EE01.1 Hypohidrosis due to genetic abnormalities of eccrine gland structure or function International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01 Hypohidrosis due to a heritable disorder of sweat gland or duct development. Sweating may be severely diminished or absent due to a paucity or absence of eccrine glands or to defective autonomic innervation. An absence of sweating leads to an inability to thermoregulate by evaporative cooling, and hyperthermia can occur with physical exertion or in a warm environment.
code elsewhere postcoordination Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.
Has causing condition - a selection is required - multiple selections are allowed [select] LA00 Anencephaly or similar anomalies – LA00.0 Anencephaly – LA00.1 Iniencephaly – LA00.2 Acephaly – LA00.3 Amyelencephaly – LA00.Y Other specified anencephaly or similar anomalies – LA00.Z Anencephaly or similar anomalies, unspecified –– LA00.00 Craniorachischisis –– LA00.0Y Other specified anencephaly –– LA00.0Z Anencephaly, unspecified LA01 Cephalocele LA02 Spina bifida – LA02.0 Spina bifida cystica – LA02.1 Spina bifida aperta – LA02.Y Other specified spina bifida – LA02.Z Spina bifida, unspecified –– LA02.00 Myelomeningocele with hydrocephalus –– LA02.01 Myelomeningocele without hydrocephalus –– LA02.02 Myelocystocele –– LA02.0Y Other specified spina bifida cystica –– LA02.0Z Spina bifida cystica, unspecified LA03 Arnold-Chiari malformation type II LA04 Congenital hydrocephalus – LA04.0 Hydrocephalus with stenosis of the aqueduct of Sylvius – LA04.Y Other specified congenital hydrocephalus – LA04.Z Congenital hydrocephalus, unspecified LA05 Cerebral structural developmental anomalies – LA05.0 Microcephaly – LA05.1 Megalencephaly – LA05.2 Holoprosencephaly – LA05.3 Corpus callosum agenesis – LA05.4 Arrhinencephaly – LA05.5 Abnormal neuronal migration – LA05.6 Encephaloclastic disorders – LA05.7 Brain cystic malformations – LA05.8 Colpocephaly – LA05.Y Other specified cerebral structural developmental anomalies – LA05.Z Cerebral structural developmental anomalies, unspecified –– LA05.50 Polymicrogyria –– LA05.51 Cortical dysplasia –– LA05.5Y Other specified abnormal neuronal migration –– LA05.5Z Abnormal neuronal migration, unspecified –– LA05.60 Porencephaly –– LA05.61 Schizencephaly –– LA05.62 Hydranencephaly –– LA05.6Y Other specified encephaloclastic disorders –– LA05.6Z Encephaloclastic disorders, unspecified LA06 Cerebellar structural developmental anomalies – LA06.0 Dandy-Walker malformation – LA06.1 Hypoplasia or agenesis of cerebellar hemispheres – LA06.2 Focal cerebellar dysplasia – LA06.Y Other specified cerebellar structural developmental anomalies – LA06.Z Cerebellar structural developmental anomalies, unspecified LA07 Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column – LA07.0 Primary tethered cord syndrome – LA07.1 Diastematomyelia – LA07.2 Amyelia – LA07.3 Primary syringomyelia or hydromyelia – LA07.4 Arnold-Chiari malformation type I – LA07.Y Other specified structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column – LA07.Z Structural developmental anomalies of the neurenteric canal, spinal cord or vertebral column, unspecified LA0Y Other specified structural developmental anomalies of the nervous system LA0Z Structural developmental anomalies of the nervous system, unspecified LA10 Structural developmental anomalies of ocular globes – LA10.0 Microphthalmos – LA10.1 Clinical anophthalmos – LA10.2 Buphthalmos – LA10.3 Congenital macrophthalmos – LA10.Y Other specified structural developmental anomalies of ocular globes – LA10.Z Structural developmental anomalies of ocular globes, unspecified LA11 Structural developmental anomalies of the anterior segment of eye – LA11.0 Blue sclera – LA11.1 Structural developmental anomalies of cornea – LA11.2 Anterior segment dysgenesis – LA11.3 Aniridia – LA11.4 Coloboma of iris – LA11.5 Congenital corneal opacity – LA11.6 Structural disorders of the pupil – LA11.Y Other specified structural developmental anomalies of the anterior segment of eye – LA11.Z Structural developmental anomalies of the anterior segment of eye, unspecified –– LA11.60 Irregular pupil of the eye –– LA11.61 Iridoschisis –– LA11.62 Anomalies of pupillary function –– LA11.6Y Other specified structural disorders of the pupil –– LA11.6Z Structural disorders of the pupil, unspecified LA12 Structural developmental anomalies of lens or zonula – LA12.0 Coloboma of lens – LA12.1 Congenital cataract – LA12.2 Congenital aphakia – LA12.3 Spherophakia – LA12.Y Other specified structural developmental anomalies of lens or zonula – LA12.Z Structural developmental anomalies of lens or zonula, unspecified LA13 Structural developmental anomalies of the posterior segment of eye – LA13.0 Congenital anomalies of the vitreous – LA13.1 Coloboma of choroid or retina – LA13.2 Coloboma of macula – LA13.3 Congenital vitreoretinal dysplasia – LA13.5 Congenital retinal aneurysm – LA13.6 Congenital malformations of choroid – LA13.7 Congenital malformation of optic disc – LA13.8 Certain congenital malformations of posterior segment of eye – LA13.Y Other specified structural developmental anomalies of the posterior segment of eye – LA13.Z Structural developmental anomalies of the posterior segment of eye, unspecified –– LA13.70 Isolated optic nerve hypoplasia –– LA13.71 Optic nerve aplasia –– LA13.72 Congenitally elevated optic disc –– LA13.73 Optic disc dysplasia –– LA13.74 Megalopapilla –– LA13.76 Coloboma of optic disc –– LA13.7Y Other specified congenital malformation of optic disc –– LA13.7Z Congenital malformation of optic disc, unspecified –– LA13.80 Anastomosis of retinal or choroidal vessels LA14 Structural developmental anomalies of eyelid, lacrimal apparatus or orbit – LA14.0 Structural developmental anomalies of eyelids – LA14.1 Structural developmental anomalies of lacrimal apparatus – LA14.2 Structural developmental anomalies of orbit – LA14.Y Other specified structural developmental anomalies of eyelid, lacrimal apparatus or orbit – LA14.Z Structural developmental anomalies of eyelid, lacrimal apparatus or orbit, unspecified –– LA14.00 Palpebral cleft or coloboma –– LA14.01 Cryptophthalmia –– LA14.02 Congenital entropion –– LA14.03 Congenital ectropion –– LA14.04 Congenital ptosis –– LA14.05 Congenital eyelid retraction –– LA14.06 Epibulbar choristoma –– LA14.07 Ankyloblepharon filiforme adnatum –– LA14.0Y Other specified structural developmental anomalies of eyelids –– LA14.10 Aplasia of lacrimal or salivary glands –– LA14.11 Agenesis of lacrimal ducts –– LA14.12 Congenital dacryocele –– LA14.13 Congenital agenesis of lacrimal punctum –– LA14.14 Congenital stenosis or stricture of lacrimal duct –– LA14.1Y Other specified structural developmental anomalies of lacrimal apparatus –– LA14.1Z Structural developmental anomalies of lacrimal apparatus, unspecified LA1Y Other specified structural developmental anomalies of the eye, eyelid or lacrimal apparatus LA1Z Structural developmental anomalies of the eye, eyelid or lacrimal apparatus, unspecified LA20 Structural anomaly of eustachian apparatus LA21 Minor anomalies of pinnae – LA21.0 Macrotia – LA21.1 Protruding ear – LA21.2 Low-set ear – LA21.3 Misshapen ear – LA21.Y Other specified minor anomalies of pinnae – LA21.Z Minor anomalies of pinnae, unspecified LA22 Structural developmental anomalies of ear causing hearing impairment – LA22.0 Microtia – LA22.1 Anotia – LA22.2 Aplasia or hypoplasia of external auditory canal – LA22.3 Structural developmental anomalies of ear ossicles – LA22.4 Structural developmental anomalies of inner ear – LA22.Y Other specified structural developmental anomalies of ear causing hearing impairment – LA22.Z Structural developmental anomalies of ear causing hearing impairment, unspecified LA23 Otocephaly LA24 Accessory auricle LA2Y Other specified structural developmental anomalies of the ear LA2Z Structural developmental anomalies of the ear, unspecified LA30 Structural developmental anomalies of teeth and periodontal tissues – LA30.0 Anodontia – LA30.1 Hypodontia – LA30.2 Oligodontia – LA30.3 Hyperdontia – LA30.4 Abnormalities of size or form of teeth – LA30.5 Anomalies in tooth resorption or loss – LA30.6 Amelogenesis imperfecta – LA30.7 Dentine dysplasia – LA30.8 Dentinogenesis imperfecta – LA30.9 Odontogenesis imperfecta – LA30.Y Other specified structural developmental anomalies of teeth and periodontal tissues – LA30.Z Structural developmental anomalies of teeth and periodontal tissues, unspecified –– LA30.50 Early exfoliation of teeth –– LA30.51 Late exfoliation of teeth –– LA30.5Y Other specified anomalies in tooth resorption or loss –– LA30.5Z Anomalies in tooth resorption or loss, unspecified LA31 Structural developmental anomalies of mouth or tongue – LA31.0 Congenital macroglossia – LA31.1 Hypoglossia or aglossia – LA31.2 Ankyloglossia – LA31.3 Macrostomia – LA31.4 Microstomia – LA31.Y Other specified structural developmental anomalies of mouth or tongue – LA31.Z Structural developmental anomalies of mouth or tongue, unspecified – LA40 Cleft lip –– LA40.0 Cleft lip, unilateral –– LA40.1 Cleft lip, bilateral –– LA40.2 Cleft lip, median –– LA40.Z Cleft lip, unspecified – LA41 Cleft lip and alveolus –– LA41.0 Cleft lip and alveolus, unilateral –– LA41.1 Cleft lip and alveolus, bilateral –– LA41.Z Cleft lip and alveolus, unspecified – LA42 Cleft palate –– LA42.0 Cleft hard palate –– LA42.1 Cleft soft palate –– LA42.2 Cleft uvula –– LA42.Z Cleft palate, unspecified – LA4Y Other specified clefts of lip, alveolus or palate – LA4Z Clefts of lip, alveolus or palate, unspecified LA50 Congenital velopharyngeal incompetence LA51 Facial clefts LA52 Facial asymmetry LA53 Macrocheilia LA54 Microcheilia LA55 Compression facies LA56 Pierre Robin syndrome LA5Y Other specified structural developmental anomalies of the face LA5Z Structural developmental anomalies of the face, unspecified LA60 Webbed neck LA61 Congenital sternomastoid tumour LA62 Congenital torticollis LA6Y Other specified structural developmental anomalies of the neck LA6Z Structural developmental anomalies of the neck, unspecified LA70 Structural developmental anomalies of the nose or cavum – LA70.0 Arrhinia – LA70.1 Bifid nose – LA70.2 Choanal atresia – LA70.3 Congenital perforated nasal septum – LA70.Y Other specified structural developmental anomalies of the nose or cavum – LA70.Z Structural developmental anomalies of the nose or cavum, unspecified LA71 Structural developmental anomalies of larynx – LA71.0 Congenital laryngomalacia – LA71.1 Laryngocele – LA71.2 Laryngeal hypoplasia – LA71.3 Congenital subglottic stenosis – LA71.Y Other specified structural developmental anomalies of larynx – LA71.Z Structural developmental anomalies of larynx, unspecified LA72 Laryngotracheooesophageal cleft LA73 Structural developmental anomalies of trachea – LA73.0 Congenital stenosis of trachea – LA73.1 Congenital tracheomalacia – LA73.Y Other specified structural developmental anomalies of trachea – LA73.Z Structural developmental anomalies of trachea, unspecified LA74 Structural developmental anomalies of bronchi – LA74.0 Congenital stenosis or atresia of bronchus – LA74.1 Congenital bronchomalacia – LA74.Y Other specified structural developmental anomalies of bronchi – LA74.Z Structural developmental anomalies of bronchi, unspecified LA75 Structural developmental anomalies of lungs – LA75.0 Accessory lobe of lung – LA75.1 Agenesis of lung – LA75.2 Congenital hypoplasia of lung – LA75.3 Congenital hyperplasia of lung – LA75.4 Congenital pulmonary airway malformations – LA75.5 Congenital lobar emphysema – LA75.6 Congenital sequestration of lung – LA75.Y Other specified structural developmental anomalies of lungs – LA75.Z Structural developmental anomalies of lungs, unspecified LA76 Structural developmental anomalies of pleura LA77 Congenital cyst of mediastinum LA7Y Other specified structural developmental anomalies of the respiratory system LA7Z Structural developmental anomalies of the respiratory system, unspecified – LA80 Anomalous position-orientation of heart –– LA80.0 Laevocardia –– LA80.1 Dextrocardia –– LA80.2 Mesocardia –– LA80.3 Extrathoracic heart –– LA80.Y Other specified anomalous position-orientation of heart –– LA80.Z Anomalous position-orientation of heart, unspecified – LA81 Abnormal ventricular relationships – LA82 Total mirror imagery – LA83 Right isomerism – LA84 Left isomerism – LA85 Congenital anomaly of an atrioventricular or ventriculo-arterial connection –– LA85.0 Discordant atrioventricular connections –– LA85.1 Transposition of the great arteries –– LA85.2 Double outlet right ventricle –– LA85.3 Double outlet left ventricle –– LA85.4 Common arterial trunk –– LA85.Y Other specified congenital anomaly of an atrioventricular or ventriculo-arterial connection –– LA85.Z Congenital anomaly of an atrioventricular or ventriculo-arterial connection, unspecified ––– LA85.00 Congenitally corrected transposition of great arteries ––– LA85.0Y Other specified discordant atrioventricular connections ––– LA85.0Z Discordant atrioventricular connections, unspecified ––– LA85.20 Double outlet right ventricle with subpulmonary ventricular septal defect, transposition type ––– LA85.21 Double outlet right ventricle with non-committed ventricular septal defect ––– LA85.22 Double outlet right ventricle with subaortic or doubly committed ventricular septal defect without pulmonary stenosis, ventricular septal defect type ––– LA85.23 Double outlet right ventricle with subaortic or doubly committed ventricular septal defect and pulmonary stenosis, Fallot type ––– LA85.2Y Other specified double outlet right ventricle ––– LA85.2Z Double outlet right ventricle, unspecified ––– LA85.40 Common arterial trunk with aortic dominance ––– LA85.41 Common arterial trunk with pulmonary dominance and interrupted aortic arch ––– LA85.4Y Other specified common arterial trunk ––– LA85.4Z Common arterial trunk, unspecified – LA86 Congenital anomaly of mediastinal vein –– LA86.0 Left superior caval vein –– LA86.1 Unroofed coronary sinus –– LA86.2 Anomalous pulmonary venous connection –– LA86.3 Congenital pulmonary venous stenosis or hypoplasia –– LA86.Y Other specified congenital anomaly of mediastinal vein –– LA86.Z Congenital anomaly of mediastinal vein, unspecified ––– LA86.20 Total anomalous pulmonary venous connection ––– LA86.21 Partial anomalous pulmonary venous connection ––– LA86.22 Scimitar syndrome ––– LA86.2Y Other specified anomalous pulmonary venous connection ––– LA86.2Z Anomalous pulmonary venous connection, unspecified – LA87 Congenital anomaly of an atrioventricular valve or atrioventricular septum –– LA87.0 Congenital anomaly of tricuspid valve –– LA87.1 Congenital anomaly of mitral valve –– LA87.3 Common atrioventricular junction without an atrioventricular septal defect –– LA87.4 Common atrioventricular junction with atrioventricular septal defect –– LA87.Y Other specified congenital anomaly of an atrioventricular valve or atrioventricular septum –– LA87.Z Congenital anomaly of an atrioventricular valve or atrioventricular septum, unspecified ––– LA87.00 Congenital tricuspid regurgitation ––– LA87.01 Congenital tricuspid valvar stenosis ––– LA87.02 Dysplasia of tricuspid valve ––– LA87.03 Ebstein malformation of tricuspid valve ––– LA87.0Y Other specified congenital anomaly of tricuspid valve ––– LA87.0Z Congenital anomaly of tricuspid valve, unspecified ––– LA87.10 Congenital mitral regurgitation ––– LA87.11 Congenital mitral valvar stenosis ––– LA87.12 Dysplasia of mitral valve ––– LA87.13 Congenital anomaly of mitral subvalvar apparatus ––– LA87.1Y Other specified congenital anomaly of mitral valve ––– LA87.1Z Congenital anomaly of mitral valve, unspecified ––– LA87.40 Atrioventricular septal defect with communication at the atrial level only ––– LA87.41 Atrioventricular septal defect with communication at the ventricular level only ––– LA87.42 Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level ––– LA87.43 Atrioventricular septal defect with communication at atrial level and unrestrictive communication at ventricular level ––– LA87.44 Atrioventricular septal defect with ventricular imbalance ––– LA87.45 Atrioventricular septal defect and tetralogy of Fallot ––– LA87.4Y Other specified common atrioventricular junction with atrioventricular septal defect ––– LA87.4Z Common atrioventricular junction with atrioventricular septal defect, unspecified – LA88 Congenital anomaly of a ventricle or the ventricular septum –– LA88.0 Congenital right ventricular outflow tract obstruction –– LA88.1 Double chambered right ventricle –– LA88.2 Tetralogy of Fallot –– LA88.3 Congenital left ventricular outflow tract obstruction –– LA88.4 Ventricular septal defect –– LA88.Y Other specified congenital anomaly of a ventricle or the ventricular septum –– LA88.Z Congenital anomaly of a ventricle or the ventricular septum, unspecified ––– LA88.20 Tetralogy of Fallot with absent pulmonary valve syndrome ––– LA88.21 Tetralogy of Fallot with pulmonary atresia ––– LA88.22 Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral artery ––– LA88.2Y Other specified tetralogy of Fallot ––– LA88.2Z Tetralogy of Fallot, unspecified ––– LA88.40 Trabecular muscular ventricular septal defect ––– LA88.41 Perimembranous central ventricular septal defect ––– LA88.42 Ventricular septal defect haemodynamically insignificant ––– LA88.4Y Other specified ventricular septal defect ––– LA88.4Z Ventricular septal defect, unspecified – LA89 Functionally univentricular heart –– LA89.0 Double inlet atrioventricular connection –– LA89.1 Tricuspid atresia –– LA89.2 Mitral atresia –– LA89.3 Hypoplastic left heart syndrome –– LA89.Y Other specified functionally univentricular heart –– LA89.Z Functionally univentricular heart, unspecified – LA8A Congenital anomaly of a ventriculo-arterial valve or adjacent regions –– LA8A.0 Congenital anomaly of pulmonary valve –– LA8A.1 Congenital pulmonary atresia –– LA8A.2 Congenital anomaly of aortic valve –– LA8A.3 Congenital supravalvar aortic stenosis –– LA8A.4 Aneurysm of aortic sinus of Valsalva –– LA8A.5 Congenital subaortic stenosis –– LA8A.6 Congenital subpulmonary stenosis –– LA8A.Y Other specified congenital anomaly of a ventriculo-arterial valve or adjacent regions –– LA8A.Z Congenital anomaly of a ventriculo-arterial valve or adjacent regions, unspecified ––– LA8A.00 Congenital pulmonary valvar stenosis ––– LA8A.01 Congenital pulmonary regurgitation ––– LA8A.0Y Other specified congenital anomaly of pulmonary valve ––– LA8A.0Z Congenital anomaly of pulmonary valve, unspecified ––– LA8A.10 Pulmonary atresia with intact ventricular septum ––– LA8A.1Y Other specified congenital pulmonary atresia ––– LA8A.1Z Congenital pulmonary atresia, unspecified ––– LA8A.20 Congenital aortic valvar stenosis ––– LA8A.21 Congenital aortic regurgitation ––– LA8A.22 Bicuspid aortic valve ––– LA8A.23 Aortic valvar atresia ––– LA8A.24 Unicuspid aortic valve ––– LA8A.2Y Other specified congenital anomaly of aortic valve ––– LA8A.2Z Congenital anomaly of aortic valve, unspecified – LA8B Congenital anomaly of great arteries including arterial duct –– LA8B.0 Congenital aortopulmonary window –– LA8B.1 Congenital anomaly of pulmonary arterial tree –– LA8B.2 Congenital anomaly of aorta or its branches –– LA8B.3 Tracheo-oesophageal compressive syndrome –– LA8B.4 Patent arterial duct –– LA8B.Y Other specified congenital anomaly of great arteries including arterial duct –– LA8B.Z Congenital anomaly of great arteries including arterial duct, unspecified ––– LA8B.21 Coarctation of aorta ––– LA8B.22 Interrupted aortic arch ––– LA8B.23 Congenital anomaly of descending thoracic or abdominal aorta ––– LA8B.24 Congenital anomaly of aortic arch branch ––– LA8B.2Y Other specified congenital anomaly of aorta or its branches ––– LA8B.2Z Congenital anomaly of aorta or its branches, unspecified – LA8C Congenital anomaly of coronary artery –– LA8C.0 Anomalous origin of coronary artery from pulmonary arterial tree –– LA8C.1 Anomalous aortic origin or course of coronary artery –– LA8C.2 Congenital coronary arterial fistula –– LA8C.Y Other specified congenital anomaly of coronary artery –– LA8C.Z Congenital anomaly of coronary artery, unspecified – LA8D Congenital pericardial anomaly – LA8E Congenital anomaly of atrial septum –– LA8E.0 Patent oval foramen –– LA8E.1 Atrial septal defect within oval fossa –– LA8E.2 Sinus venosus defect –– LA8E.3 Interatrial communication through coronary sinus orifice –– LA8E.Y Other specified congenital anomaly of atrial septum –– LA8E.Z Congenital anomaly of atrial septum, unspecified – LA8F Congenital anomaly of right atrium – LA8G Congenital anomaly of left atrium –– LA8G.0 Divided left atrium –– LA8G.Y Other specified congenital anomaly of left atrium –– LA8G.Z Congenital anomaly of left atrium, unspecified – LA8Y Other specified structural developmental anomaly of heart or great vessels – LA8Z Structural developmental anomaly of heart or great vessels, unspecified LA90 Structural developmental anomalies of the peripheral vascular system – LA90.0 Capillary malformations – LA90.1 Lymphatic malformations – LA90.2 Peripheral venous malformations – LA90.3 Peripheral arteriovenous malformations – LA90.4 Peripheral arterial malformations – LA90.5 Pulmonary arteriovenous fistula – LA90.Y Other specified structural developmental anomalies of the peripheral vascular system – LA90.Z Structural developmental anomalies of the peripheral vascular system, unspecified –– LA90.00 Hereditary haemorrhagic telangiectasia –– LA90.0Y Other specified capillary malformations –– LA90.0Z Capillary malformations, unspecified –– LA90.10 Macrocystic lymphatic malformation –– LA90.11 Microcystic lymphatic malformation –– LA90.12 Lymphatic malformations of certain specified sites –– LA90.13 Cystic hygroma in fetus –– LA90.1Y Other specified lymphatic malformations –– LA90.1Z Lymphatic malformations, unspecified –– LA90.20 Vein of Galen aneurysm –– LA90.21 Congenital portosystemic shunt –– LA90.2Y Other specified peripheral venous malformations –– LA90.2Z Peripheral venous malformations, unspecified –– LA90.30 Portal vein-hepatic artery fistula –– LA90.31 Arteriovenous malformation of precerebral vessels –– LA90.32 Uterine arteriovenous malformations –– LA90.3Y Other specified peripheral arteriovenous malformations –– LA90.3Z Peripheral arteriovenous malformations, unspecified –– LA90.40 Congenital renal artery stenosis –– LA90.41 Congenital precerebral nonruptured aneurysm –– LA90.42 Congenital cerebral nonruptured aneurysm –– LA90.4Y Other specified peripheral arterial malformations –– LA90.4Z Peripheral arterial malformations, unspecified LA9Y Other specified structural developmental anomalies of the circulatory system LA9Z Structural developmental anomalies of the circulatory system, unspecified LB00 Structural developmental anomalies of diaphragm – LB00.0 Congenital diaphragmatic hernia – LB00.1 Absence of diaphragm – LB00.Y Other specified structural developmental anomalies of diaphragm – LB00.Z Structural developmental anomalies of diaphragm, unspecified LB01 Omphalocele LB02 Gastroschisis LB03 Structural developmental anomalies of umbilical cord – LB03.0 Allantoic duct remnants or cysts – LB03.1 Single umbilical cord artery – LB03.Y Other specified structural developmental anomalies of umbilical cord – LB03.Z Structural developmental anomalies of umbilical cord, unspecified LB0Y Other specified structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord LB0Z Structural developmental anomalies of the diaphragm, abdominal wall or umbilical cord, unspecified LB10 Structural developmental anomalies of salivary glands or ducts LB11 Congenital diverticulum of pharynx LB12 Structural developmental anomalies of oesophagus – LB12.0 Congenital oesophageal web or ring – LB12.1 Atresia of oesophagus – LB12.2 Oesophageal fistula without atresia – LB12.3 Congenital stenosis or stricture of oesophagus – LB12.4 Congenital diverticulum of oesophagus – LB12.5 Congenital dilatation of oesophagus – LB12.Y Other specified structural developmental anomalies of oesophagus – LB12.Z Structural developmental anomalies of oesophagus, unspecified –– LB12.10 Atresia of oesophagus with oesophagobronchial fistula –– LB12.1Y Other specified atresia of oesophagus –– LB12.1Z Atresia of oesophagus, unspecified LB13 Structural developmental anomalies of stomach – LB13.0 Congenital hypertrophic pyloric stenosis – LB13.1 Congenital hiatus hernia – LB13.2 Congenital antral web – LB13.Y Other specified structural developmental anomalies of stomach – LB13.Z Structural developmental anomalies of stomach, unspecified LB14 Structural developmental anomalies of duodenum LB15 Structural developmental anomalies of small intestine – LB15.0 Meckel diverticulum – LB15.1 Atresia of small intestine – LB15.2 Congenital short bowel – LB15.3 Congenital diverticulitis of small intestine – LB15.4 Congenital diverticulosis of small intestine – LB15.5 Congenital diverticulum of small intestine – LB15.Y Other specified structural developmental anomalies of small intestine – LB15.Z Structural developmental anomalies of small intestine, unspecified LB16 Structural developmental anomalies of large intestine – LB16.0 Congenital absence, atresia or stenosis of large intestine – LB16.1 Hirschsprung disease – LB16.2 Immature ganglionosis of large intestine – LB16.3 Congenital hypoganglionosis of large intestine – LB16.Y Other specified structural developmental anomalies of large intestine – LB16.Z Structural developmental anomalies of large intestine, unspecified LB17 Structural developmental anomalies of anal canal – LB17.0 Anorectal malformations – LB17.1 Ectopic anus – LB17.2 Persistent cloaca – LB17.3 Cloacal exstrophy – LB17.4 Perineal groove – LB17.Y Other specified structural developmental anomalies of anal canal – LB17.Z Structural developmental anomalies of anal canal, unspecified LB18 Congenital anomalies of intestinal fixation LB1Y Other specified structural developmental anomalies of the digestive tract LB1Z Structural developmental anomalies of the digestive tract, unspecified LB20 Structural developmental anomalies of gallbladder, bile ducts or liver – LB20.0 Structural developmental anomalies of liver – LB20.1 Structural developmental anomalies of gallbladder – LB20.2 Structural developmental anomalies of bile ducts – LB20.Y Other specified structural developmental anomalies of gallbladder, bile ducts or liver – LB20.Z Structural developmental anomalies of gallbladder, bile ducts or liver, unspecified –– LB20.00 Fibropolycystic liver disease –– LB20.0Y Other specified structural developmental anomalies of liver –– LB20.0Z Structural developmental anomalies of liver, unspecified –– LB20.10 Agenesis, aplasia or hypoplasia of gallbladder –– LB20.1Y Other specified structural developmental anomalies of gallbladder –– LB20.1Z Structural developmental anomalies of gallbladder, unspecified –– LB20.20 Choledochal cyst –– LB20.21 Biliary atresia –– LB20.22 Congenital stenosis or stricture of bile ducts –– LB20.23 Structural developmental anomalies of cystic duct –– LB20.24 Accessory bile duct –– LB20.2Y Other specified structural developmental anomalies of bile ducts –– LB20.2Z Structural developmental anomalies of bile ducts, unspecified LB21 Structural developmental anomalies of pancreas – LB21.0 Annular pancreas – LB21.1 Pancreas divisum – LB21.2 Accessory pancreas – LB21.3 Agenesis-aplasia of pancreas – LB21.4 Partial agenesis of pancreas – LB21.5 Hypoplasia of pancreas – LB21.Y Other specified structural developmental anomalies of pancreas – LB21.Z Structural developmental anomalies of pancreas, unspecified LB22 Structural developmental anomalies of spleen – LB22.0 Congenital asplenia – LB22.1 Polysplenia – LB22.2 Ectopic spleen – LB22.Y Other specified structural developmental anomalies of spleen – LB22.Z Structural developmental anomalies of spleen, unspecified LB2Y Other specified structural developmental anomalies of the liver, biliary tract, pancreas or spleen LB2Z Structural developmental anomalies of the liver, biliary tract, pancreas or spleen, unspecified LB30 Structural developmental anomalies of kidneys – LB30.0 Renal agenesis or other reduction defects of kidney – LB30.1 Renal dysplasia – LB30.2 Congenital single renal cyst – LB30.3 Renal tubular dysgenesis – LB30.4 Oligomeganephronia – LB30.5 Accessory kidney – LB30.6 Fusion anomaly of kidneys – LB30.7 Ectopic or pelvic kidney – LB30.8 Medullary sponge kidney – LB30.9 Multicystic renal dysplasia – LB30.Y Other specified structural developmental anomalies of kidneys – LB30.Z Structural developmental anomalies of kidneys, unspecified –– LB30.00 Renal agenesis –– LB30.0Y Other specified renal agenesis or other reduction defects of kidney –– LB30.0Z Renal agenesis or other reduction defects of kidney, unspecified –– LB30.60 Lobulated kidney –– LB30.61 Fused pelvic kidney –– LB30.62 Horseshoe kidney –– LB30.6Y Other specified fusion anomaly of kidneys –– LB30.6Z Fusion anomaly of kidneys, unspecified LB31 Structural developmental anomalies of urinary tract – LB31.0 Congenital hydronephrosis – LB31.1 Congenital primary megaureter – LB31.2 Fetal lower urinary tract obstruction – LB31.3 Exstrophy of urinary bladder – LB31.4 Congenital diverticulum of urinary bladder – LB31.5 Duplication of urethra – LB31.6 Congenital megalourethra – LB31.7 Megacystis-megaureter – LB31.8 Atresia or stenosis of ureter – LB31.9 Agenesis of ureter – LB31.A Duplication of ureter – LB31.B Malposition of ureter – LB31.C Congenital absence of bladder or urethra – LB31.D Congenital vesico-uretero-renal reflux – LB31.Y Other specified structural developmental anomalies of urinary tract – LB31.Z Structural developmental anomalies of urinary tract, unspecified LB3Y Other specified structural developmental anomalies of the urinary system LB3Z Structural developmental anomalies of the urinary system, unspecified LB40 Structural developmental anomalies of vulva – LB40.0 Absence of vulva – LB40.1 Embryonic cyst of vulva – LB40.2 Fusion of labia – LB40.Y Other specified structural developmental anomalies of vulva – LB40.Z Structural developmental anomalies of vulva, unspecified LB41 Structural developmental anomalies of clitoris – LB41.0 Agenesis of clitoris – LB41.1 Duplication of clitoris – LB41.2 Clitoromegaly – LB41.Y Other specified structural developmental anomalies of clitoris – LB41.Z Structural developmental anomalies of clitoris, unspecified LB42 Structural developmental anomalies of vagina – LB42.0 Absence of vagina – LB42.1 Septate vagina – LB42.2 Congenital rectovaginal fistula – LB42.3 Tight hymenal ring – LB42.4 Imperforate hymen – LB42.5 Stricture or atresia of vagina – LB42.Y Other specified structural developmental anomalies of vagina – LB42.Z Structural developmental anomalies of vagina, unspecified LB43 Structural developmental anomalies of cervix uteri – LB43.0 Embryonic cyst of cervix – LB43.1 Agenesis or aplasia of cervix – LB43.Y Other specified structural developmental anomalies of cervix uteri – LB43.Z Structural developmental anomalies of cervix uteri, unspecified LB44 Structural developmental anomalies of uterus, except cervix – LB44.0 Agenesis or aplasia of uterine body – LB44.1 Hypoplasia of uterus – LB44.2 Unicornuate uterus – LB44.3 Bicornuate uterus – LB44.4 Septate uterus – LB44.5 Congenital fistulae between uterus and digestive and urinary tracts – LB44.6 Uterovaginal malformation due to diethylstilbestrol syndrome – LB44.Y Other specified structural developmental anomalies of uterus, except cervix – LB44.Z Structural developmental anomalies of uterus, except cervix, unspecified LB45 Structural developmental anomalies of ovaries, fallopian tubes or broad ligaments – LB45.0 Congenital absence of ovary – LB45.1 46,XX gonadal dysgenesis – LB45.2 Developmental ovarian cyst – LB45.3 Congenital torsion of ovary – LB45.4 Accessory ovary – LB45.5 Congenital absence of fallopian tube – LB45.6 Atresia of fallopian tube – LB45.7 Accessory fallopian tube – LB45.8 Embryonic cyst of fallopian tube – LB45.9 Embryonic cyst of broad ligament – LB45.Y Other specified structural developmental anomalies of ovaries, fallopian tubes or broad ligaments – LB45.Z Structural developmental anomalies of ovaries, fallopian tubes or broad ligaments, unspecified LB4Y Other specified structural developmental anomalies of the female genital system LB4Z Structural developmental anomalies of the female genital system, unspecified LB50 Micropenis or penis agenesis LB51 Anorchia or microorchidia LB52 Cryptorchidism – LB52.0 Ectopic testis – LB52.1 Undescended testicle, unilateral – LB52.2 Undescended testicle, bilateral – LB52.Y Other specified cryptorchidism – LB52.Z Cryptorchidism, unspecified LB53 Hypospadias – LB53.0 Hypospadias, balanic – LB53.1 Hypospadias, penile – LB53.2 Hypospadias, penoscrotal – LB53.3 Hypospadias, scrotal – LB53.4 Hypospadias, perineal – LB53.Y Other specified hypospadias – LB53.Z Hypospadias, unspecified –– LB53.00 Hypospadias, coronal –– LB53.01 Hypospadias, glandular –– LB53.0Y Other specified hypospadias, balanic –– LB53.0Z Hypospadias, balanic, unspecified LB54 Congenital chordee LB55 Epispadias LB56 Bifid scrotum LB57 Agenesis of vas deferens LB58 Polyorchidism LB59 Hypoplasia of testis or scrotum LB5Y Other specified structural developmental anomalies of the male genital system LB5Z Structural developmental anomalies of the male genital system, unspecified LB60 Breast aplasia LB61 Absent nipple LB62 Supernumerary breasts LB63 Accessory nipple LB6Y Other specified structural developmental anomalies of the breast LB6Z Structural developmental anomalies of the breast, unspecified LB70 Structural developmental anomalies of cranium – LB70.0 Craniosynostosis – LB70.1 Wormian bones – LB70.2 J-shaped sella turcica – LB70.3 Macrocephaly – LB70.Y Other specified structural developmental anomalies of cranium – LB70.Z Structural developmental anomalies of cranium, unspecified –– LB70.00 Plagiocephaly –– LB70.0Y Other specified craniosynostosis –– LB70.0Z Craniosynostosis, unspecified LB71 Structural developmental anomalies of facial bones – LB71.0 Hypotelorism – LB71.1 Hypertelorism – LB71.Y Other specified structural developmental anomalies of facial bones – LB71.Z Structural developmental anomalies of facial bones, unspecified LB72 Structural developmental anomalies of shoulder girdle – LB72.0 Cervical rib – LB72.1 Sprengel deformity – LB72.2 Deformation of scapula – LB72.Y Other specified structural developmental anomalies of shoulder girdle – LB72.Z Structural developmental anomalies of shoulder girdle, unspecified LB73 Structural developmental anomalies of spine or bony thorax – LB73.0 Occult spinal dysraphism – LB73.1 Structural developmental anomalies of chest wall – LB73.2 Structural developmental anomalies of spine – LB73.Y Other specified structural developmental anomalies of spine or bony thorax – LB73.Z Structural developmental anomalies of spine or bony thorax, unspecified –– LB73.10 Poland syndrome –– LB73.11 Bifid rib –– LB73.12 Accessory rib –– LB73.13 Structural developmental anomalies of sternum –– LB73.1Y Other specified structural developmental anomalies of chest wall –– LB73.1Z Structural developmental anomalies of chest wall, unspecified –– LB73.20 Klippel-Feil anomaly –– LB73.21 Occipitalisation of atlas –– LB73.22 Atlanto-axial instability or subluxation –– LB73.23 Aplasia or hypoplasia of the odontoid process of axis –– LB73.24 Segmentation anomalies of vertebrae –– LB73.25 Congenital scoliosis due to congenital bony malformation –– LB73.26 Sacralisation of the last lumbar vertebra –– LB73.27 Lumbarisation of the first sacral vertebra –– LB73.28 Sacrum agenesis or hypoplasia –– LB73.29 Caudal appendage –– LB73.2A Congenital spondylolisthesis –– LB73.2Y Other specified structural developmental anomalies of spine –– LB73.2Z Structural developmental anomalies of spine, unspecified LB74 Structural developmental anomalies of pelvic girdle – LB74.0 Developmental dysplasia of hip – LB74.1 Congenital subluxation of hip – LB74.2 Unstable hip – LB74.3 Congenital coxa vara – LB74.4 Congenital coxa valga – LB74.5 Wide symphysis pubis – LB74.Y Other specified structural developmental anomalies of pelvic girdle – LB74.Z Structural developmental anomalies of pelvic girdle, unspecified LB75 Brachydactyly – LB75.0 Brachydactyly of fingers – LB75.1 Brachydactyly of toes – LB75.2 Symbrachydactyly of hands or feet – LB75.Y Other specified brachydactyly – LB75.Z Brachydactyly, unspecified LB76 Triphalangeal thumb LB77 Hyperphalangy LB78 Polydactyly – LB78.0 Polydactyly of the thumb – LB78.1 Polysyndactyly – LB78.2 Postaxial polydactyly of fingers – LB78.3 Polydactyly of toes – LB78.Y Other specified polydactyly – LB78.Z Polydactyly, unspecified LB79 Syndactyly – LB79.0 Fused fingers – LB79.1 Webbed fingers – LB79.2 Fused toes – LB79.3 Webbed toes – LB79.Y Other specified syndactyly – LB79.Z Syndactyly, unspecified – LB80 Congenital deformities of fingers –– LB80.0 Clinodactyly of fingers –– LB80.2 Radial deviation of fingers –– LB80.Y Other specified congenital deformities of fingers –– LB80.Z Congenital deformities of fingers, unspecified – LB81 Congenital deformities of toes –– LB81.0 Clinodactyly of toes –– LB81.Y Other specified congenital deformities of toes –– LB81.Z Congenital deformities of toes, unspecified LB90 Joint formation defects – LB90.0 Humero-radio-ulnar synostosis – LB90.1 Humero-radial synostosis – LB90.2 Humero-ulnar synostosis – LB90.3 Radio-ulnar synostosis – LB90.4 Madelung deformity – LB90.5 Congenital digital clubbing – LB90.6 Tibio-fibular synostosis – LB90.7 Cubitus valgus – LB90.8 Cubitus varus – LB90.Y Other specified joint formation defects – LB90.Z Joint formation defects, unspecified LB91 Congenital shoulder dislocation LB92 Congenital elbow dislocation LB93 Congenital knee dislocation – LB93.0 Congenital genu recurvatum – LB93.1 Congenital genu flexum – LB93.Y Other specified congenital knee dislocation – LB93.Z Congenital knee dislocation, unspecified LB94 Congenital patella dislocation LB95 Patella aplasia or hypoplasia LB96 Congenital bowing of long bones – LB96.0 Congenital bowing of femur – LB96.1 Congenital bowing of tibia – LB96.Y Other specified congenital bowing of long bones – LB96.Z Congenital bowing of long bones, unspecified LB97 Limb overgrowth – LB97.0 Macrodactyly of fingers – LB97.1 Macrodactyly of toes – LB97.2 Upper limb hypertrophy – LB97.3 Lower limb hypertrophy – LB97.Y Other specified limb overgrowth – LB97.Z Limb overgrowth, unspecified LB98 Congenital deformities of feet – LB98.0 Congenital varus deformities of feet – LB98.1 Congenital pes planus – LB98.2 Congenital valgus deformities of feet – LB98.3 Congenital pes cavus – LB98.4 Congenital vertical talus – LB98.5 Congenital hammer toe – LB98.Y Other specified congenital deformities of feet – LB98.Z Congenital deformities of feet, unspecified –– LB98.00 Talipes equinovarus –– LB98.01 Talipes calcaneovarus –– LB98.02 Metatarsus varus –– LB98.0Y Other specified congenital varus deformities of feet –– LB98.0Z Congenital varus deformities of feet, unspecified –– LB98.20 Congenital hallux valgus –– LB98.21 Metatarsus valgus –– LB98.22 Talipes calcaneovalgus –– LB98.2Y Other specified congenital valgus deformities of feet –– LB98.2Z Congenital valgus deformities of feet, unspecified LB99 Reduction defects of upper limb – LB99.0 Amelia of upper limb – LB99.1 Humeral agenesis or hypoplasia – LB99.2 Radial hemimelia – LB99.3 Ulnar hemimelia – LB99.4 Congenital absence of upper arm or forearm with hand present – LB99.5 Congenital absence of both forearm and hand – LB99.6 Acheiria – LB99.7 Adactyly of hands – LB99.8 Split hand – LB99.Y Other specified reduction defects of upper limb – LB99.Z Reduction defects of upper limb, unspecified LB9A Reduction defects of lower limb – LB9A.0 Amelia of lower limb – LB9A.1 Tibial hemimelia – LB9A.2 Fibular hemimelia – LB9A.3 Congenital absence of thigh or lower leg with foot present – LB9A.4 Apodia – LB9A.5 Adactyly of feet – LB9A.6 Split foot – LB9A.7 Congenital absence of both lower leg and foot – LB9A.8 Femoral agenesis or hypoplasia – LB9A.Y Other specified reduction defects of lower limb – LB9A.Z Reduction defects of lower limb, unspecified LB9B Reduction defects of upper and lower limbs LB9Y Other specified structural developmental anomalies of the skeleton LB9Z Structural developmental anomalies of the skeleton, unspecified – LC00 Keratinocytic epidermal hamartoma –– LC00.0 Epidermal naevus –– LC00.Y Other specified keratinocytic epidermal hamartoma –– LC00.Z Keratinocytic epidermal hamartoma, unspecified – LC01 Pilosebaceous hamartoma – LC02 Complex epidermal hamartoma – LC0Y Other specified developmental hamartomata of the epidermis and epidermal appendages – LC10 Dermal melanocytosis – LC1Y Other specified developmental anomalies of skin pigmentation – LC20 Connective tissue hamartoma – LC2Y Other specified hamartomata derived from dermal connective tissue – LC30 Developmental defects of hair or hair growth – LC31 Developmental defects of the nail apparatus LC40 Dermoid cyst – LC50 Developmental capillary vascular malformations of the skin –– LC50.0 Salmon patch –– LC50.1 Port-wine stain –– LC50.Y Other specified cutaneous capillary vascular malformation – LC51 Developmental venous malformations involving the skin – LC52 Complex or combined developmental vascular malformations involving the skin – LC5Y Other specified developmental anomalies of cutaneous vasculature – LC5Z Developmental anomalies of cutaneous vasculature, unspecified – LC60 Aplasia cutis congenita LC7Y Other specified structural developmental anomalies of the skin LC7Z Structural developmental anomalies of the skin, unspecified LC80 Congenital adrenal hypoplasia LC8Y Other specified structural developmental anomalies of the adrenal glands LC8Z Structural developmental anomalies of the adrenal glands, unspecified LD0Y Other specified structural developmental anomalies primarily affecting one body system LD0Z Structural developmental anomalies primarily affecting one body system, unspecified LD20 Syndromes with central nervous system anomalies as a major feature LD20.0 Syndromes with cerebellar anomalies as a major feature LD20.1 Syndromes with lissencephaly as a major feature LD20.2 Syndromes with microcephaly as a major feature LD20.3 Syndromes with holoprosencephaly as a major feature LD20.4 Syndromes with brain calcifications as a major feature LD20.Y Other specified syndromes with central nervous system anomalies as a major feature LD20.Z Syndromes with central nervous system anomalies as a major feature, unspecified – LD20.00 Joubert syndrome – LD20.01 Pontocerebellar hypoplasia – LD20.0Y Other specified syndromes with cerebellar anomalies as a major feature – LD20.0Z Syndromes with cerebellar anomalies as a major feature, unspecified LD21 Syndromes with eye anomalies as a major feature LD21.0 Syndromes with microphthalmia as a major feature LD21.Y Other specified syndromes with eye anomalies as a major feature LD21.Z Syndromes with eye anomalies as a major feature, unspecified LD22 Syndromes with dental anomalies as a major feature LD23 Syndromes with vascular anomalies as a major feature LD24 Syndromes with skeletal anomalies as a major feature LD24.0 Syndromes with micromelia LD24.1 Bone diseases with increased bone density LD24.2 Bone diseases with disorganised development of skeletal components LD24.3 Spondyloepiphyseal or spondyloepimetaphyseal dysplasias LD24.4 Spondylometaphyseal dysplasias LD24.5 Spondylodysplastic dysplasias LD24.6 Multiple epiphyseal dysplasia or pseudoachondroplasia LD24.7 Multiple metaphyseal dysplasias LD24.8 Acromelic dysplasias LD24.9 Acromesomelic dysplasias LD24.A Mesomelic or rhizomesomelic dysplasias LD24.B Short rib syndromes LD24.C Bent bone dysplasias LD24.D Slender bone dysplasias LD24.E Bone dysplasias with multiple joint dislocations LD24.F Progressive ossification of skin, skeletal muscle, fascia, tendons or ligaments LD24.G Syndromic craniosynostoses LD24.H Dysostoses with predominant vertebral and costal involvement LD24.J Patellar dysostoses LD24.K Genetic bone diseases with decreased bone density LD24.Y Other specified syndromes with skeletal anomalies as a major feature LD24.Z Syndromes with skeletal anomalies as a major feature, unspecified – LD24.00 Achondroplasia – LD24.01 Hypochondroplasia – LD24.02 Thanatophoric dysplasia – LD24.03 Diastrophic dysplasia – LD24.04 Chondrodysplasia punctata – LD24.0Y Other specified syndromes with micromelia – LD24.0Z Syndromes with micromelia, unspecified – LD24.10 Osteopetrosis – LD24.11 Osteopoikilosis – LD24.1Y Other specified bone diseases with increased bone density – LD24.1Z Bone diseases with increased bone density, unspecified – LD24.20 Multiple osteochondromas – LD24.21 Exostoses with anetodermia and brachydactyly type E – LD24.22 Cherubism – LD24.23 Yunis-Varon disease – LD24.2Y Other specified bone diseases with disorganised development of skeletal components – LD24.2Z Bone diseases with disorganised development of skeletal components, unspecified – LD24.50 Achondrogenesis – LD24.51 Hypochondrogenesis – LD24.5Y Other specified spondylodysplastic dysplasias – LD24.5Z Spondylodysplastic dysplasias, unspecified – LD24.60 Pseudoachondroplasia – LD24.61 Multiple epiphyseal dysplasias – LD24.6Y Other specified multiple epiphyseal dysplasia or pseudoachondroplasia – LD24.6Z Multiple epiphyseal dysplasia or pseudoachondroplasia, unspecified – LD24.80 Langer-Giedion syndrome – LD24.8Y Other specified acromelic dysplasias – LD24.8Z Acromelic dysplasias, unspecified – LD24.B0 Short rib-polydactyly syndrome – LD24.B1 Asphyxiating thoracic dystrophy – LD24.BY Other specified short rib syndromes – LD24.BZ Short rib syndromes, unspecified – LD24.G0 Pfeiffer syndrome – LD24.G1 Crouzon disease – LD24.G2 Apert syndrome – LD24.GY Other specified syndromic craniosynostoses – LD24.GZ Syndromic craniosynostoses, unspecified – LD24.J0 Nail-patella syndrome – LD24.JY Other specified patellar dysostoses – LD24.JZ Patellar dysostoses, unspecified – LD24.K0 Osteogenesis imperfecta – LD24.KY Other specified genetic bone diseases with decreased bone density – LD24.KZ Genetic bone diseases with decreased bone density, unspecified LD25 Syndromes with face or limb anomalies as a major feature LD25.0 Oromandibular-limb anomaly syndrome LD25.1 Fronto-otopalatodigital syndromes LD25.2 Acrofacial dysostoses LD25.3 Craniofacial dysostoses LD25.Y Other specified syndromes with face or limb anomalies as a major feature LD25.Z Syndromes with face or limb anomalies as a major feature, unspecified – LD25.00 Oral-facial-digital syndrome – LD25.0Y Other specified oromandibular-limb anomaly syndrome – LD25.0Z Oromandibular-limb anomaly syndrome, unspecified LD26 Syndromes with limb anomalies as a major feature LD26.0 Combined reduction defects of upper and lower limbs LD26.1 Complex brachydactylies LD26.2 Syndromes with limb duplication, polydactyly, syndactyly or triphalangism LD26.3 Syndromes with synostoses of limbs LD26.4 Arthrogryposis syndromes LD26.5 Constriction rings LD26.6 Congenital vascular bone syndromes LD26.Y Other specified syndromes with limb anomalies as a major feature LD26.Z Syndromes with limb anomalies as a major feature, unspecified – LD26.40 Multiple pterygium syndrome – LD26.41 Arthrogryposis multiplex congenita – LD26.4Y Other specified arthrogryposis syndromes – LD26.4Z Arthrogryposis syndromes, unspecified – LD26.60 Angio-osteohypertrophic syndrome – LD26.6Y Other specified congenital vascular bone syndromes – LD26.6Z Congenital vascular bone syndromes, unspecified LD27 Syndromes with skin or mucosal anomalies as a major feature LD27.0 Ectodermal dysplasia syndromes LD27.1 Xeroderma pigmentosum LD27.2 Syndromic ichthyosis LD27.3 Genetic syndromes with hypertrichosis LD27.4 Genetic syndromes affecting nails LD27.5 Genetic hamartoneoplastic syndromes affecting the skin LD27.6 Genetic lipodystrophy LD27.Y Other specified syndromes with skin or mucosal anomalies as a major feature LD27.Z Syndromes with skin or mucosal anomalies as a major feature, unspecified – LD27.00 Incontinentia pigmenti – LD27.01 Cronkhite-Canada syndrome – LD27.02 Hypohidrotic ectodermal dysplasia – LD27.03 Hidrotic ectodermal dysplasia, Clouston type – LD27.0Y Other specified ectodermal dysplasia syndromes – LD27.60 Congenital generalised lipodystrophy – LD27.6Z Genetic lipodystrophy, unspecified LD28 Syndromes with connective tissue involvement as a major feature LD28.0 Marfan syndrome or Marfan-related disorders LD28.1 Ehlers-Danlos syndrome LD28.2 Genetically-determined cutis laxa LD28.Y Other specified syndromes with connective tissue involvement as a major feature LD28.Z Syndromes with connective tissue involvement as a major feature, unspecified – LD28.00 Congenital contractural arachnodactyly – LD28.01 Marfan syndrome – LD28.0Y Other specified Marfan syndrome or Marfan-related disorders – LD28.0Z Marfan syndrome or Marfan-related disorders, unspecified – LD28.10 Ehlers-Danlos syndrome, classical type – LD28.1Y Other specified types of Ehlers-Danlos syndrome LD29 Syndromes with obesity as a major feature LD2A Malformative disorders of sex development LD2A.0 Ovotesticular disorder of sex development LD2A.1 46,XY gonadal dysgenesis LD2A.2 Testicular agenesis LD2A.3 46,XY disorder of sex development due to a defect in testosterone metabolism LD2A.4 46,XY disorder of sex development due to androgen resistance LD2A.Y Other specified malformative disorders of sex development LD2A.Z Malformative disorders of sex development, unspecified LD2B Syndromes with premature ageing appearance as a major feature LD2C Overgrowth syndromes LD2D Phakomatoses or hamartoneoplastic syndromes LD2D.0 Peutz-Jeghers syndrome LD2D.1 Neurofibromatoses LD2D.2 Tuberous sclerosis LD2D.3 Gardner syndrome LD2D.4 Gorlin syndrome LD2D.Y Other specified phakomatoses or hamartoneoplastic syndromes LD2D.Z Phakomatoses or hamartoneoplastic syndromes, unspecified – LD2D.10 Neurofibromatosis type 1 – LD2D.11 Neurofibromatosis type 2 – LD2D.12 Neurofibromatosis type 3 – LD2D.1Y Other specified neurofibromatoses – LD2D.1Z Neurofibromatosis, unspecified LD2E Syndromes with structural anomalies due to inborn errors of metabolism LD2F Syndromes with multiple structural anomalies, without predominant body system involvement LD2F.0 Toxic or drug-related embryofetopathies LD2F.1 Syndromes with multiple structural anomalies, not of environmental origin LD2F.Y Other specified syndromes with multiple structural anomalies, without predominant body system involvement LD2F.Z Syndromes with multiple structural anomalies, without predominant body system involvement, unspecified – LD2F.00 Fetal alcohol syndrome – LD2F.01 Fetal hydantoin syndrome – LD2F.02 Embryofetopathy due to oral anticoagulant therapy – LD2F.03 Fetal Valproate Spectrum Disorder – LD2F.0Y Other specified toxic or drug-related embryofetopathies – LD2F.0Z Toxic or drug-related embryofetopathies, unspecified – LD2F.10 Prune belly syndrome – LD2F.11 VATER association – LD2F.12 Sirenomelia – LD2F.13 Meckel-Gruber syndrome – LD2F.14 MURCS association – LD2F.15 Noonan syndrome – LD2F.16 Otomandibular dysplasia – LD2F.1Y Other specified syndromes with multiple structural anomalies, not of environmental origin – LD2F.1Z Syndromes with multiple structural anomalies, not of environmental origin, unspecified LD2G Conjoined twins LD2H Syndromic genetic deafness LD2H.0 Fraser syndrome LD2H.1 Neuropathy with hearing impairment LD2H.2 Progressive deafness with stapes fixation LD2H.3 Waardenburg-Shah syndrome LD2H.4 Usher syndrome LD2H.Y Other specified syndromic genetic deafness LD2H.Z Syndromic genetic deafness, unspecified LD2Y Other specified multiple developmental anomalies or syndromes LD2Z Multiple developmental anomalies or syndromes, unspecified LD40 Complete trisomies of the autosomes LD40.0 Complete trisomy 21 LD40.1 Complete trisomy 13 LD40.2 Complete trisomy 18 LD40.Y Other specified complete trisomies of the autosomes LD40.Z Complete trisomies of the autosomes, unspecified LD41 Duplications of the autosomes LD41.0 Duplications of chromosome 1 LD41.1 Duplications of chromosome 2 LD41.2 Duplications of chromosome 3 LD41.3 Duplications of chromosome 4 LD41.4 Duplications of chromosome 5 LD41.5 Duplications of chromosome 6 LD41.6 Duplications of chromosome 7 LD41.7 Duplications of chromosome 8 LD41.8 Duplications of chromosome 9 LD41.9 Duplications of chromosome 10 LD41.A Duplications of chromosome 11 LD41.B Duplications of chromosome 12 LD41.C Duplications of chromosome 13 LD41.D Duplications of chromosome 14 LD41.E Duplications of chromosome 15 LD41.F Duplications of chromosome 16 LD41.G Duplications of chromosome 17 LD41.H Duplications of chromosome 18 LD41.J Duplications of chromosome 19 LD41.K Duplications of chromosome 20 LD41.L Duplications of chromosome 21 LD41.M Duplications of chromosome 22 LD41.N Extra ring or dicentric chromosomes LD41.P Duplications with other complex rearrangements LD41.Q Extra marker chromosomes LD41.Y Other specified duplications of the autosomes LD41.Z Duplications of the autosomes, unspecified – LD41.00 Duplications of the long arm of chromosome 1 – LD41.01 Duplications of the short arm of chromosome 1 – LD41.0Y Other specified duplications of chromosome 1 – LD41.0Z Duplications of chromosome 1, unspecified – LD41.10 Duplications of the long arm of chromosome 2 – LD41.11 Duplications of the short arm of chromosome 2 – LD41.1Y Other specified duplications of chromosome 2 – LD41.1Z Duplications of chromosome 2, unspecified – LD41.20 Duplications of the long arm of chromosome 3 – LD41.21 Duplications of the short arm of chromosome 3 – LD41.2Y Other specified duplications of chromosome 3 – LD41.2Z Duplications of chromosome 3, unspecified – LD41.30 Duplications of the long arm of chromosome 4 – LD41.31 Duplications of the short arm of chromosome 4 – LD41.3Y Other specified duplications of chromosome 4 – LD41.3Z Duplications of chromosome 4, unspecified – LD41.40 Duplications of the long arm of chromosome 5 – LD41.41 Duplications of the short arm of chromosome 5 – LD41.4Y Other specified duplications of chromosome 5 – LD41.4Z Duplications of chromosome 5, unspecified – LD41.50 Duplications of the long arm of chromosome 6 – LD41.51 Duplications of the short arm of chromosome 6 – LD41.5Y Other specified duplications of chromosome 6 – LD41.5Z Duplications of chromosome 6, unspecified – LD41.60 Duplications of the long arm of chromosome 7 – LD41.61 Duplications of the short arm of chromosome 7 – LD41.6Y Other specified duplications of chromosome 7 – LD41.6Z Duplications of chromosome 7, unspecified – LD41.70 Duplications of the long arm of chromosome 8 – LD41.71 Duplications of the short arm of chromosome 8 – LD41.7Y Other specified duplications of chromosome 8 – LD41.7Z Duplications of chromosome 8, unspecified – LD41.80 Duplications of the long arm of chromosome 9 – LD41.81 Duplications of the short arm of chromosome 9 – LD41.8Y Other specified duplications of chromosome 9 – LD41.8Z Duplications of chromosome 9, unspecified – LD41.90 Duplications of the long arm of chromosome 10 – LD41.91 Duplications of the short arm of chromosome 10 – LD41.9Y Other specified duplications of chromosome 10 – LD41.9Z Duplications of chromosome 10, unspecified – LD41.B0 Duplications of the long arm of chromosome 12 – LD41.B1 Duplications of the short arm of chromosome 12 – LD41.BY Other specified duplications of chromosome 12 – LD41.BZ Duplications of chromosome 12, unspecified – LD41.F0 Duplications of the long arm of chromosome 16 – LD41.F1 Duplications of the short arm of chromosome 16 – LD41.FY Other specified duplications of chromosome 16 – LD41.FZ Duplications of chromosome 16, unspecified – LD41.G0 Duplications of the long arm of chromosome 17 – LD41.G1 Duplications of the short arm of chromosome 17 – LD41.GY Other specified duplications of chromosome 17 – LD41.GZ Duplications of chromosome 17, unspecified – LD41.H0 Duplications of the long arm of chromosome 18 – LD41.H1 Duplications of the short arm of chromosome 18 – LD41.HY Other specified duplications of chromosome 18 – LD41.HZ Duplications of chromosome 18, unspecified – LD41.J0 Duplications of the long arm of chromosome 19 – LD41.J1 Duplications of the short arm of chromosome 19 – LD41.JY Other specified duplications of chromosome 19 – LD41.JZ Duplications of chromosome 19, unspecified – LD41.K0 Duplications of the long arm of chromosome 20 – LD41.K1 Duplications of the short arm of chromosome 20 – LD41.KY Other specified duplications of chromosome 20 – LD41.KZ Duplications of chromosome 20, unspecified LD42 Polyploidies LD42.0 Triploidy LD42.1 Tetraploidy LD42.Y Other specified polyploidies LD42.Z Polyploidies, unspecified LD43 Complete monosomies of the autosomes LD43.0 Complete monosomy of autosome LD43.1 Mosaic monosomy of autosome LD43.Y Other specified complete monosomies of the autosomes LD43.Z Complete monosomies of the autosomes, unspecified LD44 Deletions of the autosomes LD44.1 Deletions of chromosome 1 LD44.2 Deletions of chromosome 2 LD44.3 Deletions of chromosome 3 LD44.4 Deletions of chromosome 4 LD44.5 Deletions of chromosome 5 LD44.6 Deletions of chromosome 6 LD44.7 Deletions of chromosome 7 LD44.8 Deletions of chromosome 8 LD44.9 Deletions of chromosome 9 LD44.A Deletions of chromosome 10 LD44.B Deletions of chromosome 11 LD44.C Deletions of chromosome 12 LD44.D Deletions of chromosome 13 LD44.E Deletions of chromosome 14 LD44.F Deletions of chromosome 15 LD44.G Deletions of chromosome 16 LD44.H Deletions of chromosome 17 LD44.J Deletions of chromosome 18 LD44.K Deletions of chromosome 19 LD44.L Deletions of chromosome 20 LD44.M Deletions of chromosome 21 LD44.N Deletions of chromosome 22 LD44.P Deletions with other complex rearrangements LD44.Y Other specified deletions of the autosomes LD44.Z Deletions of the autosomes, unspecified – LD44.10 Deletions of the long arm of chromosome 1 – LD44.11 Deletions of the short arm of chromosome 1 – LD44.1Y Other specified deletions of chromosome 1 – LD44.1Z Deletions of chromosome 1, unspecified – LD44.20 Deletions of the long arm of chromosome 2 – LD44.21 Deletions of the short arm of chromosome 2 – LD44.2Y Other specified deletions of chromosome 2 – LD44.2Z Deletions of chromosome 2, unspecified – LD44.30 Deletions of the long arm of chromosome 3 – LD44.31 Deletions of the short arm of chromosome 3 – LD44.3Y Other specified deletions of chromosome 3 – LD44.3Z Deletions of chromosome 3, unspecified – LD44.40 Deletions of the long arm of chromosome 4 – LD44.41 Deletions of the short arm of chromosome 4 – LD44.4Y Other specified deletions of chromosome 4 – LD44.4Z Deletions of chromosome 4, unspecified – LD44.50 Deletions of the long arm of chromosome 5 – LD44.51 Deletions of the short arm of chromosome 5 – LD44.5Y Other specified deletions of chromosome 5 – LD44.5Z Deletions of chromosome 5, unspecified – LD44.60 Deletions of the long arm of chromosome 6 – LD44.61 Deletions of the short arm of chromosome 6 – LD44.6Y Other specified deletions of chromosome 6 – LD44.6Z Deletions of chromosome 6, unspecified – LD44.70 Deletions of the long arm of chromosome 7 – LD44.71 Deletions of the short arm of chromosome 7 – LD44.7Y Other specified deletions of chromosome 7 – LD44.7Z Deletions of chromosome 7, unspecified – LD44.80 Deletions of the long arm of chromosome 8 – LD44.81 Deletions of the short arm of chromosome 8 – LD44.8Y Other specified deletions of chromosome 8 – LD44.8Z Deletions of chromosome 8, unspecified – LD44.90 Deletions of the long arm of chromosome 9 – LD44.91 Deletions of the short arm of chromosome 9 – LD44.9Y Other specified deletions of chromosome 9 – LD44.9Z Deletions of chromosome 9, unspecified – LD44.A0 Deletions of the long arm of chromosome 10 – LD44.A1 Deletions of the short arm of chromosome 10 – LD44.AY Other specified deletions of chromosome 10 – LD44.AZ Deletions of chromosome 10, unspecified – LD44.B0 Deletions of the long arm of chromosome 11 – LD44.B1 Deletions of the short arm of chromosome 11 – LD44.BY Other specified deletions of chromosome 11 – LD44.BZ Deletions of chromosome 11, unspecified – LD44.C0 Deletions of the long arm of chromosome 12 – LD44.C1 Deletions of the short arm of chromosome 12 – LD44.CY Other specified deletions of chromosome 12 – LD44.CZ Deletions of chromosome 12, unspecified – LD44.G0 Deletions of the long arm of chromosome 16 – LD44.G1 Deletions of the short arm of chromosome 16 – LD44.GY Other specified deletions of chromosome 16 – LD44.GZ Deletions of chromosome 16, unspecified – LD44.H0 Deletions of the long arm of chromosome 17 – LD44.H1 Deletions of the short arm of chromosome 17 – LD44.HY Other specified deletions of chromosome 17 – LD44.HZ Deletions of chromosome 17, unspecified – LD44.J0 Deletions of the long arm of chromosome 18 – LD44.J1 Deletions of the short arm of chromosome 18 – LD44.JY Other specified deletions of chromosome 18 – LD44.JZ Deletions of chromosome 18, unspecified – LD44.K0 Deletions of the long arm of chromosome 19 – LD44.K1 Deletions of the short arm of chromosome 19 – LD44.KY Other specified deletions of chromosome 19 – LD44.KZ Deletions of chromosome 19, unspecified – LD44.L0 Deletions of the long arm of chromosome 20 – LD44.L1 Deletions of the short arm of chromosome 20 – LD44.LY Other specified deletions of chromosome 20 – LD44.LZ Deletions of chromosome 20, unspecified – LD44.N0 CATCH 22 phenotype – LD44.NY Other specified deletions of chromosome 22 – LD44.NZ Deletions of chromosome 22, unspecified LD45 Uniparental disomies LD45.0 Uniparental disomies of maternal origin LD45.1 Uniparental disomies of paternal origin LD45.Y Other specified uniparental disomies LD45.Z Uniparental disomies, unspecified LD46 Imprinting errors LD46.0 Maternal imprinting error LD46.1 Paternal imprinting error LD46.Y Other specified imprinting errors LD46.Z Imprinting errors, unspecified LD47 Balanced rearrangements or structural rearrangements LD47.0 Balanced translocation and insertion in normal individual LD47.1 Chromosome inversion in normal individual LD47.2 Balanced autosomal rearrangement in abnormal individual LD47.3 Balanced sex or autosomal rearrangement in abnormal individual LD47.4 Autosomal fragile site LD47.Y Other specified balanced rearrangements or structural rearrangements LD47.Z Balanced rearrangements or structural rearrangements, unspecified LD50 Number anomalies of chromosome X – LD50.0 Turner syndrome – LD50.1 Karyotype 47,XXX – LD50.2 Mosaicism, lines with various numbers of X chromosomes – LD50.3 Klinefelter syndrome – LD50.Y Other specified number anomalies of chromosome X – LD50.Z Number anomalies of chromosome X, unspecified –– LD50.00 Karyotype 45, X –– LD50.01 Karyotype 46, X iso Xq –– LD50.02 Karyotype 46, X with abnormal sex chromosome, except iso Xq –– LD50.03 Mosaicism, 45, X, 46, XX or XY –– LD50.04 Mosaicism, 45, X or other cell line with abnormal sex chromosome –– LD50.30 Klinefelter syndrome with karyotype 47,XXY, regular –– LD50.31 Klinefelter syndrome, male with more than two X chromosomes –– LD50.3Y Other specified Klinefelter syndrome –– LD50.3Z Klinefelter syndrome, unspecified LD51 Structural anomalies of chromosome X, excluding Turner syndrome LD52 Number anomalies of chromosome Y – LD52.0 Male with 46,XX karyotype – LD52.1 Male with double or multiple Y – LD52.Y Other specified number anomalies of chromosome Y – LD52.Z Number anomalies of chromosome Y, unspecified LD53 Structural anomalies of chromosome Y LD54 Male with sex chromosome mosaicism LD55 Fragile X chromosome LD56 Chimaera 46, XX, 46, XY – LD56.0 Androgenetic chimaera – LD56.1 Gynogenetic chimaera – LD56.Y Other specified chimaera 46, XX, 46, XY – LD56.Z Chimaera 46, XX, 46, XY, unspecified LD5Y Other specified sex chromosome anomalies LD5Z Sex chromosome anomalies, unspecified LD7Y Other specified chromosomal anomalies, excluding gene mutations LD7Z Chromosomal anomalies, excluding gene mutations, unspecified LD90 Conditions with disorders of intellectual development as a relevant clinical feature LD90.0 Angelman syndrome LD90.1 Early-onset parkinsonism - intellectual deficit LD90.2 Pelizaeus-Merzbacher-like disease LD90.3 Prader-Willi syndrome LD90.4 Rett syndrome LD90.Y Other specified conditions with disorders of intellectual development as a relevant clinical feature LD90.Z Conditions with disorders of intellectual development as a relevant clinical feature, unspecified LD9Y Other specified developmental anomalies LD9Z Developmental anomalies, unspecified
synonyms Hypohidrosis due to genetic abnormalities of eccrine gland structure or function
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