2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Congenital Anomalies 740-759 > Other Congenital Anomalies Of Nervous System 742.* > 2006 ICD-9-CM Diagnosis 742.3 Congenital hydrocephalus View the most recent version of ICD-9-CM 742.3 A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. Hydrocephalus, absence of the cerebellar vermis, and posterior fossa cyst continuous with the fourth ventricle are the main characteristics of this syndrome. About 4% of all cases of hydrocephalus are complicated by DWS. Associated disorders may include Aase-Smith syndrome 1, Aicardi, Coffin-Siris, cryptophthalmos, Ehlers-Danlos, Ellis-van Creveld, Jones syndrome, Joubert, Ruvalcaba-Myhre-Smith, Walker-Warburg, Meckel, Ritscher-Schinzel, and Veradi syndromes. Dandy-Walker syndrome associated with macrocephaly, facial anomalies, developmental delay, and brain stem dysgenesis inherited as an X-linked recessive trait was reported as a separate syndrome. 742.3 is a specific code that can be used to specify a diagnosis 742.3 contains 34 index entries View the ICD-9-CM Volume 1 742.* hierarchy Alternate Terminology Aqueduct of Sylvius: anomaly obstruction, congenital stenosis Atresia of foramina of Magendie and Luschka Hydrocephalus in newborn 742.3 Excludes hydrocephalus: acquired (331.3-331.4) due to congenital toxoplasmosis (771.2) with any condition classifiable to 741.9 (741.0)  Index entries containing 742.3: Absence (organ or part) (complete or partial) skull bone 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Agenesis - see also Absence, by site, congenital skull (bone) 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Anomaly, anomalous (congenital) (unspecified type) 759.9 aqueduct of Sylvius 742.3 with spina bifida (see also Spina bifida) 741.0 bone NEC 756.9 skull 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 skull (bone) 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 specified type NEC bone(s) 756.9 skull 756.0 with hydrocephalus 742.3 skull (bone(s)) 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Atresia, atretic (congenital) 759.89 aqueduct of Sylvius 742.3 with spina bifida (see also Spina bifida) 741.0 foramen of Luschka 742.3 with spina bifida (see also Spina bifida) 741.0 Magendie 742.3 with spina bifida (see also Spina bifida) 741.0 Block foramen Magendie (acquired) 331.3 congenital 742.3 with spina bifida (see also Spina bifida) 741.0 Cyst (mucus) (retention) (serous) (simple) Dandy-Walker 742.3 with spina bifida (see also Spina bifida) 741.0 Dandy-Walker deformity or syndrome (atresia, foramen of Magendie) 742.3 with spina bifida (see also Spina bifida) 741.0 Deformity 738.9 Dandy-Walker 742.3 with spina bifida (see also Spina bifida) 741.0 skull (acquired) 738.19 congenital 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Distortion (congenital) skull bone(s) 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Forking, aqueduct of Sylvius 742.3 with spina bifida (see also Spina bifida) 741.0 Hydrancephaly 742.3 with spina bifida (see also Spina bifida) 741.0 Hydranencephaly 742.3 with spina bifida (see also Spina bifida) 741.0 Hydrocephalus (acquired) (external) (internal) (malignant) (noncommunicating) (obstructive) (recurrent) 331.4 aqueduct of Sylvius stricture 742.3 with spina bifida (see also Spina bifida) 741.0 chronic 742.3 with spina bifida (see also Spina bifida) 741.0 congenital (external) (internal) 742.3 with spina bifida (see also Spina bifida) 741.0 due to stricture of aqueduct of Sylvius 742.3 with spina bifida (see also Spina bifida) 741.0 foramen Magendie block (acquired) 331.3 congenital 742.3 with spina bifida (see also Spina bifida) 741.0 newborn 742.3 with spina bifida (see also Spina bifida) 741.0 Hypoplasia, hypoplasis 759.89 skull (bone) 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Imperfect closure (congenital) skull 756.0 with hydrocephalus 742.3 with spina bifida (see also Spina bifida) 741.0 Obstruction, obstructed, obstructive aqueduct of Sylvius 331.4 congenital 742.3 with spina bifida (see also Spina bifida) 741.0 foramen of Monro (congenital) 742.3 with spina bifida (see also Spina bifida) 741.0 Occlusion aqueduct of Sylvius 331.4 congenital 742.3 with spina bifida (see also Spina bifida) 741.0 Septum, septate (congenital) - see also Anomaly, specified type NEC aqueduct of Sylvius 742.3 with spina bifida (see also Spina bifida) 741.0 Stenosis (cicatricial) - see also Stricture aqueduct of Sylvius (congenital) 742.3 with spina bifida (see also Spina bifida) 741.0 acquired 331.4 Stricture (see also Stenosis) 799.89 aqueduct of Sylvius (congenital) 742.3 with spina bifida (see also Spina bifida) 741.0 acquired 331.4 Syndrome - see also Disease Dandy-Walker (atresia, foramen of Magendie) 742.3 with spina bifida (see also Spina bifida) 741.0