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2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Congential Anomalies 740-759 > ICD-9-CM Diagnosis 759 Other and unspecified congenital anomalies 759 is a non-specific code that cannot be used to specify a diagnosis ICD-9-CM Diagnosis 759.0 Anomalies of spleen congenital 759.0 is a specific code that can be used to specify a diagnosis 759.0 contains 28 index entries ICD-9-CM Diagnosis 759.1 Anomalies of adrenal gland congenital 759.1 is a specific code that can be used to specify a diagnosis 759.1 contains 14 index entries ICD-9-CM Diagnosis 759.2 Anomalies of other endocrine glands congenital A cyst in the neck caused by persistence of portions of, or by lack of closure of, the primitive thyroglossal duct. (Dorland, 27th ed) 759.2 is a specific code that can be used to specify a diagnosis 759.2 contains 65 index entries ICD-9-CM Diagnosis 759.3 Situs inversus A laterization defect marked by asymmetric position of visceral organs usually occurring as a component of a wide variety of abnormalities. Transposition may be complete, with the heart, lungs and all abdominal organs reversed, or incomplete, manifesting itself as a simple reversal of the stomach or spleen. Associated malformations are variable and may include tetralogy of Fallot, transposition of great vessels, pulmonary valve stenosis, ventricular and atrial septal defects, asplenia-polysplenia. and other defects. Lateral transposition of the viscera of the thorax and abdomen. It has a familial pattern and consanguineous parents have been reported. (From Dorland, 27th ed) 759.3 is a specific code that can be used to specify a diagnosis 759.3 contains 16 index entries ICD-9-CM Diagnosis 759.4 Conjoined twins 759.4 is a specific code that can be used to specify a diagnosis 759.4 contains 16 index entries ICD-9-CM Diagnosis 759.5 Tuberous sclerosis A triad of epilepsy, mental retardation, and angiofibromas of numerous organs with intracranial hamartomatous lesions involving subependymal nodules and cerebral cortical tubers (hence the name "tuberous sclerosis." An autosomal dominant disorder which is generally classified as a phacomatosis. Pathologically, the condition is characterized by glial cell tumors which arise in the cerebral hemispheres and retina. There is an increased incidence of benign rhabdomyomas of the heart and angiomyolipomas of kidney, liver, lungs, thyroid, and testes. Clinical manifestations include MENTAL RETARDATION; adenoma sebaceum of the face (actually angiofibromas); EPILEPSY; SPASMS; INFANTILE; Shagreen patches on the trunk; and subungual fibromas. (From Adams et al., Principles of Neurology, 6th ed, p1011) Hereditary disease characterized by seizures, mental retardation, developmental delay, and skin and ocular lesions. First signs usually occur during infancy or childhood but in rare cases may not occur until 2nd or 3rd decade. 759.5 is a specific code that can be used to specify a diagnosis 759.5 contains 13 index entries ICD-9-CM Diagnosis 759.6 Other congenital hamartoses not elsewhere classified A congenital syndrome characterized by a port-wine nevus covering portions of the face and cranium (in the distribution of the ophthalmic division of the TRIGEMINAL NERVE) and angiomas of the meninges and choroid. Clinical manifestations include the onset of focal SEIZURES, progressive hemiparesis, GLAUCOMA, hemianopsia, and cognitive deficits in the first decade of life. By age two years, skull radiographs reveal "tramline calcifications" of the margins of the occipital and parietal lobes. Pathologically cortical neurons are replaced by glial tissue that undergoes calcification. (From Adams et al., Principles of Neurology, 6th ed, pp1018-9) A group of neurocutaneous disorders manifested by facial and leptomeningeal angiomas, ipsilateral gyriform calcifications of the cerebral cortex, seizures, development delay, hemiplegia, emotional and behavioral problems, and glaucoma and other ocular disorders. Nevus flammeus on the side of the face ipsilateral to angiomatosis sometimes extends to neck, chest, and back. Angiomatosis may occasionally involve the choroid plexus, thyroid, pituitary gland, lungs, gastrointestinal organs, pancreas, ovaries, and thymus. Correlation between the distribution of the nevus and the course of the trigeminal nerve is responsible for naming the syndrome "trigemino-encephalo-angiomatosis," but later findings found the relationship to be fortuitous. The syndrome frequently occurs in incomplete forms, presenting different combinations of symptoms. A hereditary disease caused by autosomal dominant mutations involving CHROMOSOME 19. It is characterized by the presence of INTESTINAL POLYPS, consistently in the JEJUNUM, and mucocutaneous pigmentation with MELANIN spots of the lips, buccal MUCOSA, and digits. A rare inherited disorder in which blood vessels grow abnormally in the eyes, brain, spinal cord, adrenal glands, or other parts of the body. People with von Hippel-Lindau syndrome have a higher risk of developing some types of cancer. An autosomal dominant disorder associated with various neoplasms including central nervous system (most often cerebellar) and retinal HEMANGIOBLASTOMA, endolymphatic sac tumors, renal cell carcinoma (see CARCINOMA, RENAL CELL), renal and pancreatic cysts, HEMANGIOMA of the spinal cord, and PHEOCHROMOCYTOMA. The most common presenting manifestations are neurologic deficits associated with intracranial hemangioblastomas which may HEMORRHAGE, causing ataxia, INTRACRANIAL HYPERTENSION, and other signs of neurologic dysfunction. (From Neurochirurgie 1998 Nov;44(4):258-66) An inherited condition characterized by generalized hamartomatous multiple polyposis of the intestinal tract. Transmitted in an autosomal dominant fashion, Peutz-Jeghers syndrome consistently involves the jejunum and is associated with melanin spots of the lips, buccal mucosa, and fingers. This syndrome is associated with abnormalities of chromosome 19. Also known as Jeghers-Peutz syndrome and Peutz's syndrome. --2004 An inherited familial cancer syndrome which is characterized by development of capillary hemangioblastomas of the central nervous system and retina; clear cell renal carcinoma; pheochromocytoma; pancreatic tumors; and inner ear tumors. The syndrome is associated with germline mutations of the VHL tumor suppressor gene, located on chromosome 3p25-26. Symptoms of VHL syndrome may not be apparent until the third decade of life. CNS hemangioblastoma is the most common cause of death, followed by clear cell renal cell carcinoma. --2004 759.6 is a specific code that can be used to specify a diagnosis 759.6 contains 60 index entries ICD-9-CM Diagnosis 759.7 Multiple congenital anomalies so described 759.7 is a specific code that can be used to specify a diagnosis 759.7 contains 5 index entries ICD-9-CM Diagnosis 759.8 Other specified congenital anomalies 759.8 is a non-specific code that cannot be used to specify a diagnosis 759.8 contains 1 index entry ICD-9-CM Diagnosis 759.81 Prader-willi syndrome A chromosomal disorder associated with a deletion of the proximal portion of the long arm of chromosome 15 (15q11-q13) in the majority of affected individuals. The abnormalities occur exclusively on the paternally derived chromosome 15. Clinical manifestations include MENTAL RETARDATION, muscular hypotonia, OBESITY, hyperphagia, short stature, hypogonadism, strabismus, and hypersomnolence. (Menkes, Textbook of Child Neurology, 5th ed, p229) A syndrome characterized at birth by the lack of spontaneous movements and protective reflexes, thus giving an appearance of severe brain damage. Profound hypotonia may cause asphyxia. Sucking and swallowing reflexes are absent or decreased. Deficient thermoregulation, amyotonia, and hypogonadism are usually associated. After a few weeks or months, the affected infants become more responsive and more alert. Areflexia disappears gradually but hypotonia may persist longer. This phase is marked mainly by mental subnormality, delayed growth and motor development, speech defect, lack of emotional control, voracious appetite leading to obesity, hypotonia, hyperlaxity, delayed bone maturation, and multiple orofacial and other disoders. There is a tendency to develop diabetes mellitus and cardiac failure in some patients. Pain insensitivity is common. Prader-Willi habitus associated with osteopenia and camptodactyly is known as the Urban-Rogers-Meyer syndrome. 759.81 is a specific code that can be used to specify a diagnosis 759.81 contains 9 index entries ICD-9-CM Diagnosis 759.82 Marfan syndrome A hereditary disorder of connective tissue characterized by tall stature, elongated extremities, subluxation of the lens, dilatation of the ascending aorta, and "pigeon breast." It is inherited as an autosomal dominant trait. 759.82 is a specific code that can be used to specify a diagnosis 759.82 contains 10 index entries ICD-9-CM Diagnosis 759.83 Fragile x syndrome A condition characterized genotypically by mutation of the distal end of the long arm of the X chromosome (at gene loci FRAXA or FRAXE) and phenotypically by cognitive impairment, hyperactivity, SEIZURES, language delay, and enlargement of the ears, head, and testes. MENTAL RETARDATION occurs in nearly all males and roughly 50% of females with the full mutation of FRAXA. (From Menkes, Textbook of Child Neurology, 5th ed, p226) An inherited disease characterized by the presence of a fragile site in the long arm of chromosome X. It is a common cause of mental retardation, second only in frequency to the Down syndrome (trisomy 21). The expression varies with mental retardation, macroorchidism, high-pitched voice, narrow face, long jaw, large ears, prominent forehead, highly arched narrow palate, and joint laxity as the most common characteristics. Microcephaly, typical facies, shortness of stature, and absence of macroorchidism characterize the Renpenning but not Martin-Bell syndrome. Major characteristics of the Martin-Bell syndrome include: Mental retardation with speech and behavioral disorders; connective tissue dysplasia; square facies with midfacial hypoplasia; slightly below normal height without intra- uterine growth retardation; average or above average head circumference; large and frequently anteverted ears; prominent forehead and supraorbital ridges; large nose; prominent mandible which becomes apparent during adolescence; joint laxity; minor limb anomalies; dermatoglyphic abnormalities; and seizures. 759.83 is a specific code that can be used to specify a diagnosis 759.83 contains 2 index entries ICD-9-CM Diagnosis 759.89 Other specified congenital anomalies 759.89 is a specific code that can be used to specify a diagnosis 759.89 contains 167 index entries ICD-9-CM Diagnosis 759.9 Congenital anomaly unspecified Congenital malformations of organs or parts. 759.9 is a specific code that can be used to specify a diagnosis 759.9 contains 18 index entries