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Length of life and treatment intensity in infants diagnosed prenatally or postnatally with congenital anomalies considered to be lethal

Journal of Perinatology volume 31, pages 387–391 (2011)Cite this article

Subjects

Abstract

Objectives:

The objectives of this study were (1) to compare age at death and the intensity and cost of medical treatment for infants diagnosed prenatally or postnatally with congenital anomalies considered to be lethal. (2) To determine whether greater treatment intensity is associated with longer life.

Study Design:

This is a retrospective cohort study of all fetuses and neonates with congenital anomalies classified as lethal who were diagnosed or treated at the University of North Carolina Hospitals from January 1998 to December 2003.

Result:

The cohort consisted of 192 fetuses and infants: 160 were diagnosed prenatally, 2 were diagnosed perinatally, and 30 were diagnosed postnatally. In all, 115 (72%) pregnancies were terminated. Of the liveborn infants, 75% died before 10 days of age and 90% before 4 months of age. Compared with postnatally diagnosed infants, prenatally diagnosed infants received less intense treatment (median average daily Neonatal Therapeutic Intervention Scoring System score 8.3 versus 14.0; P=0.02), at less cost (median direct cost of hospitalization $1550 versus $8474; P=0.03) and died sooner (median age at death <1 day versus 4 days; P=0.01). Greater treatment intensity did not correlate with longer survival (r=−0.04; P=0.66).

Conclusion:

Although some kinds of medical therapy may be appropriate for newborns with lethal congenital anomalies, highly aggressive interventions did not prolong survival and should not be offered. Even when pregnancy termination is not elected, infants diagnosed prenatally receive less intense care.

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References

  1. Peller AJ, Westgate MN, Holmes LB . Trends in congenital malformations, 1974 to 1999: effect of prenatal diagnosis and elective termination. Obstet Gynecol 2004; 104: 957–964.

    Article  PubMed  Google Scholar 

  2. Greenhouse L . Court order to treat baby with partial brain prompts debate on costs and ethics. The New York Times, 20 February 1994, Sect. 1.

  3. Clark PA . Medical futility in pediatrics: is it time for a public policy? J Public Health Policy 2002; 23: 66–89.

    Article  PubMed  Google Scholar 

  4. McGraw MP, Perlman JM . Attitudes of neonatologists toward delivery room management of confirmed trisomy 18: potential factors influencing a changing dynamic. Pediatrics 2008; 121: 1106–1110.

    Article  PubMed  Google Scholar 

  5. United States Department of Health and Human Services. The International Classification of Diseases, Ninth revision, Clinical Modification: ICD-9-CM 1991. Available from http://www.cdc.gov/nchs/icd/icd9cm.htm.

  6. Barnes AM, Chang W, Morello R, Cabral WA, Weis M, Eyre DR et al. Deficiency of cartilage-associated protein in recessive lethal osteogenesis imperfecta. N Engl J Med 2006; 355: 2757–2764.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Chen H, Liu CT, Yang SS . Achondrogenesis: a review with special consideration of achondrogenesis type II (Langer-Saldino). Am J Med Genet 1981; 10: 379–394.

    Article  CAS  PubMed  Google Scholar 

  8. Connor JM, Connor RA, Sweet EM, Gibson AA, Patrick WJ, McNay MB et al. Lethal neonatal chondrodysplasias in the West of Scotland 1970 to 1983 with a description of a thanatophoric, dysplasialike, autosomal recessive disorder, Glasgow variant. Am J Med Genet 1985; 22: 243–253.

    Article  CAS  PubMed  Google Scholar 

  9. Potter EL . Bilateral absence of ureters and kidneys: a report of 50 cases. Obstet Gynecol 1965; 25: 3–12.

    CAS  PubMed  Google Scholar 

  10. Scott JE . Fetal, perinatal, and infant death with congenital renal anomaly. Arch Dis Child 2002; 87: 114–117.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Weissman A, Diukman R, Auslender R . Fetal acrania: five new cases and review of the literature. J Clin Ultrasound 1997; 25: 511–514.

    Article  CAS  PubMed  Google Scholar 

  12. Jaquier M, Klein A, Boltshauser E . Spontaneous pregnancy outcome after prenatal diagnosis of anencephaly. BJOG 2006; 113: 951–953.

    Article  CAS  PubMed  Google Scholar 

  13. Rasmussen SA, Wong LY, Yang Q, May KM, Friedman JM . Population-based analyses of mortality in trisomy 13 and trisomy 18. Pediatrics 2003; 111: 777–784.

    Article  PubMed  Google Scholar 

  14. Hasegawa T, Harada N, Ikeda K, Ishii T, Hokuto I, Kasai K et al. Digynic triploid infant surviving for 46 days. Am J Med Genet 1999; 87: 306–310.

    Article  CAS  PubMed  Google Scholar 

  15. Lee K, Khoshnood B, Chen L, Wall SN, Cromie WJ, Mittendorf RL . Infant mortality from congenital malformations in the United States, 1970 to 1997. Obstet Gynecol 2001; 98: 620–627.

    CAS  PubMed  Google Scholar 

  16. Rodriguez MM, Reik RA, Carreno TD, Fojaco RM . Cluster of iniencephaly in Miami. Pediatr Pathol 1991; 11: 211–221.

    Article  CAS  PubMed  Google Scholar 

  17. Jones KL . Smith's Recognizable Patterns of Human Malformation. 6th edn. Elsevier Saunders: Philadelphia, 2006.

    Google Scholar 

  18. Online Mendelian Inheritance in Man, OMIM (TM). McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD) and National Center for Biotechnology Information, National Library of Medicine (Bethesda, MD) (cited 14 June 2005); available from http://www.ncbi.nlm.nih.gov/omim.

  19. Jeanty P . TheFetus.net (cited 14 July 2005); available from http://www.thefetus.net/index.php.

  20. Gray JE, Richardson DK, McCormick MC, Workman-Daniels K, Goldmann DA . Neonatal therapeutic intervention scoring system: a therapy-based severity-of-illness index. Pediatrics 1992; 90: 561–567.

    CAS  PubMed  Google Scholar 

  21. McAbee GN, Chan A, Erde EL . Prolonged survival with hydranencephaly: report of two patients and literature review. Pediatr Neurol 2000; 23: 80–84.

    Article  CAS  PubMed  Google Scholar 

  22. Trisomy 18 Foundation. Trisomy 18 foundation forum: children that live, ages & how are they developmentally. (cited 25 March 2007); available from http://trisomy18support.org/modules/newbb/viewtopic.php?topic_id=5923&forum=41.

  23. Living with Trisomy 13. Trisomy 13 syndrome—Photos, videos & updates of children living with Trisomy 13. (cited 25 March 2007); available from http://www.livingwithtrisomy13.org/trisomy-13.htm.

  24. Aytar MH, Dogulu F, Cemil B, Ergun E, Kurt G, Baykaner K . Iniencephaly and long-term survival: a rare case report. Child Nerv Syst 2007; 6: 719–721.

    Article  Google Scholar 

  25. Kaneko Y, Kobayashi J, Yamamoto Y, Yoda H, Kanetaka Y, Nakajima Y et al. Intensive cardiac management in patients with trisomy 13 or trisomy 18. Am J Med Genet 2008; 146: 1372–1380.

    Article  Google Scholar 

  26. Kosho T, Nakamura T, Kawame H, Baba A, Tamura M, Fukushima Y . Neonatal management of trisomy 18: clinical details of 24 patients receiving intensive treatment. Am J Med Genet 2006; 140: 937–944.

    Article  PubMed  Google Scholar 

  27. United States Department of Health and Human Services. Nondiscrimination on the basis of handicap; procedures and guidelines relating to health care for handicapped infants—HHS. Final rules. Fed Regist 1985; 50: 14879–14892.

    Google Scholar 

  28. Kopelman LM . Are the 21-year-old Baby Doe rules misunderstood or mistaken? Pediatrics 2005; 115: 797–802.

    Article  PubMed  Google Scholar 

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Acknowledgements

We thank Carl Bose, Cynthia Cassell, Michael McDade, Robert Meyer, Kelly Gilmore, Emily Hardisty, Anthony Johnson, Kenneth Moise, Cynthia Powell, Ben Saville, Kathleen Veness-Meehan and Honor Wolfe for their help with this project. Salary support for Andrew Courtwright was provided by a Howard Holderness Medical Fellowship Grant.

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Authors and Affiliations

  1. Department of Medicine, Massachusetts General Hospital, Boston, MA, USA

    A M Courtwright

  2. Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, NC, USA

    M M Laughon & M W Doron

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  1. A M Courtwright
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  2. M M Laughon
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  3. M W Doron
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Correspondence to M W Doron.

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Courtwright, A., Laughon, M. & Doron, M. Length of life and treatment intensity in infants diagnosed prenatally or postnatally with congenital anomalies considered to be lethal. J Perinatol 31, 387–391 (2011). https://doi.org/10.1038/jp.2010.124

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