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Epidemiology of choanal atresia with special reference to the CHARGE association.
Pediatrics. 1997 Mar; 99(3):363-7.Ped

Abstract

OBJECTIVE

To present epidemiologic data on the relatively rare malformation choanal atresia, based on a large collection of material and with special stress on the significance of the so-called CHARGE (coloboma, heart defect, choanal atresia, retarded growth and development, genital anomaly, and ear defect with deafness) association.

METHODS

Data from three large registries of congenital malformations were used. Based on more than 5 million births, 444 infants with choanal atresia were identified.

RESULTS

The average rate of choanal atresia is 0.82 per 10,000 and varies among programs. There is no statistically significant difference between races in rates, even though white infants have a higher rate than those of other races. The higher rate found in the California program is mainly attributable to unilateral, isolated cases. Unilateral atresia occurs equally often on the right and left. Among all cases of choanal atresia, the sex distribution is normal, a slightly increased risk at twinning exists, and no effect of maternal age or parity is seen. Chromosome anomalies are found in 6% of infants with choanal atresia, and 21 infants (5%) have monogenic syndromes or conditions. An analysis of associated malformations (present in 47% of the infants without chromosome anomalies) indicated that although a weak nonrandom association can be demonstrated between the malformations entering the so-called CHARGE complex, only a small proportion of infants with choanal atresia and other components of that condition probably represent this entity. The term CHARGE association seems to be overused in clinical practice.

CONCLUSION

To be meaningful, the term CHARGE should be restricted to infants with multiple malformations and choanal atresia and/or coloboma combined with other cardinal malformations (heart, ear, and genital) and with a total of at least three cardinal malformations. Growth retardation should not be used in the definition.

Authors+Show Affiliations

California Birth Defects Monitoring Program, Emeryville, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

9041289

Citation

Harris, J, et al. "Epidemiology of Choanal Atresia With Special Reference to the CHARGE Association." Pediatrics, vol. 99, no. 3, 1997, pp. 363-7.
Harris J, Robert E, Källén B. Epidemiology of choanal atresia with special reference to the CHARGE association. Pediatrics. 1997;99(3):363-7.
Harris, J., Robert, E., & Källén, B. (1997). Epidemiology of choanal atresia with special reference to the CHARGE association. Pediatrics, 99(3), 363-7.
Harris J, Robert E, Källén B. Epidemiology of Choanal Atresia With Special Reference to the CHARGE Association. Pediatrics. 1997;99(3):363-7. PubMed PMID: 9041289.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Epidemiology of choanal atresia with special reference to the CHARGE association. AU - Harris,J, AU - Robert,E, AU - Källén,B, PY - 1997/3/1/pubmed PY - 1997/3/1/medline PY - 1997/3/1/entrez SP - 363 EP - 7 JF - Pediatrics JO - Pediatrics VL - 99 IS - 3 N2 - OBJECTIVE: To present epidemiologic data on the relatively rare malformation choanal atresia, based on a large collection of material and with special stress on the significance of the so-called CHARGE (coloboma, heart defect, choanal atresia, retarded growth and development, genital anomaly, and ear defect with deafness) association. METHODS: Data from three large registries of congenital malformations were used. Based on more than 5 million births, 444 infants with choanal atresia were identified. RESULTS: The average rate of choanal atresia is 0.82 per 10,000 and varies among programs. There is no statistically significant difference between races in rates, even though white infants have a higher rate than those of other races. The higher rate found in the California program is mainly attributable to unilateral, isolated cases. Unilateral atresia occurs equally often on the right and left. Among all cases of choanal atresia, the sex distribution is normal, a slightly increased risk at twinning exists, and no effect of maternal age or parity is seen. Chromosome anomalies are found in 6% of infants with choanal atresia, and 21 infants (5%) have monogenic syndromes or conditions. An analysis of associated malformations (present in 47% of the infants without chromosome anomalies) indicated that although a weak nonrandom association can be demonstrated between the malformations entering the so-called CHARGE complex, only a small proportion of infants with choanal atresia and other components of that condition probably represent this entity. The term CHARGE association seems to be overused in clinical practice. CONCLUSION: To be meaningful, the term CHARGE should be restricted to infants with multiple malformations and choanal atresia and/or coloboma combined with other cardinal malformations (heart, ear, and genital) and with a total of at least three cardinal malformations. Growth retardation should not be used in the definition. SN - 1098-4275 UR - https://www.unboundmedicine.com/medline/citation/9041289/Epidemiology_of_choanal_atresia_with_special_reference_to_the_CHARGE_association_ L2 - http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=9041289 DB - PRIME DP - Unbound Medicine ER -