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mort08_1

2008-01-10_01 Mitochondrial disorder in 67 year old

(Chapter IV)

[The first question for 2008 is from Kathleen England, Malta:]

I have a query regarding a death in a 67 year old who died from Mitochondrial disorder. How would you code this?


[Comments:]

2008-02-05:

Switzerland /Elisabeth Gantenbein/:

We code mitochondrial myopathy to G71.3; Mitochondrial cytopathy, MELAS, MERRF and mitochondrial myoencephalopathy to G31.8, according to the German modifications of ICD10 (http://www.dimdi.de/static/de/klassi/diagnosen/icd10/htmlgm2008/fr-icd.htm? gg30.htm+) "Mitochondrial disorders" we code also to G31.8 and we ask for further details. But we would also appreciate an official regulation of these diseases.

2008-01-29:

Sau Paulo Classification Centre /Ruy Laurenti/:

The only information we have is "Mitochondrial disorder" and the code in this case is E88.8. In my opinion it is necessary to discuss this case in the MRG and URC.
2008-01-15:

Belgium /Josie Mornie/:
Good morning, Flanders codes this as E 88.8

Canada /Patricia Wood/:
Happy New Year! I'm glad that Kathleen has asked this question as we see Mitochondrial Disease/Disorder as a cause of death too. This question has come up before; 2003-03-03 Mary Heanue (Ireland) asked for a code for mitochondrial disorder and there were a few responses suggesting ICD-10 codes E888 and G713 and ICD-9 code 3588.

I notice that NCHS includes it to their index (Instruction Manual Part 2e, Volume 3) as:
Disease - mitochondrial E889

If we could reach some kind of consensus this time perhaps we could get the MRG to make a recommendation to the URC to make an official update to ICD-10.

Israel /Sam Rubin/:
This is a tricky one. One must resist the temptation to code it as "mitochondrial myopathy " (G71.3) .Mitochondrial myopathy is a sub class of mitochondrial diseases . In the absence of a more specific code in the ICD we would code it as "specified metabolic disorder " (E88.8).

A peaceful 2008 to all.

Japan /Emiko Oikawa/:
A Happy New Year ! In Japan, we code to G713.

Japan /Tadahiro Ootsu/:
Probably, in Japan we use G71.3 for Mitochondrial Disorders because the word "mitochondrial" is found in this code.

However, a reference (please see http://www.genereviews.org/profiles/mt-overview) shows that considerable clinical variability exists and many patients do not fit neatly into one particular category.

On the other hand, since the prevalence of all Mitochondrial diseases is 11.5/100,000, it may be necessary to discuss new independent codes for them.

How about it ?

Sweden /Lars Age Johansson/:
In Sweden, we try to code by the most prominent feature of the mitochondrial disease. That is, we code G71.3 for myopathy, but E88.8 if there is no information on what kind of mitochondrial disease the patient had. I certainly agree with the rest of you that the ICD needs to give a few guidelines on how to code mitochondrial diseases.

Tanzania /Yusuf Hemed/:
Here is my attempt in providing a response:
The cause of death that has been reported (Mitochondrial disorder) is very general and it would be helpful if the attending physician could provide more detail or specifics.

The index (Volume 3) does not provide for "mitochondria" or for "disorder, mitochondria". I believe, there is no international classification of disease (ICD) code for the vast majority of mitochondrial diseases (the conditions were named only recently-1984), the option that is available in completing death certificates would be to list generic codes or "unspecified" as the cause of death with a possible exception for the code for G71.3 which is for mitochondrial myopathy, not elsewhere classified.

Notes: Mitochondrial disorders are a group of conditions that affects both sexes but caused by defects in the mitochondrial genome which is inherited purely from the female parent. The conditions includes: Mitochondrial myopathy, encephalopathy, lactic-acidosis, stroke-like episodes, abbreviated to MELAS. The conditions MELAS affects many of the body's systems, particularly the brain and nervous system (encephalo-) and muscles (myopathy) were named only recently i.e.1984.

In a Feb. 28-Mar. 4, 2001 conference in San Diego, physicians and researchers met and recommended that the NCHS provide new mitochondrial disease ICD code. I am not sure if this code has been provided and whether it appears (or will appear in subsequent revisions).

2008-02-05_01 Cause of Clostridium difficile infection

(Rule R3)

[Question from John G.O'Connor, Ireland:]

We are currently querying Doctors and health care providers as to the underlying cause of Clostridium Difficile infections and results to date show antibiotic therapy as the main reply.

In light of this should we code to the underlying condition which necessitated antibiotic therapy, thereby eliminating the need to contact the health care service providers?

Example
I C diff colitis
C.O.P.D
II CVA, Prostatic cancer

We queried the underlying cause of the C.Diff. infection and the reply from the Doctor came back as 'due to antibiotic therapy'.


[Comments:]

2008-02-13:

Canada /Patricia Wood/:

Below are two examples of Canadian death certificates ... in each case we did select COPD as the underlying cause of death, given that it was the condition necessitating the drug (moxifloxacin) therapy. I think that this is in line with the principle of selecting the event that initiated the train of events leading to death. However, I suppose that some researchers (especially those interested in C. difficile infections) will have an issue with this because the C. difficile infection is not reflected in our underlying cause tabulations.

It is important to mention that we have to do this as a manual intervention when using MMDS, because the ACME decision tables do not allow for a causal relationship between C. difficile colitis (ICD-10 code A04.7) and drugs, medicaments and biological substances causing adverse effects in therapeutic use (ICD-10 codes Y40-Y59). So, if we decide to apply the underlying cause principle to cases of C. difficile arising as a complication of antibiotic drug therapy given to treat a condition, the ACME decision tables will need to be updated.

Part I
a) C-difficile colitis 9 days
b) Moxifloxacin treatment for COPD exacerbation

Part II
COPD exacerbation, abdominal wall hematoma 2 to cough

Part I
a) Myocardial Infarction
b) C-difficile colitis
c) Moxifloxacin treatment for exacerbation of COPD

Part II
CAD

2008-03-11_01 Selection of underlying cause

(Rule GP, 1-3)

[Question from Tanja Coric, Croatia:]

I have a query regarding an underlying cause of death in a 35 year old man who had these diseases:
Pneumonia
Chronic alcoholism
Schizophrenia
Sequelae of stroke


[Comments:]

2008-03-26:
Sao Paulo Classification Centre /Ruy Laurenti and Heloisa Di Nubila/:

The causes described are in Part I a, b, c and d of the DC? If yes, the rationale is: accept pneumonia as due to chronic alcoholism, but do not accept chronic alcoholism as due to schizophrenia. Thus, according to rule 1, the u.c. is chronic alcoholism F10.2.

2008-03-18:
Sweden /Lars Age Johansson/:

If we assume that all conditions were listed on the same line of the death certificate, I would first apply Rule 2 to select pneumonia as tentative underlying cause of death. After that, I would apply Rule 3 to select sequelae of stroke as the final underlying cause. This is based on the instruction in Volume 2 on how to apply Rule 3 (section 4.1.7, Rule 3), which says that pneumonia "should be considered an obvious consequence of wasting diseases (such as malignant neoplasm and malnutrition) and diseases causing paralysis (such as cerebral haemorrhage or thrombosis)." Of the conditions enumerated on this certificate, only the stroke meets these criteria.

2008-03-11_02 Code for idiopathic infantile arterial calcification

(Chapter IV)

[Question from Lars Age Johansson, Sweden:]

We recently received a death certificate for a girl, one month old, who had died from "idiopathic infantile arterial calcification". Which code would you use for that condition?


[Comments:]

2008-03-26:
Sao Paulo Classification Centre /Ruy Laurenti and Heloisa Di Nubila/:

The age of death is one month old. Thus, the first idea is that the affection is congenital. Although the affection, as described, is not exactly a "malformation" under terminological point of view, the code is

Q28.8 - other specified congenital malformation of the circulatory system.

2008-03-18:
Israel /Sam Rubin/:

Research evidence regarding IIAC is as yet inconclusive . Perhaps deficiency of inorganic Pyrophosphate which normally inhibits Hidroxyaptite can explain deposition of the latter in the internal elastic lamina of vessel wall that were observed in some cases. In our opinion either I77.8 or P29.8 is appropriate .we are still debating.

Nordic Classification Centre /Martti Virtanen/:

From Pediatr Dev Pathol. 2007 Aug 23; :1 17990935 (P,S,E,B) Idiopathic Infantile Arterial Calcification: The Spectrum of Clinical Presentations.

[My paper] Curtis Chong, Grover Hutchins .....A search of the English language medical literature identified 161 IIAC case reports. Of these, 48% of cases present in utero or at birth, with hydrops fetalis, maternal hydramnios, heart failure, or respiratory distress, and 52% present later, at a median of 3 months with sudden onset of fever, vomiting, irritability, or respiratory distress in a previously healthy infant. Significantly, 19 of 22 IIAC survivors presented at less than two weeks of age, and 15 survivors were treated with diphosphonates. Several similar reports are available.

Thus this is a poorly know congenital disease affecting obvious the whole vascular system. This is something that should be discussed in the rare disease TAG.

For coding in ICD-10 this is not a congenital anomaly, nor chromosomal abnormality. It causes a kind of atherosclerosis, but this definitely not the one intended by the codes for arteriosclerosis in the current system.

There are neither any codes general arteriosclerosis. I end up in P29.8 'Other cardiovascular disorders originating in the perinatal period [Extracted from ICD-10 Second Edition, 2005, Certain conditions originating in the perinatal period.] which is however very vague.

2008-04-01_01 Code for "critical illness syndrome"

(Chapter IV, X, XVIII)

[Question from Lars Age Johansson, Sweden:]

Some time ago we got a death certificate with "critical illness syndrome" reported as one of the causes of death. We have seen this term before, of course, but always in connection with one or other of the usual components of a "critical illness syndrome", for example rhabdomyolysis or adult respiratory distress. In such cases we have coded to that particular component. In this case there was nothing we could link to the "critical illness syndrome", so my question is how to code this term if it appears on its own, and there are no further specification of which components the syndrome had.


[Comments:]

2008-04-15:

Sao Paulo Classification Centre /Ruy Laurenti and Heloisa Di Nubila/:

 

We think that, without additional information, the candidate codes would be the ill-defined:

1) R53 - General physical deterioration

2) R68.8 - Other specified general symptoms and signs

3) R69 - Unknown and unspecified causes of morbidity, illness NOS, undiagnosed disease, not specified as to the site or system involved

2008-04-08_01 Code for cytochrome oxidase deficiency

(Chapter IV)

[Question from John G.O'Connor, Ireland:]

(a) Cardiopulmonary Arrest
(b) Metabolic Acidosis
(c) Cytochrome Oxidase Deficiency
Age 2 Years
Any suggested ICD 10 Coding suggestions would be appreciated.


[Comments:]

2008-04-29:
Canada /Patricia Wood/:
FYI - I notice that cytochrome oxidase deficiency is indexed in NCHS Part 2e, Volume 3 to ICD-10 code E888. This would suggest that MMDS users are using this code (whether they know it or not!). Of course we can discuss the most appropriate code but if something other than E888 is agreed to, NCHS should be notified so that they can update the MICAR dictionary.

2008-04-15_01 Code for abdominal compartment syndrome

(Chapter XI)

[Question from Sam Rubin, Israel:]

Another classic debate concerns "Abdominal compartment syndrome" . Can anyone suggest an appropriate ICD code? I intend to participate in the coming ICE meeting and hope to meet many of the friends.


[Comments:]

2008-04-29:
Canada /Patricia Wood/:
FYI - CIHI has included abdominal compartment syndrome in their clinical modification, ICD-10-CA:

Syndrome
- compartment (deep) (posterior) T79.6
- - abdominal (traumatic) T79.6
- - non traumatic M62.2

The classification (ICD-10-CA) does not provide a nontraumatic code for "abdominal" compartment syndrome

[My Swedish colleague Annika Näslund has reminded me that we discussed a similar issue in the MRG four or five years ago. Question 2003-11-25 Q1 was on "Codes for abdominal compartment syndrome and bariatric surgery". The codes suggested were K91.8 and Y83.4. /Lars Age]

2008-04-22:
Switzerland /Elisabeth Gantenbein/:
There are a lot of possible causes for the "ACS". I propose the Code R19.8 and to query for the cause of this symptom.

2008-04-15_02 Code for protein S deficiency and APC resistance

(Chapter III)

[Question from Lars Age Johansson, Sweden:]

We have recently encountered diagnostic terms denoting an increased risk of thrombosis, for example protein S deficiency and APC resistance. Which ICD code(s) would you suggest?


[Comments:]

2008-04-29:
Canada /Patricia Wood/:
In anticipation of a new specific code (D685) for primary thrombophilia I favour the interim code of D68.8 for protein S deficiency (and the other inclusion terms at the new code). We had two recent certificates mentioning Protein C deficiency, neither mentioning enough information to assign one or another of Robert's specific scenarios (i.e. hereditary versus acquired):

1(a) liver failure
(b) TPN use associated with short bowel syndrome
(c) hypercoagulopathy (protein C deficiency)

1(a) acute subdural hematoma
(b) protein C/S deficiency
(c) on heparin

Scotland /Colin Fischbacher/:
A potential problem with D68.2 is in the title: "clotting factors". If this is understood as "a factor that promotes clotting" then it would not apply to protein S which is an anticoagulant. "Coagulation factor" seems to me a more general term which is less open to misunderstanding.

2008-04-22:
Scotland /Colin Fischbacher/:
I think we would use D68.8 for protein S deficiency.

Switzerland /Elisabeth Gantenbein/:
In the ICD-10 major update for 2009 there's the new code D68.5 for these deficiencies. According to that new code I propose to use the code D68.8 in the meantime.

[My Swedish morbidity colleagues Annika Näslund and Åsa Fernlund also pointed out to me that these two conditions are covered by this ICD-10 update. /Lars Age]

WHO /Robert Jakob/:
The update code [D68.5] will be valid only from the next cycle of major updates (now 2010). As a result, an interim solution makes sense. This raises also the point of transition tables between the tables.

Deficiency of protein S is hereditary or acquired. It is a co-factor to factors V and VIII. The protein S is dependent on Vitamin K. As such, D68.2 might be an option for the hereditary one, D68.4 for the acquired vitamin K related one, and D68.9, if origin is unspecified.

APC (Activated Protein C) is co-factor to protein S. In several cases, resistance seems to be inherited, and is due to a defect of factor V that inhibits the action of APC; and it might be coded to D68.2. Acquired forms exist as well, e.g. from oral contraceptives, post menopausal and as a consequence of inflammatory diseases. Where the cause is specified, it might be appropriate to code to the underlying disease, unless there is a specific mention under D68 (lupus) or cross reference (pregnancy/abortion).

(see also Wikipedia, http://www.ncbi.nlm.nih.gov/pubmed/15199491,
http://www.ncbi.nlm.nih.gov/pubmed/9834008, and
http://www.ncbi.nlm.nih.gov/pubmed/10361512 )

2008-05-20_01 Code for Roberts syndrome

(Chapter XVII)

[Question from Josie Mornie, Belgium/Flanders:]

Flanders has a question : how do you code " Roberts Syndrome " (stillborn)?

 

Roberts syndrome is a rare genetic disorder characterized by growth delays before and after birth (pre- and postnatal growth deficiency); malformations of the arms and legs (limbs); distinctive abnormalities of the skull and facial (craniofacial) region. Mental retardation occurs in some cases; normal intelligence has also been reported.

 

Synonyms :Hypomelia-Hypotrichosis-Facial Hemangioma Syndrome --Pseudothalidomide Syndrome-- SC Syndrome thanks!


[Comments:]

2008-06-10:

Japan /Tadahiro Ohtsu/:
I could find another references about Roberts Syndrome.
Reference 1) shows that it is characterized by pre and postnatal growth retardation, severe shortening of limbs with radial defects, oligodactyly and characteristic facial features.
Reference 2) shows that it is an autosomal recessive disorder of symmetric limb defects, craniofacial abnormalities, pre- and postnatal growth retardation, and mental retardation.

Therefore, I think that Roberts Syndrome has not only phocomelia, but also facial malformation, and probably we should use Q87.2. Please see Figure 3, 4 and 5 of http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=16380922

References
1)Temtamy SA, Ismail S, Helmy NA.
Roberts syndrome: study of 4 new Rgyptian cases with comparison of clinical and cytogenetic findings. Genet Couns. 2006;17(1):1-13
2)Hwang K, Lee DK, Lee SI, Lee HS.
Roberts syndrome, normal cell division, and normal intelligence.
J Craniofac Surg. 2002 May;13(3):390-4

2008-05-27:

Japan /Tadahiro Ohtsu/:
A reference below shows that Roberts Syndrome is characterized by tetraphocomelia, profound growth deficiency of prenatal onset, craniofacial anomalies, microcephaly, and mental deficiency. Therefore, I think that we can use Q87.0 or Q87.2 for Roberts Syndrome. Maybe, we should use Q87.5.

How about it ?

Reference Schüle B, Oviedo A, Johnston K, Pai S, Francke U. Inactivating mutations in ESCO2 cause SC phocomelia and Roberts syndrome: no phenotype-genotype correlation. Am J Hum Genet. 2005 ;77(6):1117-28

Sweden /Annika Näslund/:
I googled for Roberts syndrome and found http://stipe.inserm.fr/abourgog/consor4.01/www/cgi-bin/Disease_Search.php?ln g=EN&data_id=971&Diseases=Roberts-syndrome&search=Disease_Search_Simple

They suggest the code Q73.8.

However, at http://www.nationmaster.com/encyclopedia/Phocomelia (and at several other web sites) they seem to think that Roberts syndrome is the same as phocomelia, and that would give the code Q73.1.

2008-05-20_02 Code for PEHO (progressive encephalopathy with edema, hypsarrhythmia and optic atrophy)

(Chapter VI)

[Question from John O'Connor, Ireland:]

I (a) Cardio Respiratory Failure 2 Days
I (b) Progressive microcephaly 11 months
I (c) PEHO Syndrome -
II Global developmental delay 1 year
Male Aged 1

PEHO (Progressive encephalopathy with Edema, Hypsarrhythmia and Optic atrophy) syndrome is a rare neurodegenerative disorder belonging to the group of infantile progressive encephalopathies.

I am considering a code of G31.8 - Other specified degenerative disease of nervous system for PEHO Syndrome but would welcome any suggestion from the forum.


[Comments:]

2008-06-03

Australia /Sue Walker/:
I looked this syndrome up on Orphanet - see http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=971&Di sease_Disease_Search_diseaseGroup=roberts-syndrome&Disease_Disease_Search_diseaseType=Pat&Diseases=Roberts-syndrome&search=Disease_Search_Simple

They recommend the use of Q73.8. Note that the Orphanet people are managing the ICD-11 TAG for Rare Diseases.

Sao Paulo Classification Centre /Ruy Laurenti/:
I agree with the code G31.8. However I would like to discuss about a code of the Chapter XVII (Congenital). Why not Q04.8?

2008-05-27:

Japan /Tadahiro Ohtsu/:
Reference 1) shows that disease onset is in early infancy, most children appear healthy at birth or may be slightly hypotonic. So, probably, we should not use Q codes for PEHO Syndrome. And, reference 2) shows that the pathological findings are early progressive atrophy of the cerebellum, brainstem, and optic nerves. Accordingly, I think that G31.8 is suitable code for PEHO Syndrome.

References
1)Huisman TA, Klein A, Werner B, Straube T, Boltshauser E.Serial MR imaging, diffusion tensor imaging, and MR spectroscopic findings in a child with progressive encephalopathy, edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome.AJNR Am J Neuroradiol. 2006 ;27(7):1555-8
2)Riikonen R. The PEHO syndrome. Brain Dev. 2001 ;23(7):765-9

2008-06-03_01 C-section in heroin addict

(Chapter XV)

[Questions from Tanja Coric, Croatia:]
I have a dilemma about underlying causes of death:

Female, 27 years old. Case history: Longterm heroin addict, at times on methadone substitution therapy. Pregnancy ends at 36th week by c-section which was conducted without complications. The child was born and treated.

Postoperatively, there is increase in the blood pressure of the deceased as well as elevated temperature of the central type along with tremor of the entire body, unrest and somnolence after which she falls into a 14-month coma and dies. It is known from case history that her last doze of heroin was taken the night before delivery. Toxicology was not conducted at that time. Urinoculture and hemoculture were sterile. The physician treating her during delivery considers the withdrawal state to be the cause of death.

Autopsy report is as follows: Sequelae of coma, Sequelae of caesarean section, Hydrocephalus, Bronchopneumonia, Decubitus ulcer.


[Comments:]

2008-06-10:

Canada /Patricia Wood/:
Withdrawal state from a heroin addiction would be coded to F11.3 in a decedent who was not pregnant or had not just given birth. Using this information I think the same circumstances in a decedent who had just given birth would be coded to O993, Mental disorders and diseases of the nervous system complicating pregnancy, childbirth and the puerperium.

On the other hand, if the withdrawal state is interpreted as a complication of the c-section, O754 could be considered ... And then, because death occurred 14 months after delivery O97, Death from sequelae of direct obstetric cause might be a possibility. However, the narrative that Tanja provided did say that the c-section was conducted without complications so this might be inappropriate.

Japan /Tadahiro Ohtsu/:
The death certificate for this case is probably as below:

Female, 27 years old.
Part 1 (a) Bronchopneumonia ( J18.0 or J18.2 )
(b) Coma ( R40.2 )
(c) Hydrocephalus ( G91.8 )
(d) Delivery at 36th week by c-section ( O82.8 )
Part 2 Heroin addict ( T40.1 )

According to p.64 of ICD-10 Volume 2 ( Second Edition ), we can not use O80-O84 for underlying cause mortality coding, and we will decide G91.8 for this case. How about it ? I would like to get your advices.

Sweden /Lars Age Johansson/:
I think that these three questions show very clearly how difficult it is to classify maternal deaths in ICD-10, and how differently the coding instructions can be interpreted. The Mortality Reference Group has discussed similar issues several times, and in 2007 the MRG made a recommendation for a new coding instruction relating to maternal deaths. The recommendation was approved by the Update Reference Committee, and from the 2010 version of ICD-10 the following instruction will be included in Volume 2:

"4.2.15 Death due to maternal (obstetric) causes
a) It is often difficult to identify a maternal death, particularly in cases of indirect obstetric causes. If there is any doubt that the cause of death is obstetrical, for example if the conditions entered in Part I are not obstetrical but there is a mention of pregnancy or delivery in Part II, additional information should be sought from the certifier. This is particularly important in countries where maternal mortality rate is high.

If no additional information can be found, deaths with a mention of pregnancy and delivery in Part I should be considered obstetrical, but not deaths where pregnancy or delivery is mentioned in Part II only."

If we apply these instructions to the three cases that Tanja sent us, the first one would count as a maternal death. I think I would go with Patricia's first suggestion, O99.3. The death occurred more than one year after the c-section, but the sequelae category available, O97, only covers direct obstetric causes. (Just to confuse matters even a little more: That too will change, and from the 2010 version O97 will include sequelae of indirect obstetric causes as well. So from 2010 the underlying cause would be O97.1, Death from sequelae of indirect obstetric cause.)

In the second and third cases the pregnancy is mentioned in Part II and the certifier has not stated that the conditions mentioned in Part I were significantly influenced by the pregnancy. Because of that the new instructions would give cardiomyopathy as underlying cause in the second case and subarachnoid hemorrhage in the third, provided that no further information can be obtained from the certifier. But, as I said, this is according to the new instructions that will take effect in 2010.

2008-06-03_02 Cardiomyopathy in pregnancy

(Chapter XV)

[Questions from Tanja Coric, Croatia:]

I have a dilemma about underlying causes of death:
Female, 28 years old.
I part:
1. Acute myocardial infraction
2. Dilated cardiomyopathy
II part:
Pregnancy 30 weeks.


[Comments:]

2008-06-10:

Canada /Patricia Wood/:
If the decedent was still pregnant at the time of death (rather than in the postpartum period) I think that this would be coded as an indirect cause as per the index entry:

Cardiomyopathy
- complicating pregnancy O994

ICD-10 code O994 excludes cardiomyopathy in the puerperium (O90.3) but I think it may be the right underlying cause of death code for a woman who dies from dilated cardiomyopathy and is pregnant at the time of death.

Japan /Tadahiro Ohtsu/:
I think that pregnancy probably imposed a burden on her dilated cardiomyopathy. So, we should apply Rule 3, and use O99.4 for this case.

Sweden /Lars Age Johansson/:

I think that these three questions show very clearly how difficult it is to classify maternal deaths in ICD-10, and how differently the coding instructions can be interpreted. The Mortality Reference Group has discussed similar issues several times, and in 2007 the MRG made a recommendation for a new coding instruction relating to maternal deaths. The recommendation was approved by the Update Reference Committee, and from the 2010 version of ICD-10 the following instruction will be included in Volume 2:

"4.2.15 Death due to maternal (obstetric) causes
a) It is often difficult to identify a maternal death, particularly in cases of indirect obstetric causes. If there is any doubt that the cause of death is obstetrical, for example if the conditions entered in Part I are not obstetrical but there is a mention of pregnancy or delivery in Part II, additional information should be sought from the certifier. This is particularly important in countries where maternal mortality rate is high.

If no additional information can be found, deaths with a mention of pregnancy and delivery in Part I should be considered obstetrical, but not deaths where pregnancy or delivery is mentioned in Part II only."

If we apply these instructions to the three cases that Tanja sent us, the first one would count as a maternal death. I think I would go with Patricia's first suggestion, O99.3. The death occurred more than one year after the c-section, but the sequelae category available, O97, only covers direct obstetric causes. (Just to confuse matters even a little more: That too will change, and from the 2010 version O97 will include sequelae of indirect obstetric causes as well. So from 2010 the underlying cause would be O97.1, Death from sequelae of indirect obstetric cause.)

In the second and third cases the pregnancy is mentioned in Part II and the certifier has not stated that the conditions mentioned in Part I were significantly influenced by the pregnancy. Because of that the new instructions would give cardiomyopathy as underlying cause in the second case and subarachnoid hemorrhage in the third, provided that no further information can be obtained from the certifier. But, as I said, this is according to the new instructions that will take effect in 2010.

2008-06-03_03 Subarachnoid hemorrhage in pregnancy

(Chapter XV)

[Questions from Tanja Coric, Croatia:]

I have a dilemma about underlying causes of death:
Female, 26 years old.
I part:
1. Subarachnoid haemorrhage
II part:
Pregnancy 8 weeks.


[Comments:]

2008-06-10:

Canada /Patricia Wood/:
Would this also be coded as a maternal death from an indirect cause, I wonder? Checking the index I see:

Hemorrhage
- subarachnoid
- - puerperal, postpartum or in childbirth or pregnancy O994

Japan /Tadahiro Ohtsu/:
Q3. I can not judge how pregnancy would influence subarachnoid haemorrhage.

She was in early stage of pregnancy, and no information about its abnormality. So, we should apply General Principle, and use I60.9 for this case.

Sweden /Lars Age Johansson/:
I think that these three questions show very clearly how difficult it is to classify maternal deaths in ICD-10, and how differently the coding instructions can be interpreted. The Mortality Reference Group has discussed similar issues several times, and in 2007 the MRG made a recommendation for a new coding instruction relating to maternal deaths. The recommendation was approved by the Update Reference Committee, and from the 2010 version of ICD-10 the following instruction will be included in Volume 2:

"4.2.15 Death due to maternal (obstetric) causes
a) It is often difficult to identify a maternal death, particularly in cases of indirect obstetric causes. If there is any doubt that the cause of death is obstetrical, for example if the conditions entered in Part I are not obstetrical but there is a mention of pregnancy or delivery in Part II, additional information should be sought from the certifier. This is particularly important in countries where maternal mortality rate is high.

If no additional information can be found, deaths with a mention of pregnancy and delivery in Part I should be considered obstetrical, but not deaths where pregnancy or delivery is mentioned in Part II only."

If we apply these instructions to the three cases that Tanja sent us, the first one would count as a maternal death. I think I would go with Patricia's first suggestion, O99.3. The death occurred more than one year after the c-section, but the sequelae category available, O97, only covers direct obstetric causes. (Just to confuse matters even a little more: That too will change, and from the 2010 version O97 will include sequelae of indirect obstetric causes as well. So from 2010 the underlying cause would be O97.1, Death from sequelae of indirect obstetric cause.)

In the second and third cases the pregnancy is mentioned in Part II and the certifier has not stated that the conditions mentioned in Part I were significantly influenced by the pregnancy. Because of that the new instructions would give cardiomyopathy as underlying cause in the second case and subarachnoid hemorrhage in the third, provided that no further information can be obtained from the certifier. But, as I said, this is according to the new instructions that will take effect in 2010.

2008-06-10_01 Code for peroxisomal deficiency in a child with biliary atresia

(Chapter IV, XVII)

[Question from Josie Mornie, Belgium/Flanders:]

What is the best ICD-10 code for peroxisomal deficiency disorder in a 2 year old boy with biliary atresia?

Thanks for answer!

2008-06-17_01 Code for mitochondrial encephalopathy

(Chapter IV, VI)

[Question from Lars Age Johansson, Sweden:]

We have received a death certificate for a girl, 10 years old, who suffered from "mitochondrial encephalomyopathy" and "infantile neuronal axonal dystrophy". Do you have any suggestions for how to code these two conditions?


[Comments:]

2008-09-09:

Canada /Patricia Wood/:
If "Infantile neuronal axonal dystrophy" (also known as Steitelberger disease) is a neurodegenerative disorder how about G318, Other specified degenerative diseases of nervous system as a provisional code?

Mitochondrial disorders (specified and unspecified) are a recurring theme for the Mortality Forum (cf. 2008-01-10 and 2003-03-03) which is a clear indication that some direction is needed. Dr. Laurenti suggested that the issue be brought forward to the MRG for further discussion. I agree with him, although somewhat reluctantly since I'm on the MRG!

2008-06-17_02 Code for infantile neuronal dystrophy

(Chapter IV, VI)

[Question from Lars Age Johansson, Sweden:]

We have received a death certificate for a girl, 10 years old, who suffered from "mitochondrial encephalomyopathy" and "infantile neuronal axonal dystrophy". Do you have any suggestions for how to code these two conditions?

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