Into the brain through the nostrils (Sri Lanka)

There is purposeful concentration, while soft and soothing music plays in the background, as different teams of doctors and nurses, scrub up and move around Operating Theatre (OT) C of the Anuradhapura Teaching Hospital.

On the operating table is a pretty middle-aged woman, but something seems unusual with her face – it seems unduly large, with the nose being pronounced.

While Consultant Anaesthetist Dr. Ayesha Abeyratne and her team are preparing the patient for surgery, two Consultants from different specialties are studying the Magnetic Resonance Imaging pictures. They are Consultant Ear, Nose and Throat (ENT) Surgeon, Dr. Daminda Dumingoarachchi who is also a Head and Neck Surgeon and Consultant Neurosurgeon, Dr. Stravinsky Perera. Although not present in the OT that day, Consultant ENT Surgeon Dr. Upul Abeysundara is also very much a part of this pioneering work in Sri Lanka.

It is a Monday morning and we too are in OT clothes and masks privy to viewing an operation not done anywhere else in the state sector, except the Anuradhapura Hospital.

Accessing the patient’s brain through the nostrils is what the ENT and Neuro Teams will do in one of many trailblazing procedures, while the Anaesthetic Team will ensure that her vitals are stable and the able theatre nursing staff will play their part.

Image-guided (navigation-assisted) endoscopic trans-nasal trans-sphenoidal pituitary surgery is what we are about to view being performed in the country only since October this year. For, the 38-year-old patient lying inert on the operating table has a tumour just above her pituitary gland, between the optic nerves and the carotid arteries, making it a difficult operation-site to access. (The pea-size pituitary gland is dubbed the ‘master gland’ as it produces many hormones for the whole body, while also stimulating other glands to produce other hormones.)

Dr. Dumingoarachchi points out that the ENT navigator which the Anuradhapura Hospital was provided with recently, is like the GPS system of the brain which shows this vital organ’s architecture.

Dr. Perera explains that the patient has acromegaly, the abnormal production of growth hormones from the pituitary, causing a large nose and big hands etc. This is while it also causes an abnormal reduction in other hormones, affecting the functioning of the whole body. The enlarged pituitary is also compressing the main artery of the brain. A major issue is that the patient’s vision is being affected and if the tumour is not decompressed, she would go blind.

Dr. Daminda Dumingoarachchi

Matters also seem complicated as the patient had undergone ‘conventional’ brain surgery to remove an earlier tumour in the same area in 2009 in another hospital, but some parts had been left behind because it was complex surgery and the slightest slip of the scalpel could cause serious harm.

We watch in fascination as the surgical teams discuss how the tumour has grown around the right carotid artery making it a challenging procedure and is pressing down (compressing) on the brain.

This pituitary macroadenoma with acute intra-tumoural haemorrhage (bleeding) is “big” — 5X3.8X3.5cms, says Dr. Perera, adding that laterally, the lesion extends to the right cavernous sinus encircling almost the full circumference of the cavernous part of the right carotid artery. The left carotid artery is spared.

Super-imposing the patient’s Computed Tomography (CT) scan with images of the real anatomy, the ‘registration’ takes place for this ‘re-do’ surgery, as he explains that the anatomy is distorted after her previous surgery done seven years ago.

In the freezing OT, we don’t feel the time passing as both the Neurosurgeon and the ENT Surgeon attend to their tasks, two pairs of hands working in tandem, not looking down at the patient but up at the two screens, while navigating the pathways through the nostrils to the brain.

Usually, such a large tumour above the pituitary gland will entail open-skull surgery, with the brain having to be retracted (drawn away from the site of the operation) to gain access to the tumour, we learn. While the dangers of such surgery are numerous, the patient also has to spend a long time in the Intensive Care Unit (ICU) in the recovery phase.

With the frontiers of medicine pushed back more and more, Dr. Dumingoarachchi says that now skull-base structures can be approached through the nose, ear and throat. “Such surgery involves dealing with pathologies located on the under-surface of the brain, with Neurosurgeons and ENT Surgeons together accessing the skull-base more easily through the nose, ear and throat.”

Pointing out that these trans-nasal and trans-labyrinthine approaches to the skull-base will help bring down the rate of revision neurosurgeries and cut down the time that the patient will have to spend in the ICU post-operatively, he adds that the patients can go back home sooner without complications and be productive more quickly. Currently, some patients even go to other countries to get these services.

With the Health Ministry providing a high definition 3-chip endoscopic camera system, an anterior skull-base set, a micro-debrider and an ENT navigator, vast strides have been made at the Anuradhapura Hospital. They include:

Dr. Stravinsky Perera

Anterior skull-base surgeries — Endoscopic anterior skull-base surgeries, starting with pituitary surgeries using the endoscopic trans-sphenoidal approach for the first time in Sri Lanka. The latest addition, in October this year, is image-guidance (navigation).

The other procedures include cranio-pharyngioma, olfactory neuroblastoma, cerebrospinal fluid leak repairs, para-nasal sinus cancer resections and odontoid (cervical spine) decompression.

The advantages of this approach include a better field of view and ease of doing revision surgery if required, while leaving no external scars.

Nasal polyposis is also now being managed by image-guided powered full house functional endoscopic sinus surgeries, resulting in the reduction of the recurrence rate. This also cuts down revision surgeries.

*  Lateral skull-base surgeries – Through microscopic lateral skull-base surgery, trans-labyrinthine acoustic neuromas have been removed. With direct access to these eighth-nerve tumours, no brain retraction has been necessitated, resulting in less post-operative morbidity. The whole tumour can be removed while preserving the seventh cranial nerve, as the hospital has also been given intra-operative cranial nerve monitoring facilities.

Paying tribute to Dr. Palitha Mahipala, Director-General of Health Services, for making the navigator available to them, Dr. Dumingoarachchi also expresses appreciation to the Deputy Director-General (DDG) of the Biomedical Division, Muditha Jayathilake, Biomedical Engineers Apsara and Kumuduni and DDG Dr. Lakshmi Somatunga for all their support.

From http://www.sundaytimes.lk/161218/news/into-the-brain-through-the-nostrils-221019.html

UAE Patient’s (Pituitary) Brain Tumour Removed Through Nostrils

Dubai: A 34-year-old patient working as a crane operator has undergone a remarkable new procedure of surgery at Thumbay Hospital, Dubai, that facilitated the removal of a brain tumour through the nostrils.

The patient, Mehnaj Khan, a Pakistani crane operator, underwent endoscopic trans-nasal trans-sphenoidal surgery in September, where the tumour was removed through the nose by endoscopic surgery without any cut or stitches on the skin. The father of five children has now made a full recovery, with improved vision, a hospital spokesperson said.

Khan first noticed something was wrong when his eyesight began to diminish, first the right eye, followed by the left eye. Although he had ignored his frequent bouts of headache for two years, Khan was compelled to visit an ophthalmologist due to vision deterioration. When an eye check-up revealed nothing was wrong, he was referred to to Thumbay Hospital, where an MRI scan of the brain revealed that he had a large tumour in the pituitary gland, pressing on the optic apparatus of brain and also hypothalamus, a very vital part of brain. This tumour was pressing on his optic nerves, causing him to slowly lose his sight.

Dr. Ishwar Chandra Premsagar, consultant neurosurgeon at Thumbay Hospital who operated on Khan, said: “Conventionally, such operations require surgeons to open the skull — a procedure known as a craniotomy. Alternatively, affected portions of the brain are reached via major incisions in the side of the face or inside the mouth, leaving behind major scars of the surgery. However, the patient’s tumour was removed by suctioning it out through his nose.”

An ear nose and throat (ENT) surgeon and an eye surgeon were consulted to plan the surgery and save further deterioration of vision while providing a chance for complete recovery.

Khan, who was nearly blind in one eye with the tumour growth, expressed his gratitude to the hospital and the teams of surgeons as he noticed improvement in his vision after the surgery. By the end of the week, he could read too. The patient was very thankful to the team of surgeons.

Dr Premsagar added: “The endoscope provides a close-up view of the pituitary, allowing the surgeon to remove the entire tumour out in one go through the nostrils, causing no disfigurement or damage to the brain. On the other hand, the procedure ensures far less danger of brain damage or stroke, and the patient usually makes a quicker recovery. Although post-surgery, deterioration of vision stops, but one cannot guarantee complete recovery of vision. This patient was lucky as his vision improved, but it may not happen in all patients. Hence, it is extremely important that one should ensure early consultation, diagnosis and surgery to ensure high chances of recovery.”

From http://gulfnews.com/news/uae/health/uae-patient-s-brain-tumour-removed-through-nostrils-1.1933841

Interview with a Doctor on Trans-Sphenoidal surgery

Dr. Julius July: Neurosurgeon at the Neuroscience Center of Siloam Hospitals Lippo Village Karawaci 

A SIMPLE AND QUICK WAY TO REMOVE TUMORS VIA SURGERY THROUGH THE NOSTRIL

The mention of the word “surgery” evokes images of lengthy and elaborate procedures that involve delicate acts of cutting, abrading or suturing different parts of the body to treat an injury or disease.

This widely-held perception has led some to develop an irrational fear of surgery–especially if an operation involves a critical organ, such as the heart, or in the case of trans-sphenoidal surgery, a procedure used to remove tumors from the hormone-regulating pituitary gland located at the base of the brain.

Though the procedure has been around in different forms for the past three decades, individuals who may be in dire need of it might fear or avoid it.

To demystify this specific method of surgery, J+ spoke with Julius July, a neurosurgeon at the Neuroscience Center of Siloam Hospitals Lippo Village Karawaci. He has performed hundreds of trans-sphenoidal operations on patients throughout the country since 2008. Below is our interview, edited for length and clarity.

Tell us more about trans-sphenoidal surgery.

The goal is to extract benign tumors of the pituitary gland that are called pituitary adenoma. The pituitary gland controls different secretions of hormones. If there is a tumor and it grows large, one of the consequences could be that a patient goes blind. It can also lead to symptoms manifesting in other parts of the body due to excess hormone production, depending on the type of hormone affected by the tumor.

What does a neurosurgeon do during the procedure?

As neurosurgeons we use an endoscope with a camera attached to it and insert the instrument through the nostril. We go through the right nostril and through the sinus to reach the tumor and remove it. Once that is done, we add a coagulant to prevent bleeding. The operation takes only an hour to 90 minutes to perform and is minimally invasive. People come in and expect the surgery to last five or six hours. They hear “surgery” and fearfully assume that. But modern trans-sphenoidal surgery is simple, only lasting one to two hours.

What’s the prognosis after surgery?

In 80 percent of cases, all it takes is one surgery to remove a tumor. However, some need repeated intervention, while others require radiation. Some tumors want to invade their surroundings. In these cases, the surrounding area is a blood vessel. We can’t totally remove that type of tumor. But such cases are rare. If a patient needs more than two operations, we usually recommend radiation, because who wants to have a lot of operations?

What are the symptoms of pituitary adenoma?

Symptoms depend on whether a tumor affects hormone production or the optic nerve. The principal complaints are related to a patient’s field of vision becoming narrower. If there is a tumor in the pituitary gland area, the eye can’t see too widely. The tumors would press on the optic nerve, which leads to the periphery of your vision getting blurry.

If the tumor affects hormone production, the symptoms depend on the specific type of hormone that the tumor has affected. Different hormones have different roles. Excess prolactin hormones can lead to women–or even men–producing breast milk. If a woman who isn’t pregnant is producing breast milk, they need to be checked. The basic ingredient of milk is calcium. Without treatment, the woman will have porous bone problems. It also leads to reduced libido. If men have an excess of these prolactin hormones, they cannot get erections and will become impotent.

How does these problem develop in the first place?

Mutations lead to the creation of these benign tumors. Some things make mutations easier, such as smoking or exposure to radiation or specific chemicals. It could be anything. You could have eaten tofu and it had formalin or some meatballs with borax. Preventing it obviously requires a healthy lifestyle, but that’s easier said than done.

It’s not just one thing that causes these tumors.

Who does this pituitary tumor affect?

It affects both genders equally, more or less. The risk of pituitary adenoma compared to all other types of brain tumors is 15 percent. Children are also affected, though the condition is statistically much more likely to afflict adults. Of my patients, two in 70 would be children.

How is it diagnosed?

The doctor will check your hormones after a blood test and identify the problem. For example, if the condition affects growth hormones, a person can grow to two meters or more in height, which leads to gigantism. Alternatively, a condition could lead to horizontal growth–a bigger tongue, bigger fingers and changing shoes each month. The tongue can become so big that it causes breathing problems. Growth hormone overproduction is like a factory with the machine working overtime. As a result, a person’s life span can get cut in half. The heart works overtime, they keep growing and they die prematurely.

How many operations do you perform a year?

I’ve been doing these operations since 2008. I handle 60 to 70 such surgeries a year.

Any notable success stories to share?

One patient from Central Java came in blind. I examined him and said that there was no way we could save his vision by removing his tumor. He was crying. He had been blind for a week. But if no action was taken, the tumor would keep growing and would lead him to becoming crippled. At the end, he decided that he still wanted the operation. Surprisingly though, after the operation, he was able to see. Three months later, he was driving and reading newspapers. It was a fascinating case.

From http://www.thejakartapost.com/news/2016/07/30/well-being-trans-sphenoidal-surgery.html

Elevated late-night salivary cortisol may indicate recurrent Cushing’s disease

Carroll TB, et al. Endocr Pract. 2016;doi:10.4158/EP161380.OR.

 

Elevated late-night salivary cortisol may serve as an early biochemical marker of recurrent Cushing’s disease, and prompt intervention may result in clinical benefits for people with Cushing’s disease, according to recent study findings.

According to the researchers, late-night salivary cortisol level is more sensitive for detecting Cushing’s disease recurrence compared with urinary free cortisol or a dexamethasone suppression test.

Ty B. Carroll, MD, assistant professor at the Medical College of Wisconsin Endocrinology Center and Clinics in Menomonee Falls, and colleagues evaluated 15 patients (14 women; mean age, 49.1 years) with postsurgical recurrent Cushing’s disease (mean time to recurrence, 3.3 years) after initial remission to determine the performance of urinary free cortisol and late-night salivary cortisol measurements for detecting recurrent Cushing’s disease.

Participants were identified as having Cushing’s disease between 2008 and 2013; there was no standard for follow-up, but after remission confirmation participants were followed at least every 6 months after surgery for 2 years and then annually thereafter. Late-night salivary cortisol was the primary biochemical test to screen for recurrence, and follow-up tests with a dexamethasone suppression test, urinary free cortisol or other tests were performed if late-night salivary results were abnormal or if suspicion of recurrence was high.

Of the cohort, 80% had normal urinary free cortisol (< 45 µg/24 hours) at recurrence. Primary transphenoidal adenoma resection was performed in all participants. Evidence of pituitary adenoma on MRI at the time of recurrence was present in seven of 12 participants with normal urinary free cortisol and two of three participants with abnormal urinary free cortisol. Normal renal function was present in all participants, and 14 underwent testing with late-night salivary cortisol, dexamethasone suppression test and urinary free cortisol.

Of participants with normal urinary free cortisol at recurrence, nine had an abnormal dexamethasone suppression test (cortisol 1.8 µg/dL), and all had at least one elevated late-night salivary cortisol measurement (> 4.3 nmol/L). Mean late-night salivary cortisol was 10.2 nmol/L, and mean urinary free cortisol was 19.9 µg/24 hours.

Therapy for recurrent Cushing’s disease was administered in 11 of the 12 participants with abnormal urinary free cortisol. Adrenocorticotropic hormone (ACTH)-staining pituitary adenoma was confirmed in three participants who underwent repeat transphenoidal adenoma resection. Pharmacotherapy was administered to seven participants with normal urinary free cortisol, and two additional participants underwent bilateral adrenalectomy.

Abnormal dexamethasone suppression test was found in two participants with elevated urinary free cortisol at the time of recurrence, and two participants had confirmed abnormal late-night salivary cortisol. All three participants with elevated urinary free cortisol at the time of recurrence underwent therapy.

“This study has shown potential clinical benefit of either surgical or medical therapy in recurrent [Cushing’s disease] patients with elevations of [late-night salivary cortisol] and normal [urinary free cortisol],” the researchers wrote. “We believe that the outcomes observed in this retrospective case series suggest that the risk/benefit ratio of early treatment needs to undergo a more rigorous prospective evaluation utilizing [late-night salivary cortisol] elevation as an early biochemical marker of recurrent [Cushing’s disease].” – by Amber Cox

Disclosure: Carroll reports being a consultant for Corcept Therapeutics. Please see the full study for a list of all other authors’ relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/online/%7B9ea4e4ed-6428-49b8-9b2a-11462cb21349%7D/elevated-late-night-salivary-cortisol-may-indicate-recurrent-cushings-disease

Cushing’s disease recurrence predictive factors: Outcome analysis of patients in VANCOUVER over 30 years

Screenshot 2016-05-27 13.12.55

 

Pituitary Disorders/Neuroendocrinology

R Chen, J Levi, M Almalki, S Yi, M Johnson, E Ur

Summary: The objective of this study was to describe the management and outcomes of patients with Cushing’s disease (CD) in the Vancouver region over a 30-year period and to investigate the predictive factors of CD recurrence. Researchers found CD recurrence in 45.8% of patients who received initial transsphenoidal surgery (TSS), and that a post-operative serum cortisol level > 140nmol/L may be a positive predictor of recurrence in these patients.

Methods:

  • Researchers retrospectively reviewed the clinical charts from endocrinologists in Vancouver who provided consent to participate in this study.
  • Included in this study were 48 patients diagnosed with CD since 1985.

Results:

  • All 48 patients received initial TSS; the mean follow-up time was 11.73 (±6.98) years.
  • More than half of the patients (n=26, 54.2%) remained in remission, and 22 patients (45.8%) received subsequent interventions due to CD recurrence.
  • Second-line therapies included repeat TSS (40.9%), stereotactic radiotherapy (18.2%), and bilateral adrenalectomy (36.4%).
  • Among patients with disease recurrence, the average post-operative serum cortisol level was significantly higher (489.0 nmol/L vs 114.7nmol/L; P=0.003).
  • The positive predictive value for recurrence with post-operative serum cortisol > 140nmol/L was 76.5% (P=0.049), while serum cortisol < 140nmol/L had an 80% predictive value for non-recurrence (P=0.035).

From http://www.mdlinx.com/endocrinology/conference-abstract.cfm/ZZ37C4C5D3BF1A4FAE9C479A696660535B/57894/?utm_source=confcoveragenl&utm_medium=newsletter&utm_content=abstract-list&utm_campaign=abstract-AACE2016&nonus=0