ICD-10-CM Documentation 2021: Essential Charting Guidance to Support Medical Necessity

Chapter 4

ENDOCRINE, NUTRITIONAL AND METABOLIC DISEASES, AND IMMUNITY DISORDERS

Introduction

Endocrine, nutritional, and metabolic disorders are contained in Chapter 4, but certain disorders involving the immune system are in Chapter 3 and are covered with diseases of the blood and blood-forming organs within that chapter.

The blocks within the endocrine, nutritional, and metabolic diseases chapter are shown below.

ICD-10-CM Blocks
E00-E07	Disorders of Thyroid Gland
E08-E13	Diabetes Mellitus
E15-E16	Other Disorders of Glucose Regulation and Pancreatic Internal Secretion
E20-E35	Disorders of Other Endocrine Glands
E36	Intraoperative Complications of Endocrine System
E40-E46	Malnutrition
E50-E64	Other Nutritional Deficiencies
E65-E68	Overweight, Obesity and Other Hyperalimentation
E70-E88	Metabolic Disorders
E89	Postprocedural Endocrine and Metabolic Complications and Disorders, Not Elsewhere Classified

Coding Notes

There are coding instructions related to neoplasms of the endocrine glands. Neoplasms of the endocrine glands, whether functionally active or not, are classified in the neoplasm chapter. This chapter note is further expanded to include the use of an additional code from Chapter 4, when appropriate, to indicate either functional activity by neoplasms and ectopic endocrine gland tissue, or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Exclusions

There is a single Excludes1 note at the chapter level.

Excludes1	Excludes2
Transitory endocrine and metabolic disorders specific to the newborn (P70-P74)	None

Chapter Guidelines

The only chapter guidelines for endocrine, nutritional, and metabolic diseases relate to diabetes mellitus. Diabetes mellitus codes are combination codes that capture the type of diabetes, the body system affected, and the complications or manifestations affecting each body system. The three components of the combination codes are as follows:

•The type of diabetes:

–Drug or chemical induced (E09)

–Due to an underlying condition (E08)

–Type 1 (E10)

–Type 2 (E11)

–Other specified diabetes mellitus (E13)

•The body system affected:

–Circulatory complications

–Hyperosmolarity

–Kidney complications

–Neurological complications

–Ophthalmic complications

–Other specified complications, which includes:

–arthropathy

–dermatitis

–hyperglycemia

–hypoglycemia

–oral complications

–skin ulcer

–Unspecified complications

–Without complications

•Specified complications/manifestations affecting that body system (See the documentation section on diabetes mellitus)

Diabetes Mellitus General Coding and Sequencing Guidelines

As many codes within a particular category as are necessary to describe all the complications of the diabetes mellitus may be assigned. Sequencing is based on the reason for the encounter. As many codes from categories E08-E13 as are necessary to completely identify all of the associated conditions that the patient has should be assigned.

Type 1 Diabetes Mellitus:

•The age of the patient is not the sole determining factor for a diagnosis of type 1 diabetes

•Type 1 diabetes typically develops before puberty and for this reason type 1 diabetes is also referred to as juvenile diabetes

Type of Diabetes Not Documented:

•If the type of diabetes mellitus is not documented in the medical record, the default is E11.- Type 2 diabetes mellitus.

Diabetes Mellitus and the Use of Insulin:

•If use of insulin is documented, but the type of diabetes mellitus is not documented:

–Assign a code from category E11 Type 2 diabetes mellitus

–Assign code Z79.4 Long-term (current) use of insulin to indicate that the patient uses insulin or assign code Z79.84 Long term (current) use of oral hypoglycemic drugs to indicate that the patient uses oral hypoglycemic drugs

–If the patient is treated with both oral medications and insulin, assign only the code for long term (current) use of insulin

–If the patient is treated with both insulin and an injectable non-insulin antidiabetic drug, assign codes Z79.4 Long-term (current) use of insulin, and Z79.899 Other long term (current) drug therapy. If the patient is treated with both oral hypoglycemics and an injectable non-insulin antidiabetic drug, assign codes Z79.84 Long-term (current) use of oral hypoglycemic drugs, and Z79.899 Other long-term (current) drug therapy

–Do not assign code Z79.4 if insulin is given temporarily to bring a Type 2 patient’s blood sugar under control during an encounter

Diabetes Mellitus in Pregnancy and Gestational Diabetes:

•For diabetes mellitus in pregnancy, see guidelines for Chapter 15 Pregnancy, Childbirth, and the Puerperium

Complications of Insulin Pump Malfunction:

•Underdose of insulin due to insulin pump failure:

–Principal/First-listed diagnosis code—Assign a code from subcategory T85.6- Mechanical complication of other specified internal and external prosthetic devices, implants and grafts to specify the type of pump malfunction (e.g., breakdown, displacement)

–Second code—Assign code T38.3x6- Underdosing of insulin and oral hypoglycemic [antidiabetic] drugs

–Additional codes—Assign additional codes to identify the type of diabetes mellitus and any associated complications due to the underdosing

•Overdose of insulin due to insulin pump failure:

–Second code—Assign code T38.3x1- Poisoning by insulin and oral hypoglycemic [antidiabetic] drugs, accidental (unintentional)

Secondary Diabetes Mellitus:

•Always caused by another condition or event (e.g., pancreatectomy, pancreatic cancer, cystic fibrosis, adverse effect of a drug, poisoning)

•Three categories, which include:

–E08 Diabetes mellitus due to underlying condition

–E09 Drug or chemical induced diabetes mellitus

–E13 Other specified diabetes mellitus

•Use of insulin

–For patients who routinely use insulin or oral hypoglycemic drugs, assign code Z79.4 Long term (current) use of insulin or Z79.84 Long term (current) use of oral hypoglycemic drugs

–If the patient is treated with both oral medications and insulin, assign only the code for long term (current) use of insulin

–Do not assign code Z79.4 for temporary use of insulin to bring a patient’s blood sugar under control during an encounter

•Assigning and sequencing secondary diabetes codes and its causes

–Sequencing of secondary diabetes codes in relation to causative codes is based on tabular instructions

»For E08 Diabetes mellitus due to underlying condition, the underlying condition is coded first followed by the diabetes code(s)

»For E09 Drug or chemical induced diabetes mellitus, the drug or chemical poisoning (T36-T65) is coded first followed by the diabetes code(s)

–Secondary diabetes due to pancreatectomy

»Principal/First listed diagnosis—Assign code E89.1 Postprocedural hypoinsulinemia

»Assign a code from category E13 Other specified diabetes mellitus

»Assign an additional code from subcategory Z90.41-Acquired absence of pancreas

»Assign code Z79.4 to identify any current, long-term use of insulin

–Secondary diabetes due to drugs

»May be caused by an adverse effect of correctly administered medications, by poisoning, or as a late effect of poisoning

»See guidelines in Chapter 19 Injury, poisoning, and certain other consequences of external causes and Chapter 20 External causes of morbidity for additional coding guidelines

General Documentation Requirements

There are some general documentation requirements related to coding endocrine, nutritional, and metabolic diseases. Like all body system chapters, intraoperative and postprocedural complications specifically affecting the endocrine system are listed within this body system chapter, although in two different categories, which will be discussed next. Gout is not listed here, but is classified to another body system. Both chronic types of gout and acute gout attacks are found within the musculoskeletal system chapter and must be reported by type, site, and the presence or lack of tophi.

Intraoperative and Postprocedural Complications NEC

Intraoperative and postprocedural complications are typically found in the same code block at the end of the chapter. However, for endocrine, nutritional, and metabolic diseases, these complications are handled differently. Because intraoperative complications apply only to the endocrine system, codes for intraoperative complications are found in category E36 at the end of the endocrine system section of Chapter 4. Postoperative complications apply to the endocrine system and to metabolic disorders and are found at the end of the chapter in category E89.

Intraoperative complications located in category E36 are those related to intraoperative hemorrhage and hematoma (E36.0-) and accidental puncture and laceration (E36.1-) of an endocrine system organ or structure. These codes are specific to the procedure performed which must be designated as complicating an endocrine system procedure or complicating a procedure on organs or structures in another body system. There is also a code for other specified intraoperative complications of the endocrine system (E36.8-).

Conditions in category E89 include those that relate to changes in endocrine function due to removal of part or all of the endocrine gland, or damage to the gland such as that caused by radiation therapy. Following removal or damage of an endocrine gland, hormones produced by that gland are absent or lower than normal. In some cases, these hormones must be replaced by medication. For example, in the case of hypoinsulinemia or hyperglycemia caused by removal of the pancreas, insulin must be administered. For other postprocedural conditions such as ovarian failure, replacement of the hormones is not always required. This category also reports postprocedural hemorrhage or hematoma. These codes differentiate between hemorrhage or hematoma that results from a procedure on an endocrine system organ or structure, and hemorrhage or hematoma that results from a procedure on another body system or organ.

Reclassification of Codes

Some conditions have traditionally or formerly been considered one particular type of disease or disorder, but were later determined to fit more appropriately in another group and have consequently been moved in their clinical listing. For instance, gout was formerly considered a metabolic disorder but is now classified as an inflammatory polyarthropathy along with conditions like rheumatoid arthritis, juvenile arthritis, and other crystal arthropathies in the musculoskeletal chapter. Some specific cerebral degenerative disorders were traditionally considered nervous system diseases, such as leukodystrophy and cerebral lipidoses, but are now listed as disorders of sphingolipid metabolism and other lipid storage disorders in category E75. Certain congenital conditions are also listed in category E78 Disorders of lipoprotein metabolism and other lipidemias that were formerly classified as congenital anomalies.

Combination Codes

As has already been discussed in the guidelines, diabetes mellitus codes are combination codes that include the type of diabetes, the body system affected, and the specific complication in that body system, as well as laterality, when applicable. It is not necessary to assign a code from the diabetes category and one or more codes from other chapters to identify the complications or manifestations of the diabetes, although additional codes may be used to identify the complication or manifestation with even greater clarity.

Code-Specific Documentation Requirements

In this section, the appropriate codes for the named condition are listed and their documentation requirements are identified. The focus is on frequently reported conditions with specific clinical documentation requirements, particularly those that may differ in their present reporting from formerly accepted classification. Though not all of the codes with updated documentation requirements are discussed, this section will provide a representative sample of the type of additional documentation required for coding endocrine, nutritional, and metabolic diseases. The section is organized alphabetically with related diseases or conditions grouped together.

Cushing’s Syndrome

Cushing’s syndrome is an endocrine disorder that occurs when the body is exposed to high levels of the hormone cortisol. This may result from excessive intake of corticosteroids (e.g., prednisone) or by an overproduction of cortisol in the adrenal glands (e.g., adrenal gland tumors). Overproduction of the hormone may also be triggered by an excess of ACTH production by the pituitary gland (e.g., pituitary tumor), or by ACTH-producing or cortisol-producing tumors in other areas of the body such as the lungs, pancreas, or thyroid.

Multiple codes are available for Cushing’s syndrome that identify the specific type or cause of Cushing’s syndrome (E24). Documentation must specifically state the condition as pituitary-dependent Cushing’s disease, drug-induced, alcohol-induced pseudo-Cushing’s syndrome, Nelson’s syndrome, Ectopic ACTH syndrome, or other specified type of Cushing’s syndrome for proper code assignment.

Coding and Documentation Requirements

Identify type of Cushing’s syndrome:

•Alcohol-induced pseudo-Cushing’s syndrome

•Drug-induced

•Ectopic ACTH syndrome

•Nelson’s syndrome

•Pituitary-dependent Cushing’s disease

•Other specified type of Cushing’s syndrome

•Unspecified Cushing’s syndrome

Use an additional code for drug-induced Cushing’s syndrome, if applicable, to identify the drug (T36-T50 with fifth or sixth character 5).

ICD-10-CM Code/Documentation
E24.0	Pituitary-dependent Cushing’s disease
E24.1	Nelson’s syndrome
E24.2	Drug-induced Cushing’s syndrome
E24.3	Ectopic ACTH syndrome
E24.4	Alcohol-induced pseudo-Cushing’s syndrome
E24.8	Other Cushing’s syndrome
E24.9	Cushing’s syndrome unspecified

Documentation and Coding Example

Intake Note: Forty-year-old Caucasian male admitted for inpatient detoxification from excessive alcohol use. He has always been a social drinker but began binge drinking approximately 12 weeks ago after the sudden death of his wife. Friends describe his moods as very erratic and he has been unable to work for the past 3 weeks. He is a chef and owns a number of high-end restaurants with a group of investors. On examination, this is an unkempt man of medium height and weight. His face has a round cushingoid appearance with supraclavicular fat pads present and a small fat pad at the base of neck/upper back. Blood is drawn for CBC, coagulation studies, comprehensive metabolic panel, serum cortisol, TSH, and toxicology screen. A urine sample is obtained for toxicology and a 24-hour urine collection for cortisol and creatinine is started. Salivary cortisol will be obtained this evening. There are numerous areas of ecchymosis in varying stages of healing, patient cannot recall exactly how or why they appeared. Patient states his last alcohol intake was about 2 hours ago. He denies use of prescription, OTC, or illegal drugs. He is oriented to the facilities and his individual program.

Progress Note: Labs are significant for elevated salivary cortisol, serum transaminase, and BGL. WBCs moderately elevated at 15,000 with no left shift to indicate acute infection, serum potassium is low. Still awaiting 24-hour urine cortisol and creatinine results. Urine and serum toxicology screen positive for ethanol only.

Diagnosis: Episodic ethanol abuse with alcohol-induced pseudo-Cushing’s syndrome.

Plan: Ethanol detoxification and behavioral therapy. Monitor labs closely. Anticipate 30 day inpatient stay.

Diagnosis Code(s)

F10.10	Alcohol abuse, uncomplicated
E24.4	Alcohol-induced pseudo-Cushing’s syndrome

Coding Note(s)

The patient is admitted for treatment of his alcohol abuse. The pseudo-Cushing’s syndrome is a secondary diagnosis. Category F10 reports alcohol related disorders and there are three subcategories—F10.1 Alcohol abuse; F10.2 Alcohol dependence; and F10.9 Alcohol use, unspecified. The physician must clearly document the condition being treated. In this case, the diagnosis is alcohol abuse without any accompanying mental or behavioral complications that would be reported in a combination code. The only related complication identified is the pseudo-Cushing’s syndrome, so code F10.10 is reported. There is also a code for alcohol abuse with other alcohol-induced disorders (F10.188), but codes in Chapter 5 are reported for mental and behavioral disorders, not for other physical or physiological medical conditions associated with the alcohol abuse, so code F10.188 would not be appropriate. The endocrine condition caused by the alcohol abuse is reported with a code from the endocrine chapter, E24.4. There is a note under category F10 to use additional code for blood alcohol level, if applicable. No blood alcohol level has been documented here, but if it had been provided, a code from category Y90 would also be reported.

Diabetes Mellitus

In ICD-10-CM, code categories include diabetes mellitus due to an underlying condition (E08); drug or chemical induced diabetes mellitus (E09); Type 1 diabetes mellitus (E10); Type 2 diabetes mellitus (E11); and other specified diabetes mellitus (E13). Other specified types of diabetes mellitus include diabetes due to identified genetic defects, such as defects of beta cell function or insulin action, as well as secondary diabetes due to surgery (postpancreatectomy, postprocedural). Diabetes not specified as due to an underlying cause and not specified as Type 1 or Type 2 is reported as Type 2 diabetes by default. Since these categories are available for identifying and reporting differing types of diabetes, it is important for the underlying cause of the diabetes to be documented.

Diabetes codes also capture the body system affected in the 4th character with the complication or manifestation typically identified by the 5th character. The 6th character allows for capture of multiple complications or manifestations often seen together such as diabetic retinopathy with macular degeneration. The 7th character denotes laterality, such as left eye or right eye. Each individual code does not, however, capture whether or not the diabetes is controlled or uncontrolled. Diabetes documented as uncontrolled or poorly controlled is captured by reporting an additional code for hyperglycemia. For example, Type II diabetes documented as poorly controlled as evidenced by elevated blood sugar (hyperglycemia) would be reported with code E11.65. As many diabetes codes as are necessary to report the complete clinical picture of all manifestations and complications of the diabetic patient should be reported.

The underlying condition, or drug or chemical poisoning identified as the cause of secondary diabetes is reported as the first-listed diagnosis with the secondary diabetes code listed next. Fourth character subcategories for secondary diabetes identify the body system affected by the complication, with the specific complication or manifestation identified by the fifth character. Some secondary diabetes codes capture multiple complications or manifestations with the sixth character, and some report laterality with a seventh character. This means that to capture the most specific code, providers will need to document not only the underlying cause but also clearly describe all complications and manifestations. Documenting any long-term insulin use is also required.

Capturing the correct code for diabetes mellitus requires clear and precise documentation of the underlying cause. Secondary diabetes is defined as a diabetic condition with an underlying cause other than genetics or environmental conditions and includes diabetes mellitus secondary to drugs and chemicals or due to an underlying disease, medical condition, surgical procedure, or trauma. Some of the drugs and chemical agents identified as causing secondary diabetes include:

•Anticonvulsants

•Antihypertensive drugs including diuretics and beta blockers

•Antipsychotics including lithium and some antidepressants

•Antiretroviral drugs

•Chemotherapy drugs

•Hormone supplements, including:

–Anabolic steroids

–Contraceptives

–Estrogen

–Growth hormones

–Hormones prescribed for prostate cancer

•Immunosuppressive drugs including corticosteroids

Some of the other causes of secondary diabetes include:

•Autoimmune diseases

•Carcinoid tumors of some sites, including:

–Gastrointestinal tract

–Lungs

•Endocrine disorders, including:

–Cushing’s syndrome

–Excessive levels of growth hormones

–Hyperthyroidism

•Hemochromatosis

•Liver diseases, including:

–Hepatitis C

–Fatty liver disease

•Pancreatic disease or injury, including:

–Chronic pancreatitis

–Pancreatic cancers

–Pancreatic damage due to malnutrition

–Other endocrine diseases that affect pancreatic function or damage the insulin-producing beta cells

•Trauma to the pancreas

Surgical procedures may also result in secondary diabetes mellitus. Procedures most often associated with secondary diabetes include total or partial removal of the pancreas for malignant neoplasm or severe pancreatic disease, or orchiectomy performed for testicular or prostate cancer.

Coding and Documentation Requirements

Identify the type of diabetes mellitus:

•Drug or chemical induced

•Due to underlying condition

•Type 1

•Type 2

•Other specified diabetes mellitus, which includes:

–Due to genetic defects of beta-cell function

–Due to genetic defects in insulin action

–Postpancreatectomy

–Postprocedural

–Secondary diabetes not elsewhere classified

Identify the body system affected, the manifestations or complications, and laterality, when applicable:

•Arthropathy

–Neuropathic

–Other arthropathy

•Circulatory complications

–Peripheral angiopathy

»With gangrene

»Without gangrene

–Other specified circulatory complication

•Hyperglycemia

•Hyperosmolarity

–with coma

–without coma

•Hypoglycemia

–With coma

–Without coma

•Ketoacidosis

–With coma

–Without coma

•Kidney complications

–Diabetic nephropathy

–Chronic kidney disease

–Other diabetic kidney complication

•Neurological complications

–Amyotrophy

–Autonomic (poly)neuropathy

–Mononeuropathy

–Polyneuropathy

–Other specified neurological complication

–Unspecified diabetic neuropathy

•Ophthalmic complications

–Diabetic retinopathy

»Mild nonproliferative

◦With macular edema

◦Without macular edema

»Moderate nonproliferative

◦With macular edema

◦Without macular edema

»Severe nonproliferative

◦With macular edema

◦Without macular edema

»Proliferative

◦With macular edema

◦Without macular edema

◦With traction retinal detachment involving the macula

◦With traction retinal detachment not involving the macula

◦With combined traction retinal detachment and rhegmatogenous retinal detachment

»Stable proliferative

»Unspecified diabetic retinopathy

◦With macular edema

◦Without macular edema

–Diabetic macular edema, resolved following treatment

–Laterality

»Right eye

»Left eye

»Bilateral

»Unspecified eye

–Diabetic cataract

–Other diabetic ophthalmic complication

•Oral complications

–Periodontal disease

–Other oral complications

•Skin complication

–Dermatitis

–Foot ulcer

–Other skin ulcer

–Other skin complication

•Other specified complication

•Unspecified complication

•Without complications

Example 1

Type 1 Diabetes Mellitus with Ophthalmic Manifestations

ICD-10-CM Code/Documentation
E10.311	Type 1 diabetes mellitus with unspecified diabetic retinopathy with macular edema	E10.319	Type 1 diabetes mellitus with unspecified diabetic retinopathy without macular edema
E10.321-	Type 1 diabetes mellitus with mild nonproliferative diabetic retinopathy with macular edema	E10.329-	Type 1 diabetes mellitus with mild nonproliferative diabetic retinopathy without macular edema
E10.331-	Type 1 diabetes mellitus with moderate nonproliferative diabetic retinopathy with macular edema	E10.339-	Type 1 diabetes mellitus with moderate nonproliferative diabetic retinopathy without macular edema
E10.341-	Type 1 diabetes mellitus with severe nonproliferative diabetic retinopathy with macular edema	E10.349-	Type 1 diabetes mellitus with severe nonproliferative diabetic retinopathy without macular edema
E10.351-	Type 1 diabetes mellitus with proliferative diabetic retinopathy with macular edema	E10.359-	Type 1 diabetes mellitus with proliferative diabetic retinopathy without macular edema
E10.352-	Type 1 diabetes mellitus with proliferative diabetic retinopathy with traction retinal detachment involving the macula	E10.353-	Type 1 diabetes mellitus with proliferative diabetic retinopathy with traction retinal detachment not involving the macula
E10.354-	Type 1 diabetes mellitus with proliferative diabetic retinopathy with combined traction retinal detachment and rhegmatogenous retinal detachment	E10.355-	Type 1 diabetes mellitus with stable proliferative diabetic retinopathy
E10.36	Type 1 diabetes mellitus with diabetic cataract	E10.39	Type 1 diabetes mellitus with other diabetic ophthalmic complication
E10.37X-	Type 1 diabetes mellitus with diabetic macular edema, resolved following treatment	Note:	Subcategories E10.32-E10.35 and E10.37 require a 7^th digit to denote laterality:
		1	right eye
		2	left eye
		3	bilateral
		9	unspecified eye

Documentation and Coding Example

Thirty-eight-year-old Caucasian female is seen in ophthalmology for ongoing diabetes related eye problems. Patient experienced onset of Type I diabetes mellitus at age seven. She has experienced rare episodes of DKA and has kept fairly tight glycemic control using an insulin pump for the past 10 years. She has no renal, hepatic, or peripheral vascular involvement, no elevated lipids or triglycerides. Last HbA1c was 6.5 %. She had stable, mild nonproliferative diabetic retinopathy for many years with no visual acuity problems; however, patient is now 6 months post-delivery of a healthy baby boy and during her pregnancy her retinopathy advanced to moderate nonproliferative in both eyes, and she developed signs of macular edema in her right eye. Fundoscopy exam performed today under stereopsis and high magnification reveals stable areas of microaneurysms in left eye and no evidence of macular edema. Exam of right eye shows microaneurysms and retinal hemorrhage, thickening, and focal edema consistent with macular edema. These findings are virtually unchanged from 3 months ago. Near and far visual acuity remains at 20/30 in each eye. Patient is advised that she is not a candidate for treatment at this time. She is encouraged to continue with her excellent control of BGL and reminded to see her endocrinologist regularly. She will return in 3 months for continued monitoring of her retinopathy and macular edema.

Diagnosis Code(s)

E10.3311	Type 1 diabetes mellitus with moderate nonproliferative diabetic retinopathy with macular edema, right eye
E10.3392	Type 1 diabetes mellitus with moderate nonproliferative diabetic retinopathy without macular edema, left eye

Coding Note(s)

A combination code captures the Type 1 diabetes mellitus, the body system affected (eye), and the specific complication, which in this case includes both moderate non-proliferative diabetic retinopathy and macular edema, present only in the right eye. Laterality is a component of diabetes codes with all types of diabetic retinopathy and also for cases of diabetic macular edema resolved following treatment. Since the left eye shows no signs of macular edema, the code for moderate non-proliferative diabetic retinopathy without macular edema is reported.

Example 2

Secondary Diabetes with Neurological Complications

ICD-10-CM Code/Documentation
E08.41	Diabetes mellitus due to underlying condition with diabetic mononeuropathy	E08.40	Diabetes mellitus due to underlying condition with diabetic neuropathy, unspecified
E09.41	Drug or chemical induced diabetes mellitus with neurological complications with diabetic mononeuropathy	E09.40	Drug or chemical induced diabetes mellitus with neurological complications with diabetic neuropathy, unspecified
E13.41	Other specified diabetes mellitus with diabetic mononeuropathy	E13.40	Other specified diabetes mellitus with diabetic neuropathy, unspecified
E08.43	Diabetes mellitus due to underlying condition with diabetic autonomic (poly) neuropathy	E08.42	Diabetes mellitus due to underlying condition with diabetic polyneuropathy
E09.43	Drug or chemical induced diabetes mellitus with neurological complications with diabetic autonomic (poly)neuropathy	E09.42	Drug or chemical induced diabetes mellitus with neurological complications with diabetic polyneuropathy
E13.43	Other specified diabetes mellitus with diabetic autonomic (poly)neuropathy	E13.42	Other specified diabetes mellitus with diabetic polyneuropathy
E08.49	Diabetes mellitus due to underlying condition with other diabetic neurological complication	E08.44	Diabetes mellitus due to underlying condition with diabetic amyotrophy
E09.49	Drug or chemical induced diabetes mellitus with neurological complications with other diabetic neurological complication	E09.44	Drug or chemical induced diabetes mellitus with neurological complications with diabetic amyotrophy
E13.49	Other specified diabetes mellitus with other diabetic neurological complication	E13.44	Other specified diabetes mellitus with diabetic amyotrophy
		E08.610	Diabetes mellitus due to underlying condition with diabetic neuropathic arthropathy
		E09.610	Drug or chemical induced diabetes mellitus with diabetic neuropathic arthropathy
		E13.610	Other specified diabetes mellitus with diabetic neuropathic arthropathy

Documentation and Coding Example

Forty-four-year-old Caucasian female is seen by PMD for c/o fatigue, dizziness, and unusual sweating. Patient has a 20 + year history of excessive alcohol use and was diagnosed with chronic calcifying pancreatitis 6 years ago resulting from her excessive alcohol consumption. She developed diabetes 2 years ago as a result of the pancreatic disease. She has cut down on her consumption of alcohol but continues to drink 2-3 glasses of wine daily. Diabetes is fairly well controlled on Lantus and Novolog insulin. She was experiencing low BG levels with Lantus at HS but less since the dose was split and she is injecting BID. WT 126 lbs., T 97.8, P 62, R 12, BP 104/50, O2 Sat 98% on RA, BGL=206. On examination, this is a thin, athletic appearing woman who looks her stated age. Skin is very tan, mucous membranes moist and pink, eyes are clear. She states she normally is able to play tennis or work out at the gym daily for 60-90 minutes. For the past month she finds she is exhausted after exercising and in the past week has become fatigued during the workout and has to stop. She has also noticed excessive perspiration even when she is not active. The dizziness occurs upon rising from bed and sometimes from sitting to standing. She denies falls. PERRLA, neck supple. HR regular. Pulses full and intact in extremities. No evidence of edema. Reflexes 2 + and muscle tone is good. Breath sounds clear, equal bilaterally. Abdomen is soft and flat, bowel sounds present in all quadrants. Supine BP 110/52, Sitting 90/50, Standing 90/52.

Impression: Diabetes due to chronic calcifying pancreatitis with related new onset autonomic neuropathy, orthostatic hypotension, and excessive sweating.

Plan: Increase salt in diet. Refer to cardiology for echocardiogram and treadmill to assess HR and BP during exercise. She is again counseled about her use of alcohol including availability of inpatient treatment programs for her dependency. She declines these services.

Note electronically sent to Cardiology and Endocrinologist. Follow up after Cardiology workup. Sooner if symptoms worsen.

Diagnosis Code(s)

K86.0	Alcohol-induced chronic pancreatitis
E08.43	Diabetes mellitus due to underlying condition with diabetic autonomic (poly)neuropathy
F10.20	Alcohol dependence, uncomplicated
R53.83	Other fatigue
I95.1	Orthostatic hypotension
R61	Generalized hyperhidrosis
Z79.4	Long term (current) use of insulin

Coding Note(s)

This patient has secondary diabetes which is due to chronic calcifying pancreatitis which has been more specifically documented as being caused by excessive alcohol use and dependence. She has developed a new manifestation of autonomic neuropathy which is due to her secondary diabetes. There is a code first note for category E08 indicating that the code for the underlying condition is listed first and the secondary diabetes code is listed as the second diagnosis.

Codes for secondary diabetes are combination codes that identify the body system affected and the type of complication, which has been identified as new onset diabetic autonomic neuropathy. The manner in which the diabetic autonomic neuropathy is manifesting could be any number of specified conditions, and since the actual neuropathic complication the patient is experiencing is not included in the code title, additional codes that further identify the specific autonomic complication(s) may also be reported.

Example 3

Diabetes with Gastroparesis

ICD-10-CM Code/Documentation
E08.43	Diabetes mellitus due to underlying condition with diabetic autonomic (poly)neuropathy
E09.43	Drug or chemical induced diabetes mellitus with neurological complications with diabetic autonomic (poly)neuropathy
E10.43	Type 1 diabetes mellitus with diabetic autonomic (poly)neuropathy
E11.43	Type 2 diabetes mellitus with diabetic autonomic (poly)neuropathy
E13.43	Other specified diabetes mellitus with diabetic autonomic (poly)neuropathy

Note: All diabetes codes reporting diabetic autonomic (poly) neuropathy have an inclusion term listed underneath for that particular type of diabetes stating ‘with diabetic gastroparesis’.

Documentation and Coding Example

Patient is a 60-year-old Hispanic female referred to GI Clinic by PMD for weight loss, bloating, and constipation. PMH is significant for Type 2 diabetes diagnosed 5 years ago and well controlled on Metformin 850 mg BID. Patient was not obese when she was diagnosed with diabetes. LADA was considered but she was subsequently found to have elevated C-Peptide levels and negative ICA, IAA, and GAD tests with no family history of autoimmune disorders. HT 63 inches, WT 94 lbs. T 96.7, P 88, R 14, BP 132/88, BGL=133. On examination, this is a thin, cachectic appearing woman who looks older than her stated age. Skin is sallow, eyes dull, hair is dry and thin. Patient states she has had a 15 lb. weight loss in 2 months due to early satiety and frequent indigestion. She also states her bowel movements have become less frequent, large and hard to pass. Oral mucosa dry and pink, throat without redness. Apical pulse normal. Breath sounds clear. Abdomen mildly distended. Bowel sounds hypoactive. There is no evidence of ascites. No pain or tenderness with palpation, liver at 2 cm below RCM, spleen at LCM. No hernia appreciated. Anal sphincter has good tone, the rectum is full of hard stool. Labs including cortisol, TSH and T4 ordered by PMD are normal.

Impression: New onset gastroparesis with underlying Type 2 diabetes.

Plan: EGD and colonoscopy to rule out obstruction or other disease process.

Procedure note: Patient had a satisfactory bowel prep and was NPO for procedure. IV started in right hand with LR infusing. Patient sedated with Versed and Fentanyl. EGD showed normal esophagus and stomach. Pyloric sphincter somewhat stiff but scope was passed into duodenum without difficulty. The duodenum has a few patchy areas of inflammation but no ulcer. The endoscope was withdrawn and with patient positioned on left side, the colonoscopy was performed without incident. There are no polyps, narrowing, or inflammation from rectum to cecum. A large hemorrhoid is present just inside the rectal sphincter.

Diagnosis: Gastroparesis

Plan: Start metoclopramide 10 mg QID, 30 minutes before meals and at bedtime. Citrucel daily. Patient would benefit from nutritional counseling since she is now underweight and she has not spoken to the dietician since her diabetes diagnosis 5 years ago. She also needs a referral to new endocrinologist, due to change in insurance. Follow up in GI clinic in 1 month.

Note electronically sent to PMD.

Diagnosis Code(s)

E11.43	Type 2 diabetes mellitus with diabetic autonomic (poly)neuropathy
K31.84	Gastroparesis

Coding Note(s)

Even though only a single code is technically required to report the type 2 diabetes with gastroparesis, since there is an inclusion term listed under code E11.43 that states “Type 2 diabetes mellitus with diabetic gastroparesis,” this specific manifestation is not identified in the code title. Because the manner in which a diagnosis of diabetic autonomic neuropathy manifests in a person could be any number of specified conditions, it is appropriate to assign an additional code to identify the gastroparesis in this patient. This is supported by AHA Coding Clinic.

Example 4

Secondary Diabetes with Foot Ulcer

ICD-10-CM Code/Documentation
E08.621	Diabetes mellitus due to underlying condition with foot ulcer
E09.621	Drug or chemical induced diabetes mellitus with foot ulcer
E13.621	Other specified diabetes mellitus with foot ulcer WITH: A code from L97.4- or L97.5- to designate site, laterality, and depth of non-pressure chronic ulcer

Documentation and Coding Example

Thirty-one-year-old Caucasian male is referred to the Wound Care Clinic by PMD for left foot ulcer. Patient has been insulin dependent x 6 years after undergoing total pancreatectomy due to blunt force trauma of the abdomen following a bicycle accident. He is very physically active and even on low doses of insulin he experiences frequent episodes of hypoglycemia. Patient is married with 2 young children and works for the county as a building inspector. Patient states he is an avid runner and sees a podiatrist regularly for toenail trimming and callus shaving. He states he noticed discomfort in left great toe 2 weeks ago but attributed it to an increase in his workouts as he trained for an event. After completing the event, he had increased pain in the toe and noticed the skin on the bottom of the toe looked red and swollen. Epson salt soaks did not relieve the inflammation or discomfort, the skin began peeling and he noticed yellow drainage on his soak. He was seen by PMD 2 days ago who did a culture and x-ray, ordered blood tests, and referred for wound care. HT 69 inches, WT 155 lbs., T 98.6, P 58, R 12, BP 104/64. On examination, this is a thin but muscular man who looks younger than his stated age. Both lower extremities have intact circulation, sensation, and movement. Pedal, dorsalis pedis, and posterior tibial pulses are all strong and equal. CRT 2-3 seconds. No sign of dystrophic nails or loss of hair. Both feet have thin plantar calluses at the edge of heels, ball of foot, and distal end of great toes. There is localized cellulitis at distal end of left great toe, with a 1 x 1 cm crater on dorsal surface that appears fairly superficial. There is a rim of hyperkeratotic tissue surrounding the crater with thick yellow exudate in the center. X-ray is reviewed and shows no bone involvement. CBC, serum glucose, glycohemoglobin, creatine, and HbA1c are all within normal limits. Culture is positive for Staph aureus, sensitive to cephalexin.

Impression: Neurogenic foot ulcer limited to skin breakdown due to diabetes. Superimposed staph infection.

Plan: Debridement, walking boot, Keflex 500 mg QID x 10 days. RTC in 1 week. Note electronically sent to PMD.

Diagnosis Code(s)

E89.1	Postprocedural hypoinsulinemia
E13.621	Other specified diabetes mellitus with foot ulcer
L97.521	Non-pressure chronic ulcer of other part of left foot limited to breakdown of skin
L03.032	Cellulitis of left toe
B95.61	Methicillin susceptible Staphylococcus aureus infection as the cause of diseases classified elsewhere
Z90.410	Acquired total absence of pancreas
S36.209S	Unspecified injury of unspecified part of pancreas, sequela
Z79.4	Long term (current) use of insulin
V19.9XXS	Pedal cyclist (driver) (passenger) injured in unspecified traffic accident, sequela

Coding Note(s)

This patient had a total pancreatectomy following a blunt trauma injury to the pancreas that resulted from a bicycle accident. Conditions such as secondary diabetes and complications of diabetes are sequela of the accident that necessitated the pancreatectomy. An injury code should be assigned with 7th character S to designate the sequela. In ICD-10-CM, an external cause code may also be assigned with 7th character S to designate the external cause of the sequela. Sequencing of secondary diabetes codes is based on Tabular List instructions and the official guidelines. Sequencing instructions in the Tabular List take precedence over sequencing instructions in the official guidelines.

In category E13 in the Tabular List, there are no instructions related to sequencing of codes. However, there are sequencing instructions in the official guidelines for secondary diabetes due to pancreatectomy. Code E89.1 is listed first to identify the postprocedural hypoinsulinemia. A code from category E13 is listed additionally along with a code from subcategory Z90.41- to identify the acquired absence of the pancreas.

The diabetes code E13.621 identifies the type of diabetes, body system affected, and the specific manifestation/complication as a foot ulcer. A second code is required to identify the specific site of the foot ulcer, and its stage. Even though the foot ulcer is not documented as chronic, it is documented as neurogenic and this type of ulcer codes to non-pressure chronic ulcer. The depth of the ulcer is described as superficial and limited to skin breakdown.

There is an Excludes2 note listed under category L97 for skin infections (L00-L08). However, since the patient has both a dorsal surface ulcer and a superimposed infection at the distal end of the same left toe, it is appropriate to assign both codes. An additional code is assigned to identify the causative agent as Staphylococcus aureus. In this case, the code for Methicillin susceptible S. aureus is used because the infection is sensitive to Cephalexin which is a cephalosporin. Cephalosporins are classified as beta lactam antibiotics and S. aureus infections that can be treated with beta lactam antibiotics, are classified as methicillin susceptible infections.

Example 5

Secondary Diabetes Mellitus with Kidney Complications

ICD-10-CM Code/Documentation
E08.21	Diabetes mellitus due to underlying condition with diabetic nephropathy
E08.22	Diabetes mellitus due to underlying condition with diabetic chronic kidney disease
E08.29	Diabetes mellitus due to underlying condition with other diabetic kidney complication
E09.21	Drug or chemical induced diabetes mellitus with diabetic nephropathy
E09.22	Drug or chemical induced diabetes mellitus with diabetic chronic kidney disease
E09.29	Drug or chemical induced diabetes mellitus with other diabetic kidney complication
E13.21	Other specified diabetes mellitus with diabetic nephropathy
E13.22	Other specified diabetes mellitus with diabetic chronic kidney disease
E13.29	Other specified diabetes mellitus with other diabetic kidney complication

Documentation and Coding Example

Forty-nine-year-old Black male is referred to nephrology by PMD for new onset fatigue and loss of appetite with elevated serum creatinine, BUN, and proteinuria. PMH is significant for asthma since childhood, often requiring steroids, HIV infection documented at age 25 and diabetes, onset nine years ago following pentamidine administration for acute Pneumocystis carinii infection. Patient is a dancer/choreographer, active in theater and performing arts. He is accompanied to the appointment today by his partner of 20 years. Current medications include Lantus Insulin 12 units HS, Novolog sliding scale AC and HS for BG correction and carbohydrate intake, Singulair 5 mg at HS, Advair Diskus 500/50 BID and Stribild 1 capsule q AM. HT 70 inches, WT 160 lbs., T 98.8, P 72, R 14, BP 140/90, O2 Sat on RA 97%, BG=112. On examination, this is a thin, muscular, immaculately groomed, articulate gentleman who appears relaxed, alert, and oriented. Patient states he has been able to maintain his active lifestyle until just a few weeks ago and thought his fatigue was simply due to holiday travel and entertainment. He had a regularly scheduled appointment with his PMD so he just planned to mention his symptoms at that time. He had routine labs done a week prior to that appointment and his doctor saw him emergently for abnormal findings. PMD’s note and all labs are available for review at this appointment. PERRLA, cranial nerves grossly intact. Neck supple without lymphadenopathy. Mucous membranes moist and pink, nares patent, TMs clear. HR regular without bruit, rub, or murmur. Breath sounds have occasional soft expiratory wheeze. CSM intact to extremities, no edema noted. Abdomen soft and flat, BS active all quadrants. Liver is palpated 1 cm below RCM, spleen 2 cm below LCM. No hernia appreciated, testes down, circumcised penis without urethral drainage. Renal ultrasound performed. Findings consistent with Kimmelstiel-Wilson disease.

Impression: Secondary diabetes due to pentamidine therapy status post 9 years ago with new onset Kimmelstiel-Wilson disease secondary to diabetes.

Plan: Discuss findings with PMD/Infectious Disease including current use of Stribild which is contraindicated with creatinine >50. Repeat labs in 2 weeks with follow up appointment at that time.

Diagnosis Code(s)

E09.21	Drug or chemical induced diabetes mellitus with diabetic nephropathy
T37.3X5S	Adverse effect of other antiprotozoal drugs, sequela
B20	Human immunodeficiency virus [HIV] disease
Z79.4	Long term (current) use of insulin

Coding Note(s)

Pentamidine is an antiprotozoal drug that may cause severe fasting hypoglycemia as an adverse effect. Some patients who have experienced severe fasting hypoglycemia due to pentamidine administration later develop insulin dependent diabetes as a sequela (late effect) of pentamidine. Diabetes that develops as a sequela of a chemical or drug is coded as secondary diabetes. The secondary diabetes code is listed first as Tabular instructions under category E09 state to use additional code for adverse effect, if applicable, to identify the drug with fifth or sixth character 5. The patient has a documented previous admission for an HIV-related illness so the code for HIV disease is assigned for every encounter. Sequencing of HIV disease is dependent on the reason for the encounter. In this case, the patient is being seen in consultation for new onset kidney disease related to diabetes, so the HIV cannot be listed first.

The combination code E09.21 captures the type of diabetes and the body system affected as well as the nature of the complication. In this case, it is Kimmelstiel-Wilson disease, which is listed as an inclusion term under code E09.21.

Example 6

Type 1 Diabetes Mellitus Poorly Controlled (with Hyperglycemia)

ICD-10-CM Code/Documentation
E10.65	Type 1 diabetes mellitus with hyperglycemia

Documentation and Coding Example

Thirteen-year-old Caucasian male is seen in Pediatric Endocrine Clinic accompanied by his mother. Patient is well known to this practice. He was diagnosed with Type 1 diabetes at age 7, presenting with acute DKA and a BGL of 535. He has had multiple hospitalizations for HHS and DKA in the past 5 years, largely due to patient non-compliance and poor follow through of mother. The family was referred to CWS 2 years ago when the patient, unsupervised by mother, gave an incorrect dose of insulin causing severe hypoglycemia and seizure. The family received counseling and support and for the most part they have been compliant with care.

Review of labs: HbA1c elevated at 7.7 and triglycerides at 360. Urine and serum positive for ketones. Anion gap 11. Kidney and liver function tests moderately elevated. HT 65 inches, WT 145 lbs., T 98.4, P 76, R 14, BP 110/66, O2 Sat on RA 99, BGL=182. On examination, this is a somewhat sullen and uncooperative adolescent male who communicates only a little better when his mother is asked to leave the room. Patient states he will be finishing the 7th grade in 2 months, his grades are passing, his main interest is skateboarding and he is largely unsupervised in his diabetes care and food preparation because his mother works all the time. PERRLA, skin is oily, he has mild acne on his forehead and chin, hair is clean and cut short. Oral mucosa moist and pink, posterior pharynx mildly red with cobblestone appearance and clear mucus drainage noted. Patient has a history of seasonal allergies treated with Zyrtec. He states he has not taken it in over a week because he ran out and forgot to tell his mother. Neck supple with no lymphadenopathy. HR regular. BS clear and equal. Abdomen soft and flat. Liver palpated at RCM, spleen is not palpated. No evidence of hernia, normal genitalia. CSM intact in both upper and lower extremities. He has areas of bruising and scabs on his right forearm and right knee that he states is from a fall while skateboarding. Patient states he is taking Lantus insulin 22 units at bedtime and Humalog with meals. He does check his BGL in the health office at school and doses for BGL and carbs with the assistance of a school nurse but admits to not always checking at other times. His morning meal is cold cereal, lunch is a sandwich or cheeseburger bought from school cafeteria and he snacks on diet soda, chips, and ice cream. Dinner is brought home by his mother usually pizza, burgers and fries, tacos, or chicken fingers. He does not have a very concrete understanding of carbohydrate counts for the foods he frequently consumes and appears to be under dosing much of the time. Patient and mother are counseled together regarding the need for tighter control of his diabetes and the health risks for his hyperglycemia.

Impression: Type 1 diabetes poorly controlled with frequent episodes of hyperglycemia. Noncompliant with diet and BGL control.

Plan: See dietician for food counseling. Patient to attend a one-week summer camp program for diabetic youth, scholarship will be arranged. Mother is given information on parent support groups. Patient will do daily AC and HS BG monitoring and keep a log. Mother will call his numbers in weekly along with insulin doses and carbohydrate calculations. RTC in 1 month. Patient will have blood drawn for CBC, comprehensive metabolic panel, cholesterol and lipids at least 2 days prior to appointment.

Diagnosis Code(s)

E10.65	Type 1 diabetes mellitus with hyperglycemia
Z91.11	Patient’s noncompliance with dietary regimen
Z91.14	Patient’s other noncompliance with medication regimen

Coding Note(s)

Diabetes described as poorly controlled or uncontrolled is reported with the code for the specific type of diabetes with hyperglycemia. Noncompliance is reported additionally, with codes for both dietary regimen and medication regimen. There are specific codes for noncompliance including codes for intentional and unintentional underdosing, and also other noncompliance. The physician has not stated whether the underdosing of insulin is intentional or unintentional, indicating that the patient does not appear to understand carbohydrate counts and appears to be underdosing. For this reason, the code Z91.14 Patient’s other noncompliance with medication regimen is reported, which includes underdosing of medication NOS.

Lipoid Metabolism Disorders

Lipids are fats present in the body. Lipid metabolism refers to the breakdown of lipids into constituents that can be used by the body. Some genetic and medical conditions can cause dysfunction of lipid metabolism. The exact manifestations of dysfunction in lipid metabolism depend on the specific metabolic disorder. Some specific types of lipids include lipoproteins, sphingolipids, glycosaminoglycans, and glycoproteins.

Lipoprotein metabolic disorders and lipidemias are some of the most common types of lipoid metabolic disorders. Two types of lipoproteins found in the blood are cholesterol and triglycerides. Elevated levels of one form of cholesterol called low density lipoprotein (LDL), along with elevated levels of triglycerides, are strongly linked to atherosclerotic heart disease. Disorders of lipoprotein metabolism are often inherited diseases that cause elevated levels of remnant lipoproteins in the blood and deposits in body organs. These diseases include Barth syndrome, Smith-Lemli-Opitz syndrome, hyperchylomicronemia, familial hypercholesterolemia, pure hyperglyceridemia, and mixed and familial combined hyperlipidemia.

Sphingolipids are a class of lipids made of fatty acid derivatives of sphingosine (e.g., ceramides, gangliosides, sphingomyelins, and cerebrosides) occurring mainly in the brain and are a constituent of nervous tissue. Sphingolipid metabolic disorders are usually monogenetic inherited diseases resulting in enzymatic defects in lysosomal sphingolipid degradation. The defects cause an accumulation of lipids in one or more body organs. Diseases caused by sphingolipid metabolic disorders include Tay-Sachs, Sandhoff, Fabry-Anderson, Gaucher, Krabbe, and Niemann-Pick. Sphingolipid disorders may be treated with enzyme replacement therapy (ERT), cell mediated therapy (CMT) including bone marrow transplant (BMT), cell mediated cross correction, and gene therapy.

Glycosaminoglycan metabolic disorders are usually inherited genetic diseases that cause a failure in the breakdown of long chain sugar molecules causing them to accumulate and damage body organs. Diseases caused by glycosaminoglycan metabolic disorders include Hurler, Hurler-Scheie, Scheie, Morquio, and Sanfilippo. Glycosaminoglycan disorders are most often treated with enzyme replacement therapy (ERT).

Glycoprotein metabolic disorders are rare inherited genetic diseases characterized by a failure to synthesize or degrade glycoprotein molecules, causing them to accumulate in body organs and tissue.

ICD-10-CM includes multiple categories with subclassified specificity for many types of conditions—particularly those formerly known as lipidoses. Codes for conditions previously identified as disorders of lipoid metabolism are classified in the following 5 categories:

•E75Disorders of sphingolipid metabolism and other lipid storage disorders

•E76Disorders of glycosaminoglycan metabolism

•E77Disorders of glycoprotein metabolism

•E78Disorders of lipoprotein metabolism and other lipidemias

•E88Other and unspecified metabolic disorders

Coding and Documentation Requirements

Identify the disorder:

•Defects of glycoprotein metabolism

–Defects in glycoprotein degradation

–Defects in post-translational modification of lysosomal enzymes

–Other specified disorders in glycoprotein metabolism

–Unspecified disorder of glycoprotein metabolism

•Disorders of glycosaminoglycan metabolism

–Mucopolysaccharidosis, type I

»Hurler syndrome

»Hurler-Scheie syndrome

»Scheie syndrome

–Mucopolysaccharidosis, type II

–Other mucopolysaccharidoses

»Morquio A

»Morquio B

»Morquio, unspecified

»Sanfilippo

»Other specified types

»Unspecified type

–Other specified disorders of glycosaminoglycan metabolism

–Unspecified disorder of glycosaminoglycan metabolism

•Disorders of sphingolipid metabolism and other lipid storage disorders

–Gangliosidosis

»GM2 type

◦Sandhoff disease

◦Tay-Sachs disease

◦Other specified GM2 type

◦Unspecified GM2 type

»Other and unspecified type

◦Mucolipidosis IV

◦Other gangliosidoses (includes GM1, GM3)

◦Unspecified type gangliosidosis

–Neuronal ceroid lipofuscinosis

–Sphingolipidosis

»Fabry (-Anderson) disease

»Gaucher disease

»Krabbe disease

»Niemann-Pick disease

◦Type A

◦Type B

◦Type C

◦Type D

◦Other specified type

◦Unspecified type

»Metachromatic leukodystrophy

»Other specified sphingolipidosis

»Unspecified sphingolipidosis

–Other specified type of lipid storage disorder

–Unspecified lipid storage disorder

•Disorders of lipoprotein metabolism and other lipidemias

–Lipidemia

»Hyperchylomicronemia

»Mixed hyperlipidemia

»Pure hypercholesterolemia

◦Familial

◦Unspecified

»Pure hyperglyceridemia

»Other hyperlipidemia

»Unspecified hyperlipidemia

–Lipodystrophy, NEC/NOS

–Lipomatosis, NEC/NOS

–Lipoprotein deficiency

–Other specified disorders of lipoprotein metabolism

»Lipoid dermatoarthritis

»Other specified type

–Unspecified disorder of lipoprotein metabolism

–Disorders of bile acid and cholesterol metabolism

»Barth syndrome

»Smith-Lemli-Opitz syndrome

»Other specified disorder

»Unspecified disorder

ICD-10-CM Code/Documentation

E75.00	GM2 gangliosidosis
E75.01	Sandhoff disease
E75.02	Tay-Sachs disease
E75.09	Other GM2 gangliosidosis
E75.10	Unspecified gangliosidosis
E75.11	Mucolipidosis IV
E75.19	Other gangliosidosis
E75.21	Fabry (-Anderson) disease
E75.22	Gaucher disease
E75.23	Krabbe disease
E75.240	Niemann-Pick disease type A
E75.241	Niemann-Pick disease type B
E75.242	Niemann-Pick disease type C
E75.243	Niemann-Pick disease type D
E75.248	Other Niemann-Pick disease
E75.249	Niemann-Pick disease unspecified
E75.25	Metachromatic leukodystrophy
E75.26	Sulfatase deficiency
E75.29	Other sphingolipidosis
E75.3	Sphingolipidosis, unspecified
E75.4	Neuronal ceroid lipofuscinosis
E75.5	Other lipid storage disorders
E75.6	Lipid storage disorder, unspecified

E78.00	Pure hypercholesterolemia, unspecified
E78.01	Familial hypercholesterolemia
E78.1	Pure hyperglyceridemia
E78.2	Mixed hyperlipidemia
E78.3	Hyperchylomicronemia
E78.41	Elevated lipoprotein (a)
E78.49	Other hyperlipidemia
E78.5	Hyperlipidemia, unspecified
E78.6	Lipoprotein deficiency
E78.70	Disorder of bile acid and cholesterol metabolism, unspecified
E78.71	Barth syndrome
E78.72	Smith-Lemli-Opitz syndrome
E78.79	Other disorders of bile acid and cholesterol metabolism
E78.81	Lipoid dermatoarthritis
E78.89	Other lipoprotein metabolism disorders
E78.9	Disorder of lipoprotein metabolism, unspecified

E77.0	Defects in post-translational modification of lysosomal enzymes
E77.1	Defects in glycoprotein degradation
E77.8	Other disorders of glycoprotein metabolism
E77.9	Disorder of glycoprotein metabolism, unspecified

E76.01	Hurler’s syndrome
E76.02	Hurler-Scheie syndrome
E76.03	Scheie’s syndrome
E76.1	Mucopolysaccharidosis, type II
E76.210	Morquio A mucopolysaccharidoses
E76.211	Morquio B mucopolysaccharidoses
E76.219	Morquio mucopolysaccharidoses, unspecified
E76.22	Sanfilippo mucopolysaccharidoses
E76.29	Other mucopolysaccharidoses
E76.3	Mucopolysaccharidosis, unspecified
E76.8	Other disorders of glucosaminoglycan metabolism
E76.9	Glucosaminoglycan metabolism disorder, unspecified

E88.1	Lipodystrophy, not elsewhere classified
E88.2	Lipomatosis, not elsewhere classified

Documentation and Coding Example

Twenty-seven-year-old female presents to Infusion Clinic for her bi-monthly intravenous VPRIV infusion. She was diagnosed with Gaucher’s Disease 6 months ago when she presented with a traumatic fracture, bone pain, and fatigue. Her Ashkenazi Jewish ancestry led to molecular genetic studies and the diagnosis of Gaucher’s was made. She had severe osteopenia on DEXA scan at that time, decreased serum glucocerebrosidase, mild anemia, thrombocytopenia, and pinguecula. She was started on oral Miglustat which she was not able to tolerate and was switched to infusions. Intravenous VPRIV has stabilized her disease with no progression of osteopenia on DEXA scan performed last week, stable pinguecula lesions in both eyes per ophthalmology, RBCs and platelets back in normal range. Temperature 97.8, HR 78, RR 12, BP 122/80. Wt. 122 lbs. Ht. 64 inches. Skin without bruising or discoloration. PERRL, neck supple without lymphadenopathy. Heart rate regular, peripheral pulses full. Breath sounds clear and equal bilaterally. Abdomen soft and non-distended. Liver palpated at 2 cm below RCM, spleen is palpated at LCM. IV started in right hand and she is pre-medicated with oral diphenhydramine and acetaminophen. VPRIV is infused over 2 hours with only mild nausea reported by patient. Angiocath removed intact, gentle pressure applied with no excess bleeding noted.

Diagnosis: Gaucher’s Disease

Plan: Patient will return in two weeks for repeat infusion. Standing lab orders are on file and she will have blood drawn prior to her infusion appointment.

Diagnosis Code(s)

E75.22

Gaucher disease

Coding Note(s)

Gaucher disease is a type of cerebral lipidoses, which is classified under sphingolipidoses. Sphingolipidoses are an inherited group of disorders of lipid metabolism that primarily affect the central nervous system. Each of the various types of sphingolipidoses is linked to a defect in specific lysosomal enzymes. The deficient enzyme in Gaucher disease is glucocerebrosidase which causes glucocerebrosides to accumulate in the red blood cells, liver, and spleen. Symptoms include hepatosplenomegaly, anemia, thrombocytopenia, bone pain, and a deformity of the femur referred to as an Erlenmeyer flask deformity. The pattern of inheritance for Gaucher disease is autosomal recessive and it affects primarily individuals of Ashkenazi Jewish descent.

Overweight and Obesity

Overweight is generally defined as excess body fat that can impair health. One measurement used to determine overweight is body mass index (BMI), which is a measurement of height and weight that is used to assess the total proportion of fat in the body. BMI is not, however, a direct measure of body fat. A BMI of 25-29.9 is considered overweight for most individuals.

Obesity and morbid obesity are large amounts of excess fat in the body often associated with profound health risks including diabetes, respiratory compromise, and cardiac disease. Obesity is a BMI of 30-39.9 or body weight at least 20% greater than normal for height. Morbid obesity is a BMI >40 or body weight 50-100% greater than normal for height.

Category E66 Overweight and obesity contains codes for overweight; obesity and morbid obesity due to excess calories; drug-induced obesity; morbid obesity with alveolar hypoventilation; other specified types of obesity; and unspecified obesity.

Coding and Documentation Requirements

Identify condition:

•Obesity

–Drug-induced

–Due to excess calories

»Morbid (severe)

»Other

–Morbid/severe with alveolar hypoventilation

–Other

–Overweight

ICD-10-CM Code/Documentation
E66.01	Morbid (severe) obesity due to excess calories
E66.09	Other obesity due to excess calories
E66.1	Drug induced obesity
E66.2	Morbid (severe) obesity with alveolar hypoventilation
E66.3	Overweight
E66.8	Other obesity
E66.9	Obesity, unspecified

Documentation and Coding Example

Thirty-three-year-old presents to pulmonology for pre-operative work-up prior to bariatric surgery. Temperature 97.6, HR 92, RR 16, BP 156/94, HT 62 inches, WT 282 lbs. BMI 51.6. Patient states she has been heavy all of her life, is recently engaged to be married, and they would like to have children. She hopes that bariatric surgery will allow her to lose weight and improve her health. She is chronically short of breath and was prescribed CPAP for sleep apnea a year ago but rarely uses it. On examination, this is a pleasant, well groomed, morbidly obese Black female. Breath sounds are clear in upper lobes and over bronchioles, muffled in middle and lower lobes. PFTs show decreased lung volume consistent with obesity. ABG drawn pH 7.3, PaCo2 52, HCO3 30, PaO2 75, O2 Sat 92%. Impression: Chronic hypercapnic respiratory failure secondary to morbid obesity. Patient is a good candidate for bariatric surgery and her respiratory condition should improve with weight loss. She is sent to cardiology to complete her pre-op testing.

Diagnosis Code(s)

E66.2	Morbid (severe) obesity with alveolar hypoventilation
Z68.43	Body mass index [BMI] 50.0-59.9, adult

Coding Note(s)

An Excludes1 note under E66.01 Morbid (severe) obesity due to excess calories indicates that E66.01 is not reported with E66.2 Morbid (severe) obesity with alveolar hypoventilation so only code E66.2 is reported for the obesity. The patient’s BMI is reported additionally.

Thyroid Gland Disorders

Disorders of the thyroid gland are the first codes listed within the endocrine chapter. There are eight categories. Some disorders, such as congenital iodine deficiency syndrome (that was previously classified with congenital hypothyroidism and reported with a single code), are identified specifically as to type: neurological type (E00.0), myxedematous type (E00.1), and mixed type (E00.2). Congenital hypothyroidism is also differentiated as with or without goiter. Some acquired kinds of hypothyroid conditions also have specific codes, such as E02 Subclinical iodine-deficiency hypothyroidism (which was previously reported under other specified acquired hypothyroidism).

Hypothyroidism, Acquired

Hypothyroidism is a condition in which the thyroid gland does not produce adequate levels of thyroid hormones (thyroxine and/or T3). The most common form of hypothyroidism is due to iodine deficiency. Code E89.0 Postprocedural hypothyroidism captures hypothyroidism due to surgical and irradiation procedures. Other hypothyroid conditions also have specific codes, including E02 Subclinical iodine-deficiency hypothyroidism and E03.3 Postinfectious hypothyroidism.

Coding and Documentation Requirements

Identify acquired hypothyroidism:

•Due to acquired atrophy of thyroid gland

•Due to medicaments/exogenous substances

•Iodine deficiency related

–Hypothyroidism

–Subclinical hypothyroidism

•Postinfectious

•Postprocedural

•Other specified type of hypothyroidism

•Unspecified type

ICD-10-CM Code/Documentation
E01.8	Other iodine deficiency related thyroid disorders and allied conditions
E02	Subclinical iodine-deficiency hypothyroidism
E03.2	Hypothyroidism due to medicaments and other exogenous substances
E03.3	Postinfectious hypothyroidism
E03.4	Atrophy of thyroid (acquired)
E03.8	Other specified hypothyroidism
E03.9	Hypothyroidism, unspecified
E89.0	Postprocedural hypothyroidism

Documentation and Coding Example

This 45-year-old female is 6 months status post radioactive iodine treatment for Graves’ disease. She comes in to the clinic today because she is concerned about symptoms that may be indicative of postablative hypothyroidism. She has classic symptoms of hypothyroidism which include cold intolerance, lethargy, and weight gain. Thyroid function studies are performed including T4, free T4, T3, and TSH. Comparison with previous thyroid function studies show a progressive decline in the amount of T4, free T4, and T3 to lower than normal levels. During the same period there has been a progressive increase in TSH. Impression: Findings are consistent with postablative hypothyroidism.

Plan: Will begin thyroid hormone replacement therapy.

Diagnosis Code(s)

E89.0

Postprocedural hypothyroidism

Coding Note(s)

Postprocedural complications and disorders that are commonly associated with a specific body system are classified in the body system chapter. These codes are usually found in the code block at the end of the chapter. Postprocedural hypothyroidism is classified together with other postprocedural endocrine and metabolic complications and disorders, such as postprocedural hypoinsulinemia, postprocedural hypoparathyroidism, postprocedural hypopituitarism, and postprocedural ovarian or testicular failure.

Hypothyroidism, Congenital

Congenital hypothyroidism is a condition that is present at birth. It occurs when the thyroid gland does not develop properly in utero or when it does not function properly. The thyroid gland may be entirely absent or much smaller than normal. It may also be in an abnormal location. In some cases, newborns have a normal-sized or enlarged thyroid gland, but the thyroid gland does not produce adequate amounts of thyroid hormones.

The code for congenital hypothyroidism without goiter (E03.1) is reported when the thyroid is absent (aplasia of thyroid), smaller than normal (atrophy), or when the thyroid does not produce enough thyroid hormones and there is no documentation of goiter or enlargement of the thyroid. Congenital iodine deficiency syndrome in a fetus or newborn is a condition where there is an inadequate level of iodine in the body which is needed to produce thyroid hormones. This results in insufficient hormone production and slowed metabolic processes that can lead to brain damage and developmental delays. This condition may manifest with neurological or myxedematous symptoms, or with a combination of both (mixed type). Neurological symptoms include deafness/hearing loss, dysarthria, muscle spasticity (primarily in the lower extremities) and cognitive deficits. Characteristics of myxedematous type include large head and edema of the genitals and extremities. Characteristics of mixed type can include large head and edema of genitals and extremities as well as cognitive deficits, muscle spasticity, and hearing loss.

Coding and Documentation Requirements

Identify congenital condition:

•Congenital hypothyroidism

–With goiter

–Without goiter

•Congenital iodine-deficiency syndrome

–Mixed type

–Myxedematous type

–Neurological type

–Unspecified

ICD-10-CM Code/Documentation
E00.0	Congenital iodine-deficiency syndrome, neurological type
E00.1	Congenital iodine-deficiency syndrome, myxedematous type
E00.2	Congenital iodine-deficiency syndrome, mixed type
E00.9	Congenital iodine-deficiency syndrome, unspecified
E03.0	Congenital hypothyroidism with diffuse goiter
E03.1	Congenital hypothyroidism without goiter

Documentation and Coding Example

Seven-day-old infant is referred to Pediatric Endocrine Clinic by PMD for abnormal Newborn Screening Test. This Hispanic male is the first child of a 33-year-old mother, 32-year-old father. He was delivered at 39.5 weeks via scheduled C-section for breech presentation. BW 3150 grams, length 51 cm, HC 35 cm. Parents report infant remained in the transition nursery for >12 hours due to sleepiness, poor tone, and problems maintaining body temperature but eventually joined mother and roomed in for the 4 days that she was hospitalized. BW on discharge was 2900 grams. Mother received assistance from a lactation consultant because infant was sleepy and feeding poorly. He was seen by pediatric nurse practitioner yesterday and parents reported infant still feeding poorly and weight was 2780 grams. During that appointment, clinic received a call from the lab indicating that the newborn screening test showed an abnormally low T4 level and TSH also out of range. Blood drawn for repeat TSH and Free T4 confirmed abnormal levels and infant is seen emergently today by pediatric endocrinologist. On exam this is a sleepy infant. Skin is dry and peeling. Head round, fontanels sunken, sutures override. Muscle tone is floppy. Generalized appearance of muscle wasting is present. PERRLA with normal red reflex. Mucous membranes moist and pink, no macroglossia. Palate intact. Weak suck. Nares patent. Ears normal in shape and location. TMs clear. Apical pulse regular. Breath sounds clear and equal. Abdomen soft and round with hypoactive bowel sounds. No evidence of hernia. Parents report green-yellow stool being passed 2-3 x day. Testis down, penis uncircumcised.

Impression: Congenital hypothyroidism, dehydration. Plan: Admit to Pediatric floor for hydration and initiate levothyroxine therapy. Possible technetium thyroid scan and radioactive iodine scan.

Diagnosis Code(s)

E03.1	Congenital hypothyroidism without goiter
P74.1	Dehydration of newborn
P92.9	Feeding problem of newborn, unspecified

Coding Note(s)

There are two codes for congenital hypothyroidism, one for with goiter and one for without goiter. There are also codes for congenital iodine deficiency syndrome. Congenital iodine deficiency syndrome may present in a fetus or newborn when there are inadequate levels of iodine to produce thyroid hormones. In this case, the documentation does not state that the newborn has congenital iodine deficiency syndrome nor is there any documentation of a goiter, so the code for congenital hypothyroidism without goiter is assigned.

The newborn also has feeding problems described only as “poor feeding”. There are more specific codes for feeding problems in newborns, such as slow feeding, underfeeding, and difficulty feeding at breast, but because the condition is documented only as poor feeding, the code for unspecified feeding problem must be assigned. The newborn is being admitted for hydration as well as treatment of congenital hypothyroidism so the dehydration is reported additionally. Note that it is a specific code for dehydration of newborn.

Simple and Unspecified Goiter/Nontoxic Nodular Goiter

Goiter refers to any abnormal enlargement of the thyroid gland. A simple goiter refers to diffuse enlargement of the thyroid gland that is not caused by inflammation or malignant disease. Thyroid function is usually normal with a simple goiter. A nontoxic goiter is an enlarged thyroid gland that may also be described more specifically as a uninodular or multinodular goiter. Nontoxic goiters do not affect thyroid function so thyroid function tests are normal.

Nontoxic goiters are reported with codes from three categories: E01 Iodine-deficiency related thyroid disorders and allied conditions, E03 Other hypothyroidism, and E04 Other nontoxic goiter. A simple goiter is classified as a nontoxic diffuse goiter and is included in category E04 Other nontoxic goiter along with codes for nontoxic uninodular and multinodular goiters.

Coding and Documentation Requirements

For nontoxic goiter, identify type:

•Diffuse

–Iodine deficiency related

–With congenital hypothyroidism

–Other nontoxic diffuse (simple)

•Multinodular

–Iodine deficiency related

–Other nontoxic multinodular

•Single thyroid nodule (uninodular)

•Other specified type nontoxic goiter

•Unspecified

–Iodine deficiency related (not described as diffuse or multinodular)

–Unspecified nontoxic goiter (nodular, NOS)

ICD-10-CM Code/Documentation
E04.0	Nontoxic diffuse goiter
E04.1	Nontoxic single thyroid nodule
E04.2	Nontoxic multinodular goiter
E04.8	Other specified nontoxic goiter
E04.9	Nontoxic goiter, unspecified
E01.0	Iodine-deficiency related diffuse (endemic) goiter
E01.1	Iodine-deficiency related multinodular (endemic) goiter
E01.2	Iodine-deficiency related (endemic) goiter, unspecified
E03.0	Congenital hypothyroidism with diffuse goiter

Documentation and Coding Example

Fifty-one-year-old Black female is seen for annual physical exam. She states she has been feeling well and has no new complaints. She continues to work full time as a pharmacist, her husband’s landscaping business is doing well and her daughters are both in high school, one active in drama and the other in sports. PMH includes dysfunctional uterine bleeding treated with endometrial ablation 3 years ago, hypertension x 20 years treated with Lopressor 50 mg BID and hypothyroid treated with Synthroid 50 mcg. QD. HT 66 in. WT 168 lbs. T 97.9, P 72, R 14, BP 138/88. On examination, this is a well-developed, well-nourished female who looks her stated age. Eyes clear, PERRLA. Nares patent. Oral mucosa moist and pink. Neck supple, there is some fullness noted in the right side of the thyroid gland, patient denies cough or hoarseness, dysphagia, or problems breathing. No lymphadenopathy appreciated. Cranial nerves grossly intact. Carotid arteries are without murmur, bruit, or rub. Apical pulse regular without murmur and peripheral pulses strong and equal. Breath sounds clear and equal bilaterally. Abdomen soft, bowel sounds present in all quadrants. Liver palpated at 2 cm below RCM, spleen is not palpated. Breast and gynecological exams are deferred as patient had a mammogram and saw her GYN 6 months ago and a note is available in the chart stating exam was WNL. There are scattered skin tags across her back, consistent with location of her bra. Discussed lab work which was done prior to visit. CBC, metabolic and cholesterol/lipid panel are WNL. Her TSH is 3.38 so she will continue on present dose of Synthroid. BP appears well controlled on Lopressor. Written RX for both medications are given to patient. Discussed the need for colonoscopy now that she is over age 50 and patient states she will make an appointment. Thyroid ultrasound can be done today in radiology and patient is sent over for that procedure and will return afterward for results.

Post procedure note: Per radiologist, thyroid ultrasound shows a 1.0 x 1.5 cystic mass on right side of the thyroid. The walls are irregular with fine debris noted within the lumen of the cyst. The thyroid vasculature appears normal and no blood vessels are within the cyst.

Impression: Hemorrhagic colloid cyst. Recommend repeat ultrasound in 2-3 months.

Patient was apprised of the findings from the ultrasound and agrees with the watch and follow plan with repeat US in 2 months.

Diagnosis Code(s)

Z00.01	Encounter for general adult medical examination with abnormal findings
E04.1	Nontoxic single thyroid nodule
I10	Essential hypertension
E03.9	Hypothyroidism, unspecified

Coding Note(s)

The reason for the encounter is an annual routine general medical examination so that is the first listed diagnosis. The patient has two chronic conditions, hypertension and hypothyroidism. These conditions were evaluated and affect the patient care so they are reported additionally. A new finding is the mass on the right side of the thyroid which is evaluated by ultrasound and determined to be cystic in nature. The thyroid cyst has been more specifically described as a colloid cyst.

There are two codes for reporting general adult medical examinations, one for without abnormal findings and another for with abnormal findings. In this case, the patient has chronic conditions as well as a new abnormal finding of a cystic mass of the thyroid, so the code for examination with abnormal findings is reported. Under Cyst, thyroid, the Alphabetic Index identifies E04.1 Nontoxic single thyroid nodule as the correct code, and colloid nodule (cystic) (thyroid) is listed as an inclusion term under E04.1 in the Tabular List. Codes for essential hypertension and unspecified hypothyroidism are also reported.

Thyrotoxicosis with or without Goiter

Thyrotoxicosis is a hypermetabolic clinical condition which occurs with elevated circulating levels of the thyroid hormones, thyroxine and/or T3, in blood serum. This condition may result from destruction of thyroid gland follicles and thyrocytes associated with thyroiditis or from the excessive intake of exogenous thyroid hormones. It may also occur due to functionally active neoplasms. Symptoms of thyrotoxicosis include sudden weight loss without decrease in caloric intake, tachycardia, increased appetite, nervousness, anxiety, restlessness, irritability, tremor, sweating, changes in menstrual cycle and bowel habits, fatigue, muscle weakness, sleep disturbances, and changes in skin and hair.

Thyrotoxicosis may occur with or without enlargement of the thyroid gland (toxic goiter) and may be complicated by thyrotoxic crisis or storm. Thyroid crisis or storm is a rare but severe complication of hyperthyroidism (elevated thyroid function). The condition is often precipitated by physical illness or stress. Symptoms can include elevated body temperature and heart rate, cardiac arrhythmias, vomiting, diarrhea, dehydration, and in extreme cases, even coma and death.

Toxic goiters are classified as diffuse or nodular. For nodular goiters there are two subcategories, one for a toxic single thyroid nodule, and another for toxic multinodular goiter. If the documentation does not specify single or multiple nodules, a code for thyrotoxicosis with toxic multinodular goiter is used. There is also a subcategory for thyrotoxicosis factitia, which is elevated thyroid function caused by the voluntary or involuntary ingestion of large amounts of exogenous thyroid hormone.

Coding and Documentation Requirements

For thyrotoxicosis, identify as with or without goiter:

•With goiter

–Diffuse (simple)

–Nodular

»Uninodular (single thyroid nodule)

»Multinodular

–Unspecified

•Without goiter

–Ectopic thyroid tissue

–Thyrotoxicosis factitia

–Other specified origin

–Unspecified

Identity presence or absence of thyrotoxic crisis or storm:

•With thyrotoxic crisis or storm

•Without thyrotoxic crisis or storm

ICD-10-CM Code/Documentation
E05.00	Thyrotoxicosis with diffuse goiter without thyrotoxic crisis or storm
E05.01	Thyrotoxicosis with diffuse goiter with thyrotoxic crisis or storm
E05.10	Thyrotoxicosis with toxic single thyroid nodule without thyrotoxic crisis or storm
E05.11	Thyrotoxicosis with toxic single thyroid nodule with thyrotoxic crisis or storm
E05.20	Thyrotoxicosis with toxic multinodular goiter without thyrotoxic crisis or storm
E05.21	Thyrotoxicosis with toxic multinodular goiter with thyrotoxic crisis or storm
E05.30	Thyrotoxicosis from ectopic thyroid tissue without thyrotoxic crisis or storm
E05.31	Thyrotoxicosis from ectopic thyroid tissue with thyrotoxic crisis or storm
E05.40	Thyrotoxicosis factitia without thyrotoxic crisis or storm
E05.41	Thyrotoxicosis factitia with thyrotoxic crisis or storm
E05.80	Other thyrotoxicosis without thyrotoxic crisis or storm
E05.81	Other thyrotoxicosis with thyrotoxic crisis or storm
E05.90	Thyrotoxicosis, unspecified without thyrotoxic crisis or storm
E05.91	Thyrotoxicosis, unspecified with thyrotoxic crisis or storm

Documentation and Coding Example

Preoperative Diagnosis: Enlarged thyroid

Postoperative Diagnosis: Hypersecretory Thyroid Malignancy with Thyrotoxicosis

History: This 26-year-old Caucasian female has been referred by her PMD for evaluation of a diffusely enlarged thyroid with symptoms of thyrotoxicosis. Over the past few months she has experienced weight loss, restlessness, and anxiety which she attributed to the stress of a new job. During her annual examination her PMD noted a diffusely enlarged thyroid. Thyroid function studies obtained at that time were elevated, consistent with hyperthyroidism. She presents today for ultrasound guided needle biopsy of her thyroid.

Operative Note: The skin over the thyroid gland was cleansed and a local anesthetic was administered. Using ultrasound guidance, the biopsy needle was introduced into the thyroid and a tissue sample was obtained. Three additional sites are biopsied using the same technique. The tissue samples are prepared and sent to the laboratory for pathological exam.

Pathological Findings: The findings are consistent with malignant neoplasm of the thyroid.

Plan: Refer to Oncology for additional work-up and treatment of her functionally active thyroid malignancy, hypersecretion of thyroid hormones, and resulting thyrotoxicosis.

Diagnosis Code(s)

C73	Malignant neoplasm of thyroid gland
E05.80	Other thyrotoxicosis without thyrotoxic crisis or storm

Coding Note(s)

The malignancy is sequenced first followed by a code from the endocrine chapter to capture the thyrotoxicosis. Even though the thyroid is enlarged, the enlargement is due to a malignant neoplasm, so the condition is not classified as a goiter. Following instructions in the chapter level note related to neoplasms that are functionally active, an additional code is assigned to capture the functional activity. The chapter level note identifies the subcategory for functional activity of the thyroid due to malignancy as E05.8-. In this case the patient is experiencing thyrotoxicosis but there is no documented thyrotoxic crisis or storm, so code E05.80 is assigned.

Thyroiditis/Other Disorders of the Thyroid

Thyroiditis is an inflammation of the thyroid gland, usually accompanied by pain and swelling. Thyroiditis is classified as acute, subacute, and chronic. Autoimmune thyroiditis is classified as a chronic form of thyroiditis. Thyroiditis may also be caused by certain drugs and medicinal agents. Acute thyroiditis is a rare condition as the thyroid gland is normally resistant to microorganisms that cause infection due to the level of iodine in the tissue, the high degree of vascularity, and a good lymphatic drainage system. The condition usually follows an upper respiratory infection and a pyriform sinus fistula is frequently present. Symptoms include fever, pain, swelling, dysphagia, and dysphonia. Thyroid hormones T3, T4, and TSH levels are usually normal. Subacute thyroiditis is usually precipitated by a viral upper respiratory infection and presents with fever, malaise, and pain/swelling in the anterior neck/thyroid gland. The condition causes thyrotoxicosis due to the sudden discharge of stored thyroid hormone. Other names for this disorder include: De Quervain’s thyroiditis, giant cell thyroiditis, granulomatous or subacute granulomatous thyroiditis. Chronic thyroiditis is usually caused by an autoimmune response. Hashimoto’s thyroiditis is the most common form of chronic thyroiditis. Other chronic types include chronic fibrous thyroiditis, ligneous thyroiditis, and Riedel thyroiditis.

Other disorders of the thyroid include hypersecretion of calcitonin and dyshormonogenetic goiter. A thyroid cyst is classified as a nontoxic single thyroid nodule and is reported with code E04.1. Sick euthyroid syndrome is no longer considered as sign or symptom and is coded within the endocrine chapter. Acquired atrophy of the thyroid is coded in category E03 Other hypothyroidism.

Coding and Documentation Requirements

Identify thyroiditis/other thyroid disorders:

•Thyroiditis

–Acute

–Autoimmune

–Chronic

»With transient thyrotoxicosis

»Other (includes fibrous, ligneous, Riedel)

–Drug-induced

–Subacute

–Unspecified

•Other thyroid disorder

–Hypersecretion of calcitonin

–Dyshormonogenetic goiter

–Other specified disorder (includes hemorrhage/infarction)

–Sick euthyroid syndrome

–Unspecified disorder

ICD-10-CM Code/Documentation
E06.0	Acute thyroiditis
E06.1	Subacute thyroiditis
E06.2	Chronic thyroiditis with transient thyrotoxicosis
E06.3	Autoimmune thyroiditis
E06.4	Drug-induced thyroiditis
E06.5	Other chronic thyroiditis
E06.9	Thyroiditis, unspecified
E07.0	Hypersecretion of calcitonin
E07.1	Dyshormonogenetic goiter
E07.81	Sick euthyroid syndrome
E07.89	Other specified disorders of thyroid
E07.9	Disorder of thyroid, unspecified

Documentation and Coding Example

This 47-year-old white male presents for follow-up on Riedel thyroiditis. Initial symptoms included a thyroid mass, hypocalcemia, and hypothyroidism. Thyroid uptake scan was performed with malignancy and Grave’s disease ruled out. Other systemic symptoms all pointed to chronic thyroiditis. A needle biopsy was performed and histopathological exam of thyroid tissue confirmed Riedel thyroiditis. There was no compression of the trachea and it was elected to begin treatment with corticosteroids. He has been treated for the past month with corticosteroids with some improvement of symptoms. On examination, the thyroid enlargement is stable. No complaints of difficulty swallowing or any symptoms indicating airway compression. Continue with corticosteroid therapy. Return in one month for re-evaluation, sooner if any worsening of symptoms.

Diagnosis Code(s)

E06.5

Other chronic thyroiditis

Coding Note(s)

Riedel thyroiditis is a rare type of chronic inflammatory thyroiditis in which dense fibrotic growth replaces the normal thyroid tissue and eventually extends beyond the thyroid capsule to invade surrounding neck structures. Cases of chronic fibrous types of thyroiditis are classified together as other chronic thyroiditis.

Vitamin, Mineral, and Other Nutritional Deficiencies

Vitamin and mineral deficiencies may result from inadequate intake—not getting enough of certain nutrients in the diet, or they may result from an inability of the body to break down and absorb, or utilize, the specific vitamin, mineral, or other nutrient properly. Nutritional deficiencies are classified into different code categories based on the specific vitamin, mineral, or other nutrient that is lacking, and sometimes the manifesting condition of that particular deficiency. Certain vitamin and mineral or other deficiencies, such as enzymes, that occur due to a metabolic dysfunction are not reported as a nutritional deficiency, but are reported as a metabolic disorder, sometimes by the particular known pathway or mechanism, and/or resulting condition.

Documentation must clearly differentiate between deficiencies caused by nutritional/dietary factors and those caused by metabolic disorders. For instance, a vitamin D deficiency from lack of dietary intake that results in rickets is reported under category E55 Vitamin D deficiency, with a code specific for rickets, E55.0 Rickets, active. Vitamin D deficiency resulting from an inherited defect in the receptors for vitamin D is reported with E83.32 Hereditary vitamin D-dependent rickets (type 1) (type 2). Rickets that results from an inherited resistance to vitamin D is reported with E83.31 Familial hypophosphatemia.

Dietary deficiencies of minerals and other nutrients are captured by codes in categories E50-E64. There is a category for other nutritional deficiencies (E63) that captures more generalized conditions such as essential fatty acid (EFA) deficiencies, imbalance of nutritional constituents due to food intake, as well as other specified types of nutritional deficiencies. Category E64 reports the sequelae of certain vitamin or other nutritional deficiencies. The documentation must clearly identify the mineral or other nutrient deficiency so that the most specific code can be assigned.

Coding and Documentation Requirements

Identify the dietary deficiency of the specific vitamin/mineral/nutrient element:

•Calcium

•Chromium

•Copper

•Iron

•Magnesium

•Manganese

•Molybdenum

•Selenium

•Vanadium

•Zinc

•Vitamin A

•Vitamin B

–Niacin

–Thiamin

–Riboflavin

–Pyridoxine

–Other B group (biotin, cyanocobalamin, folic acid, pantothenic acid)

–Vitamin C (ascorbic acid)

–Vitamin D

–Vitamin E

–Vitamin K

•Other vitamins

•Multiple nutrient elements

•Other specified nutrient elements

•Unspecified nutrient element

•Other nutritional deficiencies:

•Essential fatty acid

•Imbalance of constituents of food intake

•Other specified nutritional deficiencies

•Unspecified nutritional deficiency

Identify any sequelae of nutritional deficiencies:

•Protein-calorie malnutrition

•Vitamin A deficiency

•Vitamin C deficiency

•Rickets

•Other nutritional deficiencies

•Unspecified nutritional deficiency

ICD-10-CM Code/Documentation
E50.0	Vitamin A deficiency with conjunctival xerosis
E50.1	Vitamin A deficiency with Bitot’s spot and conjunctival xerosis
E50.2	Vitamin A deficiency with corneal xerosis
E50.3	Vitamin A deficiency with corneal ulceration and xerosis
E50.4	Vitamin A deficiency with keratomalacia
E50.5	Vitamin A deficiency with night blindness
E50.6	Vitamin A deficiency with xerophthalmic scars of cornea
E50.7	Other ocular manifestations of vitamin A deficiency
E51.11	Dry beriberi
E51.12	Wet beriberi
E51.2	Wernicke’s encephalopathy
E51.8	Other manifestations of thiamine deficiency
E51.9	Thiamine deficiency, unspecified
E52	Niacin deficiency [pellagra]
E53.0	Riboflavin deficiency
E53.1	Pyridoxine deficiency
E53.8	Deficiency of other specified B group vitamins
E53.9	Vitamin B deficiency, unspecified
E54	Ascorbic acid deficiency
E55.0	Rickets, active
E55.9	Vitamin D deficiency, unspecified
E56.0	Deficiency of vitamin E
E56.1	Deficiency of vitamin K
E56.8	Deficiency of other vitamins
E56.9	Vitamin deficiency, unspecified
E58	Dietary calcium deficiency
E59	Dietary selenium deficiency
E60	Dietary zinc deficiency
E61.0	Copper deficiency
E61.1	Iron deficiency
E61.2	Magnesium deficiency
E61.3	Manganese deficiency
E61.4	Chromium deficiency
E61.5	Molybdenum deficiency
E61.6	Vanadium deficiency
E61.7	Deficiency of multiple nutrient elements
E61.8	Deficiency of other specified nutrient elements
E61.9	Deficiency of nutrient element, unspecified
E63.0	Essential fatty acid [EFA] deficiency
E63.1	Imbalance of constituents of food intake
E63.8	Other specified nutritional deficiencies
E63.9	Nutritional deficiency, unspecified
E64.0	Sequelae of protein-calorie malnutrition
E64.1	Sequelae of vitamin A deficiency
E64.2	Sequelae of vitamin C deficiency
E64.3	Sequelae of rickets
E64.4	Sequelae of other nutritional deficiencies
E64.5	Sequelae of unspecific nutritional deficiency

Documentation and Coding Example

Twenty-four-year-old Caucasian female presents to PMD for routine physical and concerns about some unusual symptoms. Patient has spent the past 2 years doing post-graduate research in the Middle East on drought resistant crops. She was well until 2 months ago when her supply of vitamin and mineral supplements ran out. She has noticed a marked decrease in energy level, loss of body hair, and intermittent diarrhea. Additionally, she has a fine red rash that she states started on her face and has now spread to her hands and feet. She states she eats a strict vegan diet including unleavened bread and is not taking any supplemental products. Temperature 97.6, HR 60, RR 12, BP 110/72, Wt. 112, Ht. 65 inches. On examination, this is a thin, but healthy appearing young woman. She is deeply tanned and states she is outdoors much of the time and does use sunscreen on her face, but rarely on the rest of her body. The hair on her head is very thin and brittle in texture. Body hair is quite sparse. A fine red rash is noted on cheeks and forehead, hands and feet. PERRL, neck supple without lymphadenopathy. Thyroid gland is smooth and normal size. Mucous membranes are pale pink, moist. Cranial nerves grossly intact. HR regular without bruit, rub, murmur. Peripheral pulses full, reflexes normal. Breath sounds clear, equal bilaterally. Breast exam is benign. Abdomen soft with active BS. Liver palpated at RCM, spleen at LCM. Pelvic exam is unremarkable, a routine pap test is performed. Patient is monogamous and at low risk for STD. A quick internet search was done while patient was getting dressed and it is possible her symptoms are due to zinc deficiency. Discussed findings with patient and lab tests ordered including CBC, CRP, ESR, comprehensive metabolic panel, Zinc, D3, TSH, cholesterol and lipid panel, UA, stool for O & P. Patient is advised to start taking a vitamin-mineral supplement and referred to a nutritionist to evaluate her diet. RTC in 1 week for test results.

Follow-Up Visit: Labs were significant for low serum zinc levels. Patient is prescribed zinc supplements in addition to the multi-vitamin/mineral she is taking. She will see the nutritionist in 2 days and patient is given copies of all lab reports to share with her. Diarrhea has resolved and rash is fading. Overall, she is feeling more energetic. She is advised to RTC in 3 months and we will repeat labs at that time. She should return sooner if she has any concerns.

Diagnosis: Dietary zinc deficiency

Diagnosis Code(s)

E60	Dietary zinc deficiency

Summary

Endocrine, metabolic, and nutritional diseases require clear and precise documentation to capture the most specific code. In order to capture the most specific code for diabetes mellitus, the chapter guidelines must be studied and the Includes and Excludes notes for each diabetes mellitus category reviewed prior to code assignment. Diabetic codes with ophthalmic complication of diabetic retinopathy require specification of the type of retinopathy with or without macular edema and laterality. Obesity will require specific documentation as to cause. Cushing syndrome is reported based on its related causative inducement or type. Dysfunctions of lipid metabolism are grouped based on the type of lipid and the specific metabolic disorder.

Resources

Documentation checklists are available in Appendix A for the following condition(s):

•Diabetes Mellitus

Chapter 4 Quiz

1.What type of diabetes mellitus would NOT be classified in category E13 Other specified diabetes mellitus?

a.Diabetes mellitus due to pancreatectomy

b.Insulin resistant diabetes

c.Diabetes due to genetic defects in insulin action

d.Secondary diabetes not elsewhere classified

2.What diagnosis documented below would necessitate the assignment of code Z79.4 for long term (current) use of insulin?

a.Use of insulin for temporary control of blood glucose in a patient with Type 2 diabetes

b.Type 1 diabetes treated with an insulin pump

c.Postpancreatectomy diabetes mellitus treated with daily insulin injections

d.All of the above

3.Which condition described below would NOT be reported with a code from category E08 Diabetes mellitus due to underlying condition?

a.Diabetes mellitus due to Cushing’s syndrome

b.Diabetes mellitus due to hyperthyroidism

c.Diabetes mellitus juvenile type

d.Diabetes mellitus due to liver disease

4.What is the principal or first listed code for secondary diabetes due to pancreatectomy?

a.A code from category E13 Other specified diabetes mellitus

b.A code from category E08 Diabetes mellitus due to underlying condition

c.Z90.41 Acquired absence of pancreas

d.E89.1 Postprocedural hypoinsulinemia

5.What documentation must be present to assign the proper code(s) for diabetes with ophthalmic complication of diabetic retinopathy?

a.Type of diabetes and specific type of diabetic retinopathy

b.Whether or not macular edema is present, or type of retinal detachment

c.Laterality of affected eye(s)

d.All of the above

6.What information must be clearly documented to allow assignment of the most specific code for a vitamin or mineral deficiency?

a.Signs and symptoms that support a diagnosis of a vitamin or mineral deficiency

b.Any suspected conditions that might cause malabsorption of the specific vitamin or mineral

c.Laboratory results showing a lower than normal level of the vitamin or mineral in the blood

d.The underlying cause as due to a dietary/nutritional deficiency or a metabolic disorder

7.What information is captured with codes in category E24 Cushing syndrome?

a.Type and cause of Cushing’s syndrome

b.The duration of the condition

c.The drug or drugs that caused the condition

d.All of the above

8.What information is NOT needed to assign the most specific code for obesity?

a.Cause

b.Severity

c.Whether or not it is complicated by alveolar hypoventilation

d.Body mass index

9.Pure hypercholesterolemia, NOS is classified as what type of disorder?

a.Nutritional

b.Metabolic

c.Endocrine

d.Other and unspecified condition

10.The external cause of an overdose of insulin documented as due to insulin pump malfunction is always coded as:

a.An adverse effect

b.Poisoning, accidental

c.Poisoning, undetermined

d.No external cause code is assigned

Chapter 4 Answers and Rationales

1.What type of diabetes mellitus would NOT be classified in category E13 Other specified diabetes mellitus?

b.Insulin resistant diabetes

Rationale: Insulin resistant diabetes is another way of describing Type 2 diabetes mellitus which is reported with a code from category E11. The other three types—postpancreatectomy diabetes, diabetes due to genetic defects in insulin action, and secondary diabetes not elsewhere classified are all reported with codes from category E13.

2.What diagnosis documented below would necessitate the assignment of code Z79.4 for long term (current) use of insulin?

c.Postpancreatectomy diabetes mellitus treated with daily insulin injections

Rationale: Since the patient requires daily insulin injections, code Z79.4 is assigned in addition to the appropriate code(s) for other specified diabetes, where postpancreatectomy diabetes mellitus is reported. An additional code is not reported for insulin use in Type 1 diabetes mellitus. An additional code is not reported for temporary use of insulin to bring blood glucose under control in patients with Type 2 diabetes mellitus.

3.Which condition described below would NOT be reported with a code from category E08 Diabetes mellitus due to underlying condition?

c.Diabetes mellitus juvenile type

Rationale: Diabetes mellitus juvenile type is reported with a code for Type 1 diabetes.

4.What is the principal or first listed code for secondary diabetes due to pancreatectomy?

d.E89.1 Postprocedural hypoinsulinemia

Rationale: According to the coding guidelines, codes are assigned as follows:

– Principal/First listed diagnosis – Assign code E89.1 Postprocedural hypoinsulinemia

– Assign a code from category E13 Other specified diabetes mellitus

– Assign an additional code from subcategory Z90.41-Acquired absence of pancreas

5.What documentation must be present to assign the proper code(s) for diabetes with ophthalmic complication of diabetic retinopathy?

d.All of the above

Rationale: The type of diabetes must be known to choose the correct diabetes category. The particular type of diabetic retinopathy must be documented, such as specified type of proliferative or nonproliferative diabetic retinopathy. Whether or not macular edema is present, or the type of retinal detachment present, is also identified in the code selection, as is the laterality.

6.What information must be clearly documented to allow assignment of the most specific code for a vitamin or mineral deficiency?

d.The underlying cause as due to a dietary/nutritional deficiency or a metabolic disorder

Rationale: Vitamin and mineral deficiencies must be clearly identified as due to a dietary deficiency or due to a metabolic disorder. Dietary vitamin and mineral deficiencies are reported with codes in categories E50-E64. Metabolic disorders resulting in vitamin and mineral deficiencies are reported with codes in categories E70-E88.

7.What information is captured with codes in category E24 Cushing syndrome?

a.Type and cause of Cushing’s syndrome

Rationale: Codes in category E24 identify the specific type and cause of Cushing’s syndrome. The duration of the condition is not captured by the code nor is the specific drug or drugs that caused the condition when it is documented as drug-induced.

8.What information is NOT needed to assign the most specific code for obesity?

d.Body mass index

Rationale: The cause (drug-induced, due to excess calories), severity (morbid or other), and whether or not morbid obesity is complicated by alveolar hypoventilation are required to assign the most specific code. The body mass is not required, but should be reported with an additional code from category Z68 when documented.

9.Pure hypercholesterolemia, NOS is classified as what type of disorder?

b.Metabolic

Rationale: Pure hypercholesterolemia is reported with code E78.0 which is listed in code block E70-E88 Metabolic disorders. The code category E78 identifies the condition more specifically as a disorder of lipoprotein metabolism.

10.The external cause of an overdose of insulin documented as due to insulin pump malfunction is always coded as:

b.Poisoning, accidental

Rationale: According to coding guidelines, the external cause of overdose due to insulin pump malfunction is reported with code T38.3x1- Poisoning by insulin and oral hypoglycemic [antidiabetic] drugs, accidental (unintentional).