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2006-01-10_01 Spontaneous rupture of AV fistula

(Rule GP, Chapter XIV)

[Question from Tanja Coric, Croatia:]

Dear colleagues, could you please give us your suggestions on this certificate?

Effusio sanguinis externa
Ruptura spontanea aneurysmae dilatationis AV-fistule arteriae cubitalis sin.
Insuffitientio renalis cronica (Hemodialysis) Supplement information: The patient had thrombosis of AV-fistule

I've read the question which raised by Lars Age to Mortality Forum (2005-11-01).

[Here is an attempt at translating the Latin terms into English:
External blood loss
Spontaneous rupture of AV-fistula aneurysm of left cubital artery
Chronic renal insufficiency (hemodialysis) /Lars Age]


[Comments:]

2006-02-07:

Brazil /Ruy Laurenti and Heloisa Di Nubila/:
We agree with chronic renal insufficiency (N18.9) as the underlying cause of death in this case. Our interpretation is that a chronic renal insufficiency (caused by?) imposed to make a fistula AV, needed to perform hemodialysis, and this fistula complicated with thrombosis and haemorrhage.

The death certificate would be codified so:
Part I a) External blood loss (=haemorrhage) - Y84.9
          b) (Thrombosis) rupture of AV-fistula aneurysm of left cubital artery (for hemodialysis) - T82.8
          c) Chronic renal insufficiency - N18.9

2006-01-31:

Canada /Patricia Wood/:
This is a very similar situation to the one presented by Lars Age in November last year, except that in this case there is no fall or other reason for the rupture of the arteriovenous fistula and the subsequent blood loss. I think that the underlying cause of death is the chronic renal insufficiency (ICD-10 code N189). The multiple cause codes are not quite as easy to discern. I think I might consider Y841, Kidney dialysis as the cause of abnormal reaction of the patient, or of later complication, etc.
and T808, Other complications following infusion, transfusion and therapeutic injection.

2006-01-17:

Slovenia /Jozica Selb/:
We in Slovenia would code in this case Chronic renal insufficiency as the underlying cause of death - N189.

2006-01-10_02 Sequences in perinatal deaths

(Rule P1, Chapter XVI)

[Question from Kathleen England, Malta:]

Dear Mortality Forum, I have a question about deaths in infants. In Malta we do not have a perinatal death certificate and use the standard "3-line" WHO death certificate to code deaths in infants. I am always unsure how to code deaths in infants.

E.g.:
If a death is seconday to intraventricular haermorrhage secondary to severe prematurity, according to Rule P1 prematurity should not the underlying cause of death. However, intraventricular haemorrhage was a consequence of the severe prematurity. In the case of using a normal death certificate (ie not perital), should the underlying cause of death be intraventricular haemorrhage?


[Comments:]

2006-02-28:

Malta /Kathleen England/:
I agree with Norway that these are quite confusing. In Malta we sometimes have additional information if an autopsy has been performed e.g. sometimes cause of death is hypoxia due to cord round neck.In this case we also chose the cord round neck as the UCD however I am not sure if this is correct.

2006-02-20:

Norway /Gunvor Ostevold/:
I am happy for this discussion about perinatal deaths because we are really struggling with this type of deaths! Maybe this is a topic for this years Regional Mortality Meeting in Latvia? In Norway we do not have a perinatal death certificate either and we use the standard certificate, but in addition to this we receive informations from The Medical Birth Registry which tells us about pregnancy and birth. In this case we would use the ordinary rules like you all are doing. BUT then we look to The Medical Birth Registry certificate trying to find out WHY this child had a premature birth and if we find any conditions covered by the codes P00- P02 we choose this as the underlying cause of deaths. Do other countries have this additional informations like us?

2006-02-07:

Brazil /Ruy Laurenti and Heloisa Di Nubila/:
We don't use "Perinatal Death Certificate" in Brazil. Rules P1-P4 are supposed to be used for answering the specific questions presented on it
a. Main disease or condition in fetus or infant;
b. Other diseases or conditions in fetus or infants;
c. Main maternal disease or conditions affecting fetus or infant;
d. Other maternal diseases or conditions affecting fetus or infant.

We would code P53.2, using the rules presented here by our colleagues.

2006-01-31:

Canada /Patricia Wood/:
In Canada we do not have a perinatal death certificate either and we use the standard medical certificate of cause of death too. In a death caused by an intraventricular hemorrhage due to severe prematurity, we would code the underlying cause of death as P523, Unspecified intraventricular (nontraumatic) hemorrhage of fetus and newborn. We would do this because of the note in ICD-10, Volume 2, 4.1.11: P07.- not to be used if any other cause of perinatal mortality is reported.

2006-01-17:

Slovenia /Jozica Selb/:
In this case we in Slovenia would code intra ventricular hemorrhage P523.

Sweden /Lars Age Johansson/:
Like in Malta, we do not have the perinatal death certificate. Also, we do not use the rules P1 - P4, because they are designed with the perinatal death certificate in mind and cannot easily be transferred to the general death certificate. So we use the ordinary rules (GP, selection rule 1-3, modification rule A-F) for all deaths reported on the general death certificate, including the perinatal ones. So here we would first select prematurity according to the General Principle, but then re-select the underlying cause as if prematurity had not been reported, because the Note on P07-P08 in "4.1.11 Notes for use in underlying cause mortality coding" tells us not to use P07-P08 if any other cause of perinatal mortality is reported. That would bring us to the intraventricular hemorrhage, as Jozica says.

2006-01-17_01 Code for four-wheeled motorcycles

(Chapter V)

[Question from Gunvor Ostevold, Norway:]

Four-wheeled motorcycles are nowadays more and more popular. What code do you use for this kind of vehicle? It can be used as a terrain motor-vehicle and as a ordinary road motor-vehicle. Is V86 suitable?


[Comments:]

2006-02-28:

Canada /Patricia Wood/:
If "four-wheeled motorcycles" in Norway are the same as "four-wheelers" or "four-wheeled ATVs" in Canada: We use ICD-10 code V86 for accidents involving occupants of four-wheeled all-terrain vehicles because the vehicles are "of special design to enable it to negotiate rough or soft terrain or snow" (as per ICD-10, 2003, Volume 1, page 990). If the accident occurs while the vehicle is being driven on a roadway, we use the appropriate fourth character (.0 - .3) to indicate a "traffic" accident.

2006-01-31:

Sweden /Lars Age Johansson/:
The Mortality Reference Group discussed the issue at its meeting in October 2006. We arrived at the conclusion that V86 is the best code available at the moment. However, as Gunvor says, four-wheeled motorcycles are getting more and more common, and are apparently also involved in many accidents.

Someone at the MRG meeting referred to them as "organ donor vehicles", which I think sums up the situation very neatly. We would probably need a separate code, but there isn't much free in space in the ICD, at least not in the right place. So the suggestion is to use V86, and hopefully we will be able to squeeze a separate code in sooner or later.

2006-01-17_02 Trivial condition leading to a complication, no mention of surgery

(Rule A)

[Question from Michelle White, USA:]

Perhaps this is a trivial question to the experienced coders, but for us, this trivial condition is not! What is the correct code for deaths where the originating cause is a trivial condition and there is no mention of medical or surgical complications?

Ia Pulmonary embolism
b None
c Left knee injury
II None

Should this be coded to X599, exposure to unspecified factor or to I269, pulmonary embolism? No information was available as to the mechanism or time frame of the knee injury, and there is no mention of the embolism being due to medical or surgical complications in the autopsy report.


[Comments:]

2006-04-11:

Sao Paulo Classification Centre /Ruy Laurenti/:
In Brazil, "impetigo" is not considered a trivial condition in infants up to one year of age.

2006-02-28:

Canada /Patricia Wood/:
Injury, knee is indexed to S899 which is not included in the list of Trivial Conditions (see Table H in NCHS Instruction Manual Part 2c) so the application of Rule B is not an issue on this particular certificate. And, because no external cause of the knee injury was specified, the underlying cause of death would be X59.

According to Rule B, if the tentative underlying cause is a trivial condition and another more serious condition is reported, the underlying cause of death is reselected as if the trivial condition were not reported.

In Canada, we don't apply Rule B if the trivial condition is reported as causing any other condition. So, if the tentative underlying cause of death is a trivial condition (and death is not the result of an adverse reaction to treatment of the trivial condition), we will reselect the underlying cause if there is a more serious condition reported AND the trivial condition did not cause it!

For example:
Ia) impetigo
b)
c)
d)
II septicemia

Code to septicemia (A419). Impetigo, selected by the General Principle, is ignored.
Ia) septicemia
b) impetigo

Code to impetigo (L010). The trivial condition, selected by the General Principle, is not disregarded as it is reported as the cause of another condition. Rule B is not applied.

2006-01-31:

Sweden /Lars Age Johansson/:
This difficulty is covered by an update to Rule A in Vol 2, available at the WHO website, http://www.who.int/classifications/committees/ICDCombinedUpdates.pdf.

According to this update of Rule A, when a trivial condition is reported as the cause of a serious complication, it should be accepted as the underlying cause. In other words, because it has caused a serious complication it cannot be considered trivial - in that particular case.

This update was decided in October 2002 and takes effect with the 2006 version of ICD-10.

2006-01-31_01 Bolus due to Down's syndrome acceptable sequence

(Rule GP, Chapter XX)

[Question from Monika Diebold, Switzerland:]

Dear all, how do you proceed in the following situation?
"Suffocation by bolus due to Down syndrome" (or similar: Suffocation by bolus due to cerebrovascular accident). As I interpret the rules we're not allowed to take an external cause as being due to any non-external cause except for epilepsy (4.2.2(m)). From the medical point of view I would accept causality.

Thank you for your help.


[Comments:]

2006-02-07:

Canada /PatriciaWood/:
Monika is quite right about the original application of the "highly improbable" causes of accidents (ICD-10, Volume 2, 4.2.2.(m)). In ICD-10, Second Edition this guideline has been updated (4..2.2(n)) to exclude "aspiration of food (liquid or solid) of any kind (W79) reported as due to a disease that affects the ability to swallow," from the "highly probable" causes of accidents.

In the first case I would code the underlying cause of death to Q909 and the multiple causes of death to T179 and W79.
In the other case, I would code I64 as the underlying cause of death along with the same multiple causes of death.

2006-02-07_01 Bazex syndrome

(Chapter XII)

[Question from Josie Mornie, Belgium]:

Dear all, how do you code a Bazex Syndrome (=acrokeratosis paraneoplastica). It is a rare dermatosos associated with carcinomas of the upper aerodigestive tract. We had a case with cancer as the underlying cause, and Bazex as the immediate cause.


[Comments:]

2006-02-28:

Canada /Patricia Wood/:
For consideration: The NCHS index includes Paraneoplastic Syndrome at L53.9, Erythematous condition, unspecified. Would that be suitable for Bazex Syndrome?

Japan /Tadahiro & Takae Ootsu/:
A Japanese popular textbook of dermatology shows that Bazex syndrome is classified as a inherited keratosis. And, from references below, we think that Bazex syndrome should code Q828 as inherited keratosis palmaris et plantaris.

References
1) http://dermatology.cdlib.org/101/case_presentations/acrokeratosis/rao.html
2) J Med Genet. 2005 Nov;42(11):811-9.
Genetics of skin appendage neoplasms and related syndromes.
Lee DA, Grossman ME, Schneiderman P, Celebi JT.
Department of Dermatology, Columbia University Medical Center, New York, USA.

In the past decade the molecular basis of many inherited syndromes has been unravelled. This article reviews the clinical and genetic aspects of inherited syndromes that are characterised by skin appendage neoplasms, including Cowden syndrome, Birt-Hogg-Dube syndrome, naevoid basal cell carcinoma syndrome, generalised basaloid follicular hamartoma syndrome, Bazex syndrome, Brooke-Spiegler syndrome, familial cylindromatosis, multiple familial trichoepitheliomas, and Muir-Torre syndrome.

2006-02-07_02 Wernicke-Korsakoff syndrome

(Chapter V)

[Questions from Tadahiro Ootsu, Japan:]

Wernicke-Korsakoff syndrome

The code of Wernicke syndrome is E512. And, the code of Korsakoff syndrome is F10.6. How about the code of Wernicke-Korsakoff syndrome ?


[Comments:]

2006-04-11:

Sao Paulo Classification Centre /Ruy Laurenti and Heloisa Di Nubila/:
According to clinical descriptions, Wernicke syndrome is an acute manifestation of tiamine deficiency caused by alcohol abuse, inducing encephalopathy characterized by confusional state, disorientation, oftalmoplegia, nystagmus, diplopia and ataxia. Korsakoff syndrome is a late manifestation (sequelae) after recuperation of acute features, and is characterized shortly by lost of memory for recent facts and confabulation.

So, we would use the code for Korsakoff syndrome, F10.6.

2006-02-28:

Canada /Patricia Wood/:
For consideration: The NCHS index includes Wernicke-Korsakoff Syndrome at F106.

2006-02-07_03 GIST tumour

(Chapter II)

[Questions from Tadahiro Ootsu, Japan:]

GIST (Gastrointestinal stromal tumor) A paper in my hand (in English) shows that about 40-70% GISTs arise from the stomach, 20-40% arise from the small intestine, 5-15% from the colon and rectum, and GISTs can also be found in the esophagus (5%). If I can see the region and size (level of malignancy) of GIST, it is not so difficult to decide a code. But, if only GIST, which should I code, C269 or D139?


[Comments:]

2006-03-07:

WHO /Robert Jakob/:
ICD-O-3: GIST, NOS morphology is coded 8936/1 uncertain behaviour. With the knowledge that this tumour usually affects some part of the gastrointestinal tract D37.9 is the correct choice.

2006-02-28:

Canada /Patricia Wood/:
In English, the ICD-10 lead term "Tumor" is cross-referenced to Neoplasm, uncertain behaviour, and "stromal" is not indexed as a modifier. Therefore, I think that GIST, or gastrointestinal stromal tumour, when not specified as to behaviour, should be coded to D379, Neoplasm of uncertain or unknown behaviour of digestive organ, unspecified.

Of note, ICD-O-3 includes a morphology code for GIST, NOS with a /1, uncertain or unknown behaviour

Also of note, the NCHS index includes several "Tumor, stromal" entries, such as:
Tumor
- stromal
. . .
- - gastrointestinal D481
- - - benign D214
- - - malignant C494
. . .
These NCHS codes are for neoplasms of connective and other soft tissue.

2006-02-07_04 Barrett's esophagus

(Chapter II, XI)

[Questions from Tadahiro Ootsu, Japan:]

 Barrett esophagus and Barrett esophagus cancer A paper in my hand (in Japanese) shows that Barrett esophagus is caused by gastroesophageal reflux.

So, can I code K210 for Barrett esophagus?
And, for Barrett esophagus cancer, which code should I choose, C152 or C155?


[Comments:]

2006-03-07:

WHO /Robert Jakob/:
The adenocarcinoma of the oesophagus is frequently also called Barrett carcinoma as it develops based on the Barrett metaplasia. Present definitions of the latter refer to an extension of the metaplastic areas in the esophagus more then 2(3)cm oral to the gastroesophageal junction. With respect to this definition and to the definitions and descriptions in the Blue Book "Tumours of the Gastrointestinal Tract", IARC, I recommend to use C15.5.

2006-02-28:

Canada /Patricia Wood/:
As of January 2006, ICD-10 includes code K227, Barrett's esophagus. I am not sure that Barrett esophagus cancer would be properly diagnosed as a cause of death, so I would be inclined to query the certifier for more information about the site of the primary malignant neoplasm (or I'd code it to C159).

2006-02-07_05 IPMT

(Chapter II)

[Questions from Tadahiro Ootsu, Japan:]

IPMT (Intraductal papillary mucinous tumor of the pancreas) A paper in my hand (in Japanese) shows that IPMT may have various pathological observation, hyperplasia, adenoma, dysplasia, in situ carcinoma, or advanced cancer.

Can I use C253 for IPMT C253 or should I use D136?


[Comments:]

2006-03-07:

WHO /Robert Jakob/:
The name IPMT says tumour and describes a category of histological entities including every behaviour from benign to malignant. These tumours normally occur in the pancreas. So IPMT means tumour of the pancreas of uncertain behaviour. The appropriate code would be D37.7

2006-02-07_06 Autoimmune pancreatitis

(Chapter XI)

[Questions from Tadahiro Ootsu, Japan:]

Autoimmune pancreatitis. Is it correct to use the code K861 for autoimmune pancreatitis?


[Comments:]

2006-02-28:

Canada /Patricia Wood/:
As of January 2006, ICD-10 includes fourth character subcategories for K85, Acute pancreatitis. Autoimmune pancreatitis is not one of the fourth character specifications so I would code it to K858, Other acute pancreatitis

2006-02-07_07 MALT lymphoma

(Chapter II)

[Questions from Tadahiro Ootsu, Japan:]

 MALT lymphoma

A paper in my hand (in Japanese) shows that MALT lymphoma is B cell low malignancy lymphoma caused in mucosal related lymph organization, for example, salivary gland, thyroid or digestive tract. Which code should I use for MALT lymphoma? C857?


[Comments:]

2006-03-07:

WHO /Robert Jakob/:
This entity is definitely not compatible to the ICD-10. In the discussions for the revision proposal with Stein, coauthor of the Blue Books and expert in lymphomas and leukaemias the fact it was a B-cell Lymphoma seemed to be the relevant detail to be retained. Accordingly it should be coded C85.1.

2006-02-07_08 ANCA nephritis

(Chapter XIV)

[Questions from Tadahiro Ootsu, Japan:]

Antineutrophil cytoplasmic antibody (ANCA) related nephritis Papers in my hand (in Japanese) show that ANCA related nephritis has three types, only nephritis, nephritis with lung hemorrhage or interstitial pulmonary disease and nephritis with other organ disorder. I think that it is suitable to choose M31.8 for ANCA related nephritis as a vasculitis.

However, a report shows that within 1,342 cases of rapidly progressive glomerulonephritis in Japan, more than 65% of these cases were due to ANCA related nephritis and had organizational observation of crescentic glomerulonephritis. So, it may also be suitable to code N017. I would like to know the point of view for coding ANCA related nephritis. Please advise me.


[Comments:]

2006-03-07:

WHO /Robert Jakob/:
ANCA is an antibody, a lab finding, associated apparently with a wide range of diseases (Wegener, Churg-Strauss, polyangiitis, systemic lupus erythematodes, rheumatoid arthritis, primary sclerosing cholangitis, autoimmune hepatitis, inflammatory bowel disease). A nephritis can be the only symptom or one of several symptoms or not occur at all depending on the underlying disease associated with the ANCA. If the nephritis is the only manifestation, the disease itself, I would suggest to code with the specified type of nephritis. If there is evidence that the nephritis has been part of a systemic disorder (e.g. Wegener) the corresponding M code (e.g. M30-M36) should be used.

2006-02-20_01 Torsion of ovarian pedicle

(Chapter II, XIV)

[Question from Tadahiro Ootsu, Japan:]

Today, I was asked by my colleague about torsion of ovarian pedicle ( N835 ). I researched several papers and found that torsion of ovarian pedicle must have happened with ovarian tumor, for example, mature cystic teratoma.

So, I wonder should we choose N835 or D27? In Japan, we decide now that only mention of torsion of ovarian pedicle is coded N835, and if we can see any ovarian tumor, we choose D27. But, I think that we should arrange this matter like the excludes of K317 (polyp of stomach and duodenum) or K635 (polyp of colon). And, I could not find torsion of ovarian pedicle with non-tumor disease, for example, follicular cyst of ovary. How about it?


[Comments:]

2006-02-28:

Canada /Patricia Wood/:
If torsion of ovarian pedicle was certified as a cause of death, I would code N835, Torsion of ovary, ovarian pedicle and fallopian tube. If it were certified as due to an ovarian tumour, I would still code the torsion of ovarian pedicle to N835, and I would code the ovarian tumour to the most appropriate code for it. Then I would apply the General Principle to select the ovarian tumour as the underlying cause of death.

Sweden /Lars Age Johansson/:
I agree with Patricia, and I wouldn't code to ovarian tumour unless that condition is explicitly mentioned on the death certificate. It seems to me that the cases of K31.7 (polyp of stomach and duodenum) and K63.5 (polyp of colon) are slightly different, because the exclusion is there from an unspecified type of polyp to a more specific one. Here, if we would add an exclusion from N83.5 (torsion of ovary, ovarian pedicle and fallopian tube) to ovarian tumour, we would lose information on the torsion itself, and code only the cause of the torsion. That would, of course, be entirely in line with the selection rules for mortality, but perhaps our morbidity colleagues would want a possibility to code the torsion itself.

2006-02-28_01 Suspected suicide, intent not stated

(Chapter XX)

[Question from Bridget Allison, Australia:]

In cases of suspected suicide (as reported by police), coroners are notably silent when it comes to stating intent on their findings for young people. (Nor is intent stated on the death certificate). Additional police or agency documentation may indicate that there was a precipitating incident (eg argument with parents) or a history of self-harm or depression, however the death certificate and findings tend to read as in the following example:

Death certificate: 1a) Hanging
Coroners findings: 15 year old female found hanging from belt tied to ceiling beam.

Both MMDS and the index (volume 3) default to accidental for hanging unless there is any further information available from the entity legally responsible for certifying the death (in this case the coroner).

I would like to ask for your opinion on how you would code this or similar situations.


[Comments:]

2006-03-20:

Malta /Kathleen England/:
In Malta suicide is never stated on death certificate therefore in cases of hanging, fall from height and other types of possible suicide we discuss these cases with pathologist responsible for autopsy as well as police who would have evidence of circumstences of death. However usually death due to hanging is classified as suicide unless we have evidence for the contrary.

PAHO/ Roberto Becker/:
This discussion is not new, and as I mentioned in other occasions, what we should well determine is the difference between a "legal" database and a statistical/epidemiological one. I fully agree with Filippa's comments.

In terms of coding, I would accept information from any source I can really trust. Regardless what "legally" was defined as the intention.

On the other hand, the note before the group of "Undetermined intent", in fact, makes comparisons very difficult, because the interpretation varies form one to another country and even within countries. Look at the proportion of external causes coded as "undetermined intent" in recent years:
México 5.9 %, Chile 30.8%, Greece - % (zero!?), Hungary 1.5%, Poland 30.0% It is quite obvious that the interpretation is different on those countries.

I think that considering as accidents when there is no mention of intentionality nor investigations made (as indicated in the mentioned note) is not a good idea. This group of codes should rather be used as Unspecified (ignored) intent as well as undetermined. Also, Vol. 2, 4.2.13 states that expressions such as "apparently", "presumably", possibly", etc. should be ignored. And this is valid for external causes too.

In parallel, it is the same situation when a death certificate shows a diagnostic of a disease under epidemiological surveillance (Measles or polio, for example) and we get the results of lab tests with other diagnostic. I would code the final results, after a discussion with or a query to the certifier.

Sweden /Lars Age Johansson/:
I fully agree with Roberto that it would be a good idea to distinguish between cases where the cause of injury is unknown, and cases where the circumstances are known, but the intent - if any - could not be established. At the moment, however, the ICD doesn't offer that possibility, and it is quite clear that according to the ICD instructions "unknown" causes should be coded as accidents. This is evident both from the instructions in Vol 1 and from the indexing of unspecified external events in Vol 3. If this is a good idea or not could be debated, of course, and it is obvious from Roberto's examples that it is difficult to persuade some countries to follow these instructions. If Chapter XX is to be revised, introducing a section of codes for "unknown" causes of injury could be a way to deal with this. Right now, however, there are no such codes in Chapter XX, and I firmly believe that until we have Chapter XX codes for "unknown", we should follow the current ICD instructions.

2006-03-07:

Australia /Filippa Pretty/:
I understand the problem. We get "asphyxia from hanging".

Here in NSW CDRT we code the suicide if the supporting evidence i.e. P79A, interviews, police statements all indicate/state it. We also take into account the physical environment i.e. its hard to "accidentally" fall from inside the roof girder to a ready made noose without some intent to self harm. (sorry for sounding so morbid and detached). We also code out the extenuating circumstances i.e. exam pressure, peer isolation, family / social issues to try and find a thread as many are not leaving notes these days. If there is any reason for doubt then the "unknown intent" is assigned.

Canada /Patricia Wood/:
Bridget is right, unless otherwise specified as to intent, hanging (and most other external causes) is indexed to "accidental". And MMDS, being based on the Classification, makes the same assignment. In the absence of a statement of intent or manner of death, that is how I would code such a certificate.

This may be more a certification issue than a classification issue. A coroner's investigation is supposed to examine and determine, if possible, the exact circumstances of a death. If the coroner is unable to determine the intent and certifies this, we can code that to undetermined. However, "unspecified" is not synonymous with "undetermined".

Sweden /Lars Age Johansson/:
I agree with Patricia, and I think it is very important to note the difference between "unspecified" and "undetermined". In fact, the ICD instructions on the "undetermined" codes (Y10-Y34) say that they should be used when "the available information is insufficient to enable a medical or legal authority to make a distinction between accident, self-harm and assault". So, as Patricia says, this means that we, as coders, should use

Y10-Y34 only if the police, coroner, forensic specialist etc has stated that the intent cannot be determined. We shouldn't use Y10-Y34 if the death certificate or forensic report doesn't contain sufficient information.

According to the ICD, such cases should be coded as accidents.

2006-02-28_02 Space-occupying lesion of brain

(Chapter II, VI)

[Question from David Lidsky, Israel:]

I wonder whether people in other countries encounter the terms "space occupying lesion" or "SOL." in death certificates. And if so how do they code them? I have made a Google "site" search of the complete index of the Forum and have not found a single mention of these terms. Nor have I been able to find them in ICD-10 volume 3, or among the ICD-10 cumulative updates.

As far as I am aware, "space occupying lesion" occurs only once in the ICD, in the definition of R90.0: "Intracranial space-occupying lesion." We have decided to extend that code to cover "space-occupying lesion of the brain". And by analogy we use R91 for "space-occupying lesion of the lung"

In all these cases the ICD term seems to refer to a three dimensional lesion of undetermined etiology, for which the main evidence is in imaging studies; or more generally, a "lesion occupying space".

However we are not sure that that is what our certifiers mean, either in the case of lesions of brain or lung, or in those of other parts of the body. We strongly suspect that many doctors mean by SOL not a "lesion occupying space", but: "malignant tumor", or "a lesion highly suspicious as a malignant tumor". From time to time we even encounter "space occupying lesion with metastases".

This is in essence a problem in the lexicology of medical English (or at least Israeli medical English). Perhaps we should survey doctors as to their usage of these terms. Though I suspect that we would receive the reply which they feel they ought to give ( "a lesion occupying space"), rather than one reflecting their everyday usage.

In addition, we wonder whether we ought to code a malignant tumor when there is no explicit mention of malignancy on the certificate, even if we are convinced that our surmise concerning the usage of these terms is correct.

Any help will be gratefully received.


[Comments:]

2006-03-07:

Canada /Patricia Wood/:
I haven't noticed that Canadian certifiers are inclined to use the term "space-occupying lesion" of a site or organ as a cause of death. However, I notice that Lesion, intracranial is cross-referenced to Lesion, brain which is indexed to G939, so I am not sure that I would code a space-occupying lesion of the brain to R900. Whether a lesion of the brain is specified as space-occupying, or not, I would code it to G939.

It seems to me that the index is a bit inconsistent in that Lesion, organ or site NEC is cross-referenced to see Disease, by site and YET Lesion, lung is coded to R91 while Lesion, pulmonary is coded to J984.

In the situation where the space-occupying lesion is mentioned "with metastases", I would interpret this as an implication of malignancy and code accordingly. However, I would not code a space-occupying lesion as a malignant neoplasm without an implication of malignancy and/or clarification from the certifier.

Sweden /Lars Age Johansson/:
In Swedish medical records we sometimes see the expression "expansive process", which perhaps is equivalent or similar to "space-occupying lesion". It might refer to a tumour, of course, but I agree with Patricia that we shouldn't code to a malignancy unless there is a clear indication that the "space-occupying lesion" was in fact a malignant tumour. Like Patricia, I would use the code for "unspecified disorder of brain", G93.9.

2006-02-28_03 IPEX syndrome

(Chapter III)

[Question from Monique Differding, Luxemburg:]

Dear all, I need your help.

A young man ( 20 years old) died with
1. IPEX syndrom
2. Acute respiratory failure
3. Renal failure
[Codes?]


[Comments:]

2006-03-07:

Canada /Patricia Wood/:
I learned that IPEX is an abbreviation of Immune dysregulation, Polyendocrinopathy, Enteropathy and X-linked inheritance, but I'm really stumped for a classification decision. One site I was reading said that the enteropathy is often the first symptom and is present in virtually every case reported, but I'm still not sure how best to code it. Hopefully someone else will have some insight!

Japan /Tadahiro Ootsu/:
According to reference below, IPEX ( Immunodysregulation Polyendocrinopathy Enteropathy X linked ) syndrome seems that autoimmunity may stem from a lack of working regulatory T cells. So I think that the code for IPEX syndrome is D831. Therefore:

A young man (20 years old) died with
1. IPEX syndrom: D831
2. Acute respiratory failure: J960
3. Renal failure: N19
UC: D831 (to apply rule 2)

Reference
http://jmg.bmjjournals.com/cgi/content/full/39/8/537

2006-03-07_01 Pregnant woman assaulted, baby died

(Chapter XVI, XX)

[Question from Sue Walker, Australia:]

Hi colleagues - this query has been sent to us for comment. Any thoughts on it?

The mother of the deceased child was assaulted and was admitted to hospital as a result of injuries received. The deceased was 21 weeks pregnant at the time of admission. The condition of the mother of the deceased baby worsened due to traumatic pancreatitis and the deceased baby was born prematurely as a result. The baby was breathing by herself at birth however breathing was laboured. She was deemed too young to be revived and continued on in this way until she died.

Is this coded as assault because the event that started the chain of events etc etc was the assault while in-utero?

We are guessing that the child would get an underlying cause of death of P00.5, Fetus and newborn affected by maternal injury but could you assign multiple cause codes to identify that the death was due to an assault on the mother?


[Comment:]

2006-03-20:

Canada /Patricia Wood/:
I agree that the underlying cause of death for the baby is P005, Fetus and newborn affected by maternal injury. But, I think that's all I'd code. I would not assign multiple cause codes to specify the external cause that produced the injury that lead to the mother's death. Assigning a Y85-Y09 code might suggest that the baby was assaulted which is not the case. A fine line I'll admit, but I'd stay with P005 only.

PAHO /Roberto Becker/:
I would certainly assign multiple cause codes to identify the external cause (on the mother) accidental or intentional, with the corresponding code.

I think that using P00.5 plus an external cause code makes clear what happened.

2006-03-07_02 Firework factory explosion

(Chapter XX)

[Question from Kathleen England, Malta:]

I have a question about deaths due to firework factory explosion. In Malta we had 3 deaths due to firework factory explosion while these men were working inside the factory. I am not sure if I should code discharge of firework W39 or Explosion of other material (factory) W40. Since if I code W40 i would loose information on the source that is fireworks. On the other hand W39 is discharge of firework, since though they were working on the fireworks they were not discharging them at the time.


[Comments:]

2006-03-28:

PAHO /Roberto Becker/:
The only way to have all information is multiple coding, but in this case the underlying cause is W40. W39 is the additional code, together with the specific injuries, if known.

2006-03-20:

Canada /Patricia Wood/:
I think that W40 is the better code in the situation Kathleen describes.

Although it was a fireworks factory, using W39 would suggest that the three deaths were caused by the discharge of fireworks, which is not really what happened. Many years down the road, researchers might draw the wrong conclusion from Malta mortality statistics if W39 is used.

Japan /Tadahiro Ootsu & Reiko Matsumura/:
In Japan, we code W406 for fireworks factory explosion because we regard that the information of explosion is more important. And, we use place of occurrence code .6 ( please see Volume 1, second edition, 2004, p.979-83 ), so at least we do not lose the information of industrial and construction area. However, in case of W396, it seems that 6 means workshop rather than factory.

According to our national datas, in 2001-2003, W40(W406) and W39 were 21(11) and 0, 3(2) and 1, 18(13) and 12 cases.

For a change, please enjoy pictures of Japanese fireworks ! http://www.ne.jp/asahi/art/fireworks/

2006-03-21_01 Disuse syndrome

(Chapter XIII, XVII)

[Question from Tadahiro Ootsu, Japan:]

Disuse syndrome:
In Japan, we have used M625 for disuse syndrome. However, Japanese literature shows that disuse syndrome has various symptoms, joint or bone, circulatory or respiratory or autonomic nervous system, or mental disorders, etc. in addition to muscle wasting and atrophy. Therefore, it may be necessary to change the code. Please advise me the following two points.
1) In your country, do you use the term of disuse syndrome ordinarily ?
2) If your answer is yes for 1), how about a suitable code for disuse syndrome?


[Comments:]

2006-03-28:

WHO /Robert Jakob/:
While searching literature, I found an excellent overview on Pubmed (http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1011199). It might not be a very recent publication, but provides an excellent summary.

The assignment of the code M62.5 reflects only the most visible symptoms of inactivity, though nearly all body systems are concerned. Every condition causing inactivity may cause the syndrome. The category M62.5 refers to muscle wasting and atrophy and this does include conditions that are not always trivial.

However, there is no code for disuse or disuse syndrome yet in the ICD. The M62.5 is Disuse ATROPHY, as the index and tabular list say; and not just disuse. Disuse causing something goes to the organ system affected. If no specific system affected is mentioned and no specific cause is mentioned (= Disuse Syndrome), it has to go to no organ and no cause and becomes a generic symptom and sign. Tadahiro may wish to enquire the full chain: clinical use, certification behaviour, coding to clarify what is happening in Japan.

2006-03-21_02 Lupoid hepatitis

(Chapter XI)

[Question from Tadahiro Ootsu, Japan:]

Lupoid hepatitis
In Japan, it is general to regard lupoid hepatitis as an autoimmune hepatitis. But, Volume 1 ( ICD-10, second edition, 2004 ) p.558-9 shows that lupoid hepatitis is K732 and autoimmune hepatitis is K754, so we are confused. While, the term of lupoid hepatitis may be old-fashioned. Then, please advise me the following two points.
1) In your country, do you use the term of lupoid hepatitis recently ?
2) If your answer is yes for 1), do you choose K732 or K754 or the other code for lupoid hepatitis?


[Comments:]

2006-04-11:

 

Sweden /Olafr Steinum/:

For your information:

CAH (Chronic active hepatitis) (and its contrast CPH - Chronic persistant hepatitis) is a term used amongst pathologists, describing a certain histopathological picture. It is not restricted to autoimmune hepatic disease, as we have used the term during the 1980-ies and part of the '90-ies to describe pathology in chronic virus hepatitis B and C. Recent terminology, however, focus on degree of inflammation and fibrosis in the liver instead, but the older terms may still appear occationally.

2006-04-04

WHO /Robert Jakob/:
After reviewing some publications, (1,2,3,4) I come to the same conclusion as Tadahiro: Lupoid Hepatitis is just an old-fashioned term for autoimmune hepatitis. To increase the confusion, K71.5 mentions also the lupoid hepatitis. Historically the evolution of terms seems to be "chronic active hepatitis"(CAH); with the development of new laboratory tests a subtype of the CAH could be identified, the "lupoid hepatitis" (similar antibodies to those occuring in lupus erythematodes were described). With the evolution in science, it was renamed "autoimmune hepatitis" 1965 to be clearly distingishable to the hepatitis occuring with "systemic lupus erythematodes". The IND does not provide any clarification of these terms. At present both terms are merrily mixed, used as synonyms or even "lupoid h." as subtype of "autoimmune h.".

I recommend the following steps:
1.) Raise the issue in the MRG (and the URC)
2.) Inquire with specialists in the field to clarify the terminology and the content (e.g. definitions...).
3.) Test the effects on statistics, if the terms are all put into K75.4 4.)Depending on the results propose an update to Volume 1 and 3 of ICD-10 This may look cumbersome but will prevent inconsistencies in longitudinal statistics nationally and internationally and in a way harmonize the coding behaviour.

1) Manns MP, Vogel A; Autoimmune hepatitis, from mechanisms to therapy; Hepatology (Baltimore, Md.), VOL: 43 (2 Suppl 1), p. S132-44 /2006 02/

2) Poulsen LO, Thulstrup AM, Mellemkjaer L, Vilstrup H, Sørensen HT; Mortality and causes of death in patients with "lupoid hepatitis." A long-term follow-up study in Denmark; Danish medical bulletin, VOL: 49 (3), p. 263-5 /2002 08/

3) Ohnami K, Komatsu M, Tsukamoto K, Sasaki K, Satoh H, Umetsu Y, Kikuta Y, Miura M, Moteki S; A case of autoimmune hepatitis (lupoid-type) associated with positive anticentromere antibody and prevalent seroimmunological abnormalities in relatives; Nippon Shokakibyo Gakkai zasshi The Japanese journal of gastro-enterology, VOL: 90 (12), p. 3063-9 /1993 12/

4) Storch W; Immunopathogenetic aspects of chronic hepatitis; Zeitschrift für die gesamte innere Medizin und ihre Grenzgebiete, VOL: 32 (3), p. 65-70 /1977 02 01/

2006-03-28:

Canada /Patricia Wood/:
I am not sure that lupoid hepatitis is a cause of death that we encounter often, but as it is indexed to K732 where it appears as an inclusion term, I would code it to K732.

Sweden /Lars Age Johansson/:
If I get this right, "autoimmune hepatitis" is a broader group than "chronic active hepatitis". That would mean that the title of K75.4 should perhaps be "other and unspecified autoimmune hepatitis", to make it clear that specific kinds of autoimmune hepatitis, such as chronic active hepatitis, might belong to some other group in the classification. But again, I am far from sure about this, and we would probably need to know more about why the code

K75.4 (autoimmune hepatitis) was introduced back in 1997, and what kinds of hepatitis it was intended for. I don't know if Robert has that information available - according to what I was told some time ago the WHO has archived much of the documentation on ICD, and it might not be easy to retrieve.

WHO /Robert Jakob/:
After reviewing some publications, I come to the same conclusion as Tadahiro: Lupoid Hepatitis seems to be just an old-fashioned term for autoimmune hepatitis. To increase the confusion, K71.5 mentions also the lupoid hepatitis.
I recommend the following steps:
1.)Raise the issue in the MRG (and the URC)
2.)Inquire with specialists in the field to clarify the terminology and the content (e.g. definitions...).
3.)Test the effects on statistics, if the terms are all put into K75.4 4.)Depending on the results propose an update to Volume 1 and 3 of ICD-10 This may look cumbersome but will prevent inconsistencies in longitudinal statistics nationally and internationally and in a way harmonize the coding behaviour.

2006-03-21_03 Idiopathic CD4 T-cell lymphocytopenia

(Chapter II)

[Question from Tadahiro Ootsu, Japan:]

Idiopathic CD4 T-cell lymphocytopenia From the reference below, I think that the code for idiopathic CD4 T-cell lymphocytopenia is D728. Is it correct? If not, please tell me correct code.
Reference: http://www.eymj.org/abstracts/viewArticle.asp?year=2005&page=173


[Comments:]

2006-03-28:

Canada /Patricia Wood/:
I agree that D72.8 is a suitable code for idiopathic CD4 T-cell lymphocytopenia

2006-03-21_04 Burns from sulphuric acid

(Chapter XIX, XX)

[Question from Mónika Bene, Hungary:]

Dear all, Please, give us some suggestions for the following case. Which ICD-10 codes should we assign?
Female, 36 years
I. a) Septicaemia
I. b) Eroding caused by sulphuric acid
I. c) She poured a large quantity of sulphuric acid on her body – Accident


[Comments:]

2006-03-28:

Canada /Patricia Wood/:
Multiple causes: A419/T304/X49 Underlying cause: X49

I would select X49, Accidental poisoning by AND EXPOSURE TO ... corrosive aromatics, acids and caustic alkalis. I found the nature of injury code under Corrosion (chemical).

Japan /Tadahiro Ootsu and Reiko Matsumura/:
In Japan, we code external causes conforming to the instruction that causes of death should preferably be tabulated according to both Chapter XIX and Chapter XX (please see Volume 1, second edition, 2004, p.977), so we will code T542 and X44 for this case.

Sweden /Lars Age Johansson/:
In Vol 3 "Burn, caustic liquid, substance" is indexed to X49, with a "See also" reference to the table of drugs and chemicals. There, sulphuric acid is indexed to X49. Since the injury is specified as eroding, I think that T30. 4 is a better code for the injury caused by the acid than a code in the block for poisonings.

2006-03-21_05 Drowning after falling into well

(Chapter XIX, XX)

[Question from Kathleen England, Malta:]

Dear Mortality Forum,

I have a question about 2 deaths due to drowning:
In the first case a person drowned after falling into a well.
Should the first case be coded as drowning following fall into water? or should these be coded differently, and which would be best code as there is no specific code.


[Comments:]

2006-03-28:

Canada /Patricia Wood/:
There is an exclusion note at Falls (W00-W19) for fall into water (with drowning or submersion) (W65-W74), so I would select W73, Other specified drowning and submersion.

Japan /Tadahiro Ootsu and Reiko Matsumura/:
Drowning after falling into well: We will code T751 and W73 for this case.

2006-03-21_06 Drowning after car accident

(Chapter XIX, XX)

[Question from Kathleen England, Malta:]

Dear Mortality Forum,

I have a question about 2 deaths due to drowning:
In the second case a person drowned after the car he was driving went off road and ended up in the sea.
In the second case should this be coded as a traffic accident as underlying cause or drowning falling a fall.


[Comments:]

2006-03-28:

Canada /Patricia Wood/:
There is an exclusion note at Accidental drowning and submersion (W65-W74) for transport accidents, so I would select V485, Car driver injured in noncollision transport accident, traffic. This example makes an excellent case for coding and tabulating a nature of injury code as well as the underlying (external) cause of death code. That way we would also know that this person drowned, something that is not reflected in V485.

Japan /Tadahiro Ootsu and Reiko Matsumura/:
Drowning after car accident: We will code T751 and V480 for this case.

2006-03-28_01 Code for syndromic face

(Chapter XVII)

 [Question from Augusto Hasiak Santo, Brazil:]

Please submit to discussion the adequate coding of the term "syndromic facies". Some authors also designate this condition as "funny looking face".

Others consider that these terms are no longer helpful. It is meant to describe structural abnormalities of the craniofacial complex and congenital malformations. Would it be possible to use the code Q18.9 or Q87.0?


[Comments:]

2006-04-11:

PAHO /Roberto Becker/:
I got the same question on Forum-CIE and, so far, two answers agreeing to code Q87.0.

However, I found many references of "syndromic facies" in several languages, but with different descriptions. Various of the descriptions do not include any facial abnormality or deformation. Thus, I am not sure that there is only "one" such syndrome. According to what I saw, the code could be:
Q10.3 or Q87.0 or Q87.1 or Q93.4 or Q18.9 or Q89.7 and some others. I think that for now I would code Q89.9.

2006-04-04:

Canada /Patricia Wood/:
I looked through some older versions of ICD thinking that I may find a reference to "funny looking face" but I didn't. Of the two codes suggested, I prefer Q870, Congenital malformation syndromes predominantly affecting facial appearance. The diagnosis itself is making a reference to the appearance of the face, so Q870 seems suitable.

2006-03-28_02 Code for chronic thromboembolic pulmonary hypertension

(Chapter IX)

[Question from Tadahiro Ootsu, Japan:]

I would like to know the suitable code for chronic thromboembolic pulmonary hypertension, or, using the literal translation for Japanese name of the disease; idiopathic chronic pulmonary thromboembolism, pulmonary hypertension type. Would it be I269, I272 or some other code? Please advise me.


[Comments:]

2006-04-04:

Canada /Patricia Wood/:
I think that both codes may be necessary to properly code this cause of death: I272, Other secondary pulmonary hypertension and I269, Pulmonary embolism without mention of acute cor pulmonale. This is what is referred to as a multiple one-term entity in the NCHS instruction manuals; a cause of death consisting of two or more contiguous words for which the Classification does not provide a single code for the entire entity but does provide a single code for each of the components.

2006-03-28_03 Fall due to alcoholic intoxication

(Chapter XX)

[Question from Kathleen England, Malta:]

I have a question about 2 deaths which occurred due to fall after alcohol intoxication. In both cases the fall would not have occurred if they were not drunk. However they died as a result of injuries sustained in the fall.

Should these be coded as falls or as a result of the alcohol intoxication?


[Comments:]

2006-04-11:

Sweden /Olafr Steinum/:
Could it be possible to assign to the fall, but add a code from Y90-Y91 to state that there was alcohol intoxication involved?

2006-04-04:

Canada /Patricia Wood/:
There's a note in ICD-10 Second Edition, Volume 2 that is very helpful in answering Kathleen's question. In 4.2.2 Interpretation of "highly improbable" (n) accidents reported as due to any other cause outside Chapter XX are considered to be highly improbable. Thus, accidents are generally not accepted as due to disease conditions. There are five exceptions but none of them are for accidents caused by intoxication, so assignment should be made to the falls.

Japan /Reiko Matsumura and Tadahiro Ootsu/:
We will code T510 and X45 for this case.
Please see Volume 3 ( second edition, 2004 ) p.681, Alcohol - beverage, Accidental.

Sweden /Lars Age Johansson/:
A very interesting question - if "intoxicated" simply means "drunk" I would agree with Patricia, because most accidents may not be due to diseases, as Patricia says. However, if it means "poisoning" I would agree with Reiko and Tadahiro, because there is nothing in the ICD that tells us not to accept an accident as due to another accident. The MRG has discussed the issue of "intoxication" in some detail, and we concluded that, for mortality, "intoxication" should be taken to mean "poisoning", except there is a clear indication that the certifier meant just "drunk" and nothing more.

2006-03-28_04 Campomelic syndrome

(Chapter XVII)

[Question from Lars Age Johansson, Sweden:]

Does anybody have a suggestion for how to code campomelic syndrome? We have found several good descriptions of the condition, but we find it difficult to make up our minds on which code to use, not at least because the manifestations vary so much, and there are more than one chromosomal abnormality involved.


[Comments:]

2006-04-04:

Canada /Patricia Wood/:
For consideration: NCHS has included Syndrome, camptomelic in their ICD-10 index assigning it to code Q789, Osteochondrodysplasia, unspecified.

Japan /Tadahiro Ootsu/:
From reference 1) and 2), many manifestations seems to be suitable Q872. However, reference 2) shows that a mutation in SOX9, a sex-determining region of Y (SRY) related gene, located at 17q24 seems to be associated with the occurrence of both campomelic dysplasia and sex reversal, and reference 3) shows that the apparently female infant died 2 hours postpartum in respiratory distress, but was subsequently found to have a 46,XY chromosome constitution. Therefore, I think that campomelic syndrome may code Q988 (or Q986?). How about it ?

References
1) http://bchealthguide.org/kbase/nord/nord969.htm
2) http://www.thefetus.net/page.php?id=337
3)http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ui
ds=3887600&dopt=Abstract

2006-04-04_01 Linkage HIV - lymphoproliferative disease

(Chapter I, II)

[Question from Augusto Hasiak Santo, Brazil:]

I would like to submit to discussion in the Mortality Forum the issue related to the coding of the linkage between AIDS and lymphoproliferative disease, the latter coded as D47.9. We find many death certificates where lymphoproliferative disease is mentioned as due to AIDS and the automatic processing identifies D47.9 as the underlying cause, which, in my oppinion, is not correct. The last 2006 ACME decision tables do not include D47.9 as due to B20-B24 and there is not any modification figure provided between them. Chronically immunocompromised patients have a marked increased risk of developing lymphoproliferative disease. The incidence of lymphoproliferative disease is significantly higher in individuals who have congenital, acquired of iatrogenically induced immunodeficiency. In ICD-10, the category B21.- is provided to code the linkage of AIDS and malignant neoplasms, from which D47.9, neoplasm of uncertain and unknown behaviour of lymphoid, haematopoietic and related tissues, is excluded.


[Comments:]

2006-04-25:

Sweden /Lars Age Johansson/:
I agree with Augusto that this relationship should be accepted, and HIV coded as the underlying cause. Since category B21 is intended for malignant neoplasms, I also agree with Augusto that the combination D47.9 - B24 does not belong there. The only suitable code I can find is B23.8, HIV disease resulting in other specified conditions. B23.2, HIV disease resulting in haematological and immunological abnormalities, might look like a candidate, but then D47.9 isn't classified as a haematological or immunological abnormality by the ICD.

2006-04-10_01 Code for Senger syndrome

(Chapter XVII?)

[Question from Lilja Sigrún Jónsdóttir, Iceland:]

I have a death certificate that probably should be submitted to the Mortality Forum for discussion, unless you have a response to it yourself.

A 22 yrs old woman dies and her death certificate is as follows: Death of natural causes.
IA Acute rejection of a donor heart (ISHT 3B) 4 5 days IB Chronic rejection IC Heart transplant 9 yrs ago ID Senger Syndrome
According to the literature Sengers Syndrome is characterized by congenital cataracts, hypertrophic cardiomyopathy, mitochondrial myopathy and lactic acidosis.

I would like to code the Senger syndrome as underlying cause of death but it seems there is not an assigned ICD10 code yet, but it would be helpful to hear how others have or would code it. The option is probably to it code the hypertrophic cardiomyopathy (which probably was the main problem here, sinces he had a transplant) but that would miss out on the other features of the syndrome.

Hope to hear comments or discussion on this case.


[Comments:]

2006-05-17:

Sao Paulo Classification Centre /Ruy Laurenti and Heloisa Di Nubila/:
In our opinion, this case could be coded as G71.3 (Mitocondrial myopathy, not elsewhere classified) because this is the problem affecting the cardiac muscle that led to heart transplant (even other signs described in the syndrome seem to be resultants of the mitocondriopathy).

2006-04-25:

WHO /Robert Jakob/:
I would think:
Assigning this disease to category Q87.8 may look rather unsatifactory. Some authors seem to emphasize on a mitochondrial defect in the muscles (due to a genetic defect) causing the symptoms. From a prevention point of view no solution would leap to the eye and for treatment and mortality the myocardial failure seems to be the leading symptom.

But with a view on the around 30 cases (http://www.orpha.net/) described since 1975 in Europe (and maybe a few times more world wide) I am not quite sure the assignment to any of those categories has an impact on statistics.

To retain the fact it was congenital and not focussing on a particular organ system may suffice (Q87.8).

2006-04-18:

Canada /Patricia Wood/:
Multiple Causes: T862/T862/Y830/I422 Underlying cause: I422

The question of how to classify eponymous congenital syndromes has been bothering me a lot lately! More and more often we encounter syndromes that are not indexed and, when we research them, have a whole list of possible characteristic symptoms. If more than one system is affected, it seems to me that Q878, Other specified congenital malformation syndromes, not elsewhere classified, might be suitable. Mind you, we would end up with many, many syndromes classified there.

The other option is, as Lilja mentions, to classify the main problem. In this particular case that seems not a bad idea as the death is really a result of complications of the treatment of one single symptom, the hypertrophic cardiomyopathy (I422). As it is the condition necessitating the surgery, I have no problem selecting I422 as the underlying cause of death in this case. However, I think that it is neither the convention nor the intention of the ICD to classify congenital syndromes by the worst symptom, so I wouldn't like this to become our standard approach for classifying non-indexed congenital syndromes.

I am interested to hear what others think about this situation.

2006-04-10_02 Code for malignant intracranial hypertension

(Chapter VI)

[Question from David Lidsky, Israel:]

How would Mortality Forum participants code this certificate?

Four years old girl:
1a Malignant increased intracranial pressure 1b Massive sinus vein thrombosis 1c
2 Pericardial effusion

Important information may be missing here. What was the cause of the intracranial hypertension (ICH)? What was the cause of the "massive sinus vein thrombosis" (presumably an intracranial venous sinus)? What was the cause of the pericardial effusion? Is there some underlying serious disease?

But there is also a coding problem. As far as I am aware, the only ICD-10 code for increased intracranial pressure is G93.2 , and this is followed by the description "Benign intracranial hypertension" (the ICD-10 updates include a code for intracranial hypertension in neonates).

ICD-10 seems to assume that the ICH is caused by the syndrome of benign intracranial hypertension (pseudotumor cerebri). That may be reasonable in many cases, but our certificate specifically mentions the word "malignant".

I haven't managed to find a definition of "malignant intracranial hypertension", but an internet search gives the impression that it refers to severe ICH which is intractable to treatment. Coding such cases G93.2 will misleadingly include them in the benign category.

Is there any way out of this?


[Comments:]

2006-05-17:

Sao Paulo Classification Centre /Ruy Laurenti and Heloisa Di Nubila/:
We would judge it necessary, at first, to ask the medical attendant about the nature of pericardial effusion assigned in Part II. It seems really that this could be a serious complication of infectious or inflammatory disease in this child. About the "malignant increased intracranial pressure", if you look for "pressure" + "brain" at volume III, the index points to G93.5 (compression of brain, herniation of brain or brainstem), that is more compatible with the terminal cause described by the doctor.

We would agree with the underlying cause G08, if you could not obtain more information about the pericardial effusion.

2006-04-25:

Sweden /Lars Age Johansson/:
In Sweden, we arrived at the conclusion that intracranial hypertension as a cause of death does not belong in the "benign" category of G93.2. We code them to G93.8, although we lose information on the brain disorder. I don't think that any of these solutions, G93.2 or G93.8, is quite satisfactory, so this might be something for the Mortality Reference Group to look into.

2006-04-18:

Canada /Patricia Wood/:
Multiple causes: G932/G08 * I313 Underlying cause: G08

David's question brings to light something that makes me curious. In the index, at increased intracranial pressure, "benign" is a parenthetical entry, implying that it is an optional modifier, the presence or absence of which does not affect the coding of increased intracranial pressure. But when referring to the Tabular List "Benign" is included in the title of the code category, which seems much less optional.

That being said, I am not sure that the specification of increased intracranial pressure as "malignant" necessarily suggests a different code.

It does suggest that the degree or severity of the condition may be worse, but as there is only one code for intracranial hypertension, I would still use it.

The placement of the causes on the certificate indicate that the increased intracranial pressure is due to the massive sinus vein thrombosis (a causal relationship supported in the ACME decision tables) so I would select G08 as the tentative underlying cause of death. This is not linked to, or modified by, I313 so I would select G08 as the underlying cause of death.

Of course, if there is additional information available about what caused the sinus vein thrombosis, I would consider it to be the underlying cause of death.

2006-04-25_01 Reapplying Rule 3 after Rule A

(Rule A, GP-R3)

[Questions from Augusto Hasiak Santo, Brazil:]

These certificates imply first the use of Rule A.

The proposed question is about the use in a second step of Rule 3 to select a condition mentioned in Part II of the death certificate.

Rule A states that "where the selected cause is classifiable to Chapter XVIII, and a condition classifiable elsewhere is reported on the certificate, reselect the cause of death as if the condition classified to Chapter XVIII had not been reported, except to take account of that condition if it modifies the coding".

The introduction of ICD-10 changed Rule 3 and made possible the reselection of wasting diseases, diseases causing paralysis as well communicable diseases and non-trivial injuries when pneumonia and bronchopneumonia were identified as an originating antecedent causes.

Is it possible to consider the neoplasms mentioned in Part II as direct consequences of bronchopneumonias selected by application of General Principle and Rule A?

AGE SEX  DEATH CERTIFICATE
31 F  I499/A419/J180/R100*C710
85 F R092/R688/J180/R54*C710
71 M R688/A419/J180/R402*Y836 C751
90 F J969/J180/R54*C449 G309
72 F J960/J180/R54*C710
71 M J960/J180/R54*C795

[Comments:]

2006-05-02:

Canada /Patricia Wood/:
I think that Augusto's question is addressed in an update to 4.1.8 in Volume 2 (URC issue # 0157) saying that, "After application of the modification rules, selection Rule 3 should be reapplied." By the way, I did a little MMDS-ACME test on these six records and the system selected the malignant neoplasm as the underlying cause of death in each case!

2006-04-25_02 Code for PEC-oma tumours

(Chapter II)

[Questions from Augusto Hasiak Santo, Brazil:]

How should we code PEComas?
"PEComas (tumours showing perivascular epithelioid cell differentiation) are a family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumour of the lung, and a group of rare, morphologically and immunophenotypically similar lesions arising at a variety of visceral and soft tissue sites. ...A subset of PEComas behave in a malignant fashion." (Hornick JL, Fletcher CD. PEComa:what do we know so far? Histopathology 2006;48(1):75-82) In Sao Paulo we are coding as a neoplasm of uncertain or unknown behaviour and as malignant neoplasm when an implication of malignancy is mentioned on the death certificate.


[Comments:]

2006-05-02:

Canada /Patricia Wood/:
I haven't seen PEComa certified as a cause of death on a Canadian certificate yet, but I suppose it mightn't be long coming. It sounds to me like more information should be available, so a query to the certifier for specification of the type and behaviour of the PEComa may be in order.

However, if one had to code PEComa not otherwise specified, I suppose that D481, Neoplasm of uncertain or unknown behaviour of connective and other soft tissue, might be good. And, of course as Augusto says, if there is an implication of malignancy; C499.

2006-04-25_03 Code for Schwachmann syndrome

(Chapter XVII)

[Question from Lars Age Johansson, Sweden:]

We recently had a death certificate mentioning Schwachmann syndrome:
Girl, 8 years old
1a Heart failure
1b Liver and cardiac disease, anemia
1c Schwachmann syndrome
2 Hemolysis

We have found plenty of clinical information on the syndrome, including the fuller name Schwachmann-Bodian-Diamond syndrome. It didn't help a lot with the coding, however. Any suggestions?


[Comments:]

2006-05-17:

Australia /Margaret Campbell/:
I don't know whether you would agree with my reasoning BUT I reviewed the online information on Schwachmann-Bodian-Diamond syndrome and this comment..."or a genetic syndrome, i.e. constitutional pancytopenia such as Diamond Schwachmann syndrome" suggested that D61.0 Congenital pancytopenia might be an appropriate code to use What are your thoughts?

2006-05-02:

Canada /Patricia Wood/:
Another eponymous syndrome! As you indicated Lars Age, I found that there was lots of information available on Schwachman Syndrome. It seems that pancreatic insufficiency and bone marrow dysfunction cause many of the symptoms experienced, including hematological abnormalities. I don't know how best to code this and other syndromes with multiple and varied manifestations.

Incidentally, today I was asked to suggest an ICD-10 code Marinesco-Sjögren Syndrome. There's all sorts of information available about the clinical presentation of this syndrome too [a genetic disorder characterized by very small stature, cerebellar ataxia, cataracts, muscle weakness, hypogonadism and developmental and mental retardation] but I'm not sure what code to assign.

 

2006-06-13_01 Mucoviscidosis in a 79 years old

(Chapter IV)

[Question from Gérard Pavillon, France:]

A woman, 79 years old, has been certified with "mucoviscidosis (adult form)" as the underlying cause. We have checked the age and it is right. We asked the hospital. They checked the record and told us that it was a mucoviscidosis of a particular genetic form that only show among adults.

Has anybody seen something similar? Any suggestion for a code?


[Comments:]

2006-09-11:

Canada /Patricia Wood/:
I agree with the others that "mucoviscidosis" (adult form) should be coded as indexed to E849 Cystic fibrosis, unspecified. Some time ago, as a result of a question raised by our colleagues in Australia, we reviewed a few years worth of data looking at the use of E84 as an underlying cause of death code in adults (over 18 years of age). We had very few cases but the ones we did have were confirmed as correctly coded.

Cyprus /Pavlos Pavlou/:
I agree with Kathleen England and the other colleagues. However, I am curious to know the way the death certificate was written.

USA /Julia Raynor/:
We assign the code for mucoviscidosis to E849, Cystic fibrosis, unspecified. We do not use the age of the decedent to change the code to another category.

2006-09-04:

Malta /Kathleen England/:
Last year we also had a case of cystic fibrosis in a 50 year old female causing death. I was also a little perplexed about the case due to her age but on reviewing the hospital notes found that she had a particular mutation causing cystic fibrosis. It seems that there are different types of genetic mutations causing different degrees of CF, however I am not an expert on this. In our case we coded it as E84.0.

Spain Lluis Cirera Suarez/:
The Cystic Fibrosis patients can survive up to old ages, because of treatment advances (for symptoms control). Nowadays, it is not rare to find congenital diseases in adulthood; even more, this type of pathology may not have a coding for chromosomic abnormalities, as it is the case of Congenital Metabolic Diseases.

Switzerland /Elisabeth Gantenbein/:
Here is our suggestion for the mucoviscidosis case: Our small coding group proposes E84.9 Cystic fibrosis, unspecified.We already had a similar case and decided to take this code. But I'm curious what else will be suggested.

 

 

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