Thank you for the opportunity to speak to this subcommittee today.
NACHRI, the National Association of Children's Hospitals and Related Institutions, currently has 151 member and supporter hospitals. I would like to discuss how we use coding and classification systems at NACHRI particularly in our Classification Research program area and offer comments and recommendations for consideration to this Subcommittee.
NACHRI uses the International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) extensively, for evaluation of inpatient, outpatient and population based health care databases. NACHRI provides comparative analyses to the member hospitals using ICD-9-CM. NACHRI also extensively uses and analyzes ICD-9- CM through the development and maintenance of two classification systems. These two systems are the All Patient Refined Diagnosis Related Groups (APR-DRGs), which NACHRI co-develops with 3M Health Information Systems and is used for inpatient care analysis and the NACHRI Classification of Congenital and Chronic Health Conditions (CCCHC) used to evaluate population based databases for disease prevalence, utilization profiling, capitation risk adjustment, and linkage to measures of patient satisfaction.
Through the development of these two classification systems NACHRI has carefully reviewed the content, structure and clinical meaningfulness of ICD-9-CM specifically as it is used to describe and classify neonatal, perinatal and pediatric conditions. Review of the ICD-9-CM codes has entailed checking for all index entries for a given code, researching conditions/diseases assigned to a code, and seeking medical input from our medical advisory committee. Additionally, interviews have been conducted with all medical and surgical division chiefs at two of our member children's hospitals. Throughout this process we have identified and received affirmation of the strengths and weaknesses of ICD-9-CM.
The strengths of ICD-9-CM, which represents an evolution of the ICD classification system, is that it currently contains the ability to classify thousands of known causes of death, established diseases and other conditions for which patients encounter health care. It is a widely known and accepted disease classification system used for statistical and reporting purposes. Hospitals, physicians and other health care providers have long used these codes to classify diseases, index health records, conduct research and most recently to bill for patient care. The codes are used as a base of HCFA's Prospective Payment System's Diagnosis Related Groups (DRGs) and many other payment systems and, therefore ICD-9-CM has assumed a central role for payment systems. One of the greater weaknesses of ICD-9-CM, in terms of pediatric classification, would be the lack of specificity of many codes in the congenital anomaly and perinatal sections. Some codes have been found to contain many different diseases with a few codes containing as many as 100 conditions. Although many of these diseases may be rare or lower in prevalence they can account for extensive inpatient hospital stays, multiple surgical encounters and outpatient health care consumption. There is a need to have many of these conditions further broken out of these existing ICD-9-CM codes to more closely monitor the care and outcomes.
NACHRI has been active in the identification of necessary changes to ICD-9-CM by regularly submitting proposals to the ICD-9-CM Coordination and Maintenance Committee which meets twice per year. Some of these proposed changes have been adopted into ICD-9-CM. Although NACHRI is grateful to the purpose of this committee, the process is slow in meeting the requests of NACHRI for pediatric classification changes. Sometimes only three to four of NACHRI's proposals are able to be accepted, for consideration, per meeting agenda. Many categories in ICD-9-CM which need to be more finely divided have no more available codes such as available 5th digits. The need for further refinement is great such that adoption of an ICD-10-CM would achieve the greatest improvement and in the shortest time frame.
Many of NACHRI's proposals for changes to ICD-9-CM are based on reviewing the classification of these diseases in the existing ICD-10 classification system. For pediatric conditions ICD-10 has provided more specific codes particularly for congenital anomalies, perinatal and other hereditary conditions. Attached, as an example, are our most recent proposals submitted for the upcoming June meeting. Many of the proposals are recommending the ICD-10 classification system breakout of these conditions. ICD-10 has been adopted into use in many other countries. It has been considered for implementation in the United States with a clinical modification as ICD- 10-CM in the year 2000. NACHRI strongly recommends the adoption of ICD-10-CM as a standard of coding and classification of diseases and health care encounters no later than the year 2000. Changing from the ICD-9-CM to the ICD-10-CM classification system will require resources for training and changing computer databases. This is to be expected with any major system change. However, the need for greater specificity far outweighs this temporary expenditure of resources. The level of specificity of pediatric diseases is needed to further refine the earlier described classification systems as well as to improve upon ongoing analyses of pediatric health care.
In addition to ICD-9-CM disease classification review NACHRI has been actively involved with the development of ICD-10-PCS for procedural classification, through HCFA's ICD- 10-PCS Technical Advisory Panel (TAP). We are familiar with the latest refinements towards development of this system and its differences to the existing ICD-9-CM Procedure Classification system. As with its disease classification counterpart, ICD-9- CM for procedures needs to have many categories subdivided in more detail. Again the needs over extend the available space. It has been strongly recommended that there be one system adopted for procedural classification. It is confusing to remember which system (ICD-9-CM or CPT-4) is used depending on which care setting the patient encounters and which payor is reimbursing the treatment. In addition, because both systems are not consistently applied to all treatments it is difficult to consistently report and analyze the care. Crosswalks are currently used between these two systems but at times are confusing. While NACHRI does not, at this time, endorse one classification system over the other we would like to recommend that there be only one adopted for procedural classification, whether it be ICD-10-PCS or CPT-4 or a combination of both. This would simplify collection and evaluation of the data.
Another group of disorders that can be difficult to evaluate, under current practice, are neoplastic diseases. The second edition of the International Classification of Diseases for Oncology (ICD-0) provides the ability to classify the morphology (histological type and behavior) of the neoplasm. Having this additional information available can help greatly with profiling, prognosis and evaluation of utilization of services for these conditions. Most of the neoplasms, and especially the solid tumors, can be better classified using both of these classification systems together. Many facilities and health care providers have internal tumor registries and already collect the ICD-0 code in that database. These codes are usually not externally reported for other purposes and therefore only the ICD-9-CM code, which usually only describes the site of the neoplasm, exists in a health care facility's central database or outside agency databases. Many neoplasms are unique only to their age group, in that they do not occur in adults and vice versa. Studying these diseases strictly by site and age is not as clinically meaningful as knowing its tissue of origin or histological behavior. Since many facilities already internally assign and collect these codes in a separate database NACHRI recommends that a standard be adopted to require the collection of both the ICD-9- CM and ICD-0 codes when reporting neoplasms externally, as well as in a given health care provider's central database.
The following examples demonstrate the value of having both the ICD-0 and the
ICD-9-CM codes to provide meaningful information for treatment and study purposes. Neuroblastoma is a neoplasm which can occur in many different sites such as adrenal gland, abdomen and thorax. The ICD-9-CM codes for these sites do not define any of these to be neuroblastoma. There are other types of neoplasms that can occur in these same sites which could require different treatment and offer a different prognosis. Knowing the ICD-0 code of M9500/3 in addition to the site would provide more meaningful information. Another example is Wilm's tumor, nephroblastoma of the kidney, which is one of the most "curable" pediatric neoplasms. It is extremely rare in adults. In contrast, adult renal carcinoma (not occurring in children) has a very high mortality rate. Both of these codes are assigned the same ICD-9-CM (site) code of 189.0 (Neoplasm of kidney except pelvis). The ICD-0 code for Wilm's tumor is M8960/3 (Nephroblastoma, malignant primary site). The adult renal tumor may have a different ICD-0 code telling physicians and other health care providers of the tissue and behavior of the adult form of this neoplasm. Primary malignant neoplasm of the lung is another example where site and histological classification are clinically meaningful. The ICD-9- CM code for primary neoplasm of the lung is in the 162.X category. However, there are many different histological types of lung cancer which have different ICD-0 codes assigned to them such as oat cell (M8042/3), small cell (M8041/3) and adenocarcinoma (which has many ICD-0 codes such as M8140/3 and M8250/3 depending on the site). These are all treated differently and have varying prognoses. One final example is hepatoblastoma which occurs in infants and is, potentially, curable. It is assigned the ICD-9-CM code of 155.0 and the ICD-0 code of M8970/3. Its adult counterpart of hepatocellular carcinoma receives the same ICD-9-CM site code of 155.0; however, could receive a different ICD-0 code depending on its histological type. Adults diagnosed with hepatocellular carcinoma have a high mortality rate. There are other examples, however these have been provided to illustrate the need to collect both codes.
Currently the guidelines for coding and reporting define different rules depending on the type of health care setting that the patient encountered. There are differences in these guidelines depending if the patient was treated in an inpatient or outpatient setting. An example of this difference would be the reporting of a suspected condition. If the patient was admitted to an inpatient setting for a condition reported as "rule out" or "suspected", the inpatient coding guidelines instruct that the condition is reported as a confirmed diagnosis. For example, if admitted to the hospital for the diagnosis of "seizures, rule out epilepsy", the ICD-9-CM code for epilepsy (345.90) would mostly likely be reported. If the patient were seen in the emergency room with this the same diagnosis it would be coded and reported as seizures (780.3). NACHRI recommends that these guidelines be revised to adopt one set for all health care encounters.
ICD-9-CM currently has a section of V Codes which are a "supplementary classification of factors influencing health status". In ICD-10 these have been retained as part of that classification system. Many of these codes are necessary to know the current status of a disease, such as transplant status, personal history of a neoplasm, presence of a device and dependence on a device. Many times these codes are not reported because either they are not reimbursed or a health care facility adopts an internal policy that the information is not needed. NACHRI recommends that V Codes (or their equivalent ICD-10 codes) be required, where applicable, to report the status of disease processes.
As previously mentioned, NACHRI actively participates in the semi-annual meetings of the ICD-9-CM Coordination and Maintenance Committee. The role and function of this committee provides health care providers and others interested in the classification of diseases and procedures with the opportunity to regularly propose updates to this classification system. This means as new diseases are discovered, or disease processes change or are differently understood the classification can be changed to accommodate those needs. NACHRI recommends that this process be continued beyond the adoption of standards addressed by this committee, whether it be continuing to update ICD-9-CM or hopefully moving into ICD-10-CM and maintaining that system as needed, and that this process be fully staffed.
In conclusion NACHRI would like to emphasize the importance of clinically meaningful classification systems to continue to study the morbidity and mortality of disease processes, and consistency across all patient settings is most important. We believe the above recommendations will help accomplish this goal. NACHRI appreciates the opportunity to have provided input to this committee for this important decision process.
There is one code, 753.0, for renal agenesis. Using this code does not describe whether the agenesis is unilateral or bilateral. Additionally, there is no code to indicate hypoplasia versus complete agenesis. All of these conditions are currently assigned to the one code, 753.0.
Renal agenesis is the failure of the kidney to develop in utero. A baby born with bilateral renal agenesis will most likely die of renal insufficiency unless treated with dialysis and kidney transplant. People with unilateral agenesis can often live a normal life sometimes without ever knowing they have this condition. There is also significance in knowing whether the kidney is underdeveloped (hypoplasia). A person with renal hypoplasia will still have renal problems which may later require dialysis and/or transplant.
Potter's syndrome is a rare combination of renal agenesis and a distinctive set of facial appearances (wide spaced eyes, floppy ears, micrognathia, etc). Infants usually die shortly after birth.
ICD-10 introduced a seven way breakout of renal agenesis and other reduction defects of the kidney. It is recommended that the available fifth digit code level, in ICD-9-CM, be used to implement this same type of breakout.
Existing: 753.0Renal agenesis and dysgenesis
- Atrophy of kidney:
Congenital
Infantile
[Code also any associated vesicoureteral reflux (593.70-593.73)]
Proposed: 753.0Renal agenesis and other reduction deformities
753.00Renal agenesis, unilateral
753.01Renal agenesis, bilateral
753.02Renal agenesis, unspecified
753.03Renal hypoplasia, unilateral
753.04Renal hypoplasia, bilateral
753.05Renal hypoplasia, unspecified
753.06Potter's syndrome
[Code also any associated vesicoureteral reflux (593.70-593.73)]
Tethered spinal cord syndrome is currently assigned to ICD-9-CM code 742.59, other specified anomalies of spinal cord. This condition is a syndrome which is caused by a thickening of the filum terminale (filament of connective tissue from the end of the spinal cord to the base of the coccyx). This thickening causes the spinal cord to stretch leading to progressive neurologic problems. The thickening of the filum terminale is believed to be due to defects arising from improper closure of the neural tube during embryonic development. People with tethered spinal cord syndrome experience a specific set of symptoms which help to identify the condition, such as; low back pain, fatty tumors in the lower back, urologic symptoms and progressive sensory and motor deficits.
This condition may occur by itself or it may accompany spina bifida. Treatment consists of surgically removing the thick adhesions and is usually successful. Patients who have this in conjunction with spina bifida sometimes require repeated treatment or posterior rhizotomy to relieve pain.
It is important to be able to track those who have this syndrome either separately or in addition to spina bifida. Currently you cannot do this since the other anomaly conditions and terms which are indexed to 742.59 are much broader and nondescript. One of the terms indexed to 742.59, myelodysplasia, is a more generic term that is sometimes used interchangeably with other more specific anomaly diagnoses such as spina bifida.
It is recommended that a unique ICD-9-CM code be assigned to tethered spinal cord syndrome to allow it to be classified separately.
Existing: 742.59 Other specified anomalies of spinal cord
Proposed: 742.54 Tethered spinal cord syndrome
742.59 Other specified anomalies of spinal cord
Diagnosis code 771.8 is used for other infections acquired before, during or shortly after birth or via the umbilicus. It is one of the more commonly recorded diagnosis codes for newborns.
As currently defined, diagnosis code 771.8 is very broad both in terms of the specificity of the infection and the severity of the infection. It contains specific infections such as urinary tract infections as well as unspecified infections such as intra-amniotic infection of fetus not otherwise specified. It contains newborns who may be barely symptomatic as well as newborns who are septic. ICD-10 has broken out many specific sepsis organisms to separate diagnosis codes. This allows classifying sepsis in newborns separately from other types of infections.
Sepsis of newborn occurs five times more often in low birth weight babies, those with decreased respiratory functions, high risk maternal factors, and longer hospital and NICU length of stays. It is a primary cause of many neonatal deaths.
To distinguish sepsis of newborn separate from other types of infections it is recommended to add two new fifth digit level codes to diagnosis code 771.8. One code would contain sepsis of newborn and the other would be used to classify other perinatal infections. For further specificity, it is also recommended to add a coding instruction to this category suggesting that the infectious organism diagnosis code also be assigned.
Existing: 771.8Other Infection Specific to the Perinatal Period
Intra-amniotic infection of fetus:
Not otherwise specified
Clostridial
Escherichia coli (E.coli)
Intrauterine sepsis of fetus
Neonatal urinary tract infection
Septicemia (sepsis) of newborn
771.8Other Infection Specific to the Perinatal Period
771.81Septicemia (sepsis) Specific to the Perinatal Period
771.88Other Infection Specific to the Perinatal Period
Use additional code to identify specific infectious organism
Both congenital dysplasia of the hip and congenital anterversion of femur are assigned to code 755.63. These two conditions are very different in that one is a congenital deformity in the shape of the hip joint and the other is usually normal and corrects itself as the child develops and starts walking.
Congenital dysplasia of hip or developmental dysplasia of the hip (the more current terminology), results in an abnormal shape of the hip joint. This may be due to either a congenital shallowness of the acetabulum or a large femoral head. The hip may be unstable and prone to dislocate. Infants with this condition must be treated usually using a splint and prone positioning. Sometimes surgery is required to correct this problem. If left untreated the child may develop a gait instability or possibly a crippling osteoarthritis before middle age. These patients must be followed closely through their skeletal maturity.
Congenital anteversion (twisting inward) of the femur is universal in newborns. For most children this condition is self correcting as a part of their musculoskeletal maturing and development. The anteversion usually starts at about 40 degrees at birth and by teen age decreases to about 15 degrees. The child may temporarily appear to have "knock knees" and it is sometimes recommended for the child to avoid prone positioning during sleep. Few children receive treatment for this.
Developmental dysplasia of the hip belongs with the category 754.3, Congenital dislocation of hip, since this condition is usually the underlying cause of the dislocation or subluxation. ICD-10 added a code to the congenital dislocation category, for unstable (dislocatable/subluxatable) hip. It is recommended that a new 5th digit level code, in 754.3, be created for developmental dysplasia of hip without dislocation or subluxation. Anteversion would remain assigned separately to code 755.63.
Existing: 754.3 Congenital Dislocation Of Hip
Proposed: 754.3 Developmental Dysplasia Of Hip
754.36 Developmental Dysplasia of Hip Without Dislocation or
Subluxation
Congenital Dysplasia of Hip
Unstable Hip
Dislocatable Hip
Subluxatable Hip
The diagnosis code 754.2 contains both congenital scoliosis and congenital lordosis. These are two very different conditions requiring different treatments. Congenital kyphosis is assigned to code 756.19, Other anomalies of spine.
Congenital scoliosis is caused by either the failure of formation of the bony elements of the spine or the failure to develop segments of these bony elements. It results in a lateral curvature of the spine. Severe degrees of curvature can cause cardiopulmonary and neurological problems. Many patients with severe cardiopulmonary problems usually require spinal fusion to halt the progressive deformity. Early intervention is important.
Congenital lordosis is a normal anterior curvature of the lumbar spine. Later in life a person may develop an exaggeration of this curvature. This is commonly referred to as "swayback". There is usually no treatment initiated to change the curvature of this portion of the newborn spine.
Congenital kyphosis is caused by failure to form the anterior elements of the bony spine. It results in a "humpback" appearance. If untreated it can lead to irreversible neurological compromise, such as paralysis. Treatment is usually either an anterior or posterior fusion of spine. In severe cases both types of fusion are necessary.
ICD-10 recognizes the significance of congenital scoliosis by assigning it a unique code separate from congenital lordosis. It is recommended that this same approach be incorporated into ICD-9-CM by using the available 5th digit level of code 754.2 to breakout congenital scoliosis. Additionally, it is recommended to remove congenital kyphosis from 756.19 and assign it a code in the 754.2 category. This would follow a similar breakout applied to the acquired curvature codes (category 737). Since congenital lordosis is usually a normal curvature of the spine it is recommended to create an NEC/NOS code in the same category and index congenital lordosis to this code.
Existing: 754.2Certain Congenital Musculoskeletal Deformities of Spine
Congenital Postural:
Lordosis
Scoliosis
Proposed: 754.2 Certain Congenital Musculoskeletal Deformities of Spine
754.20 Congenital Scoliosis
754.21 Congenital Kyphosis
754.29 Congenital Deformity of Spine NEC/NOS
Congenital Lordosis
Congenital heart disease affects approximately .8% of the childhood population. There are many different types of congenital heart malformations, each associated with different morbidity and mortality. It is therefore important to distinguish these different malformations, and where relevant, to distinguish whether the malformation involves atresia, stenosis, insufficiency or hypoplasia
Atresia means a pathological closure of a normal opening or congenital absence of same. Stenosis, which is a less serious condition, means a constriction or narrowing of a passage or orifice. Insufficiency (of a heart valve) refers to the failure of the valve to close completely. Hypoplasia means the incomplete development or underdevelopment of an organ or tissue. Other heart malformations can include very different conditions, sometimes very mild or asymptomatic conditions and other times very serious conditions.
The ICD-9-CM diagnoses codes for congenital heart malformations sometimes make the distinction between atresia, stenosis, insufficiency and other conditions, but it does not always do so. Many of the proposed changes to the ICD-9-CM codes that follow are intended to more consistently make this distinction. Other proposed changes are to provide separate codes for distinctive malformations. Most of these changes are also made by ICD-10.
745 Bulbus Cordis Anomalies and Anomalies of Cardiac Septal Closure
Existing: 745.0 Common Truncus
This code includes terms that describe common truncus or truncus arteriosus, a complex cyanotic congenital heart malformation, usually accompanied by a ventricular septal defect and generally requiring closure of the VSD and connection of the right ventricle to the pulmonary artery by an artificial conduit and sometimes an artificial semilunar valve. Ongoing care is required including replacement of the conduit with growth. This code also includes terms for a very different condition, aortopulmonary window, a malformation which generally requires an operation to close the communication between aorta and pulmonary artery and then is usually fully corrected.
Proposed: 745.00 Common Truncus
745.01 Aortopulmonary Septal Defect
[Note: This is the same as the breakout in ICD-10.]
Existing: 745.5 Ostium Secundum Type Atrial Septal Defect
This code includes two very different conditions, an ostium secundum atrial septal defect (ASD) and a persistent foramen ovale. An ostium secundum ASD is a hole in the atrial septum due to failure of fusion between the septum secundum and the endocardial cushion. It generally requires surgical (or catheter) closure. A foramen ovale is the aperture in the septum secundum of the fetal heart that provides communication between the left and right atria while in utero. It usually is functionally closed by the flap of the septum primum within minutes or hours of birth. Anatomic closure depends on relative right and left atrial pressures. In 5% of persons without heart disease, the foramen ovale may remain patent for life. [Note, there are six times as many individuals with patent foramen ovale than with all congenital heart malformations.]
Proposed: 745.50 Ostium Secundum Type Atrial Septal Defect
745.51 Persistent Foramen Ovale
Existing: 746.1 Congenital Tricuspid Atresia and Stenosis
Proposed: 746.10 Congenital Tricuspid Valve Atresia
746.11 Congenital Tricuspid Valve Stenosis
[Note, both ICD-9-CM and ICD-10 combine these two tricuspid valve diagnoses into one code even though they are very different conditions.]
Existing: 746.2Epstein Anomaly
Proposed: 746.20 Epstein Anomaly
746.21 Other Tricuspid Valve Malformations Insufficiency
[Note, ICD-10 has separate codes for Other Specified Tricuspid Valve Malformation and Unspecified Tricuspid Valve Malformation. ICD-9-CM assigns tricuspid insufficiency and other tricuspid malformations to 746.89, Other Specified Heart Anomaly. It is recommended that ICD-9-CM pull together in one code, 746.21, all other tricuspid valve malformations and insufficiency.]
Existing: 746.3 Congenital Stenosis of Aortic Valve
Proposed: 746.30 Congenital Atresia of Aortic Valve
746.31 Congenital Stenosis of Aortic Valve
[Note, congenital atresia of aortic valve is currently assigned by ICD-9-CM to 746.89, Other Specified Heart Anomaly. ICD-10 combines it with congenital stenosis of aortic valve. These are very different conditions and it is recommended that they be separated into two codes.]
Existing: 746.4 Congenital Insufficiency of Aorta Valve
This code includes two very different conditions. The first is insufficiency or regurgitation of the aortic valve. The second is bicuspid aortic valve which is a very common condition occurring in 1% of the population. Most often, bicuspid aortic valve produces no symptoms though it can cause stenosis or insufficiency, more typically in adulthood. It would be best to have bicuspid aortic valve in a separate fourth digit code but since there are no unused fourth digit codes in the 746 series, it is recommended to separate out bicuspid aortic valve through a fifth digit code for 746.4.
Proposed: 746.40 Congenital Insufficiency of Aortic Valve
746.41 Bicuspid Aortic Valve
Use additional code if there is valvular stenosis or insufficiency associated with bicuspid aortic valve.
Existing: 746.5 Congenital Mitral Stenosis
Proposed: 746.50 Congenital Mitral Atresia
746.51 Congenital Mitral Stenosis
[Note, congenital mitral atresia is currently assigned by ICD-9-CM to 746.89, Other Specified Heart Anomaly. ICD-10 combines it with congenital mitral stenosis. These are very different conditions and it is recommended that they be separated into two codes.]
Existing: 746.7Hypoplastic Left Heart Syndrome
Proposed: 746.70 Hypoplastic Left Heart Syndrome
746.71 Hypoplastic Right Heart Syndrome
[Note, this is the same breakout as in ICD-10.]
Existing: 746.89 Other Specified Heart Anomaly
This code contains 108 indexed terms. Some of these more appropriately belong in other more discrete congenital heart anomaly codes and are addressed as part of the recommendations for these other codes.
There is one additional recommendation and this has to do with the term congenital cardiomegaly. Cardiomegaly or ventricular hypertrophy is assigned to 746.89 if congenital and to 429.3 if acquired. Cardiomegaly is a fairly high volume and important condition to be able to identify. It is recommended that it be removed from 746.89 and be assigned its own separate code, 746.88, or be merged into 429.3. Strictly speaking, congenital cardiomegaly or ventricular hypertophy is not a congenital heart malformation diagnosis but rather a status description of an infant with congenital heart disease. The congenital versus acquired distinction in the case of cardiomegaly is really a false distinction.
Existing: 747.22 Atresia and Stenosis of Aorta
Proposed: 747.22 Atresia of Aorta
747.23 Stenosis of Aorta
[Note, this is the same as the breakout in ICD-10.]
Existing: 747.3Anomalies of Pulmonary Artery
Anomalies of the pulmonary artery are among the highest volume of congenital heart malformations. ICD-10 provides a four way breakout: atresia, stenosis, NEC and NOS. It is recommended to work from this structure and then provide another category for hypoplasia of pulmonary artery, a very serious condition, and provide a further breakout stenosis of pulmonary artery to distinguish unilateral from multiple. Congenital stenosis of plmonary artery is the highest volume malformation of the pulmonary artery and since the morbidity is very different for unilateral and multiple, it is important to provide separate categories.
Proposed: 747.30 Congenital Atresia of Pulmonary Artery
747.31 Congenital Hypoplasia of Pulmonary Artery
747.32 Congenital Stenosis of Pulmonary Artery, Unilateral
747.33 Congenital Stenosis of Pulmonary Artery, Multiple
747.34 Other Specified Congenital Anomalies of Pulmonary Artery
747.39 Unspecified Anomalies of Pulmonary Artery
Existing: 747.89 Other Specified Congenital Anomalies of Circulatory System
This code includes persistent fetal circulation or persistent pulmonary hypertension, recently reassigned from 747.9, Unspecified Anomaly of Circulatory System (though inadvertently left in 747.9 as well on CD-ROM). This is a distinct, high volume condition that should be placed in its own separate ICD-9-CM code. Persistent fetal circulation is the condition when during the first days following delivery a stressed newborn (often premature) reverts to fetal type circulation. This occurs when the newborn's pulmonary arterioles constrict and the ductus arteriosus dilates, resulting in right-to-left shunting through the now patent ductus arteriosus and the reopened foramen ovale. As a consequence, the newborn becomes hypoxic. The goal of treatment is to reverse the conditions that produced pulmonary vasoconstriction.
Proposed: 747.83 Persistent Fetal Circulation
- Persistent pulmonary hypertension
[Note, this is the same breakout as in ICD-10 except that ICD-10 places it in the chapter on perinatal conditions which is really a more appropriate placement.]
The diagnosis code 754.89 includes a number of different nonteratogenic anomalies of the musculoskeletal system. The most dramatic of these is arthrogryposis multiplex congenita (AMC) or multiple congenital contractures.
AMC is a condition of congenital fibrous ankylosis of multiple joints. In classic AMC, the joints of all limbs are fixed; muscles are hypoplastic; limbs tend to be tubular and featureless; soft tissue webbing is sometimes present; and associated abnormalities are sometimes present in syndromic forms of AMC. Treatment usually involves physical therapy in the newborn period and early infancy. Surgery is sometimes required to achieve better position or greater range of motion.
ICD-10 separates AMC into a separate code. It also separates nonteratogenic anomalies related to the hand into a separate code. It is recommended that AMC be separated from 754.89 by creating a new code in the 754.8x category. It is also recommended that the nonteratogenic anomalies related to the hand be separated from 754.89 in the same way as in ICD-10.
Existing: 754.89Other Specified Nonteratogenic Anomalies
Club hand (congenital)
Congenital:
deformity of chest wall
dislocation of elbow
Generalized flexion contractures of lower limb joints,
congenital
Spade-like hand (congenital)
Proposed: 754.83Congenital Deformity of Hand
754.84Arthrogryposis Multiplex Congenita.
754.89Other Specified Nonteratogenic Anomalies
Rett's Syndrome is defined as pervasive development disorder where apparently normal early development is followed by partial or complete loss of speech, the skills of locomotion and loss of purposeful hand motions. Severe mental retardation almost invariably results. This syndrome is found only in the female population.
Asperger's Disorder is another type of pervasive developmental disorder. This disorder is characterized by abnormalities of social interaction that typifies autism, together with a restricted repetitive group of interests and activities. There is a strong tendency for these abnormalities to persist into adolescence and adult life.
The ICD-9-CM index assigns Rett's syndrome to 330.8 and Asperger's disorder to code 299.8. The 330.8 code is described in the Tabular list as "Other Specified Cerebral Degenerations in Childhood." These syndromes according to ICD-10 and DSM-IV are both considered to be pervasive developmental disorders and are not considered to be generalized encephalopathy.
It is recommended that two new codes be created, at the available fifth digit level of diagnosis code 299.8, to include Rett's Syndrome and Asperger's disorder. In addition, it is recommended to introduce a code at this level for "Other" psychoses in this category.
Existing: 299.8 Other Specified Early Childhood Psychoses
330.8 Other Specified Cerebral Degenerations In Childhood
Proposed: 299.8 Other Specified Early Childhood Psychoses
299.80Rett's Syndrome
299.81Asperger's Disorder
299.88Other Specified Early Childhood Psychoses
Thrombocytopenia refers to a decrease in the number of circulating platelets and can result from either a decrease in production or an increase in destruction of platelets. The result in either case is an increased tendency for bruising and bleeding. Thrombocytopenia may occur as a primary condition or secondary to other disease processes.
There are several forms of primary thrombocytopenia. Congenital thrombocytopenia is the form where the bone marrow does not make sufficient platelets. Immune thrombocytopenia is the form where the bone marrow makes sufficient platelets but there is immune mediated destruction of the platelets; this may be either acute or chronic.
ICD-9-CM has a single code (287.3) for primary thrombocytopenia. ICD-10 provides a breakout that distinguishes idiopathic thrombocytopenia purpura (immune thrombocytopenia purpura is the current accepted terminology) from other primary thrombocytopenias. We propose that the available 5th digit codes be used to provide a similar breakout.
Existing: 287.3 Primary Thrombocytopenia
Evans Syndrome
Megakaryocytic Hypoplasia
Purpura Thrombocytopenic
Congenital
Hereditary
Idiopathic
Thrombocytopenia:
Congenital
Hereditary
Primary
Tidal Platelet Dysgenesis
Excludes: Thrombotic thrombocytopenic purpura (446.6)
Transient thrombocytopenia of newborn (776.1)
Proposed: 287.30 Congenital Thrombocytopenia
Congenital Thrombocytopenia with Absent Radii
Congenital Thrombocytopenia without Absent Radii
287.31 Chronic Immune Thrombocytopenia
Evans Syndrome
Idiopathic Thrombocytopenia
287.32 Acute Immune Thrombocytopenia
287.38 Other and Unspecified Primary Thrombocytopenia
Prematurity is a major cause of morbidity and mortality for newborns and through the first year of life. Of all newborns, 5% to 6% are born prematurely (before 37 weeks gestation). Most problems of premature infants relate to immature functioning of organ systems and complications thereof.
There is a V Code for History of Perinatal Problems but this is not specific to problems of prematurity. It is proposed that the V 13.7 code be expanded through use of the fourth digit to specifically recognize problems relating to history of extreme prematurity and other prematurity as well as other perinatal problems. This would solve the problem of not being able to track through ICD-9-CM codes the growth and development of premature infants.
Existing: V13.7History of Perinatal Problems
Proposed: V13.70History of Extreme Prematurity with Birthweight <1,000 Grams
V13.71History of Extreme Prematurity with Birthweight 1,000 - 1,499 Grams
V13.72History of Extreme Prematurity with Birthweight 1,500 - 1,999 Grams
V13.73 History of Extreme Prematurity with Birthweight ³2,000 Grams
V13.74History of Other Prematurity with Birthweight < 1,000 Grams
V13.75 History of Other Prematurity with Birthweight 1,000 - 1,499 Grams
V13.76History of Other Prematurity with Birthweight 1,500 - 1,999 Grams
V13.77History of Other Prematurity with Birthweight ³2,000 Grams
V13.78History of Other Specified Perinatal Problems
V13.79History of Perinatal Problems Unspecified
Note: Extreme prematurity usually implies gestation age less than 28 completed weeks. Other prematurity usually implies gestation age of 28 to 37 completed weeks.
The diagnosis code, 770.8, Other Respiratory Problem After Birth, is a very high volume diagnosis recorded for newborns. It contains several different respiratory conditions, including apneic spells (pauses in breathing), cyanotic attacks, respiratory failure (in newborns) as well as respiratory distress. The apneic conditions can be sleep apnea or due to other causes. It is important to be able to differentiate these conditions.
ICD-10 has introduced a six way break out of these conditions. It is recommended that a fifth digit level of codes be added to diagnosis code 770.8 to incorporate this same level of specificity.
Existing: 770.8 Other Respiratory Problem After Birth
Proposed: 770.80 Sleep Apnea of Newborn
770.81 Other Apnea of Newborn
770.82 Cyanotic Attacks of Newborn
770.83 Respiratory Failure of Newborn
770.88 Other Specified Respiratory Conditions of Newborn
770.89 Unspecified Respiratory Conditions of Newborn
The diagnosis code 786.09, Other Respiratory Problem contains some very diverse conditions, some more specific than others and some more serious than others. This includes apnea, Cheyne-Stokes respiration (regularly alternating periods of apnea and hyperpnea), respiratory distress, respiratory insufficiency, shortness of breath, tachypnea, and wheezing.
ICD-10 provides a breakout for three of the more specific conditions - Cheyne-Stokes respiration, wheezing, and shortness of breath. It is recommended that the available fifth digit codes in 786.0X be used for a five way breakout of the more specific conditions with 786.09 reserved for other respiratory distress/insufficiency.
Existing: 786.09 Other Respiratory Problems
Distress
Insufficiency
Shortness of breath
Tachypnea
Wheezing
Proposed: 786.03Tachypnea
786.04Wheezing
786.05Shortness of Breath
786.06Cheyne-Stokes Respiration
786.07Apnea
786.09Other Respiratory Problems
Currently, ICD-9-CM assigns a wide range of craniofacial malformations to a single diagnosis code, 756.0, Anomalies of Skull and Face Bones. This includes nonexistence of skull bones, premature closure of skull sutures (craniosynostosis), malformations in which eyes are widely space (hypertelorism), malformations of jawbone, and malformations affecting multiple areas such as Crouzon's disease which includes widely space eyes, exophthalmos, optic atrophy, strabismus, acrocephaly, and a beak shaped nose. The morbidity for these conditions is very different as are the treatments. Some require a series of staged reconstructive surgeries.
ICD-10 has developed an eight way breakout of these malformations. It is recommended that ICD-9-CM incorporate these refinements.
Existing: 756.0 Anomalies of Skull and Face Bones
Absence of skull bones
Acrocephaly
Congenital deformity of forehead
Craniosynostosis
Crouzon's disease
Hypertelorism
Imperfect fusion of skull
Oxycephaly
Platybasia
Premature closure of cranial sutures
Tower skull
Trigonocephaly
Proposed: 756.0 Anomalies of Skull and Face Bones
756.01Craniosynostosis
756.02Craniofacial dysostosis
-Crouzon's Disease
756.03Hypertelorism
756.04Macrocephaly
756.05Mandibulofacial dysostosis
756.06Oculomandibular dysostosis
756.08Other specified congenital malformations of skull and face bones
756.09Congenital malformation of skull and face bones, unspecified