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Special Showcase Edition April 2013

Coding for Pediatric Chronic Lung Disease
For The Record
Vol. 25 No. 7 P. 26

Chronic lung disease (CLD) is a broad term in pediatric pulmonology representing a broad category of chronic lung disorders in children. When CLD is documented, specificity for the child’s type of lung disease/disorder should be sought. If further physician clarification cannot be obtained, then based on the ICD-9-CM index, nonspecific CLD should be assigned to code 496, Chronic airway obstruction, not otherwise specified. Since this is a nonspecific code, ideally additional documentation should be obtained to support a more specific code assignment.

Chronic restrictive lung disease is another commonly documented term that also needs further clarification as to the specific condition. According to Coding Clinic, chronic restrictive lung disease is assigned to code 518.89, Other diseases of lung, not elsewhere classified. It also says that chronic restrictive lung disease “is an ill-defined term, however, and should be used only when the condition cannot be described more specifically.” 

Infants born prematurely or requiring supplemental oxygen/ventilatory support may develop a specific type of CLD known as bronchopulmonary dysplasia (BPD) (code 770.7). This is caused by damage to the fragile lung tissue, which becomes inflamed and then ultimately scarred. Babies with BPD may have an increased risk of respiratory infections.

BPD occurs most commonly in white male babies. Those most at risk include infants born before 34 weeks gestation with a birth weight less than 4 lbs, 6 oz, a familial history of asthma, mothers who have a womb infection, a patent ductus arteriosus, and pulmonary interstitial emphysema.

BPD symptoms include respiratory distress (tachypnea, nasal flaring, and chest retractions) and a need for ventilatory or oxygen support after 36 weeks gestation. Chest X-rays may show a bubbly, spongelike appearance of the lungs. Treatment may range from supplemental oxygen, mechanical ventilation, bronchodilators, steroids, fluid restrictions, proper nutrition, and influenza immunization.1

Children also are at risk of developing CLD when they have a congenital lung disorder. Again, specificity is needed for the type of congenital lung disorder and any related manifestations. Some common congenital disorders include conditions such as the following:

• agenesis, hypoplasia, and dysplasia of lung (748.5);

• alveolar capillary dysplasia (516.64);

• bronchogenic cyst (748.4);

• congenital bronchiectasis (748.61);

• congenital cystic lung (748.4);

• congenital diaphragmatic hernia (756.6);

• congenital lobar emphysema (770.2);

• cystic fibrosis (subcategory 277.0);

• Scimitar syndrome (747.49); and

• VATER syndrome (vertebrae, anus, trachea, esophagus, renal abnormalities) (759.89). (Also assign additional codes to identify the clinically significant manifestations that are present.)

An unspecified congenital lung disorder is classified to code 748.60, and other congenital lung disorders are classified to code 748.69.

Interstitial lung disease (ILD) in children usually has an underlying cause. However, in about 19% to 27% of cases, there is an undetermined cause. The consistent feature is structural remodeling of the distal airways leading to impaired gas exchange. Two possible mechanisms include sequela of persistent inflammation or tissue injury with abnormal healing, resulting in collagenous fibroids. This fibrotic process is mostly responsible for the morbidity and mortality of ILD. The associated hypoxia causes pulmonary hypertension and cor pulmonale.

Approximately 50% of pediatric ILD occurs in infants who present with symptoms such as tachypnea, retractions, difficulty and diaphoresis in feeding, cyanosis during feeding and rest, failure to thrive, and weight loss. Older children have similar symptoms and, more predominately, a dry, nonproductive cough, anorexia, fatigue, and hemoptysis. They also report chest pain. Wheezing is a common feature in both populations.

Other causes of ILD are divided into three categories: idiopathic, known/suspected causes, and those caused by systemic diseases. A partial listing includes the following:

• acute interstitial pneumonitis;

• amyloidosis;

• bowel disease (eg, ulcerative colitis, Crohn’s disease);

• bronchiolitis obliterans;

• bronchocentric granulomatosis;

• chronic aspiration pneumonitis;

• connective tissue diseases (eg, juvenile rheumatoid arthritis, systemic lupus erythematosus);

• eosinophilic syndromes;

• exposure to organic dusts (eg, hypersensitivity pneumonitis), gases (eg, oxygen, chlorine, nitrogen dioxide, ammonia);

• idiopathic bronchiolitis obliterans organizing pneumonia, (cryptogenic organizing pneumonia);

• immunodeficiency-associated ILD;

• liver disease (eg, chronic active hepatitis, primary biliary cirrhosis);

• lymphangiomatosis;

• lymphocytic interstitial pneumonitis;

• lymphoproliferative disorders;

• neoplasia (eg, lymphoma, leukemia);

• neurocutaneous disorders (eg, tuberous sclerosis, neurofibromatosis, ataxia-telangiectasia);

• nonadenoviral bronchiolitis obliterans;

• nonspecific interstitial;

• previous lung injury;

• pulmonary alveolar proteinosis;

• pulmonary hemorrhage syndromes;

• pulmonary vasculitis (eg, polyarteritis nodosa, Wegener granulomatosis, Churg-Strauss syndrome);

• radiation exposure;

• resolving acute respiratory distress syndrome;

• sarcoidosis;

• use of antineoplastic agents or other drugs; and

• usual interstitial pneumonitis.

ILD during childhood is classified to subcategory 516.6. A fifth digit is required to identify the specific type, including the following:

• 516.61, Neuroendocrine cell hyperplasia of infancy;

• 516.62, Pulmonary interstitial glycogenosis;

• 516.63, Surfactant mutations of the lung;

• 516.64, Alveolar capillary dysplasia with vein misalignment; and

• 516.69, Other interstitial lung disease of childhood.

Note that the 2011 fourth quarter Coding Clinic (pages 117-123) provides more information on each type of childhood ILD.

Treatment of the underlying condition is essential, but additional treatment may include optimization of nutrition, prophylactic immunizations, treatment of secondary infections, bronchodilator therapy, oxygen (both for comfort and prevention of hypoxia, pulmonary hypertension, and cor pulmonale), smoke-free environmental control, and additional medication therapies, such as steroids, cytotoxic agents, immunosuppressants, IV immunoglobulin, antioxidants, and cytokine inhibitors.2

Ensure the specific underlying condition is fully documented by the provider and review for secondary diagnoses, such as chronic respiratory failure and acute or chronic respiratory failure. Additionally, ensure that the dependence on oxygen status and tracheostomy status, if applicable, is captured, as these have a large impact on severity of illness and risk of mortality.

Coding and sequencing for pediatric CLD are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding.

— This information was prepared by Cheryl Manchenton, RN, and Audrey Howard, RHIA, senior consultants with 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to more than 5,000 health care providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447.

1. Chronic lung disease of prematurity. Boston Children’s Hospital website. http://www.childrenshospital.org/az/Site2094/mainpageS2094P4.html.

2. Hagood JS, Bye MR. Children’s interstitial lung disease (ChILD). Medscape Reference website. http://emedicine.medscape.com/article/1003631-overview.


ICD-10-CM Coding for Childhood Interstitial Lung Diseases
Childhood interstitial lung diseases are classified to category J84 in ICD-10-CM. A fifth or sixth character is required to identify the specific type, including the following:

• J84.83, Surfactant mutations of the lung;

• J84.841, Neuroendocrine cell hyperplasia of infancy;

• J84.842, Pulmonary interstitial glycogenosis;

• J84.843, Alveolar capillary dysplasia with vein misalignment; and

• J84.848, Other interstitial lung diseases of childhood.

— CM, AH