1.5 MRI System 3D-C.T Scanner 3D/4D-Anomoly Scan Colour Doppler Comp Radiography Mammography X-Ray and I.I

PFT EEG Portable Ultrasonography Portable Colour Doppler Portable X-Ray Unit Portable Echocardiography Cytogenetics

Diagnostic Cardiac Intervention Therapeutic Cardiac Intervention

CASE OF THE MONTH

jAUGUST 2010

CASE 1 : HODGKIN’S LYMPHOMA – GASTRO INTESTINAL TRACT

18 year old male presented with mass in abdomen. Right hemicolectomy was done which revealed a large fleshy ulceroinfiltrative tumour involving caecum and appendix. Multiple fleshy mesenteric lymphnodes were identified at the level of the tumour.

Histopatholgy of the specimen revealed (Fig 1 A & B) polymorphic population composed of lymphocytes, eosinophils, plasma cells, Hodgkin’s cells and binucleate Reed - Stenberg (RS) cells.

Figure 1 A

Figure 1 B

Photomicrograph (40x) showing polymorphic population composed of lymphocytes, eosinophils, plasma cells, Hodgkin’s cells and binucleate Reed - Stenberg (RS) cells.

Discussion :

Hodgkin’s Lymphoma is very rare in the gastrointestinal tract. In our case, presence of classical Reed - Stenberg (RS) cells was the diagnostic clue. Immunohistochemistry was advised.

CASE OF THE MONTH

jJAN.09

FOIX-CHAVANY-MARIE SYNDROME (FCMS) / OPERCULUM SYNDROME

INTRODUCTION: Foix-Chavany-Marie syndrome (FCMS) also known as the anterior operculum syndrome, is considered the cortical type of pseudobulbar palsy, which is characterized by severe dysarthria and bilateral paralysis of the facial, lingual, pharyngeal, and masticatory muscles with preserved automatic, involuntary and emotional innervation.

Weller (1993) classified FCMS into five clinical types:

1. The classical and most common form associated with cerebrovascular diseases;

2. A subacute form caused by central nervous system infections;

3. A developmental form, probably most often related to neuronal migration disorders;

4. A reversible form in children with epilepsy; and

5. A rare type associated with neurodegenerative disorders.

CASE REPORT:

A 14 year old right handed male was admitted with difficulty in speaking. On neurological examination, showed signs of paresis in all extremities. Patient had dysphagia. MRI Brain revealed well defined almost symmetrical areas of altered signal intensity in bilateral posterior fronto-temporal regions(left slightly larger than right) with extension into the high parietal region on the left side with loss of grey as well as white matter, resultant thinning of gyri, widening of sulci and paucity of the subcortical white matter as well as mild ex-vacuo dilatation of the posterior body and trigone of both the lateral ventricles with uneven ventricular margin in this region – areas of gliosis/ encephalomalacia, as a sequel of previous vascular insult.


T1 AXIAL	T2 AXIAL	T2 CORONAL	FLAIR CORONAL


T1 AXIAL	T2 AXIAL	FLAIR


T2 AXIAL	FLAIR	FLAIR


ADC	DIFFUSION

DISCUSSION

In FCMS patients with cerebrovascular diseases, staged strokes are reported in most of these patients.

Clinical characteristics of FCMS could be summarized as follows:

Speech disturbance is severe and most of the patients are speechless, particularly in the acute stage. Some patients can produce inarticulate sounds when they attempt to speak. On the other hand, language disturbances are either not detected, or if present, are mild. Most of the patients can understand a conversation or a command and can communicate through writing or gestures, if not accompanied by paresis of the hand; this indicates the absence of aphasia. Profound dysphagia is observed, and it necessitates tube feeding in many patients, at least in the acute stage. Severe disturbance at the oral stage of swallowing is observed in most patients, but the swallowing reflex is adequate in some patients if the bolus is delivered into the pharynx. Facial appearance is atonic and the mouth is half open. The opening and closing of the mouth might be impossible or partly possible. The upper side of the face may or may not be impaired. The tongue is almost immobile and does not show any muscular atrophy and fibrillation. Automatic-voluntary dissociation is dramatically presented when patients smile, yawn, or cry under natural circumstances.

Distinction from Noncortical Type of Pseudobulbar Palsy:

The “dramatic” dissociation of automatic and voluntary movements of the bulbar muscles and atonic appearance of the face are the most distinctive features of FCMS, which distinguish it form the noncortical type of pseudobulbar palsy.

CASE OF THE MONTH ( JULY 2007)

A 18 year old female presented with pain in abdomen in left hypochondriac

and epigastric region, jaundice, abdominal distension, oliguria and bilateral pedal oedema

since one week.

Hematological investigations revealed raised bilirubin (total 2.16 mg% conjugated 1.19 mg%)

and anemia (HB 8.8 gm%). HBsAg , HCV, HIV were negative.

Ultrasound abdomen showed hepatosplenomegaly, ascites and minimal right pleural effusion.

Patient was advised CT scan of the abdomen to rule out hepatic venous outflow tract obstruction.

CT scan of the abdomen (plain and post contrast triphasic scanning) was performed using

multidetector ultrafast 64 slice CT scanner. Reconstructions were performed in sagittal and

coronal planes.

There was poor visualization of the hepatic veins which showed hypodensity within them

even on delayed scans suggestive of thrombus within them. There was significant luminal

narrowing of 60-70% of the retrohepatic IVC without any thrombus within it. The liver

showed heterogenous enhancement with only the caudate lobe showing normal

size and enhancement. Few relatively poorly enhancing foci were noted especially in the

posterior subcapsular region of segment II and III; possibly hepatic infarcts. There was

gross ascites with bilateral moderate pleural effusion.

These imaging findings were suggestive of Hepatic veno-occlusive disease (acute

Budd Chiari syndrome)

Fig 1 Thrombosed hepatic veins with

sparing of the caudate lobe

Fig 2 Enlarged heterogenously enhancing liver with compressed IVC


Fig 3 Sagittal reconstruction showinghetrogenously enhancing liver and compressed retrohepatic IVC	Fig 4 Coronal reconstruction clearly showing thrombosed hepatic veins in the left lobe

Patient was shifted to higher institution for further management. Subsequently patient

developed portal vein thrombosis and surgery was deferred. At present patient is on

anticoagulants and requires therapeutic ascitic tap once a week.

DISCUSSION

The Budd-Chiari syndrome is a rare disorder of hepatic-vein or inferior-venacaval

occlusion. In some instances the obstruction occurs at the venule level.

In most instances obstruction to the hepatic veins and/or cava is partial. Because

of this hepatic venous outflow obstruction, patients with this syndrome may present with

variable degrees of hepatic enlargement, pain, tenderness, and ascites.

Jaundice, portal hypertension, and variceal bleeding may also be present. Much

less common is the acute fulminant presentation with massive liver failure and

shock. In comparison with the frequency in the United States, the frequency of

this syndrome is higher in northern India, South Africa, and the Orient. Budd-

Chiari may be associated with polycythemia rubra vera, chronic leukemia, oral

contraceptives, neoplasms, pregnancy, trauma, and congenital abnormalities. However, the

exact etiology cannot be determined in almost two-thirds of cases.

The Budd-Chiari syndrome may be classified as primary or secondary depending

on its pathophysiology. The primary type refers to congenital obstruction of the

hepatic veins or the hepatic portion of the inferior vena cava by webs or diaphragms.

The secondary type refers to hepatic-vein or inferior-vena-cava obstruction caused by

tumor, thrombosis, or trauma. Thus, the radiographic manifestations of the syndrome may

differ significantly.

DIAGNOSTIC PROCEDURES

1} ULTRASONOGRAPHY WITH DOPPLER — For screening patients with suspected

Budd Chiari syndrome.

2} CT SCAN – PLAIN AND CONTRAST ENHANCED- for confirming the diagnosis of

hepatic veno-occlusive disease and exact delineation of the extent and level of hepatic

veins and IVC obstruction.

3} ANGIOGRAPHY –1) Inferior vena cavography for confirmation of any thrombus

within it and severity of compression with pressure gradient measurements across the

stenotic segment. 2) Hepatic wedge cavography which typically show the ‘spider web’

pattern of collateral intrahepatic veins, 3) celiac / SMA arteriography shows diffuse narrowing ,

pruning and stretching of the hepatic arteries.

6} PERCUTANEOUS BIOPSY – using large bore 14 – 16 gauge trucut needles.

Demonstration of organic thrombi within the main intrahepatic veins is diagnostic.

Cirrhosis may be seen in chronic cases.

TREATMENT

1} MEDICAL Rx – Diuretics and Anticoagulants

2} SURGICAL – 1) Percutaneous transluminal angioplasty

2) Portocaval or mesocaval shunts

3) Ballooon angioplasty