Changing Patterns of Congenital Anomalies over Ten Years in a Single Neonatal Intensive Care Unit

Sung Hoon Chung; Hee Young Kim; Ji Hee Kim; Yong-Sung Choi; Byong Sop Lee; Ki-Soo Kim; Ellen Ai-Rhan Kim

doi:10.14734/kjp.2013.24.1.11

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Chung, Kim, Kim, Choi, Lee, Kim, and Kim: Changing Patterns of Congenital Anomalies over Ten Years in a Single Neonatal Intensive Care Unit

Original Article

Korean J Perinatol 2013;24(1):11-19.

Published online: 19 March 2013

DOI: https://doi.org/10.14734/kjp.2013.24.1.11

Changing Patterns of Congenital Anomalies over Ten Years in a Single Neonatal Intensive Care Unit

Sung Hoon Chung, M.D., Hee Young Kim, R.N., Ji Hee Kim, M.D., Yong-Sung Choi, M.D., Byong Sop Lee, Ki-Soo Kim, M.D., Ellen Ai-Rhan Kim, M.D.

Division of Neonatology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan, College of Medicine, Seoul, Korea

책임저자 : 김애란, 138-736 서울특별시 송파구 올림픽로43길 88서울아산병원 소아청소년과 신생아과전화 : 02)3010-3382, 전송 : 02)3010-6978 E-mail : arkim@amc.seoul.kr

Received 1 October 2012 Revised 6 February 2013 Accepted 16 February 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose :

To investigate incidence, changing patterns, and mortality associated with congenital anomalies experienced in a single neonatal intensive care unit (NICU).

Methods :

Retrospective chart review of 29,026 neonates admitted to NICU and nursery of Asan Medical Center from January 2001 to December 2010 was done. The congenital anomalies were classified according to 76 anomalies in 8 systems registered by Korean Ministry of Health and Welfare in 2009.

Results :

A total of 3,122 neonates had at least 1 anomaly. Mean gestational age and birth weight were 38⁺²±2.3 weeks and 2,030±541 g respectively. The proportion of male is 61%. The incidence of congenital anomalies and the proportion of mothers older than 35 years increased from 8.3% to 23.0% and 6.4% to 16.4% in 2001 compared to 2010 respectively. The percentage of neonates who have multiple anomalies was almost equal from 24.0% in 2001 to 23.7% in 2010. The most common anomalies, by system, included atrial septal defect, hydronephrosis, anorectal atresia/stenosis, cystic adenomatoid malformation, cleft lip and/or palate, CATCH 22 syndrome, polydactyly, and hydrocephalus. The overall mortality at 2 years old decreased from 11.1% to 8.0% in 2001 and 2010. Most common etiologies resulting in highest mortality, by system, were hypoplastic left heart syndrome, renal agenesis, congenital diaphragmatic hernia, pulmonary hypoplasia, 18 trisomy, and anancephaly.

Conclusion :

Our data have shown that the incidence of congenital anomaly included in this study is increasing. A detailed epidemiologic study based on larger population is required in order to investigate preventive measures.

Keywords: Congenital anomalies, Single Neonatal Intensive Care Unit, Incidence, Mortality

REFERENCES

1). Yoon PW., Rasmussen SA., Lynberg MC., Moore CA., Anderka M., Carmichael SL, et al. The National Birth Defects Prevention Study. Public Health Rep. 2001. 116:Suppl.

2). Chung SH., Choi YS., Bae CW. Changes in the neonatal and infant mortality rate and the causes of death in Korea. Korean J Pediatr. 2011. 54:443–55.

3). Kim SK., Han HJ., Son SS., Hong SK., Hwang SU. A clinical review of congenital anomalies in newborn infants. Korean J Obstet Gynecol. 1984. 27:781–8.

4). Kim HJ., Kim CG., Ju GS. Clinical study for congenital anomalies in the newborn infants. Korean J Obstet Gynecol. 1988. 31:248–55.

5). Choi JH., Chung HJ., Yoon JG. Clinical studies on congenital malformations. J Korean Pediatr Soc. 1985. 28:74–81.

6). Lee HW., Whang IG., Lee KW., Kang JS. Clinical study of the congenital anomalies. Korean J Obstet Gynecol. 1990. 33:754–62.

7). Hwang CG., Lim BH., Kim KB. A clinical review of congenital anomalies in neonates. J Korean Pediatr Soc. 1988. 31:306–14.

8). Kwon YS., Oh HK., Kim JJ., Soh CO., Jung JH., Jung JY. Clinical study on congenital anomalies. J Korean Pediatr Soc. 1992. 35:315–21.

9). Yang YJ., Jung JY., Park SG. Statistical study on congenital anomalies. J Korean Soc Neonatol. 1997. 4:170–7.

10). Kim YW., Kim SJ., Hur SY., Lee GSR., Lee Y., Kim EJ, et al. The clinical study of congenital anomalies. Korean J Obstet Gynecol. 1998. 41:1698–703.

11). Koh KS., Kim A., Yang SH., Han JY., Kim ES., Kim MY, et al. Multi-center study for birth defects monitoring systems in Korea. Korean J Obstet Gynecol. 2001. 44:1609–16.

12). Jin K., Kim JS., Koh KS., Park CH. Clinical analysis of fetal congenital anomalies. Korean J Obstet Gynecol. 2002. 45:32–44.

13). Kang BH., Lee JG., Chung KH., Yang JB., Kim DY., Rhee YE, et al. Incidence of congenital anomalies and diagnosis of congenital anomalies by antenatal ultrasonography. Korean J Obstet Gynecol. 2004. 47:2070–6.

14). Choi JS., Seo K., Hahn YJ., Lee SW., Boo YG., Lee SW, et al. Congenital anomaly survey and statistics. Korea Ministry of Health and Welfare. 2009.

15). Tennant PW., Pearce MS., Bythell M., Rankin J. 20-year survival of children born with congenital anomalies: a population-based study. Lancet. 2010. 375:649–56.

16). Brent RL. Addressing environmentally caused human birth defects. Pediatr Rev. 2001. 22:153–65.

17). Garne E., Loane M., Wellesley D. Barisic I and a EUROCAT Working Group. Congenital hydronephrosis: prenatal diagnosis and epidemiology in Europe. J Pediatr Urol. 2009. 5:47–52.

18). Garnea E., Loaneb M., Addorc MC., Boydd PA., Barisice I., Dolkb H. Congenital hydrocephalus - prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol. 2010. 14:150–5.

19). Warkany J., Kalter H. Congenital malformations. N Engl J Med. 1961. 265:993–1001.

20). Marden PM., Smith DW., McDonald MJ. Congenital anomalies in the newborn infant, including minor variations. Pediatrics. 1964. 64:357–71.

21). Cho JS., Kim KS., Kim SY., Kim TY., Choi HM., Lim YG, et al. Congenital anomalies diagnosed by antenatal ultrasonography. Korean J Obstet Gynecol. 1997. 40:1228–32.

22). Bae CW. Neonatal epidemiology in Korea.

23). Kim A., Kim SR., Yang SH., Han JY., Kim MY., Yang JH, et al. Multi-center study for birth defects monitoring systems in Korea. Korean J Obstet Gynecol. 2002. 45:1924–31.

24). Kalter H. Five-decade international trends in the relation of perinatal mortality and congenital malformations: stillbirth and neonatal death compared. Int J Epidemiol. 1991. 20:173–9.

25). Wang Y., Hu J., Druschel CM. A retrospective cohort study of mortality among children with birth defects in New York State, 1983-2006. Birth Defects Res A clin Mol Teratol. 2010. 88:1023–31.

26). National Birth Defects Prevention Network (NBDPN). State birth defects surveillance program directory. Birth Defects Res A Clin Mol Teratol. 2009. 85:1007–55.

27). EUROCAT. EUROCAT Special Report: Prenatal Screening Policies in Europe. EUROCAT Central Registry, University of Ulster. 2005.

28). Wellesley D., Boyd P., Dolk H., Pattenden S. An aetiological classification of birth defects for epidemiological research. J Med Genet. 2005. 42:54–7.

29). International Clearinghouse for Birth Defects Surveillance and Research. International Clearinghouse for Birth Defects Surveillance and Research Annual report 2008. Roma, Italy: The International Centre on Birth Defects - ICBDSR Centre. 2008.

30). Wen SW., Liu S., Joseph KS., Rouleau J., Allen A. Patterns of infant mortality caused by congenital anomalies. Teratology. 2000. 61:342–6.

31). Korea Ministry of Health and Welfare. Infant mortality survey report in. 2011.

Fig. 1

The Annual incidence of congenital anomalies.

Fig. 2

Changes in annual percentage of mother older than 35, prematurity, and birth weight under 2,500 g.

Fig. 3

Changes in the proportion of major anatomical system, 2001-2010.

Fig. 4

Changes in mortality rate by two ages of congenital anomaly from 2001 to 2010. CA, congenital anomaly; CHD, congenital heart disease.

Table 1.

Classification and Number of Selected Congenital Anomalies, 2001-2010

System	Disease	No. of anomaly for 10 years
Central nervous system	Anencephaly	1
	Spinabifida	45
	Encephalocele	2
	Microcephaly	6
	Arhinencephaly/ Holoprosencephaly	25
	Congenital Hydrocephalus	47
Eye, Ear, Face	Anophthalmos	1
	Microphthalmos	3
	Congenital cataract	7
	Aniridia	0
	Congenital glaucoma	7
	Anotia	1
	Microtia	10
	Cleft lip and/or palate	173
	Choanal Atresia	7
	Macroglossia	1
Respiratory system	Sequestration of lung	101
	Congenital cystic adenomatoid malformation(CCAM)	123
	Hypoplasia and dysplasia of lung	13
GI system	Esophageal atresia/stenosis	79
	Anorectal atresia/stenosis	138
	Small intestine atresia/stenosis	77
	Duodenal atresia or stenosis	50
	Congenital megacolon	88
	Atresia of bile duct	11
	Annular pancreas	15
	Duplication of intestine	13
	Duplication of stomach	5
	Omphalocele	31
	Gastroschisis	14
	Diaphragmatic hernia	115
Circulatory system	Ventricular septal defect	466
	Atrial septal defect	628
	Patent ductus arteriosus	430
	Coarctation of aorta	106
	Aortic valve stenosis, Pulmonary valve stenosis	117
	Tetralogy of Fallot	174
	Transposition of great artery (TGA)	177
	Pulmonary valve atresia	87
	Tricuspid valve atresia	24
	Ebstein's anomaly	33
	Hypoplastic left heart syndrome	23
	Single ventricle	89
	Common truncus	11
	Total anomalous pulmonary venous return	52
Genitourinary system	Renal agenesis	24
	Hypospadias	77
	Epispadias	2
	Bladder exstrophy	6
	Renal dysplasia	17
	Cystic kidney	109
	Indeterminate sex	26
	Undescended testis	131
	Obstructive genitourinary defect	116
	Persistent cloaca	14
	Congenital hydronephrosis	302
Musculoskeletal system	Reduction deformity, upper limbs	4
	Reduction deformity, lower limbs	10
	Total Limb reduction defects (include unspecified)	0
	Congenital hip dislocation	6
	Club foot - talipes equinovarus	6
	Polydactyly	60
	Syndactyly	39
	Arthrogryposis multiplex congenital	1
	Craniosynostosis	7
	Achondroplasia/ Hypochondroplasia	14
Chromosome, gene disorder	Trisomy 13 Trisomy 18	5 7
	Down's syndrome	48
	Turner's syndrome	3
	Kleinfelter's syndrome	4
	Wolff-Hirschorn syndrome	2
	CATCH22 syndrome	104
	Beckwith-wiedemann syndrome	4
	Russell-Silver syndrome	1
	Prader-willi syndrome	19

Table 2.

Demographic Data of Congenital Anomaly

Variables	Number	Range
Total	29,026
Infants of congenital anomaly	3,122 (10.8%)
Gestational age (week)	38⁺²±2.3	25⁺³-42⁺¹
Birth weight (g)	3,030±541	396-4,740
Male	1,903 (61.0%)
Maternal age (yr)	31±4.0
Prematurity	391 (12.5%)
LBWI	349 (11.2%)

Abbreviation: LBWI, low birth weight infants ∗Average±SD (standard deviation)

Table 3.

The Incidence of Congenital Anomaly by Birth Weight, Gestational Age, and Maternal Age

		Infants of congenital anomaly
		No.	(%)
Birth weight (gm)	< 1,500	29	0.9
	1,500-2,499	320	10.2
	≥ 2,500	2,765	88.6
	Unknown	8	0.3
Gestational age (wk)	< 37	391	12.5
	37-42	2,707	86.7
	≥ 42	9	0.3
	Unknown	15	0.5
Maternal age (yr)	< 20	17	0.6
	20-35	2,367	75.8
	≥ 35	503	16.1
	Unknown	235	7.5

Table 4.

Congenital Anomalies by Major Anatomica System, 2001-2010

System	No.	(%)
Circulatory	2,415	50.4
Genitourinary	824	17.2
Gastrointestinal	636	13.3
Respiratory	237	4.9
Eye, Ear, Face	210	4.4
Chromosome, gene	197	4.1
Musculo-skeletal	147	3.1
Central Nervous	126	2.6
Total	4,792	100

Table 5.

The Annual Incidence of Multiple Congenital Anomalies

Year	Infants of multiple anomalies	2 anomalies	>2 anomalies	Infants of congenital anomaly	Percent(%)
2001	52	29	23	217	24
2002	53	27	26	208	25.5
2003	64	33	31	219	29.2
2004	63	33	30	235	26.8
2005	65	35	30	273	23.8
2006	70	38	32	302	23.2
2007	55	24	31	367	15
2008	59	30	29	373	15.8
2009	92	39	53	413	22.3
2010	122	52	70	515	23.7
Total	695	340	355	3,122	22.7

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