Pediatric and Developmental Pathology

, Volume 8, Issue 3, pp 307–319

Sudden Unexpected Death in Childhood:A Report of 50 Cases

  • Henry F. Krous
  • Amy E. Chadwick
  • Laura Crandall
  • Julie M. Nadeau-Manning
Article

Abstract

Sudden unexplained death in childhood (SUDC) is rare, with a reported incidence in the United States of 1.5 deaths per 100,000 live births compared with 56 deaths per 100,000 live births for sudden infant death syndrome in 2001. The objectives of this study include a proposal for a general definition for SUDC and presentation of 36 cases of SUDC and 14 cases of sudden unexpected death in childhood. Cases were accrued through referrals or unsolicited via our Web page (http://www.sudc.org). Our analyses tentatively suggest a SUDC profile characterized by cases being 1 to 3 years in age, predominantly male, and frequently having a personal and family history of seizures that are often associated with a fever. A history of recent minor head trauma is not uncommon. They are usually born at term as singletons and occasionally have a family history of sudden infant death syndrome or SUDC. Most are found prone, often with their face straight down into the sleep surface. Minor findings are commonly seen at postmortem examination but do not explain their deaths. Comprehensive review of the medical history and circumstances of death and performance of a complete postmortem examination including ancillary studies and extensive histologic sampling of the brain are critical in determining the cause of death in these cases of sudden unexpected childhood death. Legislation enabling research and formation of a multicenter research team is recommended to unravel the mystery of SUDC.

Keywords

sudden unexplained death in childhood sudden infant death syndrome seizure febrile seizure prone face down 

References

  1. 1.
    Beckwith JB. The sudden infant death syndrome. Curr Probl Pediatr 1973;3:1–36Google Scholar
  2. 2.
    Krous HF. The pathology of sudden infant death syndrome: an overview. In: Culbertson JL, Krous HF, Bendell RD, eds. Sudden Infant Death Syndrome. Medical Aspects and Psychological Management. Baltimore: John Hopkins University Press, 1988;18–47Google Scholar
  3. 3.
    Krous HF, Beckwith JB, Byard RW, et al. Sudden infant death syndrome and unclassified sudden infant deaths: a definitional and diagnostic approach. Pediatrics 2004;114:234–238CrossRefPubMedGoogle Scholar
  4. 4.
    Molander N. Sudden natural death in later childhood and adolescence. Arch Dis Child 1982;57:572–576PubMedGoogle Scholar
  5. 5.
    Southall DP, Stebbens V, Shinebourne EA. Sudden and unexpected death between 1 and 5 years. Arch Dis Child 1987;62:700–705PubMedGoogle Scholar
  6. 6.
    Keeling JW, Knowles SA. Sudden death in childhood and adolescence. J Pathol 1989;159:221–224CrossRefPubMedGoogle Scholar
  7. 7.
    Neuspiel DR, Kuller LH. Sudden and unexpected natural death in childhood and adolescence. JAMA 1985;254:1321–1325CrossRefPubMedGoogle Scholar
  8. 8.
    Hoffman HJ, Damus K, Hillman L, Krongrad E. Risk factors for SIDS. Results of the National Institute of Child Health and Human Development SIDS Cooperative Epidemiological Study. Ann N Y Acad Sci 1988;533:13–30PubMedGoogle Scholar
  9. 9.
    Norman MG, Taylor GP, Clarke LA. Sudden, unexpected, natural death in childhood. Pediatr Pathol 1990;10:769–784PubMedGoogle Scholar
  10. 10.
    Helweg-Larsen K, Garde E. Sudden natural death in childhood. A review of forensic autopsy protocols in cases of sudden death between the ages of one and five years, 1982–1991, with a special view to sudden unexplained death. Acta Paediatr 1993;82:975–978PubMedGoogle Scholar
  11. 11.
    Wixom C, Chadwick AE, Krous HF. Sudden, unexpected death associated with meningioangiomatosis. A case report. Pediatr Dev Pathol 2005; April 7 Epub ahead of printGoogle Scholar
  12. 12.
    Aretz HT. Myocarditis: the Dallas criteria. Hum Pathol 1987;18:619–624PubMedGoogle Scholar
  13. 13.
    Aretz HT. Diagnosis of myocarditis by endomyocardial biopsy. Med Clin North Am 1986;70:1215–1226PubMedGoogle Scholar
  14. 14.
    Valdes-Dapena M, Gilbert-Barness E. Cardiovascular causes for sudden infant death. Pediatr Pathol Mol Med 2002;21:195–211CrossRefPubMedGoogle Scholar
  15. 15.
    Towbin JA, Vatta M, Li H, Bowles NE. Genetics and cardiac arrhythmias. Adv Pediatr 2002;49:87–129PubMedGoogle Scholar
  16. 16.
    Chimenti C, Pieroni M, Maseri A, Frustaci A. Histologic findings in patients with clinical and instrumental diagnosis of sporadic arrhythmogenic right ventricular dysplasia. J Am Coll Cardiol. 16 2004;43:2305–2313CrossRefGoogle Scholar
  17. 17.
    Hauck FR. Changing Epidemiology. In: Byard RW, Krous HF, eds. Sudden Infant Death Syndrome: Problems, Progress and Possibilities. London: Arnold, 2001;31–57Google Scholar
  18. 18.
    Krous HF, Nadeau JM, Silva PD, Blackbourne BD. Neck extension and rotation in sudden infant death syndrome and other natural infant deaths. Pediatr Dev Pathol 2001;4:154–159CrossRefPubMedGoogle Scholar
  19. 19.
    Beckwith JB. Intrathoracic petechial hemorrhages: a clue to the mechanism of death in sudden infant death syndrome? Ann N Y Acad Sci 1988;533:37–47PubMedGoogle Scholar
  20. 20.
    Krous HF. The microscopic distribution of intrathoracic petechiae in sudden infant death syndrome. Arch Pathol Lab Med 1984;108:77–79PubMedGoogle Scholar
  21. 21.
    Krous HF, Jordan J. A necropsy study of distribution of petechiae in non-sudden infant death syndrome. Arch Pathol Lab Med 1984;108:75–76PubMedGoogle Scholar
  22. 22.
    Krous HF, Nadeau JM, Silva PD, Blackbourne BD. Intrathoracic petechiae in sudden infant death syndrome: relationship to face position when found. Pediatr Dev Pathol 2001;4:160–166CrossRefPubMedGoogle Scholar
  23. 23.
    Poets CF, Meny RG, Chobanian MR, Bonofiglo RE. Gasping and other cardiorespiratory patterns during sudden infant deaths. Pediatr Res 1999;45:350–354PubMedGoogle Scholar
  24. 24.
    Kemp JS, Unger B, Wilkins D, et al. Unsafe sleep practices and an analysis of bedsharing among infants dying suddenly and unexpectedly: results of a four-year, population-based, death-scene investigation study of sudden infant death syndrome and related deaths. Pediatrics 2000;106:E41CrossRefPubMedGoogle Scholar
  25. 25.
    Hauck F. Bedsharing: review of epidemiologic data examining links to SIDS. Paper presented at the Infant Sleep Environment and SIDS Risk Conference; January 9–10, 1997; Bethesda, MDGoogle Scholar
  26. 26.
    Krous HF, Nadeau JM, Byard RW, Blackbourne BD. Oronasal blood in sudden infant death. Am J Forensic Med Pathol 2001;22:346–351CrossRefPubMedGoogle Scholar
  27. 27.
    Unger B, Kemp JS, Wilkins D, et al. Racial disparity and modifiable risk factors among infants dying suddenly and unexpectedly. Pediatrics 2003;111:E127–E131CrossRefPubMedGoogle Scholar
  28. 28.
    Mosko S, Richard C, McKenna J. Infant arousals during mother-infant bed sharing: implications for infant sleep and sudden infant death syndrome research. Pediatrics 1997;100:841–849CrossRefPubMedGoogle Scholar
  29. 29.
    Mosko S, Richard C, McKenna J, Drummond S, Mukai D. Maternal proximity and infant CO2 environment during bedsharing and possible implications for SIDS research. Am J Phys Anthropol 1997;103:315–328PubMedGoogle Scholar
  30. 30.
    Ackerman MJ, Siu BL, Sturner WQ, et al. Postmortem molecular analysis of SCN5A defects in sudden infant death syndrome. JAMA 2001;286:2264–2269CrossRefPubMedGoogle Scholar
  31. 31.
    Tester DJ, Spoon DB, Valdivia HH, Makielski JC, Ackerman MJ. Targeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner’s cases. Mayo Clin Proc 2004;79:1380–1384PubMedGoogle Scholar
  32. 32.
    Krous HF, Nadeau JM, Silva PD, Blackbourne BD. A comparison of respiratory symptoms and inflammation in sudden infant death syndrome and in accidental or inflicted infant death. Am J Forensic Med Pathol 2003;24:1–8CrossRefPubMedGoogle Scholar
  33. 33.
    Guntheroth WG. Interleukin-1 as intermediary causing prolonged sleep apnea and SIDS during respiratory infections. Med Hypotheses 1989;28:121–123CrossRefPubMedGoogle Scholar
  34. 34.
    Moscovis SM, Gordon AE, Hall ST, et al. Interleukin 1-beta responses to bacterial toxins and sudden infant death syndrome. FEMS Immunol Med Microbiol 2004;42:139–145CrossRefPubMedGoogle Scholar
  35. 35.
    Moscovis SM, Gordon AE, Al Madani OM, et al. Interleukin-10 and sudden infant death syndrome. FEMS Immunol Med Microbiol 2004;42:130–138CrossRefPubMedGoogle Scholar
  36. 36.
    Blackwell C. Infection, inflammation and SIDS. FEMS Immunol Med Microbiol 2004;42:1–2CrossRefPubMedGoogle Scholar
  37. 37.
    Byard R. Sudden Death in Infancy, Childhood and Adolescence, 2nd ed. Cambridge: Cambridge University Press, 2004Google Scholar
  38. 38.
    Krous HF, Chapman AJ, Altshuler G. Cardiac hemangioma: a rare (or possible) cause of sudden death in children. J Forensic Sci 1978;23:375–378PubMedGoogle Scholar
  39. 39.
    Barnham M, Henderson DC. Group B streptococcal infection presenting as sudden death in infancy. Arch Dis Child 1987;62:419–420PubMedGoogle Scholar
  40. 40.
    Barth CWd, Bray M, Roberts WC. Sudden death in infancy associated with origin of both left main and right coronary arteries from a common ostium above the left sinus of Valsalva. Am J Cardiol 1986;57:365–366CrossRefPubMedGoogle Scholar
  41. 41.
    Bohm N, Krebs G. Solitary rhabdomyoma of the heart. Clinically silent case with sudden, unexpected death in an 11-month-old boy. Eur J Pediatr 1980;134:167–172CrossRefPubMedGoogle Scholar
  42. 42.
    Byard RW, Bourne AJ, Silver MM. The association of lingual thyroglossal duct remnants with sudden death in infancy. Int J Pediatr Otorhinolaryngol 1990;20:107–112CrossRefPubMedGoogle Scholar
  43. 43.
    Byard RW, Keeley FW, Smith CR. Type IV Ehlers-Danlos syndrome presenting as sudden infant death. Am J Clin Pathol 1990;93:579–582PubMedGoogle Scholar
  44. 44.
    Byard RW, Bourne AJ, Adams PS. Subarterial ventricular septal defect in an infant with sudden unexpected death: cause or coincidence? Am J Cardiovasc Pathol 1990;3:333–336PubMedGoogle Scholar
  45. 45.
    Byard RW, Moore L. Total anomalous pulmonary venous drainage and sudden death in infancy. Forensic Sci Int 1991;51:197–202CrossRefPubMedGoogle Scholar
  46. 46.
    deSa DJ. Isolated myocarditis as a cause of sudden death in the first year of life. Forensic Sci Int 1986;30:113–117CrossRefPubMedGoogle Scholar
  47. 47.
    Dominguez FE, Tate LG, Robinson MJ. Familial fibromuscular dysplasia presenting as sudden death. Am J Cardiovasc Pathol 1988;2:269–272PubMedGoogle Scholar
  48. 48.
    Duran AC, Angelini A, Frescura C, Basso C, Thiene G. Anomalous origin of the right coronary artery from the left aortic sinus and sudden infant death. Int J Cardiol 1994;45:147–149CrossRefPubMedGoogle Scholar
  49. 49.
    Emile JF, Rambaud C, Canioni D, Jaubert F, Brousse N, Cheron G. [Myxoma of the pulmonary infundibulum as the cause of sudden death in an 18-month-old infant]. Arch Fr Pediatr 1993;50:323–325PubMedGoogle Scholar
  50. 50.
    Herrmann MA, Dousa MK, Edwards WD. Sudden infant death with anomalous origin of the left coronary artery. Am J Forensic Med Pathol 1992;13:191–195PubMedGoogle Scholar
  51. 51.
    Hussmann H, Hachmeister U. [Panarteritis nodosa or Kawasaki disease. Sudden death due to thrombotic occlusion of aneurysmatically dilated coronary arteries in a 1 year old boy]. Med Welt 1979;30:1715–1718PubMedGoogle Scholar
  52. 52.
    Jaffe R. Neuroma in the region of the atrioventricular node. Hum Pathol 1981;12:375–376PubMedGoogle Scholar
  53. 53.
    Lipsett J, Byard RW, Carpenter BF, Jimenez CL, Bourne AJ. Anomalous coronary arteries arising from the aorta associated with sudden death in infancy and early childhood. An autopsy series. Arch Pathol Lab Med 1991;115:770–773PubMedGoogle Scholar
  54. 54.
    Matsuura H, Kitazawa Y, Tanaka M, Morooka K. Pituitary adenoma and unexpected sudden infant death: a case report. Med Pediatr Oncol 1994;22:283–286PubMedGoogle Scholar
  55. 55.
    Ness MJ, McManus BM. Anomalous right coronary artery origin in otherwise unexplained infant death. Arch Pathol Lab Med 1988;112:626–629PubMedGoogle Scholar
  56. 56.
    Potter JM, Hilton JM. Type I hyperlipoproteinemia presenting as sudden death in infancy. Aust N Z J Med 1993;13:381–383Google Scholar
  57. 57.
    Rigle DA, Dexter RD, McGee MB. Cardiac rhabdomyoma presenting as sudden infant death syndrome. J Forensic Sci 1989;34:694–698PubMedGoogle Scholar
  58. 58.
    Russell MA, Opitz JM, Viseskul C, Gilbert EF, Bargman GJ. Sudden infant death due to congenital adrenal hypoplasia. Arch Pathol Lab Med 1977;101:168–169PubMedGoogle Scholar
  59. 59.
    Ruszkiewicz AR, Vernon-Roberts E. Sudden death in an infant due to histiocytoid cardiomyopathy. A light- microscopic, ultrastructural, and immunohistochemical study. Am J Forensic Med Pathol 1995;16:74–80PubMedGoogle Scholar
  60. 60.
    Speights VO Jr, Bauserman SC. Sudden death in an infant with central nervous system abnormalities. Pediatr Pathol 1991;11:751–758PubMedGoogle Scholar
  61. 61.
    Brodsky MC, Conte FA, Taylor D, Hoyt CS, Mrak RE. Sudden death in septo-optic dysplasia. Report of 5 cases. Arch Ophthalmol 1997;115:66–70PubMedGoogle Scholar
  62. 62.
    Mohammed W, Murphy A. Cardiac fibroma presenting as sudden death in a six-month-old infant. West Indian Med J 1997;46:28–29PubMedGoogle Scholar
  63. 63.
    Boissy C, Chevallier A, Michiels JF, et al. Histiocytoid cardiomyopathy: a cause of sudden death in infancy. Pathol Res Pract 1997;193:589–593PubMedGoogle Scholar
  64. 64.
    Grech V, Ellul B, Montalto SA. Sudden cardiac death in infancy due to histiocytoid cardiomyopathy. Cardiol Young 2000;10:49–51PubMedGoogle Scholar
  65. 65.
    Maresi E, Passantino R, Midulla R, et al. Sudden infant death caused by a ruptured coronary aneurysm during acute phase of atypical Kawasaki disease. Hum Pathol 2001;32:1407–1409CrossRefPubMedGoogle Scholar
  66. 66.
    Lunetta P, Karikoski R, Penttila A, Sajantila A. Sudden death associated with a multifocal type II hemangioendothelioma of the liver in a 3-month-old infant. Am J Forensic Med Pathol.2004;25:56–59PubMedGoogle Scholar
  67. 67.
    Parker KM, Embry JH. Sudden death due to tricuspid valve myxoma with massive pulmonary embolism in a 15-month-old male. J Forensic Sci.1997;42:524–526PubMedGoogle Scholar
  68. 68.
    Hals J, Ek J, Sandnes K. Cardiac myxoma as the cause of death in an infant. Acta Paediatr Scand 1990;79:999–1000PubMedGoogle Scholar
  69. 69.
    Bird LM, Krous HF, Eichenfield LF, Swalwell CI, Jones MC. Female infant with oncocytic cardiomyopathy and microphthalmia with linear skin defects (MLS): a clue to the pathogenesis of oncocytic cardiomyopathy? Am J Med Genet 1994;53:141–148CrossRefPubMedGoogle Scholar
  70. 70.
    Iyasu S, Rowley D, Hanzlick R. Guidelines for death scene investigation of sudden, unexplained infant deaths: recommendations of the Interagency Panel on Sudden Infant Death Syndrome. MMWR 1996;45(RR-10):1–6Google Scholar
  71. 71.
    Krous HF, Byard RW. International standardized autopsy protocol for sudden unexpected infant death. Appendix I. In: Byard RW, Krous HF, eds. Sudden Infant Death Syndrome: Problems, Progress and Possibilities. London: Arnold, 2001;319–333Google Scholar
  72. 72.
    Filiano JJ, Kinney HC. Arcuate nucleus hypoplasia in the sudden infant death syndrome. J Neuropathol Exp Neurol 1992;51:394–403PubMedGoogle Scholar
  73. 73.
    Kinney HC, Filiano JJ, Sleeper LA, Mandell F, Valdes-Dapena M, White WF. Decreased muscarinic receptor binding in the arcuate nucleus in sudden infant death syndrome. Science 1995;269:1446–1450PubMedGoogle Scholar
  74. 74.
    Panigrahy A, Filiano JJ, Sleeper LA, et al. Decreased kainate receptor binding in the arcuate nucleus of the sudden infant death syndrome. J Neuropathol Exp Neurol 1997;56:1253–1261PubMedGoogle Scholar
  75. 75.
    Nachmanoff DB, Panigrahy A, Filiano JJ, et al. Brainstem 3H-nicotine receptor binding in the sudden infant death syndrome. J Neuropathol Exp Neurol 1998;57:1018–1025PubMedGoogle Scholar
  76. 76.
    Kinney HC, Filiano JJ, Assmann SF, et al. Tritiated-naloxone binding to brainstem opioid receptors in the sudden infant death syndrome. J Auton Nerv Syst 1998;69:156–163CrossRefPubMedGoogle Scholar
  77. 77.
    Panigrahy A, Filiano J, Sleeper LA, et al. Decreased serotonergic receptor binding in rhombic lip-derived regions of the medulla oblongata in the sudden infant death syndrome [in process citation]. J Neuropathol Exp Neurol 2000;59:377–384PubMedGoogle Scholar
  78. 78.
    Panigrahy A, Filiano J, Sleeper LA, et al. Decreased serotonergic receptor binding in rhombic lip-derived regions of the medulla oblongata in the sudden infant death syndrome. J Neuropathol Exp Neurol 2000;59:377–384PubMedGoogle Scholar
  79. 79.
    Kinney HC, Randall LL, Sleeper LA, et al. Serotonergic brainstem abnormalities in Northern Plains Indians with the sudden infant death syndrome. J Neuropathol Exp Neurol 2003;62:1178–1191PubMedGoogle Scholar

Copyright information

© Society for Pediatric Pathology 2005

Authors and Affiliations

  • Henry F. Krous
    • 1
    • 2
  • Amy E. Chadwick
    • 1
  • Laura Crandall
    • 3
  • Julie M. Nadeau-Manning
    • 1
  1. 1.Departments of Pathology and PediatricsChildren’s Hospital and Health CenterSan DiegoUSA
  2. 2.Departments of Pathology and PediatricsUniversity of California, San Diego School of MedicineLa JollaUSA
  3. 3.CJ Foundation for SIDSHackensackUSA

Personalised recommendations