Jansen-de Vries syndrome
Elejalde disease
Congenital conductive hearing loss
Genetic syndromes of diabetes mellitus
Trismus pseudocamptodactyly syndrome
Syndromic microphthalmia type 9
Baraitser Brett Piesowicz syndrome
X-linked intellectual disability Snyder type
Distal arthrogryposis type 7
Anterior corneal dystrophy
Polynesian bronchiectasis
Dermochondrocorneal dystrophy
Congenital atresia of external auditory canal
Biedl-Bardet syndrome
Immotile cilia syndrome due to defective radial spokes
C-like syndrome
Bardet-Biedl syndrome
Aural atresia with multiple congenital anomalies and intellectual disability syndrome
RAB18, member RAS oncogene family deficiency
Brachydactyly, scoliosis, carpal fusion syndrome
Feingold syndrome
Myhre syndrome
Absent eyebrow
Scaphocephaly
Lymphedema, lymphangiectasia, intellectual disability syndrome
VACTEL syndrome
Oculofaciocardiodental syndrome
Fibrofolliculomas with trichodiscomas and acrochordons
Partial ablepharon
Atypical long QT syndrome type 8
Choroideraemia
Pascual Castroviejo syndrome type 2
Hecht Beals syndrome
Johnson McMillin syndrome
Bowen Hutterite syndrome
Birk Barel mental retardation dysmorphism syndrome
Bohring syndrome
Adrenal cortical nodule
Sensory hearing loss
Open eye
Hydrometrocolpos, postaxial polydactyly, and congenital heart malformation
Fetal anemia
Increased tendon reflexes
Bamforth Lazarus syndrome
Craniofacial deafness hand syndrome
Oculo-facio-cardio-dental syndrome
Waardenburg syndrome type 2
Bosley Salih Alorainy syndrome
Apple peel intestinal atresia, ocular anomalies, microcephaly syndrome
Interparietal craniosynostosis
Bone age finding
Branchio-skeleto-genital syndrome
McKusick Kaufman syndrome
Congenital corneal dystrophy
Insulin resistance
Fetal akinesia-hypokinesia sequence
Young-Simpson syndrome
Periodontitis exacerbated by Cohen syndrome
Hydrops fetalis
Ear, patella, short stature syndrome
Micronodular adrenal hyperplasia
Retinopathy, anaemia, central nervous system anomalies syndrome
Congenital coloboma of iris
Waardenburg's syndrome
Ligase 4 syndrome
Microcephaly-mesobrachyphalangy-tracheoesophageal fistula syndrome
Blepharophimosis-mental retardation syndrome, Maat-Kievit-Brunner type
Pleuropulmonary blastoma family tumor susceptibility syndrome
Grubben, De Cock, Borghgraef syndrome
Sommer Young Wee Frye syndrome
German syndrome
DKC - Dyskeratosis congenita
Cutis laxa-corneal clouding-oligophrenia syndrome
Developmental delay, behavioural problems, small hands and feet, cyclic vomiting, dysmorphic features syndrome
FADS - foetal akinesia deformation sequence
Nicolaides-Baraitser syndrome
Marfanoid syndrome De Silva type
Blepharophimosis, intellectual disability syndrome, Say-Barber-Biesecker-Young-Simpson type
Timothy syndrome atypical type
Azoospermia, obstructive and chronic sinopulmonary infections
Hall Berg Rudolph syndrome
Say-Barber-Biesecker-Young-Simpson variant of Ohdo syndrome
Biemond's syndrome
Mixed hearing loss
Fibrofolliculoma
Blastogenesis defect
Waardenburg syndrome type II
Revesz syndrome
Intellectual disability Birk-Barel type
Hyperadrenalism
Oculo-digito-oesophagoduodenal syndrome
PURA-related severe neonatal hypotonia, seizure, encephalopathy syndrome
SSPS - Schöpf Schulz Passarge syndrome
Fetal acidosis
Long QT syndrome type 8
Sohar-Crisponi syndrome
Cleft iris
Posterior fossa brain malformation, haemangioma, arterial anomaly, cardiac defect and aortic coarctation, and eye abnormality syndrome
Primary ciliary dyskinesia and retinitis pigmentosa syndrome
Multiple fibrofolliculomata
Disorder of serine metabolism
Hypoplastic anemia - familial
Blepharophimosis-intellectual disability syndrome Maat-Kievit-Brunner type
Tapetochoroidal dystrophy
Wolfram-like syndrome
KLHL7 (kelch like family member 7) related Bohring-Opitz-like and Crisponi/cold-induced sweating-like overlap syndrome
Alveolar bone loss
Intellectual disability, hypotonia, facial dysmorphism syndrome
RAB18 deficiency
PIBIDS syndrome, photosensitivity with IBIDS
Timothy syndrome type 1
Autosomal recessive cutis laxa type III
Johnson neuroectodermal syndrome
X-linked Angelman-like syndrome
Hereditary disorder of lymphatic system
Adrenal gland hyperfunction
Golabi-Ito-Hall syndrome
PCD - Primary ciliary dyskinesia
11p15 deletion syndrome
Cold-induced sweating syndrome
Hypothyroidism, dysmorphism, postaxial polydactyly, intellectual disability syndrome
Keratosis palmoplantaris with periodontopathia and onychogryposis syndrome
Rutherfurd syndrome
Young syndrome
Neuroectodermal melanolysosomal disease
Metabolic disorder of fetus
11p partial monosomy syndrome
Faciooculoacousticorenal syndrome
Jejunal atresia, microcephaly, ocular anomalies syndrome
Stimmler syndrome
Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-esophageal fistula, limb defects (VACTEL) syndrome
Fixed dilated pupil
Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-esophageal fistula, limb defects syndrome
Sinusitis-infertility syndrome
XY type gonadal dysgenesis with associated anomalies syndrome
Cardiac urogenital syndrome
RERE-related neurodevelopmental syndrome
Oculodento-osseous dysplasia - severe type
Nodule of adrenal cortex
Oculodento-osseous dysplasia - mild type
Patent ductus Botalli
Keratosis palmoplantaris, cystic eyelids, hypodontia, hypotrichosis syndrome
Cole-Engmann-Zinsser syndrome
Kelch like family member 7-related Bohring Opitz-like syndrome
Branchio-otic syndrome
Carney complex, trismus, pseudocamptodactyly syndrome
Foetal anaemia
Cernunnos-XLF deficiency
Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-oesophageal fistula, renal anomalies, limb defects (VACTERL) syndrome
Adrenal hyperfunction
Intestinal lymphangiectasis
Microcephaly-oculo-digito-oesophageal-duodenal syndrome
Fetal metabolic disorder
Cochlear hearing loss
Porteous syndrome
de Barsey syndrome
Constitutional aplastic anemia
Congenital intrauterine infection-like syndrome
Hypothyroidism and cleft palate syndrome
Juvenile periodontosis with hyperkeratosis
3-Phosphoglycerate dehydrogenase deficiency
Autosomal dominant popliteal pterygium syndrome
Bilateral band-like calcification with polymicrogyria
PHACE syndrome
Sagittal craniostenosis with congenital heart disease, mental deficiency and mandibular ankylosis
Allemann syndrome
Acrodysplasia scoliosis
Pleuropulmonary blastoma familial tumor and dysplasia syndrome
Scholte syndrome
Biemond syndrome
Schöpf-Schulz-Passarge syndrome
VATER-like syndrome with pulmonary hypertension, abnormal ear and growth deficiency
Symptomatic form of Coffin-Lowry syndrome in female carrier
c-PPNAD - Carney complex-associated-primary pigmented nodular adrenocortical disease
Revesz DeBuse syndrome
Gonadal dysgenesis XY type with associated anomalies
Retarded bone age
Ablepharon - absent eyelids
Palmoplantar hyperkeratosis, cystic eyelids, hypodontia, hypotrichosis syndrome
KCNK9 (potassium two pore domain channel subfamily K member 9) imprinting syndrome
Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-esophageal fistula, renal anomalies, limb defects syndrome
Ohdo syndrome, Maat-Kievit-Brunner type
Ablepharia
Chromodomain helicase DNA binding protein 4-related neurodevelopmental disorder
Kelch like family member 7-related Bohring-Opitz-like and Crisponi/cold-induced sweating-like overlap syndrome
Timothy syndrome classic type
Foetal acidosis
KLHL7-related Crisponi/cold-induced sweating-like syndrome
Pure gonadal dysgenesis
Generalised dystonia
LIG4 syndrome
Pleuropulmonary blastoma familial tumour and dysplasia syndrome
Craniosynostosis with congenital heart disease and intellectual disability syndrome
Deletion of part of long arm of chromosome 12
Rutland ciliary disorientation syndrome
Multiple system malformation syndrome
Foetal akinesia-hypokinesia sequence
Fixed pupil up
Axial mesodermal dysplasia spectrum
Hereditary choroidal atrophy
12q15 deletion syndrome
KLHL7-related Bohring-Opitz-like and Crisponi/cold-induced sweating-like overlap syndrome
Aplasia cutis congenita secondary to malformation syndrome (Type 9)
Congenital anomaly of lymphatic structure of trunk
Renpenning syndrome
Retinopathy, anemia, central nervous system anomalies syndrome
Infantile hemangioma
Stern Lubinsky Durrie syndrome
Malabsorption of glucose
Cohen syndrome
Congenital peripheral pulmonary artery stenosis
Intestinal lymphangiectasia
Exaggeration of the deep reflexes
ICS - Immotile cilia syndrome
Benign tumour of heart
Diaphragmatic hernia-exomphalos-hypertelorism syndrome
Carney complex variant
NLS - Neu-Laxova syndrome
Congenital osteodystrophy
Scholte Begeer-van Essen syndrome
Opitz Kaveggia syndrome
Nodular hyperplasia of adrenal cortex
Alstrom syndrome
HF - Hydrops foetalis
Gomez Lopez Hernandez syndrome
Mixed type deafness
Slow to talk
Patent ductus arteriosus
PHACES syndrome
Congenital dyskeratosis
VACTERL syndrome
Vici syndrome
Anophthalmia with pulmonary hypoplasia syndrome
Sagittal synostosis
Severe combined immunodeficiency, microcephaly, growth retardation, sensitivity to ionizing radiation syndrome
Laminin subunit alpha 5-related multisystemic syndrome
Meier-Gorlin syndrome
PDA - Patent ductus arteriosus
Atypical Timothy syndrome
Stromme syndrome
KLHL7-related Bohring Opitz-like syndrome
Marfanoid physique
Immotile cilia syndrome
Autosomal recessive popliteal pterygium syndrome
Facial dysmorphism, intellectual deficit, short stature and hearing loss
Hornstein Knickenberg syndrome
PURA syndrome
Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-oesophageal fistula, renal anomalies, limb defects syndrome
Congenital lymphangiectasia
N syndrome
Progeroid syndrome of de Barsey
Francois syndrome
Developmental delay, behavioral problems, small hands and feet, cyclic vomiting, dysmorphic features syndrome
Laryngotracheal stenosis, arthropathy, prognathism and short stature
MYRF-related cardiac urogenital syndrome
Periodontitis due to Papillon-Lefèvre syndrome
Hennekam lymphangiectasia-lymphedema syndrome
Pseudoprogeria syndrome
Mixed conductive AND sensorineural hearing loss
Posterior fossa brain malformation, haemangioma, arterial anomaly, cardiac defect, aortic coarctation, eye abnormality, sternal anomalies syndrome
3-phosphoglycerate dehydrogenase deficiency neonatal form
Developmental malformation of branchial arch
Laurence-Moon-Biedl syndrome
Carney syndrome
Siegler Brewer Carey syndrome
12q24.31-q24.32 deletion syndrome
Eccrine tumors ectodermal dysplasia
MHL - Mixed hearing loss
Birt Hogg Dubé syndrome
Posterior fossa brain malformation, hemangioma, arterial anomaly, cardiac defect, aortic coarctation, eye abnormality, sternal anomalies syndrome
Hecht syndrome
Sifrim Hitz Weiss syndrome
CHD4-related neurodevelopmental disorder
X-linked retinitis pigmentosa
Arginine-glutamic acid dipeptide repeats-related neurodevelopmental syndrome
LMBB - Laurence-Moon-Bardet-Biedl syndrome
Alkuraya Kucinskas syndrome
Singleton-Merten syndrome
Photosensitivity with ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature syndrome
Patterson syndrome
Myxoma, spotty pigmentation, endocrine overactivity syndrome
Blepharophimosis-intellectual deficit syndrome, Say-Barber/Biesecker/Young-Simpson type
Autosomal dominant dyskeratosis congenita
FG syndrome
Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-esophageal fistula, renal anomalies, limb defects (VACTERL) syndrome
Oculodentodigital dysplasia
Dyskeratosis congenita
Hyperreflexia
Oculo-digito-esophagoduodenal syndrome
Delayed bone age
Bowen-Conradi Hutterite syndrome
PPS - Peripheral pulmonary stenosis
Allemann's syndrome
NHEJ1 (non-homologous end joining factor) syndrome
Swyer syndrome
Aortic aneurysm due to Loeys-Dietz syndrome
Opitz trigonocephaly-like syndrome
Dysmotile cilia syndrome
Say-Barber-Biesecker-Young-Simpson syndrome
Long QT syndrome with syndactyly
Popliteal web syndrome
Ohdo syndrome, Say-Barber-Biesecker-Young-Simpson variant
PURA-related neurodevelopmental disorder
Hennekam lymphangiectasia-lymphoedema syndrome
Matthew Wood syndrome
Laurence-Moon syndrome
Biemond syndrome type 2
KLHL7 (kelch like family member 7) related Bohring Opitz-like syndrome
KLHL7 (kelch like family member 7) related Crisponi/cold-induced sweating-like syndrome
Trichothiodystrophy
Congenital lymphangiectasis
Keutel syndrome
Notched iris
Palmoplantar keratoderma, cystic eyelids, hypodontia, hypotrichosis syndrome
Patterson pseudoleprechaunism syndrome
Deletion of part of chromosome 11
PERCHING syndrome
Pleuropulmonary blastoma family tumour susceptibility syndrome
Laband-Zimmermann syndrome
Athyroidal hypothyroidism with spiky hair and cleft palate syndrome
Zinsser-Cole-Engman syndrome
Myxoma of heart
Dystopia canthorum
Primary ciliary dyskinesia due to transposition of ciliary microtubules
Athetoid movement
HF - Hydrops fetalis
LAMA5-related multisystemic syndrome
KIAA1109-related early lethal congenital brain malformations, arthrogryposis syndrome
Carney complex
Genetic syndromic childhood obesity
Diaphragmatic hernia-exomphalos-corpus callosum agenesis
Developmental delay, hypotonia, extremities hypertrophy syndrome
X-linked intellectual disability Pai type
Waardenburg syndrome type I
Atresia of external auditory canal
Absent eyebrows and eyelashes with intellectual disability syndrome
Oculodentodigital syndrome
Hutterite syndrome
Crisponi syndrome
Dyskeratosis congenita with bilateral exudative retinopathy
Dionisi Vici Sabetta Gambarara syndrome
X-linked intellectual disability Armfield type
Pseudoleprechaunism syndrome Patterson type
X-linked intellectual deficit due to PQBP1 mutation
Generalized dystonia
Eccrine tumours ectodermal dysplasia
Microcephaly-oculo-digito-esophageal-duodenal syndrome
Patent arterial duct
Infantile haemangioma
Congenital aplastic anaemia
Congenital stenosis of pulmonary artery
Nance-Horan syndrome
Congenital lymphangiectases
Pure gonadal dysgenesis 46,XY
Rhombencephalosynapsis
Choroideremia
Cerebellotrigeminal dermal dysplasia syndrome
Meacham Winn Culler syndrome
DNA (deoxyribonucleic acid) ligase IV deficiency
Fetal anaemia
Arthrogryposis multiplex congenita, pulmonary hypoplasia syndrome
KLHL7-related Crisponi-like syndrome
Fixed dilatation of pupil
Lowry MacLean syndrome
JDVS - Jansen-de Vries syndrome
Corneo-dermato-osseous syndrome
Hypoplastic anaemia - familial
Bowen-Conradi syndrome
Branchioskeletogenital syndrome
Severe combined immunodeficiency, microcephaly, growth retardation, sensitivity to ionising radiation syndrome
Zinsser-Cole-Engmann syndrome
Juberg Hayward syndrome
Microphthalmia syndromic 2
Waardenburg syndrome
Facio-genito-popliteal syndrome
Bohring Opitz syndrome
Mixed conductive and sensorineural deafness
Pena-Shokeir syndrome type I
Congenital atrophy of optic nerve
Mixed deafness
Multiple malformation syndrome with senile-like appearance
Corneodermatoosseous syndrome
Speech delay
Bartsocas Papas syndrome
Cutis laxa, autosomal recessive
Waardenburg syndrome type 1
Congenital malformation of the eyebrow
CHD4-related neurodevelopmental syndrome
Dutch Kentucky syndrome
Severe combined immunodeficiency due to Cernunnos protein deficiency
Papillon-Lefevre syndrome
Heritable pulmonary arterial hypertension
Hydrops foetalis
Diabetes mellitus associated with genetic syndrome
Hamel cerebropalatocardiac syndrome
Hydrops, lactic acidosis, sideroblastic anemia, multisystemic failure syndrome
PURA (purine rich element binding protein A) syndrome
Floating-Harbor syndrome
Christianson syndrome
Cooper Jabs syndrome
Multiple fibrofolliculomas
Dystrophy of anterior cornea
Snyder-Robinson syndrome
FADS - fetal akinesia deformation sequence
Young's syndrome
Prata Liberal Goncalves syndrome
Barry-Perkins-Young syndrome
Carney complex-associated-primary pigmented nodular adrenocortical disease
Zimmermann-Laband syndrome
Microcephaly, intracranial calcification, intellectual disability syndrome
Periodontitis due to Papillon-Lefevre syndrome
de Barsey-Moens-Dierckx syndrome
3MC syndrome
FG syndrome type 1
Micro syndrome
Oculotrichodysplasia
Andersen Tawil syndrome
Myelin regulatory factor-related cardiac urogenital syndrome
Pallister-Hall syndrome
Purine rich element binding protein A syndrome
Armfield syndrome
Schöpf Schulz Passarge syndrome
Developmental malformation, deafness, dystonia syndrome
Pfeiffer Singer Zschiesche syndrome
Deoxyribonucleic acid ligase IV deficiency
X-linked intellectual developmental disorder Christianson type
Polyglandular hyperfunction
Currarino triad
Benign neoplasm of heart
Neu-Laxova syndrome
Marden Walker syndrome
Peripheral pulmonary artery stenosis
Timothy syndrome
Orocraniodigital syndrome
Posterior fossa brain malformation, hemangioma, arterial anomaly, cardiac defect and aortic coarctation, and eye abnormality syndrome
Lymphoedema, lymphangiectasia, intellectual disability syndrome
Congenital absence of cilia of eyelid
Aplasia cutis in Johanson-Blizzard syndrome
Andersen cardiodysrhythmic periodic paralysis
Primary ciliary dyskinesia
Microphthalmia, cataract, radiculomegaly and septal heart defect
Pseudo-TORCH syndrome
Pulmonic stenosis, brachytelephalangism, calcification of cartilage syndrome
Sagittal craniosynostosis
Papillon-Lefèvre syndrome
DICER1 syndrome
Severe combined immunodeficiency with low T- and B-cell numbers
Haim Munk syndrome
Hennekam syndrome
Cecatto de Lima Pinheiro syndrome
Branchiootic syndrome
LAMB (lentigines, atrial myxoma, blue nevi) syndrome
Chudley McCullough syndrome
Alaninuria, microcephaly, dwarfism, enamel hypoplasia, diabetes mellitus syndrome
Braddock syndrome
Russell Weaver Bull syndrome
Congenital optic atrophy
BSG syndrome
Congenital mixed conductive and sensorineural hearing loss
Weaver Williams syndrome
Multisystemic smooth muscle dysfunction syndrome
Sutherland-Haan syndrome
Ablepharon
Cardiocranial syndrome Pfeiffer type
Costello syndrome
Alopecia, anosmia, deafness, hypogonadism syndrome
Blepharophimosis, intellectual disability syndrome
Periodic paralysis
Benign tumor of heart
Popliteal pterygium syndrome
LAMB (lentigines, atrial myxoma, blue naevi) syndrome
Lethal popliteal pterygium syndrome
Snyder-Robinson x-linked mental retardation syndrome
Constitutional aplastic anaemia
Kelch like family member 7-related Crisponi/cold-induced sweating-like syndrome
Congenital anomaly of the eyebrow
Mental retardation, x-linked, Snyder-Robinson type
Meacham syndrome
Oculodento-osseous dysplasia
Curtius' syndrome I
Fried syndrome
Inner ear hearing loss
Hereditary choroidal dystrophy
Hydrops, lactic acidosis, sideroblastic anaemia, multisystemic failure syndrome
Warburg micro syndrome
X-linked Ohdo syndrome
Long QT syndrome 7
Van den Bosch syndrome
Ciliary neurotrophic factor receptor-related disorder
Congenital dystrophy of cornea
Congenital absence of eyelash
Loeys-Dietz syndrome
Craniofacial ulnar renal syndrome
Microtia, absent patellae, micrognathia syndrome
Oto-onycho-peroneal syndrome
LIG4 (ligase 4) syndrome
Oculodental syndrome
Congenital aplastic anemia
Intellectual disability, balding, patella luxation, acromicria syndrome
Donnai-Barrow syndrome

Frequently Asked Questions

What is the ICD-10 code for oth congenital malformation syndromes, nec?

The ICD-10-CM code for oth congenital malformation syndromes, nec is Q87.89. The full clinical description is "Other specified congenital malformation syndromes, not elsewhere classified". Q87.89 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code Q87.89 mean?

ICD-10-CM code Q87.89 represents “Other specified congenital malformation syndromes, not elsewhere classified”. It is classified under Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities and is a billable/specific code that can be used on a claim.

Is Q87.89 a billable code?

Yes, Q87.89 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is Q87.89 in?

Q87.89 is in Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (codes Q00-Q99).

What codes cannot be used with Q87.89?

Q87.89 has Excludes1 notes indicating codes that cannot be used together with it, including: Zellweger syndrome (E71.510).

Are additional codes required with Q87.89?

Yes, when using Q87.89, also report: code(s) to identify all associated manifestations.

What SNOMED CT codes does Q87.89 map to?

Q87.89 maps to 219 SNOMED CT concepts: 4135001, 880078001, 880081006, 880086001, 303098002, and 214 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for Q87.89?

Q87.89 is linked to 1 UMLS Concept Unique Identifier: C0869083. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does Q87.89 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like oth congenital malformation syndromes, nec affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of Q87.89?

There is no direct ICD-11 mapping available for Q87.89 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.