Journal List > Korean J Perinatol > v.24(1) > 1013672

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

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.

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Fig. 1
The Annual incidence of congenital anomalies.
kjp-24-11f1.tif
Fig. 2
Changes in annual percentage of mother older than 35, prematurity, and birth weight under 2,500 g.
kjp-24-11f2.tif
Fig. 3
Changes in the proportion of major anatomical system, 2001-2010.
kjp-24-11f3.tif
Fig. 4
Changes in mortality rate by two ages of congenital anomaly from 2001 to 2010. CA, congenital anomaly; CHD, congenital heart disease.
kjp-24-11f4.tif
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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