Rare Brain Tumour:Seventh Case in the World Literature

A 59-year-old gentleman, residing in Chitpur-Colootola area of central Kolkata, has been complaining of headache for a year. The headache used to subside with usual analgesics. Later on the intensity

increased. One day he fainted and was taken to a hospital. He also had paralysis on his left side.

CT scan and MRI scan was done. It showed a huge tumor on the right side of the brain

INSIDE

A Rare Case Of Congenital Heart Defect

‘An Unusual Case Of Memory Decline’

Laparoscopic assisted excision of Colo Vesical Fistula in a patient of Crohn’s disease

Rare Brain Tumour:Seventh Case in the World Literature

Custom Fit Joint Replacement: Another Step Forward in Joint Replacement Technology

Dear Doctor, Greetings from Fortis Hospitals, Anandapur.Your initial support and feedback has encouraged us to launch our quarterly magazine called ‘The Specialist’ for our clinicians of Kolkata and surrounding areas. ‘The Specialist’ is a magazine to give you an update on the clinical expertise and various new clinical initiatives started at our hospital in your state. This magazine is published at each of our major locations. Most doctors look forward to receive their copy of the specialist. Since our inception last year, your support has helped us to develop good programs in the area of Bone & Joint care, Cardiac care, Critical care, Digestive care, Pulmonology and Neurosurgery. Recently we have added to our team Dr. Ujjwal Debnath who will spearhead our spine surgery program and Dr. Shuvro Roy Choudhury who will spearhead our interventional radiology program. Over 50 RMOs were trained for Advanced Life Support through our Emergency care program. Dr. Raja Dhar our Interventional Pulmonologist has been doing very innovative interventional pulmonology work. Our full time interventional cardiology team led by Dr. Sunip Bannerjee has built up a strong department to handle complex elective and emergency cardiac procedures.Our center for Bone and Joint surgery did Eastern India’s first Custom fit knee surgery. In this edition of “The Specialist” we would like to showcase some of the interesting work which has been done in the areas of Cardiac surgery, Neurology, Minimally invasive brain surgery, Custom-fit knee surgery and advanced laparoscopic GI surgery. We will value your feedback and suggestions as to how to make the future issues of “The Specialist” more interesting and relevant to your needs.For more information on cases presented in the issue, please feel free to email us at debolina.bhattacharya@fortishospitals.in

Best wishesDr. Lloyd Nazareth

President & COO – Fortis Hospitals

Vol 1, July 2011

Fortis Hospitals, Anandapur, Kolkata

causing enormous pressure in the head. The tumor initially looked straightforward. However, during operation, it was found that the whole story was different. The tumor was not an innocuous one. It was one of the rarest tumors.

The patient was operated in December. A horse-shoe shaped incision was made in the right temporo-parieto-occipital region. Skin, galea and temporalis muscle were reflected in one layer. A temporo-prieto-occipital craniotomy was made. Dural tack-up sutures were placed. Dura was tense. Dura was opened in an inverted manner with its base towards the middle fossa floor. The brain was bulging. A large tumor was noted arising from the middle fossa base and was pushing the brain superiorly. The tumor also pushed midbrain medially. The tumor was very firm and was extremely vascular. Initially the arachnoid was dissected to free the tumor from surrounding brain. Later on, alternate internal decompression and dissection from the surrounding parenchyma was done. Gradually the tumor was dissected from trochlear nerve, midbrain and vein of Labbé. The base of the tumor

The headache used to subside with usual

analgesics. Later on the intensity

increased. One day he fainted and was taken

to a hospital. He also had paralysis

on his left side.

was attacked. Intermittently the tumor was gradually detached from the base. The tumor was noted to have origin from the posterior aspect of the middle fossa floor near the petromastod junction. The tumor was completely removed. The base was coaugulated. There was significant blood loss during operation. The operation went on for seven hours. We had to work in between critical structures of important arteries, veins and nerves and important parts of brain like midbrain, trochlear nerve, posterior cerebral arteries. Hemostasis was achieved. Dura was closed. Bone flap was replaced back. Wound was closed in layers. The patient was electively ventilated overnight and was extubated next morning. Postoperative MRI scan did not show any residual tumor. The patient recovered well. His paralysis improved to almost normal and he started walking. He was discharged on the fifth postoperative day.

Pre-operative scan

Investigation

Histopathological examination revealed cellular areas, composed of spindle cells, arranged in fasicular, storiform palisading fashion and at some places in an organoid arrangement. Cystic spaces were seen separating the tumor cells. There was prominence of blood vessels, which are predominantly thin walled but hyalinization of the vessel wall is also noted. Collection of foamy macrophages is present. Immunohistochemistry was negative for EMA, positive for vimentin and negative for S-100 protein.

This was an extremely rare tumor. It was a schwannoma arising from tentorium cerebelli. We have gone through the world literature. Only six such tumors have been reported in world literature till date. Moreover, the tumor was strangled in a number of critical structures. The postoperative scan looked clean. More than six months passed after operation and the patient is doing really well. He has gone back to his daily activities.

Courtesy

Dr. Amitabha Chanda

Consultant NeurosurgeonMS (General Surgery),M Ch (Neuro-Surgery),

Cerebrovascular & Skull Base Fellow

(USA), Skull Base &

Microneurosurgery Fellow (Canada),

Trained in Endoscopic Neurosurgery (Germany),

Fellow, AO Spine

Postoperative MRI scan did not show

any residual tumor. The patient recovered

well. His paralysis improved to almost

normal and he started walking. He was

discharged on the fifth postoperative day.

This was an extremely rare tumor. It was a schwannoma arising

from tentorium cerebelli.

Post-operative scan

A complex repair was done using patient’s

own tissue to re – direct the flow

The same patch of tissue was used

to close the defect between the chambers.

CourtesyDr. Ashok Bandyopadhyay

Consultant Cardiac SurgeonMS (General Surgery),

M Ch (Cardiothoracic Surgery)

EchoConfirmed the diagnosis of congenital heart defect and a large hole between the upper chambers of the heart with two of the four veins coming from the lungs, opening abnormally in the right side of the heart instead of the left. A leaking valve (Rt side of the heart) was also detected.

Blood InvestigationA rare hepatic (liver) disorder was also detected.

Surgery

This patient underwent surgery with the help of intraoperative TEE (Tran Esophageal Echo cardiogram). A complex repair was done using patient’s own tissue to re – direct flow from the abnormally opening right sided pulmonary veins to the normal left upper chamber. The same patch of tissue was used to close the defect between the chambers. At the same time the leaking valve was repaired

Pre-operative

Post-operative

- doing away with need for an artificial valve.

The entire procedure was done with the heart stopped and with the help of Heart – Lung Machine. At the end of the procedure the patient’s heart could gradually be weaned from the machine. A TEE (Tran Esophageal Echocardiogram) was done again to confirm the correctness of the repair – which was found to be perfect. The patient was keeping on artificial ventilation electively for 4 hrs and went on to make a smooth recovery. The patient was up & about by the 2nd post op day.

The patientAn 83 year old, active gentleman presented to us with increasing pain in one knee for the last couple of years. He had been seen earlier elsewhere where he had undergone

conservative treatment for osteoarthritis of his knee. He was extremely keen to have some treatment that would restore his mobility and give him relief from pain.

Custom Fit Joint Replacement: Another Step Forward In Joint Replacement Technology

Clinical examination and X-rays revealed moderately advanced arthritic changes in all compartments of the knee. After discussing with the patient we decided to proceed with

A Rare Case Of Congenital Heart Defect

PresentationGradually worsening breathlessness for more than 1year and chest pain of same duration.

InvestigationThinly built 23 year old male, unmarried with grossly abnormal heart sounds & hole in the heart suspected, which is confirmed with the following investigation:

Chest X-rayShowed a grossly enlarged heart & chambers.

A large hole between the upper chambers of the heart with two of the four veins coming

from the lungs, opening abnormally

in the right side of the heart instead of

the left.

Patient Specific Tibial jigs (Top View)

Accurate alignment has been shown to improve

the longevity of our implants. Furthermore, as the jigs are smaller and do not require an intra-medullary rod for alignment, our incisions

can be smaller and blood loss is minimized.

Consequently, recovery is faster.

CourtesyDr. Ronen Roy

Consultant Orthopaedic SurgeonMBBS FRCS (Glasgow)

a custom fit knee.

Mr KP had his CT scan and underwent his surgery as planned and his post operative recovery was uneventful. He came for follow up last week (six weeks post–op). He is extremely happy and is planning to visit his son next month.

Background

Fortis Hospitals has been the pioneer in this field (the first custom fit replacement in the East Zone was carried out in our Hospital in Kolkata and the first one in India was carried out in our sister hospital in Bangalore).

A custom fit knee is absolute cutting edge technology as far as joint replacement surgery is concerned. It is deemed to be an advancement over existing computer navigated surgery. It involves the manufacture of patient specific jigs based on the patient’s own measurements (by CT scan or MRI scan) which are e-mailed directly to the manufacturers. A preliminary design template is then sent back to the operating surgeon for review and adjustments if required. Once the go ahead is given, cutting jigs are manufactured accordingly and shipped to us for use during surgery.

Discussion

Patient specific jigs are a significant advancement in the joint replacement surgeon’s armamentarium. The cuts are exact

Patient specific jig

Patient specific tibial jigs in place

(Front View)

Copy of plan sent to surgeon before surgery

Femoral jig in place(Side View)

and help us in aligning the knee perfectly. Accurate alignment has been shown to improve the longevity of our implants. Furthermore, as the jigs are smaller and do not require an intra-medullary rod for alignment, our incisions can be smaller and blood loss is minimized. Consequently, recovery is faster. Another advantage is that as all planning is done well in advance of surgery, operating time is less – this again reduces blood loss, OT time and risk of infection.

Custom fit replacements can only be performed at present in less deformed knees. Also a time of about five to six weeks is required between the submission of the scans and the arrival of the jigs.

We believe that in appropriate patients, we will be able to offer the best quality of service with this new technology.

Clinical examination and X-rays revealed

moderately advanced arthritic changes

in all compartments of the knee.

After discussing with the patient

we decided to proceed with a custom fit knee.

The first custom fit replacement in the

East Zone was carried out in our Hospital in Kolkata and the first

one in India was carried out in our sister hospital

in Bangalore.

Laparoscopic assisted excision of Colo Vesical Fistula in a patient of Crohn’s disease

Discussion

Crohn’s disease is a non specific chronic inflammation of the bowel and may affect the gastrointestinal tract from the lips to the anal margin but ileocolonic disease is the most common presentation. It is most common in Northern America and Northern Europe. The cause of the disease is unknown. There are deep mucosal ulcerations with linear or snake like patterns and under the microscope there are focal areas of chronic inflammation involving all the layer of the intestinal wall. Non caseating granulomas are found in 60 percent of cases. The patient may present in acute form in 5 percent of cases giving symptoms and signs similar to appendicitis. Ninety five percent present in chronic form giving a history of mild diarrhea, abdominal pain, intermittent fevers, weight loss, and secondary anemia. Some may present with perianal abscess or fissure. With progression of disease they may develop intraabdominal abscess and fistulous tracts such as enteroenteric, enterovesical and enterocutaneous fistula.

Crohn’s disease is non specific chronic inflammation of the

bowel and may affect the gastrointestinal tract from the lips to the anal margin but ileocolonic

disease is the most common presentation.

Fistulous Tract

The treatment is with steroids and 5 aminosalicylic acid compounds. Surgical intervention is required for complications like intestinal obstruction, bleeding, perforation, intestinal fistula, malignant change, fulminant colitis and perianal disease.

Case Report

A 38 year old man, presented with diarrhea, mucus discharge 8-10 times daily, and anorexia since child hood. He was having pain in lower abdomen, burning micturation, low grade fever, passing stool & air in urine (Fecaluria and pneumaturia) and

subsequently urine in stool for past 5-6 months.

He was diagnosed to have Crohn’s disease at CMC, Vellore in Sept.2008, and treated conservatively with mesacol, ciprofloxacin & metronidazole. He could not continue treatment because of his poor financial state and attempted alternative methods of therapy such as Homeopathy, Ayurveda etc.

CECT showing air in urinary bladder

He was only 27 kgs prior to his surgery and

in very poor health.

The patient made uneventful recovery.

He regained his appetite and was passing urine

normally He was ambulant 24 hours after surgery and on oral feeds after

48 hours.

Laparoscopic assisted excision of the fistula

reduced his pain, hospital stay and hastened

his recovery.

CourtesyDr. Ramesh Agarwalla

ConsultantGeneral and Advanced Laparoscopic SurgeonMS, FRCS, FIAGES, FAIS

The patient was very ill, had urinary infection, fever, was unable to eat and had severe pain in the abdomen when he came to us. He also had perianal fistula. He was admitted, and treated medically with help of our Medical Gastroenterologist Dr Debasis Datta to optimize his condition before surgery. He was only 27 kgs prior to his surgery and in very poor health. He would not be able to withstand open surgical removal of fistulous tract. Laparoscopic assisted excision of the fistulous tract was done and the opening in the urinary bladder and the intestine repaired. A proximal loop ileostomy was done (which was closed after 3 months). The perianal fistula was also excised.

The patient made uneventful recovery. He regained his appetite and was passing urine normally. He was ambulant 24 hours after surgery and on oral feeds after 48 hours. He was discharged after 7 days and was readmitted for closure of colostomy .

He has recovered well, regained his appetite, gained weight (41 kgs now, was 27 kgs). He is passing urine and stool normally.

This is not a very common condition and is usually treated by conventional surgery. Laparoscopic assisted excision of the fistula reduced his pain, hospital stay and hastened his recovery. There are no reports of laparoscopic assisted excision of colovesical fistula in Crohn’s disease in literature.

AIR

‘An Unusual Case Of Memory Decline’

Introduction

New variant Creutzfeldt-Jakob disease (nvCJD) is an acquired prion disease causally related to bovine spongiform encephalopathy that has occurred predominantly in young adults. All clinical cases studied have been methionine homozygotes at codon 129 of the prion protein gene (PRNP) with distinctive neuropathological findings and molecular strain type (PrP(Sc) type 4). Modeling studies in transgenic mice suggest that other PRNP genotypes will also be susceptible to infection with bovine spongiform encephalopathy prions but may develop distinctive phenotypes.

New variant Creutzfeldt–Jakob disease (vCJD) was first identified in the UK in 1996, and was causally linked to bovine spongiform encephalopathy. Initially misnamed as ‘mad-cow disease’ it went as a taboo with beef eaters. However it has been identified

Initially misnamed as ‘mad-cow disease’

it went as a taboo with beef eaters.

as a prion disease with dissimilarities with classical CJD both clinically and radiologically throughout the world. Herein we report a case of possible nvCJD from India.

Case report

A 64-year-old man,Islam by faith,who had lived throughout his life at Calcutta,India. presented with subacute onset and progressive depression, irritability, personality change. He was first assessed by psychiatrist and antidepressants failed to arrest the course of illness. The clinical features extended to cause gait ataxia and cognitive impairment which made him seek neurological consultation.His initial MRI of the brain revealed minimal signal alterations around the thalami and he had a normal EEG.Routine blood reports were normal except a marginally raised liver enzymes.

However he continued to deteriorate and by 3 months he was needing support for his Instrumental activities of daily living.

She has by then developed myoclonic jerks in generalized fashion.This time EEG did show the typical appearance of of periodic complexes with a much slower background. His CSF study showed raised protein and normal sugar with mild Lymphocytic pleocytosis. However PCR for Tb was negative and fungal smear was also negative. Brain magnetic resonance imaging this time revealed high signal lesions involving bilateral caudate nuclei, left lentiform nucleus, bilateral dorsomedial thalami and pulvinar on fluid-attenuation inversion recovery, T2- and diffusion-weighted imaging. The patient developed akinetic mutism at 4 months and now after 6 months remains vegetative, completely bedbound. Presently she remains on Clonazepam,Sodium valproate and supportive care.

Discussion

The clinical presentation and neuroimaging findings were compatible with the nvCJD cases reported since 1996 for probable nvCJD. A human disease thought due to the same infectious agent as bovine spongiform encephalopathy (BSE), or mad cow disease.

Both the human and bovine disorders

are invariably fatal brain diseases with unusually long incubation periods

measured in years.

CourtesyDr. Debashis Chakraborty

Consultant NeurologistMD (Medicine), DNB

(Neurology), MRCP (UK)

Both the human and bovine disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent, a prion, resulting in the deposition of amyloid tissue that causes a breakdown of brain tissue leaving the infected brain with a “spongy” (“spongiform”) appearance. The disease in humans is sometimes called variant Creutzfeldt-Jakob disease (nvCJD). The BSE agent has been identified in the brain, spinal cord, retina, dorsal root ganglia (nervous tissue located near the backbone), and the bone marrow of cattle experimentally infected with this agent by the oral route. In addition to cattle, sheep are susceptible to experimental infection with the BSE agent by the oral route. Thus, in countries where flocks of sheep and goats may have been exposed to the BSE agent through contaminated feed, there exists a theoretical risk that these animals may have developed infections caused by the BSE agent and that these infections have been maintained in the flocks, even in the absence of continued exposure to contaminated feed (for example, through maternal transmission).

Everyone with nvCJD appears to have eaten beef and beef products at some time during their lives (but then so have most of the population). Investigation of a cluster of cases with nvCJD disclosed that most of the people were likely infected through their diet. Beef carcass meat appears to have been contaminated with the BSE agent in butchers’ shops where cattle heads were split.

Second MRI brain report

First MRI brain report

NEWS ROOM

Email: fhl@fortishospitals.in, Website: www.fortishospitals-kolkata.com

We look forward to hearing from you. Send in your views andsuggestions to enquiries@fortishospitals.in

730, Anandapur EM Bypass Road,

Kolkata: 700 107 Tel: 91-33-6628 4444

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