ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q87 includes defined congenital malformation syndromes that affect multiple organ systems and are not elsewhere classified, such as certain multisystem developmental disorders and named syndromes with diverse anatomic involvement. These ICD-10-CM diagnosis codes are used to document clinical presentations involving multiple congenital anomalies, capturing the extent of organ system involvement and the complexity of the congenital condition. Accurate coding within the ICD-10-CM Q87 group supports proper claim adjudication and complete clinical documentation for appropriate reimbursement.

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ICD-10-CM Q87: Other Specified Congenital Malformation Syndromes Affecting Multiple Systems Overview

This group represents congenital malformation syndromes that involve multiple organ systems and do not fit into more specific congenital anomaly categories. These conditions typically affect structural development across systems such as cardiovascular, musculoskeletal, craniofacial, and neurologic structures. Accurate coding matters because it supports complete clinical documentation and appropriate reimbursement for the complexity of multisystem care.

When to Use

Use a code from this group when a patient has a specified congenital syndrome with documented malformations affecting two or more organ systems and no more specific ICD-10-CM code applies. Select the code that most closely matches the documented syndrome and include additional codes for specific organ anomalies when required for detail.

ICD-10-CM Codes

20 rows

ICD-10-CM Code	Description
Q87.0	Congenital malformation syndromes predominantly affecting facial appearance
Q87.11	Prader-Willi syndrome
Q87.19	Other congenital malformation syndromes predominantly associated with short stature
Q87.2	Congenital malformation syndromes predominantly involving limbs
Q87.3	Congenital malformation syndromes involving early overgrowth
Q87.40	Marfan syndrome, unspecified
Q87.410	Marfan syndrome with aortic dilation
Q87.418	Marfan syndrome with other cardiovascular manifestations
Q87.42	Marfan syndrome with ocular manifestations
Q87.43	Marfan syndrome with skeletal manifestation
Q87.5	Other congenital malformation syndromes with other skeletal changes
Q87.81	Alport syndrome
Q87.82	Arterial tortuosity syndrome
Q87.83	Bardet-Biedl syndrome
Q87.84	Laurence-Moon syndrome
Q87.85	MED13L syndrome
Q87.86	Kleefstra syndrome
Q87.87	Hao-Fountain Syndrome
Q87.88	CTNNB1 syndrome
Q87.89	Other specified congenital malformation syndromes, not elsewhere classified

DRG Codes

7 rows

DRG Code	Description
052	Spinal Disorders and Injuries with CC/MCC
053	Spinal Disorders and Injuries without CC/MCC
306	Cardiac Congenital and Valvular Disorders with MCC
307	Cardiac Congenital and Valvular Disorders without MCC
564	Other Musculoskeletal System and Connective Tissue Diagnoses with MCC
565	Other Musculoskeletal System and Connective Tissue Diagnoses with CC
566	Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC

Typical Clinical Scenarios

A newborn is evaluated in the neonatal unit for multiple dysmorphic features including joint contractures, distinct facial anomalies, and congenital limb malformations noted on exam and prenatal ultrasound. The infant is admitted for multidisciplinary assessment and genetic testing because the pattern suggests a multisystem congenital malformation syndrome rather than an isolated defect, so a diagnosis from this group is assigned to capture the syndromic presentation. Typical codes: Q87.0, Q87.3
A school-age child with known chromosomal and congenital anomalies presents for routine follow-up of growth, developmental delay, and progressive scoliosis with documented vertebral and rib malformations; management involves orthopedics, rehabilitation, and genetic counseling. Because the child’s problems reflect an ongoing, specified congenital malformation syndrome affecting multiple organ systems, a code from this group is used to document the chronic multisystem condition as the primary diagnosis for continuity of care. Typical codes: Q87.2, Q87.43
An adolescent with a previously diagnosed connective tissue–related congenital syndrome is seen in the emergency department for acute respiratory distress due to previously unrecognized tracheomalacia and severe chest wall deformity complicating airway management. The acute presentation is treated emergently, but the underlying multisystem congenital malformation syndrome is coded to reflect the comorbid structural abnormalities that contributed to the acute complication. Typical codes: Q87.84, Q87.89

Related Codes

Q80-CONGENITAL-ICHTHYOSIS

ICD-10-CM Code For Congenital Ichthyosis: Group Overview

Q81-EPIDERMOLYSIS-BULLOSA

ICD-10-CM Code For Epidermolysis Bullosa: Group Overview

Q82-OTHER-CONGENITAL-MALFORMATIONS-OF-SKIN

ICD-10-CM Code For Other Congenital Malformations Of Skin: Group Overview

Q84-OTHER-CONGENITAL-MALFORMATIONS-OF-INTEGUMENT

ICD-10-CM Code For Other Congenital Malformations Of Integument: Group Overview

Q85-PHAKOMATOSES-NOT-ELSEWHERE-CLASSIFIED

ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

Q86-CONGENITAL-MALFORMATION-SYNDROMES

ICD-10-CM Code For Congenital Malformation Syndromes Due To Known Exogenous Causes: Group Overview

Q89-OTHER-CONGENITAL-MALFORMATIONS

ICD-10-CM Code For Other Congenital Malformations Not Elsewhere Classified: Group Overview

Frequently Asked Questions

References

1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.

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When to Use

ICD-10-CM Codes

20 rows

ICD-10-CM Code	Description
Q87.0	Congenital malformation syndromes predominantly affecting facial appearance
Q87.11	Prader-Willi syndrome
Q87.19	Other congenital malformation syndromes predominantly associated with short stature
Q87.2	Congenital malformation syndromes predominantly involving limbs
Q87.3	Congenital malformation syndromes involving early overgrowth
Q87.40	Marfan syndrome, unspecified
Q87.410	Marfan syndrome with aortic dilation
Q87.418	Marfan syndrome with other cardiovascular manifestations
Q87.42	Marfan syndrome with ocular manifestations
Q87.43	Marfan syndrome with skeletal manifestation
Q87.5	Other congenital malformation syndromes with other skeletal changes
Q87.81	Alport syndrome
Q87.82	Arterial tortuosity syndrome
Q87.83	Bardet-Biedl syndrome
Q87.84	Laurence-Moon syndrome
Q87.85	MED13L syndrome
Q87.86	Kleefstra syndrome
Q87.87	Hao-Fountain Syndrome
Q87.88	CTNNB1 syndrome
Q87.89	Other specified congenital malformation syndromes, not elsewhere classified

DRG Codes

7 rows

DRG Code	Description
052	Spinal Disorders and Injuries with CC/MCC
053	Spinal Disorders and Injuries without CC/MCC
306	Cardiac Congenital and Valvular Disorders with MCC
307	Cardiac Congenital and Valvular Disorders without MCC
564	Other Musculoskeletal System and Connective Tissue Diagnoses with MCC
565	Other Musculoskeletal System and Connective Tissue Diagnoses with CC
566	Other Musculoskeletal System and Connective Tissue Diagnoses without CC/MCC

Typical Clinical Scenarios

A newborn is evaluated in the neonatal unit for multiple dysmorphic features including joint contractures, distinct facial anomalies, and congenital limb malformations noted on exam and prenatal ultrasound. The infant is admitted for multidisciplinary assessment and genetic testing because the pattern suggests a multisystem congenital malformation syndrome rather than an isolated defect, so a diagnosis from this group is assigned to capture the syndromic presentation. Typical codes: Q87.0, Q87.3
A school-age child with known chromosomal and congenital anomalies presents for routine follow-up of growth, developmental delay, and progressive scoliosis with documented vertebral and rib malformations; management involves orthopedics, rehabilitation, and genetic counseling. Because the child’s problems reflect an ongoing, specified congenital malformation syndrome affecting multiple organ systems, a code from this group is used to document the chronic multisystem condition as the primary diagnosis for continuity of care. Typical codes: Q87.2, Q87.43
An adolescent with a previously diagnosed connective tissue–related congenital syndrome is seen in the emergency department for acute respiratory distress due to previously unrecognized tracheomalacia and severe chest wall deformity complicating airway management. The acute presentation is treated emergently, but the underlying multisystem congenital malformation syndrome is coded to reflect the comorbid structural abnormalities that contributed to the acute complication. Typical codes: Q87.84, Q87.89

Related Codes

Q80-CONGENITAL-ICHTHYOSIS

ICD-10-CM Code For Congenital Ichthyosis: Group Overview

Q81-EPIDERMOLYSIS-BULLOSA

ICD-10-CM Code For Epidermolysis Bullosa: Group Overview

Q82-OTHER-CONGENITAL-MALFORMATIONS-OF-SKIN

ICD-10-CM Code For Other Congenital Malformations Of Skin: Group Overview

Q84-OTHER-CONGENITAL-MALFORMATIONS-OF-INTEGUMENT

ICD-10-CM Code For Other Congenital Malformations Of Integument: Group Overview

Q85-PHAKOMATOSES-NOT-ELSEWHERE-CLASSIFIED

ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

Q86-CONGENITAL-MALFORMATION-SYNDROMES

ICD-10-CM Code For Congenital Malformation Syndromes Due To Known Exogenous Causes: Group Overview

Q89-OTHER-CONGENITAL-MALFORMATIONS

ICD-10-CM Code For Other Congenital Malformations Not Elsewhere Classified: Group Overview

Frequently Asked Questions

References

1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.

Published by

Trek Health powers OpenPayer with a leading commercial payer intelligence platform, seamlessly converting fragmented payer data into actionable insights for provider organizations.

ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview

Sign up for ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview policy alerts

ICD-10-CM Q87: Other Specified Congenital Malformation Syndromes Affecting Multiple Systems Overview

ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview

Sign up for ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview policy alerts

ICD-10-CM Q87: Other Specified Congenital Malformation Syndromes Affecting Multiple Systems Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which ICD-10-CM codes are included in the Q87 group for multisystem congenital malformation syndromes?

How does accurate coding within the `Q87` group affect reimbursement and documentation?

What types of organ systems are typically affected by conditions in the Q87 group?

Which Q87 codes specifically relate to Marfan syndrome and its manifestations?

What related DRGs may be relevant when coding Q87 diagnoses for inpatient cases?

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which ICD-10-CM codes are included in the Q87 group for multisystem congenital malformation syndromes?

How does accurate coding within the `Q87` group affect reimbursement and documentation?

What types of organ systems are typically affected by conditions in the Q87 group?

Which Q87 codes specifically relate to Marfan syndrome and its manifestations?

What related DRGs may be relevant when coding Q87 diagnoses for inpatient cases?

References