DX: Pontine cavernoma (deep cavernoma)

This was an easy one..findings are consistent with variable T2-low signal intensities in the pons region clearly suggesting a site of previous bleeding. T1 didn't show hyperintensities so acute hemorraghe is dismissed. It has diffuse surrounding edema that could explain unusual symptoms.

Here are the spot images:

Radiological findings and location makes differentials unnecesary. This is a tipical infratentorial cavernoma knows as deep cavernoma of the pontine region. Deep cavernomas are considered to be those occurring in the brainstem (midbrain, pons or medulla), cerebellar nuclei, thalamus and basal ganglia.

A cavernoma or cavernous malformation is a vascular abnormality of the central nervous system. It consists of a cluster of abnormal, dilated vessels. Pathologically, it is red to purple in colour, appearing as a raspberry. Cavernomas contain blood products at various stages of evolution and are usually less than 3 centimetres in size.

Brainstem cavernomas (or cavernous angiomas)  have recently received a great deal of attention due to enhanced imaging techniques and the realization that even small hemorrhagic events can cause significant neurological deficits. While the majority (approx. 75%) of cavernous angiomas occur in the upper (“supratentorial”) region of the brain, about 1 in 5 are located in the brainstem or in highly sensitive (“eloquent”), lower (“infratentorial “) areas of the brain. The most common symptom for brainstem lesions is focal neurological deficit as opposed to seizure or headache for lesions located in surpratentorial regions.

The nerves that transverse the brainstem control basic, involuntary functions such as respiration, gag reflex, heartbeat regulation, body temperature, pain and heat sensation, and hiccupping as well as other voluntary functions including eye movement, swallowing, facial muscle control, walking, and speech. Both cranial and “long tract” (whole body) nerves can be affected. For the individual, a brainstem cavernous angioma can manifest a disparate range of symptoms making diagnosis difficult.

In a large study reported in the Journal of Neurosurgery in 1997 (Porter et al vol 87, page 190) 37% of patients fully recovered all function following a stroke from a deep cavernoma, 36% had partial recovery and 27% had no recovery of function. This is similar to the experience at the Sydney Aneurysm and AVM Neurosurgical Centre. Whilst it is usual for superficial cavernoma bleeds to fully recover this is not the experience with the deeper cavernomas.

From the time a cavernoma is diagnosed the chances of being unaffected by bleeding over the next 5 years is 57%, over the next 10 years is 33%, over the next 20 years is 11% and over the next 25 years is 6% (Porter et al, J Neurosurg 87:190, 1997). Although some studies have suggested that the risk of bleeding is not influenced by mode of presentation it is likely that if a large number of deep cavernomas could be followed that those with a history of having bled in the past are more likely to bleed in the future.

http://www.angiomaalliance.org/pages.aspx?content=72&id=63

Thank you for your participations...till next time!

CASE 14: Mid-age male patient with neurological problems

This next case had an interesting clinical setting. Patient complains of having headaches with the prodrome quoted as the following: "The sensation of having a man standing straight in my head"...yep, you read correctly...pretty weird huh?

Anyways here are the relevant images..click to enlarge:

It was isointense on T1 and there are no post-gadolinium images..differentials please..

DX: Right lung centrilobular emphysema and left lung cystic lung disease

Patiend had no history of smoking or occupational exposure, but he was from rural origin so chronic smoke exposure because the use of firewood has to be considered.

The particularity of this case was that each lung had a different imaging spectrum:

Right lung shows an hyperlucent lung due to chronic obstructive pulmonary disease -COPD-  and centrilobular emphysema. Left uppel lobe lung shows a classical "honeycomb" pattern with variable size cysts and thickened septa.

Could this be a part of one single entity?

When lungs are affected with COPD, usually emphysema occurs. In the other hand, honeycombing appeareance is always and end-stage pulmonary disease and is formed by fibrosis and loss of pulmonary volume with trapped air cysts.

Differentials of focal cystic lung disease include:

• Pulmonary Langerhan´s cell histiocytosis
• Lymphangiomatosis
• Sarcoidosis
• Lymphocytic intesticial pneumonitis (LIP)
• Pulmonary mesenchymal cystic hamartoma (rare)
• Asbestosis
• Idiopatic pulmonary fibrosis
• Cystic fibrosis

Usually these pathologies are supleural in location, with multiple variable in size cysts but with small thickened septa. Other differential is also a bullous emphysema but septa is not thick as well.

Also, most of all pathologies tend to present as a bilateral setting, making the diagnosis of this case more difficult. 

This patient didn't underwent biopsy, he refused so was treated as Focal idiopatic pulmonary fibrosis and was recommended to discard Cystic fibrosis.

Hope this review was helpful. Even though case is incomplete, we can approach to diagnosis through these differentials.

http://www.radiologyassistant.nl/en/42d94cd0c326b#a458121a7d9f0d 

http://www.ajronline.org/content/180/3/827.long 

http://radiopaedia.org/articles/cystic-lung-disease 

CASE 13: Male adult patient with chronic cough.

Patient with non-productive cough associated with mild progressive dyspnea. To our concern there was no more relevant history at time of CXR (chest X ray).

Click to enlarge:

With above findings, we recommended a chest CT study:

Give differentials..soon I´ll post the conclusion as always..

DX: Infected permeated urachal cyst of adult

This was an unusual case. History made it more difficult to me, but findings are very clear.

Here are the spot images:

Urachal abnormalities result from incomplete regression of the foetal urachus. They are more common in children than in adults, due to urachal obliteration in early infancy.


In adults, urachal cyst is the commonest variety, with infection being the usual mode of presentation.

Diagnosis remains challenging due to the rarity of this lesion and the non-specific nature of its symptomatology. Since the first description of urachal abnormality by Cabriolus in 1550, few cases have been reported in literature.

There are five types of urachal abnormalities: 1) patent urachus, in which the entire tubular structure fails to close; 2) urachal cyst, in which both ends of the canal close leaving an open central portion; 3) urachal sinus, which drains proximally into the umbilicus; 4) vesicourachal diverticulum, where the distal communication to the bladder persists; and 5) alternating sinus, which can drain to either bladder or umbilicus.

The incidence of UC in adults is unknown but it is rare. It is more common in men than women. Modes of presentation of urachal anomalies in adults differ from those seen in children. In adults, the commonest variety is urachal cyst, with infection being usual mode of presentation. The route of infection is haematogenous, lymphatic, direct or ascending from the bladder. The commonly cultured microorganisms from the cystic fluid include Escherichia coli, Enterococcus faecium, Klebsiella pneumonia, Proteus, Streptococcus viridans and Fusobacterium. 

In this case, culture is now beeing made.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2740315/ 

http://www.jurology.com/article/S0022-5347(07)00892-0/abstract

http://www.urologyweb.com/urachal-anomalies-and-related-umbilical-disorders.html

CASE 12: Male adult patient with pelvic palpable mass. History described below

This next case was presented to me with a history of recurrent urinary infections and a palpable mass in pelvic area. According to age (mid 50´s), an enlarged prostate was suspected.

Here is the Ultrasound:

 

Patient was dissmissed with antibiotic theraphy an CT follow up according to my recommendation, only that CT was conducted yesterday. The US was made 4 months ago. Here are the relevant CT slinces:

Medical record indicates that patient had Penile cancer and a penectomy was done with a perineal urethrostomy a few months ago.



Findings and differentials please.

DX: Gastric Mucosa-Associated Lymphoid Tissue (MALToma)

This case was particular because of it's presentation. One could expect from seeing images that arises from hepatic or gastric tissue as it seems that there is no boundary between them. Here is the spot image:

History of food intolerance, thickened gastric wall that seems uncollapsed, are clue to diagnosis. If an hepatic mass extends and infiltrates to stomach ussualy it obliterates its lumen.

Endoscopy biopsy proved to be a lymphoma of gastric origin, a MALToma.

MALTomas are extranodal manifestations of marginal-zone lymphomas. Most MALTomas are a low grade, although a minority either manifest initially as intermediate-grade non-Hodgkin lymphoma (NHL) or evolve from the low-grade form. 

Malignancies that occur in mucosa-associated lymphoid tissue (MALT) are called MALT lymphomas or MALTomas. Most of the MALTomas occur in the stomach, and roughly 70% of gastric MALTomas are associated with H. pylori infection.

Thank you for your FB discussions. You can comment here also.

CASE 11: Female patient with abdominal pain and abnormal US finding

This was an outpatient with increasingly high abdominal pain and food intolerance. US findings suggested an hepatic mass, so therefore CT was recommended.

Differentials?..later on I'll post the conclusion..

DX: Mycobacterial Ependymitis + Hydrocephalus / ADEM

Well this case got me really interested. First, because of the young age and acute onset of symptoms and rapid neurological changes (both clinical and imaging) that was missed or unclear on the first evaluation with the CT scan.

CT scan was normal, but as a retrospective approach you can see some irregularity on lateral ventricle walls ( c'mon with some imagination right?  hehe). Rest was unremarkable. Then patient suffered altered mental status with history of fever, so an inflamatory setting was considered. Spinal tap was made previous MR scan.

Click to enlarge:

There was periventricular white matter hyperintensities with dilated ventricular system; also noted in left cerebellar culmen and as a surrounding lineal lesion adjacent to right frontal horn of lateral ventricle. All findings are inconclusive, but the most intriguing finding was on sagital T2 images:

 Multiple focal hyperintensities of corpus callosum that can be seen in hydrocephalus as transependymal CSF mygration but those peculiar characteristics are frequently seen in Multiple Sclerosis (Dawson's fingers) and Acute Disemminated Encephalomyelitis (ADEM).

Spinal tab revealed Mycobacterium Tubelculosis. (yup)

So..let's summarize. Maybe the focal hyperintensities on cerebellum and lateral to frontal horn are tuberculomas that can provoque local irritation to ependymae (most likely on cerebellum) and hydrocephalus can be produced.

Now just a quick review on differentials:

MS in native Guatemalan's such as in this case is almost non-existent, in fact all of Leukodystrophies.  If consider, one has to include also CADASIL (cerebral autosominal dominant arteriopathy with subcortical infarcts and lekodystrophy) wich is the most common form of hereditary stroke disorder. Findings on MRI are similar but usually occur between 40 and 50 years of age, although MRI is able to detect signs of the disease years prior to clinical manifestation of disease. So is less likely but probable.

ADEM is considered to be the borderline of MS and is a good differential because can coexist with previous viral or bacterial infection. It produces multiple inflammatory lesions in the brain and spinal cord, particularly in white matter areas. Usually these are found in the subcortical and central white matter and cortical gray-white junction of both cerebral hemispheres, cerebellum and brain stem but periventricular white matter and gray matter of the cortex, thalami and basal ganglia may also be involved. Image features are variable but are present in younger populations and is a autoimmune complication of previous infection.

Thank you for your participation on this case...feel free to comment!

CASE 10: Male teenager with loss of consciousness (follow-up included)

Ok this next case is an interesting one beacuse it has the follow up.

Patient came two months ago with history of headache and underwent a CT scan..

Now, he was admitted to ED with history of loss of consciousness, seizures and fever. Now we did an MR study, here are the images with the relevant findings..

Describe findings..submit differentials. Soon I'll post the conclusion..