ICD-10-CM Excludes1 Notes · Chapter 17 · FY 2027 Q00-QA1

Excludes1 Notes in Chapter 17: Congenital malformations, deformations and chromosomal abnormalities ICD-10-CM

An Excludes1 note means "NOT CODED HERE": the excluded code should never be reported together with the code above the note. In Chapter 17 (Q00-QA1), notes printed in the chapter name other codes 189 times, and Excludes1 Notes anywhere in the Tabular List name the chapter’s codes 301 times, covering 187 distinct codes, categories and ranges. Switch views to read the list either way.

✓ From the official FY 2027 ICD-10-CM Tabular ListDefinitions follow the Official Guidelines, Section I.A
189
References in Its Notes
301
References to Its Codes
187
Chapter Codes Named
Q00-QA1
Chapter 17
Chapter 17

Excludes1 Notes by the code that carries the note

106 of 106 shown
  • Q01 Encephalocele applies to 5 codes
    • Meckel-Gruber syndrome (Q61.9)
  • Q02 Microcephaly
    • Meckel-Gruber syndrome (Q61.9)
  • Q03 Congenital hydrocephalus applies to 4 codes
    • Arnold-Chiari syndrome, type II (Q07.0-)
    • acquired hydrocephalus (G91.-)
    • hydrocephalus due to congenital toxoplasmosis (P37.1)
    • hydrocephalus with spina bifida (Q05.0-Q05.4)
  • Q04 Other congenital malformations of brain applies to 9 codes
  • Q04.3 Other reduction deformities of brain
    • congenital malformations of corpus callosum (Q04.0)
  • Q04.6 Congenital cerebral cysts
    • acquired porencephalic cyst (G93.0)
  • Q05 Spina bifida applies to 10 codes
    • Arnold-Chiari syndrome, type II (Q07.0-)
    • spina bifida occulta (Q76.0)
  • Q07.0 Arnold-Chiari syndrome applies to 4 codes
    • Arnold-Chiari syndrome, type III (Q01.-)
    • Arnold-Chiari syndrome, type IV (Q04.8)
  • Q10 Congenital malformations of eyelid, lacrimal apparatus and orbit applies to 8 codes
    • cryptophthalmos NOS (Q11.2)
    • cryptophthalmos syndrome (Q87.0)
  • Q11.2 Microphthalmos
    • cryptophthalmos syndrome (Q87.0)
  • Q11.3 Macrophthalmos
    • macrophthalmos in congenital glaucoma (Q15.0)
  • Q15 Other congenital malformations of eye applies to 3 codes
  • Q16 Congenital malformations of ear causing impairment of hearing applies to 7 codes
    • congenital deafness (H90.-)
  • Q17 Other congenital malformations of ear applies to 8 codes
    • congenital malformations of ear with impairment of hearing (Q16.0-Q16.9)
    • preauricular sinus (Q18.1)
  • Q17.4 Misplaced ear
  • Q18 Other congenital malformations of face and neck applies to 10 codes
    • cleft lip and cleft palate (Q35-Q37)
    • conditions classified to Q67.0-Q67.4
    • congenital malformations of skull and face bones (Q75.-)
    • cyclopia (Q87.0)
    • dentofacial anomalies [including malocclusion] (M26.-)
    • malformation syndromes affecting facial appearance (Q87.0)
    • persistent thyroglossal duct (Q89.2)
  • Q20 Congenital malformations of cardiac chambers and connections applies to 9 codes
    • dextrocardia with situs inversus (Q89.3)
    • mirror-image atrial arrangement with situs inversus (Q89.3)
  • Q20.0 Common arterial trunk
    • aortic septal defect (Q21.4)
  • Q21 Congenital malformations of cardiac septa applies to 17 codes
    • acquired cardiac septal defect (I51.0)
  • Q23.0 Congenital stenosis of aortic valve
    • congenital stenosis of aortic valve in hypoplastic left heart syndrome (Q23.4)
    • congenital subaortic stenosis (Q24.4)
    • supravalvular aortic stenosis (congenital) (Q25.3)
  • Q24 Other congenital malformations of heart applies to 9 codes
    • endocardial fibroelastosis (I42.4)
  • Q24.0 Dextrocardia
    • dextrocardia with situs inversus (Q89.3)
    • isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)
    • mirror-image atrial arrangement with situs inversus (Q89.3)
  • Q25.3 Supravalvular aortic stenosis
    • congenital aortic stenosis NOS (Q23.0)
    • congenital stenosis of aortic valve (Q23.0)
  • Q25.4 Other congenital malformations of aorta applies to 10 codes
    • hypoplasia of aorta in hypoplastic left heart syndrome (Q23.4)
  • Q27.3 Arteriovenous malformation (peripheral) applies to 6 codes
    • acquired arteriovenous aneurysm (I77.0)
  • Q27.8 Other specified congenital malformations of peripheral vascular system
    • arteriovenous malformation (Q27.3-)
  • Q28 Other congenital malformations of circulatory system applies to 6 codes
    • congenital aneurysm NOS (Q27.8)
    • congenital coronary aneurysm (Q24.5)
    • ruptured cerebral arteriovenous malformation (I60.8)
    • ruptured malformation of precerebral vessels (I72.0)
  • Q30 Congenital malformations of nose applies to 6 codes
    • congenital deviation of nasal septum (Q67.4)
  • Q31 Congenital malformations of larynx applies to 7 codes
    • congenital laryngeal stridor NOS (P28.89)
  • Q32 Congenital malformations of trachea and bronchus applies to 5 codes
    • congenital bronchiectasis (Q33.4)
  • Q33.0 Congenital cystic lung
    • cystic fibrosis (E84.0)
    • cystic lung disease, acquired or unspecified (J98.4)
  • Q33.6 Congenital hypoplasia and dysplasia of lung
    • pulmonary hypoplasia associated with short gestation (P28.0)
  • Q35 Cleft palate applies to 5 codes
    • cleft palate with cleft lip (Q37.-)
  • Q36 Cleft lip applies to 3 codes
    • cleft lip with cleft palate (Q37.-)
  • Q38 Other congenital malformations of tongue, mouth and pharynx applies to 9 codes
  • Q38.0 Congenital malformations of lips, not elsewhere classified
  • Q38.5 Congenital malformations of palate, not elsewhere classified
    • cleft palate (Q35.-)
    • cleft palate with cleft lip (Q37.-)
  • Q38.7 Congenital pharyngeal pouch
    • pharyngeal pouch syndrome (D82.1)
  • Q40.1 Congenital hiatus hernia
    • congenital diaphragmatic hernia (Q79.0)
  • Q41 Congenital absence, atresia and stenosis of small intestine applies to 5 codes
    • cystic fibrosis with intestinal manifestation (E84.11)
    • meconium ileus NOS (without cystic fibrosis) (P76.0)
  • Q43.6 Congenital fistula of rectum and anus
    • congenital fistula of anus with absence, atresia and stenosis (Q42.2)
    • congenital fistula of rectum with absence, atresia and stenosis (Q42.0)
    • congenital rectovaginal fistula (Q52.2)
    • congenital urethrorectal fistula (Q64.73)
    • pilonidal fistula or sinus (L05.-)
  • Q45.3 Other congenital malformations of pancreas and pancreatic duct
    • congenital diabetes mellitus (E10.-)
    • cystic fibrosis (E84.0-E84.9)
    • fibrocystic disease of pancreas (E84.-)
    • neonatal diabetes mellitus (P70.2)
  • Block note Block Q50-Q56 Congenital malformations of genital organs applies to 91 codes
    • androgen insensitivity syndrome (E34.5-)
    • syndromes associated with anomalies in the number and form of chromosomes (Q90-Q99)
  • Q50.0 Congenital absence of ovary applies to 2 codes
    • Turner's syndrome (Q96.-)
  • Q52.1 Doubling of vagina applies to 8 codes
    • doubling of vagina with doubling of uterus and cervix (Q51.1-)
  • Q52.2 Congenital rectovaginal fistula
  • Q54 Hypospadias applies to 7 codes
  • Q55 Other congenital malformations of male genital organs applies to 18 codes
  • Q55.63 Congenital torsion of penis
    • acquired torsion of penis (N48.82)
  • Q55.64 Hidden penis
    • acquired buried penis (N48.83)
  • Q56 Indeterminate sex and pseudohermaphroditism applies to 5 codes
    • 46,XX true hermaphrodite (Q99.1)
    • androgen insensitivity syndrome (E34.5-)
    • chimera 46,XX/46,XY true hermaphrodite (Q99.0)
    • female pseudohermaphroditism with adrenocortical disorder (E25.-)
    • pseudohermaphroditism with specified chromosomal anomaly (Q96-Q99)
    • pure gonadal dysgenesis (Q99.1)
  • Q61 Cystic kidney disease applies to 11 codes
    • acquired cyst of kidney (N28.1)
    • Potter's syndrome (Q60.6)
  • Q61.4 Renal dysplasia
  • Q63 Other congenital malformations of kidney applies to 6 codes
    • congenital nephrotic syndrome (N04.-)
  • Q64.0 Epispadias
  • Q64.7 Other and unspecified congenital malformations of bladder and urethra applies to 7 codes
    • congenital prolapse of bladder (mucosa) (Q79.4)
  • Q65 Congenital deformities of hip applies to 15 codes
  • Q66 Congenital deformities of feet applies to 32 codes
    • reduction defects of feet (Q72.-)
    • valgus deformities (acquired) (M21.0-)
    • varus deformities (acquired) (M21.1-)
  • Q66.5 Congenital pes planus applies to 3 codes
    • pes planus, acquired (M21.4)
  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest applies to 9 codes
    • congenital malformation syndromes classified to Q87.-
    • Potter's syndrome (Q60.6)
  • Q67.2 Dolichocephaly
    • sagittal craniosynostosis (Q75.01)
  • Q67.3 Plagiocephaly
  • Q67.4 Other congenital deformities of skull, face and jaw
    • dentofacial anomalies [including malocclusion] (M26.-)
    • syphilitic saddle nose (A50.5)
  • Q67.5 Congenital deformity of spine
    • infantile idiopathic scoliosis (M41.0)
    • scoliosis due to congenital bony malformation (Q76.3)
  • Q68 Other congenital musculoskeletal deformities applies to 8 codes
    • reduction defects of limb(s) (Q71-Q73)
  • Q68.3 Congenital bowing of femur
    • anteversion of femur (neck) (Q65.89)
  • Q70.4 Polysyndactyly, unspecified
    • specified syndactyly of hand and feet - code to specified conditions (Q70.0- -Q70.3-)
  • Q74 Other congenital malformations of limb(s) applies to 6 codes
  • Q74.1 Congenital malformation of knee
    • congenital dislocation of knee (Q68.2)
    • congenital genu recurvatum (Q68.2)
    • nail patella syndrome (Q87.2)
  • Q74.2 Other congenital malformations of lower limb(s), including pelvic girdle
    • anteversion of femur (neck) (Q65.89)
  • Q75 Other congenital malformations of skull and face bones applies to 22 codes
    • congenital malformation of face NOS (Q18.-)
    • congenital malformation syndromes classified to Q87.-
    • dentofacial anomalies [including malocclusion] (M26.-)
    • musculoskeletal deformities of head and face (Q67.0-Q67.4)
    • anencephaly (Q00.0)
    • encephalocele (Q01.-)
    • hydrocephalus (Q03.-)
    • microcephaly (Q02)
  • Q75.01 Sagittal craniosynostosis
  • Q75.02 Coronal craniosynostosis applies to 3 codes
  • Q75.04 Lambdoid craniosynostosis applies to 3 codes
  • Q75.058 Other multi-suture craniosynostosis
    • coronal craniosynostosis, bilateral (Q75.022)
    • lambdoid craniosynostosis, bilateral (Q75.042)
  • Q76 Congenital malformations of spine and bony thorax applies to 21 codes
    • congenital musculoskeletal deformities of spine and chest (Q67.5-Q67.8)
  • Q76.0 Spina bifida occulta
    • meningocele (spinal) (Q05.-)
    • spina bifida (aperta) (cystica) (Q05.-)
  • Q76.2 Congenital spondylolisthesis
    • spondylolisthesis (acquired) (M43.1-)
    • spondylolysis (acquired) (M43.0-)
  • Q76.6 Other congenital malformations of ribs
    • short rib syndrome (Q77.2)
  • Q77 Osteochondrodysplasia with defects of growth of tubular bones and spine applies to 10 codes
  • Q77.3 Chondrodysplasia punctata
    • Rhizomelic chondrodysplasia punctata (E71.540)
  • Q79.0 Congenital diaphragmatic hernia
    • congenital hiatus hernia (Q40.1)
  • Q79.2 Exomphalos
  • Q79.5 Other congenital malformations of abdominal wall applies to 2 codes
  • Q80 Congenital ichthyosis applies to 7 codes
  • Q81.0 Epidermolysis bullosa simplex
  • Q82 Other congenital malformations of skin applies to 9 codes
    • acrodermatitis enteropathica (E83.2)
    • congenital erythropoietic porphyria (E80.0)
    • pilonidal cyst or sinus (L05.-)
    • Sturge-Weber (-Dimitri) syndrome (Q85.89)
  • Q82.2 Congenital cutaneous mastocytosis
    • 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)
  • Q82.4 Ectodermal dysplasia (anhidrotic)
    • Ellis-van Creveld syndrome (Q77.6)
  • Q82.8 Other specified congenital malformations of skin
    • Ehlers-Danlos syndromes (Q79.6-)
  • Q84.1 Congenital morphological disturbances of hair, not elsewhere classified
    • Menkes' kinky hair syndrome (E83.09)
  • Q84.3 Anonychia
    • nail patella syndrome (Q87.2)
  • Q85 Phakomatoses, not elsewhere classified applies to 11 codes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • familial dysautonomia [Riley-Day] (G90.1)
  • Q85.8 Other phakomatoses, not elsewhere classified applies to 4 codes
    • Meckel-Gruber syndrome (Q61.9)
  • Q87.1 Congenital malformation syndromes predominantly associated with short stature applies to 2 codes
    • Ellis-van Creveld syndrome (Q77.6)
    • Smith-Lemli-Opitz syndrome (E78.72)
  • Q87.8 Other specified congenital malformation syndromes, not elsewhere classified applies to 9 codes
  • Q87.A Loeys-Dietz syndrome
    • arterial tortuosity syndrome (Q87.82)
    • Ehlers-Danlos syndrome (Q79.6)
    • Marfan syndrome (Q87.40-Q87.43)
    • other systemic involvement of connective tissue (M35)
  • Q89.0 Congenital absence and malformations of spleen applies to 2 codes
    • isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)
  • Q89.1 Congenital malformations of adrenal gland
    • adrenogenital disorders (E25.-)
    • congenital adrenal hyperplasia (E25.0)
  • Q89.2 Congenital malformations of other endocrine glands
    • congenital goiter (E03.0)
    • congenital hypothyroidism (E03.1)
  • Q89.3 Situs inversus
  • Q89.7 Multiple congenital malformations, not elsewhere classified
    • congenital malformation syndromes affecting multiple systems (Q87.-)
  • Q92 Other trisomies and partial trisomies of the autosomes, not elsewhere classified applies to 9 codes
    • trisomies of chromosomes 13, 18, 21 (Q90-Q91)
  • Q92.2 Partial trisomy
    • partial trisomy due to unbalanced translocation (Q92.5)
  • Q96 Turner's syndrome applies to 7 codes
  • Q97 Other sex chromosome abnormalities, female phenotype, not elsewhere classified applies to 6 codes
    • Turner's syndrome (Q96.-)

The first view lists notes printed in this chapter (a note on a category or block applies to every code beneath it). The second lists codes of this chapter named by Excludes1 Notes anywhere in the Tabular List, including notes in other chapters.

Questions About Excludes1 Notes

What does an Excludes1 note mean?

An Excludes1 note means “NOT CODED HERE!”: the excluded code should never be used at the same time as the code above the note. It is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition (Section I.A.12.a). The FY 2027 Tabular List has 5,411 of them; 5,284 name other codes, with 5,710 references to 3,528 distinct codes, categories and ranges.

Is there any exception to an Excludes1 note?

Yes: when the two conditions are unrelated to each other. The Guidelines’ example is F45.8, whose Excludes1 note names sleep related teeth grinding (G47.63). A patient with psychogenic dysmenorrhea (an inclusion term of F45.8) and teeth grinding may have both codes reported. If it is not clear whether the conditions are related, query the provider.

Related References

ICD-10-CM Official GuidelinesICD-10-CM Code LookupICD-10-CM Codes ListCode Lists by Tag

Source: FY 2027 ICD-10-CM Tabular List (CMS and NCHS) and the ICD-10-CM Official Guidelines for Coding and Reporting FY 2027, Section I.A. ICD List is not affiliated with CMS or NCHS.