Cytomegalovirus |
Reovirus Type 3 |
Rubella |
Coxsackie B |
Hepatitis |
HIV |
Herpes |
Echovirus |
Gram Negative |
Syphilis |
Toxoplasmosis |
Tuberculosis |
Biliary Atresia |
Spontaneous Perforation of bile ducts |
Choledochal Cyst |
Cholelithiasis |
Bile ducts Strictures |
Alpha-1-antitrypsin deficiency |
Alagille's syndrome |
Galactosemia |
Hypopituitarism |
Fructosemia |
Polysplenic syndrome |
Tyrosinemia |
TPN |
Glycogenosis |
Cystic Fibrosis |
Gaucher's disease |
Congenital Hepatic Fibrosis |
Niemann-Pick |
Byler's disease |
Hypothyroidism |
Turner Syndrome |
Post NEC |
Congenital Heart Failure |
Left hypoplastic heart syndrome |
Sepsis |
Shock/Hypoperfusion |
Drug Induced |
Cholangitis | |
Sudden Cease of Bile Flow | |
Portal Hypertension | |
Esophageal Varices | |
Hypersplenism | |
Ascites | |
Essential Fatty Acid deficiency | |
Vitamisn Malabsorbtion (A,D,K,E) | |
Essential Metals deficiency | |
Pruritus |
Preoperative Factors | |
age at surgery | |
hepatic histology | |
race | |
Operative Factors | |
morphologic type | |
size ductular structures at porta hepatis | |
surgical technique | |
type of surgical reconstruction | |
Postoperative Factors | |
bile flow | |
cholangitis | |
portal hypertension |
Series |
Year |
# Pts. |
5-yr Survival |
10-yr Survival |
20-yr Survival |
Carcassone et al(50) |
1977 |
532 |
12.7% |
- |
- |
Akiyama et al(51) |
1978 |
90 |
41.1% |
- |
- |
Kobayashi (52) |
1984 |
135 |
29% |
- |
- |
Tohoku University Hospital (13) |
1953-1991 |
274 |
35% |
33.5%* |
- |
Altman (30) |
1974-1986 |
225 |
59.5% |
33% |
- |
National Biliary Atresia Registry (20) |
1976-1989 |
670 |
48% |
35%** |
- |
Toyosake et al (49) |
76 |
- |
27.6% |
7.8% |
* of 194 patients who initially survived** approximation of the graphic of survival
MALES |
FEMALES |
TOTAL |
|
0-2 mo. |
16 |
10 |
26 (16) |
2-12 mo. |
31 |
22 |
53 (33) |
12-24 mo. |
15 |
3 |
18 (11) |
2-5 y/o |
21 |
20 |
41 (26) |
> 6 y/o |
6 |
17 |
23 (14) |
TOTAL |
72 |
161 (100) |
MALES |
FEMALES |
TOTAL |
|
RIH |
37 |
32 |
69 (43) |
LIH |
29 |
18 |
47 (29) |
BIH |
23 |
22 |
45 (28) |
TOTAL |
89 |
72 |
161 (100) |
No. in ( ) are %, RIH= right inguinal hernia, LIH= left inguinal hernia,
BIH= bilateral inguinal hernia
Respiratory illness (i.e. asthma) |
40 (25) |
Congenital Heart Disease (VSD, PDA) |
6 (4) |
GU | |
Hydrocele |
23 (14) |
Cryptorchidism |
3 (2) |
Hypospadia |
1 (0.6) |
Retractile testis |
1 (0.6) |
Polycystic Kidneys |
1 (0.6) |
Umbilical Hernia |
10 (6) |
Neurological | |
V-P Shunt |
1 (0.6) |
No in ( ) are %
INITIAL DIAGNOSIS |
HS |
PPV |
OPV |
RIH 69 pts. |
28 (41) |
23 (33) |
18 (26) |
LIH 47 pts. |
19 (40) |
13 (28) |
15 (32) |
TOTAL 116 pts. |
47 (41) |
36 (31) |
33 (28) |
Residual hydrocele | 2 (1.2) |
Post-op Apnea | 1 (0.6) |
Post-intubation Croup | 1 (0.6) |
Testicular edema/atrophy | 0 |
Recurrence | 0 |
CASES |
+ FINDINGS |
- FINDINGS |
PROBABILITY |
|
(HS + PPV) |
(OPV) |
|||
SEX | ||||
Males |
66 |
37 |
29 |
NSS |
Females |
50 |
46 |
4 |
p < 0.0001 |
GESTATIONAL AGE | ||||
Term |
63 |
44 |
19 |
NSS |
Prematures |
14 |
9 |
5 |
NSS |
AGE AT OPERATION | ||||
0-2 mo |
16 |
15 |
1 |
p < 0.0005 |
2-12 mo |
42 |
25 |
17 |
NSS |
12-24 mo |
17 |
7 |
10 |
NSS |
2-5 y/o |
28 |
24 |
4 |
p < 0.01 |
> 6 y/o |
13 |
12 |
1 |
p < 0.005 |
HS= hernial sac, PPV= patent procesus vaginalis, OPV= obliterated procesus
vaginalis, NSS= not statistically significant.
OC (16) |
LC (24) |
probability |
|
Age (years) |
7.9 |
11.9 |
0.01 * |
Hx prior surgery |
5 |
1 |
0.04 * |
Cholangiogram |
7 |
1 |
0.006 * |
Prophylactic Antibiotics |
17 |
16 |
0.01 * |
OR time (min) | 114 | 84 | 0.04 * |
Postoperative Complications | 7 | 4 | 0.1 |
Diet Resumption (days) | 1.8 | 0.6 | 0.0004 * |
Pain medication (days) | 1.8 | 0.3 | 0.00009 * |
Hospital stay (days) | 7.2 | 3.1 | 0.003 * |
LC (59) |
OC (24) |
probability |
|
Age (years) |
15.1 |
13.8 |
NSS |
Sex (F:M) |
49:10 |
14:10 |
0.02 * |
Obesity |
11 |
3 |
NSS |
Emergency |
13 |
11 |
0.03 * |
Abnormal Liver Chemistry |
12 |
8 |
NSS |
Associated Illness |
27 |
8 |
NSS |
Post-surgical stay (days) |
1.5 |
3.3 |
0.0004 * |
Cholangiogram |
12 |
14 |
0.007 * |
OR time (min) |
94 |
138 |
0.03 * |
Surgical time (min) |
53 |
98 |
0.003 * |
Hospital stay (days) |
2.3 |
4.6 |
0.000001 * |
Complications |
12 |
10 |
NSS |
Diet (days) |
0.8 |
1.5 |
0.00000 * |
Pain medication (days) |
0.7 |
1.4 |
0.00000 * |