2007 ICD-9-CM Volume 1 Diagnosis Codes Home > Congenital Anomalies 740-759 >
 Other congenital anomalies of nervous system- 742 is a non-specific code that cannot be used to specify a diagnosis
Encephalocele- Cerebral tissue herniation through a congenital or acquired defect in the skull. The majority of congenital encephaloceles occur in the occipital or frontal regions. Clinical features include a protuberant mass that may be pulsatile. The quantity and location of protruding neural tissue determines the type and degree of neurologic deficit. Visual defects, psychomotor developmental delay, and persistent motor deficits frequently occur.
- 742.0 is a specific code that can be used to specify a diagnosis
- 742.0 contains 35 index entries
Microcephalus- Abnormal smallness of the head, usually associated with mental retardation. (Dorland, 27th ed)
- 742.1 is a specific code that can be used to specify a diagnosis
- 742.1 contains 21 index entries
Congenital reduction deformities of brain- Anterior midline brain, cranial, and facial malformations resulting from the failure of the embryonic prosencephalon to undergo segmentation and cleavage. Alobar prosencephaly is the most severe form and features anophthalmia; cyclopia; severe MENTAL RETARDATION; CLEFT LIP; CLEFT PALATE; SEIZURES; and microcephaly. Semilobar holoprosencepaly is characterized by hypotelorism, microphthalmia, coloboma, nasal malformations, and variable degrees of mental retardation. Lobar holoprosencephaly is associated with mild (or absent) facial malformations and intellectual abilities that range from mild mental retardation to normal. Holoprosencephaly is associated with CHROMOSOME ABNORMALITIES.
- 742.2 is a specific code that can be used to specify a diagnosis
- 742.2 contains 30 index entries
Congenital hydrocephalus- 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. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)
- 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
Other specified congenital anomalies of brain- 742.4 is a specific code that can be used to specify a diagnosis
- 742.4 contains 37 index entries
Other specified congenital anomalies of spinal cord- 742.5 is a non-specific code that cannot be used to specify a diagnosis
Diastematomyelia- 742.51 is a specific code that can be used to specify a diagnosis
- 742.51 contains 2 index entries
Hydromyelia- 742.53 is a specific code that can be used to specify a diagnosis
- 742.53 contains 2 index entries
Other specified congenital anomalies of spinal cord- 742.59 is a specific code that can be used to specify a diagnosis
- 742.59 contains 29 index entries
Other specified congenital anomalies of nervous system- An autosomal disorder of the peripheral and autonomic nervous systems limited to individuals of Ashkenazic Jewish descent. Clinical manifestations are present at birth and include diminished lacrimation, defective thermoregulation, orthostatic hypotension (HYPOTENSION, ORTHOSTATIC), fixed pupils, excessive SWEATING, loss of pain and temperature sensation, and absent reflexes. Pathologic features include reduced numbers of small diameter peripheral nerve fibers and autonomic ganglion neurons. (From Adams et al., Principles of Neurology, 6th ed, p1348; Nat Genet 1993;4(2):160-4)
- 742.8 is a specific code that can be used to specify a diagnosis
- 742.8 contains 45 index entries
Unspecified congenital anomaly of brain spinal cord and nervous system- Structural abnormalities of the central or peripheral nervous system resulting primarily from defects of embryogenesis.
- 742.9 is a specific code that can be used to specify a diagnosis
- 742.9 contains 39 index entries
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