أرشيفات التصنيف: What is New in Medicine

Infantile Scoliosis

Infantile Scoliosis


Author: Palaniappan Lakshmanan, MBBS, MS, AFRCS, FRCS (Tr & Orth), Specialist Registrar, Department of Trauma and Orthopedics, Wansbeck General Hospital, UK

Coauthor(s): Jeetender Pal Peehal, MBBS, MS, MRCS, Knee Research Fellow, Positional MRI Centre, Woodend Hospital, UK; Sashin Ahuja, MBBS, FRCS, MSc, MS, Consultant Spinal Surgeon, Department of Orthopedics, University Hospital Of Wales, Cardiff, UK

Introduction

The term scoliosis is derived from the Greek word skol, meaning “twists and turns” and refers to a sideward (right or left) curve in the spine. Scoliosis is not a simple curve to one side but, in fact, is a more complex, 3-dimensional deformity that often develops in childhood


Preoperative and postoperative radiographs show a....

Preoperative and postoperative radiographs show an increase in the space available for lung (SAL) after correction of scoliosis by VEPTR (vertical expandable prosthetic titanium rib

Preoperative and postoperative radiographs show a...

Preoperative and postoperative radiographs show an increase in the space available for lung (SAL) after correction of scoliosis by VEPTR (vertical expandable prosthetic titanium rib).

Recent studies

In a retrospective study of the treatment of patients with idiopathic infantile scoliosis, 31 consecutive patients (average age, 25 mo) with a primary diagnosis of idiopathic infantile scoliosis were reviewed. Treatment modalities included bracing, serial body casting, and vertical expandable prosthetic titanium rib (VEPTR). Of the 31 patients, 17 were treated with a brace, 9 of whom had curve progression and subsequently received other treatments. Of the 8 patients who responded to brace treatment, overall improvement was 51.2%. Patients who received body casts had a mean preoperative Cobb angle of 50.4º and had an average correction of 59.0%. Patients who were treated with VEPTR had a mean preoperative Cobb angle of 90º and had an average correction of 33.8%. The study results suggest that body casting is useful in cases of smaller, flexible spinal curves, and VEPTR is a viable alternative for larger curves.1

Another retrospective case series, of magnetic resonance imaging (MRI) findings in patients with presumed infantile idiopathic scoliosis, reviewed the medical records of 54 patients. MRI revealed a neural axis abnormality in 7 (13%) of 54 patients who underwent MRI. Of these 7 patients, 5 (71.4%) required neurosurgical intervention. Tethered cord requiring surgical release was identified in 3 patients, Chiari malformation requiring surgical decompression was found in 2 patients, and a small nonoperative syrinx was found in 2 patients. The authors concluded that on the basis of these findings, close observation may be a reasonable alternative to an immediate screening MRI in patients presenting with presumed infantile idiopathic scoliosis and a curve greater than 20º.2

A recent study reviewed the frequency of asymmetric lung perfusion and ventilation in children with congenital or infantile thoracic scoliosis before surgical treatment and the relationship between Cobb angle and asymmetry of lung function. The authors found that asymmetric ventilation and perfusion between the right and left lungs occurred in more than half of the children with severe congenital and infantile thoracic scoliosis, but the severity of lung function asymmetry did not relate to Cobb angle measurements. Asymmetry in lung function was influenced by deformity of the chest wall in multiple dimensions and could not be ascertained by chest radiographs alone.3

History of the Procedure

Probably the oldest mention of scoliosis is in ancient Hindu mythology (3500 to 1800 BC), in which Krishna corrects the hunchback of one of his followers. Hippocrates (460 to 377 BC) wrote about scoliosis and devices to correct it. The term infantile scoliosis was first used by Harrenstein in 1930 and by James in 1951 in describing the clinical entity idiopathic infantile scoliosis.4,5,6

Problem

The term infantile scoliosis is used specifically to describe scoliosis that occurs in children younger than 3 years. Other terms for scoliosis also depend on the age of onset, such as juvenile scoliosis, which occurs in children aged 4-9 years, and adolescent scoliosis, which occurs in those aged 10-18 years. These terms, however, are now being replaced by the broader terms early-onset scoliosis and late-onset scoliosis, depending on whether the scoliosis occurs before or after 5 years of age.

In 80% of cases of scoliosis, there is no obvious cause; this is termed idiopathic scoliosis. In the remaining 20% of cases, a definite cause can be found. These cases are divided into 2 types: nonstructural (functional) and structural scoliosis, which could be part of a well-recognized syndrome (syndromic scoliosis), congenital spinal column abnormalities (congenital scoliosis), neurologic disorders, and genetic conditions.

The syndromes that can produce congenital scoliosis are VATER syndrome (vertebral anomalies, anorectal anomalies, tracheo-esophageal fistula, and renal anomalies), VACTERL syndrome (vertebral anomalies, anorectal anomalies, tracheo-esophageal fistula, renal and vascular anomalies, and cardiac and limb defects), Jarcho-Levin syndrome, Klippel-Feil syndrome, Alagille syndrome, Wildervank syndrome, Goldenhar syndrome, Marfan syndrome, and MURCS association (M ü llerian, renal, cervicothoracic, and somite abnormalities).

The congenital anomalies of the vertebral spinal column include defects of segmentation (block vertebra, unilateral bar) and defects of formation (hemivertebra — fully segmented, semisegmented, incarcerated and nonsegmented, wedge vertebra). The neurologic deficits in congenital scoliosis may be secondary to the spinal deformity or may be associated with vertebral anomalies (spinal dysraphism — diastematomyelia, myelocele, myelomeningocele, meningocele). A higher incidence of idiopathic scoliosis has been reported in families of children with congenital scoliosis. Spondylocostal dysostosis (Jarcho-Levin syndrome) has a genetic etiology.7,8,9,10

Frequency

Infantile scoliosis is a rare condition, accounting for less than 1% of cases of idiopathic scoliosis in North America; in Europe, the rate is 4%.

Sex: Males account for 60% of the cases of early-onset scoliosis; 90% of the cases of early-onset scoliosis resolve spontaneously, but the other 10% of cases progress to a severe and disabling condition. Females constitute 90% of late-onset cases and need close monitoring to intervene at appropriate times.

Etiology

Although the exact cause of idiopathic infantile scoliosis is not known, hypotheses have been proposed on the basis of epidemiologic evidence7,8,9,11,12 :

* One theory holds that the mechanical factors during intrauterine life are responsible for the higher incidence of plagiocephaly, developmental dysplasia of the hip, and scoliosis on the same side of the body.

* A second hypothesis suggests multifactorial causes, including predisposing genetic factors that are either facilitated or inhibited by external factors such as defective motor development or collagen disorders, joint laxity, and nursing posture of the infant.

* Other associations include older mothers from poorer families, breech presentation, and premature and male low-birth-weight babies.

Pathophysiology

Most of the curves in the spine develop during the first year of life, and strong correlation has been found between the nursing posture of the infant and development of the curve. It is less common in the United States than in Europe, where babies are nursed in the supine position. Infants have a natural tendency to turn toward the right side, and because of plasticity of the infant’s axial skeleton, this can lead to development of plagiocephaly, bat ear on the right side, and curvature of the spine toward the left side.11

Presentation

Infantile scoliosis usually is detected during the first year of life either by the parents or by the pediatrician during routine examination of the infant. Usually, a single, long, thoracic curve to the left is present; less often, a thoracic and lumbar double curve is noted. A child who is diagnosed with scoliosis requires a thorough clinical and radiologic examination to exclude any congenital, muscular, or neurologic causes.

Indications

There are 3 management options for infantile scoliosis: observation, orthosis, and operative. The decision when to use each of these is based on the rib-vertebral angle difference (RVAD), established by Mehta in 1972 (see Image 1).13 The RVAD is a useful guide in distinguishing between resolving and progressive idiopathic infantile scoliosis

RVAD (rib-vertebral angle difference) measurement...

RVAD (rib-vertebral angle difference) measurement at apical vertebra: RVAD = b-a (concave – convex side).


The rib-vertebrae angle is measured by (1) drawing a line perpendicular to the middle of the upper or lower border of the apical vertebrae of the curve and then (2) measuring the angle this line makes with medial extension of another line drawn from the mid point of the head to the mid point of the neck of the rib, just medial to the beginning of the shaft of the rib. The difference between the right and the left side (concave and the convex side) is the RVAD.

The apical vertebra is the vertebra at the curve of the apex. If there are the same number of vertebrae between the superior and the inferior end vertebrae, there will be 2 apical vertebrae.

For scoliosis curves with an RVAD of less than 20°, observation every 4-6 months is sufficient. If the RVAD is more than 20° or if it is not flexible clinically (ie, curve cannot be corrected even slightly with different postures, especially lateral bending), then it is considered to be progressive until proven otherwise.

Management with orthosis is necessary when the curve is considered to be progressive or if a compensatory curve has developed. Various types of orthosis are available for children younger than 3 years. The most commonly used orthoses are the hinged Risser jacket; the plaster spinal jacket (Cotrel EDF [elongation, derotation, flexion] type) applied under anesthesia; the Milwaukee brace; and the Boston brace. The brace should be used for 23.5 hours a day and should be removed only for exercises and swimming. It needs to be used until skeletal maturity is attained, because curves usually do not progress after skeletal maturity; however, curves may progress in spite of using a brace.14,15

Spinal deformity in scoliosis progresses during periods of peak growth velocity. The first spinal growth peak occurs at 2 years of age, and the second peak occurs during the prepubescent period.

Operation is usually an option only for children in older age groups (ie, around age 10 years), and segmental posterior wiring to 2 L-rods without fusion is preferable until combined posterior and anterior fusion can be done. These procedures, however, have been associated with complications in 50% of patients.

Because of advances in instrumentation, pedicle screw instrumentation can be performed for children with further growth potential. In these patients, a growing rod is used, which is associated with fewer complications than surgical fixation using L-rods. The disadvantage associated with the growing rod is that every 6 months the posterior aspect has to be opened to lengthen the rod, which increases the risk of infection; however, if the curve is severe or increases despite the use of orthosis, a short anterior and posterior fusion is recommended to prevent crankshaft phenomenon.

Relevant Anatomy

The spine is made up of 33 individual vertebrae that form a column. The spine is divided into 5 regions, starting from the top:

* Cervical – 7 vertebrae

* Thoracic – 12 vertebrae

* Lumbar – 5 vertebrae

* Sacrum – 5 vertebrae

* Coccyx – 4 vertebrae

The sacrum and coccyx are fused in the adult. The spine provides a protective function for the spinal cord; bears and distributes the weight of the body; provides an area for attachment of ligaments and muscles; and is the site for production of red blood cells. Together, all the vertebrae form a flexible structure providing mobility for the body to bend forward or sideward.

Each vertebra has a cushionlike fibrous structure called a disk, which acts like a shock absorber during movements of the spine. The disk is made up of a soft, jellylike central nucleus pulposus surrounded by a ring of fibrous tissue called an anulus, which is actually a strong ligament between 2 adjacent vertebrae.

Developmentally, the spine of the fetus is C-shaped, with concavity in the front (kyphotic) of the thoracic region; this is called the primary curve. Two secondary curves develop after birth, with concavity occurring anteriorly (lordosis); one of the secondary curves develops in the cervical region as the infant starts to hold up the neck, and the second curve develops in the lumbar region when the child starts to walk. Normally, there are no sideward (scoliosis) curves, so that the spine looks straight when viewed from behind or from the front.

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Cervical Disc Disease: Treatment & Medication

Cervical Disc Disease: Treatment & Medication

Rehabilitation Program

Physical Therapy

For most cervical disc disorders, studies support conservative treatment, such as the McKenzie approach and cervicothoracic stabilization programs, combined with aerobic conditioning.

The McKenzie system identifies 3 mechanical syndromes, as follow, that cause pain and compromise function:

* The postural syndrome provokes pain when normal soft tissues are loaded statically at end ROM; pathology need not be present. Treatment aims to correct posture.

* The dysfunction syndrome produces pain when the patient, upon attempting full movement, mechanically deforms contracted scarred soft tissue. Consequently, therapy involves stretching and remodeling of such contracted tissue.

* The derangement syndrome produces intermittent pain when certain movements or postures occur. Specifically, pain may become centralized or peripheralized because of theoretical activity-dependent displacement of intradiscal material. Therapy attempts to correct derangement by promoting activity that centralizes pain.

The McKenzie theory recognizes that although patients may demonstrate similar signs and symptoms, one movement (eg, cervical extension) nevertheless may help some patients and aggravate symptoms in others. Indeed, McKenzie therapy does not use only extension-biased exercise. Consequently, treatment individualization and patient education play key roles.

Cervicothoracic stabilization limits pain, maximizes function, and prevents further injury. Such stabilization includes cervical spine flexibility, postural training, and strengthening. This program emphasizes patient responsibility through active participation.

Restoring flexibility prevents further repetitive microtrauma from poor movement patterning. Pain-free ROM is determined by placing the cervical spine in positions that produce and relieve symptoms. Initially, stabilization commences within established pain-free ROM and then progresses outside this ROM as pain diminishes. Soft tissue or joint restriction inhibiting ROM is treated quickly. Anterior and posterior neck muscles are stretched. Indeed, such spine and soft-tissue mobilization, passive ROM, self-stretching, and correct posturing collectively restore ROM.

Postural training commences with the patient, supervised by a therapist, in front of a mirror. The patient performs various transfer maneuvers while maintaining a neutral spine (ie, correct posturing), with feedback from the mirror and the therapist. Patient goals include maintenance of neutral spine and demonstrating correct posture during daily activities.

These proprioceptive skills, implemented during strengthening exercises, facilitate stable, safe, and pain-free cervical posture during strenuous activity. Indeed, cervicothoracic stabilization requires strengthening and coordination of neck, shoulder, and scapular muscles. Cervical muscles include extensors, flexors, rectus capitis anterior, rectus capitis lateralis, longissimus cervicis, and longissimus capitis. Primary thoracic stabilizers include abdominals, lumbar paraspinal extensors, and latissimus dorsi. Scapular muscles include the middle and lower trapezius, serratus anterior, and rhomboids. Chest muscles include the pectoralis major and minor. Successful stabilization also requires the training of the lumbar spine and lower extremities, which provide a foundation for the cervicothoracic spine.

Stabilization exercises proceed systematically from simple to complex. Isometric and isotonic resistive exercises employ elastic bands, weight machines, and free weights. Such conditioning distributes forces away from the cervical spine. Exercise repetition ultimately encodes an engram that commands immediate, automatic cervicothoracic stabilization during everyday activity.

Butler’s therapy techniques treat radicular symptoms by mobilizing the involved nerve. First, the therapist identifies “adverse neural tension,” defined as pathologic mechanical and physiologic responses elicited from a nerve when its stretch properties and ROM are evaluated. Specifically, the therapist performs neurodynamic testing to evaluate a nerve’s mechanical properties (eg, its mobilization around neighboring intervertebral discs) and physiological characteristics (eg, its response to ischemia, inflammation). Having tested the nerve in question, the therapist may institute treatment consisting initially of passive mobilization to provide CNS input without inciting a stress response and neurogenic massage to reduce perineural swelling. Later, the therapist progresses to active neuromobilization, because, according to Butler, recovering nervous tissue (like other connective tissue) requires movement to promote healing and restoration of optimum mechanical properties.

Butler admits that limited evidence suggests that neurodynamic mobilization improves clinical outcomes. However, he believes that optimizing tissue health and cardiovascular fitness, as well as minimizing negative beliefs and environmental factors, can be beneficial.

Functional restoration programs assist patients disabled by chronic cervical pain overcome obstacles to recovery. Such obstacles include deconditioning, secondary gain, poor motivation, and psychopathology. An occupational or physical therapist, athletic trainer, or nurse instructs the patient in cervical anatomy, biomechanics, pathology, and ergonomics. Patients employ preventive measures in order to prohibit further injury during all daily activities. These medically directed interdisciplinary programs have been successful at enabling workers’ compensation patients to return to work. Furthermore, Wright and colleagues reported lower rates of recurrent injury, new surgery, and need for health care services for patients with chronic cervical pain who successfully completed functional restoration.

Medical Issues/Complications

An intervertebral disc compressing the spinal cord can provoke myelopathy with associated weakness, hyperreflexia, and neurogenic bowel and bladder dysfunction. Radiculopathy can manifest significant upper limb weakness or numbness. Intractable axial or radicular pain may result from cervical disc disorders.

Surgical Intervention

Studies indicate that cervical HNP with radiculopathy can be managed conservatively. Surgery is warranted when neurogenic bowel or bladder dysfunction, deteriorating neurologic function, or intractable radicular or discogenic neck pain exists. Specifically, cervical spine surgical outcomes are most favorable for radicular pain, spinal instability, progressive myelopathy, or upper extremity weakness. The literature has demonstrated favorable cervical spine fusion outcomes for chronic discogenic axial neck pain when the presurgical evaluation incorporated provocative cervical discography. Provocative discography identified the painful segment(s) and confirmed adjacent pain-free levels. Fusion can increase intradiscal pressure and other stress at adjacent unfused levels, thereby accelerating postsurgical spinal degeneration.12,13,14

A 2009 study sought to determine which factors are predictive of patient outcome following anterior discectomy and fusion.15 Surgical outcomes that developed over a 2-year period were examined in patients who were treated for recalcitrant single-level subaxial radiculopathy or myelopathy. The study’s results indicated that important prognostic factors include whether or not a patient is gainfully employed, has normal sensory function prior to surgery, has higher preoperative disability scores, and is involved in spine-related litigation.

Consultations

* Consultation with an internal medicine specialist is indicated when neck pain suggests an underlying systemic illness (eg, malignancy, infection, metabolic bone disease).

* Consider consultation with a rheumatologist when neck pain suggests a rheumatologic condition (eg, polymyalgia rheumatica).

* Consultation with a surgeon for cervical disc disorders is warranted for resulting neurogenic bowel/bladder dysfunction, deteriorating neurologic status (eg, myelopathy), segmental instability, and/or intractable radicular or discogenic pain.

Other Treatment

* Physical modalities should be used to reduce pain only in the acute phase. Once past the acute phase, modalities are used sparingly on an as-needed basis.

o Superficial heat modalities relax muscle and relieve soft-tissue pain.

o Conversely, deep-heating modalities (eg, ultrasonography) should be avoided in acute cervical radiculopathy, because they augment inflammation and, consequently, exacerbate radicular pain and nerve root injury.

* Cervical traction may relieve radicular pain from nerve root compression. Traction does not improve soft-tissue injury pain. Hot packs, massage, and/or electrical stimulation should be applied prior to traction to relieve pain and relax muscles.

o Traction regimens include heavy weight-intermittent or light weight-continuous. The neck is flexed 15-20 º (ie, not extended) during traction. In the cervical spine, approximately 10 lb of force is necessary to counter gravity and 25 lb of force is necessary to achieve separation of the posterior vertebral segments.

o Light weight-continuous home traction is cost effective and provides the patient with more autonomy.

o Pneumatic traction devices afford greater patient comfort and, consequently, increased compliance.

* A soft cervical collar is recommended only for acute soft-tissue neck injuries and for short periods of time (ie, not to exceed 3-4 days’ continuous use). Risks include limiting cervical ROM and losing neck strength if the collar is worn continuously for longer periods.

o When worn for radiculopathy caused by foraminal stenosis, the wide part of the collar is placed posteriorly and the thin part is placed anteriorly to promote neck flexion, discourage extension, and open the intervertebral foramina.

o Collars can be worn during certain activities, such as sleeping or driving, for longer periods.

o Although not commonly used, a Philadelphia collar can be worn at night to position the neck rigidly in flexion, thereby maintaining open foramina.

* Spinal manipulation and mobilization may restore normal ROM and decrease pain; however, no clear therapeutic mechanism of action is known. Some believe that zygapophysial joint adjustment improves afferent signals from mechanoreceptors to peripheral and central nervous systems.

o Normalization of afferent impulses improves muscle tone, decreases muscle guarding, and promotes more effective local tissue metabolism. These physiologic modifications subsequently improve ROM and pain reduction.

o Studies document short-term improvement in the acutely injured patient and in those with cervicogenic headache and radiculopathy secondary to disc herniation.

o No evidence exists that manipulation confers long-term benefit, improves chronic conditions, or alters the natural course of the disorder.

* Cervical epidural, spinal nerve (or root), Z-joint, and sympathetic injections serve diagnostic and therapeutic roles. (See images below and Images 4-5.) These procedures can be instrumental in determining the anatomic pain generator (eg, nerve root, facet) and providing aggressive, conservative treatment

.

Right C7 cervical transforaminal epidural steroid injection demonstrating epidural and radicular spread of radiologic contrast dye

.

Cervical epidural steroid injection at the C7-T1 interlaminar space.

* Therapeutic cervical epidural injections treat radicular pain, although some literature has demonstrated reduced axial pain as well.

o An anesthetic and corticosteroid mixture may be injected into the epidural space (interlaminar) or along the nerve root (transforaminal) after precise radiologic, contrast-enhanced fluoroscopic localization.16

o The anesthetic can relieve sympathetically mediated pain.

o The corticosteroid provides long-term relief if pain results from an intense inflammatory component.

o Such injections provide a pain-free window of opportunity for more aggressive physical therapy.

* Diagnostic selective spinal nerve or ventral ramus blocks inject a small anesthetic volume extraforaminally at a single spinal segment level (eg, C5 versus C6); consequently, they are more precise than the “gun shot” interlaminar approach in identifying the symptomatic nerve.

o Precise symptomatic nerve identification permits the physician to design a more focused treatment protocol.

o Patients record pain changes in a pain diary following the injection, to confirm diagnostic accuracy.

o A double injection paradigm previously reported in the literature for facet injections can provide information to the physician for use in determining a diagnosis of radicular pain and to help confirm the symptomatic nerve level. This paradigm identifies patients who have tested false-positive or may have a tendency to respond to a placebo, by determining whether, on separate injection days, they received short-term relief with a short-acting anesthetic (eg, lidocaine) and long-term relief with a long-acting anesthetic (eg, bupivacaine).

* Adverse effects include those from anesthesia, corticosteroids, and radiologic contrast dye.

o Blood clotting parameters should be drawn prior to injection in patients with suspected bleeding diathesis. Indeed, spinal cord compression could result if bleeding occurs in the presence of relative spinal stenosis (ie, midsagittal diameter less than 12 mm) in which little room exists to accommodate an epidural hematoma.

o Nonsteroidal anti-inflammatory drugs (NSAIDs), including aspirin, should be discontinued prior to the procedure in accordance with their half-life and hematologic profile.

o Other potential risks include seizure, vertebral artery spasm, infection, temporary quadriparesis from anesthetic, and respiratory arrest.

o One study, however, suggested that selective cervical nerve blocks carry low morbidity when performed under contrast-enhanced fluoroscopic guidance.

o In any event, proper patient monitoring and emergency equipment always should be present.

* Reports of serious CNS complications, including spinal cord injuries and strokes, following cervical transforaminal steroid injections have gained the attention of many practitioners. The mechanism of the injury is believed to be related to the introduction of particulate matter within the corticosteroid preparations, causing occlusion of a vessel.

o Hodges and colleagues described 2 case reports in which intrinsic spinal cord damage resulted from cervical epidural steroid injection despite fluoroscopic guidance; the patients, because of intravenous sedation, were unable to perceive and report pain and paresthesias from needle-induced spinal cord trauma during the procedure.17

o Furman et al demonstrated a relatively high incidence of entering the intravascular space with transforaminal epidural steroid injections.18 They also showed that attempting to use a flash of blood in the needle hub to predict intravascular compromise was 97% specific but only 45.9% sensitive. This article underscored the importance of using fluoroscopy and contrast dye to ensure proper placement of the therapeutic agents. Using a flash of blood in the hub without fluoroscopy cannot reliably predict intravascular compromise.

o Brouwers et al reported a fatal case of spinal cord infarction following a cervical transforaminal steroid injection.19

o Baker et al demonstrated that a radicular artery supplying the cervical spinal cord can be infiltrated by a transforaminal epidural steroid injection.20 In this report, prior to steroid injection for a left C6-C7, contrast was administered. Using digital subtraction technique, it was clear that a radicular artery filled with contrast; the procedure was aborted without adverse effects. This report revealed a potential access point for an injection-related spinal cord infarction.

o The potentially catastrophic complications that can follow a cervical transforaminal epidural steroid injection cannot be underestimated. While these procedures are perceived as posing less of a risk than surgery, they still carry substantial hazards. They should be performed by skilled practitioners and under fluoroscopic guidance. Baker et al further suggest the use of digital subtraction, because intravascular compromise may be missed on routine spot films.20

Medication

NSAIDs are first-line pharmacologic intervention for most cervical conditions. NSAIDs reduce pain at low doses and decrease inflammation at high doses. Patients require a therapeutic NSAID plasma level to achieve an anti-inflammatory effect. NSAIDs with once-a-day dosing improve compliance and increase the probability of achieving therapeutic levels. Controlling inflammation is paramount when treating cervical radiculopathy.

Aspirin rarely is recommended, because it binds irreversibly to cyclooxygenase (COX) and incites gastritis, requiring large doses to reach anti-inflammatory effect. Traditional NSAIDs provoke multiorgan toxicity, including peptic ulcer disease, renal insufficiency, and hepatic dysfunction. COX isomer type 2 (COX-2) NSAID inhibitors confer the same analgesic/anti-inflammatory benefits without multiorgan toxicity. All NSAIDs have a dose-related ceiling point for analgesia above which higher doses fail to provide additional pain relief. The same precautions should be observed with COX-2 NSAIDs, despite their reduced risk of organ toxicity.

Use muscle relaxants to potentiate the NSAID analgesic effect and not necessarily to control muscle spasm. Muscle relaxants primarily sedate by relaxing muscle with subsequent relaxation of the patient.

Oral corticosteroids treat inflammatory cervical radiculopathy. No documented case of avascular necrosis exists in the literature when the total prednisone dose or corticosteroid equivalent stayed under 550 mg. Some providers use a methylprednisolone dose pack (tapers from 24 to 0 mg over 7 days); however, concern exists regarding adequate dosing to treat radiculopathy. A prednisone dose schedule outlined below stays within the 550-mg limiting amount.

Tricyclic antidepressants (TCAs) decrease pain and reduce nonrestorative sleep. Side effects include dry mouth, constipation, and weight gain. Selective serotonin reuptake inhibitors (SSRIs), despite lacking side effects associated with TCAs, are inferior to TCAs in treating diabetic peripheral neuropathic pain, and their efficacy in relieving neck and back pain compared with that of other antidepressants remains unknown. Additional medications include membrane-stabilizing agents (eg, gabapentin, carbamazepine). Gabapentin has demonstrated efficacy in treating diabetic peripheral neuropathic pain. Other analgesics (acetaminophen, tramadol) provide pain relief without inflammation control.

Opioids may be prescribed orally, transdermally, rectally, or sublingually on a scheduled basis. Patients on opioids should sign a medication contract restricting them to a single physician and pharmacy, scheduled medication use, no unscheduled refills, and no sharing or selling medication. Patients with a previous history of alcoholism or other addiction who are prescribed opioids long term are at risk for dependence. Therefore, consider recommending cotreatment of these patients with a psychologist or other addiction specialist.

Lastly, many short-acting opioid preparations contain acetaminophen, which may be toxic in doses above 3 g per day. Consequently, patients should be counseled to avoid toxicity by avoiding other pharmaceuticals containing acetaminophen.

Corticosteroids

Used to treat inflammatory cervical radiculopathy. Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body’s immune response to diverse stimuli.

Prednisone (Deltasone, Orasone Sterapred)

Decreases inflammation by inhibiting polymorphonuclear leukocyte and fibroblast migration, stabilizing lysosomes, and decreasing capillary permeability.

Methylprednisolone dose pack (Solu-Medrol, Medrol, Depo-Medrol)

Decreases inflammation by inhibiting polymorphonuclear leukocyte and fibroblast migration, stabilizing lysosomes, and decreasing capillary permeability.

Anticonvulsants

Use of certain anti-epileptic drugs, such as the GABA analogue Neurontin (gabapentin), has proven helpful in some cases of neuropathic pain. Have central and peripheral anticholinergic effects, as well as sedative effects, and block the active reuptake of norepinephrine and serotonin. The multifactorial mechanism of analgesia could include improved sleep, altered perception of pain, and increase in pain threshold.

Gabapentin (Neurontin)

Has anticonvulsant properties and antineuralgic effects; however, exact mechanism of action is unknown. Structurally related to GABA but does not interact with GABA receptors.

Carbamazepine (Tegretol)

May reduce polysynaptic responses and block posttetanic potentiation. Inhibits nerve impulses by decreasing influx of sodium ions into cell membrane.

Analgesics

Pain control is essential to quality patient care. Analgesics ensure patient comfort and have sedating properties, which are beneficial for patients who experience pain.

Acetaminophen (Tylenol, Feverall, Aspirin Free Anacin)

DOC for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI disease, or who are taking oral anticoagulants.

Tramadol (Ultram)

Inhibits ascending pain pathways, altering perception of and response to pain. Inhibits also reuptake of norepinephrine and serotonin.

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Target hemoglobin levels in the correction of anemia of chronic illness in patients with chronic kidney and cardiovascular disease


Anemia of Chronic Disease and Renal Failure: Follow-up

Follow-up

Complications

Hypoxia

Hypoxia is the most potent stimulus to the production of erythropoietin by the kidneys. In the healthy individual, erythropoietin exerts its effects in the bone marrow to help in the production of RBCs, thereby improving oxygen concentration in the blood, relieving the hypoxia.

Another complication that commonly occurs in those with chronic kidney disease is that of secondary hyperparathyroidism and the development of renal osteodystrophy. In these patients, the bone marrow tends to be fibrotic and, hence, less responsive to the effects of erythropoietin.

Cardiorenal anemia syndrome

Silverberg described the “cardiorenal syndrome,” which refers to a vicious cycle, whereby decreased kidney function, as seen in chronic kidney disease, leads to decreased erythropoietin production and, thence, anemia.14

Anemia, if severe, leads to a compensatory LVH. Such compensatory LVH eventually leads to precipitation of congestive heart failure (CHF), which causes a decline in blood perfusion to the kidneys, resulting in further kidney damage. Levin et al estimated that for every 1-g decrease in hemoglobin concentration, there is an increased 6% risk of LVH in patients with chronic kidney disease.15 Foley et al estimated that such a 1-g decrease in hemoglobin concentration also translated into a 42% increase in left ventricular dilatation in patients with stage 5 chronic kidney disease.16

Cardiovascular disease

As an individual ages, the risk of death from cardiovascular disease also increases. The impact of anemia in cardiovascular disease and chronic kidney disease in this elderly population cannot be understated. Cardiovascular disease remains the most common cause of mortality in this patient population, much higher than in the general population.17 Anemia has been shown to be an independent risk factor for increased cardiovascular morbidity and mortality.

The Dialysis Outcomes Practice Pattern Study (DOPPS) involved several countries and showed that as hemoglobin concentrations decreased to <11 g/dL, there was a corresponding increase in the rates of hospitalization and mortality in patients with chronic kidney disease.18 Ofsthun et al analyzed the databases from Fresenius Medical Care of North America (FMCNA) (selection restricted to patients in the census for 6 consecutive months from July 1, 1998, through June 30, 2000) and showed that the longer it took for these patients with stage 5 chronic kidney resolve their hemoglobin concentrations from <11 g/dL, the more dramatic an increase in their mortality hazard ratio.19 The investigators further added that lower hemoglobin concentrations clearly correlated positively with adverse events in these patients.

In summary, one can derive that if hemoglobin levels are maintained at the recommended target goals, these translate into decreased LVH, decreased hospitalizations related to cardiovascular disease, and decreased mortality from cardiovascular disease. Aside from these findings, however, higher quality of life (QOL) scores are also obtained: less easy fatigability and fatigue symptoms, improved physical well-being and exercise tolerance, and improved functional well-being.

The next question is what are the appropriate target levels for the correction of anemia? Certainly, this issue has received much recognition as of late, in connection with recently published literature in which it was demonstrated that targeting higher hemoglobin levels may relate positively with higher rates of death and cardiovascular disease death, as well as positively with an increased risk of death, overall. Two of the trials relating to patients with cardiovascular disease will be discussed here.

Two landmark trials tried to address the controversial issue of the upper limit to target hemoglobin concentration, namely, the Cardiovascular Risk Reduction by Early Anemia Treatment with Epoetin Beta (CREATE)20 and the Correction of Hemoglobin and Outcomes in Renal Insufficiency (CHOIR)21 studies. As a result of the CREATE and CHOIR studies, in March 2007, the US Food and Drug Administration (FDA) issued a black box warning to the labeling of epoetin alfa (Procrit) and darbepoetin alfa (Aranesp) to emphasize that use of these ESAs may increase the risk of serious cardiovascular events and death when they are dosed to achieve a target hemoglobin of >12 g/dL.

This warning was again updated in November 2007, at which time, the FDA stated that “ESAs should be used to maintain a hemoglobin level between 10 g/dL to 12 g/dL. Maintaining higher hemoglobin levels in patients with chronic kidney failure increases the risk for death and for serious cardiovascular reactions such as stroke, heart attack or heart failure.”22

The FDA further recommends that hemoglobin be measured twice per week for 2-6 weeks after a dose adjustment, the purpose of which is to ascertain that the hemoglobin has had enough time to stabilize in response to the dose adjustment. Moreover, the FDA recommends withholding the dose of the ESA if the hemoglobin is >12 g/dL or increases by 1 g/dL over a 2-week period. The latter recommendation is in stark contrast to what has been done in most clinical practices until recently.

With these latest developments, the current clinical management of anemia in chronic kidney disease will certainly be significantly affected.

Likewise, the 2007 NKF update of the target hemoglobin recommendation stated that, although the lower limit of the target hemoglobin range remains 11 g/dL, the target range is 11–12 g/dL, and patients who have already or are currently receiving an ESA should maintain a hemoglobin target of less than or 13 g/dL

Related Keyword terms:

,hemoglobin goal in cardiac patients,goal hemoglobin in cardiac disease,heamoglobin level correction,heart failure hemoglobin goal,hemoglobin 1u,hemoglobin goal i heart disease,anemia hemoglobin goal cardiac disease,hemoglobin goals for anemia,hemoglobin heart problems goal,hgb goal for patient with cardiovascular disease

New studies on MRI and Rotator Cuff Injury

New studies on MRI and Rotator Cuff Injury

Author: Michael Tuite, MD,, Director, Musculoskeletal Division, University of Wisconsin Hospital and Medical School

Coauthor(s): Matthew F Sanford, MD, Fellow in Musculoskeletal Radiology, Department of Radiology, University of Wisconsin Medical School

Introduction

Background

Shoulder pain is a common complaint by patients during physician visits, and it can be due to a variety of causes. The major cause of shoulder pain in patients older than 40 years is rotator cuff impingement and tears. With the development of new arthroscopic techniques for treating rotator cuff disorders, magnetic resonance imaging (MRI) has played an increasingly important role as a noninvasive test for determining which patients may benefit from surgery


Normal intratendinous signal

Partial-thickness tear seen better on angled oblique sagittal views

.

Full-thickness tear

A French study by Lambert et al found the positive predictive value of 3.0T MRI to be 100% for the detection of rotator cuff tendon tears requiring surgery. In this prospective, follow-up study of 48 patients from 2005 through 2007, when arthroscopy was performed based on the MRI findings, there was no change in surgical management from that determined by MRI.1

In a meta-analysis of studies on MRI, MR arthrography, and ultrasonography for rotator cuff tears, de Jesus et al found MR arthrography to be more sensitive and specific than either MRI or ultrasonography for diagnosing both full-thickness and partial-thickness tears. MRI and ultrasonography showed no significant differences in sensitivity or specificity for full- or partial-thickness tears.2

Yoo et al found that preoperative MRI variables may help predict incomplete arthroscopic repair of large to massive rotator cuff tears. On preoperative MRIs of rotator cuff tears, the authors found that fatty degeneration index (FDI) values greater than 3 on sagittal oblique sections of the supraspinatus and values greater than 2 on sagittal oblique sections of the infraspinatus, with greater than 31 mm in coronal oblique tear distance (COTD) and 32 mm in sagittal oblique tear distance (SOTD), can help predict incomplete arthroscopic repair of the torn tendon.5,6

For excellent patient education resources, visit eMedicine’s Breaks, Fractures, and Dislocations Center. Also, see eMedicine’s patient education articles, Shoulder Dislocation, Shoulder Separation, and Magnetic Resonance Imaging (MRI).

Pathophysiology

The 2 potential etiologies of rotator cuff pain are mechanical causes, such as a flap of tendon that catches under the acromion, and biologic causes, such as synovitis. Although the rotator cuff is innervated, the subacromial bursa has 20 times the number of free nerve endings compared with the rotator cuff tendon. Compression of the subacromial bursa or catching of redundant synovium may cause much of the pain in patients with rotator cuff impingement.

Rotator Cuff Tears

Although rotator cuff impingement syndrome alone can be very painful, most surgeons will only operate on patients who have a rotator cuff tear (RCT). Cuff impingement is a clinical diagnosis, but identifying an associated nonmassive RCT during physical examination can be difficult; thus, the main role of MRI in these patients is to diagnose an RCT.

There are 3 main mechanisms involved in the development of RCTs:

* Extrinsic compression of the cuff

* Intrinsic tendon degeneration

* Muscle imbalance

Charles Neer was the first to popularize the theory that RCTs in older patients were primarily the result of extrinsic compression by the anterior acromion process, coracoacromial ligament, and acromioclavicular joint7 —the structures that make up the coracoacromial arch (see Image 1). Until then, most surgical procedures had only involved repairing the rotator cuff, without decompressing the roof of the arch; unfortunately, these procedures often failed to provide patients with long-term pain relief.

Shoulder. Hooked anterior acromion. Reprinted with permission from Tuite et al.

Neer’s technique of decompression of the coracoacromial arch along with repair of the rotator cuff was extremely successful, with a 90% success rate reported by most surgeons.8 However, surgeons have since modified portions of Neer’s original theory. For example, they now believe that most anterior subacromial spurs are coracoacromial ligament enthesophytes caused by chronic impingement. Yet over 3 decades after Neer’s study, decompression of the arch with rotator cuff repair remains the standard surgical treatment of rotator cuff impingement pain and RCTs.

Despite the surgical success of subacromial decompression, several authors have stressed the role of intrinsic tendon degeneration as the main etiology in the development of RCTs. Rathburn and Macnab demonstrated a zone of relative hypovascularity in the supraspinatus tendon approximately 1 cm from the insertion onto the greater tuberosity, which corresponded with the critical zone where most RCTs were noted to occur.9 Rothman and Parke had previously shown that degeneration occurred in these same hypovascular areas of the cuff.10

Lohr and Uhthoff11 and Clark and Harryman12 took this a step further, noting that the articular side of the rotator cuff has a sparse blood supply relative to the bursal surface. Multiple studies have revealed that most partial-thickness tears of the rotator cuff involve the articular surface, which would be unusual if chronic impingement from a hooked acromion or acromioclavicular joint osteophyte was the dominant cause of the tears. In addition, Nakajima et al13 and Lee et al14 showed that although the bursal surface bundles are able to elongate with a tensile load, the articular-surface fibers do not stretch and therefore tear more easily.

Kjellin et al reported that partial RCTs originated from areas of advanced degeneration and that there was no vascular ingrowth around the tears to indicate active inflammation.15 Many authors now believe that chronic tendon overload leads to degeneration in the hypovascular region of the rotator cuff because of poor healing and that these areas can eventually progress to RCT.

Burkhart has refined this theory with 2 observations: he noted that this hypovascular region does not involve the anterior and posterior edges of the supraspinatus tendon and that a “cable” of thicker, better-perfused tissue connects these edges more medially (see Image 2).16 This thickened tendon “cable” separates the musculotendinous junction from a crescent-shaped area of the lateral aspect of the supraspinatus tendon, where most RCTs occur.

Supraspinatus tendon. Reprinted with permission from Michael Tuite, MD..

Although the rotator cuff “cable” is seen histologically, it is not normally identifiable on MRIs. Several authors now believe that even full-thickness tears isolated to this crescent-shaped portion of the rotator cuff can solely be debrided and that patients will do well without significant loss of rotator cuff function.

Finally, muscle imbalance and scapular dyskinesis may also lead to impingement syndrome and RCTs. Strengthening of the humeral head depressors and of the muscles involved in reestablishing normal scapular motion is important in the nonoperative treatment of rotator cuff impingement syndrome.

Internal impingement

Another cause of RCTs is internal, or posterosuperior, impingement. During abduction and external rotation, the undersurface of the supraspinatus and infraspinatus tendons may normally lie between the greater tuberosity and the posterosuperior glenoid. In the throwing athlete, this normal physiologic phenomenon may result in pathologic internal impingement pain.

Internal impingement was first described in the early 1990s by arthroscopists. At arthroscopy, it was noted that articular-surface posterior cuff abnormalities, posterosuperior labral irregularity, and apposition of the humeral head and labrum existed in overhead-throwing athletes who had shoulder pain. The radiology literature has confirmed that the following findings are observed in athletes with clinically diagnosed internal impingement:

* Articular-surface tears at the posterior margin of the supraspinatus tendon and/or the anterior aspect of the infraspinatus tendon

* Posterosuperior labral tears

* Cystic change of the humeral head at the insertion of the rotator cuff

Identification of this constellation of findings by MRI should alert the radiologist and arthroscopist to the diagnosis of internal impingement.

The mechanism from which internal impingement arises is uncertain. One theory states that the pain and internal impingement tears are due to the repetitive impaction against the rotator cuff and labrum. Another theory purports that reactive thickening of the posteroinferior capsule results from shoulder deceleration during the throwing motion. This capsular thickening triggers a cascade of abnormal shoulder biomechanics that alters the contact point between the humerus and glenoid, thereby stretching the anterior capsule and resulting in laxity. This acquired pathologic laxity of the shoulder may alter stabilization of the humeral head within the glenoid during the late cocking or early acceleration phase of throwing.

Thickening of the posteroinferior capsule is thought to decrease the ability of the glenohumeral joint to internally rotate, with ensuing compensatory increased external rotation. The exaggerated external rotation and the altered humeral head contact point cause the rotator cuff and posterosuperior labrum to be subjected to shearing forces that result in the pathologic changes identified at arthroscopy and on MRI.

Tuite et al identified the fact that patients with clinically diagnosed internal impingement of the rotator cuff had thickening of the posteroinferior labrocapsular complex as well as a more shallow posterior capsular recess.17 These observations may prove useful in identifying patients who are at risk for internal impingement; diagnosis by MRI is helpful in this subset of patients, as nonoperative therapy with selective stretching of the posterior inferior labrum may decrease symptoms. In those in whom conservative measures fail, a posterior capsulotomy may be of benefit.

Frequency

United States

Shoulder pain is one of the most common reasons patients give for a physician visit, third only to headache and back pain.18 The incidence of rotator cuff disease increases as people age, although rotator cuff tears (RCTs) may not always be symptomatic. Sher et al obtained MRI scans for 46 asymptomatic individuals who were older than 60 years and found that 54% had either a partial-thickness or full-thickness RCT.19 Another study found that only 28% of all RCTs are painful and that many full-thickness tears are asymptomatic.20

Mortality/Morbidity

Although rotator cuff tears (RCTs) can be asymptomatic, these injuries can also be quite painful, with many affected patients describing the pain as one that awakens them at night. A functioning rotator cuff is also necessary for many activities of daily living, such as brushing one’s hair. There is significant job-related disability for individuals who have to lift or perform activities at the shoulder level.

Race

No race predilection has been observed.

Sex

A slightly higher incidence of rotator cuff tears in men has been reported in cadaver studies, but the difference is not significant.

Age

Most RCTs occur in older individuals, although they can also occur in younger individuals who are active in sports that involve overhead movements. Sher et al found the average age of patients with full-thickness tears was greater than that of those with partial-thickness tears.19 Because some patients with chronic RCTs and supraspinatus tendon atrophy are not surgical candidates, the true average age of those in the general population who have a full-thickness tear is probably higher as well.

Anatomy

The rotator cuff is made up of tendons from 4 muscles: the supraspinatus, infraspinatus, teres minor, and subscapularis. The tendon fibers of the supraspinatus, infraspinatus, and teres minor blend 1.5 cm from their lateral margins before they insert onto the greater tuberosity, with the bulk of the supraspinatus fibers inserting onto the superior facet of the greater tuberosity, whereas the infraspinatus and teres minor tendon fibers insert along the posterior aspect. The subscapularis tendon inserts independently onto the lesser tuberosity.

The rotator cuff interval, or anterior interval, separates the supraspinatus and subscapularis tendons. This gap between the tendons contains the coracohumeral ligament and superior glenohumeral ligament, as well as allows the long head of the biceps tendon to pass from the bicipital groove through the glenohumeral joint before inserting onto the superior glenoid.

The supraspinatus tendon is clinically the most important rotator cuff tendon because it is involved, either alone or in combination with 1 or more additional tendons, in 95% of cuff tears.21 The main tendon of the supraspinatus forms within the mid portion of the muscle, but as the supraspinatus courses laterally, the tendon lies progressively more anteriorly within the muscle. The supraspinatus tendon follows the curvature of the superior humeral head and curves caudally to insert onto the superior facet of the greater tuberosity. The supraspinatus tendon is approximately 9-11 mm thick at dissection but usually appears thinner (approximately 6-8 mm) on oblique coronal MRIs in patients who are positioned with the affected arm adducted and the rotator cuff under tension.

The rotator cuff tendon is unusual in the body in that it is not surrounded by either a synovial sheath or paratenon. Superficial to the supraspinatus tendon lies the subacromial-subdeltoid bursa, which may contain a thin layer of fluid in asymptomatic individuals. The superior surface of the rotator cuff is often termed the bursal surface. The inferior or more caudal surface of the cuff, termed the deep or articular surface, lies adjacent to the capsule and synovial lining of the glenohumeral joint.

Histologically, Clark and Harryman described 5 layers that make up the rotator cuff.12 The 2 layers that form the bursal one third of the tendon contain closely packed, well-organized tendon fibers, as does the layer forming the articular surface of the cuff. In the center of the rotator cuff are 2 layers that contain less-organized fibers mixed with loose connective tissue. On fat-suppressed, T2-weighted MRI, this central third of the tendon can have an intermediate signal intensity in normal individuals, whereas the outer portions of the cuff should show low signal intensity (see Image 3).

.

Normal intratendinous signal.

The histology of the rotator cuff contributes to one of the difficulties of rotator cuff MRI interpretation, the magic-angle effect or angular anisotropy. This effect is an MRI artifact in which normally low-signal structures that are made of organized collagen fibers appear as a higher signal intensity on images that are obtained with a short echo time (TE). The artifact occurs when the long axes of the collagen fibers are oriented at 55° to the main magnetic field. In most high-field MRI scanners, the main magnetic field is oriented along the direction of the bore (the central tunnel where the patient lies). The well-organized collagen fibers in the outer portions of the rotator cuff are organized longitudinally; therefore, these normally low-signal fibers have increased signal intensity on short-TE images as the fibers curve and become oriented at the magic angle.Unfortunately, this effect occurs in the region of the critical zone where RCTs and degenerative tendinopathy are prevalent. However, the magic angle’s high signal intensity diminishes with increasing TE; thus, it is not usually a problem on the fat-suppressed, fast spin-echo (FSE), T2-weighted MRIs most radiologists currently use to image the rotator cuff.

Presentation

The classic clinical presentation of rotator cuff impingement pain is a chronic ache in the lateral aspect of the shoulder, aggravated by attempts to abduct the arm and often worse at night. Patients typically have a severely painful arc from 60-120° of abduction and forward flexion. Weakness during abduction or forward flexion also may be present, particularly in patients who have a rotator cuff tear (RCT).22

Two physical examination techniques are commonly used to confirm rotator cuff impingement as the source of pain in the affected shoulder. The Neer, or impingement, test involves forward flexion of the arm, with the elbow extended; pain is elicited at maximal elevation. The Hawkins/Jobe test begins with the shoulder flexed forward at 90° and the elbow bent. The shoulder is then internally rotated and further abducted/flexed forward in an attempt to elicit pain. In addition, lidocaine injection into the subacromial bursa that results in decreased pain during the impingement tests is further evidence that impingement is the cause of the shoulder pain.

Preferred Examination

Conventional MRI with T2-weighted images in both the oblique coronal and oblique sagittal planes is the preferred technique for imaging the rotator cuff. Most authors have found that fat-suppressed, FSE, T2-weighted images are the most accurate for detecting rotator cuff tears (RCTs); a sensitivity of 84-100% and a specificity of at least 77-97% for full-thickness tears can be expected with this pulse sequence

Partial-thickness tear seen better on angled oblique sagittal views

Although most RCTs can be seen on oblique coronal images, Patten et al reported that oblique sagittal images provide approximately a 10% improvement in accuracy for detecting RCTs, although this was not statistically significant.37 The authors found that oblique sagittal images are especially helpful for identifying tears involving the anterior edge of the supraspinatus (see Images 4a-d).

Magnetic resonance arthrography

Some people prefer to perform either direct or indirect MR arthrography for imaging the rotator cuff. The advantage of direct MR arthrography relative to MRI is that it distends the joint, thus forcing the contrast agent into a small defect. T1-weighted images, which are faster to acquire and have a superior signal-to-noise ratio, can also be used instead of T2-weighted images. The disadvantages of direct MR arthrography are that it is mildly invasive and may require imaging guidance to place a needle into the glenohumeral joint capsule. In addition, bursal-surface partial-thickness tears are not directly opacified.

Several authors have reported that direct MR arthrography is close to 100% sensitive and specific for full-thickness and articular-surface partial-thickness RCTs.38 A full-thickness tear will demonstrate the gadolinium contrast solution extending first through a defect in the cuff and then into the subacromial-subdeltoid bursa (see Image 5). Articular-surface partial-thickness tears show a focal extension of the contrast solution into the substance of the tendon (see Image 6).

Full-thickness tear

.

Rim-rent or partial-thickness articular-surface tendon avulsion (PASTA) tear.

When performing direct MR arthrography, it is important to use fat-suppression to decrease the signal intensity of the peribursal fat plane around the subacromial-subdeltoid bursa; without fat-suppression, the fat plane can mimic the contrast agent and lead to a false interpretation of an RCT.

A French study by Lambert et al found the positive predictive value of 3.0T MRI to be 100% for the detection of rotator cuff tendon tears requiring surgery. In this prospective, follow-up study of 48 patients from 2005 through 2007, when arthroscopy was performed based on the MRI findings, there was no change in surgical management from that determined by MRI.1

In a meta-analysis of studies on MRI, MR arthrography, and ultrasonography for rotator cuff tears, de Jesus et al found MR arthrography to be more sensitive and specific than either MRI or ultrasonography for diagnosing both full-thickness and partial-thickness tears. MRI and ultrasonography showed no significant differences in sensitivity or specificity for full- or partial-thickness tears.2

Yoo et al found that preoperative MRI variables may help predict incomplete arthroscopic repair of large to massive rotator cuff tears. On preoperative MRIs of rotator cuff tears, the authors found that fatty degeneration index (FDI) values greater than 3 on sagittal oblique sections of the supraspinatus and values greater than 2 on sagittal oblique sections of the infraspinatus, with greater than 31 mm in coronal oblique tear distance (COTD) and 32 mm in sagittal oblique tear distance (SOTD), can help predict incomplete arthroscopic repair of the torn tendon.5,6

Indirect MR arthrography requires only an intravenous (IV) injection, but this modality has a disadvantage in that it does not distend the joint. As in direct MR arthrography, short scanning time T1-weighted images can be used instead of T2-weighted images. Several authors have shown that compared with conventional MRIs of the rotator cuff, RCTs are better characterized on indirect MR arthrography and there is better correlation with surgical findings. One study reported that 2 radiologists improved their accuracy for detecting RCTs from 67% and 62% with conventional MRI to 92% and 96%, respectively, with indirect MR arthrography.39 Again, use of fat suppression is important, but exercising the joint does not appear to improve accuracy.

Despite these studies, MR arthrography has not been as widely accepted for evaluating the rotator cuff as it has been for imaging the glenoid labrum. Direct MR arthrography does improve the depiction of posterior articular-surface partial-thickness tears that are observed in overhead-throwing athletes, particularly if the shoulder is scanned in abduction and external rotation. However, most authors have found that fat-suppressed, FSE, T2-weighted images obtained with a quality shoulder coil are fairly accurate for most RCTs and that conventional MRI is adequate for routine imaging of the rotator cuff.

Conventional arthrography was the traditional technique for detecting RCTs. However, arthrography itself does not demonstrate bursal-sided, partial-thickness tears, and it may be difficult at times to determine the size of a tear using this modality. With improvements in computed tomography (CT) scanners, oblique coronal reformatted CT arthrogram images can provide excellent images of the rotator cuff in patients who are unable to undergo an MRI.

Limitations of Techniques

MRI is contraindicated in patients who have a cardiac pacemaker, ferromagnetic foreign bodies (particularly in the orbit), and some cochlear implants. Some patients are extremely claustrophobic in high-field-strength MRI scanners, although many of these patients can be scanned in open MRI scanners after administration of a mild sedative.

MR arthrography is mildly invasive, and because the off-label use of gadolinium is not currently approved by the US Food and Drug Administration (FDA) for intra-articular injection, it may require written, informed patient consent. Imaging is also usually necessary to correctly position the arthrogram needle within the joint capsule. Fluoroscopy is the most common method of imaging guidance, but needle placement also can be performed under CT scanning, by ultrasound, or within the MRI scanner. Conventional arthrography is also mildly invasive and has the limitation of not being a tomographic technique.

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.

NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness

Related Keyword terms:

,rotator cuff mri images,rotator cuff mri,degenerative shoulder cuff operation magic eye,MRI pictures of torn rotator cuff,mri of acromial impingement,diagram showing a bursal surface tear of the rotator cuff,partial rotator cuff shoulder injury magnetic resonance imaging,rotator cuff cable and crescent,torn rotator cuff mri image,mri with contrast arm

The need for IL-17 testing in hyper-IgE syndrome

The need for IL-17 testing in hyper-IgE syndrome

Delayed-type Hypersensitivity: Differential Diagnoses & Workup

Differential Diagnoses

B-Cell and T-Cell Combined Disorders

Measles

Chromosomal Breakage Syndromes

Severe Combined Immunodeficiency

DiGeorge Syndrome

Tuberculosis

Hodgkin Disease

Wiskott-Aldrich Syndrome

Human Immunodeficiency Virus Infection

Other Problems to Be Considered

Consider primary T-cell immunodeficiency, including severe combined immunodeficiency (SCID), when anergy is present. Other well-recognized primary immunodeficiency diseases with anergy include Wiskott-Aldrich syndrome, DiGeorge syndrome, ataxia telangiectasia, and other chromosomal breakage disorders.

Exclude malnutrition and immunosuppression with corticosteroids and other drugs. Certain malignancies, such as Hodgkin disease, are associated with anergy. Consider rheumatologic disease, especially systemic lupus erythematosus as a cause of anergy in specific clinical situations.

Mutations that effect responses to interferon (IFN)-g or its production include IFNGR1, IFNGR2, STAT-1, IL12P4, and IL12RB1. As a result, these patients may manifest altered (often excessive) delayed-type hypersensitivity (DTH) skin test reactivity.

In patients with STAT3 mutations, as seen in patients with autosomal dominant hyper immunoglobulin E (IgE) syndrome, delayed-type hypersensitivity responses may be attenuated due to impaired Th17 cell development.

Workup

Laboratory Studies

* Characteristics of the antigens determine the delayed-type hypersensitivity (DTH) skin test reactivity. Conjugation of the antigen to lipids facilitates the delayed-type hypersensitivity reaction. This explains the consistent response to mycobacteria in which antigens are isolated from the lipid cell wall. Size, valence, chemical composition, and dose are additional factors that are relevant to immunogenicity. Repetitive testing with the same antigen can cause an immediate immunoglobulin E (IgE)-mediated response and may diminish the delayed-type hypersensitivity skin test reactivity. High doses of antigens that induce predominant Th2 responses, such as in miliary tuberculosis, abrogate the delayed-type hypersensitivity responses by a negative feedback mechanism that suppresses Th1 responses.

o By convention, the antigens used for delayed-type hypersensitivity skin testing are injected intradermally into the volar surface of the forearm with a volume of 0.1 mL each. Erythema and induration are measured at 24, 48, and 72 hours. A reaction at 24 hours does not represent delayed-type hypersensitivity induced by cell-mediated immunity (CMI), or type IV reactivity. The Food and Drug Administration (FDA) –approved antigens for delayed-type hypersensitivity skin testing are limited to PPD of M tuberculosis and Candida.

o Conventionally, children are tested with Candida and Dermatophytin in a 1:10 or 1:100 dilution and tested with tetanus in a 1:10 or 1:100 dilution of the diphtheria-tetanus (DT) vaccine. The higher dilution is used when the child has undergone a significant infection or unusually frequent immunization respectively.

o Adults are initially tested with the 1:100 concentrations of these antigens.

o When interpreting delayed-type hypersensitivity skin testing, whether adequate exposure to the antigens has taken place prior to the procedure must be considered. A vigorous immune response to one antigen, such as in measles infection, leads to the abrogation of other delayed-type hypersensitivity responses, for example, to purified protein derivative (PPD) even though the patient is also infected with tuberculosis.

o Antigens that are poorly immunogenic in children and in some adults include mumps (no longer on the US market) and Trichophyton. Dinitrochlorobenzene (DNCB) and dinitrofluorobenzene (DNFB) have been superseded by in vitro assessments of cell-mediated immunity because of the risk of local tissue necrosis.

* When an absent delayed-type hypersensitivity reaction is noted, screening tests for a T-cell disorder should include an absolute lymphocyte count and a chest radiograph to detect the thymus. Cell surface marker analysis of peripheral mononuclear cells by flow cytometry and in vitro lymphocyte proliferation responses against mitogens (polyclonal stimulants) and specific antigens are then performed.

* Contact sensitivity to poison ivy and nickel is determined clinically; skin testing is not considered necessary.

* Adverse drug reactions to antibiotics, phenytoin, and carbamazepine may involve nonimmune or immune-mediated mechanisms. The clinical setting of a reaction at 3 days or later with manifestation of a fixed rash with induration is more suspicious of involvement of a delayed-type hypersensitivity response.

Imaging Studies

* A chest radiograph to determine whether the thymus is present is an appropriate screening test for T-cell disorders only in the newborn; however, the thymus may involute in stressed infants in the context of overwhelming infection or severe congenital cardiac disease.

Other Tests

* When delayed-type hypersensitivity is absent and a T-cell disorder is suspected, assess in vitro lymphocyte proliferation responses against polyclonal stimulants such as mitogens (eg, phytohemagglutinins [PHA], concanavalin A [conA], pokeweed mitogen [PWM]) and specific antigens (eg, Candida, tetanus). Measurement of production of interferon (IFN)-γ, tumor necrosis factor (TNF)-α, and interleukin (IL)-12 in response to various stimulants can be helpful for screening mutations in IFNGR1, IFNGR2, STAT-1, IL12P40, or ILRB1 when such mutations are suspected. Low levels of one or more of these cytokines increase the likelihood of these mutations.

* In patients with severe eczema, recurrent skin abscesses, elevated IgE, and history of frequent bone fractures, assessment of IL-17 production may be helpful. This is because patients with autosomal dominant hyper IgE syndrome have impaired development of Th17 cells, which is a major cellular source of IL-17.8

* Cell surface markers for monocytes, T-cells (CD4, CD8, CD28, TCR a/b, TCR g/d), and activated T cells (CD25, HLA-DR, and CD5) are reported to be normal in IFNGR1, IL12P40,STAT1, IL12RB1, STAT3 mutations. In profound primary T-cell deficiencies such as severe combined immunodeficiency (SCID), the pattern of cell surface marker expression of lymphocyte and natural killer (NK) cells may identify the type of T-cell defect in conjunction with the clinical manifestations.

* Mutational analysis for IFNGR1, IFNGR2, STAT-1,STAT-3, IL12P40, and IL12RB1 is available in specific research laboratories.

* Additional genes that control downstream immune responses initiated by IFN-γ in the delayed-type hypersensitivity response are recognized; IFNGR2 does not bind IFN-γ but is needed for the activation of STAT-1 and its translocation to the nucleus.

Procedures

* When disseminated bacille Calmette-Guérin (BCG) or nontuberculosis mycobacteria (NTM) is suspected, perform biopsy of infected sites in order to examine granuloma formation and detect acid-fast mycobacteria.

* Tissue culture to detect mycobacteria is also indicated when disseminated BCG or NTM is suspected.

Histologic Findings

* Granuloma formation in an intact delayed-type hypersensitivity response shows predominant infiltrates of activated macrophages and lymphocytes that can be identified as CD4+ T cells by immunohistochemical staining.

* When NTM infection is present, multinucleated giant cells formed by fused activated macrophages are observed in the immunocompetent host.

* In the patient with a T-cell defect, the formed granuloma lacks CD4+ T cells and these giant cells (due to ineffective macrophage activation by T cells). Instead, granulomatous lesions are characterized by infiltrate of polymorphonuclear cells, vacuolated cells, and macrophages.

* Mycobacteria may be present in abundance but are not frequently stained, although they are isolated by culture techniques.

Related Keyword terms:

,hyper ige syndrome,lymphocyte vacuolation Hyper IgE syndrome,hyper ige and il17 testing,lymphocyte vacoulation test,immunosuppressive for hyper ige,il17 acne ppt,il-17 granuloma formation,hyper ige syndrome screening test,hyper ige syndrome ppt,hyPER iGe SYNDROME POWERPOINT PRESENTATION

Maternal-fetal surgical treatment of congenital diaphragmatic hernias?

Maternal-fetal surgical treatment of congenital diaphragmatic hernias?

Diaphragmatic Hernias, Congenital: Treatment

Author: Daniel S Schwartz, MD, FACS, Assistant Clinical Professor of Cardiothoracic Surgery, Mount Sinai School of Medicine; Chief of Thoracic Surgery, Huntington Hospital

Coauthor(s): Jason M Johnson, DO, General and Laparoscopic Surgeon, Department of General Surgery, William Beaumont Army Medical Center; Sidney R Steinberg, MD, FACS, Program Director, Department of General Surgery, Spartanburg Regional Healthcare System; Consulting Surgeon, Department of Surgery, WG Hefner Veterans Affairs Medical Center

Treatment

Medical Therapy

Resuscitation with ventilatory support is of prime importance in patients born with CDH. The trend over the past few years has been a switch from conventional mechanical ventilation to high-frequency oscillatory ventilation (HFOV). HFOV serves to minimize airway pressure and, in conjunction with permissive hypercapnia, HFOV helps those with CDH suffer less traumatic lung injury and fewer long-term complications. Mortality has been shown to decrease from 49-20% when HFOV is used early in the treatment course.

Extracorporeal membrane oxygenation (ECMO) has been shown to significantly decrease the mortality of CDH but is currently reserved for individuals whose condition fails to improve with both HFOV and conventional mechanical ventilation. The decision to utilize ECMO is made early in the disease process, usually within 24 hours of birth.

With the addition of HFOV, more reports exist of decreased morbidity and mortality with early surgical intervention. However, this practice is still rather controversial. The classical teaching is that patients need to be stabilized (often with the use of ECMO) and that repair be delayed until the infant is better prepared to survive the operation.12

Surgical Therapy

* Congenital (perinatal period): Significant issues remain, and many questions are unanswered, but the possibility of maternal-fetal surgical treatment of congenital diaphragmatic hernia is on the horizon.13,14

* Congenital (neonatal period): A subcostal incision is used. An attempt is made to repair the hernia with a primary repair; however, a prosthetic material such at ePTFE or polypropylene must sometimes be used.

* Congenital (latent): Approach is through thoracotomy or laparotomy. Most surgeons approach with laparotomy so that abdominal contents can be inspected adequately. Minimally invasive approaches are currently being explored via video-assisted thoracoscopic or laparoscopic approach.

Intraoperative Details

* Congenital (neonatal period): Reduce the abdominal contents, then approximate the edges of the diaphragm with nonabsorbable suture. If the defect is large or the repair is being made while the patient is on ECMO, prosthetic mesh is used.

* Congenital (latent): This typically is repaired with prosthetic mesh in children and direct suture technique in adults with nonabsorbable suture material. Minimally invasive repair is being explored at a number of institutions.

* Traumatic rupture:15,16,17 The surgical approach depends on the timing of the diagnosis with the surgical intervention.14

Follow-up

Once an anatomic defect has been corrected, periodically assessing pulmonary function and obtaining chest radiographs is important. Although spontaneous recurrence of a repaired diaphragmatic hernia is low, small defects in the repair site have been reported, so surveillance is essential.

Complications

* Congenital defects: Remembering that an increased risk of concomitant congenital defects in an infant with CDH is extremely important. If a diaphragmatic hernia is diagnosed in utero, have a high suspicion for concomitant abnormalities. Always remember that the infants with CDH have an increased risk of postnatal respiratory failure, incarceration or strangulation of bowel, and hypoplasia of lung.

* Traumatic or adult repaired CDH: Recurrence of the hernia is possible; thus, follow-up is important with chest radiograph.

Related Keyword terms:

,fetal surgery for congenital diaphragmatic hernia s

Middle Ear, Cholesteatoma

Middle Ear, Cholesteatoma

Author: Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, University of Texas School of Human Development

Introduction

Cholesteatomas have been recognized for decades as a destructive lesion of the skull base that can erode and destroy important structures within the temporal bone. Its potential for causing central nervous system complications (eg, brain abscess, meningitis) makes it a potentially fatal lesion.

Middle ear cholesteatoma. Attic cholesteatoma. This is a typical primary acquired cholesteatoma in its earliest stages.

.

History of the Procedure

Cholesteatomas were first described in 1829 by Cruveilhier, but Muller first named them in 1858. Throughout the early half of the 20th century, cholesteatomas were managed by exteriorization. The mastoid air cells were exenterated, the posterior external auditory canal removed, and the ear canal opening into the resulting cavity enlarged to ensure adequate air exchange and to make visual inspection simple.

In the 1950s and 1960s, a new approach was promulgated by William and Howard House’s Otologic Medical Group (currently the House Ear Clinic). The surgical anatomy of the facial recess was described and clarified by William House, MD, the great pioneering otologist of the 20th century. Operating through the facial recess permitted access to the middle ear through the mastoid without removing the posterior canal wall.

By operating through the facial recess, cholesteatoma could be removed without taking down the posterior canal wall. Over time, more and more surgeons attempted to leave the basic underlying anatomic structure of the ear and temporal bone intact by preserving the canal wall. These aggressive attempts to conserve the normal anatomy of the ear created great controversy. Surgeons tended to align themselves with either the old canal-wall-down or with the new canal-wall-up philosophies.

Over the last couple of decades, most otologic surgeons have migrated to an intermediate position. Most otologic surgeons in the United States now perform both techniques, selecting one or the other of these operations depending on the individual circumstances of a particular patient.

Problem

A cholesteatoma consists of squamous epithelium that is trapped within the skull base. Squamous epithelium trapped within the temporal bone, middle ear, or mastoid can expand only at the expense of the bone that surrounds and contains it. Consequently, the complications associated with a growing cholesteatoma include injury to any of the structures normally found within the temporal bone. Occasionally, cholesteatomas escape the confines of the temporal bone and skull base. Extratemporal complications may occur in the neck, central nervous system, or both. Cholesteatomas sometimes become large enough to distort normal brain and produce brain dysfunction from mass effects.

Bony erosion occurs by 2 principal mechanisms. First, pressure effects produce bony remodeling, as occurs normally throughout the entire skeleton when pressure is applied consistently over time. Second, enzymatic activity at the margin of the cholesteatoma enhances osteoclastic activity, which greatly increases the speed of bone resorption. The level of these osteolytic enzymes appears to increase when a cholesteatoma becomes infected.1

Frequency

The incidence of cholesteatomas is unknown, but it is a relatively common reason for otologic surgery (approximately weekly in tertiary otologic practices). Death from intracranial complications of a cholesteatoma is now uncommon, which is attributable to earlier recognition, timely surgical intervention, and supportive antibiotic therapy. Cholesteatomas remain a relatively common cause of permanent, moderate conductive hearing loss in children and adults.

Etiology

Generally, 3 separate types of cholesteatomas are identified based on different etiologies: congenital, primary acquired, and secondary acquired. The evolution of these 3 different types of cholesteatomas is described in the section below on pathophysiology.

Pathophysiology

Congenital

Congenital cholesteatomas arise as a consequence of squamous epithelium trapped within the temporal bone during embryogenesis. The typical congenital cholesteatoma is found in the anterior mesotympanum or in the perieustachian tube area. They are identified most commonly in early childhood (6 mo to 5 y).

As they expand, they can obstruct the eustachian tube and produce chronic middle ear fluid and conductive hearing loss. They can also expand posteriorly to encase the ossicular chain and, by this mechanism, produce conductive hearing loss. Unlike other forms of cholesteatoma, congenital cholesteatomas are usually identified most commonly behind an intact and normal-appearing tympanic membrane. The child often has no history of recurrent suppurative ear disease, previous otologic surgery, or tympanic membrane perforation.2

Primary acquired

Primary acquired cholesteatomas arise as the result of tympanic membrane retraction. The classic primary acquired cholesteatoma develops from progressively deeper medial retraction of the pars flaccida into the epitympanum. As this process continues, the lateral wall of the epitympanum (called the scutum) is slowly eroded, producing a defect in the lateral wall of the epitympanum that slowly expands. The tympanic membrane continues retracting medially until it passes over the heads of the ossicles and into the posterior epitympanum. Ossicular destruction is common. If the cholesteatoma pokes posteriorly into the aditus ad antrum and the mastoid itself, erosion of the tegmen mastoideum, with exposure of the dura and/or erosion of the lateral semicircular canal with deafness and vertigo, may result.3

A second type of primary acquired cholesteatomas arises when the posterior quadrant of the tympanic membrane is retracted into the posterior middle ear. The drum initially adheres to the long process of the incus. As retraction continues medially and posteriorly, squamous epithelium envelops the superstructure of the stapes and retracts into the sinus tympani. Primary cholesteatomas arising from the posterior tympanic membrane are likely to produce facial nerve exposure (and occasionally paralysis) and destruction of the stapedial superstructure. Surgical removal from the sinus tympani may be extremely challenging.

Secondary acquired

Secondary acquired cholesteatomas occur as a direct consequence of some type of injury to the tympanic membrane. This injury can be a perforation that has arisen as a result of acute otitis media or trauma, or it may be due to surgical manipulation of the drum. A procedure as simple as the insertion of tympanostomy tubes could implant squamous epithelium into the middle ear, ultimately producing a cholesteatoma. Posterior marginal perforations are the most likely to result in cholesteatoma formation. Although central perforations are considered unlikely to produce cholesteatomas, central perforations occasionally result in cholesteatoma formation. Any deep retraction pocket can result in cholesteatoma formation if the retraction pocket becomes deep enough to trap desquamated epithelium.4

Presentation

The hallmark symptom of a cholesteatoma is painless otorrhea, either unremitting or frequently recurrent. When the cholesteatoma becomes infected, the infection may be extremely difficult to eradicate. Because the cholesteatoma has no blood supply, systemic antibiotics cannot be delivered to the center of the cholesteatoma. Topical antibiotics often surround a cholesteatoma, suppress infection, and penetrate a few millimeters toward its center; however, a large, infected cholesteatoma is resistant to any type of antimicrobial therapy. Consequently, otorrhea either persists or recurs, despite frequent and aggressive treatment with antibiotics.

Hearing loss is also a common symptom of cholesteatomas. Large cholesteatomas fill the middle ear space with desquamated epithelium, with or without associated mucopurulent discharge. Ossicular damage is frequently present and can produce or magnify a conductive loss.

Dizziness is a relatively uncommon symptom of cholesteatomas, but it does occur if bony erosion produces a labyrinthine fistula or if the cholesteatoma is lying directly on the footplate of the stapes. Dizziness is a worrisome symptom because it may presage the development of more serious complications.

Upon physical examination, the most common sign of a cholesteatoma is drainage and granulation tissue in the ear canal and middle ear unresponsive to antimicrobial therapy. A tympanic membrane perforation is present in more than 90% of cases. Congenital cholesteatomas are an exception and the drum often remains intact until the middle ear component is quite large. Occasionally, a cholesteatoma that is produced by surgical implantation of squamous epithelium manifests prior to disruption of the tympanic membrane, but even in such cases, the cholesteatoma eventually produces a tympanic membrane perforation.5

Quite frequently, the only finding upon physical examination is a canal filled with mucopus and granulation tissue. Sometimes eliminating the infection and resolving the granulation tissue with either systemic antibiotics or ototopical antibiotic drops is impossible. When ototopical therapy is successful, a deep tympanic retraction pocket may be seen in the pars flaccida or posterior quadrant.

Very seldom is a cholesteatoma first identified based on one of its complications; however, this occasionally happens in children. An infection associated with the cholesteatoma can erupt through the inferior mastoid cortex and manifest as an abscess in the neck. Occasionally, cholesteatomas first manifest with the signs and symptoms of central nervous complications: sigmoid sinus thrombosis, epidural abscess, or meningitis.

Indications

Virtually all cholesteatomas should be excised. Occasional exceptions include the patient whose general health is so poor that it makes a surgical procedure too risky. Some patients who have cholesteatomas in their only hearing ear are, with good reason, reluctant to undergo surgery. The risks of profound hearing loss from surgical removal are generally less than the risk associated with leaving the cholesteatoma in situ.

Contraindications

The only absolute contraindications to the surgical removal of cholesteatomas are medical in nature. Some individuals have health problems of such gravity as to make the risk of surgical intervention unacceptably high.

Absence of hearing in the contralateral ear is a relative contraindication to surgery. Frequently, the cholesteatoma presents a greater risk to residual hearing than surgery, and, more often than not, surgical removal is the management option of choice even when the cholesteatoma is in the only hearing ear.

Related Keyword terms:

,middle ear cholesteatoma,CHOLESTEATOMA PATHOPHYSIOLOGY,nursing interventions cholesteatoma,cholesteatoma nursing plan,cholesteatoma nursing interventions,marginal perforation cholesteatoma,facial recess anatomy,facial nerve recess howard house,nursing diagnosis for ear surgery,antibiotic resistance cholesteatoma

UpToDate 17.1 for PC & PDA,April-2009,Full DVD

UpToDate 17.1 for PC & PDA,April-2009,Full DVD

مجانا المرجع رقم واحد في الولايات المتحدة و العالم الغربي:
لأطباء الداخلية و الأطفال و الطوارىء و النسائية و كافة التخصصات
حيث يقدم أحدث المعلومات و العلاجات و خلاصات الأبحاث و التجارب
على طبق من ذهب
سواء للطبيب أو المريض
و هو برنامج كامل مكمل مع الكراك

و تاريخ صدوره هو 4-2009

و هذا المرجع يصدر أصدار محدث منه كل أربعة أشهر سنويا
حمل ,تعلم و كن خير معين لكل محتاج

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Fused-Segment Studies in Klippel-Feil Syndrome

Fused-Segment Studies in Klippel-Feil Syndrome

Klippel-Feil Syndrome

Author: J Andy Sullivan, MD, Clinical Professor of Pediatric Orthopedics, Department of Orthopedic Surgery, University of Oklahoma College of Medicine

Introduction

In 1912, Maurice Klippel and Andre Feil independently provided the first descriptions of Klippel-Feil syndrome. They described patients who had a short, webbed neck; decreased range of motion (ROM) in the cervical spine; and a low hairline. Feil subsequently classified the syndrome into 3 categories:

* Type I – a massive fusion of the cervical spine

* Type II – the fusion of 1 or 2 vertebrae

* Type III – the presence of thoracic and lumbar spine anomalies in association with type I or type II Klippel-Feil syndrome

In a series of articles, Samartzis and colleagues suggested a new classification system.1,2 In this classification system, type I patients have a single-level fusion; type II patients have multiple, noncontiguous fused segments; and type III patients have multiple, contiguous fused segments. Using their system, the investigators reviewed a series of patients to clarify prognosis


Posterior photo of a patient with Klippel-Feil syndrome and an anomaly of the occipitocervical junction. The image shows an elevated left shoulder due to a Sprengel anomaly; a short, webbed neck; and a low hairline.

This patient has Klippel-Feil syndrome and an anomaly of the occipitocervical junction. The patient’s flexion and extension after the occipitocervical fusion is demonstrated. His rotation was very limited.

Flexion of the cervical spine in a patient who had an occipitocervical fusion.

This photo demonstrates synkinesia. As the patient attempts to oppose the thumb and finger of the right hand, the same movement occurs involuntarily in the left.

.

Patients with Klippel-Feil syndrome usually present with the disease during childhood, but may present later in life. The challenge to the clinician is to recognize the associated anomalies that can occur with Klippel-Feil syndrome and to perform the appropriate workup for diagnosis.

Frequency

The true incidence of Klippel-Feil syndrome is unknown. No one has ever studied a cross-section of healthy people to determine the true incidence.

The incidence of Klippel-Feil syndrome has been investigated in 2 studies, using 2 different means. Gjorup and Gjorup reviewed all of the radiographic cervical spine films from a single hospital in Copenhagen.3 From these films, they determined an incidence of 0.2 cases per 1000 people. Brown and colleagues reviewed 1400 skeletons from the Terry collection, which at that time was located at the Washington University School of Medicine.4 They found an incidence of 0.71%.

Etiology

The etiology of Klippel-Feil syndrome and its associated conditions is unknown. The syndrome can present with a variety of other clinical syndromes, including fetal alcohol syndrome, Goldenhar syndrome, and anomalies of the extremities.5,6,7 Gunderson suggested that it is a genetic condition, while Gray found a low incidence of inheritance.8,9 Others have considered Klippel-Feil syndrome to be some type of global fetal insult, which could explain the other associated conditions. Some have considered it to be caused by vascular disruption.10,11

Presentation

Clinical presentation is varied because of the different associated syndromes and anomalies that can occur in patients with Klippel-Feil syndrome. A complete history and careful physical examination may reveal some associated anomalies. From an orthopedic standpoint, most of the workup involves imaging (see Workup, Imaging Studies).

Klippel-Feil syndrome is detected throughout life, often as an incidental finding. Patients with upper cervical spine involvement tend to present at an earlier age than those whose involvement is lower in the cervical spine. Most patients present with a short neck and a decreased cervical ROM, with a low hairline occurring in 40-50% of patients. Decreased ROM is the most frequent clinical finding. Rotational loss usually is more pronounced than is the loss of flexion and extension.

Other patients present with torticollis or facial asymmetry. Neurologic problems may develop in 20% of patients. Rouvreau found that 5 of 19 patients with Klippel-Feil syndrome had neurologic involvement; of these 5 patients, 2 had neurologic problems resulting from hypermobility at 1 level.12 Occipitocervical abnormalities were the most common cause of neurologic problems Some patients present with pain.

An anomaly of the occipitocervical junction in a patient with Klippel-Feil syndrome. The anomaly was unstable and was fused.

Posterior photo of a patient with Klippel-Feil syndrome and an anomaly of the occipitocervical junction. The image shows an elevated left shoulder due to a Sprengel anomaly; a short, webbed neck; and a low hairline

This patient has Klippel-Feil syndrome and an anomaly of the occipitocervical junction. The patient’s flexion and extension after the occipitocervical fusion is demonstrated. His rotation was very limited.

Flexion of the cervical spine in a patient who had an occipitocervical fusion.


Hensinger and colleagues, in a review of 50 patients with Klippel-Feil syndrome, found that 30 (60%) of them had associated scoliosis.14 In some patients with Klippel-Feil syndrome, the scoliosis is congenital (see Image 7), owing to the involvement of other parts of the thoracic or lumbar spine. Other patients develop scoliosis in the thoracic spine, to compensate for cervical or cervicothoracic scoliosis. In addition to fusion anomalies in the cervical spine, cervical spinal stenosis can occur. While uncommon, this condition can increase the risk of neurologic involvement.

This anteroposterior radiograph of the spine in a patient with Klippel-Feil syndrome demonstrates congenital scoliosis and a Sprengel deformity.

This radiograph demonstrates an omovertebral bone (marked with 2 arrows). This anomaly limits cervical spine motion.

Anomalies of the craniocervical junction can cause instability at lower segments. Traumatic tetraplegia has been reported following minor trauma.15 A Sprengel anomaly occurs in 20-30% of patients (see Image 7).16 The ROM of the shoulders must be checked, and the patient should be examined for an omovertebral bone, an osteocartilaginous connection that tethers the scapula to the spine (see Image 8). An omovertebral bone ossifies with age, further limiting the ROM. A computed tomography (CT) scan best demonstrates the presence of an omovertebral bone; however, this feature can also be detected through palpation or radiographs. Other upper extremity anomalies occur less frequently. A thorough examination of the ROM and function of the upper extremity must be performed.

Renal anomalies are common in individuals with Klippel-Feil syndrome, and they can be quite serious. Out of 41 patients in Hensinger’s series who underwent an intravenous pyelogram, 16 were found to have renal anomalies. Minor renal anomalies—including a double collecting system, renal ectopia, and bilateral tubular ectasia—were detected in 6 of these individuals. Major renal anomalies—including hydronephrosis, absence of a kidney (see Image 10), and a horseshoe kidney —were detected in 10 patients. (For patients with Klippel-Feil syndrome, ultrasound [US] scanning now serves as the initial test to determine whether both of an individual’s kidneys are functioning).17 )

This intravenous pyelogram was performed before ultrasound was available to image the kidneys. Note unilateral absence of the left kidney.

Cardiovascular anomalies, mainly septal defects, were found in 7 patients in Hensinger’s series, with 4 of these individuals requiring corrective surgery. Synkinesia, or mirror movement (see Image 9), occurred in 9 of the 50 patients. Hearing was impaired in 15 of 41 patients tested. Early audiometric and otologic evaluation are indicated in all children when the diagnosis of Klippel-Feil syndrome is established.18

.

This photo demonstrates synkinesia. As the patient attempts to oppose the thumb and finger of the right hand, the same movement occurs involuntarily in the left.

Torticollis and facial asymmetry occur in 21-50% of patients with Klippel-Feil syndrome. These persons may also have a muscular torticollis.19 Craniofacial anomalies can occur as well.

Less-common anomalies associated with Klippel-Feil syndrome include congenital limb deficiencies, craniosynostosis, ear abnormalities, iniencephaly, and craniofacial abnormalities

Indications

Patients with Klippel-Feil syndrome present at different ages with varying clinical manifestations. Indications for workup vary individually. For the orthopedic surgeon, the most frequent indications for surgery depend on the amount of deformity, its location, and its progression with time. Other indications include instability of the cervical spine and/or neurologic problems. These indications can occur with craniocervical junction anomalies and when 2 fused segments are separated by a normal segment.

Some patients present early in life with complex cervical and cervicothoracic deformity that is progressive and disfiguring. Some of these patients require cervical spine fusions to prevent progression.

Other patients may develop compensatory or associated congenital scoliosis, which also can be progressive over time and requires fusion to prevent progressive deformity. Over 50% of the patients in Hensinger’s study had scoliosis.14 Treatment of the scoliosis with bracing or surgery was required in 18 of the 50 patients.

Using their above-described classification system, Samartzis and co-investigators reviewed 28 patients radiographically and clinically.2 Mean follow-up was 8.5 years. Mean age at presentation was 7.1 years, with mean age of onset of symptoms in the symptomatic patients being 11.9 years. Sixty-four percent of patients had no symptoms. Two patients developed myelopathic symptoms (type II and type III patients). Two patients developed radiculopathic symptoms (type II and type III patients). Axial symptoms were more common in type I patients. The investigators recommended activity modification in high-risk patients.

The same authors reported on a patient who developed a symptomatic cervical disc herniation.1 The patient had occipitalization of C1 and fusion of C2-3 and C4-T1. This left only C3-4 as a hypermobile segment, so the patient was at high risk. The patient was treated successfully with a same-day, combined anteroposterior (AP) procedure.

Theiss et al reviewed 32 patients with congenital scoliosis followed for more than 10 years.26 Only 7 (22%) developed cervical or cervical-related symptoms, and only 2 required surgery for their cervical-related symptoms. No fusion pattern was identified that placed the patients at greater risk for developing symptoms

Relevant Anatomy

Auerbach et al studied spinal cord dimensions in children with Klippel-Feil syndrome.27 They reviewed magnetic resonance imaging studies and clinical records of Klippel-Feil patients and age-matched controls. Torg ratios were measured, and the Torg-Pavlov ratios were found to be identical in the 2 groups. The cross-sectional area of the spinal cord was smaller in Klippel-Feil syndrome patients at each level from C2-C7. These differences were statistically significant, with no differences in the CSF column, suggesting the cord size is smaller in children with Klippel-Feil syndrome, as compared with control subjects. Four of the 12 children with Klippel-Feil syndrome presented with neurologic symptoms that improved after posterior cervical stabilization.

Samartzis et al studied the extent of fusion in the congenital K-F segment to evaluate the presence and extent of specific fusion patterns across the involved cervical segments.28 In older patients, complete fusion was more prevalent in regard to C2-C7. In the absence of complete fusion, fusion of the posterior elements was noted more often than fusion of the anterior elements.

In another paper, Samartzis et al reviewed the role of the congenitally fused segments in 29 Klippel-Feil syndrome patients in relation to the space available to the cord (SAC) and associated cervical spine-related symptoms (CSS).29 They suggested that an arrest of normal vertebral development may affect appositional bone development. The effect on vertebral body width may delay neurologic compromise resulting from the congenital fusion process and subsequent degenerative manifestations.

Contraindications

Since Klippel-Feil syndrome is associated with a constellation of possible abnormalities, no set of definite contraindications exists. If a surgeon believes that an operation is indicated, it is incumbent upon him/her to make certain that none of the conditions that could cause morbidity or mortality are present. Cervical or occipitocervical instability could increase the risk of neurologic damage during intubation. An underlying heart defect could increase anesthetic risk. An underlying spinal stenosis or spinal cord abnormality could increase the risk of neurologic damage during spinal fusion for correction of deformity. A thorough workup of the patient is imperative prior to surgical intervention.

Related Keyword terms:

,klippel-feil syndrome,klippel-feil syndrome pictures,klippel feil syndrome pictures,after a quiste pilonidal,single level fusion,samartzis klippel-feil syndrome,samartzis klippel-feil,scoliosis klippel-feil face symmetry,pictures of a shoulder being fused,surgery to fuse cervical spine due to klippel feil syndrome

Does vitamin C supplementation reduce risk of preeclampsia?

Does vitamin C supplementation reduce risk of preeclampsia?


Preeclampsia and Eclampsia: Treatment & Medication

Author: Aashit K Shah, MD, Associate Professor of Neurology, Wayne State University; Program Director, Clinical Neurophysiology Fellowship, Department of Neurology, Detroit Medical Center

Treatment

Medical Care

Considering the significant morbidity and even deaths associated with the condition, aggressive treatment of eclampsia is warranted. Close observation of the blood pressure of pregnant women is very important. Admission to an intensive care unit is justified. Eclampsia also adversely affects the fetus; therefore, when possible, labor should be promptly induced. If fetal lung maturity is a question, expeditious administration of corticosteroid is warranted. If induction and rapid vaginal delivery is not possible, abdominal delivery should be considered. Prevention of any subsequent seizures is another goal. In the past, the choice of anticonvulsant was controversial; however, 2 large multicenter randomized trials have put an end to the controversy—magnesium sulfate is now the drug of choice.22

* Magnesium sulfate (MgSO4) is superior to phenytoin sodium and diazepam in controlling recurrent seizures and is associated with lower neonatal morbidity and mortality rates.23,24 Magnesium sulfate should be administered as soon as possible after diagnosis of preeclampsia is confirmed. It should be administered immediately after a seizure, if not administered before. In case of recurrent seizure after magnesium sulfate administration, a bolus of 2 g of additional magnesium sulfate is useful.

* Control of hypertension is very important. If administration of magnesium sulfate does not reduce the blood pressure adequately, other antihypertensive agents should be used. Some of the commonly used agents are hydralazine, labetalol, nifedipine, and sodium nitroprusside.1 Nifedipine has the advantage of ease of administration via the sublingual route; the other 2 agents can be administered intravenously. In developing countries, a lower cost antihypertensive medication alpha methyldopa is used widely.

Surgical Care

Evaluation for retained products of conception and their removal may be helpful in cases of postpartum eclampsia. In case of antepartum eclampsia, cesarean delivery is useful if immediate vaginal delivery is not feasible.

Consultations

Consulting an ophthalmologist is recommended for evaluation of papilledema or retinal pathology.

Diet

No specific dietary restriction or supplementation is needed for the treatment of eclampsia. With increasing gestational age, serum ionized and total magnesium levels decrease significantly; however, dietary supplementation of magnesium is not known to have any advantages. Several studies evaluating effects of exercise and diet, including aerobic exercise, protein restriction, protein supplementation, increasing or decreasing salt intake, magnesium supplementation, and zinc supplementation, have not produced any clear answers.

Various trials of supplementation with fish oil or oil of evening primrose, which are rich sources of long-chain fatty acids, have not shown preventative effects consistently. Early studies of dietary calcium supplementation suggest that it may be helpful in preventing toxemia in women who are at highest risk and in women with a low dietary intake of calcium. However, in a large National Institutes of Health trial with healthy nulliparous women randomly assigned at 13-21 weeks’ gestation, calcium supplementation neither reduced the incidence or severity of preeclampsia nor delayed its onset.25 One of the several hypotheses of the pathogenesis of preeclampsia focuses on the oxidative stress caused by the imbalance in prooxidant and antioxidant forces.

Although preliminary findings on vitamin E and vitamin C supplementation in preeclamptic women were originally encouraging26 , a recent study may prove otherwise.

In a multicenter, randomized, controlled trial, Villar et al studied the effect of vitamin C and E supplementation in high-risk pregnant women with low nutritional status to determine if this intervention reduced preeclampsia. There were 687 women randomized to receive vitamin C (1000 mg) and vitamin E (400 IU) and 678 women who received placebo daily until delivery. At the doses used for supplementation, vitamins C and E were not associated with a reduction of preeclampsia, eclampsia, gestational hypertension, or any other maternal outcome. Low birthweight, small for gestational age, and perinatal deaths were also unaffected.27

Activity

Patients with eclampsia are usually monitored in an intensive care setting, so activity is limited. Once they recover from eclampsia, normal activity can be resumed, depending on whether abdominal delivery was performed.

Medication

The goals of pharmacotherapy are to reduce morbidity and prevent complications.

Mineral supplements

Magnesium sulfate is effective in the treatment of eclampsia. It is superior to phenytoin sodium and diazepam in controlling recurrent seizures and is useful for prevention of seizures in women with preeclampsia. Magnesium sulfate also has favorable effects on neonatal mortality and morbidity rates.

Magnesium sulfate, MgSO4

Was used in tAntihypertensives

Aggressive use of antihypertensive medications is necessary if blood pressure remains high after administration of magnesium sulfate. Continued elevation of blood pressure in the setting of eclampsia may cause further cerebral edema or cerebral hemorrhage.

Treatment of eclampsia as early as 1906. Over the years was popular in the United States and many other countries worldwide; however, conventional antiepileptic drugs and diazepam were used as treatment of eclampsia in the UK and many other centers. This changed after 2 different trials were published in 1990s shLabetalol (Normodyne, Trandate)

Alpha1-nonselective beta-blocker that can be used effectively in hypertensive emergency via IV route.

owing clear superiority of MgSO4 over phenytoin and diazepam in prevention of recurrent seizures in eclampsia and prevention of seizures in women with preeclampsia. Dosing schedules described below were used in 2 studies mentioned above.

Hydralazine (Apresoline)

Peripheral vasodilator that can be used in hypertensive emergency to quickly lower blood pressure.

Antiplatelet agents

Toxemia of pregnancy is associated with deficiency of prostaglandins (potent vasodilators) and excess of thromboxane (vasoconstrictor). This leads to the belief that low-dose aspirin with its effects on synthesis of these agents may be useful in preventing toxemia of pregnancy. Antiplatelet agents were associated with a 19% reduction in the risk of preeclampsia when a metaanalysis of 43 trials was performed. This benefit was present in trials where all women or only women at high risk of developing preeclampsia were included.

Aspirin (Ascriptin, Anacin, Bayer Aspirin, Bayer Buffered Aspirin)

Inhibits prostaglandin synthesis, preventing formation of platelet-aggregating thromboxane A2. May be used in low dose to inhibit platelet aggregation and improve complications of venous stases and thrombosis.

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