Figure 1 Kaplan Meier survival function by baseline ART regimen

 

Congenital anomaly

Perinatal complications

Delivery method

Outcome

Case 1

Bladder exstrophy

Miscarriage X1
Renal calculus
Hydronephrosis
Recurrent UTIs
Pyelonephritis
Urosepsis
Threatened preterm labour
Cervical insufficiency

Emergency Classical caesarean section due to preterm labour

Nil significant maternal complications
Fetal:
Prematurity
Respiratory distress

Case 2a

Bladder exstrophy

Miscarriage X1 (twins)
Recurrent UTIs
Hypertension
Malpresentation
Uterine prolapse
Bicornuate uterus

Elective caesarean section

Maternal: small bowel injury
Fetal: respiratory distress

Case 2b

2nd pregnancy for case 2
Bladder exstrophy

Recurrent UTIs

Elective caesarean section

Maternal: minor bladder serosal tear
Fetal:
Minor respiratory distress

Case 3a

Cloacal exstrophy

Miscarriage X1
Malpresentation
Bicornuate uterus
Recurrent UTIs

Elective caesarean section

Nil significant complications

Case 3b

2nd pregnancy for case 3
Cloacal exstrophy

Recurrent UTIs
Low lying placenta
Stress incontinence
APH
Malpresentation

Emergency caesarean section

Maternal: Indwelling catheter pushed through a fold into vagina
Fetal
Nil

Table2 Summary of the cases

Study (Arthors)

Year

Number of women

Number of pregnancies

Outcomes

Gestation at delivery

Mode of delivery

Ref

Dy et al.

2015

12

22

Live birth 14 (64%), Terminations 4(18%), Spontaneous abortion <24 weeks 4(18%).

Mean gestational age at delivery: 36 weeks

Caesarean section in all 14 cases

22

Deans et al.

2012

52

57

Live birth 34 (56%), Miscarriage 21 (35%), Termination 1 (2%), Stillbirth 4 (7%)

Median gestational age of all live births was 37 weeks.

All patients deliered by cesarean section of which 3 were emergencies. 3 classical cesarean sections.

18

Ebert et al.

2011

2

3

Live birth 2 (67%); Miscarriage 1 (33%)

one at 39 weeks, one at 33 weeks

1 by elective LUSCS, 1 by emergency LUSCS at 33+4/40 due to preterm labour

19

Rubenwolf et al.

2016

12

17

Live birth 16 (94%); Miscarriage 1(6%)

Not mentioned

All delivered by elective caesarean section

24

Eswara et al.

2016

 

 

 

Mean gestational age at delivery: 36 weeks

 

23

Schumacher et al.

1997

6

7

Live birth 7(100%)

Not mentioned

All delivered by caesarean section

12

Mathews et al.

2003

6

11

Live birth 7 (63%), Miscarriage 2(18%), TOP 2 (18%)

Not mentioned

6 by caesarean section, 1 had uneventful vaginal birth

11

Gezer et al.

2011

1

1

Live birth 1 (100%)

36 weeks

Emergency Classical C/Section due to preterm labour

20

Giron et al

2011

14

22

Healthy babies 17 (77.2%); Miscarriage 4 (18.1%); Neonatal death 1 (4.7%)

Not mentioned

All babies were delivered through caesarean section ith the exception of one that had a premature normal birth

9

Bildirin et al.

2012

1

1

Live birth 1 (100%)

34

Vaginal birth

16

Greenwell et al.

2003

20

37

Live birth 29 (78%), Miscarriage 6 (16%), TOP 2 (5%)

Mean gestational age at delivery: 36.9 weeks (35-40)

Elective Caesarean section 13 (43%), Emergency Caesarean 7 (23%); vaginal birth 10 (34%)

10

Burbigi et al

1986

5

7

Live birth 6 (86%), 1 TOP (14%)

Not mentioned

3 caesarean sections (50%); 3 vaginal birth (50%)

17

Mantel et al.

2000

3

6

Live birth 2 (33%), Miscarriage 4 (66%)

35 and 36 weeks

 Emergency Caesarean sections for malpresentation in labour (100%)

1

Table1 Literature summary of the pregnancy outcomes of the women with congenital bladder exstrophy or cloacal exstrophy

Perinatal issues

Perinatal management

High risk for multiple complications

Pregnancy to be considered high risk, collaborative approach between an experienced obstetrician with skills in paediatric and adolescent gynaecology and a urologist with experience in congenital anomaly reconstructive surgery for antenatal care and delivery planning

Fertility issues(1)

Aware and management as per conventional treatment for sub fertility

1:70 risk of congenital bladder exstrophy in the fetus which is a 500 fold increase over the general population(2)

Prenatal USS for detection

Urinary tract obstruction; Hydronephrosis

USS of the renal tract, first at 16 weeks, thereafter Q6-8 weeks, stenting or nephrostomy if obstructed;(3) recommend percutaneous drainage of the kidneys for the following indications: pain, progressive hydronephrosis, rising serum creatinine, febrile UTIs, recommend not removing the nephrostomy tubes until 1 week postpartum. Biggest risk period for obstruction appears to occur between 20 and 28 weeks of gestation, patient at risk may require USS every 2 weeks during that time(4)

Urinary tract infections

Baseline renal function and urine microscopy; screening for asymptomatic bacteriuria weekly; adequate hydration; proactively completely empty bladder at regular intervals advised; prophylactic antibiotics if recurrent UTIs, treat only febrile UTIs (3)

Stomal prolapse or parastomal hernia

Typically resolve after delivery

Worsening renal function, development of end stage renal failure (4)

Close monitoring, regular renal consultation.

Catherisation difficulties

Indwelling catheter or change the type and length of the catheter used (e.g. Coude tip  catheter)(4)

Cervical incompetence

Monitor cervical length

Preterm labour

Having a planned delivery at late preterm gestations after steroid cover

Miscarriage

Early USS to confirm gestation, identify anomalies

Malpresentation

Planned delivery, aware of the presentation at the time of the delivery

Renal stone

Adequate hydration, proactively completely empty bladder at regular intervals, if suspicious of renal stone, USS and urology management

Urinary incontinence

Generally recover to pre-pregnancy status postpartum

Uterine prolapse including cervical prolapse

Cervical pessary, a diaphragm fitting ring, bed rest for late gestation(5)

Hypertensive disorders

Monitoring of the blood pressure and blood biochemistry and urine protein

Low lying placenta with antepartum haemorrhage

USS for detection, management according to standard low lying placenta management protocol

Delivery

Elective Caesarean section in the exstrophy population was the clear consensus.(4) Planned caesarean section at late preterm gestations after steroid cover; Surgery to be done by experienced obstetrician with urologist in theatre, with suitable neonatology service on site; High midline abdominal skin and wall incision, lower segment uterine incision or classical uterine incision pending on status of adhesion. Vaginal birth only is considered in uncomplicated pregnancies.(6)

Table3 Summary of recommendations of pregnancy management and delivery)