Decompression Illness

Dysbaric Illness (DI)
is a term that covers a broad range of complex
pathophysiological conditions associated with decompression. Although
much of the actual etiology of DI remains undefined, the primary cause is
believed to be separation and/or the appearance of gas within the body,
whether stationary or migratory, as result of decompression.
Gas bubbles form due to inert gas supersaturation or the traumatic
injection of gas into the arterial circulation following pulmonary barotrauma.
Its manifestations are protean which has led to the recommendation that any
signs and symptoms, observed in individuals recently exposed to a reduction
in environmental pressure, must be considered as being DI until proven
otherwise. This high index of suspicion should be extended to sub-acute and
chronic changes and to sub-clinical or ambiguous symptoms that may affect
the long bones, central nervous system and lungs.
Although primarily associated with compressed gas diving, DI also
occurs in association with altitude exposure and other sub-atmospheric
pressure reductions as well as in compressed air workers and health care
providers attending to patients while receiving hyperbaric oxygen therapy.
While the principles of management are similar, the environments in which
they may be applied vary greatly. This chapter focuses almost exclusively on
the consequences of compressed gas diving.
In understanding the study and classification of DI a technical distinction
needs be made between disease and disorder: Although often used
synonymously, the term disorder is usually assigned to physical
derangements, frequently slight and transitory in nature. A disease, on the
other hand, is usually a more serious, active, prolonged and deep-rooted
condition1
. Acute DI therefore begins as a disorder which, depending on its
pathophysiology and treatment, may become a disease 2-4
.
k
Although imperfect, the term Dysbaric Illness (DI) was chosen as the umbrella term for
decompression-related disorders and diseases. Although Dysbarism and Decompression
Illness have been proposed, these present linguistic challenges and their use is associated
with one particular descriptive classification. Although DI conceivably also includes
medical problems associated with compression its application in this chapter refers only to
those conditions that may develop upon decompression after breathing from a compressed
gas source.
2.2.1. Dysbaric Illness 175

  1. HISTORIC BACKGROUND
    2.1 Discovery of Dysbaric Illness
    Already in 1670, Robert Boyle demonstrated that Dysbaric Illness (DI)
    could be produced in a reptile by the sudden lowering of atmospheric
    pressure. Nearly two hundred years later the first case of DI was recorded in
    compressed air workers: In 1845 Triger reported that two men had suffered
    “very sharp pain” in the left arm and another had “pain in the knees and left
    shoulder” thirty minutes after emerging from a seven hour exposure to a
    pressure of between 2.4 atmospheres and 4.25 atmospheres 5
    . As technology
    advanced and supported longer exposures to greater pressures the incidence
    of DI gradually increased until it became recognized as an occupational
    illness in need of prevention and treatment.
    2.2 Prevention and Treatment of DI
    Prevention of DI could only follow an understanding of its cause.
    Accordingly, the first measures were aimed at merely alleviating the
    symptoms. Triger himself advocated “rubbing with spirits of wine” which,
    he reported, “soon relieved this pain in both men and they kept working on
    the following days”. Two years later, Pol and Watelle wrote that they felt
    “justified in hoping that a sure and prompt means of relief would be to
    recompress immediately, then decompress very carefully” 6
    .
    By the turn of the 19th century civil engineering had achieved proportions
    of depth and complexity that DI became more than a trivial concern: On
    15th May 1896 the Journal of the Society of Art described the landmark
    work of E. W. Moir during the digging of the Hudson river tunnel in 1889 7
    .
    Facing a DI fatality rate of over 25% of the employed workers, Moir
    installed a recompression chamber at the worksite. The result was a dramatic
    reduction in mortality – only two deaths were recorded during the next 15
    months out of a workforce of more than 120. Moir, who seemed almost
    apologetic about this empirical therapy, wrote: “With a view to remedying
    the state of things an air compartment like a boiler was made in which the
    men could be treated homeopathically, or reimmersed in compressed air. It
    was erected near the top of the shaft, and when a man was overcome or
    paralyzed, as I have seen them often, completely unconscious and unable to
    use their limbs, they were carried into the compartment and the air pressure
    raised to about 1/2 or 2/3 of that in which they had been working, with
    immediate improvement. The pressure was then lowered at the very slow
    rate of one pound per minute or even less. The time allowed for equalization
    being from 25 to 30 minutes, and even in severe cases the men went away
    quite cured”.
    176 A. Marroni et al.
    Ironically Paul Bert in 1878 had already validated this concept 8
    . He had
    demonstrated that the cause of DI was nitrogen going into gas phase in the
    tissues and that this bubble formation was responsible for symptoms. Bert
    understood that prompt recompression was the key to effective treatment.
    Remarkably, he already highlighted the existence of “silent bubbles” in the
    venous blood; used oxygen at one atmosphere following very rapid
    decompression; and observed that cardiopulmonary symptoms, but not
    spinal cord paralysis, were relieved by normobaric oxygen breathing.
    Since that time, prompt recompression followed by slow decompression
    has been universally considered the standard of care in the treatment of DI.
    Further developments delineated the best combinations of pressure, time, gas
    composition and decompression rates as well as the addition of resuscitation
    and adjunctive therapy (Fig. 2.2.1-1).
    Treatment of DI
    1900 1925 1950 1975 2000
    No treatment
    Hyperbaric air
    Hyperbaric O2
    Simple resuscitation
    Neuroprotection
    Year
    Figure 2.2.1-1. Developments in the Treatment for DI over the past century (Courtesy of
    Richard Moon, MD)
    However, even though we are now gaining a better understanding of the
    tools of therapy, we remain challenged by inconsistency and confusion in
    terminology for DI and uncertainty of diagnosis. Clearly there is little
    uncertainty in the case of severe neurological DI. However, minor forms
    may manifest in very subtle ways. These are likely to be ignored or denied
    by the affected diver unless there is an appropriate level of awareness.
    Indeed, there is reason to believe that there is widespread underreporting of
    symptoms that may cause an unquantified amount of morbidity. On the other
    hand over-emphasis of risks and hazards may cause hypochondriacal
    responses or deter pursuit of the activity altogether. It remains, therefore, an
    ongoing challenge to achieve the correct balance between denial and hysteria
    in the face of untoward symptoms following diving.
    2.2.1. Dysbaric Illness 177
    As far as prevention is concerned, the definitive contribution in the
    context of diving was made by Boycott, Damant and Haldane in 1908 9
    .
    Their work, although based on speculative mechanisms, forever changed the
    fate of those facing decompression after breathing compressed gas. For the
    next 60 years refinements in modeling theory, mathematics and statistics
    have suggested a variety of depth-time combinations followed by various
    decompression stops strung together by empirical ascent rates. Unfortunately
    the fundamental principles on which all of these systems are based are
    empirical as are their progeny of tables and decompression algorithms. Their
    limitations have become particularly evident in recreational diving where
    one half of DI cases do not appear to be the consequence of overt dive
    profile violations. These observations have led to a search for unique
    physiological factors and other unique risk factors. Possibilities have
    included pulmonary-systemic shunts (e.g., patent foramen ovale, atrial septal
    defects, intra pulmonary shunts, etc.); endothelial morphological anomalies
    and reactivity; activation of complement; and many more. The search for
    these elusive risk factors continues, as does their ambiguity 2,10,11
    .
    The introduction of precordial Doppler in the 1970’s promised a solution
    to the mystery of DI 12. Unfortunately the association between recordable
    bubbles and DI symptoms did not prove to be particularly strong: high grade
    bubbles correlated more closely with DI than lower grades, but DI has even
    been observed in the absence of any detectable bubbles. Nevertheless, this
    modality may again enjoy greater utility in the years to come: Recently there
    has been an increasing emphasis on paradoxical embolism of venous gas and
    a growing body of experimental and clinical evidence suggesting that large
    quantities of asymptomatic or “silent” venous bubbles may in fact be causing
    cellular and biological reactions and be releasing potentially damaging
    biochemical substances in the blood 13-16
    .
    Current prevention theory for DI recommends that participants: (1)
    possess and maintain an appropriate level of dive medical fitness (including
    controlling daily physiological variables such as body temperature and
    hydration status); (2) avoid / prevent air trapping with gas expansion (e.g.,
    not diving with respiratory infections and untreated reactive airway
    problems); and (3) observe appropriate decompression procedures for their
    exposure (i.e., following the recommended combination of pressure and time
    for their planned diving activities). Violation of one or more of these
    parameters increases the risk of DI 2,3
    .
    In the future our improved ability to detect bubbles and – more recently –
    to detect the biochemical injuries they cause, may ultimately generate more
    physiological approaches to decompression. Accordingly, future
    decompression safety is likely to focus on three areas of intervention: (1)
    manipulating decompression (ascent) to reduce the appearance, quantities
    and size of venous gas emboli; (2) reducing the propensity of the body to
    178 A. Marroni et al.
    form bubbles; and (3) preconditioning the body biochemically and/or
    physically to reduce the pathophysiological impact of such bubbles 17-25
    .
  2. TERMINOLOGY FOR AND CLASSIFICATION
    OF DYSBARIC ILLNESSES

    DI has suffered from great inconsistency in terminology and
    classification. There are many reasons for this, including wide variations in
    latency, severity and diagnostic certainty. In addition, the boundaries
    between arterial gas embolization and decompression sickness are blurred
    due to possible arterialization of venous gas. These factors have confounded
    research and remain an ongoing challenge.
    There are many ways to classify medical conditions in general, e.g., by
    diagnosis, prognosis, pathology, pathophysiology, clinical presentation, etc.
    The objective for classification is usually for the purpose of simplification.
    This may include improving communication between clinicians; determining
    treatment guidelines; establishing prognosis; or conducting research and
    analyzing epidemiological data.
    Unlike most other diseases, there is no definitive test for DI. It is only the
    causality to decompression and the prevalence of neurological involvement
    that respectively provide epidemiological boundaries and clinical relevance
    to defining and classifying the condition. To date no single system has
    emerged that provides both clear pathological stratification and clinical
    utility.
    3.1 Terminology
    Various clinical terms have emerged in an ongoing effort to describe and
    classify DI. These have ranged from descriptions of a limited number of
    distinct clinical syndromes (e.g., the “bends”, “chokes” and “staggers”); a
    presumptive assignment of etiology and severity (e.g., type I decompression
    sickness and arterial gas embolism); and the systematic capture of
    descriptive clinical and causal factors associated with the condition (e.g.,
    decompression illness or dysbarism, and gas bubble illness). Not only have
    these terms proven to be ambiguous, but they have been applied
    inconsistently and have introduced many linguistic challenges (e.g., the
    differentiation between illness and sickness).
    2.2.1. Dysbaric Illness 179
    3.2 The Classification Systems
    To date, three enduring systems have appeared for the classification of
    DI:
    x the traditional or Golding Classification 26
    x the descriptive or Francis / Smith Classification 27-29
    x the ICD-10 Classification
    Each of these classification systems addresses different objectives and
    accordingly has inherent strengths and weaknesses.
    3.2.1 The Traditional / Golding Classification
    The Golding classification, developed during the building of the Dartford
    Tunnel, was based on the assumption that DI was bivariate 26: It was either
    inert gas (decompression sickness or DCS) or embolic (arterial gas
    embolism or AGE) related. DCS was either mild (Type I) or severe (Type II)
    and each category had specific treatment recommendations. Then, in 1970 a
    combined form of DCS and AGE was described. It was called “Type III
    decompression sickness” in an effort to contain it within the existing
    classification system, but it demolished the previously clearly defined
    boundaries between DCS and AGE and illustrated the shortcomings of the
    classification system 30. Also the Golding Classification made no provision
    for the dynamic nature of DI (i.e., its tendency to present in one way but
    evolve into another), nor could it account effectively for the wide range of
    clinical severity and variability in prognosis contained within the given
    category of ‘Type II DCS’.
    The main benefit of the Golding classification remains its simplicity.
    Unfortunately, while its application may indeed have been equally
    uncomplicated in the days when commercial and military diving
    predominated the sub aquatic realm, this is no longer applicable to the
    nebulous world of recreational diving and its injuries. Recent comparisons
    have also shown significant discordance in a retrospective application of the
    Golding classification to cases relevant to the spectrum of DI encountered
    today31-33
    .
    Table 2.2.1-1. Modified Golding Classification for Dysbaric Illness
    x Arterial Gas Embolism
    x Decompression Sickness
  • Type I (Musculoskeletal Pain; Skin; Lymphatic; Extreme Fatigue; Peripheral Nervous
    Symptoms)
  • Type II (Neurologic; Cardiorespiratory; Audiovestibular; Shock)
  • Type III (Combined Decompression Sickness and Arterial Gas Embolism)
    180 A. Marroni et al.
    3.2.2 The Francis / Smith Classification
    To solve the inherent problems of the Golding Classification, Francis and
    Smith proposed a new descriptive classification system that focused on
    clinical presentation rather than etiology of DI 27-29,33. It contained
    descriptive categories that served a dual purpose of capturing actual clinical
    manifestations (e.g., the dynamic evolution and organ systems involved) as
    well as detailing factors relevant to etiological considerations (e.g., latency,
    inert gas burden and evidence of pulmonary barotrauma). This system
    offered flexibility, provided more relevant information and had a much
    higher degree of concordance between clinicians (see Table 2.2.1-2.).
    The greatest utility of this classification system is its ability to describe
    and adapt to the evolving signs and symptoms of presumed DI in a diver.
    However, it does not establish a diagnosis, only factors relevant to the
    probability thereof within a descriptive matrix of an evolving clinical
    presentation 34. However, a collection of these descriptions can be used for
    clinical comparison and to examine trends. The latter can then be analyzed
    in the pursuit of underlying pathophysiological mechanisms and – based on
    they probability of diagnosis generated by the various determinants – a
    specific diagnosis may be assigned with appropriate nomenclature or
    descriptions of symptom-clusters. Kelleher has shown that useful predictions
    regarding outcome can be made using such data 35
    .
    Table 2.2.1-2. The Francis & Smith Classification for Dysbaric Illness
    x Evolution
    o Spontaneously Recovery (Clinical improvement is evident)
    o Static (No change in clinical condition)
    o Relapsing (Relapsing symptoms after initial recovery)
    x Progressive (Increasing number or severity of signs)
    x Organ System:
    o Neurological
    o Cardiopulmonary
    o Limb pain exclusively
    o Skin
    o Lymphatic
    o Vestibular
    x Time of onset:
    o Time before surfacing
    o Time after surfacing (or estimate)
    x Gas Burden
    o Low (e.g., within NDL)
    o Medium (e.g., Decompression Dive)
    o High (e.g., Violation of Dive Table)
    2.2.1. Dysbaric Illness 181
    x Evidence of Barotrauma
    o Pulmonary (Yes / No)
    o Ears
    o Sinuses
    x Other Comments
    Unfortunately, although the Francis / Smith system has received
    increasing acceptance, its strength has also remained its fundamental
    deficiency – the inability to assign a specific diagnosis. Clinicians and
    statisticians ultimately need to work with diagnoses or case definitions.
    These have remained elusive. Nevertheless, the current recommendation of
    the European Committee for Hyperbaric Medicine (Type 1 recommendation)
    is to provisionally accept the Francis and Smith classification until a superior
    system emerges 28,29,36
    .
    3.2.3 The ICD-10 Classification
    In the International Classification of Diseases, 10th edition, there are
    various codes related to decompression illness. These are broad categories
    and do not serve the purpose of differentiating the clinical and etiological
    subtleties of the conditions: There is no sub-classification for caisson’s
    disease, although certain forms of barotrauma are sub-classified.
    The ICD-10 codes most frequently used are:
    x T70 (Effects of air pressure and water pressure)
    x T70.0 (Otitic barotrauma)
    x T70.1 (Sinus barotrauma)
    x T70.3 (Caisson’s disease)
    x T70.4 (Effects of high-pressure fluids)
    x T70.8 (Other effects of air pressure and water pressure)
    x T79.0 (Traumatic air embolism)
    x T79.7 (Traumatic subcutaneous emphysema)
    x M90.3 (Osteonecrosis in caisson disease – T70.3+)
    While the ICD-10 has become the international standard for diagnostic
    classification of general epidemiological data, its primary application is for
    billing and national healthcare decision-making processes rather than
    unraveling the intricacies of a given medical condition. In short the ICD-10
    has function and value but contributes little to the sub specialty of diving
    medicine.
    3.2.4 Future classification systems
    The present trend is towards developing clearly defined case definitions
    for DCS, AGE and combined forms. The dilemma in developing these is that
    182 A. Marroni et al.
    there is disparity between epidemiological and clinical objectives. The
    former requires specificity in favor of reliable diagnosis, the later sensitivity
    in favor of avoiding under-treatment. It is likely that a scoring system will
    ultimately be accepted which records risk-factors relevant to the exposure
    and clinical features consistent with a diagnosis. Then, depending on the
    objective the relative weighting of these factors will be adjusted respectively
    for greater specificity or sensitivity. The ECHM has recommended the
    development and acceptance of such an epidemiological classification
    system which will allow multi-centre, multinational, retrospective analyses
    derived from broad-based classifications that include the type of diving,
    chronological data, clinical manifestations and outcome of a two-year follow
    up for prognostic purposes.
  1. PATHOPHYSIOLOGY
    Although DI is causally related to bubbles, there are a multitude of
    pathophysiological mysteries between the physical appearance of bubbles
    and the onset of biological injury or clinical illness. Various unknown
    individual physical and physiological factors may contribute towards
    individual variability in the production of, and response to, bubbles 37,38
    .
    Venous bubbles are commonly observed following routine and even
    relatively shallow dives and appear to be largely asymptomatic. On the other
    hand, the appearance of left ventricular or arterial bubbles are thought to be
    of greater clinical importance; however these have rarely been observed,
    even in a laboratory setting. Recently, ocular tear film bubbles have
    generated some interest, but their persistence following decompression has
    limited their reliability as a predictor of decompression stress 3,39
    .
    When it comes to actually diagnosing DI, we presently rely almost
    exclusively on the diver’s history and an estimation of clinical probability
    from an enormously divergent spectrum of signs and symptoms following
    decompression. As yet, there are no specific biochemical markers for bubble
    related injury 40-42. Also, the sensitivity of radiological and nuclear medical
    examinations does not exceed that of clinical observation and, in the absence
    of diagnostic uncertainty, these rarely affect management. We must
    therefore accept that the best scientific case definition we have for DI at
    present is one of exclusion, i.e. the appearance of unexplained pain,
    cutaneous, cardiovascular or neurological abnormalities – chronologically
    associated with compressed gas diving – upon the exclusion of other
    etiologies. In a clinical setting, our index of suspicion should be high to
    avoid under-treatment. Fortunately, the risk of injury remains relatively
    2.2.1. Dysbaric Illness 183
    small: in recreational diving, for example, DI occurs with an incidence of
    one to three per 10,000 dives 43
    .
    4.1 The origin and destination of bubbles
    With decreasing pressure, a threshold is eventually reached where
    bubbles start to form in the body; this depends on the amount of inert gas
    and the extent of decompression. At extreme altitudes, DI cannot be avoided,
    even after prolonged oxygen pre-breathing 44
    .
    Although decompression bubbles are traditionally classified as intra- or
    extravascular, this division may be misleading as it does not describe the
    origin of bubbles but rather where they have been observed. In fact, there is
    no proof that bubbles form directly in blood vessels. Rather, it is believed
    that they may be admitted through endothelial gaps as they develop within
    surrounding perivascular tissues 45. Another mechanism for the intravascular
    appearance of bubbles is by traumatic introduction during pulmonary
    barotrauma 46. As little as 10% overexpansion of the lungs is enough to
    cause gas embolism 47,48. This would occur with an intratracheal pressure of
    76-80 mm Hg (or 99.2-108 cm H2O), or during a breath-hold ascent (after
    breathing compressed gas) from only 3 feet (~1 meter) of seawater to the
    surface 48. Although traumatic injection of gas vs. bubbles released by inert
    gas supersaturation represent two completely different etiological
    mechanisms, they are often difficult to differentiate clinically: physical or
    radiological evidence of pulmonary injury is often absent in AGE 49
    ,
    whereas arterialization of inert venous gas emboli may result in arterial gas
    embolization with clinically indistinguishable results.
    Although the origin of bubbles may be ambiguous, their effects are more
    distinct. Intravascular bubbles embolize tissue causing ischemia 50; they also
    traverse the microvasculature (so-called “transbolism”) and injure
    endothelium 51; they cause reperfusion injury and vasospasm 18. Bubbles can
    cause venous stasis, hemorrhage and precipitate plasma protein interactions 52
    .
    Extravascular or tissue bubbles disrupt and tear delicate tissues and blood
    vessels; they can also increase tissue compartment pressures i.e., cause
    regional compartment syndromes 53
    .
    4.2 Effects of Bubbles on various Tissues and Organ
    Systems
    To consolidate the various mechanisms involved in DI into meaningful
    clinical entities, it is useful to observe their effects on known target organs:
    (1) blood and blood vessels; (2) the lungs; (3) the central and peripheral
    184 A. Marroni et al.
    nervous systems; (4) the inner ear; (5) the skin and lymphatics, and (6) bones
    and joints.
    4.2.1 Blood and blood vessels
    Bubbles are biologically active. They interact with the cellular elements
    in blood as well as plasma protein cascades – coagulation, complement,
    kinin and plasmin. In addition, bubbles denature lipoproteins, liberating
    blood lipids 54-57. Blood vessels, on the other hand, sustain damage through
    physical contact. This may range from minimal damage to bleeding 51,58
    .
    Blood:
    Upon appearance of a bubble in blood, the catalyzing event appears to be
    the formation of a plasma-protein coat around the bubble. This bubble “skin”
    is made up of plasma glycoproteins, fibrinogen and gamma globulins 59,60. It
    is a biologically active interface that allows thrombocytes and white blood
    cells to become attached 61
    .
    In time, activation of platelets leads to aggregation and coalescence
    around bubbles with entrapment of other blood constituents. Cellular blood
    elements – such as red blood cells – may become entangled in the growing
    fibrin web. This thickening of the bubble “skin” may reduce diffusion
    producing a mechanism for bubble stabilization and survival 62. General
    platelet adhesiveness also increases in response to bubbles. Some studies
    have reported platelet depletion following decompression, even in the
    absence of symptoms 63. However, thrombocytopenia or anti-platelet
    therapies do not appear to protect against DI. Also aggressive
    anticoagulation runs the risk of precipitating hemorrhage in DI affecting the
    spinal cord and inner ear 61,62,64,65. On the other hand, DI does induce a
    hypercoagulable state with a high risk of thromboembolism aggravated by
    paralysis. This should be actively prevented.
    The activation of platelets and Hageman Factor also leads to activation of
    inflammatory cascades. Leukotrienes are released while the presence of
    gammaglobulin on the bubble skin, combined with the products of
    complement activation, attract white blood cells to the area 66. Leukocytes
    may interact directly with the bubble or with damaged endothelium. The
    relevance of inflammation in DI underlies the recommended use of antiinflammatory agents and, more recently, of lidocaine 67. Lidocaine also has
    leukocyte anti-adherent properties 68,69
    .
    DI has also been shown to result in elevations of blood lipid levels with
    as yet undefined clinical implications 70
    .
    While the role of various elements in blood in DI has become
    downplayed in recent literature, the significance has not disappeared: The
    search continues to find safe and effective drugs or interventions that may
    2.2.1. Dysbaric Illness 185
    attenuate the various pathophysiological events following exposure to
    bubbles. Recently research on nitric oxide donors and exercise have
    suggested that they may have a modifying role in vivo 17
    .
    Blood vessels
    The injection of 10-20µm bubbles into the carotid artery of a guinea pig
    has been shown to cause visible damage to the luminal surface surfactant
    layers of endothelial cells 71. This form of injury may result in alterations in
    vasomotor tone, precipitate platelet or leukocyte adhesion, and cause failure
    of the blood-brain barrier. In more extreme cases endothelial cells may
    actually be stripped, exposing the basement membrane to plasma proteins
    and platelets as well as adding bioactive cell remnants to the blood 61
    .
    4.2.2 The Lungs
    Unlike pulmonary barotrauma, pulmonary DI is an intravascular
    occlusive bubble disease. It results from the passage of venous gas emboli
    through pulmonary capillaries. The peri-alveolar network of capillaries
    serves as a trap for venous gas emboli. However, if the amount of gas is
    excessive, it may cause cardiac air locking and pulmonary outflow
    obstruction or microvascular obstruction with vasoconstriction, endothelial
    damage, inflammation, capillary leak and pulmonary edema – “the chokes”.
    The pulmonary “bubble trap” may also be overcome by massive
    embolization or be bypassed via broncho-pulmonary shunts, arterio-venous
    fistulae, or intra-cardiac shunts. A reduction in the diameter of circulating
    bubbles – such as by repetitive diving or “yoyo” diving – may allow bubble
    passage through the pulmonary capillary beds. All of these mechanisms may
    lead to arterialization of bubbles – so-called paradoxical gas embolism. The
    latter provides an attractive theoretical explanation for the poorly understood
    associations between “chokes”, patent foramen ovale, and DI of the central
    nervous system and skin 11,43
    .
    4.2.3 Nervous System
    Approximately two thirds of DI affects the nervous system. Although
    clinical features may be ambiguous a distinction is made between three
    potential locations of injury: (1) the spinal cord, (2) cerebrum and (3)
    peripheral nerves. In each case, the primary mechanism may be vascular
    (embolic) or extra-vascular.
    In a review of 1070 cases of neurological decompression sickness,
    Francis et al discovered that 56% occurred within 10 minutes – some even
    prior to surfacing 72. Even when considering the 768 cases of obvious spinal
    cord injury in this series the majority still presented within 10 minutes of
    186 A. Marroni et al.
    surfacing. Therefore any mechanistic theory for DI would have to account
    for this short latency. Conversely, there were 44% of cases that developed
    clinical manifestations as long as 48 hours after surfacing. This supports the
    probability of multiple mechanisms with varying latencies.
    4.2.3.1 Spinal Cord
    Four etiological theories have evolved to reconcile the varying
    observations of onset time, severity, response to therapy, and histopathology.
    They are (1) gas embolism; (2) venous infarction; (3) autochthonous (“in
    situ”) bubbles; and (4) hemorrhage or inflammation.
    Gas Embolism:
    The first theory for DI of the spinal cord was developed by Boycott and
    Damant. Lesions in the spinal cord of goats were found to consist, almost
    entirely, of white matter lesions 9
    . Indeed human pathology, although rarely
    observed in this mostly non-fatal condition, has also shown similar punctate,
    white matter lesions and hemorrhage. However, embolic injury to the spinal
    cord is, generally speaking, very rare. This is believed to be due to the
    relative difference in blood flow favoring embolization of the brain.
    Experimental spinal cord embolism has also been shown to produce
    ischemic grey matter pathology rather than white matter lesions 73. To
    confuse the matter further a type of DI was identified that began as rapid
    onset cerebral arterial gas embolism but then evolved into a particularly
    resistant form of spinal injury. This has been called “combined”,
    “concurrent” or “Type III decompression sickness” 30,74-76. Although the
    exact mechanism is still unknown, the predominant theory is related to
    growth of arterial gas emboli in tissues saturated with inert gas.
    Venous Infarction:
    In 1975, based on Batson’s experiments on tumor embolization via
    epidural veins 77, Hallenbeck et al postulated that DI of the spinal cord was
    due to bubble accumulation in the epidural venous plexus with subsequent
    venous infarction of the spinal cord. Although confirmed in extreme
    decompression 78-80, loss of function only occurred after several minutes and
    therefore did not offer an explanation for ultra-short-latency disease. In
    addition, the pattern of dysbaric injury was different to that observed in other
    causes of venous infarction of the spinal cord that typically affects the
    central grey matter 81
    .
    Autochthonous Bubbles:
    Francis proposed that rapid-onset spinal cord damage may be related to
    spontaneous bubble formation in the spinal cord white matter. He felt that
    this was the only mechanism that could explain both the rapid onset and the
    distribution of lesions observed in the spinal cord. In his classic experiment,
    the spinal cord of decompressed dogs was rapidly perfusion-fixed at the
    2.2.1. Dysbaric Illness 187
    moment of maximal disruption of somatosensory evoked potentials. He
    consistently found extravascular, white matter lesions – called
    autochthonous bubbles 82,83. The puzzling piece is how these small, scattered
    and isolated space occupying lesions (making up no more than 0.5% of the
    spinal cord and being no more than 20-200um in diameter) are able to
    produce such catastrophic clinical effects 84. It has been postulated that
    autochthonous bubbles could account for loss of function if more than 30%
    of the axons became dysfunctional due to direct injury, stretching or
    compression, inflammation, biochemical injury or hemorrhage. In support of
    this, Hills et al has also shown that small lesions (able to increase the spinal
    cord volume by 14-31%) can cause an increase in tissue pressures with a
    resulting spinal compartment syndrome 85
    .
    Hemorrhage and Inflammation:
    In his studies on autochthonous bubbles, Francis made three important
    additional observations 86: (1) animals that only developed abnormalities
    after 30 minutes had no demonstrable space occupying lesions suggesting
    another mechanisms for the dysfunction; (2) animals sacrificed some time
    after the development of rapid-onset dysfunction no longer had bubbles
    suggesting that they are temporary; and (3) the histological appearance of
    spinal cords from dogs with late-onset spinal symptoms was similar to that
    of spinal embolism or ischemic lesions 73. Interestingly in the animals
    harvested some time after rapid onset illness, hemorrhage and inflammation
    were observed in the same areas where autochthonous bubble injuries were
    seen in those harvested early. This could explain why some cases of rapid
    onset spinal cord DI appear resistant to recompression and would suggest
    caution in the use of anti-coagulants in DI of the spinal cord.
    Intriguingly, all of the mechanisms appear to converge within a particular
    area: a c-shaped area around the spinal cord grey matter. This area represents
    a watershed zone between the anterior and posterior spinal cord circulation
    and would therefore be susceptible to both inert gas accumulation as well as
    subsequent bubble-related ischemia. The cervical and lumbar enlargements
    are particularly vulnerable and also correspond to the areas of greatest
    clinical importance in DI.
    It is unlikely that one single mechanism can account for the wide variety
    of latencies and presentations of DI of the spinal cord and the decompression
    schedules leading to them. Rather it is probably the result of several
    interacting, compressive-ischemic mechanisms. DI of the spinal cord should
    be thought of as a spectrum of cause-and-effect over a 48-hour timecontinuum. It is an interplay between a variety of distinct, yet synergistic
    pathophysiological processes – some of which are amenable to
    recompression and adjunctive medical therapy and some which,
    unfortunately, are not.
    188 A. Marroni et al.
    Finally, in spite of the disturbing vulnerability of the spinal cord, it has a
    remarkable capacity of recovery. Many divers with residual deficits after
    recompression therapy continue to improve for years afterwards. However,
    this does not indicate that the injury has been reversed, only that the body
    has compensated for it 87,88
    .
    4.2.3.2 Cerebrum
    Cerebral decompression disorders differ from those of the spinal cord in
    that there is no experimental evidence suggesting autochthonous or venous
    stasis mechanisms. Accordingly, greater emphasis is placed on embolic and
    inflammatory mechanisms.
    Cerebral DI has a very short latency. In the review by Francis, 75% of
    the 311 cerebral DI cases became symptomatic within 10 minutes (even with
    all cases of overt pulmonary barotrauma specifically excluded) 72. This
    leaves paradoxical gas embolization as an attractive alternative possibility.
    Clinical Features Systemic Gas Embolism (SGE):
    Gas embolism is by its nature a systemic disease although clinically it
    primarily affects the myocardium and the brain. While coronary embolism
    may account for some diving fatalities, it is not associated with long term
    morbidity. Cerebral events, on the other hand, are associated with both short
    term mortality and long term morbidity.
    SGE gain access to the cerebral circulation via the carotid and vertebral
    arteries that converge at the base of the brain forming the circle of Willis.
    Depending on the volume of gas and region of the brain involved the clinical
    outcome of gas embolism ranges from instant death to spontaneous
    uneventful recovery. Relapses have been reported in up to 30% of patients
    with arterial gas embolism following submarine escape, irrespective of
    preceding or concurrent recompression 89-91. A subset of patients may also
    suffer subclinical damage only visualized by medical imaging 74
    .
    Irrespective of the cause, the ultimate outcome of cerebral gas
    embolization appears to depend on the anatomical location, gas volume,
    delivery rate, pre-embolic gas saturation as well as co-morbid factors such as
    hypotension or dysfunction of vital centers.
    4.2.3.3 Peripheral Nervous System
    The peripheral nervous system may be affected by decompression
    injuries anywhere from the posterior horn of the spinal cord, to the mixed
    nerves, brachial or lumbar plexus, and cutaneous or muscular innervations.
    The most important considerations are differential diagnosis and prognosis.
    This area probably represents one of the areas of greatest clinical ambiguity.
    However, even though the underlying pathophysiology remains obscure, the
    prognosis is usually good.
    2.2.1. Dysbaric Illness 189
    4.2.4 Inner Ear
    There are four dominant theories for the clinical and pathological
    findings associated with DI of the inner ear. They are: (1) explosive /
    hemorrhagic injuries; (2) counter diffusion; (3) gas induced osmosis; and (4)
    vascular emboli 92,93
    .
    Explosive / hemorrhagic injuries
    In 1980, Landolt et al found hemorrhage in the inner ear of squirrel
    monkeys subjected to rapid decompression from saturation 94. Three years
    later Venter was able to show an implosive injury of the semicircular canals
    as the cause for the hemorrhage 95. The mechanism, they proposed, was one
    of gas accumulation in temporal bone osteoclast pockets that then
    explosively ruptured into the inner ear during decompression. Money
    subsequently found evidence of the same type of injury in a diver who died
    56 days after left inner ear DI 96. This mechanism is plausible for deep mixed
    gas diving, but less convincing for inner ear DI following shallower dives.
    Embolism
    Blood supply to the inner ear is endarterial and consequently prone to
    embolic or vascular injury. Embolic disturbance has been shown in cardiac
    bypass surgery, but how this relates to diving remains uncertain 97
    .
    Counterdiffusion
    This theory entertains the possibility that counterdiffusion can occur
    under conditions where the inert gas in the middle ear differs from that in the
    breathing mixture. Diffusion through the round or oval window could result
    in accumulation of inert gas with bubbling, resulting in deafness or vertigo.
    This theory has developed due to a high prevalence of inner ear DI in
    helium-oxygen and mixed gas divers 27. Counterdiffusion may also occur
    within the partitions of the inner ear itself. The vascularity of the inner ear is
    not uniform: the stria vascularis supplies the endolymph directly and from
    there inert gas would diffuse to the perilymph. Therefore, with gas
    switching, it is possible that the endolymph could rapidly take up a new inert
    gas, e.g. helium, before the perilymph has had time to eliminate the former
    inert gas. Bubbles could then form within the endolymph with disruption of
    function and even rupture 27
    .
    Gas-induced Osmosis
    Finally, by a similar mechanism, inert gas accumulation in the
    endolymph could result in gas-induced osmosis: an osmotic fluid shift
    towards the endolymph resulting in a form of hydrops endolymphaticus
    analogous to Meniere’s disease.
    190 A. Marroni et al.
    4.3 Skin
    Skin bends or DI of the skin may present in a variety of ways with
    varying etiologies and clinical significance.
    Diver’s Lice
    This erythematous rash usually presents in association with dry chamber
    dives or the use of dry suits. The hypothesis is that inert gas enters the skin
    directly and causes dermal bubbles with histamine release upon
    decompression. The condition can be avoided by not having gas skin
    contact, or by heating the skin during decompression. It is not considered
    serious in the absence of other findings, and does not require recompression.
    Cutis Marmorata
    A more significant form of DI of the skin is called cutis marmorata or
    skin marbling. Although the condition itself is benign, its association with
    pulmonary and neurological DI requires careful consideration. Experimental
    work in pigs has shown that this pattern of illness is associated with venous
    congestion, inflammation, leukocyte adherence and endothelial damage 98
    .
    No bubbles have been visualized, but the manifestations usually resolve
    promptly with recompression.
    Counterdiffusion
    A rare type of DI may result from exposure to different inert gases, such
    as helium and nitrogen. Diffusion-related gas accumulation may occur when
    one gas is in contact with the skin, while another is breathed 99,100
    .
    4.4 Musculoskeletal
    Some of the first descriptions of DI or “the bends” involved painful joints
  2. Even today, musculoskeletal pain it is the most common presenting
    complaint 102
    .
    There are two bone and joint conditions associated with decompression:
    acute muskuloskeletal DI and dysbaric osteonecrosis.
    4.4.1 Acute Musculoskeletal DI
    Although they have similar blood supply, joints, and musculo-tendinous
    attachments, it is noteworthy that ‘bends’ pain only appears to affect long
    bones of the appendicular skeleton – not the axial skeleton. Adult long bones
    contain a fatty marrow cavity that could be a reservoir for inert gas and
    predispose to DI. Axial bones largely contain haemopoietic tissue which
    appears to be unaffected by decompression.
    Another interesting feature of ‘bends’ pain is that it is influenced by premorbid hyperbaric activity. In a review of more than 19,000 cases, Sowden
    2.2.1. Dysbaric Illness 191
    found that bounce divers and pilots primarily developed shoulder pain,
    whereas saturation divers and caisson workers developed knee pain 103
    .
    There are many theories but little evidence to explain this phenomenon.
    There are four theories for bubble-related pain in bones and joints. They
    involve stretching of nerve endings or inflammation occurring (1) within
    joints; (2) around the joints, such as within tendons and muscle; (3) within
    bone, due to gas expansion within fatty marrow, the medullary cavity and
    bone sinusoids (a phenomenon also associated with cancer-pain), and; (4) as
    a result of referred pain, either due to an injury to the nerves or nerve roots
    associated with the joint, or due to a generalized release of inflammatory
    modulators with flu-like symptoms and poliarthralgia.
    Intra- and periarticular pain associated with decompression can usually
    be localized and is of a non serious nature. There is a trend towards treating
    these conservatively although they respond well and promptly to
    recompression. Referred pain is part of the neurological spectrum of DI that
    has been considered elsewhere. What remains, is medullary pain.
    The discovery of sinusoid innervation has led to the concept of a venous
    congestive mechanism for cancer and osteoarthritic bone pain 104,105. This
    sinusoid congestion pain theory is also attractive as an explanation for
    ‘bends’ pain as it addresses several clinical phenomena: (1) the deep, poorly
    localized, boring pain; (2) relief achieved by the local application of pressure
    (e.g., a BP cuff); and (3) a gravity-related distribution of manifestations in
    the various patient subgroups.
    Although there is no scientific association between medullary pain and
    dysbaric osteonecrosis, it is usually viewed as a more serious form of
    musculoskeletal DI and recompression is recommended.
    4.4.2 Dysbaric Osteonecrosis
    Dysbaric osteonecrosis appears to affect predominantly saturation divers
    and caisson workers. Again it appears to be the appendicular skeleton that is
    at risk, particularly the humoral head, femoral head and juxta articular area
    of the distal femur and proximal tibia. Lesions in proximity to the femoral
    and humeral head may be symptomatic and may eventually become
    disabling whereas femoral and tibial shaft lesions remain asymptomatic. The
    question remains why it is only certain types of diving that predispose to this
    disease, and why these areas are so uniquely vulnerable 106
    .
    192 A. Marroni et al.
    4.5 Concluding Remarks on DI Mechanisms
    The confusion and controversy regarding the pathophysiology of DI is
    exemplified by the division that still exists in the use of two classification
    systems: one pathological 107, and the other clinical 28,29
    .
    While we do not always know the cause of DI, we must not ignore
    mechanisms altogether, thereby being unable to appreciate risk, determine
    probability of injury and prescribe effective and rational treatment. Astute
    clinical observation and focused research must remain the vital tools for
    unraveling the mysteries of DI.
  3. EPIDEMIOLOGY
    There are five important epidemiological observations that have been
    made regarding DI 2-4,14,21-24,108-123:
    x The incidence is very low in general – in the order of 0.01-0.05 %
    (1 to 5 per 10,000 dives).
    x There is no demonstrable gender-specific predisposition to DI.
    x Novice and experienced divers are at greatest risk for developing
    DI.
    x Dives in excess of 24 meters (80 feet) have a significantly greater
    incidence of DI.
    x Neurological involvement is by far the most common manifestation
    in recreational divers.
    5.1 Risk Factors
    A variety of risk factors have been identified over the past century in an
    effort to explain the variations in susceptibility to DI 2-4,14,21-24,108-123. These
    include:
    x Altitude Exposure: either diving at altitude or subsequent exposure
    x Exercise: before, after or during the bottom phase of a dive
    x Injury: previous DI or other acute injuries
    x Omitted decompression: missed stops / errors of depth monitoring
    x Uncontrolled ascents
    x Personal traits (gender; physiognomy; level of fitness)
    x Profiles: (variables of depth, multiple ascents, ascent rates and the
    depth prior to final ascent to the surface within the same ‘squareprofile’ or depth-time envelope)
    x Previous dives: residual nitrogen
    x Hypoventilation: CO2 build-up
    2.2.1. Dysbaric Illness 193
    x Hypothermia: gas retention-release due to solubility changes
    x Hypovolemia: dehydration and hemoconcentration
    Although the relevance of individual factors is understood and – in some
    cases – obvious, there is surprisingly little evidence on the relative
    contributions of these factors towards the overall risk 38. The following are
    discussed in more detail below:
    5.1.1 Exercise: before, after or during the bottom phase of a dive
    Exercise during exposure to increased ambient pressure (during the
    bottom phase of the dive) appears to increase the incidence of DI. The
    probable explanation of this is that the increased perfusion during exercise
    leads to an increased uptake of inert gas that must be subsequently
    eliminated during decompression. Conversely Vann et al have shown that
    exercise during decompression stops may reduce the incidence of DI by an
    opposite mechanism 25. Exercise prior to or following diving are risk factors
    however and there are at least three reasons for this:
    x Rapidly flowing blood, especially in the area of bifurcation of
    vessels, may create foci of relative negative pressure through a
    Venturi effect. Small numbers of molecules of gas from the
    surrounding supersaturated blood may then diffuse into these foci
    down a partial pressure gradient. The resulting localized collections
    of small numbers of gas molecules are known as gas micronuclei and
    are thought to act as a ‘nidus’ for bubble formation.
    x Distraction between articulating joint surfaces causes extreme
    reductions in ambient pressure and gradients of up to 270 atm. This
    process – called tribonucleation – is thought to offer some
    explanation for the variable incidence between different forms of
    diving and compressed air activities vs. the ultra-low incidence of DI
    during extravehicular activity in space. In the latter case it is thought
    that the absence of gravity reduces articular shearing forces and in
    the absence of ongoing production these micronuclei seem to
    disappear.
    x Increased local CO2 production in exercising muscle may play a role
    since CO2 is a highly diffusible gas and might contribute to the
    formation of gas micronuclei.
    5.1.2 Injury: previous DI or other acute injuries
    Recent local injury seems to lead to an increased incidence of DI
    manifested by pain at or near the site of the injury. The mechanism
    responsible for this phenomenon is unclear. Changes in local perfusion and
    194 A. Marroni et al.
    increased gas micronuclei formation in injured tissue are postulated
    mechanisms.
    5.1.3 Personal traits (gender; physiognomy; level of fitness)
    There is no convincing evidence that gender affects DI risk in
    recreational divers. Indirect measures of fitness and body mass have yet to
    yield definitive answers on relative risk for DI. Advancing age is thought to
    increase the incidence of DI for reasons that are not yet clearly known.
    5.1.4 Hypoventilation: CO2 build-up
    Even small increases in FiCO2 seem to increase the incidence of DI. The
    mechanism of this effect is not clearly understood, although increased
    availability of this highly diffusible gas for diffusion into gas micronuclei;
    vasodilatation with increased inert gas uptake; and reduction in the ‘oxygen
    window’ are all relevant factors.
    5.1.5 Hypothermia: gas retention-release due to solubility changes
    Diving in cold water tends to increase the incidence of DI. Inert gas
    uptake is generally not affected since the exercising diver is usually warm
    and has increased tissue perfusion because of exercise. However, when the
    diver leaves the bottom and reaches his decompression stop where he
    remains at rest he is likely to become cold. The resulting peripheral
    vasoconstriction may then impair inert gas elimination.
    5.1.6 Hypovolemia: dehydration and hemoconcentration
    Dehydration was reported as a factor that increases the risk of DI during
    studies on aviators during World War II, and it is still considered a
    significant risk factor by the international diving and diving medical
    communities. The mechanism is however again unclear and reliable
    scientific evidence is missing. Changes in the surface tension in serum
    favoring bubble formation has been postulated as a possible mechanism.
    Alcohol ingestion prior to diving, also seems to be a risk factor – possibly
    due to dehydration.
    2.2.1. Dysbaric Illness 195
  4. CLINICAL MANIFESTATIONS 2,3,14
    DI presents at various intervals following hyperbaric exposure 14: 50%
    occur within 30 minutes of surfacing; 85% occur within one hour of
    surfacing; 95% occur within 3 hours of surfacing; 1% delayed more than 12
    hours. However, symptoms have been reported to appear as late as 24 hours
    and more after surfacing and even longer if exposure to altitude follows the
    hyperbaric excursion.
    As far as manifestations are concerned, the clinical presentation of DI has
    been divided into two broad categories based on severity of symptoms prior
    to recompression. h
    MILD DI
    This category includes the following:
    x Limb pain
    x Lymphatic manifestations, or
    x Cutaneous manifestations,
    in the absence of any other systemic manifestations
    MODERATE TO SEVERE DI
    This includes the following:
    x Pulmonary DI
    x Central Nervous System DI (Brain; Spinal Cord; Inner Ear)
    x Shock
    x Girdle Pain (Abdominal; Thoracic; or Lumbar)
    x Extreme fatigue
    6.1
    Mild DI includes those categories traditionally assigned to Type I in the
    Golding Classification 26. Mild DI forms do not display any features of
    moderate to severe DI and this should be confirmed by a competent health
    care professional.
    Pain
    The upper extremities are involved 3 times more often than the lower
    limbs in recreational and compressed air commercial diving. The situation is
    reversed in caisson workers and in commercial saturation (Heliox) diving.
    The pain can range from slight transient discomfort (‘niggle’) to a dull,
    deep, boring and unbearable pain. It is usually not affected by movement and
    h
    As iterated in the preceding sections, DI does not present in watertight clinical
    compartments, nor can the etiology always be confirmed. It should therefore be understood
    that the clinical entities presented here may coincide or overlap with others across the
    arbitrarily assigned boundaries of mild, moderate and severe DI.
    Mild DI
    196 A. Marroni et al.
    there can occasionally be some degree of overlying local pitting edema and
    subjective numbness (refer section 4.4.1).
    Lymphatic Manifestations
    The lymphatic manifestations of DI presumably result from obstruction
    of lymphatic vessels by bubbles. The manifestations can include pain and
    swelling of regional lymph nodes, with lymph edema of those tissues
    drained by the obstructed lymph nodes.
    Cutaneous Manifestations
    Itching is commonly reported during decompression from dry chamber
    dives where the skin is surrounded by chamber atmosphere rather than
    water. It is thought to be the result of diffusion of gas from the chamber
    atmosphere directly into the skin, followed by expansion during
    decompression and a consequent itching sensation. This is not considered a
    systemic form of DI and therefore need not be treated with recompression.
    Itching with or without discoloration, signs of urticaria and/or blotchiness
    occurring after in-water diving is considered to be systemic cutaneous DI i
    .
    Cutis Marmorata or marbling is thought to result from bubble-related
    cutaneous venous obstruction. It usually presents as an area of erythema,
    frequently affecting the upper back and chest. Lesions may migrate
    spontaneously or with palpation and prominent linear purple markings are
    frequently observed. These manifestations are considered to be an overt
    manifestation of DI and should be promptly treated j
    . Recompression often,
    although not always, leads to prompt resolution.
    6.2 Moderate to severe DI
    Moderate to severe DI includes those categories traditionally assigned to
    Type II (and III) in the Golding Classification 26. The criteria for moderate to
    severe DI are met if at any stage DI presents with more than simple limb
    pain and cutaneous features:
    Pulmonary DI
    This relatively uncommon syndrome usually presents with a
    gnomonic triad of:
    i
    If a wet dive took place in a dry suit, then there may be direct absorption of gas into the skin
    as for a chamber dive. This should be distinguished from dives resulting in cutaneous
    lesions where no skin-gas contact occurred.
    j
    Cutis marmorata is associated with systemic decompensation, paradoxical embolism (e.g.,
    via a PFO, ASD or intra-pulmonary shunt) and high spinal, cerebral and audiovestibular
    forms of DI. Therefore while the condition itself is not particularly serious, its associations
    prompt attention to the other systems of greater vulnerability and justify urgent
    recompression.
    patho-
    2.2.1. Dysbaric Illness 197
    x Substernal pain: usually burning and progressively increasing.
    Initially the pain may be noted only when coughing. Over time
    the pain may become constant.
    x Cough: initially intermittent and easily provoked by cigarette
    smoking (Behnke’s sign). Paroxysms of coughing may become
    uncontrollable.
    x Progressive respiratory distress and dyspnea.
    The manifestations of pulmonary DI are believed to result from the
    combined effects of gas emboli in the pulmonary artery and obstruction of
    the vascular supply to the bronchial mucosa. Untreated Pulmonary DI may
    be fatal.
    Neurological DI
    The neurological manifestations of Dysbaric Illness are unpredictable:
    They range from minor sensory abnormalities to loss of consciousness and
    death. Clinically they may resemble acute stroke or an acute exacerbation of
    multiple sclerosis. Although an increasing number of mild sensory changes
    are being assigned to peripheral nerve and nerve root lesions (some of which
    may not necessarily be related to DI), the clinical prerogative is to assume a
    central origin until proven otherwise and to manage these presentations
    promptly.
    Cerebral DI: Brain involvement in DI appears to be especially common
    in aviators. However it is not known if this is due to paradoxical
    embolization of large volumes of venous gas; the release vasoactive
    mediators; hypoxemia due to a disruption of pulmonary circulation in
    conditions of marginal oxygenation at extreme altitude or something else
    entirely. In this group a migraine like headache often accompanies visual
    disturbances. When there is brain involvement in divers a common
    presentation is hemiparesis which is different to the clinical presentation in
    aviators. Collapse with unconsciousness are rare presentations of DI and
    more likely to be due to injected gas than bubble evolution from
    supersaturated tissues.
    Spinal Cord DI: Paraplegia is a ‘classic’ symptom of DI in divers and
    almost invariably represents spinal cord involvement 
    . Bladder paralysis
    with urinary retention and fecal incontinence frequently accompany
    paraplegia. In the last few years, cases of serious paralysis in recreational
    divers dropped from 13.4 percent in 1987 to only 2.9 percent in 1997, and
    the number of cases of divers losing consciousness dropped from 7.4 percent
    to 3.9 percent of total injuries during the same period. The incidence of loss
    of bladder function, another sign of neurological DI, dropped from 2.2
    percent to 0.4 percent during this period (DAN Diving Accident Reports) 119
    .
    
    A notable exception is embolization of the anterior cerebral arteries which can also present
    with bilateral lower extremity paralysis and loss of bladder function.
    198 A. Marroni et al.
    However this change in incidence has not been balanced by an equivalent
    rise in the frequency of pain only or skin DI. On the contrary, there seems to
    be a trend towards an increased incidence of milder neurological
    manifestations. Paresthesias, some with well and other with less clearly
    defined presentations (including ubiquitous numbness and/or tingling) as
    well as vague, ambiguous and ill-defined symptoms now predominate
    recreational DI. The diagnostic ambiguity is compounded by the fact that
    many of these manifestations appear to respond to normobaric oxygen and
    recompression. However, while suggestive, response to recompression
    cannot be considered diagnostic in these conditions. Certain non-diving
    related nerve damage also appears to respond to hyperoxygenation so that
    one of the greatest clinical challenges in DI lies in unraveling true diving
    injuries from a myriad of other transient peripheral neuropathies 124
    .
    Inner Ear DI
    Cochlear and/or vestibular DI was previously almost exclusively
    associated with saturation and experimental diving and – accordingly – quite
    rare. In recent times the incidence has been increasing due to an escalation in
    deep recreational technical diving involving mixed gas and gas switching on
    decompression. Either or both cochlear and vestibular apparatus may be
    involved presenting with tinnitus, deafness, vertigo, nausea, and vomiting.
    Nystagmus may be present on physical examination. The mechanisms
    remain unclear (refer section 4.2.4.). Inner ear DI is a serious medical
    emergency and must be treated immediately to avoid permanent damage.
    Since the nutrient arteries of the inner ear are very small, rapid reduction in
    bubble diameter, with immediate 100% oxygen administration and prompt
    recompression are essential.
    Shock
    Shock occasionally occurs in DI and is usually associated with serious
    pulmonary and cardiovascular manifestations. Fatalities are usually the
    result of shock or pulmonary forms of DI and resuscitation and immediate
    recompression are of paramount importance.
    Girdle Pain: Back, Abdominal, or Chest Pain
    Unlike limb pain, pain in these areas should be considered carefully as it
    is frequently associated with progressive spinal cord DI.
    Extreme Fatigue
    Fatigue disproportionate to the amount of preceding activity has long
    been regarded as an evolving serious manifestation of DI. The biochemical
    and pathophysiological mechanisms are unknown although there is
    increasing evidence that it may be the result of vasoactive mediators and
    cytokines released due to venous gas embolization.
    2.2.1. Dysbaric Illness 199
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