Other congenital malformations (Q80-Q89)
ICD-10 codes Q80–Q89 categorize a wide range of congenital malformations affecting skin, integument, breast, phakomatoses, malformation syndromes, and other body systems. These codes are used to precisely document birth defects and hereditary syndromes involving structural and functional abnormalities present from birth.
This range includes codes for specific skin disorders like congenital ichthyosis (Q80) with types such as Ichthyosis vulgaris and Lamellar ichthyosis, and epidermolysis bullosa types (Q81), which cause skin fragility. It also covers hereditary lymphedema (Q82.0), xeroderma pigmentosum (Q82.1) characterized by UV sensitivity, and ectodermal dysplasias (Q82.4) involving abnormalities in hair, nails, teeth, and sweat glands. Congenital breast malformations appear under Q83, while Q84 addresses various integumentary system defects including alopecia and nail abnormalities. The phakomatoses category (Q85) includes neurofibromatosis types 1 and 2 and tuberous sclerosis, known for benign tumors affecting skin and nervous tissue. The syndromes subdivision (Q86-Q87) documents malformation syndromes caused by exogenous factors and genetic syndromes like Prader-Willi syndrome (Q87.11) and Marfan syndrome (Q87.4), including manifestations in cardiovascular, skeletal, and ocular systems. Finally, Q89 captures miscellaneous congenital malformations such as asplenia and situs inversus.
These codes assist medical professionals and coders in identifying and recording a spectrum of complex congenital conditions, connecting common terms like “fetal alcohol syndrome,” “accessory nipple,” and “congenital alopecia” to specific ICD-10 codes ensuring accurate diagnosis and data tracking.
- Congenital ichthyosis (Q80)
- Epidermolysis bullosa (Q81)
- Other congenital malformations of skin (Q82)
- Congenital malformations of breast (Q83)
- Other congenital malformations of integument (Q84)
- Phakomatoses, not elsewhere classified (Q85)
- Congenital malformation syndromes due to known exogenous cau
- Other specified congenital malformation syndromes affecting
- Other congenital malformations, not elsewhere classified (Q8
Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
Other congenital malformations (Q80-Q89)
Q82 Other congenital malformations of skin
- Q82.0 Hereditary lymphedema
- Q82.1 Xeroderma pigmentosum
- Q82.2 Congenital cutaneous mastocytosis
- Q82.3 Incontinentia pigmenti
- Q82.4 Ectodermal dysplasia (anhidrotic)
- Q82.5 Congenital non-neoplastic nevus
- Q82.6 Congenital sacral dimple
- Q82.8 Other specified congenital malformations of skin
- Q82.9 Congenital malformation of skin, unspecified
Other congenital malformations of skin (Q82)
Q84 Other congenital malformations of integument
- Q84.0 Congenital alopecia
- Q84.1 Congenital morphological disturbances of hair, not elsewhere classified
- Q84.2 Other congenital malformations of hair
- Q84.3 Anonychia
- Q84.4 Congenital leukonychia
- Q84.5 Enlarged and hypertrophic nails
- Q84.6 Other congenital malformations of nails
- Q84.8 Other specified congenital malformations of integument
- Q84.9 Congenital malformation of integument, unspecified
Other congenital malformations of integument (Q84)
Q85 Phakomatoses, not elsewhere classified
Q85.0 Neurofibromatosis (nonmalignant)
- Q85.00 Neurofibromatosis, unspecified
- Q85.01 Neurofibromatosis, type 1
- Q85.02 Neurofibromatosis, type 2
- Q85.03 Schwannomatosis
- Q85.09 Other neurofibromatosis
- Q85.1 Tuberous sclerosis
Q85.8 Other phakomatoses, not elsewhere classified
- Q85.81 PTEN hamartoma tumor syndrome
- Q85.82 Other Cowden syndrome
- Q85.83 Von Hippel-Lindau syndrome
- Q85.89 Other phakomatoses, not elsewhere classified
- Q85.9 Phakomatosis, unspecified
Phakomatoses, not elsewhere classified (Q85)
Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
- Q86.0 Fetal alcohol syndrome (dysmorphic)
- Q86.1 Fetal hydantoin syndrome
- Q86.2 Dysmorphism due to warfarin
- Q86.8 Other congenital malformation syndromes due to known exogenous causes
Congenital malformation syndromes due to known exogenous causes, not elsewhere classified (Q86)
Q87 Other specified congenital malformation syndromes affecting multiple systems
- Q87.0 Congenital malformation syndromes predominantly affecting facial appearance
Q87.1 Congenital malformation syndromes predominantly associated with short stature
- 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
Q87.4 Marfan syndrome
- Q87.40 Marfan syndrome, unspecified
Q87.41 Marfan syndrome with cardiovascular manifestations
- 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
Q87.8 Other specified congenital malformation syndromes, not elsewhere classified
- 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 NEW CODE
- Q87.89 Other specified congenital malformation syndromes, not elsewhere classified
Other specified congenital malformation syndromes affecting multiple systems (Q87)
Q89 Other congenital malformations, not elsewhere classified
Q89.0 Congenital absence and malformations of spleen
- Q89.01 Asplenia (congenital)
- Q89.09 Congenital malformations of spleen
- Q89.1 Congenital malformations of adrenal gland
- Q89.2 Congenital malformations of other endocrine glands
- Q89.3 Situs inversus
- Q89.4 Conjoined twins
- Q89.7 Multiple congenital malformations, not elsewhere classified
- Q89.8 Other specified congenital malformations
- Q89.9 Congenital malformation, unspecified
Other congenital malformations, not elsewhere classified (Q89)