Into the brain through the nostrils (Sri Lanka)

Posted on December 21, 2016 by MaryO

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

Filed under: pituitary, Rare Diseases, Treatments | Tagged: , , , , | Leave a comment »

Bilateral testicular tumors resulting in recurrent Cushing’s syndrome after bilateral adrenalectomy

Posted on December 21, 2016 by MaryO
Troy Puar1,2, Manon Engels3,4, Antonius E. van Herwaarden4, Fred C. G. J. Sweep4, Christina Hulsbergen-van de Kaa5, Karin Kamphuis-van Ulzen6, Vasileios Chortis7,8, Wiebke Arlt7,8, Nike Stikkelbroeck1, Hedi L Claahsen–van der Grinten3, Ad R.M.M. Hermus1
Corresponding author: Troy Puar, MRCP (UK), Department of Medicine, Div. of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands. Phone: +31 243614599, Fax: +31 243618809, e-mail: Troy_puar@cgh.com.sg
DOI: http://dx.doi.org/10.1210/jc.2016-2702
Received: July 14, 2016
Accepted: November 29, 2016
First Published Online: November 30, 2016
Abstract
Context:

Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing’s disease is extremely rare.

Patient:

We present a rare case of a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing’s disease with complete clinical resolution. Cushingoid features recurred 12 years later, along with bilateral testicular enlargement. Hormonal tests confirmed ACTH-dependent Cushing’s. Surgical resection of the testicular tumors led to clinical and biochemical remission.

Design and Results:
Gene expression analysis of the tumor tissue by qPCR showed high expression of all key steroidogenic enzymes. Adrenocortical-specific genes were 5.1 x 105 (CYP11B1), 1.8 x 102 (CYP11B2) and 6.3 x 104 (MC2R) times higher than non-steroidogenic fibroblast control. This correlated with urine steroid metabolome profiling showing 2-5 fold increases in the excretion of the metabolites of 11-deoxycortisol, 21-deoxycortisol and total glucocorticoids. Leydig-specific genes were 4.3 x 101 (LHCGR) and 9.3 x 100(HSD17B3) times higher than control and urinary steroid profiling showed 2-fold increased excretion of the major androgen metabolites androsterone and etiocholanolone. These distinctly increased steroid metabolites were suppressed by dexamethasone, but unresponsive to hCG stimulation, supporting the role of ACTH, but not LH, in regulating tumor-specific steroid excess.
Conclusion:

We report bilateral testicular tumors occurring in a patient with recurrent Cushing’s disease 12 years after bilateral adrenalectomy. Using mRNA expression analysis and steroid metabolome profiling, the tumors demonstrated both adrenocortical and gonadal steroidogenic properties, similar to testicular adrenal rest tumors found in patients with congenital adrenal hyperplasia. This suggests the presence of pluripotent cells even in patients without CAH.

Affiliations
  • 1Department of Medicine, Division of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 2Department of Endocrinology, Changi General Hospital, Singapore 529889, Singapore.
  • 3Department of Paediatrics, Division of Endocrinology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 4Department of Laboratory Medicine, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 5Department of Pathology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 6Department of Radiology, Radboud University Medical Centre, 6500 HB Nijmegen, The Netherlands.
  • 7Institute of Metabolism and Systems Research, University of Birmingham, Birmingham N15 2TT, United Kingdom.
  • 8Centre for Endocrinology, Diabetes, and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, United Kingdom.

– See more at: http://press.endocrine.org/doi/abs/10.1210/jc.2016-2702#sthash.F4lfWg9j.dpuf

Filed under: adrenal, Cushing's, Rare Diseases, Treatments | Tagged: , , , , , , | Leave a comment »

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

Posted on December 2, 2016 by MaryO

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

Filed under: pituitary, Rare Diseases, Treatments | Tagged: , , , , , , , , , , , | Leave a comment »

Psoriatic arthritis patients face more endocrine comorbidities

Posted on July 27, 2016 by MaryO

By: DAMIAN MCNAMARA, Clinical Endocrinology News Digital Network

JULY 23, 2016

AT 2016 GRAPPA ANNUAL MEETING

VITALS

Key clinical point:Patients with psoriatic disease had a significantly higher prevalence of diabetes mellitus and some other endocrine comorbidities.

Major finding: In a univariate analysis, the risk for Cushing’s disease was notably higher among psoriatic arthritis patients, compared with controls (odds ratio, 5.31).

Data source: Retrospective, cross-sectional comparison of 3,161 patients with psoriatic arthritis and 31,610 matched controls.

Disclosures: Dr. Haddad, Dr. Coates, and Dr. Kavanaugh reported having no relevant financial disclosures.

The large, population-based cohort is a strength of the study. “We are now going back to see how many of these patients were seen by rheumatologists,” Dr. Haddad said. A lack of association with disease burden is a potential limitation, he added.

Thirty percent of patients were treated with biologics and about 67% with steroids. “That number treated with steroids seems high,” a meeting attendee commented. Dr. Haddad explained that it is the percentage ever treated with steroids, not necessarily currently on steroids.

In a separate session at the GRAPPA meeting addressing psoriatic disease treatment recommendations, an attendee asked about specific recommendations for comorbidities. For now, GRAPPA plans to include comorbidities within its overall recommendations, as it did in its most recent update, released in January 2016. A limited amount of data is a primary reason.

“As the evidence on comorbidities gets better, we may someday have separate recommendations for comorbidities,” said Laura Coates, MD, a clinical lecturer in rheumatology at the University of Leeds (England).

“The comorbidities are very important,” said Arthur F. Kavanaugh, MD, professor of medicine at the University of California, San Diego. “That’s trickier and deals with the international nature of GRAPPA. It’s hard to say, ‘Go see this specialist,’ because that might not be standard of care in that country.”

Dr. Haddad, Dr. Coates, and Dr. Kavanaugh reported having no relevant financial disclosures.

 From http://www.clinicalendocrinologynews.com/specialty-focus/diabetes/single-article-page/psoriatic-arthritis-patients-face-more-endocrine-comorbidities/f8700c8ffde9fc2534295458feb8eba3.html?utm_source=News_CEN_eNL_072616&utm_medium=email&utm_content=Psoriatic+arthritis+patients+face+more+endocrine+comorbidities

Filed under: Cushing's, Rare Diseases | Tagged: , , , , , | 1 Comment »

GH therapy increases fracture risk in patients previously treated for acromegaly

Posted on July 16, 2016 by MaryO

van Varsseveld NC, et al. Pituitary. 2016;doi:10.1007/s11102-016-0716-3.

Adult patients with severe growth hormone deficiency previously treated for acromegaly saw an increased fracture risk after 6 years of growth hormone replacement therapy, whereas those previously treated for Cushing’s disease did not experience the same risk, according to a recent observational study.

Nadege C. van Varsseveld, MD, of the department of internal medicine at VU University Medical Center in Amsterdam, and colleagues analyzed data from 1,028 patients with previous nonfunctioning pituitary adenoma (NFPA; n = 783), acromegaly (n = 65) and Cushing’s disease (n = 180), identified through the Dutch National Registry of Growth Hormone Treatment in Adults, a nationwide, long-term surveillance study in patients with severe GH deficiency. Data were collected biannually from medical records through 2009. Baseline DXA measurements were available for 414 patients; 71 (17.1%) had osteoporosis at one or more of the measured sites; 147 (35.5%) had osteopenia.

During a mean follow-up of 5.2 years, researchers found that 166 of patients with previous NFPA were prescribed osteoporosis medications (21.3%), as were 69 patients with previous Cushing’s disease (38.5%) and 22 patients with previous acromegaly (33.4%). During follow-up, 39 patients experienced fractures (3.8%; 32 experiencing one fracture), including 26 patients in the previous NFPA group, eight patients in the previous Cushing’s disease group and five patients in the previous acromegaly group. The median time between baseline and first fracture was 2.4 years (mean age, 59 years).

Researchers found that fracture risk did not differ between groups before 6 years’ follow-up. Fracture risk increased in patients with previous acromegaly after 6 years’ follow-up, but not for those with previous Cushing’s disease vs. patients with NFPA. Results persisted after adjustment for multiple factors, including sex, age, fracture history and the extent of pituitary insufficiency.

The researchers noted that patients with previous Cushing’s disease were younger and more often women and had a greater history of osteopenia or osteoporosis, whereas patients with acromegaly had a longer duration between tumor treatment and the start of GH therapy and were treated more often with radiotherapy.

“During active acromegaly, increased bone turnover has been observed, but reported effects on [bone mineral density] are heterogeneous,” the researchers wrote. “It is postulated that cortical BMD increases, whereas trabecular BMD decreases or remains unaffected.

“The increased fracture risk in the present study may be a long-term effect of impaired skeletal health due to previous GH excess, even though this was not reflected by an increased occurrence of osteopenia or osteoporosis in the medical history,” the researchers wrote. – by Regina Schaffer

Disclosure: One researcher reports receiving consultancy fees from Novartis and Pfizer.

From http://www.healio.com/endocrinology/hormone-therapy/news/online/%7B92a67ad7-3bd5-46f0-b999-0a8e3486edab%7D/gh-therapy-increases-fracture-risk-in-patients-previously-treated-for-acromegaly

Filed under: Cushing's, pituitary, Rare Diseases, Treatments | Tagged: , , , , , , , , , | Leave a comment »

« Previous Page — Next Page »