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Cutaneous Features of Neurofibromatosis

Cutaneous Features of Neurofibromatosis

Teaser: 

Sang-Eun Kim , BSc, MSc,1 Joseph M. Lam, MD, FRCPC,2

1Faulty of Medicine, University of British Columbia, BC.
2Assistant Clinical Professor, Department of Paediatrics, Associate Member, Department of Dermatology, University of British Columbia, BC.

CLINICAL TOOLS

Abstract: Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder that is characterized by café-au-lait spots, axillary or inguinal freckles, cutaneous neurofibromas, and skeletal dysplasias. Currently, there are no curative therapies for NF1 but medical therapies, including systemic sirolimus, have opened the door for significant medical advances in the treatment of NF1. Management of NF1 has been focused on routine examinations looking out for potential complications of NF1. However, many patients with NF1 are missed and may not be diagnosed early. The following review article will provide an overview of select common and uncommon cutaneous features of NF1 to help the practitioner recognize, diagnose and treat patients with NF1.
Key Words: Neurofibromatosis type 1, café-au-lait spots, axillary freckles, inguinal freckles, cutaneous neurofibromas.
Clinical diagnosis of NF1 requires the presence of at least 2 out of the 7 criteria.
Not all patients with café-au-lait spots will have NF1.
Axillary and inguinal freckling are the most specific criteria for NF1.
Three different types of cutaneous neurofibromas are dermal, subcutaneous, and plexiform neurofibromas (PNs). PNs can become malignant.
Juvenile xanthogranuloma and nevus anemicus are uncommon associated cutaneous features of NF1.
NF1 is a genetic disorder and there is no cure.
Patients should be routinely monitored for rare complications and annual exam should include BP measurement, skin and bone abnormality assessment, visual acuity checks, and ophthalmological evaluations.
Not all Cafe-au-lait spots require specialist referral however early recognition and prompt referral is essential.
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Related Terms: Dermatology, Neurofibromatosis type 1, café-au-lait spots, axillary freckles, inguinal freckles, cutaneous neurofibromas

CORE BACK TOOL 2016: New and Improved!

Members of the College of Family Physicians of Canada may claim one non-certified credit per hour for this non-certified educational program.

Mainpro+® Overview
Pre-test CME Quiz Post-test CME Quiz
Teaser: 

Dr. Julia Alleyne, BHSc(PT), MD, CCFP, Dip. Sport Med MScCH,1 Yoga Raja Rampersaud, MD, FRCSC,2 Jess Rogers3Dr. Hamilton Hall, MD, FRCSC,4

1 is a Family Physician practising Sport and Exercise Medicine at the Toronto Rehabilitation Institute, University Health Network. She is appointed at the University of Toronto, Department of Family and Community Medicine as an Associate Clinical Professor.
2Associate Professor Department of Surgery, University of Toronto, Divisions of Orthopaedic and Neurosurgery, University Health Network Medical Director, Back and Neck Specialty Program, Altum Health, Past President Canadian Spine Society, Toronto, ON.
3 is the Director at the Centre for Effective Practice (CEP). Jess' role includes developing evidence-based clinical guidance for providers. Jess was the Project Lead in executing the primary care provider education component of Ontario's Low Back Pain initiative including the CORE Back Tool. CEP is pleased to have funded the update of the CORE Back Tool 2016 to continue supporting primary care providers.
4 is a Professor in the Department of Surgery at the University of Toronto. He is the Medical Director, CBI Health Group and Executive Director of the Canadian Spine Society in Toronto, Ontario.

CLINICAL TOOLS

Abstract: Through the redesign of the already successful Clinically Organized Relevant Exam (CORE) Back Tool, primary care clinicians now have a more comprehensive, user-friendly approach to clinical decision making for patients presenting with low back pain. The key components of the tool include a high yield history connected to mechanical low back pain patterns, embedded key patient messages, clear listing of appropriate radiological indications, criteria for consultant referrals as well as a management matrix geared to office practice. A clinical case will be used to demonstrate the application of the tool to practice and instruct the reader on the key features.
Key Words: Low Back Pain, Tool, Primary Care Providers, Management.

Members of the College of Family Physicians of Canada may claim MAINPRO-M2 Credits for this unaccredited educational program.

www.cfpc.ca/Mainpro_M2

You can take quizzes without subscribing; however, your results will not be stored. Subscribers will have access to their quiz results for future reference.

Pre-test CME Quiz Post-test CME Quiz
1. Mechanical Patterns are a logical way to conceptualize, assess and manage low back pain.
2. If pain does not fit a mechanical pattern, the patient may have non-spine referred pain from organs or a chronic pain disorder.
3. Radicular (nerve) pain will have a positive straight leg raise (SLR) with reproduction of the typical leg dominant pain and possible abnormal neurological signs.
Initial patient management should include goals of reducing pain and increasing activity.
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Related Terms: Back Health, low back pain, tool, primary care providers, management

Osteoporotic Vertebral Compression Fractures: Diagnosis and Management

Osteoporotic Vertebral Compression Fractures: Diagnosis and Management

Teaser: 

Michael M.H. Yang, MD, M.Biotech,1 W. Bradley Jacobs, MD, FRCSC,2

1Division of Neurosurgery, Department of Clinical Neuroscience, University of Calgary, Calgary, Alberta, Canada.
2Division of Neurosurgery, Department of Clinical Neuroscience, University of Calgary, Calgary, Alberta, Canada.

CLINICAL TOOLS

Abstract: Osteoporotic vertebral compression fractures (VCFs) are the most common fragility fracture and have significant impact on numerous indices of health quality. High risks patients should be identified and appropriate preventative therapy initiated. The majority of VCFs can be managed in a non-operative fashion, with analgesia as required to support progressive mobilization. Patients who fail non-operative measures may be considered for percutaneous vertebral augmentation. However, the efficacy of these procedures in altering the natural history of recovery is controversial. Surgery has a limited role in the initial management of VCFs and is typically restricted to the rare circumstance of VCF associated with acute neurological dysfunction.
Key Words: osteoporosis, vertebral compression fracture, vertebroplasty, kyphoplasty.

Members of the College of Family Physicians of Canada may claim MAINPRO-M2 Credits for this unaccredited educational program.

www.cfpc.ca/Mainpro_M2

You can take quizzes without subscribing; however, your results will not be stored. Subscribers will have access to their quiz results for future reference.

Pre-test CME Quiz Post-test CME Quiz
1. Osteoporosis is under diagnosed in Canada. Early diagnosis, fragility fracture risk stratification and initiation of preventative treatment is important, as osteoporotic vertebral compression fractures (VCFs) have a significant associated personal and societal health utility cost.
2. Patients suspected of having a VCF should have an AP and lateral X-ray of the suspected region. If VCF is confirmed, an upright X-ray should be performed to assess for stability. CT and/or MR imaging has limited utility in the absence of red flag signs or symptoms.
3. VCFs should be managed with initiation of an appropriate pain management regiment, early bed rest as required for pain control and gradual mobilization. Patients with refractory pain 4–6 weeks after onset can be considered for percutaneous vertebral cement augmentation (e.g. vertebroplasty), although the clinical efficacy of such procedures remains unclear.
A few screening measurements can be performed in the office setting to help significantly improve the likelihood of detecting a VCF on radiological studies. They include prospective height loss of greater than 2cm or a height loss, or a height loss based on history of more than 6cm, a rib-to-pelvis distance of less than 2 fingerbreadths, or an occipital-to-wall distance greater than 5cm.
Most patients with osteoporotic VCFs do not need a referral to a spine surgeon. Acute pain from a new VCF usually improves over a period of 6 weeks. Non-operative management should follow the WHO analgesic ladder starting with acetaminophen/NSAIDs followed by opioids, as necessary. The goal of treatment is to provide pain relief and facilitate early functional rehabilitation.
Patients with high or medium 10-year fracture risk should be considered for pharmacotherapy to prevent the progression of low bone mineral density and osteoporotic fractures.
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Related Terms: Back Health, osteoporosis, vertebral compression fractures, vertebroplasty, kyphoplasty

Spine and Sport: Are Athlete's Back Injuries Different?

Spine and Sport: Are Athlete's Back Injuries Different?

Teaser: 

Dr. Julia Alleyne, BHSc(PT), MD, CCFP, Dip. Sport Med MScCH,

Family Physician practising Sport and Exercise Medicine at the Toronto Rehabilitation Institute, University Health Network. She is appointed at the University of Toronto, Department of Family and Community Medicine as an Associate Clinical Professor.

CLINICAL TOOLS

Abstract: Athletes participating in training and competition for an average of 8 hours a week have a one year prevalence for spine injuries as high as 68%; an average increase of 18-31% compared to non-athletes. Except for young growing athletes at risk for structural deformity, most spine injuries are soft tissue and self-limiting. Risk factors include a sudden increase in training hours, transition in strength and coordination related to growth, sustained back flexion, reduced dynamic core stability and repetitive trunk rotation and hyper extension. Decreased training levels following back injury lead to deconditioning and muscle imbalance increasing the risk of recurrence and prolonging recovery. Core stability testing can identify patients for targeted exercise.
Key Words: Sport-related, spine, hypermobility, core stability, overuse.

Members of the College of Family Physicians of Canada may claim MAINPRO-M2 Credits for this unaccredited educational program.

www.cfpc.ca/Mainpro_M2

You can take quizzes without subscribing; however, your results will not be stored. Subscribers will have access to their quiz results for future reference.

Pre-test CME Quiz Post-test CME Quiz
Frequent repetition and sustained postures in rotation, hyperextension and full flexion require advanced levels of strength and flexibility for the athlete to remain injury-free.
The two most common risk factors for low back pain in training athletes is overuse strain and excessive spinal movements.
Treatment consists of both reducing the demands on the paraspinal muscles and increasing the amount of core stability.
It is important to screen for generalized joint hypermobility syndrome (JHS) affecting all joints using the Beighton Score, as this condition may require investigation and can be an indication of other medical syndromes.
The most specific test with high inter-rater reliability to determine core stability is the single leg standing balance stork test. The patient stands on one leg and raises the other knee to 90 degrees then maintains balance for a minimum of 25 seconds.
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Related Terms: Back Health, Sports Medicine, Sport-related, hypermobility, core stability, overuse, spine

TINNITUS is an "Aura Symptom" in Need of a Multidisciplinary Approach to Facilitate Diagnosis and Treatment

TINNITUS is an "Aura Symptom" in Need of a Multidisciplinary Approach to Facilitate Diagnosis and Treatment

Teaser: 

Dr. Pradeep Shenoy, MD, FRCS, FACS, DLO,1 Dr. Eric Deschenes, Au. D.2

1Otolaryngolost , Campbellton, NB, Canada.
2Audiologist, Campbellton, NB. Canada.

CLINICAL TOOLS

Abstract: Tinnitus is a perception of sound in the absence of sound stimulation (Figure 1). Various reasons are blamed for the causes of the tinnitus. Very rarely, tinnitus is seen in normal-hearing children where no obvious cause is detected. In these instances, tinnitus does not persist for long. In some people it may occur spontaneously as in old age, and in some individuals it is induced by noise exposure, ototoxic drug use, stress, smoking, or excessive coffee consumption (Figure 2). In some, tinnitus may be associated with other symptoms like vertigo and deafness. Such symptoms can be correlated with congenital sensorineural hearing loss, wax accumulation, serous otitis media, Meniere's disease, vestibular neuronitis, acoustic neuroma, vascular causes like a/v malformation or fistulae, and also in some patients, temperomandibular dysfunction. Tinnitus can cause anxiety, depression and sleep disorders, and in some individuals, extreme anxiety can lead to suicidal tendencies. Conventional medical treatment uses medication, sound therapy and relaxation. Management using electromagnetic stimulation and low intensity laser is also reported in the literature.
Key Words: Tinnitus counselling, sound therapy, hyperacusis, ototoxic drugs, presbyacusis, noise induced deafness (acoustic trauma), electromagnetic therapy, relaxation exercises.
Tinnitus is the perception of sound without external acoustic stimuli and is often described as ringing, whistling, buzzing, gushing of water, or a pulsatile noise.
Most researchers theorize that tinnitus is caused by initial damage to the outer hair cells in the cochlea, followed by impairment of the inner hair cells.
Tinnitus can cause anxiety, depression, sleep disorders, and in some cases, extreme anxiety that can lead to suicidal tendencies.
There is no method to eliminate tinnitus entirely; the goal with patients suffering from tinnitus is to provide the tools necessary to effectively manage their reaction to tinnitus symptoms.
Tinnitus can be attributed to a wide variety of causes, and it is difficult to study and treat tinnitus because of the lack of objective diagnostic tools.
To help manage tinnitus symptoms, sound therapy (tinnitus masking and tinnitus retraining) can be used in conjunction with alternative therapies like relaxation exercises, breathing exercises, hypnosis, vitamins and herbs, low level laser treatment, and electromagnetic treatment.
To have access to full article that these tools were developed for, please subscribe. The cost to subscribe is $80 USD per year and you will gain full access to all the premium content on www.healthplexus.net, an educational portal, that hosts 1000s of clinical reviews, case studies, educational visual aids and more as well as within the mobile app.
Related Terms: Otolaryngology, Tinnitus counselling, sound therapy, hyperacusis, ototoxic drugs, presbyacusis, noise induced deafness (acoustic trauma), electromagnetic therapy, relaxation exercises

A Scaly Periorbital Rash in a Preschool-aged Boy

A Scaly Periorbital Rash in a Preschool-aged Boy

Teaser: 

Jennifer Smitten, MD, FRCPC,1 Joseph M Lam, MD, FRCPC,2

1BC Children's Hospital, University of British Columbia, BC.
2Assistant Clinical Professor, Department of Paediatrics, Associate Member, Department of Dermatology, University of British Columbia, BC.

CLINICAL TOOLS

Abstract: A healthy 4-year-old boy presented with an 8-month history of a pruritic scaly eruption around his right eye associated with several small pearly papules on the face. A clinical diagnosis of an eczematous id reaction to molluscum contagiosum was made. While up to 40% of cases of molluscum contagiosum may have an associated eczematous dermatitis, these are often under-recognized or misdiagnosed.
Key Words: Pediatrics, Dermatology, Dermatitis, Molluscum, Eczema, Id reaction, Viral exanthem, Hypersensitivity.
Eczematous id reactions to molluscum contagiosum (MC) in children are common, occurring in up to 40% of cases of MC.
Id reactions to MC can be challenging to diagnose, as they may occur at sites distant from the MC lesions.
Id reactions can be caused by a variety of infectious and noninfectious dermatoses.
Asymptomatic id reactions do not require pharmacologic treatment and a watchful waiting approach is reasonable.
1. Id reactions can be caused by a variety of infectious and noninfectious dermatoses, including allergic contact dermatitis to nickel, scabies infestation, tinea infection and molluscum infection.
2. In a unilateral eczematous dermatitis, consider molluscum dermatitis, especially in a child with no personal or family history of atopy.
3. Treatment of symptomatic id reactions may help to reduce spread of MC via autoinoculation from scratching.
To have access to full article that these tools were developed for, please subscribe. The cost to subscribe is $80 USD per year and you will gain full access to all the premium content on www.healthplexus.net, an educational portal, that hosts 1000s of clinical reviews, case studies, educational visual aids and more as well as within the mobile app.
Related Terms: Dermatology, pediatric, dermatology, dermatitis, Molluscum, Eczema, Id reaction, Viral exanthem, Hypersensitivity

Bipolar Electrofulgration with Endoscopic Approach in Allergic Turbinates

Bipolar Electrofulgration with Endoscopic Approach in Allergic Turbinates

Teaser: 

Dr. Sohail Abdul Malik, DLO, FCPS (ENT), Dr. Pooja Chodankar, MBBS, DLO, Dr. Pradeep Shenoy, MD, DLO, FRCS, FACS,

Former Head of ENT Department Armed Forces Hospital, Kuwait,
Currently the ENT service chief, Campbellton Regional Hospital, Campbellton, New Brunswick, Canada.

CLINICAL TOOLS

Abstract: Rhinological practice in Kuwait has always included a barrage of cases of allergic rhinitis. Arrays of treatment modalities like monopolar electrocautery to the inferior turbinates and laser vaporization have been experimented with and have yielded a diversity of results. Here is a synopsis of the use of endoscopic bipolar cauterization of middle and inferior turbinates in cases of allergic rhinitis, a treatment which achieves superior results in comparison with other therapeutic options.
Key Words: bipolar cauterization, bipolar electrofulguration, inferior turbinates, nasoendoscope.

Allergic rhinitis is a common problem in the Middle East region; several treatment modalities have been experimented with to improve patient symptoms.
Cautery with a specially designed bipolar probe can be used to cauterize different parts of the turbinates.
Endoscopic bipolar diathermy was deemed to be less useful in patients with moderate to severe deviation of the septum who were therefore excluded from the study.
Endoscopic bipolar diathermy demonstrated better long term results than other treatment options such as: submucosal diathermy, partial inferior turbinectomy and linear cautery.
Endoscopic bipolar diathermy as a treatment option increased nasal airflow with minimal damage to the mucocillary function and maximal destruction of submucosal tissue to the engorged portion of the inferior turbinates.
There is evidence of subjective improvement in the nasal symptoms of 89.1% of cases, and objective improvement of symptoms in 65.5% of cases which combined rhinomanometric study and nasoendoscopy.
To have access to full article that these tools were developed for, please subscribe. The cost to subscribe is $80 USD per year and you will gain full access to all the premium content on www.healthplexus.net, an educational portal, that hosts 1000s of clinical reviews, case studies, educational visual aids and more as well as within the mobile app.
Related Terms: Otolaryngology, bipolar cauterization, bipolar electrofulguration, inferior turbinates, nasoendoscope

Vertebral Metastatic Disease: A Practice Guideline for the General Practitioner

Vertebral Metastatic Disease: A Practice Guideline for the General Practitioner

Teaser: 

Michael S. Taccone,1 Markian Pahuta,2 Darren M.Roffey,3,4Eugene K. Wai,2,3,4

1Division of Neurosurgery, Department of Surgery, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada.
2Division of Orthopedic Surgery, Department of Surgery, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada.
3Ottawa Combined Adult Spinal Surgery Program, The Ottawa Hospital, Ottawa, ON, Canada.
4Clinical Epidemiology Program, Ottawa Hospital Research Institute, Ottawa, ON, Canada.

CLINICAL TOOLS

Abstract: Vertebral metastatic disease afflicts a significant proportion of cancer patients, most commonly those with breast and lung disease. Symptoms can include tumor-related pain, neurological deficit from spinal cord or nerve compression and pathological fracture with mechanical instability. Appropriate workup includes identifying the primary disease, defining the extent of spinal and extra-spinal pathology and classifying spinal stability based on the pattern of osseous involvement. Specific therapy for the vertebral metastatic disease can include pharmacologic therapy to deliver analgesia, steroids, bisphosphonate, anti-neoplastic therapy, radiation therapy as either primary or adjuvant therapy and surgical intervention for mechanical or neurologic instability.
Key Words: Vertebral metastatic disease, metastatic epidural spinal cord compression, spinal instability, spine surgery, spinal radiation therapy, pathologic fracture.

Members of the College of Family Physicians of Canada may claim MAINPRO-M2 Credits for this unaccredited educational program.

www.cfpc.ca/Mainpro_M2

You can take quizzes without subscribing; however, your results will not be stored. Subscribers will have access to their quiz results for future reference.

Pre-test CME Quiz Post-test CME Quiz
Red flags are non-specific and unreliable means of determining spinal malignancy in patients with back pain. Clinical suspicion combined with history and physical exam are best for increasing pre-test probability of imaging studies.
Initial evaluation and referral to definitive management should be made within 24 hours of detection of significant neurological deficit, significant metastatic epidural spinal cord compression or instability.
MRI is the imaging modality of choice for initial evaluation and assessment of overall spinal tumor burden.
Vertebral metastatic disease is very common in patients with cancer.
SINS, ESCCS, Tomita score, Tokuhashi score and the Modified Bauer scores are all important tools for determining the most appropriate referral.
In eligible candidates, surgery with adjuvant radiotherapy yields faster and more sustainable neurologic stability and recovery.
To have access to full article that these tools were developed for, please subscribe. The cost to subscribe is $80 USD per year and you will gain full access to all the premium content on www.healthplexus.net, an educational portal, that hosts 1000s of clinical reviews, case studies, educational visual aids and more as well as within the mobile app.
Related Terms: Back Health, Vertebral metastatic disease, metastatic epidural spinal cord compression, spinal instability, spine surgery, spinal radiation therapy, pathologic fracture

Error in Radiology: Negligence or Human Nature

D'Arcy Little, MD CCFP FRCPC, Chief of Diagnostic Imaging, Orillia Soldiers' Memorial Hospital,
Adjunct Clinical Lecturer, Department of Medical Imaging, University of Toronto, Toronto, ON,
Forensic Radiologist, Forensic Sciences and Coroners' Complex, Toronto, ON,
2015 Resident in Scientific Communications, Banff Centre, Banff, AB.

Related Terms: Radiology, radiology, errors