TABLE 6.
Frequency of Major Surgery by Type of BD Among VLBW Infants in the NRN Cohort Born in 1998–2007 Who Survived > 3 Days
| Category | No. | n (%) Who Had Major Surgery Before Dischargee |
|---|---|---|
| Chromosomal anomalies | ||
| Trisomy 13 | 13 | 3 (23.1) |
| Trisomy 18 | 49 | 2 (4.1) |
| Trisomy 21 (Down syndrome) | 87 | 36 (41.4) |
| Turner syndrome | 9 | 2 (22.2) |
| Triploidy | 9 | 1 (11.1) |
| 22q deletion | 12 | 7 (58.3) |
| Klinefelter syndrome | 5 | 1 (20.0) |
| Wolf-Hirschhorn syndrome | 4 | 1 (25.0) |
| Other chromosomal abnormalities | 66 | 22 (33.3) |
| Total | 254 | 75 (29.5) |
| Named syndromes, sequences, and associationsa | 65 | 30 (46.2) |
| Nervous system | ||
| Anencephaly | 0 | — |
| Meningomyelocele ± hydrocephalus | 15 | 13 (86.7) |
| Congenital hydrocephalus | 16 | 8 (50.0) |
| Hydranencephaly | 3 | 0 (0) |
| Holoprosencephaly | 7 | 0 (0) |
| Myotonic dystrophy/myopathy | 7 | 3 (42.9) |
| Spina bifida | 1 | 1 (100) |
| Other nervous system anomalies | 63 | 24 (38.1) |
| Total | 112 | 49 (43.8) |
| Cardiovascularb | ||
| Tetralogy of Fallot ± pulmonary atresia | 37 | 15 (40.5) |
| Transposition of great vessels | 15 | 11 (73.3) |
| Pulmonary atresia | 9 | 3 (33.3) |
| Truncus arteriosus | 3 | 0 (0) |
| Total anomalous pulmonary venous return | 4 | 1 (25.0) |
| HLHS | 13 | 3 (23.1) |
| Interrupted aortic arch | 7 | 3 (42.9) |
| Coarctation of aorta | 22 | 16 (72.7) |
| Complete atrioventricular canal | 7 | 2 (28.6) |
| Single ventricle | 0 | — |
| Double outlet right ventricle | 10 | 5 (50.0) |
| Tricuspid atresia | 0 | — |
| Other single cardiovascular anomalies | 121 | 34 (28.1) |
| Multiple cardiovascular anomalies | 75 | 38 (50.7) |
| Total | 323 | 131 (40.6) |
| Gastrointestinal anomalies | ||
| Esophageal atresia and/or tracheoesophageal fistula | 42 | 39 (92.9) |
| Duodenal/jejunal/ileal atresia | 39 | 37 (94.9) |
| Atresia of large bowel or rectum | 2 | 2 (100) |
| Imperforate anus | 9 | 8 (88.9) |
| Gastroschisis | 38 | 36 (94.7) |
| Omphalocele | 17 | 13 (76.5) |
| Diaphragmatic hernia | 7 | 5 (71.4) |
| Other gastrointestinal anomalies | 51 | 43 (84.3) |
| Total | 205 | 183 (89.3) |
| Genitourinary | ||
| Bilateral renal agenesis | 0 | — |
| Bilateral polycystic, multicystic, or dysplastic kidneys | 15 | 4 (26.7) |
| Obstructive uropathy with congenital hydronephrosis | 29 | 13 (44.8) |
| Exstrophy of the urinary bladder | 2 | 1 (50.0) |
| Other genitourinary anomalies | 51 | 17 (33.3) |
| Total | 97 | 35 (36.1) |
| Bone and skeletal anomalies | 21 | 5 (23.8) |
| Miscellaneous single system anomaliesc | 63 | 27 (42.9) |
| Inborn errors of metabolism | 36 | 8 (22.2) |
| Other multiple system anomalies | 137 | 88 (64.2) |
| Total | 1313d | 631 (48) |
Named syndromes, sequences, and associations among survivors > 3 days include the following: adducted thumb syndrome (1), amniotic band syndrome (6), Alagille syndrome (1), Baller-Gerold syndrome (1), Bart syndrome (1), Beals syndrome (1), Beckwith-Wiedemann syndrome (3), branchiootorenal (BOR) syndrome (1), caudal regression syndrome (1), CHARGE syndrome (2), Cornelia de Lange syndrome (9), Ehlers-Danlos syndrome (1), Fanconi syndrome (1), Fryns syndrome (1), Goldenhar syndrome (9), Goltz syndrome (1), Holt-Oram syndrome (1), Hurler syndrome (1), Jarcho Levin syndrome (1), Larsen syndrome (1), Miller-Dieker syndrome (1), Moebius syndrome (1), Nager syndrome (1), Noonan syndrome (1), Opitz syndrome (2), Pena-Shokeir syndrome (1), Peters plus syndrome (1), Pierre Robin syndrome (5), Poland syndrome (1), Roberts syndrome (1), Saethre-Chotzen syndrome (1), Stickler syndrome (1), thrombocytopenia-absent radius (TAR) syndrome (1), Townes-Brocks syndrome (1), and Waardenburg syndrome (2).
Thirty-three infants born in 2006–2007 who had an anomaly coded “other congenital heart defects” and survived >3 days were counted in the “other single cardiovascular anomalies” category. It was not possible to determine whether a single cardiovascular anomaly was present or multiple cardiovascular anomalies were present as a descriptive text field was not available during these 2 years.
Includes cleft palate (36 infants), congenital cystic adenomatoid malformation, and other anomalies.
Of infants with BDs, 1315 survived >3 days. Surgery information was missing for 2 infants with cardiovascular defects who survived >3 days: 1 infant with transposition of the great vessels and 1 with truncus arteriosus. One infant with a BD who died after 12 hours but for whom more precise timing of death was unknown was not included among survivors >3 days.
Where the number of infants with a specific birth defect is 0, a dash indicates that no percent is shown.