|
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 * |