Disease staging: CliniCal anD CoDeD Criteria - HCUP

Clinical Criteria with Description, Diagnostic Findings and ICD-9-CM codes
DXCat Number: NEU19
MultiStage: No
DXCat Label: Neurofibromatosis Type I [Von Recklinghausen's Disease] Specificity: A
Etiology:
Genetic (or hereditary)
Stage Description Diagnostic findings ICD-9-CM Codes
1.01 Nuerofibromatosis Type I Neurofibromatosis Type I [genetic analysis report]
DX 23770-23772;
OR cutaneous neurofibromatosis [operative/pathology report]
OR subcutaneous neurofibromatosis [operative/pathology report]
OR Autosomal dominant, chromosome 17, gene codes for neurofibromin [genetic analysis
report]
OR Two or more of the following must be present:
cafe-au-lait spot ≥ 1.5 cm in diameter for age > 13 years old
OR cafe-au-lait spot ≥ 0.5 cm in diameter for < age 13 years old
AND total of ≥ 6 cafe-au-lait spots
OR ≥ two neurofibromas of any type
OR ≥ 1 plexiform neurofibroma
OR freckling in the axillary and inguinal regions
OR ≥ 1 optic pathways glioma
OR ≥ 2 Lisch nodules (iris amartomas)
OR osseous lesion, such as sphenoid wing dysplasia or thinning of the cortex of long
bones
OR a first-degree relative with NF1 by the above criteria
2.01 with paresthesias or hypesthesia Stage 1.01
AND paresthesias
OR hypesthesia
2.02 with macroencephaly or learning disability Stage 1.01-2.01
AND learning disability
OR head diameter ≥ 95th percentile
2.03 with pheochromocytoma or medullary Stage 1.01-2.02
thyroid carcinoma or dysplastic scoliosis or AND sarcomatous degeneration [operative/pathology report]
rhabdomyosarcoma or sarcomatous
OR neurofibrosarcoma [operative/pathology report]
degeneration
OR malignant schwanomma [operative/pathology report]
OR pheochromocytoma [operative/pathology report]
OR medullary thyroid carcinoma [operative/pathology report]
OR dysplastic scoliosis [operative/pathology report]
OR rhabdomyosarcoma [operative/pathology report]
3.01 with hemiparesis or paraparesis Stage 1.01-2.03
AND hemiparesis
OR paraparesis
3.02 with hemiplegia or paraplegia Stage 1.01-3.01
AND hemiplegia
OR paraplegia
STAGE 1.01 + DX 7820;
STAGE 1.01-2.01 + (DX 3152,
7424);
STAGE 1.01-2.02 + (DXCAT END15
OR DXCAT END14 OR DX 73743,
1710-1719, 2150-2159);
STAGE 1.01-2.03 + (DX 34200-
34202, 34210-34282, 34290-
34292, 3441);
NO;
Thursday, January 07, 2010 9:22:50 AM
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