| Q80 |
Congenital ichthyosis [+]
| Excludes 1: |
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
| Excludes 1: |
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 [+]
| Excludes 1: |
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
| Excludes 1: |
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)
| Excludes 1: |
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
| Excludes 2: |
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
| Excludes 2: |
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]
| Excludes 1: |
Ehlers-Danlos syndromes (Q79.6-)
|
| Excludes 2: |
disorders of pyrophosphate metabolism (E83.82-)
|
|
| Q82.9 |
Congenital malformation of skin, unspecified
|
|
| Q83 |
Congenital malformations of breast [+]
| Excludes 2: |
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
| Excludes 1: |
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
| Excludes 1: |
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 [+]
| Excludes 1: |
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 [+]
| Excludes 1: |
Meckel-Gruber syndrome (Q61.9)
|
|
|
| Q85.81 |
PTEN hamartoma tumor syndrome
PHTS
PTEN related Cowden syndrome
| Code also |
, if applicable, genetic susceptibility to malignant neoplasm (Z15.0-)
|
|
| Q85.82 |
Other Cowden syndrome
|
| Q85.83 |
Von Hippel-Lindau syndrome
|
| 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 [+]
| Excludes 2: |
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 [+]
| Use additional |
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 [+]
| Excludes 1: |
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 [+]
| Excludes 1: |
Zellweger syndrome (E71.510)
|
|
|
| Q87.81 |
Alport syndrome
| Use additional |
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
| Code also |
, 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)
|
|
| Q87.86 |
Kleefstra syndrome
|
| Q87.87 |
Hao-Fountain Syndrome
HAFOUS
| Use additional |
code, if applicable, for associated conditions such as: |
| Use additional |
autism spectrum disorder (F84.0) |
| Use additional |
developmental speech disorder (F80.-) |
| Use additional |
epilepsy, by specific type (G40.-) |
| Use additional |
intellectual disabilities (F70-F79) |
| Use additional |
pervasive developmental disorders (F84.-) |
|
| Q87.88 |
CTNNB1 syndrome
| Use additional |
code, if applicable, for associated conditions such as: |
| Use additional |
cerebral palsy (G80.-) |
| Use additional |
congenital heart malformations (Q20.0-Q24.9) |
| Use additional |
developmental disorder of speech and language (F80.-) |
| Use additional |
exudative retinopathy (H35.02-) |
| Use additional |
intellectual disability (F70-F79) |
| Use additional |
microcephaly (Q02) |
|
| Q87.89 |
Other specified congenital malformation syndromes, not elsewhere classified
|
|
| Q87.A |
Loeys-Dietz syndrome
| Excludes 1: |
arterial tortuosity syndrome (Q87.82)
Ehlers-Danlos syndrome (Q79.6)
Marfan syndrome (Q87.40-Q87.43)
other systemic involvement of connective tissue (M35)
|
|
|
| Q89 |
Other congenital malformations, not elsewhere classified [+]
|
|
| Q89.0 |
Congenital absence and malformations of spleen [+]
| Excludes 1: |
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
| Excludes 1: |
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
| Excludes 1: |
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
| Excludes 1: |
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
| Excludes 1: |
congenital malformation syndromes affecting multiple systems (Q87.-)
|
|
| Q89.8 |
Other specified congenital malformations [+]
| Use additional |
code(s) to identify all associated manifestations |
|
|
| Q89.81 |
Kabuki syndrome
Kabuki syndrome, type 1, due to KMT2D mutation
Kabuki syndrome, type 2, due to KDM6A mutation
Niikawa-Kuroki syndrome
|
| Q89.89 |
Other specified congenital malformations
|
|
| Q89.9 |
Congenital malformation, unspecified
Congenital anomaly NOS
Congenital deformity NOS
|
|