ICD-10-CM Book 2024 Edition

Chapters  /  Chapter 17  /  Q80-Q89

Other congenital malformations (Q80-Q89)

Q80 Congenital ichthyosis
Excludes1 Refsum's disease (G60.1)
Q80.0 Ichthyosis vulgaris
Q80.1 X-linked ichthyosis
Q80.2 Lamellar ichthyosis
Collodion baby
Q80.3 Congenital bullous ichthyosiform erythroderma
Q80.4 Harlequin fetus
Q80.8 Other congenital ichthyosis
Q80.9 Congenital ichthyosis, unspecified
Q81 Epidermolysis bullosa
Q81.0 Epidermolysis bullosa simplex
Excludes1 Cockayne's syndrome (Q87.19)
Q81.1 Epidermolysis bullosa letalis
Herlitz' syndrome
Q81.2 Epidermolysis bullosa dystrophica
Q81.8 Other epidermolysis bullosa
Q81.9 Epidermolysis bullosa, unspecified
Q82 Other congenital malformations of skin
Excludes1 acrodermatitis enteropathica (E83.2)
congenital erythropoietic porphyria (E80.0)
pilonidal cyst or sinus (L05.-)
Sturge-Weber (-Dimitri) syndrome (Q85.89)
Q82.0 Hereditary lymphedema
Q82.1 Xeroderma pigmentosum
Q82.2 Congenital cutaneous mastocytosis
Congenital diffuse cutaneous mastocytosis
Congenital maculopapular cutaneous mastocytosis
Congenital urticaria pigmentosa
Excludes1 cutaneous mastocytosis NOS (D47.01)
diffuse cutaneous mastocytosis (with onset after newborn period) (D47.01)
malignant mastocytosis (C96.2-)
systemic mastocytosis (D47.02)
urticaria pigmentosa (non-congenital) (with onset after newborn period) (D47.01)
Q82.3 Incontinentia pigmenti
Q82.4 Ectodermal dysplasia (anhidrotic)
Excludes1 Ellis-van Creveld syndrome (Q77.6)
Q82.5 Congenital non-neoplastic nevus
Birthmark NOS
Flammeus Nevus
Portwine Nevus
Sanguineous Nevus
Strawberry Nevus
Vascular Nevus NOS
Verrucous Nevus
Excludes2 Café au lait spots (L81.3)
lentigo (L81.4)
nevus NOS (D22.-)
araneus nevus (I78.1)
melanocytic nevus (D22.-)
pigmented nevus (D22.-)
spider nevus (I78.1)
stellar nevus (I78.1)
Q82.6 Congenital sacral dimple
Parasacral dimple
Excludes2 pilonidal cyst with abscess (L05.01)
pilonidal cyst without abscess (L05.91)
Q82.8 Other specified congenital malformations of skin
Abnormal palmar creases
Accessory skin tags
Benign familial pemphigus [Hailey-Hailey]
Congenital poikiloderma
Cutis laxa (hyperelastica)
Dermatoglyphic anomalies
Inherited keratosis palmaris et plantaris
Keratosis follicularis [Darier-White]
Excludes1 Ehlers-Danlos syndromes (Q79.6-)
Q82.9 Congenital malformation of skin, unspecified
Q83 Congenital malformations of breast
Excludes2 absence of pectoral muscle (Q79.8)
hypoplasia of breast (N64.82)
micromastia (N64.82)
Q83.0 Congenital absence of breast with absent nipple
Q83.1 Accessory breast
Supernumerary breast
Q83.2 Absent nipple
Q83.3 Accessory nipple
Supernumerary nipple
Q83.8 Other congenital malformations of breast
Q83.9 Congenital malformation of breast, unspecified
Q84 Other congenital malformations of integument
Q84.0 Congenital alopecia
Congenital atrichosis
Q84.1 Congenital morphological disturbances of hair, not elsewhere classified
Beaded hair
Monilethrix
Pili annulati
Excludes1 Menkes' kinky hair syndrome (E83.09)
Q84.2 Other congenital malformations of hair
Congenital hypertrichosis
Congenital malformation of hair NOS
Persistent lanugo
Q84.3 Anonychia
Excludes1 nail patella syndrome (Q87.2)
Q84.4 Congenital leukonychia
Q84.5 Enlarged and hypertrophic nails
Congenital onychauxis
Pachyonychia
Q84.6 Other congenital malformations of nails
Congenital clubnail
Congenital koilonychia
Congenital malformation of nail NOS
Q84.8 Other specified congenital malformations of integument
Aplasia cutis congenita
Q84.9 Congenital malformation of integument, unspecified
Congenital anomaly of integument NOS
Congenital deformity of integument NOS
Q85 Phakomatoses, not elsewhere classified
Excludes1 ataxia telangiectasia [Louis-Bar] (G11.3)
familial dysautonomia [Riley-Day] (G90.1)
Q85.0 Neurofibromatosis (nonmalignant)
Q85.00 Neurofibromatosis, unspecified
Q85.01 Neurofibromatosis, type 1
Von Recklinghausen disease
Q85.02 Neurofibromatosis, type 2
Acoustic neurofibromatosis
Q85.03 Schwannomatosis
Q85.09 Other neurofibromatosis
Q85.1 Tuberous sclerosis
Bourneville's disease
Epiloia
Q85.8 Other phakomatoses, not elsewhere classified
Excludes1 Meckel-Gruber syndrome (Q61.9)
Q85.81 PTEN hamartoma tumor syndrome
PHTS
PTEN related Cowden syndrome
CodeAlso , if applicable, genetic susceptibility to malignant neoplasm (Z15.0-)
Q85.82 Other Cowden syndrome
Q85.83 Von Hippel-Lindau syndrome
CodeAlso manifestations
Q85.89 Other phakomatoses, not elsewhere classified
Peutz-Jeghers syndrome
Sturge-Weber(-Dimitri) syndrome
Q85.9 Phakomatosis, unspecified
Hamartosis NOS
Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
Excludes2 iodine-deficiency-related hypothyroidism (E00-E02)
nonteratogenic effects of substances transmitted via placenta or breast milk (P04.-)
Q86.0 Fetal alcohol syndrome (dysmorphic)
Q86.1 Fetal hydantoin syndrome
Meadow's syndrome
Q86.2 Dysmorphism due to warfarin
Q86.8 Other congenital malformation syndromes due to known exogenous causes
Q87 Other specified congenital malformation syndromes affecting multiple systems
UseAdditionalCode code(s) to identify all associated manifestations
Q87.0 Congenital malformation syndromes predominantly affecting facial appearance
Acrocephalopolysyndactyly
Acrocephalosyndactyly [Apert]
Cryptophthalmos syndrome
Cyclopia
Goldenhar syndrome
Moebius syndrome
Oro-facial-digital syndrome
Robin syndrome
Whistling face
Q87.1 Congenital malformation syndromes predominantly associated with short stature
Excludes1 Ellis-van Creveld syndrome (Q77.6)
Smith-Lemli-Opitz syndrome (E78.72)
Q87.11 Prader-Willi syndrome
Q87.19 Other congenital malformation syndromes predominantly associated with short stature
Aarskog syndrome
Cockayne syndrome
De Lange syndrome
Dubowitz syndrome
Noonan syndrome
Robinow-Silverman-Smith syndrome
Russell-Silver syndrome
Seckel syndrome
Q87.2 Congenital malformation syndromes predominantly involving limbs
Holt-Oram syndrome
Klippel-Trenaunay-Weber syndrome
Nail patella syndrome
Rubinstein-Taybi syndrome
Sirenomelia syndrome
Thrombocytopenia with absent radius [TAR] syndrome
VATER syndrome
Q87.3 Congenital malformation syndromes involving early overgrowth
Beckwith-Wiedemann syndrome
Sotos syndrome
Weaver syndrome
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
Excludes1 Zellweger syndrome (E71.510)
Q87.81 Alport syndrome
UseAdditionalCode code to identify stage of chronic kidney disease (N18.1-N18.6)
Q87.82 Arterial tortuosity syndrome
Q87.83 Bardet-Biedl syndrome
Q87.84 Laurence-Moon syndrome
Q87.85 MED13L syndrome
Asadollahi-Rauch syndrome
Mediator complex subunit 13L syndrome
CodeAlso , if applicable, any associated manifestations such as:
autism spectrum disorder (F84.0-)
congenital malformations of cardiac septa (Q21-)
epilepsy and recurrent seizures (G40.-)
intellectual disability (F70-F79)
Q87.89 Other specified congenital malformation syndromes, not elsewhere classified
Q89 Other congenital malformations, not elsewhere classified
Q89.0 Congenital absence and malformations of spleen
Excludes1 isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)
Q89.01 Asplenia (congenital)
Q89.09 Congenital malformations of spleen
Congenital splenomegaly
Q89.1 Congenital malformations of adrenal gland
Excludes1 adrenogenital disorders (E25.-)
congenital adrenal hyperplasia (E25.0)
Q89.2 Congenital malformations of other endocrine glands
Congenital malformation of parathyroid or thyroid gland
Persistent thyroglossal duct
Thyroglossal cyst
Excludes1 congenital goiter (E03.0)
congenital hypothyroidism (E03.1)
Q89.3 Situs inversus
Dextrocardia with situs inversus
Mirror-image atrial arrangement with situs inversus
Situs inversus or transversus abdominalis
Situs inversus or transversus thoracis
Transposition of abdominal viscera
Transposition of thoracic viscera
Excludes1 dextrocardia NOS (Q24.0)
Q89.4 Conjoined twins
Craniopagus
Dicephaly
Pygopagus
Thoracopagus
Q89.7 Multiple congenital malformations, not elsewhere classified
Multiple congenital anomalies NOS
Multiple congenital deformities NOS
Excludes1 congenital malformation syndromes affecting multiple systems (Q87.-)
Q89.8 Other specified congenital malformations
UseAdditionalCode code(s) to identify all associated manifestations
Q89.9 Congenital malformation, unspecified
Congenital anomaly NOS
Congenital deformity NOS