Maternal choledochal cysts in pregnancy: A systematic review of case reports and case series

Table 1 Article/study characteristics of choledochal cysts during pregnancy

Category	n (%)
Type of study
Case reports	56 (78.8)
Two cases	8 (11.2)
Case series (> 2)	7 (9.8)
Country
United States	21 (29.6)
India	9 (12.7)
Japan	8 (11.3)
United Kingdom	7 (9.9)
China	7 (9.9)
South Korea	3 (4.2)
Mexico	3 (4.2)
Other	13 (18.3)
West vs East	38 vs 33 (46.5 vs 53.5)
Year of publication
< 1940	2 (2.1)
1940-1960	8 (8.2)
1960-1980	5 (5.2)
1980-2000	20 (20.6)
2000-2020	36 (37.1)
Data completion
Age	97 (100)
Gestational age	97 (100)
Cyst size	67 (69)
Cyst type	80 (81.4)
Bilirubin levels	70 (72.1)
Maternal outcome	97 (100)
Fetal outcome	21 (21.6)

Table 2 Patient and choledochal cyst characteristics, n (%)

Category	Results	Number of cases with reported variable
Age	25.3 ± 4.03	97
Gestational age at cyst discovery	26.1 ± 8.2	96
Parity	1.37 ± 0.76	83
1	62 (74.6)
2	13 (15.6)
> 2	8 (9.6)
Cyst size (cm)	12.5 ± 6.4	67
0-5	6 (8.9)
5-10	20 (29.8)
10-15	19 (28.3)
15-20	16 (23.8)
> 20	6 (8.9)
Bilirubin (mmol/L)	83.2 ± 68.4	71
Cyst type	12.5 ± 6.4	84
I	62 (73.8)
IV	15 (17.8)
V	6 (7.1)
Diagnostic method		93
US	58 (62.3)
MRI	18 (19.3)
ERCP	2 (2.1)
CT	2 (2.1)
Intraoperatively	13 (13.9)
Reached gestational age (wk)		97
0-12	2 (2.2)
12-24	7 (7.2)
24-36	43 (44.3)
> 36	45 (46.3)

US: Ultrasound; MRI: Magnetic resonance imaging; ERCP: Endoscopic retrograde cholangiopancreatography; CT: Computed tomography.

Table 3 Symptomatology of maternal choledochal cysts

Symptom	n (%)
Pain	78 (81.2)
Jaundice	58 (60.4)
Fever	33 (34.3)
Nausea/vomiting	36 (37.5)
Abdominal mass	42 (43.7)
Asymptomatic	8 (8.3)
Charcot triad (fever, jaundice, pain)	28 (29.1)
Triad of choledochal cyst symptoms (abdominal mass, jaundice, pain)	41 (42.7)

Table 4 General management characteristics

Category	Management
Choledochal cyst	Expectative	Intervention	Expectative	Intervention
Pregnancy	Expectative	Expectative	Intervention	Intervention
Cases, n (%)	29 (29.8)	35 (36.0)	17 (17.5)	16 (16.4)

Table 5 Indications and types of intervention

Category	n	%
CC treatment
Conservative	46	47.9
Intervention	51	53.1
Surgical resection and reconstruction	22	22.9
Percutaneous drainage	16	16.6
Surgical drainage	7	7.2
Cholecystostomy	3	3.1
ERCP	3	3.1
Pregnancy
Expectative approach	67	69.7
Vaginal labor	35	36.4
Planned Cesarean section	12	12.5
Spontaneous abortion	2	2
Induced vaginal labor	3	3.1
NR	15	15.6
Intervention	33	34.3
Cesarean section	24	25
Induced vaginal labor	3	3.1
Induced abortion/curettage	2	2
NR	4	4.1
Indications for CC management
Cholangitis	22	22.9
Jaundice	9	9.3
Rupture	6	5.2
Sepsis	4	4.1
Pylorus obstruction	2	3.1
Bleeding	2	2
NR	9	9.3
Indications for pregnancy management
CC complications	11	11.4
Fetal compromise	6	6.2
Severe anemia	2	2
Placental abruption	2	2
Preeclampsia	5	5.2
Prevention of potential CC complications	7	7.2

CC: Choledochal cyst; NR: Not reported; ERCP: Endoscopic retrograde cholangiopancreatography.

Table 6 Maternal and fetal mortality

Outcomes	n (%)
Maternal mortality	7 (7.2)/6 (6.1)1
Peritonitis, cyst rupture	3
Surgery complications	2
Not specified	2
Fetal mortality	8 (8.2)
Spontaneous abortion	2
Induced abortion	2
Stillborns	2
Newborn deaths	2

¹The patient who died 1 year postpartum was excluded from the mortality analysis.

Table 7 Factors influencing the likelihood of intervention during pregnancy

Parameter	OR	P value	95%CI	Z statistic
Presence of the Charcot triad	4.3	0.006	1.57-11.1	3.35
Presence of the CC triad	2.88	0.017	1.20-6.92	2.37
Bilirubin (≥ 80 mmol/L vs < 80 mmol/L)	1.37	0.78	1.42-15.20	0.84
CC size (≥ 15 cm vs < 15 cm)	4.6	0.011	0.28-3.29	3.72
CC type (I vs IV)	0.96	0.97	0.34-1.80	0.15
Parity (nulliparous vs multiparous)	0.79	0.57	0.42-2.45	0.55
Gestational age (≥ 6 mo vs < 6 mo)	1.01	0.96	0.41-1.66	0.04

OR: Odds ratio; CC: Choledochal cyst.