1.

Introduction

1. Introduction

This volume of the 10th revision of the International statistical classification

of diseases and related health problems (ICD-10) contains guidelines for
recording and coding, together with much new material on practical aspects
of the classification’s use, as well as an outline of the historical background to
the classification. This material is presented as a separate volume, for ease of
handling when reference needs to be made at the same time to the classification
(Volume 1) and the instructions for its use. Detailed instructions on the use of
the Alphabetical index are contained in the introduction to Volume 3.

This manual provides a basic description of the ICD, together with practical
instructions for mortality and morbidity coders, and guidelines for the
presentation and interpretation of data. It is not intended to provide detailed
training in the use of the ICD. The material included here needs to be
augmented by formal courses of instruction that allow extensive practice on
sample records and discussion of problems.

If problems arising from the use of the ICD cannot be resolved either locally
or with the help of national statistical offices, advice is available from the
World Health Organization (WHO) Collaborating Centres for the Family of
International Classifications (FIC) (see Volume 1).

1
 2. Description of the ICD
2. Description of the ICD

2. Description of the International statistical

classification of diseases and related health
problems

2.1 Purpose and applicability

A classification of diseases can be defined as a system of categories to which
morbid entities are assigned according to established criteria. The purpose
of the ICD is to permit systematic recording, analysis, interpretation and
comparison of mortality and morbidity data collected in different countries or
areas and at different times. The ICD is used to translate diagnoses of diseases
and other health problems from words into an alphanumeric code, which
permits easy storage, retrieval and analysis of the data.

In practice, the ICD has become the international standard diagnostic

classification for all general epidemiological and many health-management
purposes. These include analysis of the general health situation of population
groups and monitoring of the incidence and prevalence of diseases and other
health problems in relation to other variables, such as the characteristics
and circumstances of the individuals affected. The ICD is neither intended
nor suitable for indexing of distinct clinical entities. There are also some
constraints on the use of the ICD for studies of financial aspects, such as
billing or resource allocation.

The ICD can be used to classify diseases and other health problems recorded
on many types of health and vital records. Its original use was to classify
causes of mortality as recorded at the registration of death. Later, its scope
was extended to include diagnoses in morbidity. It is important to note that,
although the ICD is primarily designed for the classification of diseases and
injuries with a formal diagnosis, not every problem or reason for coming into
contact with health services can be categorized in this way. Consequently,
the ICD provides for a wide variety of signs, symptoms, abnormal findings,
complaints and social circumstances that may stand in place of a diagnosis
on health-related records (see Volume 1, Chapters XVIII and XXI). It can
therefore be used to classify data recorded under headings such as ‘diagnosis’,
‘reason for admission’, ‘conditions treated’ and ‘reason for consultation’, which
appear on a wide variety of health records from which statistics and other
health-situation information are derived.

2.2 The concept of a ‘family’ of disease and health-related

classifications
Although the ICD is suitable for many different applications, it does not
serve all the needs of its various users. It does not provide sufficient detail for

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INTERNATIONAL CLASSIFICATION OF DISEASES

some specialties and sometimes information on different attributes of health

conditions may be needed. The ICD is also not useful to describe functioning
and disability as aspects of health, and does not include a full array of health
interventions or reasons for encounter.

Foundations laid by the International Conference on ICD-10 in 1989 have

provided the basis for the development of a ‘family’ of health classifications
(see Volume 1, Report of the International Conference for the 10th
Revision of the International Classification of Diseases, Section 6, Family of
classifications). In recent years, through the use of the ICD and development
of related WHO health classifications, the concept of a ‘family’ was further
developed. Currently, so-called family designates a suite of integrated
classification products that share similar features and can be used singularly
or jointly to provide information on different aspects of health and the health-
care system. For example, the ICD as a reference classification is mainly used
to capture information on mortality and morbidity. Additional aspects of
health domains, functioning and disability have now been jointly classified
in the International classification of functioning, disability and health (ICF). In
general, the WHO Family of International Classifications (WHO-FIC) aims
to provide a conceptual framework of information dimensions that are related
to health and health management. In this way, they establish a common
language to improve communication and permit comparisons of data across
countries’ health-care disciplines, services and time. WHO and the WHO-
FIC Network strive to build the family of classifications so that it is based
on sound scientific and taxonomic principles; is culturally appropriate and
internationally applicable; and focuses on the multidimensional aspects of
health, so that it meets the needs of its different users.

The WHO-FIC attempts to serve as the framework of international standards

to provide the building blocks of health information systems. Fig. 1 represents
the types of classifications in the WHO-FIC.

4
 2. Description of the ICD

Fig. 1. Schematic representation of the WHO-FIC

Related classifications Reference classifications Derived classifications

International Classification
International Classification International of Diseases for Oncology,
of Primary Care (ICPC) Classification of (ICD-O)
Diseases (ICD)
International Classification The ICD-10 classification
of Nursing Practices (ICPN) of mental and behavioural
disorders
International
International Classification Application of the ICD to
Classification of
of External Causes of Injury dentistry and stomatology,
Functioning,
(ICECI) (ICD-DA)
Disability and Health
(ICF) Application of the ICD to
The Anatomical, neurology (ICD-NA)
Therapeutic, Chemical (ATC)
classification system with Application of the ICD to
International dermatology
defined daily doses (DDD)
Classification of
Health Application of the ICD to
ISO9999 Technical aids for paediatrics
Interventions (ICHI)
persons with disabilities:
(under development) Application of the ICD
classification and
terminology to rheumatology and
orthopaedics (ICD-R & 0)

Reference classifications
These are the classifications that cover the main parameters of the health
system, such as death, disease, functioning, disability, health and health
interventions. WHO reference classifications are a product of international
agreements. They have achieved broad acceptance and official agreement
for use and are approved and recommended as guidelines for international
reporting on health. They may be used as models for the development or
revision of other classifications, with respect to both the structure and the
character and definition of the classes.

Currently, there are two reference classifications in the WHO-FIC: the ICD as
a reference classification to capture information on mortality and morbidity,
and the ICF to capture information on various domains of human functioning
and disability. WHO has been exploring the possibility of replacing the former
International classification of procedures in medicine (see Section 2.2.2 Non-
diagnostic classifications) with a new International classification of health
interventions (ICHI). This process will take place over several stages of
consultation, field-testing and approval by the WHO governing bodies.

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INTERNATIONAL CLASSIFICATION OF DISEASES

Derived classifications
Derived classifications are based upon reference classifications. Derived
classifications may be prepared by adopting the reference classification
structure and classes, providing additional detail beyond that provided by
the reference classification, or they may be prepared through rearrangement
or aggregation of items from one or more reference classifications. Derived
classifications are often tailored for use at the national or international level.

Within the WHO-FIC, the derived classifications include specialty-based

adaptations of ICF and ICD, such as the International classification of diseases
for oncology, 3rd edition (ICD-O-3), the Application of the international
classification of diseases to dentistry and stomatology, 3rd edition (ICD-DA),
the ICD-10 classification of mental and behavioural disorders (included in
Chapter V of the ICD-10) and the Application of the international classification
of diseases to neurology, 2nd edition (ICD-10-NA) (see Section 2.2.1 Diagnosis-
related classifications).

Related classifications
Related classifications are those that partially refer to reference classifications,
or that are associated with the reference classification at specific levels of the
structure only. Procedures for maintaining, updating and revising statistical
classifications of the family encourage the resolution of problems of partial
correspondence among related classifications, and offer opportunities for
increased harmony over time. Within the WHO-FIC, the related classifications
include: the International classification of primary care, 2nd edition (ICPC-2),
the International classification of external causes of injury (ICECI), Technical
aids for persons with disabilities: classification and terminology (ISO9999) and
the Anatomical therapeutic chemical classification (ATC) with defined daily
doses (ATC/DDD).

2.2.1 Diagnosis-related classifications

Special tabulation lists
The special tabulation lists are derived directly from the core classification, for
use in data presentation and to facilitate analysis of health status and trends at
the international, national and subnational levels. The special tabulation lists
recommended for international comparisons and publications are included in
Volume 1. There are five such lists, four for mortality and one for morbidity
(for further details, see Sections 5.4 and 5.5).

Specialty-based adaptations
Specialty-based adaptations usually bring together, in a single, compact
volume, the sections or categories of the ICD that are relevant to a particular
specialty. The four-character subcategories of the ICD are retained, but more

6
 2. Description of the ICD

detail is often given by means of fifth-character or sometimes sixth-character

subdivisions, and there is an Alphabetical index of relevant terms. Other
adaptations may give glossary definitions of categories and subcategories
within the specialty.

The adaptations have often been developed by international groups of

specialists, but national groups have sometimes published adaptations that
have later been used in other countries. The following list includes some of the
major specialty adaptations to date.

Oncology
The third edition of the International classification of diseases for oncology
(ICD-O-3), published by WHO in 2000, is intended for use in cancer
registries, and in pathology and other departments specializing in cancer (1).
ICD-O is a dual-axis classification with coding systems for both topography
and morphology. For most neoplasms, the topography code uses the same
three-character and four-character categories used in ICD-10 for malignant
neoplasms (categories C00–C80). ICD-O thus allows greater specificity of site
for non-malignant neoplasms than is possible in ICD-10.

The morphology code for neoplasms has been adopted by the Systematized
nomenclature of medicine (SNOMED) (2), which was derived from the 1968
edition of the Manual of tumor nomenclature and coding (MOTNAC) (3) and
the Systematized nomenclature of pathology (SNOP) (4). The morphology code
has five digits; the first four digits identify the histological type and the fifth
the behaviour of the neoplasm (malignant, in situ, benign, etc.). The ICD-O
morphology codes also appear in Volume 1 of ICD-10 and are added to the
relevant entries in Volume 3, the Alphabetical index. Tables are available for
conversion of the ICD-O-3 codes to ICD-10 codes.

Dermatology
In 1978, the British Association of Dermatologists published the International
coding index for dermatology (5), which was compatible with the ninth revision
of the ICD. The association has also published an adaptation of ICD-10 to
dermatology, under the auspices of the International League of Dermatological
Societies.

Dentistry and stomatology

The third edition of the Application of the international classification of diseases
to dentistry and stomatology (ICD-DA), based on ICD-10, was published by
WHO in 1995 (6). It brings together ICD categories for diseases or conditions
that occur in, have manifestations in, or have associations with the oral cavity
and adjacent structures. It provides greater detail than ICD-10, by means of
a fifth digit, but the numbering system is organized so that the relationship
between an ICD-DA code and the ICD code from which it is derived is

7
INTERNATIONAL CLASSIFICATION OF DISEASES

immediately obvious, and so that data from ICD-DA categories can be readily
incorporated into ICD categories.

Neurology
In 1997 WHO published an adaptation of ICD-10 to neurology (ICD-10-NA)
(7), which retains the classification and coding systems of ICD-10 but is further
subdivided at the fifth-character level and beyond, to allow neurological
diseases to be classified with greater precision.

Rheumatology and orthopaedics

The International League of Associations of Rheumatology is working on
a revision of the Application of the international classification of diseases to
rheumatology and orthopaedics (ICD-R&O), including the International
classification of musculoskeletal disorders (ICMSD), to be compatible with
ICD-10. The ICD-R&O provides detailed specification of conditions through
the use of additional digits, which allow for extra detail while retaining
compatibility with ICD-10. The ICMSD is designed to clarify and standardize
the use of terms and is supported by a glossary of generic descriptors for
groups of conditions, such as the inflammatory polyarthropathies.

Paediatrics
Under the auspices of the International Pediatric Association, the British
Paediatric Association (BPA) has published an application of ICD-10 to
paediatrics, which uses a fifth digit to provide greater specificity. This follows
similar applications prepared by BPA for ICD-8 and ICD-9.

Mental disorders
For each category in Chapter V of ICD-10 (Mental and behavioural
disorders), The ICD-10 classification of mental and behavioural disorders:
clinical descriptions and diagnostic guidelines, published by WHO in 1992,
provides a general description and guidelines concerning the diagnosis, as
well as comments about differential diagnosis and a listing of synonyms and
exclusion terms (8). Where more detail is required, the guidelines give further
subdivisions at the fifth- and sixth-digit levels. A second publication relating
to Chapter V, The ICD-10 classification of mental and behavioural disorders:
diagnostic criteria for research, was published in 1993 (9).

A version of the classification for use in primary health care (10), and another
version that uses a rearrangement of categories of childhood mental disorders
in a multiaxial system (11), to allow simultaneous assessment of the clinical
state, relevant environmental factors and the degree of disability linked to the
disease, has also been developed.

8
 2. Description of the ICD

2.2.2 Non-diagnostic classifications

Procedures in medicine
The International classification of procedures in medicine (ICPM) was published
in two volumes by WHO in 1978 (12, 13). It includes procedures for medical
diagnosis, prevention, therapy, radiology, drugs, and surgical and laboratory
procedures. The classification has been adopted by some countries, while
others have used it as a basis for developing their own national classifications
of surgical operations.

The heads of WHO Collaborating Centres for Classification of Diseases

recognized that the process of consultation that had to be followed before
finalization and publication was inappropriate in such a wide and rapidly
advancing field. They therefore recommended that there should be no revision
of the ICPM in conjunction with the 10th revision of the ICD.

In 1987, the Expert Committee on the International Classification of Diseases

asked WHO to consider updating at least the outline for surgical procedures
(Chapter 5) of the ICPM for the 10th revision. In response to this request
and the needs expressed by a number of countries, the Secretariat prepared a
tabulation list for procedures.

At their meeting in 1989, the heads of the collaborating centres agreed that
the list could serve as a guide for the national publication of statistics on
surgical procedures and could also facilitate intercountry comparisons. The
list could also be used as a basis for the development of comparable national
classifications of surgical procedures.

Work on the list will continue, but any publication will follow the issue of
ICD-10. In the meantime, other approaches to this subject are being explored.
Some of these have common characteristics, such as a fixed field for specific
items (organ, technique, approach, etc.), the possibility of being automatically
updated, and the flexibility of being used for more than one purpose.

The International classification of functioning, disability and health

The ICF was published by WHO in all six WHO official languages in 2001
(14), after its official endorsement by the 54th World Health Assembly on 22
May 2001. It has subsequently been translated into over 25 languages.

The ICF classifies health and health-related states in two parts. Part 1
classifies functioning and disability. Part 2 comprises environmental and
personal contextual factors. Functioning and disability in Part 1 are described
from the perspectives of the body, the individual and society, formulated in
two components: (i) body functions and structures, and (ii) activities and
participation. Since an individual’s functioning and disability occur in a
context, the ICF also includes a list of environmental factors.

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INTERNATIONAL CLASSIFICATION OF DISEASES

The ICF has superseded the International classification of impairments,

disabilities, and handicaps (ICIDH) (15). As a consequence, the old ICIDH
terms and definitions have been replaced by the following new ICF terms and
definitions:

Functioning is a generic term for body functions, body structures,

activities and participation. It denotes the positive aspects of the
interaction between an individual (with a health condition) and that
individual’s contextual factors (environmental and personal factors).
Disability is an umbrella term for impairments, activity limitations
and participation restrictions. It denotes the negative aspects of the
interaction between an individual (with a health condition) and that
individual’s contextual factors (environmental and personal factors).
Body functions are the physiological functions of body systems
(including psychological functions).
Body structures are anatomical parts of the body, such as organs, limbs
and their components.
Impairments are problems in body function or structure, such as a
significant deviation or loss.
Activity is the execution of a task or action by an individual.
Activity limitations are difficulties an individual may have in executing
activities.
Participation is involvement in a life situation.
Participation restrictions are problems an individual may experience in
involvement in life situations.
Environmental factors make up the physical, social and attitudinal
environment in which people live and conduct their lives.

The ICF uses an alphanumeric system in which the letters b, s, d and e are
used to denote body functions, body structures, activities and participation,
and environmental factors, respectively. These letters are followed by a
numeric code that starts with the chapter number (one digit), followed by the
second level (two digits), and the third and fourth levels (one digit each). ICF
categories are ‘nested’ so that broader categories are defined to include more
detailed subcategories of the parent category. Any individual may have a range
of codes at each level. These may be independent or interrelated.

The ICF codes are only complete with the presence of a qualifier, which denotes
a magnitude of the level of health (e.g. severity of the problem). Qualifiers
are coded as one, two or more numbers after a point (or separator). Use of
any code should be accompanied by at least one qualifier. Without qualifiers,
codes have no inherent meaning. The first qualifier for body functions and
body structures, the performance and capacity qualifiers for activities and

10
 2. Description of the ICD

participation, and the first qualifier for environmental factors all describe the
extent of problems in the respective component.

The ICF puts the notions of ‘health’ and ‘disability’ in a new light. It
acknowledges that every individual can experience a decrement in health
and thereby experience some disability. This is not something that happens
to only a minority of people. The ICF thus ‘mainstreams’ the experience of
disability and recognizes it as a universal human experience. By shifting
the focus from cause to impact, it places all health conditions on an equal
footing, allowing them to be compared using a common metric – the ruler
of health and disability. Furthermore, the ICF takes into account the social
aspects of disability and does not see disability only as a medical or biological
dysfunction. By including contextual factors, in which environmental factors
are listed, the ICF allows the impact of the environment on the person’s
functioning to be recorded.

The ICF is WHO’s framework for measuring health and disability at both
individual and population levels. While the ICD classifies diseases and causes
of death, the ICF classifies health domains. The ICD and ICF constitute the two
major building blocks of the WHO-FIC. Together, they provide exceptionally
broad yet accurate tools to capture the full picture of health.

2.2.3 Information support to primary health care

One of the challenges identified in the Global strategy for health for all by
the year 2000 (16) is the provision of information support to primary health
care. In countries without complete information, or with only poor-quality
data, a variety of approaches need to be adopted to supplement or replace the
conventional use of the ICD.

Since the late 1970s, various countries have experimented with the collection
of information by lay personnel. Lay reporting has subsequently been extended
to a broader concept called ‘non-conventional methods’. These methods,
covering a variety of approaches, have evolved in different countries as a
means of obtaining information on health status where conventional methods
(censuses, surveys, vital or institutional morbidity and mortality statistics)
have been found to be inadequate.

One of these approaches, so-called community-based information, involves

community participation in the definition, collection and use of health-
related data. The degree of community participation ranges from involvement
only in data collection to the design, analysis and utilization of information.
Experience in several countries has shown that this approach is more than a
theoretical framework. The International Conference for the 10th Revision of
the International Classification of Diseases (see Volume 1) noted in its report:

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INTERNATIONAL CLASSIFICATION OF DISEASES

The Conference was informed about the experience of countries in

developing and applying community-based health information that
covered health problems and needs, related risk factors and resources.
It supported the concept of developing non-conventional methods
at the community level as a method of filling information gaps in
individual countries and strengthening their information systems.
It was stressed that, for both developed and developing countries,
such methods or systems should be developed locally and that,
because of factors such as morbidity patterns, as well as language and
cultural variations, transfer to other areas or countries should not be
attempted.

Given the encouraging results of this approach in many countries, the

conference agreed that WHO should continue to give guidance on the
development of local schemes and to support the progress of the methodology.

2.2.4 International nomenclature of diseases

In 1970, the Council for International Organizations of Medical Sciences
(CIOMS) began the preparation of an International Nomenclature of Diseases
(IND), with the assistance of its member organizations, and five volumes
of provisional nomenclature were issued during 1972 and 1974. It was soon
realized, however, that, if the nomenclature were to be truly international,
the compilation of such a nomenclature would need much wider consultation
than was possible through the members of CIOMS alone. In 1975, the IND
became a joint project of CIOMS and WHO, guided by a technical steering
committee of representatives from both organizations.

The principal objective of the IND was to provide, for each morbid entity, a single
recommended name. The main criteria for selection of this name were that it
should be specific (applicable to one and only one disease), unambiguous, as
self-descriptive and simple as possible, and based on cause, wherever feasible.
However, many widely used names that did not fully meet the above criteria
were retained as synonyms, provided they are not inappropriate, misleading
or contrary to the recommendations of international specialist organizations.
Eponymous terms are avoided, since they are not self-descriptive; however,
many of these names are in such widespread use (e.g. Hodgkin disease,
Parkinson disease and Addison disease) that they must be retained.

Each disease or syndrome for which a name is recommended is defined as

unambiguously and as briefly as possible. A list of synonyms appears after
each definition. These comprehensive lists are supplemented, if necessary,
by explanations about why certain synonyms have been rejected or why an
alleged synonym is not a true synonym.

12
 2. Description of the ICD

The IND is intended to be complementary to the ICD. The differences

between a nomenclature and a classification are discussed in Section 2.3. As
far as possible, IND terminology has been given preference in the ICD.

The volumes of the IND published up to 1992 are: Infectious diseases (bacterial
diseases (1983) (17), mycoses (1982) (18), viral diseases (1985) (19), parasitic
diseases (1987) (20)); Diseases of the lower respiratory tract (1979) (21); Diseases
of the digestive system (1990) (22); Cardiac and vascular diseases (1989) (23);
Metabolic, nutritional and endocrine disorders (1991) (24); Diseases of the
kidney, the lower urinary tract, and the male genital system (1992) (25); and
Diseases of the female genital system (1992) (26).

2.2.5 The role of WHO

Most of the classifications described above are the product of very close
collaboration between nongovernmental organizations, other agencies, and
divisions and units of WHO, with the unit responsible for the ICD and the ICF
assuming a coordinating role and providing guidance and advice.

WHO promotes the development of adaptations that extend both the

usefulness of the ICD and the ICF and the comparability of health statistics.
The role of WHO in the development of new classifications, adaptations
and glossaries is to provide cooperative leadership and to act as a clearing-
house, giving technical advice, guidance and support when needed. Anyone
interested in preparing an adaptation of ICD-10 or the ICF should consult with
WHO as soon as a clear statement of the objectives of the adaptation has been
developed. Unnecessary duplication will thus be avoided, by a coordinated
approach to the development of the various components of the family.

2.3 General principles of disease classification

As William Farr stated in 1856 (27):

Classification is a method of generalization. Several classifications

may, therefore, be used with advantage; and the physician, the
pathologist, or the jurist, each from his own point of view, may
legitimately classify the diseases and the causes of death in the way
that he thinks best adapted to facilitate his inquiries, and to yield
general results.

A statistical classification of diseases must be confined to a limited number of

mutually exclusive categories that are able to encompass the whole range of
morbid conditions. The categories have to be chosen to facilitate the statistical
study of disease phenomena. A specific disease entity that is of particular public
health importance, or that occurs frequently, should have its own category.
Otherwise, categories will be assigned to groups of separate but related

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INTERNATIONAL CLASSIFICATION OF DISEASES

conditions. Every disease or morbid condition must have a well-defined place

in the list of categories. Consequently, throughout the classification, there will
be residual categories for other and miscellaneous conditions that cannot be
allocated to the more specific categories. As few conditions as possible should
be classified to residual categories.

It is the element of grouping that distinguishes a statistical classification from

a nomenclature, which must have a separate title for each known morbid
condition. The concepts of classification and nomenclature are, nevertheless,
closely related because a nomenclature is often arranged systematically.

A statistical classification can allow for different levels of detail if it has a

hierarchical structure with subdivisions. A statistical classification of diseases
should retain the ability both to identify specific disease entities and to
allow statistical presentation of data for broader groups, to enable useful and
understandable information to be obtained.

The same general principles can be applied to the classification of other health
problems and reasons for contact with health-care services, which are also
incorporated in the ICD.

The ICD has developed as a practical, rather than a purely theoretical

classification, in which there are a number of compromises between
classification based on etiology, anatomical site, circumstances of onset, etc.
There have also been adjustments to meet the variety of statistical applications
for which the ICD is designed, such as mortality, morbidity, social security
and other types of health statistics and surveys.

2.4 The basic structure and principles of classification of

the ICD
The ICD is a variable-axis classification. The structure has developed out of
that proposed by William Farr in the early days of international discussions on
classification structure. His scheme was that, for all practical, epidemiological
purposes, statistical data on diseases should be grouped in the following way:

• epidemic diseases
• constitutional or general diseases
• local diseases arranged by site
• developmental diseases
• injuries.

This pattern can be identified in the chapters of ICD-10. It has stood the test
of time and, though in some ways arbitrary, is still regarded as a more useful
structure for general epidemiological purposes than any of the alternatives
tested.

14
 2. Description of the ICD

The first two, and the last two, of the groups listed above comprise ‘special
groups’ that bring together conditions that would be inconveniently
arranged for epidemiological study were they to be scattered, for instance in
a classification arranged primarily by anatomical site. The remaining group,
‘local diseases arranged by site’, includes the ICD chapters for each of the main
body systems.

The distinction between the ‘special groups’ chapters and the ‘body systems’
chapters has practical implications for understanding the structure of the
classification, for coding to it, and for interpreting statistics based on it. It has
to be remembered that, in general, conditions are primarily classified to one of
the ‘special groups’ chapters. Where there is any doubt as to where a condition
should be positioned, the ‘special groups’ chapters should take priority.

The basic ICD is a single coded list of three-character categories, each of which
can be further divided into up to 10 four-character subcategories. In place of
the purely numeric coding system of previous revisions, the 10th revision uses
an alphanumeric code with a letter in the first position and a number in the
second, third and fourth positions. The fourth character follows a decimal
point. Possible code numbers therefore range from A00.0 to Z99.9. The letter
U is not used (see Section 2.4.7).

2.4.1 Volumes
ICD-10 comprises three volumes: Volume 1 contains the main classifications;
Volume 2 provides guidance to users of the ICD; and Volume 3 is the
Alphabetical index to the classification.

Most of Volume 1 is taken up with the main classification, composed of the

‘List of three-character categories’ and the ‘Tabular list of inclusions and four-
character subcategories’. The ‘core’ classification – the list of three-character
categories (Volume 1) – is the mandatory level for reporting to the WHO
mortality database and for general international comparisons. This core
classification also lists chapter and block titles. The Tabular list, giving the full
detail of the four-character level, is divided into 22 chapters.

Volume 1 also contains the following:

• Special tabulation lists for mortality and morbidity. Because the full four-
character list of the ICD, and even the three-character list, are too long to be
presented in every statistical table, most routine statistics use a tabulation
list that emphasizes certain single conditions and groups others. The four
special lists for the tabulation of mortality are an integral part of the ICD.
Lists 1 and 2 are for general mortality and lists 3 and 4 are for infant and
child mortality (ages 0–4 years). There is also a special tabulation list for
morbidity. These are set out in Volume 1. Guidance on the appropriate use

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INTERNATIONAL CLASSIFICATION OF DISEASES

of the various levels of the classification and the tabulation lists is given in
section 5 of this volume.
• Definitions. The definitions in Volume 1 have been adopted by the
World Health Assembly and are included to facilitate the international
comparability of data.
• Regulations regarding nomenclature. The regulations adopted by the World
Health Assembly set out the formal responsibilities of WHO Member
States regarding the classification of diseases and causes of death, and the
compilation and publication of statistics. They are found in Volume 1.

2.4.2 Chapters
The classification is divided into 22 chapters. The first character of the ICD
code is a letter, and each letter is associated with a particular chapter, except
for the letter D, which is used in both Chapter II, Neoplasms, and Chapter
III, Diseases of the blood and blood-forming organs and certain disorders
involving the immune mechanism, and the letter H, which is used in both
Chapter VII, Diseases of the eye and adnexa and Chapter VIII, Diseases of the
ear and mastoid process. Four chapters (Chapters I, II, XIX and XX) use more
than one letter in the first position of their codes.

Each chapter contains sufficient three-character categories to cover its

content; not all available codes are used, allowing space for future revision
and expansion.

Chapters I–XVII relate to diseases and other morbid conditions, and Chapter
XIX relates to Injury, poisoning and certain other consequences of external
causes. The remaining chapters complete the range of subject matter currently
included in diagnostic data. Chapter XVIII covers Symptoms, signs and
abnormal clinical and laboratory findings, not elsewhere classified. Chapter
XX, External causes of morbidity and mortality, was traditionally used to
classify causes of injury and poisoning, but, since the ninth revision, has
also provided for any recorded external cause of diseases and other morbid
conditions. Finally, Chapter XXI, Factors influencing health status and contact
with health services, is intended for the classification of data explaining the
reason for contact with health-care services of a person not currently sick, or
the circumstances in which the patient is receiving care at that particular time,
or otherwise having some bearing on that person’s care.

16
 2. Description of the ICD

2.4.3 Blocks of categories

The chapters are subdivided into homogeneous blocks of three-character
categories. In Chapter I, the block titles reflect two axes of classification –
mode of transmission and broad group of infecting organisms. In Chapter
II, the first axis is the behaviour of the neoplasm; within behaviour, the axis
is mainly by site, although a few three-character categories are provided for
important morphological types (e.g. leukaemias, lymphomas, melanomas,
mesotheliomas, Kaposi sarcoma). The range of categories is given in
parentheses after each block title.

2.4.4 Three-character categories

Within each block, some of the three-character categories are for single
conditions, selected because of their frequency, severity or susceptibility to
public health intervention, while others are for groups of diseases with some
common characteristic. There is usually provision for ‘other’ conditions to be
classified, allowing many different but rarer conditions, as well as ‘unspecified’
conditions, to be included.

2.4.5 Four-character subcategories

Although not mandatory for reporting at the international level, most of
the three-character categories are subdivided by means of a fourth, numeric
character after a decimal point, allowing up to 10 subcategories. Where a
three-character category is not subdivided, it is recommended that the letter
‘X’ be used to fill the fourth position, so that the codes are of a standard length
for data-processing.

The four-character subcategories are used in whatever way is most appropriate,

identifying, for example, different sites or varieties if the three-character
category is for a single disease, or individual diseases if the three-character
category is for a group of conditions.

The fourth character .8 is generally used for ‘other’ conditions belonging to the
three-character category, and .9 is mostly used to convey the same meaning as
the three-character category title, without adding any additional information.

When the same fourth-character subdivisions apply to a range of three-

character categories, they are listed once only, at the start of the range. A
note at each of the relevant categories indicates where the details are to be
found. For example, categories O03–O06, for different types of abortion, have
common fourth characters relating to associated complications (see Volume
1).

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INTERNATIONAL CLASSIFICATION OF DISEASES

2.4.6 Supplementary subdivisions for use at the level of the fifth or

subsequent character
The fifth and subsequent character levels are usually subclassifications along
a different axis from the fourth character. They are found in:

Chapter XIII – subdivisions by anatomical site;

Chapter XIX – subdivisions to indicate open and closed fractures, as well as
intracranial, intrathoracic and intra-abdominal injuries with
and without open wound;
Chapter XX – former subdivisions to indicate the type of activity being
undertaken at the time of the event have now become
optional additional information that is recorded in a
separate field.

2.4.7 Chapter XXII, ‘U’ codes

Codes U00–U49 are to be used by WHO for the provisional assignment of
new diseases of uncertain etiology. Codes U50–U99 may be used in research,
for example, when testing an alternative subclassification for a special project.
Currently the range includes Severe acute respiratory syndrome (SARS), and
special codes for bacterial agents resistant to antibiotics.

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 3. How to use the ICD

3. How to use the ICD

This section contains practical information that all users need to know in order
to exploit the classification to its full advantage. Knowledge and understanding
of the purpose and structure of the ICD are vital for statisticians and analysts
of health information, as well as for coders. Accurate and consistent use of the
ICD depends on the correct application of all three volumes.

3. How to use the ICD

3.1 How to use Volume 1
3.1.1 Introduction
Volume 1 of the ICD contains the classification itself. It indicates the categories
into which diagnoses are to be allocated, facilitating their sorting and counting
for statistical purposes. It also provides those using statistics with a definition
of the content of the categories, subcategories and tabulation list items they
may find included in statistical tables.

Although it is theoretically possible for a coder to arrive at the correct code by

the use of Volume 1 alone, this would be time-consuming and could lead to
errors in assignment. An Alphabetical index, as a guide to the classification,
is contained in Volume 3. The introduction to the index provides important
information about its relationship with Volume 1.

Most routine statistical uses of the ICD involve selection of a single condition
from a certificate or record where more than one is entered. The rules for this
selection in relation to mortality and morbidity are contained in Section 4 of
this volume.

A detailed description of the Tabular list is given in Section 2.4.

3.1.2 Use of the Tabular list of inclusions and four-character

subcategories
Inclusion terms
Within the three- and four-character rubrics,1 a number of other diagnostic
terms are usually listed. These are known as ‘inclusion terms’ and are given, in
addition to the title, as examples of the diagnostic statements to be classified
to that rubric. They may refer to different conditions or be synonyms. They
are not a subclassification of the rubric.

1
In the context of the ICD, ‘rubric’ denotes either a three-character category or a four-character subcategory.

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INTERNATIONAL CLASSIFICATION OF DISEASES

Inclusion terms are listed primarily as a guide to the content of the rubrics.
Many of the items listed relate to important or common terms belonging to
the rubric. Others are borderline conditions or sites listed to distinguish the
boundary between one subcategory and another. The lists of inclusion terms
are by no means exhaustive and alternative names of diagnostic entities are
included in the Alphabetical index, which should be referred to first when
coding a given diagnostic statement.

It is sometimes necessary to read inclusion terms in conjunction with titles.

This usually occurs when the inclusion terms are elaborating lists of sites
or pharmaceutical products, where appropriate words from the title (e.g.
‘malignant neoplasm of ...’, ‘injury to ...’, ‘poisoning by ...’) need to be understood.

General diagnostic descriptions common to a range of categories, or to all the

subcategories in a three-character category, are to be found in notes headed
‘Includes’, immediately following a chapter, block or category title.

Exclusion terms
Certain rubrics contain lists of conditions preceded by the word ‘Excludes’.
These are terms that, although the rubric title might suggest that they were
to be classified there, are in fact classified elsewhere. An example of this is
in category A46, ‘Erysipelas’, where postpartum or puerperal erysipelas is
excluded. Following each excluded term, in parentheses, is the category or
subcategory code elsewhere in the classification to which the excluded term
should be allocated.

General exclusions for a range of categories or for all subcategories in a three-

character category are to be found in notes headed ‘Excludes’, immediately
following a chapter, block or category title.

Glossary descriptions
In addition to inclusion and exclusion terms, Chapter V, Mental and
behavioural disorders, uses glossary descriptions to indicate the content of
rubrics. This device is used because the terminology of mental disorders varies
greatly, particularly between different countries, and the same name may be
used to describe quite different conditions. The glossary is not intended for
use by coding staff.

Similar types of definition are given elsewhere in the ICD, for example,
Chapter XXI, to clarify the intended content of a rubric.

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 3. How to use the ICD

3.1.3 Two codes for certain conditions

The ‘dagger and asterisk’ system
ICD-9 introduced a system, continued in ICD-10, whereby there are two codes
for diagnostic statements containing information about both an underlying
generalized disease and a manifestation in a particular organ or site that is a
clinical problem in its own right.

The primary code is for the underlying disease and is marked with a dagger (†);
an optional additional code for the manifestation is marked with an asterisk (*).
This convention was provided because coding to underlying disease alone was
often unsatisfactory for compiling statistics relating to particular specialties,
where there was a desire to see the condition classified to the relevant chapter
for the manifestation when it was the reason for medical care.

While the dagger and asterisk system provides alternative classifications for
the presentation of statistics, it is a principle of the ICD that the dagger code
is the primary code and must always be used. For coding, the asterisk code
must never be used alone. However, for morbidity coding, the dagger and
asterisk sequence may be reversed when the manifestations of a disease are
the primary focus of care. Statistics incorporating the dagger codes conform
to the traditional classification for presenting data on mortality and other
aspects of medical care.

Asterisk codes appear as three-character categories. There are separate

categories for the same conditions occurring when a particular disease is not
specified as the underlying cause. For example, categories G20 and G21 are for
forms of parkinsonism that are not manifestations of other diseases assigned
elsewhere, while category G22* is for ‘Parkinsonism in diseases classified
elsewhere’. Corresponding dagger codes are given for conditions mentioned
in asterisk categories; for example, for Syphilitic parkinsonism in G22*, the
dagger code is A52.1†.

Some dagger codes appear in special dagger categories. More often, however,
the dagger code for dual-element diagnoses and unmarked codes for single-
element conditions may be derived from the same category or subcategory.

The areas of the classification where the dagger and asterisk system operates are
limited; there are 83 special asterisk categories throughout the classification,
which are listed at the start of the relevant chapters.

Rubrics in which dagger-marked terms appear may take one of three different
forms.

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INTERNATIONAL CLASSIFICATION OF DISEASES

(i) If the symbol (†) and the alternative asterisk code both appear in the
rubric heading, all terms classifiable to that rubric are subject to dual
classification and all have the same alternative code, e.g.:

A17.0† Tuberculous meningitis (G01*)

Tuberculosis of meninges (cerebral) (spinal)
Tuberculous leptomeningitis
(ii) If the symbol appears in the rubric heading but the alternative asterisk
code does not, all terms classifiable to that rubric are subject to dual
classification but they have different alternative codes (which are listed
for each term), e.g:

A18.1† Tuberculosis of genitourinary system

Tuberculosis of:
bladder (N33.0*)
cervix (N74.0*)
kidney (N29.1*)
male genital organs (N51.-*)
ureter (N29.1*)
Tuberculous female pelvic inflammatory disease (N74.1*)

(iii) If neither the symbol nor the alternative code appears in the title, the
rubric as a whole is not subject to dual classification but individual
inclusion terms may be; if so, these terms will be marked with the
symbol and their alternative codes given, e.g.:

A54.8 Other gonococcal infections

Gonococcal:
...
peritonitis† (K67.1*)
pneumonia† (J17.0*)
sepsis
skin lesions
Other optional dual coding
There are certain situations, other than in the dagger and asterisk system, that
permit two ICD codes to be used to describe fully a person’s condition. The
note in Volume 1, Tabular list, ‘Use additional code, if desired ...’, identifies
many of these situations. The additional codes would be used only in special
tabulations.

These are:

(i) for local infections, classifiable to the ‘body systems’ chapters, codes
from Chapter I may be added to identify the infecting organism, where
this information does not appear in the title of the rubric. A block of
categories, B95–B98, is provided for this purpose in Chapter I;

22
 3. How to use the ICD

(ii) for neoplasms with functional activity. The appropriate code from
Chapter IV may be added to the code from Chapter II, to indicate the
type of functional activity;
(iii) for neoplasms, the morphology code from ICD-O, although not part
of the main ICD, may be added to the Chapter II code to identify the
morphological type of the tumour;
(iv) for conditions classifiable to F00–F09, organic, including symptomatic,
mental disorders, in Chapter V, where a code from another chapter may
be added to indicate the cause, i.e. the underlying disease, injury or
other insult to the brain;
(v) where a condition is caused by a toxic agent, a code from Chapter XX
may be added to identify that agent;
(vi) where two codes can be used to describe an injury, poisoning or other
adverse effect: a code from Chapter XIX, which describes the nature of
the injury, and a code from Chapter XX, which describes the cause. The
choice as to which code should be the additional code depends upon
the purpose for which the data are being collected. (See introduction to
Chapter XX, of Volume 1.)

3.1.4 Conventions used in the Tabular list

In listing inclusion and exclusion terms in the Tabular list, the ICD employs
some special conventions relating to the use of parentheses, square brackets,
colons, braces, the abbreviation ‘NOS’, the phrase ‘not elsewhere classified’
(NEC), and the word ‘and’ in titles. These need to be clearly understood both
by coders and by anyone wishing to interpret statistics based on the ICD.

Parentheses ( )
Parentheses are used in Volume 1 in four important situations.

(a) Parentheses are used to enclose supplementary words, which may follow
a diagnostic term without affecting the code number to which the words
outside the parentheses would be assigned. For example, in I10, the
inclusion term, ‘Hypertension (arterial)(benign)(essential)(malignant)
(primary)(systemic)’, implies that I10 is the code number for the word
‘Hypertension’ alone or when qualified by any, or any combination, of
the words in parentheses.
(b) Parentheses are also used to enclose the code to which an exclusion
term refers. For example:
H01.0 Blepharitis
Excludes: blepharoconjunctivitis (H10.5).

23
INTERNATIONAL CLASSIFICATION OF DISEASES

(c) Another use of parentheses is in the block titles, to enclose the three-
character codes of categories included in that block.
(d) The last use of parentheses was incorporated in the ninth revision and
is related to the dagger and asterisk system. Parentheses are used to
enclose the dagger code in an asterisk category or the asterisk code
following a dagger term.
Square brackets [ ]
Square brackets are used:

(a) for enclosing synonyms, alternative words or explanatory phrases; for

example:
A30 Leprosy [Hansen disease];

(b) for referring to previous notes; for example:

C00.8 Overlapping lesion of lip
[See note 5 at the beginning of this chapter];

(c) for referring to a previously stated set of fourth-character subdivisions

common to a number of categories; for example:
K27 Peptic ulcer, site unspecified
[See at the beginning of this block for subdivisions].

Colon :
A colon is used in listings of inclusion and exclusion terms when the words that
precede it are not complete terms for assignment to that rubric. They require
one or more of the modifying or qualifying words indented under them before
they can be assigned to the rubric. For example, in K36, Other appendicitis,
the diagnosis ‘appendicitis’ is to be classified there only if qualified by the
words ‘chronic’ or ‘recurrent’.

Brace }
A brace (indicated by a vertical line) is used in listings of inclusion and
exclusion terms to indicate that neither the words that precede it nor the
words after it are complete terms. Any of the terms before the brace should be
qualified by one or more of the terms that follow it. For example:

O71.6 Obstetric damage to pelvic joints and ligaments

Avulsion of inner symphyseal cartilage
Damage to coccyx obstetric
Traumatic separation of symphysis (pubis)

24
 3. How to use the ICD

‘NOS’
The letters NOS are an abbreviation for ‘not otherwise specified’, implying
‘unspecified’ or ‘unqualified’.

Sometimes, an unqualified term is nevertheless classified to a rubric for a

more specific type of the condition. This is because, in medical terminology,
the most common form of a condition is often known by the name of the
condition itself and only the less common types are qualified. For example,
‘mitral stenosis’ is commonly used to mean ‘rheumatic mitral stenosis’. These
inbuilt assumptions have to be taken into account in order to avoid incorrect
classification. Careful inspection of inclusion terms will reveal where an
assumption of cause has been made; coders should be careful not to code a
term as unqualified unless it is quite clear that no information is available that
would permit a more specific assignment elsewhere. Similarly, in interpreting
statistics based on the ICD, some conditions assigned to an apparently specified
category will not have been so specified on the record that was coded. When
comparing trends over time and interpreting statistics, it is important to be
aware that assumptions may change from one revision of the ICD to another.
For example, before the eighth revision, an unqualified aortic aneurysm was
assumed to be due to syphilis.

‘Not elsewhere classified’

The words ‘not elsewhere classified’, when used in a three-character category
title, serve as a warning that certain specified variants of the listed conditions
may appear in other parts of the classification. For example:

J16 Pneumonia due to other infectious organisms, not elsewhere

classified.
This category includes J16.0, Chlamydial pneumonia, and J16.8, Pneumonia
due to other specified infectious organisms. Many other categories are provided
in Chapter X (for example, J09–J15) and other chapters (for example, P23.-,
Congenital pneumonia) for pneumonias due to specified infectious organisms.
J18, Pneumonia, organism unspecified, accommodates pneumonias for which
the infectious agent is not stated.

‘And’ in titles
‘And’ stands for ‘and/or’. For example, cases of ‘tuberculosis of bones’,
‘tuberculosis of joints’ and ‘tuberculosis of bones and joints’ are to be classified
in the rubric A18.0†, Tuberculosis of bones and joints.

25
INTERNATIONAL CLASSIFICATION OF DISEASES

Point dash .-
In some cases, the fourth character of a subcategory code is replaced by a
dash, e.g.:

G03 Meningitis due to other and unspecified causes

Excludes: meningoencephalitis (G04.-)
meningomyelitis (G04.-)

This indicates to the coder that a fourth character exists and should be sought
in the appropriate category. This convention is used in both the Tabular list
and the Alphabetical index.

3.1.5 Categories with common characteristics

For quality control, it is useful to introduce programmed checks into the
computer system. The following groups of categories are provided as a basis
for such checks on internal consistency, grouped according to the special
characteristic that unites them.

Asterisk categories
Asterisk categories are not to be used alone; they must always be used in
addition to a dagger code.

Categories limited to one sex

Some diseases, injuries and factors influencing health status and contact
with health services are limited to, or more likely to occur in, only one sex.
A list of such conditions is given in the Annex 7.8. It is recommended that
the list be used to check the consistency of data at the time of coding. If the
reported diagnosis and the reported sex are inconsistent, clarification of the
information provided should be sought.

Guidance for handling inconsistencies between causes of death and sex of

decedents is given in Section 4.3.8.

Sequelae categories
The following categories are provided for sequelae of conditions that are no
longer in an active phase:

B90–B94, E64.-, E68, G09, I69.-, O97, T90–T98, Y85–Y89.

Guidance for coding sequelae for both mortality and morbidity purposes can
be found in Sections 4.3.6 and 4.5.2.

26
 3. How to use the ICD

Postprocedural disorders
The following categories are not to be used for underlying-cause mortality
coding. Guidance for their use in morbidity coding is found in Section 4.5.2:

E89.-, G97.-, H59.-, H95.-, I97.-, J95.-, K91.-, M96.-, N99.-

3.2 How to use Volume 3

The Introduction to Volume 3, the Alphabetical index to ICD-10, gives
instructions on how to use it. These instructions should be studied carefully
before starting to code. A brief description of the structure and use of the
Alphabetical index is given below.

3.2.1 Arrangement of the Alphabetical index

Volume 3 is divided into three sections as follows:

• Section I lists all the terms classifiable to Chapters I–XIX and Chapter
XXI, except drugs and other chemicals;
• Section II is the index of external causes of morbidity and mortality and
contains all the terms classifiable to Chapter XX, except drugs and other
chemicals;
• Section III, Table of drugs and chemicals, lists for each substance the
codes for poisonings and adverse effects of drugs classifiable to Chapter
XIX, and the Chapter XX codes that indicate whether the poisoning was
accidental, deliberate (self-harm), undetermined, or an adverse effect of a
correct substance properly administered.

3.2.2 Structure
The Alphabetical index contains ‘lead terms’, positioned to the far left of the
column, with other words (‘modifiers’ or ‘qualifiers’) at different levels of
indentation under them. In Section I, these indented modifiers or qualifiers
are usually varieties, sites or circumstances that affect coding; in Section II,
they indicate different types of accident or occurrence, vehicles involved, etc.
Modifiers that do not affect coding appear in parentheses after the condition.

3.2.3 Code numbers

The code numbers that follow the terms refer to the categories and subcategories
to which the terms should be classified. If the code has only three characters, it
can be assumed that the category has not been subdivided. In most instances
where the category has been subdivided, the code number in the Alphabetical

27
INTERNATIONAL CLASSIFICATION OF DISEASES

index will give the fourth character. A dash in the fourth position (e.g. O03.)
means that the category has been subdivided and that the fourth character
can be found by referring to the Tabular list. If the dagger and asterisk system
applies to the term, both codes are given.

3.2.4 Conventions
Parentheses
Parentheses are used in the Alphabetical index in the same way as in Volume
1, i.e. to enclose modifiers.

‘NEC’
NEC (not elsewhere classified) indicates that specified variants of the listed
condition are classified elsewhere, and that, where appropriate, a more precise
term should be looked for in the Alphabetical index.

Cross-references
Cross references are used to avoid unnecessary duplication of terms in the
Alphabetical index. The word ‘see’ requires the coder to refer to the other
term; ‘see also’ directs the coder to refer elsewhere in the Alphabetical index
if the statement being coded contains other information that is not found
indented under the term to which ‘see also’ is attached.

3.3 Basic coding guidelines

The Alphabetical index contains many terms not included in Volume 1, and
coding requires that both the Alphabetical index and the Tabular list should
be consulted before a code is assigned.

Before attempting to code, the coder needs to know the principles of

classification and coding, and to have carried out practical exercises.

The following is a simple guide intended to assist the occasional user of the
ICD.

1. Identify the type of statement to be coded and refer to the appropriate

section of the Alphabetical index. (If the statement is a disease or injury
or other condition classifiable to Chapters I–XIX or XXI–XXII, consult
Section I of the index. If the statement is the external cause of an injury
or other event classifiable to Chapter XX, consult Section II.)

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 3. How to use the ICD

2. Locate the lead term. For diseases and injuries, this is usually a noun
for the pathological condition. However, some conditions expressed as
adjectives or eponyms are included in the Alphabetical index as lead
terms.
3. Read and be guided by any note that appears under the lead term.
4. Read any terms enclosed in parentheses after the lead term (these
modifiers do not affect the code number), as well as any terms indented
under the lead term (these modifiers may affect the code number), until
all the words in the diagnostic expression have been accounted for.
5. Follow carefully any cross-references (‘see’ and ‘see also’) found in the
Alphabetical index.
6. Refer to the Tabular list to verify the suitability of the code number
selected. Note that a three-character code in the Alphabetical index with
a dash in the fourth position means that there is a fourth character to be
found in Volume 1. Further subdivisions to be used in a supplementary
character position are not indexed and, if used, must be located in
Volume 1.
7. Be guided by any inclusion or exclusion terms under the selected code,
or under the chapter, block or category heading.
8. Assign the code.
Specific guidelines for the selection of the cause or condition to be coded, and
for coding the condition selected, are given in Section 4.

29