:
 •	astrocytoma
 •	ependymoma

For cortical tumors consider:
 •	oligodendroglioma
 •	pleomorphic xanthoastrocytoma (PXA)
 •	ganglioneuroma

http://emedicine.medscape.com/article/340632-overview#a01

http://radiopaedia.org/articles/ganglioglioma

http://neuro-oncology.oxfordjournals.org/content/9/2/124.full

http://radiographics.rsna.org/content/22/5/1177.full

Sushila Ladumor, MD, FRCR, consultant radiologist with multi-modality  imaging experience, working in Medical Imaging Department, King  Abdulaziz Medical City, Riyadh, Saudi Arabia. 

An 11-year-old boy with frequent partial seizures and lip twisting for four years. Initial CT of brain was done without contrast as patient presented to ER. The patient needs further evaluation by MRI to rule out focal mass.

Ganglioglioma

Clinical History: A 7-year-old girl with day and night urge incontinence. Voiding cystourethrogram performed.

Low bladder volume with dilated urethra; no  vesico-ureteric reflux; smooth out line of urinary bladder.

Intermittent emptying of urinary bladder, wide urethra

Low capacity of bladder and patient started voiding after 80ml of contrast injection in ub through catheter. Unusual dilatation of urethra, giving “spinning top” appearance. Urinary bladder shows smooth outline. No vesico-ureteric reflux. No significant residual urine. The findings are in keeping with spinning top urethra.

Functional disorders of the bladder and urethral musculature can manifest as a delay in maturation of bladder control, hyperreflexia of the detrusor muscle with normal external sphincter activity (Urge Syndrome) and lack of coordination of the detrusor and the external sphincter (Detrusor-External Sphincteric Dyssynergia). The causes of these disorders are not certain.

 The Urge Syndrome (Unstable Bladder) is a common disorder characterized by phasic uninhibited (involuntary) detrusor contractions early in the bladder filling phase and normal external sphincter activity. It is seen usually in children between 3 and 14 years (median 6 to 8 years) and is more common in girls than in boys.

The Detrusor-External Sphincteric Dyssynergia syndrome is less common and is characterized by an incoordination, or dyscoordination, in the activity of the detrusor muscle and that of the external sphincter. The two muscles tend to contract at the same time, preventing efficient emptying of the bladder and leading to a chronically increased intravesical pressure and obstructive changes in the bladder and upper urinary tracts similar to those seen in classical neurogenic bladder. This condition occurs with equal frequency in boys and girls and is commonly encountered between ages 2 and 13 years.

In the Urge Syndrome, clinical manifestations include frequent daytime voiding and urge to void that may result in episodes of incontinence. Nocturnal enuresis is common. The patients react to detrusor contractions by increasing the external sphincter and perineal muscular activity and assume characteristic postures or develop special maneuvers to prevent urine loss. Urinary tract infections are not uncommon. The increased pelvic floor activity contributes to the development of chronic constipation and soiling. The combination of urinary tract and bowel dysfunction may be referred to as the “dysfunctional elimination syndrome.” The patient can void on command, and the act of voiding is usually well coordinated. Bladder instability is a generally benign disorder that tends to resolve spontaneously with age.

With Detrusor-External Sphincteric Dyssynergia recurrent urinary infections, nighttime wetting, and daytime urgency and frequency are common manifestations. Episodes of urinary retention may occur, and an occasional patient presents with clinical signs of chronic uremia. Dribbling between voidings, possibly an overflow phenomenon, may be observed. Voluntary initiation of voiding may be difficult, requiring much straining. The distended and firm bladder is sometimes present on physical examination. Many children with this disorder also have bowel dysfunction manifested by chronic constipation and soiling. A wide range of behavioral or psychological disturbances is common and may contribute to the severity of the symptoms and affect the response to therapy.

Imaging:

 In the Urge Syndrome, increased thickness of the bladder wall is demonstrated with ultrasound . On voiding cystourethrogram, the bladder is commonly smaller than normal and hypertonic, and may be smooth in outline or slightly trabeculated. Vesicoureteral reflux may be observed.

The patient can void with a sustained flow without residual urine in the bladder at the end of voiding. Signs and symptoms of transient detrusor contractions may be noted during the examination, including urge to void at lower bladder volumes, intermittent slowing or cessation or even reversal of the flow of contrast material in the tubing, and intermittent widening of the bladder neck and proximal urethra during bladder filling, “spinning top” urethra in females. The upper urinary tracts are usually normal. 

With Detrusor-External Sphincteric Dyssynergia, ultrasound shows an increased thickness of the bladder wall and on VCUG the scout film may show a large amount of feces in the colon. The bladder is commonly enlarged, hypertonic, and variably trabeculated, and may point upward and to the right as in classic neurogenic bladder.

Unilateral or bilateral reflux is also common, occurring in about 50 percent of the patients, and the refluxing upper tracts are frequently dilated. Unilateral or bilateral upper tract dilatation is sometimes severe. The bladder neck and urethra appear normal, but the area of the external sphincter may show a constant or intermittent narrowing (spasm) of the urethra at that level with dilatation proximally.

In girls, the urethra may also assume a “spinning top” configuration. A large post-void residual is very common. Atrophy of the renal parenchyma may be present, and renal function may be decreased. The uncoordinated activity of the detrusor and external sphincter muscles tends to improve in late childhood or early adolescence, but by this time secondary changes in the upper tracts may have progressed to renal insufficiency.

 Differential Diagnosis:

1. Neurogenic bladder 
 2. Posterior urethral valves or diaphragm 
 3. Cobb’s collar  
 4. Sphincter spasm

Treatment: 

 Current treatment consists of biofeedback training and an intensive and prolonged bladder retraining program, including frequent and “relaxed” voiding with complete emptying of the bladder and other forms of behavior modification.

Bates DG., “The Bladder and Urethra”. Caffey’s Pediatric Diagnostic Imaging. Eleventh Edition.  

A 7-year-old girl with day and night urge incontinence. Voiding cystourethrogram performed. Low capacity of bladder and patient started voiding after 80ml of contrast injection in urinary bladder through catheter. Unusual dilatation of urethra, giving “spinning top” appearance.